To investigate the influence of open heart surgery using extracorporeal circulation (ECC), on perioperative hypokalemia and also on neurohumoral factors of potassium metabolism' a prospective balance study was made in 21 patients (mean age 61 ± 43 months) with various congenital heart disease. Population of the patients were : ventricular septal defects 6, atrial septal defects 4, tetralogy of Fallot 2, complete atrioventricular septal defect 2, coarctation complex 2, triatrial heart 2, and so on. Plasma potassium was measured before and during operation, and hourly for 24 hours after closing the chest. Urinary values of potassium, aldosterone and antidiuretic hormone (ADH) were also measured in accumulated urine before and during ECC, and every 6 hours for 24 hours after operation. The following results were obtained ; 1) Plasma potassium was significantly lower during ECC (3.1 ± 0.12 mEq/L) and during the first 6 hours postoperatively (3.2 ± 0.09 mEq/L) than before operation (4.2 ± 0.08 mEq/L). 2) Urinary excretion of potassium increased markedly from the beginning of ECC and continues until closure of the chest, and this increase correlated with the urine volume (r = 0.77, p < 0.01). 3) The balance study of potassium during this period, however, demonstrated that, the amount of replenished potassium was larger than the urinary potassium excretion and the difference correlated inversely with the rectal temperature (r = -0.65, p < 0.0l). 4) Plasma potassium correlated inversely with PH (r= -0.56, p < 0.01) and positively with Pco_2 (r = 0.57, p < 0.01) during the first 6 hours postoperatively. 5) Urinary aldosterone correlated positively with urinary potassium (r = 0.54, p < 0.05) and inversely with plasma potassium (r = -0.49, p < 0.05) during the period from 18 hours until 24 hours postoperatively. 6) The urinary aldosterone and ADH increased markedly after ECC and remained high until 24 hours postoperatively, while the plasma ANP did not change significantly. These results draw us to conclude that both increased urinary excretion of potassium and potassium influx into cells during hypothermic ECC may play important roles as the causes of the hypokalemia seen in the early postoperative periods. Furthermore, respiratory alkalosis and hyperaldosteronism may exaggerate the hypokalemia.

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The results of intraoperative liver biopsy performed on 56 clinical cases and the findings of the abnormal pancreatico-choledochal duct junction in 63 expermental canine-models are discussed in this paper. Histopathological findings revealed that fibrosis, small round cell infiltration at the portal area and bile duct proliferation were present in 80% of the patients. Among the 8 infantile patients, giant cells were observed in 2 patients, 1 of them also had cirrhosis. Another of infantile patient also had cirrhosis. Edematous change was present in the jaundiced cases, but marked fibrosis was also seen in the cases without jaundice. In the patients with cystic shape of choledochus, fibrosis was frequently found, while in those with fusiform shape, it was rarely observed. There was no significant correlation between liver pathology and intrahepatic bile duct dilatation or common hepatic duct stenosis. Cases reoperated after cyst-enterostomy had marked lymphocyte infiltration and fibrosis. The second biopsy done after radical operation showed no histological improvement within 1 year, despite the disappearance of clinical symptoms. Among the exprimental canine models, the long time survival cases, especially those which survived more than 5 years, showed slight to moderate lymphocyte infiltration and fibrosis. But no cirrhosis or giant cell formation was observed. Liver damage was considered to be caused by distal bile duct stenosis originating in the abnormal pancreatico-choledochal duct junction and furthermore exacerbated by regurgitated pancreatic juice or infection of the biliary tract. Regurgitated pancreatic juice could bring about moderate fibrosis in the long term, but seems not be able to cause cirrhosis.

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Two hundred and sixty seven infants with hypertrophic pyloric stenosis (HPS) were treated at our institution from January 1966 to December 1989. According to the year of admission, the four study groups, group (66-69), group (70-74), group (75-79) and group (80-89) were devided and reviewed on differences in sex, birth weight, birth order, body weight at admission, age at presentation of vomiting, serum electrolytes and blood gas analysis at admission. No significant differences are found in demographic characteristics. On the other hand, the mean values in group (75-79) for serum chloride (96.2 ± 8.0mEq/l), base excess (0.98 ± 5.0mEq/l) are significantly different between the group (66-69) and the group (70-74). The duration of vomiting becomes shorter year by year, and is associated with the changes of clinical features. This study makes it clear that the abnormalities for serum electrolytes and base excesses in the recent cases of HPS tend to reduce and these changes result from the shortening of the duration of vomiting.

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Eight high risk cases of esophageal atresia (Gross C type) were managed by esophageal banding using a silastic sheet. Four of the patients died because of the associated major cardiac anomalies. Other patients were alive and underwent anastomosis of esophagus satisfac-torily. In all cases, there was no sign of reflux of gastric contents to the trachea after the banding. It was considered that this procedure was safe and useful in the management of extremely premature or critically ill babies with esophageal atresia and tracheoesophageal fistula. On the other hand, unfavorable complications were encountered in some of our cases. They included disruption, stricture and weakness of the banding site, histological dissection of the muscle layer and reduced peristalsis demonstrated by the esophagogram with contrast medium and by the manometric study. It was found that complications were related to tightness and duration of the banding. In conclusion, we would recommend a loose banding to the extent of "wrapping" and a short duration of the banding within two months.

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The multicystic kidney represents a severe form of dysplasia without any regular lobar development or normal caliceal drainage system. Surgeons generally have opted for nephrectomy, although the precise indications for removing these kidneys have been unclear, we assessed the effect of prenatal and postnatal diagnosis on the presentation of multicystic kidney, and documented the natural history of multicystic kidney that have been left in situ and reappraise the indications for nephrectomy. A total of 21 cases with multicystic kidney have been presented during 17 years in our institution. Of these cases 11 were referred for postnatal evaluation. Two bilateral cases had fatal outcome. Of remaining 19 infants with unilateral lesion, twelve infants have been managed conservatively and 7 underwent nephrectomy. Of the latter cases 5 infants who had no clinical symptoms underwent prophylactic nephrectomy in early part of thid study. The remaining 2, neonates underwent nephrectomy during the last 5 years, since it seemed that abdominal mass might be contributing to the failure to thrive. Anomalies of the contralateral. upper urinary tract included vesicoureteral reflux in 3, pelviureteric junction stenosis in 3, ureteral stenosis in 2, double ureter in 1 and horseshoe kidney in 1. Amang 12 children managed nonoperatively, there was no symptom except for cases with associated anomalies. None had malignant transformation or hypertension. Four patients were lost to follow up. Decrease in absolute dimensions of multicystic kidney was apparent in 6 patients. No change in absolute dimensions but relative decrease in relation to the growing child was noticed in 2 patients. We believe that a more conservative approach should be taken toware mujticystic kidney, unless other distinct reasons indicate the necessity of surgical removal. Careful lifelong surveillance of the patient is needed to detect the late complications.

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A boy with interrupted aortic arch (Celoria-Patton A type) associated with ventricular septal defect (VSD) underwent extended end-to-end direct anastomosis through the left thoracotomy at 24 days of age and patch closure of VSD at 3 months. There was no pressure gradient between the upper and the lower limbs at the time of radical operation. However, it became 30mmHg two and a half years later. The patient has been growing well. This repair of the aortic arch, a modification of direct anastomosis first used for coarctation of aorta associated with hypoplastic aortic arch, is useful also for initial repair of interrupted aortic arch because the lower risk of residual stenosis is anticipated than other methods.

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A case of adrenal malignant pheochromocytoma with liver, bone, and lymph node metastases, in a 7-year-old girl, is reported. The primary tumor and metastatic tumors of liver and lymph node were surgically excised. After operation, she was treated with combination chemotherapy for advanced neuroblastoma. By the end of 8 cycles of chemotherapy, she had a decrease in both plasma and urine catecholamine concentration and there was improvement on the recent bone scan. At present, plasma noradrenalin level is slightly elevates but she is nearly asymptomatic. Although follow-up is still short in duration, combination chemotherapy is effective in our case. Malignant pheochromocytoma is a rare neoplasm and 114 cases have been reported in japan. Among 114 patients there were 66 males and 48 females (including 7 children, one boy and 6 girls) and our patient was the youngest. In 75 patients (65.8 per cent) the primary tumors were located in the adrenal glands. The remaining 39 patients (34.2 per cent) had the extra-adrenal tumors. In two cases of malignant pheochromocytoma, multiple metastases were observed 10 to 12 years after the initial operation under the diagnosis of benign pheochromocytoma in childhood. The diagnosis of malignancy in pheochromocytoma is difficult since benign and malignant pheochromocytomas have similar histologic features. The patients with pheochromocytoma shoud be followed closely and longer.

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A case of renal and skin hemangiomas in a female infant was reported. The skin tumors were found on the buttock and on the left arm at birth, and the abdominal tumor was palpated at the age of 4 days. Echogram and CT-scan revealed a 4 × 4 cm tumor in the right kidney. Right nephrectomy and resection of the skin tumors were performed. Those tumors in the right kidney and the skin pathologically examined were capillary hemangiomas. We reviewed 61 cases of renal hemangioma reported in Japan. The case we reported was the first case of renal hemangioma found in a neonate.

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We present a case of bronchopulmonary foregut malformation in a three month old girl. An esophagogram demonstrated a large esophagobronchial fistula extending from the distal esophagus to the large bronchus on the right side. Tracheobronchogram showed a blind end of the proximal portion of the right main bronchus. We performed right pneumonectomy and resection of an esophagobronchial fistula at the fifth month and rescued the patient. Microscopically the fistula revealed tublar structure with both bronchial and esophageal wall.

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A full-term female was transferred to the neonatal intensive care unit at one day of age because of respiratory distress syndrome. The infant had passed normal meconium whle abdominal distension and bilious vomiting had been. At 3 days of age the infant began passing bloody stool. An abdominal X-ray film showed free air in the abdominal cavity. However, further sings of neither necrotizing enterocolitis nor panperitonitis developed. The gastrografin enema examination revealed no lesions in the colon. At 13 days of age, operation was performed. Jejunal atresia with perforation at the oral side was found. It was suggestive that the atresia had been caused from jejunal intussusception after birth. The postoperative course was uneventfull. Intussusception is rare in neonates. It is also well-recognized that some types of intestinal atresia take place following intrauterine intussusception. We investigated the relationship between neonatal intussusception and intestinal atresia, and reviewed 51 cases of neonatal intussusception in recent literatures. This case demonstrated clearly the fact that intussusception could be one of the cause of intestinal atresia, for only 10 days even if in neonate.

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A case of Mullerian duct cyst resected through an abdomino-sacral approach was reported. An 8-year-old boy was admitted because of recurrent urinary tract infection. At one-year-old, he had undergone removal of Wilms tumor (stage II) at our institution. He was also operated upon for his hypospadia four times at the urology between 4- to 6-year-old. At the fourth operation, the left intra-abdominal testis was removed. It pathologically showed extremely premature testicular tissue. After these surgeries, he had been frequently suffered from episodes of urinary tract infection and consulted to our clinic again. Voiding cyst-uretero-gram, CT scan and MRI examination revealed a cystic mass (6 × 2 × 3 cm) between the bladder and rectum. Right undescended testis was pexied and the cyst was successfully removed through an abdomino-sacral approach. The post operative course was uneventful and urination was satisfactory. The testicular tissue biopsied contained only premature tissue. On Pathological examination, the cystic wall consisted of squamous epithelium at the surface, and had muscle layer at the most part of the cyst and endometrium-like tissue at the bottom, which were similar to vagina and uterus respectively. Since recurrence of urinary tract infection and malignant change are reported, complete resection is curative. A combination of abdominal and sacral approach was a useful access to the cyst for complete resection.

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Congenital cystic adenomatoid malformation of the lung (CCAM) is a relatively rare pulmonary anomaly. Usually lobectomy alone is satisfactory because most of the lesions involve only one lobe. Although pneumonectomy is performed when the malformation affects all lobes of one lung, it sometimes induces respiratoy insufficiency secondary to tracheal kinking from a marked mediastinal shift. In this paper, a successful surgical treatment in a neonate with CCAM of the left upper and lower lobes is reported. A 3-day-old girl was admitted to our hospital with a symptom of severe respiratory distress. A chest roentgenogram revealed the left pleural space filled with a mass containing innumerable small cysts. The mediastinum shifted to the right chest. The pre-operative diagnosis was CCAM of the left lower lobe. Left thoracotomy was performed at the age of 4 days. A microcystic CCAM involved both the lower lobe and the segments (S_1 + 2a, S_4, S_5) of the upper lobe. Lower lobectomy and segmentectomy of the upper lobe resulted in immediate improvement of respiratory condition. Her respiratory condition grew serious on the 5th postoperative day because of the marked mediastinal shift to the left. An injection of 50ml air into the left pleural cavity promptly improved her condition. She was discharged on the 18th postoperative day. Gross and microscopic findings confirmed the diagnosis of CCAM. A follow-up roentgenogram of the chest demonstrated a midline mediastinum and a well-expanded residual lung tissue of the left upper lobe.

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We experienced a rare case of increasing jaundice appeared at an interval of long term after a surgery for Biliary Atresia (BA). The patient was a female who developed jaundice after an interval of 18 years in good condition. She underwent cyst-jejunostomy under the diagnosis of type I cyst of BA on the 95th day after birth. Jaundice disappeared after operation and she had good post operative course. However, she suffered from intermittent abdominal pain, fever, and chillness since June 1988. From July she developed jaundice which increased progressively. Total bilirubin reached 20.3mg/dl in August. Cystic lesion was detected by computed tomography. Percutaneous transhepatic cholangio-drainage (PTCD) of the cyst was performed and jaundice gradually disappeared. Cholangiography showed cystic formation of the left dilated intrahepatic duct and defects in the abnormal intrahepaticduct. Choledochal-cystectomy lateral segmentectomy of the liver and hepatico-jejunostomy were performed. Although histological examination of the liver showed cirrhosis and severe cholangitis,laboratory data of the liver function was relatively stable. She is now in good health with neither jaundice nor portal hypertension.

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An 11-year-old young woman with solid and cystic tumor of the pancreas was reported. She admitted with pain and mass in the upper abdomen after being kicked violently. Hematobiochemical studies showed normal levels of pancreatic enzymes and elevation of serum neuron-specific enolase (NSE) to 61.2 ng/ml. Abdominal CT scan showed well-defined tumor with a few low density area in the pancreas. Abdominal ultrasonogram showed a tumor with mixture of hypoechoic and echogenic area at the pancreatic body. Hypovascular tumor was seen by angiography. Bone scintigram with 99mTc showed abnormal accumulation in the area of the tumor. After admission the tumor gradually decreased in size. Three months later, 70% distal pancreatectomy was performed under the diagnosis of solid and cystic tumor of the pancreas. Gross pathologic examination revealed cystic degeneration and hemorrhagic necrosis apparently encapsulated by fibrous tissues. Microscopic examination showed solid, papillary and cystic patterns including sheets and pseudorossette formation. Solid area of the tumor contained PAS-positive droplets in the cytoplasm and intercellular portions. Immunohistochemical staining for NSE and neurofilament was positive in most of the tumor cells. Alpha-1-antitrypsin was partially positive. Electron microscopic examination showed many menbrane-bound granules with high electron density from 300 to SOOnm in diameter in the tumor cells. Eleva- tion of serum NSE and abnormal accumulation on the 99m-Tc bone scintigram should be recognized as one of the features of solid and cystic tumor of the pancreas.

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Two cases of malignant mesenchymoma in children are reported in this paper. Case 1. A 10 month-old boy was admitted to our hospital with a painless tumor in the perineal region. The tumor was located in the corpus spongiosum and could not be excised completely. Microscopically, the tumor which contained mainly rhabdomyosarcoma, chondrosarcoma and hemangiosarcoma, was diagnosed as a malignant mesenchymoma. Beginning the 4th day after operation, VAC chemotherapy with irradiation was performed for 2 years. There was no evidence of recurrence 5 years after operation. Case 2. A 4 year-old girl complained of a painful tumor in the lower abdomen. Computed tomography revealed a massive, irregular low-density area in the pelvie cavity. On the day after admission, an emergency operation was performed because of signs of peritonitis. There was bloody mucinous fluid in the peritoneal cavity and a large hard tumor in the presacral region. We could not excise the tumor. The microscopic findings were those of a rhabdomyosarcoma. On the 3rd day after operation, chemothrapy according to the IRS-2 protocol (pulse VAC) was started. The Tumor was remarkably smaller and after 4 weeks, we could no longer palpate the tumor. After 15 weeks, a second-look operation was performed. The tumor was located in the retroperitoneal space. Microscopically, the tumor consisted of three different components-rhabdomyosarcoma, chondrosarcoma and undifferentiated sarcoma. Malignant mesenchymoma is a very rare malignant tumor in children and its management is very difficult.Good results were obtained in our two cases using VAC chemothrapy.

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A 4-Year-old female was admitted to Osaka University hospital with an episode of epigastralgia. On admission, laboratory data showed high levels of serum amylase, GOT and GPT. Several diagnostic procedures, especially abdominal echography and CT-scan, revealed dilated common bile duct. Under the diagnosis of congenital dilatation of the bile duct, laparotomy was carried out. Intraoperative cholangiogram showed cylindrically dilated bile duct and its anomalous communication with short ventral pancreatic duct. In a way, dorsal pancreatic duct could not be identified. So pancreatography from the minor papilla was carried out and it showed main dorsal duct which did not communicate with ventral pancreatic duct. Resection of the dile duct followed by hepatico-jejunostomy was performed. This rare case presented cylindrically dilated bile duct anomalously communicated with ventral pancreatic duct and complete pancreas divism.

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Japanese biliary atresia registry was established in 1989 with the aid of the Japanese Research Society on Biliary Atresia. Ninety-eight cases from 43 institutions were registered for the first year. The male-female ratio was 0.5. All patients underwent collective surgery at the age from 6 to 211 days. Jaundice cleared in 52 patients after the primary operation. At the end of 1989, 50 patients are surviving without jaundice and 8 patients died within one year.

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