Acute necrotizing enterocolitis in infancy: a review of 64 cases.

Sixty-four cases of necrotizing enterocolitis are reviewed. The diagnosis was based on tissue examination in 57 and on the clinical syndrome, including pneumatosis, in 7. Three factors are important in the development of the disease: injury to the intestinal mucosa, bacteria, and feedings. The indications for surgical intervention are pneumoperitoneum, signs of peritonitis, and intestinal obstruction. The importance of stress in the etiology of the disease is confirmed by the high incidence of perinatal complications, particularly hypoxia. The mortality was high, but results are improving with the institution of early aggressive treatment.

Contemporary imaging approach to pediatric urologic problems.

This article has tried to do two things: review the more commonly used imaging modalities and apply them to the topics of obstruction and reflux. Increasingly, sonography is the initial diagnostic tool, obviously the only one usable in utero. The VCUG is the next most important method, especially in working out the often confusing sonographic images of the upper urinary tracts. Nuclear medicine remains drastically underused; it is the best way to detect parenchymal inflammatory disease and offers a method of examination and follow-up that requires low dosages and allows quantification of data. It will be interesting 10 years from now to review this subject and see what remains of the old and current methods and what will emerge.

Pediatric HIV infection in its second decade--the changing pattern of lung involvement. Clinical, plain film, and computed tomographic findings.

The changing pattern of pediatric HIV infection is illustrated in this article with plain films and computed tomographic scans. Today, vertical transmission from infected mothers results in HIV infection of exposed infants in about one third of pregnancies. Although the high mortality with catastrophic illnesses such as Pneumocystis carinii pneumonia seen in early life appears to be decreasing, chronic illness with pulmonary involvement due to diffuse lymphocytic infiltrative syndromes continues. Lymphocytic interstitial pneumonitis (LIP) has evolved in some patients to cystic lung disease and bronchiectasis. There are increasing numbers of patients infected with Mycobacterium. Masses seemingly of smooth muscle origin, thought to be leiomyosarcoma are appearing; they may be pseudotumors related to concomitant Mycobacterium avium intracellulare infection.

The radiologic and pathologic spectrum of the prune belly syndrome. The importance of urethral obstruction in prognosis.

The definition, history, and clinical presentation of the prune belly syndrome are outlined. Review of autopsy material and survivors reveal three groups of patients, and the author describes the choices of management available for both groups. Highlighted is the role of the radiologist.

Anatomic and functional abnormalities of the esophagus in infants surviving congenital diaphragmatic hernia.

As more infants with congenital diaphragmatic hernia (CDH) survive by means of extracorporeal membrane oxygenation (ECMO), new clinical observations are being made. We report anatomic and functional abnormalities of the esophagus in these infants. Twenty-two of 30 infants with CDH survived. Three of 7 non-ECMO-treated infants and 14 of 17 ECMO-treated infants had an air- or fluid-filled mediastinal mass that was confirmed by an upper gastrointestinal series as ectatic esophagus. Contrast studies demonstrated severe gastroesophageal reflux. Intra-esophageal pH monitoring confirmed significant reflux (Euler scores greater than 50) in 69% of tested patients. Prolonged acid clearance in most patients implied abnormal peristalsis. Hydramnios was correlated to the observed esophageal abnormalities, being present in 13 of 16 infants with an available amniotic history. All patients had symptomatic gastroesophageal reflux managed without antireflux surgery and were discharged 36 +/- 2 days after initiation of feeding. Follow-up (range: 7 to 56 months; mean: 32 months) showed that weight gain in affected children was slower than in those not affected. All but one are asymptomatic. Repeat upper gastrointestinal series shows persistent ectasia but less gastro-esophageal reflux. Gastroesophageal reflux/ectasia is a new observation with CDH. The associated hydramnios may result from proximal foregut obstruction by kinking the gastroesophageal junction with CDH. The radiographic appearance can be quite unusual. Clinical manifestations of gastro-esophageal reflux are manageable without surgery but may account for the observed compromised growth.

Fatal desquamative interstitial pneumonia in an infant: case report with transmission and scanning electron microscopical studies.

Desquamative interstitial pneumonia in a young infant followed a rapidly fatal course and developed morphologic features of honey-combing, giant cell pneumonia, and pulmonary alveolar proteinosis. The rapid and fatal progression of the disease in spite of steroid therapy and the polymorphous histologic appearance of the lungs at autopsy raise doubt that the disease is a relatively benign and uniform entity and invalidate the classical initial histologic features as prognostic indicators.

Inflammatory esophagogastric polyps: resolution following antireflux surgery.

Inflammatory gastroesophageal polyps are a rare manifestation of reflux esophagitis. In this report, an inflammatory gastroesophageal polyp resolved following antireflux surgery. The literature of inflammatory polyps is reviewed.

Diagnosis and management of gastrointestinal perforations in childhood dermatomyositis with particular reference to perforations of the duodenum.

Four children with dermatomyositis were recently seen with gastrointestinal perforations. The sites of perforation in the four cases were: (1) the duodenum, esophagus, and colon; (2) the duodenum; (3) the distal stomach; and (4) the traverse colon. The gastric and transverse colon perforations were intraperitoneal and easily diagnosed. The gastric perforation was treated successfully by partial gastrectomy. The patient with the colon perforation underwent exteriorization; death occurred from cerebral complications possibly related to vasculitis. Both duodenal perforations were posterior in the distal descending portion. Enzymatic dissection into the right lower quadrant produced confusing clinical and radiographic signs and extensive retroperitoneal necrosis. Successful treatment was obtained by partial gastrectomy, sump drainage of the perforation, and parenteral nutrition. Gastrointestinal perforation is a well-recognized complication of vasculitis in childhood dermatomyositis. In particular, perforations of the distal duodenum, as reported by others, are associated with delay in diagnosis and high mortality.

Neuroblastoma with intraspinal (dumbbell) extension.

Twenty-three patients with neurogenic tumors having extradural extension (dumbbell tumors) were treated over a 30-year period. Three had ganglioneuroma, 6 had ganglioneuroblastoma, and 14 had neuroblastoma. Nineteen patients with malignant tumors had symptoms of spinal-cord compression; three patients with ganglioneuroma and one with a malignant tumor had no neurologic symptoms. Delays in diagnosis were frequent ranging from 6 weeks to 3 years in 10 patients. Sixteen patients had abnormal x-rays of the spine. All patients with malignant tumors had positive myelograms. Three patients with ganglioneuroma and 13 with malignant tumors are alive and free of disease. Seven patients with malignant tumors died: five with stage IV and 1 with stage III tumors from disease, and 1 with stage II tumor during laminectomy. Age and stage were important prognostic factors; location was not. Treatment consisted of excision and radiation or radiation only. Patients receiving lower-dose (less than 2000 rad) radiation did as well as those receiving much larger doses. Morbidity in patients with malignant tumors was high; 4 have spinal deformity (3 severe) and 8 of 13 surviving patients have residual neurologic deficits. Earlier diagnosis, a surgical approach which does not introduce morbidity, and low-dose radiation in younger patients are important factors in reducing morbidity.

Urinary extravasation in the newborn male with posterior urethral valves.

Neonatal urinary extravasation is a rare condition frequently associated with posterior urethral valves in males. At the Babies Hospital, Columbia Presbyterian Medical Center during a 16-yr period, 5 neonates with urinary extravasation and valves presented--of all valve patients. Presentation was characterized by abdominal distention and varying degrees of azotemia. Treatment consisted of upper tract drainage in two cases and lower tract decompression in three. The mortality of this series was zero. At follow-up all five patients had completely normal serum renal function studies; four out of five had essentially normal radiographic studies. Extravasation appeared to protect the upper tracts from significant damage. Successful management of this condition should be tailored to the individual patient.

Plain film detection of right aortic arch in infants with esophageal atresia and tracheoesophageal fistula.

The aortic arch can be clearly demonstrated in neonatal chest radiographs by use of a high kilovoltage-filtered film with air-gap magnification. This allows identification of the presence of right aortic arch in patients with esophageal atresia and tracheoesophageal fistula; awareness of this anomaly may influence choice of surgical approach.

Therapeutic and diagnostic implications of acquired choledochal obstruction in infancy: spontaneous resolution in three infants.

Three infants aged 2 days to 11 weeks with conjugated hyperbilirubinenemia, had sonographically documented dilated common hepatic bile ducts, and echogenic material in the gallbladder. A 2-day-old infant, born to a diabetic mother, had none of the classic predisposing factors for cholelithiasis, and two infants had received total parenteral nutrition (TPN) and TPN plus furosemide. The first infant after receiving 4 1/2 weeks of TPN and furosemide, developed common duct obstruction with increasing bilirubin and hepatic duct caliber over a 12-day period. An operation was scheduled; however, on the following day the bilirubin dropped abruptly and surgery was canceled. The experience with this infant encouraged conservative management in two subsequent infants with similar clinical and sonographic findings. Spontaneous resolution occurred 9 days after the onset of common duct obstruction in one infant and after 16 days in the other.

Calcific thrombi of the inferior vena cava in infants and children.

Calcified caval thrombus should be considered in any infant or child where calcifications are noted in the high right retroperitoneal area on plain x-rays of the abdomen. Although typically bullet-shaped in configuration, the calcium distribution in the neonate may be atypical or incompletely developed, suggesting neuroblastoma. Definitive diagnosis can be made by inferior vena cavagram. As no deaths or complications have been attributed to the lesion in the cases thus far reported, no specific treatment is recommended.

Panner's disease: X-ray, MR imaging findings and review of the literature.

Panner's disease, avascular necrosis of the capitellum, was first described on X-ray examination of the elbow. It represents a major threat to the elbow joint integrity, and it is important to diagnose early. Panner's disease occurs most commonly in young children who play baseball, and in competitive gymnasts. Although considered a rare entity, it probably is underdiagnosed. We present the X-ray and MR imaging findings in a young boy with Panner's disease. The MR imaging findings in Panner's disease have not been previously reported.

Hypophosphatemic rickets in an adolescent cured by excision of a nonossifying fibroma.

An adolescent girl complaining of chronic heel pain was found to have acquired hypophosphatemic rickets and a nonossifying fibroma of the femur. The hypophosphatemic rickets was completely corrected by surgical excision of the bone lesion. This case represents another example of tumor-induced osteomalacia.