Neuroblastoma treatment remains challenging, but has been advanced by the establishment of clinical and biological variables that determine prognostic risks. Initially, stage and age were the prime determinants of survival used in clinical practice. Risk-based therapy currently is the hallmark of neuroblastoma treatment. This study reviewed the results of neuroblastoma in one center. Sixty-three patients with neuroblastoma were treated primarily at our institution from 1989 to 2003. All patients were graded according to the International Neuroblastoma Staging System (INSS) at diagnosis. There were 37 boys and 26 girls. The median age was 2.14 years (range, 33 days-10.2years). The primary site was the adrenal gland in 47, dumbbell shape extended into spinal canal in 6, retroperitoneum in 5, mediastinum in 3, and other sites in 2. The probability of 5-year overall survival (OS) and event free survival (EFS) were 46.7% and 44.2% by Kaplan-Meier method. According to INSS, there were stage 1 in 2, stage 2 in 5, stage 3 in 12, stage 4 in 42, and stage 4s in 2. There were statistically significant differences in the survival rates between patients with stage1, 2 and stage 3, 4(p<0.05). For the stage 3 and 4, the extent of surgical resection determined from the operative records and pathologic reports assessed extent of surgical resection as complete resection, no visible tumor (CR, n=17); minimal residual, visible tumor less than 5% (MR, n=15); partial resection, more residual tumor than MR (PR, n=11). The 5-year OS rate was 57.8, 51.4, and 13.6% for CR, MR, and PR. There is a trend toward higher OS with achieving the completeness of resection (p<0.05). We conclude that age and stage at diagnosis are prognostic factors, and complete excision of the primary tumor can provide better prognosis for patients with stage 3 and 4 neuroblastoma.

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%.

Falls from a height are the leading cause of injury and death among urban children. In this study,
objective
was to describe the incidence, clinical characteristics, and treatment results of the children who fell from a certain height and admitted over abdominal injury. The medical records of patients under 15 years old admitted over abdominal injury by a fall from one meter or more height between January 1997 and December 2003 at Ewha Womans University Mokdong Hospital were analyzed retrospectively. Of 585 consecutive patients who admitted to our hospital after a fall from a height of 1 to 31.2 meters, 28 patients (4.8%) suffered from blunt abdominal trauma. The male to female ratio was 2.1: 1. The children between 12 months and 13 years old were the patients with 5.5 years old of the median age, and the median height fallen was 3 meters. Fifteen patients (53.6%) were during the summer and seventy-nine percent of the falls occurred between noon and 9 pm. Eighteen (64.3%) of falls occurred in residential place and 19 (67.8%) of patients arrived at the emergency department in 30 minutes after the accidents. Only 16 patients (57.1%) complained of abdominal pain. Liver injuries were found in 12 (42.9%), spleen injuries in 5 (17.9%), kidney injuries 3 (10.7%), pancreatic injuries in 1 (3.6%) and nonspecific abdominal injuries in 9 (32.1%) of cases. The increased SGOT and SGPT were found in each 23 (82.2%) and 18 (64.3%) of cases. Eleven patients (39.3%) had been associated with head injuries and limb injuries in each 17.9% and thoracic injuries in 7.1%. Twenty-five patients (89.3%) were recovered under the conservative treatments and the median length of hospital stay was 6 days (range, 2 -20 days). Despite the absence of abdominal symptoms or shock, falls from a height in children may carry a significant intra-abdominal organ injuries. Moreover the height of free fall cannot reliably predict the degree of the abdominal injury. Accordingly after a fall from height, the abdomen should be evaluated by CT scan for potential injury.

Gastric perforation of newborn is a rare, serious, and life threatening problem. The pathogenesis of gastric perforation is less well understood, and ranges widely. That ischemia is responsible for intestinal perforation enhances the likelihood that a similar mechanism exists for gastric perforation. Twelve patients with neonatal gastric perforation who were treated upon at the Department of Pediatric Surgery, Hanyang University Hospital from 1987 to 2002 were reviewed. Eight patients were male and four female. The age of perforation was 1 day to 8 days of life. Ten patients undertook operation and 2 patients were treated conservatively. The perforation site was located at the anterior wall along the greater curvature of the stomach in 8 patients and along the lessor curvature of the stomach in 2. The precipitating factors were gastroschisis, premature baby on ventilator and mechanical intestinal obstruction each 2 cases, and cyanotic heart disease and indomethacine medication each one case. In 5 cases the cause of perforation was not identified. The mortality rate was 25%(3 of 12). Earlier recognition and treatment were throught to be crucial prognostic factors.

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.

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.

Failure of the urachus to regress completely results in anomalies that may be classified as patent urachus, urachal sinus, urachal cyst and bladder diverticula. The presenting symptoms of children with urachal anomalies are variable and uniform guidelines for diagnosis and treatment are lacking. The purpose of this study was to elucidate our conclusions regarding the presentation, diagnosis and treatment of urachal anomalies by reviewing our experiences. We retrospectively analyzed the records of 32 patients who were admitted for urachal anomalies from March 1995 to February 2005. The age distribution of these patients at presentation ranged from 1 day to 14 years old (median age 1 month) and they included 20 boys and 12 girls. The 32 cases comprised 13 cases of urachal sinus (40.6%), 10 urachal cyst (31.3%), and 9 patent urchus (28.1%). The presenting symptoms were umbilical discharge (14 patients), umbilical granuloma (8), abdominal pain and fever (3), fever (3), abdominal pain (2), and a low abdominal mass (2). In 30 patients ultrasonography was used for diagnosis and 2 patients with patent urachus were explored without using a diagnostic method. Twenty-three patients were confirmed by ultrasonography alone and 7 patients were examined using additional modalities, namely, computed tomography for 2 patients with an urachal cyst, magnetic resonance imaging for 1 patient with an urachal cyst, and fistulography for 3 patients with an urachal sinus. Excision was performed in 29 patients, and 3 patients were conservatively managed. Urachal anomalies in children most frequently presented in neonates, and the most common complaint was umbilical discharge with infection. Urachal anomalies can be diagnosed by a good physical examination and an appropriate radiographic test, and ultrasound was found the most useful diagnostic method. Complete surgical excision of an urachal anomaly is recommended to avoid recurrence, and even, though rare, carcinoma development.

Esophageal atresia without tracheoesophageal fistula accounts for 7-11% of all types of esophageal atresia and is very difficult to treat. In our hospital from 1990 to 2005, we operated upon 40 patients with esophageal atresia, and 6 had pure atresia. The preoperative characteristics, operative findings and post operative course of the six patients with pure atresia were analysed. Immediate gastrostomy was performed in all 6 patients. One patient had simultaneous cervical esophagostomy. Esophageal reconstruction procedures were transhiatal gastric pull up in 3 patients, esophagocologastrostomy utilizing left colon in 1, and transthoracic esophagoseophagostomy with esophageal bougination in 2. Postoperative complications were pneumonia, anastomosis leakage, and gastroesophageal reflux symptom. Conservative management was effective in all patients. A larger series of cases would be required to demonstrate the most effective treatment for this particular anomalous condition.

The repair of esophageal atresia with a long gap still continues to pose difficulties for the surgeon. There is general agreement that the child's own esophagus is best, but it is also believed that a primary repair is not always possible. Foker JE et al. (1997) developed a technique of esophageal lengthening using external traction sutures. We experienced one case of esophageal atresia with a 4.5cm gap (4 vertebral spaces) which was repaired using the external traction suture technique.

Chronic duodenal obstruction related to a congenital web is a rare anomaly that is sometimes difficult to diagnose preoperatively. A case of partial duodenal obstruction along with a foreign body by a congenital duodenal web in a 10-year-old girl is presented. She has had one year history of intermittent epigastric discomfort without nausea, vomiting and growth retardation. The studies including UGI series and gastroduodenoscopy disclosed a perforated web in the 2nd portion of the duodenum and a dark go stone just proximal to the web. Via a longitudinal duodenotomy across the web, the web was partly excised with preservation of ampulla of Vater and the duodenum was closed in transverse fashion. The aperture of ampulla of Vater was located at 7 o'clock direction of posterior surface of the duodenal web. This particular case reminds clinicians to consider a duodenal web in a youngster presenting with a longstanding foreign body in the duodenum as one of the underlying pathologies.

An 11-year-old girl with history of two previous attacks of acute pancreatitis was admitted to another hospital. On physical examination, she had epigastric tenderness. Laboratory studies included amylase of 657IU/L and lipase of 3131IU/L. Abdominal computed tomography (CT) scan suggested necrosis in 30% of pancreas. To establish the cause of recurrent pancreatitis, endoscopic retrograde cholangiopancreatography (ERCP) was performed after acute pancreatitis subsided. Duodenoscopic view revealed a blind sac covered by normal duodenal mucosa at the second portion of the duodenum. Barium upper gastrointestinal series (UGI) showed a large sac separated from adjacent duodenal lumen by a radiolucent band. Diagnosis of intraluminal duodenal diverticulum (IDD) was made and endoscopic excision was considered. The apex of the diverticulum was incised endoscopically using a needle knife papillotome. At a follow-up endoscopy one day after procedure, bleeding from the incised edge of diverticulum was noted. Despite hemoclipping and injection of hypertonic saline-epinephrine solution by under the endoscopy, hemostasis was unsuccessful. She was transferred to the Kyungpook National University Hospital after resuscitation. Open duodenotomy and excision of the diverticulum were performed. She has recovered well from surgery and remains asymptomatic.

Superior mesenteric artery (SMA) syndrome is a rare disorder caused by extrinsic compression of the third portion of the duodenum by the SMA. The operative treatment of choice is bypassing the obstructed duodenal segment by duodenojejunostomy. We report one case of SMA syndrome treated by laparoscopic duodenojejunostomy and followed up by 3D-reconstructive CT scan. A fifteen-year-old boy with intermittent vomiting and weight loss was admitted. Ultrasonography showed narrowing of the distance between the SMA and aorta. Hypotonic duodenography showed dilatation of duodenal third portion and barium stasis. On 3D-reconstructive CT scan, the angle between SMA and aorta was 37 °. The postoperative course was uneventful. Three months later, he had gained 3 kg of weight and the angle between SMA and aorta increased to 38-39 ° on 3D reconstructive CT scan. Laparoscopic duodenojejunostomy for bypassing the obstructive duodenum in SMA syndrome is a feasible and safe method.

A 10-year-old-girl who underwent Roux-en-Y cystojejunostomy under the diagnosis of choledochal cyst at another hospital at the age of 3 months was referred to our hospital due to abdominal pain. Abdominal ultrasonography (USG) and computed tomography (CT) showed the type I choledochal cyst and multiple gall bladder stones. Severe inflammation and adhesion made difficulty of radical resection and only partial resection of choledochal cyst with Roux-en-Y hepaticojejunostomy could be performed. She complained of intermittent abdominal pain, fever, nausea and vomiting 2 1/2 years after the second operation. Follow-up abdominal CT scan showed the polypoid nodular lesion in the remnant choledochal cyst and suspicious metastatic lesion in the segment 7 of the liver. The duodenum was obstructed by the mass arising from the remnant choledochal cyst. The USG-guided liver biopsy revealed the moderately differentiated adenocarcinoma. A secondary palliative gastrojejunostomy was performed to relieve the obstruction of duodenum. She died of hepatic insufficiency 4 months later of third operation.