Late effects of tele cobalt 60 therapy on bones and soft tissues were studied radiologically in 24 patients with neuroblastoma and Wilms' tumor. The degree of changes in spinal bodies was influenced by the dose of irradiation as well as the age of patients at the time of irradiation. In patients who had 15 to 19 Gy of irradiation at the ages under one year old, a moderate to severe degree of changes was observed. Many patients showed atrophies of iliac bone, ribs, and erector spinae and psoas muscles on the side of the irradiation. In patients who were equal to or over 12 y.o. at the time of the examination, the degree of atrophy of erector spinae muscles on the side of the irradiation was greater than that of the patients who were less than 12 y.o.. Scoliosis was observed in 71% of patients and it had a tendency to aggravate at puberty. Because there was a significant correlation between the degree of scoliosis and the severity of the atrophic erector spinae muscle, the latter was thought to contribute much to the developement of the former. At present, all patients are living with no limitation of their daily activities and no one needs medical care.
Infiltrating pattern of inflammatory cells and expression of gamma-glutamyltransferase (GGT) were examined by immunohistological and histochemical techniques in the liver of biliary atresia (BA). Twenty four specimens were obtained from 20 case of BA at the time of the radical, re-boring operation or conversion of enterostomy. The monoclonal antibodies used in these studies were CD4, CD8, CD11, CD15, CD20, antibodies to IL-1 and HLA D/DR. GGT was stained by Rutenberg's method. The area of GGT positively stained and numbers of infilatrating cells were surveyed by the immage analizer. The inflammatory cells were composed of CD4^+ (helper/inducer T) cells, CD8^+ CD11^- (cytotoxic T) cells and CD15^+ (granulo/mono) cells. The infiltrating pattern at the place of canaliculo-ductal junction (C-Dj.) was characterized by collection of CD8^+ CD11^- (cytotoxic T) cells and CD4^+ (helper/inducer T) cells. At those C-Dj., it was observed that hepatocytes and bile duct epithelial cells were progressively damaged and fell off. The cellular damage at C-Dj. led proliferation of bile duct, where GGT and deposition of bile pigments were induced intensively. Once the inflammation of C-Dj. spread into the hepatic lobule, the bile pigment and GGT, which cannot be seen normally, were observed at the bile canaiculi, because of the obstruction of bile transporting system. The number of infiltrating cells, the area of GGT and depositing bile pigment were closely related with serum total bililubin, serum GGT and other laboratory data.
Nine patients with intermediate- or high-type imperforate anus underwent posterior sagittal anorectoplasty: 7 as a primary treatment and 2 as a re-do operation. Serial assessments on anorectal function were carried out over a period of 8-21 months after operation. Frequency of bowel movement decreased in early postoperative day in all cases, indicating that fecal continence was gradually obtained. Also in re-do cases, rectal sensation and fecal continence were improved after the surgery. The anorectal manometric studies showed high maximal anal pressure, rhythmic wave and anorectal reflex, whereas no remarkable changes were observed in re-do cases. Barium enema studies showed no abnormal findings.
Urinary saturation with calcium oxalate and stone parameters were studied with special reference to ages in both non-stone-forming and stone-forming children. The CaOx-risk indices used in order to estimate the calcium-oxalate crystallization levels, in 92 hospitalized patients thus far without any stones and in 9 patients with confirmed urinary-tract stones were 689.9 and 1373.0 respectively. The mean CaOx-risk index of 92 stone-free children decreased as a function of age, without any special peak age. This decreasing trend was closely associated with the mean urinary citrate and magnesium concentrations, which increased as a function of age. The urinary calcium and creatinine concentrations also increased with age, on the average; while the urinary oxalate concentration remained low, also on the average. Even if no special metabolic disease, such as hyperoxaluria, seems to be present, care must, therefore, be taken as early after birth as possible with those patients exhibiting high indices; such indices may often indicate hypocitraturia and / or hypomagnesiuria.
Development of defecation in normal infants and children was analysed, based on inquiries regarding frequency and regularity of bowel movement, defecation during sleeping, call for defecation and self-control of defecation. Bowel movement was more than 2 times daily till 1 year of age, then gradually decreased in frequency, and became once everyday after 4 years of age. Defecation during sleeping disappeared after 6 months of age, and call for defecation began from 2 years of age, and all normal infants after 4 years of age called completely for defecation. Self-control of defecation was established between one year and a half and 4 years of age. The present study disclosed that clinical assessment of anal continence in postoperative infants with ano-rectal malformation and Hirschsprung's disease should be evaluated after 4 years of age, because self-control of defecation was completely established in normal infants after 4 years of ace.
Seven infants with hypoplastic left heart syndorome underwent palliative surgery of the original Norwood stage 1 operation in 4 babies, Doty's method in one, and the modified Norwood stage 1 in two. Only a 14-day-old baby survived the modified Norwood operation. All patients required prostaglandin El infusion, all but one also required catecholamine support, and five needed ventilatory support, preoperatively. Causes of death were myocardial damage in five, residual coarctation of the aorta or acquired stenosis of the new aorta (stenosis of the new outflow tract to the systemic circulation) in four, bleeding in two, high pulmonary blood flow in one and sepsis in one. Operation was neccesary within 5 days of life in 3 babies with closing foramen ovale who did not survive. Analysis of the experiences suggest that adequ-ate relief of the coarctation of the aorta, reconstruction of non-stennotic outflow tract to the systemic circulation, adequate size of the shunt, and good myocardial protection might be mandatory to obtain good outcome.
Controversies exist regarding indications for diagnostic angiography, and the optimal surgical management of pulmonary sequestration. This paper summarizes our diagnostic procedures employed and operations undertaken for 12 patients experienced in the last 18 years. During the period between April 1970 and March 1988, 12 patients with pulmonary sequestration were treated at the Kobe Children's Hospital. The patients' age ranged from 4 days to 7 years. They were 7 males and 5 females. Eight patients had intralobar sequestration (ILS) and 4 patients had extralobar sequestration (ELS). Angiography was done in all patients in whom ILS was suspected. This provided the definitive diagnosis with precise informations of arterial blood supply and venous drainage of the sequestered lobe. On the other hand, most of the patients with ELS were not examined by angiography, and were operated on with preoperative diagnosis of mediastinal tumor or diaphragmatic eventration. Surgical procedures undertaken in ILS include lobectomy in 4, partial or segmental resection of the sequestered lobe in 3, pneumonectomy necessitated with bilobate ILS in one. Surgical procedures in ELS include resection of the sequestered lobe in 3 and pneumonectomy in one which was unavoidable due to a progressive pulmonary tension emphysema after resection. Two patients died of respiratory failure following pneumonectomy, but others were free of any complications. In conclusion, angiography is recommended for ILS, but can be omitted for ELS when it is suspected because ELS has no vascular communication to pulmonary vein. Regarding operation, partial resection should be attempted in case of ILS in order to preserve the normal lung as much as possible in children.
A long-term follow-up study on 12 patients following palliative atrial switch operation for complete transposition of the great arteries (d-TGA) associated with pulmonary obstructive vascular disease is presented. The operations were done from 1977 to 1987. Preoperative mean pulmonary vascular resistance was 18.6 ± 8.0 unit. In 5 cases, a ventricular septal defect (VSD) was left open, and in 7 patients VSD was closed with a patch having a central hole, 5 mm in diameter. There were one operative death and three late deaths. Preoperatively all cases were in New York Heart Association functional class III. Mean PaO_2 was 39.7±7.2mmHg. Postoperatively 5 cases were in functional class I and 2 cases in class II. PaO_2 improved to 62.8±7.9 mmHg. In 7 survivers, postoperative Treadmill test was done. The results were as follows : endurance time showed above 90 percentiles of normal in one case, 50 percentiles in one, 25 percentiles in one, 10 percentiles in one, and under 10 percentiles in three. Before and after exercise, arterial oxygen saturation decreased from 95.8 ± 2.6 to 74.5 ± 16.7%. Effective funtion of holed patch was recognized. In postoperative cardiac catheterization, 2 cases who had perforated patch, suggested improved pulmonary hypertension (Pp/Ps : 0.21 and 0.41). Palliative Mustard operation provides excellent symptomatic improvement in all survivors.
To determine a favorable treatment for lymphangiomas in children, we reviewed a 9-year (1980. 1-1988. 4) ex-perience with 39 lymphangiomas in children. The patiets were divided into 3 groups according to the treatment; Surgical Excision (N = 12), Intralesionl Bleomycin (BLM) Therapy (N = 5), and Intralesional OK-432 Therapy (N = 22). Excellent results including complete disappearance of the lesions without serious complications was noted in 4 of 12 treated by surgery, in one of 4 treated by BLM therapy, and in 14 of 22 treated by OK-432 therapy. Serious complications such as facial nerve palsy (1), ugly scars due to repeated excision (3) and testicular atrophy (1) were noted in surgically treated group. In the other groups, only a fever of 2-3 days' duration and local in-fammatory reaction of 3-5 days were noted. BLM therapy, however, was discontinued because in addition to low effective rate BLM was reported to have a serious side effects of pulumonary fibrosis. The results suggest that OK-432 therapy is effective for these lesions.
From June 1984 to December 1987, endoscopic examination of the upper gastrointestinal tract was performed in 11 infants with hematomesis and/or melena. Six of them were male. Before examination, intravascular atropine sulfate 0.01mg/kg and hyoscine butylbromide 0.5mg/kg injections were given and lidocaine was sprayed on the posterior oropharynx. The patients were examined in left lateral decubitus position. The panendoscopes used were Olympus GIF X-P and X-P_10. In the all 11 patients, endoscopic examination demonstrated positive findings : esophageal ulcer and erosion in five, gastric ulcer in eight, gastric erosion in four, hemorrhagic gastritis in four, duodenal ulcer in one. Seven patients had two and more lesions. Six patients were associated with the precipitating causes (asphyxia, intracranial bleeding, MAS, Bochodalek's hernia). The endoscopic follow-up study was carried out in six patients. Esophageal ulcer, esophageal erosion, gastric erosion and hemorrhagic gastritis were completely healed 1 week after the first examination. Four of 6 gastric ulcer lesions were changed to scar within 1 week, but in two, the duration of reaching to healing stage was several weeks. Endoscopic examination of upper gastrointestinal hemorrhage in infants was useful and accurate diagnostic method.
Microsurgical orchidopexy was successfully performed by retroperitoneal approach on a 2 years and 5 months old boy with a right abdominal testicle. A Skin incision was made as a usual inguinal oblique incision and was extended by 10cm vertically from lateral end of the incision. External and internal oblique musles were cut and retroperitoneal space was roughly opened widely which enabled dissection of right testicular artery and vein to their point of connection with abdominal aorta and caval vein. Testicular vessels were cut at that point and anastomosed to the right inferior epigastric vessels end to end under operating microscope with 10-0 Nylon sutures. The testicle was fixed in the scrotum. Two years have passed after the operation and the testicle is growing normally in the scrotum. The microsurgical orchidopexy by retroperitoneal approach has its merits especially for young infants, as it gives sufficiently wide operating fieid and safe and smooth postoperative recovery without any trouble due to intestinal adhesions.
We report a case of bilateral cystic nephroblastomas, associated with prominent intralobar nephroblastomatosis, diagnosed at the age of seven months. The infant also had microcephalus and an arachnoid cyst of the posterior fossa. Three previous cases of bilateral cystic nephroblastoma have been reported, all from Japan. Two of these patients also had sarcoma botryoides of the urinary bladder, and all had malformations of the central nervous system. These cases appear to constitute a distinctive dysplastic-neoplastic syndrome, and add to the list of syndrome associated with nephroblastomas.
Case one : A 3-day-old girl with birth weight of 2.3kg was admitted for esophageal atresia and ano-rectal malformation. Chest X-ray showed complete opacification of the right lung field. Gastrostomy and cut-buck operation were performed. Inspite of vigorous treatment, she died of respiratory failure associated with septic shoch on 9th day. Autopsy revealed esophageal atresia and right lung agenesis with ectoplastic lung tissue arising from lower esophagus. Case two : A 1-day-old boy with birte weight of 1.9kg was admitted for esophageal atresia and RDS. Gastrostomy and lower esophageal banding were performed immediately. After recovering from RDS, primary anastomosis of the esophagus with division of TEF and removal of the banding tape were succeeded on the 5th day of life. Post operative course was complicated with both respiratory and cardiac failure, but he recovered by intensive treatement including "High Frequency Jet Ventilation". The right pulmonary agenesis was confirmed by lung scintiscanning and CT. He also had ASD, PDA and still have severe GER at 7 months of age.
Intra-renal teratoma is extremely rare and only 10 cases (7 children and 3 adults) were reported in the world literature including a Japanese adult. We report the first child case in Japan. One-year-old girl was admitted to our hospital because of an abdominal tumor. Complete surgical removal of the tumor was done. This tumor, 16X13X5 cm in size and 1040 g in weight, originated from the right kidney and was diagnosed as mature intra-renal teratoma, composed with three embryonic layers pathologically. She has been in tumor-free condition about 6 years and a half now post-operatively.
Two survival neonate of ruptured spleen are reported. Both infants were admitted because of abdominal disten-sion and severe anemia. Emergency laparotomy disclosed splenic rupture and splenectomy was performed. Forty-one percent of splenic rupture occurred within 24 hours after birth. Special care must be taken however, because symptoms may not develop untill several days after birth. Although splenectomized infants are susceptible to serious infection, splenectomy was judged to be the most reliable procedure to stop bleeding from the ruptured spleen. No prophylactic antibiotics was administered postoperatively and no serious infection has since been observed.
A tweleve-day-old male neonate was referred to University of Tsukuda because of dyspnea necessitating endotracheal intubation and ventilation soon after birth. He was suspected of having vascular ring by esophagography. Angiography confirmed the diagnosis of double aortic arch (Stewart & Edwards classification IA). On the fourteenth day after birth, the anterior aortic arch was divided just distal to the left subclavian artery and suspended to the anterior chest wall through left thoracotomy. Bronchoscopy performed after the procedures revealed severe tracheomalcia. In spite of the definitive operation, he could not be weaned from a respirator. After a few unsuccessful attempts to extubate. the second operation was performed 15 days after the first operation. At that operation, the ascending aorta and the right aortic arch were suspended to the 2nd, 3rd rib and sternum under direct vision by bronchoscopy. He was successfully extubated on the 9th postoperative day. He was discharged in 3 months and has been doing well up to the present. The experiences of this case has suggested : (1) aortopexy is a useful option for tracheomalacia associated with vascular ring, (2) bronchoscopy should be performed in cases of vascular ring with dyspnea to detect the co-exsitent intrinsic airway obstruction.