EXTROPHY OF THE CLOACA: REPORT OF A CASE TREATED SURGICALLY.
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PURPOSE: The aim of this study was the assessment of atresia formation after syngeneic fetal small bowel transplantation (SBTx) to clarify its pathogenesis. METHODS: Seventy Lewis rat fetuses (gestational age, 18 to 19 days) were obtained by hysterotomy, and a 30-mm long section of small bowel was excised from each fetus. Each bowel graft was then transplanted into the space between the peritoneum and the rectus abdominis in 70 adult Lewis rats to expose the grafts to ischemic stress. Transplantation was successful in 63 of 70 grafts (90%). Successfully transplanted bowel grafts were harvested for macroscopic and microscopic examination 10 days posttransplantation. RESULTS: Of the successfully transplanted grafts, only two (3%) were atresia free; 127 atretic segments were found in the remaining 61 grafts. Twenty-four grafts (38%) had a single atresia comprised of membranous stenosis (MS) in two, membranous atresia (MA) in 10, and blind ends (BEs) with or without a connecting tissue remnant in 12. Thirty-seven grafts (59%) had multiple atresias, comprised of MS, MA, or both in six, BEs alone in seven, and a combination of BEs with MS or MA in 24. CONCLUSIONS: Our model is the first to succeed in inducing experimentally membranous stenosis and a high incidence (59%) of multiple atresias. These results suggest that bowel ischemia is responsible for multiple bowel atresia formation.
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Two siblings with ileal atresia are described; one boy presented with ileal perforation and the other with meconium peritonitis, diagnosed by antenatal ultrasonography. The site and type of atresia, complications and associated anomalies of reported cases among siblings also are discussed.
We present a case of gastroschisis that was associated with progressive resorption of the extra-abdominal bowel loops and dilation of intra-abdominal bowel loops. After preterm delivery at 32 weeks, a small paraumbilical remnant was present. There was complete atresia of most of the jejunum, ileum, cecum, and the proximal half of the transverse colon. At laparotomy, the jejunum was anastomosed to the transverse colon. The neonate developed short gut syndrome and eventually received a liver and intestinal transplant.
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Amniotic fluid samples from four fetuses with intestinal obstructions (two anal imperforations, one duodenal atresia and one multiple intestinal atresia) were deficient in disaccharidase activities. These results indicate that disaccharidase assay in amniotic fluid can be used as a rapid and simple test for the prenatal detection of fetal intestinal obstructions.
Forty-six consecutive patients with biliary atresia were operated on at our institution during the 11-year period between 1978 and 1989. Their ages at operation ranged from 18 to 153 days (mean, 59 days). After dissecting the porta hepatis structures by Kasai operation, a biliointestinal anastomosis was constructed with a valved hepatic portoduodenal intestinal conduit in all cases. The intestinal valve is an intussuscepted muscular valve. Valvular function was examined radiologically. The upper gastrointestinal series demonstrated no reflux of contrast material into the conduit proximal to the valve and liver scintigraphy demonstrated that radioactive isotope drained readily into the duodenum through the valve. Thirty-nine of the forty-six patients (85%) had bile drainage after initial operation. At present 30 patients (65%) are alive without jaundice, 6 (13%) are alive with jaundice, and 10 (22%) are dead. The 5-year jaundice-free survival rate was 64%. Cholangitis occurred in 9 of 39 patients (23%) who had obtained apparent bile drainage: 5 had tractable cholangitis and 4 had refractory cholangitis. Reoperation restored bile flow in 2 of 8 patients who abruptly ceased to have bile drainage without cholangitis. In conclusion, with a valved hepatic portoduodenal intestinal conduit, the incidence of cholangitis was decreased, its medical control became easier, and the survival rate was improved.
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From 1969 to 1981 duodenal atresia was found in 21 patients and jejuno-ileal atresia in 15 patients. Associated anomalies were revealed in 14 neonates with duodenal atresia and in 2 with jejuno-ileal atresia. Vomiting was the predominant symptom in all patients with ileal atresia except one. Abdominal distension was seen in 2 cases with duodenal atresia and in 11 with jejuno-ileal atresia. The primary surgical procedure was a duodeno-jejunostomy in case of duodenal atresia, and bowel resection with end-to-end anastomosis in jejuno-ileal atresia. Of the 36 patients (8%) died. The cause of death was respiratory insufficiency in one child with duodenal atresia and one with ileal atresia. Another child with ileal atresia died of cardiac malformation. Anastomotic stricture was the most common postoperative complication and was seen in 3 cases with duodenal atresia and in 4 with ileal atresia. As jejuno-ileal atresia is caused by a vascular accident ample resection of the blind ends is recommended.
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