Bilateral enophthalmos as a manifestation of HIV infection‐related lipoatrophy

Abstract

Infectious Diseases Unit, University Hospital de Valme,Carretera de Ca´diz, s/n 41014, Seville, SpainReceived: 6 February 2004, accepted 26 May 2004Lipodystrophy in HIV-infected patients can present in threedifferent forms: the pure atrophic form, the fat accumulationform and the mixed type [1]. The atrophic form ischaracterizedbyalossofsubcutaneousfatintheextremities,the buttocks and the face, mainly at the temporal areas andat the cheeks. We present herein a case of lipoatrophy whichled rapidly to an enophthalmos as a consequence of a loss oforbitary fat. To our knowledge, this is the first reported caseof enophthalmos as a manifestation of lipoatrophy in HIV-infected patients (on the basis of a MEDLINE search, 1985–2003, with keywords HIV, antiretroviral therapy, lipodystro-phy, lipoatrophy and enophthalmos).A 42-year-old man infected with HIV and hepatitis Cvirus (HCV), who had previously been an injecting druguser, started highly active antiretroviral therapy (HAART)in June 1998. Initially, he was given zidovudine (ZDV),lamivudine (3TC) and indinavir (IDV). In December 1998,IDV was replaced with nelfinavir (NFV) because ofrecurrent renal colics. In order to simplify the therapy,the regimen was switched to ZDV, 3TC and efavirenz (EFV)in November 2000. In October 2001, ZDVwas replaced withstavudine (d4T) because of persistent neutropenia. Thepatient had remained asymptomatic from the start oftherapy and reported taking more than 98% of theprescribed doses during the follow-up period. The plasmaHIV RNA load dropped below the detection level at 6months of follow up and in all determinations thereafter.The initial CD4 cell count was 50 cells/mL. Two years afterstarting HAART, the CD4 cell count had increased to 4350cells/mL and remained at this level during the remainder ofthe follow-up period. Treatment for chronic HCV infectionwith interferon (IFN) plus ribavirin (RBV) was givenbetween January and December 2002, with only a partialresponse. The patient’s weight at the beginning and end ofthe anti-HCV therapy was 72 and 70.9 kg, respectively.In January 2002, the patient showed painful unilateralgynecomastia. Plasma triglyceride levels, which werenormal at all previous visits, became elevated at this time.While the gynaecomastia decreased subsequently, until itdisappeared 1 year later, triglyceride levels remainedelevated. Until that moment of the follow up there wereno (even minor) signs of enophthalmos.In September 2002, modest facial lipoatrophy, with lossof Bichat’s fat, was present. There were no other signs oflipodystrophy. In March 2003, the patient complained ofmild ocular discomfort and recession of both eyes withinthe orbits. Physical examination revealed bilateral en-ophthalmos. The patient’s weight at that time was 74.7 kg.He did not report traumatisms. d4T was then replaced withtenofovir (TDF). An orbital computed tomography scanshowed bilateral orbital fatty tissue atrophy (Fig. 1).Neither facial lipoatrophy nor enophthalmos was objec-tively found to progress in the next 6 months. The patientdid not feel further ocular discomfort.Enophthalmos is a process in which the ocular globesinks into the orbit. It can have a variety of causes:structural bone alterations (orbital floor fractures), meta-static carcinoma, cicatricial orbitary lesions, chronicinflammatory orbital disease or orbital tissue atrophy.Bilateral enophthalmos in the absence of previous traumais rare. Experimental observations suggest that the loss ofintraconal fat generates enophthalmos [2]. The role oforbital fat changes in post-traumatic enophthalmos hasbeen extensively discussed [3], and orbital fat changes arealso assumed to play a role in nontraumatic enophthalmos.At least three cases have been reported in association withhydrocephalus and ventriculo-peritoneal shunting, withatrophy of the orbital fatty tissue as a related phenomenon[4]. In the patient reported here, enophtalmos was probablyalso a result of orbital fat atrophy. Thus, the emergence oforbital fat atrophy as a cardinal sign of lipoatrophy shouldbe taken into account in the diagnosis of enophthalmos inHIV-infected patients.The atrophic form of lipodystrophy has been reported tobe associated with exposure to d4T more frequently than