To evaluate the long-term prognosis of biliary atresia after Kasai operation, a total of 14 patients (of the 41 patients operated upon from 1982 to 1997), who had been followed up for more than 10 years, were included in this retrospective study. Eleven out of 14 patients survived with their native livers, and their data analyzed for age at operation, clearing time of jaundice, histological outcome, postoperative complications, effectiveness after the application of an intussusception anti-reflex valve, and quality of life. Average age at surgery was 62.8 days. Serum bilirubin was normalized within three months in all patients. Six among the eleven long-term survivors had ascending cholangitis as one of the postoperative complications. The application of an intussusception anti-reflux valve did not show any statistical significance in long-term survival. Most of long-term survivors appeared to enjoy good quality of life. Kasai operation might not be the definitive treatment for biliary atresia; however, Kasai operation made it possible to achieve long-term survival for patients with biliary atresia when the patients were detected and treated as early as possible.

The aim of this study is to analyze the outcomes of the esophageal atresia with tracheoesophageal fistula over the last 2 decades. The records of 51 patients born between 1987 and 2006 were reviewed. Twenty-seven patients were male. Mean values of the age, gestational age and birth weight were 2.9 days, 296 days and 2.7kg, respectively. All patients had Gross type C anomalies. Thirty-one patients (60.7 %) had one or more associated congenital anomalies and the most common anomaly was cardiac malformation. In 48 cases, primary anastomosis was done and staged operation was done in one case. Circular myotomies in the proximal esophagus were performed in 9 cases. Postoperative complication developed in 26 cases (54 %): pulmonary complication in 12 cases, anastomotic leakage in 10 and anastomotic stricture in 10, recurrent trachoesophageal fistula in one and tracheomalasia in 2 cases. Reoperation was carried out in 2 patients with anastomotic leaks, the remaining leaks were managed non operatively. Three of the strictures were reoperated upon and the others were successfully managed by balloon dilatations. Overall mortality rate was 15.6 %. Mortality rate of the second 10 years (8 %) period decreased significantly compared to that of the first 10 years (23 %) period.

A vitelline duct (VD) anomaly is a relatively common congenital abnormality of the umbilical area. The anomalies include patent vitelline duct (PVD), cyst, fistula or sinus. The incidence is approximately 2% of the populations, but development of symptoms is rare. Recently, we experienced two cases; PVD accompanied by a smallomphalocele and intestinal volvulus due to mesenteric band between Meckel's diverticulum and the mesentery. Thereafter,we evaluated the data of vitelline duct anomalies for 27 years. From 1980 to 2006, 18 cases of VD anomalies were reviewed based on the hospital records retrospectively. There were 15 boys and 3 girls and age ranged from 2 days to 15 years. Among the 18 cases, 15 cases were symptomatic and consisted of Meckel's diverticulum (10 cases), PVD (4 cases) and umbilical polyp (1 case). Three asymptomatic cases of Meckel's diverticulum were found incidentally were and were observed without resection. Ten cases of Meckel's diverticulum were presented with intestinal bleedings (4 cases), intestinal obstructions (5 cases) and perforation (1 case). Wedge resections and segmental resections of ileum were performed in 8 patients and 2 patients, respectively. Postoperative complications were adhesive ileus (1 case) and wound seroma (1 case). Small omphaloceles were accompanied in two of 4 PVD patients. There was 1 small omphalocele case which was accompanied by a prolapse of ileum. In summary, VD anomalies were more common in male and more than half of them were found in patients less than 1 year of age. PVD was diagnosed most frequently in neonates. Meckel's diverticulum presented with intestinal obstruction more frequently than bleeding.

Tapering enteroplasty was first described by Thomas in 1969 as one method of intestinal anastomosis. The advantages of tapering enteroplasty in the intestinal atresia are: First, it makes end-to-end anastomosis possible between the atretic bowel ends with considerable differences in diameters. Second, it promotes the recover of the postoperative bowel function. Third, it prevents the possibility of the short bowel syndrome by eliminating the need of resecting the dilated bowel. A total of 22 patients with intestinal atresia who underwent tapering enteroplasty from January 1988 to December 2005 at our institute were reviewed. In 3 of 22 cases, tapering enteroplasty was the 2^nd operation after an initial end-to-oblique anastomosis. We reviewed the following items: age, sex, type and location of intestinal atresia, initial feeding and total enteral feeding start day, the length of hospital stay and complications. The average age of the patients was 7 days. Male to female ratio was 1 to 1.2 (10 cases: 12 cases). We performed the tapering enteroplasty on all types and locations of the intestinal atresia from the duodenum to the colon: type I (n=3), type II (n=4), type IIIA (n=7), type IIIB (n=5), type IIIB and IV (n=1), type IV (n=1) and type C (duodenum) and type IIIB and IV (jejunum). On the average, the oral feeds were started on the postoperative 8.8^th day, and full caloric intake via the enteric route was achieved on postoperative 13.3^th day. The average length of hospital stay was 19.6 days. There were 1 case (4.5 %) of anastomotic complication and 2 cases (9 %) of adhesive ileus among 22 patients. The tapering enteroplasty on all types of intestinal atresia is a usefull operative method when there are considerable diameter differences between the atretic bowel ends.

Inguinal hernia is the most common disease treated by the pediatric surgeon. There are several controversial aspects of management 1)the optimal timing of surgical repair, especially for preterm babies, 2)contralateral groin exploration during repair of a clinically unilateral hernia, 3)use of laparoscope in contralateral groin exploration, 4)timing of surgical repair of cord hydrocele, 5)perioperative pain control, 6)perioperative management of anemia. In this survey, we attempted to determine the approach of members of KAPS to these aspects of hernia treatment. A questionnaire by e-mail or FAX was sent to all members. The content of the questionnaire were adapted from the “American Academy of Pediatrics (AAP) Section on Surgery hernia survey revisited (J Pediatr Surg 40, 1009-1014, 2005)”. For full-term male baby, most surgeons (85.7 %) perform an elective operation as soon as diagnosis was made. For reducible hernia found in ex-preterm infants already discharged from the neonatal intensive care unit (NICU), 76.2 % of surgeons performed an elective repair under general anesthesia (85.8 %). 42.9 % of the surgeons performed the repair just before discharge. For same-day surgery for the ex-premature baby, the opinion was evenly divided. For an inguinal hernia with a contralateral undescended testis in a preterm baby, 61.9 % of surgeons choose to ‘wait and see’ until 12 month of age. The most important consideration in deciding the timing of surgery of inguinal hernia in preterm baby was the existence of bronchopulmonary dysplasia (82.4 %), episode of apnea/bradycardia on home monitoring (70.6 %). Most surgeons do not explore the contralateral groin during unilateral hernia repair. Laparoscope has not been tried. Most surgeons do not give perioperative analgesics or blood transfusion.

Hereditary pancreatitis (HP) appears as an autosomal dominant trait. If the patient has (1) more than 2 affected relatives in different generations and (2) no known etiological factors such as alcohol or gallstones, or has R122H or N29I mutation in the cationic trypsinogen (CT) gene, the diagnosis of HP can be applied. Risk of pancreatic cancer is estimated to be 53-fold higher than in a general population after the age of 50 years. We report a kindredof HP, involving three of its family together; two siblings (14 years old, 13 years old) and cousin (26 years old). The patient had complicating chronic pancreatitis and pancreatic stone, and was treated with amodified Puestow-Gillesby procedure. Her sisters showed chronic pancreatitis. Her cousin underwent a drainage procedure of the pancreatic duct for chronic pancreatitis during the high school period. All the three members showed the R122H mutation of the CT gene.

Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) is a rare disease. It has been generally recognized as type I (correctable with cystic dilatation), which means a good prognosis. From a total of 73 patients with BA who underwent operation from September 1988 to September 2003 at our institute, 7 (9.6 %) cases of type III BA with EHBC (uncorrectable with cystic dilatation) are reviewed. Clinical findings, laboratory data, radiologic findings, treatment methods and outcomes were reviewed. Female was more prevalent (male to female ratio; 2:5). All cases were type III with EHBC according to the intraoperative cholangiography, and underwent Kasai's portoenterostomy. The mean age was 57 days at operation. Three patients(42.9 %) are long term survivors. Further evaluation is needed to determine the correlation between prognostic factors and outcome for.

Appendectomy is the most common emergency surgical operation in children. The laparoscopic treatment of pediatric appendicitis is controversial, particularly in complicated cases. The purpose of this study is to evaluate laparoscopic appendectomy (LA) and open appendectomy (OA) for simple and perforated appendicitis (SA, PA) in children. A total of 188 patients, operated from January 1992 to September 2003, were reviewed. Ninety-one patients underwent OA (65 SA and 26 PA) and 97 had LA ( 67 SA, 30 PA). There was one conversion of LA to OA in PA. Operative time for LA was longer for OA in PA (55.8 vs. 45.7 min; p=0.0467). Recovery of bowel movement, diet starting time, length of hospital stay for LA were significantly shorter than those for OA. Postoperative complication rate was not different between LA and OA in each group. Laparoscopic appendectomy is a safe and effective method for both simple and perforated appendicitis.

Diagnosis of acute appendicitis in children is sometimes difficult. The aim of this study is to validate a clinical scoring system and ultrasonography for the early diagnosis and treatment of appendicitis in childhood. This is a prospective study on 59 children admitted with abdominal pain at St. Mary's Hospital, the Catholic University of Korea from July 2002 to August 2003. We applied Madan Samuel's Pediatric Appendicitis Score (PAS) based on preoperative history, physical examination, laboratory finding and ultrasonography. This study was designed as follows: patients with score 5 or less were observed regardless of the positive ultrasonographic finding, patients with score 6 and 7 were decided according to the ultrasonogram and patients above score 8 were operated in spite of negative ultrasonographic finding. The patients were divided into two groups, appendicitis (group A) and non-appendicitis groups (group B). Group A consisted of 36 cases and Group B, 23 cases. Mean score of group A was 8.75 and group B was 6.13 (p<0.001). Comparing the diagnostic methods in acute appendicitis by surveying sensitivity, specificity, positive predictive value, negative predictive value, and accuracy, PAS gave 1.0000, 0.3043, 0.6923, 1.0000, and 0.7288, and ultrasonography gave 0.7778, 0.9130, 0.9333, 0.7241, and 0.8300 while the combined test gave 1.0000, 0.8696, 0.9231, 1.0000, and 0.9490, respectively. Negative laparotomy rate was 3 %. In conclusion, the combination of PAS and ultrasonography is a more accurate diagnostic tool than either PAS or ultrasonography.

A newborn male baby was transferred to our hospital with a left inguinal mass. The mass was huge measuring 10 x 10 cm, engorged, and dark-blue colored as a result of internal hemorrhage. Unstable vital signs were combined with DIC and acute renal failure. Emergency operation was performed because of the suspicion of bowel perforation. The peritoneal cavity was full of ascitis and the distal jejunum had a 0.5 cm perforation. Segmental resection of the jejunum and incisional biopsy of the inguinal mass were performed. On pelvic and thigh MRI, the mass protruded into pelvic cavity and encircled large vessels and nerves of the thigh. Pathologic diagnosis was congenital infantile fibrosarcoma. Fifteen days after operation, primary tumor excision was undertaken. The second look operation, performed after 6 times VAC chemotherapy, revealed no remained malignant cell on microscopic section. The baby has been followed closely for the last eight months.

Diffuse cavernous hemangioma of the rectosigmoid is a rare lesion usually presented in children and young adult, often with life threatening hemorrhage. The clinical diagnosis is difficult and often delayed because of lack of awareness of the clinical features. We report a case of diffuse cavernous hemangioma of the rectosigmoid in a 12-year-old boy who had undergone suture ligation under the impression of hemorrhoid at his age 3. Sphincter-saving coloanal pull through procedure were applied as the surgical treatment. Sclerotherapy was needed with recurrent rectal bleeding 6 months after the pull through operation. The patient is well at 12 months follow-up.

We present a case of a colonic involvement associated with necrotizing pancreatitis, with a review of the literature. A 10 year old boy had an appendectomy at the local clinic ten days ago. On admission, he complained nausea, vomiting and severe constipation. His abdomen was distended and he had tenderness on the left abdomen. Laboratory and radiologic studies revealed findings consistent with acute pancreatitis with colonic complication. He was treated conservatively for 30 days but did not improve. On hospital 30th day, abdominal pain developed and his vital sign changed. Abdominal CT suggested ischemic change of the transverse colon. At laparotomy, the left colon showed stenosis. The greatly distended transverse colon was resected and a transverse end colostomy was done. He was discharged at postoperative 45th day with improvement and colostomy closure was performed 8 months later.

When jaundice persists for more than 14 days postnatally, the early diagnosis of surgical jaundice is important for the prognosis in extrahepatic biliary atresia after draining procedure. The role of diagnostic laparoscopy to differenctiate medical causes of jaundice from biliary atresia is evaluated in this report. Four patients with prolonged jaundice have been included in this study. When the gallbladder was not visualized we proceeded to laparotomy. In patients with enlarged gallbladder visualized at laparoscopy, laparoscopic guided cholangiogram was performed, and laparoscopic liver biopsy was done for those who had a patent biliary tree. Two patients had small atretic gallbladder and underwent a Kasai hepato-portoenterostomy. One patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a Kasai hepatic-portoenterostomy. One patient showed an enlarged gallbladder and laparoscopic-guided cholangiogram were normal. Laparoscopic liver biopsy was performed. There were no complications. Laparoscopy wth laparoscopic-guided cholangiogram may be a valuable method in accurate and earlier diagnosis in an infant with prolonged jaundice.

Mesenteric and omental cysts are rare intra-abdominal lesions in childhood, and may present various clinical features such as an asymptomatic mass or an acute abdomen. Therefore, these entities are frequently misdiagnosed preoperatively or are found only incidentally at operation for other conditions. We analyzed our experiences of 19 cases in a 19 year period from 1981 to 1999, at College of Medicine, Catholic University of Korea. There were 12 boys and 7 girls with a mean age of 4.8 years (range, 3 days to 15 years). Common presenting symptoms were abdominal pain (47%), abdominal distension (31%), abdominal mass (24%), vomiting (15%) and fever (10%). Ultrasonography was the most preferred method of diagnosis. Other diagnostic modalities include CT, MRI, and abdominal ascites tapping in selected patients. Location of the mesenteric cysts was small bowel mesentery in nine, the right mesocolon and retroperitoneum in one, the left mesocolon in one, and the jejunum, sigmoid-colon mesentery in one. Most of the patients underwent cyst excision, but six patients required concomitant bowel resection for complete removal of the lesions, and two patients underwent unroofing and simple aspiration respectively. There was one mortality case due to sepsis.

Childhood intussusception is usually idiopathic, and pathological lesions as the leading point are found in limited cases. Sixteen operative cases with leadpoints among 2,889 cases of childhood intussusecption treated at the surgical departments of the affiliated hospitals of Catholic University over 19 years are reviewed. The approximate incidence of pathological lesions as the leading point was 0.6%. The male to female ratio was 2:1. The mean age was 3.5 years. There was not an age preponderance. The symptoms were vomiting (63%), abdominal pain (38%), irritability (38%), bloody stools (25%), fever (25%) and abdominal mass (6%). The average duration of the symptoms was 2.4 days (1-10days). The most common lesion was Meckel's diverticulum, followed by malignant lymphomas, polyps, ectopic pancreas, and cecal duplication. An ileocolic type was most frequent, followed by ileoileocolic and ileoileal. Segmental resection or wedge resection of the ileum was done in 10 cases, ileocecectomy in 3, and right hemicolectomy in 3. Surgical reduction was done only in an ectopic pancreas, with no later recurrence. The average hospital stay was 10 days. Postoperative adhesive ileus occurred in two cases, and in one of them adhesiolysis was performed. One case of malignant lymphoma died at 28 days after surgery due to chemotherapy related complication.

Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years. The vast majority are in favorable stages of the disease (stage I, II, IV-S). The authors experienced one case of stage IV-S neuroblastoma of the adrenal gland with liver metastasis, which regressed spontaneously after removal by adrenalectomy. This patient was noticed to have an abdominal mass at prenatal ultrasonography performed at 36weeks of gestation. This tumor was a neuroblastoma of the left adrenal gland with multiple liver metastases. Left adrenalectomy and liver biopsy were performed at 3 months of age. Thirty-eight months after surgery, an MRI demonstrated that the hepatic metastatic lesions had completely regressed without chemotherapy or radiation.

Beckwith-Wiedemann syndrome presents with multisystemic patterns of congenital anomalies and macrosomia. This syndrome was independently described by Beckwith in 1963 and by Wiedemann in 1964. There is wide spectrum of clinical manifestations, including prenatal or postnatal overgrowth, neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, hemihypertrophy and a predisposition for embryonal tumors, most frequently Wilms' tumor. We managed a case of Beckwith-Wiedemann syndrome with left adrenal cortical neoplasm of undetermined malignancy.

Persistent cloaca in a female newborn is one of the most complex and challenging developmental malformations. The incidence is about 10% of all anorectal anomalies. Treatment of cloacal malformations has evolved during the past 40 years; however, it still remains one of the most difficult operations. In 1997, Pe a reported that a new technical variant called "Total urogenital mobilization" We also repaired cloacal anomaly using “Total urogenital mobilization” in 3 patients. The operations were performed between age 15- and 28-month. The length of the common channels was 2.5-3.0 cm. Two cases had double vaginas and one of them also had double uterus. The operation time was 4-5 hours and no major complications occurred. A satisfactory functional and cosmetic results were obtained.

Pediatric laparoscopic splenectomy has gained increasing acceptance in the surgical management of a variety of splenic disorders, in particular hematologic diseases. We report our experience with 16 patients who underwent this procedure because of hematologic disorders during the past 3 and a half years at the Department of Surgery, St. Mary's Hospital, Catholic University Medical College. The mean age was 10 years (Range 6-16 years) and the mean spleen weight was 210 gm (Range 85-500 gm). The indications for splenectomy were hereditary spherocytosis (6 cases), idiopatic thrombocytopenic purpura (8 cases), autoimmune hemolytic anemia (1 case), and idiopatic splenomegaly (1 case). All splenectomies were performed safely with mean estimate blood loss of 233 ml. Mean operative time and mean postoperative hospital stay was 157 min and 4.5 days. Respectively postoperative pain medication was needed in 3 case, just one injection in immediate postoperative period. Diet was started on posterative second day or third day. In conclusions, Laparoscopic splenectomy in pediatric patients surely is a safe procedure, offering better cosmesis, much less pain, and shorter hospital stay with lower post operative mobidity.

Choledochal cyst is rare in the western countries, but common in oriental countries. Complicatioins include ascending cholangitis, recurrent pancreatities, progressive biliary cirrhosis, portal hypertension, stone formation and later malignant transformation. Bile peritonitis secondary to rupture is one of the rarest complications, with an incidence of 1.8 % to 18 %. The anomalous arrangement of the pancreatobiliary ductal system with a long common channel may cause inflammation leading to perforation of the cyst. The authors found 4 cases (14.2 %) of bile peritonitis among 28 cases of choledochal cyst treated from Jan. 1983 to Jan. 1998. The patients ages ranged from 6 months to 3 years and three were female. The perforation sites were located on the common bile duct at its junction with the cystic duct in 2 cases, the distal cyst wall in 1 case and the left hepatic duct at its junction with cyst in 1 case. The types of choledochal cysts by Todani's classification were Type IVa in 3 cases and type I in 1 case. By the new Komi's classification utilizing operative cholangiogram there were 2 cases of Type Ia, 1 case of type IIb and 1 case of type III. One stage cyst excision and hepaticojejunostomy(Roux-en Y type) was done in 3 cases, and two staged operation in 1 case. All patients had an uneventful course postoperatively. The average day of discharge was 9.8th postoperatively. In conclusion, primary excision of the choledochal cyst and biliary reconstruction is a safe and effective treatment of ruptured choledochal cyst in infants.

Meconium peritonitis is a primarily aseptic, chemical peritonitis caused by the spill of meconium into the abdominal cavity through an intestinal perforation during the intrauterine or perinatal period. The perforation is known to be related to intrauterine vascular compromise. Recently, the authors experienced 4 cases of ileal atresia complicated by meconium peritonitis. The male to female sex ratio was 1 : 3, and age at operation was 1 day(2 cases), 3 days(2 cases). Three cases had generalized peritonitis, and one the cystic type of meconium peritonitis. The types of ileal atresia were IIIa(2 cases), IIIb(1 case), and II(1 case). The proximal blind ileal end was perforated in 3 cases, and distal end perforation was in 1 case of cystic type. Postoperative recovery was excellent in all cases.

Children tend to ingest foreign bodies. The majority present in children between the ages of 6 months and 3 years. We experienced 2 cases of unusual gastrointestinal complications caused by ingested foreign bodies. First case was a 10-month-old male with intestinal perforation due to two pieces of ingested magnetic beads. Second case was a 7-month-old girl with esophageal stricture due to an ingested particle of plastic toy.

Segmental intestinal dilatation is rare, which causes symptom of bowel obstruction and requires resection. The resection is not only diagnostic but also curative procedure. Recently, author experienced 2 cases of segmental dilatation of the ileum due to focal agenesis of the intestinal muscularis in 7 day & 4 day-old female neonates. The postoperative recovery was excellant in the first case after resection of dilated ileum(l5cm in length) and end to end anastimosis, and discharged at 20th day. But in the second case, the passage disturbance was not relieved after resection of dilated ileum(30cm in length), and author re-resected 80cm more of dilated proximal ileum at 2 weeks after the first operation. This baby discharged after diarrhea control with Loperin on I month after the second operation.

Final histologic examination showed I) normal population of ganglion cells in both narrowed & dilated ileum in both cases, 2) focal abscence of muscularis propria in both cases, 3) relative hypertrophy of inner circular muscle layer and thinned, multiple fragmented outer longitudinal muscle layer in case 2.

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.

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.