Hepatoblastoma (HB) and hepatocarcinoma (HC) comprise 2/3 of primary hepatic tumors of infants and children. As many as 1/3 of patients whose HB have been completely excised do not survive. In Children's Cancer Group Studies 60% of patients whose HB or HC (regardless of histology) were completely excised and treated with chemotherapy were disease free at 24 months; there was no significant difference in median survival between HB and HC; the fibrolamellar variant of HC was more readily resectalbe than typical HC but its unique histology did not signal a better outcome; mitotic activity in HB was associated with a poor prognosis; tumor necrosis and vascular invasion did not influence prognosis; pure fetal hisotlogy in completely resected HB was associated with improved outcome when compared with all other completely resected HB histologic subtypes; a "favorable" effect of pure fetal histology was not apparent for tumors which could not be completely resected; the absence of mitotic activity and the presence of differentiated elements such as osteoid or chondroid matrix and squamous epithelium was associated with improved survival in unresectalbe HB. DNA content of some HB correlates with prognosis. 50% of anaplastic or embryonal histology HB that exhibit DNA aneuploidy, also demonstrate vasuclar invasion and poor outcome. Chemother-apy aids surgical management of HB and HC rendering 59% of 34 unresectable tumors amen-able to later removal. In such cases, HB had a superior prognosis to HC. Tumors made resectable by chemotherapy may exhibit extensive necrosis. Treated HB speclmens undergo "changes" in histol-ogy and may revert solely to differentiated mesen-chymal elements. This chemotherapy effect may be important to the success of liver transplantation for unresectabel HB since vascular invasion has been associated with unfavorable results. Trisomy 20, trisomy 2q, double minute chromosomes, and translocation of 22q to 10q have been demonstrated in HB. Children of families with familial adenomatous polyposis are at increased (800 fold) risk for developing HB; their HB may be pathogenetically and clinically unique. Malignant rhabdoid tumor of the liver is a clinicopathologic entity characterized by presentation in early life, spread at time of diagnosis, a rapidly fatal course and male predominance. At least case appears to have responded to 11 month's chemotherapy so that the necrotic hepatic tumor could be removed and pulmonary metastases were no longer visible in imaging studies.

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A program in living related liver transplantation was initiated at the University of Chicago in 1989. The initial protocol studied 21 consecutive children receiving 23 living related liver transplants during a 14 month period. This initial series demonstrated that the donor operation could be performed safely and that patient and graft survival were 80 and 70 percent respectively. Based on this initial experience accumulated in our hospital, we have initiated a program in which living related liver transplantation is offered as a routine option for children requiring liver transplantation. Since this initial series, 19 patients have received liver grafting, with a live donor accounting for 40 percent of the patients transplanted during this period. Of this series, 18 of 19 patients are alive between and 3 and 12 months post transplantation. A single patient died due to septic complications of unsuccessful treatment for rejection. Two other liver grafts were replaced successfully due to arterial thrombosis. All donors have fully recovered and no chronic health problems have been observed in a total of 42 donors in the entire series. We propose that liver related transplantation is a feasible technique yielding extremely high patient and graft survival and that it offers an acceptable option for the alleviation of the shortage of liver donors.

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The etiology of meconium ileus without mucoviscidosis remains uncertain. There have been various literatures and theories posturated. None of these studies, however, has directed at the myenteric plexus. In this study, the myenteric plexus of the intestine was investigated morphologically and cytometrically in 8 cases of this disease. The number of ganglion cells in myenteric plexus showed normal level, but the size of ganglia revealed significantlly small in the nuclear are a which considered to correspond to that of 5-6 months in gestational age. At the time of enterostomy closure, intramural ganglia was observed to be matured. The results lead to the conclusion that the immature state of the myenteric plexus in the terminal ileum and colon seems to be the leading etiologic pathology of the meconium ileus without mucoviscidosis.

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A total of 383 infants with anal fistula (perirectal abscess) were studied. This disease was frequently seen in infants less than six months of age, usually started as a perianal abscess, then getting to form subcutaneous fistula to direct lateraly. Three or more lesions located in the median sites often occurred at the lower age group. The incidence of a single lesion tended to increase when patients aged one year or more, where lateral lesions were less frequent. Diseases with early onset at lower age and the presence of multiple lesions were factors to prolong treatment, The out-patient compression method seems to be the treatment of choice when self-limited condition and patients' burden are taken into account.

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Thirteen children with congenital biliary dilatation (Choledochal cyst) were treated by choledochocystoenterostomy and 2 of them had reoperation of cyst-excision and reconstruction of the biliary tract because of pancreatolithiasis and chronic relapsing pancreatitis. They had anomalous junction of pancreatobiliary ductal system (AJPBDS) . It was speculated that choledochocystoenterostomy would promote the activation of pancreatic enzymes in the choledochus, and accelarate the damage of not only the biliary tract but also the pancreatic duct and tissue under AJPBDS. Excision of choledochocyst and biliary reconstructive procedure for congenital biliary dilatation should be adopted because of these late complications.

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Four patients with severe congenital diaphragmatic hernia (CDH) , who had persistent hypoplasia of the ipsilateral lung, developed hyperinflation of the contralateral lung following successful repair of the hernias. In the first two cases, managed by conventional mechanical ventilation, the contralateral lungs developed hyperinflation 10 and 40 days, respectively, after the operations. Weaning from mechanical ventilation was very difficult in these cases. One patient died from severe lung damage 89 days after his operation. Another patient was successfully weaned from mechanical ventilation 109 days after her operation. Two other cases were treated by V-A ECMO for 48 and 85 hours, respectively, after the operations. They were managed on "lung rest" status during ECMO. Both of them also developed hyperinflation, one 16 and the other 33 days after the operations. However, they could be successfully weaned from mechanical ventilation 47 and 42 days after their operations. From our results, we deduce that ECMO support may reduce the risk of barotrauma or lung damage following repair of severe CDH.

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The purpose of this study was to investigate the rate of gastric emptying of infant formula thickened with cornstarch for the treatment of gastroesophgeal reflux. The rate of gastric emptying was determined by using a thickened formula labeled with Tc-99m DTPA and compared to the rate of standard formula. Ten infants with GER were evaluated. In two infants, thickened formula appeared to display a noticeably delayed gastric emptying compared to the standard formula. In four infants, the thickened formula indicated enhanced gastric emptying. In another 4 infants, the use of thickened formula showed the same emptying rate. These data suggest that the antireflux effect of thickened foumula may play a significant role in the treatment of gastroesophageal reflux in infants with enhanced gastric emptying, and that the antireflux effect of thickened formula may be uneffective in infants with delayed gastric emptying.

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Between December, 1986 and November, 1991, a total of 15 patients underwent correction of total anomalous pulmonary venous drainage (TAPVD) . The mean age at operation was 42.5 days (range 1 day to 4 months) Postoperative pulmonary venous obstruction (PVO) occurred in five patients between 6 weeks and 11 months after operation. The mean age at operation was 15.0 days (range 1 to 37 days) . A total of eight reoperations were performed in five patients ; one in 3 patients, two in 1, three in 1. Two patients died just after the first reoperation, and one patient died two years after the second reoperation by asthma and liver dysfunction. In one patient who underwent reoperation in 3 times, left pneumonectomy was performed two months after the third reoperation because of diffuse narrowing of the pulmonary veins and severe inflammation. He is showing good physical growth since then. Findings of postoperative PVO were divided into three types ; anastomotic stricture (in 1 patient) , discrete stenosis of pulmonary venous ostia (in 2) and mixture of both type (in 2). In addition, the ostia stenosis included diffuse narrowing of pulmonary veins in 2 patients. Although correction of anastomotic stricture is relatively straightforward, reoperation for the other types including diffuse narrowing of pulomanary veins remains a therapeutic challenge. In conclusion, it is most important to make a concordant adaptation of anastomotic line between left atrium and common pulmonary vein for the prevention of ostia stenosis.

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Opsoclonus-polymyoclonia syndrome (OPS) is one of the features of neuroblastoma. We experienced a case of neuroblastoma associated with OPS. This 3-year-old girl had complained of gait disturbance for eight months. At physical examination, a hard and large mass was palpable in the right upper abdomen, and she showed horizontal nystagmus, tremor of fingers, trunkal ataxia, and cerebellar ataxic gait. Laboratory data revealed elevation of serum LDH, NSE, and ferritin, and the urinary excretion of VMA and HVA was elevated. Both CT scan and MRI revealed a huge tumor in the right retroperitoneal space which invaded to the oposite side and involved superior mesenteric and bilateral renal arteries. Bone scan revealed no evidence of bone metastasis. Exploratory laparotomy and iliac bone marrow aspiration were performed, and pathological diagnosis was neuroblastoma (rosette-fibrillary type) stage IV B. N-myc analysis revealed single copy. After three courses of chemotherapy (CPM, THP-ADR, CDDP, and VP-16) , total excision of the tumor and intra-operative irradiation to the tumor bed (6MeV, 12Gy) were performed. After the operation, she has been still on post-operative chemothrapy, however all of the neurological symptoms have disappeared completely.

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A case of hemangioendothelioma originated in the porta hepatis with the symptom mimicking to that of biliary atresia is presented, which is the first case ever reported in the world. A 4-month-old female was admitted due to jaundice. Abdominal echogram revealed a distended gallbladder, stenosis of common hepatic duct at porta hepatis surrounded with high echoic area, and dilatation of the left intrahepatic bile duct. Percutaneous cholangiography showed complete obstruction at porta hepatis and normal bifurcation of intrahepatic bile duct. The diagnosis of biliary atresia was made, and the abdomen was explored on 139-days after birth. The hepatoduodenal ligament was invaded by sclerotic mass from porta hepatis to T-junction including cystic duct continuously, and bile duct in its sclerotic portion was obstructed completely. All sclerotic portion was resected, and portoenterostomy (original Kasai's procedure) was performed. The histological apperance of specimen was benign hemangioendothelioma surrounding common hepatic duct, cystic duct, and common bile duct. In a 37-mo follow-up, the child has remained well, and is completely asymptomatic.

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The case was a female born at the 39th week with weight of 3,040g. At 6 hours after the birth, she developed cyanosis followd by shock condition and was hospitalized. Her symptoms were improved by treatments, but a mass was palpated at her right hypochondrium, and jaundice and white feces were noted. RI examination at the initial stage showed bilialy obstruciton. The subsequent ultrasonic examination and the repeated RI sintigram illustrated the dilated commonn bile duct. At the age of 51 days, cholecystectomy and drainage operation were conducted. The surrounding tissues in the abdomen showed precipitation of bile pigments which suggested perforation of bile duct. Contrast radiography demonstrated the dilatation of common bile duct 10mm in diameter. At 4mounths of her age, the dilated choledochus was resected and Roux-en-Y hepatico-jejunostomy was conducted. After one year from the operation, she is in good condition.

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In our hospital, 66 cases of biliary atresia have been operated on for the past 15 years. Four cases of them were associated with preduodenal portal vein and other anomalies. Kasai procedure have been undertaken in these 4 cases from 63 to 85 days old. But no one could survive long enough, possibly because of presence of the abnormalities of the connective tissue and vessels at the porta hepatis in biliary atresia associated with predudenal portal vein.

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Appendicostomy has been used for decompression or drug injection in diseases of lower alimentary tarct, because of its anatomical properties and tendency of spontaneous closure after removal of the tube. We had two cases of the appendicitis in the abdominal wall after closure of appendicostomy. One case is 9-year-old boy, who had suffered from ileal atresia and had received ileal resection and appendicostomy in the neonate. The other is 13-year-old girl, who had received' resection and enterostomy of the small intestine in the neonate because of meconium plug syndrome, and had received closure of enterostomy and appendicostomy at 6 month. Both complained right lower abdominal pain, and skin rush and tenderness were recognized at the scar of appendicostomy. A hypoechoic lesion continuing to the cecum was demonstrated by the echogram. After the symptoms decreased with conservative therapy, elective appendectomy was done. In our department, we had 11 cases of appendicostomy in neonates and infants for the last 25 years. Most cases were ileal atresia. Appendicostomies made for decompression worked well, but there were several complications after removal of the tube. It seemd necessary to perform mucosal cautery or appendectomy at the closure.

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The combination of congenital biliary atresia (CBA) and hepatocellular carcinoma (HCC) is rare. However with the increasing number of patients with CBA, the significance of its assosiation with HCC is becoming more important. This paper presents a case of CBA associated with HCC. The patient was a 16 years-old girl. She was opeated on in our hospial 140 days of age after having been diagnosed as CBA (the type of I cyst) . Intraoperative cholangiography showed that development of intrahepatic bile ducts were cosiderable and the wall of the left hepatic bile duct was irregular. Histological examination of the liver revealed biliary fibrosis. After the operation she showed no further symptoms until she complained of fever, jaundice and epigastric pain at 16 years old. Laboratory data revealed high levels of AFP (> 50,000ng/ml) , CEA (14ng/ml). HBsAg was negative. A 7cm hypovascular mass was detected in the lateral segment of the liver using CT scan, echograpy and angiography. Needle biopsy was carried out and a well differantiated HCC was diagnosed. 5'FUDR was deliverd but she died 5 months after the onset of the symptom. This was thought to be the first case where a patient has been free of jaundice until association with HCC. This case would show that it is important to cosider the association with HCC in the follow-up period after an operation for CBA.

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Intrathoracic kidney in childhood is rare. The auhors expeienced two such cases and which we reported here. Case 1 : A 6-year-old boy was transferred to the authors' hospital because X-rays showed a mass shadow on the chest. He was examined using IP, echography, CT and MRI. He was diagnosed with a right intrathoracic kidney. He had a history of right diaphragmatic hernia and had undergone an operation at the age of one day. The hernia was noted in the center of the right di-aphragm, and it involved segments of intestine and the right lobe of the liver in the thin sac. Case 2 : A 6-month-old girl was admitted to the authors' hospital for the open heart surgery. Chest X-rays showed a mass shadow on the right lower posterior thorax. After examination, she was diagnosed with a right intrathoracic kidney. She had a history of omphalocele and had undergone an operation three hours after birth. The renal function of both patients are now normal, and they are being followed up using the echogarphy. Several cases of intrathoracic kidney with congenital diaphragmatic hernia have been reported already. In these cases, a kidney was involved in the hernia. But, in our case, the kidney was not involved, and it was located on the posterior median side of the right lower thorax. Moreover in our second case, the conditions was associated with an omphalocele. To the best of our knowlege, such cases has not been reported in the literature.

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Wandering spleen is an anomaly which is caused by an anomalous development of the dorsal mesogastrium. We experienced a case of wandering spleen with torsion of the pedicle. This 5-year-old boy was admitted to our hospital becuse of abdominal pain and vomiting. On physical examination, he showed tenderness and muscular defense in the whole of the abomen. A firm and movable mass was palpated in the left middle abdomen. Abdominal X-ray revealed a tumorous shadow in the left middle abdomen. Ultrasonography revealed the mass to be a mobile swollen spleen, and a wandering spleen was suspected. Both enhanced CT-scan and <99m>^Tc-sulfur colloid scanning revealed splenic blood flow remained. Color doppler ultrasonography revealed the hepatofugal splenic arterial blood flow and hepatopetal splenic venous blood flow in the site of splenic hilus. Under the diagnosis of a wandering spleen with torsion of the pedicle, urgent laparotomy was performed. At laparotomy, we found a wandering spleen attached only at its hilum by the pedicle to other abdomianl structures. The spleen had clockwise rotated about its pedicle twise, mesured 13 × 9 × 5cm in size, and appeared congested. When the torsion was released, the congestion was improved, and there was no sign of splenic necrosis. Splenopexy was performed. He has been doing well without recurrence of torsion for the last two years.

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Four cases of successful sugical repair of congenital corrected transposition of the great arteries are reported. The de Leval method repairing VSD through the mitral valve rather than the morphologic left ventriculotomy was used in all four cases. One patient whose A-V conduction system could not be confiremed during surgery was suffered from surgical A-V block. In one heart of valvular PS, pressure gradient was satisfactorily reduced by commissurotomy. In two hearts with severe infundibular PS or atresia, valved external conduit bypass was performed between right side ventricle and main pulmonary artery for reconstruction of the venous ventricular outflow tract. Postoperative courses of these cases were uneventful except for one A-V block. Color doppler echocardiography and post-operative catheterization showed the reduction of the right sided ventricular pressure, no leakage of VSD, and good ventricular function.

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A boy was transported to our hospital 45 minites after birth, who was borned via vaginal delivaryat 39 weeks in gestation and 3,454g in birth weight. The chief complaints were respiratory distress and pale colouration of the skin. Several abnormal laboratory data were obtained including 10.4g/dl of Hb, pH 7.055, BE -23.3mEq/l, HCO_3 6.3mEq/l in arterial blood analysis. The chest plain X-ray and ultrasnography reveraled both the mass at upper mediastinum and the left lung deviation to antero-caudal direction, with massive echo-free space which was thought to be compatible with hemothorax. When the chest tube was inserted, fresh blood of 75ml was drained. After chest-tube insersion, although bleeding was controllable with blood transfusion, the patient developed persistent fetal circulation. Despite the pharmacological and ventilatory support with high frequency oscillation, respiratory distress was not improved. The baby died 22 hours after birth. The autopsy showed neuroblastoma originated from the left paravertebral column at the Th2-3 level, and the capsule of caudal side of the tumor was disrupted. The histological subtype of the neuroblastoma was rossete-fibrillary type.

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