The result of over ten years follow up studies in 302 cases of esophageal atresia (including 30 cases in National Childen's Hospital) which were obtained by questionair from 52 institutions in our country were shown in this paper. In this series, two longest survivors reached the age of 27 years and 44 cases reached the age of more than 20 years. For long term period, hights and body weights in many postoperative patients of this disease were found under mean values of development of Japanese children. Especialy, in cases with A type of Atresia or esophageal reconstruction using other digestive tract, postoperative developments were markedly poorer than those in cases with C-type of atresia and primary surgery. Postperative thoracic deformity in this disease is most frequent problem. This diformity was found in 30% of postoperative patients. Passage disturbance of esophagus and recurrent respiratory sign were found in about 15% of all patients. These problems were not shown tendency of improvement through patients life. Social life in postoperative patients is contented result except for few cases. In cases with long gap atresia which could not undergo esophago-esophago stomy, jejunal interposition is satisfactory procedure in spite of complications of dysfunction of lower part of esophagus and stricture in site of lower esophageal anastomosis from our long term results.

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Twenty-nine of 44 esophageal atresia patients were survived in the period between 1969 and 1978. An attempt to evaluate the long term results was made. The follow-up period ranged from 10 to 19 years after operations. 15 gave sufficient informations for analysis. Approximately 73% had temporary swallowing difficulty. The complaint seems to have been closely related to postoperative anastomotic complications. 2 esophageal atresia patients treated by colonic interposition have not had any trouble in swallowing. Slow gain in weight and height was commonly observed. Deformed chest and breast did not seem to be serious problems in our series.

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Long-term survival after massive intestinal resection is now possible with parenteral and enteral nutrition. However, infants and children with short bowel syndrome incidentally suffer a number of difficulties and / or complications during their long lives after catch-up growth. This paper discusses the adjunct surgical procedures for the short bowel syndrome, and presents the considerations on how to cope with the difficulties and / or complications through the experiences of seventeen long-term survivals at Juntendo University Hospital for the last two decades, whose present ages range from 2 to 21 years old. Adjunct procedures for the short bowel syndrome are mainly divided into three groups; the first is to prevent intestinal resection and conserve the intestine by the second-look operation when massive resection is necessary, the second is to slow intestinal transit by antiperistaltic segments, colon interposition, intestinal valves and sphincters, recirculating loops and intestinal pacing, and the third is to increase the area of absorption by intestinal tapering or lengthening, growing neomucosa and intestinal transplantation. Among these surgical procedures, the second-look operation actually prevents intestinal resection and conserves the intestine, antiperistaltic segments, colon interposition, and intestinal valves may benefit patients with sufficient absorption area but rapid intestinal transit and intestinal tapering or lengthening may benefit selected patients with dilated bowel segments. However, growing neomucosa is not yet clinically efficacious, and the intestinal transplantation still remains unsatisfactory in spite of recent advances in immunosuppressants. The difficulties and complications observed in the 17 patients during their lives were as follows; growth retardation "stunting" caused by chronic protein energy malnutrition, adhesive ileus or stagnant loop syndrome due to the initial surgical procedure including the second-look operation, cholelithiasis, bone disorders such as rickets, fatsoluble vitamins and vitamin B_<12> deficiencies, urinary oxalocaliculi and rapidly falling serious dehydration associated with incidental diarrhea. However, most of these difficulties and complications would be prevented if the managements would be carefully continued for long-term even after catch-up growth. The most important is continued nutritional cares in the patients with massive intestinal resection.

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The growth and endocrinologicai and metabolic status were evaluated in 6 longtime survivors of short bowel syndrome. Body weight and height were below 50th percentile. Mental retardation was not observed in all cases. Hyperoxaluria was seen in all cases and renal stones was in one case. On the results of glucose toleranse test, peak IRI and insulinogenic indeces were low. Secondary sexual manifestation was not seen in one case of 15 years old.

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This paper describes results of long-term follow-up in patients with Hirschsprung's disease treated at our department and a nationwide survey on those patients over 20 years following the definitive operations. Results are summarized as follows : 1)0ne hundred and thirty-two of 155 patients with Hirschsprung's disease are still alive and well. 2)The most widely used operation was Z-shaped anastomosis seen in 116 patients. Of the 116 patients, one-hundred and ten are alive. Sixty-nine are over 10 postoperative years including 25 over 20 years. 3)After 5 years following Z-shaped anastomosis, about 80% of the patients had nearly normal ano-rectal function. 4)Physical growth, character, sports activities, and academic career in patients are almost as same as people in general. 5)Therefore, Z-shaped anastomosis is one of the most reliable and safest method as the definitive operation in Hirschsprung's disease. 6)A nationwide survey on patients over 20 postoperative years was conducted, and 222 cases were collected from major 17 institutions. 7)Swenson's method was more widely used compared with the present trend. 8)About 80% of the patients had almost normal ano-rectal function. 9)Married patients were seen in 33, and 22 families had a total of 33 children. All of them were not Hirschsprung's disease. 10)Most of the cases have taken important occupation. It is very grateful for pediatric surgeons that there are one medical doctor, three medical students, and one nurse.

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Fifty-four Children with anorectal anomaly of high and intermediate type were followed up for 10 years or more postoperatively. There were 36 boys and 18 girls. The types of radical operation were abdomino-sacro-perineal pull through in 15, sacroperineal in 34, and perineal in 4. The results were evaluated clinically in all, and barium enema and anorectal manometric study were performed in 34 children. A good result was obtained in 28 children (52%) clinically, which was not always parallel to the evaluation by the examinations. Beside the evacuation habit, combined anomalies, complications, sexual function, school life and social adaptation of the children were also studied. We understood that 52 of them (96%) were almost satisfied with their daily life. From the study, we have concluded that the sacral approach is the best to create the route for rectal pull through in high and intermediate anorectal malformations.

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This report presents the long-term results in patients who have survived for more than 10 years after surgery for biliary atresia. Fourty-eight cases ranging in age from 10 to 33 years were examined. There were 41 cases without and 7 with jaundice. Two patients died after the age of 10 years : one died at 28 from hepatic failure caused by frequent cholangitis after operation for ileus; the other died from sudden esophageal variceal bleeding at 16. Thirty-seven (77%) of 48 cases were absolutely leading their normal lives. Among them, 6 cases were enjoying their lives after overcoming the sequelae, such as portal hypertension. Two females both aged 25 years have married, but did not become pregnant yet. The morbidities of the remaining 11 (23%) long-term survivors were jaundice in 7, portal hypertension in 5 , encephalopathy after splenorenal shunt in 1 and so on. The heights and weights of all cases except a few were comparable to those of normal Japanese. Hepatomegaly still remained in 16% and splenomegaly in 29%. The biochemical data of the serum were variable and disclosed a moderate degree of abnormality in patients complicated with postoperative cholangitis. Eleven cases including 6 jaundiced cases required treatment for esophageal varices and hypersplenism. In conclusion, although the long-term survivors with persistent jaundice develop sequelae that require some kinds of treatment, the cured states of most cases without jaundice are satisfactory and eventually these former patients can achieve a favorable quality of life.

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Twenty nine patients of biliary atresia, who underwent hepatic portojejunostomy and have reached 10 years old and more, were analysed in aspects of physical growth, social activities, state of puberty, and physiological conditions. Seventeen (59%) of these 29 patients developed esophageal varices and 14 (48%) developed hypersplenism (thrombocyte count in peripheral blood less than 10 × 10^4/mm^3). Both the body height and body weight were within normal range in approximately 55% of the patients. Physical growth of the patients with portal hypertension was more retarded than that of the patients without portal hypertention. All the patients except three are attending school. Two 20-year-old patients are working as an office clerk and a salesgirl, respectively. Twenty one (72%) of 29 parients are spending their dayly life freely, whereas patients with enlarged spleen limmit exercise voluntarily. Serum total bilirubin level was less than 2 mg/dl in 23 patiens. Portal hypertension developed in 8 (35%) of these 23 patients. On the other hand, portal hypertension developed in all of 6 patients with serum bilirubin more than 2.1mg/dl. Esophageal varices and hypersplenism were well controlled by endoscopic sclerotherapy and splenectomy. The average age of menarche in 13 female patients, who have reached more than 12 yeas of age, was delayed approximately two years compaired with Japanese controls. Eight of these 13 patients complained of menstrual disorders, i. e. primary amenorrhea, secondary amenorrhea, and unestablished menstrual cycles. The serum estradiol levels in most of 13 patients were unexpectedly normal. Patients with abnormal menstruation, however, showed an over-reaction of LH in LH-RH test.

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One hundred and eighty-three patients with neuroblastoma were treated at National Children's Hospital, Tokyo, during the past 23 years from 1966 through October, 1988. Among them, six patients were found at mass-screening. At the initial stage, chemotherapy consisting of cydophosphamide and vincristine was given to the patients, but more intensive chemotherapy such as Sawaguchi's A_1 protocol is being used at present. Tumors are removed more completely at a secound look laparotomy, and intra-operalive irradiation has been employed for recent patients. To those patients who are supposed to have a poor prognosis but who showed a complete response (CR), high dose Melphalan treatment followed by autologous bone marrow transplantation is given. So far, the clinical results were excellent. Over all two-year survival rate was 41.8% in 1960s, 42.9% in 1970s and 54.8% in 1980s. Long-term follow-up results were obtained from 29 patients who survived for more than four years ; average age at follow-up being 14 years 8 months. It was found that functional disturbances were minimal in these patients.

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Ninety-nine patients with neuroblastoma have been treated in our department. Overall 5-year survival rates were 100% in stage I, 60% in stage III, 12.5% in stage IV and 70% in stage IVS. The changes in therapeutic methods and the results were evaluated and the subsequent problems were debated. Pre and post-operatfve adjuvant chemotherapy of advanced neuroblatoma has been changed from mild to agressive one (high dose cyclophosphamide treatment) and delayed primary operation was induced in 1978. Synchronization chemotherapy and second look operation were instituted in 1980. Sublethal dose chemotherapy with autologous bone marrow transplantation was performed in 1985. The survival rate of neuroblastoma was remarkably improved after 1978. However the unusual case of late recurrence 10 years after complete remission was also experienced. Therapeutic result of neurolatoma is expected to be improved gradually because of the development of treatment and the introduction of mass screening. To achieve the goal,further continued efforts have to be made in every respect.

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Sixteen long term survivors of Wilms' tumor which were experinced in our department were analyzed. No patients showed abnormalities in non-specific cell mediated immunity, endocrine function, secondary sexual development or renal function. In patients who were followed more than 10 yers after surgery, the function of the remanning kidney, as determined by DMSA uptake, was well developed. In 15 patients who were treated by radiotherapy, changes in the spinal bodies were seen in all 15, scoliosis (average 9°) in 14 and hypoplasia in the iliac bone on the irradiated side in 12. In 14 patients who were examined by CT scan, atrophies of the erector spinae and illopsoas muscles on the irradiated side were seen in all cases. A significant correlation between the severity of the atrophic erector spinae muscle and the degree of scoliosis was obtained. Sixty nine patients with Wilms' tumor who survived more than ten years after surgery were found in the files of 13 institutions in our conutry. Out of them, 25 (36%) developed late disrurbances and many of them were due to irradiation therapy.

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The development of scoliosis in the long term survivor who has received radiotherapy for Wilm's tumor is investigated using the various methods. of 28 children treated for Wilms's tumor at the university of Chiba hospital, 9 survived 10 years. The standard treatment consisted of nephrectomy, postoperative irradiation and Actinmycin-D protocol. As would be expected when large doses of ionizing irradiation have been deliverred to young children, postual changes were obseoved in years. These late effects in 5 children were investigated by Moire topography, Magnetic resonance image, and Spinal evoked response with the ordinary roentgenogram of spine. Roentgenogram showed the mild scoliosis with bony changes. Moire topography revealed 10-45mm hump and 40-90 waist line assymmetric index. MRI examination demonstrated clearly the atrophic changes of soft tissues, especilly subcutaneous fat and muscles. Abnormal neurolgical changes were not found in the Spinal evrked response. As a result, the bony changes could not be the only region to contributs the formation of scoliosis. Further investigation would be necessary to proove the rationale of the postual deformity using these modalities in future.

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Since 1967, 27 hepatoblastomas and 3 hepatocellular carcinomas in 30 childhood patients have been treated in our clinic with a 10-year suvival rate of 43.0%. Twenty three of these 30 patients underwent hepatectomy. A 10-year survival rate was 51.7% in total of these tumor resected cases, but was 65.2% in curatively resected cases and was 0% in non-resected cases. Five cases with an initially unresectable tumor, or with recurrent or metastatic tumors received "second look" or "delayed primary" operation after chemotherapy. Three of these 5 patients, whose tumor was histologically mixed mesenchymal type hepatoblastoma, were cured of disease completely. A questionnaire on childhood primary liver carcinoma was sent to all the councilors of the Japanese Society of Pediatric Surgeons, and a total of 535 primary liver malignancies, such as 417 hepatoblastomas and 79 hepatocellular carcinomas, were collected from 97 institutes. A 10-year survival rate was 32.3% in total cases,50.6% in tumor resected cases, 63.2% in curatively resected cases, and was 1.4% in non-resected cases. The significant prognostic factors in this series were the histology of tumor, the age of patient, and the stage of disease. A hepatoblastoma with a mixed mesenchymal type histology may be worthy to aggressively even if the tumor is unresectable.

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During the past 26 years (from Jannuary 1964 to April 1989, we had a surgical exprerience of 40 cases of primary liver malignancy in children under 16 years old : 26 hepato blastomas, 7 hepatocellular carcinomas, 4 mixed malignant tumors and 3 unknown histological malignacies. The mode of surgery and long term survivors more than 2 years (in the parentheses) were one left lateral segmentectomy with removal of two lymphnode metastases in the hepatoduodenal ligament ( 1 ) , 9 left lobectomies ( 3 ) , 3 extended left lobectomies including removal of the caudate lobe ( 0 ) , 3 right lobectomies ( 1 ) , 3 extended right lobectomies ( 0 ) , 9 rigth trisegmentectomies ( 6 ) , 2 extended rigth trisegmentectomies ( 1 ) , one partial resection for the recurrence ( 0 ) and 9 explorations ( 0 ) . Four died during surgery but no child died by complication. Socalled second look operation was carried out in 8. There are two long survivors : one unresectable case became resectable by VCR infusion through the hepatic artery and surviving 19 years, and one right trisegmentectomized case with tumor thrombus in the right portal branch in the first surgey underwent the resection of the lateral half of the remaining left laterral segment after recurrence, and remains disease-free 2.5 years after the second intervention. Our unique regimen and philosophy of preoperative and postoperative chemotherapy is as follows since 19 years : one intravenous shot of VCR of 1.5 mg/m^2 is given several days prior to surgery to know its effect to the neplasma utilysing the serum AFP value as a monitor. If the preoperative decrease of AFP value is not enongh, postoperative choice of drug is switched to ADR 20 mg/m^2, replacing VCR of 1.2 mg/m^2. Anyway the postoperative chemotharapy with VCR or ADR is given within 20 to 30 hours after surgery. No further chemotherapy was given in all cases. This timing is important, we believe, in order to kill the tumor cells disseminated during surgery and anchoring in other organs such as lung in the early postoperative period. Maybe by virtue of this regimen which is contrary to drastic pre and postoperative chemotherapy widely used recently, we have no pulmonary metasses since 19 years. Multiple recurrence in the remnant liver, which is not yet controlable even by the heavy chemotherapy, was the main cause of deeth. In cases of huge and unresectable tumor, however, the latest heavy chemotherapy may be indicated for possible shrinkage of the tumor that leads to the radical as well as easy removal of the tumor.

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Parenteral nutrition (PN) has been a major advances in the nutritional care of infants and children, However, a number of complications associated with this form of nutrition have been identified, including technical and metabolic complications. During the past 18 years period (1970-1988), a total of 464 patients (137 neonates, 162 infants And 165 children) were placed on PN in our Department, These 464 patients were divided into two groups according to the basic stages in management of PN ; Group 1 : 1970-1980,Group 2 ; 1981-1988 and clinical result, particularly the incidence of complications, was investigared and the following results were obtained; 1, The incidence of technical complications due to placement and position of central venous catheter for PN has been reduced significantly in Group 2 by strict attention to aseptic technique and by introduction of nonreactive silicone catheters. 2, However, hepatic dysfunction remaines one of the most common and most serious complications of PN in both groups, showing no difference in incidence of PN-assosiated liver complications between two groups.

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Ninety-three premature infants (904-2,500g, birth weight) were treated surgically at the Showa University Hospital during the period of 1970-1987. The 93 patiens were divided into two groups : before or after establishment of the NICU (1981) , i. e. 40 patients were in Group A (1971-1980) and 53 in the Group B (1981-1987) . Although there was no significant diffrence of proprotion of the total numbers of premature infants < 2,500g, in each grop period, proprotion of the infants, 1,500g increased in the later period. The overall mortality rate improved significantly in the Group B period (21.2%) , comparing with 39.5% in the Group A period. It is speculated that factors related to the improvement of mortality in the series inclded : 1 ) no death in the patients with congenital intestinal obstruction (n = 18) inclding duodenal obstruction in Group B, comparing with 4 deaths in 15 of those in Group A ; and 2 ) improvement of mortality in esophageal atresia (2/9 in Group B vs. 5/8 in Group A) . Thus, sophisticated cooperation between pediatric surgeons and neonatologists is considered to be important. Despite of progress in diagnostic and therapeutic management in neonatal surgical patients in the recent years, on the other hand, therhand, there were no significant improvement of survival outcome in premature patients with gasrointestinal perforation, diaphragmatic hernia and abdominal wall defect in the series. To enhance survival outcome for these high-risk patients : 1 ) prompt and reliable management for septic patients with gastrointestinal perforation ; and 2 ) improvement of respiratory management for severe pulmonary hypofunction in diaphragmatic hernia are desirable.

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In a 10-year period from 1978 to 1987, 42 newborn infants with acute peritonitis due to gastrointestinal perforation consisting of 31 with gastric rupture and 11 with intestinal perforation were operated upon at the Niigata University Hospital. Seventeen infants in this series suffered from sepsis with or without septic shock and 13 of them died. Of 31 infants with gastric ruture, 12 were involved by sepsis or septic shock and 8 died. Low birth-weight and acidosis were more remarkable in infants who died than in those who survived. Of 10 infants who received the limilus test, 6 were positive for endoxin in the blood and 5 died. Certain kinds of bacilli were identified in ascites of 8 infants, and 7 of them died. Bacteremia was seen in 6 of 9 infants who received blood culture test, and 5 of them died. Conclusion : 1 ) It was not easy to establish a diagnosis of sepsis on the infants with acute peritonitis due to gastrointestinal perforation. 2 ) Early treatments with protease inhibitor, γ-globulin, protanol, hydrocortison, antibiotics, increased the survival rate of the infants with sepsis. 3 ) Blood exchange seemed to be a best useful treatment for sepsis with DIC.

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This paper was concerned with a survey on the present condition of diagnosis and treatment of septic shock in pediatric surgery in Japan. We wrote to sixy-nine pediatric surgeons authorized as an experienced doctor by the Japanese Society of Pediatric Surgeons and asked to fill the questionaire on their cases of septic shock in surgical infants and children during the past five years and comment on the criteria of septic shock by themselves and the theapeutic principle. Sixty surgeons responded to our survey. As a result, 343 cases were collected including 215 neonates. In this paper the resuit was presented in detaie and discussed.

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The development and progress of pediatric anesthesia depend upon the development of pediatric surgery. In this paper, we select the cases of (1) extremely premature neonates and super extremely premature neonates and (2) airway management in surgery for tracheal stenosis and then report the method of anesthetic management during surgery and its problems. (1) Since 1978, we have experienced 36 cases of extremely premature nenates and 11 cases of super extremely premature nenates. The anesthesia for these cases has changed from GOF, J-R to NLA. During the surgery, it is very important to observe the PaO_2. SaO_2, body temperature, infused volume and Ca and glucose level. (2) In airway management, first we tried to intubate from operational field and secondly tried to ventirate by HFJV, but our experience is that neither of these methods is sufficient. Therefore, we consider using ECMO method. During the surgery of tracheal stenosis, we have to observe the patient by measurement of SaO_2, PaO_2 and PaCO_2.

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Out of 182 naonatal surgical cases, which I had experienced between 1973 and 1987, 161 (excluding inguinal hernia, phimosis, accessory ear and superficial mass) were analized about the sex, diagnosis, birth weight, operative mortality and cause of death. As the birth weight decreases, the proprotion of female cases increses. Main diseases of the cases weighing more than 1,500g at birth were common congeninal anomalies such as imperforate anus, Hirschsprung's disease, intestinal atresia and Bochdalek hernia. On the other hand, congenital anomalies extremely decreased and perforation of the G-I tract increased in the cases weighing less than 1,500g at birth. Sixty percent of this goup was NEC. The mortality rate was tremendously different with birth weight (more than 1,500g ; 14% , less than 1,500g; 56%) . The main cause of death was sepsis arizing from perforation of the G-I tract (over 50% of died cases) .Improving the management for perforation of G-I tract is important to decrease the mortality rate at present. 1,500g (Birth weight) is proper dividing line for clinical analization of the Low-Birth-Weight surgical cases.

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A six-month old girl was admitted with severe respiratosy distress. Respiratory insufficiency rapidly deteriorated in spite of mechanical ventilation. Tracheoscopy and bronchogram revealed the existence of congenital extensive tracheal stenosis. Tracheoplasty using costal cartilage graft was performed. In the operation, an associated pulmonary artery sling was released before tracheoplasty. Re-stenosis of the trachea occurred near the carina one month after the tracheoplasty. Several attempts to enlarge the stenotic segment, such as removal of the granulation tissue and high pressure pneumatic balloon dilatation, were unsuccessful. The second operation was performed at 6 months after the tracheoplasty. The stenotic segment of the trachea was resected and end to end anastomosis was carried out. Intraoperative massive bleeding was observed from the left pulmonary artery, which had penetrated into the trachea. She expired due to DIC at 8 hours after the operation. Postmortem examination of the cartilage graft revealed, (1) disappearance of cartilage cells and (2) partial replacement by the fibrous tissue. These facts suggested that the graft was in the process of absorption.

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