Spectrum of Retinal Vascular Diseases Associated with Paracentral Middle Maculopathy

Abstract

 

Purpose

 

Paracentral middle maculopathy (PAMM) is a newly described hyper-reflective, parafoveal band at the level of the inner nuclear layer (INL) on spectral domain optical coherence tomography (SD-OCT) that co-localizes with the intermediate (ICP) and deep capillary plexuses (DCP). This is an observational, retrospective, multi-centered case series to evaluate the spectrum of retinal diseases that demonstrate PAMM.

 

Methods

 

Nine patients (10 eyes) from 4 centers with PAMM lesions and associated retinal vascular diseases are included. Clinical presentations and multimodal imaging, including color photographs, near infrared reflectance, fluorescein angiography, SD-OCT, and color doppler imaging are described. Baseline and follow-up findings are correlated with demographics and systemic associations.

 

Results

 

PAMM lesions are verified by SD-OCT at baseline presentation in 5 men and 4 women (age 27 to 66 years). Follow-up SD-OCT analysis of these PAMM lesions demonstrated subsequent thinning of the INL. Novel systemic associations between retinal vasculopathy and PAMM include eye compression injury causing global ocular ischemia, sickle cell crisis, Purtscher’s retinopathy, occlusive retinal vasculitis, post-H1N1 vaccine, hypertensive retinopathy, migraine disorder, and post-upper respiratory infection.

 

Conclusions

 

PAMM lesions may develop in a wide spectrum of retinal vascular diseases. They are best identified with SD-OCT and may represent ischemia of the ICP and DCP. These lesions typically result in permanent thinning of the INL and are critical to identify in order to determine the cause of unexplained vision loss.  

 

Case 1: Prolonged globe compression leading to severe compromise in CRA and PCA blood flow as measured by color doppler imaging leading to PAMM. At baseline presentation, the flow for the CRA, NPCA, TPCA are severely reduced. Upon follow up, there was improved flow for each, except the NPCA OD, corresponding to slow reperfusion after relief of extended orbital pressure. OD: right; OS: left; CRA: central retinal artery; PCA: posterior cilary artery; NPCA: nasal PCA; TPCA: temporal PCA; OA: ophthalmic artery

 

 

Cases 2-9. F:female; M:male; OD:right; OS:left; OU:bilateral, CF:count fingers; BRAO:branch retinal artery occlusion; DVT:deep vein thrombosis, GI:gastrointestinal; OCP:oral contraceptive pills; HTN:hypertension; CHF:congestive heart failure; DM:diabetes mellitus; URI:upper respiratory infection