Roentgenographic Roentgenographic Features of the Neutrophil Dysfunction Syndromes

Abstract

The neutrophil dysfunction syndromes comprise a group of rare, often fatal disorders, usually beginning in infancy. Common to all these syndromes is an impairment of the ability of peripheral blood neutrophils (3, 14, 15, 17, 26) and monocytes (12) to kill ingested, opsonized bacteria. Although the syndromes bear diverse patterns of inheritance (14), their clinical manifestations are strikingly similar. A proposed classification by Douglas, Davis, and Fudenberg (14) includes three syndromes whose roentgenographic features are here presented (Table I). Recognition of a specific constellation of these features permits the roentgenologist to assume a key diagnostic role. The roentgenographic findings in the neutrophil dysfunction syndromes are characterized by their great diversity, reflecting involvement of multiple organs. The unusual chronicity of many of the changes may result from the persistence of viable intracellular bacteria within granulomatous lesions. The pulmonary findings include chronic and recurrent bronchial, segmental, and lobar pneumonia associated with enlarged hilar lymph nodes and pleural thickening. Pulmonary abscesses may develop, but unless they contain fluid levels their roentgenographic recognition may be impossible. Pulmonary consolidations occasionally assume an unusually discrete, spherical form (31). Calcified parenchymal pulmonary granulomas have been reported in one patient (6). In the abdomen, recurrent and sometimes chronic subdiaphragmatic and hepatic abscesses are common. Roentgenograms show elevation of the ipsilateral hemidiaphragm and sometimes an associated sympathetic pleural effusion. Telltale speckled calcifications within hepatic and splenic granulomas should be sought in roentgenograms of the abdomen (6, 25). Calcified granulomatous lymph nodes in the neck, abdomen, or elsewhere provide another diagnostic clue (6). Hepatosplenomegaly is usually demonstrated and may be striking. Hematogenous osteomyelitis, sometimes recurrent and often caused by organisms considered low-grade pathogens, is a common feature. The rarity of sequestra in infancy and childhood reflects spontaneous decompression and drainage in association with a massive turnover of pathologic bone.