Magnetic resonance imaging (MRI) of the anal levater and sphincter muscles was obtained on 4 normal volunteers and 11 patients with postoperative anorectal malformations (including 8 supra-levator type and 3 low type). Balloon catheter were inserted into the rectum and marked it as the center of a anal canal. Four normal subjects revealed the levater and sphincter muscles were thick and well developed in all sections (Sagittal, Transevse, Coronal). In most of the supra-levator type of anorectal malformations, thin levator and sphincter muscles were observed by Sagittal and Coronal scans. Transeverse scan revealed that the neorectum was not effectively pull-throughed into the puborectal muscle in one patient. Coronal scan showed the dameged external sphincter muscle. In three low types of anorectal malformations, the levator and the sphincter muscles were all well developed, but in one patient the external sphincter muscle existed at the posterior part of the anal canal. These observations were usefull in managing the postoperative care of anorectal malformations.
In reviewing 116 newborn infants with gastrointestinal perforation, clinical findings and modes of treatment for the last 32 infants were compared with those for the first 84 infants treated before 1978. It was concluded that early diagnosis of septicemia was decisive for successful treatment and the details of its treatment were as follows. 1. Whole blood exchange transfusion was performed promptly and repeatedly in neonatal septicemia. 2. Dopamine was administered preoperatively and it improved the renal and peripheral circulatory conditions. 3. Intravenous hyperalimentation performed without difficulty was useful for long term nutritional support. 4. Advance in postoperative artificial ventilation, especially CPAP, facilitated controls of acid-base balance. 5. Co-operation with neonatologists, especially in low birth weight infants, was essential for general life support. The unfavorable factors for this disease, such as low birth weight, oliguria, preoperative complications and so on, were proposed and the score based on them was helpful for the prediction of their prognosis.
Fundamental studies and clinical application of the continuous esophageal pouch elongator were performed. In the fundamental studies, the streching test of canine esophagus revealed that the force load necessary for tearing off the esophageal specimen was 4-8 kgf. The force load in theclinical application up to 200 gf was taken to be within the safty range. In this streching test, the esophagus of puppy was proven to be more easily elongated than that of adult dog. According to these measurements, a fundamentat mathematical model of esophageal viscoelasticity was calculated to be F(t)=F/K(1-e-Kt/r_1)+Ft/r_2. This is an addition of elasticity and viscosity function. A newly deviced elongator worked under direct measurement and display of the loaded force and esophageal elongation. The elongator was also devised to protect the pharyngoesophageal area from harm. The elongating part was consisted of bellows artificial artery which could dilate and deflate according to a given air pressure. This part was fixed to the mouth piece plate of the individually made helmet. Control part was regulated by a microcomputer, which worked under the input regulating data, present interval of pressure load and alarm circuit for preventing dangerous overload in reference to mathematical model. This apparatus was successfully applied to a patient with Gross A type esophageal atresia, with apart distance of 6 vertebral bodies between the pouches, in the course of 11 months. Direct anastomosis aided by an additional procedure of Livaditis myotomy was achieved without troubles.
Conventional liver function tests do not always show the exact liver function in postoperative biliary atresia (BA) patients. Although bile acid torelance test after oral administration has been employed for diagnosis of various hepatic diseases, it has never been studied in BA patients. Bile acid tolerance test following oral administration of ursodeoxycholic acid (UDCA) was performed in 11 postoperative non-jaundiced BA patients. Blood samples were collected from peripheral veins before and at 30, 60, 120, and 180 min. after administration of UDCA. UDCA and glycine-conjugated UDCA were measured by high performance liquid chromatography and 3α-hydroxysteroid dehydrogenase immobilized in column form. 1) Disappearance curve of UDCA showed a significantly higher level at 30-60 min. in sera of most BA patients as compared with normal infants. 2) Area under curve (AUC) of UDCA which indicated liver capasity to handle the loaded bile acid for a given time showed a significantly higher level in most BA patients than in normal infants. 3) Only 3 in 11 postoperative non-jaundiced BA patients were normal on this bile acid tolerance test. In conclusion, our present study indicated that disturbance in bile acid metabolism existed in most postoperative BA patients who had recovered from jaundice completely and showed normal values on conventional liver function tests. Therefore, bile acid tolerance test can be useful to evaluate the reserved function of the liver in these patients during the long term follow-up period.
Three different kinds of experiments were done. (1) Batteries were placed in the digestive fluid. Electrolysis occurred and fluid around the cathode was strongly alkalized. (2) Batteries were placed on the surface of the mucous membrane of stomach of rabbits and frogs. Ulceration of mucous membrane around the cathode developed in about an hour. It took about 24 hours for battery to collapse and to cause alkali leakage. (3) Electorical resistance of the tissue was measured and which ranged 100-500K ohm. Electorical resistance of digestive fluid was 8K ohm. Electorically induced tissue damage was negligible. In conclusion, alkalized fluid secondary to electrolysis around the cathode was the primary cause of tissue damage.
Two monoclonal antibodies against human neuroblastoma cell line (SK-N-DZ) and three monoclonal antibodies against murine neuroblastoma cell line (C-1300) were obtained by fusing mouse myeloma (P3U1) and immunized spleen cells. These antibodies were tested on the cell lines of 6 human neuroblastomas, 1 murine neuroblastoma, 1 human fetal kidney, 1 human fetal liver, 3 human hepatomas, 1 rat glioma and two lymphocytes of lymphoblastic leukemia patients (ALL) by indirect immunofluorescence. These five antibodies reacted not only on neuroblastoma cell lines but on other cell lines. They also reacted relatively well with neuroblastomas. More over, one anti-human monoclonal antibody exhibited complement-dependent cytotoxicity (CDC) against human neuroblastomas and Three anti-mouse monoclonal antibodies did it against a murine neuroblastoma (C-1300).
Measurements of intraluminal pressure of the bile duct and amylase in bile were performed in 3 patients with congenital choledochal dilatation associated with anomalous junction of pancreatico-biliary ductal system. The values of amylase in bile were 46,500S.R.U., 27,850S.R.U., and 7,290S.R.U., respectively. These values were abnormally high. Before administration of gastrin and secretin a high pressure zone was found in the sphincter of Oddi. However, it was found neither in the common channel nor in the anomalous junction. A rsting pressure of the sphincter of Oddi increased 3 minutes after administration of tetragastrin. However, no pressure change was found in the anomalous junction after administration of gastrin. On the other hand, it was shown that secretin decreased the resting pressure of sphincter of Oddi. Thus, no sphincter function, as was found in the sphincter of Oddi, was not observed in the anomalous junction of pancreatico-biliary ductal system. Therefore, it seems that this anomalous junction allows reflux of pancreatic juice into the common bile duct or reflux of bile into the pancreatic duct corresponding to the pressure gradient.
During the last 14 years, we have experienced 60 pediatric patients with blunt abdominal trauma which required hospitalizaton. 21 cases were surgically treated. The initial clinical and laboratory findings of 21 operated patients were compared with those of 31 non-operated patients. Perforation of digestive tract was seen in 7 patients. They had severe and persistent nausea and/or vomiting. Muscular guarding was observed in all patients. On the other hand, W.C.B. count and temperature were not significant to the decision-making. Operation for splenic injury was performed in 6 patients. They were pale in appearance, although their vital signs were stable and laboratory data did not reveal anemia yet. 17 patients with liver injury showed marked elevation of G.O.T., G.P.T., and L.D.H. levels. Three of them were operated on due to hemorrhagic shock. Their G.O.T. and G.P.T. levels were above 300 IU, and L.D.H. above 800 IU. Other 14 patients with liver injury were managed conservatively. 2 of them showed abnormal liver enzyme levels as high as operated cases. But, as they did not present hemorrhagic shock, conservative therapy was chosen. Pancreatic injury was encountered in 11 patients. Early diagnosis was difficult because elevation of serum amylase level was not always seen at the initial examination, and clinical findings were not specific either. In addition to initial clinical findings, ultrasonic examination was useful in determing the indication of operation.
Barium enema, anorectal manometry, and histochemical mucosal biopsy are main diagnostic procedures for chronic constipation including aganglionosis. Reliability of these examinations were evaluated quantitatively in view of the differentiation of aganglionosis. Positive caliber change in barium enema, positive rectoanal reflex in manometry, and positive nerve proliferation and/or absent of ganglion cells in biopsy were assessed as positive, and divided into two categories of true and false groups. The predictive value and efficiency were also calculated from these data. All values of barium enema were very low, suggesting that barium enema is unreliable to exact differentiation. In the neonatal period, barium enema often fails to show a caliber change (false negative), however, low predictive value of positive (a large number of false positive) was found in this series. Therefore, examination using barium enema only may lead to a misdiagnosis and overtreatment. Barium enema should be used for evaluation of extension of aganglionic area and of megarectum in cases of chronic constipation. The other two examinations, manometry and histochemical biopsy, showed excellent results for differentiation of aganglionosis: the efficiency was 98.5 in histochemical biopsy and 96.0 in anorectal manometry. Anorectal manometric study, however, is likely to be more suitable for functional evaluation of the anorectal canal than in differentiation of aganglionosis.
Correlation between changes of plasma cyclic AMP level after glucagon stimulation and grade of liver damage was investigated in patients with congenital biliary atresia: The results were as follows; 1. No characteristic pattern of plasma, cyclic AMP response to the glucagon stimulation was observed. 2. Plasma cyclic AMP levels 10 minutes after glucagon stimulation were decreased in a parallel way with progression of the hepatic fibrosis. 3. Postoperative prognosis may be estimated by repeating the glucagon stimulation test. In conclusion, the glucagon stimulation test seems to be useful as an indicator of liver damage or hepatic fibrosis in patients with congenital biliary atresia.
Three infants aged 8, 9 and 10 months, have undergone a surgical repair of bronchomalacia employing an external stent to reconstruct the bronchial lumen. The preoperative state of these patients was non-specific persisting respiratory distress. Bronchoscopy and bronchography were diagnostic and demonstrated the collapse of the bronchial lumen. The involved bronchus was left in two and bilateral in one patient. At operation, the whole cirumference of the involved bronchus was exposed and taped. An elastic silastic sheet lined with Marlex mesh inside was placed around the bronchus for the three quaters of the circumference, and was sutured to the bronchial cartilage at both edges and mid portion of the silastic sheet. The triangular radiating tension, created by elasticity of the silastic sheet, provided a patent lumen in the collapsed bronchial segment. By this procedure, respiratory distress has improved to allow the endotracheal tube to be removed shortly after operation. During the followup period of 4-12 months, a sudden death at home, the cause of which is unknown, was seen in one patient. The other two patients have been free of disease.
From 1980 to 1985, 25 children with pectus excavatum were treated by a modified Peters' repaire, with the addition of an internal Kirschner wire fixation technique for support of the sternum. The results were assessed radiographically with respect to objective anterior rib steep angle (4-6th rib) for the initial and follow-up results. Results were satisfactory for rt. fifth rib angle as follows : preoperative 36.5±4.5 degree; postoperative, 41.2±3.9 degree (p < 0.001). The follow-up results (M^-=574 days) in smaller series have shown significantly more obtuse rib angle (p < 0.05). These suggest that it is useful for the assessment of surgical repair to compare pre- and post-surgical anterior rib angles and it might reflect increasement of the chest wall thickness for rib angle to become more obtuse after operation.
During the last 10 years, 11 patients with esophageal stenosis were treated with esophageal dilatation (bougienage) at our department. Several kinds of esophageal dilators (Tucker bougie, Chivalier-Jackson bougie. Endoscopic balloon dilator, and Cohen-Gray balloon dilator) were used during this period. Usually, the bougienage with Tucker bougie is recommended to use retrogradely, but we think it is better to use antegradely to prevent gastroesophageal reflux and widening of gastro-stomy stoma. Cohen-Gray balloon dilator has a number of advantages, and is considered as the most ideal dilator today. Most of the esophageal stenosis we treated by dilatation revealed satisfactory clinical results, but some patients who had severe scarfomation were not dilated enough.
Although situs inversus (SI) is rare, it is recognized' as an important condition since a number of anomalies are associated with it. In this paper, we report 2 cases of neonatal SI associated with abdominal malformation and review the literature of SI discovered in early infancy. Case 1: A one-day-old, full term, 2,800g, male was admitted with the complaint of tachypnea. A thoraco-abdominal X-ray film demonstrated a rt. diaphragmatic hernia and dextrocardia. He underwent repair of the diaphragmatic hernia (the size of the hernia was 3.8cm by 3.8cm) and Ladd's procedure for the malrotation. The postoperative diagnosis was rt. diaphragmatic hernia and malrotation associated with SI and dextrocardia. Presentrly, at the age of 11 months, he is developing normally. Case 2: A nine-day-old, full term, 3,070g, female was admitted with the complaint of vomiting. A thoraco-abdominal plain film demonstrated dextrocardia and dilatation of duo-denum. The diagnosis of duodenal stenosis was made by the upper GI series and she underwent a duodeno-jejunostomy. She was discharged in satisfactory condition on the 25th postoperative day. We collected 28 cases of intra-abdominal anomalies associated with SI in children less than 1-year-old, including our cases, in Japan. In a literature review, intra-abdominal anomalies were found as follows: malrotation in 15 cases, congenital biliary atresia in 11, preduodenal portal vein in 7, polysplenia in 7, diaphragmatic hernia in 4, annular pancreas in 4, asplenia in 3, duodenal atresia in 2 and duodenal stenosis in 2. When situs in versus is recongnized in early infancy, the examinations of the GI tract, biliary tract and portal vein as well as a search of cardiac anomaly are specialy recommended for making a prompt and accurate diagnosis.
A 2-day-old girl was admitted to our university hospital with a tumor of vulva. The tumor was elastic hard with normal skin. Laboratory findings were within normal limits. The tumor was extirpated en bloc uneventfully. It was 3.5x2.0x2.0cm in size and weighted 10 grams. Histologically it was consisted of a mixture of smooth muscle, well differentiated adipose tissue, cartilage, connective tissue and small blood vessels, and was diagnosed as benign mesenchymoma. The benign mesenchymoma is rare tumor. To our knowledge, 68 cases of benign mesenchymoma in infancy and children have been reported in Japanese and English literature, The most common sites of the tumor are the head and neck, trunk and lower extremity. The occurrence of the vulva is very rare as this present case. Because of all the tumor infiltrate to a greater or lesser degree, we recommend wide local excision as the treatment of choice.
Cervicomediastinal cystic hygroma (CMCH) is a rare disease which is often associated with serious respiratory disturbance. This report describes one-stage operation on a infant and two children with CMCH. In either case, the median sternotomy or the cervical transverse incision were performed. The median sternotomy was rationale, well-tolerable and resulted satisfactory. The cervical transverse incision also seemed a procedure of choice in those patients with CMCH whose mediastinal lesions were relatively small.
A case of giant retroperitoneal cystic lymphangioma was reported. A 4-year-old boy was admitted, complaining of abdominal distension. Physical findings showed a characteristic compressible abdominal mass which was movable transversely but not longitudinally. Abdominal ultrasound and computed tomography revealed a giant cyst almost occupying the whole abdominal cavity. GI series and intravenous pyelogram excluded GI and urinary cysts or tumors. Complete extirpation of the giant cyst was done successfully without resection of the adjacent bowel. His postoperative course was uneventful and no recurrence was seen 10 months after operation. A review of the literature shows that retroperitoneal cystic lymphangioma is rare. Its diagnosis is not so difficult but complete extirpation is often difficult. It is suggested that location of cystic lymphangioma should be correctly clarified by GI series and other diagnostic aids, and currative extirpation should be performed, if possible, to prevent recurrence.
Malignant melanoma in soft parts is rare and no cases of malignant melanoma occuring from mesocolon have been reported in the previous literatures. A 2-year-ll-month-old girl with malignant melanoma originated from the transverse mesocolon was reported. The tumor, measured 7x7x5 cm in size and 110g in weight, was removed with part of transverse colon and regional lymphnodes. The histological findings revealed nest-formed large clear cells with vesicular nuclei and prominent nucleoli, some of the neoplastic cells had melanin pigment. Mature melanosomes in the cytoplasm of neoplastic cells were demonstrated by electron microscopy. The patho-logical diagnosis was malignant melanoma of soft parts. This case was considered primary occurence of the transverse mesocolon because no other melanotic lesion was found in other part of her body.
The authors reported a case of 4 years & 8 months boy who had paratesticular rhabdomyosarcoma, occurring in the right side testicle. According to the criteria of Intergroup Rhabdomyosarcoma Study, the case belonged to group I, and it was diagnosed histopathologi-cally as embryo-alveolar type. After the radical operation, VAC (vincristine, actinomycin D, cyclophosphamide) therapy has been continued for 2 years, and the patient has survived now 2 years and 11 months without any sign of the disease.
A successful surgical case of aortopulmonary septal defect was reported. The patient was a 12-month-old girl, who had aortopulmonary septal defect, ventricular septal defect, long narrow right pulmonary artery and left pulmonary hypertension. Aortography and echocardiography revealed a communication between the ascending aorta and the main pulmonary artery. Ventricular septal defect with bidirectional shunt was detected by right ventriculography. Operation was successfully performed under the extracorporeal circulation. Aortopulmonary septal defect was 14mm by 18mm in size. This defect was closed through aortomy using a Dacron patch and the ventricular septal defect was directly closed through right ventriculotomy. The patient recovered uneventfully. Postoperative cardiac catheterization was performed two months after operation. Right ventriculography revealed the long narrow right pulmonay artery and moderate tricuspid regurgitation. Right ventricular peak systolic pressure decreased to 56mmHg from preoperative value of 95mmHg. She was discharged two months after operation. Ventricular septal defect was detected in 5 of 56 patients undergoing surgical treatment of aortopulmonary septal defect in Japan. The defect with ventricular septal defect and long narrow right pulmonary artery was, to our knowledge, not found in Japanese literature ex-cept for our case.