The acetylcholinesterase(ACHE) activity in 37 biopsies from the patients with Hirschsprung's disease was analyzed for histochemical patterns according to age, and its evolutional behavior was also assessed. The histochemical criterion used for the diagnosis of Hirschsprung's disease was that of Chow, i.e., “the presence of many coarse discrete cholinergic fibers in the muscularis mucosae and in the immediately subjacent submucosa regardless of an infiltration of cholinergic fibers in the lamina propria.” The acetylcholinesterase activity in Hirschsprung's disease was further classified into 3 patterns, advocated by de Brito and Maksoud: Pattern I-many thick fibers exclusively in the muscularis mucosae and submucosa(newborn pattern). Pattern II-many thin fibers in the muscularis mucosae and submucosa with a clear infiltraiton of cholinergic fibers in the lamina propria(classical pattern). Pattern III-an intermediate pattern showing morphological characteristics of the two patterns with predominance of one or the other. Of 37 biopsies, Pattern 1 was seen excusively in 16 biopsies taken from the neonates. Pattern III was 11 of 37 biopsies and it was mainly seen in children between 2 to 12 months of age. Pattern II was seen in all 3 biopsies of older than 1 year of age and in 6 ones of 3 to 12 months of age. In summary, this study reinforces the impression that there is an evolutional character of the distribution and morphology of the cholinergic ACHE positive fiber with age. In other words, ACHE activity in Hirschsprung's disease appears to evolve with age from the newborn pattern to the intermediate pc.ttern finally to the classical pattern.

The clinical experience of 2,340 inguinal hernia hernia repaired by one pediatic surgeon om 2,079 children at Hanyang University Hospital from September 1979 to December 1993 was analyzed. Of 2,046 patients who had primary hernia repairs at Hanyang University Hospital, 1,636 were male and 410 female, and 55.5% of hernias occurred on the right side, 36.0 % on the left, and 8.6% were bilateral. The patients presented hernia under the age of 12 months were 45.3% and those performed herniotomy under the age of 12 months were 25.5%. Birth weight was less than 2.5kg in III patients(8.7%) of 1,279 data available patients. Ninety(6.6%) of 1,354 data available patients were premature (<37wks gestation). The proportions of bilateral inguinal hernia and the onset age under 12 months of life in low birth weight babies and premature babies were higher than in full - term babies. Incarcerated inguinal hernia occurred in 327 patients(16.0%) of whom 8 patients were strangulated hernias. The occurrence of incarceration inversely related with age of patients. The subsequent contralateral inguinal l1ernia following unilateral hernia repairs occurred in 80 patients(4.3%) among which 72 were male and 8 were female. The incidence of contralateral inguinal hernia was more frequent in boys(4.8%) than girls (2.2%) and in cases after left herniotomy(6.4%) than after right herniotomy(2.9%). Sixty percent of contralateral inguinal hernia developed within 1 year after primary hernia repair. The recurrence of inguinal hernia occurred in 6 patients(0.27%) treated at our hospital primarily. There were one or more associated congenital anomalies in 83 patients of which congenital heart diseases were the most common. Sliding hernia occurred in 25 patients consisted of 5 boys and 20 girls. Family history was noted in 35 patients and there were 28 sets of monozygotic and 3 sets of dizygotic twins.

For the prevention of later contralateral hernia as well as unnecessary contralateral exploration in pediatric patients with unilateral inguinal hernias, a reasionable indication of contralateral exploration is required. To examine the contralateral positivity, a prospective selective contralateral exploration has been performed by the author from Sept. 1985 to Dec. 1993, at Pediatric Surgical Section of the Department of Surgery, Kangnam St. Mary's Hospital, Catholic University Medical College. Among the total 1200 cases of pediatric inguinal hernias, 580 cases of contralateral side were explored at hernia operations, by the indications as; 1)male with infant onset, 2)female of all age, 3)prematurity, 4)profuse ascites due to cirrhosis, nephrotic syndrome, and ventriculoperitoneal shunt, and 5)remarkable silk sign. Overall positive rate was 71.4%, and positive rates of each indication were 80.7%, 70.4%, 73.1 %, 66.7%, and 72.0%, respectively. Right side hernia showed 67.0%, left side 75.7%, and positive familial history 71.8% of contralateral positivities. In male,getting older revealed lower positive rates and the rate suddenly dropped after 12 years of age. Birth order, mother's age at delivery, postmaturity did not show any significant differences between the rates. Recurrence was seen in 3(0.5%) ipsilateral and 2(0.3%) contralateral, both of which were negative esplorations on previons operations. Overall complication rate was 3.8%, including 1 infection, 14 fluid or blood accumulation, 5 edemas, 3 temporary testicular edemas, 2 persisting fevers, 2 enuresis and one delayed recovery from anesthesia. Among 38 cases with contralateral hernias developed after unilateral surgery by authors(6 cases) or surgeons in other institutions, 14 were males with infant onset, 4 were prematurities and 9 were females. Therefore, 27(71.7%) cases were originaly under the contralateral exploration indications. The primary site of the hermia was right in 25 and left in 13. With above results, the following indications for contralateral exploration could be suggested; 1)under one year of age, both sex, 2)prematurity, 3)remarkable silk sign, 4)in the double checked suspicions among males with infant onset, all age females, ascites, left hernia and familial history. After 12 years of age, exploration is not required. Considering complications, contralateral explorations could be considered only in the following situations ; 1)expert, experienced pediatric surgeon, 2)experienced pediatric anesthesiologist, 3)operations could be done smoothly in an hour, 4)good general condition of the patient.

Jejunal and ileal atresias are the most common cause of congenital intestinal obstruction and accounts for about 1/3 of all cases of intestinal obstruction in newborns. Despite the relative frequency of this anomaly, its survival rate was less than 10% up to 1950, more recently the survival rate has risen rapidly to 90% with the introduction of modern surgical techniques and the use of total parenteral nutrition. In 1969 Thomas described a tapering jejunoplasty to manage the discrepancy in the size of the proximal dilated lumen & contracted distal lumen, and to preserve absorptive surface when the dilated jejunum involved a long length, and Grosfeld et al.(l979) facilitated this method by using GIA staplers. Author have also used GIA stapler to resect the antimesenteric portion of the dilated proximal bowel in 8 cases of jejunoileal atresias with good results. The following results were obtained; 1. There we 3 jejunal atresias & 5 ileal atresias, and male to female sex ratio was 5 : 3. 2. The type of atresia was as follows; type IIIa was 3 cases, type IIIb was 4 cases, type IIIb+IV was 1 case. 3. In non-complication cases(5 cases), the mean hospital day was 16 days, and oral feeding was feasible from 6.2 days after operation. 4. The complications(anastomotic leakage, pneumonia) were frequently occurred in type IIIb cases and in low birth weight cases(75%). 5. Mortality rate was 25% including DAMA(discharge against medical advice) discharge case.

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.

Anal fistula and perianal abscess in pediatric patients have been reported to have several characteristics, e.g. prevalent in less than 2 years of age, male preponderence, straight course of tract, and low type of fistula. We performed a retrospective study of twenty nine pediatric patients to see these characteristics comparing with the transitional age group of adolescents. Between June 1989 and December 1993, twenty-nine pediatric (<15 year-old) and sixteen adolescent patients(≥ 15, < 25 year-old) with anal fistula and perianal abscess were treated by surgical intervention. Twenty-one(87.5%) and 10(66.7%) enteric bacterial colonies were isolated from 16 pediatric and 11 adolescent patients, respectively. Considering the predominance of low type and the organisms cultured in the pediatric group, crypt-glandular infection seems to be a major preceding event. Incision and drainage were sufficient for cure in 15 among 16 perianal abscesses, and fistulas were cured by either fistulotomy or fistulectomy in all the 14 patients. The importance of effective drainage of perianal abscess and fistulotomy including internal opening cannot be overemphasized.

Percutaneous infraclavicular subclavian catheterization has been widely used for a total parenteral nutrition, hemodynamic monitoring and for venous access in difficult clinical situations. Many authors have claimed the infraclavicular cannulation of the subclavian vein in the tiniest infants can be performed with safety and ease, but there are always possibility of serious complications in this method. We present our experiences of peripheral venous cutdown with Broviac catheter. Author routinely introduced Broviac catheter into central vein via peripheral venous cutdown. There was no life threatening complications and no catheter related death. The complication rate was very low. The catheter related sepsis was documented in only two patient(4.7%). The average catheter longivity was 19.59 days. In view of the safety and low rate of complication, we think that peripheral venous cutdown with Broviac catheter should be the method of choice when central venous access is neccesary in infants. The infraclavicular subclavian catheterization should be reserved in infants with few accessable peripheral vein.

A delayed primary esophago-esophagostomy of a case of long-gap esophageal atresia without tracheoesophageal fistula was performed in success with three months' intermittent periodic bougienage of the upper pouch via mouth as well as the lower esophagus through Janeway gastrostomy. Meanwhile, an effective continuous sump suction from the upper pouch seemed to be a critical part of the patient management. The extra length of esophagus for primary anastomosis could be achieved by a circular myotomy. Stricture at the myotomy site, found 4 months later, was treated with periodic pneumatic baloon dilations only with temporary symptomatic reliefs. After 4 months' trials, operative esophagoplasty was performed successfuly. A careful follow-up schedule for the myotomy site would be required for early detection of stricture. The previous neonatal patient is currently 8 years old, healthy schoolboy, and has a normal barium swallow without stricture or gastroesophageal reflux.

Meconium peritonitis is defined as an aseptic, chemical or foreign-body peritonitis caused by spill of meconium in the abdominal cavity related to the prenatal perforation of the intestine. Perforation is usually caused by obstruction from meconium ileus, intestinal atresia, stenosis, volvulus, internal hernia, congenital pertoneal bands, intussusception, or gastroschisis. Less commonly, no evidence of distal obstruction exists. Here, we present two cases of generalized meconium peritonitis of antenatal diagnosis. The first case, detected at 8 months of gestational age, had a perforation of the proximal blind pouch of jejunal atresia, associated with respiratory distress due to severe abdominal distension. This case was successfullY treated with resection and anastomosis and brief period of postoperative ventilatory support. The second case had a distal ileal perforation with thick meconium in the terminal ileum. In this case, there was no dilatation of ileum proximal to the perforation site. Resection and anastomosis was performed and postoperative course was uneventful.

Although bleeding was reported before as the first symptom in a few cases of biliary atresia, this association is not generally known. We treated 115-day-old female with extrahepatic biliary atresia presented with subdural hemorrhage rather than with prolonged jaundice. Four years after craniectomy and Kasai's hepatic portojejunostomy, she looks happy without jaundice nor brain damage sequela even though she had recent episode of esophageal variceal bleeding.

Segmental dilatation of the colon is a very rare disease entity of unknown etiology and may mimic Hirschsprung's disease. It is characterized by dilatation of a segment of the colon of variable length with obstruction due to lack of peristalsis in a normally innervated intestine. Recently authors experienced a case of segmental dilatation of the sigmoid colon in a 6 month-old male, who presented with severe constipation, abdominal distention, and abdominal mass since 2 months of age. Down's syndrome and congenital nystagmus were associated. Barium enema demonstrated focal dilatation of the sigmoid colon, but the rectum and descending colon proximal to the affected colon were of normal caliber. Rectal suction biopsy with acetylcholinesterase staining was normal and anorectal manometry showed normal rectosphincteric reflex. At operation, there was a massively dilated and hypertrophied sigmoid colon with increased tortuous serosal vessels, measuring 15 cm in length and 10 cm in width. Teniae coli were identifiable in the affected segment. Frozen section biopsies at the proximal, affected, and distal colon showed ganglion cells. Descending loop colostomy was constructed initially and segmental resection and end to end colocolostomy were carried out 3 months later. Final histologic examination showed 1) normal colonic mucosa with ganglion cells, 2) prominent submucosal fibrosis and marked muscular hypertrophy, 3) unremarkable acetylcholinesterase activity and immunohistochemical findings against S-100 protein. On 8 months follow-up, he has been doing well and moves bowels 1-2 times daily.

Torsion of the wandering spleen is a rare condition especially in children. Unfortunatly, splenectomy was the advocated treatment of choice for wandering spleen in many children up to 1985 in spite of the well-known possibility of overwhelming postsplenectomy sepsis. Because of the conclusive evidence of the vital function of the spleen in infection, indication of splenectomy have been revised and more attention has been paid to preserving the spleen where possible. We are presented a girl with splenic torsion, seen as a migratory abdominal mass and intermittent vague abdominal pain. Splenopexy by fixation of the spleen to the diaphragm and posterolateral peritoneum and the use of A vitene in the splenic bed was successfully performed. Complete fixation of the spleen was confirmed by ultrasound a year after this procedure was carried out. The clinical pesentation, etiology, diagnostic procedures and management are discussed also.

Two cases with papillary cystic neoplasm of the pancreas are reviewed and discussed. Up to recently, the tumors have been misclassified as nonfunctioning islet cell tumor or carcinoma, acinar cell carcinoma, papillary cystadenocarcinoma, or pancreatoblastoma. It frequently has been managed with aggressive surgery such as pancreatoduodenectomy. The tumors are well encapsulated and the cut surfaces are characteristically solid and hemorrhagic. Ultrasonography and CT scan are the most useful tools for the diagnosis. The neoplasms usually behave like a very low grade malignancy, so complete removal is the treatment of choice for the tumor arising anywhere in the pancreas. We have a boy and a girl who have papillary cystic neoplasm. The boy was 12 years old and the girl was 14 years old. Both underwent distal panereatectomy and the progress were uneventfuL We have a boy and a girl who have papillary cystic neoplasm. The boy was 12 years old and the girl was 14 years old. Both underwent distal pancreatectomy and the progress were uneventful.

Precocious puberty is difficult to define because of the marked variation in the age at which puberty begins normally, onset of puberty before 8 years of age in girls and 9 years in boys may be considered precocious. The etiology of precocious puberty in boys is usually idiopathic, but can result from adrenal and testicular tumors. The hepatoblastoma that produces hCG is a very rare functioning tumor known to cause precocious puberty in boys. Recently, author experienced one case of virilizing adrenal cortical adenoma in 22 month-old boy, one case of adrenal cortical carcinoma in 28 month-old boy, and one case of virilizing hCG-producing hepatoblastoma in 7 year-old boy and reviewed literatures.

Neuroblastoma is the most common solid malignancy in childhood. However, a neuroblastoma presenting as bilateral and multifocal is very rare. A male newborn weighing 2.7 kg was born by normal vaginal delivery at 39 weeks' gestation to a 27-year-old mother who had a normal pregnancy. He was in good condition at birth but presented palpable masses in the both upper abdomen and both side of the neck. Ultrasound examination showed the lesions to be 3 × 3cm sized calcified mass in right suprarenal area and 5 × 3cm sized homogenous mass in left suprarenal area. The abdominal mass was also examined by computed tomography with similar findings. With the impression of bilateral neuroblastoma or metastatic spread, the laparotomy was performed on the 13th day of life. Frozen section of biopsy of the left neck mass was obtained, which showed neuroblastoma. Intraoperative findings revealed bilateral adrenal masses which were distinct and anatomically separate. Bilateral adrenalectomy was accomplished and the initial postoperative course was unevenful. The patient was discharged for hopeless and expired at age 45 days. We present bilateral adrenal neuroblastoma considered to be simultaneous occurrence rather than metastases from one site to another.