To estimate the normal anal canal pressure in neonates, anal manometry was performed in 46 normal babies less than 6 days of age. Twenty-eight of the subjects were boys and 18 girls. All the subjects passed meconium within 24 hours after birth. Birth weights were above 2.4 kg. There were no sexual differences in birth weight, birth height, gestational age, postnatal age, or Apgar score (p<0.05). The mean manometry values were; anal sphincter length 18.6± 3.9 mm, high pressure zone (HPZ) 9.2 ± 3.6 mm, vector volume 2027.2 ± 2440.7 mmHg2cm, maximum pressure 42.3 ± 17.4 mmHg, and position of the maximum pressure 6.0 ± 22.4 mm. Only the HPZ of boys was longer than those of girls (p=0.005). In squeezing state, HPZ and the position of maximun pressure were not changed from resting state. HPZ, vector volume, and maximum pressure in boys were higher than those in girls. As the birth weight increased, the anal sphincter length (p=0.001) and the HPZ increased (p=0.047). The resting pressures of the anal canal were evaluated in three portions; /23 upper portion, 12.8± 8.6mmHg, middle portion, 20.3 ± 10.8mmHg, and lower portion, 26.1 ± 12.9 mmHg. These normal values may serve as guidelines for the evaluation, diagnosis and treatment of neonatal anal diseases.

The results of hepatic portojejunostomy in 34 patients with biliary atresia operated upon by one surgeon between May 1989 and December 1997 were analyzed. Eleven (32.3 %) patients were 60 days or younger, 14 patient (41.2 %) were between 60 and 90 days, and 9 (26.5 %) were over 90 days of age. Jaundice cleared in 20 cases (58.8 %). Three patients died of liver insufficiency, 2 were anicteric but died from esophageal variceal bleeding. Three patients died as a result of sepsis, heart failure and left kidney agenesis. Five patients were lost to follow-up. The five-year survival rate was 73.8 %. Two patients over 90 days of age, survived more than 5 years. Survival rates were not significantly related to the age at operation. We conclude that hepatic portojejunostomy should be considered as a primary surgical modality for biliary atresia, even at age 90 days or more. Early detection of esophageal varices and sclerotherapy may be necessary. Liver transplantation is necessary if hepatic failure develops.

The surgical treatment of intussusception during two periods, 1975 - 1978 and 1995 -1998 (Group B) were compared. There were 48 patients in Group A and 75 cases in Group B. Male were predominant in both group (2.7:1 vs 1.6:1). The mean age at operation was 6.7 ± 5.0 months (Group A) and 8.1 ± 7.0 months (Group B). The major signs and symptoms in both Groups included vomiting, hematochezia and irritability. There was a significantly higher bowel resection rate for group B (31.3 % vs 14.7 %, p=0.041 ). There were two operative deaths in group B but no deaths in group A. Hospitalization was significantly shorter in group B(7.5 ± 2.7 days vs 5.4 ± 2.1 days, p<0.001). We conclude that there were no differences in patient characteristics but surgical treatment in the 1990s results in more rapid recovery and reduced hospital stay.

To study the role of anomalous pancreatico-biliary ductal union (APBDU) in the development of choledochal cyst, we reviewed 23 cases. APBDU is defined as a long common channel(>0.4 mm). The patients ages ranged from 1 week to 112 months and the mean age was 22.5 months. Right upper quadrant pain was the most prevalent symptom. The diagnosis was made by ultrasonography and operative cholangiography in most patient. The preoperative diagnosis was made in 100 % of the cases. Gallstones were found in 5 cases. Todani type I and type IV were prevalent. A long common channel was found in all cases. The operative treatment consisted of cyst excision and Reux-en-Y hepaticojejunostomy or choledochojejunosotmy. One patient had postoperative pancreatitis. There was no mortality. We conclude that detection of choledochal cyst is occurring at a younger age and APBDU seems to play an important role in the pathogenesis of type I and IV cysts. Cyst excision is the treatment of choice to eliminate repeated cholangitis and malignant transformation.

A 6(1/2)-year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst (4.5 × 4.0 cm in size) in the left hepatic lobe and another tubular dilatation (2.0 × 0.8 cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangio-drainage (PTCD) with cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring 6.6 × 5.0 cmin size. She became afebrile and anicteric with aid of PTCD and parenteral antibiotics. However she continued to drain 45-150 cc of bile per day via the tube for over 2 weeks. Then she successfully underwent intrahepatic cystojejunostomy with guidance of intraoperative ultrasonography. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, which was successfully managed with PTCD followed by internal drainage procedure.

Splenic cysts are uncommon and classified as either primary(true) or secondary(pseudo-) depending on the presence or absence of a true epithelial lining. True cysts (epidermoid cyst) of the spleen are very rare. Three cases of splenic cysts in childhood were treated at the Yeungnam University Hospital in the last eleven years(1989 - 1999). Two of patients were girls. The ages at diagnosis were 7, 12 and 15 years. Abdominal ultrasonography and computerized tomography were utilized for the diagnosis. Radionuclide scanning was performed in one patient. Surgical resection(one partial splenectomy and two total splenectomies) was performed. The sizes of cysts were 4, 6.5 and gem in maximum demension.

Congenital afibrinogenemia is a rare disorder that refers to a congenital lack of production of fibrinogen, a key component of the hemostatic system. Bleeding manifestations of congenital afibrinogenemia vary in severity from mild to catastrophic. This is a case report of splenic rupture occurred in an eight-year-old boy with congenital afibrinogenemia. Nonoperative treatment with cryoprecipitate and virally inactivated, purified fibrinogen concentrates successfully avoided splenectomy.

Idiopathic small bowel ulceration distal to the duodenum is rare. Less than 5 % of the reported cases were in children. In the majority of the patients, a single ulcer of unknown cause is found in the jejunum or ileum. The diagnosis is difficult and usually made at the time of surgical exploration for complications, such as perforation, hemorrhage or obstruction. We treated a pediatric patient with perforation of an idiopathic ileal ulceration. The child was an ll-year-old boy who sustained blunt abdominal trauma. The involved ileal segment was resected. Pathologic findings were compatible with idiopathic small bowel ulceration. The clinical and pathological aspects of idiopathic ulcerations are discussed, and the literature reviewed.

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.

Adrenal cortical tumors are rare in adults and children. Most are malignant and functional. The principal clinical features are virilization, Cushing's syndrome, hyperaldosteronism and feminization. Recently, we treated a case of virilizing adrenal cortical tumor in a 26 month-old boy. The diagnosis was made by hormone assay, abdominal CT and tissue pathology. Right adrenalectomy was successful performed. Pathologic examination revealed an adrenal cortical adenoma with vascular invasion.