Cases reported "esophageal atresia"

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1/343. A strategy for primary reconstruction of long gap esophageal atresia using neonatal colon esophagoplasty: a case report.

    Treatment options for long gap esophageal atresia without tracheoesophageal fistula generally require several stages over many months. An early neonatal vascularized conduit would allow a tension-free anastomosis, but the precarious blood supply of the neonatal bowel makes mobilization and immediate interposition hazardous. This report describes the successful application of a strategy for primary reconstruction in the neonate using a short piece of colon mobilized into the mediastinum for subsequent delayed anastomosis. ( info)

2/343. esophageal atresia and tracheoesophageal fistula.

    esophageal atresia, with or without tracheoesophageal fistula, is a fairly common congenital disorder that family physicians should consider in the differential diagnosis of a neonate who develops feeding difficulties and respiratory distress in the first few days of life. esophageal atresia is often associated with other congenital anomalies, most commonly cardiac abnormalities such as ventricular septal defect, patent ductus arteriosus or tetralogy of fallot. Prompt recognition, appropriate clinical management to prevent aspiration, and swift referral to an appropriate tertiary care center have resulted in a significant improvement in the rates of morbidity and mortality in these infants over the past 50 years. ( info)

3/343. Clinical spectrum of infantile free sialic acid storage disease.

    Infantile free sialic acid storage disease (ISSD) is a rare autosomal recessive metabolic disorder caused by a lysosomal membrane transport defect, resulting in accumulation of free sialic acid within lysosomes. Only a few cases have been described. We report on three new cases of ISSD with different modes of presentation: an infant with nephrotic syndrome, a case of fetal and neonatal ascites with heart failure, and a case of fetal ascites with esophageal atresia type III. From these patients and a review of the literature (27 cases total) we draw the following conclusions. 1) "Coarse facies," fair complexion, hepatosplenomegaly, and severe psychomotor retardation are constant findings in this disorder. 2) nephrotic syndrome occurred in most cases (four in seven) in which renal evaluation was performed. Therefore, ISSD is an important cause of nephrosis in infants with a storage disorder phenotype. 3) Fetal/neonatal ascites or hydrops was the mode of presentation in 13 (60%) of 21 cases. Thus, ISSD enters in the differential diagnosis of hydrops fetalis with a storage disease phenotype. 4) cardiomegaly was evident in nine cases. 5) Corneae were always clear, and albinoid fundi were reported in five cases. 6) Dysostosis multiplex was not prominent. 7) bone marrow aspiration could be negative. 8) death ensued in early infancy with a mean age of 13.1 months. All reported deaths were caused by respiratory infections. ( info)

4/343. methimazole embryopathy: delineation of the phenotype.

    We report on a further case of congenital anomalies in a child exposed to methimazole during the first trimester of pregnancy (from first to seventh gestational week), and define a specific malformation pattern related to prenatal methimazole exposure and consisting of choanal and esophageal atresia, scalp defects, minor facial anomalies and psychomotor delay. ( info)

5/343. Successful treatment of tracheomalacia associated with esophageal atresia without a tracheoesophageal fistula by aortopexy: report of a case.

    tracheomalacia (TM) is well known as a complication associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF); however, the occurrence of TM requiring surgical treatment in a patient having EA without a tracheoesophageal fistula has never been reported. We describe herein a rare case of TM associated with EA without TEF. Respiratory distress was caused by compression of the trachea by a severely dilated upper esophageal pouch with weakness of the tracheal wall. Aortopexy was performed, and an excellent postoperative result was achieved. ( info)

6/343. Esophageal lung with multiple congenital anomalies: conundrums in diagnosis and management.

    BACKGROUND/PURPOSE: Communicating bronchopulmonary foregut malformations (CBPFM) are a diverse group of potentially devastating congenital anomalies with anatomy that may be difficult to delineate. The authors present a case that illustrates conundrums in the diagnosis and management of these complex disorders. methods: A term baby had esophageal atresia (EA), tracheoesophageal fistula (TEF), and tetralogy of fallot. Initially, a gastrostomy was performed, and a balloon catheter was inserted through the endotracheal tube to occlude the fistula until the patient was hemodynamically stable. Subsequently, the fistula was ligated. Postoperatively, the left lung collapsed, and bronchoscopy showed an atretic left mainstem bronchus. Repeat thoracotomy showed that the fistula ligation was intact. air was introduced through the gastrostomy tube, and, surprisingly, the left lung inflated, indicating the left mainstem bronchus arose from the esophagus distal to the ligated TEF. RESULTS: Despite reopening this fistula, ventilation remained poor, and support was withdrawn. autopsy findings confirmed a unilobed left lung arising from the esophagus, EA, TEF, an atretic left mainstem bronchus, tetralogy of fallot, and digeorge syndrome. CONCLUSIONS: This is the first report of a combination of EA and distal TEF with a second CBPFM involving the esophagus and the entire left lung. Successful correction of these anomalies will require extensive delineation of the anatomy to plan an operative strategy. ( info)

7/343. Fibroelastosis of the posterior urethra associated with urinary, cardiac and digestive anomalies.

    A case of fibroelastosis of the posterior urethra associated with ectopic opening of the ureter of a solitary kidney in the urethra is described. Oesophageal atresia and anomalous origin of the left coronary artery were also observed. Management of fibroelastosis is pointed out. ( info)

8/343. Congenital cystic adenomatoid malformation of the lung associated with esophageal atresia and tracheoesophageal fistula.

    Bronchopulmonary malformations associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) are extremely rare. The authors describe a case of type II congenital cystic adenomatoid malformation (CCAM) of the right lower lobe associated with EA and TEF (Vogt-Gross type C) in a full-term female infant. The CCAM presented as an incidental radiologic finding, and a contralateral tension pneumothorax developed shortly after surgical repair of the EA. Early recognition of this rare association is essential for correct operative management. ( info)

9/343. Novel use of neonatal cuffed tracheal tube to occlude tracheo-oesophageal fistula.

    The use of a cuffed tracheal tube is described to occlude the leak through a tracheo-oesophageal fistula (TOF) in a neonate and prevent gastric dilatation during positive-pressure lung ventilation. ( info)

10/343. Primary anastomosis in esophageal atresia type I without a gap.

    This paper reports the case of an infant born with type I esophageal atresia (EA) associated with duodenal atresia (DA). The critical condition of the patient necessitated an exploratory laparotomy, which revealed severe dilatation of the stomach and duodenum. The routine procedure for repairing type I EA is a delayed primary anastomosis after 10 weeks of age because of the long gap between the two esophageal segments. In our case, due to the concomitant DA, the lower pouch was long enough to allow primary neonatal anastomosis. A radiograph taken with a Hegar dilator in the lower segment via the gastrostomy confirmed this suspicion, and the baby underwent a thoracotomy and primary anastomosis between the esophageal pouches. The authors propose the possibility of primary esophageal anastomosis in similar cases. ( info)
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