Orbitofrontal Cholesterol Granuloma: Case Reports and A Systematic Review of the English Literature

Introduction: Primary orbitofrontal cholesterol granuloma (OFCG) is rare. We present 3 cases of OCG and a systematic literature review to examine patient characteristics, presentation, treatment, and outcome.

Methodology: Our institutional records were reviewed for OFCG cases. A systematic literature review was done using PubMed. Inclusion criteria were English-language studies with pathology-proven OFCG. Exclusion criteria were OFCG in a craniofacial sinus. The search-string yielded 172 results. Fifty-one studies met inclusion criteria (40 primary and 11 secondary) and relevant data was reviewed.

Results: In our series, there were 2 females and 1 male. They were 53, 43, and 34 years of age. Two were in the left and one was in the right lacrimal fossa. All three had proptosis on exam with inferior globe depression. All underwent a small, tailored craniotomy—two through a frontotemporal and one through a bicoronal incision—with complete curettage and lesion removal. There was no recurrence at 3 months, 10 year, and 22 year follow-up for each patient respectively. In the literature review, there were 172 patients with OFCG [mean age 42.3 (14–83) years; 154 (89.5%) male]. Fifty-five patients (32%) had a history of recent or remote trauma. Presentation symptoms included proptosis (n = 106, 61.6%), eye swelling (n = 62, 36.0%) and/or displacement (n = 8, 4.7%), diplopia (n = 53, 30.8%), vision changes (n = 21, 12.2%), and pain (n = 35, 20.3%). Mean symptom duration was 20.5 (0.25–312) months. The most common location was the right or left lacrimal fossa (n = 137, 79.7%). The most common treatment strategy was curettage through a sub-brow incision (n = 79, 44%) followed by a curvilinear scalp incision with/without craniotomy (n = 23, 12.9%). Thirty-seven patients (21.5%) had intracranial extension. There are two cases of extensive OFCG. One caused boney destruction of the lateral orbit and extended into the middle and anterior cranial fossa. Another led to a phthisical globe and extensive boney destruction and required orbital exoneration and reconstruction of the orbit with calvarial bone graft, temporalis muscle and a fascial flap. Postoperative follow-up data was available in 93 patients (54.1%) with a mean follow-up of 43.3 months (range, 2–204 months). Seven patients (4.1%) demonstrated recurrence at a mean of 65.0 (1–240) months following surgery, all after an orbital approach (p = 0.04 comparing orbital and scalp approaches for recurrence).

Conclusion: Orbital frontal cholesterol granuloma is a rare pathology that commonly presents with proptosis and diplopia. There are at least 172 reported cases of OFCG in the English-literature. We report three additional cases, for a total of 175 cases. Though expansion causing extensive boney destruction is rare, there are a few reported cases of OFCG becoming large and requiring extensive surgery. Therefore, we recommend removal of the lesions when symptomatic. Asymptomatic lesions can be observed, though the need for tissue diagnosis may lead to surgical treatment. Either primary orbital approaches or frontal flaps with or without craniotomy are acceptable, though recurrence was more likely after an orbital approach. Regardless of approach, complete surgical extirpation, including curettage and drilling of involved bone, should be the goal.