1/31. Mucous fistula refeeding in neonates with short bowel syndrome.BACKGROUND/PURPOSE: Neonates with enterostomies commonly suffer from a functional short bowel syndrome (SBS) and have a greater risk of electrolyte and fluid loss with poor weight gain. The authors describe their experience with refeeding stoma effluent into the mucous fistula in neonates. methods: A 5-year (1993 to 1997) chart review of neonates with stoma effluent refeeding was undertaken. Demographics, medical history, surgical procedures, timing, and duration of refeedings were reviewed. Enteral and total parenteral nutritional (TPN) requirements, electrolyte, and acid-base disturbances were recorded. RESULTS: Six neonates (gestational ages of 27 to 38 weeks, birth weights of 533 to 3400 g) were identified with nutritional or electrolyte complications before the commencement of refeeding. enterostomy indications included necrotizing enterocolitis (n = 2), intestinal atresia type 3b (n = 1), complications from ruptured omphalocoele (n = 1), congenital adhesive band obstruction (n = 1), and midgut volvulus after congenital diaphragmatic hernia repair (n = 1). weight gain during refeeding ranged from 5 to 25 g/kg/d with duration of refeeding lasting 16 to 169 days (two neonates were refed at home) until reanastomoses were done 6 to 44 weeks after the original surgery. There were no complications, and TPN requirements were diminished or eliminated. CONCLUSION: This technique represents a simple and safe method, which lessens the need for TPN and electrolyte supplementation in neonates with enterostomies and SBS before reanastomosis.- - - - - - - - - - ranking = 1keywords = obstruction (Clic here for more details about this article) |
2/31. Progressive perinatal bowel obstruction--a rare cause of short-bowel syndrome.A girl was born after an uneventful pregnancy of 36 weeks. Prenatally, distended bowel loops had been seen on ultrasound. Multiple small-bowel atresia was diagnosed and treated surgically. In the course of the next eleven weeks, previously patent segments of small bowel became obstructed. In 4 separate operative sessions, several segments of jejunum and ileum were resected, leaving 23 cm of ileum with the ileocecal valve in place. On microscopic examination of all resected material, necrosis of the mucosa was found consistent with ischemia. The child survived and tolerated full enteral feeding at the age of 8.5 months. The origin of the progressive obliterating process remains unknown.- - - - - - - - - - ranking = 4keywords = obstruction (Clic here for more details about this article) |
3/31. Hepatopathy in two infants with short-bowel syndrome and cytomegalovirus infection.In children with short-bowel syndrome and the need for long-term parenteral nutrition, hepatic dysfunction is a multifactorial phenomenon that has not been completely understood. Alterations in gut motility lead to intraluminal stasis which is thought to be a major etiologic factor for bacterial overgrowth and subsequent cholestasis, especially when the ileocecal valve is absent. We report on two infants with short-bowel syndrome caused by gastroschisis and intestinal atresia. The intestinal lengths after resection were 18 and 55 cm. Long-term parenteral nutrition (PN) was obligatory due to intestinal shortness in the first patient and dilatation of the preatretic bowel segment with ineffective peristalsis in the second patient. Despite multiple trials of enteral nutrition and medical therapy for gut decontamination and stimulation of bowel motility, hepatopathy developed in both patients in a similar period of time and to about the same degree. At the age of 4 and 6 weeks, respectively, increasing bilirubin values were measured. Deterioration of liver function and thrombocytopenia at the age of 3 to 4 months led to the diagnosis of acute cytomegalovirus (CMV) infection. Treatment with ganciclovir followed. Both patients died of acute liver failure at the age of 7 and 9 months, respectively. Additional hepatic injury secondary to CMV infection might have contributed to the rapid deterioration of liver disease. Screening for further hepatotoxic factors, especially infectious etiologies, is therefore recommended in children with short-bowel syndrome. liver transplantation should be considered early in cases of progressive hepatic dysfunction.- - - - - - - - - - ranking = 171.55957923796keywords = cholestasis (Clic here for more details about this article) |
4/31. Congenital short-bowel; a case study and review of the literature.A congenital short bowel (CSB) is a rare entity in pediatric surgery. We present the case of a newborn boy with a total small intestinal length of 47 cm, malrotation and gastroesophageal reflux, who is 19 months old at the time of this report. Main treatment steps were Ladd's procedure, a fundoplication and long-term parenteral nutrition. We suggest that missing physiological herniation of the gut into the coelomic cavity may impair normal intestinal growth and rotation and lead to congenital short bowel. review of all cases reported in the literature shows a considerable mortality of 88%. The limiting factor seems to be reduced motility of the short small bowel causing functional obstruction and liver failure.- - - - - - - - - - ranking = 1keywords = obstruction (Clic here for more details about this article) |
5/31. pancytopenia after removal of copper from total parenteral nutrition.patients who develop cholestatic jaundice during chronic total parenteral nutrition (TPN) can develop significant hematologic complications due to hypocupremia if copper supplementation is withheld. A 36-year-old female with short bowel syndrome developed progressive liver dysfunction 6 months after initiation of TPN. trace elements were omitted from her TPN because of cholestasis and persistent hyperbilirubinemia. Despite chronic diarrhea, absorption of some dietary copper was anticipated from her oral diet. Fifteen months later, the patient became red cell transfusion dependent, and her neutrophil and platelet counts steadily declined. After 19 months of receiving TPN without trace elements, her serum copper level was 25 microLg/dL (normal: 70 to 155 microg/dL). Provision of trace elements for 2 months was associated with increased serum copper, neutrophil and platelet counts and independence from red cell transfusions. When the serum copper level reached 186 microg/dL, copper supplementation was discontinued. Over the next 3 months, serum copper level fell to 10 microg/dL, neutrophil and platelet counts fell precipitously, and red cell transfusions were resumed. Once again, copper, neutrophil and platelet levels promptly rebounded with parenteral copper supplementation. Although anemia and neutropenia are well-recognized hematologic consequences of copper deficiency, thrombocytopenia rarely has been reported. This is the first report of pancytopenia secondary to TPN-related copper deficiency in which the association was confirmed when hypocupremia recurred.- - - - - - - - - - ranking = 171.55957923796keywords = cholestasis (Clic here for more details about this article) |
6/31. Vanishing gastroschisis and short-bowel syndrome.BACKGROUND: gastroschisis occurs in 1 of every 4000 live births resulting in a neonate with an abdominal wall defect that requires repair. Surgical correction has high survival rates. CASE: An 18-year-old primigravida had a fetus with gastroschisis detected by ultrasound performed for elevated maternal serum alpha-fetoprotein. Subsequent ultrasound found resolution of the classic sonographic features of gastroschisis and evidence of intestinal obstruction. At birth, no obvious abdominal wall defect was seen. laparotomy was done because of clinical and radiographic evidence of bowel obstruction, and we found significant bowel loss that resulted in short-bowel syndrome. CONCLUSION: gastroschisis diagnosed antenatally can resolve in utero causing necrosis of portions of the small and large bowels, causing short-bowel syndrome and increased morbidity and mortality.- - - - - - - - - - ranking = 2keywords = obstruction (Clic here for more details about this article) |
7/31. Abnormal liver functions as a result of total parenteral nutrition in a patient with short-bowel syndrome.The pathogenesis of total parenteral nutrition (TPN)-induced liver cholestasis is poorly understood. cholestasis generally occurs late in TPN therapy in association with elevated serum alkaline phosphatase and total bilirubin concentrations. Such factors as preexisting medical conditions, excessive nutrient infusion, amino-acid deficiency, absence of enteral stimulation, protracted duration of therapy, continuous infusion schedule, and hypoalbuminemia have all been suggested as possible etiologies. Various treatments have been proposed for the correction of TPN-induced cholestasis including administration of bile salt and antimicrobial therapies. To avoid potential hepatic complications associated with TPN, certain preventive measures can be considered. Administration of energy substrates should not be excessive. A mixed-fuel system that includes lipids should be implemented. TPN should be cycled if it will be used long term, and initiation of enteral nutrition should begin as soon as possible.- - - - - - - - - - ranking = 344.96339837862keywords = cholestasis, bile (Clic here for more details about this article) |
8/31. Hirschsprung's disease presenting with diffuse intestinal pneumatosis in a neonate.In the neonate, pneumatosis intestinalis is almost always associated with necrotizing enterocolitis. The manifestation of diffuse intestinal pneumatosis in Hirschsprung's disease has been reported rarely. It may occur as a result of Hirschsprung's disease complicated with enterocolitis. We report a two-day-old female baby born at term with the problems of failure to pass meconium, progressive abdominal distension and bile stained vomiting. There was an early roentgenographic presentation of pneumatosis intestinalis which might have led to a diagnosis of necrotizing enterocolitis. However, the intestinal pneumatosis resolved within 48 hours. After anorectal manometry and contrast enema examination, an ileostomy was performed at the age of 23 days, and multiple biopsies of intestine showed aganglionosis up to the ileum at the level of 85 cm above the ileocecal valve. Unfortunately, the patient developed short bowel syndrome after operation and died suddenly after an accidental choking at the age of three months. This case suggests that Hirschsprung's disease may have an unusual early roentgenographic presentation with diffuse intestinal pneumatosis in the first few days of life. Anorectal manometries and suction biopsies are crucial for further diagnosis.- - - - - - - - - - ranking = 1.8442399027056keywords = bile (Clic here for more details about this article) |
9/31. Diffuse small bowel Crohn's disease treated with side-to-side isoperistaltic strictureplasty: report of two cases and description of a variation of the original technique.Diffuse small bowel Crohn's disease is unusual and it is characterized by multiple diseased segments involving the jejunum and ileum. The most frequent indication for surgery is an intestinal obstruction, often complicated by a high grade of malnutrition. The natural history of this clinical form is not well defined and the optimal surgical approach remains controversial. We herein present our surgical policy in two cases of diffuse small bowel Crohn's disease, who were particularly at risk of developing short bowel syndrome. We focused our attention on the use of side-to-side isoperistaltic strictureplasty as described by Michelassi for the treatment of stenoses longer than 20 cm. We also propose the application of this technique for the treatment of shorter stenosis cases.- - - - - - - - - - ranking = 1keywords = obstruction (Clic here for more details about this article) |
10/31. Portal biliopathy.Herein we describe a case of obstructive jaundice and cholangitis originating from choledochal lithiasis secondary to a stricture of the common bile duct in a patient with cavernous transformation of the portal vein. In fact, portal cavernous transformation gives rise to many dilated pericholedochal and periportal collaterals that bypass the portal vein obstruction. Extrinsic compression of the common duct by dilated venous collaterals together with pericholedochal fibrosis from the inflammatory process causing portal thrombosis may lead to biliary stricture and dilatation of the proximal biliary tree. This condition sometimes causes the formation of secondary biliary stones and cholangitis. Treatment in our case could not be accomplished by a biliodigestive anastomosis because the patient suffered from a short bowel syndrome subsequent to extensive ileal resection for splanchnic venous thrombosis. We repeatedly attempted stone removal during endoscopic retrograde cholangiopancreatography (ERCP) but finally resorted to a percutaneous transhepatic approach, which permitted removal of the stones and treatment of the stricture by pneumatic dilatation. The patient remains well 3 years after the procedure and has shown no signs of jaundice or cholangitis.- - - - - - - - - - ranking = 2.8442399027056keywords = bile, obstruction (Clic here for more details about this article) |
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