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Sample records for bilateral pulmonary agenesis

  1. Pulmonary agenesis: two cases reported

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    Denis Yaraví Solano-Vázquez

    2014-11-01

    Full Text Available Background: Pulmonary agenesis is a rare anomaly (1 in 15 000 live births which consists in a total absence or severe hypoplasia of one or both lungs. The clinical spectrum of the unilateral agenesis could vary from early and severe respiratory distress, recurrent pneumonia to being an incidental finding. The prognosis is based on the presence of associated congenital abnormalities. Material and methods: We present two cases of unilateral pulmonary agenesis in patients at Tlaxcala’s Children Hospital during 2012. Results: Report details the case of a one-month old boy with left pulmonary agenesis and interatrial communication and mild pulmonary arterial hypertension. He had two resolved pneumonia incidents. The other case was a one-month old girl with right pulmonary agenesis, associated to multiple heart malformations who evolved to respiratory failure, heart failure and death.Conclusions: Pulmonary agenesis is a rare anomaly. Its outcome and prognosis varies with the hemodynamics related to its location and associated malformations.

  2. [Congenital lumbar hernia and bilateral renal agenesis].

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    Barrero Candau, R; Garrido Morales, M

    2007-04-01

    We report a new case of congenital lumbar hernia. This is first case reported of congenital lumbar hernia and bilateral renal agenesis. We review literature and describe associated malformations reported that would be role out in every case of congenital lumbar hernia.

  3. Bilateral renal agenesis in an alpaca cria

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    Poulsen, Keith P; Gerard, Mathew P.; Spaulding, Kathy A.; Geissler, Kyleigh A.; Anderson, Kevin L.

    2006-01-01

    A 3-day-old male alpaca cria was presented for lack of vigor and failure to urinate since birth. Based on the history, laboratory data, ultrasonographs, surgical findings, and postmortem examination, the cria was diagnosed with bilateral renal agenesis and hypoplastic bladder, a congenital condition rarely seen in veterinary medicine.

  4. Bilateral agenesis of the anterior cruciate ligament: MRI evaluation

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    Bedoya, Maria A.; Jaramillo, Diego [The Children' s Hospital of Philadelphia, Radiology Department, Philadelphia, PA (United States); McGraw, Michael H. [Hospitalof theUniversityof Pennsylvania, Divisionof Orthopaedics, Philadelphia, PA (United States); Wells, Lawrence [The Children' s Hospital of Philadelphia, Division of Orthopaedics, Philadelphia, PA (United States)

    2014-09-15

    Bilateral agenesis of the anterior cruciate ligament (ACL) is extremely rare. We describe a 13-year-old girl who presented with bilateral knee pain without history of trauma; she has two family members with knee instability. Magnetic resonance imaging showed bilateral absence of the ACL, and medial posterior horn meniscal tears. Bilateral arthroscopic partial meniscectomy and anterior cruciate ligament reconstruction was performed. (orig.)

  5. Unilateral Pulmonary Agenesis and Gastric Duplication Cyst: A Rare Association

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    Amir Halilbasic

    2013-01-01

    Full Text Available Lung agenesis and gastric duplication cysts are both rare congenital anomalies. Gastric duplication cysts can present with nausea, vomiting, hematemesis, or vague abdominal pain. Unilateral pulmonary agenesis can present with respiratory distress which usually occurs due to retention of bronchial secretions and inflammations. We report the unique case of right pulmonary agenesis associated with gastric duplication cyst.

  6. Pulmonary agenesis and pulmonary sling anomaly in an infant with Down syndrome.

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    Takci, S; Yigit, S; Haliloglu, M; Boduroglu, K; Kiper, N

    2013-01-01

    Pulmonary agenesis is a rare congenital anomaly presenting with normal karyotype in most of the cases. Rarely pulmonary agenesis is associated with chromosomal abnormalities and other genetic disorders such as Oculo-auriculo-vertebral spectrum, VACTERL association and velo-cardio-facial syndrome. This report presents a patient with pulmonary agenesis, pulmonary sling anomaly and Down syndrome. PMID:24341150

  7. Isolated Left Pulmonary Artery Agenesis: A Case Report

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    Tansel Ansal Balcı

    2012-08-01

    Full Text Available Unilateral pulmonary artery agenesis without any cardiovascular malformation is a rare anomaly. We present the imaging findings of a patient who was diagnosed as isolated left pulmonary artery agenesis. A 27-year-old female patient was admitted to our hospital due to dyspnea during exercise for five years. Chest X-ray revealed minimally small left pulmonary hilum and left lung. She was admitted to our clinic with the suspicion of pulmonary artery pathology. Absent perfusion of the left lung with normal ventilation was visualized on scintigraphy. MDCT angiography of pulmonary arteries showed absent left main pulmonary artery with systemic collaterals around left hemithorax. Pulmonary artery agenesis can be asymptomatic and isolated until adulthood. Both scintigraphy and CT angiography images of pulmonary artery agenesis of a patient are rare in the literature. Pulmonary ventilation- perfusion scintigraphy can be used not only for pulmonary embolism but also pathologies involving pulmonary artery and its branches. (MIRT 2012;21:80-83

  8. Agenesia pulmonar unilateral Unilateral pulmonary agenesis

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    Maura Cavada Malcon

    2012-08-01

    Full Text Available A agenesia pulmonar é uma anomalia congênita rara. Relatamos um caso de um menino de 8 anos de idade com agenesia pulmonar à esquerda sem associação com outras malformações. O diagnóstico foi realizado por achados de imagem quando o paciente apresentou sintomas como tosse, sibilância e dispneia sem melhora do quadro clínico após evolução de 30 dias.Pulmonary agenesis is a rare congenital anomaly. We report the case of an 8-year-old boy with left lung agenesis, without any other congenital malformations. When the patient presented symptoms, including cough, wheezing, and dyspnea, with no clinical improvement after a period of 30 days, imaging studies were conducted and the diagnosis was made.

  9. Isolated Left Pulmonary Artery Agenesis: A Case Report

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    Tansel Ansal Balcı; Zehra Pınar Koç; Gamze Kırkıl; Ahmet Kürşad Poyraz

    2012-01-01

    Unilateral pulmonary artery agenesis without any cardiovascular malformation is a rare anomaly. We present the imaging findings of a patient who was diagnosed as isolated left pulmonary artery agenesis. A 27-year-old female patient was admitted to our hospital due to dyspnea during exercise for five years. Chest X-ray revealed minimally small left pulmonary hilum and left lung. She was admitted to our clinic with the suspicion of pulmonary artery pathology. Absent perfusion of the left lung w...

  10. Bilateral meandering pulmonary veins

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    Thupili, Chakradhar R.; Udayasankar, Unni [Pediatric Imaging, Imaging Institute Cleveland Clinic, Cleveland, OH (United States); Renapurkar, Rahul [Imaging Institute Cleveland Clinic, Thoracic Imaging, L10, Cleveland, OH (United States)

    2015-06-15

    Meandering pulmonary veins is a rare clinical entity that can be mistaken for more complex congenital syndromes such as hypogenetic lung syndrome. We report imaging findings in a rare incidentally detected case of bilateral meandering pulmonary veins. We briefly discuss the role of imaging in diagnosing this condition, with particular emphasis on contrast-enhanced CT. (orig.)

  11. Infrahepatic inferior vena cava agenesis with bilateral renal vein thrombosis.

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    Skeik, Nedaa; Wickstrom, Kelly K; Schumacher, Clark W; Sullivan, Timothy M

    2013-10-01

    Congenital anomalies of the inferior vena cava (IVC) are rare and are estimated to be present in 0.07-8.7% of the general population. IVC agenesis (IVCA) is found in approximately 5% of cases of unprovoked lower extremity deep vein thrombosis in patients Renal vein thrombosis (RVT) is an extremely rare and unusual presentation of IVCA. We report a unique case of a 23-year-old previously healthy man presenting with infrahepatic IVCA-induced bilateral RVT with azygos and hemiazygos continuation. To our knowledge, this is the third reported case in the literature of IVCA-induced RVT and the first to affect the bilateral renal veins in the absence of any other thrombogenic risk factors or any lower extremity venous complications. We also present a literature review of IVCA-induced vein thrombosis and highlight the lack of literature to manage this condition.

  12. Polyhydramnios in Lrp4 knockout mice with bilateral kidney agenesis: Defects in the pathways of amniotic fluid clearance

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    Hiroshi Tanahashi; Qing-Bao Tian; Yoshinobu Hara; Hiroyuki Sakagami; Shogo Endo; Tatsuo Suzuki

    2016-01-01

    Amniotic fluid volume during mid-to-late gestation depends mainly on the urine excretion from the foetal kidneys and partly on the fluid secretion from the foetal lungs during foetal breathing-like movements. Urine is necessary for foetal breathing-like movements, which is critical for foetal lung development. Bilateral renal agenesis and/or obstruction of the urinary tract lead to oligohydramnios, which causes infant death within a short period after birth due to pulmonary hypoplasia. Lrp4, ...

  13. Esophageal Atresia and Tracheoesophageal Fistula with Unilateral Pulmonary Agenesis - Hypoplasia

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    Katragadda Laxmi Narsimha Rao

    2013-04-01

    Full Text Available Association of unilateral severe pulmonary hypoplasia or agenesis and esophageal atresia (EA with or without tracheoesophageal fistula (TEF is an exceedingly rare and highly lethal combination. We report a case of full term male baby who had EA with TEF and right lung hypoplasia, managed at our centre. He is alive and doing well at 10 years of age.

  14. Bilateral internal carotid agenesis: value of CT angiography and correlation to embryogenesis

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    Pilleul, F.; Rouviere, O. [Dept. of Radiology, Hopital Edouard Herriot, Lyon (France); Guibaud, L.; Pracros, J.P. [Dept. of Pediatric Imaging, Hopital Debrousse, Lyon (France); Badinand, N. [Dept. of Pediatric Neurology, Hopital Debrousse, Lyon (France)

    2001-05-01

    Bilateral internal carotid artery agenesis is an uncommon disease, difficult to differentiate from bilateral carotid artery thrombosis. A few case reports have described the contribution of conventional angiogram to make the diagnosis and recognize the anatomic details of this rare malformation. Advantages of CT angiography as a non-invasive radiologic tool are discussed in this case report. (orig.)

  15. Bilateral agenesis of the superior vena cava associated with congenital hydrothorax.

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    Römer, S; Opgen-Rhein, B; Chaoui, R; Scheer, I; Czernik, C; Obladen, M

    2006-11-01

    Agenesis of the superior vena cava is a rare anomaly that is generally asymptomatic in the neonate. We report a male neonate with bilateral (total) agenesis of the superior vena cava with obstructed thoracic duct and subsequent congenital hydrothorax, anomalies that were detected by prenatal ultrasound at 25 weeks' gestation. The cardiac anomaly was confirmed by postnatal magnetic resonance angiography. The chylothorax disappeared with conservative therapy.

  16. Polyhydramnios in Lrp4 knockout mice with bilateral kidney agenesis: Defects in the pathways of amniotic fluid clearance.

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    Tanahashi, Hiroshi; Tian, Qing-Bao; Hara, Yoshinobu; Sakagami, Hiroyuki; Endo, Shogo; Suzuki, Tatsuo

    2016-01-01

    Amniotic fluid volume during mid-to-late gestation depends mainly on the urine excretion from the foetal kidneys and partly on the fluid secretion from the foetal lungs during foetal breathing-like movements. Urine is necessary for foetal breathing-like movements, which is critical for foetal lung development. Bilateral renal agenesis and/or obstruction of the urinary tract lead to oligohydramnios, which causes infant death within a short period after birth due to pulmonary hypoplasia. Lrp4, which functions as an agrin receptor, is essential for the formation of neuromuscular junctions. Herein, we report novel phenotypes of Lrp4 knockout (Lrp4(-/-)) mice. Most Lrp4(-/-) foetuses showed unilateral or bilateral kidney agenesis, and Lrp4 knockout resulted in polyhydramnios. The loss of Lrp4 compromised foetal swallowing and breathing-like movements and downregulated the expression of aquaporin-9 in the foetal membrane and aquaporin-1 in the placenta, which possibly affected the amniotic fluid clearance. These results suggest that amniotic fluid removal was compromised in Lrp4(-/-) foetuses, resulting in polyhydramnios despite the impairment of urine production. Our findings indicate that amniotic fluid removal plays an essential role in regulating the amniotic fluid volume.

  17. Polyhydramnios in Lrp4 knockout mice with bilateral kidney agenesis: Defects in the pathways of amniotic fluid clearance.

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    Tanahashi, Hiroshi; Tian, Qing-Bao; Hara, Yoshinobu; Sakagami, Hiroyuki; Endo, Shogo; Suzuki, Tatsuo

    2016-01-01

    Amniotic fluid volume during mid-to-late gestation depends mainly on the urine excretion from the foetal kidneys and partly on the fluid secretion from the foetal lungs during foetal breathing-like movements. Urine is necessary for foetal breathing-like movements, which is critical for foetal lung development. Bilateral renal agenesis and/or obstruction of the urinary tract lead to oligohydramnios, which causes infant death within a short period after birth due to pulmonary hypoplasia. Lrp4, which functions as an agrin receptor, is essential for the formation of neuromuscular junctions. Herein, we report novel phenotypes of Lrp4 knockout (Lrp4(-/-)) mice. Most Lrp4(-/-) foetuses showed unilateral or bilateral kidney agenesis, and Lrp4 knockout resulted in polyhydramnios. The loss of Lrp4 compromised foetal swallowing and breathing-like movements and downregulated the expression of aquaporin-9 in the foetal membrane and aquaporin-1 in the placenta, which possibly affected the amniotic fluid clearance. These results suggest that amniotic fluid removal was compromised in Lrp4(-/-) foetuses, resulting in polyhydramnios despite the impairment of urine production. Our findings indicate that amniotic fluid removal plays an essential role in regulating the amniotic fluid volume. PMID:26847765

  18. Adult patient with pulmonary agenesis: focusing on one-lung ventilation during general anesthesia

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    Yu, Yuetian; Zhu, Cheng; QIAN, XIAOZHE; Gao, Yuan; Zhang, Zhongheng

    2016-01-01

    Congenital pulmonary agenesis is a rare condition with high mortality. Mechanical ventilation in these patients is challenging and there has no such case been reported in the literature. We reported a 61-year-old female with lung agenesis who presented to our hospital with pneumonia and pelvic mass. In the past, she had undergone repairing of atrial septal defect and mitral valve forming surgery at 6-year-old. Thereafter she had remained asymptomatic until this time of hospital admission. The...

  19. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

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    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Mansfield, L. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Redmond, K.C. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Dusmet, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Goldstraw, P. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Mittal, T.K. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-03-15

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution.

  20. Pulmonary Artery Agenesis Associated With Emphysema and Multiple Invasive Non-Small Cell Lung Cancers.

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    Makdisi, George; Edell, Eric S; Maleszewski, Joseph J; Molina, Julian R; Deschamps, Claude

    2015-06-01

    Pulmonary artery (PA) agenesis in the absence of associated cardiac abnormalities is a rare congenital abnormality. It may remain undiagnosed until adulthood when patients present with respiratory symptoms such as hemoptysis, dyspnea, repeated respiratory infections, or pulmonary hypertension. Herein we present a case of a 50-year-old woman who was found to have multiple, morphologically distinct non-small cell lung cancers in association with agenesis of the PA. This instance represents the fourth reported case of such association in the English literature. PMID:26046873

  1. Implant-Prosthetic Rehabilitation in Bilateral Agenesis of Maxillary Lateral Incisors with a Mini Split Crest

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    Figliuzzi, M. M.; Giudice, A.; Pileggi, S.; Pacifico, D.; Marrelli, M.; Tatullo, M.; Fortunato, L.

    2016-01-01

    The reported clinical case describes the surgical procedure of ridge augmentation by using a “split crest” technique with a partial thickness flap and a subsequent implant-prosthetic rehabilitation aimed at treating a bilateral agenesis of the upper lateral incisors. In such cases with vestibule-palatal and mesial-distal scarce bone thicknesses associated with the need of a proper functional and aesthetic rehabilitation, the split crest technique is particularly suitable. In the case we reported, because of the poor bone thicknesses, we performed a minimally invasive split crest which allowed a correct insertion of the fixtures. This technique allowed us to achieve an optimal functional and aesthetic rehabilitation; moreover, we obtained a good emergency profile, ensuring the vitality of the close teeth and ensuring a good primary stability and the following osseointegration of dental implants. PMID:27190658

  2. Implant-Prosthetic Rehabilitation in Bilateral Agenesis of Maxillary Lateral Incisors with a Mini Split Crest

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    M. M. Figliuzzi

    2016-01-01

    Full Text Available The reported clinical case describes the surgical procedure of ridge augmentation by using a “split crest” technique with a partial thickness flap and a subsequent implant-prosthetic rehabilitation aimed at treating a bilateral agenesis of the upper lateral incisors. In such cases with vestibule-palatal and mesial-distal scarce bone thicknesses associated with the need of a proper functional and aesthetic rehabilitation, the split crest technique is particularly suitable. In the case we reported, because of the poor bone thicknesses, we performed a minimally invasive split crest which allowed a correct insertion of the fixtures. This technique allowed us to achieve an optimal functional and aesthetic rehabilitation; moreover, we obtained a good emergency profile, ensuring the vitality of the close teeth and ensuring a good primary stability and the following osseointegration of dental implants.

  3. Uterine Didelphys with blind hemivagina, hematocolpos, ipsilateral renal agenesis (UD-BHRA) and bilateral ovarian tumors : a case report

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    Lee, Young Rae; Choi, Byung Ik; Park, Hae Won; Kook, Shin Ho [Kangbuk Samsung Hospital, Seoul (Korea, Republic of)

    1998-03-01

    Uterine didelphys is a congenital malformation characterized by the presence of two separated hemiuteri and hemivaginas, due to lack of midfusion of the mullerian ducts. We report a case of UD-BHRA (uterine didelphys with blind hemivagina and ipsilateral renal agencies), a rare type of uterine dedelphys characterized by symptomatic unilateral hematocolpos due to blind hemivagina after menarche and ipsilateral renal agenesis. The MRI findings in 22-year-old woman with bilateral ovarian tumors demonstrated two separated uterine horns and cervical and vaginal canals, with left hematocolpos and left agenesis. (author). 7 refs., 1 fig.

  4. Uterine Didelphys with blind hemivagina, hematocolpos, ipsilateral renal agenesis (UD-BHRA) and bilateral ovarian tumors : a case report

    International Nuclear Information System (INIS)

    Uterine didelphys is a congenital malformation characterized by the presence of two separated hemiuteri and hemivaginas, due to lack of midfusion of the mullerian ducts. We report a case of UD-BHRA (uterine didelphys with blind hemivagina and ipsilateral renal agencies), a rare type of uterine dedelphys characterized by symptomatic unilateral hematocolpos due to blind hemivagina after menarche and ipsilateral renal agenesis. The MRI findings in 22-year-old woman with bilateral ovarian tumors demonstrated two separated uterine horns and cervical and vaginal canals, with left hematocolpos and left agenesis. (author). 7 refs., 1 fig

  5. Male Fertility after Spermatocele Formation from Tunica Vaginalis in Patients with Bilateral Vas Agenesis

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    Shamsa Ali

    2008-01-01

    Full Text Available To form spermatocele from vaginal layers as a sperm reservoir and intra-uterine insemination (IUI in infertile men with bilateral vas agenesis (BVA, we studied 19 patients with azoospermia due to BVA referred to our infertility clinic from March 1992 until May 2003. The ages of the patients ranged from 20-41 (mean 29.6 ± 5.8 years. After physical examination, hormone assay, testis biopsy, and confirming normal spermatogenesis, we have performed 23 alloplastic spermatoceles from the tunica vaginal layers in 11 patients. We retrieved sperms and performed IUI in 6 patients′ wives 3 months post-operation when scrotal sonography revealed spermatocele with a good volume of seminal liquid. Among 6 patients′ wives, 2 successful preg-nancies occurred, and 2 normal babies (one boy with normal bilateral vas and one girl were delivered successfully by cesarean section. We conclude that although the method of choice for fer-tility in BVA in artificial reproductive therapy era is percutaneous epididymal sperm aspiration (PESA and intracytoplasmic sperm injection (ICSI, but when the sophisticated facilities are not available or cost-effectiveness is matter of concern, alloplastic spermatocele from tunica vaginalis and IUI may be a viable option.

  6. Bilateral spontaneous pneumothorax with pulmonary metastases of synovial sarcoma

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    The association of bilateral spontaneous pneumothorax with pulmonary tumor is uncommon and with pulmonary metastases is rare. The clinical and radiological features of bilateral spontaneous pneumothorax from a synovial sarcoma in a 14 years old boy are described. (author)

  7. Bilateral stony lung: pulmonary alveolar microlithiasis.

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    Chandra, Subhash; Mohan, Anant; Guleria, Randeep; Das, Prasenjit; Sarkar, Chitra

    2009-01-01

    A 40-year-old male paddy field worker was referred for exertional shortness of breath and non-productive cough for 4 years. He had been treated for pulmonary tuberculosis twice. Chest radiograph showed extensive bilateral nodular opacities ("sandstorm-like") in the middle and lower lobe. Pulmonary function tests revealed a restrictive ventilatory defect. High resolution CT showed widespread nodular infiltration with "crazy paving" appearance and interrupted black pleura sign. This was confirmed as pulmonary alveolar microlithiasis (PAM) by trans-bronchial lung biopsy, which showed normal respiratory lining epithelium with dilated alveolar spaces containing many calcific bodies, some of which showed concentric calcification. The possibilities of silicosis (due to exposure to husk) and tuberculosis, both of which can mimic PAM clinically and radiologically, made this case a diagnostic challenge. PMID:21686505

  8. Bilateral pulmonary sequestration: a case report

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    Koo, Ja Hong; Jeon, Eui Yong; Seo, Heung Suk; Choi, Yo Won; Jeon, Seok Chol; Hahm, Chang Kok [College of Medicine, Hanyang University, Seoul (Korea, Republic of)

    1995-05-15

    A 21-year-old woman presented with productive cough and hemoptysis. Chest radiograph shows a large thin-walled cystic lesion with an air-fluid level in medial portion of the left lower lung zone and opacity in paravertebral area of the right lower lung zone. Chest CT scan shows a thin-walled cavitary lesion in the posterior basal segment of left lower lobe with an air-fluid level. Area of consolidation containing air-filled cysts was also observed in medial aspect of right lower lobe. Selective angiogram obtained from aberrant artery arising from descending abdominal aorta showed two main branches of the artery supplying bilateral pulmonary lesions.

  9. Bilateral renal agenesis/hypoplasia/dysplasia (BRAHD: postmortem analysis of 45 cases with breakpoint mapping of two de novo translocations.

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    Louise Harewood

    Full Text Available BACKGROUND: Bilateral renal agenesis/hypoplasia/dysplasia (BRAHD is a relatively common, lethal malformation in humans. Established clinical risk factors include maternal insulin dependent diabetes mellitus and male sex of the fetus. In the majority of cases, no specific etiology can be established, although teratogenic, syndromal and single gene causes can be assigned to some cases. METHODOLOGY/PRINCIPAL FINDINGS: 45 unrelated fetuses, stillbirths or infants with lethal BRAHD were ascertained through a single regional paediatric pathology service (male:female 34:11 or 3.1:1. The previously reported phenotypic overlaps with VACTERL, caudal dysgenesis, hemifacial microsomia and Müllerian defects were confirmed. A new finding is that 16/45 (35.6%; m:f 13:3 or 4.3:1 BRAHD cases had one or more extrarenal malformations indicative of a disoder of laterality determination including; incomplete lobulation of right lung (seven cases, malrotation of the gut (seven cases and persistence of the left superior vena cava (five cases. One such case with multiple laterality defects and sirelomelia was found to have a de novo apparently balanced reciprocal translocation 46,XY,t(2;6(p22.3;q12. Translocation breakpoint mapping was performed by interphase fluorescent in-situ hybridization (FISH using nuclei extracted from archival tissue sections in both this case and an isolated bilateral renal agenesis case associated with a de novo 46,XY,t(1;2(q41;p25.3. Both t(2;6 breakpoints mapped to gene-free regions with no strong evidence of cis-regulatory potential. Ten genes localized within 500 kb of the t(1;2 breakpoints. Wholemount in-situ expression analyses of the mouse orthologs of these genes in embryonic mouse kidneys showed strong expression of Esrrg, encoding a nuclear steroid hormone receptor. Immunohistochemical analysis showed that Esrrg was restricted to proximal ductal tissue within the embryonic kidney. CONCLUSIONS/SIGNIFICANCE: The previously unreported

  10. Analysis of width/height ratio and gingival zenith in patients with bilateral agenesis of maxillary lateral incisor

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    Núbia Inocencya Pavesi Pini

    2012-10-01

    Full Text Available OBJECTIVE: The purpose of this study was to evaluate the width/length ratio and the gingival zenith (GZ, by means of dental casts and digital caliper, in patients with missing maxillary lateral incisors after treatment. METHODS: The sample was composed of 52 subjects divided into 3 groups: BRG (n = 18, patients with bilateral agenesis treated with tooth re-contouring; BIG (n = 10 patients with agenesis treated with implants and CG (n = 24, control group. The data were analyzed using Shapiro-Wilk, Spearman correlation, Wilcoxon, Kruskal-Wallis, t test and ANOVA tests (p 0.05. GZ data for the right and left sides of the smile were not considered statistically different. CONCLUSION: Although no statistical difference was found in the comparison between the groups, analysis of the descriptive values showed that group BIG showed the greatest difference in values with regard to width/length ratio. Regarding gingival zenith, BRG showed the greatest difference.OBJETIVO: o propósito desse estudo foi analisar, por meio de modelos de estudo e paquímetro digital, a proporção largura/altura e o zênite gengival (ZG em pacientes com agenesia bilateral do incisivo lateral superior após o tratamento. MÉTODOS: a amostra consistiu de 52 voluntários divididos em 3 grupos: GBR (n=18, pacientes com agenesia bilateral tratados com reanatomizações dentárias; GBI (n=10, pacientes com agenesia bilateral tratados com implantes; e GC (n=24, grupo controle. Os dados foram avaliados por meio dos testes de Shapiro-Wilk, Wilcoxon, Kruskal-Wallis, teste t, ANOVA (p0,05. Os valores obtidos para os lados direito e esquerdo foram considerados iguais dentro de cada grupo. CONCLUSÃO: embora não tenham sido encontradas diferenças estatísticas na comparação entre os grupos, pela análise descritiva dos dados, o GBI foi o grupo que apresentou as medidas mais destoantes dos demais em relação à proporção largura/altura, sendo que, para o zênite gengival, a maior

  11. Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis

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    Go Miyano

    2015-01-01

    Full Text Available We describe herein a case of unilateral pulmonary agenesis (PA with oesophageal atresia (EA/tracheoesophageal fistula (TEF that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

  12. Prenatal Diagnosis of Bilateral Ectrodactyly and Radial Agenesis Associated with Trisomy 10 Mosaicism

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    Jonathan Lévy; Jean-Marie Jouannic; Julien Saada; Ferdinand Dhombres; Jean-Pierre Siffroi; Marie-France Portnoï

    2013-01-01

    Ectrodactyly or split hand and foot malformations (SHFMs) are rare malformations of the limbs, characterized by median clefts of the hands and feet, syndactyly, and aplasia and/or hypoplasia of the phalanges. They represent a clinically and genetically heterogeneous disorder, with both sporadic and familial cases. Most of the genomic rearrangements identified to date in some forms of SHFM are autosomal dominant traits, involving various chromosome regions. Bilateral radial ray defects compris...

  13. Prenatal Diagnosis of Bilateral Ectrodactyly and Radial Agenesis Associated with Trisomy 10 Mosaicism

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    Jonathan Lévy

    2013-01-01

    Full Text Available Ectrodactyly or split hand and foot malformations (SHFMs are rare malformations of the limbs, characterized by median clefts of the hands and feet, syndactyly, and aplasia and/or hypoplasia of the phalanges. They represent a clinically and genetically heterogeneous disorder, with both sporadic and familial cases. Most of the genomic rearrangements identified to date in some forms of SHFM are autosomal dominant traits, involving various chromosome regions. Bilateral radial ray defects comprise also a large heterogenous group of disorders, including trisomy 18, Fanconi anemia, and thrombocytopenia-absent-radius syndrome, not commonly associated with ectrodactyly. The present paper describes a case of ectrodactyly associated with bilateral radial ray defects, diagnosed in the first trimester of pregnancy, in a fetus affected by trisomy 10. Only four cases of sporadic and isolated ectrodactyly, diagnosed by ultrasonography between 14 and 22 weeks’ gestation, have been reported. To our knowledge, the present case is the first report of mosaic trisomy 10 associated with SHFM and radial aplasia. Trisomy 10 is a rare lethal chromosomal abnormality, most frequently found in abortion products. Only six liveborn mosaic trisomy 10 infants, with severe malformations, dead in early infancy, have been reported. A severe clinical syndrome can be defined, comprising ear abnormalities, cleft lip/palate, malformations of eyes, heart, and kidneys, and deformity of hands and feet and most often associated with death neonatally or in early infancy.

  14. Prenatal diagnosis of bilateral ectrodactyly and radial agenesis associated with trisomy 10 mosaicism.

    Science.gov (United States)

    Lévy, Jonathan; Jouannic, Jean-Marie; Saada, Julien; Dhombres, Ferdinand; Siffroi, Jean-Pierre; Portnoï, Marie-France

    2013-01-01

    Ectrodactyly or split hand and foot malformations (SHFMs) are rare malformations of the limbs, characterized by median clefts of the hands and feet, syndactyly, and aplasia and/or hypoplasia of the phalanges. They represent a clinically and genetically heterogeneous disorder, with both sporadic and familial cases. Most of the genomic rearrangements identified to date in some forms of SHFM are autosomal dominant traits, involving various chromosome regions. Bilateral radial ray defects comprise also a large heterogenous group of disorders, including trisomy 18, Fanconi anemia, and thrombocytopenia-absent-radius syndrome, not commonly associated with ectrodactyly. The present paper describes a case of ectrodactyly associated with bilateral radial ray defects, diagnosed in the first trimester of pregnancy, in a fetus affected by trisomy 10. Only four cases of sporadic and isolated ectrodactyly, diagnosed by ultrasonography between 14 and 22 weeks' gestation, have been reported. To our knowledge, the present case is the first report of mosaic trisomy 10 associated with SHFM and radial aplasia. Trisomy 10 is a rare lethal chromosomal abnormality, most frequently found in abortion products. Only six liveborn mosaic trisomy 10 infants, with severe malformations, dead in early infancy, have been reported. A severe clinical syndrome can be defined, comprising ear abnormalities, cleft lip/palate, malformations of eyes, heart, and kidneys, and deformity of hands and feet and most often associated with death neonatally or in early infancy. PMID:23401811

  15. Successful radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia in a patient with dextrocardia due to unilateral pulmonary agenesis: a case report

    Directory of Open Access Journals (Sweden)

    Aksu T

    2015-02-01

    Full Text Available Tolga Aksu, Tumer Erdem Guler, Ebru Golcuk, Ismail Erden, Kazim Serhan Ozcan Department of Cardiology, Kocaeli Derince Education and Research Hospital, Derince, Kocaeli, Turkey Abstract: Radiofrequency catheter ablation of the slow pathway is considered to be the treatment of choice for patients with atrioventricular nodal reentrant tachycardia. We report a 34-year-old female with mirror image dextrocardia due to unilateral pulmonary agenesis who underwent successful slow pathway ablation for typical atrioventricular nodal reentrant tachycardia. Using contrast injection, cardiac anatomy was identified in a short time and successfully ablated. Keywords: dextrocardia, AVNRT, ablation, pulmonary agenesis

  16. Bilateral pulmonary emboli after bilateral mastectomy in a 15-year-old boy with hypogonadism: A case report

    OpenAIRE

    Piggott, JR; Yazdani, Arjang

    2010-01-01

    Pulmonary emboli are rare, yet serious, complications of body contouring surgery. When they occur, they more often follow as complications of long, invasive procedures in adults. The present report details a case of bilateral pulmonary emboli in an obese 15-year-old boy with hypogonadism undergoing bilateral mastectomy for gynecomastia. The diagnosis of bilateral pulmonary emboli was made on the basis of clinical presentation and positive ventilation/perfusion scan. The patient responded well...

  17. Postmortem MRI of bladder agenesis

    Energy Technology Data Exchange (ETDEWEB)

    Barber, Brendan R. [St George' s Hospital, Radiology Department, London (United Kingdom); Weber, Martin A. [Great Ormond Street Hospital for Children, Department of Histopathology, London (United Kingdom); Bockenhauer, Detlef [Great Ormond Street Hospital for Children, Department of Nephrology, London (United Kingdom); Hiorns, Melanie P.; McHugh, Kieran [Great Ormond Street Hospital for Children, Radiology Department, London (United Kingdom)

    2011-01-15

    We report a 35-week preterm neonate with bladder agenesis and bilateral dysplastic kidneys. A suprapubic catheter was inadvertently inserted into one of the larger inferior cysts of the left dysplastic kidney. A postmortem MRI scan was performed with the findings being confirmed on autopsy. We are unaware of another postmortem MRI study demonstrating bladder agenesis. (orig.)

  18. Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo; Park, Sang-Hyub; Koo, Hyun Jung; Cho, Young Hoon; Lee, Eunsol [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea, Republic of)

    2014-08-15

    Imaging findings of bilateral pulmonary vein atresia have not been described. To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia. Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes. All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days. Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children. (orig.)

  19. Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

    International Nuclear Information System (INIS)

    Imaging findings of bilateral pulmonary vein atresia have not been described. To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia. Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes. All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days. Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children. (orig.)

  20. An Unusual Radiologic Manifestation of Pulmonary Tuberculosis with Bilateral Multiple Lung Nodules and Diffuse Alveolar Hemorrhage: A Case Report

    International Nuclear Information System (INIS)

    Pulmonary tuberculosis presenting as bilateral multiple lung nodules or diffuse alveolar hemorrhage is very rare. Here, we report a case of pulmonary tuberculosis presenting as bilateral multiple lung nodules and diffuse alveolar hemorrhage mimicking granulomatous vasculitis, such as Wegener's granulomatosis.

  1. Case Report: Bilateral reexpansion pulmonary edema following treatment of a unilateral hemothorax

    OpenAIRE

    Steven P de Wolf; Deunk, Jaap; Cornet, Alexander D.; Elbers, Paul WG; Farag, Ehab; Coetzee, André

    2014-01-01

    Bilateral re-expansion pulmonary edema (RPE) is an extremely rare entity. We report the unique case of bilateral RPE following a traumatic, unilateral hemopneumothorax in a young healthy male. Bilateral RPE occurred only one hour after drainage of a unilateral hemopneumothorax. The patient was treated with diuretics and supplemental oxygen. Diagnosis was confirmed by excluding other causes, using laboratory findings, chest radiography, pulmonary and cardiac ultrasound and high resolution comp...

  2. Penile agenesis

    OpenAIRE

    Bangroo A; Khetri Ramji; Tiwari Sashi

    2005-01-01

    Penile agenesis is an extremely rare disorder with profound urological and psychological consequences. The goal of treatment is an early female gender assignment and feminizing reconstruction of the perineum.

  3. Case Report: Bilateral reexpansion pulmonary edema following treatment of a unilateral hemothorax.

    Science.gov (United States)

    de Wolf, Steven P; Deunk, Jaap; Cornet, Alexander D; Elbers, Paul Wg

    2014-01-01

    Bilateral re-expansion pulmonary edema (RPE) is an extremely rare entity. We report the unique case of bilateral RPE following a traumatic, unilateral hemopneumothorax in a young healthy male. Bilateral RPE occurred only one hour after drainage of a unilateral hemopneumothorax. The patient was treated with diuretics and supplemental oxygen. Diagnosis was confirmed by excluding other causes, using laboratory findings, chest radiography, pulmonary and cardiac ultrasound and high resolution computed tomography. His recovery was uneventful. The pathophysiology of bilateral RPE is not well known. Treatment is mainly supportive and consists of diuretics, mechanical ventilation, inotropes and steroids. In case of a pulmonary deterioration after the drainage of a traumatic pneumothorax, bilateral RPE should be considered after exclusion of more common causes of dyspnea. PMID:25713699

  4. Pulmonary Langerhans cell histiocytosis causing spontaneous bilateral pneumothorax in a child

    OpenAIRE

    Anupam Patra; Sujit K Bhattacharyay; Arnab Maji; Abhijit Mandal

    2015-01-01

    Bilateral pneumothorax is very rare in childhood. Moreover, if it is due to pulmonary involvement of Langerhans cell histiocytosis, it is even rarer in childhood. In our case, a nonsmoker 12-year-old boy presented with bilateral pneumothorax, whose high-resolution computed tomography scan was highly suggestive of pulmonary Langerhans cell histiocytosis. Excision biopsy of a clinically palpable cervical lymph node and histopathological examination and immunohistochemistry positivity for CD1a i...

  5. 53. Bilateral ductal stenting for nonconfluent pulmonary arteries in a newborn

    Directory of Open Access Journals (Sweden)

    K. Al Dhahri

    2016-07-01

    Full Text Available Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Limited data available for bilateral PDA stenting. Bilateral PDA stenting in nonconfluent pulmonary arteries is challenging procedure but can be considered as an option in the management of complex conditions like this. 12 days old Preterm (36 weeks gestation male baby with birth weight of 2.6 kg developed respiratory distress with severe cyanosis and desaturation upto 50%. Baby was intubated and started on Prostaglandin 0.05 mic/kg/mt. His saturation improved to 80%. Echocardiogram showed complex cyanotic heart disease, Situs ambiguous, dextrocardia, complete unbalanced AV septal defect, pulmonary atresia , nonconfluent small branch pulmonary arteries supplied by the bilateral patent ductus arteriosus (PDA from right aortic arch and all four pulmonary veins form a confluence and drain into superior vena cava(SVC through vertical vein with no obstruction. Baby was taken up for PDA stenting. descending aortogram showed right aortic arch with vertical tortuous duct to right pulmonary artery (RPA and another short duct with acute angle from left subclavian artery to left pulmonary artery (LPA . Both ducti stented with coronary stents. Vertical vein angiogram showed both lungs drain to a confluence and then to SVC via ascending vertical vein with no obstruction. After stenting lung perfusion improved and the baby was stable and maintained 80% saturation on room air. Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Our case is unique with Heterotaxy, TAPVC, Dextrocardia and double ducti. Eventhough bilateral ductal stenting is technically challenging it is successful through femoral artery approach.

  6. Pulmonary Langerhans cell histiocytosis causing spontaneous bilateral pneumothorax in a child

    Directory of Open Access Journals (Sweden)

    Anupam Patra

    2015-01-01

    Full Text Available Bilateral pneumothorax is very rare in childhood. Moreover, if it is due to pulmonary involvement of Langerhans cell histiocytosis, it is even rarer in childhood. In our case, a nonsmoker 12-year-old boy presented with bilateral pneumothorax, whose high-resolution computed tomography scan was highly suggestive of pulmonary Langerhans cell histiocytosis. Excision biopsy of a clinically palpable cervical lymph node and histopathological examination and immunohistochemistry positivity for CD1a indicated a diagnosis of Langerhans cell histiocytosis. Clinicians should consider pulmonary Langerhans cell histiocytosis in differential diagnoses in dealing such a case.

  7. Pulmonary Langerhans Cell Histiocytosis X Presented with Bilateral Pneumothorax: A Case Report

    OpenAIRE

    Majed Al-Mourgi

    2014-01-01

    Background: The report describes the case of a 20-year-old Saudi male patient with Pulmonary Langerhans cell histiocytosis X (PLCH). He presented with bilateral chest pain of few hours duration starting first on the right side. The patient had also dyspnea and non productive cough. The patient was heavy smoker for 6 years. General examination revealed that the patient had tachypnea but he was hemodynamically stable. Chest examination showed bilateral distant breath sounds but no hyperresonanc...

  8. Chondroblastoma with pulmonary metastasis in a patient presenting with spontaneous bilateral pneumothorax: Report of a case.

    Science.gov (United States)

    Tamura, Masaya; Oda, Makoto; Matsumoto, Isao; Sawada-Kitamura, Seiko; Watanabe, Go

    2011-10-01

    Chondroblastoma is a benign bone tumor with a relatively high incidence in older children and adolescents. Although it is generally regarded as a benign neoplasm, it sometimes grows aggressively or recurs but rarely metastasizes to the lung. We herein present a very rare case of a bilateral pneumothorax due to a pulmonary metastasis from a chondroblastoma. A 21-year-old man developed a bilateral pneumothorax 20 months after an operation for a chondroblastoma of the right ischium. A pertinent literature review revealed similar cases of chondroblastoma with pulmonary metastasis, but revealed no reports of a pneumothorax caused by a metastatic chondroblastoma.

  9. Single-stage bilateral pulmonary resections by video-assisted thoracic surgery for multiple small nodules

    Science.gov (United States)

    Yao, Feng; Yang, Haitang

    2016-01-01

    Background Surgical treatment is thought to be the most effective strategy for multiple small nodules. However, in general, one-stage bilateral resection is not recommended due to its highly invasive nature. Methods Clinical records of patients undergoing one-stage bilateral resections of multiple pulmonary nodules between January 2009 and September 2014 in a single institution were retrospectively reviewed. Results Simultaneous bilateral pulmonary resection by conventional video-assisted thoracic surgery (VATS) was undertaken in 29 patients. Ground glass opacity (GGO) accounted for 71.9% (46/64) of total lesions, including 26 pure GGO and 20 mixed GGO lesions. One case underwent bilateral lobectomy that was complicated by postoperative dyspnea. Lobar-sublobar (L/SL) resection and bilateral sublobar resection (SL-SL) were conducted in 16 and 12 cases, respectively, and most of these cases had uneventful postoperative courses. There was no significant difference with regard to postoperative complications (P=0.703), duration of use of chest drains (P=0.485), between one- and two-stage groups. Mean postoperative follow-up in cases of primary lung cancer was 31.4 (range, 10–51) months. There was neither recurrence nor deaths at final follow-up. Conclusions Single-stage bilateral surgery in selected cases with synchronous bilateral multiple nodules (SBMNs) is feasible and associated with satisfactory outcomes. PMID:27076942

  10. Compression of the Right Pulmonary Artery by a Massive Descending Aortic Aneurysm Causing Bilateral Perfusion Defects on Pulmonary Scintigraphy

    OpenAIRE

    Makis, William; Derbekyan, Vilma

    2011-01-01

    A 67-year-old woman, who presented with a 2 month history of dyspnea, had a ventilation and perfusion lung scan that showed absent perfusion of the entire right lung with normal ventilation, as well as a rounded matched defect in the left lower lung adjacent to midline, suspicious for an aortic aneurysm or dissection. CT pulmonary angiography revealed a massive descending aortic aneurysm compressing the right pulmonary artery as well as the left lung parenchyma, accounting for the bilateral p...

  11. PULMONARY LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS SIMULTANEOUS BILATERAL SPONTANEOUS PNEUMOTHORAX IN A NON-SMOKER PATIENT

    OpenAIRE

    M. Vaziri; A. Pazooki L. Zahedi

    2008-01-01

    Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was ...

  12. Chronic necrotizing pulmonary aspergillosis presenting as bilateral pleural effusion: a case report

    Directory of Open Access Journals (Sweden)

    Rajalingham Sakthiswary

    2012-02-01

    Full Text Available Abstract Introduction Chronic necrotizing pulmonary aspergillosis is an uncommon subacute form of Aspergillus infection. It typically occurs in immunocompromised individuals and in those with underlying lung disease. This interesting case highlights the occurrence of this entity of aspergillosis in an immunocompetent middle-aged woman with atypical radiological findings. To the best of our knowledge this is the first case report of chronic necrotizing pulmonary aspergillosis presenting with pleural effusion. Case presentation Our patient was a 64-year-old Malay woman with a background history of epilepsy but no other comorbidities. She was a lifelong non-smoker. She presented to our facility with a six-month history of productive cough and three episodes of hemoptysis. An initial chest radiograph showed bilateral pleural effusion with bibasal consolidation. Bronchoscopy revealed a white-coated endobronchial tree and bronchoalveolar lavage culture grew Aspergillus niger. A diagnosis of chronic necrotizing pulmonary aspergillosis was made based on the clinical presentation and microbiological results. She responded well to treatment with oral itraconazole. Conclusions The radiological findings in chronic necrotizing pulmonary aspergillosis can be very diverse. This case illustrates that this condition can be a rare cause of bilateral pleural effusion.

  13. Bilateral pulmonary edema after endoscopic sympathectomy in a patient with glucose-6-phosphate dehydrogenase deficiency.

    Science.gov (United States)

    Lan, C J; Luk, H N; Wu, C T; Chang, W K; Tsou, M Y; Lui, P W; Lee, T Y

    2001-01-01

    Transaxillary endoscopic sympathectomy of thoracic ganglia (T2-T3) has recently gained wider acceptance as the treatment of choice for palmar hyperhidrosis. It requires one-lung ventilation to facilitate the surgery. One-lung ventilation, however, is not without complications, among which acute pulmonary edema has been reported. In this case report, we present a patient with palmar hyperhidrosis complicated by glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, who received bilateral endoscopic sympathectomy under alternate one-lung anesthesia, and developed acute pulmonary edema immediately after recruitment of the successive collapsed lung. The effects of hypoxemia, G-6-PD deficiency and sympathectomy might all add to the development of acute pulmonary edema secondary to reexpansion of each individual lung after alternate one-lung ventilation. The possibilities of the inferred causes are herein discussed. PMID:11152024

  14. Mini-invasive resection and collapse therapy in patients with bilateral pulmonary tuberculosis

    Directory of Open Access Journals (Sweden)

    Korpusenko I.V.

    2015-06-01

    Full Text Available Objective. Improve the effectiveness of surgical treatment in patients with bilateral destructive pulmonary tuberculosis by mini-invasive resection and collapse therapy. Materials and Methods: Retrospective analysis of 222 patients’ cards with bilateral destructive pulmonary tuberculosis who were treated in the period from 1995 to 2014 in the thoracic department of Dnepropetrovsk regional clinical therapeutic and prophylactic association "Phthisiology". Patients were divided into 2 groups: basic (111 patients who underwent mini-invasive surgery and control (111 patients, who underwent standard surgical approach. The distribution of patients in investigated groups was representative by the majority of parameters. Results and discussion. The average duration of simultaneous bilateral VATS lung resections was 1,90 ± 0,12 hour, standard thoracotomies - 2,13 ± 0,19 per hour, estimated blood loss was 234±5,20ml and 433±3,70ml respectively. The average postoperative time in-patient was 52,40±2,63 days in basic and 80,10±3,58 days in the control group. Number of postoperative complications after lung resection with VATS was significantly lower (1.6 times, as compared with standard surgical approach. Volume of blood loss less than 400 ml was 93,40±3,20% in basic and 72,60±4,80% in the control group, the amount of intraoperative complications reduced by 2.2 times. Complete clinical response (decontamination and closing of cavities have been achieved in patients of the basic group by 1.6 times more often. Conclusions: For patients with bilateral pulmonary tuberculosis to perform mini-invasive surgical approach is the best option. Mini-invasive interventions with VATS due to its good abilities to visualize tissues and anatomical structures may significantly decrease the amount of intraoperative blood and plasma loss in the first postoperative day. It leads to the stabilization of tuberculosis process in the contralateral lung, responsible for

  15. Bilateral radial agenesis with absent thumbs, complex heart defect, short stature, and facial dysmorphism in a patient with pure distal microduplication of 5q35.2-5q35.3

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    Jamsheer Aleksander

    2013-01-01

    Full Text Available Abstract Background A partial duplication of the distal long arm of chromosome 5 (5q35-- > qter is known to be associated with a distinct phenotype referred to as Hunter-McAlpine syndrome. Clinical spectrum of this disorder mainly consists of mental retardation, microcephaly, short stature, skeletal anomalies, and craniofacial dysmorphism featuring flat facies, micrognathia, large, low-set dysplastic ears, hypertelorism, almond-shaped, down-slanted palpebral fissures, epicanthal folds, small nose, long philtrum, small mouth, and thin upper lip. Less frequent remarkable findings include craniosynostosis, heart defect, hypoplastic phalanges, preaxial polydactyly, hypospadias, cryptorchidism, and inguinal hernia. In most patients with a partial duplication of 5q the aberration occurred due to an inherited unbalanced translocation, therefore the phenotype was not reflective of pure trisomy 5q. Case presentation We report on a 9.5-year-old boy with some feature of Hunter-McAlpine syndrome including short stature, complex heart defect (dextrocardia, dextroversion, PFO, bilateral cryptorchidism, hypothyroidism, and craniofacial dysmorphism. Additionally, bilateral radial agenesis with complete absence of Ist digital rays, ulnar hypoplasia with bowing, choroidal and retinal coloboma, abnormal biliary vesicle were identified, which have never been noted in 5q trisomy patients. Karyotype analysis, sequencing and MLPA for TBX5 and SALL4 genes were unremarkable. Array comparative genomic hybridization detected a duplication on 5q35.2-5q35.3, resulting from a de novo chromosomal rearrangement. Our proband carried the smallest of all previously reported pure distal 5q trisomies encompassing terminal 5.4-5.6 Mb and presented with the most severe limb malformation attributed to the increased number of distal 5q copies. Conclusions We postulate that a terminal distal trisomy of 5q35.2-5q35.3, which maps 1.1 Mb telomeric to the MSX2 gene is causative for both

  16. Pulmonary hyalinizing granuloma. Bilateral pulmonary nodules associated with chronic idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Satti, Mohamed B; Batouk, Abdelnasir A; Abdelaziz, Muntasir M; Ahmad, Mohamed F; Abdelaal, Mohamed A

    2005-09-01

    We report a case of a 30-year-old female who had been treated periodically with steroids for idiopathic thrombocytopenic purpura ITP over the last 10 years. Recently, during the course of investigation, she was found to have incidental asymptomatic multiple pulmonary nodules on chest CT. Following a needle biopsy to exclude malignancy, 2 nodules were excised and were histologically confirmed as pulmonary hyalinizing granuloma PHG. The remaining 2 nodules regressed on increasing her dose of steroids. The case is discussed with emphasis on the histological and radiological differential diagnosis, in addition to including ITP among the spectrum of immunologic conditions associated with PHG. PMID:16155671

  17. F-18 FDG PET/CT in Bilateral Diffuse Pulmonary Lymphangitic Carcinomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Senthil, Raja; Parghane, Rahul; Kashyap, Raghava; Bhattacharya, Anish; Mittal, Bhagwant Rai [Postgraduate Institute of Medical Education and Resaarch, Chandigarh (India)

    2012-06-15

    A 51-year-old female patient, who had undergone left-sided modified radical mastectomy for left breast carcinoma 4 years ago, presented with dyspnea of 4 months duration F-18 FDG PET/CT of this patient showed diffusely in-creased FDG uptake in the bilateral lung fields along the thickened bronchovascular bundles. SUVmax of lymphangitic lung was 5.2. The standardized uptake ratio (SUR) of mediastinal blood pool to lymphangitic lung was 0.44. High resolution computed tomography (HRCT) of the same patient showed thickening of interlobular septa and bronchovaseular bundles, with preservation of normal parenchymal architecture. Multiple intrapulmonary nodules and bilateral hilar lymphadenopathy with pulmonary lymphangitic carcinomatosis (PLC). The lungs are the second most common sites for metastases after lymph nodes. These metastases are usually nodular on radiologic images. PLC with interstitial involvement constitutes only 7% of pulmonary metastastases. The most common primary sites, in order of frequency, are adenocarcinoma of the lung, breast, stomach, colon, and prostrate. HRCT has been the modality of choice in the radiologic diagnosis of PLC. Only a few studies have de-scribed the F-18 FDG PET/CT findings in pulmonary lymphangitic carcinomatosis. These studies have shown diffusely increased FDG uptake corresponding to the typical changes in the CT as the most common finding. One study has reported that F-18 FDG PET/CT is 100% specific and 86% sensitive in diagnosing PLC by subjective analysis. The mean SUV in the region of pulmonary lymphangitic lung was 1.26{+-}0.45 and that of blood pool to normal lung was 3.78{+-}1.37.

  18. CT of tracheal agenesis

    International Nuclear Information System (INIS)

    Tracheal agenesis is a rare and usually lethal anomaly. In the past, opaque contrast medium was injected via the esophagus to demonstrate the anatomy. To demonstrate the utility of helical and multidetector CT in delineating the aberrant anatomy in newborns with tracheal agenesis. Four newborns with tracheal agenesis were identified from three institutions. Imaging studies and medical records were reviewed. Each child was imaged with chest radiography. One child was imaged on a single-detector helical CT scanner and the other three on multidetector scanners. Helical and multidetector CT with 2D and 3D reconstructions clearly delineated the aberrant tracheobronchial and esophageal anatomy in each infant. Minimum intensity projection reformatted CT images were particularly helpful. One infant each had type I and type II tracheal agenesis. Two infants had type III tracheal agenesis. All four infants died. CT is a useful tool for delineating the aberrant anatomy of newborns with tracheal agenesis and thus helps in making rational clinical decisions. (orig.)

  19. CT of tracheal agenesis

    Energy Technology Data Exchange (ETDEWEB)

    Strouse, Peter J.; Hernandez, Ramiro J. [C.S. Mott Children' s Hospital, Department of Radiology, Ann Arbor, MI (United States); Newman, Beverley [Children' s Hospital of Pittsburgh, PA (United States). Department of Pediatric Radiology; Afshani, Ehsan [Children' s Hospital of Buffalo, NY (United States). Departments of Radiology and Pediatrics; Bommaraju, Mahesh [Women' s and Children' s Hospital of Buffalo, Division of Neonatology, University Pediatrics Associates, Buffalo, NY (United States)

    2006-09-15

    Tracheal agenesis is a rare and usually lethal anomaly. In the past, opaque contrast medium was injected via the esophagus to demonstrate the anatomy. To demonstrate the utility of helical and multidetector CT in delineating the aberrant anatomy in newborns with tracheal agenesis. Four newborns with tracheal agenesis were identified from three institutions. Imaging studies and medical records were reviewed. Each child was imaged with chest radiography. One child was imaged on a single-detector helical CT scanner and the other three on multidetector scanners. Helical and multidetector CT with 2D and 3D reconstructions clearly delineated the aberrant tracheobronchial and esophageal anatomy in each infant. Minimum intensity projection reformatted CT images were particularly helpful. One infant each had type I and type II tracheal agenesis. Two infants had type III tracheal agenesis. All four infants died. CT is a useful tool for delineating the aberrant anatomy of newborns with tracheal agenesis and thus helps in making rational clinical decisions. (orig.)

  20. Pulmonary circulatory changes after bilateral total knee arthroplasty during regional anesthesia

    Science.gov (United States)

    Bombardieri, Anna Maria; Memtsoudis, Stavros G.; Go, George; Ma, Yan; Sculco, Thomas; Sharrock, Nigel

    2014-01-01

    Study Objective To monitor the pulmonary hemodynamics of patients undergoing bilateral total knee arthroplasty (BTKA) intraoperatively and up to 24 hours following surgery. Design Prospective observational study. Setting University-affiliated teaching hospital. Patients 30 ASA physical status 2 and 3 patients scheduled for single-stage, cemented, BTKA during epidural anesthesia. Interventions Pulmonary artery catheters attached to all patients. Measurements Systemic vascular resistance (SVR), pulmonary vascular resistance (PVR), the ratio of PVR to SVR at baseline, at the beginning of surgery, and after each knee implantation were recorded and compared with measurements taken one day postoperatively (POD 1). Main Results On POD 1, PVR/SVR was increased by 30% compared with baseline (P < 0.0001) and by 20% versus the end of surgery (P < 0.0001). Systemic vascular resistance decreased during surgery and was significantly lower than baseline at 24 hours after surgery (P < 0.0001). No significant change in PVR was noted during surgery. Conclusion The PVR/SVR ratio on the day following BTKA was increased. This change may represent the different effects of inflammatory perioperative stresses on the pulmonary and systemic vasculature. PMID:23391339

  1. Minimally invasive surgical treatment of patients with bilateral pulmonary tuberculosis complicated with pleural empyema

    Directory of Open Access Journals (Sweden)

    Korpusenko I.V

    2015-03-01

    Full Text Available Objective of our study was to increase the effectiveness of surgical treatment of bilateral destructive pulmonary tuberculosis complicated by pleural empyema by using VTS-technologies. The study was done in Dnepropetrovsk regional clinical therapeutic and prophylactic association "Phthisiology" in the period from 2008 to 2013. A retrospective analysis of 43 cases of bilateral destructive pulmonary tuberculosis complicated by pleural empyema on one side and dissemination focus or limited destructive process on contralateral side has been performed. Selected cases were divided into 2 groups: main (eighteen cases where the following procedures were done: performed transsternal occlusion of the main bronchus, sanation of empyema cavity using videothoracosopy, in 30-45 days followed by pleuropneumectomy with usage of minithoracothomy and control (nineteen cases who had undergone drainage of the empyema cavity, sanation, in 45-60 days followed by pleuropmeumectomy with usage of anterolateral access. The distribution of main and control groups for analyzed parameters was representative. Sanation of pleural cavity with videothoracosopy usage compared with Bulau’s drainage provides better antibacterial effect, effective sanitation of the pleural cavity as evidenced by following changes: significant decrease in the number of microbial cells; normalization of total white blood cells count and rod-shaped granulocytes in the peripheral blood 10 days after treatment; normalization of leukocyte intoxication index. The use of minimally invasive surgical treatment allowed to reduce intraoperative complications by 2 times, amount of intraoperative blood loss and hemotrasfusions by 1.5 times, postoperative mortality by 2.5 times. Pleural cavity sanation with videothoracoscopy usage with following pneumoectomy leads to reduce in the incidence and severity of postoperative complications. The most promising is stage-by-stage surgical approach with consecutive use

  2. Agenesis of pancreas

    DEFF Research Database (Denmark)

    Voldsgaard, P; Kryger-Baggesen, N; Lisse, I

    1994-01-01

    Complete agenesis of pancreas is a rare and lethal condition. Four cases have previously been reported in combination with other malformations, such as severe intrauterine growth retardation, hyperglycaemia and meconium ileus. We report a case of pancreatic agenesis as a single anomaly. The child...

  3. Case Report: Bilateral reexpansion pulmonary edema following treatment of a unilateral hemothorax [v1; ref status: indexed, http://f1000r.es/4yb

    OpenAIRE

    Steven P de Wolf; Jaap Deunk; Cornet, Alexander D.; Paul WG Elbers

    2014-01-01

    Bilateral re-expansion pulmonary edema (RPE) is an extremely rare entity. We report the unique case of bilateral RPE following a traumatic, unilateral hemopneumothorax in a young healthy male. Bilateral RPE occurred only one hour after drainage of a unilateral hemopneumothorax. The patient was treated with diuretics and supplemental oxygen. Diagnosis was confirmed by excluding other causes, using laboratory findings, chest radiography, pulmonary and cardiac ultrasound and high resolution comp...

  4. Isolated bilateral transverse agenesis of the distal segments of the lower limbs at the level of the knee joint in a human fetus.

    Science.gov (United States)

    Christiaens, Antoine B; Deprez, Pierre M L; Amyere, Mustapha; Mendola, Antonella; Bernard, Pierre; Gillerot, Yves; Clapuyt, Philippe; Godfraind, Catherine; Lengelé, Benoît G; Vikkula, Miikka; Nyssen-Behets, Catherine

    2016-02-01

    Congenital limb anomalies occur in Europe with a prevalence of 3.81/1,000 births and can have a major impact on patients and their families. The present study concerned a female fetus aborted at 23 weeks of gestation because she was affected by non-syndromic bilateral absence of the zeugopod (leg) and autopod (foot). Autopsy of the aborted fetus, X-ray imaging, MRI, and histochemical analysis showed that the distal extremity of both femurs was continued by a cartilage-like mass, without joint cavitation. Karyotype was normal. Moreover, no damaging variant was detected by exome sequencing. The limb characteristics of the fetus, which to our knowledge have not yet been reported in humans, suggest a developmental arrest similar to anomalies described in chicks following surgical experiments on the apical ectodermal ridge of the lower limbs. PMID:26544544

  5. Isolated bilateral transverse agenesis of the distal segments of the lower limbs at the level of the knee joint in a human fetus.

    Science.gov (United States)

    Christiaens, Antoine B; Deprez, Pierre M L; Amyere, Mustapha; Mendola, Antonella; Bernard, Pierre; Gillerot, Yves; Clapuyt, Philippe; Godfraind, Catherine; Lengelé, Benoît G; Vikkula, Miikka; Nyssen-Behets, Catherine

    2016-02-01

    Congenital limb anomalies occur in Europe with a prevalence of 3.81/1,000 births and can have a major impact on patients and their families. The present study concerned a female fetus aborted at 23 weeks of gestation because she was affected by non-syndromic bilateral absence of the zeugopod (leg) and autopod (foot). Autopsy of the aborted fetus, X-ray imaging, MRI, and histochemical analysis showed that the distal extremity of both femurs was continued by a cartilage-like mass, without joint cavitation. Karyotype was normal. Moreover, no damaging variant was detected by exome sequencing. The limb characteristics of the fetus, which to our knowledge have not yet been reported in humans, suggest a developmental arrest similar to anomalies described in chicks following surgical experiments on the apical ectodermal ridge of the lower limbs.

  6. PULMONARY LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS SIMULTANEOUS BILATERAL SPONTANEOUS PNEUMOTHORAX IN A NON-SMOKER PATIENT

    Directory of Open Access Journals (Sweden)

    M. Vaziri

    2008-06-01

    Full Text Available Pulmonary Langerhans Cell Histiocytosis (PLCH is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was the presenting feature. The final diagnosis was made by open surgical biopsy and recurrent pneumothoraces necessitated surgical management with pleurodesis. We emphasize the early use of pleurodesis in managing patients with PLCH and spontaneous pneumothorax.

  7. Complete Right Lung Agenesis with Dextrocardia: An Unusual Cause of Respiratory Distress

    Directory of Open Access Journals (Sweden)

    Devki Nandan

    2012-01-01

    Full Text Available Pulmonary agenesis, defined as complete absence of bronchus, parenchyma, and vessels is a very rare condition. Herein, we report a 4-month-old infant who presented with fever, cough, and respiratory distress and was misdiagnosed in a peripheral hospital as a case of pneumonia. The child was, later diagnosed as having right lung agenesis with dextrocardia.

  8. Case Report: Bilateral reexpansion pulmonary edema following treatment of a unilateral hemothorax [v1; ref status: indexed, http://f1000r.es/4yb

    Directory of Open Access Journals (Sweden)

    Steven P de Wolf

    2014-12-01

    Full Text Available Bilateral re-expansion pulmonary edema (RPE is an extremely rare entity. We report the unique case of bilateral RPE following a traumatic, unilateral hemopneumothorax in a young healthy male. Bilateral RPE occurred only one hour after drainage of a unilateral hemopneumothorax. The patient was treated with diuretics and supplemental oxygen. Diagnosis was confirmed by excluding other causes, using laboratory findings, chest radiography, pulmonary and cardiac ultrasound and high resolution computed tomography. His recovery was uneventful. The pathophysiology of bilateral RPE is not well known. Treatment is mainly supportive and consists of diuretics, mechanical ventilation, inotropes and steroids. In case of a pulmonary deterioration after the drainage of a traumatic pneumothorax, bilateral RPE should be considered after exclusion of more common causes of dyspnea.

  9. Lumbosacral agenesis in a cat

    Directory of Open Access Journals (Sweden)

    Gabrielle C Hybki

    2016-01-01

    Full Text Available Case summary Lumbosacral agenesis is a rare congenital condition reported in children. We report a 17-week-old female domestic shorthair cat with lumbosacral agenesis on whole-body radiographs. The cat was euthanized shortly thereafter presentation. A necropsy was not permitted. Relevance and novel information This is the first reported feline case of lumbosacral agenesis.

  10. Two cases of sacral agenesis

    Energy Technology Data Exchange (ETDEWEB)

    Choi, J. Y.; Bae, Y. K.; Hahm, C. K.; Kang, S. R. [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    1979-06-15

    Sacral agenesis is a central component in the spectrum of anomalies comprising the caudal regression syndrome. Sacral agenesis occurs more frequently in an infant born to mother of diabetes, rubella infection in first trimester than normal mothers. In a patient of sacral agenesis, it is important to recognize the neurologic deficit, neurogenic bladder dysfunction and other congenital anomalies. A case of partial sacral agenesis of 10 years old girl and another case of complete total agenesis associated with multiple anomalies in autopsied newborn are reported.

  11. Successful Management of Intraoperative Acute Bilateral Pulmonary Embolism in a High Grade Astrocytoma Patient.

    Science.gov (United States)

    Khraise, Wail N; Allouh, Mohammed Z; Hiasat, Mohammad Y; Said, Raed S

    2016-01-01

    BACKGROUND Intraoperative pulmonary embolism (PE) is a rare life-threatening complication in patients undergoing surgical intervention. Generally, cancer patients have a higher risk for developing this complication. Unfortunately, there is no standard procedure for its management. CASE REPORT We report the case of a 39-year-old woman with high-grade glioma in the right frontal lobe who was admitted to the surgical theater for craniotomy and excision of the tumor. During the general anesthesia procedure and just before inserting the central venous line, her end-tidal CO2 and O2 saturation dropped sharply. The anesthesiologist quickly responded with an aggressive resuscitation procedure that included aspiration through the central venous line, 100% O2, and IV administration of ephedrine 6 mg, colloid 500 mL, normal saline 500 mL, and heparin 5000 IU. The patient was extubated and remained in the supine position until she regained consciousness and her vital signs returned to normal. Subsequent radiological examination revealed a massive bilateral PE. A retrievable inferior vena cava (IVC) filter was inserted, and enoxaparin anticoagulant therapy was prescribed to stabilize the patient's condition. After 3 weeks, she underwent an uneventful craniotomy procedure and was discharged a week later under the enoxaparin therapy. CONCLUSIONS The successful management of intraoperative PE requires a quick, accurate diagnosis accompanied with an aggressive, fast response. Anesthesiologists are usually the ones who are held accountable for the diagnosis and early management of this complication. They must be aware of the possibility of such a complication and be ready to react properly and decisively in the operation theater. PMID:27578311

  12. Moebius syndrome with Dandy-Walker variant and agenesis of corpus callosum

    Directory of Open Access Journals (Sweden)

    Jomol Sara John

    2013-01-01

    Full Text Available Moebius syndrome is a rare congenital neurological disorder. The most frequent mode of presentation is facial diplegia with bilateral lateral rectus palsy, but there are variations. Here, we report a rare case of Moebius syndrome in a 15-month-old child with unilateral facial palsy, bilateral abducens nerve palsy with Dandy Walker variant, and complete agenesis of corpus callosum.

  13. Post-operative Unexpected Bilateral Massive Pulmonary Embolism in a Thirty-Year-Old Woman: A Case Report

    Directory of Open Access Journals (Sweden)

    Öznur Şen

    2015-12-01

    Full Text Available Pulmonary embolism (PE is a rare, yet serious complication. That, generally follows invasive procedures in adults. The present report details the case of bilateral pulmonary embolism in a thirty-year-old women after ovarian cyst surgery. A thirty-year-old female patient, who had ovarian cystectomy ten days previously, consulted the emergency department with the complaints of sickness and syncope. Her blood pressure, pulse and respiratory rate were 90/40 mmHg, 150/min. and 35/min, respectively. During examination and phlebotomy, she had a cardiac arrest and responded to a 3-minute cardiopulmonary resuscitation (CPR with tracheal intubation. During imaging procedures, she had three further cardiac arrests and was admitted to the intensive care unit. The preliminary diagnoses were acute renal failure (ARF and PE. Fluid replacement, inotropic support, anticoagulant therapies, as well as sedation to prevent brain damage were started immediately. Thrombolytic therapy was not attempted because she had surgery ten days ago and hematoma was observed on abdominal ultrasound. Inhaled nitric oxide and inhaled iloprost with sildenafil were employed to reduce pulmonary hypertension. When discharged 136 days after hospitalization she was a tracheotomized and, spontaneously breathing home care patient with hypoxic brain injury. Early detection and treatment of PE are of great importance for a good prognosis and prevention of life-threatening complications.

  14. Bilobar thyroid agenesis with primary hyperparathyroidism: report of a case.

    Science.gov (United States)

    Şimşek, Turgay; Cantürk, Nuh Zafer; Cantürk, Zeynep; Gürbüz, Yeşim

    2015-06-01

    Congenital thyroid abnormalities are rarely seen. They mostly include hemiagenesis with or without involving the isthmus. In this report, we present a case of bilateral lobe agenesis with hypertrophied isthmus and high calcium and elevated PTH levels which were detected during routine examinations of a 49-year-old female patient. Some findings consistent with parathyroid pathology on the right side were determined in parathyroid scintigraphy. At thyroid scan and neck ultrasonography there was no sign of bilateral thyroid tissue except a mass localized isthmus. The right lower parathyroidectomy and thyroidectomy for isthmus were performed; the pathology report was shown as parathyroid adenoma and nodular colloidal goiter. This case of bilobar agenesis with incidental primary hyperparathyroidism due to single parathyroid adenoma is the first case reported in literature. PMID:25091455

  15. Docetaxel (Taxotere®)-induced cavitary change of pulmonary metastatic lesions complicated by bilateral spontaneous pneumothoraces in a patient with primary adenocarcinoma of the lung.

    Science.gov (United States)

    Kao, Hao-Lun; Lin, Wen-Chiung; Hsu, Hsian-He; Huang, Guo-Shu

    2013-06-01

    Pneumothorax is a complication that rarely occurs after chemotherapy for lung cancer. We report the chest computed tomography findings of a case of spontaneous pneumothorax complicating docetaxel (Taxotere®) treatment for pulmonary metastasis in a 70-year-old woman with pulmonary adenocarcinoma. The patient developed bilateral pneumothoraces, which was induced by changes in the cavitary pulmonary metastatic lesions, after systemic chemotherapy with docetaxel. The chest computed tomography findings and possible mechanisms of this unusual complication are discussed in this report. PMID:23820551

  16. Three new patients with FATCO: fibular agenesis with ectrodactyly.

    Science.gov (United States)

    Bieganski, Tadeusz; Jamsheer, Aleksander; Sowinska, Anna; Baranska, Dobromila; Niedzielski, Kryspin; Kozlowski, Kazimierz; Czarny-Ratajczak, Malwina

    2012-07-01

    We document three new patients with fibular agenesis, tibial campomelia, and oligosyndactyly (FATCO). Two of these individuals had tetramelic manifestations while the third had bilateral abnormalities of the lower limbs. These patients and others reported as FATCO seem to belong to the phenotype "fibular aplasia with ectrodactyly." Genetic screening for CNVs and mutations in the TP63 and WNT10B genes did not show any genetic abnormalities. © PMID:22628253

  17. Agenesis of the dorsal pancreas

    Institute of Scientific and Technical Information of China (English)

    Lale Pasaoglu; Murat Vural; Hatice Gul Hatipoglu; Gokce Tereklioglu; Suha Koparal

    2008-01-01

    Developmental anomalies of the pancreas have been reported but dorsal pancreatic agenesis is an extremely rare entity. We report an asymptomatic 62-year-old woman with complete agenesis of the dorsal pancreas.Abdominal computed tomography (CT) revealed a normal pancreatic head, but pancreatic body and tail were not visualized. Magnetic resonance imaging (MRI)findings were similar to CT. At magnetic resonance cholangiopancreatography (MRCP), the major pancreatic duct was short and the dorsal pancreatic duct was not visualized. The final diagnosis was dorsal pancreatic agenesis.

  18. Case report: Successful treatment of recurrent chordoma and bilateral pulmonary metastases following an 11-year disease-free period

    Science.gov (United States)

    Erkmen, Cherie P.; Barth, Richard J.; Raman, Vignesh

    2014-01-01

    INTRODUCTION Chordomas are rare but aggressive tumors due to local recurrence and distant metastases. They originate commonly in the sphenooccipital and sacrococcygeal regions, and metastasize to the lungs, bone, skin, liver, and lymph nodes. They occur more frequently in men and people over the age of 40. PRESENTATION OF CASE A 28 year-old female presented with sacrococcygeal chordoma for which she received wide local excision and adjuvant radiation therapy. She enjoyed an unusual disease-free survival for 11 years until a routine surveillance scan of the pelvis identified local recurrence. Further work up revealed bilateral pulmonary metastases. She underwent local excision of the recurrent tumor and video-assisted thoracoscopic (VATS) wedge resection of pulmonary metastases. She also received adjuvant radiation therapy to the recurrent resection bed. Two years later, she remains free of disease and symptoms. DISCUSSION Chordomas are commonly insensitive to chemotherapy and radiation, making surgery the most successful therapeutic modality. However, there are few guidelines on the surveillance and treatment of recurrent chordoma. We report success with aggressive surgical resection of recurrence and metastasis as well as adjuvant radiation therapy. CONCLUSION The prolonged survival of our patient underscores the importance of (1) aggressive surgical resection of chordoma, whether primary, recurrent, or metastatic, with adjuvant radiation therapy, (2) minimization of surgical seeding of tumor, and (3) diligent cancer surveillance. PMID:24907541

  19. Congenital agenesis of seminal vesicle

    Institute of Scientific and Technical Information of China (English)

    Hong-Fei Wu; Di Qiao; Li-Xin Qian; Ning-Hong Song; Ning-Han Feng; Li-Xin Hua; Wei Zhang

    2005-01-01

    Congenital agenesis of the seminal vesicle (CASV) is frequently associated with congenital absence of the vas deferens (CAVD) or ipsilateral congenital vasoureteral communication. We reported two cases of a rare condition that the vas deferens open ectopically into Mullerian duct cyst associated with agenesis of the ipsilateral seminal vesicle. The diagnosis was confirmed by vasography. Transurethral unroofing of the Mullerian duct cyst was performed in both patients with favourable results, however, assisted reproductive technology (ART) was still necessary for them to father children.

  20. Incidence of agenesis of palmaris longus in the Andhra population of India

    Directory of Open Access Journals (Sweden)

    K Devi Sankar

    2011-01-01

    Full Text Available Background : The knowledge of Palmaris longus (PL is a growing interest for its wide role in reconstructive plastic surgeries as a donor tendon for transfer or transplant. The prevalence of the PL agenesis has been well-documented by many authors in different ethnic groups or populations. Many conventional tests for determining the presence of the PL has been described, but lamentably there are many discrepancies in confirming its presence or absence. Slight modifications of the prevailing methods can still give authenticate results. Aim : This prospective study was conducted to determine the incidence of unilateral and bilateral agenesis of PL and its association with sex and side of the limb in the Andhra population of India. Materials and Methods : A total of 942 subjects of both sexes belonging to 18-23 years were used to access the PL using various tendon examination techniques including our modified Schaeffer′s test. The data collected were analyzed by Pearsons χ2 test using SPSS software. Results : Overall agenesis of muscle in both sexes was 264 (28.0%, out of which 40.2% was seen in females and 14.7% in males with the ratio of 3:1. The unilateral agenesis was seen in 70.5% and bilateral agenesis in 29.5% subjects. The left side agenesis was seen in 51.6% and right side in 48.4% subjects. Conclusions : The prevalence of bilateral and unilateral agenesis was more common on left side with a greater likelihood in the female subjects. The proposed technique could bring better results in all subjects and can be implemented in manual examination of PL.

  1. [Agenesis of the infrarenal inferior vena cava with thrombosis of the renal vein in a fetus: a case report].

    Science.gov (United States)

    Raposo Rodríguez, L; Recio Rodríguez, M; Alvarez Moreno, E; López Azorín, M

    2012-01-01

    Agenesis of the inferior vena cava, especially of the infrarenal segment, is exceptional. This condition is thought to result from thrombosis during gestation rather than from a true congenital malformation. Agenesis of the inferior vena cava can be associated with renal vein thrombosis, which in turn is related to suprarenal hemorrhage in the fetus. We present a case of agenesis of the inferior vena cava with preservation of the hepatic segment, thrombosis of the left renal vein, and secondary bilateral suprarenal hemorrhage diagnosed prenatally using sonography and magnetic resonance imaging.

  2. Semen analysis in an infertile man with seminal vesicles cysts associated with ipsilateral renal agenesis.

    Science.gov (United States)

    Andrade-Rocha, Fernando Tadeu

    2006-01-01

    A case of an infertile man with bilateral seminal vesicles cysts with ipsilateral renal agenesis is presented with emphasis on the semen analysis. Ejaculates showed very low concentration of fructose, leukocytospermia, teratozoospermia, asthenozoospermia, hyperviscosity, marked sperm agglutination and a poor hypoosmotic swelling test. Clinical value of these findings is discussed. PMID:16502061

  3. Isolated complete corpus callosal agenesis

    Directory of Open Access Journals (Sweden)

    Jaiganesh S, Venkateshwaran A, Naresh Kumar C, Rajasekhar KV

    2014-11-01

    Full Text Available Isolated complete corpus callosal agenesis is a rare entity. Usually this condition will be an associated finding in other syndromes. 3 month old male child came with complaints of deformed foot on both sides, not having a social smile and neck holding. Patient referred to the Radiology department for MRI brain which showed complete absence of corpus callosum, widely separated and parallely placed lateral ventricles, colpocephaly, high riding of 3rd ventricle and absence of cingulate gyrus and radial arrangement of gyri along the interhemispheric fissure. Hence it was reported as isolated complete corpus callosal agenesis and this article describes the Embryogenesis, anatomy, developmental anomalies and its clinical manifestations & prognosis.

  4. Agenesis of the Corpus Callosum and Skeletal Deformities in Two Unrelated Patients: Analysis via MRI and Radiography

    Directory of Open Access Journals (Sweden)

    Ali Al Kaissi

    2014-01-01

    Full Text Available Purpose. Mental retardation, mild to severe epilepsy and cerebral palsy often of hemiplegic type are common accompaniments in patients with agenesis/hypoplasia of the corpus callosum. Skeletal deformities of bilateral radiohumeral synostosis, brachydactyly, bilateral elbow dislocation, talipes equinovarus, and juxtacalcaneal accessory bones have been encountered in two unrelated children with agenesis of the corpus callosum. Methods. We report on two unrelated children who presented with the full clinical criteria of agenesis of the corpus callosum. Strikingly, both presented with variable upper and lower limb deformities. The clinical features, radiographic and MRI findings in our current patients, have been compared with previously reported cases identified through a PubMed literature review. Results. Bilateral radiohumeral synostosis associated with pyruvate dehydrogenase deficiency has been encountered in one patient. The other patient manifested bilateral elbow dislocation, coxa valga, talipes equinovarus, and bilateral juxtacalcaneal accessory bones. Conclusion. The constellation of malformation complexes in our current patients have the hitherto not been reported and expanding the spectrum of skeletal deformities in connection with agenesis of the corpus callosum.

  5. 以双侧肺结节为临床表现的肺隐球菌性炎性假瘤1例及文献回顾%Pulmonary inflammatory pseudotumor caused by Cryptococcus presenting multiple nodules in bilateral lungs

    Institute of Scientific and Technical Information of China (English)

    刘立刚; 潘铁成; 魏翔

    2006-01-01

    By now there are a few reports describing a case of pulmonary inflammatory pseudotumor, presenting multiple nodules in bilateral lungs and a pseudotumor caused by fungus infection is also a very rare disease. Here we report a rare case of pulmonary inflammatory pseudotumor with confirmed cause by Cryptococcus infection presenting multiple nodules in bilateral lungs.

  6. Agenesis of the dorsal pancreas

    Institute of Scientific and Technical Information of China (English)

    Wolfgang J Schnedl; Claudia Piswanger-Soelkner; Sandra J Wallner; Robert Krause; Rainer W Lipp

    2009-01-01

    During the last 100 years in medical literature, there are only 54 reports, including the report of Pasaoglu et al ( World J Gastroenterol 2008; 14: 2915-2916), with clinical descriptions of agenesis of the dorsal panc reas in humans . Agenes i s of the dor sal pancreas, a rare congenital pancreatic malformation,is associated with some other medical conditions such as hyperglycemia, abdominal pain, pancreatitis and a few other diseases. In approximately 50% of reported patients with this congenital malformation,hyperglycemia was demonstrated. Evaluation of hyperglycemia and diabetes mellitus in all patients with agenesis of the dorsal pancreas including description of fasting blood glucose, oral glucose tolerance test, glycated hemoglobin and medical treatment would be a future goal. Since autosomal dominant transmission has been suggested in single families,more family studies including imaging technologies with demonstration of the pancreatic duct system are needed for evaluation of this disease. With this letter to the editor, we aim to increase available information for the better understanding of this rare disease.

  7. Tracheal agenesis with broncho-esophageal fistula in VACTERL / TACRD association

    OpenAIRE

    Mandrekar, Suresh R. S.; Sangeeta Amoncar; R. G. W. Pinto

    2013-01-01

    Tracheal agenesis (TA) is an extremely rare malformation. We report here autopsy findings in a case of TA with bronchoesophageal fistula of Floyd type III. The other malformations present included laryngeal atresia, Right lung hypolobulation, ventricular septal defect in membranous portion, bilateral cystic renal dysplasia, spleninculus, Meckel's diverticulum, and imperforate anus. The constellations of malformations present in our case have overlapping features with Vertebral anomalies, Anal...

  8. Does asymptomatic septal agenesis exist? A review of 34 cases

    Energy Technology Data Exchange (ETDEWEB)

    Belhocine, Ouardia; Andre, Christine; Kalifa, Gabriel; Adamsbaum, Catherine [St Vincent de Paul Hospital, Radiology Department, Paris (France)

    2005-04-01

    Primary septal agenesis (PSA) is a rare brain malformation that can be isolated or part of developmental brain abnormalities (holoprosencephaly, septo-optic dysplasia or cortical malformation). Such associated malformation can be subtle, leading to difficulties in the prenatal management of PSA. Moreover, the neurological prognosis of isolated PSA remains debatable. The aims of the study were to specify the patterns and frequency of brain malformations associated with septal agenesis (SA), to identify the clinical prognosis, and to discuss the aetiology of PSA with the new insights provided by molecular genetics. The study consisted of a 14-year retrospective review of brain MRI in 34 patients having PSA (mean age, 5 years). Chiasm and optic nerves were not evaluated. Post-hydrocephalus SA or incomplete data were excluded. The clinical data were correlated to the MRI patterns. The study disclosed 82.5% associated lesions with MRI (28/34): 11 neuronal migration disorders, 9 holoprosencephalies (HP), 7 pituitary stalk interruptions, 1 corpus callosum partial agenesis; 17.5% (6/34) of cases were apparently isolated PAS. Clinically, the patients had motor dysfunction in 68% (23/34), mental retardation in 65% (22/34), blindness in 24% (8/34), endocrinological defects in 21% (7/34) and epilepsy in 18% (6/34) of cases. Nine percent of patients (3/34) were neurologically normal (including one with scoliosis and two infants younger than 2 years at the last follow-up). Patients with bilateral cortical anomalies and HP (even if mild) had the worst neurological prognosis. A severe motor impairment was present without evidence of hemispheric anomaly in 12% of patients (4/34). Interestingly, the frontal lobes were involved in 90% of cortical anomalies and HP, supporting the malformative aetiology of PSA. PSA rarely appears isolated and severe psychomotor impairment may occur in apparently isolated forms. These unfavourable results should be highlighted and need to be confirmed

  9. Cardiac Arrest Secondary to Bilateral Pulmonary Emboli following Arteriovenous Fistula Thrombectomy: A Case Report with Review of the Literature

    Directory of Open Access Journals (Sweden)

    Avni Shah

    2012-01-01

    Full Text Available Number of patients with End Stage Renal Disease (ESRD is growing worldwide. Hemodialysis remains the main modality of renal replacement therapy for ESRD patients. A patent hemodialysis access (arteriovenous fistula or arteriovenous graft plays a key role in successful delivery of hemodialysis. Common vascular access issues encountered by patients and nephrologists are thrombosis and infection. The thrombosed access is declotted by various percutaneous techniques these days by multiple outpatient access centers in a timely fashion. Thrombolysis can give rise to various complications, a few of which can be life threatening. A young hemodialysis patient underwent percutaneous thrombolysis of his clotted arteriovenous fistula. Outpatient access thrombectomy was complicated immediately afterwards with cardiac arrest requiring cardiac resuscitation in the recovery room. The patient was admitted to intensive care unit after life sustaining care. Work up revealed multiple pulmonary emboli to both lung fields on CT scan of the chest. Patient was anticoagulated and discharged from the hospital. Thrombolysis of clotted hemodialysis access is associated commonly with occurrences of pulmonary embolic which are usually asymptomatic. Massive pulmonary embolization due to access thrombolysis is rare. Nephrologists and radiologists should be aware of this dangerous complication particularly in patients with preexisting cardiopulmonary disease.

  10. Fetal lung growth in laryngeal atresia and tracheal agenesis.

    Science.gov (United States)

    Scurry, J P; Adamson, T M; Cussen, L J

    1989-02-01

    Three cases of airway obstruction in fetuses born at 21, 32 and 40 weeks gestation are reported. The first had laryngeal atresia, cystic dysplastic kidneys, oligohydramnios and immense fluid-filled lungs. The second had upper tracheal agenesis, a tracheo-oesophageal fistula, a cystic dysplastic horseshoe kidney, oligohydramnios and normal-sized lungs. The third had a pin-hole mucosal tract through an otherwise atretic larynx, normal kidneys, no oligohydramnios and normal-sized lungs. Lung weight:body weight ratios, radial alveolar or radial canalicular counts and point-counting of sections of lungs in cases 1 and 2 show that laryngeal or tracheal obstruction may prevent or reduce the pulmonary hypoplasia associated with renal dysplasia, and in cases 2 and 3, that grossly enlarged, hyperplastic lungs may not be seen unless obstruction is complete. PMID:2730470

  11. MDCT Findings of Tracheal Agenesis: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Jeong, Yu Mi; Kim, Jee Eun; Son, Dong Woo; Kim, Ha Na; Hwang, Hee Young [Gachon University, Gil Hospital, Incheon (Korea, Republic of)

    2009-01-15

    Tracheal agenesis is an extremely rare congenital anomaly with fatal consequences. We report a case of tracheal agenesis in a newborn infant who presented with no self-respiration and cyanosis that was confirmed by the use of 64-slice multidetector CT. Coronal and sagittal multiplanar reconstruction images clearly delineated the aberrant anatomy of tracheal agenesis and demonstrated the presence of a tracheoesophageal fistula.

  12. Bifurcation of the femur with tibial agenesis and additional anomalies

    NARCIS (Netherlands)

    van der Smagt, JJ; Bos, CFA; van Haeringen, A; Hogendoorn, PCW; Breuning, MH

    2005-01-01

    Bifurcation of the femur and tibial agenesis are rare anomalies and have been described in both the Gollop-Wolfgang Complex and the tibial agenesis-ectrodactyly syndrome. We report on two patients with bifurcation of the femur and tibial agenesis. Hand ectrodactyly was seen in one of these patients.

  13. Tracheal agenesis with broncho-esophageal fistula in VACTERL / TACRD association

    Directory of Open Access Journals (Sweden)

    Suresh R. S. Mandrekar

    2013-01-01

    Full Text Available Tracheal agenesis (TA is an extremely rare malformation. We report here autopsy findings in a case of TA with bronchoesophageal fistula of Floyd type III. The other malformations present included laryngeal atresia, Right lung hypolobulation, ventricular septal defect in membranous portion, bilateral cystic renal dysplasia, spleninculus, Meckel′s diverticulum, and imperforate anus. The constellations of malformations present in our case have overlapping features with Vertebral anomalies, Anal atresia, Cardiovascular anomalies, Tracheo-esophageal fistula, Esophageal atresia, Renal anomalies, Limb anomalies and Tracheal atresia or laryngo tracheal atresia, Cardiac anomalies, Renal anomalies, Duodenal atresia association described previously in the literature.

  14. A Case of Cryptorchidism with Ipsilateral Congenital Unilateral Absence of the Vas Deferens and Contralateral Renal Agenesis

    Science.gov (United States)

    Yu, Young Dong

    2016-01-01

    Introduction and Aims. Congenital absence of the vas deferens is an uncommon anomaly and this clinical condition is responsible for up to 1-2% of male infertility. It can be either unilateral or bilateral and the associated anomalies include cryptorchidism, seminal vesicles and ejaculatory ducts anomalies, and renal anomalies such as renal agenesis. We hereby present a case of congenital unilateral absence of vas deferens, which was found incidentally during an evaluation of undescended testis in a patient with ipsilateral renal agenesis. Case Presentation. A 10-month-old boy was referred to the urology clinic with an undescended right testis. Preoperative abdominal ultrasonography showed agenesis of the right kidney and the absence of right vas deferens and epididymis was confirmed during laparoscopic orchiectomy performed due to short right spermatic cord. There were no other concomitant anomalies of the genitourinary system observed in evaluation. Conclusion. Congenital unilateral absence of the vas deferens with cryptorchidism and renal agenesis is a rare diagnostic entity. Cryptorchidism or absent vas deferens found incidentally should lead the physician to evaluate the status of the contralateral vas deferens and conduct a renal tract ultrasound study. PMID:27597925

  15. Bloqueio pleural bilateral: analgesia e funções pulmonares em pós-operatório de laparotomias medianas Bloqueo pleural bilateral: analgesia y funciones pulmonares en pós-operatorio de laparotomias medianas Bilateral pleural block: analgesia and pulmonary functions in postoperative of median laparotomies

    Directory of Open Access Journals (Sweden)

    Karl Otto Geier

    2004-08-01

    TODO: Bloqueo pleural bilateral fue realizado en decúbito dorsal horizontal en 21 pacientes con 20 ml de bupivacaína a 0,375% con adrenalina a 1:400.000 administrados por catéter en cada hemitórax durante el pós-operatorio inmediato. Soluciones aleatorias de bupivacaína y de solución fisiológica fueron administradas por estudiantes en la práctica o enfermeras que desconocían el contenido de las jeringas, y sus resultados analgésicos evaluados de acuerdo con la escala de dolor Prince Henry al comparar los valores pre y pós-bloqueo pleural bilateral. En función del dolor pós-operatorio, ensayos espirométricos de las funciones pulmonares también fueron determinados mediante un espirómetro portátil. RESULTADOS: Analgesia pós-operatoria, con duración media de 247,75 ± 75 minutos fue constatada en todos los pacientes con la bupivacaína, aunque haya persistido dolor residual de menor intensidad en la región suprapúbica en cinco pacientes (8% y en dos pacientes en la apófisis xifóide (3,2%. Ningún efecto analgésico fue obtenido con solución fisiológica. De frente al dolor pós-operatorio, las funciones pulmonares, evaluadas antes y después de los bloqueos, registraron mejoría con la bupivacaína en CVF (p BACKGROUND AND OBJECTIVES: Notwithstanding pleural block having become almost an analgesic panacea, contradictory results have been published. This study aimed at observing analgesic and spirometric behavior of pulmonary function in the immediate postoperative period of 21 patients submitted to urgency median laparotomies under bilateral pleural block. METHODS: Bilateral pleural block was induced in the supine position with 20 mL of 0.375% bupivacaine with 1:400,000 epinephrine administered via catheter in each hemithorax during the immediate postoperative period. Randomized bupivacaine and saline were administered by residents or nurses blind to syringes content, and their analgesic outcomes were evaluated according to Prince Henry's pain score by

  16. Artery Agenesis: Ipsilateral Common Carotid Artery Hypoplasia

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    Omer Kaya

    2014-01-01

    Full Text Available A 42-year-old female patient, who had been diagnosed with an occlusion of her left internal carotid artery (ICA following Doppler ultrasonographic (US and digitally-subtracted angiographic (DSA examinations performed in an outer healthcare center in order to eliminate the underlying cause of her complaint of amorosis fugax, later applied to our hospital with the same complaint. At Doppler US performed in our hospital’s radiology department, her right common carotid artery (CCA was normal, but her left CCA was hypoplastic. The right internal artery (ICA was validated as normal. At the left side, however, the ICA was apparent only as a stump and it did not demonstrate a continuity. The diagnosis of ICA agenesis was confirmed by the utilization of Doppler US, CT, and DSA imaging, and it was concluded also that ipsilateral CCA hypoplasia could be evaluated as an important clue to the diagnosis of ICA agenesis.

  17. Pulmonary Hyalinising Granuloma: A rare pulmonary disorder

    International Nuclear Information System (INIS)

    Pulmonary hyalinising granulomas are rare, non-infectious fibros lesions of the lung which can mimic metastatic disease. It was first described in literature by Engleman et al in the year 1977. Its etiology is unknown but they may be caused by an exaggerated immune response. The patient typically presents with cough, chest pain, dyspnoea or haemoptysis in association with multiple bilateral parenchymal nodules. We report the case of a 20 years old male who presented with a 12-month history of worsening dry cough. His plain chest radiograph and subsequent CT scan revealed bilateral pulmonary nodules. A CT guided biopsy of the pulmonary lesions was consistent with Pulmonary Hyalinising Granuloma [PHG].

  18. Tracheal agenesis: A report of two cases

    Directory of Open Access Journals (Sweden)

    A V Desai

    2016-01-01

    Full Text Available Tracheal agenesis is an extremely rare congenital anomaly involving the respiratory system. It is generally associated with anomalies of other systems. Antenatal diagnosis of this condition is difficult; therefore, it presents as a medical emergency in the labor room. Intubation in these babies is difficult. As many of these babies are born prematurely, respiratory distress syndrome (RDS adds to the management difficulties. Here, we describe two babies with this lethal anomaly and RDS where esophageal intubation and surfactant therapy proved beneficial. Furthermore, described are other associated anomalies.

  19. Magnetic resonance imaging in agenesis of corpus callosum

    International Nuclear Information System (INIS)

    The recent use of computed tomography (CT) scan is providing an easy diagnoses of agenesis of corpus callosum which had been difficult to diagnose only by clinical signs and symptoms. Since, more neuropsychological studies on agenesis of corpus callosum are being done, clinical details of agenesis of corpus callosum are being clarified. We examined magnetic resonance imaging (MRI) on 3 patients who were suspected to have agenesis of corpus callosum by CT scan. And we studied the usefulness of MRI in agenesis of corpus callosum. By sagittal MRI, we could easily make a diagnosis on one case (Case 3) of hypoplasia of corpus callosum which could not been diagnosed by CT scan. On all of the cases, MRI showed the high intensity area of anterior commissure which had not been found by CT scan. Anomaly of cingurate gyrus was shown on all of the patients. MRI can show some important points in detail which CT scan can not reveal. The sagittal view especially is the most helpful for diagnosis of agenesis of corpus callosum. And we proposed that neuropsychological study on agenesis of corpus callosum should have MRI data. (J.P.N.)

  20. Marfan's syndrome presenting as bilateral spontaneous pneumothorax.

    OpenAIRE

    Gawkrodger, D J

    1981-01-01

    A case of bilateral spontaneous pneumothorax in a 14-year-old girl with previously undiagnosed Marfan's syndrome is described. The pulmonary abnormalities of Marfan's syndrome are not commonly encountered and bilateral pneumothorax is itself a rare event which, in most instances, has been reported following invasive procedures.

  1. Permanent Maxillary Canine Agenesis: A Rare Case Report

    OpenAIRE

    Halaswamy V Kambalimath; Jain, Somya; Patil, Raju Umaji; Asokan, Alexander; Kambalimath, Deepashri

    2015-01-01

    ABSTRACT Congenitally missing teeth (CMT) are among one of the commonly known dental anomalies. The most frequently missing teeth in the permanent dentition, excluding the third molars, are mandibular second premolars and maxillary lateral incisors. Exclusive agenesis of both maxillary canines is an extremely rare occurrence and only a few cases have been reported. Previous studies showed that the prevalence of maxillary canine agenesis varies between 0.07 and 0.13%. In recent studies on Indi...

  2. Facial emotion recognition in agenesis of the corpus callosum

    OpenAIRE

    Bridgman, Matthew W; Brown, Warren S.; Spezio, Michael L.; Leonard, Matthew K.; Adolphs, Ralph; Paul, Lynn K

    2014-01-01

    Background: Impaired social functioning is a common symptom of individuals with developmental disruptions in callosal connectivity. Among these developmental conditions, agenesis of the corpus callosum provides the most extreme and clearly identifiable example of callosal disconnection. To date, deficits in nonliteral language comprehension, humor, theory of mind, and social reasoning have been documented in agenesis of the corpus callosum. Here, we examined a basic social ability as yet not ...

  3. Bilateral pulmonary thromboembolism and Budd-Chiari syndrome in a patient with Crohn's disease on oral contraceptives Tromboembolismo de pulmón bilateral y síndrome de Budd-Chiari en paciente con enfermedad de Crohn y toma de anticonceptivos orales

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    M. Valdés Mas

    2009-09-01

    Full Text Available Budd-Chiari syndrome can be defined as an interruption or diminution of the normal blood flow out of the liver. Patients with Budd-Chiari syndrome present with varying degrees of symptomatology that can be divided into the following categories: fulminant, acute, subacute and chronic. The subacute form is the most common presentation. A majority of patients with Budd-Chiari syndrome have an underlying hypercoagulability state. We present the case of a young woman with Crohn's disease on oral contraceptives who developed bilateral pulmonary thromboembolism and Budd-Chiari syndrome.El síndrome de Budd-Chiari consiste en la interrupción o disminución de flujo de las venas suprahepáticas. Tiene una gran variabilidad clínica en cuanto a su forma de presentación siendo la más frecuente la forma subaguda. La gran mayoría de los pacientes responden a estados de hipercoagulabilidad. Presentamos el caso de una paciente joven con enfermedad de Crohn que estaba en tratamiento con anticonceptivos orales y desarrolló un cuadro clínico de tromboembolismo de pulmón bilateral y síndrome de Budd-Chiari.

  4. CONGENITAL VESICOUTERINE FISTULA ALONG WITH DISTAL VAGINAL AGENESIS, SOLITARY KIDNEY AND TONGUE TIE: A RARITY

    OpenAIRE

    Khalid; Amit; Rana Pratap; Ahsan; Takallum

    2015-01-01

    : Congenital vesicouterine fistula in association with vaginal agenesis and solitary renal agenesis has been rarely reported. We present a case of 19 year old female suffering from cyclical menouria for last years. Physical examination revealed absence of vagina. IVP revealed left renal agenesis and CT scan revealed left renal agenesis with vesicouterine fistula. Cystoscopy showed vesicouterine fistula located above trigone near midline. Vesicouterine fistula was repaired along wi...

  5. Patterns of tooth agenesis in patients with Crouzon or Apert syndrome

    NARCIS (Netherlands)

    J.H. Reitsma; E.M. Ongkosuwito; A.J. van Wijk; B. Prahl-Andersen

    2014-01-01

    Purpose: Dental agenesis is the most common anomaly of dental development and can be a component of a congenital syndrome. The purpose of this study was to evaluate the prevalence of agenesis and to describe patterns of tooth agenesis in patients with Crouzon or Apert syndrome compared with nonsyndr

  6. Original article patterns of tooth agenesis in patients with Crouzon or Apert syndrome

    NARCIS (Netherlands)

    J.H. Reitsma (Jacobus Harmen); E.M. Ongkosuwito (Edwin); A.J. van Wijk (Arjen); B. Prahl-Andersen (B.)

    2014-01-01

    textabstractPurpose: Dental agenesis is the most common anomaly of dental development and can be a component of a congenital syndrome. The purpose of this study was to evaluate the prevalence of agenesis and to describe patterns of tooth agenesis in patients with Crouzon or Apert syndrome compared w

  7. Bilateral hemimelia of radio in canine – case report
    Hemimelia bilateral de rádio em canino – relato de caso

    OpenAIRE

    Thalita Priscila da Silva Peres; Wilma Neres da Silva Campos; Thaís Ruiz; Yara Silva Meireles; Pedro Brandini Néspoli; Roberto Lopes de Souza

    2013-01-01

    Hemimelia or agenesis is a congenital morphological change that may affect the appendicular skeleton. This rare anomaly is the total or partial absence of one or more bones matched. It may present unilaterally or bilaterally, with the most frequent unilateral. In dogs, the most common form of the radial hemimelia is that causes deformity of the affected limb and severe functional limitations. Was Veterinary Hospital of the Federal University of Mato Grosso (HOVET/UFMT), a canine, female mongr...

  8. Msx1 Mutations: How Do They Cause Tooth Agenesis?

    OpenAIRE

    Wang, Y.; Kong, H; Mues, G.; D’Souza, R.

    2011-01-01

    Mutations in the transcription factors PAX9 and MSX1 cause selective tooth agenesis in humans. In tooth bud mesenchyme of mice, both proteins are required for the expression of Bmp4, which is the key signaling factor for progression to the next step of tooth development. We have previously shown that Pax9 can transactivate a 2.4-kb Bmp4 promoter construct, and that most tooth-agenesis-causing PAX9 mutations impair DNA binding and Bmp4 promoter activation. We also found that Msx1 by itself rep...

  9. Role of OHVIRA syndrome in renal agenesis: a case report

    Directory of Open Access Journals (Sweden)

    Hulya Ozturk

    2014-03-01

    Full Text Available A 13-year-old female was admitted with abdominal pain and dysmenorrhea. Ultrasonography and magnetic resonance imaging revealed OHVIRA syndrome (uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. A left hysterectomy was performed. The patient recovered fully following treatment with vaginal septum resection and drainage of a hematometrocolpos.

  10. Uterus didelphys with obstructed hemivagina and renal agenesis : mri findings

    OpenAIRE

    De Mey, J; Yazdi, A Talebian; De Smet, K; Ernst, C.; Desprechins, B

    2011-01-01

    Müllerian duct abnormalities (MDA) are developmental disorders leading to dysmorphism of the female genital tract. Currently the Buttram and Gibbons classification of these entities is widely used. We present a case of a young girl with uterus didelphys and ipsilateral renal agenesis.

  11. Role of OHVIRA syndrome in renal agenesis: a case report

    OpenAIRE

    Hulya Ozturk; Emine Dagistan; Tulay Ozlu

    2014-01-01

    A 13-year-old female was admitted with abdominal pain and dysmenorrhea. Ultrasonography and magnetic resonance imaging revealed OHVIRA syndrome (uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis). A left hysterectomy was performed. The patient recovered fully following treatment with vaginal septum resection and drainage of a hematometrocolpos.

  12. The relationship between smile attractiveness and esthetic parameters of patients with lateral agenesis treated with tooth recontouring or implants

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    De-Marchi LM

    2012-12-01

    Full Text Available Luciana Manzotti De-Marchi,1 Núbia Inocencya Pavesi Pini,2 Renata Corrêa Pascotto31Department of Dentistry, University Center of Maringá-CESUMAR, Maringá-PR/Brazil; 2Piracicaba Dental School, State University of Campinas, Piracicaba-SP/Brazil; 3Department of Dentistry, State University of Maringá, Maringá-PR/BrazilBackground: The purpose of this study was to associate smile esthetic judgment with dentofacial attributes of patients with unilateral and bilateral agenesis of maxillary lateral incisors treated with recontouring of canines or implants and patients with no agenesis (control.Material and methods: Forty-six participants were divided into two groups: those treated with recontouring (N = 26 and those treated with implants (N = 20. The participants in the control group (N = 22 were selected among dentistry students at the State University of Maringá, Brazil. Photographs of posed smiles (17 cm × 10 cm were evaluated with a 100-mm Visual Analog Scale. Smile attractiveness was judged by two groups: laypersons and dentists (N = 20 in each group. Judgment was classified into Unpleasant and Pleasant. Measurements of 11 smile attributes were done with ImageTool Version 3.0. These measurements were correlated with the type of judgment using the Pearson correlation coefficient.Results: The two groups of evaluators showed no rating difference (analysis of variance, P = 0.64, thus they were placed into a single group. No significant correlation was found between esthetic judgment and six smile attributes (incisor exposure, interlabial gap, width 3 to 3, smile index, right buccal corridor, and buccal corridor ratio. The control group showed more correlations with the unpleasant judgment type than the other groups.Conclusion: Some correlations between smile attributes and esthetic judgment were found, but other features of smiles not evaluated in this study may interfere in smile attractiveness.Keywords: esthetic, dental agenesis

  13. Miliary pulmonary cryptococcosis

    Science.gov (United States)

    Kelly, Shane; Marriott, Deborah

    2014-01-01

    A 32-year-old HIV positive male presents with fevers and a non-productive cough. Initial X-ray and subsequent computerised tomography of the chest shows a bilateral miliary pattern of pulmonary infiltration highly suggestive of disseminated tuberculosis. However subsequent results were consistent with disseminated cryptococcosis, including pulmonary involvement, with cryptococcus identified on transbronchial tissue biopsy, and on blood and cerebrospinal fluid cultures. Imaging features of pulmonary cryptococcosis are generally of well-defined pleural-based nodules and less commonly alveolar infiltrates, lymphadenopathy, pleural effusions or cavitating lesions. Miliary pulmonary infiltrates are an exceptionally rare presentation. PMID:25379393

  14. Renal tubular dysgenesis with hypocalvaria and ileocecal valve agenesis: an autopsy report

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    Ariel Barreto Nogueira

    2012-12-01

    Full Text Available Renal tubular dysgenesis (RTD is a rare, lethal, autosomal recessive disorder characterized by non-differentiation of the renal proximal convoluted tubules, resulting in oligohydramnios. It is usually diagnosed in the second trimester of pregnancy, following the oligohydramnios sequence, pulmonary hypoplasia and hypocalvaria. The prognosis is poor, and death usually occurs in utero or within the first few days of life. The pathogenesis of RTD is associated with the perinatal use of drugs, such as angiotensin- converting enzyme inhibitors, angiotensin II receptor antagonists, and anti- inflammatory drugs, as well as with fetal transfusion syndrome, genetic mutations in the pathway of the renin-angiotensin system pathway, cocaine snorting, or other pathological mechanisms that reduce renal blood flow. Here, we report the autopsy of a neonate born to consanguineous parents at 38 weeks of gestation, with RTD, decreased amniotic fluid, oligohydramnios sequence, hypocalvaria, pulmonary hypoplasia, and ileocecal valve agenesis. To our knowledge, the latter has never been reported associated with RTD.

  15. Waardenburg syndrome with familial unilateral renal agenesis: a new syndrome variant?

    Science.gov (United States)

    Webb, Katie M; Smith, Alisha J; Dansby, Linda M; Diskin, Charles J

    2015-06-01

    A 64-year-old man with Waardenburg syndrome presented with anuria and was subsequently discovered by renal ultrasound to have unilateral renal agenesis. The patient is one of three generations with incidental finding of renal agenesis also marked by the presence of Waardenburg syndrome. To our knowledge, there has been no mention elsewhere in the scientific literature of a variant of Waardenburg syndrome with associated renal agenesis. PMID:25402868

  16. Multivariate Analysis of Factors Affecting Presence and/or Agenesis of Third Molar Tooth

    OpenAIRE

    Alam, Mohammad Khursheed; Hamza, Muhammad Asyraf; Khafiz, Muhammad Aizuddin; Rahman, Shaifulizan Abdul; Shaari, Ramizu; Hassan, Akram

    2014-01-01

    To investigate the presence and/or agenesis of third molar (M3) tooth germs in orthodontics patients in Malaysian Malay and Chinese population and evaluate the relationship between presence and/or agenesis of M3 with different skeletal malocclusion patterns and sagittal maxillomandibular jaw dimensions. Pretreatment records of 300 orthodontic patients (140 males and 160 females, 219 Malaysian Malay and 81 Chinese, average age was 16.27±4.59) were used. Third-molar agenesis was calculated with...

  17. Corpus callosum agenesis: Role of fetal magnetic resonance imaging

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    Achour Radhouane

    2016-05-01

    Full Text Available Corpus callosum agenesis (CCA was evaluated by ultrasound examination and magnetic resonance imaging (MRI with many studies. Ultrasonography was able to suspect CCA by indirect signs but a definitive diagnosis of CCA was achieved in rare cases. MRI was able to diagnose complete CCA in majority of cases. Additional neurological abnormalities including heterotopia, gyration anomaly, asymmetry of the cerebral hemispheres, and Dandy-Walker variant were documented, as well as an ocular anomaly which was described, by MRI examination. Prenatal counseling for fetal agenesis of the corpus callosum is difficult as the prognosis is uncertain. The association with other cerebral abnormalities increases the likelihood of a poor outcome and ultrasonographic assessment of the fetal brain is limited. We found MRI to be a safe and useful additional procedure to complement ultrasonographic diagnosis or suspicion of CCA.

  18. A rare case of Turner's syndrome presenting with Mullerian agenesis.

    Science.gov (United States)

    Vaddadi, Suresh; Murthy, Ramana S V; Rahul, C H; Kumar, Vinod L

    2013-10-01

    Turner's syndrome also called as Ullrich Turner's syndrome, is a disease of unclear pathogenesis characterized by complete or partial absence of one sex chromosome, with or without cell line mosaicism in a phenotypic female with short stature. Various anomalies result in a constellation of features, of which the most disturbing is primary amenorrhea due to gonadal dysgenesis. Hormone therapy in these patients can often result in successful menstruation, and scope for subsequent pregnancy because of anatomically normal uterus and vagina. Coexisting Mullerian agenesis in these patients can jeopardize the chances of future pregnancy as they have associated structural abnormalities of the uterus and vagina. We report a rare case of middle-aged female with Turner's syndrome and Mullerian agenesis having absent secondary sexual characters and missing uterus with incompletely formed vagina. PMID:24672170

  19. BICORNUATE UTERUS WITH CERVICAL ATRESIA AND VAGINAL AGENESIS ASSOCIATED WITH OVARIAN ENDOMETRIOSIS - A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Alpana

    2013-06-01

    Full Text Available ABSTRACT: Vaginal agenesis combined with a functional uterus is a rare type of Mullerian duct an omaly. Only 7 – 8% of patients with vaginal agenesis have a functional uterus. Women born with vaginal agenesis, cervical atresia combined with a functioning endometrium typically present with hematometra , disabling pelvic pain and progressively worsening en dometriosis. Almost all need an abdominal hysterectomy for relief. We report a case of severe endometriosis in a 29 years old woman having a bicornuate uterus with cervical atresia and vaginal agenesis.

  20. CONGENITAL VESICOUTERINE FISTULA ALONG WITH DISTAL VAGINAL AGENESIS, SOLITARY KIDNEY AND TONGUE TIE: A RARITY

    Directory of Open Access Journals (Sweden)

    Khalid

    2015-11-01

    Full Text Available : Congenital vesicouterine fistula in association with vaginal agenesis and solitary renal agenesis has been rarely reported. We present a case of 19 year old female suffering from cyclical menouria for last years. Physical examination revealed absence of vagina. IVP revealed left renal agenesis and CT scan revealed left renal agenesis with vesicouterine fistula. Cystoscopy showed vesicouterine fistula located above trigone near midline. Vesicouterine fistula was repaired along with uterine preservation. Sigmoid colon neovagina was created. Patient is doing well and menstruating per vagina till last followup

  1. Thyroid isthmus agenesis associated with solitary nodule: A case report

    OpenAIRE

    Schanaider, Alberto; de Oliveira, Paschoal Josias

    2008-01-01

    Background Agenesis of the isthmus associated with nontoxic solitary nodular goiter is a rare congenital anomaly. Imaging data exceptionally has been previously reported in the English language literature. Case presentation Preoperative assessment of a 22-year-old white man patient showed an asymptomatic nodule of the thyroid at the left lobe, measuring 2.3 × 1.5 cm in diameter without regional lymphadenopathy. At surgical exploration it was seen an absence of the thyroid isthmus. Conclusion ...

  2. Tracheal agenesis: A rare but fatal congenital anomaly

    OpenAIRE

    Ergun, Suzan; Tewfik, Ted; Daniel, Sam

    2011-01-01

    In this report we describe a newborn with a rare case of Type II tracheal agenesis and bronchoesophageal fistula. Polyhydramnios and suspected esophageal atresia were identified during routine pre-natal ultrasound screening. Upon delivery, rigid bronchoscopy, esophagoscopy, and intraoperative fluoroscopy were performed, where both bronchi and the carina showed unusual horizontal orientation making it difficult to identify the fistula. However, a post mortem CT confirmed the diagnosis of an is...

  3. Agenesis of the corpus callosum. An autopsy study in fetuses.

    Science.gov (United States)

    Kidron, Debora; Shapira, Daniel; Ben Sira, Liat; Malinger, Gustavo; Lev, Dorit; Cioca, Andreea; Sharony, Reuven; Lerman Sagie, Tally

    2016-02-01

    Agenesis of the corpus callosum is currently diagnosed prenatally with ultrasound and MRI. While the diagnostic aspects of callosal defects are widely addressed, anatomo-histological data from fetal autopsies are sparse. Callosal defects were present in 50 fetal autopsies. Four distinct groups of complete, partial, hypoplastic, and mixed defects were determined by the gross and histologic details of the corpus callosum. These details helped to rule out other midline defects such as holoprosencephaly. Additional autopsy findings enabled specific diagnoses and suggested etiopathogeneses. Hypoplastic and mixed defects were associated with more abnormalities of the cerebral hemispheres and internal organs. The four groups did not differ according to gender, external dysmorphism, or cerebellar and brainstem anomalies. Defects were classified as syndromic (68 %), encephaloclastic (8 %), undetermined (14 %), or isolated (10 %) based on the autopsy findings. Isolated agenesis of the corpus callosum was diagnosed in only 10 % of the cases in this series, compared to higher numbers diagnosed by prenatal ultrasonography and MRI. Therefore, the autopsy, through its detailed, careful evaluation of external, as well as gross and histological internal features, can elucidate the etiopathogenesis of agenesis of the corpus callosum and suggest specific diagnoses which cannot be ascertained by prenatal imaging. PMID:26573426

  4. Primary pulmonary alveolar proteinosis

    OpenAIRE

    Šarac Sanja; Milić Rade; Zolotarevski Lidija; Aćimović Slobodan; Tomić Ilija; Plavec Goran

    2012-01-01

    Introduction. Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary) and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about 0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray shows bilateral perihilar infiltrates. Open-lung biopsy is the gold standard for the diagnosis. Treatment includes w...

  5. Bilateral anterior shoulder dislocation

    OpenAIRE

    Meena, Sanjay; Saini, Pramod; Singh, Vivek; Kumar, Ramakant; Trikha, Vivek

    2013-01-01

    Shoulder dislocations are the most common major joint dislocations encountered in the emergency departments. Bilateral shoulder dislocations are rare and of these, bilateral posterior shoulder dislocations are more prevalent than bilateral anterior shoulder dislocations. Bilateral anterior shoulder dislocation is very rare. We present a case of 24-year-old male who sustained bilateral anterior shoulder dislocation following minor trauma, with associated greater tuberosity fracture on one side...

  6. Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: Common presentation of an unusual variation

    OpenAIRE

    Adair, Luther; Georgiades, Maria; Osborne, Rhonda; Ng, Tsz

    2011-01-01

    Mullerian duct abnormalities are congenital malformations that are easily missed and can lead to incorrect diagnosis and unnecessary operative procedures. In this case, a young female presented with cyclic pelvic pain that continued after previous surgical resection of an ovarian cyst. Further investigation with clinical examinations and multimodality imaging demonstrated ipsilateral renal agenesis and a Class III Mullerian duct anomaly (MDA) requiring a second operative procedure. It is beli...

  7. Pulmonary Hyalinizing Granuloma in HIV/AIDS

    Directory of Open Access Journals (Sweden)

    Theresa Liu

    2007-01-01

    Full Text Available A 55-year-old man who was recently diagnosed with HIV/AIDS developed multiple bilateral pulmonary nodules after starting highly active antiretroviral therapy. Workup confirmed the diagnosis of pulmonary hyalinizing granuloma. This is the first described case of pulmonary hyalinizing granuloma in HIV/AIDS, and may represent a rare form of immune reconstitution inflammatory syndrome.

  8. Pulmonary hyalinizing granuloma in HIV/AIDS.

    Science.gov (United States)

    Liu, Theresa; Kyrollos, Maggy; Kravcik, Stephen

    2007-09-01

    A 55-year-old man who was recently diagnosed with HIV/AIDS developed multiple bilateral pulmonary nodules after starting highly active antiretroviral therapy. Workup confirmed the diagnosis of pulmonary hyalinizing granuloma. This is the first described case of pulmonary hyalinizing granuloma in HIV/AIDS, and may represent a rare form of immune reconstitution inflammatory syndrome. PMID:18923729

  9. Pulmonary Hyalinizing Granuloma in HIV/AIDS

    OpenAIRE

    Theresa Liu; Maggy Kyrollos; Stephen Kravcik

    2007-01-01

    A 55-year-old man who was recently diagnosed with HIV/AIDS developed multiple bilateral pulmonary nodules after starting highly active antiretroviral therapy. Workup confirmed the diagnosis of pulmonary hyalinizing granuloma. This is the first described case of pulmonary hyalinizing granuloma in HIV/AIDS, and may represent a rare form of immune reconstitution inflammatory syndrome.

  10. Pulmonary hyalinizing granuloma mimicking lung carcinoma.

    Science.gov (United States)

    Basoglu, A; Findik, S; Celik, B; Yildiz, L

    2006-06-01

    Pulmonary hyalinizing granuloma has rarely been reported and is a benign entity of unknown origin. The chest radiograph reveals multiple and frequently bilateral pulmonary nodules. We describe a patient with pulmonary hyalinizing granuloma who presented with a central mass in the left lung mimicking lung carcinoma. PMID:16755455

  11. Pulmonary hyalinizing granuloma in HIV/AIDS

    Science.gov (United States)

    Liu, Theresa; Kyrollos, Maggy; Kravcik, Stephen

    2007-01-01

    A 55-year-old man who was recently diagnosed with HIV/AIDS developed multiple bilateral pulmonary nodules after starting highly active antiretroviral therapy. Workup confirmed the diagnosis of pulmonary hyalinizing granuloma. This is the first described case of pulmonary hyalinizing granuloma in HIV/AIDS, and may represent a rare form of immune reconstitution inflammatory syndrome. PMID:18923729

  12. Pulmonary Aplasia in an Adult : A Case Report

    Directory of Open Access Journals (Sweden)

    Nurettin Yiyit

    2016-01-01

    Full Text Available Pulmonary aplasia is a rare congenital anomaly in which there are absence of pulmonary vessels, bronchus and parenchyma. It is distinguished from pulmonary agenesis by the presence of rudimentary stump bronchus. Patients are usually diagnosed in childhood. Patients without additional anomaly and the patients with mild disease can be diagnosed in adulthood. The left lung was not observed at the chest X-ray of 19-year-old male patient with respiratory distress in exercise. Left lung aplasia was diagnosed by computed tomography and ventilation perfusion scintigraphy. The patients with lung aplasia have an increased risk of infections. Therefore the follow-up of the patients is important.

  13. Maxillary sinus agenesis - report of two cases; Agenesia do seio maxilar - relato de dois casos

    Energy Technology Data Exchange (ETDEWEB)

    Pierre, Jorge Henrique Arraes de Alencar; Santana, Expedito Araujo

    2000-04-01

    Agenesis or aplasia of the maxillary sinuses is an extremely rare condition, and only eight cases have been reported in the world medical literature. These malformations may arise as a result of developmental defects. Two cases of unilateral agenesis of the maxillary sinus are presented and the radiological abnormalities and the embryology are discussed. The literature is also reviewed. (author)

  14. Prevalence of Third Molar Agenesis: Associated Dental Anomalies in Non-Syndromic 5923 Patients.

    Science.gov (United States)

    Sujon, Mamun Khan; Alam, Mohammad Khursheed; Rahman, Shaifulizan Abdul

    2016-01-01

    The aim of this study was to investigate the prevalence of third molar agenesis and other associated dental anomalies in Bangladeshi population and to investigate the relationship of other dental anomalies with the third molar presence/agenesis. A retrospective study was performed using panoramic radiographs of 5923 patients, who ranged in age from 10 to 50 years. All radiographs were analyzed by Planmeca Romexis® 3.0 software (Planmeca Oy, Helsinki, Finland). Pearson chi-square and one way ANOVA (Post Hoc) test were conducted. The prevalence of third molar agenesis was 38.4%. The frequency of third molar agenesis was significantly higher in females than males (p dental anomalies was 6.5%, among them hypodontia was 3.1%. Prevalence of third molar agenesis varies in different geographic region. Among the other dental anomalies hypodontia was more prevalent. PMID:27580050

  15. Agenesis of Ductus Venosus Presenting with Dilated Inferior Vena Cava with Favorable Outcome

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    Omer Dai

    2014-04-01

    Full Text Available The ductus venosus regulates the distribution of oxygen and placental nutrients by restricting the centralization of blood flow in fetal circulation. The ductus venosus is a small vein transmitting oxygen-rich blood from the umbilical vein to the fetal heart.. Increasing numbers of case reports are being published about ductus venosus agenesis with cardiac structural abnormalities, other malformations, chromosomal abnormalities, and stillbirth. Heart failure, hydrops and unexplained polyhydramnios could also be associated with ductus venosus agenesis. In the follow-up of fetuses with ductus venosus agenesis, care must be taken to examine for hydrops and heart failure. The agenesis of ductus venosus is often only found in fetuses by coincidentally. Herein we discuss the prenatal diagnosis of isolated agenesis of the ductus venosus presenting with a dilated inferior vena cava and without any disturbance of fetal hemodynamics and any other malformation. In conclusion a dilated inferior vena cava can be a sign of ductus venosus agenesia.

  16. Bilateral Gonadal Cysts and Late Diagnosis of Androgen Insensitivity Syndrome Treated by Laparoscopic Gonadectomy

    Directory of Open Access Journals (Sweden)

    D. Tourlakis

    2011-01-01

    Full Text Available Background. Complete androgen insensitivity syndrome is a rare syndrome in which the uterus is absent and testes rather than ovaries are present. Patients usually visit a gynecologist due to primary amenorrhea. Case. A forty-eight-year-old woman with lower abdominal pain and anamnesis of uterus agenesis was operated on due to bilateral cystic masses. A 5 × 3 × 1.2 cm left adnexal cyst revealed the presence of a serous cyst with a hypoplastic ductus deferens. A smaller cyst of the right adnexa revealed immature testis tissue with Leydig-cell hyperplasia. After karyotype and hormonal examinations, laparoscopic gonadectomy was performed. Conclusion. Attention should be paid in all cyst-removing operations in cases of uterus agenesis, due to the high incidence of malignancy. Not of less importance is the issue of informing the patient in the most appropriate way.

  17. Multivariate analysis of factors affecting presence and/or agenesis of third molar tooth.

    Directory of Open Access Journals (Sweden)

    Mohammad Khursheed Alam

    Full Text Available To investigate the presence and/or agenesis of third molar (M3 tooth germs in orthodontics patients in Malaysian Malay and Chinese population and evaluate the relationship between presence and/or agenesis of M3 with different skeletal malocclusion patterns and sagittal maxillomandibular jaw dimensions. Pretreatment records of 300 orthodontic patients (140 males and 160 females, 219 Malaysian Malay and 81 Chinese, average age was 16.27±4.59 were used. Third-molar agenesis was calculated with respect to race, genders, number of missing teeth, jaws, skeletal malocclusion patterns and sagittal maxillomandibular jaw dimensions. The Pearson chi-square test and ANOVA was performed to determine potential differences. Associations between various factors and M3 presence/agenesis groups were assessed using logistic regression analysis. The percentages of subjects with 1 or more M3 agenesis were 30%, 33% and 31% in the Malaysian Malay, Chinese and total population, respectively. Overall prevalence of M3 agenesis in male and female was equal (P>0.05. The frequency of the agenesis of M3s is greater in maxilla as well in the right side (P>0.05. The prevalence of M3 agenesis in those with a Class III and Class II malocclusion was relatively higher in Malaysian Malay and Malaysian Chinese population respectively. Using stepwise regression analyses, significant associations were found between Mx (P<0.05 and ANB (P<0.05 and M3 agenesis. This multivariate analysis suggested that Mx and ANB were significantly correlated with the M3 presence/agenesis.

  18. Agenesis of the dorsal mesentery presenting in an adolescent

    Directory of Open Access Journals (Sweden)

    Anith Chacko

    2013-03-01

    Full Text Available Agenesis of the dorsal mesentery is a rare occurrence that usually presents in children. It is associated with proximal small bowel malrotation as well as high jejunal atresia with discontinuity of the small bowel. We present a case report of an adolescent presenting with clinical features of proximal small bowel obstruction (confirmed on imaging as well as acute pancreatitis. At laparotomy, he was found to have no dorsal mesentery, without small bowel atresia, and the duodenum was fixed to the posterior abdominal wall. The patient recovered well and remained symptom-free.

  19. Subpleural pulmonary hyalinizing granuloma presenting as a solitary pulmonary nodule.

    Science.gov (United States)

    Na, Kook Joo; Song, Sang Yun; Kim, Jo Heon; Kim, Young Chul

    2007-08-01

    We introduce a case of pulmonary hyalinizing granuloma presented as a solitary pulmonary nodule located subpleurally. The patient was a 57-year-old man who had abnormal chest roentgenograms showing a solitary pulmonary nodule in the right lower lung field. The nodule was resected for definitive diagnosis and histopathologically proved to be pulmonary hyalinizing granuloma. In previously reported cases, most patients had ill-defined margins and usually bilateral, multiple lesions radiographically. In our case, the subpleural location is an uncommon location of this rare entity. PMID:17762348

  20. UNILATERAL AND COMPLETE AGENESIS OF RIGHT PECTORALIS MAJOR MUSCLE

    Directory of Open Access Journals (Sweden)

    Vaishali Paranjape

    2015-12-01

    Full Text Available Introduction: Pectoralis major Myocutaneous flaps (PMMC are extensively used for Head, Neck, Face and post mastectomy mammary gland reconstructive surgeries. Pectoralis major is responsible for flexion, adduction and medial rotation of shoulder joint. Its absence may obvious because of compromised movement of shoulder joint or as a part Poland’s syndrome or may be sporadic as seen in our case. Case study: Pectoralis major muscle was completely absent on right side of an adult female cadaver. Result: Complete unilateral agenesis of right Pectoralis major muscle was found in an adult female cadaver during routine undergraduate dissection in the department of Anatomy. Right Mammary gland was lying directly on hypertrophied Pectoralis minor muscle. Discussion: Awareness about absence of Pectoralis major muscle is a must for planning and success of various reconstructive surgeries, flawless interpretation of Radiographs, CT and MRI scans. Unilateral and complete agenesis of Pectoralis major muscle not associated with any other anomaly visible to naked eye as seen in our case is a rare finding and hence needs to be documented.

  1. Predicting Agenesis of the Mandibular Second Premolar from Adjacent Teeth.

    Directory of Open Access Journals (Sweden)

    Geetanjali Sharma

    Full Text Available Early diagnosis of agenesis of the mandibular second premolar (P2 enhances management of the dental arch in the growing child. The aim of this study was to explore the relationship in the development of the mandibular first molar (M1 and first premolar (P1 at early stages of P2 (second premolar. Specifically, we ask if the likelihood of P2 agenesis can be predicted from adjacent developing teeth. We selected archived dental panoramic radiographs with P2 at crown formation stages (N = 212 and calculated the likelihood of P2 at initial mineralisation stage 'Ci' given the tooth stage of adjacent teeth. Our results show that the probability of observing mandibular P2 at initial mineralisation stage 'Ci' decreased as both the adjacent P1 and M1 matured. The modal stage at P2 'Ci' was P1 'Coc' (cusp outline complete and M1 'Crc' (crown complete. Initial mineralisation of P2 was observed up to P1 'Crc' and M1 stage 'R½' (root half. The chance of observing P2 at least 'Coc' (coalescence of cusps was considerably greater prior to these threshold stages compared to later stages of P1 and M1. These findings suggest that P2 is highly unlikely to develop if P1 is beyond 'Crc' and M1 is beyond 'R½'.

  2. The WNT10A gene in Ectodermal Dysplasias and Selective Tooth Agenesis

    OpenAIRE

    Mues, Gabriele; Bonds, John; Xiang, Lilin; Vieira, Alexandre R; Seymen, Figen; Klein, Ophir; D’Souza, Rena N.

    2014-01-01

    Mutations in the WNT10A gene were first detected in the rare syndrome odonto-onycho-dermal dysplasia (OODD, OMIM257980) but have now also been found to cause about 35-50% of selective tooth agenesis (STHAG4, OMIM150400), a common disorder that mostly affects the permanent dentition. In our random sample of tooth agenesis patients, 40 percent had at least one mutation in the WNT10A gene. The WNT10A Phe228Ile variant alone reached an allele frequency of 0.21 in the tooth agenesis cohort, about ...

  3. Primary pulmonary alveolar proteinosis

    Directory of Open Access Journals (Sweden)

    Šarac Sanja

    2012-01-01

    Full Text Available Introduction. Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about 0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray shows bilateral perihilar infiltrates. Open-lung biopsy is the gold standard for the diagnosis. Treatment includes whole-lung lavage, application of granulocyte-macrophage colonystimulating factor and lung transplantation. Case report. We reported a 51 year-old man with primary form of the disease. It was the second case of this extremely rare disease in the past 30 years in our clinic. The symptoms were longlasting dry cough, fever and physical deterioration. Chest Xray revealed bilateral pulmonary infiltrates; computed tomography showed patchy ground-glass opacification with interlobular thickening. The diagnosis was established by open lung biopsy. Additional tests were performed to exclude secondary form of the disease. Conclusion. We presented a rare clinical entity with typical clinical features and clinical and radiological course of the disease, in order to improve differential diagnostic approach to patients with bilateral lung infiltrations. In patients with pulmonary alveolar proteinosis timely diagnosis and adequate treatment can improve a prognosis.

  4. Pulmonary edema

    Science.gov (United States)

    ... congestion; Lung water; Pulmonary congestion; Heart failure - pulmonary edema ... Pulmonary edema is often caused by congestive heart failure . When the heart is not able to pump efficiently, blood ...

  5. Agenesis of dorsal pancreas with eventration of diaphragm and intrapancreatic pseudocyst: a rare entity

    Directory of Open Access Journals (Sweden)

    Poras Chaudhary

    2013-02-01

    Full Text Available A case of acute pancreatitis in a 43-year-old male patient, which was eventually diagnosed as agenesis of dorsal pancreas with eventration of left hemidiaphragm and intrapancreatic pseudocyst is being reported.

  6. Lung hypoplasia and patellar agenesis in Ehlers-Danlos syndrome.

    Science.gov (United States)

    Pradhan, P; Deb, J; Deb, R; Chakrabarti, S

    2009-12-01

    A 22-year-old male patient was admitted with severe cough associated with purulent expectoration, left-sided chest pain and breathlessness. There was a history of recurrent respiratory ailments since childhood. The patient appeared younger than his chronological age. His face and ears were both dysmorphic. Clinically, the patient was diagnosed with Ehlers-Danlos syndrome (EDS). Computed tomography of the thoracic region revealed hypoplasia of the left lung and hyperplasia of the right lung. Both the patellae were absent. However, ultrasonography of his abdomen, echocardiography and other routine blood and urine examination showed no gross abnormalities. Although other respiratory tract abnormalities with EDS are not uncommon, unilateral lung hypoplasia and patellar agenesis in EDS make this case unique. PMID:20087544

  7. Obstructed hemivagina and ipsilateral renal agenesis with intestinal malrotation.

    Science.gov (United States)

    Morino, Masaaki; Hoshino, Masaya; Musha, Ikuma

    2013-08-01

    The combination of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis represents a rare congenital anomaly called Herlyn-Werner-Wunderlich syndrome (HWWS) or obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Several anomalies have recently been reported to be associated with this syndrome. The present patient with HHWS had multiple anomalies: intestinal non-rotation, anomalies of the large vessels of the abdomen including duplication of the inferior vena cava and a high-riding aortic bifurcation, and hypodontia. Hypodontia has never been reported in a patient with HWWS. The patient underwent a preventative Ladd's procedure and vaginal reconstruction. To prevent serious complications from concomitant anomalies such as intestinal malrotation, a patient with HWWS should be evaluated in detail for associated malformations. PMID:23910815

  8. Herlyn-Werner-Wunderlich syndrome (uterus didelphys, blind hemivagina and ipsilateral renal agenesis) - a case report

    OpenAIRE

    Arıkan, İlker İnan; Harma, Müge; Harma, Mehmet İbrahim; Bayar, Ülkü; Barut, Aykut

    2010-01-01

    Uterovaginal duplication with obstructed hemivagina and ipsilateral renal agenesis is referred to as the Herlyn-Werner-Wunderlich (HWW) syndrome. A 17 year old woman presented with right pelvic pain and dysmenorrhea, present since menarche at 13 and worsening over the past year. Ultrasound examination revealed a right pelvic mass (5×5 cm), double endometrial echoes, and hematocolpos. A right pelvic mass, agenesis of the right kidney, double uterus, and blind hemivagina with hematocolpos were ...

  9. Left lung agenesis discovered by a spontaneous pneumothorax in a 20-year-old girl.

    Science.gov (United States)

    Hentati, Abdessalem; Neifar, Chawki; Abid, Walid; M'saad, Sameh

    2016-01-01

    Lung agenesis is a rare condition which prognosis widely depends on associated malformations. Clinical presentation is so variable and diagnosis is often made in childhood. Here, we present a case of a 20-year-old girl who was admitted because of a spontaneous pneumothorax. Explorations concluded at a left lung agenesis, a hyperinflated right lung crossing the midline with a corresponding pneumothorax. There was no malformation else. This congenital condition and treatment for this rare presentation are discussed in detail. PMID:27051112

  10. Mutation analysis by direct and whole exome sequencing in familial and sporadic tooth agenesis

    Science.gov (United States)

    Salvi, Alessandro; Giacopuzzi, Edoardo; Bardellini, Elena; Amadori, Francesca; Ferrari, Lia; De Petro, Giuseppina; Borsani, Giuseppe; Majorana, Alessandra

    2016-01-01

    Dental agenesis is one of the most common congenital craniofacial abnormalities. Dental agenesis can be classified, relative to the number of missing teeth (excluding third molars), as hypodontia (1 to 5 missing teeth), oligodontia (6 or more missing teeth), or anodontia (lack of all teeth). Tooth agenesis may occur either in association with genetic syndromes, based on the presence of other inherited abnormalities, or as a non-syndromic trait, with both familiar and sporadic cases reported. In this study, we enrolled 16 individuals affected by tooth agenesis, prevalently hypodontia, and we carried out direct Sanger sequencing of paired box 9 (PAX9) and Msh homeobox 1 (MSX1) genes in 9 subjects. Since no mutations were identified, we performed whole exome sequencing (WES) in the members of 5 families to identify causative gene mutations either novel or previously described. Three individuals carried a known homozygous disease mutation in the Wnt family member 10A (WNT10A) gene (rs121908120). Interestingly, two of these individuals were siblings and also carried a heterozygous functional variant in EDAR-associated death domain (EDARADD) (rs114632254), another disease causing gene, generating a combination of genetic variants never described until now. The analysis of exome sequencing data in the members of other 3 families highlighted new candidate genes potentially involved in tooth agenesis and considered suitable for future studies. Overall, our study confirmed the major role played by WNT10A in tooth agenesis and the genetic heterogeneity of this disease. Moreover, as more genes are shown to be involved in tooth agenesis, WES analysis may be an effective approach to search for genetic variants in familiar or sporadic tooth agenesis, at least in more severe clinical manifestations. PMID:27665865

  11. Heterotaxy syndrome with associated agenesis of dorsal pancreas and polysplenia: A case report

    Directory of Open Access Journals (Sweden)

    Syed Althaf Ali1

    2015-01-01

    Full Text Available Heterotaxy syndrome is a rare embryological disorder comprising of polysplenia, partial agenesis of dorsal pancreas, malrotation of gut, cardiac and vascular anomalies resulting from failure of development of the usual left–right asymmetry of organs. We report a rare case of heterotaxy syndrome with polysplenia, partial agenesis of dorsal pancreas and malrotation of gut in a 28 year female presenting with subacute intestinal obstruction along with imaging illustrations, brief discussion and thorough review of literature.

  12. Novel GATA6 Mutations in Patients with Pancreatic Agenesis and Congenital Heart Malformations

    OpenAIRE

    Chao, Christina S.; McKnight, Kristen D.; Cox, Kenneth L; Anne L Chang; Kim, Seung K; Feldman, Brian J.

    2015-01-01

    Patients with pancreatic agenesis are born without a pancreas, causing permanent neonatal diabetes and pancreatic enzyme insufficiency. These patients require insulin and enzyme replacement therapy to survive, grow, and maintain normal blood glucose levels. Pancreatic agenesis is an uncommon condition but high-throughput sequencing methods provide a rare opportunity to identify critical genes that are necessary for human pancreas development. Here we present the clinical history, evaluation, ...

  13. Agenesis of premolar associated with submerged primary molar and a supernumerary premolar: An unusual case report

    Directory of Open Access Journals (Sweden)

    S. V. S. G. Nirmala

    2012-01-01

    Full Text Available The combination of submerged primary molar, agenesis of permanent successor with a supernumerary in the same place is very rare. The purpose of this article is to report a case of submerged mandibular left second primary molar with supernumerary tooth in the same region along with agenesis of second premolar in an 11-year-old girl, its possible etiological factors, and a brief discussion on treatment options.

  14. Lateral incisor agenesis, canine impaction and characteristics of supernumerary teeth in a South European male population

    OpenAIRE

    Livas, Christos; Bornstein, Michael; Delli, Konstantina

    2013-01-01

    Objective: To assess the prevalence of lateral incisor agenesis impacted canines and supernumerary teeth in a young adult male population. Materials and Methods: The panoramic radiographs of 1745 military students (mean age: 18.6 ± 0.52 years) who attended the Center of Aviation Medicine of the Armed Forces of Greece during the period 1997-2011 were initially analyzed for lateral incisor agenesis by two observers. After exclusion of the known orthodontic cases, a subgroup of 1636 examinees (m...

  15. Presentación de un caso: trombosis venosa profunda bilateral de etiología infrecuente Case report: rare bilateral deep venous thrombosis

    Directory of Open Access Journals (Sweden)

    Mónica Fernández del Castillo Ascanio

    2012-03-01

    Full Text Available La agenesia de vena cava inferior (VCI es una patología poco frecuente que suele diagnosticarse a raíz de una trombosis venosa secundaria sintomática de las venas ilíacas. Presentamos el caso de un varón de 28 años que acude al centro de salud por dolor en miembros inferiores y datos de insuficiencia venosa. Se visualiza mediante ecografía trombosis venosa profunda (TVP bilateral.Agenesis of the inferior vena cava (IVC is a rare condition usually diagnosed as secondary to symptomatic deep venous thrombosis of iliac veins. We report a 28-year-old male that was admitted in the medical center due to pain in both legs and history of venous insufficiency. Ultrasound reveáis bilateral deep venous thrombosis.

  16. Pulmonary manifestations revealing Rosai-Dorfman disease.

    Science.gov (United States)

    Goupil de Bouillé, J; de Muret, A; Diot, E; Dumont, P; Plantier, L; Diot, P; Marchand-Adam, Sylvain

    2015-01-01

    Rosai-Dorfman disease is a rare non-Langerhans cell histiocytosis, mainly involving cervical nodes. We present the case of a patient with a pulmonary form of Rosai-Dorfman disease without peripheral or intra-thoracic lymph nodes, characterized by the presence of pulmonary nodules and cysts associated with bilateral pleural effusions. PMID:26422575

  17. Primary pulmonary cryptococcosis

    OpenAIRE

    Asadi Gharabaghi, Mehrnaz; Allameh, Seyyed Farshad

    2014-01-01

    Here, we report a case of primary cryptococcal pneumonia in a 25-year-old woman who presented with several weeks’ history of cough, dyspnoea and night sweating. These symptoms started in the third trimester of her pregnancy. She was being treated for infertility and got pregnant with in vitro fertilisation. On chest imaging, there were bilateral air space consolidation and cavitary lesions. Fungal pulmonary infection was diagnosed after surgical lung biopsy. She received fluconazole 400 mg pe...

  18. Pregnancy following bilateral salpingectomy

    DEFF Research Database (Denmark)

    Oturai, Annette Bang

    2008-01-01

    This report presents a rare case of spontaneous pregnancy following bilateral salpingectomy. A woman with a history of bilateral salpingectomy was admitted to hospital because of abdominal pain and positive urine HCG. Surprisingly, ultrasound confirmed a live intrauterine fetus. The pregnancy...... was unwanted, and the woman decided to terminate the pregnancy. She was offered diagnostic examination to localise a potential fistula, but she declined. In a MEDLINE search of English literature this is only the second case of spontaneous pregnancy following bilateral salpingectomy Udgivelsesdato: 2008/4/21...

  19. Pulmonary Hyalinizing Granuloma Mimicking Metastatic Lung Cancer

    OpenAIRE

    Nuri Düzgün; Ercan Kurtipek; Hıdır Esme; Meryem İlkay Eren Karanis; İsmet Tolu

    2015-01-01

    Pulmonary hyalinizing granuloma is a very rare benign condition, which usually manifests as solitary and sometimes as multiple pulmonary nodules. Deposition of immune complexes in the lung parenchyma due to hypersensitivity reactions is implicated in the etiology of pulmonary hyalinizing granuloma. A 59-year-old female patient who presented to our clinic with complaints of chest pain and cough had bilateral, multiple, and rounded lesions with regular margins suggesting metastatic lung disease...

  20. Bilateral Ultrasound Guided Supraclavicular Block in a Patient on Antiplatelet Drugs

    Directory of Open Access Journals (Sweden)

    Lokesh Kumar KS

    2015-04-01

    Full Text Available A 63 year old male hypertensive and diabetic patient, with coronary artery and chronic obstructive pulmonary disease, presented with bilateral both bone forearm fracture. Open reduction and internal fixation was done successfully with bilateral ultrasound guided supraclavicular block. The problems associated with peripheral nerve block in an Ischemic Heart Disease (IHD patient on antiplatelet therapy are discussed.

  1. Bilateral Primary Intraocular Lymphoma

    OpenAIRE

    Mehrdad Karimi; Masoud Soheilian; Mozhgan Rezaei Kanavi

    2011-01-01

    Purpose: To report a case of bilateral primary intraocular lymphoma. Case report: A 33-year-old man presented with bilateral blurred vision since two years ago. Examination revealed large keratic precipitates, anterior chamber reaction, posterior subcapsular cataracts, and vitreous infiltration. After a short trial of topical and periocular steroids, diagnostic 25-gauge pars plana vitrectomy was performed and cytologic evaluation of the aspirate confirmed a diagnosis of intraocular lympho...

  2. Symptomatic pulmonary hyalinizing granuloma.

    Science.gov (United States)

    Ramirez, J; Mehta, J B; Taylor, R A; Byrd, R P; Roy, T M

    1998-09-01

    An otherwise healthy 37-year-old man came to the emergency room with left-sided dull chest pain of 4 weeks' duration. Physical examination, laboratory studies, and electrocardiogram were all unremarkable. A chest x-ray film revealed calcified pulmonary nodules. Computed tomography of the chest confirmed bilateral parenchymal cavitary lesions. Via limited thoracotomy, a tan nodule measuring 2.5 to 3.0 cm in diameter was excised from the left upper lobe. Histopathologic examination revealed a well circumscribed lesion and extensive lamellar hyalinization. A few foci of finely granular calcification were present within the hyalinizing areas. After surgery and short-term use of nonnarcotic analgesics, the chest pain resolved. Although pulmonary hyalinizing granuloma (PHG) is known to produce cavitating lesions, calcification at multiple sites is also consistent with this diagnosis. Clinicians should remember to include PHG in the differential diagnosis of multiple pulmonary nodules. PMID:9743062

  3. Novel GATA6 mutations in patients with pancreatic agenesis and congenital heart malformations.

    Science.gov (United States)

    Chao, Christina S; McKnight, Kristen D; Cox, Kenneth L; Chang, Anne L; Kim, Seung K; Feldman, Brian J

    2015-01-01

    Patients with pancreatic agenesis are born without a pancreas, causing permanent neonatal diabetes and pancreatic enzyme insufficiency. These patients require insulin and enzyme replacement therapy to survive, grow, and maintain normal blood glucose levels. Pancreatic agenesis is an uncommon condition but high-throughput sequencing methods provide a rare opportunity to identify critical genes that are necessary for human pancreas development. Here we present the clinical history, evaluation, and the genetic and molecular analysis from two patients with pancreatic agenesis. Both patients were born with intrauterine growth restriction, minor heart defects and neonatal diabetes. In both cases, pancreatic agenesis was confirmed by imaging studies. The patients are clinically stable with pancreatic enzymes and insulin therapy. In order identify the etiology for their disease, we performed whole exome sequencing on both patients. For each proband we identified a de novo heterozygous mutation in the GATA6 gene. GATA6 is a homeobox containing transcription factor involved in both early development of the pancreas and heart. In vitro functional analysis of one of the variants revealed that the mutation creates a premature stop codon in the coding sequence resulting in the production of a truncated protein with loss of activity. These results show how genetic mutations in GATA6 may lead to functional inactivity and pancreatic agenesis in humans. PMID:25706805

  4. Novel GATA6 mutations in patients with pancreatic agenesis and congenital heart malformations.

    Directory of Open Access Journals (Sweden)

    Christina S Chao

    Full Text Available Patients with pancreatic agenesis are born without a pancreas, causing permanent neonatal diabetes and pancreatic enzyme insufficiency. These patients require insulin and enzyme replacement therapy to survive, grow, and maintain normal blood glucose levels. Pancreatic agenesis is an uncommon condition but high-throughput sequencing methods provide a rare opportunity to identify critical genes that are necessary for human pancreas development. Here we present the clinical history, evaluation, and the genetic and molecular analysis from two patients with pancreatic agenesis. Both patients were born with intrauterine growth restriction, minor heart defects and neonatal diabetes. In both cases, pancreatic agenesis was confirmed by imaging studies. The patients are clinically stable with pancreatic enzymes and insulin therapy. In order identify the etiology for their disease, we performed whole exome sequencing on both patients. For each proband we identified a de novo heterozygous mutation in the GATA6 gene. GATA6 is a homeobox containing transcription factor involved in both early development of the pancreas and heart. In vitro functional analysis of one of the variants revealed that the mutation creates a premature stop codon in the coding sequence resulting in the production of a truncated protein with loss of activity. These results show how genetic mutations in GATA6 may lead to functional inactivity and pancreatic agenesis in humans.

  5. Tracheal Agenesis: A Challenging Prenatal Diagnosis—Contribution of Fetal MRI

    Directory of Open Access Journals (Sweden)

    Charline Bertholdt

    2015-01-01

    Full Text Available Tracheal agenesis is a rare congenital anomaly. The prevalence is less than 1 : 50 000 with a male to female ratio of 2 : 1. This anomaly may be isolated but, in 93% of cases, it is part of polymalformative syndrome. The most evocative diagnosis situation is the ultrasonographic congenital high airway obstruction syndrome. Dilated airways, enlarged lungs with flattened diaphragm, fetal ascites and severe nonimmune hydrops can be observed. In the absence of a congenital high airway obstruction syndrome, the antenatal diagnosis of tracheal agenesis is difficult. Tracheal agenesis should be suspected in the presence of an unexplained polyhydramnios associated with congenital malformations. The fetal airway exploration should then be systematically performed by fetal thoracic magnetic resonance imaging. A case of Floyd’s type II tracheal agenesis, detected during the postnatal period, is reported here. The retrospective reexamination of fetal magnetic resonance images showed that the antenatal diagnosis would have been easy if a systematical examination of upper airways had been performed. Prenatal diagnosis of tracheal agenesis is possible with fetal MRI but the really challenge is to think about this pathology.

  6. Mutations in PTF1A cause pancreatic and cerebellar agenesis.

    Science.gov (United States)

    Sellick, Gabrielle S; Barker, Karen T; Stolte-Dijkstra, Irene; Fleischmann, Christina; Coleman, Richard J; Garrett, Christine; Gloyn, Anna L; Edghill, Emma L; Hattersley, Andrew T; Wellauer, Peter K; Goodwin, Graham; Houlston, Richard S

    2004-12-01

    Individuals with permanent neonatal diabetes mellitus usually present within the first three months of life and require insulin treatment. We recently identified a locus on chromosome 10p13-p12.1 involved in permanent neonatal diabetes mellitus associated with pancreatic and cerebellar agenesis in a genome-wide linkage search of a consanguineous Pakistani family. Here we report the further linkage analysis of this family and a second family of Northern European descent segregating an identical phenotype. Positional cloning identified the mutations 705insG and C886T in the gene PTF1A, encoding pancreas transcription factor 1alpha, as disease-causing sequence changes. Both mutations cause truncation of the expressed PTF1A protein C-terminal to the basic-helix-loop-helix domain. Reporter-gene studies using a minimal PTF1A deletion mutant indicate that the deleted region defines a new domain that is crucial for the function of this protein. PTF1A is known to have a role in mammalian pancreatic development, and the clinical phenotype of the affected individuals implicated the protein as a key regulator of cerebellar neurogenesis. The essential role of PTF1A in normal cerebellar development was confirmed by detailed neuropathological analysis of Ptf1a(-/-) mice. PMID:15543146

  7. Bilateral persistent primitive trigeminal arteries associated with trigeminal neuralgia.

    Science.gov (United States)

    Son, B; Yang, S; Sung, J; Lee, S

    2013-03-01

    Persistent carotid-vertebrobasilar anastomoses (PCVBA) include the primitive trigeminal artery (PTA), the primitive otic artery (POA), the primitive hypoglossal artery and proatlantal arteries (ProAs). The PTA is the most commonly seen of these accounting for approximately 80-85% of PCVBAs. The PTA which connects the internal carotid artery (ICA) to the basilar artery (BA) may occasionally connect to the superior or posterior inferior cerebellar arteries without interposition to the BA. It is then referred to as a persistent trigeminal artery variant (PTAV), an anomalous carotid-cerebellar anastomosis. Bilateral occurrence of PTA is extremely rare. During vertebral artery (VA) development the anterior radicular artery of segment C1 from the proatlantal artery of Padget evolves into the intradural component of the VA (V4 segment) plus a short extradural segment (distal V3 segment). Agenesis of a single anterior radicular artery of ProA results in the absence of one distal VA associated with an unremarkable contralateral VA and the BA. Absence or hypoplasia of the terminal portion of one VA is a commonly observed anatomic variant. However, absence of the terminal portions of both VAs is exceptional. A rare case of bilateral PTAs is presented with unilateral PTA and a contralateral PTAV causing trigeminal neuralgia. Furthermore, the bilateral PTAs were associated with the absence of the proximal portion of the BA in addition to the bilateral lack of a distal VA. This finding comes as a logical consequence of the developmental anatomy of the vertebrobasilar junction and is consistent with the assumed congenital nature of the anatomic variant. PMID:22113402

  8. Escleritis posterior bilateral Bilateral posterior scleritis

    Directory of Open Access Journals (Sweden)

    A. Zurutuza

    2011-08-01

    Full Text Available La escleritis posterior es un proceso inflamatorio de la parte posterior de la esclera. Su prevalencia es muy baja y el diagnóstico puede resultar complicado por la ausencia de signos oculares externos. Es más frecuente en mujeres. Cuando aparece en pacientes jóvenes no suele tener otras patologías asociadas, pero en mayores de 55 años hasta un tercio de los casos tienen relación con alguna enfermedad sistémica, sobre todo la artritis reumatoide. El diagnóstico de esta patología puede requerir un abordaje multidisciplinar y la colaboración de oftalmólogos con neurólogos, internistas o reumatólogos. En este artículo se describe un caso de escleritis posterior bilateral idiopática.Posterior scleritis is an inflammatory process of the posterior part of the sclera. Its prevalence is very low and its diagnosis can be complicated due to the absence of external ocular signs. It is more frequent in women. In young patients it does not usually have other associated pathologies, but in those over 55 years nearly one-third of the cases have a relation with some systemic disease, above all rheumatoid arthritis. The diagnosis of this pathology can require a multidisciplinary approach and the collaboration of ophthalmologists with neurologists, internists or rheumatologists. This article describes a case of idiopathic bilateral posterior scleritis.

  9. Atypical Radiological Manifestation of Pulmonary Metastatic Calcification

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Eun Hae; Kim, Eun Sun; Kim, Chul Hwan; Ham, Soo Youn; Oh, Yu Whan [Korea University College of Medicine, Seoul (Korea, Republic of)

    2008-04-15

    Metastatic pulmonary calcification is a condition of calcium deposition in the normal pulmonary parenchyma, and this is secondary to abnormal calcium metabolism without any prior soft tissue damage. The predisposing factors for this condition include chronic renal failure, hypercalcemia and increased tissue alkalinity. The most common radiologic manifestation consists of poorly defined nodular opacities in the upper lung zone. These opacities reflect the deposition of calcium salts in the pulmonary interstitium. We present here a case of metastatic pulmonary calcification in a patient who recovered from pneumonia with sepsis and whose high-resolution CT (HRCT) images demonstrated localized parenchymal airspace calcification that was limited to the bilateral lower lobes. These lower lobes had been involved with pneumonic consolidation without calcification, as seen on the previous CT scan. In summary, we report here on an atypical presentation of metastatic pulmonary calcification that showed dense airspace consolidation localized to the bilateral lower lobes in a patient with primary hyperparathyroidism and pneumonia.

  10. Agenesis of the corpus callosum in a newborn with Turner mosaicism

    Directory of Open Access Journals (Sweden)

    Ester Pereira

    2014-05-01

    Full Text Available The agenesis of the corpus callosum results from a failure in the development of the largest fiber bundle that connects cerebral hemispheres. Patient’s outcome is influenced by etiology and associated central nervous system malformations. We describe a child with Turner syndrome (TS mosaicism, with particular phenotype features and a complete agenesis of the corpus callosum. To our knowledge, this is the second case report of TS mosaicism associated with complete agenesis of the corpus callosum. Anatomical brain magnetic resonance imaging and diffusion tensor imaging were useful to confirm the complete absence of the corpus callosum, evaluate associated central nervous system malformations, visualize abnormal white matter tracts (Probst bundles and assess the remaining commissures.

  11. Recessive mutations in a distal PTF1A enhancer cause isolated pancreatic agenesis.

    Science.gov (United States)

    Weedon, Michael N; Cebola, Inês; Patch, Ann-Marie; Flanagan, Sarah E; De Franco, Elisa; Caswell, Richard; Rodríguez-Seguí, Santiago A; Shaw-Smith, Charles; Cho, Candy H-H; Lango Allen, Hana; Houghton, Jayne A L; Roth, Christian L; Chen, Rongrong; Hussain, Khalid; Marsh, Phil; Vallier, Ludovic; Murray, Anna; Ellard, Sian; Ferrer, Jorge; Hattersley, Andrew T

    2014-01-01

    The contribution of cis-regulatory mutations to human disease remains poorly understood. Whole-genome sequencing can identify all noncoding variants, yet the discrimination of causal regulatory mutations represents a formidable challenge. We used epigenomic annotation in human embryonic stem cell (hESC)-derived pancreatic progenitor cells to guide the interpretation of whole-genome sequences from individuals with isolated pancreatic agenesis. This analysis uncovered six different recessive mutations in a previously uncharacterized ~400-bp sequence located 25 kb downstream of PTF1A (encoding pancreas-specific transcription factor 1a) in ten families with pancreatic agenesis. We show that this region acts as a developmental enhancer of PTF1A and that the mutations abolish enhancer activity. These mutations are the most common cause of isolated pancreatic agenesis. Integrating genome sequencing and epigenomic annotation in a disease-relevant cell type can thus uncover new noncoding elements underlying human development and disease. PMID:24212882

  12. Primary Pulmonary Epithelioid Hemangioendothelioma: A Rare Cause of PET-Negative Pulmonary Nodules

    Science.gov (United States)

    Cazzuffi, Riccardo; Calia, Nunzio; Ravenna, Franco; Pasquini, Claudio; Saturni, Sara; Cavallesco, Giorgio Narciso; Quarantotto, Francesco; Rinaldi, Rosa; Cogo, Annaluisa; Caramori, Gaetano; Papi, Alberto

    2011-01-01

    We report here a case of primary pulmonary epithelioid hemangioendothelioma diagnosed in a 67-year-old Caucasian man, presenting with exertion dyspnoea, dry cough, and multiple bilateral pulmonary nodules revealed by computed tomography. At the 18F-fluorodeoxyglucose positron emission tomography, these nodules were negative. The histopathological diagnosis was made on a pulmonary wedge resection (performed during video-thoracoscopic surgery). PMID:21869893

  13. Primary Pulmonary Epithelioid Hemangioendothelioma: A Rare Cause of PET-Negative Pulmonary Nodules

    Directory of Open Access Journals (Sweden)

    Riccardo Cazzuffi

    2011-01-01

    Full Text Available We report here a case of primary pulmonary epithelioid hemangioendothelioma diagnosed in a 67-year-old Caucasian man, presenting with exertion dyspnoea, dry cough, and multiple bilateral pulmonary nodules revealed by computed tomography. At the 18F-fluorodeoxyglucose positron emission tomography, these nodules were negative. The histopathological diagnosis was made on a pulmonary wedge resection (performed during video-thoracoscopic surgery.

  14. Bilateral inferior turbinate osteoma

    Science.gov (United States)

    Sahemey, R.; Warfield, A.T.; Ahmed, S.

    2016-01-01

    Osteomas are the most common benign osteoclastic tumours of the paranasal sinuses. However, nasal cavity and turbinate osteomas are extremely rare. Only nine middle turbinate, three inferior turbinate and one inferior turbinate osteoma cases have been reported to date. The present case report describes the management and follow-up of symptomatic bilateral inferior turbinate osteoma. A 60-year-old female presented with symptoms of bilateral nasal obstruction and right-sided epiphora. Radiological investigation found hypertrophic bony changes involving both inferior turbinates. The patient was managed successfully by endoscopic inferior turbinectomies in order to achieve a patent airway, with no further recurrence of tumour after 3 months postoperatively. To the best of our knowledge, this is the first reported case of bilateral inferior turbinate osteoma. We describe a safe and minimally invasive method of tumour resection, which has a better cosmetic outcome compared with other approaches. PMID:27534890

  15. Complicated Varicella Infection at 8-year-old Boy with Pulmonary Agenesis

    Science.gov (United States)

    Hadzovic–Cengic, Meliha; Baljic, Rusmir; Hadzic, Amir; Lukovac, Enra; Mehanic, Snjezana; Ahmetspahic-Begic, Aida

    2012-01-01

    SUMMARY CONFLICT OF INTEREST: none declared. Introduction Varicella or chickenpox is highly contagious, childhood infectious disease caused by primary infection with varicella – zoster virus from the herpes family of viruses. Usually it has a mild clinical course, rarely with described complication, mostly affecting respiratory tract and rarely the central nervous system. Case report The case present 8 year old boy hospitalized eighth day of disease with clinical pictures of varicella complication. Upon receipt tachydyspnea, high fever, tachycardia, hypotensive with positive findings on lung auscultation in the sense of pneumonia. Extremely high values of non-specific inflammatory parameters are implied on bacterial infection which is treated using triple antimicrobial therapy and antiviral. A detailed clinical, laboratory and radiological evaluation is determined of clinical disease complication under a picture of MODS that required prolonged multidisciplinary treatment in ICU. Conclusion The disease had a favorable clinical outcome in terms of training completely without consequences but, with the detected congenital absence lower lobe of right lung and transposition of the brachiocephalic trunk. PMID:24493991

  16. Bilateral Primary Intraocular Lymphoma

    Directory of Open Access Journals (Sweden)

    Mehrdad Karimi

    2011-01-01

    Full Text Available Purpose: To report a case of bilateral primary intraocular lymphoma. Case report: A 33-year-old man presented with bilateral blurred vision since two years ago. Examination revealed large keratic precipitates, anterior chamber reaction, posterior subcapsular cataracts, and vitreous infiltration. After a short trial of topical and periocular steroids, diagnostic 25-gauge pars plana vitrectomy was performed and cytologic evaluation of the aspirate confirmed a diagnosis of intraocular lymphoma. The patient was subsequently managed with intravitreal methotrexate in both eyes and responded favorably. Central nervous system workup for lymphoma was negative. Conclusion: Primary intraocular lymphoma should be considered in young adults suffering from chronic recalcitrant panuveitis.

  17. BILATERAL IMMATURE OVARIAN TERATOMA

    OpenAIRE

    Vinay; Aditya Pratap; Chetan; Ramesh; Rajlaxmi Jaysing

    2014-01-01

    : Immature teratoma (IMT) is tumor composed of tissues from ectoderm, mesoderm and endoderm and is considered the second most common germ cell tumor. IMT account for 10-20% of all ovarian neoplasias in women less than 20 years of age, with peak incidence between 15 and 19 years old. IMT rarely occurs during menopause. We herein reporting a rare case in a 3 years old girl with bilateral immature ovarian teratoma which is very rare in bilateralism of tumor as well as the fac...

  18. BILATERAL IMMATURE OVARIAN TERATOMA

    Directory of Open Access Journals (Sweden)

    Vinay

    2014-10-01

    Full Text Available : Immature teratoma (IMT is tumor composed of tissues from ectoderm, mesoderm and endoderm and is considered the second most common germ cell tumor. IMT account for 10-20% of all ovarian neoplasias in women less than 20 years of age, with peak incidence between 15 and 19 years old. IMT rarely occurs during menopause. We herein reporting a rare case in a 3 years old girl with bilateral immature ovarian teratoma which is very rare in bilateralism of tumor as well as the fact that the patient age is below the average for the occurrence of these tumors.

  19. Uterus didelphys with unilateral obstructed hemivagina and haematocolpos with ipsilateral renal agenesis - a case report.

    Science.gov (United States)

    Chowdhury, S; Ara, R; Begum, S A; Chowdhury, S B; Hussain, M A; Mirza, T T

    2015-01-01

    Mullerian anomalies are a relatively uncommon occurrence with implication for adolescents and adults as they may result in specific gynaecologic, fertility and obstetrical issues. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis is a rare congenital anomaly. Patient may be asymptomatic and unaware of having double uterus or may present with severe dysmenorrhoea or dyspareunia or a palpable mass due to unilateral haematocolpos. We report a case of 12 year old girl with this condition who was diagnosed as uterus didelphys with unilateral haematocolpos with ipsilateral renal agenesis on the basis of clinical association, physical examination and sonography and intravenous urogram. PMID:25725693

  20. Uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with ipsilateral renal agenesis

    Directory of Open Access Journals (Sweden)

    Gaurav Jindal

    2009-01-01

    Full Text Available Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn Werner-Wunderlich Syndrome is a rare congenital anomaly. It mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos. A patient with dysmenorrhea from a double uterus and an obstructed hemivagina is a diagnostic dilemma because the menses are regular. We report a case of a 14-year-old girl with this condition who was diagnosed as uterus didelphys with unilateral hematocolpos and hydrosalpinx with ipsilateral renal agenesis on the basis of sonography and confirmed by laparoscopic examination.

  1. Agenesis of multiple primary and permanent teeth unilaterally and its possible management.

    Science.gov (United States)

    Ephraim, Rena; Rajamani, T; Feroz, Tp Mohammed; Abraham, Sajith

    2015-05-01

    Oligodontia is the agenesis of numerous teeth (more than six teeth). Agenesis of teeth in primary and permanent dentition is a rare incidence and very few are reported in the dental literature. Although the etiology of congenital agenesis of teeth is unclear, several factors such as a tendency toward genetic predilection, metabolic disorders, trauma, infection, radiation or idiopathic reasons are found to be responsible. Available literature reports agenesis most often of third molars, maxillary lateral incisors, mandibular central and lateral incisors, and mandibular second premolars in decreasing order of frequency of occurrence. Males are more often affected than females. Maxillary primary teeth are more often found affected by agenesis than mandibular primary teeth. Available literature reports missing teeth to be found unilaterally or orbilaterally with a predisposition toward a similar phenomenon occurring in the permanent dentition. In congenital agenesis impaired growth of the alveolar process, reduced lower facial height, speech impairment, deep bite, restriction in the movement of the tongue due to ankyloglossia, asymmetry of the affected side of the face are clinical features evident. There has been no report of complete agenesis of primary and permanent teeth in a whole mandibular quadrant in conjunction with the absence of maxillary second and third molars on the affected side. Here, we report an incidence of a rare occurrence of complete agenesis of more than 10 primary and permanent teeth, unilaterally, in the right mandibular quadrant, in a child of 10 years presenting with a chief complaint of several missing primary teeth and difficulty in speech due to its complete absence. Treatment strategies included various orthodontic and restorative procedures to improve esthetics and function. The orthodontic procedures involved expansion of the narrow maxillary arch to obtain a functionally adapted occlusion and creation of space for future alignment, and

  2. MR diagnosis of penile agenesis: is it just absence of a phallus?

    Energy Technology Data Exchange (ETDEWEB)

    Goenka, Ajit; Jain, Vaibhav; Sharma, Raju; Gupta, Arun K. [All India Institute of Medical Sciences, Department of Radiodiagnosis, New Delhi (India); Bajpai, Minu [All India Institute of Medical Sciences, Department of Paediatric Surgery, New Delhi (India)

    2008-10-15

    Penile agenesis is an extremely rare anomaly that results from absence of the genital tubercle or its failure to develop into a penis during embryonic life. Associated anomalies of the genitourinary and distal gastrointestinal tracts are frequently present. Imaging modalities, particularly MRI and cystography, play a crucial role in establishing the diagnosis and guiding further management. We report a child with penile agenesis with a urethroanal fistula and a blind-ending anterior urethra who had been reared as a male until presentation. (orig.)

  3. Left lung agenesis discovered by a spontaneous pneumothorax in a 20-year-old girl

    Directory of Open Access Journals (Sweden)

    Abdessalem Hentati

    2016-01-01

    Full Text Available Lung agenesis is a rare condition which prognosis widely depends on associated malformations. Clinical presentation is so variable and diagnosis is often made in childhood. Here, we present a case of a 20-year-old girl who was admitted because of a spontaneous pneumothorax. Explorations concluded at a left lung agenesis, a hyperinflated right lung crossing the midline with a corresponding pneumothorax. There was no malformation else. This congenital condition and treatment for this rare presentation are discussed in detail.

  4. Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Atsushi Nakao

    2013-10-01

    Full Text Available Pancreatic agenesis is a rare disease that causes neonatal diabetes mellitus and exocrine pancreatic insufficiency. We report the case of a very low birth weight infant with congenital diaphragmatic hernia, and congenital heart disease (ventricular septal defect and patent ductus arteriosus. Failure to gain weight, despite well-managed respiratory and heart failure, was improved by infusion of subcutaneous insulin, administration of pancreatic enzyme, and nutrition of medium-chain-triglyceride -enriched formula. This is the first case of pancreatic agenesis with both malformations where the patient is discharged from the hospital. Early diagnosis and adequate treatments to compensate pancreatic function may prevent mortality and improve growth.

  5. Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia

    OpenAIRE

    Saeed, Muhammad; Anwar ul HAQ; Khaqan QADIR

    2014-01-01

    How to Cite This Article: Saeed M, Haq A, Qadir Kh.Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia. Iran J Child Neurol. 2014 Autumn;8(4): 76-79.AbstractObjectiveBart’s syndrome is defined as congenital localized absence of skin, and associated with epidermolysis bullosa. A newborn with Bart’s syndrome is reported because it is a very rare condition, especially when associated with corpus callosum agenesis and concomitant choanal atresia. Clinically it is characterized...

  6. Effect of Vestibulo-Proprioceptive Stimulations in a Child with Agenesis of the Corpus Callosum

    OpenAIRE

    Hamid Dalvand; Leila Dehghan; Dr Hossein Bagheri

    2010-01-01

    Background and Aim: The purpose of the present study was to investigate the effect of vestibulo-proprioceptive stimulations of sensory integration theory on the development of gross and fine motor, language and personal-social functions in a child with agenesis of the corpus callosum.Case: We report a 10.5 month old boy with agenesis of the corpus callosum. The intervention was administered based on sensory integration theory an hour a week for 20 weeks. The exercise intervention consisted of...

  7. Pulmonary alveolar proteinosis

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    Hadice Selimoğlu Şen

    2011-06-01

    Full Text Available Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinosis material within the alveoli. A 36 age male patient applied to our hospital with dyspnea that began 5 months ago. Bilateral diffuse infiltration on postero-anterior chest graphy was seen. Arterial blood gas measurements (ABG was: pH:7.44, pCO2: 36 mmHg, pO2: 49 mmHg, SaO2: 85%, HCO3: 24 mmol/L. High resolution computed tomography (HRCT reported as bilateral diffuse ground glass areas and interlobuler septal thickening on this areas. Asido resistant bacillus was negative in sputum at three times and was also negative in post bronchoscopic sputum and bronchoalveoler lavage material. Wedge resection was made by video-assisted thoracoscopic surgery. Histopathology report of biopsy material revealed “Pulmonary alveolar proteinosis (PAP”. Massive lung lavage under general anesthesia was planned for patient. Clinical improvement was seen in patient beginning from admission and ABG measurements entered the recovery trend in follow-up. Partial pressure of oxygen was increased to 65 mmHg and patient was followed without lung lavage. Control chest radiography and HRCT showed significant radiological improvement. After three months, radiological lesions had completely regressed at control HRCT. As a result, in patients with symptoms and radiologically bilateral diffuse infiltration physicians should consider PAP as a rare disease in the differential diagnosis.

  8. Bilateral endogenous fungal endophthalmitis

    OpenAIRE

    Michal, Wilczynski; Olena, Wilczynska; Wojciech, Omulecki

    2013-01-01

    Endogenous endophthalmitis is a rare and severe intraocular infection which can be vision-threatening. We describe a case of bilateral fungal endogenous endophthalmitis in a 64-year-old male which was successfully treated with systemic administration of fluconazole followed by pars plana vitrectomy with an intravitreous injection of amphotericin B.

  9. Bilateral akillesseneruptur hos nyretransplanterede

    DEFF Research Database (Denmark)

    Skovgaard, D; Feldt-Rasmussen, B F; Nimb, L;

    1996-01-01

    Increased incidence of tendinitis and tendon ruptures is reported in recipients of a kidney transplant. Two cases of bilateral achilles tendon rupture after minimal trauma are described. Tendon ruptures are more frequent in individuals with kidney disease in dialysis or after transplantation comp...

  10. Bilateral chronic subdural hematoma

    DEFF Research Database (Denmark)

    Andersen-Ranberg, Nina Christine; Poulsen, Frantz Rom; Bergholt, Bo;

    2016-01-01

    ) surgery. The overall retreatment rate was 21.6% (57 of 264 patients). Cases treated with unilateral surgery had twice the risk of retreatment compared with cases undergoing bilateral surgery (28.7% vs 14.1%, respectively, p = 0.002). In accordance with previous studies, the data also showed...

  11. Isolated Vaginal Agenesis Associated with Multiple Gastrointestinal Anomalies: A Case Report

    Directory of Open Access Journals (Sweden)

    Rossella Angotti

    2016-07-01

    Full Text Available More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF, anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature.

  12. Isolated Vaginal Agenesis Associated with Multiple Gastrointestinal Anomalies: A Case Report.

    Science.gov (United States)

    Angotti, R; Molinaro, F; Bulotta, A L; Ferrara, F; Sica, M; Bindi, E; Messina, M

    2016-01-01

    More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature. PMID:27433450

  13. Professional and patient-based evaluation of oral rehabilitation in patients with tooth agenesis

    DEFF Research Database (Denmark)

    Dueled, Erik; Gotfredsen, Klaus; Trab Damsgaard, Mogens;

    2009-01-01

    OBJECTIVES: The outcome of oral rehabilitation is usually monitored with clinical tests rather than by patient's perception of change. The aim of this study was to describe the objective measure and subjective perception of oral rehabilitation in patients with tooth agenesis. MATERIAL AND METHODS...

  14. Agenesis of the corpus callosum associated with paroxysmal hypothermia: Shapiro's syndrome.

    Science.gov (United States)

    Segeren, C M; Polderman, K H; Lips, P

    1997-01-01

    Spontaneous recurrent hypothermia and hyperhidrosis associated with agenesis of the corpus callosum was first described by Shapiro and Plum in 1967. Since then, several cases with similar symptoms (now known as Shapiro's syndrome or spontaneous periodic hypothermia) have been described. We report another case of this syndrome in a 21-year-old-man, and discuss possible pathogenetic mechanisms and therapeutic approaches. PMID:9038041

  15. Lateral incisor agenesis, canine impaction and characteristics of supernumerary teeth in a South European male population

    NARCIS (Netherlands)

    Delli, Konstantina; Livas, Christos; Bornstein, Michael M

    2013-01-01

    OBJECTIVE: To assess the prevalence of lateral incisor agenesis impacted canines and supernumerary teeth in a young adult male population. MATERIALS AND METHODS: The panoramic radiographs of 1745 military students (mean age: 18.6 ± 0.52 years) who attended the Center of Aviation Medicine of the Arme

  16. Unilateral lacrimal fistula in a patient with uterus didelphys and renal agenesis.

    Science.gov (United States)

    Turan-Vural, Ece; Atmaca, Fatih; Unlu, Cihan; Erdogan, Gurkan; Bayramlar, Huseyin; Bayramalar, Huseyin

    2012-02-01

    A 30-year-old female patient presented to our clinic because of lacrimation from an orifice close to the left lower eyelid. Ocular examinations and analyses revealed uterus didelphys and unilateral renal agenesis associated with a left lacrimal fistula. The patient underwent fistulectomy and external dacryocystorhinostomy. We decided to report on this patient owing to the unusual concurrent systemic abnormalities. PMID:22258183

  17. Non-syndromic tooth agenesis associated with a nonsense mutation in ectodysplasin-A (EDA).

    Science.gov (United States)

    Nikopensius, T; Annilo, T; Jagomägi, T; Gilissen, C; Kals, M; Krjutškov, K; Mägi, R; Eelmets, M; Gerst-Talas, U; Remm, M; Saag, M; Hoischen, A; Metspalu, A

    2013-06-01

    Mutations in the ectodysplasin-A (EDA) gene have been generally associated with X-linked hypohidrotic ectodermal dysplasia (XLHED). Recently, missense mutations in EDA have been reported to cause familial non-syndromic tooth agenesis. In this study, we report a novel EDA mutation in an Estonian family segregating non-syndromic tooth agenesis with variable expressivity. Affected individuals had no associated defects in other ectodermal organs. Using whole-exome sequencing, we identified a heterozygous nonsense mutation c.874G>T (p.Glu292X) in the TNF homology domain of EDA in all affected female patients. This protein-altering variant arose de novo, and the potentially causative allele was transmitted to affected offspring from the affected mother. We suggest that the dental phenotype variability described in heterozygous female carriers of EDA mutation may occur because of the differential pattern of X-chromosome inactivation, which retains reduced levels of EDA-receptor signaling in tissues involved in tooth morphogenesis. This results in selective tooth agenesis rather than XLHED phenotype. The present study broadens the mutation spectrum for this locus and demonstrates that EDA mutations may result in non-syndromic tooth agenesis in heterozygous females. PMID:23603338

  18. Bilateral matrix-exponential distributions

    DEFF Research Database (Denmark)

    Bladt, Mogens; Esparza, Luz Judith R; Nielsen, Bo Friis

    2012-01-01

    In this article we define the classes of bilateral and multivariate bilateral matrix-exponential distributions. These distributions have support on the entire real space and have rational moment-generating functions. These distributions extend the class of bilateral phasetype distributions of [1]...

  19. Pulmonary hyalinizing granuloma with hydronephrosis.

    Science.gov (United States)

    Hashimoto, Seiji; Fujii, Wataru; Takahashi, Tatsurou; Shiroshita, Koichi; Sakurai, Tetsuo; Ueda, Takahiro; Kawata, Tetsuya

    2002-06-01

    A 49-year-old man was admitted for the evaluation of a bilateral mass shadow in his chest X-ray film. No definitive diagnosis was established either by brushing cytology or biopsy through bronchoscopy. No malignancies were suggested by general work-up. Both masses were surgically removed, and were diagnosed as pulmonary hyalinizing granuloma (PHG). Fifteen months later, low grade fever continued and the renal function decreased. Laboratory examinations revealed bilateral hydronephrosis with polyclonal hypergammaglobulinemia. The findings of abdominal CT and urography were compatible with retroperitoneal fibrosis. Steroid treatment completely reversed the initial abnormality in laboratory data and the symptoms disappeared. PMID:12135180

  20. [Spontaneous bilateral Petit hernia].

    Science.gov (United States)

    Fontoura, Rodrigo Dias; Araújo, Emerson Silveira de; Oliveira, Gustavo Alves de; Sarmenghi Filho, Deolindo; Kalil, Mitre

    2011-01-01

    Petit's lumbar hernia is an uncommon defect of the posterior abdominal wall that represents less than 1% of all abdominal wall hernias. It is more often unilateral and founded in young females, rarely containing a real herniated sac. There are two different approaches to repair: laparoscopy and open surgery. The goal of this article is to report one case of spontaneous bilateral lumbar Petit's hernia treated with open surgery.

  1. Bilateral intraocular dirofilariasis

    Directory of Open Access Journals (Sweden)

    Viney Gupta

    2014-01-01

    Full Text Available Ocular dirofilariasis mostly presents as a subconjunctival or eyelid lesion. [1] Intraocular dirofilarial infestation is rare. [2],[3] We report a case of a young woman who was accidentally detected to have a live motile worm in the anterior segment in one eye and a cystic lesion on the optic disc in the other eye. To our knowledge, bilateral intraocular dirofilariasis has never been reported.

  2. Compressive Bilateral Filtering.

    Science.gov (United States)

    Sugimoto, Kenjiro; Kamata, Sei-Ichiro

    2015-11-01

    This paper presents an efficient constant-time bilateral filter that produces a near-optimal performance tradeoff between approximate accuracy and computational complexity without any complicated parameter adjustment, called a compressive bilateral filter (CBLF). The constant-time means that the computational complexity is independent of its filter window size. Although many existing constant-time bilateral filters have been proposed step-by-step to pursue a more efficient performance tradeoff, they have less focused on the optimal tradeoff for their own frameworks. It is important to discuss this question, because it can reveal whether or not a constant-time algorithm still has plenty room for improvements of performance tradeoff. This paper tackles the question from a viewpoint of compressibility and highlights the fact that state-of-the-art algorithms have not yet touched the optimal tradeoff. The CBLF achieves a near-optimal performance tradeoff by two key ideas: 1) an approximate Gaussian range kernel through Fourier analysis and 2) a period length optimization. Experiments demonstrate that the CBLF significantly outperforms state-of-the-art algorithms in terms of approximate accuracy, computational complexity, and usability. PMID:26068315

  3. Primary pulmonary cryptococcosis.

    Science.gov (United States)

    Asadi Gharabaghi, Mehrnaz; Allameh, Seyyed Farshad

    2014-01-01

    Here, we report a case of primary cryptococcal pneumonia in a 25-year-old woman who presented with several weeks' history of cough, dyspnoea and night sweating. These symptoms started in the third trimester of her pregnancy. She was being treated for infertility and got pregnant with in vitro fertilisation. On chest imaging, there were bilateral air space consolidation and cavitary lesions. Fungal pulmonary infection was diagnosed after surgical lung biopsy. She received fluconazole 400 mg per day orally for 6 months and recovered completely. PMID:24692384

  4. Pulmonary alveolar proteinosis

    International Nuclear Information System (INIS)

    Since the original description of pulmonary alveolar proteinosis (PAP) as bilaterally symmetric, confluent, perihilar infiltrates, no large series evaluating the relative frequency of radiographic findings has been published. The authors reviewed 53 histologically proved cases and found a predominantly confluent pattern in 77.4%; of these, 61% had a nodular component. A predominantly nodular pattern was seen in 22.6% often with some coalescence or reticular shadows. Distribution was perhilar 56.6%, diffuse in 26.4%, basilar in 26.4%, and asymmetric in 35.8%. The classic pattern was seen in 18.9%. The authors conclude that the spectrum of patterns in pulmonary alveolar proteinosis is wider than generally appreciated

  5. Simultaneous vs staged bilateral video-assisted thoracoscopic surgery.

    Science.gov (United States)

    Yim, A P

    1996-10-01

    It is generally thought that simultaneous bilateral chest surgery carries a high morbidity. We reviewed the results of simultaneous (under one anesthesia) vs staged bilateral video-assisted thoracoscopic surgery (VATS) from a single institution over a 35-month period. From September 1992 to July 1995, we performed simultaneous bilateral VATS on 37 patients (31 males, six females, age ranging from 15 to 55 years) with spontaneous pneumothorax (20) for bleb resections and pleurodesis; thoracodorsal sympathectomy (12) for palmar hyperhidrosis and vasospastic disease; and metastatic sarcomas (five) for wedge lung resections. During the same period, nine patients with metachronous bilateral spontaneous pneumothorax had staged procedures, as did two with digital ischemic ulcers for sympathectomy and three with metastatic pulmonary osteosarcomas for resection. Mean postoperative hospital stays in days for the simultaneous groups were 3.3 for spontaneous pneumothorax, 2.1 for sympathectomy, and 1.5 for wedge resection, compared to 2.9, 2.5, and 2.2 for the staged groups, respectively (p > 0.05 by Mann-Whitney U tests). Likewise, pain assessment by visual analogue scale as well as analgesic requirement showed no significant difference between the simultaneous and the staged groups. We conclude that simultaneous VATS is not associated with increased morbidity or prolonged hospital stay compared to the staged counterparts and provides an attractive alternative to the median sternotomy, bilateral posterolateral thoracotomy, or transternal (clam-shell) thoracotomy for selected cases of simultaneous bilateral lung surgery. PMID:8864102

  6. Pulmonary Hyalinizing Granuloma Mimicking Metastatic Lung Cancer

    Directory of Open Access Journals (Sweden)

    Nuri Düzgün

    2015-01-01

    Full Text Available Pulmonary hyalinizing granuloma is a very rare benign condition, which usually manifests as solitary and sometimes as multiple pulmonary nodules. Deposition of immune complexes in the lung parenchyma due to hypersensitivity reactions is implicated in the etiology of pulmonary hyalinizing granuloma. A 59-year-old female patient who presented to our clinic with complaints of chest pain and cough had bilateral, multiple, and rounded lesions with regular margins suggesting metastatic lung disease. A transthoracic needle biopsy of the nodule was performed in the left pulmonary anterior segment. Biopsy showed no malignancy. Since no diagnosis was made by the biopsy, the patient underwent a video-assisted thoracic surgery. The wedge biopsy reported pulmonary hyalinizing granuloma. We aimed to present the diagnosis and treatment stages of our patient who was diagnosed with pulmonary hyalinizing granuloma in the light of literature review.

  7. Pulmonary Hyalinizing Granuloma Mimicking Metastatic Lung Cancer.

    Science.gov (United States)

    Düzgün, Nuri; Kurtipek, Ercan; Esme, Hıdır; Eren Karanis, Meryem İlkay; Tolu, İsmet

    2015-01-01

    Pulmonary hyalinizing granuloma is a very rare benign condition, which usually manifests as solitary and sometimes as multiple pulmonary nodules. Deposition of immune complexes in the lung parenchyma due to hypersensitivity reactions is implicated in the etiology of pulmonary hyalinizing granuloma. A 59-year-old female patient who presented to our clinic with complaints of chest pain and cough had bilateral, multiple, and rounded lesions with regular margins suggesting metastatic lung disease. A transthoracic needle biopsy of the nodule was performed in the left pulmonary anterior segment. Biopsy showed no malignancy. Since no diagnosis was made by the biopsy, the patient underwent a video-assisted thoracic surgery. The wedge biopsy reported pulmonary hyalinizing granuloma. We aimed to present the diagnosis and treatment stages of our patient who was diagnosed with pulmonary hyalinizing granuloma in the light of literature review. PMID:26347384

  8. Pulmonary Hyalinizing Granuloma Mimicking Metastatic Lung Cancer

    Science.gov (United States)

    Düzgün, Nuri; Kurtipek, Ercan; Esme, Hıdır; Eren Karanis, Meryem İlkay; Tolu, İsmet

    2015-01-01

    Pulmonary hyalinizing granuloma is a very rare benign condition, which usually manifests as solitary and sometimes as multiple pulmonary nodules. Deposition of immune complexes in the lung parenchyma due to hypersensitivity reactions is implicated in the etiology of pulmonary hyalinizing granuloma. A 59-year-old female patient who presented to our clinic with complaints of chest pain and cough had bilateral, multiple, and rounded lesions with regular margins suggesting metastatic lung disease. A transthoracic needle biopsy of the nodule was performed in the left pulmonary anterior segment. Biopsy showed no malignancy. Since no diagnosis was made by the biopsy, the patient underwent a video-assisted thoracic surgery. The wedge biopsy reported pulmonary hyalinizing granuloma. We aimed to present the diagnosis and treatment stages of our patient who was diagnosed with pulmonary hyalinizing granuloma in the light of literature review. PMID:26347384

  9. Neuralgic Amyotrophy: A Rare Cause of Bilateral Diaphragmatic Paralysis

    OpenAIRE

    Neil Shinder; Alasdair Polson; Elizabeth Pringle; Denis E O'Donnell

    1998-01-01

    Neuralgic amyotrophy, also known as brachial neuritis, is a well described clinical entity. Diaphragmatic dysfunction, as a result of phrenic nerve root involvement (cervical roots 3 to 5), is an uncommon, but increasingly recognized association. The case of a previously healthy 61-year-old woman who, after a prodrome of neck and shoulder discomfort, presented with severe orthopnea is described. Pulmonary function and electrophysiological studies led to a diagnosis of bilateral diaphragmatic ...

  10. Multiple myeloma with pulmonary embolism: a case report

    Institute of Scientific and Technical Information of China (English)

    YING Ke-jing; ZHOU Yong; JIANG Hao; CHEN En-guo; ZHOU Pan

    2006-01-01

    @@ We report a rare case of a patient who died suddenly, in whom bilateral pulmonary artery thrombosis with multiple myeloma was found at autopsy. An estimate of the incidence of pulmonary embolism in myeloma patients based on postmortem examinations is about 3.2%. Hypercoagulability and decreased fibrinolytic capacity due to multiple myeloma were the probable causes of multiple thromboses.

  11. Pulmonary Fibrosis

    Science.gov (United States)

    Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. This ... blood may not get enough oxygen. Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue ...

  12. Sonographic findings of the uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: Report of 4 cases

    International Nuclear Information System (INIS)

    The association of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is rare anomaly. It is caused by failure in the fusion of the caudal portion of the M llerian duct, secondary to Wolffian duct anomaly. We report 4 cases of uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis. All 4 cases are diagnosed by ultrasonogram, IVU, CT and MRI findings and confirmed by surgery or diagnostic laparoscopy and vaginal examination.

  13. Sonographic findings of the uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: Report of 4 cases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jung Hae; Lee, Hyeon Keong; Choi, Dae Seob; Lee, Sung Woo; Bae, Chul Sung; Yoon, Hye Won; Kim, Mi Woon; Kim, Sung Ja [Dongguk University College of Medicine, Kyungju (Korea, Republic of)

    2001-09-15

    The association of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is rare anomaly. It is caused by failure in the fusion of the caudal portion of the M llerian duct, secondary to Wolffian duct anomaly. We report 4 cases of uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis. All 4 cases are diagnosed by ultrasonogram, IVU, CT and MRI findings and confirmed by surgery or diagnostic laparoscopy and vaginal examination.

  14. Involvement of and interaction between WNT10A and EDA mutations in tooth agenesis cases in the Chinese population.

    Directory of Open Access Journals (Sweden)

    Huiying He

    Full Text Available BACKGROUND: Dental agenesis is the most common, often heritable, developmental anomaly in humans. Although WNT10A gene mutations are known to cause rare syndromes associated with tooth agenesis, including onycho-odontodermal dysplasia (OODD, Schöpf-Schulz-Passarge syndrome (SSPS, hypohidrotic ectodermal dysplasia (HED, and more than half of the cases of isolated oligodontia recently, the genotype-phenotype correlations and the mode of inheritance of WNT10A mutations remain unclear. The phenotypic expression with WNT10A mutations shows a high degree of variability, suggesting that other genes might function with WNT10A in regulating ectodermal organ development. Moreover, the involvement of mutations in other genes, such as EDA, which is also associated with HED and isolated tooth agenesis, is not clear. Therefore, we hypothesized that EDA mutations interact with WNT10A mutations to play a role in tooth agenesis. Additionally, EDA, EDAR, and EDARADD encode signaling molecules in the Eda/Edar/NF-κB signaling pathways, we also checked EDAR and EDARADD in this study. METHODS: WNT10A, EDA, EDAR and EDARADD were sequenced in 88 patients with isolated oligodontia and 26 patients with syndromic tooth agenesis. The structure of two mutated WNT10A and two mutated EDA proteins was analyzed. RESULTS: Digenic mutations of both WNT10A and EDA were identified in 2 of 88 (2.27% isolated oligodontia cases and 4 of 26 (15.38% syndromic tooth agenesis cases. No mutation in EDAR or EDARADD gene was found. CONCLUSIONS: WNT10A and EDA digenic mutations could result in oligodontia and syndromic tooth agenesis in the Chinese population. Moreover, our results will greatly expand the genotypic spectrum of tooth agenesis.

  15. Learn About Pulmonary Fibrosis

    Science.gov (United States)

    ... www.lung.org > Lung Health and Diseases > Lung Disease Lookup > Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary fibrosis is ... while processing XML file."); } }); } } --> Blank Section Header Lung Disease Lookup Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, ...

  16. Agenesis of the corpus callosumina premature infant associated with Langdon-Down syndrome

    Directory of Open Access Journals (Sweden)

    Velisavljev-Filipović Gordana

    2005-01-01

    Full Text Available Introduction Agenesis of the corpus callosum is an abnormality of the part of the brain connecting the two cerebral hemispheres. It can be partial, complete or atypical. The fibers from the cerebral cortex project towards the homotypical region of the contra-lateral cortex passing through the corpus callosum, and crossing the middle line. The absence of corpus callosum causes failure of information transfer from one hemisphere to the other. Children with this anomaly present with learning disabilities and trouble with memorizing facts. Agenesis of corpus callosum may be of syndromic or non-syndromic type. The more common form is the one not associated with any syndrome. The agenesis of corpus callosum is more frequent in male children. Case report This paper presents a child from a twin pregnancy with partial absence of corpus callosum. The pregnancy was not controlled. It ended in premature birth. The afflicted twin is a boy, second in birth order. Apart from agenesis of corpus callosum, he also suffers from Down syndrome. The first twin is healthy, with corpus callosum and with normal karyotype. There was no consanguinity. In the 6th month of pregnancy the mother suffered from infection of the upper respiratory tract that might be the etiological factor of this anomaly. The child was born with hypotrophy, and all anthropometric parameters were below the third percentile. In the neonatal period, the agenesis of corpus callosum was diagnosed by ultrasonic examination and confirmed by CT and MR examinations. The child is now three and a half month old. Active monitoring of the psychical and motor development will show whether there will be any retardation in the psycho-motor development and later deficiency of the higher cortical functions and intelligence. Discussion and Conclusion The clinical characteristics of this anomaly are numerous. They range from asymptomatic cases, with normal intellectual capacity, to severe mental retardation

  17. Bilateral accessory thoracodorsal artery.

    Science.gov (United States)

    Natsis, Konstantinos; Totlis, Trifon; Tsikaras, Prokopios; Skandalakis, Panagiotis

    2006-09-01

    The subscapular artery arises from the third part of the axillary artery and gives off the circumflex scapular and the thoracodorsal arteries. Although anatomical variations of the axillary artery are very common, the existence of a unilateral accessory thoracodorsal artery has been described in the literature only once. There are no reports of bilateral accessory thoracodorsal artery, in the literature. In the present study, a bilateral accessory thoracodorsal artery, originating on either side of the third part of the axillary artery, is described in a 68-year-old female cadaver. All the other branches of the axillary artery had a typical origin, course, distribution and termination. This extremely rare anatomical variation apart from the anatomical importance also has clinical significance for surgeons in this area. Especially, during the dissection or mobilization of the latissimus dorsi that is partly used for coverage problems in many regions of the body and also in dynamic cardiomyoplasty, any iatrogenic injury of this accessory artery may result in ischemia and functional loss of the graft.

  18. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

    OpenAIRE

    Christopher Coleman; Aziza Nassar; Barbara McComb

    2014-01-01

    Pulmonary hyalinizing granuloma (PHG) is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET) was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two n...

  19. CASE OF BILATERAL ANTERIOR SEGMENT TUBERCULOSIS

    Directory of Open Access Journals (Sweden)

    Gopal Kishan

    2015-04-01

    Full Text Available Tuberculosis is an important cause of uveitis in our country. India has been declared the country with the highest tuberculosis burden with 25 % of the cases . (1 This is especially true in Andhra Pradesh and Telangana which have a high percentage of affected patients. However , tuberculous uveitis is usually a unilateral presen tation. We report a case of bilateral uveitis of tuberculous origin in a 55 year old woman. She presented with different tuberculous manifestations in the two eyes. While the RE showed non granulomatous uveitis with sclerosing keratitis , the LE showed gran ulomatous uveitis with mutton fat keratic precipitates. Systemic examination revealed an enlarged lymph node which was positive for tuberculosis on biopsy. She was hence diagnosed as extra pulmonary tuberculosis and was treated with ATT regimen. In this pa tient , uveitis was the initial manifestation and resulted in a systemic diagnosis.

  20. A rare case of Turner′s syndrome presenting with Mullerian agenesis

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    Suresh Vaddadi

    2013-01-01

    Full Text Available Turner′s syndrome also called as Ullrich Turner′s syndrome, is a disease of unclear pathogenesis characterized by complete or partial absence of one sex chromosome, with or without cell line mosaicism in a phenotypic female with short stature. Various anomalies result in a constellation of features, of which the most disturbing is primary amenorrhea due to gonadal dysgenesis. Hormone therapy in these patients can often result in successful menstruation, and scope for subsequent pregnancy because of anatomically normal uterus and vagina. Coexisting Mullerian agenesis in these patients can jeopardize the chances of future pregnancy as they have associated structural abnormalities of the uterus and vagina. We report a rare case of middle-aged female with Turner′s syndrome and Mullerian agenesis having absent secondary sexual characters and missing uterus with incompletely formed vagina.

  1. A case of corpus callosum agenesis presenting with recurrent brief depression

    Directory of Open Access Journals (Sweden)

    Bhattacharyya Ranjan

    2009-01-01

    Full Text Available Agenesis of corpus callosum can have various neuropsychiatric manifestations. Following case report highlights the case of a young man presenting with features of recurrent brief depressive disorder, each lasting for about 3 to 7 days, for over a year. He had history of occasional headache and episodes of swooning attack in between, usually precipitated by emotional events. His neuroimaging revealed agenesis of corpus callosum. He was experiencing swooning attacks as he became aware that some ′unusual′ findings were present in his reports. Recurrent brief depression can be a manifestation of this congenital anomaly, and conversion disorder can be present as comorbid diagnosis perhaps due to ignorance and fear of this apparently innocuous congenital malformation.

  2. [Pancreatic cancer in a patient with congenital agenesis of the dorsal pancreas].

    Science.gov (United States)

    Oki, Yusuke; Onoyama, Hirohiko; Nikaido, Mitsuhiro; Iinuma, Shoji; Endo, Koji; Tomita, Yumi; Mizuno, Katsuhiko; Yasui, Hiroshi

    2013-06-01

    A 65-year-old man with back pain showed a hypovascular lesion of the head of the pancreas on dynamic computed tomography and abdominal ultrasonography. The distal portion of the pancreas was not visible. Endoscopic retrograde cholangiopancreatography revealed pancreatic duct obstruction, and the duodenal minor papilla was not visible. Therefore, we diagnosed the patient's condition as stage IVa pancreatic cancer with congenital agenesis of the dorsal pancreas. The patient underwent successful chemotherapy with 3 courses of gemcitabine and S-1, which was followed by pancreaticoduodenectomy. Pathological staging revealed invasive ductal carcinoma, pT3, pN0, pM0, stage III. We report a rare case of pancreatic cancer with congenital agenesis of the dorsal pancreas.

  3. Segmental aplasia of the uterine horn with ipsilateral renal agenesis in a cat.

    Science.gov (United States)

    Chang, Jinhwa; Jung, Joo-hyun; Yoon, Junghee; Choi, Min-cheol; Park, Jae Hak; Seo, Kang-Moon; Jeong, Seong Mok

    2008-06-01

    A nine-month-old domestic short haired cat was admitted with the history of acute vomiting, depression and shivering. Abdominal ultrasonography revealed minimum enlargement of the right uterine horn filled with anechoic fluid. On excretory urography, functionally and anatomically normal, enlarged left kidney was found, but right kidney was absent. It was preliminary diagnosed as hydrometra with right renal agenesis. Aiming at the correction of hydrometra, we performed ovariohysterectomy. During spaying, we found a missing segment of distal part of the right uterine horn and absence of ipsilateral kidney and ureter. Compressed uterine structure and segmental aplasia of right uterine horn were found in histopathological investigation. Taken together, it was diagnosed as a segmental aplasia of uterine horn with ipsilateral renal agenesis. PMID:18628611

  4. The combination of thermal dysregulation and agenesis of corpus callosum: Shapiro′s or/and reverse Shapiro′s syndrome

    Directory of Open Access Journals (Sweden)

    Yasemin Topcu

    2013-01-01

    Full Text Available Shapiro syndrome is an extremely rare condition consisting the clinical triad of recurrent hypothermia, hyperhydrosis and agenesis of the corpus callosum. On the other hand, reverse Shapiro′s sydrome is characterized periodic hyperthermia and agenesis of the corpus callosum. Here, we describe a 3.5-year-old girl with complete agenesis of corpus callosum presenting with recurrent fever and vomiting. She also had hypothermia attacks with accompanying diaphoresis. To the best of our knowledge, there is no described case with episodes of hyperthermia, hypothermia, and vomiting associated with agenesis of the corpus callosum. Recurrent vomiting may be a newly defined symptom associated with these syndromes.

  5. Uterus didelphys with partial vaginal septum and distal vaginal agenesis: an unusual anomaly

    International Nuclear Information System (INIS)

    Complex malformations of female genital tract are not so common. Their correct identification is of paramount importance for appropriate management. A thorough knowledge of embryology, pre-operative imaging with MRI and examination under anaesthesia is essential to identify accurately the constellation of anomalies and to plan appropriate management. This case reports the coexistence of Mullerian abnormality with vaginal agenesis in an 18 years old girl which was managed by dissecting the lower half of vagina and pull-through vaginoplasty. (author)

  6. Uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with ipsilateral renal agenesis

    OpenAIRE

    Gaurav Jindal; Satish Kachhawa; G L Meena; Gopal Dhakar

    2009-01-01

    Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn Werner-Wunderlich Syndrome) is a rare congenital anomaly. It mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos. A patient with dysmenorrhea from a double uterus and an obstructed hemivagina is a diagnostic dilemma because the menses are regular. We report a case of a 14-year-old girl with this condition who was diagnosed as uterus didelphys with unilateral hematocolpos and h...

  7. Uterus Didelphys with Obstructed Right Hemivagina, Ipsilateral Renal Agenesis and Right Pyocolpos: A Case Report

    OpenAIRE

    Dhar, Hansa; Yasser A Razek; Hamdi, Ilham

    2011-01-01

    Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA Syndrome) is a rare congenital anomaly of the female genital tract. Uterus didelphys occurs when the midline fusion of the mullerian ducts is arrested, either completely or incompletely. Women with didelphic uterus may be asymptomatic and unaware of having a double uterus. They may present with complaints of dysmenorrhoea and dyspareunia. A 25 year old woman attending the infertility clinic at Nizwa regional re...

  8. Uterus didelphys with an obstructed unilateral vagina and ipsilateral renal agenesis: A rare cause of dysmenorrhoea

    OpenAIRE

    ATTAR, Rukset; Yıldırım, Gazi; Inan, Yücel; Küzılkale, Özge; Karateke, Ateş

    2013-01-01

    Didelphic uterus with obstructed hemivagina and ipsilateral renal agenesis is a rare condition. It usually presents with pelvic pain following the menarche, dysmenorrhoea, and an increase in abdominal volume or a palpable mass due to unilateral haematocolpos. We present the case of a 13-year-old girl who referred with recurrent pelvic pain, mainly at the time of menses, and irregular menstrual cycle complaints in this report. The patient underwent ultrasonography and magnetic resonance (MR) i...

  9. OHVIRA: Uterus didelphys, blind hemivagina and ipsilateral renal agenesis: Advantage MRI

    OpenAIRE

    Bajaj, Sunil K.; Ritu Misra; Thukral, Brij B; Rohini Gupta

    2012-01-01

    We present here a case of an uncommon complex uterine anomaly - Obstructed HemiVagina with Ipsilateral Renal Agenesis (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome in a 14-year-old girl along with sonographic (trans-abdominal and trans labial), and MRI findings. The patient underwent surgery wherein imaging findings were confirmed. An MRI has proved to be of great help in correct diagnosis avoiding surgical interventions/ laparoscopy, which were needed in past to diagnose this rar...

  10. Agenesis of the internal carotid artery with a large hemangioma of the tongue

    International Nuclear Information System (INIS)

    Total developmental absence of the internal carotid artery is relatively rare, with only 54 cases previously reported. Most, being without neurological symptoms, were found by chance. For this patient with cavernous hemangiomas in the facial, oral and cervical regions, conventional treatment would be embolization of the feeding vessels, combined with ligation of the external carotid artery. Angiography, however, revealed agenesis of the left internal carotid artery, abnormal origin of the aortic arch and azygos anterior cerebral artery. (orig./MG)

  11. Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report

    OpenAIRE

    Atsushi Nakao; Tomohiro Takeda; Yoshiya Hisaeda; Atsushi Hirota; Syusuke Amagata; Yuko Sakurai; Tadashi Kawakami

    2013-01-01

    Pancreatic agenesis is a rare disease that causes neonatal diabetes mellitus and exocrine pancreatic insufficiency. We report the case of a very low birth weight infant with congenital diaphragmatic hernia, and congenital heart disease (ventricular septal defect and patent ductus arteriosus). Failure to gain weight, despite well-managed respiratory and heart failure, was improved by infusion of subcutaneous insulin, administration of pancreatic enzyme, and nutrition of medium-chain-triglyceri...

  12. Unilateral renal agenesis: case review of ambulatory pediatric nephrology clinics in Cali

    Directory of Open Access Journals (Sweden)

    Consuelo Restrepo de Rovetto

    2010-03-01

    Full Text Available Introduction: Unilateral renal agenesis is a frequent renal malformation with incidence of 1 per 1000 live born children. There are no statistics nor protocols to manage and follow-up these patients. Usually asymptomatic, it can be early detected by prenatal ultrasound, allowing opportune detection and adequate follow up. The goal is to describe main features found in children with renal agenesis at four ambulatory pediatric nephrology clinics in Cali, Colombia: Hospital Universitario del Valle (HUV, Club Noel Children’s hospital and the private practice of two pediatric nephrologists.Methods: This is a retrospective descriptive study in a series of cases with congenital renal agenesis diagnosed between January, 1995 and December 2007, of patients under 18 years of age and based on a review of clinical records.Results: 43 patients were found, 51.2% males, 88% from the department of Valle del Cauca. Prenatal diagnoses were conducted in only 21% of these patients; agenesis was right in 48.8% and left in 51.2%. In 46.5%, association with other pathologies was found: occult spinal bifida, congenital scoliosis, and Klippel-Feil sequence. Compensatory renal hypertrophy was reported in 39.5% of the patients, 42% had episodes of urinary tract infection, 31% vesicoureteral reflux and 10% proteinuria. Four male patients (9.3% developed renal failure. The average follow-up was 6.6 years.Conclusions: Prenatal diagnose was not very frequent in our set of cases. Urinary tract infection and vesicoureteral reflux were common, increasing the risk of renal deterioration. Prolonged follow up is suggested, with unified protocols to prevent renal failure.

  13. Unilateral renal agenesis: case review of ambulatory pediatric nephrology clinics in Cali

    Directory of Open Access Journals (Sweden)

    Consuelo Restrepo de Rovetto

    2010-09-01

    Full Text Available Introduction: Unilateral renal agenesis is a frequent renal malformation with incidence of 1 per 1000 live born children. There are no statistics nor protocols to manage and follow-up these patients. Usually asymptomatic, it can be early detected by prenatal ultrasound, allowing opportune detection and adequate follow up. The goal is to describe main features found in children with renal agenesis at four ambulatory pediatric nephrology clinics in Cali, Colombia: Hospital Universitario del Valle (HUV, Club Noel Children’s hospital and the private practice of two pediatric nephrologists. Methods: This is a retrospective descriptive study in a series of cases with congenital renal agenesis diagnosed between January, 1995 and December 2007, of patients under 18 years of age and based on a review of clinical records. Results: 43 patients were found, 51.2% males, 88% from the department of Valle del Cauca. Prenatal diagnoses were conducted in only 21% of these patients; agenesis was right in 48.8% and left in 51.2%. In 46.5%, association with other pathologies was found: occult spinal bifida, congenital scoliosis, and Klippel-Feil sequence. Compensatory renal hypertrophy was reported in 39.5% of the patients, 42% had episodes of urinary tract infection, 31% vesicoureteral reflux and 10% proteinuria. Four male patients (9.3% developed renal failure. The average follow-up was 6.6 years. Conclusions: Prenatal diagnose was not very frequent in our set of cases. Urinary tract infection and vesicoureteral reflux were common, increasing the risk of renal deterioration. Prolonged follow up is suggested, with unified protocols to prevent renal failure.

  14. Learning and memory in individuals with agenesis of the corpus callosum

    OpenAIRE

    Paul, Lynn K.; Erickson, Roger L.; Hartman, Jo Ann; Brown, Warren S.

    2016-01-01

    Damage to long white matter pathways in the cerebral cortex is known to affect memory capacity. However, the specific contribution of interhemispheric connectivity in memory functioning is only beginning to become understood. The present study examined verbal and visual memory processing in individuals with agenesis of the corpus callosum (AgCC) using the Wechsler Memory Scale-Third Edition (WMS-III; Wechsler, 1997b). Thirty participants with AgCC (FSIQ >78) were compared against 30 healthy a...

  15. Agenesis of the corpus callosumina premature infant associated with Langdon-Down syndrome

    OpenAIRE

    Velisavljev-Filipović Gordana

    2005-01-01

    Introduction Agenesis of the corpus callosum is an abnormality of the part of the brain connecting the two cerebral hemispheres. It can be partial, complete or atypical. The fibers from the cerebral cortex project towards the homotypical region of the contra-lateral cortex passing through the corpus callosum, and crossing the middle line. The absence of corpus callosum causes failure of information transfer from one hemisphere to the other. Children with this anomaly present with learning dis...

  16. Agenesis of Ductus Venosus Presenting with Dilated Inferior Vena Cava with Favorable Outcome

    OpenAIRE

    Omer Dai; Harun Egemen Tolunay; Mehmet Murat Seval; Alper Kahraman; Korhan Kahraman; Acar Koç

    2014-01-01

    The ductus venosus regulates the distribution of oxygen and placental nutrients by restricting the centralization of blood flow in fetal circulation. The ductus venosus is a small vein transmitting oxygen-rich blood from the umbilical vein to the fetal heart.. Increasing numbers of case reports are being published about ductus venosus agenesis with cardiac structural abnormalities, other malformations, chromosomal abnormalities, and stillbirth. Heart failure, hydrops and unexplained polyhydra...

  17. Bilateral Antepartum Mastitis

    Directory of Open Access Journals (Sweden)

    Peyman Alibeigi

    2010-12-01

    Full Text Available Antepartum mastitis is a rare condition, whereas postpartum orlactation mastitis is a common problem. This report introducesa case of complicated bilateral antepartum mastitis, which wastreated successfully by drain insertion and antibiotic therapy.The patient was a 23-year-old woman in the 23rd week of herfirst pregnancy. Her chief complaint was progressive swelling,redness and radicular pain in both breasts, which had beenstarted gradually from the 18th week of pregnancy. The patientwas admitted to hospital, and received oral and intravenous antibioticsempirically, which was not effective. The patient wastreated by drainage and oral antibiotic therapy. Based on theapproaches employed and the outcomes achieved it is suggestedthat early surgical insertion in the presence of fluid collection inantepartum mastitis will shorten hospitalization and course ofintravenous antibiotic therapy.Iran J Med Sci 2010; 35(4: 327-330.

  18. Idiopathic Bilateral Bloody Tearing

    Directory of Open Access Journals (Sweden)

    Emrullah Beyazyıldız

    2015-01-01

    Full Text Available Bloody tear is a rare and distinct clinic phenomenon. We report a case presenting with the complaint of recurrent episodes of bilateral bloody tearing. A 16-year-old girl presented to our clinic with complaint of bloody tearing in both eyes for 3 months. Bloody tearing was not associated with her menses. A blood-stained discharge from the punctum was not observed during the compression of both nasolacrimal ducts. Nasolacrimal passage was not obstructed. Imaging studies such as dacryocystography and gradient-echo magnetic resonance imaging (MRI of nasolacrimal canal were normal. Intranasal endoscopic evaluation was normal. We collected samples from bloody tears two times and pathological examination was performed. Pathological analysis showed lots of squamous cells and no endometrial cells; dysplastic cells were found. Further evaluations for underlying causes were unremarkable. No abnormalities were found in ophthalmologic, radiologic, and pathologic investigations. This condition is likely a rare abnormality and the least recognized aetiology for the idiopathic phenomenon.

  19. Isolated bilateral ectopic microspherophakia

    Directory of Open Access Journals (Sweden)

    André Omgbwa Eballe

    2010-02-01

    Full Text Available André Omgbwa Eballe1, Godefroy Koki2, Emilienne Epée2, Didier Owono2, Lucienne Bella Asumpta2, Côme Ebana Mvogo21Faculty of Medicine and Pharmaceuticals Sciences, University of Douala Cameroon; 2Faculty of Medicine and Biomedical Sciences, University of Yaoundé CameroonSummary: An eight-year-old school girl presented to us with a bilateral ectopic microspherophakia. She was surgically managed in the ophthalmology unit of the Gynaeco-Obstetric and Pediatric Hospital of Yaoundé in Cameroon. The postoperative follow up was uneventful, and the patient returned to school with a best corrected visual acuity of 4/10 in both eyes to the great satisfaction of her parents.Keywords: ectopic, microspherophakia, surgery

  20. Bilateral acute corneal calcification.

    Science.gov (United States)

    Freddo, T F; Leibowitz, H M

    1985-04-01

    A 38-year-old man with brittle, juvenile onset diabetes mellitus and bilateral severe dry eyes with recurrent corneal ulcers developed atypical band-shaped calcifications of both corneas during a 24-hour period. Serum calcium, phosphate, and carbon dioxide levels all were within normal limits. The patient was mildly uremic but was not in renal failure. When EDTA chelation failed to clear the deposits, partial keratectomies were performed in both eyes and the specimens were examined by light and electron microscopy, including energy dispersive x-ray analysis. Microscopic studies revealed an atypical calcific keratopathy which involved neither Bowman's layer nor the most superficial stromal lamellae. The deposits were confined to deeper lamellae in the anterior stroma and by electron microscopy were composed of extracellular crystalline aggregates. Energy dispersive x-ray analysis of these aggregates confirmed the presence of calcium and phosphate. Corneal dessication appeared to be a major contributing factor in the rapid formation of these deposits.

  1. Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis: The Radiologic Findings of Five Cases

    International Nuclear Information System (INIS)

    To evaluate the imaging findings of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. From March 2003 to December 2008, five patients with uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis were evaluated as part of this study. We retrospectively reviewed the CT, ultrasound, and MRI findings as well as the medical records of each patient. The patients initially underwent an imaging study for abdominal pain (n=3), recurrent vaginal bleeding (n=1), and prenatal evaluation (n=1). Of the five patients that underwent US, four had hematocolpos and two of them had hematometra. Moreover, three patients underwent a CT examination. The MR examination of four patients revealed hematocolpos (n=3), hematometra (n=1), and a tubular structure resembling an ectopic ureter (n=2). The gynecologic examination of a patient without hematocolpos revealed a pinpoint hole in the vaginal septum. Two of four patients with hematocolpos underwent a vaginal septectomy, which resulted in an improvement of the symptoms. The most common finding of patients with uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is vaginal fluid collection. Hematometra is not a consistent finding and can be transient according to the menstrual cycle. MR is the most useful imaging modality for the diagnosis of an ectopic ureter

  2. Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis: The Radiologic Findings of Five Cases

    Energy Technology Data Exchange (ETDEWEB)

    Rhee, Sun Jung; Yi, Boem Ha; Park, Seong Jin; Lee, Hae Kyung; Lee, Kwon Hae; Lee, Hae Hyeog; Kim, Tae Hee [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of)

    2010-08-15

    To evaluate the imaging findings of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. From March 2003 to December 2008, five patients with uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis were evaluated as part of this study. We retrospectively reviewed the CT, ultrasound, and MRI findings as well as the medical records of each patient. The patients initially underwent an imaging study for abdominal pain (n=3), recurrent vaginal bleeding (n=1), and prenatal evaluation (n=1). Of the five patients that underwent US, four had hematocolpos and two of them had hematometra. Moreover, three patients underwent a CT examination. The MR examination of four patients revealed hematocolpos (n=3), hematometra (n=1), and a tubular structure resembling an ectopic ureter (n=2). The gynecologic examination of a patient without hematocolpos revealed a pinpoint hole in the vaginal septum. Two of four patients with hematocolpos underwent a vaginal septectomy, which resulted in an improvement of the symptoms. The most common finding of patients with uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is vaginal fluid collection. Hematometra is not a consistent finding and can be transient according to the menstrual cycle. MR is the most useful imaging modality for the diagnosis of an ectopic ureter.

  3. Effect of Vestibulo-Proprioceptive Stimulations in a Child with Agenesis of the Corpus Callosum

    Directory of Open Access Journals (Sweden)

    Hamid Dalvand

    2010-06-01

    Full Text Available Background and Aim: The purpose of the present study was to investigate the effect of vestibulo-proprioceptive stimulations of sensory integration theory on the development of gross and fine motor, language and personal-social functions in a child with agenesis of the corpus callosum.Case: We report a 10.5 month old boy with agenesis of the corpus callosum. The intervention was administered based on sensory integration theory an hour a week for 20 weeks. The exercise intervention consisted of proprioceptive and linear, sustained and low frequency vestibular stimulations on suspension device and physio roll. A Denver Developmental Screening- II and milestones skill testing was completed pre-intervention and monthly. Post-intervention, age of gross motor, fine motor adaptive, language, and personal-social functions significantly improved. Based on milestones skills, maintenance of gross motor functions (e.g. sitting and quadruped position improved. The child could roll from side to side and released objects voluntarily. The reaction time to auditory stimulations became less than 2 seconds.Conclusion: vestibulo-proprioceptive stimulations using the neuroplasticity ability of the central nervous system is effective for development of gross and fine motor, language, and personal-social functions. These exercises can be administered for a child with agenesis of the corpus callosum.

  4. Unusual ventilation perfusion scintigram in a case of immunologic pulmonary edema clinically simulating pulmonary embolism

    International Nuclear Information System (INIS)

    A case of immunologic pulmonary edema secondary to hydrochlorothiazide allergy developed in a 55-year-old woman that clinically simulated pulmonary embolism. The patient had abnormal washin images with normal washout images on an Xe-133 ventilation study. On the perfusion study, large bilateral central and posterior perfusion defects were present that showed an unusual mirror image pattern on the lateral and posterior oblique views. Resolution of radiographic and scintigraphic abnormalities occurred over a 3-day period in conjunction with corticosteroid therapy

  5. Pediatric isolated bilateral iliac aneurysm.

    Science.gov (United States)

    Chithra, R; Sundar, R Ajai; Velladuraichi, B; Sritharan, N; Amalorpavanathan, J; Vidyasagaran, T

    2013-07-01

    Aneurysms are rare in children. Isolated iliac artery aneurysms are very rare, especially bilateral aneurysms. Pediatric aneurysms are usually secondary to connective tissue disorders, arteritis, or mycotic causes. We present a case of a 3-year-old child with bilateral idiopathic common iliac aneurysms that were successfully repaired with autogenous vein grafts.

  6. Silicosis with bilateral spontaneous pneumothorax

    OpenAIRE

    Fotedar Sanjay; Chaudhary Dhruva; Singhla Vikas; Narang Rajat

    2010-01-01

    Presentation with simultaneous bilateral pneumothorax is uncommon and usually in the context of secondary spontaneous pneumothorax. The association of pneumothorax and silicosis is infrequent and most cases are unilateral. Bilateral pneumothorax in silicosis is very rare with just a few reports in medical literature.

  7. Silicosis with bilateral spontaneous pneumothorax

    Directory of Open Access Journals (Sweden)

    Fotedar Sanjay

    2010-01-01

    Full Text Available Presentation with simultaneous bilateral pneumothorax is uncommon and usually in the context of secondary spontaneous pneumothorax.The association of pneumothorax and silicosis is infrequent and most cases are unilateral. Bilateral pneumothorax in silicosis is very rare with just a few reports in medical literature.

  8. Pulmonary alveolar microlithiasis

    International Nuclear Information System (INIS)

    Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by the diffuse and bilateral presence of calcium phosphate microlite in the alveolar spaces. The progression of this potentially lethal disease is show and most of the patients remain asymptomatic during years or decades, resulting in a show deterioration of the pulmonary function. The typical finding of the sand storm in the chest X-ray is characteristic of this entity. Mutations in the SLC34A2 gene that does the coding for the type II co-transporter of sodium phosphate were identified as responsible for this disease. Of the almost 600 cases, only 6 have been reported in Colombia. We are presenting a case of pulmonary alveolar microlite in a 27 year old man, with progressive respiratory distress whose diagnosis was made by the X-ray findings and confirmed by trans bronchial biopsy. In the 2 years follow-up, shows evolution towards deterioration of his respiratory function making him a candidate for lung transplantation.

  9. Living with Pulmonary Fibrosis

    Science.gov (United States)

    ... www.lung.org > Lung Health and Diseases > Lung Disease Lookup > Pulmonary Fibrosis Living With Pulmonary Fibrosis What to Expect ... while processing XML file."); } }); } } --> Blank Section Header Lung Disease Lookup Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, ...

  10. Acute silicosis with bilateral pneumothorax.

    Science.gov (United States)

    Srivastava, G N; Prasad, Rajniti; Meena, Manoj; Hussain, Moosa

    2014-01-01

    We present a case of acute silicosis with bilateral pneumothorax of a 28-year-old man working at a stone crusher factory for 1 year. He presented to the emergency department with cough, respiratory distress and diffuse chest pain. The patient was managed with bilateral intercostal tube drainage under water seal, oxygen inhalation and conservative therapy. On follow-up he showed improvement of resting dyspnoea and was doing well. This case is being reported because of the rare complications of acute silicosis as bilateral pneumothorax. PMID:24862410

  11. CT Pulmonary Angiography and Suspected Acute Pulmonary Embolism

    Energy Technology Data Exchange (ETDEWEB)

    Enden, T.; Kloew, N.E. [Ullevaal Univ. Hospital, Oslo (Norway). Dept. of Cardiovascular Radiology

    2003-05-01

    Purpose: To evaluate the use and quality of CT pulmonary angiography in our department, and to relate the findings to clinical parameters and diagnoses. Material and Methods: A retrospective study of 324 consecutive patients referred to CT pulmonary angiography with clinically suspected pulmonary embolism (PE). From the medical records we registered clinical parameters, blood gases, D-dimer, risk factors and the results of other relevant imaging studies. Results: 55 patients (17%) had PE detected on CT. 39 had bilateral PE, and 8 patients had isolated peripheral PE. 87% of the examinations showing PE had satisfactory filling of contrast material including the segmental pulmonary arteries, and 60% of the subsegmental arteries. D-dimer test was performed in 209 patients, 85% were positive. A negative D-dimer ruled out PE detected at CT. Dyspnea and concurrent symptoms or detection of deep vein thrombosis (DVT), contraceptive pills and former venous thromboembolism (VTE) were associated with PE. The presence of only one clinical parameter indicated a negative PE diagnosis (p < 0.017), whereas two or more suggested a positive PE diagnosis (p < 0.002). CT also detected various ancillary findings such as consolidation, pleural effusion, nodule or tumor in nearly half of the patients; however, there was no association with the PE diagnosis. Conclusion: The quality of CT pulmonary angiography was satisfactory as a first-line imaging of PE. CT also showed additional pathology of importance in the chest. Our study confirmed that a negative D-dimer ruled out clinically suspected VTE.

  12. Pulmonary studies

    International Nuclear Information System (INIS)

    Radionuclide studies of the lung are described, as regards perfusion studies, ventilation studies and physiological considerations. The four principal applications for radionuclide studies of the lungs are outlined and the uses of these discussed in relation to particular entities including pulmonary embolic disease, congestive heart failure, asthma, acute, nonasthmatic, bronchial obstruction, chronic pulmonary disease and cancer. (Auth./C.F.)

  13. "Recurrent Bilateral Spontaneous Pneumothorax in Early Infancy: A Case of Langerhans Cell Histiocytosis"

    OpenAIRE

    S. Alavi; N. Hemmati; M.T. Arzanian; Ashena, Z

    2005-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by infiltration of either single or multiple organs by S100 and CD1a positive cells. Patients with pulmonary LCH are predisposed to pneumothorax due to destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH who presented at 2 months of age with simultaneous bilateral spontaneous pneumothorax.

  14. Prune belly syndrome associated with bilateral multicystic dysplastic kidneys and urethral obstruction: A case report

    Directory of Open Access Journals (Sweden)

    Arzu Akdag

    2015-06-01

    Full Text Available Prune belly syndrome is a rare congenital disorder defined by a characteristic clinical triad: Abdominal muscle deficiency, severe urinary tract abnormalities, and bilateral cryptorchidism. We describe a preterm neonate of Prune Belly syndrome who had abdominal muscle deficiency, multicystic dysplastic kidney, urethral hypoplasia and pulmonary hypoplasia. We presented this rare case with the data gathered from the literatüre.

  15. A successful laparoscopic neovaginoplasty using peritoneum in Müllerian agenesis with inguinal ovaries accompanied by primary ovarian insufficiency

    Science.gov (United States)

    Gweon, Seonghye; Lee, Jisun; Hwang, Suna; Hwang, Kyoung Joo

    2016-01-01

    The combination of Müllerian agenesis with inguinal ovaries accompanied by primary ovarian insufficiency is extremely rare. A 21-year-old Korean woman was referred to our center with primary amenorrhea. The patient was diagnosed with Müllerian agenesis with inguinal ovaries. Her hormonal profile showed hypergonadotrophic hypogonadism suggesting primary ovarian insufficiency. We performed laparoscopic neovaginoplasty using modified Davydov's procedure and reposition inguinal ovaries in the pelvic cavity. Oral estrogen replacement was applied for the treatment of primary ovarian insufficiency. This is a rare case report on Mayer-Rokitansky-Kuster-Hauser syndrome accompanied not only by inguinal ovaries but also with primary ovarian insufficiency. We present our first experience on the laparoscopic neovaginoplasty performed on the patient with müllerian agenesis accompanied by inguinal ovaries and primary ovarian insufficiency. PMID:27462606

  16. [Agenesis of the right carotid in a subject with dextrocardia. Diagnostic imaging in 1 case with unique characteristics].

    Science.gov (United States)

    Casiglia, E; Spandri, P; Mos, L; Sarti, F; Dalla Pietà, G; Bongiovì, S; Sotira, A

    1990-06-01

    A man aged 51 with dextrocardia and right common and internal carotid artery agenesis is described. Cerebral blood flow is allowed internally by the left carotid and left vertebral antegrade flow, while right artery has only the function to drain the blood from the left side of vertebral system. A small external right carotid does exist, but it is separated from the cerebral hemodynamics. Diagnosis of dextrocardia was made on the basis of the standard chest X-ray, that of carotid agenesis on the basis of selective digital arteriography, color-Doppler and magnetic resonance. The diagnosis of congenital agenesis was based on the absence of a foramen caroticus and of a canalis caroticus. Large anasthomosis between the left and right side of cerebral system permit a normal perfusion to left cerebral hemispherium and a quite normal life.

  17. MURCS (Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia): a rare cause of primary amenorrhoea.

    Science.gov (United States)

    Kumar, Sunil; Sharma, Shruti

    2016-04-01

    The agenesis of the Müllerian duct is the second most common cause of primary amenorrhoea after Turner syndrome. The abnormal development of Müllerian duct often associates with the urinary tract and skeletal abnormalities. MURCS (Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia) association is a unique and rare developmental disorder with four common features of uterine hypoplasia or aplasia, renal agenesis or ectopy, vertebral anomalies and short stature. We report a case of young female with primary amenorrhoea. She had well-developed secondary sexual characteristics along with multiple congenital developmental abnormalities such as the absence of uterus, ectopic kidney, cervical vertebral fusion, hemivertebrae, scoliosis, cervical rib, facial asymmetry and growth retardation. Our case highlights the rarity and clinical importance of this syndrome. For the evaluation of primary amenorrhoea in a female with well-developed secondary sexual characteristics, congenital anomalies should be ruled out before hormone and karyotype analyses. PMID:27099773

  18. Trigeminal neuralgia caused by the vertebral artery associated with primitive trigeminal artery and agenesis of the internal carotid artery.

    Science.gov (United States)

    Fukuda, M; Kameyama, S; Takahashi, H; Tanaka, R

    1998-06-01

    A 69-year-old female presented with trigeminal neuralgia caused by tortuous vertebrobasilar artery associated with primitive trigeminal artery (PTA) and agenesis of the ipsilateral internal carotid artery (ICA). Left vertebral angiography showed marked elongation of the left vertebral artery and filling of the left ICA via the PTA. Bone window computed tomography suggested agenesis of the left ICA. Intraoperative findings showed that the vertebrobasilar junction had compressed the root entry zone of the trigeminal nerve. The neuralgia improved immediately after microvascular decompression. There has been no recurrence for 20 months. Trigeminal neuralgia may be caused by a tortuous vertebrobasilar system due to hemodynamic stress associated with PTA and agenesis of the ICA. PMID:9689822

  19. Bilateral, independent juvenile nasopharyngeal angiofibroma

    DEFF Research Database (Denmark)

    Mørkenborg, Marie-Louise; Frendø, M; Stavngaard, T;

    2015-01-01

    BACKGROUND: Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating...... from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning...... embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. CONCLUSION: This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral...

  20. Bilateral Polydactyly in a foal

    OpenAIRE

    Carstanjen, Bianca; Abitbol, Marie; Desbois, Christophe

    2007-01-01

    The following case report describes the diagnosis and surgery of bilateral polydactyly of unknown origin in a colt. A 7-month-old Berber colt was referred for cosmetic and curative excision of supernumerary digits. Radiographic examination revealed bilateral polydactyly and well-developed first carpal bones. Surgery consisted of an osteotomy of both second metacarpal bones combined with an amputation of the supernumerary digits. The follow-up at 18 months after surgery revealed a sound horse ...

  1. 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...

  2. Pulmonary hyalinizing granuloma with ureteric fibrosis: A case report and review of relevant literature.

    Science.gov (United States)

    Agrawal, D; Deshpande, R; Maheshwari, S; Patel, A; Udwadia, Z F

    2006-01-01

    A 52-year-old, asymptomatic patient presented with bilateral lung nodules on chest radiograph. She was diagnosed to have "pulmonary hyalinizing granuloma" on an open lung biopsy. We review the clinical features of this rare disease. PMID:16970297

  3. Agenesis and not ectopia is common in North Indian children with thyroid dysgenesis

    Directory of Open Access Journals (Sweden)

    Devi Dayal

    2014-01-01

    Full Text Available Context: Ectopic Thyroid Gland (ETG is known to be the most common form of thyroid dysgenesis in children with permanent congenital hypothyroidism (CH. Recent reports indicate that agenesis or hypoplasia of thyroid gland may be commoner as compared to ETG in thyroid dysgenesis (TD. There is limited information available on the proportion of different variants of TD in Indian children. Aim: To characterize the different TD variants in a cohort of North Indian children with TD. Settings and Design: Endocrinology Unit of a large Multispecialty Pediatrics Center located in North India. Retrospective review of clinical records of children with CH due to TD diagnosed between April 2004 and March 2014. Results: Diagnoses of TD in 94 children (48 boys and 46 girls were based on combined scanning with high-resolution ultrasonography, and technetium-99m pertechnetate thyroid scintigraphy. Thyroid agenesis, ectopia and hypoplasia were diagnosed in 74 (78.7%, 14 (14.8% and 6 (6.4% patients respectively. The mean initial serum total T4 and thyroid stimulating hormone concentrations at diagnosis were 3.03 ± 2.88 μg/dL (range 0.01-8.9 and 284.52 ± 300.67 mIU/L (range 10.03-1159.0 respectively. Patients with ETG were older at the time of diagnosis as compared to patients with hypoplasia or ectopia. The mean duration of follow-up was 3.7 ± 2.85 years (range 3 months-10 years. Conclusions : Thyroid agenesis was the most common form of TD in our children with permanent CH. Hypoplasia and ectopia were uncommon. Female preponderance, noted in many previous reports, was not seen in our patients with TD.

  4. A rare case of cerebellar agenesis: a probabilistic Constrained Spherical Deconvolution tractographic study.

    Science.gov (United States)

    Mormina, Enricomaria; Briguglio, Marilena; Morabito, Rosa; Arrigo, Alessandro; Marino, Silvia; Di Rosa, Gabriella; Micalizzi, Alessia; Valente, Enza Maria; Salpietro, Vincenzo; Vinci, Sergio Lucio; Longo, Marcello; Granata, Francesca

    2016-03-01

    Aim of this study is to show the potential of probabilistic tractographic techniques, based on the Constrained Spherical Deconvolution (CSD) algorithms, in recognizing white matter fiber bundle anomalies in patients with complex cerebral malformations, such as cerebellar agenesis. The morphological and tractographic study of a 17-year-old male patient affected by cerebellar agenesis was performed by using a 3Tesla MRI scanner. Genetic and neuropsychological tests were carried out. An MRI morphological study showed the absence of both cerebellar hemispheres and the flattening of the anterior side of the pons. Moreover, it showed a severe vermian hypoplasia with a minimal vermian residual. The study recognized two thin cerebellar remnants, medially in contact with the small vermian residual, at the pontine level. The third ventricle, morphologically normal, communicated with a permagna cerebello-medullary cistern. Probabilistic CSD tractography identified some abnormal and aberrant infratentorial tracts, symmetrical on both sides. In particular, the transverse pontine fibers were absent and the following tracts with aberrant trajectories have been identified: "cerebello-thalamic" tracts; "fronto-cerebellar" tracts; and ipsilateral and contralateral "spino-cerebellar" tracts. Abnormal tracts connecting the two thin cerebellar remnants have also been detected. There were no visible alterations in the main supratentorial tracts in either side. Neuropsychiatric evaluation showed moderate cognitive-motor impairment with discrete adaptive compensation. Probabilistic CSD tractography is a promising technique that overcome reconstruction biases of other diffusion tensor-based approaches and allowed us to recognize, in a patient with cerebellar agenesis, abnormal tracts and aberrant trajectories of normally existing tracts. PMID:25832852

  5. Hernia of the bladder through the broad ligament with renal agenesis and ipsilateral ureter blind ending

    Science.gov (United States)

    El Madi, Aziz; Khattala, Khalid; Rami, Mohammed; Bouabdallah, Youssef

    2014-01-01

    Hernia through a defect of the broad ligament is extremely rare in children. These defects can result from a developmental defect or a spontaneous rupture of cystic formations remnants of mesonephric ducts or Müller. Genital anomalies associated with unilateral renal agenesis are more common in females. We report the case of a 13 months girl allowed for assessment of recurrent urinary tract infection; abdominal examination did not objectified palpable mass, the external genitalia were without abnormalities. Abdominal ultrasound revealed a left renal space is empty with a retrovesical cyst. Cystography was requested that objectified a large pelvic cystic mass retrovesical communicating with the bladder, there was also a left vesicoureteral reflux. Uro- MRI showed a cystic formation retrovesical communicating with the bladder, the right pelvic kidney; uterus is normal size for age. DMSA scintigraphy confirmed the absence of the left kidney with the right kidney that ensures 100% of total renal function. To surgical exploration we found a hernia of the bladder through the left broad ligament, the uterus was dislocated on the right side; left ovary was hypoplasic; the ipsilateral ureter was blind with renal agenesis, we performed by reduction of the bladder then closing the hernial orifice, dissection of the ureter with its ligation and section at the vesical stoma. The postoperative course was uneventful. Evolution is favorable. This observation illustrates a hernia of the bladder through the broad ligament associated with ovarian hypoplasia, renal agenesis and ipsilateral ureter blind ending; this association was not described to our knowledge in the literature. PMID:25422693

  6. Hernia of the bladder through the broad ligament with renal agenesis and ipsilateral ureter blind ending.

    Science.gov (United States)

    El Madi, Aziz; Khattala, Khalid; Rami, Mohammed; Bouabdallah, Youssef

    2014-01-01

    Hernia through a defect of the broad ligament is extremely rare in children. These defects can result from a developmental defect or a spontaneous rupture of cystic formations remnants of mesonephric ducts or Müller. Genital anomalies associated with unilateral renal agenesis are more common in females. We report the case of a 13 months girl allowed for assessment of recurrent urinary tract infection; abdominal examination did not objectified palpable mass, the external genitalia were without abnormalities. Abdominal ultrasound revealed a left renal space is empty with a retrovesical cyst. Cystography was requested that objectified a large pelvic cystic mass retrovesical communicating with the bladder, there was also a left vesicoureteral reflux. Uro- MRI showed a cystic formation retrovesical communicating with the bladder, the right pelvic kidney; uterus is normal size for age. DMSA scintigraphy confirmed the absence of the left kidney with the right kidney that ensures 100% of total renal function. To surgical exploration we found a hernia of the bladder through the left broad ligament, the uterus was dislocated on the right side; left ovary was hypoplasic; the ipsilateral ureter was blind with renal agenesis, we performed by reduction of the bladder then closing the hernial orifice, dissection of the ureter with its ligation and section at the vesical stoma. The postoperative course was uneventful. Evolution is favorable. This observation illustrates a hernia of the bladder through the broad ligament associated with ovarian hypoplasia, renal agenesis and ipsilateral ureter blind ending; this association was not described to our knowledge in the literature. PMID:25422693

  7. Laparoscopic and gasless laparoscopic sigmoid colon vaginoplasty in women with vaginal agenesis

    Institute of Scientific and Technical Information of China (English)

    ZHONG Chen-xi; WU Ji-xiang; LIANG Jie-xiong; WU Qing-hua

    2012-01-01

    Background In the past several decades we have seen multiple advances in the reconstruction for girls born with vaginal agenesis.This study aimed to evaluate the technical feasibility,anatomical and functional outcomes of one-stage laparoscopic and gasless laparoscopic vaginoplasty with sigmoid colon for the patients of vaginal agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome).Methods We did a retrospective review of a total of 150 women with Mayer-Rokitansky-Kuster-Hauser syndrome treated at Beijing Anzhen Hospital,Capital Medical University from March 2006 to August 2010.The patients were divided into the CO2 pneumoperitoneum laparoscopic group and the abdominal wall lift of gasless laparoscopic group.Sigmoid colon vaginoplasty approaches were performed in all of the patients.The surgical techniques,perioperative results,complications,anatomical and functional outcomes of vaginoplasty were recorded.Results All procedures were performed successfully.Significant differences in the operative time and intraoperative blood loss existed in the laparoscopic vaginoplasty group compared with the gasless laparoscopic vaginoplasty group.The patients who underwent sigmoid colon vaginoplasty had good cosmetic results without the problem of excessive mucus production.The postoperative complications were minimal.During a mean follow-up of 15.6 months,no stenosis or shrinkage was encountered.The subjective sexual satisfaction rate with the surgical outcomes in all patients was 83.3%.Conclusions Laparoscopic or gasless laparoscopic vaginoplasty with sigmoid colon are effective and feasible approaches for women with congenital vaginal agenesis.The procedures have satisfactory anatomical and functional results.

  8. Tooth agenesis in patients referred to an Irish tertiary care clinic for the developmental dental disorders.

    LENUS (Irish Health Repository)

    Hashem, Atef A

    2010-01-01

    PURPOSE: This study was carried out to determine the prevalence, severity and pattern of hypodontia in Irish patients referred to a tertiary care clinic for developmental dental disorders. MATERIALS AND METHODS: Details of 168 patients with hypodontia referred during the period 2002-2006 were entered in a database designed as a national record. Tooth charting was completed using clinical and radiographic examinations. The age of patients ranged from 7-50 years, with a median age of 20 years (Mean: 21.79; SD: 8.005). RESULTS: Hypodontia referrals constituted 65.5% of the total referrals. Females were more commonly affected than males with a ratio of 1.3:1. The number of referrals reflected the population density in this area; the majority were referrals from the public dental service. Mandibular second premolars were the most commonly missing teeth, followed by maxillary second premolars and maxillary lateral incisors; maxillary central incisors were the least affected. Symmetry of tooth agenesis between the right and left sides was an evident feature. Slightly more teeth were missing on the left side (n = 725) than on the right side (n = 706) and in the maxillary arch (n = 768) as compared to the mandibular arch (n = 663). Some 54% of patients had severe hypodontia with more than six teeth missing; 32% had moderate hypodontia, with four to six teeth missing. The most common pattern of tooth agenesis was four missing teeth. CONCLUSION: Hypodontia was a common presentation in a population referred to this tertiary care clinic. The pattern and distribution of tooth agenesis in Irish patients appears to follow the patterns reported in the literature.

  9. Can Kallmann syndrome be occasionally diagnosed during childhood? Genetic diagnosis in a child with associated renal agenesis and mirror movements

    Institute of Scientific and Technical Information of China (English)

    Neoklis A. Georgopoulos; Vasiliki Koika; Petros Varmavas; Alexandra Efthymiadou; Dimitra J. Marioli; Stefanos Mantagos; Dionysios Chrysis

    2009-01-01

    @@ Dear Sir, I am Neoklis A. Georgopoulos, from Department of Obstetrics and Gynaecology, Division of Reproductive Endocrinology, University of Patras Medical School,Greece. We present an early prepubertal diagnosis of Kallmann syndrome (KS) in a child with anosmia, renal agenesis and mirror movements. KS is a congenital defect characterized by the presence of hypogonadotropic hypogonadism and anosmia or hyposmia [1].Besides hypogonadism and anosmia, the clinical phenotype is characterized by the presence of other associated features, including, among others, mirror movements (synkinesis), cleft palate and renal agenesis/dysgenesis.

  10. Combined Anomaly of the Right Hepatic Lobe Agenesis and Absence of the Inferior Vena Cava: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Suh, Hee Jung; Kim, Wan Tae; Kim, Mi Young; Cho, Yun Ku [Seoul Veterans Hospital, Seoul (Korea, Republic of)

    2008-07-15

    The absence of the inferior vena cava is an uncommon congenital anomaly that has recently been identified as an important risk factor contributing to the development of deep venous thrombosis. Congenital agenesis of the right hepatic lobe is a rare anomaly which is found incidentally in radiologic examinations. We present a case of a congenital absence of the infrarenal inferior vena cava, combined with agenesis of the right hepatic lobe in a 62-year-old man presented with symptoms of deep venous thrombosis

  11. Solitary Median Maxillary Central Incisor Versus Agenesis of the Maxillary Central Incisor.

    Science.gov (United States)

    de Carvalho, Fabrício Kitazono; Arid, Juliana; De Rossi, Andiara; Paula-Silva, Francisco W G; Nelson-Filho, Paulo

    2016-01-01

    A solitary median maxillary central incisor (SMMCI) is rare and affected individuals may carry a potentially serious condition known as SMMCI syndrome. However, many of these cases do not receive proper attention because they are misdiagnosed as agenesis of the maxillary central incisor. The purpose of this manuscript is to report two cases of children with only one maxillary central incisor and draw diagnostic differences between the entities. A correct diagnosis is very important because if an SMMCI is confirmed, the patient should be referred for genetic counseling. PMID:27098718

  12. OHVIRA: Uterus didelphys, blind hemivagina and ipsilateral renal agenesis: Advantage MRI

    Directory of Open Access Journals (Sweden)

    Sunil K Bajaj

    2012-01-01

    Full Text Available We present here a case of an uncommon complex uterine anomaly - Obstructed HemiVagina with Ipsilateral Renal Agenesis (OHVIRA, also known as Herlyn-Werner-Wunderlich syndrome in a 14-year-old girl along with sonographic (trans-abdominal and trans labial, and MRI findings. The patient underwent surgery wherein imaging findings were confirmed. An MRI has proved to be of great help in correct diagnosis avoiding surgical interventions/ laparoscopy, which were needed in past to diagnose this rare anomaly. We also discuss the development of this anomaly with the help of a relatively new theory of uro-genital development by Acien and review the literature.

  13. Coexistence of Left Internal Carotid Agenesis, Klippel-Feil Syndrome and Postaxial Polydactyly

    International Nuclear Information System (INIS)

    Internal carotid artery agenesis is a rare anomaly that can be clinically asymptomatic. Klippel-Feil syndrome is a skeletal malformation characterized by vertebral fusion. Presence of postaxial polydactyly is suggestive of an underlying syndrome. We report a rare case of a 44-year-old patient with non-specific symptoms and an association between these three rare abnormalities. Vascular anomalies were found using intracranial MR angiography and multi-detector CT angiography of the supraaortic arteries. Presence of a single aforementioned anomaly requires cautious imaging assessment in order to detect possible associated anomalies and avoid diagnostic pitfalls. A possible common genetic background could explain the coexistence of these three anomalies

  14. Uterus didelphys with partial vaginal septum and distal vaginal agenesis: an unusual anomaly.

    Science.gov (United States)

    Singhal, Savita Rani; Lakra, Pinkey; Bishnoi, Pushpa; Rohilla, Seema; Dahiya, Pushpa; Nanda, Smiti

    2013-02-01

    Complex malformations of female genital tract are not so common. Their correct identification is of paramount importance for appropriate management. A thorough knowledge of embryology, pre-operative imaging with MRI and examination under anaesthesia is essential to identify accurately the constellation of anomalies and to plan appropriate management. This case reports the coexistence of Müllerian abnormality with vaginal agenesis in an 18 years old girl which was managed by dissecting the lower half of vagina and pull-through vaginoplasty. PMID:23374522

  15. The Role of Pulmonary Veins in Cancer Progression from a Computed Tomography Viewpoint

    Science.gov (United States)

    Chang, Hung; Liao, Tzu-Yao; Wen, Ming-Sheng; Yu, Chih-Teng

    2016-01-01

    Background. We studied the role of pulmonary veins in cancer progression using computed tomography (CT) scans. Methods. We obtained data from 260 patients with pulmonary vein obstruction syndrome (PVOS). We used CT scans to investigate pulmonary lesions in relation to pulmonary veins. We divided the lesions into central and peripheral lesions by their anatomical location: in the lung parenchymal tissue or pulmonary vein; in the superior or inferior pulmonary vein; and by unilateral or bilateral presence in the lungs. Results. Of the 260 PVOS patients, 226 (87%) had central lesions, 231 (89%) had peripheral lesions, and 190 (75%) had mixed central and peripheral lesions. Among the 226 central lesions, 93% had lesions within the superior pulmonary vein, either bilaterally or unilaterally. Among the 231 peripheral lesions, 65% involved bilateral lungs, 70% involved lesions within the inferior pulmonary veins, and 23% had obvious metastatic extensions into the left atrium. All patients exhibited nodules within their pulmonary veins. The predeath status included respiratory failure (40%) and loss of consciousness (60%). Conclusion. CT scans play an important role in following tumor progression within pulmonary veins. Besides respiratory distress, PVOS cancer cells entering centrally can result in cardiac and cerebral events and loss of consciousness or can metastasize peripherally from the pulmonary veins to the lungs.

  16. Flash pulmonary edema in patients with renal artery stenosis--the Pickering Syndrome

    DEFF Research Database (Denmark)

    Pelta, Anna; Andersen, Ulrik B; Just, Sven;

    2010-01-01

    We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases.......We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases....

  17. Functional Benefits of Bilateral Emphysema Reduction Surgery in an Adolescent.

    Science.gov (United States)

    Julian, Valérie; Moreau de Bellaing, Anne; Filaire, Marc; Richard, Ruddy; Labbé, André

    2016-09-01

    Acquired emphysema is a rare pathology in pediatrics. We report the case of a patient born at term with a neonatal respiratory distress, which had required mechanical ventilation. She developed gradually chronic obstructive pulmonary disease with severe emphysematous lesions, respiratory failure and functional impairments. Bilateral emphysema resection, performed at 16 years old, allowed major functional benefits at rest and during exercise. We present the results of respiratory functional evaluations, walk tests and maximal exercise tests (including measure of dynamic hyperinflation) before and after surgery, which highlights that surgery is a successful option in the treatment of compressive emphysema in childhood. PMID:27549540

  18. Neuralgic Amyotrophy: A Rare Cause of Bilateral Diaphragmatic Paralysis

    Directory of Open Access Journals (Sweden)

    Neil Shinder

    1998-01-01

    Full Text Available Neuralgic amyotrophy, also known as brachial neuritis, is a well described clinical entity. Diaphragmatic dysfunction, as a result of phrenic nerve root involvement (cervical roots 3 to 5, is an uncommon, but increasingly recognized association. The case of a previously healthy 61-year-old woman who, after a prodrome of neck and shoulder discomfort, presented with severe orthopnea is described. Pulmonary function and electrophysiological studies led to a diagnosis of bilateral diaphragmatic paralysis. The patient's clinical course and the exclusion of other nerve entrapment syndromes and neurological disorders strongly favoured the diagnosis of neuralgic amyotrophy.

  19. 38 CFR 4.26 - Bilateral factor.

    Science.gov (United States)

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Bilateral factor. 4.26... DISABILITIES General Policy in Rating § 4.26 Bilateral factor. When a partial disability results from disease... disability. The bilateral factor will be applied to such bilateral disabilities before other combinations...

  20. Bilateral hemimelia of radio in canine – case reportHemimelia bilateral de rádio em canino – relato de caso

    Directory of Open Access Journals (Sweden)

    Thalita Priscila da Silva Peres

    2013-10-01

    Full Text Available Hemimelia or agenesis is a congenital morphological change that may affect the appendicular skeleton. This rare anomaly is the total or partial absence of one or more bones matched. It may present unilaterally or bilaterally, with the most frequent unilateral. In dogs, the most common form of the radial hemimelia is that causes deformity of the affected limb and severe functional limitations. Was Veterinary Hospital of the Federal University of Mato Grosso (HOVET/UFMT, a canine, female mongrel, with 30 days of age, presenting deformity in thoracic limbs since birth, observed signs of lameness and difficulty support. After complete bone development the animal returned for re-evaluation and establishment of appropriate treatment. On radiographic examination, there was bilateral agenesis of radios, angulation of the ulnar cranially, fusion of carpal bones proximal and distal to the ranks, bilateral scapulohumeral dislocation and the presence of a bone structure with well-defined boundaries and rectangular articulated with the scapula. Due to good quality of life of the animal and reserved prognosis of surgery, we opted for conservation of members. The owner was recommended to keep the animal in an environment with soft ground and castration. Hemimelia ou agenesia é uma alteração morfológica congênita que pode acometer o esqueleto apendicular. Esta anomalia rara consiste na ausência total ou parcial de um ou mais ossos pareados. Pode se apresentar de forma uni ou bilateral, sendo a unilateral mais frequente. Em cães, a forma mais comum de hemimelia é a radial, que provoca deformidade do membro afetado e severas limitações funcionais. Foi atendido no Hospital Veterinário da Universidade Federal de Mato Grosso (HOVET/UFMT, um canino, fêmea, sem raça definida, com 30 dias de idade, apresentando deformidade nos membros torácicos desde o nascimento, claudicação e dificuldade de apoio. Após o completo desenvolvimento ósseo o animal

  1. The radiological findings of pulmonary paragonimiasis

    Energy Technology Data Exchange (ETDEWEB)

    Noh, Byeung Suk; Song, Ho Yung; Im, Sun Kyun; Choi, Ki Chul [College of Medicine, Chunbuk National University, Jeonju (Korea, Republic of); Rhee, Hak Song [Presbyterian Medical Center, Jeonju (Korea, Republic of); Hong, Young Pyo [The Korean National Tuberculosis Association, Seoul (Korea, Republic of)

    1988-02-15

    Authors analysed chest X-ray findings in 36 cases of proven pulmonary paragonimiasis which sputum smears were positive for ova of Paragonimus Westermani from October 1978 to January 1987 at Chonbuk National University Hospital, PMC and Korean Institute of tuberculosis. The results are as follows: 1. Roentgenographic findings are cyst formation, pulmonary infiltration, fibrosis, pleural thickening, pleural effusion and pneumothorax. 2. The characteristic cystic shadows were observed in 19 cases (58%). They were multiple aggregated cysts surrounded with pericystic haziness and ranged in size from 1cm to 4cm in diameter. 3. Pulmonary infiltrations showed multiple, variable ill defined area of consolidation, predominantly in periphery of mid and lower lung field, which has migratory tendency. 4. Distribution of pulmonary lesions were bilateral (72%), mid lung fields (56%), and there were tendency of peripheral lung predominance. 5. 4 cases (11%) showed normal chest X-ray findings.

  2. The radiological findings of pulmonary paragonimiasis

    International Nuclear Information System (INIS)

    Authors analysed chest X-ray findings in 36 cases of proven pulmonary paragonimiasis which sputum smears were positive for ova of Paragonimus Westermani from October 1978 to January 1987 at Chonbuk National University Hospital, PMC and Korean Institute of tuberculosis. The results are as follows: 1. Roentgenographic findings are cyst formation, pulmonary infiltration, fibrosis, pleural thickening, pleural effusion and pneumothorax. 2. The characteristic cystic shadows were observed in 19 cases (58%). They were multiple aggregated cysts surrounded with pericystic haziness and ranged in size from 1cm to 4cm in diameter. 3. Pulmonary infiltrations showed multiple, variable ill defined area of consolidation, predominantly in periphery of mid and lower lung field, which has migratory tendency. 4. Distribution of pulmonary lesions were bilateral (72%), mid lung fields (56%), and there were tendency of peripheral lung predominance. 5. 4 cases (11%) showed normal chest X-ray findings

  3. Pulmonary Atresia

    Science.gov (United States)

    ... to repair the defect. Return to main topic: Congenital Heart Disease See on other sites: MedlinePlus https://medlineplus.gov/ency/article/001091.htm Pulmonary atresia American Heart Association www. ...

  4. Pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    Anton Vonk Noordegraaf

    2016-03-01

    Full Text Available In 2015, more than 800 papers were published in the field of pulmonary hypertension. A Clinical Year in Review article cannot possibly incorporate all this work and needs to be selective. The recently published European guidelines for the diagnosis and treatment of pulmonary hypertension contain an inclusive summary of all published clinical studies conducted until very recently. Here, we provide an overview of papers published after the finalisation of the guideline. In addition, we summarise recent advances in pulmonary vasculature science. The selection we made from the enormous amount of published work undoubtedly reflects our personal views and may not include all papers with a significant impact in the near or more distant future. The focus of this paper is on the diagnosis of pulmonary arterial hypertension, understanding the success of combination therapy on the right ventricle and scientific breakthroughs.

  5. Pulmonary hypertension

    Science.gov (United States)

    ... that damage the lungs, such as scleroderma and rheumatoid arthritis Birth defects of the heart Blood clots in the lung ( pulmonary embolism ) Heart failure Heart valve disease HIV infection Low oxygen levels in the blood ...

  6. Pulmonary sequestration

    International Nuclear Information System (INIS)

    Pulmonary sequestration is a congenital affection consisting in the presence of a cystic mass of no-functional pulmonary tissue without an obvious communication with tracheobronchial tree and that receives all or most of its bloodstream of the anomalous vessels from systemic circulation. Taking into account that presentation of this affection is rare compared to other pulmonary affections (between the 1% and the 2% of all pulmonary resections) and that also the more usual is its definitive treatment before adulthood. The case of man aged 44 is presented coming to consultation due to frequent episodes of pneumonias from more 10 years ago diagnosed as a bronchiectasis. The more significant facts of embryology origin of this affection including: anatomical and pathological features, imaging diagnosis, surgical treatment details, and postoperative course. (author)

  7. PULMONARY SCEDOSPORIOSIS

    Directory of Open Access Journals (Sweden)

    SEVERO Luiz Carlos

    1998-01-01

    Full Text Available A case of a solitary pulmonary nodule due to Scedosporium apiospermum (Pseudallescheria boydii is related. A review of the pertinent literature was done and, in addition, similar lesions caused by other opportunistic fungi are commented.

  8. Pulmonary Edema

    OpenAIRE

    Tanser, Paul H.

    1981-01-01

    The physician who deals with pulmonary edema from a pathophysiologic basis will seldom make a diagnostic or therapeutic error. Recent additions to preload and afterload therapy have greatly helped in the emergency and ambulatory treatment of pulmonary edema due to left heart failure. Careful follow-up and patient self-monitoring are the most effective means of reducing hospitalization of chronic heart failure patients.

  9. Pulmonary hypertension

    OpenAIRE

    Anton Vonk Noordegraaf; Joanne A. Groeneveldt; Harm Jan Bogaard

    2016-01-01

    In 2015, more than 800 papers were published in the field of pulmonary hypertension. A Clinical Year in Review article cannot possibly incorporate all this work and needs to be selective. The recently published European guidelines for the diagnosis and treatment of pulmonary hypertension contain an inclusive summary of all published clinical studies conducted until very recently. Here, we provide an overview of papers published after the finalisation of the guideline. In addition, we summaris...

  10. Paralisia facial bilateral Bilateral facial paralysis: a case report

    Directory of Open Access Journals (Sweden)

    J. Fortes-Rego

    1976-03-01

    Full Text Available É apresentado um caso de diplegia facial surgida após meningite meningocócica e infecção por herpes simples. Depois de discutir as diversas condições que o fenômeno pode apresentar-se, o autor inclina-se por uma etiologia herpética.A case of bilateral facial paralysis following meningococcal meningitis and herpes simplex infection is reported. The author discusses the differential diagnosis of bilateral facial nerve paralysis which includes several diseases and syndromes and concludes by herpetic aetiology.

  11. Bilateral spontaneous hemotympanum: Case report

    Directory of Open Access Journals (Sweden)

    Economou Nicolas C

    2006-10-01

    Full Text Available Abstract Background The most common causes of hemotympanum are therapeutic nasal packing, epistaxis, blood disorders and blunt trauma to the head. Hemotympanum is characterized as idiopathic, when it is detected in the presence of chronic otitis media. A rare case of spontaneous bilateral hemotympanum in a patient treated with anticoagulants is presented herein. Case presentation A 72-year-old male presented with acute deterioration of hearing. In the patient's medical history aortic valve replacement 1 year before presentation was reported. Since then he had been administered regularly coumarinic anticoagulants, with INR levels maintained between 3.4 and 4.0. Otoscopy revealed the presence of bilateral hemotympanum. The audiogram showed symmetrical moderately severe mixed hearing loss bilaterally, with the conductive component predominating. Tympanograms were flat bilaterally with absent acoustic reflexes. A computerized tomography scan showed the presence of fluid in the mastoid and middle ear bilaterally. Treatment was conservative and consisted of a 10-day course of antibiotics, anticongestants and temporary interruption of the anticoagulant therapy. After 3 weeks, normal tympanic membranes were found and hearing had returned to previous levels. Conclusion Anticoagulant intake should be included in the differential diagnosis of hemotympanum, because its detection and appropriate treatment may lead to resolution of the disorder.

  12. Novel mutations in LRP6 highlight the role of WNT signaling in tooth agenesis

    Science.gov (United States)

    Ludwig, Kerstin U.; Sullivan, Robert; van Rooij, Iris A.L.M.; Thonissen, Michelle; Swinnen, Steven; Phan, Milien; Conte, Federica; Ishorst, Nina; Gilissen, Christian; RoaFuentes, Laury; van de Vorst, Maartje; Henkes, Arjen; Steehouwer, Marloes; van Beusekom, Ellen; Bloemen, Marjon; Vankeirsbilck, Bruno; Bergé, Stefaan; Hens, Greet; Schoenaers, Joseph; Poorten, Vincent Vander; Roosenboom, Jasmien; Verdonck, An; Devriendt, Koen; Roeleveldt, Nel; Jhangiani, Shalini N.; Vissers, Lisenka E.L.M.; Lupski, James R.; de Ligt, Joep; Von den Hoff, Johannes W.; Pfundt, Rolph; Brunner, Han G.; Zhou, Huiqing; Dixon, Jill; Mangold, Elisabeth; van Bokhoven, Hans; Dixon, Michael J.; Kleefstra, Tjitske

    2016-01-01

    Purpose Here we aimed to identify a novel genetic cause of tooth agenesis (TA) and/or orofacial clefting (OFC) by combining whole exome sequencing (WES) and targeted re-sequencing in a large cohort of TA and OFC patients. Methods WES was performed in two unrelated patients, one with severe TA and OFC and another with severe TA only. After identifying deleterious mutations in a gene encoding the low density lipoprotein receptor-related protein 6 (LRP6), all its exons were re-sequenced with molecular inversion probes, in 67 patients with TA, 1,072 patients with OFC and in 706 controls. Results We identified a frameshift (c.4594delG, p.Cys1532fs) and a canonical splice site mutation (c.3398-2A>C, p.?) in LRP6 respectively in the patient with TA and OFC, and in the patient with severe TA only. The targeted re-sequencing showed significant enrichment of unique LRP6 variants in TA patients, but not in nonsyndromic OFC. From the 5 variants in patients with TA, 2 affect the canonical splice site and 3 were missense variants; all variants segregated with the dominant phenotype and in 1 case the missense mutation occurred de novo. Conclusion Mutations in LRP6 cause tooth agenesis in man. PMID:26963285

  13. MKS1 mutations cause Joubert syndrome with agenesis of the corpus callosum.

    Science.gov (United States)

    Bader, Ingrid; Decker, E; Mayr, J A; Lunzer, V; Koch, J; Boltshauser, E; Sperl, W; Pietsch, P; Ertl-Wagner, B; Bolz, H; Bergmann, C; Rittinger, O

    2016-08-01

    Joubert syndrome (JS) is a clinically and genetically heterogeneous ciliopathy characterized by episodic hyperpnea and apnea, hypotonia, ataxia, cognitive impairment and ocular motor apraxia. The "molar tooth sign" is pathognomonic of this condition. Mutations in the MKS1 gene are a major cause of Meckel-Gruber syndrome (MKS), the most common form of syndromic neural tube defects, frequently resulting in perinatal lethality. We present the phenotype and genotype of a child with severe JS and agenesis of the corpus callosum (ACC). In our patient, a next generation sequencing (NGS) approach revealed the following two variants of the MKS1 gene: first, a novel missense variant [ c.240G > T (p.Trp80Cys)], which affects a residue that is evolutionarily highly conserved in mammals and ciliates; second, a 29 bp deletion in intron 15 [c.1408-35_1408-7del29], a founder mutation, which in a homozygous state constitutes the major cause of MKS in Finland. We review the MKS1-variants in all of the eleven JS patients reported to date and compare these patients to our case. To our knowledge, this is the first patient with Joubert syndrome and agenesis of the corpus callosum where a potentially causal genotype is provided. PMID:27377014

  14. PSYCHO-MOTRIC REHABILITATION IN CHILDREN WITH AGENESIS OF CORPUS CALLOSUM (Case study

    Directory of Open Access Journals (Sweden)

    Zărnica I. Manuela Mirela

    2011-12-01

    Full Text Available Agenesis of corpus callosum is a new challenge for the puluridisciplinary team, in their efforts for the rehabilitation of childrens with this diagnose, because is a rare congenital desease with an unknown frequence of emergence. Scope. This paperwork aims to present the approach modalities of the child with agenesis of corpus callousum and the means of physical therapy that may help. Methods. This is a case study of a 5 years old girl with asenesis of corpus callosum. For assessment we used motor assessment, range of motion, reflexes. Exercises from different positions were done as games and motric songs. Sensory stimulation was also done. Rezults. By direct observation of the patient we observed significant improvement in motor function. We also foud unexpected improvement of quiet stance and dynamic balance, sensory-motor coordination. Conclusions. Game, tha main therapeutic method through its form, diversity and characteristics - atractivity, directness, creativity, movement freedom – is the main supplement of child psicho-motric development. A balanced distribution of influences upon motricity, psychic, thinking and action, nonverbal and expression language, represent the qualitative peculiarity of specialist’s and parents efforts.

  15. Nonsyndromic tooth agenesis patterns and associated developmental dental anomalies: a literature review with radiographic illustrations.

    Science.gov (United States)

    Agarwal, P; Vinuth, D P; Dube, G; Dube, P

    2013-01-01

    Tooth agenesis is one of the most intriguing phenomena, because it is frequently associated with other oral anomalies, structural variations and malformations of other teeth, late eruption, transposition and crowding. The diagnosis can be quite challenging as radiographic examination is critical for the diagnosis but not always possible and the late developing teeth may be sometimes scored developmentally missing. Accurate diagnosis therefore requires radiographic, clinical, and dental cast examinations. It is an important clinical and public health problem. Patients with missing permanent teeth may suffer from a reduced chewing ability, inarticulate pronunciation, and an unfavorable aesthetic appearance. Clinically, early diagnosis of a dental anomaly can alert the clinician to the possible development of other associated dental anomalies in the same patient or family, and avoid the possible sequelae. Understanding of tooth agenesis patterns and their impact on diagnosis, prevention, and eventually therapeutics are becoming integral parts of comprehensive dental care. Dental examination with radiographic screening of hypodontia in early childhood should be emphasized as part of public oral health policy to allow early diagnosis and timely intervention. PMID:23446448

  16. Congenital agenesis of inferior vena cava: a rare cause of unprovoked deep venous thrombosis.

    Science.gov (United States)

    Parsa, Pouria; Lane, John S; Barleben, Andrew R; Owens, Erik L; Bandyk, Dennis

    2015-07-01

    Congenital anomalies of the inferior vena cava (IVC), although rare, are a risk factor for lower limb deep venous thrombosis (DVT). A 19-year-old male presented with a left flank and groin pain caused by iliofemoral venous thrombosis. Vascular imaging by computed tomography (CT) scanning and venography demonstrated agenesis of the IVC. Catheter-directed thrombolysis via a popliteal vein was attempted but did not alter the patency of the common femoral vein outflow collaterals into the retroperitoneal azygous venous system. The patient was anticoagulated using systemic heparin infusion and clinical symptoms resolved within 5 days. He was transitioned to oral Coumadin anticoagulation, and follow-up venous duplex testing demonstrated no infrainguinal DVT and phasic venous flow with respiration in the femoral vein indicating patent collateral veins. Anomalies of the IVC are present in 0.3-0.5% of otherwise healthy individuals. Agenesis of the IVC has an incidence of 0.0005-1% in the general population but is found in almost 5% of patients venous collaterals on noncontrast CT imaging. In young adults presenting with unprovoked lower limb DVT, the presence of an IVC anomaly should be considered and evaluated for by venous duplex testing and if necessary CT venography.

  17. Uterus didelphys with an obstructed unilateral vagina and ipsilateral renal agenesis: A rare cause of dysmenorrhoea.

    Science.gov (United States)

    Attar, Rukset; Yıldırım, Gazi; Inan, Yücel; Küzılkale, Ozge; Karateke, Ateş

    2013-01-01

    Didelphic uterus with obstructed hemivagina and ipsilateral renal agenesis is a rare condition. It usually presents with pelvic pain following the menarche, dysmenorrhoea, and an increase in abdominal volume or a palpable mass due to unilateral haematocolpos. We present the case of a 13-year-old girl who referred with recurrent pelvic pain, mainly at the time of menses, and irregular menstrual cycle complaints in this report. The patient underwent ultrasonography and magnetic resonance (MR) imaging of the pelvis was performed. The diagnosis was uterus didelphys with obstructed hemivagina and ipsilateral renal agenesia. Laparotomy was performed for diagnosis and treatment purposes. Two separated hemiuteri and two cervices with hematometra and hematocolpos on the right side and ipsilateral renal agenesis were detected. The vaginal septum was excised completely and Strassman metroplasty was performed. Her complaints were resolved and she was absolutely asymptomatic after surgery. Diagnosis and management of this congenital anomaly is challenging due to the complexity of the anatomic structures, nonspecific complaints, and heterogenic presentation. These anomalies must always be considered while working-up female patients presenting with episodic abdominal pain and abdominopelvic mass. PMID:24592115

  18. Pulmonary manifestation of leptospirosis

    Energy Technology Data Exchange (ETDEWEB)

    Im, Jung Gi; Yeon, Kyung Mo; Han, Man Chung; Kim, Chu Wan; Lee, Jung Sang; Kim, Suhng Gwon; Han, Yong Chol; Chang, Woo Hyun; Chi, Je Geun [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1986-02-15

    Authors analysed and present chest X-ray findings of serologically proven leptospirosis from Seoul National University Hospital, either admitted or referred for serological verification, during recent 2 years. Radiological findings were correlated with the lung specimen findings of experimentally induced leptospirosis in guinea pig. The results are as follows: 1. 24 cases (56%) showed positive X-ray findings. 2. Predominant radiological patterns of involved lung were tiny dot, small nodule, rosette density in 11 cases, massive confluent consolidation in 4 cases, and diffuse ill-defined velly increased density in 9 cases. 3. Distribution of pulmonary lesions were bilateral (100%), non-lobar, non-segmental (95%), and there were conspicuous tendency of peripheral lung predominance. 4. Extrapulmonary manifestation, such as pleural effusion or cardiomegaly was rate. 5. Pulmonary lesions resolved completely usually 5 to 10 days after their appearance. 6. From the gross and microscopic findings of serially sacrificed guinea pig's lung and a case of autopsy, authors concluded that fine dot-like density in chest X-ray was due to paleolithic hemorrhage in intraalveolar space at initial stage, growing up to ressette density or confluent consolidation as the pathetic extends to surrounding lung forming purpura and coalescent hemorrhage.

  19. Treatment to pulmonary alveolar proteinosis with total bronchoalveolar lavage

    International Nuclear Information System (INIS)

    The pulmonary alveolar proteinosis is a rare disease with variable course and characterized by the accumulation of surfactant in the alveoli. By the treatment it was used the alveolar lavage with good results. We present the experience accumulated in the Las Americas clinic of Medellin city with the treatment of three patients with diagnosis of pulmonary alveolar proteinosis by alveolar lavage. We realized seven lavages, two of them bilateral and sequential. We didn't 't have important complications and in all cases we got good clinical and physiological results. Two patients had relapsed six and seven months after the first lavage and they needed a second lavage. We conclude that the pulmonary lavage is a secure and effective procedure in the treatment of the pulmonary alveolar proteinosis and the modality of bilateral sequential lavage is a good alternative and less expensive

  20. Types of Pulmonary Hypertension

    Science.gov (United States)

    ... from the NHLBI on Twitter. Types of Pulmonary Hypertension The World Health Organization divides pulmonary hypertension (PH) ... are called pulmonary hypertension.) Group 1 Pulmonary Arterial Hypertension Group 1 PAH includes: PAH that has no ...

  1. Acute silicosis with bilateral pneumothorax

    OpenAIRE

    Srivastava, G. N.; Prasad, Rajniti; Meena, Manoj; Hussain, Moosa

    2014-01-01

    We present a case of acute silicosis with bilateral pneumothorax of a 28-year-old man working at a stone crusher factory for 1 year. He presented to the emergency department with cough, respiratory distress and diffuse chest pain. The patient was managed with bilateral intercostal tube drainage under water seal, oxygen inhalation and conservative therapy. On follow-up he showed improvement of resting dyspnoea and was doing well. This case is being reported because of the rare complications of...

  2. Herlyn-Werner-Wunderlich Syndrome with Unilateral Hemivaginal Obstruction, Ipsilateral Renal Agenesis, and Contralateral Renal Thin GBM Disease: A Case Report with Radiological Follow Up

    International Nuclear Information System (INIS)

    Herlyn-Werner-Wunderlich syndrome is a rare Mullerian ductal anomaly that is characterized by the presence of a hemivaginal septum, a didelphic uterus and ipsilateral renal agenesis. It is generally difficult to diagnose the uterine malformation before menarche owing to its small size. Therefore, a follow-up study is very important for confirming the uterine malformation in girls with renal agenesis. We report a patient with renal agenesis and microscopic hematuria, who showed symptoms before menarche. A follow-up study eventually revealed uterine didelphys with a hemivaginal obstruction. A biopsy proved that the microscopic hematuria was caused by thin glomerular basement membrane disease of the contralateral kidney

  3. Herlyn-Werner-Wunderlich Syndrome with Unilateral Hemivaginal Obstruction, Ipsilateral Renal Agenesis, and Contralateral Renal Thin GBM Disease: A Case Report with Radiological Follow Up

    Energy Technology Data Exchange (ETDEWEB)

    Park, Noh Hyuck; Park, Hee Jin; Park, Chan Sup [Myongji Hospital, Kwandong University, Koyang (Korea, Republic of); Park, Sung Il [Bucheon Hospital, Soonchunhyang University, Bucheon (Korea, Republic of)

    2010-08-15

    Herlyn-Werner-Wunderlich syndrome is a rare Mullerian ductal anomaly that is characterized by the presence of a hemivaginal septum, a didelphic uterus and ipsilateral renal agenesis. It is generally difficult to diagnose the uterine malformation before menarche owing to its small size. Therefore, a follow-up study is very important for confirming the uterine malformation in girls with renal agenesis. We report a patient with renal agenesis and microscopic hematuria, who showed symptoms before menarche. A follow-up study eventually revealed uterine didelphys with a hemivaginal obstruction. A biopsy proved that the microscopic hematuria was caused by thin glomerular basement membrane disease of the contralateral kidney

  4. A Triad of Complete Dorsal Pancreatic Agenesis, Pancake Kidney and Bicornuate Uterus. An Association or an Incidental Finding: First Case in Literature

    Directory of Open Access Journals (Sweden)

    Anupam Lal

    2015-05-01

    Full Text Available Context Agenesis of the dorsal pancreas is a rare congenital anomaly which can be detected either incidentally during imaging or encountered during investigation for pancreas related pathologies such as pancreatitis or diabetes mellitus. Few associations of this condition with heterotaxy syndrome, pancreatic tumors and congenital heart disease have been described. Case report We report a novel association of genitourinary anomalies in a case of complete dorsal pancreatic agenesis not previously described in the literature. Conclusion Knowledge about dorsal pancreatic agenesis is essential for radiologist in patients presenting with unexplained pain abdomen and its association with various conditions may help radiologist in assisting clinician in management of these patients.

  5. Pulmonary embolism

    International Nuclear Information System (INIS)

    Pulmonary embolism is a common medical problem whose incidence is likely to increase in our aging population. Although it is life-threatening, effective therapy exists. The treatment is not, however, without significant complications. Thus, accurate diagnosis is important. Unfortunately, the clinical manifestations of pulmonary embolism are nonspecific. Furthermore, in many patients the symptoms of an acute embolism are superimposed on underlying chronic heart or lung disease. Thus, a high index of suspicion is needed to identify pulmonary emboli. Laboratory parameters, including arterial oxygen tensions and electrocardiography, are as nonspecific as the clinical signs. They may be more useful in excluding another process than in diagnosing pulmonary embolism. The first radiologic examination is the chest radiograph, but the clinical symptoms are frequently out of proportion to the findings on the chest films. Classic manifestations of pulmonary embolism on the chest radiograph include a wedge-shaped peripheral opacity and a segmental or lobar diminution in vascularity with prominent central arteries. However, these findings are not commonly seen and, even when present, are not specific. Even less specific findings include cardiomegaly, pulmonary infiltrate, elevation of a hemidiaphragm, and pleural effusion. Many patients with pulmonary embolism may have a normal chest radiograph. The chest radiograph is essential, however, for two purposes. First, it may identify another cause of the patient's symptoms, such as a rib fracture, dissecting aortic aneurysm, or pneumothorax. Second, a chest radiograph is essential to interpretation of the radionuclide V/Q scan. The perfusion scan accurately reflects the perfusion of the lung. However, a perfusion defect may result from a variety of etiologies. Any process such as vascular stenosis or compression by tumor may restrict blood flow. 84 references

  6. Acute respiratory distress syndrome: Pulmonary and extrapulmonary not so similar

    OpenAIRE

    Inderpaul Singh Sehgal; Sahajal Dhooria; Digambar Behera; Ritesh Agarwal

    2016-01-01

    Acute respiratory distress syndrome (ARDS) is characterized by acute onset respiratory failure with bilateral pulmonary infiltrates and hypoxemia. Current evidence suggests different respiratory mechanics in pulmonary ARDS (ARDSp) and extrapulmonary ARDS (ARDSexp) with disproportionate decrease in lung compliance in the former and chest wall compliance in the latter. Herein, we report two patients of ARDS, one each with ARDSp and ARDSexp that were managed using real-time esophageal pressure m...

  7. Acute respiratory distress syndrome: Pulmonary and extrapulmonary not so similar

    Directory of Open Access Journals (Sweden)

    Inderpaul Singh Sehgal

    2016-01-01

    Full Text Available Acute respiratory distress syndrome (ARDS is characterized by acute onset respiratory failure with bilateral pulmonary infiltrates and hypoxemia. Current evidence suggests different respiratory mechanics in pulmonary ARDS (ARDSp and extrapulmonary ARDS (ARDSexp with disproportionate decrease in lung compliance in the former and chest wall compliance in the latter. Herein, we report two patients of ARDS, one each with ARDSp and ARDSexp that were managed using real-time esophageal pressure monitoring using the AVEA ventilator to tailor the ventilatory strategy.

  8. Sudden death of a patient with pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Nakhla, Hassan; Jumbelic, Mary I

    2005-06-01

    We report a case of sudden death due to bilateral pneumothorax in a previously healthy 16-year-old adolescent white girl. She presented with sudden onset of shortness of breath followed by loss of consciousness. Postmortem chest radiograph showed bilateral pneumothoraces. Autopsy confirmed the bilateral pneumothorax and additionally showed emphysematous changes and bullae throughout the lung tissue. Microscopic sections of the lungs showed Langerhans cell histiocytosis. To the best of our knowledge, this is the first reported case of fatal presentation of pulmonary Langerhans cell histiocytosis. PMID:15913433

  9. Pulmonary imaging

    International Nuclear Information System (INIS)

    Studies in pulmonary imaging predominantly depict the distribution of pulmonary perfusion or the ventilation-perfusion balance for the detection of pulmonary embolism, obstructive airway disease, and lung carcinoma. Much work has been done using 133Xe to study both regional ventilation and blood flow. This work has led to a greater understanding of regional blood flow and ventilation, of lung anatomy and morphometry, of the mechanical factors that influence lung function, and of the biochemical and other nonrespiratory functions of the lung. The recent development of emission tomography and the potential for receptor- and function-oriented radiopharmaceuticals have allowed several important regional physiologic processes to be measured. The physical parameters measured in radionuclide emission studies are the temporal and spatial relations of the distribution of tracer in the lungs. Tracers that are diffusible will wash into and out of the lungs at a rate proportional not only to flow but also to membrane permeability. If the radionuclide label is attached to microspheres or macro-aggregates, the amount lodging in any portion of the lung can be related to relative flow in the lung. Metabolism of the lung is reflected by the rate of extraction and disappearance of various labeled radiopharmaceuticals. The problems encountered in assessing the value of ventilation-perfusion scans for the diagnosis of pulmonary embolism are concerned not only with improving specificity, but also with better estimation of the prior probability of pulmonary embolism in the patient being evaluated

  10. Primary synchronous bilateral breast cancer

    Directory of Open Access Journals (Sweden)

    R Krishnappa

    2014-01-01

    Full Text Available Background: Primary synchronous bilateral breast cancer (PSBBC is a rare clinical entity. The reported incidence ranges between 0.3% and 12%. There are several controversial issues regarding PSBBC pertaining to the diagnostic criteria, nomenclature, and management policies. Materials and Methods: Fourteen cases of PSBBC treated between 2001 to 2010 at our institute were retrospectively analysed in regards to demographic data, management and follow up. Results: PSBBC constituted 0.19% of total breast cancer patients at our institute. Age ranged from 28 to 78 years. PSBBC were detected by clinical examination in eight cases and by mammography in six cases. Twelve patients underwent bilateral modified radical mastectomy, one had unilateral mastectomy on one side and breast conservation on the other side and one patient has bilateral breast conservation. Majority of patients belonged to stage 2 and stage 3. All patients were found to have invasive ductal carcinoma. Five cases were ER/PR positive and 8 patients were triple hormone receptor negative. Eight patients received unilateral and six received bilateral adjuvant radiotherapy. Nine patients received adjuvant chemotherapy. 5 patients received adjuvant hormonal therapy. Median follow up of patients was 15.4 months. Conclusion: PSBBC is a rare event warranting awareness and screening of the contralateral breast in patients with unilateral breast cancer. These patients require individualized treatment planning based on the tumor factors of the index lesion. Further multi institutional prospective studies are needed for adequate understanding of management of PSBBC.

  11. Spontaneous bilateral quadriceps tendon rupture.

    Science.gov (United States)

    Vigneswaran, N; Lee, K; Yegappan, M

    2007-11-01

    Spontaneous bilateral quadriceps tendon ruptures are uncommon. We present a 30-year-old man with end-stage renal failure, who sustained this injury, and subsequently had surgical repair of both tendons on separate occasions. He has since regained full range of movement of both knees.

  12. Bilateral familial nevus of Ota.

    Science.gov (United States)

    Goyal, Sunali; Uwaydat, Sami H; Phillips, Paul H; Schaefer, G Bradley

    2014-12-01

    Nevus of Ota is a benign congenital melanocytic lesion found most commonly in people of Asian ancestry. It is associated with an increased risk of glaucoma and uveal melanomas. Most cases are sporadic and unilateral. We present the first reported case of a brother and sister with familial, bilateral nevus of Ota.

  13. Characterization of novel MSX1 mutations identified in Japanese patients with nonsyndromic tooth agenesis.

    Directory of Open Access Journals (Sweden)

    Seishi Yamaguchi

    Full Text Available Since MSX1 and PAX9 are linked to the pathogenesis of nonsyndromic tooth agenesis, we performed detailed mutational analysis of these two genes sampled from Japanese patients. We identified two novel MSX1 variants with an amino acid substitution within the homeodomain; Thr174Ile (T174I from a sporadic hypodontia case and Leu205Arg (L205R from a familial oligodontia case. Both the Thr174 and Leu205 residues in the MSX1 homeodomain are highly conserved among different species. To define possible roles of mutations at these amino acids in the pathogenesis of nonsyndromic tooth agenesis, we performed several functional analyses. It has been demonstrated that MSX1 plays a pivotal role in hard tissue development as a suppressor for mesenchymal cell differentiation. To evaluate the suppression activity of the variants in mesenchymal cells, we used the myoD-promoter, which is one of convenient reporter assay system for MSX1. Although the gene products of these MSX1 variants are stable and capable of normal nuclear localization, they do not suppress myoD-promoter activity in differentiated C2C12 cells. To clarify the molecular mechanisms underlying our results, we performed further analyses including electrophoretic mobility shift assays, and co-immunoprecipitation assays to survey the molecular interactions between the mutant MSX1 proteins and the oligonucleotide DNA with MSX1 consensus binding motif or EZH2 methyltransferase. Since EZH2 is reported to interact with MSX1 and regulate MSX1 mediated gene suppression, we hypothesized that the T174I and L205R substitutions would impair this interaction. We conclude from the results of our experiments that the DNA binding ability of MSX1 is abolished by these two amino acid substitutions. This illustrates a causative role of the T174I and L205R MSX1 homeodomain mutations in tooth agenesis, and suggests that they may influence cell proliferation and differentiation resulting in lesser tooth germ formation in

  14. Bilateral Adrenal Incidentalomas: A Rare Presentation of Lung Cancer

    Directory of Open Access Journals (Sweden)

    Halit Diri

    2015-01-01

    Full Text Available Adrenal incidentalomas are found incidentally during a radiologic examination performed for indications other than an adrenal disease, and 15% of them are bilateral adrenal masses. This study describes a 51-year-old male smoker patient admitted with diabetes mellitus. An abdominal ultrasonography performed due to his anemia revealed bilateral adrenal masses. His chest X-ray showed abnormal 10 cm opacity at the right upper lung, and brain, thorax, and abdomen CT scans showed multiple lesions compatible with lung cancer metastases. The pathological examination of the transthoracic lung biopsy specimen was consistent with lung adenocarcinoma. Findings in this patient indicate that, in middle aged patients with bilateral adrenal mass and a history or finding of any malignancy, the first diagnosis which should be considered is adrenal metastasis, and confirming the diagnosis by adrenal biopsy may be useless. Furthermore, screening all smoking patients by chest X-ray or thoracic CT for lung cancer may not be accepted as a routine procedure, but in smokers admitted to a hospital due to signs and symptoms attributed to a pulmonary disease, at least a chest X-ray should be requested.

  15. Whole lung lavage for pulmonary alveolar proteinosis

    Directory of Open Access Journals (Sweden)

    Jayaraman S

    2010-01-01

    Full Text Available A 26-year-old male presented with complaints of dry cough of six months and progressive breathlessness of three months duration. He was coughing out milky white sputum for two months and had lost 12 kg weight in two months. He had an evening rise in temperature of one month duration. Clinically, the patient was in respiratory distress and the respiratory system examination revealed bilateral velcro crackles. High resolution computed tomography chest showed bilateral diffuse reticulonodular opacities and "Crazy Paving" pattern suggestive of alveolar proteinosis. Broncho alveolar lavage showed eosinophilic granular material, which was periodic acid-Schiff positive. Open lung biopsy was done to confirm the diagnosis and the histopathologic examination revealed eosinophilic secretions with granular appearance suggestive of pulmonary alveolar proteinosis. Subsequently, patient underwent bilateral sequential whole lung lavage under general anesthesia. Patient showed marked clinical and radiological improvement after sequential whole lung lavage.

  16. Mitral valve surgery in a patient with dextrocardia and 180° counter-clockwise rotated heart due to congenital agenesis of the right lung.

    Science.gov (United States)

    Atsumi, Yosuke; Tokunaga, Shigehiko; Yasuda, Shota; Fushimi, Kenichi; Masuda, Munetaka

    2013-11-01

    We report a case of severe mitral regurgitation (MR) with dextrocardia and 180° counterclockwise rotated situs solitus heart. We describe the technique for mitral valve surgery in a patient with dextrocardia and agenesis of the right lung.

  17. Agenesis of maxillary lateral incisor in an Angle Class II, Division 1 malocclusion patient

    Directory of Open Access Journals (Sweden)

    Guilherme Thiesen

    2015-10-01

    Full Text Available The present case report describes the orthodontic treatment of a patient with agenesis of maxillary left lateral incisor and Angle Class II, Division 1 malocclusion. The patient also presented with maxillary midline deviation and inclination of the occlusal plane in the anterior region. Treatment objectives were: correction of sagittal relationship between the maxilla and the mandible; correction of midline deviation, so as to cause maxillary and mandibular midlines to coincide; correction of overbite and leveling of the occlusal plane, so as to create ideal conditions for esthetic rehabilitation of anterior teeth. This case was presented to the Brazilian Board of Orthodontics and Dentofacial Orthopedics (BBO as a requirement for the title of certified by the BBO.

  18. Congenital Agenesis of the Internal Jugular Vein: An Extremely Rare Anomaly

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    Oguz Kayiran

    2015-01-01

    Full Text Available Vascular anomalies of major venous vessels are rarely seen. Moreover, congenital absence of internal jugular vein is extremely uncommon. In our case, a female patient presented with primary unknown left cervical mass. Cervical ultrasonography demonstrated absence of right internal jugular vein. In addition, computed tomography and dynamic magnetic resonance imaging scans confirmed this diagnosis. Compensatory left internal jugular vein enlargement mimicked sort of cervical mass. Venous magnetic resonance imaging images revealed the absence of right internal jugular vein with compensatory left internal jugular vein dominance. In the literature, the agenesis of IJV was mentioned in a case with concomitant multiple problems. Here, an asymptomatic case is reported with an incident diagnosis. No interventions were planned upon the patient's request. It should be kept in mind that any kind of anomalies can be seen during venous access and neck surgery.

  19. Hymen Tearing in the Treatment of Vaginal Agenesis: Implications on Women in Turkey.

    Science.gov (United States)

    Yaman, Şengül; Ayaz, Sultan

    2014-07-01

    Rupture of the hymen before marriage is a significant problem for a woman and her family. The purpose of this article is to present a case report where hymen membrane perforation was required for vaginal agenesis in a 13-year-old adolescent, identify psychosocial issues related to the torn hymen in the Turkish culture, discuss gender inequality affecting health care of women with a torn hymen, identify the impact of a torn hymen on a Turkish woman's social status, and discuss health care implications for the woman and her family. The girl could not understand the situation and experienced feelings of guilt. The parents displayed an intense reaction of shock and denial after being informed about the disorder and treatment process and felt a sense of hopelessness about the future of their daughter. Nurses are expected to fulfill their training, counseling, and advocacy roles about this issue.

  20. Tratamiento conservador en pacientes con retinoblastoma bilateral

    OpenAIRE

    Juan C. Suárez; Mabel C. Ospina; Sandra A. Arias; González, María E

    2008-01-01

    OBJETIVO: comparar el tratamiento convencional del retinoblastoma bilateral, usado hasta hace algunos años, consistente en radioterapia o enucleación bilateral, con el tratamiento conservador actual que incluye termoterapia transpupilar (TTT) o TTT/quimioterapia al menos en un ojo, en niños con diagnóstico de retinoblastoma bilateral. DISEÑO: estudio retrospectivo descriptivo. MUESTRA: 20 pacientes con diagnóstico de retinoblastoma bilateral que consultaron al Hospital Universitario San Vicen...

  1. Laparoscopic Adrenalectomy for Bilateral Metachronous Aldosteronomas

    OpenAIRE

    Rizek, Philippe; Gorecki, Piotr; Lindenmayer, Aristid; Moktan, Sabita

    2011-01-01

    Introduction: Primary aldosteronism affects 5% to 13% of patients with hypertension. Idiopathic bilateral hyperplasia (IHA) and unilateral aldosterone-producing adenoma (APA) are the most common types of primary aldosteronism. Bilateral APA is a very rare entity with only a few reports in the literature. We present the case of a patient with metachronous bilateral APA treated with metachronous bilateral total and near total adrenalectomy. Case Report: A 66-year-old female was evaluated for hy...

  2. Pulmonary histiocytosis

    Directory of Open Access Journals (Sweden)

    Milenković Branislava

    2013-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis encompasses a group of disorders of unknown origin with different clinical presentations and outcomes. It is characterized by infiltration of the involved tissues by large numbers of Langerhans cells, often organized into granulomas. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, splenomegaly, and dysfunction of organ such as the pituitary gland, lungs, liver, and bone marrow. Pulmonary histiocytosis. Adult pulmonary Langerhans cell histiocytosis is a rare disorder of unknown etiology. It occurs predominantly in male smokers, with an incidence peak between 20 and 40 years of age. High-resolution computed tomography of the chest can show nodules, cavitated nodules, and thickand thin-walled cysts. The definite diagnosis of pulmonary Langerhans cell histiocytosis requires identification of Langerhans’ cell granulomas infiltrating and destroying distal bronchioles, which is usually achieved by lung biopsy at a site selected by chest high-resolution computed tomography. Treatment. Treatment options for adults have never been clarified by a clinical trial. The published literature provides minimal data on the comparative efficacy of various treatment options which include surgery/curettage, steroids, radiation, and various chemotherapy regimens. The improved understanding of the mechanisms involved in the pathogenesis of pulmonary Langerhans cell histiocytosis should help in the development of specific therapeutic strategies and effective treatment.

  3. Simultaneous and staged bilateral total hip arthroplasty

    DEFF Research Database (Denmark)

    Lindberg-Larsen, Martin; Joergensen, Christoffer Calov; Husted, Henrik;

    2013-01-01

    Bilateral total hip arthroplasty (BTHA) and bilateral simultaneous total hip arthroplasty (BSTHA) are done increasingly. Previous studies evaluating outcomes after bilateral procedures have found different results. The aim of this study was to investigate length of hospital stay (LOS), 30 days...

  4. Bilateral pallidotomy for generalized dystonia Palidotomia bilateral para distonias generalizadas

    Directory of Open Access Journals (Sweden)

    Hélio A. G. Teive

    2001-06-01

    Full Text Available OBJECTIVE: To evaluate the efficacy and safety of bilateral pallidotomies in five patients with generalized dystonia. BACKGROUND: Generalized dystonias are frequently a therapeutic challenge, with poor responses to pharmacological treatment. GPi (globus pallidus internus pallidotomies for Parkinson's disease ameliorate all kinds of dyskinesias/dystonia, and recent studies reported a marked improvement of refractory dystonias with this procedure. METHODS: Five patients with generalized dystonias refractory to medical treatment were selected; one posttraumatic and four idiopathic. The decision to perform bilateral procedures was based on the predominant axial involvement in these patients. Dystonia severity was assessed with the Burke-Fahn-Marsden Dystonia Scale (BFM. Simultaneous procedures were performed in all but one patient, who had a staged procedure. They were reevaluated with the same scale (BFM by an unblinded rater at 1, 2, 3, 30, 60, 90, 120 and 180 days post-operatively. RESULTS: The four patients with idiopathic dystonia showed a progressive improvement up to three months; the patient with posttraumatic dystonia relapsed at three months. One patient had a marked improvement, being able to discontinue all the medications. A mean decrease in the BFM scores of 52,58% was noted. One patient had a trans-operative motor seizure followed by a transient hemiparesis secondary to rack hemorrhage; other was lethargic up to three days after the procedure. CONCLUSIONS: Our results show that bilateral GPi pallidotomies may be a safe and effective approach to medically refractory generalized dystonias; it can also be speculated that the posttraumatic subgroup may not benefit with this procedure.As distonias generalizadas são freqüentemente um desafio terapêutico, com pobres respostas aos tratamentos farmacológicos. As cirurgias estereotáxicas, como a palidotomia, têm sido utilizadas com êxito no tratamento da doença de Parkinson e estudos

  5. Development of bilateral coronary artery aneurysms in a child with Noonan syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Mauro, David M.; Flors, Lucia; Norton, Patrick T.; Hagspiel, Klaus D. [University of Virginia Health System, Department of Radiology and Medical Imaging, Charlottesville, VA (United States); Hoyer, Andrew W. [University of Virginia Health System, Department of Pediatrics, Division of Pediatric Cardiology, Charlottesville, VA (United States); Pediatric Cardiology Center of Oregon, Portland, OR (United States)

    2016-03-15

    Noonan syndrome is a constellation of congenital malformations including heart defects, facial anomalies and short stature. The cardiovascular defects are variable and extensive, with the most common being pulmonary stenosis and hypertrophic cardiomyopathy. Coronary artery anomalies have only been reported in a few cases. We report a child with Noonan syndrome status post pulmonary stenosis and atrial septal defect repair, who developed bilateral coronary artery aneurysms. The aneurysms were diagnosed with both cardiac magnetic resonance imaging and coronary computed tomography angiography. There had been no evidence of them on a cardiac MR exam 5 years previously. (orig.)

  6. Development of bilateral coronary artery aneurysms in a child with Noonan syndrome.

    Science.gov (United States)

    Mauro, David M; Flors, Lucia; Hoyer, Andrew W; Norton, Patrick T; Hagspiel, Klaus D

    2016-03-01

    Noonan syndrome is a constellation of congenital malformations including heart defects, facial anomalies and short stature. The cardiovascular defects are variable and extensive, with the most common being pulmonary stenosis and hypertrophic cardiomyopathy. Coronary artery anomalies have only been reported in a few cases. We report a child with Noonan syndrome status post pulmonary stenosis and atrial septal defect repair, who developed bilateral coronary artery aneurysms. The aneurysms were diagnosed with both cardiac magnetic resonance imaging and coronary computed tomography angiography. There had been no evidence of them on a cardiac MR exam 5 years previously. PMID:26515448

  7. Bilateral Lhermitte-Duclos disease

    Directory of Open Access Journals (Sweden)

    Bozbuga Mustafa

    2010-01-01

    Full Text Available Lhermitte-Duclos disease (LDD is a pathologic entity with progrediating, diffuse hypertrophy chiefly of the stratum granulosum of the cerebellum. Typically LDD is a unilateral lesion of the cerebellum or in vermis. Here we report a case of LDD with bilateral lesions of cerebellar hemispheres managed surgically. A 28-year-old woman presented with one-year history of progressive headache, nausea, vomiting, and blurred vision. Neurologic examination revealed a bilateral mild papilledema, mild dysmetria, and dysdiadochokinesia. The cerebellar lesions caused moderate mass effect in posterior fossa with hydrocephalus, and Chiari type I malformation. We performed the suboccipital-retrosigmoid approach, and removed completely the left intracerebellar mass. Symptoms related to elevated intracranial pressure disappeared in a short period postoperatively.

  8. Immediate Sequential Bilateral Cataract Surgery

    DEFF Research Database (Denmark)

    Kessel, Line; Andresen, Jens; Erngaard, Ditte;

    2015-01-01

    The aim of the present systematic review was to examine the benefits and harms associated with immediate sequential bilateral cataract surgery (ISBCS) with specific emphasis on the rate of complications, postoperative anisometropia, and subjective visual function in order to formulate evidence......-based national Danish guidelines for cataract surgery. A systematic literature review in PubMed, Embase, and Cochrane central databases identified three randomized controlled trials that compared outcome in patients randomized to ISBCS or bilateral cataract surgery on two different dates. Meta-analyses were...... performed using the Cochrane Review Manager software. The quality of the evidence was assessed using the GRADE method (Grading of Recommendation, Assessment, Development, and Evaluation). We did not find any difference in the risk of complications or visual outcome in patients randomized to ISBCS or surgery...

  9. Bilateral Molariform Mandibular Second Premolars

    OpenAIRE

    Sonu Acharya; Pradip Kumar Mandal; Chiranjit Ghosh

    2015-01-01

    Macrodontia is a rare dental anomaly that refers to teeth that appear larger than normal. Generalised macrodontia can be associated with certain medical conditions and syndromes. This case report presents clinical and radiographic findings of isolated bilateral macrodontia in a 14-year-old child. The patient was referred to the clinic with local crowding of maxillary and mandibular teeth. Radiographic findings revealed the presence of impacted macrodont mandibular second premolar on one side ...

  10. Compensation following bilateral vestibular damage

    Directory of Open Access Journals (Sweden)

    Bill J Yates

    2011-12-01

    Full Text Available Bilateral loss of vestibular inputs affects far fewer patients than unilateral inner ear damage, and thus has been understudied. In both animal subjects and human patients, bilateral vestibular hypofunction (BVH produces a variety of clinical problems, including impaired balance control, inability to maintain stable blood pressure during postural changes, difficulty in visual targeting of images, and disturbances in spatial memory and navigational performance. Experiments in animals have shown that nonlabyrinthine inputs to the vestibular nuclei are rapidly amplified following the onset of BVH, which may explain the recovery of postural stability and orthostatic tolerance that occurs within 10 days. However, the loss of the vestibulo-ocular reflex and degraded spatial cognition appear to be permanent in animals with BVH. Current concepts of the compensatory mechanisms in humans with BVH are largely inferential, as there is a lack of data from patients early in the disease process. Translation of animal studies of compensation for BVH into therapeutic strategies and subsequent application in the clinic is the most likely route to improve treatment. In addition to physical therapy, two types of prosthetic devices have been proposed to treat individuals with bilateral loss of vestibular inputs: those that provide tactile stimulation to indicate body position in space, and those that deliver electrical stimuli to branches of the vestibular nerve in accordance with head movements. The relative efficacy of these two treatment paradigms, and whether they can be combined to facilitate recovery, is yet to be ascertained.

  11. Pulmonary hyalinizing granuloma associated with Aspergillus infection.

    Science.gov (United States)

    Pinckard, J Keith; Rosenbluth, Daniel B; Patel, Kishor; Dehner, Louis P; Pfeifer, John D

    2003-01-01

    A 38-year-old immunocompetent man with occupational exposure to Aspergillus presented with dyspnea, pleuritic chest pain, and hemoptysis. Chest roentgenograms and computed tomography scans demonstrated multiple pulmonary nodules bilaterally. An initial set of bronchial washing cultures grew Aspergillus fumigatus, serologic testing showed an elevated anti-Aspergillus titer, and immunodiffusion testing was positive for antibody against A. fumigatus and A. niger. There was no microbiologic or serologic evidence of infection by other pathogens, and no clinical or laboratory evidence of autoimmune disease. An open lung biopsy was diagnostic of pulmonary hyalinizing granuloma. This novel association with Aspergillus infection not only expands the spectrum of pathogens linked to pulmonary hyalinizing granuloma but also documents a new pattern of lung disease that can be caused by Aspergillus. PMID:12598920

  12. Traumatic pulmonary pseudocyst: A case report

    Directory of Open Access Journals (Sweden)

    İbrahim Koç

    2014-06-01

    Full Text Available Post-traumatic pulmonary pseudocyst (TPP is an uncommon cavitary lesion of the lung. TPP is an air/fluid filled cavity without a true epithelial lining within the pulmonary parenchyma. TPPs usually occur following a motor vehicle accident or fall. Here we report a case of TPP which occurred after blunt chest trauma after a traffic accident. The chest X-ray few hours after the accident revealed mass/cyst like lesion on the right lover zone. Computed tomography of the thorax revealed bilateral cystic lesions on the parenchyma. The patient was treated conservatively with antibiotherapy. He was asymptomatic thereafter, and the traumatic pulmonary pseudocyst radiologically was completely resolved three months later. TPP might be confused with other cystic and cavitary lesions of the lung but radiological rapid onset is important in the differential diagnosis. J Clin Exp Invest 2014; 5 (2: 304-306

  13. Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren’s Syndrome

    Directory of Open Access Journals (Sweden)

    Go Makimoto

    2012-01-01

    Full Text Available Sjögren’s syndrome (SS is a systemic autoimmune disease characterized by sicca symptoms. Interstitial pulmonary fibrosis and tracheobronchial sicca are the most common symptoms of pulmonary involvement in primary SjS, and they are rarely accompanied by serositis such as pleuritis or pericarditis. We report a case of SS presenting initially with bilateral pleural effusions. A 63-year old man was admitted to our hospital with a one-month history of cough, dyspnea, and right chest pain. Chest-computed tomography revealed bilateral pleural effusions. Serum anti-SS-A antibody titer was 1 : 256. Ophthalmological examination revealed a positive Schirmer test. Lip biopsy showed atrophy and plasmacytic infiltration of the salivary gland. Corticosteroid treatment was initiated. Pleural effusions were almost completely resolved by day 30. The patient has not experienced any recurrence.

  14. Significance of diffuse pulmonary uptake in radiogallium scans: concise communication

    International Nuclear Information System (INIS)

    Diffuse pulmonary uptake of radiogallium was observed in 50 out of 510 scans (9.8%) performed in a general hospital over a period of 1 y. Of the 50 cases, 80% had bilateral, diffuse distribution, and 20% unilateral. A variety of clinical conditions produced a similar picture on the pulmonary images. The most common cause of the diffuse uptake was infectious disease (48%) followed by infiltrative disorders (30%) and neoplastic conditions (22%). On a repeat study there was significant reduction in the intensity of pulmonary radiogallium uptake following drug therapy in three patients - sarcoidosis on corticosteroid therapy, pneumocystis carinii treated with trimethoprim and sulfamethoxozole, and interstitial pulmonary fibrosis on corticosteroids. No close correlation was observed between the underlying clinical disorder and the pattern or intensity of pulmonary uptake of radiogallium

  15. Complex malformations of the urogenital tract in a female dog: Gartner duct cyst, ipsilateral renal agenesis, and ipsilateral hydrometra.

    Science.gov (United States)

    Fujita, Atsushi; Tsuboi, Masaya; Uchida, Kazuyuki; Nishimura, Ryohei

    2016-05-01

    A 10-month-old female toy poodle was referred to the University of Tokyo Veterinary Medical Center with a urogenital anomaly found during sterilization. An exploratory laparotomy revealed a cyst adhering to the cervix and a unilateral renal agenesis. Histopathology and immunohistochemical analysis of the cyst was consistent with remnants of the Wolffian duct or a Gartner duct cyst. This is a rare case of a canine Gartner duct cyst with renal agenesis and uterine anomaly. We discuss the similarity of this case to that of humans and introduce a classification in the literature for these complex urogenital malformations for further clinical research into the precise diagnosis and appropriate surgical planning. PMID:27506089

  16.  Uterus Didelphys with Obstructed Right Hemivagina, Ipsilateral Renal Agenesis and Right Pyocolpos: A Case Report

    Directory of Open Access Journals (Sweden)

    Hansa Dhar

    2011-11-01

    Full Text Available  Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA Syndrome is a rare congenital anomaly of the female genital tract. Uterus didelphys occurs when the midline fusion of the mullerian ducts is arrested, either completely or incompletely. Women with didelphic uterus may be asymptomatic and unaware of having a double uterus. They may present with complaints of dysmenorrhoea and dyspareunia. A 25 year old woman attending the infertility clinic at Nizwa regional referral hospital presented with history of dysmenorrhoea and foul vaginal discharge with right cystic pelvic mass. She was diagnosed as a case of double uterus with obstructed right hemivagina and right pyocolpos with ipsilateral renal agenesis after routine ultrasonography in the clinic followed by MRI. Excision of the right vaginal septum with drainage of 200 ml of purulent discharge was performed. She was relieved of her symptoms and conceived promptly after the surgical excision of the partial vaginal septum.

  17. Agenesis and lipoma of corpus callosum: Case report Agenesia e lipoma de corpo caloso: relato de caso

    Directory of Open Access Journals (Sweden)

    Délrio Façanha Silva

    1995-09-01

    Full Text Available The agenesis and lipoma of the corpus callosum is a very rare association. We report the case of a 18-years old woman with rare epileptic seizures since the age of 6 years, normal neurological examination, as well as normal electroencephalogram. The brain computed tomography scanning and the magnetic resonance showed the lipoma and the agenesis of the corpus callosum.A agenesia e lipoma do corpo caloso é uma associação muito rara. Relatamos o caso de uma paciente de 18 anos com raras crises epilépticas desde os 6 anos de idade, exame neurológico normal, assim como eletrencefalograma normal. A tomografia computadorizada de crânio e a ressonância magnética mostraram o lipoma e a agenesia de corpo caloso.

  18. Subxiphoid single-incision thoracoscopic surgery for bilateral primary spontaneous pneumothorax

    OpenAIRE

    Liu, Chao-Yu; Lin, Chen-Sung; Liu, Chia-Chuan

    2015-01-01

    It has been reported that single-incision thoracoscopic surgery can reduce postoperative pain without compromising the main surgical steps required for treating patients affected by primary spontaneous pneumothorax. However, all the reported thoracoscopic surgery cases with a single-incision procedure were via the intercostal route for unilateral pulmonary lesions. We present a novel single-incision thoracoscopic technique via a subxiphoid route to perform one-stage bilateral thoracoscopic su...

  19. "Recurrent Bilateral Spontaneous Pneumothorax in Early Infancy: A Case of Langerhans Cell Histiocytosis"

    Directory of Open Access Journals (Sweden)

    S. Alavi

    2005-07-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disorder characterized by infiltration of either single or multiple organs by S100 and CD1a positive cells. Patients with pulmonary LCH are predisposed to pneumothorax due to destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH who presented at 2 months of age with simultaneous bilateral spontaneous pneumothorax.

  20.  Uterus Didelphys with Obstructed Right Hemivagina, Ipsilateral Renal Agenesis and Right Pyocolpos: A Case Report

    OpenAIRE

    Yasser A Razek; Ilham Hamdi; Hansa Dhar

    2011-01-01

     Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA Syndrome) is a rare congenital anomaly of the female genital tract. Uterus didelphys occurs when the midline fusion of the mullerian ducts is arrested, either completely or incompletely. Women with didelphic uterus may be asymptomatic and unaware of having a double uterus. They may present with complaints of dysmenorrhoea and dyspareunia. A 25 year old woman attending the infertility clinic at Nizwa regional r...

  1. Agenesis and Dysgenesis of the Corpus Callosum: Clinical, Genetic and Neuroimaging Findings in a Series of 41 Patients

    OpenAIRE

    Schell-Apacik, Chayim Can; Wagner, Kristina; Bihler, Moritz; Ertl-Wagner, Birgit; Heinrich, Uwe; Klopocki, Eva; Kalscheuer, Vera M.; Muenke, Maximilian; von Voss, Hubertus

    2008-01-01

    Agenesis of the corpus callosum (ACC) is among the most frequent human brain malformations with an incidence of 0.5–70 in 10,000. It is a heterogeneous condition, for which several different genetic causes are known, for example, ACC as part of monogenic syndromes or complex chromosomal rearrangements. We systematically evaluated the data of 172 patients with documented corpus callosum abnormalities in the records, and 23 patients with chromosomal rearrangements known to be associated with co...

  2. Pulmonary Hypertension

    OpenAIRE

    Newman, John H.

    2005-01-01

    The modern era in cardiopulmonary medicine began in the 1940s, when Cournand and Richards pioneered right-heart catheterization. Until that time, no direct measurement of central vascular pressure had been performed in humans. Right-heart catheterization ignited an explosion of insights into function and dysfunction of the pulmonary circulation, cardiac performance, ventilation–perfusion relationships, lung–heart interactions, valvular function, and congenital heart disease. It marked the beg...

  3. What Causes Pulmonary Hypertension?

    Science.gov (United States)

    ... from the NHLBI on Twitter. What Causes Pulmonary Hypertension? Pulmonary hypertension (PH) begins with inflammation and changes in the ... different types of PH. Group 1 pulmonary arterial hypertension (PAH) may have no known cause, or the ...

  4. Pulmonary Arterial Hypertension

    Science.gov (United States)

    Pulmonary Arterial Hypertension What Is Pulmonary Hypertension? To understand pulmonary hypertension (PH) it helps to understand how blood ows throughout your body. While the heart is one organ, it ...

  5. Pulmonary Fibrosis Foundation

    Science.gov (United States)

    ... the most current news and updates from the Pulmonary Fibrosis Foundation. Life with PF Education & Support About PF ... open ! JOIN NOW We Imagine a World Without Pulmonary Fibrosis The Pulmonary Fibrosis Foundation mobilizes people and resources ...

  6. Idiopathic Pulmonary Fibrosis

    Science.gov (United States)

    ... Explore Idiopathic Pulmonary Fibrosis What Is... How the Lungs Work Other Names Causes Signs & Symptoms Diagnosis Treatments Living With Clinical Trials Links Related Topics How the Lungs Work Lung Transplant Pulmonary Hypertension Pulmonary Rehabilitation Respiratory Failure ...

  7. Laparoscopic Bilateral Adrenalectomy in a patient of Cushing syndrome: A Challenge for the Anaesthesiologist

    Directory of Open Access Journals (Sweden)

    Uma K Dahanukar

    2007-01-01

    Full Text Available We present a case of Cushing syndrome who underwent laparoscopic bilateral adrenalectomy and discuss her intraoperative management and postoperative course in ICU, especially pulmonary oedema, that occurred within 3 hours after resection (half life of cortisol is 80-110 minutes. [1] She was diagnosed to have bilateral adrenal hyperplasia with no pituitary involvement on CT scan. Preoperative workup revealed hypokalemia, anaemia, hypertension and hyperglycemia. She was posted for laparoscopic bilateral adrenalectomy. She received general anaesthesia; we did not give epidural analgesia as the patient had fracture of body of L1 vertebrae. Her intra-operative course was uneventful. Post-operative concerns included acute adrenal insufficiency, hypoglycaemia, hypotension and hyperkalemia, which were successfully managed in ICU. Patient was then given oral corticosteroids. One month later she was reassessed and was in better health.

  8. Bilateral ECT induces bilateral increases in regional cortical thickness.

    Science.gov (United States)

    van Eijndhoven, P; Mulders, P; Kwekkeboom, L; van Oostrom, I; van Beek, M; Janzing, J; Schene, A; Tendolkar, I

    2016-01-01

    Electroconvulsive therapy (ECT) is the most effective treatment for patients suffering from severe or treatment-resistant major depressive disorder (MDD). Unfortunately its underlying neurobiological mechanisms are still unclear. One line of evidence indicates that the seizures produced by ECT induce or stimulate neuroplasticity effects. Although these seizures also affect the cortex, the effect of ECT on cortical thickness is not investigated until now. We acquired structural magnetic resonance imaging data in 19 treatment-resistant MDD patients before and after a bilateral ECT course, and 16 healthy controls at 2 time points, and compared changes in cortical thickness between the groups. Our results reveal that ECT induces significant, bilateral increases in cortical thickness, including the temporal pole, inferior and middle temporal cortex and the insula. The pattern of increased cortical thickness was predominant in regions that are associated with seizure onset in ECT. Post hoc analyses showed that the increase in thickness of the insular cortex was larger in responders than in non-responders, which may point to a specific relationship of this region with treatment effects of ECT. PMID:27552587

  9. Pulmonary hyalinizing granuloma mimicking multiple lung metastases: report of fluorodeoxyglucose positron emission findings.

    Science.gov (United States)

    Lien, Chi-Tun; Yang, Chih-Jen; Yang, Sheau-Fang; Chou, Shah-Hwa; Huang, Ming-Shyan

    2010-05-01

    Pulmonary hyalinizing granuloma (PHG) is a rare disease characterized by multiple bilateral pulmonary nodules of uncertain etiology. We describe a 71-year-old female patient with thyroid papillary carcinoma in whom bilateral pulmonary nodules were found during a routine chest radiography examination. Subsequent fluorodeoxyglucose positron emission tomography/computed tomography scan gave the impression of multiple pulmonary metastases based on high maximum standardized uptake value. She underwent video-assisted thoracoscopic surgery with wedge resection, and PHG was diagnosed on the basis of histopathologic findings. To our knowledge, this is the first report of PHG developing in a patient as a solid cancer, mimicking multiple pulmonary metastases. We also present the first description of positron emission tomography in PHG, according to a Medline search. PMID:20351580

  10. 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

  11. BILATERAL DUPLICATION OF RENAL ARTERIES

    Directory of Open Access Journals (Sweden)

    Prajkta A Thete

    2014-09-01

    Full Text Available Routine dissection of a male cadaver revealed the presence of bilateral double renal arteries. On the right side the accessory renal artery originated from the abdominal aorta just above the main renal artery. On the left side the accessory renal artery originated from the abdominal aorta about 1 cm above the main renal artery. Knowledge of the variations of renal vascular anatomy has importance in exploration and treatment of renal trauma, renal transplantation, renal artery embolization, surgery for abdominal aortic aneurysm and conservative or radical renal surgery

  12. Bilateral molariform mandibular second premolars.

    Science.gov (United States)

    Acharya, Sonu; Kumar Mandal, Pradip; Ghosh, Chiranjit

    2015-01-01

    Macrodontia is a rare dental anomaly that refers to teeth that appear larger than normal. Generalised macrodontia can be associated with certain medical conditions and syndromes. This case report presents clinical and radiographic findings of isolated bilateral macrodontia in a 14-year-old child. The patient was referred to the clinic with local crowding of maxillary and mandibular teeth. Radiographic findings revealed the presence of impacted macrodont mandibular second premolar on one side and erupted macrodontic premolar on the other side and their distinct morphological appearance, characterized by large, multitubercular, and molariform crowns and tapering, single roots. PMID:25685564

  13. How Is Pulmonary Hypertension Treated?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Pulmonary Hypertension Treated? Pulmonary hypertension (PH) has no cure. However, ... Types of Pulmonary Hypertension." ) Group 1 Pulmonary Arterial Hypertension Group 1 pulmonary arterial hypertension (PAH) includes PH ...

  14. Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia

    Directory of Open Access Journals (Sweden)

    Muhammad SAEED*

    2014-12-01

    Full Text Available How to Cite This Article: Saeed M, Haq A, Qadir Kh.Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia. Iran J Child Neurol. 2014 Autumn;8(4: 76-79.AbstractObjectiveBart’s syndrome is defined as congenital localized absence of skin, and associated with epidermolysis bullosa. A newborn with Bart’s syndrome is reported because it is a very rare condition, especially when associated with corpus callosum agenesis and concomitant choanal atresia. Clinically it is characterized by raw beefy areas of denuded skin mainly on hands and feet.We report a rare case of a term female newborn born to non-consanguineous parents who presented with congenital absence of skin in, face, trunk and extremities. To the best of our knowledge, this is the first report presenting a case of Bart’s syndrome associated with corpus callosum agenesis.ReferencesBart BJ, Garlin RJ, Anderson VE, Lynch FW. Congenital localized absence of skin and associated abnormalities resembling epidermolysis bullosa. A new syndrome. Arch Dermatol 1966; 93: 296-304.Bart BJ. Epidermolysis bullosa and congenital localized absence of skin. Arch Dermatol 1970; 101: 78-81.Skoven I, Drzewiecki KT. Congenital localized skin defect and epidermolysis bullosa hereditaria letalis. Acta Derm Venereol 1979; 59: 533-537.Wojnarowska FT, Eady RA, Wells RS. Dystrophic epidermolysis bullosa presenting with congenital localized absence of skin: report of four cases. Br J Dermatol 1983; 108: 477-483.Kanzler MH, Smoller B, Woodley DT. Congenital localized absence of the skin as a manifestation of epidermolysis bullosa. Arch Dermatol 1992; 128:1087-90.Maman E, Maor E, Kachko L, Carmi R. Epidermolysis bullosa, pyloric atresia, aplasia cutis congenita: histopathological delineation of an autosomal recessive disease. Am J Med Genet 1998; 78: 127-133.McCarthy MA, Clarke T, Powell FC. Epidermolysis bullosa and aplasia cutis. Int J Derm 1991; 30: 481-484.Puvabanditsin S, Garrow E, Daeun K

  15. Bilateral Monteggia fracture in adults

    Directory of Open Access Journals (Sweden)

    Ristić Dejan

    2011-01-01

    Full Text Available Introduction. In 1814 Giovanni Monteggia first described two cases of fractures of the proximal third of ulna with dislocation of the radial head. These fractures are more common in children than in adults, and mutual Monteggia fracture is a rare complication. This study presents a treatment course of a patient with bilateral Monteggia fracture. Case report. A 55- year-old patient was injured by falling in the yard. Radiography showed bilateral Monteggia fracture type II (by the Badon classification. Operative treatment of fracture was done by a compression plate on the right side and by the zuggurtung technique on the left one. Closed repositioning of the radial head was done on both sides. The patient was wearing a plaster splint for the upper arm for 21 days. After removing the fixation, the function of the elbow was determined by the Broberg Morrey score (BM which was on the right side 45.5 and on the left side 47.5. After the proper physical therapy, four months after the surgery, BM score was 100 on the right side, and 93 on the left one. Conclusion. Surgical treatment and early rehabilitation is the key for the return of good function of both elbows.

  16. [Simultaneous bilateral pneumothorax. Case report].

    Science.gov (United States)

    Paolini, A; Caminiti, F; Tosato, F; Ruggieri, M; Paolini, G; Carnevale, L; Corsini, F; Marano, S; Monsellato, I

    2001-04-01

    A case report of a 44 year-old white man admitted to the surgical unit for a bilateral simultaneous pneumothorax is presented. The pneumothorax occurred on day one after a surgical operation for discal hernia; in the past the patient already presented a right spontaneous pneumothorax at 32 years of age and a left pneumothorax at 37 years of age, both treated with a pleural drainage. A thoracic drain was bilaterally positioned with a good result only in the right side. The persistence of the left pneumothorax induced the authors to perform a postero-lateral thoracotomy bullae excision and pleurectomy with a good postoperative course. After a few months a new right pneumothorax occurred and the patient was treated with a right postero-lateral thoracotomy, bullae resection and pleurectomy. On the basis of the case reported, the authors consider the different opportunities in the treatment of spontaneous pneumothorax in relation to the present knowledges and technologies. Surgical procedure is to be preferred in case of persistence of pneumothorax despite a pleural drain and in case of pneumothorax in high risk subjects. Even if thoracoscopy seems to give better results regarding postoperative pain, it is not always possible with such a method to perform a careful pleurectomy neither to obtain it in all cases (above all in secondary pneumothorax). Every case must then be carefully studied to choose the best treatment at present available. PMID:11353349

  17. Danish Exports and Danish Bilateral Aid

    DEFF Research Database (Denmark)

    Hansen, Henrik; Rand, John

    Danish bilateral development assistance is aimed at reducing poverty in the partner countries. Even so, bilateral assistance may have secondary, or knock-on, effects, which are beneficial for Denmark. An important secondary effect is the prospect of increased export from Denmark to the partner...... countries. This Evaluation Study presents an econometric analysis of Danish exports to 144 countries over the period from 1981 to 2010. The analysis is based on the gravity model of bilateral trade; a structural model developed over decades and now the central model in analyses of bilateral trade flows...... and trade policies. The main result of the study is that Danish bilateral aid has a positive and statistically significant impact on Danish exports to the recipient countries. Bilateral development assistance may affect exports through several channels. Three of the main channels are direct aid tying...

  18. Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis

    OpenAIRE

    Matthias Held; Philipp Schnabel; Arne Warth; Berthold Jany

    2012-01-01

    Introduction. Pulmonary Langerhans cell granulomatosis is a rare disease with a variable course. In pulmonary Langerhans cell granulomatosis pulmonary hypertension is frequent and has an independent prognostic impact. A vasculopathy which ist not related to ventilatory disturbance and fibrosis has been identified. An arteriopathy and even a venulopathy have been described. Due to this possible venulopathy vasodilators carry a significant risk for pulmonary congestion and edema. No drugs have ...

  19. [Pulmonary hyalinizing granuloma mimicking pulmonary carcinoma].

    Science.gov (United States)

    Uçvet, Ahmet; Tözüm, Halil; Gürsoy, Soner; Gülle, Ali Alper; Yaldiz, Sadik; Aydoğdu Dinç, Zekiye

    2006-01-01

    Pulmonary hyalinizing granuloma is a rare fibrosing nodular disease of the lung characterized by solitary or multiple pulmonary nodules. They can occur after inflammatory or post-inflammatory changes. A 60 years old asymptomatic patient admitted to our clinic because of a solid mass of 6 cm in his routine chest radiography. A lobectomy was performed and the histological diagnosis was reported as pulmonary hyalinizing granuloma. This case, mimicking pulmonary carcinoma, is rarely found in the literature. PMID:16615022

  20. [Pulmonary embolism].

    Science.gov (United States)

    Söffker, Gerold; Kluge, Stefan

    2015-01-01

    Acute pulmonary embolism is an important differential diagnosis of acute chest pain. The clinical signs are often non-specific. However, diagnosis and therapy must be done quickly in order to reduce morbidity and mortality. The new (2014) European guidelines for acute pulmonary embolism (PE) focus on risk-adapted diagnostic algorithms and prognosis adapted therapy concepts. According to the hemodynamic presentation the division in a high-risk group (unstable patient with persistent hypotension or shock) or in non-high-risk groups (hemodynamically stable) was proposed. In the high-risk group the immediate diagnosis is usually done by multidetector spiral computed tomography (MDCT) and primarily the medical therapy of right ventricular dysfunction and thrombolysis is recommended.In the non-high-risk group, this is subdivided into an intermediate-risk group and low-risk group, the diagnosis algorithm based on the PE-pretest probability--determined by validated scores. Moreover, the diagnosis is usually secured by MDCT--the new gold standard in the PE-diagnosis, scores, or it can be primarily ruled out due to the high negative predictive value of D-dimer determination. To improve the prognostic risk stratification in non-high-risk group patients the additional detection of right ventricular dysfunction (MDCT, echocardiography), cardiac biomarkers (troponin, NT proBNP) and validated scores (e.g. Pulmonary Embolism Severity Index) is recommended. Therefore, the intermediate-risk group can be further subdivided. For treatment of non-high-risk group patients, the initial anticoagulation (except those with severe renal insufficiency) using low molecular weight heparin/fondaparinux and conversion to vitamin-K antagonists or alternatively with direct oral anticoagulants (DOAK) is recommended. Hemodynamically stable patients with right ventricular dysfunction and myocardial ischemia (Intermediate-high-risk group patients) but with clinically progressive hemodynamic

  1. Drug-induced pulmonary injury: CT findings in hemopathic patients

    International Nuclear Information System (INIS)

    Objective: To investigate the spiral CT findings in hemopathic patients with drug-induced pulmonary injury. Methods: CT images obtained in 11 patients with drug-induced pulmonary injury were retrospectively analyzed. Six patients had antineoplastic agent-induced pulmonary injury and 5 patients had non-neoplastic agent-induced pulmonary injury (immunosuppressor in 2 patients, antifungal in 2 patients, antineoplastic immunomodulators in 1 patient). CT findings were reviewed by a chest radiologist. Results: All 11 patients had parenchymal abnormalities on MSCT scans, including ground-glass opacities (n=8), consolidation (n=5),interlobular septal thickening (n=3) and focal fibrosis (n=2). The abnormalities were bilateral and asymmetric in all patients. They were mainly in the peripheral lung regions in 6 patients, in the central lung regions in four, and irregularly located in one. The abnormalities involved mainly the lower lung zones in six patients, the upper lung zones in two,and all lung zones homogeneously in three. One patient had fluid in bilateral pleural cavities. Three patients were given the same agent once more after the imaging turned to normal, and they presented with same clinical symptoms and similar but more serious imaging findings. Conclusions: Drug-induced pulmonary injury usually manifests as areas of ground-glass opacity and consolidation, which most commonly involves the peripheral lungs and lower lung zones. Drug-induced pulmonary injury shows reproducible but more serious lesions when the patient is given the same agent once more. (authors)

  2. New Curious Bilateral q-Series Identities

    Directory of Open Access Journals (Sweden)

    Frédéric Jouhet

    2012-10-01

    Full Text Available By applying a classical method, already employed by Cauchy, to a terminating curious summation by one of the authors, a new curious bilateral q-series identity is derived. We also apply the same method to a quadratic summation by Gessel and Stanton, and to a cubic summation by Gasper, respectively, to derive a bilateral quadratic and a bilateral cubic summation formula.

  3. Bilateral Clavicle Fracture in Two Newborn Infants

    Directory of Open Access Journals (Sweden)

    Esra Arun Ozer

    2011-12-01

    Full Text Available Background: The fracture of clavicle is the most frequently observed bone fracture as birth trauma and it is usually unilateral. It is seen following shoulder dystocia deliveries or breech presentation of macrosomic newborns.Case Presentation: We report two macrosomic newborns with bilateral clavicle fracture and brachial plexus palsy due to birth trauma. Chest X-rays confirmed bilateral fracture of clavicles. Both patients were recovered without any sequel.Conclusion: Bilateral clavicular fracture should be considered in any neonate with bilateral absent Moro reflexes.

  4. A Case of a Newborn with Agenesis of the Corpus Callosum Complicated with Ocular Albinism

    Directory of Open Access Journals (Sweden)

    Michiko Miki

    2016-05-01

    Full Text Available Purpose: To report a case of ocular albinism found in a newborn infant in whom agenesis of the corpus callosum (ACC was indicated in utero. Case Report: This study involved a female newborn who was delivered after a gestational period of 41 weeks. The patient was referred to the Obstetrics Department at Takatsuki Hospital, Takatsuki City, Japan, after the indication of ACC by magnetic resonance imaging (MRI at a nearby clinic during the fetal period. At birth, the baby’s weight was 2,590 g, and ACC and ventricular enlargement were found by cranial sonography and cranial MRI. While initial ophthalmic findings noted partial loss of pigmentation of the iris and hypopigmentation of broad areas of the fundus in both eyes, nystagmus was not observed. The patient’s hair pigment was slightly diluted, and the color of her skin was slightly off-white. At 2 years after birth, obvious mental retardation was observed. With regard to other systemic findings, no apparent heart, kidney, or immune system abnormalities were found. Conclusion: Although the patient in question is presently growing without any major systemic problems, it will be necessary in the future to pay attention to any changes in systemic and ophthalmic findings.

  5. Is Disturbed Transfer of Learning in Callosal Agenesis due to a Disconnection Syndrome?

    Directory of Open Access Journals (Sweden)

    T. Imamura

    1994-01-01

    Full Text Available Disturbed intermanual transfer of tactile learning in callosal agenesis has been interpreted as a sign of disconnection syndrome. We observed this sign in one of four acallosal patients with a conventional form-board task, and tried to elucidate the nature of the deficit. The form-board performance of the patient with disturbed transfer of learning totally depended on motor skill, while the other acallosals and normal controls executed the task based on spatial and somesthetic information. All acallosals and normals, however, failed to show transfer of learning with another tactile task which needed motor skill but not spatial-somesthetic information. These findings suggest that the task-performing strategies in form-board learning change the state of interhemispheric transfer. Unimanual learning effect is transferred if spatial-somesthetic information is acquired in the process of learning, but is not transferred if motor skill is the exclusive content of learning. We conclude that disturbed “transfer” of learning in some acallosals is not a true disconnection sign. It should be attributed to a lack of appropriate strategy, as a result of ineffective problem solving in tactile tasks.

  6. A Case of a Newborn with Agenesis of the Corpus Callosum Complicated with Ocular Albinism

    Science.gov (United States)

    Miki, Michiko; Miyamoto, Makiko; Mitsutsuji, Tatsuma; Watanabe, Hiroko; Shimizu, Kazuhiro; Matsuo, Junko; Tonari, Masahiro; Kida, Teruyo; Sugasawa, Jun; Ikeda, Tsunehiko

    2016-01-01

    Purpose To report a case of ocular albinism found in a newborn infant in whom agenesis of the corpus callosum (ACC) was indicated in utero. Case Report This study involved a female newborn who was delivered after a gestational period of 41 weeks. The patient was referred to the Obstetrics Department at Takatsuki Hospital, Takatsuki City, Japan, after the indication of ACC by magnetic resonance imaging (MRI) at a nearby clinic during the fetal period. At birth, the baby's weight was 2,590 g, and ACC and ventricular enlargement were found by cranial sonography and cranial MRI. While initial ophthalmic findings noted partial loss of pigmentation of the iris and hypopigmentation of broad areas of the fundus in both eyes, nystagmus was not observed. The patient's hair pigment was slightly diluted, and the color of her skin was slightly off-white. At 2 years after birth, obvious mental retardation was observed. With regard to other systemic findings, no apparent heart, kidney, or immune system abnormalities were found. Conclusion Although the patient in question is presently growing without any major systemic problems, it will be necessary in the future to pay attention to any changes in systemic and ophthalmic findings. PMID:27462254

  7. Compression of the Right Pulmonary Artery by a Massive Defects on Pulmonary Scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Makis, William [Brandon Regional Health Centre, Brandon (Canada); Derbekyan, Vilma [McGill Univ. Health Centre, Montreal (Canada)

    2012-03-15

    A 67 year old woman, who presented with a 2 month history of dyspnea, had a vectilation and perfusion lung scan that showed absent perfusion of the entire right lung scan that showed absent perfusion of the entire right lung with normal ventilation, as well as a rounded matched defect in the left lower lung adjacent to mialine, suspicious for an aortic aneurysm or dissection. CT pulmonary angiography revealed a massive descending aortic aneurysm compressing the right pulmonary artery as well as the left lung parenchyma, accounting for the bilateral perfusion scan defects. We present the Xe 133 ventilation, Tc 99m MAA perfusion and CT pulmonary angiography imaging findings of this rare case.

  8. A 40-year-old woman with multiple pulmonary nodules. Pulmonary hyalinizing granuloma.

    Science.gov (United States)

    Ussavarungsi, Kamonpun; Khoor, Andras; Jolles, Howard I; Mira-Avendano, Isabel

    2014-12-01

    A 40-year-old woman (a nonsmoker) with history of idiopathic thrombocytopenic purpura and a platelet count > 90,000 cells/μL without specific medication was referred to pulmonary clinic for evaluation of multiple pulmonary nodules. The patient presented to an outside hospital with fatigue, lack of energy, and dyspnea on exertion for 2 years. She denied fever, cough, chest pain, or weight loss. An initial chest radiograph showed bilateral multiple pulmonary nodules. A chest CT scan revealed multiple nodular lesions, varying in size, in all lobes of both lungs. There was no mediastinal lymphadenopathy or pleural effusion. There was no significant hypermetabolic activity on a subsequent fluorodeoxyglucose PET scan/CT scan, and there had been no significant change. She underwent CT scan-guided percutaneous transthoracic biopsy and bronchoscopy with transbronchial biopsies, all of which were inconclusive. An open lung biopsy was considered. PMID:25451362

  9. BILATERAL VESICO-URETERAL REFLUX IN PATIENT WITH CROSSED RENAL ECTOPIA AND FUSION TYPE A

    Directory of Open Access Journals (Sweden)

    A.L Bulotta

    2012-05-01

    Full Text Available Introduction. Crossed renal ectopia with fusion is a very rare congenital anomaly and the reported incidence varies between 1:1000 and 1:7000. The kidney is located on the opposite site of the mid- line from where the ureter enters the bladder. Eighty-five percent of crossed renal ectopia kid- neys are fused from below to the normally located kidney. This anomaly is more frequent for left kidney and it’s associated with vesico-ureteral re- flux (VUR in 25-70% of cases. We report the management of a six-years-old patient with Pluri- malformative Syndrome, trisomy p16 and mono- somy q2, crossed renal ectopia with fusion type A and bilateral vesico-ureteral reflux (grade IV in the right kidney and grade III in the left.Materials and Methods. A 6-year-old boy was ammitted to our hospital for UTI in plurimalfor- mative syndrome characterized at birth by cleft palate, macrocephaly, congenital clubfeet, twisted right arm, congenital dysplasia of the hip, balanic hypospadias, bilateral inguinal hernia, right renal agenesis and epilepsy tonic-clonic. MRI revealed a fusion of the ectopic kidney with the left ortho- topic kidney (crossed renal ectopia with fusion type A. Voiding cystography showed a dilatated ureter of the crossed ectopic kidney passing across the midline and of the left ureter, and a bilateral vesico-ureteral reflux ( grade IV VUR in the right kidney and grade III VUR in the left. For this reason bilaterally endoscopic subureteral infiltra- tion was performed with Deflux ( 0.3 cc for side. Results. Patient was discharged in third day and he took antibiotic for one week. There weren’t complications like fever, obstruction or UTI. Fol- low-up after 1 month is normal and there weren’t UTI. Conclusion. Generally the outcome of patients with fused crossed renal ectopia is good. Presence of associated pathology likeVUR, could lead to a progressive deterioration of renal function. There- fore, in patient with uninhabited kidney area and

  10. Medical image of the week: bilateral atrial appendange thrombi

    Directory of Open Access Journals (Sweden)

    Ateeli H

    2015-01-01

    Full Text Available No abstract available. Article truncated at 150 words. A 63-year-old man with a past history significant for hypertension, low back pain and polysubstance abuse (tobacco and marijuana presented with shortness of breath and hemoptysis for the last 8 days prior to admission. His initial exam showed elevated jugular venous pressure and bilateral basal crackles with reduced air entry on the right lower lung zone. The patient was found to be in atrial fibrillation with a rapid ventricular response. His initial chest X-ray showed a moderate right-sided pleural effusion. Immediate bedside echo was concerning for bilateral ventricular dysfunction with concerns of right-sided heart pressure and volume overload. A chest CT angiogram was obtained and showed acute lower lobe pulmonary embolism, with possible distal infarct, moderate right sided pleural effusion, and filling defects in both atrial appendages concerning for thrombi (Figure 1, Panels A & B. The patient was started on therapeutic anticoagulation and underwent therapeutic thoracentesis, gentle diuresis, and ...

  11. Bilateral metachronous breast cancer with bilateral recurrences: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Park, So Hyun; Sohn, Yu Mee [Dept. of Radiology, Kyung Hee University Hospital, College of Medicine, Kyung Hee University, Seoul (Korea, Republic of); Kim, Eun Kyung [Dept. of Radiology, Research Institute of Radiological Science, Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2014-05-15

    The incidence of bilateral breast cancer has been reported to range from 0.4% to 14%, and it increases gradually as a result of improved early detection capabilities and longer survival times. We report a rare case where the bilateral breast cancers occurred as a metachronous bilateral breast cancer with bilateral recurrences, detected by mammography, and the rapid growth of tumor that manifested as microcalcification and skin thickening within 3 months.

  12. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures

    Directory of Open Access Journals (Sweden)

    Gultekin Gulbahar

    2015-01-01

    Full Text Available First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity.

  13. Bilateral fracture in atrophic mandible

    Directory of Open Access Journals (Sweden)

    Luciana SIGNORINI

    2006-11-01

    Full Text Available The increase of the elderly population in Brazil has brought a higher number of patients of this age level for dental treatment in several areas of dentistry.Elderly patients are exposed to many kinds of aggressor agents, such as falling down and accidents that cause maxillofacial fractures, which require the specialized assistance of a maxillofacial surgeon. The elderly present a differentiated condition involving systemic changes and use of several drugs that must be taken into consideration when planning the therapeutics ofeach case. The treatment of facial trauma must aim fast recovery along with minimal morbidity. The authors present a case of mandible bilateral fracture in edentulous elderly patient with severe atrophy, treated with titanium plate and screws osteosynthesis. The healing period was satisfactory, without postoperative complications in 18 months follow up.

  14. [Pulmonary circulation in embolic pulmonary edema].

    Science.gov (United States)

    Sanotskaia, N V; Polikarpov, V V; Matsievskiĭ, D D

    1989-02-01

    The ultrasonic method was used in acute experiments on cats with open chest under artificial lung ventilation to obtain blood flow in low-lobar pulmonary artery and vein, the blood pressure in pulmonary artery, as well as the left atrial pressure in fat (olive oil) and mechanical (Lycopodium spores) pulmonary embolism. It is shown that pulmonary embolism produces the decrease in the blood flow in pulmonary artery and vein, the increase of the pressure in pulmonary artery and left atria, the increase of lung vessels resistance. The decrease is observed of systemic arterial pressure, bradycardia, and extrasystole. After 5-10 min the restoration of arterial pressure and heart rhythm occur and partial restoration of blood flow in pulmonary artery and vein. In many experiments the blood flow in vein outdoes that in the artery--it allows to suppose the increase of the blood flow in bronchial artery. After 60-90 min there occur sudden decrease of systemic arterial pressure, the decrease of the blood flow in pulmonary artery and vein. The pressure in pulmonary artery and resistance of pulmonary vessels remain high. Pulmonary edema developed in all animals. The death occurs in 60-100 min after the beginning of embolism. PMID:2923969

  15. Apelin and pulmonary hypertension

    DEFF Research Database (Denmark)

    Andersen, Charlotte Uggerhøj; Hilberg, Ole; Mellemkjær, Søren;

    2011-01-01

    Pulmonary arterial hypertension (PAH) is a devastating disease characterized by pulmonary vasoconstriction, pulmonary arterial remodeling, abnormal angiogenesis and impaired right ventricular function. Despite progress in pharmacological therapy, there is still no cure for PAH. The peptide apelin...... vasoconstriction, and has positive inotropic and cardioprotective effects. Apelin attenuates vasoconstriction in isolated rat pulmonary arteries, and chronic treatment with apelin attenuates the development of pulmonary hypertension in animal models. The existing literature thus renders APLNR an interesting...

  16. Bilateral failure of adduction following orbital decompression.

    OpenAIRE

    Kinsella, F; Kyle, P.; Stansfield, A

    1990-01-01

    We report a case of bilateral complete failure of adduction following bilateral translid antralethmoidal orbital decompression. We believe the probable mechanism is neuropraxia (temporary dysfunction) of the third cranial nerves' supply to the medial recti, owing to these nerves' occupying an anatomically abnormal position. Partial recovery of adduction occurred over the ensuing six months.

  17. Secondary polycythaemia associated with bilateral renal lymphocoeles.

    OpenAIRE

    Burton, I E; Sambrook, P.; McWilliam, L J

    1994-01-01

    A patient with a 15 year history of secondary polycythaemia due to renal erythropoietin hypersecretion is presented. Subsequent spontaneous development of bilateral renal lymphocoeles, which contained high erythropoietin levels, was shown by computerized tomography. The lymphocoeles were successfully treated by bilateral peritoneal marsupialization. No cause for the persistent polycythaemia or lymphocoeles was found at laparotomy or on renal biopsy.

  18. Simultaneous and spontaneous bilateral quadriceps tendons rupture.

    Science.gov (United States)

    Celik, Evrim Coşkun; Ozbaydar, Mehmet; Ofluoglu, Demet; Demircay, Emre

    2012-07-01

    Simultaneous and spontaneous bilateral quadriceps tendon rupture is an uncommon injury that is usually seen in association with multiple medical conditions and some medications. We report a case of simultaneous and spontaneous bilateral quadriceps tendon rupture that may be related to the long-term use of a statin.

  19. Bilateral spontaneous adrenal haemorrhage complicating acute pancreatitis

    International Nuclear Information System (INIS)

    Bilateral adrenal haemorrhage is an event that mandates prompt diagnosis and treatment to prevent primary adrenocortical insufficiency and potential death. Presentation can be non-specific and incidentally diagnosed with imaging alone, primarily CT. We present a case of acute pancreatitis with spontaneous bilateral adrenal haemorrhage and briefly discuss imaging and treatment implications

  20. Bilateral breast involvement in acute myeloid leukemia

    Directory of Open Access Journals (Sweden)

    Hakeem A, Mandakini BT, Asif K, Firdaus, Shagufta RC

    2013-04-01

    Full Text Available Breast involvement by leukemic infiltration is usually bilateral, but may be unilateral. Clinically patients can present with either single or multiple masses, or with diffuse breast engorgement, with or without nodularity. The affected patients are predominantly young adults. We present a case of an adolescent girl with acute myeloid leukemia having bilateral breast infiltration by leukemic cells.

  1. Concurrent bilateral ectopic pregnancy: a rarity

    Directory of Open Access Journals (Sweden)

    Prabhleen Kaur

    2015-08-01

    Full Text Available Bilateral ectopic pregnancy is a rare twin gestation with only a few cases reported in the literature. We report a 30 year old woman without any high risk factor for ectopic pregnancy, who had concurrent bilateral ectopic pregnancy. A 30 year old female presented to the Gynecology emergency department complaining of vaginal bleeding and abdominal pain. The presumptive diagnosis of ruptured left sided ectopic pregnancy was made on basis of clinical findings and ultrasound finings. An emergency laparotomy was done revealed a hemoperitoneum of 1.5 liters, a ruptured left tubal pregnancy with active bleeding and right tubal un-ruptured ectopic was found. A bilateral salpingectomy was performed. Histopathology confirmed presence of chorionic villi in both tubes. In theory, laparoscopic salpingostomy is the best surgical approach in bilateral tubal pregnancy. However, bilateral salpingectomy may be necessary when both tubes are extensively damaged or are actively bleeding. Successful pregnancies have been reported after conservative surgical treatment of bilateral ectopic, but the risk of recurrence is high. Our decision for an emergency laparotomy followed by bilateral salpingectomy was based on the fact that the patient presented with acute abdomen and was haemodynamically unstable and there was extensive bilateral tubal damage. As the incidence of ectopic pregnancies is increasing concurrently with the incidences of pelvic inflammatory disease and use of assisted fertility techniques; it may be that these and ldquo;rare ectopics and rdquo; will become less uncommon. [Int J Reprod Contracept Obstet Gynecol 2015; 4(4.000: 1197-1199

  2. Acral osteolysis in bilateral compartment syndrome

    Directory of Open Access Journals (Sweden)

    Iram Saeed

    2008-08-01

    Full Text Available Carpal tunnel syndrome is a common neurological condition with rare yet potentially serious cutaneous and skeletal complications. We present a case of mutilating/ulcerating bilateral carpal tunnel syndrome in a 63 year old female. Radiographs showed symmetrical acral osteolysis in the index and middle fingers distal phalanges bilaterally. Carpal tunnel decompressions provided symptomatic relief.

  3. Simultaneous and spontaneous bilateral quadriceps tendons rupture.

    Science.gov (United States)

    Celik, Evrim Coşkun; Ozbaydar, Mehmet; Ofluoglu, Demet; Demircay, Emre

    2012-07-01

    Simultaneous and spontaneous bilateral quadriceps tendon rupture is an uncommon injury that is usually seen in association with multiple medical conditions and some medications. We report a case of simultaneous and spontaneous bilateral quadriceps tendon rupture that may be related to the long-term use of a statin. PMID:22561379

  4. Pulmonary veno-occlusive disease

    Science.gov (United States)

    Pulmonary vaso-occlusive disease ... common among children and young adults. As the disease gets worse, it causes narrowed pulmonary veins, pulmonary artery hypertension , and congestion and swelling ...

  5. Pulmonary disease in patients with human immunodeficiency virus infection

    DEFF Research Database (Denmark)

    Lundgren, J D; Orholm, Marianne; Lundgren, B;

    1989-01-01

    Pulmonary disease is the most important cause of morbidity and mortality in patients infected with human immunodeficiency virus (HIV). All parts of the hospital system are expected to be involved in the diagnosis and treatment of HIV infected patients in the coming years. Many different processes...... cause pulmonary disease alone or in combination. Bilateral interstitial infiltrates are the most frequent chest x-ray abnormality and are most frequently caused by infection with Pneumocystis carinii. Cytomegalovirus, Mycobacterium tuberculosis, nonspecific interstitial pneumonitis and pulmonary Kaposi......'s sarcoma are the most important parts of the differential diagnosis. An aggressive approach to the diagnosis of pulmonary disease in this patient population is indicated in order to provide optimal care and assess new therapies....

  6. The Guided Bilateral Filter: When the Joint/Cross Bilateral Filter Becomes Robust

    OpenAIRE

    Caraffa, Laurent; Tarel, Jean Philippe; Charbonnier, Pierre

    2015-01-01

    The bilateral filter and its variants such as the Joint/Cross bilateral filter are well known edge-preserving image smoothing tools used in many applications. The reason of this success is its simple definition and the possibility of many adaptations. The bilateral filter is known to be related to robust estimation. This link is lost by the ad hoc introduction of the guide image in the Joint/Cross bilateral filter. We here propose a new way to derive the Joint/Cross bilateral filter as a part...

  7. Novel PAX9 and COL1A2 missense mutations causing tooth agenesis and OI/DGI without skeletal abnormalities.

    Directory of Open Access Journals (Sweden)

    Shih-Kai Wang

    Full Text Available Inherited dentin defects are classified into three types of dentinogenesis imperfecta (DGI and two types of dentin dysplasia (DD. The genetic etiology of DD-I is unknown. Defects in dentin sialophosphoprotein (DSPP cause DD type II and DGI types II and III. DGI type I is the oral manifestation of osteogenesis imperfecta (OI, a systemic disease typically caused by defects in COL1A1 or COL1A2. Mutations in MSX1, PAX9, AXIN2, EDA and WNT10A can cause non-syndromic familial tooth agenesis. In this study a simplex pattern of clinical dentinogenesis imperfecta juxtaposed with a dominant pattern of hypodontia (mild tooth agenesis was evaluated, and available family members were recruited. Mutational analyses of the candidate genes for DGI and hypodontia were performed and the results validated. A spontaneous novel mutation in COL1A2 (c.1171G>A; p.Gly391Ser causing only dentin defects and a novel mutation in PAX9 (c.43T>A; p.Phe15Ile causing hypodontia were identified and correlated with the phenotypic presentations in the family. Bone radiographs of the proband's dominant leg and foot were within normal limits. We conclude that when no DSPP mutation is identified in clinically determined isolated DGI cases, COL1A1 and COL1A2 should be considered as candidate genes. PAX9 mutation p.Phe15Ile within the N-terminal β-hairpin structure of the PAX9 paired domain causes tooth agenesis.

  8. An Unusual Case of Recurrent Pyocolpos Following Midtrimester Miscarriage Revealed as Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA Syndrome

    Directory of Open Access Journals (Sweden)

    Haldar M

    2014-08-01

    Full Text Available Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA Syndrome is a rare congenital anomaly. It mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos. Sometime it presents in unusual way with prolonged vaginal bleeding and profuse vaginal discharge. Here we report case of an 18 years old married female with OHVIRA syndrome presenting late with symptoms of recurrent pyocolpos following dilatation and evacuation for midtrimester miscarriage that was diagnosed on the basis of MRI and managed by vaginal septotomy.

  9. Agenesis of the posterior arch of the atlas Agenesia do arco posterior do atlas

    Directory of Open Access Journals (Sweden)

    Martin Torriani

    2002-01-01

    Full Text Available PURPOSE: To illustrate the radiological findings and review the current literature concerning a rare congenital abnormality of the posterior arch of the atlas. CASE REPORT: An adult female without neurological symptoms presented with an absent posterior arch of the atlas, examined with plain films and helical computerized tomography. Complete agenesis of the posterior arch of the atlas is a rare entity that can be easily identified by means of plain films. Although it is generally asymptomatic, atlantoaxial instability and neurological deficits may occur because of structural instability. Computerized tomography provides a means of assessing the extent of this abnormality and can help evaluate the integrity of neural structures. Although considered to be rare entities, defects of the posterior arch of the atlas may be discovered as incidental asymptomatic findings in routine cervical radiographs. Familiarity with this abnormality may aid medical professionals in the correct management of these cases.OBJETIVO: Descrever os achados radiológicos e revisar a literatura de uma anormalidade congênita rara do arco posterior do atlas. RELATO DE CASO: Uma paciente adulta sem queixas neurológicas apresentava ausência do arco posterior do atlas, examinada através de radiografias simples e tomografia computadorizada helicoidal. A agenesia completa do arco posterior do atlas é uma entidade rara que pode ser facilmente identificada através de radiografias simples. Apesar de ser geralmente assintomática, instabilidade atlanto-axial e queixas neurológicas podem ocorrer devido à instabilidade estrutural. A tomografia computadorizada possibilita a avaliação da extensão desta anormalidade e pode auxiliar na análise da integridade de estruturas neurais. Apesar de serem considerados entidades raras, os defeitos do arco posterior do atlas podem ser achados incidentais em radiografias cervicais de rotina. A familiaridade com esta anormalidade pode

  10. Learning and memory in individuals with agenesis of the corpus callosum.

    Science.gov (United States)

    Paul, Lynn K; Erickson, Roger L; Hartman, Jo Ann; Brown, Warren S

    2016-06-01

    Damage to long white matter pathways in the cerebral cortex is known to affect memory capacity. However, the specific contribution of interhemispheric connectivity in memory functioning is only beginning to become understood. The present study examined verbal and visual memory processing in individuals with agenesis of the corpus callosum (AgCC) using the Wechsler Memory Scale-Third Edition (WMS-III; Wechsler, 1997b). Thirty participants with AgCC (FSIQ >78) were compared against 30 healthy age and IQ matched controls on auditory/verbal (Logical Memory, Verbal Paired Associates) and visual (Visual Reproduction, Faces) memory subtests. Performance was worse in AgCC than controls on immediate and delayed verbal recall for rote word pairs and on delayed recall of faces, as well as on percent recall for these tasks. Immediate recall for thematic information from stories was also worse in AgCC, but groups did not differ on memory for details from narratives or on recall for thematic information following a time delay. Groups also did not differ on memory for abstract figures or immediate recall of faces. On all subtests, individuals with AgCC had greater frequency of clinically significant impairments than predicted by the normal distribution. Results suggest less efficient overall verbal and visual learning and memory with relative weaknesses processing verbal pairs and delayed recall for faces. These findings suggest that the corpus callosum facilitates more efficient learning and recall for both verbal and visual information, that individuals with AgCC may benefit from receiving verbal information within semantic context, and that known deficits in facial processing in individuals with AgCC may contribute to their impairments in recall for faces. PMID:27091586

  11. [Differential diagnosis of pulmonary tuberculosis and community-acquired pneumonia].

    Science.gov (United States)

    Deĭkina, O N; Mishin, V Iu; Demikhova, O V

    2007-01-01

    The purpose of this investigation was to enhance the efficiency of differential diagnosis of pneumonia and pulmonary tuberculosis. A hundred and fifty-nine adult patients were examined. These included 78 patients with pulmonary tuberculosis and 81 with community-acquired p neumonia. The clinical features of infiltrative pulmonary tuberculosis (n = 48) and mild community-acquired pneumonia (n = 51) were compared. The course of caseous pneumonia (n = 30) was compared with that of moderate and severe community-acquired pneumonia (n = 30). Significant differences in the manifestations of the intoxication and bronchopulmonary syndrome were not found in patients with community-acquired pneumonia and infiltrative pulmonary tuberculosis. Physical studies showed that in patients with community-acquired pneumonia, moist rale (54.9%) and crepitation (11.8%) were prevalent, but in those with infiltrative tuberculosis rale was absent in 60.4% of cases and the pattern of respiration was unchanged in 79.2%. Chest X-ray studies indicated that in patients with community-acquired pneumonia, lower lobar inflammatory changes were predominant in 62.8% of cases whereas in those with infiltrative pulmonary tuberculosis the process was mainly bilateral (43.8%) with the presence of destructive changes (83.3%) and bronchogenic dissemination (66.7%). In patients with caseous pneumonia, the intoxication syndrome was more significant than in those with severe community-acquired pneumonia. Chest X-ray studies demonstrated that in patients with caseous pneumonia, specific changes were bilateral with the involvement of 2 lobes or more, with destruction and bronchogenic dissemination while in those with community-acquired pneumonia, the pulmonary processes were predominantly bilateral (76.6%) at the lower lobar site (36.7%).

  12. [Differential diagnosis of pulmonary tuberculosis and community-acquired pneumonia].

    Science.gov (United States)

    Deĭkina, O N; Mishin, V Iu; Demikhova, O V

    2007-01-01

    The purpose of this investigation was to enhance the efficiency of differential diagnosis of pneumonia and pulmonary tuberculosis. A hundred and fifty-nine adult patients were examined. These included 78 patients with pulmonary tuberculosis and 81 with community-acquired p neumonia. The clinical features of infiltrative pulmonary tuberculosis (n = 48) and mild community-acquired pneumonia (n = 51) were compared. The course of caseous pneumonia (n = 30) was compared with that of moderate and severe community-acquired pneumonia (n = 30). Significant differences in the manifestations of the intoxication and bronchopulmonary syndrome were not found in patients with community-acquired pneumonia and infiltrative pulmonary tuberculosis. Physical studies showed that in patients with community-acquired pneumonia, moist rale (54.9%) and crepitation (11.8%) were prevalent, but in those with infiltrative tuberculosis rale was absent in 60.4% of cases and the pattern of respiration was unchanged in 79.2%. Chest X-ray studies indicated that in patients with community-acquired pneumonia, lower lobar inflammatory changes were predominant in 62.8% of cases whereas in those with infiltrative pulmonary tuberculosis the process was mainly bilateral (43.8%) with the presence of destructive changes (83.3%) and bronchogenic dissemination (66.7%). In patients with caseous pneumonia, the intoxication syndrome was more significant than in those with severe community-acquired pneumonia. Chest X-ray studies demonstrated that in patients with caseous pneumonia, specific changes were bilateral with the involvement of 2 lobes or more, with destruction and bronchogenic dissemination while in those with community-acquired pneumonia, the pulmonary processes were predominantly bilateral (76.6%) at the lower lobar site (36.7%). PMID:17338353

  13. Pulmonary Embolism following Endovenous Laser Ablation (EVLA of the Great Saphenous Vein

    Directory of Open Access Journals (Sweden)

    Nnamdi Nwaejike

    2008-08-01

    Full Text Available

    A 70yr old lady presented to accident and emergency with sudden onset pleuritic chest pain. A pulmonary embolus (PE was diagnosed by CTPA. Ten days earlier she had bilateral EVLA for recurrent long saphenous vein disease. Confounding risk factors for pulmonary embolism included bilateral ligation and stripping of the long saphenous vein a year earlier, malignancy, EVLA and phlebitic tributary varices. EVLA has been shown to be an effective treatment for superficial venous insufficiency with low morbidity and high patient satisfaction. The investigation of confounding risk factors and possible causes should not compromise the initial treatment of PE.

  14. Pulmonary metastatic calcification: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Bozi, Lilian Christine Franchiotti [Radiology, Hospital Universitario Antonio Pedro (HUAP), Niteroi, RJ (Brazil); Melo, Alessandro Severo Alves de; Marchiori, Edson, E-mail: edmarchiori@gmail.com [Department of Radiology, School of Medicine, Universidade Federal Fluminense (UFF), Niteroi, RJ (Brazil)

    2012-09-15

    The present report describes the case of a 48-year-old female patient suffering from chronic renal failure on dialysis for 13 years. She presented with hemoptysis, fever, productive cough and dyspnoea. Chest radiography showed predominance of ill-defined opacities in the middle and lower lung fields, bilaterally. Chest computed tomography showed ground glass opacities associated with poorly defined centrilobular nodules with ground-glass attenuation. The patient was submitted to bronchoalveolar lavage that was negative for mycobacteria and fungi. On the basis of such findings, open lung biopsy was performed, which revealed metastatic pulmonary calcification. (author)

  15. Bilateral panophthalmitis in dengue fever

    Directory of Open Access Journals (Sweden)

    Sangeetha Sriram

    2015-01-01

    Full Text Available We report the case of a 25-year-old male patient who presented with bilateral panophthalmitis as the initial ocular manifestation of dengue fever. The diagnosis was a little confusing as he initially presented with features suggestive of retrobulbar hemorrhage secondary to his very low platelet count, which is a common feature of dengue fever. Ophthalmic complications are usually seen in young adults who often present at the nadir of thrombocytopenia. Ocular findings may include anterior uveitis, vitritis, retinal hemorrhages, retinal vascular sheathing, yellow subretinal dots, retinal pigment epithelium (RPE mottling, foveolitis that is clinically seen as a round subretinal yellowish lesion at the fovea, retinochoroiditis, choroidal effusion, optic disc swelling, optic neuritis, neuroretinitis, and oculomotor nerve palsy. [1] There is only one reported case of unilateral endogenous panophthalmitis due to dengue fever. Hence, clinicians and ophthalmologists have to be aware of this vision-threatening complication of dengue for early recognition and prompt treatment to save the vision of these young patients and prevent morbidity.

  16. Bilateral inferior petrosal sinus sampling

    Science.gov (United States)

    Grossrubatscher, Erika; Dalino Ciaramella, Paolo; Boccardi, Edoardo

    2016-01-01

    Simultaneous bilateral inferior petrosal sinus sampling (BIPSS) plays a crucial role in the diagnostic work-up of Cushing’s syndrome. It is the most accurate procedure in the differential diagnosis of hypercortisolism of pituitary or ectopic origin, as compared with clinical, biochemical and imaging analyses, with a sensitivity and specificity of 88–100% and 67–100%, respectively. In the setting of hypercortisolemia, ACTH levels obtained from venous drainage of the pituitary are expected to be higher than the levels of peripheral blood, thus suggesting pituitary ACTH excess as the cause of hypercortisolism. Direct stimulation of the pituitary corticotroph with corticotrophin-releasing hormone enhances the sensitivity of the procedure. The procedure must be undertaken in the presence of hypercortisolemia, which suppresses both the basal and stimulated secretory activity of normal corticotrophic cells: ACTH measured in the sinus is, therefore, the result of the secretory activity of the tumor tissue. The poor accuracy in lateralization of BIPSS (positive predictive value of 50–70%) makes interpetrosal ACTH gradient alone not sufficient for the localization of the tumor. An accurate exploration of the gland is recommended if a tumor is not found in the predicted area. Despite the fact that BIPSS is an invasive procedure, the occurrence of adverse events is extremely rare, particularly if it is performed by experienced operators in referral centres. PMID:27352844

  17. [True hermaphroditism with bilateral ovotestis].

    Science.gov (United States)

    Güitrón, A; Forsbach, G; González, J M; Garza, J G; Vázquez, J; Espinoza, G

    1998-01-01

    A nineteen years old woman with ambiguous external genitalia was studied. This condition had been previously identified as a newborn, but her parents refused medical attention and it was reared as a girl. At 12-years, she began spontaneous mammary development, appearing pubic and axillary hair, and clitoral enlargement. The menarche occurred at 15-years and it was followed by irregular periods. Physical examination, showed absence of hirsutism and acne, normal mammary development equivalent to grade V of Tanner. The external genitalia showed fused labio-scrotal folds with an small introitus. The urethral meatus was absent and was later located inside the introitus. There was a big phallus similar to an adult penis with a normal glans, flexed by a chordee. Hormonal determinations discarded congenital adrenal hyperplasia. The karyotype was 46,XX and testosterone levels were in adult male range. Pelvic ultrasonography disclosed a normal uterus and both gonads in confirmed by laparoscopy identifying bilateral ovotestis. Testicular tissue was removed and plastic reconstruction of female genitals was done. PMID:9528217

  18. Bilateral inferior petrosal sinus sampling.

    Science.gov (United States)

    Zampetti, Benedetta; Grossrubatscher, Erika; Dalino Ciaramella, Paolo; Boccardi, Edoardo; Loli, Paola

    2016-07-01

    Simultaneous bilateral inferior petrosal sinus sampling (BIPSS) plays a crucial role in the diagnostic work-up of Cushing's syndrome. It is the most accurate procedure in the differential diagnosis of hypercortisolism of pituitary or ectopic origin, as compared with clinical, biochemical and imaging analyses, with a sensitivity and specificity of 88-100% and 67-100%, respectively. In the setting of hypercortisolemia, ACTH levels obtained from venous drainage of the pituitary are expected to be higher than the levels of peripheral blood, thus suggesting pituitary ACTH excess as the cause of hypercortisolism. Direct stimulation of the pituitary corticotroph with corticotrophin-releasing hormone enhances the sensitivity of the procedure. The procedure must be undertaken in the presence of hypercortisolemia, which suppresses both the basal and stimulated secretory activity of normal corticotrophic cells: ACTH measured in the sinus is, therefore, the result of the secretory activity of the tumor tissue. The poor accuracy in lateralization of BIPSS (positive predictive value of 50-70%) makes interpetrosal ACTH gradient alone not sufficient for the localization of the tumor. An accurate exploration of the gland is recommended if a tumor is not found in the predicted area. Despite the fact that BIPSS is an invasive procedure, the occurrence of adverse events is extremely rare, particularly if it is performed by experienced operators in referral centres. PMID:27352844

  19. Bilateral inferior petrosal sinus sampling

    Directory of Open Access Journals (Sweden)

    Benedetta Zampetti

    2016-08-01

    Full Text Available Simultaneous bilateral inferior petrosal sinus sampling (BIPSS plays a crucial role in the diagnostic work-up of Cushing’s syndrome. It is the most accurate procedure in the differential diagnosis of hypercortisolism of pituitary or ectopic origin, as compared with clinical, biochemical and imaging analyses, with a sensitivity and specificity of 88–100% and 67–100%, respectively. In the setting of hypercortisolemia, ACTH levels obtained from venous drainage of the pituitary are expected to be higher than the levels of peripheral blood, thus suggesting pituitary ACTH excess as the cause of hypercortisolism. Direct stimulation of the pituitary corticotroph with corticotrophin-releasing hormone enhances the sensitivity of the procedure. The procedure must be undertaken in the presence of hypercortisolemia, which suppresses both the basal and stimulated secretory activity of normal corticotrophic cells: ACTH measured in the sinus is, therefore, the result of the secretory activity of the tumor tissue. The poor accuracy in lateralization of BIPSS (positive predictive value of 50–70% makes interpetrosal ACTH gradient alone not sufficient for the localization of the tumor. An accurate exploration of the gland is recommended if a tumor is not found in the predicted area. Despite the fact that BIPSS is an invasive procedure, the occurrence of adverse events is extremely rare, particularly if it is performed by experienced operators in referral centres.

  20. [An autopsy case of pulmonary tumor thrombotic microangiopathy complicated with interstitial pneumonia and lipoid pneumonia].

    Science.gov (United States)

    Ota, Kyoko; Matsuyama, Masashi; Kokuho, Nariaki; Masuko, Hironori; Hayashi, Hiroki; Iizuka, Takashi; Hayashibara, Kenji; Saito, Takefumi; Kawabata, Yoshinori

    2009-06-01

    A 67-year-old woman was admitted to our hospital because of progressive dyspnea, cough, bloody sputum, and backache. Chest radiography and CT scans showed bilateral diffuse interstitial shadows, bilateral pleural effusion and dilatation of the pulmonary artery. Echocardiography indicated pulmonary hypertension, and the serum tumor marker levels were elevated. We performed right cardiac catheterization, and withdrew some blood from a pulmonary artery catheter in the wedge position. We confirmed moderate pulmonary hypertension, and adenocarcinoma-like malignant cells were seen in the aspirated blood. The patient died of progressive respiratory failure despite supportive care. In addition to PTTM and lymphangiosis carcinomatosa, autopsy of the right lung revealed interstitial pneumonia and lipoid pneumonia, both of which were not reported before to be associated with PTTM. PMID:19601530

  1. Pulmonary function in advanced pulmonary hypertension.

    OpenAIRE

    Burke, C. M.; Glanville, A R; Morris, A J; Rubin, D.; Harvey, J. A.; Theodore, J; Robin, E. D.

    1987-01-01

    Pulmonary mechanical function and gas exchange were studied in 33 patients with advanced pulmonary vascular disease, resulting from primary pulmonary hypertension in 18 cases and from Eisenmenger physiology in 15 cases. Evidence of airway obstruction was found in most patients. In addition, mean total lung capacity (TLC) was only 81.5% of predicted and 27% of our subjects had values of TLC less than one standard deviation below the mean predicted value. The mean value for transfer factor (TLC...

  2. High-resolution CT findings in pulmonary hyalinizing granuloma.

    Science.gov (United States)

    Shibata, Yoshihiro; Kobayashi, Takeshi; Hattori, Yuki; Matsui, Osamu; Gabata, Toshifumi; Tamori, Shunichi; Minato, Hiroshi; Ohta, Yasuhiko

    2007-11-01

    A 47-year-old man with pulmonary hyalinizing granuloma is herein presented. The patient, whose chief complaint was a mild cough, was found by chest radiograph to have multiple bilateral nodules. Subsequent high-resolution computed tomography demonstrated multiple slightly irregular nodules, perinodular ground-glass opacity, peribronchovascular interstitial thickening, and cysts. A mild enlargement of systemic lymph nodes was also noted. Laboratory tests disclosed a slight elevation in the C-reactive protein, gamma-globulin, interleukin-6, and soluble interleukin-2 receptor levels. A histopathologic examination of the specimen yielded from a thoracoscopic lung biopsy resulted in a definite diagnosis of pulmonary hyalinizing granuloma. PMID:18043399

  3. Bilateral Petit’s Triangle Hernia

    Directory of Open Access Journals (Sweden)

    Sanjay Kumar Bhasin, Arshad Bashir Khan, Sanjay Sharma

    2006-07-01

    Full Text Available Lumbar traingle hernia that occurs through lumbar triangles is very rare type of hernia. Only about 300 cases havebeen reported till date. Bilateral Petit’s triangle hernia find further rarity and the case under reference is probably thefirst ever reported case of Primary bilateral Petit’s triangle hernia. The present case is of a 46 years old married,multigravida female who presented with 1 year duration of LBA and subsequently notice of swelling both sides oflow back. FNAC revealed lipoma and on exploration it turned out to be rarest extra peritoneal bilateral Petit’s trianglehernia, fat as contents.

  4. Diffuse Pulmonary Hemorrhage After Fibrinolytic Therapy for Acute Myocardial Infarction in a Cocaine Abuser Patient

    OpenAIRE

    Mohammad Parsa Mahjoob; Isa Khaheshi; Koosha Paydary

    2014-01-01

    We report a 45-year-old man with antroseptal myocardial infarction who developed bilateral basal alveolar infiltrates after initiating the fibrinolytic therapy. Although thrombolytic therapy with streptokinase is generally used in the course of acute myocardial infarction and has diminished morbidity and mortality, pulmonary hemorrhage is an uncommon, but a potentially life-threatening complication that should be regarded as one of the differential diagnoses of pulmonary infiltrates or droppi...

  5. Severe pulmonary hypertension associated with Takayasu arteritis

    Directory of Open Access Journals (Sweden)

    Arevalo Guerrero, Edwin

    2014-10-01

    Full Text Available We describe the case of a 57 year-old woman with chronic hypertension, dyspnea, general symptoms, malaise, fatigability and exercise intolerance, impaired functional capacity and occasional episodes of pre-syncope. Physical examination disclosed arterial hypertension, with a difference of more than 10 mm Hg between the pressures of the right and the left upper limbs, holosystolic murmur grade III/VI in the aortic valve area, left subclavian murmur, and decreased intensity in the peripheral pulses of the left arm. Noninvasive studies showed severe pulmonary hypertension and right ventricular dysfunction, also confirmed by cardiac catheterization. Chest tomography and pulmonary arterial angiography showed bilateral pulmonary artery stenosis. Cardiac magnetic resonance revealed concentric stenosis (6 mm, affecting the origin of the upper lobe branch and circumferential involvement of the left branch (8 mm and the branch to the lower lobe. Endoluminal irregularities were observed in the aorta and the neck vessels, both in the resonance and the angiography. With these findings diagnoses of Takayasu arteritis and associated severe pulmonary hypertension were established. Treatment was started with prednisolone and methotrexate.

  6. A influência da perda bilateral do primeiro molar inferior permanente na morfologia dentofacial: um estudo cefalométrico The influence of bilateral lower first permanent molar loss on dentofacial morfology: a cephalometric study

    Directory of Open Access Journals (Sweden)

    David Normando

    2010-12-01

    than first molars, cases of agenesis and patients under 16 years of age were excluded from the sample. Only individuals who reported losing teeth at least 5 years earlier were evaluated. RESULTS: It was found that bilateral loss of lower first permanent molars leads to smooth closure of GnSN angle (P=0.05, counterclockwise rotation of occlusal plane (P=0.0001, mild decrease in lower anterior face height (P=0.05, pronounced lingual tipping (P=0.04 and retrusion of mandibular incisors (P=0.03. Moreover, bilateral loss of lower first permanent molars did not affect the maxillomandibular relationship in the anteroposterior direction (P=0.21, amount of treatment (P=0.45, inclination of upper incisors (P=0.12 and anteroposterior position of maxillary incisors (P=0.46. CONCLUSION: Bilateral loss of lower first molars can produce marked changes in lower incisor positioning and in the occlusal plane as well as a mild reduction of the face in the vertical direction

  7. Exercise-Induced Pulmonary Edema in a Triathlon

    Directory of Open Access Journals (Sweden)

    Hirotomo Yamanashi

    2015-01-01

    Full Text Available Introduction. Family physicians have more opportunities to attend athletic competitions as medical staff at first-aid centers because of the increasing popularity of endurance sports. Case. A 38-year-old man who participated in a triathlon race experienced difficulty in breathing after swimming and was moved to a first-aid center. His initial oxygen saturation was 82% and a thoracic computed tomography scan showed bilateral ground glass opacity in the peripheral lungs. His diagnosis was noncardiogenic pulmonary edema associated with exercise or swimming: exercise-induced pulmonary edema (EIPE or swimming-induced pulmonary edema (SIPE. Treatment with furosemide and corticosteroid relieved his symptoms of pulmonary edema. Discussion. Noncardiogenic pulmonary edema associated with endurance sports is not common, but knowledge about EIPE/SIPE or neurogenic pulmonary edema associated with hyponatremia, which is called Ayus-Arieff syndrome, is crucial. Knowledge and caution for possible risk factors, such as exposure to cold water or overhydration, are essential for both medical staff and endurance athletes. Conclusion. To determine the presence of pulmonary edema associated with strenuous exercise, oxygen saturation should be used as a screening tool at a first-aid center. To avoid risks for EIPE/SIPE, knowledge about these diseases is essential for medical staff and for athletes who perform extreme exercise.

  8. Exercise-Induced Pulmonary Edema in a Triathlon.

    Science.gov (United States)

    Yamanashi, Hirotomo; Koyamatsu, Jun; Nobuyoshi, Masaharu; Murase, Kunihiko; Maeda, Takahiro

    2015-01-01

    Introduction. Family physicians have more opportunities to attend athletic competitions as medical staff at first-aid centers because of the increasing popularity of endurance sports. Case. A 38-year-old man who participated in a triathlon race experienced difficulty in breathing after swimming and was moved to a first-aid center. His initial oxygen saturation was 82% and a thoracic computed tomography scan showed bilateral ground glass opacity in the peripheral lungs. His diagnosis was noncardiogenic pulmonary edema associated with exercise or swimming: exercise-induced pulmonary edema (EIPE) or swimming-induced pulmonary edema (SIPE). Treatment with furosemide and corticosteroid relieved his symptoms of pulmonary edema. Discussion. Noncardiogenic pulmonary edema associated with endurance sports is not common, but knowledge about EIPE/SIPE or neurogenic pulmonary edema associated with hyponatremia, which is called Ayus-Arieff syndrome, is crucial. Knowledge and caution for possible risk factors, such as exposure to cold water or overhydration, are essential for both medical staff and endurance athletes. Conclusion. To determine the presence of pulmonary edema associated with strenuous exercise, oxygen saturation should be used as a screening tool at a first-aid center. To avoid risks for EIPE/SIPE, knowledge about these diseases is essential for medical staff and for athletes who perform extreme exercise.

  9. [Vasomotor reactions of the pulmonary circulation upon hypothalamic stimulation].

    Science.gov (United States)

    Tsybenko, V A; Guinh-van-Tam

    1977-01-01

    The blood flow in the lung lobe perfused under constant pressure was recorded with photoelectric drop recorder in acute closed-chest experiments in anesthetized dogs. Stimulation of supraoptic and lateral mamillary nuclei in most cases decreased flow rate and increased vascular resistance in perfused pulmonary lobe, while stimulation of sympatho-inhibitory area exerted opposite effect. Bilateral vagotomy and administration of atropin and inderal did not block the lesser circulation responses to the hypothalamic stimulation. However, phentolamine and bilateral ablation of stellate and upper thoracic ganglia eliminated chnages of blood flow and vascular resistance in the perfused lobe. The data obtained suggest that stimulation of some hypothalamic areas evoke obvious vasomotor responses in the lesser circulation and that these responses occur mainly through alpha-adrenergic sympathetic innervation of pulmonary vessels. PMID:832755

  10. Miliary pulmonary cryptococcosis

    Directory of Open Access Journals (Sweden)

    Shane Kelly

    2014-10-01

    Imaging features of pulmonary cryptococcosis are generally of well-defined pleural-based nodules and less commonly alveolar infiltrates, lymphadenopathy, pleural effusions or cavitating lesions. Miliary pulmonary infiltrates are an exceptionally rare presentation.

  11. Pulmonary alveolar proteinosis

    Science.gov (United States)

    Alveolar proteinosis; Pulmonary alveolar phospholipoproteinosis ... In some cases, the cause of pulmonary alveolar proteinosis is unknown. In others, it occurs with lung infection or an immune problem. It also can occur with cancers of the blood system, ...

  12. THE EUROPEAN UNION’S BILATERAL APPROACH

    Directory of Open Access Journals (Sweden)

    Ludmila BORTA

    2014-12-01

    Full Text Available The EU is a world economic power and a major trading partner for most countries. All the time, this region has been interested and has acted towards a free and fair trade. The decrease and even the elimination of tariff and non-tariff barriers in the world trade are among the main objectives of the EU strategy for international trade. At the moment, the elusive outcome of the WTO Doha Round has led to the proliferation of bilateral trade agreements worldwide. Although the EU remains committed to further development of the multilateral trading system, however, the EU still has appealed also to the development of bilateral trade relations. The aim of this paper is to illustrate the current bilateral dimension of the common commercial policy of the EU. In conclusion, to describe this bilateral approach of the EU we are using one word, namely “diversity”.

  13. Bilateral areolar and periareolar pityriasis versicolor.

    Science.gov (United States)

    Sárdy, Miklós; Korting, Hans Christian; Ruzicka, Thomas; Wolff, Hans

    2010-08-01

    An adolescent boy presented with isolated, symmetrical, bilateral areolar and periareolar pityriasis versicolor. This extremely rare condition should be considered in the differential diagnosis of light brown patches on the areolae.

  14. FLOWING BILATERAL FILTER: DEFINITION AND IMPLEMENTATIONS

    Directory of Open Access Journals (Sweden)

    Maxime Moreaud

    2015-06-01

    Full Text Available The bilateral filter plays a key role in image processing applications due to its intuitive parameterization and its high quality filter result, smoothing homogeneous regions while preserving the edges of the objects. Considering the image as a topological relief, seeing pixel intensities as peaks and valleys, we introduce a way to control the tonal weighting coefficients, the flowing bilateral filter, reducing "halo" artifacts typically produced by the regular bilateral filter around a large peak surrounded by two valleys of lower values. In this paper we propose to investigate exact and approximated versions of CPU and parallel GPU (Graphical Processing Unit based implementations of the regular and flowing bilateral filter using the NVidia CUDA API. Fast implementations of these filters are important for the processing of large 3D volumes up to several GB acquired by x-ray or electron tomography.

  15. An unusual cause of bilateral diaphragmatic paralysis.

    OpenAIRE

    Pandit, A; Kalra, S.; Woodcock, A

    1992-01-01

    In a patient who had a sudden onset of bilateral diaphragmatic paralysis after forceful neck manipulation complete, though gradual, recovery in lung function and transdiaphragmatic pressures was seen over three years. This is a previously unrecognised risk of neck osteopathy.

  16. Extensive pulmonary alveolar microlithiasis

    OpenAIRE

    Rittayamai, Nuttapol; Muangman, Nisa; Ruangchira-Urai, Ruchira

    2013-01-01

    Pulmonary alveolar microlithiasis is a rare pulmonary disorder that is caused by abnormal sodium-dependent phosphate co-transporter from the mutation of SLC34A2 gene, leading to accumulation of microliths in the alveoli. We report the extensive pulmonary alveolar microlithiasis in an elderly woman who presented with progressive dyspnea for 2 months. Chest radiograph revealed diffuse pulmonary calcification. Tissue histopathology from open lung biopsy demonstrated widespread intra-alveolar lam...

  17. Sequential presentation of bilateral Brown syndrome.

    Science.gov (United States)

    Sekeroğlu, Hande Taylan; Türkçüoğlu, Peykan; Sanaç, Ali Şefik; Sener, Emin Cumhur

    2012-04-01

    Brown syndrome, characterized by a limitation of elevation in adduction and positive forced duction testing, is usually unilateral but occurs bilaterally in 10% of all cases. It may present as a congenital condition in one eye and develop in the other eye with no apparent cause. We present a case of bilateral Brown syndrome in which the right eye became involved within 1 year of surgery on the left eye for congenital Brown syndrome.

  18. Bilateral synchronous plasmacytoma of the testis.

    Science.gov (United States)

    Narayanan, Geetha; Joseph, Rona; Soman, Lali V

    2016-04-01

    Extramedullary plasmacytoma (EMP) is usually seen in the head and neck regions and in the upper respiratory, gastrointestinal, and central nervous systems. Testis is a rare site for EMP, and bilateral synchronous testicular plasmacytoma occurring as an isolated event at initial presentation has been reported only once previously. We present herein the second such report in a 70-year-old man who underwent bilateral orchidectomy. PMID:27034568

  19. Bilateral anophthalmia with septo-optic dysplasia

    Directory of Open Access Journals (Sweden)

    Manisha Jana

    2010-01-01

    Full Text Available Bilateral anophthalmia is a rare entity and association with septo-optic dysplasia is an even rare condition. The condition is characterized by absent eyeballs in the presence of eyelids, conjunctiva or lacrimal apparatus. Though anophthalmia can be diagnosed clinically, imaging plays a crucial role in delineating the associated anomalies. In addition, often clinical anophthalmia may prove to be severe microphthalmia on imaging. We describe the imaging findings in an infant with bilateral anophthalmia and septo-optic dysplasia.

  20. Symptoms, Diagnosis and Treatment for Pulmonary Fibrosis

    Science.gov (United States)

    ... www.lung.org > Lung Health and Diseases > Lung Disease Lookup > Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, Causes & Risk Factors Pulmonary ... while processing XML file."); } }); } } --> Blank Section Header Lung Disease Lookup Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, ...

  1. Didelphic uterus with imperforate unilateral vagina and ipsilateral renal agenesis: Magnetic resonance. Utero didelfo con vagina unilateral imperforada y agenesia renal ipsilateral: resonancia magnetica

    Energy Technology Data Exchange (ETDEWEB)

    Rodrigo, R.M.; Saez, F.; Astigarraga, E.; Rodriguez, O. (Hospital de Cruces. Servicio de Radiodiagnostico. Baracaldo.Vizcaya (Spain))

    1993-01-01

    We present two cases of didelphic uterus with the clinical appearance of a pelvic mass produced by the hematometra-hematocolpos retained by an imperforate unilateral hemivagina. In both cases, this anomaly was accompanied by ipsilateral renal agenesis. We basically review the diagnosis of this disorder, focusing on magnetic resonance as the technique of choice. (Author) 10 refs.

  2. Novel missense mutation in the L1 gene in a child with corpus callosum agenesis, retardation, adducted thumbs, spastic paraparesis, and hydrocephalus

    NARCIS (Netherlands)

    Sztriha, L; Frossard, P; Hofstra, RMW; Verlind, E; Nork, M

    2000-01-01

    Corpus callosum agenesis, retardation, adducted thumbs, spastic paraparesis, and hydrocephalus (CRASH syndrome) is an X-linked recessive disorder caused by mutations in the neuronal cell adhesion molecule L1 (L1CAM) gene. L1 plays a key role in axon outgrowth and pathfinding during the development o

  3. Deep Venous Thrombosis of the Leg, Associated with Agenesis of the Infrarenal Inferior Vena Cava and Hypoplastic Left Kidney (KILT Syndrome in a 14-Year-Old Child

    Directory of Open Access Journals (Sweden)

    Sakshi Bami

    2015-01-01

    Full Text Available Agenesis of the inferior vena cava (IVC is a rare anomaly which can be identified as incidental finding or can be associated with iliofemoral vein thrombosis. IVC agenesis has a known association with renal anomalies which are mainly confined to the right kidney. We describe a case of a 14-year-old male who presented with left leg swelling and pain. Ultrasonography confirmed the presence of left leg deep vein thrombosis (DVT. No underlying hematologic risk factors were identified. A CT scan was obtained which demonstrated absent infrarenal IVC and extensive thrombosis in the left deep venous system and development of collateral venous flow into the azygous/hemiazygous system, with extension of thrombus into paraspinal collaterals. An additional finding in the patient was an atrophic left kidney and stenosis of an accessory left renal artery. Agenesis of the IVC should be considered in a young patient presenting with lower extremity DVT, especially in patients with no risk factors for thrombosis. As agenesis of the IVC cannot be corrected, one should be aware that there is a lifelong risk of lower extremity DVT.

  4. Beware of the devastating pulmonary aspergillosis syndromes In certain environments

    LENUS (Irish Health Repository)

    Kooblall, M

    2016-02-01

    Nowadays with more stem cell transplants and immunosuppressive therapies there has been a rise in pulmonary aspergillosis syndromes. The following illustrates such a case. A 49 year old man had a past history of ankylosing spondylitis with a bilateral hip replacement. He was also on surveillance for a superficial bladder tumour since 2007. His chest x-ray in 2008 was normal. In 2010 his CXR showed patchy opacification in the right apex. CT thorax confirm fibrotic changes.

  5. Pulmonary Idiopathic Alveolar Ossification in a Raccoon (Procyon lotor)

    OpenAIRE

    Hamir, Amir N; Rupprecht, Charles E.

    2010-01-01

    Here we describe gross and histopathologic findings in a laboratory-confined adult male raccoon (Procyon lotor) with microscopic ossified areas in pulmonary alveoli. At the time of necropsy, gross lesions were present in the kidneys and in one thyroid gland. Noteworthy microscopic findings included multifocal foci of osseous tissue within the alveoli of the lungs, bilateral thyroid adenomas, pancreatic islet cell amyloidosis, cortical kidney infarcts, cystic adenomatous hyperplasia of urinary...

  6. MODERN VIEWS ON BILATERAL BREAST CANCER

    Directory of Open Access Journals (Sweden)

    Ye. A. Fesik

    2014-01-01

    Full Text Available Presented modern literature data on the features of the pathogenesis, course, clinical and morphological expression and tumor characteristics, parameters and nodal metastasis of hematogenous bilateral breast cancer. Highlight the results of domestic and foreign studies in recent years to determine the prognostic factors and recurrence of synchronous and metachronous bilateral breast cancer. It was revealed that the frequency of bilateral breast tumor lesions varies widely, ranging from 0.1 to 20%, with metachronous tumors recorded significantly higher (69.6% than the synchronous (22.7%. The probability of occurrence of metachronous breast cancer is higher in women with a family history, as well as if they have a gene mutation BRCA-1. Found that the most common histological type of breast tumor with bilateral lesions is invasive ductal. However, the incidence of invasive lobular cancer and non-invasive lobular cancer is slightly higher among synchronous bilateral cancer compared with unilateral disease. Studies have shown that in a double-sided synchronous breast cancer tumor, as a rule, has a lower degree of differentiation, and the higher the expression level of estrogen receptors and progesterone receptors. Relevance of the issue because the identification of patterns in the study of lymphatic and hematogenous features bilateral metastasis of mammary tumors provides a basis for speculation about the differences in the progression of neoplastic disease in these groups and is a cause for further detailed research in this area to identify and evaluate the prognosis and also the choice of tactics of such patients.

  7. Bilateral Keratectasia 34 Years after Corneal Transplant

    Directory of Open Access Journals (Sweden)

    Xavier Valldeperas

    2010-07-01

    Full Text Available We report the clinical findings of a patient with severe bilateral keratectasia 34 years after a penetrating keratoplasty (PK in both eyes. An otherwise healthy 67-year-old man complained of deterioration of the eyesight in both eyes over the last 6 months. The patient was diagnosed with bilateral keratoconus at the age of 32 years, and he underwent a bilateral PK. At presentation, visual acuity was 20/200 in the right eye and light perception in the left eye. A Pentacam pachymetric map revealed a central pachymetry of 720 µm in the right eye and of 710 µm in the left eye, as well as an average paracentral pachymetry of 436 and 270 µm in the 9-mm zone in the right and the left eye, respectively. Corneal topography revealed bilateral irregular and asymmetric bowing with generalized steepening and high corneal power. We describe a case of bilateral keratectasia 34 years after PK in a patient who was originally diagnosed with bilateral keratoconus.

  8. Microsurgical one-stage treatment of intracranial mirror aneurysms via bilateral frontolateral approaches

    Directory of Open Access Journals (Sweden)

    Hong-guang WANG

    2016-08-01

    Full Text Available Objective To investigate surgical strategies, clinical effects and complications of microsurgical one-stage treatment of intracranial mirror aneurysms via bilateral frontolateral approaches.  Methods Review clinical data of 18 cases with anterior circulation mirror aneurysms who underwent one-stage clipping via bilateral frontolateral approaches from July 2010 to July 2015 admitted to Department of Neurosurgery in Tianjin Huanhu Hospital. The operative efficacy was evaluated according to postoperative Glasgow Outcome Scale (GOS.  Results The 36 aneurysms in 18 patients were successfully clipped via bilateral frontolateral approaches at one-stage, including 18 posterior communicating artery (PCoA mirror aneurysms in 9 cases and 18 middle cerebral artery (MCA mirror aneurysms in 9 cases. GOS score of 5 was discovered in 16 cases, and 4 was discovered in 2 cases after operation. One case underwent ventriculoperitoneal shunting (VPS due to communicating hydrocephalus, one case got postoperative pulmonary infection and no death occurred. Intracranial CTA at 6 months postoperatively showed aneurysms of 18 patients were clipped completely, the parent artery blood flow was smooth, and no recurrence was found.  Conclusions Microsurgical one-stage clipping via bilateral frontolateral approaches for treating intracranial mirror aneurysms is a sugrical method with small incision, fitting surgical field, high safety, satisfactory effect and good prognosis, which is a new minimally invasive neurosurgical technique. DOI: 10.3969/j.issn.1672-6731.2016.08.012

  9. Relation between agenesis and shape anomaly of maxillary lateral incisors and canine impaction

    Directory of Open Access Journals (Sweden)

    Anísio Bueno de Carvalho

    2012-12-01

    Full Text Available OBJECTIVE: The purpose was to obtain information about the relation between agenesis and shape anomaly of maxillary lateral incisors and canine impaction. METHODS: Seventy-three patients with canine impaction and 73 control patients, without canine impaction, were evaluated. The mesiodistal distances of the maxillary lateral incisors adjacent to the impacted canines and the correspondent mandibular lateral incisors were measured. The adjacent lateral incisors were classified in: 1 - absent, 2 - small, 3 - peg-shaped, 4 - standard. RESULTS: The results showed that among the patients with impacted canines, there were 21 anomalous teeth (small and peg-shaped and among the control patients there were only three small and peg-shaped teeth, with a statistically significant difference (p = 0.001. No patients were found with impacted canines and absent lateral incisors. CONCLUSION: It was concluded that in patients with anomalous lateral incisors (small and peg-shaped there is a probability to present impacted canines and this must be considered.OBJETIVO: o objetivo foi obter informação sobre a relação existente entre a agenesia e/ou anomalia de forma de incisivos laterais superiores e impacção de caninos. MÉTODOS: foram avaliados 73 pacientes com impacção de caninos e 73 pacientes controle, sem impacção de caninos. Foram medidas as distâncias mesiodistais dos incisivos laterais superiores adjacentes aos caninos impactados e os incisivos laterais correspondentes inferiores. Os incisivos laterais adjacentes foram classificados em: 1 - ausentes; 2 - pequenos; 3 - conoides; 4 - normais. RESULTADOS: os resultados mostraram que no grupo de pacientes com caninos impactados foram encontrados 22 dentes anômalos (pequenos e conoides, e no grupo controle apenas três dentes pequenos e conoides, sendo uma diferença estatisticamente significativa (p=0,001. Não foram encontrados pacientes com canino impactado e incisivo lateral ausente. CONCLUS

  10. Candidate gene analysis of tooth agenesis identifies novel mutations in six genes and suggests significant role for WNT and EDA signaling and allele combinations.

    Science.gov (United States)

    Arte, Sirpa; Parmanen, Satu; Pirinen, Sinikka; Alaluusua, Satu; Nieminen, Pekka

    2013-01-01

    Failure to develop complete dentition, tooth agenesis, is a common developmental anomaly manifested most often as isolated but also as associated with many developmental syndromes. It typically affects third molars or one or few other permanent teeth but severe agenesis is also relatively prevalent. Here we report mutational analyses of seven candidate genes in a cohort of 127 probands with non-syndromic tooth agenesis. 82 lacked more than five permanent teeth excluding third molars, called as oligodontia. We identified 28 mutations, 17 of which were novel. Together with our previous reports, we have identified two mutations in MSX1, AXIN2 and EDARADD, five in PAX9, four in EDA and EDAR, and nine in WNT10A. They were observed in 58 probands (44%), with a mean number of missing teeth of 11.7 (range 4 to 34). Almost all of these probands had severe agenesis. Only few of the probands but several relatives with heterozygous genotypes of WNT10A or EDAR conformed to the common type of non-syndromic tooth agenesis, incisor-premolar hypodontia. Mutations in MSX1 and PAX9 affected predominantly posterior teeth, whereas both deciduous and permanent incisors were especially sensitive to mutations in EDA and EDAR. Many mutations in EDAR, EDARADD and WNT10A were present in several families. Biallelic or heterozygous genotypes of WNT10A were observed in 32 and hemizygous or heterozygous genotypes of EDA, EDAR or EDARADD in 22 probands. An EDARADD variant were in seven probands present together with variants in EDAR or WNT10A, suggesting combined phenotypic effects of alleles in distinct genes. PMID:23991204

  11. Candidate gene analysis of tooth agenesis identifies novel mutations in six genes and suggests significant role for WNT and EDA signaling and allele combinations.

    Directory of Open Access Journals (Sweden)

    Sirpa Arte

    Full Text Available Failure to develop complete dentition, tooth agenesis, is a common developmental anomaly manifested most often as isolated but also as associated with many developmental syndromes. It typically affects third molars or one or few other permanent teeth but severe agenesis is also relatively prevalent. Here we report mutational analyses of seven candidate genes in a cohort of 127 probands with non-syndromic tooth agenesis. 82 lacked more than five permanent teeth excluding third molars, called as oligodontia. We identified 28 mutations, 17 of which were novel. Together with our previous reports, we have identified two mutations in MSX1, AXIN2 and EDARADD, five in PAX9, four in EDA and EDAR, and nine in WNT10A. They were observed in 58 probands (44%, with a mean number of missing teeth of 11.7 (range 4 to 34. Almost all of these probands had severe agenesis. Only few of the probands but several relatives with heterozygous genotypes of WNT10A or EDAR conformed to the common type of non-syndromic tooth agenesis, incisor-premolar hypodontia. Mutations in MSX1 and PAX9 affected predominantly posterior teeth, whereas both deciduous and permanent incisors were especially sensitive to mutations in EDA and EDAR. Many mutations in EDAR, EDARADD and WNT10A were present in several families. Biallelic or heterozygous genotypes of WNT10A were observed in 32 and hemizygous or heterozygous genotypes of EDA, EDAR or EDARADD in 22 probands. An EDARADD variant were in seven probands present together with variants in EDAR or WNT10A, suggesting combined phenotypic effects of alleles in distinct genes.

  12. Pulmonary vascular diseases.

    Science.gov (United States)

    Mélot, C; Naeije, R

    2011-04-01

    Diseases of the pulmonary vasculature are a cause of increased pulmonary vascular resistance (PVR) in pulmonary embolism, chronic thromboembolic pulmonary hypertension (CTEPH), and pulmonary arterial hypertension or decreased PVR in pulmonary arteriovenous malformations on hereditary hemorrhagic telangiectasia, portal hypertension, or cavopulmonary anastomosis. All these conditions are associated with a decrease in both arterial PO2 and PCO2. Gas exchange in pulmonary vascular diseases with increased PVR is characterized by a shift of ventilation and perfusion to high ventilation-perfusion ratios, a mild to moderate increase in perfusion to low ventilation-perfusion ratios, and an increased physiologic dead space. Hypoxemia in these patients is essentially explained by altered ventilation-perfusion matching amplified by a decreased mixed venous PO2 caused by a low cardiac output. Hypocapnia is accounted for by hyperventilation, which is essentially related to an increased chemosensitivity. A cardiac shunt on a patent foramen ovale may be a cause of severe hypoxemia in a proportion of patients with pulmonary hypertension and an increase in right atrial pressure. Gas exchange in pulmonary arteriovenous malformations is characterized by variable degree of pulmonary shunting and/or diffusion-perfusion imbalance. Hypocapnia is caused by an increased ventilation in relation to an increased pulmonary blood flow with direct peripheral chemoreceptor stimulation by shunted mixed venous blood flow. PMID:23737196

  13. Efficacy of total lymphoid irradiation for chronic allograft rejection following bilateral lung transplantation

    International Nuclear Information System (INIS)

    Purpose: To assess the safety and efficacy of total lymphoid irradiation (TLI) in patients experiencing chronic rejection following bilateral lung transplantation (BLT). Patients and Materials: Eleven patients received TLI for chronic allograft rejection (bronchiolitis obliterans syndrome) refractory to conventional treatment modalities. Radiation therapy (RT) was prescribed as 8 Gy delivered in 10 0.8-Gy fractions, 2 fractions/week, via mantle, paraaortic, and inverted-Y fields. Serial pre- and post-RT pulmonary function values, complete blood counts, and immunosuppressive augmentation requirements [use of methylprednisolone, murine anti-human mature T-cell monoclonal antibody (OKT3), polyclonal antithymocyte globulin (ATG), and tacrolimus] were monitored. Results: In the 3 months preceding TLI, the average decrease in forced expiratory volume in 1 s (FEV1) was 34% (range 0-75%) and the median number of immunosuppression augmentations was 3 (range 0-5). Only 4 of 11 patients completed all 10 TLI treatment fractions. Reasons for discontinuation included progressive pulmonary decline (four patients), worsening pulmonary infection (two patients), and persistent thrombocytopenia (one patient). Seven of the 11 patients failed within 8 weeks of treatment cessation. One patient had unabated rejection and received bilateral living related-donor transplants; he is alive and well. Six patients died. Two of these deaths were due to pulmonary infection from organisms isolated prior to the start of RT; the other four deaths were from progressive pulmonary decline. The four remaining patients had durable positive responses to TLI (mean follow-up of 47 weeks; range 24-72). Comparing the 3 months preceding RT to the 3 months following treatment, these four patients had improvements in average FEV1 (40% decline vs. 1% improvement) and fewer median number of immunosuppressive augmentations (3.5 vs. 0). None of these patients has developed lymphoproliferative disease or has died

  14. Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

    Institute of Scientific and Technical Information of China (English)

    Sathya; Chinnaa; Chandan; J; Das; Sanjay; Sharma; Prabhjot; Singh; Amlesh; Seth; Suvendu; Purkait; Sandeep; R; Mathur

    2014-01-01

    Peripheral primitive neuroectodermal tumor(PNET) of the kidney is a rare, aggressive tumor known for its recurrence and metastatic potential. Despite the frequency of venous extension to the renal veins and inferior vena cava, pulmonary tumor embolism at the initial presentation is not common. We report a case of 22-year-old female with PNET of the kidney who presented with tumor embolism in the inferior vena cava(IVC) and bilateral pulmonary artery. The patient underwent surgical resection and histopathological analysis confirmed the presence of tumor within the IVC and pulmonary arteries. The patient received adjuvant chemotherapy and is currently doing well on follow-up.

  15. Benign metastasizing leiomyomatosis (BML: A rare cause of cavitary and cystic pulmonary nodules

    Directory of Open Access Journals (Sweden)

    Joshua S. Jolissaint

    2015-01-01

    Full Text Available Benign metastasizing leiomyomatosis (BML is a rare cause of pulmonary lesions found in reproductive age women who have undergone a hysterectomy for uterine leiomyoma. Given the relative rarity of the disease, the management of these lesions varies from surgical (oopherectomy or medical antiestrogen hormonal therapy to clinical observation and survelliance. The disease generally presents asymptomatically with multiple, well-defined pulmonary nodules discovered incidentally on imaging. We report an atypical presentation of a 46-year-old woman with incidentally found bilateral pulmonary cavitating nodules and cysts, concerning for lymphangioleiomyomatosis (LAM, who was ultimately diagnosed with BML.

  16. Multidetector computed tomography pulmonary angiography in childhood acute pulmonary embolism

    Energy Technology Data Exchange (ETDEWEB)

    Tang, Chun Xiang; Zhang, Long Jiang; Lu, Guang Ming [Medical School of Nanjing University, Department of Medical Imaging, Jinling Hospital, Nanjing, Jiangsu (China); Schoepf, U.J. [Medical School of Nanjing University, Department of Medical Imaging, Jinling Hospital, Nanjing, Jiangsu (China); Medical University of South Carolina, Department of Radiology and Radiological Science, Charleston, SC (United States); Medical University of South Carolina, Department of Pediatrics, Charleston, SC (United States); Chowdhury, Shahryar M. [Medical University of South Carolina, Department of Pediatrics, Charleston, SC (United States); Fox, Mary A. [Medical University of South Carolina, Department of Radiology and Radiological Science, Charleston, SC (United States)

    2015-09-15

    Pulmonary embolism is a life-threatening condition affecting people of all ages. Multidetector row CT pulmonary angiography has improved the imaging of pulmonary embolism in both adults and children and is now regarded as the routine modality for detection of pulmonary embolism. Advanced CT pulmonary angiography techniques developed in recent years, such as dual-energy CT, have been applied as a one-stop modality for pulmonary embolism diagnosis in children, as they can simultaneously provide anatomical and functional information. We discuss CT pulmonary angiography techniques, common and uncommon findings of pulmonary embolism in both conventional and dual-energy CT pulmonary angiography, and radiation dose considerations. (orig.)

  17. Simultaneus bilateral spontaneus pneumothoraces: A Retrospective Analysis of 11 Cases

    Directory of Open Access Journals (Sweden)

    Ufuk Cobanoglu

    2011-09-01

    Full Text Available Spontaneous pneumothorax (SP is relatively common in clinical practice and occurs more frequently in young, tall thin men, and in smokers. However, simultaneous bilateral spontaneous pneumothorax (SBSP is a rare clinical condition that often presents with significant respiratory distress. It is often dangerous; therefore, the chest drain should be inserted immediately. In this study, simultaneus bilateral spontaneus pneumothoraces cases were divided into two groups and retrospectively evaluated according to age, sex, diagnostic methods, treatments, and results. Material and Methods :Between January 2006 and May 2009, 11 patients with SBSP were enrolled into our study. Age, gender, underlying lung disease, smoking history, symptoms, diagnosis, treatment type, surgical indication, morbidity, recurrence, mortality, duration of chest tube and postoperative hospital stay of the patients were reviewed. Arterial blood gas values (before and after intervention in patiens with primary and secondary spontaneous pneumothorax were evaluated. Results; 7 patients (63.63 % were male and 4 patients (36.37 % were female and their mean age was 34,5±6.81 years. There were 4 (36.37% primary SP and 4 (36.37% secondary SP patients. Chronic obstructive pulmonary disease (COPD was the most common cause in secondary SP patients. In two (18.18% patients recurrency were observed. Eight (72.72% patients had smoking history. The most common symptom in both groups was dyspnea. All patients had immediate bilateral chest tubes on admission. We treated these patients with chest drain insertion, VATS (Video-Assisted Thoracoscopic Surgery, axillary thoracotomy, and chemical pleurodesis. Postoperative morbidity was detected in 3 (27.27% patients (prolonged air leak in 1 case, empyema in 1 case and pneumonia in 1 case. No mortality was observed in alll cases. Recurrence developed in 3 (27.27% patients in this series. Conclusions: An urgent and effective treatment requires in the

  18. Bilateral ovarian cystic teratomata mimicking bilateral pure ovarian hemangiomata: case report.

    Science.gov (United States)

    Feuerstein, I M; Aronson, B L; McCarthy, E F

    1984-01-01

    This report describes a case of bilateral, benign cystic ovarian teratomata which were composed predominantly of cavernous hemangiomatous elements. The right-sided lesion, in particular, mimicked a pure ovarian hemangioma. The clinical presentation, bilaterality of the lesions, the sizes of the hemangiomata, and the subsequent hemangioma of the leg are all of interest in this very rare lesion. PMID:6511164

  19. Bilateral electric energy contracts: return and risk

    Energy Technology Data Exchange (ETDEWEB)

    Gunn, Laura K.; Silva, Elisa B.; Correia, Paulo B. [State University of Campinas (UNICAMP), SP (Brazil). College of Mechanical Engineering

    2009-07-01

    In Brazil electricity is traded through three segments: the spot market that balances offer and demand, with prices calculated by a cost-based computational model; the regulated market , where prices are settled in public auctions, and the free market for bilateral contracts. As spot and regulated market prices are public information, a seller is able to calculate his opportunity price to trade a bilateral contract in the free market by using the non-arbitrage principle. Thus, the seller searches the price of a bilateral contract in the free market that balances his/her revenues with the value expected in case it were negotiated in the regulated and the spot market. Besides the expected revenue, the seller may also consider the CVaR to measure the risk of her/his bilateral contract in the free market. So this paper develops a binomial lattice approach to price bilateral contracts in the free market, considering the seller's opportunity of negotiations in both regulated and spot markets, and measuring the contract risk directly. (author)

  20. Pulmonary Alveolar Proteinosis in Association with Congenital Dyserythropoietic Anemia: A Case Report

    OpenAIRE

    Carden, Marcus A.; Ashish Barman; Gita Massey

    2012-01-01

    A two-year-old girl with congenital dyserythropoietic anemia (CDA) acutely developed fever, tachypnea, and increased oxygen requirement. Chest X-ray revealed bilateral interstitial infiltrates and mild cardiomegaly. Blood cultures grew no infectious agents, while pulmonary specimens grew cytomegalovirus (CMV). Treatment with intravenous ganciclovir was initiated but without response. Final cytologic preparations of bronchoalveolar lavage (BAL) fluid revealed eosinophilic amorphous material co...

  1. Unsuspected pulmonary alveolar proteinosis in a patient with acquired immunodeficiency syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Niazi Masooma

    2011-02-01

    Full Text Available Abstract Introduction Diffuse lung infiltrates are a common finding in patients with acquired immunodeficiency syndrome and causes range from infectious processes to malignancies or interstitial lung diseases. Pulmonary alveolar proteinosis is a rare pulmonary disorder rarely reported in patients infected with human immunodeficiency virus. Secondary pulmonary alveolar proteinosis is associated with conditions involving functional impairment or reduced numbers of alveolar macrophages. It can be caused by hematologic malignancies, inhalation of toxic dust, fumes or gases, infectious or pharmacologic immunosuppression, or lysinuric protein intolerance. Case presentation A 42-year-old African American man infected with human immunodeficiency virus was admitted with chronic respiratory symptoms and diffuse pulmonary infiltrates. Chest computed tomography revealed bilateral spontaneous pneumothoraces, for which he required bilateral chest tubes. Initial laboratory investigations did not reveal any contributory conditions. Histological examination of a lung biopsy taken during video-assisted thoracoscopy showed pulmonary alveolar proteinosis concurrent with cytomegalovirus pneumonitis. After ganciclovir treatment, our patient showed radiologic and clinical improvement. Conclusion The differential diagnosis for patients with immunosuppression and lung infiltrates requires extensive investigations. As pulmonary alveolar proteinosis is rare, the diagnosis can be easily missed. Our case highlights the importance of invasive investigations and histology in the management of patients infected with human immunodeficiency virus and pulmonary disease who do not respond to empiric therapy.

  2. Fistulectomy as a surgical option for pulmonary arteriovenous malformation

    Institute of Scientific and Technical Information of China (English)

    ZHANG Shao-yan; ZHANG Zhi-tai; OU Song-lei; HU Yan-sheng; SONG Fei-qiang; LI Xin; MA Xu-chen; MA Xin-xin; LIANG Lin; LI Dong; GUO Lin; SUN Zhen

    2009-01-01

    Background Surgical resection remains the treatment of choice for pulmonary arteriovenous malformation but removes some normal lung parenchyma. This study aimed to evaluate the effect and safety of the lung-saving procedure of fistulectomy as an alternative to lung resection.Methods From July 2003 to July 2008, 6 selected patients with pulmonary arteriovenous malformations underwent fistulectomies. Among them, 1 patient underwent emergency operation and 2 underwent bilateral operations. One patient received postoperative embolotherapy.Results No hospital deaths or postoperative morbidity occurred. PaO_2 increased significantly after operation. All patients were free of symptoms and hypoxia during a follow-up for 9 months to 5 years.Conclusions Fistulectomy is a safe and effective procedure for patients with pulmonary arteriovenous malformation and may be an alternative to lung resection.

  3. Chronic Bilateral Tibial Stress Fractures with Valgus Treated with Bilateral Intramedullary Nailing: A Case Report

    Directory of Open Access Journals (Sweden)

    Steven K Dailey

    2014-01-01

    Full Text Available Introduction: Stress fractures are overuse injuries most commonly seen in athletes, military recruits, and individuals with endocrine abnormalities. It has been demonstrated that chronic cases of anterior tibial stress fractures refractory to conservative management respond well to intramedullary nailing. To our knowledge, only one report has been published concerning patients with bilateral tibial stress fractures treated with bilateral intramedullary nailing. All patients in the series were high-level athletes. We present the case of a non-athletic patient with chronic bilateral tibial stress fractures and associated deformity successfully treated with bilateral intramedullary nails. Case Report: A 23-year-old Caucasian female full-time student presented with chronic bilateral shin pain for approximately five years. She had failed an extensive regimen of conservative management. She was diagnosed with chronic bilateral tibial stress fractures based on history, physical examination, and radiologic findings. She subsequently underwent sequential intramedullary nailing of her tibiae. Both tibiae were in valgus alignment; however, this did not preclude nail placement. The nails deformed upon insertion into the sclerotic canals to conform to the deformation. Post operatively the tibiae united and patient was relieved of her symptoms. Conclusion: Bilateral intramedullary nailing of chronic bilateral tibial stress fractures should be considered as a treatment option for all patients, not just high-level athletes, who fail a trial of conservative management. Additionally, mild to moderate tibial malalignment does not necessarily preclude tibial nailing as the smaller nails placed in sclerotic canals will likely deform on insertion and conform to the canal. Keywords: Bilateral intramedullary nailing, bilateral tibial stress fractures, valgus

  4. Awake operative videothoracoscopic pulmonary resections.

    Science.gov (United States)

    Pompeo, Eugenio; Mineo, Tommaso C

    2008-08-01

    moderately increasing the depth of sedation while maintaining spontaneous breathing. Finally, as long as the physiologic impact of awake metastasectomy is definitively elucidated, the authors believe this modality should be used for unilateral procedures, while deserving a staged bilateral approach for bilateral lung metastasectomy. Avoidance of general anesthesia results in a faster recovery with immediate return to many daily life activities, including drinking, eating, and walking, and a reduction in hospital stay and procedure-related costs. If confirmed with future studies, these results could advocate earlier resection of peripheral solitary pulmonary nodules, reducing the risk for delaying a diagnosis of unexpected pulmonary malignancy. Furthermore, potential new frontiers of awake thoracoscopic surgery might include assessment of feasibility and safety of anatomic resections in properly selected instances. Ethical and economical concerns push remorselessly for less frequent and less-invasive surgery. Administrators advocate minimal hospitalization and cost-saving treatments, whereas patients ultimately ask for appropriate health care. Thoracic surgeons of the third millennium must accept the challenge of this dynamic and rapidly evolving scenario without loosing the right root, which probably lays just between well-established conventional surgery techniques and newly available advanced technology tools. Awake thoracic surgery will benefit from evidence-based data that are progressively accumulating. Findings will stimulate experts to continue an active clinical investigation in this unpredictably evolving surgical field, which might ultimately lead to a better understanding of cardiorespiratory physiology and effects of the surgical pneumothorax and thoracic epidural anesthesia on perioperative, respiratory function in awake patients. As the Italian architect Renzo Piano recently stated, "Recovering in the past can be reassuring but the future is the only place

  5. Deep venous thrombosis caused by congenital inferior vena cava agenesis and heterozygous factor V Leiden mutation – a case report

    Science.gov (United States)

    Vasco, Pablo Guisado; López, Angel Ruedas; Piñeiro, María Laiño; Rivera, José Ignacio Gallego

    2009-01-01

    The unusual clinical presentation, importance of imaging techniques and role of low molecular weight heparin are described for an initial treatment of thrombosis in inferior vena cava agenesis associated with heterozygous factor V Leiden. The patient, a 36-year-old woman, presented to the emergency room with sudden onset of back pain, swelling of the legs and thighs, and claudication while walking. Abdominal ultrasonography was immediately ordered. Anomalies in vascular blood flow were detected. Computed tomography was performed, and initially showed a complete absence of the infrarenal segment of inferior vena cava caudally to the origin of both renal veins. Treatment with enoxaparin (1 mg/kg twice per day) was started. The patient was discharged and returned to her activities of daily living two weeks after admission. This vascular abnormality is mostly incidentally diagnosed in adults and only a few cases are described as being associated with thrombophilia. PMID:22477517

  6. Pulmonary arterial hypertension : an update

    NARCIS (Netherlands)

    Hoendermis, E. S.

    2011-01-01

    Pulmonary arterial hypertension (PAH), defined as group 1 of the World Heart Organisation (WHO) classification of pulmonary hypertension, is an uncommon disorder of the pulmonary vascular system. It is characterised by an increased pulmonary artery pressure, increased pulmonary vascular resistance a

  7. Mechanisms responsible for pulmonary hypertension

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ Pulmonary hypertension is a pathophysiologic process characterized by progressive elevation of pulmonary vascular resistance and right heart failure, which is a common complication of many diseases. Pulmonary hypertension with no apparent causes (unknown etiology) is termed primary pulmonary hypertension or, more recently, idiopathic pulmonary arterial hypertension (IPAH).

  8. Prognosis of synchronous bilateral breast cancer

    DEFF Research Database (Denmark)

    Holm, Marianne; Tjønneland, Anne; Balslev, Eva;

    2014-01-01

    Currently, no consistent evidence-based guidelines for the management of synchronous bilateral breast cancer (SBBC) exist and it is uncertain how presenting with SBBC affects patients' prognosis. We conducted a review of studies analyzing the association between SBBC and prognosis. The studies...... that reported adjusted effect measures were included in meta-analyses of effect of bilaterality on breast cancer mortality. From 57 initially identified records 17 studies from 11 different countries including 8,050 SBBC patients were included. The quality of the studies varied but was generally low with small...... sample sizes, and lack of consistent, detailed histo-pathological information. When doing meta-analysis on the subgroup of studies that provided adjusted effect estimates on breast cancer mortality (nine studies including 3,631 SBBC cases), we found that bilaterality in itself had a negative impact...

  9. Bilateral Sequential Pneumolabyrinth Resulting from Nose Blowing.

    Science.gov (United States)

    Lee, Joong Seob; Kwon, Sae Young; Kim, Ji Heui; Kim, Hyung-Jong

    2015-12-01

    Pneumolabyrinth describes a condition with entrapped air in the labyrinth and usually occurs in temporal bone fractures that involve the otic capsule. While sporadic cases of bilateral pneumolabyrinth have been reported, cases lacking head trauma are very rare. We report the case of a 43-year-old man who had sudden hearing loss bilaterally after blowing his nose at an interval of 1 year. Although conservative management for the right ear and exploratory tympanotomy with sealing of the possible site of perilymphatic leakage in the left ear were performed, hearing outcome was poor in both ears. To our knowledge, this is the first case of bilateral pneumolabyrinth occurring as a result of nose blowing. PMID:26771019

  10. Langerhans Cell Histiocytosis in Bilateral Mastoid Cavity

    Directory of Open Access Journals (Sweden)

    Kazım Bozdemir

    2013-01-01

    Full Text Available A 39-year-old male was admitted to our clinic with symptoms of headache, dizziness, nausea, otalgia, otorrhea, tinnitus, and hearing loss in both ears for 3 weeks. Physical examination revealed edema in the tympanic membrane and external ear canal, and pain by palpation in the mastoid area bilaterally. There was no nystagmus, and the rest of the physical examination was otherwise normal. Temporal bone high resolution computed tomography (CT showed a lesion causing erosion in the mastoid cortex, tegmen tympani, ossicles, and in the bone covering the sigmoid sinus bilaterally. There was also erosion in the superior semicircular canal and petrous bone on the left side. Cortical mastoidectomy was performed under general anesthesia. Histopathologic examination of the tissue revealed Langerhans cell histiocytosis (LCH. In this paper a case with LCH, presenting with bilateral mastoid involvement which has been rarely reported in the literature, is discussed with the existing literature.

  11. X-ray and high resolution CT findings in pulmonary alveolar proteinosis and their relationship with pulmonary function

    International Nuclear Information System (INIS)

    Objective: To study X-ray and high resolution computed tomograghy (HRCT) features and their relationship with pulmonary function test in pulmonary alveolar proteinosis (PAP). Methods: The findings of X-ray and HRCT and the results of pulmonary function tests in 19 patients with PAP were reviewed retrospectively. X-ray and HRCT scores were rated in the aspects of opacity density, extent, and severity. The relationship between the imaging scores and pulmonary function tests were determined. Results X-ray showed a symmetrical perihilar predominance appearance in patients with PAP. HRCT scan showed bilateral clear-defined pathchy areas of ground-glass opacity and interlobular septal thickening, a pattern commonly characterized as crazy paving. Nineteen PAP respiratory function results were: DLCO/Pred (54.49 ± 16.78)%, FEV1/FVC=(86.00±6.70)%. Chest radiographic extent score and severity score correlated with diffusing capacity (r=-0.661, P1/FVC, r=0.573, P<0.05). Conclusions: In patients with PAP, there is close relationship between X-ray, HRCT findings and pulmonary function. HRCT correlates more closely with pulmonary function. (authors)

  12. Pulmonary Extramedullary Hematopoiesis Involving the Pulmonary Artery

    OpenAIRE

    Varun Monga; Margarida Silverman

    2015-01-01

    Extramedullary hematopoiesis (EMH) occurs as a complication of hematologic disorders such as myelofibrosis, sickle cell anemia and thalassemia. The extramedullary tissue usually involves liver, spleen and lymph nodes, less frequently the chest. We present a recent case of a man with myeloproliferative neoplasm who developed pulmonary hemorrhage secondary to EMH in the lung and pulmonary artery. Radiation therapy was considered the best approach, but it didn’t work and the patient died a week ...

  13. Congenital bilateral perisylvian syndrome with hydrocephalus

    Directory of Open Access Journals (Sweden)

    Salih Hattapoğlu

    2012-12-01

    Full Text Available Congenital bilateral perisylvian syndrome (CBPS, whichis seen by indications of mental retardasyon, epilepsi,speech disorder and pseudobulbar palsy, is a diseasewhich comes up with genetic and non-genetic reasons.Revealing characteristic indications (like polymicrogyriawith MR imaging and clinic indications contributes makingdiagnosis. In present paper, we aimed to present 18month girl case report who diagnosed as CBPS with hydrocephaliindication. J Clin Exp Invest 2012; 3(4: 552-554Key words: Epilepsy, congenital bilateral perisylviansyndrome, polymicrogyria, hydrocephalus, pseudobulbarpalsy

  14. [Bilateral choroidal osteoma--a case report].

    Science.gov (United States)

    Jędrychowska-Jamborska, Justyna; Kulig-Stochmal, Agnieszka; Markiewicz, Anna; Jakubowska, Barbara; Romanowska-Dixon, Bożena

    2014-01-01

    Choroidal osteoma is a an extremely rare (especially located bilaterally), benign, intraocular tumor, the type of choristoma. It occurs between 2-3 decades of life, women are particularly vulnerable. The main complication in 1/3 cases is a subretinal neovascularization which may cause bleeding. The gradually progressive decalcification develops within the tumour over time, which causes atrophy of the retinal pigment epithelium and Bruch's membrane deformity. The article presents a case of a 26-year-old woman with bilateral choroidal osteoma complicated by subretinal hemorrhage; the diagnosis was based on clinical examination (biomicroscopy and indirect ophthalmoscopy) as well as specialised tests including: ultrasonography, optical coherence tomography, and fluorescein angiography.

  15. Bilateral hypertrophic olivary degeneration in Wilson disease

    Energy Technology Data Exchange (ETDEWEB)

    Otto, Josephin; Guenther, Pete; Hoffmann, Karl Titus [Leipzig University Hospital, Leipzig (Greece)

    2013-04-15

    Hypertrophic olivary degeneration resulting from lesions of the dento-rubro-olivary pathway, also called Guillain-Mollaret-triangle, has been described previously in a number of cases. Reports about bilateral hypertrophic olivary degeneration of the inferior olivary nuclei are very limited, and the magnetic resonance imaging findings of hypertrophic olivary degeneration in Wilson disease have not yet been described to the best of our knowledge. Herein, we present the first report of bilateral hypertrophic olivary degeneration diagnosed by magnetic resonance imaging in a patient suffering from Wilson disease.

  16. Amiodarone-Induced Pulmonary Toxicity – A Frequently Missed Complication

    Science.gov (United States)

    Sweidan, Alexander J.; Singh, Navneet K.; Dang, Natasha; Lam, Vinh; Datta, Jyoti

    2016-01-01

    INTRODUCTION Amiodarone is often used in the suppression of tachyarrhythmias. One of the more serious adverse effects includes amiodarone pulmonary toxicity (APT). Several pulmonary diseases can manifest including interstitial pneumonitis, organizing pneumonia, acute respiratory distress syndrome, diffuse alveolar hemorrhage, pulmonary nodules or masses, and pleural effusion. Incidence of APT varies from 5–15% and is correlated to dosage, age of the patient, and preexisting lung disease. DESCRIPTION A 56-year-old male with a past medical history of coronary artery disease and chronic obstructive pulmonary disease was admitted for a coronary artery bypass graft. Post-operatively, the patient was admitted to the ICU for ventilator management and continued to receive his home dose of amiodarone 400 mg orally twice daily, which he had been taking for the past 3 months. The patient was found to be hypoxemic with a PaO2 52 mmHg and bilateral infiltrates on chest x-ray. Patient also complained of new onset dyspnea. Physical exam found bilateral rhonchi with bibasilar crackles and subcutaneous emphysema along the left anterior chest wall. Daily chest x-rays showed worsening of bilateral interstitial infiltrates and pleural effusions. A chest high-resolution computed tomography on post-operative day 3 showed extensive and severe bilateral ground glass opacities. APT was suspected and amiodarone was discontinued. A course of oral prednisone without antibiotics was initiated, and after one week of treatment the chest film cleared, the PaO2 value normalized and dyspnea resolved. DISCUSSION APT occurs via cytotoxic T cells and indirectly by immunological reaction. Typically the lungs manifest a diffuse interstitial pneumonitis with varying degrees of fibrosis. Infiltrates with a ‘ground-glass’ appearance appreciated on HRCT are more definitive than chest x-ray. Pulmonary nodules can be seen, frequently in the upper lobes. These are postulated to be accumulations of

  17. Pulmonary fungal infections after bone marrow transplantation

    International Nuclear Information System (INIS)

    Of 319 pediatric patients treated with bone marrow transplantation (BMT) during a 10-year period, 27 developed pulmonary fungal infections (PFI). Only 2 patients (7%) survived. Twenty-three patients (85%) had been treated with systemic anti-fungal therapy immediately before or at the time of diagnosis. Nineteen patients (70%) were neutropenic, and 4 of the 8 patients who were not neutropenic were being treated with systemic steroids for graft vs. host disease (GVHD). Seven patients (26%) died within 7 days of diagnosis. The diagnosis was made ante-mortem in 9 patients (33%). Radiographic abnormalities were variable. At the onset of chest X-ray (CXR) change, the pulmonary infiltrates were unilateral in 14 patients (52%) and, at diagnosis, bilateral in 18 (66%). At diagnosis the infiltrates were interstitial in 3 patients (11%), alveolar in 20 (74%) and mixed in 4 (15%). Six patients (22%) developed cavitary lesions. The infecting agents were Aspergillus in 21 patients (78%), Candida in 7 (26%), Mucormycosis in 3 (11%), and Fusarium in 1 (4%). Five patients (19%) had mixed fungal infections and 7 (26%) had concurrent cytomegalovirus (CMV) pulmonary infections. Although the radiographic changes are often nonspecific in PFI, alveolar or nodular infiltrates in neutropenic patients or in those being treated for GVHD should strongly suggest a fungal etiology. (orig.)

  18. Effect of pulmonary hypertension on outcome of pulmonary tuberculosis

    OpenAIRE

    Majid Marjani; Parvaneh Baghaei; Majid Malekmohammad; Payam Tabarsi; Babak Sharif-Kashani; , Neda Behzadnia; Davood Mansouri; Mohammad Reza Masjedi; Ali Akbar Velayati

    2014-01-01

    Background: This study performed at the National Research Institute of Tuberculosis and Lung Disease, Tehran, Iran, aimed to evaluate the effect of concomitant pulmonary hypertension on the outcome of pulmonary tuberculosis. Methods: New cases of pulmonary tuberculosis were recruited for the study. Pulmonary hypertension was defined as systolic pulmonary arterial pressure ≥35 mm Hg estimated by transthoracic Doppler echocardiography. We assessed the relationship between pulmonary hypertensi...

  19. Handbook of pulmonary emergencies

    Energy Technology Data Exchange (ETDEWEB)

    Spaquolo, S.V.; Medinger, A

    1986-01-01

    This book presents information on the following topics: clinical assessment of the patient with pulmonary disease; interpretation of arterial blood gases in the emergency patient; life-threatening pneumonia; extrapulmonic ventilatory failure; acute inhalation lung disease; pulmonary edema; near drowning; chest trauma; upper airway emergencies; chronic lung disease with acute respiratory decompensation; acute respiratory failure in the patient with chronic airflow obstruction; asthma; hemoptysis; embolic pulmonary disease; superior vena cava syndrome; catastrophic pleural disease; ventilatory assistance and its complications; and ventilator emergencies.

  20. Cystic pulmonary hydatidosis

    Science.gov (United States)

    Sarkar, Malay; Pathania, Rajnish; Jhobta, Anupam; Thakur, Babu Ram; Chopra, Rajesh

    2016-01-01

    Cystic echinococcosis (CE) is a zoonotic parasitic disease caused by the larval stages of the cestode Echinococcus granulosus. Worldwide, pulmonary hydatid cyst is a significant problem medically, socially, and economically. Surgery is the definitive therapy of pulmonary hydatidosis. Benzimidazoles may be considered in patients with a surgical contraindication. This review will focus on pathogenesis, lifecycle, clinical features, and management of pulmonary hydatid disease. PMID:27051107

  1. How Is Pulmonary Hypertension Diagnosed?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Pulmonary Hypertension Diagnosed? Your doctor will diagnose pulmonary hypertension (PH) ... To Look for the Underlying Cause of Pulmonary Hypertension PH has many causes, so many tests may ...

  2. Liver Disease and Pulmonary Hypertension

    Science.gov (United States)

    Liver Disease Pulmonary & PH Hypertension Did you know that if you have liver disease, you are at risk for pulmonary ... to the liver without cirrhosis. How does liver disease relate to pulmonary hypertension? Liver disease can cause what is known ...

  3. Pulmonary involvement in systemic sclerosis: A clinical profile

    Directory of Open Access Journals (Sweden)

    A S Deepa

    2016-01-01

    Full Text Available Background: Systemic sclerosis is a generalized disorder of connective tissue affecting skin and internal organs. Lung involvement accounts for significant morbidity and is a leading cause of mortality in patients. Objectives: This study intends to study the frequency of occurrence of pulmonary involvement in progressive systemic sclerosis (PSS and to describe the clinical and radiological picture of pulmonary involvement in PSS. Materials and Methods: This was a descriptive cross-sectional study. A detailed history, modified Rodnan score, clinical examination, routine investigation, antinuclear antibody, immuno biot, chest X-ray (CXR, pulmonary function test (PFT, and 6 min walk test (6MWT were performed on all patients. High resolution computed tomography was done on those who consented. Results: Hundred subjects with PSS were included in the study; 90 were females and 10 were males. Common presenting complaints were skin thickening in 98% and Raynaud's phenomenon in 98%. Skin thickening of digits beyond metacarpo phalangeal was seen in 98%, face and neck in 92%, and hands in 92%. Chest wall thickening was seen in 40 subjects (40%. 90 (90% of the studied subjects had pulmonary involvement, longer duration of disease was significantly associated with pulmonary involvement (P < 0.05. Dyspnea, cough, bilateral crepitations, CXR, Borg score, and Rodnan score was found to be significantly associated with severe pulmonary involvement (P < 0.05. Conclusion: The prevalence of pulmonary involvement in this cohort study was 90%. Almost 1/3rd of patients, that is 29 (29% were detected to have pulmonary involvement despite being asymptomatic for respiratory complaints, hence early screening and evaluation is recommended. PFT and 6MWT are noninvasive, cost-effective, and easily available screening tests which can be used in resource-limited settings.

  4. Bilateral Diabetic Papillopathy and Metabolic Control

    DEFF Research Database (Denmark)

    Ostri, Christoffer; Lund-Andersen, Henrik; Sander, Birgit;

    2010-01-01

    patient-years of observation, bilateral diabetic papillopathy developed in 5 patients. During the year preceding this incident, all 5 patients had experienced a decrease in glycosylated hemoglobin A(1c) (HbA(1C)) at a maximum rate of -2.5 (mean) percentage points per quarter year, which was significantly...

  5. Bilateral breast cancer : mammographic and clinical findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Eun Kyung; Oh, Ki Keun; Jun, Hwang Yoon; Lee, Byung Chan; Lee, Kyong Sik; Lee, Yong Hee [Yonsei Univ. College of Medicine, Seoul (Korea, Republic of)

    1997-06-01

    To evaluate the mammographic and clinical features of bilateral breast cancer. We retrospectively reviewed clinical records(n=23) and mammograms (n=15) of 23 patients with bilateral breast cancer. Patients' age, location of the tumor and pathologic staging were determined from clinical records. Mammographic features were classified as spiculated mass, nonspiculated mass, mass with microcalcification, microcalcification only, asymmetric density, and normal. Of the 23 cases of bilateral breast cancer, 8(34.8%) were synchronous and 15(65.2%) were metachronous. Age at diagnosis of cancer in the first breast was between 27 and 59(mean 43) years ; there was no statistically significant difference in mean age between patients with synchronous and metachronous cancer. The mean interval between the diagnosis of each lesion of the metachronous pairs was 9.1 years. In 11 of 23 cases(48%), tumors were locaated in the same quadrant, and in the other 12 cases(52%), they were in different quadrant. At mammography, five of 15 metachronous cancers(33%) were similar in appearance and 10 pairs(67%) were different. In 4 of 23 cases(17%), cancer in the first breast was at stage 0 and stage 1, and in 13 of 23(57%), cancer in the second breast was at this same stage. In bilateral breast cancer, the two breasts frequently show different mammographic features. Cancer of the second breast was at an early stage; this suggest that regular examination and mammography are important and can allow early detection of contralateral breast cancer.

  6. Occipital seizures presenting with bilateral visual loss

    OpenAIRE

    Hadjikoutis S; Sawhney I

    2003-01-01

    Transient visual loss may occur with occipital seizures as an ictal or post-ictal phenomenon. Its duration varies from less than one minute to days, or can be permanent. We describe a 61-year-old man presenting with headache, vomiting and bilateral visual loss. EEG revealed persistent spike discharge in the occipital lobes suggesting occipital seizures. His vision improved with carbamazepine.

  7. Simulations of bilateral energy markets using MATLAB

    International Nuclear Information System (INIS)

    This paper presents simulation results of energy bilateral markets using MATLAB/SIMULINK. The algorithm for congestion management and transmission pricing is implemented by means of DC load flow. The simulation results show how elasticity of market participants to transmission usage charges can affect the overall network usage and its cost. (Author)

  8. Bilateral eyelid edema : Cutis laxa or blepharochalasis?

    NARCIS (Netherlands)

    Braakenburg, A; Nicolai, JPA

    2000-01-01

    A 59-year-old woman with massive bilateral edema of the upper and lower eyelids is presented. The edema occurred suddenly and without provocation. No cause could be identified despite a multitude of examinations. Initially the patient was diagnosed as having blepharochalasis, but later skin biopsy s

  9. A Bilateral Traumatic Hip Obturator Dislocation

    Science.gov (United States)

    Karaarslan, Ahmet Adnan; Acar, Nihat; Karci, Tolga; Sesli, Erhan

    2016-01-01

    A case of a bilateral simultaneous traumatic obturator dislocation of both hip joints in an 18-year-old young man following a traffic accident is presented. We reduced the dislocated femoral heads immediately under general anesthesia followed by passive and active exercises and early full-weight bearing mobilization. After 5 years, the result was excellent. PMID:26977327

  10. Bilateral synchronous rupture of the quadriceps tendon.

    LENUS (Irish Health Repository)

    Ellanti, P

    2012-09-01

    Bilateral simultaneous rupture of the quadriceps tendon is a rare entity. They are often associated with degenerative changes of the tendons and predisposing conditions such as diabetes or excessive steroid use. They most commonly tend to occur in patients of 40 years of age or older.

  11. Fast and Provably Accurate Bilateral Filtering.

    Science.gov (United States)

    Chaudhury, Kunal N; Dabhade, Swapnil D

    2016-06-01

    The bilateral filter is a non-linear filter that uses a range filter along with a spatial filter to perform edge-preserving smoothing of images. A direct computation of the bilateral filter requires O(S) operations per pixel, where S is the size of the support of the spatial filter. In this paper, we present a fast and provably accurate algorithm for approximating the bilateral filter when the range kernel is Gaussian. In particular, for box and Gaussian spatial filters, the proposed algorithm can cut down the complexity to O(1) per pixel for any arbitrary S . The algorithm has a simple implementation involving N+1 spatial filterings, where N is the approximation order. We give a detailed analysis of the filtering accuracy that can be achieved by the proposed approximation in relation to the target bilateral filter. This allows us to estimate the order N required to obtain a given accuracy. We also present comprehensive numerical results to demonstrate that the proposed algorithm is competitive with the state-of-the-art methods in terms of speed and accuracy. PMID:27093722

  12. Bilateral segmental neurofibromatosis diagnosed during pregnancy.

    Science.gov (United States)

    Maldonado Cid, P; Sendagorta Cudós, E; Noguera Morel, L; Beato Merino, M J

    2011-01-01

    Bilateral segmental neurofibromatosis is a rare subtype of neurofibromatosis type 1 defined by lesions affecting a single segment of the body and crossing the midline, with no systemic involvement. We present a case diagnosed during pregnancy because of the characteristic increase in size of the lesions during this period. PMID:21635828

  13. Synchronous bilateral breast cancer in a male

    OpenAIRE

    Rubio Hernández, María Caridad; Díaz Prado, Yenia Ivet; Pérez, Suanly Rodríguez; Díaz, Ronald Rodríguez; Aleaga, Zaili Gutiérrez

    2013-01-01

    Male breast cancer, which represents only 1% of all breast cancers, is occasionally associated with a family history of breast cancer. Sporadic male breast cancers presenting with another primary breast cancer are extremely rare. In this article, we report on a 70-year-old male patient with bilateral multifocal and synchronous breast cancer and without a family history of breast cancer.

  14. Bilateral Facial Nerve Palsy: A Diagnostic Dilemma

    Directory of Open Access Journals (Sweden)

    Sohil Pothiawala

    2012-01-01

    Conclusion. We reinforce the importance of considering the range of differential diagnosis in all cases presenting with bilateral FNP. These patients warrant admission and prompt laboratory and radiological investigation for evaluation of the underlying cause and specific further management as relevant.

  15. "Ostrich sign" indicates bilateral vertebral artery dissection.

    Science.gov (United States)

    Rose, David Z; Husain, M Rizwan

    2012-11-01

    Vertebral artery dissections (VADs) comprise about 2% of ischemic strokes and can be associated with trauma, chiropractic manipulation, motor vehicle collisions, whiplash, amusement park rides, golfing, and other motion-induced injuries to the neck. We present a case of bilateral extracranial VAD as a complication of conducting an orchestra. To our knowledge, this has not been documented in the literature. Conceivably, vigorous neck twisting in an inexperienced, amateur conductor may place excessive rotational forces upon mobile portions of the verterbral arteries, tear the intima, deposit subintimal blood that extends longitudinally, and cause neck pain and/or posterior fossa ischemic symptoms. Magnetic resonance angiography examinations of axially oriented slices of bilateral VADs resemble the face of an ostrich. This observation is similar to the "puppy sign," in which bilateral internal carotid artery dissections resemble the face of a dog. Craniocervical dissections of either the carotid or vertebral arteries have the potential to form an aneurysm, cause artery-to-artery embolism, or completely occlude the parent artery, resulting in an ischemic stroke. Because bilateral VADs in axial magnetic resonance angiographic sections stand out like the eyes of an ostrich, and because the fast identification of VADs is so critical, we eponymize this image the "ostrich sign."

  16. Severe Hyperkalemia and Bilateral Adrenal Metastasis

    Directory of Open Access Journals (Sweden)

    Michael Nagler

    2009-01-01

    Full Text Available Adrenal metastases are a common finding in metastatic lung and breast cancer. Often there are no clinical symptoms suggesting them. In this paper, we present a case of a 66-year-old man with metastatic lung cancer suffering from severe hyperkaliemia due to hypoaldosteronism as a result of bilateral adrenal metastasis.

  17. Bilateral carpal tunnel syndrome in Graves' disease.

    Science.gov (United States)

    Manganelli, P; Pavesi, G; Salaffi, F

    1987-01-01

    Carpal tunnel syndrome (CTS) may be associated with endocrinopathies, such as hypothyroidism and acromegaly. A direct relationship between CTS and hyperthyroidism has recently been suggested. We now report a case in which bilateral CTS developed after treatment of Graves' disease, thus, questioning the possibility of a relationship between these two disease processes.

  18. Regulation of pulmonary inflammation by mesenchymal cells

    NARCIS (Netherlands)

    Alkhouri, Hatem; Poppinga, Wilfred Jelco; Tania, Navessa Padma; Ammit, Alaina; Schuliga, Michael

    2014-01-01

    Pulmonary inflammation and tissue remodelling are common elements of chronic respiratory diseases such as asthma, chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), and pulmonary hypertension (PH). In disease, pulmonary mesenchymal cells not only contribute to tissue

  19. Dermatomyositis masquerading as pulmonary embolism

    Directory of Open Access Journals (Sweden)

    Mroz RM

    2009-12-01

    Full Text Available Abstract A 61-year-old Caucasian was admitted to Department of Chest Diseases and Tuberculosis, Medical University of Bialystok, Poland for progressive muscle weakness and weight loss. Eighteen months prior to admission, the patient had been diagnosed with pulmonary embolism. At that point he was started on Enoxaparin QD. Past medical history was unremarkable. In the interim, the patient developed fever, myalgia and progressive dyspnea. Physical examination on admission revealed a rash on his upper torso and back, and the extensor surfaces of all four extremities. Laboratory values included CPK 8229, MB fraction 219, LDH 981. Chest X-ray and CT scan revealed bilateral patchy consolidations and ground-glass opacities. EMG was consistent with myositis. The patient was started on solumedrol 40 mg i.v., b.i.d., and then switched to prednisone 40 mg b.i.d. His symptoms and muscle strength improved remarkably. The patient was discharged with prednisone with an outpatient follow up.

  20. Fetal development of the corpus callosum: Insights from a 3T DTI and tractography study in a patient with segmental callosal agenesis.

    Science.gov (United States)

    Scola, Elisa; Sirgiovanni, Ida; Avignone, Sabrina; Cinnante, Claudia Maria; Biffi, Riccardo; Fumagalli, Monica; Triulzi, Fabio

    2016-10-01

    Commissural embryology mechanisms are not yet completely understood. The study and comprehension of callosal dysgenesis can provide remarkable insights into embryonic or fetal commissural development. The diffusion tensor imaging (DTI) technique allows the in vivo analyses of the white-matter microstructure and is a valid tool to clarify the disturbances of brain connections in patients with dysgenesis of the corpus callosum (CC). The segmental callosal agenesis (SCAG) is a rare partial agenesis of the corpus callosum (ACC). In a newborn with SCAG the DTI and tractography analyses proved that the CC was made of two separate segments consisting respectively of the ventral part in the genu and body of the CC, connecting the frontal lobes, and the dorsal part in the CC splenium and the attached hippocampal commissure (HC), connecting the parietal lobes and the fornix. These findings support the embryological thesis of a separated origin of the ventral and the dorsal parts of the CC. PMID:27549148

  1. Laparoscopic total extraperitoneal repair of preoperatively diagnosed bilateral obturator and incidental bilateral femoral herniae.

    Science.gov (United States)

    Malik, Muhammad Usman; Connelly, Tara M; Hamid, Mustafa; Pretorius, Frederik

    2016-01-01

    Obturator hernia (OH), a rare type of hernia, is associated with high morbidity and mortality. Diagnosis is often delayed as clinical symptoms are typically non-specific. OH is frequently associated with other occult inguinopelvic herniae. Early diagnosis is vital to decrease morbidity and mortality. We report the case of a 75-year-old woman who presented to the surgical outpatients' department with non-specific bilateral groin pain radiating to the thighs. CT of the pelvis demonstrated bilateral OH with no radiological evidence of bowel obstruction. Semiurgent elective laparoscopic total extraperitoneal mesh repair was performed. Intraoperative findings confirmed bilateral obturator herniae as well as incidental bilateral femoral herniae. This case highlights the need for a high index of suspicion for such concomitant hernias that, in the presence of OH, may only be identified intraoperatively. PMID:27113790

  2. Bilateral Facial Paralysis Caused by Bilateral Temporal Bone Fracture: A Case Report and a Literature Review

    Directory of Open Access Journals (Sweden)

    Sultan Şevik Eliçora

    2015-01-01

    Full Text Available Bilateral facial paralysis caused by bilateral temporal bone fracture is a rare clinical entity, with seven cases reported in the literature to date. In this paper, we describe a 40-year-old male patient with bilateral facial paralysis and hearing loss that developed after an occupational accident. On physical examination, House-Brackmann (HB facial paralysis of grade 6 was observed on the right side and HB grade 5 paralysis on the left. Upon temporal bone computed tomography (CT examination, a fracture line exhibiting transverse progression was observed in both petrous temporal bones. Our patient underwent transmastoid facial decompression surgery of the right ear. The patient refused a left-side operation. Such patients require extensive monitoring in intensive care units because the presence of multiple injuries means that facial functions are often very difficult to evaluate. Therefore, delays may ensue in both diagnosis and treatment of bilateral facial paralysis.

  3. Agenesis of the internal carotid artery with a trans-sellar anastomosis: CT and MRI findings in late-onset congenital hypopituitarism

    Energy Technology Data Exchange (ETDEWEB)

    Mellado, J.M.; Merino, X.; Ramos, A.; Salvado, E.; Sauri, A. [Inst. de Diagnostic per la Imatge, Centre de Ressonancia Magnetica de Tarragona (Spain)

    2001-03-01

    A 29-year-old woman with a history of hypothyroidism since early childhood developed hypopituitarism. CT and MRI revealed anterior pituitary hypoplasia, an ectopic posterior lobe, a Chiari I malformation and agenesis of the right internal carotid artery with a trans-sellar anastomosis. This constellation of findings constitutes a previously unreported association in congenital hypopituitarism of late onset. The usefulness of imaging modalities and the pathogenic implications are also discussed. (orig.)

  4. An Infant Born to a Mother with Gestational Diabetes Presenting with 49,XXXXY Syndrome and Renal Agenesis-A Case Report

    OpenAIRE

    Sumathipala, Dulika; Gamage, Thilini; Wijesiriwardena, Bandula; Jayasekara, Rohan W.; Dissanayake, Vajira H. W.

    2012-01-01

    49,XXXXY is a rare sex chromosome polysomy with an incidence of 1 in 85 000 male births. It has a characteristic triad of mental retardation, skeletal malformation and hypogonadism. This is the first case report of a child with 49,XXXXY syndrome and renal agenesis. This child was referred for genetic testing at 14 years of age due to facial dysmorphism and hypergonadotropic hypogonadism. He had coarse facial features, cryptorchidism of the right testis, genu valgus deformities, and patent duc...

  5. A 54 Mb 11qter duplication and 0.9 Mb 1q44 deletion in a child with laryngomalacia and agenesis of corpus callosum

    Directory of Open Access Journals (Sweden)

    Lall Meena

    2011-09-01

    Full Text Available Abstract Background Partial Trisomy 11q syndrome (or Duplication 11q has defined clinical features and is documented as a rare syndrome by National Organization of Rare Disorders (NORD. Deletion 1q44 (or Monosomy 1q44 is a well-defined syndrome, but there is controversy about the genes lying in 1q44 region, responsible for agenesis of the corpus callosum. We report a female child with the rare Partial Trisomy 11q syndrome and Deletion 1q44 syndrome. The genomic imbalance in the proband was used for molecular characterization of the critical genes in 1q44 region for agenesis of corpus callosum. Some genes in 11q14q25 may be responsible for laryngomalacia. Results We report a female child with dysmorphic features, microcephaly, growth retardation, seizures, acyanotic heart disease, and hand and foot deformities. She had agenesis of corpus callosum, laryngomalacia, anterior ectopic anus, esophageal reflux and respiratory distress. Chromosome analysis revealed a derivative chromosome 1. Her karyotype was 46,XX,der(1t(1;11(q44;q14pat. The mother had a normal karyotype and the karyotype of the father was 46,XY,t(1;11(q44;q14. SNP array analysis showed that the proband had a 54 Mb duplication of 11q14q25 and a 0.9 Mb deletion of the submicroscopic subtelomeric 1q44 region. Fluorescence Insitu Hybridisation confirmed the duplication of 11qter and deletion of 1qter. Conclusion Laryngomalacia or obstruction of the upper airway is the outcome of increased dosage of some genes due to Partial Trisomy 11q Syndrome. In association with other phenotypic features, agenesis of corpus callosum appears to be a landmark phenotype for Deletion 1q44 syndrome, the critical genes lying proximal to SMYD3 in 1q44 region.

  6. Whole genome sequencing reveals novel non-synonymous mutation in ectodysplasin A (EDA) associated with non-syndromic X-linked dominant congenital tooth agenesis.

    Science.gov (United States)

    Sarkar, Tanmoy; Bansal, Rajesh; Das, Parimal

    2014-01-01

    Congenital tooth agenesis in human is characterized by failure of tooth development during tooth organogenesis. 300 genes in mouse and 30 genes in human so far have been known to regulate tooth development. However, candidature of only 5 genes viz. PAX9, MSX1, AXIN2, WNT10A and EDA have been experimentally established for congenitally missing teeth like hypodontia and oligodontia. In this study an Indian family with multiple congenital tooth agenesis was identified. Pattern of inheritance was apparently autosomal dominant type with a rare possibility to be X-linked. Whole genome sequencing of two affected individuals was carried out which revealed 119 novel non-synonymous single nucleotide variations (SNVs) distributed among 117 genes. Out of these only one variation (c.956G>T) located at exon 9 of X-linked EDA gene was considered as pathogenic and validated among all the affected and unaffected family members and unrelated controls. This variation leads to p.Ser319Ile change in the TNF homology domain of EDA (transcript variant 1) protein. In silico analysis predicts that this Ser319 is well conserved across different vertebrate species and a part of putative receptor binding site. Structure based homology modeling predicts that this amino acid residue along with four other amino acid residues nearby, those when mutated known to cause selective tooth agenesis, form a cluster that may have functional significance. Taken together these results suggest that c.956G>T (p.Ser319Ile) mutation plausibly reduces the receptor binding activity of EDA leading to distinct tooth agenesis in this family. PMID:25203534

  7. Whole genome sequencing reveals novel non-synonymous mutation in ectodysplasin A (EDA associated with non-syndromic X-linked dominant congenital tooth agenesis.

    Directory of Open Access Journals (Sweden)

    Tanmoy Sarkar

    Full Text Available Congenital tooth agenesis in human is characterized by failure of tooth development during tooth organogenesis. 300 genes in mouse and 30 genes in human so far have been known to regulate tooth development. However, candidature of only 5 genes viz. PAX9, MSX1, AXIN2, WNT10A and EDA have been experimentally established for congenitally missing teeth like hypodontia and oligodontia. In this study an Indian family with multiple congenital tooth agenesis was identified. Pattern of inheritance was apparently autosomal dominant type with a rare possibility to be X-linked. Whole genome sequencing of two affected individuals was carried out which revealed 119 novel non-synonymous single nucleotide variations (SNVs distributed among 117 genes. Out of these only one variation (c.956G>T located at exon 9 of X-linked EDA gene was considered as pathogenic and validated among all the affected and unaffected family members and unrelated controls. This variation leads to p.Ser319Ile change in the TNF homology domain of EDA (transcript variant 1 protein. In silico analysis predicts that this Ser319 is well conserved across different vertebrate species and a part of putative receptor binding site. Structure based homology modeling predicts that this amino acid residue along with four other amino acid residues nearby, those when mutated known to cause selective tooth agenesis, form a cluster that may have functional significance. Taken together these results suggest that c.956G>T (p.Ser319Ile mutation plausibly reduces the receptor binding activity of EDA leading to distinct tooth agenesis in this family.

  8. Medical image of the week: bilateral pneumothorax, pneumomediastinum, and massive subcutaneous emphysema

    Directory of Open Access Journals (Sweden)

    Ahmed Z

    2014-03-01

    Full Text Available No abstract available. Article truncated at 150 words. An 80 year old man with chronic obstructive pulmonary disease (COPD presented to the emergency department with respiratory distress and poor oxygen saturation. Physical exam revealed an obese male in respiratory distress with poor air entry bilaterally and scattered wheezing. His chest, neck, tongue, and lips were swollen. The patient was intubated for respiratory failure, felt to be due to angioedema. His oxygen saturation immediately improved, however the patient developed progressive swelling throughout his body including his eyelids, fingers and toes. Diffuse crepitus was felt on palpation. Chest radiography and computed tomography (CT of the chest revealed large bilateral pneumothoraces, pneumomediastinum, and subcutaneous emphysema (Figure 1. Bilateral chest tubes were inserted with re-expansion of both lungs. Over the next several days his respiratory parameters improved, with full re-expansion of the lungs and reabsorption of the pneumomediastinum and subcutaneous emphysema. The patient was extubated successfully and was discharged in good health. ...

  9. Evaluation of Tl-201 lung uptake and impairment of pulmonary perfusion on scintigraphies in pulmonary tuberculosis

    Energy Technology Data Exchange (ETDEWEB)

    Fujii, Tadashige [Shinshu Univ., Matsumoto, Nagano (Japan). School of Allied Medical Sciences; Tanaka, Masao; Koizumi, Tomonori; Kubo, Keishi

    2000-02-01

    Tl-201 lung uptake in 74 patients (85 lesions) and pulmonary perfusion in 105 patients were studied to evaluate clinical usefulness of Tl-201 lung uptake and perfusion lung scintigraphy in pulmonary tuberculosis, using a scintillation camera with a mini-computer system. As indices of Tl-201 lung uptake, lung (lesion) to upper mediastinum uptake ratio (L/M) and visual grading were used. L/M in pulmonary tuberculosis was 1.96{+-}0.66, which was significantly larger than 1.04{+-}0.24 in healthy controls and lower than that in heart diseases with left heart failure and idiopathic interstitial pneumonia, and showed no significant differences with that in acute pneumonia, pyothorax, primary lung cancer and malignant mediastinal tumor. L/M in pulmonary tuberculosis did not correlate with CRP, erythrocyte sedimentation rate, Gaffky number of sputum and body temperature. It correlated with the type of pulmonary tuberculosis according to the Gakken Classification reflecting the disease activity. It was larger in the exudative type, caseo-infiltrative one, disseminated one, one with cavity in infiltrative lesion than the fibro-caseous one. On perfusion lung scintigram, impairment of pulmonary perfusion larger than area of the entire unilateral lung was observed in 68 cases (64.8%). Area of hypoperfused lung field, which correlated with % vital capacity (r=0.60, p=0.0002) and PaO{sub 2} (r=0.39,p=0.0024), was significantly larger in patients with silicosis and those with bilateral pleural involvements such as pleural callosity than in those with type III according to the Gakkai Classification. Most of the patients showed decreased pulmonary perfusion and Tl-201 accumulation of which grade reflects the disease activity in active tuberculous lesion. Patients with miliary tuberculosis and those with silicotuberculosis showed diffuse Tl-201 accumulation in the both lungs. Tl-201 lung scintigraphy seems to be useful for visualizing active tuberculous lesions, particularly the ones

  10. Evaluation of Tl-201 lung uptake and impairment of pulmonary perfusion on scintigraphies in pulmonary tuberculosis

    International Nuclear Information System (INIS)

    Tl-201 lung uptake in 74 patients (85 lesions) and pulmonary perfusion in 105 patients were studied to evaluate clinical usefulness of Tl-201 lung uptake and perfusion lung scintigraphy in pulmonary tuberculosis, using a scintillation camera with a mini-computer system. As indices of Tl-201 lung uptake, lung (lesion) to upper mediastinum uptake ratio (L/M) and visual grading were used. L/M in pulmonary tuberculosis was 1.96±0.66, which was significantly larger than 1.04±0.24 in healthy controls and lower than that in heart diseases with left heart failure and idiopathic interstitial pneumonia, and showed no significant differences with that in acute pneumonia, pyothorax, primary lung cancer and malignant mediastinal tumor. L/M in pulmonary tuberculosis did not correlate with CRP, erythrocyte sedimentation rate, Gaffky number of sputum and body temperature. It correlated with the type of pulmonary tuberculosis according to the Gakken Classification reflecting the disease activity. It was larger in the exudative type, caseo-infiltrative one, disseminated one, one with cavity in infiltrative lesion than the fibro-caseous one. On perfusion lung scintigram, impairment of pulmonary perfusion larger than area of the entire unilateral lung was observed in 68 cases (64.8%). Area of hypoperfused lung field, which correlated with % vital capacity (r=0.60, p=0.0002) and PaO2 (r=0.39,p=0.0024), was significantly larger in patients with silicosis and those with bilateral pleural involvements such as pleural callosity than in those with type III according to the Gakkai Classification. Most of the patients showed decreased pulmonary perfusion and Tl-201 accumulation of which grade reflects the disease activity in active tuberculous lesion. Patients with miliary tuberculosis and those with silicotuberculosis showed diffuse Tl-201 accumulation in the both lungs. Tl-201 lung scintigraphy seems to be useful for visualizing active tuberculous lesions, particularly the ones that could

  11. AB 73. Pulmonary hyalinizing granuloma: case report

    Science.gov (United States)

    Papaemmanouil, Stiliani; Cheva, Aggeliki; Dimitriadis, John; Sakkas, Leonidas

    2012-01-01

    Background Pulmonary hyalinizing granuloma is an unusual condition, characterized by fibrosing nodules, consisting of the either unilateral or bilateral central whorled deposits of lamellar collagen hyaline. Although the etiology is uncertain there are evidences that suggest an autoimmune origin. Clinically and with imaging techniques it may be minic primary or metastatic carcinoma or nodular amyloidosis. Patients and methods We report case of 74 years old woman who admitted in our hospital with cough, dyspnea and chest pain. A CT scan of the chest revealed a mass located in the middle lobe of the right lung. The patient underwent thoracotomy for surgical excision of the mass macroscopic examination of the excised lobe revealed a well-circumscribed firm, grayish-white mass, measuring 3.1 cm × 2 cm × 1.3 cm. The sections were examined with H+E, PAS, and Congo-Red and followed by immunohistochemical study for Amyloid A and P, CD138, κ and λ light chains. Results The microscopic examination with H+E showed that the lung parenchyma presented extensive hyaline fibrosis PAS positive, areas of calcification and foci of bone metaplasia, accompanied by an inflammatory reaction. Congo-Red stain was positive but screening for birefringent material under polarized light was negative. Amyloid A and P were negative. The plasma calls were positive for CD138, κ and λ light chains. These histological findings were consistent with diagnosis of pulmonary hyalinizing granuloma. Conclusions The pulmonary hyalinizing granuloma should be considered in the differential diagnosis of pulmonary nodules or masses, even when they are cavitary or contain calcifications.

  12. Pulmonary alveolar microlithiasis

    OpenAIRE

    Gayathri Devi, H.J.; K N Mohan Rao; Prathima, K. M.; Das, Jayanth K.

    2011-01-01

    Pulmonary alveolar microlithiasis is a rare disease of unknown cause. We report a case in a young boy who presented with history of failure to thrive and chest X-ray finding suggestive of miliary mottling. Open lung biopsy revealed pulmonary alveolar microlithiasis.

  13. Pregnancy and pulmonary hypertension

    NARCIS (Netherlands)

    Pieper, Petronella G.; Lameijer, Heleen; Hoendermis, Elke S.

    2014-01-01

    Pulmonary hypertension during pregnancy is associated with considerable risks of maternal mortality and morbidity. Our systematic review of the literature on the use of targeted treatments for pulmonary arterial hypertension during pregnancy indicates a considerable decrease of mortality since a pre

  14. Pulmonary hypertension CT imaging

    International Nuclear Information System (INIS)

    Full text: The right heart catheterization is the gold standard in the diagnosis and determines the severity of pulmonary hypertension. The significant technical progress of noninvasive diagnostic imaging methods significantly improves the pixel density and spatial resolution in the study of cardiovascular structures, thus changes their role and place in the overall diagnostic plan. Learning points: What is the etiology, clinical manifestation and general pathophysiological disorders in pulmonary hypertension. What are the established diagnostic methods in the diagnosis and follow-up of patients with pulmonary hypertension. What is the recommended protocol for CT scanning for patients with clinically suspected or documented pulmonary hypertension. What are the important diagnostic findings in CT scan of a patient with pulmonary hypertension. Discussion: The prospect of instantaneous complex - anatomical and functional cardiopulmonary and vascular diagnostics seems extremely attractive. The contrast enhanced multislice computed (CT ) and magnetic resonance imaging are very suitable methods for imaging the structures of the right heart, with the possibility of obtaining multiple projections and three-dimensional imaging reconstructions . There are specific morphological features that, if carefully analyzed, provide diagnostic information. Thus, it is possible to avoid or at least reduce the frequency of use of invasive diagnostic cardiac catheterization in patients with pulmonary hypertension. Conclusion: This review focuses on the use of contrast-enhanced CT for comprehensive evaluation of patients with pulmonary hypertension and presents the observed characteristic changes in the chest, lung parenchyma , the structures of the right half of the heart and pulmonary vessels

  15. "Multicystic dysplastic kidney (Potter type II syndrome) and agenesis of corpus callosum (ACC) in two consecutive pregnancies: a possible teratogenic effect of electromagnetic exposure in utero".

    Science.gov (United States)

    Tonni, Gabriele; Azzoni, Daniela; Ventura, Alessandro; Ambrosetti, Fabrizio; De Felice, Claudio

    2008-01-01

    Agenesis of the corpus callosum is found in about 5 per 1,000 births and it is due to maldevelopment or, secondary, to destructive lesions. Multicystic dysplastic kidneys is a consequence of either developmental failure of the mesonephric blastema to form nephrons or to early urinary obstruction due to urethral or ureteric atresia and can be found in about 1 per 1,000 live births. A case of fetal multicystic dysplastic kidney disease (Potter type II syndrome) and complete agenesis of the corpus callosum demonstrated by the presence of Probst bundles associated with colpocephaly occurring in the same mother in her two consecutive pregnancies is reported. Data regarding possible teratogenetic effect due to electromagnetic exposure in utero have also been investigated and raised suspicionus as a potential risk factor. In cases of suspected second trimester ultrasound diagnosis of agenesis of corpus callosum (ACC), the following clinical management should be recommended: fetal karyotype; a second level scan with differentiation between underlying conditions such as hydrocephalus and holoprosencephaly; antenatal MRI to enhance the diagnostic accuracy of possible associated neuronal migration (when possible); and direct demonstration of the presence of the Probst bundles to neurohistology.

  16. Hardware Implementation of a Bilateral Subtraction Filter

    Science.gov (United States)

    Huertas, Andres; Watson, Robert; Villalpando, Carlos; Goldberg, Steven

    2009-01-01

    A bilateral subtraction filter has been implemented as a hardware module in the form of a field-programmable gate array (FPGA). In general, a bilateral subtraction filter is a key subsystem of a high-quality stereoscopic machine vision system that utilizes images that are large and/or dense. Bilateral subtraction filters have been implemented in software on general-purpose computers, but the processing speeds attainable in this way even on computers containing the fastest processors are insufficient for real-time applications. The present FPGA bilateral subtraction filter is intended to accelerate processing to real-time speed and to be a prototype of a link in a stereoscopic-machine- vision processing chain, now under development, that would process large and/or dense images in real time and would be implemented in an FPGA. In terms that are necessarily oversimplified for the sake of brevity, a bilateral subtraction filter is a smoothing, edge-preserving filter for suppressing low-frequency noise. The filter operation amounts to replacing the value for each pixel with a weighted average of the values of that pixel and the neighboring pixels in a predefined neighborhood or window (e.g., a 9 9 window). The filter weights depend partly on pixel values and partly on the window size. The present FPGA implementation of a bilateral subtraction filter utilizes a 9 9 window. This implementation was designed to take advantage of the ability to do many of the component computations in parallel pipelines to enable processing of image data at the rate at which they are generated. The filter can be considered to be divided into the following parts (see figure): a) An image pixel pipeline with a 9 9- pixel window generator, b) An array of processing elements; c) An adder tree; d) A smoothing-and-delaying unit; and e) A subtraction unit. After each 9 9 window is created, the affected pixel data are fed to the processing elements. Each processing element is fed the pixel value for

  17. A review of bilateral training for upper extremity hemiparesis.

    Science.gov (United States)

    Stoykov, Mary Ellen; Corcos, Daniel M

    2009-01-01

    Upper extremity hemiparesis is the most common post-stroke disability. Longitudinal studies have indicated that 30-66% of stroke survivors do not have full arm function 6 months post-stroke. The current gold standard for treatment of mild post-stroke upper limb impairment is constraint-induced therapy but, because of the inclusion criteria, alternative treatments are needed which target more impaired subjects. Bilateral arm training has been investigated as a potential rehabilitation intervention. Bilateral arm training encompasses a number of methods including: (1) bilateral isokinematic training; (2) mirror therapy using bilateral training; (3) device-driven bilateral training; and (4) bilateral motor priming. Neural mechanisms mediating bilateral training are first reviewed. The key bilateral training studies that have demonstrated evidence of efficacy will then be discussed. Finally, conclusions are drawn concerning clinical implications based on the reviewed literature. PMID:19517519

  18. Bilateral cerebellar activation in unilaterally challenged essential tremor

    Directory of Open Access Journals (Sweden)

    Marja Broersma

    2016-01-01

    Conclusions: Our results expand on previous findings of bilateral cerebellar involvement in ET. We have identified specific areas in the bilateral somatomotor regions of the cerebellum: lobules V, VI and VIII.

  19. Extralobar pulmonary sequestration

    Directory of Open Access Journals (Sweden)

    Ulys A

    2011-04-01

    Full Text Available Albertas Ulys, Narimantas Evaldas Samalavicius, Saulius Cicenas, Tadas Petraitis, Mantas Trakymas, Dmitrij Sheinin, Leonid GatijatullinInstitute of Oncology, Vilnius University, Santariskiu, Vilnius, LithuaniaAbstract: Prevalence of pulmonary sequestration accounts for up to 6.4% of all congenital pulmonary malformations. We report on a 40-year-old woman who underwent excision of an aberrant solid retroperitoneal mass in the left subdiaphragmatic area. The mass was identified to be an extralobar pulmonary sequestration. The diagnosis could be made without surgery by percutaneous tissue biopsy and imaging. We encourage keeping in mind pulmonary sequestration anomaly presenting as an aberrant retroperitoneal mass. The aim of this case report is to increase awareness about the condition and review the criteria for its definitive diagnosis and treatment.Keywords: retroperitoneal aberrant mass, extralobar pulmonary sequestration

  20. Tratamiento conservador en pacientes con retinoblastoma bilateral

    Directory of Open Access Journals (Sweden)

    Juan C. Suárez

    2008-11-01

    Full Text Available OBJETIVO: comparar el tratamiento convencional del retinoblastoma bilateral, usado hasta hace algunos años, consistente en radioterapia o enucleación bilateral, con el tratamiento conservador actual que incluye termoterapia transpupilar (TTT o TTT/quimioterapia al menos en un ojo, en niños con diagnóstico de retinoblastoma bilateral. DISEÑO: estudio retrospectivo descriptivo. MUESTRA: 20 pacientes con diagnóstico de retinoblastoma bilateral que consultaron al Hospital Universitario San Vicente de Paúl, de Medellín, Colombia, entre 1997 y 2007. MÉTODO: se hizo enucleación del ojo con el tumor de mayor tamaño. En el otro ojo se hizo tratamiento con TTT, con el láser diodo (810 nm, spot amplio, solo o combinado con otras terapias. RESULTADOS: se dividió a los pacientes en dos grupos: 16 pacientes (32 ojos en el grupo 1 tratados conservadoramente y 4 pacientes (8 ojos en el grupo 2 con tratamiento convencional. El rango de edad fue de 1-72 meses en el grupo 1 y de 1-12 meses en el grupo 2. El tiempo de seguimiento fue de 7-67 meses para el grupo 1 y de 13-73 meses para el grupo 2. En el grupo 1 se hizo enucleación de 16 ojos (50%, radioterapia externa de uno (3,1%, quimioterapia más termoterapia de 5 (15,6% y quimioterapia más termoterapia más crioterapia de 10 (31,3%. En todos los pacientes se logró preservar al menos un ojo. En el grupo 2, se enuclearon 7 ojos (87,5% y se hizo radioterapia externa más enucleación en un paciente (12.5%. Además, todos los pacientes recibieron quimioterapia. CONCLUSIÓN: la terapia conservadora actual consistente en tratamiento local (termoterapia, crioterapia o braquiterapia y quimiorreducción permite preservar al menos un ojo y en algunos casos de los dos, muchas veces con buena agudeza visual, en niños con retinoblastoma bilateral; se evitan así la enucleación bilateral y la radioterapia externa usada en el tratamiento convencional con todos sus efectos secundarios. La enucleación contin