For the purpose of studying the embryology and genetics of imperforate anus, we attempted to produce this disease in a large number of swine. In the anatomical evaluation and the process of treating and raising the swines, we had various experiences on this disease. In the studies of 31 swine materials, we found a similarity to the human anomaly. In male swines there was no external perineal fistula. There were one recto-vesical fistula with severe reno-ureteral anomaly and 19 recto-urethral fistulas. In female swines, there were 3 recto-vaginal and 5 recto-vestibular fistulas. We operated on neonatal piglets, employing various methods such as colostomy, perineal and sacro-perineal operations. We finally succeeded in establishing the proper operative method. A closure of recto-urethral fistula was essential for male. Posterior saggittal approach by Pena was also employed for treatment of recto-cloacal fistula in female. In this way, swine is a suitable therapeutic model for perineal and sacro-perineal operations, including the closure of recto-urethral fistula. But the swine has no muscles identical to the pubo-rectalis and the pelvic diaphragm which are seen in the human. Therefore, it is not possible to use the swine as the model for evaluation of fecal continence. Post-operative anal stenosis occured almost always without bouginage and prolapse of the mucosa (or anus) occured very often, if a loose anus was produced. The proper operative method and management, preventing these complications were investigated.
Effect of pneumococcal capsular polysaccharide vaccine on pneumococcal bacteremia was studied in three-week-old Sprague-Dawley rats. Seventy two animals were divided into the following six equal groups. I-sham operation plus saline administration II-sham operation plus type 6 pneumococcal vaccine III-sham operation plus killed type 6 pneumococci IV-splenectomy plus saline administration V-splenectomy plus type 6 pneumococcal vaccine VI-splenectomy plus killed type 6 pneumococci The intravascular clearance of type 6 Streptococcus pneumoniae and the number of pneumococci in the liver and spleen were compared among the six groups. The serum opsonic activity of all animals after vaccination was also measured before bacterial challenge. Bacteremia was induced by intravascular injection of 10^5 type 6 S. pneumoniae. Splenectomy produced significant impairment of intravascular clearance of pneumococci compared to clearance in sham-operated animals. Administration of type 6 pneumococcal vaccine and killed type 6 pneumococci significantly improved pneumococcal clearance compared to the non-vaccinated groups in both sham-operated and splenectomized rats. Significantly less number of pneumococci were cultured in the liver of vaccinated rats than in the liver of controls. The serum opsonic activity in vaccinated rats was significantly higher than in non-vaccinated animals, and this higher opsonic activity seemed to contribute to improvement of pneumococcal clearance by vaccination. These results suggest that pneumococcal capsular polysaccharide vaccine could provide protection against pneumococcal sepsis in splenectomized children.
The mortality of open heart surgery for infants under the age of 12 months remained higher than it in the older-age group. The weakness and premature function of organs were considered as one of the reasons. If so, the surgical technique should be chosen very carefully for such infants. Then, we reviewed surgical techniques in 116 open heart cases under the age of 12 months, operated upon from Jan. 1977 to Dec. 1982, in The Heart Institute Japan, Tokyo Women's Medical College. A various techniques, such as profound hypothermia and circulatory arrest induced with combined surface and core cooling, moderate hypothemia with bypass, with or without aortic cross-clamping, and staged operation were discussed. The results suggested that various techniques suitable for different procedures should be chosen for improvement of surgical mortality in sick infants. Consequently, the most recent protocols for infant open heart surgery were summarized as the followings: (1) In a case of VSD with PH, closure is made under moderate hypothermia induced with bypass, and aortic cross-clamping with Young solution.(2) In a case of TAPVC, intracardiac repair is performed under moderate hypothermia induced with bypass, and no aortic cross clamping. (3) In a case of d-TGA's, atrial switch procedure is made under profound hypothermia induced witch combined surface and core cooling and circulatory arrest. (4) In a case of CoA, or Interrupted aortic arch, two-staged operations were preferable with lower mortality. In the first stage, reconstruction of aortic root and pulmonary banding through left thoracotomy, are scheduled, and closure of VSD around the age of two years, in the second stage.
Total parenteral nutrition (TPN) which has recently made a marked progress is now an indispensable supporting technique in pediatric surgery. However, there are some remaining problems, in TPN especially in pediatric patients such as the route of catheterization into the "central vein", the proper dose of amino acid and fat, and the proper amount of fat emulsion supply in intermittent TPN for prevention of hypoglycemia. For those problems, this study was carried out in infants and children in whom TPN has been undertaken for various reasons during a period of 5 years (1978-1983). 1. By our experience with 200 pediatric patients of TPN using a subclavian vein puncture technique, the safety and efficacy of this technique was confirmed for a route of inserting catheter of TPN. 2. To determine a proper amount of amino acid, aminograms in plasm and levels of blood ammonia were examined in the patients receiving 1.5-3.5 g/kg/day of amino acid as a nutriment. As a result, it was assumed to be dangerous for overdosis if children less than 5 years of age are given over 2.5 g/kg/day of conventional amino acid solutions. 3. In a comparative studies of fatgrams and lipoproteinograms in plasm in patients who were given 1-3 g/kg/day of fat and control patients, it was demonstrated that the most adequate intake of fat is about 2 g/kg/day in young children. 4. With an adition of fat emulson of 1 g/kg/day to TPN solution, a decrease of blood glucose level and insulin in plasm was not observed after an abrupt interruption of TPN. These results indicate that the intermittent TPN is able to carry out with safe even in infants if fat emulsion is used together.
Hepatobiliary scintigraphy with <99m>^Tc-E-HIDA was carried out in 13 patients who had undergone hepatic portoenterostomy with original Roux-en-Y for "non-correctable" type of congenital biliary atresia. In all patients, there were active bile flow and disappearance of jaundice after surgery. Elimination of <99m>^Tc-E-HIDA from the liver was slow (below 2% / min.) during the first 3 postoperative months despite of adequate bile flow. The elimination rate of the radioactive agent from the liver at the third postoperative month ranged form 2 to 3% / min. There was a delay in the elimination of the radioactive agent from the liver in those who had surgery after 70 days of age or in those who had suffered from repeated ascending cholangitis. The elimination rate of the radioactive agent from the liver after the 9th postoperative month was almost the same as those who had undergone resection of the cyst and hepatico-jejunostomy for idiopathic dilatation of the extrahepatic bile ducts (4.49 ± 0.47% / min.).
The purpose of this report is to clarify the curing process and pathophysiology of idiopathic gastric volvulus in neonates.Eighteen neonates with gastric volvulus were diagnosed by UGI series. There were 11 males and 7 females. Thirteen patients were followed by X-ray study at every 3 months. Seven patients were radiologically classified as the mild and 6 as the severe group. In the mild group, vomiting disappeared in all patients within 3 months. In the severe group, vomiting disappered in 4 within 3 months and in 2 patients within 5 months. Six patients of the mild group were cured completely within 6 months radiologically. On the other hand, 3 patients of the severe group healed within 6 months, and one and two patients within 9 and 12 months respectively. This suggests that more time is needed for complete cure of gastric volvulus even after disappearance of vomiting. At diagnosis, the gastric antrum has been shown to be higher and the fundus lower than usual. The most remarkable change in healing process was gradual descent of the elevated antrum to the normal position. The cardia and pylorus did not change the position throughout the process in most patients. In healing process the axis of the volvulus changed from mesenteroaxial to organoaxial and thereafter became normal. It means that both orientations merely represent the different stage of healing process of this disease and the axis of the stomach with volvulus is fundamentally combined.
We have anastomosed various duct-structures (vessels, urogenital and digestive tract etc.) under the microscope clinically and experimentally during the period from July 1981 through October 1983. Clinically, the central splenorenal shunt was performed by utilizing 8 - 0 monofilament nylon and Zeiss OPM-microscope in 5-yr-old female, because the diameter of splenic vein was 3.5 mm. There were 2 cases with the dismembered pyeloplasty for congenital hydroneph-rosis and 12 cases with the urethroplasty for hypospadias. We have usually used the synthetic absorbable suture material (6 - 0 or 8 - 0 Polyglycolic Acid Suture of special make) for these procedures. The dissection procedure has been carried out under the KONAN-microseope in 10 cases with lymphangioma, haemangioma, congenital biliary atresia, bronchiogenic and thyroglossal cyst. Experimentally, the infuence of the microsurgical technique and the suture material was studied. The interrupted suture-technique was more desirable than the contineous suturertech-nique, because suture sites were angiographically strictured in the latter in microvascular anas-tomosis of rats. The synthetic absorbable suture material(Polyglycolic Acid Suture) seems to be superior to the nonabsorbable suture material for the anastomosis of digestive and urogenital tract. The microsurgical technique may be more applied to the pediatric surgery, because the duct-structures are very small and the tissues are delicate in infants.
This paper describes a 6-month-old infant with aberrant systemic artery to pulmonary vein fistula without pulmonay sequestration. The patient had history of recurrent upper respiratory infection and persistent stridor, severe pulmonary congestion and dextroversion of the enlarged cardiac silhouette on the chest roentgenogram. Severe pulmonary hypertension without left-to-right shunt in the pulmonary artery was demonstrated by the cardiac catheterization and the aortogram revealed the two aberrant arteries originating from the descending aorta just below the diaphragm, entering the posterior basal segment of the right lung, branching into many vascular channels and draining promptly into the right pulmonary vein. The bronchogram showed a normal branching of the right bronchi. Ligation and division of the aberrant arteries were carried out successfully and the patient is asymptomatic and has no signs of recurrence 3 years postoperativelly. Only ten similar cases were reported so far in the literatures but none of the cases demonstrated pulmonary hypertension. Our case indicates the possibility of developing pulmonary hypertension in those cases reported and the importance of early diagnosis and surgical treatment before any irreversible pulmonary vascular change might occure.
Angiomatoid Malignant Fibrous Histiocytoma (AMFH) is a newly described fibrohistiocytic sarcoma which occures primarily in the extremities of children and young adults. It manifests as a nodular subcutaneous mass and clinically has often been mistaken for a hematorna or a hemangioma. A case we recently encountered was an one year and seven months old boy who had a painless subcutaneous nodular tumor in his mid back. It measured 25×18×15 mm. The cut surface of the excised tumor was red tan coulored and had a cyst filled with old blood. The histological findings were compatible to those of AMFH including the three characteristic features described by Enzinger. The patient has been free from any sign of recurrence or matastasis 1 year and 5 months after surgery without any other adjuvant chemotherapy or radiotherapy.
The patient was delivered by cesarean section at 33 weeks of gestation, because of rupture of the amniotic sac in 18th week and subsequent sepsis. He was born as a triplet with the birth weight of l,500g. Apgar score immediately after the delivery was eight. Abdominal distention and billious vomiting started from the first day of life and scout film of the abdomen showed a large air bubble in the right side. Distention of the abdomen increased and patient's condition deteriorated on the third day of life and pneumoperitoneum was found. Emergency laparotomy disclosed peritonitis with perforated cystic Meckel's diverticulum (7 cm in diameter) located at 5cm from ileocecal valve. The diverticulum was removed and found to contain no ectopic tissue whatsoever. Dilated portion of the diverticulum was lined by one layer of cuboidal epithelial cells. Proper muscle layer around the perforation was absent. Marked congestion, hemorrhage and infiltration of the inflammatory cells were found in the submucosal as well as subserosal layers. The patient's postoperative course was uneventful except transient hyper bilirubinemia. Fourteen cases of perforated Meckel's diverticulum in newborn infants were summerized from Japanene literature and circulatory disturbance in the wall of the diverticulum by ballooning and / or torsion of the neck of diverticulum was suggested as a pathogenesis of the perforation in newborn period.
Achalasia of the esophagus, although commonly encountered in adults, is rare in infants and children. Esophageal achalasia in a 4 year old female with Down's syndrome is reported and pathophysiology of the lower esophagus in this patient is discussed in this paper. The patient is a 4 year old female. She was noted to vomit since the age of 7-months, and regurgitation occured after every meal. Dilatation and marked narrowing at the gastro-esophageal junction was found by an upper gastro-intestinal series. Esophageal manometric study showed that lower esophageal sphincter (LES) pressure was elevated (66cmH_2O), and LES relaxation corresponding to swallowing was not found. After a modified esophagomyectomy had been performed, partial fundoplication was added for preventation of gastro-esophageal reflux. After esophagomyectomy, LES pressure has significantly decreased. Post-operative fluoroscopic examination revealed good passage of barium. No ganglion cell was found in the biopsied specimen.
A six-month-old boy with a tail attached immediately to the right of the anus was reported. The tail was 3.5cm long and its diameter was 1.5cm at the base. A hemangioma was noted at its base. Neither bone nor cartilage could be appreciated in it. It exhibited no spontaneous motion. At surgery, the tail was found to have a central muscular core which connected to the external anal sphincters. Histologic examinations demonstrated skin, adipose tissue, nerve fiber and a small amounts of skeletal muscle.
A 9-year-old boy was admitted to our department with a chief complaint of dyspnea. Emergency thoracotomy revealed a huge hard mass which arised from the right 9th intercostal muscle and occupied the almost all of the right hemithorax. Total resection of the tumor with right 8th, 9th and 10th ribs was performed. 14 cases of rhabdomyosarcoma arising from the chest wall were collected from the Japanese literature. Among them, no long-survivors were found except in the patients with total re-section.
A case of newborn female infant with omphalocele and Prune belly Syndrome was reported. She was born at full term: the delivery was spontaneous from a cephalic presentation. Her birth weight was 2,820 g. There was no family history of anomalies. In the lower abdominal region, there were two large bulging areas lacking covering skin. The upper one was an omphalocele measuring 5cm in diameter. The another bulging was an exstrophied bladder. One stage repair of the abdominal wall and the bladder could be performed. After the operation, we noticed that the abdominal wall was flaccid and thin. CT scaner of the abdomen showed absence of musculture of the abdominal wall and left hydronephrosis. Drip Infusion Pyelography showed left non-visualizing kidney. The post operative course has been satisfactory, but carefull follow up of renal function and grouth is mandatory.