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Imaging of Chronic Granulomatous Disease in Children¹

¹ From the Departments of Radiology (G.K., S.C.K., Y.S.) and Pathology (P.K.), University of Iowa College of Medicine, Iowa City. Recipient of a Certificate of Merit award for an education exhibit at the 2004 RSNA Annual Meeting. Received January 25, 2005; revision requested March 7 and received April 14; accepted April 19. All authors have no financial relationships to disclose. Supported by grant R43DK061079-01 from the National Institute of Mental Health; G.K. supported by grant K30HL04117-01A1 from the Iowa Scholars in Clinical Investigation Program. Address correspondence to G.K., Department of Radiology, University of Iowa Hospitals and Clinics, 200 Hawkins Dr, Iowa City, IA 52242 (e-mail: geetika-khanna{at}uiowa.edu).

Chronic granulomatous disease (CGD) is a rare immunodeficiency disorder. The inability of phagocytic cells to kill catalase-positive organisms, such as Staphylococcus and Aspergillus species, causes recurrent infections, persistent inflammation, and granuloma formation. The imaging findings in nine cases of CGD were studied. Recurrent pulmonary infection was the most common abnormality (seven cases). Its complications included pulmonary abscesses, bronchiectasis, mediastinal abscesses, osteomyelitis, sepsis, and brain abscesses. Suppurative cervical adenitis was the second most common abnormality (four cases) and was also the presenting abnormality in the youngest patient (aged 31 days). Abdominal manifestations included hepatosplenomegaly, recurrent hepatic and splenic abscesses, necrotic mesenteric adenopathy, and gastric outlet obstruction. Osteomyelitis occurred in two cases secondary to hematogenous spread or spread of contiguous infection from the lung. Persistent infections led to formation of chronic inflammatory masses and granulomas in five cases. With improvements in therapy, the prognosis of CGD patients has improved and the general consensus is that most patients will survive into adulthood. Hence, radiologists are more likely to encounter the complications of CGD and should familiarize themselves with the spectrum of imaging findings.

© RSNA, 2005

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