Three infants died perinatally of asphyxia due to thoracic narrowing. Their uniform radiographic findings are different from previously described and recognized chondrodysplasias and possibly represent a distinct syndrome. Three identical or very similar cases are found in the literature.
Developmental patterns of sphenoidal sinuses were determined from lateral radiographs of 1702 patients from newborn to 15 years of age. Pneumatization of the conchal sinuses was seen as early as 6 months of age; 90% showed sphenoidal sinus pneumatization by 4 years of age; 100% by 10 years of age. Extension of pneumatization into the basisphenoid may be evident after 4 years of age, and 95% showed basisphenoidal pneumatization after age 12. An apparent lack of pneumatization of the sphenoidal sinuses in patients over 10 should stimulate investigation for sphenoidal sinus disease. Although isolated sphenoidal sinus abnormalities were rare, sphenoidal sinus involvement was common (67--75%).
Two boys presented with variable signs and symptoms of infectious disease that challenged diagnosis. One of the two patients had aortic valve vegetations and lower extremity aneurysms, and the other had calvarial osteomyelitis, epidural abscess, pleural effusions, and pulmonary nodules. Only after a battery of bacterial and fungal agglutination tests was the unsuspected diagnosis made in each of brucellosis from Brucella canis.
Radiographic, computed tomographic, and magnetic resonance imaging of LCH yield a variety of unusual manifestations. Recognition of these varied appearances of LCH may prevent confusion of such appearances with other pathologic processes. When the unusual manifestation occurs as the initial finding of the disease, LCH should be included in the differential diagnosis.
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