Neuroblastoma is the most common extracranial solid tumor in children. We retrospectively analyzed the results of neuroblastoma treatment of 191 patients (116 males and 75 females) treated between January 1986 and December 2005 at the Department of Pediatric Surgery and the Department of Pediatrics, Seoul National University Children's Hospital. The mean age at diagnosis was 3.1 years (0.1 yrs - 13.5 yrs). Forty-seven patients were under 1 year of age. The mean follow-up period was 57.3 months (24 days - 19.1 yrs). Patients were classified into two groups according to the completeness of resection of the primary tumor; (1) gross total resection (GTR) and (2) incomplete resection (IR). The number of patients in stages I, II, III, IV, IV-S were 17 (8.9 %), 12 (6.3 %), 43 (22.5 %), 114 (59.7 %), 4 (2.1 %), respectively. GTR was achieved in 120 patients and IR in 71 (22 stage III, 47 stage IV, 1 stage IV-S, 1 brain). Overall survival (OS) was 65.2 % and event-free survival (EFS) was 48.6 %. EFS were 100 %, 75 %, 66.8 %, 31.3 %, 75 % at stage I, II, III, IV, IV-S, respectively. There was no significant difference in EFS according to the completeness of resection. EFS was improved in GTR group (p=ns) of stage III, but by contrast, stage IV patients showed worse EFS in GTR group. EFS was improved significantly after the introduction of autologous stem cell transplantation (ASCT) (58.1% vs. 40.6%, p=.029). The EFS improved significantly after the introduction of ASCT in IR group (p=.009) rather than GTR group (p=ns). The EFS of the patients under 1 year of age (N=47) was better than the patients over 1 year of age (N=144) significantly (75.5 % vs. 39.4 %, p=.0034). The prognosis of neuroblastoma was related to the INSS stage and age at diagnosis. The survival of IR group significantly improved after ASCT.

The survival of Very Low Birth Weight (VLBW) infants has been improved with the advancement of neonatal intensive care. However, the incidence of accompanying gastrointestinal complications such as necrotizing enterocolitis has also been increasing. In intestinal perforation of the newborn, enterostomy with or without intestinal resection is a common practice, but there is no clear indication when to close the enterostomy. To determine the proper timing of enterostomy closure, the medical records of 12 VLBW infants who underwent enterostomy due to intestinal perforation between Jan. 2004 and Jul. 2007 were reviewed retrospectively. Enterostomy was closed when patients were weaned from ventilator, incubator-out and gaining adequate body weight. Pre-operative distal loop contrast radiographs were obtained to confirm the distal passage and complete removal of the contrast media within 24-hours. Until patients reached oral intake, all patients received central-alimentation. The mean gestational age of patients was 26⁺² wks (24⁺¹~33⁺⁰ wks) and the mean birth weight was 827 g (490~1450 g). The mean age and the mean body weight at the time of enterostomy formation were 15days (6~38 days) and 888 g (590~1870 g). The mean body weight gain was 18 g/day (14~25 g/day) with enterostomy. Enterostomy closure was performed on the average of 90days (30~123 days) after enterostomy formation. The mean age and the mean body weight were 105 days (43~136 days) and 2487 g (2290~2970 g) at the time of enterostomy closure. The mean body weight gain was 22 g/day after enterostomy closure. Major complications were not observed. In conclusion, the growth in VLBW infants having enterostomy was possible while supporting nutrition with central-alimentation and the enterostomy can be closed safely when the patient's body weights is more than 2.3 kg.

Sialoblastoma is a rare tumor of salivary gland origin, developing in the fetal or neonatal period. Most tumors arise in parotid gland, and rarely in submandibular gland. Because of its rare incidence, diagnosis of the sialoblastoma in head and neck tumors of children is not easy. The case of a congenital submandibular gland sialoblastoma is presented. A neonate was transferred right after birth due to a submandibular mass. CT scan showed a lobulating mass located posterior to the left submandibular gland, suggesting neurogenic tumor or myofibroma. The tumor was excised easily after division of a duct-like structure connecting with the submandibular gland. The microscopic findings showed the basaloid cells and ductules forming cellular nests, separated by thin fibrous tissue. Immunohistochemical staining was positive for cytokeratin, vimentin and Ki-67, which was consistent with sialoblastoma.

The survival rate for rhabdomyosarcoma (RMS) has significantly improved after the introduction of combined multimodality treatment. We report the 20-year treatment outcome of pediatric rhabdomyosarcoma in a single institution. The medical records of 16 patients treated for rhabdomyosarcoma between December 1986 and August 2007 at the Department of Pediatric Surgery, Seoul National University Children's Hospital, were retrospectively reviewed. Mean age at diagnosis was 7.1 years (range: 1.3-14.2 years). Retroperitoneum was the most common primary site (n=7, 43.8%), and embryonal type was predominant (n=11, 6%). Before the treatment, most patients were in advanced TNM stage (stage III 50%, IV; 25%). The patient distribution according to the Intergroup Rhabdomyosarcoma Study Clinical Grouping System (IRS-CGS) was as follows; Group I 31.3%, Group II 12.5%, Group III 31.3% and Group IV 25%. Patients were classified into three groups according to the extent of resection of the primary tumor; complete resection (CR, n=5; 31.3%), gross total resection (GTR, n=7; 43.8%) and incomplete resection (IR, n=4; 25%). Recurrence was observed in 9 patients (56.3%) while there was no recurrence in CR patients. All patients with recurrence were identified as moderate or high-risk according to the IRS-V Risk Group. Pre-treatment TNM stage of RMS in our institution was advanced with aggressive clinical feature, however post-surgical conditions according to IRS-CGS were similar to the previous reports by IRS. This suggests that down-staging of IRS-CGS was achieved with multimodality treatment with CR or GTR. It also suggests that complete resection is the most important prognostic factor in the treatment of RMS in children. Patients classified as moderate or high-risk need close follow-up due to high recurrence rate. In case of localized recurrence, better outcome may be achieved with multimodality treatment including limited surgery.

Inflammatory myofibroblastic tumor (IMT) is a rare reactive lesion characterized by the feature of myofibroblasts and a mixed inflammatory infiltrate that rarely undergoes malignant transformation. Extrapulmonary IMTs in children have been described involving the mesentery, omentum, retroperitoneum, abdominal soft tissues, liver, bladder, mediastinum, head and neck, extremity, appendix, and kidney. Medical records of children treated with abdominal IMT between 1985 and 2005 were reviewed retrospectively. Seven children were treated for IMT with the mean age of 3y 2m (range, 1y 1m to 14y). Tumors were located in transverse mesocolon (n=2), omentum (n=1), porta hepatis (n=2), complex site (antrum, duodenum, common bile duct, porta hepatis) (n=2). The symptoms included abdominal mass, fever, jaundice, abdominal pain and anemia. The masses were excised totally in transverse mesocolon, omentum IMT and there is no evidence of recurrence (follow-up periods: 6y 8m, 8y 9m, 4y 10m). In porta hepatis IMT, liver transplantations were performed and there is no evidence of recurrence (follow period: 6y 8m, 8y 7m). In one case of complex site IMT, partial excision of mass was performed and he still survived with no change of the residual tumor during follow-up period. The other one of complex site IMT denied further treatment after the biopsy. In conclusion, complete surgical excision including liver transplantation and close follow-up are mandatory for the abdominal IMT in child.

The usefulness of totally implantable central venous port for long-term intravenous infusion is widely accepted in children. Usually the catheters are placed through the internal or external jugular vein. In case of jugular vein cut-down, two separate incisions are needed for catheter and port respectively. Patients also feel uncomfortable as the catheter run through the neck. However these disadvantages can be overcome by using the cephalic vein (CV). We reviewed our experiences on CV cut-down for totally implantable central venous port in children. From January 2002 to December 2006, 201 patients (M:F=127:74) underwent 218 central venous port insertions. Mean age at operation was 5.9 years (2 months - 19 years). Indications included chemotherapy (N=167), long-term intravenous antibiotics infusion (N=36), and total parenteral nutrition (N=15). CV was selected preferentially. The incision includes the deltopectoral triangle laterally, and both the CV cut-down and port insertion were achieved with a single incision. The number of insertion through external, internal jugular vein, and CV was 77, 66 and 75, respectively. The median age, height and body weight were higher in CV cut-down group. The youngest age for CV cut-down was 8 months, the shortest height was 69 cm and the smallest body weight was 5.9 kg. Of 118 trials of CV cut-down, cut-down was successful in 75 cases (63.6 %). CV was absent in 10 cases(8.4 %) and CV was sacrificed after catheter tip malposition in 10 cases (8.4 %). There was only one complication, in which the catheter was inserted into the minute branch of subclavian artery. The CV cut-down method for totally implantable central venous port was safe and feasible in selected groups of patients in children. In addition, preservation of jugular vein and a more favorable cosmetic effect are other benefits of CV cut-down.

Migration of a peritoneal catheter of a ventriculoperitoneal shunt into the scrotum is a rare complication. We treated a case of catheter migration in the scrotum. A 12-year old boy, who had had a ventriculoperitoneal shunt at the age of 4 months due to neonatal hydrocephalus, visited the outpatient clinic because of a right inguinal hernia. On physical examination, a firm mass was found in the left scrotum. Pelvic X-ray demonstrated a coiled catheter in the left scrotum. The catheter was successfully removed by exploring the left patent processus vaginalis after high ligation of the hernia sac. This case suggests a suction action of the patent processus vaginalis and the possibility of catheter migration long after shunt catheter insertion.

Adrenocortical tumors are very rare in children and the clinical course is not clearly understood. The aim of this study is to review the clinical characteristics and courses of pediatric adrenocortical tumors. The medical records of patients who underwent surgery for primary adrenal tumor at the Department of Surgery, Seoul National University hospital, from Jan. 1986 to Feb. 2006 were reviewed. There were 10 adrenocortical tumor patients; 5 had adrenocortical adenoma and 5 adrenocortical carcinoma. All of the adrenocortical adenomas presented as functioning tumors, i.e., Cushing syndrome or virilization. However, only 2 patients had functioning adrenocortical carcinoma. Median size of adenoma was 5 (3.3–6) cm, and carcinoma 12.5 (6.5–13)cm. Adenomas were smaller than 6 cm and carcinomas were larger than 6.5cm. Surgical resection alone cured all adrenocortical adenoma patients, and they were all alive without recurrence. Three of 5 adrenocortical carcinoma patients died of tumor recurrence despite radical surgery and chemotherapy. There were 2 long-term survivals for adrenocortical carcinoma, one patient survived 10 years without recurrence until he died of newly developed osteosarcoma, and the other patient is alive without recurrence for 20 years. As the prognosis of pediatric adrenocortical carcinoma is poor, peri-operative aggressive chemotherapy is suggested in addition to radical surgery.

We reviewed the records of 25 patients who were re-operated upon after primary repair of esophageal atresia with or without fistula at the Department of Pediatric Surgery, Seoul National University Children's Hospital, from January 1997 to March 2007. Types of the esophageal atresia anomalies were Gross type A in 5 patients, C in 18, and E in 2. The indications for re-operation were anastomosis stricture (n = 14), tracheo-bronchial remnant (n = 4), persistent anastomosis leakage (n = 3), recurrent tracheo-esophageal fistula (n = 2) and esophageal web (n = 2). The interval between primary and secondary surgery was from 48 days to 26 years 5 months (mean: 2 years and 4 months). Four patients required a third operation. The interval between the second and third operation was between 1 year 1 month and 3 year 10 month (mean: 2 years 5 months). Mean follow up period after last operation was 35 months (1 years–8 years 6 months). The secondary surgery was end-to-end esophageal anastomosis in 15, esophagoplasty in 5, gastric tube replacement in 5. After secondary operation, 6 patients had anastomosis stricture (4 patients were relieved of the symptoms by balloon dilatation, 2 patients underwent tertiary operation). Five patients had leakage (sealed on conservative management in all). Two patients had recurrent tracheo-esophagel fistula (1 patient received chemical cauterization and 1 patient underwent tertiary operation). Currently, only one patient has feeding problems. There were no mortalities. Secondary esophageal surgery after primary surgery for esophageal atresia was effective and safe, should be positively considered when complications do not respond to nonoperative therapy.

Abnormal distribution of enteric nerves such as adrenergic, cholinergic and non-adrenergic non-cholinergic nerves (NANC) may cause the failure of relaxation at the involved bowel segment in Hirschsprung's disease (HD). Nitric oxide (NO) is a major inhibitory NANC neurotransmitter in the gastrointestinal tract. NO is synthesized by activation of nNOS (neuronal nitric oxide synthase) in the intramural ganglion cells and regulates bowel movement. To assess the distribution of nNOS in HD, immunohistochemical staining to nNOS was utilized on paraffin embedded specimens. Ten control colon specimens were tested for feasibility of staining. Immunohistochemisrty was done on ganglionic colon as well as aganglionic segment of 15 patients with HD. nNOS immunoreactivity was observed in the neuronal cells, small cells and nerve fibers in the muscle layer and submucosal neuronal cells of control specimens. This finding was also observed in the ganglionic segments of HD. But, there was no nNOS immunoreactivity in aganglionic segments of HD. In conclusion nNOS immunohistochemical staining of paraffin embedded specimen is feasible and reliable. And the results suggest that the relaxation failure of the aganglionic bowel in HD is related to the absence of nNOS containing cells and nerve fibers.

Solid pseudopapillary tumor of pancreas in children is a tumor with low malignant potentiality, and is rarely associated with distant metastasis. A 13-year-old girl was hospitalized because of abdominal pain of one week duration. Abdominal CT revealed not only a 12×6cm sized mass at the pancreatic body and tail but also a 1cm sized mass in left lobe of the liver. The patient underwent a near-total pancreatectomy and tumorectomy of the liver. A solid pseudopapillary tumor with liver metastasis was confirmed by pathology. She has undergone 13 courses of chemotherapy and has been well for 13 months without any sign of recurrence.

Choledochal cysts have been associated with complications such as cholangitis, pancreatitis, and malignancy of the biliary tract. Recently, the incidence of choledochal cyst in neonate and young infant is increasing due to advances in diagnostic imaging. The aim of this study is to investigate the rationale of excision of choledochal cyst during the neonatal period. The clinical outcome and correlation between age at surgery and the degree of liver fibrosis were reviewed retrospectively. A total of 198 patients with choledochal cyst who were managed surgically between January 1985 and December 2000 at the Department of Surgery, Seoul National University Children's Hospital were included in this study. The overall outcome and the outcome of patients who were managed surgically during the neonatal period were compared. Correlation between age and the degree of liver fibrosis was evaluated by chi-square test and Pearson exact test. The mean age of the patients was 2 years 7 months (ranged from 5 days to 15 years). Mean postoperative follow-up period was 7 years 1 month (7 months to 20 years). The results are as follows. Twelve patients (6%) had postoperative complications, cholangitis (7), bleeding (4) and ileus (1). Eleven patients operated during the neonatal period had no postoperative complications. The positive correlation between age group and degree of liver fibrosis was statistically significant (chi-square: p=0.0165, Pearson exact test: p=0.019). The results support the rationale that excision of choledochal cyst can be performed safely without increasing morbidity in neonates.

With the development of fetal ultrasonography, detection of fetal ovarian cysts has been increased. Although ovarian cyst formation during the perinatal period is a self limiting process, there is still considerable controversy regarding the best treatment of the fetal ovarian cyst. The purpose of this study is to evaluate the natural history of fetal ovarian cysts and to analyze the result of treatment. From 1995 to 2004, 31 consecutive fetuses with ovarian cysts were followed by ultrasonography during the perinatal period. The fetal ovarian cyst was diagnosed by prenatal ultrasonography between 25weeks and 38 weeks and the mean size of the cysts was 5cm (ranged from 2 to 8cm). At birth, 3 cysts disappeared. In 2 cases, the diagnoses were changed to multicystic kidney disease and intestinal duplication. During following up of 26 cysts, 15 cysts have resolved completely. Seven cysts required oophorectomy because of cyst torsion (n=3), differentiation of tumorous condition (n=2), increased size of cyst (n=1), and large size (8cm) of cyst at birth (n=1). Fetal ovarian cyst should primarily be observed, and only in the limited cases, surgical treatment would be required for the risk of complications such as torsion and differentiation from benign to malignant pathology.

The history of esophageal replacement in infants or children is the history of development of various kinds of alternative conduits such as stomach, colon, and small bowel. The gastric tube has been the most widely used conduit. From January 1988 to May 2003, 23 esophageal replacements with gastric tube were performed at the Department of Pediatric Surgery, Seoul National University Childrens Hospital. Statistical analysis was performed using Windows SPSS11.0 Pearson exact test. There were Gross type A(n=10), type B(n=1), type C(n=11), type D(n=1). Ten patients who had long gap esophageal atresia (type A-8, type B-1, type C-1) and 13 patients (type A 2, type C-10, type D-1) who had stenosis, leakage, recurred tracheoesophageal fistula, and esophagocutaneous fistula after previous corrective operations, had esophageal replacement with gastric tube. Mean follow-up periods were 4 year 2 months (7 months-15 year 1 month). There were postoperative complications including GERD in 16 (69.6 %), leakages in 7 (30.4 %), diverticulum at anastomosis in 2 (8.7 %), anastomosis site stenosis in 4 (17.3 %), and distal stenosis of the gastric tube in 1 (4.3 %). There was no statistical significance between operation types and postoperative leakage and gastroesophageal reflux. In conclusion, esophageal replacement with gastric tube may be a useful surgical option in esophageal atresia with long gap and esophageal atresia complicated by previous corrective operation.

As prenatal ultrasonography becomes popular, the number of prenatal diagnosis of congenital surgical diseases is also increasing. To evaluate the impact of antenatal ultrasonography on outcome the mortality rate in neonatal surgical emergencies was studied. The authors retrospectively reviewed 281 patients (congenital diaphragmatic hernia: 44, tracheoesophageal fistula: 78, intestinal atresia: 98, omphalocele: 28 and gastroschisis: 33 who had been managed at Seoul National University Childrens Hospital, from January 1991 to December 2000. The patients were divided into two groups; group A (1991 to 1995; 139 patients) and group B (1996 to 2000; 142 patients). These two groups were subdivided into prenatally diagnosed subgroup and postnatally diagnosed subgroup. We analyzed the changes of prenatal diagnosis rate, total mortality rate, and mortality rate of subgroups. Prenatal diagnosis rate was increased significantly in group B (Group A: 24.5 % and Group B: 45.1 %). Total mortality rate of group A was 21.6 %, and that of group B was 10.6 %, showing a significant decrease in group B. However, in both group A and B, when compared antenatally diagnosed subgroup with postnatally diagnosed subgroup, the mortality rate was lower in postnatally diagnosed subgroups but statistically not significant. The authors conclude that although prenatal diagnosis rate has been increased, prenatal diagnosis itself has not resulted in significant improvement in outcome.

A re-pull-through operation for Hirschsprung's disease is performed when the primary operation has failed because of the remnant or acquired aganglionosis, anastomotic stricture and/or fistula. The purpose of this study is to review our experience of the re-pull-through procedure for Hirschsprung's disease. From May 1978 to July 2003, 26 patients who underwent re-pull-through operations at the Department of Pediatric Surgery, Seoul National University Children's Hospital, were studied retrospectively by means of chart review as well as telephone interview. The mean age at primary operation and re-operation were 11months (2 months - 10 years) and 43 months (1 year - 23 years 3 months), respectively. Initial operation for Hirschsprung's disease was Duhamel's procedure in 17, Swenson's in 6 and Soave's 3. Causes of failure of primary operation were remains of secondary aganglionic segment (n=23), vascular arcade injury (n=1), rectoperineal fistula (n=2, due to mesenteric torsion and poor blood supply). Mean interval between the primary operation and the re-operation was 34 months (6 months-22 years). Reoperation methods were Soave's in 12, Duhamel's in 8, APSP (abdomino-posterosagittal pull-through procedure) in 5, and Swenson's in 1 case. In 2 cases of repeated rectoperineal fistula or rectourethral fistula, re-APSP were performed 3 times, respectively. A total of 29 re-pull-through operations were performed. Postoperative complications were wound infection (n=1), adhesive ileus (n=1), rectoperineal fistula (n=3), rectourethral fistula (n=2), and death due to pulmonary embolism (n=1). Mean follow up period of reoperation was 78 months (1 month-23years). In current state, 2 patients have an ileostomy because of repeated rectoperineal fistula and rectourethral fistula. Of the remaining 23 patients, 21(91 %) are totally continent with or without minimal soiling. Reoperation for Hirschsprung's disease was effective and safe for the patients complicated to the initial pull-through operation.

Continuous Ambulatory Peritoneal Dialysis (CAPD) has now become an established form of renal replacement therapy in children. Despite of technical improvements, there are various complications in CAPD. We reviewed medical records of children who received CAPD at Seoul National University Children's Hospital in the period between May 1991 and June 2002. Ninety-three procedures of CAPD catheter insertion in 70 patients were included in this study. Complication rate was 64.5%, and CAPD catheter related peritonitis was most common. In conclusion, CAPD catheter related peritonitis develops in considerable number of pediatric patients. Although the peritonitis could be treated with empirical antibiotics therapy, further investigation to prevent complication is required.

Inguinal hernia is a major surgical disease in pediatric surgery, occurring in 3.5% to 5% of all mature newborns and 9% to 11% of all premature babies. The
objective
of this study is to analyze the predisposing factors in association with recurrences of inguinal hernias in infants and children. In the period from January 1995 to September 2001, 1,575 infants and children who had primary inguinal hernias and recurrent inguinal hernias operated on at the Department of Pediatric Surgery at Seoul National University Hospital were evaluated retrospectively. We evaluated the data by medical records and by telephone interview. The sex, age, location of hernia, comorbidity, prematurity, incarceration, interval to operation after incarceration, postoperative complications were analyzed as predisposing factors in associated with hernia recurrence. Operative findings of recurrent inguinal hernia were reviewed. The data were statistically analyzed with Pearson Chi-Square test and Fisher-exact test. A total of eighteen (1.14%) out of 1,575 patients underwent an operation due to recurrent inguinal hernia. In 5 (27.8%) out of 18 recurred patients, institution of the primary herniorrhaphy was our hospital and in the other 13 (72.2%) was outside hospital. No impact on the development of recurrences was seen for sex, age, interval to operation after incarceration, and postoperative complications. The significant predisposing factors of recurrent inguinal hernias were left inguinal hernias (p=0.002), comorbidity (p=0.002), prematurity (p=0.006), incarceration (p=0.017) and technical error of first herniorrhaphy. We expect that knowledge for predisposing factors of recurrent inguinal hernias and experienced skill of pediatric surgeons will decrease recurrence rate in primary inguinal hernia.

The aim of this study was to evaluate the posterior sagittal anorectoplasty (PSARP) as a re-do operation in patients who failed initial repair of anorectal malformation. Nine patients (4 boys and 5 girls) who had previous failed surgery for anorectal malformation underwent secondary operations through posterior sagittal approach. The main reasons of surgery were constipation (n=3) and persistent anatomical derangement in spite of previous correction surgery (n=6). In addition to constipation, the former group (n=3) had various anatomical defects, and the latter group (n=6), of course, had constipation in some degrees. Patients ranged in age from 2 to 19 years (median 3 years) with only one over the age of 6 years. The primary procedures included PSARP (n=8) and anoplasty (n=1). The rectum was mobilized from surrounding structures through posterior sagittal approach and anatomical defects were corrected. The rectum underwent reconstruction, which involved relocation of the rectum and anus within the limits of the intact muscle complex. Patients underwent follow-up for periods ranging from 6 to 77 months (mean 37 months) after surgery. Anatomical corrections of all the defects were successfully fulfilled in 9 patients. All the patients were satisfied with the functional results after redo-PSARP compared with the preoperative defecatory function. This study suggests that (1) some of the patients with troublesome constipation may have anatomical defects, prominent or hidden, (2) surgeons should suspect the possibility of anatomical defect as the cause of incontinence and (3) preoperative thorough investigation to reveal the anatomical defects should be included in estimating patients with severe incontinence after previous surgery and planning the correction for failed previous surgery as well.

Mesenchymal hamartoma of the liver is a rare benign tumor, usually presenting in early childhood. Five children with mesenchymal hamartoma of the liver pathologically verified at Seoul National University Children's Hospital between 1978 and 2000 were analyzed retrospectively. There were two girls and three boys, and their mean age at the operation was 16.0months (range, 4 - 32 months). Three patients presented with abdominal distension. A patient was detected incidentally, and another was detected by prenatal ultrasongraphic examination. Tumor size ranged from 10x8.5cm to 34x29cm. Three tumors were located in the right lobe and two in the left lobe. Four cases underwent complete surgical resection, and the other one underwent incomplete surgical resection and marsupialization. Recurrence or malignant transformation was not noted. Five patients survived without evidence of disease for 35, 36, 38, 142 and 228 months. In conclusion, although mesenchymal hamartoma of the liver is benign lesion, it may be confused, and mixed with embryonal sarcoma. A recent report showed recurrence or malignant transformation after partial excision of the tumor. Therefore, complete excision of the tumor with surrounding normal liver tissue is recommended.

Ventriculoperitoneal shunt (VP shunt) for hydrocephalus is thought to inhibit the closure of processus vaginalis by increasing intraabdominal pressure, thus it promotes the inguinal hernia. We reviewed the incidence and characteristics of the inguinal hernia in VP shunted patients, and tried to estimate the patency rate of processus vaginalis in early childhood. A reprospective review of patients undergone insertion of VP shunt between January 1980 and May 1998 at Seoul National University Children Hospital was done. 262 patients were included in this study. Among them, 28 patients developed inguinal hernia (10.7%). Six patients developed inguinal hernia before the insertion of VP shunt. According to the age of VP shunt, the inguinal hernia developed in 16.2% (12/74) of patients who had undergone VP shunt before 6 months old, 12.4% (11/89) between 6 months and 2 years old and 5.1% (5/99) after 2 years old. Among 22 patients excluding 6 patients who developed hernia before VP shunt, the incidence of inguinal hernia after VP shunt was 8.6% (22/256) with male predominance (M:F=18:4). 8 patients developed inguinal hernia bilaterally (36.4%). It is suggested that at least 14% of processus vaginalis is patent until 2 years old.

Spontaneous rupture of the eventrated diaphragm is not common. The authors report a case of spontaneous rupture of the congenital diaphragmatic eventration. An 8 year-old girl with right congenital diaphragmatic eventration and nephrotic syndrome was seen in emergency room because of severe abdominal pain and vomiting. She had intermittent abdominal pain for 1 year. Plain chest X-ray and ultrasonography showed entrapped bowels in the right thoracic area. Exploratory laparotomy revealed a ruptured right eventration. THE displaced abdominal viscera were repositioned into the abdominal cavity and the ruptured diaphragm was trimmed and plicated. The postoperative course was uneventful. Only one case of spontaneous rupture of eventrated diaphragmatic has been reported in the English literature.

The WDHA syndrome characterized by watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome) is rare, and with neurogenic tumors. A 20-month-old girl presenting with symptoms of WDHA syndrome was transferred to our hospital. She had a ganglioneuroblastoma of the adrenal gland. The serum level of VIP was elevated. After complete excision of the tumor, all symptoms related to the WDHA syndrome were relieved and serum VIP level dropped to normal. The postoperative course was uneventful. The patient was treated with postoperative chemotherapy and radiation therapy. There was no evidence of disease 33 months after operation.

Fecal incontinence is not rare in post-op. children who had anorectal malformation, Hirschsprung's disease, and meningomyelocele. It can negatively impact the patient's emotional and social development. Among the options, antegrade continence enema (ACE) was introduced to overcome the demerits in 9 cases from January 1998 to June 1999. All patients have meningomyelocele. The cleanliness and post-operative complications were evaluated. The operative technique has three categories including reversed appendicocecostomy, in situ appendicostomy and neoappendicostomy with cecal flap. All of the patients achieved complete cleanliness. Four patients had post-operative complications. There were two patients with stoma leakage, one with abdominal pain on irrigation and one with stoma stenosis. The leakage was minimal and the pain on irrigation improved spontaneously. The stoma stenosis required revision operation on post-operative 6th month. The ACE has been shown to be safe and highly effective.

Neurocristopathy is characterized as having a common origin in aberrant neural crest development. Congenital central hypoventilation syndrome (Ondine's curse) is characterized by marked depression of respiratory drive during sleep and normal ventilation while awake because of no response to both hypercapnea and hypoxia. The girl was full-term, weighing 3020 grams. The girl had poor respiratory effort at birth, but improved with oxygen supply and stimulation. abdominal distention and calcification were noted. During laparotomy transitional zone was found at distal jejunum; a jejunostomy was constructed. Numerous attempts at extubation failed because of apnea. The results of an apnea work-up, including brain sonography, echocardiogram, were normal. The girl died of sepsis at 37 days of age. para-aortic ganglioneuroblastoma was found on autopsy. We experienced a newborn with congenital central hypoventilation syndrome, Hirschsprung's disease and congenital ganglioneuroblastoma representative of neurocristopathy.

Undifferentiated (embryonal) sarcoma is a rare malignancy of the liver in children and young adults. Seven cases of undifferentiated (embryonal) sarcoma of the liver pathologically verified at Seoul National University Children's Hospital between 1986 and 1999 were retrospectively analyzed. There were three girls and four boys, and their mean age at diagnosis was 12.1 years (range 7–13 years). Six patients presented with an abdominal mass or pain, and one with weight loss. Tumor size ranged from 8.0 × 8.0 cm to 15.0 × 15.0 cm. Four tumors were located in the right lobe, two in the left lobe and one in both. One patient died during chemotherapy. Initial complete resection was accomplished in three patients. Two patients underwent complete resection after chemotherapy. Five patients with complete resection survived without evidence of disease for 8, 11, 13, 28, and 84 months. A patient with partial resection and chemotherapy died of sepsis during chemotherapy 19 months after complete surgical resection. Adjuvant chemotherapy and radiotherapy were performed in all patients after complete surgical resection. In conclusion, though undifferntiated (embryonal) sarcoma of the liver is highly malignant, the combination therapy of surgery, chemotherapy and radiotherapy appears to result in a favorable prognosis

The association of congenital colonic atresia with imperforate anus is extremely rare. There are only three cases reported in the English literatures. The authors have recently managed one case in female infant.

Lymphangioma is a congenital malformation of the lymphatic system that expands into the surrounding tissues in a manner similar to a malignant tumor. Surgical excision has been the treatment of choice, but the results are often unsatisfactory due to incomplete resection, fluid collection and recurrences. Intralesional injection of OK-432(lyophilized incubation mixture of Group A Streptococcus pyogenes of human origin) was employed in treating 24 patients with lymphangiomas between 1992 and 1997. The method was used in 21 patients as a primary therapy, and in 3 patients for unresectable or recurrent cases as a secondary treatment. The results were excellent in 8 patients (33.3 %), good in 7 patients (21.8 %) and poor in 7 patients (21.8 %). The side effects were fever under 39℃ and local inflammation. However these symptoms subsided in a few days without serious sequelae. These results suggest that intralesional injection of OK -432 is safe and effective treatment for primary lymphangioma as well as a secondary therapy for unresectable or recurrent cases.

Six children with solid and papillary epithelial neoplasm of the pancreas were studied retrospectively. There were 2 boys and 4 girls. The mean age at operation was 11 years(range; 8-13years). Three patients had incidental abdominal mass, in two patients the mass was non-tender, in one patient the mass was tender. The minimum size of tumor was 6.5 × 6.0 cm and the maximum was 10.5 × 8.0 cm. Five tumors were located in the head of the pancreas, and the other one in the tail. Local invasion or metastasis was not noticed. Tumors were removed completely by performing the following operations: 3 pylorous preserving pancreaticoduodenectomy, 2 Whipple's operation and 1 distal pancreatectomy. There was no mortality. The histologic findings were characteristic. There were no recurrences during a follow-up of 0.5 to 12 years (mean; 5.0 years).

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.

Accidental ingestion of caustic substance is one of the common problems among children around the world. Acid intake accounts for a mere 5% of all reported cases of corrosive ingestion in the West. Because of the esophageal sparing effect of acid, clinically significant esophageal involvement after acid ingestion occurs in only 6 to 20 per cent of the instances. Despite effort of prevention, 7% to 15% of children sustaining caustic esophageal burns develop esophageal strictures. If balloon dilatation or bougie dilatation fails to resolve the esophageal strictures, successful outcome following replacement by colon or stomach has been reported in children. But the complications and morbidity following these operations are still relatively high. Seven patients with corrosive-acid induced esophageal strictures who were operated upon at the Department of Pediatric Surgery, Seoul National University Children's Hospital from 1991 to 1995 were reviewed. Primary resection and anastomosis was performed in all of 7 patients. The stricture involved short segments of the esophagus at the level of the lower cervical and the upper thoracic vertebra. The operations were approached through a left cervical incision or a left thoracotomy. In one patient, operative repair of anastomotic leakage was done, and three patients required re-resection of anastomotic strictures postoperatively, and one patient required a third operation(reversed gastric tube} due to an anastomotic stricture. The other anastomotic leaks, strictures or pulmonary complications were resolved with conservative treatment. In conclusion, primary resection and anastomosis of the esophagus was performed successfully on the 6 of 7 children with acid induced corrosive esophageal strictures. This approach is physiologic, especially in children who should have a long life expectancy, and recommended for the treatment of short-segment acid induced corrosive esophageal strictures.