Neuroblastoma originates from the sympathetic ganglions. Therefore, catecholamine and acetylcholine are very important substances for elucidating the origin of the tumor or maturation of tumor cells. In this research we examined the survival rate and the outbread frequency in viewing on preoperative urinary VMA and 9 item sush as the age of initial examination, the site of origin, tissue type etc., basing on the 330 cases of neuroblastoma collected from 11 facilities in Japan. We summarized some new facts on VMA as follows: (1) Under 1 year of age, the survival rate of the group with positive preoperative urinary VMA was higher than that with negative urinary VMA. In the group aged from 11/2 to 4 years, this observation was found to reverse. The incidence of positive preoperative urinary VMA increased by an advance of patients' age. (2) The survival rate in the group with + + and + + + of VMA was higher than in those with negative VMA, and in cases with a lesion in the adrenal gland, the relation was reversal. (3) In cases with metastasis to the lymph nodes, bones, bone marrow and orbital area,the patients with + + to + + + on VMA showed the higher survival rate than those with negative VMA. The incidence of metastasis was higher in the group with + + and + + + of VMA than those with negative VMA. (4) In cases with preoperative ESR value over 60mm in 2 hours, the number of patients with + + and + + + of VMA was greater than that with negative VMA. In cases with the preoperative LDH over 1,500, the result was reversal.
Neuroblastoma and Wilms' tumor are representative malignant abdominal tumors in Japan. In this paper, we studied the differetiation of 12 tumors (7: neuroblastoma, 4: Wilms' tumor and 1: pheochromocytoma); mostly of abdomen, by CT scanning. The results were as follows: 1. The 3 abdominal neuroblastomas originating from the adrenal gland were found to the central abdominal cavity, whereas, the 3 Wilms' tumors were found to grow outward laterally. 2. Tumor density was less remarkable in Wilms' tumor than in neuroblastoma. However, fine calcifications were observed in the high density area in neuroblastoma. 3. By contrast enhancement, neuroblastoma could be identified from the kidney, while Wilms' tumor unidentified. However, the displaced or deformed renal pelvis and/or calyces were characteristic in Wilms' tumor. 4. Hepatic metastases of neuroblastoma was visualized with a characteristic mosaic pattern by contrast enhancement. 5. In 50% of the cases in our study, CT scanning gives more information for diagnosing tumors than the usual X-ray methods previously used. In conclusion, we recommend CT scanning as a useful method for differential diagnosis of neuroblastoma and Wilms' tumor, especially for the original tumor.
According to the findings of operative cholangiography, 26 cases of congenital dilatation of biliary duct were divided into two groups; one with anomalous arrangement of pancreaticobiliary duct (anomalous group) and the other without such finding (unclarified group). Signs and symptoms in the unclarified group were those of typical choledochal cyst and obstructive cholangiopathia. On the other hand, the main symptom in the anomalous group was remittent abdominal pain, but mass and jaundice were not frequently seen. This condition was due to recurrent cholangitis caused by reflux of pancreatic juice. To minimize the risk of complications such as perforation of the bilialy duct, future development of malignancy and intrahepatic cholelithiasis, it is very important to recognize and to treat the anomalous arrangement of the pancreatico-bilialy duct in patients with choledochal cyst.
In spite of improvement of the outcome of the management in neuroblastoma and rhabdomyosarcoma, survival in patients with advanced neuroblastoma has remained unchanged. Accordingly, a treatment with high dose cyclophosphamide (CPM) was carried out in these patients before the operation. Then their operability and outcome were studied. Materials and Methods: CPM 40-100 mg/kg was preoperatively administered for ten patients with advanced neuroblastoma treated in our clinic during the past 2 years. The ages were from 10 months to 13 years old. There were 3 primary sites in the adrenal and 5 in the retroperitoneum. CPM was administered for 12 hours in one or two successive days. The operation was performed 3-4 weeks after this treatment. The same treatment with CPM was continued postoperatively. Results: The general condition of patients was markedly improved after a few days. The huge tumor decreased in size after one week in all cases, and was resected almost completely except for one patient. Pleural effusion seen in 2 patients, hemiplegia in one, and orbital echymosis in 5 before CPM treatment, disappeared completely. Toxicities of CPM treatment were leucopenia in 8 patients, alopecia in 9, and hematuria in one. They were all controlled well. Seven out of 10 patients are still arive for 4 to 14 months after operation. Three patients died 8, 9 and 10 months after operation. Mean survival time prolonged over 5 month in deceased patients. High dose CPM treatment is a useful preoperative chemotherapy for patients with advanced neuroblastoma.
Clinicopathological studies have been carried out in 28 cases with neuroblastoma and following results were obtained. 1) The prognosis in the 28 children was affected by age when the treatment was initiated, the primary site of the tumor and the stage of the disease. 2) In electron microscopic studies, neuroblastoma tissue was classified into four types, which are immature, low differentiated, differentiated and ganglionic. 3) Three types of catecholamine granules were seen. First, catecholamine granules in round shape with the diameter of about 50 nm were located mostly in nerve processes, Secondly, the granules with the diameter of about 100 nm were also round shaped, and located in the tumor cells and nerve processes. Thirdly, the granules of oval shape with different diameters were present in the cells with many round catecholamine granules. 4) Morphological true synaps was found only in the differentiated tissue from the tumor. In the undifferentiated tissue, synaps-like structure without the cluster of synaptic vesicles or desmosome was observed. Synaptic element was not found in the tissue of the immature type. 5) No significant differences in ultrastructures of neuroblastoma were recognized among the specimens from various age groups. It is considered that good prognosis of infants under 12 months of age with the tumor is more related to host factors rather than to tumor cell factors. 6) Neuroblastoma originated from extraadrenal tissue had more differentiated ultrastructure than adrenal neuroblastoma. In three cases of extraadrenal neuroblastoma giant mitochondria were observed, but in cases of adrenal neuroblastoma they were absent. 7) In one case,concentric lamination was found encircling the tumor cell. This lamination structure was identified as nerve processes. In another case glycogen granules were found in the cytoplasm and nerve process of the low differentiated type of tumor cells. 8) Electron microscopic change was found in Ithe tumor cells from neuroblastoma after conventional chemotherapy and radiation. In two patients so treated marked proliferation and tangle of neurofilaments in the cytoplasm and neuropiles were induced by vincristine.
VX-2 carcinoma cells were injected subcutaneously into the rabbit fetuses and the young rabbits. The growth of VX-2 carcinoma in rabbit fetuses was more prominent than in young rabbits, represented by the slightly larger size of tumors, higher mitotic rate, 2.2%; higher percentage of tumor cells in the tumor area, 79.8%; and the invasion into the local tissues making the entire mass fixed. In the young rabbits carcinoma was well circumscribed and abundant in the fibrous stroma. The wandering cells were prominent by found at the periphery of the tumor. The difference in the gross and microscopic findings in the fetuses and young rabbits seems to be due, at least in part, to the difference of the immunological competence of the host. It is suggested that the same phenomena, progressive growth during embryonic life and prevention of growth after birth, may occur in case with immunogenic congenital cancer.
Middle sternal cleft with incomplete partial thoracic ectopia cordis in a 30-day-old female infant was successfully repaired by partial removal of the sternal band and primary suture closure of the cleft. The patient presented with a spindle shaped cleft (4 × 2.5cm) in the sternum, through which a part of the heart and left lung protruded. On X-ray examination, the heart and mediastinum shifted to the right and mediastinal flutter with paradoxical respiration prompted surgical intervention. Skin covering the cleft is normal but hemangiomas were fouud in the face and head. Sternal cleft is classified into the complete and the incomplete cleft. The incomplete cleft is further subclassified int the upper, the lower and the middle sternal cleft according to the site of separation. Embryology, classification, treatment and prognosis of the sternal cleft and relationship between sternal cleft and ectooia cordis were briefly discussed.
A Japanese male infant, 3,280 grams, was born at 39 weeks after an uneventful pregnancy and delivery. On the fourth day, his abdomen developed distend with good general condition. Abdominal upright films showed a free air under the right side diaphragm, an air fluid level in the stomach, and the presence of bowel gas. Other laboratory studies revealed no septic findings. At laparotomy, a pin-point perforation was found at the tip of a small Meckel's Diverticulum which was about 2.0 cm long located about 20cm oral from the ileo-cecal junction. There were no pus and turbid fluid in the peritoneal cavity. A diverticulectomy was performed. Microscopically, the ectopic gastric mucosa (fundic type) was recognized at the site of perforation. Postoperative course was uneventful and he was discharged 16 days later. To our knowledge, 10 cases of perforation of Meckel's Diverticulum in the newborn period have been reported in Japanese literature.
Three cases of congenital pyloric atresia are described and a review of 100 cases reported in the literature is presented. Two of the cases were the membranous type. For these cases, excision or incision of the diaphragm with pyloroplasty was performed. The first patient was alive following the second operation of gastroduodenostomy for stenosis after excision. The second premature patient (1,380g of birth weight) died of sepsis on 58th postoperative day. The type of the third case was the fibrous cord, which required a resection of pylorus with end-to-end gastroduodenostomy. The patient did well postoperatively. With a review of literature, incidence, etiology, type of atresia, sex ratio, polyhydramios, birth weight, consanquinity, familial occurrence, symptoms, diagnosis, treatment and mortality rate were discussed. The treatment should be surgical and the operative procedure is variable due to the type of atresia. Excision or incision of the diaphragm with pyloroplasty and gastroduodenostomy is the procedure of choice. Gastrojejunostomy should be avoided because of its high mortality rate.
Gastric volvulus was surgically corrected in three patients with asplenic syndrome associaed with severe cardiac anomalies. One patient whose stomach was necrotic, died postoperatively of intraabdominal abcess. The other two patients have been well. Four intraabdominal common abnomalities were found in three asplenic patients; 1, lacking of gastro-diaphragmatic ligaments, 2, lacking of gastro-splenic ligaments, 3, loose fixation of pylorus, 4, close location of pylorus to cardia. Cardiac surgeons and pediatricians who treat asplenic syndrome should pay attention to extra cardiac abnomalities and should avoid undue delay of emergency operation for gastric volvulus.
A 31/(2) years old male was admitted with bilateral Wilms' tumor. A nephrectomy was carried out for the left kidney in August 1977. The right kidney was irradiated with 1,300 rads and the whole abdomen with 600 rads postoperatively. Simultaneously, a proper dose of actinomicin D and vincristine was given. However, the tumor in the right kidney continuously grew after a transient reduction. In January 1979, a right nephrectomy was done with an intraoperative irradiation to the right renal fossa, the metastatic lesions of the liver, and the para-aortic lymph node. By chronic hemodialysis, uremia was well controlled. But the metastatic liver tumor continued to enlarge, and the patient became gradually emaciated and died on May 17, 1979. Autopsy showed numerous hepatic, para-aortic lymph node, and bilateral lung metastases. Histo-pathological examination of the para-aortic lymph node suggested the effectiveness of intraoperative irradiation. In this paper, 40 cases of bilateral Wilms' tumor were reviewed from Japanease literature. Four survival cases for more than two years after surgery were included in these 40 cases. All survival cases received aggressive surgical treatment. Three of these cases underwent combined unilateral nephrectomy and partial resection of the opposite kidney, and one had bilateral heminephrectomy.
Follow-up studies were carried out on 46 patients with congenital hypertrophic stenosis 1 to 12 years after the Ramstedt operation. Their chief complaints were loss of appetite, vomiting and abdominal pain in this order of frequency. Barium gastrointestinal examination showed abnormal findings in 16 patients (39%) and chief findings were deformities of duodenal bulb and pylorus, gastric ulcer and duodenal ulcer. About a half of the patients 1 to 4 years after operation showed the tendency of hypergastrinemia.
A statistical investigation on external inguinal hernia was performed on 55,054 children, who visited the clinic for "three-year-old health check" in Aomori Prefecture. The oval-all incidence of external inguinal hernia was 1.66 per cent (915/55,054) and a marked familial predisposition was noted. Seventy-four per cent of the cases occured in male. Of the 624 hernias studied, 33 per cent had been noted before the age of three months. Fourty-four per cent of the cases were right-sided, 33 per cent left-sided, and 23 per cent bilateral. At the time of the investigation, 89 per cent of the children with hernia already undergone surgical repair, and most of the repairs were done soon after the recognition. This fact may accounts for a relatively high incidence (12.2%) of contralateral hernia following operation. Spontaneous cure was found in 6.3 per cent and was observed more often in girls, and the cure rate was higher in cases in whom the lapse of time from recognition of the disease to judgement of cure was shorter. The occurrence of irreducible hernial condition was experienced in 46 per cent, and more than a half of those had occured within three months after recognition of the hernia.
Incidence of congenital biliary atresia in Mie Prefecture during the period from 1974 through 1978 was 12 cases in 124,756 live births, which means a rate of 1.01±0.38 per 10,000 live births. There was no statistical significance comparing with incidence of nation wide survey in Japan or tne survey of Japanese and Caucasians born in Hawaii.
Mental and emotional disturbance which is occasionally observed in children after operation is concerned greatly with preoperative psychogenic anxieties. Furthermore, it seems closely that the intramuscular administration may-be the cause of muscle contractures. During the period from July 1977 to May 1979, 154 children under 8 years of age underwent oral administration of atropine, hydroxyzine pamoate, and diazepam or triclofos, 75min. prior to operation instead of conventional parenteral premedication of these agents. Fourty children, as the control group, underwent intramuscular administration of atropine, hydroxyzine pamoate, and pethilorfan. The result of both groups with oral and parenteral premedication was compared. The results are summarized as followed: The oral administration method has an advantage by obtaining the fine preoperative sedative effect and the good postmedicative effect.
A clinical follow-up study in five patients with hiatal hernia who were treated by Nissen fundoplication is reported. There was no sign of recurrence by clinical and radiological studies in all five patients. None of these patients has developed gasbloat syndrome. Growth and development after operation was within normal range. It should be noted that one patients is able to burp freely. Manometric study showed good LES (Lower-Esophageal Shincter) function. LESP (LES pressure) and LES-length were within normal range. Three of these five patients showed swallowing relaxation clearly. The postoperative investigation of LES function including manometric study revealed satisfactory results in this series.
A new classification of congenital dilatation of the biliary ducts was proposed by the Working Group of the Japanese Society of Pediatric Surgeons in 1977, and 497 cases collected from 21 Institutes in Japan were analyzed according to the type of dilatation, surgical procedure and follow-up results. A typical cystic dilatation of the choledochus belonged to be 51% and fusiform dilatation to be 10.6% without any other dilatation, but concomitant dilatation of choledochus and main hapatic ducts was seen in 28.5% of all cases. Primary excision of the cyst was carried out with Roux-en-Y anastomosis between the hapatic duct and a limb of jejunum in 219 cases and 80% of the operated cases showed excellent long-term results. Cystojejunostomy showed also favorable results in contrast to less satisfactory results of cystoduodenostomy which showed a significant incidence of later complications.