CT and MRI of Hepatic Abscess in Patients with Chronic Granulomatous Disease

García-Eulate, Reyes; Hussain, Nadeem; Heller, Theo; Kleiner, David E.; Malech, Harry L.; Holland, Steven M.; Choyke, Peter L.

doi:10.2214/ajr.05.1386

Cited by 40 publications

(22 citation statements)

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“…6 Radiological findings of abscess may differ in CGD, as described recently. 7 Aggressive surgical management has been shown to be more effective than medical intervention alone for large abscesses, reducing the mortality from 27% to 6%. 8,9 With earlier diagnosis, IFN-␥ and antimicrobial prophylaxis, and aggressive surgical and medical management, mortality for patients with CGD has plummeted in just a few decades with many patients now surviving well into adulthood.…”

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confidence: 99%

Hepatic abnormalities in patients with chronic granulomatous disease

et al. 2007

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Chronic granulomatous disease (CGD) is a rare congenital disorder characterized by repeated bacterial and fungal infections. Aside from a high incidence of liver abscess, little is known about hepatic involvement in CGD. The aim of this study was to describe the spectrum of liver abnormalities seen in CGD. The charts of 194 patients with CGD followed at the NIH were reviewed, with a focus on liver abnormalities. Liver enzyme elevations occurred on at least one occasion in 73% of patients during a mean of 8.9 years of follow-up. ALT elevations were generally transient. Although transient alkaline phosphatase (ALP) elevations were also common, persistent ALP elevations lasting up to 17.6 years were seen in 25% of patients. Liver abscess occurred in 35% of patients. Drug-induced hepatotoxicity was documented in 15% of patients but likely occurred more frequently. Hepatomegaly was found in 34% and splenomegaly in 56% of patients. Liver histology showed granulomata in 75% and lobular hepatitis in 90% of specimens. Venopathy of the portal vein was common (80%) and associated with splenomegaly. Venopathy of the central vein was also common (63%) and was associated with the number of abscess episodes. Nodular regenerative hyperplasia (NRH) was seen in 9 patients, including 6 of 12 autopsy specimens. C hronic granulomatous disease (CGD) is a rare inherited disorder occurring in 1 in 250,000 individuals. 1 The underlying defect is a loss or inactivation of a component of the reduced NADPH complex, resulting in a defective oxygen metabolic burst with inadequate production of superoxide and peroxide and, perhaps most importantly, an inability of phagocytic cells to kill certain bacteria and fungi. 2 These defects predispose affected individuals to recurrent infectious complications and significantly reduced long-term survival. 3 Four principal CGD genotypes have been described, with important differences in phenotypic expression. Because the gp91 phox genotype is X-linked and accounts for 70% of patients in large cohort studies, CGD has a significant male predominance. 4 The other described genotypes, p22 phox , p47 phox , and p67 phox , are autosomal recessive. 4 In addition to being more common, X-linked CGD (gp91 phox ) portends a worse prognosis with a higher annual mortality compared with the other genotypes. 1 The reduced NADPH is composed of cell membrane (p22 phox , gp91 phox ) or cytoplasmic moieties (p47 phox , p67 phox ). 1 Although pulmonary infections predominate in CGD, with 79% of a 368-patient registry reporting a history of pneumonia, other sites are also commonly affected, including suppurative adenitis (53%), subcutaneous abscess From the

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Hepatic abnormalities in patients with chronic granulomatous disease

et al. 2007

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“…Histological features and absence of bacteria microscopically identifiable suggest an atypical slow-healing process in patients partially responsive to antibiotic therapy [8]. Absence of a large colliquative cavity precludes these lesions from treatment by percutaneous drainage [4]. …”

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confidence: 99%

“…Hepatic abscesses may present as solid or target-like lesion in patients with chronic granulomatous disease, an inherited immunodeficiency of childhood characterized by primary phagocyte defect [4]. These abscesses are recurrent and often multiple and present no or tiny liquefaction nucleus in early stages; the rim of these lesions strongly enhances on postcontrast imaging but the main solid component usually enhances less than the surrounding hepatic parenchyma [4, 11, 12].…”

Section: Discussionmentioning

confidence: 99%

“…These abscesses are recurrent and often multiple and present no or tiny liquefaction nucleus in early stages; the rim of these lesions strongly enhances on postcontrast imaging but the main solid component usually enhances less than the surrounding hepatic parenchyma [4, 11, 12]. …”

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confidence: 99%

“…Among solid infected lesions of the liver, usually due to pyogenic pathogens, Brucella or parasites [4–7], organizing hepatic abscesses show peculiar features on computed tomography (CT) and magnetic resonance imaging (MRI) scans, which may suggest the correct diagnosis [8]. …”

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confidence: 99%

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CT, MRI and DWI Features of a Solid Organizing Hepatic Abscess

Savastano

Pellizzer

Grazia

et al. 2014

Case Reports in Radiology

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Solid organizing hepatic abscess is a rare form of focal infection, which needs differentiation from benign and malignant solid masses. We report a case of a 30-year-old man with a solid organizing hepatic abscess, diagnosed by imaging and ex juvantibus criteria. CT and MRI findings are presented and role of DWI is outlined. Noninvasive diagnosis of a solid organizing hepatic abscess is possible in the appropriate clinical setting; percutaneous or surgical biopsy may be indicated in equivocal cases.

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Magnetic Resonance Imaging

Bergin¹,

Mitchell²

2015

Yamada' S Textbook of Gastroenterology

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CT and MRI of Hepatic Abscess in Patients with Chronic Granulomatous Disease

Cited by 40 publications

References 13 publications

Hepatic abnormalities in patients with chronic granulomatous disease

Hepatic abnormalities in patients with chronic granulomatous disease

CT, MRI and DWI Features of a Solid Organizing Hepatic Abscess

Magnetic Resonance Imaging

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