Sonography in newborns with cutaneous manifestations of spinal abnormalities.

High-resolution ultrasound is the screening method of choice for neonates with cutaneous signs of underlying spinal pathology. This examination does not require sedation, does not use radiation and is less expensive than computed tomography or magnetic resonance imaging. Ideally, sonograms should be obtained as soon as the cutaneous marker (sacral dimple, hairy patch, skin-covered mass) is found at the newborn examination.

Reconsideration of the anatomy of sling left pulmonary artery: the association of one form with bridging bronchus and imperforate anus. Anatomic and diagnostic aspects.

In sling (retrotracheal) left pulmonary artery (SLPA), the tracheobronchial pattern is generally considered basically normal. Analysis of dissected specimens and/or bronchograms and other preparations from five studied and 32 reported patients suggests that there are two different forms of SLPA: (1) types 1A and B, with normal TB pattern [with (A) or without (B) a right pre-eparterial (tracheal bronchus)], and the aberrant left pulmonary artery causing TB compression. The tracheal bifurcation in type 1 SLPA is usually demonstrable at the fourth to fifth thoracic vertebral level; (2A) SLPA type 2A, with bridging bronchus (BB), in which condition the right main bronchus supplies the right upper lobe, but the bronchus supplying the right middle and lower lobes (the bridging bronchus) arises from the left main bronchus (LMB), posterior to which the SLPA courses; (2B) SLPA type 2B, with absence of the right bronchial tree, and the right lung (usually hypoplastic) supplied by a BB from the LMB, posterior to which the SLPA courses. SLPA types 2A and B have in common varying degrees of tracheal stenosis with abnormal cartilage rings and absent tracheal pars membranacea, abnormally low tracheal "bifurcation" (pseudocarina) at average level T6, increased bronchial angles with "inverted T" pattern, and lower level of anterior esophageal indentation by the SLPA than SLPA type 1. Imperforate anus occurred in 8/58 (14%) of patients with SLPA types 2A or B, but possibly in none with SLPA type 1. SLPA type 2 is the predominant form of SLPA, with the incidence of type 2B being twice that of type 2A.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary lesions of bone in the 1st decade of life: retrospective survey of biopsy results.

Primary bone tumors in early childhood are uncommon, and the incidence of malignancy in the 1st decade of life has not been evaluated adequately in the literature. For this reason, we conducted a retrospective review of 268 biopsies of the bone in 249 patients who were less than 10 years of age. Benign tumors were found much more frequently than malignant lesions. Osteochondroma and histiocytosis X were the most common lesions. The incidence of several benign lesions in our series was deceptively low, as many lesions showed typical radiographic findings, precluding the need for biopsy or excision. Of 41 primary malignant bone tumors, Ewing sarcoma was slightly more common than osteosarcoma. The rib was the most frequent site of the former; the distal femur, of the latter.

Pulmonary alveolar septal calcinosis causing progressive respiratory failure in acute lymphoblastic leukemia in childhood.

A syndrome of pulmonary alveolar septal calcinosis, pneumothorax, and pneumomediastinum, leading to rapidly progressive acute respiratory insufficiency and death was observed in 2 children with acute lymphoblastic leukemia (ALL). Primary clinical and radiological considerations in these patients were pulmonary edema and infection, and the diagnosis of pulmonary alveolar septal calcification was established only at autopsy. One patient, a 15-year-old girl, was found also to have parathyroid hyperplasia typical of familial hyperparathyroidism. The other, a 16-month-old girl, showed osteitis fibrosa of the bones and parathyroid hyperplasia of secondary type, suggesting that the pulmonary calcinosis resulted from hypercalcemia caused by a parathormone or prostaglandin-secreting tumor. The cause of pneumothorax and pneumomediastinum may have been rupture of calcified alveolar septa induced by high PEEP during ventilation of these patients. Other possible mechanisms contributing to hypercalcemia and pulmonary calcinosis in children with acute leukemia include bone resorption due to marrow infiltration, immobilization syndrome, renal failure, and administration of calcium, phosphate, or bicarbonate. This complication of acute leukemia in childhood is rare (2 patients in 430 autopsied over the period 1961-1982 at Childrens Hospital of Los Angeles). How often the process can be reversed if diagnosed before severe respiratory insufficiency is present is not known.

Congenital syphilitic hepatitis: a radionuclide study.

A two-month-old girl with congenital syphilitic hepatitis had bizarre liver scintigraphic features showing diminished hepatic uptake of radiocolloid with accentuated pulmonary and bone marrow accumulation. These features were reversible following penicillin therapy and to our knowledge are previously undescribed manifestations of this multisystemic disease.

Radiographic features of fucosidosis.

Fucosidosis is an uncommonly recognized lysosomal storage disease which presents with psychomotor deterioration and progressing in some cases to death. The roentgenographic findings in five boys from three families are described. Skeletal abnormalities of a dysostosis multiplex nature are present and though not diagnostic should direct the clinician to perform appropriate biochemical and tissue assay studies to establish the diagnosis.

Cystic diseases of the kidney in children.

Many genetically determined cystic kidney disorders occur in children. Presenting manifestations related to the kidney include failure to thrive, abdominal enlargement or flank masses, progressive renal failure, or urinary tract infection. Radiological techniques often yield information helpful in establishing the type and extent of renal lesions. Cataloging the cystic renal disease in terms of genetic patterns, the involvement of liver or another organs, and the nature of the disorder of renal architecture is helpful in establishing the proper handling of affected children, and in genetic counseling.