The existence of HLA-DR antigens was investigated in biliary atresia, using monoclonal anti-HLA-DR antibody.In 4 of the 5 patients with BA, biliary epithelial cells of the frozen liver biopsy specimen taken at the time of Kasai operation showed positive for anti-HLA-DR antibody. Because of the availability of anti-HLA-DR antibody that can be used for the formalin-fixed spacimen, 15 liver biopsy specimen previously taken from 9 patients with BA were investigated for the positivity. In 11 of the 15 specimens, HLA-DR antigen was positive, while 4 was negative. All of these negative 4 were the specimen taken at reoperations performed at after 130 days old. As there are recent reports on increased expression of HLA-DR antigen of epithelium in the patients with various immunopathologial conditions such as primary biliary cirrhosis, rejected liver graft, and graft-versus-host disease. This increased expression of HLA-DR antigens in BA suggests that the pathogenesis of BA be an immunological assalt.
The Purpose of this study is to investigate the effect of portal administration of Adriamycin (ADM) for primary liver tumor in children as a step toward complete resection, using experimental and clinical studies. In the experimental study, 26 mongrel dogs were divided into 4 groups according to the route of administration of ADM (0.2mg/kg) ; Group I : Bolus injection via peripheral vein in 10 dogs. Group II : Bolus injection via portal vein in 6 dogs. Group III : Continuous infusion (30 min.) via portal vein in 5 dogs. Group IV : Bolus injection via portal vein following the ligation of hepatic artery in 5 dogs. The data can be summarized; 1. The average concentration of ADM in the liver tissue in group II was almost twice of that in group I. 2. The concentrations of ADM in heart and kidney tissues in group II were significantly lower than those in group I. 3. The concentrations of ADM in liver tissue in group III were similar to those in group II. But the concentration in heart and kidney tissues was the highest in the group III. In the clinical study, the intra-portal ADM infusion chemotherapy were carried out in 2 children with hepatoblastoma as an initial therapy. Although the patients died of pulmonary edema or sepsis before operation, respectively, the ADM concentration of central portion of the tumor was 5 times higher than that of normal liver tissue at autopsy. The study suggested that the intra-portal chemotherapy can be an effective step as a preoperative chemotherapy for the malignant liver tumor.
Free intestinal transplant with microvascular anastomosis has become a useful procedure for reconstruction of the cervical esophagus in adult patients. We intend to introduce this technique for young infants. In these cases, however, dysfunctions of the grafted jejunal segment such as dysphagia and regurgitation are said to be problems. These problems must be solved before clinical application. In this paper, a new ischemic canine model and histometrical studies in the ischemic jejunum by the ischemic jejunum by the image analyzing system (TAS Plus) are described. And in this study, we tried to clarify the deadline for ischemic time of the free intestinal grafts. Ischemia was made in the jejunal segments of this model. The ischemic time was verified in each segment from 1 to 4 hours. Then the histometrical studies of ischemic damage especially in the Auerbach's plexus were done. The ratio of the area of the nerve plexus to a ganglion cell was calculated, and we found it decreased when the ischemic time exceeded more than 2 hours. We concluded that the ischemic time of the free jejunal transplant should be within 2 hours.
From 1965 to 1985, pulmonary stenosis with intact ventricular septum (PS with IVS) was diagnosed in 176 patients by cardiac catheterization at the Meijo Hospital. Sixty-four cases underwent corrective surgery (55 valvotomy and muscle resection, 4 simple outflow patch, 5 transannular monocusp outflow patch). Three had pulmonary valvular dysplasia. There were 3 hospstal death. Preoperative recatheterization showed progressive high systolic right ventricular pressure (PRV) in all case over 80mmHg of PRV and in some of 65-80mmg at the first catheterization. Preoperative PRV correlated well to pulmonary valve area measured intraoperatively (r=-0.455). The higher preoperative PRV cases showed the higher postoperative PRV. Eight cases without outflow patch, who were over 3-year-old and had high PRV over 80mmHg, had persistent high PRV over 80mmHg at one month after operation. The intraoperative peak systolic right ventricular pressure versus systemic arterial pressure ratio (PRV/PSYS) (x) well correlated to postoperative PRV/PSYS (y) (r=0.834, y=0.16+0.50x). It is concluded that (1) PRV over 80mmHg is anindication for operation, and 65-80mmHg may be followed conservatively with caution, (2) the elder patient with higher PRV requires an outflow patch, especially over 5-year-old and 150mmHg, (3) the transannular monocusp outflow patch is useful for pulmonary valvular dysplasia, (4) prediction of prognosis and decision of surgical technique are suggested by measuring of the intraoperative PRV/PSYS, (5) we must cautiously decide the surgical indication and technique for PS, not only preoperatively, but also during operation.
3-hydroxy-5-cholenoic acid which is one of monohydroxy bile acid with hepatotoxic effects, has been considered the product of an altered pathway of biosynthesis of bile acid in the liver. In the serum of cholestatic patients, it increases as a form of either sulfate-conjugated bile acid or glucuronide-conjugated bile acid. Bile acids in serum patients with biliary atresia and neonatal hepatitis were measured by high performance liquid chromatography (HPLC) combined with immobilized enzyme in colum form and new analytical method using sulfatase and β-glucuronidase. In preoperative biliary atresia group and neonatal hepatitis group, sulfate-conjugated bile acid was more dominant than glucuronide-conjugated bile acid. However, in postoperative biliary atresia group with persistent jaundice, consentration of glucuronide-conjugated bile acid was as much as that of sulfate-conjugated bile acid. Our studies suggested that glucuronide-conjugation might be related to development of UDP-glucuronyl-transferase activity and might play an important role in excreting abnormal bile acid into urine as well as sulfate-conjugation.
Clinical and manometric studies on anorectal function were performed in 76 patients treated by the posterior triangular colonic flap (PTCF) method or Lynn's rectal myectomy. The majority of the patients took a favorable course after operation. Although a small number of patients showed poor continence and/or soiling during their postoperative courses, the bowel habits gradually improved with the growth of patients. In patients who underwent the PTCF method, the resting pressure of anal canal decreased after the operation and recovered to the normal level within 3 years after the operation, probably with the development of the surrounding muscle components by the growth. The resting pressure of the rectum in these patients elevated just after the operation, but decreased to the normal level in about 3 years after the operation. These findings correlate with the improvement of patients' bowel habits. On the other hand in patients with rectal myectomy, the resting pressure of the anal canal was lowered just after the operation and maintained at the levels lower than that of the normal controls. This fact was attributed to the resection of the muscle layer in the anal canal. The resting pressure of the rectum elevated and remained high for more than 3 years in them. This findings can be explained by the high tonus of the rectum oral to the aganglionic bowel which was left after rectal myectomy. Data of basal rhythmic waves in the postoperative patients showed no significant change in comparison with the normal controls. There was no relationship between the presence of recto-anal reflex and clinical bowel habits in these patients. According to the results of the manometric studies, the patients with unsatisfactory surgical outcome were divided into 2 groups. One is the patients with a high anal canal pressure which might be caused by remaining of the internal sphincter achalasia, and the other is the patients with a low anal canal pressure presumably due to developmental retardation of the muscle components around the rectum or a lack of the toilet training after the operation.
We experienced five cases of acute hepatic failure following open-heart surgery congenital heart diseases: two cases of tetralogy of Fallot, one each case of ventricular septal defect with mitral regurgitation, coarctation of the aorta with ventricular septal defect, and transposition of the great arteries. Their ages at operation ranged from two months to five years. The exchange transfusion for acute hepatic failure was performed with 150-200ml/kg of fresh whole blood. After the exchange transfusion, the level of GOT, GPT and LDH decreased significantly. The level of total birilubin. ALP, BUN, and Crdecreased as well, but not significantly. In the results three of five patients survived and two died. We thought that acute hepatic failure following open-heart surgery was not always associated with low cardiac output syndrome and might not have any causes. Postoperative hepatic failure influenced upon hemodynamic state unfavorably. Exchange transfusion for such patients was very effective for not only the improvement of their hepatic function but also the stabilization of their hemodynamic state. We concluded that postoperative hepatic failure occurred within one week after operation, and exchange transfusion was useful for their treatment.
Our pre-, and postoperative adjuvant therapy of advanced neuroblastoma was changed from mild chemotherapy to aggressive one in 1978. In order to evaluate the clinical effect of this policy, 39 cases before 1978 and 37 cases after 1978 were reviewed retrospectively using UICC staging system. Both clinical staging (CS) and postsurgical histopathological staging (PS) showed almost 100% cure rate in their stage I and II. Curability of CS III and PS IIIA/B was remarkably improved after 1978, but that of CS IV and PS IV remained miserable except a disease free survival. Eight cases which were classified in CS II-IV at first, were reclassified in PS III and all of them survived. Disease free survival of advanced neuroblastoma could be obtained by complete or nearly complete resection of tumor mass accompanied by aggressive adjuvant chemotherapy. No clear difference in prognosis was seen between the two stagings of Japanese Society of Pediatric Surgeons and UICC.
To study the urological problems in the newborn, we analysed the clinical data of 41 patients with congenital urinary tract anomalies which had been detected in his or her first month of life between 1973 and 1986. There were 10 patients with posterior urethral valve, 7 with pelvi-ureteric stenosis (hydronephrosis), 6 with ureterocele, 6 with multicystic kidney, 5 with vesicoureteral reflux (VUR), and 7 others. Most common signs and symptoms indicating urological survey were abdominal mass (21 patients, 51%) and urinary tract infections (18 patients, 44%). Other signs and symptoms were abnormal voiding in the patients with urethral valve, failure to thrive of the patients in renal dysfunction and urine ascites. Prenatal diagnosis was made in 5. They were transfered to us and evaluated during the newborn period before clinical symptoms appeared. Prompt urological management was required in 12 patients. Of 10 neonates who had had enlarged bladder from urinary retention, urethral catheter was indwelt in 7 and cystostomy was performed in 3. Nephrostomy was placed in 2 patients with hydronephrotic solitary kidney. The follow up study of these patients showed impaired renal function in 9 (22%) and urinary incontinence in 6 (15%).
Three patients with advanced neuroblastoma were treated with a multimodal therapy, which protocol consist of chemotherapy, delayed primary operation, intraoperative irradiation and autologus bone marrow transplantation (ABMT). High dose cyclophosphamide, THP-adriamycin, vincristine and cisdiamminedi-chloroplatinum were administered as chemotherapeutic agents, and high dose L-phenylalanine mustard (L-PAM) as preconditioning of ABMT. 1) This protocol of chemotherapy was found to be very effective in spite of severe bone marrow suppression. 2) Serum neuron-specific enolase (NSE) is useful in the decision of the timing when the operation and ABMT should be performed. 3) Intraoperative irradiation is effective, especially in infants and children. 4) It is very important to keep good renal function in order to carry out this protocol. 5) ABMT is very useful because up to 10 times of conventional doses of chemotherapeutic agents, for example, high dose L-PAM 180mg/m^2 can be administered. 6) Moderate chemotherapy should be continued during 2 years after ABMT. We cannot conclude the result of our treatment because the maximum follow-up period is 2 years 4 months, but all of three patients are in disease-free condition now.
From June 1983 through May 1986, 4 patients, aged from 3 to 11-year-old, with corrected transposition of the great arteries (c-TGA) underwent repair of associated morphological tricuspid valve regurgitation (TR). TR became severe in 2 patients after closure of ventricular septal defect (VSD) or radical correction for VSD and pulmonary atresia. One of them required tricuspid valve replacement (TVR) 9 years postoperatively, and the other underwent TVR on the day after radical operation. Severe TR was detected in 2 infants. These 2 patients required TVR at the age of 3 or 6 years. There is two type of TR associated with c-TGA; one is due to the malformation of the morphological tricuspid valve, and the other develops postoperatively by the enlargement of the morphological right ventricle or the damage of the morphological tricuspid valve. With this in mind, we should follow up the patients with c-TGA after the radical correction. Repair of the morphological tricuspid valve is not feasible as reported, and TVR is the method of choice even for the young child. The children can be managed easily with Warfarin and Dipyridamole after TVR. The SJM valve is a satisfactory cardiac valve substitute for the pediatric age group.
Thirty-eight patients who had survived cardiovascular surgery in neonatal period were the subject of this study. These patients were divided into three groups. The first group includes total anomalous pulmonary venous return (TAPVR), coarctation of the aorta (Co/A), and patent ductus arteriosus (PDA). The patients of this group underwent primary correction, and are in good condition on a long term basis except one who underwent intracardiac repair of TAPVR. The second group includes Co/A complex, interruption of the aortic arch (IAA), complete transposition of arteries (dTGA) with high pulmonary blood flow, pulmonary atresia with intact ventricular septum (PPA), and pulmonary stenosis with intact ventricular septum (PPS). The patients of this group underwent two-staged correction. All of them are in good condition on a long term basis. The third group includes the other complicated cardiac malformations, and the patients of this group have not undergone intracardiac repair yet. Some of them underwent multiple palliation. In some cases, the feasibility of intracardiac repair cannot be found so far although they started to undergo palliative surgery from their neonatal period.
In this study, we discuss about the clinical value of enema as a diagnostic aid for appendicitis in children in the base of our experiences during recent 4 years and half. The candidate for barium enema was the patients who was considered not to have appendiceal perforation or which diagnosis was indistinct from other clinical data. Its findings were classified to five types. Type I is non-filling of appendix with cecal or ileal compression, Type 2 shows also non-filling of appendix, but no deformities on cecum or ileum in this type. Type 3 is partially filling appendix, type 4 is irregularly filling appendix and type 5 shows completely filled appendix to its tip. In type 2 and 3, there are so many false positive cases that we must be careful to decide the indication for operation. On the other hand, it was very diagnostic in type 1 and 5. Barium enema should be indicated when the diagnosis of appendicitis is uncertain.
A 11-year-old boy having adult type hepatocellular carcinoma associated with positive hepatitis B surface antigen was reported. On admission his upper abdomen was protruded and hard mass was palpated. Laboratory examination revealed elevated serum AFP, 589ng/ml.; cholesterol, 730mg/dl.; GOT, 168U/ml., GPT, 185U/ml. Serum HBs antigen was positive, but HBe antigen was negative. The patient was suspected to have been infected by maternal vertical transmission. In radiographic finding CT showed multiple low density area, and angiography revealed hypervascular tumorous lesion feeded by right and left hepatic artery. Tumor resection was impossible, and limited only to biopsy, so infusion chemotherapy of 5-FU and MMC from proper hepatic artery was performed. There was no improvement, and he died of tumor progression on the 51st hospital day. Postmortem examination of the liver revealed posthepatitic cirrhotic hepatocellular carcinoma complicated with bleeding, necrosis, and fatty degeneration. Review of the Japanese literature disclosed 10 other cases of HBs antigen positive hepatocellular carcinoma in children. All the cases were male, and HBs antigen and HBe antibody were postive, but HBs antibody and HBe antigen were negative. In laboratory finding serum AFP was elevated in all, but the value of cholesterol was not so high and in nearly half the cases below 300mg/dl. In radiographic finding CT showed multiple low density area, and angiography revealed hypervascular tumorous circulation.
A 43-day-old female infant weighing 2,800g was referred to the surgical department because of cyanosis, tachypnea, subcostal recession and poor feeding. Pre-operative echocardiogram and cardiac catheterization showed anomalous origin of the right pulmonary artery from the aorta. Using cardiopulmonary bypass, surgical correction was successfully performed with ligation of the origin of the right pulmonary artery from the ascending aorta and side-to-side anastomosis between the right pulmonary artery and the main pulmonary artery. Ligation was also performed for the patent ductus arteriosus which was discovered during operation. Her sternum was able to be closed on the 4th postoperative day and the tracheal tube was extubated 11 days after operation due to the prolonged post-operative cardiac and respiratory failure. However, the patient's body weight is increasing gradually and she appears to be in good condition four months postoperatively.
We report 5 cases of various types of urinary tract anomalies associated with cyanotic congenital heart diseases. The urological management of the patients depended on a degree of urgency in urinary tract anomalies and the cardiac condition of an individual patient: temporary urinary diversion was performed in 3 cases, and one-staged definitive surgery in 2 cases. Though 4 cases in this series required urological surgery before the corrective operation for the cardiac anomalies, 1 case could have undergone a urological operation after the cardiac surgery. It is suggested that the principles of the urological management in a patient with associated urological and cardiac anomalies can not be standardized but should be individualized.
This study is a case report of our patient with segmental dilatation of the ileum and a review of literatures described in English and German. Two-year-old girl presented epigastralgia, vomiting, and abdominal distension. She was roentgenologically found to have giant intestinal gas on the right hypochondrium. Laparotomy revealed segmentally dilated ileum. She underwent segmental resection of the affected bowel and end-to-end anastomosis. We collected 34 cases of this disease from literatures. Of the 34 patients, 24 were in the small intestine, and remaining 10 in the colon. Of the 24 cases with lesion in the small intestine, 14 were situated in the terminal ileum. A single, well defined segment of dilated terminal ileum has been described as segmental dilatation, giant Meckel's diverticulum, communicating giant vitelline duct cyst, or ileal dysgenesis. Among these disorders saccular segmental dilatation of the ileum quite resembles giant Meckel's diverticulum. Segmental dilatation of the small bowel, especially ileal cases, often had heterotopic tissues, but it was rare in colonic cases. Small bowel cases had also different clinical features. To judge from this diversity, it seemed that segmental dilatation is not a single distinct pathological entity. The cases of the colon may be different clinical entity from those of the small intestine, and embryogenesis of ileal segmental dilatation is probably quite related to that of giant Meckel's diverticulum.
A case of asymmetric conjoined twins (Epigastrius) was successfully operated. The autosite had omphalocele. The parasite was attached to the xiphoid region, shared the abdominal cavity with the autosite, and had well developed lower lims, pelvis, kidney, bladder, ovaries, and intestine. At operation, it is important to remove all the tissues or organs belonging to the parasite.
Authors presented a new operative method for treating a congenital external fistula of the main parotid duct. A skin flap surrounding the orifice of the duct was made and the duct was isolated for the distance of 1.5cm. An oblique tunnel through the cheek was created and the skin flap was introduced into the oral cavity and sutured to the maocosa. The early and late results after this operation are good.
A patient with delayed presentation of right-sided diaphragmatic hernia was reported. This patient was a boy who had operation for gastric rupture at 3 days of age. He had been on mechanical ventilation for 3 days postoperatively. Chest x-ray taken during this period showed no abnormality. On the 6th postoperative day, when he developed cyanosis suddenly, chest x-ray disclosed atelectasis of the right upper lobe and infiltrate of the right lower lobe. A diagnosis of right diaphragmatic hernia was made on day 41 by the findings of repeated chest x-rays, ultrasonography, and liver scintigram. Thoracotomy performed at 53 days of life disclosed a 8X7cm right-sided diaphragmatic hernia with herniation of a right lobe of the liver. The defect was repaired with uneventful recovery. This case suggests the following conclusion: 1. A previously normal chest x-ray does not necessarily preclude the presence of congenital diaphragmatic hernia. 2. When a chest x-ray shows an abnormal shadow in the lower lobe after an abdominal operation or mechanical ventilation, the possibility of congenital diaphragmatic hernia should be considered.
This is a report of a case of Pelvic abscess which was treated successfully by per rectal catheter drainage using ultrasound guidance. A 7-year-old female, who had undergone an appendectomy several days ago at another hospital, was admitted with persistent high fever, abdominal pain, nausea and vomiting. A large tender abdominal mass was found in her lower abdomen. Ultrasound examination was performed using a 5 MHz linear transducer. An abnormal hypoechoic mass with an internal echo and a hyperechoic irregularly thick wall was shown by sonogram in Douglas's pouch. Under general anesthesis, a 14 Fr. trochar catheter was inserted into the abscess cavity through the rectum under real time ultrasound observation, and the purulent exudate was fully aspirated. After aspiration and generous lavage, the catheter was placed on continuous suction. On the next day, the patient became cheerful and was without fever or abdominal pain, and she left our hospital on the 15th day. Percutaneous drainage with ultrasoud guidance for patients with a suspected free abdominal abscess is not so popular because of the unclear picture, possible peritoneal contamination or puncture of the bowel. However, an abscess in Douglas's pouch becomes clearly seen in the echo window behind the urinary bladder, and the abscess can be drained safely without a significant incidence of complications.
One hundred and seventy eight cases were analysed in this report. Tumor-free two-year survival rate was used for the study. For the site of tumor occurence, testis was signicantly better than ovary and sacrococcygeal teratoma only in the Stage I. In the testicular tumors, there was no difference of results between the former 5 years and latter 5 years. No significant therapeutic factors were shown concerning on radiotherapy and chemotherapy. In ovarial tumors, dysgerminoma was better in prognosis than yolk sac tumor as a whole stage. The effect of radiotherapy was not shown. In sacrococcygeal teratoma, only 3 out of 25 survived over 2 years free of tumors. Two of them were in Stage IV. Chemotherapy would be the key point in good combination with operation and radiotherapy. In retroperitoneal teratomas, all of ten were fatal in 2 years.