Recent techinical improvements for the repair of hypospadias have made it possible to get the meatus to the tip of the glans. We have performed meatal advancement in 15 patients who had undergone the repair of hypospadias previously and had subcoronal meatus. Postoperative complications included 2 cases of fistula and 1 case of mild stenosis. The details of the surgical procedures are described.
This is a case report on Wilms tumor reptured through blunt trauma, complicated with direct invasion to the right lobe of the liver and the horse-shoe kidney in a 2-year-old boy. He initially underwent right hepaticlobectomy under the diagnosis of hepatoblastoma and subsequently right nephrectomy after the definitive diagnosis of Wilms' tumor. He was treated with NWTS-4 Regimen DD Postoperatively. There has been no sign of relapse of the disease in one year and 9 months after the operation. Although many pulmonary and hepatic metastases in the cases of Wilms' tumor have been described, but direct invasion of the liver has been rarely reported in the cases of this tumor. With regard to treatment for Wilms' tumor it is responsive to the combination of surgery, chemotherapy and radiation. Aggressive surgical management, nephrectomy and hepatic lobectomy, should be carried out not only for hepatic metastasis but also for direct invasion.
This full-term male newborn with maternal history of polyhydroamnios was intubated just after birth because of severe respiratory distress. The tube was recognized to be situated in the esophagus. Further examination revealed that the trachea was absent and both main bronchi originated from the lower part of the esophagus, heading upward. On the second day, he underwent gastrostomy and banding of the esophagus at the esophagocardiac junction. The tip of the endoesophageal tube was placed at the tracheoesophageal fistula and positive pressure ventilation was continued. However, maintenance of the adequate airway was difficult due to collapse of the fistula at the esophageal orifice. After struggling of 23 days, the patient died of severe pulmonary emphysema and subsequent right heart failure. Autopsy disclosed multiple anomalies; congenital heart disease, skeletal anomalies, etc. Sixty-five cases of tracheal agenesis were collected. Eight of them underwent surgical intervention in order to create an airway, to prevent aspiration of saliva, to prevent regurgitation of gastric contents, and to provide a route for feeding. There was no long-term survivor and it is still a challenging clinical problem to establish a stable airway for tracheal agenesis.
We report on a girl with a dermal primary congenital sarcoma of the left abdominal wall and we discuss this case with reference to the literature. Since the tumor in the left abdominal wall, already observed at birth, showed a tendency towards enlargement from 1 month after birth, she underwent resection at 3 months of age. Although the histopathological findings of the excised specimen clearly indicated a malignant sarcoma, the tumor cells showed a variegated histopathological picture. Based on the normal histological and electron microscopic examination, we were unable to determine a specific tissue type. On the basis of an immunohistochemical examination, she was disagnosed as having a rhabdomyosarcoma.
This is a case report of a liver cell adenoma occurring in a 2-year-old boy with no history of drug use. He was admitted for gradual abdominal distension and the presence of a tumor. Investigation revealed a slight elevation of serum α-fetoprotein, but the other liver function parameters were normal. CT showed a right lobe hepatic tumor with central necrosis, and <99m>Tc phytate liver scintigram revealed slight positive tracer localization in the tumor. Extended right hepatic lobectomy was performed, and a tumor measuring 9×7×7.5cm, and consisting of several cords and sheets of well differentiated hepatocytes without bile ducts or portal triad, was found. These findings were compatible with liver cell adenoma. Electron microscopy findings are also reported.
A 12-year-old boy was admitted with complaints of anal bleeding and anorectal pain. Barium enema and colonoscopic examination revealed polypoid lesions on both lateral walls of the lower rectum. The polypoid lesions (2.3×0.9×0.7cm and 3.0×1.2×0.9cm in size) were locally resected by transanal approach. Microscopic examination of the lesion revealed marked fibromuscular hyperplasia of the lamina propria, and the histological diagnosis was mucosal prolapse syndrome (MPS). The patient was instructed to avoid straining at defecation, and he has been asymptomatic for 12 months after the operation. MPS is a rare condition in childhood, and only 16 pediatric cases (including solitary ulcer syndrome of the rectum) are reported in Japan including this case. Twelve of the 16 cases are of boys, and polypoid lesions are reported in all cases. The treatment of MPS is not established. At present, the best treatment is local resection of large polypoid lesions and of ulcers, and instruction to avoid straining.
In recent years, an accepted operative procedure for choledochal cyst is excision of the cyst with hepaticoenterostomy. However, in 1971, before the pathological problem of a long common channel were recognized, we saw one case in which biliary reconstruction could be achieved by hepaticocholedochostomy after excision of a choledochal cyst. In 1989, 18 years after surgery, we had the opportunity to examine the patient and perform ERCP and echography. The patient has had no symptoms of pancreatitis or jaundice. The long common channel persists, but no dilatation, stenosis, stones, or malignancy, were found. This unique experience led us to two conclusions : 1. Stenosis is an important etiological factor in choledochal cysts. 2. Pancreatitis may not occur when the gallbladder is absent, when pancreatic duct pressure is always higher than biliary pressure.
Complete congenital atresia of female urethra is the rarest condition and is incompatible with life except the case with another route of urinary egress. We report a female case of congenital urethral atresia with urethrovaginal fistula, who had undergone cystostomy on the first day of life, and underwent reconstruction of the urethra when she was seven months old with result of complete continence.
A rare case of colo-colic intussusception caused by a adenomatous polyp in a 13-year-old male is presented. The patient admitted to the hospital complaining of abdominal pain and bloody stool. Abdominal plain X-ray examination revealed a filling defect in the descending colon. Ultrasonography showed a so called target sign in the same portion indicating a colo-colic intussusception. The intussusception was reduced successfully with a hydrostatic pressure. A barium enema demonstrated a polyp in the descending colon serving as a leading point. The following day, the polyp was removed by colonoscopic polypectomy. Pathological diagnosis was tubular adenoma.
A thirteen-year-old boy was admitted because of acute suppurative thyroiditis. Bilateral pyriform sinus fistulae were diagnosed by esophagogram. Before surgery we used microlaryngoscopy, and a Fogarty catheter was in serted into the fistula and we resected the fistula with the guide of this catheter. Bilateral pyriform sinus fistulae are extremely rare; only two cases have been reported, including our own case.
We report a case 2-year-old girl with total incontinence caused by complete epispadias. To gain the continence, bladder neck reconstruction was performed successfully. The operation resulted in 3cm length of neourethra and 50cm H_2O of maximum closed urethral pressure. The patient became completely continent 6 months later. Detailed surgical technique is described.
Two infants who developed intracardiac fungal masses secondary to total parenteral nutrition (TPN) were reported. One patient died, but the other was sucessfully saved. Case 1 was a newborn female infant who was admitted to the hospital with the diagnosis of omphalocele and ventricular septal defect. After the operations for them, she had been receiving TPN because of intolerance of enteral feeding. At 14 months of age, she developed candida sepsis, and at 23 months of age, she died of sudden hypoxemia. Autopsy findings revealed that the tricusped valve was occluded with fungal mass. Case 2 was a newborn female infant who was admitted to the hospital for chronic functional ileus. While she was treated with TPN for 5 months after birth, she suddenly developed candida sepsis. Two-dimentional echocardiography revealed a fungal mass in the right atrium. The fungal mass was detached during medical treatment for candida sepsis and pulmonary embolism supervened. She was treated with anti-coagulant therapy with heparin, urokinase and antithrombin III with favorable results. In this report, the diagnosis and treatment of intracardiac fungal mass and pulmonary, embolism in infants were also discussed.
Hemorrhagic necrosis and perforation of the intestine in patients with SHP has recently attracted attention as a surgical emergency. This is a case report of a 6 year 4 month-old girl who had three sections of hemorrhagic necrosis of the ileum at the first laparotomy, each of them was more than 10cm in length. The patient underwent peritoneal drainage and an appendectomy, because it was difficult to determine the viability of the subserosal hemorrhagic wall of the intestine. Fecal discharge from the drains prompted us to perform a reoperation 11 days after the initial operation. Subserosal hemorrhage had disappeared completely by the time of re-laparotomy, and only 10cm of stenotic and perforated intestine had to be resected without a massive intestinal resection. The authors recommend simple drainage and a staged operation for hemorrhagic necrosis in patients with HSP in order to avoid the risk of anastomotic leakage and massive resection of the intestine.