A safe and simple surgical technique for creation of an atrial septal defect is reported. The atrial septum was incised with two surgical steel wires which had penetrated through the atrial septum from outside the right and left atria. Fourteen dogs were operated upon by this technique with satisfactory results. The advantages of this new technique include 1) obviation of even temporary circulatory occlusion, 2) avoidance of air embolism, 3) no need for special instruments, and 4) technical easiness.
Histochemical diagnosis using acetylcholinesterase (AChE) staining is very useful for differentiation of Hirschsprung's disease from idiopathic constipation. This method can show marked proliferation of nerve fibers in aganglionic segment of the colon. We have studied histochemically the localization of AChE and ChE (nonspecific cholinesterase) in specimens obtained by either operation or suction biopsy. ChE activities were observed in nerve plexus and not detected in nerve fibers. Electromicroscopically, the localization of ChE were detected mainly around the ganglion cells and neuropils. AChE activities in specimens obtained by rectal biopsy can be falsely negative or positive. In our studies, false negative cases could be avoided by suitable position of rectal biopsy or appropriate evaluation of AChE activities. False positive findings were observed in two cases of CIIPS (chronic idiopathic intestinal pseudo-obstruction syndrome) and a case of septic neonate. In these cases, ChE staining was useful for detection of ganglion cells. Though the AChE staining alone can produce false negative or false positive result, we could get good result by using both AChE and ChE staining in the same materials. For these reasons, we recommend ChE staining for supportive diagnostic method of Hirschsprung's disease and examination of intestinal dysfunction.
Open liver biopsy specimens in sixty cases of biliary atresia were studied histopathologically, with particularly concerning the degeneration of intrahepaticbile duct (IHBD). Histopathological findings of the IHBD were classified into three groups, according to the severity of degenerative changes of bile duct: In group I (13 cases), degenerative changes were minimal and no bile plugs were identified. In group II (27 cases), degeneration was moderate and bile plugs were found in less than 10% of the total bile ducts. In group III (20 cases), severe degeneration and bile plugs were found in more than 10% of the total bile ducts. This classification of the degeneration of the IHBD was closely correlated to the age at operation and the degree of liver fibrosis. Severe bile cuct degeneration resulted poor bile flow after operation. Thus, the classifi-cation of the degenerative change of the IHBD is one of the most important factors, determin-ing the prognosis in biliary atresia patients.
This experiment showed that the characteristic difference in the regulation system of blood flow between normal and maligmant tissues was produced by angiotensin II induced hypertension (150 mmHg). Blood flow in tumor tissue of VX-2 carcinoma transplanted intramuscularly in rabbit was remarkably increased by angiotensin II induced hypertension, while blood flow in normal tissue of rabbit (liver, kidney, muscle), was almost constant after induction of hypertension. Based on this findings, a new approach to cancer chemotherapy, combined chemotherapeutic agent and angiotensin II, was discussed. Murine neuroblastoma (C-1300) transplanted intracutaneously in A/J mouse were employed in this experiment. Marked enhancement of the chemotherapeutic effect was noted in the group receiving active type cyclophosphamide (40487) intravenously under angiotensin II induced hypertension, compered with the control group and the group receiving active type cyclophosphamide (40487) alone intravenously. When angiotensin II and active type cyclophosphamide (40487) were given intraperitonealy, above enhancement of chemotherapeutic effect din not find out.
To invetigate the effects of the refluxed pancreatic juice on the biliary duct due to the age factor of canine models, pancreatico-choledochal anastomoses were performed experimentally on 24 canines, 12 in adult and 12 in puppy, as a model of anomalous arrangement of the pancreaticobiliary ductal system (AAPABIDS). The following results were obtained: 1) The dilatation of the choledochus was noted within one or two weeks after operation and the types of dilatation were spindle or cylindric in adult dogs and puppies. 2) The mean dilatation ratios of the choledochus were about 4.8-fold in adult dogs and 8.6-fold in puppies. 3) The mean elevation ratio of the amylase content level of adult dogs was higher than that of puppies. However, no significant difference of amylase level was found between adult dogs and puppies. 4) Common histological findings in these experimental models were desquamation of the lining epithelium, inflammatory cell infiltration and fibrosis of the wall. Mucous glands were seen in adult dogs and puppies. Goblet cells and argyrophil cell were seen in a puppy.
A total of ninety-eight children underwent surgical treatment for mitral valvular disease from 1963 to June, 1984. Thirty-four patients (34.7%) (Congenital: 8, Aquired: 26) had mitral valve replacement, twenty patients (20%) had mitral valvuloplasty or mitral annuloplasty and forty-two patients with incomplete endocardial cushion defect (ECD) and two patients with complete forms of ECD had surgical repairs of the atrial septal defect and the mitral and tricuspid valve. The conclucions were as followes; 1) The first choice for surgical treatment for mitral valvular disease in children should be preservation of the natural valve by a conservative technique. 2) Prosthetic valve replacement in children is a life saving procedure, but the early failure in porcine heterograft valve replacement in children brought us to discontinue the routine use. 3) Surgical repair for mitral valve cleft with incomplete ECD should be performed in cases with a small regurgitation on angiocardiogram. Good repair reduced the incidence of cadriac failure and endocarditis in the postoperativd period. 4) We emphasized early primary repair of complete ECD in infancy because of un-controllable heart failure and early development of irreversibele pulmonary vascular obstructive disease.
Between 1959 and June, 1984, 63 patients with congenital mitral valve disease 43 parsistent common atrioventricular canal (CAVC) and mital incompetence with/without other cardiac anomalies) surgical repair. Mitral valve replacement was performed in 7 (11%) out of these 63 patients. The hospital mortality of 30 cases of incomplete CAVC was 33% (10/30), 38.5% (5/13) in the complete CAVC and 15% (3/20) in patients with mitral incompetence. Seven out of 10 hospital deaths in incomplete CAVC belonged to the early period of this series and there has been no death since 1981. Three out of 5 hospital deaths in complete CAVC was due to increased pulmonary vascular resistance during infancy. There was no late death. Six patients (9.5%) underwent reoperation mainly for residual mitral incompetenc (3), degenerative change of bioprosthesis (2) and as a second stage operation (1). Reconstructive surgery such as annuloplasty, chordoplasty and simple cleft suture for mitral lesions is most advisable procedure for infants and children, because prosthetic valve replace-ment in children is a palliative procedure at best. Mitral valve replacement with ventricular septum extension using mitral anterior leaflet in cases of persistent CAVC proposed by Asano is advisable procedure to protect His bundle and to release the obstruction of the left ventricular outflow tract by the implanted prosthesis. Preservation of posterior leaflet with its chordae and papillary muscles at MVR maintains left ventricular contraction and performance immediately after surgery and promises markedly improved recovery course.
During the past 18 years surgical treatment for the neonate with Hirschsprung's disease has been performed to 112 cases at the National Children's Hospital, Tokyo. Intestinal perforation was complicated in 4 cases at the time admission, and all of these cases were admitted within 3 days of life. Seven cases were died from enterocolitis and associated anomalies. From our experiences points of diagnosis and treatment of the neonatal Hirschsprung's disease are as follows. 1) Most of cases could be diagnosed by Barium enema only. 2) Barium enema should be repeated when the initial study failed to demonstrate the typical findings of aganglionosis and the surgical intervention could be wait by conservative managements. 3) Anorectal manometry and histochemical study should be performed actively in the period of waiting for the surgical treatment. 4) Colostomy is best made on the most distal portion of ganglionic colon. There is no necessity for remaining colostomy after the definitive operation. 5) Early primary operation could be indicated for some selected cases. Indications for early primay operation are full termed, well nutrished patients without enterocolitis. 6) Soave-Denda's procedure is considered feasible as a definitive operation for young patients.
The time of passage of the first stool was studied in 39 patients with Hirschsprung's disease and 2,471 normal neonates. Only 25.6% of the patients with Hirschsprung's disease passed their first stool by 24 hours with no aids to promote colonic evacuation, while 97.2% of 2,471 normal neonates passed their first stool within this period. About 10% of preterm and low-birth-weight neonates without Hirschsprung's disease passed their first stool after 24 hours, but the delay of meconium passege was remarkable in the patients with Hirschsprung's disease. In this study it was ascertained that failure to pass meconium within 24 hours after birth implies the possibility of Hirschsprung's disease, but it was erroneous that no delay in the first stool denies the possibility of Hirschsprung's disease. Patients with Hirschsprung's disease was divided into 5 groups by the length of aganglionic bowel, and the time of the first stool was studied in each 5 groups. The lack of positive correlation was recognized between the length of aganglionic bowel and the time of first stool. The time of the first meconium passage is not a useful diagnostic tool to estimate the length of aganglionic bowel.
During the period from 1961 to 1983, we have experienced 29 cases of esophageal atresia, and 5 cases were found to be associated with tacheomalacia. They were intubated for minimum 14 to muximum 196 days with intensive respiratory care, and all cases were successfully extubated. The etiology of the tracheomalacia associated with TEF and effective respiratory care and/or surgical treatment of this pathological conditions are discussed.
Motility of the ano-rectum was studied in 25 children who had undergone rectoplasty with posterior triangular colonic flap for Hirschsprung's disease. The examinations were also carried out in 40 age-matched controls. Postoperative sphincter control was fair in 2, and poor in 20 patients within 6 months after the operation, but good in 5, fair in 6, and poor in 0 in 11 patients at 2 or more years after the operation. Measurements of resting pressure profiles of the anorectum disclosed that higher rectal pressure and decreased basal rhythmic contraction were characteristic of the patients with Hirschsprung's disease. Recto-anal reflex was absent in all the patients. Postoperatively, decrease of rectal pressure, increase of anal canal pressure, increase in length of anal canal, and increase of basal thythmic contraction of anal canal were observed. Recto-anal reflex appeared in 9 out of 11 patients at more than 2 years after the operation. Rectal compliance was markedly less in the patients within 6 months after surgery than in the controls. Increase of rectal compliance was noted thereafter, and there was no significant difference of rectal compliance between the patients at more than 2 years after surgery and the age-matched controls. Mass contraction of the rectum was not observed in the controls and patients before the operation. The contraction was noted in all the patients within 6 months after surgery and in 9 out of 11 patients at more than 2 years after the operation. Observations in cine-defecography and measurements of the mass contraction of the rectum were in accordance in that the motility of the "neorectum" was gradually stabilized after the operation. Improvements of sphincter control of the postoperative patients were considered to be due to; stabilized motility of the neorectum, preservation of sphincter muscles, and maturation of reservoir function.
Radiological studies in 60 case of congenital biliary dilatation showed that the stricture is the most significant etiologic factor for the condition of the bile duct. Cystic dilatation of extrahepatic bile duct was successfully created by the ligation of distalbile duct in infant rats. As for the etiologic factor of biliary dilation in our animal model which choledochopancreatic ductal anastomosis was performed in puppies, stricture nt the site of anastomosis was supposed to be more significant than the reflux of pancreatic juice. These clinical and experimental studies showed a definite evidence which the congenital stricture of the biliary tract associated with choledochopancreatic long common channel is the most important etiologic factor for the dilatation of the bile duct, particularly, in Type I (cystic dilatation), the stricture was supposed to be occured by the malfusion of the ventral pancreas and dorsal pancreas, at the site where the common bile duct was buried into the pancreatic parenchyma. In Type II (fusiform dilatation), the stricture occured at the site of intraduodenal common channel or choledochopancreatic ductul junction.
Five patients with bile stained vomiting due to external obstruction of duodenum and duodenojejunal junction are presented. Intestinal rotation seen in these patients was defferent from typical malrotation described by with Grob's, Snyder', or Louw'. We suggest that following 7 factors should be described for correct understanding of anatomy; degree of rotation of duodenal loop, degree of rotation and fixation of ceco-colic loop, presence or absence of volvulus, duodenal band, Ladd's band, adhesion of intestine, and kinking or angulation of intestine. Upper GI X-ray with barium meal is essential for the diagnosis, and individually modified surgical procudure should be considered according to the type of the disease.
A neonate with hepatic herniation through the foramen of Morgagni was reported. On the 6th day after birth, the baby was transferred to our neonatal ICU because of severe cynosis and tachypnea with a large mass occupying right hemithorax in a chest x-ray film. The baby was operated upon at age 35 days and a hepatic herniation through the foramen of Morgagni was found. In this case, the sac which contained a part of the right lobe of the liver of embriological shape measuring, 5×7 cm in size protruded into the pleural cavity com-pressing the right atrium and ventricle. The defect of the diaphragm was directly closed and reinforced with a Dacron velour patch. The postoperative course was uneventful. The value of PO_2 was improved but still remained al the lower level. In the postoperative perfusion scan of the lung, R-L shunt through Patent Foramen Ovale was found in spite of the decom-pression of the heart and lung. One of the reasons for the lower PO_2 may be ascribed to immaturity of the pulmonary vascular bed and alveoli, seen in Bochdalek hernia. The patient is currently well 2 years after the repair of hernia with minimal or without detectable pulmonary sequalae. He is classified as the late gestation or neonatal acquired congenital diaphragmatic hernia.
Familial occurrence of thyroid carcinoma, one of the patients was in childhood, has not been reported in Japan. We have experienced the carcinoma occurring in a mother and her son. Case 1: A 14-year-old boy with bilateral cervical tumors was admitted to our hospital and total thyroidectomy was performed. Histological diagnosis was papillary carcinoma of the thyroid. Case 2: (mother of Case 1) A 37-year-old female with right cervical tumor underwent right hemithyroidectomy. Histological diagnosis was follicular carcinoma of the thyroid. She has been completery free from any recurrence or metastsis for the following eight years. Eighty-two cases of thyroid carcinoma in childhood, including our cases, were collected from Japanese literatures arid reviewed. We were able to collect a total of thirty-five cases of familial occurrence of thyroid carcinoma from European and American literatures.
A six year old boy presented jaundice and cardiac murmur at the age of three. Laboratory tests showed moderate cholestasis and the liver biopsy disclosed intrahepatic bile duct hy-poplasia. Cardiac catheterization showed peripheral pulmonary stenosis and atrial septal defect. His face and vertebral deformity was characteristic with arteriohepatic dysplasia, Alagilles' syndrome. He underwent direct closure of atrial septal defect, right ventricular outflow and pulmonary arterial patch plasty. Postoperative course was uneventful and no hepatic derrangement was observed. This is the first reported case of open heart surgery carried out on a patient with this syndrome.
A case of a female neonate with congenital ileal atresia with a giant abdominal mass accompanied by calcification was reported. The giant abdominal mass was a unilocular cyst fulled with thick fluid and attached to the blind end of ileal atresia. The cyst was removed and a ileostomy was performed. The ileostomy was closed four months later and the patient was cured. In this case, there was no perforation of the alimentary tract, and the wall of the cyst was devoid of mucosal epithelium, and consisted of intestinal wall with marked granulation. There was also submucosal diffuse calcification and a strong response of atypical giant cells.
A 39-day-old female with multiple pyogenic liver abscess was treated effectively by US guided aspiration and antimicrobial chemotherapy. She had no history of umbilical vein catheterization and/or inflammation. About one week after birth, an abscess was found at her anterior chest wall. We considered that this subcutaneous abscess lead to general bacteremia and liver abscess in this case. CT and Ultrasonography showed three abscess in the liver. Aspiration of all abscess was performed under US guided. Staphylococcal aureus was grown in cultures of the pus. Aspiration of the pus and appropriate antimicrobial chemotherapy, cured liver abscess within one month. Drainage of multiple liver abscess is difficult and mortality rate of multiple liver abscess is higher than that of solitary one. Many authors have reported US guided drainage of the liver abscess, but all the reported cases were adult patients. Multiple liver abscess in infants is very serious disease, but US guided aspiration of abscess seems to be effective even in infants.
Pulmonary parenchyma and pleuma are common affected sites in the pulmonary fibrous histiocytoma and bronchogenic one is an extremely rare lesion. Only 11 cases of broncho genie fibrous histiocytoma could be found in the literatures reported from occidental countries. Therefore, this appears to be the first reported ease in Japan. The patient is an 8 year old girl who suffered from recurrent pneumonia and atelectasis caused by right endobronchial tumor. A bronchoscopical biopsy of the tumor revealed benign fibrous histiocytoma. Because of exacerbation of the symptomes she underwent right upper lobectomy and wedge resection of the bronchus including the tumor. Her postoperative course was uneventful and discharged on the 13th postoperative day. She is doing well without symptomes nor evidence of recurrence 16 months after surgery. The pathology, diagnosis and surgery of bronchogenic fibrous histiccytoma are also discussed in relation tothis case.
We experienced a case of female infant with very low birth weight (1,009g), whose ileum was obstructed with tenacious meconium. Resection of stenosed ileum and tube ileostomy were performed. At the beginning, we could not rule out meconium ileus by trypsin activity test of meconium. Therefore we gave her N-acetyl cysteine. In 3 months we could rule out mucoviscidosis by clinical findings and sweat test. We finally diagnosed it as "meconium disease" named by Rickham, but it was also similar to "meconium plug syndrome" named by Clalworthy. In this paper we discussed about meconium disease in comparison with meconium ileus and meconium plug syndrome.