Authors: Derek Mai, Julie Morin, Allan Oryschak, Andrew Kulaga, Karim Punja

Author Disclosure Block: D. Mai: None. J. Morin: None. A. Oryschak: None. A. Kulaga: None. K. Punja: Membership on advisory boards or speakers’ bureaus; Alcon Canada Inc., Allergan Inc., Clarion Medical Technologies. Description of relationship(s); Consultant/Advisor.

Abstract Body:

Purpose: This study showcases the utility of intralesional peribulbar triamcinolone injection complemented by surgical debulking in the management of orofacial granulomatosis (OFG).
Study Design: Retrospective case studies
Methods: Orofacial granulomatosis (OFG) is becoming an increasingly recognized entity in the differential diagnosis of patients presenting with chronic idiopathic periorbital and facial soft tissue swelling. The periorbital edema and erythema can be so remarkable as to mimic severe preseptal cellulitis and can cause visually obstructing blepharoptosis. The etiology of this rare clinical entity remains unknown. The classic histopathology shows peri-lymphatic non-caseating granulomatous inflammation in a background of dermal edema. When presenting with two additional features of fissured tongue and facial nerve palsy, the triad is referred to as Melkersson-Rosenthal syndrome. When presenting with the monosymptom of perioral swelling, it has been called granulomatous chelitis. OFG remains often a diagnosis of exclusion after other more common and potentially sight threatening etiologies such as thyroid eye disease and infectious diseases have been ruled out, which may explain for the typical delay in the diagnosis and treatment of OFG. Furthermore, there is a dearth of literature on treatment modalities of OFG.
We present a single-provider, single-institution retrospective case series from 2011 to 2018 of 6 eyes (4 patients) with biopsy-confirmed OFG who were treated with peribulbar triamcinolone acetonide injections, and in some cases, augmentation with surgical debulking.
Results: The ages of patients range from 30 to 71, with 1 male and 3 females. No patient had the complete Melkersson-Rosenthal syndrome triad. One patient had fissured tongue, and one patient had fissured tongue with intraoral lesions and lip edema. Three of the cases had surgical debulking of the upper eyelid in addition to peribulbar steroid injections. The other three cases experienced significant improvement of the periorbital edema and erythema with steroid injections alone. The number of injections ranges from 1 to 8 injections of 40 to 80mg of triamcinolone acetonide, with a frequency of injections ranging from once to every 2 to 6 months depending on clinical response. Patients were followed for a minimum of 6 months up to 24 months, and all cases experienced remarkable clinical improvement. None of the cases required concurrent long-term systemic immunosuppression.
Conclusions: Our study highlights the utility of intralesional peribulbar triamcinolone injection, and in some cases augmentation with surgical debulking, in the management of OFG.