Sedation in pediatric CT: national survey of current practice.

To document current sedation practices in computed tomographic (CT) examination of children, a questionnaire was sent to a random sample of 2,000 hospitals with CT scanners in the United States. Responses were received from 834 hospitals (42%). Of these, 450 were reported to conduct pediatric CT with sedation. Approximately one-half of the examinations were conducted in pediatric hospitals or medical schools. Most hospitals did not require signed consent for CT with light sedation, even when intravenously or orally administered contrast medium was used. Signed consent for CT with deep sedation was required in 62% of hospitals. Monitoring techniques and personnel present during CT with sedation varied greatly, as did oral intake protocols for examinations with oral contrast material or no contrast material. Use of intubation during CT with oral contrast medium was rare. Orally administered chloral hydrate was the most frequently used first-line drug for sedation in most types of CT studies. The great variation in practices indicates a lack of settled standards for sedation during pediatric diagnostic examinations. Many procedures reported for pediatric CT with sedation are at variance with recommendations of the American Academy of Pediatrics.

Double aortic arch associated with esophageal atresia and tracheoesophageal fistula.

Esophageal atresia (EA) with tracheoesophageal fistula (TEF) is commonly associated with significant cardiovascular anomalies. Mortality is higher with cardiovascular abnormalities, especially those undiagnosed prior to surgical repair of the EA/TEF. Advances in cardiac imaging techniques, most notably echocardiography, have made noninvasive evaluation of the heart and great vessels easy to perform and reliable. Thus, the preoperative cardiovascular anatomy of these infants should be routinely assessed prior to surgical correction. A case of EA/TEF associated with an unsuspected double aortic arch is described. Although a wide variety of cardiovascular lesions have been associated with EA/TEF, coexistence of a double aortic arch has not been previously reported. Preoperative recognition of this anomaly would have altered the surgical approach, and perhaps reduced the postoperative morbidity and mortality.

Transthoracic Nissen fundoplication for gastroesophageal reflux in patients with severe kypho-roto-scoliosis.

Transthoracic Nissen fundoplication was used for the correction of gastroesophageal reflux in five mentally retarded patients with severe kypho-roto-scoliosis. This deformity may result in a gastroesophageal junction so high above the left costal margin that transabdominal fundoplication is extremely difficult. Operative morbidity was minimal. One wrap disruption occurred that required reoperation. Results have been satisfactory 6 to 24 months postoperatively.

Multifocal ganglioneuroblastoma coexistent with total colonic aganglionosis.

Neuroblastoma and Hirschsprung's disease are considered aberrations of neural crest cell growth, migration, or differentiation. Their coexistence, however, is rare. We present the case of an only child with total colon Hirschsprung's disease diagnosed shortly after birth, who was found to have noncontiguous ganglioneuroblastomas without metastases at age 16 months. The spectrum of neural crest anomalies, long segment Hirschsprung's disease and multifocal neuroblastoma, in this child is unique and previously unreported.

Intraperitoneal bladder rupture in a battered child.

Visceral manifestations of child abuse are less commonly diagnosed than are the characteristic skeletal injuries. We describe a child with intraperitoneal bladder rupture caused by blunt abdominal trauma. Intraperitoneal resorption of the extravasated urine led to laboratory evidence of renal failure.

Bone disease induced by anticonvulsant therapy and treatment with calcitriol (1,25-dihydroxyvitamin D3).

To evaluate the effects of calcitriol (1,25-dihydroxyvitamin D3) therapy for the bone disease induced by long-term treatment with anticonvulsants, we reviewed the medical records of 330 institutionalized oligophrenic children and young adults under 26 years of age to identify the 144 children who required anticonvulsant therapy. Of this latter group, 52 children were found to have serum alkaline phosphatase levels elevated more than 2 SDs above normal and were enrolled into this prospective three-year study. To achieve rapid resolution of the bone disease, we elected to use calcitriol at 0.25 to 0.75 micrograms/d. After 1195 patient-months of treatment, our data suggest that the dystrophic process was reversed in 42.3% of the cases, as judged by decreases in serum alkaline phosphatase levels at six months, 65.4% of cases at 12 months, and 83.3% of cases at 13 to 18 months. By 30 months of follow-up, all patients showed significant lowering of serum alkaline phosphatase levels. The improvements were slow and gradual. Twenty-six patients in the treatment series of 52 patients initially showed signs of rickets or osteomalacia on roentgenograms of the wrists. Of these 26 patients, 12 (46%) showed improvement on roentgenograms within 24 months of the beginning of treatment. With reference to complications, hypercalcemia (calcium level, greater than 11 mg/dL [2.74 mmol/L]) was encountered at the rate of one episode per 44 patient-months of treatment. Our results strongly suggest that calcitriol is effective in healing anticonvulsant-related osteomalacia among children and youths, with a low incidence of complications.

Ossification centers of the pediatric elbow: a rare normal variant.

A 7-year 9-month-old boy sustained a transcondylar humeral fracture during a fall. Radiographs revealed a trochlear ossification center without evidence of a medial epicondylar center. This combination, although apparently quite rare, should be recognized as a normal variant.

Gastrografin aspiration in a 3 3/4-year-old girl.

A case of Gastrografin aspiration in a 3 3/4-year-old child admitted for multiple trauma is described.

Chest radiographic findings in neonates on extracorporeal membrane oxygenation.

Eight full-term neonates with pulmonary failure were managed with extracorporeal membrane oxygenation (ECMO). Chest radiographs obtained before and during ECMO support were reviewed. During periods of increased flow requirements, the chest radiographs were difficult to evaluate because of increasing pulmonary opacity. During periods of the infants' clinical improvement, their chest radiographs tended to improve as well. A direct cause-and-effect relationship between the pulmonary density and the ECMO flow requirements has not yet been established. The chest radiographs were reliable in confirming correct catheter positions. Routine daily examinations did not demonstrate unsuspected abnormality. Chest radiography during periods of clinical instability, however, added confirmatory evidence to some clinical diagnoses and assisted us in making the diagnosis of an unexpected pneumothorax.

Nephrogenic diabetes insipidus. Radiological and clinical features.

Two brothers with nephrogenic diabetes insipidus are presented. The importance of early recognition and treatment to reverse the consequential complications are underscored. The disorder has a sex-linked recessive transmission, where all male offspring of an affected mother show the extreme thirst characteristic of the disease; in whom hyposthenuria persisted despite arginine vasopressin infusion, and in whom the serum arginine vasopressin concentrations correlate with the serum osmolality which serves to discriminate nephrogenic diabetes insipidus from the pituitary diabetes insipidus. The radiological features of "non-obstructive" hydronephrosis and hydroureters, the combined use of hydrochlorothiazide and the potassium-sparing diuretic amiloride in comparison to the prostaglandin inhibitor are discussed.

Calcium and phosphate metabolism in children with idiopathic hypoparathyroidism or pseudohypoparathyroidism: effects of 1,25-dihydroxyvitamin D3.

Two children with congenital hypoparathyroidism and two children with pseudohypoparathyroidism were given maintenance doses of 15 to 45 ng/kg/day 1,25-dihydroxyvitamin D3 for a total of 255 months. The urinary calcium excretion showed an upward elevation after the first 2 years of treatment but was not significantly higher than that in 10 normal control subjects. The renal threshold for phosphate excretion stayed within the normal ranges compared with control values. Two hypercalcemic and two hypocalcemic episodes occurred during this period of treatment. Hypercalcemia was reversed within 1 week after withdrawal of 1,25-dihydroxyvitamin D3. Hypocalcemia was countered by increasing the dose of 1,25-dihydroxyvitamin D3. Renal functions were not adversely affected, as estimated by creatinine clearance and reciprocals of serum creatinine concentrations. The mean serum calcium concentration during 1,25-dihydroxyvitamin D3 treatment was significantly higher (P = 0.001) compared with that obtained during vitamin D2 treatment at a dose of 500 to 3000 IU/kg/day. These data provide additional support for the long-term use of 1,25-dihydroxyvitamin D3 in idiopathic hypoparathyroidism and pseudohypoparathyroidism.

Abdominal pseudotumor caused by gastric displacement of the spleen: sonographic demonstration.

An infant with a factitious abdominal mass due to splenic displacement by the distended stomach is described. Two other cases of this phenomenon have been previously reported, both in infants and both diagnosed by radionuclide scans and plain radiographs. In our patient, diagnosis was made with ultrasound without the need for further studies.

An angiographic catheter technique for selective endobronchial intubation.

Three children requiring selective endobronchial intubation were treated with a technique utilizing an angiographic catheter as a guide for the endotracheal tube. The procedure was successful in all three children and there were no long-term complications. Performed properly the technique is rapid, safe and avoids uncontrolled attempts at endobronchial intubation or the need for a flexible fiberoptic bronchoscope.

Pulmonary arteriovenous malformations related to Rendu-Osler-Weber syndrome.

Interest in Rendu-Osler-Weber (ROW) syndrome has been renewed because of new treatment for the pulmonary artery fistulae that occur in approximately one-half of the patients. Pulmonary arteriovenous malformations (AVM) can be occluded safely by the transvenous placement of a silicone balloon thus avoiding the many potential complications of thoracotomy. Thirty-three members of four generations of a family with ROW illustrate the varied manifestations of the syndrome and provide a basis for review of clinical findings and therapeutic approaches to the management of pulmonary AVMs during the last 25 years. Special attention is given to early detection and prevention of major complications. Since ROW syndrome is an inherited trait, informed genetic counseling is an important component of the overall management of families with this disorder.

Ultrasonographic diagnosis of gangrenous bowel in neonatal necrotizing enterocolitis.

The identification of gangrenous bowel in neonates with enterocolitis is essential for proper timing of surgical intervention. The use of sonography to identify the presence of gangrenous bowel is illustrated.

Chronic granulomatous disease of childhood. Probable diffuse gastric involvement.

Diffuse gastric infiltration in a child with CGD is described as a cause for gastric outlet obstruction.