There have been few documents for histopathological examination on congenital esophageal atresia. Recent increasing number of reports in which there is coexistence of congenital esophageal stenosis due to tracheobronchial remnants and esophageal atresia has led to the histopathological study of the anomaly. The specimens from 6 babies not operated upon were studied. Microscopic examination showed seromucous glands, resembling tracheo-bronchial glands, in the muscle coat of the fistula in five of the six specimens examined. In three of the specimens, fragments of cartilage were identified. The transition from the fistula to normal esophagus occured at a variable distance from the tracheo-bronchial end - from 1 to 24mm.
Serum immunosuppressive acidic protein(IAP) was measured by single radial immunodiffusion method in 21 patients with neuroblastoma. Serum IAP of normal children was 382.3±133.6μg/ml(n=31)(mean ± SD). Serum IAP of patients with neuroblastoma correlated with clinical stage except stage IVS. It was significantly higher in patients with stage III and IV than in normal children. It was within normal range in patients with stage IVS. Our results suggested that immunological response of patients with stage IVS is not impaired. Serum IAP did not relate with histology of tumors and urin VMA, but did with serum LDH (r=0.483). It was found that serum IAP before treatment was higher in dead patients that in alive patients. In conclusion, serm IAP is an useful marker for neuroblastoma in anticipating the prognosis of these patients.
Manometric studies including both resting pressure tracing and esophageal motillty tests were performed in twenty six children with hiatal hernia and following conclusions were obtained. 1. The resting pressure tracings before operation could classified into three patterns, indicating the phisiological condition of the esophago-cardiac junction and the right crus of diaphragm. 2. The anti-reflux function before operation was directly influenced by whether the sphincter mechanism at the esophagocardiac junction is preserved or not. 3. GER inducing test was valuable for evaluation of the anti-reflux function both before and after operation. 4. The high pressure zone at the esophago-cardiac junction remained at the low level shortly after opertion, but it increased gradually and reached the normal level by one year. 5. The esophageal motility function was not impaired both before and after operation.
The presence of NES and S-100 protein was investigated immunohistochemically using paraffin sections of surgically resected tumor tissues of 31 neuroblastoma group tumors in children. The tumors studied consisted of 26 neuroblastomas, 4 ganglioneuroblastomas and 1 ganglioneuroma. Malignant Schwannoma, Wilms' tumor, hepatoblastoma and rhabdomyosarcoma were also examined to compare with neuroblastoma tumors. NSE was detected in 30 of 31 neuroblastoma group tumors and 31 neurs Hastoma group in out of rhabdomyosarcoma. Som ganglion-like cells appearing in the ganglioneuroblastoma and ganglioma were atained strongly with anti-NSE but others were negative. 4 ganglioneuroblastoma and 1 ganglioneuroma were positive for S-100 protein and all other kind of tumors investigated were negative. In tissues of the ganglioneuroblastoma ane ganglioneuroma, Schwann cells and the satellite cells of the ganglion were stained strongly. It is concluded that NSE stain is a reliable method for distinguishing neuroblastoma fram other non-neuronal round cell tumors in children, and that there was a good correlation between the intensity of NSE stain and histological differentiation of the neuroblastoma group tumors. If was conduded also that the fumor wite positive cells for S-100 protein have a good prognosis.
Three patients (case 1,2,3) with cor triatriatum were operated successfully. Preoperative diagnosis was established by cardiac catheterization, angiography and/or echocardiography. All cases were associated with atrial septal defect, connecting the accessory chamber in case 1 and 2, and the true chamber of left atrium in case 3. A excision of the abnormal membrane in left atrium and closure of atrial septal defect were carried out through the right atrial approach. The operation were elective in case 1, and urgent in case 2 and 3 because of pulmonary hypertension and congestive heart failure. The clinical features and hemodynamics in this anomaly are characterized by size and localization of the communication between accessory and true left atrium. Satisfactory operative results can be achieved by a complete excision of the abnormal membrane in the left atrium, based on a correct preoperative diagnosis.
During the 17-year period, 1968 to 1984, eighteen children underwent splenectomy for hereditary spherocytosis (HS) at our department. There were 9 males and 9 females. Patient's ages ranged 3 years to 12 years (average 6 years). Eleven patients (61%) had a history of familial transmission of HS. Anemia and/or jaundice were the most common presenting symptoms. Splenomegaly was noted in 16 (89%) of patients at time of initial physical examinations. The clinical diagnosis of HS were confirmed in all prtients by the presence of spherocytes in the peripheral blood, an elevated serum bilirubin level, reticulocytosis, a negative result of Coombs' test, as well as a positive result of the osmotic fragility test. Accessory spleen was found in 10 (56%) of the patients, Splenectomy and cholecystectomy were performed simultaneously in one patient who had a preoperative sonographic diagnosis of cholelithiasis. All 18 patients improved following splenectomy. Ther was no case of postsplenectomy sepsis (overwhelming postsplenectomy infection) in our series.
CT was applied to 5 normal children an 16 children with various types of anorectal malformations, in order to make direct image of anal sphincters. In normal and low type anomalies, CT demonstrated the clear images of puborectal muscle and external sphincters. Among high type anomalies, the distribution of sphincter muscles in rectovesical fistula is totally different from those in rectovesical fistula. In rectourethral fistula, puborectal muscle is attached not only around the urethra, but also around the blind rectum, and external sphincters are also present as a mass beneath the perineum. In the rectovesical fistula, however, puborectal muscle can not be identified and external sphincters exist only as a strand like structure. CT was also useful to assess postoperativly whether the colon was pulled through correctly within the center of sphincter muscles. CT has demonstrated clearly the differences among three types of postoperative incontinence, namely, sphincter dysfunction, missed puborectalis and markedly hypo plastic sphincters. CT has been proved to be an adequate imaging modality for the evaluation of anorectal malformations.
Total parentaral nutrition (TPN) by central vein catheterisation has become one of the important treatments for saving lives of ill infants. In paticular, the route for achieving superior vena cava catheterisation provides a most satisfactory method and has been used widely. However, sepsis associated with the catheterisation still remains as a hazardous complication. To protect patients from the catheter-associated sepsis during TPN, the new procedure of Subclavian Vein Catheterisation (SVC) with subcutaneous tunnel was devised. The feature of this technique was to use a Peel-off Cannula for the introducer of the catheter and a vennula needle for making the subcutaneous tunnel wth ease and safety. In this Paper, our experience of SVC with subcutaneous tunnel during the past 2 yr. from Dec. 1982 through Dec. 1984 is reported. And the advantages of subcutaneous tunnel to protect the catheter-inserted site of the skin from the infection is emphasised.
We experienced 5 neonates with total anomaious pulmonary venous connection, includidng one with common pulmonary vein atresia. Alll babies but one had cyanosis and tachypnea soon after birth. Chest x-ray showed small heart and fine reticular shadowing in the lung fields. Preoperative pulmonary arterial pressure was high as same as the systemic level in all babies, and pulmonary vein pressure was 27-44 mmHg. The operation was performed 2-19 (9±9) days of life and body weight at the surgery was 2.5-3.3 (2.7±0.3) kg. Total circulatory arrest was used in 4 babies. Three of them survived the operation, and two died. One was operated upon under the cardio-pulmonary bypass without total circulatory arrest and reguired the longest aortic cross clamping time, dying to low cardiac output and sepsis. The other died of severe low cardiac output due to myocardial rupture during operation.
From 1974 to 1983, a total of 116 children (202 ureters) with primary vesicoureterl reflux (VUR) were treated by the ureteroneocystostomy. There were 60 males and 56 females. Their ages ranged from 4 months to 15 years, with a mean age of 6.2 years. Our principal surgical techniques were modified Paquin and modified Politano-Leadbetter (P-L) methods. In either method, the submucosal tunnel was created as long as 3 to 4 cm and the new ureteral orifice was located about 1cm distal to the original one. Postoperative complications were as follows: modified Paquin method (140 ureters) - distal ureteral stricture (4 ureters), paraureteral diverticulum (2 ureters) and persistent reflux (1 ureter), modified P-L method (44 ureters) - paraureteral diverticulum (1 ureter) and persistent reflux (1 ureter). Therefore, the success rate of the both methods was 95%. It is concluded that most of VUR can be successfully corrected by intravesical ureteroneocystostomy such as modified P-L method. However, there are a few VUR with massively dilated ureter for which combined intra-and extravsical procedure such as modified Paquin method is necessary, and surgeons must also be familiar with this method.
In two cases of biliary atresia associated with preduodenal portal vein, liver biopsies were done twice for each patient to see chronological histologic changes before corrective radical surgery. The histology demonstrated significant information on early changes of the liver in such cases. In the first case, liver biopsies were obtained on the 3rd and 52th days of her life before radical operation performed on the 70th day. The first specimen showed almost normal appearance of the liver of a newborn, while the second revealed portal fibrosis, cholestasis and giant cell transformation typical in biliary atresia. In the second case, liver biopsies were done on the 26th and 56th days of his life and radical operation was performed on the 66th day. he first specimen exhibited moderate cholestasis and mild giant cell transformation without portal fibrosis. On the other hand, the second specimen showed severe giant cell transformation, portal fibrosis and ductular proliferation containing occasional bile plugs as often seen in biliary atresia. It is considered that extrahepatic biliary obstruction has been completed at birth in biliary atresia with preduodenal portal vein, because the pathogenesis of this particular entity is thought to be related to developmental anomaly. In our present cases, hepatic changes were minimal in the early days of life, and progressed rapidly with the time. It is, therefore, suggested that, hepatic changes in biliary atresia develops mainly after birth as the results of obstruction of the bile duct.
Malignant ovarian tumors of childhood are infrequent and most of them are of germ cell origin. The literature on malignant pediatric ovarian tumors makes it difficult for the physiciam confronting this disease to find and utilize an optimal therapeutic approach. In our hospital, malignant mixed germ cell tumor, combined with dysgerminoma and endodermal sinus tumor, in a 8-year-old female was treated without sign of recurrence, by surgery, radiation (2,970 rad at lower abdomen, and 3,000 rad at para-aortic region), and VAB therapy (Vinblastine, Actinomycin-D, and Bleomycin). With a review of literature, embryology, classification, treatment, and prognosis of the mixed germ cell tumor were briefly discussed.
Circular myotomy was required in two patients with esophageal atresia to achieve an tension-free end-to-end esophageal anastomosis. One case on whom the blunt dissection of muscle layer at myotomy site was performed, developed a marked ballooning at the site of myotomy causing recurrent aspiration pneumonias. The other case on whom simple circular myotomy without further procedure was performed, did not develop esophageal dilatation and showed a good postoperative course despite poor lower esophageal clearance. The ballooning at myotomy site causes poor emptying of the esophagus and when concomitant gastroesophageal reflux, anastomotic strictue or poor motility of lower esophagus are present, they aggravate esophageal stasis and lead to the occurrence of dysphagia and trouble-some repeated aspiraton pneumonia. Therefore, it seems important to avoid the muscular dissection at Livaditis operation to keep the amuscular segment short.
Reversed rotation is a rare type of intestinal malrotation. Four of the 61 cases with malrotation treated at our department during the period from 1963 to June 1985, were reversed rotation. A 2-day-old baby boy was the retroarterial left-sided cecum type of reversed rotation. 12-year-old boy and 5-day-old baby boy were the right-sided cecum type. A 13-year-old boy was the prearterial right-sided cecum type. Four were admitted with bilious vomiting. Three were treated with postioning nonrotation of Ladd's Procedure and one was treated with modified Bill's procedure.
Two pediatric patients with acute pancreatitis are described. The disease was speculated to have been induced by cholelithiasis. The first patient was a 5-year-old girl, who complained of upper abdominal pain and vomiting. The levels of serum and urine amylase were markedly elevated, indicating that she had pancreatitis. Ultrasonography revealed the dilatation of both the gall bladder and common bile duct. ERCP showed a small gall stone in the common bile duct. The stone was removed by operation, which was mainly composed of cholesterol. The second patient was a 15-year-old boy, who had , nausea, vomiting and upper abdominal pain. He was diagnosed as having pancreatitis because of the extremely high values of amylase and lipase both in the serum and urine. A plain X-ray film of the abdomen and ultrasonography revealed cholelithiasis. The stone was spontaneously excreted in the feces after 7 days of observation, which contained only a tiny amont of calcium phosphate.
A 7-month-old girl with Cushing's syndrome caused by left adrenocortical carcinoma and the result of determination of hormones in the supernatant of tumor cell culture were reported. The baby was admitted with the following clinical manifestation of Cushing's syndrome: general obesity, moon fade, hypertension and hypertrichosis. The urinary excretion of 17 KS and 17 OHCS and the concentration of serum cortisol were elvated almost 5 times as nomal baby. A left adrenal tumor was diagnosed by ultrasound, CT scan and angiography. Complete extirpation of the tumor was performed successfully. Pathological diagnosis was adrenocortical carcinoma. Postoperative replacement therapy with hydrocortisone and predonine was given for 330 days. Remarkable elevation of adrenocortical hormones was demonstrated in the culture supernatant of the tumor cells. The patient is healthy with no evidence of recurrence 16 months after the operation.
This is a report of one year and 9 months old boy with a congenital cervical esophageal membrane stenosis. Typical symptoms consisting of reccurrent choking, cyanotic episode during the feeding and dysphagia with semi solid or solid foods were presented. Cine- or video-esophagram with bottle feeding of Barium was very valuable for the diagnosis. Esophageal endoscopy was carried out for diagnosis and treatment. The membrane was excised endoscopically with an electro surgical unit and bouginage was added. Ten cases of congenital esophageal membrane stenosis have been reported in Japanese literature but the case with the cervical esophageal stenosis has not been reported.
A very low birth-weight female infant (852 g) was surgically treated for necrotizing enterocolitis. At the age of 6 days, she was diagnosed as having intestinal perforation by transillumination test. Laparotomy revealed multiple lesions in the whole intestines with multiple perforations in the colon. A double barreled distal ileostomy was made. Closure of patent ductus arteriosus was done because of progressive heart failure. Partial colectomy was made because of the stricture of the colonic lesion. On 234 days after birth she was discharged at 3,875 g body weight. She is 2 years and 6 months old with satisfactory mental and physical condition at present. Surgical care of necrotizing enterocolitis in a very low birth-weight infant is discussed.
We experienced a case of gastric rupture associated with the left diaphragmatic hernia with sac seen in a 5-day-old female neonate. She was treated by two-staged-operations with success; suture of the gastric rupture with drainage on the day of admission and repair of the diaphragmatic hernia on the 53rd day of life. In the Japanese literature, 21 neonatal cases of gastric rupture associated with diaphragmatic hernia have been reported. As gastric rupture seems to be one of the most common and the most hazardous complications of the left diaphragmatic hernia, especially with sac, the hernia should be repaired as soon as possible, even if no symptoms are noted.