Purpose : Our modified anal sphincteromyotomy designed to improve the frequency of spontaneous bowel movements in children with idiopathic chronic constipation is assessed. Methods : Over the past 10 years (January 1989 to January 1998), we treated 6 children using our technique. A diagnosis of idiopathic chronic constipation was made when there was an absence of spontaneous bowel movements over a three-month period and no underlying organic pathology. Anorectal manometry was performed using a triple lumen catheter attached to a hydraulic manometry infusion system. All patients had an elevated resting anal sphincter tone on anorectal manometry and were unresponsive to medical treatment over a three-month period. All patients underwent our modified sphincteromyotomy, which involves resection of one third of the anal sphincter muscle and subsequent digital anal dilatation. Results : In 5 patients. a notable improvement in symptoms with regularization of bowel activity was obtained. Conclusion : The simplicity of the surgical technique, the absence of complications, and successful outcome confirm the validity of our modified sphincteromyotomy for the treatment of idiopathic chronic constipation in childhood.

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Purpose : To analyze the value of ultrasound (US) in the preoperative recognition of a contralateral patent processus vaginalis (CPPV), the presence of a CPPV was investigated by US in children with clinically diagnosed unilateral inguinal hernia. Materials and Methods : One hundred and sixty-six patients (80 boys and 86 girls) with unilateral inguinal hernia underwent US examination with a 7.5-MHz linear transducer. A CPPV was best visualized as hydrocele at straining and US scanning was performed while the patient was standing or crying. The presence of hydrocele in the inguinal canal at straining was regarded as a CPPV and the length and vertical diameter were recorded. The groins with CPPV were explored surgically and the US findings were compared with surgical results. In the preliminary study with 50 patients, 3 out of 4 cases with hydrocele under 10 mm in length were false positive. Then in the following 116 cases, hydrocele longer than 10 mm in length was considered as a CPPV. Results : In 166 patients, 38 cases (22.9%) were diagnosed as CPPV preoperatively and underwent surgery bilaterally. Thirty six out of 38 cases (94.7%) were confirmed to have a CPPV surgically. Conclusion : US is a noninvasive and accurate method for evaluating the presence of a CPPV. A CPPV was detected in 36 out of 166 patients (21.7%) with clinically diagnosed unilateral inguinal hernia, and a preventive operation against the second operation for the contralateral side was performed.

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Congenital lingual cystic teratoma are very rare. We report a case of female infant delivered at 38 weeks of gestation by caesarian section indicated by prenatal ultrasound showing a large mass occupying the oral cavity. She underwent tracheal intubation at birth due to respiratory obstruction. After tracheostomy and gastrostomy, OK432 was injected into the cyst with a presumptive diagnosis of lymphangioma according to MRI findings. A milky substance was aspirated. Cytological and pathological examination of the aspirate and cyst wall suggested thyroglossal duct cyst. The cysts were resected through a sagittal glossal split on day 138 after birth. The final pathological diagnosis was lingual cystic teratoma. Postoperative course was uneventful.

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A case of persistent pulmonary hypertension of neonate (PPHN) associated with congenital diaphragmatic hernia (CDH) was successfully treated with phosphodiesterase III inhibitor. The patient was a girl born by Cesarean section at 40 weeks of gestation because of fetal distress. She showed severe respiratory distress after birth, and was referred to our hospital. She was immediately treated with nitric oxide inhalation therapy and was operated on at 2 days of age. When PPHN deteriorated postoperatively, she received infusions of both trinitroglycerin and prostaglandin E1. They were effective for PPHN, although there were side effects. However, infusion of phosphodiesterase III inhibitor had an excellent effect on PPHN. She was satisfactorily weaned from artificial ventilation and nitric oxide inhalation while being infused with PDE III. Vasodilatory and inotropic actions of phosphodiesterase III inhibitor may be benefitial for the treatment of PPHN associated with CDH.

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Appendicitis is a common disease in children, but extremely rare in neonates. We present a neonatal case of perforated appendicitis with an abdominal tumor. Following unknown dyspnea during 2 days after birth, he had abdominal symptoms including vomitting, abdominal distension and a tumor with a diameter of 4 cm in the right lower abdomen at the age of 14 days. We had provisional diagnosis of intestinal duplication or ileal volvulus, and an operation was performed at the age of 21 days. A laparotomy revealed ileocecal abscess covered with the intestinal loops, and vanished appendix, which was suggestive of perforated appendicitis.

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Three cases of Morgagni hernia are described with emphasis on the clinical features of pediatric cases. Two were female and one was male. The hernia was right-sided in two cases and bilateral in one. Two cases were asymptomatic, but one case manifested respiratory distress after birth. The case with respiratory distress had bilateral large-sized hernias and was misdiagnosed as eventration of the diaphragm. All three cases underwent surgery. Although this disease is often diagnosed in adults as an incidental asymptomatic finding, in pediatric cases, this might be classified into two distinct groups : symptomatic with respiratory distress and incidental asymptomatic. Diagnosis requires chest plain radiography, contrast upper and lower GI tract imaging, and ultrasonography.

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We report a case of Peutz-Jeghers (PJS) syndrome with an appendiceal polyp. A 2-year-old girl presented with abdominal pain and she was diagnosed as intussusception. At laparotomy, an appendiceal polyp was found in addition to a cecal polyp. Histologically, the appendiceal polyp was characterized by branching bundles of smooth muscle fiber and many dilated cystic spaces containing mucinous exudate. Multiple hamartomatous polyps in PJS are commonly seen throughout the gastrointestinal tract except for the esophagus, but an appendiceal polyp is very rare. To our knowledge, this is the first report of a case with appendiceal polyp of PJS in children in the Japanese literature.

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We report a rare association of congenital pyloric atresia and diaphragmatic eventration. A 2, 506-g girl was born at 38 weeks and six days of gestation without a history of hydramnios. On the day of birth she began vomiting, and seven days after birth she was admitted to our hospital. A plain radiograph of the chest and the abdomen showed a dilatated stomach bubble partially protruding into the left thoracic cavity and no gas beyond the pylorus. A contrast study revealed an obstruction in the pyloric region. On the eighth day we performed a laparotomy. A median part of the left diaphragm protruded 30×25 mm in size into the thoracic cavity and was diagnosed as partial diaphragmatic eventration. In order to repair the diaphragm, we performed a plication. The pylorus showed a membranous obstruction, and we performed an excision of the membrane and pyloroplasty according to the Heineke-Mikulicz method. One day after the operation the tracheal tube was removed. On the fifth postoperative day, oral feeding was started. Congenital pyloric atresia is a rare malformation. To our knowledge congenital pyloric atresia associated with diaphragmatic eventration has not been described in the literature.

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We report a neonatal Richter's hernia with small bowel obstruction. Richter's hernia is quite rare in children, particularly in neonates. Only 3 cases were reported by Shanbhogue in 1984. All of the neonates, including our case, had external inguinal hernia. Diagnosis is often delayed, resulting in peritonitis due to perforation of the intestine. Richter's hernia should be considered to be one of the diseases with bowel obstruction in neonates and infants, as the differential diagnosis, especially with soft and reddish mass in the groin.

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