In order to clarify the jaundice clearance pattern immediately after operation for biliary atresia, cases exhibiting both satisfactory post-operative clinical course (total serum bilirubin levels (T-Bil) of 1.0mg/dl or less) and also those exhibiting unsatisfactory progress (postoperative jaundice clearance but T-Bil > 1.0mg/dl) were monitored in terms of their T-Bil. In all cases T-Bil fell to half of the preoperative level within 45 days after operation, irrespective of the satissfactoriness or othewise of postoperative clinical progress, and in the case of the satisfactory clinical course cases T-Bil normalized (< / = 1.0mg/dmg/dl) within 90 days of operation. A comparison with cases of common bile duct dilatation showed the rate of T-Bil reduction to be significantly longer, even amongst the group with satisfactory postoperative clinical progress. Clearly, therefore, there would appear to be intrahepatic cholestasis even in cases where postoperative clinical progress was otherwise satisfactory. These results suggest that the prevention of ascending cholangitis is of paticular importance during the first 3 postoperative months. In cases where T-Bil had failed to normalize more than 90 days after operation, it was assumed that liver transplantation might become necessary at some future point.
To recommend therapeutic regimen for neuroblastoma patients diagnosed before 1 year of age, we conducted a survey for clinical characteristics and present status of treatment in the patients through a questionaire all over Japan. 537 cases were registered. 314 were male and 223 were female. 355 cases were detected asymptomatically by mass screening. 45 were detected incidentally by physical findings at medical examination. 137 were diagnosed by clinical symptoms. The site of primary tumor was abdomen in 456 cases (including the adrenal gland in 275 and retroperitoneum in 156) , followed by mediastinum in 62 and neck in 11. There was no characteristic primary site for all of these cases. The extent of diseases at diagnosis was Stage I (141) , Stage II (154) , Stage III (92) , Stage IV A (37) , Stage IV B (34) , Stage IV S (73) , and unknown stages (6) . 77% of the cases detected by mass screening were in Stage I, II or IV S, and 23% were in Stage III, IV A or IV B. 53% of the others were in Stage I, II or IV S, and 45% were in Stage III, IV A or IV B. 50% of the cases diagnosed by clinical symptoms were in Stage III, IV A or IV B.
The small intestinal transplantation is considered to be the radical operation for short bowel syndrome in pediatric patients. Because the small intestine contains an extensive number of immunologically competent cells, the most complex and difficult problems to overcome have been the immunologic responses by the host and graft following small-intestinal transplantation. The use of different inbred strains allows experimental designs for special immunological questions. We have examined two kinds of procedures for graft harvesting and compared each other. One is the graft harvesting with the short vascular pedicle (Group I, n = 90) , and the other is that with long vascular pedicle (Group II, n = 106) . In spite of taking the longer time for harvesting the graft, the technical success rate of recipient operation was significantly higher in Group II than that in Group I. The precise technique of Group II was described in this paper. The main technical failures of Group I were thrombus, congestion, and hypoperfusion, while, that of Group II was hemorrhage. In the course of graft harvesting, one of the most important points was to keep the sufficient blood flow of the graft by ligating the distal abdominal aorta, renal arteries, and the celiac artery in the early stage of the operation.
5 patients with congenital biliary atresia (type I : two cases, type III : three cases) were operated on by hepatoporto-enterostomy during the neonatal period. Both type I cases were detected by antenatal ultrasonographic examination. Jaundice cleared (total bilirubin lnE 1.0mg) in all the patients, but recurrence of jaundice and fever occured in one case at age eight. Two cases of type III suffered from portal hypertension. Histological findings from liver specimens, did not indicate ongoing fibrosis in three of five cases. These data suggest that operation for patients with CBA during the neonatal period is effective, but that long-term followup of postoperative status is required.
We analysed 28 patients with urological problems who have been evaluated or treated with endoscopy during neonatal periods. The underlying urological disorders included hydronephrosis in 9, ureterocele in 4, multicystic kidney in 3, megauteter in 3, posterior urethral valve in 2, ectopic ureter in 2, VUR in 2, and 3 other disorders. Endoscopic operations with either electrode or cold knife was done on 7 patients with posterior urethral valve, ureterocele or uethral stricture. Retrograde ureteral catheterization was done on 17 patients, mainly with PUJ stenosis, multicystic kidney or megaureter. Endoscopic examination was done on 4 patients with VUR or ectopic ureter to see location and configuration of the ureteral orifice. Accurate diagnosis was made on 27 neonates (96%) through endoscopy. The remaining one patient required urodynamic study because no anatomical abnormality was found on endoscopy. Intraoperative or early postoperative complication was seen in 6 patients including 5 with edema of the penile shaft or bleeding from the urethra and one with extravasation of contrast medium from the lower ureter during the retrograde study. Late complication was seen in 2 patients ; one with incomplete resection of the urethral valve, and another with recurrence of urethral stricture. Most of the complications can be prevented by careful and gentle technic.
We studied infants of necrotizing enterocolitis (NEC) who were born at our perinatal referring center. Between 1981 and 1991, fifteen newborns developed NEC with Bell's stage II and III, including 9 extremely-low-birth weights (ELBW ; < 1000g), and 5 very-low-birth weights (VLBW ; 1000-1499g). The incidence of NEC was 0.5% for all the patients who were housed in the NICU, 2.1% for ELBW, and 0.8% for VLBW. Fou rrecoovered with intensive medical treatment. Two ELBW infants had no indication for operation because of very severe intracranial hemorrhage and septic shock. Nine including 6 who did not improve with medical treatment and 3 who showed very rapid deterioration within 24 hours after the onset underwent operation. The indications for operation were persisting metabolic acidosis in 2, and intestinal perforation in 7. Resection of necrotic bowel with jejunostomy or ileostomy was done in all infants except one who died during operation. After the operation, whole blood exchange transfusion was performed for each patient 1 to 4 times. Six tolerated the operation, and 3 died during the operation or within 3 days after operation. Positive blood culture, uncorrected metabolic acidosis and anuria at the time of operation werecondidered to be poor prognotic factors. Early operation was recommended to improve the prognosis of NEC.
In 32 patients with congenital dilatation of the bile duct, biopsy specimens of liver tissue at operations were analized histopathologically. Sixteen patients with jaundice were compaired with 16 without jaundice. Abnormal chenges of liver tissue were found in 69% of cases with jaundice and 44% of cases without jaundice. Liver cirrhosis were shown in 2 cases with jaundice and 1 case without jaundice. Incidence of abnormal change of liver tissue was high in cases with jaundice than in those without jaundice. Cases without jaundice exhibited more than slight changs of liver tissue. Liver damage was thought to be induced not only by obstruction of the bile duct but also by activated pancreatic enzymes regurgitating into the bile duct through anomalous junctions of the pancreatico-biliary ductal system.
A 24 day old female infant was referred to our hospital with apnea, abdominal distension and bilious vomiting. The apnea proved to result from congenital central alveolar hypoventilation, so called "Ondine's Curse" , and respiratory management required mechanical ventilation. At the age of 27 days a laparotomy was performed, and total colonic aganglionosis was histologically diagnosed. Ileostomy was performed at that time. She underwent ileocolic, side-to-side anastomosis at 5 months of age and a Swenson-type pull-through of the ileocolostomy segment at 8 months. She also had congenital deafness. All her disorders could be understood as manifestations of neurocristopathy, the etiology of which was proposed by Bolande in 1974.
We report the patients with extrahepatic biliary atresia seen in the sibships, which is the fourth report in Japan. First patient is a full-term boy with 3,020g in birth weight and the second is a full-term girl with 2,732g in birth weight. Both presented with subdural hematoma and jaundice at the age of 1 month. Pregnancy and delivery were normal and they have no significant history of exposure to infectious disease, illness and drug. A hepatoportojejunostomy was performed at the age of 48 days and 55 days respectively. Both patients had biliary drainage but subsequently had frequent episodes of cholangitis, which led to biliary cirrhosis. Both died at the age of 5 years 6 months and 1 year 4 months respectively. These cases suggest that there might be some kind of inheritance and maternal factor concerned with an etiology of biliary atresia.
A-1-month old female infant was admitted to this hospital with the primary complaint of abdominal distension and vomiting. Although intestinal X-ray series revealed diffusely distended colon, caliber change was not observed. Cystography revealed dilation of the bladder and urinary retention 78ml was observed by catheterization. Recto-anal reflex was present. Biopsy of the rectum revealed mature ganglion cells, while acetyl-cholinesterase steining did not reveal increased formation of nerve fibers. Upper intestinal manometric study revealed decreased number of contractions and decreased pressure at the 2nd portion of the doudenum, but did not reveal phase 3 interdigestive motor complex. Nutrition of the infant was managed by intravenous hyperalimentation and oral intake of elemental diet. In addition, colonic irrigation was performed to prevent the occurrence of enteritis using a tube inserted in the descending colon per anus. Although the infant is presently one year old and is demonstrating favorable physical and mental development, oral ingestion other than elemental diet is not possible yet. Oral administration of cisapride (0.3mg/kg/day) was ineffective.
We report a case of 26-month-old boy with WDHA syndrome in association with ganglioneuroblastic VIPoma. A patient was admitted for dehydration from protoracted watery diarrhea. Serum analysis revealed hypokalemia (2.4mEq/L) and high VIP levels (220pg/dl). Abdominal roentogenogram and computed tomography demonstrated a calcified tumor in the left paravertevral region. VIP secreting neurogenic tumor was highly suspected. In operation a well encapsulated tumor measuring 45×32×30mm was removed. The histological diagnosis was ganglioneuroblastoma. VIP level of the tumor was high (5500-95000ng/gwet). Soon after resection of the tumor, the diarrhea improved and serum VIP level returned within normal level. Forty-three months after the surgery, the patient remained healthy without evidence of tumor recurrence. In the literatures 45 cases of WDHA syndrome associated with neurogenic VIPoma have been reported since 1973, and we discussed them further.
A 14-year-old girl was admitted to our hospital because of an abdominal mass. Physical examination showed abdominal distension associated with fever, general malaise, and dull periumbilical pain. A large elastic hard mass was palpated in the middle abdomen. Laboratory data showed inflammatory reactions. Ultrasonography and CT scan revealed a well-defined, multi-loculated cystic mass occupying almost all of the abdominal cavity. Small intestine was displaced to the right posterior space. In MRI examination, the mass showed hypointensity on T1-weighted image and hyperintensity on T2-weighted image. It was huge mass extending from the left epigastrium to the pelvis. Superior mesenteric artery was displaced to the right. Angiography revealed it was a hypovascular mass, blood supply of which was from the branches of the first jejunal artery. Under the diagnosis of a mesenteric cyst, laparotomy was performed. At laparotomy, we found a huge cystic tumor originating from the jejunal mesentery. The cyst was extirpated completely without resection of the intestine. The cyst mesured 320x190x70mm in size, weighted 3050g, and contained clear and yellow-colored fluid. Pathological diagnosis was cavernous lymphangioma. She is doing well 9 months after the operation with no evidence of recurrence.
The results of surgical repair of paraesophageal hiatus hernia in six children aged from 9 months to seven years old were analyzed. They had no associated conditions. Postprandial borborygmus in thorax was audible in all, and anemia was detected in three. Hiatus hernia was found incidentally in a case on a chest X ray for an other indication. The diagnosis of paraesophageal hiatus hernia was established by barium swallow and endoscopy. More than half of the stomach had herniated in all patients, and a portion of the transverse colon in two. A posterior crural repair was employed in all children, and Nissen fundoplication was added also in two. Two months after surgery, a mixed typed hiatus hernia recurred in a child who underwent crural repair only. Crural repair was performed again transthoraxically in this case. They have been followed up for an average of six years, and they have been leading a normal school life. An addition of the antireflux procedure, especially Nissen fundoplication, is controversial. Because gastroesophageal reflux is rare and not severe in paraesophageal hiatus hernia. Antireflux procedure, however, should be added to surgical correction of the anatomic defect, if evidence of gastroesophageal reflux is present or lower esophagus is mobilized extensively for the purpose to remove large hernia sac and to repair the crus posteriorly.
We experienced a case of pulmonary tuberculosis in childhood. This 4-year-old boy was admitted to our hospital because of an abnormal shadow on the chest X-ray. He had a history of having been in the Philippines and complained of asthma during that time. At physical examination, his general condition was quite good except for weak respiratory sounds in the right upper lung field. Laboratory data showed mild inflammatory reactions. No bacteria grew in the culture of sputum. Tuberculin test showed weak positive. Chest X-ray revealed a tumorous shadow in the right upper lung field. Both CT-scan and MRI revealed a tumorous mass in the upper and middle lobe of the right lung. No aberrant artery was demonstrated by angiography. Bronchoscopy revealed severe stenosis of the right upper bronchus. Under the suspicion of a lung tumor, thoracotomy was performed. At thoracotomy, the right upper lobe proved to be the mass and the middle lobe was atelectatic. The right upper lobe was resected and when it was cut, atheromatous content and pus appeared. Mycobacterium was evident in them. Pathological examination revealed the presence of granulomas of which centers consisted of fibrosis and epithelioid cells. Langhans' giant cells and lymphoid cell infiltration were seen around them. The diagnosis was pulmonary tuberculosis. After the operation, he was treated by RFP, INH, EB and is doing well now.
A 4-day-old female neonate was admitted to Kansai Medical University Hospital because of cyanosis and anuria. Cystogram revealed a dilated urinary bladder. CT scan revealed two cystic regions in the presacral space behind the urinary bladder. We performed subtotal excision of the cyst at the age of 48 days. One of the cysts was the hydrovagina, which was evacuated. The remaining cyst was completely resected at the age of 16 months. The final pathological diagnosis of the presacral cyst was cystic mature teratoma. At the second operation, we could not insert a Foley catheter into the urethra from the external meatus. Cystogram done from the catheter cystostomy and cystoscopy revealed the urethrovaginal fistula. The pathogenesis of the urethrovaginal fistula might be an aquired antenatal anatomical maldevelopment of the urogenital structures caused by the compression of the large presacral teratoma.
The use of positive end-expiratory pressure (PEEP) in acute bronchial asthma has traditionally discouraged, because PEEP may produce distension of the lung and barotrauma. We reports its use in 10 month-old baby who got status asthmaticus after being induced anesthesia with oxygen and sevoflurane. Ausculation revealed wheezing and airway pressure was increased. When PEEP of 5 cmH_2O was applied, the ausculation revealed no wheezing, and on flow-volume curve, expiratory tidal volume and early expiratory flow were increased, the time constant was decreased. This observation suggests that PEEP may produce the improvement of functional airway obstruction, the reduction of airway resistance, and the increase of expiratory flow volume in acute bronchial asthma.
We analyzed the amplification of the N-myc oncogene in 72 neuroblastoma patients. Tumor staging of these patients revealed 9 cases of stage I, 16 cases of stage II, 19 cases of stage III, 19 cases of stage IV A, 5 cases of stage IV B, and 4 cases of stage IV S. In our series, we found an interesting case of amplified change in the N-myc oncogene at the site of tumor origin and in the metastatic regions. We suspect this was due to a decrease in tumor cells caused by intensive chemotherapy before surgery. In the analysis of N-myc oncogene amplification in resected specimens it is important to examine the tumor cells microscopically.
Gastric cancer in childhood is extremely rare, and its prognosis is very poor. We experienced a case of gastric cancer in childhood. This 13-year-old boy had complained of vomiting for one month. Gastrogram revealed severe stenosis at the antral area of the stomach. Gastroscopy revealed a cancerous ulcer on the antral area. Under the diagnosis of advanced gastric cancer, operation was performed. At laparotomy, two gastric cancer regions were revealed. One was Borrmann 3 type cancer of 7 × 5 cm on the antral area, the other was Borrmann 3 type cancer of 5 × 4 cm on the anterior wall of the upper corpus. There was no evidence of distant metastasis and peritoneal dissemination. Operative diagnosis was PO, HO, N 2 (+) , S 2 . Total gastrectomy with removal of the regional lymph nodes (R2 operation) was performed. Reconstruction of the alimentary tract was achieved by esophago-jejunostomy with Roux-ρ. Pathological diagnosis was poorly differentiated adenocarcinoma (scirrhous type) , ss, 1 y 2 , v 2 , INFγ, n(-) .stage II, and the operation was absolute curative resection. Though 1 year and 10 months have passed since the operation, the patient is alive uneventfuly.
A 12-year-old boy with hepatocellular carcinoma (HCC) and 2-year-old boy with poorly differeutiated hepatoblastoma were primarily treated with transcatheter arterial chemoembolization with lipiodol and adriamycin (L-TAE) , because their tumors were diagnosed unresectable at the time of admission. Hepatectomies with tumors were successfully performed one month and two months after L-TAE, respectively, with marked shrinkage of the tumors and decrease of AFP levels in sera. In the patient of HCC, serum AFP decreased to normal levels 50 days after left hepatectomy, and he is doing well without evidence of disease for 3 years. In the child with hepatoblastoma, L-TAE was performed in 3 times. Although, he is now undergoing an aggressive chemotherapy for lung metastases, AFP levels in sera indicate around 50ng/ml. L-TAE has widely been used in adults for the treatment of HCC with favorable results. However, L-TAE seems to be performed infrequently in infants and children with hepatic malignancy. Our data suggest that L-TAE can be reduced tumor size in short-term, and facillitates radical hepatectomy for primary hepatic malignancies in childhood.
Congenital malignant melanoma can be classified into three types. The first type is transplacental metastasis of the maternal malignant melanoma. The second type is congenital malignant melanoma developed in a giant melanocytic nevus. The last type is primary congenital malignant melanoma. We report here a case of primary congenital malignant melanoma in a five week old girl. After the excision of the tumor, she was treated by a chemotherapy of 11 courses. Despite of the deep invasion into the subcutaneous tissue and the maximal tumor thickness of 13mm, she has been free of tumor for 3 years. A review of the literature revealed twenty-four cases of congenital malignant melanoma. Discussion was made on prognosis of these cases.