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.

To determine whether bile juice exclusion can prevent the mucosal damage, and Insulin-like growth factor-I can promote mucosal regeneration in ischemia- reperfusion injury of the bowel, 39 weanling rats with 10 cm of Thiry-Vella loop were studied. Animal groups were; Control, BL(common bile duct ligation), IGF{insulin- like growth factor- I(IGF-I) infusion} and IGF-BL(combined treatment). IGF-I(1.5 mg/kg/day) was continuously delivered through a subcutaneously implanted miniosmotic pump. After 15 minutes of superior mesenteric artery clamping, a tissue specimen(P) was taken after 30 minutes of reperfusion. Intestinal continuity was restored to allow oral feeding. A specimen of main tract(M) and another of the Thiry-Vella 100p(T) were collected for histomorphometry after 48 hours of reperfusion and free feeding. Villus size ratio(VSR), crypt depth(CD), crypt-depth/villus-height ratio(CVR) and injury score(IS) were measured in 15 consecutive villi. The postoperative mortalities of bile duct ligation groups(BL and IGF-BL) were higher than those of other groups. In control group, VSR of M was lower(P<0.05) than P or T, but not in the other groups. VSR of M in conrol was lower than those in other groups. CD of T in control, IGF and IGF-BL group were higher than those of M. CD of M and T showed gradual increaments from control, IGF and IGF-BL group, respectively. CVR of M and T in IGF group were higher than those in control. CVR in IGF-BL group, T was higher than M, and M was higher than P. About IS, M of BL(20.1 ± 2.5) and IGF-BL(20.9 ± 3.3) groups were significantly lower than that of controI(32.4 ± 2.5). These results suggest that the exclusion of bile juice reduces the severity of the reperfusion injury of the mucosa, by inability to activate pancreatic enzymes and IGF-I stimulates mucosal regeneration in injured bowel, and the effect is potentiated by bile juice exclusion.

The anatomical variations of the external genitalia including the hymen were observed prospectively in 1,500 female infants born between March, 1992 and July, 1992 at the Taegu Fatima Hospital. Careful inspection of the external genitalia was done within 24 hours after birth, and abnormal findings photographed. Almost all hymenal tissue appeared to be redundant. The hymen was annulus in 89.1 % of patients. Ninteen point nine percent had a central orifice and the remainder a ventral orifice. External ridges, intravaginal ridges, and clefts were present in 71.5 %, 50.7 % and 40.5 %, respectively. Congenital anomalies of the external genitalia were found in 20 patients(1.4 %). There were sixteen cases of hymenal cysts, two hymenal polyps, one imperforate hymen, and one Skene's duct cyst. Seven of the 16 hymenal cysts with stalks were excised and those without stalk were aspirated. Hymenal polyps were excised. Imperforate hymen was incised and drained, and Skene's duct cyst was aspirated successfully. The surgical procedure were done without anesthesia, and the results have been good. In conclusion, routine physical examination of the female newborn infant detects frequent developmental anomalies. The significance of those anomalies will be clarified after longitudinal long-term followup studies.

Seventy neonates with congenital intestinal atresia and stenosis who were treated at pediatric surgical service, Hanyang University Hospital from September 1979 to December 1996 were analyzed retrospectively. The lesion occurred in 27 cases at the duodenum, in 26 cases at the jejunum, in 13 cases at the ileum and in 2 cases at the pylorus and colon each. There were 10 multiple atresias and 7 apple-peel anomaly cases. The atresia predominated over the stenosis by the ratio of 4 : 1. Male to female ratio was 1.3 : 1. The average gestational age was 38 weeks, and the average birth weight was 2,754 grams. Though 22.9 % were borne prematurely and 34.3 % had low birth weight, 92.3 % of them had a weight appropriate for gestational age. Polyhydramnios(40 %) was more frequently observed in duodenal and jejunal atresia while microcolon in ileal atresia(58.3 %). Weight loss and electrolyte imbalance occurred more frequently in the duodenal stenosis cases because of delayed diagnosis. Twenty(55.6 %) of 37 jejunoileal atresia cases had evidence of intrauterine vascular accident: 4 intrauterine intussusception, 3 intrauterine volvulus and 3 strangulated intestine in gastroschisis, and 10 cases of intrauterine peritonitis. There were one or more associated anomalies in 45 patients(64.3 %). Preoperatively proximal loop volvulus developed in 3 cases and proximal loop perforation in 5 cases and one case each of distal loop perforation, duodenal perforation and midgllt volvulus occurred in the jejunoileal atresia. Overall mortality rate was 20 %.

Reduction of intussusception using air or oxygen has wide acceptance as an alternative to conventional hydrostatic reduction. This study was undertaken to evaluate the results and complications of air pressure enema in 948 pediatric inc tussusception. One hundred and twenty nine cases were operated on at the Department of Surgery, Masan Samsung Hospital from 1985 to 1996 because of air reduction failure. The success rate was 86.4 %. Twenty-one patients(2.2 %) showed perforation during air reduction. Risk prone factors of perforation were; age less than 3 months(42.9 % vs 11.1 %), duration of symptoms greater than 48 hours(66.7 % vs 33.3 %), and presence of pathologic leading point(28.6 % vs 3.7 %). Vomitting and spontaneous rectal bleeding revealed higher prediction to the complication. In ninteen cases, bowel infarction, coagulated necrosis and hemorrhage suggested that the cause of perforation was due to the preexisting strangulation. In conclusion, when doing an air pressure enema reduction, care must be taken if the patient is of a young age or the symptoms are of long duration.

Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage. With the neoajuvant chemotherapy, improved resectability and survival have been reported. Twenty children with biopsy proven hepatoblastoma were treated during the period between January 1987 and June 1995. Median age at diagnosis was 13 months(2 months to 7 year and 10 months), and 13 were male. Histologic profile was 13 epithelia1(5 fetal, 4 mixed, 1 embryonal, 3 undetermined), and 5 mixed mesenchymal and epithelial and 2 of undetermined type. Chemotherapy effectively reduced the tumor volume(p=0.008), and' was able to convert 7 out of 9 initially unresectable cases(78%) to resectable ones. Twelve radical and 2 palliative operations were done with or without adjuvant chemotherapy. The Median follow up period was 33 months and the median survival was 26 months. The group with curative resection had a 61.1 % 5 year survival rate, but none of palliative resection grpup survived more than 13 months(p=0.000l). In univariate analysis for prognostic factors revealed, large tumor size at diagnosis and abscence of thrombocytopenia were associated with poor survival, but these differences were not statistically significant. Histological pure fetal type did not mean a better prognosis. Even with a recent neoadjuvant chemotherapy, the strategy should be focused on the radical resection as early as possible.

Bacillus Calmette-Guerin(BCG) lymphadenitis is a complication of post-BCG vaccination and its treatment is still controversial. Eighty patients who had been operated for BCG lymphadenitis from 1987 to 1996 were reviewed. Thirty-one of them were treated with antituberculous drugs(mean dunition: 3 months) preoperatively and 49 patients were not given preoperative antituberculous medication. No one was treated with antituberculous drugs postoperatively. Operation methods were excision(72) and partial excision with curettage(8). There was no statistical difference in recurrence rate between groups In two patients(2.5 %) treated with preoperative antituberculous drugs, the lesions recurred after operation. The results suggest that preoperative antituberculous medication does not play any role in the treatment of BCG lymphadenitis and in preventing recurrence in surgically excised cases.

The omphalomesenteric duct normally obliterates by the sixth week of intrauterine life. Incomplete obliteration results in various abnormalities which may be apparent in the newborn infant. These include fistula resembling ileum, a prolapsed loop of intestine through the umblicus and a fistula draining intstinal contents. The tract may contain ectopic tissue of stomach, colon or pancreas. Although this malformation should be recognizable at birth, 40 % of patients are not treated until after the first month of life. In the past 28 months since .the Samsung Medical Center opened its doors to public, the authors have treated 4 cases of patent omphalomesenteric duct remnant includng one case of T-shaped total prolapse of the duct and adjacent ileum. These cases will be discussed and the literature reviewed.

Transhiatal gastric transpositions were performed in two case of long gap esophageal atresia without tracheoesophageal fistula. The patients were a 12 months old female and an 18 months old male. Stamm type gastrostomies were performed at other hospitals in both cases. The stomach was mobilized preserving the right gastric artery, the right gastroepiploic artery and spleen. The proximal and distal esophageal pouches were excised by transcervical and transhiatal route, respectively. The mobilized stomach was pulled up to the neck through esophageal hiatus and posterior mediastinal route. The esophagogastrostomy, the only one anastomosis of this procedure, was performed in the neck. There was no clinical evidence of anastomotic leakage, stricture, regurgitation, difficulty of gastric emptying, hoarseness or respiratory problem. Transhiatal gastric transposition seems to be a safe and easy alternative surgical procedure for esophageal replacement in long gap esophageal atresia.

Three cases of laparoscopic splenectomy for hereditary spherocytosis are reported. The average operation time was 100 minutes. This was longer than traditional open splenectomy for the same entity( 63 minutes). Average hospitalization period was 3 days. This was shorter than the hospitalization period for the traditional group(6.2 days).

Conjoined twins are one of the rarest and most challenging congenital anomalies in pediatric surgery. Successful surgical separation is difficult because it the majority of conjoined twins in is technically typical to separate shared vital organs successfully. The timing of separation is variable, but separation is usually delayed until such infants are relatively mature(i.e, 9-12 months of age). Operative survival was 50 % in the neonatal period, but 90 % in those over 4 months of age. The present case was successfully separated early beacase of cardiac problems in one of the twins. These twins were omphalopagus and only the liver was shared through a bridge. The vascular and biliary trees were independent from each other. Successful surgical separation was on 11th day of life, but one died of sepsis 18 days ofter operation.

Appendiceal perforation is uncommon in the neonate. Diess reported the firs case in 1908. Approximately HI additional cases have been reported since that time. However, with exclusion of neonatal appendicitis' associated with inguinal or umbilical hernias, n,ecrotizing enterocolitis, meconium plug, and Hirschsprung's disease, there are only 36 cases of primary neonatal appendicitis. We treated a 12 days old boy with perforation of the appendix. The infant was 3000 g at birth and had a normal spontaneous vaginal delivary at 35 weeks of gestation. The mother was 31-year-old and had premature rupture of membrane. After normal feeding for the first 5 days of life, the infant had emesis of undigested milk, decreased activity and jaundice. The baby was admitted to the Pediatrics. Progressive abdominal distension, fever, decreased activity, and vomitting developed over the next six days. Erect abdominal radiography showed pneumoperitoneum. At exploratory laparotomy, a 0.8 × 0.6 em sized perforation was noted at antime-senteric border of midportion of the appendix. Trasmural inflammation and the presence of ganglion cells were noticed on histology.