11/91. Multiple gastrointestinal atresias, with intraluminal calcifications and cystic dilatation of bile ducts: a newly recognized entity resemblin "a string of pearls".The siblings reported here represent a new and complex congential anomaly. A genetic basis is strongly suggested. The natural preparation of closed loop duodenal obstruction and cystic dilatation of the bile ducts is a unique finding. The hallmark of this new entity--intraluminal calcifications strung out through small and large intestine like pearls--is a striking radiographic feature and, in the proper clinical setting, pathognomonic. This entity has been uniformly lethal. Once the diagnosis is confirmed, no known therapy is useful.- - - - - - - - - - ranking = 1keywords = obstruction, bile duct, bile, duct (Clic here for more details about this article) |
12/91. Fetal small bowel obstruction: report of two cases.Fetal small bowel obstruction is usually diagnosed by sonography in the late second or early third trimester. We report two such cases of different etiology: a case of meconium ileus due to cystic fibrosis and a case of intestinal atresia. The only sonographic finding that allowed differentiation between them was the echogenic bowel in the case of cystic fibrosis.- - - - - - - - - - ranking = 2.3711915390932keywords = obstruction (Clic here for more details about this article) |
13/91. Simultaneous correction of biliary atresia and small bowel atresia in the neonate.The coexistence of biliary atresia and small bowel atresia has been described. However, attempts to correct the biliary atresia at the same time as small bowel atresia repair have not been detailed. In 1986, a 2,170-g female was delivered at 36 weeks' gestation. Postpartum, duodenal obstruction was suspected after oral feedings were not tolerated. At 37 hours after birth, laparotomy showed unsuspected biliary atresia in addition to intestinal malrotation, duodenal web, and type IIIb jejunal atresia with an apple-peel configuration of the upper jejunum and mesentery supplied by the superior mesenteric artery with normal small bowel length and normal ileal mesentery. A modified Roux-en-Y hepatic portoenterostomy was performed using the blind end of the apple-peel configuration of jejunum, with a side-to-side duodenojejunostomy 25 cm from the portoenterostomy. Microscopic examination of the fibrous extrahepatic biliary tissue showed a disarray of small bile ductules. The maximal ductal size at the portoenterostomy site was 600 microns. A liver biopsy specimen showed normal architecture with eosinophils infiltrating the portal triads and periductal areas. Postoperatively, she has maintained normal growth and development with no episodes of cholangitis. liver biopsies at 1 year and 3 years were normal. At 4 years, total and conjugated bilirubin, serum proteins, liver enzymes, coagulation factors, and trace elements were normal. We conclude that simultaneous correction of biliary atresia and small bowel atresia can be accomplished in the neonate with excellent early results.- - - - - - - - - - ranking = 8.6596296228442keywords = extrahepatic, obstruction, bile duct, bile, duct (Clic here for more details about this article) |
14/91. Ileal atresia associated with a congenital vascular band anomaly: observations on pathogenesis.We report the case of a newborn, who developed intestinal obstruction soon after birth. Exploratory laparotomy revealed a congenital vascular band anomaly extending from the antimesenteric border of the terminal ileum to the gallbladder in association with ileal atresia. Surgical intervention was performed for correction of the disorder. A review of the embryology and congenital vascular bands is presented together with discussion as to possible etiopathogenesis leading to small bowel atresia.- - - - - - - - - - ranking = 0.47423830781864keywords = obstruction (Clic here for more details about this article) |
15/91. Double atresia of hindgut with ileal stenosis: a case report.An unusual case of imperforate anus with sigmoid colonic atresia and ileal stenosis is described. Only six cases of imperforate anus associated with colonic atresia have previously been reported in japan. The possibility of other alimentary obstructions should thus be considered when anorectal anomalies are observed.- - - - - - - - - - ranking = 0.47423830781864keywords = obstruction (Clic here for more details about this article) |
16/91. prenatal diagnosis of intestinal obstruction due to ileal atresia.A case of intestinal obstruction due to ileal atresia where the diagnosis was made prenatally by ultrasound is presented. Close monitoring of the fetus was done ultrasonographically to look for any evidence of meconium peritonitis. The baby was delivered preterm but weighed 3.3 kg. laparotomy and enterostomy was done and the baby is currently well.- - - - - - - - - - ranking = 2.3711915390932keywords = obstruction (Clic here for more details about this article) |
17/91. pregnancy associated aplastic anemia--a series of 10 cases with review of literature.Introduction: pregnancy induced aplastic anemia is a rare entity and the association is not well explained. There are approximately 80 cases in the literature and we are presenting the largest series, so far, of 10 cases.Results: Total of 10 cases had 11 pregnancies. Mean age at presentation was 25.45 years and mean gestation when symptoms first developed was 17.09 weeks. pallor and bleeding manifestations were the most common presenting complaints. Mean Hb, TLC, ANC and platelets were 4.97 g/dl, 2.74 x 10(9)/l, 1.11 x 10(9)/l and 41 x 10(9)/l, respectively. Bone biopsy cellularity ranged from <5 to 25%. Nine out of 11 (81%) pregnancies were successful of which 7 was full term and 2 were premature. Two babies were small for dates. One spontaneous abortion and one intra uterine death (IUD) were observed. Two out of 11 mothers died due to disease after delivery. Two of the 8 surviving mothers, had spontaneous partial response (22%); 4 mothers were asymptomatic after therapy with immunosuppressives given for 6 months and 3 were lost to follow up without response. Specific therapy (cyclosporin) was tried in two mothers antenatally with partial response in one. One child whose mother was given cyclosporin antenatally had jejunal atresia at birth.Conclusion: pregnancy associated aplastic anemia is a rare association. Spontaneous remission can occur in 25-30% of patients. In the first trimester patients, pregnancy can be terminated while in advanced pregnancy patients can be followed up with stringent supportive care. Cyclosporin may be a safe drug antenatally in such patients. patients with established aplastic anemia should avoid pregnancy.- - - - - - - - - - ranking = 0.012560810031577keywords = duct (Clic here for more details about this article) |
18/91. The diagnostic value of the triple bubble sign in proximal jejunal atresia: a case report.Proximal jejunal atresia (PJA) is a common cause of intestinal obstruction in the newborn. Despite the need for an early surgical intervention to minimize morbidity and mortality, a timely identification is frequently precluded by the absence of specific clinical and investigative clues. Against the background of the limitations in making a timely diagnosis of PJA in a tropical setting, where opportunities for high-tech imaging tools are few, we report the diagnostic value of the "triple bubble" sign on the plain radiograph of a Nigerian infant. This radiologic finding led to an early diagnosis and ultimately a prompt surgical extirpation. The paper suggests that the presence of this sign should be a pointer to an early diagnosis of PJA.- - - - - - - - - - ranking = 0.47423830781864keywords = obstruction (Clic here for more details about this article) |
19/91. Hereditary multiple intestinal atresia: thirty years later.BACKGROUND: Hereditary multiple intestinal atresia (HMIA) is an unusual form of intestinal atresia with a presumed autosomal recessive mode of inheritance. The aim of this study was to review the authors' experience with this disease, 30 years after its first description. methods: All cases of HMIA treated at the authors' institution were reviewed with a particular focus on presence of close consanguinity in the families, prenatal diagnosis, radiologic and surgical findings, pathology report, and outcome. RESULTS: Sixteen cases were identified. Two patients were siblings (1 newborn and 1 aborted foetus) and close consanguinity was proven in 1 other case. Bowel obstruction was suspected on prenatal ultrasound scan in 6 patients, but HMIA could not be diagnosed specifically. Radiologic, surgical, and pathologic findings were compatible with the standard description of this disease in the literature. All the patients died. Mean survival time was 50 days. CONCLUSIONS: Thirty years after its first description, HMIA remains a disease without reliable prenatal diagnosis nor effective surgical therapy. An autosomal recessive mode of inheritance is suspected. Until accurate in utero diagnosis becomes available, children with HMIA should be oriented toward palliative care.- - - - - - - - - - ranking = 0.47423830781864keywords = obstruction (Clic here for more details about this article) |
20/91. Hirschsprung's disease complicating colonic atresia.A case of colonic atresia associated with Hirschsprung's disease is described in a full term neonate presented with intestinal obstruction. laparotomy revealed type III colonic atresia. Histopathological examination suggested total aganglionosis in the postatretic colonic segment. The child recovered satisfactorily following two stage Duhamel - Martin's pull through procedure. Authors present their experience with the present case and the pertinent literature.- - - - - - - - - - ranking = 0.47423830781864keywords = obstruction (Clic here for more details about this article) |
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