Hepatic Abscess in Patients With Chronic Granulomatous Disease

Abstract

ObjectiveTo evaluate the clinical presentation, diagnostic procedures, and surgical management of hepatic abscesses in patients with chronic granulomatous disease (CGD). Summary Background DataChronic granulomatous disease is a rare inherited primary immunodeficiency in which phagocytes cannot destroy catalase-positive bacteria and fungi. Defects in the phagocytic cells' respiratory burst lead to life-threatening infections, including hepatic abscess. These abscesses are recurrent and often multiple and are treated differently from bacterial abscesses in patients without CGD. MethodsBetween 1980 and 2000, 61 cases of hepatic abscess in 22 patients with CGD were treated at the National Institutes of Health. Clinicopathologic features were investigated by retrospective review of the medical records, radiographs, and histopathology. ResultsTwelve of the 61 cases were primary hepatic abscesses. Twenty-nine of the cases were recurrent hepatic abscesses, and 20 cases were persistent hepatic abscesses. The median age at the time of initial hepatic abscess presentation was 14 years. Subjective fever was the most frequent presenting symptom, and the erythrocyte sedimentation rate was elevated in 98% of cases. Fifty-two cases were managed surgically and eight cases were managed with percutaneous drainage. One patient refused surgery. The surgical complication rate was 56%; however, there were no deaths directly related to the hepatic abscesses. Staphylococcus aureus was the most frequent organism identified in culture (88% of positive cultures). Aggressive surgery and antibiotics ultimately resulted in successful treatment of all patients.

Hepatic abnormalities in patients with chronic granulomatous disease

“…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. 9,10 Aside from the association with liver abscess, little is known about liver disease in CGD.…”

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

“…Whereas sporadic abscesses usually show central cystic cavities [4,13], the abscesses associated with CGD tend to remain homogeneously enhancing while small and then develop multiple locules separated by thick enhancing septations as they enlarge [12]. There is often an intense halo of enhancement around the abscess, likely representing granulation tissue.…”

“…Several attempts were made to drain these abscesses percutaneously early in our experience but these proved unsuccessful, and since then surgery has become the standard method of treatment [12]. As a result, these patients showed extensive postoperative changes (lobulated contour and scarring) during the follow-up period.…”

Modern management of chronic granulomatous disease

“…Larger liver abscesses (e.g. >5 cm) require surgical excision and drainage in addition to a 1-2 months course of antibiotic therapy, as liver abscesses in CGD are not simply an encapsulated collection of pus, but rather a semisolid, multiloculated mass of microabscesses and granulomas (Garcia-Eulate et al, 2006), cure by percutaneous drainage alone is rare and the relapse rate is high (Lublin et al, 2002). In a few cases, when surgery was contraindicated, several experimental approaches have been tried successfully: Intralesional granulocyte instillation (Lekstrom-Himes et al, 1994), percutaneous transhepatic alcoholization (Alberti et al, 2002) and percutaneous radiofrequency thermal ablation as used for treatment of liver cancer (Haemmerich & Wood, 2006).…”