The children with congenital biliary atresia (CBA) tend to develop pulmonary complications postoperatively, e. g. pneumonitis and reccurrent atelectasis. Their management was often very difficult. Even when diseases free, their chest X-ray films show diffuse parenchyma! changes of the lungs. Alteration of the pulmonary structure and on pulmonary surfactant were studied in 18 CBA autopsy cases. The most characterisitc morphologic changes of the lungs included thickening of the interalveolar septa, increase in the cellularity of alveolar walls and formation of the multiple A-V fistula. With electron microscopy, the most remarked findings were swelling and increased vacuolization of the endothelial celles, increase in the microthrombi in the pulmonary capillaries, prolifiration of the type II alveolar pneumocytes and decrease in the number of the type I pneumocytes. As for the surfactant, the amount of disaturated phosphatidyl choline increased in the lung in CBA in comparison with controls. These changes were confirmed in the experimental study. The Wister rats with ligated common bile duct developed progressive hepatic and pulmonary changes. The hepatic change consisted of generalized fibrosis and bile duct proliferation. The pulmonary changes were exactly same as seen in human CBA.

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Studies on the necessity of early surgical closure of a large ventricular septal defect in infancy were carriedout comparing the two groups of infants requiring operation under 12 months of age and those at 12 to 24 months of age from the viewpoints of preoperative status and postoperative courses. In the infants under 12 months of age with symptoms, postoperative complications appeared more frequently as a reflection of the presence of preoperative complications. The hospital motality was 10.5%. Therefore, careful postoperative management is required in this group. However, the risk of operation is acceptable and surgical improvement is satisfactory. On the other hand, in the infants 12 to 24 months of age, postoperative complications appeared less frequently. There is on hospital death and all survivors are asymptomatic. Whereas the complications of surgery appear no gerater in infancy, we prefer to perform early surgical closure of a large ventricular septal defect in the infants with symptom.

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Much literature has been written about intussusception in infancy and children ; however, the possibility of familial incidence has received only little attention. The National Kagawa Children'sHospital is reporting fourteen cases of familial intussusception. The familial cases of intussusception were as follows: two groups of a mother and her daughter; a mother and her son; five sibling groups; two groups of an aunt and nephew; and four groups of causins. High familial incidence of intussusception was noted (1 in 13). It is suggested that heredity may be a factor in the etiology of idiopathic intussusception in infancy and children, and should be investigated further,

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Cystourethrography has assumed rmijer importance in classifjcaiion of nno-rectal anomalies. A technique for cystourethrography utilizing a small caliber catheter that is left in the posterior urethra during the entire examination is described. In male neonate or infant, 4 or 6 Fr. catheter is inserted 7 cm or 9 cm into the urethra, respectively. The catheter is taped in position and remained in place throughout the examination. This is accomplished by placing a strip of the tape on the lower abdomen, winding the tape round the shaft of the penis and the catheter without gap. The contrast is infused under pressure with syringe. Usually voiding reflex occurs soon after infusion but the contrast infusion is continued. The contrast is delivered in a steady dropled form. Repeated spot films are taken during filling of the entire urethra, combined with cologram in post-colostomy patient. A few among several spot films can accurately define the rectal fistula in relation to the bladder or urethra. This technique can be applied to post-rectoplasty defecography. After filling of the rectum with liquid barium through a small caliber catheter, the contrast is injected into the posterior urethra via the catheter. Urination may induce defecation and defecography can be performed.

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A morphological study of the remnant of extrahepalic bile duct and the wedge liver biopsies has been performed in 50 eases of uncorreciable biliary atresia with no associated anomalies and 14 combined by preduodenal porlal vein. There were 8 cases with rudiment1 or agenesis of the gallbladder, and 10 cases with agenesis of the common bile duct in the remnant of the extrahepatic bile duct combined by preduodenal portal vein. We classified these histologic specimens of the remnants into 3 types (Type I, Ⅱ and Ⅲ), according to the findings of the ductal structure and the surrounding tissue. Almost all cases (96%) with no associated anomlies had the gallbadder of Type I (the internal diameter of the bile duct was more than 100 μ), but the gallbladders in two cases combined by preduodenal portal vein were replaced by the fibrous tissue as Type Ⅲ (fibrosis without any duct or ductuli structure). Liver fibrosis in patients combined by preduodenal portal vein was more severe than those with no associated anomalies. We suppose that the difference of these morphological findings in the remnants and the liver biopsies may be the result of various developmental stages of the pathogenic process.

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There have been only a few reports about relationships between operative results and myocardial protection in pediatric cardiac surgery. The purpose of this paper is to evaluate the effects of myocardial protection in patients undergoing total correction for tetralogy of Pallet (TF): Thirty three patients with TF had undergone corrective surgery during a period from 1977 to 1979, In 9 patients, intermittent aortic cross clamping was performed (Group A). Thirteen patients received cold cardiopegia only (Group B) and other 11 cold cardioplegia with topical cooling (Group C). The operations were performed with trans-right venticular or trans-atrial trans-pulmonary arterial approach under mild hypothermic extracorporeal circulation. The duration of aortic cross clamping was significantry longer in cardioplegic groups than in non-cardioplegic group. The heart started to beat spontaneously after aortic de-clamping in 22.2% of patieots In group A. 23.1% in group B and 72.7% in group C. The rate of the spontaneous recovery of beating in group C was significantly better than those in group A and B. The dose of isoproterenol after operation was less in group B and C than in group A significanly. The mortality C was 33.3%, 7.7% and 9.1% in group A, B and respectively. Myocardial protection by cardioplegia with topical cooling provided longer aortic cross clamping period. The method of myocardial protection reported herein is one of the factors which have improved the result of corrective surgery for tetralogy of Fallot.

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Twenty four patients with esophageal atresia associated with tracheo-esophageal fistula, who were classified as Waterstone C group, were reviewed. Thirteen patients survived after primary esophageal anastomosis with a division of the tracheo-esophageal fistula. This procedure was performed on eight patients shortly after admission, bul only four survived. On the other hand, it was performed on ten patients few days or few weeks after admission and nine survived. None of the patients who hac! undergone only gasirostomy or no operation were alive. Femimonia was successfully treated by respiratory care including tracheal suction, physiotherapy, mechanical ventilation and antibiotics, in spite of the presence of tracheo-esophageal fistula in must of patients within two weeks after admission. Adequate nutritional support was provided by a transpyloric or jejunostomy tube until delayed primary anastomosis was performed. Duodenal obstruction and imperforate anus were treated before primary anastomosis was performed. A patient with progressive congenstive heart failure due ro aor to -pulmonary septal defect was treated surgically before esophageal operation. Most of other cardiovascular anomalies did not require any surgical treatment before delayed primary anastomosis. A premature less than 1,500 gm without any severe anomalies and complication survived afler delayed primary anastomosis. In conclusion, delayed primary anastomosis can be superior to staged procedures tor poor risk patients today when proper respiratory care and nutritional management are available in most pediatric surgical centers.

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A study of 171 children with gastrointestinal bleeding occurring during the past 14 years wa carried out with special references to preoperative diagnosis of bleeding Meckel's diverticulum and surgical aspects of Schonlein-Henoch's purpura. The diagnosis of bleeding Meckel's diverticulum in 3 children was established preoperatively using 99mTc-pertechnetate. These abdominal scannings were of value in the management of patients with rectal bleeding after ruling out other disorders by hematologic tests, usual radiologic studies and endoscopic examinations. Four cases of Schonlein-Henoch purpura who had undergone surgical intervention were reported. Our emphasize is that it is difficult to differentiate pain, vomiting, and bleeding due to purpura from those occurring secondary to coexisting intersusception, but if there is continuing doubt concerning the surgical complications of Schonlein-Henoch's purpura, exploratory operation should be undertaken.

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Total parenteral nutrition (TPN) has gained wide acceptance as valuable adjunct in the care of critically ill patients. However, the risk of catheter-related sepsis is still of continued concern. The investigation on the current state of TPN and incidence of catheter-related sepsis was performed by the questionnaire method to 1,064 general hospitals throughout Japan in 1980. Replies submitted to the questionnaire were obtained from 569 hospitals (53.5%). Catheter-related sepsis was defined as an episode of sepsis, for which no anatomic locus could be identified, that is resolved on removal of the catheter. This time, catheter-related sepsis in pediatric patients seen in 126 hospitals was especially reviewed and analyzed. Catheter-related sepsis in 1979 was associated with 16.2% of catheters and occurred in 13.0% of patients. This incidence was little higher comparing with 11.2% in adult patients. The predominant organism of catheter-tip culture was Staph. epidermidis. The aseptic technique provided at each institution was also investigated. By this analysis, futher need for a precise protocol delivered by an experienced team is emphasized to minimize the incidence of catheter-related sepsis.

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We performed surgical reconstruction for the hypertrophied clitoris in 4 girls with congenital aclrenocortical hyperplasia. We used the technique of removing the phallic shaft and preserving the glans which was described by Spence and Allen. Results in 4 cases have been all satisfactry especially in cosmetic aspect. In this report, we discussed the surgical techniques, the time of operation and the steroid replacement procedure.

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In our experiences of 22 congenital bile duct dilatations (CBD), 3 cases were accompanied by chronic calculous pancreatitis. Through the management of those 3 cases, we obtained following suggestions. 1) Some of 'pancreatic calculi of unknown etiology' (which are more often found in female than in male) may be associated with anomalous arrangements of the pancraticobiliary ducts. 2) Pancreatolithiasis in chole doc ho-pancreatic common channel anomalies and alcohol abuse may differ in pathogenesis and composition of the stone. 3) An appropriate drainage procedure for the pancreatic duct system should be included in the treatment of CBD with pancreatolifhasis.

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Two handred of the 310 children that died in the Netherlands over a 10 year period as a result of a liver or biliary tract condition could have been potential candidates for liver transplantation. Seventy five percent of these candidates had biliary atresia of which the incidence in this country is estimated to be 0.93 per 10,000 life births. At an actual annual birth rate of 180,000, sixteen to seventeen new caces may be expected per year of which 13 to 14 will die, if the results of treatment remain unchanged. Even if the Japanese high standard of treatment can be reached, a considerable number of patients with biliary atresia will progress to end stage hepatic disease. The sesuts of livertransplantation achieved in these patients up till now are poor, showing the need for further experimental work.

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Experience of 14 patients with Hirschsprung's disease underwent definitive operation was reviewed and these early postoperative complications and functional results were analysed. Of these 14 cases, 8 were treated by Rehbein procedure, 3 by low anterior resection with rectal myectomy (L.A.R. with myectomy) and 3 by PEARM procedure, The early postoperative complications encountered in this series were mild anastomotic stricture in 3 of 8 patients of Rehbein procedure and fecal fistula in 1 of 3 patients with PEARM. Functional results were evaluated by clinical course and manometric study. Of 7 cases who had undergone Rehbein procedure, 2 have developed constipation, requiring suppositories or enema. Manometric study has shown that patients with Hirschsprung's disease after Rehbein procedure tend to retain higher resting pressure in the anal canal than normal subject as before operation while the patients after 3 of L.A.R. with myectomy have normal pressure in the anal canal achieving good fuunctional results. Thus, it seemed that an anal stretching is an insufficient procedure for sphincter achalasia remaining after Rehbein procedure suggesting sphincter achalasia should be treated by rectal myectomy.

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Fibrous hamartoma is a relatively rare subcutaneous soil tissue tumour in children. A one-year and four-month old hoy with fibrous hamartoma occupying in the right buttock was reported. The tumour was 3.O × 2.O × 1.0 em in size and elastic soft, which was firstly noticed at the age of 6 months and had grown gradually over the following 8 months. At surgery, the tumour was not circumscribed and was adherent to the adipose tissues. Historically, it showed the organoid structure consisting of three components: (1) traversing fibrocollagenous tissue; (2) immature inesenchymal tissue with mucoid matrix nnd (3) mature adipose tissue. His postoperative course was uneventful and no recurrence has been noticed for more than one year, at the time of this report.

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Two of 56 cases with infantile hypertrophic pyloric stenosis(IHPS)began to vomit at 12 hour and 24liour after birth, Pyloromyotomy performed on 4 day and 9 day of age disclosed a typical pyloric tumor in both cases. Neither of them was the first born child and clinical features common to both cases included jaundice, hemateraesis, constipation, oliguria or anuria, hemoconcentration without hypochloremia, high blood urea nitrogen concentration, free gastroesophageal reflux, marked obstruction of pyloric canal with typical roentgenographic manifestations of IHPS prior to operation and somewhat smaller pyloric tumor than in the average cases. The average age at onset of IHPS in our institution is 22 days seen in the cases from 1959 to 1969 and 16.7 days in those from 1970 to 1981. Seventeen percent of cases in the earlier group began to vomit before 15 days of life but this figures increased to 51% in the latter group. Etiology of IHPS remians to be elucidated but acquired causes, such as milk allergy, could not be considered in these cases.

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Case 1: A male infant underwent a sigmoid colostomy for supralevator imperforaie anus. A urological study revealed recto-vesical fistula and urethro-ejaculatory reflux. At 3 months of age the boy had painful swelling on the left scrotum. An acute epididymitis was diagnosed by surgical exploration. The symptoms subsided by oral antibiotics administraticn. Case 2: A newborn baby underwent a sigmoid colostomy for supralevator anorecral malformaion. At 6 months of age he suffered from left scrotal pain and swelling. From the experience of preveous case acute epidiymitis was diagnosed. The administration of oral antibiotics resulted in an improvement of the symptoms. At 11 months of age a urethro-ejaculatory reflux was proven by voiding cystourethrogram. Case 3 : A newborn baby complained of constipation and abdminal distension and a diagnosis of rectal stensis was made at 4 days after his birth. Dilatation of the rectum by bougie was repealed in another hospital. He visited to our Center at one month of age and a colostomy was performed for constipation and colitis. At 4 months of age swelling and redness were observed on his left scrotum. Exploratory operation was performed because there was a doubt of testicular torsion. Acute epididymitis was diagnosed again and he was treated by antibiotics, which resulted disappearence of the symptoms. Acute epididymitis is uncommon in children. The presented three cases of acute epididymitis were associated with anorectal malformation in all. This malformation may be pre-exsisting factors which may induce an acute epididymitis in the special case. Urological examination should be performed in the early course of treatment for anorectal anomalies. If urethroejaculatory reflux was diagnosed, care must be taken to prevent epididymitis and oral administration of antibiotcs should be given before the radical surgery is advisable.

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A female child, 5 years and 5 months of age, who had had longstanding obstructive jaundice with hepatic dysfunction form the age of 6 months, underwent a Roux-Y hepaticojejunostomy after excision of the entire extrahepatic bile duct with severe periductal fiibrosis. At the time, liver biopsy already showed a progress of biliary cirrhosis with prominent ductal proliferation in the Glisson triad. Although good bile drainage to the intestine was obtained early after the operation, disappearance of jaundice and ascites with improvement of liver function has required a lapse of 2 years. The patient is now 13 years old and doing well with moderately impaired liver function. No esophageal varices have been seen by endoscopy lately. The clinical course and data are suggestive of an abortive form of biliary atresia.

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A case of Meckel's diverticulitis with abscess formation diagnosed by ultarsound is reported. This is the first case report diagnosed by ultrasound so far. A 5-year-old girl presented to our hospital with 3 days history of high fever and right hypochondralgia. In newborn period, she had undergone abdominal drainage with the same symptoms with an intraabdominal abscess of unknown origin. On admission, ultrasonographic study revealed a cystic lesion at the right hypochondral region. A Meckel's diverticulum was diagnosed with ultrasonically guided percutaneous contrast medium injection to the abscess lumen. Drainage catheter was inserted to the diverticulum using a real time puncture transducer. Subsequently the diverticulum was resected radically. Meckel's diverticulum in the neonate is rare. This case showed unusual complication of diverticulitis in the newborn period. The diagnosis of Meckel's diverticulum prior to laparotomy is difficult and uncertain though several diagnostic techniques have been employed including barium examination of the small intestine, angiography and isotope scanning. However in this report the diverticulum was possibly diagnosed preoperatively by ultrasound. The usefulness of this method in diagnosis of this disease has been demonstrated.

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Duodenal rupture caused by abdominal trauma is rare in child. Up to this lime, only 25 cases have been reported in Japan. We report here such a case who is 10-year-old boy and has been successfully treated. During play, he was contused at his upper abdomen by a horizontal bar, and admitted to our hospital on the next day. The emergency operation was performed, which revealed that abdominal organs were intact. However, the retroperitoneum near the duodenum was inflammatory. The 2nd portion of the duodenum was found to be ruptured in an extent of about two thirds of the duodenal circumference. After debridmement and closure of the wound, a decompression tube was inserted into the duodenum, and a Penrose drain into the retroperitoneal space near the wound. Although postoperative anastomotic leakage happened, continuous suction from the retroperitoneal space has provided a perfect closure and he was discharged on the 32nd day after the operation. After discharge, clinical course was uneventful for more than one year.

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