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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.

View larger version (41K): [in a new window]   Figure 1.  Activation of NADPH oxidase in a normal phagocyte. Phagocytosis of a microbe into a vacuole leads to activation of NADPH oxidase in the wall of the vacuole, generating oxygen radicals in the vacuole. Cytoplasmic granules also release enzymes into the vacuole, resulting in microbial killing.

 

View larger version (152K): [in a new window]   Figure 2a.  Pulmonary infection in an 18-year-old patient with CGD who presented with B cepacia sepsis. Chest radiograph (a) and CT scan (b) show interstitial thickening and miliary nodules in both lungs, an appearance consistent with hematogenous spread of infection.

 

View larger version (152K): [in a new window]   Figure 2b.  Pulmonary infection in an 18-year-old patient with CGD who presented with B cepacia sepsis. Chest radiograph (a) and CT scan (b) show interstitial thickening and miliary nodules in both lungs, an appearance consistent with hematogenous spread of infection.

 

View larger version (136K): [in a new window]   Figure 3a.  Pulmonary infection in an 8-year-old patient with CGD who presented with a fever and respiratory distress. (a) Frontal radiograph shows consolidation in the left lung with an air-fluid level in the left lower lobe (arrow), a finding suggestive of an abscess. (b) CT scan obtained 4 years later shows severe volume loss in the left lung and a honeycomb pattern in the parenchyma (arrow). Note the mosaic attenuation of the right lung with associated pleural thickening (*) and calcified hilar lymph nodes (arrowhead). (c) Lung section obtained at autopsy shows scarring in the lower lobe with hilar fibrosis (arrows) and consolidation (arrowhead).

 

View larger version (151K): [in a new window]   Figure 3b.  Pulmonary infection in an 8-year-old patient with CGD who presented with a fever and respiratory distress. (a) Frontal radiograph shows consolidation in the left lung with an air-fluid level in the left lower lobe (arrow), a finding suggestive of an abscess. (b) CT scan obtained 4 years later shows severe volume loss in the left lung and a honeycomb pattern in the parenchyma (arrow). Note the mosaic attenuation of the right lung with associated pleural thickening (*) and calcified hilar lymph nodes (arrowhead). (c) Lung section obtained at autopsy shows scarring in the lower lobe with hilar fibrosis (arrows) and consolidation (arrowhead).

 

View larger version (108K): [in a new window]   Figure 3c.  Pulmonary infection in an 8-year-old patient with CGD who presented with a fever and respiratory distress. (a) Frontal radiograph shows consolidation in the left lung with an air-fluid level in the left lower lobe (arrow), a finding suggestive of an abscess. (b) CT scan obtained 4 years later shows severe volume loss in the left lung and a honeycomb pattern in the parenchyma (arrow). Note the mosaic attenuation of the right lung with associated pleural thickening (*) and calcified hilar lymph nodes (arrowhead). (c) Lung section obtained at autopsy shows scarring in the lower lobe with hilar fibrosis (arrows) and consolidation (arrowhead).

 

View larger version (151K): [in a new window]   Figure 4a.  Pulmonary infection in a 20-year-old man with known CGD who presented with back pain. (a) Chest CT scan shows focal consolidation in the posterior lower lobe of the right lung (arrow). A specimen of the infiltrate obtained with CT-guided biopsy showed growth of Aspergillus. (b) Follow-up CT scan obtained 1 year later shows persistent pleural thickening (*) with associated periostitis of the adjacent rib (arrow), a finding suggestive of osteomyelitis. (c) Photomicrograph (original magnification, x40; hematoxylineosin stain) of a specimen from the rib shows osseous trabeculae with chronic and granulomatous inflammation replacing the marrow (arrows). (d) Photomicrograph (original magnification, x400; Grünwald-Masson stain) shows fungal hyphae within a granuloma (arrows).

 

View larger version (146K): [in a new window]   Figure 4b.  Pulmonary infection in a 20-year-old man with known CGD who presented with back pain. (a) Chest CT scan shows focal consolidation in the posterior lower lobe of the right lung (arrow). A specimen of the infiltrate obtained with CT-guided biopsy showed growth of Aspergillus. (b) Follow-up CT scan obtained 1 year later shows persistent pleural thickening (*) with associated periostitis of the adjacent rib (arrow), a finding suggestive of osteomyelitis. (c) Photomicrograph (original magnification, x40; hematoxylineosin stain) of a specimen from the rib shows osseous trabeculae with chronic and granulomatous inflammation replacing the marrow (arrows). (d) Photomicrograph (original magnification, x400; Grünwald-Masson stain) shows fungal hyphae within a granuloma (arrows).

 

View larger version (198K): [in a new window]   Figure 4c.  Pulmonary infection in a 20-year-old man with known CGD who presented with back pain. (a) Chest CT scan shows focal consolidation in the posterior lower lobe of the right lung (arrow). A specimen of the infiltrate obtained with CT-guided biopsy showed growth of Aspergillus. (b) Follow-up CT scan obtained 1 year later shows persistent pleural thickening (*) with associated periostitis of the adjacent rib (arrow), a finding suggestive of osteomyelitis. (c) Photomicrograph (original magnification, x40; hematoxylineosin stain) of a specimen from the rib shows osseous trabeculae with chronic and granulomatous inflammation replacing the marrow (arrows). (d) Photomicrograph (original magnification, x400; Grünwald-Masson stain) shows fungal hyphae within a granuloma (arrows).

 

View larger version (190K): [in a new window]   Figure 4d.  Pulmonary infection in a 20-year-old man with known CGD who presented with back pain. (a) Chest CT scan shows focal consolidation in the posterior lower lobe of the right lung (arrow). A specimen of the infiltrate obtained with CT-guided biopsy showed growth of Aspergillus. (b) Follow-up CT scan obtained 1 year later shows persistent pleural thickening (*) with associated periostitis of the adjacent rib (arrow), a finding suggestive of osteomyelitis. (c) Photomicrograph (original magnification, x40; hematoxylineosin stain) of a specimen from the rib shows osseous trabeculae with chronic and granulomatous inflammation replacing the marrow (arrows). (d) Photomicrograph (original magnification, x400; Grünwald-Masson stain) shows fungal hyphae within a granuloma (arrows).

 

View larger version (107K): [in a new window]   Figure 5a.  Lymph node involvement in a 10-year-old boy with known CGD who presented with swelling of the right side of the neck and a fever. (a) Contrast-enhanced CT scan shows a large abscess with enhancing septa in the right side of the neck (arrow). The abscess displaces the carotid space medially (arrowhead) and the sternocleido-mastoid muscle posteriorly (*). A specimen from the abscess showed growth of Haemophilus aphrophilus. (b) Follow-up CT scan obtained 2 years later shows persistence of the hypoattenuating fluid collection (arrow) deep to the sternocleidomastoid muscle despite aggressive management with drainage and antibiotics. Seven milliliters of pus was drained, which again showed growth of H aphrophilus.

 

View larger version (117K): [in a new window]   Figure 5b.  Lymph node involvement in a 10-year-old boy with known CGD who presented with swelling of the right side of the neck and a fever. (a) Contrast-enhanced CT scan shows a large abscess with enhancing septa in the right side of the neck (arrow). The abscess displaces the carotid space medially (arrowhead) and the sternocleido-mastoid muscle posteriorly (*). A specimen from the abscess showed growth of Haemophilus aphrophilus. (b) Follow-up CT scan obtained 2 years later shows persistence of the hypoattenuating fluid collection (arrow) deep to the sternocleidomastoid muscle despite aggressive management with drainage and antibiotics. Seven milliliters of pus was drained, which again showed growth of H aphrophilus.

 

View larger version (69K): [in a new window]   Figure 6a.  Lymph node involvement in a 6-week-old boy with a persistent fever and diffuse swelling of the neck. (a) US scan shows enlarged lymph nodes (arrows). (b) CT scan of the neck shows parapharyngeal (*) and posterior triangle (arrowhead) abscesses. Blood cultures were positive for Candida. (c) Photomicrograph (original magnification, x100; hematoxylineosin stain) of a lymph node shows effacement of normal lymph node architecture by a large necrotizing granuloma (arrows).

 

View larger version (128K): [in a new window]   Figure 6b.  Lymph node involvement in a 6-week-old boy with a persistent fever and diffuse swelling of the neck. (a) US scan shows enlarged lymph nodes (arrows). (b) CT scan of the neck shows parapharyngeal (*) and posterior triangle (arrowhead) abscesses. Blood cultures were positive for Candida. (c) Photomicrograph (original magnification, x100; hematoxylineosin stain) of a lymph node shows effacement of normal lymph node architecture by a large necrotizing granuloma (arrows).

 

View larger version (217K): [in a new window]   Figure 6c.  Lymph node involvement in a 6-week-old boy with a persistent fever and diffuse swelling of the neck. (a) US scan shows enlarged lymph nodes (arrows). (b) CT scan of the neck shows parapharyngeal (*) and posterior triangle (arrowhead) abscesses. Blood cultures were positive for Candida. (c) Photomicrograph (original magnification, x100; hematoxylineosin stain) of a lymph node shows effacement of normal lymph node architecture by a large necrotizing granuloma (arrows).

 

View larger version (123K): [in a new window]   Figure 7a.  Osteomyelitis in a 13-year-old boy with CGD who presented with a fever and right shoulder pain. (a) Magnified radiograph of the right side of the upper chest shows pleural thickening at the lung apex with widening of the second rib (arrow). (b) Technetium 99m–methylene diphosphonate bone scan (anterior view) shows increased uptake in the right second rib (thick arrow) and adjacent vertebral body (arrowhead), findings suggestive of osteomyelitis. Mild increased uptake is also noted in the right third rib (thin arrow). (c) CT scan shows a moth-eaten appearance of the right second rib (arrow) with associated involvement of the vertebral body (arrowhead), findings indicative of osteomyelitis. Underlying pleural thickening is also noted (*).

 

View larger version (104K): [in a new window]   Figure 7b.  Osteomyelitis in a 13-year-old boy with CGD who presented with a fever and right shoulder pain. (a) Magnified radiograph of the right side of the upper chest shows pleural thickening at the lung apex with widening of the second rib (arrow). (b) Technetium 99m–methylene diphosphonate bone scan (anterior view) shows increased uptake in the right second rib (thick arrow) and adjacent vertebral body (arrowhead), findings suggestive of osteomyelitis. Mild increased uptake is also noted in the right third rib (thin arrow). (c) CT scan shows a moth-eaten appearance of the right second rib (arrow) with associated involvement of the vertebral body (arrowhead), findings indicative of osteomyelitis. Underlying pleural thickening is also noted (*).

 

View larger version (99K): [in a new window]   Figure 7c.  Osteomyelitis in a 13-year-old boy with CGD who presented with a fever and right shoulder pain. (a) Magnified radiograph of the right side of the upper chest shows pleural thickening at the lung apex with widening of the second rib (arrow). (b) Technetium 99m–methylene diphosphonate bone scan (anterior view) shows increased uptake in the right second rib (thick arrow) and adjacent vertebral body (arrowhead), findings suggestive of osteomyelitis. Mild increased uptake is also noted in the right third rib (thin arrow). (c) CT scan shows a moth-eaten appearance of the right second rib (arrow) with associated involvement of the vertebral body (arrowhead), findings indicative of osteomyelitis. Underlying pleural thickening is also noted (*).

 

View larger version (43K): [in a new window]   Figure 8a.  Osteomyelitis in a previously healthy 32-month-old boy with a history of recurrent subcutaneous abscesses on his leg. (a) Bone scan shows increased uptake in the right calcaneus (arrow). (b) Plain radiograph shows a lytic area in the calcaneus with surrounding sclerosis (arrow), an appearance suggestive of osteomyelitis. Serratia marcescens was cultured from a biopsy specimen of the calcaneus.

 

View larger version (92K): [in a new window]   Figure 8b.  Osteomyelitis in a previously healthy 32-month-old boy with a history of recurrent subcutaneous abscesses on his leg. (a) Bone scan shows increased uptake in the right calcaneus (arrow). (b) Plain radiograph shows a lytic area in the calcaneus with surrounding sclerosis (arrow), an appearance suggestive of osteomyelitis. Serratia marcescens was cultured from a biopsy specimen of the calcaneus.

 

View larger version (72K): [in a new window]   Figure 9a.  Osteomyelitis in a 4-year-old patient with known CGD who presented with a limp. Anteroposterior (a) and lateral (b) radiographs of the lower leg show a lytic lesion with surrounding sclerosis in the tibial diaphysis (arrow). Osteomyelitis was proved at tibial biopsy.

 

View larger version (54K): [in a new window]   Figure 9b.  Osteomyelitis in a 4-year-old patient with known CGD who presented with a limp. Anteroposterior (a) and lateral (b) radiographs of the lower leg show a lytic lesion with surrounding sclerosis in the tibial diaphysis (arrow). Osteomyelitis was proved at tibial biopsy.

 

View larger version (177K): [in a new window]   Figure 10.  Gastric outlet obstruction in a symptomatic 9-year-old boy with CGD. US scan shows thickening of the antral wall (cursors), which causes narrowing of the lumen (arrow).

 

View larger version (127K): [in a new window]   Figure 11.  Gastric outlet obstruction in a symptomatic 9-year-old boy with CGD. Image from a barium study shows marked narrowing and elongation of the pyloric channel (arrow) with thickening of the gastric folds (arrowhead).

 

View larger version (130K): [in a new window]   Figure 12a.  Intraabdominal infection in a previously healthy 2-month-old child with a fever of unknown origin and a protuberant abdomen. (a) Contrast-enhanced CT scan shows multiple hypoattenuating mesenteric lymph nodes (arrows) that displace the bowel loops around them. Blood cultures were positive for Candida. (b, c) Follow-up abdominal radiograph (b) and CT scan (c) obtained at 5 years of age show multiple calcified mesenteric lymph nodes (arrows).

 

View larger version (144K): [in a new window]   Figure 12b.  Intraabdominal infection in a previously healthy 2-month-old child with a fever of unknown origin and a protuberant abdomen. (a) Contrast-enhanced CT scan shows multiple hypoattenuating mesenteric lymph nodes (arrows) that displace the bowel loops around them. Blood cultures were positive for Candida. (b, c) Follow-up abdominal radiograph (b) and CT scan (c) obtained at 5 years of age show multiple calcified mesenteric lymph nodes (arrows).

 

View larger version (128K): [in a new window]   Figure 12c.  Intraabdominal infection in a previously healthy 2-month-old child with a fever of unknown origin and a protuberant abdomen. (a) Contrast-enhanced CT scan shows multiple hypoattenuating mesenteric lymph nodes (arrows) that displace the bowel loops around them. Blood cultures were positive for Candida. (b, c) Follow-up abdominal radiograph (b) and CT scan (c) obtained at 5 years of age show multiple calcified mesenteric lymph nodes (arrows).

 

View larger version (162K): [in a new window]   Figure 13.  Splenic abscesses in a 6-week-old boy with sepsis. US scan of the spleen shows multiple hypoechoic lesions, findings suggestive of fungal abscesses. Blood cultures were positive for Candida; this result led to a diagnosis of CGD.

 

View larger version (125K): [in a new window]   Figure 14a.  Hepatic abscesses in a 10-year-old patient with CGD. (a, b) Contrast-enhanced CT scans (a obtained at a higher level than b) show multiple hypoattenuating, peripherally enhancing lesions located predominantly in the right hepatic lobe (arrow), findings consistent with abscesses. Culture of a specimen from an abscess was positive for S aureus. (c) Transverse US scan obtained 3 months later shows lack of flow in the right portal vein (cursors), a finding consistent with thrombosis. Resection of the right hepatic lobe was performed. (d) CT scan obtained at 18 years of age shows compensatory hypertrophy of the left hepatic lobe. Multiple recurrent abscesses are noted in the left lobe (arrows). Also note the thickening of the gastric wall, predominantly at the antrum (arrowhead). (e) Photomicrograph (original magnification, x40; hematoxylineosin stain) of the liver shows hepatic lobules (bottom part of image) with fairly abrupt fibrosis (arrows) and multiple large necrotizing granulomas (arrowheads).

 

View larger version (129K): [in a new window]   Figure 14b.  Hepatic abscesses in a 10-year-old patient with CGD. (a, b) Contrast-enhanced CT scans (a obtained at a higher level than b) show multiple hypoattenuating, peripherally enhancing lesions located predominantly in the right hepatic lobe (arrow), findings consistent with abscesses. Culture of a specimen from an abscess was positive for S aureus. (c) Transverse US scan obtained 3 months later shows lack of flow in the right portal vein (cursors), a finding consistent with thrombosis. Resection of the right hepatic lobe was performed. (d) CT scan obtained at 18 years of age shows compensatory hypertrophy of the left hepatic lobe. Multiple recurrent abscesses are noted in the left lobe (arrows). Also note the thickening of the gastric wall, predominantly at the antrum (arrowhead). (e) Photomicrograph (original magnification, x40; hematoxylineosin stain) of the liver shows hepatic lobules (bottom part of image) with fairly abrupt fibrosis (arrows) and multiple large necrotizing granulomas (arrowheads).

 

View larger version (78K): [in a new window]   Figure 14c.  Hepatic abscesses in a 10-year-old patient with CGD. (a, b) Contrast-enhanced CT scans (a obtained at a higher level than b) show multiple hypoattenuating, peripherally enhancing lesions located predominantly in the right hepatic lobe (arrow), findings consistent with abscesses. Culture of a specimen from an abscess was positive for S aureus. (c) Transverse US scan obtained 3 months later shows lack of flow in the right portal vein (cursors), a finding consistent with thrombosis. Resection of the right hepatic lobe was performed. (d) CT scan obtained at 18 years of age shows compensatory hypertrophy of the left hepatic lobe. Multiple recurrent abscesses are noted in the left lobe (arrows). Also note the thickening of the gastric wall, predominantly at the antrum (arrowhead). (e) Photomicrograph (original magnification, x40; hematoxylineosin stain) of the liver shows hepatic lobules (bottom part of image) with fairly abrupt fibrosis (arrows) and multiple large necrotizing granulomas (arrowheads).

 

View larger version (127K): [in a new window]   Figure 14d.  Hepatic abscesses in a 10-year-old patient with CGD. (a, b) Contrast-enhanced CT scans (a obtained at a higher level than b) show multiple hypoattenuating, peripherally enhancing lesions located predominantly in the right hepatic lobe (arrow), findings consistent with abscesses. Culture of a specimen from an abscess was positive for S aureus. (c) Transverse US scan obtained 3 months later shows lack of flow in the right portal vein (cursors), a finding consistent with thrombosis. Resection of the right hepatic lobe was performed. (d) CT scan obtained at 18 years of age shows compensatory hypertrophy of the left hepatic lobe. Multiple recurrent abscesses are noted in the left lobe (arrows). Also note the thickening of the gastric wall, predominantly at the antrum (arrowhead). (e) Photomicrograph (original magnification, x40; hematoxylineosin stain) of the liver shows hepatic lobules (bottom part of image) with fairly abrupt fibrosis (arrows) and multiple large necrotizing granulomas (arrowheads).

 

View larger version (221K): [in a new window]   Figure 14e.  Hepatic abscesses in a 10-year-old patient with CGD. (a, b) Contrast-enhanced CT scans (a obtained at a higher level than b) show multiple hypoattenuating, peripherally enhancing lesions located predominantly in the right hepatic lobe (arrow), findings consistent with abscesses. Culture of a specimen from an abscess was positive for S aureus. (c) Transverse US scan obtained 3 months later shows lack of flow in the right portal vein (cursors), a finding consistent with thrombosis. Resection of the right hepatic lobe was performed. (d) CT scan obtained at 18 years of age shows compensatory hypertrophy of the left hepatic lobe. Multiple recurrent abscesses are noted in the left lobe (arrows). Also note the thickening of the gastric wall, predominantly at the antrum (arrowhead). (e) Photomicrograph (original magnification, x40; hematoxylineosin stain) of the liver shows hepatic lobules (bottom part of image) with fairly abrupt fibrosis (arrows) and multiple large necrotizing granulomas (arrowheads).

 

View larger version (118K): [in a new window]   Figure 15a.  Brain abscesses in an 8-year-old patient with known CGD and a history of Aspergillus pneumonia who presented with diplopia and headaches. Axial contrast-enhanced T1-weighted MR images show multiple ring-enhancing lesions primarily located at the gray matter–white matter junction, findings suggestive of abscesses. Aspergillus was cultured from a biopsy specimen.

 

View larger version (137K): [in a new window]   Figure 15b.  Brain abscesses in an 8-year-old patient with known CGD and a history of Aspergillus pneumonia who presented with diplopia and headaches. Axial contrast-enhanced T1-weighted MR images show multiple ring-enhancing lesions primarily located at the gray matter–white matter junction, findings suggestive of abscesses. Aspergillus was cultured from a biopsy specimen.

 

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