Localized ocular crystal-storing histiocytosis and associated lymphoma - Report of two cases and review of literature.

PURPOSE: To add to the existing yet limited body of knowledge around crystal-storing histiocytosis (CSH) with two case reports of localized ocular CSH and associated mucosa-associated lymphoid tissue (MALT) lymphoma involving the lacrimal and orbital soft tissues without underlying systemic lymphoproliferative disorders and to provide a literature review of all cases of CSH with associated ophthalmic findings reported to date. OBSERVATIONS: A 62-year-old male presented with a one-year history of right greater than left upper eyelid swelling and epiphora. Ophthalmic exam and computed tomography (CT) head scan revealed bilateral soft tissue masses superior to the globe encasing the supraorbital artery with poor margins from the superior rectus muscle. A biopsy of the lesion showed low grade B-cell lymphoma and associated CSH with lymphoma making up the bulk of the tumor and with CSH comprising a minor component of the overall tumor volume. Further investigations did not show any evidence of systemic lymphoproliferative disorders. He received local irradiation of orbits, which resulted in complete resolution of disease.An 85-year-old female with no significant past ocular history referred to ophthalmology services for an incidental finding of an enlarged left lacrimal gland on a CT head scan. Ophthalmic exam and subsequent magnetic resonance imaging (MRI) demonstrated an enlarged left lacrimal gland. A biopsy of the lesion showed MALT lymphoma associated with CSH. In this case, CSH comprised the bulk of the clinical mass rather than lymphoma. Following negative systemic investigations, she received a short course of localized radiotherapy with a 50% regression of disease seen on follow-up CT scan. CONCLUSION AND IMPORTANCE: These two cases demonstrate a spectrum of morphology associated with CSH. In addition, they show that although localized ocular CSH is rare, CSH should be considered in the differential of an orbital mass and should lead to consideration of further investigation for systemic lymphoproliferative disorders.

Periocular mass lesions secondary to dermatologic fillers: report of 3 cases.

OBJECTIVE: To describe findings in patients who received dermal fillers and later developed peri-ocular mass lesions. DESIGN: Retrospective case series. PARTICIPANTS: Patients who presented with peri-ocular masses secondary to dermal filler use. METHODS: Retrospective chart review. RESULTS: Three patients with remote filler injection (hyaluronic acid and polyalkylimide), not volunteered on initial history, presented with peri-orbital swelling and/or inflammation that was suspicious in each case for more serious pathology. CONCLUSIONS: It is important for the injecting physician, the ophthalmologist, and the patient to recognize this complication to permit appropriate investigation and management.

IgG4-related disease with hypergammaglobulinemic hyperviscosity and retinopathy.

Immunoglobulin G4-related disease (IgG4-RD) is a recently described entity with protean manifestations. We describe a novel case of IgG4-RD with hypergammaglobulinemic hyperviscosity responsive to fludarabine and rituximab. A 33-year-old Asian man developed bilateral lacrimal gland and submandibular salivary gland swelling with cervical lymphadenopathy. Biopsies of the affected tissues revealed reactive follicular hyperplasia. Seven years later, he presented with bilateral retinal hemorrhages due to hyperviscosity syndrome from profound polyclonal increase in IgG, including marked IgG4 elevation. Despite plasmapheresis, overproduction of IgG continued and he was refractory to systemic steroids, azathioprine, interferon alpha, and cyclophosphamide. IgG4-RD was suspected following a myocardial infarction and detection of aneurysmal coronary arteries indicating large vessel vasculitis. Review of the cervical lymph node and lacrimal gland biopsies with immunohistochemical staining for IgG4-positive plasma cells confirmed IgG4-RD. B-cell depletion with rituximab produced a partial response, but clinical symptoms and elevated protein levels persisted. Fludarabine was added to rituximab to suppress T-cell activity, and this resulted in an excellent clinical and biochemical response. Combination therapy with fludarabine and rituximab in IgG4-RD has not previously been reported and can be considered in patients with severe refractory disease.

A case of an enlarged medial rectus muscle.

We present a case of an enlarged medial rectus from mucosal associated lymphoid tissue (MALT) lymphoma that was misdiagnosed as thyroid orbitopathy for over 3 years. Extraocular muscles are most commonly associated with thyroid orbitopathy (TO), however, it is important to remember that they may also be manifestations of other conditions.

Eyelid fistula: a feature of occult sinus disease.

PURPOSE: To describe the clinical characteristics of patients presenting with eyelid fistula as a complication of occult sinus disease. METHODS: A retrospective review of patients presenting, to the Orbital Clinic at Moorfields Eye Hospital, with eyelid fistula due to chronic sinus disease; the review including clinical features, management and outcome. RESULTS: Eight patients presented with discharge from an upper lid or medial canthal fistula, of whom 6 had a history of variable lid swelling, 3 had ptosis and 2 had proptosis with restricted upgaze. All patients had ipsilateral frontal or ethmoid sinus opacification on CT, 5 had erosion of the bone and 2 had sinus mucocoeles. Management involved functional endoscopic sinus surgery to establish drainage of the affected sinuses and direct excision of the fistular tract. CONCLUSIONS: A discharging eyelid fistula may indicate occult sinus disease, which can remain undiagnosed for long periods. There is typically a history of recurrent upper lid erythema and swelling, with spontaneous drainage through a fistula sited in the supero-medial eyelid sulcus; local features include contracture and thickening of the skin and underlying tissues around the fistula.

Basal cell carcinoma of the caruncle.

An 82-year-old white man presented with a 2- to 3-month history of a lesion in his left medial canthal region. Surgical excision of the lesion was performed, and histopathology showed a nodular basal cell carcinoma of the caruncle. Because of incomplete surgical margins after the initial excision, a second excision was performed, followed by a course of adjuvant radiotherapy. No tumor recurrence was detected in the caruncle after 6 months of follow-up.