Motility disorders of the gut in children have become a matter of increasing concern for the pediatric surgeon. Infantile hyper trophic pyloric stenosis is the most common disease requiring surgery in early infancy. While this entity was first described as early as 1888 by Harold Hirschsprung, its etiology and pathogenesis are still an enigma. Fortunately, its surgical treatment is simple and safe, which cannot be said of all other motility disorders of the infantile gut. Small-bowel dysmotility in atresia of the small bowel and in gastroschisis is not related to innervation disorders, but entirely secondary to damaged intestinal muscles caused by concomitant ischemia of the bowel wall. In contrast, the temporarily adynamic bowel of the prematurely born child, as well as Hirschsprung's disease and related disorders, are the result of anomalies of the intestinal innervation. Although aganglionosis has long been recognized as a congenital anomaly of the bowel, neuronal dysplasia(abnormal tissue development) of the gut is still a mystery to surgeons and physicians alike. With his pressure studies of the colon, Swenson first recognized Hirschsprung's disease for what it was. This led to the resection of the manometrically diagnosed abnormal colon, which was found to be aganglionic. Histological investigation of the bowel wall became the conclusive tool, replacing manometry, in the diagnosis of Hirschsprung's disease. Its reliability has become a point of discussion again, as the finding of either ganglionosis or aganglionosis is not the definite discriminating factor between normally and abnormally innervated bowel. Nor does it provide sufficient informing to explain the cause of all innervation disorders of the gut in infancy and childhood. Many hypotheses have been proposed. The enteric nervous system is still an enigma, although its origin is known, at least in birds. Why neural crest cells travel, along what paths, how they reach their destination, and what may go wrong during this migratory prcess are questions that must be answered before a full understanding and, consequently, proper treatment of neuronal dysplasia of the gut will be achieved.

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Twenty-eight patients with externally draining conduit following Kasai procedure for biliary atresia were subjects for quantitation of bile flow and for assessment of the physiologic respons to choleretics. Bile acids were determined by using an enzymatic method and high-perfomance liquied chromatography(HPLC). Schedule of choleretics is given as follows. Administration of dehydrocholic acid is continued until serum bilirubin falls less than 3mg/dl. Prednisolone is given on 7th postoperative day. The dose of prednisolone is reduced every two days and ceased 8 days later. When bile flow is not significant and serum bilirubin level does not fall by 2 weeks after operation, we given glucagon, prostaglandin E_2 and prostaglandin F_2α as alternative choleretics. 1) We devided our cases into 4 types based on their postoperative course. 2) Serum bile acids level before operation was higher in jaundice-cleared group than in group with persistent jaundice. 3) Bile flow and bilirubin excretion correlated significantly with bile salts excretion. This suggested a presence of bile salts dependent and independent bile secretion mechanism. Bile salts excretion was more than 0.9m mol/day and bilirubin excretion was more than 10mg/day in jaundice-cleared group. 4) The composition of bile acids in each group was mainly glycocholic acid and ratio of free bile acids was higher in group with persistent jaundice than in jaundice-cleared group. 5) We examined thoroughly efficacy of each choleretics after jaundice was dissapeared. Volume and concentration of bile salts in bile increases markedly after dehydrocholic acid infusion, but bilirubin excretion decreased. Bile flow increased slightly after glucagon infusion. Bile flow, bilirubin and bile salts excretion increase also after prednisolone infusion. These results suggest that measurement of bile acids in serum and bile is useful to presume prognosis. Dehydrocholic acid and prednisolone infusion is effective in early stage after operation, and administration of optimum choleretics based on pathophysiology of liver is important.

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This paper presents results of retrospectsve study on the fate of 89 survivors of cardiovascular surgery in neonate performed over the last 8 years before July 1985. In the group of 34 survivors of definitive operations(5 open-heart and 29 non-open-heart cases), 5 infants died in the first postoperative year and none after the second postoperative year. Factors relating to late death were pulmonary venous obstruction in TAPVR, combined congenital airway anomaly in PDA and bronchopulmonary dysplasia in PDA in very low birth weight infant. Actuarial survival rate at one-year was 83.3% and was 85.4% after 2-year in this group. In the group of 55 survivors of palliative operations(17 cases with CoA・IAA complex, 11 with TGA, 9 with pure pulmonary atresia(PPA), 5 with TA, 8 with PA and VSD and 5 others), late death occured in 11 cases which were mainly related to the presence of congenital anomaly of other organs(especially of airway), asplenia syndrome and to later definitive open-heart operation. Actuarial survival rate was 85.5% is one-year, 83.3% in 2-years, 81.3% in 3-years, and 77.8% after 4-years in this group. During infancy, body weight of majority of long-term survivors was below 3-percentile in the standard body weigt curve for Japanese normal children, but they showed rapid catch up growth after 1 year and by 3 years average body weight of patients exceeded 25-percentile level except for groups of patients with PDA in very low birth weight infant, PPA and shunts for cyanotic heart disease. In those 3 groups, average body weight exceeded 10-percentile level by 3 years of age.

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Four patients with IAA were treated surgically in our institution. Three patients(case 1, 3 and 4) were diagnosed as type A IAA and the remaining(case 2) was type B IAA. Only case 3 was IAA without pulmonary-ductus-descending aorta(PDDT). All cases were associated with ventricular septal defect(VSD), and Taussig-Bing complex was complicated in case 1. Two patients(case 1 and 2) died during palliative operation because of cardiac failure. Case 3 with type A IAA without PDDT, in which pulmonany artery(PA) banding and anastomosis between left subclavian artery and descending aorta had been performed palliatively, was successfully followed 6 months later by VSD closure and PA debanding. Early two stage operation at the interval of 10 days was performed in case 4 with a good result; ligation of patent ductus arteriosus(PDA) and reconstruction of aortic arch with polytetrafluoro-ethylene graft at first and then VSD closure. In regards to surgical procedures to be chosen for the treatment of IAA, there are some conflicting views. Even after successful radical operation, long term follow up is necessary for early detection of pulmonary hypertension or stenosis of reconstructed part of aortic arch.

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BCG is extensively used in the world for the prevention of tuberculosis. Although Japanese strain of BCG(Tokyo 172) has been considered to be less virulent than those used in other countries, cases with BCG complications have been increasingly reported in Japan. 19 patients with lymphadenitis or granuloma after BCG vaccination were treated in our hospital. The ages vaccinated were varied from 2 weeks to 16 months. Lesions were noticed 1 week to 16 months after vaccination, most of which were noticed less than 2 months. Axillar lymph nodes were involved in 14 patients, upper arm in 4, and supraclavicular region in 4(including 3 with axillar lesion). In 11 patients lesions were resected and diagnosed histopathologically as tuberculous lymphadenitis or tuberculous granuloma. Acid-fast bacilli were isolated from only 2 lesions out of 12 examined. In early cases, surgical removal of these lesions were indicated to assess the pathological diagnosis, followed by antituberculosis chemotherapy. After those experiences, some lesions were placed upon a strict observation and spontaneous decrease in size was observed without any chemotherapy. Only lesions which become fluctuant or suppurated in the course of observation were resected and followed by antituberculosis chemotherapy. According to these clinical and pathological resection with antituberculosis chemotherapy should be performed in a limited manner only when(1) any signs of generalized BCG infection are noticed, or(2) lesions become fluctuant or suppurate.

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Our experiences in endoscopy and transurethral surgery in 394 children(250 boys and 144 girls) during the last 17 years were reported. Age ranged from one month to 16 years. More than 62 per cent of children was under 5 years and 9.1 per cent was under one year. Most of diseases for endoscopic evaluation and surgery were congenital. The most predominant one was primary VUR, followed by mechanical lower urinary tract obstruction, ureterocele, ectopic ureter, secondary VUR, hydronephrosis, imperforate anus, external genital anomalies, non-refluxing megaureter, bladder diverticulum, etc. Items for evaluation of theses were described. As for transurethral surgery by miniature resectoscope, experience and prudence was necessary. The results of transurethral resection of posterior urethral valve, anterior urethral diverticulum(or valve) and bulbous urethral ring stricture was quite satisfactory. Indications for transurethral distal incision of ureterocele was also described. Non-everting simple ureterocele was effectively treated by this method without inviting VUR. Even in ectopic one, this was not only a useful diagnostic modality but also a supplementary treatment.

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Results of cardiovascular operetion in 22 infants born in low birth weight and weighing less than 2,500 gr at the time of operation were reviewed. Seventeen infants had patent ductus arteriosus(PDA-group) and 5 other had various congenital heart disease(CHD-group). Birth weight and gestational age in PDA-group were less developed than those in CHD-group. There was no operative death in PDA-group but 2 died in CHD-group. Combination of congenital anomalies of digestive organs in PDA-group was less frequent than in CHD-group. In the surgical treatment of cardiovascular anomalies in infants weighing less than 2,500 gr, meticulous pre- and post-operative respiratory and circulatory care is of prime importance beside with early accurate diagnosis, preferably by non-invasive technique and selection of proper operative method acceptable to small, poorly nourished infants.

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We recently observed the neurological syndrome(including areflexia and ataxia) associated with vitamin E deficiency in 2-year-old boy with corrected biliary atresia resulting in jaundice and severe cholestasis. High-dose oral supplementation of vitamin E was not effective in normalizing serum vitamin E, but neurological improvement occurred after restoration of normal vitamin E levels by parenteral therapy. In order to assess intestinal absorption of vitamin E in children with corrected biliary atresia, the oral vitamin E tolerance test(OVETT) was performed on 3 patients with jaundice (group A: 3-4 y) and 5 patients without jaundice(group B: 3-7y). In group A, vitamin E deficiency was demonstrated by low serum vitamin E levels and low serum vitamin E/lipids ratios(E/lipids). During OVETT, no rise occurred in serum E/lipids in any of group A patients. In contrast, normal vitamin E nutritional status in group B was identified by normal serum vitamin E and E/lipids, and during OVETT, serum E/lipids rose significantly from baseline in group B patients. In group A patients, parenteral vitamin E therapy was effective in normalizing serum vitamin E levels and serum E/lipids. We therefore recommend parenteral vitamin E therapy for children with jaundice who cannot achieve normal serum vitamin E levels with oral therapy.

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A seven-month-old, well nourished female, born to a nondiabetic mother, was admitted to a regional hospital because of convulsion. On admission blood sugar was 27mg/dl and immunoreactive insulin(IRI) value 28μU/ml. Other hormonal values were within normal limits. The administration of Diazoxide(30mg/day) had been effective to improve and control hypoglycemia. However, after the age of one year and 10 months, hypoglycemic episodes have occurred frequently in spite of continuous administration of Diazoxide. At the age of two years and 3 months, a subtotal pancreatectomy(90%) was performed. Intravenous administration of insulin was required for several days after operation, and thereafter, the blood sugar level became stable in normal range without administration of Diazoxide. The resected pancreas was grossly normal but histologically nesidioblastosis. By morphometric examination, the percentage of islet tissue was estimated 2.0% which is in the normal range. Immunohistochemical study showed the relative percentages of the four endocrine cell types as follows, insulin; 46.5%, glucagon; 41.4%, somatostatin; 11.3%, pancreatic polypeptide; 0.8%. In our case, the relative proportions of insulin and somatostatin were lower and that of glucagon was higher than normal. Pathognomonic basis of hyperinsulinemic hypoglycemia was discussed in this paper.

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Pancreatitic symptoms such as nausea, vomiting, fever, abdominal pain or hyper-amylasemia are somtimes associated with congenital biliary ductal dilatation(CBD) in infancy. The symptoms are thought to be attributed to acute pancreatitis due to reflux of bile into the pancreatic duct caused by the coexisting anomalous choledocho-pancreatic junction. We recently encountered 5-year-old girl with CBD who underwent an external drainage of the common bile duct because of severe pancreatitic symptoms with progressive dilatation of choledochal and pancreatic ducts. The patient underwent cyst excision, cholecystectomy and Roux-en-Y hepaticojejunostomy 1 month after the external drainage. We could detect a considerable amount of phospholipase A_2 and lysolecithin in contents of the common bile duct. These harmful substances seem to act not only on the pancreas but on the biliary ducts, both of which possibly cause pancreatic symptoms in the course of CBD.

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A male baby with esophageal atresia(Type C), imperforate anus(recto-urethral fistula), vertebral anomaly and polydactylia was reported. The patient underwent gastrostomy and colostomy on the first day of life, followed by division of tracheo-esophageal fistula and end-to-end esophageal anastomosis on the second day. At the age of 10 months, the baby underwent resection of the supernumerary thumb and sacro-perineal anoplasty, and is doing well. This case represents the 13th case of the complete form of VATER association, reported in the Japanese literature. The authors also collected 57 cases of VATAR association from the Japanese literature and the hospital record of their own, and attempted to classify these patients into the complete and incomplete forms. Thirteen patients(22.8%) had four factors(V, A, TE & R)(complete form), while 15 cases(26.3%) had three factors and 29 cases(50.9%) had two factors.

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An unusual case of vitelline duct remnant(VDR) in a 16-month-old boy was reported. He was admitted because, of a cystic mass underneath a normal umbilicus. The mass was found at four months before his admission and gradually enlarged. The cyst originated within subcutaneous tissue between the diastatic rectus and extended beneath the left rectus muscle. Shape of the cyst was irregular with small out-pouchings. A small polypoid lesion was found on the posterior wall and removed with fibrous cyst wall. The cyst wall had neither mucosal lining nor structures attributable to VDR except at the polypoid lesion. Laparotomy failed to disclose any VDR in the peritoneal cavity. The polypoid lesion consisted of small bowel mucosa, smooth muscle layers and heterotopic pancreas(Heinlich 1 type) but no gastric mucosa. Removal of the polypoid lesion is curable in such a case.

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A 5-year-old boy was admitted with complaint of nonproductive cough and right chest-back pain. A mass with carcification was found in the right, upper posterior mediastinum. Computed tomography and myelography showed that the mass expanded into the epidural space(dumb-bell type), but he had no symptom of spinal-cord compression. Urinary VMA was negative. The tumor was totaly recected by right thoracotomy and right hemi-laminectomy(Th5,Th6). Histological diagnosis was neuroblastoma with rosette formation. He received post-operative combination chemotherapy and irradiation. For 5 months after the operation, he is tumor-free, and has no neurologic abnormality. Most dumb-bell type neuroblastomas are associated with neurologic deficit due to the spinal-cord compression. Following the operation, most of these patients have insufficient recovery of neurologic function. Earlier diagnosis and treatment prior to the onset of neuro-logic abnormalities are the most important factors to improve the functional prognosis of dumb-bell type neuroblastomas.

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This paper describes clinical characteristics of malignant rhabdoid tumor of the kidney(MATK) from our experiences and the literatures. In two infants experienced in our institute, the MRTK were found in association with brain tumors, one of which was cerebellar medulloblastoma and the other was metastatic MRTK. Both patients died of rapid growth of the brain tumors. For the last 15 years, only ten of infantile malignant renal tumors(MRTK or neonatal Wilms') had been reported in Japan. Among them, all of MRTK(five cases) had expired within one or eight months postoperatively. Associated brain tumors were found in three of five MRTK in this series. Since the rapid appearance of local recurrences or multiple metastases of the tumor is seen, additional extensive studis in attempt to develop the effective treatment for these highly malignant renal tumors are necessary.

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The authors report two patients, 6 & 7-year-old boys, with bone metastasizing renal tumor. Histological examination of the tumors demonstrated the typical features of clear cell sarcoma of kidney(CCSK) in case 1, or hyalinizing pattern of CCSK in case 2. Multiple agent chemotherapy including adreamycin seemed to be effective against our patients to a certain extent of their clinical courses, even though they died at approximately 2 years after surgery. For the last 15 years, seven cases of bone metastasizing Wilms' tumor and twenty one of sarcomatous renal tumor(SW), in which the rhabdoid tumors were excluded, had been reported in the Japanese literatures. Only three of them had survived more than five years. Marked male predominancy(M: F=20: 8) of the patients and frequent bone metastases of SW or CCSK were also seen in this series. The appropriate treatments currently being used for patients with those SW were described briefly.

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36 cases of congenital intrinsic duodenal obstruction have been treated at the Niigata University Hospital during the past 15 years. 5 cases(14%) of them were associated with gastric rupture(2 cases), gastric perforation(one case) or impending rupture(2 cases). 3 cases of them were cured. Sites of rupture and perforation including impending rupture were along the anterior midportion of the greater curvature in all of these 5 patients. Operative diagnoses of duodenal atresia associated with gastric rupture and perforation including impending rupture were made except for one case, in whom the diagnosis were made at autopsy. Preoperative diagnoses were gastric rupture in 3 cases and duodenal atresia in one and jejunal atresia in one. In this paper, we would like to discuss the diagnosis and management of the duodenal atresia associated with the gastric rupture and perforation.

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Intra-arterial administration of adriamycin in combination with cip-platinum and 5-fluoro-uracil dispersed in a lipid contrast medium(Lipiodol), "FAC-LIP.", was used in an infant with initially unresectable hepatoblastoma. "FAC-LIP" selectively remains in the tumor tissue more than 1 month, and a significant reduction in tumor size was noted. Subsequent resection is not performed because of the evidence of pulmonary metatasis. "FAC-LIP" was also useful to avoid the the toxicities of the anti-tumor drugs to the host.

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Two cases of the breast cyst in children were reported. Case 1: three-year-old female having a breast mass during two years was transferred to the authors' hospital. Removal of the cystic mass was performed. Case 2: three-year-old male was admitted to the authors' hospital because of a rapid growth of a breast mass being noted from his birth. Resection of the cystic mass was done. Pathologically, both of the masses were diagnosed as retention cysts of the breast ductal system.

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Blood containing cardioplegic solution with low ionized calcium level was prepared independently of pump-oxygenator perfusate blood, so as to contain invariable concentration of each composite at any time. Using this solution, open heart surgery combined with prolonged aortic cross-clamping time ranging from 117 to 184 minintes(mean: 149) was performed for 7 young infants with complete transposition of the great arteries(TGA) with the median age of two months(34 days-3 months). Four patients in age of 39 days, 41 days, 2 months, 3 months survived and showed uneventful postoperative hemodynamics. Two dead patients had no indications for Jatene's anatomic repair due to their low left ventricular pressures. Another dead infant with large ventricular septal defect of muscular type showed postoperative residual shunt and its cause of death could not necessarilly imputed to the myocardial protection. Judging from these experiences of four survivors undergoing prolonged aortic clamp times ranging from 117 to 160 min in early infancy, our cardioplegic solution and its administrationtechnique could be considerably promising and expectant for young infants heart surgery.

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A 4-year-old boy with severe abdominal pain and vomiting was transfered to our hospital on June 20th, 1985. The abdominal plain X-ray film showed bi-lateral subphrenic free air. Emergency operation was performed and the rupture was found in the first portion of duodenum. Debridement and closure of the rupture lesion, cholecystostomy and peritoneal drainage were done. IVH was administered for 2 weeks postoperatively. The postoperative course was uneventful and he was discharged on the 20th-postoperative day. Traumatic duodenal rupture was very rare in infants and children. Only twenty cases of traumatic duodenal rupture in childhood including our case were collected from Japanese literatures from 1966 to 1985 and reviewed. The rupture of duodenum occurred at the second and third portion of duodenum in 75 percent and was mostly prevalent in a 7 or an 8-year-old boy. The rate of complication was 50 percent in the cases which were treated over 24 hrs. after the injury. Overall mortality rate was 10 percent.

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