Localization of SP and VIP in normal, transitional and aganglionic colorectal segments of Hirschsprung's disease was examined by immunohistochemical electronmicroscopy. Following results were obtained. 1. SP- and VIP-immunoreactive nerve cells and nerve endings were observed within the intramural plexuses of the normal colonic segment. Immunoreactive nerve fibers were also oberved in the muscular layers, especially in the circular layer, and VIP-immunoreactive nerve endings showed neuromuscular junction-like structures in the circular layer. 2. In the transitional segment SP-immunoreactive nerve cells and nerve fibers were found to disappear within the muscular layers, but VIP-immunoreactive nerve fibers were still observed there. 3. In the narrow segment neither SP・VIP-immunoreactive nerve cells nor fibers were observed in the muscular layers. Very few nerve fibers were found in the submucosal or subserosal layers and were considered to be originating from extraneous nerves. 4. In the lower recto-sigmoid narrow segment many SP・VIP-immunoreactive nerve fibers were observed in the muscular layers, but no neuromuscular connection-like structure was found there. These results suggested that SP- and VIP-mediated intrinsic neurons have some participations in the normal colonic movements and their absence in the narrow segment was supposed to contribute to causation of aperistalsis and narrowing there. In the lower recto-sigmoid aganglionic segment many extraneous SP・VIP-immunoreactive nerve fibers were observed, but no apparent neuromuscular connection-like structure was found there.
It has been reported that humoral and cellular immunity in patients with biliary atresia (BA) was depressed. Ninety seven cases with BA were treated at the 1st Department of Surgery, Nagoya City University Medical School from 1970 to 1984. Most of them have suffered from severe infectious complications such as ascending cholangitis after Kasai's operation. We examined also at random the changes of the humoral and cellular immune reactions regarding plasma immunoglobulin (IgG, M, A), plasma components (C_3, C_4), white blood cell count and nitroblue tetrazolium test (NBT test) of neutrophils in 44 cases with BA before and after operation. We concluded as follows: 1) The post-operative immunoreactions in BA with good bile excretion, which are currently almost free from signs and symptoms, returned to the control levels at about two years, but those in BA with poor bile excretion or with recurrent ascending cholangitis despite bile drainage were suppressive in all their clinical courses. 2) Incidence of ascending cholangitis has decreased in about two years after Kasai's operation.
Intrahepatic cholestasis is known as uncontrollable and sometimes fatal complication often seen infants receiving TPN from neonatal period. Though many etilogical concepts on this disorder have been proposed, the details of its mechanism remain obscure. Seven infants receiving TPN for 17 days to 2 yrs 4 mos from neonatal period were subjected to the present study and pathophysiological investigation of the cholestasis was carried out by immunoelectron-microscopic localization of IgA, SC (markers derived from biliary epithelia) and C_3, C_4 (markers derived from hepatic cells) in the liver. Consequently, cholestasis containing IgA and SC began from at the level of bile canaliculi, which suggested that initial site of biliary obstruction located at the canals of Hering or their a little distal bile ductules. Then, as hepatic cells and biliary epithelia received more damage, cholestasis developed proximally to the hepatic cells and distally to the interlobular bile ducts. Every cholestasis contained IgA, SC as well as C_3, C_4. Positive staining of C_3, C_4 in the bile suggested that the complements were transferred from hepatic cells into bile canaliculi. In conclusion, the present study using IgA, SC and C_3, C_4 as markers derived from biliary epithelia and hepatic cells respectively, revealed more clearly the mechanism and process of intrahepatic cholestatic disease associated with TPN. Immunological analysis of the results obtained from this study should be achieved in the future.
Gastroesophageal Reflux (GER) has been incriminated as a cause of respiratory symptoms and Sudden Infant Death Syndrome (SIDS) in newborns and infants. Upper G.I. series, esophageal manometric studys, and distal esophageal pH monitoring have been used to assess the significance of GER. However, these methods gave only slight informations regarding the diagnosis of GER to the upper portion of the esophagus. In the present investigation, upper and distal esophageal pH monitoring was done in newborns and infants. Nienteen patients with asymptomatic GER, and ten patients GER with respiratory symptoms or near miss SIDS were evaluated in this study. 1) From the results of two-points pH monitoring, GER was assumed to be present in cases with pH score above 8 points in the distal esophagus and 6 points in the upper esophagus. 2) Distal esophageal pH monitoring is one of the most useful methods in diagnosis of GER. 3) GER to the upper esophagus which causes respiratory symptoms and SIDS was presumed by distal esophageal pH monitoring to some extent. Direct upper esophageal pH monitoring, however, was more valuable to assess the GER up to the upper esophagus. 4) Seventeen patients with GER alone were treated concervatively by general management. And seven patients with respirator respiratory symptoms or near miss SIDS were treated conservatively also, but with intravenous hyperalimentation (IVH). All of them had good clinical results and GER disappeared after these therapy.
The HRP-labeled antibody method was used for intrahepatic localization of complements (C_3, C_4, C_<1q>) in 8 infants with congenital biliary atresia (CBA) and 4 with neonatal hepatitis (NH), as well as in 7 with cholestatic disease associated with total parenteral nutrition (TPN). Normal hepatic localization of C_3 and C_4 was limited to hepatic cells, sinusoid, Disses space, vascular endothelium, Glisson's connective tissue and stellate Kupffer cell. The localization of C_<1q> was limited to hepatic cells. On the other hand, in the cholestatic disease, positive staining of the complements was also observed in the congested bile. Moreover, in CBA, the interlobular and ductular epithelial cells had positive staining of C_3, C_4, but not in NH or TPN. The present study suggests that the C_3, C_4, and C_<1q> transfer from hepatic cell into bile canaliculus and that biliary epithelial cell of CBA may possess ectopic function to produce the complements or may be the site of immunoreaction involving C_3 and C_4.
Thirty-six cases teratoma in children were treated in our hospital of which 26 cases were of benign. The problems on the diagnosis and treatment of these benign teratomas according to primary site were evaluated. 1. All of the 3 cases occured in the neck were of benign. One of them was a huge teratoma noticed since birth, grew rapidly and needed the urgent operation. 2. Three mediastinal teratomas were 12, 12 and 13-year-old and all the cases were benign. CT was most useful to make a diagnosis of mediastinal teratoma. 3. Six out of seven cases of retroperitoneal teratoma were benign. The youngest case was 3-month-old and it was found to be fetus in fetus after operation. Retroperitoneal mass developed in adrenal region needed to be differentiated from neuroblastoma by US, CT and tumor markers. 4. Four cases of benign ovarian teratoma had abdominal pain and abdominal mass. Calcification on the x-ray films and mixed pattern of cystic, solid mass and calcification on the US or CT films were very important findings of teratomas. 5. Nine cases of 13 sacrococcygeal teratomas were benign. There was no case beyond 2-year-old. It was necessary to measure AFP for differentiation benign type from malignant type.
Myocardial protection and tissue metabolism were studied during cardiopulmonary bypass and after open heart surgery in 20 infants with cyanosis and 33 infants without cyanosis (VSD). The effects of core cooling, topical cooling and anoxic arrest on plasma catecholamine levels on circulatory variables were examined. The biological effects of high plasma catecholamine levels under the conditions of hypothermia were difficult to assess. In both groups, insulin (IRI) and C-peptide (CPR) levels decreased during total cardiopulmonary bypass, and myoglobin and CPK-MB levels, on the other hand, increased immediately after declamping of the aorta.
Sixteen infants with coarctation of the aorta (CoA) and ten infants with interruption of the aortic arch (IAA) under 12 months of age underwent urgent repair during the recent 10 year-period untill December 1985. Nine infants (34.6%) were in newborn period at operation, 9 (34.6%) under 3 months, 4 (15.4%) under 6 months and 4 (15.4%) under 12 months of age. Subclavian fllap method was performed in 7 (1 death), end-to-end anastomosis in 6 (2) and Blalock-Park shunt in 2 (1) infants with coarctation of the aorta. In seven infants associated with CoA and VSD+PH, one-staged correction of CoA and VSD was performed in one (1) and staged corection in 6 (1). In staged operation 4 (1) had undergone VSD closure on urgency and 2 had on elective basis, followed by CoA repair. Van Praagh operation was performed in 7 (3 early deaths, 1 late death) and Blalock-Park shunt in 3 (3) infants with interruption of the aortic arch. Infants admitted with CoA and IAA in congestive heart failure should have prompt surgical intervention after initial medical treatment and complete evaluation by echocardiography and radioisotope injection method. Subclavian flap or end-to-end anastomosis is considerd the procedure of choice in infants with CoA. Indication for Van Praagh operation should be restricted to the cases with hypoplasia of the ascending aorta or aortic arch.
Two cases of the entire colon aganglionosis were presented; one was involved with the aganglionosis bellow the 25 cm from the terminal ileum and an ileostomy was made at 55 cm from the terminal ileum. The other, involved bellow the cecum and an ileostomy was made at 25cm from the terminal ileum. A low residue diet (LRD), clinimeal^<[○!R]> was substituted for the postoperative oral nutrition without using intravenous hyperalimentation. Daily amount of stool excreted from the ileostomy stoma, ranged from 50〜100 g, and their body weight gain was sufficient. They were undergone Duhamel-Ikeda's operation with Martin's procedure at the age of 5 and 6 months respectively. The postoperative courses were uneventful with the low residue diet. The laboratory dada through their clinical courses, revealed relatively low levels of serum total cholesterol and transient high alkaline phosphatase levels. Althogh the analysis of fatty acid in plasma showed normal in one case, the analysis of amino acid in plasma showed mild elevation of valine, proline, and glycin in both cases. In conclusion, clinimeal is considered to be very effective for the postoperative nutritional management in the patient with entire colon aganglionosis.
This article reviews our clinical experiences of fetal thoraco-abdominal abnormalities detected before birth. Eighteen surgically significant anatomic abnormalities have been diagnosed antenatally at our university hospital and affiliated institutions over the past five and a half years. Antenatal diagnosis by ultrasonograghic examination has remarkably improved perinatal management of pregnant mothers carrying infants with significant surgical anomalies. Elective cesarean section or transference of mothers to regional centers which facilitate neonatal surgery can be properly planned for in advance. It should be emphasized that in congenital abnormalities, such as omphalocele, esophageal atresia, and diaphragmatic hernia, prenatal detection followed by postnatal operative correction would improve their survival.
To evaluate nephrotoxicity of cisplatin (CDDP) in the treatment schedule in Kyushu area for children with advanced neuroblastoma, renal function during CDDP treatment, histologica' change and platinum accumulation of kidney were studied in 12 children with tumor (Neuroblastoma; 10 cases, other tumors; 2 cases, age; 1y6mo.〜14y, total dose: of cisplatin 100 to 1,000mg/m^2). Renal function was evaluated by BUN, serum creatinine, creatinine clearance, and urinary N-acetyl-β-D-glucosaminidase (NAG). BUN and creatinine did not change during the treatment in all cases. Creatinine clearance transiently decreased in some cases ( < 60ml/min), but it improved in about 2 weeks after the treatment. Urinary NAG transiently increased in the first course of CDDP treatment in the uninephrectomized cases, but they were within normal limit over 2nd courses. Even in the non-nephrectomized paint administrated 1,000 mg/m^2 CDDP in total dose, renal function was normal. Platinum (Pt.) accumulation in kidney was examined in 9 patients (total dose 100〜600 mg/m^2, Pt. level in kidney: 0.13〜1.94μg/g). In the non-nephrectomized cases, renal histological changes had correlation with Pt. level in kidney. One the other hand, in uninephrectomized cases degeneration of renal tissue were more prominent than that of non-nephrectomized cases. Those results suggested that CDDP could be administrated to the non-nephrectomized case until 1,000 mg/m^2 in total dose, and that nephrectomy on the surgical treatment might be avoided as far as possible. In addition, high frequency of hearing loss (8,000〜4,000 Hz.) appeared at lower dosage of CDDP in this study, suggesting that ototoxicity might be more important dose-limiting factor.
Right aortic arch (RAA) was recognized in 18 of 571 patients with congenital heart disease. The main branches of RAA showed mirror image branching in all patients. The heart diseases associated with RAA consisted of TOF (10 patients), VSD with PS (3 patients), VSD with PA (3 patients), cor biloculare with PA (1 patient), and type I truncus artriosus (1 patient). RAA was thus found to be frequently associated with VSD and anomalies of the right ventricular outflow tract. Palliative surgery was performed in 9 patients. Blalock-Taussing operation was performed in 5 potients with TOF and in 3 patients with PA. One patient died of hypoxia. Brock procedure was conducted in 1 patient with TOF. In 14 patients, radical surgery was performed. Total correction for TOF was performed in 10 patients. Two patients died of low cardiac output syndrome. In 3 patients with VSD and PS, infundibular resection and closure of VSD were performed. In 1 patient with truncus arterious, Rastelli procedure was performed, but the patient died of a respiratory failure.
Torsion of the gallbladder which occurs mostly in adults is extremely uncommon in children. Only 15 pediatric cases have been published in Japanese literature so far. Because of its rarity, it is difficult to make a correct preoperative diagnosis. Only two cases was accurately diagnosed in the series of 15 cases. In this paper, we described a case of 13-year-old boy who was diagnosed as having acute cholecystitis by ultrasonography before surgery. At laparotomy, it was revealed that the gallbladder was enlarged and necrotic because of torsion at the bladder neck of 540 degree clockwise. Cholecystectomy was performed, and the patient was discharged uneventfully on the 13th day after operation. This case suggests that we should consider torsion of the gallbladder in acute abdominal emergencies.
A 6-year-old girl of the malignant small cell tumor of the thoracopulmonary region in childhood so called "Askin tumor" was presented. She had a huge mass occupying the left thorax. Total resection of the mass with combined excision of 7th and 8th ribs was performed. The defect of the chest wall was reconstructed with silastic cheet. Histological examination showed the small round cell, with PAS positive, hyperchromic nuclei, scanty cytoplasm and frequently presented nucleoli. Mitotic figures were also frequently seen. Homer-Wright rosette were present, though scanty in numbers, and immunohistichemical reactivity for NSE was positive. Immunostaining of Desmin, Myoglobin, LCA were all negative. After operation she received irradiation chemotherapy, and is currently free of disease for 12 months.
Recently an unusual mass presenting in the left neck of a 16-day-old boy was removed. The histology showed a thymic tissue. Previous cases reported in Japanese literatures were reviewed. Literatures revealed 11 infant cases and 18 adults in whom the thymus presented in the neck as a cervical mass. In infants, 8 cases demonstrated normal thymic tissues, 3 thymic cysts and one thymolipoma. In adults normal thymic tissue was observed in one, thymic cyst in 5, thymoma in 8 and malignant thymoma in 4. Ectopic thymic tissue of the neck in infants should be removed, because cystic degeneration or malignant change may occur in future. Unilateral thymic removal has little influence on patient's growth, but in cases with bilateral involvement only unilateral thymus should be removed in infants.