[Perioperative management of a neonate with aglossia-adactylia syndrome].

Aglossia-adactylia syndrome is a rare disorder characterized by aglossia and deformity of the limbs of variable degree. We managed a 2-day-old neonate with ileojejunal atresia with aglossia-adactylia syndrome. He was scheduled for the repair of the intestinal atresia, gastrostomy and palatal impression taking, under general anesthesia with sevoflurane supplemented with fentanyl. In this case we could achieve tracheal intubation by direct laryngoscopy with cricoid pressure applied, and could manage without any complications. But many of the patients with this syndrome are complicated with cleft palate and micrognathia, and we should be careful of the difficult airway at the induction of general anesthesia and aspiration during perioperative period.

[An extremely low birth weight infant with pulmonary atresia complicated with necrotizing enterocolitis].

We experienced an extremely low birth weight (ELBW) infant complicated with pulmonary atresia and necrotizing enterocolitis. She was born at 25 weeks of gestation with a birth weight of 752 g. Five hours after birth, she manifested cyanosis and was diagnosed as having pulmonary atresia with intact ventricular septum (PAIVS). Infusion of lipo-prostaglandin E1 (PGE1) was started to keep the ductus open along with infusion of dopamine and dobutamine. At 8 days of life she developed hypotension and metabolic acidosis, and the diagnosis of intestinal perforation was made by free air in the abdomen. The excessive shunt flow to the pulmonary vasculature via the ductus was suspected to have caused an inadequate systemic flow leading to the intestinal ischemia and necrotizing enterocolitis. Lipo-PGE1 was discontinued to decrease the shunt flow through the ductus. Brock's operation (closed transventricular pulmonary valvotomy) was performed at the age of 8 and 11 days and the ligation of the ductus arteriosus was performed at the age of 13 days. Propranolol was administered to inhibit the pulmonary outflow tract constriction along with catecholamines to stabilize hemodynamics. She recovered slowly and her trachea was extubated at 58 days of life.

Diagnosis of carcinoma of the papilla of Vater by duodenofiberscopy: simultaneous attempt on endoscopic observation, aspiration cytology, retrograde pancreatocholangiography and biopsy.

Clinical features and validity of a duodenofiberscopic examination for the diagnosis of carcinoma of the papilla of Vater were evaluated on 13 cases preoperatively diagnosed endoscopically. Followings are the conclusions derived. 1) Average age was 59.8 years. Incidence was equal among men and women. 2) As initial symptoms, general fatigue and easy fatigability or symptoms of cholangitis should be emphasized. 3) Other important symptoms include jaundice, fever with chills and weight loss. Abdominal pain, when present, was noted as one of symptoms of cholangitis in most cases. 4) Important laboratory findings include elevated serum bilirubin and alkaline phosphatase, elevated erythrocyte sedimentation rate, positive occult blood in stool. 5) Carcinomas of the papilla of Vater were divided into 3 types (type I, II and III) according to endoscopical and pathological findings. Clinical features and laboratory findings were discussed in relation to the type of lesions. Characteristic endoscopic findings of each type of the lesions were described. Validity of aspiration cytology, pancreatocholangiography and biopsy under duodeno-fiberscopic observation was also discussed. As a result, duodenofiberscopy was considered to be the most useful method for the diagnosis of carcinoma of the papilla of Vater because it provides us with an opportunity to perform simultaneously an endoscopic observation, aspiration cytology, pancreatocholangiography and biopsy.

Generalized peritonitis with pneumoperitoneum caused by the spontaneous perforation of pyometra without malignancy: report of a case.

Spontaneous perforation is a very rare complication of pyometra. We report herein the case of an 88-year-old woman who presented with muscular rigidity and free air on abdominal X-ray films. Perforation of the gastrointestinal tract was diagnosed preoperatively, and an emergency laparotomy was performed. A total hysterectomy with bilateral salpingo-oophorectomy was carried out under the diagnosis of generalized peritonitis caused by the spontaneous perforation of pyometra. The culture of purulent fluid from the abdominal cavity showed only Escherichia coli, with no anaerobic bacteria. Histological examination revealed pyometra with necrosis of the endometrium and no evidence of malignancy. The patient was discharged on postoperative day 68 without any major complications. Pyometra is an unusual cause of peritonitis, but it must be considered as a possible diagnosis in elderly women presenting with an acute abdomen. Following this case report, we discuss the problems associated with establishing a correct preoperative diagnosis of generalized peritonitis caused by the spontaneous perforation of pyometra.