Radioisotopic splenoportography (RI-splenoportography) was performed in 11 children (6 patients with biliary atresia, 3 with extrahepatic portal obstruction, 1 with congenital hepatic fibrosis and 1 with pediatric liver cirrhosis). Collateral pathways were found in 9 patients by RI-imaging, of whom 6 patients had ascending pathways alone, 1 had both ascending and descending pathways, and 2 had only descending pathways. Esophageal varices were found endoscopically in all 7 patients with ascending collateral pathways, but were not found in 2 patients with the descending collateral pathways alone or in 2 patients without collateral pathway images. Endoscopic injection sclerotherapy (EST) was performed in one patient with biliary atresia who had had episodes of rupture of esophageal varices. Ascending collateral pathways were recognized by RI-splenoportography before EST, but they disappeared completely after three sessions of EST. In two patients with extrahepatic portal obstruction and a patient with congenital hepatic fibrosis who underwent meso-caval shunt operations, shunt patency and disappearance of ascending collateral pathways were proved clearly by RI-splenoportography after the operations. RI-splenoportography is very useful for investigation of portal venous collateral circulation in portal hypertension or for confirming patency of portosystemic shunts.
The cause of constriction of the aganglionic intestine in Hirschsprung's disease still remains obscure and is controversial. An immunohistochemical approach was taken to clarify the reason of the abnormal constriction. The neuromuscular junctions and the synapses of normal (n = 4 ) and aganglionic intestines (n = 4 ) were stained with monoclonal antibody 171 B5 which is able to identify the synaptic vesicle-specific 38,000 Dalton protein. The distribution and quantity of the synaptic formations of the enteric nervous system in both the intestines of Hirschsprung's disease and the human normal intestine were obtained. Particular attention was paid to the synapses of the muscular tunicae and the nerve terminals of abnormally proliferated extrinsic nerve fibers in the aganglionic intestine. In normal intestines, many synapses which seemed to be neuromuscular junctions were identified as fluorescent spots in the muscular tunicae. They showed a regular and dense distribution that is well recognizable. A lot of synapses were seen densely gathered around the ganglion cells in the areas of the Auerbach and Meissner plexi. However, in the aganglionic intestion of Hirschsprung's disease, the synapses and the neuromuscular junctions were very coarsely distributed in the muscular tunicae and were seen as faint fluorescent spots. Some synapses were localized massively only around or near the abnormally innervated extrinsic nerve fiber bundies between the inner circular and the outer longitudinal muscle layers. Thus, the smooth muscles of the aganglionic segment seemed to be almost completely denervated. These findings indicate that the lack of the innervation of intestinal may be the main reason for intestinal constriction in Hirschsprung's disease.
Congenital biliary dilatation is recognized as one of the anomalies of the bile duct. Our study was aimed at clarifying whether or not the unusual junction patterns of proximal (main) hepatic bile ducts are particularly common in this disease. The junction patterns of the proximal hepatic ducts were evaluated on 22 cases of congenital biliary dilatation. Unusual patterns were noted in 27.5%. This proportion is very similar to that seen in other diseases in adults. The unusual patterns appear more frequently in the fusiform type than in the cystic type cases of congenital biliary dilatation. There was one case in which the right hepatic duct joined directly with the cystic duct. Such unusual types do not appear so frequently in congenital biliary dilatation, but it is necessary to take care with the junction patterns of the proximal hepatic ducts during surgery.
Taking note of the correlation between the onset and stresses including hypoxia, immaturity of gastric defense factors and environmental changes in the perinatal stage, we experimentally investigated rupture of the stomach. The results were as follows : 1) The body of the stomach tends to be affected by the stress of water immersion and by hypoxic conditions than does the antrum. 2) Acute gastric mucosal lesions were aggravated parti-cularly in the presence of hypofunction of the sympathe-tic nervous system under the stresses applied. 3) The rats under hypoxic conditions developed gastric mucosal lesions earlier than did those stresssed by water immersion, and the influence of hypoxic conditions was more than that of stress by water immersion. 4) These results suggest that a hypoxic condition may be one of the causes of neonatal gastric rupture, as well as perinatal stress and hypofunction of the sympathetic nervous system.
Over the last 5 years, 245 children, ranging in age from 3Y7M to 18Y, were scheduled for continuous epidural block for alleviating perioperative pain, and placement of an epidural catheter into the epidural space was successful in 237 cases. Complications occurred in two cases. One was attributable to an overdose of local anesthetic during surgery, resulting in a convulsuion in the recovery room. The other case was an accidental intravascular cannulation into the perivertebral venous plexus, which was clearly demonstrated by contrast medium injected through the catheter. Thirty-nine patients under-going surgery for vesico-urethral reflux were allocated randomly to four groups depending on the concentration of local anesthetic used and whether or not caudal block was added, in order to investigate which was more effec-tive for postoperative pain relief. However, none was no-ticeably superior. It is concluded that although continuous epidural block is certainly effective, we should not rely solely on pharmaceutical support, but should also use the mothers' psychological assurance to achieve satisfactory postoperative pain relief in children.
During an 11-year period, we surgically treated eight patients with gastroesophageal reflux who neurologically impaired. Their symptoms were hematemesis, anemia, stridor, frequent rerspiratory infection and poor weight gain. Apneic episodes occurred in four patients. Marked reflux of contrast medium into esophagus was in all patients. The interval from the onset of symptom to operation ranged from 5 years to 16 years. Nissen fundoplication, approximation of the esophageal hiatus and gastropexy were carried out in 6 patients, who showed no recurrence. Recurrence was noted in two patients, one who underwent only fundoplication, and the other, fundoplication and approximation of hiatus. Postoperative complication occurred in four patients : intestinal obstruction, prolonged gastric emptying, wound infection and intussusception of small intestine. Symptoms were alleviated in the patients without recurrence. These results suggest that surgery for GER in neurologically impaired patients requires close communication between the staff of the institute and families caring for the neurologically handicapped patients to ensure early treatment
Recently, cases of antenatal diagnosis in neonatal surgical patients have been rapidly increased by progress in perinatal medicine and fetal ultrasonography in our country. The data in this paper were obtained from 15 pediatric surgical institutions and 8 obstetrial insutitutions during the past 5 years. Two handred and seventy-seven cases (18.9%) out of total neonatal surgical patients were antenatally detected their anomalies. Intestinal atresia, meconium peritonitis, ovarian cyst and disorders of urinary tract including kidney were detected frequently by fetal ultrasonography. On the other hand, it was very difficult to detect intestinal malrotation, aganglionosis and imperforate anus. Antenatal diagnosis for neonatal surgical diseases offer many benefits as follows : improvement of perinatal care for fetus with congenital defects, maternal transport, change of the timing or mode of delivery, cooperative medical team for treatment and the appropriate family councering before birth. Out of 185 patients with antenatal diagnosis whose perinatal managements were clearly investigated by question-naries, 138 cases (74.6%) were delivered and treated at the same hospital. Fourty-six cases (24.8%) were changed timing of delivery and 74 cases (40%) were delivered by elective cesarean section. Many cases with abdominal wall defect or diaphragma-tic hernia were selected timing or mode of delivery and were performed surgical treatment immediately after birth.
During the past 5 years and 4 months, 5 patients (4 boys and 1 girl) developed postoperative intus-susception (POI) at Kagoshima University Hospital. The age ranged from 4 days to 10 months. POI occurred following colostomy (1 case) and closure of colostomy (2 cases) in anorectal malformation, antireflux surgery (1 case) for hiatus hernia and hepatic portojejunostomy (Roux-en-Y) (1 case) for biliary atresia. The common symptoms are abdominal distension, bilious vomiting, and increase nasogastic aspirate. Rectal bleeding occurred in only one patient with ileo-colic intussusception. The symptoms occurred 3 to 11 days after the initial surgery. The site of intussusception was ileo-ileal in 4 patients and ileo-colic in one. The ileo-colic intussusception was successfully reduced by pneumatic reduction method. One of 4 cases with ileo-ileo intussusceptior who required operative management, was needed resection of strangulated ileum because of mucosal necrosis During the same period, 495 cases excepting for cases with postoperative intestinal obstruction underwent laparotomy at our institute. The number of postoperative intestinal obstruction (POIO) was 26 cases. The rate of POIO in laparotomized cases was 5.25% and that of POI was 1.01%. POI accounts for 19.2% of POIO. Ninety percent of POIO developed the symptomes more than 30 days after the initial laparotomy. On the other hand, all of 5 cases of POI developed the symptoms within 2 weeks after the initial surgery. POI should be suspected in children with signs of bowel obstruction in the early postoperative period and the prompt surgical management prevents bowel resection.
Hickman's dual lumen catheter (HDLC) was placed in 50 pediatric oncology patients for 56 times, and its usefulness was compared to a concurrent series of 42 patients receiving the Broviac catheter (BRO). All HDLCs were used for the purpose of medications, routine blood sampling and transfusions, and also used for total parenteral nutrition. Three HDLCs developed catheter-related sepsis (CRS) (5.4% /catheter, 0.51/1000 catheter days) while 4 BROs developed CRS (8.7% /catheter, 0.64/1000 catheter days). Complication rate with HDLC was not higher than with BRO. Patients with HDLC were completely relieved from the pain with puncturing peripheral vein for blood sampling and making infusion route. Based on the low incidence of complication, as well as on the improved patients' quality of life, HDLC can be safely utilized in the pediatric oncology patients.
The association of malignant neoplasms with certain congenital disorders is well known. A female infant with liver tumor and hemihypertrophy is reported. The patient was born at term after a normal pregnancy. Left hemihypertrophy and right macroglossia were noted at birth. At the age of six months, a large palpable mass in the right upper portion of abdomen was noted with high serum levels of alpha-fetoprotein. An arteriogram revealed a large mass in the right lobe of the liver. Tumor blood supply was from the superior mesenteric artery and a malignant liver tumor was suspected. At operation, the tumor was totally excised by right hepatectomy : The diagnosis was hepatoblastoma, en-bryonal type, confirmed by histopathological examination. Nine weeks after surgery, the patient developed severe thrombocytopenia. The thrombocytopenia was treated with steroids and gamma-globulin, resulting in rapid recovery. The increased tendency of chirdren with congenital hemihypertrophy to develop Wilms'tumor or adrenal neoplasm is well described. Association with hepatoblastoma or hemangioma is not so well recognized. This is the twelfth known case with the association In patients with hemihypertrophy we currently recommend not only excretory urograms but also liver ultrasonography at intervals of six months.
Congenital esophago-bronchial fistula in early childhood is exremely rare. The clinical details of a 22-month-old boy with esophago-bronchial fistula are reported. He was referred to our hospital for recurrent pneumonia and abdominal distention. Digital subtraction esophago graphy revealed a fistula between the mid-point of the esophagus and the left main bronchus. Bronchoscopy showed left bronchomalacia and an opening in the fistula at the membraneous portion of the left main bronchus. Surgery was performed through a right extrapleural thoracotomy at the 5th costal floor. After excision of a short wide fistulous tract resembling a cleft, the esophagus and left main bronchus were repaired with 4/0 vic-ryl sutures and 5/0 nylon sutures, respectively. Stenosis of the left main bronchus developed after the operation and was identified by bronchofiberscopy. The stenosis was successfully enlarged by balloon catheter dilatation. The patient is now doing well.
We have seen 10 cases of malignant lymphoma (non-Hodgkin's lymphoma). Three of them were recognized initially because of jaundice and abdominal tumor. The first patent seemed to have a pancreatic tumor from preoperative signs but was diagnosed as having a malignant lymphoma by histology of the biopsied specimen from the tumor. The other 2 patients had almost identical findings and were preoperatively diagnosed as having malignant lymphomas. In all cases, only a biopsy of the tumor was performed, but the tumor and jaundice disappeared immediatly after effective chemotherapy. We thus concluded that a child with an abdominal tumor and jaundice may likely have a malignant lymphoma. Effective chemotherapy must be undertaken as soon as possible once a histologic diagnosis is made from the biopsy specimen.
A infant with CDH developed failure were found upon echo-PFC after surgery. Blood gas cardiographic analysis. The levels and the general condition amount of tolazolin and fluids of the infant were stabilized by administered were determined by the administration of tolazoline arterial blood gas findings and postoperatively. Severe pulmonary echocardiographic analysis, hypertension and right heart Cardiac function and the atrioventricular pressure gradient can be noninvasively and easily measured by using echocardiographic analysis. Echocardiographic analysis was useful for managing infants with CDH pre-and post-operatively.
A two year, ten month old boy who has had tenesmus since three months of age, consulted a doctor for diarrhea. An anomaly of the rectum was noted and he was diagnosed as having congenital rectal stenosis after undergoing a barium enema at the Department of Pediatrics in our hospital. At the time of admission to our clinic, abdominal distension and a dilated intestine were noted. A smaal finger-sized circular stenosis, 4 cm from the anal orifice on the oral side was comfiemed by digital examination. The stenotic lesion was resected completely by a sacral approach and sutured with an end to end anastomosis. The diameter of the narrow portion was approximatory 7 mm in a resected specimen. Histological analysis showed a marked thickness of the muscular layer and partial squamous metaplasia, but nervous plexus were preserved normally. A minor leakage at the anastomosed portion was noted, however the patient was able to discharge on the 57th day after the operation without any after effects.
A female infant with Cornelia de Lange syndrome (CdLS) underwent the right Bochdalek hernia repair on the first day of life. Four years later, she presented hematemesis and dysphagia. X-ray examination revealed small hiatal hernia with stricture and marked gastroesophageal reflux (GER) with delayed gastroesophgeal emptying. We reviewed the cases of 217 patients with CdLS in Japanese and English literature from April 1974 to May 1989. Feeding difficulty was seen in 31 (70.5%) of 44 Japanese patients, in which one fourth required tube feeding. The common radiologic findings in 17 patients of Japanese and English literature were GER in 10 (58.5%), hiatal hernia in 8 (47.1%), lack of primary esophageal peristalsis in 7 (41.2%), nasopharyngeal reflux in 5 (29.4%) in order of fre-quency. Patients with CdLS have functional and/or structural abnormalities of upper gastrointestinal tract, which cause the life threatening and recurrent pneumo-nia. In the management of patients with CdLS, it is essen-tial to evaluate the nature of feeding disorder in order to make appropriate treatment without delay.
Mediastinal cysts of foregut origin are not rare in children, but the histologically defined combination of esophageal cyst and bronchogenic cyst is extremely rare. We report here a case of 6-year-old girl. She was admitted with a mass shadow in the upper mediastinum shown in the X-ray film of the chest. An operation revealed a cyst protruding from the right side of the esophagus just inferior to the azygos vein. The cyst was removed with the muscle layer of the esophagus. Histologically, the cyst consisted of esophageal cyst, bronchogenic cyst and multilocular cyst lined by variant respiratory epithelium. The postoperative course was uneventful. There are several cystic lesions of foregut origin that involve esophago-bronchogenic cyst. We discuss here about the etiology of these lesions.
A 3-year old girl with juvenile Granulosa Cell Tumor (JGCT) was reported and 11 cases with JGCT taken from Japanese literatures are reviewed. The patient, presenting with abdominal pain and distension, was referred to our hospital. An ultrasonic examination and CTscan demonstrated tumors at the bilateral ovaries and right adrenal region. Bilateral salpingo-oophorectomy and partial resection of the right adrenal tumors was carried out. Subsequently, she re-cieved several courses of chemotherapy consisting of cyclophosphamide, vincristine, adreamycin and cisplatin. However, no obvious therapeutic response was obtained and the patient died 4 months after her initial presentation. Although, the prognosis of JGCT is reported to be better than that of adult type. But it may be unfavorable in advanced cases with JGCT. Clearly, more effective treatment for advanced cases must be established.