Diaphrgmatic hernia was experimentally produced in fetal rabbits from 21st to 25th gestational day by rup-turing the left diaphragm in utero. The pulmonary vascular system of 18 out of 48 fetuses successfully recovered was examined. Radius and thickness of the media of the pulmonary artery were measured using Suwa's histometrical techniques. Total length of the acinar arteries and capillaries were measured by stereological methods. There were no differences between models and controls in the radius of the pulmonary trunk, segmental artery, and acinar artery. The proportion of the thickness of the media to the radius increased significantly in the models, and the total length of the acinar as well as capillaries was remarkably reduced in the models. These findings were all in the bilateral lungs. These results suggest that vascular disorders caused by diaphragmatic hernia in fetal life result in absolute reduction of total pulmonary vascular capacity.
Seven infants who had coarctation of the aorta (CoA) with ventricular septal defect (VSD) underwent surgical correction between 1983 and December 1987. All seven patients were less than 3 months of age at the time of operation. In six of 7 patients, preoperative cardiac catheterization was omitted to save time and to avoid deterioration of congestive heart failure. Diagnosis was established by two dimensional echocardiography combined with an aortography by radial arterial injection. A subclavian flap aortoplasty was used in all 7 patients for the repair of CoA and in the recent 3. simultaneous pulmonary artery banding was not performed. For CoA with VSD, we currently recommend the repair without pulmonary artery banding, followed by VSD closure if indicated. In 6 of 7 Patients, secondary VSD closure was performed 7 days to 15 months after the repair of CoA. There was no operative nor late death. All 7 infants have been followed up a period of 22 months on average and recurrent stenosis has developed in one of 4 patients who were less than 60 days old at the time of operation. We believe that subclavian flap aortoplasty is effective for the repair of coarctation of the aorta in infants, but if the patient is less than 60 days old, it is necessary to follow up strictly, because of a significant risk of recurrent stenosis.
To examine a method of the intraperitoneal administration of albumin for intrauterine treatment of nonimmunologic hydrops fetalis, fetal rabbits were used and albumin was administered to them with a catheter inserted in the fetal peritoneal cavity. The efficacy and safety of this method of administration were studied. Intrauterine surgery to introduce a silicone catheter into the fetal peritoneal cavity was performed on the 27th day of pregnancy in nine rabbits. This procedure was followed by the administration of 80mg of rabbit albumin for four days. After the administration on the 31st day of pregnancy, the fetuses were recoverd by Caesarean section or by vaginal delivery. Examinations of the fetuses with the indwelling catheter revealed no premature delivery or fetal hypoplasia and showed that the albumin-treated group had a significant increase in serum albumin level over the control group. By using another four pregnant rabbits measurements were made of fetal circulating blood volume of five fetuses by the dye dilution method. The results showed that after the administration of albumin for four days, the total dose administered in the fetal peritoneal cavity (320mg of albumin) finally produced an increase of 8.4% in the serum level of albumin in the fetal circulating blood. These results suggested that the administration of albumin by way of an inserted catheter is an effective method to increase the serum level of a albumin in the fetus.
In order to prove the clinical usefulness in diagnosis of neuroblastoma, comparative studies between iodine-131 metaiodobenzylguanidine (131^I-MIBG) scintigraphy and other related tumor markers were attempted. Sixteen children diagnosed as having a neuroblastoma in recent 2 years were examined. In 5 postoperative patients in complete remission, who were negative to other tumor markers, showed no pathological accumulation of 131^I-MIBG (specificity 100%). In other 11 patients with remains of neuroblastoma, 131^I-MIBG were negative only in 2 patients (sensitivity 82%) and these 2 patients showed negative urinary excretion of cathecholamim metabolites (VMA). (Negative urinary VMA was proved in 3 of 11 patients). Serum neuron-specific enolase (NSE) was elavated in all 8 preoperative patients, but only in 2 of 11 postoperative patients. On the other hand, 131^I-MIBG was positive in 9 among these 11 postoperative patients in whom neuroblastoma remained. Similar relationship was obtained between 131^I-MIBG scintigraphy and serum LDH. On the basis of our present experience, we like to regard 131^I-MIBG scitigraphy as one of the most sensitive parameters for neuroblastoma during a follow-up period after treatment.
Eighteen cases of diaphragmatic hernia who developed clinical symptom after neonatal period are reported. Their ages ranged from 1 month to 3 2/12 years. Five cases (28%) were rightsided diaphragmatic hernia. Three cases (11%) had hernial sac. Fourteen cases presented nausea, vomiting, appetite loss, etc. as primary symptoms, whereas 3 cases aged under 5 months presented respiratory distress. Primary repair was performed in all cases and postoperative courses were unesentful. In operative findings, 13 cases revealed an incarcerated organ to be only intestine, while other cases involved liver and/or spleen besides intestine. Its mortality rate is quite different from encountered during neonatal period, i.e. 31% (9/29). In 5 cases, previously taken X ray showed normal findings. This fact suggests that in those cases herniation may have developed after birth, and that normal X-ray views do not always deny letent diaphragmatic hernia.
Eleven cases of anomalous arrangement of the pan-creatico-biliary ductal system were studied biochemically and histopathologically including three cancerous lesion. Amylase levels in the bile as well as tripsin levels were extremely high in 7 cases out of nine. In the bile of choledochal cysts without cancer, deoxycholic acid fractions were increased, compared to those of the bile in the cholelithiasis with normal pancreatico-biliary union. In the bile with the cancer arising from the congenital cystic dilatation, taurodeoxycholic acid (TDCA) was increased. Lithocholic acid fractions (LCA) were increased in the bile with the bile duct cancer arising from the cylindrical dilatation as well as in the bile with the gallbladder carcinoma without biliary dilatation. The increasing of DCA and LCA were supposed to be a causative factor of biliary cancer with anomalous union, because these bile acid fractions are known to have the promoting effect on colon carcinogenesis. CEA was demonstrated in the cancer nest by an immunohistochemical staining method. CA19-9 was stained both in the cancerous part and in the non-cancerous epithels.
The alveolar hydatid disease of the liver is uncommon in childhood; 106 patients with this disease have been treated in our department during the past 50 years. Six were less than 15 years old. It was 5.7% of all 106 cases. Four were female and two were male. It will take more than 10 years to grow clinical symptoms in adults after infestation. The alveolar hydatid disease in children seems to gorw more rapidly than adult. Among the first three patients, two had hepatomegaly, and the third patients had liver dysfunction. The latest three patients were detected by serological test and ultrasonographic examination in the mass screening when the lesions were too small to produce symptoms. Hepatectomy were performed for five patients; one lateral segmentctomy, three lobectomy and one partial hepatectomy. They are all alive and doing well now respectively. One patient had an unresectable lesions and died after three years. For the good prognosis, an early diagnosis and complete removal of the lesion are indispensable. The alveolar hydatid disease occurs in a limited area. So the mass screening has been effectively performed.
A successful surgical case of anomalous origin of right pulmonary artery from ascending aorta was reported. The patient was a 7 month-old girl with poor weight gain, who had also patent ductus arteriosus and left pulmonary hypertension. Operation was performed under extracorporeal circulation with a pulsatile assisting device and with mild hypothermia. After the double ligation of the ductus arteriosus, right pulmonary artery was cut off from ascending aorta and was anastomosed with main pulmonary artery retroaortically. Postoperative clinical course was almost uneventful. Postoperative cardiac catherization showed mild increase of pulmonary arterial pressure, but pulmonary arteriogram showed no abnormal findings such as stenosis of the anastomotic site or compression by the aorta. Lung scan also showed that the distribution of pulmonary blood flow recovered in the early postoperative period. The patient is doing well tow years after the surgery.
Two cases of necrotizing enterocolitis (NEC) after protracted diarrhea were presented. A 27-day-old boy showed gangrenous ileum with multiple ulcer at laparotomy and was died of septic shock 4 days after the resection of ileum and fashioning of enterostomy. A 79-day-old boy, who developed ileal gangrene with multiple ulcer, underwent the end-to-end anastomosis with the resection of the ileal lesion. Four days after the surgery, he had a perforation near the anastomotic site but survived by the exteriorization of the lesion. Histological findings of the resected ileum from both cases were identical to those observed in premature infant with NEC except for the absence of pneumatosis intestinalis. In literatures, most of NEC developed in the older infants and children after a protracted period of diarrhea or gastroenteritis indicated poor prognosis. The early recognition and subsequent therapy should decrease the mortality of NEC in this age group as samely as in neonates.
A 2-year-old girl suffering from congenital mid-ureter stenosis due to an aberrant vessel underwent division of the vessel and Y-V plasty of the ureter. The vessel originated from the mesoureter suggests that the vessel had been a remnant of temporary renal artery which appeared during the cranial migration of the metanephros in fetal life. Histology of the stenotic portion of the ureter revealed almost normal muscular development, suggesting that the compression of the ureter by the vessel occurred comparatively late in fetal life. The patient required resection of the plastied ureter portion and end-to-end anastmosis because of suture insufficiency. The course after the reoperation was uneventful. In this paper, a rare case of congenital mid-ureter stenosis was reported and the cause of ureteral stenosis associated with an aberrant vessel was discussed.
Emergency balloon valvuloplasty was performed due to severe congenital aortic stenosis in a one month-old infant with severe congestive heart failure. In addition to peak pressure gradient between aorta and left ventricle decreasing from 155mmHg to 116mmHg, his general condition improved immediately. Two weeks after the balloon valvuloplasty, an open aortic commissurotomy was performed with a cardiopulmonary bypass. He recovered well and was discharged on the 22nd day after the operation with a further decrease in peak pressure gradient between LV and Ao of 15mmHg. Balloon dilatating angioplasty was performed on the stenosis of an arterial anastomosis in a one month-old infant following a Blalock-Park operation and was successful. Five days after balloon angioplasty, the intracardiac repair for ASD + VSD was completed under cardiopulmonary bypass. He also recovered well and was discharged on the 37th postoperative day. These results suggest that balloon-plasty is a very useful technique to improve an infant's condition from severe congestive heart failure and prepare for radical operation under cardiopulmonary bypass.
Familial occurrence of papillary thyroid carcinoma, one of the patients was in childhood, is extremely rare in Japan. Two cases with the carcinoma of the thyroid, occurring in a mother and her daughter with Graves' disease, were described. Case 1 : A 14-year-old girl, who had previously been treated for Graves' disease with propyl-thyouracil for five years, was admitted in March 1987 with left cervical tumor. Sub-total thyroidectomy was carried out, and histological findings showed papillary carcinoma of the thyroid and hyperthyroidism. Case 2 : (mother of Case l) A 22-year-old female with left cervical tumor underwent left hemithyroidectomy in July 1970. Histological diagnosis showed papillary carcinoma of the thyroid. We are inclined to suppose that in Case 1 Graves' disease and some familial factor, probably genetic, play an important role in carcinogenes of the developing thyroid gland. So, it is important to pay careful attention to a family history of thyroid carcinoma and concurrent car cinoma in treating Graves' disease.
A 1-year-old girl, diagnosed cat cry syndrome with the karyotype of 46, XX, 5p-, was admitted to our institute for constipation and abdominal distension. Hirschsprung's disease was diagnosed by the histochemical examination of the rectal mucosa, and the Z-shaped colorectal anastomosis using the GIA autosuture was carried out. Although an usual case, Hirschsprung's disease associated with cat cry syndrome has been reported in the occidental literature, this is the first case in Japan.
Paucity of intrahepatic bile duct with characteristic face, peripheral pulmonary stenosis, vertebral anomaly and physical and mental retardation has been named Alagille's syndrome. This disease is now thought to be a different from extrahepatic biliary atresia. Recently we experienced one cae o Alagille's syndrome with extrahepatic billiary atresia, so we reported here. A 44-day-old girl was admitted to our hospital because of heart murmur and jaundice. Her characteristic face, peripheral pulmonary stenosis and vertebral anomaly highly suspected Alagille's syndrome. But preoperative ERCP examination showed the abscence of in-trahepatic bile ducts and bilateral hepatic ducts, so ex-trahepatic biliary atresia could not be ruled out. In operation, bilateral hepatic ducts was replaced with granulation tissue and the finding of surgical cholangiography was same as that of ERCP. Hepatoenterostomy (SURUGA Ilnd procedure) was performed. Pathological investigation demonstrated the marked reduction of intralobular bile ducts in portal areas in biopsied liver and no ductular stuructures in granulation tissue. So the patient was diagnosed as Alagille's syndrome with ex-trahepatic biliary atresia. Her post-operative course had become worse and she died from massive intestinal bleeding with liver cirrhosis after 3 years.
A five-year-old boy with left pyriform sinus fistula was presented. He repeated the episodes of redness and swelling in the anterior neck. Diagnosis was made by esophagogram. We firstly performed fistelectomy, but the same symptoms recurred at the six months after surgery. At the time of the second operation, we inserted a catheter and poured indigocarmine into the fistula for easy detection. We however couldn't detect the fistula. Then we performed pharyngotomy and removed the fistula with caudal portion of pyriform sinus. Histological examinations showed a tubular structure lined by a pseudostratified ciliated epithelium. The ectopic thymus was found by the fistula. The thyroid tissue was noted to have interfollicular fibrosis and lymphocytic infiltration. Seventy four cases of pyriform sinus fistulae, ours included, were reviewed in Japanese literature. First sign of symptoms started about 7.5 years of age, on the average, with exceptions those of four adult cases. There is no sex difference in frequency. Sixty nine patients had the fistula on the left side of the pyriform sinus. Esophagogram was usualy of value for it's diagnosis. It is very important for a permanent cure to detect the fistula and to excise it in operation. Although 47 out of 74 patients had a fistulectomy in literature, 4 patients had a recurrence. We would like to recommend pharyngotomy and excision of pyriform sinus fistula for the case of re-current cases after fistulectomy and the undetectable fistula.
Retroperitoneal teratoma is a relatively rare tumor in infancy and childhood. This is the report of a giant re-troperitoneal teratoma associated with renovascular hypertension in a one-month-old female. She was admitted to our hospital for the evaluation of an abdominal tumor. Her abdominal tumor was diagnosed to be a retroperitoneal teratoma by CT, US and angiography. On the 10th day of her admission, systolic blood pressur rose suddenly to 164mmHg and her hypertension remained high till the 6th post operative day. Preoperative angiography revealed displacement of the right kidney in the caudal direction and stretching of the right renal artery. Inferior venacavography revealed stretching, compression and displacement of IVC ante riorly by the tumor. So we considered her hypertension as renovascular hypertension caused by mass effect of a giant retroperitonel teratoma. The tumor was resected completely without injury to IVC and renal vessels. Histology of the tumor was mature teratoma (without renal tissue) Renovascular hypertension is an extremely rare complication of retroperitoneal teratomas discribed in literatures. Angiography, especially inferior venacavography, should always be included into preperative evaluation modality program for safe and successful operation of such a giant retroperitoneal teratoma.
Congenital pyloric atresia is a rare disease, and only 38 cases have been reported in Japan. A 2-year-old girl was admitted to our hospital because of hypoproteinemia, anemia, and vomiting. She was diagnosed as blind loop syndrome with cholelithiasis due to the previous gastrojejunostomy which had been performed 2days after her birth for congenital pyloric atresia. Since atresia was fibrous-cord type, an end-to-end gastroduodenostomy after resection of the fibrous-cord with cholecystectomy was performed. After the operation, she has been asymptomatic. Congenital pyloric atreia is classified into 4types as follows; I. web or diaphragm, II. fibrous cord, III. segmental defects, IV. double membrane. It was suggested that an adequate operation should be selected for the congenital pyloric atresia according to its type of atresia in order to avoid the post-operative complications including blind loop syndrome and cholelithiasis.
A 12-year-old girl with twisted parovarian cyst who admitted to GCMC because of lower abdominal pain was reported. She presented a smoothly margined, elastic hard mass in lower abdomen. The diagnosis of twisted ovarial cyst was made preoperatively from US and CT findings. At laparotomy, a huge cyst existed in the parovarium with normal right ovary. The cyst was removed with gangrenous ipsilateral salpinx. Clinically, it is difficult to differenciate the parovarian cyst from ovarial ones. It seems likely to parovarian cyst that the cystic mass located superior uterine fundus by US and a ipsilateral ovary is demonstrated by US or CT.