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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In many children with cholestasis, ultrasonography can rule out the possibility of biliary atresia. In the few cases when a diagnosis cannot be established by ultrasonography, laparoscopy is still justified as an initial procedure, as the amount of trauma involved is still minimal. Of the 36 children with cholestasis on which a laparoscopy was performed, one-third eventually underwent laparotomy because of biliary atresia. The question was whether the primary laparoscopy was really advantageous. In comparison to laparotomy no advantages were found with regard to anesthesia time; morbidity, or complications. The diagnostic accuracy was comparable to that of laparotomy. The only complication was a small scar hernia in a premature baby.
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PMID:Experience with laparoscopy for the evaluation of cholestasis in newborns. 213 62

The site and nature of lesions producing gastrointestinal bleeding was evaluated in pediatric patients admitted to Tokai University Hospital. The differential diagnosis was possible based upon the character of the bleeding and the age of the patient. Upper endoscopy is the diagnostic maneuver of choice in evaluating the upper gastrointestinal bleeders. Sigmoidoscopy, colonoscopy, technetium scans, tagged red cell scans and intraoperative angiography were helpful in locating bleeding sites of lower bleeders. Common causes of bleeding were as follows: Hemorrhagic disease, necrotizing enterocolitis, and midgut volvulus in neonates; intussusception and internal hernia in infants; juvenile polyp and infectious diarrhea in children; duodenal ulcer and ulcerative colitis in adolescents. Gastro-duodenal ulcers were found in all age groups. One neonate died of indomethacin induced bleeding, however, bleeding from acute ulcer was usually controlled by conservative treatments. Increasing frequency of variceal bleeding due to portal hypertension after successful Kasai procedure for congenital biliary atresia was emphasized.
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PMID:[Gastrointestinal bleeding in children]. 258 65

Transfer of antibiotic (cefmetazole, CMZ) into the liver tissues of the infant with hepatic dysfunction, 6 cases of congenital biliary atresia, 4 cases of congenital bile duct dilatation, 1 case of congenital biliary hypoplasia, hepatic hemangioma and umbilical hernia with congenital heart disease is reported here. CMZ level in the liver tissues with hepatic dysfunction shows extremely low. Our study revealed that poor transfer of CMZ into the liver tissues might be a main cause of the poor excretion of CMZ into the bile in case of jaundiced infant.
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PMID:[Transfer of antibiotics into the liver tissue of infants with hepatic dysfunction]. 386 94

Situs inversus (SI) complicating neonatal bowel obstruction presents a challenging complex, and to facilitate rational decision making for treatment, we have reviewed 23 cases of abdominal SI seen in our hospital over the last 25 years. Preoperative roentgenographic studies most always predicted SI, the specific patient groups including: 12 abdominal SI with dextrocardia, 10 abdominal SI with levocardia, and 1 with partial heterotaxia. Major intraabdominal anomalies produced surgical emergencies in 7 neonates in the first year, 6 of these 7 being in the first month of life. In these 7 patients, multiple anomalies occurred including 1 child with a rotational anomaly with reversible ischemia secondary to midgut volvulus, and 4 with a rotational anomaly without volvulus, all being treated with a modified Ladd procedure. One of these children had an unrecognized intraluminal duodenal membrane, 1 an operatively diagnosed intraluminal membrane, 1 had annular pancreas, and 1 had a discontinuous jejunal atresia. A preduodenal portal vein was present in 4 of the 7 children, a branch being divided in 1 and the full vein bypassed in 2 of the other 3 patients. Two patients had biliary atresia, one of whom also had a diaphragmatic hernia. Five of the 7 neonates had associated major congenial heart disease accounting for 2 of the 3 deaths in this series. This review emphasizes the protean nature of abdominal SI, especially as it may cause or contribute to neonatal intestinal obstruction; and it is this understanding which is a prerequisite to optimal operative management.
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PMID:Situs inversus: the complex inducing neonatal intestinal obstruction. 666 1

This article reviews the pathophysiology of congenital defects and tumors that are of interest to the pediatric surgeon. Reliable information has been obtained through prenatal diagnosis of fetal anomalies and careful studies of fetal animal models. The topics covered include fetal diaphragmatic hernia, oligohydramnios-induced pulmonary hypoplasia, renal dysplasia, prune belly, gastrointestinal obstruction, biliary atresia, Wilms' tumor, and neuroblastoma. In addition, some recent experimental studies delineating the ability of the fetus to heal without scarring may have implications for all surgeons.
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PMID:The developmental pathophysiology of surgical disease. 806 34

Kabuki syndrome is a rare multiple congenital anomalies/mental retardation syndrome comprising a distinct facial appearance and fetal fingertip pads. We observed two patients with Kabuki syndrome and describe unusual life-threatening complications, including stenosis of the central airways (not previously reported), extrahepatic biliary atresia, and congenital diaphragmatic hernia.
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PMID:Unexpected life-threatening complications in Kabuki syndrome. 1098 74

Kabuki make-up syndrome (KMS, OMIM 147920) is an MCA/MR syndrome of unknown cause. It is characterized by a dysmorphic face, postnatal growth retardation, skeletal abnormalities, mental retardation, and unusual dermatoglyphic patterns. Approximately more than 350 cases have been reported from all over the world. Besides these five cardinal manifestations, joint laxity (74%), dental abnormalities (68%), and susceptibility to infections including recurrent otitis media (63%) were well recognized as other frequent features. A variety of visceral anomalies such as cardiovascular anomalies (42%), renal and/or urinary tract anomalies (28%), biliary atresia, diaphragmatic hernia, and anorectal anomaly were also reported. Some patients were said to have normal intelligence (16%) and normal heights, suggesting that they may have reproductive fitness to have their children. At least eight patients had lower lip pits with or without cleft palate, known as a feature of van der Woude syndrome. There have been 13 chromosomal abnormalities associated with KMS. However, no common abnormalities or breakpoints that possibly contribute to positional cloning of the putative KMS gene(s) are known. Although clinical manifestations of KMS are well established, its natural history, useful for genetic counseling, remains to be studied.
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PMID:Kabuki make-up syndrome: a review. 1256 Oct 59

Bilateral pulmonary agenesis (PA) is a rare embryological defect incompatible with life. Unilateral PA has a wide range of clinical presentations: its prognosis depends on the presence and severity of other associated anomalies. Fetal biliary atresia has been associated with a number of congenital anomalies, but the etiology is still not understood. An unusual case of a child with right PA, right diaphragmatic hernia, and delayed diagnosed biliary atresia leading to liver failure is presented herein. At the age of 4 months the patient was referred to the Transplant Department at Children Healthcare of Atlanta at Egleston with cholestasis and failure to thrive. With a rapidly progressive liver insufficiency, this child was evaluated for liver transplantation. In the absence of any respiratory symptom, the patient received a deceased donor size-matched left lateral segment liver transplant, which covered the diaphragmatic defect, with no further repair required. Twenty-seven months post-transplant, the patient has good graft function, a normal Z-score and is thriving. In spite of the increased physiological and surgical challenges (absence of right lung tissue, hemi-diaphragm, and ectopic position of the liver in the right chest), liver transplantation was performed with positive outcome in this high-risk child. Whether PA, may have developmentally contributed to expression of biliary atresia will need further investigation.
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PMID:Liver transplant in a four-month-old child with biliary atresia, unilateral pulmonary agenesis, and diaphragmatic hernia: first case report. 1671 13

Diaphragmatic hernia is a rare complication following solid organ transplantation. We here report three pediatric patients suffering from posttransplant enterothorax. One patient with biliary atresia presented with clinical signs of peritonitis without showing pulmonary symptoms four weeks following liver transplantation. The second patient was admitted with suspected pneumonia, whereas the third patient presented with recurrent abdominal pain over weeks and physical examination revealed the unexpected diagnosis of enterothorax. All patients received split liver transplants. Unspecific clinical signs mislead to suspected infectious complication under immunosuppression. No apparent risk factors for diaphragmatic hernia could be identified. Diaphragmatic hernia can present with a variety of atypical clinical symptoms. Severe or prolonged abdominal complains should lead to x-ray examination. We speculate that the split liver technique used in our center could lead to this rare complication due to the different anatomic position of the liver transplant in the abdomen.
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PMID:Diaphragmatic hernia resulting in enterothorax following pediatric liver transplantation: a rare complication. 1692 4

Diaphragmatic hernia is a rare complication following pediatric LT. Here, four children who developed right-sided posteromedial diaphragmatic hernias after LT are reported. The primary disease was biliary atresia in two patients, hemangioendothelioma in one, and angiosarcoma in one patient. All of the patients underwent living-donor LT using a left lateral graft. The patients presented with abdominal and/or pulmonary signs and symptoms. The diaphragmatic hernias were diagnosed at 28 days to seven months post-transplant by standard radiographs or chest CT. The defects were located at the posteromedial aspect of the diaphragm and were closed by primary closure. After diaphragm repair, the post-operative course was unremarkable and there were no recurrences. Thermal or mechanical injuries to the bare area, especially in cases of excessive adhesion between the liver and diaphragm after Kasai operation, were the possible causes of the posteromedial diaphragmatic hernia after pediatric LT.
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PMID:Posteromedial diaphragmatic hernia following pediatric liver transplantation. 2123 8


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