The prognosis of the advanced neuroblastoma still remains very poor in spite of the recent advances in the therapies of children's cancers. For the purpose of improving the prognosis, we organized a chemotherapeutic group study to treat patients with Stage III and IV (exceptIV-S) neuroblastoma in Kyushu area in Japan, which started in March 1982. The protocol was divided into A and B, and was scheduled as follows : Protocol-A ; 3courses of cyc lophosphamide (CPA,40 mg/kg×2 days) was given every 3 weeks and then 3courses of cisplatin (CDDP,20 mg/m^2×5 days)+VM-26 (100mg/m^2,after 48 hrs) were sequentially administered, protocol-B ; the same dose of CPA and the same doses CDDP+VM-26 were given alternately every 3 weeks. Until March, 1984, 19 cases were registered, among whom 15 were evaluated. The therapeutic results are as follows : Protocol-A ; 3 of 6 cases are alive, Protocol-B ; all of 6 cases are alive (5 more than 12 months). Three were recurrent, all of whom died. These preliminary results suggested that the combined high dose chemotherapy such as Protocol-B using CPA, CDDP and VM-26 were much more effective for the advanced neuroblastema. The side effects especially of CDDP are also discussed.
A new ascites-type cell, NAs-1, was obtained in culture from mouse neuroblastoma C1300. The cells were growing anchorage independently in F-10 medium containing 10% FCS, and retained the ability of growing and producing ascites fluid when intraperitonealy injected into mice. A morphology of NAs-1 growing in culture medium was small and round without any neuronal processes. Karyotype analysis showed a homogeneous chromosome number, 40, with a marker chromosome [t(13: 16)] and a minichromosome. Catecholamines, norepinephrine and dopamine, were found in the cell extracts and the contents of dopamine was particularly high. Neuron specific enolase (γ-subunit) was also detected. The treatment of cells by dibutyryl cyclic AMP, prostaglandin E_1, or BL191 (phosphodiesterase inhibitor) induced the biochemical differentiation in terms of catecholamine and cyclic AMP contents, but failed to promote typical morphological differentiation including the extention of process or the significant pro-motion of adherence onto the flask surface. 15 day after intraperitoneal injection, most of NAs-1 invaded along peritoneum and brougt about infiltrative metastasis in varied organs such as liver, kidney, lung, and esophagus.
The excretion of antibiotics in bile was studied in 5 patients with biliary atresia. Three of them were Kasai type I atresia and received cholecystostomy for initial bile drainage prior to the radical operation. Remaining 2 cases were received hepatic porto-jejunostomy by Roux-en-Y fashion with gastrojejunostomy. Consecutive excretion of antibiotics in bile was studied on 1 case with cholecystostomy at the early postoperative period. Antibiotics used for this study were followings; CPZ, LMOX, CMZ, AMK, and GM. All of these antibiotics were administered by intravenous one shot injection. Total amount of bile flow was determined using the catheter placed into the cholecystostomy or jejunostomy. The concentration of antibiotics in bile was measured using bioassay method. The results of the presented studies were followings; 1) The maximal concentration of CEPs and AGs in bile was higher than MIC to the causative organisms of the postoperative ascending cholangitis, whereas the clinical efficiency of antibiotics was influenced by the organisms in bile or intestine. 2) The excretion of antibiotics in bile was not influenced by the re-feeding of bile. 3) The result from consecutive pharmacokinetic study in early postoperative period indicated a relationship in excretion rate of antibiotics between bile and urine.
Biofeedback conditioning is a new method for the treatment of fecal soiling. The principle of the training is as follows: a) rectal sensitivity is first treated with the stimulation induced by rectal distension. b) the anorectal contraction in response to rectal distension is directly showed on the writing instrument. Over a 3-year period (1981-1984), 4 children with fecal soiling were treated with biofeed-back conditioning using a manometric technique. The cause of incontinence was encopresis. The age ranged from 5 to 10 years (average 7.0 years). The follow-up period ranged from 5 to 17 months (average 9. 0 months). 4 patients achived a very good clinical response. Psychogenic incontinence responded to this method, and both manometric rectal sensitivity and anorectal contraction improved significantly. This training influence psychological aspect successfully as well.
Electrical activity of the external sphincter in fifteen patients with anorectal malformations (7 patients with the high type anomaly, 4 with the intermediate type, and 4 with the low type), aged 3 to 14, was investigated. The results were compared with those of 3 normal subjects, aged 6 to 10. The electrical activity of the external sphincter in the patients with high type anomaly was found to be disturbed from the points of tonic activity, inflation reflex, and activity during further rectal inflation. The patients with intermediate type or those with the low type, how-ever, showed good preserved electrical activity of the external sphincter as well as normal control did. The phasic activity of EMG was found in the 3 types of anorectal malformations. This result indicated that patients with the high type anomaly could gain good voluntary control of defecation by having a training of voluntary contraction.
Thirteen cases with sacrococcygeal teratoma were treated during the past 19 years. Ten cases with benign teratoma were all permanently cured. Among 3 cases with malignant teratoma, 2 died of local recurrence and lung metastasis, respectively. Of the remaining 1 patient whose operative staging was stage I, no further treatments were afforded. At 2 months postoperatively, however, her serum AFP showed abnormally high value and at 3 months postoperatively, a left lung metastases have developed. Subsequently, pulmonary wedge resection and chemotherapy were administered. In spite of these treatments her AFP values are keeping still high levels at present. These clinical experience may strongly suggest the importance of routine use of postoperative cancer chemotherapy even for the stage I disease of yolk sac carcinoma.
Endoscopic retrograde cholangiopancreatography (ERCP) was performed in 19 cases of our series. The subjects consisted of three cases with the ages of under three years who required the general anesthesia, nine cases with ages ranged from four to nine and seven cases from 10 to 15 years of age. The success rate of ERP (pancreatography) was 100% but was only 70% in ERC (cholangiography). The final diagnosis made by ERCP or for which investigated further by the method were ten cases of congenital biliary dilatation, five cases of unknown causes of abdominal pain and/or vomiting, and one case of pancreatitis, biliary atresia, biliary hypoplasia and hepatitis respectively. Our study confirmed that there is no doubt that ERCP is a valuable procedure in the investigation for various biliary and pancreatic lesions in children, even though there are still many technical problems which should be solved particularly in young infant.
Total Parenteral Nutrition (TPN) has provided a great progress in neonatal surgery, however catheter-related sepsis still remains unsolved. Of 69 surgical neonates receiving TPN from November 1982 to October 1983, 10 cases were diagnosed as a catheter-related sepsis. Catheter-related sepsis was defined as an episode of sepsis resolved by removal of cather followed by associated therapies. Serial platelet count, leukocyte count, CRP and coagulation studies (PT, PTT, Fibrinogen) were performed before and after removal of catheter. Thrombocytopenia less than 100,000/mm^3 was found in 7 cases and sudden fall of platelet count was found in another 3 cases. Leukocyte count and coagulation studies did not correlate with platelet count. Thrombocytopenia, particulary sudden fall of platelet count is considered to be useful for the early identification of catheter-related sepsis in neonates.
A three-month-old well nutritioned girl, who suffered from cough and stridor, was admitted to our hospital. Chest roentgenogram, computed tomography, echocardiography showed a large mediastinal mass attached to the heart. Operation was contemplated on account of relief of progressive respiratory distress. A right anterolateral thoracotomy was performed through the fifth intercostal space. The pericardium was thickend and distended. About 20ml of straw-colored fluid was obtained from the pericardial sac at the time of opening the pericardium. The tumor, 5.5×4.5×5.0cm in size, was firmly attached to right atrium and ascending aorta. It was completely removed successfully and histologically diagnosed as immature teratoma (Ti). The postoperative course was uneventful. As for its anatomical position, intrapericardial teratoma is rare in frequency, but it occasionaly causes death due to cardiac tamponade. The first reported case of this disease was described by Joel in 1890. Since then, 55 cases of this disease have been reported in the literature during the period 1890-1983. In Japan Kume reported the first case in adult in 1979. Our case seems to be the first reported case in infancy. Age and sex, clinical findings, diagnotic examinations, treatments and histology of intrapericardial teratoma were discussed in already reported 55 cases addeding our one case.
Adrenocortical carcinoma is rare in children. In this paper, two pediatric patients of the adrenocortical carcinoma were presented. Both patients showed Cushing's syndrome and adrenogenital syndrome. The first case was a girl, age of 3, showing a moon face, precocious puberty, hypertension and hypokalemia. She had an adrenocortical carcinoma of the left adrenal gland, which was successfully resected. The clinical stage was III. She died of the recurrence seven months after the operation. The second case was a boy, age of 10, showing a moon face, precocious puberty and hypertension. He had a right adrenal tumor, which was unresectable because of the local invasion and the distant metastases. The clinical stage was IV. The histological examination revealed an adrenocortical carcinoma. He died of DIG nine days after the operation.
We experienced the young patient (15 year old) who had had obstructive jaundice, and the endscopic retrograde cholangiogram showed a long stenosis of the distal biliary tract. This patient had been treated for chronic renal failure for 10 years at the department of medicine. Frequent microscopic examination of bile showed Papanicoloau class II-IIIb. As the benign tumor like adenoma was most suspected, cholecysto-duodenostomy was performed. However, the microscopic findings of the tumor showed adenocarcinoma and tubular adenoma in the same specimen. As the renal function was decompensated after the first operation, it took 6 months untill the radical operation (pancreato-duodenectomy, Child's operation). On the macroscopic and microscopic findings of the resected tumor, the invasion extended beyond the choledocal wall but not remarkable in pancreatic stroma. The metastatic lymph node was not confirmed. Postoperative diagnosis; Bi, circ., c, 1.2×0.9×0.9, Hinf_0, G_0, Panc_1, D_0, V_0, P_0, N (-), stage I. The developmental process of the tumor was discussed that the adenocarcinoma had been arised from adenoma. This case was very interesting from clinical and pathological views.
We reported a newborn case of vaginal polyp with imperforate anus. After histological diagnosis of the vaginal polyp by biopsy the polyp was removed at second day. The tumor was botryoid shape and dark-red so that it diagnosed as sarcoma botryoides. The size of the polyp was 1.7×3.2×1.5cm and histological findings showed hamartomatous lesions which consisted of squamous epithelium, stromal fibrous tissue, hemangiomatous portions, scattered young muscle fibers with striations and so on. For six months after resection the baby has been well without recurrence.
A case of biliary atresia with preduodenal portal vein was reported. Laparotomy revealed a symmetric liver in association with the fibrosis (F_1) but the patient had not polysplenia, malrotation and other anomalies. At present, two years after hepatic portoenterostomy, the patient is free of jaundice. Pathoembryologically, it is supposed that biliary atresia with preduodenal portal vein is caused by a developmental anomaly in contrast to that of the usual type of congenital biliary atresia. The prognosis of this association has been poor because anatomical variations at the porta hepatis made the portoenterostomy difficult in addition to the presence of severe congenital hepatic fibrosis. Embryology of preduodenal portal vein and anatomy of the porta hepatis in congenital biliary atresia with preduodenal portal vein were briefly discussed.
A 6-day-old neonate with cyanosis and tachypnea was found to have total anomalous pulmonary venous connection below diaphragm by two-dimensional echocardiography. Emergency operation was performed successfully with cardiopulmonary bypass at moderate hypothermia. Postoperatively continuous peritoneal dialysis was used for acute renal failure and tracheal intubation was required for 10 days. The patient is doing well and showing normal physical and mental development at 7 months of age. Emergency operation should be performed in patients with infradiaphragmatic total anomalous pulmonary venous connection.