Twenty patients aged 5 days to 14 Years underwent implantation of cardiac pacemakers and were followed up for an average of 6 years (range 8 months to 6 years and 2 months) . Complete heart block was present in 17 patients and sinus nodal dysfunction in three. The etiology of heart block was surgical in 14 cases, congenital in three. Five patients had symptoms of cardiac failure and one had syncopal attack. Pacing was required in 14 patients for postoperative rhythm control. One patient died of prosthetic valve malfunction after 11 months of pacing. Others are all alive and well. The VVI mode was used in all. For lead insertion, a left anterolateral thoracotomy was used in 19 patients, a sternotomy in one. The pacemaker generator was implanted in extrapleural space in five cases, and beneath the fascia of the left upper abdominal rectal muscle in 15. As complications of pacemaking, infection occured in three, lead fracture in two, high pacing threshold in two and muscle twich in one. No difference was noted in the incidence of complications between the two implantation sites. The intrathoracic implantation presented technical difficulties at the time of generator change compared to the implantation in the abdominal wall. We consider that the implantation beneath the fascia of abdominal rectal muscle is a procedure of choice for infants and children in whom repeated generator changes are required in the future.
To investigate the predisposing factors of hypokalemia following extracorporeal circuletion (ECC), 31 patients with various congenital heart diseases were retrospectively studied. Fourteen were male, 17 were female and their ages ranged from 1 to 69 months (38±18 months). Cardiac diagnosis were ventricular septal defect 14, atrial septal defect 7, pulmonary stenosis 2, tetralogy of Fallot 3, transposition of the great arteries 1, double outlet right ventricle 1 and pulmonary venous drainage 3. Plasma potassium were routinely studied before and during operation, and was studied hourly after closing chest for 24 hours. Thirteen patients developed hypokalemia (< 3.0mEq/L) within 6 hours after closing chest (Group I), while 18 patients maintained normal plasma postassium throughout the procedures (Group II). Following results were obtained. 1) Significant decrease of plasma potassium in Group I initiated within 2 hours after closing chest. 2) After termination of ECC, urine volume was larger in Group I than in Group II for 12 hours. 3) In Group I, plasma potassium correlated positively with Pco2 and inversely with PH. 4) Age, body weight and preoperative plasma potassium in Group I revealed statistically significant differences (P < 0.05) from those in Group II. 5) Cyanotic heart disease was more prevalent in Group I than in Group II. 6) Deep hypothermic ECC was more commonly employed in Group I than in Group II and the difference was statistically significant (P < 0.05). 7) Perioperative dosage of diuretics and catecholamines did not reveal statistically significant differences between the groups. These results drow us to conclude that, in infants and children who undergo intracardiac repair with the aid of ECC, younger age (less than 2 years), low body weight, low preoperative plasma potassium, are the strong predictors of hypokalemia at the immediate post-operative periods. Cyanotic heart disease and deep hypothermia during ECC may predispose hypokalemia after surgery although neither of these disclosed statistical significance. Both increased urinary excretion of potassium and respiratory alkalosis may exaggerate hypokalemia in this setting.
Eleven members of a family with MEN2A who were 19 years old or younger were evaluated from the viewpoint of appearance of clinical signs or symptoms during infancy, childhood and adolescence and a screening program with measurement of serum calcitonin concentrations with pentagastrin stimulation (O.5μg/kg 5sec. I. V.) was perfomed for the early diagnosis of medullary thyroid carcinoma (MTC). This screening revealed 2 additional patients aged 16 and 19, and total thyroidectomies with regional lymph node dissections were performed. For early detection of MTC in those at high risk for MEN2A, screening programs should be established and performed in their childhood and adolescence on the basis of the precise natural history of this syndrome.
Martin's operation is a standard procedure for total colonic aganglinonsis (TCA) . Recently several complications after Martin's operation have been reported. Four patients who were operated on by original and modified Martin's operation were studied on postoperative complications and postoperative course. One case was Hirschsorune disease with colonic atresia. The other three cases were TCA. Original Martin's operation was performed in case 1 and 2. Modified Martin's operation was done on case 3 and 4. Early postoperative complications were anastmotic leakage and ileus due to adhesion. Most sereous complication was postoperative enteritis. Such complications were successfully managed by irrigation and finger bougie. In general postoperative course were satisfactry. In conclusion, though the results of Martin's operation are satisfactory, long term follow-up and modifications of the procedure are required.
The incidence of infectious diseases and changes of immune function after partial splenic embolization (PSE) were studied prospectively in five children with portal hypertension. These patients had no severe infectious diseases, whereas six among seven patients with portal hypertention, who were treated by splenectomy, showed some infections. Serum IgA, IgG and IgM levels showed a moderate increase in the PSE group compared with those in the splenectomy group, but the difference was not significant. T-cell subsets did not change in the PSE group; on the other hand OKT 3 and OKT 4 decreased significantly in the splenectomized patients. PSE in children with portal hypertension is a more useful treatment than splenectomy as it does not depress the immune function.
We analysed immediate and late results of 12 biliary atresia patients who underwent hepatic portocholecystostomy. Postoperative cholangitis was not evident after this procedure. Jaundice disappeared in 6 of 12 cases. One of these 6 cases required re-operation by hepatic portojejunostomy before clearance of jaundice, because this patient developed biliary peritonitis after hepatic portocholecystostomy. Five of 6 cases whose corrective operations failed eventually died of hepatic failure. We could not find any relationship between the surgical results and the junctional form of the pancreatic duct and the common bile duct. The clinical problems in jaun-dice-free patients who had this procedure were slow resolution of jaundice and late complication such as portal hypertension. We conclude that, although hepatic portocholecystostomy is effective for prevention of postoperative cholangitis, this procedure is not thought to be the operation of choice for patients with subtype "a" biliary atresia.
Late complications were observed in 33 of 98 patients who have survived for more than 4 years after operation of biliary atresia. There were cholangitis associated with jaundice in 16 patients, symptomatic portal hypertention or hypersplenism in 15, rupture of esophageal varices in 9 , recurrence of jaundice without cholangitis in 5 , ileus in 3 , intestinal bleeding in 2 and so on. These complications were seen in any age groupe from 4 to 31 years old. It is concluded that the high risk patients for late complications are those with history of cholangitis in the early postoperative courses, with high portal vein pressure at the time of corrective operation with a history of late operation (later than 91 days of age), and with slow clearing of jaundice more than 3 months.
Seven children with urachal anomalies were encountered in this institution during a 5-year period from 1984 to 1988. Of these 7 cases, 1 was classified as urachal hypogenesis, 1 as urachal cyst, 1 as urachal vesical diverticulum and 4 were classified as urachal umbilical sinus. Ultrasonography seemed to be the most effective tool for diagnosis. All cases except one, in which the urachal cyst resolved spontaneously, were treated by excision. In cases with an umbilical sinus, coring out the opening of sinus was helpful to prevent peritoneal injury. From the aspect of genesis of the urachus, we proposed a new classification for urachal anomalies as follows 1 (1) urachal dysgenesis was classified into (1) a urachal agenesis and (1) b urachal hypogenesis, and (2) urachal remnant was classified into (2) a urachal vesicoumbilical sinus, (2) b urachal umbilical sinus, (2) c urachal cyst and (2) d urachal vesical diverticulum.
We have observed 5 cases of ileal perforation in Premature infants without necrotizing enterocolitis. These 5 cases had inspissated meconium in the distal part of the perforated ileum and no apparent necrotic change was observed around the perforation site. Typical caliber change could be observed in ileum from the distal site of perforation and the entire colon showed the features of unused microcolon. Multiple biopsies of narrow segments and microcolon showed normal sized ganglion cells and their population was also normal. A study of the trypsin activity of meconium in these perforated 5 cases demonstrsted that all patients whose meconium could be collected showed normal trypsin activity. These findings indicate that these 5 ileal perforations occurred due to meconium ileus without mucovicidosis. (Meconium disease) There have been a number of reports which described meconium disease in Japan but no reports of perforation due to meconium disease. Seven out of 25 patients who have been reported in Japanese articles were trested only with Gastrografin irrigation or enema and the remaining patients were treated by surgical ileostomy. The fact that ileal perforation can occurr due to meconium disease and the difficulties of differential diagnosis between meconium disease and aganglionosis of the entire colon indicates that surgical intervention might be the treatment of choice even in patients without perforations.
Twenty-seven patients with neuroblastoma discovered by a mass screening program for infants in Kyoto, Japan, during the 16-year period from 1972 to 1988, have been treated at our hospital. All patients were diagnosed as having a neuroblastoma before 9 month of age and were classified as follows '. 1 patients in stage I, 6 in stage II, 5 in stage IVs, 2 in stage III, and 7 in stage IV. All patients received surgery for removal of a primary tumor mass and chemotherapy. The initial 3 patients in this program received radiotherapy. Twenty two patients were treated by intravenous vincristine (VCR) and cyclophosphamide (CPM) administration for 6 months followed by oral CPM administration for 6-12 months. Four of the remaining 5 patients were in stage IV and were treated intensively by a combination of drugs such as VCR, CPM, THP-ADR, and CDDP. Twenty-six patients (96%) are alive now. One patient with stage IV diseses relapsed and died following the progress of disease. As a result, we suggest that for patients in stage I, II, IVs, chemotherapy of a combination of VCR and CPM intravenously for 6 months is satisfactory. However, for patients with stage IV, disease more intensive chemotherapy such as a combination of VCR, CPM, THP-ADR, and CDDP is more effective. The necessity for oral CPM administration is discussed.
Anthropometric measurements of 171 postoperative children, who had radical operations for neonatal surgical diseases from 1974 to 1985 in our institutions, were performed. Twenty-two percent of those out-patient children were classified as stunted (H/A<95%), and 26% as wasted (W/H<90%) according to Waterlow's classification. About 40% of the children had mild protein-energy malnutrition, although theier visceral protein status was preserved. In paticular, wasting was noted in patients with congeital esophageal atresia, abdominal wall defects and Hirschsprung's disease, while stunting was noted in patinets with congeital duodenal atresia. The usefulness of H/A and W/H for evaluating the long-term prognosis of post-operative children is discussed.
A child of malrotation with volvulus was successfully treated by second look operation. The first surgery revealed the extensive poorly-circulated small intestine due to volvulus, which was simply reduced with the intension to avoid massive resection of the small intestine. The 2nd look operation was performed 39 hours thereafter, revealing an improvement in circulation of the lesion. Resection of small intestine was avoided, but superior mesenteric vessels remained occluded. Selective superior mesenteric angiography, taken 15 months after the first operation confirmed the occlusion. Now, the child presents good development 6 years there-after. In this report, the outline of the course of the child and her angiographic and pathohistological findings are presentpd.
Iatrogenic esophageal perforation has been increasingly recognized as a complication of modern neonatal resuscitation occurring most frequentry in the premature infants. It usually results from endotracheal intubation, vigorous oropharyngeal suctioning or the passage of a nasogastric tube. The clinical and roentgenographic findings in this condition can mimic esophageal atresia or esophageal duplication. Two cases which were misdiagnosed as esophageal duplication are reported. Case 1 is an infant with submucosal penetration of the esophagus because of vigorous oropharyngeal suctioning after birth. An esophagogram demonstrated a classic "double esophagus" that showed a long narrow tract parallel and posterior to the esophageal column. She was mistakenly operated on with a diagnosis of esophageal duplication. Case 2 is a premature baby weighing 960g at 26 weeks gestation. She required endotracheal intubation, causing esophageal perforation into the retromediastinum. An esophagogram showed a blind pouch that ended at the level of the diaphragm. Both cases were treated successfully with conservative management. In general, esophageal perforation in neonates can be managed medically with removal of a nasogastric tube, administration of antibiotics, peripheral intravenous nutrition or feeding through a nasogastric tube when possible. Closed chest dranage may be necessary in some cases. This iatrogenic disorder should be prevented with caution.
A ten-month-old girl was admitted to our hospital with the complaints of stridor and cyanosis. Chest computed tomography and echocardiography revealed the intrapericardial tumor with massive pericardial effusion. Operation was performed through median sternotomy. The tumor occupied the right lateral portion of the pericardial cavity and was attached to the ascending aorta. The tumor (5.5 × 5 × 3cm in size) was completely removed, and the postoperative course was uneventful. Histological diagnosis was mature cystic teratoma. She was the 68th patient with intrapericardial teratoma ever reported in the world.
A case of 2-year-11-month old girl with pulmonary blastoma assosiated with left kidney agenesis was reported. The tumor originated in S8 segment of the left lung. As the pathology of this tumor was compatible with Wilms' tumor, the patient recieved chemotherapy according to the protocol of NWTS-III after removal of the tumor. Three years and 1 month after surgery, she was alive and well without any sign of reccurence. From the world literatures, we have reviewed 40 cases of pulmonary blastema in children younger than 15 years of age. Of these cases, we summalized on the characters of this tumor as;(1) they were 1.5 times commoner in girls than in boys, (2) they were commoner in younger children less than 4 year of age, and (3) they were commoner in lower lobes without a bias of the laterality. Two-year survival rate of the tumor in childhood was taken to be far better than in adulthood.
Two infants with Cornelia de Lange syndrome (CDL syndrome) associated with congenital duodenal atresia and esophageal hiatal hernia are reported.This syndrome was characterized by typical facial features, deformities of the extremities, growth and developmental delay, and was complicated by congential duodenal atresia and esophageal hiatal hernia. Both of infants were born after full term gestation, were small-for-date, and showed frequent vomiting after birth. They underwent gastrojejunostomy or duodeno-duodenostomy for duodenal atresia in their neonatal periods, but vomiting and recurrent respiratory infections were persistent after their operations. Subsequent upper Gl contrast studies performed at 10 or 17 months of age revealed hiatal hernia with gastresophageal reflux (GER). Nissen fundoplications were performed for GER with satisfactory results. CDL syndrome associated with gastrointestinal abnormalities is rare. Our cases suggest that early diagnosis of associated abnormalities followed by appropriate surgical treatment improves the delayed physical development and clinical manifestations.
We experienced a very rare familial occurrence of congenital esophageal atresia with trachoesophageal fiatula of Gross C type in a brother and a sister. The elder brother underwent two stage operation because of pneumonia at the time of admission, and the younger sister had one stage operation. Both siblings,currently 4 years and 2 mounths old,and 1 year old respectively, have been doing well expect for mild growth failure. Familial occurrence of esophageal atresia had been denied. However, because of 43 pairs with this disease were reported in literatures, there is a need to change the conventional belief. In Japanese literatures, there were three reports of familial occurrence in a brother and sister (Fujimoto, 1962), in identical twins (Yamada, 1968), and in brothers (Tauchi, 1983). Our case is the fourth pair with this condition so far reported in japan.