Functional radiological examinations of rectum and anal canal before and during defecation were performed using a video-tape recorder system in 20 children with normal defecation aged 2 months to 14 years old. Following results were obtained. 1. Rectal sensation was mesured by means of the amount of 150% Barium sulfate injected into the rectum. The average amount was 100ml (40-150ml) in 10 infants aged 4 years to 11 years old. There were no correlation between the age and the amount of Barium in these ages. 2. The anterior wall of anorectum protruded softly in girls and became remarkable in older age. 3. At resting state, the anorectal angulation became sharp by age, and it reached at same level (60°-90°) after 5 or 6 years old. 4. By squeezing anal sphincter muscles, the length of the anal canal became 1.2-1.5 times longer than that of resting state. 5. Two types of defecation were observed, one was reflex type and another was straining type. Reflex type of defecation, which was mainly observed in younger age group, was followed by the contruction of the rectum. Straining type of defecation, which was mainly observed in older age group, was accomplished by abdominal pressure of straining. 6. In normal fedecation, no leakage of Barium was observed throughout the injection and resting period. These results will make it reasonable and clear to assess postoperative problems of defection radiologically.
Cholangiogram of postoperative uncorrectable biliary atresia has shown abnormal intrahepatic bile ducts and it was classified into three types; invisible, radiating and main duct. Experimental cholangiogram was performed on fetal rabbits to study development of bile ducts related to the pathogenesis of the abnormal intrahepatic bile ducts in biliary atresia. Cholangiogram was done on fetus of 17 days gestation and thereafter by inserting tube into stomach or gallbladder under microscope. Cholangiogram was impossible on fetus of 17 days as intra- and extrahepatic bile ducts were filled with solid cells proved histologically. On 18 days gestation, vague shadow continueing to extrahepatic bile ducts appeared at the porta hepatis into the liver, but no main intrahepatic bile ducts could be recognized. On 19 days and thereafter, apparent main ducts could be observed. It means that the intrahepatic bile ducts opens at 18 days and develops to show main ducts after 19 days of gestation. Histoligic study of the liver of human fetus revealed that 6 weeks fetus is apparently like that of rabitts of 18 days in that opening of extrahepatic bile ducts is finished but only a few short branches of intrahepatic bile ducts are just open. These results suggest the abnormal pattern of bile ducts in biliary atresia may be attributed to the developmental abnomality due to pathogenic process that occured on various different developmental stages, may be 6 weeks of gestation and thereafter to interrupt the development of biliary system.
Twenty newborn patients with birth asphyxia, who were operated during neonatal period, were reviewed and their problems in perioperative period were discussed. Among 112 patients who had various surgical procedures for congenital gastrointestinal anomalies within 14 days of life, twenty patients (18%) showed 6 or less of Apgar score at 1 minutes. Twelve of them were transferred from other hospitals. Six died in postoperative period, but causes of their death were not related to birth asphyxia. Transient myocardial ischemia was noted in four (20%) and six patients developed complications of the central nervous system (30%). It was impressive that the patients transferred for emergency operation necessitated more intensive preoperative treatment than the inborn patients and their prognosis was dependent upon the cardiopulmonary status on admission. In case of asphyxiated newborn patients, particular considerations should be paid for complications of the central nervous system.
Hydronephrosis causing hypertension was first suspected by Friedriks in 1835. Then, extensive investigations on the renal hypertension accompanied by hydronephrosis were carriedb out clinically and experimentally by many authors. Pediatric cases have been also reported by Abeshouse (1941), Garret (1970), Davis (1973) and Chapman (1975). During the past 5 years we have experienced 9 cases of unilateral congenital hydronephrosis in children younger than 8 years of age. Eight of them revealed hypertension associcated with elevated levels of plasma renin activity (PRA). Renal hypertension has normalized in 6 of them soon after urinary decompression as reported by Garret, Davis and Chapman. Renal biopsy revealed increased production of renin granules in the juxtaglomerular apparatus. These results suggested that the elevated levels of PRA are attributed to increased release of renin from the affected kidney. The urinary decompression failed to reduce the renal hypertension in 2 cases. One patient showed severe damage of the hydronephrotic kidney, although some amounts of JGA necessary for elevation of PRA were probably intact. Another patient had been associated with uncontrollable renovascular hypertension secondary to hydronephrosis. The 2 patients had to inevitably undergo nephrectomy. These clinical observations suggest that renal hypertension accompanied by hydronephrosis reflects varying pathological states of the affected kidneys. Therefore, serial blood pressure and PRA determinations performed before and after the urinary decompression appear to be useful for clinical evaluation of the hydronephrosis. I this paper, pathogenesis of renal hypertension accompanied by hydronephrosis and its clinical significance were discussed in relation to our 9 children.
Angiotensin II induced hypertensive chemotherapy has not been reported in malignant solid tumor of childhood. The purpose of this paper is to determine how to combine angiotensin II and cyclophosphamide which is masked compound, because cyclophosphamide has been frequently used for treatment of malignant solid tumor in childhood. Then the concentration of active type of cyclophosphamide in blood and tumor tissue were measured sequentially after injection of inactive type of cyclophosphamide intra venously to A/Jax mice bearing C-1300 neuroblastoma. The peak time which appeared active type of cyclophosphamide in blood and tumor tissue of A/Jax mice was 60 minutes after the injection of inactive type of cyclophosphamide. When angiotensine II induced hypertension was continued during 60 minutes after the injection of inactive type of cyclophosphamide followed above data, the effect of cyclophosphamide to C-1300 neuroblastoma in A/Jax mice were enhanced. This combination chemotherapy of angiotensin II and cyclophosphamide was applied to a 9 year old girl with rhabdomyosarcoma, who had lung metastasis and multiple dissemination of abdominal cavity. Followed this combination chemotherapy of angiotensin II, cyclophosphamide, vincristine and actinomycine D, her abdominal circumference was decreased sooner than after simple type of VAC chemotherapy.
Recently, it has be reported that amino acid residues may be absorbed more rapidly from di- and tripeptide than from free amino acid during the protein digestion. The aim of the present study is to compare the absorption effect of enterally administered oligopeptides and amino acids. Two isonitrogenous chemically defined diets (CDD), one containing a partial enzymic hydrolysate of casein which approximately 67% of nitrogen content was present as oligopeptide (peptide CDD), and the other containing a mixtures of L-synthetic amino acid (amino acid CDD), were administered to Thiry Vella loops of weanling rats. The other composition like carbohydrate, fat, mineral, and vitamin, were just same. The comparison study was done by following parameters; residual fluid volume in the loop, residual nitrogen weight in the loop, plasma amino acid concentrations of portal vein. The residual nitrogen weight in the loop significantly decreased at 15min. (p<0.05), 30min. (p<0.01), 120min. (p<0.01) after the administration in the peptide CDD rats rather than in the amino acid CDD rats. The concentrations of free amino acid in plasma of portal vein were significantly greater at 15 min. (p<0.01) after the administration in the peptide CDD rats than in the amino acid CDD rats. Amino acids which were promptly absorbed in the administration of each CDD, were Thr. Phe. Ala. Ser. in the peptide CDD whereas Arg. Leu. Cys. in the amino acid CDD. These results suggested that there might be nutritional advantages especially in the younger, if peptides rather than L-synthetic amino acids were used as a nitrogen source in chemically defined diet.
Scanning electron microscope (SEM) studies of the liver in animal and adult humans have been reported by many authors recently. However, as far as cholestatic diseases of infancy are concerned, no reports have appeared in literature. The present study extends SEM observations to the luminal surface of the hepatic biliary system in order to clarify among the various cholestatic diseases of infancy. Liver biopsy specimens from 50 patients [26 with biliary atresia (BA), 4 with intrahepatic cholestasis (IHC), 3 with paucity of intrahepatic bile ducts, 12 with choledochal cyst and 5 normal controls (1mo. 7mo. 1y. 2y. 54y.)] were studied. 1) In the cases studied with choledochal cysts, hepatic cells were already transformed into one-cell-thick plate in all cases more than 2 years old, and bile canaliculi (BC) and interlobular bile duct (BD) appeared to be normal. In the cases with dilated intrahepatic bile ducts, BC and BD appeared to be almost normal. When jaundice presented as acomplication just before or at the time of operation, however, the evidence of secondary damage was observed at the level of BC and BD. It is therefore suggested that pathological changes a ssociated with choledochal cysts in located at the large extra- and intra-hepatic bile ducts. 2) In IHC, BC dilatation and disappearance of the BC microvilli was demonstrated.In contrast, BD showed and almost normal appearance. It is therefore suggested that regurgitation of bile in IHC occurs at the level of the BC or canaliculo-ductular junciton (C-DJ). 3) With paucity of intrahepatic bile ducts, pathological change of the BC appeared to be similar to that of IHC. BD was partly abnormal. Discontinuity of BD was demonstrated in some portions of the liver in a few cacses. 4) In BA the majority of cases at the time of the initial operation had two-cell-thick plates of liver cells, the same as that of normal controls less than one year old, BC and BD were moderately injured. At the second biopsy of the liver 2 or 3 months after the successful initial operation, marked improvement of BC was observed, and BD had recoverd to some degree. Improvement of BC suggests that obstruction of bile does not occur at the level of neither the BC, C-DJ nor bile ductules. At the third biopsy of the liver 2 or 3 years after the operation without cholestasis, BC were almost normal and hepatic cells had transformed into one-cell-thick plates, but occasionally in some portions of the liver, two-cell-chick plates remained. In contrast, impairment of the microvilli and cilia of the BD was still evident in all cases, especially in those cases complicated with postoperative cholangitis. Therefore the biliary system in BA does not show complete resolution 2 or 3 years afters operation.
Transfusion in utero for a fetus dying due to Rh incompatibility was first attempted back in 1960's. With an introduction of ultrasound into a modern obstetrics, this fetal management in utero has further developed into a totally new area in perinatal medicine. In 1980, hydrocephalus was first decompressed in utero using an ultrasound-guided percutaneous catheterization technique, and so was hydronephrosis in 1981. Animal experiments to define the feasibility of surgical correction in utero for other congenital anomalies, eg. congenital diaphragmatic hernia, spina bifida, Intrauterine Growth Retardation, has also been undertaken. The fetal treatment project, however, has raised various questions, eg. risk vs. benefit, ethical issues and so on. This paper summarizes the recent movement in fetal treatment in the United States.
High incidence of biliary tract carcinoma in cases of choledochopancreatic long common channel syndrome (CPLCCS) is now widely recognized including our study which twelve carcinomas were found in 74 cases of CPLCCS. However as to the average age of patients with biliary tract carcinoma, no significant difference was found between patients with CPLCCS and the patient without CPLCCS. As an experimental animal model of CPLCCS, choledochopancreatic end to side ductal anastomosis was successfully performed in one hundred puppies, which pancreatocholedochal reflux occured in all. Histological findings of bile duct wall were mainly hyperplastic one and no malignant change was found. The action of pancreatic juice to the bile duct wall is very mild and chronic, however the postoperative period became longer, the histological changes became more remarkable. Therefore pancreatocholedochal reflux requires either a long time or other etiologic factor (for example a biliary infection) in order to produce a biliary tract carcinoma.
We have experienced 5 children with massive necrotic change in the intestine, and were able to preserve as much intestine as possible by the method of second look operation.This paper reports these five cases and gives an evaluation of the method and its efficacy. Since it is difficult to make a clinical determination of the viability of an injured intestinal segment at the time of one stage operation, importance of perioperative intensive care, is stressed. Intestinal exteriorization should be considered depending on the general condition and length of the injured intestine.
Barium enema studies revealed that postoperative mucosal prolapse in patients with ano-rectal abnormalities is entirely due to abnormaly elongated distance of the anal canal between the attachment of the puborectal sling and that of the external anal sphincter. Anoplasty for these patients was done to remove the elongated portion of the anal canal and to approximate the distance between the puborectal sling and the external anal sphincter muscle, without use of sliding skin graft methods. Our anoplasty was used 23 times in 19 patients, and gave satisfactory results.
Purpose of the present study is to evaluate the effect of cold potassium cardioplegia in pediatric open heart surgery. Since January 1980, cold potassium cardioplegia has been used in 42 consecutive patients less than 7 years old undergoing correction of various congenital heart diseases. In comparison with previous method of intermittent aortic cross clamping, aortic cross clamp and the use of cold potassium cardioplegia reduced the operative mortality (9.5%), the incidence of postoperative innotropic requirement (35.7%) and postoperative serum CPK levels. The incidence of spontaneous recovery of cardiac action after releasing aortic cross clamp, also increased to 81%. Judging from significantly higher activities of postoperative serum CPK and CPK-MB isozymes in children compared with adult, however, need for further improvement of the method of cardioplegic myocardial protection was suggested.
In order to determine the balloon size for IABP in children, measurements of the aorta were made by cineangiography. The data revealed linear correlations between the internal diameters of the descending aorta and body length. In five patients who were from 6 months to 5 years after open heart surgery IABP was used. In all patient there was appreciable effect of IABP on the arterial pulse contour. Maniaturization of the equipment has permitted IABP to be used effectively in pediatric patients.
A total of 123 children underwent inguinal explorations for nonpalpable testes. Unilateral absence of testis (monorchism) was encountered in 70 children. The left testis was absent in 80% and spermatic vessels with or without vas deferens were present in 76% of them. Fifty-three children (66 testes) had undescended testes. Thirty-eight testes were located intra-abdominally. Orchiopexy was performed to 48 testes, and 18 testes were removed. Techniques for preoperative localization of the nonpalpable testis include pelvic pneumography, inguinal herniography, gonadal venography, C.T.scanning and laparoscopy. But their use is considerably limited in children and is not recommended as a routine procedure. Absence of testis is proved by definitive indentification of blind-ending spermatic vessels at surgery. A bling-ending vas or epididymis does not have the same connotation. When the testis is found intra-abdominally and does not descend readily by conventional orchiopexy techniques, either testicular vessel transection (long loop vas orchiopexy) or staged orchiopexy can be chosen. The former technique must be decided early so that a wide peritoneal strip is left attached to the vas and the testis to preserve vasal blood supply. Orchiectomy is indicated in only carefully selected cases.
Pediatric day care surgical programs have been actively utilized in the United Kingdom since the early 20th century. Recently, day surgery of pediatric inguinal herniorrhaphy is carried out in Japan. Advantages of this day surgery program are cost saving, improved utilization of staff and hospital facilities, and reduction of stress for the pediatric patient and his family. But postoperative pyrexia (≧37.5℃) makes it difficult to discharge on the day of operation. In order to practice day surgery of pediatric inguinal herniorrhaphy safely, records of 187 children were reviewed. 96 patients were febrile and 91 patients were afebrile postoperatively. Statification according to body weight, duration of NPO, duration of operation, duration of anesthesia, intraoperative infusion volume and postoperative infusion volume revealed that mean body weight of febrile groups (8.77±2.90kg) was significantly smaller than that of afebrile groups (14.88±5.29kg, p<0.005). Then, it is concluded that patient under about 10kg weight is apt to be febrile postoperatively and that body weight limitation is more suitable than age limitation for the selection of children when day surgery of pediatric inguinal herniorrhaphy is carried out.
Pulmonary aspergillosis is not rare in adults but is rarely encountered in children. Especially with the exception of the allergic type, pulmonary aspergillosis is very rarely described in literatures. We encountered a case of aspergillus pulmonary abscess in a 7-year-old girl occurred from recurrent pneumonia. At first, she developed pneumonia in June, 1982, and despite antibiotic treatment, radiological examination in May, 1983 again revealed an abnormality. Lobectomy of the right lower lobe was performed under the diagnosis of pulmonary abscess in Aug., 1983. Aspergillus fumigatus was detected in the fluid from the cavitary dilated bronchus. On the basis of this finding, the diagnosis of aspergillus abscess of the lung was established. The postoperative course was uneventful the patient was discharged 3 weeks after the operation. She has been well without any evidence of recurrent pneumonia.
An 11 year old girl, diagnosed as tyrosinosis at the age of 8, developed right upper quadrant pain. Liver scintigram showed space occupying lesion in the right lobe. The serum alpha-fetoprotein was also elevated. At laparotomy the liver showed cirrhotic and right hepatic lobectomy was carried out. Histopathologically tumor was adult type hepatocellular carcinoma and liver tissue showed macronodular cirrhosis. The serum alpha-fetoprotein level decreased rapidly following the operation. No adjuvant chemotherapy was performed because of impaired hepatic function. Four months later, alpha-fetoprotein level began to increase with signs of relapse and intra-arterial chemotherapy was attempted. However, its side effects made it impossible to continue the chemotherapy. The patient died of progressive disease 9 months after the operation. In Japan 19 cases of tyrosinosis have been reported but hepatomas were associated only in 2 cases of them. In these 2 cases the tumor was found at autopsy. The present case is the first one operated in Japan. Chronic form of tyrosinosis is always associated with liver cirrhosis and is often complicated with hepatoma. Although hepatocellular carcinoma in infancy and childhood is rarely associated with cirrhosis, patient with chronic form of tyrosinosis is the "high-risk" patient against hepatoma. The serum alpha-fetoprotein mesurement and other radiological diagnostic procedures are helpful to detect hepatoma in these patients.
Atypical fibrous histiocytoma is classified as an intermediate type of fibrous histiocytoma. A five year-old girl had a painless tumor in her right lumber region following blunt injury on this site. Computed tomography revealed a subcutaneous tumor extended to the major psoas muscle. An extensive excision of the tumor and surrounding normal tissues were carried out. Histological examination revealed atypical fibrous histiocytoma. Neither postoperative irradiation nor adjuvant chemotherapies has been made. She is doing well without any sign of recurrence for one and a half years after the operation.
A twelve-day old girl was transfered to Sendai Red Cross Hospital with an abdominal tumor. Computer tomography scanning and echogram showed a large irregular solid mass at the presacral region. Exproratory laparotomy revealed a large tumor arising from presacral retroperitoneal region and a specimen was taken. Histopathological, histochemical and ultrastructural examinations identified the diagnosis of malignant schwannoma. Actinomycin D was given in the amount of 10μg/kg intravenously daily for 5 days. The day after initiation of chemotherapy, rapid decrease in white blood cells and platelets resulted bleeding tendency, general convulsion and finally caused a death of this patient. Usefullness of histochemical and electronmicroscopical examinations in the diagnosis for neurogenic tumors is discusseed in relation to this case.
Two patients with type A Interrupted Aortic Arch (3 days old and 12 months old) underwent staged operation. Aortic arch reconstruction with Blalock-Park's method, PDA division, and PA-banding were performed as the first operation. Subsequently one of them underwent the second operation (VSD closure and debanding) and is now doing well. The other baby is growing well and the second operation is not yet performed because of aortic valve deformity and possible need of valve replacement. Postoperative pressure gradient across the Blalock-Park's anastomosis was slight and perfusion to lower half of the body was favorable. There also recongnized the growth of the reconstructed aortic arch on the postoperative aortography. As the preoperative condition of patients with interrupted aortic arch is usually severe, staged operation is recommended. Aortic arch reconstruction with Blalock-Park's method, PDA division, and PA-banding were thought to be a preferable method as the first operation.