New findings in short rib syndrome.

This white infant, born at 37 weeks of gestation, had a large cranium, bilateral anophthalmia, a midline cleft lip and palate, hypoplastic chest with short ribs, slightly protuberant abdomen, short limbs, bilateral single transverse palmar creases, a single umbilical artery, normal female external genitalia, normal (46 XY) chromosomes, and radiographic findings suggesting a short-rib (polydactyly) syndrome type IV (Beemer-Langer). Autopsy showed pulmonary hypoplasia, bilateral renal cystic dysplasia, intrahepatic bile duct cysts with periportal fibrosis, pancreatic cysts, absent internal genitalia, an atrophic optic chiasm, absent optic nerves, a single left anterior cerebral artery, polymicrogyria, and fusion of the frontal lobes, preoptic region, mammillary bodies, and thalami.

Usual and unusual radiologic manifestations of acquired immunodeficiency syndrome (AIDS) and human immunodeficiency virus (HIV) infection in children.

AIDS is being recognized with ever-increasing frequency in children. This article includes a discussion of the common radiographic manifestations of AIDS as well as some rarely encountered problems. It is hoped that with heightened awareness of this spectrum of radiographic findings, radiologists may contribute to a prompt diagnosis of AIDS so that appropriate short-term therapy and counseling may be instituted.

CaxP and Ca/P in the parenteral feeding of preterm infants.

Preterm infants requiring prolonged intravenous feeding frequently develop pathologic fractures and rickets. Infants who receive large amounts of calcium have fewer fractures. This observation led us to determine the maximal amounts of calcium and phosphate that can be added to parenteral nutrition solutions without the precipitation of calcium phosphate and to determine the optimal ratio of calcium to phosphate in these solutions. Clinical observations and in vitro experiments indicate that the product of calcium x phosphate (CaxP) in the dextrose-amino acid solution should not exceed 75 square millimolar (square millimole per square liter) to prevent calcium phosphate precipitation in barium-impregnated silicone rubber catheters and should not exceed 100 square millimolar in solutions administered through peripheral veins. Seven intake and output studies were performed in preterm infants to determine the ratio of calcium to phosphate (Ca/P) in the total parenteral nutrition solutions that minimized urinary losses. A Ca/P ratio of 5.0 minimized the sum of the calcium plus phosphate losses in the urine. However, experience with long-term total parenteral nutrition in preterm infants, awareness of the acute and life-threatening effects of body phosphate depletion, and an unmeasured endogenous enteric calcium secretion all suggest that a Ca/P ratio of approximately 3.0 provides a safer compromise between the acute and serious complications of phosphate deficiency and the chronic problems of fractures and rickets due to calcium deficiency.

Neonatal fellowship. Neonatal pneumopericardium: successful treatment with nitrogen washout technique.

Pneumopericardium in the neonate is a rare event but is associated with significant morbidity and mortality. We describe a neonate with mild respiratory distress and cardiovascular compromise after delivery and resuscitation. A large isolated pneumopericardium was diagnosed. Treatment with an oxygen hood (FiO2 1.00) resulted in a complete resolution of the pneumopericardium within 12 hours.

Necrotizing enterocolitis in the unfed infant.

From 1974 to 1981, 139 infants with neonatal necrotizing enterocolitis (NEC) were treated at our institution. Fourteen of these infants had not been fed prior to development of NEC. The unfed infants who developed intestinal necrosis had lower birth weights, were less mature, and had lower Apgar scores. The incidence of respiratory distress syndrome (RDS) and perinatal asphyxia were significantly higher in the entire unfed group. Unfed infants generally had longstanding indwelling umbilical artery catheters. Pneumatosis intestinalis was not often seen in unfed infants, even in those who developed intestinal necrosis and perforation. Anatomic location of the disease as determined at surgery was sometimes atypical. The pathologic lesion present in the intestine of the unfed infant suggested a primarily, if not purely, ischemic etiology.

The masquerading effect of Paget's disease.

The record and roentgen findings in a patient with Paget's disease with coexisting multiple myeloma have been presented. Their rare or even unique association is recorded to refresh ones recollection that patients with Paget's disease may be host to a second disease, which if recognized, may be of inestimable profit to the medical care and comfort of the patient.

Mesenteric vascular gas secondary to ischemic bowel in transmesenteric hernia.

A transmesenteric herniation of bowel with subsequent bowel ischemia in a thirteen-year-old boy is reported. Radiographic findings were that of abdominal mass and branching radiolucencies secondary to gas within the mesenteric vasculature. The unusual distribution of vascular gas (mesenteric rather than portal) is thought to be secondary to obstruction of mesenteric veins by strangulation.

CT of small-bowel obstruction in children: sensitivity and specificity.

UNLABELLED: OBJECTIVE.:The aim of this study was to determine the sensitivity, specificity, and accuracy of CT in the diagnosis of small-bowel obstruction in children. MATERIALS AND METHODS: The CT scans of 30 children with surgically proven small-bowel obstruction, 22 children with ileus, and 29 children who served as controls were retrospectively reviewed by two of four interpreters who were unaware of the children's final diagnoses. Causes of obstruction in the patients included 19 adhesions, six cases of volvulus, five intussusceptions, four strictures, and two cases each of internal hernia and abscess. Eight obstructions had multiple causes. The CT scans were evaluated for the presence of small-bowel obstruction using a scale with five degrees of confidence. In cases of discrepancy of more than one level of certainty, a third interpreter was consulted. Criteria for small-bowel obstruction included a discrepancy in caliber between the proximal dilated and the more distal small bowels or generalized small-bowel dilatation (>2.5 cm) in the presence of a collapsed colon. An interpreter's rating that an obstruction was either present or probable was considered a positive finding; a rating indicating that the interpreter was not sure whether an obstruction was present or believed that an obstruction was not probable or saw normal anatomic structures was considered a negative finding for small-bowel obstruction. The cause and level of obstruction also were recorded. RESULTS: There were 26 true-positive (87%) and four false-negative (13%) interpretations for small-bowel obstruction. Among the interpretations of scans of patients with ileus, 68% were true-negative and 32% were false-positive interpretations for small-bowel obstruction. Among the control group, there were no false-positive readings. Sensitivity of CT was 87%, specificity was 86%, and accuracy was 86%. In the scans of children 2 years and younger, CT had a sensitivity of 100% and specificity of 0%. Of the patients with surgically confirmed levels of obstruction, the correct level of obstruction was described by both interpreters in 12 (86%) of 14 scans. The causes of obstruction were correctly identified in 14 (47%) of 30 scans. CONCLUSION: CT is both sensitive and specific for use in diagnosing small-bowel obstruction in children, especially in children older than 2 years.