In a retrospective survey of 627 neonates with major pediatric surgical disorders treated during the last 15 years, cardiovascular malformations (CVMs) were present in 80 patients. Association of CVM was highest in patients with esophageal atresia (37.5%) followed by omphalocele (27.5%) and congenital duodenal atresia (19.2%). Of various CVMs, those characterized by increased pulmonary blood flow such as VSD, PDA, ECD, and CoA・IAA syndrome were the commonest (63.8%) of all. Severelity of the associated CVM was the most significant determinant of patient's prognosis and it was considered as main cause of death in 52% and was judged indirectly responsible for patient's deteriolation in 30% of the cases. Mortality (including operative, hospital and late death earlier than 6 months following initial operation) in patients with associated CVM was 56% in contrast to 6.7% for patients without CVM. It has decreased to 38% during the recent 5 years in contrast to 65% for the preceding 10 years and this decrease was most striking in patients with CVM characterized by increased pulmonary blood flow. It is concluded that early recognition, early noninvasive diagnosis and early surgical intervention when indicated of CVM are essential for the better outcome in the management of patients with associated CVM.
Absorbable sutures were used for anastomosing the subclavian artery to the pulmonary artery in 20 cases undergoing Blalock-Taussig operation and the results were compared with those obtained with nonabsorbable suture in 22 cases. 1) With absorbable sutures, gratifying operative results were obtained with as high a shunt patency rate as 100%, even though an additional shunt operation was required not infrequently. 2) There was no operative mortality, nor did occur rupture or aneurysm at the anastomotic site due to its diminished anti-tensile strength. 3) Cardio-angiographic evidence indicates that the use of absorbable sutures well prevented, the occurrence of stenosis at the anastomotic site, where inner vessel surfaces remained virtually smooth and, in some instances, the vessel itself was even growing. 4) Macroscopically, the anastomotic site was smooth-appearing and there were no visible sutures; histologically tissue reactions at the anastomotic site were modest. 5) From the facts that their use was associated with virtually nonoccurrence of stenosis of the anastomosed vessel and a good patency rate of the shunt, absorbable sutures were concluded to provide a useful suture material for use in Blalock-Taussig operation.
Histopathological condition of intrahepatic bile ducts with special reference to lectin binding capability were examined on liver in 13 cases of biliary atresia (BA). Seven types of FITC labeled or biotinylated lectin were used in this study. Among of them, Helix Pomatia Agglutinin (HPA) only showed characteristic binding pattern to bile duct epithelium. HPA binding capability with negative Alcian-Blue staining of the bile duct epithelium was normally seen only in the fetus and infants younger than one month of age. In cases of BA, HPA positive epithelium of the proliferative bile ducts were observed until 5 months of age in the central portion of the portal triad. Results of this study strongly suggested that persistence of fetal charactericity of the bile duct in BA patients remains several months longer than in normal infants.
Carnitine levels in serum and urine obtained from 136 infants with normal diet were compared with that from 80 infants with carnitine-free diet. The result showed no significant age-related difference in the levels of carnitines. The reference values of serum free, acyl, and total carnitine were 20.2 - 83.4, 15.9 - 54.9, and 46.8 - 119.7 nmol/ml, respectively. Urinary carnitine levels were different between each individuals and the reference values of free, acyl, and total carnitine were 0 - 55.5, 0 - 56.3, and 3.8 - 125.8nmol/m/ respectively. However the free and total carnitine level in serum of the infants with carnitine-free diet were lower than that of the infants with normal diet (p < 0. 01), there was little difference in acyl carnitine level in serum between these two groups. These results suggested that acyl carnitine might have more important roles in human than free carnitine, and were considered to be the reason why the infants with carnitine-free diet didn't suffer from "carnitine deficiency". The carnitine levels of the gastric juice, intestinal juice, and ascitic fluid obtained from 46 specimens were similar to the serum carnitine level of the same individuals. In pediatric surgery, post-operative patients are generally limited to taking exogenous carnitine and lose endogenous carnitine through the loss of gastric juice, intestinal juice and ascitic fluid. The importance ,of carnitine metabolism in pediatric surgery should always be kept in mind.
BUN, serum creatinine, creatinine clearance, fractional excretion of sodium (FENa), serum β_2-microglobulin, urinary β2-microglobulin, and urinary N-acetyl-β-D-glucosaminidase (NAG) were measured in eight patients with advanced neuroblastoma who were receiving cis-diammine-dichloroplatinum (II) (CDDP). These parameters changed within the first week after CDDP treatment. Increased urinary excretion of NAG was observed untill the second week had passed after CDDP administration. But the value of each parameter on the 3rd day after CDDP administration was not significantly correlated with the cummulated dose of CDDP administered. These results indicate that these parameters show acute renal injury due to CDDP treatment, but the long-term latent nephrotoxicity of CDDP cannot be predicted from these parameters.
In order to recognize the present status of surgical treatment of this disease as a whole in Japan, questionnaires were forwarded to 86 main institutions. The questionnaires were filled out and returned by 67 institutions, and the data of the total 653 cases were complied for this study and analysed by computer. The results of this study and the problems in the treatment of this disease were as follows: 1. More than 60% of institutions in Japan had experienced less than 2 cases of biliary atresia in a year. The cases were widely scattered among various institutions. 2. Early diagnosis has not been achieved yet. About 20% of the patients were operated upon 80 days after their births. 3. The surgical results are not satisfactory. Only 46% of the patients are free of jaundice. 4. The results of re-operation is especially poor. Jaundice cleared in only 29% of the re-operated cases. The indication and timing for re-portoenterostomy should be settled. 5. Postoperative cholangitis is still a serious problem. Forty-three % of patients were complicated with postoperative cholangitis. 6. Recurrence of obstructive jaundice frequently occurs. About a half of jaundice free patients have developed recurrence of jaundice during their postoperative courses. 7. Indication of liver transplantation should be established as early as possible. 8. In the United States, Biliary Atresia Registry was introduced in 1978. The necessity of a registration system is also recognized in Japan.
In children, cholelithiasis is relatively rare and frequently result from some etiologic factors such as hemolytic disease, anatomic malformation of the biliary tract and disturbance of the enterohepatic circulation of bile salts, especially in infants. Seven cases with cholelithiasis have been treated in our department during the 13-year-6-month period between April 1, 1972 and October 31, 1985. Of these cases, 3 were associated with congenital biliary dilatation, one with hereditary spherocytosis and 2 in siblings with familial intrahepatic cholestasis. The remaining one was diagnosed on autopsy incidentally, in a patient with omphalocele and died of aspiration pneumonia. Cholelithiasis associated with familial, intrahepatic cholestasis is very rare, only four cases have been reported previously in the world. One hundred and twenty nine cases, including our 7 cases, have been collected in Japanese previous literatures. These cases were evaluated with regard to causes, clinical symptoms, diagnostic methods, etc. Recently, reported cases of cholelithiasis in children have gradually increased and were diagnosed mostly by ultrasonography, because of this examination is easy and not invasive.
Thirty-seven cases of hepatic injuries experienced for 20 years were reviewed. Using a classification based on the severity of the hepatic injuries, the problems on the diagnosis and the treatment were evaluated. Hepatic injuries were divided into 3 groups; minor vessel (s) injury (I), major vessel (s) injury (II) and hilar injury (III). Deaths were encountered in 2 of group I and 5 of group II patients. In group II, the hepatectomy was indicated. A pre-established patient management program based on the classification was needed as a method for improving the results in treatment of the patients with hepatic injuries.
Two patients with a pancreatic neoplasm were reported. In both cases the tumor occurred in adolescent female (15 and 16-year-old) who complained of abdominal pain and presented with a palpable mass in one case. One required nearly total resection of the tumor with combined resection of coIonic mesentery, and the other required a partial resection of the tail of pancreas. Those tumors were approximately 6 × 6 × 5 cm and 8 × 8 × 8 cm in size. Gross pathological examinations revealed apparent encapsulation, cystic degeneration, and hemorrhagic necrosis. Microscopically, the tumors were characterized by distinctive solid and papillary patterns. Such lesions have been reported recently in the literature as "solid and cystic" tumor of the pancreas. Approximately 36 such cases have been reported to date in the Japanese literature. There are only two patients died from distant metastasis or local invasion. Our patients presented here are alive and well 22 and 23 months after surgery. Assessing the degree of malignancy is difficult since this tumor is capable of local invasion but usually does not metastasize. However, according to therapeutic results from literatures, this kind of pancreatic tumor is potentially curable by surgical resection.
The authors reported a case of malignant rhabdoid tumor of the left kidney. The case was a 3-year and 11-month-old boy, who had a 9 × 15 cm sized tumor in the left retroperitoneal space. The tumor showed no invasive extension, and was successfully exstirpated along with the left kidney and the regional and para-aortic lymphnodes. Metastatic legions in the paraaortic lymphnodes were proved histologically. Therefore, the patient was classified into stageIII MRTK After the radical operation, regimen DD of the NWTS-4 (ACD + VCR + ADR + Radiation) was continued for 15 months without any signs of the disease. Prognosis of this disease is poor, because of the relapse which frequently occurs within 4 months after operation and the majority of the patients dies within 1 year. Seven among 47 suvivors accumulated in the literatures were clinically reviewed in this report.
A 1-year and 5-month-old boy of renovasucular hypertension was treated with reconstruction of the renal artery. Arteriography revealed the right renal artery stenosis with post stenotic dilatation and complete obstruction of the left renal artery. It was difficult to control the hypertension, so we decided the operation. The stenotic portion of the right renal artery was resected and it was reanastmosed to the aorta by intermittent sutures with 6-0 prolene. The left nephrectomy was added. After operation, the blood pressure was easily controlled with captopril only and symptomes disappeared. Renovasucular hypertension is curable with surgical procedure. Children who are suffering from renovasucular hypertension should be treated with surgical procedure, including percutaneous transluminal angioplasty.
An 11-month-old girl who presented with jaundice was found to have a solitary intrahepatic biliary dilatation by a CT-scan. After medical treatment for 13 months, she was referred to our pediatric service for further examination and surgical treatment. An ERCP revealed that both intra- and extra-hepatic bile duct were moderately dilated, and the diagnosis of congenital biliary dilatation with intra-hepatic involvement wa established. She established. She underwent an excision of the dilated extra-hepatic bile duct with hepaticojejunoistomy (Roux-en-Y) and did well thereafter. We reviewed symptomatology and diagnosis of our 47 patients with congenital biliary dilatation, and found that the proportion of early cases without a palpable mass has increased recently; no cases presented with an abdominal mass in the last five years. The importance of the detection of intra-hepatic biliary dilatation by a CT-scan and ultra-sonography in such patients was emphasized.
The high mortality in infants with pulmonary artery sling is due to the coexistence of this condition with tracheal stenosis, bronchial stenosis, or both. The association between pulmonary artery sling and long segment tracheal stenosis, which is called "ring-sling complex" is an uncommon and lethal congenital anomaly. A two-month-old boy with severe respiratory distress due to the ring-sling complex was transferred to our hospital. He underwent surgery three differrent times. The first operation was a left pulmonary arteriopexy. A tracheoplasty with a pericardial patch was performed as the 2nd operation. The narrowing of the trachea was due to complete cartilagenious rings. After this operation, his respiratory condition became somewhat better. But twelve days after the operation, suddenly developed respiratory difficulty and the pericardial patch ruptured. The third operation done was a esophageal wall tracheoplasty. However, he died three monthe after the first tracheoplasty. The operative procedures for the ring-sling complex and pulmonary artery slings reported in Japan were reviewed.
Diaphragmatic paralysis due to the phrenic nerve injury occurred in 4 out of 98 closed cardiac surgical procedure. All four were less than 3 months of age, and three of them were after Blalock-Taussig shunt operation and one after pulmonary artery banding. One after Blalock-Taussig operation who did not undergo plication of the diaphragm died of severe hypoxemia. The other one after pulmonary artery banding tolerated well conservative treatment for left diaphragmatic paralysis and required more than 50 days until dismissal. Remaining two underwent surgical plication of the diaphragma in early postoperative stage and resulted in early extubation and dismissal.
Three-year-old girl complained fever, caugh, dyspnea and chest pain. Plain chest X-ray film on admission showed a mass in the left lower lung area and a coin lesion in the upper area of the same lung. The left lower lobe was occupied by a huge tumor which was resected and a single mass locating in the upper lobe was enucleated. Both of these tumors were histologically proved to be rhabdomyosarcoma, pleomorphic subtype. She has been treated with pulse VAC and adriamycin and has now been free from disease for seven months since surgery. A mass in the left upper lobe was supposed to be a matastasis from the lower lobe. Rhabdomyosarcoma originating from the lung is quite rare and only nine cases have been reported in the literature. Furthermore, this case is quite characteristic in histology and the site of metastasis. Reviewing the past reports, rhabdomyosarcoma in children were embryonic subtype in histology and the metastases were found in the following frequency; lymph nodes (33%), central nervous system (19%), liver (14%), the other side of the lung (10%). Our case will be the first report of a solitary metastasis to the ipsilateral lung.
Congenital dilatation of biliary duct with anomalous pancreatico-biliary ductal junction was found in one of a monozygotic twins. The patient was the second baby of the twins with a birth weight of 2,190 g. She developed abdominal pain and vomiting at the age of 2-year-old. Endoscopic retrograde cholangio-pancreatogram revealed cogenital dilatation of biliary duct with anomalous pancreatico-biliary ductal junction. She underwent resection of the dilated extrahepatic biliary duct and gallbladder and hepatico-jejunostomy with Roux-en-Y anastmosis. Endoscopic retrograde cholangio-pancreatogram on the older sister showed normal pancreatico-biliary ductal junction. Genetic factors may have little importance for the causes of the disease in this case.
Among 194 cases of hepatic tumors, 144 hepatoblastomas were mainly analysed in this report. Tumor-free two-year survival rate was used for evaluating prognosis. All of hepatocarcinoma was fatal in two years, in spite of the treatments. Analysis was focused on hepatoblastoma. On operation, total resection and non-total resection of the primary hepatic tumor were compared in relation to age, operation method and operative treatment of lymph node. In age, tumor-free two-year survival rate was significantly greater in total resection than in non-total resection. Second look operation was performed only in 9 cases, being too small in number for analysis. The irradiation rate was 12% (16/133). The effect of irradiation was not demonstrated in any way. On chemotherapy, tumor-free two-year survival rate was analysed. In post-operative therapy, CPM, 5FU, ADR, MMC and VCR were compared in various doses. The group without use of the agents was always significantly better than the group with use of them.