Panretinal acute multifocal placoid pigment epitheliopathy: a novel posterior uveitis syndrome with HLA-A3 and HLA-C7 association.

BACKGROUND: The purpose of this study is to report a previously undescribed clinical entity resembling acute posterior multifocal placoid pigment epitheliopathy (APMPPE) but with an atypical, panretinal and diffuse presentation in young patients with an HLA-A3 and HLA-C7 association. We describe a cluster of three, young, healthy patients who experienced acute visual loss, aged 16 through 27 years exhibiting an unusual clinical entity over an 8-month period. FINDINGS: Our patients demonstrated a unique presentation with acute retinal lesions similar to APMPPE but had widespread presentation of multiple lesions in the peripheral retina. All three of our patients exhibited an acute loss of vision, two of them bilaterally. Ophthalmoscopy and fundus photography demonstrated a diffuse distribution of lesions located extensively throughout the retina. On fluorescein angiography, the lesions showed a characteristic 'blocking early and staining late' pattern similar to APMPPE. The average duration of activity was 6 weeks (range 4 to 8 weeks), and there were no recurrences and good visual prognosis. HLA-A3 and HLA-C7 was noted in 100% of the patients. Ocular coherence tomography during the acute stage in one patient demonstrated thickening at the RPE layer. CONCLUSIONS: To our knowledge, this cluster of young patients represents a previously undescribed clinical entity, with clinical features similar to APMPPE, relentless placoid chorioretinitis and ampiginous. Due to the diffuse distributions of the active lesions, the acute clinical course without recurrences, good visual prognosis, and HLA-A3/C7 association, we believe it to be distinct from these other white dot syndromes. All three patients experienced preceding viral-like prodrome which, when combined with major histocompatibility commonalities, may predispose these individuals to an immune response. We call this entity panretinal acute multifocal placoid pigment epitheliopathy or PAMPPE.

Radial optic neurotomy for central retinal vein occlusion: 117 consecutive cases.

PURPOSE: Venous occlusive disease is the second leading cause of permanent retinal vascular blindness. The anatomy of the optic disk including the cribriform plate and scleral ring may contribute to the development of retinal vasoocclusive diseases. Neurovascular compression within the confined space at this location (scleral outlet) may play a pathoetiologic role in central retinal vein occlusion (CRVO). We developed a surgical procedure (radial optic neurotomy [RON]) to open this space and relieve pressure on the central retinal vein. METHODS: Pars plana vitrectomy with RON was performed on 117 consecutive patients with CRVO and severe loss of vision (defined as 20/200 or worse). Patients were observed with serial fundus photography, fluorescein angiography, determination of Snellen visual acuity, and biomicroscopy for anterior segment neovascularization. RESULTS: There were no serious complications noted with this procedure. Anatomical and clinical improvement as determined by fundus examination, fundus photography, and fluorescein angiography was found in 95% of patients. Snellen visual acuity improved by an average of 2.5 lines (range, 1-12 lines) in 71% of patients. Two or more lines were gained in 53% of patients, and > or = 4 lines were gained in 25%. Anterior segment neovascularization was found in 6% of patients with CRVO. CONCLUSIONS: Surgical decompression of CRVO via RON is a technically feasible and safe procedure that was associated with anatomical resolution of CRVO in 95% patients and improved visual function in 71%.

Radial optic neurotomy with adjunctive intraocular triamcinolone for central retinal vein occlusion: 63 consecutive cases.

PURPOSE: Central retinal vein occlusion (CRVO) is a leading cause of permanent retinal vascular blindness. In a previous communication the authors reported the results of radial optic neurotomy (RON) in 117 consecutive patients with severe CRVO. Persistent cystoid macular edema (CME) and macular pigmentation (MP) were observed and correlated with worse macular function. Intraocular triamcinolone (IOK) has been used to treat patients with CME and CRVO. The authors performed RON with simultaneous, adjunctive IOK (RON/IOK) in patients with CRVO to ascertain any anatomic or visual benefit of this combined approach. METHODS: Pars plana vitrectomy and RON were performed on a case-by-case basis on 63 consecutive patients with CRVO and visual acuity of 20/200 or worse. At the end of the case, 4 mg of triamcinolone was injected into the vitreous cavity (RON/IOK). Patients were observed with serial fundus photographs, fluorescein angiography (FA), Snellen visual acuity (VA), intraocular pressures (IOP), and biomicroscopy for anterior segment neovascularization (ANV). Anatomic and visual outcomes were compared to a previous series of 117 patients with severe CRVO undergoing RON alone. RESULTS: Clinical improvement as determined by fundus examination, photography, and FA was noted in 93% of patients following RON/IOK. Snellen VA improved by an average of three lines (range one to seven) in 68% of all patients. Two or more lines were gained in 44% of patients and four or more lines were gained in 20% of patients. ANV developed in 7% of patients following RON/IOK. Persistent CME and MP were noted in 17% and 28% of patients, respectively. These outcomes were similar to patients undergoing RON alone without IOK. Elevated IOP was noted in 25% of patients and one patient developed endophthalmitis following RON/IOK. CONCLUSIONS: Surgical decompression of CRVO via RON/IOK is a technically feasible procedure. Clinical resolution of the CRVO and improved visual function noted in RON/IOK paralleled outcomes following RON alone. RON/IOK was associated with a higher incidence of elevated IOP and endophthalmitis.