Immunohistochemical studies were performed on localization of substance P (SP) and vasoactive intestinal polypeptide (VIP) in human gut from patients with Hirschsprung's (H) disease. Gut from patients with other than H-disease was also studied. The following results were obtained; 1. In the normal human intestines; SP- and VIP-activities (A) were found in the whole layer. In the my enteric (Au) plexus, there were SP- and VIP-positive (P) axonal terminals surrounding nerve cells. In the longitudinal and circular muscle layers, beads-like SP- and VIP-P nerve fibers were observed. In the submucosal (Me) plexus, There were SP- and VIP-P axonal terminals surrounding nerve cells. In the submucosal and the muscularis mucosal layers, a few SP and normal level VIP-P nerve fibers were observed. 2. In the H-disease intestines; In the enlarged intestinal segment, SP- and VIP-A in the Au and Me plexus and both circular and longitudinal muscle layers were normal, or oc-casionally slightly less in cases of than normal. In the enlarged segment neighboring on the transitional segment, SP-A in the Au and Me plexuses diminished abruptly, accompanied by a decrease in SP-A in the proper muscle layers, and the activities were found to disappear in the whole layer, preceding disappearance of ganglion cells. While, an increase in VIP-P nerve cells in the Au plexus and VIP-P fibers in the muscle layers were observed. In the transitional segment, VIP-A in the Au and Me plexuses and the intermuscular space were reduced. In the aganglionic segment, there were a few VIP-A in the intermuscular space and intramuscularty, and some VIP-P fibers in the circular muscle layer, while, no VIP-P fibers were discernible within the muscular bundles. VIP-A was totally lost in the whole layer of the ileum and colon of extensive aganglionosis.
Clinical course of critically ill infants and children is sometimes complicated by unexpected thrombocytopenia following surgical interventions during anticancer therapies. The concen-tration of plasma β-TG and PF-4 which are considered to be the most sensitive markers of in vivo platelet activation was measured simultaniously with platelet count and aggregation in surgical patients, aged 20 days to 13 years, in order to evaluate the mechanisms of the loss of circulating platelet. In the case of thrombocytopenia induced by suppressed or impaired thrombogenesis of bone marrow resulting from sepsis or anticancer therapy, the levels of β-TG and PF4 remaind lower than normal range, however, those levels immediately increased following the improvement of bone marrow function. In the cases of Kasabach-Merritt syndrome, these proteins changed parallel with the size of hemangioma. In the case of idiopathic thrombocytopenic purpura (ITP), their platelets increased after splenectomy followed by the immediate increase of β-TG and PF4 above normal levels, suggesting the increase of absolute volume of intravascular destruction of plate-lets. In the recovery stage of disseminated intravascular coagulation (DIG), these proteins also increased parallel to the restoration of platelet count. In cases of neuroblastoma and a case of snake bite injury. platelet functions were accentuated by unknown mechanisms and β-TG :and PF4 concentrations also showed high levels in plasma. Although the cases are small in number in this study, it may be concluded that the measure-ment of β-TG and PF4 are useful in order to evaluate the abnormal platelet kinetics often observed in critically ill infant and children. Further investigation is neccessary whether the measurement of these proteins is valuable in early diagnosis of DIG.
The role of extracellular matrices during the development of ganglion cells of the chick colon was studied. Hematoxylin eosin and acetylcholinesterase stains were used to recognize differentiated ganglion cells. Organ culture techniques were used to know the stages when undifferentiated precursor cells migrate. Localization of three extracellular matrices, hyalu-ronic acid, sulfated GAG_s and fibronectin in the developing gut was examined using alcian blue stain (pH 2.5) with or without hyaluronidase digestion, 35^S-autoradiography, and immu-nohistochemistry respectively. In the distal colon of the chick embryo, ganglion cells were not recognized until stage 33 (8-day embryo). Organ culture studies revealed that the undifferentiated precursor cells had already migrated into distal colon at stage 29 (6-day embryo). Hyaluronic acid and fibronectin were rich along the way where precursor cells might migrate in the colon. Hyaluronic acid was depleted from the extracellular environment with migratory arrest and differentiation into the ganglion cells. Fibronectin, however, remained in the extracellular matrices during the ganglion cell differentiation. These results seem to indicate that hyaluronic acid and fibronectin promote the migration of undifferentiated cells and fibronectin accelerates the differentiation of ganglion cells.
In the surgical procedure of uretero-vesical anastomosis, submucosal tunnel method is widely used to prevent vesico-ureteral reflux (VUR). For the reimplantation of the dilated ureter in children, several techniques of ureteroplasty have been proposed to gain the appro-priate ureteral caliber to submucosal tunnel length ratio. The ureteroplasty which have been popularized for megaureter surgery is the resectional tailoring (tapering), in which a lateral ureteral strip is excised for a various length. There is always the risk to damage the ureteral blood supply with subsequent ischemic stenosis. To avoid this complication, we adopted, in recent 4 years, 2 new techniques of ureteroplasty without resecting the ureteral wall. One is plication technique, in which excess ureteral wall is inverted in the lumen of the ureter. Another is folding technique involving a running stitch to exclude a portion of ureteral lumen which is then folded around the ureter. The use of non-resecting technique is possible for any type of dilated ureter except for extremely widened and thickened ureteral wall. In the past 13 years, ureteroplasty for dilated ureter has been used on 33 ureters in 32 children. Of these ureters, 15 had primary obstruction, 8 had primary VUR, 6 had ectopic orifice and 4 had ureterocele. The postoperative evaluation was made on 32 ureters in 31 children, and an over-all success rate was 84 per cent. Of the 17 ureters operated on through tapering method, success rate was 71%. All ureters with non-resecting technique achieved satisfactory result. Analysis of the cases with unsatisfying result indicated the importance to differentiate the causes of ureteral dilatation. We emphasize that the secondary type of dilated ureter and nonrefluxing, nonobstructed megaureter must be excluded from the primary recon-structive surgery.
Eleven surgically significant neonatal anomalies have been diagnosed in utero by ultra-sonographic examination at our institution over the past three years. The experience with these cases reported here demonstrates following results: 1) Ultrasonographic diagnosis was specific in 8 cases (i.e. actites, pleural effusion, ompha-locele, pyloric atresia, duodenal atresia, diaphragmatic hernia, hydronephrosis), on the other hand it was not specific in 3 cases (i.e. ovarial cyst, choledochal cyst). 2) Antenatal diagnosis has been useful, since it has been possible: a) to get the rapid neonatal care without delay, b) to determin the appropriate obstetric management, c) to determin the fetal treatment, d) to observe the pathophysiology and natural course of the congenital anomaly. 3) In our limited experience, antenatal diagnosis has not been life-saving in the cases of diaphragmatic hernia and ascites.
During the last one year between April 1985 and March 1986, a total of 805 patients have undergone surgical procedures for a variety of lesions on the day care. Of these 805 patients, general anesthesia was employed in 777 and local analgesia in 28. The patients age ranged between 1 and 6 years in 513 patients (66%). Two patients out of 805 reguired over night hospitalization for observation of possible vesical injury during hernia repaire (1) and pneu-mothorax after removal of the intrasternal nail (1). A survey was carried out by mailing a questionaire to 200 random patients for postopera-tive course. In 148 responding patients, 50 patients (38%) had some degree of pain and 16 (11%) temperature elevation. Otherwise, there was no major complication. This study con-cludes that surgical therapy on day care basis in pediatric group of patients could be safely carried out.
Clinical and anorectal manometric assessments of bowel control was done in 4 patients with total colon aganglionosis and 1 with extensive aganglionosis. All of these 5 patients had undergone Martin's operation. The frequency of bowel movements in patients with total colon aganglionosis decreased 3 times a day 1 year postoperatively and 1 time a day 2 years postoperatively. On the con-trary, the frequency of bowel movements in the patients with extensive aganglionosis was 8 times in a day 1 year postoperatively. With regard to consistency of stools the patients with total colon aganglionosis had loose stool 3 months after Martin's operation, and formed stool 2years after Martin's operation. On the other hand, the patient with extensive aganglioncsis began to have loose stool 6 months after Martin's operation. Anorectal manometric study showed a gradual increase of rectal compliance with the passage of time. Therefore, these results indicate that an achievement of fecal continence after Martin's operation is depend on the increasing of rectal compliance as well as on a re-absorptive capacity of the residual aganglionic colon.
During 13 years between 1972 through 1985, 129 patients with biliary atresia were treated at the Tohoku University Hospital. Nine of them showed special form (subtype "d") of obstruction of extrahepatic bile duct. They can be divided into 3 groups. Group 1: Type III biliary atresia with large cystic dilatation of choledochus, Group 2: Type II and III biliary atresia with small cystic dilatation of choledochus which was surrounded by thick fibrous tissue, Group 3: Type III biliary atresia with solitary small cyst at hepatic duct or choledochus. Postoperative course was uneventful incases of group 1. All patients in group 2 showed prolonged jaundice, and the patients in group 3 showed high portal pressure at the time of operation. Mutual relationship among subtypes was suggested by their clinical and histological findings.
Dismembered pyeloureterostomy was performed for 46 pediatric cases of hydronephrosis caused by ureteropelvic junction stenosis. Postoperative shrinkage of dilated renal pelvis was remarkable in all cases but 1 case. In 1 case, adhesive kinking was occured on anastomosis portion and postoperative improvement was not obtained. From these results, this simple proced-ure was seemed to be first choice for hydronephrosis repair in children, however, anastmosis technique should be very meticulous and microsurgical technique would be advisable for better results.
The valve replacement has been performed in 18 children less than 15 years of age between 1968 and 1985. These valve replacements consisted of 8 left atrio-ventricular valve replacements, 7 aortic valve replacements, 2 right atrio-ventricular valve replacements and one pulmonary valve replacement. Ault-sized prosthetic valves were implanted in all cases except for three cases of less than five years of age. There were seven operative deaths. Of the 11 operative survivors, one death occurred within the cumulative follow-up period of 49.74 patient-years. The remaining 10 patients improved by at least one NYHA class and all are in Functional class I. Thrombo-embolic episode was encountered in one child who had sick sinus syndrome after MVR. In this patient, Dipyridamole and Aspirin were given for anticoagulation. Hemorrhagic complications occurred in 6 cases who were maintained on Warfarin medication, but thrombo-embolism was never seen in 9 patients with Warfarin. Based on these clinical experiences, we believe that prosthetic valve replacement is a reliable method for children with severe valvular heart diserses in spite of the difficulty and inherent dangers in controlling dosage of anticoagulant drugs.
Vitamin A absorption test was performed in 15 patients after hepatic portoenterostomy (1 to 7 years old); eleven patients were jaundice free (T. Bil.< 2.0mg/dl), four patients were jaundiced (T. Bil.> 2.0mg/dl). In patients without jaundice, plasma vitamin A levels were 56.4±26.5μg/dl at fasting and showed a rise to about six time the fasting level on average during the 4 to 6 hours following administration of a test dose of 7,500u/kg retinol palmitate. In patients with jaundice, plasma vitamin A levels were 21.4±5.8μg/dl and vitamin A absorption test showed lower absorption curves. These results suggest that vitamin A absorption is disturbed in patients with jaundice whereas patients without jaundice showed almost normal absorption.
Since birth the boy frequently developed vomiting and diarrhea, eventualy his weight gain was only 400g in the first year of life. Then he frequently had abdominal pain and severe diarrhea which needed repeated hospitarization. In his 12th year the GI series revealed Crohn's disease, and in his 16th year when his body weight was only 18.5kg, height 130cm, ileostomy associated with intraabdominal abscess and secondary cystitis developed. He was treated with no-oral-intake and intravenous hyperalimentation. Two months later resection of the damaged part of ileum was done. During three months after the operation he gained 5kg in weight and 2cm in height without symptom. Purberty, however, was not yet observed.
We describe a case with ectopia of the vas deferens in 5-year old boy who has the asso-ciation of rectal anomalies. He was evaluated because of the episodes of hematuria. Uro-logical examinations revealed right renal aplasia, vesicoureteral reflux in the left ureter and the ectopic insertion of vas deferens into the lower third of the ureter on both sides. Bilateral ureters issued in the normal position in the bladder. Right nephrectomy and left ureterocystoneostomy was performed. Bilateral vasa were implanted in the bladder neck through submucosal tunnel method. Postoperative course was uneventful. Histological examination revealed miniature seminal vesicle at the junction of the vas and the ureter on the right side. The right ureter showed partial vasation and the left vas was partially ureterized. The right kidney was severely dysplastic.
Three rare types of Wilms' tumor were reported with collective review. Case 1, 6-year-old male with right lower abdominal mass was operated. A tennis ball sized tumor was found in place with no relation to the kidney. He developed metastases in both lungs 2 years 8 months after the operation. Those pulmonary lesions were removed. He has been in good health. He is now 14-year-old. Case 2, 9-month-old female, the first born of non-identical twins, was operated because of a fist sized left abdominal mass. The tumor involved the upper and lower portion of the kidney. Complete excision was successful. The NWTS stage was II. She had been well for 7 years after the operation. The second born also had Wilms' tumor which involved both kidneys. She who had not been operated, died 2 years after the diagnosis was made. Case 3, 1-year 7-month-old female was admitted with a tennis ball sized left abdominal mass. Abdominal aortography disclosed that the left kidney was involved but right was not. The tumor involved the lower pole of the left kidney which was removed completely. The middle portion of the right kidney was involved and only involved portion was removed. Histologically, both lesions were nephroblastic type of Wilms' tumor. Lymph nedes were not involved. She had been well for 4 years 6 months since the operation.
We report a case of disseminated candidiasis in a patient with congenital duodenal atresia. A full-term male infant, birth weight 2,540g, was admitted to our hospital on day 2 because of bile vomitus with severe dehydration and metabolic alkalosis, Duodeno-duodenostomy was performed on day 5 for congenital duodenal atresia. He had been given cefotaxim and amikacin postoperatively and progressed well until the 4th postoperative day when he developed a temperature of 38.4℃. Several different antibiotics were tried but fever continued. His general condition worsened and finally complications developed, including DIG. Frequent exchange blood transfusions antifungal drugs (Amphotericin B and 5-Fluorocytosin) were effective in curing the DIG and eliminating positive Candidas of the blood, CFS, urine and skin lesions. On the 73rd postoperative day an indirect fundoscopic examination showed 2 white lesions in his right fund us and one white lesion in his left fundus. His left eye lesion developed a vitreous lesion despite antifungal therapy, although the right eye lesion disappeared. On the 117th postoperative day antifungal drugs were discontinued, although one small lesion in his left eye remained. The lesion is gradually improving 3 months after discontinuation of the drugs.
A case of herniation of the appendix, which formed an appendiceal-umbilical fistula, into the umbilical cord was reported. Herniation of the appendix into the umbilical cord is ex- tremely rare and only a few cases have been reported. According to these reports, its etiology remains unknown but two possibilies are suggested: 1) Accidental herniation of the appendix through the weak umbilical ring. 2) Persistent vitelline duct, which connects the appendix with the umbilical ring.
A 3-day-old male neonate was referred to this hospital presenting with frequent vomiting and abdominal distension. He had passed only a small quantity of mucous stool since birth. Physical examination revealed marked abdominal distension and absence of bowel sounds, and approximately 60 ml of greenish yellow fluid mixed with 30 ml of air was aspirated through the intragastric tube. An upright X-ray film of the abdomen revealed a markedly distended stomach and a minimal amount of gas located distal to the pylorus. On Ba-enema in a supine position, a vast amount of irregularly overlapped intragastric air was noticed, by which the microcolon was displaced to the caudal. An emergency operation revealed a markedly distended stomach associated with a slight dilatation of the duodenum. Annular pancreas was detected, and a seromuscular tear with a length of 11 cm in the posterior wall along the greater curvature was extended from fundus to distal body, part of the gastric mucosa being herniated through this defect. The defect was repaired with 2 layers seromuscular sutures without mucosal resection. The patient'srecovery was uneventful. Impending rupture of the stomach in the newborn was discussed, and a large, irregularly overlapped, abnormal intragastric shadow was considered as an important finding of impen-ding rupture of the stomach in the newborn.
A case of pancreatic carcinoma in a 7-year-old female is presented. The clinical and histopathological features are discussed as follow. There are no "squamoid" patterns in the histology ot this tumor. The immunohistochemistry showed that the tumor cells were positive for alpha-fetoprotein (AFP) and pancreatin amylase, and negative for carcino-embryonic anti-gen, somatostatin, insulin and glucagon. The above mentioned characteristic of this tumor strongly suggest acinus origin, rather than "pancreaticoblastoma" group which has been reported by Horie, et al. The serum AFP level was high and well correrated with the change of the tumor size. We could not find any case reports of such a AFP producing pancreatic carcinoma of acinus origin in adults. This fact many suggest that there are biological difference between the pancreatic acinar cell carcinoma in adults and children. It seems helpful to apply the recently available tumor markers such as pancreatin amylase and pancreatico oncofetal antigen (POA) for the elucidation of the histogenesis of this type of tumors. We treated this patient with combination chemotherapy of VAC, CDDP, and FT, resulting in only transient antitumor effects.
A case of congenital diaphragmatic hernia successfully treated by ECMO was reported. A female baby, weighing 3,468g at birth, was transferred to our hospital because of respiratory distress which started immediately after birth. Her left diaphragmatic hernia was repaired through the abdomen at 5 hours after birth. She belonged to the third group according to our classification, because her AaDO_2 level was 650mmHg on admission and 672mmHg after the operation. She temporarily recovered from persistent fetal circulation (PFC) at 12 hours of age by respiratory care and administration of tolazoline. But, after 9 hours, she reverted into PFC again which could not be controlled by any other therapeutic methods. ECMO was started at 59 hours after birth and the maximum flow rate was 312 ml/min (92ml/kg/min). Since her general condition had improved after ECMO was started, the setting of the respirator was decreased into FiO_2: 0.4, PIP: 20cmH_2O, Rate: 10times/min. 60 hours after the start of ECMO, it was weaned and the patient was converted to conventional respiratory care, which was discontinued when the patient was 13-day-old. There have been many cases where respiratory distress was successfully treated by ECMO in the world literature, but this case seems to be the first newborn case in Japan.
Two patients (6y, 3m) with endocardial cushion defect (ECD) and tetralogy of Fallot,and a patient (9m) with ECD, outlet right ventricle and pulmonary hypertension underwent corrective surgery with endocardial cushion prosthesis. Right ventricular outflow tract was reconstructed with an outflow patch only, in all cases. A 3-month-old boy, whose preoperative status was stomy, requiring 3 times of plasmophoresis for sepsis, died of low cardiac output. Two others survived the operation. Endocardial cushion prosthesis is considered to be appli-cable in patients with ECD and conotruncal anomaly.
Most of generalized type meconium peritonitis, in which perforation of the intestine occurred during or just prior to birth, accompanies massive ascites containing meconium. We experienced 2 cases of generalized type meconium peritonitis. The second case was diagnosed antenataly by fetal ultrasonography. Both cases accompanied severe respiratory and circulatory distress, and immediately underwent laparotomy for decompression and ileostomy. The first case was cured, but the second case died of generalized lymphangiectasia. We discussed the 19 reported cases of generalized type meconium peritonitis including our cases.
A female infant was born at the 38th week of pregnancy after an uncomplicated gestation of a 31-year-old mother. The birth weight was 2,550gr. and the APGAR score was 10. The child had a large solid tumor on the left-auricle. The mass was excised five days after the delivery. Microscopically, it was composed of spindle-shaped cells arranged in bundles, immature tumor cells, prominent vascular spaces with focal endothelial proliferation. The diagnosis was a congenital fibrosarcoma. Seven days after the 1st excision, radiotherapy followed by a wide local excision was performed. The patient was well and without recurrence 1 year after these treatment. A review of the medical literature indicates that wide local excision appears to be the treatment of choice for the congenital fibrosarcoma.
A 15-year-old girl with acute leukemia who had undergone Kasai's operation for congenital biliary atresia (CBA) at the age of 78 days is presented. Several episodes of ascending cholangitis occurred postoperatively but were controlled and jaundice had not been evident since the 70th day after the operation. At the age of 10 years, massive hematemesis from esophageal varices suddenly developed, and led to a esophageal transection, perigastric de-vascularization and splenectomy. At 15 years and 11 months of age, she suffered from high fever, anorexia, general fatigue and slight jaundice. Blood analysis revealed pre-B cell leukemia. Immediately after the diagnosis, she underwent antileukemic treatment, and a complete remission was obtained 1 month after the treatment. The long-term survivors of CBA have many problems mainly due to the hepatic dys-function. However, no case of malignancy has been reported yet, in spite of possibly higher carcinogenetic potential than in the general population, because of high levels of bile acid and abnormal bile acid in the serum which may cause malignancies. Moreover, impairment of the immunological surveillance system for malignancy occurs and possibly results in development of malignant cells in the patients undergoing splenectomy as well as those with hepatic dys-function.