The intrahepatic bile duct of so-called uncorrectable type of biliary atresia was studied histopathologically using image processing system. Total area of all intrahepatic bile ductuli (B), which contains both lumen and epithelium, in one field and the total area of all fibrotic area (F), which contains bile ductuli and vesseles, in one field were measured in wedge biopsy specimens obtained from 25 patients. B/F ratio was calculated and used as an indicator which means the percentage of the area of intrahepatic bile ducts in the interstitial fibrotic area of Glisson's sheath. B/F ratio was low in the patients was showed excretion of a large amount of bile after the operation, while the ratio was high in those who showed excretion of small amount of bile after the operation. The present study suggested that there was a close correlation between B/F ratio of liver biopsy specimen and the postoperative bile flow.
Thirty-three infants with hepato-biliary disorders, including 19 cases of biliary atresia, were studied by hepato-biliary scintigraphy with Tc-99m-EHIDA. The purpose of this study was to evaluate the hepatic function and bile excretion in these patients. After intravenous injection of Tc-99m-EHIDA, the activity of Tc-99m-EHIDA was counted in the hepatic area for one minute using the gamma scintillation camera. Counting continued for 60 minutes and time-activity curve was made. Time-activity curve of patients were divided into 4 types according to peak time, peak shape and clearance index; 1) Well excretive type, 2) Inter-mediate type, 3) Poor excretive type, 4) Extra hepatic excretive type. Uptake phase of the time-activity curve related to glutamic oxaloacetic transaminase activity, and excretive phase related to γ-glutamyl transpeptidas activity. This non-invasive examination is useful not only for evaluation of the hepatic function and bile excretion, but for deciding reoperation in cases with cessation of once observed bile flow after the operation.
In spite of improvement in the treatment modality, the survival rate of the patients with the advanced neuroblastoma remains poor. The result of clinicopathological study in 14 autopsied children who had been treated by either James' therapy or high-dose CPM therapy for advanced neuroblastomawere presented. 1. Seven children treated by James' therapy died of progression of the primary tumor,or bleeding tendency due to the side effect of the chemotherapy. 2. The remaining 7 children treatedby high-dose CPM therapy died of dyspnea caused by bloody pleural effusion and compression by the enlarged liver, or bleeding from the liver metastasis. 3. There was no significant difference of thesite or number of the metastasized organs between two groups. 4. High-dose CPM therapy was effective for the primary tumor, but ineffective for control of bone, lymph nodes, and liver metastasis. An effective therapy for the control of metastasis in the bone, lymph nodes and liver would be necessary to improve the results of the treatment of the advanced neuroblastorna.
Portial recanalization of the thrombosed portal vein and the development of multiple small collateral venous channels adjacent to the portal vein are presumed to account for the cavernous transformation of the portal vein. The importance of sepsis in association with fever and dehydration in neonatal period is stressed. Nevertheless, there is a high proportion of cases with no obviousetiology. Clinical findings of 36 cases under 16 years of age were analyzed and the results were as following. 1) The extrahepatic portal venous pressure did not decrease with aging. 2) Intrahepatic portal vein was patent in most instances and the occlusive process was not progressive. 3) The degree of hepatopetal collaterals was not correlated with age, but increased with aging in individual cases. 4) The mean portal venous pressure of the group with highly developed hepatopetal collaterals wassignificantly low relative to the groups with slightly and moderately developed collaterals. 5) Histological findings of the liver wer grouped as following; (a)hepatic fibrosis, (b)chronic cholagitis, (c)chronic hepatitis, (d)congest ion, (e)normal liver tissure group. 6) Some of the chronic hepatic lesions above mentioned were presumed to have caused the portal occlusion in neonatal age. Experiments were designed to study the effect of gradual portal venous occlusion on the development of hepatopetal and hepatofugal collateral veins in the rat. Young adult Sprague-Dawley rats were randamized into two groups; (A) sham-operated in ten rats, (B) operated in 20, with placement of ameroid constrictor around the portal trunk. Nine weeks after the application of the constrictor, portal venous pressure was elevated to a level about twice of normal range. Five patterns of collateral veins developed in responce to the extrahepatic portal vein occlusion; (a) vein directly bridging across the occluding ameroid constrictor, (b) paraesophageal and submucosal vein, (c) veins to the left adrenal and renal vein, (d) retroperitoneal vein,(e) veins in ometoparietal adhesions. The lumen size of the submucosal esophageal veins in group (B) increased threefold compared to in groug (A). The occluded portal vein and the surrouding hepatoduodenal ligament in group (B) contained three types of the cavernous hamangiorna-like structure. This structure consisted of partial recanalization of the thrombosed portal vein and the development of multiple small venous collateral channels adjacent to the portal vein. This experimental production of the cavenous hamangioma-like structure suggests the cavernous transformation in the clinical extrahepatic portal hypertension is proved to be acquired in origin excluding rare cases of the portal venous atresia.
Thirty-three infants with hepato-biliary disorders, including 19 cases of biliary atresia, were studied by hepato-hiliary scintigraphy with Tc-99m-EHIDA. The purpose of this study was to evaluate the hepatic function and bile excretion in these patients. After intravenous injection of Tc-99m-EHIDA, the activity of Tc-99m-EHIDA was counted in the hepatic area for one minute using the gamma scintillation camera. Counting continued for 60 minutes and time-activity curve was made. Time-activity curve of patients were divided into 4 types according to peak time, peak shape and clearance index; 1) Well excretive type, 2) Inter-mediate type, 3) Poor excretive type, 4) Extra hepatic excretive type. Uptake phase of the time-activity curve related to glutamic oxaloacetic transaminase activity, and excretive phase related to γ-glutamyl transpeptidas activity. This non-invasive examination is useful not only for evaluation of the hepatic function and bile excretion, but for deciding reoperation in cases with cessation of once observed bile flow after the operation.
A surgical technique for extensive aganglionosis involving the distal ileum consisting of: (1)initial ileostomy, (2) elective longitudinal side-to-side ileocobstomy and (3) a Swenson type rectal"pull through" with the ileocolostomy segment completely dividing the mesocolon attached to the colonic segment has been previously reported. In this report, a refined surgical technique is described. At creation of side-to-side ileostomy, the mesocolonic vessels are divided employing the preserved ileocolonic artery as the vascular stalk to the colonic segment that is pulled through at definitive operation. In this revised technique, the distal part of the normal ileum, that has been used for ileocolostomy in the original technique, is preserved so that this relatively small sized bowel is used for Swenson type anastomosis at the pelvic bottom at definitive operation. The technical details have been described with a case report.
In the early postoperative intravenous hyperalimentation (IVH), there are some problems such as the period of surgical diabetes and infusion of fat emulsion. We studied the period of suryical diabetes and the effectiveness of the infusion of fat emulsion from the early postoperative period. 15 infants were divided into three groups. Each group received total calories of 86Cal/kg/day with 2g/kg/day of amino acids. In Group I; fat was not given, In Group II; fat was infused in the amount of 10% of total calories and in Group III; 20% of total calories. Maximum blood sugar (BS) level immediately after operation was about 200mg/dl in each group, but BS level decreased within the normal range by 6 hours after operation. IRI had maximum level at 3 hours after operation and decreased at 6 hours after operation. The change of C-peptide was almost parallel to IRI. The duration of surgical diabetes in infants was, therefore, assumed for 6 hours after operation. One day after operation, BS level of Group land II were slightly high but in Group III BS level was within the normal range. 0n the 3,5, 7th day after operation BS level was within the normal range but in Group III BS level was most stable. Therefore, infusion of fat emulsion in the amount of 20% of total caloriesis efficient with respect to carbohydrate metabolism. In all groups no liver dysfunction was noted.
In 18 infants with severe constipation presenting with abdominal distension, staining of underpants, and overflow incontinence, radiologic, manometric, and histochetnical studies of the rectum were performed. Radiologic study: By barium enema examination, rectal dilatation ratio (Rectal transverse diameter divided by pelvic transverse diameter on frontal X-ray view) was investigated. It was greater than 60% in all cases. The PC-R angle (angle of rectal floor line against P-C line on lateral X-ray view described in figure) was found to range from +20°to -20°. Manometric study: Anorecta reflex was present in all cases. Rectal pressure was 10.8±4.3cm H_2O, and anal resting pressure 25.3±7.1cmH_2O. This pressure study did not show statistical difference from that of normal infants. The rectal dilatation ratio was related to the PC-R angle. The anal resting pressure seemed to be related to the rectal dilatation ratio. Patients with an anal resting pressure above 30cmH_2O and a dilatation ratio greater than 90% have high incidence of severe constipation. All cases were treated conservatively suppository, clyster and catharics, and improved to good condition within 6 months. One case was treated for 3 years without improvement and operative internal-sphincterectomy is planned in the near future.
The cases of siblings with congenital multicystic kidney were reported. The elder is a 25 days old male infant and the younger is a 9 days of female infant. Affected side was left in the former and bilateral in the latter. Affected kidneys were removed in both cases. Female patient with bilateral involvement is doing well five years after the operation as the functioning area were remained at the upper portion of left kidney. Though congenital unilateral multicystic kedney is well defined group of unilateral, not hereditary, relatively rare disease, bilaterality or other pathology of the contralateral side were reported in the recent years. 86 cases of unilateral and a case of bilateral type were collected from Japanese literature during 1960 through 1982. Our cases are the first reported cases of occurrence in siblings and the female case is the second reported case of bilateral involvement in Japan. The nomenclature, congenital unilateral multicystic kidney became inadequate since clinical and pathological evidence are accurnlated, and this condition are now named multicystic dysplasia of the kidney or multicystic dysplastic kidney. Therefore, further investigation of this disease will be required under the new concept that this disease is dysplasia of the kidney.
Familial occurrence of Hirschsprung's disease has been frequently documented but that of extensive aganglionosis in which aganglia extends to the midgut or the jejunum is very rare. Only six familial cases have been reported up to date in Japan and the surgical results remain poor. Recently, we have experienced the extensive aganglionosis occurred in brother and sister. The elder brother was admitted to our hospital on December 5, 1979, with an abdominal distension and vomiting, and was finally performed Martin-Ikeda's procedure. The younger sister was admitted on February 21, 1981, and finally underwent Swenson's pull-through procedure coupled with Kimura's longtudinal ileocolostomy. The both children have now been doing well. Thirty-one familial cases (69 patients) of Hirschsprung's disease were collected from the Japanese literature and reviewed.
A l-year-7-month-old girl with dyspnea and cyanosis was admitted. Because of respiratory distress due to the large anterior mediastinal mass, emergent operation was performed and the mediastinal tumor was extirpated subtotally. Histologically, it was diagnosed as malignant thymoma of epithelial type. Radiation therapy to the anterior mediastinum and intensive chemotherapy were given and the remission appeared to be obtained. However, relapse occurred with rapid progression and the tumor showed no response to the anticancer drugs. The patient died 3 months after operation. The case presented here showed that the rapidly progressive clinical course is that must serious problem in the therapy of thymoma in childhood. 34 cases of thymoma under 15 years of age, including our case, were collected from Japanese literatures and reviewed. By analysis of them the preoperative chemotherapy or radiation therapy seemed to be effective for the advanced thymoma.
An 1-year old boy was admitted to our hospital because of difficulty in breathing presenting for 2 weeks. Roentgenologic examination of the chest showed complete opacification of the left hemithorax. Computer tomography scanning showed a large irregular mass at the level of the left diaphragm. An attempted thoraceutesis on the left side gave bloody fluid. Cytological examinatio demonstrated the presence of small malignant tumor cells. Exploratory laparotomy revealed a large hard mass which arised from the left diaphragm and infiltrated into the lesser curvature of the stomach and the lymphnodes. Multiple metastases measuring 5 to 20 mm in diameter were noted all over the omentum. Biopsy of a distant metastatic lesion was done and the specimen was processed to pathological studies with light microscopy and electron microscopy. Final diagnosis was made as embryonal rhabdomyosarcoma. The patient did not well respond to postoperative chemotherapy and radiation therapy. He died in the hospital within a period of 5 months. We were able to collect a total of 40 cases with primary diaphragmatic tumor from the Japanese literature. Among these 40 cases, 23 are benign, whereas 17 are malignant. The cystic type was most frequently seen, namely in 12 cases. Olafsson stated that fibrosarcoma had the highest incidence among malignant tumors, namely in 9 of 33 cases. On the contrary, in the Japanese series, rhabdomyosarcoma was the most frequen type seen, namely in 9 of 17 cases.
Chylous cysts of mesentery are extremely rare. This report describes 3 cases of this disease, observed at Chiba Univ. Hospital in recent years. As shown in case 1 and 2, good clinical condition was seen in pre- and post operative period. As shown in case 3, however, intestinal volvulus caused by tumor was occurred. In addition we have reviewed the literature since 1917 including 16 children's case reports, and showed the present concepts in the pathogenesis and treatment of these rare pathological conditions.
A case of neonatal small left colon syndrome was reported. A 2 days old female neonate was admitted with abdominal distension and bile-stained vomiting. Barium enema showed a small left colon. Differential diagnosis included long segment aganglionosis, meconium plug syndrome and neonatal small left colon syndrome. Ano-rectal manometoly showed normal reflex. In rectal suction biopsy, the bowel showed normal ganglion cells by H-E staining but showed immaturity ganglion cells by acetylcholinesterase staining, Concervative therapy was performed. Although no defecation of the meconium plug was observed, abdominal distension and vomiting subsided and thereafter she had normal bowel movements. The final diagnosis was neonatal small left colon syndrom, and a small left colon has been observed for a year. We reported a first survived case of neonatal small left colon syndrome in Japan and we suggest a immatrity ganglion cells for the etiology of the neonatal small left colon syndrome.
The esophageal cyst is a relatively rare disease. Only 25 cases have been reported in literature in Japan. A case of the esophageal cyst in the left mediastinum was presented in this paper. The patient was a 6-year-old girl who was hospitalized with cough and fever. Echogram and CT-scan were useful for the diagnosis of the cyst. Thoracotomy was performed under the diagnosis of the mediastinal cystic lesion. The cyst was located at the left lower posterior mediasinum, and it was not communicated to the bronchus and the esophagus. Resional pneumonia was observed at the surrounding of the cyst. Histlogically, the cyst wall was lined with the stratified squamous epithelium and two muscular layers. There was no cartilage. Based on these fingings, the diagnosis was confirmed as the esophageal cyst. In addition, the clinical features, diagnosis, therapy and definition of the esophageal cyst were discussed and the literature reviewed.
A l-year-3-month old girl who had Persistent Truncus Arteriosus (Type II) was operated on using the Hancock 14 mm diameter valved conduit under the deep hypothermia, circulatory arrest and limited cadio-pulmonary bypass. Post-operatively, unexpected right to left intracardiac shunt was found and the patient developed severe heart failure. On the 1-st post-operative day, emergency cardiac catheterization and angiography demonstrated PLSVC draining into the left atriurn and right upper and middle PAPVC into right SVC. Under the cardio-pulmonary bypass with hypothermic low flow perfusion technique, an intraatrial repair was carried out immediately. Despite of the total repair, patient died of severe heart failure on the second post-operative day. Anatomical findings at autopsy were:Persistent Truncus Arteriosus (Type II) associated PLSVC draining into the left atrium, bilateral PAPVC (right upper & middle PV into right SVC and left upper PV into the coronary sinus), a small ASD and PDA. The incidence, diagnosis and surgical technique of this rare anomaly are discussed with some literatual considerations.