Despite of advances in perinatal management and treatment modalities congenital diaphragmatic hernia(CDH) remains a frustrating problem. Although the sheep has proven to be a reliable experimental model for the production of intrauterine CDH, the rabbit may have some advantages. These include lower cost, smaller body size, year-round availability, high number of fetuses per pregnancy, and short gestational period. To evaluate the feasibility of the rabbit model of CDH, twenty-seven pregnant New Zealand rabbits were utilized. Hysterotomy and an operative procedure for creating a diaphragmatic defect on gestational day 24 or 25, in two fetuses of each pregnant rabbit were performed. In one fetus of one cornu of the uterus, the left fetal diaphragm was excised through an open thoracotomy(DH group). In another fetus in the other cornu, CDH was created and the trachea clipped(Surgiclip, USSC, Norwalk, Conn., USA) (TL group). Delivery was by Cesarean section on 30 days of gestation. Among twenty- seven pregnant rabbits, 12 in the DH group and eight in the TL group were born alive. The most common herniated organ was the left lobe of the liver. In thee DH group, the lungs were hypoplastic with decreased lung weightlbody weight ratio, reduced numbers of alveoli, thicker media of the pulmonary arteries, and immature alveoli. In TL group, the alveoli were more mature and did not differ from the control animals. In conclusion, (1) pulmonary hypoplasia develops in the fetal rabbit diaphragmatic hernia model and (2) simultaneous tracheal ligation prevents pulmonary hypoplasia.

Multisystem organ failure resulting from gram negative bacterial sepsis is associated with high morbidity and mortality in surgical neonates. There are differences in the clinical characteristics of organ failure in neonates and adults. The purpose of this study is to identify the differences and determine the order of organ failure between baby rats and adult rats after induction of gram negative sepsis. Fifty baby rats less than 30-day-old and another 50 adult rats more than 2-month-old were divided into control group (G1) and experimental group (G2). The G1 consisted of 10 baby- and 10 adult-rats, and the G2 consisted of 40 babies and 40 adults. E. coli (10⁸/mL per 100g of body weight) were injected into the peritoneal cavity in G2 and same amount of saline was injected in G1. Blood samples were obtained before injection, 24 hour, 48 hour, 72 hour and after death. WEC, platelet, PaO₂, PaCO₂, total bilirubin, BUN, creatinine, albumin and abdominal wall thickness were measured to evaluate the sequence of organ failure. The mortality was 55.0 % in G2-babies and 32.5 % in G2-adults. In baby rats, microvascular, hematologic and renal failure appeared within 24 hours after injection and pulmonary failure followed. Pulmonary, renal and liver failure developed within 24-48 hours in adult rats; however, microvascular failure did not appear until they were moribund. Thrombocytopenia, hypoalbuminemia, increased BUN and generalized edema was the earlist sign of sepsis in baby rats.

This paper includes our 9-year experience of 34 infants with biliary atresia with introduction of a new non-invasive diagnostic method, that is, ultrasonographic "triangular cord" (TC) sign. TC sign was defined as visualization of a triangular or a band-like echogenicity just cranial to the portal vein. Ultrasonographic TC sign seemed to be a simple, non-invasive, time-saving and useful tool in the diagnosis of biliary atresia, representing 84% sensitivity. Active bile excretion was restored in 90% of the patients who were treated between 31-60days, 78% of those between 61-90 days, and 33% of those being 91days or older. The incidence of postoperative cholangitis was 36%, and construction of antireflux valve in the Roux-en -Y loop did not affect the incidence of postoperative cholangitis (P=0.18). As for the surgical outcome, of 34 infants with biliary atresia, 23 (68%) are alive for 2-102 months period, and 12 of them are alive for more than 5 years . Five-year estimate survival by Kaplan-Meier method was 66 %.

Fecal incontinence is not rare in post-op. children who had anorectal malformation, Hirschsprung's disease, and meningomyelocele. It can negatively impact the patient's emotional and social development. Among the options, antegrade continence enema (ACE) was introduced to overcome the demerits in 9 cases from January 1998 to June 1999. All patients have meningomyelocele. The cleanliness and post-operative complications were evaluated. The operative technique has three categories including reversed appendicocecostomy, in situ appendicostomy and neoappendicostomy with cecal flap. All of the patients achieved complete cleanliness. Four patients had post-operative complications. There were two patients with stoma leakage, one with abdominal pain on irrigation and one with stoma stenosis. The leakage was minimal and the pain on irrigation improved spontaneously. The stoma stenosis required revision operation on post-operative 6th month. The ACE has been shown to be safe and highly effective.

Evaluation of the sacrum in anorectal malformations (ARMs) is important because of the frequent association with ARMs and functional outcome after correction of ARMs. Sacral defects are not easily detected because of immaturity of sacrum in children and overlooking of pediatric surgeons. The authors utilized the sacral ratio in normal children and patients with ARMs. In normal children, the mean true sacral ratio and mean sacrococcygeal ratio were 0.60±0.08 and 0.72±0.13 respectively. Sacral ratio was not correlated with age and did not changed with age in same patient. However, true sacral ratio and sacrococcygeal ratio were significantly lower in patients with high type ARMs than those of normal children (p<0.001). There was no difference between patients with low type ARMs and normal children. These results suggest that abnormal sacrums are more frequently encountered in patients with high type ARMs than in normal children, and that true sacral ratio and sacrococcygeal ratio can be used in the evaluation of the abnormal sacrum.

Congenital esophageal stenosis (CES) is a narrowing of the esophageal lumen from birth. Three types of CES have been described; tracheobronchial remnants (TBR), membranous web (MW), and fibromuscular stenosis (FMS). We reviewed the clinical features and the surgical outcome of 14 patients, pathologically confirmed as CES. Nine patients had TBR, 3 FMS, and 2 MVV. The mean age at operation was 3.8 years. Five patients were boys and 9 girls. Four patients had other congenital anomalies. Segmental resection of the lesion and end to end anastomosis was utilized in all cases except one who underwent myotomy. The stenotic segment was located at the distal esophagus in all patients. There were 8 complications in 6 patients, but no mortality. The mean follow-up period was 68 months. There were no feeding problems but 3 patients had minor gastroesophageal reflux. Our result indicates that segmental resection and anastomosis is a satisfactory surgical procedure in the management of CES.

Henoch-Schonlein purpura is a systemic vasculitis of unknown etiology that is probably related to autoimmune phenomenon. Henoch-Schonlein purpura is characterized by purpuric rash, arthragia, nephritis, and gastrointestinal manifestations. We reviewed 169 children hospitalized with a diagnosis of Henoch-Schonlein purpura at Asan Medical Center between 1989 and 1998. One- hundred thirty-nine (82.2%) had gastrointestinal manifestations including abdominal pain, nausea, vomiting and gastrointestinal bleeding. Surgical consultations were obtained for ten children, and laparotomy was performed on five children. Three children suspected of appendicitis underwent appendectomy. None had the evidence of appendicitis. One child who was suspected of intestinal strangulation revealed, in pathologic review, the hemorrhagic edema of the proximal jejunum and of the distal ileum at laparotomy. Another child underwent resection for hemorrhagic infarct of distal ileum. High suspicion of this disease entity in differential diagnosis of abdominal pain in children can avoid unnecessary laparotomy in most cases. However, the life-threatening gastrointestinal complications may occur in a small percent of cases. The prompt recognition and adequate radiologic evaluation of abdominal manifestation of this entity is needed for an early surgical intervention.

Pathologic lead points are found in a few intussusception patients. To evaluate the pathologic lead points in childhood intussusception, a retrospective review of 227 operated cases of intussusception treated at the Yeungnam University Hospital from January 1986 to April 1999. The patients were divided into 2 groups; idiopathic group 209 cases, (92.1 % and lead points group 18 cases, 7.9 %). Intussusception developed between age two months and six months in both groups. Enteroenteric type of intussusception was relatively more frequent in the lead point group than in idiopathic group. The lead points were veil (10 cases, 52.6 %), Meckel's diverticulum(3 cases, 15.8 %), lymphoma(3 cases, 15.8 %), ectopic pancreas(2 cases, 10.5 %), Henoch-Schonlein purpura(l cases, 5.3 %). The bowel resection rate was 44.4 % in the lead point group and 8.6% in idiopathic group. The recurrence rate was 5.56 % in lead points group and 1.44 % in idiopathic group.

The WDHA syndrome characterized by watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome) is rare, and with neurogenic tumors. A 20-month-old girl presenting with symptoms of WDHA syndrome was transferred to our hospital. She had a ganglioneuroblastoma of the adrenal gland. The serum level of VIP was elevated. After complete excision of the tumor, all symptoms related to the WDHA syndrome were relieved and serum VIP level dropped to normal. The postoperative course was uneventful. The patient was treated with postoperative chemotherapy and radiation therapy. There was no evidence of disease 33 months after operation.

Spontaneous rupture of the eventrated diaphragm is not common. The authors report a case of spontaneous rupture of the congenital diaphragmatic eventration. An 8 year-old girl with right congenital diaphragmatic eventration and nephrotic syndrome was seen in emergency room because of severe abdominal pain and vomiting. She had intermittent abdominal pain for 1 year. Plain chest X-ray and ultrasonography showed entrapped bowels in the right thoracic area. Exploratory laparotomy revealed a ruptured right eventration. THE displaced abdominal viscera were repositioned into the abdominal cavity and the ruptured diaphragm was trimmed and plicated. The postoperative course was uneventful. Only one case of spontaneous rupture of eventrated diaphragmatic has been reported in the English literature.

Although intraperitoneal teratomas are rare, teratomas of the greater omentuma are seldom reported. A single case of omental teratoma with torsion in a 26-month-old girl is reported. The patient presented with abdominal pain and a palpable mass. Ultrasonography and CT of the abdomen showed a large cystic mass with a non homgenious solid component composed of fat and calcification. The lesion was surgically excised. Pathology revealed a mature omental teratoma. The hospital course was uneventful.

A case of primary hyperparathyroidism due to parathyroid adenoma is presented. A 14-y-o male came in with voiding difficulty. The intravenous pyelogram (IVP) demonstrated a stone at the proximal one third of the left ureter and marked hydronephrosis of the left kidney, and the Tc-99m sestamibi nuclear scan demonstrated an hot spot below the lower pole of the left lobe of the thyroid. Laboratory study demonstrated hypercalcemia (12.4mg/dL) and elevated parathyroid hormone (143.67pg/mL). The excised parathyroid gland which was located below the lower pole of the left lobe of the thyroid, turned out to be parathyroid adenoma, consisting of mainly chief cells. Postoperatively he complained of transient hypocalcemic symptoms, which resolved by administration of calcium preparation and vitamin D.