Blood ketone body levels and plasma free fatty acid levels during 7 post operative days were measured in 25 patients with minor operation and 62 patients with major operation. The results were as follows: 1. Blood ketone body levels elevated after operation, and the values after major operation were significantly higher and remained high in longer periods than the values after minor operation. 2. Acetoacetic acid, 3-hydroxybutyric acid and total ketone body of the patients with higher caloric intake (more than 50 Cal/Kg/day) were significantly lower than those of the patients with low caloric intake (under 50 Cal/Kg/day) during early post operative days. 3. Ketone bodies levels of the patients with congenital biliary atresia were lower than those of the other patients with major operation. Moreover, free fatty acid/ketone body ratio of the former was greater than that of the latter after operation These suggest that the patients with congenital biliary atresia have limited ability of ketogenesis after operation. 4. Plasma free fatty acid levels were also elevated by surgical insult, and there was a significant correlation between blood ketone body and plasma free fatty acid during post operative days.
Microcomputer-aided data logging and processing system was applied to 24-hr esophageal pH monitoring and its usefulness was evaluated. On 15 asymptomatic infants, the examination was undertaken with this system. The output of pH-meter was input into microcomputor (L-16A) via 8 bit A/D converter. The data were saved into casette MT every hourly for 24 hours. The pH-spectrums in 24 hours and 25 parameters were precisely calculated by the computer afterwards. Gastroesophageal refluxes in these asymptomatic infants were proved to be more frequent than previous reports. The data analysis with this system was shown to be very quick, precise and flexible, that means any newly desired parameters could be calculated easily. This system was considered to be very useful in performing the continuous esophageal pH monitoring and analysing the results.
A large solitary liver cyst in a female infant is reported. The patient was admitted with a complaint of abdominal distension at the age of 5 months. The computed tomography of the abdomen and scintigraphy of the biliary tract were of value, in making the diagnosis of a large liver cyst preoperatively. At operation a 14 by 13 by 11 cm. solitary unilocular cyst was found in both lobes of the liver. The serous fluid contained in the cyst was evacuated and the wall was partially excised. The remaining cyst wall on the liver left in place to communicate with the free peritoneal cavity. The postoperative course was uneventful. A partial excision of the liver cyst seemed to be a safe and effective procedure, especially for a large one involving the both lobes.
We exterienced a case of undifferentiated (embryonal) sarcoma of the liver which is rarely seen in children in Japan. An 8-year-old girl, was surgically treated for a large abdominal mass occupying the dome of the liver and bulging up the diaphragm. The tumor in the liver was removed by extended right hepatic lobectomy. Ten months later, however, the tumor recurred and she died. This paper presented the clinical course and pathohistological findings of undifferentiated (embryonal) sarcoma of the liver seen in our patient, which Stocker and Isak had described.
Hepatoblastoma with precocious puberty is a rare disease. An 11-month-old boy, who had undergone primary resection of the hepatic tumor was readmitted because of a metastatic tumor in the lung and sexual precocity which was caused by excretion of ectopic gonadotropin. Following removal of the metastatic tumor, regression of sexual precocity and decrease of serum gonadotropin have been observed. The gonadotropin of this patient was proved to be Human chorionic Gonadotropin (HCG) by the radioimmunoassay method. He died of cachexia 12th months after the primary operation. The clinical course and hormonal abnormalities are presented and discussed.
A six year old boy was catheterized preoperatively and tetralogy of Fallot with anomalous coronary artery was diagnosed. During operation, he was found to have double outlet right ventricle with subaortic VSD, pulmonary stenosis and the anterior descending coronary artery originating from the right coronary artery. The left outflow tract was made by suturing a internal conduit from VSD to the aorta. The anomalous coronary artery was made free from the surrounding tissue and mobilized to enable a pericardial patch sutured by lifting the artery to relieve the pulmonary stenosis. In young children, the proposed method is preferable compared with other methods employing some types of grafting with a valved conduct or AC bypass procedures.
Carcinoma of the stomach in childhood is extremely rare. Only 6 cases under 10 years of age have been reported in Japan. This paper reports a case of carcinoma of the stomach in a 7-year and 5-month-old boy. This is the youngest case in so-called adult type carcinoma of the stomach in Japan. The tumor located mainly in the cardial region of the stomach. Total gastrectomy with splenectomy and combined resection of the pancreas tail was performed. Reconstruction of the alimentary tract was achieved by esophago-jejunostomy with Roux-Y in retro-colic fashion. Histological study of the specimen demonstrated anaplastic adenocarcinoma. A literal review collected 62 cases of carcinoma of the stomach in childhood under 15 years of age reported in Japan. When there is a persistent gastro-intestinal symptom like epigastralgia and vomiting in childhood, it is essential to consider the possibility of gastric malignancy regardless of the age of the patient.
A successful surgical treatment of congenital cystic adenomatoid malformation in a male infant is reported. The patient was admitted at 20th day of age for severe respiratory distress with pH 7.18, PaO_2 30. 8 torr, and PaCO_2 70.0 torr on May 8, 1980. An emergency left lower lobectomy was performed. His postoperative course was not complicated and he was discharged with complete expansion of the right lung and the left upper lobe on the 14th day after surgery. The lesion in the resected left lower lobe was composed of multiple tension cyst and adenomatoid bronchiolar proliferation. The histological diagnosis was congenital cystic adenomatoid malformation of the lung.
This is a case report of the essential fatty acid deficiency caused bv ED-AC. Three patients of Hirschsprung's disease with a short aganglionic segment were initially treated by ED-AC. All patients progressed well and did not require creation of a colostomy. Definitive operation (Duhamel's procedure) was performed when the body weight reached 5kg. Sparse hair was seen in one of the patients after ED-AC feeding for 2 months. Deficiency of essential fatty acids was thought to be the cause of sparse hair. The patient was then treated with Intralipid, which contains necessary fatty acids. Satisfactory hair growth was seen after the Intralipid treatment. For deficiency of essential fatty acids, it is important to use Intralipid while the patient is managed by ED-AC.
MEN, type III is an extremely rare syndrome. Only six cases have been reported in Japan. Recently we experienced a case with medullary thyroid carcinoma, multiple mucosal neuroma and Marfanoid habitus in childhood, which is the youngest case in Japanese literatures. A 6-year-old boy was admitted with a complaint of left cervical tumors. "Bumpy" lips, Marfanoid habitus and whitish papillary lesions on the tongue tip which was histologically proved plexiform neuroma were characteristic. Megacolon, polyps of colon, diverticulosis coli and medullated corneal nerves were also detected. Under the diagnosis of thyroid cancer, resection of the left lobe of the thyroid with bilateral modified radical neck dissection was carried out. Postoperative calcitonin level remained high (5,674 pg/ml, indicating incomplete removal of the metastatic medullary thyroid carcinoma in the anterior mediastinum. External radiotherapy by 60^Co was performed postoperatively. A series of endocrinological examination failed to find a pheochromocytoma. A careful study of the family history gave a negative result.
Twenty-four-hour distal esophageal pH monitoring (pH monitoring) is one of the most reliable methods for evaluation of gastro esophageal reflux (GER). Thirty nine patients were subjected for a comporative study of pH monitoring and esophageal manometry. The results were scored applying Johnson's scoring method. Thirty nine patients were divided into two groups (normal group and GER group) according to the value of pH score (15.44). The GER group included the patients with gastric outlet obstruction and the patients with tracheoesophageal fistula. So, the GER group was divided into the three subgroups (dys-function of E-C junction, gastric outlet obstruction and tracheoesophageal fistula). Esophageal manometry was compared among these four groups (normal, dysfunction of E-C junction, gastric outlet obstructin and tracheoesophageal fistula). The normal group showed low pH and high manometric score. The group of dysfunction of E-C junction was with high pH and low manometric score. The group of gastric outlet obstruction presented high pH and high manoraetric score. The group of tracheoesophageal fistula was with high pH score because of the esophageal dysmotility, although the number reflux episodes was small. The pH scores in the groups of gastric outlet obstruction and tracheo esophageal fistula did not directry reflect the disturbed function of E-C junction. The pH score in these types of GER is not distinguished from GER due to disfunction of the E-C junction. When an operation for GER is considered, the function of the E-C junction must be evaluated with esophageal manometry and pH monitoring in addition to clinical symptom.
In order to study morphology and prognosis of congenital aortic stenosis, twelve surgical patients aged from 6 to 54 years were reviewed. All patients had the bicuspidal aortic valves. The bicuspidal valves were classified into two types according to the morphological features-symmetric type and asymmetric type. We defined "symmetric type" as that two commissures divide the inner circumference of the valvular ring into equal length and that the two cusps are nearly same size. All other cases were classified as "asymmetric type". Three out of six children showed symmetric type, and other three asymmetric type. Three adults showed symmetric, two asymmetric and one was indeterminable because apico-aortic bypass operation was done in which the aortic valve was not inspected. All six children in both types underwent valvotomy, in which the two commissures were incised to enlarge the valve orifice. All the cases of symmetric type had good post-operative course for 11 to 15 years after the valvotomy. Asymmetric type cases had less satisfactory post-operative course with a tendency of residual stenosis or restenosis. As most of the adult cases had the calcified aortic valves which were replaced by the prosthetic valves and the long-term prognosis of valvotomy was well in symmetric aortic stenosis, we suggest that valvotomy of the bicuspid valves should be performed in the early stage. However, in asymmetric type of aortic stenosis, a new device of valvotomy should be considered.
Four patients of esophageal atresia with a wide-gap (2 cases of Gross' type A, one type B and one type C) were successfully primarily treated using circular esophagomyotomy for elongation of the esophagus. In 3 infants, the proximal and distal esophageal pouches were elongated by bouginage for 5 to 11 months before operation. Esophagomyotomies could further elongate the proximal esophagus by approximately 2 cm at each myotomy. It is thought that combining these two techniques may allow a successful primary esophageal anastomosis in infants with a wide-gap. Furthermore even in newborn babies with T. E. F. with a "wide-gap" recognized at surgery, intraoperative circular myotomy may usuful for primary anastomosis of the esophagus. Operative method and results of follow-up of our patients are discussed.
A fourteen monthes old girl with congenital biliary dilatation initially presenting pancreatit is was reported. The operative cholangiogram via gallbladder demonstrated a very characteristic feature, massive reflux of the rediopaque dye into the pancreatic duct without visualization of the common channel and the duodenum. The patient progressed in satisfactory condition after hepaticojejunostomy (R-Y) after division of the communication between the common bile duct and the pancreatic duct. The choledocho-pancreatic end-to-side ductal anastomosis was successfully performed in 35 puppies as an experimental animal model of the choledocho-pancreatic long common channel disorders. This animal model was considered to be the most similar model to the human pathological disorders. In autopsy specimens, two of 35 puppies showed the most remarkable histologic changes of the pancreatic periductal parenchyma with dilatation of the pancreatic duct. Further-more in these cases, the choledocho-pancreatic ductal junction (anastomotic site) revealed special anatomical condition which the massive bile reflux into the pancreatic duct easily occured. These clinical and experimental results may suggest that the case of congenital biliary dilatation presenting pancreatitis has a special anatomical condition of the choledocho-pancreatic ductal junction which may permit massive reflux of the bile into the pancreatic duct.
Accurate early diagnosis is the most important in the management of patients with appendi citis. When the clinical findings are not diagnostic, a barium enema (B.E.) is a useful and harmless procedure. A series of 53 B.E.s were performed in patients of possible appendicitis to rule out other appendiceal diseases when the clinical signs and findings were not conclusive. Of these 53 patients, 36 who had shown positive B.E. findings before operation were proved to have acute appendicitis by pathological examination of the excied appendix. Thirteen patients with sufficient filling of the appendix were treated conservatively and did well. In only one patient (2.6%), B.E. was not helpfull in diagnosis. There was no complication resulting from this procedure in this series.
The so-called Hatakoshi repair was performed upon 1,368 children (c-6 yr.) from 1966 through 1970; high ligation of the hernial sac with closure of the transversalis fascia was performed upon 1,294 children under the comparable conditions from 1975 to 1980. Contralateral hernial appearance rate was 11.16% and 5.53%, respectively. This significance may be due to the difference of surgical procedure. These results have lead a conclusion that high ligature of the sac with or without a couple of sutures to the transversalis fascia is essential. Procedures which may interfer the valve and shutter mechanism of the contralateral side (e.g., the sutures of the aponeurosis of the internal oblique muscle to the Poupart's ligament) should be avoided.
During the period from 1975 to 1981, endoscopic retrograde cholangio-pancreatography (ERCP) has been attemted for 48 times in 45 infants and children, resulting in successful cannulation in 36 patients. The patients age ranged from 35 days to 15 years. Sixteen patients were congenital choledocal dilatation and various types such as, anomalous junction of pancreatico-biliary ductal system without choledocal dilatation, cholecystolithiasis, infantile hepatitis, pancreatitis, traumatic pancreas injury, Alagilles syndrom and 5 were congenital biliary atresia. In 6 patients, no abnormality was found. The overall success rate in cannulation was 75%. Three types of duodenoscopes, JF-B_2 (Olympus Co.), FGS-PE_a (Machida Co.) and XPJF (Olympus Co.) were employed. FGS-PE_2 and XPGF are the newly designed duodenoscopes for pediatric patients. FGS-PE_2 has been developed in co-operation with our department. The outer diametr of FGS-PE_2 and XPJF are 8.0 and 8.8mm respectively. These two new products of FGS-PE_2 and XPJF, have been available since 1980. Since then, success rate in cannulation has been improved to 100%, including 8 patients under 100 days of age. We have found that these two new duodenoscopes specially designed for pediatric patients are quite useful for ERCP in Infants and Children.