Children who underwent reparative operations for esophageal atresia (EA) with or without tracheoesophageal fistula (TEF), are confronted with many gastrointestinal or respiratory problems, especially during the early years of life. We reviewed the medical records of 50 patients who underwent repairs of EA with or without TEF at the Division of Pediatric Surgery, Samsung Medical Center, from December 1994 to December 2005. Current status of children was accessed by telephone-interview, but only 27 of them were accessible. Of 50 patients, 3 patients (6%) were type A, 45 patients (90%) were type C, and 2 patients (4%) were type E. The mean interval between primary operation and interview was 5.5 years. The incidences of growth retardation (<10 percentile of height/weight) were 39% and 21 % during the first 5 years after repairs, respectively. The incidences of dysphagia or gastroesophageal reflux and recurrent respiratory infections were 33% and 39 %, respectively. However, these problems were likely to improve as the children grew. The incidences of growth retardations (<10 percentile of height/weight) were 11% and 11% for the children more than five years postoperative. The incidences of dysphagia or gastroesophageal reflux and recurrent respiratory infections were 22% and 22%, respectively. Children with EA with or without TEF are faced with many obstacles. Close observation and adequate treatment for delayed postoperative complications are necessary to improve the quality of life for these children.

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

Infantile hepatic hemangioendothelioma (IHHE) is the most common benign vascular hepatic tumor in children. We analyzed the 17-year experience of IHHE. The medical records of 16 patients (M:F=8:8) treated at the Department of Pediatric Surgery and the Department of Pediatrics Seoul National University Children's Hospital between January 1991 and January 2008 were reviewed retrospectively. Mean age at presentation was 87 days (1 day-551 days). Seventy five percent of patients were diagnosed with imaging study and 25% with biopsy. Major symptoms were hepatomegaly (N=5), palpable abdominal mass (N=4) and congestive heart failure (N=3). Six patients had no symptoms. Kasabach-Merritt syndrome was combined in one patient. Nine patients (56.3%) underwent operation and 2 patients (12.5%) underwent only medical treatment. Clinical observation was tried on 5 patients (31.3%) without any treatment. Operation was performed on the patient with clinical symptoms or on patients where the differentiation between begin and malignant could not be determined. Patients who had clinical symptoms but tumor was unreresectabile were treated medically. Among the 5 patients who had been observed for their clinical course, 2 patients showed complete regression and the tumors of the remaining 3 patients were regressing. Clinical symptoms, the age at presentation, the size of tumor and α-FP, all had no significant statistical relationship with the time required for complete tumor regression. There was no relationship between the size change of the tumor and the change of α-FP level. Only the size of tumor was related with clinical symptoms. One patient died of post-operatvie bleeding. Treatment plan was determined by the extent of the tumor and the presence of clinical symptoms. Observation was enough for the patients without clinical symptoms and complete resection was curative for patients with clinical symptoms. Medical treatment is an alternative for the patient whose tumor is unresectable.

Necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency in the neonatal population. The aim of this study is to evaluate surgical indication and prognostic factors of NEC. Clinical data of seventy patients, diagnosed as NEC between January 2000 & January 2007, were reviewed retrospectively. Fifty-seven patients had medical treatment and 7 of them died. Thirteen patients who presented with pneumoperitoneum on plain abdominal film or were refractory to medical treatment received surgical treatment, and 5 of them died. All the expired 12 patients weighed less than 2500 g. Twenty out of seventy patients showed thrombocytopenia, and 11 patients of them died. The finding of pneumoperitoneum and thrombocytopenia could be the most important surgical indication. Prematurity, low birth weight and thrombocytopenia were related to a bad prognosis. NEC patients who presents with these findings must be considered for close observation and intensive care.

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 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.

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.

Forme fruste choledochal cyst (FFCC) is one of the diverse types of choledochal cyst with little or no dilatation of the extrahepatic bile duct (EHBD). It is considered that FFCC has to do with the pancreatobiliary malunion (PBM). In children, 3 to 6 millimeters of EHBD is assumed to be normal. Even though there is no clear-cut definition, FFCC is likely to be associated with bile duct dilatation less than 10 millimeters. Almost all cases have PBMs and symptoms of the pancreatitis or cholangitis. We experienced a case of FFCC in a 4-year-old boy. His EHBD measures 10mm diameter. He had symptoms of pancreatitis and elevated hepatic transaminases. The pancreatobiliary common channel was 28 millimeters. He underwent EHBD resection and Roux-en-Y hepaticojejunostomy and was discharged with no specific complications.

Wandering spleen is very rare condition in children characterized by migration of the spleen from its normal position due to laxity or absence of the supporting splenic ligaments. We experienced a case of splenic infarction due to torsion of a wandering spleen in a 6-year-old boy who presented with fever, vomiting, and abdominal pain of 2 day's duration. On physical examination, there was severe tenderness in the left upper quadrant of the abdomen. The plain abdominal radiograph showed marked colonic gaseous distension. Contrast-enhanced abdominal computed tomography scan showed decreased density of spleen in the normal position, consistent with infarction. At emergency laparotomy, a wandering spleen twisted 360° on its pedicle was found. Despite splenic detorsion, blood flow could not be restored. Splenectomy was therefore performed. The child was discharged 7 days after surgery without any complications.

Colorectal cancer is extremely rare in children. Unlike adult colorectal cancer, the overall prognosis of colorectal cancer in children is poor. Delayed diagnosis, advanced stages of the disease at presentation, and mucinous type of histology are the major determinants of poor outcome in childhood. A 13-year-old boy with abdominal pain visited our hospital. Physical examination andabdominal ultrasonography identified acute appendicitis with perforation. He underwent appendectomy and then the pathologic findings revealed mucinous adenocarcinoma. The cancer was located at the transverse colon and had metastases on peritoneal wall at 2^nd laparotomy. Extended right hemicolectomy was performed. He underwent palliative chemotherapy. After 4 months later, hepatic metastasis and aggravated peritoneal seedings developed. He died of renal failure and pneumonia 13 months after operation. We need to have a high index of suspicion for the possibility of a malignant colorectal tumor in any childhood case with nonspecific signs and symptoms.

Intraabdominal cystic lymphangioma is an uncommon lesion. It is usually found incidentally in patients presenting with an acute abdomen. Laparoscopic excision of intraabdominal cystic lymphangioma is an easy and safe procedure in children. We report one case of cystic lymphangioma in a 6-year-old female. The lesion was located on the left side of the transverse mesocolon. Laparocopic excision of the cyst was performed without complications.

The term “Littre's hernia” was originally defined by Reinke in 1841 as “the presence of a Meckel's diverticulum in any hernia sac.” Littre's hernia is a very rare disease, which accounts for less than 1% of all Meckel's diverticula. We report a case of Littre's hernia experienced in a 45 day-old infant.