Congenital diaphragmatic hernia (CDH) in the past was considered a surgical emergency requiring immediate operation. Several groups now advocate preoperative stabilization and delayed surgery. The treatment strategy for CDH in this institution is delayed surgery after preoperative stabilization. The aim of this study was to evaluate the results of delayed surgery. A retrospective review of 16 neonates with CDH was performed. Surfactant, conventional mechanical ventilation, high frequency oscillation, and nitric oxide were utilized for preoperative stabilization as necessary. The difference in outcome between two groups differentiated by the duration of the preoperative stabilization periods with mechanical ventilation (≤ 8 hours and >8 hours) was determined. Chi-square test was used to analyze the data. There were 7 right-sided hernias and 9 left. The average duration of stabilization was 32.4 hours. Hepatic herniation through the defect was found in 6 cases and all died. The most common postoperative complication was pneumothorax. The mortality rate of the right side hernia was higher than the left (85.7% vs. 33.3%, p=0.036). Mortality rate of the group (N=8) whose preoperative stabilization period was 8 hours or less was better than that (N=6) whose preoperative stabilization period was more than 8 hours (25.0% vs. 83.3%, p=0.031). The overall mortality rate was 56.3%. The better prognosis was noticed in left side hernia, no liver herniation, or shorter preoperative stabilization period.

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.

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.

Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is characterized by a single aganglionic segment of the colon extending distally to the anal margin. In surgical treatment, the surgeon performs a frozen section biopsy to confirm whether there are ganglion cells. If there are intervening ganglionic sites in aganglionic bowel, there may be confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was performed on a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and required two more operations to find the true nature of the disease. There were aganglionic segments from the anal margin to the terminal ileum 3.7cm proximal to the ileocecal valve. The entire transverse colon and appendix were normally ganglionated.

Infantile hypertrophic pyloric stenosis(IHPS) is common in full-term babies, and relatively rare in prematures. The diagnosis of IHPS in premature infants may be obscured because of the lack of classic symptoms and signs and the absence of the standard criteria for ultrasonic diagnosis. The purpose of this study is to discover the clinical differences between premature and full-term infants with pyloric stenosis, and determine the appropriate diagnostic methods for early diagnosis in premature infants. The clinical records of 52 IHPS patients who had been operated upon from October, 1994 to April, 1997 were reviewed. The incidence of IHPS in premature infants was 25 %. The onset of symptom was 4.7 weeks of age in premature, and 2.9 weeks in full-term babies. Diagnosis was established by typical symptoms. signs. and diagnostic imaging studies. In two premature infants, diagnosis was confirmed by upper gastrointestinal(GI) series, because ultrasonography did not meet the diagnostic criteria. Two premature infants initially diagnosed as gastroesophageal reflux by esophagography. were found to have IHPS by upper GI series. For the diagnosis of IHPS, a new set of criteria for premature babies has to be developed.

The omphalomesenteric duct normally obliterates by the sixth week of intrauterine life. Incomplete obliteration results in various abnormalities which may be apparent in the newborn infant. These include fistula resembling ileum, a prolapsed loop of intestine through the umblicus and a fistula draining intstinal contents. The tract may contain ectopic tissue of stomach, colon or pancreas. Although this malformation should be recognizable at birth, 40 % of patients are not treated until after the first month of life. In the past 28 months since .the Samsung Medical Center opened its doors to public, the authors have treated 4 cases of patent omphalomesenteric duct remnant includng one case of T-shaped total prolapse of the duct and adjacent ileum. These cases will be discussed and the literature reviewed.

Pulmonary sequestration is a complex anomaly involving the pulmonary parenchymal tissue and its vasculature. It presents as a cystic mass of nonfunctional lung tissue without communication with the tracheobronchial system. Usually, it receives blood supply from anomalous systemic vessels. Therefore, preoperative diagnosis of the pulmonary sequestration is difficult, especially when it is located in the abdomen and combined with congenital cystic adenomatoid malformation (CCAM). We encountered such a mass(CCAM type 2) detected prenatally by ultrasonography. It was a kidney bean shaped, pinkish mass straddling the thorax and abdomen on the right side. Because of the sonographic appearance, neuroblastoma was diagnosed preoperatively. The mass was completely extirpated without difficulty.

This is a case report of a sliding hiatal hernia with severe gastroesophageal reflux (GER) after repair of congenital diaphragmatic hernia(CDH). It was not possible to determine whether the hiatal hernia is a de novo lesion which was missed at the original operation or a consequence of overzealous repair of the Bochdalek defect at the expense of weakening of the diaphragmatic crura. This case demonstrates that a sliding hiatal hernia can be a cause of severe gastroesophageal reflux that should be managed surgically.