Immunoglobulin A vasculitis presenting as bilateral upper eyelid erythema: A case report and review of literature.

Immunoglobulin A vasculitis (IgAV) is the most common vasculitis of childhood characterized by petechial or purpuric rash, abdominal pain, arthralgia, and renal involvement. Ophthalmic manifestations of IgAV are uncommon. Herein, we describe a case of bilateral upper eyelid erythema presenting in a 6-year-old male, leading to a diagnosis of IgAV.

Preventing Retraction With Use of Primary Eyelid Spacer Grafts in Cosmetic Lower Lid Blepharoplasty.

BACKGROUND: Lower eyelid retraction is a challenging complication that can develop after a cosmetic lower eyelid blepharoplasty. Factors such as proptosis and negative orbital vector may place patients at a higher risk for developing postblepharoplasty retraction. Rather than addressing this complication after it occurs postoperatively, this study focuses on preventing its occurrence through the use of primary eyelid spacer grafts during the initial blepharoplasty. OBJECTIVES: The goal of this study was to review the outcomes of primary eyelid spacer graft placement during the initial cosmetic lower lid blepharoplasty. METHODS: A retrospective chart review was performed at Emory Eye Center between January 1, 2014, and January 1, 2022. Patients that underwent lower eyelid blepharoplasty with primary eyelid spacer graft placement were identified and included in the study. Fifteen patients with Hertel measurements of >17 and adequate preoperative and postoperative photographs were analyzed. RESULTS: Fifteen patients with exophthalmometry measurements of >17 and adequate preoperative and postoperative photographs were analyzed. The mean change in marginal reflex distance 2 was 0.19 mm (range, -1.05 to 1.24 mm). Two patients developed eyelid retraction at their long-term follow-up. Both patients developed retraction about 2 years after the initial surgery. CONCLUSIONS: Although this study was limited by its retrospective nature and had a small sample size, none of these high-risk patients developed immediate post blepharoplasty retraction. Careful preoperative evaluation should be performed to identify these high-risk patients, and placement of a primary eyelid spacer graft during the initial lower eyelid blepharoplasty should be considered in this population.

Occult Colon Adenocarcinoma and Multiple Myeloma Associated With Clostridium septicum Panophthalmitis With Orbital and Chiasmal Extension: A Case Report.

A previously healthy 62-year-old African American female presented with a fulminant orbital cellulitis of the right eye with diffuse scleritis and orbital inflammation extending to the optic chiasm on neuroimaging. She was taken for an emergent orbitotomy with an orbital fat biopsy and started on broad-spectrum intravenous (IV) and topical antibiotics. Within 36 hours of presentation, scleral thinning and a corneal melt ensued, ending in enucleation. Intraoperative cultures were positive for Clostridium septicum, leading to a systemic work-up exposing a previously undiagnosed colon adenocarcinoma and metastatic multiple myeloma.

Orbital osteomas associated with Gardner's syndrome: a case presentation and review of literature.

Gardner syndrome (GS) is a rare genetic disorder characterized by numerous intestinal colon polyps with various extraintestinal manifestations. Osteomas are a known extracolonic manifestation of GS and can affect the orbit, as seen in our patient, as well as 13 other cases documented in literature. Excision of large orbital osteomas can be successful with a multi-disciplinary approach as presented in this article. Ophthalmologists can even be the first to diagnose GS, usually via the presence of congenital hypertrophy of the retinal pigment epithelium (CHRPE) lesions. Untreated, 100% of colon polyps will transform into cancer, thus it is important to be aware of this rare syndrome with ophthalmic manifestations and screen patients with osteomas for GS.

Dilated Superior Ophthalmic Vein: Clinical and Radiographic Features of 113 Cases.

PURPOSE: Dilated superior ophthalmic vein (SOV) is an uncommon radiographic finding. The authors review the presentation, etiology, radiography, and visual implications of 113 patients with dilated SOV. METHODS: An observational case series and multicenter retrospective chart review were conducted. There were 113 patients with a dilated SOV. Outcome measures included patient demographics, clinical features, radiographic findings, diagnosis, and treatment, and treatment outcomes were assessed. RESULTS: Cases included 75 women (66%) and 38 men (34%) with a mean age of 49 ± 24 years (range, 0.4-90 years). Diagnoses fell under 6 categories: vascular malformation (n = 92, 81%), venous thrombosis (n = 11, 10%), inflammatory (n = 6, 5%), traumatic hemorrhage (n = 2, 2%), lymphoproliferative (n = 1, 1%), and infectious (n = 1, 1%). Imaging modalities utilized included MRI (n = 98, 87%), digital subtraction angiography (n = 77, 68%), CT (n = 29, 26%), and ultrasonography (n = 4, 4%). Disease status at last follow up included no evidence of disease (n = 57, 50%), alive with persistent disease (n = 53, 47%), and expired from disease (n = 3, 3%). Treatment and management was tailored to the underlying disease process with a mean follow up of 18 months (range, 1 day to 180 months). Visual impairment observed at presentation and last follow up across all cases was 26% and 22%, respectively. CONCLUSION: Dilated SOV is a rare radiographic finding resulting from a wide spectrum of etiologies with clinical implications ranging from benign to sight- and life-threatening. Dilated SOV is most often found with dural-cavernous fistula or carotid-cavernous fistula, orbital or facial arteriovenous malformation, and venous thrombosis. Recognition of this finding and management of the underlying condition is critical.

A 15-year review of secondary and tertiary optic nerve sheath fenestration for idiopathic intracranial hypertension.

PURPOSE: Optic nerve sheath fenestration (ONSF) is a common surgical option for patients with idiopathic intracranial hypertension (IIH) with vision loss refractory to medical management. Little is known about the visual benefit of repeated ONSF. The authors aimed to assess the efficacy of secondary and tertiary ONSF in patients with IIH. METHODS: A retrospective chart review was performed on all patients with repeat ONSF for IIH at Emory University from 1999 to 2016. Primary outcome measures included visual acuity, optic nerve head findings, and visual field results. RESULTS: A total of nine eyes in seven patients (five females and two males) with repeat ONSF were identified. Two of the seven patients had repeat ONSF in both eyes, while the remaining five patients had only one eye repeated. Five of seven patients (five eyes) improved or remained stable after the secondary ONSF. Two patients (three eyes) continued to worsen despite the secondary fenestration surgery and underwent tertiary ONSF at an average of 13.2 months (SD 5.5 months) after the failed secondary ONSF. Both patients that underwent the tertiary fenestration showed improvement. Six of the patients had either improvement or stability in their clinical findings at their last documented follow-up, but one continued to worsen despite intervention. CONCLUSIONS: This study suggests that secondary and tertiary nerve sheath fenestration is a viable management option for patients with progressive vision loss from IIH. Repeat ONSFs do not appear to have increased complication or failure rates compared to prior documented studies regarding primary fenestrations.

Review of periorbital nerve enlargement and biopsy techniques.

Periorbital nerve enlargement commonly indicates perineural invasion of malignancy or inflammatory conditions. This study reviews the role of supraorbital and infraorbital nerve biopsies in patients presenting with radiographic enlargement and to elucidate the surgical technique involved. A retrospective chart review (1997-2014) was performed at a single tertiary center. Patients with radiographic confirmation of enlarged supraorbital/infraorbital nerves that underwent biopsy were included. Charts were reviewed for: patient demographics and history, clinical symptoms and findings, radiographic findings, surgical method, and treatment. Five patients (4 female, 1 male) met inclusion criteria. Average age was 72.4 years (range 36-90). Four patients had history of cutaneous malignancy. All presented with diplopia and/or dysesthesias. Clinical examination confirmed decreased V1 and/or V2 sensation for 4 patients. Imaging revealed enlargement of V1, V2, and/or V3 in all patients. Infraorbital nerve biopsies were performed in 3 patients via transconjunctival fornix-based orbitotomy with subperiosteal dissection along orbital floor followed by unroofing of infraorbital canal. The remaining 2 underwent supraorbital nerve biopsy via sub-brow incision onto superior orbital rim with reflection of periosteum. Biopsies confirmed squamous cell carcinoma(3), mucoepidermoid carcinoma(1), and idiopathic orbital inflammation(1). Three patients initiated treatment in <1 month. One decided to follow-up closer to home, one was lost to follow-up. For patients presenting with enlarged supraorbital/infraorbital nerves, biopsy can rapidly confirm the underlying condition and facilitate early treatment. A sub-brow approach offers direct access to supraorbital nerve while transconjunctival fornix-based anterior orbitotomy with canal unroofing allows access to infraorbital nerve.

Periorbital ecthyma gangrenosum: a case report and review of the literature.

Ecthyma gangrenosum is a cutaneous manifestation of a Pseudomonas aeruginosa infection that is extremely rare in the periorbital region. The authors present a 48-year-old woman with a newly diagnosed acute promyelocytic leukemia with necrosis of her bilateral upper and lower eyelids and the lacrimal system. Following treatment with intravenous antibiotic and surgical debridement with delayed reconstruction, the patient had healed well.

A cyst-like foreign body reaction to porcine decellularized membrane (TarSys).

A 58-year-old Caucasian woman with thyroid eye disease underwent a bilateral lower eyelid blepharoplasty with porcine decellularized membrane (TarSys) eyelid spacer graft placement. Three months postoperatively, she developed unusual cyst-like masses in both lower eyelids that were excised. These were found to be consistent with inflammatory cysts with a foreign body reaction. No such reaction has ever been reported.

Chronic recurrent multifocal osteomyelitis causing optic neuropathy.

We present the case of an 8-year-old girl with chronic recurrent multifocal osteomyelitis (CRMO) involving the orbit and facial bone and causing left optic neuropathy. She presented with intermittent left periorbital swelling and a history of CRMO diagnosed 5 years earlier. Her disease responded well to adalimumab; however, delayed presentation resulted in permanent unilateral optic nerve atrophy and reduced vision. Orbital inflammatory disease is a rare manifestation of CRMO, and early recognition of the disease and treatment may prevent irreversible vision loss.