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Sample records for cleft lip-palate patients

  1. Management of feeding Problem in a Patient with Cleft Lip/Palate.

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    Goswami, Mridula; Jangra, Babita; Bhushan, Urvashi

    2016-01-01

    In a child with cleft lip and/or palate, nutrition is the first priority as for any other child. These children have specific physical limitations. To fulfill their nutritional requirement, these children need modifications in order to thrive and grow. Failure to adjust to these needs could place the children into a potential life-threatening situation. One of the immediate problems to be addressed in a newborn with cleft lip/palate is difficulty in feeding. Nasal regurgitation and choking are common because of inability of the palate to separate the nasal and oral cavities. The case presented here discusses the management of feeding problem in the infant with cleft lip/palate. How to cite this article: Goswami M, Jangra B, Bhushan U. Management of feeding Problem in a Patient with Cleft Lip/ Palate. Int J Clin Pediatr Dent 2016;9(2):143-145. PMID:27365936

  2. Management of feeding Problem in a Patient with Cleft Lip/Palate

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    Goswami, Mridula; Bhushan, Urvashi

    2016-01-01

    ABSTRACT In a child with cleft lip and/or palate, nutrition is the first priority as for any other child. These children have specific physical limitations. To fulfill their nutritional requirement, these children need modifications in order to thrive and grow. Failure to adjust to these needs could place the children into a potential life-threatening situation. One of the immediate problems to be addressed in a newborn with cleft lip/palate is difficulty in feeding. Nasal regurgitation and choking are common because of inability of the palate to separate the nasal and oral cavities. The case presented here discusses the management of feeding problem in the infant with cleft lip/palate. How to cite this article: Goswami M, Jangra B, Bhushan U. Management of feeding Problem in a Patient with Cleft Lip/ Palate. Int J Clin Pediatr Dent 2016;9(2):143-145. PMID:27365936

  3. The status of oral hygiene in cleft lip, palate patients after surgical correction

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    Pandey S

    2005-01-01

    Full Text Available The cleft lip and palate patients usually present a number of problems viz. altered oral anatomy leading to changes in oral physiology diminishing the self-cleansing ability of individual. The handicapped children are unable to maintain their oral hygiene properly. The present study was formulated with the aim that does normalization of oral anatomy have its effect on improvement of oral hygiene? An assessment of oral hygiene index-simplified was performed between preoperative and postoperative values in the same patient at KGMU and KGDU. A total of 50 cases were recorded in two groups of 25 each: (i < 6 years old and (ii > 6 years. The observations are statistically analyzed by paired ′t′ test to get the significance of results. Results: The data analyzed showed the significant decrease in oral hygiene indices observed in both groups. A relative significance in oral hygiene status following surgery was observed. Both groups expressed greater significance when compared pre and postoperatively which is indicative of considerable improvement of oral hygiene after surgical correction. The study concludes that oral hygiene improves more in older cleft lip-palate cases following reconstruction of palatal vault, premaxilla and anterior lip seal by secondary bone grafting method when compared with oral hygiene indices results in primary periosteoplasty cases. The surgical correction of cleft lip palate enhances self-cleaning ability and better compliance to maintain oral hygiene in children as the age advances.

  4. Accelerated wound closure in vitro by fibroblasts from a subgroup of cleft lip/palate patients: role of transforming growth factor-α.

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    Beyeler, Joël; Schnyder, Isabelle; Katsaros, Christos; Chiquet, Matthias

    2014-01-01

    In a fraction of patients surgically treated for cleft lip/palate, excessive scarring disturbs maxillary growth and dento-alveolar development. Since certain genes are involved in craniofacial morphogenesis as well as tissue repair, a primary defect causing cleft lip/palate could lead to altered wound healing. We performed in vitro wound healing assays with primary lip fibroblasts from 16 cleft lip/palate patients. Nine foreskin fibroblast strains were included for comparison. Cells were grown to confluency and scratch wounds were applied; wound closure was monitored morphometrically over time. Wound closure rate showed highly significant differences between fibroblast strains. Statistically, fibroblast strains from the 25 individuals could be divided into three migratory groups, namely "fast", "intermediate", and "slow". Most cleft lip/palate fibroblasts were distributed between the "fast" (5 strains) and the "intermediate" group (10 strains). These phenotypes were stable over different cell passages from the same individual. Expression of genes involved in cleft lip/palate and wound repair was determined by quantitative PCR. Transforming growth factor-α mRNA was significantly up-regulated in the "fast" group. 5 ng/ml transforming growth factor-α added to the culture medium increased the wound closure rate of cleft lip/palate strains from the "intermediate" migratory group to the level of the "fast", but had no effect on the latter group. Conversely, antibody to transforming growth factor-α or a specific inhibitor of its receptor most effectively reduced the wound closure rate of "fast" cleft lip/palate strains. Thus, fibroblasts from a distinct subgroup of cleft lip/palate patients exhibit an increased migration rate into wounds in vitro, which is linked to higher transforming growth factor-α expression and attenuated by interfering with its signaling. PMID:25360592

  5. Comparison of oral hygiene and periodontal status in patients with clefts of palate and patients with unilateral cleft lip, palate and alveolus

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    Mutthineni Ramesh

    2010-01-01

    Full Text Available Aim: This study was conducted to analyze and compare the oral hygiene and periodontal status in patients with clefts of palate (CP and patients with unilateral cleft lip, palate and alveolus (UCLPA. Materials and Methods: The study group consisted of 120 cleft patients. Subjects were divided into two groups of 60 each. Group I - patients with UCLPA and Group II - patients with CP. For comparison, all the four quadrants were defined, Q1-right upper quadrant, Q2-left upper quadrant, Q3-left lower quadrant and Q4-right lower quadrant, in both groups and the following parameters were recorded: Plaque Index (PI, Silness and Loe, Sulcus Bleeding Index (SBI, Muhlemann and Son, Probing Pocket Depth (PPD, Clinical Attachment Level (CAL, Mobility Index (Miller and Radiographic Amount of Bone Loss. Results: The periodontal destruction was seen to be higher in UCLPA patients compared with CP patients. The poor oral hygiene status, as indicated by higher values of PI, and the periodontal status, evaluated by SBI, PPD, CAL, mobility and Radiographic Amount of Bone Loss, were higher in patients with UCLPA than in patients with CP. Conclusion: In this study, patients with cleft lip, palate and alveolus had poor oral hygiene and periodontal status compared with patients with cleft palate.

  6. Comparative Evaluation of Prevalence of Upper Cervical Vertebrae Anomalies in Cleft Lip/Palate Patients: A Retrospective Study

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    Datana, Sanjeev; Kumar, Prasanna; Kumar Roy, Supriya; Londhe, Sanjay

    2014-01-01

    ABSTRACT% Purpose: The patients with cleft lip and palate have a higher risk of cervical vertebrae anomalies than do patients in general population. The aim of present study was to determine the prevalence of various upper cervical spine anomalies in different type of clefts. Procedures: Lateral cephalograms of 128 patients (66 males, 62 females) with cleft lip and palate, and 125 (60 males, 65 females) non syndromic patients without cleft lip and palate were selected at random from archive. Cephalograms of the patients were traced and the diagnosis of any cervical vertebrae anomaly was noted. Anomalies were categorized as either: posterior arch deficiency or fusions. Main findings: Prevalence of cervical vertebrae anomalies in the c lef t group was 20. 3% while it was 6.4% in the control group. Further cervical vertebrae anomalies were 16.6% in the CPO group, 19.1% in the BCLP group, and 22.2% in the UCLP group. Conclusion: A higher prevalence of cervical vertebrae anomalies was observed in cleft lip and palate patients. The prevalenc e obser ved is 3 times more in clef t group than c ontrol group. How to cite this article: Datana S, Bhalla A, Kumar P, Roy SK, Londhe S. Comparative Evaluation of Prevalence of Upper Cervical Vertebrae Anomalies in Cleft Lip/Palate Patients: A Retrospective Study. Int J Clin Pediatr Dent 2014;7(3):168-171. PMID:25709295

  7. The status of oral hygiene in cleft lip, palate patients after surgical correction

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    Pandey S; Pandey R

    2005-01-01

    The cleft lip and palate patients usually present a number of problems viz. altered oral anatomy leading to changes in oral physiology diminishing the self-cleansing ability of individual. The handicapped children are unable to maintain their oral hygiene properly. The present study was formulated with the aim that does normalization of oral anatomy have its effect on improvement of oral hygiene? An assessment of oral hygiene index-simplified was performed between preoperative and postoperati...

  8. Computer-Assisted Orthognathic Surgery for Patients with Cleft Lip/Palate: From Traditional Planning to Three-Dimensional Surgical Simulation

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    Lonic, Daniel; Pai, Betty Chien-Jung; Yamaguchi, Kazuaki; Chortrakarnkij, Peerasak; Lin, Hsiu-Hsia; Lo, Lun-Jou

    2016-01-01

    Background Although conventional two-dimensional (2D) methods for orthognathic surgery planning are still popular, the use of three-dimensional (3D) simulation is steadily increasing. In facial asymmetry cases such as in cleft lip/palate patients, the additional information can dramatically improve planning accuracy and outcome. The purpose of this study is to investigate which parameters are changed most frequently in transferring a traditional 2D plan to 3D simulation, and what planning parameters can be better adjusted by this method. Patients and Methods This prospective study enrolled 30 consecutive patients with cleft lip and/or cleft palate (mean age 18.6±2.9 years, range 15 to 32 years). All patients received two-jaw single-splint orthognathic surgery. 2D orthodontic surgery plans were transferred into a 3D setting. Severe bony collisions in the ramus area after 2D plan transfer were noted. The position of the maxillo-mandibular complex was evaluated and eventually adjusted. Position changes of roll, midline, pitch, yaw, genioplasty and their frequency within the patient group were recorded as an alternation of the initial 2D plan. Patients were divided in groups of no change from the original 2D plan and changes in one, two, three and four of the aforementioned parameters as well as subgroups of unilateral, bilateral cleft lip/palate and isolated cleft palate cases. Postoperative OQLQ scores were obtained for 20 patients who finished orthodontic treatment. Results 83.3% of 2D plans were modified, mostly concerning yaw (63.3%) and midline (36.7%) adjustments. Yaw adjustments had the highest mean values in total and in all subgroups. Severe bony collisions as a result of 2D planning were seen in 46.7% of patients. Possible asymmetry was regularly foreseen and corrected in the 3D simulation. Conclusion Based on our findings, 3D simulation renders important information for accurate planning in complex cleft lip/palate cases involving facial asymmetry that is

  9. [Ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome, report of a case with variable expressivity].

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    Meza Escobar, Luis Enrique; Isaza, Carolina; Pachajoa, Harry

    2012-10-01

    The ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome is a rare entity associated with mutations in the genes that express the protein p63. We present a case of a patient with right foot ectrodactyly associated with cleft lip and palate, without other evident anomalies. The patient has a positive familiar history for cleft lift and palate and mortality during the perinatal period. The management of each case must be specific and multidisciplinary. PMID:23070194

  10. Genetics Home Reference: ankyloblepharon-ectodermal defects-cleft lip/palate syndrome

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    ... Home Health Conditions AEC syndrome ankyloblepharon-ectodermal defects-cleft lip/palate syndrome Enable Javascript to view the expand/collapse ... Open All Close All Description Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome is a form of ectodermal dysplasia, ...

  11. Patterns of some extracellular matrix gene expression are similar in cells from cleft lip-palate patients and in human palatal fibroblasts exposed to diazepam in culture

    International Nuclear Information System (INIS)

    Prenatal exposure to diazepam, a prototype sedative drug that belongs to Benzodiazepines, can lead to orofacial clefting in human newborns. By using real-time PCR, in the present study we investigated whether diazepam elicits gene expression alterations in extracellular matrix (ECM) components, growth factors and gamma-aminobutyric acid receptor (GABRB3), implicated in the coordinate regulation of palate development. Palate fibroblasts were treated with diazepam (Dz-N fibroblasts) and compared to cleft lip-palate (CLP) fibroblasts obtained from patients with no known exposure to diazepam or other teratogens. Untreated fibroblasts from non-CLP patients were used as control. The results showed significant convergences in gene expression pattern of collagens, fibromodulin, vitronectin, tenascin C, integrins and metalloprotease MMP13 between Dz-N and CLP fibroblasts. Among the growth factors, constitutive Fibroblast Growth Factor 2 (FGF2) was greatly enhanced in Dz-N and CLP fibroblasts and associated with a higher reduction of FGF receptor. Transforming Growth Factor beta 3 (TGFβ3) resulted up-regulated in CLP fibroblasts and decreased in Dz-N fibroblasts. We found phenotypic differences exhibited by Dz-N and CLP fibroblasts in GABRB3 gene regulation, so further studies are necessary to determine whether GABAergic system could be involved in the development of diazepam mediated CLP phenotype. Taken together the results elucidate the molecular mechanisms underlying possible toxicology effects induced by diazepam. Counselling of women on the safety of diazepam exposure is clinically important, also for the forensic consequences

  12. Feeling Normal? Long-Term Follow-up of Patients with a Cleft Lip-Palate after Rhinoplasty with the Derriford Appearance Scale (DAS-59).

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    Albers, Andreas E; Reichelt, Andreas C; Nolst-Trenité, Gilbert J; Menger, Dirk Jan

    2016-04-01

    The stigma of nasal deformity due to a congenital cleft lip-palate has an undeniable influence on the affected patient's life. It is therefore of interest to investigate if efforts to reduce esthetic and functional impairments by rhinoplasty (single or multiple) can result in an increased satisfaction with appearance and a self-perception similar to the noncleft population. Retrospective scoring before and after rhinoplasty using the validated Derriford Appearance Scale (DAS-59) and subsequent statistical evaluation and comparison to datasets available in the literature for further classification was used. Of the 61 patients who underwent at least one rhinoplasty, 26 responded to all questions. The mean age of responders was approximately 30 years of age and the male:female ratio was 1:1.2. The scale showed a significant overall improvement after surgery. The full scale and all subscale scores of the DAS-59 were significantly reduced after surgery demonstrating an improvement in the respective categories. Most importantly, if postoperative results were compared with a population concerned and unconcerned about appearance, no difference "facial self-consciousness" of appearance was apparent. Also postoperative subscores for "general self-consciousness" (GSC) and "social self-consciousness" of appearance (SSC) showed no difference from those obtained from the population concerned about appearance. The postoperative subscore for "sexual and bodily self-consciousness" of appearance (SBSC) indicated improvement beyond the level found in the concerned control population. Due to only a low improvement in the difference compared with the subscore representing a "negative self-concept," a statistically significant difference to the concerned population remained, possibly indicating that therapy beyond surgery is needed for improvement. After rhinoplasty, the investigated group of cleft lip-palate patients with nasal deformities showed an improvement in their self

  13. Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome: A case report of "Incomplete syndrome"

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    P K Shivaprakash; Joshi, Hrishikesh V.; Hina Noorani; Venugopal Reddy

    2012-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) is a rare syndrome having ectrodactyly, ectodermal dysplasia, and cleft lip/palate. So far, very few cases have been reported in literature. However, we report a case of incomplete EEC syndrome having ectrodactyly and cleft lip and palate with absence of signs of ectodermal dysplasia with no other systemic anomalies. Other feature noted is the syndactyly of toes which is reported rarely in this syndrome. A multidisciplinary approa...

  14. Ectrodactyly, Ectodermal dysplasia, and Cleft Lip-Palate Syndrome; Its Association with Conductive Hearing Loss

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    Robinson, Geoffrey C.; And Others

    1973-01-01

    Conductive hearing loss associated with the ectrodactyly, ectodermal dysplasia, and cleft lip palate syndrome was reported in one sporadic case and in a pedigree with four cases in three generations. (GW)

  15. Comparison of Hyoid Bone Position Among Cleft Lip Palate and Normal Subjects

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    Objective: To compare the hyoid bone position between cleft lip/palate and normal patients. Study Design: Cross-sectional analytical study. Place and Duration of Study: Dr. Ishrat-ul-Ebad Khan Institute of Oral Health Sciences (DIEKIOHS), Dow University of Health Sciences (DUHS), Karachi, from March 2012 to February 2013. Methodology: The sample for this study consisted of total 68 subjects among which Group 1 consisted of 34 nonsyndromic cleft lip and palate (both unilateral and bilateral) and Group 2 included 34 subjects with normal facial morphology. Both groups included males and females with mean age 14.3 +- 0.1 year. Analysis was done on lateral cephalometric radiographs of patients including angulations taken as Hp-Mp, Hp-Ol, Hp-Sn, Cvt-Evt, Opt-Cvt, Nke, Sn-Vert, Cvt-Vert, Sn-Vert and linear distance of hyoid bone measured as C3-H, C3-RGN, and H-RGN. Results: Statistically significant differences (p < 0.001) in C3-H, C3-Rgn, H-Rgn, and Sn-Cvt were found between cleft lip/palate group and normal group. Conclusion: Hyoid bone is located anteriorly and caudally in cleft lip and palate group. (author)

  16. Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome: A case report of "Incomplete syndrome"

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    P K Shivaprakash

    2012-01-01

    Full Text Available Ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC is a rare syndrome having ectrodactyly, ectodermal dysplasia, and cleft lip/palate. So far, very few cases have been reported in literature. However, we report a case of incomplete EEC syndrome having ectrodactyly and cleft lip and palate with absence of signs of ectodermal dysplasia with no other systemic anomalies. Other feature noted is the syndactyly of toes which is reported rarely in this syndrome. A multidisciplinary approach for treatment is needed which is co-ordinated by pedodontist or pediatrician.

  17. Cleft Lip/Palate, Short Stature, and Developmental Delay in a Boy with a 5.6-Mb Interstitial Deletion Involving 10p15.3p14

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    Gamba, Bruno F.; Rosenberg, Carla; Costa, Silvia; Richieri-Costa, Antonio; Ribeiro-Bicudo, Lucilene A.

    2015-01-01

    The chromosome interval 10p15.3p14 harbors about a dozen genes. This region has been implicated in a few well-known human phenotypes, namely HDR syndrome (hypoparathyroidism, sensorineural deafness, and renal dysplasia) and DGS2 (DiGeorge syndrome 2), but a number of variable phenotypes have also been reported. Cleft lip/palate seems to be a very unusual finding within the clinical spectrum of patients with this deletion. Here, we report a male child born with short stature, cleft lip/palate, and feeding problems who was found to have a 5.6-Mb deletion at 10p15.3p14. PMID:25852446

  18. Cleft characteristics and treatment outcomes in hemifacial microsomia compared to non-syndromic cleft lip/palate.

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    Dentino, K M; Valstar, A; Padwa, B L

    2016-06-01

    The goal of this study was to describe the clinical characteristics and treatment outcomes of patients with hemifacial microsomia (HFM) and cleft lip/palate (CL/P), and to compare them to a historic cohort of patients with non-syndromic CL/P treated at the same centre. A retrospective review of patients with HFM and CL/P was performed; the main outcome measures assessed were cleft type/side, surgical outcome, midfacial retrusion, and speech. Twenty-six patients (13 male, 13 female; mean age 22.7±14.9, range 1-52 years) with cleft lip with/without cleft palate (CL±P) were identified: three with cleft lip (12%), two with cleft lip and alveolus and an intact secondary palate (8%), and 21 with cleft lip and palate (CLP) (81%; 15 unilateral and six bilateral). Four patients (19%) had a palatal fistula after palatoplasty. Twelve of 22 patients aged >5 years (55%) had midfacial retrusion and two (9%) required a pharyngeal flap for velopharyngeal insufficiency (VPI). Fisher's exact test demonstrated a higher frequency of complete labial clefting (P=0.004), CLP (P=0.009), midfacial retrusion (P=0.0009), and postoperative palatal fistula (P=0.03) in HFM compared to non-syndromic CL±P. There was no difference in VPI prevalence. This study revealed that patients with HFM and CL±P have more severe forms of orofacial clefting than patients with non-syndromic CL±P. Patients with HFM and CL±P have more severe midfacial retrusion and a higher palatal fistula rate compared to patients with non-syndromic CL±P. PMID:26775633

  19. Effect of cleft lip palate repair on craniofacial growth

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    Naqvi, Zuber Ahamed; Shivalinga, BM; Ravi, S; Munawwar, Syeda Sarah

    2015-01-01

    Objective: The aim of this cross-sectional study was to compare craniofacial growth among operated and unoperated unilateral cleft lip and palate non-syndromic subjects. Materials and Methods: A sample of 180 subjects of Indian origin was selected. Of them, 90 were operated, and 90 were unoperated complete unilateral cleft lip and palate individuals. The subjects were divided into three age groups of 3–5, 8–10, and 20–25 years comprised of 30 patients in each group. The following measurements were evaluated: Angle and length of the cranial base; maxillary spatial positioning and length; mandibular spatial positioning; morphology and length; maxillomandibular relationship. Comparative analysis of the means between the groups was performed with Student's t-test at the significance levels of 5%. The ANOVA test has been performed to test the effect of time. Results: No significant differences were observed between the measurements that represented the angle and length of the cranial base of unoperated and the operated patients (P>0.05). There was statistically significant decrease (P˂0.05) in the maxillary length (Co-A; 69.00 mm in 3–5 years, 68.33 mm in 8–10 years, and 67.17 mm in 20–25 years age group), and SNA angle (74.83° in 3–5 years, 74.17 ° in 8–10 years and 73.17 ° in 20–25 years age group) in operated group. No significant difference noticed on cephalometric values of the mandible, except Ar-Go-Me angle (P˂0.05), which showed vertical growth pattern in unoperated patients (132.50 ° in 3–5 years, 132.00 ° I 8–10 years and 138.33 ° in 20–25 years age group). Conclusion: Lip and palate repair has a significant influence on the maxilla and resulting in retarded growth of maxilla, which causes midface deficiency beyond acceptable sagittal limits. The Gonial angle showed vertical growth pattern in unoperated patients, but the cranial base angle and length of unoperated and the operated patients were similar. PMID:26229945

  20. Novel FGFR1 and KISS1R Mutations in Chinese Kallmann Syndrome Males with Cleft Lip/Palate

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    Xu, Hao; Niu, Yonghua; Wang, Tao; Liu, Simin; Xu, Hua; Wang, Shaogang; Liu, Jihong; Ye, Zhangqun

    2015-01-01

    Kallmann syndrome (KS) is characterized by isolated hypogonadotropic hypogonadism (IHH) with anosmia and is sometimes associated with cleft lip/palate (CLP). In order to describe the clinical features, genetic etiology, and treatment outcome of KS males with CLP, we performed genetic screening for 15 known causal IHH genes (KAL1, FGFR1, NELF, FGF8, CHD7, WDR11, SEMA3A, KISS1R, KISS1, PROKR2, PROK2, TAC3, TACR3, GNRH1, and GNRHR) in four KS with CLP patients and six IHH patients without CLP. Two novel heterozygous missense mutations in FGFR1, (NM_001174066): c.776G>A (p.G259E) and (NM_001174066): c.358C>T (p.R120C), were identified in a 23-year-old KS male with cleft lip and an 18-year-old KS patient with cleft lip and palate, dental agenesis, and high arched palate, respectively. These two mutations were not presented in their healthy parents and 200 normal controls. One novel heterozygous missense mutation in KISS1R, (NM_032551): c.587C>A (p.P196H), was identified in an 18-year-old KS male with cleft lip and dental agenesis who developed sperm after being treated with gonadotropin. This mutation was also presented in his healthy father and grandfather. These results have implications for the diagnosis, genetic counseling, and treatment of KS and CLP males with mutations in FGFR1 gene. PMID:26199944

  1. A rare case of sirenomelia with total bilateral cleft lip palate and kyphoscoliosis

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    Sangeeta Arya

    2013-04-01

    Full Text Available Sirenomelia, a rare congenital deformity, has fused lower limbs with variable combination of visceral anomalies. This condition is fatal within a day or two of birth so early antenatal detection and pregnancy termination is advocated. Antenatally it can be diagnosed by ultrasonography showing oligohydramnios, renal agenesis and fibulae positioned between the tibiae. A 24 years old Muslim primigravida came for first antenatal check up at 18 weeks of pregnancy. She didn’t turn up and came directly in emergency at 37 weeks in active labor and delivered a baby weighing 1.8 kg with a small sized placenta, complete bilateral cleft lip palate, Kyphoscoliosis and fused lower limbs upto ankles. Baby expired after 25 minutes of birth. Till now around 300 cases of Sirenomelia have been reported worldwide. All human cases of Sirenomelia have been found to be associated with variable degree of renal and urethral dysplasia or renal agenesis. External genitalia are affected, whereas gonads remain unaffected. In rare cases Sirenomelia is associated with upper body defects including cleft palate, cervical and upper thoracic vertebral abnormalities, pulmonary hypoplasia and cardiac defects. [Int J Reprod Contracept Obstet Gynecol 2013; 2(2.000: 242-244

  2. Risk variants in BMP4 promoters for nonsyndromic cleft lip/palate in a Chilean population

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    Suazo José

    2011-12-01

    Full Text Available Abstract Background Bone morphogenetic protein 4 gene (BMP4 plays a key role during maxillofacial development, since orofacial clefts are observed in animals when this gene is conditionally inactivated. We recently reported the existence of association between nonsyndromic cleft lip/palate (NSCLP and BMP4 polymorphisms by detecting transmission deviations for haplotypes that include a region containing a BMP4 promoter in case-parent trios. The aim of the present study was to search for possible causal mutations within BMP4 promoters (BMP4.1 and BMP4.2. Methods We analyzed the sequence of BMP4.1 and BMP4.2 in 167 Chilean NSCLP cases and 336 controls. Results We detected three novel variants in BMP4.1 (c.-5514G > A, c.-5365C > T and c.-5049C > T which could be considered as cleft risk factors due to their absence in controls. Additionally, rs2855530 G allele (BMP4.2 carriers showed an increased risk for NSCLP restricted to males (OR = 1.52; 95% C.I. = 1.07-2.15; p = 0.019. For this same SNP the dominant genotype model showed a higher frequency of G/G+G/C and a lower frequency of C/C in cases than controls in the total sample (p = 0.03 and in the male sample (p = 0.003. Bioinformatic prediction analysis showed that all the risk variants detected in this study could create new transcription factor binding motifs. Conclusions The sex-dependent association between rs2855530 and NSCLP could indirectly be related to the differential gene expression observed between sexes in animal models. We concluded that risk variants detected herein could potentially alter BMP4 promoter activity in NSCLP. Further functional and developmental studies are necessary to support this hypothesis.

  3. Comparison of videonasoendoscopy and auditory-perceptual evaluation of speech in individuals with cleft lip/palate

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    Paniagua, Lauren Medeiros

    2014-01-01

    Full Text Available Introduction: The velopharyngeal sphincter (VPS is a muscle belt located between the oropharynx and the nasopharynx. Investigations of velopharyngeal function should include an auditory-perceptual evaluation and at least 1 instrument-based evaluation such as videonasoendoscopy. Aim:To compare the findings of auditory-perceptual evaluation (hypernasality and videonasoendoscopy (gap size in individuals with cleft lip/palate. Method: This was a retrospective, cross-sectional study assessing 49 subjects, of both sexes, with cleft lip/palate followed up at the Otorhinolaryngology Service and the Speech Therapy outpatient clinic of Hospital de Clínicas de Porto Alegre (HCPA. The results from the auditory-perceptual evaluation and the videonasoendoscopy test were compared with respect to the VPS gap size. Results: Subjects with moderate/severe hypernasality had more severe velopharyngeal closure impairment than those with a less severe condition. The interaction between hypernasality severity and the presence of other speech disorders (p = 0.035, whether compensatory and/or obligatory, increased the likelihood of having a moderate-to-large gap in the velopharyngeal closure. Conclusions: We observed an association between the findings of these 2 evaluation methods.

  4. Speech Analysis of Bengali Speaking Children with Repaired Cleft Lip & Palate

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    Chakrabarty, Madhushree; Kumar, Suman; Chatterjee, Indranil; Maheshwari, Neha

    2012-01-01

    The present study aims at analyzing speech samples of four Bengali speaking children with repaired cleft palates with a view to differentiate between the misarticulations arising out of a deficit in linguistic skills and structural or motoric limitations. Spontaneous speech samples were collected and subjected to a number of linguistic analyses…

  5. Siamese twins with craniofacial duplication and bilateral cleft lip/palate in a ceramic representation of the Chimú culture (Peru): a comparative analysis with a current case.

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    Pachajoa, Harry; Hernandez-Amaris, Maria F; Porras-Hurtado, Gloria Liliana; Rodriguez, Carlos A

    2014-06-01

    Craniofacial duplication or diprosopus is a very rare malformation that is present in approximately 0.4% of conjoined twins. Here is presented a case of craniofacial duplication in association with bilateral cleft lip/palate in both heads found in a ceramic representation from the early Chimú culture from Peru. A comparative analysis is made with a current case of a 28-week-old fetus with similar characteristics. After reviewing the medical literature on conjoined twins, very few reports of facial cleft in both twins were found, with no reports at all of bilateral cleft lip/palate. This ceramic crock is considered one of the first representations suggestive of craniofacial duplication, and probably the first reporting it in association with facial cleft. PMID:24774755

  6. Cleft Lip and Palate Surgery

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    ... The experts in face, mouth and jaw surgery. Cleft Lip / Palate and Craniofacial Surgery This type of surgery is ... the carefully orchestrated, multiple-stage correctional program for cleft lip and palate patients. The goal is to help restore the ...

  7. Epidemiology of non-syndromic cleft lip/palate in the high level natural background radiation areas (HLNRA) of the South west coast of India

    International Nuclear Information System (INIS)

    All consecutive births in selected government hospitals in and around the high level natural background radiation areas (HLNRA) of Kerala were monitored for congenital malformations observable at birth since 1995. The HLNR area, with natural deposits of monazite sand containing thorium (8-10%) and Uranium (0.3%), is a coastal strip of land about 55 km in length and 0.5 km in breadth from Purakkad in the north in Alleppey district to Sakthikulangara in the south of Quilon district and is one among the most prominent background radiation areas of the world. Patchy and non-uniform distribution of Monazite sand causes wide variation in dose ranging from <1 to 45 mGy/year. High population density, limited migration, ethnic diversity, good literacy, health awareness, institutionalized births and acceptance of small family norm are some of the key features of the population. Areas with a mean radiation dose of more than 1.5 mGy/year were treated as HLNR areas and areas with a dose level of 1.5 mGy/year or less were treated as normal level radiation areas (NLNRA). A total of 134,178 newborns were monitored and non-syndromic cleft lip/palate (NSCLP) was detected in 143 newborns (1.07%). Cleft lip with cleft palate (59.4%) was more common than cleft lip (18.2%) or Cleft palate (22.4%) alone and 11.9% had other malformation(s) together with NSCLP. There was no evidence to suggest that NSFC was significantly associated with prevailing dose level of area of mother's residence, maternal age birth, gravida, ethnicity or consanguinity

  8. How various surgical protocols of the unilateral cleft lip and palate influence the facial growth and possible orthodontic problems? Which is the best timing of lip, palate and alveolus repair? literature review.

    Science.gov (United States)

    Farronato, Giampietro; Kairyte, Laima; Giannini, Lucia; Galbiati, Guido; Maspero, Cinzia

    2014-01-01

    OBJECTIVES. Cleft lip palate is congenital growth disease with unknown etiology, probably linked to both genetically and external causes. The aim of this work consists in presenting the effects of these diseases on cranio facial growth and the surgical protocols described in literature. MATERIALS AND METHODS. The literature review articles conducted by Medline ranged from 1998 to 2011 have been selected. The key words of the research were "cleft lip palate", "cleft lip palate facial growth", "cleft lip palate surgery". The inclusion criteria were articles that analyzed surgical protocols and the growth of unilateral lip and palate clefts, the timing repair of lip, palate and alveolus. We excluded case reports, studies without control group in the sample and the other types of publication as thesis or conference presentation. 60 articles had the selection criteria of the research. RESULTS. The cleft lip and palate is one of the most common birth defects that needs long rehabilitation between birth and adulthood. Several authors have presented surgical protocols and timing. The effects of these diseases on cranio facial growth and the importance of the early intervention have been described. CONCLUSIONS. The review describes the main surgical protocols and treatment strategies of the unilateral lip and palate clefts. The review discusses how surgery effects the midfacial skeletal growth. Studies agree that the palate repair is the main cause of the maxilla growth disturbances. About the timing of palate repair in the unilateral clefts it can be concluded that most studies found no difference between one or two stages palate repair techniques for the midfacial growth. Also from the research, studies agree that delayed hard palate repair has more positive effects on maxillary growth than that of early hard palate repair. Nevertheless good results, delayed hard palate repair technique is abandoned by many hospital centres because of worse speech outcome. The best

  9. Case report: unusual dental morphology in a child with ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome.

    LENUS (Irish Health Repository)

    Fitzgerald, K

    2012-02-01

    BACKGROUND: Anomalies of dental anatomy are common in the ectodermal dysplasia syndromes. These anomalies, when found in combination with dental caries, can pose a restorative challenge for the paediatric dentist. Modification of traditional techniques and approaches may help the practitioner provide a successful treatment outcome. CASE REPORT: A 3 years and 11 months old girl with a diagnosis of ankyloblepharon-ectodermal dysplasiacleft lip\\/palate (AEC) syndrome was referred for treatment to a specialist paediatric dental service. Her abnormal dental anatomy, hypodontia and dental caries formed a triad of challenges for the team. Under general anaesthesia, her dentition was restored using a combination of restorative approaches and techniques, including the placement of both composite resin and preformed metal crown restorations. FOLLOW-UP: At 18-month followup, the family had successfully implemented good home care and dietary practices, and the local dental service had instituted a preventive programme consisting of regular examination, advice and fluoride varnish placement. The restorations remained intact and no further caries was detected. At 24-month follow-up, the first permanent molars were partially erupted, and displayed unusually deep fissures. There was also a degree of ectopic eruption of the first permanent molars, and possibly of one of the maxillary permanent incisors. CONCLUSION: Dental care for children with AEC syndrome is optimised by early intervention, good home care and regular professional review. Dental care providers should be aware of the possibility of complex dental anatomy, and bear this in mind should it become necessary to formulate a restorative treatment plan.

  10. A Comparative Study of Facial Asymmetry in Philippine, Colombian, and Ethiopian Families with Nonsyndromic Cleft Lip Palate

    Directory of Open Access Journals (Sweden)

    Liliana Otero

    2012-01-01

    Full Text Available Objective. To compare the asymmetry displayed by Philippine, Colombian, and Ethiopian unaffected parents of patients with nonsyndromic cleft palate (NSCLP and a control population. Methods. Facial measurements were compared between unaffected parents of NSCLP patients and those in the control group for three populations from South America, Asia, and Africa by anthropometric and photographic measurements. Fluctuating and directional asymmetries, height and width proportions, were analyzed and compared. Results. Fluctuating asymmetries (ear length, middle line to Zigion perpendicular for left and right sides and variations in the facial thirds demonstrated statistical significance in the study group of unaffected parents from Colombia and Philippines, while increased interorbital distance was evident in the unaffected Ethiopian parents of NSCLP patients. Conclusions. The facial differences in unaffected parents could indicate an underlying genetic liability. Identification of these differences has relevance in the understanding of the etiology of NSCLP.

  11. Post-surgical dentofacial deformities and dental treatment needs in cleft-lip-palate children: A clinical study

    OpenAIRE

    V. Krishna Priya; J Sharada Reddy; Ramakrishna, Y.; C Pujita Reddy

    2011-01-01

    Background: Cleft lip and palate is a common congenital defect. It is one of the most common facial deformities occurring in major racial and ethnic groups. Aims: The aim of the present study was to record the post-surgical dentofacial deformities in operated cleft lip and palate children, as well as to assess the multitude and magnitude of their dental and other related problems so as to formulate an appropriate treatment plan for complete oral rehabilitation of these children. Materials and...

  12. Oral health in 4-6 years children with cleft lip/palate: A case control study

    Directory of Open Access Journals (Sweden)

    Amandeep Chopra

    2014-01-01

    Full Text Available Background: Oro-facial clefts are a major public health problem. Children with clefts rarely escape dental complications. Aims: This study was to determine differences in the dental caries experience, gingival health, and prevalence malocclusion, enamel defects and oral mucosal lesions among 4-6 year old children with and without cleft in Panchkula. Materials and Methods: The sampling frame consisted of 4-6 year old children with clefts visiting Swami Devi Dyal Hospital and Dental College, Panchkula, India. As a control group an age (±3 months and gender-matched sample from the same geographical areas were recruited. Dental caries status, gingival health status, developmental defect of enamel, malocclusion and oral mucosal health were assessed and compared between the two groups. Results: Significant differences in dental caries and gingival health status were found between children with and without cleft. Anterior open-bite, increased overjet and oral mucosal lesions (P 0.05. Conclusion: Differences of oral health status exist among 4-6 year old children with and without clefts. Children fare worse in terms of dental caries, gingival health, oral mucosal health and malocclusion.

  13. Longitudinal study of growth of children with unilateral cleft-lip palate from birth to two years of age

    Science.gov (United States)

    Marques, Ilza L.; Nackashi, John A.; Borgo, Hilton C.; Martinelli, Ângela P. M. C.; Pegoraro-Krook, Maria I.; Williams, William N.; Dutka, Jeniffer C. R.; Seagle, Michael B.; Souza, Telma V.; Garla, Luis A.; Neto, José S. M.; Silva, Marcos L. N.; Graciano, Maria I. G.; Moorhead, Jacquelyn; A Piazentin-Penna, Sílvia H.; Feniman, Mariza R.; Zimmermann, Maria C.; Bento-Gonçalves, Cristina G. A.; Pimentel, Maria C. M.; Boggs, Steve; Jorge, José C.; Antonelli, Patrick J.; Shuster, Jonathan

    2009-01-01

    Objective To study the growth of children with complete unilateral cleft lip and palate (UCLP) from birth to 2 years of age and to construct specific UCLP growth curves. Design Physical growth was a secondary outcome measure of a NIH sponsored longitudinal, prospective clinical trial involving the University of Florida (USA) and the University of São Paulo (Brazil). Patients 627 children with UCLP, nonsyndromic, both genders. Methods Length, weight, and head circumference were prospectively measured for a group of children enrolled in a clinical trial. Median growth curves for the 3 parameters (length, weight, head circumference) were performed and compared to the median for the National Center for Health Statistics (NCHS, 2000) curves. The median values for length, weight, and head circumference at birth, 6, 12, 18 and 24 month of age were plotted against NCHS median values, and statistically compared at birth and 24 months. Setting Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru, Brazil (HRAC-USP). Results At birth, children of both genders with UCLP presented with smaller body dimensions in relation to NCHS median values, but the results suggest a “catch up growth” for length, weight, and head circumference for girls and for weight (to some degree) and head circumference for boys. Conclusions Weight was the most compromised parameter for both genders followed by length and then head circumference. There was no evidence of short statue. This study established growth curves for children with UCLP. PMID:19860503

  14. A novel c.1037C > G (p.Ala346Gly) mutation in TP63 as cause of the ectrodactyly-ectodermal dysplasia and cleft lip/palate (EEC) syndrome

    OpenAIRE

    Leandro Ucela Alves; Eliete Pardono; Otto, Paulo A.; Regina Célia Mingroni Netto

    2014-01-01

    Ectrodactyly – ectodermal dysplasia and cleft lip/palate (EEC) syndrome (OMIM 604292) is a rare disorder determined by mutations in the TP63 gene. Most cases of EEC syndrome are associated to mutations in the DNA binding domain (DBD) region of the p63 protein. Here we report on a three-generation Brazilian family with three individuals (mother, son and grandfather) affected by EEC syndrome, determined by a novel mutation c.1037C > G (p.Ala346Gly). The disorder in this family exhibits a broad ...

  15. Hereditary effects of low and chronic radiation exposure: a case-control study of mental retardation and cleft lip/palate in the monazite bearing coastal areas of Southern Kerala

    International Nuclear Information System (INIS)

    The high level natural radiation (HLNR) areas of Kerala in the south west coast of Kerala is suitable for direct human studies to assess the genetic and teratogenic effect of low and chronic radiation exposures. The radiation level in the region (about 55 km long and 0.5 km wide belt) range from ≤1.0 mGy to 45.0 mGy per year and is due to natural deposit of monazite sand containing Thorium (8-10%), Uranium (0.3%) and corresponding decay products. A population based case-control study was carried out in the area to assess the role, if any, of HLNR as a causative factor in the etiology of two relatively frequent and easily identifiable congenital malformations, viz., mental retardation (MR) and cleft lip/palate (CLP). Findings from the study are discussed in this paper

  16. Effects of an early psychological intervention on parents of children with cleft lip/palate%早期心理干预对唇腭裂患儿父母的影响

    Institute of Scientific and Technical Information of China (English)

    王杨洋; 信燕华; 马坚; 辛秀红; 石冰; 黄永清

    2013-01-01

    目的 对唇腭裂患儿父母的心身健康状况进行评估并进行早期心理干预,探讨早期心理干预对唇腭裂患儿父母的影响.方法 采用症状自评量表(SCL-90)对102名唇腭裂患儿的父亲或母亲在入院当天进行调查,在住院期间及出院后3个月内定期地进行心理干预,并于出院当天及3个月后再进行问卷调查.以126名正常儿童的父亲或母亲作为对照组.结果 病例组患儿父母在躯体化、强迫症状、抑郁、焦虑等9个维度上分值均显著高于对照组(P<0.05);病例组3个亚组(唇裂组、腭裂组、唇腭裂组)间差异无统计学意义(P>0.05);病例组出院当天与入院时的统计结果差异无统计学意义(P>0.05),出院后3个月与入院时的统计结果有显著差异(P<0.05).结论 唇腭裂患儿父母的心身健康状况较差,早期心理干预对唇腭裂患儿家长具有重大影响,而且对患儿的心身健康也具有积极的意义.%Objective To provide basis for effects of an early psychological intervention on parents of children with cleft lip/palate,and investigate the effects of an early psychological intervention to them.Methods One selfadministered questionnaire (SCL-90) was applied in 102 parents of children with cleft lip/palate,compared to 126 parents of healthy individuals on the day of admission.They were given the psychological intervention during hospitalization and 3 months after discharge.The questionnaire (SCL-90) was again applied to them on the day of discharge and 3 months after discharge.Results Using the questionnaire(SCL-90),the answer scores of somatization,obsessivecompulsive,depression and anxiety etc.were significantly higher than those of the control group (P<0.05).There were no statistical differences during 3 subgroups in the case group (P>0.05).There were no statistical differences on the day of admission and on the day of discharge (P>0.05),but there were statistical difference on the day

  17. A Rare Interstitial Duplication of 8q22.1–8q24.3 Associated with Syndromic Bilateral Cleft Lip/Palate

    Directory of Open Access Journals (Sweden)

    Regina Ferreira Rezek

    2014-01-01

    Full Text Available We present a rare case of 8q interstitial duplication derived from maternal balanced translocations in a patient with bilateral cleft lip and palate in syndromic form associated with other congenital malformations. G-banding cytogenetic analysis revealed a chromosomal abnormality in the form of the karyotype 46,XX der(22t(8;22(q22.1;p11.1mat. Chromosome microarray analysis evidenced a 49 Mb duplicated segment of chromosome 8q with no pathogenic imbalances on chromosome 22. Two siblings also carry the balanced translocation. We have compared this case with other “pure” trisomies of 8q patients reported in the literature and with genome wide association studies recently published. This work highlights the involvement of chromosome 8q in orofacial clefts.

  18. 唇腭裂患儿父母心身健康状况调查与思考%A study on the psychosomatic states of parents of children with cleft lip/palate

    Institute of Scientific and Technical Information of China (English)

    王杨洋; 翟堃; 信燕华; 马坚; 黄永清; 辛秀红

    2012-01-01

    目的:了解唇腭裂患儿父母躯体及心理健康状况,为其进行心理治疗提供依据.方法:采用康奈尔医学指数(CMI)、家庭功能量表(FAD)及社会支持评定量表(SSRS)对102名唇腭裂患者的父亲或母亲及126名正常儿童的父亲或母亲进行调查,以了解唇腭裂患儿父母的躯体表现特征、心理健康状况及父母所面对的家庭、社会方面问题.结果:CMI中,病例组父母在躯体性疾病如眼和耳、心血管系统等方面以及在精神症状方面如不适应、抑郁等方面各因子分值均显著高于对照组(P<0.05);FAD及SSRS中,病例组父母各因子分值显著高于对照组(P<0.01).结论:唇腭裂患儿的父母心身健康状况较差,所获得的家庭及社会支持较少,应给予足够的重视并积极开展心理干预.%Objective: To provide basis for the emotional support and psychological therapy for parents of the children with cleft lip/pa late. Methods; Three self-administered questionnaires ( CMI, FAD and SSRS) were applied in 102 parents (experimental group) of the children with cleft lip/palate and 126 parents(controls) with healthy children respectively. Results:Using the instrument of CMI, the answer scores of somatization such as eye and ear, cardiovascular system etc. And the answer scores of mental symptoms such as maladjustment, depression etc. In the experimental group were significantly higher than those in the control (P <0. 05). Using the instruments of FAD and SSRS, the factor scores in the experimental group were significantly higher than those in the control ( P < 0. 01). Conclusion; The psychosomatic states of parents of children with cleft lip/palate is poor, and they get little support from family and society. They should be given active reconstruction and intervening.

  19. Cleft Lip and Cleft Palate

    Science.gov (United States)

    ... side. There are three primary types of clefts: • Cleft lip/palate refers to the condition when both the palate ... one in 1,000 babies are born with cleft lip/palate. About 50 percent of all clefts More common ...

  20. A novel c.1037C > G (p.Ala346Gly) mutation in TP63 as cause of the ectrodactyly-ectodermal dysplasia and cleft lip/palate (EEC) syndrome.

    Science.gov (United States)

    Alves, Leandro Ucela; Pardono, Eliete; Otto, Paulo A; Mingroni Netto, Regina Célia

    2015-03-01

    Ectrodactyly - ectodermal dysplasia and cleft lip/palate (EEC) syndrome (OMIM 604292) is a rare disorder determined by mutations in the TP63 gene. Most cases of EEC syndrome are associated to mutations in the DNA binding domain (DBD) region of the p63 protein. Here we report on a three-generation Brazilian family with three individuals (mother, son and grandfather) affected by EEC syndrome, determined by a novel mutation c.1037C > G (p.Ala346Gly). The disorder in this family exhibits a broad spectrum of phenotypes: two individuals were personally examined, one presenting the complete constellation of EEC syndrome manifestations and the other presenting an intermediate phenotype; the third affected, a deceased individual not examined personally and referred to by his daughter, exhibited only the split-hand/foot malformation (SHFM). Our findings contribute to elucidate the complex phenotype-genotype correlations in EEC syndrome and other related TP63-mutation syndromes. The possibility of the mutation c.1037C > G being related both to acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome and SHFM is also raised by the findings here reported. PMID:25983622

  1. A novel c.1037C > G (p.Ala346Gly mutation in TP63 as cause of the ectrodactyly-ectodermal dysplasia and cleft lip/palate (EEC syndrome

    Directory of Open Access Journals (Sweden)

    Leandro Ucela Alves

    2015-03-01

    Full Text Available Ectrodactyly – ectodermal dysplasia and cleft lip/palate (EEC syndrome (OMIM 604292 is a rare disorder determined by mutations in the TP63 gene. Most cases of EEC syndrome are associated to mutations in the DNA binding domain (DBD region of the p63 protein. Here we report on a three-generation Brazilian family with three individuals (mother, son and grandfather affected by EEC syndrome, determined by a novel mutation c.1037C > G (p.Ala346Gly. The disorder in this family exhibits a broad spectrum of phenotypes: two individuals were personally examined, one presenting the complete constellation of EEC syndrome manifestations and the other presenting an intermediate phenotype; the third affected, a deceased individual not examined personally and referred to by his daughter, exhibited only the split-hand/foot malformation (SHFM. Our findings contribute to elucidate the complex phenotype-genotype correlations in EEC syndrome and other related TP63-mutation syndromes. The possibility of the mutation c.1037C > G being related both to acro-dermato-ungual-lacrimal-tooth (ADULT syndrome and SHFM is also raised by the findings here reported.

  2. Is an isolated cleft lip an isolated anomaly?

    NARCIS (Netherlands)

    Deelder, J. D.; Breugem, C. C.; de Vries, Iris; de Bruin, M.; van der Molen, A. B. Mink; van der Horst, C. M. A. M.

    2011-01-01

    Introduction: It is well known that patients with cleft lip/palate or cleft palate can have associated anomalies. However, there is a relative paucity of information about the possible anomalies associated with an isolated cleft lip. A recent study (Vallino et al., 2008) showed that children with cl

  3. Recurrence of split hand/foot malformation, cleft lip/palate, and severe urogenital abnormalities due to germline mosaicism for TP63 mutation.

    Science.gov (United States)

    Enriquez, Annabelle; Krivanek, Michael; Flöttmann, Ricarda; Peters, Hartmut; Wilson, Meredith

    2016-09-01

    We describe two sibling fetuses with urogenital abnormalities detected by prenatal ultrasound, in which post-delivery examination showed split hand and foot malformation, and bilateral cleft lip and palate. These findings are consistent with ectrodactyly-ectodermal dysplasia-cleft lip with or without cleft palate syndrome (EEC). Both fetuses were found to have the same missense mutation in TP63 (c.1051G > A; p.D351N). Parental clinical examinations and lymphocyte DNA analyses were normal. This report illustrates the potential severity of urogenital defects in TP63-related disorders, which may be detectable with fetal ultrasonography. It highlights the need to counsel for the possibility of germline mosaicism in TP63-associated disorders. © 2016 Wiley Periodicals, Inc. PMID:27351625

  4. Current Training and Continuing Education Needs of Preschool and School-Based Speech-Language Pathologists regarding Children with Cleft Lip/Palate

    Science.gov (United States)

    Bedwinek, Anne P.; Kummer, Ann W.; Rice, Gale B.; Grames, Lynn Marty

    2010-01-01

    Purpose: The purpose of this study was to obtain information regarding the education and experience of preschool and school-based speech-language pathologists (SLPs) regarding the assessment and treatment of children born with cleft lip and/or palate and to determine their continuing education needs in this area. Method: A 16-item mixed-methods…

  5. Técnicas cirúrgicas correntes para fissuras lábio-palatinas, em Minas Gerais, Brasil Current surgical techniques for cleft lip-palate in Minas Gerais, Brazil

    Directory of Open Access Journals (Sweden)

    Lívia Maris Ribeiro Paranaíba

    2009-12-01

    Full Text Available Fissuras do lábio e/ou palato (FL/P representam as anomalias congênitas crânio-facial mais comuns. OBJETIVO: Avaliar as técnicas cirúrgicas correntes na reabilitação de FL/P em um Serviço de referência no Estado de Minas Gerais. MATERIAL E MÉTODOS: Realizou-se estudo retrospectivo, 2002 a 2007, avaliando 109 portadores de FL/P não sindrômicas que tiveram o tratamento concluído. As dimensões de análise (identificação pessoal, classificação das FL/P e tratamento cirúrgico realizado foram obtidas a partir dos prontuários, sendo posteriormente construído banco de dados e as análises estatísticas realizadas pelo programa SPSS 13.0. Seguiu-se análise descritiva dos procedimentos cirúrgicos em função do tipo de FL/P encontrada. RESULTADOS: Entre os 109 pacientes, 65,1% foram do gênero masculino e 34,8% do feminino. Verificou-se que 45% dos pacientes apresentaram fissuras lábio-palatinas, 37,6% fissuras labiais e 17,4% fissuras palatinas. As técnicas correntes empregadas foram as de Millard e Spina para as queiloplastias, McComb para as rinoplastias e as de Veau e Van Langenbeeck para as palatoplastias. CONCLUSÃO: Este estudo é o primeiro a abordar reabilitação em FL/P em Minas Gerais. Nas FL/P unilaterais houve a associação das técnicas de McComb, Veau e Millard, respectivamente, para rinoplastia, palatoplastia e queiloplastia, em 76,9% dos pacientes.Cleft lip and palate (CL/P are the most common congenital anomalies of the craniofacial region. AIM: to evaluate the surgical techniques used in CL/P treatment in a craniofacial deformities ward, in Minas Gerais. MATERIALS AND METHODS: In this retrospective study, carried out between 2002 and 2007, we studied 109 individuals with non-syndromic CL/P submitted to treatment. The aspects analyzed (personal identification, classification of CL/P and surgical treatment performed were obtained from patient charts, and then we built a database and ran statistical analyses

  6. Prenatal diagnosis of cleft lip/palate: The surface rendered oro-palatal (SROP) view of the fetal lips and palate, a tool to improve information-sharing within the orofacial team and with the parents.

    Science.gov (United States)

    Levaillant, Jean-Marc; Nicot, Romain; Benouaiche, Laurence; Couly, Gérard; Rotten, Daniel

    2016-07-01

    The ultrasonographic surface rendered oro-palatal (SROP) view is a 3D reconstructed view of the fetal perioral region, which combines ultrasound insonation in a trans oral, upward directed axial direction and the surface rendered mode. It allows the simultaneous visualization on a single scan of the superior lip, alveolar ridge and secondary palate. It corresponds prenatally to the submental intra oral photography of the palate of neonates. The aim of the study was to demonstrate the benefice of using the SROP view in the management of cleft lip with or without cleft palate, uni- or bi-lateral, diagnosed prenatally (22-28 gestational weeks). The SROP view allowed the representation on a single view of the characteristics of the defect useful to the different members of the orofacial team to exactly evaluate the difformity and to plan the ulterior therapeutic steps (e.g. side, extension of the cleft to the secondary palate, tooth organization). Also, being easier to read by lay people thanks to the use of a surface rendered representation rather than the usual multiplanar reconstructions in the three traditional orthogonal planes, the SROP view makes it easier to bring exact information to the parents about the malformation and its consequences. PMID:27211349

  7. Avaliação da idade materna, paterna, ordem de paridade e intervalo interpartal para fissura lábio-palatina Maternal and paternal age, birth order and interpregnancy interval evaluation for cleft lip-palate

    Directory of Open Access Journals (Sweden)

    Daniella Reis Barbosa Martelli

    2010-02-01

    Full Text Available Fissuras do lábio e/ou palato representam as anomalias congênitas craniofaciais mais comuns. OBJETIVO: Avaliar fatores de risco ambientais em pacientes com fissuras lábio-palatinas não-sindrômicas, em um Serviço de Minas Gerais. CASUÍSTICA E MÉTODO: Realizou-se estudo caso-controle, avaliando 100 crianças com fissuras e 100 crianças sem alterações clínicas. As dimensões de análise (idade, cor de pele, sexo, classificação das fissuras, idade materna e paterna, ordem de paridade e intervalo interpartal foram obtidas a partir de um questionário, sendo posteriormente construído banco de dados e as análises realizadas pelo programa SPSS 17.0. Os resultados foram analisados com risco relativo para cada variável, para estimar odds ratios com intervalo de confiança de 95% seguido de análise bivariada e multivariada. RESULTADOS: Entre as 200 crianças, 54% foram do sexo masculino e 46% do feminino. Com relação à cor da pele, houve predomínio de parda, branca e preta, respectivamente. Entre os tipos de fissuras, as mais comuns foram as fissuras lábio-palatinas (54%, seguidas pela fissura labial (30% e fissura palatina (16%. CONCLUSÃO: Embora com uma população limitada, verificou-se associação entre idade materna e risco aumentado para fissuras lábio-palatinas, porém idade paterna, ordem de paridade e intervalo interpartal não foram significantes.Cleft lip and palate (CL/P are the most common congenital craniofacial anomalies. AIM: To evaluate environmental risk factors for non-syndromic CL/P in a reference care center in Minas Gerais. MATERIALS AND METHODS: we carried out a case-controlled study, assessing 100 children with clefts and 100 children without clinical alterations. The analysis dimensions (age, skin color, gender, fissure classification, maternal and paternal age, birth order and interpregnancy interval, obtained from a questionnaire; and later we build a data base and the analyses were carried out by the

  8. MSX 1 gene variant and non-syndromic clefting: Association or rejection?

    OpenAIRE

    Naveen Admala Reddy; Adusumilli Gopinath; JayaprakashThirumala Reddy; Raghu Devanna; Pichai Saravanan; Rohra, Mayur G

    2014-01-01

    Context: Non-syndromic cleft lip/palate (NSCL/P) is a congenital anomaly with significant medical, psychological and social ramifications. There is sufficient evidence to hypothesize that locus for this condition can be identified by candidate genes. Aims: The aim of this study is to amplify the chosen region (799 G >T) of MSX 1 gene, investigate the degree of association and perform a mutation research from Raichur cleft lip and palate patient sample. Settings and Design: Case history...

  9. What is the Risk of Having Offspring with Cleft Lip/Palate in Pre-Maternal Obese/Overweight Women When Compared to Pre-Maternal Normal Weight Women? A Systematic Review and Meta-Analysis

    Science.gov (United States)

    Izedonmwen, Omoroghogho Maria; Cunningham, Claudia

    2015-01-01

    ABSTRACT Objectives The purpose of the study was to identify the risk of orofacial cleft in the offspring of women with pre-maternal obesity/overweight when compared with pre-maternal normal weight women. Material and Methods MEDLINE and EMBASE were searched from 1980 to July 2014 for cohort, case control and cross sectional studies. BMI were categorized according to WHO recommendation: normal weight (BMI 18.5 - 24.9), overweight (BMI 25 - 29.9) and obese (BMI ≥ 30). Results Six studies were identified; three case control studies which were used for the meta-analysis and two cross sectional studies and one cohort study. Compared with women of recommended BMI, obese women were at increased odds of pregnancy affected by CLP (OR = 1.16; 95% CI 1, 1.34) and CP (OR = 1.14; 95% CI 0.95, 1.37). Overweight women were also at increased odds of pregnancy affected by CLP (OR = 1.06; 95% CI 0.93, 1.21) but not CP (OR = 0.89; 95% CI 0.75, 1.06). The results of the risk ratios reported in the cross sectional and cohort studies were similar to the results of the meta-analysis. Conclusions The results of this study reveal that there is an increased risk of having offspring with orofacial cleft in obese/overweight women. The reason for this association is not known. Although, the risk is small, it is important because of the increasing incidence of obesity. PMID:25937872

  10. Isolated cleft of the ala nasi: A report of seven cases

    Directory of Open Access Journals (Sweden)

    J Rajesh Jinka

    2012-01-01

    Full Text Available Craniofacial clefts other than cleft lip & palate are reported to be 1.4 to 4.9 per 100,000 live births. Of these, clefts of nose are usually associated with other clefts. Isolated cleft of Ala is rare, 0.7% of all clefts reported by Monasterio. In an analysis of photographic records of 3,500 consecutive patients with craniofacial clefts including cleft lip & palate registered with us between 1985- 2012 which were accessed through our data base, 13 patients with nasal clefts were identified, seven out of which had Isolated cleft of the Ala. All were treated by a rotation flap of the Ala with good results with the longest follow up of 14Yrs. The authors have emphasised the rarity of the condition and presented a simple surgical procedure for correction. In the opinion of the authors this very simple procedure which can be performed by the junior surgeon gives a good long term result in the management of cleft Ala.

  11. Development of the Object Permanence Concept in Cleft Lip and Palate and Noncleft Lip and Palate Infants.

    Science.gov (United States)

    Pecyna, Paula M.; And Others

    1987-01-01

    The development of the concept of object permanence was investigated with eight infants with cleft lip/palate and four nonimpaired infants. Superior performance of the cleft lip/palate group was found, possibly due to increased environmental stimulation provided by parents. (DB)

  12. Orthognathic Consequences of Sphincter Pharyngoplasty in Cleft Patients: A 2-Institutional Study

    Science.gov (United States)

    Yoshikane, Frances; Lai, Li Han; Hui, Brian K.; Martins, Deborah B.; Farias-Eisner, Gina; Mandelbaum, Rachel S.; Hoang, Han; Bradley, James P.; Wilson, Libby

    2016-01-01

    Background: Understanding long-term sequelae of cleft treatment is paramount in the refinement of treatment algorithms to accomplish optimized immediate and long-term outcomes. In this study, we reviewed sphincter pharyngoplasties as a method of velopharyngeal insufficiency (VPI) treatment in relationship to orthognathic surgery. Methods: Cleft lip/palate and cleft palate patients, 15 years of age and older, were reviewed for demographics, VPI surgery, revisions, and subsequent orthognathic surgery at 2 institutions. Chi-square test, Student’s t test, and logistic regression analyses were performed. Results: In 214 patients reviewed (mean age, 19.5 years), 61.7% were male, 18.2% had isolated cleft palate, 61.2% had unilateral cleft lip and palate, and 20.6% had bilateral cleft lip and palate. A total of 33.6% were diagnosed with VPI and received a sphincter pharyngoplasty (mean age, 11.9 years). When subsequent orthognathic surgery was examined, sphincter pharyngoplasty was not associated with maxillary advancement (P = 0.59) but did correlate with an increase in mandibular surgery from 2.8% to 11.1% (P = 0.02). The indications for mandibular surgery in the pharyngoplasty population were related to congenital micrognathia. When cephalometric analyses were evaluated, sphincter pharyngoplasty resulted in a decreased sella-to-nasion-to-B point angle (mean, 79.0–76.3 degrees, P = 0.02) and a higher incidence of normal to class II maxillomandibular relationships as defined by A point-to-nasion-to-B point angles >0.5 (P = 0.02). Conclusions: Sphincter pharyngoplasty decreases anterior mandibular growth and the discrepancy between maxillomandibular skeletal relationships because of the frequent predisposition of cleft patients to maxillary hypoplasia. In patients with congenital mandibular micrognathia, a small increase in mandibular surgeries may occur. PMID:27200238

  13. The Use of Postoperative Restraints in Children after Cleft Lip or Cleft Palate Repair: A Preliminary Report

    OpenAIRE

    Huth, Jennifer; Petersen, J. Dayne; Lehman, James A

    2013-01-01

    Purpose. This study examines whether the use of elbow restraints after cleft lip/palate repair has a relationship to postoperative complications. Methods. A comparative descriptive design was used to study a convenience sample of children undergoing repair of cleft lip/palate at Akron Children’s Hospital with Institutional Review Board approval. The children were randomized into intervention or control groups with use of elbow restraints considered the intervention. The study consists of two ...

  14. APR-246/PRIMA-1MET rescues epidermal differentiation in skin keratinocytes derived from EEC syndrome patients with p63 mutations

    OpenAIRE

    Shen, Jinfeng; van den Bogaard, Ellen H.; Kouwenhoven, Evelyn N.; Vladimir J.N. Bykov; Rinne, Tuula; Zhang, Qiang; Tjabringa, Geuranne S.; Gilissen, Christian; Van Heeringen, Simon J.; Schalkwijk, Joost; Van Bokhoven, Hans; Wiman, Klas G.; Zhou, Huiqing

    2013-01-01

    p53 and p63 share extensive sequence and structure homology. p53 is frequently mutated in cancer, whereas mutations in p63 cause developmental disorders manifested in ectodermal dysplasia, limb defects, and orofacial clefting. We have established primary adult skin keratinocytes from ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) syndrome patients with p63 mutations as an in vitro human model to study the disease mechanism in the skin of EEC patients. We show that these patien...

  15. Clinical performance of cuffed versus uncuffed preformed endotracheal tube in pediatric patients undergoing cleft palate surgery

    Science.gov (United States)

    Mukhopadhyay, S; Mukhopadhyay, S; Bhattacharya, D; Bandyopadhyay, BK; Mukherjee, M; Ganguly, R

    2016-01-01

    Background: Uncuffed endotracheal tubes are commonly used in children but due to several decade preferred in paediatric oral surgery. Due to lack of conclusive evidences in this regard, we have conducted this study to compare post-operative morbidity following use of cuffed and uncuffed endotracheal tubes in paediatric patients undergoing cleft lip-palate surgery. Methods: This randomised controlled trial was conducted on children aged 2 to 12 years.110 patients were allocated in two parallel groups using computer generated list of random numbers. Post operative extubation stridor, sore throat, time to first oral intake and regaining of normal voice were compared between two groups. Results: The incidence of sore throat was significantly more (P value > 0.005) in patients of uncuffed group compared to cuffed group. The time to first oral intake and time to regain normal voice were significantly earlier in cuffed group compared to the other. Conclusion: With standard care, preformed cuffed ET tube has shown reduced incidence of post operative sore throat. Cuffed group has earlier oral intake and normal voice regain compared to uncuffed group. PMID:27051374

  16. Association between alleles of the transforming growth factor alpha locus and cleft lip and palate in the Chilean population

    Energy Technology Data Exchange (ETDEWEB)

    Jara, L.; Blanco, R.; Chiffelle, I. [Univ. of Chile, Santiago (Chile)] [and others

    1995-07-17

    Two RFLPs at the TGFA locus were studied in 39 unrelated Chilean (Caucasoid-Mongoloid) patients with non-syndromic cleft lip/palate [CL(P)] and 51 control individuals. A highly significant association between BamHI A2 allele and CL(P) was detected ({chi}{sub 2} = 6.00; P = 0.014), while no association was found between TaqI RFLPs and clefting. No significant differences were found when comparing genotypes by type of cleft and a positive or negative family history of clefting. Our results seem to support rather definitively the association between TGFA and clefting but not support the hypothesis that TGFA is a major causal gene of CL(P). 29 refs., 5 tabs.

  17. A Case of Ectrodactyly, Ectodermal Dysplasia, Cleft Lip and Palate Syndrome Associated with Hydrocephaly

    OpenAIRE

    Buket Uysal Aladag; Fatma Hilal Yilmaz; Nadir Kocak; Ali Annagur

    2013-01-01

    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 atr...

  18. An Otorhinological study of patients with cleft lip and palate

    OpenAIRE

    Bariar, L. M.; Ahmad, Imran; S C Sharma

    2001-01-01

    32 patients of cleft palate with or without cleft lip were subjected to otorhinological study from January 1998 to September 2000, with special attention to the rhinological anomalies, ear pathology, any deafness, discharge or any other findings relevant to the middle ear. Hearing loss and ear changes were not seen in any patient with cleft lip alone, hence these patients were not included in this study. These changes were confined to the patients with cleft palate only, with or without cleft...

  19. Comparison of periodontal status among patients with cleft lip, cleft palate, and cleft lip along with a cleft in palate and alveolus

    OpenAIRE

    Boloor Vinita; Thomas Biju

    2010-01-01

    Background and Objectives : A healthy periodontium is an important prerequisite for unhindered dentition and long-term oral health. In cleft subjects, especially in those with cleft lip, alveolus and palate (CLAP), maintenance of oral hygiene is a difficult task for the patients because of the patent oro-nasal communication. Crowding of teeth in cleft patients is a common finding, especially in those with CLAP and those with cleft palate (CP). In the case of multiple tooth-malpositions , tran...

  20. Base of the skull morphology and Class III malocclusion in patients with unilateral cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Mariana Maciel Tinano

    2015-02-01

    Full Text Available OBJECTIVE: The aim of the present study was to determine the morphological differences in the base of the skull of individuals with cleft lip and palate and Class III malocclusion in comparison to control groups with Class I and Class III malocclusion. METHODS: A total of 89 individuals (males and females aged between 5 and 27 years old (Class I, n = 32; Class III, n = 29; and Class III individuals with unilateral cleft lip and palate, n = 28 attending PUC-MG Dental Center and Cleft Lip/Palate Care Center of Baleia Hospital and PUC-MG (CENTRARE were selected. Linear and angular measurements of the base of the skull, maxilla and mandible were performed and assessed by a single calibrated examiner by means of cephalometric radiographs. Statistical analysis involved ANCOVA and Bonferroni correction. RESULTS: No significant differences with regard to the base of the skull were found between the control group (Class I and individuals with cleft lip and palate (P > 0.017. The cleft lip/palate group differed from the Class III group only with regard to CI.Sp.Ba (P = 0.015. Individuals with cleft lip and palate had a significantly shorter maxillary length (Co-A in comparison to the control group (P < 0.001. No significant differences were found in the mandible (Co-Gn of the control group and individuals with cleft lip and palate (P = 1.000. CONCLUSION: The present findings suggest that there are no significant differences in the base of the skull of individuals Class I or Class III and individuals with cleft lip and palate and Class III malocclusion.

  1. Difference in the Surgical Outcome of Unilateral Cleft Lip and Palate Patients with and without Pre-Alveolar Bone Graft Orthodontic Treatment

    Science.gov (United States)

    Chang, Chun-Shin; Wallace, Christopher Glenn; Hsiao, Yen-Chang; Chiu, Yu-Ting; Pai, Betty Chien-Jung; Chen, I-Ju; Liao, Yu-Fang; Liou, Eric Jen-Wein; Chen, Philip Kuo-Ting; Chen, Jyh-Ping; Noordhoff, M. Samuel

    2016-01-01

    Presurgical orthodontic treatment before secondary alveolar bone grafting (SABG) is widely performed for cleft lip/palate patients. However, no randomized controlled trial has been published comparing SABG outcomes in patients with, and without, presurgical orthodontic treatment. This randomized, prospective, single-blinded trial was conducted between January 2012 and April 2015 to compare ABG volumes 6 months postoperatively between patients with and without presurgical orthodontic treatment. Twenty-four patients were enrolled and randomized and 22 patients completed follow-up. Patients who had presurgical orthodontics before SABG had significantly improved inclination (p < 0.001) and rotation (p < 0.001) of the central incisor adjacent to the defect, significantly improved ABG fill volume (0.81 ± 0.26 cm3 at 6 months compared to 0.59 ± 0.22 cm3; p < 0.05) and less residual alveolar bone defect (0.31 ± 0.08 cm3 at 6 months compared to s 0.55 ± 0.14 cm3; p < 0.001) compared to patients who did not have presurgical orthodontic treatment. In conclusion, orthodontic treatment combined with SABG results in superior bone volume when compared with conventional SABG alone. PMID:27041697

  2. Complex segregation analysis of nonsyndromic cleft lip/palate in a Chilean population

    Directory of Open Access Journals (Sweden)

    Blanco Rafael

    1998-01-01

    Full Text Available A população urbana Chilena contemporânea deriva da mistura de ameríndios nativos com espanhóis, apresentando uma incidência média de fissura labial não sindrômica associada ou não a fissura palatina (NSCLP de 1,8 por 1000 nascimentos vivos. A análise de segregação complexa usando o programa de computador POINTER foi feita em 249 pedigrees estendidos, distribuídos em 202 famílias simplex e 47 famílias multiplex obtidas de probands de NSCLP afetados (157 homens e 92 mulheres. Esses pedigrees deram origem a 326 indivíduos afetados e mais de 1454 parentes. Oito modelos hipotéticos foram examinados e comparados pelo teste c 2 log2 razão de máxima verossimilhança. Os modelos que postulam que NSCLP não era transmitida nestas famílias foram rejeitados, assim como os modelos que postulam apenas um componente multifatorial (P < 0,0001. O modelo que postula não haver componente poligênico para a transmissão não pôde ser rejeitado mas o modelo de não transmissão do efeito mais importante foi rejeitado (P < 0,0001. Entre os modelos do locus mais importante apenas o modelo recessivo de transmissão foi rejeitado, enquanto que as heranças codominante e dominante sem um componente multifatorial não puderam ser excluídas. O modelo não restrito sugere que a freqüência do alelo de suscetibilidade a NSCLP no locus mais importante é 0,0037 e sua penetrância é de 92%.

  3. A rare case of sirenomelia with total bilateral cleft lip palate and kyphoscoliosis

    OpenAIRE

    Sangeeta Arya; Anil Kumar Verma; Kiran Pandey; Sunil Kumar Verma

    2013-01-01

    Sirenomelia, a rare congenital deformity, has fused lower limbs with variable combination of visceral anomalies. This condition is fatal within a day or two of birth so early antenatal detection and pregnancy termination is advocated. Antenatally it can be diagnosed by ultrasonography showing oligohydramnios, renal agenesis and fibulae positioned between the tibiae. A 24 years old Muslim primigravida came for first antenatal check up at 18 weeks of pregnancy. She didn’t turn up and came...

  4. Assessment of folic acid and DNA damage in cleft lip and cleft palate

    OpenAIRE

    Sivakumar Brooklyin; Rashmoni Jana; Singaravelu Aravinthan; Bethou Adhisivam; Parkash Chand

    2014-01-01

    Studies have identified the risk factors like folic acid deficiency during gestational period, family history for orofacial clefts, drugs like antiepileptic, vitamin A. But, the data regarding the folic acid status in children with cleft lip/palate is hardly evaluated in depth. Here, an assessment of folic acid and DNA damage were carried out in children with orofacial anomalies. Folic acid level and DNA damage were evaluated by folic acid assay (direct chemiluminescent technology) and single...

  5. Simultaneous Repair of Cleft Hard Palate by Vomer Flap along with Cleft Lip in Unilateral Complete Cleft Lip and Palate Patients

    OpenAIRE

    Ferdous, Kazi Md. Noor-ul; Saif Ullah, M.; Shajahan, M.; Mitul, M. Ashrarur Rahman; Islam, M. Kabirul; Das, Kiorsh Kumar; M. A. Mannan; Rahman, M. Junaed; Biswas, Sanjoy; Salek, A. J. M.; Das, Bijoy Krishna

    2013-01-01

    The purpose of the study was to see the short-term outcome of simultaneous repair of cleft lip and cleft hard palate with vomer flap against cleft lip repair alone in patients with unilateral complete cleft lip and palate (UCLP). A prospective observational study was carried out in 35 patients with unilateral complete cleft lip and palate who under-went cleft lip and cleft hard palate repair with vomer flaps simultaneously. After 3 months, cleft soft palate was repaired. During 1st and 2nd op...

  6. Skull thickness in patients with clefts

    DEFF Research Database (Denmark)

    Arntsen, T; Kjaer, I; Sonnesen, L;

    2010-01-01

    The purpose was to analyze skull thickness in incomplete cleft lip (CL), cleft palate (CP), and combined cleft lip and palate (UCLP).......The purpose was to analyze skull thickness in incomplete cleft lip (CL), cleft palate (CP), and combined cleft lip and palate (UCLP)....

  7. Associations of chromosomes 17q22, 10q25.3 and ABCA4 gene polymorphisms with non-syndromic cleft lip/palate in Ningxia Hui and Han population%宁夏回汉族人群中染色体17q22、10q25.3和ABCA4基因多态性与非综合征型唇腭裂的关联

    Institute of Scientific and Technical Information of China (English)

    周忠伟; 杨雄; 万应彪; 信燕华; 翟堃; 马坚; 黄永清; 姜敏; 王怡瑞

    2013-01-01

    with or without cleft palate (NSCL/P) in Ningxia Hui and Han population. Methods The study consisted of 415 NSCL/P patients( Han = 191 ,Hui = 224) and their parents (303 fathers and 311 mothers, including 158 complete families). They were divided into 3 groups: the cleft lip only (CLO), the cleft palate only(CPO) and both the cleft lip and palate( CLP) groups, and we merged the CLO and CLP groups into the cleft Up with or without palate ( CL/P) group. In addition, 385 healthy college students (Han =261, Hui = 104) consisted of the control group. The single nucleotide polymorphisms ( SNPs) were genotyped by TaqMan SNPs genotyping and the data were analyzed by case-control analysis, transmission disequilibrium test and family based association test. Results ① The distribution of alleles at rs227731 on chromosome 17q22 was significantly different between CLO and control groups in the Hans (P < 0.05), and significant difference was found between CL/P and control groups in the Hans (P<0.05). There were significant differences in the genotypic and allelic frequencies of CLP and CL/P groups between the two ethnics. ② The distributions of both genotypes and alleles at rs560426 in ABCA4 gene were also significantly different between CLP and control groups in the Hans (P < 0.05), and significant difference was found between CL/P and control groups in the Hans ( P < 0. 05 ). There were significant differences in the genotypic and allelic frequencies of CLO groups between the two ethnics. ③ No differences were found in genotypic and allelic frequencies of rs7078160 on chromosome 10q25 between the patients and their parents and the controls, and between the two ethnics. Conclusion rs227731 and rs560426 SNPs are associated with NSCL/P in Ningxia Han population, and rs7078160 SNPs are not associated with NSCL/P in Ningxia population.

  8. Influence of lip closure on alveolar cleft width in patients with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Schmelzle Rainer

    2011-01-01

    Full Text Available Abstract Background The influence of surgery on growth and stability after treatment in patients with cleft lip and palate are topics still under discussion. The aim of the present study was to investigate the influence of early lip closure on the width of the alveolar cleft using dental casts. Methods A total of 44 clefts were investigated using plaster casts, 30 unilateral and 7 bilateral clefts. All infants received a passive molding plate a few days after birth. The age at the time of closure of the lip was 2.1 month in average (range 1-6 months. Plaster casts were obtained at the following stages: shortly after birth, prior to lip closure, prior to soft palate closure. We determined the width of the alveolar cleft before lip closure and prior to soft palate closure measuring the alveolar cleft width from the most lateral point of the premaxilla/anterior segment to the most medial point of the smaller segment. Results After lip closure 15 clefts presented with a width of 0 mm, meaning that the mucosa of the segments was almost touching one another. 19 clefts showed a width of up to 2 mm and 10 clefts were still over 2 mm wide. This means a reduction of 0% in 5 clefts, of 1-50% in 6 clefts, of 51-99% in 19 clefts, and of 100% in 14 clefts. Conclusions Early lip closure reduces alveolar cleft width. In most cases our aim of a remaining cleft width of 2 mm or less can be achieved. These are promising conditions for primary alveolar bone grafting to restore the dental bony arch.

  9. Periodontal Status Among Patients With Cleft Lip (CL), Cleft Palate (CP) and Cleft Lip, Alveolus and Palate (CLAP) In Chennai, India. A Comparative Study

    Science.gov (United States)

    John, Joseph

    2015-01-01

    Background: Long term health of the stomatognathic system as well as esthetic aspects is the therapeutic goals in patients with oro facial clefts. Aim: The aim of this study was to assess and compare the periodontal status of patients with cleft lip (CL), cleft palate (CP) and cleft lip, alveolus and palate (CLAP) reporting to a hospital in Chennai, India. Materials and Methods: The study group consisted of 80 cleft patients. Subjects were divided into three groups. Group 1: patients with cleft lip (CL), Group 2: subjects with cleft palate (CP) and Group 3: subjects with cleft lip alveolus and palate (CLAP). Community Periodontal Index for Treatment needs CPITN Index was recorded. Results: Among the 80 study subjects, 51 (63.8%) were males and 29 (36.2%) were females. Among the 26 study subjects with cleft lip, 10 (38.5%) had healthy periodontium, 4 (15.4%) had bleeding on probing and 12 (46.1%) had calculus. Mean number of sextants coded for healthy and bleeding was maximum among the subjects with cleft palate. Mean number of sextants coded for calculus was maximum among the subjects with cleft lip alveolus and palate. Prevalence of periodontal disease is high among patients with cleft lip, alveolus and palate (35%) than in Cleft lip (32.5%) and Cleft Palate (32.5%). Conclusion: Gingivitis and Calculus is predominantly high in patients with Cleft Palate and Cleft Lip respectively. PMID:25954706

  10. Incidencia de labio leporino y paladar hendido en la Maternidad del Hospital Clínico de la Universidad de Chile y en las maternidades chilenas participantes en el Estudio Colaborativo Latino Americano de Malformaciones Congénitas (ECLAMC período 1991-1999 Incidence of orofacial cleft in the University of Chile Maternity Hospital and other hospitals participating in the Latin American Collaborative Study of Congenital Malformations (ECLAMC

    Directory of Open Access Journals (Sweden)

    Julio Nazer H

    2001-03-01

    Full Text Available Background: ECLAMC is a registry, aimed to assess the incidence of congenital malformations, that started in 1967 and Chile incorporated to it in 1969. Aim: To report the incidence of cleft lip/palate, updated to 1999 in the University of Chile Maternity Hospital and other Chilean hospitals participating in the ECLAMC. Patients and methods: A review of the ECLAMC database that registers all births or stillbirths of more than 500 g. Results: The incidence of orofacial cleft, at the University of Chile Maternity Hospital, in the period 1991-1999 was 17.8 per 10000 (12.6 for cleft lip and 5.2 for cleft palate. The incidence in the rest of participating hospitals was 12.04 and 4.6 respectively. Males had a higher incidence of cleft lip and 80% of children with cleft palate, had other malformations, most of them as part of a syndrome (13 and 18 trisomy, holoproscencephalia, Pierre Robin, Apert en EE syndromes, anencephaly etc. In three of 12 children with cleft lip but without cleft palate, there was a relative with the same malformation. Conclusions: It is proposed that both entities, cleft lip with or without cleft palate and cleft palate without cleft lip, are two etiopathogenically different conditions (Rev Méd Chile 2001; 129: 285-93.

  11. Holoprosencephaly and ectrodactyly: Report of three new patients and review of the literature.

    Science.gov (United States)

    Keaton, Amelia A; Solomon, Benjamin D; van Essen, Anthonie J; Pfleghaar, Kathleen M; Slama, Michael A; Martin, Judith A; Muenke, Maximilian

    2010-02-15

    Holoprosencephaly (HPE) and ectrodactyly represent congenital malformations of the developing forebrain and developing digits, respectively. The combination of these conditions is rare, with only 15 cases known to date (12 previously reported, and 3 new cases described here). While the findings in these patients overlap with previously described genetic conditions, the similarity in phenotypes among these patients has led to the establishment of a at least one distinct syndrome: HPE, ectrodactyly, and bilateral cleft lip-palate syndrome (OMIM 300571). There has been great interest in identifying a genetic cause for the findings in patients with HPE and ectrodactyly; however the cause(s) of this rare association still remain unknown. PMID:20104609

  12. A Review of Hearing Loss in Cleft Palate Patients

    OpenAIRE

    Bilal Gani; Kinshuck, A. J.; Sharma, R.

    2012-01-01

    Background. Cleft palate is associated with recurrent otitis media with effusion and hearing loss. This study analysed the way these patients' hearing is managed in Alder Hey Children's Hospital. Method. A retrospective audit was carried out on cleft palate patients in Alder Hey Children's Hospital. Audiology assessment and treatment options were reviewed. Comparisons were made between the use of ventilation tubes (VTs) and hearing aids (HAs). The types of cleft, types of hearing loss, and th...

  13. Bilateral optic disc pit with maculopathy in a patient with cleft lip and cleft palate

    Science.gov (United States)

    Seth, Anisha; Gupta, Rajat; Gupta, Anika; Raina, Usha K; Ghosh, Basudeb

    2015-01-01

    Optic disc pit (ODP) is small, gray-white, oval depression found at the optic nerve head. It is a congenital defect that occurs due to imperfect closure of superior edge of the embryonic fissure. Cleft lip and palate are also congenital midline abnormalities occurring due to defect in the fusion of frontonasal prominence, maxillary prominence and mandibular prominence. There is only one case report describing the occurrence of ODP in a young patient with cleft lip and palate who also had basal encephalocele. We describe a 52-year-old patient with congenital cleft lip and palate with bilateral ODP with maculopathy but without any other midline abnormality. PMID:26044478

  14. Reconstruction of alveolar defects in patients with cleft lip and palate - 111 consecutive patients

    DEFF Research Database (Denmark)

    Andersen, Kristian

    2012-01-01

    Reconstruction of alveolar defects in patients with cleft lip and palate - 111 consecutive patients......Reconstruction of alveolar defects in patients with cleft lip and palate - 111 consecutive patients...

  15. Correlations between initial cleft size and dental anomalies in unilateral cleft lip and palate patients after alveolar bone grafting

    OpenAIRE

    Jabbari, Fatemeh; Reiser, Erika; Thor, Andreas; Hakelius, Malin; Nowinski, Daniel

    2016-01-01

    Objective To determine in individuals with unilateral cleft lip and palate the correlation between initial cleft size and dental anomalies, and the outcome of alveolar bone grafting. Methods A total of 67 consecutive patients with non-syndromic unilateral complete cleft lip and palate (UCLP) were included from the cleft lip and palate-craniofacial center, Uppsala University Hospital, Sweden. All patients were operated by the same surgeon and treated according to the Uppsala protocol entailing...

  16. Prevalence of congenital heart diseases in oral cleft patients.

    Science.gov (United States)

    Barbosa, M M; Rocha, C M G; Katina, T; Caldas, M; Codorniz, A; Medeiros, C

    2003-01-01

    To establish the prevalence of congenital heart diseases (CHDs) in cleft patients, the type of cleft and the presence of a syndrome were coded in 220 patients. A Doppler echocardiogram with color-flow mapping (DE) was obtained in all patients. Mean age was 112.0 +/- 101.2 months (range, 1-576 months), and 56.8% (125) were males. Cleft lip and palate occurred in 144 patients (65.5%), cleft lip in 40 (18.2%), and cleft palate in 36 (16.4%). Cleft palates were more frequent among females. Twenty-four CHDs were diagnosed in 21 of 220 patients (9.5%): 7 mitral valve prolapses, 6 atrial septal defects, 4 patent ductus arteriosus, 3 ventricular septal defects, 2 cases of tetralogy of Fallot, 1 pulmonary stenosis, and 1 bicuspid aortic valve. The presence of CHD did not correlate with the type of cleft. Syndromes occurred in 28 patients (12.7%), and this association was higher among patients with a cleft palate. PMID:12522651

  17. Growth hormone deficiency in cleft lip and palate patients

    Directory of Open Access Journals (Sweden)

    Shahin AbdollahiFakhim

    2015-11-01

    Full Text Available Introduction: Failure to thrive (FTT is relatively common among cleft patients, most commonly attributed to feeding problems during the first months of life. Close association between midline clefts and pituitary gland abnormalities prompted us to determine the frequency of growth hormone deficiency in cleft patients, which is easily treated. Methods: Any cleft patient with FTT was studied and when the patient’s height was under the 3rd percentile of normal, growth hormone was checked after clonidine administration. Growth hormone was checked before and 30, 60 and 90 minutes after clonidine use. Results: Of 670 patients with cleft lip or palate, 31 patients (4% had some kind of growth retardation according to weight, height or head circumstance. Eighteen patients were under the 3rd percentile of normal height. Growth hormone deficiency was detected in 8 patients out of 18 patients and overall frequency of growth hormone deficiency among cleft patients with growth retardation was 25.8% (8 out of 31. Seven patients of 8 were male whereas one was female and half of the patients were syndromic. Conclusion: Cleft patients have many problems with normal feeding and all kind of support should be provided to achieve near-normal feeding and they should be monitored for normal growth. Any patient with growth retardation, especially height decrease, should be assessed for growth hormone deficiency.

  18. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome)

    OpenAIRE

    Mohita Marwaha; Kanwar Deep Singh Nanda

    2012-01-01

    The ectodermal dysplasias (EDs) are a large and complex group of diseases. More than 170 different clinical conditions have been recognized and defined as ectodermal dysplasias. Commonly involved ectodermal-derived structures are hair, teeth, nails, and sweat glands. In some conditions, it may be associated with mental retardation. We report a case of 10-year-old male child with ectrodactyly, syndactyly, ED, cleft lip/palate, hearing loss, and mental retardation.

  19. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome

    Directory of Open Access Journals (Sweden)

    Mohita Marwaha

    2012-01-01

    Full Text Available The ectodermal dysplasias (EDs are a large and complex group of diseases. More than 170 different clinical conditions have been recognized and defined as ectodermal dysplasias. Commonly involved ectodermal-derived structures are hair, teeth, nails, and sweat glands. In some conditions, it may be associated with mental retardation. We report a case of 10-year-old male child with ectrodactyly, syndactyly, ED, cleft lip/palate, hearing loss, and mental retardation.

  20. Reorganization of craniofacial/cleft care delivery: the Massachusetts experience.

    Science.gov (United States)

    Borah, G L; Hagberg, N; Jakubiak, C; Temple, J

    1993-05-01

    Until 1989, the Commonwealth of Massachusetts operated a mandated care program known as Services for Handicapped Children (SHC) for children with cleft lip/palate or craniofacial anomalies. During the mid 1980s, the federal government reduced its block grant funds and encouraged the Commonwealth of Massachusetts to develop Project SERVE to address this changing fiscal reality. The principal outcome of Project SERVE was the recommendation that the SHC direct care programs, including all craniofacial and cleft palate clinics, should be dismantled over a number of years. However, due to the economic recession, all government funding was suddenly withdrawn from cleft palate teams and the state-run SHC clinics were abruptly dissolved. To treat patients left without coordinated care, former team members reassembled and began a new craniofacial team based at the University of Massachusetts Medical Center. Difficulties with the transition of the clinic included recruiting and retaining team members; remuneration procedures for team members; maintenance of patient records previously kept by the state; coordination of clinical/clerical responsibilities; identifying a physical locale to hold the clinics; and solicitation of referring health care provider referrals and follow-up. All these issues required specific interventions that are presented in this paper. Project SERVE, begun under federal auspices, in the Commonwealth of Massachusetts, has recently been promoted as a model for a new and improved approach to the management of cleft palate and craniofacial care delivery nationwide. Awareness of the potential for abrupt, radical change in funding for federally mandated cleft/craniofacial care is essential, and a successful transition to a medical center-based model is possible using the procedures established at our center. PMID:8338866

  1. Aspiration pneumonia in patients with cleft palate

    International Nuclear Information System (INIS)

    To assess the incidence of aspiration pneumonia in infants with cleft palate and to compare the incidence between complete and incomplete types of cleft palate. A review of medical records revealed 100 infants who had undergone initial surgery to repair cleft palate in our hospital during a recent three-year period. Aspiration pneumonia was defined as the coexistence of pneumonia at chest radiography with a history of frequent choking during feeding. The anatomic distribution of aspiration pneumonia was analyzed, and the incidences of aspiration pneumonia in infants with complete and incomplete cleft palate were compared. Among 100 children, aspiration pneumonia was found in 35 (35%). Those with complete and incomplete cleft palate showed similar incidences of the condition (27 of 70 [39%] vs 8 of 30 [27%], p=0.36). Pneumonia was most commonly seen in the left lower lobe (11 of 35), followed by the right upper and lower lobes. Aspiration pneumonia is frequently associated with infants with cleft palate. There is no statistical difference in the incidence of aspiration pneumonia between the complete and the incomplete cleft palate group

  2. Correlations between initial cleft size and dental anomalies in unilateral cleft lip and palate patients after alveolar bone grafting

    Science.gov (United States)

    Jabbari, Fatima; Reiser, Erika; Thor, Andreas; Hakelius, Malin; Nowinski, Daniel

    2016-01-01

    Objective To determine in individuals with unilateral cleft lip and palate the correlation between initial cleft size and dental anomalies, and the outcome of alveolar bone grafting. Methods A total of 67 consecutive patients with non-syndromic unilateral complete cleft lip and palate (UCLP) were included from the cleft lip and palate-craniofacial center, Uppsala University Hospital, Sweden. All patients were operated by the same surgeon and treated according to the Uppsala protocol entailing: lip plasty at 3 months, soft palate closure at 6 months, closure of the residual cleft in the hard palate at 2 years of age, and secondary alveolar bone grafting (SABG) prior to the eruption of the permanent canine. Cleft size was measured on dental casts obtained at the time of primary lip plasty. Dental anomalies were registered on radiographs and dental casts obtained before bone grafting. Alveolar bone height was evaluated with the Modified Bergland Index (mBI) at 1 and 10-year follow-up. Results Anterior cleft width correlated positively with enamel hypoplasia and rotation of the central incisor adjacent to the cleft. There was, however, no correlation between initial cleft width and alveolar bone height at either 1 or 10 years follow-up. Conclusions Wider clefts did not seem to have an impact on the success of secondary alveolar bone grafting but appeared to be associated with a higher degree of some dental anomalies. This finding may have implications for patient counseling and treatment planning. PMID:26923345

  3. Correlations between initial cleft size and dental anomalies in unilateral cleft lip and palate patients after alveolar bone grafting.

    Science.gov (United States)

    Jabbari, Fatima; Reiser, Erika; Thor, Andreas; Hakelius, Malin; Nowinski, Daniel

    2016-03-01

    Objective To determine in individuals with unilateral cleft lip and palate the correlation between initial cleft size and dental anomalies, and the outcome of alveolar bone grafting. Methods A total of 67 consecutive patients with non-syndromic unilateral complete cleft lip and palate (UCLP) were included from the cleft lip and palate-craniofacial center, Uppsala University Hospital, Sweden. All patients were operated by the same surgeon and treated according to the Uppsala protocol entailing: lip plasty at 3 months, soft palate closure at 6 months, closure of the residual cleft in the hard palate at 2 years of age, and secondary alveolar bone grafting (SABG) prior to the eruption of the permanent canine. Cleft size was measured on dental casts obtained at the time of primary lip plasty. Dental anomalies were registered on radiographs and dental casts obtained before bone grafting. Alveolar bone height was evaluated with the Modified Bergland Index (mBI) at 1 and 10-year follow-up. Results Anterior cleft width correlated positively with enamel hypoplasia and rotation of the central incisor adjacent to the cleft. There was, however, no correlation between initial cleft width and alveolar bone height at either 1 or 10 years follow-up. Conclusions Wider clefts did not seem to have an impact on the success of secondary alveolar bone grafting but appeared to be associated with a higher degree of some dental anomalies. This finding may have implications for patient counseling and treatment planning. PMID:26923345

  4. Assessing Angle's malocclusion among cleft lip and/or palate patients in Jammu

    OpenAIRE

    Gupta, Akshay; Gupta, Anur; Bhardwaj, Amit; Vikram, S.; Gomathi, Ajeetha; Singh, Karanprakash

    2016-01-01

    Objective: The study was conducted to examine the patients with abnormalities of cleft lip and/or palate and its association with different types of malocclusion. Materials and Methods: This descriptive study was done among 168 patients with abnormalities of cleft lip and/or palate. Angle's classification of malocclusion was applied for assessment of occlusion as Class I, Class II, and Class III. The types of oral clefts classification such as cleft lip unilateral and cleft lip bilateral, cle...

  5. Presurgical nasoalveolar moulding treatment in cleft lip and palate patients

    Directory of Open Access Journals (Sweden)

    Grayson Barry

    2009-10-01

    Full Text Available Presurgical infant orthopedics has been employed since 1950 as an adjunctive neonatal therapy for the correction of cleft lip and palate. Most of these therapies did not address deformity of the nasal cartilage in unilateral and bilateral cleft lip and palate as well as the deficiency of the columella tissue in infants with bilateral cleft. The nasolaveolar molding (NAM technique a new approach to presurgical infant orthopedics developed by Grayson reduces the severity of the initial cleft alveolar and nasal deformity. This enables the surgeon and the patient to enjoy the benefits associated with repair of a cleft deformity that is minimal in severity. This paper will discuss the appliance design, clinical management and biomechanical principles of nasolaveolar molding therapy. Long term studies on NAM therapy indicate better lip and nasal form, reduced oronasal fistula and labial deformities, 60 % reduction in the need for secondary alveolar bone grafting. No effect on growth of midface in sagittal and vertical plane has been recorded up to the age of 18 yrs. With proper training and clinical skills NAM has demonstrated tremendous benefit to the cleft patients as well as to the surgeon performing the repair.

  6. Advances in the Description of the Speech of People with Lip-palatal Fissure

    OpenAIRE

    Maryluz Camargo Mendoz

    2006-01-01

    This article compiles information of currentresearch in the speech description of people withcleft lip and palate, in order to present the advancesin the field of clinical phonetics in theformation of the Speech Language Pathologists.This article also explores the case study of a 12years old subject with cleft palate, hypernasalspeech, nasal air escapes and glottal stops, whichwere detected perceptual and spectrographically.

  7. Assessing Angle's malocclusion among cleft lip and/or palate patients in Jammu

    Science.gov (United States)

    Gupta, Akshay; Gupta, Anur; Bhardwaj, Amit; Vikram, S.; Gomathi, Ajeetha; Singh, Karanprakash

    2016-01-01

    Objective: The study was conducted to examine the patients with abnormalities of cleft lip and/or palate and its association with different types of malocclusion. Materials and Methods: This descriptive study was done among 168 patients with abnormalities of cleft lip and/or palate. Angle's classification of malocclusion was applied for assessment of occlusion as Class I, Class II, and Class III. The types of oral clefts classification such as cleft lip unilateral and cleft lip bilateral, cleft palate (CP), unilateral cleft lip with palate (UCLP) and bilateral cleft lip with palate (BCLP) was considered. Chi-square test was applied to analyze the data at P clefts patients as cleft lip (81), CP (31), and both cleft lip and palate (53). The occurrence of unilateral cleft lip (44) was maximum among the sample followed by UCLP (39), and bilateral cleft lip (31). Maximum subjects with Class II (10.7%) and Class III (4.9%) malocclusion were seen with unilateral cleft lip deformities. None of the patients with UCLP had Class III malocclusion. Conclusion: Cleft lip was the most commonly observed deformity and high frequency of Class II and III malocclusion was evident. Therefore, patients with such abnormalities should be screened timely. PMID:27195223

  8. Prosthodontic treatment of the edentulous adult cleft palate patient.

    Science.gov (United States)

    Sykes, Leanne M

    2003-03-01

    Clefts of the upper lip and plate are relatively common, yet dental treatment of these patients is still very poor and many grow up suffering dental neglect. Dental practitioners should become involved in the treatment team as dental needs are present from birth to death. Adult cleft patients often need tooth replacement with obturation of any residual clefts. They are best treated with tooth-supported removable appliances including partial and complete overdentures, thus preservation of their natural dentition is desirable. Edentulous cleft palate patients present with restorative difficulties due to their compromised maxillary arches as well as the presence of scar tissue in their palates and lips. An outline of these complications and guidelines for their treatment is illustrated in the form of three case reports from members of one family all presenting with varying cleft lip and palate defects. This article highlights the need for dental students to be exposed to dentally compromised patients so that they will feel confident enough to treat them in private practice. PMID:12800267

  9. SURGICAL METHODS OF CLEFT LIP, CLEFT PALATE AND COMBINED CLEFT LIP WITH CLEFT PALATE - OUR EXPERIENCE

    OpenAIRE

    Polisetti Ravi; Durga Prasad; De, Soumya; Krishna Sasanka

    2015-01-01

    INTRODUCTION Cleft lip and cleft palate is the most common congenital malformation of the face and its pattern varies with geography world wide. This study was done in 67 patients presenting to Department of ENT, Santhiram Medical College, Nandyal with cleft deformities to assess the surgical outcome of cleft lip, cleft palate and combined cleft lip cleft palate cases.

  10. A study of nasal cavity volume in patients with cleft lip and palate by magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Nakano, Kenichi [Showa Univ., Tokyo (Japan). School of Medicine

    1996-02-01

    Nasal cavity volume was studied in 11 patients with cleft lip and palate by magnetic resonance imaging. The areas of horizontal sections of the nasal cavity on the cleft and non-cleft sides were measured with the help of a personal computer and image analyzing software. Nasal cavity volume was determined by integrated volume calculation. The volume of each side was measured before and after cleft lip repair. Before cleft lip repair nasal cavity volume on the non-cleft side was larger than on the cleft side. However there was no significant difference in the volume of the cleft and non-cleft sides after cleft lip repair. (author)

  11. A study of nasal cavity volume in patients with cleft lip and palate by magnetic resonance imaging

    International Nuclear Information System (INIS)

    Nasal cavity volume was studied in 11 patients with cleft lip and palate by magnetic resonance imaging. The areas of horizontal sections of the nasal cavity on the cleft and non-cleft sides were measured with the help of a personal computer and image analyzing software. Nasal cavity volume was determined by integrated volume calculation. The volume of each side was measured before and after cleft lip repair. Before cleft lip repair nasal cavity volume on the non-cleft side was larger than on the cleft side. However there was no significant difference in the volume of the cleft and non-cleft sides after cleft lip repair. (author)

  12. Advances in the Description of the Speech of People with Lip-palatal Fissure

    Directory of Open Access Journals (Sweden)

    Maryluz Camargo Mendoz

    2006-12-01

    Full Text Available This article compiles information of currentresearch in the speech description of people withcleft lip and palate, in order to present the advancesin the field of clinical phonetics in theformation of the Speech Language Pathologists.This article also explores the case study of a 12years old subject with cleft palate, hypernasalspeech, nasal air escapes and glottal stops, whichwere detected perceptual and spectrographically.

  13. Is there an optimal resting velopharyngeal gap in operated cleft palate patients?

    OpenAIRE

    Rajesh Yellinedi; Mukunda Reddy Damalacheruvu

    2013-01-01

    Context: Videofluoroscopy in operated cleft palate patients. Aims: To determine the existence of an optimal resting velopharyngeal (VP) gap in operated cleft palate patients Settings and Design: A retrospective analysis of lateral view videofluoroscopy of operated cleft palate patients. Materials and Methods: A total of 117 cases of operated cleft palate underwent videofluoroscopy between 2006 and 2011. The lateral view of videofluoroscopy was utilised in the study. A retrospective analysis o...

  14. Surgical management of cleft lip in pedo-patients.

    Science.gov (United States)

    Taware, C P; Kulkarni, S R

    1991-01-01

    The Present article describes in short etiology of cleft lip and cleft palate. With this in-born defect, patient develops crucial problems with feeding, phonation, overall growth and development of affected and allied soft and hard tissue structures. This in turn results in deformity and asymmetry which is going to affect functional requirements as well as aesthetic outlook. Hence it really becomes mandatory to correct this defect surgically as early as possible, at stipulated timings so as to avoid present and future anticipated problems. PMID:1820390

  15. Radiographic study on maxillary sinus development and nasal septum deviation in cleft palate patient

    International Nuclear Information System (INIS)

    This study was designed to investigate the effects of the maxillary sinus development and nasal septum deviation on diseases of maxillary sinus with cleft palate. The materials was 152 cephalometric Waters' projections consist of 76 cleft patients and 76 normal subjects. The results were as follows: 1. The disease of maxillary sinus was present in 49% of a cleft group and 14% of a control group, and prevalent in cleft side. 2. It showed no statistically significant difference in size of the maxillary sinus in cleft palate patients compared to the control population and in the cleft side to the noncleft side (p<0.05). 3. Nasal septum deviation was more severe in the cleft patient its average value was 3.55mm, compared to the control group, 0.99 mm (p<0.01) and 77% of the deviated nasal septum was deviated to the cleft side.

  16. Strategies for Treating Compensatory Articulation in Patients with Cleft Palate

    OpenAIRE

    Del Carmen Pamplona, Maria; Ysunza, Antonio; Morales, Santiago

    2014-01-01

    Patients with cleft palate frequently show compensatory articulation (CA). CA requires a prolonged period of speech intervention. Some scaffolding strategies can be useful for correcting placement and manner of articulation in these cases. The purpose of this paper was to study whether the use of specific strategies of speech pathology can be more effective if applied according to the level of severity of CA. Ninety patients with CA were studied in two groups. One group was treated using stra...

  17. Linkage analysis in a large Brazilian family with van der Woude syndrome suggests the existence of a susceptibility locus for cleft palate at 17p11.2-11.1.

    OpenAIRE

    Sertié, A L; Sousa, A V; Steman, S; Pavanello, R C; Passos-Bueno, M. R.

    1999-01-01

    van der Woude syndrome (VWS), which has been mapped to 1q32-41, is characterized by pits and/or sinuses of the lower lip, cleft lip/palate (CL/P), cleft palate (CP), bifid uvula, and hypodontia (H). The expression of VWS, which has incomplete penetrance, is highly variable. Both the occurrence of CL/P and CP within the same genealogy and a recurrence risk

  18. Ectrodactyly, Ectodermal Dysplasia, Cleft Lip, and Palate (EEC Syndrome) with Tetralogy of Fallot: A Very Rare Combination

    OpenAIRE

    Sharma, Deepak; Kumar, Chetan; Bhalerao, Sanjay; Pandita, Aakash; Shastri, Sweta; Sharma, Pradeep

    2015-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC) syndrome is a rare genetic disorder with an incidence of around 1 in 90,000 in population. It is known with various names including split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand, or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot) who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysi...

  19. Ectrodactyly, Ectodermal Dysplasia, Cleft Lip, and Palate (EEC Syndrome) with Tetralogy of Fallot: A Very Rare Combination.

    Science.gov (United States)

    Sharma, Deepak; Kumar, Chetan; Bhalerao, Sanjay; Pandita, Aakash; Shastri, Sweta; Sharma, Pradeep

    2015-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC) syndrome is a rare genetic disorder with an incidence of around 1 in 90,000 in population. It is known with various names including split hand-split foot-ectodermal dysplasia-cleft syndrome or split hand, cleft hand, or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot) who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis. PMID:26137453

  20. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome) with Tetralogy of Fallot: a very rare combination

    OpenAIRE

    Deepak eSharma; Chetan eKumar; Sanjay eBhalerao; Aakash ePandita; Sweta eShastri; Pradeep eSharma

    2015-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC) syndrome is a rare genetic disorder with an incidence of around 1:90,000 live births. It is known with various names which includes split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot) who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis...

  1. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome with Tetralogy of Fallot: a very rare combination

    Directory of Open Access Journals (Sweden)

    Deepak eSharma

    2015-06-01

    Full Text Available Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC syndrome is a rare genetic disorder with an incidence of around 1:90,000 live births. It is known with various names which includes split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis.

  2. Ectrodactyly, Ectodermal Dysplasia, Cleft Lip, and Palate (EEC Syndrome) with Tetralogy of Fallot: A Very Rare Combination

    Science.gov (United States)

    Sharma, Deepak; Kumar, Chetan; Bhalerao, Sanjay; Pandita, Aakash; Shastri, Sweta; Sharma, Pradeep

    2015-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC) syndrome is a rare genetic disorder with an incidence of around 1 in 90,000 in population. It is known with various names including split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand, or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot) who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis. PMID:26137453

  3. Cleft Palate

    OpenAIRE

    Kosowski, Tomasz R.; Weathers, William M.; Wolfswinkel, Erik M.; Ridgway, Emily B.

    2012-01-01

    Our understanding of cleft palates has come a long way over the last few decades. A better understanding of the long-term consequences of a cleft palate and its effect on speech development challenges surgeons to not only effectively repair the cleft, but to also restore function of the palate for adequate speech. Coordination with speech pathologists is integral for effective management of cleft palate patients, particularly as children begin to develop language. In this article, the authors...

  4. Postoperative Speech Outcomes and Complications in Submucous Cleft Palate Patients

    Science.gov (United States)

    Park, Tae Seo; Nam, Su Bong; Kang, Kyung Dong; Sung, Ji Yoon

    2016-01-01

    Background The postoperative speech outcomes of submucous cleft palate (SMCP) surgery are known to be poorer than those of other types of cleft palate. We attempted to objectively characterize the postoperative complications and speech outcomes of the surgical treatment of SMCP through a comparison with the outcomes of incomplete cleft palate (ICP). Methods This study included 53 SMCP patients and 285 ICP patients who underwent surgical repair from 1998 to 2015. The average age of the patients at the time of surgery was 3.9±1.9 years for the SMCP patients and 1.3±0.9 years for the ICP patients. A retrospective analysis was performed of the complications, the frequency of subsequent surgical correction for velopharyngeal dysfunction (VPD), and speech outcomes. Results In both the SMCP and ICP patients, no cases of respiratory difficulty, bleeding, or wound disruption were noted. Delayed wound healing and fistula occurred in 18.9% and 5.7% of the SMCP patients and in 14% and 3.2% of the ICP patients, respectively. However, no statistically significant difference in either delayed wound healing or fistula occurrence was observed between the two groups. The rate of surgical correction for VPD in the SMCP group was higher than in the ICP group. In the subset of 26 SMCP patients and 62 ICP patients who underwent speech evaluation, the median speech score value was 58.8 in the SMCP group and 66 in the ICP group, which was a statistically significant difference. Conclusions SMCP and ICP were found to have similar complication rates, but SMCP had significantly worse speech outcomes. PMID:27218023

  5. Prevalence of Associated Anomalies in Cleft Lip and/or Palate Patients

    Directory of Open Access Journals (Sweden)

    Shahin Abdollahi Fakhim

    2016-03-01

    Full Text Available Introduction: Orofacial clefts are among the most common congenital anomalies. Patients presenting with orofacial clefts often require surgery or other complex procedures. A cleft lip or palate can be a single anomaly or a part of multiple congenital anomalies. The reported prevalence of cleft disease and associated anomalies varies widely across the literature, and is dependent on the diagnostic procedure used. In this study we determined the prevalence of associated anomalies in patients with a cleft lip and/or palate, with a specific focus on cardiac anomalies. Materials and Methods: In this cross-sectional study, 526 patients with a cleft lip and /or palate admitted to the children’s referral hospital between 2006 and 2011 were evaluated. All associated anomalies were detected and recorded. Patient information collected included age, gender, type and side of cleft, craniofacial anomalies and presence of other anomalies, including cardiac anomalies. Data were analyzed using SPSS version 16.   Results: Of the 526 patients enrolled in the study, 58% (305 were male and 42% (221 were female. In total, 75% of patients (396 were aged between 4 and 8 years and 25% (130 were aged less than 4 years. The most common cleft type in our study was bilateral cleft palate. The most commonly associated anomaly among cleft patients, in 12% of cleft patients, was a cardiac anomaly. The most common cardiac anomaly was atrial septal defect (ASD.   Conclusion:  The prevalence of associated anomalies among orofacial cleft patients is high. The most common associated anomaly is cardiac anomaly, with ASD being the most common cardiac anomaly. There are no significant relationships between type of cleft and associated cardiac anomalies.

  6. 3D computed tomographic evaluation of secondary alveolar bone grafts in cleft lip and palate patients

    International Nuclear Information System (INIS)

    Alveolar bone grafting in patients with cleft lip and palate has becomes a routine part of most treatment regimes. This study was undertaken to estimate how much bone needs to be grafted into the cleft cavity and to evaluate the grafted bone using 3-DCT over a period from the early postoperative stage to after one year. Seventy-five patients divided into four groups according to the type of cleft were studied. All patients underwent secondary alveolar bone grafting using particulate cancellous bone from the anterior iliac crest. The bone graft areas were divided into two regions: the extra-cleft region and the intra-cleft region. The weight and the volume of the grafted bone were correlated and the average density was 1.5 g/ml regardless of the cleft type. The bone in the extra-cleft region could be seen in almost all slices of the CT scans, from the lower alveolar process to the piriform aperture. The extra-cleft graft ratio of unilateral and bilateral cleft lip and palate is higher than that of cleft lip and alveolus. The extra-cleft grafting is necessary to restore facial symmetry. The grafted bone was decreased in both height and volume following three months and adequate bone bridging was maintained for one year. We concluded that 3-DCT findings are one of the most valuable methods to evaluate postoperative conditions after alveolar bone grafting. (author)

  7. 3D computed tomographic evaluation of secondary alveolar bone grafts in cleft lip and palate patients

    Energy Technology Data Exchange (ETDEWEB)

    Ohkubo, Fumio; Akai, Hidemi; Hosaka, Yoshiaki [Showa Univ., Tokyo (Japan). School of Medicine

    2001-04-01

    Alveolar bone grafting in patients with cleft lip and palate has becomes a routine part of most treatment regimes. This study was undertaken to estimate how much bone needs to be grafted into the cleft cavity and to evaluate the grafted bone using 3-DCT over a period from the early postoperative stage to after one year. Seventy-five patients divided into four groups according to the type of cleft were studied. All patients underwent secondary alveolar bone grafting using particulate cancellous bone from the anterior iliac crest. The bone graft areas were divided into two regions: the extra-cleft region and the intra-cleft region. The weight and the volume of the grafted bone were correlated and the average density was 1.5 g/ml regardless of the cleft type. The bone in the extra-cleft region could be seen in almost all slices of the CT scans, from the lower alveolar process to the piriform aperture. The extra-cleft graft ratio of unilateral and bilateral cleft lip and palate is higher than that of cleft lip and alveolus. The extra-cleft grafting is necessary to restore facial symmetry. The grafted bone was decreased in both height and volume following three months and adequate bone bridging was maintained for one year. We concluded that 3-DCT findings are one of the most valuable methods to evaluate postoperative conditions after alveolar bone grafting. (author)

  8. Investigations on the palatal rugae pattern in cleft patients. Part I: A morphological analysis.

    Science.gov (United States)

    Kratzsch, H; Opitz, C

    2000-01-01

    The characteristics of the palatal rugae zone (number of rugae, relief type, posterior limitation) were investigated on the maxillary casts of 44 patients with unilateral cleft lip and palate and 28 patients with bilateral clefts by means of reflex microscopy, a three-dimensional, computer-assisted, touch-free measuring system for the metrical registration and analysis of the parameters directly on the maxillary casts for the segments of the 2 cleft groups. The features "number of palatal rugae" and "relief type" (primary rugae) were determined both before and after surgical repair of the cleft palate. Both segments in unilateral cleft lip and palate and both lateral segments in bilateral clefts most commonly had 4 to 5 palatal rugae. The number of rugae in cleft patients is thus in a range that other authors have reported for non-cleft individuals. Following palatal cleft repair, the rugae counts per segment decreased significantly in patients with unilateral and bilateral cleft lip and palate but the 3rd rugae was never lost after surgery. The relief type identified in unilateral and bilateral cleft lip and palate was the same as in isolated cleft palates and did not differ from that in non-cleft subjects. The posterior limitation of the palatal rugae zone was determined both in a tooth-defined manner and as an absolute linear distance (at all time points). The most frequent tooth-defined posterior limitation of the rugae zone in unilateral and bilateral clefts was the second deciduous molar, which is also the position identified for non-cleft individuals. The linear distance from the tuberosity line to the rugae zone increased in all segments of unilateral and bilateral clefts during the interval up to palatal cleft repair, indicating sagittal maxillary development in the posterior area of the palate. Surgical repair of the cleft palate resulted in a significant shortening of the distance in both segments of the unilateral cleft, most likely due to the

  9. PATTERN OF DISOCCLUSION IN PATIENTS WITH COMPLETE CLEFT LIP AND PALATE

    OpenAIRE

    Daniella Andaluza Dias Matos; Marcelo Lucchesi Teixeira; João Henrique Nogueira Pinto; José Fernando Scarelli Lopes; Gisele da Silva Dalben

    2006-01-01

    OBJECTIVE: to analyze the pattern of disocclusion during excursive mandibular movements and presence or absence of occlusal interferences and occlusal pathologies (gingival recession and abfraction). METHOD: examination of 120 individuals divided into two groups, as follows: Group 1 - 90 patients with complete cleft lip and palate (study group), subdivided into 30 patients with complete left unilateral cleft lip and palate, 30 patients with complete right unilateral cleft lip and palate and 3...

  10. Bone and Soft Tissue Changes after Two-Jaw Surgery in Cleft Patients

    OpenAIRE

    Yun, Yung Sang; Uhm, Ki Il; Kim, Jee Nam; Shin, Dong Hyeok; Choi, Hyun Gon; Kim, Soon Heum; Kim, Cheol Keun; Jo, Dong In

    2015-01-01

    Background Orthognathic surgery is required in 25% to 35% of patients with a cleft lip and palate, for whom functional recovery and aesthetic improvement after surgery are important. The aim of this study was to examine maxillary and mandibular changes, along with concomitant soft tissue changes, in cleft patients who underwent LeFort I osteotomy and sagittal split ramus osteotomy (two-jaw surgery). Methods Twenty-eight cleft patients who underwent two-jaw surgery between August 2008 and Nove...

  11. Frequency of homologous blood transfusion in patients undergoing cleft lip and palate surgery

    Directory of Open Access Journals (Sweden)

    Adeyemo Wasiu

    2010-01-01

    Full Text Available Aim: The study aims to determine the frequency of homologous blood transfusion in patientsundergoing cleft lip and palate surgery at the Lagos University Teaching Hospital, Nigeria. Setting and Design: A prospective study of transfusion rate in cleft surgery conducted at the Lagos University Teaching Hospital, Nigeria. Material and Methods: One hundred consecutive patients who required cleft lip and palate surgery were recruited into the study. Data collected included age, sex and weight of patients, type of cleft defects, type of surgery done, preoperative haematocrit, duration of surgery, amount of blood loss during surgery, the number of units of blood cross-matched and those used. Each patient was made to donate a unit of homologous blood prior to surgery. Results: There were 52 females and 48 males with a mean age of 64.4 ± 101.1 months (range, 3-420 months. The most common cleft defect was isolated cleft palate (45% followed by unilateral cleft lip (28%. Cleft palate repair was the most common procedure (45% followed by unilateral cleft lip repair (41%. The mean estimated blood loss was 95.8 ± 144.9 ml (range, 2-800ml. Ten (10% patients (CL=2; CP=5, BCL=1; CLP=2 were transfused but only two of these were deemed appropriate based on percentage blood volume loss. The mean blood transfused was 131.5 ± 135.4ml (range, 35-500ml. Six (60% of those transfused had a preoperative PCV of < 30%. Only 4.9% of patients who had unilateral cleft lip surgery were transfused as compared with 50% for CLP surgery, 11% for CP surgery, and 10% for bilateral cleft lip surgery. Conclusions: The frequency of blood transfusion in cleft lip and palate surgery was 10% with a cross-match: transfusion ratio of 10 and transfusion index of 0.1. A "type and screen" policy is advocated for cleft lip and palate surgery.

  12. Rhinopharyngeal autologous fat injection for treatment of velopharyngeal insufficiency in patients with cleft palate

    OpenAIRE

    Piotet, Elsa; Beguin, Céline; Broome, Martin; Iglesias, Katia; Olivier, Frédéric; Leuchter, Igor; Zbinden, Chantal; Hohlfeld, Judith; de Buys Roessingh, Anthony; Schweizer, Valérie; Pasche, Philippe

    2016-01-01

    Patients with cleft palate are prone to velopharyngeal insufficiency. In minor cases or when hypernasal speech does not resolve after velopharyngoplasty, an augmentation pharyngoplasty with autologous fat can be proposed. The aim of the present study is to evaluate the short-term (within 2 months) and long-term efficiency (during the 24 months following the procedure) of our procedure in the setting of velopharyngeal insufficiency related to a cleft palate. Twenty-two patients with cleft pala...

  13. Occlusal Disorders among Patients with Total Clefts of Lip, Alveolar Bone, and Palate

    OpenAIRE

    Anna Paradowska-Stolarz; Beata Kawala

    2014-01-01

    Clefts are common birth defects. They are accompanied by various malformations, including disturbances in facial look as well as skeletal disorders that include malocclusions, most frequently crossbites and class III anomalies. The aim of the study was to present the commonest malocclusions in patients with total cleft of the lip, alveolar bone and palate (n = 154) and compare the results to the healthy on-cleft patients (n = 151). Normal occlusion, characteristic for I angle class, was obser...

  14. A Retrospective Study of Cleft lip and palate Patients' Satisfaction after Maxillary Distraction or Traditional Advancement of the Maxilla

    DEFF Research Database (Denmark)

    Andersen, Kristian; Nørholt, Sven Erik; Küseler, Annelise;

    2012-01-01

    A Retrospective Study of Cleft lip and palate Patients' Satisfaction after Maxillary Distraction or Traditional Advancement of the Maxilla......A Retrospective Study of Cleft lip and palate Patients' Satisfaction after Maxillary Distraction or Traditional Advancement of the Maxilla...

  15. Morbidity of chin bone transplants used for reconstructing alveolar defects in cleft patients

    NARCIS (Netherlands)

    Booij, A; Raghoebar, GM; Jansma, J; Kalk, WWI; Vissink, A

    2005-01-01

    Objective: The aim of this study was to evaluate the objective and subjective morbidity of symphyseal chin bone harvesting used for reconstruction of alveolar defects in young cleft patients. Design: All patients who had undergone chin bone harvesting for alveolar cleft reconstruction in the period

  16. 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

  17. Long-term outcome of secondary alveolar bone grafting in cleft lip and palate patients

    DEFF Research Database (Denmark)

    Meyer, Steffen; Pedersen, Kirsten Mølsted

    2013-01-01

    The objective was to assess the long-term outcome of secondary alveolar bone grafting (SABG) in cleft lip and palate patients and to examine relationships between preoperative and postoperative factors and overall long-term bone graft success. The records of 97 patients with cleft lip and palate......, who had secondary alveolar bone grafting of 123 alveolar clefts, were examined. Interalveolar bone height was assessed radiographically a minimum of 10 years after grafting using a 4-point scale (I-IV), where types I and II were considered a success. After an average follow-up of 16 years after SABG...... to the cleft. No significant differences were found with regard to the other parameters investigated. The timing of secondary alveolar bone grafting is critical with regard to the age of the patient and the stage of eruption of the tooth distal to the cleft....

  18. Cleft rhinoplasty.

    Science.gov (United States)

    Baskaran, M; Packiaraj, I; Arularasan, S Gidean; Divakar, T K

    2015-08-01

    It is universally accepted that correction of cleft lip nose deformity remains a formidable challenge for any cleft surgeon. The nose is a prominent part of the face, and hence a masterly executed cleft lip repair directs the beholders' eyes from the deformed lip to the deformed nose. A deformed nose that results from unilateral cleft of the lip and palate is likened to a tent whose one side is depressed. Many investigators believe that the deformity of the nose is produced by the malpositioning of essentially normal structures, on the other hand some cleft surgeons contend that it is the intrinsic defects in nasal structures that result in cleft nasal deformity. Depressed and hypoplastic bony scaffolding is the most important aspect of cleft nose deformity and addressing this aspect of cleft nose deformity is the secret of success of a perfect secondary rhinoplasty. Controversy still exists on timing of cleft nasal deformity. Proponents of delayed nasal repair suggest that altering the cartilages in early nasal repair at the time of lip repair would complicate future corrective nasal surgeries if the primary repair would prove unsatisfactory. The correction of nasal deformity could be performed with closed or open technique. This paper highlights one such challenging unilateral cleft lip nasal deformity in a adult patient treated by secondary rhinoplasty by open technique. PMID:26538945

  19. Development and Validation of the Quality-of-Life Adolescent Cleft Questionnaire in Patients With Cleft Lip and Palate

    OpenAIRE

    Piombino, Pasquale; Ruggiero, Federica; Dell’Aversana Orabona, Giovanni; Scopelliti, Domenico; Bianchi, Alberto; De Simone, Federica; Carnevale, Nina; Brancati, Federica; IENGO, MAURIZIO; Grassia, Maria Gabriella; Cataldo, Rosanna; Califano, Luigi

    2014-01-01

    Abstract Only a few reports in the literature have described the use of specific instruments for assessing the quality of life in adolescents and young adults with cleft lip and palate (CLP). This condition markedly affects their lifestyle, even after surgical treatment. In the present study, we aimed to develop a quality-of-life assessment tool specifically designed for such patients with CLP. Our multidisciplinary team created a questionnaire focused on the physical, psychological, and soci...

  20. Cone-Beam Computed Tomography Assessment of Lower Facial Asymmetry in Unilateral Cleft Lip and Palate and Non-Cleft Patients with Class III Skeletal Relationship.

    Directory of Open Access Journals (Sweden)

    Yifan Lin

    Full Text Available To evaluate, using cone-beam computed tomography (CBCT, both the condylar-fossa relationships and the mandibular and condylar asymmetries between unilateral cleft lip and palate (UCLP patients and non-cleft patients with class III skeletal relationship, and to investigate the factors of asymmetry contributing to chin deviation.The UCLP and non-cleft groups consisted of 30 and 40 subjects, respectively, in mixed dentition with class III skeletal relationships. Condylar-fossa relationships and the dimensional and positional asymmetries of the condyles and mandibles were examined using CBCT. Intra-group differences were compared between two sides in both groups using a paired t-test. Furthermore, correlations between each measurement and chin deviation were assessed.It was observed that 90% of UCLP and 67.5% of non-cleft subjects had both condyles centered, and no significant asymmetry was found. The axial angle and the condylar center distances to the midsagittal plane were significantly greater on the cleft side than on the non-cleft side (P=0.001 and P=0.028, respectively and were positively correlated with chin deviation in the UCLP group. Except for a larger gonial angle on the cleft side, the two groups presented with consistent asymmetries showing shorter mandibular bodies and total mandibular lengths on the cleft (deviated side. The average chin deviation was 1.63 mm to the cleft side, and the average absolute chin deviation was significantly greater in the UCLP group than in the non-cleft group (P=0.037.Compared with non-cleft subjects with similar class III skeletal relationships, the subjects with UCLP showed more severe lower facial asymmetry. The subjects with UCLP presented with more asymmetrical positions and rotations of the condyles on axial slices, which were positively correlated with chin deviation.

  1. Prenatal Counseling, Ultrasound Diagnosis, and the Role of Maternal-Fetal Medicine of the Cleft Lip and Palate Patient.

    Science.gov (United States)

    James, Jeffrey N; Schlieder, Daniel W

    2016-05-01

    A multidisciplinary team is the standard of care and the cornerstone of management of cleft patients. With readily improving advanced diagnostic modalities, early prenatal diagnosis of cleft lip and palate increasingly becomes a topic of importance for both the team caring for and families of cleft patients. Maternal-fetal medicine is a fellowship subspecialty of obstetrics that can offer high-quality care and coordination to the cleft team. Both 3-D and 4-D sonography lead to early prenatal diagnosis of cleft patients; however, differences in training result in variations in its diagnostic accuracy. PMID:26928557

  2. Dental management of Rapp-Hodgkin syndrome associated with oral cleft and hypodontia.

    Science.gov (United States)

    Karthikeyani, Shanmugasundaram; Thirumurthy, Velliangattur Ramasamy; Yuvaraja, Bindhoo A

    2016-01-01

    Rapp-Hodgkin syndrome (RHS) is a rare type of autosomal dominant disorder characterized by association of ectodermal dysplasia (ED) with cleft lip/palate. The main features include dry, brittle hair with alopecia in adulthood, dental anomalies (hypodontia, microdontia with delayed eruption, fissured tongue, and retruded maxilla), hypohidrosis, dysplastic nails, and clefting. Palmar-plantar keratoderma is seen frequently. RHS has signs and symptoms that overlap considerably with those of ankyloblepharon-ED-clefting syndrome and ectrodactyly-ED-clefting syndrome. This manuscript discusses a case of RHS, one of the four members in three generations who had ED with variable degree of involvement of hair, teeth, nail, and sweat glands. PMID:27080974

  3. Strategies for treating compensatory articulation in patients with cleft palate.

    Science.gov (United States)

    Del Carmen Pamplona, Maria; Ysunza, Antonio; Morales, Santiago

    2014-03-01

    Patients with cleft palate frequently show compensatory articulation (CA). CA requires a prolonged period of speech intervention. Some scaffolding strategies can be useful for correcting placement and manner of articulation in these cases. The purpose of this paper was to study whether the use of specific strategies of speech pathology can be more effective if applied according to the level of severity of CA. Ninety patients with CA were studied in two groups. One group was treated using strategies specific for their level of severity of articulation, whereas in the other group all strategies were used indistinctively. The degree of severity of CA was compared at the end of the speech intervention. After the speech therapy intervention, the group of patients in which the strategies were used selectively, showed a significantly greater decrease in the severity of CA, as compared with the patients in whom all the strategies were used indistinctively. An assessment of the severity of CA can be useful for selecting the strategies, which can be more effective for correcting the compensatory errors. PMID:24711749

  4. DENTAL CAST STUDY OF ADULT PATIENTS WITH UNTREATED UNILATERAL CLEFT-LIP OR CLEFT-LIP AND PALATE IN INDONESIA COMPARED WITH SURGICALLY TREATED PATIENTS IN THE NETHERLANDS

    NARCIS (Netherlands)

    SPAUWEN, PHM; HARDJOWASITO, W; BOERSMA, J; LATIEF, BS

    1993-01-01

    To determine differences in maxillary and dentoalveolar relationships between untreated and treated patients having unilateral clefts of the lip and alveolus (UCLA) or lip and palate (UCLP), dental cast assessments were done on 70 untreated adult Indonesian patients (UCLA-I, UCLP-I) and 67 Dutch pat

  5. Dual embryonic origin of maxillary lateral incisors: clinical implications in patients with cleft lip and palate

    Science.gov (United States)

    Garib, Daniela Gamba; Rosar, Julia Petruccelli; Sathler, Renata; Ozawa, Terumi Okada

    2015-01-01

    Introduction: Cleft lip and palate are craniofacial anomalies highly prevalent in the overall population. In oral clefts involving the alveolar ridge, variations of number, shape, size and position are observed in maxillary lateral incisors. The objective of this manuscript is to elucidate the embryonic origin of maxillary lateral incisors in order to understand the etiology of these variations. Contextualization: The hypothesis that orofacial clefts would split maxillary lateral incisor buds has been previously reported. However, recent studies showed that maxillary lateral incisors have dual embryonic origin, being partially formed by both the medial nasal process and the maxillary process. In other words, the mesial half of the lateral incisor seems to come from the medial nasal process while the distal half of the lateral incisor originates from the maxillary process. In cleft patients, these processes do not fuse, which results in different numerical and positional patterns for lateral incisors relating to the alveolar cleft. In addition to these considerations, this study proposes a nomenclature for maxillary lateral incisors in patients with cleft lip and palate, based on embryology and lateral incisors position in relation to the alveolar cleft. Conclusion: Embryological knowledge on the dual origin of maxillary lateral incisors and the use of a proper nomenclature for their numerical and positional variations renders appropriate communication among professionals and treatment planning easier, in addition to standardizing research analysis. PMID:26560830

  6. Dual embryonic origin of maxillary lateral incisors: clinical implications in patients with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Daniela Gamba Garib

    2015-10-01

    Full Text Available Introduction:Cleft lip and palate are craniofacial anomalies highly prevalent in the overall population. In oral clefts involving the alveolar ridge, variations of number, shape, size and position are observed in maxillary lateral incisors. The objective of this manuscript is to elucidate the embryonic origin of maxillary lateral incisors in order to understand the etiology of these variations.Contextualization: The hypothesis that orofacial clefts would split maxillary lateral incisor buds has been previously reported. However, recent studies showed that maxillary lateral incisors have dual embryonic origin, being partially formed by both the medial nasal process and the maxillary process. In other words, the mesial half of the lateral incisor seems to come from the medial nasal process while the distal half of the lateral incisor originates from the maxillary process. In cleft patients, these processes do not fuse, which results in different numerical and positional patterns for lateral incisors relating to the alveolar cleft. In addition to these considerations, this study proposes a nomenclature for maxillary lateral incisors in patients with cleft lip and palate, based on embryology and lateral incisors position in relation to the alveolar cleft.Conclusion:Embryological knowledge on the dual origin of maxillary lateral incisors and the use of a proper nomenclature for their numerical and positional variations renders appropriate communication among professionals and treatment planning easier, in addition to standardizing research analysis.

  7. Evaluation of the cephalometric changes of Tweed triangle in patients with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Mohsen Shirazi

    2015-12-01

    Full Text Available Background and Aims: Cleft lip and palate patients require orthodontic treatments during their childhood and adolescence. Tweed diagnostic triangle as well as cephalometric assessments provides important data regarding the skeletal patterns for the treatment and diagnostic purposes. The present study determined the cephalometric changes of Tweed triangle in the cleft lip and palate patients compared to normal patients. Materials and Methods: In total, 101 cleft and palate patients as well as 95 normal individuals with the balanced age, gender and race were evaluated. All the cleft and palate patients had similar treatment histories. Radiographic clichés were obtained from both groups and the images were traced after identifying the anatomic landmarks. The studied landmarks included points, lines, and plans as well as dental and skeletal angles and distance ratios measured in radiographic images. The landmarks were statistically analyzed using Student t test. Results: Significant differences were found between the cleft lip and palate patients and normal individuals regarding craniofacial complex morphology (P0.05. Conclusion: In total, significant differences were observed between normal and cleft lip and palate individuals regarding Tweed diagnostic triangle area. These differences were decreased IMPA and increased FMA and FMIA angles. No significant differences were found in terms of other indices.

  8. Clefting of the Alveolus: Emphasizing the Distinction from Cleft Palate.

    Science.gov (United States)

    Wirtz, Nicholas; Sidman, James; Block, William

    2016-05-01

    Oral clefting is one of the most common significant fetal abnormalities. Cleft lip and cleft palate have drastically different clinical ramifications and management from one another. A cleft of the alveolus (with or without cleft lip) can confuse the diagnostic picture and lead to a false assumption of cleft palate. The cleft alveolus should be viewed on the spectrum of cleft lip rather than be associated with cleft palate. This is made evident by understanding the embryological development of the midface and relevant terminology. Cleft alveolus carries significantly different clinical implications and treatment options than that of cleft palate. Accurately distinguishing cleft alveolus from cleft palate is crucial for appropriate discussions regarding the patient's care. PMID:26906186

  9. Enxerto autógeno de crista ilíaca na reconstrução do processo alveolar em portadores de fissura labiopalatina: estudo de 30 casos Iliac crest autogenous graft in reconstruction of alveolar process in cleft patients: study of 30 cases

    Directory of Open Access Journals (Sweden)

    João Luiz Carlini

    2000-12-01

    Full Text Available A reconstrução do processo alveolar em pacientes portadores de fissuras labiopalatais (FLP é fundamental para erupção de dentes permanentes e para o restabelecimento estético através de implantes osteointegrados. Assim, considerado o alto grau de reabsorção do enxerto de osso autógeno puro e triturado, optou-se no Centro de Atendimento Integral ao Fissurado Labiopalatino, Curitiba, Paraná (1997 a 1999, pelo estabelecimento de dois grupos de 15 pacientes. No primeiro, foi utilizada crista ilíaca triturada e no outro combinada com hidroxiapatita biorreabsorvível. Ao fim de seis meses, os resultados cirúrgicos foram avaliados através de radiografia periapical digital, determinando quatro linhas limites para a reconstrução óssea. A primeira linha foi traçada na região cervical do dente adjacente à fissura que apresentava menor distorção. As outras linhas foram traçadas paralelamente à linha 1, distando 3mm entre cada uma delas. O espaço entre as linhas 1 e 2 foi denominado de A, entre 2 e 3 de B e 3 e 4 de C. Para critérios de avaliação, quando o enxerto permaneceu no espaço A, foi considerado de sucesso, em contraposição aos espaços B e C, quando foram denominados de procedimentos malsucedidos. Como resultados finais, o espaço A foi constatado em 46,7% dos pacientes submetidos ao procedimento controle com crista ilíaca e 80% para o grupo experimental, quando o enxerto ósseo foi empregado conjuntamente com o material aloplástico (hidroxiapatita.The reconstruction of alveolar process in cleft lip palate patients is mandatory to enable the eruption and movement of permanent teeth through the clefted area by orthodontic procedure, beside the establisment of aesthetics with prostetic procedure by osteointegrated implants. Considering that autogenous bone grafts provide a high reabsortion level determining a reduction of orthodontic movement or making impossible to place implants, to minimize this problem, we

  10. Pre: Surgical orthopedic pre-maxillary alignment in bilateral cleft lip and palate patient

    Directory of Open Access Journals (Sweden)

    Vijaya Prasad Kamavaram Ellore

    2012-01-01

    Full Text Available Pre-surgical orthopedic appliances are mainly used to retract and align the protruded and deviated pre-maxilla and to facilitate initial lip repair. This article presents a case report of a five year old male child patient with bilateral cleft lip and palate in whom a special custom made pre-surgical orthopedic appliance was delivered. Use of a special custom made presurgical orthopedic appliance for repositioning pre-maxilla in bilateral cleft lip and palate patient is discussed in this article.

  11. Current status of presurgical infant orthopaedic treatment for cleft lip and palate patients: A critical review

    Directory of Open Access Journals (Sweden)

    P Priyanka Niranjane

    2014-01-01

    Full Text Available Rehabilitation of cleft lip and palate (CLP patients is a challenge for all the concerned members of the cleft team, and various treatment modalities have been attempted to obtain aesthetic results. Presurgical infant orthopaedics (PSIO was introduced to reshape alveolar and nasal segments prior to surgical repair of cleft lip. However, literature reports lot of controversy regarding the use of PSIO in patients with CLP. Evaluation of long-term results of PSIO can provide scientific evidence on the efficacy and usefulness of PSIO in CLP patients. The aim was to assess the scientific evidence on the efficiency of PSIO appliances in patients with CLP and to critically analyse the current status of PSIO. A PubMed search was performed using the terms PSIO, presurgical nasoalveolar moulding and its long-term results and related articles were selected for the review. The documented studies report no beneficial effect of PSIO on maxillary arch dimensions, facial aesthetics and in the subsequent development of dentition and occlusion in CLP patients. Nasal moulding seems to be more beneficial and effective in unilateral cleft lip and palate patients with better long-term results.

  12. Assessment of middle ear function in patients with cleft palate treated under "Smile Train Project"

    Directory of Open Access Journals (Sweden)

    Prachi Gautam

    2011-01-01

    Full Text Available Objective: To evaluate the middle ear function in patients of cleft palate treated under "Smile Train Project". Materials and Methods: Sixty patients (120 ears; 32 male and 28 female of cleft palate with or without cleft lip were evaluated in pediatrics and ENT OPD in preoperative period. A general medical check up was performed in pediatrics OPD to evaluate the associated craniofacial anomalies. Middle ear function was evaluated in ENT OPD. Otoscopy was done in all patients. Cases under five years of age were evaluated for middle ear function by tympanometry and cases with more than five years of age were evaluated by tympanometry for middle ear function and pure tone audiometry to establish the type of hearing loss. The results are presented as number of ears. Results: A total of 50% of the patients in the study group were in zero- to two-year age group. Otoscopy findings in 120 ears showed that 66.66% of the patients had dull tympanic membrane suggesting a diagnosis of otitis media with effusion. Tympanometry was done in 110 ears, of which, 72.72% of ears had type B tympanogram suggesting otitis media with effusion. Pure tone audiometry in more than five years age group showed that 81.25% ears had conductive hearing loss. Conclusion: This study suggests the need of preoperative ENT check up and audiological assessment in patients of cleft palate undergoing palatal surgery under "Smile Train Project". The association of otitis media with effusion in patients with cleft palate appears strong and a high degree of suspicion for the disease should be kept in them. The anatomical defect and possible causative pathophysiology also supports the inference. Otitis media with effusion causing hearing loss in these patients causes speech delay and unfavorable outcome despite aggressive speech therapy in postoperative period. A co-ordinate team approach of concerned specialists involved in managing these patients would definitely improve their outcome.

  13. Influence of palatoplasty on occlusion of patients with isolated cleft palate

    Directory of Open Access Journals (Sweden)

    Omar Gabriel da Silva Filho

    2012-04-01

    Full Text Available OBJECTIVE: This study compared the dental arch morphology of adult patients with isolated cleft palate in order to verify the influence of palatoplasty on occlusion. METHODS: Cast models of 77 patients, 30 males and 47 females, with an average age of 21 years and no syndromes were taken. They were in the permanent dentition and had not undergone orthodontic treatment. The sample was divided into non-operated and operated patients, the latter having been submitted to palatoplasty at a mean age of 2.2 years. RESULTS: Almost 80% of the sample exhibited sagittal discrepancies in the inter-arch relationship, with a Class II malocclusion prevailing (59.74% followed by Class III (20,78%, regardless of palatoplasty. Transverse analysis showed a 23% incidence of posterior crossbite also not influenced by palatoplasty. Intra-arch relationship indicated that constriction and crowding on the upper arch were more frequent in the operated group (p=0.0238 and p=0.0002, respectively, showing an influence of palatoplasty on its morphology. The predominant morphological characteristics in patients with isolated cleft palate were a Class II malocclusion, upper dental arch constriction and upper and lower anterior crowding. CONCLUSION: The influence of palatoplasty was restricted to constriction and crowding of the upper dental arch, with no interference from the extension of the cleft, except for the upper crowding, which occurred more in patients with complete cleft palates.

  14. Orthognathic Surgery for Achieving Facial Balance and Harmony in Cleft Patients

    Institute of Scientific and Technical Information of China (English)

    Kenneth E Salyer; XU Hai-song

    2015-01-01

    Cleft surgery requires an expert team performing ongoing treatment in order to achieve optimal outcomes. The senior author's (KES) experiences of more than 2 000 patients with cleft lip and palate treated by a surgical-orthodontic protocol were introduced. The paper here will concentrate on not only correcting the occlusion as others have described, but also on what one surgeon can do to achieve optimal aesthetic balance, harmony and beauty. The results of orthognathic surgery in respect to function, stability, cosmesis, and complications are also audited.

  15. Skeletal facial balance and harmony in the cleft patient: Principles and techniques in orthognathic surgery

    Directory of Open Access Journals (Sweden)

    Salyer Kenneth

    2009-10-01

    Full Text Available The management of the palatal cleft, dental arch, and subsequent maxillary form is a challenge for the craniomaxillofacial surgeon. The purpose of this paper is to present the experience of a senior surgeon (KES who has treated over 2000 patients with cleft lip and palate. This paper focuses on the experience of a recent series of 103 consecutive orthognathic cases treated by one surgeon with a surgical-orthodontic, speech-oriented approach. It will concentrate on not only correcting the occlusion, as others have described, but also on how a surgeon who was trying to achieve optimal aesthetic balance, harmony, and beauty, approached this problem.

  16. APR-246/PRIMA-1(MET) rescues epidermal differentiation in skin keratinocytes derived from EEC syndrome patients with p63 mutations.

    Science.gov (United States)

    Shen, Jinfeng; van den Bogaard, Ellen H; Kouwenhoven, Evelyn N; Bykov, Vladimir J N; Rinne, Tuula; Zhang, Qiang; Tjabringa, Geuranne S; Gilissen, Christian; van Heeringen, Simon J; Schalkwijk, Joost; van Bokhoven, Hans; Wiman, Klas G; Zhou, Huiqing

    2013-02-01

    p53 and p63 share extensive sequence and structure homology. p53 is frequently mutated in cancer, whereas mutations in p63 cause developmental disorders manifested in ectodermal dysplasia, limb defects, and orofacial clefting. We have established primary adult skin keratinocytes from ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) syndrome patients with p63 mutations as an in vitro human model to study the disease mechanism in the skin of EEC patients. We show that these patient keratinocytes cultured either in submerged 2D cultures or in 3D skin equivalents have impaired epidermal differentiation and stratification. Treatment of these patient keratinocytes with the mutant p53-targeting compound APR-246/PRIMA-1(MET) (p53 reactivation and induction of massive apoptosis) that has been successfully tested in a phase I/II clinical trial in cancer patients partially but consistently rescued morphological features and gene expression during epidermal stratification in both 2D and 3D models. This rescue coincides with restoration of p63 target-gene expression. Our data show that EEC patient keratinocytes with p63 mutations can be used for characterization of the abnormal molecular circuitry in patient skin and may open possibilities for the design of novel pharmacological treatment strategies for patients with mutant p63-associated developmental abnormalities. PMID:23355676

  17. Anatomical Variations in Clefts of the Lip with or without Cleft Palate

    OpenAIRE

    Carroll, K; Mossey, P.A

    2012-01-01

    Objective. Few orofacial cleft (OFC) studies have examined the severity of clefts of the lip or palate. This study examined associations between the severity of cleft of the lip with cleft type, laterality, and sex in four regional British Isles cleft registers whilst also looking for regional variations. Design. Retrospective analysis of cleft classification in the data contained in these four cleft registers. Sample. Three thousand and twelve patients from cleft registers based in Scotland,...

  18. Influence of palatoplasty on occlusion of patients with isolated cleft palate

    OpenAIRE

    Omar Gabriel da Silva Filho; Elisa Teixeira Marinho; Rita de Cássia Moura Carvalho Lauris

    2012-01-01

    OBJECTIVE: This study compared the dental arch morphology of adult patients with isolated cleft palate in order to verify the influence of palatoplasty on occlusion. METHODS: Cast models of 77 patients, 30 males and 47 females, with an average age of 21 years and no syndromes were taken. They were in the permanent dentition and had not undergone orthodontic treatment. The sample was divided into non-operated and operated patients, the latter having been submitted to palatoplasty at a mean age...

  19. Development and Validation of the Quality-of-Life Adolescent Cleft Questionnaire in Patients With Cleft Lip and Palate

    Science.gov (United States)

    Piombino, Pasquale; Ruggiero, Federica; Dell’Aversana Orabona, Giovanni; Scopelliti, Domenico; Bianchi, Alberto; De Simone, Federica; Carnevale, Nina; Brancati, Federica; Iengo, Maurizio; Grassia, Maria Gabriella; Cataldo, Rosanna; Califano, Luigi

    2014-01-01

    Abstract Only a few reports in the literature have described the use of specific instruments for assessing the quality of life in adolescents and young adults with cleft lip and palate (CLP). This condition markedly affects their lifestyle, even after surgical treatment. In the present study, we aimed to develop a quality-of-life assessment tool specifically designed for such patients with CLP. Our multidisciplinary team created a questionnaire focused on the physical, psychological, and social satisfaction of adolescents and young adults with CLP, which was adapted from 3 dimensions of the 36-item Short-Form Health Survey. The questionnaire was administered to a randomized sample of 40 adolescents and young adults (aged 16–24 years) with CLP who had completed treatment protocols and 40 (aged 16–24 years) who were not affected by CLP. The statistical results stated that the questionnaire had good reliability and validity; the Cronbach α coefficient was found to be 0.944. Moreover, factorial analysis confirmed the presence of 3 subscales that were the fundamental components of this questionnaire, which is consistent with the areas theoretically proposed and from which the items were designed and selected. Thus, we validated our novel questionnaire that was administered in the present study and proved its consistency. However, further investigations on a larger population would be useful to confirm these findings. PMID:25010834

  20. Bilateral acute visual loss from Rathke's cleft cyst apoplexy in a patient with dengue fever

    Directory of Open Access Journals (Sweden)

    Ana Cláudia De Franco Suzuki

    2014-10-01

    Full Text Available Hemorrhagic complications of optic pathway diseases are extremely rare causes of acute visual loss associated with dengue fever. In this paper we report a patient presenting with dengue fever and bilateral acute visual loss caused by chiasmal compression due to Rathke's cleft cyst apoplexy. Considering the importance of early diagnosis and treatment to visual recovery, apoplexy of sellar and suprasellar tumors should be considered in the differential diagnosis of patients with acute visual loss and dengue fever.

  1. Bilateral acute visual loss from Rathke's cleft cyst apoplexy in a patient with dengue fever

    OpenAIRE

    Ana Cláudia De Franco Suzuki; Rafael Barbosa de Araújo; Eduardo Cunha de Souza; Mário Luiz Ribeiro Monteiro

    2014-01-01

    Hemorrhagic complications of optic pathway diseases are extremely rare causes of acute visual loss associated with dengue fever. In this paper we report a patient presenting with dengue fever and bilateral acute visual loss caused by chiasmal compression due to Rathke's cleft cyst apoplexy. Considering the importance of early diagnosis and treatment to visual recovery, apoplexy of sellar and suprasellar tumors should be considered in the differential diagnosis of patients with acute visual lo...

  2. Velopharyngeal Insufficiency Rates After Delayed Cleft Palate Repair: Lessons Learned From Internationally Adopted Patients.

    Science.gov (United States)

    Follmar, Keith E; Yuan, Nance; Pendleton, Courtney S; Dorafshar, Amir H; Kolk, Craig Vander; Redett, Richard J

    2015-09-01

    Most surgeons recommend cleft palate repair between 6 and 12 months of age. Internationally adopted patients often undergo delayed repair due to social circumstances. There are few outcomes studies on this population. We conducted a 13-year retrospective review of all patients undergoing primary cleft palate repair at a single tertiary-care academic medical center between 1993 and 2006. The primary outcome was velopharyngeal insufficiency, defined as the recommendation for corrective surgery after multiple formal speech assessments. Two hundred one patients (102 males and 99 females) were identified. One hundred eighty-three repairs were performed before 18 months of age (standard repair group). Eighteen repairs were performed after 18 months of age (delayed repair group), with international adoption being a circumstance in 16 cases. The delayed and standard repair groups were similar with regard to sex, presence of craniofacial syndrome, Veau class, cleft size and laterality, type of repair, and operating surgeon. Mean follow-up was 9.3 years, with minimum follow-up of 5.0 years. Six (33%) of 18 patients in the delayed repair group developed velopharyngeal insufficiency compared to 23 (13%) of 183 in the standard repair group (P = 0.03 by Fisher exact test). These data demonstrate that internationally adopted patients undergoing delayed palate repair suffer especially poor speech outcomes. Physiologic differences in patients undergoing late repair, as well as social factors including adaptation to a new language and culture, may be factors. Early repair should be performed when possible. PMID:25046662

  3. Risk of leukemia in first degree relatives of patients with nonsyndromic cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Eduardo GONÇALVES

    2014-01-01

    Full Text Available The aim of this study was to determine the frequency of leukemia in parents of patients with nonsyndromic cleft lip and/or cleft palate (NSCL/P. This case-control study evaluated first-degree family members of 358 patients with NSCL/P and 1,432 subjects without craniofacial alterations or syndromes. Statistical analysis was carried out using Fisher’s test. From the 358 subjects with NSCL/P, 3 first-degree parents had history of leukemia, while 2 out of 1,432 subjects from the unaffected group had a family history of leukemia. The frequency of positive family history of leukemia was not significantly increased in first-degree relatives of patients with NSCL/P.

  4. Preparation of Plaster Moulage (Cast in Plastic Surgery patients

    Directory of Open Access Journals (Sweden)

    Rana R

    2003-01-01

    Full Text Available The purpose of this paper is to describe the technique of making casts using alginate compound for negative and dental stone plaster for positive impressions. With certain modifications a cast could be made of any part of the body and one can make a museum of interesting cases. Casts serve as useful teaching material especially in cleft lip and palate patients to study the effect of surgery on growth and development of the cleft lip-palate-nose complex in relation to the remaining face. It also helps in planning reconstruction in cases of facial defects, recording serial changes in multistage surgery, pre-operative and post-operative comparison as in rhinoplasty, ear reconstruction, hand etc; for comparing results before and after treatment in keloid and hypertrophic scars, fabrication of implants and preparation of prosthesis. In spite of newer modalities like 3-D imaging and stereolithography, the usefulness of this old technique in certain interesting cases can not be denied.

  5. Closure of Huge Palatal Fistula in an Adult Patient with Isolated Cleft Palate: A Technical Note

    OpenAIRE

    Amin Rahpeyma, DDS; Saeedeh Khajehahmadi, DDS

    2015-01-01

    Summary: Closure of huge palatal fistula surrounded by fully erupted permanent dentition in the adult patients with cleft is a challenge. Posteriorly based buccinator myomucosal flap is a neurovascular pedicled flap, with inherent nature of thin thickness, saliva secretion, and axial pattern blood supply. Vicinity of donor site to the palate and low donor-site morbidity are the other advantages. It is an ideal choice in such situation. In this article, the details of surgical technique and th...

  6. Inferior Turbinate Flap for Nasal-side Closure of Palatal Fistula in Cleft Patients: Technical Note

    Directory of Open Access Journals (Sweden)

    Amin Rahpeyma

    2014-12-01

    Full Text Available Summary: Residual palatal fistula after repair of palatal cleft is common. Repair of residual oronasal fistula is not always successful. Two-layer closure techniques that close these fistulas with soft tissue are a common practice. Turnover flaps are the most used flaps and often the sole method for nasal-side closure of fistula. Anteriorly based inferior turbinate flap can be used to provide soft tissue for nasal-side closure when turnover flaps will not provide sufficient tissue for this purpose. Under general anesthesia with nasotracheal intubation, inferior turbinate was released from posterior attachment. After removing the inferior conchal bone, mucoperiosteal flap was used for nasal-side closure of anterior palatal fistula in patients with cleft. Anteriorly based inferior turbinate flap was used for nasal-side closure of residual palatal fistula in 3 patients with cleft. Age of the patients was 14, 16, and 18, and recurrence of palatal fistula has not occurred. Anteriorly based inferior turbinate flap is an axial pattern flap with appropriate surface of the paddle and vicinity to the oral cavity roof. It can be used in large anterior, palatal fistula for reconstruction of nasal floor. Considering appropriate another flap for oral side coverage of such fistula is mandatory.

  7. Impaired epithelial differentiation of induced pluripotent stem cells from ectodermal dysplasia-related patients is rescued by the small compound APR-246/PRIMA-1MET

    OpenAIRE

    Shalom-Feuerstein, Ruby; Serror, Laura; Aberdam, Edith; Müller, Franz-Josef; Van Bokhoven, Hans; Wiman, Klas G.; Zhou, Huiqing; Aberdam, Daniel; PETIT Isabelle

    2013-01-01

    Ectodermal dysplasia is a group of congenital syndromes affecting a variety of ectodermal derivatives. Among them, ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) syndrome is caused by single point mutations in the p63 gene, which controls epidermal development and homeostasis. Phenotypic defects of the EEC syndrome include skin defects and limbal stem-cell deficiency. In this study, we designed a unique cellular model that recapitulated major embryonic defects related to EEC. ...

  8. Cleft lip and palate and related factors: A 10 years study in university hospitalised patients at Mashhad — Iran

    Science.gov (United States)

    Noorollahian, Morteza; Nematy, Mohsen; Dolatian, Atiyeh; Ghesmati, Hengameh; Akhlaghi, Saeed; Khademi, Gholam Reza

    2015-01-01

    Background: Oral-facial clefts including cleft lip and palate are the most common congenital malformations of the head and neck. Environmental factors such as maternal hormonal disorders, use of psychiatric medications, vitamin and folic acid deficiency, hypoxia, cigarette smoking and maternal obesity and overweight can affect the incidence of these disorders. In Iran, one of the associated problems is a lack of accurate statistics regarding the present status of the patients, which can cause a disturbance in the health programmes of Ministry of Health and Medical Education. The aim of this study was to report the status of 398 cases of cleft lip and palate in Sheikh and Imam Reza Hospitals of Mashhad over a 10-year period. Materials and Methods: This retrospective descriptive study was performed using data collection method and included the evaluation of the recorded files and completing the data forms. In this study, the file records of 398 patients referring to Mashhad Sheikh and Imam Reza (P.U.H) Hospitals were studied, from the beginning of 2002 to the end of 2011; the obtained data from the files were collected and classified. Results: The highest frequency was related to cleft palate alone (40.7%); frequencies were lower regarding the cleft lip and palate and cleft lip alone (34.41% and 24.87%, respectively). Approximately, half of the patients were from rural areas of the city and had articulation disorders. Most of the patients were the first-born children of the family and their parents were consanguineously married; about one-third of the patients had a family history of the disease. Conclusion: According to the results of the present study, cleft lip is more frequent in males and cleft palate is more prevalent in females; the obtained results are consistent with the global statistics. PMID:26712297

  9. Three dimensional analysis of CT image on naso-maxillary complex in cleft lip and palate patients

    Energy Technology Data Exchange (ETDEWEB)

    Ma, Rong-Rong [Tokyo Medical and Dental Univ. (Japan). School of Dentistry

    1994-12-01

    This study was designed to clarify the three dimensional features of naso-maxillary complex in cleft lip and palate (CLP) by using computed tomography (CT) and to examine its change following an upper dental arch expansion. Sequential CT images with 2mm-thickness were obtained for 11 unilateral CLP boys (UCLP), 6 bilateral CLP boys (BCLP) and 4 boys without cleft (non-cleft). Additionally, two serial sets of upper dental cast before and after dental arch expansion coupled with CT images in UCLP were used to evaluate the effect of dental arch expansion on the naso-maxillary complex. UCLP demonstrated a remarkable naso-maxillary deformity characterized by a decreased volume of maxillary sinus in comparison with the non-cleft patients. Both the volume and shape of nasal cavity were significantly different between the cleft and non cleft side. Naso-maxillary morphology of BCLP, however, was similar to that of the non cleft except for the decreased volume of alveolar arch. Comparative study of UCLP and BCLP showed a significant difference in naso-maxillary morphology. There were some significant correlations between the dental arch expansion and change of each naso-maxillary component, suggesting the effect of expansion stress on the naso-maxillary complex in UCLP. However, deformation caused by expansion stress varied, depending on each component of the naso-maxillary complex. (author) 61 refs.

  10. Three dimensional analysis of CT image on naso-maxillary complex in cleft lip and palate patients

    International Nuclear Information System (INIS)

    This study was designed to clarify the three dimensional features of naso-maxillary complex in cleft lip and palate (CLP) by using computed tomography (CT) and to examine its change following an upper dental arch expansion. Sequential CT images with 2mm-thickness were obtained for 11 unilateral CLP boys (UCLP), 6 bilateral CLP boys (BCLP) and 4 boys without cleft (non-cleft). Additionally, two serial sets of upper dental cast before and after dental arch expansion coupled with CT images in UCLP were used to evaluate the effect of dental arch expansion on the naso-maxillary complex. UCLP demonstrated a remarkable naso-maxillary deformity characterized by a decreased volume of maxillary sinus in comparison with the non-cleft patients. Both the volume and shape of nasal cavity were significantly different between the cleft and non cleft side. Naso-maxillary morphology of BCLP, however, was similar to that of the non cleft except for the decreased volume of alveolar arch. Comparative study of UCLP and BCLP showed a significant difference in naso-maxillary morphology. There were some significant correlations between the dental arch expansion and change of each naso-maxillary component, suggesting the effect of expansion stress on the naso-maxillary complex in UCLP. However, deformation caused by expansion stress varied, depending on each component of the naso-maxillary complex. (author) 61 refs

  11. An unusual type of sucking habit in a patient with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Satyaprasad Savitha

    2009-01-01

    Full Text Available Digit sucking, a form of non-nutritive sucking, is a habit of concern to specialist in various fields such as psychiatrist, psychologist, pediatricians, pediatric dentists, orthodontist, speech pathologist and plastic surgeon. The habits have harmful unbalanced pressures to be born by the immature highly malleable alveolar ridges. Sucking behaviors have long been recognized to affect occlusion and dental arch characteristics. As early as 1870s, Campbell and Chandler recognized that prolonged finger or thumb sucking habits had deleterious effects on certain occlusal traits including anterior open bite, increased over jet and class II canine and molar relationships. However, little is known about digit sucking habit and its effect in a cleft lip and palate child as there is no literature till now reported on the digit sucking in a cleft lip patient.

  12. Analysis for speech and esthetics in sixty consecutive patients with cleft lip and palate

    Science.gov (United States)

    Shiraganvi, Mahantesh S; Kumar, N; Desai, A; Kiran, TUR; Gopalkrishnan, K

    2012-01-01

    Background A double-blind retrospective study was carried out at our oral and maxillofacial surgery department to assess speech and esthetics of primary cheiloplasty in patients operated for unilateral complete cleft lip, alveolus and palate. Materials and Methods Total sample size was 60. All were operated for unilateral complete cleft lip, alveolus and palate. Age range was between 1 and 21 years. Results of surgeries performed by two surgeons were assessed. The speech of all these patients was judged by a single speech therapist who was unaware of the operating surgeon. All patients were assessed for articulatory errors, namely, omission, distortion, substitution, addition and intelligibility. Sixty-eight words in local language (Kannada) were selected by the speech pathologist. All patients were subdivided into three age groups: ≤5 years, 6-10 years and ≥11 years. The cheiloplasty was assessed using VLS (V: vermilion, L: lip, S: scar) scale for vermilion, lip and scar patterns. Results and Conclusion Patients undergoing palatoplasty in ≤1 year showed good articulation between the age of 5 and 10 years. Articulation and intelligibility was maximum in patients ≥11 years. Patients at this age try various compensatory mechanisms to overcome communication disabilities. Based on the VLS scale, scarring was the least in all patients. Vermilion and lip patterns showed satisfactory results in most of the patients. PMID:25756010

  13. A Model Humanitarian Cleft Mission: 312 Cleft Surgeries in 7 Days

    Directory of Open Access Journals (Sweden)

    Ghulam Qadir Fayyaz, MBBS, DSS, MS

    2015-03-01

    Conclusion: A locoregional cleft team can be more effective to care for the patients with cleft in countries where surgical and other expertise can be utilized by proper organization of cleft missions on a national level.

  14. Prosthodontic Rehabilitation Alternative of Patients with Cleft Lip and Palate (CLP: Two Cases Report

    Directory of Open Access Journals (Sweden)

    Emrah Ayna

    2009-01-01

    Full Text Available Although patients with cleft lip and palate (CLP are not seen regularly in general dental practice, this is a frequent congenital anomaly; approximately one in every 800 live births results in a CLP. The cause of CLP is unknown, but possible causes are malnutrition and irradiation during pregnancy, psychological stress, teratogenic agents, infectious agents (viruses, and inheritance. Most clefts are likely caused by multiple genetic and nongenetic factors. Prosthetic reconstruction of the anterior maxilla is important for these patients. This paper describes the prosthetic rehabilitation of two patients with CLP, 19-year-old and 21-year-old women, both with surgically treated CLP. In both, an examination revealed a residual palatal defect of 2×3 mm and missing maxillary lateral incisors. The 19-year-old was treated with a fiber-reinforced composite resin-bonded fixed partial denture. The 21-year-old was treated with a removable partial denture with an extracoronal attachment system. The prosthetic rehabilitation of the two patients with CLP was evaluated clinically. In both, well-planned prosthetic, periodontal, and surgical therapy resulted in satisfactory function and esthetics, alleviating their deformities. With education and appropriate recall, the patients should be able to maintain their oral health.

  15. Closure of huge palatal fistula in an adult patient with isolated cleft palate: a technical note.

    Science.gov (United States)

    Rahpeyma, Amin; Khajehahmadi, Saeedeh

    2015-02-01

    Closure of huge palatal fistula surrounded by fully erupted permanent dentition in the adult patients with cleft is a challenge. Posteriorly based buccinator myomucosal flap is a neurovascular pedicled flap, with inherent nature of thin thickness, saliva secretion, and axial pattern blood supply. Vicinity of donor site to the palate and low donor-site morbidity are the other advantages. It is an ideal choice in such situation. In this article, the details of surgical technique and the effectiveness of this method are presented. PMID:25750845

  16. Closure of Huge Palatal Fistula in an Adult Patient with Isolated Cleft Palate: A Technical Note

    Directory of Open Access Journals (Sweden)

    Amin Rahpeyma, DDS

    2015-02-01

    Full Text Available Summary: Closure of huge palatal fistula surrounded by fully erupted permanent dentition in the adult patients with cleft is a challenge. Posteriorly based buccinator myomucosal flap is a neurovascular pedicled flap, with inherent nature of thin thickness, saliva secretion, and axial pattern blood supply. Vicinity of donor site to the palate and low donor-site morbidity are the other advantages. It is an ideal choice in such situation. In this article, the details of surgical technique and the effectiveness of this method are presented.

  17. Secondary alveolar bone grafting in cleft of the lip and palate patients

    Directory of Open Access Journals (Sweden)

    Abhilashaa Walia

    2011-01-01

    Full Text Available Aim: The aim was to restore the function and form of both arches with a proper occlusal relationship and eruption of tooth in the cleft area. Materials and Methods: Eleven patients were selected irrespective of sex and socio-economic status and whose age was within the mixed dentition period. Iliac crest is grafted in cleft area and subsequently evaluated for graft success using study models, and periapical and occlusal radiographs. Results: At the time of evaluation teeth were erupted in the area and good alveolar bone levels were present. Premaxilla becomes immobile with a good arch form and arch continuity. There are no major complications in terms of pain, infection, paraesthesia, hematoma formation at donor site without difficulty in walking. There is no complication in terms of pain, infection, exposure of graft, rejection of graft, and wound dehiscence at the recipient site. Discussion: It is evident that secondary alveolar grafting during the mixed dentition period is more beneficial for patients at the donor site as well as the recipient site. Conclusion: Long-term follow-up is required to achieve maximum advantage of secondary alveolar grafting; the age of the patient should be within the mixed dentition period, irrespective of sex, socio-economic status. It may be unilateral or bilateral.

  18. The Management of Iatrogenic Obstructive Sleep Apnoea Syndrome Following Bimaxillary Surgery in a Patient with Cleft Lip and Palate.

    Science.gov (United States)

    Gerbino, Giovanni; Gervasio, Fernando Carmine; Blythe, John; Bianchi, Francesca Antonella

    2016-07-01

    A 26-year-old man presented with a 6-year history of severe obstructive sleep apnoea syndrome followed a bimaxillary osteotomy procedure for a class III skeletal pattern. The patient was born with a unilateral cleft lip and palate and underwent primary lip and palate repair and later a pharyngeal flap for severe velopharyngeal insufficiency. Surgical management of obstructive sleep apnoea syndrome with conventional osteotomy, in cleft lip and palate patients, is a difficult problem. Distraction osteogenesis may provide a safer alternative. The authors describe and discuss the indications and the technical challenge of a multistage treatment protocol with distraction osteogenesis. PMID:27391499

  19. Morphology of bone defects in patient with unilateral cleft lip and palate. Cone beam x-ray CT evaluation

    International Nuclear Information System (INIS)

    Orthodontic treatment planning of the cleft lip and palate vary according to the morphology of the alveolar bone and palatal bone. The purpose of this study is to evaluate the three-dimensional anatomy of the alveolar and palatal bone in children with complete unilateral cleft lip and palate. Thirty-three nonsyndromic consecutive patients with complete unilateral cleft lip and palate were treated by the cleft palate team at Showa University. Each patient had lip and palate surgeries at Showa University. Cone beam CT radiographs (CB MercuRay, Hitachi) were taken prior to secondary bone grafting, and were classified according to the method of Kita et al. 1997. Cone beam CT radiographs showed multiple types of alveolar and palatal bone morphology, and focused on special types described in the method of Kita et al. It was most frequently found that bone defects in the alveolar crest showed similar patterns in both buccal and palatal aspect, and the buccal bone defect in the nasal floor was larger than the palatal bone defect in the nasal floor. In 80% of the patients, the palatal bone defect showed similar patterns in both anterior and posterior aspects, and the anterior palatal bone defect was smaller than the posterior palatal bone defect. In addition, inadequate bone bridges were frequently found at the cleft site. It is suggested that patients with unilateral cleft lip and palate have various types of alveolar and palatal bone morphology, and are required to take three-dimensional radiographic X-rays prior to any orthodontic treatment. (author)

  20. Value of acoustic perceptual method for analysis of compensatory articulation errors in postoperative patients with cleft palate

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objective To establish an acoustic perceptual method analyzing the compensatory articulation errors in children with operated cleft palate via the formants of Chinese pure vowels. Methods The first three formants which represent vocal transmission character in Chinese pure vowels of 84 subjects with operated cleft palate, were measured by Computerized Speech Signal Processing System (CSSPS). The Chinese vowel graph of postoperative patients with cleft palate was stated by the first formant frequencies (F1) and the second formant frequencies (F2) of the Chinese pure vowels between the two groups. Results Values of F1 and F2 of vowels except [a] in the poor articulation group (Group A) were significantly different from those in the good articulation group (Group B) (P<0.05 or P<0.01). As compared with that in Group B, the vowel graph demonstrated the decreased perceptual distances in Group A. These findings indicated that there might still be the backward movements of tongue, perverted mandibular movements and disharmonious lip movements in addition to the velopharyngeal insufficiency (VPI) in Group A. Conclusion The speech articulation of children with repaired cleft palate should be gained by correcting the aberrant compensatory articulation errors in the condition of velopharyngeal competence. Computerized Speech Signal Processing System (CSSPS), which is regarded as the content of objective quantitative measurement, is a precise, simple, reliable and atroumatic technique for children with cleft palate to analyze pathological compensatory articulation errors.

  1. American College of Prosthodontists

    Science.gov (United States)

    ... Mouth Broken or Chipped Tooth C Cavities or Tooth Decay Cleft Lip/Palate Congenital Dental Defects Cracked Tooth ... in Mouth Broken or Chipped Tooth Cavities or Tooth Decay Cleft Lip/Palate Congenital Dental Defects Cracked Tooth ...

  2. Evaluation of nasal cartilage using three-dimensional soft tissue images in patients with unilateral cleft lip

    International Nuclear Information System (INIS)

    In the treatment of nasal deformities associated with cleft lip and palate, deformities of the alar cartilage and upper lateral cartilage are usually repaired. It is very useful if deformities of the nasal cartilage are evaluated preoperatively. We created three-dimensional CT images of soft tissues by the volume rendering method, the nasal cartilage. In 26 patients with unilateral cleft lip and palate, the alar cartilage, upper lateral cartilage, and septal cartilage were evaluated morphologically. As a result, in each case, these cartilages were deviated and deformed. However, the size of both the alar cartilage and the upper lateral cartilage on the cleft side were approximately similar to those on the healthy side. It is suggested that using this method formulated for the imaging of cartilaginous morphology, preoperative planning and follow-up can be performed easily. (author)

  3. Cleft Lip and Cleft Palate

    Science.gov (United States)

    ... months of age. Your child may need more surgery for oral clefts as he grows. Your baby gets treated ... months of age. Your child may need more surgery for oral clefts as he grows. Your baby gets treated ...

  4. Amelogenin gene influence on enamel defects of cleft lip and palate patients

    Directory of Open Access Journals (Sweden)

    Fernanda Veronese OLIVEIRA

    2014-08-01

    Full Text Available The aim of this study was to investigate the occurrence of mutations in the amelogenin gene (AMELX in patients with cleft lip and palate (CLP and enamel defects (ED. A total of 165 patients were divided into four groups: with CLP and ED (n=46, with CLP and without ED (n = 34, without CLP and with ED (n = 34, and without CLP or ED (n = 51. Genomic DNA was extracted from saliva followed by conducting a Polymerase Chain Reaction and direct DNA sequencing of exons 2 through 7 of AMELX. Mutations were found in 30% (n = 14, 35% (n = 12, 11% (n = 4 and 13% (n = 7 of the subjects from groups 1, 2, 3 and 4, respectively. Thirty seven mutations were detected and distributed throughout exons 2 (1 mutation – 2.7%, 6 (30 mutations – 81.08% and 7 (6 mutations – 16.22% of AMELX. No mutations were found in exons 3, 4 or 5. Of the 30 mutations found in exon 6, 43.34% (n = 13, 23.33% (n = 7, 13.33% (n = 4 and 20% (n = 6 were found in groups 1, 2, 3 and 4, respectively. c.261 C > T (rs2106416, a silent mutation, was detected in 26 subjects, and found more significantly (p = 0.003 in patients with CLP (groups 1 and 2 – 23.75%, compared with those without CLP (groups 3 and 4 – 8.23%. In the groups without ED, this silent mutation was also found more significantly (p = 0.032 among subjects with CLP (17.65% in group 2, compared with those without CLP (7.8% in group 4. In conclusion, this study suggested that AMELX may be a candidate gene for cleft lip and palate.

  5. IRF6 Sequencing in Interrupted Clefting.

    Science.gov (United States)

    Cuddapah, Sanmati R; Kominek, Selma; Grant, John H; Robin, Nathaniel H

    2016-05-01

    In a retrospective review of patients seen at the University of Alabama at Birmingham Cleft and Craniofacial Center, four patients with rare interrupted clefting were identified who had undergone genetic testing. Each of these patients had a typical cleft lip, with intact hard palate and cleft of the soft palate. Given this picture of mixed clefting, IRF6 sequencing was done and was negative for mutations in all four patients. As genetic testing for single-gene mutations and exome sequencing become clinically available, it may be possible to identify novel mutations responsible for this previously unreported type of interrupted clefting. PMID:26090788

  6. Association of Single Nucleotide Polymorphisms in IRF6 and TGFA Genes With Nonsyndromic Cleft Lip With Or Without Cleft Palate in Chinese Patients

    Institute of Scientific and Technical Information of China (English)

    Ya Shen; Yugui Cu; Weidong Wan; Xiaoping Zhou; Lu Cheng; Zuhong Lu; Jiayin Liu

    2009-01-01

    Objective:Nonsyndromic cleft lip with or without cleft palate(NSCL/P) is a common birth defect with unclear etiology.Both genetic and environmental factors may contribute to NSCL/P.Many genes have been identified as candidate genes associated with this disease.Interferon regulatory factor6(IRF6) gene and transforming growth factor-a(TGFA) gene seem to be cmcial in the predisposition of NSCL/P.Here we evaluated some single nucleotide polymorphisms(SNPs) loci of TGFA and IRF6 genes in Chinese nuclear families consisting of fathers,mothers and affected offspring with NSCL/P.Methods:Fifty patients of NSCL/P were confirmed by the plastic surgeons.They and their parents were included in the study,all with the informed consents.SNPs loci of TGFA and IRF6 genes were analyzed by microarray technology.Some PCR products were randomly chosen and sequenced to check microarray results.The distribution of gene type and allele frequency between patient group and parents group were compared.Then a Haplotype Relative Risk(HRR) and Transmis-sion Disequilibrium Test(TDT) were performed.Results:The sequences of randomly selected PCR products were all consistent with the microarray results.All loci were in Hardy-Weinberg equilibrium.There were no significant differences in the distribution of genotypes and alleles between patients and their parents.Using HRR and TDT analyses the V2741 of IRF6 was associated with NSCL/P,while another SNP locus of IRF6 was not.Strong evidence of linkage disequilibrium was found between the2 SNP loci of TGFA and disease with the HRR analysis,but not with the TDT analysis.Conclusion:Our study confirms the contribution of IRF6 in the etiology of NSCL/P in populations of Asian ancestry.The association of TGFA with NSCL/P requires further research.

  7. Parental cigarette smoking, transforming growth factor-alpha gene variant and the risk of orofacial cleft in Iranian infants

    Science.gov (United States)

    Ebadifar, Asghar; Hamedi, Roya; KhorramKhorshid, Hamid Reza; Kamali, Koorosh; Moghadam, Fatemeh Aghakhani

    2016-01-01

    Objective(s): We investigated the influence of genetic variation of the transforming growth-factor alpha (TGFA) locus on the relationship between smoking and oral clefts. Materials and Methods: In this study 105 Iranian infants with non-syndromic cleft lip/palate and 218 controls with non-cleft birth defects were examined to test for associations among maternal exposures, genetic markers, and oral clefts. Maternal and parental smoking histories during pregnancy were obtained through questionnaire. DNA was extracted from newborn screening blood samples, and genotyping of the BamHI polymorphism in the TGFA gene was performed by polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) methods. A number of factors including gender of the newborns, type of oral cleft, consanguinity of the parents, as well as the mother’s age and education were evaluated as potential confounders and effect modifiers. Results: Maternal smoking, in the absence of paternal smoking, was associated with an increased risk for CL/P (OR = 19.2, 95% CI = [(6.2-59.5)]) and cleft palate only (OR =48.7, 95% CI = [(8-29.3)]). If both parents smoked, risks were generally greater (OR = 55.6, 95% CI = [12-20.25]). Analyses for the risk of clefting from maternal smoking, stratified by the presence or absence of the TGFA/BamH1variant, revealed that the risk of clefting among the infants with the TGFA/BamH1 variant when their mothers smoked cigarettes was much greater than the infants who had non-smoker mothers (P=0.001, OR=10.4,95% CI=[3.2,33.6]). Conclusion: The results of this study indicate that first-trimester maternal smoking and infant TGFA locus mutations are both associated with nonsyndromic cleft lip and/or palate (CL/P). PMID:27279979

  8. Lip movement in patients with a history of unilateral cleft lip.

    NARCIS (Netherlands)

    Rutjens, C.A.W.; Spauwen, P.H.M.; Lieshout, P.H.H.M. van

    2001-01-01

    OBJECTIVE: The influence of a repaired cleft lip on the stability of coordination between upper and lower lip in nonspeech and speech tasks was investigated. DESIGN: First, we looked at the effects of a secondary cleft lip repair in three individuals. Second, we compared subjects with a history of r

  9. Genomic expression in non syndromic cleft lip and palate patients: A review

    OpenAIRE

    Mehrotra, D.

    2015-01-01

    Cleft lip and palate are common congenital anomalies with significant medical, psychological, social, and economic ramifications, affecting one in seven hundred live births. Genetic causes of non syndromic cleft lip and/or palate (NSCLP) include chromosomal rearrangements, genetic susceptibility to teratogenic exposures, and complex genetic contributions of multiple genes.

  10. IRF6 polymorphisms in Mexican patients with non-syndromic cleft lip

    Science.gov (United States)

    Ibarra-Arce, Aurora; García-Álvarez, Martín; Cortés-González, Daniel; Ortiz de Zarate-Alarcón, Gabriela; Flores-Peña, Laura; Sánchez-Camacho, Sandra; Arenas-Díaz, Silvia; Romero-Valdovinos, Mirza; Olivo-Díaz, Angélica

    2015-01-01

    Cleft lip with or without cleft palate (CL/P) is one of the most common birth defects; it is a multifactorial disease affecting > 1/1,000 live births in Europe, and its etiology is largely unknown, although it is very likely genetic and environmental factors contribute to this malformation. Orofacial development is a complex process involving many genes and signaling pathways. Mutations in the gene for the interferon regulatory factor 6 (IRF6) cause a hereditary dominant malformation syndrome including CL/P, and polymorphisms are associated with non-syndromic CL/P (MIM 119530). Five SNPs at the locus with high heterozygosity in Caucasian populations were chosen for the present research due to their very strong association with CL/P. A case–parent trio study was performed using 292 samples from Mexico. Association with the rs1319435-C/C genotype (P = 0.02) was found in patients (73) as compared to pseudocontrols (219), while the genotype rs1319435-T/C was related with protection (P = 0.041) in the triad design. Significant over-transmission of the G allele for marker rs2235375 (P = 0.049) was found. Only the TACGT haplotype was diminished in the affected child, either in single (P = 0.0208) or double (P = 0.0208) dose. The pairwise analysis showed rs2235543 and rs2235371 were in strong linkage disequilibrium. These results point to a substantial contribution of IRF6 in the etiology of non-syndromic CL/P in a sample of the Mexican population. PMID:25853057

  11. Presurgical orthodontic treatment of patients with complete bilateral cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Radojičić Julija

    2014-01-01

    Full Text Available Introduction. Cleft lips and palates are the most common congenital orofacial anomaly. This type of clefts is the most severe from the orthodontic-surgical therapy aspect. Case report. A female newborn with a complete cleft of the primary and the secondary palate was admitted to the clinic, where a multiple-role orthodontic device was specially designed and applied to primarily manage the closure of the existing cleft and help to improve the suckling ability of the baby. Besides the fact that it allows breastfeeding, it has a significant orthodontic effect, too. Conclusion. Specificity of this device is the lack of extraoral fixation. What can easily be observed is a progressive reduction of the cleft between the separated segments and the premaxilla retrusion. It, thus, allows the creation of much better conditions for further surgical management of the said defect.

  12. Evaluation of alveolar bone grafting in unilateral cleft lip and palate patients using a computer-aided diagnosis system

    Energy Technology Data Exchange (ETDEWEB)

    Sutthiprapaporn, Pipop; Kongsomboon, Supaporn; Limmonthol, Saowaluck; Pisek, Poonsak; Keinprasit, Chutimaporn [Khon Kaen University, Khon Kaen (Thailand); Tanimoto, Keiji; Nakamoto, Takashi [Dept. of Oral and Maxillofacial Radiology, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima (Japan)

    2012-09-15

    This study aimed to evaluate the trabecular bone changes after alveolar bone grafting in unilateral cleft lip and palate (UCLP) patients using a computer-aided diagnosis (CAD) system. The occlusal radiographs taken from 50 UCLP patients were surveyed retrospectively. The images were categorized as: 50 images in group 0 (before bone grafting), 33 images in group 1 (one month after bone grafting), 24 images in group 2 (2-4 months after bone grafting), 15 images in group 3 (5-7 months after bone grafting), and 21 images in group 4 (8 or more months after bone grafting). Each image was grouped as either 'non-cleft side' or 'cleft side'. The CAD system was used five times for each side to calculate the pixel area based on the mathematical morphology. Significant differences were found using a Wilcoxon signed ranks test or paired samples t test. The pixel area showed a significant difference between the 'non-cleft side' and 'cleft side' in group 0 (404.27{+-}103.72/117.73{+-}92.25; p=0.00), group 1 (434.29{+-}86.70/388.31{+-}109.51; p=0.01), and group 4 (430.98{+-}98.11/366.71{+-}154.59; p=0.02). No significant differences were found in group 2 (423.57{+-}98.12/383.47{+-}135.88; p=0.06) or group 3 (433.02{+-}116.07/384.16{+-}146.55; p=0.19). Based on the design of this study, alveolar bone grafting was similar to normal bone within 2-7 months postoperatively.

  13. Evaluation of alveolar bone grafting in unilateral cleft lip and palate patients using a computer-aided diagnosis system

    International Nuclear Information System (INIS)

    This study aimed to evaluate the trabecular bone changes after alveolar bone grafting in unilateral cleft lip and palate (UCLP) patients using a computer-aided diagnosis (CAD) system. The occlusal radiographs taken from 50 UCLP patients were surveyed retrospectively. The images were categorized as: 50 images in group 0 (before bone grafting), 33 images in group 1 (one month after bone grafting), 24 images in group 2 (2-4 months after bone grafting), 15 images in group 3 (5-7 months after bone grafting), and 21 images in group 4 (8 or more months after bone grafting). Each image was grouped as either 'non-cleft side' or 'cleft side'. The CAD system was used five times for each side to calculate the pixel area based on the mathematical morphology. Significant differences were found using a Wilcoxon signed ranks test or paired samples t test. The pixel area showed a significant difference between the 'non-cleft side' and 'cleft side' in group 0 (404.27±103.72/117.73±92.25; p=0.00), group 1 (434.29±86.70/388.31±109.51; p=0.01), and group 4 (430.98±98.11/366.71±154.59; p=0.02). No significant differences were found in group 2 (423.57±98.12/383.47±135.88; p=0.06) or group 3 (433.02±116.07/384.16±146.55; p=0.19). Based on the design of this study, alveolar bone grafting was similar to normal bone within 2-7 months postoperatively.

  14. Hybrid Maxillofacial Prosthesis: A Case Report

    OpenAIRE

    Thomas, Sunitha; Shyam Mohan, A.; Rupesh, P L

    2010-01-01

    The role of a prosthodontist in the management of cleft lip & palate patients is pertinent involving restoration of mastication, facial harmony, dental harmony and phonation. This article presents the prosthodontic rehabilitation of a congenitally bilateral cleft lip and palate patient with a unique method which fulfilled the patient’s needs, esthetics and psychological well being.

  15. Selected aspects of the oral environment in cleft palate patients – a problem evidently beyond dentists’ scope

    Directory of Open Access Journals (Sweden)

    Joanna AntoszewskaABCD

    2010-12-01

    Full Text Available Introduction:The oral cavity is a specialized ecosystem composed of dentition and mucosa exposed to the effects of saliva and gingival liquid. Its structure provides advantageous conditions for various microorganisms, both aerobic streptococci and anaerobic bacilli. The dynamic balance of the oral cavity ecosystem can be threatened by various factors. Lip and palatal clefts are the most frequent disorders in embryonic facial development. The aim of the study was to evaluate whether Streptococcus mutans and Lactobacillus acidophilus and oral cavity hygiene in patients with cleft palate treated orthodontically are significantly different compared with patients without these congenital malformations.Material/Methods:The study group consisted of 200 patients aged 6–21 who were divided into two groups depending on the presence of cleft palate and treatment method. Group C (control consisted of 50 orthodontically untreated patients with occlusal dental defects. The performed studies consisted of 2 parts: the clinical-laboratory part and statistical analysis.Clinical-laboratory partResults:High bacteria levels of Streptococcus mutans and Lactobacillus acidophilus in saliva of patients were comparably frequent between groups, but there were statistically significant differences in inter-group comparisons.Conclusions:As the orthodontic treatment changes the oral environment, control of proper hygienic behavior is an essential element of health education. Introduction of preventive programs is also very important.

  16. Reconstruction of maxilla alveolus for application of dental implant in patients with cleft defect.

    Science.gov (United States)

    Dusková, M; Kot'ová, M; Urban, F; Sosna, B; Jirkalová, R; Strnadel, T; Kristen, M; Leamerová, E; Gojis, O

    2004-01-01

    The prospective study of dental implant application into the reconstructed maxilla alveolus of cleft patients was started in 2001. Its aims are to specify precisely the indications, conditions and techniques, as well as the factors justifying the assumption that the results will be successful. Indication was based on an examination by a multidisciplinary therapeutic team (plastic surgeon, dental surgeon, orthodontist, and prosthetist), with the proviso that the patient should have a positive approach. Successful orthodontic treatment of intergnathic relation and shape of the dental arches served as the basis. It was followed by a reconstruction of the defective alveolus using autologous cancellous bone grafts, harvested from an iliac crest. Dental implants were inserted 12-15 weeks after the transplantation and subsequently a prosthetic component was applied with a time delay of at least 2 months. After the orthodontic preparation, reconstruction of the alveolus has so far been performed in 38 patients. Their age was at least 14 years, i.e. the age when growth of the orofacial region was finalised or already complete. The crucial tasks at this stage are to form a stable, three-dimensional voluminous alveolar crest, and to cover the grafts with a sufficient amount of quality soft tissue. We needed an average amount of 3.7 ccm of cancellous bone graft for the reconstruction. This amount can be harvested only from an enchondral bone. For soft tissue shell the mucoperiosteal shift of flap from cleft segment was used. In more serious cases contralateral or even bilateral shift was performed. The relief of tissue tension was performed by double cut-back. Using the approach described we attained a 84.2% success rate. Our results and experience derived within this project show that the success rate of this procedure depends on the continuous flow of the alveolar arch with a good intergnathic relation, with a length of defect between crowns of border teeth of at least 8 mm

  17. Impaired epithelial differentiation of induced pluripotent stem cells from ectodermal dysplasia-related patients is rescued by the small compound APR-246/PRIMA-1MET.

    Science.gov (United States)

    Shalom-Feuerstein, Ruby; Serror, Laura; Aberdam, Edith; Müller, Franz-Josef; van Bokhoven, Hans; Wiman, Klas G; Zhou, Huiqing; Aberdam, Daniel; Petit, Isabelle

    2013-02-01

    Ectodermal dysplasia is a group of congenital syndromes affecting a variety of ectodermal derivatives. Among them, ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) syndrome is caused by single point mutations in the p63 gene, which controls epidermal development and homeostasis. Phenotypic defects of the EEC syndrome include skin defects and limbal stem-cell deficiency. In this study, we designed a unique cellular model that recapitulated major embryonic defects related to EEC. Fibroblasts from healthy donors and EEC patients carrying two different point mutations in the DNA binding domain of p63 were reprogrammed into induced pluripotent stem cell (iPSC) lines. EEC-iPSC from both patients showed early ectodermal commitment into K18(+) cells but failed to further differentiate into K14(+) cells (epidermis/limbus) or K3/K12(+) cells (corneal epithelium). APR-246 (PRIMA-1(MET)), a small compound that restores functionality of mutant p53 in human tumor cells, could revert corneal epithelial lineage commitment and reinstate a normal p63-related signaling pathway. This study illustrates the relevance of iPSC for p63 related disorders and paves the way for future therapy of EEC. PMID:23355677

  18. Psychological issues in cleft lip and cleft palate

    Directory of Open Access Journals (Sweden)

    Sousa Avinash

    2009-01-01

    Full Text Available Vocational and social issues affect rehabilitation and development of patients with cleft lip and cleft palate. However, psychological problems like lowered self esteem and difficulties in social interaction have also been noted in them. Not many pediatric reconstructive surgery teams have a psychiatrist on their panel. It is likely that psychological problems are higher in incidence than literature actually suggests. Hence it is very essential that such cases are identified by the surgical team to maximize positive outcome of surgery and rehabilitation. This study discusses psychological issues revolving around cleft lip and cleft palate along with lacunae in many psychological research studies.

  19. Prevalence and management of natal/neonatal teeth in cleft lip and palate patients

    Science.gov (United States)

    Yilmaz, R. Burcu Nur; Cakan, Derya Germec; Mesgarzadeh, Nasim

    2016-01-01

    Objective: The aim of this study was to determine the prevalence and distribution of natal/neonatal teeth in infants with cleft lip and palate (CLP) according to gender, involving jaw and side and to show the management of some cases. Materials and Methods: A retrospective study was carried out on medical history and photographic records of 69 infants with CLP, who were treated at the CLP clinic of Yeditepe University between years 2014–2015. The presence of neonatal teeth was determined, and if present the gender, type of cleft, and position were recorded. Statistical analysis was performed. Results: Neonatal teeth were observed in 7% of the study group. No significant differences were found between cleft types and gender (P > 0.05). The prevalence of neonatal teeth in bilateral, unilateral and isolated cleft type was 16.5%, 6.5%, and none, respectively. All neonatal teeth were located in the maxilla and on the cleft-side (100%). Conclusion: The presence of natal/neonatal teeth in infants with CLP was not rare. In all of these cases the teeth were located adjacent to the cleft region. In isolated palatal cleft, where the alveolar region including the teeth buds are away from the cleft, no neonatal teeth were observed. It may be concluded that neonatal teeth in infants with CLP are frequently present and located inside the borders of the presurgical orthopedic treatment (POT) plate. Therefore, if possible, immediate extraction of the neonatal teeth is advised or if not possible because of systemic health reasons, modifications of the plate are required. PMID:27011740

  20. The clinical study of ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome%先天性缺指(趾)-外胚层发育不良-唇/腭裂综合征的临床研究

    Institute of Scientific and Technical Information of China (English)

    宋宇鹏; 杨庆华; 蒋海越; 庄洪兴

    2010-01-01

    目的 探讨先天缺指(趾)-外胚层发育不良-唇/腭裂综合征的临床表现,诊断标准,遗传学特点及治疗措施.方法 2008年3月至2009年9月收集先天缺指(趾)-外胚层发育不良-唇/腭裂综合征女性患者一例,针对上唇缺损行手术修补,对其进行家系问卷和DNA检查,观察患者的临床表型和发病特点,分析可能的遗传方式.结果 术后患者伤口愈合良好,无并发症发生,此例患者随访半年,术后明显改善了患者的外观,研究收集的这个典型病例未追溯到明显家族遗传史.结论 收集的一个患者属典型的散发病例.通过对该患者的诊疗,证明治疗应是多学科的,主要是针对外观进行整形外科手术,临床的早期检查和正确诊断对后期治疗具有重要意义.产前诊断意义尤为重大.

  1. 先天缺指(趾)-外胚叶发育不良-唇/腭裂综合征的病例研究%Cases Study of Chinese Kindreds with Ectrodactyly, Ectodermal Dysplasia and Cleft Lip/palate Syndrome

    Institute of Scientific and Technical Information of China (English)

    张景霞; 郝杰兵; 金辉喜; 边专; 叶晓茜

    2007-01-01

    目的:探讨先天缺指(趾)-外胚叶发育不良-唇/腭裂综合征(EEC)的临床表型和遗传学特点.方法:收集具有典型症状的EEC病例并进行家系问卷和口腔检查,观察各家系患者的临床表型和发病特点,分析可能的遗传方式,绘制系谱图.结果:研究收集的2个EEC病例均未追溯到明显家族遗传史,患者均表现出明显的双侧多个缺指(趾),并指,唇/腭裂,家系2患者有毛发稀疏,指甲无光泽等明显外胚层发育不良表型,符合典型的EEC综合征.2名不同患者的严重程度存在明显差异.结论:收集的2个患者均属典型的散发EEC病例,临床的早期检查和正确诊断对后期治疗具有重要意义.

  2. Patients and professionals have different views on online patient information about cleft lip and palate (CL/P).

    Science.gov (United States)

    van den Bosch, S; Koudstaal, M; Versnel, S; Maal, T; Xi, T; Nelen, W; Bergé, S; Faber, M

    2016-06-01

    Parents of children with a cleft lip and/or palate (CL/P) and patients with CL/P actively search for online information about CL/P. The quality of this information has not been sufficiently evaluated. The aim of this study was to define quality criteria for online information about CL/P and assess the quality of frequently accessed websites. Patients, parents, and professionals were equally involved in all stages of this study. A literature review was performed to obtain known quality criteria for online information. These criteria were prioritized by patients, parents, and professionals. The most important criteria were used to rate the quality of four websites on CL/P. Forty-two quality items were extracted from the literature. Patients, parents, and professionals agreed on the importance of 16 of these items. New groups of patients, parents, and professionals assessed four websites on CL/P. Although the groups were like-minded in their overall assessment of the quality of the websites, distinct differences emerged between the groups in relation to certain items. This study shows the importance of patient participation in healthcare research, as well as a feasible approach to do so. Involving patients in composing online health information will set different priorities, which is necessary in establishing high quality information. PMID:26718136

  3. Dental anomalies in different cleft groups related to neural crest developmental fields contributes to the understanding of cleft aetiology

    DEFF Research Database (Denmark)

    Riis, Louise Claudius; Kjær, Inger; Mølsted, Kirsten

    2014-01-01

    , radiographs, dental casts, and medical records. PATIENTS: Ninety individuals (30 cleft lip, 30 cleft palate, and 30 combined cleft lip and palate), aged 5-27 years. MAIN OUTCOME MEASURES: Visual evaluation of tooth number and tooth morphology. RESULTS: Cleft lip: Dental deviations were predominantly observed...... in the frontonasal field. Supernumerary lateral incisors occurred significantly more often in cleft lip compared to other cleft types. Cleft palate: Dental deviations were observed in the maxillary and palatal fields indicating that both fields are involved in the development of cleft palate....... Malformed roots were seen significantly more often in cleft palate. Combined cleft lip and palate: Number and type of dental deviations differed significantly from deviations in other cleft types, e.g. significantly more ageneses. CONCLUSIONS: Cleft lip seems to be caused by a disorder in neural crest...

  4. Oral health related quality of life in cleft lip and palate patients rehabilitated with conventional prostheses or dental implants

    OpenAIRE

    Papi, Piero; Giardino, Rita; Sassano, Pierpaolo; Amodeo, Giulia; Pompa, Giorgio; Cascone, Piero

    2015-01-01

    Objectives: Cleft lip and/or palate (CLP) is the most common congenital craniofacial abnormality, with a prevalence of 9.92 per 10,000 live births. In treating patients with CLP, oral rehabilitation is definitely a very important phase of the treatment in order to improve the patient's oral health related quality of life (OH-QoL). The aim of this retrospective study is to assess the OH-QoL in patients rehabilitated with different prosthetic options, thus comparing the conventional treatments,...

  5. Diagnosis and fine localization of deletion region in Wolf Hirschhorn syndrome patients

    Institute of Scientific and Technical Information of China (English)

    JI Tao-yun; David CHIA; WANG Jing-min; WU Ye; LI Jie; XIAO Jing; JIANG Yu-wu

    2010-01-01

    Background Wolf-Hirschhorn syndrome (WHS) results from the partial deletion of 4p. This study aimed to identify and fine map the chromosome deletion regions of Chinese children with Wolf-Hirschhorn syndrome among the developmental delay/mental retardation (DD/MR) patients.Methods We analyzed the relationship of phenotype and genotype. Inclusion criteria were: moderate to severe DD/MR, no definite perinatal brain injury, and no trauma, toxication, hypoxia, infection of central nervous system; routine karyotyping was normal, no evidence of typical inherited metabolic disorder or specific neurodegenerative disorders from cranial neuro-imaging and blood/urinary metabolic diseases screening; no mutation of FMR1 in male patients, no typical clinical manifestation of Rett syndrome in female patients. Multiplex ligation-dependent probe amplification (MLPA) and Affymetrix genome-wide human SNP array 6.0 assays were applied to accurately define the exact size of subtelomeric aberration region of four WHS patients.Results All four WHS patients presented with severe DD, hypotonia and microcephaly, failure to thrive, 3/4 patients with typical facial features and seizures, 2/4 patients with congenital heart defects and cleft lip/palate, 1/4 patients with other malformations. The length of the deletions ranged from 3.3 Mb to 9.8 Mb. Two of four patients had "classic" WHS, 1/4 patients had "mild"-to-"classic" WHS, and 1/4 patients had "mild" WHS.Conclusions WHS patients in China appear to be consistent with those previously reported. The prevalence of signs and symptoms, distribution of cases between "mild" and "classic" WHS, and the correlation between length of deletion and severity of disease of these patients were all similar to those of the patients from other populations.

  6. Cleft Lip – A Comprehensive Review

    OpenAIRE

    Shkoukani, Mahdi A.; Chen, Michael; Vong, Angela

    2013-01-01

    Orofacial clefts comprise a range of congenital deformities and are the most common head and neck congenital malformation. Clefting has significant psychological and socio- economic effects on patient quality of life and require a multidisciplinary team approach for management. The complex interplay between genetic and environmental factors play a significant role in the incidence and cause of clefting. In this review, the embryology, classification, epidemiology, and etiology of cleft lip ar...

  7. Exposure of Cleft Lip and Palate Patients to Toxic Elements Released during Orthodontic Treatment in the Study of Non-Invasive Matrices

    Science.gov (United States)

    Mikulewicz, Marcin; Kachniarz, Krzysztof; Chojnacka, Katarzyna

    2015-01-01

    The Objective The aim of the study was evaluation of metal ions (nickel and chromium) released from orthodontic appliances in cleft lip and palate patients and the usefulness of non-invasive matrices (saliva and hair). Materials and Methods The material studied consisted of 100 individuals, including 59 females and 41 males of 5 to 16 years of age, which were divided into 3 groups: experimental–patients with cleft lip and palate (36 individuals, the average treatment time 5.74 years); control group–patients without cleft lip and palate, during orthodontic treatment (32 individuals, the average treatment time 1.78 years) and the control group patients without cleft lip and palate, without any orthodontic appliances (32 individuals). Samples (saliva, hair) were collected and subjects underwent a survey by questionnaire. Multi-elemental analyses of the composition of non-invasive matrices was conducted in an accredited laboratory by inductively coupled plasma spectrometry technique ICP-OES. The results were reported as mean contents of particular elements (Cd, Cr, Cu, Fe, Mn, Mo, Ni, Si) in hair and in saliva. Results The concentration of Cr, Ni, Fe and Cu ions in saliva of cleft lip and palate patients were several times higher as compared with not treated orthodontically control groups and higher than in the group with orthodontic appliances. Among the assessed matrices, hair of cleft lip and palate patients seem to be not a meaningful biomarker. Conclusion It was found that orthodontic appliances used in long-term treatment of cleft lip and palate patients do not release toxic levels of Cr and Ni ions. PMID:26544176

  8. Exposure of Cleft Lip and Palate Patients to Toxic Elements Released during Orthodontic Treatment in the Study of Non-Invasive Matrices.

    Directory of Open Access Journals (Sweden)

    Marcin Mikulewicz

    Full Text Available The aim of the study was evaluation of metal ions (nickel and chromium released from orthodontic appliances in cleft lip and palate patients and the usefulness of non-invasive matrices (saliva and hair.The material studied consisted of 100 individuals, including 59 females and 41 males of 5 to 16 years of age, which were divided into 3 groups: experimental-patients with cleft lip and palate (36 individuals, the average treatment time 5.74 years; control group-patients without cleft lip and palate, during orthodontic treatment (32 individuals, the average treatment time 1.78 years and the control group patients without cleft lip and palate, without any orthodontic appliances (32 individuals. Samples (saliva, hair were collected and subjects underwent a survey by questionnaire. Multi-elemental analyses of the composition of non-invasive matrices was conducted in an accredited laboratory by inductively coupled plasma spectrometry technique ICP-OES. The results were reported as mean contents of particular elements (Cd, Cr, Cu, Fe, Mn, Mo, Ni, Si in hair and in saliva.The concentration of Cr, Ni, Fe and Cu ions in saliva of cleft lip and palate patients were several times higher as compared with not treated orthodontically control groups and higher than in the group with orthodontic appliances. Among the assessed matrices, hair of cleft lip and palate patients seem to be not a meaningful biomarker.It was found that orthodontic appliances used in long-term treatment of cleft lip and palate patients do not release toxic levels of Cr and Ni ions.

  9. 3-D shape analysis of palatal surface in patients with unilateral complete cleft lip and palate

    Czech Academy of Sciences Publication Activity Database

    Rusková, H.; Bejdová, Š.; Peterka, Miroslav; Krajíček, V.; Velemínská, J.

    2014-01-01

    Roč. 42, č. 5 (2014), e140-e147. ISSN 1010-5182 Grant ostatní: GA UK(CZ) 309611; GA MŠk(CZ) MSM0021620843 Institutional support: RVO:68378041 Keywords : unilateral cleft of lip and palate * palate shape * surface scanning Subject RIV: FF - HEENT, Dentistry Impact factor: 2.933, year: 2014

  10. Craniofacial, craniocervical, and pharyngeal morphology in bilateral cleft lip and palate and obstructive sleep apnea patients

    NARCIS (Netherlands)

    Oosterkamp, B.C.M.; Remmelink, H.J.; Pruim, G.J.; Hoekema, A.; Dijkstra, P.U.

    2007-01-01

    Objective: The aim of this study was to analyze craniofacial, craniocervical, and pharyngeal morphology in surgically treated bilateral cleft lip and palate (BCLP) men, untreated men with obstructive sleep apnea (OSA), and a reference group of men. Subjects and methods: Lateral cephalograms were obt

  11. Contribution to facial morphology study of the unilateral cleft lip and palate patients, by cephalometric radiographs, in frontal norm

    International Nuclear Information System (INIS)

    A study by cephalometric radiography, in frontal norm, is used to measure the magnitude of some linear dimensions of the face, in a sample of a 100 Brazilian, caucasian people. They were from both sexes, with average age of 20 years and were distributed into two groups: control group and unilateral cleft lip and palate group. Cephalometric radiography in frontal norm is obtained of each patient. In each teleradiography some parameters were measured by using a computer. An statistic analysis of the values obtained is presented. (M.A.C.)

  12. 唇腭裂患者口腔健康状况的研究进展%Research progress on oral health status of patient with oral clefts

    Institute of Scientific and Technical Information of China (English)

    柴丛娜

    2011-01-01

    唇腭裂患者的口腔健康状况为近年来的研究热点之一.大量调查表明,唇腭裂患者是龋病和牙周组织疾病的易患人群.其龋病和牙周组织疾病的发生与外科手术、解剖因素、唇腭裂类型因素等密切相关.不同唇腭裂类型的患者口腔卫生状况不一致,不同国家地区的唇腭裂患者的口腔卫生状况也不尽相同.本文就唇腭裂患者的龋病流行特征、牙周疾病特征和口腔卫生状况等研究进展作一综述.%The oral health status of oral clefts has become a hot research spot in recent years. The survey shows that patients with oral clefts remain in the high-risk group for dental caries and periodontal disease. Surgical interventions combined anatomical defects, and cleft status was major factors for dental caries and periodontal disease in patients with oral clefts. For the past few years, significant difference was found in oral hygiene among different cleft types and in different countries of the cleft subjects. The studies of the caries prevalence, periodontal disease, influencing factors and oral hygiene of patients with oral clefts were summarized.

  13. Inferior Turbinate Flap for Nasal-side Closure of Palatal Fistula in Cleft Patients: Technical Note

    OpenAIRE

    Amin Rahpeyma; Saeedeh Khajehahmadi

    2015-01-01

    Summary: Residual palatal fistula after repair of palatal cleft is common. Repair of residual oronasal fistula is not always successful. Two-layer closure techniques that close these fistulas with soft tissue are a common practice. Turnover flaps are the most used flaps and often the sole method for nasal-side closure of fistula. Anteriorly based inferior turbinate flap can be used to provide soft tissue for nasal-side closure when turnover flaps will not provide sufficient tissue for this pu...

  14. Three-dimensional imaging methods for quantitative analysis of facial soft tissues and skeletal morphology in patients with orofacial clefts: a systematic review.

    Directory of Open Access Journals (Sweden)

    Mette A R Kuijpers

    Full Text Available BACKGROUND: Current guidelines for evaluating cleft palate treatments are mostly based on two-dimensional (2D evaluation, but three-dimensional (3D imaging methods to assess treatment outcome are steadily rising. OBJECTIVE: To identify 3D imaging methods for quantitative assessment of soft tissue and skeletal morphology in patients with cleft lip and palate. DATA SOURCES: Literature was searched using PubMed (1948-2012, EMBASE (1980-2012, Scopus (2004-2012, Web of Science (1945-2012, and the Cochrane Library. The last search was performed September 30, 2012. Reference lists were hand searched for potentially eligible studies. There was no language restriction. STUDY SELECTION: We included publications using 3D imaging techniques to assess facial soft tissue or skeletal morphology in patients older than 5 years with a cleft lip with/or without cleft palate. We reviewed studies involving the facial region when at least 10 subjects in the sample size had at least one cleft type. Only primary publications were included. DATA EXTRACTION: Independent extraction of data and quality assessments were performed by two observers. RESULTS: Five hundred full text publications were retrieved, 144 met the inclusion criteria, with 63 high quality studies. There were differences in study designs, topics studied, patient characteristics, and success measurements; therefore, only a systematic review could be conducted. Main 3D-techniques that are used in cleft lip and palate patients are CT, CBCT, MRI, stereophotogrammetry, and laser surface scanning. These techniques are mainly used for soft tissue analysis, evaluation of bone grafting, and changes in the craniofacial skeleton. Digital dental casts are used to evaluate treatment and changes over time. CONCLUSION: Available evidence implies that 3D imaging methods can be used for documentation of CLP patients. No data are available yet showing that 3D methods are more informative than conventional 2D methods

  15. A new primary cleft lip repair technique tailored for Asian patients that combines three surgical concepts: Comparison with rotation--advancement and straight-line methods.

    Science.gov (United States)

    Funayama, Emi; Yamamoto, Yuhei; Furukawa, Hiroshi; Murao, Naoki; Shichinohe, Ryuji; Hayashi, Toshihiko; Oyama, Akihiko

    2016-01-01

    Various techniques have been described for unilateral cleft lip repair. These may be broadly classified into three types of procedure/concept: the straight-line method (SL; Rose-Thompson effect); rotation-advancement (RA; upper-lip Z-plasty); and the triangular flap method (TA; lower-lip Z-plasty). Based on these procedures, cleft lip repair has evolved in recent decades. The cleft lip repair method in our institution has also undergone several changes. However, we have found that further modifications are needed for Asian patients who have wider philtral dimples and columns than Caucasians, while following the principles of the original techniques mentioned above. Here, we have incorporated the advantages of each procedure and propose a refined hybrid operating technique, seeking a more appropriate procedure for Asian patients. To evaluate our new technique, a comparison study was performed to evaluate RA, SL, and our technique. We have used our new technique to treat 137 consecutive cleft lip cases of all types and degrees of severity, with or without a cleft palate, since 2009. In the time since we adopted the hybrid technique, we have observed improved esthetics of the repaired lip. Our technique demonstrated higher glance impression average scores than RA/SL. PMID:26653337

  16. A STUDY OF AIRWAY COMPLICATIONS AMONG THE PATIENTS OF CLEFT LIP & CLEFT PALATE DURING INTRA OPERATIVE & POST OPERATIVE PERIOD AT GOVERNMENT GENERAL HOSPITAL, GUNTUR

    OpenAIRE

    Polaiah; Madhavi; Thokala

    2015-01-01

    BACK GROUND : As Anesthesia for the children undergoing cleft lip and palate repairs surgeries are associated with a variety of airway related problems , we have to study in detail about this kind of morbidity in the study group. OBJECTIVES: 1.To identify the demographic profiles of the study group. 2. To know the various airway complicatio ns developed among the study subject during intra and post - operative periods. STUDY DESIGN : It...

  17. Oral health related quality of life in cleft lip and palate patients rehabilitated with conventional prostheses or dental implants

    Science.gov (United States)

    Papi, Piero; Giardino, Rita; Sassano, Pierpaolo; Amodeo, Giulia; Pompa, Giorgio; Cascone, Piero

    2015-01-01

    Objectives: Cleft lip and/or palate (CLP) is the most common congenital craniofacial abnormality, with a prevalence of 9.92 per 10,000 live births. In treating patients with CLP, oral rehabilitation is definitely a very important phase of the treatment in order to improve the patient's oral health related quality of life (OH-QoL). The aim of this retrospective study is to assess the OH-QoL in patients rehabilitated with different prosthetic options, thus comparing the conventional treatments, which include removable partial dentures and fixed partial dentures, with the implant-supported prostheses. Materials and Methods: Sixty-three patients were enrolled in this retrospective survey [44 females (69.84%) and 19 males (30.16%)] with a mean age of 34.93 ± 7.04 years (age range 21–53 years). They were all treated for CLP and rehabilitated with a conventional prosthesis or an implant-supported denture. Two different questionnaires were used in the present study to evaluate patients’ OH-QoL: The Italian version of the 49-item Oral Health Impact Profile (OHIP-49) and the Italian version of the Cleft Evaluation Profile (CEP). Statistical analysis was performed using analysis of variance (ANOVA) test, with a significant P < 0,05. Results: Data analysis revealed that patients rehabilitated with implant-supported dentures and fixed partial dentures showed a good level of satisfaction with their prostheses, scoring low values in the OHIP-49 and high values in the CEP, while subjects with removable partial dentures scored the highest values in the OHIP-49 and the lowest values in the CEP, which means an unsatisfactory feeling (P < 0.05). Conclusions: OH-QoL is a challenging demand for all prosthodontists. Our results show, clearly, that patients rehabilitated with implant-supported dentures are more satisfied compared to subjects with fixed partial dentures and removable partial dentures. PMID:26759802

  18. Management of the alveolar cleft.

    Science.gov (United States)

    Santiago, Pedro E; Schuster, Lindsay A; Levy-Bercowski, Daniel

    2014-04-01

    Orthopedic and orthodontic management of patients born with clefts of the lip, alveolus and palate is based on the application of basic biomechanical principles adapted to the individualized cleft anatomy. This article focuses on orthopedic and orthodontic preparation for 2 stages of interdisciplinary orthodontic/surgical cleft care: presurgical infant orthopedics (nasoalveolar molding) for lip/alveolus/nasal surgical repair and maxillary arch preparation for secondary alveolar bone grafting. These preparatory stages of orthopedic/orthodontic therapy are undertaken with the goal of restoring normal anatomic relationships to assist the surgeon in providing the best possible surgical care. PMID:24607190

  19. Controversies in the Management of Patients with Cleft Lip and Palate.

    Science.gov (United States)

    Rodman, Regina E; Tatum, Sherard

    2016-08-01

    Cleft lip and palate is one of the most common congenital anomalies. For many years, surgeons have been attempting to reduce the severity of the deformity before the surgical repair to achieve a better outcome. The nasoalveolar molding technique uses acrylic nasal stents attached to the vestibular shield of an oral molding plate to mold the nasal alar cartilages into a more normal form and position during the presurgical period. Proponents of nasoalveolar molding claim several benefits, including improved aesthetic outcome, reduced overall costs, and a psychosocial benefit to the family. Research on these outcomes is not conclusive. PMID:27400840

  20. EEC syndrome without ectrodactyly: report of two new families.

    OpenAIRE

    Fryns, J P; Legius, E.; Dereymaeker, A M; van den Berghe, H

    1990-01-01

    In this report we describe two families with variable manifestations of the EEC syndrome. The findings in these families confirm that no symptom is obligatory for the diagnosis of EEC syndrome. In the absence of cleft lip/palate, EEC patients have a characteristic facial morphology with maxillary hypoplasia, short philtrum, and broad nasal tip.

  1. Susceptibility to DNA damage as a molecular mechanism for non-syndromic cleft lip and palate.

    Directory of Open Access Journals (Sweden)

    Gerson Shigeru Kobayashi

    Full Text Available Non-syndromic cleft lip/palate (NSCL/P is a complex, frequent congenital malformation, determined by the interplay between genetic and environmental factors during embryonic development. Previous findings have appointed an aetiological overlap between NSCL/P and cancer, and alterations in similar biological pathways may underpin both conditions. Here, using a combination of transcriptomic profiling and functional approaches, we report that NSCL/P dental pulp stem cells exhibit dysregulation of a co-expressed gene network mainly associated with DNA double-strand break repair and cell cycle control (p = 2.88×10(-2-5.02×10(-9. This network included important genes for these cellular processes, such as BRCA1, RAD51, and MSH2, which are predicted to be regulated by transcription factor E2F1. Functional assays support these findings, revealing that NSCL/P cells accumulate DNA double-strand breaks upon exposure to H2O2. Furthermore, we show that E2f1, Brca1 and Rad51 are co-expressed in the developing embryonic orofacial primordia, and may act as a molecular hub playing a role in lip and palate morphogenesis. In conclusion, we show for the first time that cellular defences against DNA damage may take part in determining the susceptibility to NSCL/P. These results are in accordance with the hypothesis of aetiological overlap between this malformation and cancer, and suggest a new pathogenic mechanism for the disease.

  2. Epiphysical clefts

    International Nuclear Information System (INIS)

    Defects or clefts may be seen in the growing epiphysis and are usually observed just before puberty. The basal epiphysis of the proximal phalanx of the great toe is the most common site but similar defects have been observed in a numer of other epiphyseso At least some of these defects develop within a single normal appearing epiphysis and are not associated with signs or symptoms suggestive of fracture. The mechanism of formation of these defects is not clear. The defects probably close spontaneously some-time around late puberty. (orig.)

  3. Genetics Home Reference: X-linked intellectual disability, Siderius type

    Science.gov (United States)

    ... Cleft Lip / Cleft Palate Bottle Feeding Cleveland Clinic: Cleft Lip & Palate Surgery Genetic Testing Registry: Siderius X-linked mental ... PJ, Schofield CJ. PHF8, a gene associated with cleft lip/palate and mental retardation, encodes for an Nepsilon-dimethyl ...

  4. Secondary Alveolar Bone Grafting and Iliac Cancellous Bone Harvesting for Patients With Alveolar Cleft.

    Science.gov (United States)

    Pan, Weiyi; Wu, Chenzhou; Yang, Zheng; Duan, Zexi; Su, Zhifei; Wang, Peiqi; Zheng, Qian; Li, Chunjie

    2016-06-01

    To assess the efficacy of present interventions optimizing the result of secondary alveolar bone grafting (SABG) and the interventions alleviating the donor site morbidity after iliac cancellous bone harvesting. Researches were identified by searching the electronic database of MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Chinese BioMedical Literature Database, and the China National Knowledge Infrastructure. In addition, relevant journals and references of the included studies were searched manually. The Oxford 2011 Levels of Evidence were applied to assess the methodological quality of selected studies, and the best evidence synthesis system was applied afterward to measure the strength of evidence. As a result, 42 studies were considered eligible and included, among which 4 were of high quality while 38 were of low quality. Thirty lines of evidences were acquired after the synthesis, among which 13 were rated as moderate while 17 were rated as insufficient. As for the interventions optimizing the result of SABG, moderate evidence confirmed the efficacy of preoperative orthodontic treatment, the superiority of performing SABG before the eruption of canine, and the accuracy of cone beam computed tomography in preoperative estimation of the cleft volume. As for the interventions alleviating the morbidity of iliac cancellous bone harvesting, moderate evidence confirmed the treatment benefit of the interventions below: minimally invasive technique, including trephine and Shepard osteotomy; preemptive analgesia, including continuous bupivacaine infusion or transversus abdominis plane block. As for the rest interventions, only insufficient evidence was found. PMID:27244214

  5. Cleft lip and palate repair

    Science.gov (United States)

    Orofacial cleft; Craniofacial birth defect repair; Cheiloplasty; Cleft rhinoplasty; Palatoplasty; Tip rhinoplasty ... A cleft lip is a birth defect: A cleft lip may be just a small notch in the lip. It may also be a complete split in the ...

  6. A study on the dental anomalities and site of cleft associated with cleft lip and/or palate

    International Nuclear Information System (INIS)

    The purpose of this study is to investigate possible correlation between the dental anomalies and site of cleft in cleft lip and palate. In this study, 142 patients who had cleft lip and/or cleft palate were examined. The results are as follows. 1. The incidence of missing tooth was high in the permanent dentition as compared to the incidence in the deciduous dentition. 2. There was not much difference of incidence of supernumerary tooth between deciduous and permanent dentition in the group of patients who had cleft lip and jaw with or without cleft palate. 3. In the group of patients who had cleft lip and jaw with or without cleft palate, the frequency of incidence of cleft sides was higher in unilateral than bilateral cases. And, incidence of left sides was higher than right sides. 4. The type of cleft between central incisor and canine with missing lateral incisor was most frequent in permanent dentition and the type of cleft between central and lateral incisor was most frequent in deciduous dentition. 5. The type of cleft associated with tooth position in deciduous dentition was not almost the same in the succeeding permanent dentition.

  7. Unusual case of cleft hand

    Directory of Open Access Journals (Sweden)

    Sahasrabudhe Parag

    2007-01-01

    Full Text Available We present a case of a six-year-old male child with cleft hand deformity involving the dominant right hand. It was a rare case of atypical cleft hand with no missing tissue but cleft extending to metacarpal level and associated hypoplasia of thumb and index finger. As per Manske′s classification of cleft hand our patient belongs to the Class III variety. There was associated malposition of the index finger with absence of first web space and syndactly of thumb and index finger at the metacarpal level. A modified Snow-Littler procedure was planned. The surgical plan involved closure of cleft, release of thumb and index finger syndactly and reconstruction of the first web space. The functional outcome was good considering hypoplasia of the index finger and thumb. Depending upon the function of the thumb tendon transfers can be planned to augment thumb function at a later date along with correction of rotational deformities of the index and middle finger.

  8. Cleft Palate Foundation

    Science.gov (United States)

    ... available in English , Spanish , and Mandarin ! Information on Cleft Lip and Palate Our booklets and factsheets address a variety of issues related to cleft lip and palate, such as speech, hearing, genetics, and what to ...

  9. Prejuízo no crescimento de crianças com diferentes tipos de fissura lábio-palatina nos 2 primeiros anos de idade: um estudo transversal Growth impairment of children with different types of lip and palate clefts in the first 2 years of life: a cross-sectional study

    Directory of Open Access Journals (Sweden)

    Luiz C. Montagnoli

    2005-12-01

    treated at the Craniofacial Anomaly Rehabilitation Hospital, (University of São Paulo, Bauru, SP, Brazil. Age ranged from 1 to 24 months. Three types of clefts were evaluated: isolated cleft lip (181/20.5%, isolated cleft palate (157/17.8% and cleft lip + palate (543/61.6%. Weight and length measurements and data regarding breast-feeding and socioeconomic level were obtained. Children with weight and length below the 10th percentile of the NCHS reference were considered to have impaired growth. RESULTS: Sample distribution according to cleft type and sex was similar to that observed in other epidemiological studies. Breast-feeding was more frequent in the isolated cleft lip group (45.9% then in the isolated cleft palate (12.1% or cleft lip + palate group (10.5%. Isolated cleft lip children showed less marked impairment of weight (23.8% and length (19.3% compared to the cleft lip + palate group (35.7% and 33.1%, respectively. In the latter group, the proportion of children with weight and length below the 10th percentile was very close to that of the isolated cleft palate group (34.4% and 38.9%. CONCLUSIONS: The impairment in weight and length was more severe in cleft lip + palate and isolated cleft palate children and may be attributed to feeding difficulties compared to the isolated cleft lip group.

  10. Cleft palate in Williams syndrome

    Science.gov (United States)

    Domenico, Scopelliti; Orlando, Cipriani; Graziana, Fatone Flavia Maria; Papi, Piero; Giulia, Amodeo

    2013-01-01

    Williams–Beuren syndrome (WBS) is a genomic neurodevelopmental disorder, estimated to occur in approximately 1 in 10,000 persons. It is caused by a deletion of the “elastin” gene on chromosome 7q11.23 and was described officially in 1961 by Williams, Barrat-Boyes, and Lowe. Cleft palate is not considered in the medical literature as a part of the multisystem disorders of the Williams syndrome but it was yet described. We present our experience of a patient who presents cleft palate among other congenital malformations. PMID:23662266

  11. Cleft palate in Williams syndrome

    OpenAIRE

    Domenico, Scopelliti; Orlando, Cipriani; Graziana, Fatone Flavia Maria; Papi, Piero; Giulia, Amodeo

    2013-01-01

    Williams–Beuren syndrome (WBS) is a genomic neurodevelopmental disorder, estimated to occur in approximately 1 in 10,000 persons. It is caused by a deletion of the “elastin” gene on chromosome 7q11.23 and was described officially in 1961 by Williams, Barrat-Boyes, and Lowe. Cleft palate is not considered in the medical literature as a part of the multisystem disorders of the Williams syndrome but it was yet described. We present our experience of a patient who presents cleft palate among othe...

  12. Disease: H00752 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00752 Ankyloblepharon-ctodermal defects-cleft lip/palate (AEC) syndrome and Rapp-H...odgkin syndrome Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome (also known as Hay-Wells ...syndrome) and Rapp-Hodgkin syndrome are rare ectodermal dysplasias characterized by ankyloblepharon filiforme adnatum and cleft

  13. RELATIVE FREQUENCY OF CONGENITAL HEART DISEASES IN BABIES WITH CLEFT LIP AND/OR PALATE

    OpenAIRE

    Ravikumar; Mohammed Manekkat Thekke; Tradib Arekanadth

    2016-01-01

    Orofacial clefts are among the most common congenital anomalies. A significant number of patients with cleft palate have other associated malformations that may result in cardiac, limb or other system defects. Establishing a significant relationship of associated anomalies in cleft lip and cleft palate is important for several reasons. Firstly, this will improve screening and evaluation methods in departments dealing with cleft lip and palate and other craniofacial anomalies becau...

  14. International Task Force on Volunteer Cleft Missions.

    Science.gov (United States)

    Yeow, Vincent K L; Lee, Seng-Teik T; Lambrecht, Thomas J; Barnett, John; Gorney, Mark; Hardjowasito, Widanto; Lemperle, Gottfried; McComb, Harold; Natsume, Nagato; Stranc, Mirek; Wilson, Libby

    2002-01-01

    The International Task Force on Volunteer Cleft Missions was set up to provide a report to be presented at the Eighth International Congress of Cleft Palate and Associated Craniofacial Anomalies on September 12, 1997, in Singapore. The aim of the report was to provide data from a wide range of different international teams performing volunteer cleft missions and, thereafter, based on the collected data, to identify common goals and aims of such missions. Thirteen different groups actively participating in volunteer cleft missions worldwide were selected from the International Confederation of Plastic and Reconstructive Surgery's list of teams actively participating in volunteer cleft missions. Because of the time frame within which the committee had to work, three groups that did not respond by the stipulated deadline were omitted from the committee. The represented members and their respective institutions have undertaken more than 50 volunteer cleft missions to underdeveloped nations worldwide within the last 3 years. They have visited over 20 different countries, treating more than 3,500 patients worldwide. Based on the data collected and by consensus, the committee outlined recommendations for future volunteer cleft missions based on 1) mission objectives, 2) organization, 3) personal health and liability, 4) funding, 5) trainees in volunteer cleft missions, and 6) public relations. The task force believed that all volunteer cleft missions should have well-defined objectives, preferably with long-term plans. The task force also decided that it was impossible to achieve a successful mission without good organization and close coordination. All efforts should be made, and care taken, to ensure that there is minimal morbidity and no mortality. Finally, as ambassadors of goodwill and humanitarian aid, the participants must make every effort to understand and respect local customs and protocol. The main aims are to provide top-quality surgical service, train local

  15. A Retrospective Study of Cleft lip and palate Patients´ Satisfaction after Maxillary Distraction or Traditional Advancement of the Maxilla

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    Kristian Andersen

    2012-06-01

    Full Text Available Objectives: To compare cleft lip and palate patients’ satisfaction with aesthetics and functional parameters after conventional advancement of the maxilla or by the use of distraction osteogenesis.Material and methods: Case series observational study. Group of distraction osteogenesis (DO consisted of 15 patients treated with distraction osteogenesis while group conventional (CONV included 10 patients treated with traditional advancement of the maxilla. Patients were asked to fill out a questionnaire about their subjective evaluation of satisfaction with facial aesthetics and functional parameters on a continuous visual analog-scale (VAS when the treatment was finished.Results: The total response rate was 76%. Preoperatively the two groups did not differ significantly according to group characteristics. At follow-up both groups were satisfied with aesthetics and functional parameters. The DO group was less satisfied with the duration of the treatment than the CONV group. There were no statistically significant differences among the groups regarding functional parameters or facial aesthetics.Conclusions: Cleft lip and palate patients experienced a high level of satisfaction with functional parameters and aesthetics as a result of surgical maxillary advancement. The patients treated with distraction osteogenesis were less satisfied with the duration of the treatment. Further studies are needed.

  16. Cleft Palate; A Multidiscipline Approach.

    Science.gov (United States)

    Stark, Richard B., Ed.

    Nineteen articles present a multidisciplinary approach to the management of facial clefts. The following subjects are discussed: the history of cleft lip and cleft palate surgery; cogenital defects; classification; the operation of a cleft palate clinic; physical examination of newborns with cleft lip and/or palate; nursing care; anesthesia;…

  17. Facts about Cleft Lip and Cleft Palate

    Science.gov (United States)

    ... children with orofacial clefts may have issues with self-esteem if they are concerned with visible differences between themselves and other children. Parent-to-parent support groups can prove to be ...

  18. Dental anomalies inside the cleft region in individuals with nonsyndromic cleft lip with or without cleft palate

    Science.gov (United States)

    Sá, Jamile; Araújo, Luana; Guimarães, Laís; Maranhão, Samário; Lopes, Gabriela; Medrado, Alena; Coletta, Ricardo

    2016-01-01

    Background Individuals with non syndromic cleft lip with or without cleft palate (NSCL±P) present high frequency of dental anomalies, which may represent complicating factors for dental treatment. The aim of this study was to investigate the prevalence of dental anomalies inside cleft area in a group of Brazilians with NSCL±P. Material and Methods Retrospective analysis of 178 panoramic radiographs of patients aged from 12 to 45 years old and without history of tooth extraction or orthodontic treatment was performed. Association between cleft type and the prevalence of dental anomalies was assessed by chi-square test with a significance level set at p≤ 0.05. Results Dental anomalies were found in 88.2% (n=157) of the patients. Tooth agenesis (47.1%), giroversion (20%) and microdontia (15.5%) were the most common anomalies. Individuals with unilateral complete cleft lip and palate (CLP, p<0.0001), bilateral complete CLP (p=0.0002) and bilateral incomplete CLP (p< 0.0001) were more affected by tooth agenesis than individuals with other cleft types. The maxillary lateral incisors were the most affected teeth (p<0.0001). Conclusions The present study revealed a high frequency of dental anomalies inside cleft region in NSCL±P patients, and further demonstrated that patients with unilateral complete CLP and bilateral incomplete CLP were frequently more affected by dental anomalies. Moreover, our results demonstrate that dental anomalies should be considered during dental treatment planning of individuals affected by NSCL±P. Key words:Nonsyndromic cleft lip with or without palate, dental anomaly, tooth agenesis, microdontia. PMID:26615505

  19. Implementing the Brazilian Database on Orofacial Clefts

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    Isabella Lopes Monlleó

    2013-01-01

    Full Text Available Background. High-quality clinical and genetic descriptions are crucial to improve knowledge of orofacial clefts and support specific healthcare polices. The objective of this study is to discuss the potential and perspectives of the Brazilian Database on Orofacial Clefts. Methods. From 2008 to 2010, clinical and familial information on 370 subjects was collected by geneticists in eight different services. Data was centrally processed using an international system for case classification and coding. Results. Cleft lip with cleft palate amounted to 198 (53.5%, cleft palate to 99 (26.8%, and cleft lip to 73 (19.7% cases. Parental consanguinity was present in 5.7% and familial history of cleft was present in 26.3% subjects. Rate of associated major plus minor defects was 48% and syndromic cases amounted to 25% of the samples. Conclusions. Overall results corroborate the literature. Adopted tools are user friendly and could be incorporated into routine patient care. The BDOC exemplifies a network for clinical and genetic research. The data may be useful to develop and improve personalized treatment, family planning, and healthcare policies. This experience should be of interest for geneticists, laboratory-based researchers, and clinicians entrusted with OC worldwide.

  20. Concomitant pituitary adenoma and Rathke's cleft cyst

    International Nuclear Information System (INIS)

    We reviewed the clinical, radiological and surgical findings in patients with both pituitary adenoma and Rathke's cleft cyst. We retrospectively selected patients with both lesions from the 374 patients in whom a sellar/juxtasellar lesion was detected on MRI at 1.5 tesla. All patients received intravenous contrast medium. Concomitant pituitary adenoma and Rathke's cleft cyst were found in eight patients (2.1 %). The frequency of the combination was 3.5 % of pituitary adenomas and 11 % of Rathke's cleft cysts. Symptoms were always due to the adenoma, secreting adrenocorticotrophin in two patients and growth hormone in six. The adenoma was larger in five patients, and the cyst in three. The cysts gave variable signal. The adenoma was adjacent to the cyst in seven patients, and enclosed it in the other patient. As a result of experience with MRI, concomitant pituitary adenoma and Rathke's cleft cyst are now known not to be as rare as thought previously. When a nonenhancing cyst-like structure is demonstrated in a patient with pituitary adenoma, the possibility of a coexisting Rathke's cleft cyst should be considered. (orig.)

  1. Congenital Cleft Hand

    OpenAIRE

    Aritamur, Ayhan; Cakmak, Mehmet; Taser, Omer; Berk, Hasan

    2004-01-01

    Congenital cleft hand deformity, which is also known with such names as cleft hand, lobster claw hand and Ectrodactyiy, is characterized by the absence of one or two fingers in the mid portion of the hand. A case of bilateral cleft hand deformity four years old, which is considerabiy rare, was reconstructed surgically. The result obtained has been presented. Because no sufficient experience has accumulated due to the fact that it is observed considerably rare, the therapeutical principales re...

  2. Cleft lip and palate: recommendations for dental anesthetic procedure based on anatomic evidences

    Directory of Open Access Journals (Sweden)

    Ivy Kiemle Trindade-Suedam

    2012-02-01

    Full Text Available Patients with cleft lip and palate usually present dental anomalies of number, shape, structure and position in the cleft area and the general dentist is frequently asked to restore or extract those teeth. Considering that several anatomic variations are expected in teeth adjacent to cleft areas and that knowledge of these variations by general dentists is required for optimal treatment, the objectives of this paper are: 1 to describe changes in the innervation pattern of anterior teeth and soft tissue caused by the presence of a cleft, 2 to describe a local anesthetic procedure in unilateral and bilateral clefts, and 3 to provide recommendations to improve anesthetic procedures in patients with cleft lip and palate. The cases of 2 patients are presented: one with complete unilateral cleft lip and palate, and the other with complete bilateral cleft lip and palate. The patients underwent local anesthesia in the cleft area in order to extract teeth with poor bone support. The modified anesthetic procedure, respecting the altered course of nerves in the cleft maxilla and soft tissue alterations at the cleft site, was accomplished successfully and the tooth extraction was performed with no pain to the patients. General dentists should be aware of the anatomic variations in nerve courses in the cleft area to offer high quality treatment to patients with cleft lip and palate.

  3. Congenital heart defects in children with oral clefts

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    Nahvi H.

    2007-09-01

    Full Text Available   Background: Oral clefts are among the most common congenital anomalies. Infants with oral clefts often have other associated congenital defects, especially congenital heart defects. The reported incidences and the types of associated malformations and congenital heart defects vary between different studies. The purpose of this study was to assess the incidence of associated congenital heart defects in children with oral clefts. Methods: All infants with cleft lip and palate referred to the Children's Medical Center and Bahramy; the teaching Hospitals of the Tehran University of Medical Sciences from 1991 to 2005 were prospectively enrolled in this study group. All patients were examined and noted by an academic cleft team contain; a pediatrician and a pediatric surgeon, and received cardiac consultation and echocardiography by a pediatric cardiologist. non cardiac associated anomalies, still born and patients without echocardiography were excluded from the study.  Data including age, gender, exposure to contagions and high risk elements ,consanguinity and familial history of oral cleft, type of oral cleft, results of cardiac consultation and echocardiography and associated cardiac anomalies were cumulated and analyzed by SSPS version 13.5Results: Among the 284 infants with oral clefts, 162 were male (57% and 122 were female (43%. Seventy-nine patients (27.8% had cleft lip, 84 (29.5% had cleft palate and 121 (42.6% had both cleft lip and palate. Of all the patients, 21.1% had congenital heart defects. the most common type Of these congenital heart defects(28.3%  was atrial septal defect.Conclusions: For patients with cleft lip and palate, we recommend preoperative cardiac consultation, careful examination and routine echocardiography for associated cardiac anomalies, as well as appropriate management and prophylactic antibiotic therapy for those with associated congenital heart anomaly.

  4. Comparative study of three techniques of palatoplasty in patients with cleft of lip and palate via instrumental and auditory-perceptive evaluations

    Directory of Open Access Journals (Sweden)

    Paniagua, Lauren Medeiros

    2010-03-01

    Full Text Available Introduction: Palatoplasty is a surgical procedure that aims at the reconstruction of the soft and/or hard palate. Actually, we dispose of different techniques that look for the bigger stretching of the soft palate joint to the nasofaryngeal wall to contribute in the appropriate operation of the velopharyngeal sphincter. Failure in its closing brings on speech dysfunctions. Objective: To compare the auditory-perceptive' evaluations and instrumental findings in patients with cleft lip and palate operate through three distinctive techniques of palatoplasty. Method: A prospective transversal study of a group of patients with complete unilateral cleft lip and palate. Everybody was subjected to a randomized clinical essay, through distinctive techniques of palatoplasty performed for a single surgeon, about 8 years. In the period of the surgery, the patients were divided in three distinctive groups with 10 participants each one. The present study has evaluates: 10 patients of the Furlow technique, 7 patients of the Veau-Wardill-Kilner+Braithwaite technique and, 9 patients of the Veau-Wardill-Kilner+Braithwaite+Zetaplasty technique; having a total sample of 26 individuals. All the patients were subjected to auditory-perceptive evaluation through speech recording. An instrumental evaluation was also performed through video endoscopy exam. Results: The findings were satisfactory in the three techniques, in other words, the majority of the individuals does not present hyper nasality, compensatory articulatory disturbance and audible nasal air emission. In addition, in the instrumental evaluation, the majority of the individuals of the three techniques of palatoplasty present an appropriate velopharyngeal function. Conclusion: Was not found statistically significant difference between the palatoplasty techniques in both evaluations

  5. Is alveolar cleft reconstruction still controversial? (Review of literature)

    OpenAIRE

    Seifeldin, Sameh A.

    2015-01-01

    Cleft lip and palate (CL/P) is a frequent congenital malformation that manifests in several varieties including unilateral or bilateral and complete or incomplete. Alveolar cleft reconstruction remains controversial with regard to timing, graft materials, surgical techniques, and methods of evaluation. Many studies have been conducted addressing these points to develop an acceptable universal protocol for managing CL/P. The primary goal of alveolar cleft reconstruction in CL/P patients is to ...

  6. Correlation of vermilion symmetry to alveolar cleft defect in unilateral cleft lip repair.

    Science.gov (United States)

    Bonanthaya, K; Rao, D D; Shetty, P; Uguru, C

    2016-06-01

    Asymmetry is a major problem in repaired unilateral cleft lip (UCL). One of the important manifestations of this is the asymmetry of the vermilion. The aim of this study was to correlate the severity of the asymmetry in the vermilion to the size of the alveolar defect. Twenty patients aged between 6 and 18 months with complete unilateral cleft lip, alveolus, and palate were included. An impression of each patient's alveolus at the time of cheiloplasty was taken using silicon rubber base material, and a study cast was prepared. The width of the cleft alveolus was measured on these casts using a transparent grid. Frontal photographs were taken at 6 months postoperative and vermilion symmetry was measured as the ratio between the cleft and non-cleft sides. The results obtained in this study showed a direct correlation between the size of the alveolar defect and the vermilion symmetry in repaired UCL. The wider the cleft alveolus and greater the antero-posterior discrepancy, the greater is the vermilion asymmetry. The asymmetry of the vermilion in UCL after repair is directly dependent on the size of the alveolar defect. The alveolar discrepancy causes 'in-rolling' of the vermilion on the cleft side and affects the vermilion symmetry. PMID:26754270

  7. Diagnosis and presurgical orthopedics in infants with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Fundagul Bilgic

    2015-01-01

    Full Text Available Cleft lip and palate are one of the most common congenital craniofacial malformations. While preoperative treatment for infants with cleft lip and palate is still a scientific debate, patients with this malocclusion usually have to be treated from infancy to adulthood. Orthodontist plays an important role in the treatment of patients with cleft lip and palate. The purpose of this review is to give information about cleft lip and palate and presurgical nasoalveolar molding.

  8. Diagnosis and presurgical orthopedics in infants with cleft lip and palate

    OpenAIRE

    Fundagul Bilgic; Ozlem Akinci Sozer

    2015-01-01

    Cleft lip and palate are one of the most common congenital craniofacial malformations. While preoperative treatment for infants with cleft lip and palate is still a scientific debate, patients with this malocclusion usually have to be treated from infancy to adulthood. Orthodontist plays an important role in the treatment of patients with cleft lip and palate. The purpose of this review is to give information about cleft lip and palate and presurgical nasoalveolar molding.

  9. Surgical outcome and complications following cleft lip and palate repair in a teaching hospital in Nigeria

    OpenAIRE

    Taiwo O Abdurrazaq; Adeyemi O Micheal; Adeyemo W Lanre; Ogunlewe M Olugbenga; Ladeinde L Akin

    2013-01-01

    Background: Measurement of treatment outcome is important in estimating the success of cleft management. The aim of this study was to assess the surgical outcome of cleft lip and palate surgery. Patients and Methods: The surgical outcome of 131 consecutive patients with cleft lip and palate surgeries between October 2008 and December 2010 were prospectively evaluated at least 4 weeks postoperatively. Data collected included information about the age, sex, type of cleft defects, and type of su...

  10. Cleft lip and palate incidence among the live births in the Republic of Korea.

    OpenAIRE

    Kim, Sukwha; Kim, Woo Jung; Oh, Changhyun; Kim, Jae Chan

    2002-01-01

    We present an epidemiologic study of cleft lip and palate in the Republic of Korea from January 1, 1993 through December 31, 1993. In 1993, the number of total live births was 715,817. And from 1993 through 1995, a total of 1,293 new patients with cleft lip and palate who were born in 1993 were identified. The incidence of cleft lip and palate was 1.81 per 1000, that is, 1 per 554 live births. The cleft lip: cleft lip and palate: cleft palate alone ratio was 1.13:1:1.19. The male: female rati...

  11. Molecular contribution to cleft palate production in cleft lip mice

    OpenAIRE

    Sasaki, Yasunori; Taya, Yuji; Saito, Kan; Fujita, Kazuya; Aoba, Takaaki; Fujiwara, Taku

    2014-01-01

    Cleft palate following cleft lip may include a developmental disorder during palatogenesis. CL/Fr mice fetuses, which develop cleft lip and palate spontaneously, have less capability for in vivo cell proliferation in palatal mesenchyme compared with CL/Fr normal fetuses. In order to know the changes of signaling molecules contributing to cleft palate morphogenesis following cleft lip, the mRNA expression profiles were compared in palatal shelves oriented vertically (before elevation) in CL/Fr...

  12. Cleft palate lateral synechia syndrome

    OpenAIRE

    Sybil, Deborah; Sagtani, Alok

    2013-01-01

    Cleft lip and palate are the most common congenital craniofacial anomaly in humans. The presence of oral synechia along with cleft palate is a rare syndrome. We encountered one case that had a cleft palate accompanied by congenital oral synechia due to a membranous adhesion between the floor of the mouth and the free margin of the cleft palate.

  13. Estudio morfológico en tres planos del meato inferior en las fisuras unilaterales operadas Morphological study of the inferior meatus in three planes in patients with operated unilateral clefts

    Directory of Open Access Journals (Sweden)

    Vicente Contreras

    2011-09-01

    Full Text Available Objetivo: Comparar el área disponible en tres planos del meato inferior en pacientes operados de fisura labio-máxilo-palatina unilateral, con respecto a la de los pacientes no fisurados. Material y método: Correspondió a un estudio de corte transversal. La muestra consistió en un grupo estudio de 29 pacientes operados de fisura labio-máxilo-palatina unilateral entre 5 y 17 años, controlados en el Hospital San Borja Arriarán y Facultad de Odontología de la Universidad de Chile, y un grupo control de 29 pacientes no fisurados, pareados por género y edad con el grupo estudio. El área disponible del meato inferior fue definida como el espacio anatómicamente disponible en cada meato inferior para el flujo aéreo. Esta área fue medida en cada fosa nasal a distintas profundidades en el eje antero-posterior mediante tomografía computarizada de haz cónico, y sometida a un análisis estadístico de modelos mixtos para datos anidados. Resultados: Los valores del promedio del área disponible del meato inferior en el grupo estudio fueron significativamente menores que en el grupo control, en cada profundidad estudiada (pObjective: To compare the available inferior meatus area in three planes between operated unilateral cleft lip and palate patients and non-cleft patients. Materials and methods: Cross-sectional study design. The study group consisted of 29 complete unilateral cleft patients between 5 and 17 years-old, attending San Borja Arriarán Hospital and the University of Chile's School of Dentistry. Twenty nine non-cleft patients on orthodontic treatment served as controls. The groups were matched by age and sex. The available inferior meatus area was defined as the anatomically available area in each meatus for airflow. This area was measured in each nasal fossa at different depths across the anteroposterior axis by cone-beam computed tomography. A mixed model regression for nested data was performed for data analysis. Results: The

  14. Transverse facial cleft: A series of 17 cases

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    L K Makhija

    2011-01-01

    Full Text Available Introduction: Transverse facial cleft (Tessier type 7 or congenital macrostomia is a rare congenital anomaly seldom occurring alone and is frequently associated with deformities of the structures developing from the first and second branchial arches. The reported incidence of No. 7 cleft varies from 1 in 60,000 to 1 in 300,000 live births. Material and Methods: Seventeen patients of transeverse facial cleft who presented to us in last 5 years were included in the study. Their history regarding familial and environmental predispositions was recorded. The cases were analysed on basis of sex, laterality, severity, associated anomalies and were graded according to severity. They were operated by z plasty technique and were followed up for 2 years to look for effectiveness of the technique and its complications. Result: Out of the seventeen patients of transverse cleft, none had familial predilection or any environmental etiology like antenatal radiological exposure or intake of drugs of teratogenic potential. Most of the patients (9/17 were associated with hemifacial microsomia and 1 patient was associated with Treacher Colin′s Syndrome. Out of the 6 cases of Grade I clefts, 4 were isolated transverse clefts and of the 10 patients of Grade II clefts, 7 were associated with hemifacial microsomia. We encountered only one case of Grade III Transverse Cleft which was not only associated with hemifacial microsomia but also had cardiac anomaly. Out of the17 cases, 15 were operated and in most of them the outcome was satisfactory.

  15. Median cleft lip: A new method of surgical repair

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    Khandekar B

    2010-01-01

    Full Text Available The aim is to discuss a new method of muscle repair in midline cleft lip. Three patients with midline cleft lip were repaired with our technique of muscle repair and the results evaluated. Our new method of muscle repair in the form of ′Z′ helps in forming the philtral dimple.

  16. Molecular contribution to cleft palate production in cleft lip mice

    Science.gov (United States)

    Sasaki, Yasunori; Taya, Yuji; Saito, Kan; Fujita, Kazuya; Aoba, Takaaki; Fujiwara, Taku

    2014-01-01

    Cleft palate following cleft lip may include a developmental disorder during palatogenesis. CL/Fr mice fetuses, which develop cleft lip and palate spontaneously, have less capability for in vivo cell proliferation in palatal mesenchyme compared with CL/Fr normal fetuses. In order to know the changes of signaling molecules contributing to cleft palate morphogenesis following cleft lip, the mRNA expression profiles were compared in palatal shelves oriented vertically (before elevation) in CL/Fr fetuses with or without cleft lip. The changes in mRNA profile of cleft palate morphogenesis were presented in a microarray analysis, and genes were restricted to lists contributing to cleft palate development in CL/Fr fetuses with cleft lip. Four candidate genes (Ywhab, Nek2, Tacc1 and Frk) were linked in a gene network that associates with cell proliferation (cell cycle, MAPK, Wnt and Tgf beta pathways). Quantitative real-time RT-PCR highlighted the candidate genes that significantly changed in CL/Fr fetuses with cleft lip (Ywhab, Nek2 and Tacc1). The results of these molecular contributions will provide useful information for a better understanding of palatogenesis in cleft palate following cleft lip. Our data indicated the genetic contribution to cleft palate morphogenesis following cleft lip. PMID:24206222

  17. Surgical repair of the isolated incomplete median cleft lip.

    Science.gov (United States)

    Topkara, A; Özkan, A; Özcan, R H; Öksüz, M

    2016-02-01

    Median cleft lip refers to a vertical cleft on the midline of the upper lip. It is a rare congenital craniofacial anomaly brought about by a fusion failure in the medial nasal prominence. A novel surgical approach to median cleft lips and their repair is reported herein, with reference to a clinical case. The patient had a cleft in the lower half of the upper lip. There were no other craniofacial anomalies in this patient other than the cleft. Within the framework of the patient's surgical treatment, a functional and cosmetically satisfactory result was achieved by performing a V-Y advancement flap on the columella base, Z-plasty in the vermillion zone, and Z pattern muscular tissue repair, without having to resort to any tissue excisions. PMID:26364580

  18. Patau sendromlu üç olgu: Sendroma klinik triad (mikroftalmi, yarık dudak/ damak, polidaktili) her zaman eşlik eder mi?

    OpenAIRE

    Hazan, F; Olukman, Ö; Gökaslan, F; Çalkavur, Ş; Meşe, T.; Tavlı, V; Atlıhan, F

    2013-01-01

    Trisomy 13 (Patau's syndrome) is a chromosomal disease characterized by midline defects, central nervous system, heart, urogenital system anomalies and motor mental retardation. The prevalence is between 1:10.000 and 1:20.000 births. A vast majority of the patients die in the first year. Patau's syndrome is characterized by the clinical triad including microphtalmia, cleft lip/palate, and polydactyly. Here, we present three patients who were diagnosed with Patau's syndrome both clinically and...

  19. Role of SOX9 in the Etiology of Pierre-Robin Syndrome

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    Selvi R

    2013-05-01

    Full Text Available Objective(s:Cleft lip/palate are common congenital anomalies, affecting approximately 2/1000 live births. Pierre Robin Sequence is a subgroup of the cleft palate population. Chromosomal abnormalities near the SOX9 gene disrupt the regulation of this gene and prevent the SOX9 protein from properly controlling the development of facial structures, which leads to isolated PRS. The present study was conducted to identify the role of the SOX9 gene in the etiology of Pierre robin syndrome and to study the association of SOX9 and PRS in regulating morphogenesis of the face in individuals with Cleft lip/Palate using the PCR technique and GTG banding. Materials and Methods:Molecular and cytogenetic analysis was performed in 27 subjects with cleft lip/palate and 13 age matched controls. DNA was isolated and PCR was performed for the amplification of the gene of interest and the products were run on a 2% Agarose gel and the band patterns were analyzed.The chromosomal abnormalities were analyzed from the cultured lymphocytes after GTG banding. Result:Out of 27 patients screened, deletion of the SOX9 gene was observed in 1 case for exon1 and in 2 cases for exon2. The cytogenetic analysis showed no structural or numerical abnormalities and all the patients showed normal karyotype. Conclusion: The results of molecular methods showed a positive association suggesting that the SOX9 gene is of particular importance, but the cytogenetic study didn’t seem to show a stronger association suggesting that, this method would not identify disease genes acting via other mechanisms of genetic dominance and also due to the fact that Cleft lip / palate has a multifactorial inheritance.

  20. Laryngo-tracheo-oesophageal clefts

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    Leboulanger Nicolas

    2011-12-01

    Full Text Available Abstract A laryngo-tracheo-esophageal cleft (LC is a congenital malformation characterized by an abnormal, posterior, sagittal communication between the larynx and the pharynx, possibly extending downward between the trachea and the esophagus. The estimated annual incidence of LC is 1/10,000 to 1/20,000 live births, accounting for 0.2% to 1.5% of congenital malformations of the larynx. These incidence rates may however be underestimated due to difficulty in diagnosing minor forms and a high mortality rate in severe forms. A slightly higher incidence has been reported in boys than in girls. No specific geographic distribution has been found. Depending on the severity of the malformation, patients may present with stridor, hoarse cry, swallowing difficulties, aspirations, cough, dyspnea and cyanosis through to early respiratory distress. Five types of laryngo-tracheo-esophageal cleft have been described based on the downward extension of the cleft, which typically correlates with the severity of symptoms: Type 0 laryngo-tracheo-esophageal cleft to Type 4 laryngo-tracheo-esophageal cleft. LC is often associated with other congenital abnormalities/anomalies (16% to 68%, mainly involving the gastro-intestinal tract, which include laryngomalacia, tracheo-bronchial dyskinesia, tracheo-bronchomalacia (mostly in types 3 and 4, and gastro-esophageal reflux disease (GERD. The syndromes most frequently associated with an LC are Opitz/BBB syndrome, Pallister Hall syndrome, VACTERL/VATER association, and CHARGE syndrome. Laryngeal clefts result from failure of fusion of the posterior cricoid lamina and abnormal development of the tracheo-esophageal septum. The causes of the embryological developmental anomalies leading to LC are not known but are thought to be multifactorial. LC appears to be mostly sporadic although some familial cases with suspected autosomal dominant transmission have been reported. The age of diagnosis depends mainly on the severity of

  1. 桂中地区先天性唇腭裂1021例发病因素分析%Analysis of Risk Factors of 1 021 Patients with Congenital Cleft Lip and Palate in Central Region of Guangxi

    Institute of Scientific and Technical Information of China (English)

    王伯钧; 韦元强; 玉铭; 秦小云; 韦进

    2012-01-01

    Objective To explore the risk factors of congenital cleft lip and palate in central region of Guangxi, and to investigate the preventive methods of congenital cleft lip and palate. Methods A clinical data of 1021 patients with congenital cleft lip and palate in central region of Guangxi were analyzed in the study. Results Of 1 021 patients, there were 652 men and 369 women, the ratio of male to female was 1.77 : 1,609 patients( 59.65 % ) with cleft lip, 299 patients ( 29.29% ) with cleft palate,113 patientsC 11.07% ) with cleft lip and palate. 940 patientsC 92.07% ) came from rural areas,81 patientsC 7.93% ) from towns;The proportion of patients from rural areas was significantly higher than that of patients from the city. The risk factors ranked in the top three as follows: flu occurred in the first 3 months of pregnancy ( 53. 57% ), pregnancy malnutrition( 14. 40% ), medicine intake during pregnancy( 9. 40% ). 954 patients ( 93. 43% ) with congenital cleft lip and palate experienced risk factors in the early stage of pregnancy,46 patients ( 4.50% ) had familial genetic history. Conclusion Virus infection and nutritional factors are the major risk factors of incidence of congenital cleft lip and palate. It should be paid more attention to universal health care knowledge during pregnancy to reduce the incidence of congenital cleft lip and palate.%目的 分析桂中地区先天性唇腭裂的发病因素,探讨先天性唇腭裂的预防方法.方法 对桂中地区先天性唇腭裂1 021例患者的临床资料进行分析.结果 1 021例患者中,男652例,女369例,男女之比为1.77:1;唇裂609例(59.65%),腭裂299例(29.29%),唇腭裂113例(11.07%).患者来自农村 940例(92.07%),城镇81例(7.93%),农村比例明显高于城市.发病因素前3位分别是妊娠前3个月感冒(53.57%)、孕期营养缺乏(14.40%)、孕期服药(9.30%).孕早期经历危险因素954例占93.43%,有家族遗传病史者46例占4.51%.结论 病毒感染及营养因素是先

  2. Genetics of Cleft Lip and Cleft Palate

    OpenAIRE

    Leslie, Elizabeth J.; Marazita, Mary L.

    2013-01-01

    Orofacial clefts are common birth defects and can occur as isolated, nonsyndromic events or as part of Mendelian syndromes. There is substantial phenotypic diversity in individuals with these birth defects and their family members: from subclinical phenotypes to associated syndromic features that is mirrored by the many genes that contribute to the etiology of these disorders. Identification of these genes and loci has been the result of decades of research using multiple genetic approaches. ...

  3. Crecimiento sagital maxilar en fisurados unilaterales operados funcionalmente Sagittal maxillary growth in unilateral cleft lip and palate patients following functional surgery

    Directory of Open Access Journals (Sweden)

    F. Donoso Hofer

    2007-06-01

    Full Text Available Objetivo. Comparar el crecimiento sagital maxilar en pacientes con fisura labio-máxilo-palatina unilateral operados a los 6 meses con criterio funcional con pacientes normales que tengan relación consanguínea directa con los anteriores. Diseño del estudio. Análisis arquitectural y craneofacial de Delaire en telerradiografías de perfil en ambos grupos de pacientes cuyas edades fluctúan actualmente entre los 7 y los 12 años, determinando el crecimiento sagital del maxilar a través de la medida del ángulo del pilar maxilar anterior (C1/F1, sometiendo las medidas al test T de Student con una significación del 99,5%. Resultados.Se determinó el valor real y esperado para el ángulo del pilar maxilar anterior en todos los casos. Al comparar estadísticamente los resultados, no se encontraron diferencias significativas en los valores promedios obtenidos. Conclusiones.El crecimiento sagital maxilar de los pacientes con fisura labio-máxilo-palatina unilateral operados a los 6 meses con criterio funcional no difiere del de aquellos pacientes normales.Objective. To compare the sagittal maxillary growth between unilateral cleft lip and palate patients operated under functional criterion at the age of 6 months and normal patients who were blood-related. Design. Delaire’s Architectural and Structural craniofacial analysis in conventional lateral radiographs of all the patients with an age range of 7-12 years, determining the sagittal maxillary growth by the anterior maxillary pillar angle (C1/F1. These measurements were analyzed using the T-test with a 99.5% significance. Results. The real and expected value of the anterior maxillary pillar angle was determined in all cases. By comparing the results statistically, no significant differences were found in the mean values obtained. Conclusion. Maxillary sagittal growth in unilateral cleft lip and palate patients operated at the age of 6 months under functional criterion, does not differ from the

  4. Acro-cardio-facial syndrome

    OpenAIRE

    Dallapiccola Bruno; Digilio Maria

    2010-01-01

    Abstract Acro-cardio-facial syndrome (ACFS) is a rare genetic disorder characterized by split-hand/split-foot malformation (SHFM), facial anomalies, cleft lip/palate, congenital heart defect (CHD), genital anomalies, and mental retardation. Up to now, 9 patients have been described, and most of the reported cases were not surviving the first days or months of age. The spectrum of defects occurring in ACFS is wide, and both interindividual variability and clinical differences among sibs have b...

  5. Skin symptoms in four ectodermal dysplasia syndromes including two case reports of Rapp-Hodgkin-Syndrome.

    Science.gov (United States)

    Knaudt, Björn; Volz, Thomas; Krug, Markus; Burgdorf, Walter; Röcken, Martin; Berneburg, Mark

    2012-01-01

    The skin, hair and nail changes in four distinct ectodermal dysplasia syndromes are compared and reviewed. These syndromes comprise Christ-Siemens-Touraine syndrome; ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome; ankyloblepharon-ectodermal defects-cleft lip/palate syndrome and Rapp-Hodgkin syndrome. A comprehensive overview of the dermatological signs and symptoms in these syndromes was generated from the database of the Ectodermal Dysplasia Network Germany, the clinical findings in the patients seen in our department and an extensive review of the literature. The findings included abnormalities of skin, sweating, hair and nails. These clinical findings are discussed in relation to the underlying molecular defects known to play a role in these four ectodermal dysplasia syndromes. PMID:22759387

  6. Problems of middle ear and hearing in cleft children

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    Sharma Ramesh

    2009-10-01

    Full Text Available The hearing loss in a cleft patient is a well known complication, but generally gets ignored. These children continue to have recurrent otitis media with effusion that affects the hearing abilities. Unfortunatley the middle ear function may not improve with palatoplasty.Cleft palate teams need to follow up all such children beginning at birth and going into adulthood, decades after a ′successful′ palate repair. These patients should have careful otological and audiological surveillance with appropriate interventions whenever required. The review article discusses the current status of hearing management in patients with cleft palate.

  7. Clinical Features and Management of a Median Cleft Lip

    Science.gov (United States)

    Kim, Do Yeon; Oh, Tae Suk

    2016-01-01

    Background Median cleft lip is a rare anomaly consisting of a midline vertical cleft through the upper lip. It can also involve the premaxillary bone, the nasal septum, and the central nervous system. In our current report, we present the clinical features of 6 patients with a median cleft lip and their surgical management according to the accompanying anomalies. Methods From December 2010 to January 2014, 6 patients with a median cleft lip were reviewed. Five of these cases underwent surgical correction; alveolar bone grafting was performed in a patient with a median alveolar cleft. The surgical technique included inverted-U excision of the upper lip and repair of the orbicularis oris muscle. The mean follow-up period was 20.4 months (range, 7.4–44.0 months). Results The study patients presented various anomalous features. Five patients received surgical correction, 4 with repair of the median cleft lip, and one with iliac bone grafting for median alveolar cleft. A patient with basal sphenoethmoidal meningocele was managed with transoral endoscopic surgery for repair of the meningocele. Successful surgical repair was achieved in all cases with no postoperative complications. Conclusions Relatively mild forms of median cleft lip can be corrected with inverted-U excision with good aesthetic outcomes. In addition, there is a broad spectrum of clinical features and various anomalies, such as nasal deformity, alveolar cleft, and short upper frenulum, which require close evaluation. The timing of the operation should be decided considering the presence of other anomalies that can threaten patient survival. PMID:27218021

  8. The Repair of International Clefts in the Current Surgical Landscape.

    Science.gov (United States)

    Persing, Sarah; Patel, Anup; Clune, James E; Steinbacher, Derek M; Persing, John A

    2015-06-01

    Cleft lip and palate (CLP) constitute a significant global disease burden. There are two general models that exist to deliver cleft care: surgical missions and comprehensive cleft centers (CCC). While surgical missions offer high quality surgical care to patients who would be unlikely to ever receive treatment, they may fail to provide sustainable solutions. The development of CCC is growing in popularity worldwide. CCC are permanent centers that offer a multidisciplinary team approach to the treatment of cleft lip and palate. Operation Smile has adopted the concept of specialized surgical care centers. These centers are shown to be safe, cost-effective, and provide sustainable solutions for cleft care. The authors discuss some of the benefits and drawbacks of the classic mission-based model and highlight why there may be a paradigm shift towards CCC. PMID:26080140

  9. Presurgical nasoalveolar moulding in unilateral cleft lip and palate

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    Mohammed Zuhaib

    2016-01-01

    Full Text Available Context: Presurgical nasoalveolar moulding (PNAM is a non-surgical method of reshaping the cleft lip, alveolus, palate and the nose to minimize the severity of the cleft deformity, before primary cheiloplastyand palatoplasty. In this context, PNAM proves to be an invaluable asset in the management of unilateral cleft lip and palate. Aims: The study was conducted to evaluate the effi cacy of PNAM in the management of unilateral cleft lip and palate with the following objectives: (1 To assess and compare the degree of reduction in the size of cleft palate and alveolus (pre-PNAM and post-PNAM. (2 To evaluate and compare the improvement in columellar length and correction of columellar deviation (pre-PNAM and post-PNAM. (3 To assess the changes in the position of the alar base and the alar cartilages. Settings and Design: Prospective study. Subjects and Methods: A prospective study consisting of, which included 20 patients with complete unilateral cleft lip and palate was conducted. The age at the start of PNAM treatment of the infants ranged from 2 to 44 days of age reporting to our institute between December 2011 and August 2013. All the patients underwent PNAM therapy before primary cheiloplasty at 6 months of age; clinical parameters were assessed pre- and post-therapy using photographs and dental study models of the maxilla. Statistical Analysis Used: Student's t-test for paired comparisons. Results: Results of the study showed a promising reduction in the cleft size before the surgery, signifi cant improvement in nasal symmetry, including the columellar length on the cleft side. Conclusions: PNAM is a valuable adjunct to our surgical armamentarium in dealing with the challenges of primary closure of unilateral cleft lip and palate thereby enhancing the overall surgical outcome. The advantages of this method include the simplicity of the procedure and improving the quality of surgical repair, particularly in obtaining tension free muscle

  10. Analysis of polymorphic TGFB1 codons 10, 25, and 263 in a German patient group with non-syndromic cleft lip, alveolus, and palate compared with healthy adults

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    Gressner Axel M

    2004-06-01

    Full Text Available Abstract Background Clefts of the lip, alveolus, and palate (CLPs rank among the most frequent and significant congenital malformations. Leu10Pro and Arg25Pro polymorphisms in the precursor region and Thr263Ile polymorphism in the prodomain of the transforming growth factor β1 (TGF-β1 gene have proved to be crucial to predisposition of several disorders. Methods In this study, polymorphism analysis was performed by real-time polymerase chain reaction (LightCycler and TGF-β1 levels determined by enzyme-linked immunosorbent assay. Results Only 2/60 Caucasian non-syndromic patients with CLP (3.3% carried the Arg25Pro and another 2/60 patients (3.3% the Thr263Ile genotypes, whereas, in a control group of 60 healthy Caucasian blood donors, these heterozygous genotypes were more frequent 16.7% having Arg25Pro (10/60; p Conclusions The genetic differences in codons 25 and 263 suggest that TGF-β1 could play an important role in occurrence of CLP, however, functional experiments will be required to confirm the mechanisms of disturbed development.

  11. Risk factor for pituitary dysfunction in children and adolescents with Rathke's cleft cysts

    OpenAIRE

    Lim, Han Hyuk; Yang, Sei Won

    2010-01-01

    Purpose This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke's cleft cysts. Methods Forty-four patients with Rathke's cleft cysts younger than 19 years who visited Seoul National University Children's Hospital between January 1995 and September 2009 were enrolled. Rathke's cleft cysts were confirmed histologically through an operation in 15 patients and by brain magnetic resonance imaging (MRI) in 29 patients....

  12. Root development of permanent lateral incisor in cleft lip and palate children: A radiographic study

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    Amarlal Deepti

    2007-01-01

    Full Text Available Objective: The objective of this study was to compare the root development of lateral incisor on the cleft side with the root development of its contralateral tooth in cleft lip and palate children. Setting: Cleft lip and palate wing, Meenakshi Ammal Dental College and Hospital, Chennai, South India. Materials and Methods: A sample of 96 orthopantamograms of patients with unilateral or bilateral cleft lip and/or cleft palate was selected, regardless of sex and race. Main Outcome Measure: Orthopantamograms were analyzed for root development of lateral incisor on the cleft and noncleft side. Associated anomalies like hypodontia, supernumerary teeth, malformed lateral incisors and root development of canine, if present, were recorded. Findings and Conclusions: Root development of permanent lateral incisor was delayed on the cleft side compared to the noncleft side. There was a statistically significant relationship between levels of root development of lateral incisors on the cleft side within the different study groups ( P < 0.05. Incidence of hypodontia increased in proportion to cleft severity. Frequency of missing second premolars, supernumerary teeth and malformed lateral incisors increased in cleft lip and palate patients. Root development of canine showed a slight delay on the cleft side when compared to the canine on the noncleft side.

  13. Clefting and psychosocial adjustment. Influence of facial aesthetics.

    Science.gov (United States)

    Tobiasen, J M; Hiebert, J M

    1993-10-01

    This article briefly reviewed the research literature on the psychosocial correlates of facial clefts and described a program of research to study the relationship between severity of cleft impairment and psychosocial adjustment. In the past 40 years, there has been increasing recognition and research literature on the psychologic implications of facial clefts to patients and their families. Advances in both the knowledge base and the science of the psychologic correlates of facial clefts have been made. Children with clefts are not at greater risk for psychopathology than are individuals without clefts; however, they are at significant risk for social competence problems relating to development of friendships, progress in school, and participation in organizations. Problems with social competence have a negative effect on development. The ability of all children to make friends and to be liked by others is considered by most parents, teachers, and child development specialists to be a major developmental milestone. Not having friends and social withdrawal can cause parents or teachers to refer noncleft children to mental health professionals and is a predictor of impaired adult social competence and mental health. Studies of adults with clefts are consistent with studies of adults without clefts. Adults with repaired clefts are less likely to marry than are their noncleft siblings, and they have more problems with social withdrawal. Because facial attractiveness is well-known to affect peer acceptance, we hypothesized that the severity of the cleft deformity may have a significant impact on social competence. Consequently, we undertook a program of research to examine this question.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8275628

  14. Alimentação da criança com fissura de lábio e/ou palato: um estudo bibliográfico Alimentación del niño com fisura del labio y/o paladar: um estudio bibliográfico Alimentation of children with lip-palate malformation: a bibliographic study

    Directory of Open Access Journals (Sweden)

    Raimunda da Costa Araruna

    2000-04-01

    ón.The present study aimed at describing the children with congenital lip-palate malformation, emphasizing their alimentation, such as the difficulties and alternative methods to feed these children and nursing care in this process. The bibliographic review showed that there are many aspects in the alimentation care to fissured children, involving complex affective, social, economic and cultural relations that influence in mother-child relationship, in social discrimination of the fissured children and in mothers' difficulty to feed and give care to the child. Authors found the need to implement breast feeding, pointing out the physiological, psychological and social limitations that involve the child and the family as well as the importance of alimentation to physical and emotional development, favouring the surgical correction of lips and/or palate fissures as well as the rehabilitation process.

  15. Atendimento fonoaudiológico intensivo em pacientes operados de fissura labiopalatina: relato de casos Intensive speech therapy in patients operated for cleft lip and palate: case report

    Directory of Open Access Journals (Sweden)

    Maria do Rosário Ferreira Lima

    2007-09-01

    Full Text Available Devido à carência de fonoaudiólogos para atendimento ao paciente com fissura labiopalatina em várias regiões do Brasil, novos programas de atendimento devem ser desenvolvidos para esses indivíduos. A terapia intensiva de fala tem sido relatada na literatura como uma modalidade alternativa. Este trabalho relata a experiência com alguns casos de atendimento fonoaudiológico intensivo, e compara o desempenho na produção da fala de quatro pacientes operados de fissura palatina, antes e após a terapia fonoaudiológica intensiva. Foram atendidos, no período de férias escolares, três adultos e um adolescente que apresentavam distúrbios articulatórios compensatórios. O atendimento teve duração de três horas diárias para cada paciente, durante dez dias, divididos em terapia individual e em grupo. No início e fim do período de terapia, os pacientes foram avaliados por uma fonoaudióloga que não participou dos atendimentos. Também foi gravada em vídeo uma amostra de fala espontânea, contagem de 1 a 20 e repetição de uma lista de palavras e frases com fonemas oclusivos orais e fricativos. Todos os pacientes mostraram evolução satisfatória na terapia intensiva, com adequação dos fonemas trabalhados na fala dirigida, necessitando ainda de acompanhamento fonoterápico para sua automatização. A terapia intensiva mostrou ser uma alternativa eficaz e viável nesses casos, podendo também ser uma estratégia durante o início do tratamento fonoaudiológico convencional.Due to the lack of speech therapists at various regions of Brazil to assist patients with cleft lip and palate, new intervention programs must be developed for these individuals. Intensive speech therapy has been cited in literature as an alternative modality. This article relates the experience of four cleft lip patients, comparing their speech performances before and after the intensive intervention. The subjects, three adults and one adolescent with compensatory

  16. Kyphosis reduction and the rate of cement leaks after vertebroplasty of intravertebral clefts

    Energy Technology Data Exchange (ETDEWEB)

    Krauss, Martin; Tomandl, Bernd; Baer, Ingrid [Klinikum Sued, Institute for Diagnostic and Interventional Radiology, Nuernberg (Germany); Hirschfelder, Horst; Lichti, Gabriele [Klinikum Sued, Department of Physical and Rehabilitative Medicine, Nuernberg (Germany)

    2006-05-15

    To assess the results of vertebroplasty in patients with intravertebral clefts compared to patients with normal osteoporotic fractures, we evaluated the pre- and postoperative images and pain scores (VAS) of 114 patients with 192 vertebroplasty procedures treated between March 2002 and February 2005. Intravertebral clefts were identified on conventional radiographs, MR or CT images as gas- or fluid-filled spaces adjacent to an endplate of a fractured vertebra. Forty-four vertebrae showed intravertebral clefts. All clefts were filled with PMMA showing a typical filling pattern. Due to the prone positioning of the patient during vertebroplasty, a significant reduction of the kyphosis angle was achieved in the cleft group. Cement leakage occurred in 18.2% of clefts and 46% of regular osteoporotic fractures. In all patients, good filling of the cleft was achieved no matter where the needle tip was placed in the vertebra. The VAS score was 9.1 preoperatively, 3.6 before discharge and 3.9 6 months postoperatively, showing no significant difference between both groups. Patients with intravertebral clefts show a significant reduction of the kyphosis angle compared to non-cleft patients and have a significantly lower risk of experiencing cement leakage during vertebroplasty. Pain reduction is the same in both groups. (orig.)

  17. Kyphosis reduction and the rate of cement leaks after vertebroplasty of intravertebral clefts

    International Nuclear Information System (INIS)

    To assess the results of vertebroplasty in patients with intravertebral clefts compared to patients with normal osteoporotic fractures, we evaluated the pre- and postoperative images and pain scores (VAS) of 114 patients with 192 vertebroplasty procedures treated between March 2002 and February 2005. Intravertebral clefts were identified on conventional radiographs, MR or CT images as gas- or fluid-filled spaces adjacent to an endplate of a fractured vertebra. Forty-four vertebrae showed intravertebral clefts. All clefts were filled with PMMA showing a typical filling pattern. Due to the prone positioning of the patient during vertebroplasty, a significant reduction of the kyphosis angle was achieved in the cleft group. Cement leakage occurred in 18.2% of clefts and 46% of regular osteoporotic fractures. In all patients, good filling of the cleft was achieved no matter where the needle tip was placed in the vertebra. The VAS score was 9.1 preoperatively, 3.6 before discharge and 3.9 6 months postoperatively, showing no significant difference between both groups. Patients with intravertebral clefts show a significant reduction of the kyphosis angle compared to non-cleft patients and have a significantly lower risk of experiencing cement leakage during vertebroplasty. Pain reduction is the same in both groups. (orig.)

  18. A Cross-sectional Comparison of Cleft Lip Severity in 3 Regional Populations

    OpenAIRE

    Lanteri, Alexis Caitlin; Parcells, Bertrand William; Lizarraga, Ana Karina; Magee, William; Bermudez, Luis

    2012-01-01

    Objective: The purpose of this cross-sectional study was to compare the severity of unilateral cleft lips in populations of Asia, Sub-Saharan Africa, and Northern Africa and the Middle East. We hypothesize that severity of unilateral cleft lips shows significant variation between these populations. Methods: Medical photographs of 780 patients with primary unilateral cleft lips treated by Operation Smile during November 2007 were reviewed. Photographs of 352 patients from Asia (China, Philippi...

  19. Contemporary Approaches in the Repair of Alveolar Clefts

    Directory of Open Access Journals (Sweden)

    Ufuk Tatli

    2014-08-01

    Full Text Available Cleft lip and palate is one of the most common craniofacial anomalies. The repair of the alveolar clefts is an important part of the treatment for patients with cleft lip and palate. The treatment concepts of alveolar bone grafting are still controversial. The corresponding controversial issues are; timing of alveolar bone grafting, graft materials, and timing of the orthodontic expansion. In the present article, aforementioned controversial issues and contemporary treatment modalities of the maxillary alveolar clefts were reviewed in the light of current literature. In conclusion, the most suitable time for alveolar bone grafting is mixed dentition period. Grafting procedure may be performed in the early or late phases of this period depending on some clinical features. Adjunct orthodontic expansion procedures should be performed before and/or after grafting depending on the patient's current features. [Archives Medical Review Journal 2014; 23(4.000: 563-574

  20. Influence of the Alveolar Cleft Type on Preoperative Estimation Using 3D CT Assessment for Alveolar Cleft

    Directory of Open Access Journals (Sweden)

    Hang Suk Choi

    2012-09-01

    Full Text Available Background The bone graft for the alveolar cleft has been accepted as one of the essentialtreatments for cleft lip patients. Precise preoperative measurement of the architecture andsize of the bone defect in alveolar cleft has been considered helpful for increasing the successrate of bone grafting because those features may vary with the cleft type. Recently, somestudies have reported on the usefulness of three-dimensional (3D computed tomography(CT assessment of alveolar bone defect; however, no study on the possible implication of thecleft type on the difference between the presumed and actual value has been conducted yet.We aimed to evaluate the clinical predictability of such measurement using 3D CT assessmentaccording to the cleft type.Methods The study consisted of 47 pediatric patients. The subjects were divided according tothe cleft type. CT was performed before the graft operation and assessed using image analysissoftware. The statistical significance of the difference between the preoperative estimationand intraoperative measurement was analyzed.Results The difference between the preoperative and intraoperative values were -0.1±0.3cm3 (P=0.084. There was no significant intergroup difference, but the groups with a cleftpalate showed a significant difference of -0.2±0.3 cm3 (P<0.05.Conclusions Assessment of the alveolar cleft volume using 3D CT scan data and image analysissoftware can help in selecting the optimal graft procedure and extracting the correct volumeof cancellous bone for grafting. Considering the cleft type, it would be helpful to extract anadditional volume of 0.2 cm3 in the presence of a cleft palate.

  1. Considerations Regarding Age at Surgery and Fistula Incidence Using One- and Two-stage Closure for Cleft Palate

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    Simona Stoicescu

    2013-12-01

    Full Text Available Introduction: Although cleft lip and palate (CLP is one of the most common congenital malformations, occurring in 1 in 700 live births, there is still no generally accepted treatment protocol. Numerous surgical techniques have been described for cleft palate repair; these techniques can be divided into one-stage (one operation cleft palate repair and two-stage cleft palate closure. The aim of this study is to present our cleft palate team experience in using the two-stage cleft palate closure and the clinical outcomes in terms of oronasal fistula rate. Material and methods: A retrospective analysis was performed on medical records of 80 patients who underwent palate repair over a five-year period, from 2008 to 2012. All cleft palate patients were incorporated. Information on patient’s gender, cleft type, age at repair, one- or two-stage cleft palate repair were collected and analyzed. Results: Fifty-three (66% and twenty-seven (34% patients underwent two-stage and one-stage repair, respectively. According to Veau classification, more than 60% of them were Veau III and IV, associating cleft lip to cleft palate. Fistula occurred in 34% of the two-stage repairs versus 7% of one-stage repairs, with an overall incidence of 24%. Conclusions: Our study has shown that a two-stage cleft palate closure has a higher rate of fistula formation when compared with the one-stage repair. Two-stage repair is the protocol of choice in wide complete cleft lip and palate cases, while one-stage procedure is a good option for cleft palate alone, or some specific cleft lip and palate cases (narrow cleft palate, older age at surgery

  2. Syntelencephaly associated with connected transhemispheric cleft of focal cortical dysplasia.

    Science.gov (United States)

    Fujimoto, S; Togari, H; Banno, T; Wada, Y

    1999-05-01

    The authors report a female with syntelencephaly associated with a connected transhemispheric cleft of focal cortical dysplasia. Syntelencephaly is a rare anomaly characterized by fusion of the hemispheres in the posterior frontal and parietal regions and is considered a new variant of holoprosencephaly. Cranial magnetic resonance imaging of the patient revealed syntelencephaly associated with bilateral fused clefts of focal cortical dysplasia without the pial-ependymal seam, which was regarded as an incomplete type of schizencephaly. The underlying mechanism is discussed. PMID:10371387

  3. ENDOTRACHEAL INTUBATION IN A CHILD HAVING OCCIPITAL ENCEPHALOCELE WITH BILATERAL CLEFT LIP AND CLEFT PALATE: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Shridhar N

    2014-11-01

    Full Text Available : Basically endotracheal intubation in pediatric age group especially in infants is difficult due to large head, relatively large tongue, anteriorly placed larynx, longer and stiffer epiglottis which protrudes at 450 angle and short neck. When such pediatric patient comes with craniofacial congenital malformations, the management of airway becomes more challenging. Here we report a case of occipital encephalocele associated with bilateral cleft lip and cleft palate coming for V P shunt procedure.

  4. Unilateral Cleft Lip and Nasal Repair: Techniques and Principles

    Directory of Open Access Journals (Sweden)

    Mohammad-Ali Raisolsadat

    2011-06-01

    Full Text Available The Mashhad University of Medical Sciences and the Sheikh Hospital in Mashhad sponsored a Cleft Lip and Palate Workshop 30 April - 1 May 2009. During the Workshop, 6 surgical cases were performed and televised live to the audience attending the conference. Two of those cases were unilateral cleft lip repairs. The surgical technique used to repair these patients by the primary author (JGM is a hybrid technique. It has evolved over the last decade as a result of prior surgical literature as well as first hand observation of various surgical colleagues. The following manuscript describes the surgical technique used at the Cleft Workshop in a step-wise or atlas-like fashion. The technique portion of the paper describes the repair of the unilateral cleft lip and nasal deformity in roughly the order the first author typically performs the procedure. More importantly, the final section of the paper details the principles that form the foundation for the techniques described.

  5. Variability in palatal shape and size in patients with bilateral complete cleft lip and palate assessed using dense surface model construction and 3D geometric morphometrics

    Czech Academy of Sciences Publication Activity Database

    Bejdová, Š.; Krajíček, V.; Peterka, Miroslav; Trefný, P.; Velemínská, J.

    2012-01-01

    Roč. 40, č. 3 (2012), s. 201-208. ISSN 1010-5182 Grant ostatní: GA MŠk(CZ) MSM0021620843 Institutional research plan: CEZ:AV0Z50390512 Keywords : orofacial cleft * palatal shape * laser scranning Subject RIV: EA - Cell Biology Impact factor: 1.610, year: 2012

  6. Cleft lip and palate: recommendations for dental anesthetic procedure based on anatomic evidences

    OpenAIRE

    Ivy Kiemle Trindade-Suedam; Bruno Felipe Gaia; Cheong Kuo Cheng; Paulo Alceu Kiemle Trindade; José Carlos da Cunha Bastos; Beatriz Silva Câmara Mattos

    2012-01-01

    Patients with cleft lip and palate usually present dental anomalies of number, shape, structure and position in the cleft area and the general dentist is frequently asked to restore or extract those teeth. Considering that several anatomic variations are expected in teeth adjacent to cleft areas and that knowledge of these variations by general dentists is required for optimal treatment, the objectives of this paper are: 1) to describe changes in the innervation pattern of anterior teeth and ...

  7. Clinical Outcomes of Primary Palatal Surgery in Children with Nonsyndromic Cleft Palate with and without Lip

    OpenAIRE

    Seunghee Ha; Kyung S. Koh; Heewon Moon; Seungeun Jung; Tae Suk Oh

    2015-01-01

    This study presents clinical outcomes of primary cleft palate surgery, including rate of oronasal fistula development, rate of velopharyngeal insufficiency (VPI) requiring secondary surgery, and speech outcomes. We examined the effect of cleft type on the clinical outcomes. Retrospective analysis was performed using clinical records of all patients who received a primary palatoplasty at the Cleft Palate Clinic at Seoul Asan Medical Center, South Korea, between 2007 and 2012. The study include...

  8. 中国汉族不伴唇腭裂的缺指(趾)-外胚层发育异常-唇腭裂综合征TP63基因的杂合性突变一例%Heterozygous TP63 mutation in a Chinese patient with ectrodactyly-ectodermal dysplasia clefting syndrome without clefting

    Institute of Scientific and Technical Information of China (English)

    韩冬; 吴华; 张晓霞; 冯海兰

    2010-01-01

    Objective To determine if alteration in TP63 is responsible for a Chinese patient with ectrodactyly-ectodermal dysplasia clefting (EEC) syndrome, but without cleft palate/lip. Methods Screening of TP63 gene was performed in the patient with EEC syndrome and his family members using PCR-single strand conformational polymorphism (SSCP) analysis, then performed by direct sequencing of the coding region. Results A C > T substitution at nucleotide position 838 in exon 7 was detected in the patient, and the change predicted a heterozygous missense mutation, Arg280Cys. His parents showed the wild type. Conclusions The results indicate that the de novo mutation Arg280Cys of the TP63 gene observed in the patient maybe contribute to his EEC syndrome.%目的 研究不伴唇腭裂表现的缺指(趾)-外胚层发育异常-唇腭裂(ectrodactylyectodermal dysplasia clefting,EEC)综合征1例患者的TP63基因突变,探讨该病基因型与表型的关系.方法 利用DNA单链构象多态性实验对1个中国汉族不伴唇腭裂表现的EEC综合征核心家系进行突变初筛,聚合酶链反应扩增目的 基因片段,直接测序进行突变检测,以200名无先天性缺牙的健康者作为对照.结果 在患者TP63基因第7外显子cDNA838位存在C>T的单碱基杂合性点突变,使其编码的第280位精氨酸替换为半胱氨酸(Arg280Cys,R280C).患者父母在该位点均显示正常的野生基因型.结论 TP63基因的单碱基杂合性突变(Arg280Cys,R280C)是引起该患者EEC综合征的致病原因,此突变为新生突变.

  9. Pattern of disocclusion in patients with complete cleft lip and palate Padrão de desoclusão em indivíduos com fissura lábio-palatina completa

    Directory of Open Access Journals (Sweden)

    Daniella Andaluza Dias Matos

    2006-06-01

    Full Text Available OBJECTIVE: to analyze the pattern of disocclusion during excursive mandibular movements and presence or absence of occlusal interferences and occlusal pathologies (gingival recession and abfraction. METHOD: examination of 120 individuals divided into two groups, as follows: Group 1 - 90 patients with complete cleft lip and palate (study group, subdivided into 30 patients with complete left unilateral cleft lip and palate, 30 patients with complete right unilateral cleft lip and palate and 30 patients with complete bilateral cleft lip and palate; Group 2 - 30 individuals without clefts (control group. RESULTS: 58.8% of patients in Group 1 presented unilateral or bilateral canine guidance, 26.6% presented unilateral or bilateral group function and 54.4% presented lateral movements through the posterior teeth. Regarding protrusive movements, 80% presented anterior guidance and 20% presented posterior guidance. In Group 2, 69.6% of individuals presented unilateral or bilateral canine guidance, 43.2% presented unilateral or bilateral group function and only 13.3% presented lateral movements through the posterior teeth; 3.4% presented protrusion through the posterior teeth. CONCLUSIONS: there was no difference in the pattern of disocclusion between subgroups of patients with clefts. Group 2 presented predominance of bilateral group function, whereas Group 1 presented a higher prevalence of posterior guidance during lateral movements. Protrusion occurred primarily through anterior guidance in Group 2 and through the posterior teeth in Group 1. There was high prevalence of occlusal interferences at the molar area for both groups, yet with no correlation with occlusal pathologies (recession and abfraction.OBJETIVO: avaliar o padrão de desoclusão apresentado nos movimentos excursivos mandibulares e a presença ou não de interferências oclusais e patologias relacionadas à oclusão (recessão gengival e abfração. MÉTODO: exame de 120 indiv

  10. Is alveolar cleft reconstruction still controversial? (Review of literature

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    Sameh A. Seifeldin

    2016-01-01

    Full Text Available Cleft lip and palate (CL/P is a frequent congenital malformation that manifests in several varieties including unilateral or bilateral and complete or incomplete. Alveolar cleft reconstruction remains controversial with regard to timing, graft materials, surgical techniques, and methods of evaluation. Many studies have been conducted addressing these points to develop an acceptable universal protocol for managing CL/P. The primary goal of alveolar cleft reconstruction in CL/P patients is to provide a bony bridge at the cleft site that allows maxillary arch continuity, oronasal fistula repair, eruption of the permanent dentition into the newly formed bone, enhances nasal symmetry through providing alar base support, orthodontic movement and placement of osseointegrated implants when indicated. Other goals include improving speech, improvement of periodontal conditions, establishing better oral hygiene, and limiting growth disturbances. In order to rehabilitate oral function in CL/P patients alveolar bone grafting is necessary. Secondary bone grafting is the most widely accepted method for treating alveolar clefts. Autogenous bone graft is the primary source for reconstructing alveolar cleft defects and is currently the preferred grafting material.

  11. The trochlear cleft: the ''black line'' of the trochlear trough

    International Nuclear Information System (INIS)

    The ''cartilage black line sign'' is a recently described T2 dark cartilage lesion that we have identified appearing as a cleft in the trochlear trough. The purpose of our study was to define the MR imaging characteristics of a trochlear cleft, determine its incidence, and correlate the MR findings with arthroscopy. A total of 1,300 consecutive MR examinations of the knee were retrospectively reviewed by consensus of two fellowship-trained musculoskeletal radiologists. The MR imaging characteristics and location of a trochlear cleft were determined. Imaging results were compared to arthroscopy when available. Patient age and gender were compared to 25 randomly selected control patients without trochlear clefts. A total of 25 (1.9%) individuals (11 females and 14 males; age range 19-45 years; mean age 28 years) were diagnosed with a trochlear cleft. The control group consisted of 11 females and 14 males; age range 19-83 years; mean age 46 years. Mean cleft length was 7 mm (range 6-12 mm); cleft location was consistently in the lower trochlear trough. No full-thickness cartilage defects were identified in the eight individuals in whom arthroscopic correlation was available. A grade 2 cartilage lesion was identified in a single individual; another progressed from grade 0 to a full-thickness trochlear lesion over an 8-month interval. Eight individuals were athletes. No significant difference in gender was noted between the two groups, however, the study group was significantly younger p < 0.0001. A trochlear cleft is a rare finding in young active individuals. It most likely indicates an incomplete cartilage fissure which may rarely progress to a full-thickness defect. (orig.)

  12. Cleft palate and gonadotrophin deficiency.

    OpenAIRE

    Gillis, P H; Peeters, R.

    1984-01-01

    A boy who had previously had a cleft lip and palate repaired and bilateral orchiopexies presented at 16 years of age with delayed puberty. Isolated gonadotrophin deficiency and testicular hyporesponsiveness to human chorionic gonadotrophin were found. The possibility of bilateral cryptorchidism due to gonadotrophin deficiency should be considered in boys with either cleft lip or palate, or both.

  13. Patrones de cierre velofaringeo: Estudio comparativo entre población sana y pacientes con paladar hendido Patterns of velopharyngeal closure: comparative study between healthy population and patients with cleft palate

    Directory of Open Access Journals (Sweden)

    J.R. Prada Madrid

    2010-12-01

    its frequency of appearance in a non-probabilistic sample with healthy volunteers and to compare these findings with the ones obtained in patients with surgically repaired cleft lip and palate. We designed a descriptive study of cross section. The evaluations were made in a fourth level hospital and in the Rehabilitation Centre for Children with Cleft lip and Palate (FISULAB, Santa fe de Bogotá (Colombia. One hundred healthy volunteers (university students and 82 patients with surgically repaired cleft palate participated in the study. Videonasopharyngoscopy was performed to determine patterns of velopharyngeal closure present and their frequency of appearance. The statistical analysis indicate that there is no difference in the frequency of presentation of the patterns of velopharyngeal closure between healthy volunteers and the patients with cleft palate. In the healthy group, the most frequently found pattern was the circular (56% of the sample, followed by the coronal (29 % and circular with Passavant´s ridge (15 %. In the group of patients with cleft lip and palate, the circular pattern also prevails (39,02 %, followed by the coronal pattern (24,39 %, and the circular pattern with Passavant´s ridge (19,51 %. In neither group was found the sagittal pattern. As a conclusion, there was not any statistical difference in the frequency of presentation of the patterns of velopharyngeal closure between the healthy volunteers and the patients with cleft palate.

  14. Genetic determinants of facial clefting

    DEFF Research Database (Denmark)

    Jugessur, Astanand; Shi, Min; Gjessing, Håkon Kristian;

    2009-01-01

    to infer haplotypes. HAPLIN on the other hand estimates the full haplotype distribution over a set of SNPs and estimates relative risks associated with each haplotype. For isolated cleft lip with or without cleft palate (I-CL/P), TRIMM and HAPLIN both identified significant associations with IRF6 and......BACKGROUND: Facial clefts are common birth defects with a strong genetic component. To identify fetal genetic risk factors for clefting, 1536 SNPs in 357 candidate genes were genotyped in two population-based samples from Scandinavia (Norway: 562 case-parent and 592 control-parent triads; Denmark...... ADH1C in both populations, but only HAPLIN found an association with FGF12. For isolated cleft palate (I-CP), TRIMM found associations with ALX3, MKX, and PDGFC in both populations, but only the association with PDGFC was identified by HAPLIN. In addition, HAPLIN identified an association with ETV5...

  15. A review of cleft lip and palate management: Experience of a Nigerian Teaching Hospital

    Science.gov (United States)

    Efunkoya, Akinwale Adeyemi; Omeje, Kelvin Uchenna; Amole, Ibiyinka Olushola; Osunde, Otasowie Daniel; Akpasa, Izegboya Olohitae

    2015-01-01

    Background: Cleft lip (CL) and palate (CLP) management is multidisciplinary. A cleft team was formed in a Nigerian Tertiary Hospital to address the health needs of cleft patients in the centre. Aim: This paper aims at documenting the Aminu Kano Teaching Hospital (AKTH) management protocol for orofacial clefts and also to review our experience with CLP surgeries performed at AKTH since our partnering with Smile Train. Materials and Methods: A retrospective review of all the cleft patients surgically treated from January 2006 to December 2014 under Smile Train sponsorship was undertaken. A descriptive narrative of the cleft team protocol was also given. Results: One hundred and fifty-five patients (80 males, 75 females) had surgical repairs of either the lip or palate. CL patients were 83 (53.55%), while CLP patients were 45 (29.03%) and isolated cleft palate patients were 27 (17.42%). Conclusion: The inclusion of various specialities in the cleft team is highly desirable. Poverty level amongst our patients frequently limits our management to surgical treatment sponsored by the Smile Train, despite the presence of other residual problems. PMID:26712291

  16. CLEFT PALATE. FOUNDATIONS OF SPEECH PATHOLOGY SERIES.

    Science.gov (United States)

    RUTHERFORD, DAVID; WESTLAKE, HAROLD

    DESIGNED TO PROVIDE AN ESSENTIAL CORE OF INFORMATION, THIS BOOK TREATS NORMAL AND ABNORMAL DEVELOPMENT, STRUCTURE, AND FUNCTION OF THE LIPS AND PALATE AND THEIR RELATIONSHIPS TO CLEFT LIP AND CLEFT PALATE SPEECH. PROBLEMS OF PERSONAL AND SOCIAL ADJUSTMENT, HEARING, AND SPEECH IN CLEFT LIP OR CLEFT PALATE INDIVIDUALS ARE DISCUSSED. NASAL RESONANCE…

  17. Epidemiologic Research on Malformations Associated with Cleft Lip and Cleft Palate in Japan

    Science.gov (United States)

    Koga, Hiroshi; Iida, Koichi; Maeda, Tomoki; Takahashi, Mizuho; Fukushima, Naoki; Goshi, Terufumi

    2016-01-01

    To investigate malformations associated with cleft lip and cleft palate, we conducted surveys at neonatal intensive care units (NICUs) and other non-NICU facilities and to determine whether there are differences among facilities. The regional survey investigated NICU facilities located in Oita Prefecture, including 92 patients with cleft lip and palate (CLP) or cleft palate (CP) that occurred between 2004 and 2013, and the national survey investigated oral surgery, plastic surgery, and obstetrics and gynecology facilities located in Japan, including 16,452 patients with cleft lip (CL), CLP, or CP that occurred since 2000. The incidence per 10,000 births was 4.2, 6.2, and 2.8 for CL, CLP, and CP, respectively, according to the national survey, and 6.3 and 2.9 for CLP and CP, respectively according to the regional survey. These results indicated comparable incidences between the two surveys. In contrast, when the survey results on malformations associated with CLP and CP according to the ICD-10 classification were compared between the national survey conducted at oral surgery or plastic surgery facilities and the regional survey conducted at NICU facilities, the occurrence of associated malformations was 19.8% vs. 41.3% for any types of associated malformation, 6.8% vs. 21.7% for congenital heart disease, and 0.5% vs. 16.3% for chromosomal abnormalities. These results indicated that the incidences of all of these associated malformations were significantly greater in the survey conducted at NICU facilities and similar to the findings from international epidemiological surveys. When comparing the survey conducted at obstetrics facilities vs. NICU facilities, the occurrence of associated malformations was similar results as above. The incidence of CLP and CP was not different between surveys conducted at NICU facilities vs. non-NICU facilities; however, when conducting surveys on associated malformations, it is possible to obtain accurate epidemiological data by

  18. Influence of intravertebral cleft on percutaneous vertebroplasty outcome of osteoporotic vertebral compression fractures

    International Nuclear Information System (INIS)

    Objective: To evaluate the influence of intravertebral cleft on percutaneous vertebroplasty (PVP)outcome and the efficacy in the treatment of osteoporotic compression fracture and compare to those without intravertebral cleft. Methods: A retrospective study was conducted to review 95 consecutive PVP procedures for 176 compression fractures. Patients were excluded with more than a single vertebral body involvement neoplasm history, lack of complete imaging materials and follow-up of incoordinated patients. Group A consisted of 18 patients with intravertebral cleft, while group B comprised 25 patients without intravertebral cleft. PMMA leakages were classified as intradiscal, perivertebral soft tissue, perivertebral venous and epidural types. The frequencies of leakage were compared between two groups using χ2 and Fisher exact tests. Visual analogue scale (VAS)and Owestry disability index (ODI)scores were recorded before hand. Results: After PVP, all patients showed significant pain relief and improvement of daily activity function(P 0.05)between the two groups. PMMA leakage occurred in 11 (61.1%)of 18 fractures with intravertebral clefts and 15 (60%)of 25 fractures without intravertebral clefts, revealing no significant difference, but existing between the most frequent seen types in both groups (P < 0.05). Conclusions: PVP is an effective treatment for osteoporotic compression fractures with and without intravertebral cleft. There was no influence of intravertebral clefts on pain relief, improvement of daily activity function and incidence of PMMA leakage besides the PMMA leakage types. (authors)

  19. Stability after Cleft Maxillary Distraction Osteogenesis or Conventional Orthognathic Surgery

    DEFF Research Database (Denmark)

    Andersen, Kristian; Svenstrup, Martin; Pedersen, Thomas Klit;

    2015-01-01

    OBJECTIVES: To compare stability of maxillary advancements in patients with cleft lip and palate following distraction osteogenesis or orthognathic surgery. MATERIAL AND METHODS: INCLUSION CRITERIA: 1) cleft lip and palate, 2) advancement > 8 mm. Eleven patients comprised the distraction...... osteogenesis group (DOG). Seven patients comprised the orthognathic treatment group (CONVG). Skeletal and soft tissue points were traced on lateral cephalograms: T1 (preoperatively), T2 (after surgery), T3 (follow-up). Group differences were analyzed using Students t-test. RESULTS: At T1-T2, advancement of 6...

  20. Frequency of oronasal fistulae in complete cleft palate repair

    International Nuclear Information System (INIS)

    To determine the frequency of oro-nasal fistula in patients undergoing complete cleft palate repair by two flappalatoplasty. Study Design: Case series. Place and Duration of Study: Department of Plastic Surgery, Services Hospital, Lahore, from January to December 2013. Methodology: Patients admitted to the study place for repair of cleft palate after informed consent obtained were included. Cleft palate was repaired by two-flap palatoplasty, using Bardach technique. Patients were discharged on the second postoperative day and followed-up at third week postoperatively. During follow-up visits, fistulae formation and their sites were recorded on pre-designed proforma. Results: Among the total 90 patients, 40 patients (44.4%) were male and 50 patients (55.6%) were female. The mean age was 6.4 +- 5.7 years ranging from 9 months to 20 years. At third week follow-up, 5 patients (5.6%) had fistulae formation. Four patients (80%) had anterior fistulae and one patient (20%) had posterior fistula. Conclusion: With two-flap palatoplasty Bardach procedure for repair of cleft palate, the complication of fistula formation was uncommon at 5.6%, provided the repair was tension free and multi-layered. (author)

  1. Surgical treatment of cleft lip

    OpenAIRE

    Mateus Domingues Miachon; Pedro Luiz Squilacci Leme

    2014-01-01

    We performed a systematic review of the literature on the surgical treatment of cleft lip, emphasizing the prevalence, complications associated with the treatment and the points of disagreement between authors. We conducted a literature cross-sectional search that analyzed publications in books, articles and on the databases SciELO - Scientific Electronic Library Online, PubMed, of the National Center for Biotechnology Information. We conclude that: 1) the severity of the cleft will indicate ...

  2. Bilateral cleft lip nasal deformity

    OpenAIRE

    Singh Arun; Nandini R.

    2009-01-01

    Bilateral cleft lip nose deformity is a multi-factorial and complex deformity which tends to aggravate with growth of the child, if not attended surgically. The goals of primary bilateral cleft lip nose surgery are, closure of the nasal floor and sill, lengthening of the columella, repositioning of the alar base, achieving nasal tip projection, repositioning of the lower lateral cartilages, and reorienting the nares from horizontal to oblique position. The multiplicity of procedures in the li...

  3. Current Controversies in Diagnosis and Management of Cleft Palate and Velopharyngeal Insufficiency

    Directory of Open Access Journals (Sweden)

    Pablo Antonio Ysunza

    2015-01-01

    Full Text Available Background. One of the most controversial topics concerning cleft palate is the diagnosis and treatment of velopharyngeal insufficiency (VPI. Objective. This paper reviews current genetic aspects of cleft palate, imaging diagnosis of VPI, the planning of operations for restoring velopharyngeal function during speech, and strategies for speech pathology treatment of articulation disorders in patients with cleft palate. Materials and Methods. An updated review of the scientific literature concerning genetic aspects of cleft palate was carried out. Current strategies for assessing and treating articulation disorders associated with cleft palate were analyzed. Imaging procedures for assessing velopharyngeal closure during speech were reviewed, including a recent method for performing intraoperative videonasopharyngoscopy. Results. Conclusions from the analysis of genetic aspects of syndromic and nonsyndromic cleft palate and their use in its diagnosis and management are presented. Strategies for classifying and treating articulation disorders in patients with cleft palate are presented. Preliminary results of the use of multiplanar videofluoroscopy as an outpatient procedure and intraoperative endoscopy for the planning of operations which aimed to correct VPI are presented. Conclusion. This paper presents current aspects of the diagnosis and management of patients with cleft palate and VPI including 3 main aspects: genetics and genomics, speech pathology and imaging diagnosis, and surgical management.

  4. How to make young children produce cleft sentences.

    Science.gov (United States)

    Hupet, M; Tilmant, B

    1989-06-01

    The present study focuses on the effects of contextual demands on the selection of a particular syntactic device, in asking whether French-speaking children from 4 to 10 years old will spontaneously produce it-cleft sentences if there is a functional necessity arising from the context. Taking into account recent studies that have specified the discourse function(s) served by this marked sentence form, it was hypothesized that the cleft formulation would be more likely than its uncleft counterpart whenever the child's intention was to contrast their own belief or knowledge with that of their addressee. The study showed this to be the case when the matter of the disagreement concerned the agent of an action: that situation elicited an overwhelming majority (from 80% to 97%) of cleft constructions, even from the youngest children. On the other hand, when the matter of the disagreement concerned the patient, there were only a few cleft constructions, even with the oldest children; contrastive stress on the object constituent was the predominant device employed for marking information in that situation. The high proportion of clefts in the Agent condition, and the high proportion of stressed object constituents in the Patient condition, both differ from previously reported data. These differences are discussed with reference to differences in task requirements and to differences between the prosodic constraints of French and English. PMID:2760126

  5. Associated syndromes and other genetic variations at a South African cleft lip and palate clinic

    Directory of Open Access Journals (Sweden)

    H.J.S. van den Berg

    2009-09-01

    Full Text Available A retrospective study was done of data on all patients registered at one of the largest cleft lip and palate clinics in South Africa (n = 3174. The associated syndromes and other genetic variations [(abbreviation: ASGV] found in the population of persons suffering from facial cleft deformities (FCD were analysed. 832 (26.2% cleft lip and/ or palate patients presented with ASGV. Fifty-seven different types of syndromes were recorded of which the Fairbaim-Robin appearance (FRA (or Pierre Robin sequence 169 (5.3%, the Demarque-van der Woude syndrome 40 (1.3%, and the holoprosencephaly sequence cases 32 (1.0% were the three most common ones. The three most common genetic variations found in the non-syndromic patients, were heart involvement 53(1.7%, club foot 42 (1.3% and various eye problems 39 (1.2%. The main facial cleft deformity, namely the cleft lip, alveolus and palate (CLAP, was found in 26.2% of the ASGV-group. This particular cleft deformity was recorded at 39.7% in the FCD clinic. On the other hand, the hard and soft palate cleft (hPsP group was found in 32.9% of patients who also had ASGV; in the total group of patients registered at the clinic, it accounted for only 16.6%. This means that ASGV occur less commonly in the CLAP group of patients, than in the hPsP group of patients.

  6. Options for the nasal repair of non-syndromic unilateral Tessier no. 2 and 3 facial clefts

    Directory of Open Access Journals (Sweden)

    Srinivas Gosla Reddy

    2014-01-01

    Full Text Available Background: Non-syndromic Tessier no. 2 and 3 facial clefts primarily affect the nasal complex. The anatomy of such clefts is such that the ala of the nose has a cleft. Repairing the ala presents some challenges to the surgeon, especially to correct the shape and missing tissue. Various techniques have been considered to repair these cleft defects. Aim: We present two surgical options to repair such facial clefts. Materials and Methods: A nasal dorsum rotational flap was used to treat patients with Tessier no. 2 clefts. This is a local flap that uses tissue from the dorsal surface of the nose. The advantage of this flap design is that it helps move the displaced ala of a Tessier no. 2 cleft into its normal position. A forehead-eyelid-nasal transposition flap design was used to treat patients with Tessier no. 3 clefts. This flap design includes three prongs that are rotated downward. A forehead flap is rotated into the area above the eyelid, the flap from above the eyelid is rotated to infra-orbital area and the flap from the infraorbital area that includes the free nasal ala of the cleft is rotated into place. Results and Conclusions: These two flap designs show good results and can be used to augment the treatment options for repairing Tessier no. 2 and 3 facial clefts.

  7. Tissue-plastinated vs. celloidin-embedded large serial sections in video, analog and digital photographic on-screen reproduction: a preliminary step to exact virtual 3D modelling, exemplified in the normal midface and cleft-lip and palate

    Science.gov (United States)

    Landes, Constantin A; Weichert, Frank; Geis, Philipp; Wernstedt, Katrin; Wilde, Anja; Fritsch, Helga; Wagner, Mathias

    2005-01-01

    This study analyses tissue-plastinated vs. celloidin-embedded large serial sections, their inherent artefacts and aptitude with common video, analog or digital photographic on-screen reproduction. Subsequent virtual 3D microanatomical reconstruction will increase our knowledge of normal and pathological microanatomy for cleft-lip-palate (clp) reconstructive surgery. Of 18 fetal (six clp, 12 control) specimens, six randomized specimens (two clp) were BiodurE12-plastinated, sawn, burnished 90 µm thick transversely (five) or frontally (one), stained with azureII/methylene blue, and counterstained with basic-fuchsin (TP-AMF). Twelve remaining specimens (four clp) were celloidin-embedded, microtome-sectioned 75 µm thick transversely (ten) or frontally (two), and stained with haematoxylin–eosin (CE-HE). Computed-planimetry gauged artefacts, structure differentiation was compared with light microscopy on video, analog and digital photography. Total artefact was 0.9% (TP-AMF) and 2.1% (CE-HE); TP-AMF showed higher colour contrast, gamut and luminance, and CE-HE more red contrast, saturation and hue (P < 0.4). All (100%) structures of interest were light microscopically discerned, 83% on video, 76% on analog photography and 98% in digital photography. Computed image analysis assessed the greatest colour contrast, gamut, luminance and saturation on video; the most detailed, colour-balanced and sharpest images were obatined with digital photography (P < 0.02). TP-AMF retained spatial oversight, covered the entire area of interest and should be combined in different specimens with CE-HE which enables more refined muscle fibre reproduction. Digital photography is preferred for on-screen analysis. PMID:16050904

  8. Bright Promise for Your Child with Cleft Lip and Cleft Palate. Revised Edition.

    Science.gov (United States)

    McDonald, Eugene T.; Berlin, Asa J.

    Intended for parents of children with cleft lip and cleft palate, the booklet provides an overview of the condition. Addressed are the following topics (sample subtopics in parentheses): prenatal development and birth defects (facial development); possible causes of cleft lip/cleft palate (common misconceptions, genetic factors, environmental…

  9. Management of cleft lip and palate in adults

    Directory of Open Access Journals (Sweden)

    Murthy Jyotsna

    2009-10-01

    Full Text Available Introduction: With advancement of medical services in developed countries and awareness among the patients, it is rare to find an adult with an unoperated cleft lip and palate. However, the scenario is totally different in developing countries. Working as a part of a team in developing country, where co-coordinated team work is primitive, resources to provide treatment are very thin, public awareness of availability of treatment for this anomaly is minimal, the age of patients reaching for primary treatment varies from few days to late forties. Though the aim and aspiration is to provide holistic multidisciplinary care, the priority is getting treatment for all cleft patients. In such situation, the management of cleft lip and palate demands changes of approach, techniques and philosophy. Aims and Objectives: The deformed anatomy especially the facial bones and dentition is described. Due to well established deformities, the approach for management is individualized. The procedures and modification of procedures has been described. Results and Outcome: The outcome of the primary repair is adults certainly have less than satisfactory outcome for obvious reasons. The expected outcome and expectation of patients and families following primary surgeries in cleft lip and palate has been discussed. Though all adult patients got some improvement in speech after palate repair, achieving normal speech was difficult. The naso-labial appearance was not perfect, but well accepted by the patients and families. There are many psychosocial problems in these patients, the objective evaluation could not be done due to too many variables. However, primary repair of cleft lip and palate is justified and beneficial for the patients.

  10. Risk of Oral Clefts in Twins

    DEFF Research Database (Denmark)

    Grosen, Dorthe; Bille, Camilla; Petersen, Inge;

    2011-01-01

    ratio for twins versus singletons, stratified for 3 subphenotypes. Probandwise concordance rates and heritability for twins were estimated for 2 phenotypes-cleft lip with or without cleft palate (CL/P) and cleft palate (CP). RESULTS:: The prevalence of oral cleft was 15.8 per 10,000 twins and 16.6 per......BACKGROUND:: Small studies have indicated that twinning increases the risk of oral cleft. METHODS:: We used data from a Danish national population-based cohort study to investigate whether twinning was associated with isolated oral cleft, and to estimate the twin probandwise concordance rate and...... heritability. Twins (207 affected/130,710) and singletons (7766 affected/4,798,526) born from 1936 through 2004 in Denmark were ascertained by linkage among the Danish Facial Cleft Database, the Danish Twin Registry, and the Civil Registration System. We computed oral cleft prevalence and prevalence proportion...

  11. Surgical outcome and complications following cleft lip and palate repair in a teaching hospital in Nigeria

    Directory of Open Access Journals (Sweden)

    Taiwo O Abdurrazaq

    2013-01-01

    Full Text Available Background: Measurement of treatment outcome is important in estimating the success of cleft management. The aim of this study was to assess the surgical outcome of cleft lip and palate surgery. Patients and Methods: The surgical outcome of 131 consecutive patients with cleft lip and palate surgeries between October 2008 and December 2010 were prospectively evaluated at least 4 weeks postoperatively. Data collected included information about the age, sex, type of cleft defects, and type of surgery performed as well as postoperative complications. For cleft lip repair, the Pennsylvania lip and nose (PLAN score was used to assess the surgical outcome, while the integrity of the closure was used for cleft palate repair. Results: A total of 92 patients had cleft lip repair and 64 had palate repair. Overall, 68.8% cases of cleft lip and palate repair had good outcomes; 67.9% of lip repairs had good lip and nose scores, while 70.2% of palatal repair had a good surgical outcome. Oro-fistula was observed in 29.8% of cleft palate repairs Inter-rater reliability coefficient was substantially significant. Conclusions: The fact that 25.7% of those treated were aged >1 year suggests a continued need to enlighten the public on the availability of cleft lip and palate expertise and treatment. Although an overall good treatment outcome was demonstrated in this study, the nasal score was poorer than the lip score. Complication rate of about 14% following surgical repair is consistent with previous reports in the literature.

  12. Unilateral Le Fort I Osteotomy for Rehabilitating the Large Alveolar Cleft and Vertical Malocclusion With the Distraction Technique.

    Science.gov (United States)

    Baykul, Timuçin; Aydin, Mustafa Asim; Findik, Yavuz; Esenlik, Elçin

    2016-05-01

    Rehabilitation of the large alveolar clefts with autogenous graft or distraction osteogenesis is one of the most common treatment choices. Depending on the clinical situation such as vertical deficiencies, linear transport of the segment does not always ensure a proper closure of the cleft space. In this report, the authors present a patient in whom large unilateral cleft and vertical alveolar deficiency were closed by unilateral Le Fort I osteotomy and distraction technique using the orthodontic elastics. PMID:27054420

  13. Aesthetic Evaluation of the Nasolabial Region in Children with Unilateral Cleft Lip and Palate Comparing Expert versus Nonexperience Health Professionals

    OpenAIRE

    2014-01-01

    Esthetic evaluation of cleft lip and palate rehabilitation outcomes may assist in the determination of new surgical interventions and aid in reevaluation of treatment protocols. Our objective was to compare esthetics assessments of the nasolabial region in children with a unilateral cleft lip and palate between healthcare professionals who were experienced in the treatment of cleft lip and palate and those who were inexperienced. The study group included 55 patients between 6 and 12 years of ...

  14. Anterior Segmental Distraction Osteogenesis in the Hypoplastic Cleft Maxilla: Report of five cases

    OpenAIRE

    Sruthi Rao (Janardhan); Kotrashetti, S. M.; J. B. Lingaraj; Pinto, P. X.; Keluskar, K. M.; Siddharth Jain; Piyush Sone; Santhosh Rao

    2013-01-01

    Orthognathic surgery and distraction osteogenesis play a prime role in the correction of maxillary hypoplasia in patients with cleft lip and palate (CLP). Advancement of the anterior maxilla alone without interfering with the velopharyngeal sphincter may be advantageous in cleft patients, who more commonly have speech deficits and dental crowding. We present a case series of anterior maxillary segmental distraction for maxillary hypoplasia in 5 CLP patients with a one-year follow-up. A custom...

  15. CT appearances of unilateral cleft palate 20 years after bone graft surgery

    Energy Technology Data Exchange (ETDEWEB)

    Kolbenstvedt, A.; Aaloekken, T.M. [Rikshospitalet, Oslo (Norway). Dept. of Radiology; Arctander, K. [Rikshospitalet, Oslo (Norway). Dept. of Plastic Surgery; Johannessen, S. [Inst. of Clinical Dentistry, Oslo (Norway)

    2002-11-01

    Purpose: To describe CT appearances in patients with unilateral cleft lip and palate (CLP) 20 years after bone graft surgery. Material and Methods: Eighteen consecutive patients with unilateral CLP were examined. All patients had been treated with primary closure, both in infancy and early childhood, supplemented with bone grafting at the age of around 10 years. The CT examination of the upper jaw included a dental CT program. The CT appearances of the cleft side were compared with those of the untreated non-cleft side. Results: Abnormal CT appearances included skew nasal aperture (n=17), nasal septal deviation (n=17), low floor of nasal aperture (n=15) at or towards the cleft side, and deviation of anterior nasal spine towards the non-cleft side (n=18). The posterior part of the bone cleft was visible in all patients, and the dental arch was V-shaped in 8. Conclusion: Although adherence to the present treatment protocol is considered to give satisfactory functional and cosmetic results, certain abnormalities persist. A knowledge of these is a prerequisite for a complete and final evaluation of the surgical and orthodontic regimen. Cleft palate nasal cavity abnormalities CT.

  16. CT appearances of unilateral cleft palate 20 years after bone graft surgery

    International Nuclear Information System (INIS)

    Purpose: To describe CT appearances in patients with unilateral cleft lip and palate (CLP) 20 years after bone graft surgery. Material and Methods: Eighteen consecutive patients with unilateral CLP were examined. All patients had been treated with primary closure, both in infancy and early childhood, supplemented with bone grafting at the age of around 10 years. The CT examination of the upper jaw included a dental CT program. The CT appearances of the cleft side were compared with those of the untreated non-cleft side. Results: Abnormal CT appearances included skew nasal aperture (n=17), nasal septal deviation (n=17), low floor of nasal aperture (n=15) at or towards the cleft side, and deviation of anterior nasal spine towards the non-cleft side (n=18). The posterior part of the bone cleft was visible in all patients, and the dental arch was V-shaped in 8. Conclusion: Although adherence to the present treatment protocol is considered to give satisfactory functional and cosmetic results, certain abnormalities persist. A knowledge of these is a prerequisite for a complete and final evaluation of the surgical and orthodontic regimen. Cleft palate nasal cavity abnormalities CT

  17. Clinical photography among African cleft caregivers

    Directory of Open Access Journals (Sweden)

    Peter Babatunde Olaitan

    2011-01-01

    Full Text Available Objective: The aim of this paper is to document the practice of photography among clinicians whose daily work depends and is influenced so much by medical photography. Materials and Methods: Questionnaires documenting the bio data, place of practice, and experience of cleft caregivers with clinical photography were distributed. Knowledge of rules guiding clinical photography and adherence to them were also asked. Types of camera used were documented and knowledge of the value of clinical photographs were also inquired. Results: Plastic surgeons constitute the highest proportion of 27 (38.6%, followed by Oral and Maxillofacial surgeons with 14 (20.0%. Twenty one (30.0% of the respondents always, 21 (30.0% often, 12 (17.1% frequently, while 9 respondents sometimes took photographs of their patients. Suggested uses of clinical photographs included training, 52 (74.3%, education, 51 (72.9%, medicolegal, 44 (62.9% and advertisement, 44 (62.9% among others. Twenty two (31.4% did not know that there were standard guidelines for taking clinical photographs. Twenty three (32.9% of them did not seek the consent of the patients before taking clinical photographs. Conclusion: While the practice of clinical photography is high among African cleft caregivers, there is a need for further education on the issues of standard rules and obtaining consent from patients.

  18. Three-Dimensional Assessment of Early Surgical Outcome in Repaired Unilateral Cleft Lip and Palate: Part 2. Lip Changes.

    LENUS (Irish Health Repository)

    Ayoub, Ashraf

    2010-09-08

    Abstract Objective: To evaluate 3D lip morphology, following primary reconstruction in children with unilateral cleft lip and palate (UCLP) relative to contemporaneous non-cleft data Design: Prospective, cross-sectional, controlled study Setting: Glasgow Dental Hospital & School, University of Glasgow, The UK. Patients and Participants: Two groups of 3-year old children (21 with unilateral cleft lip and palate and 96 controls) with facial images taken using a 3D vision based capture technique. Methods: 3D images of the face were reflected so the cleft was on the left side to create a homogenous group for statistical analysis. Three-dimensional co-ordinates of anthropometric landmarks were extracted from facial images. 3D generalised Procustes superimposition was employed and a set of linear measurements were utilised to compare between cleft and control subjects for right and left sides, adjusting for sex differences. Results: Christa philteri on both the cleft and non-cleft sides were displaced laterally and posteriorly; there was also a statistically significant increase in philtrum width. No significant differences between cleft and control regarding the cutaneous height of the upper lip. The lip in the cleft cases was flatter than in the non-cleft cases with less prominence of labialis superioris. Conclusions: Stereophotogrammetry allows detection of residual dysmorphology following cleft repair. There was significant increase of the philtrum width. The lip appeared flatter and more posterior displaced in Unilateral Cleft Lip and Palate (UCLP) cases compared with control. Keywords: child, cleft lip and palate, lip repair, three-dimensional imaging.

  19. Cleft lip: The historical perspective

    Directory of Open Access Journals (Sweden)

    Bhattacharya S

    2009-10-01

    Full Text Available The earliest documented history of cleft lip is based on a combination of religion, superstition, invention and charlatanism. While Greeks ignored their existence, Spartans and Romans would kill these children as they were considered to harbour evil spirits. When saner senses prevailed Fabricius ab Aquapendente (1537-1619 was the first to suggest the embryological basis of these clefts. The knowledge of cleft lip and the surgical correction received a big boost during the period between the Renaissance and the 19th century with the publication of Pierre Franco′s Petit Traite and Traite des Hernies in which he described the condition as "lievre fendu de nativite" (cleft lip present from birth. The first documented Cleft lip surgery is from China in 390 BC in an 18 year old would be soldier, Wey Young-Chi. Albucasis of Arabia and his fellow surgeons used the cautery instead of the scalpel and Yperman in 1854 recommended scarifying the margins with a scalpel before suturing them with a triangular needle dipped in wax. The repair was reinforced by passing a long needle through the two sides of the lip and fixing the shaft of the needle with a figure-of-eight thread over the lip. Germanicus Mirault can be credited to be the originator of the triangular flap which was later modified by C.W. Tennison in 1952 and Peter Randall in 1959. In the late 50s, Ralph Millard gave us his legendary ′cut as you go′ technique. The protruding premaxilla of a bilateral cleft lip too has seen many changes throughout the ages - from being discarded totally to being pushed back by wedge resection of vomer to finally being left to the orthodontists.

  20. Surgical treatment of cleft lip

    Directory of Open Access Journals (Sweden)

    Mateus Domingues Miachon

    2014-06-01

    Full Text Available We performed a systematic review of the literature on the surgical treatment of cleft lip, emphasizing the prevalence, complications associated with the treatment and the points of disagreement between authors. We conducted a literature cross-sectional search that analyzed publications in books, articles and on the databases SciELO - Scientific Electronic Library Online, PubMed, of the National Center for Biotechnology Information. We conclude that: 1 the severity of the cleft will indicate the technique presenting more advantages; 2 the different approaches indicate that there is no consensus on the optimal technique; and 3 the surgeon experience contributes to choosing the best option.

  1. Evaluation of cleft palate speech.

    Science.gov (United States)

    Smith, Bonnie; Guyette, Thomas W

    2004-04-01

    Children born with palatal clefts are at risk for speech/language delay and speech problems related to palatal insufficiency. These individuals require regular speech evaluations, starting in the first year of life and often continuing into adulthood. The primary role of the speech pathologist on the cleft palate/craniofacial team is to evaluate whether deviations in oral cavity structures, such as the velopharynx, negatively impact speech production. This article focuses on the assessment of velopharyngeal function before and after palatal surgery. PMID:15145667

  2. Oral cleft prevention program (OCPP

    Directory of Open Access Journals (Sweden)

    Wehby George L

    2012-11-01

    Full Text Available Abstract Background Oral clefts are one of the most common birth defects with significant medical, psychosocial, and economic ramifications. Oral clefts have a complex etiology with genetic and environmental risk factors. There are suggestive results for decreased risks of cleft occurrence and recurrence with folic acid supplements taken at preconception and during pregnancy with a stronger evidence for higher than lower doses in preventing recurrence. Yet previous studies have suffered from considerable design limitations particularly non-randomization into treatment. There is also well-documented effectiveness for folic acid in preventing neural tube defect occurrence at 0.4 mg and recurrence with 4 mg. Given the substantial burden of clefting on the individual and the family and the supportive data for the effectiveness of folic acid supplementation as well as its low cost, a randomized clinical trial of the effectiveness of high versus low dose folic acid for prevention of cleft recurrence is warranted. Methods/design This study will assess the effect of 4 mg and 0.4 mg doses of folic acid, taken on a daily basis during preconception and up to 3 months of pregnancy by women who are at risk of having a child with nonsyndromic cleft lip with/without palate (NSCL/P, on the recurrence of NSCL/P. The total sample will include about 6,000 women (that either have NSCL/P or that have at least one child with NSCL/P randomly assigned to the 4 mg and the 0.4 mg folic acid study groups. The study will also compare the recurrence rates of NSCL/P in the total sample of subjects, as well as the two study groups (4mg, 0.4 mg to that of a historical control group. The study has been approved by IRBs (ethics committees of all involved sites. Results will be disseminated through publications and presentations at scientific meetings. Discussion The costs related to oral clefts are high, including long term psychological and socio-economic effects. This study

  3. Holoprosencephaly and Ectrodactyly: Report of Three New Cases and Review

    Science.gov (United States)

    Keaton, Amelia A.; Solomon, Benjamin D.; van Essen, Anthonie J.; Pfleghaar, Kathleen M.; Slama, Michael A.; Martin, Judith A.; Muenke, Maximilian

    2009-01-01

    Holoprosencephaly (HPE) and ectrodactyly represent congenital malformations of the developing forebrain and developing digits, respectively. The combinationof these conditions is rare, with only 15 cases known to date (12 previously reported, and 3 new cases described here). While the findings in these patients overlap with previously described genetic conditions, the similarity in phenotypes among these patients has led to the establishment of a at least one distinct syndrome: HPE, ectrodactyly, and bilateral cleft lip-palate syndrome (OMIM 300571). There has been great interest in identifying a genetic cause for the findings in patients with HPE and ectrodactyly; however the cause(s) of this rare association still remain unknown. PMID:20104609

  4. The Effect of Cleft Palate Repair on Contractile Properties of Single Permeabilized Muscle Fibers From Congenitally Cleft Goats Palates

    Science.gov (United States)

    A cleft palate goat model was used to study the contractile properties of the levator veli palatini (LVP) muscle which is responsible for the movement of the soft palate. In 15-25% of patients that undergo palatoplasty, residual velopharyngeal insufficiency (VPI) remains a problem and often require...

  5. Bone grafting, corticotomy, and orthodontics: treatment of cleft alveolus in a chinese cohort.

    Science.gov (United States)

    Mao, Li-Xia; Shen, Guo-Fang; Fang, Bing; Xia, Yun-Hui; Ma, Xu-Hui; Wang, Bo

    2013-11-01

    Objective : A multimodal therapy was applied to solve a set of related problems including collapse of the posterior segment, high level gingival margin of canine, and resorption of grafted bone in a cohort of Chinese youngsters with cleft lip and palate. This study aimed to evaluate the benefits of this treatment procedure. Methods : Thirty patients with unilateral cleft lip and palate were included in this prospective study. All patients had previously undergone only cleft lip and palate repair and presented with alveolar cleft and an obvious step in the gingival margin between the canine tooth and the teeth beside it. A multimodal therapy that included bone grafting, corticotomy, and orthodontics was applied to solve these problems. Grafted bone volume, parallelism of the roots, root resorption, gingival margin, and mobility of the canine on the cleft side were established before surgery, 1 week after surgery, and after straightening of the canine. Results : Less than 25% of the grafted bone was reabsorbed in 25 of the 30 patients, while less than 50% was resorbed in the remaining five. The roots of the canines on the cleft side were mostly parallel to the adjacent teeth. Root resorption and mobility of the canines were slight. The difference in the gingival margin between the canines on the cleft side and the other side was small. Conclusions : Canines moved into the grafted bone safely and effectively, thus achieving a normal gingival margin and retaining grafted bone volume in one operation. PMID:22849663

  6. Syndromic association of cleft palate, bilateral choanal atresia, curly hair, and congenital hypothyroidism.

    OpenAIRE

    Buntincx, I M; Van Overmeire, B; Desager, K; Van Hauwaert, J

    1993-01-01

    We describe a patient with the unusual association of cleft palate, bilateral choanal atresia, curly hair, and congenital hypothyroidism. This association has been reported before in two brothers and may represent a new syndrome.

  7. A study on the cephalometric similarity between parents and offspring in cleft lip with or without palate

    International Nuclear Information System (INIS)

    The purpose of this study was to determine whether any similarity existed in craniofacial morphology between parents and offspring in cleft lip with or without cleft palate. Thirty three measurements of the various regions of cranium and face were obtained from lateral cephalometric radiograms in 28 families comprising 28 fathers, 28 mothers and 28 cleft patients. The measurements of cleft patients compared with those of their fathers, mothers and midparents. The obtained results were as follows: 1. There were similar measurements between the cleft patients and their fathers; ramal height (Ar-Go), mandibular angle (angle MP-RP). 2. There were similar measurements between the cleft patients and their mothers; cranial base angle (angle NSBa), relation of maxilla to the cranial base (angle SNA), relation of maxilla to the cranial base(soft tissue: ?BaNSn), angle of inferior border of mandible (angle SNL-MP ) and convexity of nose apex (soft tissue: angle NPrnPog). 3. There were similar measurements between the cleft patients and their midparents; ramal height (Ar-Go), cranial base angle (angle NSBa), relation of maxilla to the cranial base (soft tissue: angle BaNSn), Y axis angle (angle NSGn) and mandibular angle (angle MP-RP). 4. There was no similar measurements between the cleft patients and their fathers and mothers simultaneously

  8. A study on the cephalometric similarity between parents and offspring in cleft lip with or without palate

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Su Beom; Lee, Un Gyeong; Na, Seung Mog; Koh, Kwang Joon [Dept. of Oral and Maxillofacial Radiology, College of Dentistry, Chonbuk National University, Chonju (Korea, Republic of)

    1994-08-15

    The purpose of this study was to determine whether any similarity existed in craniofacial morphology between parents and offspring in cleft lip with or without cleft palate. Thirty three measurements of the various regions of cranium and face were obtained from lateral cephalometric radiograms in 28 families comprising 28 fathers, 28 mothers and 28 cleft patients. The measurements of cleft patients compared with those of their fathers, mothers and midparents. The obtained results were as follows: 1. There were similar measurements between the cleft patients and their fathers; ramal height (Ar-Go), mandibular angle (angle MP-RP). 2. There were similar measurements between the cleft patients and their mothers; cranial base angle (angle NSBa), relation of maxilla to the cranial base (angle SNA), relation of maxilla to the cranial base(soft tissue: ?BaN{sup S}n), angle of inferior border of mandible (angle SNL-MP) and convexity of nose apex (soft tissue: angle N'PrnPog'). 3. There were similar measurements between the cleft patients and their midparents; ramal height (Ar-Go), cranial base angle (angle NSBa), relation of maxilla to the cranial base (soft tissue: angle BaN{sup S}n), Y axis angle (angle NSGn) and mandibular angle (angle MP-RP). 4. There was no similar measurements between the cleft patients and their fathers and mothers simultaneously

  9. An evaluation of surgical outcome of bilateral cleft lip surgery using a modified Millard′s (Fork Flap technique

    Directory of Open Access Journals (Sweden)

    W L Adeyemo

    2013-01-01

    Full Text Available Background: The central third of the face is distorted by the bilateral cleft of the lip and palate and restoring the normal facial form is one of the primary goals for the reconstructive surgeons. The history of bilateral cleft lip repair has evolved from discarding the premaxilla and prolabium and approximating the lateral lip elements to a definitive lip and primary cleft nasal repair utilising the underlying musculature. The aim of this study was to review surgical outcome of bilateral cleft lip surgery (BCLS done at the Lagos University Teaching Hospital. Materials and Methods: A review of all cases of BCLS done between January 2007 and December 2012 at the Lagos University Teaching Hospital was done. Data analysis included age and sex of patients, type of cleft deformity and type of surgery (primary or secondary and whether the cleft deformity was syndromic and non-syndromic. Techniques of repair, surgical outcome and complications were also recorded. Results: A total of 39 cases of BCLS involving 21 males and 18 females were done during the period. This constituted 10% (39/390 of all cases of cleft surgery done during the period. There were 5 syndromic and 34 non-syndromic cases. Age of patients at time of surgery ranged between 3 months and 32 years. There were 24 bilateral cleft lip and palate deformities and 15 bilateral cleft lip deformities. Thirty-one of the cases were primary surgery, while 8 were secondary (revision surgery. The most common surgical technique employed was modified Fork flap (Millard technique, which was employed in 37 (95% cases. Conclusion: Bilateral cleft lip deformity is a common cleft deformity seen in clinical practice, surgical repair of which can be a challenge to an experienced surgeon. A modified Fork flap technique for repair of bilateral cleft lip is a reliable and versatile technique associated with excellent surgical outcome.

  10. Irf6 directly regulates Klf17 in zebrafish periderm and Klf4 in murine oral epithelium, and dominant-negative KLF4 variants are present in patients with cleft lip and palate

    Science.gov (United States)

    Liu, Huan; Leslie, Elizabeth J.; Jia, Zhonglin; Smith, Tiffany; Eshete, Mekonen; Butali, Azeez; Dunnwald, Martine; Murray, Jeffrey; Cornell, Robert A.

    2016-01-01

    Non-syndromic (NS) cleft lip with or without cleft palate (CL/P) is a common disorder with a strong genetic underpinning. Genome-wide association studies have detected common variants associated with this disorder, but a large portion of the genetic risk for NSCL/P is conferred by unidentified rare sequence variants. Mutations in IRF6 (Interferon Regulatory Factor 6) and GRHL3 (Grainyhead-like 3) cause Van der Woude syndrome, which includes CL/P. Both genes encode members of a regulatory network governing periderm differentiation in model organisms. Here, we report that Krüppel-like factor 17 (Klf17), like Grhl3, acts downstream of Irf6 in this network in zebrafish periderm. Although Klf17 expression is absent from mammalian oral epithelium, a close homologue, Klf4, is expressed in this tissue and is required for the differentiation of epidermis. Chromosome configuration capture and reporter assays indicated that IRF6 directly regulates an oral-epithelium enhancer of KLF4. To test whether rare missense variants of KLF4 contribute risk for NSCL/P, we sequenced KLF4 in approximately 1000 NSCL/P cases and 300 controls. By one statistical test, missense variants of KLF4 as a group were enriched in cases versus controls. Moreover, two patient-derived KLF4 variants disrupted periderm differentiation upon forced expression in zebrafish embryos, suggesting that they have dominant-negative effect. These results indicate that rare NSCL/P risk variants can be found in members of the gene regulatory network governing periderm differentiation. PMID:26692521

  11. Six years analysis of cleft palate in a university hospital center

    Directory of Open Access Journals (Sweden)

    Farahvash M

    1999-08-01

    Full Text Available Cleft palate is a congenital condition that occurs with the incidence rate of one out of 2000 births. This anomaly produces intraoral pressure changes (increase or decrease, can cause speech, sucking and feeding problems of involved patient. On the other hand, if cleft palate is associated with cleft of the lip or alveolar area, growth and alignment of teeth may change the appearance of the patient and affect the psychologic and occupational future of the patient. Eustatian tube malfunction in involved neonates increase. Many procedures are used to repair the cleft palate and correction of palatal muscles which are: 1 Von Langenbeck (18.5%. 2 Veau-Wardil-Kilner (72.5%. 3 Double opposing Z-Plasty (9%. In this research the demographic criteria of patients including age of the patient at operation rime (mean age 30.14 months, city of residence, family history of cleft palate (12.4%, familial relation of parents (15.2%, associated anomalies, complete or incomplete lesion, weight of patients at the time of surgery (mean 11.28 Kg, hemoglobin (11.3 mg/dl, complications, otitis media and the side of cleft palate are studied in 178 admitted patients to Imam General Hospital between 1989 and 1995.

  12. Association of generalized aggressive periodontitis and ectrodactyly-ectodermal dysplasia-cleft syndrome

    OpenAIRE

    Rosamma Joseph; Nath, Sameera G.

    2012-01-01

    Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly, ectodermal dysplasia, and facial clefting. Even though literature has documented the association of various genetic disorders with aggressive periodontitis, the periodontal manifestations in patients with EEC syndrome have never been addressed. This case report presents the periodontal status of three patients in a family with EEC syndrome. The presence of gener...

  13. Bilateral cleft lip nasal deformity

    Directory of Open Access Journals (Sweden)

    Singh Arun

    2009-01-01

    Full Text Available Bilateral cleft lip nose deformity is a multi-factorial and complex deformity which tends to aggravate with growth of the child, if not attended surgically. The goals of primary bilateral cleft lip nose surgery are, closure of the nasal floor and sill, lengthening of the columella, repositioning of the alar base, achieving nasal tip projection, repositioning of the lower lateral cartilages, and reorienting the nares from horizontal to oblique position. The multiplicity of procedures in the literature for correction of this deformity alludes to the fact that no single procedure is entirely effective. The timing for surgical intervention and its extent varies considerably. Early surgery on cartilage may adversely affect growth and development; at the same time, allowing the cartilage to grow in an abnormal position and contributing to aggravation of deformity. Some surgeons advocate correction of deformity at an early age. However, others like the cartilages to grow and mature before going in for surgery. With peer pressure also becoming an important consideration during the teens, the current trend is towards early intervention. There is no unanimity in the extent of nasal dissection to be done at the time of primary lip repair. While many perform limited nasal dissection for the fear of growth retardation, others opt for full cartilage correction at the time of primary surgery itself. The value of naso-alveolar moulding (NAM too is not universally accepted and has now more opponents than proponents. Also most centres in the developing world have neither the personnel nor the facilities for the same. The secondary cleft nasal deformity is variable and is affected by the extent of the original abnormality, any prior surgeries performed and alteration due to nasal growth. This article reviews the currently popular methods for correction of nasal deformity associated with bilateral cleft lip, it′s management both at the time of cleft lip repair

  14. Cleft lip and cleft palate relationship with familial marriage: a study in 136 cases

    Directory of Open Access Journals (Sweden)

    Azimi C

    2010-02-01

    Full Text Available "n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Arial; mso-bidi-theme-font:minor-bidi;} Background: Clefts of the lip and palate are one of the most common congenital birth anomalies. Genetic factors play a great role in the etiology of them and the high percentage of the consanguineous marriage of the parents of the affected persons is one of the reasons. These defects not only make abnormal changes on appearance of the neonate, but also make a lot of stress and psychological problems for the patients and their families. Study on the prevalence of clefts, their risk factors and also genetic counseling for affected persons and their families can be a guideline for general population and probably reduce these anomalies over the generations."n"nMethods: Patients referred to the Department of Genetics, Imam Khomeini Hospital, Tehran, Iran were studied. A total of 7374 pedigrees of all the patients admitted to the Department, were studied during 2002-2005 and 99 pedigrees with the patients with cleft lip± palate or isolated cleft palate were separated. The total number of cases among these 99 pedigrees was 136. The effects of consanguineous marriage, positive family history and sex were investigated among cases."n"nResults: 70.8% of patients with syndromic clefts and 58.7% of patients with nonsyndromic CL

  15. 腭裂不同程度与腭裂语音关系的临床研究%The clinical study of cleft palate speech with different cleft palate

    Institute of Scientific and Technical Information of China (English)

    杨静; 李永生; 冯雁; 王瑜; 邹弘驹; 曹阳

    2013-01-01

    Objective: To investigate the influence of speech for different cleft palate. Methods: 45 cases include 15 cases of soft cleft palate and 15 cases of uncompleted cleft palate and 15 cases of complete cleft palate had been studied with speech evaluate. Results: The speech unclearness appeared positive relation with severe degree of cleft palate. The fact is speech of soft palate cleft is most mild than that of other two groups, the speech of two groups of uncompleted and completed cleft were most severe , and there is no obviously difference between both of them. Conclusion: The pronunciation of patients with every kind of cleft palate were not clear, cleft palate must be accepted palate plastic operation.%目的:探讨不同程度的腭裂对患者语音的影响。方法选取45例腭裂患者,包括软腭裂、不完全性腭裂及完全性腭裂患者各15例,行语音评估。结果腭裂程度越重,患者腭裂语音越明显。软腭裂患者腭裂语音较轻,不全腭裂和完全腭裂组最重。结论不同程度的腭裂均会影响患者发音,腭裂均应行手术修复。

  16. 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.

  17. Incidence of Cleft Lip and Palate in Uganda

    NARCIS (Netherlands)

    Dreise, Marieke; Galiwango, George; Hodges, Andrew

    2011-01-01

    Objective: The purpose of the study was to estimate the need for resources for cleft repairs in Uganda by determining the overall incidence of oral-facial clefts and the ratio of isolated cleft lip to isolated cleft palate to cleft lip and palate. Design: A 1-year prospective study was implemented i

  18. Secondary bone grafting for alveolar cleft in children with cleft lip or cleft lip and palate

    NARCIS (Netherlands)

    J. Guo; C. Li; Q. Zhang; G. Wu; S.A. Deacon; J. Chen; H. Hu; S. Zou; Q. Ye

    2011-01-01

    BACKGROUND: Secondary alveolar bone grafting has been widely used to reconstruct alveolar cleft. However, there is still some controversy. OBJECTIVES: To compare the effectiveness and safety of different secondary bone grafting methods. SEARCH STRATEGY: The final electronic and handsearches were car

  19. Esthetic, Functional, and Everyday Life Assessment of Individuals with Cleft Lip and/or Palate

    Science.gov (United States)

    Papamanou, Despina A.; Karamolegkou, Marina; Dorotheou, Domna

    2015-01-01

    Objectives. To evaluate the level of satisfaction of individuals with cleft lip and/or palate (CLP) and their parents concerning the esthetic and functional treatment outcomes, the impact of the cleft on everyday life, and potential associations with treatment outcome satisfaction. Subjects and Methods. The sample consisted of 33 patients (7 CP, 20 unilateral CLP, and 6 bilateral CLP; median age: 17.1, range: 9.0–33.1 years) and 30 parents, who responded to a questionnaire in an interview-guided session. All participants received their orthodontic treatment at the Department of Orthodontics in the University of Athens. Results. Patients and their parents were quite satisfied with esthetics and function. Patients with UCLP primarily were concerned about nose esthetics (BCLP about lip esthetics and CP about speech). Increased satisfaction was associated with decreased influence of the cleft in everyday life (0.35 < rho < 0.64, P < 0.05). Parents reported significant influence of the cleft on family life, while patients did not. Conclusions. Despite the limited sample size of subgroups, the main concerns of patients with different cleft types and the importance of satisfying lip, nose, and speech outcomes for an undisturbed everyday life were quite evident. Thus, the need for targeted treatment strategies is highlighted for individuals with cleft lip and/or palate. PMID:26064918

  20. Argon laser photocoagulation of cyclodialysis clefts after cataract surgery

    International Nuclear Information System (INIS)

    Three patients with cyclodialysis clefts, hypotony and hypotonic retinopathy subsequent to cataract surgery were treated with argon laser photocoagulation. The hypotony was reversed in each patient and their visual acuity was normalized. Laser photocoagulation is a noninvasive treatment that can be repeated easily and safely. The complications of the treatment are minor. A hypertensive episode commonly occurs in the early postoperative period. (au) 8 refs

  1. Patterns of Cleft Lip and Cleft Palate in Northern Pakistan

    OpenAIRE

    Mansoor Khan; Hidayat Ullah; Shazia Naz; Tahmeed Ullah; Hafeezullah Khan; Muhammad Tahir; Obaid Ullah

    2012-01-01

    Objectives: To determine the frequency of different types of cleft lip and palate, geographical distribution and its association with consanguinity, family history and other syndromes in the Northern Pakistani population. Study design: Descriptive cross-sectional study. Settings: This study was performed in Plastic and Reconstructive units of Hayat Medical Complex Peshawar Pakistan, Aman Hospital Peshawar Pakistan and Abasin Hospital Peshawar, Pakistan from November 2010 to December 20...

  2. Puente adhesivo en paciente con labio leporino y fisura velopalatina: Una solución temporal con compromiso estético Adhesive bridge in a patient with cleft lip and palate: A temporary solution with esthetic commitment

    Directory of Open Access Journals (Sweden)

    N Leiva

    2013-04-01

    Full Text Available En la actualidad el aumento de la demanda estética y la necesidad de procedimientos mínimamente invasivos ha llevado a un aumento de la técnica adhesiva. Ribbond® es una marca de fibra de polietileno que permite un eficiente traspaso de fuerzas, es virtualmente plegable y se adapta fácilmente a la morfología dentaria y al contorno del arco dentario. Dentro de sus principales características podemos encontrar: Biocompatibilidad, inerte, translúcido y durable. Dentro de la odontología se le han dado distintos usos para esta fibra, ya sea como mantenedor de espacio, puente adhesivo, poste y núcleo endodóntico, ferulización, restauraciones tipo inlay y estabilización postortodóncica. En el caso de los pacientes fisurados se ha hecho relevante el uso de Ribbond® como puente adhesivo ya que permite mejorar la estética y rehabilitar temporalmente los espacios desdentados causados por la ausencia de un incisivo, principalmente una vez terminado el tratamiento de ortodoncia y en espera de una rehabilitación definitiva, aún más cuando el paciente no ha terminado su crecimiento y desarrollo. En este artículo se presenta un caso clínico de un paciente con labio leporino y fisura velopalatina operado demostrativo de la técnica de rehabilitación con Ribbond®.Today the increasing demand of aesthetic and minimally invasive procedures has led to a boom of the adhesive technique. Ribbond® is a polyethylene fiber that allows an efficient transfer of forces, is virtually foldable and is easily adapted to tooth morphology and dental arch contour. Among its main features we can find: Biocompatibility, inert, translucent and durable. In dentistry, different uses have been given to this fiber, whether as space maintainer, adhesive bridge, endodontic post and core, splinting, inlay type restorations and postorthodontic stabilization. In the case of cleft lip and palate patients the use of Ribbond® as an adhesive bridge has become relevant, as

  3. Cleft and Craniofacial Mission Care: Management of Facial Clefts: International Missions.

    Science.gov (United States)

    Moses, Jeffrey J; Rochelle, Whitney J

    2016-05-01

    This article guides readers in the venture of creating, funding, or volunteering with a facial-cleft mission treatment team. This outline is intended to emphasize longitudinal care for all patients. Advanced planning is key to mission success. Part of this is researching and becoming familiar with the safety, regulations, culture, facilities available, and local service organizations. Many of the service organizations listed in this article have valuable contacts with local dentists and surgeons within the community. They also have insight into the local facilities, hospitals, water sources, sanitation conditions, and equipment potential. PMID:27150306

  4. Evaluation and integration of disparate classification systems for clefts of the lip

    Directory of Open Access Journals (Sweden)

    KathieHWang

    2014-05-01

    Full Text Available Orofacial clefting is a common birth defect with wide phenotypic variability. Many systems have been developed to classify cleft patterns to facilitate diagnosis, management, surgical treatment, and research. In this review, we examine the rationale for different existing classification schemes and determine their inter-relationships, as well as strengths and deficiencies for subclassification of clefts of the lip. The various systems differ in how they describe and define attributes of cleft lip phenotypes. Application and analysis of the cleft lip classifications reveal discrepancies that may result in errors when comparing studies that use different systems. These inconsistencies in terminology, variable levels of subclassification, and ambiguity in some descriptions may confound analyses and impede further research aimed at understanding the genetics and etiology of clefts, development of effective treatment options for patients, as well as cross-institutional comparisons of outcome measures. Identification and reconciliation of discrepancies among existing systems is the first step towards creating a common standard to allow for a more explicit interpretation that will ultimately lead to a better understanding of the causes and manifestations of phenotypic variations in clefting.

  5. Environmental factors related to the occurrence of oral clefts in a Brazilian subpopulation

    Science.gov (United States)

    Campos Neves, Ana Thereza de Saboia; Volpato, Luiz Evaristo Ricci; Espinosa, Mariano Martinez; Aranha, Andreza Maria Fabio; Borges, Alvaro Henrique

    2016-01-01

    Background: A cross-sectional study was conducted at the Craniofacial Rehabilitation Center in the General Hospital of the University of Cuiabá, Cuiabá city, Mato Grosso, Brazil. Materials and Methods: Poisson regression model was used to analyze the relationship between antenatal factors and the occurrence of oral clefts in 116 patients. Results: Oral clefts were more common in males (64.66%) and White race (46.02%). The mean age of the children was 21.91 months. The most common type of cleft was cleft lip and palate (CLP, 55.17%). Maternal and paternal smoking in the first trimester of pregnancy and parity were significantly associated with the occurrence of CLP. Parent's age, educational level, and occupation did not interfere in the occurrence of oral clefts. There was also no significant association between maternal illness, medication use, alcohol consumption, and maternal exposure to chemicals in the first trimester of pregnancy and the occurrence of clefts in this population. Conclusion: The analysis of the environmental factors present during the pregnancy of children with oral clefts revealed a significant association between parity (second onward), maternal smoking, and paternal smoking and the occurrence of CL and/or palate in this population. PMID:27397957

  6. Quantitative assessment of healthy and reconstructed cleft lip using ultrasonography

    Science.gov (United States)

    Devadiga, Sumana; Desai, Anil Kumar; Joshi, Shamsunder; Gopalakrishnan, K.

    2016-01-01

    Purpose: This study is conducted to investigate the feasibility of echographic imaging of tissue thickness of healthy and reconstructed cleft lip. Design: Prospective study. Materials and Methods: The study was conducted in SDM Craniofacial Unit, Dharwad and was approved by Local Institutional Review Board. A total of 30 patients, age group ranging from 4 to 25 years, of which 15 postoperative unilateral cleft lip constituted the test group. The remaining 15 with no cleft deformities, no gross facial asymmetry, constituted the control group. The thickness of the mucosa, submucosa, muscle and full thickness of the upper lip were measured with the transversal images using ultrasonography at midpoint of philtrum, right and left side philtral ridges and vermillion border, at 1, 3, 6 months interval. Results: There was an increase in muscle thickness at the vermillion border (mean = 6.9 mm) and philtral ridge (5.9 mm). Equal muscle thickness were found between the normal and test group at 6 months follow-up in a relaxed position, which was statistically significant (P = 0.0404). Conclusion: Quantitative assessment of thickness and echo levels of various lip tissues are done with proper echographic calibration. Diagnostic potentials of this method for noninvasive evaluation of cleft lip reconstructions were achieved by this study. PMID:27134448

  7. Causal attributions of cleft lip and palate across cultures.

    Science.gov (United States)

    Mednick, Lauren; Snyder, Julie; Schook, Carolyn; Blood, Emily A; Brown, Shan-Estelle; Weatherley-White, R C A

    2013-11-01

    Objective : To describe and compare the causal beliefs associated with cleft lips and/or palates across several different countries. Design : Cross-sectional survey. Setting : Operation Smile surgery screenings in six developing countries. Participants : Two hundred seventy-nine adult patients and parents of children with cleft lips and/or palates in Kenya, Russia, Cambodia, India, Egypt, and Peru. Interventions : In person interviews were conducted with interpreters. Main Outcome Measure : As part of a larger study, a semistructured questionnaire was created to explore cleft perceptions, belief systems that affect these perceptions, and social reactions to individuals with clefts. Results : Causal attributions were grouped by category (environment, self-blame, supernatural, chance, unknown, or other) and type of locus of control (external, internal, or unknown). Results indicate significant difference by country for both causal attribution category (P culture. As harmful beliefs about cause may continue to impact affected individuals and their families even after a repair, it is insufficient to provide surgical care alone. Care of the entire person must include attempts to change misinformed cultural beliefs through educating the broader community. PMID:23030676

  8. Análise do trato vocal em pacientes com nódulos, fendas e cisto de prega vocal Vocal tract analysis in patients with vocal fold nodules, clefts and cysts

    Directory of Open Access Journals (Sweden)

    Raquel Buzelin Nunes

    2009-04-01

    the frequency of supraglottic vocal tract adjustments in dysphonic women with nodules, clefts and cysts. METHODS: We assessed 31 dysphonic women, with age ranging between 18 and 45 years, with vocal alteration and a diagnosis of nodules, middle-posterior cleft and cyst, and we carried out a summarized evaluation of the sensory-motor and oral systems and the patients were submitted to video-laryngostroboscopy and nasal and laryngeal fibroscopy. Three distinct groups were selected: patients with bilateral nodules, clefts and cysts, with similar glottic configuration. Their vocal tracts were visually analyzed through exams of nasal and laryngeal fibroscopy, by speech and hearing therapists and otorhinolaryngologists, checking the following parameters: supraglottic constriction, larynx vertical mobility, pharyngeal constriction and tongue mobility. The data was statistically described and treated. RESULTS: during visual analysis we did not find statistically significant differences which would separate the glottic alterations groups. CONCLUSION: There was no correlation between supraglottic tract adjustments with any particular type of glottic alteration. These are individual behaviors that generate adjustments and justify the different vocal qualities in patients with the same type of laryngeal alteration.

  9. FOXE1 Association with both Isolated Cleft Lip with or without Cleft Palate; and Isolated Cleft Palate

    DEFF Research Database (Denmark)

    Moreno, Lina M; Mansilla, Maria Adela; Bullard, Steve A;

    2009-01-01

    Nonsyndromic orofacial clefts are a common complex birth defect caused by genetic and environmental factors and/or their interactions. A previous genome-wide linkage scan discovered a novel locus for cleft lip with or without cleft palate (CL/P) at 9q22-q33. To identify the etiologic gene, we......) and rs4460498 (p=6.51E-12) were located inside a 70Kb high LD block containing FOXE1. Association signals for Caucasians and Asians clustered 5' and 3' of FOXE1, respectively. Isolated cleft palate (CP) was also associated indicating that FOXE1 plays a role in two phenotypes thought to be genetically...

  10. Oral Clefts and Academic Performance in Adolescence

    DEFF Research Database (Denmark)

    Clausen, Nicola G; Pedersen, Dorthe A; Pedersen, Jacob K;

    2016-01-01

    OBJECTIVE:   Early life exposure to anesthesia and surgery is suspected to associate with cognitive impairment later in life. We compared academic achievement among adolescents with cleft lip only (CL), cleft palate only (CP), and cleft lip and cleft palate (CLP) with a noncleft control group to...... investigate whether outcome depends on timing and number of operations during childhood and/or type of oral cleft. DESIGN:   Nationwide register-based follow-up study. SETTING:   Danish birth cohort 1986 to 1990. PARTICIPANTS:   Five hundred fifty-eight children with isolated CL (n = 171), CLP (n = 222), or...... CP (n = 195), of which 509 children had been exposed to anesthesia and one or more cleft operation(s), and a 5% sample of the birth cohort (n = 14,677). MAIN OUTCOME MEASURES(S):   Test score in the Danish standardized ninth-grade exam and proportion of nonattainment, defined as "results for ninth...

  11. Cleft Lip and Palate Care in Nigeria

    OpenAIRE

    Adeyemi, Tokunbo Abigail

    2016-01-01

    BACKGROUND; The World Health Organisation has recommended the need to standardise cleft care globally. In Europe, the Eurocleft project was a concerted effort to improve on the standards of care for children with cleft lip and palate. Certain recommendations were made that were used to judge the standards of care offered, this eventually led to reorganization of services. Improving on standards of cleft care in Nigeria, would require a starting point, by determining what is currently being of...

  12. Face facts: Genes, environment, and clefts

    Energy Technology Data Exchange (ETDEWEB)

    Murray, J.C. [Univ. of Iowa, Iowa City IA (United States)

    1995-08-01

    Cleft lip and/or palate provides an ideal, albeit complex, model for the study of human developmental anomalies. Clefting disorders show a mix of well-defined syndromic causes (many with single-gene or environmental etiologies) coupled with their more common presentation in the nonsyndromic form. This summary presents some insight into the genetic causes of, etiology of and animal models for cleft lip and/or palate. 79 refs.

  13. Cleft Lip and Cleft Palate--What to Know and Who Can Help

    Science.gov (United States)

    Apel, Laura

    2008-01-01

    Craniofacial defects such as cleft lip and cleft palate are among the most common of all birth defects in the United States, with one in every 600 newborns affected. Cleft lip and/or palate can occur as an isolated condition or may be one component of an inherited disease or syndrome. Dealing with the condition is an extremely difficult and…

  14. A clinical and demographic profile of the cleft lip and palate in Sub-Himalayan India: A hospital-based study

    Directory of Open Access Journals (Sweden)

    Jyoti Dvivedi

    2012-01-01

    Full Text Available Objectives: To study the cleft lip and cleft palate in the poverty stricken Sub-Himalayan Garhwal region of India, being a commonly seen congenital abnormality and scarcity of studies about the demography of cleft in this region. Design: A prospective cohort observational case series was performed on 4657 cleft patients at a Tertiary care Hospital in Dehradun, India, over a period of 5 years. Outcome measures: The authors investigated the differences between age and sex with cleft status and family history of clefts, birth order, religion, socioeconomic status, parent literacy, source of information for treatment, haematological investigations showing the status of infection and coagulation in such children and satisfaction after treatment. Results: Seventy-two percent parents of cleft lip patients were illiterate, and only 8% were graduates, the majority of patients were from the low socioeconomic class. The siblings of 1.1% of the cleft patients had similar deformity. Anemia was seen in 83.16% cases which was commonly microcytic hypochromic type and eosinophilia was seen in 25.50% of cases. In the coagulation profile, International Nationalized Ratio was found to be raised in 52.12%. Almost 95% of the families were fully satisfied by the treatment and results. Conclusion: This study will provide baseline information on the status of these less privileged cleft patients in this mountainous region for future reference to health workers.

  15. Percutaneous balloon kyphoplasty of osteoporotic vertebral compression fractures with intravertebral cleft

    Directory of Open Access Journals (Sweden)

    Bao Chen

    2014-01-01

    Full Text Available Background: Intravertebral cleft is a structural change in osteoporotic vertebral compression fractures (OVCF, which is the manifestation of ischemic vertebral osteonecrosis complicated with fracture nonunion and pseudoarthrosis and appears in the late stage of OVCF. Despite numerous studies on OVCF, few aim to evaluate the clinicoradiological characteristics and clinical significance of intravertebral cleft in OVCF. This study investigates clinicoradiological characteristics of intravertebral cleft in OVCF and the effect on the efficacy of percutaneous balloon kyphoplasty (PKP. Materials and Methods: PKP was performed on 139 OVCF patients without intravertebral cleft (group A and 44 OVCF patients with intravertebral cleft (group B. The frequency distribution of the affected vertebral body, bone cement infusion volume, imaging manifestation, leakage rate and type, preoperative and postoperative height of the affected vertebral body, visual analog scale (VAS and Oswestry disability index (ODI score were evaluated. Results: Significant differences were found in the frequency distribution of the affected vertebral body and bone cement leakage type between the two groups ( P 0.05 were not detected. In both groups, the postoperative height of the affected vertebral body was significantly improved ( P 0.05. Conclusion: Intravertebral cleft exhibits specific clinical and imaging as well as bone cement formation characteristics. PKP can effectively restore the affected vertebral body height, alleviate pain, and improve daily activity function of patients.

  16. Dental anomalies in children with cleft lip and palate in Western Australia

    Science.gov (United States)

    Nicholls, Wendy

    2016-01-01

    Objective: The purpose of this paper was to describe the prevalence and type of dental anomalies in the primary and permanent dentition in children with a cleft condition at Princess Margaret Hospital in Perth, Western Australia. Materials and Methods: The details of 162 current dental patients extracted from the main dental database through their year of birth for the period 1998–2001 were selected consecutively. Dental records and X-rays were examined by one examiner (WN) and verified by a second examiner (RB) to determine dental development. The mean age of the subjects was 10.8 years with equal numbers of males and females. Subjects were further divided into cleft type; unilateral cleft lip (UCL) and palate, bilateral cleft lip (BCL) and palate, UCL, BCL, and cleft palate. Results: One hundred sixty-two subjects were grouped into 21 categories of anomaly or abnormality. Prevalence rates for the categories were calculated for the overall group and for gender and cleft type. Conclusion: Overall, 94% of patients were found to have at least one dental anomaly, with fifty-six (34%) patients having more than one anomaly or abnormality. PMID:27095907

  17. Evaluation of the lesions of synovial-lined clefts in the Hoffa's infrapatella fat pad with MR imaging

    International Nuclear Information System (INIS)

    Within Hoffa's infrapatellar fat pad there are two synovial clefts, horizontal and vertical, which communicate with the intra-articular space. Intra-articular lesions can also occur in these clefts, and are often difficult to differentiate from extra-articular lesions. The purpose of this study is to evaluate, using MR imaging, the lesions occurring in these synovial lined clefts, as well as associated abnormalities. Thirty-one knees of 31 patients with lesions in horizontal and vertical clefts in Hoffa's infrapatellar fat pad were retrospectively evaluated. Using a 1.5T MR imager, axial, sagittal, and coronal MR images of knees were obtained. Lesions in clefts, degree of joint effusion and associated knee abnormalities were analyzed. Horizontal cleft lesions were noted in 21 cases; there were 17 cystic dilatations, two loose bodies, one synovial chondroma, and one case of pigmented villonodular synovitis(PVNS). Vertical cleft lesions were noted in 15 cases; these comprised 11 cystic dilatations, two loose bodies, one synovial osteochondromatosis, and one PVNS. Among all cases, three cystic dilatations, one loose body, and one PVNS occurred in both horizontal and vertical clefts. Among 25 knees with cystic dilatations of clefts, five showed grade 1, joint effusion, ten grade 2, and ten grade 3, Associated abnormalities were meniscal tear in 16 cases, osteoarthritis in 13, cruciate ligament tear in five, osteochondritis dissecans in three, osteochondral fracture in two, osteonecrosis in one, loose body in one, and synovitis in one. Among synovial-lined clefts in Hoffa's fat pad, the most common lesion was cystic dilatation;there were various associated abnormalities and a close relationship to joint effusion. An awareness of the types of lesions found in clefts is helpful for narrowing the differential diagnosis of lesions occurring in the area of Hoffa's fat pad.=20

  18. Disease: H00638 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00638 Ectrodactyly-ectodermal dysplasia cleft-palate syndrome (EEC syndrome) Ectro...dactyly-ectodermal dysplasia-cleft lip/palate (EEC) syndrome is a rare autosomal dominant genetic disorder. ...todermal dysplasia affecting skin, hair, and nails, and cleft lip with or without cleft... Costa VD, Verde RB, Barros DS Do you know this syndrome? Ectrodactyly - ectodermal dysplasia - cleft lip/pa...n MM Ectrodactyly, ectodermal dysplasia and cleft palate (EEC syndrome). Report o

  19. Hypertrophic scarring in cleft lip repair: a comparison of incidence among ethnic groups

    Directory of Open Access Journals (Sweden)

    Soltani AM

    2012-07-01

    Full Text Available Ali M Soltani, Cameron S Francis, Arash Motamed, Ashley L Karatsonyi, Jeffrey A Hammoudeh, Pedro A Sanchez-Lara, John F Reinisch, Mark M UrataDivision of Plastic and Maxillofacial Surgery at Children's Hospital Los Angeles, CA, USA; The Division of Plastic and Reconstructive Surgery at the Keck School of Medicine of the University of Southern California, Los Angeles, CA, USABackground: Although hypertrophic scar (HTS formation following cleft lip repair is relatively common, published rates vary widely, from 1% to nearly 50%. The risk factors associated with HTS formation in cleft patients are not well characterized. The primary aim of this retrospective study of 180 cleft lip repairs is to evaluate the frequency of postoperative HTS among various ethnic groups following cleft lip repair.Methods: A retrospective chart view of patients undergoing primary cleft lip repair over a 16-year period (1990–2005 by the senior surgeon was performed. The primary outcome was the presence of HTS at 1 year postoperatively. Bivariate analysis and multivariable logistic regression were used to evaluate potential risk factors for HTS, including ethnicity, type and laterality of cleft, and gender.Results: One hundred and eighty patients who underwent cleft lip repair were included in the study. The overall rate of postoperative HTS formation was 25%. Ethnicity alone was found to be an independent predictor of HTS formation. Caucasian patients had the lowest rate of HTS formation (11.8% and were used as the reference group. HTS rates were significantly higher in the other ethnicities, 32.2% in Hispanic patients (odds ratio [OR]: 3.51; 95% confidence interval [CI]: 1.53–8.85, and 36.3% for Asian patients (OR 4.27; 95% CI: 1.36–13.70. Sex, cleft type, and cleft laterality were not associated with increased rates of HTS.Conclusions: Differences in ethnic makeup of respective patient populations may be a major factor influencing the wide variability of reported

  20. Presurgical nasoalveolar molding in unilateral cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Rahul J Hegde

    2015-01-01

    Full Text Available Unilateral cleft lip and palate (UCLP patients have an esthetic and functional compromise of the middle third of the face and nasal structures. To improve the esthetic result of lip repair, the concept of presurgical nasoalveolar molding (PNAM was brought into conception. PNAM is an easy and passive method of bringing the alveolus and lips together by redirecting the forces of natural growth. This case report documents a 2-year follow-up of PNAM in UCLP.

  1. Presurgical nasoalveolar molding in unilateral cleft lip and palate

    Science.gov (United States)

    Hegde, Rahul J.; Kharkar, Viraj R.; Kamath, Shamika

    2015-01-01

    Unilateral cleft lip and palate (UCLP) patients have an esthetic and functional compromise of the middle third of the face and nasal structures. To improve the esthetic result of lip repair, the concept of presurgical nasoalveolar molding (PNAM) was brought into conception. PNAM is an easy and passive method of bringing the alveolus and lips together by redirecting the forces of natural growth. This case report documents a 2-year follow-up of PNAM in UCLP. PMID:26681868

  2. The trochlear cleft: the ''black line'' of the trochlear trough

    Energy Technology Data Exchange (ETDEWEB)

    Wissman, Robert D.; Nepute, Joshua; Fischer, Nathaniel von; Radhakrishnan, Rupa; Hendry, Daniel [University of Cincinnati Medical Center, Department of Radiology, Cincinnati, OH (United States); Ingalls, Jerrell [Desert Radiologists, Las Vegas, NV (United States); Kenter, Keith [University of Cincinnati Medical Center, Department of Orthopaedic Surgery, Cincinnati, OH (United States)

    2012-09-15

    The ''cartilage black line sign'' is a recently described T2 dark cartilage lesion that we have identified appearing as a cleft in the trochlear trough. The purpose of our study was to define the MR imaging characteristics of a trochlear cleft, determine its incidence, and correlate the MR findings with arthroscopy. A total of 1,300 consecutive MR examinations of the knee were retrospectively reviewed by consensus of two fellowship-trained musculoskeletal radiologists. The MR imaging characteristics and location of a trochlear cleft were determined. Imaging results were compared to arthroscopy when available. Patient age and gender were compared to 25 randomly selected control patients without trochlear clefts. A total of 25 (1.9%) individuals (11 females and 14 males; age range 19-45 years; mean age 28 years) were diagnosed with a trochlear cleft. The control group consisted of 11 females and 14 males; age range 19-83 years; mean age 46 years. Mean cleft length was 7 mm (range 6-12 mm); cleft location was consistently in the lower trochlear trough. No full-thickness cartilage defects were identified in the eight individuals in whom arthroscopic correlation was available. A grade 2 cartilage lesion was identified in a single individual; another progressed from grade 0 to a full-thickness trochlear lesion over an 8-month interval. Eight individuals were athletes. No significant difference in gender was noted between the two groups, however, the study group was significantly younger p < 0.0001. A trochlear cleft is a rare finding in young active individuals. It most likely indicates an incomplete cartilage fissure which may rarely progress to a full-thickness defect. (orig.)

  3. Vitamin A and clefting: putative biological mechanisms

    NARCIS (Netherlands)

    Ackermans, M.M.; Zhou, J.H.; Carels, C.E.L.; Wagener, F.A.D.T.G.; Hoff, J.W. Von den

    2011-01-01

    Nutritional factors such as vitamin intake contribute to the etiology of cleft palate. Vitamin A is a regulator of embryonic development. Excess vitamin A can cause congenital malformations such as spina bifida and cleft palate. Therefore, preventive nutritional strategies are required. This review

  4. Cleft Lip and Palate (For Parents)

    Science.gov (United States)

    ... with your doctor about the treatment plan. Surgical Treatment A cleft lip is usually surgically repaired in the hospital using general anesthesia when a child is 3 to 6 months old. If the cleft lip is wide, special procedures like lip adhesion or a molding plate device might help bring ...

  5. Maternal genes and facial clefts in offspring

    DEFF Research Database (Denmark)

    Jugessur, Astanand; Shi, Min; Gjessing, Håkon Kristian;

    2010-01-01

    -parent triads) and Denmark (235 case-parent triads). We analyzed isolated cleft lip with or without cleft palate (iCL/P) and isolated cleft palate only (iCP) separately and assessed replication by looking for genes detected in both populations by both methods. In iCL/P, neither TRIMM nor HAPLIN detected more......BACKGROUND: Fetal conditions can in principle be affected by the mother's genotype working through the prenatal environment. METHODOLOGY/PRINCIPAL FINDINGS: Genotypes for 1536 SNPs in 357 cleft candidate genes were available from a previous analysis in which we focused on fetal gene effects [1...... associated with iCP in TRIMM analyses of the Norwegian triads. CONCLUSION/SIGNIFICANCE: Except for FLNB, HIC1 and ZNF189, maternal genes did not appear to influence the risk of clefting in our data. This is consistent with recent epidemiological findings showing no apparent difference between mother...

  6. Invariant properties of representations under cleft extensions

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    The main aim of this paper is to give the invariant properties of representations of algebras under cleft extensions over a semisimple Hopf algebra. Firstly, we explain the concept of the cleft extension and give a relation between the cleft extension and the crossed product which is the approach we depend upon. Then, by making use of them, we prove that over an algebraically closed field k, for a finite dimensional Hopf algebra H which is semisimple as well as its dual H*, the representation type of an algebra is an invariant property under a finite dimensional H-cleft extension . In the other part, we still show that over an arbitrary field k, the Nakayama property of a k-algebra is also an invariant property under an H -cleft extension when the radical of the algebra is H-stable.

  7. Cleft deformities in adults and children aged over six years in Nigeria: Reasons for late presentation and management challenges

    Directory of Open Access Journals (Sweden)

    Wasiu L Adeyemo

    2009-11-01

    Full Text Available Wasiu L Adeyemo1, Mobolanle O Ogunlewe1, Ibironke Desalu2, Akinola L Ladeinde1, Bolaji O Mofikoya3, Michael O Adeyemi4, Adegbenga A Adepoju4, Olufemi O Hassan41Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, 2Department of Anaesthesia, 3Department of Surgery, Faculty of Clinical Sciences, College of Medicine, University of Lagos, Lagos, Nigeria; 4Department of Oral and Maxillofacial Surgery, Lagos University Teaching Hospital, Lagos, NigeriaAbstract: In developing countries, untreated cleft lips and palates are found with increasing frequency and patients often present to the surgeon far past the optimal time for closure of the cleft deformities. A prospective study was conducted between March 2007 and September 2009, to identify the reasons and treatment challenges of delayed presentation of cleft lip and palate deformities at the Lagos University Teaching Hospital, Nigeria. Out of a total of 150 patients with cleft defects during the period, 43 (28.7% were adults and children aged over six years. The mean age of these patients at the time of presentation was 17.3 years. The most common reasons for late presentation were lack of money (56.7%, lack of health care services nearby (18.4%, and lack of awareness of treatment availability (13.3%. Common challenges in these patients included surgical, orthodontic, speech, anesthetic, and psychological. Although adult clefts were significantly enlarged in three dimensions the anatomic landmarks were easier to discern than in an infant. However, extensive soft tissue dissection in adult cleft lip repair resulted in significant postoperative edema. Closure of wide palatal cleft often required the use of adjunct intraoral flaps. Despite late presentation, surgical outcome of these patients was satisfactory and comparable to cleft repair in infants.Keywords: cleft deformities, adults, adolescents, late presentation, management, challenges

  8. Early Orthopedic Retraction of the Premaxilla in Bilateral Complete Cleft Lip and Palate: An Innovative Approach to a Difficult Problem.

    Science.gov (United States)

    Mahmood, Rizwan; Flood, Tim; Robinson, Stephen; Al-Gholmy, Mohammed

    2016-05-01

    Bilateral cleft lip and palate occurs in 9.2% of cleft patients. Many approaches have been adopted to manage the protrusive premaxillary segment in patients with bilateral cleft lip and palate. Some advocate the use of intraoral appliances, occasionally combined with invasive surgery, which often requires revision at a later date. The authors describe the case of a 3-year-old child born with bilateral cleft lip and palate presenting with a protuberant premaxilla and an overjet greater than 25 mm. Prompt intervention was warranted in this case due to the potential for traumatic compromise to the dentition of the premaxillary segment and a distinct lack of social integration reported by the parents. The patient was managed with a novel, innovative approach using orthodontic traction and minimally invasive surgery. The literature has been reviewed and the patient's subsequent physiological and psychosocial development has been monitored. He has since undergone successful alveolar bone grafting. PMID:25647517

  9. X-linked genes and risk of orofacial clefts

    DEFF Research Database (Denmark)

    Jugessur, Astanand; Skare, Øivind; Lie, Rolv T; Wilcox, Allen J; Christensen, Kaare; Christiansen, Lene; Nguyen, Truc Trung; Murray, Jeff; Gjessing, Håkon K

    2012-01-01

    Orofacial clefts are common birth defects of complex etiology, with an excess of males among babies with cleft lip and palate, and an excess of females among those with cleft palate only. Although genes on the X chromosome have been implicated in clefting, there has been no association analysis o...

  10. Velopharyngeal sphincter pathophysiologic aspects in the in cleft palat

    Directory of Open Access Journals (Sweden)

    Collares, Marcus Vinicius Martins

    2008-09-01

    Full Text Available Introduction: Cleft lip and palate are common congenital abnormalities with typical functional disorders on speech, deglutition and middle ear function. Objective: This article reviews functional labiopalatine disorders through a pathophysiological view. Method: We performed a literature search on line, as well as books and periodicals related to velopharyngeal sphincter. Our sources were LILACS, MEDLINE and SciELO databases, and we applied to the research Keywords of interest on the velopharyngeal pathophysiology, for articles published between 1965 and 2007. Conclusion: Velopharyngeal sphincter plays a central role in speech, swallowing and middle ear physiology in patients with labiopalatine cleft. At the end of our bibliographic review, pursuant to the velopharyngeal physiology in individuals with this disorder in the functional speech, deglutition and otologic function, we observed that although there is a great number of published data discussing this issue, further studies are necessary to completely understand the pathophysiology, due to the fact they have been exploited superficially.

  11. Parents' age and the risk of oral clefts

    DEFF Research Database (Denmark)

    Bille, C.; Skytthe, A.; Vach, W.;

    2005-01-01

    BACKGROUND: Some malformations are clearly associated with older maternal age, but the effect of older age of the father is less certain. The aim of this study is to determine the degree to which maternal age and paternal age independently influence the risk of having a child with oral clefts....... METHODS: Among the 1,489,014 live births in Denmark during 1973-1996, there were 1920 children with nonsyndromic cleft lip with or without cleft palate and 956 children with nonsyndromic cleft palate. We used logistic regression to assess the impact of parental age on the occurrence of cleft lip with or...... without cleft palate and cleft palate. Interaction between mother's and father's age was included in the analysis. RESULTS: Separate analyses of mother's and father's age showed that older age was associated with increased risk of both cleft lip with or without cleft palate and cleft palate only. In a...

  12. Congenital orofacial clefts: Etiology and Frequency

    Directory of Open Access Journals (Sweden)

    Kamil Serkan Ağaçayak

    2014-06-01

    Full Text Available Orofacial clefts are congenital structural anomalies of the lip and/or palate. These anomalies affect ~1/1000 in the community. The etiology of orofacial clefts is complex, including various genetic and environmental agents. Syndrome is not accompanied by any isolated orofacial clefts are more common, although environmental factors often play a role in the etiology. Mendelian or teratogenic origins; the non-syndromic forms of orofacial clefts are more common and are likely due to secondary gene–environment interactions. Latest researches in both molecular and quantitative approaches have begun to identify the genes responsible for the rare syndromic forms of cleft and have also identified both candidate genes and loci for the more common and complex non-syndromic variants. Animal models, have also contributed greatly to an comprehension of these anomalies. We aimed to describes genes that are involved in orofacial clefts in humans and animal models and explores genetic approaches to identifying additional genes and gene–environment interactions that constitute the many factors of orofacial clefts in this review.

  13. Association of generalized aggressive periodontitis and ectrodactyly-ectodermal dysplasia-cleft syndrome

    Directory of Open Access Journals (Sweden)

    Rosamma Joseph

    2012-01-01

    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. Even though literature has documented the association of various genetic disorders with aggressive periodontitis, the periodontal manifestations in patients with EEC syndrome have never been addressed. This case report presents the periodontal status of three patients in a family with EEC syndrome. The presence of generalized aggressive periodontitis was noticed in these patients. EEC syndrome could be a new addition to the group of genetic disorders associated with aggressive periodontitis.

  14. 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. PMID:25737931

  15. An update on the aetiology of orofacial clefts

    OpenAIRE

    Wong, FK; Hägg, U

    2004-01-01

    Objective. To review recent data on the aetiology of cleft lip and palate. Data sources. MEDLINE literature search (1986-2003). Study selection. Literature and data on aetiology of cleft lip and palate using the following key words: 'cleft lip', 'cleft palate', 'aetiology', and 'genetics'. Data extraction. Relevant information and data were reviewed by the authors. Data synthesis. Cleft lip and palate is one of the most common types of congenital malformation. The aetiology seems complex, but...

  16. Short mandible - a possible risk factor for cleft palate with/without a cleft lip

    DEFF Research Database (Denmark)

    Hermann, Nuno Vibe; Darvann, Tron Andre; Ersbøll, Bjarne Kjær;

    2014-01-01

    Structured Abstract Objectives To estimate the influence of a short mandible on the risk of developing a cleft palate with/without a cleft lip (CP). Setting and sample population The retrospective sample consisted of 115 2-month-old Danish infants with CP, and 70 control infants with unilateral...... incomplete cleft lip (UICL). Material and Methods Cephalometric X-rays were obtained. Mandibular length (L-m) was measured and corrected for body length (L-b) to remove influence of varying body length in the sample. Logistic regression was applied to the corrected mandibular length (L-mc) to calculate the...... risk of having a cleft palate. Results The mean mandibular length in the group with CP was about 4mm shorter than in the control group. Odds ratio (OR) was calculated to be 0.58 (95% confidence interval 0.48-0.68), implying that an individual's risk of cleft palate with/without a cleft lip increases...

  17. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip–Cleft Palate

    OpenAIRE

    Lovya George; Sunil K Jain

    2015-01-01

    Preterm infants (PIs) often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require co...

  18. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip–Cleft Palate

    Science.gov (United States)

    George, Lovya; Jain, Sunil K.

    2015-01-01

    Preterm infants (PIs) often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require continued intubation and mechanical ventilation. We are presenting the first case report of a PI with cleft lip and palate who was managed by biphasic nasal continuous positive airway pressure. PMID:26495158

  19. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip–Cleft Palate

    Directory of Open Access Journals (Sweden)

    Lovya George

    2015-10-01

    Full Text Available Preterm infants (PIs often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require continued intubation and mechanical ventilation. We are presenting the first case report of a PI with cleft lip and palate who was managed by biphasic nasal continuous positive airway pressure.

  20. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip-Cleft Palate.

    Science.gov (United States)

    George, Lovya; Jain, Sunil K

    2015-10-01

    Preterm infants (PIs) often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require continued intubation and mechanical ventilation. We are presenting the first case report of a PI with cleft lip and palate who was managed by biphasic nasal continuous positive airway pressure. PMID:26495158

  1. The effect of infant orthopedics on the occlusion of the deciduous dentition in children with complete unilateral cleft lip and palate (Dutchcleft).

    NARCIS (Netherlands)

    Bongaarts, C.A.M.; Kuijpers-Jagtman, A.M.; Hof, M.A. van 't; Prahl-Andersen, B.

    2004-01-01

    OBJECTIVE: Evaluation of the effect of infant orthopedics (IO) on the occlusion of the deciduous dentition in patients with unilateral cleft lip and palate (UCLP). DESIGN: Prospective, two-arm, randomized, controlled clinical trial with three participating cleft palate centers (Dutchcleft). SETTING:

  2. MAXILLARY GROWTH PATTERNS IN ROMANIAN CHILDREN WITH CLEFT PALATE DURING THE FIRST 6 YEARS OF LIFE

    Directory of Open Access Journals (Sweden)

    Liliana-Gabriela Halitchi

    2012-12-01

    Full Text Available In order to visualize, evaluate and measure the maxillary growth in patients with cleft palate and to identify the reactive morphological pattern that could be identified from the dimensional changes produced along the first six years of life, maxillary impressions were made in two groups of study, by means of standard trays and silicone impression materials. The maxillary casts resulted had been 3D scanned at Multinr, in Sf. Gheorghe. The study was carried on a number of 34 patients with cleft palate, 16 boys and 18 girls, with ages between 2 months and 6 years, operated by the same surgical team in “St. Mary” University Children Hospital of Iaşi, as well as on 60 normal children. Bivariate Student’s t test established statistically significant negative differences between the mean values of the anterior and posterior width of the maxillary alveolar arch, positive non significant differences for the length and positive differences for the depth of the dehiscent palate in the experimental group, comparatively with normal children. Cleft palate patients have narrowed and shorter maxillary alveolar arch and flattened palate. At least therapeutically, the cleft palate group, operated at different ages, from 2 to 4 years, could not react like other groups of study from important European Cleft Centers and benefit from a good residual growth.

  3. Hidrosefali Ile Ilişkili Ektrodakli, Ektodermal Displazi, Yarık Dudak Ve Damak Sendromulu Bir Vakanın Değerlendirmesi

    OpenAIRE

    Aladağ, Buket Uysal; Yılmaz, Fatma Hilal; Koçak, Nadir; Annagür, Ali

    2013-01-01

    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...

  4. Congenital hypothyroidism, spiky hair, and cleft palate.

    OpenAIRE

    Bamforth, J S; Hughes, I A; Lazarus, J. H.; Weaver, C. M.; P.S. Harper

    1989-01-01

    Two brothers are described with athyroidal hypothyroidism, spiky hair, choanal atresia, cleft palate, and bifid epiglottis. Polyhydramnios was present in the third trimester of each pregnancy. These abnormalities appear to represent a new syndrome.

  5. Current concepts in genetics of nonsyndromic clefts

    Directory of Open Access Journals (Sweden)

    Murthy Jyotsna

    2009-01-01

    Full Text Available Nonsyndromic cleft lip and palate is a complex genetic disorder with variable phenotype, largely attributed to the interactions of the environment and multiple genes, each potentially having certain effects. Numerous genes have been reported in studies demonstrating associations and/or linkage of the cleft lip and palate phenotypes to alleles of microsatellite markers and single nucleotide polymorphisms within specific genes that regulate transcription factors, growth factors, cell signalling and detoxification metabolisms. Although the studies reporting these observations are compelling, most of them lack statistical power. This review compiles the evidence that supports linkage and associations to the various genetic loci and candidate genes. Whereas significant progress has been made in the field of cleft lip and palate genetics in the past decade, the role of the genes and genetic variations within the numerous candidate genes that have been found to associate with the expression of the orofacial cleft phenotype remain to be determined.

  6. Technique in Cleft Rhinoplasty: The Foundation Graft.

    Science.gov (United States)

    Gassner, Holger G; Schwan, Franziska; Haubner, Frank; Suárez, Gustavo A; Vielsmeier, Veronika

    2016-04-01

    Secondary cleft rhinoplasty represents a particular surgical challenge. The authors have identified the deficit in skeletal projection of the cleft-sided piriform rim as an important contributor to the pathology. A graft is described to augment the piriform crest on the cleft side. This foundation graft is suture fixated to the piriform crest after complete release of all soft tissue attachments to the alar base. The foundation graft is articulated with a long alar strut graft, which allows for powerful projection of the cleft-sided nasal tip. An advancement flap of vestibular skin is described to correct the vestibular stenosis. A transplant of diced cartilage in fascia is added to augment maxillary soft tissue volume. Subjective and objective measures of form and function are presented in a retrospective series of five cases, illustrating the efficacy of the techniques described. PMID:27097143

  7. Effect of cleft palate repair on the susceptibility to contraction-induced injury of single permeabilized muscle fibers from congenitally-clefted goat palates.

    Science.gov (United States)

    Despite cleft palate repair, velopharyngeal competence is not achieved in ~ 15% of patients, often necessitating secondary surgical correction. Velopharyngeal competence postrepair may require the conversion of levator veli palatini muscle fibers from injury-susceptible type 2 fibers to injury-resi...

  8. [Laser navigation guided cleft lip repair].

    Science.gov (United States)

    Bing, Shi

    2016-06-01

    A new method using the ideal mid-facial line as the navigating reference was introduced to improve the outcome of cleft lip repair. Using the verticle coordinate crossing the middle point of the intercanthus line, surgeons could observe and correct the distortion of the fine structures in labial-nasal area. This laser projecting mid-facial-line navigation was repeatable, while not interfere the operating. In conclusion, generalizing laser navigation is a valuable supplementary for cleft lip repair. PMID:27526442

  9. Study of cleft lip and palate deformities among the residents of the Semipalatinsk nuclear test site area in Kazakhstan

    International Nuclear Information System (INIS)

    The aim was to investigate the association between long-term radiation exposure and the high prevalence of cleft lip and palate anomalies among the residents from exposed areas and to compare to non-exposed areas. A retrospective study of 716 case reports was carried out on cleft lip and palate deformities patients (1978-1998). The case reports were screened and studied for frequency of cleft lip and palate by gender and number of patients, including epidemiological studies of cleft lip and palate anomalies cases in 1000 newborns in the three zones of radiation risk where the hospitalized patients resided. The statistical analyses of the retrospective study of cleft lip and palate patients were estimated by X2-test and performed with the Stat View 5.0 statistical analysis program. 5,10 cases of cleft lip and palate patients per 1000 live births were calculated in the zone of maximum radiation risk, which is extremely high, and 2,30 cases of the anomalies per 1000 among the newborns in the zone of heightened radiation risk and both were significantly higher than those in the zone of minimum risk. The incidence varied in different years, from 5,66 per 1000 live births in 1978-1988 (at the time of nuclear testing) to 4,14 per 1000 live births in 1990-1998 (after the nuclear testing was stopped) in the area of maximum radiation risk and showed that the number of cleft lip and palate anomalies cases was significantly higher in both periods of time compare to the zones of heightened and minimum radiation risk. This study suggests that the high prevalence cleft lip and palate anomalies among the newborns from the exposed areas was due to the long-term radiation exposure.

  10. Three-Dimensional Assessment of Early Surgical Outcome in Repaired Unilateral Cleft Lip and Palate: Part 1. Nasal changes.

    LENUS (Irish Health Repository)

    Ayoub, Ashraf

    2010-08-11

    Abstract Objective: To evaluate 3D nasal morphology following primary reconstruction in children with unilateral cleft lip and palate relative to contemporaneous non-cleft data Design: Prospective, cross-sectional, controlled study. Setting: Glasgow Dental Hospital & School, Faculty of Medicine, Glasgow University Patients and Participants: Two groups of 3-year old children (21 with unilateral cleft lip and palate and 96 controls) with facial images taken using a 3D vision based capture technique. Methods: 3D images of the face were reflected so the cleft was on the left side to create a homogenous group for statistical analysis. Three-dimensional co-ordinates of anthropometric landmarks were extracted from facial images by a single operator. A set of linear measurements was utilised to compare cleft and control subjects on right and left sides, adjusting for sex differences Results: the mean nasal base width and the width of the nostril floor on right and left sides differed significantly between control and Unilateral Cleft Lip and Palate (UCLP) groups. The measurements were greater in UCLP children. The difference in the mean nasal height and mean nasal projection between the groups were not statistically significant. Mean columellar lengths were different between the left and right sides in UCLP cases. Conclusions: There were significant nasal deformities following the surgical repair of UCLP. Keywords: child, cleft lip and palate, three-dimensional imaging.

  11. Retrospective subjective evaluation of aesthetic outcome in secondary cleft lip deformities operated with Abbe's flap

    Directory of Open Access Journals (Sweden)

    Kamal M. Kumar

    2015-01-01

    Full Text Available Background: The Abbe's flap is most commonly used to repair the full thickness defects of the lip that do not involve the commissure. The Abbe flap was first introduced to correct the secondary deformity of bilateral cleft lip. By this pedicled flap, we are able to equalize the disparity which existed between the tight upper lip and excessive lower lip. Aims to study retrospective subjective evaluation of aesthetic outcome in secondary cleft lip deformities operated with Abbe's flap. Methods: 29 patients operated during the period of January 2007 - December 2011 for correction of secondary cleft lip and nasal deformity with Abbe's flap with or without rhinoplasty were included in our retrospective study. The secondary corrective surgeries were performed by a single surgeon. The photographs of the patients were retrospectively assessed by two plastic surgeons and one oral and maxillofacial surgeon, other than the one who performed the surgery. 29 patients operated during the period of January 2007 - December 2011 for correction of secondary cleft lip and nasal deformity with Abbe's flap with or without rhinoplasty were included in our retrospective study. The secondary corrective surgeries were performed by a single surgeon. The photographs of the patients were retrospectively assessed by two plastic surgeons and one oral and maxillofacial surgeon, other than the one who performed the surgery. Results: Out of 29 patients with secondary cleft deformities 12 were male and 17 were female. Average follow up period was 3 years. Multiple variables of the nose and the lips were used to assess the aesthetic outcome of Abbe's flap. Conclusion: As per the subjective retrospective analysis of our study we have come to the conclusion that Abbe's flap is a work horse for correction of shortage of tissue for the secondary cleft lip deformities. Uniform satisfactory outcome was obtained with a more natural contour and more satisfactory proportion of both lips

  12. An Extraoral Nasoalveolar Molding Technique in Complete Unilateral Cleft Lip and Palate

    Directory of Open Access Journals (Sweden)

    Qi Wang, PhD, DDS

    2013-07-01

    Full Text Available Summary: Although nasoalveolar molding is commonly performed before cleft lip surgery, customized palatal plate availability is limited for patients far from a hospital. This case report describes a preformed extraoral nasoalveolar molding (PENAM appliance and treatment approach for presurgical nasoalveolar molding in newborns with complete unilateral cleft lip and palate. A 12-day-old boy presented with complete unilateral cleft lip and palate. The PENAM device was supported by an adhesive-taped upper lip, which consisted of a lip nasal stent made from a 0.5-mm stainless steel wire. The spring was activated monthly. The shape of the cartilaginous septum, alar cartilage tip, medial crus, lateral crus, and alveolar segments was molded to resemble the normal shape of these structures. The 9.3-mm alveolar gaps were reduced and approximated. The approximation mostly came from the major alveolus segment with approximately 6.4-mm movement. Cleft side nostril height increased 5.5 mm and deviation of the columella was corrected by 42°. PENAM can be helpful in infants with unilateral cleft lip and palate because it has benefits for long-term forced delivery, requires less frequent activations, and is suitable for patients who live far from a hospital.

  13. Dental and maxillofacial characteristics of six Japanese individuals with ectrodactyly-ectodermal dysplasia-clefting syndrome.

    Science.gov (United States)

    Okamura, Erika; Suda, Naoto; Baba, Yoshiyuki; Fukuoka, Hiroki; Ogawa, Takuya; Ohkuma, Mizue; Ahiko, Nozomi; Yasue, Akihiro; Tengan, Toshimoto; Shiga, Momotoshi; Tsuji, Michiko; Moriyama, Keiji

    2013-03-01

    Objective : Ectrodactyly-ectodermal dysplasia-clefting syndrome is a congenital anomaly characterized by ectodermal dysplasia, ectrodactyly, cleft lip and palate, and lacrimal duct anomalies. Because this syndrome is frequently accompanied by a congenital lack of teeth, narrow palate, and malocclusion, comprehensive orthodontic intervention is required. Design : To highlight the specific dental and maxillofacial characteristics of ectrodactyly-ectodermal dysplasia-clefting syndrome, six Japanese individuals diagnosed with the syndrome are described here. Patients : The subjects consisted of two boys and four girls (age range, 6.0 to 13.9 years) diagnosed with ectrodactyly-ectodermal dysplasia-clefting syndrome by medical and dental specialists. Their conditions included ectodermal dysplasia (hypodontia, microdontia, enamel hypoplasia, and abnormalities in hair and nails), cleft lip and/or palate, and ectrodactyly. Cephalograms, panoramic x-rays, and dental casts were taken; systemic complications were recorded at the first visit to our dental hospital. Results : All individuals had severe oligodontia with 9 to 18 missing teeth. The missing teeth were mainly maxillary and mandibular incisors and second bicuspids, arranged in a symmetrical manner. Cephalometric analysis showed retruded and short maxilla due to cleft lip and/or palate. It is interesting that all individuals showed a characteristically shaped mandibular symphysis with a retruded point B. It is likely that this unusual symphyseal morphology is due to the lack of mandibular incisors. Conclusions : This study demonstrates the presence of severe oligodontia in the incisal and premolar regions and describes a characteristic maxillary and mandibular structure in Japanese individuals with ectrodactyly-ectodermal dysplasia-clefting syndrome. PMID:22236363

  14. MAXILLARY GROWTH PATTERNS IN ROMANIAN CHILDREN WITH CLEFT PALATE AGED BETWEEN 0-6 YEARS

    Directory of Open Access Journals (Sweden)

    Liliana-Gabriela Halitchi

    2011-12-01

    Full Text Available A study documenting spontaneous and coordinated growth changes in young Romanian children with cleft palate is developed, for identifying the functional adaptations of oral cavity to the disrupted architecture of palate in children with cleft palate, the 3D analysis of maxillary casts being applied to visualize, evaluate and measure maxillary growth in patients with cleft palate, as well as to identify the reactive morphological pattern that could be imagined from the dimensional changes produced in the first 6 years of life. Two experimental groups were involved in the study, a former one, including 44 patients with cleft palate (16 boys and 28 girls, aged between 2 months and 6 years, operated by the same surgical team in the St.Mary University Children Hospital from Ia[i. The resulting maxillary casts were 3D scanned at Multinr company, in Sf. Gheorghe. The maxillary alveolar arch lines, the 4 Stilmann points (TD, TS, CD, CS and the maxillary interincisive point were identified and marked by the orthodontist, being reproduced with 6 degrees of tridimensional freedom. The coordinates of this point were transferred to the CAD system, to graphically recreate the computerized three-dimensional alveolar arch. 4 direct measurements have been made, namely: anterior and posterior diameter of the alveolar maxillary arch, length of the alveolar arch and depth of the palate. Student’s t test, both simple and bivariate, performed with SPSS 13.0 soft, showed statistic significance for the dimensional differences of the alveolar maxillary arch in children with cleft palate, comparatively with the healthy control group. Conclusion: Cleft palate patients have narrower and shorter maxillary alveolar arch and a flattened palate.

  15. White Roll Vermilion turn down flap in primary unilateral cleft lip repair: A novel approach

    Directory of Open Access Journals (Sweden)

    R K Mishra

    2015-01-01

    Full Text Available Aim: Numerous modifications of Millard′s technique of rotation - advancement repair have been described in literature. This article envisions a new modification in Millard′s technique of primary unilateral chieloplasty. Material and Methods: Eliminating or reducing the secondary deformities in children with cleft lip has been a motivating factor for the continual refinement of cleft lip surgical techniques through the years. Vermilion notching, visibility of paramedian scars and scar contracture along the white roll are quite noticeable in close-up view even in good repairs. Any scar is less noticeable if it is in midline or along the lines of embryological closure. White Roll Vermilion turn down Flap (WRV Flap, a modification in the Millard′s repair is an attempt to prevent these secondary deformities during the primary cleft lip sugery. This entails the use of white roll and the vermilion from the lateral lip segment for augmenting the medial lip vermilion with the final scar in midline at the vermilion. Result: With an experience of more than 100 cases of primary cleft lip repair with this technique, we have achieved a good symmetry and peaking of cupid′s bow with no vermilion notching of the lips. Conclusion: WRV flap aims to high light the importance of achieving a near normal look of the cleft patient with the only drawback of associated learning curve with this technique.

  16. Haploinsufficiency of MEIS2 is associated with orofacial clefting and learning disability.

    Science.gov (United States)

    Johansson, Stefan; Berland, Siren; Gradek, Gyri Aasland; Bongers, Ernie; de Leeuw, Nicole; Pfundt, Rolph; Fannemel, Madeleine; Rødningen, Olaug; Brendehaug, Atle; Haukanes, Bjørn Ivar; Hovland, Randi; Helland, Gunnar; Houge, Gunnar

    2014-07-01

    MEIS2 is a homeodomain-containing transcription factor of the TALE superfamily that has been proven important for development. We confirm and extend a recent single clinical report stating that deletions in MEIS2 can cause cleft palate [Crowley et al. (2010); Am J Med Genet 152A:1326-1327]. Here we report on five additional patients with 15q14 deletions of sizes 0.6, 0.6, 1.0, 1.9, and 4.8 Mb, respectively, all involving MEIS2. In addition, we present a family with four affected individuals and an intragenic 58 kb direct duplication disrupting MEIS2. In total, 7/9 cases had clefting, from mild (submucous cleft palate) to severe (cleft lip and palate), and 3/9 cases had ventricular septal defects. All cases had delayed motor development and most had learning disability, at worst in the mild intellectual disability range. The cases had overlapping facial features (broad forehead, finely arched eyebrows, mildly shortened philtrum, and tented upper lip) but individually they were not considered to be dysmorphic. Our results show that MEIS2 is a gene needed for palate closure. In syndromic cases of cleft palate, MEIS2 should be considered among the candidate genes, for example, in cases without 22q11.2 deletions. PMID:24678003

  17. Evaluation of cleft lip and palate by computed tomography with 2 mm thin slice scanning

    Energy Technology Data Exchange (ETDEWEB)

    Tanaka, Hiroshi

    1988-07-01

    Computed tomography was carried out in 65 patients of cleft lip and palate with continuous 2 mm slice scanning. The cleft lip and palate was classified by shape of the hard palate as normal, hypoplasia, and aplasia, depending on its developmental degree. The shape of alveolus was also grouped as circular, triangular, and asymmetric forms for the evaluation of maxillar development. The hard palatal development well correlated with the shape of the alveolus. Frequency of sinusitis and mastoiditis increased with the severity of hard palatal malformation. Evaluation of the hard palate by thin slice scanning is usefull standpoint of presumption of future maxillary development.

  18. Evaluation of cleft lip and palate by computed tomography with 2 mm thin slice scanning

    International Nuclear Information System (INIS)

    Computed tomography was carried out in 65 patients of cleft lip and palate with continuous 2 mm slice scanning. The cleft lip and palate was classified by shape of the hard palate as normal, hypoplasia, and aplasia, depending on its developmental degree. The shape of alveolus was also grouped as circular, triangular, and asymmetric forms for the evaluation of maxillar development. The hard palatal development well correlated with the shape of the alveolus. Frequency of sinusitis and mastoiditis increased with the severity of hard palatal malformation. Evaluation of the hard palate by thin slice scanning is usefull standpoint of presumption of future maxillary development. (author)

  19. Notch-free vermillion after unilateral cleft lip repair: The Charles Pinto centre protocol

    Directory of Open Access Journals (Sweden)

    Narayanan P

    2008-01-01

    Full Text Available A notch on the vermillion is one of the most common complications following the repair of a unilateral cleft lip. Several methods have been described for the secondary correction of a notch. However, there are only a few reports on how the notch can be prevented during primary lip repair. Causes of a vermillion notch were analysed at the Charles Pinto Centre for Cleft Lip and Palate and each possible cause addressed by an appropriate procedure. This protocol was then followed in every patient. In this manner, we have been able to avoid notches in unilateral cleft lips altogether and more significantly, junior trainees in our department have also been able to consistently avoid a notch in their repairs.

  20. "The open sky rhinoplasty" for correction of secondary cleft lip nose deformity, Technique and recent results.

    Science.gov (United States)

    Tschopp, H M

    1988-01-01

    The typical nose deformity after cleft-lip repair still represents great challenge for the plastic surgeon in this field. Many methods have been designed in attempts to correct the deformity, either during primary closure of the lip or as a secondary procedure when growth of the nose is complete. In this paper the author's own technique of secondary rhinoseptoplasty in cleft-lip patients is presented. The different cartilaginous and osseous structures are approached through a transcolumellar incision and the skin of the nose completely reflected upwards. This so-called "open sky view" gives a total perspective on the entire pathology of the cleft-lip nose deformity and makes it possible to correct it accordingly. Emphasis is put on some important surgical manoeuvers. Recent results are presented and the pros and cons of this procedure discussed. PMID:3187450

  1. Reconstruction during phase II of unilateral cleft lip improving function of musculus orbicularis oris%单侧唇裂Ⅱ期整复改善口轮匝肌功能

    Institute of Scientific and Technical Information of China (English)

    马新亮; 陈传俊; 韩立显

    2002-01-01

    @@ Background:Nasolabial deformity secondary to operation of cleft lip seriously affect patients' physiological and psychological function.Plasticity or reposition of musculus orbicularis oris received more attention in recent years.Since 1995,nasolabial deformity secondary to operation of unilateral complete cleft lip was reduced in 17 patients,and therapeutic effect is satisfying.

  2. Optimization of dental status improves long-term outcome after alveolar bone grafting in unilateral cleft lip and palate.

    Science.gov (United States)

    Jabbari, Fatima; Skoog, Valdemar; Reiser, Eicka; Hakelius, Malin; Nowinski, Daniel

    2015-03-01

    Objective : To evaluate the importance of dental status for long-term outcome after alveolar bone grafting in patients with unilateral cleft lip and palate. Design : Retrospective longitudinal study. Setting : Cleft lip and palate-craniofacial center, Uppsala University Hospital, Sweden. Patients : A total of 67 consecutive patients with unilateral complete cleft lip and palate. Interventions : Secondary alveolar bone grafting, prior to the eruption of the permanent canine, was performed at the average age of 10.0 years (range, 8.5 to 12.0 years). Main Outcome Measures : Alveolar bone height was evaluated with the modified Bergland index at 1 and 10 years after surgery. Results : Of the patients, 97% had modified Bergland index grade I and the remaining 3% had modified Bergland index grade II at 1 year after surgery. At 10 years' follow-up, 43% showed modified Bergland index grade I; 55%, modified Bergland index grade II; and 2% (one patient), modified Bergland index grade III. The degree of dental anomalies in the cleft area, such as enamel hypoplasia, incisor rotation, incisor inclination, canine inclination, and oral hygiene registered preoperatively, all correlated negatively to the modified Bergland index at 10 years after surgery. Enamel hypoplasia (ρ = 0.70195, P < .0001), followed by canine inclination (ρ = 0.55429, P < .0001), showed the strongest correlation to reduced bone height in the cleft area. Conclusions : In patients with unilateral cleft lip and palate, excellent results from secondary alveolar bone grafting in terms of bone height in the alveolar cleft tend to decrease with time. This seems to be correlated with factors that might to some extent be treated preoperatively through adequate planning and execution of the orthodontic treatment. PMID:24568558

  3. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome

    OpenAIRE

    Reema Sharma Dhar; Amitava Bora

    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 ro...

  4. Risk of Oral Clefts (Cleft Lip and/or Palate) in Infants Born to Mothers Taking Topamax (Topiramate)

    Science.gov (United States)

    ... and/or ear infections. Most infants born with oral clefts undergo surgery to close the lip and/or palate during their first year of life. 2 Oral clefts happen early in pregnancy, before many women ...

  5. Dominantly inherited cleft lip and palate in two families.

    OpenAIRE

    Temple, K; Calvert, M; Plint, D; Thompson, E.; Pembrey, M

    1989-01-01

    Two families with non-syndromic cleft lip and cleft palate are described. The linear pattern of inheritance through several generations is difficult to explain by conventional multifactorial models. The pedigrees strengthen the suggestion that a dominantly inherited mutation exists with a major influence on clefting of the lip and palate alone.

  6. Diagnostic efficacy of radiological examinations in clefts of the hard palate

    International Nuclear Information System (INIS)

    The aim of the work has been: 1) evaluating the efficiency of individual radiological methods in visualizing the bone structure of the hard palate; 2) elaboration of a method for skull examination, by means of which the hard palate region in patients would be best visible; 3) presentation of radiological symptomatology of hard palate clefts; 4) establishing algorithms of diagnostic procedure and determining the type of radiological examination most helpful in planning the treatment of this anomaly. Selected problems from normal anatomy of the hard palate are presented, and the technique of radiological examination in the form of occlusal radiograms, pantomography and computerized tomography (CT) are discussed. Clinical material encompassed the total of 312 patients. A total of 470 radiograms were performed, 150 occlusal ones of hard palate, 200 pantomograms (jointly with the control group) as well as 120 scannings during CT examination. It has been stated the greatest efficiency and effectiveness in planning the treatment are ascribed to computerized tomography, the second place goes to pantomography, on the third position are occlusal radiograms targeted at the region of the cleft. Algorithms have been provided for roentgen-diagnostic procedure in cases of the hard palate clefts, with an emphasis that the very first examination of a child should include the occlusal radiograms targeted at the cleft region and pantomogram; in the course of conservative treatment only pantomogram is proposed to be made, and in case of planned operative procedure - CT examination. For evaluating the calcification of the cleft, the best and with the least irradiation are the intraoral occlusal radiograms, targeted at the region of the cleft, performed 12 months after the operation. (author). 100 refs, 21 figs, 12 tabs

  7. An autosomal recessive syndrome of cleft palate, cardiac defect, genital anomalies, and ectrodactyly (CCGE).

    OpenAIRE

    Giannotti, A; Digilio, M C; Mingarelli, R; Dallapiccola, B.

    1995-01-01

    We report a brother and sister affected by a constellation of malformations, including cleft palate, cardiac defect, genital anomalies, and ectrodactyly (CCGE). A similar association has been reported previously by Richieri-Costa and Orquizas in a male patient born to consanguineous parents. An autosomal recessive pattern of inheritance is proposed for this syndrome.

  8. Histological comparison of the alar nasal cartilages in unilateral cleft lip

    Directory of Open Access Journals (Sweden)

    Modolin Miguel

    2002-01-01

    Full Text Available Patients with unilateral cleft lip display characteristic nasal changes that are independent of the degree of deformity. Defenders of the intrinsic theory consider these deformities to be due to embryogenic alterations of the alar nasal cartilages. Those that propose the extrinsic theory defend the thesis that the deformity is due to disorganization of the perioral muscles deformed by the cleft. The purpose of this study is to contribute histological evidence to help clarify the issue. PATIENTS AND METHODS: Specimens of the lateral portion of both the healthy and the cleft side of the alar cartilages were obtained from 18 patients. These uniformly cut specimens were stained by hematoxylin and eosin. Samples from 2 patients were excluded due to imperfections. The same pathologist examined all the slides. He was unaware of the origins of the specimens; he counted the number of chondrocytes and quantified the cartilage matrixes. RESULTS: All data was analyzed statistically, and no significant statistical differences were apparent, either in the number of chondrocytes or the cartilage matrix between the healthy side and the cleft side. DISCUSSION: These results apparently support the group that defend the extrinsic theory; nevertheless, the doubt about the composition of the cartilage matrix remains, not only concerning the glycosaminoglycans that compose them, but also regarding elastin and collagen and its linkages that can cause different degrees of collagen consistency.

  9. Weight Gain in Children with Cleft Lip and Palate without Use of Palatal Plates

    Directory of Open Access Journals (Sweden)

    Renato da Silva Freitas

    2012-01-01

    Full Text Available Goals/Background. To evaluate children’s growth in the first year of life, who have cleft palate and lip, without the use of palatal plates. Materials/Method. Chart review was conducted, retrospectively, in the Center for Integral Assistance of Cleft Lip and Palate (CAIF, in Brazil, between 2008 and 2009. Results for both genders were compared to the data published by the World Health Organization (WHO regarding average weight gain in children during their first year of life. Results. Patients with syndromic diagnosis and with cleft classified as preforamen were excluded, resulting in a final number of 112 patients: 56 male and 56 female. Similar patterns were seen comparing the two genders. Although it was observed weight gain below the average until the 11th month in male patients and until 9 months in female patients, both genders remained at the 50th percentile (p50 and improved after the 4th month of age for boys and the 9th month of age for girls. Conclusion. Children with cleft palate weigh less than regular children during their first months of life. At the end of the first year, weight gain is similar comparing normal and affected children. However, factors that optimized weight gain included choosing the best treatment for each case, proper guidance, and multiprofessional integrated care.

  10. The influence of age at operation for clefts on the development of the jaws

    OpenAIRE

    Egyedi, P.; Blijdorp, P.

    1984-01-01

    In this follow-up study of adult cleft patients with a unilateral cheilognathopalatoschisis, the effect on maxillo-mandibular development of the age at which the palatoplasty had been performed was investigated. It was concluded that it makes little difference whether the hard palate is closed at the age of 3 or 6 years.

  11. Neu-Laxova syndrome: a case report.

    Science.gov (United States)

    Roy, S; Begum, J; Sharifunnaher, B; Saha, A K; Afroza, S; Islam, M Z

    2014-01-01

    Neu-Laxova syndrome (NLS) is a rare lethal syndrome found in both consanguinous and non-consanguinous couple. This is characterized by terrible face with unusual craniofacial appearance with exophthalmos, spectrum of central nervous system malformation, like microcaphaly, hypoplastic cerebellum, cleft lip/palate, ichthyosis and oedema. The diagnosis is made on the basis of clinical parameter. We report a 4 hour old male term newborn with IUGR of Neu-Laxova syndrome presented with anencephaly, rudimentary cerebellum, exophthalmos of right eye, bilateral cleft lip and palate and cryptorchidism. Anencephaly and cryptorchidism are two recently reported findings of NLS. We are presenting this case in addition from Bangladesh to lend further support to those two new findings as component of Neu-Laxova syndrome. Outcome of this syndrome is not good. Most of the patients are died of infection within hours to days. PMID:24584393

  12. Characteristics of Rathke's cleft cyst in MR imaging

    International Nuclear Information System (INIS)

    The purpose of this study was symptoms, macroscopic appearances and microscopic findings of Rathke's cleft cysts with magnetic resonance (MR) imaging. We analyzed the data from 31 patients with pathologically confirmed Rathke's cleft cysts. MR appearances were evaluated on T1WI, T2WI and contrast T1WI. Symptoms, macroscopic appearances and pathological findings were obtained from available medical records. We analyzed the images according to the following criteria: findings on the location and shape of the lesions and form of the lesional wall; the relationship between the maximum diameter of the lesions and symptoms; the relationship between MR and macroscopic appearance; the sites of adjacent contrast enhancement. The lesions were located mostly in both the intrasellar and suprasellar regions for a total of 87%. All lesions revealed either an oval or dumbbell shape with a smooth lesional wall. When correlated with physical symptoms, asymptomatic cases were associated with smaller lesions, while visual disturbances and dizziness were associated with relatively larger lesions. MR lesion signal intensity was related to the content of macroscopic appearance to some degree: the selected lesions showed shortening of T1 and T2 relaxation times in relation to the increase in protein concentration. This should have been macro-scopically reflected in the color and turbidity of the liquid within the cyst. Adjacent contrast enhancement around the lesion was found at various sites, but anterior enhancement was most frequent. Circumferential enhancement was revealed to be derived from inflammatory changes. Rathke's cleft cyst exhibits a varied MR signal. It may be difficult to differentiate from craniopharyngioma from the intensity alone. (author)

  13. [Presentation of a flap web space laterodigital in cleft foot].

    Science.gov (United States)

    Szwebel, J; Haddad, R; Mitrofanoff, M

    2012-08-01

    Cleft foot deformity is characterized by the absence of one or more median rays of the foot. This rare polymorphous congenital anomaly occurs more frequently in males, with a frequent autosomal dominant type of transmission. The purpose of surgical treatment is to narrow the width of the foot, but also to improve its global aesthetic look. Toe reparation, and more specifically web space reconstruction, provide the main technical challenges. We present an adaptation to the foot of a laterodigital cutaneous flap published by Barsky in 1964 for commissural reconstruction in cleft hand syndroms. The anatomical structure of fingers and toes commisures being different, this flap seems more adapted to the surgery of the foot. We gathered seven patients' files treated for ectrodactyly of the foot with this technique by the same surgeon from 2005 to 2008. No particular postoperative complications were noted, and the patients all expressed their satisfaction regarding the improvement of the appearance of their foot. We recommend to add the use of this flap in the "tool box" of the surgeon in charge of the management of foot deformities. PMID:20947236

  14. Oblique facial clefts in Johanson-Blizzard syndrome.

    Science.gov (United States)

    Corona-Rivera, Jorge Román; Zapata-Aldana, Eugenio; Bobadilla-Morales, Lucina; Corona-Rivera, Alfredo; Peña-Padilla, Christian; Solis-Hernández, Elizabeth; Guzmán, Celina; Richmond, Erick; Zahl, Christian; Zenker, Martin; Sukalo, Maja

    2016-06-01

    Johanson-Blizzard syndrome (JBS) is considered as an infrequent, but clinically easily recognizable autosomal recessive entity by the pathognomonic combination of congenital exocrine pancreatic insufficiency and hypoplastic alae nasi, in addition to other distinctive findings such as scalp defects, hypothyroidism, and rectourogenital malformations. There are few reports of patients with JBS in association with facial clefting, referring all to types 2 to 6 of Tessier's classification that can be characterized properly as oblique facial clefts (OFCs). We describe the clinical aspects in four patients with JBS and extensive OFCs. In all of them, the diagnosis of JBS was confirmed by the demonstration of homozygous or compound-heterozygous mutations in the UBR1 gene. Additionally, we review three previously reported cases of JBS with OFCs. Taking into account a number of approximately 100 individuals affected by JBS that have been published in the literature we estimate that the frequency of OFCs in JBS is between 5% and 10%. This report emphasizes that extensive OFCs may be the severe end of the spectrum of facial malformations occurring in JBS. No obvious genotype phenotype correlation could be identified within this cohort. Thus, UBR1 should be included within the list of contributory genes of OFCs, although the exact mechanism remains unknown. © 2016 Wiley Periodicals, Inc. PMID:26989884

  15. Fistula Rate after primary palatal repair with intravelarveloplasty: a retrospective three-year audit of six units (NorCleft) in the UK.

    Science.gov (United States)

    Moar, Kanwalraj K; Sweet, Christopher; Beale, Victoria

    2016-07-01

    Our aim was to evaluate the rate of fistulation after one-stage palatal repair by intravelarveloplasty in the NorCleft Cleft Services (Scotland and Northern England), this being a primary outcome measure after repair of a cleft palate. We designed a retrospective, three-year clinical audit of six cleft units in the UK, and retrospectively reviewed the casenotes of babies with cleft palate born in 2006-2008 who were treated by intravelarveloplasty. We recorded type of cleft and procedure, including lateral relieving incisions, and our main outcome measure was the presence of a fistula behind the incisive foramen at 3 years of age, or a history of repair of a fistula. A total of 743 patients had cleft palates, but 69 (9%) were excluded (because they had not been operated on, or had not been reviewed by the age of 3 years, or their records were unavailable). A total of 626 patients had had a Sommerlad intravelarveloplasty repair, and 48 had had mixed procedures including Veau-Wardill-Kilner, Furlow, or two-stage repairs, and were not studied further. Eighty-seven (14%) who had had intravelarvoloplasty had a fistula behind the incisive foramen. There was no significant difference in age at time of repair between those who developed a fistula and those who did not (p=0.65). The fistula rate of 14% is comparable with that of Sommerlad. The fistula rate was higher in patients who had had lateral releasing incisions (58/275, 21%) or who had bilateral cleft palate (16/63, 25%). To our knowledge this is the largest review of the fistula rate in patients who had primary palatal repair using the intravelarveloplasty technique in the UK, and shows significant correlation between lateral releasing incisions and formation of a fistula, except in the unilateral cleft lip and palate group (p=0.12). PMID:27118617

  16. Fetal genetic risk of isolated cleft lip only versus isolated cleft lip and palate: A subphenotype analysis using two population-based studies of orofacial clefts in scandinavia

    DEFF Research Database (Denmark)

    Jugessur, Astanand; Shi, Min; Gjessing, Håkon Kristian;

    2010-01-01

    BACKGROUND: Cleft lip only (CLO) and cleft lip and palate (CLP) are commonly regarded as variants of the same defect and are traditionally combined to form the single group of cleft lip with or without cleft palate (CL/P) prior to analysis. However, recent data have suggested that at least a...... subgroup of isolated CLO may be etiologically distinct from isolated CLP. METHODS: To explore fetal genetic risk of isolated CLO separately from isolated CLP, we performed a subphenotype analysis using two population-based studies of clefts in Scandinavia. One hundred twenty-one isolated CLO, 190 isolated...... CLP, and 592 control triads were available from Norway (1996-2001), and a further 76 isolated CLO and 107 isolated CLP triads were available from Denmark (1991-2001). Genotypes for 1315 SNPs in 334 autosomal cleft candidate genes were analyzed using two complementary statistical methods, Triad Multi...

  17. Neuroembryology and functional anatomy of craniofacial clefts

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    Ewings Ember

    2009-10-01

    Full Text Available The master plan of all vertebrate embryos is based on neuroanatomy. The embryo can be anatomically divided into discrete units called neuromeres so that each carries unique genetic traits. Embryonic neural crest cells arising from each neuromere induce development of nerves and concomitant arteries and support the development of specific craniofacial tissues or developmental fields. Fields are assembled upon each other in a programmed spatiotemporal order. Abnormalities in one field can affect the shape and position of developing adjacent fields. Craniofacial clefts represent states of excess or deficiency within and between specific developmental fields. The neuromeric organization of the embryo is the common denominator for understanding normal anatomy and pathology of the head and neck. Tessier′s observational cleft classification system can be redefined using neuroanatomic embryology. Reassessment of Tessier′s empiric observations demonstrates a more rational rearrangement of cleft zones, particularly near the midline. Neuromeric theory is also a means to understand and define other common craniofacial problems. Cleft palate, encephaloceles, craniosynostosis and cranial base defects may be analyzed in the same way.

  18. Isolated cleft sternum: Neonatal surgical treatment

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    Kanojia Ravi

    2007-01-01

    Full Text Available Isolated cleft sternum is a rare congenital defect of the anterior chest wall and is the result of failed ventral midline fusion of sternal bands. We present two cases operated 18 and 24 days after birth with satisfactory results. Surgery is indicated to protect the heart and mediastinal contents.

  19. Dating brittle tectonic movements with cleft monazite

    DEFF Research Database (Denmark)

    Berger, Alfons; Gnos, E.; Janots, E.; Whitehouse, M.; Soom, M.; Frei, Robert; Waight, Tod Earle

    2013-01-01

    phases. This allows the high precision isotope dating of cleft monazite. 232Th/208Pb ages are not affected by excess Pb and yield growth domain ages between 8.03 ± 0.22 Ma and 6.25 ± 0.60 Ma. Monazite crystallization in brittle structures is coeval or younger than 8 Ma zircon fission track data, and...

  20. Controle químico da microflora oral em pacientes fissurados labiopalatais durante o tratamento ortodôntico-cirúrgico: estudo piloto Oral microflora chemical control in cleft lip and palate patients during orthodontic-surgical treatment pilot study

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    Alcion Alves Silva

    2004-06-01

    Full Text Available O risco de infecção é uma preocupação durante o tratamento ortodôntico-cirúrgico, principalmente em pacientes como os fissurados lábio palatais, pois as condições anatômicas e oclusais contribuem para a proliferação microbiana. Métodos manuais para o controle da microflora oral são muitas vezes insuficientes para a obtenção de uma higiene satisfatória. Técnicas alternativas, como agentes químicos, têm sido propostas como procedimentos auxiliares. O objetivo deste estudo foi comparar a eficiência de dois agentes químicos como auxiliares no controle da microflora, em pacientes ortodôntico-cirúrgicos (fissurados lábio-palatais. O delineamento da pesquisa foi um estudo exploratório, microbiológico (piloto, duplo-cego, para testar os seguintes agentes químicos: triclosan 0,03% (Plax-Colgate Palmolive e clorexidina digluconada 0,12% (Duplak-Herpro/Dentsply. A amostra foi composta por 30 voluntários divididos em 3 grupos: Grupo I (controle, Grupo II (teste _ clorexidina digluconada 0,12% e Grupo III (teste _ triclosan 0,03%. Todos os grupos receberam orientação de higiene bucal prévia à primeira coleta de material. Depois de sete dias de uso dos agentes químicos uma segunda coleta foi realizada e o uso do agente foi suspenso. Uma terceira coleta foi realizada após sete dias a partir da suspensão do agente. A técnica semiquantitativa proposta por Pilonetto e Pilonetto20 foi adotada para avaliar o crescimento de colônias bacterianas. Os resultados mostraram diferenças entre a eficácia dos agentes químicos, sendo que o Grupo II mostrou uma redução mais significativa da microflora quando comparado ao grupo III.The infection risk is a preoccupation during orthodontic-surgical treatment, mainly at cleft lip and palate patients, for their anatomical and occlusal condictions, that contributs to oral microflora proliferation. Manual methods for microflora control are frequently unsuccessful to obtain a sctisfactory

  1. Fetal cleft lip with and without cleft palate: Comparison between MR imaging and US for prenatal diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Wang Guangbin, E-mail: wgb7932596@hotmail.com [Shandong Medical Imaging Research Institute, 324 Jingwu Road, Jinan, 250021 (China); Shan Ruiqin [Jinan Maternity and Child Care Hospital, Jinan (China); Zhao Lianxin; Zhu Xiangyu; Zhang Xinjuan [Shandong Medical Imaging Research Institute, 324 Jingwu Road, Jinan, 250021 (China)

    2011-09-15

    Objective: To describe the magnetic resonance (MR) imaging findings of fetal CL/CP and evaluate its diagnostic value. Methods and materials: Twelve fetuses with CL/CP diagnosed by transabdominal US underwent MR imaging within 2 days of US. Half-Fourier acquisition single-shot turbo spin echo (HASTE) sequence on T2-weighted images was performed on sagittal, coronal, and axial planes anatomic to the fetuses during maternal breath holding. US and MR imaging findings were compared with final diagnoses obtained from post-natal physical examination or fetal autopsy. Results: Final diagnoses confirmed incomplete midline cleft lip (n = 1), unilateral cleft lip and palate (n = 7), bilateral cleft lip and palate (n = 1), midline cleft lip and palate (n = 3). US and MR imaging diagnosed all 12 fetuses with cleft lip and the laterality. 5 (45.5%) of 11 cleft palates were identified with US, 2 of 7 fetuses with unilateral cleft palate, 2 of 3 fetuses with midline cleft palate, and one fetus with bilateral cleft palate. On MR imaging, 10 (91%) of 11 cleft palates were correctly detected. One fetus with unilateral cleft palate was not detected. No false-positives occurred. Conclusion: MR imaging is valuable for diagnosis of fetal CL/CP. It can demonstrate additional findings and provide more information compared with US.

  2. Fetal cleft lip with and without cleft palate: Comparison between MR imaging and US for prenatal diagnosis

    International Nuclear Information System (INIS)

    Objective: To describe the magnetic resonance (MR) imaging findings of fetal CL/CP and evaluate its diagnostic value. Methods and materials: Twelve fetuses with CL/CP diagnosed by transabdominal US underwent MR imaging within 2 days of US. Half-Fourier acquisition single-shot turbo spin echo (HASTE) sequence on T2-weighted images was performed on sagittal, coronal, and axial planes anatomic to the fetuses during maternal breath holding. US and MR imaging findings were compared with final diagnoses obtained from post-natal physical examination or fetal autopsy. Results: Final diagnoses confirmed incomplete midline cleft lip (n = 1), unilateral cleft lip and palate (n = 7), bilateral cleft lip and palate (n = 1), midline cleft lip and palate (n = 3). US and MR imaging diagnosed all 12 fetuses with cleft lip and the laterality. 5 (45.5%) of 11 cleft palates were identified with US, 2 of 7 fetuses with unilateral cleft palate, 2 of 3 fetuses with midline cleft palate, and one fetus with bilateral cleft palate. On MR imaging, 10 (91%) of 11 cleft palates were correctly detected. One fetus with unilateral cleft palate was not detected. No false-positives occurred. Conclusion: MR imaging is valuable for diagnosis of fetal CL/CP. It can demonstrate additional findings and provide more information compared with US.

  3. Middle ear cleft in chronic otitis media: a clinicohistopathological study.

    Science.gov (United States)

    Sharma, Karan; Manjari, Mridu; Salaria, Neha

    2013-12-01

    Chronic mucosal diseases of middle ear cleft or chronic suppurative otitis media has been traditionally defined as a chronic inflammation of the middle ear and mastoid usually associated with perforation of the tympanic membrane and otorrhoea. Understanding the pathology and pathogenesis of chronic suppurative otitis media is important in predicting the management, prognosis and sequelae of the disease. The present prospective study was conducted to evaluate the clinical, intraoperative and histopathological changes in middle ear cleft. 100 patients diagnosed with CSOM who underwent surgery were taken. The mucosa and granulation tissue was removed along with ossicles wherever indicated and sent for histopathological examination. On clinical examination, 72 cases were found to be of tubotympanic type and 28 cases of atticoantral variety. However, intraoperatively, of the tubotympanic cases 8 were found to be of unsafe type which was also proven histologically. Stratified squamous epithelium was revealed in most of the cases accompanied by changes in the submucosa. Ossicular chain was involved in 40 cases with incus being the commonest bone to be eroded. PMID:24427703

  4. Is Early Nasal Shaping With a Limited Alar Base Incision Possible in the Repair of Cleft Lips?

    Science.gov (United States)

    Kapi, Emin; Bozkurt, Mehmet; Ozer, Torun; Celik, Mehmet Yusuf

    2016-05-01

    The repair of cleft lips has an important place in plastic and reconstructive surgery. In the treatment of these deformities, the aim was to restore the normal lip morphology in the cleft area as well as repairing any coexisting nasal deformities. Various methods are in use for this purpose. One of the most commonly employed surgical methods is Millard repair. However, this method may lead to additional scarring in the alar base on the cleft side subsequent to the incision. In this study, the results obtained from a group who have been applied alar base incisions during the modified Millard repair are compared to a group who have undergone intranasal wide dissections.The patients enrolled in the study were randomized into 2 groups. The first group were applied the modified Millard repair. In the second group, the rotation, advancement, and C-flaps were prepared according to Millard surgical repair technique; however, instead of an alar base and nasofacial groove incision on the cleft side, a nasal and maxillary supraperiosteal wide dissection was made through the incision in the mucosa of the nostril.The intranasal dissection performed during the study was observed to provide the targeted outcome and a more satisfactory cosmetic result through the modified Millard repair. In conclusion, the authors are of the opinion that the limited alar base incision and the wide supraperiosteal dissection performed in patients with cleft lips is an alternative method that can be employed in a wide range of patients. PMID:27092908

  5. Functional and aesthetic correction of secondary unilateral cleft lip nasal deformities

    Directory of Open Access Journals (Sweden)

    Cohen Mimis

    2009-10-01

    Full Text Available The treatment of patients with unilateral cleft lip has undergone significant development during the last decades. With better understanding of the anatomy of the unilateral cleft lip and nasal deformities, primary correction of the nasal deformity at the time of lip repair, critical evaluation of short and long-term results following various treatment protocols, and constant striving for perfection in both aesthetics and function, we have been able to design improved treatment strategies and more accurate surgical techniques so as to achieve overall superior and long-lasting results. In this review article, we present our protocols and experience for functional and aesthetic correction of secondary unilateral cleft lip nasal deformities and a retrospective review of 219 consecutive patients treated at our Craniofacial Centre for correction of secondary unilateral cleft lip nasal deformities. The protocols used in the treatment of 219 consecutive patients at our Craniofacial Centre for correction of secondary unilateral cleft lip nasal deformities were reviewed. In addition, analysis of the most recent 51 consecutive patients who underwent complete clinical and functional evaluation with rhinomanometry followed by correction of the cleft lip nasal deformity was performed. A variety of time-honoured techniques of rhinoplasty were applied in the correction of the residual deformities to achieve symmetry, aesthetic balance, and functional correction of the nose. Follow-up ranged from 5-11 years. Analysis of the data revealed that 39 patients (76.47% had significant functional and aesthetic improvement; seven patients (13.07% had significant aesthetic improvement but a modest functional improvement; and five patients (9.8% required additional surgery to improve their appearance and had no functional improvement. Further analysis demonstrated that five out of seven patients in the second group had pharyngeal flaps in place that were primarily

  6. Cranio-facial clefts in pre-hispanic America.

    Science.gov (United States)

    Marius-Nunez, A L; Wasiak, D T

    2015-10-01

    Among the representations of congenital malformations in Moche ceramic art, cranio-facial clefts have been portrayed in pottery found in Moche burials. These pottery vessels were used as domestic items during lifetime and funerary offerings upon death. The aim of this study was to examine archeological evidence for representations of cranio-facial cleft malformations in Moche vessels. Pottery depicting malformations of the midface in Moche collections in Lima-Peru were studied. The malformations portrayed on pottery were analyzed using the Tessier classification. Photographs were authorized by the Larco Museo.Three vessels were observed to have median cranio-facial dysraphia in association with midline cleft of the lower lip with cleft of the mandible. ML001489 portrays a median cranio-facial dysraphia with an orbital cleft and a midline cleft of the lower lip extending to the mandible. ML001514 represents a median facial dysraphia in association with an orbital facial cleft and a vertical orbital dystopia. ML001491 illustrates a median facial cleft with a soft tissue cleft. Three cases of midline, orbital and lateral facial clefts have been portrayed in Moche full-figure portrait vessels. They represent the earliest registries of congenital cranio-facial malformations in ancient Peru. PMID:26010214

  7. Definition of critical periods for Hedgehog pathway antagonist-induced holoprosencephaly, cleft lip, and cleft palate.

    Directory of Open Access Journals (Sweden)

    Galen W Heyne

    Full Text Available The Hedgehog (Hh signaling pathway mediates multiple spatiotemporally-specific aspects of brain and face development. Genetic and chemical disruptions of the pathway are known to result in an array of structural malformations, including holoprosencephaly (HPE, clefts of the lip with or without cleft palate (CL/P, and clefts of the secondary palate only (CPO. Here, we examined patterns of dysmorphology caused by acute, stage-specific Hh signaling inhibition. Timed-pregnant wildtype C57BL/6J mice were administered a single dose of the potent pathway antagonist vismodegib at discrete time points between gestational day (GD 7.0 and 10.0, an interval approximately corresponding to the 15th to 24th days of human gestation. The resultant pattern of facial and brain dysmorphology was dependent upon stage of exposure. Insult between GD7.0 and GD8.25 resulted in HPE, with peak incidence following exposure at GD7.5. Unilateral clefts of the lip extending into the primary palate were also observed, with peak incidence following exposure at GD8.875. Insult between GD9.0 and GD10.0 resulted in CPO and forelimb abnormalities. We have previously demonstrated that Hh antagonist-induced cleft lip results from deficiency of the medial nasal process and show here that CPO is associated with reduced growth of the maxillary-derived palatal shelves. By defining the critical periods for the induction of HPE, CL/P, and CPO with fine temporal resolution, these results provide a mechanism by which Hh pathway disruption can result in "non-syndromic" orofacial clefting, or HPE with or without co-occurring clefts. This study also establishes a novel and tractable mouse model of human craniofacial malformations using a single dose of a commercially available and pathway-specific drug.

  8. Definition of Critical Periods for Hedgehog Pathway Antagonist-Induced Holoprosencephaly, Cleft Lip, and Cleft Palate

    Science.gov (United States)

    Heyne, Galen W.; Melberg, Cal G.; Doroodchi, Padydeh; Parins, Kia F.; Kietzman, Henry W.; Everson, Joshua L.; Ansen-Wilson, Lydia J.; Lipinski, Robert J.

    2015-01-01

    The Hedgehog (Hh) signaling pathway mediates multiple spatiotemporally-specific aspects of brain and face development. Genetic and chemical disruptions of the pathway are known to result in an array of structural malformations, including holoprosencephaly (HPE), clefts of the lip with or without cleft palate (CL/P), and clefts of the secondary palate only (CPO). Here, we examined patterns of dysmorphology caused by acute, stage-specific Hh signaling inhibition. Timed-pregnant wildtype C57BL/6J mice were administered a single dose of the potent pathway antagonist vismodegib at discrete time points between gestational day (GD) 7.0 and 10.0, an interval approximately corresponding to the 15th to 24th days of human gestation. The resultant pattern of facial and brain dysmorphology was dependent upon stage of exposure. Insult between GD7.0 and GD8.25 resulted in HPE, with peak incidence following exposure at GD7.5. Unilateral clefts of the lip extending into the primary palate were also observed, with peak incidence following exposure at GD8.875. Insult between GD9.0 and GD10.0 resulted in CPO and forelimb abnormalities. We have previously demonstrated that Hh antagonist-induced cleft lip results from deficiency of the medial nasal process and show here that CPO is associated with reduced growth of the maxillary-derived palatal shelves. By defining the critical periods for the induction of HPE, CL/P, and CPO with fine temporal resolution, these results provide a mechanism by which Hh pathway disruption can result in “non-syndromic” orofacial clefting, or HPE with or without co-occurring clefts. This study also establishes a novel and tractable mouse model of human craniofacial malformations using a single dose of a commercially available and pathway-specific drug. PMID:25793997

  9. FOXE1 association with both isolated cleft lip with or without cleft palate, and isolated cleft palate

    Science.gov (United States)

    Moreno, Lina M.; Mansilla, Maria Adela; Bullard, Steve A.; Cooper, Margaret E.; Busch, Tamara D.; Machida, Junichiro; Johnson, Marla K.; Brauer, David; Krahn, Katherine; Daack-Hirsch, Sandy; L'Heureux, Jamie; Valencia-Ramirez, Consuelo; Rivera, Dora; López, Ana Maria; Moreno, Manuel A.; Hing, Anne; Lammer, Edward J.; Jones, Marilyn; Christensen, Kaare; Lie, Rolv T.; Jugessur, Astanand; Wilcox, Allen J.; Chines, Peter; Pugh, Elizabeth; Doheny, Kim; Arcos-Burgos, Mauricio; Marazita, Mary L.; Murray, Jeffrey C.; Lidral, Andrew C.

    2009-01-01

    Nonsyndromic orofacial clefts are a common complex birth defect caused by genetic and environmental factors and/or their interactions. A previous genome-wide linkage scan discovered a novel locus for cleft lip with or without cleft palate (CL/P) at 9q22–q33. To identify the etiologic gene, we undertook an iterative and complementary fine mapping strategy using family-based CL/P samples from Colombia, USA and the Philippines. Candidate genes within 9q22–q33 were sequenced, revealing 32 new variants. Concurrently, 397 SNPs spanning the 9q22–q33 2-LOD-unit interval were tested for association. Significant SNP and haplotype association signals (P = 1.45E − 08) narrowed the interval to a 200 kb region containing: FOXE1, C9ORF156 and HEMGN. Association results were replicated in CL/P families of European descent and when all populations were combined the two most associated SNPs, rs3758249 (P = 5.01E − 13) and rs4460498 (P = 6.51E − 12), were located inside a 70 kb high linkage disequilibrium block containing FOXE1. Association signals for Caucasians and Asians clustered 5′ and 3′ of FOXE1, respectively. Isolated cleft palate (CP) was also associated, indicating that FOXE1 plays a role in two phenotypes thought to be genetically distinct. Foxe1 expression was found in the epithelium undergoing fusion between the medial nasal and maxillary processes. Mutation screens of FOXE1 identified two family-specific missense mutations at highly conserved amino acids. These data indicate that FOXE1 is a major gene for CL/P and provides new insights for improved counseling and genetic interaction studies. PMID:19779022

  10. Nasoalveolar Molding: A new Method for Cleft Lip and Palate Rehabilitation

    Directory of Open Access Journals (Sweden)

    A Jahanbin

    2014-04-01

    Full Text Available Introduction: Cleft lip and palate is a congenital anomaly occurring in 3 in 1000 live birth. One the major concern in this patient is about cleft gap and its surgical problems. Several surgical and non-surgical procedures have been done to decrease this gap. They aimed to achieve the more esthetic results as well as feeding success. Nasoalveolar Molding (NAM is a new method for reshaping nasal and alveolar bones, presurgically. Matsuo et al described that auricular cartilage could be molded permanently when treatment was done within 6 weeks of life. High levels of maternal estrogen in the fetal circulation can triggers hyaluronic acid which can alter the cartilage, ligament and connective tissue elasticity.Estrogen level continue to drop after 6 weeks of age. This concept was applied for the correction of nasal deformities in cleft lip patients. Nasolaveolar molding may stimulate immature nasal chondroblasts and produce interstitial expansion.   Aims of Nasoalveolar Molding: a. Active molding and repositioning of the deformed nasal cartilages and alveolar processes. b. Appropriate Lengthening of the columellac. Better bone healing after surgey due to reducing the gaped. Reduces the need for secondary alveolar bone grafts. Correction of lip position with minimal scarf. Reducing hospital stay for nasal esthetic surgeryg. Better weight gain in early infancy   Conclusion: Management of cleft lip and palate has been changed with more emphasis on the nasal and alveolar molding prior to the primary lip repair. This method reduces the number reconstructive surgeries  for the purpose of esthetics as well as may cause better feeding in early infancy.   Keywords: Cleft Lip and Palate, Infant Orthopedics, Nasal Molding.  

  11. TPM1 polymorphisms and nonsyndromic orofacial clefts susceptibility in a Chinese Han population.

    Science.gov (United States)

    Qian, Yajing; Li, Dandan; Ma, Lan; Zhang, Hongchuang; Gong, Miao; Li, Sheng; Yuan, Hua; Zhang, Weibing; Ma, Junqing; Jiang, Hongbing; Pan, Yongchu; Wang, Lin

    2016-05-01

    Located at 15q22 a susceptibility region for nonsyndromic orofacial clefts (NSOC), TPM1 encodes a group of highly conserved ubiquitous actin-binding proteins involved in the muscle contraction and cytoskeleton organization. Considering the multiple functions of TPM1 gene, we investigated the potential relationship between TPM1 polymorphisms and risk of NSOC in a Chinese Han population. Four tag single nucleotide polymorphisms (tSNPs) of TPM1 (rs11071720, rs3803499, rs12148828, and rs1972041) were selected to conduct a case-control study with 673 NSOC patients and 705 unrelated healthy controls from a Chinese Han population. The SNPs were genotyped by the IPLEX Sequenom MassARRAY platform. SNP rs1972041GA showed a decreased risk of NSOC in heterozygotes (P = 0.038, OR = 0.77, 95%CI = [0.61, 0.99]). Further stratified analysis revealed an enhanced protective effect of the minor allele G at rs197204 on lip with cleft palate (CLP) and cleft lip with or without cleft palate (CL/P) groups under a codominant or dominant model. No association was observed between the remaining three markers (rs11071720, rs3803499, and rs12148828) and NSOC as well as its subgroups. TPM1 polymorphisms might contribute to the etiology of NSOC, and more emphasis should be placed on TPM1 during craniofacial development. © 2016 Wiley Periodicals, Inc. PMID:26792422

  12. Bone Regeneration in Artificial Jaw Cleft by Use of Carbonated Hydroxyapatite Particles and Mesenchymal Stem Cells Derived from Iliac Bone

    Directory of Open Access Journals (Sweden)

    Motoko Yoshioka

    2012-01-01

    Full Text Available Objectives of the Study. Cleft lip and palate (CLP is a prevalent congenital anomaly in the orofacial region. Autogenous iliac bone grafting has been frequently employed for the closure of bone defects at the jaw cleft site. Since the related surgical procedures are quite invasive for patients, it is of great importance to develop a new less invasive technique. The aim of this study was to examine bone regeneration with mesenchyme stem cells (MSCs for the treatment of bone defect in artificially created jaw cleft in dogs. Materials and Methods. A bone defect was prepared bilaterally in the upper incisor regions of beagle dogs. MSCs derived from iliac bone marrow were cultured and transplanted with carbonated hydroxyapatite (CAP particles into the bone defect area. The bone regeneration was evaluated by standardized occlusal X-ray examination and histological observation. Results. Six months after the transplantation, perfect closure of the jaw cleft was achieved on the experimental side. The X-ray and histological examination revealed that the regenerated bone on the experimental side was almost equivalent to the original bone adjoining the jaw cleft. Conclusion. It was suggested that the application of MSCs with CAP particles can become a new treatment modality for bone regeneration for CLP patients.

  13. Columella Lengthening with a Full-Thickness Skin Graft for Secondary Bilateral Cleft Lip and Nose Repair

    Science.gov (United States)

    Lee, Yoon Seok; Shin, Dong Hyeok; Choi, Hyun Gon; Kim, Jee Nam; Lee, Myung Chul; Kim, Soon Heum; Kim, Cheol Keun; Jo, Dong In

    2015-01-01

    Background Various techniques for lengthening short columellae have been used for bilateral cleft nose repair. However, previous methods have not yielded satisfactory results. We performed a full-thickness skin graft to lengthen short columellae during secondary cleft nose repair in adult patients. Methods Ten bilateral cleft lip and nose patients underwent secondary cheiloplasty with open rhinoplasty between July 2008 and August 2014. The patients underwent a full-thickness skin graft on the medial crura to elongate the columella. The average age of the patients at the time of surgery was 22.2 years. Nasal profiles were evaluated before and after the operation using the photogrammetric method. Results The nasal profiles were improved in all patients, and all skin grafts were well taken, with the exception of one patient. Columellar height, nostril height, and columella-lip angle increased, and nasal width decreased significantly. The ratios of columellar height to nasal height, columellar height to nasal width, and nasal height to nasal width increased to a statistically significant extent. Conclusions Columella lengthening with a full-thickness skin graft is a simple and effective method for the repair of severely short columellae in bilateral cleft nose patients. We had satisfactory outcomes, with good color matching and aesthetically pleasing contours. PMID:26618116

  14. Oronasal fistula in cleft palate surgery

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    Sadhu Partha

    2009-10-01

    Full Text Available Oronasal fistula (ONF is the commonest complication associated with cleft palate surgery. The main symptoms associated with ONF are nasal regurgitation of food matter and hypernasality of voice. Repair of cleft palate under tension is considered to be the main reason of ONF though vascular accidents and infection can also be the cause. Most of the ONFs are situated in the hard palate or at the junction of hard and soft palate. Repair of ONF depends on its site, size and mode of presentation. A whole spectrum of surgical procedures starting from small local flaps to microvascular tissue transfers have been employed for closure of ONF. Recurrence rate of ONF is 25% on an average after the first attempt of repair.

  15. Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies - USP (HRAC-USP) - Part 2: Pediatric Dentistry and Orthodontics

    OpenAIRE

    FREITAS, José Alberto de Souza; GARIB, Daniela Gamba; OLIVEIRA, Thais Marchini; LAURIS, Rita de Cássia Moura Carvalho; de ALMEIDA, Ana Lúcia Pompéia Fraga; Neves, Lucimara Teixeira; TRINDADE-SUEDAM, Ivy Kiemle; YAEDÚ, Renato Yassutaka Faria; SOARES, Simone; PINTO, João Henrique Nogueira

    2012-01-01

    The aim of this article is to present the pediatric dentistry and orthodontic treatment protocol of rehabilitation of cleft lip and palate patients performed at the Hospital for Rehabilitation of Craniofacial Anomalies - University of São Paulo (HRAC-USP). Pediatric dentistry provides oral health information and should be able to follow the child with cleft lip and palate since the first months of life until establishment of the mixed dentition, craniofacial growth and dentition development. ...

  16. Treatment outcome and long-term stability of skeletal changes following maxillary distraction in adult subjects of cleft lip and palate

    OpenAIRE

    Satinder Pal Singh; Ashok Kumar Jena; Vidya Rattan; Ashok Kumar Utreja

    2012-01-01

    Aim : To evaluate the treatment outcome and long-term stability of skeletal changes following maxillary advancement with distraction osteogenesis in adult subjects of cleft lip and palate. Materials and Methods: Total 12 North Indian adult patients in the age range of 17-34 years with cleft lip and palate underwent advancement of maxilla by distraction osteogenesis. Lateral cephalograms recorded prior to distraction, at the end of distraction, 6 months after distraction, and at least 24 month...

  17. A genome-wide association study of cleft lip with and without cleft palate identifies risk variants near MAFB and ABCA4

    DEFF Research Database (Denmark)

    Beaty, Terri H; Murray, Jeffrey C; Marazita, Mary L;

    2010-01-01

    Case-parent trios were used in a genome-wide association study of cleft lip with and without cleft palate. SNPs near two genes not previously associated with cleft lip with and without cleft palate (MAFB, most significant SNP rs13041247, with odds ratio (OR) per minor allele = 0.704, 95% CI 0...

  18. Ehlers-Danlos syndrome associated with cleft lip and palate

    OpenAIRE

    Tan, Pearlie WW; Song, Colin; Lalonde, Donald

    2009-01-01

    Ehlers-Danlos syndrome (EDS) is a hereditary connective tissue disorder caused by abnormal collagen synthesis and presents with classic features of hyperextensibility, fragility of skin and joint hypermobility. It has not been commonly described as being associated with cleft lip and/or palate, and a literature search has disclosed only one case (1984) of type IV EDS with cleft lip and palate. The authors have seen another case of EDS associated with cleft lip and palate. The etiology and cli...

  19. Stability after Cleft Maxillary Distraction Osteogenesis or Conventional Orthognathic Surgery

    Directory of Open Access Journals (Sweden)

    Kristian Andersen

    2015-06-01

    Full Text Available Objectives: To compare stability of maxillary advancements in patients with cleft lip and palate following distraction osteogenesis or orthognathic surgery. Material and Methods: Inclusion criteria: 1 cleft lip and palate, 2 advancement > 8 mm. Eleven patients comprised the distraction osteogenesis group (DOG. Seven patients comprised the orthognathic treatment group (CONVG. Skeletal and soft tissue points were traced on lateral cephalograms: T1 (preoperatively, T2 (after surgery, T3 (follow-up. Group differences were analyzed using Students t-test. Results: At T1-T2, advancement of 6.98 mm (P = 0.002 was observed in DOG. Horizontal overjet increased 11.62 mm (P = 0.001. A point-nasion-B point (ANB angle increased 8.82° (P = 0.001. Aesthetic plane to upper lip was reduced 5.44 mm (P = 0.017 and the naso-labial angle increased 16.6° (P = 0.001. Vertical overbite (VOB increased 2.27 mm (P = 0.021. In T2-T3, no significant changes were observed in DOG. In T1-T2, horizontal overjet increased 8.45 mm (P = 0.02. The ANB angle, 9.33° (P = 0.009 in CONVG. At T2-T3, VOB increased, 2.35 mm (P = 0.046, and the ANB angle reduced, 3.83° (P = 0.003. In T2-T3, no parameters changed in CONVG. At follow-up (T3, VOB increased in CONVG compared with DOG, (P = 0.01. Vertical position of A point differed between the groups (P = 0.04. No significant intergroup differences between soft tissue parameters occurred. Conclusions: Distraction osteogenesis resulted in a stable position of the maxilla and movement upwards in vertical plane, however in case of orthognathic treatment sagittal relapse and a continued postoperatively downward movement was registered.

  20. Manifestations of Gorlin-Goltz syndrome

    DEFF Research Database (Denmark)

    Bygum, Anette; Larsen, Anne Kristine; Mikkelsen, Dorthe Bisgaard;

    2014-01-01

    INTRODUCTION: Gorlin-Goltz syndrome is an uncommon hereditary condition caused by mutations in the PTCH1 gene causing a wide range of developmental abnormalities. Multiple basal cell carcinomas, palmoplantar pits and jaw cysts are cardinal features. Many clinicians are unfamiliar with the different......, Denmark, in the period from 1994 to 2013. RESULTS: A total of 17 patients from eight families fulfilled the diagnostic criteria. In all, 14 patients had basal cell carcinomas, 12 patients had jaw cysts and ten patients had calcification of the falx cerebri. Other clinical features were frontal bossing......, kyphoscoliosis, rib anomalies, coalitio, cleft lip/palate, eye anomalies, milia and syndactyly. In one family, medulloblastoma and astrocytoma occurred. Traditional treatment principles of basal cell carcinomas were used including radiotherapy performed in six patients. PTCH1 mutations were identified in five...

  1. 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.

  2. 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. PMID:25231046

  3. The development of speech production in children with cleft palate

    DEFF Research Database (Denmark)

    Willadsen, Elisabeth; Chapman, Kathy

    The purpose of this chapter is to provide an overview of speech development of children with cleft palate +/- cleft lip. The chapter will begin with a discussion of the impact of clefting on speech. Next, we will provide a brief description of those factors impacting speech development for this...... population of children. Finally, research examining various aspects of speech development of infants and young children with cleft palate (birth to age five) will be reviewed. This final section will be organized by typical stages of speech sound development (e.g., prespeech, the early word stage, and...

  4. 唇腭裂患者的牙周健康状况及其影响因素%Periodontal status and its influencing factors in patients with cleft lip and palate

    Institute of Scientific and Technical Information of China (English)

    章筱悦; 陈振琦

    2014-01-01

    index compared with normal controls. Patients with unilateral and bilateral CLP(UCLP and BCLP) exhibit significant plaque accumulation and gingivitis bleeding. The prevalence of plaque accumulation and gingivitis is higher in patients with CLP than in controls, and plaque accumulation is significantly higher in patients with BCLP than in patients with UCLP. The cleft sides present a higher plaque index and probing depth than the noncleft sides. Periodontal problems in soft and hard tissues have often been reported in patients with CLP. Such problems include reduced keratinized gingiva, gingival recession, gingival inflammation, and decreased alveolar bone height, and all of which can affect the periodontal status. The colonization rate of Saccharomyces in patients with CLP is significantly higher than in healthy control subjects and is highest in patients with CLP who had undergone at least three surgeries and in patients with BCLP. Grafting an alveolar bone to the cleft area is a method to improve the alveolar morphology and increase bone mass. This method helps improve the periodontal status and provides healthy periodontal support for the teeth adjacent to the cleft. The incidence of gingival inflammation is also reduced. Therefore, the aim of this study is to summarize the oralhygiene and periodontal status, including its influencing factors, of patients with CLP.

  5. Research on orthodontic treatment for complete unilateral cleft lip and palate patients in late permanent dentition%恒牙晚期单侧完全性唇腭裂患者正畸治疗临床疗效研究

    Institute of Scientific and Technical Information of China (English)

    刘毅; 王春玲; 孙慧芳; 王海任

    2011-01-01

    Objective By measuring the post-surgery characteristics of complete unilateral cleft lip and palate (UCLP) patients' teeth, occlusion, soft and hard tissues in late permanent dentition, the purpose of this study was to investigate the clinical effects of orthodontic treatments for complete UCLP patients on face types, maxillary dental arch and occlusion relations and to provide reliable theoretic basis for treating these kinds of patients. Methods Ten post-surgery complete UCLP patients who had cleft lip and palate in late permanent dentition( From 15 to 17 years old) were selected in this research. Every patient was treated with maxillary slow arch expansion and fixed appliance. The related data about X-ray lateral skull films before therapy and after six months' maxillary expansion, as well as teeth models before expansion, after expansion and after six months' were measured. The Statistical Methods were paired t-test for dependent samples and two-way ANOVA, LSD-test were used for the mutual comparison between two groups. Results After orthodontic treatments, some measured data of soft and hard tissues in complete UCLP patients' lateral skull films had relatively significant changes (P <0.01 ). Arch width increased significantly in canine area and premolar area( P < 0. 01 ). The increase in premolar area could be maintained steadily (P < 0.05). Conclusions After orthodontic treatments, bone, soft and hard tissues and occlusion relations of complete UCLP patients in late permanent dentition who had get cleft lip and palate had relatively ideal effects.%目的 通过测量恒牙晚期单侧完全性唇腭裂(UCLP)患者术后牙(牙合)、软硬组织的相关数据,观察正畸治疗对患者面型、牙弓及咬合关系的临床疗效.方法 选择10例UCLP恒牙晚期(15~17岁)术后患者,采用上颌慢速扩弓联合固定矫治,对治疗前、扩弓保持半年后X线头颅侧位片及扩弓治疗前、扩弓结束后、保持半年后牙(牙合)

  6. Characterization of subtle brain abnormalities in a mouse model of Hedgehog pathway antagonist-induced cleft lip and palate.

    Directory of Open Access Journals (Sweden)

    Robert J Lipinski

    Full Text Available Subtle behavioral and cognitive deficits have been documented in patient cohorts with orofacial clefts (OFCs. Recent neuroimaging studies argue that these traits are associated with structural brain abnormalities but have been limited to adolescent and adult populations where brain plasticity during infancy and childhood may be a confounding factor. Here, we employed high resolution magnetic resonance microscopy to examine primary brain morphology in a mouse model of OFCs. Transient in utero exposure to the Hedgehog (Hh signaling pathway antagonist cyclopamine resulted in a spectrum of facial dysmorphology, including unilateral and bilateral cleft lip and palate, cleft of the secondary palate only, and a non-cleft phenotype marked by midfacial hypoplasia. Relative to controls, cyclopamine-exposed fetuses exhibited volumetric differences in several brain regions, including hypoplasia of the pituitary gland and olfactory bulbs, hyperplasia of the forebrain septal region, and expansion of the third ventricle. However, in affected fetuses the corpus callosum was intact and normal division of the forebrain was observed. This argues that temporally-specific Hh signaling perturbation can result in typical appearing OFCs in the absence of holoprosencephaly--a condition classically associated with Hh pathway inhibition and frequently co-occurring with OFCs. Supporting the premise that some forms of OFCs co-occur with subtle brain malformations, these results provide a possible ontological basis for traits identified in clinical populations. They also argue in favor of future investigations into genetic and/or environmental modulation of the Hh pathway in the etiopathogenesis of orofacial clefting.

  7. Behavioral pattern in Chinese school-aged children with cleft lip and palate

    Institute of Scientific and Technical Information of China (English)

    Pin Ha; Xu-Qing Zhuge; Qian Zheng; Bing Shi; Cai-Xia Gong; Yan Wang

    2013-01-01

    Objective:To obtain descriptive information of behavioral pattern inChinese school-aged children with cleft lip and palate.Methods:A total of93 cleft lip and palate patients between the age of6-11 year-old and treated atWestChinaStomatologyHospital were selected.And another 100 unaffected controls, matched for age and gender, were recruited randomly from a common primary school inChengdu.Chart review of medical records was used to obtain psychosocial checklists.Scores were compared with published norms and controls to evaluate the risk of problems, separately for three diagnostic groups.Results:The patients group had lower scores of social and academic competencies, especially those with facial deformity or speech problem. No difference was found in the aspect of activity competency.All patients showed elevations in behavior problems.But the type of behavior problems varied in different genders.Conclusions:Chinese school-aged children with cleft lip and palate are at raised risk for social and academic difficulties.Specific pattern of behavior problems displays differently depending on gender of the patient.

  8. "Nara" knot for suturing of cleft lip in children to make removal easy

    Directory of Open Access Journals (Sweden)

    Obaidullah

    2006-01-01

    Full Text Available Cleft patients usually go through a lifetime of repeated hospital admissions and multiple procedures. Suture removal at a tender age and on a sensitive area like the lip becomes a challenge for the nursing staff. It is also emotionally demanding on the part of the parents. Hence, in most centres these patients are at least sedated if not anaesthetised. We have been using a simple knot and running prolene material so that undoing of the knot becomes easy and suture removal more or less atraumatic. We would like to share our experience with readers through this article. An analysis of 53 cleft lip repairs has shown that this knot is safe and easily removable.

  9. Further evidence for the involvement of human chromosome 6p24 in the aetiology of orofacial clefting.

    Science.gov (United States)

    Davies, A F; Imaizumi, K; Mirza, G; Stephens, R S; Kuroki, Y; Matsuno, M; Ragoussis, J

    1998-01-01

    Chromosomal translocations affecting the 6p24 region have been associated with orofacial clefting. Here we present a female patient with cleft palate, severe growth retardation, developmental delay, frontal bossing, hypertelorism, antimongoloid slant, bilateral ptosis, flat nasal bridge, hypoplastic nasal alae, protruding upper lip, microretrognathia, bilateral, low set, and posteriorly rotated ears, bilateral microtia, narrow ear canals, short neck, and a karyotype of 46,XX,t(6;9)(p24;p23). The translocation chromosomes were analysed in detail by FISH and the 6p24 breakpoint was mapped within 50-500 kb of other breakpoints associated with orofacial clefting, in agreement with the assignment of such a locus in 6p24. The chromosome 9 translocation breakpoint was identified to be between D9S156 and D9S157 in 9p23-p22, a region implicated in the 9p deletion syndrome. Images PMID:9783713

  10. Synovial sarcoma of the neck masquerading as a malignant second branchial cleft cyst

    OpenAIRE

    Teng, Yao-Shu; Lin, Zhi-Hong; Li, Yong; Cao, Xiao-lin; Lin, Feng-Chun; Xiang, Jing-Jing

    2013-01-01

    Synovial sarcoma is an uncommon, aggressive malignant tumor of the soft tissues primarily involving the extremities of young adults. Head and neck synovial sarcoma is rare, and its diagnosis and therapy are still challenging.We report a case of a young patient with synovial sarcoma, clinically masquerading as cystic mass of the neck and malignant second branchial cleft cyst. The pathological diagnosis of the sarcoma was confirmed by a multimodality diagnostic protocol, including histological,...

  11. Three-Dimensional Upper Lip and Nostril Sill Changes After Cleft Alveolus Reconstruction Using Autologous Bone Grafting Versus Recombinant Human Bone Morphogenetic Protein-2.

    Science.gov (United States)

    Raposo-Amaral, Cassio Eduardo; Denadai, Rafael; Alonso, Nivaldo

    2016-06-01

    Cleft alveolus in patients with unilateral complete cleft lip and palate has been alternatively reconstructed with recombinant human bone morphogenetic protein (rhBMP)-2. However, its effects on upper lip and nostril sill anatomy are not known. Thus, the objective of this investigation was to assess and compare upper lip and nostril sill changes after cleft alveolus reconstruction with autologous bone from the iliac crest region and rhBMP-2. Patients were randomly allocated into 2 groups. In group 1, autologous bone from the iliac crest region was used to fill the cleft alveolus (n = 4), and in group 2, rhBMP-2 was used to fill the cleft alveolus (n = 8). Preoperatively and at one after the surgery, computerized tomography (CT) was performed. Reformatted CT imaging was used to perform cephalometric linear measurements of the upper lip and nostril sill regions. Inter- and intragroup data of the pre and postoperative reformatted CT measurements of the upper lip and nostril sill regions did not show differences (P >0.05) in cutaneous upper lip height and projection, nostril sill elevation, and subnasale projection. There were no significant upper lip and nostril sill anatomical changes after cleft alveolus reconstruction using autologous bone grafting and rhBMP-2. PMID:27244210

  12. Changing lifestyles and oral clefts occurrence in Denmark

    DEFF Research Database (Denmark)

    Bille, Camilla; Knudsen, Lisbeth B.; Christensen, Kaare

    2005-01-01

    The aim of this project was to access whether any changes in the birth prevalence of cleft lip with/without cleft palate (CL(P)) occurred in Denmark during the period 1988 through 2001. In this period an official recommendation of a supplementation of folic acid to pregnant women was introduced; ...

  13. Prenetal Detection of Oral Clefts : Diagnostic, Genetic and Ethical Aspects

    NARCIS (Netherlands)

    Maarse, W.

    2015-01-01

    Since the introduction of routine prenatal screening with ultrasound in the Netherlands in 2007, parents are confronted with the diagnosis of oral cleft (OC) already during pregnancy. This imposed a new dimension in cleft care in the Netherlands. As a consequence to increasing prenatal detection rat

  14. Feeding Techniques for Children Who Have Cleft Lip and Palate.

    Science.gov (United States)

    Klein, Marsha Dunn

    This pamphlet on feeding techniques for children who have cleft lip and palate emphasizes the role of the parent as part of a team involving many specialists. The pamphlet begins with explanations of complete and incomplete separations of the lip, unilateral and bilateral cleft lips, corrective surgical procedures, etc. The importance of weight…

  15. Diagnostic efficacy of radiological examinations in clefts of the hard palate; Skutecznosc diagnostyczna badan rentgenowskich w rozszczepach podniebienia twardego

    Energy Technology Data Exchange (ETDEWEB)

    Cieslinska-Wilk, G. [Pomorska Akademia Medyczna, Szczecin (Poland)

    1992-12-31

    The aim of the work has been: (1) evaluating the efficiency of individual radiological methods in visualizing the bone structure of the hard palate; (2) elaboration of a method for skull examination, by means of which the hard palate region in patients would be best visible; (3) presentation of radiological symptomatology of hard palate clefts; (4) establishing algorithms of diagnostic procedure and determining the type of radiological examination most helpful in planning the treatment of this anomaly. Selected problems from normal anatomy of the hard palate are presented, and the technique of radiological examination in the form of occlusal radiograms, pantomography and computerized tomography (CT) are discussed. Clinical material encompassed the total of 312 patients. A total of 470 radiograms were performed, 150 occlusal ones of hard palate, 200 pantomograms (jointly with the control group) as well as 120 scannings during CT examination. It has been stated the greatest efficiency and effectiveness in planning the treatment are ascribed to computerized tomography, the second place goes to pantomography, on the third position are occlusal radiograms targeted at the region of the cleft. Algorithms have been provided for roentgen-diagnostic procedure in cases of the hard palate clefts, with an emphasis that the very first examination of a child should include the occlusal radiograms targeted at the cleft region and pantomogram; in the course of conservative treatment only pantomogram is proposed to be made, and in case of planned operative procedure - CT examination. For evaluating the calcification of the cleft, the best and with the least irradiation are the intraoral occlusal radiograms, targeted at the region of the cleft, performed 12 months after the operation. (author). 100 refs, 21 figs, 12 tabs.

  16. Cone-Beam computed tomography evaluation of maxillary expansion in twins with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Luciane Macedo de Menezes

    2012-04-01

    Full Text Available OBJECTIVE: The establishment of normal occlusal relationships in patients with cleft lip and palate using rapid maxillary expansion may promote good conditions for future rehabilitation. OBJECTIVE: This study describes the clinical case of monozygotic twins with unilateral cleft lip and palate at the age of mixed dentition, who were treated using the same rapid maxillary expansion protocol, but with two different screws (conventional and fan-type expansion screw. Results were evaluated using plaster models, intraoral and extraoral photographs, and Cone-Beam computed tomography (CBCT scans obtained before the beginning of the treatment, (T1. METHODS: The patients were followed up for 6 months after maxillary expansion, when the same tests requested at T1 were obtained again for review (T2. T1 and T2 results were compared using lateral cephalometric tracings and measurements of the intercanine and intermolar distances in the plaster models using a digital caliper. RESULTS: The two types of expansion screws corrected the transverse discrepancy in patients with cleft lip and palate. The shape of the upper arches improved at 10 days after activation. CONCLUSION: CBCT scans provide detailed information about craniofacial, maxillary and mandibular changes resulting from rapid maxillary expansion. The most adequate screw for each type of malocclusion should be chosen after detailed examination of the dental arches.

  17. Photographs of dental casts or digital models: rating dental arch relationships in bilateral cleft lip and palate.

    NARCIS (Netherlands)

    Leenarts, C.M.; Bartzela, T.N.; Bronkhorst, E.M.; Semb, G.; Shaw, W.C.; Katsaros, C.; Kuijpers-Jagtman, A.M.

    2012-01-01

    Yardsticks have been developed to measure dental arch relations in cleft lip and palate (CLP) patients as diagnostic proxies for the underlying skeletal relationship. Travelling with plaster casts to compare results between CLP centres is inefficient so the aim of this study was to investigate the r

  18. TESSIER CLEFT NO. 7: REPORT OF 12 CASES

    Directory of Open Access Journals (Sweden)

    Utpal

    2014-06-01

    Full Text Available Tessier cleft 7, also known as Transverse facial cleft or congenital macrostomia, is a rare congenital anomaly. It presents with varying degree of severity and is associated with anomalies of other structures developing from first and second branchial arches. We report twelve cases of Tessier cleft 7 presenting to us at various ages. The cases were studied on the basis of antenatal and family history, age, sex, laterality, severity and other associated anomalies. The clefts were classified complete or incomplete on the basis of their lateral extension. Surgical correction of the clefts consisted of soft tissue repair and correction of associated deformities with satisfactory functional and aesthetic results, without any intra-operative and post-operative complications.

  19. Corneal changes in ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome: case series and literature review.

    Science.gov (United States)

    Felipe, Anthony F; Abazari, Azin; Hammersmith, Kristin M; Rapuano, Christopher J; Nagra, Parveen K; Peiro, Baltasar Moratal

    2012-10-01

    The aim of this study is to describe the corneal changes in three unrelated patients with ectrodactyly-ectodermal dysplasia-cleft lip and palate (EEC) syndrome and review the literature on the possible etiology and clinical presentation of similar cases. Case 1 is an 18-year-old female with cleft lip and palate, syndactyly, and bilateral corneal pannus superiorly and inferiorly. She was initially diagnosed and treated as herpes simplex virus keratitis. Case 2 is a 3-year-old female born with cleft lip and palate, absent radial digits in both hands, and bilateral lacrimal stenosis. She developed progressive stromal scarring and neovascularization in both eyes. Her cornea perforated after developing infectious ulceration. Case 3 is a 49-year-old male with cleft palate, claw-hand deformities, absent meibomian glands and lacrimal duct, right ankyloblepharon, and a superior wedge-shaped opacity in the left cornea. The clinical findings demonstrated the different spectrum of keratopathy seen in patients with EEC. All patients were treated medically and without any surgical intervention. Limbal stem cell deficiency (LSCD) is presumed to be the cause in all three cases. Corneal changes in EEC can have variable presentation. LSCD seems to be the etiology of such keratopathy. Recurrent infection from lacrimal drainage obstruction and tear film instability are other risk factors for disease severity and progression. PMID:22618129

  20. THE MAIN DIRECTIONS IN THE COMPLEX REHABILITATION OF CHILDREN WITH CLEFT LIP AND PALATE

    Directory of Open Access Journals (Sweden)

    Yulia Vladimirovna Stepanova

    2013-03-01

    Full Text Available Complex treatment of children with cleft lip and palate is complicated and multi-step. Treatment is conducted by high-skilled specialists. The organization and execution of this complex are possible only at the large specialized center. Coordinator of this work is the maxillofacial surgeon. The performance of rehabilitation circuits includes preoperative orthodontic and orthopedic treatment, operative intervention (reconstructive and plastic surgery, orthodontic and orthopedic treatment after operation. Post-operative conservative treatment prevents the development of secondary deformities of the nose and upper lip. Professional psychological help and long supervision promote the achievement of good social adaptation of patients with congenital cleft lip and palate, improvement of their health.

  1. ALTERNATIVE APPROACH IN THE TREATMENT OF A GINGIVAL CLEFT ASSOCIATED WITH LABIAL FRENULUM. A 3-YEAR FOLLOW-UP. (Case Report

    Directory of Open Access Journals (Sweden)

    Kamen Kotsilkov

    2015-07-01

    Full Text Available INTRODUCTION: The term Gingival cleft refers to a fissure in the gingival tissues and is usually caused by traumatic oral hygiene, abnormal frenula, trauma from occlusion, orthodontic, or pierce related trauma. Gingival clefts are classified depending on the extent of the inclusion of the gingival thickness into red and white. The recommended treatment approach for the incomplete white clefts is the gingivectomy of the affected keratinized tissue followed by a coronally advanced flap for the root coveradge, while the complete white clefts are treated with a laterally moved, coronally advanced flap, or a free gingival graft procedure. OBJECTIVE: This report presents a case with a spontaneous healing of an incomplete gingival cleft associated with a maxillary labial frenulum after an alternative frenuloectomy approach. METHODS: M.G. (45 with a localized chronic periodontitis, an abnormal papillary maxillary frenulum and an incomplete white cleft on the marginal gingiva of the left central incisor. An alternative frenuloectomy approach combined with a free gingival graft was selected for the correction of the abnormal frenulum. RESULTS:On the sixth month after the surgical procedure an almost complete recovery of the gingival margin was observed.The result at the third year demonstrates a stable gingival margin with a complete tissue recovery. CONCLUSION: The applied combined surgical approach led to a complete gingival recovery of the incomplete white gingival cleft without the commonly used gingivectomy and CAF. Further research is recommended to clarify the adjunctive benefits of the FGG in patients with gingival clefts associated with abnormal frenula.

  2. Hoffa's recess: incidence, morphology and differential diagnosis of the globular-shaped cleft in the infrapatellar fat pad of the knee on MRI and cadaver dissections

    International Nuclear Information System (INIS)

    We frequently observed a fluid-like indentation at the inferior posterior margin of Hoffa's fat pad of the knee and sought to establish the incidence and differential diagnostic criteria of this cleft. In total, 133 MRI studies and 35 cadaver specimens were analyzed for the location, size, and shape of clefts at the inferior posterior margin of Hoffa's fat pad. The incidence of a fluid-like ovoid cleft on MR images was 13.5% and in cadavers 14.3%. The cleft was located just below the insertion of the infrapatellar synovial fold (plica synovialis infrapatellaris, ligamentum mucosum). More linear-shaped indentations at the posterior margin were visible in all patients and cadavers due to the horizontal course of the alar folds. A fluid-filled indentation within the inferior posterior margin of Hoffa's fat pad has to be expected in more than 10% of knee studies and should not be confused with tumors like ganglion cysts. We term this cleft the infrahoffatic recess. One hypothesis of its origin concerns the embryological regression process of the infrapatellar membrane into the infrapatellar synovial fold. It should not be confused with linear clefts due to the alar folds. (orig.)

  3. The incidence of congenital anomalies associated with cleft palate/cleft lip and palate in neonates in the Konya region, Turkey.

    Science.gov (United States)

    Altunhan, Hüseyin; Annagür, Ali; Konak, Murat; Ertuğrul, Sabahattin; Ors, Rahmi; Koç, Hasan

    2012-09-01

    Additional congenital anomalies have often been found in patients with orofacial clefts. We wanted to find out the incidence and type of congenital malformations that may accompany cleft palate (CP) and cleft lip and palate (CLP) in babies born in the Konya region. A total of 121 newborn babies with CP or CLP were prospectively included in the study, and all were assessed in detail for congenital anomalies. Of 121 babies, 86 (71%) had CLP and 35 (29%) had CP. There was at least one congenital malformation in 80 (66%) of the cases. Additional congenital malformations were seen in 26 (74%) of the 35 with isolated CP, and 54 (63%) in the 86 patients with CLP (p<0.05). The most common congenital malformation was congenital heart disease, followed by head and neck anomalies. The most common congenital heart disease was atrial septal defect. A serious chromosomal anomaly was found in 18/121 patients with CP or CLP (15%). Of the 80 babies in whom congenital malformations were found, 31 (39%) had dysmorphic features. While 21 (68%) of dysmorphic cases had isolated CP, 10 (32%) had CLP (p<0.05). The rates of premature delivery, intrauterine growth retardation, and consanguinity between parents were higher in patients with CP or CLP. The neonatal mortality was 20% (n=24). Our results indicate that at least one congenital anomaly is also present in about two-thirds of newborn babies with CP and CLP, and these anomalies significantly increase their morbidity and mortality. All newborn babies with CP and CLP should be screened for additional congenital anomalies, particularly of the cardiovascular system. PMID:21880407

  4. Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP Part 4: Oral Rehabilitation

    Directory of Open Access Journals (Sweden)

    Jose Alberto de Souza FREITAS

    2013-06-01

    Full Text Available Treatment of patients with cleft lip and palate is completed with fixed prostheses, removable, total, implants and aims to restore aesthetics, phonetics and function and should be guided by the basic principles of oral rehabilitation, such as physiology, stability, aesthetics, hygiene and the expectations of the patient. In order to obtain longevity of a prosthetic rehabilitation, the periodontal and dental tissue as well as the biomechanics of the prosthesis are to be respected. The purpose of this article is to describe the types of prosthetics treatment, which are performed at HRAC/USP for the rehabilitation of cleft area in adult patients.

  5. Nonsyndromic cleft lip with or without cleft palate: New BCL3 information

    Energy Technology Data Exchange (ETDEWEB)

    Amos, C.; Hecht, J.T. [Univ. of Texas Medical School, Houston, TX (United States); Gasser, D. [Univ. of Pennsylvania School of Medicine, Philadelphia, PA (United States)

    1996-09-01

    We did not previously provide LOD scores for linkage assuming heterogeneity, as suggested by Ott for the linkage analysis of cleft lip with or without cleft palate (CL/P) and BCL3, ApoC2, and D19S178 in the paper by Stein et al. The results from analysis using the HOMOG program, allowing for heterogeneity under the reduced penetrance model, gave a maximum LOD score of 1.85 for ApoC2, 0.41 for BCL3, 0.03 for D19S178, and 1.72 for multipoint analysis in the interval. For the affecteds-only model, the values are 1.96 for ApoC2, 0.41 for BCL3, 0.01 for D19S178, and 1.44 for the multipoint analysis. 8 refs.

  6. Osteogenesis effect of guided bone regeneration combined with alveolar cleft grafting: assessment by cone beam computed tomography.

    Science.gov (United States)

    Xiao, W-L; Zhang, D-Z; Chen, X-J; Yuan, C; Xue, L-F

    2016-06-01

    Cone beam computed tomography (CBCT) allows for a significantly lower radiation dose than conventional computed tomography (CT) scans and provides accurate images of the alveolar cleft area. The osteogenic effect of guided bone regeneration (GBR) vs. conventional alveolar bone grafting alone for alveolar cleft defects was evaluated in this study. Sixty alveolar cleft patients were divided randomly into two groups. One group underwent GBR using acellular dermal matrix film combined with alveolar bone grafting using iliac crest bone grafts (GBR group), while the other group underwent alveolar bone grafting only (non-GBR group). CBCT images were obtained at 1 week and at 3 months following the procedure. Using Simplant 11.04 software, the bone resorption rate was calculated and compared between the two groups. The bone resorption rate from 1 week to 3 months following bone grafting without the GBR technique was 36.50±5.04%, whereas the bone resorption rate using the GBR technique was 31.69±5.50% (P=0.017). The application of autogenous iliac bone combined with the GBR technique for alveolar bone grafting of alveolar cleft patients can reduce bone resorption and result in better osteogenesis. PMID:26876144

  7. Disruption of teeth eruption in piglets with cleft lip with/without cleft palate

    Czech Academy of Sciences Publication Activity Database

    Putnová, I.; Odehnalová, S.; Stehlík, L.; Štembírek, Jan; Usvald, Dušan; Horák, Vratislav; Míšek, Ivan; Buchtová, Marcela

    Plzeň, 2009. s. 135-135. [Morphology 2009. 45th International Congress on Anatomy and 46th Lojda symposium on Histochemy. 07.09.2009-09.09.2009, Plzeň] R&D Projects: GA ČR(CZ) GP304/08/P289 Institutional research plan: CEZ:AV0Z50450515 Keywords : teeth * cleft palete * piglets Subject RIV: EA - Cell Biology

  8. Significant association between IRF6 820G→A and non-syndromic cleft lip with or without cleft palate in the Thai population

    OpenAIRE

    Srichomthong, C; Siriwan, P; Shotelersuk, V

    2005-01-01

    Background: Previous data have shown an association between DNA sequence variants in the IRF6 gene and an increased risk of non-syndromic cleft lip with or without cleft palate (CL/P) in some populations.

  9. Genetic determinants of facial clefting: analysis of 357 candidate genes using two national cleft studies from Scandinavia.

    Directory of Open Access Journals (Sweden)

    Astanand Jugessur

    Full Text Available BACKGROUND: Facial clefts are common birth defects with a strong genetic component. To identify fetal genetic risk factors for clefting, 1536 SNPs in 357 candidate genes were genotyped in two population-based samples from Scandinavia (Norway: 562 case-parent and 592 control-parent triads; Denmark: 235 case-parent triads. METHODOLOGY/PRINCIPAL FINDINGS: We used two complementary statistical methods, TRIMM and HAPLIN, to look for associations across these two national samples. TRIMM tests for association in each gene by using multi-SNP genotypes from case-parent triads directly without the need to infer haplotypes. HAPLIN on the other hand estimates the full haplotype distribution over a set of SNPs and estimates relative risks associated with each haplotype. For isolated cleft lip with or without cleft palate (I-CL/P, TRIMM and HAPLIN both identified significant associations with IRF6 and ADH1C in both populations, but only HAPLIN found an association with FGF12. For isolated cleft palate (I-CP, TRIMM found associations with ALX3, MKX, and PDGFC in both populations, but only the association with PDGFC was identified by HAPLIN. In addition, HAPLIN identified an association with ETV5 that was not detected by TRIMM. CONCLUSION/SIGNIFICANCE: Strong associations with seven genes were replicated in the Scandinavian samples and our approach effectively replicated the strongest previously known association in clefting--with IRF6. Based on two national cleft cohorts of similar ancestry, two robust statistical methods and a large panel of SNPs in the most promising cleft candidate genes to date, this study identified a previously unknown association with clefting for ADH1C and provides additional candidates and analytic approaches to advance the field.

  10. Blepharophimosis, ptosis, epicanthus inversus syndrome (BPES and cleft lip and palate. Report of two Brazilian families

    Directory of Open Access Journals (Sweden)

    N.M. Kokitsu-Nakata

    1998-06-01

    Full Text Available We have evaluated a girl and a boy with the blepharophimosis, ptosis and epicanthus inversus syndrome (BPES. The girl presented cleft palate and the boy showed cleft lip and palate as additional clinical signs. Both showed familial recurrence in fourth and third generations, respectively. The other family members also presented blepharophimosis, ptosis, and epicanthus inversus, but without lip and palatal involvement. There were no additional clinical signs nor infertility in these patients. To our knowledge this is the first instance of cleft lip and palate reported as additional signs of the BPES syndrome.Os autores descrevem uma menina e um menino com a síndrome de blefarofimose, ptose e epicanto inverso (BPES. A menina apresentou fissura de palato e o menino mostrou fissura de lábio e palato como sinais clínicos adicionais. Ambos mostraram recorrência familial em quatro e três gerações, respectivamente. Outros membros de ambas as famílias apresentaram também blefarofimose, ptose e epicanto inverso, mas sem envolvimento de lábio e palato. Não existem sinais clínicos adicionais nem infertilidade nestes pacientes. De acordo com o nosso conhecimento, este é o primeiro relato de fissura de lábio e palato registrada como sinal adicional na síndrome BPES.

  11. [Evaluation of the timing of orthodontic arch expansion and graft in cleft lip and palate].

    Science.gov (United States)

    Chang, Le; Wang, Yingnan; Liu, Hongyan

    2016-04-01

    Maxillary transverse growth is inhibited by congenital cleft, early surgical scar strain, and oppression of lipmuscles in patients with cleft lip and palate. Clinical manifestations have shown severely constricted maxilla, insufficientmaxillary width, mismatch of upper and lower dental arches, and crossbite. Alveolar bone graft and arch expansion can effectively correct the deficiency in maxillary width. This paper discusses the timing and success rate of alveolar bone graft, as wellas the relationship between alveolar bone graft and arch expansion. Secondary alveolar bone graft is optimally performed beforepermanent canine eruption, especially when the teeth have formed between half and three quarters of their roots. Rapid maxillaryexpansion prior to alveolar bone graft is beneficial because this process increases the gap of the cleft, expands bone graft, andreduces the difficulty. However, the stability of this process remains controversial. Small-scale studies have reported that rapidmaxillary expansion after alveolar bone graft can open the midpalatal suture without bone graft loss. Slow maxillary expansioncan provide continuous light forces to reconstruct the bone. However, these studies are coordinated with fixed orthodontictreatment. Further research is necessary to determine the effects of maxillary expansion on long-term stability of teeth. PMID:27337934

  12. CORRECTION OF ALVEOLAR ARCH MALALIGNMENT AFTER TOTAL CORRECTION IN UNILATERAL AND BILATERAL GR III CLEFTS

    Directory of Open Access Journals (Sweden)

    Mohana Rao

    2015-06-01

    Full Text Available AIMS AND OBJECTIVES: Aim of this study is to document and analyse the pre - operative and postoperative alveolar arch impressions . MATERIALS AND METHODS: 20 cases of both unilateral and bilateral GR III clefts selected for this study. These pati ents were operated at the age of not less than 9 months, preferably below the age of 18 months. Below the age of 9 months general condition of these children may not cope up the duration of general anesthesia and amount of surgical trauma. After intubating the patient preoperatively, with the help of custom made metal arch plates, at first, Medical grade Alginate material with optimal hydration used to get negative impression, from these , positive impressions were obtained by the use of optimally hydrated m edical grade stone powder routinely used by dental surgeons. These positive impressions along with negative impression s and metal plates allowed to settle down for 10 hours undisturbed, after that positive impressions retrieved carefully without breaking a nd stored for analysis. Similarly postoperative impressions were taken after a gap of 2 years in the same manner like that of pre - operative ones under general anesthesia. Both impressions w ere compared and analysis was done regarding alveolar arch malalign ment, asymmetry and arch collapse correction. RESULTS & CONCLUSION: The status of the alveolar arch after single stage operation for cleft lip and palate in the unilateral cleft was much improved and there was considerable correction of the arch collapse a s well; in the bilateral cleft the correction of the premaxillary protrusion was better appreciated than the degree of correction of the alveolar gap.

  13. Comparison of patients’ age receiving therapeutic services in a cleft care team in Isfahan

    Science.gov (United States)

    Soheilipour, Saeed; Soheilipour, Fatemeh; Derakhshandeh, Fatemeh; Hashemi, Hedieh; Memarzadeh, Mehrdad; Salehiniya, Hamid; Soheilipour, Fahimeh

    2016-01-01

    Background: Due to numerous difficulties in patients suffering from varieties of cleft lip and palate, their therapeutic management involves interdisciplinary teamwork. This study was conducted to compare the age of commencing treatments such as speech therapy, secondary palate and alveolar bone grafting and orthodontics between those who sought treatment early and late. Materials and Methods: In this retrospective study, 260 files of patients with cleft lip and palate based on their age at the time of admission to a cleft care team were divided into two groups: The early admission and late admission. Both groups compared based on four variables including the mean age of beginning speech therapy, palatal secondary surgery, alveolar bone grafting, and receiving orthodontics using t-test. Results: Based on the results, among 134 patients admitted for speech therapy, the mean age of initiating speech therapy in early clients was 3.3 years, and in the late ones was 9 years. Among 47 patients with secondary surgery, the mean age in early clients was 3.88 years, and in the late clients was 15.7 years. Among 17 patients with alveolar bone grafting, the mean age in the first group was 9 years, and in the other was 16.69 years. Among 24 patients receiving orthodontic services, the mean age in early clients was 7.66 years, and in the second group was 17.05 years. Conclusion: There was a significant difference between the age of performing secondary surgery and alveolar bone grafting and the age of beginning speech therapy and receiving orthodontic services in early references and late references to the team. PMID:27274350

  14. A modified presurgical orthopedic (nasoalveolar molding) device in the treatment of unilateral cleft lip and palate.

    Science.gov (United States)

    Subramanian, Chitravelu Siva; Prasad, N K K Koteswara; Chitharanjan, Arun B; Liou, Eric Jein Wein

    2016-01-01

    Nasoalveolar molding (NAM) can be done effectively to reshape the nasal cartilage and mold the maxillary dentoalveolar arch before surgical cleft lip repair and primary rhinoplasty. Presurgical NAM helps as an adjunct procedure to enhance the esthetic and functional outcome of the surgical procedures. We have developed a modified NAM device to suit to the needs of the patients coming from distant places for the treatment. This device helps in reducing the number of frequent visits the patient needs to take to the craniofacial center. The purpose of this presentation is to report this treatment technique and discuss its application. PMID:27403068

  15. Ankyloglossia with cleft lip: A rare case report

    Science.gov (United States)

    Jangid, Kritika; Alexander, Aurelian Jovita; Jayakumar, Nadathur Doraiswamy; Varghese, Sheeja; Ramani, Pratibha

    2015-01-01

    Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman syndrome or Simpson-Golabi-Behmel syndrome. This article presents a rare case of ankyloglossia associated with cleft lip treated with diode laser in a 12-year-old Indian boy who had undergone surgical correction of associated cleft lip soon after birth. Correction of ankyloglossia at a young age would lead to enhanced phonetics, improved oral hygiene, and overall personality development. PMID:26941523

  16. Cleft lip as a presentation of congenital syphilis

    Directory of Open Access Journals (Sweden)

    Richa Gupta

    2012-01-01

    Full Text Available Congenital syphilis may present with unusual symptoms in early stages which needs to be identified for prompt treatment. Here, we present a case of 13-day-old female child with congenital syphilis presenting with cleft lip.

  17. Helping parents cope with a cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Jenny Steyn

    1980-09-01

    Full Text Available For the layman, the area of cleft palate is one shrouded in mystery and half-knowledge. An unrepaired cleft lip and palate is an unpleasant, if not alarming sight, and parents are not always totally convinced by the reassuring words of doctors and nurses that “ plastic surgeons do a marvellous job on cleft palate babies.” They need proof that their baby will indeed look and be like any other baby within a few months. Equally important, they need reassurance that whatever feelings they may experience when their babies are born are natural and appropriate ones. As the baby grows and develops parents may need practical assistance, emotional support and information regarding their baby’s condition. It is essentially for these reasons that a fellowship group has been established under the auspices of SAIDA (Southern Africa Inherited Disorders Association to help parents and families of cleft lip and palate children.

  18. Proteomic Analysis of Unbounded Cellular Compartments: Synaptic Clefts.

    Science.gov (United States)

    Loh, Ken H; Stawski, Philipp S; Draycott, Austin S; Udeshi, Namrata D; Lehrman, Emily K; Wilton, Daniel K; Svinkina, Tanya; Deerinck, Thomas J; Ellisman, Mark H; Stevens, Beth; Carr, Steven A; Ting, Alice Y

    2016-08-25

    Cellular compartments that cannot be biochemically isolated are challenging to characterize. Here we demonstrate the proteomic characterization of the synaptic clefts that exist at both excitatory and inhibitory synapses. Normal brain function relies on the careful balance of these opposing neural connections, and understanding how this balance is achieved relies on knowledge of their protein compositions. Using a spatially restricted enzymatic tagging strategy, we mapped the proteomes of two of the most common excitatory and inhibitory synaptic clefts in living neurons. These proteomes reveal dozens of synaptic candidates and assign numerous known synaptic proteins to a specific cleft type. The molecular differentiation of each cleft allowed us to identify Mdga2 as a potential specificity factor influencing Neuroligin-2's recruitment of presynaptic neurotransmitters at inhibitory synapses. PMID:27565350

  19. Repair of bilateral cleft lip and its variants

    Directory of Open Access Journals (Sweden)

    Mulliken John

    2009-10-01

    Full Text Available The surgeon who lifts a scalpel to repair a bilateral cleft lip and nasal deformity is accountable for: 1 precise craftsmanship based on three-dimensional features and four-dimensional changes; 2 periodic assessment throughout the child′s growth; and 3 technical modifications during primary closure based on knowledge gained from long-term follow-up evaluation. These children should not have to endure the stares prompted by nasolabial stigmata that result from outdated concepts and technical misadventures. The principles for repair of bilateral complete cleft lip have evolved to such a level that the child′s appearance should be equivalent to, or surpass, that of a unilateral complete cleft lip. These same principles also apply to the repair of the variants of bilateral cleft lip, although strategies and execution differ slightly.

  20. Reconstruction of Congenital Nose, Cleft Primary Palate, and Lip Disorders.

    Science.gov (United States)

    Fiani, Nadine; Verstraete, Frank J M; Arzi, Boaz

    2016-07-01

    Clefts of the primary palate in the dog are uncommon, and their repair can be challenging. The aims of this article are to provide information regarding pathogenesis and convey practical information for the repair of these defects. PMID:26965528

  1. Ankyloglossia with cleft lip: A rare case report

    Directory of Open Access Journals (Sweden)

    Kritika Jangid

    2015-01-01

    Full Text Available Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman syndrome or Simpson-Golabi-Behmel syndrome. This article presents a rare case of ankyloglossia associated with cleft lip treated with diode laser in a 12-year-old Indian boy who had undergone surgical correction of associated cleft lip soon after birth. Correction of ankyloglossia at a young age would lead to enhanced phonetics, improved oral hygiene, and overall personality development.

  2. Lexical selectivity in Danish toddlers with cleft palate

    DEFF Research Database (Denmark)

    Willadsen, Elisabeth

    2013-01-01

    . Methods: All participants were video recorded at 18 months of age during play interaction with a parent. The video recordings were transcribed according to the IPA and an individual consonant inventory was established for each participant. The video recordings were also analysed with respect to word......Objective: To study if Danish toddlers with cleft palate display lexical selectivity in their early lexicon at 18 months of age. Design: A cross-sectional study. Participants: Thirty-four children with unilateral cleft lip and palate and 35 children without cleft palate, matched for gender and age...... productions establishing an observed productive vocabulary size for each participant. Results: At 18 months of age Danish toddlers with cleft palate showed marked lexical selectivity in their early words. The distribution of consonant classes observed at 11 months of age in a previous study of the children...

  3. Reproductive patterns among Danish women with oral clefts

    DEFF Research Database (Denmark)

    Yttri, Janne Elin; Christensen, Kaare; Knudsen, Lisbeth;

    2011-01-01

    Abstract Objective: The aim of the study was to compare the reproduction pattern among Danish women born with isolated oral clefts with the Danish background population. Design and setting: A nationwide population based historic cohort-study based on three registers: The Danish Facial Cleft Regis...... and biological reasons might be responsible for the reduced fertility. KEY WORDS: oral clefts, birth defects........7 years of age) when having their first child (difference = 2.6 years, CI: [2.41-2.80]). Conclusion: Danish women born with an oral cleft are more often childless or get their first child later - but if they get children they tend to get as many children as other Danish women. Both social, psychological...

  4. Computational Embryology and Predictive Toxicology of Cleft Palate

    Science.gov (United States)

    Capacity to model and simulate key events in developmental toxicity using computational systems biology and biological knowledge steps closer to hazard identification across the vast landscape of untested environmental chemicals. In this context, we chose cleft palate as a model ...

  5. Un Futuro Prometedor para su Nino con Labio Hendido y Paladar Hendido. Edicion Refundida (Bright Promise for Your Child with Cleft Lip and Cleft Palate. Revised Edition).

    Science.gov (United States)

    McDonald, Eugene T.; Berlin, Asa J.

    The booklet, written in Spanish, is intended to help parents of babies with cleft lip and/or cleft palate. Topics covered include the sequence of prenatal development and the effects of birth defects, common misconceptions about what causes the conditions, possible hereditary and environmental causes, and what it means to have a cleft palate or a…

  6. Variable expressivity in Patau syndrome is not all related to trisomy 13 mosaicism.

    Science.gov (United States)

    Hsu, Hui-Fang; Hou, Jia-Woei

    2007-08-01

    Patau syndrome (trisomy 13) is very rare in live-born babies. Individuals with this chromosomal syndrome have a short lifespan and are rarely seen beyond infancy. This study is aimed at the clinical spectrum, natural history, and survival of patients with trisomy 13. We reviewed the detailed data of 13 Patau syndrome live-born babies. Among them two individuals were delivered from continuation of pregnancy even after prenatal diagnosis. The remaining 11 patients were born to younger mothers who did not undergo amniocentesis because no major anomalies except for cleft lip/palate were found on prenatal sonograms. The common features of Patau syndrome including the clinical triad (microphthalmia, cleft lip/palate, and polydactyly) and non-cyanotic heart defects were always found in our series. However, certain serious central defects (holoprosencephaly, omphalocele, and single umbilical artery), which are easily recognized from prenatal sonogram, occurred less frequently than those stated in the literature. The median survival time was 95 days and was longer than that previously reported. There were two infants with trisomic mosaicism with different outcomes in both clinical spectrum and survival. Otherwise, we also found the increased recurrence risks of aneuploidy in two individuals, and the longest survivor (84 months) of non-mosaic trisomy 13 in Taiwan. We thus suggest that long-term survival in our series is strongly correlated with different expressivity after prenatal selection, in addition to cytogenetic mosaicism. Less associated anomalies such as polyhydramnios, oligohydramnios, intrauterine growth retardation, single umbilical artery, eye defects, holoprosencephaly, omphalocele, and polycystic kidney may contribute to their clinical courses. PMID:17603803

  7. EEC syndrome sans clefting: Variable clinical presentations in a family

    OpenAIRE

    Thakkar Sejal; Marfatia Yogesh

    2007-01-01

    Ectrodactyly, ectodermal dysplasia and cleft palate/lip syndrome (EEC) is a rare autosomal dominant syndrome with varied presentation and is actually a multiple congenital anomaly syndrome leading to intra- and interfamilial differences in severity because of its variable expression and reduced penetrance. The cardinal features include ectrodactyly, sparse, wiry, hypopigmented hair, peg-shaped teeth with defective enamel and cleft palate/lip. A family comprising father, daughter and son prese...

  8. Ectrodactyly, cleft lip and palate in two half sibs.

    OpenAIRE

    Lewis, M B; Pashayan, H M

    1981-01-01

    Two half sibs with bilateral complete cleft lip and complete cleft of the palate associated with ectrodactyly of the hands and feet, born to the same phenotypically normal mother, are reported. The younger of the two sibs also has dominantly inherited tremors (also referred to as essential heredofamilial tremors) as did her biological father. Possible genetic causes to explain the recurrence of the facial and limb malformations in the half sibs with additional central nervous system malformat...

  9. Ankyloglossia with cleft lip: A rare case report

    OpenAIRE

    Kritika Jangid; Aurelian Jovita Alexander; Nadathur Doraiswamy Jayakumar; Sheeja Varghese; Pratibha Ramani

    2015-01-01

    Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman...

  10. Pre-operative haematological investigations in paediatric orofacial cleft repair: Any relevance to management outcome?

    Directory of Open Access Journals (Sweden)

    Anthony T Adenekan

    2012-01-01

    Full Text Available Aim and Objectives: To determine the value of routine pre-operative haematologic investigations in children undergoing orofacial cleft repair. Background: Although routine pre-operative laboratory screening tests are carried out traditionally, some studies suggest that they are not absolutely necessary in the management of elective surgical patients. Materials and Methods: This is a prospective cohort study carried out at a tertiary health facility located in Nigeria. A review of the laboratory investigations in 116 paediatric orofacial cleft patients undergoing surgery during a 6-year period was undertaken. Pre-operative laboratory investigations and peri-operative transfusion records were analysed for the frequency and impact of abnormal results on treatment plan and outcome using the Statistical Packages for the Social Scientists 16.0. Results: All the children had pre-operative packed cell volume (PCV check on admission for surgery. The PCV ranged from 23% to 43%, mean was 32.9 (±3.7%. Twenty-two children (18.6% had sub-optimal PCV (<30%. Patients with the lowest PCV values (23% and 26% were transfused pre-operatively. The lowest post-operative PCV was 23%, mean 30.8 (±3.3%. There was no occasion of post-operative blood transfusion. Eighty-six patients (72.9% had full or partial serum electrolyte and urea analysis. Screening for sickle-cell disease was rarely done. Fourteen intra- and post-operative complications were recorded. None of these were predictable by the results of pre-operative screening tests carried out. All the children were discharged home in satisfactory condition. Conclusions: Routine laboratory testing has minimal impact on management and outcome of orofacial cleft surgeries. However, haematocrit screening may be appropriate, particularly in clinically pale patients.

  11. Psychological status as a function of residual scarring and facial asymmetry after surgical repair of cleft lip and palate.

    Science.gov (United States)

    Millar, Keith; Bell, Aileen; Bowman, Adrian; Brown, Denise; Lo, Tsz-Wai; Siebert, Paul; Simmons, David; Ayoub, Ashraf

    2013-03-01

    Objective : Objective measure of scarring and three-dimensional (3D) facial asymmetry after surgical correction of unilateral cleft lip (UCL) and unilateral cleft lip (UCLP). It was hypothesized that the degree of scarring or asymmetry would be correlated with poorer psychological function. Design : In a cross-sectional design, children underwent 3D imaging of the face and completed standardized assessments of self-esteem, depression, and state and trait anxiety. Parents rated children's adjustment with a standard scale. Setting : Glasgow Dental School, School of Medicine, College of Medical, Veterinary and Life Sciences. Patients : Fifty-one children aged 10 years with UCLP and 43 with UCL were recruited from the cohort treated with the surgical protocol of the CLEFTSIS managed clinical network in Scotland. Methods : Objective assessment to determine the luminance and redness of the scar and facial asymmetry. Depression, anxiety, and a self-esteem assessment battery were used for the psychological analysis. Results : Cleft cases showed superior psychological adjustment when compared with normative data. Prevalence of depression matched the population norm. The visibility of the scar (luminance ratio) was significantly correlated with lower self-esteem and higher trait anxiety in UCLP children (P  =  .004). Similar but nonsignificant trends were seen in the UCL group. Parental ratings of poorer adjustment also correlated with greater luminance of the scar. Conclusions : The objectively defined degree of postoperative cleft scarring was associated with subclinical symptoms of anxiety, depression, and low self-esteem. PMID:21846256

  12. A case report of brachial cleft cyst in the neck

    International Nuclear Information System (INIS)

    Branchial cleft cyst is the most common lateral neck cyst ; the vast majority are of the second branchial cleft origin. This presumably reflects the greater depth and longer persistence of the second cleft, compared with the first, third, and fourth clefts. We experienced a 49-year-old male whose chief complaint was a abnormal mass of the cleft parotid gland area and neck. As a result of careful analysis of clinical, radiological, and histopathological findings. We diagnosed it as a second branchial cleft cyst in the neck and obtained results as follows: 1. In clinical examination, there was a 10 X 15 cm sized, fluctuant painful mass in the left neck and parotid area. 2. In radiographic examination, a low echogenic mass with internal cystic change in the inferior parotid gland area was noted sonographically. Computed tomograph showed a 3 X 4 cm sized, well-defined cystic mass with heterogenous solid component in the anterior border of sternocleidomastoid muscle. MRI revealed 5 X 6 cm sized, well-marginated multiseparated mass in the same area. 3. In histopathological examination, lining of cyst was stratified squamous epithelium with typical lymph node pattern and inflammatory cell infiltration.

  13. Comparing caries risk profiles between 5- and 10- year-old children with cleft lip and/or palate and non-cleft controls

    DEFF Research Database (Denmark)

    Sundell, Anna Lena; Ullbro, Christer; Marcusson, Agneta;

    2015-01-01

    cleft lip and/or palate (CL(P)) children with non-cleft controls in the same age using a computerized caries risk assessment model. METHODS: The study group consisted of 133 children with CL(P) (77 subjects aged 5 years and 56 aged 10 years) and 297 non-cleft controls (133 aged 5 years and 164 aged 10......BACKGROUND: Previous studies have suggested that children with oral clefts may have higher caries prevalence in comparison with non-cleft controls but the relative importance of the potential risk factors is not clear. The aim of this study was to compare the caries risk profiles in a group of...

  14. Getting under the skin of p63.

    OpenAIRE

    Rinne, T.K.

    2010-01-01

    Mutations in the transcription factor gene p63 are causative for human developmental syndromes characterized by three main hallmarks: ectodermal dysplasia, limb malformations and orofacial clefting. Five different dominantly inherited human syndromes and two non-syndromic conditions have been linked to p63 gene defects. Ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome (EEC) is the most common p63-associated condition, since approximately 60 percent of all p63 mutations have be...

  15. p63 Gene Mutations in EEC Syndrome, Limb-Mammary Syndrome, and Isolated Split Hand–Split Foot Malformation Suggest a Genotype-Phenotype Correlation

    OpenAIRE

    van Bokhoven, Hans; Hamel, Ben C. J.; Bamshad, Mike; Sangiorgi, Eugenio; Gurrieri, Fiorella; Duijf, Pascal H. G.; Vanmolkot, Kaate R. J.; van Beusekom, Ellen; van Beersum, Sylvia E. C.; Celli, Jacopo; Merkx, Gerard F. M.; Tenconi, Romano; Fryns, Jean Pierre; Verloes, Alain; Newbury-Ecob, Ruth A.

    2001-01-01

    p63 mutations have been associated with EEC syndrome (ectrodactyly, ectodermal dysplasia, and cleft lip/palate), as well as with nonsyndromic split hand–split foot malformation (SHFM). We performed p63 mutation analysis in a sample of 43 individuals and families affected with EEC syndrome, in 35 individuals affected with SHFM, and in three families with the EEC-like condition limb-mammary syndrome (LMS), which is characterized by ectrodactyly, cleft palate, and mammary-gland abnormalities. Th...

  16. p63-associated disorders.

    OpenAIRE

    Rinne, T.K.; Brunner, H.G.; Bokhoven, J.H.L.M. van

    2007-01-01

    Heterozygous mutations in the transcription factor gene p63 are causative for several syndromes, with ectodermal dysplasia, orofacial clefting and limb malformations as the key characteristics. Different combinations of these features are seen in five different syndromes, of which ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome (EEC) is the most common one. Mutations in p63 can also cause non-syndromic single malformations, such as split hand foot malformation (SHFM4) and iso...

  17. Secondary bilateral cleft lip-nose deformity correction by rhinoplasty with simultaneous Abbe flap

    Directory of Open Access Journals (Sweden)

    Nitin J Mokal

    2014-01-01

    Full Text Available Aim: The purpose of this article is to review modification and outcome of secondary rhinoplasty along with Abbι flap for correction of secondary bilateral cleft lip deformity. Materials and Methods: A total of thirteen patients of secondary bilateral cleft lip-nose deformity having tight upper lip, lack of acceptable philtral column, Cupid′s bow definition, irregular lip scars, and associated nasal deformity were selected. All the patients received Abbι flap and simultaneous nasal correction. All cases were treated during a period of three years. Mean patient age at the time of the operation was 21 years, and ranged from 16 to 27 years. The average follow-up period was three years. Results: Assessment of results was based on comparing preoperative and postoperative clinical photographs done by surgeon and patient relatives and patient satisfaction questionnaires. The columellar lengthening and upper lip vermillion correction achieved was satisfactory. There were no perioperative complications such as airway obstruction, bleeding, infection, wound disruption, or flap necrosis.

  18. Considerations Regarding Age at Surgery and Fistula Incidence Using One- and Two-stage Closure for Cleft Palate

    OpenAIRE

    Simona Stoicescu; Dm Enescu

    2013-01-01

    Introduction: Although cleft lip and palate (CLP) is one of the most common congenital malformations, occurring in 1 in 700 live births, there is still no generally accepted treatment protocol. Numerous surgical techniques have been described for cleft palate repair; these techniques can be divided into one-stage (one operation) cleft palate repair and two-stage cleft palate closure. The aim of this study is to present our cleft palate team experience in using the two-stage cleft palate closu...

  19. Three Familial Cases with Ectrodactyly Ectodermal Displazia-Clefting Syndrome

    Directory of Open Access Journals (Sweden)

    Selma Bakar Dertlioğlu

    2012-03-01

    Full Text Available A family of four children where mother and father had history of consanguineous marriage presented at our clinic due to lack of hair growth, thinning of all body hair and finger deformities in their three daughters aged 10 years, six years and three months. Dermatological examination of the cases showed thinning of the hair on the scalp, eyebrows, and extremities, subungual hyperkeratosis in nails, palmoplantar hyperkeratosis, cutaneous xerosis and loss of sweating, conical appearance and hypodontia in the teeth. All three siblings were seen to have the 3rd and 4th digits on the hand and the 2nd, 3rd and 4th digits on the feet to be proximally conjoined. Histopathologic examination of the cutaneous biopsy materials taken from the patients revealed a decrease in the number and size of hair follicles and sebaceous glands. We present these familial cases who were diagnosed with Ectrodactyly-ectodermal dysplasia-clefting syndrome as a result of clinical and histopathologic evaluation due to their rarity.

  20. Postoperative analgesia for cleft lip and palate repair in children

    Science.gov (United States)

    Reena; Bandyopadhyay, Kasturi Hussain; Paul, Abhijit

    2016-01-01

    Acute pain such as postoperative pain during infancy was ignored approximately three decades ago due to biases and misconceptions regarding the maturity of the infant's developing nervous system, their inability to verbally report pain, and their perceived inability to remember pain. More recently, these misconceptions are rarely acknowledged due to enhanced understanding of the developmental neurobiology of infant pain pathways and supraspinal processing. Cleft lip and palate is one of the most common congenital abnormalities requiring surgical treatment in children and is associated with intense postoperative pain. The pain management gets further complicated due to association with postsurgical difficult airway and other congenital anomalies. Orofacial blocks like infraorbital, external nasal, greater/lesser palatine, and nasopalatine nerve blocks have been successively used either alone or in combinations to reduce the postoperative pain. Since in pediatric population, regional anesthesia is essentially performed under general anesthesia, association of these two techniques has dramatically cut down the risks of both procedures particularly those associated with the use of opioids and nonsteroidal anti-inflammatory drugs. Definitive guidelines for postoperative pain management in these patients have not yet been developed. Incorporation of multimodal approach as an institutional protocol can help minimize the confusion around this topic. PMID:27006533

  1. Association of TFAP2A gene polymorphism with susceptibility to non-syndromic cleft lip with or without palate risk in south Indian population.

    Science.gov (United States)

    Babu Gurramkonda, Venkatesh; Syed, Altaf Hussain; Murthy, Jyotsna; V K S Lakkakula, Bhaskar

    2016-09-01

    The aetiology of non-syndromic cleft lip with or without cleft palate (NSCL/P) is complex involving multiple interacting genes and environmental factors. The primary objective of the present study was to investigate the role of TFAP2A gene single nucleotide polymorphisms (SNPs) in the pathogenesis of NSCL/P. In this study, 173 unrelated NSCL/P patients and 176 controls without clefts were genotyped with TFAP2A rs1675414 (Exon 1), rs3798691 (Intron 1), and rs303050 (Intron 4) variants by allele-specific amplification using the KASPar SNP genotyping system. The method of multifactor dimensionality reduction (MDR) was used to analyze gene-gene interactions. TFAP2A polymorphisms are not found to be associated with non-syndromic cleft lip with or without cleft palate (NSCL/P) at either the genotype or allele levels. No linkage disequilibrium (LD) was found between TFAP2A variants. MDR analysis did not show a significant effect of the TFAP2A gene polymorphisms on susceptibility to NSCL/P (p > 0.05). These results suggest that the analyzed variations in TFAP2A gene might not be associated with NSCL/P pathogenesis in south Indian population. PMID:27617216

  2. Report of a child with aortic aneurysm, orofacial clefting, hemangioma, upper sternal defect, and marfanoid features: possible PHACE syndrome.

    Science.gov (United States)

    Slavotinek, Anne M; Dubovsky, Elizabeth; Dietz, Harry C; Lacbawan, Felicitas

    2002-07-01

    We report a female patient who had a scalp hemangioma, a cleft uvula, an upper sternal defect, pectus excavatum, arachnodactyly, pes planus, and joint hypermobility. She had rupture of an aortic aneurysm after minor trauma at 11 years of age. At 17 years of age, elective repair of a dilated, ectatic aorta was complicated by cerebral ischemia. Other vascular abnormalities in the proband included an aneurysm of the left subclavian artery, atresia of the right carotid artery, and calcified cerebral aneurysms. We believe that the proband's physical anomalies are best described by the PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) phenotypic spectrum. This spectrum of physical anomalies also includes sternal clefting and hemagiomas as part of the sternal malformation/vascular dysplasia (SM/VD) association, as found in our patient, and the acronym PHACES has also been used. We consider that the PHACE phenotypic spectrum is likely to be broader than previously recognized and includes orofacial clefting and aortic dilatation and rupture. Our patient also had skeletal anomalies that lead to consideration of Marfan syndrome as a diagnosis. It should be recognized that there is clinical overlap between PHACE syndrome and Marfan syndrome when aortic dilatation is present. We would also like to emphasize the minor nature of the cutaneous findings in our patient despite her severe vascular complications. This is in contrast to previous reports of large or multiple hemangiomas in PHACE syndrome. PMID:12116239

  3. Using 501c3 Foundations in the Care of Cleft and Craniofacial Children.

    Science.gov (United States)

    Smith, Kevin S; Henry, Byron T

    2016-05-01

    This article relates to the use of 501c3 foundations in the care of patients with cleft and craniofacial disorders. Both authors are medical directors and founders of foundations that serve these children: A Smile for a Child Foundation was set up to help children locally; Free to Smile was set up as an international missions foundation. This article explores the advantages and disadvantages of each type of foundation as well as the struggles and successes foundations face to help children locally and internationally. PMID:27150307

  4. Correction of nasal deformity in infants with unilateral cleft lip and palate using multiple digital techniques.

    Science.gov (United States)

    Zheng, Yaqi; Zhang, Dapeng; Qin, Tian; Wu, Guofeng

    2016-06-01

    Presurgical correction of severe nasal deformities before cheiloplasty is often recommended for infants with cleft lip and palate. This article describes an approach for the computer-aided design and fabrication of a nasal molding stent. A 3-dimensional photogrammetric system was used to obtain the shape information of the nosewing that was then built as the nostril support for the nasal molding stent. The stent was fabricated automatically with a rapid prototyping machine. This technique may be an alternative approach to presurgical nasal molding in the clinic. Moreover, the patient's nasal morphology can be saved as clinical data for future study. PMID:26724850

  5. Evaluation of cleft lip and palate by computed tomography with 2 mm thin slice scanning, 2

    International Nuclear Information System (INIS)

    Computed tomography was performed on 104 patients with cleft lip and palate by continuous 2 mm slice scanning. The type of hard palate was classified as normal, hypoplasia and aplasia, depending on its developmental degree. The shape of alveolus was also classified as circular, triangular and asymmetric forms for the evaluation of the maxillary development. The hard palate development correlated with the shape of the alveolus, the diameter of maxillary and mandibular bone, and frequency of sinusitis and otitis media. Evaluation of the hard palate by thin slice scanning is useful in presumption of future fecial development. (author)

  6. Evaluation of cleft lip and palate by computed tomography with 2 mm thin slice scanning, 2

    Energy Technology Data Exchange (ETDEWEB)

    Uchiyama, Mayuki; Tanaka, Hiroshi; Harada, Junta (Jikei Univ., Tokyo (Japan). School of Medicine)

    1992-06-01

    Computed tomography was performed on 104 patients with cleft lip and palate by continuous 2 mm slice scanning. The type of hard palate was classified as normal, hypoplasia and aplasia, depending on its developmental degree. The shape of alveolus was also classified as circular, triangular and asymmetric forms for the evaluation of the maxillary development. The hard palate development correlated with the shape of the alveolus, the diameter of maxillary and mandibular bone, and frequency of sinusitis and otitis media. Evaluation of the hard palate by thin slice scanning is useful in presumption of future fecial development. (author).

  7. Neuropsychological development in adolescents: cognitive and emotional model for considering risk factors for adolescents with cleft.

    Science.gov (United States)

    Richman, L C

    1995-03-01

    This article reviews neurologic, endocrinologic, and neuropsychological developments that affect our understanding of the adolescent patient. Neuroimaging and neuroradiologic techniques have assisted in identifying brain-behavior relationships and how different neuropsychological patterns result in different ways of thinking. Psychoneuroendocrinologic studies have shown that sex differences in maturation and hormonal effects on behavior need taking into account. At adolescence, the individual with a cleft or craniofacial condition may be at risk for adjustment problems due to earlier developmental events, which may affect language, behavior, and self-esteem. PMID:7748881

  8. Cleft lip and palate review: Epidemiology, risk factors, quality of life, and importance of classifications

    Directory of Open Access Journals (Sweden)

    Laureen Supit

    2008-12-01

    Full Text Available Cleft lip with or without cleft palate is the most occurring craniofacial anomaly in human, resulting from a complex etiology involving multiple genetic and environmental factors. The defect carries lifelong morbidity and economic burden. Children with clefts will require continuous medical interventions for at least the first 18 years of life, affecting many aspects of their lives. The extent and complexity of clefts vary infinitely, later determining individual management and outcome. Identification and classification play significant roles in initial assessment of these unique cleft cases, which affect options for following correctional attempts. Some classifications even allow measurement of progress after anatomical repositioning, and success rate after surgical repairs. The challenge of developing one such widely inclusive classification is discussed. (Med J Indones 2008; 17: 226-39Keywords: Cleft lip, cleft palate, congenital anomaly, cleft  classfications

  9. Cleft-lift operation for pilonidal sinuses under tumescent local anesthesia

    DEFF Research Database (Denmark)

    Bertelsen, Claus Anders

    2011-01-01

    The use of tumescent local anesthesia in the Bascom cleft-lift procedure has not been described before.......The use of tumescent local anesthesia in the Bascom cleft-lift procedure has not been described before....

  10. Comparison of bupivacaine alone and in combination with fentanyl or pethidine for bilateral infraorbital nerve block for postoperative analgesia in paediatric patients for cleft lip repair: A prospective randomized double blind study

    Directory of Open Access Journals (Sweden)

    Rajesh S Mane

    2011-01-01

    Conclusion: Thus we conclude that addition of fentanyl or pethidine to bupivacaine for Bilateral Intraoral Infraorbital Nerve Block prolong the duration of analgesia with no complications and can be used safely in paediatric patients.

  11. Craniofacial pattern of parents of children having cleft lip and/or cleft palate anomaly.

    Science.gov (United States)

    Raghavan, R; Sidhu, S S; Kharbanda, O P

    1994-01-01

    The craniofacial patterns of 38 sets of parents who had children with cleft lip and/or cleft palate anomalies (experimental group) were compared with the 24 sets of parents of healthy (noncleft) children (control group). Using a computerized program, 248 cephalograms (124 lateral and 124 frontal) were digitized and analyzed. The parents in the experimental group exhibited a distinct craniofacial morphology, including a significant decrease in upper anterior facial height (N-Ans) and total anterior face height (V-Gn). Anterior nasal spine (Ans) and maxillary alveolar process (A) were positioned more anteriorly and superiorly in the experimental group, which contributed to a significant increase in the length of the palate (Ans-Pns) and an anterosuperior rotation of the palatal plane. The cranial base angle in the experimental group was significantly obtuse and the articular angle was smaller than that of the controls. The counterclockwise rotation of the mandible was mitigated by a significant increase in the gonial angle. Parents in the experimental group also tended to have faces which were smaller in both transverse and vertical dimensions. PMID:8010522

  12. Subphenotyping and Classification of Orofacial Clefts: Need for Orofacial Cleft Subphenotyping Calls for Revised Classification.

    Science.gov (United States)

    McBride, W A; McIntyre, G T; Carroll, K; Mossey, P A

    2016-09-01

    Nonsyndromic orofacial clefting (OFC) describes a range of phenotypes that represent the most common craniofacial birth defects in humans, with an overall birth prevalence of 1:700 live births. Because of the lifelong negative implications on health and well-being associated with OFC and the numbers of people affected, quality research into its etiology, diagnosis, treatment outcomes, and preventative strategies is essential. A range of different methods is used for recording and classifying OFC subphenotypes, one of which is the International Classification of Diseases (ICD) system. However, there is a general perception that research is being hampered by a lack of sensitivity and specificity in grouping those with OFC into subphenotypes, with potential heterogeneity and confounding in epidemiologic, genetic, and genotype-phenotype correlation studies. This article provides a background to the necessity of OFC research, discusses current controversies within cleft subphenotyping, and provides a brief overview of current OFC classifications as well as their limitations. The LAHSHAL classification is described in the context of a potentially useful tool for OFC that could complement the ICD-10/ICD-11 Beta coding systems to become a simply understood, universally accepted, clinically friendly, and research-sensitive instrument. Empowering registries, clinicians, and researchers to use a common classification system would have significant implications for OFC research across the world at a time when accurate subphenotyping is crucial and health care research is becoming increasingly tailored toward the individual. PMID:26171570

  13. Cleft lip with or without cleft palate in Shanghai, China: Evidence for an autosomal major locus

    Energy Technology Data Exchange (ETDEWEB)

    Marazita, M.L. (Virginia Commonwealth Univ., Richmond, VA (United States)); Hu, Dan-Ning; Liu, You-E. (Zhabei Eye Institute, Shanghai (China)); Spence, A. (Univ. of California, Los Angeles, CA (United States)); Melnick, M. (Univ. of Southern California, Los Angeles, CA (United States))

    1992-09-01

    Orientals are at higher risk for cleft lip with our without cleft palate (CL[+-] P) than Caucasians or blacks. The authors collected demographic and family data to study factors contributing to the etiology of CL[+-]P in Shanghai. The birth incidence of nonsyndromic CL[+-]P (SHanghai 1980-87) was 1.11/1,000, with a male/female ratio of 1.42. Almost 2,000 nonsyndromic CL[+-]P probands were ascertained from individuals operated on during the years 1956-83 at surgical hospitals in Shanghai. Detailed family histories and medical examinations were obtained for the probands and all available family members. Genetic analysis of the probands' families were performed under the mixed model with major locus (ML) and multifactorial (MFT) components. The hypothesis of no familial transmission and of MFT alone could be rejected. Of the ML models, the autosomal recessive was significantly most likely and was assumed for testing three complex hypothesis: (1) ML and sporadics; (2) ML and MFT; (3) ML, MFT, and sporadics. None of the complex models were more likely than the ML alone model. In conclusion, the best-fitting, most parsimonious model for CL[+-]P in Shanghai was that of an autosomal recessive major locus. 37 refs., 1 tab.

  14. Study on the short-term effect of maxillary expansion using self-ligating brackets in patients with cleft lip and palate%自锁托槽对唇腭裂腭扩展短期效果的研究

    Institute of Scientific and Technical Information of China (English)

    曾飞煌; 钱玉芬; 潘晓岗

    2014-01-01

    目的:探讨自锁托槽对单侧完全性唇腭裂患者上颌腭扩展的短期治疗效果。方法选取5例恒牙期单侧完全性唇腭裂(UCLP)患者,未行牙槽突裂骨移植手术,上颌牙齿粘贴自锁托槽(AO, Time2),放置高弹性镍钛弓丝,采集患者治疗前和腭扩展6~17个月后的牙颌模型、头颅定位后前位片进行测量,分析上颌腭扩展前后患者的牙弓、牙槽骨、腭穹窿以及颌骨的形态变化。结果牙颌模型测量结果提示:第一前磨牙区牙弓宽度明显增大,其次为尖牙区牙弓宽度,第一磨牙区牙弓宽度增加最少,第二磨牙区宽度减小。牙弓长度变化不明显。第一磨牙近中颊向扭转。腭部宽度增加,深度减小。头影测量结果提示:鼻腔宽度、上颌基骨宽度和上颌磨牙宽度稍有增加。结论唇腭裂自锁托槽腭扩展后上颌牙颌形态在横向宽度的变化较矢状向的变化明显。短期腭扩展治疗效果以牙齿移动和牙槽改建为主。%Objective To assess the short-term effects of maxillary palatal expansion using self-ligating brackets in patients with unilateral complete cleft lip and palate. Methods Five unilateral cleft lip and palate patients (UCLP) with severe maxillary constriction and without alveolar bone transplant were included in this study. Self-ligating brackets (AO, Time2) with superelastic nickel-titanium archwires were used during expan-sion. Tooth and jaws models and head positioning X-ray before treatment and after expansion for 6~17 months were gathered and measured to analyze the morphological changes of dental arch, alveolar bone, palatal vault, and jaw through digital cephalometric and model analysis methods. Result The model analysis demonstrated that a significant increase on arch width appeared especially at the area of first premolar, followed by the area of canine, and the area of first molar was the least, while the width of the area of second

  15. Exclusion of linkage between cleft lip with or without cleft palate and markers on chromosomes 4 and 6

    Energy Technology Data Exchange (ETDEWEB)

    Blanton, S.H. [Univ. of Virginia, Charlottesville, VA (United States); Malcolm, S.; Winter, R. [Institute of Child Health, London (United Kingdom)] [and others

    1996-01-01

    Nonsyndromic cleft lip with or without associate cleft palate (CLP) is a common craniofacial defect, occurring in {approximately}1/1,000 live births. While the defect generally occurs sporadically, multiplex families have been reported. Segregation analyses have demonstrated that, in some families, CLP is inherited as an autosomal dominant/codominant disorder with low penetrance. Several clefting loci have been proposed on multiple chromosomes, including 6p24, 4q, and 19q13.1. Association studies and linkage studies suggested a locus that mapped to 6p24. We were unable to confirm this in a linkage study of 12 multigenerational families. A subsequent linkage study by Carinci et al., however, found evidence for linkage to this region in 14 of 21 clefting families. Additionally, Davies et al. studied the chromosomes of three individuals with cleft lip and palate, all of whom had a rearrangement involving 6p24. Their investigation supported a locus at 6p24. Carinci et al. reported that the most likely position for a clefting locus was at D6S89, which is centromeric to EDN1. This is in contrast to the findings of Davies et al., who suggested a placement telomeric to EDN1. F13A, which had been implicated in the initial association studies, is telomeric to EDN1. Thus, the region between F13A and D6S89 encompasses the regions proposed by both Davies et al. and Carinci et al. A second clefting locus, at 4q, was proposed by Beiraghi et al., who studied a single multigenerational family by linkage analysis. Their data suggested a locus near D4S175 and D4S192. 10 refs., 1 tab.

  16. MRI findings of fetal cleft lip and palate

    International Nuclear Information System (INIS)

    Objective: To investigate the MR findings of fetal cleft lip (CL) and evaluate the advantages and limitations of MRI in the diagnosis. Methods: Twelve pregnant women suspicious of fetal CL/cleft palate (CP) on ultrasonography were enrolled in the study. The findings of ultrasonography, MRI and following-up were compared. Results: MRI and ultrasonography detected 12 fetuses with CL/CP. The following-up results showed 1 case with incomplete cleft lip and the other 11 cases with complete cleft lips and cleft palates. MRI and unltrasonography were consistent with the follow-up in CL detection, showing completed or uncompleted soft tissue interruption of the fetal lips with amniotic fluid filling which is high signal on T2WI. On MRI, CP showed discontinuous of the soft tissue which were interrupted by long T2 signal and communicating with oral cavity ad nasal cavity. MRI missed 1 case and excluded 1 case of CP. Ultrasonography predicted 5 case of CL, excluded 1 CP but missed 6 cases. The accuracy, sensitivity and specificity in detection CL/CP was 91.7% (11/12), 90.9% (10/11), 100% (1/1) for MRI and 50.0% (6/12), 45.5% (5/11), 100% (1/1) for ultrasonography, respectively. Conclusion: MR imaging had advantage over ultrasonography in detecting CP, MRI is an essential when CP is suspicious on ultrasonography. (authors)

  17. Facial soft-tissue asymmetry in three-dimensional cone-beam computed tomography images of children with surgically corrected unilateral clefts.

    Science.gov (United States)

    Starbuck, John Marlow; Ghoneima, Ahmed; Kula, Katherine

    2014-03-01

    Cleft lip with or without cleft palate (CL/P) is a relatively common craniofacial malformation involving bony and soft-tissue disruptions of the nasolabial and dentoalveolar regions. The combination of CL/P and subsequent craniofacial surgeries to close the cleft and improve appearance of the cutaneous upper lip and nose can cause scarring and muscle pull, possibly resulting in soft-tissue depth asymmetries across the face. We tested the hypothesis that tissue depths in children with unilateral CL/P exhibit differences in symmetry across the sides of the face. Twenty-eight tissue depths were measured on cone-beam computed tomography images of children with unilateral CL/P (n = 55), aged 7 to 17 years, using Dolphin software (version 11.5). Significant differences in tissue depth symmetry were found around the cutaneous upper lip and nose in patients with unilateral CL/P. PMID:24531247

  18. Evidence for an association between nonsyndromic cleft lip with or without cleft palate and a gene located on the long arm of chromosome 4

    Energy Technology Data Exchange (ETDEWEB)

    Mitchell, L.E.; Healey, S.C.; Chenevix-Trench, G. [St. Louis Univ. Health Sciences Center, MO (United States)]|[Queensland Institute of Medical Research, Brisbane (Australia)

    1995-11-01

    Recent studies suggest that the familial aggregation of nonsyndromic cleft lip with or without cleft palate (CL{+-}P) is likely to be attributable to the effects of several susceptibility loci, acting in a multiplicative fashion. Two potential CL{+-}P susceptibility loci (CSL), transforming growth factor alpha (TGFA) and retinoic acid receptor (RARA), have been identified through association studies. In addition, recent evidence of linkage between CL{+-}P and two markers (D4S175 and D4S192) in the region 4q25-4q31.3 raised the possibility that a CSL, with a larger effect than either TGFA or RARA, may reside within this region of the human genome. The present analyses were undertaken to determine whether D4S175 or D4S192 is significantly associated with CL{+-}P in a sample of unrelated patients that have previously provided evidence of associations between CL{+-}P and both TGFA and RARA. The results of these analyses provide further, tentative, evidence for the presence of a CSL locus on the long arm of chromosome 4 and help to refine the location of this locus in the region of D4S175 and D4S192. 28 refs., 4 tabs.

  19. Prosthodontic Management in Conjunction with Speech Therapy in Cleft Lip and Palate: A Review and Case Report

    OpenAIRE

    Dhakshaini, M R; M. Pushpavathi; Garhnayak, Mirna; Dhal, Angurbala

    2015-01-01

    Cleft lip and palate (CLP), a congenital disturbance needs a lot of attention with respect to its rehabilitation as a person suffering from this defect can lead a normal life given with the right type of treatment at the right time. However, if a patient cannot get surgical treatment at the appropriate time either due to availability or economic constraints, it is still possible to improve their social and psychological well-being with prosthodontic rehabilitation. The literature search was c...

  20. Theme and Information structure in French and English : A contrastive study of journalistic clefts

    OpenAIRE

    Carter-Thomas, Shirley

    2002-01-01

    In this article I will discuss factors motivating the use of the French c'est cleft structure, a marked-word order structure encountered with greater frequency in French discourse than the comparable it-cleft in English. Previous studies dealing with the English it-cleft, or predicated theme as it is usually termed in systemic linguistics, have generally sought to account for the structure in terms of contrastivness or specification, with the clefted element providing a certain thematic promi...