Abnormalities in numbers or function of phagocytes account for 20% of cases of PID. CGD is the most frequent of the phagocytic disorders, its prevalence being 1/220,000. In patients with CGD, the functional activity of NADPH oxidase is significantly diminished or completely absent, resulting in no production of superoxide derivatives. Affected patients have recurrent infections involving the lymph nodes, lungs, soft tissue, and liver, the causative organisms being catalase-positive bacteria and fungi such as Staphylococcus aureus, Klebsiella pneumoniae, Escherichia coli, Aspergillus species, and Candida species, which cannot be killed because of the absence of reactive oxygen [4].
The other clinical characteristic of CGD is granuloma formation associated with excessive inflammation; these can form in the gastrointestinal tract, lung, and genitourinary organs. Such granulomas are believed to form as a result of persistent activation of intracellular inflammatory signals by reactions to microorganisms, production of inflammatory cytokines because apoptotic calls are not eliminated, and persistence of activated inflammasomes attributable to abnormal autophagy [5,6,7,8].
Hepatic abscesses develop in 14–51% of patients with CGD [2, 9]. More than half of them are due to S. aureus; Serratia spp. and Nocardia spp. are also frequently isolated [10]. However, cultures of hepatic abscesses are often negative and blood cultures are rarely positive, resulting in resistance to antimicrobials [2].
There is a high rate of recurrence of hepatic abscesses in patients with CGD [9]. Hepatic dysfunction with portal venopathy and nodular regenerative hyperplasia (NRH) is reportedly strongly associated with liver abscesses and is a major predictor of mortality in patients with CGD [11]. The mechanism is presumed to be noncirrhotic portal hypertension caused by microvascular insults from repeated abscesses [11]. It is possible that hepatic regeneration after surgery causes venopathy and NRH [12].
Although there are a few published case reports and retrospective discussions of treatment of hepatic abscesses in patients with CGD, their management is not well-defined. Corticosteroids or long-term administration of antibiotics are reportedly effective forms of medical treatment [4]. Corticosteroids are considered effective against the granuloma formation that characterizes CGD. In the present case, however, because the presence of active infection was not ruled out, we avoided corticosteroids because they may have masked or favored dissemination of the pathogen. The patient’s inflammatory findings improved postoperatively; therefore, corticosteroids were unnecessary.
However, many cases are resistant to these approaches because of the difficulty in detecting pathogenic bacteria and characteristic granuloma formation, as mentioned previously. Consistent with this, in our case, blood cultures and culture of the resected specimen were negative. We believe that the failure in medical treatment was attributable to the hepatic abscess being aseptic.
To our knowledge, this is the first reported case of successful hepatectomy for hepatic abscess in a patient with CGD in Japan. Previous cases of CGD successfully treated by surgery were reported in the English language literature. Some researchers have recommended surgical treatment. Roback et al. in 1971 outlined treatment options gathered from their management of 6 hepatic abscesses in 25 patients with CGD [13]. They recommended partial hepatic lobectomy for peripheral abscesses and full and extensive debridement for central abscesses. Mouy et al. managed 14 hepatic abscesses in 48 patients with CGD over a 16-year period. Nine cases required operation; 2 were managed successfully with percutaneous drainage [14]. Lublin et al. reported the efficacy of surgery and percutaneous drainage in a retrospective study of 61 cases of hepatic abscess in patients with CGD [2]. These authors pointed out that pyogenic abscesses are generally filled with homogenous purulent material, whereas abscesses in patients with CGD are characteristically dense, septated masses with fibrous pseudocapsules containing thick, inspissated fluid. Because abscesses in patients with CGD can rarely be treated by percutaneous drainage, they recommended aggressive surgical management [2]. Chen et al. emphasized that primary hepatic resection is preferable to percutaneous drainage because it can be curative and is associated with shorter hospitalization [3].
However, the complication rate is higher in standard hepatic surgery because these patients are immunodeficient. The complication rate is reportedly 50–56%, complications including persistent hepatic abscess, wound dehiscence, pancreatitis, and pneumonia [2]. In patients who develop wound dehiscence, steroids are paradoxically effective because the slow wound healing is considered to be related to granuloma formation [2].
Based on the above findings, hepatectomy is considered appropriate for patients in whom medical treatment and percutaneous drainage have failed or are contraindicated, the abscess is surgically resectable, and a hepatic reserve can be ensured. Assessment of hepatic function is particularly important because transplantation may be necessary due to the progression of hepatic fibrosis or repeated bouts of inflammation.
Because peritonitis may occur by spillage of the purulent content during hepatectomy for liver abscesses, it is important to perform en bloc resection without perforation of the abscess. Although dissecting along the pseudocapsule plane effectively minimizes injury to major vessels, the most appropriate method must be determined by the location of the abscess. For deep abscesses, the liver can be split along its intersegmental planes to expose the pseudocapsule for enucleation. Preservation of the hepatic parenchyma is especially important in patients with CGD because of the high frequency of abscess recurrence. This minimizes the loss of hepatic parenchyma and leaves the vascular anatomy intact for future excisions. Additionally, minimal hepatic resection rather than major hepatectomy may be sufficient to control these abscesses while likely minimizing postoperative complications. Furthermore, adequate perioperative management is required.
In the present case, because the abscess was close to the root of Glisson’s sheath of S4a and S5, we performed S4a + 5 anatomical hepatic resection instead of limited nonanatomic resection to ensure that complete resection was achieved. Additionally, because it was necessary to resect the liver while exposing Glisson’s sheath from the first ramification to the anterior segment, we selected laparotomy instead of laparoscopic surgery in consideration of the complexity of the surgical procedure. We plan to apply a laparoscopic approach for peripheral small lesions in the future. In the present case, the abscess was completely resected by this procedure.
In our case, hepatectomy succeeded in controlling a hepatic abscess in a patient with CGD and enabling him to undergo BMT. It is important to control infection before subjecting patients with CGD to this radical treatment. Surgery should be carefully considered in patients whose abscesses prove resistant to medical treatment.
