Seventy-one neonates underwent cardiovascular operations for congenital heart diseases diseases from 1975 to 1984. There were 47 male and 24 female neonates ranging in age at operation from one day to 28 days (average 14. 2 days), and in weight at operation from 700 g to 4,150 g (average 2,900g). There were 15 cases of total anomalous pulmonary venous return (TAPVR), 8 cases of interruption of the aortic arch (IAA), 12 cases of coarctation of the aortic arch (Co/A), 18 cases of pulmonary atresia (PPA) or severe pulmonary stenosis (PPS) with intact ventricular septum, 5 other diseases with decreased pulmonary flow and 13 other diseases with increased pulmonary flow. There were 30 operative deaths in this series. The posterior approach without circulatory arrest and aortic cross clamp contributed to the improvement of the surgical results of TAPVR. The staged operation for IAA and Co/A complex was safe and preferable procecure, except for cases with the complicated anomalies. Regarding to PPA and PPS, the neonates with small right ventricle were treated with Blalock-Taussig shunt, whereas the neonates with large right ventricle were candidates for Brock procedure. These results suggested that appropriate palliative operations in neonates followed by later difinitive operation improved the surgical results of the congenital heart dideases at present.
The accurate diagnosis of malignant tumor type is essential to determine the correct therapeutic regimen and to predict a patient's prognosis. However, the differential diagnosis of "small-round-cell tumors", represented by neuroblastoma, rhabdomyosarcoma, leukemia/lymphoma and Ewing's sarcoma, can occasionally be difficult by conventional and biochemical methods. If monoclonal antibodies which would react with these tumor cell surface antigens were available, a membrane phenotype determined by these monoclonal antibodies could provide rapid and accurate diagnostic aids. In the present study, a panel of 9 monoclonal antibodies raised against hematopoietic cells (BA-1, BA-2, J-5 and B7/21), brain cells (UJ-13A, UJ-127-11 and anti-Thy-1), and neuroblastoma cells (HSAN1. 2 and PI 153/3) was used to analyze 7 fresh bone marrow tumor cell specimens: 6 neuroblastoma and 1 non-Hodgkin lymphoma. A rapid (within 2 hours) and confident diagnosis of each tumor type could be made on the basis of the membrane phenotype study.
Localization of substance P (SP) and vasoactive intestinal polypeptide (VIP) in intestinal segments of congenitally and experimentally aganglionic rats were examined using immunohistochemical methods. 1. In the ileum and colon of the normal rats: SP- and VIP-positive (P) axonal terminals surrounding nerve cells and SP- and VIP-P fibers interconnecting nerve cells in the plexuses of my enteric (Au) and submucosal (Me) were observed. In the enlarged intestinal segment of aganglionic rats; SP- and VIP-activities in the Au and Me were normal. In the enlarged segment neighboring on the histologically transitional segment, a definite increase in VIP-P nerve cells in the Me and a slight increase in VIP-P fibers in the circular muscle layer were observed. In the transitional segment, SP- and VIP-P nerve cells in the Au and Me and SP- and VIP-P fibers in the circular muscle layer were reduced. In the aganglionic segment, SP-activity was totally lost in the whole layer, while a few VIP-activities were noted in the circular muscle and mucosal layers at the portion neighboring on the transitional segment, but the activity disappeared in the mucosal layer at first, then did in the whole layer. 3. In the aganglionic colon of experimental aganglionosis, which was produced by previously described method: SP- and VIP-activities were lost totally in the whole layer.
The relationship between the type of obstruction and the course of the hepatic artery, cystic artery, and portal vein was analysed in 121 cases of biliary atresia. An accurate sketch of the bile duct, courses of hepatic artery, cystic artery and portal vein was made at the time of operation. Each anatomical situation was compared with the postoperative bile excretion condition. The following results were obtained: 1. Various anatomical relationships between the bile ducts and the course of vessels were observed in biliary atresia. However, the rate of the variations found was almost the same as that found in the normal distribution. 2. Differences in the type of vessel course positions were observed according to the type of biliary atresia. 3. The rate of the active bile excretion after operation was different not only according to the type of the obstruction, but also according to the anatomical positions of the bile ducts and the vessels.
Catheter-related infection is sometimes fatal, but is not rare complication accompanying intravenous hyperalimentation through a catheter inserted in central vein. From July, 1978 to June, 1985, 66 surgical infants underwent 156 cannulizations into central vein. They were divided into two groups according to the sites of cannulation: subclavian vein puncture (group A) was carried out 127 times and another venous cut-down (group B) 29 times. The groups were compared with respect to various clinical and laboratory parameters of infection. The incidence of catheter-related sepsis in group B (24.1%) was significantly higher than in group A (9.4%). In both groups the incidence of catheter-related infection increased after 30 days of cannulation. In conclusion, to avoid the catheter-related infection : 1) periodic exchange of the infusion line and stelization of catheter-insertion region are necessary, 2) the residual central venous catheter should not be kept for more than 30 days and 3) subclavian vein puncture is useful technique for repeated central venous alimentation even in infant.
Fifty seven children underwent appendectomies with acute appendicitis in the last twelve years in our university hospital. In a retrospective study, we have tried to elucidate the relationship between their preoperative course including their laboratory data and the histological findings of resected specimens. The result of our study disclosed that all of the cases who developed appendicitis under 6-year-old, and about half of the cases who were operated after 72 hours from their onsets, had the perforated appendixes. Therefore, we strongly suggest the importance of early diagnosis especially for children who showed any sign of appendicitis.
We encountered four cases of battered child syndrome (B.C.S.) with substantial background problems. The first case was a girl with facial burns resulting from violence from her father who himself had a history of being a victim of B.C.S. in his youth. The second case was a girl who had been repeatedly hit on her entire body by an alcoholic father. She had a congenital anomaly (rectocloal deforming). Her parents refused the necessary colostomy required to save her life in the beginning. The operation was eventually accepted but during successive follow up examinations after the operations many signs of B.C.S. were found on her body. The third case had a traumatic pancreatic pseudocyst after receiving a blow from her mother. The child, a boy, was a stepson of mother who also had a natural son of the same age. The fourth case was a rupture of the jejunum due to blows by the mother who had given responsibility for nursing the child. It should be considered that improvement of the patient's background factors through social services as well as the treatment of physical and/or psychological injury is necessary for the cure of the patients with B.C.S.
The incidense of congenital intestinal atresia and stenosis is about one from every 5,000 births. Eighteen cases with congenital intestinal atresia and stenosis have been treated in our department from April, 1972 to October, 1985. Of the eighteen cases, there are seven duodenal stenoses, four jejunal atresias, two jejunal stenoses, three ileal atresias, an ileal stenosis and a colon atresia. Five of the seven duodenal stenoses are annular pancreas. Three of the four jejunal atresias are apple-peel type of atresia. Two cases with ileal atresia, which had polypoid protrusion within the distal ileum, are considered to be caused by fetal intussusception. Although Down's syndrome is often associated with duodenal atresia and stenosis, only one cas was associated with Down's syndrome in our series. Although five cases (28%) have been died of congenital heart disease, idiopathic respiratory distress syndrome and anastomotic insufficiency, two cases with ileal atresia complicated with meconium peritonitis are alive and well.
Manometry was performed in a recto-vestibular fistula and five ano-vestivular fistulas. Contraction waves of anal canal and recto-anal reflex were recognized in both recto-vestibular and ano-vestibular fistulas. The presence of these phenomena were, therefore, not useful to distinguish ano-vestibular fistula from recto-vestibular fistula. It seemed that longer high pressure zone and higher resting pressure of recto-vestibular fistula were useful findings for differentiation. The fact recto-anal reflex was seen in reco-vastibular fistula means that the fistula was surrounded by internal anal sphincter which is thought the effector of the reflex, or that the reflex is otherwise, produced by puborectalis muscle.
During fifteen years, twenty patients with several types of anorectal stenosis were treated in our hospital the series of 350 anorectal malformations. Three male patients with anal stenosis presented with unusual appearance of the anus; their anus was buried deeply in their buttocks and were not relaxed and protruded even under caudal block or general anesthesia. An infant of them had H-type anourethral fistula associated with anal stenosis, which was diagnosed by urethrogram and was repaired through perineal approach. The other two had presacral teratoma and sacral bony defect with anal stenosis. Complete resection of the mass improved their bowel movements. It is suggestive from our experiences that unusual appearance of the anus with anal stenosis may be a sign of the co-existence of the combined malformations.
High Frequency Oscillation (HFO) was tried in 52-day-old infant who underwent ligation of patent ductus arteriosus (PDA) and developed postoperative regional atelectasis which was not improved by conventional mechanical ventilation. Remarkable improvement was observed in the blood gases analysis as well as chest X-ray findings on the 1st day of its application, presumably due to effective removal of tracheal secretion resulting in improvement of oxygenation in the lung. Therefore this method may be further applied for patients developing postoperative atelectasis.
Congenital "H-type" ano-urethral fistula is an extemely rare condition which is often accompanied with tracheoesophageal fistula (TEF) and some urological anomalies, but seldom with anal deformitiy. Clinical details of two patients which we experienced recently are reported here, together with the discussion of pathoembryology of this anomaly. One of the patients was 8-year-old boy, and he had hypospadia and undescended testis. Another patient was 4-year-old boy who had urethral stenosis, dysplastic kidney and undescended testis. In the world literatures, 13 cases with congenital "H-type" ano-urethral fistula could be found. Among 15 cases including ours, 7 had TEF and 12 had urological anomalies such as urethral stenosis, ectopic ureteral orifice, cross renal ectopia, and others. Anal anomalies were found in only three cases (1 covered anus, 1 rectal stenosis, 1 anterior perineal anus). Although much to be debated on the pathoembryology of this condition, it seems that the misaligned components of urorectal septum which lead incomplete devision of cloaca play a critical role to make up this rare condition.
A case of severe cyanosis with hepatic cirrhosis in a five-year-old girl who had undergone corrective surgery of congenital biliary atresia previously was reported. She had severe cyanosis, clubbed fingers and toes, polycytemia and squatting on exercise. Intracardiac shunt was ruled out by two-dimensional echocardiography. Contrast echocardiography. Contrast echocardiogram and lung per fusion scanning indicates the presence of intrapulmonary right-to-left shunt. The cardiac catheterization and pulmonary wedge angiography were done at age of five years, but intracardiac right-to-left shunt and congenital anomarous pulmonary vessels were not demonstrated. From those findings, we conclude that the cause of her cyanosis was intrapulmonary right-to-left shunt with multiple dilatation of pulmonary cappilary. This is a first case of cyanosis due to intrapulmonary right-to-left shunt complicating postoperative hepatic cirrhosis in congenital biliary atresia.
A 7-year-old boy presented with duodenal obstruction due to intramural hematoma of the duodenum. A conservative treatment by means of nasogastric intubation and total parenteral nutrition was instituted. The obstruction persisted for 3 weeks, but the size of the hematoma has decreased on imaging with US done 2 weeks of treatment. The upper G. I. series carried out after 35 days of treatment demonstrated a satisfactory passage through the duodenum. The size of the hematoma on CT scanning also dramatically decreased. The patient was discharged after 45 days of conservative treatment with a satisfactory condition. As a long-term parenteral nutrition is possible and a close observation of the hematoma by imaging with US and CT scanning, a conservative treatment is the first choice for management of intramural hematoma of the duodenum.
Vesicointestinal fissure is a complex malformation which consists of exstrophy of the bladder and the intestine, and associates many other anomalies, such as omphalocele, imperforate anus, short colon, malrotation of intestine, urogenital anomalies, separation of symphysis, with a high frequency. We have treated 5 cases with this malformation, three of them being alive and well. Their ages range from 3 years and 8 months to 16 years at present. Two cases died of nutritional depletion and sepsis. Conservative therapy was performed for omphalocele in all cases. Hernia sac shrank rapidly, and abdominal wall closed in about 40 days. For intestinal anomalies, one of 3 survivals had a permanent ileostomy; two had an initial ileostomy with later conversion to an end-colostomy. An anoplasty was performed is one of the two since his external sphincter muscle reacted well to electric stimulation and distal colonic segment developed about 30 cm in length. For bladder exstrophy, three had primary closure. They could't achieve urinary continence at all. One of them underwent an ileal-coduit afterward. For separation of symphysis, two had iliac osteotomy. We collected 63 cases from the Japanese literature and found that only 12 of them survived. Treatments of vesico-intestinal fissure were discussed from several aspects.
We recently encountered two female pediatric cases of paraurethral cyst associated with ipsilateral renal agenesis. Paraurethral cyst is a rare lesion and its etiology remains obscure. Therefore, we attempted to investigate the pathogenesis from a viewpoint of renal development at the beginning of metanephric period. The metanephrogegesis is organized by the ureteric bud developing from the mesonephric duct. Then, failure to form a ureteric bud may cause not only ipsilateral renal agenesis but persistent mesonephric duct: the mesonephric duct presists in the wall of the urogenital sinus, a part of urethral primordium. In this fashion it is suggested that the persistent mesonephic duct has a chance to grow into a paraurethral cyst. The high frequency of simultaneus occurrence of paraurethral cyst and renal agenesis may support this theory which has been also proposed by Mackie and Stephens in 1975. In this paper diagnosis and treatment of this lesion were also described.
A newborn case of congenital solitary nonparasitic cyst of the liver was reported here. A large intra-abdominal cystic mass was found through antenatal ultrasonic examination in the 31th week of pregnancy. At the birth, a large abdominal mass was notified by palpation. Series of preoperative examinations suggested a large cyst in the abdominal cavity. The removal of the cyst was performed on the 33th day of life. At the operation, a large solitary cyst was identified originating from the antero-inferior margine of the right lobe of the liver and contained approximately 220 ml of straw colored fluid. The cyst was resected up to the liver edge. Postoperative course was uneventful and the liver regeneration was pretty good 6 months after operation.
We treated three patients with advanced stage neuroblastoma with cisdiamminedichloro-platinum (CDDP), high-dose cyclophosphamide, vincristin, and adriamycin. The patients were 3 years 9 months, 1 year 11 months, and 5 years 11 months of age at diagnosis respectively, In summary 1) These combination chemotherapy was quite effective for the initial remission induction of the three cases, 2) The CDDP was also effective for the treatment oi metastatic bone lesion of one of the cases. 3) Major side effects of this regimen were electrolyte abnormalities, renal dysfunction and severe diarrhea, all of which were well-tolerated with the supportive care. 4) The profound bone marrow suppression was associated with this therapy, and disturbed the pursuit of the regimen. We prophylactically applied leukocyte rich blood transfusion, which helped to complete the schedule. 5) We concluded that further aggressive cure-oriented therapy such as combination of delayed-primary surgery, and autologous or allogeneic bone marrow transplantation should be considered after achievement of complete remission with such an intensive chemotherapy.
During past seven years, 64 neonates and infants underwent central venous total parenteral nutrition. Five of 64 patients (7.8%) had persistent left superior vena cava (PLSVC) with atresia of the esophagus, duodenum or anorectum. One of the five who had congenital esophageal atresia (Gross C type) died of thrombosis localized in the coronary sinus and right atrium followed by sepsis. In conclusion, high incidence of PLSVC is suggested in pediatric surgical patients, and LSVC should be avoided as a route of central venous catheterization because of high risk of thrombosis.
Adrenocortical carcinoma is very rare disease in childhood. Here we reported our 5 cases of acrenocortical carcinoma and reviewed reported cases in Japan. Case 1 was Cushing syndrome and the other were adrenogenital syndrome (AGS). One was male and 4 were females. The distribution of age were from 5-month to 5-year 9-month-old. Their main clinical symptoms were abdominal tumor and hypertrophied clitolis. Serum and urinary hormonal analysis demonstrated high level of 17-OHCS (case 1) and 17-KS, DHEA (case 2, 4, 5). CT, angiography and 131I-rdosterol scintigraphy were effective measures to obtain the information of tumor. Complete tumor resection were performed in case 4, 5 and only these cases has been survived for more than two years postoperatively without the evidence of recurrence. In literature, only 50 cases were reported in Japan. Compared with adult cases, their clinical features were markedly differented (AGS 26, Cushing 9, combined 10, non-fuctioning 3 and unknown 2. Their prognosis were poor, however, recent advances of oncology and diagnostic methods has been enable to improve them.
Three hundred and seventy one cases of Wilms' tumor were analysed in this report. Tumor-free two-year survival rate was used for this study as in the first report. On operation, total resection and non-total resection of the primary tumor were compared in relation to age, resective method of lymph node, and the existence of distant metastasis. Under one year, and between 2 and 4 years of age, the tumor-free two-year survival rates were signicantly greater in the group of total resection. On radiotherapy, the tumor-free two-year survival rates were compared between with and without the therapy, in relation to stages. Twenty-three cases with preoperative irradiation were examined without any significance. The significance of post-operative irradiation was examined in each stage and each microscopic type. On chemotherapy, tumor-free two-year survival rates and reccurence rates were examined. In preoperative therapy, no significant results were given. In post-operative therapy, Actinomycin D, Vincristine and the combination of both were compared in various dosages. When Actinomycin D was solely given, the group with over 450 micrograms had significantly greater two-year survival rate. When the combination of Actinomycin D and Vincristine was gigen, the group with full dosage had significantly greater two-year survival rate.