• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.103-107
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• 2006

Antireflux surgery has been indicated in gastroesophageal reflux disease (GERD) that does not respond to medical treatments. Although the most commonly performed operation is Nissen fundoplication, Belsey Mark IV fundoplication is indicated for more complicated cases, such as, in cases of a failed Nissen operation or a long lasting hiatal hernia. Here, we report a case of Belsey Mark IV fundoplication for a failed Nissen fundoplication. The infant developed frequent times of aspiration pneumonia after initial Nissen for a hiatal hernia with GERD during the newborn period. At 15 months of age, a $2^{nd}$ Nissen operation was attempted, but fundoplication was not available because of excessive mesenteric adherence to the liver and cardia. Therefore, Belsey Mark IV fundoplication was performed via trans-thoracic approach, which can provide full esophageal mobilization and better visualization of the herniated fundus and the surrounding tissues. Subsequently, she has shown an improved general condition without GERD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.92-97
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• 2006

Congenital diaphragmatic eventration is the abnormal elevation of the diaphragm into the thoracic cavity. Sometimes, it is not easy to differentiate congenital diaphragmatic eventration from diaphragmatic hernia by either prenatal sonography or postnatal chest radiography. However, differential diagnosis of both diseases is practical because of different prognosis and surgical approaches. Careful interpretation of postnatal serial chest X-rays is mandatory to differentiate between both diseases. We report two neonates with congenital diaphragmatic eventration of left diaphragm that initially misdiagnosed as diaphragmatic hernia by prenatal sonography and postnatal chest radiography.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.1
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• pp.108-112
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• 2002

Solid and papillary epithelial neoplasm (SPEN) of the pancreas is an uncommon tumor and is found predominantly in young females. The most common clinical presentation is an abdominal mass. The tumor has a low grade malignant potential and complete removal is the treatment of choice. We report a case of SPEN in a 13-year-old girl who presented with abdominal pain and increasing size of an abdominal mass after abdominal trauma. CT and sonographic findings showed a well-demarcated mass in the pancreas tail with solid and cystic portion. She got a distal pancreatectomy and pathologic finding was SPEN of pancreas.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.1
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• pp.96-100
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• 2002

Colitis cystica profunda is a rare benign condition characterized by the presence of mucus-filled cysts in the submucosa of the colon and the rectum. Although it may diffusely involve the entire colon, this disease primarily affects the pelvic colon and rectum. It has rarely been described in the pediatric literature. The surgical treatment has been widely advocated. We report a 7-month-old case, successfully treated by colonoscopic polypectomy without complication. Histologically, components of juvenile retention polyp were mixed with colitis cytsica profunda.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.206-211
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• 2002

Wilson's disease is a treatable autosomal recessive inherited disorder of copper metabolism due to mutation of the copper transporting gene. The basic strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering both a low copper diet and copper-chelating agents. D-penicillamine is the first choice as a copper-chelating agent. Some serious side effects could occur in 3~5% of all patients following D-penicillamine therapy. We report a 19 year-old male with Wilson's disease who developed nephrotic syndrome 6 months after the initiation of D-penicillamine therapy. Prednisolone was administered to control nephrotic syndrome and D-penicillamine was switched to trientine. Urinary remission was achieved within a week and maintained thereafter. Nephrotic syndrome was proven to be MCNS by kidney biopsy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.199-205
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• 2002

Congenital hypopituitarism is a possible cause of neonatal cholestasis, but the mechanism is still unknown. The pathogenesis of cholestasis may be due to hormone deficiency, which has effects on the physiological maturation of bile acid synthesis and transport. We experienced a case presenting with cholestasis and recurrent hypoglycemia associated with congenital hypopituitarism. Cholestasis resolved with thyroxine and hydrocortisone replacement therapy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.186-191
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• 2002

Choledochal cyst is a congenital anomaly with classic triad of abdominal pain, jaundice and right upper abdominal mass. Bile peritonitis caused by cyst rupture is relatively not rare in infancy. The mechanism of rupture must be epithelial irritation of the biliary tract by refluxed pancreatic juice caused by pancreatico-biliary malunion associated with mural immaturity in infancy, rather than an abnormal rise in ductal pressure or congenital mural weakness at a certain point. We experienced a case of bile peritonitis caused by spontanenous rupture of choledochal cyst in a 10-month-old girl presented with abdominal distension, persistent fever, diarrhea, irritability and intractable ascites. She was presumed as having bile peritonitis by bile colored ascitic fluid with elevated bilirubin level and diagnosis was made by $^{99m}Tc$ DISIDA hepatobiliary scan showing extrahepatic biliary leak. The perforated cyst was surgically removed and the biliary tree was reconstructed with a Roux-en-Y hepaticojejunostomy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.262-266
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• 1999

Obstruction of the extrahepatic bile ducts is the most common cause of conjugated hyperbilirubinemia in early infancy. More than 90% of such obstructive lesions are accounted for by extrahepatic biliary atresia. A rare lesion is obstruction of the common duct by impacted, thickened secretions and bile. Bile plug syndrome is defined as extrahepatic obstruction of the bile ducts by bile sludge in term infants without anatomic abnormalities, congenital chemical defects of bile, or hepatocellular lesions. Obstruction of extrahepatic ducts by plugs of biliary material apperas to be due to the inspissation and precipitation of bile and mucus within the lumen of the ducts. Cholestasis and precipitation of bile develop in association with abnormal composition of bile in cystic fibrosis, hepatocellular damage, prolonged erythroblastic jaundice, altered biliary dynamics with total parenteral nutrition, gut dysfunction, diuretic therapy, exchange transfusions and perinatal hemolysis. In those cases, the term inspissated bile syndrome is used. The clinical and laboratory findings in bile plug syndrome are identical to those observed in biliary atresia and choledochal cyst. The diagnosis can be suspected based on the findings of clinical and laboratory examinations together with hepatobiliary imaging, ultrasonography, radionuclide scan and liver biopsy. We experienced a case of spontaneous resolution of bile plug syndrome in a 4-year-old girl. We report this case with brief review related literatures.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.233-239
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• 1999

Acrodermatitis enteropethica (AE) is a rare autosomal recessive disorder of zinc absorption leading to chronic diarrhea and characteristic skin lesion. The term is also applied to any acquired zinc deficiency state resulting in the same clinical pictures. We experienced one case of AE in 1 month old male infant who had bacterial enterocolitis. The skin around mouth, anus, eyes, ears, hands and legs became reddish, vesicular and eczematoid. Serum zinc level was decreased to $51.4\;{\mu}g/dL$ (N=70~150). Endoscopic finding revealed pale gastric mucosa and villous atrophy of small intestine. Biopsy finding of small intestine showed no villi due to mucosal atrophy. On 13 day of admission jaundice with DIC were noted and AST & ALT were elevated to 110 & 36.8 IU/L, respectively. Diarrhea was improved but jaundice and liver function were not recovered until discharge from hospital. After discharge when the patient was 4 months of age serum bilirubin and AST/ALT had not been normalized. CMV shell vial culture of urine and CMV Ig G antibody were positive. So intravenous ganciclovir injection of 7.5 mg/kg, two times a day for 2 weeks and then 10 mg/kg/day for 3 months was done from 4 to 6 months of age. No virus was found in the urine and AST & ALT were normalized at 2 months after stopping ganciclovir treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.222-226
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• 1999

Segmental necrotizing jejunitis is characterized by severe abdominal pain of acute onset, bilious vomitings and foul smelling loose stools containing blood. Pathologic features include circumferential intestinal wall inflammation ranging from edema with minimal congestion to severe congestion, hemorrhage with necrosis, ulceration, and gangrene with perforation. Early diagnosis and suitable supportive measures prevent unnecessary laparatomy and complications. There was no report of this disease entity in children in Korea. We experienced a case of segmental necrotizing jejunitis with fever, abdominal pain and bloody stools, which was diagnosed by exploration and was treated successfully by antibiotics and supportive measures.