Neuroblastoma presenting as central nervous system disease.

Neuroblastoma may be extremely difficult to recognize, particularly when the tumor presents as a primary central nervous system disease. Central nervous system involvement may be considered as primary intracerebral neuroblastoma, metastases to the cranium from an occult primary, primary intraorbital neuroblastoma originating in the ciliary ganglion, metastatic intraorbital neuroblastoma from an occult primary, primary intraspinal neuroblastoma originating in dorsal root ganglia, intraspinal metastatic disease, and distant effects such as myoclonic encephalopathy. Primary neuroblastoma within the ciliary ganglion and primary intraspinal neuroblastoma are extremely rare entities. Illustrative cases the demonstrate the broad spectrum of neurologic presentations are offered. The second known report of neuroblastoma in association with primary pulmonary hypoventilation (Ondine curse) is included.

Esophageal replacement with jejunum in children: an 18 to 33 year follow-up.

Since 1947 a total of 32 staged jejunal interpositions have been performed in children for total esophageal replacement. There have been no failures of the jejunum to reach the neck, no loss of graft, and no deaths. The first 16 of these 32 children have now reached adulthood and form the basis for this report on the late functional results of staged jejunal interposition. Among these 16 patients there occurred four cervical fistulas which healed without sequelae (25%), one cervical stricture which necessitated dilatation but not revision (6%), one early cervical revision for necrosis of the distal tip of the graft (6%), and no complications related to the distal anastomosis. Long-term follow-up (range 18 to 33 years; mean 27 years) was obtained in 100% (16/16) of patients. A barium swallow was obtained in 81% (13/16) at a mean of 25 years following initial reconstruction (range 14 to 33 years). The fact that all patients could eat a regular diet at normal speeds indicates satisfactory long-term function. Two patients (13%) reported mild cervical dysphagia. A barium swallow in one of these patients revealed no abnormalities. The other was found to have an apparent esophageal diverticulum which resulted because the jejunum was end-to-side to the esophagus. This was recently corrected 27 years after the initial reconstruction and represented the only late complication of the procedure. There were no late strictures or peptic ulcerations. Motility of the jejunal limb was normal in 12 of the 13 patients studied. These results demonstrate that staged jejunal interposition can be accomplished reliably and safely in children with excellent long-term functional results and a minimum of late complications. The jejunum should be considered as an alternative to colon and gastric tubes for total esophageal replacement in children.

Malignant facial tumors in children: radiologic evaluation. Stressing value of conventional and computerized tomography.

Twenty-five cases of malignant tumors of the face in children under 16 years of age were reviewed. In each case the presenting complaint was related to a mass of the face, without a known primary malignancy. Rhabdomyosarcoma was the most common malignancy, followed by equal numbers of histiocystic malignancies and malignant lymphomas. Chloroma and neuroblastoma were less common. Plain films were obtained on every patient and were always felt to be an appropriate part of the work-up. The plain films suggested or made the diagnosis of malignancy in 16 of the patients. The most helpful additional study was tomography. CT was the modality of choice for tumors originating in or extending into the orbit, and for intracranial extension. Xeroradiography best demonstrated a superficial soft tissue tumor. In evaluating facial malignancies, the number and sequence of examinations must be individualized according to the presentation.

Pulmonary alveolar proteinosis: an uncommon cause of chronic neonatal respiratory distress.

A full-term newborn infant had the onset of respiratory distress immediately after birth. She required supplemental oxygen from birth, and had pulmonary alveolar proteinosis (PAP) documented by open lung biopsies at 6 and at 12 weeks of age. Light and electron microscopy showed dense, PAS-positive granular, homogeneous material within the alveolar spaces, diagnostic of PAP. No Pneumocystic carinii organisms were demonstrated on silver staining, and bacterial and viral cultures were negative. The infant died of progressive respiratory insufficiency at 1 yr or age. Pulmonary alveolar proteinosis should considered a rare cause of chronic respiratory distress in the newborn infant.

Rickets as a complication of intravenous hyperalimentation in infants.

Fifteen newborn infants developed roentgenographic evidence of rickets while on long-term intravenous hyperalimentation. In each instance, the initial diagnosis of rickets was suggested on the chest roentgenogram, where characteristic cupped and frayed upper humeral metaphyses were noted; subsequent knee and wrist roentgenograms substantiated these findings. Factors which may have predisposed to the development of rickets include inadequate doses of vitamin D, prematurity and a rapid change in body weight during hyperalimentation therapy.

Pulmonary complications associated with the prune-belly syndrome.

Eight patients are presented who demonstrate many of the pulmonary complications seen in the prune-belly syndrome. The patients are divided into two major groups: Group I includes pulmonary hypoplasia; Group II includes lobar atelectasis and pneumonia. The etiology, pathogenesis, and radiographic features of these complications are discussed. Pulmonary complications become more important as renal dialysis and transplantation spare more of these patients from an early uremic death. Prompt recognition of the type and the extent of pulmonary disease in patients with the prune-belly syndrome may lead to increased survival.

Roentgenographic features of American Burkitt's lymphoma.

Studies of American Burkitt's lymphoma in 12 children showed a high incidence of abdominal involvement, including ileocecal masses with intussusception, retroperitoneal extension, and diffuse bowel involvement. Pleural effusions were common, and 2 patients had pulmonary nodules. Three patients had diffuse renal involvement. The mandibles and other bones were not affected in any of the 9 patients tested. The first 2 patients survived 0.5 to 8.5 months; the last 2 were treated much more aggressively and were still free of disease at the time this paper was written, 14 and 16 months after treatment, respectively. Hopefully, early recognition of the clinical and roentgenographic features of American Burkitt's lymphoma will contribute to improved survival.

Gallbladder disease in cystic fibrosis.

In 84 consecutive patients with cystic fibrosis, oral cholecystography was abnormal in 39 (46.4%). The incidence of abnormal cholecystograms increased with patient age. The 26 patients with a nonvisualized gallbladder following double-dosage oral cholecystography were evaluated with intravenous cholangiography; 19 (70.3%) of these were abnormal. Ten patients were found to have calculi, an incidence of 11.9%. Awareness of the high incidence of cholecystographic abnormalities and calculi should be helpful in the evaluation of patients with cystic fibrosis, particularly since symptoms of abdominal pain are frequent in such patients and may lead to radiographic investigations.

Clinical observations on the biliary system in cystic fibrosis.

The biliary tree of 66 patients with cystic fibrosis was examined by conventional roentgenographic methods. Forty-five per cent of the oral cholecystograms were judged abnormal by our criteria. A study was considered abnormal if there was no visualization or if there was visualization of a microgallbladder or structural abnormality including marginal irregularities, septate gallbladder or cholelithiasis. Intravenous cholangiography was used to further study the 22 patients who did not visualize on the oral study. Again, anatomic abnormalities were prevalent but six patients in this group had normal appearing gallbladders. Abdominal pain, a frequent symptom in cystic fibrosis, was not associated with roentgenographic abnormality. No correlation was seen between the external biliary tree abnormalities and multifocal biliary cirrhosis which was present in 40% of these patients. Further, no correlation was seen between serum gamma glutamyl transpeptidase levels and either of these lesions.