We conducted a cooperative study to assess the usefulness of intralestional injection of OK-432 in childhood lymphangioma. The concentration of OK-432 (Picibanil) was set at 0.5 KE/10 ml of physiological saline in group A and at 1.0 KE/10 ml in group B on the basis of a phase II clinical study. The tumor redcuction rate was more than 80% in both groups A and B when evaluated at 6 months after the start of treatment. Side effects occurring during treatment were similar in both groups. Fever, swelling and reddening of the tumor, an increased white blood cell conut, and an inreased CRP level were common, but none of these reactions were serious. In the case of cervical lymphangioma, however, care must be taken to avoid tracheal compression when the mass becomes swollen following the intralesional injection of OK-432. When the usefulness of OK-432 was evaluated on the basis of the degree of tumor reduciton and the safety, more than 70% of the patients in both groups A and B received an evalutation of "useful" at 6 months of treatment. This therapy acheived a good cosmetic result, and was suggested to be a promising alternative to conventional therapy.

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A 2 month-old male infant with dyspnea was admitted to our department. CT and MRI showed a mediastinal cystic mass compressed the left main bronchus, and caused left lung emphysema. Someday dyspnea deteriorated suddenly, and chest X-ray film revealed right lung emphysema and atelectasis of left lung. This episode returned to the former condition 5 dyas later. Operation revealed a gourd-shaped paratracheal cyst constricted by azygos vein. This cyst attached to the trachea by a bronchus-like tissue involving cartilage, but no luminal connection. The cyst was multicystic and contained colorless mucinous fluid. The cyst wall was composed of ciliated cylindrical epitheliums, cartilages, smooth muscles and bronchial glands. Histological diagnosis was mediastinal bronchogenic cyst. Infantile mediastinal bronchogenic cyst often causes dyspnea because of airway weakness. In this case, it was peculiar that the side of emphysematous lung changed by minimal motive preoperatively.

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We reported a case of tracheal agenesis with complicated heart anomaly and otic imperforation. A female infant weighing 1938g, was born at 31 weeks' gestation with respiratory distress. Endoscopic examination demonstrated agenesis of trachea and existence of bronchoesophageal fistula. With esophageal ventilation, abdominal esophageal banding and gastrostomy was performed. Because of aggravation of respiratory status, we performed cervical esophagostomy, but she died at 8 days of age of esophageal rupture and pneumomediastinum. We have experienced 4 cases of tracheal agenesis and one of them has been alive for 5 years and 9 months. Three dimension helical CT, performed in the living case and the recent case, showed clear anatomical structure. In the former case, tracheoesophageal fistula was large, and there was the lower part of trachea. On the other hand, in the later case, the bronchoesopahgeal fistula was very narrow, and the trachea was completely absent. It is considered that the existence of large tracheoesophageal fistula or insert of endotracheal tube into the fistula would be required for stability of the endotracheal tube. In addition, we summarized 23 domestic cases of tracheal atresia, including our 4 cases.

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Two cases of cholelithiasis in children were reported. [Case l] A 2 year and 2 month old boy was admitted with upper abdominal pain and vomiting. The levels of liver and biliary enzymes were high. He was diagnosed as cholecystolithiasis by abdominal ultrasonography. On ERCP examination, cystic duct was mostly parallel to common hepatic duct and gallbladder was displaced to-ward porta hepatis i. e. "transeverse position of gallbladder". He had neither choledocholithiasis nor anomalous pancreaticobiliary ductal union. Cholecystectomy was performed and a stone of body in gallbladder and a stone of cystic duct were found. These stones were made of bilirubin. The patient is active and well at one year and nine months after surgery. [Case 2] A 4 year and 2 month old boy was admitted with abdominal pain and vomiting. On abdominal X-ray study, four calcifications of bean size were found at right side of 1st lumbar vertebra. Anomalous pancreaticobiliary ductal union didn't exist on ERCP examination. Intraoperative cholangiography revealed neither intrahepatic stone nor choledocholithiasis. Cholecystectomy was carried out and four stones made of calcium carbonate were fonud in gallbladder. The patient is active and well at five months after surgery.

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There have been reported congenital remnants which caused bowel obstruction in infants. Because of the variability and nonspecificity of symptoms, the diagnosis is often not made until intraperitoneal detection. A 76-day-old premature baby, who had undergone Ramstedt's operation at 17 days old, was tranferred to the Department of Pediatric Surgery because of vomiting with bile free vomitus. On admission, physical examination noted the patient was afebrile and in no distress. At operation, fibrous band aproximately 5 mm in diameter and 4 cm in length was found. It was extended from the antimensenteric site of the ileum approximately 50 cm from the ileocecal valve to the fundus of an normal appearing gallblader. The band was ligated at its margins and resected. The ileum was constricted at the site of attachment to the band. The strictured portion of the ileum was resected and an end-to-end anastomosis was performed. His postoperative course was uneventful. A review of the literature suggests that this band is mostly consist with persistence of a remnant of the distal vitelline veins.

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Paraurethral cysts in the female neonates are uncommon lesions. A 7-day-old and a 13-day-old female neonates were found to have a 2 cm and a 1.5 cm cystic mass respectively at the introitus and inferior to the urethral orifice. Simple marsupialization were done. There have been no signs of recurrence. The cyst wall was lined with stratified squamous epithelium. Many congenital cysts of the different origin at the external genitalia have been reported in newborns and infants. The histologic findings of the cyst walls are correlated with, and furthermore identify their embryologic origin. Therefore, histologic examination is important.

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A 5 year-old boy was admitted into our hospital for a urinary tract infection (UTI) after 4 years interval of the first reference. An ultrasonogram (USG) and an intravenous pyelography (IVP) revealed a caliceal diverticulm of the left kidney. He had two episodes of UTI. An USG and an IVP had been performed recurrently, but caliceal diverticulm had not been found previously. The etiology of a caliceal diverticulm is usually discribed as congenital : i. e., an embryological maldevelopment of the pelvio-calyceal system. But this theory is only a suggestion and has not been proven. In our case, a caliceal diverticulm that had not been found in infancy was detected at 5-year old. His caliceal diverticulm was considered to be acquired. His clinical course has been carefully followed and there has been no change in size or in morphology during the past 6 months. In literture, there have been no reported cases of acquired calceal diverticulm, and few reports about careful follow up. Our case is considered to be interesting for the investigation of the etiology of caliceal diverticulm.

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One of the complications of necrotizing enterocolitis is intestinal stenosis. A report of colonic stricture in 14-day-old male infant born at fullterm is presented. He was admitted with compaints of vomiting and abdominal distension. Barium enema demonstrated stricture in descending colon. At laparotomy, stenotic bowel 2.5cm in length was resected and primary anastomosis was performed. Histological examination of the resected tissue showed healing phase with granulation and fibrosis. The infant had a past history of pneumonia in the first week after birth. At the time he showed digestive tract symptoms and pneumatosis intestmalis which were in agreement with the clinical picture of necrotizing enterocolitis. This is a complication which should be kept in mind during the recovery stage of necrotizing entercolitis.

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Four children underwent appendectomy under the diagnosis of appendicitis at our hospital. At operation a moderate amount of clear ascites was found in each case. In 3 of the 4 patients, there were pine worms in the appendix. Histological examination revealed moderate inflammation of the appendix and showed a worm in the appendices in all 4 patients. Enterobius vermicularis is known to parasite in the human terminal ileum, cecum and appendix. The female worm migrates to the anal and perineal region to lay off eggs in the night, which causes intolerable itching, sleeplessness and irritability. Sometimes parasitic lesion affects the appendix and gives rise to acute appendicitis. Inflammatory reaction of the appendix caused by Enterobius vermicularis characteristically leads to a mild to moderate amount of clear ascites. Histological examination usually showed mild degree of inflammation of mucosa in appendix.

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The occurence of intramural hematoma of the duodenum is relatively rare in Japan. The majority of these cases are due to blunt abdominal trauma, and have a high prevalence in children, because of their physical properties. We present here in five cases of traumatic intramural hematoma of the duodenum in children; three cases recovered with conservative therapy while the other two cases needed surgical therapy. All patients had abdominal pain and vomiting soon or a little after the trauma. Ultrasonography, upper Gl series, CT were useful to make the diagnosis or to distinguish the other associated injuries. In two cases, duodenal endoscopy was undertaken, which was useful for not only making the diagnosis but also being an aid of the conservative treatment. With recent progresses of intravenous hyperalimentation, non-operative therapy has been the first choice for the treatment of this condition. Howevere, surgical intervention should be considered in cases of failure of conservative therapy or in cases of supervening complications.

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A 5-months old girl was admitted complaining of fever and abdominal distension. Constipation had necessitated daily enema after 3 montsh of age. The abdominal distension was caused by the fecal masses and the distended bladder due to a large presacral abscess, which was incised and drained immediately for relieving of urinary and colonic obstruction. Though the abscess being incised, constipation and mucopurulent discharge from the anus have persisted since this episode. Contrast studies of the rectum revieled a presacral mass with arborescent draining fistulas associated with anorectal stenosis. MR imaging suggested that the abscess was tightly adhered to the lower sacrum and the coccyx. At the age of 12, a sacroperineal total resection of the presacral abscess with coccyx for eradicating the abscess and rectal stenosis was performed but, actually, recurred a week later. Seven months later second attempt of total extirpation of the abscess was done combining with resection of the 5th sacrum and the rectum according to original Soave procedure for Hirschsprung disease. However, 4 weeks later mucus discharge was obseved again through the anus. The resected specimen were composed of fibrous tissue and of various-sized mucus containig cysts lined by squamous, transitional and columnar epithelia surrounded with smooth muscle but not cartilage or neural tissues suggesting a teratoma. These histological findings were compatible with tailgut cyst, which predominantly occur in adult female but extremely rare in children.

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A successful management of esophageal stricture in a case of Gross A type esophageal atresia is reported. The postoperative esophageal stenosis was associated with gastroesophageal reflux, and moreover, was complicated by a perforation at an attempt to dilate the stenosis, which resulted in a complete stricture. Endoscopically a quide wire was introduced into a pinhole stricture of the esophagus, then pulled by a string through from the mouth to the gastrostomy. A string-guided bougienage using a newly developed vinyl chloride cement apparatus was successfully performed. Sucking milk, allowed during the preoperative period with the aid of continuous suction of the proximal esophageal pouch, seemed to have prevented from the later difficult feeding problem. the patient is currently 4 years old and has normal baruim swallow without stricutre of gastroesophageal reflux.

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Two cases of successful reversal from PFC (persistent fetal circulation) by inhaled NO are reported. CASE 1 : A male neonate, 2580 gm/37 weeks, with perforated ileal atresia was transferred in a shock condition. After resuscitation, resection of the necrotic segment and ileo-ileostomy were done. Postoperative course was complicated with necrotizing fasciitis of abdominal wall, sepsis, and PFC. Intracranial hemorrhage and ongoing hemolysis precluded ECMO. Inhaled NO therapy (20ppm) dramatically reversed PFC and was discotinued at 48 hrs of therapy. CASE 2 : A male neonate, 2330 gm/36 wks, was transferred due to congenital diaphragmatic hernia (pH 7.19, AaDO_2 631mmHg). He was stabilized with HFO and underwent repair, using Cortex sheet and skin flap closure of the abdomen. PFC developed but was reversed by NO therapy (6ppm). Retrospectively, this case was associated with severe lung hypoplasia. The inhaled Nitric Oxide (NO) therapy is an effective therapeutic modality for PFC in neonates and is more easily accessible than ECMO.

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