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.

Thyroid nodules are less common in children than in adults and their management is still controversial. The clinical presentations, operations, complications, histopathologic findings, and postoperative progressions of 34 pediatric patients that underwent thyroidectomy for palpable thyroid nodule at the Department of Surgery, Seoul National University Children Hospital between 1986 and 2003 were studied retrospectively by reviewing medical records and telephone interviews. The mean age of the patients was 11.8 years old. There were 23 females (67.6 %) and 11 males (32.4 %). Surgical indications were clinical need of histological confirmation (n=15), unresponsiveness to thyroxin replacement therapy (n=10), suggestion of the carcinoma on fine needle aspiration cytology (n=5), cosmetic purpose-a huge benign nodule (n=2), completion thyroidectomy for medullary thyroid carcinoma (n=1), and prophylactic thyroidectomy in a MENIIpatient (n=1). Unilateral Lobectomy was performed in 20 patients (57.1 %), subtotal thyroidectomy in 8 (22.9 %), total thyroidectomy in 5 (14.7 %), and completion thyroidectomy in 1 (2.9 %). Lymph node dissection was performed in 9 cases. Benign tumor was found in 23 patients (67.6 %), adenomatous goiter (n=18) and follicular adenoma (n=5). Malignant tumor was found in 11 children (32.4 %), 9 papillary carcinomas (26.5 %), and 2 medullary carcinomas (5.9 %). Of the 9 papillary carcinomas, 7 cases (77.8 %) had lymph node metastasis. No lymph node metastasis was found in 2 medullary carcinomas. Complications developed in 5 cases - transient hypocalcaemia (n=2), and temporary hoarseness (n=3). There was no mortality. Median follow-up period was 7.4 years (0.5-18 years). One patient showed recurrence in cervical lymph nodes 10 years after surgery and modified radical neck dissection was performed. Because of the high incidence of malignancy and advanced stage at initial presentation, more meticulous diagnostic work up is necessary for children with thyroid nodule, and more radical surgical treatment should be performed when malignant nodule is suggested.

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.

A 3-year-old girl with a primitive neuroectodermal tumor (PNET) and a 6-year-old girl with acute lymphoid leukemia were referred to us because of problems with their implantable central venous ports (Port-A-Cath®). On physical examination, the ports were upside-down, so a needle could notbe inserted through the membrane of the port. Right lateral side view of the chest radiogram confirmed port inversion in both cases. At operation, the ports were inverted and the transfixing sutures were totally absorbed. The ports were rotated 180 ° and anchoring sutures placed.

Ulcerative colitis, one of the inflammatory bowel disease, is primarily managed medically with combinations of 5-ASA and steroids. However, this chronic disease requires surgical management if symptoms persist or complications develop despite medical management. The clinical course, the indications and outcome for surgical management of pediatric ulcerative colitis patients were studied from medical records retrospectively. Twenty-one patients under the age of 15 who were endoscopically diagnosed with ulcerative colitis at the Seoul National University Children's Hospital between January, 1988 and January, 2003 were subjected to the study. Mean follow up period was 3 years and 10 months. The mean age was 10.3 years old. All patients received medical management primarily after diagnosis and 8 patients (38%) eventually required surgical management. Of 13 patients who received medical management only, 7 patients (53%) showed remission, 4 patients are still on medical management, and 2 patients expired due to congenital immune deficiency and hepatic failure owing to sclerosing cholangitis respectively. In 8 patients who received surgical management, 1 patient underwent surgery due to sigmoid colon perforation and 7 patients due to intractability of medical management. The perforated case received colon segmental resection and the other 7 patients recieved total protocolectomy with ileal pouch-anal anastomosis. One patient expired postoperatively due to pneumonia and sepsis. One patient is still on medical management because of mild persistent hematochezia after surgery. The other operated patients are showing good prognosis without any management. Pediatric ulcerative colitis patients can be surgically managed if the patient is intractable to medical management or if complications such as perforation is present. Total protocolectomy & ileal pouch-anal anastomosis is thought to be the adequate surgical method.

The purpose of this study is to review the operative management and outcome of operation for Crohn's disease. The medical records of 17 patients who underwent operations due to Crohn's disease at Seoul National University Children's Hospital from January of 1988 to June of 2005 were reviewed and retrospective study was carried out. The male-to-female ratio was 1.8: 1. The median age at the onset of symptom and the time of diagnosis was 9 years 6 months and 11 years 6 months respectively. The median interval time from diagnosis to operation was 2 years and 1 month (0 month~8 years). Ileocolic or ileocecal region was the most common site of involvement. The indications of operation were intractable symptoms (8 cases) and obstruction or stricture (7 cases). The median postoperative hospitalized days were 14.4 days (8~35 days). Five patients (29%) experienced postoperative complications. Symptom free state or symptom relief was observed in 11 cases after surgery and 6 cases had wax and wane course of aggravation and improvement. In pediatric Crohn's disease patients who present with intractable symptoms despite medical treatment or develop surgical complications, symptom free state or symptom relief can be achieved by minimal resection of involved lesion.

Primary liver tumors are uncommon in childhood, with a relative frequency of 3% of childhood tumors. Seventy three cases of pediatric primary liver tumors which were operated on at single institution between 1986 and 2002 were reviewed. There were 37 cases of hepatoblastoma, 11 hepatocellular carcinomas, 6 undifferentiated (embryonal) sarcomas, and 1 mixed germ-cell tumor in malignancies. Benign tumors constitute only 24.6% of liver tumors, including 7 hemangioendotheliomas, 5 mesenchymal hamartomas, 3 congenital cysts, and one each with focal nodular hyperplasia and hemangioma. The common presenting clinical features were abdominal mass or abdominal distension. Anatomical hepatic resections were carried out in 38 cases, and non anatomical resections in 34 cases. One patient died of a direct result of hepatic resection(1.4%), and complication rate was 16.4%.

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.

Visceral leishmaniasis is a life-threatening systemic infection caused by a protozoa of the genus leishmania and transmitted by sandfly. We report the first case of visceral leishmanaisis in Korea. The patient was a one-year-old girl admitted with the symptoms of fever, abdominal distension, pancytopenia, and purpura. She traveled to Argentina 5 months ago, and has had symptoms such as fever and pale appearance. Laboratory findings were: WBC, 12,680/mm³; Hg, 3.7g/dL; platelet, 100K; total bilirubin, 1.2mg/dL; AST/ALT, 48/10 U/L. CT scan and MRI showed hepato-splenomegaly. On laparotomy, excision of an accessory spleen and splenic hilar lymph nodes were performed. Many amastigotes were microscopically identified in histiocytes from the biopsy tissues. Sodium stibogluconate was administrated for 2 weeks, which did not relieve the symptoms. After administration of the additional Amphotericin B for 3 weeks, symptoms were improved.

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.

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.

Despite of advances in perinatal management and treatment modalities congenital diaphragmatic hernia(CDH) remains a frustrating problem. Although the sheep has proven to be a reliable experimental model for the production of intrauterine CDH, the rabbit may have some advantages. These include lower cost, smaller body size, year-round availability, high number of fetuses per pregnancy, and short gestational period. To evaluate the feasibility of the rabbit model of CDH, twenty-seven pregnant New Zealand rabbits were utilized. Hysterotomy and an operative procedure for creating a diaphragmatic defect on gestational day 24 or 25, in two fetuses of each pregnant rabbit were performed. In one fetus of one cornu of the uterus, the left fetal diaphragm was excised through an open thoracotomy(DH group). In another fetus in the other cornu, CDH was created and the trachea clipped(Surgiclip, USSC, Norwalk, Conn., USA) (TL group). Delivery was by Cesarean section on 30 days of gestation. Among twenty- seven pregnant rabbits, 12 in the DH group and eight in the TL group were born alive. The most common herniated organ was the left lobe of the liver. In thee DH group, the lungs were hypoplastic with decreased lung weightlbody weight ratio, reduced numbers of alveoli, thicker media of the pulmonary arteries, and immature alveoli. In TL group, the alveoli were more mature and did not differ from the control animals. In conclusion, (1) pulmonary hypoplasia develops in the fetal rabbit diaphragmatic hernia model and (2) simultaneous tracheal ligation prevents pulmonary hypoplasia.

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.

To assess the clinical and nutritional status of long-term survivors of biliary atresia, history taking, medical record review, physical examination (height, weight, MAC, TSF), blood tests (LFT, prothrombin time, platelet count, prealbumin, calcium) and liver needle biopsy were performed in 12 patients in whom Kasai procedure were performed more than 10 years ago at Department of Pediatric Surgery in Seoul National University Hospital. None were below the 5th percentile in height and weight. TSF was above the 75th percentile in all patients and showed good subcutaneous fat deposition. MAC was above the 5th percentile in all patients. Serum prealbumin level was abnormal in 2 patients with abnormal liver function and revealed visceral protein malnutrition. Serum calcium level was decreased below normal range in 4 patients with abnormal liver function. One patient had mild ascites. Five patients had abnormal liver function and 7 patients showed clinical manifestation of portal hypertension. Liver needle biopsy was performed in 5 patients and no cirrhotic change was observed. Although some patients who have survived for more than 10 years after Kasai procedure developed protein malnutrition and vitamin deficiencies, growth and development and nutritional status were generally satisfactory. Five patients(42%) showed normal liver function and no portal hypertension. In conclusion, Kasai procedure is satisfactory as a primary treatment in biliary atresia but significant portion of long-term survivors had abnormal liver function and portal hypertension. Continuous and careful follow-up is necessary to determine when liver transplantation may be indicated.

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.