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.

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.

Spontaneous gastric perforation is an important but rare cause of gastrointestinal perforation in neonates. Just over 200 cases have been reported in the literatures. In spite of recent surgical advances in its managements, mortality rate has been reported as high as 25~50%. Because of physiologic differences, immature immune mechanisms, variations in gastrointestinal flora and poor localization of perforation, a neonate with gastric perforation is at high risk. The pathogenesis is greatly debated. Five patients with spontaneous neonatal gastric perforation who were operated upon at the Department of Pediatric Surgery, Seoul National University Hospital from 1980 to 1993 were reviewed. Four patients were male and one female. The first indication of perforation was 1 day to 6 days of life. All of 5 perforations were located along the greater curvature of the stomach. The size of perforation ranged from 2 cm to 10 cm. Debridement and primary closure were performed in all patients. The operative mortality was 40%(2 of 5). The cause of perforation was not identified in all cases. Prematurity and necrotizing enterocolitis, synchronous or metachrotlous, were thought to be crucial prognostic factors. Earlier recognition and surgical intervention are necessary to reduce morbidity and mortality.

Aberrant migration of thymic tissue occurs as an ectopic thymus in the mediastinum, base of skull, tracheal bifurcation, and cervical region. A recent review of the literature by Nowak et al. showed over 70 reported cases of aberrant thymus or thymic cyst in patients who presented with primary neck masses. Authors experienced a case of ectopic cervical thymus and reviewed the literature. A one-year-old boy with left neck swelling which had been noticed since one month of age visited out patient clinic. Ultrasonography showed a well-defined cystic mass containing homogeneous, low-echogenic content locating in the lateral aspect of the left carotid sheath. Operation was performed under the impression of branchial cleft cyst. At surgery, a multiseptated, well-encapsulated, brownish and doughy mass which was extending into the vicinity of the carotid bifurcation with sland stalk-like portion ending between the hypoglossal nerve and external carotid artery was excised completely. The cut-surface showed homogenous solid mass, and on frozen section the tissue revealed a normal thymic histology. Postoperative ultrasonography showed bilateral thymus in the superior mediastinum. The patient has no immunologic problem and is doing well now.

Since 1988, we have performed esophageal replacement with the reversed gastric tube on four esophageal atresia patients. Three patients had long-gap esophageal atresias and one patient had recurrent tracheoesophageal fistula that was previously operated on three times. One combined imperforate anus.

The youngest patient was 6-month-old and the oldest, 34-month-old at the time of procedure. The technique of gastric tube construction is described. There have been both major and minor complications. Although two patients had shown distal tube strictures as late complications, those were solved with tuboplasties on 29 months and 48 months, postoperatively.

Growth and development have been acceptable in all four patients, although most remain in the lower percentiles for growth and height, a condition that usually predates the esophageal substitution.

Conclusively, reversed gastric tube interposition has proved very satisfactory for long-gap esophageal atresia that cannot be anastomosed primarily even by spiral myotomy and esophageal atresia with recurrent tracheoesophageal fistula having destroyed esophagus due to previous operation.

To study the clinical profiles and outcome of surgery in infants with esophageal atresia, we reviewed 96 esophageal atresia cases who were treated from April, 1978 to June, 1995. There were 51 male and 45 female infants, a ratio of 1.1:1. The low birth weight(<2500g) patients were 32%. Clinical findings at the time of diagnosis inculded drooling in 57%, choking in 50%, cyanosis in 38%, respiratory distress in 27% and swallowing difficulty in 20%. Gross classification included 6 cases of type A esophageal atresia(6%), 79 cases of type C(82%). 3 cases of type E (3%) and 8 cases of type F(8%). Associated anomalies occurred in 34 infants(35%). Among them, cardiac anomalies were most common(60%). A primary repair of the defect was carried out in 76 patients with type A or C. A staged operation comprising a repair or gastric tube interposition after gastrostomy was performed in 8 patients. In all 3 infants with H-type, a division of fistula was performed. Esophageal resection and anastomosis was done in 8 infants with esophageal stenosis. In one infant, a gastrostomy was performed and he expired before staged operation. Anastomotic complications incuded leakage in 16 cases(l7%), stricture in 37 cases(39%) and recurrent tracheoesopohageal fistula in 3 cases(3%). The mortality rate was 14% and the leading cause of death was peumonia. The overall survival rate was 86%, and according to Waterston cirteria, the survival rates were 93%, 85% and 58% in class A, Band C, respectively. 75 patients were followed up with median follow up 6.4 years. Among them, 93% were uneventful and 7% had frequent pneumonia.

We reviewed 45 cases of ovarian tUmors treated at Seoul National University Children's Hospital from 1983 to 1993. Forty-five patients were operated upon for 52 ovarian tumors. The most common pathologic diagnosis was mature teratoma. The next were functional cyst, the tumors of epithelial cell origin, and those of stromal origin in order of frequency. Six patients(13%) had malignant tumor. There were one malignant teratoma, two dysgerminomas, one endodermal sinus tumor, and two granulosa cell tumors. Four cases were diagnosed as torsion of ovarian cyst preoperatively, and emergency exploratory laparotomy were performed. There were three cases of ovarian tumors associated with precocious puberty. The most widely used diagnostic tool was ultrasonography.

In the treatment of these 45 patients, unilateral oophorectomy was done in 38 cases, unilateral oophorectomy with wedge resection of contralateral ovary was done in 5 cases, unilateral oophorectomy with contralateral simple cystectomy was done in one case and total abdominal hysterectomy with bilateral salpingooophorectomy was done in one case. Of the six cases of malignancy, five patients are alive 2 to 6 years after operation and one case was lost to be followed up.

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.