Anorectal manometry with perfused side opening catheter was carried out in 86 normal infants and children, 9 infants with Hirschsprung's disease, and 10 children with idiopathic chronic constipation. Studies on pressure volume curve of the rectum and rectal compliance were carried out in 37 normal infants and children, 4 infants with Hirschsprung's disease, and 10 children with idiopathic chronic constipation. Decreased basal rhythmic contraction of the anal canal, absent recto-anal reflex, steep elevation of pressure volume curve of the rectum and low rectal compliance were characteristic in infants with Hirschsprung's disease. Resting pressure profile of the anorectum, recto-anal reflex, pressure volume curve of the rectum and rectal compliance were normal in chronically constipated children without mega-rectum, whereas remarkably slow elevation of pressure volume curve of the rectum and high rectal compliance was characteristic in those with megarectum. Normal resting pressure profile of the anorectum and abnormally high rectal compliance seen in constipated children with megarectum suggest that this type of constipation is a result of primary rectal ectasia, whereas normal resting pressure profile and rectal compliance in constipated children without megarectum indicate that constipation is a result of disturbed motility of the colon in these patients.
With reference to psychosomatic growth after pediatric surgery, analytical studies were done using questionnair upon 310 cases who received surgery at Showa University Hospital in the past 10 years. Of these, 154 answers were divided into 127 major 27 cases of minor surgical groups. Post surgical health condition is quite satisfactory. At the present time, mental and emotional development is normal compared with healthy children. Complications resulting from the original disease are seen in 18 cases. 65% of the families have serious anxiety for relapse of the illness in the future As for the surgical scar, 11 patients desire to receive plastic repair in the future. In comparison of pre and post surgical conditions, 80 cases have no change, but 5 cases aggravated in the movement, and 26 cases improved in the health. From the view point of the Kaup Index in pre and post surgical conditions, almost all cases have showed remarkable improvement. In the psychological tests, the D. Q. (Development Quotient) values have improved in process of age. Analysis of the PAT (Parent Attitude Test) suggestes that the mental and emotional development after surgery is affected by patient's parent. In conelusion, the pediatric surgeons must give an adequate explanation of disease and post surgical course to parents for a better understanding of the situation for both patients and parents, thereby contributing to a healthy development of the character and mind of the children. For healthy psychosomatic development after surgery, recognition of patient's disease by the parents is the most important factor.
An intraoperative irradiation with an electron dose of 1,000-1,500rads carried out in three cases of abdominal tumor. In this radiotherapy technique, radiation damage to the normal structures is minimized, since the normal organs adjacent to the tumor can be shifted from the field so that the lesion may be exposed directly to high dose radiation. Thus, a sufficient dose can be delivered to the lesions without affecting the small intestine or other organs. Indication of intraoperative irradiation for children with abdominal tumor is summarized as follows : a) the primary tumor must be extirpated surgically, b) the residual tumor should be microscopic or small and unresectable because of its location adjacent to the important organs. Case 1 : Five year old boy with bilateral Wilms'tumor, who received left nephrectomy fifteen months ago, underwent right nephrectomy under hemodialysis. The tumor was 17.2×10×7.5cm in size located right retroperitoneal area with invasion to liver and lymph node metastases along the abdominal aorta. The primary tumor was removed but elimination of the metastatic lesions was incomplete. A single dose of 1,000rads was delivered to the infiltrated retroperitoneal tissue including the contact metastases to the liver through 8cm diameter field and the lymph node metastases through 10×8cm field. Case 2: The patient was one year and eleven month old girl with right Wilm's tumor. The primary tumor was 11 X 9. 0×6.5 cm in size and was removed by right nephrectomy. A single dose of 1,500rads was given to the tumor bed through 8cm diameter field. Case 3: The patient was nine month old boy with neuroblastoma, which could not be determined the primary lesion, The mass of paraaortic lymph node was removed and found multiple mesenteric lymph node metastases.Infiltrated paraaortic lesion and mesenteric lymph node metastases were irradiated with 1,500rads through 6cm diameter and 6×8cm field. Indication, irradiation technique, and dose of intraoperative irradiation for the abdominal tumor in children were discussed.
The experience with 20 ceses of congenital esophageal atresia during the past 17 years from 1963 to 1979 at First Department of Surgery, Kyoto Prefectural University of Medicine was reviewed. The result was analyzed regarding the aspects of age at diagnosis, preoperative condition, and surgical procedures. Of the tweleve patients who underwent primary anastomosis of the esophagus from 1963 to 1971, only one (17%)was alive. On the other hand, 5 of 6 patients (83%) were alive from 1972 to 1979. This means that the surgical treament of esophageal atresia have definitely improved recently. The progress in anesthesia, postoperative care, and operative technique including use of extrapleural approach is the important contributing factor in achieving better result. Extrapleural approach especially has greately contributed to the imporovement in the treatment of congenital esophageal atresia. From these data, the protocol in our clinic the treatment of congenital esophageal atresia is to perform primary anastomosis following gastrostomy and to carry a single layer anasto-mosis of the esophagus using extrapleural approach.
Five patients with recurrent rectourinary fistula as postoperative complication of pull through procedure for anorectal anomaly were presented. In four patients, the initial surgery was perpormed at the other institution and the accurate classification of the lesion was not avail-able. They were all complicated with urinary discharge from the anus, starting at one month to two years after the initial operation. The rectourinary fistula was located at 3cm proximal to anal mucocutaneous junction in four cases. Proctoscopic observation of the fistula orifice was possible in these cases. They were all successfully treated by direct closure of the fistula through perineal approach. A creation of the colostomy was indicated in one patient for severe perineal erosion. In one patient, a sacroperineal pull through operation had been performed for rectourethral fistula at our institute. Anterior wall of the rectum was injured longitudinally at division of th rectourethral fistula, which was closed carefully in two layers. Postoperatively the urine examination revealed positive E. coli repeatedly. A urethrogram revealed a recurrent rectouri-nary fistula at rather proximal portion comparing to the other four cases. A endorectal pull through procedure was successfully performed for this case. By the experience of these five cases, the authors have concluded as follows: 1) Cause of the recurrent rectourinary fistula: (1) Wrong pull through procedure without recognition of the presenting fistula (in two). (2) Anterior rectal wall injury (in one). (3) Intra and/or postoperative injury of the urethra caused by bougie (suspected in two cases). 2) Treatment of recurrent rectourinary fistula: (1) Perineal closure of the fistula is indicated for low type fistula. Pull through of the anterior wall of the rectum over the site of the fistura is important. Colostomyis not always nescessary. (2) Endorectal pull through is indicated for high type fistula.
Eighty-nine cases of biliary atresia were surgically treated at Tohoku University Hospital during the period from January, 1971 to November, 1979. Re-operations were carried out on 16 patients. These were revision of portojejunostomy for 10, conversion of portocholecystostomy for 4, and conversion of hepaticojejunostomy to portojejunostomy for 2 patients. Excellent bile excretion was obtained in 12 patients after re-operation. By analyses of these 16 cases, following conclusion was obtained: 1) There is a definite indication for re-operation in the patients in whom bile excretion discontinues after excellent bile excretion was once achieved. 2) There is an indication for re-operation in patients of Type I lesion, even if bile excretion was not obtained after the first operation. In these circumstances, optimal time for re-operation is about a month after the first ope-ration. 3) Relative indication is for the patient whose first operation was done before 60 days after birth. In this occasion, the re-operation also should be performed before a month after the first operation in order to do the second surgery within 90 days after birth.
Our experience on one stage urethroplasty performed in 8 children with distal hypospadia was reported. It was primarily successful in 6 patients. Of the remaining 2, one developed slightly crooked glans which is of no consequence and the other one required secondary closure of urethrocutaneous fistula. The concept of cutaneous chordee and its amenability to skin lysisalone was presented. Our modified King's method was suggested to be tried as relatively foulproof one stage urethroplasty.
A case of esophageal atresia classified as Gross C type associated with two fistulas arising from the lower esophagus to the trachea and the left main bronchus was experienced. We successfully reconstructed the esophagus by division of the TEFs and primary esophago-esophageal anastomosis, although there was a 3cm gap.
A neonate who had gastric rupture and necrotizing enterocolitis was reported. On the histological investigation of the involved stomach, jejunum and colon, it was characteristic that there were minimum infiltrations of the inflamatory cells and marked intramural bleeding. A review of the cases with gastric rupture and the cases with necrotizing enterocolitis disclosed a high incidence combined with perinatal hypoxia in both conditions. It seems that there may be a close etiological relation between idiopathic gastric rupture and necrotizing enterocolitis.
A 2-month-old boy was admitted to the hospital because of tachypnea and heart murmur. Cardiac catheterization and cineangiocardiography revealed truncus arteriosus (Collett & Edwards'type I) associated with interruption of the aortic arch distal to the left common carotid artery (Type B), namely Van Praagh's type A4. Surgery was performed at 60 days of age with the weight of 2,340g through a left poste-lolateral thoracotomy in the 3rd intercostal space. Combined bilateral main pulmonary arterial banding, division of PDA and an end to end anastomosis between the left common carotid artery and the descending aorta were carried out. The postoperative hemodynamics was stable but the patient died 4 hours after operation because of respiratory accident. This anomaly is seldom to see and this paper is the first case report of surgical repair to the truncus arteriosus type A4 in Japan.
A six years old boy presenting with positive HBs antigen underwent a successful resection of the liver tumor by a left hepatic trisegmentectomy. The family history revealed his mother with positive HBs antigen and negative HBs antibody and his grandfather of the mother side died of liver cancer. The histological specimen of the tumor showed an adult type liver cancer and cirrhosis of the liver. By a literal review, our boy is a youngest case of an adult type liver cancer with an association of positive HBs antigen. The positive HBs antigen may be due to maternal transmission but this could be the cause of liver cancer as reported elsewhere in the older age group. This paper presents with our experience with a discussion of an etio-logical relation of positive HBs antigen and development of adult type liver cancer.
A one-month-old infant was operated on for intestinal obstruction with vomiting and abdominal distension. At operation, a cystic tubular mass resembling like some part of the intestine in apperance, was found. There was no attachment of this mass to the normal alimentary tract, but there was an isolated mesenteric structure. Histology of the mass showed the intestinal structure. There was an associated vertebral anomaly, which is usually seen in an alimentary tract duplication proving a notochordal theory. The etiology and general concepts' of alimentary duplication are discussed.
A seven-days-old hypoxic and moribund baby with double outlet right ventricle, pulmonary atresia and patent ductus arteriosus underwent formalin fixation of the ductus for palliation. Prostaglandin E_1 was used during surgery. The baby survived and a good continuous murmer was audible after surgery. One and half month later, however, the ductus murmer became less apparent and completely disappered 3 months postoperatively. Subsequenly, a successful right Blalock-Taussing shunt with interposition of the Gore-Tex graft was performed. The indication of this procedure should be strictly limitted because of the poor long term effect, but may be utilized for severely hypoxic and moribund infant with complex cardiac anomalies
Six cases of congenital diaphragmatic hernia and 8 cases of neonatal gastric perforation have been experienced at the National Kagawa Children's Hospital during the past 6 years. One case had a combination of the two diseases. Case report: A full term boy, weighing 3,280g with normal delivery developed abdominal distension and vomiting on the second day after birth. On the third day, he was admitted to our hospital because of respiratory distress. An abnormal eventration of the left diaphragm and free air in the abdominal cavity were recognized by plain x-ray films. A congenital anomaly of the left diaphragm and neonatal gastric perforation were diagnosed. At operation, gastric perforation located in the posterior wall of the stomach and left posterolateral diaphrag-matic hernia with a thin sac measured 2x4cm in width were found. The diaphragmatic hernia and gastric perforation were primarily repaired. The postoperative course was uneventful ex-cept for atelectasis of the left lung. He is now well at the age of 2 years.
A neonate who showed right-sided diaphragmatic elevation on a chest X-ray is presented. Transabdominal plication of the right hemidiaphragm was carried out at 13 days of age. Dur-ing the operation both hemidiaphragms were found to be hypoplastic. The patient died at 15 days of age due to respiratory insufficiency. Diaphragmatic eventration refers to absence or deficiency of diaphragmatic muscle, with resultant elevation. Bilateral diaphragmatic eventration is rare, and its prognosis is extremely poor. The literatures including 20 cases of bilateral diaphragmatic eventration in Japan and the Western Countries were reviewed. The diagnosis by chest X-ray films is rather difficult and complicated. Of these 20 cases, 8 actually showed bilateral elevation. But 6 cases were reported to have unilateral diaphragmatic elevation on the chest X-rays, as presented in our case. Recognition of the existence of the "masked" bilateral diaphragmatic hypoplasia is one of the important factors in managing postoperative respiratory care. Therefore, transabdominal plication of the diaphragm is preferable as the operative procedure, because it is much easier to find bilateral diaphragmatic hypoplasia than thoracic approach.
A case of 6 years old developing acute pancreatitis in assocation with double gallbladder is reported. The patient developed a pancreatic pseudocyst after primary drainage operation for pancreatitis, to which a pseudpcystogastrostomy was performed. In revewing reports in Japanese journals of pediatric patients with acute pancreatitis and pancreatic pseudocysts, 135 for the former and 75 for the latter including 75 cases of adults, an association of double gallbladder was noted. Among the etiological factors of pancreatitis, anomaly in the biliary tract including con-genital dilatation of the bile duct is considered to be most likely cause. In the adult cases of pancreatic pseudocysts, two thirds are due to pancreatitis, whereas in the chilldren, two thirds are due to traumatic injury. Since four out five patients with pancreatic pseudocyst in the Japanese series were associ-ate with double gallbladder, we presume that the double gallbladder has some possible re-levance to the development of acute pancreatitis.
Surveies on neonatal surgery in Japan were made in 1964, 1968 and 1973, and the results were presented at the 2nd, 6th and 11th annual meetings of Japanese Society of Pediatric Surgeons (J. S. P. S.) respectively. In 1978, the 4th survey was made, reporting that 2,149 neonatal patients were surgically treated at 315 institutes all over Japan in one year period. These materials were analyzed and presented by A. Tsunoda (the President of J. S. P. S.) at the 16th annual meeting. However, there are some intereating data that have not been included in Tsunoda's presentation. This paper summarizes those materials as the additional study. With the patients in the survey in 1978, including 153 congenital esophageal atresia, 360 ano-rectal malformation, 114 malrotation of the intestine, 318 congenital intestinal atresia 101 gastric rupture, 147 omphalocele and 44 gastroschisis, an analysis was undertaken on each disease group regarding 1)incidence, 2)relationship of birth-weight, types and surgical tre-atment to motality, 3) effect of hyperalimentation to outcome of surgical management, 4)ass-ociated anomalies, and 5)cause of death.