Serum levels of nervous system-specific enolase (NSE) in patients with neuroblastoma and in control subjects were determined with a sensitive solid-phase sandwich enzyme immunoassay system, using a mono-specific antibody to the γ subnit of rat NSE for solid-phase preparation and for enzyme-labeled antibody. Using this assay system, the NSE in human sera can be estimated with a minimum measurable consentration of 0.6ng γγ equivalent/ml, with less than 10% of coefficients of variation for within-run (n=20) and between-run (n=15) assays. Serum levels of NSE in healthy adults ranged from 1.4-5.7ng/ml (2.87±1.18ng/ml, n=20), and those in control children (1-7 years old) were from 2.6 to 10.8ng/ml (5.76±2.42ng/ml, n=20). Serum samples (n=11) from patients with neuroblastoma contained high levels of NSE, distributing 13.6 to 330ng/ml (mean, 107ng/ml), however those from ganglioneuroblastoma patients were within a normal range (3.0-25.0ng/ml, mean, 8.3ng/ml). These results suggest that the NSE in serum might be a valuable marker substance for the screening and therapeutic monitoring of neuroblastoma.
One hudred and fiftyfive patients with anorectal malformation have been surgically treated from 1962 to 1980 at our institute. Postoperative anorectal functions were evuluated in 116 cases (more than 1 year postoperatively and older than 2 years of age) by Kelly's clinical score. In several cases, radiological examination and anorectal manometric studies were also performed. Low type: Surgical procedure was done by perineal approach. 64 of 73 cases (89%) showed "good" results. Postoperative anal musosal prolapse or stenosis required anoplasty in 12 cases (16%). Intermediate type: 8 cases were operated on by abdominoperineal approach, and 7 cases by perineal approach. 10 of 15 cases (68%) showed "good" results. As to results, no signficance was observed between these two different surgical approaches. The only case showing "poor" result was associated with Down's syndrome. High type: 28 cases were surgically treated by abdomino-perineal approach, and 39% of them had "poor" results. Pickerell's or Kottmeier's procedures were performed for incontinence in 7 cases, and anoplasty for anal mucosal prolaps in 21 cases. Sixteen cases with high and intermediate anomaly were evaluated twice by the same method 5 years ago and currently. Nine 9 of 16 cases showed improvement. In 4 of these 9 cases there was an improvement in clinical score by Kottmeier's operation or anoplasty, whereas the improvement was obtained without any surgical treatment in 5. Twenty-eight patients were studied by radiological examination and anorectal anorectal manometries. In "good" or "fair" cases, the pressure gradient between the rectum and the anal canal was more than 11 cmH_2O, and the anal resting pressure was more than 18 cmH_2O. In these cases, the PC-R angle (angle of the rectal floor line against the P-C line on lateral X-ray view) ranged from -10° to +20°, and the rectal dilation ratio (the rectal transverse diameter divided by the pelvic transverse diameter on frontal X-ray view) ranged from 40% to 60%. The cause of "poor" result with incontinence or severe constipation could be differentiated by evaluation of the rectal dilatation ratio and the anal resting pressure.
Dysfunction of the esophagus was observed after esophagoesophagostomy in patients with esophageal atresia. In these patients, simultaneous or spastic contraction of the esophagus or no contraction were observed by manometric studies. By useing experimental models which were made in dogs similar to esophageal atresia, esophageal function was studied by manometry. In the group of 10 dogs, the esophagus was divided and its continuity was restored by end-to-end anastomosis. The manometric studies were carried out at two weeks after the procedure. Normal peristalsis of the esophagus and relaxation of the lower esophageal sphincter (LES) were observed in all dogs. In the second group of 10 dogs, division of the esophagus was performed and the divided esophageal ends were anastomosed after the vagal nerve supplies were sacrificed. Two weeks after the procedure, peristalsis of the esophagus was not observed in all dogs. Simultaneous contraction was obsered in 7 out of 10 dogs and spastic contraction in 3 dogs. The activity of the LES remained unchanged by the procedure. In order to determine whether the esophageal motor dysfunction is congenital or acquired, manometric studies were performed before esophageal reconstruction in two patients of Gross A type and Gross B type through the gastrostomy. Irregular spastic contraction of the lower esophagus was observed in these patients. Same pattern was observed after esophageal reconstruction. By these studies, it was concluded that dysfunction of the esophagus after repair of esophageal atresia is caused by denervation of the esophagus and esophageal motor disorder in esophageal atresia is of congenital origin.
Using 62 canine models, the difference in the pathological changes of liver, choledochal duct and pancreas were studied in three groups of adult, puppy and neonate. The models of pancreatico-cholecystostomy (P-Cy) and pancreatico-choledochostomy (P-Ch) were created employing the technique which was described in the previous report. Creation of P-Ch in neonate puppy was technically impossible and only P-Cy has been created. Direct cholangiography and histological studies of these structures with HE, elastica van Gieson, Azan Maroly, PAS and alcian blue stainings were performed at 1 to 12 months after creation of the model. In cholangiographic findings in P-Cy models, there was no significant difference in choledochal dilatation among these three groups. In P-Ch models, the mean dilatation rate was 2.2 times in 5 puppies and 3.3 times in 17 adult dogs. In histopathological findings in P-Cy models, mucosal proliferation was less marked in neonatal puppies, while it was greater in puppies and adult dogs. In neonatal puppies, there was less cellular infiltration, and less decrease of the elastic fibers in choledochal wall. No tendency was observed, of predisposing the pathological changes of congenital biliary atresia or neonatal hepatitis. In histological changes of P-Ch model in adult dogs, mucosal thickening and proliferation were remarkable in choledochal and cholecystic mucosa. But there was no observation of true gastric or intestinal mucosal metaplasia in any animal in 12 months after creation of these models.
The experience of choledochal cyst during the past 20 years was reviewed. The result was reviewed. The result was analized regarding the aspects of symptoms, diagnosis, and operative findings and procedures. Radiologic examinations, such as abdominal echogram, CT scanning and liver scintigram, are the most helpful tools in establishing the correct preoperative diagnosis. A review of Operative procedures has revealed that the excision of the cyst and Roux-Y hepatico-jejunostomy carries a mortality risk no higher than cyst-enterostomies, and with fewer post-operative complications.
Electromyogram of the abdominal wall has been recorded in 6 children with peritonitis and 20 control patients. Abdominal tenderness and Blumberg's sign (rebound phenominon) have provided a certain pattern of the spike waves on electromyogram except for one newborn baby with severe pneumo-peritoneum due to duodenal perforation. In this patients, the absence of abdominal tenderness and Blumberg's sign was thought due to the absence of inflammation of the peritoneum in the abdominal wall, which was caused by insufflated air separating the abdominal wall from duodenal contents released in the peritoneal cavity by duodenal perforation. The control patients with no abdominal disease showed no spike wave on electromyogram by compression of the abdominal wall or abrupt release of compression. The record of electromyogram of the abdominal wall is useful to recognise abdominal tenderness or Blumberg's sign as an objective sign.
According to the literature, the term of "recurrence of T. E. F." is limitted to postoperative complication of esophageal repair in C-type esophageal atresia, but "recommunication of T. E. F." is also found during waiting for radical repair of esophagus after separating T. E. F. by primary operation. Of our 18 cases of esophageal atresia with TEF, we have reported 2 cases of the former type and 3 cases of the latter type, of which 2 cases died of pneumonia and were found recummunicating TEF at autopsy. From our experience, it was concluded as follows: 1) Two types of recurrent TEF exsist after and before radical repair of congenital esophageal atresia with TEF, and many cases of recommunication before esophageal repair are suspected to be dead without difinitive diagnosis. 2) Prejudice of "previous separation of TEF" inhibits to establish the diagnosis. Reseparation must be tried soon after improvement of lung findings as the mortality is high with conservative therapy. 3) Findings at autopsy and reoperation in the latter type suggest that minor leakage and abscess may penetrate the blind end of the lower esophagus and the trachea and the loosened ligature materials are expelled. Then abscess wall is firmly built and forms "recommunication of TEF" as if it had not been separated.
Influence of myocardial protection method to the postoperative hemodynamics was studied in two groups of infants under 12 months of age who had undergone a radical operation for congenital heart disease. The daily doses of diuretic were slightly different between the group of 9 patients who had recieved cold GIK (K: 20mEq/L, Insulin: 10 unit) injection after aoatic clamping at the operation, and the non GIK group of 11 patients who had received intermittent aortic cross-clamping. Both methods were effective.
The report describes operative complications seen in 76 surgical neonates over the past 10 years. The incidence of postoperative complications was 47.4% and postoperative mortality 23.7%. No difference in the incidence of postoperative complications was seen in two groups of the early period (from 1971 to 1975) and the late period (from 1976 to 1980). However, postoperative mortality was significantly low in the late period group(16.1%)comparing with the early period group (45%). The primary causes of death in the 1971-1975 period were pulmonary complication (6 patients), cardiac insufficiency (2) and peritonitis (1). Neonates complicated with respiratory distress before operation died of respiratory or cardiac insufficiency in the early stage of post operative course. Pulmonary problems (6 patients) were the most common complication in the 1976-1980 period, but deaths in the early stage of postoperative course has reduced and recovery from pulmonary complications was seen in some neonates in the late period. In addition, two neonates died of disseninated intravascular coagulopathy and one anastomotic leakage. Factors that have contributed to improvement of results in the late period are early diagnosis and prompt treatment, improvement of surgical treatment for neonates over 2,500g of body weight, choice of staged operation for esophageal atresia, anal atresia and gastroschisis, ventilatory support for prevention and treatment of pulmonary complications and intravenous hyperalimentation.
Multiple duplication is a very rare disease. This is report of a 6-month-old boy with the duplications in the mediastinum and the ileum. He was admitted to Kyushu University Hospital with an abnormal finding in the chest X-ray. Thoracotomy was performed under the diagnosis of the mediastinal tumour, which was actually a gastric cyst. Further investigation by means of ^<99m>Tc scintigram showed a presence of another duplication in the ileum, which was resected at the age of 3 years. Only 14 cases of multiple duplication have been reported in Japan. Duplication of the separate resions as seen in this case has ever been seen in only to cases. In this cace, ^<99m>Tc scintigram examination was very useful to diagnose the duplication in the ileum.
A 5 Year-old girl was admitted with abdominal pain lasting for 24 hours. With the pre-operative diagnosis of acute generalized peritonitis due to perforated appendictis, a laparotomy was performed. Operative findings included intestinal volvulus involving a part of the ileum, cecum and ascending colon with an absence of the dorsal mesentery. In addition, a shortend intestinal loop presented such a figure of an "apple-peel" appearance as seen in the newborn babies with proximal jejunal atresia. Resection of the infarcted bowel was performed at the most peripheral part included in the volvulus, with the fixation of the cecum to the lateral abdominal wall. The post-operative course was uneventful for more than one year after surgery without any episodoes of recurrence.
Multiple atresia of the colon is very rare. Only three cases have been successfully treated. A 3 day old full-term (3,200g) male infant was transferred to our hospital for abdominal distension and bilious vomiting. At the 1st operation, the total colon was found to be collapsed, while the terminal ileum was markedly dilated. A cecostomy was performed. At the 2nd operation 1 year later, type-I atresias (Louw's classification) of the ascending and sigmoid colons were found. The transverse colon was normal. The ascending and sigmoid colons were resected followed by an ileo-colostomy and a distal colostomy with good results. The etiology of atresia in this case may be explained by Tandler's theory, because the vacuoles at the obstructed areas were coverd with the immature epithelia, but didn't have any muscular layer.
Congenital biliary atresia was seen in female siblings, which is the second time in Japan. Jaundice was first seen in both patients at the first week of life. They were born to healthy parents without diseases of the hepatobiliary system. The elder patient died at the age of 10 months without operation. The younger was operated on (hepatic porto-enterostomy roux-Y) at 80 days of age (IIIbn). She was associated with polysplenia and is alive at 7 months after the operation. The Liver biopsy showed slight fibrosis, round cell infiltration and bile stasis. Except that their father was positive for HB antibody, these cases and their parents were within normal limit for our serous virolological studies. The cause of occurense of this disease in siblings was not obtained.
Simple ulcer of the cecum is a very rare condition especially in children. This is a report of chronic simple ulcer of the cecum seen in an eleven year old girl. The patient had noted postprandial abdominal pain. She had undergone appendectomy six months before visit to our hospital. However release of pain had not been achieved. A barium enema revealed a large ulcer in the cecum. For trial treatment, PAS, INH and streptomycin have been administered with some regression of the ulcer. It recurred soon while the treatment was continued. Ileocecal resection was performed six months after the first visit to our hospital. A sharply demarcated deep ulcer measuring 4cm×3cm was in the cecum. Pathological examination revealed no specific findings such as tuberculosis, Crohn's disease, parasites or malignant change. After the operation, she has gained 12kg of weight in a year. Simple ulcer of the cecum is a chronic disease and it may be the cause of retardation. It is often misdiagnosed as appendisitis. The recurrence rate seems rather low after the resection of the ulcer.
A case of intramuscular hemangioma was reported, because it was difficult to differentiate the tumor from malignant hemangioendothelioma. A 1 year and 9 month old male presented with a poorly defined, soft, smooth and tender mass measuring 3×4cm in the right lateral surface of the chest. The mass was excised with the adjacent muscle, because it was poorly demarcated. Histological examination demonstrated that it was an angiomatous tumor infiltrating into the striated muscle bundles and the endothelial cells had plumped nuclei and mitotic activity. According to these pathological findings, this tumor was suspected to be malignant hcmangioendothelioma by some pathologist. But this tumor was diagnosed as benign small vessel intramuscular hemangioma, because this was mainly composed of the endothelial cells and the small vessels of which the diameter was equal to the size of the red cell were predominantly seen. On follow up a year later, the patient was free of recurrence without radiation therapy and chemotherapy.
We have experienced a case of Wilms' tumor in whom a cystic pattern was domonstrated by ultrasonography. It was an unusual finding in our series of 15 patients with Wilms' tumor. In such situation, it is rather difficult to differentiate Wilms' tumor from other benign tumors during pre- and intra-operative periods. Differential diagnosis between Wilms' tumor and benign tumors, such as multiocular cyst of the kidney and partially differentiated cystic nephroblastoma, was discussed with a review of literature. It was concluded that intraoperative pathological diagnosis of such cystic diseases of the kidney is very important because operative procedure and its prognosis are entirely different if the tumor is malignant.
We have experienced a case of ileal atresia associated with total colon aganglionosis. The dianosis was initially made by histological observation of aganglionosis in the resected distal ileum and colon. Aganglionosis was found in the entire bowel distal to the atresia while the proximal bowel was normal. Etiology and pathogenesis of the association of ileal atresia with aganglionosis were discussed. The association of intestinal atresia with aganglionosis is very rare. No more than 14 cases have been found in the reports as far as we could determine. Five patients similar to ours have been reported. Three of these 6 cases including ours were complicated with malrotation and one with meconium peritonitis. These complications might have caused vascular accident, which may have interfered of the intramural ganglions from the oral to the anal direction at the early intrauterine life. It is doubtful, however, that all cases had vascular accident at the oligoganglionic segment.