Six neonates in the endotoxin shock were treated by hemoperfusion and/or hemodialysis. Three of them recovered from shock and survived. Three cases died. Two out three dead cases needed the resuscitation when they came to our hospital. Thrombocytopenia was improved in five cases. Metabolic acidosis returned to normal range in our cases. B.U.N. and creatine were also improved in five cases and in six cases respectively. But urine volume was not restored in three dead cases. Measurement of serum endotoxin level showed disappearance or decreasing tendency after those procedures. Charcoal filter DHP-1, which was clinically used for hemoperfusion, was tested experimentally for it's effectiveness to absorve endotoxin in saline. Our in vitro experiment demonstrated that endotoxin of 0.5μg/ml concentration in saline was completely cleared within 30 minutes by charcoal filter DHP-1. Hemoperfusion and hemodialysis were considered to be promising for the treatment of endotoxin shock.
We evaluated the external sphincter muscle by the application of electromyography in 52 preoperative patients of ano-rectal malformation and 10 patients of them had been sequentially examined to check the development of the muscle. Insertions of needle electrode within 5mm in depth were made at twenty five different points in 1 cm square at the perineum which had been determined to be the center of anus from the skin findings. EMG score was calculated by the summation of the points from which action potentials were picked up. The distribution map of the external sphincter was made by connecting those points. The EMG score was 18.1 ± 4.7 / 25 in low-type malformations. Otherwise, it was 11.0 ± 5.2 and 11.9 ± 4.1 in intermediate and high-type malformations respectively. In low-type malformations, the external sphincter was abundantly present in the area of 5 to 10mm in width and 10mm in length. Otherwise, intermediate and high malformations showec remarkably narrower distribution of the muscle than that of low-type malformations. In 15 patients (29%), the deviated distribution of the muscle from the dimple was demonstrated indicating the technical difficulty to preserve the muscle around the newly-constructed anus without preperrative electromyographic examination. The development of the muscle was shown even in high-type malformation and the increase of EMG score was +3.7 and +5 in the patient of intermediate and high-type malformation respectively. These results of electromyographic examination lead us to stress that preoperative electromyographic examination is extremely valuable technique to know the precise locality and volume of the external sphincter muscle which should be preserved to get the satisfactory sphincteric function.
Intestinal smooth myosin B was prepared from muscle layers around the lesion in four patienst with congenital intestinal atresia. And mypsin B ATPase activity was compared between the proximal dilated segment and the distal segment to atresia. In three, whose atresia might occur at an early period of gestation, proximal bowels showed less activity of myosin B ATPase than distal bowels. However, in another, whose atresia might occur due to intrauterine intussusception at a late stage of gestation, the proximal bowel indicate higher ATPase activity of myosin B that the distal bowel. These data meaned that contractibility of the proximal intestine to atresia was depressed in the former patients and was accelerated in the latter patient. These results suggested that the extensive resection of dilated proximal bowel in the congenital intestinal atresia is not always necessary to obtain good postoperative intestinal dynamics at the operation of the atresial lesions which may be induced at a late period of gestation.
The advantages of the upper gastrointestinal study for the diagnosis of midgut malrotation would be summerized as follows: (i) The upper gastrointestinal study is the most reliable method for demonstrating midgut malrotation with or without volvulus. (2) Once we establish the basal points of a normal duodenal loop, it is easy to make a diagnosis. (3) To evaluate the duodenal loop, it is necessary to perform roentgenograms in the supine position.
Our clinical experience and our experimental study suggested that administration of adequet nutrients is utmost important for successful management of short gut. Preferable formula for short gut must contein some amount of protein as one of the stimulator for hyperplasia of mucosal cells, and simple carbohydrate to prevent troublesome diarrhea due to carbohydrate intolerance. In addition, it must be low in osmolarity and contain less fat than the ordinary formula. The formula was made of carbohybrate-free milk and dextrin, which was hypo-osmotic (125 mOsm/l) even at full strength and contained no hazardous carbohydrate. It has been used for three patients with short gut secondary to apple-peel atresia. The length of remained small bowel was 26cm (from the pylorus), 36cm, 24cm respectively. The result was encouraging. They have not suffered from attacks of severe enteritis and have achieved a steady gain in weight.
Twenty seven cases of aganglionosis extending into the small intestine were treated during the past 16 years. Among 16 cases of total colonic aganglionosis, which includes the aganglionosis involving ileum within 30cm. orally from the ileocecal valve, 10 cases (62.5.%) survived. Of 11 cases of the more extensive aganglionosis, 2 (18.2%) survived. The Soave's operation was used for 4 cases with total colonic aganglionosis, while the Martin's procedure was indicated for 6 cases with total colonic and for 2 infants with the .more extensive aganglionosis. The feces of all patients received the Martin's procedure became semiformed within one year after the operation. Clinical observation indicates that the reahsorptive capacity of the Martin's operation is well preserved in the aganglionosis with small bowel involvement. The purpose of this paper is to report the operative results of total colonic and the more extensive aganglionosis and to discuss on some points of treatment of this condition.
The functional evaluation of anorectum in Hirschsprung's disease was performed before and after operation using VTR-endoscopic method we have independently devised. During 3 years, more than 30 children with Hirschsprung's disease were submitted to this investigation. In normal children, a reflex relaxation of rectum and anal canal was observed to the pressure stimulation. On the other hand, no reflex movement was caught in Hirschsprung's disease, and when inflated to get an appropriate view, the aganglionic segment remained open just like a cavern. In the postoperative review, some patients who were operated on by Duhamel's procedure and Z-plasty showed reflex relaxation mainly in the posterior wall that was pulled through and anal canal. These findings give us many important suggestions concerning the mechanism of anorectal function.
Nineteen cases of childhood rhabdomyosarcoma was reviewed. Ten were male and nine were female. The age of the patients was from 6 months to 11 years, and 13, about two-thirds were under 3 years old. The sites of the primary lesion were various, and 11 were in the genitourinary system. The histologic type was embryonal 15, alveolar 3, and pleomorphic 1. Before 1975, there were 8 cases and only one was survived. It was sharply contrasted with 11 cases from 1975 to 1980, in which 8 are surviving without disease. The better result was obtained by multimodal therapy, especially introduction of effective chemotherapy namely VAC. It was shown by angiography that rhabdomyosarcoma is a vascular-rich tumor. So, recently we use intraarterial catheter to infuse chemotherapeutic agents. After intraarterial infusion of agents with radiation, the shrinkage of the tumor was remarkable and histological examination revealed nearly complete disappearance of tumor cells. With this method, it is possible that patients can be cured by more restricted operation, especially when the lesion is in the genitourinary system or in extremity.
We have observed the developmental change of the trachea and the main bronchi at the various gestational age in six fetuses using a scanning electron microscope. The surface of the trachea and the main bronchi at 4 months of gestation showed a predominance of non-ciliated cells covered with microvilli and protruding into the lumen. The ciliated cells, which appeared solitary or grouped, were scattered among the non-ciliated cells covered with microvillous and cilia-like projections of varying lengths. Along with the advance of gestational age, the number of the ciliated cells was observed to increase. The ciliated cells at 7 and 9 months of gestation were found to cover almost of all the luminal surface, and the diameter and length of the cilia were uniform, approximately 6 u to 7u.
Sixty-eight infants under 2 weeks of age have heen admitted to the department of pediatric surgery in the last 4 years. Nineteen infants (28%) developed neonatal hyperbilirubinemia (NHB) and were treated with the phototherapy and/or exchange transfusion. During the last 1 year, 500 infants have been born in our hospital. Seventy (14%) of this group developing pathological jaundice were treated in the same manner. The incidence of NHB was statistically much higher in surgical group than in non-surgical group (p < CO. 005). In the surgical group, eight of 12 low birth weight infants (67%) developed NHB. On the other hand, 11 of 56 infants (20%) weighing more than 2,500g developed NHB (p < 0.005). The surgically treated infants were divided into two groups : gruop I was composed of 47 infants with gastrointestinal obstruction and group II was composed of 21 infants without gastrointestinal obstruction. The incidence of NHB was 30% in group I and 24% in group II. However, there was no significance in these two groups. Five infants of group I were complicated with perforation of the gastrointestinal tract and peritonitis. Three of these 5 infants showed NHB. Therefore it is suggested that the perforation and peritonitis increase the risk for NHB. Seven of 17 infants (41%) with severe dehydration, in whom percent weight loss was more than 10, developed NHB. On the other hand, NHB was found in only 12 of infants (24%) associated with mild or moderate dehydration. Although there was no statistical significance, it is not possible to ignore the fact that severe dehydration is one of the developing factors of NHB. Our own experience indicate that the risk factors of NHB in neonatal surgery are low birth weight, perforation of the gastrointestinal tract and peritonitis, and severe dehydration. The presence of gastrointestinal obstruction may not be risk factors of NHB if dehydration and starvation have been corrected by proper management.
Cholangigrams in 14 cases with biliary atresia were evaluated. The intrahepatic bile ducts were visualized by retrograde cholangiography (operative or postoperative) in 6 cases of type I atresia (atresia at the common bile duct) and by percutaneous transhepatic cholangiography in 8 cases of type III atresia (atresia at the porta hepatis) and in one case of type I atresia. The intrahepatic bile ducts were abnormal in all cases. Repeat postoperative cholangiography showed that the intrahepatic bile dusts had been degenerated progressively in the cases with poor operative result. Postoperative percutaneous transhepatic cholangiography showed that the intrahepatic bile ducts had become almost normal in certain cases with satisfactory result.
A tracking chart for the evaluation of therapeutic effectiveness and over-all condition in critically ill infants has been designed on the basis of clinical investigation of infants with respiratory distress. The applicability and usefulness of the chart, which is composed of Modified Respiratory Index (M index) and arterial pH (pHa), have been confirmed in the study of 10 infants with Bochdalek hernia and 11 infants with gastric rupture. The effectiveness of surgical treatment for both groups is shown as a distinctive contrast on the chart (Figure 1 and 2). The difference is considered to be related to the pathological bases for each disease. More than 80% of trials for weaning from ventilatory supports were successful when the weaning is carried out within the "weaning zone" of the chart (Figure 3). When the infants are clinically diagnosed as being in shock, all most of their data are located within the "extremely severe condition area" (Figure 3). It is concluded that this chart may be helpful in evaluating the therapeutic effectiveness as well as in predicting of the perioperative course of these surgical infants.
Thirty four patients with biliary atresia have undergone Kasai operation in our clinic during the past 12 years. Of 21 patients with good bile excretion, 8 have been surviving for more than 2 years. Four of these survivors and 2 who subsequently died of hepatic failure after successful operation achieving bile excretion have developed portal hypertension with esophagel varices. More than 30 similar patients have been reported in the literature. Esophageal varices after successful Kasai operation develop in the one fourth of the patients who are expected to be cured. Half of these will develop hemorrhage. Portal hypertension related to biliary atresia can be classified into the acute and chronic types. The acute type was with poor prognosis due to severe postoperative cholangitis. Almost all patients with the chronic type survived, if the esophageal varices were well controlled conservatively or operatively. Transthoracic esophageal transection with paraesophagogastric devascularization seems to be the treatment of choice, and splenectomy should be added through the diaphragm in patients with hypersplenism.
During the past 20 years, 40 cases of TCRV have been operated on at our clinic. Eighteen were female and 22 male. Twenty six cases were under 15 years of age. Thirty cases (77.5%) were combined with VSD. Two cases were combined with VSD and AR which seemed to orignate from infected endocarditis. In 31 cases, the VSD was located at the right ventricular inflow-tract. Reoperations including closure of large VSD, resuspension of right coronary cusp and closure of residual VSD were done in three cases. Two cases died of complete A-V block and from accidental injury of the sinus of the valsalva. Long range clinicalt result of surgery was excellent in all but a case who had arrhythmia such as atrial fibrillation. We emphasize the importance of prevention of postoperative RBBB also.
Eighteen neonates with surgically treated congenital heart disease have been reviewed. The rate of operative mortality was 56 percent (10 deaths). Only six patients (33%) in this series are postoperatively survived. Nine patients had undergone open heart surgery under hypothermia and three have survived. Three of other 9 patients who had undergone palliative operation have survived. There were five patients with total anomalous venous drainage (TAPVD), of whom two survived after operation. There were four patients with ventricular septal defect and coarctation of the aorta or interruption of the aortic arch. Two of them underwent total correction and were expired. The remaining two patients who underwent coarctectomy or closure of the ventricular septal defect survived after operation. There were three patients with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. One of these survived after pulmonary valvotomy. Three patients underwent the ligation of the patient ductus arteriosus and two survived. There were one patients with coarctation of the aorta, one with tricuspid atresia and one with ectopia cordis. Causes of deaths were technical failure of operation in four patients, myocardial damage in three and respiratory failure and sepsis in one patient. Myocardial damage was acutemyocardial infarction, which was considered due to inappropriate myocardial protection during surgery.
This paper presented a 4-year-old boy suffering from congenital bronchial atresia of the anterior and apico-posterior segments of the left upper lobe with a bronchogenic cyst. Our case had a mass-like atelectatic shadow of the left upper lung field on a plain chest radiograph and was not associated with the occurence of localized emphysema as well as an abnormal hilus shadow representing the mucous-filled blind bronchial stump. The diagnosis was established by a bronchogram which showed a filling defect in the affected part of the lung. On the aortogram, there was no abnormal vessels from the thoracic aorta into the mass-like shadoow. A left upper lobectomy with the bronchogenic cyst was performed. Several dilatated bronchi in the resected specimens showed an appearance of bronchial mucocele which contained a plenty of yellow mucous fluid. Bronchial atresia with a bronchogenic cystwas very rare, which was interesting in relation to congenital bronchial atresia. In addition to the report of bur case, we discussed about the review of 38 cases from the literetures.
A parasitic body with a arm and legs attached to the epigastric region of a male newborn at full-term birth. The autosite weighed 3,400g at birth. The Fallot complex and omphalocele in size of 7× 6 × 6 cm were found. Under the amniotic sac the parasitic bowel was seen. An Upper GI tract X-ray series, Ba enema, IVP, cystogram and angiography via umblical artery revealed no organ communication between the autosite and the parasitic body. Inorder to avoid the rupture of the omphalocele, contamination, clotting troubles by circulatory insufficiency and respiratory burden, the radical resection with primary repair of the omphalocele was performed en the 3rd day of birth. The parasite was successfully detached without leaving any tissue in the autosite body. The abdominal wall was primarily closed. The parasite weighed 380 g. The lower extremities were well developed on both sides, but the arm not well developed. The head, heart, thorax, vertebral column and an arm were defective The sagittal sections in series of the extirpated pelvis were examined microscopically. The parasite had the bladder, colon, sacrum, prostate, external genitalia without testicles and atretic anus. The testicles were found on the skin-edge of the resected arm. Both testicles were underdeveloped for his age. By these findings,"it was pointed out that the organs of the parasite which are left in the autosite at operation may have potential of neoplastic development the autosite. Reviewing the Japanese literatures, the authors found 13 cases of the Epigastrius. Seven case are still-biith and 6 operated. Five cases are alive, but only one of the abdominal communicatioi is alive. The association of omphalocle is found in high incidence.
Rhabdomyosarcoma of the kidney is extremely rare both in children and adults. Recently, a 1 year and 10 month old girl was surgically treated for rhabdomyosarcoma of the right kidney at Tohoku University Hospital. The preoperative diagnosis of this patient was nephroblastoma. The right kidney measuring 9 × 6 × 6 cm was successfully removed. The histological examination showed embryonal rhabdomyosarcoma. The clinical course of the patient was reported with a review of literature.
We have experienced three cases of meconium obsruction without cystic fibrosis of the pancreas. These 3 cases are considered to correspond to meconium disease of Rickham or meconium plug syndrome of Clatworthy. From the standpoints of diagnosis and treatment, we thought it the worthwhile to classify meconium obstruction without mucoviscidosis into small intestine type and the colon type depending upon the site of the obstruction. All these 3 cases we had experienced were 2-day-old females. By the preoperative and intraoperative findings, the differential diagnosis of meconium disease from meconium ileus was difficult. However, mucoviscidosis was ruled out in all three cases by trypain activity test of meconium and duodenal juice and by sweat test. All cases underwent surgery. Two were cured, but one died of pulomaonary disease at 4 months after surgery although the obstruction was successtully relieved.