Histochemical staining of acetylcholinesterase (AChE) activity of rectal suction biopsy is a reliable method in the diagnosis of Hirschsprung's disease. Diagnosis of Hirchsprung's disease can be established by the increased AChE positive nerve fibers in the inucosa and submucosa of the rectum. But there have been several reports on the presence of false negative reaction. Some of these may depend upon technical fault such as an insufficient incubation solution. We have applied an improved AChE staining technique using rubeanic acid to 32 children with constipation. Eleven cases were confirmed to have Hirschsprung's disease by this method. No false positive or negative reaction was encountered. The sites of AChE activities were observed as black deposits with sharp contrast. This new method will be expected to be widely used for the basic and clinical study of Hirschsprung's disease.
Varous disorders, according to the anomalous pancreatico-biliary ductal union, have beer supposed to be due to the refluxed pancreatic juice into the biliary tree. But the real causes of these disorders have not been clarified so far. In the successive study on the canine models, we used two kind of models: Pancreatico-choledochostomy (P-Ch) and Pancreatico-cholecystostomy (P-Cy). Twenty five P-Ch models and 11 P-Cy models were used in this experiment. Bile juice was collected from gall-bladder in re-laparotomy in one to twelve months after the model-producing procedure. In the daily study on a P-Ch model, the amylase value was changing remarkably, suggesting uneven reflux of pancreatic juice through the biliary tree. Besides the irregularity of zymogen volume, the activity of trypsin was unsettled in this model. It was almost nothing in many instances while the trypsinogen of high volume level as 400-500×10^4U/ml equivalent was kept inactive. In the daily study of a P-Cy model, the amylase and the activated trypsin were kept constantly in high level. Inactive trypaicnogen was kept very little in this model. The direct secretion of pancreatic juice into gall-bladder would be the cause of this phenomenon. The correlation of the amylase and the secreted trypsinogen amount was studied on 31 bile samples of P-Ch and 22 samples of P-Cy. In both of the models, the amylase and the secreted trypsinogen value were correlated. (r=0.596, r=0.640) The activated trypsin ratio among the whole secreted trypsinogen was different in each model. In P-Ch, the activation was very little in the samples with greater amount of whole secreted trypsinogen volume. On the other hand in the bile with small amount of secreted value, the activity was almost full. We concluded that the activation of trypsin and elastase in bile were proven in most of the canine models. And the activation values were changing, time to time. In bile with greater amount of secreted zymogen, the activation was always little. We suggested on the dangerous process in cyst-duodenostomy, according to the result of the experiment. Great amount of trypsinogen and other zymogen in bile would be explosively and completely activated by enterokinase drained from the duodenum, when the cyst-duodenostomy was performed.
Studies were undertaken to evaluate the radiotherapeutic response of five human tumor lines serially transplanted in nude mice (BALB/c, nu/nu). They included a neuroblastoma (CH-NB-1), Wilms tumor (CH-W-4), hepatoblastoma (CH-HB-1), yolk sac tumor (CH-YS-l), and rhabdomyosarcoma (CH-RS-2). Their histological appearance and growth rates were stable, with volume doubling times ranging from 3.5 to 6.0 days. When the therapeutic response was measured by growth-delay method, there were wide differences in response to radiotherapy. Neuroblastomas and yolk sac tumors responded well; Wilms tumors and hepatoblastomas showed modest response; rhabdomyosarcomas responded poorly. The results were similar to those reported in the patients treated with the same agents. Studies of in-vivo cell survival were made in hepatoblastoma xenograft line (CH-HB-1). The survival curve showed an initial sensitive component, a Do of 1.1 Gy and a radioresistant component, a Do of 3.4 Gy. This suggests that there is hypoxic cell fraction which is radioresistant in this tumor.
In the poor bile flow (10 cases), the initial bile flow after portenterostomy (KASAI-SAWAGUCHI) contained low levels of total bile acids and high levels of lithocolic acid, deoxycholic acid and free bile acids, Urusodeoxycholic acid was detected at a high level and free bile acids were still maintained at high levels 4 weeks later. In the good bile flow group (3 cases), jaundice disappeared, but the total bile acid level was low and the urusodeoxycholic acid level was high. The composition of bile acids in this group was similar to that observed 4 weeks later in the poor bile flow group. These results suggest that abnormal metabolism of bile acid in the liver cell and disturbance of bile secretion at the level of bile canaliculi, especially that of bile acid-dependent bile secretion, may continue after KASAI'S operation for congenital billiary atresia.
The histopathological findings as well as clinical and follow-up data in 7 patients with adult-type liver cell carcinoma and in 17 patients with hepatoblastoma have been analysed and discussed particularly with regard to histopathological prognostic factors. All 7 patients with adult-type liver cell carcinoma died of cachexia with metastases to the lungs and lymph nodes. Primary liver tumors in these patients were of a morphologically diffuse type without pseudocapsules. Vascular invasion was noted in all of them. Nine of hepatoblastoma patients are alive and well without any evidence of recurrence; they have survived for periods from 1 to 17 years following resection of primary or metastatic tumors. Fifteen hepatoblastomas had pseudocapsules and 7 patients with tumors having no capsular invasions are all alive. Five of 6 patients with pure, well-differentiated hepatoblastoma are all alive, exhibiting excellent prognosis. Five of 7 patients with hepatoblastoma accompanied by vascular invasion died mainly because of metastases to the lungs. The histology of the neoplastic tissue in vascular invasion and metastatic foci of these patients was mainly of a poorly differentiated variety. Regardless of the extent of predominance in histologic differentiation, patients with portions of poorly differentiated foci should be followed up very closely by observing the serum level of α-fetoprotein. Through reference to histological types and the presence or absence of capsular and vascular invasion, as some of relevant prognostic factors, the prognosis of primary liver carcinomas in infancy and childhood can be envisaged
Anorectal manometry was carried out in 57 newborn infants using an infused side-opening catheter. Twenty-eight babies were preterm (gestational age less than 37 weeks) and 28 low birth weight (birth weight less than 2,500gm). The examination was carried out 100 times between 8 hours and 43 days of age. Histometry of the intramural ganglion cells of rectum was also carried out in 11 atitopsied cases, of which 5 were preterm infants. The results of the present studies disclosed that: 1. Anal canal pressure is low in new born infants within 3 days after birth, but there is no correlation between anal canal or rectal pressure and total age (gestational age plus age after birth in weeks). Length of the anal canal and its frequencies of basal rhythmic contraction increase with the advance of total age and body weight at the time of examination. There is no correlation, however, between the length of the anal canal or its frequencies of basal rhythmic contraction and the age in weeks after birth. 2. Rectoanal reflex was positive at the first examination in 53 of 57 cases. Rectoanal reflex was, however, negative or atypical at the first examination in 4 cases. Typical rectoanal reflex appeared at the second or third examination in all these 4 cases. Rectoanal reflex was positive in all 38 examinations carried out in those of which the total age was over 37 weeks and body weight was over 2,500gm. On the other hand, the reflex was negative or atypical in 5 of 41 examinations carried out in those of which the total age was under 36 weeks, or in 5 of 58 examinations carried out in those of which body weight was under 2,500gm. 3. The mean diameter of nuclei of ganglion cells in intramural plexus in 3 cases whose total age was over 41 weeks was significantly larger than in 4 cases who was below 32 weeks. The mean diameter of nuclei of ganglion cells was variable in 4 cases of which total age was between 33 and 40.
Coexistence of intrahepatic bile duct dilatation is not so uncommon in congenital dilatation of the bile duct. Intrahepatic bile duct dilatation was disclosed by operative cholangiography in 39 of the 47 patients who had undergone radical operation of choledochal cypt at 2nd Department of Surgery, Tohoku University Hospital. Primary excision of the cyst was employed for these patients and intrahepatic bile duct involvement of the bile ducts had been left at the time of operation. In follow-up studies ranging 1 to 12 years after the operation, none of these 39 patients had complication such as ascending cholangitis, intrahepatic lithiasis and cancer arising from intrahepatic bile ducts. Ultrasonography of the liver taken at follow-up study revealed no dilatation in all of cases showing fusiform dilatations and in 7 of 9 cases showing cystic dilatations at the time of radical operation. The size of 2 cases of giant cystic dilatation of intrahepatic bile ducts scarcely unchanged. The results of these follow up studies will justify an employment of free drainage procedure at the porta hepatis for surgical treatment of congenital dilatation of the bile duct. Careful follow-up study should be required, however, when patient has a huge cystic dilatation of the intrahepatic bile duct.
Surgical treatment of Hirschsprung's disease has well been established and good results were reported in various operations. But there have been few reports on secondary operations following the primary bowel resection. From 1963 to 1983 we have experienced 129 cases of Hirschsprung's disease, of which 14 cases required second operation. The failed first operations included the following procedures; Duhamel in 3 cases, modified Duhamel in 5 cases, Z-shaped anastomosis in 3 cases, and anorectal myectomy in 3 cases. 6 cases treated by Duhamel's or its modification by Grob developed rectal blind pouch with a fecalona. Clushing of the septum or Z-shaped anastomosis were effective for these cases. Preoperative digital dilatation of the anal canal is essential before using oval shaped clushing clarnp. Carefull preparation of the bowel should be performed preoperatively because complications such as intestinal fistula, abscess formation, anal stenosis, result in bad anorectal function in spite of the secondary repair. In retrorectal transanal pull-through procedures, posterior half transverse incision should be placed at dentate line for the release of the sphincter achalasia. The rate of re-operation following anorectal myectomy was high (42.9%) in our own cases.
The records of 48 patients with gastrointestinal and biliary tract perforations experienced at the Department of Surgery I, Gunma University school of Medicine and the Department of Surgery, Gunma Children's Hospital Medical Center during the period from 1956 to 1983 were analysed. Thirty-four of 48 patients were newborn infants and nine were infants beyond the neonatal period. Anatomic distribution of the perforation showed the small intestine (19 patients), the stomach (12), the large intestine (6), the duodenum and the biliary tract (4 each) in order of frequency. In the remaining three patients, the site of perforation was not identified. An over-all survival rate accounted for 69 per cent. However, during the last 7 year period, a survival rate in newborn infants has been improved from 59 per cent to 71 per cent. The prognosis was closely related to factors such as age at onset, birth weight, etiology and the site of perforation, interval between the onset and definitive treatment, degree of preoperative acidosis and urinary output. However, increased incidence of survivals achieved in the recent years must be mainly due to improved pre- and postoperative care, especially fluid and respiratory control. It is also suggested that the diagnosis in the stage of impending perforation and avoidance of perinatal stress are essential of further improvement of prognosis.
In the treatment of biliary atresia, postoperative good bile excretion is the first step to provide a cure. According to the reported results of various workers in 1978, the average rate of successful bile drainage was 62%, and the rate of disappearance of jaundice accounted 41%. The purpose of this paper is to introduce our new surgical procedure for the establishment of higly successful biliary drainage and the classification of topographic anatomy of porta hepatis for the dissection. For the successfull biliary drainage, a superextensive dissection of the portahepatis, including the area between the second branches of right portal vein, was carried out, with placing a tape around each branch of the hepatic artery and portal vein, so that intestinal stoma for anastomosis could cover up the whole area where the potentially usable remnants of hepatic duct were present. Since 1978, fifteen infants with non-correctable type of biliary atresia have undergone the superextensive dissection of portahepatis. In the topograpic anatomy of portahepatis, usual simple branching of portal vein into right and left portal branches (Type A) were noted in only 5 Infants. Nine infants had a lateral branching of right portal vein (Type B), and remaining one infant had a more complex branching (Type C). In type B and C, posterior row of the anastomotic line was made invaginate the medial branches of portal vein. We were able to achieve the successful bile drainage in all 15 infants. The disappearance rate of jaundice became 80%.
Of 60 patients of tracheoesophageal fistula who have been treated during the past 13 years, four were subsequently found to have an association of esophageal stenosis of congenital origin in the distal esophagus. The patients have developed obstructive symptom when feeding of solid food was began several months after repair of tracheoesophageal fistula. The contrast study was diagnostic in these patients. Three patients had musculofibrous stenosis and one had tracheobronchial remmant. Three patients have undergone a surgical repair and one has been treated conservativelyy with several dilations. Attention has been focused on the anastomotic stenosis in these patients, which was the cause of delay of correct diagnosis and therapy. When the patient develop esophageal obstruction after repair of tracheoesophageal fistula, a careful investigation will be needed for an association of esophageal stenosis.
During six years period from 1977 through 1982, five female infants with imperforale anus and rectocloacal fistula were treated in our institution. From the experiences obtained by the management of these cases, our conclusions are as follows; 1. Simultaneous three organs radiogram was very important not only for the exact diagnosis but also for the choice of surgical procedure. 2. For cases of low confluence in the radiogram, which showed the vagina, urethra and rectum communicated above I line, Hendren's perineal vaginoplasty and anoplasty were indicated. Its operative results were excellent. 3. For cases of high confluence in the radiogram, which showed the vagina, urethra and rectum communicated above P-C line, combined abdomino-perineal vaginal and rectal pullthrough were indicated. 4. Raffensperger's neonatal vaginal pullthrough was performed in the case with huge hydrometrocolpos, whereas in the case of normal sine vagina, Hendren's abdomino-perineal vaginal pullthrough was performed. Hendren's operation is very meticulous procedure and might have some risks of injury to innervation to bladder neck. More investigation should be made to abdomino-perineal vaginal pullthrough.
Fifteen cases of recto-bulbar fistula were reviewed. Twelve cases had a fistula entering into the bulbous urethra below the membranous urethra, whereas 3 had the fistula entering into the penile urethra. The latter is called an unusual type. Radiographic findings of the rectum ending below the I-line and the presence of a long fine fistula entering into the penile urethra are characteristic to this type of anomaly. It was interesting to recognize several vertical folds in the mucosal surface of the blind end of the rectum which might be called "anal crypt" in these cases. Since similar finding was observed in most low type deformities including covered anuscomplete (7 out of 8 cases), ano-cutaneous fistula and anal membrane stenosis, the unusual type might had better classified in the low type anomaly. Although these three unusual cases were treated by sacro-perineal approach described by Stephens, et al., they might be operated perineally even in the neonatal period avoiding colostomy. Management of a long fistula to the penile urethra remains to be solved in treating these cases perineally.
A review of 47 surgically sick newborns transported by our general surgery team in 1982, is presented and primary prehospital care is discussed. These 47 transferred newborns (during the first 28 days of life) accounts for 65% of 72 newborns entered into oure surgical wards, 35% of 136 newborns admitted to out neonatal intensive care units, and 22% of 209 cases transferred by our hospital-based ambulance. Forty-five of these 47 eases were less than 7 days of age and 39 cases (83%) were referred directly from obstetricians. About half of them were transported before 8: 30 or after 16: 30, so 24-hours-a-day transport service is required. In 47 referrals, 28 cases had undergone emergenncy operations within 6 hours after hospitalization, and 12 cases were operated upon electively. Almost all of them showed acute abdominal conditions or obvious malformations and were transported without trouble by simple means, such as placing a gastric tube and being kept under optimal thermal environment. But in only a few cases (Bochdalek hernia and gastric rupture), cardio-pulmonary critical care was needed. It must be mentioned that the transportation of surgically-ill newborn by pediatric surgeons has some benefits for making an early diagnosis and supplying optimal preoperative care for the early stage of surgical conditions.
Clinical and anorectal manometric assessment of bowel control was done in 8 patients who had undergone surgical intervention for meningomyelocele 2 weeks to 6 months before. Of 6 patients with closed skin surface, 4 were with normal bowel control. On the other hand, 2 patients with open skin surface were with constipation or frequent bowel movement. Three out of the 4 patients with normal bowel control showed the normal patterns of anorectal resting pressure and anorectal reflex. Two patients with constipation showed the hypotonic pattern of anal resting pressure, and another 2 patients with frequent bowel movement characteristically had the exaggerated pattern of anorectal reflex. Frequent bowel movement could be controlled by rectal suppository or enma. Therefore, the achievement of satisfactory fecal continence in patients with meningomyelocele is accomplished by prevention of constipation. In addition, further careful follow-up study is necessary.
We have studied the course of spontaneous resolution of pancreatic pseudocysts due to trauma in a patient using a series of ultrasonic examinations. The pseudocysts had been formed and were completely resolved six days and seven weeks, respectively, after the trauma. Serum amylase of the patient varied in proportion to the size of the pseudocysts.
Antral dysmutility is a disease entity which was first described by W. J. Byrne in 1981. Its main symptom is chronic vomiting in infancy and unique radiographic findings in the antrum on uppergastrointestinal series. Normal or elevated lower esophageal sphincter pressure and absence of reflux with one hour intraesophageal pH monitoring are reported to be characteristic findings. Recently, we experienced a male infant of four and half month old. By upper gastrointestinal radiographic, endoscopic and esophageal motility studies, he was diagnosed to have antral dysmotility and was treated conservatively with cure. He seems to be the first case of antral dysmotility reported in the Japanese literature.
A unusual tumor involving the left lobe of the liver seen in a young child is reported, which was difficult to differenciate from malignant hepatic tumors. We were able to find only three previous reports of a similar case in the literature. A malignant pattern was demonstrated by both angiographic and operative findings. Only histologically, the features of inflammatory pseudotumor was diagnosed. There were localized benign proliferations, composed predominantely of plasma cell and other elements supported by fibrous stroma and unusual vascular changes, which was designated as occulusive phlebitis. Now, it was concluded that these lesions are nonneoplastic' but of unknown etiology. On following up a year later, the patient was free from disease without any therapy.
Most of mediastinal teratoma is known to occur in the anterior mediastinum. The authors have encountered a case of teratoma in the posterior mediastinum. The patient is six year old male. He was admitted to our department because of an abnormal shadow in routine chest X-ray examination. The tumor was removed succesfully and histological findings were of mature teratoma. Post operative course was uneventful.
A 2yr-10mo-old-boy was admitted to the Yamagata City Hospital Saiseikan because of severe abdominal pain and high fever. A dark-red colored cystic tumor, 24x181x3 cm, arising from the lesser omentum was removed. Histological findings disclosed that this tumor was lymphangioma.
Congenital tracheal stenosis is an extremely rare lesion and resection of tracheal stenosis end to end anastomosis has been rarely reported. This paper reports the successful treatment of a congenital segmental stenosis of the trachea in a 3-month-old female infant and the problem of the tracheal resection in infant was discussed.