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.

Thyroid carcinoma is relatively rare in children. Eight cases of thyroid carcinoma were among 18 patients operated upon for thyroid tumors at Pediatric Surgery, Asan Medical Center in 11 years' period between 1989 and 2000. Five patients were boys and three were girls. The age distribution ranged from 10 to 14 years with a median age of 11.6 years. We studied clinical presentations, diagnostic workup, pathology, treatment, and follow-up (recurrence, mortality, and survival). All patients presented with anterior neck mass but one with multiple cervical lymph nodes enlargement. Familial history of thyroid cancer was seen in one case. All patients had a cold nodule by I-131 thyroid scan. Fine needle aspiration (FNA) biopsy was performed in all patients. The right lobe was involved in four patients and the left lobe in two. Two had both lobes involvement. Pathologically, six cases were papillary carcinoma, one was follicular carcinoma, and one insular carcinoma. Four patients underwent unilateral lobectomy and isthmectomy. Total thyroidectomy was performed in three patients. The insular carcinoma case was preoperatively diagnosed as follicular neoplasm by FNA. After confirmation of the pathology by initial right thyroid lobectomy, total thyroidectomy was subsequently done. Cervical lymph node metastases were presented in three cases (37.5%), and lung metastasis in two cases (25%). Three patients received postoperative 1-131 ablation. After a median follow-up of 53 months, all patients were alive without evidence of recurrence. In conclusion, thyroid carcinoma in children is frequently associated with lymph nodes involvement and distant metastasis, however, the prognosis is relatively good.

Eleven children with gastroesophageal reflux disease underwent fundoplication. Eight had neurological impairment, two hiatal hernia and one had history of esophageal repair for esophageal atresia. The most common and significant symptom was vomiting (81.8%), followed by recurrent respiratory infections (72.7%) and failure to thrive (72.7%). The most common diagnostic tool was 24 hour esophageal pH study, which showed a pH less than 4 for more than 10% of the total recorded time in 6 among 9 patients. Nissen fundoplication was performed in 10 patients. Thal fundoplication was carried out in one patient with esophageal atresia. Stamm gastrostomy was added for nutritional and/or swallowing problem in all 8 patients with neurological impairment. The median follow up period was 13 months. There were one late death of unrelated cause and one recurrence. The quality of life after antireflux surgery was greatly improved. Antireflux surgery should be done if indicated, and a simultaneous gastrostomy be considered in a patient with neurological impairment.

The purpose of the survey was to clarifY the clinical features and management of the anorectal malformations (ARM) in Korea. Twenty-seven members of the Korean Association of Pediatric Surgeons were surveyed. The members completed a case registration form for each patient during the two years period of 1996-1997, and a questionnaire that contained their management preferences for primary and complicated patients. Twenty-four members of the 22 institutions registered 295 cases of the ARM, and 27 out of 34 members (77.1 %) responded to the questionnaire. The patients were classified by the Wingspread classification of ARM(1984). The case registration form was a modification of Wingspread workshop for the postoperative assessment and case registration. The male to female ratio was 1.7: 1. The average number of patients per surgeon was 6.1 cases per year. Prematurity(> 36 weeks) was present in 1.9 % of cases and low birth weight (< 2.6 Kg) in 12.1 %. Among 187 male patients, 62 were higheR) type, 29 intermediate(l) type, 88 low(L), and 8 unclassified. In female, there were 2 cases of cloacal anomalies(C), 4 H type, 30 I type, 66 L type and 6 unclass ified. For male, 87.9 % of H and I type were operated by the Pena procedure, but only 7.9 % in L types. In female, all of C, H and I types, and 40.9 % of L type were done by the Pena procedure. One case expired (MR; 0.003%) as a result of surgical complication. Over-all complication rate was 12.5 %. Among 6 cases of reoperation, 4 were failed Pena procedures. Among 140 colostomies sigmoid colon was utilized in 75.7%, and loop colostomy was commonly used. Ten surgeons prefered primary maturation of the stoma. In conclusion, posterior sagittal anorectoplasty is popular for high types of ARM in this country, but considerable number of patients are still suffering from failed operations. For better understanding and analysis, necessity of prospective study by new classification was discussed

Femoral hernias are very uncommon in children and very easily misdiagnosed. During a period of three years, three children of femoral hernia were treated by one pediatric surgeon at Severance Hospital. Only one case was diagnosed correctly before surgery; the other two were thought to be either an indirect inguinal hernia or groin mass. Only one patient had curative hernioplasty (McVay hernioplasty) at the first operation; the other two did not have curative hernioplasty at the first operation. Femoral hernia in childhood is a challenging clinical problem because of its rarity and similar clinical presentation as indirect inguinal hernia. The frequency with which an incidental indirect inguinal hernia sac or patent processus vaginalis can be found at surgery can perpetuate a misdiagnosis. The absence of an expected indirect inguinal hernia sac or an apparent recurrence of an indirect inguinal hernia should lead to consideration of possible femoral hernia.

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

Solid and papillary cystic neoplasm of pancreas is an uncommon low grade malignant tumor found predominantly in young female in their second or third decade of life, and amenable cure by surgical treatment. The authors report two cases of solid and papillary neoplasm of pancreas pathologically verified at Kyung Hee university hospital. The first case was 11-years old male patient and the other case was 12-years old male patient. Symptoms of two patients were abdominal discomfort, nausea and vomiting and abdominal pain in the female patient. CT finding of solid and papillary neoplasm of pancreas depict a well-demarcated mass with solid and cystic necrosis component. In female patient, large hematoma was shown. Gross findings of tumor revealed apparent encapsulation, cystic degeneration and hemorrhagic necrosis. Microscopically the tumors were characterized by distinctive solid and papillary patterns of cellular arrangement without local invasion. All patients were discharged and follow up without any problem.

Pancreatoblastoma is uncommon in children and is exceedingly rare in adults. Prognosis is known to be good if the tumor is discovered prior to metastasis. Complete tumor resection is the most important factor for long survival. We report two cases of pancreatoblastoma in a 4 year and 7 year-old females. Palpable abdominal mass was accidentally discovered by their parents. Abdominal CT scan showed huge retroperitoneal mass of unknown origin in one case and of the pancres in the other case. At laparotomy, well encapsulated tumor mass (10×10 cm in diameter) was noted in the body of pancreas in one case, and well encapsulated tumor mass (8×7 cm in diameter) in the tail of pancreas in the other case. We resected the tumor and saved spleen in both ases. Histologic examination revealed that the tumors were pancreatoblastoma. Patients received postoperative chemotherapy with 6 cycles of cisplatinum, adriamycin, ifosfamide and etoposide and are alive in good condition 19 months and 17 months after operation.

Pseudoaneurysm of splenic artery may arise from a vascular erosion by the inflammatory processes around the splenic artery, particularly in acute pancreatitis and chronic pancreatitis, which may cause rupture of pseudoaneurysm and life threatening hemorrhage. Collective experience with this massive hemorrhage is attended by a high mortality rate even with prompt therapy, and conservative management is associated with an almost 100 per cent of mortality rate. Identification of the bleeding site at laparotomy may be exceedingly difficult, which makes the preoperative detection of bleeding source desirable. Peripancreatic vascular lesions can be identified by angiography, and in selected cases the risk of urgent operation to control massive hemorrhage may be obviated by embolization. The authors have recently experienced a case of ruptured splenic artery pseudoaneurysm combined with a pancreatic pseudocyst in a 6 years old boy. A bolus enhanced CT scan and angiography were essential to confirm these complications of pancreatic pseudocyst. We managed this child successfully with an urgent procedure of transcatheter arterial embolization and another elective surgery of pancreatic pseudocyst.

Gastroduodenal intussusception, an invagination of a part of the gastric wall through the pyloric canal into the duodenum is a rare condition. Gastroduodenal intussusception is caused by a mobile usually benign gastric tumor. However, gastroduodenal intussusception by gastric submucosal hemangiomatosis is not documented. We have managed a case of gastric submucosal tumor leading to gastroduodenal intussusception in 2 years and 10 months old boy. The tumor was 10 × 5 × 3 cm in size in posterior wall of gastric antrum. Laparotomy, manual reduction of the intussusception, and wedge resection of posterior gastric wall including the tumor were performed. Pathologic diagnosis was a submucosal hemangiomatosis.

Gastric volvulus is a rare surgical disorder in the pediatric population. We experienced a case of gastric volvulus. A 2-year-old boy was admitted to hospital with abdominal distension. An upper gastrointestinal series showed reversal of the greater and lesser curvatures. Surgical exploration revealed an organoaxial volvulus of the stomach, and anterior gastropexy was performed.

Neuroblastoma arises from the embryonic tissue of the adrenergic rest. It is commonly found in children and mostly in nonrenal tissue. We present a case of intrarenal neuroblastoma which was initially thought to be a Wilms' tumor. The patient was a 18 months-old girl treated with radical nephrectomy and adjuvant chemotherapy after operation. The neoplasm within the kidney in children cannot always indicate Wilms' tumor. Neuroblastoma of the adrenal gland or retroperitoneal tissue may often compress or invade the kidney directly or arise from the kidney. Clinical aspects that differentiate between neuroblastoma and Wilms' tumor are discussed with a review of the literatures.

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