Lethal autosomal recessive syndrome with intrauterine growth retardation, intra- and extrahepatic biliary atresia, and esophageal and duodenal atresia.
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Polysplenia is a congenital disorder affecting the asymmetric organs, including the heart, lungs, bronchi, liver, stomach, pancreas, intestines, and spleen. Primary manifestations are heterogeneous and can include congenital heart disease, atypical placement of major abdominal organs, biliary atresia, and intestinal malrotation. Knowledge of these congenital abnormalities will enhance patient care. In this report, we present an elderly, asymptomatic individual with polysplenia to highlight the cardinal features and management issues.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
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BACKGROUND/PURPOSE: Disturbed intestinal transit (DIT) associated with bowel dilation occurs in some postoperative patients with jejunal atresia. Bowel plication (BP) has been introduced to prevent the DIT, but the long-term results of BP are unclear. METHODS: The authors reviewed the preoperative and operative records and postoperative clinical courses (for 2.3 to 7.0 years; mean period, 3.7 years) of 19 jejunal atresia patients, 4 of whom had undergone additional BP with primary anastomosis after dilated bowel resection or tapering jejunoplasty at neonatal surgery. The degree of DIT was evaluated by the clinical symptoms, weight gain, whether reoperation was performed, and duration from the operation to receiving an oral feeding volume of at least 130 mL/kg/d. The patients then were assigned 4 grades (0 to 3). To determine the presence or absence of bowel dilation at the BP site, the plain abdominal x-rays were reviewed. RESULTS: (1) Within 2 months after surgery, 4 patients without BP underwent operation because of severe DIT. The degree of postoperative DIT in patients who had received additional BP at neonatal surgery was less than that in patients without BP (mean grade, 0.50 v. 2.08). (2) The preoperative clinical features, operative method, and postoperative weight gain were almost similar in patients with and without BP. (3) On abdominal x-ray the bowel dilation remained 6 to 12 months after the operation, but was not observed over 1 year after the operation. CONCLUSIONS: In this preliminary study, the addition of BP after tapering jejunoplasty or resection of dilated bowel may be effective in preventing early postoperative DIT. Further study is necessary to evaluate the long-term results of additional BP at neonatal surgery. J Pediatr Surg 36:1752-1756.
A case of low atresia of the ileum, diagnosed prenatally by real-time ultrasound scanning, is presented. The ultrasound examination showed progressive distension of intestinal loops, with strong peristaltic movements. The stomach was also distended, presenting as a large cystic area in the upper left abdomen. Real-time ultrasound technique is most advantageous in the diagnosis of fetal ileus. Prenatal diagnosis of fetal intestinal obstruction is of great importance, making early and safe treatment of the newborn possible.
An infant with the rare association of colon atresia, total colonic Hirschsprung's disease, and tetralogy of Fallot is presented. While Hirschsprung's disease associated with atresia of the colon has been reported five times previously, this is the first case with associated congenital cardiac disease. As in previous reports, initial operations to relieve obstruction due to the colon atresia failed because of the unrecognized aganglionosis. This infant initially had an ileostomy performed as a newborn and then two failed attempts at closure of the ileostomy. In this case, as in four previously reported cases, histological examinations of resected colon from the initial operations were first reported as normal and only under the pressure of increasing clinical evidence was the pathology reviewed and the absence of ganglion cells appreciated. We stress the importance of suspecting the coexistence of Hirschsprung's disease and colon atresia when a functional obstruction is present after repair of the atresia.