A 7-year-old girl was admitted with symptoms of abdominal pain, frequency, dysuria, and urgency. A urine culture showed a colony count of 100,000 Escherichia coli per high-power field. She had a history of three urinary tract infections within the previous two-year period. Each time, she had pyuria and was treated with sulfisoxazole for two weeks. Follow-up urinalyses had normal results.On physical examination, she was found to be a well-developed, wel l \x=req-\ nourished, normal-appearing 7-year\x=req-\ old girl. She had a well-healed thoracotomy scar secondary to repair of a coarctation of the aorta at 1 month of age. There was a questionably palpable pelvic mass. Laboratory data from admission studies included a normal urinalysis and no pathogens on urine culture.An initial cystogram, an excretory urogram, and a second cystogram were obtained (Fig 1, 2, and 3).
We report an unusual form of chondrodystrophy identified in a female infant at birth. She was severely delayed developmentally and expired at one year of age. The dwarfism was characterized by brachymelia, dysmorphic features and unusual radiologic findings of the extremities and thorax. The spine, hand and foot X-rays were normal. The clinical and radiologic manifestations are unique and unrelated to other described entities.
Vertical retrosternal density paralleling the sternum, with haziness of a hemithorax on chest roentgenogram, often challenges the clinical acumen of pediatricians, chest physicians, and pediatric radiologists alike. Its presence has been historically linked to either pulmonary underdevelopment and/or accessory hemidiaphragm. The surgical and radiological pediatric literature has long debated this topic, but unfortunately, it has not received much attention in the general pediatric or in pediatric pulmonology literature. The similar radiologic appearance of these two entities can cause confusion both in diagnostic and the therapeutic approach. Many authors advocate an invasive modality, including thoracotomy, as the only definitive way to differentiate between them. We present two similar cases with retrosternal densities, review the pertinent literature, and propose a rational approach to diagnosis and treatment.
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