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Sample records for bilateral pulmonary agenesis

  1. [Rare case of bilateral pulmonary agenesis and prenatal diagnosis].

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    Veluppillai, C; Jossic, F; Quéré, M-P; Philippe, H-J; Le Vaillant, C

    2014-01-01

    Bilateral pulmonary agenesis (BPA) is a rare congenital lung malformation. The prognosis is severe as it is incompatible with extra-uterine life. Although multiple prenatal imaging modalities are developed, the prenatal diagnosis of BPA remains problematic. We report a case of BPA observed in our unity and for which the diagnosis was not clearly identified during the evaluation. This report illustrates the need to consider all the imaging aspects and particularly during US examination suspecting BPA.

  2. Prenatal diagnosis of bilateral pulmonary agenesis: a case report.

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    Lee, Kyung A; Cho, Jeong Yeon; Lee, Seung Mi; Jun, Jong Kwan; Kang, Jieun; Seo, Jeong-Wook

    2010-01-01

    We report a case of bilateral pulmonary agenesis (BPA), which was suspected during a prenatal US examination and diagnosed by fetal magnetic resonance imaging (MRI). BPA is an extremely rare congenital anomaly and, although many fetal structural defects can be detected with a high degree of confidence after introducing high-resolution US, the prenatal diagnosis of BPA remains problematic. Other thoracic abnormalities, such as a congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, and pulmonary sequestration, should be excluded from the list of possible diagnoses before coming to the conclusion of BPA, because BPA is absolutely incompatible with extrauterine life, and an accurate internal diagnosis can prevent a futile intervention from being performed.

  3. Prenatal Diagnosis of Bilateral Pulmonary Agenesis: a Case Report

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    Lee, Kyung A; Cho, Jeong Yeon; Lee, Seung Mi; Jun, Jong Kwan; Kang, Ji Eun; Seo, Jeong Wook [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2010-02-15

    We report a case of bilateral pulmonary agenesis (BPA), which was suspected during a prenatal US examination and diagnosed by fetal magnetic resonance imaging (MRI). BPA is an extremely rare congenital anomaly and, although many fetal structural defects can be detected with a high degree of confidence after introducing high-resolution US, the prenatal diagnosis of BPA remains problematic. Other thoracic abnormalities, such as a congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, and pulmonary sequestration, should be excluded from the list of possible diagnoses before coming to the conclusion of BPA, because BPA is absolutely incompatible with extrauterine life, and an accurate internal diagnosis can prevent a futile intervention from being performed.

  4. Cloacal dysgenesis sequence with bilateral renal agenesis and normal pulmonary development in twin pregnancy.

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    Jegadeesh, Sundram; Mahajan, Jai Kumar

    2016-01-20

    Cloacal dysgenesis sequence (CDS) is a rare congenital anomaly. It is characterised by a smooth perineum with absence of anal and genitourinary orifices, with reported incidence of 1:50,000-250,000 births. Association with bilateral renal agenesis is still rarer and resultant severe oligohydramnios is associated with pulmonary hypoplasia. Only a few cases of CDS with bilateral renal agenesis have been reported in the English language literature, with associated pulmonary hypoplasia as a default phenomenon. We report a case of CDS and bilateral renal agenesis without associated pulmonary hypoplasia in a twin pregnancy, which, to the best of our knowledge, is the second reported case of this amalgamation.

  5. Pulmonary Agenesis.

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    Chawla, Rakesh K; Madan, Arun; Chawla, Aditya; Arora, Harsh Nandini; Chawla, Kiran

    2015-01-01

    Unilateral opaque lung with ipsilateral mediastinal shift is an uncommon cause of respiratory distress in newborn which can be found on simple radiograph of the chest. Pulmonary agenesis is a rare cause of unilateral opaque lung in the newborn. Nearly 50% cases of pulmonary agenesis are associated with other congenital defects including cardiovascular, skeletal, gastrointestinal or genitourinary systems. We report an infant with agenesis of the right lung associated with other congenital anomalies.

  6. Unilateral pulmonary agenesis.

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    Malcon, Maura Cavada; Malcon, Claudio Mattar; Cavada, Marina Neves; Caruso, Paulo Eduardo Macedo; Real, Lara Flório

    2012-01-01

    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.

  7. Horseshoe lung associated with left-lung hypoplasia, left pulmonary artery sling and bilateral agenesis of upper lobe bronchi.

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    Oguz, Berna; Alan, Serdar; Ozcelik, Ugur; Haliloglu, Mithat

    2009-09-01

    Horseshoe lung, a rare congenital anomaly, is almost always associated with unilateral (usually right-sided) lung hypoplasia, and, in most cases, in conjunction with the scimitar syndrome. We present an 8-month-old boy with horseshoe lung associated with left-lung hypoplasia, left pulmonary artery sling and bilateral agenesis of the upper lobe bronchi, diagnosed by multidetector CT (MDCT) imaging. The study also revealed an anomalous origin of the left vertebral artery as the last branch of the aortic arch, distal to the left subclavian artery, and an anomalous origin of the left common carotid artery from the brachiocephalic trunk. A hemivertebral anomaly of the seventh cervical vertebra was incidentally detected. MDCT with high-quality multiplanar and three-dimensional reconstructions is a noninvasive and rapid technique for detecting the complex combination of vascular, tracheobronchial and parenchymal anomalies, and any potential bone anomalies, in one imaging study.

  8. Horseshoe lung associated with left-lung hypoplasia, left pulmonary artery sling and bilateral agenesis of upper lobe bronchi

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    Oguz, Berna; Haliloglu, Mithat [Hacettepe University Faculty of Medicine, Department of Radiology, Ankara (Turkey); Alan, Serdar; Ozcelik, Ugur [Hacettepe University Faculty of Medicine, Department of Pediatrics, Ankara (Turkey)

    2009-09-15

    Horseshoe lung, a rare congenital anomaly, is almost always associated with unilateral (usually right-sided) lung hypoplasia, and, in most cases, in conjunction with the scimitar syndrome. We present an 8-month-old boy with horseshoe lung associated with left-lung hypoplasia, left pulmonary artery sling and bilateral agenesis of the upper lobe bronchi, diagnosed by multidetector CT (MDCT) imaging. The study also revealed an anomalous origin of the left vertebral artery as the last branch of the aortic arch, distal to the left subclavian artery, and an anomalous origin of the left common carotid artery from the brachiocephalic trunk. A hemivertebral anomaly of the seventh cervical vertebra was incidentally detected. MDCT with high-quality multiplanar and three-dimensional reconstructions is a noninvasive and rapid technique for detecting the complex combination of vascular, tracheobronchial and parenchymal anomalies, and any potential bone anomalies, in one imaging study. (orig.)

  9. Left Pulmonary Agenesis with Right Lung Bronchiectasis in an Adult.

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    Kumar, Prabhat; Tansir, Ghazal; Sasmal, Gargi; Dixit, Juhi; Sahoo, Ratnakar

    2016-09-01

    Pulmonary agenesis is a rare congenital anomaly characterized by the absence of pulmonary parenchyma and vasculature. Bilateral pulmonary agenesis is incompatible with extrauterine life. Unilateral agenesis is often associated with other congenital cardiovascular, genitourinary and gastrointestinal malformations. Right lung agenesis is more frequently associated with congenital anomalies and has poor prognosis as compared to left lung agenesis. Diagnosis is often made in childhood but can be delayed, if the clinician is not aware about this entity. Chest radiograph in unilateral lung agenesis shows opaque hemithorax and these patients are often confused with other common causes of opaque hemithorax like collapse, pleural effusion and diaphragmatic hernia. We report a case of left lung agenesis with right lung bronchiectasis in a middle-aged adult who was treated for tuberculous pleural effusion and was referred to our institute for persistent symptoms despite treatment.

  10. Left Pulmonary Agenesis with Right Lung Bronchiectasis in an Adult

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    Tansir, Ghazal; Sasmal, Gargi; Dixit, Juhi; Sahoo, Ratnakar

    2016-01-01

    Pulmonary agenesis is a rare congenital anomaly characterized by the absence of pulmonary parenchyma and vasculature. Bilateral pulmonary agenesis is incompatible with extrauterine life. Unilateral agenesis is often associated with other congenital cardiovascular, genitourinary and gastrointestinal malformations. Right lung agenesis is more frequently associated with congenital anomalies and has poor prognosis as compared to left lung agenesis. Diagnosis is often made in childhood but can be delayed, if the clinician is not aware about this entity. Chest radiograph in unilateral lung agenesis shows opaque hemithorax and these patients are often confused with other common causes of opaque hemithorax like collapse, pleural effusion and diaphragmatic hernia. We report a case of left lung agenesis with right lung bronchiectasis in a middle-aged adult who was treated for tuberculous pleural effusion and was referred to our institute for persistent symptoms despite treatment. PMID:27790501

  11. Pulmonary agenesis and respiratory failure in childhood.

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    Dinamarco, Paula Vanessa Valverde; Ponce, Cesar Cilento

    2015-01-01

    Pulmonary agenesis (PA) is a rare congenital anomaly, which may be unilateral or bilateral. Unilateral PA may be associated with nonspecific respiratory symptoms. We report the case of 5-month-old infant who presented a normal development until the age of 4 months when a respiratory infection caused an acute respiratory distress syndrome with a fatal outcome. The autopsy findings depicted the right lung agenesis without any other concomitant malformation. Although respiratory symptoms represent frequent complaints in pediatrics, the aim of this study is not only to draw attention to the unilateral pulmonary agenesis as a possible underlying malformation in children who present recurrent and severe respiratory symptoms, but also to report a case diagnosed at autopsy.

  12. Pulmonary agenesis and respiratory failure in childhood

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    Paula Vanessa Valverde Dinamarco

    2015-03-01

    Full Text Available Pulmonary agenesis (PA is a rare congenital anomaly, which may be unilateral or bilateral. Unilateral PA may be associated with nonspecific respiratory symptoms. We report the case of 5-month-old infant who presented a normal development until the age of 4 months when a respiratory infection caused an acute respiratory distress syndrome with a fatal outcome. The autopsy findings depicted the right lung agenesis without any other concomitant malformation. Although respiratory symptoms represent frequent complaints in pediatrics, the aim of this study is not only to draw attention to the unilateral pulmonary agenesis as a possible underlying malformation in children who present recurrent and severe respiratory symptoms, but also to report a case diagnosed at autopsy.

  13. 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.

  14. Array-CGH and quantitative PCR genetic analysis in a case with bilateral hypoplasia of pulmonary arteries and lungs and simultaneous unilateral renal agenesis.

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    Hussein, Kais; Steinemann, Doris; Scholz, Henrike; Menkhaus, Ralf; Feist, Henning; Kreipe, Hans

    2010-08-18

    We describe the clinical course and have characterised anatomically and genetically a unique case of a newborn with bilateral hypoplasia of pulmonary arteries, consecutive extremely hypoplastic lung tissue and associated unilateral renal agenesis. Intrauterine oxygenation by the placenta seemed to have allowed normotrophic body maturity but immediately after delivery, in the third trimester, progressive hypoxemia developed and the newborn succumbed to acute respiratory failure. Genetic analysis by array-based comparative genomic hybridisation and quantitative PCR revealed duplication of 1p21, which, however, might not be the disease causing aberration. This case might represent an extreme form of previously reported, rare cases with simultaneous dysorganogenesis of lungs and kidneys.

  15. Mitral valve replacement in an adult with left pulmonary agenesis.

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    Saskin, Huseyin; Idiz, Mustafa; Duzyol, Cagri; Macika, Huseyin; Aksoy, Rezan

    2015-06-26

    Pulmonary agenesis is associated with the absence of pulmonary vessels, bronchi, or parenchyma. This condition usually occurs between the 4th and 5th week of gestation during the embryonic phase. Etiopathogenic factors associated with pulmonary agenesis are not fully understood. In the literature, genetic and teratogenic factors, viral infections, and vitamin-A deficiency are shown to be associated with pulmonary agenesis [Malcon 2012]. This condition may be seen unilaterally or bilaterally. Although the precise rate of incidence is unknown, it is estimated to occur in one of every 10,000 to 12,000 live births [Yetim 2011]. There is a 1.3:1 female predominance with unilateral agenesis [Halilbasic 2013].

  16. Isolated Bilateral Congenital Iris Sphincter Agenesis

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    Aparna Rao

    2011-01-01

    Full Text Available Purpose. We herein report a patient with bilateral congenital total iris sphincter agenesis with no other abnormality detected on systemic examination. Methods. A 24-year-old laborer presented to us for a routine checkup with complaint of photophobia and inability to work under sunlight. Examination revealed bilateral absence of sphincter and 6.5 mm pupil in both eyes in the undilated state. Results. Accommodation was poor in both eyes. Systemic examination was within normal limits. He was prescribed bifocal photochromic glasses for constant wear. Conclusions. Congenital sphincter agenesis can occur in an isolated form without systemic abnormalities which can be managed conservatively.

  17. [Left pulmonary agenesis diagnosed late].

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    Deleanu, Oana; Pătraşcu, Natalia; Nebunoiu, Ana-Maria; Vintilă, V; Ulmeanu, Ruxandra; Mihălţan, F D

    2010-01-01

    We present the case of a 51 years old female-patient, with severe dextroscoliosis, having like unique symptom progressive dyspnea. The blood samples reveals polycythemia, the radiological exam shows the opacification of 2/3 of the left thorax, the absence of the lung structure in the other 1/3, the deviation of the mediastinum, and dextroscoliosis; the computed tomography reveals the absence of the left lung artery and the left airways, compensatory hyperinflation of the right lung and dilatation of the trunk and right pulmonary artery; the bronchoscopy does not visualize the carina or the left main bronchus, typical for pulmonary agenesis. Echocardiography confirmed the absence of left pulmonary artery and shows mild pulmonary hypertension (systolic pressure in the pulmonary artery of 33 mmHg) with dilatation of the right cavities, but good cinetics. We face a case of pulmonary agenesis lately diagnosed, with modest functional cardiologic implications, limited therapeutic options and good survival, justified by the late appearance of the pulmonary hypertension of low severity and without worsening in time.

  18. [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.

  19. Unilateral right pulmonary agenesis in adulthood.

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    Gunbey, Hediye Pinar; Gunbey, Emre; Sayit, Asli Tanrivermis; Bulut, Taner

    2014-06-01

    Congenital malformations of the lung, which may vary in degrees of severity, are very rare diseases. Pulmonary artery agenesis is a rare anomaly that may occur during the early involution of the proximal portions of the sixth aortic arch, during embryological development of the heart. This agenesis may be accompained by a complete or partial absence of the lung and its bronchus on the same side, which is diagnosed as pulmonary agenesis. In the great majority of the cases, the diagnosis is usually made at or soon after birth and it can be associated with multiple anomalies. However, extremely rare asymptomatic cases may go unnoticed until adulthood. We are presenting a patient with unilateral right pulmonary agenesis, who survived through adulthood without any symptoms and other congenital anomalies. The multislice computed tomography findings and differential diagnoses have been discussed.

  20. Pulmonary Artery Agenesis: A Case Series

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    Meltem Ağca

    2015-04-01

    Full Text Available Pulmonary artery agenesis is a rare congenital abnormality in which atresia was encountered in the short segment of the right or left pulmonary arteries. It can be isolated or associated with cardiac abnormalities such as tetralogy of Fallot, septal defects or pulmonary stenosis.The majority of cases are diagnosed in childhood whereas some cases yield no symptoms until adulthood. We evaluated retrospectively 5 pulmonary artery agenesis cases diagnosed in our clinics between 1998-2010 with respect to the literature.

  1. 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.)

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

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    Amir Halilbasic; Fahrija Skokic; Nesad Hotic; Edin Husaric; Gordana Radoja; Selma Muratovic; Nermina Dedic; Meliha Halilbasic

    2013-01-01

    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.

  3. 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.

  4. Fetal MR imaging diagnosis of pulmonary agenesis.

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    Kuwashima, Shigeko; Kaji, Yasushi

    2010-01-01

    A woman was referred to our institution with an ultrasound (US) suggestive of right-sided heart in fetus at 34 weeks' gestation. Magnetic resonance (MR) imaging revealed right-sided heart, small right hemithorax, and completely absent right main bronchus and right pulmonary artery. From our experience with this case, we point out 5 important MR imaging findings needed for prenatal diagnosis of pulmonary agenesis. Fetal MR imaging also provided information about anomalies of other organs.

  5. Pulmonary agenesis associated with dextrocardia, sternal defects, and ectopic kidney.

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    Eroglu, Atilla; Alper, Fatih; Turkyilmaz, Atila; Karaoglanoglu, Nurettin; Okur, Adnan

    2005-12-01

    Pulmonary agenesis is a rare embryological defect, usually unilateral, and is associated with skeletal, cardiovascular, and other anomalies. A 22-month-old girl was admitted to our clinic with recurrent pulmonary infections. A chest X-ray and multidetector computed tomography (MDCT) revealed pulmonary agenesis with dextrocardia, sternal defects, and a right pelvic ectopic kidney. We report on the first known case of right-sided pulmonary agenesis combined with isolated dextrocardia, sternal defects, and an ectopic kidney. The importance of MDCT for the diagnosis of pulmonary agenesis and associated other anomalies is demonstrated.

  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.

  7. Unilateral pulmonary agenesis associated with tracheal stenosis : a case report

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    Lee, Yong Suk; Yoon, Chong Hyun; Kim, Kyung Sook; Kim, Ki Soo; Pi, Soo Young [Univ. of Ulsan, Ulsan (Korea, Republic of). Colle. of Medicine

    1998-02-01

    Unilateral pulmonary agenesis is a rare congenital anomaly and is frequently associated with other congenital anomalies. We report a case of left pulmonary agenesis associated with congenital tracheal stenosis in a newborn infant. Simple chest radiographs showed an overinflate right lung and mediastinal shifting to the left side. Chest ST and reconstructed three-dimensional images showed left pulmonary agenesis and tracheal stenosis. These anomalies of the tracheobronchial system were confirmed by bronchography. (author). 10 refs., 3 figs.

  8. 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

  9. Right-side pulmonary agenesis with atrial septal defect in adult.

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    Das Shukla, Amitabh; Agrawal, Neha; Chandra, Alok; Anantha, Shreenivasa; Chaudhary, Abhinav

    2016-04-01

    Pulmonary agenesis, a rare congenital condition, is incompatible with life when present bilateral, while unilateral agenesis is usually detected in infancy or early childhood. Rare asymptomatic patients may reach adulthood undiagnosed, with signs mimicking common conditions presenting as radiopaque hemithorax with ipsilateral mediastinal shift. Here, we describe a case of a young lady, with history of consanguinity, who presented with complaints, suggestive of lower respiratory tract infection, and was investigated and diagnosed to be a case of right-side pulmonary agenesis with large ostium secondum atrial septal defect. Our present case emphasizes the importance of presence of pulmonary agenesis with cardiac congenital anomaly, remaining asymptomatic until adulthood, particularly in patients born of parents with consanguineous marriages.

  10. 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.

  11. 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...

  12. Pulmonary Artery Agenesis with Bronchial Asthma

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    Hassan Ghobadi

    2014-05-01

    Full Text Available Unilateral Pulmonary Artery Agenesis (UPAA is a rare congenital anomaly during the 4 th  week of gestational age. It is defined as an absence of pulmonary parenchyma and its supporting artery. A 9-year-old girl was admitted to our hospital because of chronic cough. Chest examination showed a decrement in lung sound of right hemi-thorax with expiratory wheeze. Chest radiography (CXR revealed a semi-opaque right hemi-thorax. Chest CT with intra-venous contrast demonstrated absence of the right pulmonary artery and lung parenchyma with hyper-inflated left lung and dextro-position of mediastinum. This case emphasizes that in patients with respiratory compliant and chronic cough CXR must be done to rule out similar diagnosis other than asthma.    

  13. Association of pulmonary artery agenesis and hypoplasia of the lung.

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    Vitiello, Renato; Pisanti, Chiara; Pisanti, Antonello; Silberbach, Michael

    2006-09-01

    Pulmonary artery agenesis and hypoplasia of the homolateral lung occasionally occurs as an isolated lesion, but more often has associated congenital cardiac anomalies. We present a case where pulmonary artery agenesis was the sole lesion in an asymptomatic child. Pulmonary artery agenesis should be suspected in asymptomatic patients if a plain chest X-ray shows asymmetric lung fields, lung hypoplasia, or hyperinflation of the contralateral lung. Echocardiography is the best tool to establish the diagnosis. In our opinion, invasive procedures, such as cardiac catheterization, may be postponed if there is no echocardiographic evidence of pulmonary hypertension.

  14. [Unilateral pulmonary agenesis, aplasia and dysplasia].

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    Dembinski, J; Kroll, M; Lewin, M; Winkler, P

    2009-04-01

    Unilateral pulmonary anomalies are rare events of unknown etiology and large clinical variability. Neonatal history does not allow for a reliable prognosis. Interdisciplinary mangament includes prenatal diagnostics and obstetrics, genetics, neonatology, pediatric cardiology and surgery as well as pediatric orthopedics. Neonatal history and long-term follow-up in three patients are presented here including a discussion of prenatal diagnostics and the embryo-genetic basics of lung development. In three term neonates the diagnoses of unilateral pulmonary agenesis, aplasia and dysplasia, respectively, were based on angiography, MRI and bronchoscopy. Neonatal presentation and long-term consequences were studied in the context of the current literature. Neonatal complications ranged from mild repiratory distress to pulmonary failure requiring mechanical ventilation. One patient developed scoliosis on long-term follow-up. Cardiac failure or pulmonary hypertension did not occur during follow-up, in one case lung malformation was accompanied by VACTER-association. Unilateral lung malformation is frequently associated with other, singular or complex anomalies (e.g., renal and vascular). A possible relationship to disrupted regulation of embryo-genetic factors such as T-BOX genes, PITX2 and growth factors ( FGF10), which regulate ASYMMETRICAL pulmonary morphogenesis is discussed. Disruptive unilateral pulmonary malformations may serve as a model for embryological lung development and other anomalies (e.g., congenital diaphragmatic hernia, unilateral hypoplasia and CCAM). Prenatal diagnosis is characterized by unilateral hyperechogenicity of the affected lung. Neonatal presentation is determined by mediastinal shift which may be corrected by tissue-expander implantation. Associated anomalies require cytogenetic analysis and sequencing of currently known mutations. Long-term follow-up by echocardiography and pulmonary function testing is mandatory in these patients.

  15. Pulmonary artery agenesis associated with coronary collaterals among adults.

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    Darwazah, Ahmad K; Alhaddad, Imad A

    2016-07-16

    Unilateral agenesis of the pulmonary artery is a rare congenital anomaly, which commonly involves the right side. Cases are associated with systemic collaterals, that may also rarely arise from the coronary arteries.Two adult patients are presented with a right pulmonary artery agenesis associated with collaterals from the right coronary artery. The implications of such an anomaly on pulmonary artery pressure and lung pathology differs among both cases. The association of coronary collaterals is rare and its implication is variable among various patients.

  16. 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.

  17. Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome.

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    Muensterer, Oliver; Abellar, Rosanna; Otterburn, David; Mathew, Rajamma

    2015-06-01

    Pulmonary agenesis is a rare congenital disorder with large variability in presentation and prognosis. We describe a full-term infant born with right-sided pulmonary agenesis who underwent thoracoscopic placement of a tissue expander. He ultimately died of pulmonary hypertension. Immunohistology showed intimal hyperplasia without the loss of endothelial caveolin-1 expression. A literature review revealed that while some of these patients have favorable outcome, many succumb despite therapy.

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

    Directory of Open Access Journals (Sweden)

    Shraddha Verma

    2013-03-01

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. Unilateral primary pulmonary agenesis and hypoplasia in monozygotic twins.

    Science.gov (United States)

    Alsaadi, Muslim; Al Muqhem, Badr; Boukai, A; Iqbal, Shaikh M

    2012-01-01

    We describe 10-month-old identical female twin infants, one with primary left-sided pulmonary agenesis and the other with primary left-sided pulmonary hypoplasia. They came to our outpatient clinic complaining of persistent dry cough. The clinical examination revealed decreased air entry over the left hemithorax. Chest x-rays showed complete left-sided radio-opacity in both the twins. The chest computed tomography scan with contrast confirmed the diagnoses of left-sided pulmonary agenesis (twin A) and left-sided hypoplasia (twin B). No other associated congenital anomaly was noted in either of the twins. To our knowledge, such a condition in live monozygotic twins has not been previously reported in published studies.

  1. Report of a girl with vacterl syndrome and right pulmonary agenesis.

    Science.gov (United States)

    Avcu, S; Akgun, C; Temel, H; Arslan, S; Akbayram, S; Unal, O

    2009-01-01

    Report of a girl with VACTERL syndrome and right pulmonary agenesis: VACTERL syndrome is a combination of vertebral anomalies (V), anal atresia (A), congenital heart defects (C), tracheo-esophageal fistula (T), esophageal atresia (E), abnormalities of kidneys (renal anomalies, R) and limbs (L). In the present patient right pulmonary agenesis is co-occurring with VACTERL syndrome. We report on this case because the association of right pulmonary agenesis and VACTERL syndrome is rare.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

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

    Science.gov (United States)

    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.

  4. Hemoptysis and pulmonary artery agenesis: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Lopez-Majano, V.; Sobti, P.

    1985-08-01

    The combination of a pulmonary scintigram using radioactively labeled albumin macroaggregates (MAA) and a study of the circulation in the bronchial artery was performed in one patient. This noninvasive methodology showed that there was increased circulation to the vascular territory of the lung in which the pulmonary artery was missing. This could have resulted from abnormal communications between the bronchial artery and the pulmonary vessels or an increased blood supply to the right lung from bronchial arteries arising from the aorta. The absence of pulmonary circulation in the right lung was proved by the absence of radioactivity in the right lung after an intravenous injection of labeled albumin MAA.

  5. Case Report: Chronic Recurrent Unilateral Pulmonary Infection: Result of Congenital Unilateral Agenesis of Pulmonary Artery.

    Science.gov (United States)

    Al Jabbari, Odeaa; Abu Saleh, Walid K; Ramchandani, Mahesh; Scheinin, Scott

    2016-01-01

    Unilateral agenesis of the pulmonary artery (UAPA) is a rare congenital anomaly. This report describes a 52-year-old female who gave a long history of chronic, recurrent, left-sided pulmonary infections related to UAPA. For many years, she was managed medically but the infection continued to recur. She eventually underwent left pneumonectomy and made a good recovery.

  6. Surgical Treatment of a Rare Isolated Bilateral Agenesis of Anterior and Posterior Cruciate Ligaments

    Directory of Open Access Journals (Sweden)

    G. Cerulli

    2014-01-01

    Full Text Available The isolated bilateral agenesis of both cruciate ligaments is a rare congenital disorder. A 17-year-old male came to our attention due to an alteration in gait pattern, pain, and tendency to walk on the forefoot with his knee flexed. The patient did not recall previous injuries. Upon physical examination anterior and posterior chronic instability were observed. Radiographic examination of both knees showed hypoplasia of the tibial eminence, a hypoplastic lateral femoral condyle, and a narrow intercondylar notch. MRI brought to light a bilateral agenesis of both posterior cruciate ligaments. Arthroscopic evaluation confirmed bilateral isolated agenesis of both cruciate ligaments. We recommended a rehabilitation program to prepare the patient for the arthroscopic construction of both cruciate ligaments.

  7. Surgical treatment of a rare isolated bilateral agenesis of anterior and posterior cruciate ligaments.

    Science.gov (United States)

    Cerulli, G; Amanti, A; Placella, G

    2014-01-01

    The isolated bilateral agenesis of both cruciate ligaments is a rare congenital disorder. A 17-year-old male came to our attention due to an alteration in gait pattern, pain, and tendency to walk on the forefoot with his knee flexed. The patient did not recall previous injuries. Upon physical examination anterior and posterior chronic instability were observed. Radiographic examination of both knees showed hypoplasia of the tibial eminence, a hypoplastic lateral femoral condyle, and a narrow intercondylar notch. MRI brought to light a bilateral agenesis of both posterior cruciate ligaments. Arthroscopic evaluation confirmed bilateral isolated agenesis of both cruciate ligaments. We recommended a rehabilitation program to prepare the patient for the arthroscopic construction of both cruciate ligaments.

  8. Treatment of spontaneous pneumothorax in a patient with right pulmonary agenesis.

    Science.gov (United States)

    Boulton, Bryon J; Force, Seth D

    2011-08-01

    Pulmonary agenesis is a rare congenital disease that is associated with many other congenital anomalies. We present the case of a patient, with right pulmonary agenesis and transmediastinal lung herniation, who presented with a spontaneous pneumothorax. This congenital anomaly and the treatment for this rare presentation is discussed in detail.

  9. Incidentally detected right pulmonary artery agenesis with right coronary artery collateralization.

    Science.gov (United States)

    Mikaberidze, Nino; Goldberg, Ythan; Khosraviani, Khashayar; Taub, Cynthia

    2014-01-01

    Unilateral pulmonary artery agenesis (UPAA) with pulmonary hypoplasia is a rare congenital anomaly. We describe a 71-year old male who was incidentally diagnosed with the right UPAA and a hypoplastic right lung supplied by collateralized right coronary.

  10. Unilateral pulmonary agenesis: a report of four cases, two diagnosed antenatally and literature review.

    Science.gov (United States)

    Kayemba-Kay's, S; Couvrat-Carcauzon, V; Goua, V; Podevin, G; Marteau, M; Sapin, E; Levard, G

    2014-03-01

    Pulmonary agenesis is a rare congenital malformation of lung development defined as complete absence of lung tissues, bronchi, and pulmonary vessels; it may be uni- or bilateral. The right-sided form carries the poorest prognosis due to severity of co-existent anomalies. Its diagnostic circumstances are variables: first reported cases were diagnosed at autopsy, but early postnatal as well as fortuitous discovery have been reported. In recent years, progress in obstetrical imaging has made antenatal diagnosis possible so that fetal ultrasound and MRI allow early diagnosis and refinement by permitting the elimination of differential diagnoses (diaphragmatic hernia, cystic adenomatoid malformation of the lung, giant lobar emphysema, and situs inversus). This anomaly is compatible with normal life provided co-existent malformations are thoroughly investigated and managed in a multidisciplinary setting. We report four cases of lung agenesis two of which were diagnosed antenatally at 23rd and 30th weeks of gestation respectively. Our aim is to describe the circumstances having led to diagnosis and report both follow-up and outcome of our patients.

  11. Congenital right pulmonary artery agenesis with atrial septal defect and pulmonary hypertension.

    Science.gov (United States)

    Orun, Utku Arman; Yilmaz, Osman; Bilici, Meki; Karademir, Selmin; Uner, Cigdem; Senocak, Filiz; Dogan, Vehbi

    2012-01-01

    Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.

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

    Science.gov (United States)

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

    2016-02-05

    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.

  13. Surgical Treatment of a Rare Isolated Bilateral Agenesis of Anterior and Posterior Cruciate Ligaments

    OpenAIRE

    2014-01-01

    The isolated bilateral agenesis of both cruciate ligaments is a rare congenital disorder. A 17-year-old male came to our attention due to an alteration in gait pattern, pain, and tendency to walk on the forefoot with his knee flexed. The patient did not recall previous injuries. Upon physical examination anterior and posterior chronic instability were observed. Radiographic examination of both knees showed hypoplasia of the tibial eminence, a hypoplastic lateral femoral condyle, and a narrow ...

  14. Neonatal repair of total anomalous pulmonary venous connection and lung agenesis.

    Science.gov (United States)

    Kaku, Yuji; Nagashima, Mitsugi; Matsumura, Goki; Yamazaki, Kenji

    2015-07-01

    Here we report a neonatal case of total anomalous pulmonary venous connection with left lung agenesis. Diagnostic imaging demonstrated that the left pulmonary veins were totally absent and the right pulmonary veins connected with the common pulmonary chamber. Drainage from the common pulmonary venous chamber entered the persistent left suerior vena cava. In addition, it revealed complete absence of the left main bronchus and left lung vessels. The neonate successfully underwent surgical repair 18 days after birth.

  15. Possible role of WT1 in a human fetus with evolving bronchial atresia, pulmonary malformation and renal agenesis.

    Science.gov (United States)

    Loo, Christine K C; Algar, Elizabeth M; Payton, Diane J; Perry-Keene, Joanna; Pereira, Tamara N; Ramm, Grant A

    2012-01-01

    The association of peripheral bronchial atresia and congenital pulmonary airway malformation (CPAM) has recently been recognised, but the pathology of the lesions evolving together has not been described. We present autopsy findings in a 20 week fetus showing areas of peripheral bronchial destruction and airway malformation consistent with developing CPAM in the right lung supporting a causal relationship between these lesions. This fetus also had congenital heart defect, bilateral renal agenesis and syndactyly. We identified another fetus from our autopsy files, with bilateral renal agenesis, similar right sided pulmonary malformation and cardiac defects. Similar bilateral renal agenesis and defects of the heart and lungs are found in wt1(-/-) mice and we have investigated the expression of WT1 in these fetuses. We hypothesise that the cardiac, liver, renal and possibly lung lesions in these two cases may arise due to mesenchymal defects consequent to WT1 misexpression and discuss evidence for this from the scientific literature. We used immunoperoxidase stains to analyse WT1 expression in autopsy hepatic tissue in both fetuses. We also investigated the expression of α-smooth muscle actin (α-SMA), a marker of activated hepatic stellate cells/myofibroblasts, and desmin in hepatic mesenchyme and compare these findings with control fetuses, without congenital malformations. We found reduced WT1 expression in hepatic mesothelium in both fetuses with malformations. There was also increased expression of α-SMA in liver perisinusoidal cells, as seen in the wt1(-/-) mouse model. We therefore propose that abnormality of WT1 signalling may be an underlying factor, as WT1 is expressed in coelomic lining cells from which mesenchyme is derived in many organs.

  16. A spectrum of skeletal anomalies associated with pulmonary agenesis: Possible neural crest injuries

    Energy Technology Data Exchange (ETDEWEB)

    Osborne, J.; Masel, J.; McCredie, J.

    1989-07-01

    Six cases of unilateral pulmonary agenesis with skeletal and other deformities have been diagnosed in our hospitals. The various pulmonary, spinal, rib and limb anomalies with their possible interrelationships were examined and described in detail and comparison with previously reported cases was made. It became apparent that the limb abnormalities which most constantly involved hypoplasia of the phalanges of a thumb with varying metacarpal and radial anomalies, were ipsilateral to the pulmonary agenesis in all cases. The spinal deformities involved degrees of failure of segementation of T1-T3 with other vertebrae randomly involved. Rib abnormalities also varied and did not necessarily correspond to the same side as the pulmonary agenesis. The concept of the anomalies all being part of a group of neural crest injuries was then explored. (orig.).

  17. Adult presentation of right lung agenesis and left pulmonary artery sling.

    Science.gov (United States)

    Espinosa, L; Agarwal, P

    2008-02-01

    The combination of right lung agenesis and left pulmonary artery (LPA) sling is a rare entity that has been described only in the pediatric population. Cross-sectional imaging modalities such as magnetic resonance imaging (MRI) and computed tomography (CT) have an advantage over echocardiography and pulmonary angiography in demonstrating the anomalous left pulmonary artery, particularly in the presence of coexisting lung agenesis, as exemplified in this case. We report the first case of this rare entity in an adult. It is important to be aware that this abnormality, though rare, can present even in adulthood, and therefore close attention should be paid to the course of the pulmonary artery to ensure detection of a sling in association with lung agenesis.

  18. Adult Presentation of Right Lung Agenesis and Left Pulmonary Artery Sling

    Energy Technology Data Exchange (ETDEWEB)

    Espinosa, L.; Agarwal, P. (Div. of Cardiothoracic Radiology, Dept. of Radiology, Univ. of Michigan, Ann Arbor, MI (US))

    2008-02-15

    The combination of right lung agenesis and left pulmonary artery (LPA) sling is a rare entity that has been described only in the pediatric population. Cross-sectional imaging modalities such as magnetic resonance imaging (MRI) and computed tomography (CT) have an advantage over echocardiography and pulmonary angiography in demonstrating the anomalous left pulmonary artery, particularly in the presence of coexisting lung agenesis, as exemplified in this case. We report the first case of this rare entity in an adult. It is important to be aware that this abnormality, though rare, can present even in adulthood, and therefore close attention should be paid to the course of the pulmonary artery to ensure detection of a sling in association with lung agenesis

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

    OpenAIRE

    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...

  20. Right pulmonary artery agenesis presenting with uncontrolled asthma in an adult: a case report

    Directory of Open Access Journals (Sweden)

    Thammasitboon Supat

    2011-08-01

    Full Text Available Abstract Introduction Unilateral absence of the pulmonary artery (UAPA or pulmonary artery agenesis is a rare congenital disorder presenting with a wide spectrum of symptoms. The clinical presentation is variable and many patients can be asymptomatic for many years and even throughout their lives. Case presentation We report the case of a 53-year-old African-American woman who was diagnosed with right pulmonary artery agenesis after presenting with uncontrolled asthma and recurrent bronchopulmonary infections. Conclusion In an unexplained case of recurrent respiratory infections and shortness of breath, the possibility of a rare congenital anomaly like UAPA should be considered and an appropriate evaluation should be done.

  1. Unilateral pulmonary agenesis and esophageal atresia with a tracheoesphageal fistula-23 year followup

    Directory of Open Access Journals (Sweden)

    Michael Curci

    2015-04-01

    Full Text Available Pulmonary agenesis, esophageal atresia and a tracheoesophageal fistula (EA + TEF are a rare combined congenital anomalies associated with a high morbidity and mortality. For those patients that have survived these malformation, there has been limited long-term follow up. This case report describes a 23-year followup with evaluation of the patient's pulmonary, cardiac and gastrointestinal function.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  3. Imaging Features of Isolated Unilateral Pulmonary Artery Agenesis: a review of four cases

    OpenAIRE

    Batur, Abdussamet; Ödev, Kemal; Pekcan, Sevgi

    2015-01-01

    To highlight the variation in imaging and clinical manifestations of four cases of isolated unilateral absence of pulmonary artery presented in varied ages. Four patients with unilateral pulmonary artery agenesis were referred to our institution They underwent a series of investigations, including chest radiography, computed tomography and magnetic resonance imaging. Two of the four patients had absence of the right main pulmonary artery, while the remaining two patients had absence of the le...

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

    Science.gov (United States)

    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.

  5. Isolated right pulmonary artery agenesis with agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply from celiac axis with normal venous drainage.

    Science.gov (United States)

    Chaudhry, A; Rathore, M; Banavaliker, J N

    2014-01-01

    Isolated unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly. When detected in infancy, the condition is commonly associated with cardiovascular defects which are more frequently associated with left pulmonary artery agenesis. Patients with isolated right pulmonary artery agenesis survive into adulthood with minimal or no symptoms and are diagnosed incidentally on the chest radiographs. We report a case of a 19-year-old female patient who presented to us with recurrent haemoptysis. She was symptomatic since the age of four years. We report the rare occurrence of UAPA on right side, agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply of right lung from coeliac axis in this patient.

  6. Right pulmonary agenesis in an elderly woman complicated by transient ischemic attack.

    Science.gov (United States)

    Ueda, Takashi; Nozoe, Masahiko; Nakamoto, Yasuhisa; Irie, Yoshikazu; Mizushige, Katsufumi

    2011-01-01

    There are few case reports regarding patients with right lung agenesis living to old age because of both severe mediastinal and cardiac displacements. We report a 61-year-old woman with right pulmonary agenesis complicated by a transient ischemic attack that was evaluated by a three-dimensional reconstruction of helical computed tomography and an echocardiography. This patient was able to survive until old age because she had no critical anomalies in other organs including the heart. A mitral valve prolapse was detected by a two-dimensional echocardiography and we treated her with anti-platelet aggregation therapy for the prevention of recurrent stroke.

  7. Prenatal diagnosis of isolated right pulmonary agenesis using sonography alone: case study and systematic literature review.

    Science.gov (United States)

    Meller, Cesar H; Morris, R Katie; Desai, Tarak; Kilby, Mark D

    2012-12-01

    Pulmonary agenesis is a rare congenital anomaly, estimated to complicate around 1 per 15,000 pregnancies, in which there is complete absence or severe hypoplasia of one or both lungs, frequently associated with other abnormalities. A prospective prenatal diagnosis is a challenge, and a substantial proportion of cases are diagnosed by fetal magnetic resonance imaging, postnatal computed tomography, or postmortem. Thus, there are only a few reported cases of prenatal diagnosis in the literature. We report the prenatal diagnosis of isolated right lung agenesis diagnosed with sonography alone at a relatively early gestational age. We also present a systematic review of the literature for this condition to accompany this case study.

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

    Science.gov (United States)

    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 patient underwent operation for the pelvic mass with one-lung ventilation (OLV) under general anesthesia. We highlighted the use of protective ventilation (PV) strategy during OLV.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  11. Single, mediastinal, unilobar lung - a rare form of subtotal pulmonary agenesis

    Energy Technology Data Exchange (ETDEWEB)

    Markowitz, R.I.; Rosenfield, N.S.; Frederick, W.; Duray, P.H.; Seashore, J.H.

    1987-05-01

    We describe a case of a full-term infant with severe respiratory failure and pulmonary insufficiency caused by an anomaly consisting of a single, unilobar lung arising from the trachea and situated in the middle mediastinum. Plain film, echocardiogram, and surgical aspects will be described and correlated with the post mortem findings and embryologic considerations. We were unable to find a similar case reported in the literature and conclude that this rare lesion represent an intermediate form between total and unilateral pulmonary agenesis.

  12. Bilateral subcortical heterotopia with partial callosal agenesis in a mouse mutant.

    Science.gov (United States)

    Rosen, G D; Azoulay, N G; Griffin, E G; Newbury, A; Koganti, L; Fujisaki, N; Takahashi, E; Grant, P E; Truong, D T; Fitch, R H; Lu, L; Williams, R W

    2013-04-01

    Cognition and behavior depend on the precise placement and interconnection of complex ensembles of neurons in cerebral cortex. Mutations that disrupt migration of immature neurons from the ventricular zone to the cortical plate have provided major insight into mechanisms of brain development and disease. We have discovered a new and highly penetrant spontaneous mutation that leads to large nodular bilateral subcortical heterotopias with partial callosal agenesis. The mutant phenotype was first detected in a colony of fully inbred BXD29 mice already known to harbor a mutation in Tlr4. Neurons confined to the heterotopias are mainly born in midgestation to late gestation and would normally have migrated into layers 2-4 of overlying neocortex. Callosal cross-sectional area and fiber number are reduced up to 50% compared with coisogenic wildtype BXD29 substrain controls. Mutants have a pronounced and highly selective defect in rapid auditory processing. The segregation pattern of the mutant phenotype is most consistent with a two-locus autosomal recessive model, and selective genotyping definitively rules out the Tlr4 mutation as a cause. The discovery of a novel mutation with strong pleiotropic anatomical and behavioral effects provides an important new resource for dissecting molecular mechanisms and functional consequences of errors of neuronal migration.

  13. Right pulmonary artery agenesis with patent ductus arteriosus and Eisenmenger syndrome: a rare case diagnosed during the postpartum period.

    Science.gov (United States)

    Beker-Acay, Mehtap; Ozkececi, Gulay; Unlu, Ebru; Hocaoglu, Elif; Kacar, Emre; Onrat, Ersel

    2014-01-01

    Unilateral absence of a pulmonary artery a very rare congenital disorder. We here present a case of a 22-year-old female patient with agenesis of the right pulmonary artery accompanying patent ductus arteriosus and Eisenmenger syndrome, diagnosed by chest X-ray and multidetector computed tomography 5 days after giving birth.

  14. Right pulmonary artery agenesis and coronary-to-bronchial artery aneurysm.

    Science.gov (United States)

    De Dominicis, Florence; Leborgne, Laurent; Raymond, Alexandre; Berna, Pascal

    2011-03-01

    Isolated unilateral pulmonary artery agenesis is a rare congenital anomaly that may be complicated with hemoptysis, recurrent pulmonary infections or pulmonary hypertension. To our knowledge the occurrence of a coronary syndrome associated with a coronary-to-bronchial artery saccular aneurysmal collateralization has never been described before. A 44-year-old female presented a congenital right pulmonary artery agenesis associated with a hypotrophic and multicystic right lung complicated with recurrent bronchitis. This patient had a coronary syndrome for which the coronary artery imaging showed a coronary-to-bronchial artery collateralization with an aneurysm at this level. It gives rise to a coronary syndrome by coronary steal. Two bronchial collaterals arising from a diaphragmatic artery and the subclavian artery were also found on the computed tomography (CT)-scan. This last collateral also showed another saccular aneurysm. We first performed an embolization of those two aneurysms in order to decrease the risk of hemorrhage and coronary steal, before performing a right pneumonectomy. In this case, the surgery was indicated because of the pathological lung and the risk of postembolization ischaemia. The postoperative course was uneventful and the patient was doing well six months later.

  15. Severe stenosis of a long tracheal segment, with agenesis of the right lung and left pulmonary arterial sling.

    Science.gov (United States)

    Munro, Hamish M; Sorbello, Andrea M C; Nykanen, David G

    2006-02-01

    A baby presented at term with respiratory distress was managed with extracorporeal membrane oxygenation. Bronchoscopy revealed tracheal hypoplasia, complete tracheal rings, and agenesis of the right main bronchus. Echocardiography showed a left pulmonary arterial sling arising from the proximal part of the right pulmonary artery. Cardiac catheterization demonstrated abnormal pulmonary vasculature in the left lung which would have prevented survival, even after surgical repair. Diagnostic catheterization was important in delineating the anatomy, and aided in the decision not to proceed with surgical repair.

  16. Isolated Hypoplasia of Left Pulmonary Artery with Agenesis of Left Lobe of Thyroid: A Case Report.

    Science.gov (United States)

    Khadir, Mohammed Abdul; Narayana, Ganesh; Ramagopal, Ganavi; Nayar, Pradeep G

    2016-12-01

    Isolated Unilateral hypoplasia or agenesis of a branch of pulmonary artery is very rare. It is usually seen associated with congenital heart diseases such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia. It occurs as a result of lack of embryological development of either the left or right sixth aortic arch and has been found to present itself with various clinical manifestations as during childhood it presents as contralateral pulmonary hypertension and in adults as haemoptysis. Early diagnosis and early surgical indication avoids the evolution of pulmonary hypertension to unfavourble state of more severe and progressive degrees and also prevents the development of pulmonary systemic collateral circulation, which is mainly responsible for subsequent haemoptysis in the adulthood. We hereby, report the case of an infant who presented with features of lower respiratory tract infection and later diagnosed as isolated congenital hypoplasia of left pulmonary artery and hence planned for proper follow-up for early surgery thereby preventing complications in the future.

  17. Congenital rubella with agenesis of the inferior cerebellar vermis and total anomalous pulmonary venous drainage.

    Science.gov (United States)

    Cluver, C; Meyer, R; Odendaal, H; Geerts, L

    2013-08-01

    Congenital rubella infection has been associated with a number of abnormalities including cardiac, central nervous system and placental complications. We present a case with multiple fetal abnormalities detected on prenatal ultrasound, and confirmed postnatally, that included a single umbilical artery, severe tricuspid regurgitation, micrognathia and agenesis of the inferior cerebellar vermis. Postnatal echocardiography additionally revealed unobstructed total anomalous pulmonary venous drainage (TAPVD) into the coronary sinus. Placental examination showed signs of placentitis, and polymerase chain reaction on neonatal serum was positive for rubella. Following a multidisciplinary team review, it was decided to provide only supportive care, and the infant died at 6 months of age owing to a respiratory tract infection. To our knowledge, TAPVD and agenesis of the inferior cerebellar vermis have not been reported previously in association with congenital rubella infection. This case illustrates how congenital infection may present in atypical ways and stresses the importance of considering congenital infection in the differential diagnosis of fetal anomalies when multiple features are present.

  18. 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

  19. Rare case of truncus arteriosus with anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) and unilateral left pulmonary artery agenesis.

    Science.gov (United States)

    Mittal, Kartik; Dey, Amit K; Gadewar, Rohit; Sharma, Rajaram; Pandit, Nilesh; Rajput, Priya; Hira, Priya

    2015-04-01

    The incidence of congenital heart disease (CHD) is 2.4-3.8/1000 live births. Up to 70.7 % of all cases of CHD are reported to be benign; complex heart anomalies are extremely rare. Our case is extremely rare, as we report three very rare findings-truncus arteriosus, anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA), and unilateral left pulmonary artery agenesis-in a single patient. Congenital complex cardiac abnormalities are very rare, and two-dimensional echocardiography screening should be supported by cardiac computed tomography (CT). We report a case of truncus arteriosus associated with ARCAPA and left pulmonary artery agenesis diagnosed by cardiac computed tomography; we believe that such an unusual case with all three of these entities has never been reported before.

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

    Science.gov (United States)

    Miyano, Go; Morita, Keiichi; Kaneshiro, Masakatsu; Miyake, Hiromu; Koyama, Mariko; Nouso, Hiroshi; Yamoto, Masaya; Nakano, Reiji; Tanaka, Yasuhiko; Nishiguchi, Tomizo; Kawamura, Takakazu; Fukumoto, Koji; Urushihara, Naoto

    2015-01-01

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  2. [Unilateral pulmonary artery agenesis with ipsilateral pulmonary hypoplasia as incidental finding in an asthmatic patient].

    Science.gov (United States)

    Contreras-Arias, Catalina; Duarte, Diana; Ramírez, Luis F; Serrano, Carlos D

    2014-01-01

    Unilateral absence of a pulmonary artery is an uncommon congenital heart disease. It can be related to respiratory symptoms such as asthma, an unusual finding in some of these patients. This paper reports the case of a 4-year-old male with recurrent respiratory infections and asthma symptoms, in who further studies found agenesia of right pulmonary artery with pulmonary hypoplasia of the same side.

  3. Isolated agenesis of the right pulmonary artery with late manifestation of pulmonary hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Huebsch, P.; Pichler, W.; Lang, I.; Mlczoch, J.

    1987-01-01

    The case of a woman patient of 25 years of age with acute cardiac decompensation is presented. The chest X-ray as well as the lung scan showed the typical features of absence of the right pulmonary artery. The diagnosis was confirmed angiographically. The sudden and late onset of symptoms of pulmonary hypertension is a remarkable feature.

  4. Isolated right pulmonary artery agenesis with aplasia of right upper lobe and with anomalous arterial supply from celiac axis, anomalous venous drainage

    Directory of Open Access Journals (Sweden)

    Tushar Madhav Kalekar

    2015-01-01

    Full Text Available Pulmonary artery agenesis is the complete absence of the right or left pulmonary artery. This lesion is commonly associated with other cardiac anomalies and is usually diagnosed incidentally. Unilateral absence of pulmonary artery with lung hypoplasia is very rare. Ipsilateral hypoplastic or absent central pulmonary artery in these patients will have a systemic arterial supply to peripheral pulmonary arteries of the affected lung from the descending thoracic or upper abdominal aorta. These findings are usually characterized as pulmonary veno lobar syndrome or scimitar syndrome when associated with ipsilateral total or partial anomalous pulmonary venous return from the lower lobe which drains into the inferior vena cava or less commonly to the hepatic, azygous, or portal vein, or into the right atrium and lung agenesis/hypoplasia.

  5. Familial congenital bilateral agenesis of the acromion : a radiologically illustrated case report

    NARCIS (Netherlands)

    Hermans, JJ; Mooyaart, EL; Hendriks, JGE; Diercks, RL

    1999-01-01

    Familial congenital bilateral acromion absence was found in four members of one family. Only one of them presented with gradually increasing pain in his left shoulder, resembling a shoulder impingement syndrome. The other members did not have any symptoms. This is the first report of familial occurr

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

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    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

  7. A modified rat model of isolated bilateral pulmonary contusion

    OpenAIRE

    Wang, Shaohua; Ruan, Zheng; Jie ZHANG; ZHENG, JIN

    2012-01-01

    The aim of the present study was to create a feasible specific rat model of isolated bilateral pulmonary contusion (PC) and to evaluate the relationship between severity of hypoxemia and quantity of contusion lesions. Anesthetized rats were placed in a prone position. Injury energy ranging from 2.1 to 3.0 J was produced by a falling weight passed through a specially designed arched shield to the bilateral chest wall of rats. After injury (4 h), the contusion volume was measured using computer...

  8. 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.

  9. 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.)

  10. Adult presentation of symptomatic left lung agenesis.

    Science.gov (United States)

    Kaya, Omer; Gulek, Bozkurt; Yilmaz, Cengiz; Soker, Gokhan; Esen, Kaan; Akin, Mehmet Ali; Dilek, Okan

    2017-03-01

    Pulmonary agenesis is a rarely encountered congenital anomaly, and its average prevalence is about 1 in 100,000 births. Anomalies of the cardiovascular, musculoskeletal, gastrointestinal, or genitourinary systems may accompany in nearly half of the cases. The diagnosis of pulmonary agenesis is usually made during childhood, but the diagnosis may be delayed until adulthood in case of an absence of comorbid anomalies . Herein, we present a case of pulmonary agenesis that was diagnosed during adulthood.

  11. Adult presentation of symptomatic left lung agenesis

    OpenAIRE

    Kaya, Omer; Gulek, Bozkurt; Yilmaz, Cengiz; Soker, Gokhan; Esen, Kaan; Akin, Mehmet Ali; Dilek, Okan

    2017-01-01

    Pulmonary agenesis is a rarely encountered congenital anomaly, and its average prevalence is about 1 in 100,000 births. Anomalies of the cardiovascular, musculoskeletal, gastrointestinal, or genitourinary systems may accompany in nearly half of the cases. The diagnosis of pulmonary agenesis is usually made during childhood, but the diagnosis may be delayed until adulthood in case of an absence of comorbid anomalies . Herein, we present a case of pulmonary agenesis that was diagnosed during ad...

  12. Adult presentation of symptomatic left lung agenesis

    Directory of Open Access Journals (Sweden)

    Omer Kaya, MD

    2017-03-01

    Full Text Available Pulmonary agenesis is a rarely encountered congenital anomaly, and its average prevalence is about 1 in 100,000 births. Anomalies of the cardiovascular, musculoskeletal, gastrointestinal, or genitourinary systems may accompany in nearly half of the cases. The diagnosis of pulmonary agenesis is usually made during childhood, but the diagnosis may be delayed until adulthood in case of an absence of comorbid anomalies . Herein, we present a case of pulmonary agenesis that was diagnosed during adulthood.

  13. 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.)

  14. A modified rat model of isolated bilateral pulmonary contusion.

    Science.gov (United States)

    Wang, Shaohua; Ruan, Zheng; Zhang, Jie; Zheng, Jin

    2012-09-01

    The aim of the present study was to create a feasible specific rat model of isolated bilateral pulmonary contusion (PC) and to evaluate the relationship between severity of hypoxemia and quantity of contusion lesions. Anesthetized rats were placed in a prone position. Injury energy ranging from 2.1 to 3.0 J was produced by a falling weight passed through a specially designed arched shield to the bilateral chest wall of rats. After injury (4 h), the contusion volume was measured using computer-generated three-dimensional reconstruction from a chest computed tomographic scan and expressed as a percentage of total lung volume. Arterial partial pressure of oxygen (PaO(2)) in blood gas analysis and contusion volume percentage were used to assess the severity of contusion. Heart and lung biopsy was used to confirm the diagnosis and rule out the existence of myocardial contusion. There were 3 cases of death and 1 case of death in the 3.0 J and the 2.4 J group, respectively. PaO(2) in the 2.7 J group was significantly lower than that in the lower energy groups (Ppulmonary contusion in the 2.7 J group was significantly higher compared to that of the lower energy groups (Pcontusion percentage (R(2)=0.76). Hemorrhage, edema and neutrophil infiltration were determined by lung biopsy. No evidence of myocardial contusion was documented in multiple heart biopsies. The method illustrated in this research effectively duplicates isolated bilateral pulmonary contusion in rats, the severity of which is highly correlated with the contusion size. Thus, 2.7 J can be regarded as the maximal energy for sublethal injury.

  15. Right lung agenesis

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    Kumar B

    2008-01-01

    Full Text Available Congenital pulmonary agenesis or aplasia is extremely rare. Although more than fifty percent of patients die before first five years of age, some individuals may remain asymptomatic throughout their life. A three-month-old female child with right pulmonary agenesis presented to us with severe respiratory distress. She was misdiagnosed as a case of foreign body bronchus at the peripheral health centre. Bronchoscopy confirmed the diagnosis and relieved the symptoms. It is recommended that invasive diagnostic procedures and prophylactic surgery should not be done in asymptomatic cases.

  16. MDCT and 3D evaluation of type 2 hypoplastic pulmonary artery sling associated with right lung agenesis, hypoplastic aortic arch, and long segment tracheal stenosis.

    Science.gov (United States)

    Lee, Edward Y

    2007-11-01

    The early diagnosis and complete anatomic evaluation of pulmonary artery sling, a congenital vascular anomaly in which left pulmonary artery arises from the right pulmonary artery, is paramount for proper patient management, because patients with this disorder frequently have other congenital anomalies resulting in high morbidity and mortality. Until recently, pulmonary artery sling in the neonate has been established with standard radiologic imaging studies such as plain radiographs, barium swallow studies, fluoroscopy-guided airway studies, and echocardiograms. However, with the development and widespread availability of multidetector computed tomography, pulmonary artery sling is increasingly evaluated with this newer technology. This case report presents a rare incidence of type 2 hypoplastic pulmonary artery sling in a neonate associated with right lung agenesis, hypoplastic aortic arch, and long segment tracheal stenosis. Multidetector computed tomography combined with 3-dimensional evaluation was particularly helpful in making a correct diagnosis of the complicated anatomic anomalies found in this case.

  17. A Rare Case of Bilateral Agenesis of Central Lower Incisors Associated With Upper Impacted Canine- A Case Report.

    Science.gov (United States)

    Porumb, Anca; Ignat Romanul, Ioana; Dalai, Camelia; Ciavoi, Gabiela; Tig, Ioan Andrei

    2016-03-01

    This case of a female patient, 14 yr old with association of the two anomalies, which we came across with in 2014, is rarely met in the specialty practice. The impacted canines are part of the group of dental anomalies of position, while the agenesis is part of the group of dental number anomalies. The orthodontic treatment in the two arches has to be differentiated, the therapeutic objectives being, also different in the two arches.

  18. A case of congenital agenesis of the right pulmonary artery presenting with hemoptysis and mimicking pulmonary hemosiderosis.

    Science.gov (United States)

    Simsek, Pelin Ozlem; Ozcelik, Ugur; Celiker, Alpay; Yalcin, Ebru; Cobanoglu, Nazan; Pekcan, Sevgi; Alehan, Dursun; Ucar, Canan; Dogru, Deniz; Kiper, Nural

    2009-02-01

    Congenital unilateral absence of a pulmonary artery is a rare anomaly most frequently accompanied by other cardiovascular anomalies. We report a 10-year-old girl presenting with fatigue and recurrent hemoptysis who was initially misdiagnosed with idiopathic pulmonary hemosiderosis. Her symptoms did not resolve despite treatment so she was referred to our center for further evaluation. We carried out an angiography which revealed the absence of the right pulmonary artery and multiple collaterals originating from the right subclavian and right internal mammary arteries supplying the right lung. During the follow-up the patient developed a severe episode of pulmonary infection and pulmonary hypertension which responded well to medical treatment. Physicians should be aware of the congenital absence of the right pulmonary artery especially in patients presenting with recurrent respiratory symptoms. Although this condition is generally considered to have a good prognosis, close observation is mandatory in order to prevent further complications and comorbidities.

  19. 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

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

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    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.

  1. Diagnosis of unilateral pulmonary arterial agenesis using scintiangiography - a case report -

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ok Hwa; Kim, Choon Yul; Shinn, Kyung Sub; Bahk, Yong Whee [Catholic Medical College, Seoul (Korea, Republic of)

    1986-08-15

    A 7-year-old boy presented with productive cough and mild dyspne a. He had a history of intermittant attacks of bronchopneumonia, but otherwise he was healthy. He had a small right lung on chest roentgenograms. The diagnosis of developmental hypoplasia of the right lung due to congenital absence of the right pulmonary artery was made by radionuclide angiocardiography and subsequently confirmed by cardiac catheterization and angiography.

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

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    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.

  3. NONIMMUNE HYDROPS-FETALIS AND BILATERAL PULMONARY HYPOPLASIA IN A NEWBORN-INFANT WITH EXTRALOBAR PULMONARY SEQUESTRATION

    NARCIS (Netherlands)

    BRUS, F; NIKKELS, PGJ; VANLOON, AJ; OKKEN, A

    1993-01-01

    Extralobar pulmonary sequestration was found in a newborn premature infant that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphati

  4. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    Jeong, Seo In; Seon, Hyun Ju; Kim, Yun Hyeon [Dept. of Radiology, Chunnam National University Hospital, Gwangju (Korea, Republic of); Choi, Sung [Dept. of Radiology, Chunnam National University Hwasun Hospital, Hwasun(Korea, Republic of)

    2011-12-15

    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.

  6. 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

  7. Congenital Agenesis of the Left Lung: A Rare Case

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    Tülin Durgun Yetim

    2011-01-01

    Full Text Available Pulmonary agenesis is a rare congenital anomaly, the etiology of which is not clearly known. Other systemic comorbidities such as cardiovascular, gastrointestinal, musculoskeletal, and urogenital system anomalies can be observed in more than half of the patients. It is usually diagnosed during childhood. Diagnosis in adulthood is very rare. We present a case of pulmonary agenesis diagnosed in an adult.

  8. A Rare Case of Bilateral Agenesis of Central Lower Incisors Asso¬ciated With Upper Impacted Canine- A Case Report

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    Anca PORUMB

    2016-03-01

    Full Text Available This case of a female patient, 14 yr old with association of the two anomalies, which we came across with in 2014, is rarely met in the specialty practice. The impacted canines are part of the group of dental anomalies of position, while the agenesis is part of the group of dental number anomalies. The orthodontic treatment in the two arches has to be differentiated, the therapeutic objectives being, also different in the two arches. Keywords: Agenesis, Impacted canines, Orthodontic appliances

  9. Scimitar syndrome with renal agenesis

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    Hasan Kahraman

    2012-01-01

    Full Text Available Partial pulmonary venous connection anomaly is relatively uncommon form of congenital heart diseases. The quite rare combination of this anomaly with hypoplasia of the right lung and dextroposition of the heart is designated as scimitar syndrome. Most cases are presented in infantile period and adult presentation is exceedingly rare. Our patient, a 38-year-old man, was admitted to a doctor with flu-like complaint and because of abnormalities on chest X-ray he was sent to our clinic. He did not have any chronic complaints such as shortness of breath and fatigue. After investigation, scimitar syndrome was diagnosed. Left renal agenesis was determined with abdominal examination. Best of our knowledge in literature we did not detect any case both with Scimitar syndrome and renal agenesis, and we wanted to report the asymptomatic adult Scimitar syndrome case with left renal agenesis.

  10. Bilateral chylothorax in a patient with chronic central vein thrombosis and chronic thromboembolic pulmonary hypertension

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    Avdhesh Bansal

    2015-01-01

    Full Text Available The chylothorax is not a common presentation, and bilateral chylothorax in patients with chronically high central venous pressure secondary to venous thrombosis is a rare in incidence. We reported a case of bilateral chylothorax in a patient of chronic deep vein thrombosis (DVT in central veins with chronic thromboembolic pulmonary hypertension who presented with 2 weeks history of increased breathlessness, bilateral chest discomfort and weakness. Work-up with chest X-ray and ultrasonography-chest showed gross left sided and mild right sided pleural effusion, thoracocentesis was consistent with chylothorax. Contrast enhanced computed tomography-chest showed multiple collateral formation of left side subclavian vein, venous Doppler showed old DVT in right and left subclavian veins and two-dimensional echocardiogram showed finding of severe pulmonary hypertension. After 24 h of fasting and conservative management, pleural drain became clear and decreased in the amount. Patient′s video assisted thoracoscopic surgery was done, and thoracic duct was ligated and cut down at diaphragmatic level and bilateral talc pleurodesis done. Patient improved clinically and radiologically.

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

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    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.

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

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    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

  13. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome

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    Jane Jackie David

    2016-01-01

    Full Text Available We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner′s syndrome.

  14. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner's syndrome.

    Science.gov (United States)

    David, Jane Jackie; Mohanlal, Smilu; Sankhe, Punam; Ghildiyal, Radha

    2016-01-01

    We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD) with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner's syndrome).

  15. Bilateral Morgagni hernia: operative discovery of appendix lying on superior pulmonary vein.

    Science.gov (United States)

    Lammy, S; Stewart, M; Carnochan, F M; Walker, W S

    2013-08-01

    A patient presented having an acute abdomen on a background of a twelve-month history of worsening asthma. Computed tomography showed giant bilateral intrathoracic hernias extending to both thoracic apices. Our case was unusual as the defect was bilateral and left-sided. Surgical repair revealed each hernia sac measuring >20 cm and to contain the entirety of the small bowel and colon (including retroperitoneal bowel). The appendix was discovered adjacent to right superior pulmonary vein. Both sacs were excised and the defects dissected and transfixed in a single stage operation. In the post-operative stage, he developed a 6.3 cm fluid collection anterior to the right atrium and a left-sided pleural effusion. Morgagni hernias can escape detection and be attributed to other diagnoses courtesy of false localising signs on clinical examination and symptoms in the history.

  16. [Bilateral coronary artery-pulmonary artery fistulas in a case with unstable angina pectoris].

    Science.gov (United States)

    Kepez, Alper; Kaya, Ergün Bariş; Aytemir, Kudret; Oto, Ali

    2008-03-01

    Bilateral coronary artery fistulas originating from both right and left coronary arteries are rare congenital abnormalities. A 58-year-old man presented with chest pain unrelated to exertion. Coronary angiography showed a fistula originating from the level of the first diagonal branch of the left anterior descending (LAD) coronary artery and a 95% stenosis just distal to the fistula. Right coronary angiography showed another fistula originating from the ostium of the right coronary artery. Both fistulas drained into the pulmonary artery. Coronary bypass surgery was performed for the LAD lesion using the left internal mammary artery graft, during which both fistulas were ligated. No complications were encountered postoperatively.

  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. 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

  19. Callosal agenesis and Open lip Schizencephaly.

    Science.gov (United States)

    Prasad, Maya; Iype, Mary; Nair, P M C

    2006-09-01

    We report a case of a new born who presented with neonatal seizures; and who had coexistence of a Corpus Callosum Agenesis with a bilateral Open lip Schizencephaly and a Dandy Walker malformation. The investigations for an underlying etiology, however was futile.

  20. 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

  1. 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

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

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    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.

  3. Axillary artery transection and bilateral pulmonary embolism after anterior shoulder dislocation: case report

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    Leclerc Betty

    2017-01-01

    Full Text Available Introduction: Anterior shoulder dislocation can be associated with vascular and neurological complications. However, axillary artery injury associated with shoulder dislocation is rare and extremely rare without bone fracture. An early diagnosis of these complications allows predicting long-term functional outcomes. Methods: This article reports the case of a 66-year-old patient who presented an anterior shoulder dislocation after a ski fall without any neurological dysfunction or pulse deficit. Results: The first reduction attempts were unsuccessful and during the new attempt, we observed a hematoma. A CT scan showed a disruption of the axillary artery and a bilateral pulmonary embolism. Conclusion: Neurovascular injury must be systematically sought before and after reduction, and a multidisciplinary approach is always necessary.

  4. 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.)

  5. Unilateral lung agenesis--detrimental roles of surrounding vessels.

    Science.gov (United States)

    Chou, An-Kou; Huang, Shu-Chien; Chen, Shyh-Jye; Huang, Pei-Ming; Wang, Jou-Kou; Wu, Mei-Hwan; Chen, Yih-Sharng; Chang, Chung-I; Chiu, Ing-Sh; Wu, En-Ting

    2007-03-01

    Unilateral lung agenesis is a rare congenital defect and could be associated with multiple abnormalities. The patients usually have poor long-term outcomes especially in those with right lung agenesis. We reviewed the 10-year experience in our hospital to describe special clinical features and try to delineate the causes of poor outcomes. From 1995 to 2005, 14 patients less than 18 years of age with unilateral lung agenesis (4 with left agenesis, 10 with right agenesis) were enrolled. Medical records reviewed included diagnosis, presentation, chromosome anomalies, cardiovascular anomalies and interventions, outcomes. We found that the mechanisms of severe airway disease in right lung agenesis included (1) trachea compression by the aortic arch, (2) the presence of "pseudo-ring-sling complex," (3) distended pulmonary artery due to left to right shunt which impinged the only bronchus, and finally (4) the persistent LSVC that restricts the growth of trachea. The etiologies of airway complication in left lung agenesis included anomalous aortic arch compression on trachea and the coexisting heart disease with significant left to right shunt, which impinged on the bronchus. In conclusion, unilateral lung agenesis has frequently associated airway problems due to its surrounding vessels. Satisfactory airway intervention remains challenging. This disease still requires great effort to improve patient outcomes.

  6. 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...

  7. Bilateral Renal Agenezinin Prenatal Tanısı:Olgu Bildirimi

    OpenAIRE

    YANIK, F.F.; YANIK, A.; AKPOLAT, İ.

    2010-01-01

    Prenatal Diagnosis of Bilateral Renal Agenesis: Case Report Bilateral renal agenesis is a congenital anomaly incompatible with life and is observed in every 4000 births. Severe oligohydramnios, failure to identify fetal bladder and fetal kidneys are among the prenatal ultrasonographic findings. Here we present a case of bilateral renal agenesis observed in a pregnancy achieved by ovulation induction; as well as the literature review. Bilateral renal agenezi 4000 doğumda bir ...

  8. 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.

  9. Hereditary hemorrhagic telangiectasia with bilateral pulmonary vascular malformations: A case report

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    Lončarević Olivera

    2016-01-01

    Full Text Available Introduction. Hereditary hemorrhagic telangiectasia (HHT also known as Osler-Weber-Rendu syndrome is an autosomal dominant disease that occurs due to vascular dysplasia associated with the disorder in the signaling pathway of transforming growth factor β (TGF-β. The clinical consequence is a disorder of blood vessels in multiple organ systems with the existence of telangiectasia which causes dilation of capillaries and veins, are present from birth and are localized on the skin and mucosa of the mouth, respiratory, gastrointestinal and urinary tract. They can make a rupture with consequent serious bleeding that can end up with fatal outcome. Since there is a disruption of blood vessels of more than one organic system, the diagnosis is very complex and requires a multidisciplinary approach. Case report. We reported a 40-year-old female patient with a long-time evolution of problems, who was diagnosed and treated at the Clinic for Lung Diseases of the Military Medical Academy in Belgrade, Serbia, because of bilaterally pulmonary arteriovenous malformations associated with HHT. Embolization was performed in two acts, followed with normalization of clinical, radiological and functional findings with the cessation of hemoptysis, effort intolerance with a significant improvement of the quality of life. Conclusion. HHT is a rare dominant inherited multisystem disease that requires multidisciplinary approach to diagnosis and treatment. Embolization is the method of choice in the treatment of arteriovenous malformations with minor adverse effects and very satisfying therapeutic effect.

  10. Unitary attention in callosal agenesis.

    Science.gov (United States)

    Dell'acqua, R; Jolicoeur, P; Lassonde, M; Angrilli, A; De Bastiani, P; Pascali, A

    2005-01-01

    The interhemispheric organisation of two specific components of attention was investigated in three patients affected by partial or complete agenesis of the corpus callosum. A visuospatial component of attention was explored using a visual search paradigm in which target and distractors were displayed either unilaterally within a single visual hemifield, or bilaterally across both visual hemifields in light of prior work indicating that split-brain patients were twice as fast to scan bilateral displays compared to unilateral displays. A central component of attention was explored using a psychological refractory period (PRP) paradigm in which two visual stimuli were presented laterally at various stimulus onset asynchronies (SOAs), with each stimulus associated with a different speeded two-alternative choice task. The stimulus-response compatibility in the second task was systematically manipulated in this paradigm, in light of prior work indicating that split-brain patients exhibited a close-to-normal PRP effect (i.e., slowing of the second response as SOA is decreased), with, however, abnormally decreasing effects of the manipulation of the response mapping on the second task speed as SOA was decreased. The present results showed that, although generally slower than normals in carrying out the two tasks, the performance of each of the three acallosal patients was formally equivalent to the performance of a matched control group of normal individuals. In the visual search task, the search rate of the acallosal patients was the same for unilateral and bilateral displays. Furthermore, in the PRP task, there was more mutual interference between the lateralised tasks for the acallosal patients than that evidenced in the performance of the matched control group. It is concluded that the visuospatial component and the central component of attention in agenesis of the corpus callosum are interhemispherically integrated systems.

  11. Repair of a ventricular septal defect in a patient with left lung agenesis.

    Science.gov (United States)

    Guo, Hong-Wei; Pan, Shi-Wei; Song, Yun-Hu; Hu, Sheng-Shou

    2011-09-01

    Congenital heart disease combined with lung agenesis is extremely rare. We report a case of a 5-year-old female with a ventricular septal defect (VSD) and left lung agenesis with severe pulmonary hypertension who underwent successful closure of the VSD. 

  12. 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.

  13. Right Lung Agenesis; Isolated and with Accompanied Anomalies

    Directory of Open Access Journals (Sweden)

    Yakup Canıtez

    2013-12-01

    Full Text Available Right lung agenesis is a rare anomaly that can be isolated or accompanied by system anomalies such as cardiac, skeletal or urinary systems. Case 1, a four-month-old girl, was brought because of respiratory distress. Patient had polydactyly, syndactyly of right thumb, right mandibular hypoplasia and low-set dysmorphic ears. Lung x-ray and thorax computerized tomography (CT were consistent with right pulmonary agenesis and butterfly vertebra was evident in the 7th thoracic level. Thoracic CT angiography revealed narrowing of the left main bronchus and esophagus due to compression of aorta, left pulmonary artery and right atrium. In bronchoscopy, narrowing due to compression at the carina level was seen and right main bronchus was not seen. In abdominal ultrasonography, right kidney placement anomaly (pelvic ectopia was present and renal scintigraphy revealed fusion in both kidneys. With these findings, it was found that right pulmonary agenesis was accompanied by ipsilateral radial ray anomaly, renal anomaly, vertebral anomaly and hemifacial microsomia. Case 2, a fifteen-year-old male patient, was diagnosed as pulmonary agenesis via chest x-ray, pulmonary CT and bronchoscopy after a wheezing episode when he was 2 months old. Patient had no complaint except for exhaustion that is aggravated by exercise during last year. In physical examination, he had a mild scoliosis toward right, respiratory sounds were diminished on right hemithorax and heart sounds were heard on right side. Chest x-ray and thoracic CT were consistent with right lung agenesis. No abnormality was found in echocardiography and abdominal ultrasonography was normal. Accompanied cardiovascular anomalies, distortions of intrathoracic structures and recurrent infections are main factors that affect mortality and morbidity. Here, two cases with right lung agenesis, isolated and accompanied by multiple anomalies, were presented. (Jo­ur­nal of Cur­rent Pe­di­at­rics 2013; 11: 134-7

  14. Lumbosacral agenesis in a cat

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    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.

  15. Congenital right hemidiaphragmatic agenesis

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    Bilal Mirza

    2012-01-01

    Full Text Available Congenital diaphragmatic hernia is a congenital defect of the diaphragm through which intestine and other viscera herniate into the chest. In extreme form of diaphragmatic maldevelopment, there might be a complete agenesis of diaphragm. A 45-day old male infant was presented with fever, cough and respiratory distress for a week. Chest radiograph showed right-sided congenital diaphragmatic hernia. The patient underwent surgical exploration and found to have an unusual and large defect of right hemidiaphragm. The diaphragm was absent on anterior and lateral aspects of the chest wall and only a small rim of diaphragm was present on posterior aspect. The defect was identified as agenesis of right hemidiaphragm and successfully managed by suturing the posterior rim of diaphragm to the intercostal muscles and ribs. This report describes successful management of hemidiaphragmatic agenesis without incorporating a prosthetic material.

  16. 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.

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

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    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.

  18. MRI findings of type II sacral agenesis: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang A; Kim, Myung Soon; Kwon, Woo Cheol [Dept. of Radiology, Yonsei University Wonju College of Medicine, Wonju Severance Christian Hospital, Wonju (Korea, Republic of)

    2016-07-15

    Sacral agenesis (or caudal regression syndrome) is a rare congenital anomaly involving various levels of coccygeal, sacral, and even lumbar or lower thoracic vertebral dysgenesis, as well as spinal cord abnormalities. A few cases have been previously reported in Korea, especially based upon MRI findings. We describe a case of a 4-year-old girl with partially bilateral agenesis of the sacrum (type II), and club-shaped (chisel-shaped) spinal cord disruption. We also review MRI findings of sacral agenesis, focused on classification and radiological findings.

  19. 小儿先天性肺不发育和发育不良的X线诊断(附13例X线分析)%An Analyses of X-ray Diagnosis of 13 Cases of Congenital Pulmonary Agenesis and Hypoplasia in Children

    Institute of Scientific and Technical Information of China (English)

    陈豹; 顾海滨; 赵晨

    2001-01-01

    Objective:To evaluate the efficacy of imaging diagnisis of congenital pulmonary agenesis and hypoplasia in children.Methods:The 13 cases of congenital pulmonary agenesis and hypoplasia were veritfied by bronchcscopy and operation or pathology the characteristics of the X-ray,CT and bronchography were analysed.Results:the X-ray features included:(1)capacity decreased and density increased of diseased lung;(2)small hilar pulmonis;(3)mediastinum shifted to the affected side.Conclusion:Radiography is a good procedure in diagnosis of the most cases of congenital pulmonary agenesis and hypoplasia.%目的:评价X线检查对小儿先天性肺不发育和发育不良的诊断作用。方法:对13例先天性肺不发育和发育不良经支气管镜检、手术或病理证实的X线平片、CT扫描、支气管造影分析其影像学表现。结果:本病X线检查的影像特征为:(1)患肺容积小、密度增高;(2)小肺门;(3)纵隔移向患侧。结论:X线检查对先天性肺不发育和发育不良是一很好的诊断方法。

  20. Cardiac defect with diaphragmatic hernia and left lung agenesis--heart disease and other anomalies.

    Science.gov (United States)

    Palma, G; Giordano, R; Russolillo, V; Vosa, C

    2010-10-01

    This report describes a rare case of left pulmonary agenesis associated with congenital diaphragmatic hernia and congenital heart disease in a 2-year-old child with pulmonary hypertension. We performed direct radical correction of the congenital heart defect. The postoperative course was challenging but without major complications.

  1. 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.

  2. Callosal Agenesis and Interhemispheric Cysts

    OpenAIRE

    J Gordon Millichap

    2001-01-01

    Imaging studies of 25 cases of agenesis of the corpus callosum with interhemispheric cyst were retrospectively reviewed at the University of California, San Francisco, and Harvard Medical School, Boston.

  3. 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.

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

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    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.

  5. Agenesis of the lung--a rare congenital anomaly of the lung.

    Science.gov (United States)

    De, Arun

    2013-01-01

    Pulmonary agenesis is a very rare condition and many of them are associated with a variety of cardiac and non-cardiac malformations. We report an eight-month old girl with chronic lung infection due to right sided pulmonary agenesis without any associated major cardiac or non-cardiac abnormalities. The case brings in forth the importance of investigating any infant presenting with features of chronic lung infection for any congenital abnormality of the lung including aplasia of the lung. This case also emphasizes that mildness of the attack does not exclude right sided aplasia of the lung.

  6. Agenesis of the Lung: A Rare Congenital Anomaly of the Lung

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    Arun De

    2013-01-01

    Full Text Available Pulmonary agenesis is a very rare condition and many of them are associated with a variety of cardiac and non-cardiac malformations. We report an eight-month old girl with chronic lung infection due to right sided pulmonary agenesis without any associated major cardiac or non-cardiac abnormalities. The case brings in forth the importance of investigating any infant presenting with features of chronic lung infection for any congenital abnormality of the lung including aplasia of the lung. This case also emphasizes that mildness of the attack does not exclude right sided aplasia of the lung.

  7. Lung Aplasia with Pulmonary Artery Sling Like Anomaly

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    Onur Isik

    2016-04-01

    Full Text Available Unilateraly pulmonary agenesis is an extremely rare documented congenital anomaly. It may be defined as total absence of the pulmonary parencilyma, its vascular supporting structure, and bronchi beyond the bifurcation. The present case with reconstructed images is one of the different cases of agenesis reported in the literature.

  8. [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.

  9. Isolated complete corpus callosal agenesis

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    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.

  10. Agenesis of the iliac veins.

    Science.gov (United States)

    Thomas, M L; Posniak, H V

    1984-01-01

    Three case reports of patients with the rare anomaly of agenesis of the iliac veins are presented. It is emphasised that full phlebographic investigation should be carried out in such patients before surgical treatment is considered. It is pointed out that surgical ablation may exacerbate the symptoms of leg swelling and varicose veins for which the patients seek advice.

  11. 高热、咳嗽、呼吸困难、双肺多发实变%High Fever,Cough,Dyspnea and Bilateral Multiple Pulmonary Consolidation

    Institute of Scientific and Technical Information of China (English)

    余碧芸; 吴宏成; 汤耀东; 周莹艳; 汪春年

    2015-01-01

    临床有多种疾病可因双肺多发实变而发生急性呼吸衰竭,但病情危重病理检查又无法提供明确诊断时会让临床医师感到棘手。本文报道1例高热、咳嗽、呼吸困难、双肺多发实变的危重患者,经皮肺穿刺活检病理检查后诊断仍不明确,笔者所在科室对其临床资料进行了分析讨论,临床考虑为重症隐源性机化性肺炎或急性间质性肺炎,最终抢救成功。%many diseases cause acute respiratory failure as a result of bilateral multiple pulmonary consolidation,and it puts physicians in a difficult situation when the patients is in critical condition and the pathological examination can′t provide a definite diagnosis. This paper reported a case of a critical patient with high fever, cough, dyspnea and bilateral multiple pulmonary consolidation. The pathological examination by percutaneous pulmonary biopsy could not give a definite diagnosis. After the analysis of the clinical data,the possibility of critical cryptogenic organizing pneumonia or acute interstitial pneumonia was considered,and the patient was successfully salvaged.

  12. 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.

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

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    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.

  14. Prevalence and Characteristics of Tooth Agenesis in Permanent Dentition of Subjects from Tîrgu Mureș

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    Bernadette Kerekes-Máthé

    2013-08-01

    Full Text Available Background: Identification of the characteristics of tooth agenesis is an important component in the understanding of the ethiology of this common developmental dental anomaly. The aim of the present study was to determine the prevalence and the characteristics of tooth agenesis among patients from Tîrgu Mureș. Material and method: The present study is retrospective and descriptive, based on the evaluation of the patient’s dental records from 2004 to 2012 belonging to a pediatric dental office from Tîrgu Mureș. Orthopantomograms and anamnestic data of 947 children and young adults (365 male and 582 female were analyzed. Results: The prevalence of tooth agenesis, excluding third molars, was 7.39%. The difference between the genders was statistically not significant (p = 0.09. The most frequently missing teeth were the upper second incisors, followed by the lower second premolars. The difference between the distribution of agenesis in the upper and lower jaws was statistically significant (p <0.0001. The distribution of dental agenesis between the anterior region and the lateral region of the maxilla and the mandible showed significant differences (p <0.0001. Symmetrical distribution of tooth agenesis was found more frequently (54.54% than assymetrical distribution. Conclusions: The maxillary anterior region and the mandibular lateral region were the most affected by dental agenesis in the permanent dentition of the studied population. The maxilla was more affected than the mandible and bilateral forms of agenesis were more frequently than unilateral forms. The more extreme forms of agenesis were found amongst female

  15. Combined spinal epidural anesthesia for cesarean section in a patient with left lung agenesis.

    Science.gov (United States)

    Babita, G; Rashmi, M; Deb, K

    2001-04-01

    Agenesis of the lung is a rare developmental defect characterized by complete absence of one lung, or hypoplasia of one or both lungs. The majority of reported cases have been associated with other congenital anomalies. It is usually detected in childhood as a result of either pulmonary symptoms or associated anomalies. Although pulmonary agenesis has been reported in adults, there is no report of the management of a pregnant patient for cesarean section in the anesthetic literature. We report the management of a 23-year-old pregnant female at 36 weeks' gestation with oligohydramnios and left lung agenesis for elective cesarean section. She presented with a history of repeated chest infection and was found to have restrictive lung function. She had no other congenital anomalies. Combined spinal epidural anesthesia was administered for cesarean section. The intraoperative course was uneventful except for bradyarrhythmias followed by tachyarrhythmias on manipulation of the uterus. She was given low dose epidural morphine with local anesthetic and intramuscular diclofenac for postoperative analgesia. We believe that normal pregnancy can safely be undertaken in the presence of left lung agenesis. In view of associated congenital anomalies in more than 50% of the cases, anesthetic management should include a particularly careful survey of other systems. The patient should otherwise be managed like any patient with limited pulmonary reserve. Combined spinal epidural anesthesia is safe and appropriate for such patients.

  16. Complete agenesis of dorsal pancreas

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    Malwinder Singh

    2014-04-01

    Full Text Available Introduction: Complete agenesis of body and tail of pancreas is a very rare type of developmental anomaly of pancreas. It is important regarding its presentations of diabetes mellitus, pancreatitis, and exocrine insufficiency. Case Report: An old man had presented with atypical symptoms of obstructive jaundice with exocrine insufficiency. CECT helped to reveal the complete absence of the body and tail of pancreas with radiologically normal head with no signs of pancreatitis or mass lesion. Conclusions: The cause of agenesis of the dorsal pancreas is currently not well understood. It can also present lately as the presenting case. The presentations are usually related to secretory malfunctions. CECT is an initial investigation for diagnosis

  17. Coincidence of congenital agenesis of left lung and common atrium: a very rare case.

    Science.gov (United States)

    Nabati, Maryam; Bagheri, Babak; Habibi, Valiolla

    2013-05-01

    Pulmonary agenesis is characterized by undeveloped pulmonary vessels, bronchi as well as lung parenchyma and is a rare congenital anomaly with unknown etiology. It is usually diagnosed during childhood. Nearly, one third of these patients have concomitant congenital heart diseases. While more than 50% of these patients die before the age of 5 years, some other patients may be asymptomatic throughout their life. On the other hand, common atrium, another rare congenital anomaly, is characterized by complete absence of the atrial septum and is usually accompanied by atrioventricular valve malformations. An 18-year-old male presented with concomitant pulmonary agenesis and common atrium and had not undergone surgery due to high risk for mortality.

  18. Isolated complete corpus callosal agenesis

    OpenAIRE

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

    2014-01-01

    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 an...

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

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    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.

  20. Agenesia isolada da artéria pulmonar direita ou esquerda: avaliação da evolução natural e a longo prazo, após intervenção corretiva Isolated unilateral pulmonary artery agenesis: evaluation of natural and long term evolution after corrective surgery

    Directory of Open Access Journals (Sweden)

    Edmar Atik

    2006-10-01

    Full Text Available OBJETIVO: Agenesia da artéria pulmonar, direita ou esquerda, rara como anomalia isolada, foi relatada em 119 casos desde 1978. Apresenta-se clinicamente com hipertensão pulmonar (HP na infância e com hemoptise no adulto. Intervenções como reconstrução arterial pulmonar e lobectomia foram realizadas em 17% dos casos. Analisamos quatro destes casos, sendo dois em evolução natural e dois com regressão da HP, após longo tempo da correção operatória. MÉTODOS: Quatro crianças, três do sexo masculino, com 22,10 e 35 meses de idade e uma do sexo feminino com 20 meses, com insuficiência cardíaca direita (ICD e cianose na primeira e ICD na segunda e cansaço aos esforços nas outras duas. Todas tinham sinais de HP, sobrecarga ventricular direita no ECG e cardiomegalia. Cateterismo cardíaco mostrou pressões sistêmicas na artéria pulmonar contralateral na agenesia à direita em três e na agenesia à esquerda em um caso. RESULTADOS: Restabelecimento cirúrgico da continuidade arterial pulmonar foi possível em dois casos, na criança de 22 e na de 10 meses de idade, pela interposição de Goretex de diâmetro de 7 mm entre as artérias pulmonares até o hilo pulmonar contralateral hipoplásico. Houve regressão dos sinais de HP em período imediato e tardio, com 7 e 2,5 anos de idade respectivamente. A relação das pressões entre os ventrículos direito e esquerdo era de 30 e 40%, nos dois casos. A perfusão pulmonar aumentou de 8 para 44% e de 8 para 23%, nos dois casos. O mesmo procedimento foi programado para os outros pacientes. CONCLUSÃO: Esta técnica se torna a operação de escolha para casos similares, raramente descritos na literatura, mesmo em presença de acentuada HP e hipoplasia arterial pulmonar contralateral.OBJECTIVE: Unilateral pulmonary artery agenesis is an uncommon isolated anomaly and since 1978 only 119 cases have been reported. In general the condition presents as pulmonary hypertension (PH in children and

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

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    Sangeeta Arya

    2013-04-01

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

  2. Gallbladder agenesis in a Chihuahua.

    Science.gov (United States)

    Kamishina, Hiroaki; Katayama, Masaaki; Okamura, Yasuhiko; Sasaki, Jun; Chiba, Satoshi; Goryo, Masanobu; Sato, Reeko; Yasuda, Jun

    2010-07-01

    A 4-year-old neutered male Chihuahua was presented with a history of anorexia and vomiting. Continuous elevation of liver enzymes was found on repeated blood examinations and the dog was referred to us for further evaluation. The absence of gallbladder was suspected on ultrasonography. Exploratory laparotomy and retrograde cholangiography confirmed gallbladder agenesis and a possible hypoplasia of the right medial and lateral liver lobes. Histologically, proliferation of bile ductules associated with portal fibrosis and pseudolobular formation were apparent in the liver lobes.

  3. 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

  4. 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.

  5. Unilateral absence of a pulmonary artery: Report of 3 cases

    Energy Technology Data Exchange (ETDEWEB)

    Bae, Oh Keun; Choi, Chul Seung; Choi, Yo Won; Jeon, Seok Cheol; Seo, Heung Suk; Hahm, Chang Kok [College of Medicine, Hanyang University, Seoul (Korea, Republic of)

    1994-07-15

    Unilateral absence of a pulmonary artery is an uncommon anomaly, which presents as an isolated lesion or in combination with other congenital heart disease such as TOF or PDA. We encountered three cases of isolated unilateral absence of a pulmonary artery; one was left pulmonary artery agenesis with right sided aortic arch and the others were right pulmonary artery agenesis with left sided aortic arch. Plain chest radiograph showed considerable loss of unilateral lung volume and lack of ipsilateral hilar shadow. Pulmonary angiogram which was done in two cases, revealed proximal interruption of a pulmonary artery. Chest CT was done in only one case, on which right pulmonary artery was absent and was replaced by adipose tissue. CT with its clean demonstration pulmonary artery without any evidence of acquired obstruction of a pulmonary artery by pulmonary embolism or tumor invasion, maybe a valuable method for evaluation of the unilateral absence of a pulmonary artery.

  6. 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

  7. Interhemispheric Connections between the Primary Visual Cortical Areas via the Anterior Commissure in Human Callosal Agenesis.

    Science.gov (United States)

    van Meer, Nathalie; Houtman, Anne C; Van Schuerbeek, Peter; Vanderhasselt, Tim; Milleret, Chantal; Ten Tusscher, Marcel P

    2016-01-01

    Aim: In humans, images in the median plane of the head either fall on both nasal hemi-retinas or on both temporal hemi-retinas. Interhemispheric connections allow cortical cells to have receptive fields on opposite sides. The major interhemispheric connection, the corpus callosum, is implicated in central stereopsis and disparity detection in front of the fixation plane. Yet individuals with agenesis of the corpus callosum may show normal stereopsis and disparity vergence. We set out to study a possible interhemispheric connection between primary visual cortical areas via the anterior commissure to explain this inconsistency because of the major role of these cortical areas in elaborating 3D visual perception. Methods: MRI, DTI and tractography of the brain of a 53-year old man with complete callosal agenesis and normal binocular single vision was undertaken. Tractography seed points were placed in both the right and the left V1 and V2. Nine individuals with both an intact corpus callosum and normal binocularity served as controls. Results: Interhemispheric tracts through the anterior commissure linking both V1 and V2 visual cortical areas bilaterally were indeed shown in the subject with callosal agenesis. All other individuals showed interhemispheric visual connections through the corpus callosum only. Conclusion: Callosal agenesis may result in anomalous interhemispheric connections of the primary visual areas via the anterior commissure. It is proposed here that these connections form as alternative to the normal callosal pathway and may participate in binocularity.

  8. Diagnostic value of echocardiography in isolated congenital unilateral lung agenesis combined with different anomalies: Two rare cases in children.

    Science.gov (United States)

    He, Huan; Sun, Feifei; Ren, Weidong; Wang, Yonghuai; Xu, Shu

    2016-10-01

    Unilateral lung agenesis, a rare congenital deformity, is secondary to abnormal embryonic development, leading to a shift of the mediastinum and remaining lung. In our case reports, we describe the diagnostic courses of two pediatric patients with unilateral lung agenesis admitted to our hospital in 2014. We present and compare the findings in transthoracic echocardiography with that in CT. Echocardiography played a pivotal role in revealing pulmonary deformities and leading to the diagnosis. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:514-517, 2016.

  9. Clinical Correlates of Corpus Callosal Agenesis

    OpenAIRE

    J Gordon Millichap

    1998-01-01

    The clinical correlates of agenesis of the corpus callosum were examined in 56 cases (37 adult, 19 pediatric) reported to the British Neurological Surveillance Unit (BNSU) and surveyed at the Department of Psychological Medicine, Institute of Psychiatry, London, UK.

  10. Unilateral Absence of the Pulmonary Artery Incidentally Found in Adulthood

    Directory of Open Access Journals (Sweden)

    Cenk Aypak

    2012-01-01

    Full Text Available Unilateral absence of the pulmonary artery (UAPA or pulmonary artery agenesis is a rare congenital disorder presenting with a wide spectrum of symptoms. UAPA is usually associated with cardiac anomalies and surgically treated in childhood. We report a rare case of a 50-year-old woman who was diagnosed with left pulmonary artery agenesis without any other cardiac anomalies. Clinicians should be aware of the possibility of undiagnosed cases of UAPA in patients through a chest radiograph that suggests the diagnosis. Confirmation of the diagnosis and anatomic details can be discerned by CT scanning.

  11. 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

  12. Radiographic assessment of agenesis, impaction, and pararadicular radiolucencies in relation with third molar in Nashik City of Maharashtra

    Directory of Open Access Journals (Sweden)

    Bhushan Sukdeo Ahire

    2016-01-01

    Full Text Available Introduction: The third molar develops entirely after birth and also it is the last tooth to erupt in all ethnic groups despite racial variations in the eruption sequence. Tooth development with effects on tooth size, shape, position, and total absence is affected due to environmental factors, systemic diseases, genetic polymorphisms, and teratogens. Aims and Objectives: The aim of this study was to assess the prevalence of agenesis, impaction, and pararadicular radiolucencies in relation with third molars. Materials and Methods: One hundred digital orthopantomograph (OPG scans of patients of age ranging from 18 to 25 years were selected randomly from the digital OPG database of the Department of Oral Medicine and Radiology, and then assessment and analysis were carried out. Results: Overall agenesis rate was about 14.5%. Bilateral agenesis of maxillary and mandibular third molars was about 14% and 13%, respectively. Overall impaction rate was about 21.92%. Mesioangular impaction rate was about 57.33% whereas distoangular impaction rate was about 26.66%. Prevalence of pararadicular radiolucencies was only about 0.8% with mandibular predilection. Conclusion: As the agenesis of the third molars is increasing, they can be considered as vestigial. Prevalence of impaction of the third molars is increasing. Pararadicular radiolucencies are mostly seen with mandibular third molar.

  13. Callosal agenesis followed postnatally after prenatal diagnosis.

    Science.gov (United States)

    Imataka, George; Nakagawa, Eiji; Kuwashima, Shigeko; Watanabe, Hiroshi; Yamanouchi, Hideo; Arisaka, Osamu

    2006-09-01

    Callosal agenesis is a congenital brain anomaly caused by embryonal hypogenesis of the corpus callosum. Concerning the neurological prognosis, epilepsy and motor disturbance are noted in some cases, while many cases are asymptomatic and the prognosis is good. We report a fetus tentatively diagnosed with hydrocephaly on prenatal echo-encephalography, which was performed without adequate explanation to and understanding of the parents. The parents had not expected an abnormality before the screening, and were subsequently not psychologically prepared for the discovery of the congenital brain anomaly on imaging. Moreover, they received no guidance on how to deal with any possible abnormalities. The pregnant mother was referred to our hospital. Prenatal MRI was performed after informed consent was obtained, and the fetus was diagnosed with callosal agenesis. The patient was followed for 5 years, and neurological development was normal. However, the parents have remained anxious while raising the child. Thus, the prenatal diagnosis of callosal agenesis in this case caused unnecessary mental burden to the parents. Here, we report the course of the case, and discuss the way prenatal ultrasonography should be used as a prenatal screening method, and the importance of counseling before the test.

  14. Parkinsonism "Plus" Syndrome With Incidental Corpus Callosal Agenesis

    Directory of Open Access Journals (Sweden)

    Shanbhogue K R

    1999-01-01

    Full Text Available A case of Parkinsonism ′Plus′ syndrome with cranial CT scan and MRI showing corpus callosal agenesis is reported. There were no clinical signs of callosal separation. The causes for the discrepancies between the lack of clinical signs of callosal separation and imaging appearances are discussed. The callosal agenesis is in all probability incidental.

  15. Parkinsonism "Plus" Syndrome With Incidental Corpus Callosal Agenesis

    OpenAIRE

    Shanbhogue K R; Bennett D; Shankar V; Murugesan S; Stephen A S K; Karnan B; Srikanth S

    1999-01-01

    A case of Parkinsonism ′Plus′ syndrome with cranial CT scan and MRI showing corpus callosal agenesis is reported. There were no clinical signs of callosal separation. The causes for the discrepancies between the lack of clinical signs of callosal separation and imaging appearances are discussed. The callosal agenesis is in all probability incidental.

  16. Agenesis of internal carotid artery associated with congenital anterior hypopituitarism

    Energy Technology Data Exchange (ETDEWEB)

    Moon, W.-J. [Department of Diagnostic Radiology, Samsung Medical Center, Seoul (Korea); Institute of Neuroradiology, University of Frankfurt (Germany); Porto, L.; Lanfermann, H.; Zanella, F.E. [Institute of Neuroradiology, University of Frankfurt (Germany); Weis, R. [Department of Pediatric Neurology, University of Frankfurt (Germany)

    2002-02-01

    We report a rare case of unilateral agenesis of the internal carotid artery in association with congenital anterior hypopituitarism. The collateral circulation is supplied by a transsellar intercavernous anastomotic vessel connecting the internal carotid arteries. These abnormalities are well depicted on MRI and MRA. The agenesis of the internal carotid artery may explain the pathogenesis of some of congenital anterior hypopituitarism. (orig.)

  17. Management of a Patient With Tetralogy of Fallot, Congenital Diaphragmatic Hernia, and Complete Left Lung Agenesis.

    Science.gov (United States)

    Labovsky, Kristen; Hoffman, George; Scott, John

    2016-07-01

    We describe the rare case of an infant with congenital diaphragmatic hernia, unilateral lung agenesis, and unpalliated single-ventricle physiology. Infants with congenital diaphragmatic hernia and parallel circulation are at risk for maldistribution of systemic and pulmonary blood flow. Optimal perioperative management should include an assessment of the ratio of pulmonary to systemic blood flow (Qp:Qs). Traditionally, arterial and systemic venous oxygen (SvO2) saturations are needed to calculate Qp:Qs. However, in this case, SvO2 measurement was not feasible. On the basis of a previously described relationship, we used 2-site near-infrared spectroscopy to calculate a near-infrared spectroscopy-derived SvO2, which was then used to estimate Qp:Qs and guide goal-directed interventions.

  18. 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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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.

  20. Unilateral Maxillary Canine Agenesis: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Nagihan Koç

    2014-01-01

    Full Text Available Congenital absence of maxillary permanent canines is an extremely rare condition, which may appear as part of a syndrome or as a nonsyndromic form. Nonsyndromic canine agenesis combined with other types of tooth agenesis has occasionally been described in the literature but isolated cases are rarely observed. This report presents an isolated case of maxillary permanent canine agenesis in a healthy 18-year-old female patient and a literature review on the prevalence, etiology, and differential diagnosis of the condition.

  1. Diffuse pulmonary infiltrates in immunocompromised patients

    NARCIS (Netherlands)

    Fijen, JW; van der Werf, TS; Ligtenberg, JJM; Tulleken, JE; Zijlstra, JG

    1999-01-01

    The differential diagnosis of bilateral interstitial pulmonary infiltrates in immunocompromised patients is very extensive. We describe two immunocompromised patients with diffuse pulmonary infiltrative changes. Bronchoscopic bronchoalveolar lavage after orotracheal intubation using topical anaesthe

  2. Unilateral and bilateral dental transpositions in the maxilla

    DEFF Research Database (Denmark)

    Danielsen, Jakob Christian; Karimian, K; Ciarlantini, R;

    2015-01-01

    AIM: This was to elucidate dental and skeletal findings in individuals with unilateral and bilateral maxillary dental transpositions. MATERIAL AND METHODS: The sample comprised of radiographic materials from 63 individuals with maxillary dental transpositions from the Departments of Odontology...... and lateral incisor (Type 2). The dentitions were analysed regarding agenesis and dental morphological anomalies on panoramic radiographs, and craniofacial aspects were cephalometrically analysed on profile images The results were statistically evaluated. RESULTS: All groups demonstrated increased occurrences...... retrognathia (more pronounced in Type 1B). Type 2 showed a significant posterior inclination of the maxilla. CONCLUSION: Transpositions of maxillary canines involve dental and skeletal deviations. Dental deviations were predominantly taurodontic root morphology and agenesis. Regarding skeletal deviations...

  3. Interhemispheric transfer of kinesthetic information and line bisection task performance in patient with callosal agenesis.

    Science.gov (United States)

    Makashvili, M; Chichinadze, K; Domianidze, T

    2009-09-01

    Patient G.J., male, 7 yrs, with callosal agenesis, was found perfectly able to cross-replicate hand postures in right-to left and left-to-right directions. Bimanual coordination as well as touch localization and intermanual matching were performed without errors. He failed to name 2 out of 8 objects, palpated with the left hand. At the age of 13 patient performed like normal controls in line bisection task, was successful in intermanual replication of hand postures and intermanual matching while failed to name 8 out of 12 familiar objects palpated with the left hand. G.J.'s case does not support idea about bilateral presentation of language centers and development of compensatory ipsilateral afferents in patients with callosal agenesis. Presence of anterior and interictal commissures in G.J. did not contribute to the exchange of information between sensory areas of the right hemisphere and language centers of the left half brain. However, normal intermanual matching and replication of hand postures, as well as high level of line bisection task performance suggests, that anterior and/or intertectal commissure could contribute to the functional integration of sensory areas of the two hemispheres.

  4. Prenatal diagnosis of a fetus with anencephaly and thumb agenesis.

    Science.gov (United States)

    Barone, Chiara; Bartoloni, Giovanni; Cataliotti, Antonella; Indaco, Lara; Pappalardo, Elisa; Barrano, Barbara; Ettore, Giuseppe; Bianca, Sebastiano

    2012-03-01

    Severe anomalies of the forebrain together with reduction limb anomalies are a rare congenital anomalies association. We report a prenatal diagnosis of acalvaria, anencephaly and thumb agenesis in a voluntary terminated fetus and discuss the role of genetic counseling.

  5. Delusional Disorder in a Patient with Corpus Callosum Agenesis

    Science.gov (United States)

    Saha, Rashmita; Doval, Nimisha

    2016-01-01

    Agenesis of corpus callosum is rare and associated neuropsychiatric abnormalities reported are epilepsy, Asperger’s syndrome, learning problems, depression, schizophrenia, conduct disorder and conversion symptoms. Schizophrenia is the most common psychiatric disorder reported among corpus callosum agenesis. We report a rare case of delusional disorder with corpus callosum agenesis and seizure disorder. The patient presented with delusions of persecution towards younger brother and mother, disturbed sleep and reduced appetite. She had a history of seizure disorder of ten years duration, which was controlled with carbamazepine and levetiracetam. Neurological examination was normal. On MRI, corpus callosum agenesis was detected. She was put on an atypical antipsychotic quetiapine to which her psychiatric symptoms responded completely. PMID:28208982

  6. Agenesis of the corpus callosum and autism: a comprehensive comparison

    OpenAIRE

    Paul, Lynn K.; Corsello, Christina; Kennedy, Daniel P.; Adolphs, Ralph

    2014-01-01

    The corpus callosum, with its ∼200 million axons, remains enigmatic in its contribution to cognition and behaviour. Agenesis of the corpus callosum is a congenital condition in which the corpus callosum fails to develop; such individuals exhibit localized deficits in non-literal language comprehension, humour, theory of mind and social reasoning. These findings together with parent reports suggest that behavioural and cognitive impairments in subjects with callosal agenesis may overlap with t...

  7. 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 ...

  8. Early visual evoked potentials in callosal agenesis.

    Science.gov (United States)

    Barr, Melodie S; Hamm, Jeff P; Kirk, Ian J; Corballis, Michael C

    2005-11-01

    Three participants with callosal agenesis and 12 neurologically normal participants were tested on a simple reaction time task, with visual evoked potentials collected using a high-density 128-channel system. Independent-components analyses were performed on the averaged visual evoked potentials to isolate the components of interest. Contrary to previous research with acallosals, evidence of ipsilateral activation was present in all 3 participants. Although ipsilateral visual components were present in all 4 unilateral conditions in the 2 related acallosal participants, in the 3rd, these were present only in the crossed visual field-hand conditions and not in the uncrossed conditions. Suggestions are made as to why these results differ from earlier findings and as to the neural mechanisms facilitating this ipsilateral activation.

  9. 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

  10. Current status of pulmonary metastasectomy

    DEFF Research Database (Denmark)

    Hornbech, Kåre; Ravn, Jesper; Steinbrüchel, Daniel Andreas

    2011-01-01

    for unresected patients. In this article, we discuss the current status on pulmonary metastasectomy. Preoperative assessment and selection of surgical candidates is covered. The different surgical strategies including surgical approach, unilateral versus bilateral exploration, lymph node dissection, and repeat...

  11. Agenesis of the corpus callosum and autism: a comprehensive comparison.

    Science.gov (United States)

    Paul, Lynn K; Corsello, Christina; Kennedy, Daniel P; Adolphs, Ralph

    2014-06-01

    The corpus callosum, with its ∼200 million axons, remains enigmatic in its contribution to cognition and behaviour. Agenesis of the corpus callosum is a congenital condition in which the corpus callosum fails to develop; such individuals exhibit localized deficits in non-literal language comprehension, humour, theory of mind and social reasoning. These findings together with parent reports suggest that behavioural and cognitive impairments in subjects with callosal agenesis may overlap with the profile of autism spectrum disorders, particularly with respect to impairments in social interaction and communication. To provide a comprehensive test of this hypothesis, we directly compared a group of 26 adults with callosal agenesis to a group of 28 adults with a diagnosis of autism spectrum disorder but no neurological abnormality. All participants had full-scale intelligence quotient scores >78 and groups were matched on age, handedness, and gender ratio. Using the Autism Diagnostic Observation Schedule together with current clinical presentation to assess autistic symptomatology, we found that 8/26 (about a third) of agenesis subjects presented with autism. However, more formal diagnosis additionally involving recollective parent-report measures regarding childhood behaviour showed that only 3/22 met complete formal criteria for an autism spectrum disorder (parent reports were unavailable for four subjects). We found no relationship between intelligence quotient and autism symptomatology in callosal agenesis, nor evidence that the presence of any residual corpus callosum differentiated those who exhibited current autism spectrum symptoms from those who did not. Relative to the autism spectrum comparison group, parent ratings of childhood behaviour indicated children with agenesis were less likely to meet diagnostic criteria for autism, even for those who met autism spectrum criteria as adults, and even though there was no group difference in parent report of current

  12. Outcomes after singleversus bilateral lung transplantation for idiopathic pulmonary fibrosis%单肺与双肺移植治疗特发性肺间质纤维化的临床研究

    Institute of Scientific and Technical Information of China (English)

    孙敏; 李慧星; 谢于峰; 徐辉; 刘峰; 陈静瑜

    2016-01-01

    Objective To compare outcomes after single and bilateral lung transplantation in patients with idiopathic pulmonary fibrosis (IPF).Methods 104 patients with IPF who underwent lung transplantation at Wuxi People' s Hospital from January 2008 to February 2015 were evaluated.Recipients were divided into single lung transplantation (STL)group(41 cases)and bilateral lung transplantation(BLT) group (63 cases).Preoperative characteristics,postoperative outcomes,postoperative complications,functional outcomes,survival and causes of death between the two groups were analyzed retrospectively.The patients were followed up by letter,call and outpatient visit.Results Preoperative characteristics:The SLT group were significantly older (P < 0.05),and had significantly higher systolic (P < 0.05) pulmonary artery pressures than the BLT group.Postoperative outcomes:The intraoperative blood loss of SLT group as significantly less than BLT group (P < 0.05).The cold ischemic time of SLT group as significantly shorter than BLT group (P < 0.05).The BLT group was significantly more frequently required extracorporeal membrane oxygenation (ECMO) support than SLT group (P < 0.05).There was no statistically significant difference inventilator support,reoperation,reintubation,length of ICU stay and death between the SLT and the BLT group (P > 0.05).Postoperative complications:The BLT group had a higher incidence of primary graft dysfunction than the SLT group (P < 0.05).There was no statistically significant difference in chest complications,acute rejection,airway complications,pulmonary infection between the SLT and the BLT group (P>0.05).Functional outcomes:FVC%,FEV1%,DLCO and 6-MWD,were significantly higher in the BLT group than the SLT group (P <0.05).Survival:The overall survival rates at 1,2 and 3 years were not different between the SLT and the BLT group (75.3%,61.6% and 46.2% vs 79.1%,68.6% and 53.4%,P > 0.05).Conclusions The lung transplantationis

  13. Polytopic anomalies with agenesis of the lower vertebral column

    NARCIS (Netherlands)

    Bohring, Axel; Lewin, Susan O.; Reynolds, James F.; Voigtländer, Theda; Rittinger, Olaf; Carey, John C.; Köpernik, Michael; Smith, Rosemarie; Zackai, Elaine H.; Leonard, Norma J.; Gritter, Hilda L.; Bamforth, J. Stephen; Okun, Nannette; McLeod, D. Ross; Super, Maurice; Powell, Peter; Mundlos, Stefan; Hennekam, Raoul C.M.; Van Langen, Irene M.; Viskochil, David H.; Wiedemann, Hans-Rudolf; Opitz, John M.

    1999-01-01

    We describe clinical, pathological and radiological findings in 15 cases of sporadic and familial lower spine agenesis with additional anomalies of the axial skeleton and internal organs and speculate about the cause and pathogenesis of this malformation complex. We show that all of these findings a

  14. Anterior commissure absence without callosal agenesis: a new brain malformation.

    Science.gov (United States)

    Mitchell, T N; Stevens, J M; Free, S L; Sander, J W; Shorvon, S D; Sisodiya, S M

    2002-04-23

    The authors report a novel human brain malformation characterized by the absence of the anterior commissure without callosal agenesis, but associated with gross unilateral panhemispheric malformation incorporating subependymal heterotopia, subcortical heterotopia, and gyral abnormalities including temporal malformation and polymicrogyria. In contrast, a normal anterior commissure was found in 125 control subjects and in 113 other subjects with a range of brain malformations.

  15. Language and Development in FG Syndrome with Callosal Agenesis.

    Science.gov (United States)

    McCardle, Peggy; Wilson, Bruce

    1993-01-01

    The FG syndrome is characterized by unusual facies; sudden infant death; developmental delay; and abnormalities of the cardiac, gastrointestinal, and central nervous systems. Serial evaluations of one case with isolated agenesis of the corpus callosum found consistent patterns over time in specific language impairments in syntactic and…

  16. C syndrome with skeletal anomalies, mental retardation, eyelid chalazion, Bitot’s spots and agenesis of the corpus callosum in an Egyptian child

    Directory of Open Access Journals (Sweden)

    Rabah M. Shawky

    2017-01-01

    Full Text Available We report a 2.5 year old female child, third in order of birth of healthy non consanguineous Egyptian parents with C syndrome. The patient had moderate mental retardation, trigonocephaly, protruding forehead, low anterior hair line, wide upslanted palpebral fissures, depressed nasal bridge, broad nose, high arched palate, microretrognathia, low set ears, short neck, scoliosis, hypertrichosis over the back, talipes equinovarus as well as interatrial septal defect. The patient had in addition chalazion in left lower eyelid as well as bilateral Bitot’s spots most probably due to vitamin A deficiency. MRI brain revealed agenesis of the corpus callosum.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. Bilateral ankle edema with bilateral iritis.

    Science.gov (United States)

    Kumar, Sunil

    2007-07-01

    I report two patient presented to me with bilateral symmetrical ankle edema and bilateral acute iritis. A 42-year-old female of Indian origin and 30-year-old female from Somalia both presented with bilateral acute iritis. In the first patient, bilateral ankle edema preceded the onset of bilateral acute iritis. Bilateral ankle edema developed during the course of disease after onset of ocular symptoms in the second patient. Both patients did not suffer any significant ocular problem in the past, and on systemic examination, all clinical parameters were within normal limit. Lacrimal gland and conjunctival nodule biopsy established the final diagnosis of sarcoidosis in both cases, although the chest x-rays were normal.

  19. Predictors of Cardiogenic and Non-Cardiogenic Causes in Cases with Bilateral Chest Infiltrates

    OpenAIRE

    Lee, Yeon Joo; Lee,Jinwoo; Park, Young Sik; Lee, Sang-min; Yim, Jae-Joon; Yoo, Chul-Gyu; Kim, Young Whan; Han, Sung Koo; Lee, Chang-Hoon

    2013-01-01

    Background Differentiating cardiogenic pulmonary edema from other bilateral lung diseases such as pneumonia is frequently difficult. We conducted a retrospective study to identify predictors for cardiogenic pulmonary edema and non-cardiogenic causes of bilateral lung infiltrates in chest radiographs. Methods The study included patients who had newly developed bilateral lung infiltrates in chest radiographs and patients who underwent echocardiography. Cases were divided into two groups based o...

  20. Assessing prenatal white matter connectivity in commissural agenesis.

    Science.gov (United States)

    Kasprian, Gregor; Brugger, Peter C; Schöpf, Veronika; Mitter, Christian; Weber, Michael; Hainfellner, Johannes A; Prayer, Daniela

    2013-01-01

    Complete or partial agenesis of the corpus callosum are rather common developmental abnormalities, resulting in a wide spectrum of clinical neurodevelopmental deficits. Currently, a significant number of these cases are detected by prenatal sonography during second trimester screening examinations. However, major uncertainties about a detailed morphological diagnosis and the clinical significance do not allow accurate prenatal counselling. Here, we were able to demonstrate the 3D connectivity of aberrant commissural tracts in 16 cases with complete and four cases with partial callosal agenesis using the foetal magnetic resonance imaging techniques of diffusion tensor imaging and tractography in utero and in vivo between gestational weeks 20 and 37. The 'misguided' pre-myelinated callosal axons that represent the bundle of Probst were non-invasively visualized, and they showed a degree of structural integrity similar to that of the callosal pathways of age-matched foetuses without cerebral pathologies. In two foetuses, we were able to prove, by post-mortem histology, that diffusion tensor imaging allows the depiction of the bundle of Probst, even during early stages of pre-myelination at 20 and 22 gestational weeks. In cases with partial callosal agenesis, an aberrant sigmoid-shaped bundle was prenatally depicted, confirming the findings of heterotopic interhemispheric connectivity in adults with partial callosal agenesis. In addition to the corpus callosum, other white matter pathways were also involved, including somatosensory and motor pathways that showed significantly higher fractional anisotropy values in cases with callosal agenesis compared with control subjects. A detailed prenatal assessment of abnormal white matter connectivity in cases of midline anomalies will help to explain and understand the clinical heterogeneity in these cases, taking future foetal neurological counselling strategies to a new level.

  1. [Pleomorphic adenoma with bilateral pulmonary metastasis].

    Science.gov (United States)

    De Kerangal, X; Poirrier, P; Soulard, R; Dot, J M; Ségneuric, J B; L'Her, P; Jancovici, R; Saint-Blancart, P

    2001-11-01

    We report a new case of pleomorphous adenoma of the submaxillary glands with multiple lung metastases. Histological proof was obtained on the thoracoscopic surgical specimen. Clinically, this benign tumor presents as a malignant tumor. Diagnosis has been a subject of debate; surgical resection is indicated. Diagnosis is achieved by elimination in a patient with one or several nodules occurring in a context of recurrent pleomorphous adenoma.

  2. 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.

  3. Dental anomalies in an orthodontic patient population with maxillary lateral incisor agenesis

    Science.gov (United States)

    Citak, Mehmet; Cakici, Elif Bahar; Benkli, Yasin Atakan; Cakici, Fatih; Bektas, Bircan; Buyuk, Suleyman Kutalmış

    2016-01-01

    ABSTRACT Introduction: The purpose of this study was to evaluate the prevalence of dental anomalies in a subpopulation of orthodontic patients with agenesis of maxillary lateral incisors (MLI). Methods: The material of the present study included the records of the 1964 orthodontic patients. Panoramic radiographs and dental casts were used to analyze other associated eight dental anomalies, including agenesis of other teeth, dens invaginatus, dens evaginatus, peg shaped MLI, taurodontism, pulp stone, root dilaceration and maxillary canine impaction. Results: Out of the 1964 patients examined, 90 were found to have agenesis of MLI, representing a prevalence of 4.6%. The most commonly found associated-anomalies were agenesis of other teeth (23.3%), peg-shaped MLIs (15.6%), taurodontism (42.2%), and dilacerated teeth (18.9%). Conclusion: Permanent tooth agenesis, taurodontism, peg-shaped maxillary lateral incisor, and root dilacerations are frequently associated with maxillary lateral incisor agenesis. PMID:28125145

  4. Dental anomalies in an orthodontic patient population with maxillary lateral incisor agenesis

    Directory of Open Access Journals (Sweden)

    Mehmet Citak

    Full Text Available ABSTRACT Introduction: The purpose of this study was to evaluate the prevalence of dental anomalies in a subpopulation of orthodontic patients with agenesis of maxillary lateral incisors (MLI. Methods: The material of the present study included the records of the 1964 orthodontic patients. Panoramic radiographs and dental casts were used to analyze other associated eight dental anomalies, including agenesis of other teeth, dens invaginatus, dens evaginatus, peg shaped MLI, taurodontism, pulp stone, root dilaceration and maxillary canine impaction. Results: Out of the 1964 patients examined, 90 were found to have agenesis of MLI, representing a prevalence of 4.6%. The most commonly found associated-anomalies were agenesis of other teeth (23.3%, peg-shaped MLIs (15.6%, taurodontism (42.2%, and dilacerated teeth (18.9%. Conclusion: Permanent tooth agenesis, taurodontism, peg-shaped maxillary lateral incisor, and root dilacerations are frequently associated with maxillary lateral incisor agenesis.

  5. Bilateral Duane syndrome and bilateral aniridia.

    Science.gov (United States)

    Khan, Arif O; Aldahmesh, Mohammad

    2006-06-01

    Duane retraction syndrome has been reported in association with structural abnormalities of the eye, including epibulbar dermoid, keratoconus, iris dysplasia, heterochromia iridis, persistent fetal vasculature, cataract, choroidal coloboma, microphthalmia, and optic nerve dysplasia. A novel association, that of bilateral Duane syndrome with bilateral aniridia, is the subject of this report.

  6. Hind brain agenesis a rare imaging findings in cerebro cerebellar lissencephalic syndrome.

    Science.gov (United States)

    Mundaganur, Praveen M; Solwalkar, Pradeep; Nimbal, Vishal

    2014-01-01

    A case report of cerebro cerebellar lissencephaly shows complete agenesis of cerebellum and brainstem which is rare imaging finding of group lissencephaly (type I lissencephaly). Though agenesis of cerebellum and brainstem were included in literature, in most of the cases we saw a hypoplasia or atrophy of cerebellum in lissencephaly syndrome. The CT scan findings of this patient shows features of lissencephaly with complete agenesis of brain stem and cerebellum associated with multiple congenital abnormalities.

  7. Bilateral orbital cavernous haemangiomas.

    OpenAIRE

    Fries, P D; Char, D. H.

    1988-01-01

    Simultaneous bilateral orbital lesions are rare. The differential diagnosis includes orbital pseudotumour, metastasis, leukaemia, lymphoma, Wegener's granulomatosis, and neurofibromatosis. We report what we believe to be the first case of bilateral orbital cavernous haemangiomas.

  8. 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.

  9. 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.

  10. Bilateral otogenic cerebellar abscesses.

    Directory of Open Access Journals (Sweden)

    Nadkarni T

    1993-01-01

    Full Text Available An unusual presentation of bilateral otogenic cerebellar abscesses observed in two of our patients is reported. Both gave a history of otorrhoea, fever, headache, vomiting and had bilateral cerebellar signs and conductive hearing loss. The abscesses were detected on computerised tomography. X-rays revealed bilateral mastoiditis. The therapy followed was excision of abscesses, mastoidectomy and antibiotic therapy.

  11. The role of the anterior commissure in callosal agenesis.

    Science.gov (United States)

    Barr, Melodie S; Corballis, Michael C

    2002-10-01

    Two individuals with callosal agenesis (J.P. and M.M.) and 10 neurologically normal participants were tested on tasks requiring interhemispheric visual integration. M.M., whose anterior commissure was within normal limits, was much worse at matching colors and letters between visual fields than within visual fields, whereas J.P., whose anterior commissure was greatly enlarged, showed no evidence of interhemispheric disconnection. This suggests that in some cases of callosal agenesis, probably a minority, an enlarged anterior commissure may compensate for the lack of the corpus callosum. Neither acallosal participant showed interhemispheric disconnection on tasks requiring integration of location and orientation, however, suggesting that the anterior commissure plays no role in such tasks. These tasks may depend on subcortical commissures, such as the intertectal commissure.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. Angle Class I malocclusion and agenesis of lateral incisors

    OpenAIRE

    2011-01-01

    p. 137-147 Orthodontic planning for patients with agenesis of lateral incisors should include extremely relevant esthetic and functional considerations so that a satisfactory clinical result is achieved. Both space closure and space opening or maintenance have advantages and disadvantages that should be evaluated according to the patient's individual characteristics. Some of the important factors that affect planning are the skeletal pattern, the type of malocclusion and the color and shap...

  14. Computed tomography of partial unilateral agenesis of the pectoralis muscles.

    Science.gov (United States)

    Demos, T C; Johnson, C; Love, L; Posniak, H

    1985-01-01

    The most common congenitally absent muscles are the pectoralis major and minor. Absence is usually incomplete. This anomaly is often one component of a syndrome associated with other hand (Poland syndrome) and thoracic anomalies. Computed tomography can identify partial absence of the pectoralis muscle and exactly define the altered anatomy. A patient with myasthenia gravis and isolated partial agenesis of the pectoralis muscle is presented.

  15. Aphallia (Penile agenesis: A preliminary report of three cases

    Directory of Open Access Journals (Sweden)

    Prashant Sadashiv Patil

    2017-01-01

    Full Text Available Aphallia (penile agenesis is an extremely rare abnormality with the reported incidence of 1 in 30 million births. The cause of this anomaly is associated with no genital tubercle formation or its development impairment. The majority of patients have 46XY Karyotype. The scrotum, testes and testicular function are usually normal. We report the preliminary experience with 3 cases of aphallia in different age groups along with a review of the literature.

  16. Renal Agenesis with Full Length Ipsilateral Refluxing Ureter

    Directory of Open Access Journals (Sweden)

    DilipKumar Pal

    2016-04-01

    Full Text Available Unilateral renal agenesis with vesicoureteral reflux in the ipsilateral full length ureter is a rare phenomenon. Herein we report a case of 10-year old boy who presented with recurrent urinary tract infections. No renal tissue was identified on left side in various imaging studies. Micturating cystourethrogram (MCUG showed left sided refluxing and blind ending ureter. Left ureterectomy was done because of recurrent UTI in the refluxing system.

  17. 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.

  18. Agenesis of Permanent Mandibular Central Incisors: A Concordant Condition in Siblings

    Science.gov (United States)

    Namineni, Srinivas; Tupalli, Abhinaya Reddy; Challa, Santhosh Kumar

    2016-01-01

    ABSTRACT Concordance is an identical expression of phenotype in two related individuals. Concordance expression of hypodontia is an uncommon condition where associated individuals are affected with exactly similar kind and number of missing teeth. There is very limited documentation of this condition either in twins or in siblings, and literature shows paucity of data with regard to this anomaly. To the best of our knowledge, there is only one such case reported in the literature, which has actually showed similar missing lower central incisors in siblings. This report presents a case of two girl siblings aged 11 and 13 years with congenital bilateral missing of permanent mandibular central incisors, which is an absolute concordant condition. Apart from discussing etiology, clinical implications and management, this article highlights the significance of concordant and discordant condition of hypodontia and expression of this condition in twins and siblings. How to cite this article: Kagitha PK, Namineni S, Tupalli AR, Challa SK. Agenesis of Permanent Mandibular Central Incisors: A Concordant Condition in Siblings. Int J Clin Pediatr Dent 2016;9(1):74-77. PMID:27274160

  19. Pulmonary edema

    Science.gov (United States)

    Lung 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, ...

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

    OpenAIRE

    Mónica Fernández del Castillo Ascanio; Sonia Pascual Pérez; Carlos Gálvez García

    2012-01-01

    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 ...

  1. 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)

  2. Bilateral ekstrauterin graviditet

    DEFF Research Database (Denmark)

    Kirkegaard, Ida; Kruse, Christina

    2009-01-01

    Bilateral tubal pregnancies are extremely rare and they are usually found after assisted reproductive techniques have been applied. A rare case of bilateral tubal pregnancy after natural conception, occurring in a woman without any predisposing factors for ectopic pregnancy, is presented....... The condition was diagnosed during laparoscopic surgery, and she was optimally treated with conservative tubal surgery. A short review of the literature is provided and discussed along with the clinical features, diagnostic difficulties and treatment options of bilateral tubal pregnancy. Udgivelsesdato: 2009...

  3. 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.

  4. Pulmonary toxocariasis: a case report and literature review.

    Science.gov (United States)

    Ranasuriya, G; Mian, A; Boujaoude, Z; Tsigrelis, C

    2014-06-01

    Toxocariasis is a parasitic disease caused by Toxocara canis or T. cati. We report a patient with toxocariasis who presented with dyspnea, high-grade eosinophilia, and bilateral pulmonary nodules. To further characterize the pulmonary manifestations of toxocariasis, we have reviewed 11 previously published pulmonary toxocariasis cases. The most common pulmonary symptoms in our review were cough and dyspnea, and the most common finding on chest imaging was bilateral pulmonary nodules. Risk factors for Toxocara infection primarily included exposure to dogs. Most patients received albendazole and responded well. A high index of suspicion is needed to diagnose this otherwise preventable parasitic disease.

  5. Agenesis of corpus callosum and emotional information processing in schizophrenia

    Directory of Open Access Journals (Sweden)

    Emmanuel eStip

    2012-02-01

    Full Text Available Corpus callosum (CC is essential in providing the integration of information related to perception and action within a subcortico-cortical network, thus supporting the generation of a unified experience about and reaction to changes in the environment. Its role in schizophrenia is yet to be fully elucidated, but there is accumulating evidence that there could be differences between patients and healthy controls regarding the morphology and function of CC, especially when individuals face emotionally laden information. Here, we report a case study of a patient with partial agenesis of corpus callosum (agCC patient with agenesis of the anterior aspect, above the genu and we provide a direct comparison with a group of patients with no apparent callosal damage (CC group regarding the brain activity during the processing of emotionally-laden information. We found that although the visual cortex activation in response to visual stimuli regardless of their emotional content was comparable in agCC patient and CC group both in terms of localization and intensity of activation, we observed a very large, non-specific and non-lateralized cerebral activation in the agCC patient, in contrast with the CC group, which showed a more lateralized and spatially localized activation, when the emotional content of the stimuli was considered. Further analysis of brain activity in the regions obtained in the CC group revealed that the agCC patient actually had an opposite activation pattern relative to most participants with no CC agenesis, indicating a dysfunctional response to these kind of stimuli, consistent with the clinical presentation of this particular patient. Our results seem to give support to the disconnection hypothesis which posits that the core symptoms of schizophrenia are related to aberrant connectivity between distinct brain areas, especially when faced with emotional stimuli, a fact consistent with the clinical tableau of this particular patient.

  6. 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.

  7. Rubinstein-Taybi syndrome with agenesis of corpus callosum.

    Science.gov (United States)

    Mishra, Shubhankar; Agarwalla, Sunil Kumar; Potpalle, Dnyaneshwar Ramesh; Dash, Nishant Nilotpal

    2015-01-01

    Rubinstein-Taybi syndrome (RSTS) is a rare genetic disorder with characteristic morphological anomaly. Our patient was a 4.5-year-old girl came with features like broad thumbs, downward slanting palpebral fissures and mental retardation. Systemic abnormalities such as repeated infection, seizure with developmental delay were also associated with it. She was having head banging behavior abnormal slurring speech, incoordination while transferring things from one hand to other. Galaxy of clinical pictures and magnetic resonance imaging report helped to clinch the diagnosis as a case of "RSTS with corpus callosal agenesis" which to the best of our knowledge has never been reported in past from India.

  8. Rubinstein-Taybi syndrome with agenesis of corpus callosum

    Directory of Open Access Journals (Sweden)

    Shubhankar Mishra

    2015-01-01

    Full Text Available Rubinstein-Taybi syndrome (RSTS is a rare genetic disorder with characteristic morphological anomaly. Our patient was a 4.5-year-old girl came with features like broad thumbs, downward slanting palpebral fissures and mental retardation. Systemic abnormalities such as repeated infection, seizure with developmental delay were also associated with it. She was having head banging behavior abnormal slurring speech, incoordination while transferring things from one hand to other. Galaxy of clinical pictures and magnetic resonance imaging report helped to clinch the diagnosis as a case of "RSTS with corpus callosal agenesis" which to the best of our knowledge has never been reported in past from India.

  9. [Hemorrhagic bilateral renal angiomyolipoma].

    Science.gov (United States)

    Benjelloun, Mohamed; Rabii, Redouane; Mezzour, Mohamed Hicham; Joual, Abdenbi; Bennani, Saâd; el Mrini, Mohamed

    2003-09-01

    Renal angiomyolipoma is a rare benign tumour, often associated with congenital diseases especially de Bourneville's tuberous sclerosis. Bilateral angiomyolipoma is exceptional. The authors report a case of bilateral renal angiomyolipoma in a 33-year-old patient presenting with haemorrhagic shock. In the light of this case and a review of the literature, the authors discuss the diagnostic and therapeutic aspects of this disease.

  10. Bilateral microform cleft lip

    OpenAIRE

    Pace, David; Attard Montalto, Simon; Grech, Victor E.

    2006-01-01

    Microform cleft lip (MCL), also called congenital healed cleft lip or cleft lip "frustré", is a rare congenital anomaly. MCL has been described as having the characteristic appearance of a typical cleft lip which has been corrected in utero. We present a girl with bilateral microform cleft lip associated with a preauricular sinus and bilateral camptodactyly.

  11. Bilateral assymetric epidural hematoma

    Directory of Open Access Journals (Sweden)

    Edmundo Luis Rodrigues Pereira

    2015-01-01

    Full Text Available Background: Acute bilateral extradural hematoma is a rare presentation of head trauma injury. In sporadic cases, they represent 0.5-10% of all extradural hematomas. However, higher mortality rates have been reported in previous series. Case Description: The authors described the case of a 28-year-old male presenting head injury, comatose, Glasgow Coma Scale of 6, anisocoric pupils without puppilary light reflex. Computed tomography showed asymmetric bilateral epidural hematomas, effacement of the lateral ventricles and sulci, midline shift and a bilateral skull fracture reaching the vertex. Surgical evacuation was performed with simultaneous hematoma drainage. Patient was discharged on the 29 th postoperative day with no neurological deficit. Conclusion: The correct approach on bilateral epidural hematomas depends on the volume, moment of diagnosis, and neurological deficit level. Simultaneous drainage of bilateral hematomas has been demonstrated to be an effective technique for it, which soon decreases the intracranial pressure and promotes an efficient resolution to the neurological damage.

  12. 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.

  13. Sound lateralization in subjects with callosotomy, callosal agenesis, or hemispherectomy.

    Science.gov (United States)

    Hausmann, Markus; Corballis, Michael C; Fabri, Mara; Paggi, Aldo; Lewald, Jörg

    2005-10-01

    The question of whether there is a right-hemisphere dominance in the processing of auditory spatial information in human cortex as well as the role of the corpus callosum in spatial hearing functions is still a matter of debate. Here, we approached this issue by investigating two late-callosotomized subjects and one subject with agenesis of the corpus callosum, using a task of sound lateralization with variable interaural time differences. For comparison, three subjects with left or right hemispherectomy were also tested by employing identical methods. Besides a significant reduction in their acuity, subjects with total or partial section of the corpus callosum exhibited a considerable leftward bias of sound lateralization compared to normal controls. No such bias was found in the subject with callosal agenesis, but merely a marginal reduction of general acuity. Also, one subject with complete resection of the left cerebral cortex showed virtually normal performance, whereas another subject with left hemispherectomy and one subject with right hemispherectomy exhibited severe deficits, with almost total loss of sound-lateralization ability. The results obtained in subjects with callosotomy indicate that the integrity of the corpus callosum is not indispensable for preservation of sound-lateralization ability. On the other hand, transcallosal interhemispheric transfer of auditory information obviously plays a significant role in spatial hearing functions that depend on binaural cues. Moreover, these data are compatible with the general view of a dominance of the right cortical hemisphere in auditory space perception.

  14. MR in complete dorsal pancreatic agenesis: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Shruti Thakur

    2014-01-01

    Full Text Available The morphogenesis of the pancreas is a complex process having a very low frequency of anatomic variation. The congenital anomalies are rare. Complete pancreatic and ventral pancreatic agenesis are incompatible with life. Dorsal pancreatic agenesis is exceedingly rare with less than 100 cases reported in the world literature. Patients with this anomaly may be asymptomatic or may present with abdominal pain, hyperglycemia, diabetes mellitus, and acute or chronic pancreatitis. Such anomalies are rarely reported; therefore, clinical awareness of agenesis of the dorsal pancreas as a cause of these symptoms can expand the differential diagnosis and improve patient management.

  15. 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...

  16. Bilateral Control - Operational enhancements

    OpenAIRE

    Altınışık, Ahmet; Altinisik, Ahmet

    2006-01-01

    A succinct definition of the word bilateral is having two sides [1]. In robotics the term bilateral control is used to define the specific interaction of two systems by means of position and/or force. Bilateral systems are composed of two sides named master and slave side. The aim of such an arrangement is such that position command dictated by master side is followed by a slave side, and at the same time the force sensation of the remote environment experienced by slave is transferred to the...

  17. 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.

  18. Breathing machine experience in the treatment of 21 patients with bilateral flail chest complicated with severe pulmonary contusion using%双侧连枷胸合并严重肺挫伤21例的呼吸机治疗体会

    Institute of Scientific and Technical Information of China (English)

    陈希涛; 孙筱笛; 韩立波; 丛巍; 杜江; 张林

    2013-01-01

    Objective To study the effect of mechanical ventilation in the treatment of traumatic flail chest complicated with pulmonary contusion and respiratory failure. Methods 21 patients with bilateral flail chest combined with severe pulmonary contusion and respiratory failure were treated with invasive mechanical ventilation, and then the blood gas and value of pH, PaO2 , PaCO2 , and HCO3 were compared before and after the treatment. Results The values of PH, PaO2 , PaCO2 , and HCO3 were all better improved after the treatment. Conclusion The mechanical ventilation is an effective method in the treatment of patients with flail chest complicated with severe pulmonary contusion and respiratory failure.%目的 探讨机械通气治疗创伤性连枷胸合并肺挫伤、呼吸衰竭的疗效.方法 对21例双侧创伤性连枷胸合并严重肺挫伤、呼吸衰竭未行手术内固定之患者行机械通气治疗后分析血气分析等各种参数的变化.结果 机械通气治疗后动脉血PH值、氧分压(PaO2) 、二氧化碳分压(PaCO2) 、碳酸根离子(HCO-3)、氧合指数(PaO2/FiO2)等均较前明显改善.结论 对于双侧创伤性连枷胸合并严重肺挫伤、呼吸衰竭的患者,机械通气内固定治疗行之有效.

  19. Sound localization in callosal agenesis and early callosotomy subjects: brain reorganization and/or compensatory strategies.

    Science.gov (United States)

    Lessard, Nadia; Lepore, Franco; Villemagne, Jean; Lassonde, Maryse

    2002-05-01

    In order to evaluate the callosal involvement in sound localization, the present study examined the response accuracy of acallosal and early callosotomized subjects to monaural and binaural auditory targets presented in three-dimensional space. In these subjects, bilateral localization cues, such as interaural time and level differences, are integrated at the cortical and subcortical levels without the additional support of the callosal commissure. Because acallosal and early-callosotomized subjects have developed with this reduced source of binaural activation of cortical cells, they might have perfected their ability to use monaural sound localization cues. This hypothesis was tested by assessing localization performance under both binaural and monaural listening conditions. Five subjects with callosal agenesis, one callosotomized subject operated early in life and 19 control subjects were asked to localize broad-band noise bursts (BBNBs) of fixed intensity in the horizontal plane in an anechoic chamber. BBNBs were delivered through randomly selected loudspeakers. Two conditions were tested: (i) localization of a stationary sound source; and (ii) localization of a moving sound source. Listeners had to report the apparent stimulus location by pointing to its perceived position on a graduated perimeter. The results indicated that the acallosal subjects were less accurate than controls, but only in the binaural moving sound condition. More interestingly, in the monaural testing conditions, some of the acallosal subjects and the early-callosotomized subject performed significantly better than control subjects. This suggests that, because of the absence of the corpus callosum, these subjects compensate for their reduced access to cortically determined binaural cues by making more efficient use of monaural cues.

  20. Pulmonary Thromboendarterectomy for Pulmonary Hypertension Before Considering Transplant

    Science.gov (United States)

    Kooperkamp, Hannah; Mehta, Inder; Fary, David; Bates, Michael

    2017-01-01

    Background: In cases of chronic thromboembolic pulmonary hypertension (CTEPH), referral for possible surgical intervention is important because surgery can be curative. Surgery necessitates cardiopulmonary bypass and deep circulatory arrest with pulmonary thrombectomy and bilateral endarterectomy (PTE). If surgery fails, lung transplant is the next best surgical option. Medical treatment is also an important adjunct. Case Report: A 35-year-old female presented 3 months after a pulmonary embolus was found to be completely occluding her left pulmonary artery. She was found to have pulmonary hypertension with a pulmonary artery pressure of 81/33 mmHg, with a mean pressure of 52 mmHg. The right atrial pressure was also severely elevated at 29 mmHg, and her echocardiogram revealed severe tricuspid regurgitation and severe right ventricular dysfunction. She underwent PTE and postoperatively was followed by the heart failure team. Her 6-minute walk distance improved from 396 meters at 1 month to 670 meters at 7 months, and her pulmonary artery pressure improved significantly to 55/17 mmHg with a mean pressure of 31 mmHg. The patient's right atrial pressure also improved significantly from 29 mmHg to 13 mmHg. Conclusion: CTEPH is likely underrecognized, and patients with pulmonary hypertension or a history of pulmonary embolism should be screened for CTEPH. This case illustrates the surgical treatment for CTEPH and discusses alternative and adjunctive treatments. Residual pulmonary hypertension after PTE occurs in approximately 35% of patients. Overall, 4-year mortality rates after surgery appear to be approximately 15%, and mortality rates correlate with the postoperative pulmonary vascular resistance. Recognition of chronic pulmonary thromboembolic disease as the etiology of pulmonary hypertension warrants evaluation for surgery.

  1. Staged bilateral carotid endarterectomy

    DEFF Research Database (Denmark)

    Schroeder, T; Sillesen, H; Engell, Hans Christian

    1986-01-01

    In a series of 56 staged bilateral carotid endarterectomies, new neurologic symptoms developed in 5% and 20% following the first and second procedure, respectively. All complications were transient or minor. The incidence of postendarterectomy hypertension was significantly higher following...

  2. Pulmonary embolus

    Science.gov (United States)

    ... Blood clot - lung; Embolus; Tumor embolus; Embolism - pulmonary; DVT-pulmonary embolism; Thrombosis - pulmonary embolism ... area). This type of clot is called a deep vein thrombosis (DVT) . The blood clot breaks off and travels ...

  3. Echinoderms have bilateral tendencies.

    Science.gov (United States)

    Ji, Chengcheng; Wu, Liang; Zhao, Wenchan; Wang, Sishuo; Lv, Jianhao

    2012-01-01

    Echinoderms take many forms of symmetry. Pentameral symmetry is the major form and the other forms are derived from it. However, the ancestors of echinoderms, which originated from Cambrian period, were believed to be bilaterians. Echinoderm larvae are bilateral during their early development. During embryonic development of starfish and sea urchins, the position and the developmental sequence of each arm are fixed, implying an auxological anterior/posterior axis. Starfish also possess the Hox gene cluster, which controls symmetrical development. Overall, echinoderms are thought to have a bilateral developmental mechanism and process. In this article, we focused on adult starfish behaviors to corroborate its bilateral tendency. We weighed their central disk and each arm to measure the position of the center of gravity. We then studied their turning-over behavior, crawling behavior and fleeing behavior statistically to obtain the center of frequency of each behavior. By joining the center of gravity and each center of frequency, we obtained three behavioral symmetric planes. These behavioral bilateral tendencies might be related to the A/P axis during the embryonic development of the starfish. It is very likely that the adult starfish is, to some extent, bilaterian because it displays some bilateral propensity and has a definite behavioral symmetric plane. The remainder of bilateral symmetry may have benefited echinoderms during their evolution from the Cambrian period to the present.

  4. Anesthetic dilemma in planning bilateral cataract surgery for an infant associated with congenital cardiac anomaly

    Directory of Open Access Journals (Sweden)

    Devalina Goswami

    2015-01-01

    Full Text Available In a patient with tetralogy of Fallot (TOF and pulmonary atresia, treating the cardiac problem or the associated congenital illness is always a challenge. We describe the challenges and successful initial management of bilateral cataract to prevent visual loss in an infant with TOF with pulmonary atresia.

  5. Anesthetic dilemma in planning bilateral cataract surgery for an infant associated with congenital cardiac anomaly.

    Science.gov (United States)

    Goswami, Devalina; Seetharamaiah, Shwetha; Kedia, Sraban Kumar; Nayak, Bhagabat Kumar; Akshat, Shiv

    2015-06-01

    In a patient with tetralogy of Fallot (TOF) and pulmonary atresia, treating the cardiac problem or the associated congenital illness is always a challenge. We describe the challenges and successful initial management of bilateral cataract to prevent visual loss in an infant with TOF with pulmonary atresia.

  6. 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.

  7. Neonatal diabetes mellitus due to pancreatic agenesis and pervasive developmental disorder

    Directory of Open Access Journals (Sweden)

    Giannattasio Alessandro

    2009-07-01

    Full Text Available Abstract Recent studies suggested a link between type 1 diabetes mellitus and pervasive developmental disorder. Moreover, permanent neonatal diabetes mellitus due to pancreatic agenesis can be associated with neurological deficit involving cerebellar functions, but no association with pervasive developmental disorder has been described so far. Clinical and neuropsychological evaluation of a child with pancreatic agenesis, mental retardation and pervasive developmental disorder is reported.

  8. Congenital agenesis of internal carotid artery with ipsilateral Horner presenting as focal neurological symptoms

    OpenAIRE

    Wassim Farhat; Rechdi Ahdab; Hassan Hosseini

    2011-01-01

    Wassim Farhat, Rechdi Ahdab, Hassan HosseiniService de Neurologie, Hôpital Henri Mondor, APHP, Faculté de Médecine, Université Paris XII, Créteil, FranceAbstract: Internal carotid artery (ICA) agenesis is a rare developmental anomaly and is most frequently asymptomatic, but it may also present as cerebrovascular accidents. The association with Horner’s syndrome is exceptional. We present three cases of agenesis of ICA associated ...

  9. 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

  10. Delayed dental maturity in dentitions with agenesis of mandibular second premolars

    DEFF Research Database (Denmark)

    Daugaard, S; Christensen, I J; Kjaer, I

    2010-01-01

    To evaluate dental maturity in the mandibular canine/premolar and molar innervation fields in children with agenesis of the 2nd mandibular premolar and to associate these findings with normal control material.......To evaluate dental maturity in the mandibular canine/premolar and molar innervation fields in children with agenesis of the 2nd mandibular premolar and to associate these findings with normal control material....

  11. Congenital Unilateral Agenesis of the Parotid Gland: A Case Report and Review of the Literature

    Science.gov (United States)

    Teymoortash, Afshin

    2016-01-01

    Congenital unilateral agenesis of the parotid gland is a rare condition with only few cases reported in the literature. A review of 21 cases in the available literature is presented in this article. We report on a further case of a 34-year-old woman with agenesis of the left parotid gland and lipoma of the right cheek. Clinicopathological characteristics of described cases in the literature were discussed. PMID:27895943

  12. Language lateralization in a patient with temporal lobe epilepsy and callosal agenesis.

    Science.gov (United States)

    Alsaadi, Taoufik; Shahrour, Tarek M

    2015-01-01

    The corpus callosum has been proposed as a mechanism of interhemispheric inhibition that allows language dominance to develop [1]. Callosal agenesis or dysgenesis provides a test of this hypothesis, as patients lacking a normal corpus callosum should also lack normal language lateralization [2]. We report pre- and postoperative functional magnetic resonance imaging (fMRI) and neuropsychological testing in a patient with partial callosal agenesis who underwent a right temporal lobectomy for medically refractory seizures.

  13. Language lateralization in a patient with temporal lobe epilepsy and callosal agenesis

    Directory of Open Access Journals (Sweden)

    Taoufik Alsaadi

    2015-01-01

    Full Text Available The corpus callosum has been proposed as a mechanism of interhemispheric inhibition that allows language dominance to develop [1]. Callosal agenesis or dysgenesis provides a test of this hypothesis, as patients lacking a normal corpus callosum should also lack normal language lateralization [2]. We report pre- and postoperative functional magnetic resonance imaging (fMRI and neuropsychological testing in a patient with partial callosal agenesis who underwent a right temporal lobectomy for medically refractory seizures.

  14. Language lateralization in a patient with temporal lobe epilepsy and callosal agenesis

    OpenAIRE

    Taoufik Alsaadi; Shahrour, Tarek M.

    2014-01-01

    The corpus callosum has been proposed as a mechanism of interhemispheric inhibition that allows language dominance to develop [1]. Callosal agenesis or dysgenesis provides a test of this hypothesis, as patients lacking a normal corpus callosum should also lack normal language lateralization [2]. We report pre- and postoperative functional magnetic resonance imaging (fMRI) and neuropsychological testing in a patient with partial callosal agenesis who underwent a right temporal lobectomy for me...

  15. A Turkish newborn infant with cerebellar agenesis/neonatal diabetes mellitus and PTF1A mutation.

    Science.gov (United States)

    Tutak, E; Satar, M; Yapicioğlu, H; Altintaş, A; Narli, N; Hergüner, O; Bayram, Y

    2009-01-01

    Classical neonatal diabetes mellitus is defined as hyperglycemia that occurs within the first month of life in term infants. It can be either permanent or transient. Cerebellar agenesis and permanent neonatal diabetes has been previously reported as a new autosomal recessive disorder. Pancreas Transcription Factor 1 Alpha (PTF1A) mutations have been related with this constellation of abnormalities. Here we report a new case of cerebellar agenesis and neonatal diabetes mellitus whose parents are PTF1A mutation carriers.

  16. 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.

  17. 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.

  18. 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.

  19. 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

  20. Pulmonary Artery Aneurysm Thrombosis with Combined Pulmonary Fibrosis and Emphysema: A Case Report

    OpenAIRE

    Agrawal, Mitali Bharat; Awad, Nilkant Tukaram

    2016-01-01

    We report a rare case of Pulmonary Artery Aneurysm (PAA) thrombosis with Combined Pulmonary Fibrosis and Emphysema (CPFE) with pulmonary hypertension. A 75-year-old male presented with haemoptysis, dyspnoea, clubbing and bilateral fine end inspiratory rales on examination. He was diagnosed to have PAA thrombosis with CPFE on the basis of computed tomographical angiography and high resolution computed tomography. He was then managed conservatively with pirfenidone for the interstitial lung dis...

  1. Emotional Intelligence in Agenesis of the Corpus Callosum.

    Science.gov (United States)

    Anderson, Luke B; Paul, Lynn K; Brown, Warren S

    2017-01-23

    People with agenesis of the corpus callosum (AgCC) with normal general intelligence have deficits in complex cognitive processing, as well as in social cognition. It is uncertain the extent to which impoverished processing of emotions may contribute to social processing deficiencies. We used the Mayer-Salovey-Caruso Emotional Intelligence Test to clarify the nature of emotional intelligence in 16 adults with AgCC. As hypothesized, persons with AgCC exhibited greater disparities from norms on tests involving more socially complex aspects of emotions. The AgCC group did not differ from norms on the Experiential subscale, but they were significantly below norms on the Strategic subscale. These findings suggest that the corpus callosum is not essential for experiencing and thinking about basic emotions in a "normal" way, but is necessary for more complex processes involving emotions in the context of social interactions.

  2. Bilateral segmental regression of the carotid and vertebral arteries with rete compensation in a Western patient

    Energy Technology Data Exchange (ETDEWEB)

    Mahadevan, J.; Batista, L.; Alvarez, H.; Lasjaunias, P. [Service de Neuroradiologie Diagnostique et Therapeutique, Hopital de Bicetre, 94275 Le Kremlin Bicetre, 94275, Le Kremlin Bicetre (France); Bravo-Castro, E. [Instituto De Neurocirugia Asenjo, c/Jose Manuel Infante, Santiago (Chile)

    2004-06-01

    We report a case of symptomatic symmetrical, bilateral absence of the cavernous internal carotid (ICA) and transdural vertebral artery (VA) segments with formation of a rete mirabile. There have been similar reports in Asian patients; ours is the first in the Western population. A 29- year-old woman presented with recurrent temporary blindness and an episode of minor subarachnoid haemorrhage. Angiography demonstrated absence of the C5 and C6 segments of the ICA and the C1/2 segments of the VA bilaterally, with typical carotid rete mirabile formation to reconstitute the distal flow. Comparative anatomy and embryology show retia in several species and point to their secondary origin. The anomalies of the ICA cannot be called agenesis but rather absence since they are likely to result from perinatal disappearance of the artery rather its failure to develop. (orig.)

  3. Bilateral parotitis as the initial presentation of childhood sarcoidosis.

    Science.gov (United States)

    Banks, Gretchen C; Kirse, Daniel J; Anthony, Evelyn; Bergman, Simon; Shetty, Avinash K

    2013-01-01

    The differential diagnosis of bilateral parotid gland enlargement in children includes infectious, inflammatory, and neoplastic disorders. We present the case of a 13-year-old male who presented with a 5-week history of bilateral parotid swelling. On exam, both parotid glands were nontender, smooth, and diffusely enlarged. He had slightly elevated inflammatory markers, but other lab results were normal. A neck CT revealed symmetric enlargement of the parotid, submandibular, and sublingual glands. A chest CT revealed scattered peripheral pulmonary nodules and bilateral hilar adenopathy. A parotid gland biopsy showed multiple noncaseating granulomas with multinucleated giant cells surrounded by lymphocytes, consistent with the diagnosis of sarcoidosis. Special stains for acid-fast and fungal organisms were negative. Using this illustrative case, we discuss the differential diagnosis of bilateral salivary gland enlargement in children and review the etiology, diagnosis, clinical manifestations, and treatment of pediatric sarcoidosis.

  4. 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.

  5. 双侧肺同期手术中不同体位和单肺通气对病人呼吸力学的影响%Effects of different positions and one-lung ventilation modes on respiratory mechanics of patients during bilateral pulmonary surgery

    Institute of Scientific and Technical Information of China (English)

    马宏伟; 耿恩江; 李凤茹; 杨永斌; 丁丽景; 马辉

    2012-01-01

    Objective To investigate the effects of different positions and one-lung ventilation modes on respiratory mechanics of patients during bilateral pulmonary surgery. Methods Analysis on 142 cases who underwent bilateral pulmonary surgery from June, 2007 to December, 2010. PIP,Pplat,Raw and Cdyn measures were continuous monitored by side stream spirometry while SpO2 and PETCO2 were observed on the Patient Monitor. After dual cavity bronchus vessel was fixed and different ventilation modes were applied, blood gas analyses were made after 10 min with patients in lateral positions. Results When patient was changed to a lateral position under double-lung ventilation and when one-lung ventilation mode was applied, PIP、PplatRaw increased while Cdyn decreased, and PH decreased while PETCO2 and PaCO2 increased with SpO2 and PaO2 decreasing( P < 0. 05 ). When a different ventilation mode was applied under double-lung ventilation, PIP, Pplat and Raw decreased while Cdyn increased, PH decreased while PaO2 、SpO2 、PaCO2、PETCO2 increased ( P <0. 05 );when 38 cases were applied with a different ventilation mode under one-lung ventilation, PIP, Pplat and Raw decreased significantly while Cdyn increased, PH,PaO2 and SpO2 increased while PaCO2 and PETCO2 decreased( P <0. 05 ). Conclusion Different positions and ventilation modes have great effect on respiratory mechanics of patients during bilateral pulmonary surgery, and appropriate ventilation modes can improve respiratory mechanics and prevent hypoxemia, hypercarbia and barotraumas to protect lungs.%目的 观察双侧肺同期手术中体位改变和单肺通气时对病人呼吸力学的影响.方法 选取我院2007年6月至2010年12月双侧肺同期手术病人142例,经气道旁路采用旁气流通气连续监测病人PIP、Pplat、Raw、Cdyn等呼吸力学指标,监测SpO2、PETCO2指标,分别在双腔支气管导管定位后,平卧改侧卧,单肺通气及改换通气方式后10 min抽取动

  6. Agenesis of the Dorsal Pancreas with Chronic Calcific Pancreatitis. Case Report, Review of the Literature and Genetic Basis

    Directory of Open Access Journals (Sweden)

    Vallath Balakrishnan

    2006-11-01

    Full Text Available Context Agenesis of the dorsal pancreas is a rare developmental anomaly. This anomaly may be complicated by recurrent acute and chronic pancreatitis.Case report We report the case of a 28-year-old female with agenesis of the dorsal pancreas and chronic calcific pancreatitis. The diagnosis of agenesis of the dorsal pancreas is discussed and the genetic changes leading to it are reviewed. The possibility of the patient having tropical pancreatitis is mentioned.Conclusions This is probably the first report of chronic calcific pancreatitis complicating agenesis of the dorsal pancreas

  7. Bilaterally Incarcerated Morgagni Hernia

    Directory of Open Access Journals (Sweden)

    Zuhal Demirhan Yananli

    2013-06-01

    Full Text Available Morgagni hernia is a rare congenital diaphragmatic hernia. It is seen rarely bilaterally. Patients are usually asymptomatic. Therefore, diagnosis may be delayed until adulthood. Significant morbidity can occur in case complications arise and diagnosis is delayed. The patient, a 74 year-old female, presented in this article, was admitted to the emergency department with abdominal pain, vomiting, and shortness of breath. The plain abdominal radiograph of the patient revealed bowel obstruction and suspicious appearence in favor of the diaphragmatic hernia on both sides of the sternum. Computed tomography revealed bilaterally incarcerated Morgagni hernia with strangulated omentum in the right side of the sternum and a part of colon in the left side of sternum. Incarcerated organs were withdrawn to peritoneal cavity and defects of hernia were sutured primarily on laparatomy. Because bilateral incarcerated Morgagni hernia can be seen rarely, this case was reported.

  8. 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

  9. 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.

  10. Coincidence of congenital left-sided diaphragmatic hernia and ductus venosus agenesis: Relation between altered hemodynamic flow and lung-to-head-ratio?

    Directory of Open Access Journals (Sweden)

    T. Klein

    2015-06-01

    Full Text Available Left-sided diaphragmatic hernia (CDH as well as ductus venosus agenesis (ADV are rare complex congenital malformations. We present a case of coincidence of these malformations and an abnormally high lung-head-ratio (LHR. The left-sided liver-up CDH and the ADV were diagnosed in prenatal ultrasound examination. In CDH cases lung volume is decreased due to the herniation of abdominal organs into the thorax. With 1.4 the LHR of our patient exceeded the normal ratio in liver-up CDH cases considerably. One explanation for this unusually high LHR might be an altered blood flow due to the coinciding ADV. In ADV cases less blood bypasses the lung through the foramen ovale. Consecutively pulmonary circulation is improved which may constitute as an advantage in CDH cases. Diagnosis, prognostic factors, physiology, and therapy strategy are discussed.

  11. A rare cause of anuria: Bilateral synchronous isolated mid-ureteric tubercular lesions

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    Anuj D Dangi

    2015-01-01

    Full Text Available A young female presenting with right flank pain, fever, raised creatinine and bilateral hydronephrosis was treated with antibiotics elsewhere, with presumptive diagnosis of bilateral pyelonephritis. She had partial relief in symptoms and her creatinine level showed an improvement. Three months later during evaluation at our center she had anuria, hypertensive crisis and pulmonary edema which were managed with emergency bilateral percutaneous nephrostomies. Cross-sectional imaging and ureteroscopy suggested bilateral synchronous intramural mid-ureteric lesions as underlying pathology. Histopathology of the ureteric segments during laparotomy revealed caseating granulomas suggestive of tuberculosis. This clinical presentation has not been previously described in urinary tuberculosis.

  12. Bilateral Naevus Of OTA

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    Ahmad Qazi Masood

    2001-01-01

    Full Text Available Naevus of Ota is a type of dermal melanocytic naevus characterized by extensive blue patch of dermal melanocytic pigmentation of the sclera and the skin adjacent to the eye. The condition is usually unilateral. Here we report a patient with bilateral naevus of Ota in view of the rarity of this condition.

  13. Bilateral adrenal histoplasmosis in an immunocompetent man Histoplasmose adrenal bilateral em um homem imunocompetente

    Directory of Open Access Journals (Sweden)

    Carlos Frederico Lopes Benevides

    2007-04-01

    Full Text Available Histoplasmosis is a fungal disease that is endemic in Brazil. It may present as chronic pulmonary infection or in disseminated form. Disseminated histoplasmosis frequently affects the adrenal gland; however, unilateral involvement in immunosuppressed patients is the usual presentation. We report a case of an elderly immunocompetent male with history of weight loss, fever and bilateral adrenal mass who was successfully treated with itraconazole.Histoplasmose é uma doença fúngica endêmica no Brasil que pode se apresentar como infecção pulmonar crônica ou na forma disseminada. A histoplasmose disseminada freqüentemente acomete a glândula adrenal; entretanto, ocorre mais em pacientes imunossuprimidos e de forma unilateral. Relatamos um caso de um homem idoso imunocompetente com história de perda de peso, febre e massa adrenal bilateral que foi tratada com itraconazol, com sucesso.

  14. Congenital agenesis of internal carotid artery with ipsilateral Horner presenting as focal neurological symptoms

    Directory of Open Access Journals (Sweden)

    Hosseini H

    2011-01-01

    Full Text Available Wassim Farhat, Rechdi Ahdab, Hassan HosseiniService de Neurologie, Hôpital Henri Mondor, APHP, Faculté de Médecine, Université Paris XII, Créteil, FranceAbstract: Internal carotid artery (ICA agenesis is a rare developmental anomaly and is most frequently asymptomatic, but it may also present as cerebrovascular accidents. The association with Horner’s syndrome is exceptional. We present three cases of agenesis of ICA associated with Horner’s syndrome and hypochromia iridum presenting as focal neurological symptoms. A system of collaterals develops as a consequence of agenesis of the ICA, making the majority of cases asymptomatic. Three types of collateral circulations have been described. These collaterals increase the risk of aneurysm formation and the occurrence of life-threatening subarachnoid hemorrhages. The association of congenital Horner’s syndrome and hypochromia iridum without anhidrosis is highly suggestive of sympathetic pathway injury early in life. Such signs should prompt further diagnostic evaluation to demonstrate the presence of the agenesis of the carotid canal. Early diagnosis is essential to rule out potentially life-threatening associated vascular anomalies.Keywords: carotid artery agenesis, Horner’s syndrome, heterochromia iridum

  15. Systematic review of sexual function and satisfaction following the management of vaginal agenesis.

    Science.gov (United States)

    McQuillan, Sarah K; Grover, Sonia R

    2014-10-01

    Historically, sexual satisfaction following the management of vaginal agenesis was assessed subjectively. Standardized sexual function questionnaires are being used more frequently as instruments to accurately and more objectively assess the subjective nature of sexual outcomes as part of a more holistic approach to the care of women with vaginal agenesis. Articles concerning the management of vaginal agenesis were systematically reviewed, with specific focus on those that discussed functional outcomes, sexual satisfaction and psychosomatic outcomes, and in particular attempted to measure these outcomes. A total of 6,691 articles on vaginal agenesis were identified, with 106 of these reporting sexual satisfaction and psychosomatic outcomes. Only 1 randomized control trial (RCT) was identified, the remaining articles being made up of case series or case reports. Only 17 articles used standardized objective assessment of sexual satisfaction. While the bowel technique had the longest vaginal length at 12.87 cm, it had the most number of complaints of dyspareunia (4.8%), stenosis (10.5%) and the lowest average subjective sexual satisfaction. The Davydov method used standardized sexual function assessments most frequently. This technique had a higher average score than both the bowel vaginoplasty technique in the only RCT and the Vecchietti method in a prospective assessment. Overall, the management of vaginal agenesis requires a multidisciplinary approach to fully support these patients from initial diagnosis, through management decision-making and long-term follow-up, through transition to adulthood.

  16. 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.

  17. 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]...

  18. 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 ...

  19. Pulmonary Rehabilitation

    Science.gov (United States)

    ... shortness of breath and increase your ability to exercise. You may have heard that pulmonary rehabilitation is only for people with COPD (chronic obstructive pulmonary disease). We now know that ...

  20. Bilateral chronic subdural hematoma

    DEFF Research Database (Denmark)

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

    2017-01-01

    OBJECTIVE Bilateral chronic subdural hematoma (bCSDH) is a common neurosurgical condition frequently associated with the need for retreatment. The reason for the high rate of retreatment has not been thoroughly investigated. Thus, the authors focused on determining which independent predictors...... are associated with the retreatment of bCSDH with a focus on surgical laterality. METHODS In a national database of CSDHs (Danish Chronic Subdural Hematoma Study) the authors retrospectively identified all bCSDHs treated in the 4 Danish neurosurgical departments over the 3-year period from 2010 to 2012....... Univariate and multivariate analyses were performed to determine the relationship between retreatment of bCSDH and clinical, radiological, and surgical variables. RESULTS Two hundred ninety-one patients with bCSDH were identified, and 264 of them underwent unilateral (136 patients) or bilateral (128 patients...

  1. Bilateral tibial hemimelia I.

    Science.gov (United States)

    Suganthy, J; Rassau, Marina; Koshi, Rachel; Battacharjee, Suranjan

    2007-05-01

    Congenital absence of tibia is a rare anomaly. We report a case of bilateral tibial hemimelia born to phenotypically normal parents. The two amputated legs with tibial dysplasia obtained from a 3-year-old boy were studied by radiography and anatomical dissection. The radiological evaluation revealed a normal hip joint. The lower end of femur was normal without any bifurcation, shortening or bowing. Fibula was present on both legs and there was no sign of bowing or doubling. Both right and left tibiae were absent. In addition, on the right side, five tarsal bones, two metatarsals and the corresponding digital rays were absent. On the left side, three tarsal bones were absent. Dissection of the amputated segments showed the presence of extensor digitorum longus, peroneus tertius, peroneus longus and brevis, gastrocnemius, and soleus. Following bilateral knee disarticulation the patient was fitted with prosthesis and is doing well.

  2. Agenesis of the gallbladder: A dangerously misdiagnosed malformation

    Institute of Scientific and Technical Information of China (English)

    Nicolas Peloponissios; Michel Gillet; René Cavin; Nermin Halkic

    2005-01-01

    Isolated agenesis of the gallbladder is a rare anomaly,often asymptomatic. However, one patient out of four presented with right upper abdominal pain, nausea, and fatty food intolerance. The condition is frequently mistaken with an excluded or sclero-atrophic gallbladder, regardless of the imaging modality used. Consequently, AG often leads to unnecessary and potentially dangerous laparoscopic surgery as described in a few case reports over the last10 years. The aim of this study is to clarify the diagnostic and therapeutic approach of this unusual pathology. Two cases seen in our institutions were retrospectively reviewed, together with a review of the American and European literature. During laparoscopy, the absence of normal anatomical structures and the impossibility of pulling on the gallbladder to expose and dissect the triangle of Callot increases the risk of iatrogenic injury to biliary or portal structures. Depending on the experiment of the surgeon in laparoscopic procedure, this has to be taken into account to decide a conversion to laparotomy.A high index of suspicion is necessary when interpreting the radiological images. In case of doubt, a MRJ-cholangiography is mandatory. Because of possible inherited transmission,relatives with a history of biliary symptoms should be investigated.

  3. [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.

  4. Bilateral lunate intraosseous ganglia

    Energy Technology Data Exchange (ETDEWEB)

    Pablos, J.M. [Department of Radiology, Hospital San Juan de Dios, Seville (Spain); Valdes, J.C. [Department of Radiology, Cemedi, Seville (Spain); Gavilan, F. [Department of Pathology, Hospital Universitario Virgen del Rocio, Seville (Spain)

    1998-12-01

    An intraosseous ganglion is a relatively uncommon, benign, cyst-like lesion that occurs in young and middle-aged adults. Most commonly seen adjacent to the hip, ankle, knee, or wrist, they are histologically identical to their soft tissue counterparts. A review of the literature revealed only two previously reported examples of bilateral symmetrical ganglia of the lunate bones. (orig.) With 3 figs., 10 refs.

  5. Ischemic Bilateral Opercular Syndrome

    OpenAIRE

    Aysel Milanlioglu; Mehmet Nuri Aydın; Alper Gökgül; Mehmet Hamamcı; Mehmet Atilla Erkuzu; Temel Tombul

    2013-01-01

    Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum. Its clinical presentations include anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional activities of these structures are preserved. In the...

  6. 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...... compared with patients receiving other organ transplantations. It is therefore more likely that tendon ruptures are related to metabolic changes associated with kidney disease rather than with transplantation or with glucocorticoid treatment per se. Clinical symptoms of achilles tendinitis should...

  7. Bilateral Malignant Brenner Tumour

    Directory of Open Access Journals (Sweden)

    Nasser D Choudhary, S.Manzoor Kadri, Ruby Reshi, S. Besina, Mansoor A. Laharwal, Reyaz tasleem, Qurrat A. Chowdhary

    2002-10-01

    Full Text Available Bilateral malignant Brenner tumour ofovary is extremely rate. A case ofmalignant Brenner tumourinvolving both the ovaries with mctastasis to mesentery in a 48 year femalc is presented. Grosslyo'arian masses were firm with soft areas, encapsulated and having bosselated external surfaces.Cut sections showed yellowish white surface with peripheral cysts (in both tumours. Microscopyrevealed transitional cell carcinoma with squamoid differentiation at places. Metastatic deposits werefound in the mesentery. Endometrium showed cystic glandular hyperplasia.

  8. Agenesis of the dorsal pancreas and its association with pancreatic tumors.

    Science.gov (United States)

    Sakpal, Sujit Vijay; Sexcius, Lucretia; Babel, Nitin; Chamberlain, Ronald Scott

    2009-05-01

    Morphogenesis of the pancreas is a complex process; nevertheless, congenital anomalies are rare. At embryogenesis, the pancreas develops from the endoderm-lined dorsal and ventral buds of the duodenum. The ventral bud gives rise to the lower head and uncinate process of the pancreas; whereas, the dorsal bud gives rise to the upper head, isthmus, body, and tail of the pancreas. Rarely, developmental failure of the dorsal pancreatic bud at embryogenesis results in the agenesis of the dorsal pancreas--neck, body, and tail. Even rarer is the association of pancreatic tumors with agenesis of the dorsal pancreas. In addition to citing our case, we provide a comprehensive review on agenesis of the dorsal pancreas and its association with pancreatic tumors.

  9. Callosal agenesis, chorioretinal lacunae, absence of infantile spasms, and normal development: Aicardi syndrome without epilepsy?

    Science.gov (United States)

    Prats Viñas, Jose Maria; Martinez Gonzalez, María Jesús; Garcia Ribes, Ainhoa; Martinez Gonzalez, Sonia; Martinez Fernandez, Ricardo

    2005-06-01

    Aicardi syndrome is defined by the clinical triad of infantile spasms, agenesis of the corpus callosum, and pathognomonic chorioretinal lacunae. Almost all patients are females with severe cognitive and physical disabilities. All of the cases reported in the literature have had early-onset seizures. Most cases of Aicardi syndrome exhibit very slow development, even when seizures are eventually controlled, and the cases with a relatively favourable outcome are associated with low intelligence quotient levels. A relationship between chorioretinal changes or severity of the agenesis of the corpus callosum and prognosis of Aicardi syndrome has been claimed, but few data are available about the clinical features that can predict clinical outcome. We describe a case of Aicardi syndrome in a female aged 24 months. Magnetic resonance imaging showed complete agenesis of the corpus callosum and ophthalmoscopy revealed chorioretinal lacunae in the left eye. She had never had seizures and her psychomotor and language development were normal for age.

  10. Sex differences in the incidence of total callosal agenesis in BALB/cCF mice.

    Science.gov (United States)

    Manhães, Alex C; Medina, Alexandre E; Schmidt, Sergio L

    2002-06-14

    Corpus callosum (CC) development and adult morphology seems to be affected by sex. Here we analyzed the incidence of total callosal agenesis in 341 adult male and 318 female BALB/cCF mice. This strain of mice presents total or partial callosal agenesis in approximately 20-30% of its population. No significant differences were found in overall distributions of CC lengths and in average callosal lengths (totally acallosal excluded) between male and female mice. However, a highly significant difference in the incidence of total callosal agenesis was demonstrated: 18% (n=56) of the female mice presented such trait as opposed to 10% of males (n=34). This last result suggests that sex is a relevant factor in callosal development in its earliest stages of formation.

  11. 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.

  12. 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

    variables were acceptable in 92% of the implant reconstructions and for 83% of the tooth-supported fixed dental prostheses (FDPs). The total OHIP score was inferior in rehabilitated patients with tooth agenesis to that of the control group without tooth agenesis. The total OHIP score after rehabilitation......: The study included 129 patients with tooth agenesis rehabilitated with implant- or tooth-supported reconstructions, and a control group of 58 patients. Professional assessments included biological, technical and aesthetic variables. An aesthetic index score included mucosal discoloration, crown morphology......% of the patients in whom orthodontic treatment had been performed. Twelve percent of patients had implants with 5-7 mm peri-implant bone defects. Mucosal discoloration was recorded in 57% of the patients. Twelve percent of the patients had metal visible on the buccal side. The median scores for all five aesthetic...

  13. Pulmonary thromboembolism and right heart thromboemboli--an experience with tenecteplase.

    Science.gov (United States)

    Sharma, Anil; Udgire, Prashant P; Khan, Shanavas; Goyal, B K; Gautam, Shalima

    2012-10-01

    The presence of right heart thromboemboli complicating pulmonary thromboemboli carries with it an increased mortality rate compared to pulmonary thromboemboli alone, but little is known about the optimal management of this difficult clinical situation. We report a case of bilateral pulmonary thromboembolism with right heart thrombi treated successfully with thrombolysis with tenecteplase.

  14. Paradoxically greater interhemispheric transfer deficits in partial than complete callosal agenesis.

    Science.gov (United States)

    Aglioti, S; Beltramello, A; Tassinari, G; Berlucchi, G

    1998-10-01

    Symptoms of interhemispheric disconnection are typically much less severe in callosal agenesis than after surgical section of the corpus callosum. Sperry [Sperry, R. W., Plasticity of neural maturation. Developmental Biology, 1968, 2 (Suppl.), 306-327.] has attributed this difference to two interconnected factors: (1) the callosal section is usually performed after the brain has lost the maximal degree of functional plasticity associated with the early stages of development and (2) the removal of an already formed structure is more disruptive for functional brain organization than the failure of the same structure to develop. It has been suggested that functional compensation is less efficient if callosal agenesis is partial rather than complete [Dennis, M., Impaired sensory and motor differentiation with corpus callosum agenesis: A lack of callosal inhibition during ontogeny? Neuropsychologia, 1976, 14, 455-469.]. This suggestion is supported by the present findings of partial left-hand anomia, partial left-field alexia and poor tactile cross-localization in a subject with a congenital absence of the posterior part of the corpus callosum due to an arteriovenous malformation. In agreement with many previous studies, similar, though more severe, symptoms of interhemispheric disconnection were found in a subject with a complete section of the corpus callosum, but not in a subject with complete callosal agenesis. Praxic control of the left hand on verbal commands was severely deficient in the callosotomy subject, but it was normal in the subject with callosal hypogenesis. The lesser degree of compensation in partial compared to complete callosal agenesis may be explained by a reduced pressure to develop extracallosal means of interhemispheric communication, contingent on the partial existence of callosal connections, as well as by the later occurrence in development of the causes of callosal hypogenesis compared to those of total callosal agenesis.

  15. Pulmonary vasculitis.

    Science.gov (United States)

    Lally, Lindsay; Spiera, Robert F

    2015-05-01

    Pulmonary vasculitis encompasses inflammation in the pulmonary vasculature with involved vessels varying in caliber from large elastic arteries to capillaries. Small pulmonary capillaries are the vessels most commonly involved in vasculitis affecting the lung. The antineutrophil cytoplasmic antibody-associated vasculitides, which include granulomatosis with polyangiitis (formerly Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), are the small vessel vasculitides in which pulmonary vasculitis is most frequently observed and are the major focus of this review. Vasculitic involvement of the large pulmonary vessels as may occur in Behçet syndrome and Takayasu arteritis is also discussed.

  16. 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.)

  17. Incidentally discovered colpocephaly and corpus callosum agenesis in asymptomatic adult patient

    Directory of Open Access Journals (Sweden)

    Huseyin Buyukgol

    2016-09-01

    Full Text Available The corpus callosum is the biggest commissural structure located in two brain hemispheres and it ensures the connection between cortical and subcortical neurons. The agenesis of the corpus callosum is generally diagnosed in the prenatal period or childhood. It is observed along with congenital abnormalities such as mental and motor retardation, seizures, myelomeningocele. In this article, we reported a case of colpocephaly and corpus callosum agenesis with no symptoms that diagnosed incidentally. [Cukurova Med J 2016; 41(3.000: 564-566

  18. A case of single atrium and single ventricle physiology with bilateral cleft lip and palate for lip repair surgery

    Directory of Open Access Journals (Sweden)

    Rakhee Goyal

    2012-01-01

    Full Text Available Bilateral cleft lip and palate may occasionally be associated with complex congenital cyanotic heart disease. An infant with common atrium and single ventricle with infundibular pulmonary stenosis (Blalock-Taussig shunt done recently presented for lip repair surgery. Balanced general anesthesia was administered using sevoflurane along with a regional nerve block to maintain optimal pulmonary and systemic vascular resistance.

  19. Concomitant pulmonary arteriovenous and inferior vena cava malformations. A case report

    Energy Technology Data Exchange (ETDEWEB)

    Hawass, N.D.; Kolawole, T.M.; Badawi, M.G.

    1988-05-01

    A case of pulmonary arterio-venous malformation (AVM) presenting with dyspnoea diagnosed by computer tomography (CT) and pulmonary angiography is reported. Venous anomalies in the form of agenesis of the iliac veins and the inferior vena cava (IVC), were discovered through femoral venograms performed after technical difficulties were encountered at pulmonary angiography performed via the femoral route. These venous anomalies co-existed with normal but dilated azygos and hemiazygos systems, and with the azygos appearing as a right hilar mass lesion which showed the 'candy cane sign' on the lateral view on venography. A dilated varix at the confluence of the right renal vein and the IVC collaterals was also noted. This association of a pulmonary AVM and IVC agenesis has not been previously reported in the literature. The embryogenesis of the inferior vena cava and the azygos system is presented in an attempt to explain the rare association of these anomalies. The various techniques of diagnosing the disease entities, expecially by CT, are discussed. The rare co-existence of pulmonary AVM and the absence of the IVC is emphasized.

  20. Bilateral optic neuropathy with bilateral putaminal lesions: a case report.

    Science.gov (United States)

    Togawa, Jumpei; Ohi, Takekazu

    2015-01-01

    Bilateral optic neuropathy with bilateral putaminal lesions may be caused by methanol or cyanide poisoning or mitochondrial disorders including Leber hereditary optic neuropathy and Leigh syndrome. We report the case of a 34-year-old Japanese man who developed bilateral visual loss 5 days after the development of gastrointestinal symptoms. Magnetic resonance imaging of the brain on admission revealed high-intensity signal areas in the bilateral putamina on diffusion-weighted and T2-weighted images as well as a high-intensity signal area in the left middle cerebellar peduncle that had been identified 3 years previously. We diagnosed bilateral optic neuropathy with bilateral putaminal lesions caused by preceding infection-triggered demyelination. We administered methylprednisolone, but his vision did not recover.

  1. Ischemic Bilateral Opercular Syndrome

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    Aysel Milanlioglu

    2013-01-01

    Full Text Available Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum. Its clinical presentations include anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional activities of these structures are preserved. In the present case, an 81-year-old male presented with acute onset of anarthria with difficulties in chewing, speaking, and swallowing that was diagnosed with opercular syndrome.

  2. Ischemic bilateral opercular syndrome.

    Science.gov (United States)

    Milanlioglu, Aysel; Aydın, Mehmet Nuri; Gökgül, Alper; Hamamcı, Mehmet; Erkuzu, Mehmet Atilla; Tombul, Temel

    2013-01-01

    Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum. Its clinical presentations include anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional activities of these structures are preserved. In the present case, an 81-year-old male presented with acute onset of anarthria with difficulties in chewing, speaking, and swallowing that was diagnosed with opercular syndrome.

  3. Comprehension of humor in primary agenesis of the corpus callosum.

    Science.gov (United States)

    Brown, Warren S; Paul, Lynn K; Symington, Melissa; Dietrich, Rosalind

    2005-01-01

    Individuals with agenesis of the corpus callosum (ACC) can, in some cases, perform normally on standardized intelligence tests. Nevertheless, recent studies suggest that individuals with ACC and normal IQ scores have deficits in domains of fluid and social intelligence. Anecdotal reports from families suggest diminished appreciation of the subtleties of social interactions, and deficits in the comprehension of jokes and stories. In this research, both the cartoon and narrative joke subtests of a humor test (developed by Brownell et al. [Brownell, H., Michel, D., Powelson, J., & Gardner, H. (1983). Surprise but not coherence: sensitivity to verbal humor in right-hemisphere patients. Brain and language, 18(1), 20-27] and Bihrle et al. [Bihrle, A. M., Brownell, H. H., Powelson, J. A., & Gardner, H. (1986). Comprehension of humorous and non-humorous materials by left and right brain-damaged patients. Brain and Cognition, 5(4), 399-411]) were given to 16 adults with complete ACC (all with IQs>80) and 31 controls of similar age and IQ. Individuals with ACC performed worse than controls on the narrative joke subtest (p<.025) when VIQ was controlled. However, on the cartoon subtest the two groups were not significantly different. Covarying age, forms of IQ, narrative memory, set-switching, and literal language comprehension did not substantially alter the group difference. However, covarying comprehension of nonliteral language and proverbs eliminated the difference, suggesting a common origin for the comprehension of jokes, nonliteral language, and proverbs, most likely related to capacity for understanding second-order meanings.

  4. Proverb comprehension in individuals with agenesis of the corpus callosum.

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    Rehmel, Jamie L; Brown, Warren S; Paul, Lynn K

    2016-09-01

    Comprehension of non-literal language involves multiple neural systems likely involving callosal connections. We describe proverb comprehension impairments in individuals with isolated agenesis of the corpus callosum (AgCC) and normal-range general intelligence. Experiment 1 compared Gorham Proverb Test (Gorham, 1956) performance in 19 adults with AgCC and 33 neurotypical control participants of similar age, sex, and intelligence. Experiment 2 used the Proverbs subtest of the Delis-Kaplan Executive Function System (D-KEFS, 2001) to compare 19 adults with AgCC and 17 control participants with similar age, sex, and intelligence. Gorham Proverbs performance was impaired in the AgCC group for both the free-response and multiple-choice tasks. On the D-KEFS proverbs test, the AgCC group performed significantly worse on the free-response task (and all derivative scores) despite normal levels of performance on the multiple-choice task. Covarying verbal intelligence did not alter these outcomes. However, covarying a measure of non-literal language comprehension considerably reduced group differences in proverb comprehension on the Gorham test, but had little effect on the D-KEFS group differences. The difference between groups seemed to be greatest when participants had to generate their own interpretation (free response), or in the multiple choice format when the test included many proverbs that were likely to be less familiar. Taken together, the results of this study clearly show that proverb comprehension is diminished in individuals with AgCC compared to their peers.

  5. CASE OF BILATERAL ANTERIOR SEGMENT TUBERCULOSIS

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    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.

  6. 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.

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

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    Campeau, R.J.; Faust, J.M.; Ahmad, S.

    1987-11-01

    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.

  8. Paralinguistic Processing in Children with Callosal Agenesis: Emergence of Neurolinguistic Deficits

    Science.gov (United States)

    Brown, W.S.; Symingtion, M.; VanLancker-Sidtis, D.; Dietrich, R.; Paul, L.K.

    2005-01-01

    Recent research revealed impaired processing of both nonliteral meaning and affective prosody in adults with agenesis of the corpus callosum (ACC) and normal intelligence. Since normal children have incomplete myelination of the corpus callosum, it was hypothesized that paralanguage deficits in children with ACC would be less apparent relative to…

  9. 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

  10. Robot-assisted excision of seminal vesicle cyst associated with ipsilateral renal agenesis

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    Marcello Scarcia

    2016-01-01

    Full Text Available Seminal vesicle cysts (SVCs associated with other genitourologic abnormalities are rare. Often associated with ipsilateral renal agenesis in a symptomatic patient. In symptomatic patients open surgical excision is the treatment of choice. The laparoscopic approach is a less invasive option. Recently robot-assisted management has gained a primary role for the treatment of this condition.

  11. Pulmonary hypertension

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    Lauro Martins Júnior

    2014-12-01

    Full Text Available Pulmonary hypertension is a pathological condition associated with various diseases, which must be remembered by the physicians, since early diagnosis may anticipate and avoid dangerous complications and even death if appropriate measures were not taken. The relationship with chronic obstructive pulmonary disease (COPD, important pathological process that is in increasing prevalence in developing countries, and leading position as cause of death, emphasizes its importance. Here are presented the classifications, pathophysiology, and general rules of treatment of pulmonary hypertension.

  12. Perawatan Teknik Begg Pada Maloklusi Klas I Dengan Kaninus Impaksi dan Insisivus Lateral Agenesis

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    Kristina Wijaya Gunawan

    2013-06-01

    Full Text Available Impaksi kaninus maksila sering dijumpai pada sisi palatal daripada labial. Agenesis adalah anomali pertumbuhan akibat tidak ada satu atau lebih benih gigi. Laporan kasus ini bertujuan memaparkan kemajuan perawatan kasus Maloklusi klas I dengan kaninus impaksi dan insisivus lateral agenesis menggunakan alat cekat teknik Begg. Seorang pasien usia 20 tahun datang mengeluhkan gigi-gigi depan atas dan bawah yang berjejal sehingga mengganggu penampilan. Perawatan bertujuan untuk koreksi Maloklusi Angle klas I tipe dentoskeletal dengan deepbite, crossbite gigi 25 terhadap 35, pergeseran midline dental maksila dan mandibula ke kanan sebesar 2,5 mm dan 3,0 mm, 13 impaksi vertikal pada sisi labial, 42 agenesis, dan edentulous parsial regio 36. Koreksi dilakukan dengan pencabutan 14, 25, pemanfaatan ruang bekas pencabutan 36 dan exposure gigi kaninus yang impaksi. Tahap pertama teknik Begg adalah leveling, unraveling, dan bite opening, diikuti dengan koreksi midline dan penutupan sisa ruang bekas pencabutan. Kesimpulan: perawataan ortodontik menggunakan teknik Begg yang dilakukan simultan dengan exposure kaninus impaksi labial dengan closed eruption technique dapat memberikan hasil yang memuaskan. Treatment for Class I Malocclusion with Impacted Canine and Agenesis Lateral Incisor Using Begg Technique. Maxillary canine impaction occurs commonly on the palatal than labial side. Agenesis is a developmental anomaly condition because of the absence of one or more tooth buds. This case report aims to explain the treatment progress of class I malocclusion with impacted canine and agenesis lateral incisor using fixed appliance through Begg technique. A 20-year-old female patient complained about her upper and lower anterior dental crowding that disturbed her appearance. The treatment aims to correct the Angle class I malocclusion dentoskeletal types with deepbite, crossbite 25 to 35, maxillary and mandibulary dental midline shift to the right by 2.5 mm and 3.0 mm

  13. Bilateral Pneumothoraces Following Central Venous Cannulation

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    F. Pazos

    2009-01-01

    Full Text Available We report the occurrence of a bilateral pneumothoraces after unilateral central venous catheterization of the right subclavian vein in a 70-year-old patient. The patient had no history of pulmonary or pleural disease and no history of cardiothoracic surgery. Two days earlier, she had a median laparotomy under general and epidural anaesthesia. Prior to the procedure, the patient was hemodynamically stable and her transcutaneous oxygen saturation was 97% in room air. We punctured the right pleural space before cannulation of the right subclavian vein. After the procedure, the patient slowly became hemodynamically instable with respiratory distress. A chest radiograph revealed a complete left-side pneumothorax and a mild right-side pneumothorax. The right-side pneumothorax became under tension after left chest tube insertion. The symptoms finally resolved after insertion of a right chest tube. After a diagnostic work-up, we suspect a congenital “Buffalo chests” explaining bilateral pneumothoraces and a secondary tension pneumothorax.

  14. Bilateral Antepartum Mastitis

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    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.

  15. [Bilateral cochlear implantation].

    Science.gov (United States)

    Kronenberg, Jona; Migirov, Lela; Taitelbaum-Swead, Rikey; Hildesheimer, Minka

    2010-06-01

    Cochlear implant surgery became the standard of care in hearing rehabilitation of patients with severe to profound sensorineural hearing loss. This procedure may alter the lives of children and adults enabling them to integrate with the hearing population. In the past, implantation was performed only in one ear, despite the fact that binaural hearing is superior to unilateral, especially in noisy conditions. Cochlear implantation may be performed sequentially or simultaneously. The "sensitive period" of time between hearing loss and implantation and between the two implantations, when performed sequentially, significantly influences the results. Shorter time spans between implantations improve the hearing results after implantation. Hearing success after implantation is highly dependent on the rehabilitation process which includes mapping, implant adjustments and hearing training. Bilateral cochlear implantation in children is recommended as the proposed procedure in spite of the additional financial burden.

  16. Idiopathic Bilateral Bloody Tearing

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    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.

  17. 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.

  18. Sequence analysis of candidate genes in two Roma families with severe tooth agenesis

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    Gabriková Dana

    2016-01-01

    Full Text Available Selective tooth agenesis is the most common congenital disorder affecting the formation of dentition in humans. Both its forms (hypodontia and more severe oligodontia can be found either in isolated form and they can be associated with systemic condition (syndromic tooth agenesis. In addition to previously known genes (PAX9, MSX1 and AXIN2 mutations in EDA, EDARADD and WNT10 gene were recently found to be involved in isolated forms of tooth agenesis. The objective of this study was to characterize the phenotype of affected members in two large families of Roma origin segregating severe isolated tooth agenesis with very variable phenotype and to perform mutation analysis of seven genes with aim to find causal mutation. 26 family members were clinically examined and coding regions of seven genes (MSX1, PAX9, AXIN2, EDA, EDAR, EDARADD and WNT10A were sequenced. With exclusion of third molars, average number of missing teeth was 8.2 ± 4.9 in family 1 and 7.1 ± 2.3 in family 2. The most frequently missing teeth were maxillary lateral incisors and first premolars and mandibular central incisors. Sequencing revealed four potentially damaging variants (g.Ala40Gly in MSX1, g.Ala240Pro in PAX9, g.Pro50Ser in AXIN2 and g.Met9Ile in EDARADD; however, none of them was present in all affected family members. Variable phenotype in both families examined in this study is in favour of heterogeneous genetic cause of tooth agenesis in these families: possible interaction of several defected genes, sequence variants in regulatory regions and additional environmental factors is assumed.

  19. Type II congenital cystic pulmonary malformation in an esophageal lung.

    Science.gov (United States)

    Martínez-Martínez, Blanca E; Furuya, María Elena Yuriko; Martínez-Muñiz, Irma; Vargas, Mario H; Flores-Salgado, Rosalinda

    2013-01-01

    A seven-month-old girl, born prematurely (birth weight 1000 g) from a twin pregnancy, was admitted to hospital due to recurrent pneumonia and atelectasis. She experienced cough and respiratory distress during feeding. The right hemithorax was smaller than the left, with diminished breath sounds and dullness. Chest x-rays revealed decreased lung volume and multiple radiolucent images in the right lung, as well as overdistention of the left lung. An esophagogram revealed three bronchial branches arising from the lower one-third of the esophagus, corresponding to the right lung and ending in a cul-de-sac. A diagnosis of esophageal lung was established. On bronchography, the right lung was absent and the trachea only continued into the left main bronchus. Echocardiography and angiotomography revealed agenesis of the pulmonary artery right branch. The surgical finding was an esophageal right lung, which was removed; the histopathological diagnosis was type II congenital pulmonary airway malformation in an esophageal lung.

  20. Silicosis with bilateral spontaneous pneumothorax

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    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.

  1. 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.

  2. CT Pulmonary Angiography and Suspected Acute Pulmonary Embolism

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    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.

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

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    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

  4. Pulmonary alveolar microlithiasis

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    Surender Kashyap

    2013-01-01

    Full Text Available Pulmonary alveolar microlithiasis (PAM is a rare, chronic lung disease with bilateral intra-alveolar calcium and phosphate deposition throughout the lung parenchyma with predominance to lower and midzone. Although, etiology and pathogenesis of PAM is not fully understood, the mutation in SLC34A2 gene that encodes a sodium-phosphate co-transporter in alveolar type II cells resulting in the accumulation and forming of microliths rich in calcium phosphate (due to impaired clearance are considered to be the cause of the disease. Chest radiograph and high-resolution CT of thorax are nearly pathognomonic for diagnosing PAM. HRCT demonstrates diffuse micronodules showing slight perilobular predominance resulting in calcification of interlobular septa. Patients with PAM are asymptomatic till development of hypoxemia and cor-pulmonale. No therapy has been proven to be beneficial except lung transplantation.

  5. Traumatic bilateral hip dislocation with bilateral sciatic nerve palsy

    Institute of Scientific and Technical Information of China (English)

    Ajay Pal Singh; Amarjit Singh Sidhu; Arun Pal Singh

    2010-01-01

    Bilateral hip dislocation rarely occurs.In this paper, a case of bilateral hip dislocation associated with bilateral sciatic nerve palsy resulted from a road traffic acci-dent is reported.Both hips were emergently reduced under general anaesthesia.Acetabular reconstruction was done bilaterally due to the unstable hips.The patient subsequently developed heterotopic ossification and avascular necrosis on the left hip and underwent total hip arthroplasty.The sciatic nerve on the right side achieved complete recovery but that on the left side only partly recovered and was aug-mented by tendon transfer.Such injuries are serious and one should be aware of the complications because they can resurface and so patients should be followed up for a long time.To the best of our knowledge, this kind of injury has not been reported in the English .language literature.

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

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    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.

  7. 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.

  8. 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 ...

  9. Pulmonary aspergilloma

    Science.gov (United States)

    ... grows on dead leaves, stored grain, bird droppings, compost piles, and other decaying vegetation. Cavities in the ... Histoplasmosis Lung cancer - small cell Pulmonary tuberculosis Sarcoidosis Review Date 8/31/2014 Updated by: Jatin M. ...

  10. Uterine conserving surgery in a case of cervicovaginal agenesis with unicornuate uterus

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    Vineet Mishra

    2016-01-01

    Full Text Available The presence of cervicovaginal agenesis with unicornuate uterus is a very rare mullerian anomaly. Its true incidence is still unknown. The presence of functioning unicornuate uterus poses a great challenge for a gynecologist because a successful repair could restore normal menses and may preserve a patient′s fertility. Hence, we report a case of 16-year-old unmarried female who presented with chief complaints of primary amenorrhea with cyclical lower abdominal pain. On clinical and radiological evaluation, she was diagnosed with complete cervicovaginal agenesis with right unicornuate uterus (hematometra and hematosalpinx. She underwent vaginoplasty (McIndoes method along with uterovaginal anastomosis by neocervix formation, in order to preserve her uterus. On follow-up, her vagina was completely healed, and she was menstruating normally.

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

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    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.

  12. Uterine conserving surgery in a case of cervicovaginal agenesis with unicornuate uterus

    Science.gov (United States)

    Mishra, Vineet; Saini, Suwa Ram; Nanda, Sakshi; Choudhary, Sumesh; Roy, Priyankur; Singh, Tanvir

    2016-01-01

    The presence of cervicovaginal agenesis with unicornuate uterus is a very rare mullerian anomaly. Its true incidence is still unknown. The presence of functioning unicornuate uterus poses a great challenge for a gynecologist because a successful repair could restore normal menses and may preserve a patient's fertility. Hence, we report a case of 16-year-old unmarried female who presented with chief complaints of primary amenorrhea with cyclical lower abdominal pain. On clinical and radiological evaluation, she was diagnosed with complete cervicovaginal agenesis with right unicornuate uterus (hematometra and hematosalpinx). She underwent vaginoplasty (McIndoes method) along with uterovaginal anastomosis by neocervix formation, in order to preserve her uterus. On follow-up, her vagina was completely healed, and she was menstruating normally. PMID:28216917

  13. [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.

  14. [Seminal vesicle cyst associated with homolateral renal agenesis and megaureter. Apropos of a case].

    Science.gov (United States)

    Rabii, R; Aboutaieb, R; Joual, A; el Mrini, M; Benjelloun, S

    1997-01-01

    We report a case of a 24-year-old male patient with cystic dilatation of the seminal vesicle associated with renal agenesis and megaureter. This patient complained of left renal colic with pollakiuria. We performed intravenous urography and ultrasound showed a cyst in the seminal vesicle associated with left megaureter and right renal agenesis. Treatment consisted of resection of the cyst and reimplantation of the left ureter into the bladder. No postoperative complications were observed. This association is rare and can be explained by the common embryological origin of the seminal vesicle and kidney and ureter. Its diagnosis is now facilitated by ultrasound and CT. The treatment of this tumor is surgical only when the lesions are responsible for clinical symptoms.

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

    Directory of Open Access Journals (Sweden)

    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.

  16. Dental root agenesis following radiation and antineoplastic therapy: A Case Report

    Directory of Open Access Journals (Sweden)

    Abdul Hafiz

    2016-01-01

    Full Text Available The survival rates of patients suffering from various childhood neoplasms have improved dramatically with the advent of chemo-radiation therapy. The harmful effects of chemo-radiation therapy in the oro-facial region such as root agenesis, short roots, impaired amelogenesis, dentinogenesis, radiation caries, and other soft tissue pathologies are well recognized. In spite of these documented risks, the antineoplastic treatment modalities continue to serve the patient for overall improvement in survival and quality of life. However, a thorough understanding of the growth and development process and its relation with the complex antineoplastic treatment is very important for all clinicians. Such awareness could significantly improve the status of patients in the posttreatment period with the implementation of proper preventive and interceptive measures. This article intends to document a case of root agenesis that developed secondary to chemo-radiation therapy in a 12-year-old girl.

  17. Agenesis of the dorsal pancreas: systematic review of a clinical challenge

    Directory of Open Access Journals (Sweden)

    Javier A. Cienfuegos

    Full Text Available Background: Agenesis of the dorsal pancreas is a rare malformation. Since 1911 and until 2008, 53 cases have been reported. Several authors have recently described the association of this anomaly with neoplasia of the ventral pancreas, thus we performed a systematic review of the literature from 2008 to 2015. Methods: A systematic review of the Medline and ISI Web of Science Databases from 2008 until 2015 was carried out, and 30 articles which met the inclusion criteria were identified that included a total of 53 patients: 7 children and 46 adults. Conclusions: Although dorsal pancreatic agenesis is a rare malformation, given its association with non-alcoholic pancreatitis and neoplasia of the residual pancreas, physicians should maintain an expectant attitude.

  18. 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....

  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 Olecranon Tophaceous Gout Bursitis

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    Güzelali Özdemir

    2017-01-01

    Full Text Available In this case, we present a patient with the diagnosis of bilateral olecranon tophaceous gout. After the surgical treatment, there was no limitation of range of motion or wound problem at 6th month control.

  1. Pulmonary hypertension

    OpenAIRE

    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...

  2. 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.

  3. 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.

  4. Bilateral Morgagni Hernia in Adult

    Directory of Open Access Journals (Sweden)

    Ali Celik

    2014-03-01

    Full Text Available       Morgagni hernia is a congenital anterior diaphragma hernias. Although it generally seen in childhood and on the right side, rarely seen bilaterally and adult. Computarize tomography is helpful in diagnosis for this lesions asymptomatic in adult. In this article, bilaterally morgagni hernia diagnosed a sixty-five year old male patient looked for due to dyspne was presented.

  5. Bilateral sarkoidose i glandula parotis

    DEFF Research Database (Denmark)

    Hahn, Pernille; Krogdahl, Annelise; Godballe, Christian

    2012-01-01

    We describe an unusual case of sarcoidosis in which the patient presented with a bilateral swelling of the parotid salivary glands and no other manifestation of the disease. Sarcoidosis is a multisystem granulomatous disorder of unknown cause in which there may be multiple exocrine involvement......, including the salivary glands. This case emphasises the importance of including sarcoidosis in the differential diagnosis of bilateral parotid swelling....

  6. 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.

  7. 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.

  8. Diffusion-weighted imaging in fetuses with unilateral cortical malformations and callosal agenesis.

    Science.gov (United States)

    Glenn, O A; Quiroz, E M; Berman, J I; Studholme, C; Xu, D

    2010-06-01

    DWI was performed in fetuses with callosal agenesis and unilateral cortical malformations. ADC values were retrospectively measured in the developing white matter underlying the cortical malformation and compared with the corresponding contralateral white matter. In all 3 patients, ADC values were lower under the areas of cortical malformation compared with the normal contralateral side. Our findings suggest that there are structural differences in the developing white matter underlying areas of cortical malformation.

  9. The Syndrome of Frontonasal Dysplasia, Callosal Agenesis, Basal Encephalocele, and Eye Anomalies - Phenotypic and Aetiological Considerations.

    Science.gov (United States)

    Richieri-Costa, Antonio; Guion-Almeida, Maria Leine

    2004-01-01

    We report ten sporadic cases of Brazilian patients with facial midline defects, callosal agenesis, basal encephalocele, and ocular anomalies. This very rare cluster of anomalies has been well reported before. However, only until recently it is recognized as a syndrome belonging to frontonasal dysplasia spectrum. The ten cases confirm a distinct clinical entity and help to define the phenotype more precisely than previously. Up to now etiology remains unknown, although we conjecture that it is due to a mutation in TGIF gene.

  10. Interhemispheric Connections between the Primary Visual Cortical Areas via the Anterior Commissure in Human Callosal Agenesis

    OpenAIRE

    van Meer, Nathalie; Houtman, Anne C.; Van Schuerbeek, Peter; Vanderhasselt, Tim; Milleret, Chantal; ten Tusscher, Marcel P.

    2016-01-01

    Aim: In humans, images in the median plane of the head either fall on both nasal hemi-retinas or on both temporal hemi-retinas. Interhemispheric connections allow cortical cells to have receptive fields on opposite sides. The major interhemispheric connection, the corpus callosum, is implicated in central stereopsis and disparity detection in front of the fixation plane. Yet individuals with agenesis of the corpus callosum may show normal stereopsis and disparity vergence. We set out to study...

  11. 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.

  12. Mental State Understanding in Children with Agenesis of the Corpus Callosum.

    Science.gov (United States)

    Lábadi, Beatrix; Beke, Anna M

    2017-01-01

    Impaired social functioning is a well-known outcome of individuals with agenesis of the corpus callosum. Social deficits in nonliteral language comprehension, humor, social reasoning, and recognition of facial expression have all been documented in adults with agenesis of the corpus callosum. In the present study, we examined the emotional and mentalizing deficits that contributing to the social-cognitive development in children with isolated corpus callosum agenesia, including emotion recognition, theory of mind, executive function, working memory, and behavioral impairments as assessed by the parents. The study involved children between the age of 6 and 8 years along with typically developing children who were matched by IQ, age, gender, education, and caregiver's education. The findings indicated that children with agenesis of the corpus callosum exhibited mild impairments in all social factors (recognizing emotions, understanding theory of mind), and showed more behavioral problems than control children. Taken together, these findings suggest that reduced callosal connectivity may contribute to the development of higher-order social-cognitive deficits, involving limits of complex and rapidly occurring social information to be processed. The studies of AgCC shed lights of the role of structural connectivity across the hemispheres in neurodevelopmental disorders.

  13. Mental State Understanding in Children with Agenesis of the Corpus Callosum

    Science.gov (United States)

    Lábadi, Beatrix; Beke, Anna M.

    2017-01-01

    Impaired social functioning is a well-known outcome of individuals with agenesis of the corpus callosum. Social deficits in nonliteral language comprehension, humor, social reasoning, and recognition of facial expression have all been documented in adults with agenesis of the corpus callosum. In the present study, we examined the emotional and mentalizing deficits that contributing to the social-cognitive development in children with isolated corpus callosum agenesia, including emotion recognition, theory of mind, executive function, working memory, and behavioral impairments as assessed by the parents. The study involved children between the age of 6 and 8 years along with typically developing children who were matched by IQ, age, gender, education, and caregiver's education. The findings indicated that children with agenesis of the corpus callosum exhibited mild impairments in all social factors (recognizing emotions, understanding theory of mind), and showed more behavioral problems than control children. Taken together, these findings suggest that reduced callosal connectivity may contribute to the development of higher-order social-cognitive deficits, involving limits of complex and rapidly occurring social information to be processed. The studies of AgCC shed lights of the role of structural connectivity across the hemispheres in neurodevelopmental disorders. PMID:28220087

  14. 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.

  15. Neurogenic Pulmonary Edema (A Case Report

    Directory of Open Access Journals (Sweden)

    Funda Gümüş

    2012-08-01

    Full Text Available Neurogenic pulmonary edema is a life threatening complication of severe central nervous system injury. The most common cause of neurogenic pulmonary edema is subarachnoid hemorrhage followed by head trauma and epilepsy. The rare causes are cervical spine trauma, multiplesclerosis, cerebellar hemorrhage and intracranial tumors. Neurogenic pulmonary edema is characterized by an increase in extravascular lung water in patients who have sustained a sudden change in neurologic condition. The exact pathophysiology is unclear but it probably involves an adrenergic response to the central nervous system injury which leads to increased catecholamine, pulmonary hydrostatic pressure and increased lung capillary permeability. The presenting symptoms are nonspecific and often include dyspnea, tachypnea, tachycardia, hypoxemia, pinkfroty secretion, bilateral pulmonary infiltrates and crackles. These symptoms start within minutes or hours and resolves 48-72 hours that typically for neurogenic pulmonary edema. Basic principles of treatment, surgical decompression, reduce intracranial pressure, controlled ventilation with suplemental oxygen, positive end expiratory pressure and diuresis. We report a case with neurogenic pulmonary edema that occured after head trauma. (Journal of the Turkish Society Intensive Care 2012; 10: 59-62

  16. Bilateral zeta functions and their applications

    OpenAIRE

    Shibukawa, Genki

    2011-01-01

    We introduce a new type of multiple zeta functions, which we call bilateral zeta functions, analogous to the Barnes zeta functions. The bilateral zeta function is a periodic function and shares certain basic properties of Barnes zeta function. Especially, we prove that the bilateral zeta function has a nice Fourier series expansion and the Barnes zeta function can be expressed as a finite sum of bilateral zeta functions. By these properties of the bilateral zeta functions, We obtain simple pr...

  17. 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.

  18. 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.

  19. Prevalence of tooth agenesis and peg-shaped maxillary lateral incisor associated with palatally displaced canine (PDC) anomaly.

    Science.gov (United States)

    Peck, S; Peck, L; Kataja, M

    1996-10-01

    Fifty-eight nonsyndromic North American white orthodontic patients with palatal displacement of one or both maxillary canine teeth were studied for associated tooth agenesis and peg-shaped maxillary lateral incisors. Agenesis of permanent teeth was identified by x-ray film analysis. Conical crown-size reduction (peg-shape anomaly) of the maxillary lateral incisor (l2) was determined by direct observation. Increases in absence of third molars and second premolars associated with the palatally displaced canine (PDC) anomaly were statistically very highly significant compared with normative data for tooth-agenesis prevalence. In contrast, the prevalence of l2 agenesis in the PDC sample showed no difference statistically compared with reference values. Reasons for this posterior site-specific suppression of tooth formation are not clear. The l2 peg-shape anomaly exceeded a 10-fold elevation in expression in the PDC sample, a very highly significant increase from normal prevalence. The findings are consistent with a hypothesis that the anomalies of tooth agenesis, tooth-size reduction, and PDC are biologic covariables in a complex of genetically related dental disturbances.

  20. Nasal Septal Agenesis and Attenuated Lower and Upper Lateral Cartilages in a 5-Year-Old Child: A Sporadic Finding

    Science.gov (United States)

    Aldaghri, Faris; Mrad, Mohamed Amir

    2016-01-01

    Introduction: Cartilaginous nasal septal agenesis is a rare finding. In fact, just one case has been reported to have congenital agenesis of all nasal cartilages in a 6-year-old child by Bakhshaee et al. The literature review shows another case that was reported by Ozek et al in Turkey, where they reported a case of total nasal agenesis that was associated with Tessier no. 30. We could not find a similar case in the literature where only agenesis of the nasal cartilaginous septum was present. Methods/Case Report: This is a case report of a 5-year-old child presenting to our clinic with agenesis of his nasal septum and attenuation of the upper and lower lateral cartilages. His parents were seeking a corrective procedure to improve the shape of his nose. He was a male child with a right unilateral cleft lip and palate that were corrected surgically in 2009 (lip repair) and 2010 (palate repair), respectively. Results: On postoperative week 3, the patient's mother brought him to the emergency department with a history of falling on his face while playing at home. Examination revealed swelling of the nose but no breathing difficulties. He still had an acceptable augmented nose but with sings of deviation and collapse. Discussion: We report this case to find an answer to how such cases can be approached in the future in terms of the surgical intervention required and to study the options of redoing such cases. PMID:28101289

  1. [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.

  2. 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.

  3. 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.

  4. Bilateral breast in brothers - abreast

    Directory of Open Access Journals (Sweden)

    Altamash Mohammed Yusuf Shaikh

    2013-01-01

    Full Text Available Gynecomastia is a common occurrence in pubertal age group, and is physiological in up to 65 percent of cases. When occurs in the family it should be investigated in order not to miss on a treatable etiology. Two brothers within the same family, presenting with bilateral gynecomastia of different causes and requiring different treatment are presented.

  5. Bilateral acetabular fracture without trauma

    OpenAIRE

    De Rosa, M. A.; G. Maccauro; D’Arienzo, M.

    1999-01-01

     In the absence of trauma fracture of the acetabulum is an extremely rare injury. We describe a 70 year old man who spontaneously developed fractures in both acetabulae due to bony insufficiency. It was successfully treated by bilateral total hip replacement.

  6. 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.

  7. 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.

  8. 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

  9. 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.

  10. Disrupted developmental organization of the structural connectome in fetuses with corpus callosum agenesis.

    Science.gov (United States)

    Jakab, András; Kasprian, Gregor; Schwartz, Ernst; Gruber, Gerlinde Maria; Mitter, Christian; Prayer, Daniela; Schöpf, Veronika; Langs, Georg

    2015-05-01

    Agenesis of the corpus callosum is a model disease for disrupted connectivity of the human brain, in which the pathological formation of interhemispheric fibers results in subtle to severe cognitive deficits. Postnatal studies suggest that the characteristic abnormal pathways in this pathology are compensatory structures that emerge via neural plasticity. We challenge this hypothesis and assume a globally different network organization of the structural interconnections already in the fetal acallosal brain. Twenty fetuses with isolated corpus callosum agenesis with or without associated malformations were enrolled and fiber connectivity among 90 brain regions was assessed using in utero diffusion tensor imaging and streamline tractography. Macroscopic scale connectomes were compared to 20 gestational age-matched normally developing fetuses with multiple granularity of network analysis. Gradually increasing connectivity strength and tract diffusion anisotropy during gestation were dominant in antero-posteriorly running paramedian and antero-laterally running aberrant pathways, and in short-range connections in the temporoparietal regions. In fetuses with associated abnormalities, more diffuse reduction of cortico-cortical and cortico-subcortical connectivity was observed than in cases with isolated callosal agenesis. The global organization of anatomical networks consisted of less segregated nodes in acallosal brains, and hubs of dense connectivity, such as the thalamus and cingulate cortex, showed reduced network centrality. Acallosal fetal brains show a globally altered connectivity network structure compared to normals. Besides the previously described Probst and sigmoid bundles, we revealed a prenatally differently organized macroconnectome, dominated by increased connectivity. These findings provide evidence that abnormal pathways are already present during at early stages of fetal brain development in the majority of cerebral white matter.

  11. 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.

  12. 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

  13. 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.

  14. Pulmonary Aplasia with Unusual Associations in a Woman

    Directory of Open Access Journals (Sweden)

    Arshad Altaf Bachh

    2014-03-01

    Full Text Available Failure of development of the primitive lung bud leads to an extremely rare congenital anomaly with a prevalence of 34 per 10 lac live births termed pulmonary aplasia. In half of such cases, associated congenital malformations of the cardiovascular, skeletal, gastrointestinal, or genitourinary systems are present. The Mayer-Rokitansky-Küster-Hauser (MRKH syndrome is defined as the congenital aplasia of the uterus and the upper two thirds of the vagina with normal secondary sexual characteristics, ovaries, and a normal karyotype (46, XX. We report an extremely rare association of right lung aplasia, MRKH syndrome, and right renal agenesis with left pelvic kidney, which to the best of our knowledge is the first such association reported.

  15. Pulmonary hypertension complicating pulmonary sarcoidosis

    NARCIS (Netherlands)

    Huitema, M P; Grutters, J C; Rensing, B J W M; Reesink, H J; Post, M C

    2016-01-01

    Pulmonary hypertension (PH) is a severe complication of sarcoidosis, with an unknown prevalence. The aetiology is multifactorial, and the exact mechanism of PH in the individual patient is often difficult to establish. The diagnostic work-up and treatment of PH in sarcoidosis is complex, and should

  16. Diagnosis of Bilateral Tonsil Cancers via Staging PET/CT: Case Report and Review

    Directory of Open Access Journals (Sweden)

    Edward M. Mannina

    2011-01-01

    Full Text Available Diagnostic workup of metastatic head and neck squamous cell carcinoma of unknown primary site has traditionally included CT and/or MRI imaging and endoscopic biopsies. Routine bilateral tonsillectomy is highly controversial and the role of PET/CT is evolving, both for identification of potential primary sites and the detection of distant metastases. We report a case of cervical nodal metastasis of squamous cell carcinoma from an unknown primary site, in which dual-modality PET/CT led to the unexpected diagnosis of synchronous bilateral tonsillar cancers. In addition, PET/CT correctly distinguished pulmonary sarcoidosis from metastatic disease in this patient.

  17. 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.

  18. Multidisciplinary approach for the aesthetic treatment of maxillary lateral incisors agenesis: thinking about implants?

    Science.gov (United States)

    de Avila, Érica Dorigatti; de Molon, Rafael Scaf; de Assis Mollo, Francisco; de Barros, Luiz Antonio Borelli; Capelozza Filho, Leopoldino; de Almeida Cardoso, Mauricio; Cirelli, Joni Augusto

    2012-11-01

    Missing maxillary lateral incisors create an esthetic problem with specific orthodontic and prosthetic considerations. Implants are commonly used to replace congenitally missing lateral incisors in adolescent orthodontic patients. However, an interdisciplinary approach should be observed during the diagnosis, prognosis, and treatment plan to provide a result with good predictability and meet the esthetic and functional expectations of the patient. The present study describes a case of a young patient with tooth agenesis of maxillary lateral incisors, which was conducted with an integrated planning. After 5-year follow-up of 2 fixed implant-supported prostheses, clinical and radiographic examination showed the treatment to be successful.

  19. Angle Class II, subdivision, with agenesis of mandibular second molars and extrusion of maxillary second molars

    Directory of Open Access Journals (Sweden)

    Rubens Rodrigues Tavares

    2015-04-01

    Full Text Available This clinical case reports the treatment of an Angle Class II malocclusion in a young woman with a balanced face affected by agenesis of second and third mandibular molars and subsequent extrusion of second maxillary molars. The atypical and peculiar occlusal anomaly led to individualized treatment proposed in order to normalize dental malpositions, with subsequent rehabilitation of edentulous areas by means of a multidisciplinary approach. This case was presented to the Brazilian Board of Orthodontics and Dentofacial Orthopedics (BBO in partial fulfillment of the requirements for obtaining the title of certified by the BBO.

  20. Gingival Cyst of the Adult: Report of an Inconspicuous Lesion Associated with Multiple Agenesis

    Directory of Open Access Journals (Sweden)

    Juliana Mançano Melhado Brod

    2017-01-01

    Full Text Available Gingival cyst of the adult is a rare slow growing and asymptomatic lesion that arises from the rests of the dental lamina. The present report describes the case of a miniscule adult gingival cyst in the lower anterior gingiva in a 51-year-old male with agenesis of lower premolars and lateral incisors. This paper contrasts the literature concerning the differentiation between the gingival cyst of the adult and the lateral periodontal cysts as well as the possible misguided concept that the former may be such rare an occurrence.

  1. Three-dimensional colour Doppler of ductus venous agenesis in the first trimester

    Directory of Open Access Journals (Sweden)

    Divya Singh

    2016-11-01

    Full Text Available Ductus venosus (DV has a pivotal role in the fetal circulation. It serves as a conduit connecting the fetal umbilical and portal venous system with the inferior vena cava (IVC. The absence of DV is an uncommon anomaly. In case of agenesis of DV, the umbilical vein joins the fetal systemic venous circulation via the intra-hepatic or extra-hepatic route. We report a case of absent DV with associated anomaly diagnosed in the first trimester using three-dimensional (3D colour Doppler.

  2. Arthrogryposis multiplex congenita with callosal agenesis and dentato-olivary dysplasia.

    Science.gov (United States)

    Saito, Yoshiaki; Hayashi, Masaharu; Miyazono, Yayoi; Shimogama, Tatsuro; Ohno, Kousaku

    2006-05-01

    We report the autopsy case of a boy with arthrogryposis multiplex congenita, associated with callosal agenesis and dentato-olivary dysplasia. The patient manifested with dysmorphic facial features and suffered from intractable epilepsy during the neonatal period. These sets of complications suggest that a common molecular mechanism may be involved in the development of corpus callosum and the folding of the dentate and inferior olivary nuclei. Deep brain structures, including the brainstem and the cerebellum, may be involved in the pathophysiology of symptomatic generalized epilepsy. The differential diagnoses for the clinical and pathological characteristics of this patient are discussed.

  3. Unilateral agenesis of the abdominal wall musculature: An early muscle deficiency.

    Science.gov (United States)

    Gerard-Blanluet, Marion; Port-Lis, Marylin; Baumann, Clarisse; Perrin-Sabourin, Laurence; Ebrad, Patrick; Audry, Georges; Delezoide, Anne-Lise; Verloes, Alain

    2010-11-01

    Prune-belly sequence (PBS) usually results from early urethral obstruction. In rare cases, PBS seems to be due to a faulty primary development of the parietal mesenchyme leading to underdevelopment of the abdominal wall musculature, and disorganization of the smooth muscles in the urinary tract. We report on two patients with segmental, unilateral wall musculature deficiency associated with homolateral agenesis of ribs. One patient also had hemivertebrae and the other one ipsilateral diaphragmatic eventration and aplasia cutis. This combination of anomalies may represent a localized deficiency in the development of somitic mesoderm mesenchyme during early embryogenesis.

  4. 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.)

  5. Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren's Syndrome

    OpenAIRE

    Go Makimoto; Michiko Asano; Nobukazu Fujimoto; Yasuko Fuchimoto; Katsuichiro Ono; Shinji Ozaki; Koji Taguchi; Takumi Kishimoto

    2012-01-01

    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-c...

  6. 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 ...

  7. 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

  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 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.

  10. Assessment of sexual functioning, mental health, and life goals in women with vaginal agenesis.

    Science.gov (United States)

    Morgan, Elizabeth M; Quint, Elisabeth H

    2006-10-01

    Vaginal agenesis is a congenital disorder defined by the incomplete formation of the vagina and other reproductive organs, often including the cervix, uterus, and fallopian tubes. For the patient, this frequently means infertility and an underdeveloped vagina with the subsequent difficulty to have vaginal intercourse. The patient with vaginal agenesis and her family then encounter a variety of psychological concerns that must be addressed during diagnosis, including weighing treatment options, managing interventions, and coping with long-term issues following diagnosis and treatment, such as partnership concerns and infertility. In this study, seven patients between the ages of 18 and 34 completed questionnaires assessing demographic information, sexual functioning, mental health, self-esteem, and life goals. Sexual functioning results were highly variable. Participants reported significant emotional reactions at diagnosis as well as anxiety about the disorder, specifically its role in relationships. However, overall, the group showed average levels of mental health and self-esteem. Participants also showed positive coping techniques through conceptualization of life goals.

  11. 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.

  12. 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.

  13. 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.

  14. 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 ...

  15. 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

  16. 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.

  17. Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren's Syndrome

    Science.gov (United States)

    Makimoto, Go; Asano, Michiko; Fujimoto, Nobukazu; Fuchimoto, Yasuko; Ono, Katsuichiro; Ozaki, Shinji; Taguchi, Koji; Kishimoto, Takumi

    2012-01-01

    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. PMID:23198246

  18. Massive Hematometra due to Congenital Cervicovaginal Agenesis in an Adolescent Girl Treated by Hysterectomy: A Case Report

    Directory of Open Access Journals (Sweden)

    Turki Gasim

    2013-01-01

    Full Text Available A case of massive hematometra with a bicornuate uterus in a 14-year-old mentally handicapped girl complicated by vaginal agenesis and absent cervix is presented. She was managed by abdominal hysterectomy and right salpingo-oophorectomy that included the ovarian cystadenoma. The left ovary was conserved. This treatment was considered appropriate for this patient.

  19. Genetic Variants of BMP2 and Their Association with the Risk of Non-Syndromic Tooth Agenesis

    Science.gov (United States)

    Wang, Yuting; Gu, Ning; Ma, Lan; Xu, Min; Ma, Junqing; Zhang, Weibing; Pan, Yongchu; Wang, Lin

    2016-01-01

    Non-syndromic tooth agenesis (or non-syndromic congenitally missing tooth) is one of the most common congenital defects in humans affecting the craniofacial function and appearance. Single nucleotide polymorphisms (SNPs) have been associated with an individual’s susceptibility to these anomalies. The aim of the present study was therefore to investigate the roles of the potentially functional SNPs of BMP2 in the occurrence of tooth agenesis. Overall, four potentially functional SNPs of BMP2 (rs15705, rs235768, rs235769 and rs3178250) were selected, and their associations with the susceptibility of tooth agenesis were evaluated in a case-control study of 335 non-syndromic tooth agenesis cases and 444 healthy controls. The SNPs rs15705 and rs3178250 were found to be associated with an individual’s risk of tooth agenesis (P = 0.046 and P = 0.039, respectively). Both SNPs showed an increased risk of mandibular incisor agenesis (rs15705, AA/AC vs. CC = 1.58, 95% CI = [1.06–2.34], P = 0.024; rs3178250, TT/TC vs. CC = 1.60, 95% CI = [1.08–2.37], P = 0.020). Bioinformatics analysis indicated that these two SNPs located at the 3’-untranslated region (3’-UTR) of BMP2 might alter the binding ability of miR-1273d and miR-4639-5p, respectively, which was confirmed by luciferase activity assays in the 293A and COS7 cell lines (P < 0.001 in 293A and P < 0.01 in COS7 for miR-1273d; and P < 0.001 in both cells for miR-4639-5p). Furthermore, BMP2 mRNA expression decreased after transfecting either miR-1273d or miR-4639-5p into these two cell lines (P < 0.01 in 293A and P < 0.001 in COS7 for miR-1273d, and P < 0.01 in both cell lines for miR-4639-5p). Taken together, our findings indicate that rs15705 and rs317250 are associated with the susceptibility of non-syndromic tooth agenesis by possibly affecting miRNAs and mRNA interaction. PMID:27362534

  20. Genetic Variants of BMP2 and Their Association with the Risk of Non-Syndromic Tooth Agenesis.

    Directory of Open Access Journals (Sweden)

    Yun Lu

    Full Text Available Non-syndromic tooth agenesis (or non-syndromic congenitally missing tooth is one of the most common congenital defects in humans affecting the craniofacial function and appearance. Single nucleotide polymorphisms (SNPs have been associated with an individual's susceptibility to these anomalies. The aim of the present study was therefore to investigate the roles of the potentially functional SNPs of BMP2 in the occurrence of tooth agenesis. Overall, four potentially functional SNPs of BMP2 (rs15705, rs235768, rs235769 and rs3178250 were selected, and their associations with the susceptibility of tooth agenesis were evaluated in a case-control study of 335 non-syndromic tooth agenesis cases and 444 healthy controls. The SNPs rs15705 and rs3178250 were found to be associated with an individual's risk of tooth agenesis (P = 0.046 and P = 0.039, respectively. Both SNPs showed an increased risk of mandibular incisor agenesis (rs15705, AA/AC vs. CC = 1.58, 95% CI = [1.06-2.34], P = 0.024; rs3178250, TT/TC vs. CC = 1.60, 95% CI = [1.08-2.37], P = 0.020. Bioinformatics analysis indicated that these two SNPs located at the 3'-untranslated region (3'-UTR of BMP2 might alter the binding ability of miR-1273d and miR-4639-5p, respectively, which was confirmed by luciferase activity assays in the 293A and COS7 cell lines (P < 0.001 in 293A and P < 0.01 in COS7 for miR-1273d; and P < 0.001 in both cells for miR-4639-5p. Furthermore, BMP2 mRNA expression decreased after transfecting either miR-1273d or miR-4639-5p into these two cell lines (P < 0.01 in 293A and P < 0.001 in COS7 for miR-1273d, and P < 0.01 in both cell lines for miR-4639-5p. Taken together, our findings indicate that rs15705 and rs317250 are associated with the susceptibility of non-syndromic tooth agenesis by possibly affecting miRNAs and mRNA interaction.

  1. "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.

  2. [Pulmonary strongyloidiasis].

    Science.gov (United States)

    Lozada, Heiler; Daza, Jorge E

    2016-10-01

    Strongyloidiasis is an infection caused by the parasite Strongyloides stercoralis, which can be asymptomatic and means a high morbidity and mortality in immunocompromised hosts, severe malnutrition and coinfection with HTLV-1 virus. The parasite has the potential to produce and multiply internal autoinfection in humans, thus an hyperinfection can be developed. A case of pulmonary infection by this parasite is presented in this study, infection which advanced into a respiratory failure and required mechanical ventilation and hemodynamic support in an intensive care unit. The standard treatment combined with ivermectin and albendazole was provided, achieving an appropriate response.

  3. 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.

  4. Pulmonary biomarkers in chronic obstructive pulmonary disease

    NARCIS (Netherlands)

    Barnes, Peter J.; Chowdhury, Badrul; Kharitonov, Sergei A.; Magnussen, Helgo; Page, Clive P.; Postma, Dirkje; Saetta, Marina

    2006-01-01

    There has been increasing interest in using pulmonary biomarkers to understand and monitor the inflammation in the respiratory tract of patients with chronic obstructive pulmonary disease (COPD). In this Pulmonary Perspective we discuss the merits of the various approaches by reviewing the current l

  5. [Idiopathic pulmonary hemosiderosis with dendriform pulmonary ossification].

    Science.gov (United States)

    Barrera, Ana Madeleine; Vargas, Leslie

    2016-12-01

    Pulmonary ossification is a rare and usually asymptomatic finding reported as incidental in lung biopsies. Similarly, idiopathic pulmonary hemosiderosis is a rare cause of pulmonary infiltrates. We report the case of a 64-year old man with chronic respiratory symptoms in whom these two histopathological findings converged.

  6. 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

  7. 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.

  8. Amelogenesis imperfecta with bilateral nephrocalcinosis.

    Science.gov (United States)

    Poornima, P; Katkade, Shashikant; Mohamed, Roshan Noor; Mallikarjuna, Rachappa

    2013-05-24

    A 12-year-old patient presented with a severe delay of eruption in permanent maxillary and mandibular incisors. On examination, there was over-retained primary teeth and delayed eruption of permanent teeth. Retained primary teeth showed light yellow discolouration whereas permanent teeth were distinct yellow with thin or little enamel. Subsequent imaging revealed all the premolars except maxillary left first premolar showed signs of intra-alveolar coronal resorption, nephrocalcinosis with bilateral multiple calculi and small papillary tip calcifications, marked increase in alkaline phosphatase. Subsequent dental treatment for restoring the functional and aesthetic requirement followed by appropriate treatment for renal problem was undertaken.

  9. 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

  10. Bilateral zosteriform extragenital lichen sclerosus.

    Science.gov (United States)

    Kumar, Piyush; Jha, Abhijeet Kumar; Mallik, Sambeet Kumar; Raihan, Mohammed

    2014-01-01

    A 35-year-old man presented with asymptomatic eruption on both forearms and lower aspects of the legs for 6 months. The lesions first appeared on his inner aspects of the wrist, the dorsal surface of the hands, and legs and progressed to involve proximal aspects of the extremities. There was no significant past history. On examination, multiple pearly white papules and depigmented atrophic plaques were found bilaterally on the flexors of the arms and the extensors of the legs. The lesions were arranged in a linear manner, following the lines of Blaschko (Figures 1 and 2). The surface of the atrophic plaques was notable for prominent telangiectasia, giving an erythematous appearance. The genitalia, oral cavity, palms, and soles were spared. Systemic examination was noncontributory. Lichen striatus and extragenital lichen sclerosus (ELS) were considered the differential diagnosis. Clinically, the age of the patient, the absence of scaling, and the presence of atrophic plaques and telangiectasia were in favor of ELS. A punch biopsy from an atrophic plaque was performed, and it revealed hyperkeratosis, atrophic epidermis, basal layer vacuolar degeneration, mild lymphocytic infiltration in the dermis, edema, and homogenization of collagen of the upper portion of the dermis (Figures 3 and Figure 4). Histopathologic findings were consistent with lichen sclerosus. A diagnosis of bilateral zosteriform ELS was made.

  11. 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...

  12. 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...

  13. Clinical and MRI findings of cerebellar agenesis in two living adult patients

    Directory of Open Access Journals (Sweden)

    Fazil Mustafa Gelal

    2016-01-01

    Full Text Available Cerebellar agenesis (CA is an extremely rare entity. We present two adult patients with CA. The 61-year-old man had ataxia, dysarthria, abnormalities in cerebellar tests, severe cognitive impairment, and moderate mental retardation. The 26-year-old woman had dysmetria, dysdiadochokinesia, and dysarthria as well as mild cognitive impairment and mild mental retardation. Magnetic resonance imaging (MRI showed complete absence of the cerebellum with small residual vermis. Brainstem was hypoplastic and structures above tentorium were normal. Supratentorial white matter bundles were unaffected in diffusion tensor tractography. Only few adult patients with CA have so far been published. These cases show that patients with CA present with a variety of developmental, clinical, and mental abnormalities; and emphasize the role of the cerebellum in normal motor, language, and mental development.

  14. 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.

  15. 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.

  16. Prenatal Diagnosis of Cantrell's Pentalogy Associated with Agenesis of Left Limb in a Twin Pregnancy

    Science.gov (United States)

    Cakiroglu, Yigit; Doger, Emek; Babaoglu, Kadir; Caliskan, Eray; Yucesoy, Gulseren

    2014-01-01

    Pentalogy of Cantrell is a rare malformation described in the literature. We report a case of pentalogy of Cantrell associated with left limb deficiency in a twin pregnancy. The fetus with multiple anomalies revealed kyphosis, ectopia cordis, and a large defect with protruding liver and bowel loops at 12 weeks and 3 days of gestational age on ultrasound scan. The other fetus was ultrasonographically normal. We diagnosed a case of pentalogy of Cantrell in a twin pregnancy after exclusion of limb body wall complex, body stalk anomaly, and amniotic band syndrome and after delivery of the fetuses. Macroscopic examinations were ectopia cordis, extrusion of the abdominal organs without membranes surrounding, and agenesis of the left limb. PMID:24782934

  17. 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.

  18. [Our experience with bilateral cochlear implantation].

    Science.gov (United States)

    Carmel, Eldar; Taitelbaum-Swead, Ricky; Migirov, Lela; Hildesheimer, Minka; Kronenberg, Jona

    2008-03-01

    Cochlear implantation is a standard method of hearing rehabilitation among patients with severe to profound bilateral sensorineural hearing loss. In recent years there have been an increasing number of studies showing superior hearing with bilateral cochlear implantation in comparison with a unilateral procedure. In this study we present our experience with 15 patients, children and adults, who had bilateral cochlear implant surgery. Speech perception test results demonstrated a hearing benefit in bilateral cochlear implantation in comparison with a unilateral device, mainly by improvement in the identification of speech in noise tests.

  19. 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 ...

  20. 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.

  1. 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.

  2. Bilateral microvascular second toe transfer for bilateral post-traumatic thumb amputation

    OpenAIRE

    Rajendra Nehete; Anita Nehete; Sandeep Singla; Harshad Adhav

    2012-01-01

    In bilateral thumb amputations, the functional impairment is serious and every attempt should be made to reconstruct the thumb. We report a case of bilateral post traumatic thumb amputation, reconstructed with bilateral second toe transfer. Only two such cases have been reported in literature so far. Though there are various modalities for the reconstruction of thumb, microvascular toe transfer has its own merits. The convalescent period is minimal with excellent function. It is bilaterally s...

  3. 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

  4. The effects of callosal agenesis on the susceptibility to seizures elicited by pentylenetetrazol in BALB/cCF mice.

    Science.gov (United States)

    Medina, Alexandre E; Manhães, Alex C; Schmidt, Sergio L

    2002-01-01

    The effects of callosal agenesis in sensitivity to pentylenetetrazol (PTZ) were studied in 199 (95 males and 104 females) mice of the BALB/cCF strain. This strain presents agenesis of the corpus callosum (CC) in approximately 30% of its population. Seizures were elicited by intraperitoneally injected PTZ. Animals were tested with doses of 40 and 50 mg/kg. Seizure severity was expressed by the following scoring scale: 0 (no abnormal behavior, NAB); 1 (myoclonus, M); 2 (running bouncing clonus, RBC); 3 (tonic hindlimb extension, THE). For the 40-mg/kg dose, abnormal mice were found to be more susceptible, displaying more severe seizures more often then normal mice. Normal female mice were also more susceptible to PTZ than males for this dose. No significant differences were found for the 50-mg/kg dose as a result of the fact that most animals displayed RBC. These data indicate that callosal development and sex are important factors affecting seizure susceptibility.

  5. 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)

  6. [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%).

  7. Radiologic findings of diffuse Pulmonary hemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Mi Ra; Song, Koun Sik; Lee, Jin Seong; Lim, Tae Hwan [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1998-12-01

    To describe the chest radiographic and CT findings of diffuse pulmonary hemorrhage. Two radiologists retrospectively analysed the chest radiographic and CT findings of six patients with diffuse pulmonary hemorrhage. Using open lung biopsy(n=3D2) and transbronchial lung biopsy or bronchoalveolar lavage(n=3D4), diagnosis was based on the presence of hemosiderin-laden macrophage or intra-alveolar hemorrhage. Underlying diseases were Wegener's granulomatosis(n=3D2), antiphospholipid antibody syndrome(n=3D2), Henoch-Schonlein purpura(n=3D1), and idopathic pulmonary hemosiderosis(n=3D1). In all patients, sequential chest radiographs, obtained during a one to six-month period, were available. HRCT scans were obtained in five patinets, and conventional CT scans in one. Follow-up HRCT scans were obtained in two. We also analyzed the patterns of involvement, distribution and sequential changes in the pulmonary abnormalities seen on chest radiographs and CT scans. Chest radiographs showed multifocal patchy consolidation(n=3D6), ground-glass opacity(n=3D3), and multiple granular or nodular opacity(n=3D3). These lesions were intermingled in five patients, while in one there was consolidation only. Sequential chest radiographs demonstrated the improvement of initial pulmonary abnormalities and appearance of new lesions elsewhere within 5-6 days, though within 7-25 (average, 13) days, these had almost normalized. HRCT scans showed patchy consolidation(n=3D5), multiple patchy ground-glass opacity(n=3D5), or ill-defined air space nodules(n=3D4). These lesions were intermingled in five patients, and in one, ground-glass opacity only was noted. In two patients there were interlobular septal thickening and intalobular reticular opacity. The distribution of these abnormalities was almost always bilateral, diffuse with no zonal predominancy, and spared the apex of the lung and subpleural region were less affected. Although chest radiographic and CT findings of diffuse pulmonary

  8. Discordant tooth agenesis and peg-shaped in a pair of monozygotic twins: Clinical and molecular study

    OpenAIRE

    Lívia Azeredo Alves Antunes; Erika Calvano Küchler; Marcelo de Castro Costa; Leonardo Santos Antunes; José Mauro Granjeiro

    2013-01-01

    The present report aimed to study an uncommon case of a pair of twins that presented a discordant dental phenotype. Family investigation, clinical, radiographic examination and molecular analysis were performed in both girls. Molecular analysis confirmed the monozygosity by deoxyribonucleic acid chip technology. One twin presented tooth agenesis in left upper lateral incisor and peg-shaped on the contra lateral side while the other twin had no dental alterations. The dental casts study employ...

  9. Type II Congenital Pulmonary Airway Malformation in an Esophageal Lung

    Directory of Open Access Journals (Sweden)

    Blanca Estela Martínez-Martínez

    2013-01-01

    Full Text Available A seven-month-old girl, born prematurely (birth weight 1000 g from a twin pregnancy, was admitted to hospital due to recurrent pneumonia and atelectasis. She experienced cough and respiratory distress during feeding. The right hemithorax was smaller than the left, with diminished breath sounds and dullness. Chest x-rays revealed decreased lung volume and multiple radiolucent images in the right lung, as well as overdistention of the left lung. An esophagogram revealed three bronchial branches arising from the lower one-third of the esophagus, corresponding to the right lung and ending in a cul-de-sac. A diagnosis of esophageal lung was established. On bronchography, the right lung was absent and the trachea only continued into the left main bronchus. Echocardiography and angiotomography revealed agenesis of the pulmonary artery right branch. The surgical finding was an esophageal right lung, which was removed; the histopathological diagnosis was type II congenital pulmonary airway malformation in an esophageal lung.

  10. 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.

  11. 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

  12. 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.

  13. Bilateral locked facets in the thoracic spine

    NARCIS (Netherlands)

    M.H.A. Willems; Braakman, R. (Reinder); B. van Linge (Bert)

    1984-01-01

    textabstractTwo cases of traumatic bilateral locked facets in the thoracic spine are reported. Both patients had only minor neurological signs. They both made a full neurological recovery after surgical reduction of the locked facets. Bilateral locked facets are very uncommon in the thoracic spine.

  14. 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.

  15. Spontaneous bilateral adrenal hemorrhage following cholecystectomy.

    Science.gov (United States)

    Dahan, Meryl; Lim, Chetana; Salloum, Chady; Azoulay, Daniel

    2016-06-01

    Postoperative bilateral adrenal hemorrhage is a rare but potentially life-threatening complication. This diagnosis is often missed because the symptoms and laboratory results are usually nonspecific. We report a case of bilateral adrenal hemorrhage associated with acute primary adrenal insufficiency following laparoscopic cholecystectomy. The knowledge of this uncommon complication following any abdominal surgery allows timey diagnosis and rapid treatment.

  16. Differential diagnosis problem of pulmonary changes in ulcerative colitis

    Directory of Open Access Journals (Sweden)

    Đurić Mirna

    2010-01-01

    Full Text Available Introduction. Inflammatory intestinal diseases are often accompanied with extraintestinal and even pulmonary manifestations. The treatment of these intestinal diseases includes sulphasalazine and mesalazine, which may have undesirable allergic and other side effects, including hypersensitive pneumonitis. Case report. Having performed colonoscopy due to abdominal pains and mushy stools, the diagnosis of ulcerous colitis was established in a 20-year old female patient and the treatment with mesalazine initiated. However, the patient developed slimy and bloody stools, slightly increased body temperature, dry cough and bilateral lung lesions. The bronchological exploration established interstitial and organizing pneumonia. Despite the treatment with antibiotics, corticosteroids and mesalazine, dry cough, fever and bloody stools persisted, accompanied with bilateral inhomogeneous lesions of the pulmonary parenchyma. Upon discontinuation of the treatment with mesalazine, clinical symptoms and pulmonary lesions disappeared. Conclusion. The differential diagnosis of pulmonary lesions in patients with ulcerous colitis may be a problem. In the reported case, pulmonary lesions were interpreted as interstitial pneumonitis accompanying ulcerous colitis. However, they are most probably a consequence of allergic response to mesalazine.

  17. Emergent Unilateral Renal Artery Stenting for Treatment of Flash Pulmonary Edema: Fact or Fiction?

    Directory of Open Access Journals (Sweden)

    Asaad Akbar Khan

    2015-01-01

    Full Text Available Flash pulmonary edema is characteristically sudden in onset with rapid resolution once appropriate therapy has been instituted (Messerli et al., 2011. Acute increase of left ventricular (LV end diastolic pressure is the usual cause of sudden decompensated cardiac failure in this patient population. Presence of bilateral renal artery stenosis or unilateral stenosis in combination with a single functional kidney in the susceptible cohort is usually blamed for this condition. We describe a patient who presented with flash pulmonary edema in the setting of normal coronary arteries. Our case is distinct as our patient developed flash pulmonary edema secondary to unilateral renal artery stenosis in the presence of bilateral functioning kidneys. Percutaneous stent implantation in the affected renal artery resulted in rapid resolution of pulmonary edema.

  18. Emergent Unilateral Renal Artery Stenting for Treatment of Flash Pulmonary Edema: Fact or Fiction?

    Science.gov (United States)

    Khan, Asaad Akbar; McFadden, Eugene Patrick

    2015-01-01

    Flash pulmonary edema is characteristically sudden in onset with rapid resolution once appropriate therapy has been instituted (Messerli et al., 2011). Acute increase of left ventricular (LV) end diastolic pressure is the usual cause of sudden decompensated cardiac failure in this patient population. Presence of bilateral renal artery stenosis or unilateral stenosis in combination with a single functional kidney in the susceptible cohort is usually blamed for this condition. We describe a patient who presented with flash pulmonary edema in the setting of normal coronary arteries. Our case is distinct as our patient developed flash pulmonary edema secondary to unilateral renal artery stenosis in the presence of bilateral functioning kidneys. Percutaneous stent implantation in the affected renal artery resulted in rapid resolution of pulmonary edema. PMID:25793128

  19. [A case report of right-sided cardiac and pulmonary thromboembolism treated by emergent operation].

    Science.gov (United States)

    Asaoka, M; Sasaki, M; Masumoto, H; Kajiyama, M; Seki, A

    1996-05-01

    A forty-four-year-old man with a clinical diagnosis of diabetes melitus and severe obesity (height 170 cm, weight 108 kg) was admitted to the hospital on 12th January 1995 because of acute myocardial infarction, and on 21st January, he was referred to our hospital with sudden onset of shock, bradycardia, loss of consciousness in spite of having recovered well from myocardial infarction. The echocardiography and pulmonary arteriography revealed a pulmonary embolism and a tumor in the right atrium. Administration of tissue plasminogen activator (TPA) was not sufficiently effective. An emergency operation (pulmonary arteriotomy, right atriotomy, milking of bilateral lungs) with cardiopulmonary bypass revealed a massive consecutive thrombus, which occupied the right atrium, right ventricle and bilateral pulmonary artery. The postoperative course was uneventful.

  20. Bilateral zeta functions and their applications

    CERN Document Server

    Shibukawa, Genki

    2011-01-01

    We introduce a new type of multiple zeta functions, which we call bilateral zeta functions, analogous to the Barnes zeta functions. The bilateral zeta function is a periodic function and shares certain basic properties of Barnes zeta function. Especially, we prove that the bilateral zeta function has a nice Fourier series expansion and the Barnes zeta function can be expressed as a finite sum of bilateral zeta functions. By these properties of the bilateral zeta functions, We obtain simple proofs of some formulas, for example the reflection formula for the multiple gamma function, the inversion formula of the Dedekind eta function, Ramanujan's formula, Fourier expansion of the Barnes zeta function and multiple Iseki's formula.

  1. 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.

  2. 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. PMID:26229538

  3. 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.

  4. Tenecteplase in the treatment of acute pulmonary thrombo-embolism.

    Science.gov (United States)

    Bhuvaneswaran, J S; Premchand, Rajendra Kumar; Iyengar, S S; Rajeev Khare; Chabra, C B; Padmanabhan, T N C; Sharma, S K; Jain, Alkesh; Pandian, S A; Rajdev, S; Modi, N; Kumar, V

    2011-05-01

    This is a retrospective study documenting the use of tenecteplase in 41 cases of suspected or confirmed pulmonary embolism receiving in-hospital tenecteplase as per weight-adjusted dosing in addition to standard heparin and oral anticoagulant therapy. The presenting symptoms of dyspnoea, chest pain, hemoptysis and syncope were found in 40 (97.56%), 19 (46.34%), 6 (14.63%) and 9 (21.95%) patients, respectively. There was one case of mortality who was a 26 yrs old female of postpartum pulmonary thrombo-embolism with severe hypotension, cyanosis, bilateral crepitations in lungs and pulmonary hypertension. In the 40 survived patients, there was alleviation of dyspnoea and hemoptysis in all patients. Significant reduction in tachycardia (P tenecteplase therapy. Resolution of pulmonary embolism on CT pulmonary angiography was documented in only two patients. No bleeding events or any other adverse events were reported during this study. The present study suggests favourable efficacy of tenecteplase in patients with suspected or confirmed acute pulmonary embolism. Although no major adverse events were noted, a large prospective study on the use of tenecteplase in pulmonary embolism is suggested.

  5. 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.

  6. Histoplasmosis - acute (primary) pulmonary

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000098.htm Histoplasmosis - acute (primary) pulmonary To use the sharing features on this page, please enable JavaScript. Acute pulmonary histoplasmosis is a respiratory infection that is caused by ...

  7. 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.

  8. Pulmonary Hemorrhage in Cryoglobulinemia

    Directory of Open Access Journals (Sweden)

    G Kirkpatrick

    2015-01-01

    Full Text Available Pulmonary manifestations of cryoglobulinemia are uncommon and their clinical behaviour is unpredictable, ranging from mild dyspnea to life-threatening presentations. A patient with cryoglobulinemia who presented with hypoxic respiratory failure attributed to pulmonary hemorrhage is reported.

  9. CT findings of pulmonary tuberculosis and tuberculous pleurisy in diabetes mellitus patients

    Science.gov (United States)

    Kim, Jihyun; Lee, In Jae; Kim, Joo-Hee

    2017-01-01

    PURPOSE We aimed to assess computed tomography (CT) findings of pulmonary tuberculosis (TB) and TB pleurisy in diabetes mellitus (DM) patients and to evaluate the effect of duration of DM on radiologic findings of pulmonary TB and TB pleurisy. METHODS Ninety-three consecutive patients diagnosed as active pulmonary TB with underlying DM were enrolled in our study. As a control group, 100 pulmonary TB patients without DM were randomly selected. TB patients with DM were subdivided into two subgroups depending on diabetes duration of ≥10 years or <10 years. Medical records and CT scans of the patients were retrospectively reviewed and compared. RESULTS Bilateral pulmonary involvement (odds ratio [OR]=2.39, P = 0.003), involvement of all lobes (OR=2.79, P = 0.013), and lymph node enlargement (OR=1.98, P = 0.022) were significantly more frequent CT findings among TB patients with DM compared with the controls. There were no statistically significant differences in CT findings of pulmonary TB depending on the duration of DM. CONCLUSION Bilateral pulmonary involvement, involvement of all lobes, and lymph node enlargement are significantly more common CT findings in TB patients with underlying DM than in patients without DM. Familiarity with the CT findings may be helpful to suggest prompt diagnosis of pulmonary TB in DM patients. PMID:28185999

  10. 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

  11. Pulmonary Hypertension Association

    Science.gov (United States)

    ... at www.AHeartCures.org . Help Kickoff November’s Pulmonary Hypertension Awareness Month Want to help raise awareness for ... Heart2CurePH | Help promote Awareness Month Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Awareness Chronic thromboembolic pulmonary hypertension (CTEPH) is ...

  12. Pulmonary Hypertension Overview

    Science.gov (United States)

    ... chest X-ray, a breathing test called a pulmonary function test and an echocardiogram (sometimes called an “echo”). Your doctor may also need to do other tests to find out whether another medical condition is causing your pulmonary hypertension. TreatmentHow is pulmonary hypertension treated?If the ...

  13. Sound localization ability of young children with bilateral cochlear implants.

    NARCIS (Netherlands)

    Beijen, J.W.; Snik, A.F.M.; Mylanus, E.A.M.

    2007-01-01

    OBJECTIVE: To evaluate the benefit of bilateral cochlear implantation in young children. STUDY DESIGN: Clinical trial comparing a group of bilaterally implanted children with a group of unilaterally implanted children. SETTING: Tertiary referral center. PATIENTS: Five bilaterally implanted children

  14. Heterochronic bilateral ectopic pregnancy after ovulation induction.

    Science.gov (United States)

    Zhu, Bo; Xu, Gu-feng; Liu, Yi-feng; Qu, Fan; Yao, Wei-miao; Zhu, Yi-min; Gao, Hui-juan; Zhang, Dan

    2014-08-01

    Ectopic pregnancy is identified with the widely-applied assisted reproductive technology (ART). Bilateral ectopic pregnancy is a rare form of ectopic pregnancy which is difficult to be diagnosed at the pre-operation stage. In this paper, we presented an unusual case of heterochronic bilateral ectopic pregnancy after stimulated intrauterine insemination (IUI), where there has been a delay of 22 d between the diagnoses of the two ectopic pregnancies. Literature was reviewed on the occurrence of bilateral ectopic pregnancy during the past four years in the MEDLINE database. We found 16 cases of bilateral ectopic pregnancy reported since 2008, and analyzed the characteristics of those cases of bilateral ectopic pregnancy. We emphasize that ovulation induction and other ARTs may increase the risk of bilateral ectopic pregnancy. Because of the difficulty in identification of bilateral ectopic pregnancy by ultrasonography, the clinician should be aware that the treatment of one ectopic pregnancy does not preclude the occurrence of a second ectopic pregnancy in the same patient and should pay attention to the intra-operation inspection of both side fallopian tubes in any ectopic pregnancy case.

  15. Bilateral optic neuropathy in acute cryptococcal meningitis

    Institute of Scientific and Technical Information of China (English)

    Qi Zhe Ngoo; Li Min Evelyn Tai; Wan Hazabbah Wan Hitam; John Tharakan

    2016-01-01

    We reported a case of cryptococcal meningitis presenting with bilateral optic neuropathy in an immunocompetent patient. A 64-year-old Malay gentleman with no medical comorbidities presented with acute bilateral blurring of vision for a week, which was associated with generalised throbbing headache and low grade fever. He also had som-nolence and altered consciousness. Visual acuity in both eyes was no perception of light with poor pupillary reflexes. Extraocular muscle movements were normal. Anterior segments were unremarkable bilaterally. Fundoscopy revealed bilateral optic disc swelling. CT scan of the brain showed multifocal infarct, but no meningeal enhancement or mass. Cerebrospinal fluid opening pressure was normal, while its culture grew Cryptococcus neoformans. A diagnosis of cryptococcal meningitis with bilateral optic neuropathy was made. Patient was treated with a six-week course of intravenous flu-conazole and started concomitantly on a fortnight's course of intravenous amphotericin B. After that, his general condition improved, but there was still no improvement in his visual acuity. On reviewing at two months post-initiation of treatment, fundi showed bilateral optic atrophy. Bilateral optic neuropathy secondary to cryptococcal meningitis was rare. The prognosis was guarded due to the sequelae of optic atrophy. Anti-fungal medication alone may not be sufficient to manage this condition. However, evidence for other treatment modalities is still lacking and further clinical studies are required.

  16. Metastatic endometrial stromal sarcoma masquerading as pulmonary lymphangioleiomyomatosis.

    OpenAIRE

    Mahadeva, R; Stewart, S.; Wallwork, J

    1999-01-01

    A 39 year old female presented with bilateral pneumothoraces and interstitial shadowing on chest x ray. A diagnosis of lymphangioleiomyomatosis was made following an open lung biopsy. Over the next eight years she developed respiratory failure leading to single lung transplantation but she died in the immediate postoperative period. Necropsy examination and review of the previous open lung biopsy revealed multiple pulmonary metastases from a low grade endometrial stromal sarcoma of the uterus...

  17. 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.

  18. 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.

  19. 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

  20. Pulmonary Kaposi sarcoma in six children

    Energy Technology Data Exchange (ETDEWEB)

    Theron, Salomine [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); University of Stellenbosch, Department of Radiology, Tygerberg Hospital, Cape Town (South Africa); Andronikou, Savvas; Plessis, Jaco du; George, Reena; Mapukata, Ayanda; Grobbelaar, Marie; Hayes, Murray [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); Goussard, Pierre [University of Stellenbosch, Department of Child Health, Tygerberg Academic Hospital, Cape Town (South Africa); Wieselthaler, Nicky [University of Cape Town, Department of Radiology, Red Cross Children' s Hospital, Cape Town (South Africa); Davidson, Alan [University of Cape Town, Department of Oncology, Red Cross Children' s Hospital, Cape Town (South Africa)

    2007-12-15

    Pulmonary involvement in Kaposi sarcoma is rare in children and can be difficult to distinguish from other pathology. To describe the radiological findings in paediatric pulmonary Kaposi sarcoma. Sequential chest radiographs of six children and CT scans of four of these children were evaluated retrospectively. Their ages ranged from 18 months to 10 years; four were male and two were female. All six children were HIV-positive. The observers were two radiologists. Chest radiographs revealed air-space (100%) and reticular (83%) opacification in the mid- and lower lung zones; pleural effusions were present in 83% of the children. All the children showed progressive air-space opacification on follow-up radiography. CT demonstrated bilateral air-space opacification in a perihilar distribution in all the children; reticular opacification was seen in 75%. All the children had mediastinal and axillary lymphadenopathy; 75% had bilateral hilar lymphadenopathy. In both adults and children, chest radiography demonstrates perihilar and lower zone involvement. Pleural effusions are more common on radiographs in children. Air-space disease and lymphadenopathy are much more common on CT in children than adults. (orig.)

  1. Unilateral renal hypoplasia and contralateral renal agenesis: a new association with 45,X/46,XY mosaicism.

    Science.gov (United States)

    Wax, J R; Prabhakar, G; Giraldez, R A; Hutchins, G M; Stetten, G; Blakemore, K J

    1994-05-01

    The association of urinary anomalies with Turner's syndrome is well established. This report describes an unusual antenatal presentation of this cytogenetic disorder and the first reported case of unilateral renal hypoplasia and contralateral renal agenesis in a 45,X/46,XY fetus. The fetus presented with severe third trimester oligohydramnios and symmetrical intrauterine growth retardation at 29 weeks and 2 days' gestation. Chorionic villus cultures revealed a 45,X karyotype. A phenotypically male infant weighing 1833 g was delivered at 35 weeks and 2 days. Chromosomal analysis of the newborn showed a 45,X/46,XY mosaicism, and surgical exploration revealed absence of the left kidney and a hypoplastic right kidney. The infant died at 11 months of age from renal failure and peritonitis. This case demonstrates that monosomy X may be encountered in fetuses with marked growth delay and oligohydramnios. The etiology of the oligohydramnios in this case was a fetal renal malformation not previously described in Turner's syndrome. Antenatal cytogenetic findings should be confirmed postnatally, with a search for mosaicism, when monosomy X is encountered.

  2. 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.

  3. 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.

  4. Agenesis of the Corpus Callosum Due to Defective Glial Wedge Formation in Lhx2 Mutant Mice.

    Science.gov (United States)

    Chinn, Gregory A; Hirokawa, Karla E; Chuang, Tony M; Urbina, Cecilia; Patel, Fenil; Fong, Jeanette; Funatsu, Nobuo; Monuki, Edwin S

    2015-09-01

    Establishment of the corpus callosum involves coordination between callosal projection neurons and multiple midline structures, including the glial wedge (GW) rostrally and hippocampal commissure caudally. GW defects have been associated with agenesis of the corpus callosum (ACC). Here we show that conditional Lhx2 inactivation in cortical radial glia using Emx1-Cre or Nestin-Cre drivers results in ACC. The ACC phenotype was characterized by aberrant ventrally projecting callosal axons rather than Probst bundles, and was 100% penetrant on 2 different mouse strain backgrounds. Lhx2 inactivation in postmitotic cortical neurons using Nex-Cre mice did not result in ACC, suggesting that the mutant phenotype was not autonomous to the callosal projection neurons. Instead, ACC was associated with an absent hippocampal commissure and a markedly reduced to absent GW. Expression studies demonstrated strong Lhx2 expression in the normal GW and in its radial glial progenitors, with absence of Lhx2 resulting in normal Emx1 and Sox2 expression, but premature exit from the cell cycle based on EdU-Ki67 double labeling. These studies define essential roles for Lhx2 in GW, hippocampal commissure, and corpus callosum formation, and suggest that defects in radial GW progenitors can give rise to ACC.

  5. Genetic and functional analyses identify DISC1 as a novel callosal agenesis candidate gene.

    Science.gov (United States)

    Osbun, Nathan; Li, Jiang; O'Driscoll, Mary C; Strominger, Zoe; Wakahiro, Mari; Rider, Eric; Bukshpun, Polina; Boland, Elena; Spurrell, Cailyn H; Schackwitz, Wendy; Pennacchio, Len A; Dobyns, William B; Black, Graeme C M; Sherr, Elliott H

    2011-08-01

    Agenesis of the corpus callosum (AgCC) is a congenital brain malformation that occurs in approximately 1:1,000-1:6,000 births. Several syndromes associated with AgCC have been traced to single gene mutations; however, the majority of AgCC causes remain unidentified. We investigated a mother and two children who all shared complete AgCC and a chromosomal deletion at 1q42. We fine mapped this deletion and show that it includes Disrupted-in-Schizophrenia 1 (DISC1), a gene implicated in schizophrenia and other psychiatric disorders. Furthermore, we report a de novo chromosomal deletion at 1q42.13 to q44, which includes DISC1, in another individual with AgCC. We resequenced DISC1 in a cohort of 144 well-characterized AgCC individuals and identified 20 sequence changes, of which 4 are rare potentially pathogenic variants. Two of these variants were undetected in 768 control chromosomes. One of these is a splice site mutation at the 5' boundary of exon 11 that dramatically reduces full-length mRNA expression of DISC1, but not of shorter forms. We investigated the developmental expression of mouse DISC1 and find that it is highly expressed in the embryonic corpus callosum at a critical time for callosal formation. Taken together our results suggest a significant role for DISC1 in corpus callosum development.

  6. Clinical prevalence of palmaris longus agenesis: a systematic review and meta-analysis.

    Science.gov (United States)

    Yammine, Kaissar

    2013-09-01

    We report a systematic review and a proportion meta-analysis of prevalence studies evaluating the prevalence of palmaris longus agenesis (PLA) in the literature. The overall PLA rate was defined to be the primary outcome. Secondary outcomes were rates of PLA in relation to ethnicity, laterality, side, gender, age, and hand dominance. We identified 26 articles which met the inclusion criteria. Meta-analyses showed an overall PLA pooled rate of 20.25%, higher than the commonly reported overall rate of 15%. Our results also showed significantly lower pooled rates in Africans (11.3%) and East Asians (4.5%) when compared to Arab Middle Eastern population (41.7%). A subgroup analysis of the African group showed a pooled rate of 2.71%, the lowest, in the East and South East African population. The pooled rate was 26.3% among Caucasians, 26.16% among South and Southeast Asians and 34.13% among Turkish. In discordance with the literature, PLA was statistically more predominant on the right side. No significant differences in PLA rates were found for laterality, gender, the combination of gender and side or the combination of gender and laterality. The lowest rate of PLA found in East and South East African populations might be indicative of the subsequent phylogenetic degeneration of the palmaris longus muscle in modern humans after the "Out of Africa" migration.

  7. Extensive Bilateral Naevus Comedonicus Exacerbating During Pregnancy

    Directory of Open Access Journals (Sweden)

    Rao M.V

    1999-01-01

    Full Text Available Naevus comedonicus is a rare developmental anomaly of the pilosebaceous apparatus. It occurred bilaterally in a 23 year old pregnant woman. She noted exacerbations during two pregnancies, hitherto unreported in the literature.

  8. 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.

  9. 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.

  10. Genetics of Infantile Bilateral Striatal Necrosis

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    J Gordon Millichap

    2006-09-01

    Full Text Available The gene mutation causing autosomal recessive infantile bilateral striatal necrosis (IBSN was identified in eight consanguineous Israeli Bedouin families, in a study at Schneider Children’s Medical Center, Petah Tikva, Israel, and other centers.

  11. Bilateral giant juvenile fibroadenoma of breasts

    Directory of Open Access Journals (Sweden)

    Mukhopadhyay Madhumita

    2009-01-01

    Full Text Available An 11-year-old girl with rapidly enlarging bilateral breast lumps is reported. It was diagnosed as a case of juvenile fibroadenoma following fine needle aspiration cytology and confirmed on histopathological examination of the excised specimens.

  12. BILATERAL TESSIER CLEFT 3: A CASE REPORT

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    Utpal

    2014-06-01

    Full Text Available Tessier cleft 3 is a very rare congenital anomaly, (2 especially the bilateral form. Very few cases have been reported worldwide. (1,2 I report a case of bilateral Tessier cleft 3 presenting at the age of three months with clefts extending from philtral regions, undermining the nasal alar bases to the medial canthal areas bilaterally. There were bilateral complete alveolar clefts with mild protrusion of the pre-maxilla, but the rest of the maxilla including the palate was not involved. Surgical correction was started at the age of three months and completed at the age of one and half years in three stages. There was no intra-operative or postoperative complications and the final result was satisfactory.

  13. 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”.

  14. 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.)

  15. 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.

  16. Clinical subgroups in bilateral Meniere disease

    OpenAIRE

    2016-01-01

    Meniere disease (MD) is a heterogeneous clinical condition characterized by sensorineural hearing loss, episodic vestibular symptoms and tinnitus associated with several comorbidities such as migraine or autoimmune disorders (AD). The frequency of bilateral involvement may range from 5-50% and it depends on the duration of the disease. We have performed a two-step cluster analysis in 398 patients with bilateral MD to identify the best predictors to define clinical subgroups with a potential d...

  17. 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.

  18. 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

  19. Bilateral Keratectasia 34 Years after Corneal Transplant

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    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.

  20. 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.

  1. 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.

  2. Bilateral microvascular second toe transfer for bilateral post-traumatic thumb amputation.

    Science.gov (United States)

    Nehete, Rajendra; Nehete, Anita; Singla, Sandeep; Adhav, Harshad

    2012-01-01

    In bilateral thumb amputations, the functional impairment is serious and every attempt should be made to reconstruct the thumb. We report a case of bilateral post traumatic thumb amputation, reconstructed with bilateral second toe transfer. Only two such cases have been reported in literature so far. Though there are various modalities for the reconstruction of thumb, microvascular toe transfer has its own merits. The convalescent period is minimal with excellent function. It is bilaterally symmetric and aesthetically superior to the osteoplastic reconstruction. The technical details are discussed, and the long term functional and aesthetic results are presented.

  3. Bilateral microvascular second toe transfer for bilateral post-traumatic thumb amputation

    Directory of Open Access Journals (Sweden)

    Rajendra Nehete

    2012-01-01

    Full Text Available In bilateral thumb amputations, the functional impairment is serious and every attempt should be made to reconstruct the thumb. We report a case of bilateral post traumatic thumb amputation, reconstructed with bilateral second toe transfer. Only two such cases have been reported in literature so far. Though there are various modalities for the reconstruction of thumb, microvascular toe transfer has its own merits. The convalescent period is minimal with excellent function. It is bilaterally symmetric and aesthetically superior to the osteoplastic reconstruction. The technical details are discussed, and the long term functional and aesthetic results are presented.

  4. 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).

  5. 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

  6. 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

  7. 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.

  8. 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.

  9. 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.

  10. Hantavirus Pulmonary Syndrome in Manitoba

    Directory of Open Access Journals (Sweden)

    L Robbin Lindsay

    2001-01-01

    Full Text Available The first confirmed case of hantavirus pulmonary syndrome in Manitoba was diagnosed in 1999. To define better the risk of exposure to hantaviruses in this area, the clinical features and epidemiological factors pertaining to this case were described, and a serological survey of rodents collected near the patient's residence was undertaken. Small mammals were collected using live traps, were anesthetized via inhalation of isoflurane and were bled. Human and mouse serologies were undertaken using an ELISA to detect hantavirus-specific immunoglobulin G and/or immunoglobulin M antibodies. In addition, a full medical and epidemiological assessment, as well as individual risk factor and exposure analysis, were conducted. A 27-year-old Manitoba woman presented with severe respiratory distress and diffuse bilateral air space disease radiologically. Despite extremely aggressive measures, including mechanical ventilation, antibiotics, fluid management and inotropic support, the patient's condition rapidly deteriorated, and she died 8 h after admission. Hantavirus pulmonary syndrome was confirmed by the detection of immunoglobulin M and immunoglobulin G antibodies to the Sin Nombre virus (SNV in her sera and by the demonstration of SNV genomic sequences in her lung tissue. Exposure to hantavirus likely occurred in and around the home or in the rural area in which she resided. A total of 252 small mammals, primarily deer mice (Peromyscus maniculatus, were collected from 17 different sites at or near where the patient lived. Antibodies to SNV were detected in 28 of 244 (11.5% deer mice, which were collected within 9 km of the residence of the fatal case, indicating that these rodents are a significant reservoir for SNV in this area.

  11. Discordant tooth agenesis and peg-shaped in a pair of monozygotic twins: Clinical and molecular study.

    Science.gov (United States)

    Antunes, Lívia Azeredo Alves; Küchler, Erika Calvano; Costa, Marcelo de Castro; Antunes, Leonardo Santos; Granjeiro, José Mauro

    2013-11-01

    The present report aimed to study an uncommon case of a pair of twins that presented a discordant dental phenotype. Family investigation, clinical, radiographic examination and molecular analysis were performed in both girls. Molecular analysis confirmed the monozygosity by deoxyribonucleic acid chip technology. One twin presented tooth agenesis in left upper lateral incisor and peg-shaped on the contra lateral side while the other twin had no dental alterations. The dental casts study employed digital caliper to compare morphological dimensions and showed alteration only in peg-shaped tooth. In conclusion, this study provide support that one or more mutated gene could cause discordances in dental phenotype in these monozygotic twins.

  12. The Syndrome of Frontonasal Dysplasia, Callosal Agenesis, Basal Encephalocele, and Eye Anomalies – Phenotypic and Aetiological Considerations

    Directory of Open Access Journals (Sweden)

    2004-03-01

    Full Text Available We report ten sporadic cases of Brazilian patients with facial midline defects, callosal agenesis, basal encephalocele, and ocular anomalies. This very rare cluster of anomalies has been well reported before. However, only until recently it is recognized as a syndrome belonging to frontonasal dysplasia spectrum. The ten cases confirm a distinct clinical entity and help to define the phenotype more precisely than previously. Up to now etiology remains unknown, although we conjecture that it is due to a mutation in TGIF gene.

  13. Frontonasal dysplasia, callosal agenesis, basal encephalocele, and eye anomalies syndrome with a partial 21q22.3 deletion.

    Science.gov (United States)

    Guion-Almeida, Maria Leine; Richieri-Costa, Antonio; Jehee, Fernanda Sarquis; Passos-Bueno, Maria Rita Santos; Zechi-Ceide, Roseli Maria

    2012-07-01

    We describe a girl with a phenotype characterized by frontonasal dysplasia, callosal agenesis, basal encephalocele, and eye anomalies who presents a 46,XX,r(21) karyotype. Array-comparative genomic hybridization using the Afflymetrix 100K DNA oligoarray set showed an interstitial deletion 21q22.3 of approximately 219 kb. Conventional karyotype of both parents was normal, and it was not possible to perform the molecular studies. In this report we raise the hypothesis that the deleted genes located at 21q22.3 could account to the phenotype.

  14. Unilateral Congenital Lacrimal Fistula with Renal Agenesis and Pelvic Kidney: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    A. Altun

    2015-01-01

    Full Text Available A 12-year-old boy presented to the clinic of ophthalmology because of watering and discharge from his left lower eyelid. The inspection examination revealed an orifice that was associated with congenital lacrimal fistula (CLF. He underwent a complete ophthalmologic and systemic evaluation to explore possible associated findings. Systemic evaluation revealed multiple renal anomalies: right renal agenesis and left ectopic pelvic kidney. This case is unique because this is the first reported case of CLF accompanied with ectopic pelvic kidney in the literature.

  15. Unilateral Congenital Lacrimal Fistula with Renal Agenesis and Pelvic Kidney: A Case Report and Review of the Literature.

    Science.gov (United States)

    Altun, A; Kurna, S A; Sengor, T; Altun, G; Oflaz, A; Sonmez, H S

    2015-01-01

    A 12-year-old boy presented to the clinic of ophthalmology because of watering and discharge from his left lower eyelid. The inspection examination revealed an orifice that was associated with congenital lacrimal fistula (CLF). He underwent a complete ophthalmologic and systemic evaluation to explore possible associated findings. Systemic evaluation revealed multiple renal anomalies: right renal agenesis and left ectopic pelvic kidney. This case is unique because this is the first reported case of CLF accompanied with ectopic pelvic kidney in the literature.

  16. 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

  17. Pulmonary fungal infections after bone marrow transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Allan, B.T.; Patton, D.; Ramsey, N.K.C.; Day, D.L.

    1988-02-01

    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.

  18. 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.

  19. 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

  20. 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 ...