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Sample records for developmental hip dysplasia

  1. Relationship between flexible flat foot and developmental hip dysplasia.

    Science.gov (United States)

    Ponce de León Samper, M C; Herrera Ortiz, G; Castellanos Mendoza, C

    2015-01-01

    To evaluate the possible relationship between flexible flat foot and developmental hip dysplasia in children between six and 15 years of age. Cross-sectional study including 65 patients that had undergone surgery due to residual hip dysplasia or hip dislocation and compared against 75 healthy patients. Flexible flat foot prevalence was measured in each group, with the results showing that 61% of the group with residual hip dysplasia or hip dislocation had this condition, vs. 12% in the healthy group. The statistical analysis shows that the chances of suffering from flexible flat foot, are five times greater in the hip dysplasia or hip dislocation group, than in the healthy group. There is no evidence in the literature showing a relationship between these two conditions, even though they have a common etiology. This study shows a potential measurable relation between this two conditions. Patients with hip dysplasia or dislocation may have a higher chance of presenting flexible flat foot during late childhood, adolescence and adulthood, a fact that suggests a relationship between these two pathologies. Also, patients who seek assistance for the first time because of a flexible flat foot condition without having been evaluated during the first year of life for hip dysplasia, would be better off if evaluated for residual hip dysplasia. Copyright © 2014 SECOT. Published by Elsevier Espana. All rights reserved.

  2. Hip Ultrasonography in the Diagnosis of Developmental Dysplasia of the Hip: Bakırköy Experience

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    Altuğ Duramaz

    2014-12-01

    Full Text Available Aim: The purpose of the study was to determine the prevalence, incidence, and etiology as well as the risk factors for developmental dysplasia of the hip in newborns in whom we performed ultrasonography for screening using Graff’s method in our clinic. Methods: We retrospectively evaluated 2632 hip ultrasonography records of 1316 babies performed between 2008 and 2013. We analyzed the questionnaires of the Turkish Pediatric Orthopaedic Society which were filled by the physician during examination. The babies were divided into two groups according to ultrasonographic hip angles as pathological and normal. Results: The study is made on 1316 babies [680 girls (51.6%, 636 boys (48.4%]. The risk for developmental dysplasia of the hip was higher in girls, babies with a family history, babies with metatarsus adductus and those have been swaddled before. The mean gestational age and gestational weight was statistically significantly lower in the pathological group (p=0.0011. Conclusion: In our cross-sectional study, the incidence of developmental dysplasia of the hip was 0.5%. Female gender, positive family history of developmental dysplasia of the hip,metatarsus adductus and swaddling are still risk factors. Researching risk factors carefully, patient education and adding hip ultrasonograpy to newborn routine screening program are important measures in preventing developmental dysplasia of the hip.

  3. Reconstruction of the Acetabulum in Developmental Dysplasia of the Hip in Total Hip Replacement

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    Vasileios Sakellariou

    2014-09-01

    Full Text Available Developmental dysplasia of the hip (DDH or congenital hip dysplasia (CDH is the most prevalent developmental childhood hip disorder. It includes a wide spectrum of hip abnormalities ranging from dysplasia to subluxation and complete dislocation of the hip joint. The natural history of neglected DDH in adults is highly variable. The mean age of onset of symptoms is 34.5 years for dysplastic DDH, 32.5 years for low dislocation, 31.2 years for high dislocation with a false acetabulum, and 46.4 years for high dislocation without a false acetabulum. Thorough understanding of the bony and soft tissue deformities induced by dysplasia is crucial for the success of total hip arthroplasty. It is important to evaluate the existing acetabular deformity three-dimensionally, and customize the correction in accordance with the quantity and location of ace tabular deficiencies. Acetabular reconstruction in patients with DDH is hallenging. Interpretation of published data is difficult and should be done with caution because most series include patients with different types of hip disease. In general, the complication rate associated with THA is higher in patients with hip dysplasia than it is in patients with osteoarthritis. Overall, clinical and functional outcomes following THA in patients hip dysplasia (DDH differ from those treated for primary hip osteoarthritis, possibly due to the lower age and level of activity. Although function scores decline with age, the scores for pain and range of motion presented with a statistically significant improvement in the long-term.

  4. Screening for Developmental Dysplasia of the Hip

    NARCIS (Netherlands)

    Boere-Boonekamp, Magdalena M.; Verkerk, Paul H.

    1998-01-01

    The success rates of screening programmes for Developmental Dysplasia of the Hip (DDH) vary widely. Studies on screening programmes for DDH based on a Medline search for the years 1966–1997 are reviewed. The percentage treated in most studies, especially those using ultrasound, are high and suggest

  5. Developmental hip dysplasia in adolescence

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    Vukašinović Zoran

    2009-01-01

    Full Text Available The authors define adolescence and developmental dysplasia of the hip (DDH. Special attention is paid to pathological findings characteristic of DDH in adolescence (unrecognized and untreated DDH; treated DDH, but non-terminated treatment; DDH diagnosed with delay, inadequately treated, with complications. The authors emphasise that DDH treatment has to be successfully terminated well before the adolescence; possibilities are explained on management modes at the time of adolescence, and possible persons guilty for the persistence of later hip problems are indicated. Based on the authors' experience and having in mind all surgical possibilities for the treatment (pelvic osteotomies, femoral osteotomies, trochanteroplasties, leg length equalization procedures the authors propose treatment protocols. The intention is to provide better treatment results and to prevent secondary hip arthrosis. Furthermore, how to improve the struggle against DDH is suggested.

  6. Concomitant achondroplasia and developmental dysplasia of the hip

    OpenAIRE

    Malcolm, Tennison L.; Phan, Duy L.; Schwarzkopf, Ran

    2015-01-01

    Achondroplasia (ACH) is the most common form of hereditary dwarfism and presents with multiple musculoskeletal anomalies but is not normally associated with premature hip arthritis. Developmental dysplasia of the hip (DDH) is a spectrum of disease resulting in shallow acetabular depth and a propensity for chronic femoral subluxation or dislocation; it is among the most common causes of premature arthritis. This case report describes the diagnosis of symptomatic DDH in a patient with ACH and h...

  7. Internet search term affects the quality and accuracy of online information about developmental hip dysplasia.

    Science.gov (United States)

    Fabricant, Peter D; Dy, Christopher J; Patel, Ronak M; Blanco, John S; Doyle, Shevaun M

    2013-06-01

    The recent emphasis on shared decision-making has increased the role of the Internet as a readily accessible medical reference source for patients and families. However, the lack of professional review creates concern over the quality, accuracy, and readability of medical information available to patients on the Internet. Three Internet search engines (Google, Yahoo, and Bing) were evaluated prospectively using 3 difference search terms of varying sophistication ("congenital hip dislocation," "developmental dysplasia of the hip," and "hip dysplasia in children"). Sixty-three unique Web sites were evaluated by each of 3 surgeons (2 fellowship-trained pediatric orthopaedic attendings and 1 orthopaedic chief resident) for quality and accuracy using a set of scoring criteria based on the AAOS/POSNA patient education Web site. The readability (literacy grade level) of each Web site was assessed using the Fleisch-Kincaid score. There were significant differences noted in quality, accuracy, and readability of information depending on the search term used. The search term "developmental dysplasia of the hip" provided higher quality and accuracy compared with the search term "congenital hip dislocation." Of the 63 total Web sites, 1 (1.6%) was below the sixth grade reading level recommended by the NIH for health education materials and 8 (12.7%) Web sites were below the average American reading level (eighth grade). The quality and accuracy of information available on the Internet regarding developmental hip dysplasia significantly varied with the search term used. Patients seeking information about DDH on the Internet may not understand the materials found because nearly all of the Web sites are written at a level above that recommended for publically distributed health information. Physicians should advise their patients to search for information using the term "developmental dysplasia of the hip" or, better yet, should refer patients to Web sites that they have

  8. [Application of computer-aided osteotomy template design in treatment of developmental dysplasia of the hip with steel osteotomy].

    Science.gov (United States)

    Tong, Kuang; Zhang, Yuanzhi; Zhang, Sheng; Yu, Bin

    2013-06-01

    To provide an accurate method for osteotomy in the treatment of developmental dysplasia of the hip with steel osteotomy by three-dimensional reconstruction and Reverse Engineering technique. Between January 2011 and December 2012, 13 children with developmental dysplasia of the hip underwent steel osteotomy. 3D CT scan pelvic images were obtained and transferred via a DICOM network into a computer workstation to construct 3D models of the hip using Materialise Mimics 14.1 software in STL format. These models were imported into Imageware 12.0 software for steel osteotomy simulation until a stable hip was attained in the anatomical position for dislocation or subluxation of the hip in older children. The osteotomy navigational templates were designed according to the anatomical features after a stable hip was reconstructed. These navigational templates were manufactured using a rapid prototyping technique. The reconstruction hips in these children show good matching property and acetabulum cover. The computer-aided design of osteotomy template provides personalized and accurate solutions in the treatment of developmental dysplasia of the hip with steel osteotomy in older children.

  9. Management of developmental dysplasia of the hip in less than 24 months old children

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    Mehmet Bulut

    2013-01-01

    Full Text Available Background: There is no consensus on the treatment of developmental dysplasia of the hip in children less than 24 months of age. The aim of this study was to present the results of open reduction and concomitant primary soft-tissue intervention in patients with developmental dysplasia of the hip in children less than 24 months of age. Materials and Methods: Sixty hips of 50 patients (4 male, 46 female with mean age of 14.62 ± 5.88 (range 5-24 months months with a mean followup of 40.00 ± 6.22 (range 24-58 months months were included. Twenty five right and 35 left hips (10 bilaterally involved were operated. Open reduction was performed using the medial approach in patients aged < 20 months (with Tönnis type II-III and IV hip dysplasias and for those aged 20-24 months with Tönnis type II and III hip dysplasias ( n = 47. However for 13 patients aged 20-24 months with Tönnis type IV hip dysplasias, anterior bikini incision was used. Results: Mean acetabular index was 41.03 ± 3.78° (range 34°-50° in the preoperative period and 22.98 ± 3.01° (range 15°-32° at the final visits. Mean center-edge angle at the final visits was 22.85 ± 3.35° (18°-32°. Based on Severin radiological classification, 29 (48.3% were type I (very good, 25 (41.7% were type II (good and 6 (10% were type III (fair hips. According to the McKay clinical classification, postoperatively the hips were evaluated as excellent ( n = 42; 70%, good ( n = 14; 23.3% and fair ( n = 4; 6.7%. Reduction of all hip dislocations was achieved. Additional pelvic osteotomies were performed in 14 (23.3% hips for continued acetabular dysplasia and recurrent subluxation. (Salter [ n = 12]/Pemberton [ n = 2] osteotomy was performed. Avascular necrosis (AVN developed in 7 (11.7% hips. Conclusion: In DDH only soft-tissue procedures are not enough, because of the high rate of the secondary surgery and AVN for all cases aged less than 24 months. Bone procedures may be necessary in the walking

  10. Concomitant achondroplasia and developmental dysplasia of the hip

    Directory of Open Access Journals (Sweden)

    Tennison L. Malcolm, MD

    2015-12-01

    Full Text Available Achondroplasia (ACH is the most common form of hereditary dwarfism and presents with multiple musculoskeletal anomalies but is not normally associated with premature hip arthritis. Developmental dysplasia of the hip (DDH is a spectrum of disease resulting in shallow acetabular depth and a propensity for chronic femoral subluxation or dislocation; it is among the most common causes of premature arthritis. This case report describes the diagnosis of symptomatic DDH in a patient with ACH and highlights difficulties of primary total hip arthroplasty (THA as a treatment option. Intraoperative radiographic imaging is advised to ensure proper prosthesis placement. Femoral osteotomy may aid visualization, reduction, and avoidance of soft tissue injury. Concomitant ACH and DDH is a challenging problem that can be successfully treated with modified THA.

  11. Gait symmetry and hip strength in women with developmental dysplasia following hip arthroplasty compared to healthy subjects : A cross-sectional study

    NARCIS (Netherlands)

    Leijendekkers, Ruud A.; Marra, Marco A.; Kolk, Sjoerd; Van Bon, Geert; Schreurs, B. Wim; Weerdesteyn, Vivian; Verdonschot, Nico

    2018-01-01

    Introduction: Untreated unilateral developmental dysplasia of the hip (DDH) results in asymmetry of gait and hip strength and may lead to early osteoarthritis, which is commonly treated with a total hip arthroplasty (THA). There is limited knowledge about the obtained symmetry of gait and hip

  12. Spica MRI after closed reduction for developmental dysplasia of the hip

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    Desai, Aditi A. [Vanderbilt University School of Medicine, Nashville, TN (United States); Martus, Jeffrey E.; Schoenecker, Jon [Vanderbilt University School of Medicine, Department of Orthopaedics and Rehabilitation, Monroe Carroll Jr. Children' s Hospital at Vanderbilt, Nashville, TN (United States); Kan, J.H. [Vanderbilt University School of Medicine, Department of Radiology and Radiological Sciences, Monroe Carroll Jr. Children' s Hospital at Vanderbilt, Nashville, TN (United States)

    2011-04-15

    Spica MRI is a fast and effective tool to assess morphology after closed reduction for developmental dysplasia of the hip (DDH) without the need for sedation. The multiplanar capabilities allow depiction of coronal and axial reduction of the hips. Due to MRI's inherent ability to delineate soft tissue structures, both intrinsic and extrinsic obstacles to failed reduction may be identified. Technical and interpretative challenges of spica MRI are discussed. (orig.)

  13. Congenital dislocation of knee with ipsilateral developmental dysplasia of hip

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    Sameer Kakar

    2017-01-01

    Full Text Available We present a rare case of a newborn having congenital knee dislocation (CDK with ipsilateral developmental dysplasia of hip (DDH. This case report shows how abnormal intrauterine pressure leads to dislocation of various joints in utero. We managed this conservatively with Pavlik Harness for DDH and serial corrective casting with manipulation for CDK with a satisfactory result after follow-up of 6 months.

  14. Vojta method in the treatment of developmental hip dysplasia – a case report

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    Kiebzak W

    2016-08-01

    Full Text Available Wojciech Kiebzak,1,2 Arkadiusz Żurawski,2 Michał Dwornik3 1Center for Pediatrics, Regional Hospital in Kielce, Kielce, Poland; 2Department of Physiotherapy, Faculty of Medicine and Health Sciences, Jan Kochanowski University, Kielce, Poland; 3Department of Osteopathic Medicine and Physiotherapy, Medical College of Podkowa Lesna, Podkowa Lesna, Poland Background: Developmental dysplasia of the hip joint is one of the most common congenital defects and often results in functional and structural disorders. Such cases particularly demand optimizing therapeutic effects and maximally reducing the duration of therapy. Purpose: The aim of this case report is to present the therapeutic process in a child with developmental hip dysplasia. Case report: This is a case report of a female child with a birth weight of 2,800 g and an Apgar score of 9 points born to a gravida 3 para 3 mother at 37 weeks. The child was delivered by cesarean section, and the pregnancy was complicated by oligohydramnios. Subluxation of the left hip joint was diagnosed by an orthopedist in the third month of life. The treatment followed was the Vojta method (the first phase of reflex turning and reflex crawling. Results: During the 6 weeks of the Vojta treatment, the left half of the femoral head was centralized, and the process of formation of the hip joint acetabulum was influenced effectively enough to change the acetabulum’s Graff type from the baseline D to IIb after 41 days of treatment. Conclusion: The diagnostic work-up of congenital hip joint dysplasia should involve a physiotherapist who will investigate the child’s neuro­muscular coordination, in addition to a neonatologist and a pediatrician. The therapy for a disorder of hip joint development of neuromotor origin should involve the application of global patterns according to Vojta. Children with congenital dysplasia of the hip joint should commence rehabilitation as early as possible. Keywords: global pattern

  15. Ultrasound screening for developmental dysplasia of the hip and its socioeconomic impact: Experience of tertiary care health level

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    Khaled Aly Matrawy

    2014-03-01

    Conclusion: Screening ultrasound is a useful tool for detection of hip dislocation and dysplasia especially among the population of infants at increased risk of developmental dysplasia of the hip. Limitation of screening ultrasound programs for those at risk only reduces the financial burden with better outcome in choosing candidates for further workup especially surgical intervention.

  16. Ultrasound in the diagnosis and treatment of developmental dysplasia of the hip. Evaluation of a selective screening procedure

    DEFF Research Database (Denmark)

    Strandberg, C.; Konradsen, L.A.; Ellitsgaard, N.

    2008-01-01

    INTRODUCTION: With the intention of reducing the treatment frequency of Developmental Dysplasia of the Hip (DDH), two hospitals in Copenhagen implemented a screening and treatment procedure based on selective referral to ultrasonography of the hip (US). This paper describes and evaluates...... 0.03%. No relationship was seen between morphological parameters at the first US and the outcome of hips classified as minor dysplastic or not fully developed (NFD). A statistically significant relationship was seen between the degree of dysplasia and the time until US normalization of the hips (p......= 0.02). There was no relapse of dysplasia after treatment. The median duration of treatment was six, eight and nine weeks for mild, moderate and severe dysplasia respectively. CONCLUSION: The procedure resulted in a low rate of treatment and a small number of late diagnosed cases. Prediction...

  17. Psychosocial predictors of parental participation in ultrasound screening for developmental dysplasia of the hip

    NARCIS (Netherlands)

    Witting, M.; Boere-Boonekamp, M.M.; Sakkers, R.J.B.; Fleuren, M.A.H.; IJzerman, M.J.

    2013-01-01

    Ultrasound screening for developmental dysplasia of the hip (DDH) is an innovation in preventive child health care in the Netherlands. Parental participation in the screening will be essential for the success of implementation of the screening. The aim of the current study was to investigate whether

  18. Experiences of young women living with developmental dysplasia of the hip: insight into their experiences of surgery and recovery.

    Science.gov (United States)

    Gambling, Tina S; Long, Andrew F

    2013-03-01

    To explore the experiences of young women with developmental dysplasia of the hip explicating the impact of peri-acetabular osteotomy surgery and recovery in the short and longer term. Postings of five, selected women on an online active message board aimed at women with developmental dysplasia of the hip were analysed. Interest lay on their postings after they had had peri-acetabular osteotomy surgery. Data analysis was performed through the approach of interpretive phenomenological analysis. The time length of the postings for the cases ranged from 1 year to 6 years, and the number of postings varied substantially, from 48 to 591. Two major concepts were prominent across participants' accounts. The first concept, 'body image', centred on affects on the women's self-esteem and body image. The second, 'the long road to recovery', highlighted 'the emotional and physical battle of learning to walk' and concerns with 'saving my joints'. Developmental dysplasia of the hip potentially provides a critical case for exploration of the process of how a disability can affect confidence, self-esteem and body image. Recovery from this condition requires enormous effort, resilience and commitment from the women.

  19. Pilot evaluation of anterior dynamic ultrasound screening for developmental dysplasia of the hip in an Australian Regional Hospital

    DEFF Research Database (Denmark)

    Charlton, S.; Muir, L.; Skinner, T. C.

    2012-01-01

    Introduction: Developmental Dysplasia of the Hip (DDH) is the most common notifiable musculoskeletal birth defect in South Australia (SA). Despite routine screening by physical examination of the hips in the neonatal period and at 6 weeks of age, the risk of late diagnosis is increased in rural a...

  20. Hip Dysplasia: Clinical Signs and Physical Examination Findings.

    Science.gov (United States)

    Syrcle, Jason

    2017-07-01

    Hip dysplasia is a common developmental disorder of the dog, consisting of varying degrees of hip laxity, progressive remodeling of the structures of the hip, and subsequent development of osteoarthritis. It is a juvenile-onset condition, with clinical signs often first evident at 4 to 12 months of age. A tentative diagnosis of hip dysplasia can be made based on signalment, history, and physical examination findings. The Ortolani test is a valuable tool for identifying juvenile dogs affected with this condition. Further diagnostics can then be prioritized, contributing to prompt diagnosis and appropriate treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Screening for developmental dysplasia of the hip: current practices in Ireland.

    LENUS (Irish Health Repository)

    O'Grady, M J

    2012-01-31

    OBJECTIVE: To ascertain the current approach to screen for developmental dysplasia of the hip in the Republic of Ireland. METHODS: Two-pronged prospective and retrospective study. (1) Postal questionnaire to consultant paediatricians responsible for the routine neonatal care of infants in the Irish Republic in June 2006. (2) Retrospective database review to identify infants undergoing radiological follow-up and their outcome. RESULTS: All maternity units surveyed responded. Most units (84%) were dependent on radiographs at 4-6 months for imaging hips, only two units primarily used ultrasound (10.5%). We estimate that neonatal hip examination is performed by an experienced examiner in less than 30% of routine newborn examinations. On retrospective analysis, 94% of radiographs performed were normal. CONCLUSIONS: The most effective interventions, selective ultrasound and examination by an experienced clinician are not widely practiced. There is a need for the development of national guidelines based on available resources.

  2. New treatment method for developmental dysplasia of the hips after walking age. Arthroscopic reduction with limboplasty based on the findings of preoperative imaging

    International Nuclear Information System (INIS)

    Kitano, Toshio; Morita, Mitsuaki; Nakagawa, Keisuke; Wada, Mayuko; Kuroda, Takaaki; Imai, Yuuki; Sakai, Toshiyuki; Eguchi, Yoshitaka

    2010-01-01

    What makes treatment choice for developmental dysplasia of the hips diagnosed after walking age difficult is the poor understanding of prereduction conditions that obstruct the reduction in spatial terms. To evaluate these problems, we employed subtraction three-dimensional imaging to search for the factors involved in intraarticular obstruction. On the basis of the findings of preoperative subtraction three-dimensional imaging from computed tomography, we developed a new method, a minimum invasive arthroscopic reduction with limboplasty, for reduction of developmental dysplasia of the hips after walking age. The purposes of this report were to: describe the technique of the arthroscopic procedure, and evaluate our new method using radiographic parameters. Ten patients with ten hips with developmental dysplasia after walking age treated by arthroscopic reduction with limboplasty were included in this study. The mean age of the patients at reduction was 22.6 months (range, 18.6-29.7 months); mean age at follow up was 7.2 years (range, 3.9-10.9 years); and mean follow up was 5.4 years (range, 1.7-9.0 years). These ten hips were evaluated using radiographic measurements. Moderate or severe avascular necrosis of the femoral head was not observed. Two hips that had a spherical-shaped head with minimal residual height loss or coxa magna were classified as Kalamchi and MacEwen grade 1. Additional surgery had been performed for two hips classified as Severin group 4 during the course of follow up. These two hips were classified as Severin group 1 at final examination. One more hip was classified as Severin group 4 at final examination, and additional surgery was recommended. The remaining seven hips (70%) therefore obtained good evaluations by arthroscopic reduction with limboplasty alone. We developed a new reduction method by using an arthroscopic procedure for the reduction of developmental dysplasia of the hips after walking age when this dysplasia failed to be reduced

  3. Radiological and clinical outcomes of medial approach open reduction by using two intervals in developmental dysplasia of the hip

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    Afsar T. Ozkut

    2018-03-01

    Full Text Available Objective: To evaluate the midterm clinical and radiological outcomes of the medial approach using two intervals for developmental hip dysplasia (DDH. Methods: The study involved 62 hips of 47 patients (41 girls, 6 boys treated with medial approach for DDH from 1999 to 2010. The age of the patients at surgery was 18.7 ± 2.25 months. Follow up of the patients was 11.3 ± 3.07 years. The age of the patients at the last follow up was 12.6 ± 1.74 years. According to the Tönnis classification, 13 hips were grade II, 27 hips were grade III and 22 hips were grade IV. Patients were evaluated according to Omeroglu radiological criteria and modified McKay functional criteria. The presence of avascular necrosis (AVN of the hip was questioned using the KalamchiMacEwen classification. Results: Radiologically, forty eight (77% hips were evaluated as “excellent”, 8 (13% hips as “good” and 5 (8% hips as “fair plus” and 1 (%2 hip as “fair minus”. Two (3% patients had type 1 temporary AVN and one (1% patient had type 4 AVN with coxa magna and overgrowth of the greater trochanter. According to McKay functional criteria, 56 (90% hips had “excellent” and 6 (10% had “good” results. Two (3.2% hips of one patient had to be reoperated with Salter osteotomy and femoral shortening + derotation osteotomy. Conclusion: Medial approach using two separate intervals for tenotomy and capsulotomy does not jeopardize the medial circumflex or the femoral vessels and yields satisfactory midterm results for children 18 months old with dysplasia of the hip. Level of evidence: Level IV, therapeutic study. Keywords: Developmental hip dysplasia, Safety, Avascular necrosis of the femoral head, Medial approach, Surgical approach

  4. Long-term outcome following medial open reduction in developmental dysplasia of the hip: a retrospective cohort study

    OpenAIRE

    Gardner, Richard O. E.; Bradley, Catharine S.; Sharma, Om P.; Feng, Lin; Shin, Michelle EyunJung; Kelley, Simon P.; Wedge, J. H.

    2016-01-01

    Introduction Avascular necrosis (AVN) is a serious complication of treatment for developmental dysplasia of the hip. There is ongoing controversy regarding AVN and its influence on hip development following medial open reduction (MOR). Purpose The aim of our study was to (1) determine the long-term prevalence of AVN following MOR, (2) evaluate hip development after MOR, and (3) identify predictors of AVN and radiographic outcome at skeletal maturity after MOR. Methods A retrospective cohort a...

  5. Risk of developmental dysplasia of the hip in breech presentation: the effect of successful external cephalic version

    NARCIS (Netherlands)

    Lambeek, A. F.; de Hundt, M.; Vlemmix, F.; Akerboom, B. M. C.; Bais, J. M. J.; Papatsonis, D. N. M.; Mol, B. W. J.; Kok, M. [=Marjolein

    2013-01-01

    To evaluate the effect of successful external cephalic version on the incidence of developmental dysplasia of the hip (DDH) requiring treatment in singleton breech presentation at term. Observational cohort study. Three large teaching hospitals in the Netherlands. Women with a singleton breech

  6. The natural history of developmental dysplasia of the hip: sonographic findings in infants of 1-3 months of age

    NARCIS (Netherlands)

    Roovers, E.A.; Boere-Boonekamp, Magdalena M.; Mostert, Adriaan K.; Castelein, René M.; Zielhuis, Gerhard A.; Kerkhoff, Antoon

    2005-01-01

    The natural history of sonographic developmental dysplasia of the hip was determined in a population-based study in which 5170 infants were screened by ultrasound using Graf's method. Of the normal hips at the age of 1 month, 99.6% were still normal at the age of 3 months. Of the immature type

  7. Late-presenting developmental dysplasia of the hip in Jordanian males. A retrospective hospital based study

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    Omar Q. Samarah

    2016-02-01

    Full Text Available Objectives: To describe the pattern of developmental dysplasia of the hip (DDH in late presenting Jordanian male patients and identify the risk factors and associated findings. Methods: This is a retrospective study of 1145 male patients who attended the Pediatric Orthopedic Clinic for a DDH check up. This study was carried out in the Orthopedic Section, Special Surgery Department, Faculty of Medicine, The University of Jordan, Amman, Jordan between March 2011 and October 2014. Data was collected from medical records, and x-ray measurements were evaluated. Results: Of the 1145 male patients, 43 (3.75% with 70 involved hips were diagnosed with late- presenting DDH. Being a first-born baby resulted in 41.9% increased risk for DDH. Cesarian delivery was significantly associated with an increased risk of hip dislocation (p=0.004 while normal delivery was significantly associated with acetabular dysplasia (p=0.004. No predictable risk factors were found in 44.2% patients with DDH. Bilateral cases were more common than unilateral cases: (26 [60.5%] versus 17 [39.5%]. Limited abduction was a constant finding in all dislocated hips (p less than 0.001. Associated conditions, such as club foot and congenital muscular torticollis were not observed. Conclusion: Cesarian section is a significant risk for dislocated hips while normal delivery is significantly associated with acetabular dysplasia. Bilateral DDH is more common than the unilateral. Club foot and torticollis were not observed in this series.

  8. [Effect of abducens orthosis combined with walker on developmental dysplasia of the hip].

    Science.gov (United States)

    Hu, Zhiyong; Xu, Yongqiang; Liang, Jieyu; Li, Kanghua; Liao, Qiande

    2009-07-01

    To evaluate the effect of abducens orthosis combined with walker on developmental dysplasia of the hip (DDH). A total of 126 patients (224 hips) with DDH aged 6-36 months in Xiangya Hospital was randomly divided into 2 groups: an orthosis combined with walker group and an improved hip frog cast fixation group. Seventy patients (130 hips) were treated by the orthosis combined with walker and 56 patients (94 hips) were treated by the improved hip frog cast fixation. We compared the effect and complications of the 2 groups. The fineness rates of the orthosis combined with walker group and the improved hip frog cast fixation group were 89.2% and 90.4%, respectively, with no significant difference (P>0.05). The rate of femoral head osteonecrosis in the orthosis combined with walker group was significantly lower than that in the improved hip frog cast fixation group (1.5% vs. 5.3%,Pwalker has a lower proportion of femoral head osteonecrosis, but a higher proportion of re-dislocation.

  9. Cranial acetabular retroversion is common in developmental dysplasia of the hip as assessed by the weight bearing position

    DEFF Research Database (Denmark)

    Troelsen, Anders; Mikkelsen, Lone Rømer; Jacobsen, Steffen

    2010-01-01

    The appearance of acetabular version differs between the supine and weight bearing positions in developmental dysplasia of the hip. Weight bearing radiographic evaluation has been recommended to ensure the best coherence between symptoms, functional appearance, and hip deformities. Previous...... prevalence estimates of acetabular retroversion in dysplastic hips have been established in radiographs recorded with the patient supine and with inclusion only if pelvic tilt met standardized criteria. We assessed the prevalence and the extent of acetabular retroversion in dysplastic hip joints in weight...

  10. RELIABILITY AND VALIDITY OF CLINICAL AND ULTRASOUND EXAMINATIONS OF DEVELOPMENTAL HIP DYSPLASIA

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    Predrag Grubor

    2011-03-01

    Full Text Available Developmental dysplasia of the hip (DDH is the most common congenital deformation of the musculoskeletal system and its successful treatment is closely related to early diagnosis. The study is aimed at examining the incidence of developmental dysplasia of the hip (DDH and at analysing the validity of clinical examination, which is used for the early detection of DDH in the neonatal period, compared to ultrasound examination.The study involved 400 neonates born in the Banja Luka Region. A new questionnaire was open during the first regular ultrasound and clinical examination of the neonates’ hips and anamnestic and clinical data were recorded in it: the asymmetry of the gluteal, inguinal and femoral folds (Bade sign, the result of abduction testseparately for each hip, the Ortolani sign of luxation and the Palmen sign of reposition, then hip sonography. A Toshiba ultrasound machine with a 7.5 MHz linear probe was used. The method employed was Professor Reinhard Graf’s. Out of the total number of the children with a positive sonographic finding for DDH, 63.16% of them have one of the clinical signs of DDH. The ability of a clinical finding to identify those patients who do not have DDH and have a negative sonographic finding is 79.8%. Out of the total number of the examined children with a positive clinical finding, only 15.58% of them also have a positive sonographic finding for DDH. This research has showed that clinical examination of the hips is of low sensitivity, specificity and reliability, and that not all types of DDH can be detected. Clinical examination must remain an integral part of every infant’s examination, but it constitutes a complementary diagnostic procedure to ultrasound examination. The ultrasound examination of DDH has created new possibilities and has filled the void that existed due to the deficiency of clinical tests, and at the same time it has reduced the number of X-ray examinations of the hips. This research has

  11. Hip dysplasia and congenital hip dislocation

    Energy Technology Data Exchange (ETDEWEB)

    Lingg, G.; Nebel, G.; von Torklus, D.

    1981-11-01

    In human genetics and orthopedics quite different answers have been given to the question of hereditary transmission and frequency of hip dysplasia in families of children with congenital hip dislocation. We therefore have made roentgenometric measurements of 110 parents of children with congenital hip dislocation. In 25% we found abnormal flat acetabulae, whereas 12% had pathologic deep hips. This may propose a new concept of morphology of congenital hip dysplasia.

  12. Evaluation of the results of operative treatment of hip dysplasia in ...

    African Journals Online (AJOL)

    El-Sayed Abdel Halim Abdullah

    2012-02-25

    Feb 25, 2012 ... developmental dysplasia of the hip, either secondary to de- layed diagnosis or failed ... the end of follow-up. Trendelenburg test, limping, hip stability ... modified MacKay criteria and modified Harris hip score (Ta- ble 1).9,10 ...

  13. TREATMENT OF HIP DYSPLASIA

    OpenAIRE

    Iulian ICLEANU; Mariana CORDUN

    2015-01-01

    In this thesis, our purpose is to show that using physiotherapy on patients with hip dysplasia from the very beginning, in the first months of life, helps treating them faster. Common literature proposes to use physiotherapy on patients with hip dysplasia either after their recovery or in the terminal phase of recovery, claiming that any earlier intervention will prolong the hip recovery. The effects of hip dysplasia reflect over the whole musculoskeletal system, while it hinders the knees (g...

  14. Hip instability: a review of hip dysplasia and other contributing factors

    Science.gov (United States)

    Kraeutler, Matthew J.; Garabekyan, Tigran; Pascual-Garrido, Cecilia; Mei-Dan, Omer

    2016-01-01

    Summary Background Hip instability has classically been associated with developmental dysplasia of the hip (DDH) in newborns and children. However, numerous factors may contribute to hip instability in children, adolescents, and adults. Purpose This review aims to concisely present the literature on hip instability in patients of all ages in order to guide health care professionals in the appropriate diagnosis and treatment of the various disorders which may contribute to an unstable hip. Methods We reviewed the literature on the diagnosis and surgical management of hip dysplasia and other causes of hip instability. Conclusions Multiple intra- and extra-articular variables may contribute to hip instability, including acetabular bony coverage, femoral torsion, femoroacetabular impingement, and soft tissue laxity. Physical examination and advanced imaging studies are essential to accurately diagnose the pathology contributing to a patient’s unstable hip. Conservative management, including activity modification and physical therapy, may be used as a first-line treatment in patients with intra-articular hip pathology. Patients who continue to experience symptoms of pain or instability should proceed with arthroscopic or open surgical treatment aimed at correcting the underlying pathology. Level of evidence V. PMID:28066739

  15. The gothic arch: a reliable measurement for developmental dysplasia of the hip.

    Science.gov (United States)

    Herickhoff, Paul K; O'Brien, Megan K; Dolan, Lori A; Morcuende, Jose A; Peterson, Jonathan B; Weinstein, Stuart L

    2013-01-01

    The "Gothic Arch" is a radio-graphic finding on AP pelvis x-rays postulated to be predictive of hip osteoarthritis. The purpose of this study was to determine the reliability of measurement of the Gothic Arch in patients with no known hip pathology and patients with unilateral developmental dysplasia of the hip (DDH). After obtaining IRB approval, nine skeletally mature patients (18 hips) with no known hip pathology were selected to serve as the control group. The AP pelvis x-rays at skeletal maturity of eight patients (16 hips) with unilateral DDH treated with closed reduction and casting comprised the comparison group. A digitizing program was designed to measure the Gothic Arch based on landmarks identified by the user. Two pediatric orthopaedic surgeons and two orthopaedic residents completed the program on two separate occasions. Intra-and interobserver reliability were determined using intraclass cor-relation coefficients (ICC) for continuous variables. Both the unilateral DDH group and the control group demonstrated excellent inter- and intraobserver reliability (ICC >0.70) for base, height, area, and orientation of the Gothic Arch, but poor reliability (ICC Gothic Arch can be reliably measured on AP pelvis x-rays of patients with normal and dysplastic hips. III, Diagnostic study. See the Guidelines for Authors for a complete description of levels of evidence.

  16. Late presentation of developmental dysplasia of the hip.

    LENUS (Irish Health Repository)

    Gul, R

    2012-02-03

    BACKGROUND: A neonatal screening programme for developmental dysplasia of the hip (DDH) is ongoing in Cork. Despite early screening, infants continue to present at later ages with DDH. The impact of late diagnosis is significant. Established DDH causes significant morbidity and may have major medicolegal implications. AIM: To identify the reasons for the late presentation of DDH in the presence of a screening programme. METHODS: In a retrospective study all cases of late DDH presenting from 1988 to 2000 were identified using inpatient database. RESULTS: Forty-nine cases of DDH were diagnosed. The mean age of diagnosis was 14.8 months (range 6-47). Multiple risk factors were identified in four patients only. More than one risk factor was identified in 10 patients. CONCLUSION: Despite screening, children continue to present with late DDH. In this study, only 14 patients had multiple risk factors and only four patients had more than two risk factors, highlighting the low incidence of suspicion in this patient group.

  17. MRI after operative reduction with femoral osteotomy in developmental dysplasia of the hip

    Energy Technology Data Exchange (ETDEWEB)

    Ranawat, Vijai [Great Ormond Street Hospital for Children NHS Trust, Orthopaedic Department, London (United Kingdom); London (United Kingdom); Rosendahl, Karen [Great Ormond Street Hospital for Children NHS Trust, Radiology Department, London (United Kingdom); Jones, David [Great Ormond Street Hospital for Children NHS Trust, Orthopaedic Department, London (United Kingdom)

    2009-02-15

    The use of MRI scanning has been described after open reduction of the hip in developmental dysplasia of the hip (DDH) to check hip position, but has not previously been reported after open reduction with femoral osteotomy and the use of metalwork. We report a prospective study to determine whether MRI scanning can be used to confirm satisfactory reduction of the hip following surgery for DDH, even in the presence of metalwork in the proximal femur. MRI scans were performed in 12 consecutive children, and all gave diagnostic information indicating satisfactory reduction. Sedation was not required and the mean scanning time was 3 min 45 s. Satisfactory images, the lack of need for sedation, comparable time and cost to CT scanning and most importantly the lack of exposure of the child to ionizing radiation make MRI a most appealing method for imaging. We therefore recommend it as the investigation of choice in this patient group. (orig.)

  18. MRI after operative reduction with femoral osteotomy in developmental dysplasia of the hip

    International Nuclear Information System (INIS)

    Ranawat, Vijai; Rosendahl, Karen; Jones, David

    2009-01-01

    The use of MRI scanning has been described after open reduction of the hip in developmental dysplasia of the hip (DDH) to check hip position, but has not previously been reported after open reduction with femoral osteotomy and the use of metalwork. We report a prospective study to determine whether MRI scanning can be used to confirm satisfactory reduction of the hip following surgery for DDH, even in the presence of metalwork in the proximal femur. MRI scans were performed in 12 consecutive children, and all gave diagnostic information indicating satisfactory reduction. Sedation was not required and the mean scanning time was 3 min 45 s. Satisfactory images, the lack of need for sedation, comparable time and cost to CT scanning and most importantly the lack of exposure of the child to ionizing radiation make MRI a most appealing method for imaging. We therefore recommend it as the investigation of choice in this patient group. (orig.)

  19. Pathophysiology, diagnosis, and treatment of canine hip dysplasia

    International Nuclear Information System (INIS)

    Cook, J.L.; Tomlinson, J.L.; Constantinescu, G.M.

    1996-01-01

    Dogs with hip dysplasia are commonly presented to veterinarians for evaluation. Although many causes of the condition have been proposed, a definitive cause has not been established. The multifactorial nature of canine hip dysplasia can confuse client education and management ofthe disease. The basic concept involved is the biomechanical imbalance between the forces on the coxofemoral joint and the associated muscle mass; the result is joint laxity in young, growing dogs. This laxity leads to incongruity; the eventual result is degenerative joint disease. Canine hip dysplasia can affect any breed but is most often reported in large and giant breeds. Understanding the pathophysiology and biomechanics involved with this developmental disease is important in providing clients with diagnostic, therapeutic, and prognostic information. The selection of treatment is influenced by the following factors:the age, health, and intended use of the patient; clinical signs; diagnostic findings; the availability of treatment; and the financial constraints of the owner. This article discusses the current concepts concerning the pathophysiology and biomechanics of canine hip dysplasia and outlines diagnostic and therapeutic options. The objective of the article is to provide practitioners with a reference for decision making and client education

  20. Long-term outcome following medial open reduction in developmental dysplasia of the hip: a retrospective cohort study.

    Science.gov (United States)

    Gardner, Richard O E; Bradley, Catharine S; Sharma, Om P; Feng, Lin; Shin, Michelle EyunJung; Kelley, Simon P; Wedge, J H

    2016-06-01

    Avascular necrosis (AVN) is a serious complication of treatment for developmental dysplasia of the hip. There is ongoing controversy regarding AVN and its influence on hip development following medial open reduction (MOR). The aim of our study was to (1) determine the long-term prevalence of AVN following MOR, (2) evaluate hip development after MOR, and (3) identify predictors of AVN and radiographic outcome at skeletal maturity after MOR. A retrospective cohort analysis of 60 patients (70 hips) who underwent MOR with a mean follow-up of 10.83 years (5.23-16.74) was conducted. AVN was recorded according to Bucholz and Ogden classification and radiographic outcome based on Severin grading. AVN and hip morphology related to length of follow-up were evaluated. Chi-squared and t-tests were used to identify relationships between AVN and other variables. Logistic regression was used to assess predictors of AVN and Severin outcome. The rate of clinically significant AVN (types 2-4) following MOR was 32.9 % with type 2 accounting for 82.6 % of these cases. While early acetabular development was satisfactory, long-term outcome was unsatisfactory in 26 % of cases with AVN (vs 8.7 % of cases without AVN). A higher rate of AVN was identified when hips were immobilized in ≥60° of abduction postoperatively. A higher rate of poor Severin outcome was identified in hips with AVN. Our findings suggest that there is a high rate of AVN and unsatisfactory long-term outcome following MOR. AVN remains a significant concern following MOR surgery for developmental dysplasia of the hip that may not be apparent until long-term evaluation.

  1. Utility of immediate postoperative hip MRI in developmental hip dysplasia: closed vs. open reduction.

    Science.gov (United States)

    Jadhav, Siddharth P; More, Snehal R; Shenava, Vinitha; Zhang, Wei; Kan, J Herman

    2018-04-25

    Magnetic resonance imaging (MRI) of the hips is being increasingly used to confirm hip reduction after surgery and spica cast placement for developmental dysplasia of the hip (DDH). To review a single institutional experience with post-spica MRI in children undergoing closed or open hip reduction and describe the utility of MRI in directing the need for re-intervention. Seventy-four patients (52 female, 22 male) who underwent post-spica hip MRI over a 6-year period were retrospectively reviewed. One hundred and seven hips were included. Data reviewed included age at intervention, gender, type of intervention performed, MRI findings, the need for re-intervention and the interval between interventions. Gender was compared between the closed and open reduction groups via the Fisher exact test. Age at the first procedure was compared via the Wilcoxon rank test. Rates of re-intervention after closed and open reduction were calculated and the reasons for re-intervention were reviewed. The mean age at the time of the first intervention was 16.4 months (range: 4 to 63 months). Mean age for the closed reduction group was 10.5 months (range: 4-24 months) and for the open reduction group was 23.7 months (range: 5-63 months), which was significant (P-value open reduction, MRI was useful in deciding re-intervention in only 1 (2%). This patient had prior multiple failed closed and open reductions at an outside institute. Post intervention hip spica MRI is useful in determining the need for re-intervention after closed hip reduction, but its role after open reduction is questionable.

  2. Adverse Outcomes in Infantile Bilateral Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Morbi, Abigail H M; Carsi, Belen; Gorianinov, Vitalli; Clarke, Nicholas M P

    2015-01-01

    It is believed that bilateral developmental dysplasia of the hip (DDH) has poorer outcomes with higher rates of avascular necrosis (AVN) and reintervention, compared with unilateral DDH. However, there is limited evidence in the literature, with few studies looking specifically at bilateral cases. A retrospective review of 36 patients (72 hips) with >4 years of follow-up. Patient population included surgically treated DDH including late presentations and failures of conservative treatment. The dislocated hips underwent either simultaneous closed or 1 open and 1 closed, or sequential open reduction. AVN and secondary procedures were used as endpoints for analysis as well as clinical and radiologic outcomes. At the last follow-up, 33% of hips had radiologic signs of AVN. Those hips that had no ossific nucleus (ON) at the time of surgery had an odds ratio of developing AVN of 3.05 and a statistically significant association between the 2 variables, whereas open/closed or simultaneous/sequential reduction did not increase the risk for AVN. In addition, 45.8% of those hips required further surgery. The estimated odds ratio of needing additional surgery after simultaneous reduction was 4.04. Clinically, 79.2% of the hips were graded as McKay I, whereas radiologically only 38.8% were Severin I. The AVN rate in bilateral DDH treated surgically is greater than the rate noted in unilateral cases from the same institution undergoing identical protocols. There was no difference in AVN rates between simultaneous and sequential or between the first and second hip to be sequentially reduced. Presence of ON decreases the risk for AVN, suggesting that in bilateral cases, awaiting the appearance of the ON is an important tool to reduce the incidence of AVN. IV.

  3. Changes of gluteus medius muscle in the adult patients with unilateral developmental dysplasia of the hip

    Directory of Open Access Journals (Sweden)

    Liu RuiYu

    2012-06-01

    Full Text Available Abstract Background The gluteus medius muscle is essential for gait and hip stability. Changes that occur in the gluteus medius muscles in patients with developmental dysplasia of the hip (DDH are not well understood. A better understanding of DDH related changes will have positive repercussions toward hip soft tissue reconstruction. Methods 19 adult patients with unilateral DDH scheduled for total hip arthroplasty were assessed for: cross-sectional area (CSA, radiological density (RD and the length of gluteus medius using computed tomograhpy(CT (scanned before THA. Hip abductor moment arm and gluteus medius activation angle were also measured via hip anteroposterior radiographs. Results Both CSA and RD of gluteus medius muscle were significantly reduced (p  Conclusions The gluteus medius showed substantial loss of CSA, RD as well as decreased length in patients with DDH in the affected hip. These changes should be considered in both hip reconstruction and postoperative rehabilitation training in patients with DDH.

  4. TREATMENT OF HIP DYSPLASIA

    Directory of Open Access Journals (Sweden)

    Iulian ICLEANU

    2015-11-01

    Full Text Available In this thesis, our purpose is to show that using physiotherapy on patients with hip dysplasia from the very beginning, in the first months of life, helps treating them faster. Common literature proposes to use physiotherapy on patients with hip dysplasia either after their recovery or in the terminal phase of recovery, claiming that any earlier intervention will prolong the hip recovery. The effects of hip dysplasia reflect over the whole musculoskeletal system, while it hinders the knees (genu valgum, the ankles (ankle valgus, calcaneal valgus and the spine (scoliosis especially at the lumbar level. The most spectacular are at the hip level, that is why we made an analytical evaluation only for this joint. To show the importance of physiotherapy for children with hip dysplasia we started from the hypothesis: untimely treatment for children with hip dysplasia has improved results in functional recovery and in obtaining a better stability, without the necessity of orthopedics or surgical interventions. The research methods used in this study are: the observation method, the bibliographic study method, the experimental method, the graphics method and the statistical mathematical method to process the data and to represent the results graphically. In the end, the results obtained are significantly different from the initial evaluations and we came to the conclusion that starting an untimely analytical kinetic treatment and globally personalizing it to every patient improves stability and biomechanical parameters for the hip.

  5. Two-Stage Progressive Femoral Lowering Followed by Cementless Total Hip Arthroplasty for Treating Crowe IV-Hartofilakidis Type 3 Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Binazzi, Roberto

    2015-05-01

    High developmental dysplasia of the hip is commonly treated with total hip arthroplasty and shortening osteotomy. We present a two stage technique, consisting of progressive femoral lowering followed by total hip arthroplasty. The clinico-radiographic results of eleven patients (twelve hips) who were operated on with the two-stage technique were evaluated at a mean follow-up of 11 ± 5 years. At the final follow-up, ten patients (eleven hips) had a mean Harris hip score of 85 ± 5 points with no implant loosening. One patient (one hip) was revised at 5 years due to infection. No neurovascular complications were observed in any patients. With this technique, we could place the cup in the anatomical position and obtain complete limb symmetry with excellent clinical results at long-term. Copyright © 2014 Elsevier Inc. All rights reserved.

  6. Evaluation of a training program for general ultrasound screening for developmental dysplasia of the hip in preventive child health care

    NARCIS (Netherlands)

    Boere-Boonekamp, Magdalena M.; Ramwadhdoebe, S.; Sakkers, R.J.B.; Uiterwaal, Cuno S.P.M.; Beek, Frederik J.A.

    2010-01-01

    Background: A research study in the Netherlands showed that general ultrasound (US) screening was cost-effective in the detection of developmental dysplasia of the hip (DDH). This study was followed by a pilot implementation study. Part of this pilot implementation study is to investigate whether

  7. Evaluation of surgical outcome in advanced age patients with developmental hip dysplasia.

    Science.gov (United States)

    Çiçekli, Özgür; Doğan, Metin

    2018-04-01

    This study aimed at determining the efficacy of the surgical treatment conducted on children with delayed presentation developmental dysplasia of the hip (DDH). The objective was to provide a good comparison of functional and radiographic outcomes and to determine various surgical treatment options. In this study, we evaluated the results of 38 hips of 27 patients aged 4 years onset which had been operated due to DDH. Radiographic outcomes were evaluated by using acetabular index, CE angle, Tonnis and the Severin classification system. Clinical evaluation was made by using IOWA hip scores. Degenerative changes were evaluated according to Boyer and avascular necrosis according to Kalamchi-MacEwen criteria. The mean age of the patients at the operation time was 10 years 3 months (range: 4 years 3 months-30 years). While the mean acetabular index was 37.97 preoperatively, in the last follow-up it was 19.92. Thirty-six hips (94%) had an excellent and good outcomes, and two hips (%6) had a fair outcome with respect to the Severin criteria. The mean hip score with respect to IOWA was 93.736 (range: 75-98) and the rate of excellent and good outcomes was 98%. For the treatment of patients with DDH of late onset, a one-staged operative procedure is recommended. This method is applied successfully and enables us to achieve good clinical and radiographic results. Copyright © 2018 IJS Publishing Group Ltd. Published by Elsevier Ltd. All rights reserved.

  8. Morphological characteristics of the bony birth canal in patients with developmental dysplasia of the hip (DDH). Investigation by three-dimensional CT

    Energy Technology Data Exchange (ETDEWEB)

    Kojima, Seiichi; Kobayashi, Seneki; Saito, Naoto; Nawata, Masashi; Horiuchi, Hiroshi; Takaoka, Kunio [Shinshu Univ., Matsumoto, Nagano (Japan). School of Medicine

    2001-07-01

    We investigated the three-dimensional morphological characteristics of the pelvis in adult female patients with developmental dysplasia of the hip (DDH), using computerized tomography (CT) images. Forty-two subjects with normal hips and 40 DDH patients were recruited for the study. In the DDH group, the average transverse diameter of the pelvic inlet was significantly less and the average transverse diameter of the pelvic outlet was significantly greater than the measurements in the normal group. Further, the bony birth canal in DDH patients exhibited a higher incidence of anthropoid-type geometry, as defined by a longer sagittal diameter relative to the transverse diameter of the pelvic inlet. These findings indicate a characteristic pelvic geometry and suggest different development of the pelvis in the transverse direction in DDH patients. In addition, the obstetric conjugate length/transverse diameter of the pelvic inlet ratio was correlated to the degree of severity of acetabular dysplasia. This finding suggests that DDH is a manifestation of a developmental characteristic of the pelvis. (author)

  9. Radiographic Underestimation of In Vivo Cup Coverage Provided by Total Hip Arthroplasty for Dysplasia.

    Science.gov (United States)

    Nie, Yong; Wang, HaoYang; Huang, ZeYu; Shen, Bin; Kraus, Virginia Byers; Zhou, Zongke

    2018-01-01

    The accuracy of using 2-dimensional anteroposterior pelvic radiography to assess acetabular cup coverage among patients with developmental dysplasia of the hip after total hip arthroplasty (THA) remains unclear in retrospective clinical studies. A group of 20 patients with developmental dysplasia of the hip (20 hips) underwent cementless THA. During surgery but after acetabular reconstruction, bone wax was pressed onto the uncovered surface of the acetabular cup. A surface model of the bone wax was generated with 3-dimensional scanning. The percentage of the acetabular cup that was covered by intact host acetabular bone in vivo was calculated with modeling software. Acetabular cup coverage also was determined from a postoperative supine anteroposterior pelvic radiograph. The height of the hip center (distance from the center of the femoral head perpendicular to the inter-teardrop line) also was determined from radiographs. Radiographic cup coverage was a mean of 6.93% (SD, 2.47%) lower than in vivo cup coverage for these 20 patients with developmental dysplasia of the hip (Pcup coverage (Pearson r=0.761, Pcup (P=.001) but not the position of the hip center (high vs normal) was significantly associated with the difference between radiographic and in vivo cup coverage. Two-dimensional radiographically determined cup coverage conservatively reflects in vivo cup coverage and remains an important index (taking 7% underestimation errors and the effect of greater underestimation of larger cup size into account) for assessing the stability of the cup and monitoring for adequate ingrowth of bone. [Orthopedics. 2018; 41(1):e46-e51.]. Copyright 2017, SLACK Incorporated.

  10. Influence of hip dysplasia on the development of osteoarthritis of the hip

    NARCIS (Netherlands)

    A.M. Lievense (Annet); S.M. Bierma-Zeinstra (Sita); A.P. Verhagen (Arianne); J.A.N. Verhaar (Jan); B.W. Koes (Bart)

    2004-01-01

    textabstractBackground: It has been suggested that in some patients with primary hip osteoarthritis (OA), the disease occurs as a consequence of acetabular dysplasia or hip dysplasia (HD). Objective: To carry out a systematic review to investigate the association between acetabular dysplasia and hip

  11. Treatment of Developmental Dysplasia of the Hip: Short and Mid-term Outcome

    Directory of Open Access Journals (Sweden)

    Asim AM

    2011-03-01

    Full Text Available OBJECTIVE: To review the presentation and outcome of developmental dysplasia of the hip (DDH cases in the last 6 years. METHODOLOGY: We retrospectively reviewed the presentation as well as short and mid-term outcome of 17 children (18 hips with DDH seen and treated from 2004 to 2010. Treatment was either splinting with Pavlik harness, closed or open reduction depending on age at presentation and the individual needs of each patient. RESULTS: The average age at presentation was 31.1 months, and the male to female ratio was 1:4.6. Six patients (35% presented early (less than 6 months old while 7 patients (41% presented between 6 -24 months. In these 2 groups splinting or closed reduction under anaesthesia was initially utilized for treatment depending on age, and open reduction was used after failure of closed reduction and for those presented late. Average age at the time of closed reduction was 11 months, and 6 years for those underwent open reduction. The average period of follow up for all patients was 29.4 months. The final outcome was successful in 12 hips (66% in 11 patients. Ten of these presented at less than 2 years old, in the other 6 hips in which there was failure, 50% presented after 3 years of age. CONCLUSION: Late presentation of DDH is still common in Malaysia, which necessitates more complex management and more frequent occurrences of unfavourable results.

  12. Posterosuperior Placement of a Standard-Sized Cup at the True Acetabulum in Acetabular Reconstruction of Developmental Dysplasia of the Hip With High Dislocation.

    Science.gov (United States)

    Xu, Jiawei; Xu, Chen; Mao, Yuanqing; Zhang, Jincheng; Li, Huiwu; Zhu, Zhenan

    2016-06-01

    We sought to evaluate posterosuperior placement of the acetabular component at the true acetabulum during acetabular reconstruction in patients with Crowe type-IV developmental dysplasia of the hip. Using pelvic computed tomography and image processing, we developed a two-dimensional mapping technique to demonstrate the distribution of preoperative three-dimensional cup coverage at the true acetabulum, determined the postoperative location of the acetabular cup, and calculated postoperative three-dimensional coverage for 16 Crowe type-IV dysplastic hips in 14 patients with a mean age of 52 years (33-78 years) who underwent total hip arthroplasty. Mean follow-up was 6.3 years (5.5-7.3 years). On preoperative mapping, the maximum three-dimensional coverage using a 44-mm cup was 87.31% (77.36%-98.14%). Mapping enabled the successful replacement of 16 hips using a mean cup size of 44.13 mm (42-46 mm) with posterosuperior placement of the cup. Early weight-bearing and no prosthesis revision or loosening during follow-up were achieved in all patients. The postoperative two-dimensional coverage on anteroposterior radiographs and three-dimensional coverage were 96.15% (89.49%-100%) and 83.42% (71.81%-98.50%), respectively. This technique may improve long-term implant survival in patients with Crowe-IV developmental dysplasia of the hip undergoing total hip arthroplasty by allowing the use of durable bearings, increasing host bone coverage, ensuring initial stability, and restoring the normal hip center. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. Does the optimal position of the acetabular fragment should be within the radiological normal range for all developmental dysplasia of the hip? A patient-specific finite element analysis.

    Science.gov (United States)

    Wang, Xuyi; Peng, Jianping; Li, De; Zhang, Linlin; Wang, Hui; Jiang, Leisheng; Chen, Xiaodong

    2016-10-04

    The success of Bernese periacetabular osteotomy depends significantly on how extent the acetabular fragment can be corrected to its optimal position. This study was undertaken to investigate whether correcting the acetabular fragment into the so-called radiological "normal" range is the best choice for all developmental dysplasia of the hip with different severities of dysplasia from the biomechanical view? If not, is there any correlation between the biomechanically optimal position of the acetabular fragment and the severity of dysplasia? Four finite element models with different severities of dysplasia were developed. The virtual periacetabular osteotomy was performed with the acetabular fragment rotated anterolaterally to incremental center-edge angles; then, the contact area and pressure and von Mises stress in the cartilage were calculated at different correction angles. The optimal position of the acetabular fragment for patients 1, 2, and 3 was when the acetabular fragment rotated 17° laterally (with the lateral center-edge angle of 36° and anterior center-edge angle of 58°; both were slightly larger than the "normal" range), 25° laterally following further 5° anterior rotation (with the lateral center-edge angle of 31° and anterior center-edge angle of 51°; both were within the "normal" range), and 30° laterally following further 10° anterior rotation (with the lateral center-edge angle of 25° and anterior center-edge angle of 40°; both were less than the "normal" range), respectively. The optimal corrective position of the acetabular fragment is severity dependent rather than within the radiological "normal" range for developmental dysplasia of the hip. We prudently proposed that the optimal correction center-edge angle of mild, moderate, and severe developmental dysplasia of the hip is slightly larger than the "normal" range, within the "normal" range, and less than the lower limit of the "normal" range, respectively.

  14. Joint space width in dysplasia of the hip

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig; Søballe, K

    2005-01-01

    In a longitudinal case-control study, we followed 81 subjects with dysplasia of the hip and 136 control subjects without dysplasia for ten years assessing radiological evidence of degeneration of the hip at admission and follow-up. There were no cases of subluxation in the group with dysplasia....... Neither subjects with dysplasia nor controls had radiological signs of ongoing degenerative disease at admission. The primary radiological discriminator of degeneration of the hip was a change in the minimum joint space width over time. There were no significant differences between these with dysplasia...... and controls in regard to age, body mass index or occupational exposure to daily repeated lifting at admission.We found no significant differences in the reduction of the joint space width at follow-up between subjects with dysplasia and the control subjects nor in self-reported pain in the hip...

  15. Secondary influences and ectopic causes of canine hip dysplasia. Critical constructive thoughts to hip dysplasia diagnostics and to current breeding strategies

    International Nuclear Information System (INIS)

    Schawalder, P.; Spreng, D.; Dietschi, E.; Dolf, G.; Gaillard, C.

    1996-01-01

    This article questions the stagnation of breeding results by hip dysplasia screening and its current etiology. The ''Coxa Valga Antetorta'', anteversion of the hip joint and ''soft tissue displasia'' are discussed besides a multitude of primary diseases leading to growth deformities and other diseases in the hip. Ectopic primary causes like malformation of the spine leading to a secondary ''dysplatic'' osteoarthritis of the hip (functional secondary dysplasia) are also discussed in this article. Recent and very promising breeding strategies (estimation of the breeding value) in the battle against hip dysplasia are presented. Biochemical, immunological and structural aspects are presented as a not totally utopic perspecitive. Molecular genetic markers for hip dysplasia will offer in the near future a possibility to detect carriers ct the disease by a blood sample ion before clinical manifestation

  16. US of the hips in skeletal dysplasias and chromosomal aberrations

    International Nuclear Information System (INIS)

    Langer, R.; Langer, M.F.J.; Zwicker, C.

    1987-01-01

    Since January 1984 all newborns and infants with skeletal dysplasias and chromosomal aberrations were investigated by hip US, in addition to plain x-ray surveys. The authors observed one chondroectodermal dysplasia, one congenital spondyloepiphysial dysplasia, one cleidocranial dysplasia, one fibrochondrogenesis, two diastrophic dysplasias, and eight trisomies. The abnormalities of the hip joints could be demonstrated, and were compared with the findings on plain films. Especially skeletal dysplasias with abundant presence of cartilage were well visible. The newborn with trisomies showed normal hip joints. In the authors' opinion, all newborns with skeletal dysplasias should be investigated by hip sonography, in addition to skeletal radiography

  17. A patient-specific model of the biomechanics of hip reduction for neonatal Developmental Dysplasia of the Hip: Investigation of strategies for low to severe grades of Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Huayamave, Victor; Rose, Christopher; Serra, Sheila; Jones, Brendan; Divo, Eduardo; Moslehy, Faissal; Kassab, Alain J; Price, Charles T

    2015-07-16

    A physics-based computational model of neonatal Developmental Dysplasia of the Hip (DDH) following treatment with the Pavlik Harness (PV) was developed to obtain muscle force contribution in order to elucidate biomechanical factors influencing the reduction of dislocated hips. Clinical observation suggests that reduction occurs in deep sleep involving passive muscle action. Consequently, a set of five (5) adductor muscles were identified as mediators of reduction using the PV. A Fung/Hill-type model was used to characterize muscle response. Four grades (1-4) of dislocation were considered, with one (1) being a low subluxation and four (4) a severe dislocation. A three-dimensional model of the pelvis-femur lower limb of a representative 10 week-old female was generated based on CT-scans with the aid of anthropomorphic scaling of anatomical landmarks. The model was calibrated to achieve equilibrium at 90° flexion and 80° abduction. The hip was computationally dislocated according to the grade under investigation, the femur was restrained to move in an envelope consistent with PV restraints, and the dynamic response under passive muscle action and the effect of gravity was resolved. Model results with an anteversion angle of 50° show successful reduction Grades 1-3, while Grade 4 failed to reduce with the PV. These results are consistent with a previous study based on a simplified anatomically-consistent synthetic model and clinical reports of very low success of the PV for Grade 4. However our model indicated that it is possible to achieve reduction of Grade 4 dislocation by hyperflexion and the resultant external rotation. Copyright © 2015 Elsevier Ltd. All rights reserved.

  18. Developmental dysplasia of hip screening using ortolani and barlow testing on breech delivered neonates.

    Science.gov (United States)

    Sulaiman, Ar; Yusof, Zakaria; Munajat, I; Lee, Naa; Zaki, Nik

    2011-11-01

    We conducted this study to compare the specificity and sensitivity of the Ortolani and Barlow tests performed by dedicated examiners, and to ascertain the incidence of developmental dysplasia of the hip (DDH) in breech babies. A dedicated examiner underwent specific training and testing by a paediatric orthopaedic surgeon. Routine examiners were medical officers who had basic training in medical school and were briefly trained by their superiors. The dedicated examiner examined 170 babies. Thirty babies including 5 babies with positive tests (according to the dedicated examiner) were examined by a blinded routine examiner. RESULTS of Ortolani and Barlow tests on 30 babies were compared with ultrasound examination by blinded radiologist. Five babies had positive Ortolani and Barlow tests. The routine examiner did not detect positive Ortolani and Barlow tests. The incidence of positive Ortolani and Barlow tests among breech babies was 2.8%. Result of Ortolani and Barlow tests by dedicated hip screener were better than results performed by routine examiner. Ortolani and Barlow, Dedicated Examiner, Routine Examiner, Breech, Ultrasound.

  19. Comparison between Graf method and pubo-femoral distance in neutral and flexion positions to diagnose developmental dysplasia of the hip

    Energy Technology Data Exchange (ETDEWEB)

    Teixeira, Sara R., E-mail: steixeira@hcrp.usp.br [Division of Radiology, Department of Internal Medicine, Ribeirao Preto Medical School, University of Sao Paulo, Av. Bandeirantes, 3900 Ribeirao Preto, Sao Paulo 14049-090 (Brazil); Dalto, Vitor F., E-mail: fdalto@gmail.com [Division of Radiology, Department of Internal Medicine, Ribeirao Preto Medical School, University of Sao Paulo, Av. Bandeirantes, 3900 Ribeirao Preto, Sao Paulo 14049-090 (Brazil); Maranho, Daniel A., E-mail: dacmaranho@gmail.com [Division of Pediatric Orthopaedics, Department of Biomechanics, Medicine, and Rehabilitation of the Locomotor System, Ribeirao Preto Medical School, University of Sao Paulo, Av. Bandeirantes, 3900 Ribeirao Preto, Sao Paulo 14049-090 (Brazil); Zoghbi-Neto, Orlando S., E-mail: zoghbi47@gmail.com [Division of Radiology, Department of Internal Medicine, Ribeirao Preto Medical School, University of Sao Paulo, Av. Bandeirantes, 3900 Ribeirao Preto, Sao Paulo 14049-090 (Brazil); Volpon, José B., E-mail: jbvolpon@fmrp.usp.br [Division of Pediatric Orthopaedics, Department of Biomechanics, Medicine, and Rehabilitation of the Locomotor System, Ribeirao Preto Medical School, University of Sao Paulo, Av. Bandeirantes, 3900 Ribeirao Preto, Sao Paulo 14049-090 (Brazil); Nogueira-Barbosa, Marcello H., E-mail: marcello@fmrp.usp.br [Division of Radiology, Department of Internal Medicine, Ribeirao Preto Medical School, University of Sao Paulo, Av. Bandeirantes, 3900 Ribeirao Preto, Sao Paulo 14049-090 (Brazil)

    2015-02-15

    Highlights: • The article adds information about the pubo-femoral distance (PFD) used as a simple tool to detect dysplastic hips in neonates. This articles shows that the PFD is comparable with the “gold standard” Graf method for the diagnosis of developmental dysplasia of the hip and it can be used as a screening tool for its diagnosis, regardless the radiologists’ experience, with high accuracy. - Abstract: Purposes: To evaluate whether the pubo-femoral distance (PFD) can be used as an accurate screening test to diagnose developmental dysplasia of the hip (DDH) in an at-risk population compared with the Graf method. Second, to determine whether PFD assessment is feasible and reproducible regardless of the observer's experience. Materials and methods: IRB approved this retrospective single-institution study. Written informed consent was waived. Between January 2010 and March 2012, 116 neonates at risk for DDH were included. Infants’ hips were distributed into two groups according to recommendation for treatment: non-dysplastic (ND; Graf I/IIA; 211 hips; 69 females/37 males) and dysplastic hip (DH; Graf IIB/IIC/III/D/IV; 21 hips; 8 females/3 males). One resident and one experienced radiologist reviewed ultrasonography images performed in the fourth week. To compare the groups, Student's t and Mann–Whitney tests for normally and non-normally distributed covariates were performed. Accuracy of PFD to diagnose DDH was calculated. Intraclass correlation coefficient (ICC) was calculated to assess inter-observer agreement. Results: Mean PFDs of ND group were 3.09 mm at neutral position and 3.64 mm with the hip flexed. Mean PFDs of DH group were 6.29 mm and 7.59 mm, respectively. Sensitivity, specificity, and accuracy of PFD were 94.4%, 93.4%, and 97.2% (cut-off = 4.6 mm) at neutral position and 94.4%, 89.0%, and 95.5% (cut-off = 4.9 mm) with hip flexed. ICCs were 0.852 and 0.864, respectively. Conclusions: PFD is comparable with Graf method, enabling

  20. Comparison between Graf method and pubo-femoral distance in neutral and flexion positions to diagnose developmental dysplasia of the hip

    International Nuclear Information System (INIS)

    Teixeira, Sara R.; Dalto, Vitor F.; Maranho, Daniel A.; Zoghbi-Neto, Orlando S.; Volpon, José B.; Nogueira-Barbosa, Marcello H.

    2015-01-01

    Highlights: • The article adds information about the pubo-femoral distance (PFD) used as a simple tool to detect dysplastic hips in neonates. This articles shows that the PFD is comparable with the “gold standard” Graf method for the diagnosis of developmental dysplasia of the hip and it can be used as a screening tool for its diagnosis, regardless the radiologists’ experience, with high accuracy. - Abstract: Purposes: To evaluate whether the pubo-femoral distance (PFD) can be used as an accurate screening test to diagnose developmental dysplasia of the hip (DDH) in an at-risk population compared with the Graf method. Second, to determine whether PFD assessment is feasible and reproducible regardless of the observer's experience. Materials and methods: IRB approved this retrospective single-institution study. Written informed consent was waived. Between January 2010 and March 2012, 116 neonates at risk for DDH were included. Infants’ hips were distributed into two groups according to recommendation for treatment: non-dysplastic (ND; Graf I/IIA; 211 hips; 69 females/37 males) and dysplastic hip (DH; Graf IIB/IIC/III/D/IV; 21 hips; 8 females/3 males). One resident and one experienced radiologist reviewed ultrasonography images performed in the fourth week. To compare the groups, Student's t and Mann–Whitney tests for normally and non-normally distributed covariates were performed. Accuracy of PFD to diagnose DDH was calculated. Intraclass correlation coefficient (ICC) was calculated to assess inter-observer agreement. Results: Mean PFDs of ND group were 3.09 mm at neutral position and 3.64 mm with the hip flexed. Mean PFDs of DH group were 6.29 mm and 7.59 mm, respectively. Sensitivity, specificity, and accuracy of PFD were 94.4%, 93.4%, and 97.2% (cut-off = 4.6 mm) at neutral position and 94.4%, 89.0%, and 95.5% (cut-off = 4.9 mm) with hip flexed. ICCs were 0.852 and 0.864, respectively. Conclusions: PFD is comparable with Graf method, enabling

  1. [Long-term clinical and radiological outcomes in a serie of 26 cases of symptomatic adult developmental dysplasia of the hip managed with bernese periacetabular osteotomy].

    Science.gov (United States)

    Alcobía Díaz, B; Luque Pérez, R; García Bullón, I; Moro Rodríguez, L E; López-Durán Stern, L

    2015-01-01

    Developmental hip dysplasia is a frequent cause of coxofemoral pain in young adults. Bernese periacetabular osteotomy emerges as a possible option for the management of pain relief and functional limitation, in order to delay the need for arthroplasty in these patients. A descriptive-retrospective study was conducted on 26 selected patients with symptomatic developmental hip dysplasia treated with bernese periacetabular osteotomy between 1996 and 2009 (94% women). Mean age at time of surgery was 39.8 y (15-49 y), with a mean follow-up of 10 years. Osteoarthritis (OA Tönnis scale), acetabular index and Wiberg angle were evaluated by radiology and functionality was valued by using the de Merle-D'Aubigné-Postel scale. The mean hospitalization time was 10 days. At 10 years, the mean radiography value of acetabular index was 9.03° and 38.3° for Wiberg angle (17° and 27° correction, respectively, above the mean pre-operative values). Joint lock was referred to by 43% of patients, and 53% to non-evidence based limb failure. Mean functional value was 14.30 (Good). At 5 years of follow-up, 20% advanced at least by one grade in OA Tönnis scale compared to their pre-operative status, increasing to 55% at 10 years. At 10 years after surgery, 83% patients did not need arthroplasty and 85% showed high satisfaction level. Bernese periacetabular osteotomy is a useful alternative in young adults with symptomatic developmental hip dysplasia that can improve pain relief, femoral head coverage, and slow down coxofemoral osteoarthrosis progression in order to delay arthroplasty. Copyright © 2014 SECOT. Published by Elsevier Espana. All rights reserved.

  2. Differential genetic regulation of canine hip dysplasia and osteoarthritis.

    Directory of Open Access Journals (Sweden)

    Zhengkui Zhou

    2010-10-01

    Full Text Available Canine hip dysplasia (HD is a common polygenic trait characterized by hip malformation that results in osteoarthritis (OA. The condition in dogs is very similar to developmental dysplasia of the human hip which also leads to OA.A total of 721 dogs, including both an association and linkage population, were genotyped. The association population included 8 pure breeds (Labrador retriever, Greyhounds, German Shepherd, Newfoundland, Golden retriever, Rottweiler, Border Collie and Bernese Mountain Dog. The linkage population included Labrador retrievers, Greyhounds, and their crosses. Of these, 366 dogs were genotyped at ∼22,000 single nucleotide polymorphism (SNP loci and a targeted screen across 8 chromosomes with ∼3,300 SNPs was performed on 551 dogs (196 dogs were common to both sets. A mixed linear model approach was used to perform an association study on this combined association and linkage population. The study identified 4 susceptibility SNPs associated with HD and 2 SNPs associated with hip OA.The identified SNPs included those near known genes (PTPRD, PARD3B, and COL15A1 reported to be associated with, or expressed in, OA in humans. This suggested that the canine model could provide a unique opportunity to identify genes underlying natural HD and hip OA, which are common and debilitating conditions in both dogs and humans.

  3. The role of the acetabular labrum in hip dysplasia

    DEFF Research Database (Denmark)

    Hartig-Andreasen, Charlotte; Søballe, Kjeld; Troelsen, Anders

    2013-01-01

    A periacetabular osteotomy (PAO) is the preferred joint preserving treatment for young adults with symptomatic hip dysplasia and no osteoarthritis. In symptomatic dysplasia of the hip, there is labral pathology in up to 90% of cases. However, no consensus exists as to whether a labral tear should...... be treated before the periacetabular osteotomy (PAO), treated simultaneously with the PAO, or left alone and only treated if symptoms persist after the PAO. This review is an update of aspects of labral anatomy and function, the etiology of labral tears in hip dysplasia, and diagnostic assessment of labral...... tears, and we discuss treatment strategies for coexisting labral tears and hip dysplasia....

  4. Pseudoaneurysm Accompanied by Crowe Type IV Developmental Dysplasia of the Hip: A Case Report

    Directory of Open Access Journals (Sweden)

    Hirotake Yo

    2012-01-01

    Full Text Available We report the case of a 72-year-old woman whose pseudoaneurysm was difficult to diagnose and treat. The patient had a history of congenital dislocated hip and was undergoing anticoagulation therapy with warfarin due to the mitral valve replacement. Her chief complaint was pain and enlargement of the left buttock, and the laboratory tests revealed severe anemia. However, her elderly depression confused her chief complaint, and she was transferred to a psychiatric hospital. Two months after the onset of the symptoms, she was finally diagnosed with a pseudoaneurysm by contrast-enhanced CT and angiography. IDC coils were used for embolization. A plain CT showed hemostasis as well as a reduced hematoma at 2 months after the embolization. The possible contributing factors for the pseudoaneurysm included bleeding due to warfarin combined with an intramuscular hematoma accompanied by Crowe type IV developmental dysplasia of the hip that led to an arterial rupture by impingement between pelvis and femoral head. Since the warfarin treatment could not be halted due to the valve replacement, embolization was chosen for her treatment, and the treatment outcome was favorable.

  5. The association between gender and familial prevalence of hip dysplasia in Danish patients

    DEFF Research Database (Denmark)

    El Jashi, Rima; Gustafson, Maria B; Jakobsen, Mette B

    2017-01-01

    BACKGROUND: The development of hip dysplasia is associated with several risk factors. 1 of these risk factors is gender, since 80% of patients with symptomatic hip dysplasia are females. Another risk factor for hip dysplasia is familial predisposition of hip dysplasia. Several studies indicate...... that the risk of hip dysplasia is increased with familial prevalence of hip dysplasia. However, little is known about the association between the familial prevalence and gender and the development of hip dysplasia. PURPOSE: The aim of the study was to estimate the prevalence of hip dysplasia among relatives...... to Danish patients with hip dysplasia operated with periacetabular osteotomy (PAO), and the degree of relationship of affected family members. Furthermore, to assess the association between gender and family predisposition in the same group of patients. METHOD: The study is a cross-sectional study...

  6. Quantitative measurement and analysis for detection and treatment planning of developmental dysplasia of the hip

    Science.gov (United States)

    Liu, Xin; Lu, Hongbing; Chen, Hanyong; Zhao, Li; Shi, Zhengxing; Liang, Zhengrong

    2009-02-01

    Developmental dysplasia of the hip is a congenital hip joint malformation affecting the proximal femurs and acetabulum that are subluxatable, dislocatable, and dislocated. Conventionally, physicians made diagnoses and treatments only based on findings from two-dimensional (2D) images by manually calculating clinic parameters. However, anatomical complexity of the disease and the limitation of current standard procedures make accurate diagnosis quite difficultly. In this study, we developed a system that provides quantitative measurement of 3D clinical indexes based on computed tomography (CT) images. To extract bone structure from surrounding tissues more accurately, the system firstly segments the bone using a knowledge-based fuzzy clustering method, which is formulated by modifying the objective function of the standard fuzzy c-means algorithm with additive adaptation penalty. The second part of the system calculates automatically the clinical indexes, which are extended from 2D to 3D for accurate description of spatial relationship between femurs and acetabulum. To evaluate the system performance, experimental study based on 22 patients with unilateral or bilateral affected hip was performed. The results of 3D acetabulum index (AI) automatically provided by the system were validated by comparison with 2D results measured by surgeons manually. The correlation between the two results was found to be 0.622 (p<0.01).

  7. Assessment of adult hip dysplasia and the outcome of surgical treatment

    DEFF Research Database (Denmark)

    Troelsen, Anders

    2012-01-01

    Hip dysplasia and hip joint deformities in general are recognized as possible precursors of osteoarthritic development. Early and correct identification of hip dysplasia is important in order to offer timely joint preserving treatment. In the contemporary literature, several controversies exist......, and some of these were the focus of this doctoral thesis. Categorized into subjects, the major findings and their possible importance are listed below. DIAGNOSTIC ASSESSMENT OF HIP DYSPLASIA: A multi-observer study quantified the variability of different methods for diagnostic assessment of hip dysplasia...... and osteoarthritis and resulted in general recommendations regarding diagnostic assessment of hip dysplasia. Pelvic tilt was shown to differ significantly between the supine and weight-bearing positions in patients with dysplastic hip joints. This is a finding that adds controversy to the application of neutral...

  8. Gait symmetry and hip strength in women with developmental dysplasia following hip arthroplasty compared to healthy subjects: A cross-sectional study.

    Directory of Open Access Journals (Sweden)

    Ruud A Leijendekkers

    Full Text Available Untreated unilateral developmental dysplasia of the hip (DDH results in asymmetry of gait and hip strength and may lead to early osteoarthritis, which is commonly treated with a total hip arthroplasty (THA. There is limited knowledge about the obtained symmetry of gait and hip strength after the THA. The objectives of this cross-sectional study were to: a identify asymmetries between the operated and non-operated side in kinematics, kinetics and hip strength, b analyze if increased walking speed changed the level of asymmetry in patients c compare these results with those of healthy subjects.Women (18-70 year with unilateral DDH who had undergone unilateral THA were eligible for inclusion. Vicon gait analysis system was used to collect frontal and sagittal plane kinematic and kinetic parameters of the hip joint, pelvis and trunk during walking at comfortable walking speed and increased walking speed. Furthermore, hip abductor and extensor muscle strength was measured.Six patients and eight healthy subjects were included. In the patients, modest asymmetries in lower limb kinematics and kinetics were present during gait, but trunk lateral flexion asymmetry was evident. Patients' trunk lateral flexion also differed compared to healthy subjects. Walking speed did not significantly influence the level of asymmetry. The hip abduction strength asymmetry of 23% was not statistically significant, but the muscle strength of both sides were significantly weaker than those of healthy subjects.In patients with a DDH treated with an IBG THA modest asymmetries in gait kinematics and kinetics were present, with the exception of a substantial asymmetry of the trunk lateral flexion. Increased walking speed did not result in increased asymmetries in gait kinematics and kinetics. Hip muscle strength was symmetrical in patients, but significantly weaker than in healthy subjects. Trunk kinematics should be included as an outcome measure to assess the biomechanical

  9. Assessment of hip dysplasia and osteoarthritis: Variability of different methods

    International Nuclear Information System (INIS)

    Troelsen, Anders; Elmengaard, Brian; Soeballe, Kjeld; Roemer, Lone; Kring, Soeren

    2010-01-01

    Background: Reliable assessment of hip dysplasia and osteoarthritis is crucial in young adults who may benefit from joint-preserving surgery. Purpose: To investigate the variability of different methods for diagnostic assessment of hip dysplasia and osteoarthritis. Material and Methods: By each of four observers, two assessments were done by vision and two by angle construction. For both methods, the intra- and interobserver variability of center-edge and acetabular index angle assessment were analyzed. The observers' ability to diagnose hip dysplasia and osteoarthritis were assessed. All measures were compared to those made on computed tomography scan. Results: Intra- and interobserver variability of angle assessment was less when angles were drawn compared with assessment by vision, and the observers' ability to diagnose hip dysplasia improved when angles were drawn. Assessment of osteoarthritis in general showed poor agreement with findings on computed tomography scan. Conclusion: We recommend that angles always should be drawn for assessment of hip dysplasia on pelvic radiographs. Given the inherent variability of diagnostic assessment of hip dysplasia, a computed tomography scan could be considered in patients with relevant hip symptoms and a center-edge angle between 20 deg and 30 deg. Osteoarthritis should be assessed by measuring the joint space width or by classifying the Toennis grade as either 0-1 or 2-3

  10. Comparison of three methods to diagnose hip dysplasia in dogs

    International Nuclear Information System (INIS)

    Sharma, Vikas; Mohindroo, J.

    2009-01-01

    The present study was designed to compare the usefulness of goniometry, radiography and distraction index in diagnosis of hip dysplasia in dogs. During the study 25 clinical cases (50 joints) suspected for hip dysplasia were evaluated. Norberg angle was found to have a significant positive correlation with extension, flexion, abduction, and adduction angles and a significant negative correlation with distraction index (DI) measurements. It could be inferred that all the six parameters (NA, DI, extension, flexion, abduction, and adduction) were reliable indicators for early diagnosis of hip dysplasia.Goniometry could be used as a safe and easy method for preliminary suspicion of hip dysplasia

  11. Muscle Damage After Total Hip Arthroplasty Through the Direct Anterior Approach for Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Kawasaki, Masashi; Hasegawa, Yukiharu; Okura, Toshiaki; Ochiai, Satoshi; Fujibayashi, Takayoshi

    2017-08-01

    Total hip arthroplasty (THA) through the direct anterior approach (DAA) is known to cause less muscle damage than other surgical approaches. However, more complex primary cases, such as developmental dysplasia of the hip (DDH), might often cause muscle damage. The objective of the present study was to clarify the muscle damage observed 1 year after THA through the DAA for DDH using magnetic resonance imaging. We prospectively compared the muscle cross-sectional area (M-CSA) and fatty atrophy (FA) in muscles by magnetic resonance imaging and the Harris hip score before and at 1-year follow-up after THA through the DAA in 3 groups: 37 patients with Crowe group 1 DDH (D1), 13 patients with Crowe group 2 and 3 DDH (D2 + 3), and 12 patients with osteonecrosis as a control. THA through the DAA for D1 displayed significantly decreased M-CSA and significantly increased FA in the gluteus minimus (Gmini), the tensor fasciae latae (TFL), and the obturator internus (OI). Patients with D2 + 3 group did not have decreased M-CSA in the TFL or increased FA in the Gmini. Postoperatively, a significant negative correlation was observed between the M-CSA and FA for the OI in patients with D1 and D2 + 3. THA through the DAA for DDH caused the damage in the Gmini, the TFL, and the OI; severe damage was observed in the OI, showing increased FA with decreased M-CSA in patients with both D1 and D2 + 3. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Ipsilateral Hip Dysplasia in Patients with Sacral Hemiagenesis: A Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Tadatsugu Morimoto

    2015-01-01

    Full Text Available Sacral agenesis (SA is a rare condition consisting of the imperfect development of any part of the sacrum. This paper describes two cases of the rare cooccurrence of ipsilateral SA and developmental dysplasia of the hip (DDH and analyzes possible contributory factors for SA and DDH. Each of a 16-year-old female and 13-year-old female visited our hospital for left hip pain and limping. The findings of physical examinations showed a lower limb length discrepancy (left side in both cases, as well as left hip pain without limitations of the range of motion or neurological deficits. Initial radiographs demonstrated left subluxation of the left hip with associated acetabular dysplasia and partial left sacral agenesis. MRI revealed a tethering cord with a fatty filum terminale, and periacetabular osteotomy combined with allogeneic bone grafting was performed. After the surgery, the patients experienced no further pain, with no leg length discrepancy and were able to walk without a limp, being neurologically normal with a normal left hip range of motion. The cooccurrence of SA and DDH suggests a plausible hypothesis to explain the embryogenic relationship between malformation of the sacrum and hip.

  13. Inclination and anteversion of Collum femoris in hip dysplasia and coxarthritis

    Energy Technology Data Exchange (ETDEWEB)

    Madsen, J. S.; Svalastoga, E. [Kongelige Veterinaer- og Landbohoejskole, Copenhagen (Denmark)

    1994-07-01

    Femoral neck angles were measured radiographically in 41 dogs examined for hip dysplasia. Steep femoral neck inclination was found to be a phenomenon of hip dysplasia and coxofemoral joint laxity. The altered biomechanics of a steep femoral neck inclination may be a factor in the pathogenesis of hip dysplasia and secondary osteoarthritis.

  14. Total hip replacement with a superolateral bone graft for osteoarthritis secondary to dysplasia: a long-term follow-up

    NARCIS (Netherlands)

    de Jong, P. T.; Haverkamp, D.; van der Vis, H. M.; Marti, R. K.

    2006-01-01

    We evaluated the long-term results of 116 total hip replacements with a superolateral shelfplasty in 102 patients with osteoarthritis secondary to developmental dysplasia of the hip. After a mean follow-up of 19.5 years (11.5 to 26.0), 14 acetabular components (12%) had been revised. The cumulative

  15. Assessment of hip dysplasia and osteoarthritis: Variability of different methods

    Energy Technology Data Exchange (ETDEWEB)

    Troelsen, Anders; Elmengaard, Brian; Soeballe, Kjeld (Orthopedic Research Unit, Univ. Hospital of Aarhus, Aarhus (Denmark)), e-mail: a_troelsen@hotmail.com; Roemer, Lone (Dept. of Radiology, Univ. Hospital of Aarhus, Aarhus (Denmark)); Kring, Soeren (Dept. of Orthopedic Surgery, Aabenraa Hospital, Aabenraa (Denmark))

    2010-03-15

    Background: Reliable assessment of hip dysplasia and osteoarthritis is crucial in young adults who may benefit from joint-preserving surgery. Purpose: To investigate the variability of different methods for diagnostic assessment of hip dysplasia and osteoarthritis. Material and Methods: By each of four observers, two assessments were done by vision and two by angle construction. For both methods, the intra- and interobserver variability of center-edge and acetabular index angle assessment were analyzed. The observers' ability to diagnose hip dysplasia and osteoarthritis were assessed. All measures were compared to those made on computed tomography scan. Results: Intra- and interobserver variability of angle assessment was less when angles were drawn compared with assessment by vision, and the observers' ability to diagnose hip dysplasia improved when angles were drawn. Assessment of osteoarthritis in general showed poor agreement with findings on computed tomography scan. Conclusion: We recommend that angles always should be drawn for assessment of hip dysplasia on pelvic radiographs. Given the inherent variability of diagnostic assessment of hip dysplasia, a computed tomography scan could be considered in patients with relevant hip symptoms and a center-edge angle between 20 deg and 30 deg. Osteoarthritis should be assessed by measuring the joint space width or by classifying the Toennis grade as either 0-1 or 2-3

  16. Canine hip dysplasia: significance of early bony spurring

    International Nuclear Information System (INIS)

    Morgan, J.P.

    1987-01-01

    It is the purpose of this study to call attention to new bone production that often occurs early in the sequence of pathological changes associated with canine hip dysplasia. New bone production extending to bony remodeling, as well as femoral head subluxation, both occur in the sequence of pathologic changes associated with canine hip dysplasia. Subluxation is considered primary, while osteoarthrosis is a secondary feature, and both are used in the diagnosis of canine hip dysplasia. This report concerns the significance of the presence of a solitary bony osteophyte, or spur, that is frequently evident on the caudal aspect of the femoral neck as viewed on the conventional ventrodorsal projection. This report utilizes findings from pelvic radiographs of 605 dogs (five breeds). There was a greater frequency (54%)of this bony change in cases diagnosed radiographically as dysplastic than in cases diagnosed as normal(15%).Thus, it is suggested that this minimal radiographic change can be used as an indicator of early canine hip dysplasia, especially in the absence of subluxation of the femoral head

  17. Hip dysplasia: a significant risk factor for the development of hip osteoarthritis. A cross-sectional survey

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig

    2005-01-01

    joint space width (JSW) /=60 yr of age. Of factors entered into logistic regression analyses, only age (P LT 0.001 for right hips and P LT 0.001 for left hips) and hip dysplasia (P LT 0.001 for right hips and P = 0.004 for left hips) were significantly associated with hip OA prevalence in women. In men......, only hip dysplasia was associated with hip OA prevalence, P LT 0.001 in right hips and P = 0.001 in left hips. CONCLUSIONS: Of the individual risk factors investigated in this study, only age and hip dysplasia were associated with the development of hip osteoarthritis.......OBJECTIVES: The aim of this cross-sectional survey of 2232 women and 1336 men (age range 20-91 yr) was to investigate individual risk factors for hip joint osteoarthritis (OA). METHODS: Standardized, weight-bearing pelvic radiographs were evaluated. Radiological hip joint OA was defined as minimum...

  18. Association between open or closed reduction and avascular necrosis in developmental dysplasia of the hip

    Science.gov (United States)

    Wang, Ya-Jie; Yang, Fan; Wu, Qi-Jun; Pan, Shi-Nong; Li, Lian-Yong

    2016-01-01

    Abstract Background: The risk of avascular necrosis of the femoral head (AVN) after treatment of developmental dysplasia of the hip is associated with the method of reduction. Some authors have suggested that open reduction is a risk factor for AVN; however, this is controversial. To our knowledge, a quantitative comparison of the incidence of AVN between closed and open reduction has not been conducted. Methods: Published studies were identified by searching PubMed, EMBASE, and the Cochrane Library up to May, 2015, focusing on the incidence of AVN after closed or open reduction for developmental dysplasia of the hip in children aged <3 years. Patients were age-matched who were treated by either closed or open reduction, but without pelvic or femoral osteotomy. Two authors independently assessed eligibility and abstracted data. Discrepancies were discussed and resolved by consensus. We pooled the odds ratios (ORs) and 95% confidence intervals (95%CIs) from individual studies using a random-effects model and evaluated heterogeneity and publication bias. Results: Nine retrospective studies were included in this analysis. The pooled OR for comparing open reduction with closed reduction for all grades of AVN was 2.26 (95%CI = 1.21–4.22), with moderate heterogeneity (I2 = 44.7%, P = 0.107). The pooled OR for grades II to IV AVN was 2.46 (95%CI = 0.93–6.51), with high heterogeneity (I2 = 69.6%, P = 0.003). A significant association was also found for the further surgery between open and closed reduction, with a pooled OR of 0.30 (95%CI = 0.15–0.60) and moderate heterogeneity (I2 = 46.4%, P = 0.133). No evidence of publication bias or significant heterogeneity between subgroups was detected by meta-regression analyses. Conclusion: Findings from this meta-analysis suggest that open reduction is a risk factor for the development of AVN compared with closed treatment. Future studies are warranted to investigate how open reduction

  19. Reliability of ultrasonographic measurements in suspected patients of developmental dysplasia of the hip and correlation with the acetabular index

    Directory of Open Access Journals (Sweden)

    Cem Copuroglu

    2011-01-01

    Full Text Available Background: Ultrasonography is accepted as a useful imaging modality in the early detection of developmental dysplasia of the hip (DDH. Early detection and early treatment of DDH prevents hip dislocation and related physical, social, economic, and psychological problems. The purpose of this study was to evaluate the reliability of ultrasonographic and roentgenographic measurements measured by seven different observers. Materials and Methods: The alpha angles of 66 hips in 33 patients were measured using the Graf method by seven different observers. Acetabular index degrees on plane roentgenograms were measured in order to assess the correlation between the ultrasonographic alpha angle and the radiographic acetabular index, which both show the bony acetabular depth, retrospectively. Results: The interclass correlation coefficient, measuring the interobserver reliability, was high and statistically significant for the ultrasonographic measurements. There was a negative correlation between the alpha angle and the acetabular index. Conclusions: Ultrasonography, when applied properly, is a reliable technique between different observers, in the diagnosis and follow up of DDH. When assessed concomitantly with the roentgenographic measurements, the results are reliable and statistically meaningful.

  20. The incidence of avascular necrosis and the radiographic outcome following medial open reduction in children with developmental dysplasia of the hip: a systematic review.

    Science.gov (United States)

    Gardner, R O E; Bradley, C S; Howard, A; Narayanan, U G; Wedge, J H; Kelley, S P

    2014-02-01

    The incidence of clinically significant avascular necrosis (AVN) following medial open reduction of the dislocated hip in children with developmental dysplasia of the hip (DDH) remains unknown. We performed a systematic review of the literature to identify all clinical studies reporting the results of medial open reduction surgery. A total of 14 papers reporting 734 hips met the inclusion criteria. The mean follow-up was 10.9 years (2 to 28). The rate of clinically significant AVN (types 2 to 4) was 20% (149/734). From these papers 221 hips in 174 children had sufficient information to permit more detailed analysis. The rate of AVN increased with the length of follow-up to 24% at skeletal maturity, with type 2 AVN predominating in hips after five years' follow-up. The presence of AVN resulted in a higher incidence of an unsatisfactory outcome at skeletal maturity (55% vs 20% in hips with no AVN; p AVN was identified when surgery was performed in children aged AVN.

  1. [Risk of developmental dysplasia of the hip in patients subjected to the external cephalic version].

    Science.gov (United States)

    Sarmiento Carrera, Nerea; González Colmenero, Eva; Vázquez Castelo, José Luis; Concheiro Guisán, Ana; Couceiro Naveira, Emilio; Fernández Lorenzo, José Ramón

    2018-03-01

    Developmental dysplasia of the hip (DDH) refers to the spectrum of abnormalities of maturation and development of the hip. Breech presentation is associated with DDH. This risk factor can be modified by external cephalic version (ECV). The aim of this study is to evaluate the incidence of DDH in patients who successfully underwent ECV, as well as to evaluate need for these children (breech for a period during gestation) to be included in the DDH screening protocol. A prospective cohort study was conducted in the Hospital Universitario de Vigo from January 1, 2015 to December 31, 2015. It included children born in cephalic presentation after a successful ECV, as well as children born in breech presentation. They all were screened for DDH by ultrasound examination of the hip. Out of a total of 122 newborns included in the study, ECV was attempted on 67 (54.9%), of which 35 (52.2%) were successful. Out of the 14 children diagnosed with DDH, 3 of those born in cephalic presentation after a successful ECV were found to be normal on physical examination. Successful ECV is associated with a lower incidence of DDH as regards breech presentation. However, these patients should be included in the DDH screening protocol for the early detection of this disorder. Copyright © 2017 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  2. [New classification of Crowe type IV developmental dysplasia of the hip].

    Science.gov (United States)

    Ma, Hai-yang; Zhou, Yong-gang; Zheng, Chong; Cao, Wen-zhe; Wang Sen; Wu, Wen-ming; Piao, Shang; Du, Yin-qiao

    2016-02-01

    To compare differences between Crowe IV developmental dysplasia of the hip (DDH) with secondary acetabulum and Crowe IV DDH without secondary acetabulum,and determine whether it is necessary to divide Crowe IV DDH into two subtypes. From June 2007 to May 2015,145 hips of 112 Crowe N patients who underwent total hip arthroplasty (THA) using S-ROM stem were divided into two groups: secondary acetabulum formaton group (group A) and no secondary acetabulum formaton group (group B). In group A,there were 12 females, 96 males,with an average age of (39.38 ± 11.19) years old. In group B, there were 2 females, 35 males, with an average age of (38.19 ± 10.92) years old. All the patients were evaluated by using Harris Hip Score. Radiographic evaluations were made preoperatively and during follow up. The differences between two groups were compared on dislocation height, canal flare index (CFI), subtrochanteric shortening osteotomy (SSTO) usage, pre- and post-operation Harris scores, complications. The dislocation height for group A was (4.74 ± 1.57) cm, while the dislocation height for group B was (3.12 ± 1.15) cm. Significantly difference was detected between two groups. The CFI for group A was 2.69 ± 0.68, while the CFI for group B was 3.42 ± 0.79, and the significantly difference was detected between two groups. Harris scores were totally improved from 58.18 ± 15.67 preoperatively to 91.20 ± 3.79 post-operatively and the difference was significant. Pre-operative Harris scores was 58.1 ± 15.3 in group A, 58.3 ± 16.9 in group B. Post-operative Harris scores was 91.0 ± 4.1 in group A, 91.0 ± 5.1 in group B. No significant difference was found on Harris scores between A and B preoperatively and post-operatively. Complications of 4 cases peri-prosthesis fracture, 4 cases dislocation and 4 cases nerve injury occur in group A; While only one case dislocation and one case nerve injury occur in group B. No statistical significance was detected. Crowe IV DDH with

  3. Pelvic orientation and assessment of hip dysplasia in adults

    DEFF Research Database (Denmark)

    Jacobsen, S.; Holm, S.S.; Lund, B.

    2004-01-01

    on the measurements of radiographic indices of hip dysplasia. MATERIAL AND METHODS: We investigated the effect of varying pelvic orientation on radiographic measurements of acetabular dysplasia using a cadaver model. Results from the cadaver study were used to validate the radiographic assessments of acetabular...... radiograph was recorded at each 3 degrees increment. The most widely used radiographic parameters of hip dysplasia were assessed. 2) Critical limits of acceptable rotation and inclination/reclination of pelvises were determined on 4151 standing, standardised pelvic radiographs of the CCHS cohort. RESULTS......BACKGROUND: The study was performed to qualify the source material of 4151 pelvic radiographs for the research into the relationship between unrecognised childhood hip disorders and the development of hip osteoarthrosis, and to investigate the effect of varying degrees of pelvic tilt and rotation...

  4. [Locomotor development in infants with developmental dysplasia of the hip or idiopathic clubfoot undergoing orthopedic treatment. Prospective comparative study].

    Science.gov (United States)

    Masquijo, J J; Campos, L; Torres-Gómez, A; Allende, V

    2013-10-01

    Several disorders of early childhood, such as developmental dysplasia of the hip (DDH) and clubfoot, requires orthopedic treatment that limits active mobility of the lower extremities for a period of time. The aim of our study was to evaluate the impact on locomotor development of the orthopedic treatment in infants less than one year-old. The study included a prospective cohort of consecutive patients diagnosed with developmental dysplasia of the hip (Group A, 24 patients), and clubfoot (Group B, 32 patients) treated from January 2007 to June 2009. A third group (Group C) of 50 healthy children was used as control. The variables evaluated were: months with a brace, age to sit without support, age at the start of crawling, and age at the beginning of walking. The results obtained were analyzed. Comparisons between the three groups were performed using the Kruskal-Wallis test and Mann-Whitney test. We chose a value of P<.05 as level of statistical significance. The analysis of independent samples showed that the mean age at which the patients began to sit were similar: 6.12, 6.42 and 6.19 months, respectively (P=.249). The mean age for crawling was similar, although with a slight trend toward statistical significance: 8.84, 9.38 and 9.17 months, respectively (P=.08). The age at which they started walking was different between the three groups: 12.14, 13.21 and 12.41 months, respectively (P<.001). Orthopedic treatment of DDH and clubfoot in children less than one year-old slightly slows down the course of normal locomotor development. Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  5. Overhead Bryant's Traction Does Not Improve the Success of Closed Reduction or Limit AVN in Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Sucato, Daniel J; De La Rocha, Adriana; Lau, Karlee; Ramo, Brandon A

    2017-03-01

    Preoperative Bryant's overhead traction before closed reduction (CR) in developmental dysplasia of the hip (DDH) remains controversial and its success in increasing CR rates and reducing avascular necrosis (AVN) rates has not been specifically reported in a large cohort. IRB-approved retrospective study of patients (below 3 y)who were treated with attempted CR for idiopathic DDH from 1980 to 2009. Successful CR was defined as a hip that remained reduced and did not require repeat CR or open reduction. Patients were grouped by age, hip instability [Ortolani positive (reducible) vs. fixed dislocation], and Tonnis classification and rates of successful CR were compared between groups with PAVN between the traction (18%) and no-traction (8%) groups for all patients (P=0.15). In this retrospective series, preoperative Bryant's traction does not improve the rate of a successful CR for patients with DDH and has no protective effect on the development of AVN of the femoral head. These results suggest that Bryant's overhead traction may not be warranted for patients below 3 years of age with DDH. Level III.

  6. Two-dimensional and three-dimensional CT analysis of congenital hip dysplasia in the older child and adult

    International Nuclear Information System (INIS)

    Magid, D.; Fishman, E.K.; Brooker, A.F.; Sponseller, P.D.

    1987-01-01

    In the older child or adult with congenital dysplasia of the hip, a spectrum of developmental abnormalities of the femoral head, neck, and acetabulum produce progressive hip dysfunction, pain, and premature arthritis. Treatment is oriented to restoring normal and pain-free function, although (unlike the infant with successfully treated dislocation) a truly normal hip cannot be restored. For these patients, who may require extensive reconstruction, transaxial CT with both static 2D (coronal and sagittal) and animated volumetric 3D image reformatting offers the most complete, accurate, and integrated approach to diagnosis and therapeutic planning. Parameters preoperatively assessed include head subluxation, neck anteversion, quadrilateral plate thickness, degree of dysplasia, potential for concentric reduction, acetabular capacity, and muscle status. Postoperatively, both immediate and long-term results or complications are easily assessed and readily compared

  7. Progression of hip dysplasia in 40 police working dogs: a retrospective study

    International Nuclear Information System (INIS)

    Zorko, B.; Ivanusa, T.; Pelc, R.

    2005-01-01

    The aim of the study was to evaluate the effects of progression of CHD and degenerative joint disease on a working capability of population of police working dogs. In the first part of the study, we reviewed the whole health documentation of all dogs and gathered all necessary radiographs of hip joints that were taken at first initial procurement. In the second part, clinical examination and radiographs of all dogs were taken 60 months after first examination and the progression of hip dysplasia was evaluated by the FCI scheme. All dogs were male, 35 German shepherds and 5 Rottweilers. The Norberg-Olsson angle in the first set of radiographs was 105.54 +/- 3.22 deg in 37 dogs without hip dysplasia and 100.17 +/- 2.99 deg in 3 dogs with initial mild dysplasia. In the second set of radiographs taken after 60 months of service the Norberg- Olsson angle was 105.60 +/- 3.67 deg in 23 dogs with no signs of hip dysplasia and 101.62 +/- 4.49 deg in 17 dogs with hip dysplasia. On the first set of radiographs, secondary degenerative changes were found in 3 dogs with initial mild dysplasia (7.5 %) and in 14 dogs (35 %) on second radiographs. The position of the centre of the femoral head on first radiographs was outside of dorsal acetabular rim in 25 (31.25 %) of estimated hip joints (n = 80); at the level of the rim in 30 (37.5 %) and inside in 25 (31.25 %). On second radiographs it was outside in 41 (51.25 %); at the level of the rim in 26 (32.5 %); and inside in 13 (31.25 %) of estimated hip joints. The mean femoral angle of inclination for all 40 dogs was 132.50 +/- 4.39? deg . If it has occured, the increase in degree of hip dysplasia was generally more than one degree. In 57.5 % of cases hip dysplasia was not determined on second radiographs. All dogs with some degree of hip dysplasia were German shepherds, but only one of them had clinical symptoms connected to CHD. The progression of hip dysplasia did not correlate with work period of the dogs, and has no influence on

  8. Joint space width in dysplasia of the hip

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig; Søballe, K

    2005-01-01

    . Neither subjects with dysplasia nor controls had radiological signs of ongoing degenerative disease at admission. The primary radiological discriminator of degeneration of the hip was a change in the minimum joint space width over time. There were no significant differences between these with dysplasia...

  9. A simple technique to strengthen the initial and mid-term to long-term stability of the cup during total hip arthroplasty in developmental dysplasia of the hip.

    Science.gov (United States)

    Guan, Mingqiang; Zhou, Guanming; Li, Xue

    2018-04-01

    To assess the effects of a technique of cup blocking screws combined with impaction bone grafting during total hip arthroplasty (THA) for patients with developmental dysplasia of the hip (DDH). From August 2011 to July 2015, 53 patients (59 hips) with DDH in our hospital were treated with THA using the technique of cup blocking screws combined with impaction particulate bone grafting. These patients were prospectively followed, and the clinical and imaging results were collected. Harris hip score (HHS) was raised from 41.24 before surgery to 91.49 at the latest follow-up (p less than 0.001). Length discrepancy (LLD) was reduced from 28.97 mm before surgery to 6.08 mm after surgery (p less than 0.001). No loosening of the cup was detected at the last follow-up. The differences were insignificant in cup inclination and rate of cup coverage among the 3 groups of Crowe type II, type III, and type IV DDH (p greater than 0.05). The technique of cup blocking screws combined with impaction particulate bone grafting is simple and reliable, and it not only simplifies the attainment of initial stability, but also strengthens the mid-term to long-term stability during THA in DDH.

  10. MRI findings of the residual subluxation after reduction of developmental dysplasia of the hip

    International Nuclear Information System (INIS)

    Wakabayashi, Kenjirou; Wada, Ikuo; Horiuchi, Osamu; Otsuka, Takanobu

    2007-01-01

    After reduction of developmental dysplasia of the hip (DDH), not a few patients suffer from residual subluxation as well as acetabular dysplasia. Corrective surgery for these residual subluxations is sometimes performed before school-child age. It is occasionally difficult to determine whether the patient is candidate for the corrective surgery in the case with minor morphologic aberration. Therefore, we studied various MRI findings in residual subluxation, and examined if these MRI findings have an effect on natural courses such as growth or concentricity of the hip joint after reduction for DDH. The usefulness of MRI for determining the indication for the corrective surgery was also investigated. We studied 235 patients who underwent primary treatment for DDH during the past decade (between October, 1988 and September, 1998). Of the 235 patients, we studied 23 patients (23 cases; all unilateral cases) who showed residual subluxation and whose MR images were taken when they were around 3 years old, and during a follow-up period was available. Corrective surgery is performed in 11 patients, whereas the remaining 12 patients were conservatively observed the course of the residual subluxation. We investigated the existence of high signal intensity area (hereinafter referred to as HSIA) within the weight-bearing acetabular cartilage on T2-weighted MR coronal section images. Furthermore, conservatively observed patients were divided into two groups according to T2-weighted MR images. Based on simple X-ray images with time in both groups, the acetabular angle and the CE angle were measured. As results, many patients with residual subluxation showed localized HSIA in the weight-bearing acetabular cartilage on T2-weighted MR images. Although all patients who underwent corrective surgery showed HSIA, HSIA disappeared or decreased after the surgery. Of patients who were conservatively observed the course of the residual subluxation, patients showed HSIA on MR image had poor

  11. Measurement of center-edge angle in developmental dysplasia of the hip: a comparison of two methods in patients under 20 years of age

    Energy Technology Data Exchange (ETDEWEB)

    Oemeroglu, H. [Department of Orthopaedics and Traumatology, Osmangazi University Hospital, Eskisehir (Turkey); Genclik Cad., Ankara (Turkey); Bicimoglu, A. [Department of Orthopaedics and Traumatology, Ankara Numune Hospital, Ankara (Turkey); Agus, H. [Department of Orthopaedics and Traumatology, S.S.K. Tepecik Hospital, Izmir (Turkey); Tuemer, Y. [Orthopaedics and Traumatology Center, Ankara (Turkey)

    2002-01-01

    Objective: To analyse in detail the two methods for the measurement of the center-edge (CE) angle in developmental dysplasia of the hip (DDH) in children and adolescents. Design: Four observers independently interpreted the radiographs of 51 surgically treated and 15 unaffected hips on two occasions. CE angle was measured by using two methods: classic (Wiberg) and refined (Ogata et al.). Intraobserver and interobserver variations of both methods were calculated and the effect of age and treatment type on the two measurement methods were analysed. Results: Following 528 measurements in 66 hips, the mean classic CE angle was 28.2 and the mean refined CE angle, 19.9 . The classic CE angle had nearly 1 less intraobserver and interobserver variation than the refined CE angle. Classic angle measurements were obviously higher than refined ones in the hips of children under 9 years of age and in hips in which an innominate osteotomy had been performed. Conclusion: Although CE angle of Wiberg, which is an important radiographic indicator, has an adequate level of reliability and reproducibility it may not reflect the true lateral femoral head coverage in some cases. (orig.)

  12. Delayed ossification of the femoral head in dogs with hip dysplasia

    International Nuclear Information System (INIS)

    Madsen, J.S.; Reimann, I.; Svalastoga, E.

    1991-01-01

    In humans, delayed ossification of the caput femoris is often seen associated with hip dysplasia in babies. This phenomenon may possibly exist in dogs. In this study, the radiographic appearance of the caput femoris of 13 German shepherd dogs was examined. The dogs underwent pelvic radiography at the age of 14 to 15 days, six weeks, and 12 months. A significant relationship was shown between hip dysplasia and the late appearance of the epiphysis of the caput femoris (P=0–02). At the age of 14 to 15 days it was not possible to see both epiphyses in 54 per cent of the dogs. All of these dogs had hip dysplasia when they were 12 months old. This was in contrast to the six dogs which had both epiphyses visible when they were 14 to 15 days old. At 12 months of age, four of these dogs (67 per cent) did not show any sign of hip dysplasia

  13. Reliability of radiographic parameters in adults with hip dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Terjesen, Terje [Oslo University Hospital, Rikshospitalet, Department of Orthopaedics, Oslo (Norway); Gunderson, Ragnhild B. [Oslo University Hospital, Rikshospitalet, Department of Radiology, Oslo (Norway)

    2012-07-15

    To assess the reliability of radiographic measurements in adults previously treated for developmental dysplasia of the hip (DDH) and to clarify whether these parameters differ according to position of the patient (supine versus standing). Fifty-one patients (41 females and 10 males) with 63 affected hips were included in the study. The mean follow-up period was 45 (44-49) years in the patients who had not undergone total hip replacement (THR). Anteroposterior radiographs of the pelvis were taken with the patient in the supine and in the standing position. Measurements used for residual hip dysplasia were center-edge (CE) angle and migration percentage (MP). The joint space width (JSW) was measured at three or four locations of the upper, weight-bearing part of the joint, and the shortest distance was termed the minimum joint space width (minJSW). One radiologist and one orthopaedic surgeon, each with more than 30 years of experience, independently measured the radiographic parameters. The limits of agreement (LOA) of the CE angle (mean interobserver difference {+-} 2SD) were within the range -8 to 7 . The LOA of the MP were in the range -8 to 8% and of the minJSW -0.6 to 1.1 mm. The mean differences in CE angle between supine and standing radiographs (supine - standing) ranged from -1.1 to 0.0 and the mean differences in MP between supine and standing positions were below 1%. The mean positional differences in minJSW were below 0.1 mm and were not statistically significant. The interobserver variations with regard to CE angle, MP, and minJSW were moderate, indicating that these are reliable measurements in clinical practice. Femoral head coverage and JSW did not significantly differ between supine and weight-bearing positions. (orig.)

  14. Muscle-tendon related pain in 100 patients with hip dysplasia: prevalence and associations with self-reported hip disability and muscle strength

    DEFF Research Database (Denmark)

    Jacobsen, Julie Sandell

    2017-01-01

    Introduction Intra-articular injury has been described as primary cause of pain in hip dysplasia. At this point, it is unknown whether external muscle-tendon related pain coexists with intra-articular pathology. The primary aim was to identify muscle-tendon related pain in 100 patients with hip...... dysplasia. The secondary aim was to test if muscle-tendon related pain is linearly associated to self-reported hip disability and muscle strength in patients with hip dysplasia. Materials and methods One hundred patients (17 men) with a mean age of 29+9 years were included. Clinical entity approach...... (phip dysplasia with a high...

  15. Shielding during x-ray examination of pediatric female patients with developmental dysplasia of the hip

    International Nuclear Information System (INIS)

    Tsai, Yi-Shan; Liu, Yi-Sheng; Chuang, Ming-Tsung; Wang, Chien-Kuo; Lai, Cheng-Shih; Tsai, Hong-Ming; Lu, Chia-Hsing; Lin, Chii-Jeng

    2014-01-01

    Patients with developmental dysplasia of the hip (DDH) generally undergo multiple x-ray examinations of both hip joints. During these examinations, the gonads are completely exposed to radiation, unless shielded. Although many types and sizes of gonad shields exist, they often do not provide adequate protection because of size and placement issues; additionally, these shields are frequently omitted for female patients. Our aim was to assess gonad protection during x-ray examination that is provided by gonad shields designed for individual female patients with DDH. We retrospectively retrieved data from the Picture Archiving and Communication System database; pelvic plain x-ray films from 766 females, 18 years old or younger, were included in our analysis. Based on x-ray measurements of the anterior superior iliac spine, we developed a system of gonad shield design that depended on the distance between anterior superior iliac spine markers. We custom-made shields and then examined shielding rates and shielding accuracy before and after these new shields became available. Standard (general-purpose) shields were used before our custom design project was implemented. The shielding rate and shielding accuracy were, respectively, 14.5% and 8.4% before the project was implemented and 72.7% and 32.2% after it was implemented. A shield that is more anatomically correct and available in several different sizes may increase the likelihood of gonad protection during pelvic x-ray examinations. (paper)

  16. Shielding during x-ray examination of pediatric female patients with developmental dysplasia of the hip.

    Science.gov (United States)

    Tsai, Yi-Shan; Liu, Yi-Sheng; Chuang, Ming-Tsung; Wang, Chien-Kuo; Lai, Cheng-Shih; Tsai, Hong-Ming; Lin, Chii-Jeng; Lu, Chia-Hsing

    2014-12-01

    Patients with developmental dysplasia of the hip (DDH) generally undergo multiple x-ray examinations of both hip joints. During these examinations, the gonads are completely exposed to radiation, unless shielded. Although many types and sizes of gonad shields exist, they often do not provide adequate protection because of size and placement issues; additionally, these shields are frequently omitted for female patients. Our aim was to assess gonad protection during x-ray examination that is provided by gonad shields designed for individual female patients with DDH.We retrospectively retrieved data from the Picture Archiving and Communication System database; pelvic plain x-ray films from 766 females, 18 years old or younger, were included in our analysis. Based on x-ray measurements of the anterior superior iliac spine, we developed a system of gonad shield design that depended on the distance between anterior superior iliac spine markers. We custom-made shields and then examined shielding rates and shielding accuracy before and after these new shields became available. Standard (general-purpose) shields were used before our custom design project was implemented. The shielding rate and shielding accuracy were, respectively, 14.5% and 8.4% before the project was implemented and 72.7% and 32.2% after it was implemented. A shield that is more anatomically correct and available in several different sizes may increase the likelihood of gonad protection during pelvic x-ray examinations.

  17. Knee joint changes in patients with neglected developmental hip dysplasia: a prospective case-control study.

    Science.gov (United States)

    Li, Qiwei; Kadhim, Muayad; Zhang, Lijun; Cheng, Xiangjun; Zhao, Qun; Li, Lianyong

    2014-12-01

    Few reports are available describing knee changes in neglected developmental dysplasia of the hip (DDH). The purpose of this study was to assess the radiographic morphology of knee joints in adults with neglected DDH. Thirty-seven patients (35 females and two males) with neglected DDH were prospectively recruited with an average age of 32.6 years. Twenty-three patients had unilateral and 14 patients had bilateral neglected DDH. Thirty-seven healthy individuals were recruited to form a matched control group. Three groups of knee joints were examined: affected knees (on the same side of the neglected DDH), unaffected knees (contralateral to the neglected DDH in patients with unilateral involvement), and control knees. A series of radiographic parameters of the knee joint were measured in the coronal and sagittal plane, and they were compared between patients and normal controls. In the coronal plane, the affected knees had increased valgus angulation related to increased height of the medial femoral condyle, decreased height of the lateral femoral condyle and decreased lateral distal femoral angle compared to control knees. In the sagittal plane, both distal femoral and proximal tibial joints of the affected knees developed a decrease in posterior angles. Additionally, the unaffected knees also developed radiographic changes compared to control knees. Patients with neglected DDH may develop changes in both knee joints. These changes should be considered during surgery to the hip, femur and knee to prevent potential complications. Level 2. Copyright © 2014 Elsevier B.V. All rights reserved.

  18. Walking pattern in adults with congenital hip dysplasia: 14 women examined by inverse dynamics

    DEFF Research Database (Denmark)

    Pedersen, Eva Natalia G.; Simonsen, Erik B; Alkjaer, T

    2004-01-01

    Knowledge of the gait dynamics in patients with hip dysplasia may help to understand the consequences of the mechanical changes in the hip.......Knowledge of the gait dynamics in patients with hip dysplasia may help to understand the consequences of the mechanical changes in the hip....

  19. Muscle-tendon-related abnormalities detected by ultrasonography are common in symptomatic hip dysplasia

    DEFF Research Database (Denmark)

    Jacobsen, Julie Sandell; Bolvig, Lars; Hölmich, Per

    2018-01-01

    INTRODUCTION: Hip dysplasia is characterized by reduced acetabular coverage of the femoral head leading to an increased mechanical load on the hip joint and the acting hip muscles. Potentially, the muscles and tendons functioning close to the hip joint may present with overuse......-related ultrasonography findings. The primary aim was to report the prevalence of muscle-tendon-related abnormalities detected by ultrasonography in 100 patients with symptomatic hip dysplasia. The secondary aim was to investigate correlations between muscle-tendon-related abnormalities detected by ultrasonography......-tendon-related abnormalities detected by ultrasonography in the hip and groin region are common in patients with symptomatic hip dysplasia, and the ultrasonography findings of the iliopsoas and gluteus medius/minimus tendons are weakly to moderately correlated to pain related to muscles and tendons in these structures. Both...

  20. Developmental dysplasia of the hip: impact of sonographic newborn hip screening on the outcome of early treated decentered hip joints-a single center retrospective comparative cohort study based on Graf's method of hip ultrasonography.

    Science.gov (United States)

    Tschauner, Christian; Fürntrath, Frank; Saba, Yasaman; Berghold, Andrea; Radl, Roman

    2011-12-01

    PURPOSE/BACKGROUND/INTRODUCTION: The aim of this study was to retrospectively evaluate the impact of neonatal sonographic hip screening using Graf's method for the management and outcome of orthopaedic treatment of decentered hip joints with developmental dysplasia of the hip (DDH), using three decades (1978-2007) of clinical information compiled in a medical database. Three representative cohorts of consecutive cases of decentered hip joints were selected according to different search criteria and inclusion and exclusion parameters: (1) cohort 1 (1978-1982; n = 80), without sonographic screening; (2) cohort 2.1 (1994-1996; n = 91), with nationwide established general sonographic screening according to the Graf-method; (3) cohort 2.2 (2003-2005; n = 91), with sonographic screening including referred cases for open reduction from non-screened populations. These three cohorts were compared for the following parameters: age at initial treatment, successful closed reduction, necessary overhead traction, necessary adductor-tenotomy, rate of open reduction, rate of avascular necrosis (AVN) and rate of secondary acetabuloplasty. The age at initial treatment was reduced from 5.5 months in the first cohort to 2 months in the two subsequent two cohorts and the rate of successful closed reduction increased from 88.7 to 98.9 and 95.6%, respectively. There was a statistically significant improvement in six out of seven parameters with sonographic hip screening; only the rate of secondary acetabuloplasty did not improve significantly. Compared to the era before the institution of a sonographic hip screening programme according to the Graf-method in Austria in 1992, ultrasound screening based-treatment of decentered hip joints has become safer, shorter and simpler: "safer" means lower rate of AVN, "shorter" means less treatment time due to earlier onset and "simpler" means that the devices are now less invasive and highly standardized.

  1. Early Detection of Development Dysplasia of the Hip in the Netherlands: The Validity of a standardized Assessment Protocol in Infants

    NARCIS (Netherlands)

    Boere-Boonekamp, Magdalena M.; Kerkhoff, Antoon; Schuil, Paul B.; Zielhuis, Gerhard A.

    1998-01-01

    The advantages of an early diagnosis of developmental dysplasia of the hip are well established. Early diagnosis means a shorter, less invasive course of treatment with more favorable results and fewer complications. In the Netherlands, the number of neonatally diagnosed cases is very low. Early

  2. Analysis of cause-effect relationship of hip dysplasia in pre-school children

    Directory of Open Access Journals (Sweden)

    Anna Rudenko

    2015-12-01

    Full Text Available Purpose: to analyze and scientifically substantiate peculiarities of cause-effect relationship of hip dysplasia in pre-school children. Material and Methods: analysis and systematization of scientific and methodological literature, medical histories, anamneses, interviews and questionings. Results: it is specified that failure to timely identify and eliminate the symptoms of hip dysplasia in pre-school children leads to negative consequences, namely limited amplitude of hip joint movements; lower limp muscle weakness; valgus and varus deformations of lower limp; increasing of L-lordosis; skewness of hip bones; scoliosis; claudication. Conclusions: the modern state of the problem of hip dysplasia in pre-school children is analyzed. The cause-effect relationship is defined, their mutual transition is projected. All cause-effect relationships are in direct proportion and in constant interaction: the cause the forms effect and the effect influences the cause

  3. Risk factors for avascular necrosis after closed reduction for developmental dysplasia of the hip.

    Science.gov (United States)

    Schur, Mathew D; Lee, Christopher; Arkader, Alexandre; Catalano, Anthony; Choi, Paul D

    2016-06-01

    The purpose of this study was to identify and evaluate risk factors of avascular necrosis (AVN) after closed treatment for developmental dysplasia of the hip (DDH). A retrospective review of children diagnosed with DDH at a tertiary-care children's hospital between 1986 and 2009 was performed. The presence of AVN was assessed according to Salter's classification system. Eighty-two affected hips in 70 children with an average age of 10 months at closed reduction (range 1-31 months) and 5 years (range 2-19 years) of follow-up met the inclusion criteria. Twenty-nine (of 82, 35 %) affected hips developed AVN. The use of pre-reduction traction (p = 0.019) increased the risk of AVN, while preoperative Pavlik harness or brace trial (p = 0.28), presence of ossific nucleus at the time of closed reduction (p = 0.16), and adductor tenotomy (p = 0.37) were not significant factors. Laterality (right vs. left) was also not a significant risk factor (p = 0.75), but patients who underwent closed reduction for bilateral DDH were less likely to develop AVN (p = 0.027). Overall, the degree of abduction did not affect the rate of AVN (p = 0.87). However, in patients treated with closed reduction younger than 6 months of age, the rate of AVN was increased with abduction ≥50° (9/15, 60 %) compared to abduction AVN were more likely to require subsequent surgery (p = 0.034) and more likely to report a fair/poor clinical outcome (p = 0.049). The risk of AVN (35 %) following closed reduction and spica casting for DDH is high. The degree of abduction in spica casts appears to be a risk factor in patients ≤6 months old. The authors recommend that abduction in spica casts should be limited to <50° in children younger than 6 months of age. IV.

  4. Developmental dysplasia of the hip: A computational biomechanical model of the path of least energy for closed reduction.

    Science.gov (United States)

    Zwawi, Mohammed A; Moslehy, Faissal A; Rose, Christopher; Huayamave, Victor; Kassab, Alain J; Divo, Eduardo; Jones, Brendan J; Price, Charles T

    2017-08-01

    This study utilized a computational biomechanical model and applied the least energy path principle to investigate two pathways for closed reduction of high grade infantile hip dislocation. The principle of least energy when applied to moving the femoral head from an initial to a final position considers all possible paths that connect them and identifies the path of least resistance. Clinical reports of severe hip dysplasia have concluded that reduction of the femoral head into the acetabulum may occur by a direct pathway over the posterior rim of the acetabulum when using the Pavlik harness, or by an indirect pathway with reduction through the acetabular notch when using the modified Hoffman-Daimler method. This computational study also compared the energy requirements for both pathways. The anatomical and muscular aspects of the model were derived using a combination of MRI and OpenSim data. Results of this study indicate that the path of least energy closely approximates the indirect pathway of the modified Hoffman-Daimler method. The direct pathway over the posterior rim of the acetabulum required more energy for reduction. This biomechanical analysis confirms the clinical observations of the two pathways for closed reduction of severe hip dysplasia. The path of least energy closely approximated the modified Hoffman-Daimler method. Further study of the modified Hoffman-Daimler method for reduction of severe hip dysplasia may be warranted based on this computational biomechanical analysis. © 2016 The Authors. Journal of Orthopaedic Research Published by Wiley Periodicals, Inc. on behalf of Orthopaedic Research Society. J Orthop Res 35:1799-1805, 2017. © 2016 The Authors. Journal of Orthopaedic Research Published by Wiley Periodicals, Inc. on behalf of Orthopaedic Research Society.

  5. Radiographic signs of acetabular dysplasia of the adult hip

    Energy Technology Data Exchange (ETDEWEB)

    Lingg, G.; von Torklus, D.

    1981-06-01

    Many papers have been published about the different types of measurements used in the evaluation of hip dysplasia in the pediatric age group. However, there are no publications that deal with this topic regarding adults. The authors present an initial series of measurements of the angle of the roof of the acetabulum in 100 randomized asymptomatic adult males. The normal value ranged from -10/sup 0/ to +10/sup 0/. These measurements were correlated with those described by Wiberg. The combination of both methods yields highly reliable information in the evaluation of hip dysplasia.

  6. Diagnosis, prevention, and management of canine hip dysplasia: a review

    Directory of Open Access Journals (Sweden)

    Schachner ER

    2015-05-01

    Full Text Available Emma R Schachner, Mandi J Lopez Department of Veterinary Clinical Sciences, School of Veterinary Medicine, Louisiana State University, Baton Rouge, LA, USA Abstract: Canine hip dysplasia (CHD is a polygenic and multifactorial developmental disorder characterized by coxofemoral (hip joint laxity, degeneration, and osteoarthritis (OA. Current diagnostic techniques are largely subjective measures of joint conformation performed at different stages of development. Recently, measures on three-dimensional images generated from computed tomography scans predicted the development of OA associated with CHD. Continued refinement of similar imaging methods may improve diagnostic imaging techniques to identify dogs predisposed to degenerative hip joint changes. By current consensus, joint changes consistent with CHD are influenced by genetic predisposition as well as environmental and biomechanical factors; however, despite decades of work, the relative contributions of each to the development and extent of CHD signs remain elusive. Similarly, despite considerable effort to decipher the genetic underpinnings of CHD for selective breeding programs, relevant genetic loci remain equivocal. As such, prevention of CHD within domestic canine populations is marginally successful. Conservative management is often employed to manage signs of CHD, with lifelong maintenance of body mass as one of the most promising methods. Surgical intervention is often employed to prevent joint changes or restore joint function, but there are no gold standards for either goal. To date, all CHD phenotypes are considered as a single entity in spite of recognized differences in expression and response to environmental conditions and treatment. Identification of distinct CHD phenotypes and targeting evidence-based conservative and invasive treatments for each may significantly advance prevention and management of a prevalent, debilitating condition in canine companions. Keywords: canine

  7. Total hip reconstruction in acetabular dysplasia.

    NARCIS (Netherlands)

    Schller, H.M.; Dalstra, M.; Huiskes, R.; Marti, R.K.

    1993-01-01

    In acetabular dysplasia, fixation of the acetabular component of a cemented total hip prosthesis may be insecure and superolateral bone grafts are often used to augment the acetabular roof. We used finite element analysis to study the mechanical importance of the lateral acetabular roof and found

  8. Depression and anxiety before and after limb length discrepancy correction in patients with unilateral developmental dysplasia of the hip.

    Science.gov (United States)

    Liu, Ruiyu; Li, Yongwei; Fan, Lihong; Mu, Mingchao; Wang, Kunzheng; Song, Wei

    2015-12-01

    Limb length discrepancy (LLD) is common in patients with developmental dysplasia of the hip (DDH) and may influence the psychological status of these patients. The present study aims to investigate depression and anxiety in DDH patients with different extents of LLD and to assess the effect of LLD correction on these two psychological factors. 161 patients with DDH were recruited and divided into two groups based on whether they could perceive LLD preoperatively. The patients who could not perceive LLD were assigned to group N, and those who could perceive LLD were assigned to group P. Depression/anxiety, Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) scores and LLD were assessed one week before, six months and two years after total hip arthroplasty (THA). Depression and anxiety were significantly higher in group P patients compared to group N patients. The patients in group N presented significant improvement in depression and anxiety six months after arthroplasty, while DDH patients in group P did two years after arthroplasty. Correlation analyses revealed their improvement was associated with pain relief and improved hip function in both groups of patients and was also related to changes in the perception of LLD in group P patients. Depression and anxiety levels were higher in DDH patients with perceived LLD. Their improvement was related to pain relief and improved hip function following THA. In DDH patients with perceived LLD, a change in the perception of LLD also played a part in their improvement. Copyright © 2015 Elsevier Inc. All rights reserved.

  9. Muscle-tendon-related pain in 100 patients with hip dysplasia: prevalence and associations with self-reported hip disability and muscle strength

    DEFF Research Database (Denmark)

    Jacobsen, Julie Sandell; Hølmich, Per; Thorborg, Kristian

    2018-01-01

    The primary aim was to identify muscle-tendon-related pain in 100 patients with hip dysplasia. The secondary aim was to test whether muscle-tendon-related pain is associated with self-reported hip disability and muscle strength in patient with hip dysplasia. One hundred patients (17 men......-tendon-related pain and hip extension a significant inverse linear association between muscle-tendon-related pain and muscle strength was found ranging from -0.11 to - 0.12 Nm/kg in the adjusted analysis (P hip dysplasia with a high prevalence......) with a mean age of 29 years (SD 9) were included. Clinical entity approach was carried out to identify muscle-tendon-related pain. Associations between muscle-tendon-related pain and self-reported hip disability and muscle strength were tested with multiple regression analysis, including adjustments for age...

  10. Preliminary application of computer-assisted patient-specific acetabular navigational template for total hip arthroplasty in adult single development dysplasia of the hip.

    Science.gov (United States)

    Zhang, Yuan Z; Chen, Bin; Lu, Sheng; Yang, Yong; Zhao, Jian M; Liu, Rui; Li, Yan B; Pei, Guo X

    2011-12-01

    The considerable variation in anatomical abnormalities of hip joints associated with different types of developmental dysplasia of hip (DDH) makes reconstruction in total hip arthroplasty (THA) difficult. It is desirable to create patient-specific designs for THA procedures. In the cases of adult single DDH, an accuracy-improved method has been developed for acetabular cup prosthesis implantation of hip arthroplasty. From October 2007 to November 2008, 22 patients with single DDH (according to the Crowe standard, all dysplasia hips were classified as type I) were scanned with spiral CT pre-operatively. These patients scheduled for THA were randomly assigned to undergo either conventional THA (control group, n = 11) or navigation template implantation (NT group, n = 11). In the NT group, three-dimensional (3D) CT pelvis image data were transferred to a computer workstation and 3D models of the hip were reconstructed using the Mimics software. The 3D models were then processed by the Imageware software. In brief, a template that best fitted the location and shape of the acetabular cup was 'reversely' built from the 3D model, the rotation centre of the pathological hip determined by mirroring that of the healthy site, and a guiding hole in the template was then designed. The navigational templates were manufactured using a rapid prototyping machine. These navigation templates guide acetabular component placement. Based on the predetermined abduction angle 45° and anteversion angle 18°, after 1 year follow-up, the NT group showed significantly smaller differences (1.6° ± 0.4°, 1.9° ± 1.1°) from the predetermined angles than those in the control group (5.8° ± 2.9°, 3.9° ± 2.5°) (P < 0.05). The template designs facilitated accurate placement of acetabular components in dysplasia of acetabulum. The hip's center of rotation in DDH could be established using computer-aided design, which provides a useful method for the accurate

  11. Surgical advances in periacetabular osteotomy for treatment of hip dysplasia in adults

    DEFF Research Database (Denmark)

    Troelsen, Anders

    2009-01-01

    Hip dysplasia is characterized by an excessively oblique and shallow acetabulum with insufficient coverage of the femoral head. It is a known cause of pain and the development of early osteoarthritis in young adults. The periacetabular osteotomy is the joint-preserving treatment of choice in young...... adults with symptomatic hip dysplasia. The surgical aim of this extensive procedure is to reorient the acetabulum to improve coverage and eliminate the pathological hip joint mechanics. Intraoperative assessment of the achieved acetabular reorientation is therefore crucial. The "classic" surgical...

  12. Muscle-tendon-related pain in 100 patients with hip dysplasia: prevalence and associations with self-reported hip disability and muscle strength

    DEFF Research Database (Denmark)

    Jacobsen, Julie Sandell; Hölmich, Per; Thorborg, Kristian

    2017-01-01

    The primary aim was to identify muscle-tendon-related pain in 100 patients with hip dysplasia. The secondary aim was to test whether muscle-tendon-related pain is associated with self-reported hip disability and muscle strength in patient with hip dysplasia. One hundred patients (17 men......-tendon-related pain and hip extension a significant inverse linear association between muscletendon- related pain and muscle strength was found ranging from 0.11 to0.12 Nm/kg in the adjusted analysis (Phip dysplasia with a high prevalence......) with a mean age of 29 years (SD 9) were included. Clinical entity approach was carried out to identify muscle-tendon-related pain. Associations between muscle-tendon-related pain and self-reported hip disability and muscle strength were tested with multiple regression analysis, including adjustments for age...

  13. Periacetabular Osteotomy in patients with Hip Dysplasia investigated with Imaging Modalities

    DEFF Research Database (Denmark)

    Mechlenburg, Inger

    2016-01-01

    , cartilage and blood perfusion after PAO in patients with hip dysplasia. Furthermore, to investigate the relationship between the acetabular angles and health-related quality of life (QoL) after PAO. And finally, to study the level of radiation to the surgeon during PAO. Chapters 3 to 7 investigate the first......The minimal invasive periacetabular osteotomy (PAO) is a joint-preserving procedure that effectively corrects hip dysplasia, provides pain relief, improved radiographic results and a low rate of complications. The aim of this doctoral dissertation was to examine biological changes in bone...... is applied on 26 patients scheduled for PAO. In chapter 4, a cohort of patients with hip dysplasia are followed with Dual-energy X-ray absorptiometry (DXA) prior to and 1 and 2½ years after PAO to investigate changes in acetabular bone mineral density after PAO. Moreover, to examine whether bone mineral...

  14. Muscle-tendon related pain in 100 patients with hip dysplasia: prevalence and associations with self-reported hip disability and muscle strength

    DEFF Research Database (Denmark)

    Jacobsen, Julie Sandell

    2017-01-01

    dysplasia. The secondary aim was to test if muscle-tendon related pain is linearly associated to self-reported hip disability and muscle strength in patients with hip dysplasia. Materials and methods One hundred patients (17 men) with a mean age of 29+9 years were included. Clinical entity approach...

  15. STANDARDISED CLINICAL EXAMINATION OF SOFT-TISSUE PAIN IN PATIENTS WITH HIP DYSPLASIA USING THE CLINICAL ENTITIES APPROACH

    DEFF Research Database (Denmark)

    Jacobsen, Julie Sandell; Hölmich, Per; Thorborg, Kristian

    2016-01-01

    Introduction In patients with symptomatic hip dysplasia soft-tissue pain may be a prevalent condition that might affect the outcome of periacetabular osteotomy (PAO). However, the distribution of soft-tissue pain in hip dysplasia has never been examined systematically using a standardised...... and reliable protocol. The aim of this study was to investigate five clinical entities in 100 patients with hip dysplasia using the clinical entities approach identifying the anatomic location of soft-tissue pain. The first 50 patients are presented in this paper. Material and Methods Fifty patients (10 males...... prevalence in the iliopsoas and the hip abductors. This indicates that patients with hip dysplasia also experience pain related to the surrounding soft-tissues, and not only from the hip joint. References (1) Holmich P, Holmich LR, Bjerg AM. Clinical examination of athletes with groin pain: an intraobserver...

  16. Evaluation of Medial Acetabular Wall Bone Stock in Patients with Developmental Dysplasia of the Hip Using a Helical Computed Tomography Multiplanar Reconstruction Technique

    Energy Technology Data Exchange (ETDEWEB)

    Rui Yu Liu; Kun Zheng Wang; Chun Sheng Wang; Xiao Qian Dang; Zhi Qin Tong (Second Hospital Affiliated to the Medical College of Xi' an Jiaotong Univ., Xi' an Shaanxi (China))

    2009-08-15

    Background: The technique of medialization has been used to reconstruct acetabula at the level of true acetabula in total hip arthroplasty (THA) in patients with developmental dysplasia of the hip (DDH). Appreciation of the bone stock in the medial acetabular wall is significant for making an optimal acetabular reconstruction plan and avoiding complications. Purpose: To evaluate the bone stock of the medial acetabular wall and its relation to the degree of subluxation in patients with DDH using computed tomography (CT). Material and Methods: Helical CT scans of 27 hips were obtained from 21 patients with osteoarthritis secondary to DDH who were scheduled for total hip arthroplasty. Eleven hips belonged to Crowe class I, while 16 hips belonged to Crowe class II/III. The raw CT data were reprocessed in various planes by scrolling multiplanar reformation (MPR). Acetabular opening, depth, and medial bone stock, as indicated by the minimum thickness of the medial acetabular wall, were measured in the transverse reformed MPR plane. Results: The minimum thicknesses of the medial acetabular wall in Crowe-I and Crowe-II/III hips were 3.8+-2.1 mm and 7.1+-3.1 mm, respectively, with statistically significant differences between the groups (P<0.05). Furthermore, the bone stock in the medial acetabular wall correlated with the degree of subluxation (R=0.69) and the acetabular depth (R= ;- ;0.71). Conclusion: There was significantly more bone stock in the medial acetabular wall in patients with higher-degree subluxation than there was in the less-severe class. This difference should be taken into consideration when reconstructing acetabula in THA in patients with DDH using the technique of medialization

  17. Evaluation of Medial Acetabular Wall Bone Stock in Patients with Developmental Dysplasia of the Hip Using a Helical Computed Tomography Multiplanar Reconstruction Technique

    International Nuclear Information System (INIS)

    Rui Yu Liu; Kun Zheng Wang; Chun Sheng Wang; Xiao Qian Dang; Zhi Qin Tong

    2009-01-01

    Background: The technique of medialization has been used to reconstruct acetabula at the level of true acetabula in total hip arthroplasty (THA) in patients with developmental dysplasia of the hip (DDH). Appreciation of the bone stock in the medial acetabular wall is significant for making an optimal acetabular reconstruction plan and avoiding complications. Purpose: To evaluate the bone stock of the medial acetabular wall and its relation to the degree of subluxation in patients with DDH using computed tomography (CT). Material and Methods: Helical CT scans of 27 hips were obtained from 21 patients with osteoarthritis secondary to DDH who were scheduled for total hip arthroplasty. Eleven hips belonged to Crowe class I, while 16 hips belonged to Crowe class II/III. The raw CT data were reprocessed in various planes by scrolling multiplanar reformation (MPR). Acetabular opening, depth, and medial bone stock, as indicated by the minimum thickness of the medial acetabular wall, were measured in the transverse reformed MPR plane. Results: The minimum thicknesses of the medial acetabular wall in Crowe-I and Crowe-II/III hips were 3.8±2.1 mm and 7.1±3.1 mm, respectively, with statistically significant differences between the groups (P<0.05). Furthermore, the bone stock in the medial acetabular wall correlated with the degree of subluxation (R=0.69) and the acetabular depth (R= ;- ;0.71). Conclusion: There was significantly more bone stock in the medial acetabular wall in patients with higher-degree subluxation than there was in the less-severe class. This difference should be taken into consideration when reconstructing acetabula in THA in patients with DDH using the technique of medialization

  18. Radiographic assessment of developmental dysplasia of the hip – A novel radiology reporting process and one year review of referrals

    International Nuclear Information System (INIS)

    Snaith, Beverly; Buckley, Kirsty

    2013-01-01

    Introduction: Developmental dysplasia of the hip (DDH) is the most common musculoskeletal disorder of childhood. Despite the introduction of ultrasound screening radiography remains the most common imaging investigation for children over 4–6 months. The use of picture archive and communications system (PACS) tools to review key measures is standard but annotation of these images supplemented by a radiology report has not been described previously. Method: Referral and demographic data were identified of all children under the age of 2 years attending for a non-trauma pelvic radiograph within a single NHS Trust between January and December 2012. Retrospective review of all radiographs and reports identified the clinical history, co-morbidities and outcome. Results: A total of 313 referrals were received, 230 initial and 83 follow-up examinations. 37 examinations identified a dysplastic acetabulum (n = 37/230; 16.1%) with the majority being girls (n = 23/37; 62.1%). A total of 25 abnormal findings in relation to the femoral head epiphyses were identified (n = 25/230; 10.9%) with 12 also having acetabular dysplasia. Children with a dysplastic acetabulum were significantly more likely to have a subluxation or dislocation (X 2 183.78, 2df, p ≤ 0.001). Children with acetabular dysplasia were significantly more likely to have been born in the autumn or winter months (proportional difference = 11.0%; 95% CI [0.019,0.202]; p = 0.024). Conclusion: This article presents a novel reporting process which is helping to standardise the results in young children undergoing investigation for DDH. The review of referrals confirmed the published evidence that DDH is more prevalent in girls and children born in the autumn and winter months

  19. Lumbar nerve root avulsions with secondary ipsilateral hip dysplasia in a child

    Energy Technology Data Exchange (ETDEWEB)

    Polyzoidis, Konstandinos; Vranos, Georgios [Department of Neurosurgery, Medical School, University of Ioannina, 45110, Ioannina (Greece); Petropoulou, Calliope; Argyropoulou, Paraskevi I.; Argyropoulou, Maria I. [Department of Radiology, Medical School, University of Ioannina, 45110, Ioannina (Greece); Sarmas, Ioannis [Department of Neurology, Medical School, University of Ioannina, 45110, Ioannina (Greece)

    2002-09-01

    We report on an 8-year-old child with avulsions of the left L3, L4 and L5 nerve roots and traumatic meningoceles that were not associated with lumbar spine or pelvic girdle fractures. The patient had a history of a road traffic accident. Plain radiographs of the pelvis revealed left hip dysplasia. The magnetic resonance imaging findings of the lumbar spine are illustrated. The pathogenesis of lumbar nerve root avulsions and their association with ipsilateral hip dysplasia are discussed. (orig.)

  20. Lumbar nerve root avulsions with secondary ipsilateral hip dysplasia in a child

    International Nuclear Information System (INIS)

    Polyzoidis, Konstandinos; Vranos, Georgios; Petropoulou, Calliope; Argyropoulou, Paraskevi I.; Argyropoulou, Maria I.; Sarmas, Ioannis

    2002-01-01

    We report on an 8-year-old child with avulsions of the left L3, L4 and L5 nerve roots and traumatic meningoceles that were not associated with lumbar spine or pelvic girdle fractures. The patient had a history of a road traffic accident. Plain radiographs of the pelvis revealed left hip dysplasia. The magnetic resonance imaging findings of the lumbar spine are illustrated. The pathogenesis of lumbar nerve root avulsions and their association with ipsilateral hip dysplasia are discussed. (orig.)

  1. Reliability of a New Radiographic Classification for Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Narayanan, Unni; Mulpuri, Kishore; Sankar, Wudbhav N; Clarke, Nicholas M P; Hosalkar, Harish; Price, Charles T

    2015-01-01

    Existing radiographic classification schemes (eg, Tönnis criteria) for DDH quantify the severity of disease based on the position of the ossific nucleus relative to Hilgenreiner's and Perkin's lines. By definition, this method requires the presence of an ossification centre, which can be delayed in appearance and eccentric in location within the femoral head. A new radiographic classification system has been developed by the International Hip Dysplasia Institute (IHDI), which uses the mid-point of the proximal femoral metaphysis as a reference landmark, and can therefore be applied to children of all ages. The purpose of this study was to compare the reliability of this new method with that of Tönnis, as the first step in establishing its validity and clinical utility. Twenty standardized anteroposterior pelvic radiographs of children with untreated DDH were selected purposefully to capture the spectrum of age (range, 3 to 32 mo) at presentation and disease severity. Each of the hips was classified separately by the IHDI and Tönnis methods by 6 experienced pediatric orthopaedists from the United States, Canada, Mexico, United Kingdom, and by 2 orthopaedic senior residents. The inter-rater reliability was tested using the Intra Class Correlation coefficient (ICC) to measure concordance between raters. All 40 hips were classifiable by the IHDI method by all raters. Ten of the 40 hips could not be classified by the Tönnis method because of the absence of the ossific nucleus on one or both sides. The ICC (95% confidence interval) for the IHDI method for all raters was 0.90 (0.83-0.95) and 0.95 (0.91-0.98) for the right and left hips, respectively. The corresponding ICCs for the Tönnis method were 0.63 (0.46-0.80) and 0.60 (0.43-0.78), respectively. There was no significant difference between the ICCs of the 6 experts and 2 trainees. The IHDI method of classification has excellent inter-rater reliability, both among experts and novices, and is more widely

  2. Differentiating subluxation from developmental dislocation of the hip

    Directory of Open Access Journals (Sweden)

    Joao O. Tavares

    2012-02-01

    Full Text Available The radiological and clinical picture of a developmental hip dislocation and a severe subluxation are identical. According to Leveuf and Wiberg the diagnosis can only be made by arthrography. The differential diagnosis is critical, as treatment differs dependent on the diagnosis. In this study, the diagnosis of subluxation was based on a plain radiograph of the pelvis. A radiograph of the pelvis with the hips abducted at least 45° and internally rotated (AIR view was used to differentiate these two entities. In subluxations, the femoral head will relocate into the acetabulum with perfect or near perfect reconstitution of the Shenton’s line. It will fail to do so in true dislocations. Five patients, mean age 14.6 months (range 9 to 20 months, presented with delayed diagnosis of hip dysplasia. The examination revealed minimal or no limitation of hip abduction, a leg length discrepancy, and a Trendelenburg gait in the three walking age girls. The radiograph suggested a hip dislocation. The diagnosis of hip subluxation was based on the relocation of the femoral head with the abduction/internal rotation radiograph. All were successfully treated with an Ilfeld abduction splint. None had examination with general anesthesia, arthrograms, traction or immobilization in spica cast. Avoiding over diagnosis of hip dislocation in cases of subluxation is important. This is necessary to prevent overtreatment and to accurately assess the results of treatment. The abduction/internal rotation view may achieve this goal while avoiding diagnostic and therapeutic procedures, such as arthrograms, cast immobilization and surgery.

  3. Procollagen type-III aminoterminal peptide in serum and synovial fluid of dogs with hip dysplasia and coxarthrosis

    DEFF Research Database (Denmark)

    Madsen, J S; Jensen, L T; Strøm, H

    1990-01-01

    Hip dysplasia is an affection of the coxofemoral joint that progresses until stabilization is caused by fibrosis and osteoarthritic changes. This stabilization process can be examined by clinical and radiographic methods. The capability of evaluating the procollagen concentrations in liquids......-III-NP) concentration was measured in serum and in synovial fluid from coxofemoral joints in 20 dogs. Dogs were grouped on the basis of evidence of dysplasia and osteoarthritic changes of the hip: (1) a control group of 6 dogs without clinical or radiographic signs of hip dysplasia, and (2) dysplastic group of 14 dogs...

  4. Genetic variances, trends and mode of inheritance for hip and elbow dysplasia in Finnish dog populations

    NARCIS (Netherlands)

    Mäki, K.; Groen, A.F.; Liinamo, A.E.; Ojala, M.

    2002-01-01

    The aims of this study were to assess genetic variances, trends and mode of inheritance for hip and elbow dysplasia in Finnish dog populations. The influence of time-dependent fixed effects in the model when estimating the genetic trends was also studied. Official hip and elbow dysplasia screening

  5. Adult hip dysplasia and osteoarthritis. Studies in radiology and clinical epidemiology

    DEFF Research Database (Denmark)

    Jacobsen, Steffen

    2006-01-01

    studies. To develop a comprehensible and reproducible radiographic discriminator of hip OA with as close an association to self reported hip pain as possible. To identify prevalences of hip OA and HD in a Caucasian, urban background population and investigate the influence of sex, age, physical...... to be a significant risk factor for hip OA in men. However, only obesity was found to determine an event of hip replacement surgery. In a longitudinal study of 81 subjects and with mild or moderate hip dysplasia followed for a decade we did not document a tendency for radiological degeneration compared to 136 control...

  6. Evaluation of late redislocation in patients who underwent open reduction and pelvic osteotomy as treament for developmental dysplasia of the hip.

    Science.gov (United States)

    Tuhanioğlu, Ümit; Cicek, Hakan; Ogur, Hasan U; Seyfettinoglu, Firat; Kapukaya, Ahmet

    2017-10-16

    The goal in the treatment of developmental dysplasia of the hip (DDH) is to achieve a stable and concentric reduction and to create a congruent relationship between the femoral head and the acetabulum. This study discusses the causes of loss of reduction in DDH patients who had a concentrically reduced hip at the time of removal of the hip spica cast and cessation of brace use and who later appeared with hip redislocation after mobilisation and ambulation. In addition, the possible interventions in such cases are also discussed. A retrospective evaluation was made of 13 patients diagnosed with DDH who developed redislocation following primary surgery. 6 of them had undergone the 1st surgery in our department between 2008 and 2016 and 7 had udergone surgery in another centre. For comparison reasons a 2nd group was formed of 13 demographically and clinically matched patients who had no loss of reduction. The groups were compared in terms of acetabular index, pelvic length, pelvic width, abduction degree of plaster, ossifying nucleus diameter, acetabular depth, and acetabular volume parameters. The average age of the patients was 23 months at initial surgery and 29 months at the time of revision surgery. No significant difference was found between the groups in terms of acetabular inclination angle, ossifying nucleus diameter, pelvic size, pelvic width, centre edge angle, acetabular volume, and depth. Contracted inferomedial capsule was found in 1 patient who underwent revision surgery and intact transverse acetabular ligament was seen in 1 patient. The loss of reduction in the remaining 11 patients was associated with high total anteversion of the femoral head and acetabulum. Correction of increased combined anteversion by femoral osteotomy can create a safe zone in terms of redislocation and can significantly contribute to the stability provided by capsulorrhaphy and pelvic osteotomy.

  7. Hip dysplasia and osteoarthrosis: a survey of 4151 subjects from the Osteoarthrosis Substudy of the Copenhagen City Heart Study

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig; Søballe, K

    2005-01-01

    Acta Orthop. 2005 Apr;76(2):149-58. Related Articles, Links Hip dysplasia and osteoarthrosis: a survey of 4151 subjects from the Osteoarthrosis Substudy of the Copenhagen City Heart Study. Jacobsen S, Sonne-Holm S, Soballe K, Gebuhr P, Lund B. Department of Orthopaedic Surgery, Copenhagen...... University Hospital, Rigshospitalet, Denmark. sjac@dadlnet.dk INTRODUCTION: Hip dysplasia (HD) is assumed to be an etiological factor in the development of premature hip osteoarthrosis (OA). We established the prevalences of HD and OA in adults according to qualified radiographic discriminators.......6 degrees). Applying a CE cut-off value of 20 degrees for designation of definite hip dysplasia, we found a prevalence of hip dysplasia of 3.4%. Approximately 2% of cases were unilateral and 1.4% of cases were bilateral. We found significant relationships between radiographic OA discriminators and the CE...

  8. Pelvic proportions in x-ray pictures of german shepherd dog, rottweiler and great dane, a contribution to hip dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Koeppel, E. (Veterinaermedizinische Univ., Vienna (Austria))

    1983-01-01

    Measurements of length and breadth were done in 439 X-ray pictures of the pelvis of German Shepherd Dog, Rottweiler and Great Dane. The relation of pelvic proportions to hip dysplasia was checked. In 66 X-ray pictures of the pelvis of young dogs the ischiadic part of the acetabulum was measured. In all breeds the ischium formed 68,8 percent of the acetabulum. Ilium and ischium showed changes in relation to hip dysplasia, which could be proved unequivocally only in Rottweilers. Changes of pelvic proportions in other breeds were not perceived. In Rottweilers also those changes can be seen distinctly only in severe cases of hip dysplasia. For the special case valid accordance with any degree of hip dysplasia could not be found.

  9. [Developmental dysplasia of the hip in children with a psychomotor disorder. A risk factor for a poor outcome?].

    Science.gov (United States)

    Pipa-Muñiz, Iván; de Los Llanos Rodríguez-Rodríguez, María; Felgueroso-Juliana, M Blanca; Riera-Campillo, Manuela; González-Herranz, Pedro

    2016-09-01

    Orthopaedic treatment of developmental dysplasia of the hip (DDH) has a high success rate in cases that are diagnosed early. However, the outcomes of these patients are not really known when they are subsequently diagnosed with some type of cerebral impairment. A retrospective observational study was conducted on cases of DDH with a poor outcome after orthopaedic treatment, being unknown if they had any type of psychomotor disorder. The patients were clinically and radiologically assessed, and afterwards received neurological valuation by the Child Neurology Unit. Of the 325 cases of DDH diagnosed in 293 patients, 10 patients (3%) with 16 hips with DDH were diagnosed of any cerebral impairment. All them were initially treated orthopedically. Clinical and radiologically evolution was succesful only in 4 cases (25%) being necessary any surgical procedure in the remaining 12 cases. After surgical treatment we got an improvement in the Acetabular Index (p=0.005) and Reimers Extrusion Index (p=0.042). Neck-shaft angle and Wiberg CE angle also improved but this difference was not statically significant. Cerebral impairment was diagnosed at 2,5 years of age and the begining of walking was delayed at 2.4 years of age. Cerebral impairment can lead to an unfavourable outcome in the treatment of DDH, with the relative risk of a poor outcome being 7.2 times higher in these patients. An unfavourable outcome with conventional treatment of DDH must make us suspect the presence of some type of neurological disorder, particularly if there is a delay in walking. Copyright © 2015 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  10. Gonadal dosage during hip dysplasia radiography in the dog.

    Science.gov (United States)

    Wood, A K; Reynolds, K M; Leith, I S; Burns, P A

    1977-01-01

    Thermoluminescent dosemeters were used to estimate gonadal dosage during hip dysplasia radiography of labrador retriever dogs. The mean radiation dose to the unshielded testes was 100 millirad (mrad) and the estimated dose to the shielded testes was 9 mrad. It was considered unnecessary to shield the ovaries.

  11. Monitoring Hip and Elbow Dysplasia achieved modest genetic improvement of 74 dog breeds over 40 years in USA.

    Directory of Open Access Journals (Sweden)

    Yali Hou

    Full Text Available Hip (HD and Elbow Dysplasia (ED are two common complex developmental disorders of dogs. In order to decrease their prevalence and severity, the Orthopedic Foundation for Animals (OFA has a voluntary registry of canine hip and elbow conformation certified by boarded radiologists. However, the voluntarily reports have been severely biased against exposing dogs with problems, especially at beginning period. Fluctuated by additional influential factors such as age, the published raw scores barely showed trends of improvement. In this study, we used multiple-trait mixed model to simultaneously adjust these factors and incorporate pedigree to derive Estimated Breeding Values (EBV. A total of 1,264,422 dogs from 74 breeds were evaluated for EBVs from 760,455 hip scores and 135,409 elbow scores. These EBVs have substantially recovered the reporting bias and the other influences. Clear and steady trends of genetic improvement were observed over the 40 years since 1970. The total genetic improvements were 16.4% and 1.1% of the phenotypic standard deviation for HD and ED, respectively. The incidences of dysplasia were 0.83% and 2.08%, and the heritabilities were estimated as 0.22 and 0.17 for hip and elbow scores, respectively. The genetic correlation between them was 0.12. We conclude that EBV is more effective than reporting raw phenotype. The weak genetic correlation suggested that selection based on hip scores would also slightly improve elbow scores but it is necessary to allocate effort toward improvement of elbow scores alone.

  12. Avascular necrosis of the hip in multiple epiphyseal dysplasia

    International Nuclear Information System (INIS)

    Mackenzie, W.G.; Bassett, G.S.; Mandell, G.A.; Scott, C.I. Jr.

    1989-01-01

    We observed radiographic changes of avascular necrosis (AVN) of the capital femoral epiphysis in 9 hips of 11 patients with multiple epiphyseal dysplasia (MED). Plain roentgenography, bone scintigraphy, and magnetic resonance imaging (MRI) studies all revealed characteristic asymmetric changes in the presence of AVN superimposed on dysplastic femoral heads

  13. Hip dysplasia in the cat: a report of three cases

    International Nuclear Information System (INIS)

    Patsikas, M.N.; Papazoglou, L.G.; Komninou, A.; Dessiris, A.K.; Tsimopoulos, G.

    1998-01-01

    Hip dysplasia was diagnosed in three cats. Two were presented with a history of hindlimb lameness and the other had a history of constipation. All were confined for two weeks and showed considerable clinical improvement. At follow-up examination the cats were free of clinical signs despite the deterioration in the radiological appearance of their hips. Luxation or subluxation of the hips, insufficient development of the craniolateral acetabular edges, loss of the arched shape of the cranial subchondral acetabular bones, shallow acetabula and secondary degenerative changes on the femoral heads and necks were the main radiological findings in the affected cats

  14. Percutaneous medial hemi-epiphysiodesis using a transphyseal screw for caput valgum associated with developmental dysplasia of the hip.

    Science.gov (United States)

    Shin, Chang Ho; Hong, Wan Kee; Lee, Doo Jae; Yoo, Won Joon; Choi, In Ho; Cho, Tae-Joon

    2017-11-14

    The purpose of this study was to evaluate the radiologic outcome of percutaneous medial hemi-epiphysiodesis using a transphyseal screw for the management of caput valgum associated with developmental dysplasia of the hip (DDH). Eighteen hips (18 patients) having caput valgum treated with screw hemi-epiphysiodesis were followed for more than 2 years, and were included in this study. The mean age at the time of the index operation was 8.3 years (range, 4.3 to 10.7 years) and age at the latest follow-up was 12.2 years (range, 9.4 to 16.4 years). The screw in 5 hips was changed into a longer one at postoperative 21.8 months (range, 14 to 29 months) because the proximal femur outgrew the screw. The screws in 11 hips were removed at the mean age of 10.9 years (range, 8.0 to 14.5 years). We retrospectively analyzed the change in various radiologic parameters over time. The mean Hilgenreiner-epiphyseal angle (HEA) of the operated side was 5.1 ± 11.3° preoperatively, and increased to 20.6 ± 11.3° at the latest follow-up (p = 0.001). The mean difference of the HEA between the operated and contralateral sides was 16.9 ± 15.1° preoperatively, which decreased to 2.4 ± 12.4° at the latest follow-up (p = 0.008). The mean articulo-trochanteric distance of the operated side, which was 3.2 ± 5.5 mm longer than that of the contralateral side preoperatively, became 5.6 ± 9.1 mm shorter at the latest follow-up (p = 0.001). The ratio of femoral neck length of the operated side to that of the contralateral side decreased over the follow-up period. Acetabular shape as measured by the Sharp angle and acetabular roof angle and femoral head coverage as measured by lateral center-edge angle did not change significantly by the index operation. The ratio of medial joint space width of the operated side to that of the contralateral side did not change significantly. Screw medial hemi-epiphysiodesis can effectively correct caput valgum

  15. Total hip reconstruction in acetabular dysplasia : a finite element study

    NARCIS (Netherlands)

    Schüller, H.M.; Dalstra, M.; Huiskes, H.W.J.; Marti, R.K.

    1993-01-01

    In acetabular dysplasia, fixation of the acetabular component of a cemented total hip prosthesis may be insecure and superolateral bone grafts are often used to augment the acetabular roof. We used finite element analysis to study the mechanical importance of the lateral acetabular roof and found

  16. Risk of developmental dysplasia of the hip in breech presentation: the effect of successful external cephalic version.

    Science.gov (United States)

    Lambeek, A F; De Hundt, M; Vlemmix, F; Akerboom, B M C; Bais, J M J; Papatsonis, D N M; Mol, B W J; Kok, M

    2013-04-01

    To evaluate the effect of successful external cephalic version on the incidence of developmental dysplasia of the hip (DDH) requiring treatment in singleton breech presentation at term. Observational cohort study. Three large teaching hospitals in the Netherlands. Women with a singleton breech presentation of 34 weeks of gestation or more, who underwent an external cephalic version attempt. We made a comparison of the incidence of DDH between children born in breech presentation and children born in cephalic presentation after a successful external cephalic version. The incidence of DDH requiring either conservative treatment, with a harness, or surgical treatment. A total of 498 newborns were included in the study, of which 40 (8%) were diagnosed with DDH and 35 required treatment. Multivariate analysis showed that female gender (OR 2.79, 95% CI 1.23-6.35) and successful external cephalic version (OR 0.29, 95% CI 0.09-0.95) were independently associated with DDH. A successful external cephalic version is associated with a lower incidence of DDH, although a high percentage of children born after a successful external cephalic version still appear to have DDH. A larger cohort study is needed to establish the definite nature of this relationship. Until then, we recommend the same screening policy for infants born in cephalic position after a successful external cephalic version as for infants born in breech position. © 2012 The Authors BJOG An International Journal of Obstetrics and Gynaecology © 2012 RCOG.

  17. Cam deformity and acetabular dysplasia as risk factors for hip osteoarthritis

    NARCIS (Netherlands)

    Hosnijeh, Fatemeh Saberi; Zuiderwijk, Maria E; Versteeg, Mathijs; Smeele, Hieronymus T W; Hofman, Albert; Uitterlinden, André G; Agricola, Rintje; Oei, Edwin H G; Waarsing, Jan H; Bierma-Zeinstra, Sita M; van Meurs, Joyce B J

    2017-01-01

    Objective: Cam deformity and acetabular dysplasia have been recognized as relevant risk factors for hip osteoarthritis (OA) in a few prospective studies with limited sample sizes. To date, however, no evidence is available from prospective studies regarding whether the magnitude of these

  18. Ilfeld abduction orthosis is an effective second-line treatment after failure of Pavlik harness for infants with developmental dysplasia of the hip.

    Science.gov (United States)

    Sankar, Wudbhav N; Nduaguba, Afamefuna; Flynn, John M

    2015-02-18

    Closed reduction and spica casting is the most commonly recommended choice for infants with developmental dysplasia of the hip (DDH) for whom Pavlik harness treatment has failed, but it requires general anesthesia in addition to the challenges of spica cast care. The purposes of this study were to evaluate the effectiveness of Ilfeld bracing for infants for whom Pavlik harness treatment is unsuccessful and to compare these results with those for a similar cohort of patients directly undergoing closed reduction and spica casting. We reviewed the cases of a consecutive series of children with DDH who had failure of Pavlik harness treatment and were subsequently managed with Ilfeld bracing (the BR cohort) and compared this cohort with a similar historical group of infants who had failure of Pavlik harness treatment but had standard closed reduction and spica casting (the CR cohort). The cohorts were compared with respect to clinical and ultrasonographic data at the time of Pavlik discontinuation. At one year, the hip stability and acetabular index were assessed; the presence of osteonecrosis was graded according to the criteria described by Salter et al. Twenty-eight hips (nineteen infants) made up the BR cohort and twenty-two hips (sixteen infants) made up the CR cohort. Ultrasonographic indices (including the alpha angle and the percentage of femoral head coverage) were comparable between the two cohorts (p=0.66 and 0.19, respectively). Following treatment, a stable reduction was achieved in twenty-three (82%) of twenty-eight hips in the BR cohort compared with twenty (91%) of twenty-two hips in the CR cohort. At one year, acetabular indices were similar between both cohorts (mean and standard deviation, 27°±6° for the BR cohort versus 27°±5° for the CR cohort; p=0.62); however, osteonecrosis developed in three hips in the CR cohort compared with none in the BR cohort. In our series of infants with DDH for whom Pavlik harness treatment had failed, Ilfeld

  19. Evaluation of shelf arthroplasty as a treatment for hip dysplasia in dogs.

    Science.gov (United States)

    Oakes, M G; Lewis, D D; Elkins, A D; Hosgood, G; Dial, S M; Oliver, J

    1996-06-01

    To evaluate effects of shelf arthroplasty on coxofemoral joint laxity and progression of degenerative joint disease in young dogs with hip dysplasia. Prospective, controlled study. 10 dogs between 10 and 24 months old and weighing between 20 and 27 kg. All dogs had bilateral coxofemoral joint laxity (i.e., an Ortolani's sign). In all dogs, shelf arthroplasty was performed on the right coxofemoral joints, and a sham procedure was performed on the left. Dogs were evaluated before and after surgery by means of lameness assessment, coxofemoral joint palpation and goniometry, thigh circumference measurement, and radiography. There were no significant changes in coxofemoral joint mobility, range of motion, joint laxity, degree of degenerative joint disease, or thigh circumference during the study. A greater amount of periacetabular bone formed on the right side than on the left side; however, dogs did not develop large bony shelves, and the amount of periarticular bone decreased over time. The polymer implants remained in their original position and were encapsulated by fibrous tissue. There was no histologic evidence of osteoconduction by the implants. The polymer implants used in this procedure do not appear to be osteoconductive. Shelf arthroplasty was associated with minimal morbidity and was not associated with serious adverse sequelae in this study, but the procedure did not alter the progression of hip dysplasia in these dogs. We cannot advocate shelf arthroplasty using this polymer as a treatment for dogs with hip dysplasia.

  20. The Fate of the Iliopsoas Muscle in Long-term Follow-up After Open Reduction With a Medial Approach in Developmental Dysplasia of the Hip. Part 2: Isokinetic Muscle Strength Evaluation.

    Science.gov (United States)

    Yilmaz, Serdar; Aksahin, Ertugrul; Ersoz, Murat; Bicimoglu, Ali

    2017-09-01

    The impact on long-term weakness of hip flexion of complete iliopsoas tenotomy during open reduction of developmental hip dysplasia with a medial approach has not yet been fully clarified. The purpose of this study was to investigate the isokinetic muscle strength (IMS) of hip flexor and extensor muscles in these patients and also to analyze the effect of spontaneous reattachment of the iliopsoas muscle on IMS measurements. The study included 20 patients. Earlier magnetic resonance imaging examination of all the patients revealed spontaneous reattachment of the iliopsoas in 18 (90%) patients. IMS measurements were performed at 60 and 150 degrees/s. The peak torque, total work (TW), average power (AP), work fatigue, and agonist to antagonist muscle ratio of the operated and nonoperated hips were recorded separately for flexors and extensors. The effect of iliopsoas reattachment on IMS was also evaluated. The mean follow-up period was 16.65±2.16 (13 to 20) years. Total work (P=0.013) and average power (P=0.009) of the flexor muscles and work fatigue of the extensor muscles (P=0.030) of the operated hip were significantly decreased when compared with the nonoperated hips at 150 degrees/s. There was no significant difference between the flexor muscles of the operated and nonoperated hips (Phips. Flexor muscle strength was decreased in the operated hip against low resistance in long-term follow-up after iliopsoas tenotomy. This may reflect that hip muscle strength was decreased after prolonged activities such as sports. However, in forceful activities flexor muscle strength was retained due to iliopsoas reattachment. On the basis of this study we thought that spontaneous reattachment of the iliopsoas tendon substantially preserves muscle strength. Nonetheless possible efforts should be made to surgically reattach the psoas tendon to preserve strength of the muscle. Therapeutic level IV.

  1. Standard Hip Ventrodorsal Leg Extended View In The Diagnosis Of ...

    African Journals Online (AJOL)

    Canine hip dysplasia (CHD) is hereditary developmental condition that involves a lack of conformity between the femoral head and acetabulum. It invariably leads to osteoarthritis. We hereby review the standard hip ventrodorsal leg extended view to be adopted by our Tanzanian veterinarians. Diagnostic radiography is the ...

  2. Joint Genomic Prediction of Canine Hip Dysplasia in UK and US Labrador Retrievers

    Directory of Open Access Journals (Sweden)

    Stefan M. Edwards

    2018-03-01

    Full Text Available Canine hip dysplasia, a debilitating orthopedic disorder that leads to osteoarthritis and cartilage degeneration, is common in several large-sized dog breeds and shows moderate heritability suggesting that selection can reduce prevalence. Estimating genomic breeding values require large reference populations, which are expensive to genotype for development of genomic prediction tools. Combining datasets from different countries could be an option to help build larger reference datasets without incurring extra genotyping costs. Our objective was to evaluate genomic prediction based on a combination of UK and US datasets of genotyped dogs with records of Norberg angle scores, related to canine hip dysplasia. Prediction accuracies using a single population were 0.179 and 0.290 for 1,179 and 242 UK and US Labrador Retrievers, respectively. Prediction accuracies changed to 0.189 and 0.260, with an increased bias of genomic breeding values when using a joint training set (biased upwards for the US population and downwards for the UK population. Our results show that in this study of canine hip dysplasia, little or no benefit was gained from using a joint training set as compared to using a single population as training set. We attribute this to differences in the genetic background of the two populations as well as the small sample size of the US dataset.

  3. Walking pattern in 9 women with hip dysplasia 18 months after periacetabular osteotomy

    DEFF Research Database (Denmark)

    Pedersen, Eva N G; Alkjaer, Tine; Søballe, Kjeld

    2006-01-01

    . METHODS: The gait pattern of 9 women (median age 39 years) with hip dysplasia who were treated with unilateral periacetabular osteotomy was analyzed before periacetabular osteotomy and an average of 1.5 years afterwards. Data were collected using 5 video cameras and 2 force plates. An inverse dynamics...... upright walking pattern but continued to relieve the hip joint by maintaining a reduced flexor moment....

  4. A radiologist's guide to the imaging in the diagnosis and treatment of developmental dysplasia of the hip. Pt. 1. General considerations, physical examination as applied to real-time sonography and radiography

    International Nuclear Information System (INIS)

    Gerscovich, E.O.

    1997-01-01

    Developmental dysplasia of the hip (DDH) has a broad spectrum of presentation with the minor findings resolving spontaneously and the most severe ones resulting in disability, if not diagnosed early in life. Diagnosis in the first few months of life allows conservative treatment with complete resolution in most cases. Suspicion of DDH is based on ethnic, family, and pregnancy history, and on physical examination of the newborn. Imaging assists in the diagnosis and follows the treatment. Different modalities have their own advantages and disadvantages. This article deals with the description of the disease, risk factors, statistics, the physical examination as applied to real-time sonography, and imaging (plain radiography, arthrography, computed tomography, and magnetic resonance imaging). (orig.). With 26 figs., 1 tab

  5. The Fate of Iliopsoas Muscle in the Long-term Follow-up After Open Reduction of Developmental Dysplasia of the Hip by Medial Approach. Part 1: MRI Evaluation.

    Science.gov (United States)

    Yilmaz, Serdar; Aksahin, Ertugrul; Duran, Semra; Bicimoglu, Ali

    2017-09-01

    There has been little information about the long-term status of the iliopsoas, which is the main flexor of the hip, after iliopsoas tenotomy in the treatment of developmental dysplasia of the hip (DDH). The aim of this study was to assess the status of the iliopsoas muscle and other flexors and extensors of the hip in long-term follow-up with magnetic resonance imaging after complete iliopsoas tenotomy in patients with unilateral DDH treated with open reduction with a medial approach. The study included 20 patients who underwent open reduction with a medial approach for unilateral DDH and had long-term follow-up. Magnetic resonance imaging assessment of iliopsoas, rectus femoris, tensor fasia lata, sartorius, and gluteus maximus muscles was applied and the muscles of the hip that was operated on were compared with the unoperated hip. In addition, the iliopsoas muscle was examined for reattachment and the effect of reattachment was evaluated. The mean age at the time of operation was 10.53±3.61 months (range, 5 to 18 mo), and mean follow-up was 16.65±2.16 years (range, 13 to 20 y). Spontaneous reattachment of the iliopsoas was observed in 18 patients (90%), either in the lesser trochanter (65%) or the superior part of it (25%). There was no significant difference between the hips that were operated on and those that were not with regard to the mean cross-sectional areas (CSA) of the tensor fascia lata, rectus femoris, sartorius, and gluteus maximus muscles. The CSA of the tensor fascia lata, rectus femoris, sartorius, and gluteus maximus muscles showed no significant difference (P>0.05); however, CSA of iliopsoas muscle was significantly reduced in the operated hip (P<0.001). Although the iliopsoas tendon was atrophied after complete iliopsoas tenotomy, it was reattached in 90% of the patients spontaneously in long-term follow-up. There was no statistically significant compensatory hypertrophy in any muscles in response to iliopsoas atrophy. Level IV-Therapeutic.

  6. A murine model for developmental dysplasia of the hip: ablation of CX3CR1 affects acetabular morphology and gait

    Directory of Open Access Journals (Sweden)

    George Feldman

    2017-11-01

    Full Text Available Abstract Background Developmental dysplasia of the hip (DDH is a debilitating condition whose distinguishing signs include incomplete formation of the acetabulum leading to dislocation of the femur, accelerated wear of the articular cartilage and joint laxity resulting in osteoarthritis. It is a complex disorder having environmental and genetic causes. Existing techniques fail to detect milder forms of DDH in newborns leading to hip osteoarthritis in young adults. A sensitive, specific and cost effective test would allow identification of newborns that could be non-invasively corrected by the use of a Pavlik harness. Previously, we identified a 2.5 MB candidate region on human chromosome 3 by using linkage analysis of a 4 generation, 72 member family. Whole exome sequencing of the DNA of 4 severely affected members revealed a single nucleotide polymorphism variant, rs3732378 co-inherited by all 11 affected family members. This variant causes a threonine to methionine amino acid change in the coding sequence of the CX3CR1 chemokine receptor and is predicted to be harmful to the function of the protein To gain further insight into the function of this mutation we examined the effect of CX3CR1 ablation on the architecture of the mouse acetabulum and on the murine gait. Methods The hips of 5 and 8 weeks old wild type and CX3CR1 KO mice were analyzed using micro-CT to measure acetabular diameter and ten additional dimensional parameters. Eight week old mice were gait tested using an inclined treadmill with and without load and then underwent micro-CT analysis. Results (1 KO mice showed larger a 5–17% larger diameter left acetabula than WT mice at both ages. (2 At 8 weeks the normalized area of space (i.e. size discrepancy between the femur head and acetabulum is significantly larger [38% (p = 0.001–21% (p = 0.037] in the KO mice. (3 At 8 weeks gait analysis of these same mice shows several metrics that are consistent with impairment in

  7. A radiologist`s guide to the imaging in the diagnosis and treatment of developmental dysplasia of the hip. Pt. 1. General considerations, physical examination as applied to real-time sonography and radiography

    Energy Technology Data Exchange (ETDEWEB)

    Gerscovich, E.O. [Department of Radiology, University of California, Davis Medical Center, 2516 Stockton Blvd., Ticon II Building, Sacramento, CA 95817 (United States)

    1997-07-07

    Developmental dysplasia of the hip (DDH) has a broad spectrum of presentation with the minor findings resolving spontaneously and the most severe ones resulting in disability, if not diagnosed early in life. Diagnosis in the first few months of life allows conservative treatment with complete resolution in most cases. Suspicion of DDH is based on ethnic, family, and pregnancy history, and on physical examination of the newborn. Imaging assists in the diagnosis and follows the treatment. Different modalities have their own advantages and disadvantages. This article deals with the description of the disease, risk factors, statistics, the physical examination as applied to real-time sonography, and imaging (plain radiography, arthrography, computed tomography, and magnetic resonance imaging). (orig.). With 26 figs., 1 tab.

  8. Early detection of canine hip dysplasia: comparison of two palpation and five radiographic methods

    International Nuclear Information System (INIS)

    Adams, W.M.; Dueland, R.T.; Meinen, J.; O'Brien, R.T.; Giuliano, E.; Nordheim, E.V.

    1998-01-01

    Hip joint laxity was evaluated in four breeds (i.e., greyhound, Labrador retriever, Irish setter, hound mixed-breed) of puppies (n=32) by Ortolani's and Bardens' maneuvers, by subjective assessment of radiographs (Orthopedic Foundation for Animals [OFA] method), and by four radiographic measurement indices. Puppies were studied at four, six-to-10, 16-to-18, and 52 weeks of age. The purpose of this study was to compare palpation and radiographic methods of hip laxity detection in puppies for predicting the development of degenerative joint disease (DJD) by one year of age. Twenty-seven (42%) hips developed DJD. Ortolani's method was not a reliable predictor of hip dysplasia at six-to-10 weeks; it was significantly predictive at 16-to-18 weeks but had a high incidence of false negatives. Bardens' and subjective (OFA) assessment methods were not reliable at six-to-10 or 16-to-18 weeks. Radiographic measurements taken with femurs in a neutral position and hips distracted (distraction index [DI] and Norberg angle) and measurements taken with femurs extended in OFA position (Norberg angle) of six- to 10-week-old puppies accurately predicted DJD occurrence by one year of age (p less than 0.01). Distraction index measurement (PennHIP method) was the most accurate in predicting the development of DJD (p less than 0.001). Distraction index radiography in puppies six-to-10 and 16-to-18 weeks of age was the most reliable predictor of hip dysplasia. Norberg angle measurement was more reliable during hip distraction than when hips were measured in the OFA position in 16- to 18-week-old puppies, but had similar reliability in six- to 10-week-old puppies

  9. Developmental dysplasia of the hip, age, BMI, place of residence and tobacco abuse increase the odds of aseptic loosening in Chinese patients.

    Directory of Open Access Journals (Sweden)

    Chuanlong Wu

    Full Text Available PURPOSE: The purpose of this hospital-based case-control study was to evaluate the patient-related risk factors for aseptic loosening after total hip arthroplasty (THA and total knee arthroplasty (TKA in Chinese patients. METHODS: From January 2000 to December 2012, 67 patients undergoing THA and TKA who developed aseptic loosening were detected as case subjects and 336 patients without aseptic loosening, matched by the year of index surgery and type of surgery, were selected as controls. Conditional logistic regression was used to compute odds ratios (ORs and 95% confidence intervals (CIs. RESULTS: The demographic factors and comorbid conditions associated with a risk-adjusted increase in aseptic loosening (in decreasing order of significance were a rural place of residence (OR = 2.28; 95% CI: 1.21-4.30; p = 0.011, body mass index (BMI ≥ 28 kg/m(2 (vs. 18.5-28 kg/m(2 (OR = 2.29; 95% CI: 1.19-4.41; p = 0.013, developmental dysplasia of the hip (DDH (OR = 2.91; 95% CI: 1.11-7.66; p = 0.030, tobacco abuse (OR = 2.88; 95% CI: 1.05-7.89; p = 0.039, and age <45 years (vs. 45-65 years (OR = 2.63; 95% CI: 1.01-6.80; p = 0.047. CONCLUSIONS: Patients aged <45 years and those with a BMI of ≥ 28 kg/m(2, a preoperative diagnosis of DDH, history of tobacco abuse, or living in rural areas are at increased risk for aseptic loosening after THA and TKA in Chinese population. Additional systematic large-scale studies are needed to verify these results.

  10. Dysplasia epiphysealis multiplex - problems in diagnosis and treatment of the hip

    Energy Technology Data Exchange (ETDEWEB)

    Karski, T.; Ostrowski, J.; Karski, J. [Akademia Medyczna, Lublin (Poland)

    1991-12-31

    Dysplasia epiphysealis multiplex (DEM) is a generalized affection of the skeletal system involving the hips, knees, feet, shoulders, elbows and hands as well as the spine. It presents complex diagnostic problems, especially in babies and small children, in whom the symptoms are not distinctly developed. From the clinical points of view, DEM has to be distinguished from Perthes` disease. (author). 5 refs.

  11. The Demographics of Canine Hip Dysplasia in the United States and Canada

    Directory of Open Access Journals (Sweden)

    Randall T. Loder

    2017-01-01

    Full Text Available Canine hip dysplasia (CHD is a common problem in veterinary medicine. We report the demographics of CHD using the entire hip dysplasia registry from the Orthopedic Foundation for Animals, analyzing differences by breed, sex, laterality, seasonal variation in birth, and latitude. There were 921,046 unique records. Each dog was classified using the American Kennel Club (AKC and Fédération Cynologique Internationale (FCI systems. Statistical analysis was performed with bivariate and logistic regression procedures. The overall CHD prevalence was 15.56%. The OR for CHD was higher in females (1.05, those born in spring (1.14 and winter (1.13, and those in more southern latitudes (OR 2.12. Within AKC groups, working dogs had the highest risk of CHD (OR 1.882 with hounds being the reference group. Within FCI groups, the pinscher/molossoid group had the highest risk of CHD (OR 4.168 with sighthounds being the reference group. The similarities between CHD and DDH are striking. Within DDH there are two different types, the typical infantile DDH and the late onset adolescent/adult acetabular dysplasia, with different demographics; the demographics of CHD are more similar to the later onset DDH group. Comparative studies of both disorders should lead to a better understanding of both CHD and DDH.

  12. Homozygous mutations in IHH cause acrocapitofemoral dysplasia, an autosomal recessive disorder with cone- shaped epiphyses in hands and hips

    NARCIS (Netherlands)

    Hellemans, J; Coucke, PJ; Giedion, A; De Paepe, A; Kramer, P; Beemer, F; Mortier, GR

    Acrocapitofemoral dysplasia is a recently delineated autosomal recessive skeletal dysplasia, characterized clinically by short stature with short limbs and radiographically by cone-shaped epiphyses, mainly in hands and hips. Genome-wide homozygosity mapping in two consanguineous families linked the

  13. Joined at the hip? A paleoepidemiological study of developmental dysplasia of the hip and its relation to swaddling practices among indigenous peoples of North America.

    Science.gov (United States)

    Blatt, Samantha H

    2013-01-01

    Clinical prevalence of developmental dysplasia of the hip (DDH) is the highest among modern indigenous populations of North America, yet no systematic study of the paleoepidemiology of this group exists. This study discusses the skeletal criteria, epidemiology, pathophysiology, and risk factors for DDH. A range of cases of DDH from an archaeological Native American population are described, and the clinical and prehistoric prevalence of DDH among indigenous populations of North America are calculated and discussed within a biocultural perspective. Pelves of 390 adults from the Late Prehistoric (1490 BP ± 70) Buffalo site, West Virginia were examined for DDH. Morphology of true and false acetabula was classified and other changes of the pelvis, lower limb, and spine were noted along with cranial deformation, providing evidence of infant restriction. Prevalence of DDH among living and archaeological indigenous peoples of North America were calculated and compared. DDH was identified in eighteen adults from Buffalo, resulting in a prevalence of 46.15, within the range reported in modern indigenous groups in North America. However, there is a significant difference between the DDH prevalence in prehistory and today in the target population. Indigenous peoples of North America have the highest reported global prevalence of DDH today and in prehistory. The etiology of DDH suggests that components of both genetic predisposition and swaddling practices have combined to create a high-risk environment for the development of DDH, contributing to its high prevalence within archaeological populations, like Buffalo, and modern Native American/Aboriginal groups within North America. Copyright © 2013 Wiley Periodicals, Inc.

  14. Towards effective implementation strategies for ultrasound hip screening in child health care. Meet the parents.

    NARCIS (Netherlands)

    Witting, Marjon

    2012-01-01

    Early diagnosis and treatment of developmental dysplasia of the hip (DDH) is essential to allow for the normal development of the hip. In the Netherlands, the current screening for DDH consists of physical examination and identification of risk factors. In previous studies, ultrasound screening was

  15. Dosimetry of computerized tomography in the evaluation of hip dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Guyer, B.; Bassano, D.A.; Levinsohn, E.M.; Smith, D.S.; Cady, R.B.

    1984-07-01

    The usefulness of computerized tomography (CT) in the assessment of hip dysplasia has recently been given attention in the literature and concern regarding radiation dose has been raised. This study was undertaken to measure the radiation dose, both in and out of plaster, for plain films, arthrography, tomography, and CT. A method is suggested to reduce dosage by 80% without compromising diagnostic information. Our experience with 25 scans of patients aged 4 months to 39 years is presented.

  16. The early diagnosis of developmental dysplasia of the hip using ultrasonography. The importance of following up cases with physiological immaturity

    International Nuclear Information System (INIS)

    Esparza, J.; Gonzalez, A.; Garcia, S.; Elso, J.; Cordero, J. L.

    1999-01-01

    To determine the efficiency of an early diagnosis programme using ultrasounds (US) of development dysplasia of the hip (DDH), using different criteria to detect patients according to the sex: universal to girls, but only including boys with a DDH risk factor (a physical pathological examination of new born babies, babies born feet first, family history and orthopaedic deformities). Of the 3,469 children born at our hospital during one specific year, 1,721 were included in the programme. US was carried out on all of them, following the Graf technique, when they were a month old, with the exception of the new born babies who were studied during their first week by means of a physical pathological examination. The babies who were diagnosed with physically immature hips in this first study using US; were followed up. Thirty-two babies born during the year suffered from DDH (a rate of 9.2 per thousand). Among them there was only 1 case of delayed DDH (that was not detected in the programme). Seven babies were diagnosed with DDH thanks to the follow up procedures carried out on babies with physically immature hips. Putting this programme for early diagnosis into practices has achieved the almost total disappearance of delayed DDH. When comparing the results with previous periods, where different early detection systems were used, the duration and the aggressiveness of the treatment has reduced considerably. Follow up procedures using US of physically immature hips is obligatory. (Author) 14 refs

  17. Emerging insights into the genetic basis of canine hip dysplasia

    Directory of Open Access Journals (Sweden)

    Ginja M

    2015-05-01

    Full Text Available Mário Ginja,1 Ana Rita Gaspar,1 Catarina Ginja,2,3 1Department of Veterinary Sciences-CITAB, University of Trás-os-Montes and Alto Douro, Vila Real, Portugal; 2Ce3C – Centro de Ecologia, Evolução e Alterações Ambientais, Faculdade de Ciências, Universidade de Lisboa, Lisboa, Portugal; 3CIBIO-InBIO – Centro de Investigação em Biodiversidade e Recursos Genéticos, Universidade do Porto, Vairão, Portugal Abstract: Canine hip dysplasia (CHD is the most common inherited polygenic orthopedic trait in dogs with the phenotype influenced also by environmental factors. This trait was described in the dog in 1935 and leads to a debilitating secondary hip osteoarthritis. The diagnosis is confirmed radiographically by evaluating signs of degenerative joint disease, incongruence, and/or passive hip joint laxity. There is no ideal medical or surgical treatment so prevention based on controlled breeding is the optimal approach. The definitive CHD diagnosis based on radiographic examination involves the exposure to ionizing radiation under general anesthesia or heavy sedation but the image does not reveal the underlying genetic quality of the dog. Phenotypic expression of CHD is modified by environmental factors and dogs with a normal phenotype can be carriers of some mutations and transmit these genes to their offspring. Programs based on selection of dogs with better individual phenotypes for breeding are effective when strictly applied but remain inferior to the selection of dogs based on estimation of breeding values. Molecular studies for dissecting the genetic basis of CHD are ongoing, but progress has been slow. In the future, the recommended method to improve hip quality in controlled breeding schemes, which will allow higher selection pressure, would be based on the estimation of the genomic breeding value. Since 2012, a commercial DNA test has been available for Labrador Retrievers using a blood sample and provides a probability for

  18. Radiological findings for hip dysplasia at skeletal maturity. Validation of digital and manual measurement techniques.

    Science.gov (United States)

    Engesæter, Ingvild Øvstebø; Laborie, Lene Bjerke; Lehmann, Trude Gundersen; Sera, Francesco; Fevang, Jonas; Pedersen, Douglas; Morcuende, José; Lie, Stein Atle; Engesæter, Lars Birger; Rosendahl, Karen

    2012-07-01

    To report on intra-observer, inter-observer, and inter-method reliability and agreement for radiological measurements used in the diagnosis of hip dysplasia at skeletal maturity, as obtained by a manual and a digital measurement technique. Pelvic radiographs from 95 participants (56 females) in a follow-up hip study of 18- to 19-year-old patients were included. Eleven radiological measurements relevant for hip dysplasia (Sharp's, Wiberg's, and Ogata's angles; acetabular roof angle of Tönnis; articulo-trochanteric distance; acetabular depth-width ratio; femoral head extrusion index; maximum teardrop width; and the joint space width in three different locations) were validated. Three observers measured the radiographs using both a digital measurement program and manually in AgfaWeb1000. Inter-method and inter- and intra-observer agreement were analyzed using the mean differences between the readings/readers, establishing the 95% limits of agreement. We also calculated the minimum detectable change and the intra-class correlation coefficient. Large variations among different radiological measurements were demonstrated. However, the variation was not related to the use of either the manual or digital measurement technique. For measurements with greater absolute values (Sharp's angle, femoral head extrusion index, and acetabular depth-width ratio) the inter- and intra-observer and inter-method agreements were better as compared to measurements with lower absolute values (acetabular roof angle, teardrop and joint space width). The inter- and intra-observer variation differs notably across different radiological measurements relevant for hip dysplasia at skeletal maturity, a fact that should be taken into account in clinical practice. The agreement between the manual and digital methods is good.

  19. Radiological findings for hip dysplasia at skeletal maturity. Validation of digital and manual measurement techniques

    Energy Technology Data Exchange (ETDEWEB)

    Engesaeter, Ingvild Oevsteboe [University of Bergen, Department of Surgical Sciences, Bergen (Norway); Haukeland University Hospital, Department of Orthopaedic Surgery, Bergen (Norway); Haukeland University Hospital, Department of Radiology, Bergen (Norway); Haukeland University Hospital, The Norwegian Arthroplasty Register, Department of Orthopaedic Surgery, Bergen (Norway); Laborie, Lene Bjerke; Rosendahl, Karen [University of Bergen, Department of Surgical Sciences, Bergen (Norway); Haukeland University Hospital, Department of Radiology, Bergen (Norway); Lehmann, Trude Gundersen; Fevang, Jonas; Engesaeter, Lars Birger [University of Bergen, Department of Surgical Sciences, Bergen (Norway); Haukeland University Hospital, Department of Orthopaedic Surgery, Bergen (Norway); Sera, Francesco [University College London Institute of Child Health, Medical Research Council Centre of Epidemiology for Child Health, London (United Kingdom); Pedersen, Douglas; Morcuende, Jose [University of Iowa Hospital and Clinics, Department of Orthopaedics and Rehabilitation, Iowa City, IA (United States); Lie, Stein Atle [Uni Health, Uni Research, Bergen (Norway)

    2012-07-15

    To report on intra-observer, inter-observer, and inter-method reliability and agreement for radiological measurements used in the diagnosis of hip dysplasia at skeletal maturity, as obtained by a manual and a digital measurement technique. Pelvic radiographs from 95 participants (56 females) in a follow-up hip study of 18- to 19-year-old patients were included. Eleven radiological measurements relevant for hip dysplasia (Sharp's, Wiberg's, and Ogata's angles; acetabular roof angle of Toennis; articulo-trochanteric distance; acetabular depth-width ratio; femoral head extrusion index; maximum teardrop width; and the joint space width in three different locations) were validated. Three observers measured the radiographs using both a digital measurement program and manually in AgfaWeb1000. Inter-method and inter- and intra-observer agreement were analyzed using the mean differences between the readings/readers, establishing the 95% limits of agreement. We also calculated the minimum detectable change and the intra-class correlation coefficient. Large variations among different radiological measurements were demonstrated. However, the variation was not related to the use of either the manual or digital measurement technique. For measurements with greater absolute values (Sharp's angle, femoral head extrusion index, and acetabular depth-width ratio) the inter- and intra-observer and inter-method agreements were better as compared to measurements with lower absolute values (acetabular roof angle, teardrop and joint space width). The inter- and intra-observer variation differs notably across different radiological measurements relevant for hip dysplasia at skeletal maturity, a fact that should be taken into account in clinical practice. The agreement between the manual and digital methods is good. (orig.)

  20. Articular distractor in the early radiographic diagnosis of canine hip dysplasia

    International Nuclear Information System (INIS)

    Tôrres, R.C.S.; Araújo, R.B.; Rezende, C.M.F.

    2005-01-01

    Aiming the canine hip dysplasia (CHD) early diagnosis, 60 dogs of both sexes (32 females and 28 males) and of different breeds had their hip joints radiographically studied. The X ray examinations were taken in early age (7.2± 1.2 months) and repeated at adult age (14.4± 1.6 months) using the conventional radiographic method (CRM) and the radiographic distraction method (RDM) performed, by its turn, with a new device, specially designed or this experiment. In order to quantify the relationship between the femoral head and the acetabulus the Norberg Angle (NA) was measured at CRM and the distraction index (DI) was calculated at RDM. There was a significant statistical correlation (P [pt

  1. Identification of quantitative trait loci (QTL for canine hip dysplasia and canine elbow dysplasia in Bernese mountain dogs.

    Directory of Open Access Journals (Sweden)

    Sophia Pfahler

    Full Text Available A genome-wide association study for canine hip dysplasia (CHD and canine elbow dysplasia (CED using the Illumina canine high density bead chip had been performed for 174 Bernese mountain dogs. General and mixed linear model analysis identified two different regions with single nucleotide polymorphisms (SNPs on dog chromosome (CFA 14 significantly associated with CHD and a further significantly CHD-associated region on CFA37. For CED, four SNPs on CFA11 and 27 were significantly associated. The identified SNPs of four associated regions included nearby candidate genes. These possible positional candidates were the genes PON2 on CFA14 and FN1 on CFA37 for CHD and the genes LMNB1 on CFA11 and WNT10B on CFA27 for CED.

  2. Low revision rate after total hip arthroplasty in patients with pediatric hip diseases

    DEFF Research Database (Denmark)

    Engesæter, Lars B; Engesæter, Ingvild Ø; Fenstad, Anne Marie

    2012-01-01

    Background The results of primary total hip arthroplasties (THAs) after pediatric hip diseases such as developmental dysplasia of the hip (DDH), slipped capital femoral epiphysis (SCFE), or Perthes' disease have been reported to be inferior to the results after primary osteoarthritis of the hip (OA.......9%) were operated due to pediatric hip diseases (3.1% for Denmark, 8.8% for Norway, and 1.9% for Sweden) and 288,435 THAs (77.8%) were operated due to OA. Unadjusted 10-year Kaplan-Meier survival of THAs after pediatric hip diseases (94.7% survival) was inferior to that after OA (96.6% survival......). Consequently, an increased risk of revision for hips with a previous pediatric hip disease was seen (risk ratio (RR) 1.4, 95% CI: 1.3-1.5). However, after adjustment for differences in sex and age of the patients, and in fixation of the prostheses, no difference in survival was found (93.6% after pediatric hip...

  3. Frequency and asymmetry of hip dysplasia in German Shepherd dog

    International Nuclear Information System (INIS)

    Torres, R.C.S.; Ferreira, P.M.; Silva, D.C.

    1999-01-01

    A study of frequency and asymmetry of hip dysplasia was carried out using 181 X-rays plates of German Shepherd dogs, both males and females, from June 1986 to March 1993. The X-ray plates were taken with the animals on the ventrodorsal position, with the limbs parallel and the pelvis symmetric. The average age of the dogs was 16.6 months. Results showed 72.4% of dysplastic dogs, being 5.3% unilateral, 89.4% bilateral simmetric and 5.3% bilateral asymmetric. (author) [pt

  4. A retrospective study on findings of canine hip dysplasia screening in Kenya

    Directory of Open Access Journals (Sweden)

    Peter Kimeli

    2015-11-01

    Full Text Available Aim: The current study was undertaken to evaluate the findings of canine hip dysplasia screening in Kenya. Materials and Methods: Records for 591 dogs were included in this study. The data was obtained from the national screening office, Kenya Veterinary Board, for the period between the years 1998 and 2014. Monthly screening records were assessed and information relating to year of evaluation, breed, sex, age, and hip score captured. Descriptive statistics of hip scores was computed based on year, sex, age, and breed. Results: A total of 591 records from the year 1998 to 2014 were retrieved at the National Screening Centre, the Kenya Veterinary Board. Each record was examined and data pertaining to year of screening, the breed, sex, age of the dogs, and the total hip score were recorded. The highest number of dogs screened for hip dysplasia (HD was in the year 2009 and the lowest in the year 1998. More females than males were screened for HD and the mean age of all the dogs was 22.9±12.7 months. The most common breeds of dogs screened during the study period were German Shepherd (67.0%, Rottweiler (15.6%, and Labrador Retriever (12.2%. The mean hip score for the 591 dogs was 15.1±10.9 and the median 12.0. The mean hip scores per breed were; German Shepherd (16.3±12.1; Golden Retriever (16.0; Hungarian Vizla (15.0; Labrador Retriever (3.0±6.7; Great Dane (13.3±3.2; Rottweiler (12.2±8.2; Doberman (10.3±4.2; Rhodesian Ridgeback (9.6±3.8; and Boxer (9.3±0.6. Based on the hip score, moderate to severe HD was diagnosed in 16.6% of the dogs, mild HD in 32.7%, Borderline HD in 37.7%, fair HD in 6.9%, and good HD in 6.1%. Conclusion: Canine HD is a common occurrence in Kenya with most dogs suffering mild to border line HD. In addition, German Shepherd and Golden Retriever appear to be the most affected breeds. It is therefore recommended that stringent measures be imposed to dog breeding programs to avoid transmission of this undesirable trait

  5. Advances in the early diagnosis of congenital hip dysplasia by real-time sonography

    Energy Technology Data Exchange (ETDEWEB)

    Brockmann, W.P.; Wilmsdorf, H.v.; Weh, L.; Korn, U.

    1984-05-01

    Twenty-four infants and children aged five days to three years had their hips examined by ultrasound, and in 17 the findings were compared with the radiographic appearances. Real-time sonography, because of its flexibility, is a suitable screening method for the early diagnosis of hip dysplasia. It has the typical characteristics of a screening method: so far there have been no false negative findings. False positive results are rare, but cannot be completely excluded. The patients examined so far were mostly abnormal, and a final assessment of false positive findings will have to await larger clinical material, including normals. 6 figs.

  6. Hip ultrasound

    Energy Technology Data Exchange (ETDEWEB)

    Martinoli, Carlo, E-mail: carlo.martinoli@libero.it [Radiologia, DISC, Università di Genova, Largo Rosanna Benzi 8, I-16132 Genoa (Italy); Garello, Isabella; Marchetti, Alessandra; Palmieri, Federigo; Altafini, Luisa [Radiologia, DISC, Università di Genova, Largo Rosanna Benzi 8, I-16132 Genoa (Italy); Valle, Maura [Radiologia, Gaslini Children Hospital, Genova (Italy); Tagliafico, Alberto [Radiologia, National Institute for Cancer Research, Genoa (Italy)

    2012-12-15

    In newborns, US has an established role in the detection and management of developmental dysplasia of the hip. Later in childhood, when the limping child is a major diagnostic dilemma, US is extremely helpful in the identification of the varied disease processes underlying this condition, as transient synovitis, septic arthritis, Perthes disease and slipped femoral capital epiphysis. In adolescent practicing sporting activities, US is an excellent means to identify apophyseal injures about the pelvic ring, especially when avulsions are undisplaced and difficult-to-see radiographically. Later on, in the adulthood, US is an effective modality to diagnose tendon and muscle injuries about the hip and pelvis, identify effusion or synovitis within the hip joint or its adjacent bursae and guide the treatment of these findings. The aim of this article is to provide a comprehensive review of the most common pathologic conditions about the hip, in which the contribution of US is relevant for the diagnostic work-up.

  7. Physical and Clinical Evaluation of Hip Spica Cast applied with Three-slab Technique using Fibreglass Material

    Directory of Open Access Journals (Sweden)

    Bitar KM

    2016-11-01

    Full Text Available Introduction: Hip spica casting is an important component of treatment for developmental dysplasia of the hip (DDH and popular treatment method for femur fractures in children. Breakage at the hip region is a relatively common problem of this cast. We have developed a three-slab technique of hip spica application using fibreglass as the cast material. The purpose of this review was to evaluate the physical durability of the spica cast and skin complications with its use. Methodology: A retrospective review of children with various conditions requiring hip spica immobilisation which was applied using our method. Study duration was from 1st of January 2014 until 31st December 2015. Our main outcomes were cast breakage and skin complications. For children with hip instability, the first cast would be changed after one month, and the second cast about two months later. Results: Twenty-one children were included, with an average age of 2.2 years. The most common indication for spica immobilisation was developmental dysplasia of the hip. One child had skin irritation after spica application. No spica breakage was noted. Conclusion: This study showed that the three-slab method of hip spica cast application using fibreglass material was durable and safe with low risk of skin complications.

  8. A radiological study of the incidence of unilateral canine hip dysplasia

    International Nuclear Information System (INIS)

    Citi, S.; Vignoli, M.; Modenato, M.; Rossi, F.; Morgan, J.P.

    2005-01-01

    The authors reviewed pelvic radiographs of 891 dogs in a retrospective study, to determine the incidence of Unilateral Canine Hip Dysplasia (UCHD). Results show that 149 (16.7%) dogs had UCHD. Comparing dogs affected uni- and bilaterally, results show a maximum of 37.6% with UCHD in dogs less than 12 month old, 22.8% in dogs between 12-24 months of age, 25.5% in dogs between 25-72 months and 14.1% in dogs older than 73 months

  9. Imaging of the hip and bony pelvis. Techniques and applications

    Energy Technology Data Exchange (ETDEWEB)

    Davies, A.M. [Royal Orthopaedic Hospital, Birmingham (United Kingdom). MRI Centre; Johnson, K.J. [Princess of Wales Birmingham Children' s Hospital (United Kingdom); Whitehouse, R.W. (eds.) [Manchester Royal Infirmary (United Kingdom). Dept. of Clinical Radiology

    2006-07-01

    This is a comprehensive textbook on imaging of the bony pelvis and hip joint that provides a detailed description of the techniques and imaging findings relevant to this complex anatomical region. In the first part of the book, the various techniques and procedures employed for imaging the pelvis and hip are discussed in detail. The second part of the book documents the application of these techniques to the diverse clinical problems and diseases encountered. Among the many topics addressed are congenital and developmental disorders including developmental dysplasia of the hip, irritable hip and septic arthritis, Perthes' disease and avascular necrosis, slipped upper femoral epiphysis, bony and soft tissue trauma, arthritis, tumours and hip prostheses. Each chapter is written by an acknowledged expert in the field, and a wealth of illustrative material is included. This book will be of great value to musculoskeletal and general radiologists, orthopaedic surgeons and rheumatologists. (orig.)

  10. Efficient selection against categorically scored hip dysplasia in dogs is possible using best linear unbiased prediction and optimum contribution selection: a simulation study

    DEFF Research Database (Denmark)

    Malm, S; Sørensen, Anders Christian; Fikse, W F

    2013-01-01

    Breeding to reduce the prevalence of categorically scored hip dysplasia (HD), based on phenotypic assessment of radiographic hip status, has had limited success. The aim of this study was to evaluate two selection strategies for improved hip status: truncation selection based on phenotypic record...

  11. An indication of major genes affecting hip and elbow dysplasia in four Finnish dog populations

    NARCIS (Netherlands)

    Maki, K.; Janss, L.L.G.; Groen, A.F.; Liinamo, A.E.; Ojala, M.

    2004-01-01

    The aim of the study was to assess the possible existence of major genes influencing hip and elbow dysplasia in four dog populations. A Bayesian segregation analysis was performed separately on each population. In total, 34 140 dogs were included in the data set. Data were analysed with both a

  12. Secondary hip dysplasia in Legg-Calvé-Perthes disease - a long-term case-control study

    DEFF Research Database (Denmark)

    Froberg, Lonnie; Christensen, Finn; Pedersen, Niels Wisbech

    Poor long-time results in Legg-Calvé-Perthes disease (LCP) have in previous reports been attributed to the presence of degenerative joint disease due to deformities of the femoral head. Reports have stated that hip dysplasia (HD) pre-dispose to hip osteoarthritis (OA). The increased risk of OA...... excluded, however we do not have any reason to believe that they were excluded systematically. The reviewed Stulberg classification was used. The advantages of the classification included the simplicity of application and reliability with good inter- and intraobserver agreement. Previous report has also...

  13. Radiographic evaluation of coxofemoral joint laxity in dogs part II: Comparison of stress-radiographic positioning techniques in dogs with hip dysplasia

    International Nuclear Information System (INIS)

    Duangdaun Kaenkangploo; Phiwipha Kamonrat; Marissak Kalpravidh

    2002-01-01

    Two stress-radiographic positioning techniques for evaluation of coxofemoral joint laxity in dogs with hip dysplasia were compared with the standard technique. Forty, healthy, large breed dogs were divided into two groups of 20 dogs. Group 1 had normal hips. Group 2 were dogs with mild to moderated grade of hip dysplasia according to the Orthopedic Foundation for Animals (OFA) standard. Dogs were anesthetized and placed in dorsal recumbency before 3 radiographic techniques, standard hip-extended, 60 deg and 90 deg stress techniques, were taken. For the 60 deg stress technique, hind legs were extended in parallel to each other at 60 deg to the table top and femoral heads were manually pushed craniodorsally during exposure. For the 90 deg stress technique, femurs were positioned perpendicular to the table top, stifles were 90 deg flexed and adducted and femoral heads were manually pushed in a craniodorsal direction during exposure. Subluxation index (SI) and dorsolateral subluxation score (DLS score) of coxofemoral joints were assessed from radiographs. The SI of normal dogs from standard, 60 deg and 90 deg stress techniques were 0.15, 0.20 and 0.23 and of dysplastic dogs were 0.34, 0.40 and 0.41 respectively. The degress of subluxation assessed from the two stress technique radiographs were significantly greater (p0.05) than those shown on the standard technique radiographs in both groups of dogs. DLS scores of normal dogs from standard, 60 deg and 90 deg stress techniques were 65.1, 64.3 and 61.0 percent and of dysplastic dogs were 55.4, 53.6 and 47.6 percent respectively. Mean of DLS scores assessed from the 90 deg radiographs was significantly lower (p0.05) than those assessed from radiographs of other two teachniques in both groups of dogs. The findings suggested that the 90 deg stress technique is more efficient than the standard and 60 deg stress techniques for radiographic evaluation of coxofemoral joint laxity in dogs with mild hip dysplasia and early

  14. Developmental tumors and adjacent cortical dysplasia: single or dual pathology?

    Science.gov (United States)

    Palmini, André; Paglioli, Eliseu; Silva, Vinicius Duval

    2013-12-01

    Developmental tumors often lead to refractory partial seizures and constitute a well-defined, surgically remediable epilepsy syndrome. Dysplastic features are often associated with these tumors, and their significance carries both practical and conceptual relevance. If associated focal cortical dysplasia (FCD) relates to the extent of the epileptogenic tissue, then presurgical evaluation and surgical strategies should target both the tumor and the surrounding dyslaminated cortex. Furthermore, the association has been included in the recently revised classification of FCD and the epileptogenicity of this associated dysplastic tissue is crucial to validate such revision. In addition to the possibility of representing dual pathology, the association of developmental tumors and adjacent dysplasia may instead represent a single developmental lesion with distinct parts distributed along a histopathologic continuum. Moreover, the possibility that this adjacent dyslamination is of minor epileptogenic relevance should also be entertained. Surgical data show that complete resection of the solid tumors and immediately adjacent tissue harboring satellites may disrupt epileptogenic networks and lead to high rates of seizure freedom, challenging the epileptogenic relevance of more extensive adjacent dyslaminated cortex. Whether the latter is a primary or secondary abnormality and whether dyslaminated cortex in the context of a second lesion may produce seizures after complete resection of the main lesion is still to be proven. Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.

  15. Ranking of computed tomography in congenital hip dysplasia and hip dislocation

    Energy Technology Data Exchange (ETDEWEB)

    Lingg, G.; Nebel, G.; Thomas, W.; Hering, L.

    1983-12-01

    A lot of methods of measurement have been developed to record precisely the degrees of luxation and the deviations from the normal form in the hip joints of the child and adult. The orthopaedic surgeon needs those data for a preoperative ''coxometric program'' before performing acetabuloplastic operations with osteotomia, osteotomia of the pelvis and combined operations. Computed tomography yields a series of informations and of possibilities of measurements which can determine the operative procedure in particular. These informations concern among other things the dimensions of the dysplasia of the acetabulum in the horizontal plane with the extension of the posterior lip and the angle of the acetabular opening, furthermore the physiological or pathological congruence between the head of the femur and the acetabulum, the angle of antetorsion of the neck of the femur and, in the small infant, the direct noninvasive imaging of the tube of the capsule with imaging of possible obstacles to reposition.

  16. Avaliação clínica e radiológica após procedimento de Salter e Ombrédanne na displasia de desenvolvimento do quadril Clinical and radiological evaluation on developmental hip dysplasia after salter and Ombrédanne procedure

    Directory of Open Access Journals (Sweden)

    Válney Luiz da Rocha

    2011-01-01

    Full Text Available OBJETIVO: Avaliar o resultado clínico e radiológico em médio prazo do tratamento cirúrgico da displasia do desenvolvimento do quadril através da osteotomia do osso inominado de Salter e o encurtamento femoral de Ombrédanne. MÉTODOS: Foram avaliados 14 pacientes, 18 quadris (sete quadris do lado direito e 11 do lado esquerdo, todos tratados cirurgicamente entre 1998 e 2008 pela técnica proposta. Foi realizada avaliação clínica e radiológica pelos critérios de Dutoit e Severin, respectivamente. RESULTADOS: Nos sete quadris do lado direito o índice pré-operatório médio foi de 43,3º (40º a 50º, sendo corrigido cirurgicamente, em média, para 31,57º (24º a 42º, enquanto os 11 quadris do lado esquerdo tinham média pré-operatória de 42,1º (36º a 56º evoluindo para 30,36º (20º a 44º, sendo que as avaliações dos índices acetabulares pré e pós-operatórios apresentaram significância estatística com P 0,05. Como complicações, ocorreram uma subluxação, uma osteonecrose e uma osteonecrose associada à subluxação. CONCLUSÃO: O procedimento combinado de Salter e Ombrédanne é uma opção viável para o tratamento da displasia de desenvolvimento do quadril após o início da marcha.OBJECTIVE: To evaluate the clinical and radiological medium-term results from surgical treatment of developmental hip dysplasia through Salter innominate bone osteotomy and Ombrédanne femoral shortening. METHODS: Fourteen patients were evaluated, with surgical treatment on 18 hips (seven right-side hips and eleven left-side hips using the proposal technique, performed between 1998 and 2008. The Dutoit and Severin criteria were used respectively for clinical and radiographic evaluations. RESULTS: The average preoperative index for the seven right-side hips was 43.3º (40º to 50º, and this was corrected through surgery to an average of 31.57º (24º to 42º. The average preoperative index for the eleven left-side hips was 42.1º (36º

  17. Femoral anteversion is correlated with acetabular version and coverage in Asian women with anterior and global deficient subgroups of hip dysplasia: a CT study

    International Nuclear Information System (INIS)

    Akiyama, Mio; Nakashima, Yasuharu; Fujii, Masanori; Sato, Taishi; Yamamoto, Takuaki; Mawatari, Taro; Motomura, Goro; Matsuda, Shuichi; Iwamoto, Yukihide

    2012-01-01

    Morphological correlation between the acetabulum and femur at the hip joint is still controversial. We tested the hypothesis that femoral anteversion correlates with acetabular version and coverage in patients with developmental dysplasia of the hip (DDH). Using pelvic computed tomography (CT) images of 79 hips in 49 Asian women with DDH and 49 normal hips, we measured femoral anteversion, the axial and vertical acetabular version and the acetabular sector angle (ASA) to demarcate femoral head coverage. Depending on the location of the acetabular bone defect, dysplastic hips were divided into three subgroups: the anterior, global and posterior deficiency groups. We performed a comparative analysis between dysplastic and normal hips using the Wilcoxon rank sum test, and a relative analysis between femoral anteversion and acetabular measurements in dysplastic hips using Pearson's correlation coefficient. The amount of femoral anteversion in dysplastic hips was greater and more variable than in normal hips (p < 0.0001, p = 0.0277 respectively). Femoral anteversion in dysplastic hips correlated significantly with acetabular anteversion in the groups with anterior and global deficiency subgroups (p < 0.05, r = 0.2990, p < 0.05, r = 0.451 respectively), but not with the posterior deficiency subgroup. Femoral anteversion also correlated with vertical acetabular version. When acetabular coverage was examined, significant correlations were noted between femoral anteversion and anterior and superior coverage, but not with posterior coverage. These correlations were not observed in normal hips. Our results showed significantly greater and more variable femoral anteversion in DDH, and a significant correlation between femoral anteversion and acetabular version and coverage in DDH with anterior and global acetabular bone deficiency. (orig.)

  18. Femoral anteversion is correlated with acetabular version and coverage in Asian women with anterior and global deficient subgroups of hip dysplasia: a CT study

    Energy Technology Data Exchange (ETDEWEB)

    Akiyama, Mio; Nakashima, Yasuharu; Fujii, Masanori; Sato, Taishi; Yamamoto, Takuaki; Mawatari, Taro; Motomura, Goro; Matsuda, Shuichi; Iwamoto, Yukihide [Kyushu University, Department of Orthopaedic Surgery, Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka (Japan)

    2012-11-15

    Morphological correlation between the acetabulum and femur at the hip joint is still controversial. We tested the hypothesis that femoral anteversion correlates with acetabular version and coverage in patients with developmental dysplasia of the hip (DDH). Using pelvic computed tomography (CT) images of 79 hips in 49 Asian women with DDH and 49 normal hips, we measured femoral anteversion, the axial and vertical acetabular version and the acetabular sector angle (ASA) to demarcate femoral head coverage. Depending on the location of the acetabular bone defect, dysplastic hips were divided into three subgroups: the anterior, global and posterior deficiency groups. We performed a comparative analysis between dysplastic and normal hips using the Wilcoxon rank sum test, and a relative analysis between femoral anteversion and acetabular measurements in dysplastic hips using Pearson's correlation coefficient. The amount of femoral anteversion in dysplastic hips was greater and more variable than in normal hips (p < 0.0001, p = 0.0277 respectively). Femoral anteversion in dysplastic hips correlated significantly with acetabular anteversion in the groups with anterior and global deficiency subgroups (p < 0.05, r = 0.2990, p < 0.05, r = 0.451 respectively), but not with the posterior deficiency subgroup. Femoral anteversion also correlated with vertical acetabular version. When acetabular coverage was examined, significant correlations were noted between femoral anteversion and anterior and superior coverage, but not with posterior coverage. These correlations were not observed in normal hips. Our results showed significantly greater and more variable femoral anteversion in DDH, and a significant correlation between femoral anteversion and acetabular version and coverage in DDH with anterior and global acetabular bone deficiency. (orig.)

  19. Presença de "Linha Morgan" como indicador de displasia coxofemoral em cães da raça Pastor-Alemão Presence of Morgan-Line as an indicator of canine hip dysplasia in German Shepherd dogs

    Directory of Open Access Journals (Sweden)

    R.C.S. Tôrres

    1999-04-01

    Full Text Available The aim of this study was to determine the relationship between the presence of Morgan line and hip dysplasia. From June, 1986 to October, 1993, 190 X-ray plates of German Shepherd dogs, from both sexes were collected and analyzed. The results showed that out of the 190 dogs evaluated, 136 (71.6% had hip dysplasia, 43 (22.6% presented Morgan line and 41 (21.5% showed both of them. Just two (1.1% dogs presented Morgan line without hip dysplasia. No statistically significant difference was found between males (21.8% and females (23.3%. From this study it can be concluded that the presence of the Morgan line can be considered indicative to hip dysplasia diagnosis, even though the absence of the line does not exclude the possibility of hip dysplasia.

  20. Risk factors for the need of hip arthroscopy following periacetabular osteotomy

    Science.gov (United States)

    Hartig-Andreasen, Charlotte; Troelsen, Anders; Thillemann, Theis M.; Gelineck, John; Søballe, Kjeld

    2015-01-01

    Despite the frequency of labral tears in symptomatic developmental dysplasia of the hip, no consensus exists regarding the treatment of coexisting dysplasia of the hip and tearing of the acetabular labrum. The purpose of this prospective, MR arthrography (MRA) based 2-year follow-up study was to identify risk factors predicting the need for a hip arthroscopy (HA) after periacetabular osteotomy (PAO). Ninety-nine patients (104 hips) scheduled for PAO were evaluated preoperatively and at 2-year follow-up. MRA was performed in all patients prior to PAO. At follow-up, patients were divided into a non-arthroscopy and arthroscopy group. The two groups were compared clinical and radiological, and risk factors for HA after PAO were calculated. Patient reported outcome measures (WOMAC, Oxford Hip and SF36) were filled out before PAO and at follow-up. Ninety-five hips (91.3%) were evaluated. Twenty-six hips (27%) required an arthroscopy within 2 years of the PAO. Risk factors were preoperative borderline dysplasia, acetabular retroversion and complete labral detachment. Labral tearing, degeneration or hypertrophy did not negatively affect the outcome of PAO. Patients not requiring an arthroscopy had a statistically significant better outcome measured by patients reported outcome measures. After PAO, 27% of the hips needed intra-articular assessment. Conventional radiographs and MRA analysis can be used to identify predictors for patients requiring HA after PAO. At 2-year follow-up, the clinical outcome improved in all patients. However, those patients who had no need of a HA after their PAO had superior results. PMID:27011862

  1. Incidence, risk factors and heritability of hind limb lameness in dogs with canine hip dysplasia in a 8 year follow up study of a birth cohort of Dutch boxer dogs

    NARCIS (Netherlands)

    Hagen, van M.A.E.; Ducro, B.J.; Broek, van de J.; Knol, B.W.

    2005-01-01

    Objective-To determine incidence, risk factors, and heritability estimates of hind limb lameness caused by hip dysplasia in a birth cohort of Boxers. Animals-1,733 Boxers from 325 litters. Procedure-Status of Boxers with respect to clinical signs of canine hip dysplasia (cCHD) was registered during

  2. Trends in hip dysplasia control: analysis of radiographs submitted to the orthopedic foundation for animals, 1974 to 1984

    International Nuclear Information System (INIS)

    Corley, E.A.; Hogan, P.M.

    1985-01-01

    From 1974 through 1984, the Orthopedic Foundation for Animals evaluated 143,218 radiographic submissions representing 151 breeds of dogs. All breeds from which there were 35 or more evaluations had some frequency of dysplasia. Seventy breeds, each with over 100 submissions, were tabulated and ranked according to frequency of hip dysplasia. Frequency of dysplasia varied from 0.6% in the Borzoi to 46.9% in the Saint Bernard. These data were compared with data obtained earlier (1966 to 1973) on evaluations in 38 breeds for changes in frequency. There was significant (P less than 0.05) reduction in frequency of dysplasia in 27 breeds, a significant (P less than 0.05) increase in frequency in only 1 breed (German Shorthaired Pointer), and no significant change in frequency in 10 breeds. The median significant decrease was 22.4%, and the range was from 3.1% in the Chesapeake Bay Retriever to 48.7% in the Keeshond. The reduction in frequency of hip dysplasia demonstrated the value of a control program. There were 5 breeds with a significant (P less than 0.05) decrease in frequency of dysplasia that had over 5,000 evaluations from 1974 to 1984. The decreases in frequency were independent of changes in American Kennel Club registrations for these breeds (a dramatic decline in registrations for the German Shepherd Dog and Old English Sheepdog, and a dramatic increase for the Rottweiler, Golden Retriever, and Labrador Retriever). Frequency regressed linearly in the German Shepherd Dog and Old English Sheepdog, but regressed nonlinearly in the other 3 breeds. The percentage reduction in frequency from the base frequency (1966 to 1973) for these breeds was 17.5% for the German Shepherd Dog, 23.1% for the Old English Sheepdog, 9.1% for the Rottweiler, 10.1% for the Golden Retriever, and 6.8% for the Labrador Retriever

  3. Atraumatic Anterior Dislocation of the Hip Joint

    Directory of Open Access Journals (Sweden)

    Tadahiko Ohtsuru

    2015-01-01

    Full Text Available Dislocation of the hip joint in adults is usually caused by high-energy trauma such as road traffic accidents or falls from heights. Posterior dislocation is observed in most cases. However, atraumatic anterior dislocation of the hip joint is extremely rare. We present a case of atraumatic anterior dislocation of the hip joint that was induced by an activity of daily living. The possible causes of this dislocation were anterior capsule insufficiency due to developmental dysplasia of the hip, posterior pelvic tilt following thoracolumbar kyphosis due to vertebral fracture, and acetabular anterior coverage changes by postural factor. Acetabular anterior coverage changes in the sagittal plane were measured using a tomosynthesis imaging system. This system was useful for elucidation of the dislocation mechanism in the present case.

  4. Determinants of parental satisfaction with ultrasound hip screening in child health care.

    NARCIS (Netherlands)

    Witting, Marjon; Boere-Boonekamp, Magdalena M.; Fleuren, M.A.H.; Sakkers, R.J.B.; IJzerman, Maarten Joost

    2012-01-01

    Prior research has shown ultrasound (US) screening for developmental dysplasia of the hip (DDH) in preventive child health care to be more effective than the current screening method. In the present study, 3-month-old infants were screened for DDH with US. The objective of this study was to examine

  5. Determinants of parental satisfaction with ultrasound hip screening in child health care

    NARCIS (Netherlands)

    Witting, M.; Boere-Boonekamp, M.M.; Fleuren, M.A.H.; Sakkers, R.J.B.; IJzerman, M.J.

    2012-01-01

    Prior research has shown ultrasound (US) screening for developmental dysplasia of the hip (DDH) in preventive child health care to be more effective than the current screening method. In the present study, 3-month-old infants were screened for DDH with US. The objective of this study was to examine

  6. Radiographic measurements of hip dysplasia at skeletal maturity - new reference intervals based on 2,038 19-year-old Norwegians

    Energy Technology Data Exchange (ETDEWEB)

    Laborie, Lene Bjerke; Rosendahl, Karen [University of Bergen, Department of Surgical Sciences, Bergen (Norway); Haukeland University Hospital, Department of Radiology, Bergen (Norway); Engesaeter, Ingvild Oevsteboe [University of Bergen, Department of Surgical Sciences, Bergen (Norway); Haukeland University Hospital, Department of Radiology, Bergen (Norway); Haukeland University Hospital, Department of Orthopaedic Surgery, Bergen (Norway); Lehmann, Trude Gundersen; Engesaeter, Lars Birger [University of Bergen, Department of Surgical Sciences, Bergen (Norway); Haukeland University Hospital, Department of Orthopaedic Surgery, Bergen (Norway); Sera, Francesco; Dezateux, Carol [UCL Institute of Child Health, MRC Centre of Epidemiology for Child Health, London (United Kingdom)

    2013-07-15

    Normative references for radiographic measurements commonly used in the diagnosis of developmental dysplasia of the hip at skeletal maturity are incomplete. The present study therefore aimed to establish new gender-specific standards for measurements reflecting the acetabular morphology, namely Sharp's angle, the acetabular roof angle of Toennis (AA) and the acetabular depth-width ratio (ADR), and measurements reflecting the position of the femoral head related to the acetabulum, namely the center-edge (CE) angle of Wiberg, the refined CE angle of Ogata, and the femoral head extrusion index (FHEI). The joint space width (JSW) is also reported. The population-based 1989 Bergen Birth Cohort (n = 3,935) was invited at age 19 years to a follow-up during 2007-09, of which 2,038 (52 %) attended. A standardized antero-posterior radiograph was assessed. The normative references are presented as mean {+-} standard deviation (SD) and 2.5-97.5 percentiles with 95 % confidence intervals. A total of 2,011 (841 males, 1,170 females, mean age 18.6 (SD 0.6)) radiographs were analyzed. Sharp's angle was 38.8 {+-} 3.5 in males and 40.7 {+-} 3.5 in females, with 97.5 percentiles of 46 and 47 , respectively. The CE angle was 32.1 {+-} 6.1 in males and 31.0 {+-} 6.1 in females, with 2.5 percentiles of 21 and 20 , respectively. The FHEI was 86.0 % {+-} 6.3 % in males and 85.6 % {+-} 6.6 % in females, with 2.5 percentiles of 74 and 73 , respectively. Updated gender-specific reference ranges for radiographic measurements commonly used for hip dysplasia at skeletal maturity are reported, similar to or slightly wider than those described in the literature. Statistically significant gender differences have been confirmed for most of the measurements. (orig.)

  7. Reliability of early radiographic evaluations for canine hip dysplasia obtained from the standard ventrodorsal radiographic projection

    International Nuclear Information System (INIS)

    Corley, E.A.; Keller, G.G.; Lattimer, J.C.; Ellersieck, M.R.

    1997-01-01

    To determine reliability of preliminary evaluations for canine hip dysplasia (CHD) performed by the Orthopedic Foundation for Animals on dogs between 3 and 18 months of age. Retrospective analysis of data from the Orthopedic Foundation for Animals database. 2,332 Golden Retrievers, Labrador Retrievers, German Shepherd Dogs, and Rottweilers for which preliminary evaluation had been performed between 3 and 18 months of age and for which results of a definitive evaluation performed after 24 months of age were available. Each radiograph was evaluated, and hip joint status was graded as excellent, good, fair, or borderline phenotype or mild, moderate, or severe dysplasia. Preliminary evaluations were performed by 1 radiologist; definitive evaluations were the consensus of 3 radiologists. Reliability of preliminary evaluations was calculated as the percentage of definitive evaluations (normal vs dysplastic) that were unchanged from preliminary evaluations. Reliability of a preliminary evaluation of normal hip joint phenotype decreased significantly as the preliminary evaluation changed from excellent (100%) to good (97.9%) to fair (76.9%) phenotype. Reliability of a preliminary evaluation of CHD increased significantly as the preliminary evaluation changed from mild (84.4%) to moderate (97.4%) CHD. Reliability of preliminary evaluations increased significantly as age at the time of preliminary evaluation increased, regardless of whether dogs received a preliminary evaluation of normal phenotype or CHD. Results suggest that preliminary evaluations of hip joint status in dogs are generally reliable. However, dogs that receive a preliminary evaluation of fair phenotype of mild CHD should be reevaluated after 24 months of age

  8. Shepherd's Crook Deformity of Polyostotic Fibrous Dysplasia Treated with Corrective Osteotomy and Dynamic Hip Screw

    Directory of Open Access Journals (Sweden)

    Wei-Jen Chen

    2005-07-01

    Full Text Available Fibrous dysplasia, a condition in which the skeleton fails to develop normally, is characterized by fibroblastic stroma and immature bone. Bowing of the long bones occurs frequently in the polyostotic form, and stress fractures often result. Shepherd's crook deformity is a characteristic feature of fibrous dysplasia. The goal of its treatment is to obtain normal walking ability and relieve pain due to pathologic fracture secondary to the deformity; however, correction of the deformity is a surgical challenge. We present 2 cases of shepherd's crook deformity treated with corrective osteotomy and a dynamic hip screw. Both cases showed good bone healing and no recurrent deformity. The gross deformities were corrected, and both patients were pain-free after operation.

  9. Three-dimensional MRI Analysis of Femoral Head Remodeling After Reduction in Patients With Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Tsukagoshi, Yuta; Kamada, Hiroshi; Kamegaya, Makoto; Takeuchi, Ryoko; Nakagawa, Shogo; Tomaru, Yohei; Tanaka, Kenta; Onishi, Mio; Nishino, Tomofumi; Yamazaki, Masashi

    2018-05-02

    Previous reports on patients with developmental dysplasia of the hip (DDH) showed that the prereduced femoral head was notably smaller and more nonspherical than the intact head, with growth failure observed at the proximal posteromedial area. We evaluated the shape of the femoral head cartilage in patients with DDH before and after reduction, with size and sphericity assessed using 3-dimensional (3D) magnetic resonance imaging (MRI). We studied 10 patients with unilateral DDH (all female) who underwent closed reduction. Patients with avascular necrosis of the femoral head on the plain radiograph 1 year after reduction were excluded. 3D MRI was performed before reduction and after reduction, at 2 years of age. 3D-image analysis software was used to reconstruct the multiplanes. After setting the axial, coronal, and sagittal planes in the software (based on the femoral shaft and neck axes), the smallest sphere that included the femoral head cartilage was drawn, the diameter was measured, and the center of the sphere was defined as the femoral head center. We measured the distance between the center and cartilage surface every 30 degrees on the 3 reconstructed planes. Sphericity of the femoral head was calculated using a ratio (the distance divided by each radius) and compared between prereduction and postreduction. The mean patient age was 7±3 and 26±3 months at the first and second MRI, respectively. The mean duration between the reduction and second MRI was 18±3 months. The femoral head diameter was 26.7±1.5 and 26.0±1.6 mm on the diseased and intact sides, respectively (P=0.069). The ratios of the posteromedial area on the axial plane and the proximoposterior area on the sagittal plane after reduction were significantly larger than before reduction (P<0.01). We demonstrated that the size of the reduced femoral head was nearly equal to that of the intact femoral head and that the growth failure area of the head before reduction, in the proximal posteromedial

  10. [Congenital dysplasia of the hip in children of immigrants from developing countries].

    Science.gov (United States)

    Azzoni, R; Sutti, G

    1997-10-01

    Aim of study was to ascertain the possible presence of congenital dysplasia of the hip in infants (CDH) in the population immigrated from countries where the problem is surely neglected from the local health service and estimate its incidence. Moreover to observe the impact of the CDH problem on the parents of these children, sometimes of scarce culture or rather of scarce comprehension of the language. Finally, to verify the answer of parents to the medical prescriptions and to the not simple therapeutic formality. From 1989 to 1996, the hip has been examined by Graf ultrasounds (US) method on a population of 181 infants born from developing countries' immigrants in Italy; 77 of them were Egyptians. Even if the density of the population studied was not very high, the results presented in this paper are innovative and differ from the literature on this matter. Among the 362 hips examined with US, only 5 hips were found to be pathologic (4 Egyptians and 1 Philippine; 2 type uD and 3 type IIC of Graf), and they were treated and recovered with a harm in about 3 months. This work underlines the necessity that all immigrated infants should be studied with US hip exams and that CDH in these people should not be under-evaluated: the very low occurrence of this pathology in developing countries is probably mainly due to scarsity of studies on this matter. This allows not only to avoid hip dislocation in these subjects, but also to the society to avoid the necessary cost of the subsequent treatment of well more serious diseases such as osteoarthritis of the hip on dysplastic basis, in subjects that likely will stay in our country definitely, becoming Italian citizens to all effects.

  11. Hip arthroscopy in patients with recurrent pain following Bernese periacetabular osteotomy for acetabular dysplasia: operative findings and clinical outcomes

    Science.gov (United States)

    Cvetanovich, Gregory L.; Heyworth, Benton E.; Murray, Kerri; Yen, Yi-Meng; Kocher, Mininder S.; Millis, Michael B.

    2015-01-01

    To report the operative findings and outcomes of hip arthroscopy for recurrent pain following periacetabular osteotomy (PAO) for acetabular dysplasia. A departmental database was used to identify patients who underwent hip arthroscopy following PAO between 2000 and 2009. Demographic data, arthroscopic findings, functional outcome scores and patient satisfaction were analysed. Of 556 PAO patients, 17 hips in 16 patients (3.1%) underwent post-PAO hip arthroscopy. Mean age at PAO was 23.8 years, and mean age at arthroscopy was 27.0 years. Common hip arthroscopy findings included labral tears (13 hips, 81.3%), significant (≥grade 2) chondral changes (12 hips, 75%), cam impingement (7 hips, 43.8%) and pincer impingement (6 hips, 37.5%). At mean follow-up 2.8 years after arthroscopy, additional procedures had been performed in six hips (37.5%), including total hip arthroplasty in one hip. Post-PAO arthroscopy questionnaire revealed 85.7% of patients with improved hip pain, 57.1% improved hip stiffness and 57.1% improved hip function. There was no significant difference in functional outcome measures. Common post-PAO hip arthroscopy findings include labral tears, chondral changes and femoroacetabular impingement. Many patients reported subjective hip improvement from post-PAO arthroscopy, but hip outcome scores were unchanged and one-third of patients had further surgery. PMID:27011852

  12. Increased body mass index is a predisposition for treatment by total hip replacement

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig

    2005-01-01

    -joint degeneration and dysplasia. Sequential body mass index (BMI) measurements from 1976 to 1992, age, exposure to daily lifting and hip dysplasia were entered into logistic regression analyses. The prevalence of hip dysplasia ranged from 5.4% to 12.8% depending on the radiographical index used. Radiological hip OA...... prevalence was 1.0--2.5% in subjects or=60 years of age. While radiological OA was significantly influenced by hip dysplasia in men and hip dysplasia and age in women, the risk of THR being performed was only influenced by BMI assessed in 1976. Hip......We investigated the radiological and epidemiological data of 4,151 subjects followed up from 1976 to 2003 to determine individual risk factors for hip osteoarthritis (OA), hip pain and/or treatment by total hip replacement (THR). Pelvic radiographs recorded in 1992 were assessed for evidence of hip...

  13. Evaluation of risk factors for degenerative joint disease associated with hip dysplasia in dogs

    International Nuclear Information System (INIS)

    Smith, G.K.; Popovitch, C.A.; Gregor, T.P.; Shofer, F.S.

    1995-01-01

    Passive coxofemoral joint laxity of dogs, as quantitated by a distraction-stress radiographic method, may have important prognostic value in determining susceptibility to hip dysplasia. Data from 151 dogs, representing 13 breeds, were included in a logistic regression model to evaluate the contribution of factors such as age, breed, weight, sex, distraction index, and Norberg angle to the risk of developing degenerative joint disease (DJD) of the coxofemoral joint. Of the factors studied, the amount of passive hip laxity, as quantitated by the distraction index, was the most significant (P < 0.0001) determinant of the risk to develop DJD of the coxofemoral joint. In the longitudinal and cross-sectional components of the study, distraction index was a significant (P < 0.001) risk factor for DJD, irrespective of age at evaluation (4, 12, or 24 months). The strength of the hip laxity:DJD correlation increased with the age of dog. In contrast, the Norberg angle, a measure of hip laxity on the standard hip-extended radiograph, was not found to be a significant risk factor for DJD, either in the longitudinal or cross-sectional analyses. Breed-specific probability curves of DJD susceptibility indicated that German Shepherd Dogs had a significantly (P < 0.05) greater risk of developing DJD than did the pool of non-German Shepherd Dogs. The information derived from this statistical model will help to scientifically characterize the role of passive hip laxity as a component in the pathogenesis of DJD of the coxofemoral joint

  14. Intra-articular membranous interposition detected by MRI in developmental dysplasia of the hip

    Energy Technology Data Exchange (ETDEWEB)

    Watanabe, W.; Itoi, Eiji; Sato, Kozo [Akita Univ. (Japan). Dept. of Orthopedic Surgery

    2000-12-01

    Intra-articular membranous interposition was detected by MRI in the hip joint with residual subluxation of a girl aged 5 years 10 months. This structure, which had low signal intensity on both T1- and T2-weighted images, separated the femoral head from the acetabulum. Histological examination revealed chondrometaplasia, which suggested that this interposition might be transformed to a surface cartilaginous tissue of the secondary acetabulum often observed in residual subluxation of the hip. (orig.)

  15. Three-dimensional computed tomography analysis of non-osteoarthritic adult acetabular dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Ito, Hiroshi; Matsuno, Takeo; Hirayama, Teruhisa; Tanino, Hiromasa; Yamanaka, Yasuhiro [Asahikawa Medical College, Department of Orthopaedic Surgery, Asahikawa (Japan); Minami, Akio [Hokkaido University School of Medicine, Department of Orthopaedic Surgery, Sapporo (Japan)

    2009-02-15

    Little data exists on the original morphology of acetabular dysplasia obtained from patients without radiographic advanced osteoarthritic changes. The aim of this study was to investigate the distribution and degree of acetabular dysplasia in a large number of patients showing no advanced degenerative changes using three-dimensional computed tomography (3DCT). Eighty-four dysplastic hips in 55 consecutive patients were studied. All 84 hips were in pre- or early osteoarthritis without radiographic evidence of joint space narrowing, formation of osteophytes or cysts, or deformity of femoral heads. The mean age at the time of CT scan was 35 years (range 15-64 years). 3D images were reconstructed and analyzed using recent computer imaging software (INTAGE Realia and Volume Player). Deficiency types and degrees of acetabular dysplasia were precisely evaluated using these computer software. The average Harris hip score at CT scans was 82 points. Twenty-two hips (26%) were classified as anterior deficiency, 17 hips (20%) as posterior deficiency, and 45 hips (54%) as lateral deficiency. No significant difference was found in the Harris hip score among these groups. The analysis of various measurements indicated wide variations. There was a significant correlation between the Harris hip score and the acetabular coverage (p < 0.001). Our results indicated wide variety of deficiency type and degree of acetabular dysplasia. Hips with greater acetabular coverage tended to have a higher Harris hip score. (orig.)

  16. Three-dimensional computed tomography analysis of non-osteoarthritic adult acetabular dysplasia

    International Nuclear Information System (INIS)

    Ito, Hiroshi; Matsuno, Takeo; Hirayama, Teruhisa; Tanino, Hiromasa; Yamanaka, Yasuhiro; Minami, Akio

    2009-01-01

    Little data exists on the original morphology of acetabular dysplasia obtained from patients without radiographic advanced osteoarthritic changes. The aim of this study was to investigate the distribution and degree of acetabular dysplasia in a large number of patients showing no advanced degenerative changes using three-dimensional computed tomography (3DCT). Eighty-four dysplastic hips in 55 consecutive patients were studied. All 84 hips were in pre- or early osteoarthritis without radiographic evidence of joint space narrowing, formation of osteophytes or cysts, or deformity of femoral heads. The mean age at the time of CT scan was 35 years (range 15-64 years). 3D images were reconstructed and analyzed using recent computer imaging software (INTAGE Realia and Volume Player). Deficiency types and degrees of acetabular dysplasia were precisely evaluated using these computer software. The average Harris hip score at CT scans was 82 points. Twenty-two hips (26%) were classified as anterior deficiency, 17 hips (20%) as posterior deficiency, and 45 hips (54%) as lateral deficiency. No significant difference was found in the Harris hip score among these groups. The analysis of various measurements indicated wide variations. There was a significant correlation between the Harris hip score and the acetabular coverage (p < 0.001). Our results indicated wide variety of deficiency type and degree of acetabular dysplasia. Hips with greater acetabular coverage tended to have a higher Harris hip score. (orig.)

  17. Hip2Norm: an object-oriented cross-platform program for 3D analysis of hip joint morphology using 2D pelvic radiographs.

    Science.gov (United States)

    Zheng, G; Tannast, M; Anderegg, C; Siebenrock, K A; Langlotz, F

    2007-07-01

    We developed an object-oriented cross-platform program to perform three-dimensional (3D) analysis of hip joint morphology using two-dimensional (2D) anteroposterior (AP) pelvic radiographs. Landmarks extracted from 2D AP pelvic radiographs and optionally an additional lateral pelvic X-ray were combined with a cone beam projection model to reconstruct 3D hip joints. Since individual pelvic orientation can vary considerably, a method for standardizing pelvic orientation was implemented to determine the absolute tilt/rotation. The evaluation of anatomically morphologic differences was achieved by reconstructing the projected acetabular rim and the measured hip parameters as if obtained in a standardized neutral orientation. The program had been successfully used to interactively objectify acetabular version in hips with femoro-acetabular impingement or developmental dysplasia. Hip(2)Norm is written in object-oriented programming language C++ using cross-platform software Qt (TrollTech, Oslo, Norway) for graphical user interface (GUI) and is transportable to any platform.

  18. Results of ultrasound screening of the hips in newborns and infants

    Directory of Open Access Journals (Sweden)

    Džoleva-Tolevska Roza

    2012-01-01

    Full Text Available The aim of this study is to analyze the results of ultrasound screening of the hips in newborns and infants and to establish the importance of ultrasonography in early diagnosis and treatment of developmental dysplasia of the hips (DDH. Material and Methods: In 2010, at the Clinic for orthopedic surgery in Skopje, 6333 newborns and infants were examined. They were classified in 2 groups: first group consisted of patients with normal ultrasound findings and second group consisted of patients with DDH on ultrasound finding. Patients underwent clinical examination and ultrasonography of the hips. Results: We examined 6333 newborns and infants up to 6 months of age. 3213 were female and 3120 were male. In the first group there were 5932 (93.67% patients with normal ultrasound of the hip-Graf Type 1. In the second group there were 401 (6.33% patients with DDH on ultrasound. The patients of the second group were divided in 3 types according to Graf method. Graf Type 2-Patients with dysplasia 378 (5.97% subdivided in 2a- 260 (4.11% patients, 2b 85 (1.34% patients and 2c 33 (0.52% patients. Graf Type 3 - Patients with subluxation of the hip 9 (0.1%, subdivided in 3a 3 (0.05% patients and 3b 3 (0.5% patients. Graf Type 4 -Patients with luxation of the hip 17 (0.27% patients. 124 patients (30.5% with DDH had an associated risk factors (65 patients with positive family history, 48 patients with breech delivery and 11 patients with clubfoot deformity. 387 patients with dysplasia and subluxation of the hips were treated with abduction brace and Pavlik harness. 17 patients with luxation of the hips were treated with exercises and overhead traction of the muscles, close reduction of the hip placed in spica cast or open reduction. Conclusion: Ultrasound screening of hips in newborns and infants is important for early diagnosis of DDH. This is necessary for adequate treatments. If this disease is not treated properly it gives long term morbidity such as gait

  19. Ultrasonography of the pediatric hip and spine

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Yeo Ryang; Koo, Joon Bun [Dept. of Radiology, Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang (Korea, Republic of)

    2017-07-15

    Ultrasonography (US) is a useful screening method for the diagnosis of developmental dysplasia of the hip (DDH) and congenital spinal anomalies in infants. In addition, US is a useful, noninvasive imaging modality for the diagnosis of transient synovitis in children. The early detection of DDH leads to a better prognosis without surgical intervention. Moreover, spinal US allows the detection of congenital malformations and can also demonstrate normal anatomy and normal variants that may resemble disorders. Therefore, radiologists should be familiar with the sonographic anatomy of the normal infant hip, scanning and measurement techniques for DDH, the US features of transient synovitis in children, spinal US techniques, and the US features of normal anatomical structures, normal variants, and congenital spinal anomalies.

  20. Ultrasonography of the pediatric hip and spine

    International Nuclear Information System (INIS)

    Kang, Yeo Ryang; Koo, Joon Bun

    2017-01-01

    Ultrasonography (US) is a useful screening method for the diagnosis of developmental dysplasia of the hip (DDH) and congenital spinal anomalies in infants. In addition, US is a useful, noninvasive imaging modality for the diagnosis of transient synovitis in children. The early detection of DDH leads to a better prognosis without surgical intervention. Moreover, spinal US allows the detection of congenital malformations and can also demonstrate normal anatomy and normal variants that may resemble disorders. Therefore, radiologists should be familiar with the sonographic anatomy of the normal infant hip, scanning and measurement techniques for DDH, the US features of transient synovitis in children, spinal US techniques, and the US features of normal anatomical structures, normal variants, and congenital spinal anomalies

  1. Localised form of spondylo-epiphyseal dysplasia congenita

    International Nuclear Information System (INIS)

    Hoeffel, J.C.; Mohy, R.; Collignon, P.; Moog, G.

    1988-01-01

    We report an unusual case of spondylo-epiphyseal dysplasia congenita which affected only the hips and the thoraco-lumbar spine. The epiphysis of the long bones are normal apart from the hips. Our child has a bilateral epiphyseal dysplasia of both proximal femoral epiphysis discovered incidentally at 11 months and confirmed later on at 8 years, associated with abnormalities of the superior margin of the vertebral bodies from T11 to L2. Very few similar cases have been reported anteriorly. (orig.) [de

  2. Acetabular Dysplasia and Surgical Approaches Other Than Direct Anterior Increases Risk for Malpositioning of the Acetabular Component in Total Hip Arthroplasty

    DEFF Research Database (Denmark)

    Gromov, Kirill; Greene, Meridith E; Huddleston, James I

    2016-01-01

    BACKGROUND: Persistent acetabular dysplasia (AD) after periacetabular osteotomy has been hypothesized to increase the risk for malpositioning of the acetabular component. In this study, we investigate whether AD is an independent risk factor for cup malpositioning during primary total hip...... arthroplasty (THA). METHODS: Patient demographics, surgical approach, presence of AD assessed using the lateral center-edge angle, and acetabular cup positioning determined using Martell Hip Analysis Suite were investigated in 836 primary THA patients enrolled in a prospective multicenter study. RESULTS: We...

  3. Evaluation of articular cartilage following rotational acetabular osteotomy for hip dysplasia using T2 mapping MRI.

    Science.gov (United States)

    Shoji, Takeshi; Yamasaki, Takuma; Izumi, Soutaro; Sawa, Mikiya; Akiyama, Yuji; Yasunaga, Yuji; Adachi, Nobuo

    2018-04-27

    Rotational acetabular osteotomy (RAO) is one of the surgical treatments for acetabular dysplasia, and satisfactory results have been reported. We evaluated the postoperative changes of articular cartilage and whether the pre-operative condition of the articular cartilage influences the clinical results using T2 mapping MRI. We reviewed 31 hips with early stage osteoarthritis in 31 patients (mean age, 39.6 years), including three men and 28 women who underwent RAO for hip dysplasia. Clinical evaluations including Japanese Orthopedic Association (JOA) score and Japanese Orthopedic Association Hip Disease Evaluation Questionnaire (JHEQ), and radiographical evaluations on X-ray were performed. Longitudinal qualitative assessment of articular cartilage was also performed using 3.0-T MRI with T2 mapping technique preoperatively, 6 months, and at 1 and 2 years postoperatively. There was no case with progression of osteoarthritis. The mean JOA score improved from 70.1 to 93.4 points, the mean postoperative JHEQ score was 68.8 points, and radiographical data also improved postoperatively. We found that the T2 values of the cartilage at both femoral head and acetabulum increased at 6 months on coronal and sagittal views. However, they significantly decreased 1 and 2 years postoperatively. The T2 values of the center to anterolateral region of acetabulum negatively correlated with postoperative JHEQ score, particularly in pain score. This study suggests that biomechanical and anatomical changes could apparently cause decreased T2 values 1-2 years postoperatively compared with those preoperatively. Furthermore, preoperative T2 values of the acetabulum can be prognostic factors for the clinical results of RAO.

  4. The history of biomechanics in total hip arthroplasty

    Directory of Open Access Journals (Sweden)

    Jan Van Houcke

    2017-01-01

    Full Text Available Biomechanics of the hip joint describes how the complex combination of osseous, ligamentous, and muscular structures transfers the weight of the body from the axial skeleton into the appendicular skeleton of the lower limbs. Throughout history, several biomechanical studies based on theoretical mathematics, in vitro, in vivo as well as in silico models have been successfully performed. The insights gained from these studies have improved our understanding of the development of mechanical hip pathologies such as osteoarthritis, hip fractures, and developmental dysplasia of the hip. The main treatment of end-stage degeneration of the hip is total hip arthroplasty (THA. The increasing number of patients undergoing this surgical procedure, as well as their demand for more than just pain relief and leading an active lifestyle, has challenged surgeons and implant manufacturers to deliver higher function as well as longevity with the prosthesis. The science of biomechanics has played and will continue to play a crucial and integral role in achieving these goals. The aim of this article, therefore, is to present to the readers the key concepts in biomechanics of the hip and their application to THA.

  5. Comparison of susceptibility for hip dysplasia between Rottweilers and German Shepherd Dogs

    International Nuclear Information System (INIS)

    Popovitch, C.A.; Smith, G.K.; Gregor, T.P.; Sholer, F.S.

    1995-01-01

    Passive laxity of the coxofemoral joints, as measured quantitatively by radiographing the joints under stress, has been shown to be an accurate measure of the risk for developing degenerative joint disease (DJD) of the coxofemoral joints. Seventy-four Rottweilers between 12 and 40 months old were evaluated subjectively for radiographic evidence of DJD, using the ventrodorsal view of the pelvis with the coxofemoral joints fully extended and the knees internally rotated (standard hip-extended view). Effect of age, sex, weight, and distraction index on the risk of developing DJD was evaluated by use of a logistic regression model. Results were compared with those from a group of German Shepherd Dogs. Results indicated that in Rottweilers the distraction index was the only statistically significant predictor of the risk of developing DJD of the coxofemoral joint. When German Shepherd Dogs were included in the model, they had a significantly greater risk of developing DJD than did Rottweilers. This finding provides further support for the theory that there are differences in disease susceptibility among breeds and emphasizes the need to develop disease susceptibility curves for all breeds affected by hip dysplasia to facilitate accurate, scientifically based recommendations for breeding or treatment

  6. Degeneration in dysplastic hips. A computer tomography study

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Rømer, Lone; Søballe, Kjeld

    2005-01-01

    BACKGROUND: Hip dysplasia is considered pre-osteoarthritic, causing degeneration in young individuals. OBJECTIVE: To determine the pattern of degenerative change in moderate to severely dysplastic hips in young patients. DESIGN AND PATIENTS: One hundred and ninety-three consecutively......-referred younger patients with hip pain believed to be caused by hip dysplasia constituted the study cohort. The average age was 35.5 years (range, 15-61 years). They were examined by close-cut transverse pelvic and knee computed tomography and antero-posterior radiographs (CT). We identified 197 hips...

  7. Increased body mass index is a predisposition for treatment by total hip replacement

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig

    2005-01-01

    We investigated the radiological and epidemiological data of 4,151 subjects followed up from 1976 to 2003 to determine individual risk factors for hip osteoarthritis (OA), hip pain and/or treatment by total hip replacement (THR). Pelvic radiographs recorded in 1992 were assessed for evidence of hip......-joint degeneration and dysplasia. Sequential body mass index (BMI) measurements from 1976 to 1992, age, exposure to daily lifting and hip dysplasia were entered into logistic regression analyses. The prevalence of hip dysplasia ranged from 5.4% to 12.8% depending on the radiographical index used. Radiological hip OA...

  8. Hip pathology in Majewski osteodysplastic primordial dwarfism type II.

    Science.gov (United States)

    Karatas, Ali F; Bober, Michael B; Rogers, Kenneth; Duker, Angela L; Ditro, Colleen P; Mackenzie, William G

    2014-09-01

    Majewski osteodysplastic primordial dwarfism type II (MOPDII) is characterized by severe prenatal and postnatal growth failure with microcephaly, characteristic skeletal dysplasia, an increased risk for cerebrovascular disease, and insulin resistance. MOPDII is caused by mutations in the pericentrin (PCNT) gene and is inherited in an autosomal-recessive manner. This study aimed to determine the incidence of hip pathology in patients with molecularly confirmed MOPDII and to describe the functional outcomes of surgical treatment. Thirty-three enrolled patients had a clinical diagnosis of MOPDII. Biallelic PCNT mutations or absent pericentrin protein was confirmed in 25 of these patients. Twelve patients (7 female) had appropriate clinical and radiographic records at this institution and were included in this study. The data collected included age at presentation, age at surgery, sex, body weight and height, weight-bearing status at diagnosis, and the clinical examination. Four patients (31%) had coxa vara: 3 unilateral and 1 bilateral. Three unilateral patients had in situ pinning at a mean age 4 years. The patient with bilateral coxa vara had valgus osteotomy at the age of 5 years. Two children had bilateral hip dysplasia and subluxation with no surgery. One patient had bilateral developmental hip dislocations. The patient was treated by open reduction-spica cast and 2 years after surgery, coxa valga was noted. Another patient was diagnosed at an age of 12 years with bilateral avascular necrosis of the hips. Four patients did not have hip pathology. Hip pathology is common among children with MOPDII; coxa vara is the most frequent diagnosis. Routine clinical and radiographic hip evaluation is important. The capital femoral epiphysis appears to slip down along the shaft, giving the appearance of a proximal femoral epiphysiolysis. A hip diagnosed with slipped capital femoral epiphysis in early life may progress to severe coxa vara. Level IV.

  9. [Efficacy analysis on hip replacement for hip-joint diseases with Parkinson disease].

    Science.gov (United States)

    Sun, Qi-Cai; Ru, Xuan-Liang; Xia, Yan-Fei; Liu, Xiao-Li; Song, Bai-Shan; Qiao, Song; Yan, Shi-Gui; Wang, Xiang-Hua

    2017-12-25

    To explore clinical efficacy of hip replacement for hip-joint diseases with Parkinson disease. From December 2011 to December 2016, 18 patients with hip-joint diseases with Parkinson disease treated by hip replacement, including 8 males and 10 females aged from 59 to 87 years old with an average of 71 years old. Among them, 3 cases were developmental dysplasia of hip, 3 cases were femoral head necrosis and 12 cases were femoral neck fracture. All patients manifested with obvious pain and limitation of stepping ability. Postoperative complications were observed and Harris score were used to compare hip joint function after operation. The incision were healed well, and pain were alleviated or disappeared, and hip joint function were improved. Eighteen patients were followed up from 1 to 3 years with an average of 2.3 years. At the latest follow up, 14 cases recovered freedom-walk, 2 cases could walk with walking stick, 1 case could walk with walking aid and 1 case was died. Among 18 patients, 2 cases were occurred dislocation, and 1 case were died for cardiac disease at 3 months after operation. Four patients were occurred slight pain. There were significant differences in Harris scores among preoperative (41.7±1.4), 6 months after operation(80.1±5.4) and the final follow-up (83.4±2.1), and 10 cases got excellent result, 4 good, 1 fair and 2 poor. Application of hip replacement for hip-joint diseases with Parkinson disease is a safe and effective clinical therapy, and has advantages of less complications and rapid recovery of hip joint function.

  10. Radiographic Prevalence of Dysplasia, Cam, and Pincer Deformities in Elite Ballet.

    Science.gov (United States)

    Harris, Joshua D; Gerrie, Brayden J; Varner, Kevin E; Lintner, David M; McCulloch, Patrick C

    2016-01-01

    The demands of hip strength and motion in ballet are high. Hip disorders, such as cam and pincer deformities or dysplasia, may affect dance performance. However, the prevalence of these radiographic findings is unknown. To determine the prevalence of radiographic cam and pincer deformities, borderline dysplasia, and dysplasia in a professional ballet company. Cross-sectional study; Level of evidence, 3. An institutional review board-approved cross-sectional investigation of a professional ballet company was undertaken. Male and female adult dancers were eligible for inclusion. Four plain radiographs were obtained (standing anteroposterior pelvis, bilateral false profile, and supine Dunn 45°) and verified for adequacy. Cam and pincer deformities, dysplasia, borderline dysplasia, and osteoarthritis were defined. All plain radiographic parameters were measured and analyzed on available radiographs. Student t test, chi-square test (and Fisher exact test), and Spearman correlation analyses were performed to compare sexes, groups, and the effect of select radiographic criteria. A total of 47 dancers were analyzed (21 males, 26 females; mean age (±SD), 23.8 ± 5.4 years). Cam deformity was identified in 25.5% (24/94) of hips and 31.9% (15/47) of subjects, with a significantly greater prevalence in male dancers than females (48% hips and 57% subjects vs 8% hips and 12% subjects; P ballet company, a high prevalence of radiographic abnormalities was found, including cam and pincer deformity and dysplasia. The results also revealed several sex-related differences of these abnormalities in this unique population. The long-term implications of these findings in this group of elite athletes remain unknown, and this issue warrants future investigation. © 2015 The Author(s).

  11. Radiographic parameters of the hip joint from birth to adolescence

    Energy Technology Data Exchange (ETDEWEB)

    Than, P.; Kranicz, J.; Bellyei, A. [Dept. of Orthopaedics, Univ. of Pecs, Medical Faculty, Ifjusag utja (Hungary); Sillinger, T. [Dept. of Orthopaedics, Szt Gyorgy County Hospital, Szekesfehervar (Hungary)

    2004-03-01

    Background: Various qualitative and quantitative radiological geometrical parameters can be of great help when assessing dysplasia of the hip joint and in understanding developmental processes of the infant hip. There are few data on the normal values of the hip joint at different ages. Objective: To perform radiographic measurements on hip joints considered to be anatomically normal and to provide data for each age group, thus describing features of the radiographic development of the hip. Materials and methods: Radiographs were examined from 355 children (age 0-16 years) undergoing examination for scoliosis (long film), urography or plain abdominal radiography. Qualitative and quantitative signs were observed and measured, focusing on the Hilgenreiner, Wiberg and Idelberger angles and the decentric distance. Results: Before the age of 9 years measurable data from neighbouring age groups differed significantly, indicating typical radiological changes of the joint. For the same age range, qualitative changes could also be observed. After 9 years of age, radiological development of the normal hip joint during childhood is much slower. (orig.)

  12. Minimum Lateral Bone Coverage Required for Securing Fixation of Cementless Acetabular Components in Hip Dysplasia

    Directory of Open Access Journals (Sweden)

    Masanori Fujii

    2017-01-01

    Full Text Available Objectives. To determine the minimum lateral bone coverage required for securing stable fixation of the porous-coated acetabular components (cups in hip dysplasia. Methods. In total, 215 primary total hip arthroplasties in 199 patients were reviewed. The average follow-up period was 49 months (range: 24–77 months. The lateral bone coverage of the cups was assessed by determining the cup center-edge (cup-CE angle and the bone coverage index (BCI from anteroposterior pelvic radiographs. Further, cup fixation was determined using the modified DeLee and Charnley classification system. Results. All cups were judged to show stable fixation by bone ingrowth. The cup-CE angle was less than 0° in 7 hips (3.3% and the minimum cup-CE angle was −9.2° (BCI: 48.8%. Thin radiolucent lines were observed in 5 hips (2.3%, which were not associated with decreased lateral bone coverage. Loosening, osteolysis, dislocation, or revision was not observed in any of the cases during the follow-up period. Conclusion. A cup-CE angle greater than −10° (BCI > 50% was acceptable for stable bony fixation of the cup. Considering possible errors in manual implantation, we recommend that the cup position be planned such that the cup-CE angle is greater than 0° (BCI > 60%.

  13. The Fate of DDH Hips Showing Cartilaginous or Fibrous Tissue-filled Joint Spaces Following Primary Reduction.

    Science.gov (United States)

    Kim, Hui Taek; Lee, Tae Hoon; Ahn, Tae Young; Jang, Jae Hoon

    Because the use of magnetic resonance imaging is still not universal for the patients with developmental dysplasia of the hip patients, orthopaedists do not generally distinguish widened joint spaces which are "empty" after primary treatment (and therefore still reducible), from those which are filled and much more difficult to treat. To date no studies have focused on the latter hips. We treated and observed the outcomes for 19 hips which showed filled joint spaces after primary treatment. We retrospectively reviewed 19 cases of developmental dysplasia of the hip: (1) who showed a widened joint space on radiographs after primary treatment; and (2) whose magnetic resonance imaging showed that the widened joint space was accompanied by acetabular cartilage hypertrophy and/or was filled with fibrous tissues. All patients were over 1 year old at the time of primary reduction (reduction was closed in 4 patients, open in 6, and open with pelvic osteotomy in 9). Thirteen patients received at least 1 secondary treatment. Final results were classified using a modified Severin classification. Final outcomes were satisfactory in 10 (52.6%) and unsatisfactory in 9 (47.4%). The widened joint spaces gradually filled with bone, resulting in a shallow acetabulum in the patients with unsatisfactory results. Of 9 patients who underwent combined pelvic osteotomy at the time of primary reduction, results were satisfactory in 6 (66.7%), whereas all patients who had only closed or open primary reduction had unsatisfactory results. Combined pelvic osteotomy at the time of primary reduction is advisable in hips with widened joint spaces. However, hips with filled joint spaces after primary treatment often have unsatisfactory results even after additional pelvic and/or femoral osteotomy. Level IV-prognostic study.

  14. Medial versus anterior open reduction for developmental hip dislocation in age-matched patients.

    Science.gov (United States)

    Hoellwarth, Jason S; Kim, Young-Jo; Millis, Michael B; Kasser, James R; Zurakowski, David; Matheney, Travis H

    2015-01-01

    The difference between medial (MAOR) and anterior (AAOR) approaches for open reduction of developmental hip dysplasia in terms of risk for avascular necrosis (AVN) and need for further corrective surgery (FCS, femoral and/or acetabular osteotomy) is unclear. This study compared age-matched cohorts undergoing either MAOR or AAOR in terms of these 2 primary outcomes. Prognostic impact of presence of ossific nucleus at time of open reduction was also investigated. Institutional review board approval was obtained. Nineteen hips (14 patients) managed by MAOR were matched with 19 hips (18 patients) managed by AAOR based on age at operation (mean 6.0; range, 1.4 to 14.9 mo). Patients with neuromuscular conditions and known connective tissue disorders were excluded. Primary outcomes assessed at minimum 2 years' follow-up included radiographic evidence of AVN (Kalamchi and MacEwen) or requiring FCS. MAOR and AAOR cohorts were similar regarding age at open reduction, sex, laterality, and follow-up duration. One hip in each group had AVN before open reduction thus were excluded from AVN analysis. At minimum 2 years postoperatively (mean 6.2; range, 1.8 to 11.7 y), 4/18 (22%) MAOR and 5/18 (28%) AAOR met the same criteria for AVN (P=1.0). No predictors of AVN could be identified by regression analysis. Presence of an ossific nucleus preoperatively was not a protective factor from AVN (P=0.27). FCS was required in 4/19 (21%) MAOR and 7/19 (37%) AAOR hips (P=0.48). However, 7/12 (54%) hips failing closed reduction required FCS compared with 4/26 (16%) hips without prior failed closed reduction (P=0.024). Cox regression analysis showed that patients who failed closed reduction had an annual risk of requiring FCS approximately 6 times that of patients without a history of failed closed reduction (hazard ratio=6.1; 95% CI, 1.5-24.4; P=0.009), independent of surgical approach (P=0.55) or length of follow-up (P=0.78). In this study of age-matched patients undergoing either MAOR or

  15. Retrospective radiographic evaluation of treatment results of developmental dysplasia of the hip in walking-age children

    NARCIS (Netherlands)

    Heesakkers, Nicole A. M.; Witbreuk, Melinda M. E. H.; Besselaar, Philip P.; van der Sluijs, Johannes A.

    2013-01-01

    We evaluated treatment results of 22 children (32 hips) with idiopathic hip dislocation after walking age in two Dutch academic hospitals. The Tonnis classification was used preoperatively. Outcome was measured using the Severin and Kalamchi classification. The mean age at treatment was 24 months

  16. THE SYSTEM OF TREATMENT OF HIP DYSPLASIA IN CHILDREN (CONCEPTION OF SRICO N.A. H.I. TURNER

    Directory of Open Access Journals (Sweden)

    Mikhail Mikhailovich Kamosko

    2013-03-01

    Full Text Available The system of treatment of hip dysplasia that is developed, applied and perfected at FSBI "Scientific and Research Institute for Children's Orthopedics n.a. H. Turner" involves the early entirely functional treatment (Lorenz method, other methods are similar in gist, providing immobilization with a cast or other devices, are not applied. The operative therapy is performed in primary patients with intrauterine or teratogenic dislocations of the hip. Technology of operative treatment is extremely gentle, sparing. Operations on deepening of socket, iatrogenic affection on cartilage cover, capsular arthroplasty are excluded from the arsenal of surgeons. Anatomy of a children’s dysplastic of hip joints (not subjected to inappropriate conservative or surgical treatment has several fairly standard options. For each option, there is a combination of classical operational techniques (tenomyotomy of lumbar-iliac muscle, capsulotomy, detorsion, varization, shortening, medialization, changes in the spatial orientation of the arch or of the whole acetabulum, which have proven on the vast clinical material to be very effective with the right technical performance.

  17. The fate of the hip in spondylo-epi-metaphyseal dysplasia: clinical and radiological evaluation of adults with SEMD Handigodu type

    Energy Technology Data Exchange (ETDEWEB)

    Siddesh, N.D.; Shah, Hitesh; Joseph, Benjamin [Kasturba Medical College, Paediatric Orthopaedic Service, Manipal, Karnataka State (India)

    2012-08-15

    This study was undertaken to document the fate of the hip with reference to its structure and function in patients with spondylo-epi-metaphyseal dysplasia tarda Handigodu type (SEMD{sub HG}). Radiographs of 271 adult patients with SEMD{sub HG} were studied to identify the pattern of long-term sequelae in the hips. Several measurements of the proximal femur and acetabulum were made to quantify morphological alterations in the hip. Fifty-four adult patients were examined and administered a questionnaire to evaluate the extent of disability attributable to the hips. Three patterns of changes in the hips were noted: 35% had acetabular protrusio, 33% had subluxation of the hip, and 32% had no protrusio or subluxation. Distinctly different anthropometric measurements and dimensional alterations around the hip were noted in these three patterns. Patients with protrusio were relatively tall while those with subluxation were the shortest. All the patients had developed degenerative arthritis of the hips by the fourth decade of life irrespective of the pattern of hip involvement. The reduction in the range of hip motion and fixed deformities were most severe in patients with protrusio. All the patients had significant disability and very low functional hip scores. Degenerative arthritis of the hip develops in the majority of patients with SEMD{sub HG}; the symptoms are severe enough to warrant reconstructive surgery by the fourth decade of life. Protrusio or subluxation develops in a third of the patients each; both these complications will influence the surgical approach if total hip arthroplasty is planned. (orig.)

  18. The fate of the hip in spondylo-epi-metaphyseal dysplasia: clinical and radiological evaluation of adults with SEMD Handigodu type

    International Nuclear Information System (INIS)

    Siddesh, N.D.; Shah, Hitesh; Joseph, Benjamin

    2012-01-01

    This study was undertaken to document the fate of the hip with reference to its structure and function in patients with spondylo-epi-metaphyseal dysplasia tarda Handigodu type (SEMD HG ). Radiographs of 271 adult patients with SEMD HG were studied to identify the pattern of long-term sequelae in the hips. Several measurements of the proximal femur and acetabulum were made to quantify morphological alterations in the hip. Fifty-four adult patients were examined and administered a questionnaire to evaluate the extent of disability attributable to the hips. Three patterns of changes in the hips were noted: 35% had acetabular protrusio, 33% had subluxation of the hip, and 32% had no protrusio or subluxation. Distinctly different anthropometric measurements and dimensional alterations around the hip were noted in these three patterns. Patients with protrusio were relatively tall while those with subluxation were the shortest. All the patients had developed degenerative arthritis of the hips by the fourth decade of life irrespective of the pattern of hip involvement. The reduction in the range of hip motion and fixed deformities were most severe in patients with protrusio. All the patients had significant disability and very low functional hip scores. Degenerative arthritis of the hip develops in the majority of patients with SEMD HG ; the symptoms are severe enough to warrant reconstructive surgery by the fourth decade of life. Protrusio or subluxation develops in a third of the patients each; both these complications will influence the surgical approach if total hip arthroplasty is planned. (orig.)

  19. Genomic prediction of traits related to canine hip dysplasia

    Directory of Open Access Journals (Sweden)

    Enrique eSanchez-Molano

    2015-03-01

    Full Text Available Increased concern for the welfare of pedigree dogs has led to development of selection programs against inherited diseases. An example is canine hip dysplasia (CHD, which has a moderate heritability and a high prevalence in some large-sized breeds. To date, selection using phenotypes has led to only modest improvement, and alternative strategies such as genomic selection may prove more effective. The primary aims of this study were to compare the performance of pedigree- and genomic-based breeding against CHD in the UK Labrador retriever population and to evaluate the performance of different genomic selection methods. A sample of 1179 Labrador Retrievers evaluated for CHD according to the UK scoring method (hip score, HS was genotyped with the Illumina CanineHD BeadChip. Twelve functions of HS and its component traits were analyzed using different statistical methods (GBLUP, Bayes C and Single-Step methods, and results were compared with a pedigree-based approach (BLUP using cross-validation. Genomic methods resulted in similar or higher accuracies than pedigree-based methods with training sets of 944 individuals for all but the untransformed HS, suggesting that genomic selection is an effective strategy. GBLUP and Bayes C gave similar prediction accuracies for HS and related traits, indicating a polygenic architecture. This conclusion was also supported by the low accuracies obtained in additional GBLUP analyses performed using only the SNPs with highest test statistics, also indicating that marker-assisted selection would not be as effective as genomic selection. A Single-Step method that combines genomic and pedigree information also showed higher accuracy than GBLUP and Bayes C for the log-transformed HS, which is currently used for pedigree based evaluations in UK. In conclusion, genomic selection is a promising alternative to pedigree-based selection against CHD, requiring more phenotypes with genomic data to improve further the accuracy

  20. Triple pelvic osteotomy in the treatment of hip dysplasia

    Directory of Open Access Journals (Sweden)

    Vukašinović Zoran

    2009-01-01

    coverage (compared to Salter osteotomy. Conclusion. Triple pelvic osteotomy is the method of choice in the management of acetabular dysplasia and other disturbances of hip joint containment in adolescent age.

  1. Hip Joint Osteochondroma: Systematic Review of the Literature and Report of Three Further Cases

    Directory of Open Access Journals (Sweden)

    Asim M. Makhdom

    2014-01-01

    Full Text Available The aim of this study is to systematically review the literature with regards to surgical treatment of patients with hip joint osteochondromas, and to report our surgical management of three paediatric patients who had femoral neck or acetabular osteochondromas in association with acetabular dysplasia. We performed a systematic review using PubMed and Embase databases for all studies that reported surgical treatments for patients with peritrochanteric or acetabular osteochondroma with or without acetabular dysplasia. We also retrospectively reviewed three patients who were diagnosed with a hip osteochondroma in association with actetabular dysplasia. These patients were known to have hereditary multiple exostoses (HME. The systematic review revealed 21 studies that met our inclusion criteria. All studies were case reports and retrospective in nature and failed to conclude a uniform treatment plan. The three reported cases illustrate successful excision of hip osteochondromas and treatment of acetabular dysplasia. Early excision of hip osteochondromas might prevent acetabular dysplasia in HME patients. Routine radiographic pelvic survey at the time of diagnosis of HME is recommended for early detection of hip osteochondromas and acetabular dysplasia in these children.

  2. Hip morphologic measurements in an Egyptian population.

    Science.gov (United States)

    Aly, Tarek A

    2011-04-11

    The study of acetabular morphology has shown that there are geographic differences in the morphology and prevalence of acetabular dysplasia among different ethnic groups. However, few data exist on the shape of the acetabulum in various populations around the world. In this study, we examined samples of pelvic radiographs from Egyptian adults. Acetabular dysplasia in adults is characterized by a shallow and relatively vertical acetabulum.The aim of this study was to examine acetabular morphology to determine the prevalence of hip dysplasia in adult Egyptians. This included 244 adults, 134 men and 110 women between 18 and 60 years, who were used to measure center edge angle, acetabular Sharp angle, acetabular head index on anteroposterior radiographic views of the hip joints, and vertical center anterior margin angle on false profile views. The radiographs were taken of patients with no hip complaints at Tanta University Hospital.The results were statistically studied according to the age, height, and weight of patients. The prevalence of acetabular dysplasia was 2.25% for Egyptian men and 3.6% for women with respect to center edge angles, vertical center anterior margin angle, and acetabular head index.We concluded that gender variations in the morphology of the acetabulum and sex influences geometrical measurements of the acetabulum. Egyptian women were more dysplastic than men using the 4 parameters of hip measurements. There are also racial variations in hip morphology. Copyright 2011, SLACK Incorporated.

  3. De novo interstitial deletion of 9q32-34.1 with mental retardation, developmental delay, epilepsy, and cortical dysplasia

    DEFF Research Database (Denmark)

    Tos, T; Alp, M Y; Karacan, C D

    2014-01-01

    In this report we describe a 10 year-old female patient with interstitial deletion of 9q32-q34.1 associated with mental retardation, developmental delay, short stature, mild facial dysmorphism, epilepsy, abnormal EEG and brain MRI findings consistent with focal cortical dysplasia. Interstitial...

  4. Hip sonography in the newborn

    International Nuclear Information System (INIS)

    Riboni, G.; Serantoni, S.; De Simoni, M.; Bascape', P.; Facchini, R.; Pirovano, G.

    1991-01-01

    The authors report the data relative to 1507 cases studied with clinical and US examinations, in the neonatal period, in order to exclude hip dysplasia dislocation. US examination was carried out according to Graf's technique and the newborns were classified according to US hip type, to clinical examination and to possible risk factors. The patients were included in a protocol including orthopedic and US controls. Seventeen treated infants were considered as pathologic. Ten of them had IIc or D hips ar birth; the other 7, with IIa hips at birth, presented a X-ray pathologic hip after the 4th months of life. At about one year of age all infants could normally walk, excpet for one who was being treated with herness. No statistically significant differences were observed between the number of pathologic infants in the risk group (1.7%) and that in the no-risk group (0.8%). Clinical examination of the newborn has low sensitivity in detecting pathologic hips. On the basis of their results, thw authors belive US examination of the newborn to be a valuable screening method to diagnose hip dysplasia/dislocation. Moreover, Graf's morphologic method is the best one for US screening of the hip in the neonatal period

  5. MR imaging findings of acetabular dysplasia in adults

    Energy Technology Data Exchange (ETDEWEB)

    James, Steven; Connell, David [The Royal National Orthopaedic Hospital, Radiology Department, London, Middlesex (United Kingdom); Miocevic, Miranda; Malara, Frank; Pike, Jonathan [Victoria House Hospital, Radiology Department, Melbourne (Australia); Young, David [Melbourne Orthopaedic Group, Orthopaedic Surgery, Melbourne (Australia)

    2006-06-15

    To evaluate the diagnostic accuracy of MR imaging in the identification of labral and articular cartilage lesions in patients with acetabular dysplasia. Pre-operative MR imaging was performed on 27 hips in 25 consecutive patients (16 males, 9 females, age range 19-52 years, mean age 31.2 years) with radiographic evidence of acetabular dysplasia (centre-edge angle of Wiberg <20 degrees). The average duration of symptoms was 16.2 months. Two musculoskeletal radiologists assessed MR images in consensus for the presence of abnormality involving the acetabular labrum and adjacent acetabular articular cartilage. A high resolution, non-arthrographic technique was used to assess the labrum and labral chondral transitional zone. Surgical correlation was obtained in all cases by a single surgeon experienced in hip arthroscopy and ten patients with normal hip MRI were included to provide a control group. The acetabular labra in the dysplastic hips demonstrated abnormal signal intensity, and had an elongated appearance when compared with the control group (mean length 10.9 mm vs 6.4 mm). Morphological appearances in the labra included surface irregularity, fissures and cleft formation. MR imaging correctly identified the severity of chondral abnormality in 24 of 27 hips (89%) when compared with arthroscopic findings. MR imaging demonstrates an elongated labrum, focal intra-substance signal change and irregularity and fissuring of the margins in patients with acetabular dysplasia. Abnormality is also identified at the labral chondral transitional zone, where fissuring, focal clefts, chondral deficiency and subchondral cyst formation may be apparent. A high-resolution, non-arthrographic technique can provide an accurate preoperative assessment and evaluate the presence of premature osteoarthritis. (orig.)

  6. MR imaging findings of acetabular dysplasia in adults

    International Nuclear Information System (INIS)

    James, Steven; Connell, David; Miocevic, Miranda; Malara, Frank; Pike, Jonathan; Young, David

    2006-01-01

    To evaluate the diagnostic accuracy of MR imaging in the identification of labral and articular cartilage lesions in patients with acetabular dysplasia. Pre-operative MR imaging was performed on 27 hips in 25 consecutive patients (16 males, 9 females, age range 19-52 years, mean age 31.2 years) with radiographic evidence of acetabular dysplasia (centre-edge angle of Wiberg <20 degrees). The average duration of symptoms was 16.2 months. Two musculoskeletal radiologists assessed MR images in consensus for the presence of abnormality involving the acetabular labrum and adjacent acetabular articular cartilage. A high resolution, non-arthrographic technique was used to assess the labrum and labral chondral transitional zone. Surgical correlation was obtained in all cases by a single surgeon experienced in hip arthroscopy and ten patients with normal hip MRI were included to provide a control group. The acetabular labra in the dysplastic hips demonstrated abnormal signal intensity, and had an elongated appearance when compared with the control group (mean length 10.9 mm vs 6.4 mm). Morphological appearances in the labra included surface irregularity, fissures and cleft formation. MR imaging correctly identified the severity of chondral abnormality in 24 of 27 hips (89%) when compared with arthroscopic findings. MR imaging demonstrates an elongated labrum, focal intra-substance signal change and irregularity and fissuring of the margins in patients with acetabular dysplasia. Abnormality is also identified at the labral chondral transitional zone, where fissuring, focal clefts, chondral deficiency and subchondral cyst formation may be apparent. A high-resolution, non-arthrographic technique can provide an accurate preoperative assessment and evaluate the presence of premature osteoarthritis. (orig.)

  7. Magnetic resonance imaging of labral cysts of the hip

    International Nuclear Information System (INIS)

    Schnarkowski, P.; Steinbach, L.S.; Tirman, P.F.J.; Peterfy, C.G.; Genant, H.K.

    1996-01-01

    Objective. To present the magnetic resonance (MR) imaging findings in patients with labral cysts adjacent to the acetabulum and to examine their association with hip pathology. Design. MR images and conventional radiographs of seven patients with paralabral cysts were retrospectively reviewed by three musculoskeletal radiologists. Patients. The patients included three men and four women with hip pain, ranging in age from 29 to 82 years. Two patients had developmental dysplasia of the hip and six had a history of remote trauma/dislocation. Clinical history and follow-up were obtained in all patients. Surgery was performed on one patient. Results and conclusions. Paralabral cysts were located in the posterosuperior aspect of the hip joint in five patients and in the anterior aspect in two patients. A tear of the adjacent acetabular labrum was confirmed surgically in one patient, and in all patients the MR features suggested the presence of an abnormal labrum. Osteoarthritis was observed in three patients and there was associated subchondral cyst formation in the acetabulum adjacent to the cyst in three patients. The paralabral cyst of the hip is well visualized on MR imaging and is seen in patients with a predisposition to labral pathology. (orig.). With 3 figs., 1 tab

  8. Magnetic resonance imaging of labral cysts of the hip

    Energy Technology Data Exchange (ETDEWEB)

    Schnarkowski, P. [Department of Radiology, University of California, San Francisco, CA (United States)]|[Department of Radiology, Ludwig-Maximilians-University, Munich (Germany); Steinbach, L.S. [Department of Radiology, University of California, San Francisco, CA (United States); Tirman, P.F.J. [Department of Radiology, University of California, San Francisco, CA (United States)]|[San Francisco Magnetic Resonance Center, San Francisco, CA (United States); Peterfy, C.G. [Department of Radiology, University of California, San Francisco, CA (United States); Genant, H.K. [Department of Radiology, University of California, San Francisco, CA (United States)

    1996-11-01

    Objective. To present the magnetic resonance (MR) imaging findings in patients with labral cysts adjacent to the acetabulum and to examine their association with hip pathology. Design. MR images and conventional radiographs of seven patients with paralabral cysts were retrospectively reviewed by three musculoskeletal radiologists. Patients. The patients included three men and four women with hip pain, ranging in age from 29 to 82 years. Two patients had developmental dysplasia of the hip and six had a history of remote trauma/dislocation. Clinical history and follow-up were obtained in all patients. Surgery was performed on one patient. Results and conclusions. Paralabral cysts were located in the posterosuperior aspect of the hip joint in five patients and in the anterior aspect in two patients. A tear of the adjacent acetabular labrum was confirmed surgically in one patient, and in all patients the MR features suggested the presence of an abnormal labrum. Osteoarthritis was observed in three patients and there was associated subchondral cyst formation in the acetabulum adjacent to the cyst in three patients. The paralabral cyst of the hip is well visualized on MR imaging and is seen in patients with a predisposition to labral pathology. (orig.). With 3 figs., 1 tab.

  9. Profundization of acetabular cup uncemented in total substitution of hip in-patient with acetabular dysplasia - Experience university hospital - Clinica San Rafael

    International Nuclear Information System (INIS)

    Dimian Mayorga, Omar David; Sandoval Daza, Alejandro; Vargas Turriago, Marcela; Perez Torres, Javier

    2005-01-01

    14 patients with acetabular dysplasia were treated at Hospital Universitario Clinica San Rafael with total hip arthroplasty with uncemented cup internization. According to Crowe classification, one was type 1, eight were type 2 and five were type 3. The average follow up was twenty-four months. The Harris hip score was used for the clinical evaluation with a pre operative average of 35 points and 37 points post operative. The average cup internization was four millimeters, with an average cup protrutio of 47% and an average of cup coverage of 81%. The average internization of the femoral head's center was 26mm. screws for cup fixation were used in 3 patients. We did not have complications nor implant revision at the time of follow up

  10. A Huge Capital Drop with Compression of Femoral Vessels Associated with Hip Osteoarthritis

    Directory of Open Access Journals (Sweden)

    Tomoya Takasago

    2015-01-01

    Full Text Available A capital drop is a type of osteophyte at the inferomedial portion of the femoral head commonly observed in hip osteoarthritis (OA, secondary to developmental dysplasia. Capital drop itself is typically asymptomatic; however, symptoms can appear secondary to impinge against the acetabulum or to irritation of the surrounding tissues, such as nerves, vessels, and tendons. We present here a case of unilateral leg edema in a patient with hip OA, caused by a huge bone mass occurring at the inferomedial portion of the femoral head that compressed the femoral vessels. We diagnosed this bone mass as a capital drop secondary to hip OA after confirming that the mass occurred at least after the age of 63 years based on a previous X-ray. We performed early resection and total hip arthroplasty since the patient’s hip pain was due to both advanced hip OA and compression of the femoral vessels; moreover, we aimed to prevent venous thrombosis secondary to vascular compression considering the advanced age and the potent risk of thrombosis in the patient. A large capital drop should be considered as a cause of vascular compression in cases of unilateral leg edema in OA patients.

  11. The role of triple pelvic osteotomy in therapy of residual hip dysplasia and sequel of AVN: long-term experience.

    Science.gov (United States)

    Dungl, P; Rejholec, M; Chomiak, J; Grill, F

    2007-01-01

    Triple pelvic osteotomy was performed for sequel of DDH including AVN between 1981 and 2002 for 329 patients (351 hips, 280 females, 49 males, average age at surgery 16.5 years, range 9-41 years, follow-up 4-25 years). A small modification of Steel's technique consisting of strictly subperiostal resection of segment from both pubic and ischial bone was used. Average gain of lengthening extremity was 1.8 cm. The average CE angle was improved from 7.8 to 35.5 degrees. Clinical results were evaluated according to Merle d'Aubigne and reflected to the preoperative clinical and radiological findings. There were 146 hip joints in 128 patients (76%) with excellent results in the group of congruent hips without arthrosis. In 182 hips in 178 patients with hip joints with some deformity, limited ROM and decentration, 40% were excellent, 32% good, 23% fair and 5% unsatisfactory results were achieved. The group of decentrated hip joints in young adults in incongruency, limited ROM and sometimes severe arthrosis consisted of 23 monolateral surgeries with 39% unsatisfactory, 39% fair and 22% good results, respectively. No major neurovascular complications were seen. Non-unions were recorded in 19 patients (5.4%), including 2 triple and 2 double non-unions. Based on our long-term experience, we can conclude that triple pelvic osteotomy according to Steel in our modification is a safe method and gives regularly excellent or good results for correction of clinical and radiographic appearance of acetabular dysplasia when there is a proper indication.

  12. Fate of bulk auto grafts in uncemented total hip arthroplasty: Evaluation by bone scintigraphy

    International Nuclear Information System (INIS)

    Simsek, A.; Cila, E.; Sener, E.; Senkoylu, A.; Sipahioglu, S.; Atasever, T.; Akdemir, Ozgur

    2006-01-01

    Objective was to review short term results of uncemented cup implantation and the fate of bulk femoral head autografts in patients with acetabular bone deficiency due to dysplasia of the hip. We used bone scintigraphy to assess the viability of the grafts. We treated 19 hip joints of 17 patients with osteoarthritis due to developmental dysplasia of the hip with uncemented total hip arthroplasty between 1997-2003 in the Department of Orthopedics and Traumatology, Gazi University, Ankara, Turkey. The average age was 49.1 (31-72 years), and the average follow up period was 36 months. We used femoral head autografts to reconstruct superolateral segmental deficiencies of the acetabuli. We evaluated the patients clinically and radiologically to assess acetabular loosening, and we used three-phase bone scintigraphy to evaluate the viability of the autograft.There was no acetabular component revision throughout the follow up period. There was no radiological evidence of graft resorption or graft displacement in any of the patients. In bone scintigraphy, we observed hyperemia in the blood pool phase, and the osteoblastic activity of the bone graft was at the level of the neighboring iliac bone. The application of a non-cemented acetabular component with femoral autograft in superolateral acetabulum deficiency increases the stability of the implant and increases the bone stock. It is not easy to evaluate the viability of the graft by the use of radiological methods. Bone scintigraphy gives sufficient information about the viability of the graft as a non-invasive method. (author)

  13. Does bony hip morphology affect the outcome of treatment for patients with adductor-related groin pain?

    DEFF Research Database (Denmark)

    Hölmich, Per; Thorborg, Kristian; Nyvold, Per

    2014-01-01

    BACKGROUND: Adductor-related groin pain and bony morphology such as femoroacetabular impingement (FAI) or hip dysplasia can coexist clinically. A previous randomised controlled trial in which athletes with adductor-related groin pain underwent either passive treatment (PT) or active treatment (AT......) showed good results in the AT group. The primary purpose of the present study was to evaluate if radiological signs of FAI or hip dysplasia seem to affect the clinical outcome, initially and at 8-12 years of follow-up. METHODS: 47 patients (80%) were available for follow-up. The clinical result......, there was no significant difference in the distribution of Tönnis grades between hips that had an unchanged or improved outcome compared with hips that had a worse outcome over time (p=0.145). CONCLUSIONS: No evidence was found that bony hip morphology related to FAI or dysplasia prevents successful outcome...

  14. Cryptorchidism as a caudal developmental field defect. A new description of cryptorchidism associated with malformations and dysplasias of the kidneys, the ureters and the spine from T10 to S5

    DEFF Research Database (Denmark)

    Cortes, D; Thorup, J M; Beck, B L

    1998-01-01

    individuals with tritonmelia, the male variant of sirenomelia. Sirenomelia/tritonmelia is an extreme degree of abnormal differentiation of the caudal developmental field, also called caudal dysplasia, the caudal regression syndrome and the caudal regression malformation sequence. Caudal developmental field...

  15. Anesthesia for a patient with Fanconi anemia for developmental dislocation of the hip: a case report

    Directory of Open Access Journals (Sweden)

    Zafer Dogan

    2014-05-01

    Full Text Available Fanconi anemia is a rare autosomal recessive inherited bone marrow failure syndrome with congenital and hematological abnormalities. Literature regarding the anesthetic management in these patients is limited. A management of a developmental dislocation of the hip was described in a patient with fanconi anemia. Because of the heterogeneous nature, a patient with fanconi anemia should be established thorough preoperative evaluation in order to diagnose on clinical features. In conclusion, we preferred caudal anesthesia in this patient with fanconi anemia without thrombocytopenia, because of avoiding from N2O, reducing amount of anesthetic, existing microcephaly, hypothyroidism and elevated liver enzymes, providing postoperative analgesia, and reducing amount of analgesic used postoperatively. Keywords: Fanconi anemia, Caudal anesthesia, Developmental dislocation of the hip

  16. The correlation between Salter's criteria for avascular necrosis of the femoral head and Kalamchi's prognostic classification following the treatment of developmental dysplasia of the hip.

    Science.gov (United States)

    Tsukagoshi, Y; Kamegaya, M; Kamada, H; Saisu, T; Morita, M; Kakizaki, J; Tomaru, Y; Yamazaki, M

    2017-08-01

    The aim of this study was to evaluate the correlation between Salter's criteria and Kalamchi's classification of avascular necrosis in patients treated for developmental dysphasia of the hip (DDH). The study involved a retrospective analysis of 123 patients (123 hips) with DDH treated by operative and non-operative reduction before the age of two years, with a minimum follow-up of ten years. Salter's criteria (S1 to S4) were determined from radiographs obtained at one to two years post-reduction, whilst the Kalamchi grade was determined from radiographs obtained at ten or more years of age. Early post-reduction radiographs were also used to evaluate the centre-head distance discrepancy (CHDD) and the occurrence of a dome-shaped deformity of the proximal femoral metaphysis (D-shaped metaphysis). The prognosis was described as good (Kalamchi grade K0 or KI), fair (Kalamchi grade KII) or poor (Kalamchi grade KIII or KIV) for analysis and correlation with the early Salter criteria, CHDD and D-shaped metaphysis. S1 and S2 criteria were predictive of a poor prognosis. The outcome following S3, S4 and S3 + S4 varied; 18 (40%) had a good prognosis, 17 (38%) a fair prognosis and ten (22%) a poor prognosis. A CHDD ≥ 10% and a D-shaped metaphysis were also predictive of a poor prognosis. The Salter criteria were predictive of the Kalamchi grade of avascular necrosis in patients with DDH aged ten or more years after reduction of the hip. Cite this article: Bone Joint J 2017;99-B:1115-20. ©2017 The British Editorial Society of Bone & Joint Surgery.

  17. Generalized dysplasia epiphysealis hemimelica with contralateral sacro-iliac joint involvement

    International Nuclear Information System (INIS)

    Karam, Adib R.; Birjawi, Ghina A.; Khoury, Nabil J.; Saghieh, Said; Tawil, Ayman

    2008-01-01

    Dysplasia epiphysealis hemimelica (or Trevor's disease) is a rare developmental bone dysplasia characterized by benign osteocartilaginous overgrowth involving one or multiple epiphyses, usually of a single lower extremity. It is classified as localized form, classical form (most common), and generalized form. In this report we describe a case of generalized form of dysplasia epiphysealis hemimelica with involvement of the contralateral sacroiliac joint, which is an extremely rare presentation. (orig.)

  18. Generalized dysplasia epiphysealis hemimelica with contralateral sacro-iliac joint involvement

    Energy Technology Data Exchange (ETDEWEB)

    Karam, Adib R.; Birjawi, Ghina A.; Khoury, Nabil J. [American University of Beirut Medical Center, Department of Diagnostic Radiology, Beirut (Lebanon); Saghieh, Said [American University of Beirut Medical Center, Department of Orthopedic Surgery, Beirut (Lebanon); Tawil, Ayman [American University of Beirut Medical Center, Department of Pathology, Beirut (Lebanon)

    2008-12-15

    Dysplasia epiphysealis hemimelica (or Trevor's disease) is a rare developmental bone dysplasia characterized by benign osteocartilaginous overgrowth involving one or multiple epiphyses, usually of a single lower extremity. It is classified as localized form, classical form (most common), and generalized form. In this report we describe a case of generalized form of dysplasia epiphysealis hemimelica with involvement of the contralateral sacroiliac joint, which is an extremely rare presentation. (orig.)

  19. Epiphyseal dysplasia of the hip. Diagnosis and differential diagnosis with MRI. Epiphysaere Dysplasie des Hueftgelenks, Diagnostik und Differentialdiagnostik mit der MRT

    Energy Technology Data Exchange (ETDEWEB)

    Grimm, J. (Univ. Mainz, Orthopaedische Klinik und Poliklinik (Germany)); Just, M. (Univ. Mainz, Klinik mit Poliklinik fuer Radiologie (Germany))

    1992-07-01

    Sixteen hip joints of eight patients with multiple epiphyseal dysplasia were examined by clinical investigation, plain films and by magnetic resonance imaging (MRI), using T[sub 1]- and T[sub 2]-weighted images and gradient echoes. MRI is useful in demonstrating the congruity of the joint, in the changes of the epiphyseal signal intensities and the epiphyseal line. Individual changes of the signal intensity patterns on T[sub 1]- and T[sub 2]-weighted scans are described. Although MRI exhibits a variety of patterns in different patients, a clearcut differentiation from Legg-Calve-Perthes' disease is possible. (orig.).

  20. Treatment Algorithm for Patients with Non-arthritic Hip Pain, Suspect for an Intraarticular Pathology

    DEFF Research Database (Denmark)

    Jørgensen, Rasmus Wejnold; Dippmann, Christian; Dahl, L

    2016-01-01

    BACKGROUND: The amount of patients referred with longstanding, non-arthritic hip pain is increasing, as are the treatment options. Left untreated hip dysplasia, acetabular retroversion and femoroacetabular impingement (FAI) may lead to osteoarthritis (OA). Finding the right treatment option...... associated with acetabular retroversion described in the literature were the crossover sign, the posterior wall sign and the ischial spine sign, while Wiberg's lateral center-edge angle (CE-angle) together with Leqeusne's acetabular index indicate hip dysplasia. A Tönnis index >2 indicates osteoarthritis...... for the right patient can be challenging in patients with non-arthritic hip pain. PURPOSE: The purpose of this study was to categorize the radiographic findings seen in patients with longstanding hip pain, suspect for an intraarticular pathology, and provide a treatment algorithm allocating a specific treatment...

  1. Primary abductor hip contracture as diagnostic, prognostic and therapeutic problem in child hip pathology

    Directory of Open Access Journals (Sweden)

    Pajić Miloš

    2007-01-01

    Full Text Available Coxa obliqua represents a special functional entity in the pathology of the child hip. Authors have confirmed the results of S.L. Weissman and B. Strinovic which claimed that the abductor contracture of the hip was a primary congenital condition that developed as a result of intrauterine malposition, leading later to the contralateral adductor contracture. Critical period for the development of complications was between 6 and 8 month after birth, adductor contracture might keep persisting together with the development of acetabular dysplasia, and later on with ipsilateral subluxation. This malformation has usually been diagnosed within 3 and 6 months of age. It could be connected with some other signs of malposition, such as plagiocephaly, torticollis or infantile thoracic C scoliosis. For the diagnosis of coxa obliqua, the examination of hips in the prone position was very important and the ultrasonic and radiological examinations were crucial. The applied treatment used to be exclusively physical rehabilitation. Wide diapering has been contraindicated. In this study, we included 2,500 newborns, 1,300 boys and 1,200 girls (5,000 hips. In 22 cases of coxa obliqua (10‰, the excellent results were obtained in 96% of cases. In two unsuccessfully treated cases, a contralateral dysplasia developed, and in one untreated, subluxation. The authors are advocating a systematic and early detection and treatment of the primary coxa obliqua. .

  2. Perinatal Autopsy Findings in a Case of De Novo Hypohidrotic Ectodermal Dysplasia.

    Science.gov (United States)

    Chikkannaiah, Panduranga; Nagaraju, Smitha; Kangle, Rajit; Gosavi, Mansi

    2015-01-01

    Ectodermal dysplasia are group of inherited disorders involving the developmental defects of ectodermal structures like hair, teeth, nails, sweat glands, and others. X-linked recessive inheritance is most common. Here we describe perinatal autopsy findings in a case of de novo ectodermal dysplasia in a female fetus. To the best of our knowledge, this is the first fetal autopsy description in a case of ectodermal dysplasia.

  3. Early diagnosis of congenital dislocation of the hip

    International Nuclear Information System (INIS)

    Grill, F.

    1984-01-01

    Neonatal hip examination is only effective with the help of a large number of experts. Teamwork between pediatrician, pediatric orthopedic surgeon and general practitioner seems to be essential. Best results can be achieved with a hip examination during the first 4 days of life and controls with 3 months and with 12 months. The technic of clinical examination and diagnosis is described. Because of hip dysplasias without any clinical signs the importance of an x-ray of the hip with an age of 3 months is emphasized. (Author)

  4. Femoral head shape differences during development may identify hips at risk of degeneration.

    Science.gov (United States)

    Vanden Berg-Foels, Wendy S; Schwager, Steven J; Todhunter, Rory J; Reeves, Anthony P

    2011-12-01

    Developmental dysplasia of the hip (DDH) is a common cause of elevated contact stress and early onset osteoarthritis (OA). We hypothesized that adaptation to focal loading during postnatal development would result in signature changes to the shape of the femoral head secondary center of ossification (SCO). SCO shape was evaluated in a canine model of DDH at ages 14 and 32 weeks. The evolving 3D morphology of the SCO was captured using serial quantitative computed tomography. A discrete medial representation shape model was fit to each SCO and served as the basis for quantitative thickness and bending measurements. Shape measurements were tested for associations with hip subluxation and degeneration. At 32 weeks, the SCO was thinner (flatter) in the perifoveal region, the site of focal loading; a greater bend to the SCO was present lateral to the site of thinning; SCO thinning and bending were associated with less femoral head coverage and with a higher probability of degeneration. Shape changes were not detected at 14 weeks. Measurement and visualization of SCO shape changes due to altered loading may provide a basis for identifying hips at risk of early onset OA and a tool for surgical planning of hip restructuring.

  5. Ultrasound screening and follow-up of congenital hip dislocation

    International Nuclear Information System (INIS)

    Ballerini, G.; Avanzini, A.; Colombo, T.; Micucci, E.

    1990-01-01

    US screening for hip dysplasia was performed on 1421 full-term newborns. The study was aimed at: 1) evaluating US feasibility in an unselected maternity ward population; 2) determining the frequency of the different types of hips, and correlating ours with literature data; 3) evaluating the efficacy of both an early prevention and treatment. All US examination were performed within the first week of life and the 2842 hips classified according to Graf. At birth, normal hips (Ia, Ib) were 2064 (72.6%) (group I); Physiologically immature hips (IIa) were 721 (25.4%) (group II); pathological hips (group III) were: 43 (1.5%) IIc, 8 (o.3%) IId, 6 (0.2%) III. Hips in group B were cheked at 3 months: 502 were normal, 51 were type IIb, and 1 was type IId: the latter 52 hips were treated and normalized in the following months. Hips in group C (types IIc, IId, and III=57 hips) were treated and cheked every seventh week until normalization. Our experience confirm US value in the evaluation of hip dysplasia in the newborn. In our opinion, the use of this method shoul be encouraged, although US screening of all newborns remains controversial on a cost-benefit ratio. Multicentric studies will better define US sensitivity, specificity and reliability. The correlation of ours results with literature data was difficult, because study populations are not always homogeneous. As for therapy, we found the plastic splint very effective and easy to use: In 2 cases, Milgram devices were used for a few months

  6. Treatment of the Developmental Dysplasia of the Hip with an Abduction Brace in Children up to 6 Months Old

    Directory of Open Access Journals (Sweden)

    Raphaël Wahlen

    2015-01-01

    Full Text Available Introduction. Use of Pavlik harness for the treatment of DDH can be complicated for parents. Any misuse or failure in the adjustments may lead to significant complications. An abduction brace was introduced in our institution, as it was thought to be easier to use. Aim. We assess the results for the treatment of DDH using our abduction brace in children of 0–6 months old and compare these results with data on treatments using the Pavlik harness. Method. Retrospective analysis of patients with DDH from 0 to 6 months old at diagnosis, performed from 2004 to 2009. Outcomes were rates of reduction of the hip and avascular necrosis of the femoral head (AVN. Follow-up was at one year and up to 4 years old. Results. Hip reduction was successful in 28 of 33 patients (85%, with no AVN. Conclusion. Our results in terms of hip reduction rate and AVN rate are similar to those found in literature assessing Pavlik harness use, with a simpler and comfortable treatment procedure.

  7. Intra-Articular Hyaluronic Acid Compared to Traditional Conservative Treatment in Dogs with Osteoarthritis Associated with Hip Dysplasia

    Directory of Open Access Journals (Sweden)

    Gabriel O. L. Carapeba

    2016-01-01

    Full Text Available The purpose of this study was to compare the efficacy of the intra-articular (IA hyaluronic acid injection to traditional conservative treatment (TCT in dogs with osteoarthritis (OA induced by hip dysplasia. Sixteen dogs were distributed into two groups: Hyal: IA injection of hyaluronic acid (5–10 mg, and Control: IA injection with saline solution (0.5–1.0 mL in combination with a TCT using an oral nutraceutical (750–1000 mg every 12 h for 90 days and carprofen (2.2 mg/kg every 12 h for 15 days. All dogs were assessed by a veterinarian on five occasions and the owner completed an assessment form (HCPI and CPBI at the same time. The data were analyzed using unpaired t test, ANOVA, and Tukey’s test (P<0.05. Compared with baseline, lower scores were observed in both groups over the 90 days in the veterinarian evaluation, HCPI, and CPBI (P<0.001. The Hyal group exhibited lower scores from 15 to 90 and 60 to 90 days, in the CBPI and in the veterinarian evaluation, respectively, compared to the Control group. Both treatments reduced the clinical signs associated with hip OA. However, more significant results were achieved with intra-articular hyaluronic acid injection.

  8. Outcomes of Hip Arthroscopy in Competitive Athletes.

    Science.gov (United States)

    Perets, Itay; Hartigan, David E; Chaharbakhshi, Edwin O; Ashberg, Lyall; Ortiz-Declet, Victor; Domb, Benjamin G

    2017-08-01

    To evaluate the minimum 2-year postoperative clinical outcomes and the rate of return to sports in athletes who underwent capsular plication for the treatment of ligamentous laxity and/or borderline dysplasia during hip arthroscopy for the treatment of femoroacetabular impingement and labral pathology. Since 2008, data were prospectively collected on patients who underwent hip arthroscopy for the treatment of femoroacetabular impingement and/or labral tears. Inclusion criteria were as follows: athlete at the high school, collegiate, or professional levels preoperatively, underwent capsular plication, and preoperatively recorded patient-reported outcome scores including modified Harris hip score (mHHS), nonarthritic athletic hip score (NAHS), hip outcome score-sports-specific subscale (HOS-SSS), and visual analog scale (VAS). Exclusion criteria were as follows: 1, and previous hip conditions. Sports activity and competitive levels were collected at a minimum of 2 years postoperatively. Fifty-one hips (49 patients) met the inclusion criteria, and 41 hips (39 patients) had minimum 2-year follow-up (80.4% follow-up). Mean mHHS increased from 67.1 preoperatively to 83.5 (P arthroscopies allowed the patients to return to sports at follow-up. Thirty-four (82.9%) hip arthroscopies allowed the patients to maintain their competitive physical abilities at follow-up. Patient-reported outcomes and VAS in athletes significantly improved at a minimum of 2 years after capsular plication as a part of hip arthroscopy addressing varying pathologies. In addition, most patients returned to sports at similar or higher competitive levels. These results suggest that capsular plication is a favorable treatment option in athletes with ligamentous laxity and/or borderline dysplasia. Level IV, therapeutic case series. Copyright © 2017 Arthroscopy Association of North America. Published by Elsevier Inc. All rights reserved.

  9. Ultralydskanning ved diagnostik og behandling af hoftedysplasi. Vurdering af en selektiv screeningsprocedure

    DEFF Research Database (Denmark)

    Strandberg, C.; Konradsen, L.A.; Ellitsgaard, N.

    2008-01-01

    INTRODUCTION: With the intention of reducing the treatment frequency of Developmental Dysplasia of the Hip (DDH), two hospitals in Copenhagen implemented a screening and treatment procedure based on selective referral to ultrasonography of the hip (US). This paper describes and evaluates...... 0.03%. No relationship was seen between morphological parameters at the first US and the outcome of hips classified as minor dysplastic or not fully developed (NFD). A statistically significant relationship was seen between the degree of dysplasia and the time until US normalization of the hips (p......= 0.02). There was no relapse of dysplasia after treatment. The median duration of treatment was six, eight and nine weeks for mild, moderate and severe dysplasia respectively. CONCLUSION: The procedure resulted in a low rate of treatment and a small number of late diagnosed cases. Prediction...

  10. Rapid Prototyping 3D Model in Treatment of Pediatric Hip Dysplasia: A Case Report.

    Science.gov (United States)

    Holt, Andrew M; Starosolski, Zbigniew; Kan, J Herman; Rosenfeld, Scott B

    2017-01-01

    Rapid prototyping is an emerging technology that integrates common medical imaging with specialized production mechanisms to create detailed anatomic replicas. 3D-printed models of musculoskeletal anatomy have already proven useful in orthopedics and their applications continue to expand. We present the case of a 10 year-old female with Down syndrome and left acetabular dysplasia and chronic hip instability who underwent periacetabular osteotomy. A rapid prototyping 3D model was created to better understand the anatomy, counsel the family about the problem and the surgical procedure, as well as guide surgical technique. The intricate detail and size match of the model with the patient's anatomy offered unparalleled, hands-on experience with the patient's anatomy pre-operatively and improved surgical precision. Our experience with rapid prototyping confirmed its ability to enhance orthopedic care by improving the surgeon's ability to understand complex anatomy. Additionally, we report a new application utilizing intraoperative fluoroscopic comparison of the model and patient to ensure surgical precision and minimize the risk of complications. This technique could be used in other challenging cases. The increasing availability of rapid prototyping welcomes further use in all areas of orthopedics.

  11. The early diagnosis of developmental dysplasia of the hip using ultrasonography. The importance of following up cases with physiological immaturity; Diagnostico precoz por ultrasonidos de la displasia de desarrollo de la cadera. Importancia del seguimiento de los casos con inmadurez fisiologia

    Energy Technology Data Exchange (ETDEWEB)

    Esparza, J.; Gonzalez, A.; Garcia, S.; Elso, J.; Cordero, J. L. [Hospital Vigen del Camino. Pamplona (Spain)

    1999-07-01

    To determine the efficiency of an early diagnosis programme using ultrasounds (US) of development dysplasia of the hip (DDH), using different criteria to delect patients according to the sex: universal to girls, but only including boys with a DDH risk factor (a physical pathological examination of new born babies, babies born feet first, family history and orthopaedic deformities). Of the 3,469 children born at our hospital during one specific year, 1,721 were included in the programme. US was carried out on all of them, following the Graf technique, when they were a month old, with the exception of the new born babies who were studied during their first week by means of a physical pathological examination. The babies who were diagnosed with physically immature hips in this first study using US; were followed up. Thirty-two babies born during the year suffered from DDH (a rate of 9.2 per thousand). Among them there was only 1 case of delayed DDH (that was not detected in the programme). Seven babies were diagnosed with DDH thanks to the follow up procedures carried out on babies with physically immature hips. Putting this programme for early diagnosis into practices has achieved the almost total disappearance of delayed DDH. When comparing the results with previous periods, where different early detection systems were used, the duration and the aggressiveness of the treatment has reduced considerably. Follow up procedures using US of physically immature hips is obligatory. (Author) 14 refs.

  12. Magnetic resonance imaging of hip joint cartilage and labrum

    Directory of Open Access Journals (Sweden)

    Christoph Zilkens

    2011-09-01

    Full Text Available Hip joint instability and impingement are the most common biomechanical risk factors that put the hip joint at risk to develop premature osteoarthritis. Several surgical procedures like periacetabular osteotomy for hip dysplasia or hip arthroscopy or safe surgical hip dislocation for femoroacetabular impingement aim at restoring the hip anatomy. However, the success of joint preserving surgical procedures is limited by the amount of pre-existing cartilage damage. Biochemically sensitive MRI techniques like delayed Gadolinium Enhanced MRI of Cartilage (dGEMRIC might help to monitor the effect of surgical or non-surgical procedures in the effort to halt or even reverse joint damage.

  13. p63 in skin development and ectodermal dysplasias

    Science.gov (United States)

    Koster, Maranke I.

    2010-01-01

    The transcription factor p63 is critically important for skin development and maintenance. Processes that require p63 include epidermal lineage commitment, epidermal differentiation, cell adhesion, and basement membrane formation. Not surprisingly, alterations in the p63 pathway underlie a subset of ectodermal dysplasias, developmental syndromes in which the skin and skin appendages do not develop normally. This review summarizes the current understanding of the role of p63 in normal development and ectodermal dysplasias. PMID:20445549

  14. Bilateral femoral head dysplasia and osteochondritis. Multiple epiphyseal dysplasia tarda, spondylo-epiphyseal dysplasia tarda, and bilateral Legg-Perthes disease

    Energy Technology Data Exchange (ETDEWEB)

    Andersen, P.E. Jr.; Schantz, K.; Bollerslev, J.; Justesen, P.

    Multiple epiphyseal dysplasia tarda (MEDT) and spondylo-epiphyseal dysplasisa tarda (SEDT) are genetically transmitted conditions affecting the hips, which may resemble bilateral Legg-Perthes disease (LPD). Misdiagnoses are not uncommon, with serious implications for treatment, prognosis and genetic counseling. An epidemiologic study of MEDT and SEDT in a well-defined population of 453 921 persons in Denmark was performed. A population prevalence of 0.7 per 100 000 inhabitants with SEDT and 4.0 per 100 000 inhabitants with MEDT was found. Distinguishing features between MEDT, SEDT and bilateral LPD based on radiologic findings in the hips, other joints, and spine were ascertained. Bilateral LPD is always asymmetric, exhibits patches of increased density in the epiphyses and often metaphyseal cyst-like changes. No spinal lesion or affection of other joints is present, and the acetabula are normal. In MEDT and SEDT the capital femoral epiphyses are symmetrically flattened, fragmented and uniformly slightly sclerotic. Generalised platyspondyly is a constant finding in SEDT.

  15. The importance of the first ultrasonic exam of newborn hips.

    Science.gov (United States)

    Grubor, Predrag; Asotic, Mithat; Biscevic, Mirza; Grubor, Milan

    2012-01-01

    Developmental hip disorder (DHD) is a disorder in development of the acetabulum which remains abrupt (dysplasia) and probably consequential cranialisation of the femur head (luxation). The aim of this paper is to establish the total number of DHD and its subtypes at the first clinical and ultrasound exam of newborns in a retrospective-prospective study made in the period from 1st Jan 2006 through to 31 Dec 2010 at the Clinic for orthopaedics and traumatology in Banja Luka. In total 6132 patients were examined and 99 cases diagnosed with DHD (dysplasia and luxation). Ultrasonic exam was done by means of electronic probe of 5-12 MHz according to standard method after Graph. Girls were significantly more present (96%). Positive family anamnesis on DHD was present with 7.8% examinee, mainly with primiparas, and/ or with 77.8% children with DHD. Dominant intrauterine risk factors for DHD were: mal position of foetus in uterus (78.6%), oligoamnion (17.9%), malformation of the spinal column of the pregnant woman (3.6%), whereas with 38.4% of children with a certain form of DHD the following were found: breech presentation, caesarean section or twin pregnancy. The clinical exam indicated DHD with 8.87% examinee, out of which hip looseness was found with 5% examinees. Ultrasonic finding was positive with 99 examinee, that is with 1.61% of them (deficient and badly formed acetabulum, sleeked protrusion; 8 luxations and 91 dysplasia). Prophylactic measures were requested by 58.6% children (abductive bending and exercises), whereas 41.4 % needed non-intervention therapeutic measures (traction, Pavlik's straps, Graph's knickers, plastering), after which there were no children needing surgical correction of DHD. These data indicate that clinical exam is unreliable for DHD diagnostics, and that Ultrasonic diagnostics and treatment of DHD should start as early as possible applying atraumatic helping devices and procedures in the period when all structures are elastic, flexible and

  16. Poly-epiphyseal overgrowth: description of a previously unreported skeletal dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Pazzaglia, Ugo E.; Bonaspetti, Giovanni [University of Brescia, Orthopaedic Clinic, Brescia (Italy); Beluffi, Giampiero [Fondazione IRCCS Policlinico San Matteo, Department of Paediatric Radiology, Pavia (Italy); Marchi, Antonietta; Bozzola, Mauro; Savasta, Salvatore [Fondazione IRCCS Policlinico San Matteo, Paediatric Clinic, University of Pavia, Pavia (Italy)

    2007-10-15

    A skeletal dysplasia with previously unreported features is presented. Its evolution was characterized by growth abnormalities of bones without involvement of other organs. Advanced bone age, increased stature and irregular epiphyseal ossification with stippling of the main long bones were documented. Physeal overgrowth was massive in the left proximal humerus and femur. Furthermore, the hip joint appeared fused with an abundant mass of pathological calcific tissue extending from the femur to the ilium. Pathological epiphyses were characterized by anarchic cartilaginous proliferation with multiple ossification centres, while lamellar bone apposition and remodelling were normal. The observed bone changes were different from those in any previously reported syndrome, metabolic defect or bone dysplasia. However, they clearly indicated a defect of endochondral ossification with some resemblance to phenotypes observed in dysplasia epiphysealis hemimelica. (orig.)

  17. Poly-epiphyseal overgrowth: description of a previously unreported skeletal dysplasia

    International Nuclear Information System (INIS)

    Pazzaglia, Ugo E.; Bonaspetti, Giovanni; Beluffi, Giampiero; Marchi, Antonietta; Bozzola, Mauro; Savasta, Salvatore

    2007-01-01

    A skeletal dysplasia with previously unreported features is presented. Its evolution was characterized by growth abnormalities of bones without involvement of other organs. Advanced bone age, increased stature and irregular epiphyseal ossification with stippling of the main long bones were documented. Physeal overgrowth was massive in the left proximal humerus and femur. Furthermore, the hip joint appeared fused with an abundant mass of pathological calcific tissue extending from the femur to the ilium. Pathological epiphyses were characterized by anarchic cartilaginous proliferation with multiple ossification centres, while lamellar bone apposition and remodelling were normal. The observed bone changes were different from those in any previously reported syndrome, metabolic defect or bone dysplasia. However, they clearly indicated a defect of endochondral ossification with some resemblance to phenotypes observed in dysplasia epiphysealis hemimelica. (orig.)

  18. High frequency of labral pathology in dysplastic hips with a CE angle between 20-25

    DEFF Research Database (Denmark)

    Jakobsen, Stig Storgaard; Hartig-Andreasen, Charlotte; Mikkelsen, Lone Rømer

    Background: Hip dysplasia becomes symptomatic due to labral pathology and secondary muscular pain. A CE angle dysplasia in PAO centres in Denmark. However, it is debated whether a CE angle between 20 and 25 is borderline. Purpose / Aim of Study: We aimed...... to investigate the degree of labral pathology in symptomatic patients with CE between 20 and 25 compared with patients with CE hips) with a mean age 34.1 years (range 14.5- 58.9 years) consecutively scheduled for PAO due to symptomatic DDH were enrolled...... in the study. Five patients were excluded from the study and four patients failed to show up at follow- up, hence 90 patients were evaluated. Indication for PAO were persisting hip pain, a center-edge angle of Wiberg 15, hip flexion

  19. Hip malformation is a very common finding in young patients scheduled for total hip arthroplasty

    DEFF Research Database (Denmark)

    Karimi, Dennis; Kallemose, Thomas; Troelsen, Anders

    2018-01-01

    and prevalences of hip malformations were; CAM-deformity 50.9 and 25.5%, coxa profunda 33 and 27.4%, acetabular retroversion 33 and 29.2%, and acetabular dysplasia 10.4 and 3.8%. All patients showed minimum of one malformation. Prevalences of Tönnis grade 0-1 were 22.6% and 2-3 were 77.4%. CONCLUSION: All......INTRODUCTION: In Denmark, 20% of all registered total hip arthroplasties (THA) from 1995 to 2014 has been patients younger than 60 years with primary idiopathic osteoarthritis (OA). It is speculated that hip malformations may be a major contributor to early OA development. It has been shown...... that hip malformation may compromise implant position and, therefore, identifying and knowing the incidence of malformations is important. Our aim was to assess the prevalence and type of hip malformations in a cohort of younger patients undergoing THA. MATERIALS AND METHODS: In this prospective two center...

  20. Volume of the ligamentum capitis femoris in osteoartritic hip joints of adult dogs

    International Nuclear Information System (INIS)

    Mande, J.D.; Mbithi, P.M.F.; Mbugua, S.W.; Buoro, I.B.J.; Gathumbi, P.K.

    2003-01-01

    Ventrodorsal pelvic radiographs were made of 32 adult dogs under general anaesthesia. The hip joints were evaluated according to the severity of osteoarthritic changes graded as 0, 1, 2 or 3. The dogs were euthanased, the hip joints opened and the ligamentum capitis femoris dissected out in toto. The volume of each ligament was determined using a water displacement technique and the mean volume compared to the four radiographic grades of osteoarthritis. There was an inverse correlation (r = -0.75) between the mean volume of the ligamentum capitis femoris and the increasing severity of osteoarthritis as assessed by radiography. The results confirmed the crucial role of radiography in the clinical evaluation of hip dysplasia and osteoarthritis in the adult dog. Assessment of the volume of the ligamentum capitis femoris revealed that it is an important tool for research in canine hip dysplasia and osteoarthritis

  1. Volume of the ligamentum capitis femoris in osteoarthritic hip joints of adult dogs

    Directory of Open Access Journals (Sweden)

    J.D. Mande

    2003-06-01

    Full Text Available Ventrodorsal pelvic radiographs were made of 32 adult dogs under general anaesthesia. The hip joints were evaluated according to the severity of osteoarthritic changes graded as 0, 1, 2 or 3. The dogs were euthanased, the hip joints opened and the ligamentum capitis femoris dissected out in toto. The volume of each ligament was determined using a water displacement technique and the mean volume compared to the four radiographic grades of osteoarthritis. There was an inverse correlation (r = -0.75 between the mean volume of the ligamentum capitis femoris and the increasing severity of osteoarthritis as assessed by radiography. The results confirmed the crucial role of radiography in the clinical evaluation of hip dysplasia and osteoarthritis in the adult dog. Assessment of the volume of the ligamentum capitis femoris revealed that it is an important tool for research in canine hip dysplasia and osteoarthritis.

  2. Bilateral Cerebellar Cortical Dysplasia without Other Malformations: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Jung Seok; Ahn Kook Jin; Kim, Jee Young; Lee, Sun Jin; Park, Jeong Mi [Catholic University Yeouido St. Mary' s Hospital, College of Medicine, Seoul (Korea, Republic of)

    2010-06-15

    Recent advances in MRI have revealed congenital brain malformations and subtle developmental abnormalities of the cerebral and cerebellar cortical architecture. Typical cerebellar cortical dysplasia as a newly categorized cerebellar malformation, has been seen in patients with Fukuyama congenital muscular dystrophy. Cerebellar cortical dysplasia occurs at the embryonic stage and is often observed in healthy newborns. It is also incidentally and initially detected in adults without symptoms. To the best of our knowledge, cerebellar dysplasia without any related disorders is very rare. We describe the MRI findings in one patient with disorganized foliation of both cerebellar hemispheres without a related disorder or syndrome

  3. Ultralydskanning ved diagnostik og behandling af hoftedysplasi. Vurdering af en selektiv screeningsprocedure

    DEFF Research Database (Denmark)

    Strandberg, Charlotte; Konradsen, Lars Aage Glud; Ellitsgaard, Niels

    2008-01-01

    With the intention of reducing the treatment frequency of Developmental Dysplasia of the Hip (DDH), two hospitals in Copenhagen implemented a screening and treatment procedure based on selective referral to ultrasonography of the hip (US). This paper describes and evaluates the procedure....

  4. Dental Management of Ectodermal Dysplasia: Two Clinical Case Reports

    Directory of Open Access Journals (Sweden)

    Somayeh Hekmatfar

    2012-06-01

    Full Text Available Ectodermal dysplasia is a hereditary disorder associated with abnormal development of embryonic ectodermally-derived organs including teeth, nails, hair and sweat glands. Hypodontia of the primary and permanent dentition is the most common oral finding. Therefore, affected patients need dental prosthetic treatments during their developmental years. This report presents two cases of children affected by ectodermal dysplasia with anodontia. Oral rehabilitation was accomplished with removable acrylic prostheses. Treatment had major impacts on self-esteem, masticatory function, speech and facial esthetic.

  5. Malignant fibrous histiocytoma following radiation therapy of fibrous dysplasia: case report

    Energy Technology Data Exchange (ETDEWEB)

    Amin, R.; Ling, R. [Royal Devon and Exeter Hospital (United Kingdom)

    1995-10-01

    Malignant fibrous histiocytoma commonly occurs spontaneously. In some cases it follows previous therapeutic or incidental irradiation, or miscellaneous pre-existing osseous conditions. Recently, it has been associated with total hip arthroplasty. We report a case of malignant fibrous histocytoma following radiation therapy of fibrous dysplasia and review literature. (author).

  6. Craniofacial fibrous dysplasia - A review of current management techniques

    Directory of Open Access Journals (Sweden)

    Yadavalli Guruprasad

    2012-01-01

    Full Text Available Fibrous dysplasia is a pathologic condition of bone of unknown etiology with no apparent familial, hereditary or congenital basis. Lichtenstein first coined the term in 1938 and in 1942 he and Jaffe separated it from other fibro-osseous lesions. It is a bone tumor that, although benign, has the potential to cause significant cosmetic and functional disturbance, particularly in the craniofacial skeleton. Its management poses significant challenges to the surgeon. Craniofacial fibrous dysplasia is 1 of 3 types of fibrous dysplasia that can affect the bones of the craniofacial complex, including the mandible and maxilla. Fibrous dysplasia is a skeletal developmental disorder of the bone-forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Fibrous dysplasia represents about 2, 5% of all bone tumors and over 7% of all benign tumours. Over the years, we have gained a better understanding of its etiology, clinical behavior, and both surgical and non-surgical treatments.

  7. Actis Total Hip System 2 Year Follow-up

    Science.gov (United States)

    2017-08-14

    Osteoarthritis; Traumatic Arthritis; Rheumatoid Arthritis; Congenital Hip Dysplasia; Avascular Necrosis of the Femoral Head; Acute Traumatic Fracture of the Femoral Head or Neck; Certain Cases of Ankylosis; Non-union of Femoral Neck Fractures; Certain High Sub-Capital & Femoral Neck Fractures in Elderly

  8. Bone and soft tissue tumors of hip and pelvis

    Energy Technology Data Exchange (ETDEWEB)

    Bloem, Johan L., E-mail: j.l.bloem@lumc.nl [Leiden University Medical Center, Department of Radiology, PO Box 9600, 2300 RC Leiden (Netherlands); Reidsma, Inge I., E-mail: i.i.reidsma@lumc.nl [Leiden University Medical Center, Department of Radiology, PO Box 9600, 2300 RC Leiden (Netherlands)

    2012-12-15

    Objective is to identify epidemiologic and radiologic criteria allowing specific diagnoses of tumors and tumor-like lesions in the hip region and pelvis, and to optimize pre-operative staging. Patients with pelvic tumors are usually older, and their tumors are larger relative to patients with tumors in extremities. The majority of tumors in the pelvis are malignant (metastases, myeloma, chondrosarcoma, Ewing-, osteo-, and MFH/fibrosarcoma), while those in the proximal femur are in majority benign (fibrous dysplasia, solitary bone cyst, and osteoid osteoma). Soft tissue masses in the thigh in the elderly are typically sarcomas without tumor specific signs. Common tumor-like lesions occurring in the hip and pelvis that can mimic neoplasm are: infections (including tuberculosis), insufficiency/avulsion fractures, cysts, fibrous dysplasia, aneurysmal bone cyst, Langerhans cell histiocytosis, and Paget's disease. Local MR staging is based on the compartmental anatomy. The psoas and gluteal muscles are easily invaded by sarcoma originating in the ileum. The pectineus muscle protects the neurovascular bundle at the level of the hip. The thigh is separated into three compartments, some structures (Sartorius muscle) cross borders between compartments. Immobile joints (SI-joints, osteoarthritic hip) are relatively easily crossed by sarcoma and giant cell tumor.

  9. Refractory pain following hip arthroscopy: evaluation and management

    Science.gov (United States)

    de SA, Darren L; Burnham, Jeremy M; Mauro, Craig S

    2018-01-01

    ABSTRACT With increased knowledge and understanding of hip pathology, hip arthroscopy is rapidly becoming a popular treatment option for young patients with hip pain. Despite improved clinical and radiographic outcomes with arthroscopic treatment, some patients may have ongoing pain and less than satisfactory outcomes. While the reasons leading to failed hip arthroscopy are multifactorial, patient selection, surgical technique and rehabilitation all play a role. Patients with failed hip arthroscopy should undergo a thorough history and physical examination, as well as indicated imaging. A treatment plan should then be developed based on pertinent findings from the workup and in conjunction with the patient. Depending on the etiology of failed hip arthroscopy, management may be nonsurgical or surgical, which may include revision arthroscopic or open surgery, periacetabular osteotomy or joint arthroplasty. Revision surgery may be appropriate in settings including, but not limited to, incompletely treated femoroacetabular impingement, postoperative adhesions, heterotopic ossification, instability, hip dysplasia or advanced degeneration. PMID:29423245

  10. Association of Hip Radiograph Findings With Pain and Function in Patients Presenting With Low Back Pain.

    Science.gov (United States)

    Prather, Heidi; Cheng, Abby; Steger-May, Karen; Maheshwari, Vaibhav; VanDillen, Linda

    2018-01-01

    Relationships between low back pain (LBP) and the hip in patient cohorts have been described primarily in patients with moderate to severe hip osteoarthritis (OA). Less is known about the links of LBP with hip radiographic findings of hip deformity and minimal OA. To describe the incidence of radiographic hip deformity or hip OA; to describe and compare spine- and hip-related pain and function in the subset of patients who were found to have radiographic hip deformity or hip OA; and to compare patients with evidence of radiographic hip deformity or hip OA to patients without hip radiographic findings. Prospective cohort study with cross-sectional design. Tertiary university. A total of 63 patients (40 women, 23 men) with a mean age of 48.5 ± 14 years with LBP and a minimum of one positive provocative hip test. Hip radiographs were assessed by an independent examiner for hip OA and deformity. Comparisons of hip and lumbar spine pain and function were completed for patients with radiographic findings of hip OA or deformity. Moderate to severe hip OA was found in 12 of 60 patients (20.0%). At least one measurement of femoroacetabular impingement (FAI) was found in 14 of 60 patients (23.3%) to 33 of 45 patients (73.3%). At least one measurement of developmental hip dysplasia (DDH) was found in 7 of 60 patients (11.6%) to 11 of 63 patients (17.4%). Greater pain and reduced hip and lumbar spine function were found in the patients with moderate to severe hip OA. Patients with LBP and FAI were found to have significantly greater extremes of pain and reduced lumbar spine function. Links between the hip and the spine affecting pain and function may be found in patients with LBP and hip deformity and before the onset of radiographic hip OA, and may be associated with hip deformity. Further investigation is needed to better understand these links and their potential impact on prognosis and treatment of LBP. II. Copyright © 2018 American Academy of Physical Medicine and

  11. Total Hip Arthroplasty in Mucopolysaccharidosis Type IH

    Directory of Open Access Journals (Sweden)

    S. O'hEireamhoin

    2011-01-01

    Full Text Available Children affected by mucopolysaccharidosis (MPS type IH (Hurler Syndrome, an autosomal recessive metabolic disorder, are known to experience a range of musculoskeletal manifestations including spinal abnormalities, hand abnormalities, generalised joint stiffness, genu valgum, and hip dysplasia and avascular necrosis. Enzyme therapy, in the form of bone marrow transplantation, significantly increases life expectancy but does not prevent the development of the associated musculoskeletal disorders. We present the case of a 23-year-old woman with a diagnosis of Hurler syndrome with a satisfactory result following uncemented total hip arthroplasty.

  12. Upper-extremity phocomelia reexamined: a longitudinal dysplasia.

    Science.gov (United States)

    Goldfarb, Charles A; Manske, Paul R; Busa, Riccardo; Mills, Janith; Carter, Peter; Ezaki, Marybeth

    2005-12-01

    focal deficiency, were common in those patients. Eleven limbs in ten patients were identified as having severe combined dysplasia, which was type A in seven of them and type B in four. Four patients with severe combined dysplasia had congenital cardiac anomalies, and four had associated musculoskeletal abnormalities. Three of the four patients with the type-B disorder had a contralateral ulnar longitudinal dysplasia. We propose that cases previously classified as upper-extremity phocomelia represent a spectrum of severe longitudinal dysplasia, as none of the sixty extremities that we studied demonstrated a true intercalary deficiency. These findings have both developmental and genetic implications.

  13. Does the gestation age of newborn babies influence the ultrasonic assessment of hip condition?

    Directory of Open Access Journals (Sweden)

    Simić Slavica

    2009-01-01

    Full Text Available Introduction. Ultrasound represents a method of examination of hips of newborn babies capable of defining hip condition and distinguishing stable and unstable hips based on morphological elements. It is accepted in a large number of countries as a method of examination of high risk newborns, or as a method of systematic screening. Objective. The objective of this study was to investigate correlation between ultrasonically estimated hip maturity and respective gestation maturity both in premature and term-born babies, and to investigate the influence of different delivery types on hips condition. Methods. In our study 2045 patients, 1141 males and 904 females, were examined in at the Institute of Neonatology over a period of 5 years. The average age was 34.04 gestation weeks. There were significantly more premature (1698 or 83.03% than term-born babies (347 or 16.97%. Ultrasound hip examination, as a screening method, was carried out according to Graf. It was followed by clinical examination. Results were analyzed by appropriate statistical methods (χ2-test, one-way ANOVA, multifactor ANOVA. Results. The overall frequency of unstable hips was 3.2%, 1.88% in males and 4.87% in females (p<0.05. 96.8% babies had stable hips, out of which 35.21% were mature and 61.59% immature. In the study of the breech presentation, out of 183 babies, unstable hips were found in 1.58% of male cases, and in 10.23% of female cases. Conclusion. Clinical screening of developmental dysplasia of the hip is insufficient for early diagnosis and decision about the treatment of premature babies. The high frequency of unstable hip type IIc (risky and IId (decentralized in premature babies requires early diagnosis and therapy. Wide swaddling for prematures should be applied up to eight months of age. Gentle manipulation is necessary while nursing and conducting physiotherapy of a premature baby.

  14. Polyostotic Fibrous Dysplasia of Cranio-Maxillofacial Area

    Energy Technology Data Exchange (ETDEWEB)

    Han, Jin Woo; Kwon, Hyuk Rok; Lee, Jin Ho; Park, In Woo [Dept. of Oral and Maxillofacial Radiology, College of Dentistry, Kangnung National University, Kangnung (Korea, Republic of)

    2000-06-15

    Fibrous dysplasia is believed to be a hamartomatous developmental lesion of unknown origin. This disease is divided into monostotic and polyostotic fibrous dysplasia. Polyostotic type can be divided into craniofacial type, Lichtenstein-Jaffe type, and McCune-Albright syndrome. In this case, a 31-year-old female presented spontaneous loss of right mandibular teeth before 5 years and has shown continuous expansion of right mandibular alveolus. Through the radiographic view, the coarse pattern of the mixed radiopaque-lucent lesion was seen on the right mandibular body, and there was diffuse pattern of the mixed radiopaque-lucent lesion with ill-defined margin in the left mandibular body. In the right calvarium, the lesion had cotton-wool appearance. Partial excision for contouring, multiple extraction, and alveoloplasty were accomplished under general anesthesia for supportive treatment. Finally we could conclude this case was polyostotic fibrous dysplasia of cranio-maxillofacial area based on the clinical, radiologic finding, and histopathologic examination.

  15. Polyostotic Fibrous Dysplasia of Cranio-Maxillofacial Area

    International Nuclear Information System (INIS)

    Han, Jin Woo; Kwon, Hyuk Rok; Lee, Jin Ho; Park, In Woo

    2000-01-01

    Fibrous dysplasia is believed to be a hamartomatous developmental lesion of unknown origin. This disease is divided into monostotic and polyostotic fibrous dysplasia. Polyostotic type can be divided into craniofacial type, Lichtenstein-Jaffe type, and McCune-Albright syndrome. In this case, a 31-year-old female presented spontaneous loss of right mandibular teeth before 5 years and has shown continuous expansion of right mandibular alveolus. Through the radiographic view, the coarse pattern of the mixed radiopaque-lucent lesion was seen on the right mandibular body, and there was diffuse pattern of the mixed radiopaque-lucent lesion with ill-defined margin in the left mandibular body. In the right calvarium, the lesion had cotton-wool appearance. Partial excision for contouring, multiple extraction, and alveoloplasty were accomplished under general anesthesia for supportive treatment. Finally we could conclude this case was polyostotic fibrous dysplasia of cranio-maxillofacial area based on the clinical, radiologic finding, and histopathologic examination.

  16. Results of hip arthroplasty using Paavilainen technique in patients with congenitally dislocated hip

    Directory of Open Access Journals (Sweden)

    R. M. Tikhilov

    2014-01-01

    Full Text Available The purpose of the study was to analyze the medium- and long-term results of hip arthroplasty using Paavilainen technique in patients with the congenitally dislocated hip. Methods: From 2001 to 2012 180 operations were carried out were using the Paavilainen technique in 140 patients with high dislocation of the hip (Crowe IV. All patients were clinically evaluated using the Harris Hip Score (HHS, VAS and radiography. Statistical analysis was performed using the Pearson correlation coefficients, multiple regression analysis and classification trees analysis. Results: The average Harris score improved from preoperative 41.6 (40,3-43,5 to 79.3 (77,9-82,7 at final follow-up, and the difference was significant. Early complications were 9% (the most frequent were fractures of the proximal femur, later - 16.7% (pseudoarthrosis of the greater trochanter, 13.9%; disclocations-1,1%, aseptic loosening of the components - 1.7%, reoperation performed in 8.3% of cases. Such factors as age and limb length has statistically significant effect on functional outcomes. Established predictive model allows to get the best possible functional outcome in such patients with severe dysplasia. Conclusions: Total Hip arthroplasty using the Paavilainen technique is an effective method of surgical treatment in patients with the congenitally dislocated hip, but it is technically difficult operation with a high incidence of complications in comparison with standard primary total hip replacement.

  17. Developmental coxa vara associated with spondylometaphyseal dysplasia (DCV/SMD): ''SMD-Corner Fracture Type'' (DCV/SMD-CF) demonstrated in most reported cases

    International Nuclear Information System (INIS)

    Currarino, G.; Birch, J.G.; Herring, J.A.

    2000-01-01

    Background. This paper reports three children with short stature: developmental coxa vara unilateral in the first case and bilateral in the other two; somewhat squared and ''ovoid'' vertebral bodies in the first patient, and normal to slightly tall vertebral bodies in the third; metaphyseal changes in some long tubular bones including bone fragments similar to the corner fractures seen in child abuse in all three patients. Materials and methods. The first and second patients were sisters; their mother, also quite short, had surgical procedures in early life for bilateral ''coxa vara''; their brother, also of short stature, had bilateral coxa valga with otherwise normal femoral heads and necks, and mild metaphyseal changes associated with two minute ''corner fractures'' in the proximal metaphysis of the left tibia. Results. A review of reported cases of developmental coxa vara associated with spondylometaphyseal dysplasia revealed that simulated corner fractures were present in most instances. (orig.)

  18. The role of arthroscopy in the dysplastic hip—a systematic review of the intra-articular findings, and the outcomes utilizing hip arthroscopic surgery

    Science.gov (United States)

    Jo, Suenghwan; Lee, Sang Hong; Wang, Sung Il; Smith, Bjorn; O’Donnell, John

    2016-01-01

    Acetabular dysplasia is one of the most common sources of hip arthritis. With the recent innovation in hip arthroscopy, the question has been raised whether arthroscopy can be used to treat dysplastic hip conditions. The purposes of this systematic review are (i) describe the prevalence of intra-articular pathologies and (ii) report the outcomes of dysplastic hip treatment with hip arthroscopy as a sole treatment. Medical databases were searched for articles including arthroscopic findings and treatment of dysplastic hip with predetermined criteria. PubMed, Ovid database and CINAHL (Cumulative Index to Nursing and Allied Health Literature) were searched up until 7 January 2015. Two reviewers independently assess the eligibility of retrieved studies using titles, abstracts and full-text articles. Thirteen studies were eligible to be included for the systematic review. Overall, labral tear was the most common pathology with a prevalence rate of 77.3%. All of the four studies describing arthroscopic treatment for only borderline dysplasia reported favorable outcome. With regard to more severely dysplastic hips, two out of three studies reported acceptable outcomes while one study reported negative results. This review indicates that intra-articular pathology is commonly observed in symptomatic dysplastic hips with a labral tear being the most common pathology. Arthroscopic treatment of borderline dysplasia could provide benefits whereas treatment of more dysplastic hips is controversial. Nevertheless, there is a lack of evidence for using arthroscopy alone in hips with a center edge angle <20°. Level IV, systematic review of Level IV studies. PMID:27583155

  19. Case report of imaging analysis of the dysplasia epiphysealis hemimelic (Trevor's disease)

    International Nuclear Information System (INIS)

    Shu, Jang Ho; Cho, Kil Ho

    2013-01-01

    Trevor's disease, also known as dysplasia epiphysealis hemimelica, is a rare developmental disorder presented with epiphyseal overgrowth involving one or multiple epiphyses. Here we report the radiologic findings of two cases of dysplasia epiphysealis hemimelica in a 4-year-old boy in the knee without symptom and a 10-year-old boy in the ankle with pain. The former was observed for eight years and the latter was treated with surgical resection.

  20. Spondylo-megaepiphyseal-metaphyseal dysplasia: an unusual bone dysplasia

    International Nuclear Information System (INIS)

    Agarwal, Prachi Pragya; Srinivasan, Ashok; Sharma, Raju; Gupta, Arun Kumar; Kabra, Madhulika

    2003-01-01

    A rare case of spondylo-megaepiphyseal-metaphyseal dysplasia is reported in a 10-year-old boy. The features were metaphyseal dysplasia, markedly defective ossification of vertebral body centres and enlarged epiphyses. Although it shares some features with spondylo-metaphyseal dysplasia, oto-spondylo-megaepiphyseal dysplasia and cleidocranial dysplasia, the presence of several unusual radiological findings sets it apart. (orig.)

  1. TREATMENT OF NEUROLOGICAL CONGENITAL HIP LUXATION

    Directory of Open Access Journals (Sweden)

    Iulian ICLEANU

    2015-11-01

    Full Text Available Congenital hip luxation is a disorder which evolves in time. Teratological hip dislocation is a distinct form of hip luxation, which usually appears with other disorders. These hips are dislocated before birth. In this thesis we will try to elaborate a recovery program, through physical exercises, which will help us realize our treatment objectives: diminishing articular stiffness, increasing articular mobility, increasing muscle strength, recalibration of agonist and antagonist balances and reeducating gait. The specific objectives of the study consist of the particularization of the recovery programs based on age, illness stage (dysplasia or luxation and either surgical or non-surgical intervention. To show the importance of physiotherapy in gait rehabilitation of a child with hip dislocation we started from the hypothesis: using an adequate rehabilitation program after an individualized methodology, optimizes the functional recovery and ensures the gains of hip stability and the formation of an engram of gait as close as it could be to the normal one. We present a case of neurological congenital hip dislocation where the treatment initiated early is showing good results. Results obtained are significantly different and we came to the conclusion that starting an untimely analytical kinetic treatment and globally personalizing it to every patient has better biomechanical results for the hip.

  2. A case of Mondini dysplasia with recurrent Streptococcus pneumoniae meningitis.

    Science.gov (United States)

    Yilmaz Ciftdoğan, Dilek; Bayram, Nuri; Ozdemir, Yasemin; Bayraktaroğlu, Selen; Vardar, Fadil

    2009-12-01

    Mondini's dysplasia is a developmental anomaly of the middle ear characterized by cochlear malformation with dilation of the vestibular aquaduct, vestibule, and ampullar ends of the semicircular canals. These deformities may result in a connection between subarachnoid space and the middle ear resulting in recurrent episodes of meningitis. Additionally, it is commonly associated with hearing impairment. We describe here a boy with recurrent meningitis and unilateral sensorineural hearing loss. Mondini dysplasia was demonstrated with computed tomographic scans of the temporal bones in the search for pathogenesis of recurrent meningitis.

  3. Hip and pelvis diseases on lumbar AP radiographs including both hip joints

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Hyun Soo; Juhng, Seon Kwan; Kim, Eun A; Kim, Jeong Ho; Song, Ha Heon; Shim, Dae Moo [Wonkwang University School of Medicine, Iksan (Korea, Republic of)

    2002-12-01

    To determine the frequency of disease, and to evaluate the methods used for lumbar spine radiography in Korea. Sixty university and training hospitals were randomly selected and asked to describe the projections, film size and radiographic techniques employed for routine radiography in patients with suspected disease of the lumbar spine. Plain radiographs of 1215 patients, taken using 14x17 inch film and depicting both hip joints and the lumbar region, were analysed between March 1999 and February 2000. In 15 patients (1.2%), the radiographs revealed hip or pelvic lesion, confirmed as follows: avascular necrosis of the femoral head (n=11, with bilateral lesion in four cases); sustained ankylosing spondylitis (n=2); acetabular dysplasia (n=1); and insufficiency fracture of the pubic rami secondary to osteoporosis (n=1). In 11 or the 20 hospitals which responded, 14{sup x}17{sup f}ilm was being used for lumbar radiography, while in the other nine, film size was smaller. Plain radiography of the lumbar spine including both hip joints, may be a useful way to simultaneously evaluate lesions not only of the lumbar spine but also of the hip and/or pelvis.

  4. Camptomelic dysplasia: A case report

    Directory of Open Access Journals (Sweden)

    Koš Radmila

    2007-01-01

    Full Text Available Campomelic/camptomelic dysplasia is a very rare, severe osteochondrodysplasia characterised by severe skeletal and nonskeletal malformations and lethal outcome mainly in neonatal period. Characteristic abnormality by which the syndrome got its name is short, bowed long bones of lower extremities, most often of femur, manifested by short and bowed legs. Skin dimpling on tibial anterior side is another prominent characteristic of this syndrome. Severe cases are inherited by autosomal dominant trait, by mutation Sox9 gene on chromosome 17, with lethal outcome in the first days of life. Less severe forms of the disease are due to balanced translocation t (13;17 with life span up to the third decade of life. A majority of karyotypic males present as phenotypic females. We report a case of a female neonate, without consanguinity between parents, with characteristic signs of camptomelic dysplasia with short birth length of 46 cm, macrocephaly (head circumference 39 cm, dolichocephaly, hydrocephalus, short trunk and legs. Narrow rib cage, bowed lower extremities, short hand and foot phalanges, nail hypoplasia were noticed. Anterior fontanelle was enlarged, high forehead, face small and flat, hypertelorism, low nasal bridge, micrognathia, low set ears, cleft palate, were found. Characteristic skin dimpling on anterior side of tibia was present on both legs. Bone X-ray studies presented the following changes: anterior bowing of shortened femurs, hip dislocation, cervical vertebrae, scapulas, eleven pairs of slender ribs. Hip luxation. Karyotype was normal for a female, 46 XX. Respiratory insufficiency was present since birth, exacerbated, and led to lethal outcome in the second day of life, as described in the majority of these patients. .

  5. Osteosclerotic metaphyseal dysplasia: a skeletal dysplasia that may mimic lead poisoning in a child with hypotonia and seizures

    International Nuclear Information System (INIS)

    Mennel, Emilie A.; John, Susan D.

    2003-01-01

    We report the case of a 23-month-old male with hypotonia, developmental delay, and complex seizures. Radiographs revealed profound sclerosis of the metaphyses and epiphyses of the long and short bones in the extremities, with a unique pattern of distribution. Sclerosis also involved the anterior ribs, iliac crests, talus, and calcaneus. The skull and vertebral bodies appeared unaffected. Blood lead levels were normal. We believe that this constellation of clinical and radiographic abnormalities closely resembles osteosclerotic metaphyseal dysplasia (OMD) due to an autosomal recessive defect. Characteristic skeletal findings were instrumental in determining the diagnosis. OMD is a very rare sclerosing bone disorder, first described in 1993. The syndrome is characterized clinically by developmental delay of a progressive nature, hypotonia, elevated alkaline phosphatase, and late-onset spastic paraplegia. We encountered a young child with these neurologic symptoms who displayed sclerotic metaphyseal changes on hand radiographs obtained to determine the bone age. Lead poisoning, a known cause of metaphyseal sclerosis, was initially suspected. Careful analysis of the metaphyseal bone changes helped to distinguish this bone dysplasia from lead poisoning and other causes of metaphyseal sclerosis. (orig.)

  6. Radiological features of bilateral hereditary micro-epiphyseal dysplasia - a distinct entity in the skeletal dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Mostert, A.K. [Isala Clinics, Location Weezenlanden, Dept. of Orthopaedic Surgery, Zwolle (Netherlands); Dijkstra, P.F. [Jan van Breemen Inst., Dept. of Radiology, Amsterdam (Netherlands); Horn, J.R. van [Univ. Hospital Groningen, Dept. of Orthopaedic Surgery, Groningen (Netherlands); Jansen, B.R.H. [Reinier de Graaf Hospital, Dept. of Orthopaedic Surgery, Delft (Netherlands); Heutink, P. [Erasmus MCRotterdam, Dept. of Clinical Genetics, Rotterdam (Netherlands); Lindhout, D. [Univ. Medical Centre Utrecht, Dept. of Medical Genetics, Utrecht (Netherlands)

    2002-07-01

    Aim: To prove that bilateral hereditary micro-epiphyseal dysplasia (BHMED), first described by Elsbach in 1959, is a distinct disorder radiologically as well as clinically, compared with multiple epiphyseal dysplasia (MED). Material and Methods: We used the data of the revised pedigree with 84 family members, performed a medical history, physical examination and made a radiological evaluation for defining a clinical and radiological phenotype of BHMED family members. We used blood samples for genetic analysis. Results: Although there is a clear clinical picture of the dysplasia, the radiological signs are more reliable for making the diagnosis. Especially the typical deformity of the hip and knee joint are diagnostic for BHMED. By linkage analysis we excluded linkage with the three known MED-loci (EDM1, EDM2 and EDM3). Conclusion: BHMED is indeed an entity that is distinct from common multiple epiphyseal dysplasia (MED), clinically, as well as radiologically and genetically. (orig.) [German] Ziel: Es sollte dargelegt werden, dass sich eine vererbliche, laterale Mikro-Epiphysendysplasie (BHMED), Erstbeschreibung durch Elsbach 1959, klinisch, radiologisch und genetisch von einer mutiplen Epiphysendysplasie (MED) unterscheidet. Material und Methode: Anhand der Daten eines ueberarbeiteten Stammbaumes mit 84 Familienmitgliedern wurde der medizinische Werdegang rekonstruiert. Es erfolgte eine physische Untersuchung der Familienmitglieder. Schliesslich wurde eine radiologische Auswertung durchgefuehrt, um einen klinischen und radiologischen Phaenotyp der von BHMED betroffenen Familienmitglieder zu definieren. Fuer eine genetische Analyse wurden Blutproben entnommen. Ergebnisse: Obwohl es ein deutliches klinisches Bild einer Dysplasie gibt, sind die radiologischen Kennzeichen fuer die Diagnose zuverlaessiger. Insbesondere die typische Deformation der Huefte und des Kniegelenks ist diagnostisch fuer BHMED. Durch Linkage-Analyse konnte eine Verbindung zu den drei bekannten

  7. Septo-optic dysplasia plus: clinical presentation and magnetic resonance imaging findings

    International Nuclear Information System (INIS)

    Matushita Junior, Joao Paulo K.; Tiel, Chan; Py, Marco; Batista, Raquel Ribeiro; Gasparetto, Emerson L.

    2010-01-01

    Septo-optic dysplasia (SOD) is a heterogeneous developmental malformation characterized by optic nerve hypoplasia associated with dysgenesis of the septum pellucidum and other cerebral malformations. The clinical manifestations include psychomotor retardation, visual impairment, thermoregulatory disturbances, conjugated hyperbilirubinemia and seizures. In 2000, Miller et al. first named the association of SOD and cortical dysplasia as SOD-plus. In this report, all the cases had severe clinical impairment, presenting global developmental delay and spastic motor deficits. Subsequent reports of SOD-plus also stressed the psychomotor development delay, spastic motor deficits and seizures seen in these patients, emphasizing the severity of the brain involvement. Recently, Kwak et al. reported a case of SOD-plus presenting with cortical dysplasia involving the insular cortex bilaterally. However, differently from all the previous reports, the patient had no signs or symptoms of cortical dysfunction, except for one episode of seizure. We report an additional case of SOD-plus, which presented extensive cortical malformation, with no signs or symptoms of cortical dysfunction. We suggest that similar to the classical form of SOD, the clinical presentation in patients with SOD-plus can also range from mild to extremely severe. (author)

  8. Septo-optic dysplasia plus: clinical presentation and magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Matushita Junior, Joao Paulo K. [Instituto de Pos-Graduacao Medica Carlos Chagas (IPGMCC), Rio de Janeiro, RJ (Brazil); Tiel, Chan; Py, Marco [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Inst. de Neurologia Deolindo Couto; Batista, Raquel Ribeiro [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Hospital Universitario Clementino Fraga Filho; Gasparetto, Emerson L., E-mail: egasparetto@gmail.co [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Dept. de Radiologia

    2010-04-15

    Septo-optic dysplasia (SOD) is a heterogeneous developmental malformation characterized by optic nerve hypoplasia associated with dysgenesis of the septum pellucidum and other cerebral malformations. The clinical manifestations include psychomotor retardation, visual impairment, thermoregulatory disturbances, conjugated hyperbilirubinemia and seizures. In 2000, Miller et al. first named the association of SOD and cortical dysplasia as SOD-plus. In this report, all the cases had severe clinical impairment, presenting global developmental delay and spastic motor deficits. Subsequent reports of SOD-plus also stressed the psychomotor development delay, spastic motor deficits and seizures seen in these patients, emphasizing the severity of the brain involvement. Recently, Kwak et al. reported a case of SOD-plus presenting with cortical dysplasia involving the insular cortex bilaterally. However, differently from all the previous reports, the patient had no signs or symptoms of cortical dysfunction, except for one episode of seizure. We report an additional case of SOD-plus, which presented extensive cortical malformation, with no signs or symptoms of cortical dysfunction. We suggest that similar to the classical form of SOD, the clinical presentation in patients with SOD-plus can also range from mild to extremely severe. (author)

  9. Pathogenetics of alveolar capillary dysplasia with misalignment of pulmonary veins

    NARCIS (Netherlands)

    Szafranski, P.; Gambin, T.; Dharmadhikari, A.V.; Akdemir, K.C.; Jhangiani, S.N.; Schuette, J.; Godiwala, N.; Yatsenko, S.A.; Sebastian, J.; Madan-Khetarpal, S.; Surti, U.; Abellar, R.G.; Bateman, D.A.; Wilson, A.L.; Markham, M.H.; Slamon, J.; Santos-Simarro, F.; Palomares, M.; Nevado, J.; Lapunzina, P.; Chung, B.H.; Wong, W.L.; Chu, Y.W.; Mok, G.T.; Kerem, E.; Reiter, J.; Ambalavanan, N.; Anderson, S.A.; Kelly, D.R.; Shieh, J.; Rosenthal, T.C.; Scheible, K.; Steiner, L.; Iqbal, M.A.; McKinnon, M.L.; Hamilton, S.J.; Schlade-Bartusiak, K.; English, D.; Hendson, G.; Roeder, E.R.; DeNapoli, T.S.; Littlejohn, R.O.; Wolff, D.J.; Wagner, C.L.; Yeung, A.; Francis, D.; Fiorino, E.K.; Edelman, M.; Fox, J.; Hayes, D.A.; Janssens, S.; Baere, E. De; Menten, B.; Loccufier, A.; Vanwalleghem, L.; Moerman, P.; Sznajer, Y.; Lay, A.S.; Kussmann, J.L.; Chawla, J.; Payton, D.J.; Phillips, G.E.; Brosens, E.; Tibboel, D.; Klein, A.; Maystadt, I.; Fisher, R.; Sebire, N.; Male, A.; Chopra, M.; Pinner, J.; Malcolm, G.; Peters, G.; Arbuckle, S.; Lees, M.; Mead, Z.; Quarrell, O.; Sayers, R.; Owens, M.; Shaw-Smith, C.; Lioy, J.; McKay, E.; Leeuw, N. de; Feenstra, I.; Spruijt, L.; Elmslie, F.; Thiruchelvam, T.; Bacino, C.A.; Langston, C.; Lupski, J.R.; Sen, P.; Popek, E.; Stankiewicz, P.

    2016-01-01

    Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a lethal lung developmental disorder caused by heterozygous point mutations or genomic deletion copy-number variants (CNVs) of FOXF1 or its upstream enhancer involving fetal lung-expressed long noncoding RNA genes

  10. Assessment of the hip reduction angle for predicting osteoarthritis of the hip in the Labrador Retriever.

    Science.gov (United States)

    Corfield, G S; Read, R A; Eastley, K A; Richardson, J L; Robertson, I D; Day, R

    2007-06-01

    Hip palpation has been used to provide semiquantitative information regarding passive joint laxity and susceptibility to hip dysplasia. The purpose of this study was to: (1) evaluate the intra- and inter-examiner repeatability of the hip reduction angle measured at 4 months of age by three examiners using manual goniometry and an electromagnetic tracking system; (2) compare the hip reduction angle measured with manual goniometry to the hip reduction angle measured with the electromagnetic tracking system; and (3) evaluate the hip reduction angle, distraction index and Ortolani manoeuvre at 4 months of age as predictors of the development of hip osteoarthritis at 12 months of age in 11 Labrador Retriever dogs. Intra- and inter-examiner repeatability was demonstrated for both the manual and electromagnetic goniometric measurement of the hip reduction angle (coefficient of variation goniometry was moderately correlated to the hip reduction angle measured by the electromagnetic tracking system (r = 0.603, P goniometry was a poor predictor of osteoarthritis at 12 months of age (r = 0.231, P < 0.062, and r = 0.321, P < 0.01). The distraction index was moderately correlated with the development of osteoarthritis by 12 months of age (r = 0.493, P < 0.0000). The Ortolani sign was sensitive (100%) but not specific (41%) for the development of osteoarthritis at 12 months of age. The hip reduction angle did not further quantify the Ortolani manoeuvre as a predictor of osteoarthritis in Labrador Retrievers.

  11. A Modified Triple Pelvic Osteotomy for the Treatment of Hip Hypoplasia

    Directory of Open Access Journals (Sweden)

    Hassan Rahimi

    2013-09-01

    Full Text Available Background: The prevalence of hip dysplasia is 1 in 1000. Several pelvic osteotomy methods have been developed to prevent early osteoarthritis, such as triple osteotomy. In this study we are going to introduce our new technique that was done on 4 patients with favorable short-term results.   Methods: Four patients underwent triple osteotomy and fixation using a reconstruction plate and early weight bearing was started. Results: The Harris Hip Score, limb length, center-edge angle, and acetabular inclination showed improvement. Conclusion: This modified technique is suggested for corrective surgery on adult dysplastic hips.

  12. Is Cup Positioning Challenged in Hips Previously Treated With Periacetabular Osteotomy?

    DEFF Research Database (Denmark)

    Hartig-Andreasen, Charlotte; Stilling, Maiken; Søballe, Kjeld

    2014-01-01

    After periacetabular osteotomy (PAO), some patients develop osteoarthritis with need of a total hip arthroplasty (THA). We evaluated the outcome of THA following PAO and explored factors associated with inferior cup position and increased polyethylene wear. Follow-up were performed 4 to 10years...... after THA in 34 patients (38 hips) with previous PAO. Computer analysis evaluated cup position and wear rates. No patient had dislocations or revision surgery. Median scores were: Harris hip 96, Oxford hip 38 and WOMAC 78. Mean cup anteversion and abduction angles were 22(o) (range 7°-43°) and 45......° (range 28°-65°). Outliers of cup abduction were associated with persisting dysplasia (CE...

  13. Case report of imaging analysis of the dysplasia epiphysealis hemimelic (Trevor's disease)

    Energy Technology Data Exchange (ETDEWEB)

    Shu, Jang Ho; Cho, Kil Ho [Dept. of Diagnostic Radiology, College of Medicine, Yeungnam University, Daegu (Korea, Republic of)

    2013-08-15

    Trevor's disease, also known as dysplasia epiphysealis hemimelica, is a rare developmental disorder presented with epiphyseal overgrowth involving one or multiple epiphyses. Here we report the radiologic findings of two cases of dysplasia epiphysealis hemimelica in a 4-year-old boy in the knee without symptom and a 10-year-old boy in the ankle with pain. The former was observed for eight years and the latter was treated with surgical resection.

  14. Surgical Management of Hip Problems in Myelomeningocele: A Review Article

    Directory of Open Access Journals (Sweden)

    Taghi Baghdadi

    2016-07-01

    Full Text Available Background: Children with myelomeningocele (MMC develop a wide variety of hip deformities such as muscle imbalance, contracture, subluxation, and dislocation. Various methods and indications have been introduced for treatment of muscle imbalances and other hip problems in patients with MMC but there is no study or meta-analysis to compare the results and complications. This review aims to find the most acceptable approach to hip problems in patients with MMC. Methods: MEDLINE was searched up to April 2015. All study designs that reported on the outcomes of hip problems in MMC were included. From 270 screened citations, 55 were strictly focused on hip problem in MMC were selected and reviewed. Results: Complex osseous and soft tissue reconstructive procedures to correct hip dysplasia and muscle balancing around the hip are rarely indicated for MMC patients without good quadriceps power. Conclusion: Over the years a consensus on the best algorithm for treatment of hip dislocation in myelomeningocele has been missing, however, muscular balancing with/out osseous procedure seems a reasonable approach especially in unilateral mid-lumbar MMC.

  15. Identification and validation of quantitative trait loci (QTL for canine hip dysplasia (CHD in German Shepherd Dogs.

    Directory of Open Access Journals (Sweden)

    Lena Fels

    Full Text Available Canine hip dysplasia (CHD is the most common hereditary skeletal disorder in dogs. To identify common alleles associated with CHD, we genotyped 96 German Shepherd Dogs affected by mild, moderate and severe CHD and 96 breed, sex, age and birth year matched controls using the Affymetrix canine high density SNP chip. A mixed linear model analysis identified five SNPs associated with CHD scores on dog chromosomes (CFA 19, 24, 26 and 34. These five SNPs were validated in a by sex, age, birth year and coancestry stratified sample of 843 German Shepherd Dogs including 277 unaffected dogs and 566 CHD-affected dogs. Mean coancestry coefficients among and within cases and controls were <0.1%. Genotype effects of these SNPs explained 20-32% of the phenotypic variance of CHD in German Shepherd Dogs employed for validation. Genome-wide significance in the validation data set could be shown for each one CHD-associated SNP on CFA24, 26 and 34. These SNPs are located within or in close proximity of genes involved in bone formation and related through a joint network. The present study validated positional candidate genes within two previously known quantitative trait loci (QTL and a novel QTL for CHD in German Shepherd Dogs.

  16. Prosthodontic management of hypohidrotic ectodermal dysplasia: a case report

    Directory of Open Access Journals (Sweden)

    Emilija Bajraktarova Valjakova

    2015-09-01

    Full Text Available Introduction: Ectodermal dysplasia (ED is a hereditary disorder associated with developmental disorders of two or more structures of ectodermal embryonic origin. Hypodontia or anodontia of the primary and permanent dentition, poorly developed alveolar ridges and improper maxillo – mandibular relations, are the most common oral manifestations. Management of patients with ectodermal dysplasia requires a multidisciplinary team approach. Case presentation: A 6.5 year-old boy with hypohidrotic ectodermal dysplasia (HED presented in this article, had typical features of HED: hypohidrosis, hypotrichosis, severe hypodontia, atrophic alveolar ridges, old-looking facial expression. According to the patient’s age and clinical findings, removable complete over-denture prosthesis in both arches was the treatment of choice. Conclusion: In patients with ED, it is important to establish correct maxillo – mandibular relations and normal function of the dento-facial system (chewing, swallowing, and speaking. Prosthodontic treatment has a major impact on aesthetics and functions,facilitates psychological development and improves emotional condition and social life of the patient.

  17. Hip Joint Trevor Disease: Literature Review and a Case Report

    Directory of Open Access Journals (Sweden)

    Mohammad Hallaj Moghadam

    2018-01-01

    Full Text Available Trevor disease or dysplasia epiphysealis hemimelica (DEH is an extremely rare condition with incidence of about 1:1,000,000. Male to female ratio of reporting case is 3:1, and usually diagnosed between two and eight years old. It usually affects the medial portion of the joint, but lateral involvement is not uncommon. Hip-joint was affected in less than 4% of existing cases in the literature. It would be very important to precisely mange the hip involvement to prevent from further articular cartilage destruction in this very young age. We report an infant boy with isolated DEH of hip. We found a total of 271 cases of DEH that reported between 1926 and 2017.The most sites of involvement are ankle joint and around the knee. Our search reaches out to ten cases of hip involvement. Hip involvement needs a patient specified decision. We observed our patient for three years with a desirable hip joint function.

  18. Ectodermal dysplasias

    Science.gov (United States)

    Anhidrotic ectodermal dysplasia; Christ-Siemens-Touraine syndrome; Anondontia; Incontinentia pigmenti ... These resources can provide more information on ectodermal dysplasias: Ectodermal Dysplasia Society -- www.ectodermaldysplasia.org National Foundation for ...

  19. Gross Instability After Hip Arthroscopy: An Analysis of Case Reports Evaluating Surgical and Patient Factors.

    Science.gov (United States)

    Yeung, Marco; Memon, Muzammil; Simunovic, Nicole; Belzile, Etienne; Philippon, Marc J; Ayeni, Olufemi R

    2016-06-01

    Gross hip instability is a rare complication after hip arthroscopy, and there is limited literature surrounding this topic. This systematic review investigates cases of gross hip instability after arthroscopy and discusses the risk factors associated with this complication. A systematic search was performed in duplicate for studies investigating gross hip instability after hip arthroscopy up to October 2015. Study parameters including sample size, mechanism and type of dislocation, surgical procedure details, patient characteristics, postoperative rehabilitation protocol, and level of evidence were analyzed. The systematic review identified 9 case reports investigating gross hip instability after hip arthroscopy (10 patients). Anterior dislocation occurred in 66.7% of patients, and most injuries occurred with a low-energy mechanism. Common surgical factors cited included unrepaired capsulotomy (77.8%) and iliopsoas release (33.3%), whereas patient factors included female gender (77.8%), acetabular dysplasia (22.2%), and general ligamentous laxity (11.1%). Postoperative restrictions and protocols were variable and inconsistently reported, and their relation to post-arthroscopy instability was difficult to ascertain. This systematic review discussed various patient, surgical, and postoperative risk factors of gross hip instability after arthroscopy. Patient characteristics such as female gender, hip dysplasia, and ligamentous laxity may be risk factors for post-arthroscopy dislocation. Similarly, surgical risk factors for iatrogenic hip instability may include unrepaired capsulotomies and iliopsoas debridement, although the role of capsular closure in iatrogenic instability is not clear. The influences of postoperative restrictions and protocols on dislocation are also unclear in the current literature. Surgeons should be cognizant of these risk factors when performing hip arthroscopy and be mindful that these factors appear to occur in combination. Level IV

  20. Recurrent bacterial meningitis in a child with mondini dysplasia.

    Science.gov (United States)

    Kepenekli-Kadayifci, Eda; Karaaslan, Ayşe; Atıcı, Serkan; Binnetoğlu, Adem; Sarı, Murat; Soysal, Ahmet; Altınkanat, Gülşen; Bakır, Mustafa

    2014-01-01

    Mondini dysplasia, also known as Mondini malformation, is a developmental abnormality of the inner and middle ears that can cause hearing loss, cerebrospinal fluid (CSF) leakage, and recurrent bacterial meningitis (RBM), which is defined as two or more episodes of meningitis separated by a period of convalescence and the complete resolution of all signs and symptoms. An accurate diagnosis of the underlying pathology is crucial to prevent further episodes from occurring. Herein, we present a three-year-old boy with RBM and unilateral sensorineural hearing loss. During the evaluation to determine the etiology of the RBM, cystic malformation in the cochlea and vestibular dilatation consistent with Mondini dysplasia were detected via computerized tomography (CT) of the temporal bone.

  1. Corrections of lower limb deformities in patients with diastrophic dysplasia.

    Science.gov (United States)

    Al Kaissi, Ali; Kenis, Vladimir; Melchenko, Eugeniy; Chehida, Farid Ben; Ganger, Rudolf; Klaushofer, Klaus; Grill, Franz

    2014-11-01

    Accurate understanding of the cause of the underlying pathology in children with diastrophic dysplasia would help in designing targeted management of their locomotion. Diastrophic dysplasia was diagnosed in twelve patients (nine girls and three boys; age range 1-14 years), all of whom presented with small stature and apparent short extremities. Club foot (mostly talipes equinovarus) was the most frequent and consistent abnormality. Concomitant abnormalities such as hip flexion contracture, flexion contractures of the knees with excessive valgus deformity and lateral patellar subluxation, were also encountered. Muscle ultrasound and muscle magnetic resonance imaging imaging showed no myopathic changes and muscle biopsies and the respiratory chain were normal. Serum choline kinase and plasma lactate concentrations were normal. Surgical correction of the foot and ankle in patients with diastrophic dysplasia is extremely difficult because of the markedly distorted anatomy. In all of these children, plantigrade foot was achieved along with the improved function of the locomotor system. Mutations of the diastrophic dysplasia sulfate transporter (also known as solute carrier family 26 member 2) were encountered. Arthrogryposis multiplex is the usual terminology used to describe the abnormality in infants with multiple contractures. Diligent orthopaedic care should be provided based on an accurate understanding of the associated syndromes in such children. © 2014 Chinese Orthopaedic Association and Wiley Publishing Asia Pty Ltd.

  2. Multicentre study on capsular closure versus non-capsular closure during hip arthroscopy in Danish patients with femoroacetabular impingement (FAI)

    DEFF Research Database (Denmark)

    Dippmann, Christian; Kraemer, Otto; Lund, Bent

    2018-01-01

    in patients without additional risk factors for instability such as hypermobility or dysplasia of the hip. We hypothesised that capsular closure will lead to a superior outcome in hip arthroscopy for femoroacetabular impingement syndrome (FAIS) compared with non-capsular closure. METHODS AND ANALYSIS...... years and FAIS according to the Warwick agreement. Exclusion criteria are: previous hip surgery in either hip, previous conditions of Legg-Calvé-Perthes or slipped capital femoral epiphysis, malignant disease, recent hip or pelvic fractures, arthritis, Ehlers-Danlos or Marfan disease, recent (within 6...

  3. Genetics of hip dysplasia, elbow dysplasia and patellar luxation in purebred dogs

    NARCIS (Netherlands)

    Lavrijsen, I.C.M.

    2014-01-01

    Hereditary developmental orthopaedic diseases pose a serious threat to the quality of life of dogs. The pain as well as the detrimental effect on mobility that can accompany these disorders have a major impact on the dogs as well as their owners. The studies described in this thesis aim to give more

  4. Recurrent Bacterial Meningitis in a Child with Mondini Dysplasia

    Directory of Open Access Journals (Sweden)

    Eda Kepenekli-Kadayifci

    2014-01-01

    Full Text Available Mondini dysplasia, also known as Mondini malformation, is a developmental abnormality of the inner and middle ears that can cause hearing loss, cerebrospinal fluid (CSF leakage, and recurrent bacterial meningitis (RBM, which is defined as two or more episodes of meningitis separated by a period of convalescence and the complete resolution of all signs and symptoms. An accurate diagnosis of the underlying pathology is crucial to prevent further episodes from occurring. Herein, we present a three-year-old boy with RBM and unilateral sensorineural hearing loss. During the evaluation to determine the etiology of the RBM, cystic malformation in the cochlea and vestibular dilatation consistent with Mondini dysplasia were detected via computerized tomography (CT of the temporal bone.

  5. Risk factors for the need of hip arthroscopy following periacetabular osteotomy

    DEFF Research Database (Denmark)

    Hartig-Andreasen, Charlotte; Troelsen, Anders; Thillemann, Theis M

    2015-01-01

    was to identify risk factors predicting the need for a hip arthroscopy (HA) after periacetabular osteotomy (PAO). Ninety-nine patients (104 hips) scheduled for PAO were evaluated preoperatively and at 2-year follow-up. MRA was performed in all patients prior to PAO. At follow-up, patients were divided into a non-arthroscopy...... and arthroscopy group. The two groups were compared clinical and radiological, and risk factors for HA after PAO were calculated. Patient reported outcome measures (WOMAC, Oxford Hip and SF36) were filled out before PAO and at follow-up. Ninety-five hips (91.3%) were evaluated. Twenty-six hips (27%) required...... an arthroscopy within 2 years of the PAO. Risk factors were preoperative borderline dysplasia, acetabular retroversion and complete labral detachment. Labral tearing, degeneration or hypertrophy did not negatively affect the outcome of PAO. Patients not requiring an arthroscopy had a statistically significant...

  6. Can 3D ultrasound identify trochlea dysplasia in newborns? Evaluation and applicability of a technique

    Energy Technology Data Exchange (ETDEWEB)

    Kohlhof, Hendrik, E-mail: Hendrik.Kohlhof@ukb.uni-bonn.de [Clinic for Orthopedics and Trauma Surgery, University Hospital Bonn, Sigmund-Freud-Str. 25, 53127 Bonn (Germany); Heidt, Christoph, E-mail: Christoph.heidt@kispi.uzh.ch [Department of Orthopedic Surgery, University Children' s Hospital Zurich, Steinwiesstrasse 74, 8032 Switzerland (Switzerland); Bähler, Alexandrine, E-mail: Alexandrine.baehler@insel.ch [Department of Pediatric Radiology, University Children' s Hospital Berne, Freiburgstrasse 18, 3010 Berne (Switzerland); Kohl, Sandro, E-mail: sandro.kohl@insel.ch [Department of Orthopedic Surgery, University Hospital Berne, Freiburgstrasse 18, 3010 Berne (Switzerland); Gravius, Sascha, E-mail: sascha.gravius@ukb.uni-bonn.de [Clinic for Orthopedics and Trauma Surgery, University Hospital Bonn, Sigmund-Freud-Str. 25, 53127 Bonn (Germany); Friedrich, Max J., E-mail: Max.Friedrich@ukb.uni-bonn.de [Clinic for Orthopedics and Trauma Surgery, University Hospital Bonn, Sigmund-Freud-Str. 25, 53127 Bonn (Germany); Ziebarth, Kai, E-mail: kai.ziebarth@insel.ch [Department of Orthopedic Surgery, University Hospital Berne, Freiburgstrasse 18, 3010 Berne (Switzerland); Stranzinger, Enno, E-mail: Enno.Stranzinger@insel.ch [Department of Pediatric Radiology, University Children' s Hospital Berne, Freiburgstrasse 18, 3010 Berne (Switzerland)

    2015-06-15

    Highlights: • We evaluated a possible screening method for trochlea dysplasia. • 3D ultrasound was used to perform the measurements on standardized axial planes. • The evaluation of the technique showed comparable results to other studies. • This technique may be used as a screening technique as it is quick and easy to perform. - Abstract: Femoro-patellar dysplasia is considered as a significant risk factor of patellar instability. Different studies suggest that the shape of the trochlea is already developed in early childhood. Therefore early identification of a dysplastic configuration might be relevant information for the treating physician. An easy applicable routine screening of the trochlea is yet not available. The purpose of this study was to establish and evaluate a screening method for femoro-patellar dysplasia using 3D ultrasound. From 2012 to 2013 we prospectively imaged 160 consecutive femoro-patellar joints in 80 newborns from the 36th to 61st gestational week that underwent a routine hip sonography (Graf). All ultrasounds were performed by a pediatric radiologist with only minimal additional time to the routine hip ultrasound. In 30° flexion of the knee, axial, coronal, and sagittal reformats were used to standardize a reconstructed axial plane through the femoral condyle and the mid-patella. The sulcus angle, the lateral-to-medial facet ratio of the trochlea and the shape of the patella (Wiberg Classification) were evaluated. In all examinations reconstruction of the standardized axial plane was achieved, the mean trochlea angle was 149.1° (SD 4.9°), the lateral-to-medial facet ratio of the trochlea ratio was 1.3 (SD 0.22), and a Wiberg type I patella was found in 95% of the newborn. No statistical difference was detected between boys and girls. Using standardized reconstructions of the axial plane allows measurements to be made with lower operator dependency and higher accuracy in a short time. Therefore 3D ultrasound is an easy

  7. Can 3D ultrasound identify trochlea dysplasia in newborns? Evaluation and applicability of a technique

    International Nuclear Information System (INIS)

    Kohlhof, Hendrik; Heidt, Christoph; Bähler, Alexandrine; Kohl, Sandro; Gravius, Sascha; Friedrich, Max J.; Ziebarth, Kai; Stranzinger, Enno

    2015-01-01

    Highlights: • We evaluated a possible screening method for trochlea dysplasia. • 3D ultrasound was used to perform the measurements on standardized axial planes. • The evaluation of the technique showed comparable results to other studies. • This technique may be used as a screening technique as it is quick and easy to perform. - Abstract: Femoro-patellar dysplasia is considered as a significant risk factor of patellar instability. Different studies suggest that the shape of the trochlea is already developed in early childhood. Therefore early identification of a dysplastic configuration might be relevant information for the treating physician. An easy applicable routine screening of the trochlea is yet not available. The purpose of this study was to establish and evaluate a screening method for femoro-patellar dysplasia using 3D ultrasound. From 2012 to 2013 we prospectively imaged 160 consecutive femoro-patellar joints in 80 newborns from the 36th to 61st gestational week that underwent a routine hip sonography (Graf). All ultrasounds were performed by a pediatric radiologist with only minimal additional time to the routine hip ultrasound. In 30° flexion of the knee, axial, coronal, and sagittal reformats were used to standardize a reconstructed axial plane through the femoral condyle and the mid-patella. The sulcus angle, the lateral-to-medial facet ratio of the trochlea and the shape of the patella (Wiberg Classification) were evaluated. In all examinations reconstruction of the standardized axial plane was achieved, the mean trochlea angle was 149.1° (SD 4.9°), the lateral-to-medial facet ratio of the trochlea ratio was 1.3 (SD 0.22), and a Wiberg type I patella was found in 95% of the newborn. No statistical difference was detected between boys and girls. Using standardized reconstructions of the axial plane allows measurements to be made with lower operator dependency and higher accuracy in a short time. Therefore 3D ultrasound is an easy

  8. Hip Subluxation, first clinical manifestation in a boy with illness of Charcot Marie Tooth

    International Nuclear Information System (INIS)

    Marin Nino, Jorge Enrique; Rosselli Cock, Pablo

    2005-01-01

    Charcot-Marie-Tooth disease is a motor and sensitive neuropathy characterized by limb atrophy and weakness, cavus feet and in some cases acetabular dysplasia. We present a case of bilateral hip subluxation caused by this disease, which needed surgical correction

  9. Desnervação capsular percutânea ou aberta no tratamento da dor na displasia coxofemoral canina Percutaneous versus open hip denervation in the treatment of canine pain hip dysplasia

    Directory of Open Access Journals (Sweden)

    Gisele Francine da Silva

    2012-04-01

    Full Text Available A desnervação capsular (DC se baseia na desperiostização da borda acetabular e tem sido utilizada como uma das técnicas de alívio da dor articular em cães displásicos. Compararam-se as técnicas de DC percutânea e aberta, aplicadas em 25 cães, que apresentaram diagnóstico clínico e radiográfico de displasia coxofemoral, totalizando 31 cirurgias. Os efeitos das técnicas foram comparados, em período de 180 dias, por meio de avaliação subjetiva de claudicação e dor, perimetria da coxa, graus máximos de flexão e extensão da articulação coxofemoral e amplitude de movimento articular. Os graus de claudicação e dor apresentaram decréscimo significativo ao longo do período avaliado em ambos os grupos, sem apresentarem diferenças quando comparados entre si. Com relação à perimetria da coxa, flexão, extensão e amplitude de movimento articular, foi observado o aumento dos valores numéricos destas variáveis ao longo do estudo. Baseado nos achados, concluiu-se que a abordagem percutânea ou aberta na DC produz similitude nas variáveis avaliadas, e ambas trazem bons resultados.Joint capsule denervation is based on removal of sensitive fibers from the acetabular edge and has been used as one of the techniques to joint pain relief in the canine dysplastic hip. This study compared the techniques of percutaneous and open hip denervation in 25 dogs that showed clinical and radiographic signs of hip dysplasia, totaling 31 surgeries. The effects of the techniques were evaluated and compared in a period of 180 days following surgery, including subjective evaluation of lameness and pain, thigh girth, maximum degree of hip flexion and extension and range of motion (ROM. Degree of lameness and pain significantly decreased during the study period for both groups, and no difference could be observed between groups. Thigh girth, degree of flexion, extension and ROM presented an increase in the numerical values throughout the study

  10. Multiple epiphyseal dysplasia: radiographic abnormalities correlated with genotype

    Energy Technology Data Exchange (ETDEWEB)

    Unger, S.L. [Ahmanson Department of Pediatrics, Steven Spielberg Pediatric Research Center, Los Angeles, CA (United States); Briggs, M.D.; Holden, P. [Wellcome Trust Centre for Cell-Matrix Research, University of Manchester (United Kingdom); Zabel, B. [Children' s Hospital, Univ. of Mainz (Germany); Ala-Kokko, L.; Paassilta, P.; Lohiniva, J. [Dept. of Medical Biochemistry, Univ. of Oulu (Finland); Rimoin, D.L. [Ahmanson Department of Pediatrics, Steven Spielberg Pediatric Research Center, Los Angeles, CA (United States); Department of Pediatrics, UCLA School of Medicine, Los Angeles, CA (United States); Lachman, R.S. [Ahmanson Department of Pediatrics, Steven Spielberg Pediatric Research Center, Los Angeles, CA (United States); Department of Pediatrics, UCLA School of Medicine, Los Angeles, CA (United States); Department of Radiology, UCLA School of Medicine, Los Angeles, CA (United States); Cohn, D.H. [Ahmanson Department of Pediatrics, Steven Spielberg Pediatric Research Center, Los Angeles, CA (United States); Department of Human Genetics, UCLA School of Medicine, Los Angeles, CA (United States)

    2001-01-01

    Multiple epiphyseal dysplasia (MED) is an osteochondrodysplasia characterized clinically by mild short stature and early-onset degenerative joint disease and radiographically by epiphyseal hypoplasia/dysplasia. MED is genetically heterogeneous, with autosomal dominant cases resulting from mutations in at least three genes: the cartilage oligomeric matrix protein (COMP) gene (EDM1) and the COL9A2 (EDM2) and COL9A3 (EDM3) genes of type IX procollagen. We present here a comparison of the radiographic phenotypes of MED patients with type IX collagen gene mutations and those with COMP gene mutations. We reviewed radiographs from two patients with MED produced by COMP mutations, two families with COL9A2 mutations, and one family with a mutation in COL9A3. The data demonstrated that the patients with type IX collagen defects had more severe joint involvement at the knees and relative hip sparing, while the patients with COMP mutations had significant involvement at the capital femoral epiphyses and irregular acetabuli. This pattern of joint involvement was consistent regardless of overall degree of severity of the phenotype. (orig.)

  11. Hypohidrotic ectodermal dysplasia with anodontia: A rare case-rehabilitation by prosthetic management

    Directory of Open Access Journals (Sweden)

    M Naveen Kumar

    2012-01-01

    Full Text Available Ectodermal dysplasia is a hereditary disorder characterized by developmental dystrophies of ectodermal derivatives- It is characterized by triad of signs comprising sparse hair, abnormal or missing teeth and inability to sweat. This case of 12-year-old boy with hypohidrotic ectodermal dysplasia and complete anodontia of both primary and permanent dentition is presented. Owing to the need for treatment at an early age for anodontia and due to the ill-formed maxillary and mandibular residual ridges, the prosthetic management can be difficult. Complete dentures with soft liners and hollow maxillary denture were provided to encourage normal psychological development and to improve the function of the stomatognathic system.

  12. Pacman dysplasia: a lethal skeletal dysplasia with variable radiographic features

    Energy Technology Data Exchange (ETDEWEB)

    Miller, S.F. [Dept. of Radiology, Children' s Hospital of the King' s Daughters, Norfolk (United States); Proud, V.K. [Dept. of Genetics, Children' s Hospital of the King' s Daughters, Norfolk (United States); Werner, A.L. [Dept. of Pathology, Children' s Hospital of the King' s Daughters, Norfolk (United States); Field, F.M.; Wilcox, W.F.; Lachman, R.S.; Rimoin, D.L. [International Skeletal Dysplasia Registry, Cedars-Sinai Medical Center, Los Angeles (United States)

    2003-04-01

    Background: Punctate or stippled cartilaginous calcifications are associated with many conditions, including chromosomal, infectious, endocrine, and teratogenic etiologies. Some of these conditions are clinically mild, while others are lethal. Accurate diagnosis can prove instrumental in clinical management and in genetic counseling. Objective: To describe the diagnostic radiographic features seen in Pacman dysplasia, a distinct autosomal recessive, lethal skeletal dysplasia. Materials and methods: We present the fourth reported case of Pacman dysplasia and compare the findings seen in our patient with the three previously described patients. Results: Invariable and variable radiographic findings were seen in all four cases of histologically proven Pacman dysplasia. Conclusion: Pacman dysplasia presents both constant and variable diagnostic radiographic features. (orig.)

  13. Determinants of parental satisfaction with ultrasound hip screening in child health care.

    Science.gov (United States)

    Witting, Marjon; Boere-Boonekamp, Magda M; Fleuren, Margot A H; Sakkers, Ralph J B; Ijzerman, Maarten J

    2012-06-01

    Prior research has shown ultrasound (US) screening for developmental dysplasia of the hip (DDH) in preventive child health care to be more effective than the current screening method. In the present study, 3-month-old infants were screened for DDH with US. The objective of this study was to examine parental satisfaction with the screening and determinants that affect satisfaction. Parental satisfaction was measured using a questionnaire. Independent variables included socio-demographic determinants, structure, process and outcome-related determinants and the meeting of expectations. Satisfaction with the screening was high. Parents who perceived the screener as competent, had enough time to ask questions, perceived the proceeding as fluent, perceived a low burden on their infant and whose expectations were met, were more likely to be satisfied. Satisfaction was influenced by process-related factors and not by factors related to the structure and the outcome of the screening. Good information provision before the screening and communication during the screening are means by which parental satisfaction can be influenced positively.

  14. Developmental coxa vara associated with spondylometaphyseal dysplasia (DCV/SMD): ''SMD-Corner Fracture Type'' (DCV/SMD-CF) demonstrated in most reported cases

    Energy Technology Data Exchange (ETDEWEB)

    Currarino, G. [Texas Univ., Dallas, TX (United States). Dept. of Radiology; Department of Radiology, Texas Scottish Rite Hospital for Children, 2222 Welborn Street, Dallas, TX 75219 (United States); Birch, J.G.; Herring, J.A. [Department of Orthopedic Surgery, Texas Scottish Rite Hospital for Children, Dallas, TX (United States)

    2000-01-01

    Background. This paper reports three children with short stature: developmental coxa vara unilateral in the first case and bilateral in the other two; somewhat squared and ''ovoid'' vertebral bodies in the first patient, and normal to slightly tall vertebral bodies in the third; metaphyseal changes in some long tubular bones including bone fragments similar to the corner fractures seen in child abuse in all three patients. Materials and methods. The first and second patients were sisters; their mother, also quite short, had surgical procedures in early life for bilateral ''coxa vara''; their brother, also of short stature, had bilateral coxa valga with otherwise normal femoral heads and necks, and mild metaphyseal changes associated with two minute ''corner fractures'' in the proximal metaphysis of the left tibia. Results. A review of reported cases of developmental coxa vara associated with spondylometaphyseal dysplasia revealed that simulated corner fractures were present in most instances. (orig.)

  15. Cementless Hip Stem Anteversion in the Dysplastic Hip: A Comparison of Tapered Wedge vs Metaphyseal Filling.

    Science.gov (United States)

    Taniguchi, Naofumi; Jinno, Tetsuya; Koga, Daisuke; Hagino, Tetsuo; Okawa, Atsushi; Haro, Hirotaka

    2017-05-01

    Appropriate stem anteversion is important for achieving stability of the prosthetic joint in total hip arthroplasty. Anteversion of a cementless femoral stem is affected by the femoral canal morphology and varies according to stem geometry. We investigated the difference and variation of the increase in anteversion between 2 types of cementless stems, and the correlation between each stem and the preoperative femoral anteversion. We retrospectively compared 2 groups of hips that underwent total hip arthroplasty using a metaphyseal filling stem (78 hips) or a tapered wedge stem (83 hips). All the patients had osteoarthritis due to hip dysplasia. Computed tomography was used to measure preoperative femoral anteversion at 5 levels and postoperative stem anteversion. The increase in anteversion of the tapered wedge stem group (22.7° ± 11.6°) was more than that of the metaphyseal filling stem group (17.2° ± 8.3°; P = .0007). The variation of the increase in the tapered wedge stem group was significantly larger than that in the metaphyseal filling stem group (P = .0016). The metaphyseal filling stem group was more highly and positively correlated with femoral anteversion than the tapered wedge stem group. Femoral anteversion affects stem anteversion differently according to stem geometry. The tapered wedge stems had greater variation of the increase in anteversion than did the metaphyseal filling stems. Based on the results of this study, it is difficult to preoperatively estimate the increase in stem anteversion for tapered wedge stems. Copyright © 2016 Elsevier Inc. All rights reserved.

  16. Three-dimensional imaging of acetabular dysplasia: diagnostic value and impact on surgical type classification

    Energy Technology Data Exchange (ETDEWEB)

    Smet, Maria-Helena E-mail: marleen.smet@uz.kuleuven.ac.be; Marchal, Guy J.; Baert, Albert L.; Hoe, Lieven van; Cleynenbreugel, Johan van; Daniels, Hans; Molenaers, Guy; Moens, Pierre; Fabry, Guy

    2000-04-01

    Objective: To investigate the diagnostic value and the impact on surgical type classification of three-dimensional (3D) images for pre-surgical evaluation of dysplastic hips. Materials and methods: Three children with a different surgical type of hip dysplasia were investigated with helical computed tomography. For each patient, two-dimensional (2D) images, 3D, and a stereolithographic model of the dysplastic hip were generated. In two separate sessions, 40 medical observers independently analyzed the 2D images (session 1), the 2D and 3D images (session 2), and tried to identify the corresponding stereolithographic hip model. The influence of both image presentation (2D versus 3D images) and observer (degree of experience, radiologist versus orthopedic surgeon) were statistically analyzed. The SL model choice reflected the impact on surgical type classification. Results: Image presentation was a significant factor whereas the individual observer was not. Three-dimensional images scored significantly better than 2D images (P=0.0003). Three-dimensional imaging increased the correct surgical type classification by 35%. Conclusion: Three-dimensional images significantly improve the pre-surgical diagnostic assessment and surgical type classification of dysplastic hips.

  17. Three-dimensional imaging of acetabular dysplasia: diagnostic value and impact on surgical type classification

    International Nuclear Information System (INIS)

    Smet, Maria-Helena; Marchal, Guy J.; Baert, Albert L.; Hoe, Lieven van; Cleynenbreugel, Johan van; Daniels, Hans; Molenaers, Guy; Moens, Pierre; Fabry, Guy

    2000-01-01

    Objective: To investigate the diagnostic value and the impact on surgical type classification of three-dimensional (3D) images for pre-surgical evaluation of dysplastic hips. Materials and methods: Three children with a different surgical type of hip dysplasia were investigated with helical computed tomography. For each patient, two-dimensional (2D) images, 3D, and a stereolithographic model of the dysplastic hip were generated. In two separate sessions, 40 medical observers independently analyzed the 2D images (session 1), the 2D and 3D images (session 2), and tried to identify the corresponding stereolithographic hip model. The influence of both image presentation (2D versus 3D images) and observer (degree of experience, radiologist versus orthopedic surgeon) were statistically analyzed. The SL model choice reflected the impact on surgical type classification. Results: Image presentation was a significant factor whereas the individual observer was not. Three-dimensional images scored significantly better than 2D images (P=0.0003). Three-dimensional imaging increased the correct surgical type classification by 35%. Conclusion: Three-dimensional images significantly improve the pre-surgical diagnostic assessment and surgical type classification of dysplastic hips

  18. Arthrodiastasis for stiff hips in young patients

    OpenAIRE

    Cañadell, J.M. (J. M.); Gonzales, F. (F.); Barrios, R.H. (Raúl H.); Amillo, S. (Santiago)

    1993-01-01

    Joint distraction (arthrodiastasis) with a unilateral fixator was used to treat 9 patients with stiffness of the hip which had followed Perthes' disease (3), epiphysiolysis (2), congenital dysplasia (2), tuberculosis (1) and idiopathic chondrolysis (1). Their average age was 14 years, and they all had pain, limp and shortening of the leg. Distraction of 0.5 to 1 cm was maintained for an average of 94 days. The average range of movement subsequently was 65 degrees compared with 20 degrees befo...

  19. Recurrent Bacterial Meningitis in a Child with Mondini Dysplasia

    OpenAIRE

    Kepenekli-Kadayifci, Eda; Karaaslan, Ayşe; Atıcı, Serkan; Binnetoğlu, Adem; Sarı, Murat; Soysal, Ahmet; Altınkanat, Gülşen; Bakır, Mustafa

    2014-01-01

    Mondini dysplasia, also known as Mondini malformation, is a developmental abnormality of the inner and middle ears that can cause hearing loss, cerebrospinal fluid (CSF) leakage, and recurrent bacterial meningitis (RBM), which is defined as two or more episodes of meningitis separated by a period of convalescence and the complete resolution of all signs and symptoms. An accurate diagnosis of the underlying pathology is crucial to prevent further episodes from occurring. Herein, we present a...

  20. A new holder and surface MRI coil for the examination of the newborn infant hip

    Energy Technology Data Exchange (ETDEWEB)

    Krasny, R. (Dept. of Diagnostic Radiology, Univ. of Technology, Aachen (Germany)); Casser, H.R. (Dept. of Orthopedics, Univ. of Technology, Aachen (Germany)); Requardt, H. (Siemens AG, Erlangen (Germany)); Botschek, A. (Dept. of Diagnostic Radiology, Univ. of Technology, Aachen (Germany))

    1993-11-01

    A special holder was developed for examination of the infant hip joint using MRI. This holder allows the infant hip joint to be examined both in a neutral position and in various defined functional positions. A special integrated surface coil, also developed for this purpose, provides the high spatial resolution required for assessment of the fine joint structures. Thirty infants were examined and the new device has proved useful in advanced hip dysplasia, therapy-resistant subluxation and luxation, and for operative therapy planning (reconstruction of the acetabular roof, redirectional osteotomies). Interpretation errors due to misprojection can be eliminated to a large extent since the holder allows standardized and reproducible positioning. (orig.)

  1. Reliability of the Crowe und Hartofilakidis classifications used in the assessment of the adult dysplastic hip

    Energy Technology Data Exchange (ETDEWEB)

    Decking, Ralf; Brunner, Alexander; Puhl, Wolfhart [University of Ulm, Orthopaedic Department, RKU, Ulm (Germany); Decking, Jens [Johannes Gutenberg University School of Medicine, Department of Orthopaedic Surgery, Mainz (Germany); Guenther, Klaus-Peter [University of Ulm, Orthopaedic Department, RKU, Ulm (Germany); University-Hospital Carl Gustav Carus, Department of Orthopaedics, Dresden (Germany)

    2006-05-15

    To assess the inter-observer and intra-observer reliability of two commonly used radiographic classification systems in the evaluation of hip dysplasia in skeletally mature adults. Three observers with different levels of training independently classified 62 dysplastic hips on 51 standard anteriorposterior pelvis radiographs according to the criteria defined by Crowe and by Hartofilakidis. To assess intra-observer reliability, the same radiographs were reviewed 3 months later by the same observers. At the time of the radiographic examination, the mean age of the 51 patients had been 54 years (range 18-82 years). A high correlation concerning the inter- and intra-observer reliability of both systems was demonstrated. Inter-observer reliability displayed a weighted kappa coefficient of 0.82 for the Crowe and 0.75 for the Hartofilakidis classification. Intra-observer reliability showed a kappa coefficient of 0.86 and 0.79, respectively. Both classification systems can be recommended to compare collectives of adult patients with congenital dysplasia of the hip. However, for future clinical practice, it would be advisable to agree on one universally accepted system as a standard in the literature. (orig.)

  2. Reliability of the Crowe und Hartofilakidis classifications used in the assessment of the adult dysplastic hip

    International Nuclear Information System (INIS)

    Decking, Ralf; Brunner, Alexander; Puhl, Wolfhart; Decking, Jens; Guenther, Klaus-Peter

    2006-01-01

    To assess the inter-observer and intra-observer reliability of two commonly used radiographic classification systems in the evaluation of hip dysplasia in skeletally mature adults. Three observers with different levels of training independently classified 62 dysplastic hips on 51 standard anteriorposterior pelvis radiographs according to the criteria defined by Crowe and by Hartofilakidis. To assess intra-observer reliability, the same radiographs were reviewed 3 months later by the same observers. At the time of the radiographic examination, the mean age of the 51 patients had been 54 years (range 18-82 years). A high correlation concerning the inter- and intra-observer reliability of both systems was demonstrated. Inter-observer reliability displayed a weighted kappa coefficient of 0.82 for the Crowe and 0.75 for the Hartofilakidis classification. Intra-observer reliability showed a kappa coefficient of 0.86 and 0.79, respectively. Both classification systems can be recommended to compare collectives of adult patients with congenital dysplasia of the hip. However, for future clinical practice, it would be advisable to agree on one universally accepted system as a standard in the literature. (orig.)

  3. Concomitant Hip Arthroscopy and Periacetabular Osteotomy.

    Science.gov (United States)

    Domb, Benjamin G; LaReau, Justin M; Hammarstedt, Jon E; Gupta, Asheesh; Stake, Christine E; Redmond, John M

    2015-11-01

    To detail our early experience using concomitant hip arthroscopy and periacetabular osteotomy (PAO) for the treatment of acetabular dysplasia. We prospectively collected and retrospectively reviewed the surgical and outcome data of 17 patients who underwent concomitant hip arthroscopy and PAO between October 2010 and July 2013. Preoperative and postoperative range of motion, outcome and pain scores, and radiographic data were collected. Intraoperative arthroscopic findings and postoperative complications were recorded. The group consisted of 3 male and 14 female patients with a mean follow-up period of 2.4 years. Three patients had undergone previous surgery on the affected hip. Chondrolabral pathology was identified in all 17 patients. Twelve patients underwent labral repair, and five patients underwent partial labral debridement. No patient was converted to total hip arthroplasty or required revision surgery at short-term follow-up. All 4 patient-reported outcome scores showed statistically significant changes from baseline to latest follow-up (P arthroscopy and PAO has been favorable. We noted that all our patients have evidence of chondrolabral damage at the time of PAO when the joint is distracted and evaluated. All patients in this series had intra-articular pathology treated arthroscopically and showed satisfactory mean clinical improvement. Hip arthroscopy with PAO did not appear to introduce complications beyond the PAO alone. Level IV, therapeutic case series. Copyright © 2015 Arthroscopy Association of North America. Published by Elsevier Inc. All rights reserved.

  4. A new tilt on pelvic radiographs: a pilot study

    Energy Technology Data Exchange (ETDEWEB)

    Richards, P.J. [North Staffordshire Royal Infirmary, Department of Radiology, Stoke-on-Trent, Staffordshire (United Kingdom); Pattison, J.M. [University Hospital of North Staffordshire, Department of Radiology, Stoke on Trent (United Kingdom); Belcher, J. [Keele University, Department of Mathematics, Keele, Staffordshire (United Kingdom); DeCann, R.W. [IMECS, Department of Radiology, Market Drayton, Shropshire (United Kingdom); Anderson, Suzanne [University of Melbourne, Department of Radiology, Melbourne (Australia); Wynn-Jones, C. [University Hospital of North Staffordshire, Department of Orthopaedic Surgery, Stoke on Trent (United Kingdom)

    2009-02-15

    The aim of this study was to evaluate pelvic tilt on commonly performed measurements on radiography in primary protrusio acetabuli and developmental dysplasia of the hip. A dry assembled pelvis and spine skeleton was positioned in an isocentric skull unit and films exposed with increasing degrees of angulation of pelvic tilt. The films were then read by two independent readers for seven different measurements used to evaluate the hips and acetabular: acetabular line to ilioischial line, teardrop appearance, intercristal/intertuberous ratio, co-ordinates of femoral head, centre edge angle, acetabular depth/width ratio and acetabular angle. There was so much variation in the protrusio results that no formal recommendation of any standard radiographic test can be given. Only the inter tuberous distance is not effected by pelvic tilt. The acetabular angles for developmental dysplasia of the hip showed the most potential with pelvic tilt below 15 . As pelvic tilt increases, measurements used in protusio become unreliable, and computed tomography/magnetic resonance imaging are probably going to be more accurate as one can directly visualise pelvic intrusion. We recommend a lateral view to assess the degree of pelvic tilt in patients with protrusion to ensure these measurements are valid. (orig.)

  5. A Multi-centre Study to Assess the Long-term Performance of the Pinnacle™ Cup With a Polyethylene-on-metal Bearing in Primary Total Hip Replacement

    Science.gov (United States)

    2017-06-27

    Rheumatoid Arthritis; Osteoarthritis; Post-traumatic Arthritis; Collagen Disorders; Avascular Necrosis; Traumatic Femoral Fractures; Nonunion of Femoral Fractures; Congenital Hip Dysplasia; Slipped Capital Femoral Epiphysis

  6. Early treatment with the von Rosen splint for neonatal instability of the hip is safe regarding avascular necrosis of the femoral head

    Science.gov (United States)

    Wenger, Daniel; Samuelsson, Hanna; Düppe, Henrik; Tiderius, Carl Johan

    2016-01-01

    Background and purpose — Avascular necrosis of the femoral head (AVN) is a complication in treatment of developmental dysplasia of the hip (DDH). We evaluated the risk of AVN after early treatment in the von Rosen splint and measured the diameter of the ossific nucleus at 1 year of age. Children and methods — All children born in Malmö, Sweden, undergo clinical screening for neonatal instability of the hip (NIH). We reviewed 1-year radiographs of all children treated early for NIH in our department from 2003 through 2010. The diameter of the ossific nucleus was measured, and signs of AVN were classified according to Kalamchi-MacEwen. Subsequent radiographs, taken for any reason, were reviewed and a local registry of diagnoses was used to identify subsequent AVN. Results — 229 of 586 children referred because of suspected NIH received early treatment (age ≤ 1 week) for NIH during the study period. 2 of the 229 treated children (0.9%, 95% CI: 0.1–3.1) had grade-1 AVN. Both had spontaneous resolution and were asymptomatic during the observation time (6 and 8 years). 466 children met the inclusion criteria for measurement of the ossific nucleus. Neonatally dislocated hips had significantly smaller ossific nuclei than neonatally stable hips: mean 9.4 mm (95% CI: 9.1–9.8) vs. 11.1 mm (95% CI: 10.9–11.3) at 1 year (p < 0.001). Interpretation — Early treatment with the von Rosen splint for NIH is safe regarding AVN. The ossification of the femoral head is slower in children with NIH than in untreated children with neonatally stable hips. PMID:26730503

  7. A study of the clinical and radiological features in a cohort of 93 patients with a COL2A1 mutation causing spondyloepiphyseal dysplasia congenita or a related phenotype

    DEFF Research Database (Denmark)

    Terhal, Paulien A; Nievelstein, Rutger Jan A J; Verver, Eva J J

    2015-01-01

    -like dysplasia (n = 2). The remaining 13 patients had normal stature with mild SED, Stickler-like syndrome or multiple epiphyseal dysplasia. Over 50% of the patients had undergone orthopedic surgery, usually for scoliosis, femoral osteotomy or hip replacement. Odontoid hypoplasia was present in 56% (95% CI 38......Type 2 collagen disorders encompass a diverse group of skeletal dysplasias that are commonly associated with orthopedic, ocular, and hearing problems. However, the frequency of many clinical features has never been determined. We retrospectively investigated the clinical, radiological......, and genotypic data in a group of 93 patients with molecularly confirmed SEDC or a related disorder. The majority of the patients (80/93) had short stature, with radiological features of SEDC (n = 64), others having SEMD (n = 5), Kniest dysplasia (n = 7), spondyloperipheral dysplasia (n = 2), or Torrance...

  8. Anesthesia for a patient with Fanconi anemia for developmental dislocation of the hip: a case report

    Directory of Open Access Journals (Sweden)

    Zafer Dogan

    2014-06-01

    Full Text Available Fanconi anemia is a rare autosomal recessive inherited bone marrow failure syndrome with congenital and hematological abnormalities. Literature regarding the anesthetic management in these patients is limited. A management of a developmental dislocation of the hip was described in a patient with fanconi anemia. Because of the heterogeneous nature, a patient with fanconi anemia should be established thorough preoperative evaluation in order to diagnose on clinical features. In conclusion, we preferred caudal anesthesia in this patient with fanconi anemia without thrombocytopenia, because of avoiding from N2O, reducing amount of anesthetic, existing microcephaly, hypothyroidism and elevated liver enzymes, providing postoperative analgesia, and reducing amount of analgesic used postoperatively.

  9. Treatment of a femoral shaft fracture in a patient with congenital hip disease: a case report

    Directory of Open Access Journals (Sweden)

    Koutsostathis Stefanos D

    2010-07-01

    Full Text Available Abstract Introduction We present a rare case of two concomitant morbidities treated in one operation. To our knowledge, this is the first report of its kind in the literature. Case presentation A 57-year-old Greek woman was admitted to the emergency department having sustained a spiral mid-shaft femoral fracture. She also suffered from an ipsilateral hip congenital dysplasia with ankylosed hip joint due to severe arthritis. She was treated with a total hip arthroplasty using a long stem performing as an intramedullary nail. Conclusion We undertook a complex operative treatment of both co-morbidities in a one stage procedure with a satisfactory clinical result.

  10. Lessons learned from study of congenital hip disease in adults.

    Science.gov (United States)

    Hartofilakidis, George; Lampropoulou-Adamidou, Kalliopi

    2016-12-18

    Orthopaedic surgeons specialising in adult hip reconstruction surgery often face the problem of osteoarthritis secondary to congenital hip disease (CHD). To achieve better communication among physicians, better treatment planning and evaluation of the results of various treatment options, an agreed terminology is needed to describe the entire pathology. Furthermore, a generally accepted classification of the deformities is necessary. Herein, the authors propose the use of the term "congenital hip disease" and its classification as dysplasia, low dislocation and high dislocation. Knowledge of the CHD natural history facilitates comprehension of the potential development and progression of the disease, which differs among the aforementioned types. This can lead to better understanding of the anatomical abnormalities found in the different CHD types and thus facilitate preoperative planning and choice of the most appropriate management for adult patients. The basic principles for improved results of total hip replacement in patients with CHD, especially those with low and high dislocation, are: Wide exposure, restoration of the normal centre of rotation and the use of special techniques and implants for the reconstruction of the acetabulum and femur. Application of these principles during total hip replacement in young female patients born with severe deformities of the hip joint has led to radical improvement of their quality of life.

  11. Asphyxiating thoracic dysplasia

    International Nuclear Information System (INIS)

    Franzcr, J.; Kozlowski, K.

    2008-01-01

    Asphyxiating Thoracic Dysplasia is the most frequent form of Small Thorax - Short Rib Syndromes. Asphyxiating Thoracic Dysplasia in two patients with different clinical course is reported. Radiographic examination is the only method to diagnose Asphyxiating Thoracic Dysplasia with certainty. The correct diagnosis is important for prognostication and genetic counseling. It also excludes the necessity of further, often expensive investigations. (author)

  12. Phenotypic and genetic evaluation of elbow dysplasia in Dutch Labrador Retrievers, Golden Retrievers, and Bernese Mountain Dogs

    NARCIS (Netherlands)

    Lavrijsen, I.C.M.; Heuven, H.C.M.; Voorhout, G.; Meij, B.P.; Theyse, L.F.H.; Leegwater, P.A.J.; Hazewinkel, H.A.W.

    2012-01-01

    Canine elbow dysplasia encompasses four developmental diseases: ununited anconeal process, osteochondrosis of the medial part of the humeral condyle, fragmented medial coronoid process (FCP), and incongruity of the elbow joint. Four radiographic views per joint were used to evaluate 2693 Labrador

  13. Dose reduction of radiographs of the pediatric pelvis for diagnosing hip dysplasia using a digital flat-panel detector system; Dosisreduktion bei Roentgenaufnahmen des kindlichen Beckenskelettes zur Diagnostik der Hueftgelenksdysplasie unter Verwendung eines digitalen Flachdetektorsystems

    Energy Technology Data Exchange (ETDEWEB)

    Ludwig, K.; Ahlers, K.; Kloska, S.; Vieth, V.; Meier, N.; Heindel, W. [Inst. fuer Klinische Radiologie, Westfaelische Wilhelms-Univ. Muenster (Germany); Sandmann, C.; Gosheger, G. [Orthopaedische Klinik, Westfaelische Wilhelms-Univ. Muenster (Germany)

    2003-01-01

    Purpose: To evaluate a possible dose reduction in pediatric pelvic radiographs in congenital hip dysplasia using a digital flat-panel system instead of a phosphor-storage system. Materials and Methods: During a six-month period, all pediatric patients referred for pelvic radiography for the evaluation of congenital hip dysplasia were randomely assigned to be examined by either a phosphor-storage system or a digital flat-panel system, whereby the latter system was operated with half the radiation dose. Thirty pairs of radiographs were assessed for the visibility of 16 anatomic details and for 5 orthopedic-radiographic measurements (5-point scale with 1 = excellent; three independent observers). The projection indices of Ball and Kommenda and of Toennis and Brunken were calculated for all radiographs. The Student's t-test was used to compare the flat-panel and the phosphor-storage radiographs for observers' assessments, patients' age and projection indices. Results: In a total of 7560 observations, the scores for the visibility of anatomic details and orthopedic-radiographic measurements were respectively 2.72 and 2.64 for the flat-panel system and 2.93 and 2.79 for the phosphor-storage system. No significant differences were found between both systems (p > 0.05) and between patient age and projection indices (p > 0.05). Conclusion: Pediatric pelvic radiographs can be obtained with a digital flat-panel system using half the radiation dose instead of a phosphor-storage system without sacrificing relevant information in the diagnosis of congenital hip dysplasia. (orig.) [German] Zielsetzung: Evaluation einer moeglichen Dosisreduktion bei kindlichen Beckenroentgenaufnahmen zur Diagnostik der Hueftgelenksdysplasie mit einem digitalen Flachdetektorsystem im Vergleich zu einem digitalen Speicherfoliensystem. Material und Methoden: Prospektiv wurden alle ueber einen Zeitraum von 6 Monaten zur Roentgenaufnahme des Beckenskelettes im Rahmen der Diagnostik der

  14. p53 expression and mutation analysis of odontogenic cysts with and without dysplasia.

    Science.gov (United States)

    Cox, Darren P

    2012-01-01

    Overexpression of p53 protein is well described in odontogenic cystic lesions (OCLs), including those with epithelial dysplasia; however, most p53 antibodies stain both wild-type and mutated p53 protein and may not reflect genotype. Direct sequencing of the p53 gene has not identified mutations in OCLs with dysplasia. The purpose of this study was to determine the molecular basis of p53 expression in several types of OCLs with and without dysplasia. The study material comprised 13 OCLs: odontogenic keratocyst (n = 5), orthokeratinized odontogenic cyst (n = 5), dentigerous cyst (n = 2), lateral periodontal cyst (n = 1), and unspecified developmental odontogenic cyst (UDOC) (n = 1). Five of these had features of mild or moderate epithelial dysplasia. One intraosseous squamous cell carcinoma (SCC) that was believed to have arisen from an antecedent dysplastic orthokeratinized OC was also included. Immunohistochemistry was performed using the DO7 monoclonal antibody that recognizes wild-type and mutated p53. DNA was extracted from microdissected tissue for all samples and exons 4 to 8 of the p53 gene direct sequenced. In 4 of 5 OCLs with dysplasia there was strong nuclear staining of basal and suprabasal cells. In all cases without dysplasia, nuclear expression in basal cells was either negative or weak and was absent in suprabasal cell nuclei. A mutation in exon 6 of the p53 gene (E224D) was identified in both the dysplastic orthokeratinized OC and the subsequent intraosseous SCC. OCLs with features of dysplasia show increased expression of p53 protein that does not reflect p53 mutational status. One dysplastic OC shared the same p53 mutation with a subsequent intraosseous SCC, indicating that p53 mutation may be associated with malignant transformation in this case. Copyright © 2012 Elsevier Inc. All rights reserved.

  15. Cloverleaf skull with generalised bone dysplasia

    International Nuclear Information System (INIS)

    Kozlowski, K.; Warren, P.S.; Fisher, C.C.; Royal Hospital for Women, Camperdown

    1985-01-01

    A case of cloverleaf skull with generalised bone dysplasia is reported. The authors believe that bone dysplasia associated with cloverleaf is neither identical with thanatophoric dysplasia nor achondroplasia. Until identity of thanatophoric dysplasia and cloverleaf skull with generalised bone dysplasia is proved the diseases should be looked upon as separate entities and the wording ''thanatophoric dysplasia with cloverleaf skull'' should be abolished. (orig.)

  16. Ectodermal dysplasia-skin fragility syndrome

    Directory of Open Access Journals (Sweden)

    Vijay S Adhe

    2011-01-01

    Full Text Available Ectodermal dysplasia-skin fragility (EDSF syndrome is a rare and first described inherited disorder of desmosomes. It occurs due to loss-of-function mutations in PKP1 gene resulting in poorly formed desmosomes and loss of desmosomal and epidermal integrity. We report a case of a 2-year-old Indian male child who presented with palmoplantar hyperkeratosis with fissuring, short, sparse, and easily pluckable scalp hair, nail dystrophy, and multiple erosions over the skin. Skin biopsy showed epidermal hyperplasia with widening of intercellular spaces. His developmental milestones were delayed but intelligence was normal. Echocardiography, X-ray chest, and electrocardiogram were normal. Very few cases of this syndrome have been reported in the literature. We consider this as the first case report from India.

  17. The impairment of MAGMAS function in human is responsible for a severe skeletal dysplasia.

    Directory of Open Access Journals (Sweden)

    Cybel Mehawej

    2014-05-01

    Full Text Available Impairment of the tightly regulated ossification process leads to a wide range of skeletal dysplasias and deciphering their molecular bases has contributed to the understanding of this complex process. Here, we report a homozygous mutation in the mitochondria-associated granulocyte macrophage colony stimulating factor-signaling gene (MAGMAS in a novel and severe spondylodysplastic dysplasia. MAGMAS, also referred to as PAM16 (presequence translocase-associated motor 16, is a mitochondria-associated protein involved in preprotein translocation into the matrix. We show that MAGMAS is specifically expressed in trabecular bone and cartilage at early developmental stages and that the mutation leads to an instability of the protein. We further demonstrate that the mutation described here confers to yeast strains a temperature-sensitive phenotype, impairs the import of mitochondrial matrix pre-proteins and induces cell death. The finding of deleterious MAGMAS mutations in an early lethal skeletal dysplasia supports a key role for this mitochondrial protein in the ossification process.

  18. Cloverleaf skull with generalised bone dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.; Warren, P.S.; Fisher, C.C.

    1985-09-01

    A case of cloverleaf skull with generalised bone dysplasia is reported. The authors believe that bone dysplasia associated with cloverleaf is neither identical with thanatophoric dysplasia nor achondroplasia. Until identity of thanatophoric dysplasia and cloverleaf skull with generalised bone dysplasia is proved the diseases should be looked upon as separate entities and the wording ''thanatophoric dysplasia with cloverleaf skull'' should be abolished.

  19. Outcome of periacetabular osteotomy for the management of acetabular dysplasia: experience in an academic centre.

    LENUS (Irish Health Repository)

    Burke, Neil G

    2011-02-01

    Periacetabular osteotomy (PAO) is a very effective reconstructive procedure for treatment of acetabular dysplasia. An orthopaedic paediatric surgeon and a reconstructive hip arthroplasty surgeon performed this procedure together in the early phase of their learning curve and then performed it individually. The early clinical and radiographic results of 85 consecutive PAOs performed in this academic orthopaedic unit were reviewed. The mean Merle-d\\'Aubigné score increased from 12.4 preoperatively to 16 at follow-up. Pre-operatively 73 hips were anteverted and 12 were neutral or retroverted. The mean angle of Wiberg improved from 5 degrees to 21 degrees (p < 0.0001) in anteverted hips, and from 9 degrees to 30 degrees in neutral or retroverted hips. The mean angle of Lequesne and de Sèze improved from 6 degrees to 35 degrees (p < 0.0001) in anteverted hips, and in neutral or retroverted hips from 9 degrees to 30 degrees (p < 0.0001). The acetabular index improved from 26 degrees to 8 degrees (p < 0.0001) in anteverted hips, and from 21 degrees to 7 degrees (p < 0.0001) in neutral or retroverted hips. Over the 7 year period the blood loss and operative time improved from 2000 ml to 900 ml and 4 hours to 2 hours respectively. Four hips (four patients) required conversion to total hip replacement. The radiographic correction and improved clinical scores are similar to those in previous studies. This study shows a survival rate of 94% at 58 months following periacetabular osteotomy. The learning curve and the early results of this procedure performed in our academic unit are encouraging.

  20. Spondyloepiphyseal dysplasia congenita

    International Nuclear Information System (INIS)

    Macpherson, R.I.; Wood, B.P.

    1980-01-01

    Spondyloepiphyseal dysplasia congenita is a form of primarily short trunk dwarfism, that is manifest at birth but generally has not been regarded as a cause of lethal neonatal dwarfism. Seven neonates with severe dwarfism are presented. The first survived the newborn period, but the other six were early neonatal deaths. All displayed the clinical and radiologic features of spondyloepiphyseal dysplasia congenita. The striking similarities between spondyloepiphyseal dysplasia congenita and achondrogenesis type 2 are discussed. (orig.) [de

  1. Twin infant with lymphatic dysplasia diagnosed with Noonan syndrome by molecular genetic testing.

    Science.gov (United States)

    Mathur, Deepan; Somashekar, Santhosh; Navarrete, Cristina; Rodriguez, Maria M

    2014-08-01

    Noonan Syndrome is an autosomal dominant disorder characterized by short stature, congenital heart defects, developmental delay, dysmorphic facial features and occasional lymphatic dysplasias. The features of Noonan Syndrome change with age and have variable expression. The diagnosis has historically been based on clinical grounds. We describe a child that was born with congenital refractory chylothorax and subcutaneous edema suspected to be secondary to pulmonary lymphangiectasis. The infant died of respiratory failure and anasarca at 80 days. The autopsy confirmed lymphatic dysplasia in lungs and mesentery. The baby had no dysmorphic facial features and was diagnosed postmortem with Noonan syndrome by genomic DNA sequence analysis as he had a heterozygous mutation for G503R in the PTPN11 gene.

  2. Familial ectodermal dysplasia: a peers' agony.

    Science.gov (United States)

    Hegde, Karthik; Kashyap, Roopashri Rajesh; Nair, Gopakumar; Nair, Preeti P

    2013-07-23

    Ectodermal dysplasias include a various group of inherited disorders which share primary defect in the development of two or more tissues of embryonic ectodermal origin. Though there are many subtypes, ectodermal dysplasias are mainly hidrotic ectodermal dysplasia and hypohidrotic ectodermal dysplasia, among which the most common variety is X linked hypohidrotic ectodermal dysplasia. We report a rare case of X linked hypohidrotic ectodermal dysplasia occurring in a family with various skin, hair and oral abnormalities.

  3. Radiographic Evidence of Hip Microinstability in Elite Ballet.

    Science.gov (United States)

    Mitchell, Ronald J; Gerrie, Brayden J; McCulloch, Patrick C; Murphy, Andrew J; Varner, Kevin E; Lintner, David M; Harris, Joshua D

    2016-06-01

    To determine prevalence, magnitude, and predisposing radiographic features of hip subluxation in elite ballet dancers. A cross-sectional investigation of professional male and female ballet dancers was performed using 5 plain radiographs. A "splits" anteroposterior (AP) radiograph was performed with legs abducted parallel to the trunk in the coronal plane (splits position; grand écart facial). Hip center position (HCP) was measured on standing AP pelvis and AP pelvis splits views and the difference calculated (subluxation distance) to determine prevalence and magnitude of femoral head subluxation. Student t test compared HCP on AP pelvis and splits radiographs. Pearson correlations were used to correlate splits HCP with radiographic measures of femoroacetabular impingement and dysplasia. Analyzing 47 dancers (21 men, 26 women; 23.8 ± 5.4 years), mean HCP on standing AP pelvis was 9.39 ± 3.33 mm versus 10.8 ± 2.92 mm on splits radiograph, with mean subluxation distance of 1.41 mm (P = .035). Forty-two dancers' femoral heads translated laterally with splits positioning, and 17 dancers (36%) exhibited a "vacuum sign" (bilateral in 71% of subjects with at least 1 hip vacuum sign). There was strong positive correlation (r = 0.461, P = .001) with splits HCP and alpha angle (Dunn 45°), and moderate negative correlation (r = -0.332, P = .022) with subluxation distance and neck-shaft angle. In men, splits HCP increased as lateral center edge angle (CEA) decreased (r = -0.437, P = .047), as anterior CEA decreased (r = -0.482, P = .027), as Tönnis angle increased (r = 0.656, P = .001), and as femoral head extrusion index increased (r = 0.511, P = .018). In women, there was moderate negative correlation (r = -0.389, P = .049) with subluxation distance and neck-shaft angle. Hip subluxation occurs during splits in most professional ballet dancers, with a significantly greater magnitude of subluxation in women than men. Subluxation magnitude

  4. The role of imaging in early hip OA.

    Science.gov (United States)

    Siebelt, M; Agricola, R; Weinans, H; Kim, Y J

    2014-10-01

    Hip osteoarthritis (OA) is characterized by cartilage degradation, subchondral bone sclerosis and osteophyte formation. Nowadays, OA is thought to develop via different etiologies that all lead to a similar form of end stage joint degradation. One of these subtypes is related to an abnormal shaped hip joint, like acetabular dysplasia and a cam deformity. These bony abnormalities are highly predictive for development of hip OA, but they are likely to already be present from childhood. This suggests that these deformations induce OA changes in the hip, well before extensive hip degradation becomes present three to four decades later. Accurate detection and successful characterization of these early OA events might lead to better treatment options for hip OA besides nowadays available invasive joint replacement surgery. However, current diagnostic imaging techniques like radiographs or plain magnetic resonance imaging (MRI), are not sensitive enough to detect these subtle early OA changes. Nor are they able to disentangle intertwined and overlapping cascades from different OA subtypes, and neither can they predict OA progression. New and more sensitive imaging techniques might enable us to detect first OA changes on a cellular level, providing us with new opportunities for early intervention. In this respect, shape analysis using radiography, MRI, computed tomography (CT), single photon emission computed tomography (SPECT)/CT, and positron emission tomography (PET) might prove promising techniques and be more suited to detect early pathological changes in the hip joint. A broad application of these techniques might give us more understanding what can be considered physiological adaptation of the hip, or when early OA really starts. With a more clear definition of early OA, more homogenous patient populations can be selected and help with the development of new disease modifying OA interventions. Copyright © 2014 Osteoarthritis Research Society International

  5. Modern radiological imaging of osteoarthritis of the hip joint with consideration of predisposing conditions

    International Nuclear Information System (INIS)

    Weber, Marc-Andre; Rehnitz, C.; Merle, C.; Gotterbarm, T.

    2016-01-01

    Osteoarthritis is the most common disease of the hip joint in adults and has a high socioeconomic impact. This review article discusses the value of three imaging modalities in the diagnosis of osteoarthritis of the hip joint: projection radiography, computed tomography, and magnetic resonance imaging (MRI). Besides established imaging diagnostics of osteoarthritis, this review also outlines new MRI techniques that enable the biochemical analysis of hip joint cartilage and discusses predisposing deformities of the hip joint including femoroacetabular impingement (FAI) with labral pathologies, hip joint dysplasia, malrotation, and, finally, femoral head necrosis, for which early detection and an exact description of the extent and localization of the necrotic area are extremely important. Conventional X-rays remain indispensable for the diagnosis of osteoarthritis, while MRI is able to depict additional early symptoms and signs of activity of the disease. With the increasing number of joint-preserving interventions such as surgical hip luxation and hip joint arthroscopy for treating FAI, high-resolution imaging is gaining further importance for both pre- and postoperative diagnostics because it can accurately recognize early stages of joint damage. With high-resolution MR sequences and MR arthrography, the detailed depiction of the thin cartilaginous coating of the hip joint has become quite possible.

  6. [Characteristics in treatment of the hip in patients with Down syndrome].

    Science.gov (United States)

    Peterlein, C-D; Schiel, M; Timmesfeld, N; Schofer, M D; Eberhardt, O; Wirth, T; Fernandez, F F

    2013-12-01

    The treatment of hip instability in patients with Down syndrome is challenging. We have performed different pelvic osteotomies and corrections at the proximal femur for this indication. This retrospective study was conducted to evaluate the clinical and radiological outcome of each intervention. All in all, 166 patients with Down syndrome were treated at our orthopaedic department in the observation period. Problems related to the hip joint were diagnosed in 63 of those patients. Only patients who underwent surgery were included in this study. The charts and X-rays of these 31 patients were evaluated with respect to the following parameters: incidence of the hip problem, concomitant diseases, temporal progress, kind of operation method and date, duration of stay in the hospital, after-care, follow-on surgery related to complications, AC angle, CE angle, ACM angle, CCD angle, index of migration according to Reimers, classification of Bauer and Kerschbauer and general morphology of the femoral head. The group was compared with an age-matched group of 21 patients with hip dysplasia. Those patients underwent the same sort of operation in the same year. In the Morbus Down group, we performed surgery for preservation of the hip in 49 cases. This included 13 osteotomies according to Chiari, 11 triple osteotomies according to Tönnis, 10 corrections by femoral varus derotation osteotomy, 8 pelvic osteotomies according to Pemberton, 5 pelvic osteotomies according to Salter and 2 open reductions of the hip. With respect to the moment of surgery, we detected three peaks of age. There was no difference in course of disease and quantity of complications between the groups. Satisfactory results concerning clinical and radiological outcome were achieved predominantly by complete redirectional acetabular osteotomies. Half of the patients who were solely treated by femoral varus derotation osteotomy needed follow-on surgery in the form of pelvic osteotomy. Comparison of

  7. Retrospective analysis for genetic improvement of hip joints of cohort labrador retrievers in the United States: 1970-2007.

    Directory of Open Access Journals (Sweden)

    Yali Hou

    2010-02-01

    Full Text Available Canine Hip Dysplasia (CHD is a common inherited disease that affects dog wellbeing and causes a heavy financial and emotional burden to dog owners and breeders due to secondary hip osteoarthritis. The Orthopedic Foundation for Animals (OFA initiated a program in the 1960's to radiograph hip and elbow joints and release the OFA scores to the public for breeding dogs against CHD. Over last four decades, more than one million radiographic scores have been released.The pedigrees in the OFA database consisted of 258,851 Labrador retrievers, the major breed scored by the OFA (25% of total records. Of these, 154,352 dogs had an OFA hip score reported between 1970 and 2007. The rest of the dogs (104,499 were the ancestors of the 154,352 dogs to link the pedigree relationships. The OFA hip score is based on a 7-point scale with the best ranked as 1 (excellent and the worst hip dysplasia as 7. A mixed linear model was used to estimate the effects of age, sex, and test year period and to predict the breeding value for each dog. Additive genetic and residual variances were estimated using the average information restricted maximum likelihood procedure. The analysis also provided an inbreeding coefficient for each dog. The hip scores averaged 1.93 (+/-SD = 0.59 and the heritability was 0.21. A steady genetic improvement has accrued over the four decades. The breeding values decreased (improved linearly. By the end of 2005, the total genetic improvement was 0.1 units, which is equivalent to 17% of the total phenotypic standard deviation.A steady genetic improvement has been achieved through the selection based on the raw phenotype released by the OFA. As the heritability of the hip score was on the low end (0.21 of reported ranges, we propose that selection based on breeding values will result in more rapid genetic improvement than breeding based on phenotypic selection alone.

  8. Genetics Home Reference: boomerang dysplasia

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Boomerang dysplasia Boomerang dysplasia Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Boomerang dysplasia is a disorder that affects the development ...

  9. Diagnostic and prognostic values of standard and dynamic ultrasound in early detection and treatment of developmental hip deformity

    Directory of Open Access Journals (Sweden)

    Pajić Miloš

    2007-01-01

    Full Text Available Introduction A clinical examination of a newborn infant is indispensable, but certainly insufficient for a diagnosis of developmental deformity of the hip (DDH to be made. The use of the ultrasound in the diagnostics of DDH, beside the visualization and making the respective clinical findings objective, made possible verification (still without a distinction of the tissues of the two basic categories of the primary condition of a newborn baby hips. Objective The purpose of this study was to find out the optimal methodological procedure for the early detection of DDH in newborn infants and suckling. Method During 2002 and 2003, at the Neonatal Department of the Clinic for Gynecology and Obstetrics, Novi Sad, as well as at the Institute for Orthopedic Surgery "Banjica" Belgrade, there were 4016 newborn infants examined, i.e. 8032 hips clinically and by ultrasound. The standard Graf’s methodological procedure was applied completely, supplemented by the dynamic examination by pushing back and stretching femora along, that is by the techniques of Couture and Harcke. Results In order to categorize the condition of the hip of a newborn infant, the sonographic classification of R. Graaf, Th. Harcke and D. Pajić was used. The ultrasound analysis demonstrated a frequency of the sonotype Ia in 552 (13.08% of the newborn infants, the sonotype Ib in 2934 (73.00%, the sonotype IIa+ in 481 (11.97% and the pathological cases with sonotypes IIg 42 (1.04%, IId 17 (0.42%, IIIa 15 (0.37% and IV 5 (0.12%. The total number of unstable critical, discentering and discentered cases of DDH was 79 (1.95%. The incidence of DDH was three times more frequent in girls, mostly bilateral; when unilateral, it was more frequent in the left one. The results of the early treatment were uniformly excellent, but in two cases there was established osteochondritis of the first degree (Pavlik’s harness 1, Von Rosen’s splint 1. Conclusion It has been proven that the clinical

  10. Early childhood anomalies of the hip occur at a similar frequency in patients with idiopathic scoliosis and in healthy individuals – questionnaire for parents

    Directory of Open Access Journals (Sweden)

    Stępień Agnieszka

    2017-12-01

    Full Text Available Introduction: Improper hip joint development may lead to numerous unfavourable changes in the musculoskeletal system. The aim of this research was to determine how often adolescents with idiopathic scoliosis and their healthy counterparts experienced anomalies of the hip in their infancy period and to examine the correlation between the occurrence of hip anomalies and idiopathic scoliosis. Material and methods: The research was conducted in medical centres as well as in schools. The parents of adolescents with idiopathic scoliosis and parents of healthy adolescents completed a questionnaire on the basis of their child’s development history included in the medical records book and other medical documentation. Results: 533 questionnaires were taken into consideration, included 145 questionnaires completed by parents of adolescents with scoliosis (121 girls - 13.8 years, SD 1.9; 24 boys - 12.9 years, SD 2.5 and 388 questionnaires from the group of adolescents without scoliosis (194 girls - 13.5 years, SD 2.0; 194 boys - 13.4 years, SD 2.1. No significant differences were noted in the incidence of hip anomalies between the groups of girls and boys with and without scoliosis, no correlations between anomalies of the hip and scoliosis were found (girls χ2=0.840; Cramer V=0.052; p=0.36; boys χ2=1.205; Cramer V=0.074; p=0.27. Conclusions: Hip anomalies such as hip dysplasia, movement asymmetry or range of motion limitations diagnosed in the infancy period did not correlate with idiopathic scoliosis. Further research aimed at a separate analysis of the influence of hip dysplasia on the occurrence of idiopathic scoliosis should be carried out.

  11. Penatalaksanaan Oligodontia pada Anak 14 Tahun dengan Sindrom Ectodermal Dysplasia: Laporan Kasus

    Directory of Open Access Journals (Sweden)

    Anie Apriani

    2015-12-01

    Full Text Available Ectodermal dysplasia is a hereditary disorder that is rarely seen with approximately 7000 cases in the world. In this disorder, Ectodermal dysplasia occurs as a developmental disorder of organ structures derived from the ectodermal layer such as hair, teeth, nails, and sweat glands. This is a case report of ectodermal dysplasia in a 14-year-old girl with clinical manifestations of oligodontia (tooth loss, more than 6 teeth on the upper and lower jaw, thin and curly hair, abnormal toenail shape, and impaired excretion of sweat. The purpose of the treatment was to make the patient regains her esthetic and masticatory functions, which were disrupted by the absence of permanent tooth buds, as well as to maintain the normal growth of the developing jaws. Treatment for the patient’s with oligodontia included the use of removable denture in lower jaw and application of acrylic crowns on all upper and lower anterior teeth resulting in improved esthetics and masticatory function. It is concluded that the treatment has been able to improve the esthetics and to return the masticatory function of the patient while maintaining the normal growth of the jaws.

  12. Progressive pseudorheumatoid dysplasia misdiagnosed as ...

    African Journals Online (AJOL)

    Progressive pseudorheumatoid dysplasia misdiagnosed as seronegative juvenile idiopathic arthritis. Ozgur Taspinar, Fatih Kelesoglu, Yasar Keskin, Murat Uludag. Abstract. Background: Progressive pseudorheumatoid dysplasia (PPD) is a rare spondylo- epi-metaphyseal dysplasia (SEMD). It can be confused with juvenile ...

  13. Prenatal diagnosis of boomerang dysplasia.

    Science.gov (United States)

    Wessels, Marja W; Den Hollander, Nicolette S; De Krijger, Ronald R; Bonifé, Luisa; Superti-Furga, Andrea; Nikkels, Peter G; Willems, Patrick J

    2003-10-01

    Boomerang dysplasia, atelosteogenesis type 1 and Piepkorn dysplasia are bone dysplasias with an overlapping clinical spectrum characterized by deficient formation and ossification of specific elements of the skeleton. Typical symptoms include micromelia with diminished ossification, and a characteristic bowed and boomerang-like aspect of the long tubular bones. We report here a new case of boomerang dysplasia, which was detected prenatally in the 16th week of gestation by ultrasound. Copyright 2003 Wiley-Liss, Inc.

  14. Arthroscopic decompression and notchplasty for long-standing anterior cruciate ligament impingement in a patient with multiple epiphyseal dysplasia: a case report

    Directory of Open Access Journals (Sweden)

    Trehan RK

    2008-05-01

    Full Text Available Abstract Introduction Multiple epiphyseal dysplasia is a genetically and clinically heterogeneous osteochondroplasia with symmetrical involvement. It is characterized by joint pain in childhood and early adulthood with early onset of osteoarthritis, mainly affecting the hips. Case presentation We report the case of a 20-year-old man of Asian origin with multiple epiphyseal dysplasia presenting with bilateral knee pain, stiffness and instability found to be caused by bilateral anterior cruciate ligament impingement on abnormal medial femoral condyles. Bilateral staged arthroscopic notchplasty was performed successfully, resulting in subjective relief of pain, and improved range of movement and stability. Conclusion Care should be taken not to exclude a diagnosis of multiple epiphyseal dysplasia when few of the characteristic radiographic features are evident but clinical suspicion is high. This case highlights the scope for subjective symptomatic improvement following a minimum of surgical intervention. We recommend limiting early intervention to managing symptomatic features rather than radiographic abnormalities alone.

  15. Acetabular retroversion as a rare cause of chronic hip pain: recognition of the ''figure-eight'' sign

    Energy Technology Data Exchange (ETDEWEB)

    Banks, Kevin P. [Brooke Army Medical Center, Department of Radiology, Fort Sam Houston, TX (United States); Grayson, David E. [Wilford Hall Medical Center, Department of Radiology, Lackland Air Force Base, TX (United States)

    2007-06-15

    While well-recognized in the orthopedic literature as a cause of chronic hip pain, acetabular retroversion has not been specifically described in the radiologic literature. Acetabular retroversion represents a particular form of hip dysplasia characterized by abnormal posterolateral orientation of the acetabulum. This pathophysiology predisposes the individual to subsequent anterior impingement of the femoral neck upon the anterior acetabular margin and fibrous labrum. Without treatment, cases may progress to damage of the anterior labrum and cartilage, with eventual early onset of osteoarthritic disease. This impinging condition has been described as occurring in isolation or as part of a complex dysplasia. We describe two cases of acetabular retroversion diagnosed by conventional radiographic evaluation of the pelvis, one in isolation and one occurring in the setting of a larger congenital syndrome. These cases illustrate the utility of the ''figure-eight'' sign in identifying abnormalities of acetabular version and thus assisting clinicians in properly identifying these individuals so that appropriate therapy may be instituted. (orig.)

  16. Complex orthopaedic management of patients with skeletal dysplasias

    Directory of Open Access Journals (Sweden)

    A. G. Baindurashvili

    2014-01-01

    Full Text Available Skeletal dysplasias are challenging for diagnostics and treatment. We present a series of fifteen patients with different forms of skeletal dysplasias with age ranged from 6 to 17 years with variable clinical presentations managed as a part of the project of scientific cooperation between Turner Paediatric Orthopaedic Institute and Orthopaedic Hospital Vienna-Speising. The spectrum of diagnoses included multiple epiphyseal dysplasia, spondyloepiphyseal dysplasia congenita, diastrophic dysplasia, metaphyseal dysplasia, spondylometaphyseal dysplasia, Stickler syndrome, Kniest dysplasia, and anauxetic dysplasia. Complex treatment, which included axial correction and juxta-articular realignment, was performed as a single-stage, or consecutive surgery. Surgical techniques included corrective osteotomies with internal fixation, guided growth technique and external fixation devices. Best results (full axial correction, normal alignment of the joint were achieved in 8 patients, including 2 patients with metaphyseal dysplasia, 2 patients with multiple epyphyseal dysplasia, 2 patients with spondyloepyphyseal dysplasia, patient with Stickler syndrome and patient with spondylometaphyseal dysplasia. Good results (partial correction at the present time were seen in 4 patients (2 patients with Kniest dysplasia, 1 - with multiple epyphyseal dysplasia and 1 - with anauxetic dysplasia. Satisfactory results (non-progressive condition in previous progression were obtained in 2 patients with diastrophic dysplasia, and poor results (progression of the deformity - in 1 patient with diastrophic dysplasia. Positive results in most of the cases of our series make promising future for usage of complex approach for orthopedic management of children with skeletal dysplasias; advanced international cooperation is productive and helpful for diagnostics and management of rare diseases.

  17. The Bone Dysplasia Ontology: integrating genotype and phenotype information in the skeletal dysplasia domain

    Directory of Open Access Journals (Sweden)

    Groza Tudor

    2012-03-01

    Full Text Available Abstract Background Skeletal dysplasias are a rare and heterogeneous group of genetic disorders affecting skeletal development. Patients with skeletal dysplasias suffer from many complex medical issues including degenerative joint disease and neurological complications. Because the data and expertise associated with this field is both sparse and disparate, significant benefits will potentially accrue from the availability of an ontology that provides a shared conceptualisation of the domain knowledge and enables data integration, cross-referencing and advanced reasoning across the relevant but distributed data sources. Results We introduce the design considerations and implementation details of the Bone Dysplasia Ontology. We also describe the different components of the ontology, including a comprehensive and formal representation of the skeletal dysplasia domain as well as the related genotypes and phenotypes. We then briefly describe SKELETOME, a community-driven knowledge curation platform that is underpinned by the Bone Dysplasia Ontology. SKELETOME enables domain experts to use, refine and extend and apply the ontology without any prior ontology engineering experience--to advance the body of knowledge in the skeletal dysplasia field. Conclusions The Bone Dysplasia Ontology represents the most comprehensive structured knowledge source for the skeletal dysplasias domain. It provides the means for integrating and annotating clinical and research data, not only at the generic domain knowledge level, but also at the level of individual patient case studies. It enables links between individual cases and publicly available genotype and phenotype resources based on a community-driven curation process that ensures a shared conceptualisation of the domain knowledge and its continuous incremental evolution.

  18. Morgan line and its relationship with distraction index, angle of inclination and degenerative joint disease in the diagnosis of canine hip dysplasia

    Directory of Open Access Journals (Sweden)

    F.G. Miranda

    2016-08-01

    Full Text Available ABSTRACT We evaluated 160 hip joint radiographs of 40 dogs of different large breeds (25 females and 15 males from the metropolitan area of Belo Horizonte, Minas Gerais, Brazil. The radiographs of each dog were obtained at two different stages: stage 1 (mean 7.23 months and stage 2 (mean 14.25. The conventional radiographic method (CRM and the radiographic distraction method (RDM were used, carried out in both stages. CRM measured the Norberg angle (NA, the angle of inclination (AI and evaluated the presence of degenerative joint disease (DJD. The MRD was performed to establish the distraction index (DI. The aims were to evaluate the presence of the Morgan line and other signs of DJD and correlate them with the degree of canine hip dysplasia (CHD and also check if the DI greater than 0.3 (first stage was associated with the presence of ML (second stage. It was found that DI, AI and changes of femoral neck and the formation of osteophytes were associated with the presence of ML. It was observed that if the DI is greater than 0.3 at the first stage, the chance of a positive outcome of ML in the second stage increases by 7.2 times. Thus, 49 joints showed DI > 0.3 at the first stage, in which 31 (63.3 % presented ML at the second stage. Of the 31 animals that showed DI ≤ 0.3 at first, six (19.4% had LM at the second stage. There has been a significant association between the presence of ML and the degree of CHD. The more severe the CHD, the higher the percentage of positive ML results. Thus, among the 24 (60 % animals that showed ML, 11 (45.83 % were classified as severe dysplastics, 5 (20.83% as moderate and 8 (33.33 % as mild. None of the animals classified as normal or borderline presented ML. Among the 8 animals classified as mild dysplastics, 5 showed only ML as DJD.

  19. Osseous dysplasia (cemento-osseous dysplasia) of the jaw bones in western Pennsylvania patients: analysis of 35 cases.

    Science.gov (United States)

    Owosho, Adepitan A; Potluri, Anitha; Bilodeau, Elizabeth A

    2013-01-01

    The purpose of this study is to analyze the demographic, clinical, and radiographic presentations of osseous dysplasia of the jaws in western Pennsylvania patients and its associated complications. The clinical records and radiographs of patients diagnosed with osseous (cement-osseous) dysplasia were retrieved from the electronic health record of the University of Pittsburgh, School of Dental Medicine from 2007 to 2012. All cases were reviewed; the WHO criteria and classification for osseous dysplasia was used. Clinical and demographic data, radiographic findings, and final diagnoses were collected and analyzed. 35 cases of osseous dysplasia were retrieved over the six-year period.The majority (33) were females [94.3%], with ages ranging from 26 to 89 years, with a mean age of 53.9 years +/- standard deviation of 15.6 years, 32 [91.4%] were African Americans and 3 [8.6%] were Caucasians. 17 [48.6%] were florid osseous dysplasia, 13 [37.1%] periapical osseous dysplasia and 5 [14.3%] focal osseous dysplasia. Of the 35 patients only 8 [22.9%] patients were symptomatic. All florid osseous dysplasia patients were African American females, with 7 of the patients being symptomatic and the commonest symptom being pain. Also, all periapical osseous dysplasia patients were African Americans (12 females and 1 male), with 1 of the patients presenting with widening of the diastema. Of the focal osseous dysplasia patients, 3 were Caucasians and 2 African American (4 females and 1 male). The cases occurred mostly in African American females with a peak incidence in the fifth and sixth decades of life; most cases occurred in the mandible. The commonest form of osseous dysplasias was the florid osseous dysplasia which is most likely to present with symptoms.

  20. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome).

    Science.gov (United States)

    Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

    2014-01-01

    Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

  1. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome)

    OpenAIRE

    Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

    2014-01-01

    Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

  2. Spondylometaphyseal dysplasia with hypercalcemia

    International Nuclear Information System (INIS)

    Bagga, A.; Srivastava, R.N.; Gupta, S.; Gupta, A.

    1989-01-01

    Kozlowski's spondylometaphyseal dysplasia is characterised by short-trunk dwarfism, platyspondyly, metaphyseal dysplasia and retarded bone age. We report an association of this syndrome with asymptomatic, hypocalciuric hypercalcemia, a previously undocumented finding. (orig.)

  3. Expression and cellular distribution of multidrug transporter proteins in two major causes of medically intractable epilepsy: Focal cortical dysplasia and glioneuronal tumors

    NARCIS (Netherlands)

    Aronica, E.; Gorter, J. A.; Jansen, G. H.; van Veelen, C. W. M.; van Rijen, P. C.; Leenstra, S.; Ramkema, M.; Scheffer, G. L.; Scheper, R. J.; Troost, D.

    2003-01-01

    The cell-specific distribution of multidrug resistance extrusion pumps was studied in developmental glioneuronal lesions, including focal cortical dysplasia (15 cases) and ganglioglioma (15 cases) from patients with medically intractable epilepsy. Lesional, perilesional, as well as normal brain

  4. A familial case of alveolar capillary dysplasia with misalignment of pulmonary veins supports paternal imprinting of FOXF1 in human

    Science.gov (United States)

    Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare developmental lung disorder that is uniformly lethal. Affected infants die within the first few weeks of their life despite aggressive treatment, although a few cases of late manifestation and longer survival have ...

  5. Oral rehabilitation of a young patient with hypohidrotic ectodermal dysplasia: A clinical report

    Directory of Open Access Journals (Sweden)

    A Anuroopa

    2012-01-01

    Full Text Available Ectodermal dysplasia (ED represents a group of patients with mild to severe congenital and developmental anomalies. Dentists are the first person to identify ED in young patients. The impairment is not just the form and function but extends to the social outlook as well as the psychology of the affected individual. This case report describes management of ED with a long-span fixed partial denture fabricated using a Broadrick flag.

  6. Arthroscopic Labral Base Repair in the Hip: 5-Year Minimum Clinical Outcomes.

    Science.gov (United States)

    Domb, Benjamin G; Yuen, Leslie C; Ortiz-Declet, Victor; Litrenta, Jody; Perets, Itay; Chen, Austin W

    2017-10-01

    Arthroscopic labral base repair (LBR) in the hip is a previously described technique designed to restore the native functional anatomy of the labrum by reproducing its seal against the femoral head. LBR has been shown to have good short-term outcomes. Hypothesis/Purpose: The purpose was to evaluate clinical outcomes of an LBR cohort with a minimum 5-year follow-up. It was hypothesized that patients who underwent LBR would continue to have significant improvement from their preoperative scores and maintain scores similar to their 2-year outcomes. Case series; Level of evidence, 4. Data for patients undergoing primary hip arthroscopic surgery with LBR from February 2008 to May 2011 with a minimum 5-year follow-up were prospectively collected and retrospectively reviewed. Patients with preoperative Tonnis osteoarthritis grade ≥2, previous hip conditions (slipped capital femoral epiphysis, avascular necrosis, Legg-Calv-Perthes disease), severe dysplasia (lateral center-edge angle hip surgery were excluded. Statistical equivalence tests evaluated patient-reported outcomes (PROs) including the modified Harris Hip Score (mHHS), Non-Arthritic Hip Score (NAHS), Hip Outcome Score-Sport-Specific Subscale (HOS-SSS), visual analog scale (VAS) for pain, and patient satisfaction (0-10 scale; 10 = very satisfied). Of the 70 patients (74 hips) who met inclusion and exclusion criteria, 60 (85.7%) patients (64 hips) were available at a minimum 5-year follow-up. All PRO scores significantly improved from preoperative values with a mean follow-up of 67.8 ± 7.4 months (range, 60.0-89.7 months). The mean mHHS increased from 64.4 ±13.8 to 85.3 ± 17.7 ( P hip arthroscopic surgery has yet to be determined; however, these midterm results demonstrate the rates of additional procedures (both secondary arthroscopic surgery and conversion to total hip arthroplasty), that may be necessary after 2 years.

  7. Regulation of ciliary function by fibroblast growth factor signaling identifies FGFR3-related disorders achondroplasia and thanatophoric dysplasia as ciliopathies

    Czech Academy of Sciences Publication Activity Database

    Kunová Bosáková, M.; Vařecha, M.; Hampl, Marek; Duran, I.; Nita, A.; Buchtová, Marcela; Dosedělová, Hana; Machat, R.; Xie, Y.; Ni, Z.; Martin, J. H.; Chen, L.; Jansen, G.; Krakow, D.; Krejčí, P.

    2018-01-01

    Roč. 27, č. 6 (2018), s. 1093-1105 ISSN 0964-6906 R&D Projects: GA MŠk EF15_003/0000460 Institutional support: RVO:67985904 Keywords : achondroplasia * thanatophoric dysplasia Subject RIV: EB - Genetics ; Molecular Biology OBOR OECD: Developmental biology Impact factor: 5.340, year: 2016

  8. Two or more impingement and/or instability deformities are often present in patients with hip pain.

    Science.gov (United States)

    Tibor, Lisa M; Liebert, Gunnar; Sutter, Reto; Impellizzeri, Franco M; Leunig, Michael

    2013-12-01

    Damage to the hip can occur due to impingement or instability caused by anatomic factors such as femoral and acetabular version, neck-shaft angle, alpha angle, and lateral center-edge angle (CEA). The associations between these anatomic factors and how often they occur in a painful hip are unclear but if unaddressed might explain failed hip preservation surgery. We determined (1) the influence of sex on the expression of impingement-related or instability-related factors, (2) the associations among these factors, and (3) how often both impingement and/or instability factors occur in the same hip. We retrospectively reviewed a cohort of 170 hips (145 patients) undergoing MR arthrography of the hip for any reason. We excluded 58 hips with high-grade dysplasia, Perthes' sequelae, previous surgery, or incomplete radiographic information, leaving 112 hips (96 patients). We measured femoral version and alpha angles on MR arthrograms. Acetabular anteversion, lateral CEA, and neck-shaft angle were measured on pelvic radiographs. We observed a correlation between sex and alpha angle. Weak or no correlations were observed between the other five parameters. In 66% of hips, two or more (of five) impingement parameters, and in 51% of hips, two or more (of five) instability parameters were found. Patients with hip pain frequently have several anatomic factors potentially contributing to chondrolabral damage. To address pathologic hip loading due to impingement and/or instability, all of the anatomic influences should be known. As we found no associations between anatomic factors, we recommend an individualized assessment of each painful hip.

  9. Sponastrime dysplasia

    International Nuclear Information System (INIS)

    Lachman, R.S.; California Univ., Los Angeles, Torrance, CA; Stoss, H.; Spranger, J.

    1989-01-01

    The 2nd family with Sponastrime Dysplasia is described. The clinical, radiologic and chondro-osseous morphology of boy and girl siblings are presented. The facial appearance is an 'oriental look' with midface hypoplasia and a saddle nose. The radiological findings include the spinal changes of lordosis, osteoporosis and pear-shaped vertebrae, as well as striated metaphyses (osteopathia striata). The morphological findings suggest a disturbance in the formation of cartilage, with a defect in collagen and proteoglycans synthesis in this rare autosomal recessive skeletal dysplasia. (orig.)

  10. Mandibulo-acral dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Hoeffel, J.C.; Mainard, L. [Dept. of Radiology, Children' s Hospital, Vandoeuvre (France); Chastagner, P. [Dept. of Medicine, Children' s Hospital, Vandoeuvre (France); Hoeffel, C.C. [UFR Faculte de Medecine Cochin, Paris (France)

    2000-11-01

    We report on a 7 year-old-girl with mandibulo-acral dysplasia. When she was 3 years of age it mimicked scleroderma because of skin atrophy and later on a Hutchinson-Gilford progeria syndrome (HGP). Acro-mandibular dysplasia was diagnosed because of facial hypoplasia and mandibular hypoplasia. The bilateral proximal mid-humeral notch seen in this case is unusual. (orig.)

  11. Osteochondritis Dissecans of the Hip

    International Nuclear Information System (INIS)

    Linden, B.; Jonsson, K.; Redlund-Johnell, I.

    2003-01-01

    Purpose: To investigate the clinical and radiological characteristics of osteochondritis dissecans (OD) of the hip and the outcome of this condition after treatment. Material and Methods: Twelve male and 3 female patients with OD were retrospectively studied. Six patients had a history of Legg-Calve-Perthes disease (LCPD) and among them 2 also had had a trauma to the hip. A further 5 had had trauma and 1 a developmental dislocation of the hip (DDH). The remaining 3 patients had no history of previous hip disease or trauma. All patients were examined with plain radiography, 7 with MR, 3 with CT and 2 with hip arthrography. Results: All OD lesions were detected at plain radiography, and most of them were located near the fovea. At MR the lesions had low signal intensity at T1-weighted sequences, and 6/7 had edema or fluid collection in or adjacent to the lesion on T2-weighted sequences. The early treatment in 7 patients was surgery, 2 had had conservative treatment and 6 no treatment. At follow-up 12 years after radiological diagnosis, 5 patients had hip arthrosis, 4 of whom were treated with arthroplasty. All but 3 had reduced hip rotation and all but 2 (with arthroplasty) had load pain. Three of the patients with earlier surgery had not developed arthrosis. Conclusions: OD lesions are usually well seen with plain radiography. There is a great risk of developing early arthrosis and it seems that early surgery is connected with arthrosis development. Thus only symptomatic treatment is recommended

  12. Osteochondritis Dissecans of the Hip

    Energy Technology Data Exchange (ETDEWEB)

    Linden, B. [Hoeglands Hospital, Eksjoe (Sweden). Dept. of Orthopedic Surgery; Jonsson, K. [Univ. Hospital, Lund (Sweden). Center for Medical Imaging and Physiology; Redlund-Johnell, I. [Univ. Hospital, Malmoe (Sweden). Dept. of Diagnostic Radiology

    2003-03-01

    Purpose: To investigate the clinical and radiological characteristics of osteochondritis dissecans (OD) of the hip and the outcome of this condition after treatment. Material and Methods: Twelve male and 3 female patients with OD were retrospectively studied. Six patients had a history of Legg-Calve-Perthes disease (LCPD) and among them 2 also had had a trauma to the hip. A further 5 had had trauma and 1 a developmental dislocation of the hip (DDH). The remaining 3 patients had no history of previous hip disease or trauma. All patients were examined with plain radiography, 7 with MR, 3 with CT and 2 with hip arthrography. Results: All OD lesions were detected at plain radiography, and most of them were located near the fovea. At MR the lesions had low signal intensity at T1-weighted sequences, and 6/7 had edema or fluid collection in or adjacent to the lesion on T2-weighted sequences. The early treatment in 7 patients was surgery, 2 had had conservative treatment and 6 no treatment. At follow-up 12 years after radiological diagnosis, 5 patients had hip arthrosis, 4 of whom were treated with arthroplasty. All but 3 had reduced hip rotation and all but 2 (with arthroplasty) had load pain. Three of the patients with earlier surgery had not developed arthrosis. Conclusions: OD lesions are usually well seen with plain radiography. There is a great risk of developing early arthrosis and it seems that early surgery is connected with arthrosis development. Thus only symptomatic treatment is recommended.

  13. A Case of Ectrodactyly, Ectodermal Dysplasia, Cleft Lip and Palate Syndrome Associated with Hydrocephaly

    Directory of Open Access Journals (Sweden)

    Buket Uysal Aladag

    2013-06-01

    Full Text Available Ectrodactyly, ectodermal dysplasia, cleft lip, and palate syndrome (EEC is a genetic developmental disorder characterized by ectrodactyly, ectodermal dysplasia and orofacial clefts (cleft lip/ palate. A few cases have been reported in literature. The cardinal components of the syndrome are ectrodactyly and syndactyly of the hands and feet, cleft lip with or without cleft palate, and abnormalities ectodermal structures including skin (i.e. hypopigmented and dry skin, hyperkeratosis, skin atrophy, hair (sparse hair and eye brows, teeth (small, absent or dysplastic teeth, nails (nail dystrophy and exocrine glands (reduction/ absence of sweat, sebaceous and salivary glands. A multidisciplinary approach for treatment is needed which is co-ordinated by orthopedic, plastic, dental surgeons, ophthalmologist, dermatologists and speech therapists, psychologists. We presented EEC syndrome case with hydrocephaly by the literature. [Cukurova Med J 2013; 38(3.000: 531-535

  14. Femoro-acetabular impingement and hip pain with conventionally normal x-rays.

    LENUS (Irish Health Repository)

    Baker, J F

    2010-06-01

    There has in recent years been a fundamental change in the understanding of hip pain in the young adult and hip pain without plain radiographic findings of arthritis. Pain in these groups has long represented a diagnostic and therapeutic challenge. With new appreciation of hip biomechanics, pathological processes and the arrival of modern imaging modalities we now have a greater understanding of non-arthritic hip pathology. One of the commonest yet least well recognized \\'new\\' diagnoses around the hip is femoro-acetabular impingement (FAI). FAI is a developmental condition of the hip joint that is associated with abnormal anatomical configuration and thus joint mechanics on either the femoral or acetabular sides or both. It is hypothesized to have a variety of precipitants and may ultimately lead to labral and chondral injury and what has previously been referred to as \\'primary\\' or \\'idiopathic\\' hip osteoarthritis.

  15. Hip Joint Replacement Using Monofilament Polypropylene Surgical Mesh: An Animal Model

    Directory of Open Access Journals (Sweden)

    Jacek Białecki

    2014-01-01

    Full Text Available Hip joint dysplasia is a deformation of the articular elements (pelvic acetabulum, head of the femur, and/or ligament of the head of the femur leading to laxity of the hip components and dislocation of the femoral head from the pelvic acetabulum. Diagnosis is based on symptoms observed during clinical and radiological examinations. There are two treatment options: conservative and surgical. The classic surgical procedures are juvenile pubic symphysiodesis (JPS, triple pelvic osteotomy (TPO, total hip replacement (THR, and femoral head and neck resection (FHNE. The aim of this experiment was to present an original technique of filling the acetabulum with a polypropylene implant, resting the femoral neck directly on the mesh. The experiment was performed on eight sheep. The clinical value of the new surgical technique was evaluated using clinical, radiological, and histological methods. This technique helps decrease the loss of limb length by supporting the femoral neck on the mesh equivalent to the femoral head. It also reduces joint pain and leads to the formation of stable and mobile pseudarthrosis. The mesh manifested osteoprotective properties and enabled the formation of a stiff-elastic connection within the hip joint. The method is very cost-effective and the technique itself is simple to perform.

  16. Histochemical Analysis of Renal Dysplasia with Ureteral Atresia

    International Nuclear Information System (INIS)

    Kawate, Toyoko; Kawamura, Ryuki; Uchida, Takenori; Takahashi, Kyosuke; Hasegawa, Tomohiro; Futamata, Haruo; Katoh, Ryohei; Takeda, Sen

    2009-01-01

    Unilateral small kidney with ureteral obstruction was discovered in a 74-year-old female cadaver during an anatomical dissection course. In order to elucidate the histogenesis of renal dysplasia, we carried out histochemical and immunohistochemical analyses. On macroscopic view, the kidney was approximately 3 cm in length, 2 cm in width and weighed only 9 g. Although the ureter ran from the renal hilus to the bladder, its width was under 2 mm. The renal parenchyma was extremely thin and there was a large congested vein in the renal sinus. On microscopic examination of the kidney, we observed that numerous developing renal tubules had cytokeratin-positive epithelia, most of which were surrounded by concentric fibrosis. However, we could not detect any structures resembling the collecting duct, renal tubules, renal pelvis, or glomeruli. The concentric mesencymal fibrous tissue surrounding the immature renal tubules contained the smooth muscles that were positive for h-caldesmon. Serial sections of the ureter revealed several small and discontinuous lacunae lined by cuboidal and transitional epithelium, which did not constitute a patent lumen through the bladder. This case is a rare case of renal dysplasia with defect in recanalization of the ureteral bud during the early developmental stage

  17. Ectodermal Dysplasia: A Case Report

    Science.gov (United States)

    2011-01-01

    Ectodermal dysplasia is a hereditary disease characterized by dysplasia of tissues of ectodermal origin. The incidence of ectodermal dysplasia is rare (1 in 100,000 birth). This case report discusses the features, classification and prosthetic treatment plan (upper partial denture and lower complete denture for upper partial and lower complete edentulous arches respectively). This treatment plan would be able to provide psychological and functional boost to the sufferer. PMID:27678241

  18. Focal cortical dysplasia – review

    International Nuclear Information System (INIS)

    Kabat, Joanna; Król, Przemysław

    2012-01-01

    Focal cortical dysplasia is a malformation of cortical development, which is the most common cause of medically refractory epilepsy in the pediatric population and the second/third most common etiology of medically intractable seizures in adults. Both genetic and acquired factors are involved in the pathogenesis of cortical dysplasia. Numerous classifications of the complex structural abnormalities of focal cortical dysplasia have been proposed – from Taylor et al. in 1971 to the last modification of Palmini classification made by Blumcke in 2011. In general, three types of cortical dysplasia are recognized. Type I focal cortical dysplasia with mild symptomatic expression and late onset, is more often seen in adults, with changes present in the temporal lobe. Clinical symptoms are more severe in type II of cortical dysplasia usually seen in children. In this type, more extensive changes occur outside the temporal lobe with predilection for the frontal lobes. New type III is one of the above dysplasias with associated another principal lesion as hippocampal sclerosis, tumor, vascular malformation or acquired pathology during early life. Brain MRI imaging shows abnormalities in the majority of type II dysplasias and in only some of type I cortical dysplasias. The most common findings on MRI imaging include: focal cortical thickening or thinning, areas of focal brain atrophy, blurring of the gray-white junction, increased signal on T2- and FLAIR-weighted images in the gray and subcortical white matter often tapering toward the ventricle. On the basis of the MRI findings, it is possible to differentiate between type I and type II cortical dysplasia. A complete resection of the epileptogenic zone is required for seizure-free life. MRI imaging is very helpful to identify those patients who are likely to benefit from surgical treatment in a group of patients with drug-resistant epilepsy. However, in type I cortical dysplasia, MR imaging is often normal, and also in both

  19. A comparative assessment of hip joints some morphometrical criteria for Rottweilers and other breed dogs

    International Nuclear Information System (INIS)

    Kozinda, O.; Bruveris, Z.

    2006-01-01

    The aim and objective of the investigation were to make the diagnosis of the hip dysplasia as impartial and precise as possible by using several x-ray morphometrical measurements, to make a comparative analysis of these measurements by using a linear regression analysis, and finally to calculate the value of these parameters and ranks. In total 36 radiogrammes of Rottweiler breed dogs and 24 radiogrammes of other breed dogs of different ages of both sexes, which belonged to owners, were used for the research. In each of the radiogramme 7 morhometrical measurements of the right and left hip joint were made: the Norberg angle, the inclination angle, the tangential angle, the geometrical centre placement of the CFH; the width of the lateral joint fissure gap the width of the medial joint fissure gap and distraction index. From the obtained results the following conclusions can be drawn: to make the diagnosis in dogs of Rottweiler breed, the most essential parameters are distraction index on right hip joint and the tangential angle and width of medial joint fissure on left hip joint

  20. Genetics Home Reference: osteoglophonic dysplasia

    Science.gov (United States)

    ... 1 link) Genetic Testing Registry: Osteoglophonic dysplasia Other Diagnosis and Management Resources (1 link) Seattle Children's Hospital: Dwarfism and Bone Dysplasias General Information from MedlinePlus (5 ...

  1. Presentation of hypohidrotic ectodermal dysplasia in two siblings

    Directory of Open Access Journals (Sweden)

    Uday Ginjupally

    2015-01-01

    Full Text Available Ectodermal dysplasias are a large hereditary group of disorders which are usually manifested as X-linked recessive disorders and have a full expression in males, whereas females show little to no signs of the disorder. The two most common types of ectodermal dysplasias are hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome and hidrotic ectodermal dysplasia (Clouston syndrome. Hypohidrotic ectodermal dysplasia is characterized by hypodontia, hypotrichosis, and hypohidrosis. Here, we present two female sibling cases of hypohidrotic ectodermal dysplasia.

  2. Steel syndrome: dislocated hips and radial heads, carpal coalition, scoliosis, short stature, and characteristic facial features.

    Science.gov (United States)

    Flynn, John M; Ramirez, Norman; Betz, Randal; Mulcahey, Mary Jane; Pino, Franz; Herrera-Soto, Jose A; Carlo, Simon; Cornier, Alberto S

    2010-01-01

    A syndrome of children with short stature, bilateral hip dislocations, radial head dislocations, carpal coalitions, scoliosis, and cavus feet in Puerto Rican children, was reported by Steel et al in 1993. The syndrome was described as a unique entity with dismal results after conventional treatment of dislocated hips. The purpose of this study is to reevaluate this patient population with a longer follow-up and delineate the clinical and radiologic features, treatment outcomes, and the genetic characteristics. This is a retrospective cohort study of 32 patients in whom we evaluated the clinical, imaging data, and genetic characteristics. We compare the findings and quality of life in patients with this syndrome who have had attempts at reduction of the hips versus those who did not have the treatment. Congenital hip dislocations were present in 100% of the patients. There was no attempt at reduction in 39% (25/64) of the hips. In the remaining 61% (39/64), the hips were treated with a variety of modalities fraught with complications. Of those treated, 85% (33/39) remain dislocated, the rest of the hips continue subluxated with acetabular dysplasia and pain. The group of hips that were not treated reported fewer complaints and limitation in daily activities compared with the hips that had attempts at reduction. Steel syndrome is a distinct clinical entity characterized by short stature, bilateral hip and radial head dislocation, carpal coalition, scoliosis, cavus feet, and characteristic facial features with dismal results for attempts at reduction of the hips. Prognostic Study Level II.

  3. Ultrasound versus Magnetic Resonance Arthrography in Acetabular Labral Tear Diagnostics: A Prospective Comparison in 20 Dysplastic Hips

    Energy Technology Data Exchange (ETDEWEB)

    Troelsen, A.; Jacobsen, S.; Bolvig, L.; Gelineck, J.; Roemer, L.; Soeballe, K. [Orthopedic Research Unit and Dept. of Radiology, Univ. Hospital of Aarhus, A arhus (Denmark)

    2007-11-15

    Background: Acetabular labral tears are highly associated with hip dysplasia. Magnetic resonance arthrography (MR arthrography) is the expensive and time-consuming contemporary gold-standard method in the radiological assessment of acetabular labral tears. Purpose: To assess the diagnostic ability of noninvasive ultrasound (US) examination compared to MR arthrography in diagnosing acetabular labral tears in dysplastic hip joints. Material and Methods: The study compared US examination and MR arthrography diagnosis of labral tears in 20 consecutively referred dysplastic hip joints. Results: The ability to diagnose acetabular labral tears upon US examination was calculated: sensitivity 44%, specificity 75%, positive predictive value 88%, and negative predictive value 25%. Conclusion: The ability of US examination in diagnosing acetabular labral tears is not yet good enough. The technique is still to be developed, and more experience, especially with the interpretation of US examinations, is needed.

  4. Oral epithelial dysplasia classification systems

    DEFF Research Database (Denmark)

    Warnakulasuriya, S; Reibel, J; Bouquot, J

    2008-01-01

    At a workshop coordinated by the WHO Collaborating Centre for Oral Cancer and Precancer in the United Kingdom issues related to potentially malignant disorders of the oral cavity were discussed by an expert group. The consensus views of the Working Group are presented in a series of papers....... In this report, we review the oral epithelial dysplasia classification systems. The three classification schemes [oral epithelial dysplasia scoring system, squamous intraepithelial neoplasia and Ljubljana classification] were presented and the Working Group recommended epithelial dysplasia grading for routine...... use. Although most oral pathologists possibly recognize and accept the criteria for grading epithelial dysplasia, firstly based on architectural features and then of cytology, there is great variability in their interpretation of the presence, degree and significance of the individual criteria...

  5. Recessive multiple epiphyseal dysplasia (rMED with homozygosity for C653S mutation in the DTDST gene - Phenotype, molecular diagnosis and surgical treatment of habitual dislocation of multilayered patella: Case report

    Directory of Open Access Journals (Sweden)

    Bonafé Luisa

    2010-06-01

    Full Text Available Abstract Background Multiple epiphyseal dysplasia (MED is one of the more common generalised skeletal dysplasias. Due to its clinical heterogeneity diagnosis may be difficult. Mutations of at least six separate genes can cause MED. Joint deformities, joint pain and gait disorders are common symptoms. Case Presentation We report on a 27-year-old male patient suffering from clinical symptoms of autosomal recessive MED with habitual dislocation of a multilayered patella on both sides, on the surgical treatment and on short-term clinical outcome. Clinical findings were: bilateral hip and knee pain, instability of femorotibial and patellofemoral joints with habitual patella dislocation on both sides, contractures of hip, elbow and second metacarpophalangeal joints. Main radiographic findings were: bilateral dislocated multilayered patella, dysplastic medial tibial plateaus, deformity of both femoral heads and osteoarthritis of the hip joints, and deformity of both radial heads. In the molecular genetic analysis, the DTDST mutation g.1984T > A (p.C653S was found at the homozygote state. Carrier status was confirmed in the DNA of the patient's parents. The mutation could be considered to be the reason for the patient's disease. Surgical treatment of habitual patella dislocation with medialisation of the tibial tuberosity led to an excellent clinical outcome. Conclusions The knowledge of different phenotypes of skeletal dysplasias helps to select genes for genetic analysis. Compared to other DTDST mutations, this is a rather mild phenotype. Molecular diagnosis is important for genetic counselling and for an accurate prognosis. Even in case of a multilayered patella in MED, habitual patella dislocation could be managed successfully by medialisation of the tibial tuberosity.

  6. Bone scintigraphy in polyostotic fibrous dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Wadhwa, S.S.; Mansberg, R.; Fernandes, V.B. [Illawarra Regional Hospital, Wollongong, NSW, (Australia)

    1998-03-01

    Fibrous dysplasia is a benign skeletal disorder of unknown aetiology. Fibrous dysplasia characteristically involves the fibrous replacement of portions of the medullary cavities of a single bone (monostotic) or multiple bones (polyostotic). Bones typically involved include the femurs, tibiae, ribs and maxillae. The polyostotic form may be accompanied by skin pigmentation and endocrine abnormalities (McCune Allbright Syndrome). Radiological findings in fibrous dysplasia are variable, ranging from completely radiolucent to radio-opaque lesions, depending on the amount of fibrous or osseous tissue deposited in the medulla. The most common radiographic finding is that of a ground glass-like semi-opaque lesion. Case reports on scintigraphic manifestation of fibrous dysplasia are scanty. We present radiological and scintigraphic findings of polyostotic fibrous dysplasia in a young male. (authors). 3 refs., 1 fig.

  7. Recurrence of alveolar capillary dysplasia with misalignment of pulmonary veins in two consecutive siblings

    OpenAIRE

    Benevides, Gabriel Nuncio; Picciarelli de Lima, Patricia; Felipe-Silva, Aloisio; Lovisolo, Silvana Maria; Pereira de Melo, Ana Maria Andrello Gonçalves

    2015-01-01

    Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, developmental lung disorder, which has been increasingly reported. This entity usually presents as neonatal persistent pulmonary hypertension that is unresponsive to treatment, and is known to be uniformly fatal. Recent discoveries in the genetic field, and intensive treatments, may change the natural course of this disease, permitting easier diagnosis and giving new hope for the dismal prognosis. The autho...

  8. Displacement of the hip center of rotation after arthroplasty of Crowe III and IV dysplasia: a radiological and biomechanical study.

    Science.gov (United States)

    Abolghasemian, Mansour; Samiezadeh, Saeid; Jafari, Davood; Bougherara, Habiba; Gross, Allan E; Ghazavi, Mohammad T

    2013-06-01

    To study the direction and biomechanical consequences of hip center of rotation (HCOR) migration in Crowe type III and VI hips after total hip arthroplasty, post-operative radiographs and CT scans of several unilaterally affected hips were evaluated. Using a three-dimensional model of the human hip, the HCOR was moved in all directions, and joint reaction force (JRF) and abductor muscle force (AMF) were calculated for single-leg stance configuration. Comparing to the normal side, HCOR had displaced medially and inferiorly by an average of 23.4% and 20.8%, respectively, of the normal femoral head diameter. Significant decreases in JRF (13%) and AMF (46.13%) were observed in a presumptive case with that amount of displacement. Isolated inferior displacement had a small, increasing effect on these forces. In Crowe type III and IV hips, the HCOR migrates inferiorly and medially after THA, resulting in a decrease in JRF, AMF, and abductor muscle contraction force. Copyright © 2013 Elsevier Inc. All rights reserved.

  9. Jansen type of spondylometaphyseal dysplasia

    International Nuclear Information System (INIS)

    Campbell, J.B.; Kozlowski, K.; Lejman, T.; Sulko, J.

    2000-01-01

    Metaphyseal dysplasia, type Jansen (JMD), is a rare skeletal dysplasia with characteristic radiographic abnormalities. Of the various types of metaphyseal dysplasia, JMD shows the most severe alteration in metaphyseal architecture. All of the long tubular bones, including those of the hands and feet, show metaphyseal irregularity with a fragmented appearance and slight widening. The adjacent physes are abnormally widened, while the epiphyses tend to be slightly enlarged, rounded but otherwise normal. The spine in infancy and childhood usually appears normal. This report describes a young girl with metaphyseal changes typical of JMD except for the hands and feet, which appeared normal. She also showed very unusual abnormalities of the spine. This appears, therefore, to represent a unique osteochondrodysplasia for which we propose the term spondylometaphyseal dysplasia, type Jansen. (orig.)

  10. Treatment of athletes with symptomatic intra-articular hip pathology and athletic pubalgia/sports hernia: a case series.

    Science.gov (United States)

    Larson, Christopher M; Pierce, Bradley R; Giveans, M Russell

    2011-06-01

    The purpose of the study was to evaluate the results of surgical treatment in athletes with associated intra-articular hip pathology and extra-articular sports pubalgia. Between December 2003 and September 2009, 37 hips (mean patient age, 25 years) were diagnosed with both symptomatic athletic pubalgia and symptomatic intra-articular hip joint pathology. There were 8 professional athletes, 15 collegiate athletes, 5 elite high school athletes, and 9 competitive club athletes. Outcomes included an evaluation regarding return to sports and modified Harris Hip Score, Short Form 12 score, and visual analog scale score. We evaluated 37 hips at a mean of 29 months (range, 12 to 78 months) after the index surgery. Thirty-one hips underwent thirty-five athletic pubalgia surgeries. Hip arthroscopy was performed in 32 hips (30 cases of femoroacetabular impingement treatment, 1 traumatic labral tear, and 1 borderline dysplasia). Of 16 hips that had athletic pubalgia surgery as the index procedure, 4 (25%) returned to sports without limitations, and 11 (69%) subsequently had hip arthroscopy at a mean of 20 months after pubalgia surgery. Of 8 hips managed initially with hip arthroscopy alone, 4 (50%) returned to sports without limitations, and 3 (43%) had subsequent pubalgia surgery at a mean of 6 months after hip arthroscopy. Thirteen hips had athletic pubalgia surgery and hip arthroscopy at one setting. Concurrent or eventual surgical treatment of both disorders led to improved postoperative outcomes scores (P pubalgia or intra-articular hip pathology in this patient population, outcomes were suboptimal. Surgical management of both disorders concurrently or in a staged manner led to improved postoperative outcomes scoring and an unrestricted return to sporting activity in 89% of hips. Level IV, therapeutic case series. Copyright © 2011 Arthroscopy Association of North America. Published by Elsevier Inc. All rights reserved.

  11. Genetics Home Reference: thanatophoric dysplasia

    Science.gov (United States)

    ... thanatophoric dysplasia and their potential therapeutic implications for achondroplasia. Am J Med Genet A. 2010 Jan;152A( ... of fibroblast growth factor receptor 3 disorders: the achondroplasia family of skeletal dysplasias, Muenke craniosynostosis, and Crouzon ...

  12. [Total hip arthroplasty in post-dysplastic hip arthritis. Can type and position of the acetabular component influence longevity of the prosthesis?].

    Science.gov (United States)

    Fousek, J; Indráková, P

    2007-02-01

    The aim of the study was a retrospective evaluation of our patients with post-dysplastic hips treated by cemented or non-cemented total hip arthroplasty (THA) in order to ascertain which type and position of the acetabular component was most effective. In the years 1999-2002, 111 THA procedures were performed in 93 patients, 76 women and 17 men, with post-dysplastic hip arthritis. The average age of the patients at the time of implantation was 52.6 years. On the basis of pre-operative radiographic findings, the patients' conditions were evaluated using the Hartofilakidis classification into three disease categories: dysplasia, low dislocation and high dislocation, and the patients were placed in two groups. Group 1 included 78 patients, and group 2 comprised 26 patients. None of our patients was classified as having high dislocation. Thirty-nine of these patients had previously undergone surgery for dysplastic hips. A total of 104 THAs were evaluated, because radiographic data was incomplete in seven cases. In addition to X-ray findings, the prosthesis type (cemented, hybrid, non-cemented), post-operative complications and signs of loosening were included in the evaluation. Clinical outcomes were assessed by the Harris score. The follow-up terminating on 31st December 2005 was 67 months on the average. In group 1 patients, the average Harris score increased from 38.6 to 80.3 points and in group 2 patients from 35.5 to 84.9 points, mostly with excellent and good results. In 72.1 % of the hips, a press-fit acetabular component was implanted. In 55.8 % of the cases, the acetabular component was implanted off the anatomical center of rotation, into the high hip center, with the range from 9 to 20 mm and an average of 15 mm. The average limb lengthening was 2.5 cm, ranging from 1.0 to 3.5 cm. Our results show that it is more effective to use non-cemented THA for post-dysplastic hips. The implantation of a noncemented acetabular component into the high center provides

  13. Gracile bone dysplasias

    International Nuclear Information System (INIS)

    Kozlowski, Kazimierz; Masel, John; Sillence, David O.; Arbuckle, Susan; Juttnerova, Vera

    2002-01-01

    Gracile bone dysplasias constitute a group of disorders characterised by extremely slender bones with or without fractures. We report four newborns, two of whom showed multiple fractures. Two babies had osteocraniostenosis and one had features of oligohydramnios sequence. The diagnosis in the fourth newborn, which showed thin long bones and clavicles and extremely thin, poorly ossified ribs, is uncertain. Exact diagnosis of a gracile bone dysplasia is important for genetic counselling and medico-legal reasons. (orig.)

  14. Gracile bone dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, Kazimierz [Department of Medical Imaging, The Children' s Hospital at Westmead, Locked Bag 4001, Westmead 2145, NSW (Australia); Masel, John [Department of Radiology, Royal Children' s Hospital, Brisbane (Australia); Sillence, David O. [Department of Paediatrics and Child Health, The University of Sydney (Australia); Arbuckle, Susan [Department of Anatomical Pathology, The Children' s Hospital at Westmead, NSW (Australia); Juttnerova, Vera [Oddeleni Lekarske Genetiky, Hradec Kralove (Czech Republic)

    2002-09-01

    Gracile bone dysplasias constitute a group of disorders characterised by extremely slender bones with or without fractures. We report four newborns, two of whom showed multiple fractures. Two babies had osteocraniostenosis and one had features of oligohydramnios sequence. The diagnosis in the fourth newborn, which showed thin long bones and clavicles and extremely thin, poorly ossified ribs, is uncertain. Exact diagnosis of a gracile bone dysplasia is important for genetic counselling and medico-legal reasons. (orig.)

  15. DNA Fingerprinting Abnormalities Can Distinguish Ulcerative Colitis Patients with Dysplasia and Cancer from Those Who Are Dysplasia/Cancer-Free

    Science.gov (United States)

    Chen, Ru; Rabinovitch, Peter S.; Crispin, David A.; Emond, Mary J.; Koprowicz, Kent M.; Bronner, Mary P.; Brentnall, Teresa A.

    2003-01-01

    Patients with extensive ulcerative colitis (UC) of longer than 8 years duration are at high risk for the development of colorectal cancer. The cancers in these patients appear to develop in a stepwise manner with progressive histological changes from negative for dysplasia → indefinite for dysplasiadysplasia → cancer. The aim of this study was to determine the timing and extent of genomic instability in the progression of UC dysplasia and cancer. Using two polymerase chain reaction (PCR)-based DNA fingerprinting methods, arbitrarily primed PCR and intersimple sequence repeat PCR, we assessed DNA sequence variation in biopsies across the spectrum of cancerous, dysplastic, and nondysplastic mucosa. UC patients with dysplasia/cancer had substantial genomic instability in both their dysplastic and nondysplastic colonic mucosa, whereas instability was not present in the majority of UC patients without dysplasia/cancer. The degree of instability in nondysplastic tissue was similar to that of dysplastic/cancerous mucosa from the same patient, suggesting that this instability was widespread and reached the maximum level early in neoplastic progression. These results suggest that UC patients who develop dysplasia or cancer have an underlying process of genomic instability in their colonic mucosa whereas UC patients who are dysplasia-free do not. PMID:12547724

  16. Expansive focal cemento-osseous dysplasia.

    Science.gov (United States)

    Bulut, Emel Uzun; Acikgoz, Aydan; Ozan, Bora; Zengin, Ayse Zeynep; Gunhan, Omer

    2012-01-01

    To present a case of expansive focal cemento-osseous dysplasia and emphasize the importance of differential diagnosis. Cemento-osseous dysplasia is categorized into three subtypes on the basis of the clinical and radiographic features: Periapical, focal and florid. The focal type exhibits a single site of involvement in any tooth-bearing or edentulous area of the jaws. These lesions are usually asymptomatic; therefore, they are frequently diagnosed incidentally during routine radiographic examinations. Lesions are usually benign, show limited growth, and do not require further surgical intervention, but periodic follow-up is recommended because occasionally, this type of dysplasia progresses into florid osseous dysplasia and simple bone cysts are formed. A 24-year-old female patient was referred to our clinic for swelling in the left edentulous mandibular premolarmolar region and felt discomfort when she wore her prosthetics. She had no pain, tenderness or paresthesia. Clinical examination showed that the swelling in the posterior mandible that was firm, nonfluctuant and covered by normal mucosa. On panoramic radiography and computed tomography, a well defined lesion of approximately 1.5 cm in diameter of mixed density was observed. The swelling increased slightly in size over 2 years making it difficult to use prosthetics and, therefore, the lesion was totally excised under local anesthesia, and surgical specimens were submitted for histopathological examination. The histopathological diagnosis was focal cemento-osseous dysplasia. In the present case, because of the increasing size of the swelling making it difficult to use prosthetics, young age of the patient and localization of the lesion, in the initial examination, cemento-ossifying fibroma was suspected, and the lesion was excised surgically; the histopathological diagnosis confirmed it as focal cemento-osseous dysplasia. We present a case of expansive focal cemento-osseous dysplasia. Differential diagnosis

  17. Ectodermal dysplasia

    Directory of Open Access Journals (Sweden)

    Sonia Saggoo

    2009-01-01

    Full Text Available Hereditary hypohidrotic ectodermal dysplasia, also called the Christ-Siemens-Touraine Syndrome is characterized by congenital dysplasia of one or more ectodermal structures and is manifested by hypohidrosis, hypotrichosis and hypodontia. It is usually an X-linked recessive mendelian character which is rarely seen in males. It results from abnormal morphogenesis of cutaneous and oral embryonic ectoderm. Patients with this disorder exhibit smooth , thin and dry skin, fine and blond scanty hair. Intra-orally anodontia or hypodontia, with impaired development of alveolar process is seen. A case report of a rare case of this disorder in a female patient aged 18 years is hereby presented.

  18. Avascular necrosis in children with cerebral palsy after reconstructive hip surgery

    Science.gov (United States)

    Phillips, L.; Hesketh, K.; Schaeffer, E. K.; Andrade, J.; Farr, J.; Mulpuri, K.

    2017-01-01

    Abstract Purpose Progressive hip displacement is one of the most common orthopaedic pathologies in children with cerebral palsy (CP). Reconstructive hip surgery has become the standard treatment of care. Reported avascular necrosis (AVN) rates for hip reconstructive surgery in these patients vary widely in the literature. The purpose of this study is to identify the frequency and associated risk factors of AVN for reconstructive hip procedures. Methods A retrospective analysis was performed of 70 cases of reconstructive hip surgery in 47 children with CP, between 2009 and 2013. All 70 cases involved varus derotation osteotomy (VDRO), with 60% having combined VDRO and pelvic osteotomies (PO), and 21% requiring open reductions. Mean age at time of surgery was 8.82 years and 90% of patients were Gross Motor Function Classification System (GMFCS) 4 and 5. Radiographic dysplasia parameters were analysed at selected intervals, to a minimum of one year post-operatively. Severity of AVN was classified by Kruczynski's method. Bivar- iate statistical analysis was conducted using Chi-square test and Student's t-test. Results There were 19 (27%) noted cases of AVN, all radio- graphically identifiable within the first post-operative year. The majority of AVN cases (63%) were mild to moderate in severity. Pre-operative migration percentage (MP) (p = 0.0009) and post-operative change in MP (p = 0.002) were the most significant predictors of AVN. Other risk factors were: GMFCS level (p = 0.031), post-operative change in NSA (p = 0.02) and concomitant adductor tenotomy (0.028). Conclusion AVN was observed in 27% of patients. Severity of displacement correlates directly with AVN risk and we suggest that hip reconstruction, specifically VDRO, be performed early in the 'hip at risk' group to avoid this complication. PMID:29081846

  19. Avascular necrosis in children with cerebral palsy after reconstructive hip surgery.

    Science.gov (United States)

    Phillips, L; Hesketh, K; Schaeffer, E K; Andrade, J; Farr, J; Mulpuri, K

    2017-10-01

    Progressive hip displacement is one of the most common orthopaedic pathologies in children with cerebral palsy (CP). Reconstructive hip surgery has become the standard treatment of care. Reported avascular necrosis (AVN) rates for hip reconstructive surgery in these patients vary widely in the literature. The purpose of this study is to identify the frequency and associated risk factors of AVN for reconstructive hip procedures. A retrospective analysis was performed of 70 cases of reconstructive hip surgery in 47 children with CP, between 2009 and 2013. All 70 cases involved varus derotation osteotomy (VDRO), with 60% having combined VDRO and pelvic osteotomies (PO), and 21% requiring open reductions. Mean age at time of surgery was 8.82 years and 90% of patients were Gross Motor Function Classification System (GMFCS) 4 and 5. Radiographic dysplasia parameters were analysed at selected intervals, to a minimum of one year post-operatively. Severity of AVN was classified by Kruczynski's method. Bivar- iate statistical analysis was conducted using Chi-square test and Student's t-test. There were 19 (27%) noted cases of AVN, all radio- graphically identifiable within the first post-operative year. The majority of AVN cases (63%) were mild to moderate in severity. Pre-operative migration percentage (MP) (p = 0.0009) and post-operative change in MP (p = 0.002) were the most significant predictors of AVN. Other risk factors were: GMFCS level (p = 0.031), post-operative change in NSA (p = 0.02) and concomitant adductor tenotomy (0.028). AVN was observed in 27% of patients. Severity of displacement correlates directly with AVN risk and we suggest that hip reconstruction, specifically VDRO, be performed early in the 'hip at risk' group to avoid this complication.

  20. Results of Chiari pelvic osteotomy for acetabular dysplasia in adults; Association with bone scintigraphic findings

    Energy Technology Data Exchange (ETDEWEB)

    Nakamura, Nobuo; Ozono, Kenji; Sugano, Nobuhiko; Takaoka, Kunio; Ono, Hiroo (Osaka Univ. (Japan). Faculty of Medicine)

    1993-02-01

    In an attempt to determine indications of Chiari pelvic osteotomy in acetabular dysplasia, postoperative outcome of hip joint (64 joints) was examined on the basis of findings of bone scintigraphy. The subjects were 61 patients with osteoarthrosis of hip joint who underwent preoperative bone scintigraphy. The follow-up period ranged from 2 years to 9 years and 7 months with a mean of 4 years and 9 months. According to X-ray findings, 37 osteoarthrosis joints were staged as early and 27 as progressive. Preoperative bone scintigraphic findings fell into three: (I) normal or slight hot type (33 joints), (II) hot type at the weighting part (16 joints), and (III) double hot type in the weighting part and inside part (15 joints). None of the patients had severe surgical complications such as deep-seated infection, neuroparalysis and pseudojoint. According to the clinical staging for hip joint function, 7 (47%) of 64 joints were judged as poor after osteotomy, belonging to type III. Deterioration of osteoarthrosis was seen in 11 joints (41%) on X-ray films. Of these, 9 had type III. In conclusion, Chiari pelvic osteotomy should not be indicated when type III is shown on bone scintigrams. (N.K.).

  1. Clinically and radiological aspects of alterations in humeroradiulnar and hip joints in fila brasileiro dogs

    International Nuclear Information System (INIS)

    Araujo, Roberto B. de.

    1995-01-01

    Humeroradiulnar and hip joints of 100 fila brasileiro dogs were clinically and radiological studied. the objective was the demonstration of pathologies and their frequency in the metropolitan area of Belo Horizonte, Minas Gerais. The animals were 47 females and 53 males, aged between 9 and 120 months, although 74% of them had less than 36 months and were investigated according their city region and kind of food. Most of the animals came from region III (37%) and used to eat commercial food (32%) and mixed diet composed by commercial food meat and domestic food (26%). Diets with higher density and protein concentrations were more frequent in regions I and III and domestic food in region V. the alterations frequency in the humeroredioulnar joint was 33%. The most frequent pathologies observed were the fragmentation of the coronoid process (39.4%), osteochondrosis dissecans of the medial humeral condyle (31.8%) and ununited anconeal process (28.8%). It was observed significant difference in the frequency of hip dysplasia between the female group (46.5%) and male group (66.7%). For the entire group this frequency was 58%. No significant difference was found in the average inclination angle for normal dogs (149.73 deg ± 1.55 deg) and dysplastic dogs dysplasia. Control measures must be applied aiming the reduction of the joint alterations, as the fila brasileiro is a relative new breed. (author)

  2. Prevalence of malformations of the hip joint and their relationship to sex, groin pain, and risk of osteoarthritis: a population-based survey

    DEFF Research Database (Denmark)

    Gosvig, Kasper Kjaerulf; Jacobsen, Steffen; Sonne-Holm, Stig

    2010-01-01

    ratio, 2.2). Acetabular dysplasia and the subject's sex were not found to be significant risk factors for the development of hip osteoarthritis (p = 0.053 and p = 0.063, respectively). The prevalence of hip osteoarthritis was 9.5% in men and 11.2% in women. The prevalence of concomitant malformations...... of Østerbro, Copenhagen, Denmark. The inclusion criteria for this study were met by 1332 men and 2288 women. On the basis of radiographic criteria, the hips were categorized as being without malformations or as having an abnormality consisting of a deep acetabular socket, a pistol grip deformity......, or a combination of a deep acetabular socket and a pistol grip deformity. Hip osteoarthritis was defined radiographically as a minimum joint-space width of 0.13). A deep acetabular socket was a significant risk factor for the development of osteoarthritis (risk ratio, 2.4), as was a pistol grip deformity (risk...

  3. Ectodermal dysplasia: a genetic review.

    Science.gov (United States)

    Deshmukh, Seema; Prashanth, S

    2012-09-01

    Ectodermal dysplasia is a rare hereditary disorder with a characteristic physiognomy. It is a genetic disorder affecting the development or function of the teeth, hair, nails and sweat glands. Depending on the particular syndrome ectodermal dysplasia can also affect the skin, the lens or retina of the eye, parts of the inner ear, the development of fingers and toes, the nerves and other parts of the body. Each syndrome usually involves a different combination of symptoms, which can range from mild to severe. The history and lessons learned from hypohidrotic ectodermal dysplasia (HED) may serve as an example for unraveling of the cause and pathogenesis of other ectodermal dysplasia syndromes by demonstrating that phenotypically identical syndromes can be caused by mutations in different genes (EDA, EDAR, EDARADD), that mutations in the same gene can lead to different phenotypes and that mutations in the genes further downstream in the same signaling pathway (NEMO) may modify the phenotype quite profoundly. The aim of this paper is to describe and discuss the etiology, genetic review, clinical manifestations and treatment options of this hereditary disorder. How to cite this article: Deshmukh S, Prashanth S. Ectodermal Dysplasia: A Genetic Review. Int J Clin Pediatr Dent 2012; 5(3):197-202.

  4. Lower Lid Ectropion in Hypohidrotic Ectodermal Dysplasia

    Directory of Open Access Journals (Sweden)

    Xiaoyun Zhang

    2015-01-01

    Full Text Available We report a case of a lower lid ectropion with ectodermal dysplasia and ectropion blepharoplasty surgery experience. A 14-year-old Han nationality male patient with typical characteristics of hypohidrotic ectodermal dysplasia presented to our clinic for his right lower lid eversion. The patient was diagnosed as having hypohidrotic ectodermal dysplasia and underwent an uneventful blepharoplasty surgery. The lower lid maintained normal position during the 10-month follow-up period. Patients with ectodermal dysplasia could firstly visit ophthalmologist for their ectropion and blepharoplasty surgery could be useful for the disease.

  5. Pelvic radiograph in skeletal dysplasias: An approach

    Directory of Open Access Journals (Sweden)

    Manisha Jana

    2017-01-01

    Full Text Available The bony pelvis is constituted by the ilium, ischium, pubis, and sacrum. The pelvic radiograph is an important component of the skeletal survey performed in suspected skeletal dysplasia. Most of the common skeletal dysplasias have either minor or major radiological abnormalities; hence, knowledge of the normal radiological appearance of bony pelvis is vital for recognizing the early signs of various skeletal dysplasias. This article discusses many common and some uncommon radiological findings on pelvic radiographs along with the specific dysplasia in which they are seen; common differential diagnostic considerations are also discussed.

  6. Towards a unified classification of the ectodermal dysplasias: opportunities outweigh challenges.

    LENUS (Irish Health Repository)

    Irvine, Alan D

    2012-02-01

    The ectodermal dysplasias include a complex and highly diverse group of heritable disorders that share in common developmental abnormalities of ectodermal derivatives. The broader definition of ectodermal dysplasias (as heritable disorders involving at least two of the ectodermal derivatives nails, teeth, hair, and eccrine sweat glands) encompasses 170-200 conditions. Some conditions included by this definition are relatively common; others are rare and, in some cases, family-specific. Classification of the ectodermal dysplasias has largely been approached by categorizing patterns of clinical findings (phenotypic grouping). In the last 2 decades great progress has been made in understanding the molecular pathogenesis and inter-relatedness of some of these conditions and a new consensus approach to classification that incorporates this new information is needed. A comprehensive and definitive classification of these disorders would be highly valuable for the many stakeholders in ED. As disease-specific molecular treatments are developed, accurate classification will assume greater importance in designing registries to enable rapid identification of those with rare disorders who may wish to participate in clinical trials. Ideally a working classification of such a disparate collection of conditions would have a design and architecture that would facilitate easy accessibility by each of the key stakeholder groups and would encourage enhanced interaction between these parties. Attaining this objective is a major challenge but is achievable. This article reviews the historical-clinical perspective and the impact of recent developments in molecular biology in the field. Reflections are offered as to the future direction of classification systems in these disorders.

  7. Radiological features of bilateral hereditary micro-epiphyseal dysplasia - a distinct entity in the skeletal dysplasias

    NARCIS (Netherlands)

    Morstert, AK; Dijkstra, PF; van Horn, [No Value; Jansen, BRH; Heutink, P; Lindhout, D

    Aim: To prove that bilateral hereditary micro-epiphyseal dysplasia (BHMED), first described by Elsbach in 1959 [1], is a distinct disorder radiologically as well as clinically, compared with multiple epiphyseal dysplasia (MED). Material and Methods: We used the data of the revised pedigree with 84

  8. Meyer's dysplasia epiphysealis

    International Nuclear Information System (INIS)

    Toro P, Alvaro de Jesus; Lopez C, Meisser A

    2005-01-01

    The skeletal dysplasias are a group of heterogeneous conditions since the clinical and genetic point of view, that to date they are about 200 different disorders. They have as common denominator an inconvenience of the normal process of growth and development of the bony weaving and their cartilaginous precursors. In this article the case of an adolescent of 16 years is presented with Meyer's dysplasia epiphysealis whose diagnostic was delayed and that as opposed to the cases reported in the literature required surgical treatment due to the persistent symptoms, and the literature is reviewed

  9. Limitations of cost-benefit evaluation of non-systematic radiological screening of congenital dislocatable or dislocated hip in Germany

    International Nuclear Information System (INIS)

    Weidtman, V.; Heereman, B. von; Herrmann, F.

    1987-01-01

    Cost-benefit analyses in the field of screening procedures not only comprise economic aspects, but they also require a thorough analysis of possible health risks, which are imminent in these examinations. Many publications on this topic during the past years have proven that even in such a well investigated problem, like the dislocated or dislocatable hip, this can lead to difficulties. We encountered in Germany these questions connected to hip joint screening, when we tried to analyse the data from the German General Screening Program (GGSP). It ws found that 10 times as many children were treated than one would have expected from epidemiological data. In addition a similarly large number of tentative diagnoses was found, which in summary caused great concern amongst the interested social pediatricians. It was to be expected, that the special examinations and treatments resulting from the screening of children had caused considerable and partially useless costs and unnecessary x-ray exposures. Considerable concern was stirred by information from orthopedic clinics, which reported that still cases of hip luxations and hip dysplasias are seen past the half year margin

  10. Two Patients with Severe Short Stature due to a FBN1 Mutation (p.Ala1728Val) with a Mild Form of Acromicric Dysplasia.

    Science.gov (United States)

    de Bruin, Christiaan; Finlayson, Courtney; Funari, Mariana F A; Vasques, Gabriela A; Lucheze Freire, Bruna; Lerario, Antonio M; Andrew, Melissa; Hwa, Vivian; Dauber, Andrew; Jorge, Alexander A L

    2016-01-01

    Acromicric dysplasia (AD) and geleophysic dysplasia 2 (GD2) belong to the category of acromelic dysplasia syndromes, consisting of severe short stature, short hands and feet and skin thickening. Both can result from missense mutations in the transforming growth factor beta 5 domain of the fibrillin-1 gene (FBN1). Two patients (P1 age 10, and P2 age 7) from unrelated families presented to their endocrinologist with severe short stature (approx. -4 SDS). They were otherwise asymptomatic and only had mild facial dysmorphisms. Extensive endocrine work-up did not reveal an underlying etiology. Exome sequencing was performed in each family. Exome sequencing identified the presence of the same heterozygous missense variant c.C5183T (p.Ala1728Val) in the FBN1 gene in both P1 and P2. This variant was previously reported in a patient with GD2 and associated cardiac valvulopathy and hepatomegaly. Detailed clinical re-examination, cardiac and skeletal imaging did not reveal any abnormalities in P1 or P2 other than mild hip dysplasia. This report broadens the phenotypic spectrum of growth disorders associated with FBN1 mutations. Identical mutations give rise to a wide phenotypic spectrum, ranging from isolated short stature to a more classic picture of GD2 with cardiac involvement, distinct facial dysmorphisms and various skeletal anomalies. © 2016 S. Karger AG, Basel.

  11. Stereographic processing of CT figures in the hip joints

    Energy Technology Data Exchange (ETDEWEB)

    Tomihara, Mitsuo (Kinki Univ., Osakasayama, Osaka (Japan). School of Medicine)

    1990-12-01

    CT scan is widely used in orthopedic examinations. By three-dimentional display of the form of bone and joint, the change of form can be identified and the lesion can be located. CT figures were stereographically processed using a personal computer to display the images of the hip joints. The outlines of the bone seen on the CT film were directly traced with a digitizer, then the A/D converted coordinate data were obtained. The data for each slice were input to the personal computer. After conversion to normal coordinate and magnification, the data were filed on the floppy disc. These data were regained from the disk and made affin transformation to display the stereographic images of the hip joints. In a case of solitary bone cyst of the coxal bone, this method helped us to decide the appropriated surgical approach. For the congenital dysplasia of the hip, acetabular coverage was examined three-dimensionally and postoperative changes were assessed in the cases of shelf operation and intertrochanteric extension osteotomy. In the cases of aseptic necrosis of the femoral head, location and size of the necrotic area were clearly visualized and the appropriate surgical procedure could be decided. Moreover, it was useful to decide the degree of rotation of the femoral head in rotational osteotomy. (author) 61 refs.

  12. Spondylometaphyseal dysplasia with hypercalcemia. [Radiological studies

    Energy Technology Data Exchange (ETDEWEB)

    Bagga, A.; Srivastava, R.N.; Gupta, S.; Gupta, A.

    1989-08-01

    Kozlowski's spondylometaphyseal dysplasia is characterised by short-trunk dwarfism, platyspondyly, metaphyseal dysplasia and retarded bone age. We report an association of this syndrome with asymptomatic, hypocalciuric hypercalcemia, a previously undocumented finding. (orig.).

  13. One-stage hip reconstruction in children with cerebral palsy: long-term results at skeletal maturity.

    Science.gov (United States)

    Mallet, Cindy; Ilharreborde, B; Presedo, A; Khairouni, A; Mazda, K; Penneçot, G F

    2014-05-01

    Hip subluxation is common in children with cerebral palsy (CP). Surgery is indicated in case of pain or progressive increase of Reimers index on radiographs. Peri-iliac osteotomy combined with femoral osteotomy is one of the numerous operative techniques available, but results at skeletal maturity remain unclear. The purpose of this radiological study was to report the long-term results of this procedure. Twenty hips in 20 children were retrospectively evaluated at skeletal maturity. Mean age at surgery was 8.1 years and follow-up averaged 9.1 years. All patients underwent Dega acetabuloplasty, soft-tissue release and femoral-shortening varus derotation osteotomy without open reduction. Reimers index, acetabular angle (AA) and neck-shaft angle (NSA) were compared on preoperative, postoperative and latest follow-up radiographs. Dega osteotomy significantly improved the AA and the correction remained stable at maturity. The NSA significantly decreased postoperatively (153°-115°), but recurrence of the valgus deformity (130°) of the proximal femur was observed at maturity. Consequently, Reimers index followed the same evolution. No case of osteonecrosis was reported but one hip dislocated and one subluxated during follow-up. Progressive recurrence of the valgus deformity of the proximal femur, attributable to adductors spasticity and gluteus medius weakness, led to a significant increase in the Reimers index. However, hip coverage remained >70 % at maturity in 90 % of the hips. This one-stage procedure without hip dislocation efficaciously corrected acetabulum dysplasia and successfully treated neurological hips in CP patients. retrospective study.

  14. Hoftedysplasi: klinik, billeddiagnostik og henvisning

    DEFF Research Database (Denmark)

    Troelsen, Anders; Rømer, Lone; Søballe, Kjeld

    2007-01-01

    Hip dysplasia is a common congenital disorder, and identified as a source of pain, functional impairment and increased risk for development of osteoarthritis. Early diagnosis and reference is essential in symptomatic hip dysplasia, since patients may be candidates for joint preserving surgery....... Assessment concentrates on history, physical examination and plain radiography. If radiography confirms the diagnosis (CE-angle hip dysplasia should be well-known to GPs, orthopaedics and radiologists. Udgivelsesdato: 2007...

  15. Hoftedysplasi

    DEFF Research Database (Denmark)

    Troelsen, Anders; Rømer, Lone; Søballe, Kjeld

    2007-01-01

    Hip dysplasia is a common congenital disorder, and identified as a source of pain, functional impairment and increased risk for development of osteoarthritis. Early diagnosis and reference is essential in symptomatic hip dysplasia, since patients may be candidates for joint preserving surgery....... Assessment concentrates on history, physical examination and plain radiography. If radiography confirms the diagnosis (CE-angle hip dysplasia should be well-known to GPs, orthopaedics and radiologists....

  16. How to decrease bronchopulmonary dysplasia in your neonatal intensive care unit today and "tomorrow".

    Science.gov (United States)

    Nelin, Leif D; Bhandari, Vineet

    2017-01-01

    Bronchopulmonary dysplasia, or BPD, is the most common chronic lung disease in infants. Genetic predisposition and developmental vulnerability secondary to antenatal and postnatal infections, compounded with exposure to hyperoxia and invasive mechanical ventilation to an immature lung, result in persistent inflammation, culminating in the characteristic pulmonary phenotype of BPD of impaired alveolarization and dysregulated vascularization. In this article, we highlight specific areas in current management, and speculate on therapeutic strategies that are on the horizon, that we believe will make an impact in decreasing the incidence of BPD in your neonatal intensive care units.

  17. A case report of the fibrous dysplasia

    International Nuclear Information System (INIS)

    You, Dong Soo

    1975-01-01

    The author observed a rare case of fibrous dysplasia in 12 year old female who came to the Infirmary of Dental College, Seoul National University, complaining of facial asymmetry of 3 years' duration in right maxillofacial region. The serial radiograms has been taken, and the nature of the lesion established as a typical fibrous dysplasia according to the interpreted findings in their images. The author has obtained the results as follows: 1. Fibrous dysplasia occurred at 3 years of age in this case. 2. On familial tendency, traumatic history and endocrine disturbances were not noted in this patient. 3. The serial radiograms revealed a typical fibrous dysplasia encroaching right zygomatic bone.

  18. Congenital distal humeral dysplasia: a case report

    International Nuclear Information System (INIS)

    Joseph, Benjamin; Varghese, Renjit A.

    2003-01-01

    Congenital dysplasia of the humerus is very rare. It is characteristically seen in omodysplasia and has also been reported as one of the associated features of Larsen's syndrome. We report a 4-year-old girl with bilateral humero-ulnar dysplasia, with dislocation of the elbows, facial dysmorphism, ball-and-socket ankles and foot deformities. Although the elbow dysplasia is similar to that seen in Larsen's syndrome, other pathognomic features of Larsen's syndrome were absent. The changes seen in the elbows in this patient are also different from those encountered in omodysplasia. We believe that this condition may be a distinct form of skeletal dysplasia hitherto undescribed. (orig.)

  19. A case report of the fibrous dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    You, Dong Soo [Department of Radiology, College of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    1975-11-15

    The author observed a rare case of fibrous dysplasia in 12 year old female who came to the Infirmary of Dental College, Seoul National University, complaining of facial asymmetry of 3 years' duration in right maxillofacial region. The serial radiograms has been taken, and the nature of the lesion established as a typical fibrous dysplasia according to the interpreted findings in their images. The author has obtained the results as follows: 1. Fibrous dysplasia occurred at 3 years of age in this case. 2. On familial tendency, traumatic history and endocrine disturbances were not noted in this patient. 3. The serial radiograms revealed a typical fibrous dysplasia encroaching right zygomatic bone.

  20. Dentomaxillofacial characteristics of ectodermal dysplasia.

    Science.gov (United States)

    Nakayama, Yumiko; Baba, Yoshiyuki; Tsuji, Michiko; Fukuoka, Hiroki; Ogawa, Takuya; Ohkuma, Mizue; Moriyama, Keiji

    2015-02-01

    The aim of this retrospective hospital-based study was to elucidate the dentomaxillofacial characteristics of ectodermal dysplasia. Six Japanese individuals (one male and five female; age range, 12.7-27.2 years) underwent comprehensive examinations, including history recording, cephalometric analysis, panoramic radiography, and analysis of dental models. All the subjects had two or more major manifestations for clinical diagnosis of ectodermal dysplasia (e.g., defects of hair, teeth, nails, and sweat glands). They presented hypodontia (mean number of missing teeth, 9.5; range, 5-14), especially in the premolar region, and enamel dysplasia. Five subjects had bilateral molar occlusion, whereas one subject had unilateral molar occlusion. The common skeletal features were small facial height, maxillary hypoplasia, counterclockwise rotation of the mandible, and mandibular protrusion. Interestingly, the maxillary first molars were located in higher positions and the upper anterior facial height was smaller than the Japanese norm. The results suggest that vertical and anteroposterior maxillary growth retardation, rather than lack of occlusal support due to hypodontia, leads to reduced anterior facial height in individuals with ectodermal dysplasia. © 2014 Japanese Teratology Society.

  1. Chondroectodermal dysplasia: a rare syndrome.

    Directory of Open Access Journals (Sweden)

    Dana Tahririan

    2014-06-01

    Full Text Available Chondroectodermal dysplasia (Ellis-Van Creveld syndrome is a rare autosomal recessive congenital abnormality. This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures. The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers. Clinical reports outline the classical and unusual oral and dental manifestations, which help health care providers diagnose chondroectodermal dysplasia, and refer patients with this syndrome to appropriate health care professionals to receive treatment to prevent further cardiac complications and bone deformities.

  2. Generation and characterization of function-blocking anti-ectodysplasin A (EDA) monoclonal antibodies that induce ectodermal dysplasia.

    Science.gov (United States)

    Kowalczyk-Quintas, Christine; Willen, Laure; Dang, Anh Thu; Sarrasin, Heidi; Tardivel, Aubry; Hermes, Katharina; Schneider, Holm; Gaide, Olivier; Donzé, Olivier; Kirby, Neil; Headon, Denis J; Schneider, Pascal

    2014-02-14

    Development of ectodermal appendages, such as hair, teeth, sweat glands, sebaceous glands, and mammary glands, requires the action of the TNF family ligand ectodysplasin A (EDA). Mutations of the X-linked EDA gene cause reduction or absence of many ectodermal appendages and have been identified as a cause of ectodermal dysplasia in humans, mice, dogs, and cattle. We have generated blocking antibodies, raised in Eda-deficient mice, against the conserved, receptor-binding domain of EDA. These antibodies recognize epitopes overlapping the receptor-binding site and prevent EDA from binding and activating EDAR at close to stoichiometric ratios in in vitro binding and activity assays. The antibodies block EDA1 and EDA2 of both mammalian and avian origin and, in vivo, suppress the ability of recombinant Fc-EDA1 to rescue ectodermal dysplasia in Eda-deficient Tabby mice. Moreover, administration of EDA blocking antibodies to pregnant wild type mice induced in developing wild type fetuses a marked and permanent ectodermal dysplasia. These function-blocking anti-EDA antibodies with wide cross-species reactivity will enable study of the developmental and postdevelopmental roles of EDA in a variety of organisms and open the route to therapeutic intervention in conditions in which EDA may be implicated.

  3. Generation and Characterization of Function-blocking Anti-ectodysplasin A (EDA) Monoclonal Antibodies That Induce Ectodermal Dysplasia*

    Science.gov (United States)

    Kowalczyk-Quintas, Christine; Willen, Laure; Dang, Anh Thu; Sarrasin, Heidi; Tardivel, Aubry; Hermes, Katharina; Schneider, Holm; Gaide, Olivier; Donzé, Olivier; Kirby, Neil; Headon, Denis J.; Schneider, Pascal

    2014-01-01

    Development of ectodermal appendages, such as hair, teeth, sweat glands, sebaceous glands, and mammary glands, requires the action of the TNF family ligand ectodysplasin A (EDA). Mutations of the X-linked EDA gene cause reduction or absence of many ectodermal appendages and have been identified as a cause of ectodermal dysplasia in humans, mice, dogs, and cattle. We have generated blocking antibodies, raised in Eda-deficient mice, against the conserved, receptor-binding domain of EDA. These antibodies recognize epitopes overlapping the receptor-binding site and prevent EDA from binding and activating EDAR at close to stoichiometric ratios in in vitro binding and activity assays. The antibodies block EDA1 and EDA2 of both mammalian and avian origin and, in vivo, suppress the ability of recombinant Fc-EDA1 to rescue ectodermal dysplasia in Eda-deficient Tabby mice. Moreover, administration of EDA blocking antibodies to pregnant wild type mice induced in developing wild type fetuses a marked and permanent ectodermal dysplasia. These function-blocking anti-EDA antibodies with wide cross-species reactivity will enable study of the developmental and postdevelopmental roles of EDA in a variety of organisms and open the route to therapeutic intervention in conditions in which EDA may be implicated. PMID:24391090

  4. CT Imaging of Craniofacial Fibrous Dysplasia

    Directory of Open Access Journals (Sweden)

    Zerrin Unal Erzurumlu

    2015-01-01

    Full Text Available Fibrous dysplasia is a benign fibroosseous bone dysplasia that can involve single (monostotic or multiple (polyostotic bones. Monostotic form is more frequent in the jaws. It is termed as craniofacial fibrous dysplasia, when it involves, though rarely, adjacent craniofacial bones. A 16-year-old girl consulted for a painless swelling in the right posterior mandible for two years. Panoramic radiography revealed ground-glass ill-defined lesions in the three different regions of the maxilla and mandible. Axial CT scan (bone window showed multiple lesions involving skull base and facial bones. Despite lesions in the skull base, the patient had no abnormal neurological findings. The lesion was diagnosed as fibrous dysplasia based on radiological and histopathological examination. In this paper, CT findings and differential diagnosis of CFD are discussed. CT is a useful imaging technique for CFD cases.

  5. Ectodermal dysplasia: otolaryngologic evaluation of 23 cases.

    Science.gov (United States)

    Yildirim, Muzeyyen; Yorgancilar, Ediz; Gun, Ramazan; Topcu, Ismail

    2012-02-01

    The aim of this prospective study was to improve the quality of life of and reduce morbidity for patients with ectodermal dysplasia by assessing their actual and potential ENT pathologies, and offering methods of prevention and treatment. The study was conducted between 2006 and 2008 and included 23 patients diagnosed with ectodermal dysplasia. The major symptoms of ectodermal dysplasia were evaluated. Patient histories were obtained in all cases, and a complete head and neck examination was carried out. Of the 23 patients (11 males and 12 females, aged 5 to 45 years) diagnosed with ectodermal dysplasia, 22 had hypohidrotic ectodermal dysplasia and 1 had ectrodactyly-ectodermal dysplasia-clefting syndrome. In all patients diagnosed with hypohidrotic ectodermal dysplasia, the salivary glands were examined by ultrasonography and, when necessary, by scintigraphy. Hearing defects in patients with otologic problems were determined by audiometric examination: 39.1% of the patients had hearing loss, 43.5% had otitis media, and 39.1% had impacted cerumen. The most common rhinologic findings were saddle nose deformity in 56.5%, nasal obstruction and nasal dryness (52.2% each), and chronic rhinitis/rhinosinusitis (34.8%). The most common oral and oropharyngeal findings were difficulty chewing in 82.6% and dry mouth in 78.3%. All 23 patients had required dental work. Because this disorder affects several aspects of the body, its treatment requires a multidisciplinary approach, with the otolaryngologist being a vital part of the management team.

  6. Cleidocranial Dysplasia: Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Avinash Kshar

    2010-01-01

    Full Text Available Cleidocranial dysplasia constitutes a congenital disorder manifested primarily in the development of facial and cranial bones, as well as partial development or complete absence of the clavicles and problems also arise on the number and eruption of teeth. It is a rare disease with autosomal dominant but 40% cases represented spontaneous mutations. This disease has no sex predilection. It is characterized by a generalized skeletal dysplasia. 1 In this article we are reporting two cases of cleidocranial dysplasia in son and father with characteristic clinical and radiographic features.

  7. Ectodermal dysplasia (ED) syndrome.

    Science.gov (United States)

    Chee, Siew-Yin; Wanga, Chung-Hsing; Lina, Wei-De; Tsaia, Fuu-Jen

    2014-01-01

    Ectodermal dysplasia (ED) syndrome comprises a large, heterogeneous group of inherited disorders that are defined by primary defects in the development of 2 or more tissues derived from the embryonic ectoderm. The tissues primarily involved are the skin and its appendages (including hair follicles, eccrine glands, sebaceous glands, nails) and teeth. The clinical features include sparse hair, abnormal or missing teeth, and an inability to sweat due to lack of sweat glands. One such case report of ectodermal dysplasia is presented here.

  8. Mondini Dysplasia Presenting as Otorrhea without Meningitis

    OpenAIRE

    Chien-Yu Lin; Hung-Ching Lin; Chun-Chih Peng; Kuo-Sheng Lee; Nan-Chang Chiu

    2012-01-01

    Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus preventing morbidity and neurologic sequelae. Hearing impairment may be the only manifestation of Mondini dysplasia, and the benefit of hearing screening is emp...

  9. Computed tomography measurements of the acetabulum in adult dysplastic hips: Which level is appropriate

    Energy Technology Data Exchange (ETDEWEB)

    Anda, S.; Kvistad, K.A. (Dept. of Radiology, Trondheim Univ. (Norway)); Terjesen, T. (Dept. of Orthopedic Surgery, Trondheim Univ. (Norway))

    1991-05-01

    A study has been performed to evaluate whether one or several levels are needed with comuted tomography (CT) study to provide sufficient information regarding anteversion and acetabular support to the femoral head. A total of 23 hips in 14 adults with uni- or bilateral congenital hip dysplasia (center-edge angle less than 20deg) were assessed by obtaining 5-mm contiguous CT slices and performing acetabular measurements at four levels. Both anterior and posterior acetabular supports as quantified by the anterior and posterior acetabular sector angles were significantly lower than normal at all levels. The sector angles increased in the proximal cuts, whereas the acetabular anteversin increased caudally. Because no important additional information was gained by measuring at different levels, we conclude that CT study at one level is sufficient for acetabular measurements and suggest that the slice through the center of the femoral head is the most appropriate one. (orig.).

  10. Kidney Dysplasia

    Science.gov (United States)

    ... whose mothers used certain prescription medications or illegal drugs during pregnancy What are the signs of kidney dysplasia? Many ... the use of certain prescription medications or illegal drugs during pregnancy. Pregnant women should talk with their health care ...

  11. [Mid-term effectiveness of total hip arthroplasty with collum femoris preserving prosthesis].

    Science.gov (United States)

    Li, Mingqing; Hu, Yihe; Li, Kanghua; Liao, Qiande; Wen, Ting; Zhong, Da

    2012-08-01

    To discuss the clinical application of total hip arthroplasty (THA) with collum femoris preserving (CFP) prosthesis and to analyze the mid-term effectiveness. Between January 2004 and February 2007, 45 patients (48 hips) underwent THA with CFP prosthesis. There were 29 males (31 hips) and 16 females (17 hips) with an average age of 48.8 years (range, 38-60 years), including 20 left hips, 22 right hips, and 3 bilateral hips. The causes of hip replacement were osteoarthritis (20 cases), avascular necrosis of femoral head (13 cases), dysplasia (4 cases), rheumatoid arthritis (3 cases), posttraumatic osteoarthritis (2 cases), ankylosing spondylitis (2 cases), and Perths disease (1 case). The average disease duration was 6.1 years (range, 2-13 years). Harris scores, visual analogue scale (VAS) score, and the hip range of motion (ROM) were recorded at pre- and post-operation. The X-ray films were taken at pre- and post-operation to observe the position, loosening of the prosthesis, and ectopic ossification. The gait of patients were also evaluated during follow-up. Short-form 36 health survey scale (SF-36) was used to evaluate the life quality of patients. All 45 patients were followed up 5-8 years with an average of 6.4 years. All the incisions healed by first intention. No infection, hip dislocation, nerve injury, or deep vein thrombosis occurred. Six cleavage fractures (13.3%) of the lateral femoral diaphysis at the distal prosthesis occurred during operation, which healed at 8 months postoperatively without any treatment. Mild ectopic ossification occurred in 4 patients (8.9%) who had no discomfort. Five patients (11.1%) had bone mineral density loss in the region of the proximal femur. The survival rates of the cups and stems were all 100% at last follow-up. The results of Harris score, VAS score, and ROM of the hip joint at 1 year postoperatively and last follow-up were significantly better than preoperative ones (P 0.05) except the Harris score (P fair in 6 hips

  12. Knee radiography in the diagnosis of skeletal dysplasias

    International Nuclear Information System (INIS)

    Kwee, Thomas C.; Beek, Frederik J.A.; Nievelstein, Rutger A.J.; Beemer, Frits A.

    2006-01-01

    Flattening of the epiphyses of long bones is seen in several skeletal dysplasias and standardized measurements on a radiograph of the knee to detect skeletal dysplasias using this feature have been described. Since then only two other studies in which this method was used have been published, and both included only a small number of children and neither had a control group. In addition, the Dutch National Working Group on Skeletal Dysplasias began to have doubts about the reliability of the method. We therefore decided to re-evaluate its accuracy in a population of children with and without a skeletal dysplasia. To determine the diagnostic value of standardized measurements on conventional AP radiographs of the knee in children with a skeletal dysplasia. Subjects and methods: We measured the distal femoral metaphysis and epiphysis according to the published method on conventional AP radiographs of the knee in 45 healthy children and 52 children with a skeletal dysplasia. We compared graphically the height of the distal femoral epiphysis with its width and with the width of the femoral metaphysis. Receiver operating characteristic (ROC) curves were calculated for each group of children. All graphs showed a considerable overlap between children with a skeletal dysplasia and healthy children. The size of the area under the ROC curves for the different groups was small, varying between 0.567 and 0.653. This method does not discriminate between children with a skeletal dysplasia and healthy children. We therefore consider it to be of little diagnostic value. (orig.)

  13. Analysis of the acetabulum by CT scan in Japanese with osteoarthritis of the hip

    International Nuclear Information System (INIS)

    Ishibashi, Masanori

    1997-01-01

    The morphology of the acetabulum was analyzed by CT scan in 66 Japanese patients with osteoarthritis of the hip mainly following congenital dislocation or dysplasia. The CT scan data was analyzed to standardize the pelvic inclination in all directions. In these patients, the acetabular floor had thickened and the acetabulum was located in the anterolateral portion. The bilateral difference in the acetabular anteversion angle was not different between patients and normal individuals, but the acetabular sector angle in patients was smaller than in the normal subjects. As the stage of osteoarthritis of the hip advanced, the thickness of the acetabular floor in the center and posterior portions and the anterior sector angle increased, and the lateralization of the acetabulum advanced, but the anteversion angle decreased. A bone-forming change is characteristic of the anterior acetabulum and acetabular fossa in the center and posterior portions in Japanese patients with osteoarthritis of the hip. In comparison with data obtained by standard radiography, these changes detected by CT scan corresponded to an increase of the width of a tear drop, the lateralization of the femoral head and the lack of the support of the femoral head from the acetabulum. (author)

  14. Analysis of the acetabulum by CT scan in Japanese with osteoarthritis of the hip

    Energy Technology Data Exchange (ETDEWEB)

    Ishibashi, Masanori [Keio Univ., Tokyo (Japan). School of Medicine

    1997-11-01

    The morphology of the acetabulum was analyzed by CT scan in 66 Japanese patients with osteoarthritis of the hip mainly following congenital dislocation or dysplasia. The CT scan data was analyzed to standardize the pelvic inclination in all directions. In these patients, the acetabular floor had thickened and the acetabulum was located in the anterolateral portion. The bilateral difference in the acetabular anteversion angle was not different between patients and normal individuals, but the acetabular sector angle in patients was smaller than in the normal subjects. As the stage of osteoarthritis of the hip advanced, the thickness of the acetabular floor in the center and posterior portions and the anterior sector angle increased, and the lateralization of the acetabulum advanced, but the anteversion angle decreased. A bone-forming change is characteristic of the anterior acetabulum and acetabular fossa in the center and posterior portions in Japanese patients with osteoarthritis of the hip. In comparison with data obtained by standard radiography, these changes detected by CT scan corresponded to an increase of the width of a tear drop, the lateralization of the femoral head and the lack of the support of the femoral head from the acetabulum. (author)

  15. Accuracy of Cup Positioning With the Computed Tomography-Based Two-dimensional to Three-Dimensional Matched Navigation System: A Prospective, Randomized Controlled Study.

    Science.gov (United States)

    Yamada, Kazuki; Endo, Hirosuke; Tetsunaga, Tomonori; Miyake, Takamasa; Sanki, Tomoaki; Ozaki, Toshifumi

    2018-01-01

    The accuracy of various navigation systems used for total hip arthroplasty has been described, but no publications reported the accuracy of cup orientation in computed tomography (CT)-based 2D-3D (two-dimensional to three-dimensional) matched navigation. In a prospective, randomized controlled study, 80 hips including 44 with developmental dysplasia of the hips were divided into a CT-based 2D-3D matched navigation group (2D-3D group) and a paired-point matched navigation group (PPM group). The accuracy of cup orientation (absolute difference between the intraoperative record and the postoperative measurement) was compared between groups. Additionally, multiple logistic regression analysis was performed to evaluate patient factors affecting the accuracy of cup orientation in each navigation. The accuracy of cup inclination was 2.5° ± 2.2° in the 2D-3D group and 4.6° ± 3.3° in the PPM group (P = .0016). The accuracy of cup anteversion was 2.3° ± 1.7° in the 2D-3D group and 4.4° ± 3.3° in the PPM group (P = .0009). In the PPM group, the presence of roof osteophytes decreased the accuracy of cup inclination (odds ratio 8.27, P = .0140) and the absolute value of pelvic tilt had a negative influence on the accuracy of cup anteversion (odds ratio 1.27, P = .0222). In the 2D-3D group, patient factors had no effect on the accuracy of cup orientation. The accuracy of cup positioning in CT-based 2D-3D matched navigation was better than in paired-point matched navigation, and was not affected by patient factors. It is a useful system for even severely deformed pelvises such as developmental dysplasia of the hips. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. An Uncommon Reason of Osteoporosis: Spondyloepiphyseal Dysplasia Congenita

    Directory of Open Access Journals (Sweden)

    Onur Elbasan

    2017-12-01

    Full Text Available Skeletal dysplasia is a complex and rare disease group that presents with clinical and radiological findings that differ from classical metabolic bone diseases in which bone and cartilage tissue are affected together. Spondyloepiphyseal dysplasia conjenita with involvement of the spine and long bone epiphyses is seen with short stature and short body from birth. Although bone deformities such as shortness of neck and vertebrae, kyphosis, scoliosis, pectus carinatum, genu varum or valgum are frequent, association with osteopenia/osteoporosis has been rarely reported. Although spondyloepiphyseal dysplasia tarda and osteopenia are coexisting in the literature, there is no evidence of the association of spondyloepiphyseal dysplasia tarda and conjunctiva with osteoporosis. In our case report, we presented a patient who was applied to our center with short stature, diagnosed with osteoporosis and spondyloepiphyseal dysplasia congenita by detecting femur head aplasia in radiological imaging.

  17. [Florid cemento-osseous dysplasia of the jaws].

    Science.gov (United States)

    Benazzou, S; Boulaadas, M; El Ayoubi, A; Nazih, N; Essakalli, L; Kzadri, M

    2011-06-01

    Florid cemento-osseous dysplasia is a benign and rare tumor of the jaws. It is more commonly seen in middle-aged black women. Most cases are asymptomatic and are found during routine radiographic examination. We report two complicated cases of florid cemento-osseous dysplasia, one with facial deformity and the other with chronic osteitis. The diagnosis of florid cemento-osseous dysplasia is based on clinical and radiological features. The lesions are commonly bilateral and symmetrical. Copyright © 2011. Published by Elsevier Masson SAS.

  18. Tibial lengthening for unilateral Crowe type-IV developmental dysplasia of the hip

    Directory of Open Access Journals (Sweden)

    Jun Wan

    2014-01-01

    Conclusions: Tibial lengthening may effectively correct gait and satisfactorily improve body image in young patients with unilateral Crowe type-IV DDH. Mono-lateral external fixator allows for accelerated postoperative rehabilitation and optimal preservation of ankle movements. Lengthening along with intramedullary nails may significantly reduce the external fixation time and the risk of fixator-related complications.

  19. Hypohidrotic and hidrotic ectodermal dysplasia: a report of two cases.

    Science.gov (United States)

    Vasconcelos Carvalho, Marianne; Romero Souto de Sousa, José; Paiva Correa de Melo, Filipe; Fonseca Faro, Tatiane; Nunes Santos, Ana Clara; Carvalho, Silvia; Veras Sobral, Ana Paula

    2013-07-14

    Ectodermal dysplasias are a large group of syndromes characterized by anomalies in the structures of ectodermal origin. There are 2 major types of this disorder, based on clinical findings: hypohidrotic ectodermal dysplasia and hidrotic ectodermal dysplasia. This clinical classification is very important because clinical professionals involved with this disease need first a clear and practical method of diagnosis. The main oral manifestation of ectodermal dysplasia may be expressed as hypodontia. Thus, dental professionals may be the first to diagnose ectodermal dysplasia. The present article reports one case of each of the main types (hypohidrotic and hidrotic) of ectodermal dysplasia and the authors review the literature regarding the pathogenesis, clinical features, and therapeutic management of this condition.

  20. Imaging of the hip joint. Computed tomography versus magnetic resonance imaging

    Science.gov (United States)

    Lang, P.; Genant, H. K.; Jergesen, H. E.; Murray, W. R.

    1992-01-01

    The authors reviewed the applications and limitations of computed tomography (CT) and magnetic resonance (MR) imaging in the assessment of the most common hip disorders. Magnetic resonance imaging is the most sensitive technique in detecting osteonecrosis of the femoral head. Magnetic resonance reflects the histologic changes associated with osteonecrosis very well, which may ultimately help to improve staging. Computed tomography can more accurately identify subchondral fractures than MR imaging and thus remains important for staging. In congenital dysplasia of the hip, the position of the nonossified femoral head in children less than six months of age can only be inferred by indirect signs on CT. Magnetic resonance imaging demonstrates the cartilaginous femoral head directly without ionizing radiation. Computed tomography remains the imaging modality of choice for evaluating fractures of the hip joint. In some patients, MR imaging demonstrates the fracture even when it is not apparent on radiography. In neoplasm, CT provides better assessment of calcification, ossification, and periosteal reaction than MR imaging. Magnetic resonance imaging, however, represents the most accurate imaging modality for evaluating intramedullary and soft-tissue extent of the tumor and identifying involvement of neurovascular bundles. Magnetic resonance imaging can also be used to monitor response to chemotherapy. In osteoarthrosis and rheumatoid arthritis of the hip, both CT and MR provide more detailed assessment of the severity of disease than conventional radiography because of their tomographic nature. Magnetic resonance imaging is unique in evaluating cartilage degeneration and loss, and in demonstrating soft-tissue alterations such as inflammatory synovial proliferation.

  1. [Cochleovestibular dysplasia: a case report].

    Science.gov (United States)

    Darbi, A; Cenoui, F; Atmane, A; Amil, T; Hanine, A

    2010-04-01

    Cochleovestibular dysplasia or Mondini malformation is exceptional. Cochlear dysplasia is due to early cessation of the development of the inner ear during embryonic life. We report on the case of an infant who presented with perception deafness and repeated meningitis. CT allowed the diagnosis of Mondini malformation. We underline the importance of high-resolution CT in the diagnosis of Mondini malformation. Copyright 2010 Elsevier Masson SAS. All rights reserved.

  2. Müllerian agenesis with hypohidrotic ectodermal dysplasia syndrome.

    Science.gov (United States)

    Whaley, Katie; Winter, Jordan; Eyster, Kathleen M; Hansen, Keith A

    2012-04-01

    To describe the association of müllerian agenesis with hypohidrotic ectodermal dysplasia. Case report. University medical center. A 17-year-old woman with hypohidrotic ectodermal dysplasia referred for evaluation of primary amenorrhea. History, physical examination, and ultrasound. Physical findings of these two syndromes. Physical examination and ultrasound demonstrated müllerian agenesis with findings of hypohidrotic ectodermal dysplasia. This is the first description of the association of müllerian agenesis with ectodermal dysplasia. This rare case might provide further insight into the development of the uterus and the ectoderm as well as its derivatives. Copyright © 2012 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

  3. [Mondini dysplasia: traumatic cerebrospinal fluid otorrhea with meningitis].

    Science.gov (United States)

    Kaftan, H; Adamaszek, M; Hosemann, W

    2006-08-01

    Mondini dysplasia is a rare malformation of the inner ear commonly associated with loss of hearing and vestibular function. Children with Mondini dysplasia are predisposed to developing a spontaneous cerebrospinal fluid (CSF) leak and recurrent meningitis. If there is no CSF leak but a unilateral hearing loss, the condition may go undiagnosed for years. We describe a 65-year-old man with unrecognized unilateral Mondini dysplasia who presented with CSF leak and meningoencephalitis after minor head trauma. Two operative interventions were undertaken to close the defect properly. Patients with Mondini dysplasia or their parents should be cautioned about the potential hazards of any head trauma.

  4. Focal cemento-osseous dysplasia: review and a case report.

    Science.gov (United States)

    Salem, Y M Y; Osman, Y I; Norval, E J G

    2010-10-01

    Focal cemento-osseous dysplasia is a benign fibro-osseous condition that can be seen in dentate and edentulous patients. It is an asymptomatic lesion and needs no treatment; however follow-up is essential due to the possibility that focal cemento-osseous dysplasia can progress to a condition called florid osseous dysplasia that involves multiple sites. A case report is presented here, along with a review of the differential diagnoses considered in order to reach a final diagnosis of focal cemento-osseous dysplasia.

  5. Anhidrotic ectodermal dysplasia presenting as atrophic rhinitis.

    Science.gov (United States)

    Barman, Debasis; Mandal, Satadal; Nandi, Santanu; Banerjee, Pranabashish; Rashid, M A

    2011-11-01

    Ectodermal dysplasia is a complex group of familial disorders with numerous clinical characteristics, with an incidence of 7 in 10000 born alive children. Ectodermal dysplasia affects structures of ectodermal origin like the skin and its appendages as well as other non-ectodermal structures. The most common sites of involvement are the defects in the skin, hair, teeth, nails and sweat glands,which are of ectodermal origin. Though the dermatologists and paediatricians often manage such cases, we report one case of ectodermal dysplasia presenting with atrophic rhinitis.

  6. Expanding the phenome and variome of skeletal dysplasia.

    Science.gov (United States)

    Maddirevula, Sateesh; Alsahli, Saud; Alhabeeb, Lamees; Patel, Nisha; Alzahrani, Fatema; Shamseldin, Hanan E; Anazi, Shams; Ewida, Nour; Alsaif, Hessa S; Mohamed, Jawahir Y; Alazami, Anas M; Ibrahim, Niema; Abdulwahab, Firdous; Hashem, Mais; Abouelhoda, Mohamed; Monies, Dorota; Al Tassan, Nada; Alshammari, Muneera; Alsagheir, Afaf; Seidahmed, Mohammed Zain; Sogati, Samira; Aglan, Mona S; Hamad, Muddathir H; Salih, Mustafa A; Hamed, Ahlam A; Alhashmi, Nadia; Nabil, Amira; Alfadli, Fatima; Abdel-Salam, Ghada M H; Alkuraya, Hisham; Peitee, Winnie Ong; Keng, W T; Qasem, Abdullah; Mushiba, Aziza M; Zaki, Maha S; Fassad, Mahmoud R; Alfadhel, Majid; Alexander, Saji; Sabr, Yasser; Temtamy, Samia; Ekbote, Alka V; Ismail, Samira; Hosny, Gamal Ahmed; Otaify, Ghada A; Amr, Khalda; Al Tala, Saeed; Khan, Arif O; Rizk, Tamer; Alaqeel, Aida; Alsiddiky, Abdulmonem; Singh, Ankur; Kapoor, Seema; Alhashem, Amal; Faqeih, Eissa; Shaheen, Ranad; Alkuraya, Fowzan S

    2018-04-05

    PurposeTo describe our experience with a large cohort (411 patients from 288 families) of various forms of skeletal dysplasia who were molecularly characterized.MethodsDetailed phenotyping and next-generation sequencing (panel and exome).ResultsOur analysis revealed 224 pathogenic/likely pathogenic variants (54 (24%) of which are novel) in 123 genes with established or tentative links to skeletal dysplasia. In addition, we propose 5 genes as candidate disease genes with suggestive biological links (WNT3A, SUCO, RIN1, DIP2C, and PAN2). Phenotypically, we note that our cohort spans 36 established phenotypic categories by the International Skeletal Dysplasia Nosology, as well as 18 novel skeletal dysplasia phenotypes that could not be classified under these categories, e.g., the novel C3orf17-related skeletal dysplasia. We also describe novel phenotypic aspects of well-known disease genes, e.g., PGAP3-related Toriello-Carey syndrome-like phenotype. We note a strong founder effect for many genes in our cohort, which allowed us to calculate a minimum disease burden for the autosomal recessive forms of skeletal dysplasia in our population (7.16E-04), which is much higher than the global average.ConclusionBy expanding the phenotypic, allelic, and locus heterogeneity of skeletal dysplasia in humans, we hope our study will improve the diagnostic rate of patients with these conditions.GENETICS in MEDICINE advance online publication, 5 April 2018; doi:10.1038/gim.2018.50.

  7. Renal dysplasia and MRI: a clinician's perspective

    Energy Technology Data Exchange (ETDEWEB)

    Greenbaum, Larry A. [Emory University, Division of Pediatric Nephrology, Children' s Healthcare of Atlanta, Atlanta, GA (United States)

    2008-01-15

    Renal dysplasia is a common abnormality in children. The role of MRI in evaluating children with renal dysplasia is evolving. More information is clearly necessary before MRI replaces conventional imaging modalities. In order to appropriately use MRI, the radiologist must have an understanding of the clinical questions that are important in the management of children with renal dysplasia. This review provides background information on renal dysplasia for the pediatric radiologist. The focus is on unilateral disease, especially multicystic dysplastic kidneys, and bilateral dysplasia, which is the most common cause of kidney failure in children. The emphasis is on the important clinical issues, and the potential of MRI as a methodology for providing clinically useful information not otherwise available from other imaging modalities. (orig.)

  8. Mondini Dysplasia Presenting as Otorrhea without Meningitis

    Directory of Open Access Journals (Sweden)

    Chien-Yu Lin

    2012-12-01

    Full Text Available Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus preventing morbidity and neurologic sequelae. Hearing impairment may be the only manifestation of Mondini dysplasia, and the benefit of hearing screening is emphasized. Temporal bone computed tomography should be considered in children with unilateral sensorineural or mixed-type hearing impairment.

  9. Malignant transformation of fibrous dysplasia into chondroblastic osteosarcoma

    International Nuclear Information System (INIS)

    Kaushik, Shaifali; Smoker, Wendy R.K.; Frable, William J.

    2002-01-01

    A case of malignant transformation of polyostotic fibrous dysplasia into maxillary chondroblastic osteosarcoma is presented. The clinical, radiographic, CT, MR imaging features and pathological findings of polyostotic fibrous dysplasia and its malignant transformation are described. Malignant transformation of fibrous dysplasia is rare and has not previously been described in the English literature in this location in McCune-Albright syndrome and in the absence of radiation treatment. (orig.)

  10. Three-dimensional delayed gadolinium-enhanced magnetic resonance imaging of hip joint cartilage at 3 T: A prospective controlled study

    Energy Technology Data Exchange (ETDEWEB)

    Zilkens, Christoph, E-mail: christoph.zilkens@med.uni-duesseldorf.de [Univ. Dusseldorf, Medical Faculty, Department of Orthopaedic Surgery, Moorenstrasse 5, D-40225 Dusseldorf (Germany); Miese, Falk, E-mail: falk.miese@med.uni-duesseldorf.de [Univ. Dusseldorf, Medical Faculty, Department of Diagnostic and Interventional Radiology, Moorenstrasse 5, D-40225 Dusseldorf (Germany); Kim, Young-Jo, E-mail: young-jo.kim@childrens.harvard.edu [Department of Orthopaedic Surgery, The Children' s Hospital Boston, 300 Longwood Ave., Boston, MA 02115 (United States); Hosalkar, Harish, E-mail: hhosalkar@rchsd.org [Department of Orthopaedic Surgery, Rady Children' s Hospital San Diego, 3030 Childrens Way Ste 410, San Diego, CA 92123 (United States); Antoch, Gerald, E-mail: antoch@med.uni-duesseldorf.de [Univ. Dusseldorf, Medical Faculty, Department of Diagnostic and Interventional Radiology, Moorenstrasse 5, D-40225 Dusseldorf (Germany); Krauspe, Ruediger, E-mail: krauspe@med.uni-duesseldorf.de [Univ. Dusseldorf, Medical Faculty, Department of Orthopaedic Surgery, Moorenstrasse 5, D-40225 Dusseldorf (Germany); Bittersohl, Bernd, E-mail: bbittersohl@partners.org [Univ. Dusseldorf, Medical Faculty, Department of Orthopaedic Surgery, Moorenstrasse 5, D-40225 Dusseldorf (Germany)

    2012-11-15

    Purpose: To assess acetabular and femoral hip joint cartilage with three-dimensional (3D) delayed gadolinium-enhanced magnetic resonance imaging (dGEMRIC) in patients with degeneration of hip joint cartilage and asymptomatic controls with morphologically normal appearing cartilage. Methods and materials: A total of 40 symptomatic patients (18 males, 22 females; mean age: 32.8 {+-} 10.2 years, range: 18-57 years) with different hip joint deformities including femoroacetabular impingement (n = 35), residual hip dysplasia (n = 3) and coxa magna due to Legg-Calve-Perthes disease in childhood (n = 2) underwent high-resolution 3D dGEMRIC for the evaluation of acetabular and femoral hip joint cartilage. Thirty-one asymptomatic healthy volunteers (12 males, 19 females; mean age: 24.5 {+-} 1.8 years, range: 21-29 years) without underlying hip deformities were included as control. MRI was performed at 3 T using a body matrix phased array coil. Region of interest (ROI) analyses for T1{sub Gd} assessment was performed in seven regions in the hip joint, including anterior to superior and posterior regions. Results: T1{sub Gd} mapping demonstrated the typical pattern of acetabular cartilage consistent with a higher glycosaminoglycan (GAG) content in the main weight-bearing area. T1{sub Gd} values were significantly higher in the control group than in the patient group whereas significant differences in T1{sub Gd} values corresponding to the amount of cartilage damage were noted both in the patient group and in the control group. Conclusions: Our study demonstrates the potential of high-resolution 3D dGEMRIC at 3 T for separate acetabular and femoral hip joint cartilage assessment in various forms of hip joint deformities.

  11. Frontofacionasal Dysplasia

    African Journals Online (AJOL)

    rme

    226. Frontofacionasal Dysplasia: Another Observation eye. Other ocular features include small eyes, iris colobomas and cataracts. A frontally situated lipoma, causing a swelling at the nasion, was reported in one patient and an encephalocele has been described.3. Mental retardation has been reported in one patient with.

  12. Dental Perspectives in Fibrous Dysplasia and McCune-Albright Syndrome

    Science.gov (United States)

    Akintoye, Sunday O.; Boyce, Alison M.; Collins, Michael T.

    2013-01-01

    McCune-Albright syndrome (MAS) is a rare multisystem disorder characterized by the triad of polyostotic fibrous dysplasia (PFD), endocrine disorders and café-au-lait skin pigmentation. Ninety percent of MAS patients have FD lesions in the craniofacial area, resulting in significant orofacial deformity, dental disorders, bone pain and compromised oral health. Maxillo-mandibular FD is also associated with dental developmental disorders, malocclusion, and high caries index. There is limited data on the outcomes of dental treatments in maxillo-mandibular FD/MAS patients, because clinicians and researchers have limited access to patients, and there are concerns that dental surgery may activate quiescent jaw FD lesions to grow aggressively. This report highlights current perspectives on dental management issues associated with maxillo-mandibular FD within the context of MAS. PMID:23953425

  13. Fibrous dysplasia of the femoral neck

    International Nuclear Information System (INIS)

    Savage, P.E.; Stoker, D.J.

    1984-01-01

    Fibrous dysplasia of the femur is usually observed in the intertrochanteric region. It is rarely confined to the femoral neck. We present four cases illustrating the radiographic appearance and spectrum of this condition which all showed the relatively lucent variety of fibrous dysplasia with varying degrees of expansion and surrounding sclerosis. The natural history of this condition is discussed. (orig.)

  14. Mondini dysplasia presenting as otorrhea without meningitis.

    Science.gov (United States)

    Lin, Chien-Yu; Lin, Hung-Ching; Peng, Chun-Chih; Lee, Kuo-Sheng; Chiu, Nan-Chang

    2012-12-01

    Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus preventing morbidity and neurologic sequelae. Hearing impairment may be the only manifestation of Mondini dysplasia, and the benefit of hearing screening is emphasized. Temporal bone computed tomography should be considered in children with unilateral sensorineural or mixed-type hearing impairment. Copyright © 2012. Published by Elsevier B.V.

  15. How to decrease bronchopulmonary dysplasia in your neonatal intensive care unit today and “tomorrow”

    Science.gov (United States)

    Nelin, Leif D.; Bhandari, Vineet

    2017-01-01

    Bronchopulmonary dysplasia, or BPD, is the most common chronic lung disease in infants. Genetic predisposition and developmental vulnerability secondary to antenatal and postnatal infections, compounded with exposure to hyperoxia and invasive mechanical ventilation to an immature lung, result in persistent inflammation, culminating in the characteristic pulmonary phenotype of BPD of impaired alveolarization and dysregulated vascularization. In this article, we highlight specific areas in current management, and speculate on therapeutic strategies that are on the horizon, that we believe will make an impact in decreasing the incidence of BPD in your neonatal intensive care units. PMID:28503300

  16. Anomalies of tooth formation in hypohidrotic ectodermal dysplasia

    DEFF Research Database (Denmark)

    Lexner, Michala O; Bardow, Allan; Hertz, Jens Michael

    2007-01-01

    OBJECTIVE: The X-linked hypohidrotic ectodermal dysplasia (HED) is the most common type of ectodermal dysplasia. The clinical identification of possible heterozygous females can be difficult because of the varying degrees of clinical signs caused by X-chromosome inactivation. This study is the fi......OBJECTIVE: The X-linked hypohidrotic ectodermal dysplasia (HED) is the most common type of ectodermal dysplasia. The clinical identification of possible heterozygous females can be difficult because of the varying degrees of clinical signs caused by X-chromosome inactivation. This study...... affected males had multiple missing permanent teeth and tooth malformations. The heterozygous females had a significantly higher frequency of agenesis of permanent teeth compared to normative data. The heterozygous females had an increased prevalence of tooth malformations and reduced tooth size...

  17. Spondylometaepiphyseal dysplasia in a mother and her child

    Energy Technology Data Exchange (ETDEWEB)

    Pettersson, H; Nilsson, K O [Section of Pediatric Radiology, Department of Diagnostic Radiology, and the Department of Pediatrics, Malmoe Allmaenna Sjukhus, Malmoe, Sweden

    1979-01-01

    Variant types of spondylometaepiphyseal dysplasia in a mother and her child is reported. Several stages of the disorder are presented, demonstrating the principal difficulties in distinguishing variant types of skeletal dysplasia.

  18. The scapula as a window to the diagnosis of skeletal dysplasias

    International Nuclear Information System (INIS)

    Mortier, G.R.; Rimoin, D.L.; Lachman, R.S.

    1997-01-01

    Evaluation of the scapula can be useful in the diagnosis of skeletal dysplasias and helpful for the classification and delineation of new entities. A review of 2100 computerized cases of skeletal dysplasias in the International Skeletal Dysplasia Registry was performed. We found that the Luton type of platyspondylic lethal skeletal dysplasia differed radiographically from the San Diego type and Torrance type by the presence of two spikes at the inferior angle of the scapula. Hypoplasia of the body of the scapula, which is characteristic for campomelic dysplasia but not for kyphomelic dysplasia, is also present in Antley-Bixler syndrome. Radiographic and clinical similarities between campomelic dysplasia and Antley-Bixler syndrome suggest that they might be related disorders and that the latter condition should be included in the bent-bone dysplasia group. Similarity between the metaphyseal regions of the scapula and the metaphyses of the long tubular bones in the different types of short-rib polydactyly syndrome illustrates the importance of evaluation of the scapula in this group as well as in other well-defined or unknown osteochondrodysplasias. (orig.). With 8 figs

  19. Fibrous dysplasia with cartilaginous differentiation (''fibrocartilaginous dysplasia''): a review, with an illustrative case followed for 18 years

    International Nuclear Information System (INIS)

    Kyriakos, Michael; McDonald, Douglas J.; Sundaram, Murali

    2004-01-01

    A 21-year-old man with an 18-year history of progressive, and deforming, monomelic fibrous dysplasia with massive cartilaginous differentiation (fibrocartilaginous dysplasia) is described. A review is made of all prior reported examples of this entity in the English language medical literature. The radiologic and histologic differential diagnoses are described, distinguishing the lesion from chondrosarcoma and from fibrocartilaginous mesenchymoma. (orig.)

  20. HPLC/DAD Intercomparison on Phytoplankton Pigments (HIP-1, HIP-2, HIP-3 and HIP-4)

    OpenAIRE

    CANUTI Elisabetta; RAS Josephine; GRUNG Merete; ROTTGERS Rudiger; COSTA GOELA Priscilla; ARTUSO Florinda; CATALDI Dario

    2016-01-01

    From 2009 to 2015, in the context of the MERIS (Medium Resolution Imaging Spectrometer) validation activities, the JRC Marine Optical Laboratory organised four HPLC Intercomparison exercises for Phytoplankton Pigment measurements (HIP-1, HIP-2, HIP-3 and HIP-4), involving seven European accredited and reference laboratories. The objectives of these intercomparison exercises were: creating a reference community at European level for phytoplankton pigment analysis capable of supporting satel...

  1. Magnetic resonance imaging findings compared with histological findings of the labrum in hip osteoarthritis

    International Nuclear Information System (INIS)

    Kanezaki, Shiho; Nakamura, Shigeru; Matsushita, Takashi; Yamamoto, Asako; Osawa, Marie

    2015-01-01

    Patients with disorders such as acetabular dysplasia or femoroacetabular impingement are at risk of developing hip osteoartbritis. Assessment of the cartilage and labrum in the hip joint based on magnetic resonance imaging (MRI) has been challenging because of the low signal-to-noise ratio (SNR) due to its deep location, ball and socket structure, and small volume of those structures compared with the whole joint size. To achieve better imaging assessment, direct MR. arthrography (d-MRA) and other techniques such as T2* mapping, T2 mapping, Tlrho, and delayed gadolinium-enhanced MRI (dGEMRIC) have been developed along with the increasing use of high-field MRI. In patients with no apparent osteoarthritic changes such as joint space narrowing or osteophyte and subchondral cyst formation on radiographs, these techniques can detect early cartilage or labral damage. A recently developed semiquantitative MRI-based scoring system for hip osteoarthritis includes evaluation of the labrum, and its application as a potential therapeutic monitoring tool is anticipated. The labrum shows pathological changes such as macroscopic hypertrophy and histological degeneration in hip osteoartbritis, but the pathological background is not well understood when evaluated by MRI. Kubo et al. compared radial MRI findings with histological changes of the labrum in ll hips with osteoarthritis using 1.5-T MRJ and found that fibrous separation and mucoid deposition occurred in the labrum with a ''diffuse high signal'' or ''obscure'' pattern. However, to the best of our knowledge, no studies have demonstrated a correlation between MRI fmdings and histological evidence of the severity of degeneration of the labrum. We hypothesized that radially reconstructed images of the acetabular labrum acquired by 3-T MRI can depict degenerative changes of the labrum. In this study, we sought to determine the correlation between MRI and histological findings of the

  2. Magnetic resonance imaging findings compared with histological findings of the labrum in hip osteoarthritis

    Energy Technology Data Exchange (ETDEWEB)

    Kanezaki, Shiho; Nakamura, Shigeru; Matsushita, Takashi [Teikyo University School of Medicine, Department of Orthopaedic Surgery, Itabashi-ku, Tokyo (Japan); Yamamoto, Asako; Osawa, Marie [Teikyo University School of Medicine, Department of Radiology, Itabashi-ku, Tokyo (Japan)

    2015-06-01

    Patients with disorders such as acetabular dysplasia or femoroacetabular impingement are at risk of developing hip osteoartbritis. Assessment of the cartilage and labrum in the hip joint based on magnetic resonance imaging (MRI) has been challenging because of the low signal-to-noise ratio (SNR) due to its deep location, ball and socket structure, and small volume of those structures compared with the whole joint size. To achieve better imaging assessment, direct MR. arthrography (d-MRA) and other techniques such as T2* mapping, T2 mapping, Tlrho, and delayed gadolinium-enhanced MRI (dGEMRIC) have been developed along with the increasing use of high-field MRI. In patients with no apparent osteoarthritic changes such as joint space narrowing or osteophyte and subchondral cyst formation on radiographs, these techniques can detect early cartilage or labral damage. A recently developed semiquantitative MRI-based scoring system for hip osteoarthritis includes evaluation of the labrum, and its application as a potential therapeutic monitoring tool is anticipated. The labrum shows pathological changes such as macroscopic hypertrophy and histological degeneration in hip osteoartbritis, but the pathological background is not well understood when evaluated by MRI. Kubo et al. compared radial MRI findings with histological changes of the labrum in ll hips with osteoarthritis using 1.5-T MRJ and found that fibrous separation and mucoid deposition occurred in the labrum with a ''diffuse high signal'' or ''obscure'' pattern. However, to the best of our knowledge, no studies have demonstrated a correlation between MRI fmdings and histological evidence of the severity of degeneration of the labrum. We hypothesized that radially reconstructed images of the acetabular labrum acquired by 3-T MRI can depict degenerative changes of the labrum. In this study, we sought to determine the correlation between MRI and histological findings of the

  3. Long-term retrospective study on the placement of the cementless acetabular cup and clinical outcomes in patients undergoing femoral head autografting for hip dysplasia and total hip arthroplasty.

    Science.gov (United States)

    Ozden, Vahit Emre; Dikmen, Goksel; Beksac, Burak; Tozun, Ismail Remzi

    2018-05-01

    Placement of acetabular cup in the dysplastic hip is a challenging procedure. Using bulk femoral head autograft to increase the bony coverage of the cup is one of the techniques, which have been described. The impact of cup position on cup and autograft survival is a controversial issue. We aimed to determine whether the position of cementless acetabular cup used in conjunction with femoral head autograft in dysplastic hips affected the autograft-host incorporation with its final radiographic appearance and the cup survivorship into the second decade. Thirty-eight dysplastic hips with varying Crowe types in 31 patients (30 women and one man) were included. The mean age was 47 years (range, 29-64 years) and the mean follow-up was 20.3 years (range, 14.8-25.9 years). The initial postoperative and final radiographs were evaluated. The survival rate of the cups was analysed using Kaplan-Meier statistics and the log-rank test. Multivariate analysis was used to evaluate the effect of variables (Crowe type, radiographic initial host bone coverage over the cup and position of the cup) on survivorship. The acetabular cups were positioned anatomical in 27/38 hips according to Ranawat measurement technique. Trabecular bridging at graft-host interface was seen in all cases at an average 22.1 months. Neither acetabular cup position nor initial host bone coverage over acetabular cup less than 50% had any significant effect on either cup survival or final radiographic appearance of the graft. The 20-year cup survival rate without aseptic revision was 66% (95 CI, 52%-84%). No revision was performed due to graft resorption. Twenty-year survival rate of the cementless cup combination with femoral head autograft showed no significant differences whether it was placed at high or anatomic hip centre. The final radiographic appearance of the autograft was not affected from either the cup location or the initial radiographic horizontal host bone coverage. Copyright © 2018 The Japanese

  4. [Radiographic appraisal between metal and bone interosculate backfill after total hip arthroplasty with trabecular metal cup].

    Science.gov (United States)

    Li, Wei; Zhou, Yi-Xin; Wu, Jian; Xu, Hui; Ji, Song-Jie

    2009-02-15

    To evaluate the bone refilling in the interface between the trabecular metal (TM) acetabular shell and the bone surface according to consecutive X film measuring after surgery. From July 2006 to July 2007, 35 patients (40 hips) accepted total hip replacement using trabecular metal monoblock acetabular cup system (TM). The cup was made of a ellipse shaped press fit Tantalum shell and high cross-linked PE liner (Longevity) with 28 mm inner diameter. The patients demography was: 16 male (20 hips), 19 female (20 hips), 5 bilateral hip replacements, age from 41 - 71 (mean 53), including 18 avascular necrosis hips, 16 osteoarthritis hips (including those secondary to a dysplasia hip), 4 avascular necrosis hips after femoral neck fracture, 2 Ankylosis Spondylitis. All the 40 total hip replacements used posterior approach, using hemispherical acetabular reamer and 2 mm press fit of final metal shell without screw fixation. The consecutive X film was taken at the end time of surgery and 2, 6, 12, 24 weeks, and 12 months. The clinical results was evaluate according to Harris scoring system, and the standard pelvis AP X film was measured at the interface between metal shell and the acetabular bone surface, witch was divided into five regions (A, B, C, D, E). Totally 32 patients (37 hips) were followed with average 8.7 months (7 - 12 months). The Harris before surgery was 50.5 (32 - 85), promoted to 91.0 (72 - 100), including 29 excellent, 6 good, 2 fair, and the total excellent and good rate was 94.6%. Complications include 4 patients leg length discrepancy from 1 - 2 cm, 3 patients moderate thigh pain and released after conservative therapy. No infection and dislocation was found. Twenty-one patients (23 hips) were found lucent line at the bone-metal interface from 1 - 5 mm, most common in B region and BC boundary than C, D, and CD boundary. All the patients followed was found the lucent line disappeared and refilled with bone at X film 24 weeks after surgery, however, no

  5. Locally Aggressive Fibrous Dysplasia Mimicking Malign Calvarial Lesion.

    Science.gov (United States)

    Ogul, Hayri; Keskin, Emine

    2018-05-01

    Fibrous dysplasia is an unusual benign bone tumor. It is divided into 3 groups as monostotic, polyostotic, and craniofacial form. The authors reported an unusual patient with fibrous dysplasia with an aggressive radiologic appearance.

  6. Morphometric analysis of acetabular dysplasia in cerebral palsy: three-dimensional CT study.

    Science.gov (United States)

    Gose, Shinichi; Sakai, Takashi; Shibata, Toru; Murase, Tsuyoshi; Yoshikawa, Hideki; Sugamoto, Kazuomi

    2009-12-01

    Three-dimensional computed tomography (3D-CT) eliminates the positioning errors and allows the clinician to more accurately assess the radiographic parameters present. To elucidate the 3D geometry of the acetabulum and the extent of hip subluxation/dislocation in patients with cerebral palsy (CP), quantitative morphometric analysis was performed using 3D-CT data. We evaluated 150 hips in 75 patients with bilateral spastic CP. The mean age of the patients was 5.4 years (range: 2.7 to 6.9 y). The fitting plane of the ilium was projected onto the coronal plane and then onto the sagittal plane, and then the angle formed with a horizontal line was defined as CTalpha (the lateral opening angle) and CTbeta (the sagittal inclination angle), respectively. The center of the acetabulum and the femoral head were defined, and the distance between these centers was divided by the femoral head diameter, defined as CT migration percentage (CTMP, %). In 123 (82%) of the 150 hips, the femoral head center was located posteriorly, superiorly, and laterally relative to the acetabular center. Large CTalpha cases tended to show large CTMP. CTalpha and CTMP were significantly larger in the cases with Gross Motor Functional Classification System (GMFCS) level IV/V and spastic quadriplegia, than in the cases with GMFCS level II/III and spastic diplegia. CTbeta showed significant correlation with the acetabular defect on the lateral 3D reconstructed images. Three-dimensional acetabular geometry and migration percentage in CP patients can be analyzed quantitatively using 3D-CT regardless of the abnormal spastic posture. The extent of acetabular dysplasia and subluxation is more severe in patients with GMFCS level IV/V and spastic quadriplesia. Level 4.

  7. The association between cervical dysplasia, a short cervix, and preterm birth.

    Science.gov (United States)

    Miller, Emily S; Sakowicz, Allie; Grobman, William A

    2015-10-01

    We sought to determine whether cervical dysplasia in the absence of an excisional procedure is associated with an increased risk of preterm birth (PTB) and whether that risk is independent of the presence of a short cervix. This is a cohort study including women with a singleton pregnancy who underwent routine cervical length assessment between 18-23 6/7 weeks of gestation, stratified by cervical dysplasia (ie, no prior dysplasia, prior dysplasia but no excisional procedure, or prior excisional procedure). The frequency of a short cervix (≤2.5 cm) and PTB were compared between groups and multivariable analyses were performed to identify whether: (1) dysplasia alone or a prior excisional procedure was associated with PTB; and (2) whether these factors remained independently associated with PTB after adjusting for the presence of a short cervix. Of the 18,528 women who met inclusion criteria, 3023 (16.3%) had prior dysplasia alone and 1356 (7.3%) had a prior excisional procedure. The frequency of a short cervix for women without dysplasia, with prior dysplasia alone, or with a prior excisional procedure was 0.8%, 1.0%, and 2.2%, respectively (P cervix. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. An unusual case of ectodermal dysplasia: combating senile features at an early age

    Science.gov (United States)

    Gupta, Mudit; Sundaresh, Kumbar Jayadevappa; Batra, Manu; Rathva, Vandana J

    2014-01-01

    Ectodermal dysplasia (ED) refers to a group of inherited diseases that have developmental defects in at least two major structures derived from the ectoderm, that is, hair, teeth, nails and sweat glands. Although more than 192 distinct disorders have been described, the most common is X-linked recessive hypohidrotic ED (Christ-Siemens-Touraine syndrome). Since such patients usually presents with missing teeth, dentists are usually the first person to diagnose such cases. Diagnosis of such cases is important because absence of sweat glands can lead to hyperthermia which can be life-threatening if proper care is not taken. Through this manuscript, we report a case of anhidrotic ED affecting deciduous and permanent dentition, which is rare. PMID:24493109

  9. Genetics Home Reference: X-linked spondyloepiphyseal dysplasia tarda

    Science.gov (United States)

    ... Educational Resources (6 links) Cincinnati Children's Hospital: Coxa Vera Disease InfoSearch: Spondyloepiphyseal dysplasia tarda X-linked Johns ... Free article on PubMed Central Savarirayan R, Thompson E, Gécz J. Spondyloepiphyseal dysplasia tarda (SEDL, MIM #313400). ...

  10. Transposition of the acetabulum after iliac ischial osteotomy in the treatment of hip dysplasia in infants

    Directory of Open Access Journals (Sweden)

    Vladimir E Baskov

    2016-06-01

    Conclusion. Transposition of the acetabulum after iliac and ischial pelvic osteotomy is an effective treatment for dysplastic instability of the acetabulum in children aged 9–16 years. The procedure is indicated when it is necessary to rotate the acetabular fragment by more than 25°, and there is no need for hip medialization.

  11. Thanatophoric Dysplasia: A Rare Entity

    Directory of Open Access Journals (Sweden)

    N.S. Naveen

    2011-05-01

    Full Text Available Thanatophoric dysplasia is the lethal skeletal dysplasia characterized by marked underdevelopment of the skeleton and short-limb dwarfism. The child will be having a short neck, narrow thoracic cage and protuberant abdomen. Other anatomical features include a relatively enlarged head with frontal bossing, prominent eyes, hypertelorism and the depressed nasal bridge. The diagnosis is usually made with the ultrasonography in the second trimester. In this study we report a case of this rare entity with emphasis on its anatomical features, abnormalities and clinical profile with relevant review of literature.

  12. Hip Resurfacing: An Alternative to Conventional Hip Replacement?

    Science.gov (United States)

    ... Is hip resurfacing a good alternative to hip replacement? Answers from Mark Spangehl, M.D. Hip resurfacing has lost favor with many surgeons ... women with poor bone quality. Unlike traditional hip replacement, hip resurfacing doesn't completely replace the "ball" ...

  13. Papillary bile duct dysplasia in primary sclerosing cholangitis.

    Science.gov (United States)

    Ludwig, J; Wahlstrom, H E; Batts, K P; Wiesner, R H

    1992-06-01

    A 62-year-old man with a 20-year history of chronic ulcerative colitis and a 9-year history of primary sclerosing cholangitis (PSC) underwent orthotopic liver transplantation because of symptoms related to PSC and cholangiographic features compatible with a biliary neoplasm. Study of the excised liver revealed papillary mucosal lesions in the common hepatic duct and the right and left hepatic ducts as well as cholangiectases and other features typically associated with PSC. The papillary lesions consisted of abundant fibrovascular stroma covered by biliary epithelium with low-grade and high-grade dysplasia. Some periductal glands were also dysplastic. These features distinguished papillary dysplasia from classic biliary papillomatosis. Only one focus of microinvasion was found; there were no metastases. Among 60 cases of PSC in whom the entire liver could be studied after orthotopic liver transplantation, this was the only instance of unequivocal dysplasia. However, in one specimen, papillary hyperplasia was found. Detailed macroscopic and microscopic rereview of 23 livers from our patients with the longest history of PSC (range, 5-24 years) failed to reveal any additional cases with dysplasia. It is concluded that (a) papillary mucosal lesions in PSC may represent papillary dysplasia without invasion; (b) these lesions may evolve from papillary hyperplasia; (c) the process may be largely, if not entirely, in situ; and (d) the prevalence of dysplasia and carcinoma of bile ducts may be less than the 7%-9% reported in the literature for malignancies associated with PSC.

  14. Thanatophoric Dysplasia; a Rare Case Report on a Congenital Anomaly

    Directory of Open Access Journals (Sweden)

    Maria Francis Yuvaraj

    2017-01-01

    Full Text Available The rare form of skeletal dysplasia is thanatophoric dysplasia. The meaning for thanatophoric dysplasia is death bearing which is derived from Greek word. It occurs 1in 20,000 to 50,000. It is mainly due to mutations in the fibroblast growth factor receptor 3gene. Features of thanatophoric dysplasia are frontal bossing, prominent eyes, narrow thorax, protruded abdomen and bowed legs. The knowledge about this condition is useful in the fields of Anatomy, Paediatrics, Obstetrics and Gynaecology, Ultrasonagraphy and Genetics, for future research purpose.

  15. The Ectodermal Dysplasias : Severe Palmoplantar Hyperkeratosis And Chronic Angular Cheilitis

    Directory of Open Access Journals (Sweden)

    Mahajan Vikram K

    2003-01-01

    Full Text Available The ectodermal dysplasias are congenital, non-progressive and diffuse disorders affecting primarily the tissues derived from ectoderm. Over a period, their classification has become confusing due to indiscriminate use of them “ectodermal dysplasia” for numerous syndromes with a defect in one or more epidermal defect in each element of skin; their precise classification appears difficult as yet. Only X-linked recessive ectodermal dysplasia (Christ-Siemens-Touraine syndrome remains best defined. This paper describes three cases of ectodermal dysplasias highlighting their overlapping features.

  16. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome

    Directory of Open Access Journals (Sweden)

    Reema Sharma Dhar

    2014-01-01

    Full Text Available Ectrodactyly-ectodermal dysplasia-cleft (EEC syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report.

  17. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome.

    Science.gov (United States)

    Dhar, Reema Sharma; Bora, Amitava

    2014-01-01

    Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report.

  18. Progressive pseudorheumatoid dysplasia in North and West Africa ...

    African Journals Online (AJOL)

    Progressive pseudorheumatoid dysplasia is a rare autosomal recessive spondyloepiphyseal dysplasia characterized by predominant involvement of articular cartilage with progressive joint stiffness and enlargement in the absence of inflammation. Short stature, joint contractures, gait disturbance, and scoliosis and/or ...

  19. MR imaging of the hip

    International Nuclear Information System (INIS)

    Kramer, J.

    2006-01-01

    Full text: MRI has been shown to gain increasing importance in recent years in the evaluation of disorders of the hip and pelvis. Nowadays, conventional or fast spin echo (T1, Turbo (Fast) T2 (or PD)) MR imaging sequences are used most commonly to examine the hip and pelvis. Three dimensional (3D) gradient echo (GE) imaging allows acquisition of very thin (1 mm) sections. MR imaging with fat suppression technique (STIR, TIRM, FAT SAT T2) represent an effective screening technique for the assessment of fluid, hemorrhage, pus, or tumor in the marrow, joint or soft tissues. These techniques increase the sensitivity for the detection of a variety of pathologic processes. Fat suppression MR imaging, when combined with intravenous (or intraarticular) administration of a gadolinium compound, also is effective and often is employed when evaluating neoplastic or infectious diseases (intravenous injection), abnormalities of articular cartilage or labrum, or possible intraarticular osteocartilaginous bodies (intraarticular injection). A lesion of the acetabular labrum based is one cause of chronic hip pain in patients with developmental dysplasia of the hips, as well as in patients with a history of hip trauma or femoroacetabular impingement. MR imaging and, in particular, MR arthrography, however, have been shown to be highly accurate in the detection of subtle intraarticular abnormalities such as labral lesion. Conventional MR imaging has shown poor accuracy in detecting labral lesions, due to its inability to depict small tears and the difficulty in determining if intralabral signal abnormalities extend to the surface of the labrum. MR arthrography, however has been shown to overcome these difficulties and to have excellent accuracy in detecting labral pathology. As labral abnormalities are believed to be a precursor of osteoarthritis, orthopedic surgeons believe that surgical intervention will be influenced substantially by the type, extension and location of labral

  20. Overexpression of Indian hedgehog partially rescues short stature homeobox 2-overexpression-associated congenital dysplasia of the temporomandibular joint in mice.

    Science.gov (United States)

    Li, Xihai; Liang, Wenna; Ye, Hongzhi; Weng, Xiaping; Liu, Fayuan; Lin, Pingdong; Liu, Xianxiang

    2015-09-01

    The role of short stature homeobox 2 (shox2) in the development and homeostasis of the temporomandibular joint (TMJ) has been well documented. Shox2 is known to be expressed in the progenitor cells and perichondrium of the developing condyle. A previous study by our group reported that overexpression of shox2 leads to congenital dysplasia of the TMJ via downregulation of the Indian hedgehog (Ihh) signaling pathway, which is essential for embryonic disc primordium formation and mandibular condylar growth. To determine whether overexpression of Ihh may rescue the overexpression of shox2 leading to congenital dysplasia of the TMJ, a mouse model in which Ihh and shox2 were overexpressed (Wnt1-Cre; pMes-stop shox2; pMes-stop Ihh mice) was utilized to assess the consequences of this overexpression on TMJ development during post-natal life. The results showed that the developmental process and expression levels of runt-related transcription factor 2 and sex determining region Y-box 9 in the TMJ of the Wnt1-Cre; pMes-stop shox2; pMes-stop Ihh mice were similar to those in wild‑type mice. Overexpression of Ihh rescued shox2 overexpression-associated reduction of extracellular matrix components. However, overexpression of Ihh did not inhibit the shox2 overexpression-associated increase of matrix metalloproteinases (MMPs) MMP9, MMP13 and apoptosis in the TMJ. These combinatory cellular and molecular defects appeared to account for the observed congenital dysplasia of TMJ, suggesting that overexpression of Ihh partially rescued shox2 overexpression‑associated congenital dysplasia of the TMJ in mice.

  1. Spondyloepiphyseal dysplasia congenita. A cause of lethal neonatal dwarfism

    Energy Technology Data Exchange (ETDEWEB)

    Macpherson, R.I.; Wood, B.P.

    1980-07-01

    Spondyloepiphyseal dysplasia congenita is a form of primarily short trunk dwarfism, that is manifest at birth but generally has not been regarded as a cause of lethal neonatal dwarfism. Seven neonates with severe dwarfism are presented. The first survived the newborn period, but the other six were early neonatal deaths. All displayed the clinical and radiologic features of spondyloepiphyseal dysplasia congenita. The striking similarities between spondyloepiphyseal dysplasia congenita and achondrogenesis type 2 are discussed.

  2. Treatment of oral dysplasia with 5% imiquimod cream: short communication.

    Science.gov (United States)

    Mullins, R; Ansell, M; Laverick, S

    2016-11-01

    We report what we think is the first treatment of oral dysplasia with 5% imiquimod cream. A 60-year-old man presented with varying degrees of dysplasia on the soft palate. A cover plate was fabricated and the patient was prescribed 5% imiquimod cream, a topical imunomodulator, for six weeks. The lesion improved and histological examination of an incisional biopsy found no features of dysplasia. This case highlights the efficacy of imiquimod cream in the treatment of dysplasia, and the need for development of a preparation suitable for the oral mucosa. Copyright © 2016. Published by Elsevier Ltd.

  3. Pseudoachondroplastic dysplasia.

    Directory of Open Access Journals (Sweden)

    Khungar A

    1993-04-01

    Full Text Available Pseudoachondroplasia is a heterogeneous inherited skeletal dysplasia in which dwarfism is a major feature. We report here a case of a 7 year old girl misdiagnosed as rickets, who presented with short stature, lordosis, genu varum and flexion deformities at both the elbows. Skeletal survey revealed epiphyseal and metaphyseal irregularities. A review of literature is also presented.

  4. [Protein-energy malnutrition in patients with connective tissue dysplasia].

    Science.gov (United States)

    Lialiukova, E A

    2013-01-01

    In the conditions of the specialized Center of a dysplasia of a connecting tissue the assessment of an protein--energy malnutrition at 121 patients with signs of a dysplasia of a connecting tissue is carried out. High frequency of an oligotrophy at patients with a dysplasia of a connecting tissue is registered. The I degree of a gipotorofiya is taped at 26.21% of the patients, II degree--at 18.44%, the III degree--at 3.88% of patients.

  5. Prosthodontic management of a patient with ectodermal dysplasia.

    Science.gov (United States)

    Nandini, Yamini

    2013-12-01

    Ectodermal dysplasia is a rare congenital disease that affects the ectodermal structures. It is characterized by hypotrichosis, hypohidrosis and hypodontia. A 14-year-old boy with ectodermal dysplasia presenting with oligodontia and marked resorption of the maxillary and mandibular alveolar ridges is reported. Prosthetic rehabilitation in the form of a maxillary and mandibular partial denture was made with metal crowns on existing lower teeth to achieve appropriate vertical dimension. Significant improvement in speech, masticatory function and facial esthetics was achieved. Removable prosthodontics can provide an acceptable solution to esthetic, functional and psychological rehabilitation in patients with ectodermal dysplasia.

  6. Immunohistochemical Expression of MCM-2 in Oral Epithelial Dysplasias.

    Science.gov (United States)

    Zakaria, Samar H; Farag, Heba A; Khater, Dina S

    2016-03-17

    Oral cancer is one of the most frequent cancers in the world. It arises from epithelial dysplasia. Hence, identifying these lesions in an early stage could prevent their malignant transformation. The aim of the present work was to assess the cell proliferative activity of minichromosome maintenance protein (MCM-2) in oral epithelial dysplastic lesions and to correlate the results with different grades of epithelial dysplasia in an attempt to use MCM-2 in the early detection of malignancy. MCM-2 expression was determined by the nuclear count in a total of 30 oral epithelial dysplastic specimens roughly classified into 10 cases of mild, moderate, and severe dysplasia. Five cases of early invasive squamous-cell carcinomas and 5 cases of epithelial hyperplasia were also included. The MCM-2 immunostaining was found to increase gradually from mild to moderate to severe dysplasia and reached its maximum value in early invasive squamous cell carcinoma. MCM-2 is of prognostic value in cases of oral dysplasia that have a tendency to undergo malignant transformation.

  7. Concurrent follicular dysplasia and interface dermatitis in Boxer dogs.

    Science.gov (United States)

    Rachid, Milene A; Demaula, Christopher D; Scott, Danny W; Miller, William H; Senter, David A; Myers, Sherry

    2003-06-01

    Recurrent or persistent follicular dysplasia and interface dermatitis are described in nine Boxers. Data on age, sex, seasonality of alopecia and histopathological features of the follicular dysplasia in these nine Boxers are comparable with those described in previous reports. The interface dermatitis was characterized by multifocal annular crusted lesions confined to the areas of follicular dysplasia. The inflammatory lesions were neither pruritic nor painful and affected dogs were otherwise healthy. Histopathologically the clinically inflammatory lesions were characterized as an interface dermatitis. Immunohistochemical studies failed to demonstrate immunoglobulins or complement at the basement membrane zone or within blood vessel walls. In dogs with recurrent or persistent disease, the follicular dysplasia and interface dermatitis ran identical, concurrent courses of spontaneous remission and recurrence, or persistence, respectively. One dog with persistent disease was treated successfully with tetracycline and niacinamide for the interface dermatitis, and melatonin for the follicular dysplasia. Although the aetiopathogenesis of this newly described condition and the relationship between the two histological reaction patterns are not known, photoperiod and genetic predisposition appear to play a role.

  8. Hip, Hip, Soret!

    Science.gov (United States)

    Müller-Plathe, Florian

    Many years ago, Ludwig did detect the behaviour now called the Soret effect. Sodium sulphate in eighteen-fifty-six did not obediently follow Fick's first law. But if he cooled down one side the salt went left, the water to the right. He was surprised in every way. Hip, hip, Soret!

  9. Mondini dysplasia

    International Nuclear Information System (INIS)

    Takeda, Taizo; Kakigi, Akinobu; Takeuchi, Shunji; Saito, Haruo

    1992-01-01

    Recent advances in imaging technique, including high resolution thin section computed tomography scanning and magnetic resonance imaging (MRI), permit the easy diagnosis of congenital malformations of the osseous labyrinth, which have so far been lumped together as 'Mondini dysplasia'. In the present study, the anatomic patterns from the radiogrpahic appearance and the clinical manifestation were examined in 18 patients (23 ears) with radiographic abnormalities of the inner ear. The most common abnormal configuration was a large vestibule (20 of 23 ears). This anomaly of the vestibule often involved the lateral semicircular canal, revealed as a round high signal zone on T2-weighted MRI. However, a large vestibule was not always associated with an abnormal cochlea. Abnormal cochleas were found in 8 of the 20 ears with a large vestibule, and most of these 8 ears had total or profound deafness. But 4 of the 10 ears with residual hearing had low tone deafness and 6 had fluctuating hearing loss, which was frequently associated with attacks of dizziness. These clinical manifestations of Mondini dysplasia are similar to those of patients with endolymphatic hydrops. (author)

  10. Distrator articular no diagnóstico radiográfico precoce da displasia coxofemoral em cães Articular distractor in the early radiographic diagnosis of canine hip dysplasia

    Directory of Open Access Journals (Sweden)

    R.C.S. Tôrres

    2005-02-01

    Full Text Available Foram estudadas radiograficamente as articulações coxofemorais de 60 cães de diferentes raças (32 fêmeas e 28 machos, com o objetivo de se estabelecer o diagnóstico precoce da displasia coxofemoral (DCF. Os exames foram feitos em idade jovem (7,2± 1,2 meses e repetidos em idade adulta (14,4± 1,6 meses. Utilizaram-se os métodos radiográfico convencional (MRC e radiográfico por distração (MRD, feito com o auxílio de um dispositivo, especialmente idealizado e confeccionado para o experimento. Observou-se correlação (PAiming the canine hip dysplasia (CHD early diagnosis, 60 dogs of both sexes (32 females and 28 males and of different breeds had their hip joints radiographically studied. The X ray examinations were taken in early age (7.2± 1.2 months and repeated at adult age (14.4± 1.6 months using the conventional radiographic method (CRM and the radiographic distraction method (RDM performed, by its turn, with a new device, specially designed or this experiment. In order to quantify the relationship between the femoral head and the acetabulus the Norberg Angle (NA was measured at CRM and the distraction index (DI was calculated at RDM. There was a significant statistical correlation (P<0.001 between DI of the first examination and NA of the second examination. The cut off for sick and normal subjects for DI was 0.35. The dogs with DI values greater than 0,35 presented radiographic signals of degenerative joint disease (DJD. Interestingly, the highest was the DI; the greatest were the radiographic signals of DJD. Four animals considered normal when CRM was performed at second examination had DI superior than 0.35 at first examination. This fact shows that false negative results can occur when only the conventional method is used. Two of three sick dogs that had DI values bellow 0.35 were classified as borderline by CRM. This confirms the indefinite of this class. The association of CRM and the proposed method showed to be

  11. Outcome of one-stage treatment of developmental dysplasia of hip in older children

    Directory of Open Access Journals (Sweden)

    Basant Kumar Bhuyan

    2012-01-01

    Conclusions: Young children having DDH can safely be treated with an extensive one-stage triple procedure of open reduction, femoral shortening derotation, and Salter′s osteotomy, without increasing the risk of AVN. Early diagnosis and intervention is the successful treatment of patients suffering from DDH.

  12. Sessile serrated adenomas with dysplasia: morphological patterns and correlations with MLH1 immunohistochemistry.

    Science.gov (United States)

    Liu, Cheng; Walker, Neal I; Leggett, Barbara A; Whitehall, Vicki Lj; Bettington, Mark L; Rosty, Christophe

    2017-12-01

    Sessile serrated adenomas are the precursor polyp of approximately 20% of colorectal carcinomas. Sessile serrated adenomas with dysplasia are rarely encountered and represent an intermediate step to malignant progression, frequently associated with loss of MLH1 expression. Accurate diagnosis of these lesions is important to facilitate appropriate surveillance, particularly because progression from dysplasia to carcinoma can be rapid. The current World Health Organization classification describes two main patterns of dysplasia occurring in sessile serrated adenomas, namely, serrated and conventional. However, this may not adequately reflect the spectrum of changes seen by pathologists in routine practice. Furthermore, subtle patterns of dysplasia that are nevertheless associated with loss of MLH1 expression are not encompassed in this classification. We performed a morphological analysis of 266 sessile serrated adenomas with dysplasia with concurrent MLH1 immunohistochemistry with the aims of better defining the spectrum of dysplasia occurring in these lesions and correlating dysplasia patterns with MLH1 expression. We found that dysplasia can be divided morphologically into four major patterns, comprising minimal deviation (19%), serrated (12%), adenomatous (8%) and not otherwise specified (79%) groups. Minimal deviation dysplasia is defined by minor architectural and cytological changes that typically requires loss of MLH1 immunohistochemical expression to support the diagnosis. Serrated dysplasia and adenomatous dysplasia have distinctive histological features and are less frequently associated with loss of MLH1 expression (13 and 5%, respectively). Finally, dysplasia not otherwise specified encompasses most cases and shows a diverse range of morphological changes that do not fall into the other subgroups and are frequently associated with loss of MLH1 expression (83%). This morphological classification of sessile serrated adenomas with dysplasia may represent an

  13. Bilateral anophthalmia with septo-optic dysplasia

    Directory of Open Access Journals (Sweden)

    Manisha Jana

    2010-01-01

    Full Text Available Bilateral anophthalmia is a rare entity and association with septo-optic dysplasia is an even rare condition. The condition is characterized by absent eyeballs in the presence of eyelids, conjunctiva or lacrimal apparatus. Though anophthalmia can be diagnosed clinically, imaging plays a crucial role in delineating the associated anomalies. In addition, often clinical anophthalmia may prove to be severe microphthalmia on imaging. We describe the imaging findings in an infant with bilateral anophthalmia and septo-optic dysplasia.

  14. Urethral dysontogenic metaplasia in cat with bilateral renal dysplasia

    Directory of Open Access Journals (Sweden)

    Carolina da Fonseca Sapin

    2017-10-01

    Full Text Available This paper to describe a case of dysontogenic urethral metaplasia in a one month old mongrel feline who also had bilateral renal dysplasia. Dysontogenic metaplasia in cats are scarce and this change may be associated with renal dysplasia and/or lower urinary tract. The animal had history of abdominal enlargement since birth and dysuria, eliminating urine only dropwise. Due to the poor prognosis we opted for euthanasia. At necropsy was observed enlarged and distended bladder, reduced kidneys and dilated and tortuous ureters. The urethra was thickened, hard to cut, and histologically, was replacing the connective tissue, cartilage and endochondral ossification areas, which features dysontogenic metaplasia. Both kidneys presented primitive appearance featuring dysplasia. Dysontogenic metaplasia in urinary tract feline with renal dysplasia, has not been described.

  15. Adamantinoma, osteofibrous dysplasia and differentiated adamantinoma

    International Nuclear Information System (INIS)

    Kahn, Leonard B.

    2003-01-01

    For just over 100 years, adamantinoma has been recognized as a primary bone tumor with epithelial characteristics and predominantly involving the tibia. Osteofibrous dysplasia is a fibro-osseous lesion also predominantly confined to the tibia with radiologic features similar to those of adamantinoma. This lesion has been shown by immunohistochemical studies to frequently contain cytokeratin-positive epithelial cells. More recently, a third group of cases with clinical and radiologic features similar to those of osteofibrous dysplasia have demonstrated more overt strands of epithelial cells within a fibro-osseous background and have been categorized as ''differentiated'', ''regressive'', ''juvenile intracortical'' or ''osteofibrous dysplasia-like'' adamantinoma. Cytokeratin subset immunohistochemical stains and cytogenetic studies performed in recent years suggest a common histogenesis for these three entities. This article reviews the clinical, radiologic and pathologic features of these entities as well as their prognostic significance. It also reviews the results of the immunohistochemical and cytogenetic studies which establish a common histogenetic relationship. (orig.)

  16. Variable manifestations of dysplasia epiphysealis hemimelica

    Energy Technology Data Exchange (ETDEWEB)

    Azouz, E.M.; Slomic, A.M.; Marton, D.; Rigault, P.; Finidori, G.

    1985-01-01

    Dysplasia epiphysealis hemimelica (DEH) is an osteocartilaginous overgrowth involving one or multiple epiphyses or ossification centers, usually in a lower extremity on one side of the body. Characteristically the involvement is hemimelic, i.e., either the medial or lateral part of the ossification center is involved. The authors have studied 24 patients with DEH and are adding 15 new cases to the literature. Because of the variable manifestations of the dysplasia and its different degrees of involvement in the affected children, they have subdivided it into localized, classical and generalized. In the generalized form, there is involvement of a whole lower extremity from the pelvis to the foot, and some of these patients show megaepiphyses with enlargement of a whole epiphyseal center, not only its medial or lateral part. The authors have also described and illustrated other special features of the dysplasia especially the advanced bone age and the metaphyseal and growth plate involvement.

  17. Fibrous Dysplasia versus Juvenile Ossifying Fibroma: A Dilemma

    Directory of Open Access Journals (Sweden)

    Sreelakshmi N. Nair

    2016-01-01

    Full Text Available Fibrous dysplasia (FD is a condition characterized by excessive proliferation of bone forming mesenchymal cells which can affect one bone (monostotic type or multiple bones (polyostotic type. It is predominantly noticed in adolescents and young adults. Fibrous dysplasia affecting the jaws is an uncommon condition. The most commonly affected facial bone is the maxilla, with facial asymmetry being the chief complaint. The lesion in many instances is confused with ossifying fibroma (OF. Diagnosis of these two lesions has to be done based on clinical, radiographic, and microscopic findings. Here, we present a case of fibrous dysplasia of maxilla in a nine-year-old boy mimicking juvenile ossifying fibroma.

  18. The Possible Relationship Between Mammary Dysplasia and Breast ...

    African Journals Online (AJOL)

    Aim: There is need to resolve the continuing difficult question regarding the possible relationship between mammary dysplasia and breast cancer. Method: This is a 30-year study of the incidences of both mammary dysplasia and breast cancer occurring among the Igbos, a major ethnic group in Nigeria, West Africa. Results: ...

  19. Ectodermal dysplasia associated with sickle cell disease.

    Science.gov (United States)

    Volpato, Luiz Evaristo Ricci; Volpato, Maria Carmen Palma Faria; de Carvalhosa, Artur Aburad; Palma, Vinicius Canavarros; Borges, Alvaro Henrique

    2014-01-01

    Ectodermal dysplasia and sickle cell anaemia are inherited disorders that affect, respectively, the tissues derived from the embryonic ectoderm and the production of erythrocytes by the bone marrow. The simultaneous occurrence of both disorders is extremely rare. This is a case of both ectodermal dysplasia and sickle cell anaemia reported in a 6-year-old. The patient had been diagnosed with sickle cell anaemia for only six months when he sought treatment presenting with the following: hypotrichosis, dry skin, periocular hyperpigmentation, protruding lips, hypodontia, and morphologically altered teeth. The clinical features combined with his medical history led to the diagnosis of ectodermal dysplasia. Dentists should be prepared to recognise patterns that escape normality to aid in the diagnosis of systemic changes, even in patients with other previous diagnoses.

  20. Ectodermal Dysplasia Associated with Sickle Cell Disease

    Directory of Open Access Journals (Sweden)

    Luiz Evaristo Ricci Volpato

    2014-01-01

    Full Text Available Ectodermal dysplasia and sickle cell anaemia are inherited disorders that affect, respectively, the tissues derived from the embryonic ectoderm and the production of erythrocytes by the bone marrow. The simultaneous occurrence of both disorders is extremely rare. This is a case of both ectodermal dysplasia and sickle cell anaemia reported in a 6-year-old. The patient had been diagnosed with sickle cell anaemia for only six months when he sought treatment presenting with the following: hypotrichosis, dry skin, periocular hyperpigmentation, protruding lips, hypodontia, and morphologically altered teeth. The clinical features combined with his medical history led to the diagnosis of ectodermal dysplasia. Dentists should be prepared to recognise patterns that escape normality to aid in the diagnosis of systemic changes, even in patients with other previous diagnoses.