Gastric ruptures in neonates are still one of the most unfavorable diseases. For the explanation of the genesis and unfavorable prognosis, gastric ruptures were produced experimentally in puppies by increasing intragastric pressure. Experimental gastric ruptures occurred along the lesser curvature different from clinical cases. Prior to the gastric mucosal rupture, tears of gastric muscle layer and serosa were observed in clinical impending rupture. Histology showed defects of muscle layer at ruptured edge, seemingly a result of contraction of the ruptured muscle. However, wide defects of muscle layer seen in the clinical cases could not be explained by contraction of ruptured muscle. Factors other than the increase of intragastric pressure, therefore, seemed to be important. Regarding a preoperative management (of gastric rupture), our experiment indicated that infusion therapy should be done within 4 hours after admission and surgery be started as soon as diuresis is obtained. Since severe peritonitis may be induced by formula milk intake, prohibition of milk intake along with the gastric decompression is considered to be an important procedure for suspected cases.
Four Moncclonal Antibodies (MoAb) were produced by hybridomas fusing immunized (by human neuroblastoma) mouse speen cells with mouse myeloma cell line p3-X63-Ag8653. These MoAb showed restricted reactivity for human neuroblastoma cell lines by ^<51>Cr release cytotoxicty assay in vitro, but did'nt react with human melanoma, mouse neuroblastoma and fibrosarcoma cell lines. In the immunohistochemical experiment of indirect immuncfluorescence using these MoAb, they reacted specifically with only neuroblastoma cells. By Avidin-Biotin-Complex (ABC) technique, paraffin section of human neuroblastoma tissues stained positively, but some of them cross-reacted with rhabdomyosarcoma specimen. This result suggests that these MoAb may by particularly useful reagent for the clinical diagnosis of human neuroblastoma.
Large portion of anorectal anomalies in swine was believed to be hereditary in the literature. In our series of experiments on the defect in swine, we have completed a radical operation technique in neonatal age and then performed deliberate matings among grown-up male and femal pigs with the corrected anomalies. Sixty-four pigs with defect were included in this study : 35 were males and 29 were females. We could analyze the heredity of this anomaly mainly in 3 swine families. Among 18 deliveries after mating, we could observe anorectal in 9 deliveries. The existence of heredity has been ensured. The hereditary trait was studied, mainly on 3 pig families. In K Farm family, the autosomal recessive genes were indicated. In Y Farm family, the autosomal dominant genes were indicated. Particularly, the mating between a male impeforate anus and a normal female sibling resulted to give 2 males with imperforate anus of high type and 9 females with rectovestibular fistula. The total occurence of the defect in this delivery is supported to be due both to dominant genes from grand father and recessive genes from grand mother. In the I Farm family, the autosomal reccssive genes were suggested by the analysis on 6 generations of this pig family tree. The genes to cause the defect were concluded to be common between male and female, as far as our experiments were concerned.
The quantitative measurement of plasma endotoxin was made in 64 cases of surgical neonates using the chromogenic assay (Toxicolour test). The preoperative values of plasma endotoxin were 64±59pg/ml in perforative peritonitis (n=9), 63±51pg/ml in gastroschisis (n=7) and 15±16pg/ml in intestinal obstruction (n=28) respectively, while the value in 20 cases without intestinal obstruction was 6±5pg/ml. In the serial measurements of plasma endotoxin in 28 neonates, the values on the first postoperative day increased significantly compared with the preoperative values. Then, they decreased gradually to normal range within a week in 15 cases without postoperative complication, plasma endotoxin remained at high level. The usufulness of evaluating endotoxemia in surgical neonates was discussed.
A morphometric analysis of the mitochondria in hepatocytes of the hepatobiliary diseases in childhood (biliary atresia, congenital dilatation of common bile duct, portal hypertention) are performed on wedge biopsy. The result obtained per unit volume of cytoplasm were as follows. 1) Volumetric density was 12.65±2.92% (Mean±1SD). 2) Average cut surface area of single mitochondria was 0.41±0.11μ^2. 3) Volumetric density of single mitochondria was 0.22±0.06%. 4) Numerical density was 405±133/1,000μ^3. 5) Surface density of outermembrane was 0.95±0.17μ^2/μ^3. 6) Surface density of outermembrane-to-volumetric density ratio was 7.78±1.04μ^2/μ^3. 7) Morphometric values had no statistical significance among the diseases. 8) In biliary atresia, morphometric values had no statistical significance before and after 60th day of age. 9) In the cases of scarce bile flow and prolonged hyperbilirubinemia after first portoenterostomy in biliary atresia, marked fibrosis were recognized under light microscopy and volumetric density of mitochondria increased and numerical density of mitochondria decreased. The results of the current study indicate that the function of the mitochondria itself in biliary atresia do not change after 60th day of age, but in the cases of prolonged hyperbilirubinemia after first portoenterostomy, fibrosis advance and the function of mitochondria are also disturbed. Therefore the procedure of first protoenterostomy is important for the prognosis in biliary atresia.
A rationale for postoperative adjuvant immunochemotherapy in patients with advanced neuroblastoma was searched through experiments used A/J mic bearing C-1300 murine neuroblastoma. 1. Cell kinetic of residual tumor cells after debulking surgery showed an increase of proliferative fraction from 18 to 42 hours after reduction surgery, while that of the bone marrow cells were seen from 24 to 96 hours later. 2. Administration of Na_2 ^<51>CrO_4 fluid intravenously into the operated tumor bearing mice demonstrated accelerated isotope intake in the residual tumor mass at any point postoperatively until day 5 than preoperative point, especialy at 6 hours and on day 3, which suggested enough blood flow and/or drug distribution to the residual tumor. 3. Early drug administration with the one fourth of murine LD_<50> on day 1 after reduction surgery, when cell kinetic of the residual tumor cells showed a biggest proliferative fraction, could not bring about a better chemotherapeutic effect than late administration on day 3. 4. Postoperative ip administration of small dose (75 or 15mg/kg) of cyclophosphamide (CPM) induced a remarkable delay of tumor growth and prolongation of the life span, which effect was largely abrogated on the immunoodifficient mice exposed to thymectomy and irradiation. 5. Transfer of the spleen cells from the tumor bearing operated mice shortened the latency time of tumor appearrence on the recipient mice, while transfer of the spleen cells from mice operated and administered with CPM did not influence the latency time of the recipients. Early postoperative administration of small dose of which is cytotoxic to suppressor cell fraction is seemingly promising. 6. Administration of CPM on day 3 after reduction surgery, when the proliferative fraction of bone marrow cells was biggest, caused a transient spleen reduction and the following rebound overshool swelling. Present data suggest the antitumor activity of CPM following reduction surgery of C-1300 neuroblastoma is mediated by both pharmacological and immunologic mechanisms.
This study is a review of our patients with biliary atresia for determination of predictability in prognosis after Kasai procedure in terms of liver transplantation. Sixty-six patients who have undergone kasai procedure for biliary atresia durng a period from 1971 through 1981 were subjected to this study. Fifty-six had a bile drainage and the remaining 10 did not. Seventeen became anicteric with serum bilirubin below 1.0mg% during 6 months after operation and have been free of jaundice for 3-12 years. In 9 patients with delayed subsidence of jaundice, only 4 surviyed. Twelve of the remaining 26 deaths were due to hepatic disorders aggravated by cholangitis. A constant increase of serum bilirubin was characteristic in this group and all died in one year after serum bilirubin reached 10mg%. This study has suggested : (1) determination of serum bilirubin at 6 months after operation is an important index for future course, (2) liver transplantation should be urgently considered for those with recurrent jaundice with constant increase of serum bilirubin exceeding 10mg%, (3) those with an absence of initial bile drainage or with deterioration of hepatic disorder at 6 months after operation be candidates of liver transplantation.
We performed the Limulus test (L-test) to evaluate endotoxemia in 14 patiets who had gastrointestinal perforations (7 gastric and 7 intestinal) in the neonatal period, and had the following results : 1) Eight (3 gastric and 5 intestinal) of the 14 cases (57.1%) were diagnosed as the endotoxemia by the positive result of L-test. 2) The L-test-positive cases showed high mortality ; five patients in 14 boses died (62.5%), but the L-test-negative cases were all survived, so that the endotoxin may greatly affect on the prognosis of these cases. 3) In all autopsy cases (5 cases), hemorrhage was observed in various organs, such as the lung, kidney, adrenal gland and gastrointestinal tract, however, thrombi were detected histologically in two of three cases who had been clinically diagnosed as disseminated intravasular coagulation. Thus, we think that L-test is essential evaluation of clinical course and prognosis of the patients with gastrointestinal perforation in the neonatal period.
Five infants with parotid hemangioma are reported with successful conservative treatment. Male to female ratio was 2 : 3. The onset of infantile parotid hemangiomas was less than 3 months of age, and tumors grew up rapidly. Compression of the external auditory canal and pharynx was found in 2 infants. Pathologically, resected spcimens showed capillary hemangioma in all. Total parotidectomy was done in 1 infant with mild facial palsy for 14 years after surgery. Partial parotidectomy was performed in 2 infants with prednisolone therapy which was given orally 20 or 30mg every other day for 4 and 6 months respectively. Three years after treatments, the residual mass disappeared completely in both. The remaining 2 infants underwent the every other day prednisolone therapy alone with successful regression. Parotid hemangiomas in infants usually tend to regress spontaneously, and can be treated with only prednisolone when they are so large in size. Prednisolone can be administered 20 or 30mg every other day without any complication and changing the circadian rhythm of cortisol levels in serum.
During a period of 13 years from 1970 to 1983, 38 patients have been treated for thoracic empyema (non-tuberculous) at the Kobe Children's Hospital. Seventeen patients were treated by serial thoracenteses and 28, including 7 children without improvement by thoracenteses, by an insertion of a chest tube. Two patients have undergone decorication and right upper lobectomy respectively, after unsuccessful chest tube drainage. There was no death in this series. The group treated by an insertion of a chest tube as the primary therapy has provided the best result with earlier subsidence of fever and improvement in the x-ray findings. In a long term follow up, a thick peel observed at the end of therapy has become unremarkable several years after. The observation in this series provides a auggestion that an insertion of a chest tube is the choice of initial treatment, and once the symptoms subside, a spontaneous resolution of the pleural peel can be expected in several years.
In 22 patients aged from 1 month to 9 years who have undergone the repair of tracheoesophageal fistula during the neonatal period, a review of clinical course, upper gastrointestinal x-ray series and esophageal pH monitoring were carried out for associated gastroesophageal reflux (GER). Eleven patients were symptomatic for possible association of GER. In 9 patients, GER was observed by upper GI series. In 3 of those with GER observed by upper GI series, clinical symptom was suggestive. Esophageal pH monitoring test was positive to GER in 2 patients in whom the clinical course and upper GI series were significant. Of 22 patieints, three have ultimately undergone a fundoplication. It was interesting that these 3 patients belonged to the group of patients (5) who have undergone a gastrostomy at the time of repair of tracheoesophageal fistula. There was no GER patient in the group of patients (17) in whom the gastrostomy was not created.
Thirteen patients with hypertrophic pyloric stenosis (H.P.S.) were studied preoperatively and seven were also examined following Ramstedt pyloromyotomy with real-time ultrasound (U.S.G.). For the diagnosis of H.P.S., it was important that the pyloric diameter≧14mm, muscle thickness≧3mm, stenotic index (S.I.=muscle thickness×2/pyloric diameter)≧50%, and morphological change and motility of antro-pyloric region were observed. Postoperative findings of the portion of pyloromyotomy was seen as "wedge sign" and it was difficult to see by 2 weeks after surgery. In comparison with preoperative finding and 7 days after surgery, S.I. was decreased from 52.6% to 35.3% and muscle thickness was from 3.8mm to 2.5mm. Postoperative vomiting were observed in the patients with the less decreasing rate of S.I.
Many recent articles describe an increase of survivors in biliary atresia. Very little is known, however, about the studies of long term follow up. This paper reports the results of follow-up studies of long term survivors who are living more than 10 years after surgery. During the period from June 1967 to August 1974, a total of 61 patients had been treated by Kasai's hepatic portoenterostomy operation in originany reported fashion. Among these, 21 patients, that is 34.4% of total, are alive and so all of these survivors are more than 10 years old now. The oldest 2 patients have become 16 year old. None of these 21 patients was accompanied with ascending cholangitis early after surgery, but mild cholangitis developed in 12 patients later. The height in 2 patients and the body weight in 5 patients are considerably lower than the average. Hepatomegary exists in 12 and splenomegary exists in 9 patients including a case with hypersplenism. Liver function tests are thoroughly normal in 10 patients, but some of transaminase, alkaline phosphatase or the colloid reaction still maintain higher level than that of normal range in all of the remaining 11. Portal hypertension has been diagnosed in 9 patients by the endoscopic examination of esophageal varices. Mild varices in one patient disappeared spontaneously, but 6 of these 9 patients bled varices. An esophageal transection was indicated for 4 and an endoscopic sclerosing therapy was performed for the remaining 2 successfully. Out of 21 long term survivors, 10 are free from any clinical symptoms, abnormality of the liver function tests or the sign of portal hypertension. The remaining 11 patients, however, have some problems in such aspects and they should be closely followed up hereafter.
A 10-month-old female with a swelling of right breast for a month period was repoted. It was suspected as a breast tumor, but after examinations it was diagnosed to be a hemangioma under the breast tissue. At 11 months of age, complete removal of the hemangioma was done because of rapid enlargement of the size. During the operation, the hemangioma was noted to be located in the pectralis major muscle. Pathological diagnosis was a covernous intramuscular hemangioma.
Side-to-side duodenojejunostomy had commonly been used for congenital intrinsic duodenal obstruction such as annular pancreas and membranous stenosis in newborn period until a diamond-shaped duodenoduodenostomy was developed. Blind loop syndrome rarely occurs, but is an abominable complication after shunt operations of intestinal surgery. A girl, 13 years of age, with blind loop syndrome developing after duodenojejunostomy in the newborn period is reported. She complained of right upper abdominal pain, restriction of daily diet and weight loss for recent 2 years. After resecting a membranous (windsock web) of the duodenum and double duodenojejunostomy revealed by laparotomy, an end-to-back dudenojejunostomy has been performed to get a physiological pathway with favorable results. Long term follow-up study seems to be essential to patients who underwent duodnojejunostomy for congenital duodenal obstruction in newborn period.
Rectal atresia with a normal anus is a rare anomaly in ano-rectal malformations. A 8 month old boy was treated successfully by non-suture method using a newly developed instrument and a colonofiberscope. This instrument is set up of a supporting bar, a pair of rings and a spring. At his newborn period, a left transeverse colostomy was made for decompression and further investigations of rectal blind pouch. The details of this procedure are summarrized as follows ; 1) insert a nylon thread into rectal blind pouch from anal side penetrating the atretic region 2) pick up the thread by forceps under a direct vision through colonofiberscope resulting in a passing of the thread from anus to distal orifice of colostomy 3) place a proximal part of the instrument in rectal blind pouch by using the nylon thread 4) place a distal part of the instrument including a spring in anal blind pouch and accomplish the compression of an atretic regin. Preoperative careful examinations are recommended to avoid the damage to puborectal muscle which is important for a continence. Distance between two blind pouches and position of blind pouches in relation to a puborectal muscle should de precisely evaluated. If indication is exactly selected, this procedure is useful because of it's simplicity.
A Japanese boy weighing 3,100 grams, was admitted with progressive dyspnea and cyancsis sixteen hours after birth. Chest X-ray revealed left bochdalek hernia and a herniorrhaphy was done. On the fifteenth post operative day, the chylothorax was complicated and continuous drainage of the pleural cavity had to be done. He was then placed on a MCT diet, with good clinical improvement, in six days.
Stricture of the intestine caused by a blunt trauma of the abdomen is rare and less than 30 cases have been reported in the literature including adults so far. In this paper, we report a case of 8-year-old boy with intestinal stricture, with a review of the world literature. He received a blunt force of the upper abdomen with a seat-belt of car being drived by his mother which run into a guard-rail, resulting in recurrent intestinal obstruction and vomiting. The boy had been in relatively good condition until approximately 7 weeks after the trauma when he started symptoms of a complete obstruction of the intestine. On 51 days after the trauma, intesital stricture was confirmed by GI series and surgery was done. At operation, the ileum of 110cm from Treiz's ligament was remarkably narrow and the omentum adhered to this part, therefore, the ileum of 8cm including stricture part was resected. Histologically, there were scar tissues and ulcer in accordance with part of the rupture of muscular coat in the intestinal wall. Postoperative course was uneventful and he was discharged on the 17th day after surgery.
A one-month-old infant presented prolonged jaundice, hepatomegaly, and systolic murmur. Bilirubin was scarcely detected in his duodenal juice nor feces. HIDA Tc 99m did not appeared in the bowel after 2h. Endoscopic retograde cholangiopancreatography performed at 64 days of age showed the narrow common bile duct, the gall bladder, and proximal portion of the common hepatic duct but not revealed intrahepatic bile ducts. He was dignosed as biliary atresia and operated upon. Hepatic portoenterostomy was performed because repeated intraoperative cholangiograms failed to demonstrate intrahepatic bile ducts and fibrous remnants were detected at the porta hepatis. Liver biopsy revealed no interlobular bile ducts in portal tracts. He was made diagnosis of paucity of interlobular bile ducts postoperatively. After the operation bile juice did not flow out from external fistula and his jaundice had not been disappeared. Our experience suggested that hepatic portoenterostomy was not effective in a patient with paucity of interlobular bile ducts with fibrous remants at the porta hepatis. It was concluded that open liver biopsy specimens taken from both lobes should be immediately examined and diagnosed histopathologically in a patient with fibrous remants whose diagnosis of biliary atresia is doubtful.