Congenital esophageal stenosis (CES) is a narrowing of the esophageal lumen from birth. Three types of CES have been described; tracheobronchial remnants (TBR), membranous web (MW), and fibromuscular stenosis (FMS). We reviewed the clinical features and the surgical outcome of 14 patients, pathologically confirmed as CES. Nine patients had TBR, 3 FMS, and 2 MVV. The mean age at operation was 3.8 years. Five patients were boys and 9 girls. Four patients had other congenital anomalies. Segmental resection of the lesion and end to end anastomosis was utilized in all cases except one who underwent myotomy. The stenotic segment was located at the distal esophagus in all patients. There were 8 complications in 6 patients, but no mortality. The mean follow-up period was 68 months. There were no feeding problems but 3 patients had minor gastroesophageal reflux. Our result indicates that segmental resection and anastomosis is a satisfactory surgical procedure in the management of CES.

Diagnosing Hirschprung's disease is one of the clinical challenges of this disorder. In the stomach and the intestines, Cathepsin D was readily detected in cytoplasm of the rat gastric and in intestinal ganglion cells of the autonomic nervous system. The
objective
s of the present study were to examine cathepsin D expression in ganglion cells of the submucosal and myenteric plexuses of the intestine of children and to determine the utility of immunohistochemical staining of cathepsin D for detection of immature ganglion cells. Paraffin blocks of 35 intestinal segments were reviewed for immunohistochemical staining with polyclonal antibody to cathepsin D and hematoxylineosin stainings from the compatible specimens. There were 9 aganglionic segments and 9 ganglionic segments of neonates with Hirschsprung's disease, 8 intestinal segments with non-Hirschsprung's disease in neonates and 9 intestinal segments with non-Hirschsprung's disease infants over the age of 10 months. All ganglion cells showed intense granular cytoplasmic reactivity for cathepsin D regardless of maturity and all aganglionic segments had no expression for cathepsin D in the submucosal and myenteric plexuses of the intestine. However, histiocytes within the laminar propria and submucosa stained positively for cathepsin D. In conclusion, intestinal ganglion cells in children have reactivity for cathepsin D, threrfore immunohistochemical staining for cathepsin D can be used for identification of ganglion cells in neonates.

Extended porta hepatis dissection and hepatic porto jejunostomy was performed on 14 biliary atresia patients during last 13 years by a single surgeon. The average age at operation was 68 days(range from 37 days to 98 days). The patients were admitted for 8 weeks postoperatively for administration of parenteral antibiotics. There was one operative mortality due to acute hepatic necrosis. Among 13 patients remaining, 12(92.5 %) became chemically jaundice-free within 36 weeks postoperatively(average 16.8 weeks), the earliest 8 weeks, and in one patients jaundice persisted. Five( 38.5 %) patients developed cholangitis after operation. Among jaundice-free patients, one patient died of unrelated disease 2 years after hepatic porto jejunostomy, who underwent left lateral segmentectomy because of a biloma. Eleven survivors(78.6 %) are jaundice-free. The oldest one is 13 years old, enjoying a normal life. The mean period of follow-up is 7 years and 3 months.

Eight pediatric patients with Peutz-Jeghers syndrome were treated from 1984 to 1994 at the Department of Surgery, Seoul National University Children's Hospital. We reviewed the clinical features of our own 8 cases as well as 56 cases reported in Korean literatures. The results were compared to those of western reports. Gastrointestinal complications in our series were more frequent than in the Korean series, but the distribution of polyps was similar. The Korean series showed the following characteristics compared to the western reports; demographic backgrounds were similar; gastrointestinal symptoms were more common; location of the polyp was more frequent in colon (2 times); and the most prevalent site of malignant change was the colon in Korean cases.

The incidence of Meckel's diverticulum(MD) in general population has been assessed as 2 percent. The major complications of MD are bleeding, perforation, and intestinal obstruction. In spite that the complication rate of Meckel's diverticulum is relatively high(about 4.2% during a lifetime), the preoperative diagnostic rate of complicated MD is very low. Authors investigated the clinical characteristics of complicated MD to improve the diagnostic rate. 16 patients with complicated Meckel's diverticulum who were operated upon at the Department of Pediatric Surgery, Seoul National University Children's Hospital from June 1985 to December 1993 were reviewed. Among the 16 patients with complicated MD, 12 patients(75%) were under 2 year-old and male were predominant(88%). The most common complication was bleeding patients with bleeding MD (8 cases) were diagnosed preoperatively as MD. 8 patients with other complications(perforation : 4 cases, obstruction: 4 cases) could not be suspected as complicated MD except one patient who had previous history of melena. These patients were diagnosed after exploratory laparotomy under the various impression other than MD. Among 12 patients with ulcer related complications such as bleeding and perforation, heterotopic gastric mucosa was found in 11 patients. In conclusion, in any children with unexplained acute abdomen, especially under 2 years old, complicated MD must be included in differential diagnosis. In children with obscure lower gastrointestinal bleeding,^99m Tc-pertechnetate scintigraphy is a useful diagnostic tool to rule out bleeding MD.