Primary Periocular Orofacial Granulomatosis: Case Series and Systematic Review.

PURPOSE: There is no known optimal treatment for primary periocular orofacial granulomatosis (PPOFG), a disorder that results in periocular edema. This case series and systematic review identifies management strategies and their reported improvement. METHODS: Systematic review and case series. PubMed and MEDLINE databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines to identify published cases of PPOFG. Cases were included when edema involved the periocular tissues and when a biopsy was interpreted to be PPOFG. Cases were excluded when edema did not involve the periocular tissues or when the edema was secondary to another process. The electronic medical records of the pathology department were studied to identify cases that were in keeping with PPOFG. The clinical charts were examined to confirm the diagnosis and provide a local case series. RESULTS: There are 38 published cases of PPOFG. An additional 9 cases were identified locally. These cases were combined and analyzed. Most PPOFG has eyelid swelling in isolation, without other facial swelling (36/47; 76.6%). It is most commonly a bilateral disease (30/47; 63.8%). Fissured tongue and facial nerve palsy occur, just as they do in other cases of orofacial granulomatosis. Treatment with surgical debulking or intralesional steroids resulted in high rates of symptomatic improvement of eyelid swelling, but recurrences were common. CONCLUSIONS: In light of no curative or highly successful treatment currently available, intralesional steroids and/or surgical debulking are therapies in the treatment of eyelid swelling associated with PPOFG that demonstrate reasonable short- and medium-term results. There is no established therapy that can offer disease remission or long-term symptom improvement.

Orofacial granulomatosis presenting as bilateral eyelid swelling.

Orofacial granulomatosis (OFG) is an uncommon but increasingly recognized disease of unknown etiology. The typical presentation is chronic swelling of the perioral soft tissue, but eyelid edema can be the sole manifestation. Terminology of this disease can be confusing as it may also be referred to as granulomatous cheilitis and a monosymptomatic presentation of Melkersson-Rosenthal syndrome (MRS). Crohn's disease and sarcoidosis should also be considered in the differential as the histopathology can be similar. Corticosteroids are the mainstay of treatment but can lack efficacy. Atypical presentations and the possibility of systemic disease involvement can further challenge the management. We describe an unusual case in which OFG manifests solely as chronic eyelid swelling. This 69-year old Asian female patient had a delayed diagnosis that responded well to intralesional corticosteroid injection with surgical skin reduction. In addition to describing this unusual presentation of OFG, we review the relevant literature and evaluate the current terminology used to describe this entity.

Facial nerve paralysis: an unrecognized cause of lower eyelid entropion in the pediatric population.

PURPOSE: To describe the association between entropion and pediatric facial nerve paralysis in the pediatric population. METHODS: A retrospective case series was collected from 5 pediatric patients who required surgical correction for symptomatic entropion with a history of facial nerve palsy. RESULTS: All 5 patients presented with epiphora and punctate keratopathy. Following surgical correction of the entropion, all ocular symptoms resolved. CONCLUSIONS: To the best of our knowledge, this is the first report linking facial nerve paralysis with entropion in the pediatric population. Ophthalmologists and pediatricians should consider this in the evaluation of children with craniofacial abnormalities and facial nerve paralysis.

Reconstruction of complex orbitocranial deformities using bioresorbable mesh, sterilized orbital models, and in situ contouring.

PURPOSE: To assess the efficacy of bioresorbable mesh in reconstruction of orbitocranial deformities. METHODS: A retrospective case series evaluating 6 orbits of 6 patients, ages 12 to 70 years old, with large multicontoured orbitocranial defects that were repaired using bioresorbable macroporous mesh. Both functional (presence of diplopia, ocular motility, exophthalmometry, and vertical globe position) and cosmetic outcomes were evaluated. RESULTS: Follow-up time ranged from 20 to 48 months (mean, 29.1 months). After surgery, all patients reported cosmetically pleasing results. Diplopia resolved in all patients. Ocular motility, exophthalmos, enophthalmos, and vertical globe positioning improved in all patients. One patient had new onset of mild enophthalmos and hypoglobus after surgery. No cases of implant exposure or instability occurred. Complications included one patient with skin breakdown over a previous site of irradiation and one patient with ptosis requiring surgical correction. CONCLUSIONS: Bioresorbable macroporous mesh combined with bone grafts is a useful and accurate method to reconstruct complex multicontoured orbitocranial defects.

Natural killer/T-cell lymphoma with ocular and adnexal involvement.

PURPOSE: To review the clinical, radiological, and histopathologic features in 8 patients with natural killer/T-cell lymphoma (NKTL) involving the orbit and/or ocular adnexa, and to describe the responses of these patients to various treatment regimens. DESIGN: Retrospective observational case series. PARTICIPANTS: Eight patients (5 male, 3 female) with NKTL involving the orbit and/or ocular adnexa were identified from 1999 through 2005. The mean age at presentation was 45 years (range, 26-65). METHODS: We retrospectively identified patients with NKTL of the ocular adnexa treated in the authors' medical centers from 1999 through 2004 using computerized diagnostic index retrieval. The clinical records and radiologic studies were analyzed to define modes of presentation and progression, response to therapy, and areas of anatomic involvement. Histopathologic findings, including the presence of CD3, CD56, and Epstein-Barr virus-encoded mRNA in each patient, were reviewed. MAIN OUTCOME MEASUREMENTS: Time of survival from presentation to last known follow-up and tumor-related death. RESULTS: Four of the 8 patients (50%) with NKTL involving the orbit or ocular adnexa had systemic involvement at presentation. Five of the 8 patients (62.5%) had concurrent sinonasal involvement, whereas 3 (37.5%) had orbital involvement alone. All lesions demonstrated CD3, CD56, and/or Epstein-Barr virus positivity on immunopathology studies. Therapy consisted of various chemotherapeutic regimens typically employed in the treatment of non-Hodgkins lymphoma, steroids, surgical intervention, and radiation. Seven (87.5%) patients died 5 weeks to 13 months after presentation, and 1 (12.5%) is alive without disease (5-year follow-up). CONCLUSIONS: Natural killer/T-cell orbital lymphoma is a rare Epstein-Barr virus-associated neoplasm that may occur with or without associated sinonasal involvement. Our series, the largest cohort reported to date, demonstrates the high lethality of this condition despite aggressive conventional therapy, suggesting that new treatment options should be considered early in the course of treatment of patients with this disorder.