Humoral and cellular immunity against bacterial infection is a very important defense mechanism in pediatric surgery. However, only few report about granulocyte function in a variety of pediatric surgical patients has been seen. In this paper, we established following assay systems to evaluate oxygen-dependent bactericidal activity of human granulocytes; (1) intracellular killing activity of Staphylococcus aureus, (2) superoxyide- generating activity, and (3) activity of myeloperoxydase. For ingestion of Staphyloccus aureus, 10% normal fresh serum in the medium has provided the maximal intracellular killing activity. Of the granulocyles in patients of biliary atresia (BA), the function of intracellular killing is depressed as compared with that of control group. The production of superoxide anion is also depressed, but the activity of myeloperoxidase is not depressed in granulocytes in BA group. In patients of BA, it is suggested that, the production of superoxide anion is depressed, and followed by depression of intracellular killing activity among the oxygen-dependent micro-bicidal system. Thus, these assay systems are useful for the assessment of granulocyte function in the field of pediatric surgery.
In relation to the etiology of the chronic constipation with simple megarectum, much attention have been paid to the distended rectosigmoid. In this study, the anorectal manometry was performed on the chronically constipated patients with and without megarectum, and it was found that the most of the constipated patients with simple megarectum showed an extremely high resting pressure of the anal canal. The authors propose "high anal pressure syndrome (HAPS)" for such constipated patients with simple megarectum. As to the surgical treatment, 6 patients with simple megarectum had a posterior internal sphincterotomy and the postoperative results were excellent or good in our series. In conclusion, we suppose that the etiology of chronic constipation with simple megarectum is mainly due to the hypertonic sphincter activity, and the internal sphincterotomy should be the first choice of operation for such constipated patients with megarectum.
Recent advances of nutritional care in pediatrics has led to the establishment of methods for nutritional assessment. Plasma rapid turnover proteins; such as prealbumin (PA), retinol binding protein (RBP) and transferrin (Tf) have been proved to be useful as a sensitive indicator for evaluating protein-calorie malnutvition. These plasma proteins of 87 healthy infants and 76 surgical patients (2mo. acd 2y.) were measured and compared with their anthropometric measurements; height/age and weight/height (W/H). Normal values for PA, RBP and Tf were 19.3±3.2, 3.2±0.6 and 338±74 mg/dl respectively. Of 66 preoperative infants, 27 cases with wasting (W/H < 90%) showed significantly low values of these plasma proteins. The usefullness of PA and RBP was demonstrated for assessing the nutritional state and postoperative protein metabolism in surgical infants.
Pathogenesis of congenital intestinal stenosis and atresia was investgated by the experi-mental fetal surgery. The segments of small and large intestines of 123 fetuses in 72 pregnant rabbits were dcvascularized by the partial exteriorization method at various stages of gestation. 73 fetuses (59.4%) were born alive one to eight post-operative days. Devascularized small intestine was created in 45 fetuses in which 22 perforations, 13 stenosis, 5 atresias and 5 other lesions were presented. On the other hand, 5 perforations, 13 stenosis, 7 atresias and 3 other lesions were observed in 28 alive delivered fetuses with devascularized large bowel. Pathohistological findings such as hemorrhagic infarct, regeneration of the mucosal epi-thelium and replacement by the granulation tissue were considered as the process to develop stenosis and atresia in the devascularized small and large bowels. However, the more defi-nitive stenosis and atresia that seemed to be due to the mucosal changes which might have occured in the earlier period and the muscular or serosal changes in the later period were observed in the devascularized large bowels. Electron microscopical study of the involved lesion showed regeneration, absorption and division of the mitochondria in the mucosal epithelial cells near the infarct which showed metabolic activities within these cells. Furthermore, swelling of the Auerbach plexus with the large nucleus of the neuron and increase of the glia cells near the infarct seemed to be re-ferable to disturbance of peristalsis of the devascularized segment. In conclusion, accidental interruption of the blood flow to the intestinal segment in fetus may be the cause of congenital stenosis or atresia of the small and large bowels.
The catecholamine concentration and the amount of α-adrenergic receptor in aganglionic colon were measured and responses to electrical field stimulation were investigated in order to elucidate the pathophysiology of the aganglionic segment in Hirschsprung's disease. The catecholamine concentration was measured by high performance liquid chromato-graphy with electrochemical detection, which was found to be more accurate than histochemical or fluorometric method. Norepinephrine (NE) concentration in the aganglionic colon was appoximately two times greater than in the normal colon. NE in the ganglionic colon was 300.2±120.3ng/g(n=7) in contrast to 623.3±300.Ong/g (n=7) in the aganglionic segment of Hirschsprung's disease. On the other hand, no significant difference was found in the amount of other two catecholamines; epinephrine and dopamine. In extensive aganglionosis, NE concentration differed for each part of the colon; 295.1±91.6ng/g (n=4) in the ascending, 326.8±122.9ng/g (n=4) in the transverse, 489.8±221.8ng/g (n=4) in the descending and 693.4±355.Ong/g (n=4) in the recto-sigmoid colon. α-adrenergic receptor was assayed by measuring the specific binding activity of 3^H-dihydro-α-ergocryptine. The ganglionic colon contained 196f mol/mg prot. receptor sites and the aganglionic segment had 207f mol/mg prot.. There was no significant difference between the two segments of the colon in Hirschsprung's disease. Response to electrical field stimulation was also investigated to evaluate the existence of neuro-muscular junction in the aganglionic colon. The aganglionic segment showed only cholinergic contraction, whereas, non-adrenergic relaxation was dominant in the normal colon. From the results of the studies, it is confirmed that in Hirschsprung's disease, cholinergic innervation and the absence of the non-adrenergic inhibitory nerve cause the contractile state, and even NE concentration is much higher than normal colon.
Histopathological studies of the liver and the bile duct remnants were carried out in 13 patients who had undergone hepatic portoenteroslomy for "non-correctable" type of biliary atresia. The patients were divided into two groups according to their postoperative course; Group A consisted of 10 patients who showed good excretion of bile and complete disappearance of jaundice postoperatively. Group B included 3 patients who showed good excretion of bile and complete disappearance of jaundice but prolonged liver dysfunction (n=2), or in whom jaundice persisted postoperatively (n=l). There was no remarkable difference of liver histology between these 2 groups, but de-generative changes of the bile duct epithelium in Group B. Histopathological examinations of the fibrous tissue transected at the porta hepatis disclosed that there were numerous minute bile ducts in all the specimens studied. Degenerative changes of the bile duct epithelium were, however, found in the specimens take from the patients who belonged to Group B. Three-dimensional reconstruction of the intraheptic bile ducts was carried out in 3 patients with biliary atresia; one belonged to Group A and other two patients belonging to Group B. Proliferation of intrahepatic bile ducts were noted but the main interlobular bile ducts and their branches were well preserved in the patient belonging to Group A, whereas there were obstruction and irregularity of the main interlobular bile ducts and their branches in the patients who belonged to Group B. The results of the present studies suggested that morphologic as well as functional changes of the inlrahepatic bile ducts are important to the prognosis of the patients with biliary atresia.
Management of testicular tumors in children has been variable. There is no general agreement regarding further optimal therapy. Embryonal carcinoma is the most common in testicular tumors of the childhood, and it is usually managed with high inguinal orchiectomy, retroperitoneal lymphadenectomy, radiotherapy and chemotherapy. Various recommendation for further therapy with either retroperitoneal lymphadenectomy, irradiation to retroperitoneal lymph nodes, chemotherapy, or a combination of these modalities have been made in many studies. Survival rate increases if many therapies are combined. But the growth of most embryonal carcinoma of testes in children is localized at time of diagnosis. Retroperitoneal dissection is a risky and incomplete procedure for small children. Prophylactic irradiation is hazardous in the growing child, while chemotherapy seems to be useful initial management. During the last 17 years, 11 cases of embryonal carcinoma of the testis were experienced. Ten of 11 cases are alive. Nine cases were managed with orchiectomy and chemotherapy. The others were managed with combination therapy. Successful chemotherapy for a retro-peritoneal metastasis Stage III was presented, which was treated with Adriamycin, Vincristine and Cyclophosphamide, Based on oure experiences 11 cases and review of the literature, we advocate high inguinal orchiectomy and chemotherapy if metastasis is not manifested.
In the past 9 years, we treated 39 patients of various urological complications caused b the radical operations of imperforate anus. While associated anomaly of the genitourinary tracts and vertebral column are more or less evenly distributed between both sexes and between high and low type rectal anomalies, the complications of pelvic surgery were 5 to 7 times more common in the male and in high type. Analysis of the patietns showed the urological complications to fall into 3 main groups, urethral fistula and diverticulum, urethral stenosis, and neurogenic dysfunction. 1) Urethral fistula was detected in 3 children, and urethral diverticulum in 10 children. The problems arised from recanalization of rectourethral fistula or injury of the urethra other than the rectal fistula. Perineal extirpation was performed in 6, and one large diverticulum, which exceeded the bladder neck, was operated on through abdomino-peri-neal approach. 2) Among 15 children with organic urethral stenosis, 3 had the history of urethral resection during the radical operation. For these strictures, optic internal urethrotomy was the first choice of operations. One child of severe stricture was treated by Johanson procedure. Neurogenic dysfunction is likely to accompany in 4 children who showed no imporvement after the urological operations. 3) On cystometric examinations, 70% of the patients showed abnormal intravesical pres-sure. About half of the high type anomaly revealed hypotonic pattern, which implies detrusor hypotonia from pelvic nerve injury. From EMG test of external urethral sphincter, 45% exhibited silent or low amplitude activity, which can result from pudendal nerve injury or organization of the sphincter through pullthrough procedure. Some typical cases were presented.
An extremely rare case of rectocloacal type of anorectal malformation was presented. The urinary bladder and urogenital sinus have formed a huge cloacal cavity and the border between these two structures was not able to identify. A very hypoplastic double vagina opened into this huge cloacal cavity. The right ureter which entered the vesical diverticulum was hydroureteric due to the stricture at the uretero-diverticular junction. The left double pyeloureteric system demonstrated vesicoureteric reflux. The rectocloacal fistula joined to the distal narrow portion of the cloacal sinus. As for treatment, firstly, sacroabdominoperineal rectal pull through was carried out and secondarily the bilateral reimplantation of ureter (Politano-Leadbetter procedure) with excision of bladder diverticula and tapering of right megaureter were done. Now patient is doing quite well on both, micturition and defecation two years after surgery. The recontrutive surgery of the vagina is the future problem.
The successful staged total correction of a Type-A interrupted aortic arch with VSD and PDA is described. 5 years old girl, who had undergone an operation of bilateral pulmonary artery banding in 57 days after birth, was doing well postoperatively but developed mild CHF and cyanosis gradually. Total correction was performed under deep hypothermic circulatory arrest with combination of bilateral pulmonary artery debanding, aortic arch reconstraction and VSD patch closure. Operation was performed with direct anastomosis between assending aorta and tube formed from anterior wall of the main pulmonay artery connected with PDA. Cardiac catheterization and angiography were performed 6 months later, and demonstrated a satisfactory reconstraction of aortic arch and recovering of right ventricular overload state. Surgical management of interrupted aortic arch and method of aortic arch reconstraction are discussed.
A 5-year-old boy was admitted with several days history of severe abdominal pain and vomiting. He showed tenderness and muscle guarding on lower abdomen, and pelvic mass was palpable on rectal digital examination. Acute appendicitis was suspected and emergent operation was performed. At laparotomy, we found pelvic tumor composed of the ileum, rectal and bladder wall. As mesenteric nodes showed marked swelling, we suspected malignant tumor. Because pathologic diagnosis on frozen sections was inflammation, we excised the perforated ileum only. However, final pathologic diagnosis was non-Hodgikin's malignant lymphoma, large cell type, hence radiation and chemotherapy including VCR, Prednisolone, L-asparaginase, 6-MP, MTX were added. Now, seven months after the operation, he is well without evidence of recurrent disease. Between January 1, 1967 and Decembar 31, 1982, 41 cases of malignant lymphoma were admitted to the National Children's Hospital, four cases (10%) of which originated from the ileum. This case was operated on suspicion of acute appendicitis, and other three cases were operated for intussusception secondary to the disease. When the child is reffered for abdominal pain, especially in the case of intussusception seen in a child elder than 2 years old, malignant lymphoma should be taken into account of, although those of the ileum are very rare.
We experienced a case of giant cystic meconium peritonitis (GCMP) which is a rare and severe form of meconium peritonitis, characterized by giant pseudocyst formation. This female patient was delivered with a massively distended abdomen and abnormal vaginal discharge of meconium. At emergency laparotomy, a giant meconium pseudocyst that walled off most of the intestines was found, but perforation site was not identified. She underwent external drainage of the cyst, and neither decortication or enterostomy performed. Rapid reduction of the size of cyst was observed under total parenteral nutrition. An ileal perforation proximal to an ileal atresia was noted at second operation performed at 1 mo of age, and an ileostomy to exteriorize the perforation was created without any difficulties. She underwent uneventful repair of her ileal atresia and closure of her ileostomy at 3.5 mo of age. Bowel function and growth are normal at age 1 yr. Our case suggests the drainage procedure without dangerous decortication for the GCMP can offer the good opportunity for survival. In addition, we reviewed 16 cases of GCMP from the Japanese and English literatures, and discussed its clinical features and surgical treatment.
A 5-year-old boy was admitted to our hospital because of an abdominal tumor at the right costal margin. Noninvasive examination confirmed the diagnosis of hydrops of the gallbladder using C-T Scanning, Echogram and X ray. The boy underwent the operation. The gallbladder measured 10.5X3X3cm in greatest dimension. About 1cm from the fundus of the gallbladder was a shallow external groove. The lumen was divided into two compartments by a complete transverse septum at the site of the groove. The proximal portion contained bile and the distal portion contained trans-parent serous fluid. Cholecystectomy was done. Histologically, the transverse septum was consisted of the smooth muscle and the epithe-lium of the gallbladder.
Congenital stenosis of the trachea (CST) is one of the most unusual anomalies of the respiratory tract. Recentry we experienced a case of CST associated with multiple malfor-mations. Among the reported cases up to the present, this is the first case who has associated with gastrointestinal malformations such as Hirschsprung's disease and malrotation. The patient was surgically treated for these alimentary tract anomalies except that of trachea by the 20th day after birth. However her postoperative course became worse and she died of respiratory distress on the 5th postoperative day. An autopsy reveald a full-length tracheal stenosis due to encircled cartilagenous ring.