A 2950-gm infant girl was born at 36 weeks' gestation to a 25year-old mother with two previous live born children, one of whom had gastroschisis. The pregnancy was complicated by oligohydramnios and a sonogram demonstrating markedly dilated cerebral ventricles, a cleft palate, and club foot. At birth, the child was noted to have flexion deformities of the right upper extremity digits, hydramnios, cleft palate, and a left club foot. Apgars were 2 (10 minutes) and 5 (40 minutes). An echocardiogram demonstrated very poor left ventricular function. There was slight macrocrania, and a cranial sonogram (Figure 1) was performed. The child had a cardiorespiratory arrest and died soon after the cranial sonogram. The parents declined further investigation or autopsy except for skin biopsy.

No abstract

A 2410-g infant was born at 38 weeks' gestation after an uneventful pregnancy and labor. The child was noted to be cyanotic and to have a 4-cm substernal omphalocele, which was pulsatile. The sternum was thought to be bifid inferiorly. Plain radiographs of the chest and abdomen (Figure 1 DENOUEMENT AND DISCUSSION Pentalogy of CantrellThe abdominal sonogram and MRI demonstrated an omphalocele containing a small portion of liver, bowel loops, and a trilobed tubular structure with arterial flow that could be traced directly to a diverticulum arising from the cardiac apex. These studies also demonstrated the bifid sternum and defect in the diaphragm through which the ventricular diverticulum passed into the omphalocele. The cardiac sonogram demonstrated tricuspid and pulmonic atresia with a hypoplastic right heart. A diagnosis of pentalogy of Cantrell was made based on these findings. The patient was taken to the operating room. A trilobed left ventricular diverticulum was resected from the single left ventricle. A right Blalock-Taussig shunt was performed and the omphalocele repaired.This lesion, previously called thoracoabdominal ectopia cordis, was described in detail by Cantrell in 1958 and henceforth referred to as the pentalogy or pentad of Cantrell. 1,2 The pentalogy of Cantrell consists of (a) a midline supraumbilical anterior abdominal wall defect, (b) a cleft of the lower sternum, (c) a crescentic shape anterior diaphragmatic defect, (d) a defect of diaphragmatic pericardium allowing pericardial±peritoneum communication, and

Figure 2. Anteroposterior radiographs of upper (A) and lower (B) extremities. Bowing (most marked in the lower extremities) and fractures are present.

Kushner DC, Herman TE, Cleveland RH, Kleinman RE, Goodsitt MM. Reduction of radiation exposure during gastrointestinal biopsy procedures in children. Invest Radio1 1988;23:211-215.ERORAL BIOPSY OF the gastrointestinal tract is an impor-P tant diagnostic procedure in children. The procedure requires patient preparation and tolerance, operator skill and radiation exposure. Many techniques and different types of biopsy equipment have been described, but all recommend the use of fluoroscopy to determine the position of the biopsy device within the intestinal lumen before the sample is obtained. '-6 Fluoroscopy is an excellent diagnostic tool for the evaluation of any dynamic process such as a biopsy procedure. However, fluoroscopy using conventional equipment requires relatively high levels of radiation Radiation exposure can be reduced by careful attention to radiation safety guidelines, particularly exposure time, and by the use of specialized This report documents the use of low dose scanning beam digital radiography (American Science and Engineering, Inc., Cambridge, Mass.) as a substitute for conventional fluoroscopy to perform the imaging required for successful gastrointestinal biopsy in children. This technique permits the performance of projection radiography at low radiation From the *Department of Radiology and the 7Children.s Service,