Seventy-six patients with biliary atresia (BA) were seen at the Kobe Children's Hospital during the period from 1971 to 1989. We reviewed the medical records of these patients to clarify the timing of orthotopic liver transplantation (OLT) and to elucidate the role of re-do portoenterostomy. Seventy-six patients were divided into three groups according to their postoperative courses; Group I (n=35): good bile flow and serum bilirubin≦1.0 mg/dl within 2 yrs after operation. There were 3 deaths. The cause of death was ruptured esophageal varices. Another three developed intrapulmonary shunt. A few patients even without jaundice in this category, cirrhosis gradually progressed, ultimately requiring OLT. Portal vein thrombosis and intrapulmonary shunt should be carefully followed. Group II (n=27): fair bile flow but serum bilirubin>1.0mg/dl 2 yrs after operation. There were 19 deaths. Growth retardation, ascites accumulation and variceal bleeding sequentially developed in these patients when serum bilirubin exceeded 10 mg/dl and chE became low (150-200 IU/l). This seems to be the proper timing of OLT because nothing is gained from awaiting OLT at this point. Group III (n=14): no bile flow. All died except for one who had OLT. Nutritional managent is mandatory in this category for a successful OLT. Re-do Kasai operation was done in 22 patients and 11 of these are now alive including one who survived OLT. Re-do portoenterostomy for failed Kasai is encouraging because it can achieve biliary drainage good enough in about half of the patients to survive long-term in a satisfactory quality of life. OLT can be deferred, allowing the patients to grow in the meantime.

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Liver transplantation in children poses a variety of surgical problems which are distinct from those in adults. The topic of this presentation will be a review of the patterns of complications observed after liver transplantation in children. Although early reports of liver transplantation suggest that the great majority of candidates were older children, the epidemiology of liver disease and the experience of centers in the United States suggest that most patients who require liver transplantation do so before their second year. Therefore, the primary problem of pediatric liver tranpslantation is addressing the needs of these very small children. Common complications which are observerd in pediatric liver transplantation include hepatic artery thrombosis, which occurs between 5 and 25 percent of children in large series. Surgical management of this includes early diagnosis, thrombectomy and vascular reconstruction or liver replacement. While acute hepatic necrosis occasionally occurs, the most common presentation is due to fever and jaundice, biliary tract sepsis, common bile duct necrosis, or hepatic abscess. These lesions tend to be recognized late and are best managed with liver replacement. Primary non-function of a liver graft occurs in between 5 and 10 percent of transplants. It may be more common in small pediatric donors, since the donor selection criteria are often less rigorous because of the scarcity of small donors. While hypofunction can occasionally be managed expectantly with support of the prothrombin time, early retransplantation is usually the safest measure. Segmental liver transplants occasionally cause complications due to biliary leakage or necrosis along the cut section which requires reoperative therapy and drainage. Portal vein thrombosis is occasionally seen after liver transplantation in children. It never is a cause of graft failure and is usually managed readily by thrombectomy and release of tension on the portal vein. This occasionally requires interposition grafting. Biliary leaks occur after liver transplantation in children and are nearly always associated with hepatic antery thrombosis. If the hepatic artery is patent, revision of the hepatico jejunostomy over a stent affoeds a very high success rate. Ptients with biliary atresia may have extensive visceral adhensions and due to the extensive dissection required, have a 5 to 10 percent risk of intesitnal perforation. A readiness to re-explore children with fever and abdominal distension between seven and fourteen days after transplantation can prevent the severe complication of uncontrolled peritonitis. The prophylactic use of oral antibiotics to prevent abdominal infections has reduced the morbidity of these problems. Overall, surgical complications account for the majority of graft and patient loss in pediatric liver transplantation. Attention to detail and the use of good livers can reduce the incidence of these complications.

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Several therapeutic modalities have been introduced to deal with the graft shortage in pediatric liver transplantation. We have performed thrity living related liver transplantation (LRLT) procedures over the past 19 months in 30 patients who were 8 months to 15 years of age; 23 had biliary atresia, 2 liver cirrhosis, 2 Budd-Chiari syndrome, 1 Protoporphyria and 2 progressive intrahepatic cholestasis. Sixteen lateral segmentectomies, 13 left lobectomies and one right lobectomy were performed for graft harvesting. A partial orthotopic live transplant with an intact inferior vena cava was performed. Our standard immunosuppression consists of FK 506 and low dose steroids. Postoperative management has been done on Redox theory. Arterial ketone body ratio (AKBR) was serially measured every 12 hours over the first postopertaive week and once a day later as an indicator for evaluating the viabilitiy of graft liver until the graft survival was obtained. Twenty four recipients are alive and well with the original graft and normal live function. Patient survival rate was 88% (22/25) in elective cases. 6 patients died with functioning graft due to the extrahepatic complications; one aspiration asphyxia, two cardiac insufficiency, one candida infection and one lymphoproliferative disorder. Graft complications were encountered in 5 recipients. Two patients had biliary stenosis, one portal vein thrombosis, one arterial thrombosis and two hepatic vein stenosis. Three rejection episodes were obsebed in 7 ABO compatible cases and one episode in 3 incompatible cases. In all 30 cases, AKBR increased above 1.0 within 2 days after transplantation. However, the increased AKBR above 1.0 decreased again in 5 cases. The causes of the AKBR redecrease were determined to be hepatic artery thrombosis, portal vein thrombosis, massive intraabdominal hematoma, hepatic stenosis and lymphoproliferative infiltration into the liver, respectively. The decreased AKBR recovered promptly following successful surgical interventions in 4 cases. All donors have perfectly normal hepatic function and came back to the normal social lives. Our results suggested that LRLT is a very important option toward resolving the graft shortage in pediatric liver transplantation and post operative management based on the Redox theory is crucial for outcome.

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Questionaires on the number of endosopic procedures in infants and children and its complications in five yerar from 1987 to 1991 were replied by 40 institutes (74.1%) of Japan Society of Pediatric Endoscopy. Number of total endoscopic procedures was 8,722. procedures in upper gastrointestinal tract 5,408 (858 :number of endoscopic treatment in parenthesis), endoscopic retrograde cholangio-pancreticography 478 (5), colonoscopy 1,519 (229), laparoscopy 13 (2), and bronchoscopy 1,304 (124). Number of every procedure has remarkably increased in comparison with the previous observations in 1978 and 1983. Undesired side effects and complications of endosopy were reported in 18 cases (0.206%): cardiorespiratory 6, gastrointestinal bleeding 5, perforation 4, pancreatitis 2, and mediastinitis 1. The complication rate of gastro-intestinal endoscopy is 0.102 per cent, colonoscopy 0.263 per cent, and endoscopic treatment 0.092 per cent.

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Five children were diagnosed as primary hyperparathyroidism and treated in our hospital. Male to female ratio was 3 to 2 and the mean age was 12.2 years old. Hypercalcemia due to a single adenoma of parathyroid gland was found in all cases. They showed various clinical symptoms, such as abdominal pain, nausea, convulsions during sleeping and disturbance of walking. Parathyriod tumors usually could be easily identified with ultrasonogrphy or CAT scan or thyroid subtraction scintigraphy. But in two cases parathyroid tumor was not found during operation, because these examinations could not point the right localization. Then angiography and/or venous sampling were performed and they showed the right localization. In one case hypercalcemia developed after first operation. MEN was suspected but there was no familiar occurence and she had no pituitary or pancreatic tumor.

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Between January, 1985 and December, 1990, a total of 48 patients underwent Blalock-Taussig shunt operation. Of these patients, twenty-seven patients who had no preceding shunt and stenosis of anastomotic site were reviewed in terms of pulmonary artery growth in relation to the operation ages. The mean age at operation and duration of palliation were 9.9 (range 0.2 to 37) and 34.2 (range 12 to 70) months, respectively. Anomalies of these patients were: tetralogy of Fallot in 11, transposition of great arteries in 3, double outlet right ventricle in 3, tricuspid atresia in 3, common A-V canal with PS in 3, pulmonary atresia with intact ventricular septum in 4. As an index of pulmonary artery growth, pulmonary artery index (PAl) ratio (post-shunt PAI/pre-shunt PAl) was calculated. The PAI ratio significantly correlated with the operation age (r=0.57, p<0.0l), and the correlation changed at 2 or 12 month old. Accordingly, twenty-seven patients were divided into following two groups: patients aged over 12 months (group 12≦, n=11); those under 12 months (group 12&gl;, n=16), the latter group patients were divided into two subgroups: patients aged over 2 months (group 2≦, n=7); those under 2 months (group 2>, n=9). Group 12> showed a significantly increased PAI ratio compared to group 12≦ (12≦ : 121 ± 28, 12> : 219 ± 95%, p<0.005), and then, the PAI ratio increased significantly in group 2>compared to group 2≦(2≦ : 158 ± 22,2> : 264 ± 104, p<0.025). In summary, the greatest growth of pulmonary artery was observed in patients operated under 2 month old. Therefore, in order to expect a sufficient growth of pulmonary artery, Blalock-Taussig shunt should be performed in early infancy.

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Between April 1984 and December 1991, six patients, three boys and three girls, with unilateral multicystic dysplastic kidney (MDK) were referred to the Department of Pediatric Surgery, Kagoshima University Hospital. The prenatal diagnosis by ultrasonography was unilateral hydronephrosis in 2 cases, bilateral hydronephrosis in 1 and cystic lesion of the kidney in 3. The accurate diagnosis of unilatefal MDK was made by ultrasonography after birth in all cases. The right kidney was affected in 2 cases and left in 4. Four patients were asymptomatic. One patient had intestinal obstruction due to the enlarged affected kidney. The urgent nephrostomy was performed in one patient with contralateral hydronephrosis and anuria. Further examinations, including intravenous pyelography, computed tomography and 99mTc-DTPA (diethylen triamine pentaacetic acid) renography, revealed normal contralateral kidney in 5 patients. Four of them had resection of affeted kidney. The age at operation was ranged from 28 days to 1 year. The diagnosis was confirmed microscopically. The management of the asymptomatic patient with unilateral MDK is controversial problem. The long follow-up must be a stress for the patient and his or her family. We conclude that earoy surgical intervention for this anomaly is recommended due to the possibility of hypertension or malignant change.

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Five cases with congenital diaphragmatic hernia who developed symptoms within the 1st 24 hours were reviewed. The value of the AaO_2 measured on admission was 570mmHg on the average. The difference in p02 between the right radial artery and the arteria dorsalis pedis (hereafter abbreviated as PPD) was examined. The PPD within 15 hours after birth were more than 30mmHg in all five cases and in four the PPD dissappeared within three days after birth. The real-time two-dimentional doppler echocardiography by use of an autocorrelator with a high speed calculator and a color processor (hereafter abbreviated as CD-Echo) was examined. CD-Echo showed the enlargement of the right atrium and the right ventricle, the shunting through the ductus arteriosus or the foramen ovale and significant tricuspid regurgitation in all five cases. By modified Bernoulli equation maximal systolic pressure gradient between the right ventricle and the right atrium (hereafter abbreviated as ⊿P) can be estimated by the peak flow velocity of the tricuspid regurgitant jet. When the pulumonary valve or the outflow of the right ventricle are not stenotic, pulmonary arterial pressure can be conjectured by the value of the ⊿P. Transition of the shunting and ⊿P was coincident with clinical course. If the value of the ⊿P does not declined, it is necessary to research and treat the cause of persistent pulmonary hypertension. The importance of this observation for new born infants with severe congenital diaphragmatic hernia is that a reliable, noninvasive technique exsists for estimating ⊿P and, by inference, PA pressure and through this finding the effective therapeutic manipulations based on the infant's hemodynamic state can be made.

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We report two cases of congenital biliary dilatation (CBD) with anomalous arrangement of the pancreatico-biliary ductal system (AAPBDS) associated with annular pancreas. One was a twelve years old boy who had associated disorder of pancreatolith and the other case was a three years old girl who had pancreatic heteropia. It is considered that a combination of AAPBDS and annular pancreas is very rare. A series of disorders of the ventral pancreas may cause the formation of these anomalies at about the six week of gestation. We consider that when we see neonates with annular pancreas, contrast studies of the pancreatico-biliary duct are necessary.

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Two cases of pancreatoblastoma in childhood are repoted. Case 1: A 6-year-old girl was admitted with jaundice. She was found at surgery to have a 4.5×4.5×4cm mass at the head of the pancreas with a solitary liver metastasis. The tumor was successfully treated with pancreaticoduodenectomy, and ethanol infusion therapy was performed for the liver metastasis. Tumor cells were positive for α_1-antitrypsin (α_1-AT), CEA, AFP, and chromogranin. Case 2: A 4-year-old boy was referred for evaluation of a large abdominal mass. Serum AFP was elevated. An exploratory laparotomy revealed an encapsulated tumor mass with a smooth surface measuring 12.5×11×6cm. No metastasis or invasion was found. The tumor together with the tail of the pancreas was completely excised. Tumor cells were positive for AFP and α_1-AT. Postoperative convalescence in both patients was uneventful. Chemotherapy was given, and both have been well during 8-month follow-up periods. In both patients, serum trypsin and elastase I values were useful in the determination of clinical course. A review of the Japanese literature regarding pancreatoblastoma published between 1977 and 1991 revealed 22 other cases, patients aged 4 to 5 years and boys predominated. The chief complaint was abdominal mass, followed by abdominal pain and distention. Jaundice was an uncommon feature. Pancreatoblastoma was accompanied by metastasis or direct invasion in 16 cases, and the most common site was the liver. Eighteen patients underwent complete removal of the primary tumor, while in 6 it was inoperable due to the advanced stage of the disease. Chemotherapy and/or radiotherapy was performed in 17 patients. Eleven were still alive while 9 had died during follow-up periods ranging from 1 to 52 months (mean, 19 months) after the time of diagnosis.

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Abdominal injuries caused by automobile seat belts have recently become a great danger to the passengers. Although many pediatric cases have been reported overseas, only two have been reported in Japanese literature. The authors successfully treated the case described below. A sleeping 6 year-old girl, wearing a lap-type belt sitting in the rear, was involved in a high-speed car accident. She was transferred to the authors' hospital because of abdominal pain with a contusion, diffuse tenderness, and muscular defense on the abdominal wall. A plain X-ray film and CT scan showd free air in the abdominal cavity. A Laparotomy was performed, on suspicion of intestinal perforation. This confirmed a huge hematoma in the root of mesentery, laceration of the mesentery including a 5 cm-long devascularized jejunal intestine, and also perforation in the ileum. Resection of devascularized jejunum, repair of perforation and laceration were performed. She recovered uneventfully. By contrast, the patient's 12-year-old elder siste, also asleep in the rear suffered only a bruise to the knee. It is notable taht she wore no seat belt.

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A case of 7-day-old girl presented with so-called spontaneous pneumoperitoneum. She was referred to Ishikawa Prefectural Central Hospital becasuse of gastroinetestinal bleeding and pneumoperitoneum. On suspicion of peptic ulcer perforation, upper gastrointestinal endoscopy, followed by laparotomy, was performed under general anesthesia. Although endoscopic examination revealed an active duodenal ulcer, no perforation site was found out at laparotomy. Without endoscopic examination, she would have been diagnosed as spontaneous pneumoperitoneum. Her postoperative course was uneventful, managed with anti-biotics and H_2 blocker. The final diagnosis was spontaneous closure of duodenal ulcer perforation. Endoscopic examination is a useful method in order to clear up the cause of pneumoperitoneum in the newborn, when laparotomy has failed to find out the gastrointestinal perforation.

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Rectal prolapse is a common complication after pull-through operation for high imperforate anus. The varying degrees of exposed mucosa often causes a flow of mucus or soiling, occasional bleeding, and pain. Simple resection of the redundant mucosa is unsatisfactory and leads to frequent recurrences or strictures. Millard and Rowe have reported a technique designed to correct rectal prolapse using two perineal flaps, thus providing a skin-lined anal canal. We have operated on three patients with postoperative rectal mucosal prolapse using the same technique. With an average follow-up of 2-years, none of them presented recurrence of the prolapse or a significant stenosis. The skin lined anal canal provided some sensation in a boy with a high imperforate anus. We describe the technical details of this easier and safer anoplasty for rectal prolapse after pull-through operation.

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This is a case report of a mature teratoma originated from intraabdominal undescended testicle in an infant.The patient is a 10 months old male. He was transferred to our hospital with right sided abdominal tumor and intestinal obstruction. He had left undescended testicle. On examination his abdominal tumor was thought to be ateratoma. Laparotomy was performed and the tumor was found to be originated from his left undescended testicle.Histopathological examination of this tumor revealed mature teratoma.

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Cystic neuroblastoma is a rare form of neuroblastoma. Two cases of cystic neuroblastoma are reported. The first case was a one-year and six-month-old girl who had a large mass in the left quadrant. 14×9×8cm tumor in the retroperitoneal space was removed. The tumor was multilocular, and contained bloody fibrinous exudate. The pathological diagnosis of the cyst wall was neuroblastoma with rosette formations. The patient did well after surgery and had no evidence of metastasis or recurrent tumor at 1-year and 10-month follow up. The second case was a seven-month-old boy whose chest X-ray showed soft tissue mass protruding from mediastinum to the right lung. At surgery, 7×5×5cm tumor in the posterior mediastinum was removed. The tumor was unilocular and contained yellowish fluid. The pathological diagnosis was ganglioneuroblastoma. He had no recurrence of the tumor in the 19 years since surgery. Sixteen cases of cystic neuroblastoma have been reported to date. The clinical features and pathogenesis were reviewed in the literature.

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We treated a case of congenital pyloric atresia of a 4-day old male baby. The mother of the baby had polyhydramnios. Ultrasonography revealed a large cystic mass in the abdominal cavity at 33 weeks of gestation. At that time, an upper gastrointestinal atresia was suspected. The baby was referred to our hospital soon after his birth. A plain X-ray of the abdomen showed a single gastric air bubble, and the upper gastrointestinal series revealed that the barium was not passed through the second portion of the duodenum. On the 6th day after birth, the operation was performed. There was a membranous diaphragm completely occluding the lumen in the region of the pylorus, and there was an ulcer on the posterior wall of the pylorus. The excision of the diaphragm was done, and Heineke-Mikulicz pyloroplasty was followed. Congenital pyloric atresia is a rare case and 48 cases have been reported in Japan, and 10 cases were associated with epidermolysis bullosa. Our case was not associated with epidermolysis bullosa. There are 7 cases including our case which have been diagnosed before delivery through ultrasonography. The early diagnosis is expected to improve the prognosis of such a case.

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Operative repair of giant omphalocele remains some technical problems yet. We described one unruptured giant omphalocele with severe congenital heart disease of which delayed primary closure could be achieved. At bedside, sticking plasters were adhered over the skin of the lateral abdominal wall. Twice per day the attached plasters was squeezed, the omphalocele sac along with the herniated viscera were inverted into the abdominal cavity under no anesthesia. While squeezing sticking plasters, the venous pressure of her lower extrimity was continually monitered to avoid pressures greater than 20cmH_2O. After 16 days, the linea alba was approximated while leaving the sac intact and folding it into the abdominal cavity under general anesthesia. The simple technique requires no anesthesia and avoids complications associated with operative silo placement

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