Between 1961 and June, 1989, 23 patients with Ebstein's anomaly were evaluated at Sapporo Medical College. Three patients were followed without surgery and two of them died with congestive heart failure at 9 months and at 10 years after the follow up. Eleven patients had four different types of palliative operations (Glenn in 5, Blalock-Taussing in 2, Brock in 3, and closure of the intra atrial communication in 4). Two patients had Hardy's plication. But none of these 11 patients with palliative operations and two patients after Hardy's operation survived for long-term periods. Twelve patients had tricuspid valve replacement with one operative and three late deaths. Eight patients were followed ranging from 6 months to 24 years (mean 11years). Six of the eight survivors were in NYHA class I, and two were in class II. Clinical results of valve replacement for Ebstein's anomaly were satisfactory, and it provided excellent long-term results.
The serum and tissue levels of the α subunit of GTP-Binding Protein Go (Goα) was measured to know its possibility of a tumor marker for neuroblastoma. The concentration of serum Goα was elevated in 77% of the 57 neuroblastoma patients. The concentration of tissue Goα in neuroblastomas was higher about 10 folds than those of other pediatric solid tumors. The relationship between the prognosis of neuroblastoma patients and the serum Goα level was found statistically significant. These data suggest that serum Goα is an useful marker for the diagnosis of neuroblastoma and the prediction of the patients prognosis, and that investigations of Goα in tumor tissues are useful for the differential diagnosis of the neuroblastoma.
An 11day-old boy with 13q-syndrome associated with Hirschsprung's disease is reported. He was admitted to the NICU in our hospital because of SFD. Abdominal distension and vomiting had been seen since he was 5 days old because of Hirschsprung's disease. Diagnosis of Hirschsprung's disease was established and a colostomy was performed at 13 days of age. A cytogenetic study was carried out and interstitial deletion of the distal long arm segment of chromosome 13 was observed. The karyotype was 46, XY, del (13) (q14.3-q32.1). Cases of 13q-syndrome associated with abnormalities of the gastrointestinal tract are rare. Four cases of 13q-syndrome associated with Hirschsprung's disease have been reported, including our case, and in two of them resection of the affected segment was performed. At 7 months of age, his body weight is only 5200g, there is evidence of mental retardation and attacks of cyanosis are sometimes seen.
Retrospective study was carried out on 70 infants weighing less than 6kg who underwent open heart surgery for congenital heart disease to clarify the interrelation between the intraoperative factors (perfusion flow, pressure and lowest rectal temperature) and the water balance and urinary output during total cardiopulmonary bypass (CPB). The urinary volume during total bypass corelated significantly with the perfusion flow, lowest rectal temperature (RT) during CPB and especially with the perfusion pressure (r= 0.735, p<0.001). The water balance (BAL) during CPB corelated especially with RT (r=-0.704, p<0.001). When patients were divided into three groups by the priming components : A Blood containing CPD and ringer solusion (18.8 ±3.2% dilution) ; B : heparinized blood (3.7± 0.7%) ; C : heparinized blood and fresh frozen plasma (18.0 ± 2.5%), the urinary output during CPB was maintained well in group (B) and (C). The water infusion during CPB was minimized in group (C). In group C, the water balance did not corelate with urinary output but corelated with RT (r=-0.704, p<0.001). Among 15 infants in group C who underwent the closure of ventricular septal defect, postoperative AaDO_2 corelated significantly with BAL (r= 0.450, p<0.01) and RT (r=-0.744, p<0.001) . These data suggest that the excessive core cooling should be limitted to reduce the body fluid shift into interstitial apace and the sufficient perfusion flow to achieve enough pressure (< 70mmHg) would be important to maintain the renal function during CPB. Our recent protocol for CPB have the followings as an objects ; high perfusion flow (2.4-3l/min/SQm) ,high perfusion pressure (more than 70 mmHg as systolic) and moderate hypothermia (more than 28℃ as RT) under the primings of heparinized blood and frozen plasma.
The Wangensteen-Rice invertography has often been used to judge the level of the rectal blind pouch of an imperforate anus in newborn infants. This modality, however, possibly can lead a misdiagnosis on the pouch level when the swallowed gas does not descend to the distal-most part of the blind bowel. Two newborn babies with low defect were erroneously considered as high defect by the invertography due to an impacted meconium in the blind pouch and an excessive contraction of the puborectal muscle complex. While, 4 infants were accurately evaluated the pouch levels by a real-time ultrasonography using 5 -MHz mechanical sector scanner. Two of them were exactly diagnosed as low defect with the distance between the pouch level and the perineal surface (P-P distance) measuring 5 mm each by ultrasonography, and the remaining 2 were high defect with the P-P distance measuring 19mm and 30mm, respectively. Real-time ultrasonography with small scanner can provide a non-invasive and accurate method to determine the site of the distal-most rectal pouch in babies with imperforate anus shortly after birth. Ultrasonography should be performed with the invertography for the level diagnosis of an imperforate anus in newborn infants.
Predeposited autologous blood transfusion (PABT) can protect patients from unnecessary exposure to allogenic blood. We report our experience of 10 pediatric surgical patients who received PABT. Ten patients ranged in age from 6 months to 13 years with weight of 18 ± 12kg. There were 3 choledochal cyst, 3 anorectal anomaly, 1 hydronephrosis, 1 pulmonary sequestration, 1 mediastinal tumor and 1 pectus excavatum. Prede-posit was carried out 1-3 times 1-2 weeks prior to the operation. We used blood storage bag specially designed for children. The mean volume collected per patient was 11.3±5.1ml/kg. Eight patients received iron supplement during the period from immediately after the blood withdrawal to the operation. The percentage of the actual predeposit in prearranged blood was 81±26% (32-100%). The intraoperative bleeding exceeded the predeposit in 3 cases but none of them needed homologous blood transfusion. There was no bacterial contamination, coagulation, hemolysis of predeposit autologous blood. PABT would be applicable to infants and children provided that volume of predeposit is accurately predicted in each operation.
Tracheotomy in children has been associated with many complications compared to that in aduls in the medical literature. But the serious complications of long-term translaryngeal intubation for ventilatory support such as laryngeal injury are well known. Fourteen children less than 3 years of age who had tracheotomies for prolonged ventilator support from January 1988 to April 1991 are reviewed. Ten patients (71.4%) were under 1 year of age. The mean time of translaryngeal intubation prior to tracheotomy was 229 days. All patients had difficulties in weaning from mechanical ventilation before tracheotomy. All had an improvement with tracheotomy and eleven patients had been weaned from mechanical ventilation. Seven were discharged from the hospital to home care. None had early complication and 3 patients (21.4%) developed 6 late complications in tracheotomy. None of the complications of tracheotomy was serious. Subglottic stenosis by prolonged translarygeal intubation developed in four patients. The managements for these problems were difficult. The tracheotomy tube was replaced by the cuff button-like cannula in seven children who had improvements of respiratory state. Two of these 7 were decannu-lated safely. We conclude that tracheotomy can be performed safely, and for patients who require long-term intubation, we would decide to perform an early tracheotomy. The cuff button-like cannula is very effective for home care and decannulation.
Nissen-Hill fundoplication with gastrostomy has been carried out in 13 neurologically impaired children at our institutions over the past three and a half years. Six of the 13 patients has previously failed a trial of nonsurgical management with prokinetic drugs. No patients showed persistent vomiting and/or hematoemesis postoperatively. The surgical treatments were effective for stridor in four patients (33%), repeated pneumonia in 5 patients (83%), cough attack in 1 patients (50%) and apnea in 2 patients (100%), Enteral nutrition became possible after the operation in 3 patients who had been previously managed only by parenteral nutrition. Marked nutritional improvement was noted in most cases with significant increases in weight for age and elevated levels of Hb and serum Fe. The gastric bleeding in 2 patients, wound infection in 1 patient, intestinal perforation following small bowel obstrution in 1 patient, pulmonary atelectasis in 1 patient and dumping syndrome in 1 patient. The incidence of late postoperative complications was 31%, which included gas bloat syndrome in 3 patients and small bowel obstruction in 1 patients. Two of the children have died, none from causes related to the surgical procedure. Clinical follow-up evaluations of all survivors did not show a persistence or recurrence of gastroesophageal reflux during a mean follow-up period of 21 months. Nissen-Hill fundoplication with gastrostomy appears to be safe and beneficial with the disappearance of vomiting/hematoemesis, a significant weight gain and a satisfactory decrease of pulmonary disease in neurologically impaired children. Further research should be undertaken to improve the effects of surgical treatment.
Ninety-one children with perforated appendicitis treated surgically at the authors' hospital are reviewed. The organisms isolated from their peritoneal fluid at operation were 138 strains, such as Escherichia coli (37%), Bacteroides fragilis (23.2%), Pseudomonas aeruginosa (12.3%), Enterococcus species (7.2%) and et al. Though the antibiotics which was sensitive to Escherichia coli and Bacteroides fragilis and was not sensitive to Pseudomonas aeruginosa and Enterococcus species was chosen intra-and post-operatively, there were no postoperative complications such as wound infection and intraabdominal abscess. It is concluded that because there are little role in pathogenesity of Pseudomonas aeruginosa and Enterococcus species in perforated appendicitis, the antibiotics therapy for perforated appendicitis should be performed mainly for Escherichia coli and Bacteroides fragilis.
Three cases of pancreatoblastoma were reported with reference to clinical and immunohistopathological findings. Cases 1 : A 3-yesr-old girl was admitteed to Osaka University Hospital with a large abdominal mass. The tumor originated in the pancreatic tail and was resected. The patient died at 7 year of age because of multiple liver metastasis and local recurrence. Cases 2 : A 5-yesr-old boy was admitteed with a large upper abdominal mass. A tumor was arising from the pancreatic body and was resected. Althoug there was no recurrent sign, the patient died of unknown disease at 8-year-old. Cases 3 : A 5-yesr-old boy admitted with complaints of appetite loss and palpable abdominal mass. A huge abdominal mass arising from the pancreatic head with multiple liver metastasis was found. Biopsy was performed and the patient was treated with systemic and intra-hepatic arterial chemotherapy. The patient died 1 month after admission. Later pathological examination revealed all three cases being pancreatoblastoma. Immunohistochemical staining of tumor cells demonstrated the presence of CEA and NSE positive cells in all cases, α-1 antitrypsin in case 1, 3 and α-fetoprotein in case 1, 2. However, insulin, glucagon, somatostain, pancreatic polypeptide were negative in all three cases.
The presence of mesenchymal tissue, such as bone or cartilage in the hepatoblastoma, is a well recognized occurrence. However, multiple lines of tissue differentiation in a hepatoblastoma have rarely been observed. Such case of so-called teratoid type hepatoblastoma is presented. A 3-year-old Japanese girl was admitted to Osaka University Hospital with a right upper abdominal mass. Serum α-fetoprotein level was remarkably elevated (208000ng/ml). NSE and CEA was slightly elevated. The tumor lesion occupied the right lobe of the liver. Extended right lobectomy was performed. The tumor was 12×10×9 cm in size, and consisted of multiple nodules. Post-operative chemotherapy was performed for one year. The patient is alive 5 years after the operation with good health. In this tumor, most lesion consisted of fetal type hepatoblastoma, but there were some areas showing embryonal hepatoblastoma. Furthermore, various component of tridermic tissues such as immature neural tissue, keratoid tissue, melanin producing cells, caltilage. bone, striated muscle, glands, and columnar epithelium were found in the tumor tissue. Immunohis-tochemical studies confirmed various differentiation of tumor cells into tridermic tissue and histological diagnosis was made as a teratoid type hepatoblastoma.
True hepatic teratoma is very rare. We have experienced a case of hepatic teratoma in a 14-month-old girl. This tumor was successfully removed with normal liver tissue surrounding the tumor by right hepatolobectomy. Microscopic examination showed a mature teratoma containing tissue representative of all three germ layers, which were intestine, bronchus, bone, cartilage. striped muscle, brain, and squamous epithelium. She did well postoperatively. A review of the literature on hepatic teratomas was also presented. There were 18 cases reported in the last twenty years, including 4 in adults and 14 in children. Only two out of 18 cases were malignant, and the remaining 16 cases were thought to be benign. Removal of the tumor was carried out successfully in 13 patients, and all of them are alive and well. At the persent time, surgical resection of hepatic teratoma has been most effective.
Congenital tracheoesophageal fistula without esophageal atresia is a rare anomaly, and 50 cases have been reported in Japan. A male infant 27 days old, 3106g of birth weight, with cyanosis and dyspnea at feeding was referred to our hospital. Esophagography and endoscopy revealed that he had tracheo-esophageal fistula without esophageal atresia. Fistelectomy was performed via the right cervical approach. The tracheoesophageal fistula without esophageal atresia can be strongly suspected by clinical symptoms, but it is very difficult sometimes to diagnose its existance correctly. Not only a verification of the presence of the fistula is important, but also it is very important to diagnosis its localization accurately for selecting a most applicable operative approach. Endoscopy is particularly important to demonstrate the fistula. And esophagography is also necessary examination for investigating associated anomalies in this lesion.
A female patient was diagnosed as having obstructive jaundice and underwent choledochocystoduodenostomy at the age of 45 days. Macroscopic biliary cirrhosis was observed. Liver biopsy and operative cholangiography were not performed. Postoperatively, she was free from jaundice but had ascending cholangitis frequently. When she was 9 years old, she was diagnosed as having portal hypertension and received detailed examinations. Cholangiography revealed pancreaticobiliary maljunction, the presence of distal bile duct and an abnormal finding of intrahepatic bile duct. The presence of remnant choledochal cyst was not clearly identified. Liver biopsy revealed the finding of mild hepatic fibrosis. It was impossible to determine whether she had choledochal cyst or biliary atresia.
A 20-month-old male with a gastric teratoma is reported. He was admitted to Osaka University Hospital with progressive upper abdominal tumor. Laboratory data showed slightly elevated plasma AFP and LDH levels. Calcified hepatic teratoma was suspected by preoperative examinations. At operation, the tumor was located at the lesser curvature and fused to the gastric wall. The tumor was considered to be of gastric origin. Tumor was removed together with the muscular layer of the stomach. Histological examination revealed combined teratoma with york sac tumor. Postoperative chemotherapy was not given. Since operation, he has been healthy without signs of recurrence for these 3 years. And plasma AFP level remains low. 101 cases of gastric teratoma were reviewed in the literature.
Synovial sarcoma is rarely encountered in childhood. A 4-year-old boy was admitted because of a recurrent soft mass located in the right hand joint. The mass was first removed at the age of 18 months, but recurred twice. Plain X-ray and CT-scan of the chest showed multiple coin lesions, and biopsy of the recurrent mass and the chest lesions revealed histologically that the boy had synovial sarcoma of the hand joint with pulmonary metastases. Almost all metastatic lesions were removed and the patient received chemotherapy, consisting of vincristine. actinomycin-D, cyclophosphamide, and VP-16. The boy is so far well 11 months after the last surgery. The pertinent literature was reviewed, and the diagnosis and management of this rarely encountered synovial sarcoma was discussed. It is emphasized that a mass lesion which may look like a "ganglion" should have histological examination in order to rule out a possiblity of this malignant tumor.
Cest wall chondrosarcoma in early childhood is extremely rare. A case of a four-month-old boy was reported. The patient, presenting poor feeding and dyspnea, was referred to Osaka University Hospital. X-ray, ultrasonic examination and CT scan revealed a mass at the inside of the left posterior chest wall with massive pleural effusion. Operation was carried out and the tumor was resected combined with the 7th, 8th, and 9th ribs. The chest wall defect was directly closed. The tumor was 6×4×3 cm in size, and the histological report showed chondrosarcoma (well differentiated type). The postoperative course was uneventful. Postoperative chemotherapy was not performed. Mild scoliosis remained but no respiratory disturbance. The patient is growing well with no signs of recurrence. Eleven cases of chest wall chondrosarcoma in children have been reviewed in the world literature.
A 22-month-old boy, whose VMA screening test was negative at 6 month after birth, fell from a height of 50 cm to get a bruise on the flank and became shocked. He underwent emergecy laparotomy as splenic rupture was suspected at a local hospital. But there was only massive retroperitoneal hematoma, and no bleeding was observed in the abdominal cavity. Because of continuous fever in the postoperative course, ultrasonographic examination was performed at our institution and adrenal tumor was suspected. Left adrenal tumor was removed and para-aortic lymphnodes were resected by surgery. We staged it as neuroblastoma stage 3 with findings of O_S ・ C_2 ・ N_2 ・ B_0 ・ E_0 ・ V_0 ・ Bm_0 ・ H_0 ・ D_0. We experienced rare neuroblastoma found by blunt abdominal trauma.
A congenital neuroblastoma diagnosed by sonography in a 14 day old girl is reported. A small adrenal mass on the right was found accidentally, when the patient was examined sonographically for a heart murmur. VNA and HVA urine levels were normal. <131>^I-MIBG scintingraphy was effective for the differential diagnosis of adrenal neuroblastoma and adrenal bleeding.
A boy aged 6 years was admitted with a history of recurrent attacks of non-bilious vomiting for approximately one year. Ultrasound, X-ray and endoscopic examinations showed nearly comlete obstruciton of the pylorus and malrotation of the intestine. Partial gastrectomy and Ladd's procedure were performed under the preoperative diagnosis of pyloric obstruciton due to channel ulcer. Pathologic examination revealed heterotopic pancreatic tissue (Heinrich type I) with periductal inflammatory reaction, marked hypertrophy of the pyloric muscle and nodular hyperplasia of Brunner's gland. The stenosis was caused by hypertrophy of the pyloric muscle associated with heterotopic pancreatic tissue and concomitant inflammation.
A case of mesenteric angiomyolopoma in a two-year-old girl, who had no evidence of tuberous sclerosis, was reported. She was admitted to our hospital with a complaint of abdominal tumor, two hard masses were palpable in her abdomen, one was 8×5 cm in size in the upper abdomen and the other was 13×6 cm in size in the lower abdomen. Abdominal ultrasonography and CT-scan revealed a large solid mass extending from the upper abdomen to the pelvic cavity. In the abdominal aortography and superior mesenteric arteriography, superior and inferior mesenteric arteries were compressed by the tumor, but neovascularization or tumor stain was not observed. MRI showed branches of the superior mesenteric artery in the tumor. The preoperative diagnosis was mesenteric tumor, and laparotomy was performed. The tumor was composed of cavernous solid parts, cystic parts, and focal adipose tissue, growing on both sides of the mesentery. The tumor was completely resected preserving the mesenteric vessels. Microscopically, the tumor was composed of vessels, smooth muscle fibers, and adipose tissue, and was diagnosed as angiomyolipoma.