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Congential bilateral absence of diaphragm
INTERNATIONAL ABSTRACTS
with abnormal chest radiographs due to BPD.--Randall W. Powell
207 Middle Aortic Syndrome as a Cause of Heart Failure in Children and Its Management. S. Gupta, B. Goswami, D. C.
Ghosh, et al. Thorax 36:63-65, (January), 1981. Congenital Diaphragmatic Hernia. Intrauterine Repair in Fetal Sheep. K. J. Hardy, A. W. Auldist, and A. Shulkes.
Med J Aust 2:223-225, 1982. A congenital diaphragmatic hernia was created surgically in three sheep fetuses in midgestation. After allowing at least 1 mo for interference with lung development, an inutero repair was performed 3 wk before delivery. The study indicates that the interauterine repair of congenital diaphragmatic hernia would be a relatively easy operation. However, the risks to the human mother need to be assessed.--N. A. Myers Congential Bilateral Absence of Diaphragm. N. Toran and J.
L. Emery. Thorax 36:157 158, (February), 1981.
This necroscopy report on an infant surviving for 20 min after full-term normal delivery describes the very rare occurrence of bilateral absence of the diaphragm.~J. F. R. Bentley HEART A N D GREAT VESSELS Congenitally Corrected Transposition of the Great Arteries with Tricuspid Incompetence: Successful Treatment by Xenograft Valve Replacement in a 15-too-old Child. A. Ross
and C. Lincoln. Thorax 36:217-218, (March), 1981.
Symptomatic tricuspid valve incompetence is seldom the sole associated lesion with congenitally corrected transposition of the great vessels. Xenograft valve replacement in these circumstances has not been described previously.--J. F. R. Bentley Transatrial Repair of Double Outlet Right Ventricle. /9. A.
Goor, C. Massini, A. Shem-tor, et al. Thorax 37:371-375, (May), 1982.
The repair of a double outlet right ventricle is usually accomplished through a ventriculotomy. In the presence of a perimembraneous VSD, a small crista supraventricularis, and in the absence of infundibular pulmonary stenosis, as determined by angiocardiography, the correction may be undertaken using transatrial access. Excellent results are reported in three infants, one having side-by-side great arteries.--J. F. R. Bentley Successful Correction of a Subendocardial Hamartoma of the Left Ventricular Free Wall and Mitral Valve Papillary Muscles. A. P. Rocchini, W. Brott, M. Robinowitz, et al.
Chest 80:226-228, (August), 1981. A 4V2-yr-old male presented with repeated pneumonias and severe congestive failure. Two previous cardiac catheterizations led to diagnoses of mitral stenosis at age 5 mo and left ventricular endocardial fibroelastosis at 1~/2 yr. An echocardiogram revealed a mass in the left ventricle, and at surgery a hamartoma, which divided the left ventricle into two chambers with marked decreased capacity, was resected. Echocardiograms and cardiac catheterization 3 yr later revealed normal ventricular function with no evidence of recurrence.--Randall W. Powell
Middle aortic syndrome is an acquired coarctation of the descending aorta due to Takayasu's disease. It usually occurs in young adults. The occurenee of the syndrome in two girls from Calcutta is reported. One, aged 11 yr, died without surgical intervention; the other, aged 9 yr, remained well 8 mo after the narrowing was relieved by an onlay Dacron patch.--J. F. R. Bentley Dissecting Aortic Aneurysm in Childhood and Adolescence: Case Report and Literature Review. C. R. Fikar, J. A.
Amrhein, J. P. Harris, et al. Clin Pediatr 20:578-583, (September), 1981.
A 15-yr-old male presented with severe left-sided abdominal and back pain. While being evaluated in an emergency room he arrested and could not be resusitated. Post-mortem examination revealed an aortic dissection (origin 5 cm distal to left subclavian) with rupture into the pleural cavity. Histologic examination revealed cystic medial necrosis. Aortic dissections in the pediatric age group are rare (7 of 505 and 31 of 580 patients <20 yr of age) and usuaUy are associated with congenital cardiovascular anomalies (coarctation, bicuspid aortic valves, or aortic stenosis), connective tissue disorders (Marfan's, Ehlers-Danlos, familial cystic medial necrosis, and Turner's syndrome), pregnancy, inflammatory diseases, (Takayasu's arteritis and rheumatic fever), cystinosis (one patient with associated hypertension and renal failure), and trauma. Sudden severe chest or back pain occurs in 85%-95% of adult patients and this pain is migratory in 60%-80%. Neurologic findings occur in 15%-45% of adult patients. The clinical history in association with the aboveassociated factors suggests the diagnosis. Patient salvage requires prompt surgical correction.--Randall I4I. Powell ALIMENTARY TRACT Congenital Oesophageal Stenosis Caused by Tracheobronchial Structures in the Oesophageal Wall. N. B. N./braham
and R. J. Sandy. Thorax 36:465-468, (June), 1981.
Successful reaction of narrowing of the lower esophagus due to tracheobronchial structures in the wall in two infants is reported and the 16 cases previously documented are reviewed. The first was a male infant aged 6 mo at the time of operation. The second was a female with multiple anomalies of the alimentary tract. Oesophageal atresia and tracheooesophageal fistula was corrected after birth, a duplication of the duodenum required operative treatment at 18 mo, and the lower oesophagus was resected at 6 yr, when a duplication of the stomach was also recognized.--J. F. R. Bentley Paralysis of the Right Hernidiaphragm Following Primary Anastomosis for Esophageal Atresia and Tracheo-Esophagaal Fistula. D. Man, M. H. Wheildon and 14. B. Eckstein. Z
Kinderchir 37:32-33, 1982. Dissection of a tracheo-esophageal fistula and tensionless primary anastomosis of the esophagus was performed in a 1500 g female infant. The baby required intermittent positive pressure ventilation (IPPV) postoperatively. When an
with abnormal chest radiographs due to BPD.--Randall W. Powell
207 Middle Aortic Syndrome as a Cause of Heart Failure in Children and Its Management. S. Gupta, B. Goswami, D. C.
Ghosh, et al. Thorax 36:63-65, (January), 1981. Congenital Diaphragmatic Hernia. Intrauterine Repair in Fetal Sheep. K. J. Hardy, A. W. Auldist, and A. Shulkes.
Med J Aust 2:223-225, 1982. A congenital diaphragmatic hernia was created surgically in three sheep fetuses in midgestation. After allowing at least 1 mo for interference with lung development, an inutero repair was performed 3 wk before delivery. The study indicates that the interauterine repair of congenital diaphragmatic hernia would be a relatively easy operation. However, the risks to the human mother need to be assessed.--N. A. Myers Congential Bilateral Absence of Diaphragm. N. Toran and J.
L. Emery. Thorax 36:157 158, (February), 1981.
This necroscopy report on an infant surviving for 20 min after full-term normal delivery describes the very rare occurrence of bilateral absence of the diaphragm.~J. F. R. Bentley HEART A N D GREAT VESSELS Congenitally Corrected Transposition of the Great Arteries with Tricuspid Incompetence: Successful Treatment by Xenograft Valve Replacement in a 15-too-old Child. A. Ross
and C. Lincoln. Thorax 36:217-218, (March), 1981.
Symptomatic tricuspid valve incompetence is seldom the sole associated lesion with congenitally corrected transposition of the great vessels. Xenograft valve replacement in these circumstances has not been described previously.--J. F. R. Bentley Transatrial Repair of Double Outlet Right Ventricle. /9. A.
Goor, C. Massini, A. Shem-tor, et al. Thorax 37:371-375, (May), 1982.
The repair of a double outlet right ventricle is usually accomplished through a ventriculotomy. In the presence of a perimembraneous VSD, a small crista supraventricularis, and in the absence of infundibular pulmonary stenosis, as determined by angiocardiography, the correction may be undertaken using transatrial access. Excellent results are reported in three infants, one having side-by-side great arteries.--J. F. R. Bentley Successful Correction of a Subendocardial Hamartoma of the Left Ventricular Free Wall and Mitral Valve Papillary Muscles. A. P. Rocchini, W. Brott, M. Robinowitz, et al.
Chest 80:226-228, (August), 1981. A 4V2-yr-old male presented with repeated pneumonias and severe congestive failure. Two previous cardiac catheterizations led to diagnoses of mitral stenosis at age 5 mo and left ventricular endocardial fibroelastosis at 1~/2 yr. An echocardiogram revealed a mass in the left ventricle, and at surgery a hamartoma, which divided the left ventricle into two chambers with marked decreased capacity, was resected. Echocardiograms and cardiac catheterization 3 yr later revealed normal ventricular function with no evidence of recurrence.--Randall W. Powell
Middle aortic syndrome is an acquired coarctation of the descending aorta due to Takayasu's disease. It usually occurs in young adults. The occurenee of the syndrome in two girls from Calcutta is reported. One, aged 11 yr, died without surgical intervention; the other, aged 9 yr, remained well 8 mo after the narrowing was relieved by an onlay Dacron patch.--J. F. R. Bentley Dissecting Aortic Aneurysm in Childhood and Adolescence: Case Report and Literature Review. C. R. Fikar, J. A.
Amrhein, J. P. Harris, et al. Clin Pediatr 20:578-583, (September), 1981.
A 15-yr-old male presented with severe left-sided abdominal and back pain. While being evaluated in an emergency room he arrested and could not be resusitated. Post-mortem examination revealed an aortic dissection (origin 5 cm distal to left subclavian) with rupture into the pleural cavity. Histologic examination revealed cystic medial necrosis. Aortic dissections in the pediatric age group are rare (7 of 505 and 31 of 580 patients <20 yr of age) and usuaUy are associated with congenital cardiovascular anomalies (coarctation, bicuspid aortic valves, or aortic stenosis), connective tissue disorders (Marfan's, Ehlers-Danlos, familial cystic medial necrosis, and Turner's syndrome), pregnancy, inflammatory diseases, (Takayasu's arteritis and rheumatic fever), cystinosis (one patient with associated hypertension and renal failure), and trauma. Sudden severe chest or back pain occurs in 85%-95% of adult patients and this pain is migratory in 60%-80%. Neurologic findings occur in 15%-45% of adult patients. The clinical history in association with the aboveassociated factors suggests the diagnosis. Patient salvage requires prompt surgical correction.--Randall I4I. Powell ALIMENTARY TRACT Congenital Oesophageal Stenosis Caused by Tracheobronchial Structures in the Oesophageal Wall. N. B. N./braham
and R. J. Sandy. Thorax 36:465-468, (June), 1981.
Successful reaction of narrowing of the lower esophagus due to tracheobronchial structures in the wall in two infants is reported and the 16 cases previously documented are reviewed. The first was a male infant aged 6 mo at the time of operation. The second was a female with multiple anomalies of the alimentary tract. Oesophageal atresia and tracheooesophageal fistula was corrected after birth, a duplication of the duodenum required operative treatment at 18 mo, and the lower oesophagus was resected at 6 yr, when a duplication of the stomach was also recognized.--J. F. R. Bentley Paralysis of the Right Hernidiaphragm Following Primary Anastomosis for Esophageal Atresia and Tracheo-Esophagaal Fistula. D. Man, M. H. Wheildon and 14. B. Eckstein. Z
Kinderchir 37:32-33, 1982. Dissection of a tracheo-esophageal fistula and tensionless primary anastomosis of the esophagus was performed in a 1500 g female infant. The baby required intermittent positive pressure ventilation (IPPV) postoperatively. When an