Abstract
BackgroundChronic granulomatous disease (CGD), a rare inherited disorder, is characterized by impaired ability of phagocytic cells to kill certain bacteria and fungi. Although liver abscess is a common manifestation of CGD, its optimal management in these patients is unknown. Here, we present a case of successful hepatectomy for hepatic abscess in a patient with CGD.Case presentationAn adolescent patient with previously diagnosed CGD presented to the pediatrics department of our institution with fever. Blood tests showed high concentrations of inflammatory markers. A computed tomography (CT) scan showed a multilocular mass measuring 52 mm × 34 mm in hepatic segment 4 (S4). Blood cultures were negative. Despite administration of antibiotics and γ-globulin, his fever and high concentrations of inflammatory markers persisted and the mass did not change on CT scan images. Because the medications had proved ineffective and percutaneous drainage would have been difficult because of the honeycombing in the abscess, we performed hepatic S4a + S5 anatomic resection and cholecystectomy. Culture of the excised specimen was negative. The patient’s postoperative course was uneventful. On day 62, CT showed no abscess around the resection stump. On day 81, he was transferred to undergo bone marrow transplantation.ConclusionsSurgical treatment for hepatic abscess can be effective when medical treatment has failed.
Highlights
Chronic granulomatous disease (CGD), a rare inherited disorder, is characterized by impaired ability of phagocytic cells to kill certain bacteria and fungi
Five distinct genetic forms affect the components of nicotinamide adenine dinucleotide phosphate oxidase—gp91phox, p22phox, p47phox, p67phox, and p40phox—resulting in diminished phagocyte respiratory bursts [1]
Hepatic abscess is a common manifestation of this disease, its optimal management has not been clearly defined in published reports
Summary
Chronic granulomatous disease (CGD), a form of inherited primary immunodeficiency (PID), is characterized by the inability of phagocytes to produce reactive oxygen intermediates because of a defect in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Because previous studies have shown that cultures of hepatic abscesses in patients with CGD are often negative and percutaneous drains seem to be a focus of secondary liver infection, we did not perform a liver biopsy considering his immunologically deficient state [2, 4]. On day 20 after admission, a CT scan showed the mass had enlarged, measuring 83 mm × 59 mm; γ-globulin was administered and amphotericin B substituted for voriconazole His fever and high concentrations of inflammatory markers (CRP 15.52 mg/dL, PCT 0.78 ng/mL) persisted, and on day 28, the mass had not changed on CT imaging (Fig. 1b). On day 62, a CT scan showed no abscess around the resection stump, and on day 81, he was transferred to undergo bone marrow transplantation
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