Preliminary results from NWTS-3 currently suggest that (1) a 10 weeks course of AMD and VCR for "favorable histology" Stage I Wilms' tumor is as effective as a 6 months course, (2) flank irradiation is not statistically advantageous for "favorable histology" Stage II tumors, (3) ADR does not significantly improve outcome for "favorable histology" Stage II or III tumors, (4) Stage I anaplastic Wilms' tumors probably do not require radiotherapy or intensified chemotherapy, and (5) the prognosis for Stage I CCSK is markedly improved by radiotherapy and intensified chemotherapy. A new classification of nephroblastomatosis is briefly introduced, that has both practical and theoretical importance with relation to Wilms' tumor and other childhood renal tumors.
For investigation of the mechanism of postoperative defecatory disturbance, anorectal manometry using an infused open-tip method was carried out in 38 patients who have been operated on for Hirschspring's disease with Swenson's procedure and Z-shaped anastomosis. The same examinatoin was performed in 10 children with chronic constipation and 15 normal children as controls. In patients with Z-shaped anastomosis, pressure difference between the rectum and the anal canal, and rectal compliance were lower than those with Swenson's procedure. In the group of patients with postoperative normal continence, resting pressure profile of the anorectum, pressure volume curve of the rectum, and rectal compliance were normal. Rectoanal reflex was observed in 58% patients in this group. In the group with postoperative constipation, pressure difference between the rectum and the anal canal was normal, whereas decreased basal rythmic contruction of the anal canal, slow rise in pressure volume curve of the rectum, high rectal compliances, and dilatation of the rectum were observed. Rectoanal reflex was absent in this group. In the group with postoperative incontinence, low pressure difference between the rectum and anal canal, abrupt rise in pressure volume curve of the rectum, and low rectal compliance were observed. Rectoanal reflex was observed in 14% of patients in this group. Pressure difference between the rectum and the anal canal and rectal compliance were found to correlate well with the postoperative continenc. It was suggested that the causes of incontinence are due to disturbance of the rectal reservoir and anal sphincteric functions.
Among twelve patients with biliary atresia (BA) who have undergone a hepatic porto-jejunostomy (or porto-gastrostomy) since 1970, pathological fracture involving long bones (Clavicle, Radius, Ulna and Femur) was observed in six at the age from 2 years to 4 years and 7 months. Therefore, we measured serum level of 25-hydroxyvitamin D (25-OH-D), calcitonin, parathyroid hormone (PTH) and radiological changes in bone in eleven patients. Since bile juice is necessary for vitamin D absorption, we classified the postoperative BA patients into three groups; 1) internal jejunostomy, 2) oral-sided and 3) anal-sided external jejunostomy. The group of anal-sided external jejunostomy showed a large amount of bile loss (10.8±3.8ml/kg/day) from jejunostomy, and significantly low serum 25-OH-D level less than 5.0ng/ml. On the other hand, a small amount of bile loss (4.1±2.7ml/kg/day) and relative decrease in serum 25-OH-D level (17.8±13.0ng/ml) were recognized in the group of oral-sided external jejunostomy. However, serum levels of calcitonin and PTH were almost within normal range with wide deviation, and no significant relation to the operative methods was recognized. Since radiological examination of long bones revealed no rachitic changes except relatively thin cortex and decrease in bone density, pathogenesis of postoperative fracture in BA patients might be due to not only vitamin D defficiency, but also growth disturbance of osteoid tissues of the other causes.
Intraluminal pressure of the lower esophageal sphincter (LES) of dogs was measured by manometric study in order to confirm the role of vagus on relaxation of LES during swallowing. Relaxation of LES occurred during swallowing in the group in which the esophagus was severed but the vagus was preserved. However, relaxation was not seen in the group in which both esophagus and vagus were severed. Relaxation of LES was also seen in the groups in which the vagus was severed and the esophagus was reanstomosed after severance in various manners, such as in all layers, in muscular layer or in mucosal layer. These results mean that relaxation of LES during swallowing is mediated via the vagus. However, this phenomenon could be also mediated through the esophageal wall.
Twenty-six patients with congenital diaphragmatic hernia who developed symptoms within the first 24 hours were reviewed. They were classified into the three groups according to the results of the pre-and post-operative blood gas analyses. Group 1 (9 patients): The preoperative AaDO_2 was lower than 500 mmHg. The post-operative course had been uneventful with no mortality. One patient developed persistent fetal circulation (PFC) which had been treated successfully. Group 2 (8 patients): The preoperative AaDO_2 was higher than 500 mmHg and the post-operative PaO_2 elevated higher than 200 mmHg shortly after operation. Half of them, however, developed PFC with three death. They had had so-call honeymoon period when high PaO_2 was maintained during several hours after the operation, and slipped into PFC with even minimum stimuli. Intensive cares including mechanical ventilation and administration of α-blockers were effective in these PFC patients. Group 3 (7 patients): The preoperative AaDO_2 was as high as that of the group 2, but there was no elevation in PaO_2 postoperatively. Neither respiratory care, nor pharmacologic treatment was effective in these patients. Hypoxia seen in these patients may be caused by possible hypoplastic lungs. There was no survivor in this group. In conclusion, the above mentioned classification may be useful to anticipate the outcome of these patients and to select the best therapeutic techniques which are available at the present moment.
In six cases of congenital dilatation of bile ducts, hepaticoduodenostomies with end to side anastomosis were performed after resection of dilated choledochus. The age distribution of the patients undergoing the operation ranged from 2 months to 14 years old. The period postoperative follow up study ranged from 9 months to 7 years and 2 months. Postoperative course was uneventful in all cases and all showed good flow of bile and none of the patients developing ascending cholangitis. The advantages of this procedure are summarized as follows; 1) This method is suitable for the reconstruction of congenital dilation of bile ducts, since mixing of gastric juice is more physiological than with the other common method of reconstruction. 2) The time necessary for operation is relatively short because only a single anastomosis to the duodenum is performed. 3) The large diameter of resected bile duct stoma helps to prevent cholangitis because of perfect bile drainage. 4) Since only the restricted anatomical areas are involved, incidence of postoperative complications such as adhesive ileus are much less than those of other complicated methods.
The patient, a 5-year and 6-month old girl, who had a large neuroblastoma (stage III) originating from 1-lumbar sympathetic ganglion with the residual mass attached to the inferior mesenteric artery and abdominal aorta, was treated with alpha-interferon (IFN), 1×10^6 IU×2/week, intramuscularly in combination with chemotherapy. A total of five courses of intensive chemotherapy [Adriamycin+Vincristine+Cyclophosphamide (CPM)] were given, and thereafter either CPM, intravenously, or CPM, orally, has been singly administered with IFN. During the 31 postoperative months, no metastasis or local recurrence of a primary tumor has been observed. IFN administration has been continued and the total dosage is now 2.73×10^8 IU. Adverse effects were not observed except for mild pyrexia which disappeared after the initial several months. She has shown normal physical and mental development. Our results showed that the patient of stage III neuroblastoma with a large mass and regional lymph node metastases can be expected to be responsive to IFN treatment and chemotherapy after removal of most of the primary tumor and intensive chemotherapies without acute and chronic serious adverse effects. We further discussed the general effectiveness of IFN on patients with neuroblastoma. This study indicates that the combination of IFN and chemotherapy is aafe and effective regimen.
Palliative operations were performed on seven patients with pulmonary atresia which was associated with intact ventricular septum (2), large ventricular septal defect (3), and asplenia syndrome (2). Although the infants with intact ventricular septum survived the Brock operation and symptomatically improved remarkably, the right ventricular pressure still remained high for approximately one year postoperatively requiring corrective surgery in the near future in both cases. In the group of pulmonary atresia with large ventricular septal defect and large aortopulmonary collateral arteries providing all of the pulmonary blood flow, the operative methods should be determined by the precise preoperative findings of the true pulmonary arteries. These arteries should be examined with selective angiography of all the aortopulmonay collateral arteries and by wedge angiography of the pulmonary vein. Operative results for pulmonary atresia with asplenia syndrome were unsatisfactory due to association with total anomalous pulmonary venous drainage (TAPVD). In such cases, intraoperative interventions are necessary to verify the association of TAPVD. Anastomosis between the common pulmonary vein and the common atrium should be performed concomitantly if the association with TAPVD is confirmed.
Thirty-eight children with lymphangioma were treated at the second Department of Surgery, Kyoto University Hospital, from May 1976 to March 1983. In only 5 out of 13 cases, a complete excision of the lesion was carried out. Bleomycin (BLM) was injected into the cavity of lymphangioma in 34 patients primarily or after surgical treatment. In 33 cases, BLM was used in a form of sphere-in-oil emulsion developed by Hashida in 1976. Injection of 0.6 mg BLM emulsion per kg body weight was repeated at intervals of 4 to 6 weeks. In 27 cases (81%) lymphangioma regressed and in 13 cases (39%) it almost disappeared. Cystic hygroma was particularly sensitive to this therapy. In responding cases, the total injected dose was 0.6 to 4 mg/kg body weight. BLM emulsion was not effective in 6 cavernous lymphangiomas in extremities (4) and in the chest wall (2). Fever was the most common side effect (53%). Pulmonary fibrosis was not observed. The most serious complication was dyspnea which was seen in a 7 month old girl due to transient swelling of cervicomediastinal lymphangioma after BLM emulsion injection. There was no re-growing of the residual induration after BLM emulsion therapy.
In order to know the proper site of hepatic portal dissection in biliary atresia, the site of hepatic ducts in 80 cases from 4 to 85 years of age without biliary duct disease were studied ultrasonographycally. The relationship between biliary ducts and portal veins was studied with real-time sonography. The course of the 1st branch of the right hepatic duct could be identified in 68 out of 80 and the course of the 1st branch of the left hepatic duct in 50. The right hepatic duct was located ventral to the main branch of right portal vein in 54 (80%), cranial in 9 (14%), and dorsal in 5 (7%). It was located ventral to the 1st branch of right portal vein in 44 (65%), cranial in 19 (28%), and dorsal in 5 (7%). The left hepatic duct was located ventral to the main branch of left portal vein in 40 (80%), and cranial in 10 (20%). It was located cranial to the umbilical portion of left portal vein in all cases. Our studies indicate that dissection along dorsal aspect of the 1st branch of right portal vein and cranial to the umbilical portion of left portal vein would be a proper access to the main intrahepatic bile ducts.
A case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS), complicated with gastric perforation is presented. This was the only case of gastric perforation in 28 MMIHS cases ever reported in the world literature. The etiology of the perforation was thought to be a large defect in the musculature of the stomach, complicated with distal obstruction due to hypoperistalsis. A review of the literature revealed that there have been many common characteristcs; flaccid abdomen, megacystis, malrotation, undescended testis, and so on; descrived in MMIHS and prune belly syndrome (PBS). In view of so much similarity between these two syndromes, we suggest that MMIHS might be a female manifestation and PBS being a male type expression of the same underlying process.
Two 8-year-old boys having hepatocellular carcinoma (liver-cell carcinoma, adult type) associated with hepatitis B surface antigen (HBs-Ag) positive were reported and discussed with 5 cases in Japan riviewed in the literature. The first patient was HBs-Ag positive in serological examination. He was suspected to be a case of maternal (vertical) transmission, because he was pointed out as suffering from hepatomegaly at the age of three months and his mother was an HBs-Ag positive carrier. The second case had not undergone serological examination but histologically Orsein-stainpositive cells were present in non-neoplastic liver tissues at autopsy. He also had liver cirrhosis type B. HB virus infection, because of maternal transmission or horizontal tansmission in children less than 4 years of age, results in HBs-Ag positive carrier, chronic hepatitis and liver cirrhosis type B. These hepatocellular carcinomas associated with HBs-Ag positive demonstrate that HB virus infection will cause hepatocellular carcinoma not only in children as well.
Extremely immature infants of triplets with respiratory distress syndrome were experienced at Juntendo Izu Nagaoka Hospital. Of these three, two had persistant ducuts arteriosus (PDA). One of these two received indomethacin and the other underwent ligation of PDA respectively. Body weight, duration of respiratory support and cardiothoracic ratio were compared among three. All patients were cured without any difference in growth. Indication of either medical or surgical treatment for PDA in premature infants, should be determined by the condition for each individual patient.
A 9-year-old boy who had a soft tissue tumor of the scalp and the multiple polyposis of the colon was reported. The soft tissue mass was found at birth and excised at age 11 months. The pathological diagnosis is was fibromatosis, desmoid type. The colonic polyp was biopsied and diagnosed as adenomatous polyp. This case then was classified as of Gardner's syndrome, incomplete type. His father had familial polyposis, diffusely studded type. It may be that this patient develops the same lesion in near future. The total colectomy combined with Soave's procedure would be a treatment of choice in this paticular case. The 45 cases under the age of 20 years in Engligh literature since 1951 were reviewed. There are 21 cases of the complete Gardner type and 24 imcomplete. The total colectomy combined with ileoproctostomy and subtotal colectomy combined with ileoproctostomy were done in the majority of the cases.
A two year old girl first presented with pancreatitis, subsequently who was found to have a slightly dilated common bile duct. Operative cholangiopancreaticography via gallbladder demonstrated an elongated common channel. Biopsies of the pancreas showed a definite chronic pancreatits. Pancreatitis seen in this patient was supposed to be caused by the bile reflux into the pancreatic duct through the long common channel. Division of the common bile duct and hepaticojejunostomy (Roux-Y) performed, with a satisfactory result. Our new classification of anomalous choledocho-pancreatic junction was proposed as follows Type I consists of cases with elongated common channel. Type II consists of cases with other anomalous ductal junction. Furthermore, Type I was divided into three subtypes. Subtype Ia: cystic dilatation of the bile duct. Subtype Ib: fusiform dilatation of the bile duct. Subtype Ic: no dilatation of the bile duct.
We report two cases of SLE, which developed acute abdomen and perforation of the small intestine. A 15-year-old girl presented with gastrointestinal hemorrhage, for which an emergency operation was performed. Jejunal perforation andileocecal ulcer were found, that required partial jejunal and ileocecal resection. In both sections, the pathological finding was ulcer with angitis. Angitis was considered to be the main cause of the ulcer. An eight-year-old girl, presented with symptoms of pan peritonitis. Roentgenography of the abdominal region revealed pneumatosis intestinalis. An emergency operation was performed for possible necrotizing enterocolitis (NEC). Multiple perforations and pneumatosis intestinalis were found in the upper jejunum. Histopathologically, the findings were pneumatosis and bacterial colonies in the intestinal wall, which resembled the pathological picture of necrotizing enterocolitis in neonates. There was no finding of angitis. It was considered that intestinal infection developing under the condition of very much decreased resistance against infection due to decreased leukocytes, decreased complement titer, and steroid therapy, had created the pathological picture of NEC. Since SLE is associated with various systemic organ disturbances, and multiple organ failure (MOF) was liable to develop postoperatively. The first case died of MOF, and the 2nd case, though she developed MOF once postoperatively, survive by intensive care. Thorough postoperative care is believed to be necessary in case of SLE when operation is performed for an intestinal complication.
Transverse testicular ectopia is very rare. Recently, a 9-year-old boy was operated on for left undescended testis. At age of five, he underwent an exploration of the right side undescended testis. The right testis was not found. Monoorchism was the diagnosis at that time. At this operation, two testes were found in the left inguinal canal. The left testis was snugly placed in the right scrotum through the scrotal septal window. The left testis was placed in the scrotum in usual fashion.
2 cases of infantile hemangioendothelioma of the liver were presented. The first case, a 14-day-old female newborn, presented with an abdominal tumor and a dermal hemangioma on her left shoulder. The abdominal tumor was resected by the left lateral segmentectomy of the liver. The histological examination revealed an infantile hemangioendothelioma of the liver. The second case, a 1 year and 7 months old boy, presented with an abdominal tumor. The tumor was resected by the left lateral segmentectomy of the liver. The histological examination revealed an infantile hemangioendothelioma of the liver. Various kinds of managements for the infantile hemangioendothelioma were discussed. We treated 2 cases of infantile hemangioendothelioma of the liver by hepatectomy.
A 4-month-old male infant was referred to Kanazawa Medical University Hospital because of a history of cough, dyspnea and difficulty of feeding since age 1 month. He was never cyanotic, nor febrile. Inspiratory stridor and dysphagia were present and were accentuated by excitement. Physical examination disclosed a 1.5cm×1.5cm, smooth, cystic mass under the base of the tongue which of the neck projected toward oropharynx. Lateral view of X-ray film showed a mass which located at the base of tongue overlying the epiglottis. Ultrasonogram confirmed a cystic mass. Thyroid scan showed the thyroid gland in the normal position. Marsupialization of the mass was performed. Histologic examination revealed a cyst the wall of which consisted of the connective tissue with mild chronic inflammation. The epithelial lining was lost. Postoperative course was uneventful. Two months later, the child remains asymptomatic without recurrence. The thyroglossal duct cyst offers the most logical explanation for the occurence of this lingual cyst.
Chondroid hamartoma of the lung occurs most often in persons after the age of 40, but quite rare among children. This paper reports an 11-year-old boy with pulmonany hamartoma with typical loburated mass in right upper lobe. The patient was asymptomatic, but a well-demarcated density with popcorn shaped carcification was pointed on chest X-ray films. An upper lobectomy was carried out to remove the lesion. His postoperative course was uneventful. Six months postoperatively, the child was symptom-free and has been developing normally.