Dark Side Of The Moon – A Rare Case Of Bilateral Progressive Idiopathic Annular Lipid Keratopathy

Our purpose is to report a case of a patient referred to our Ophthalmology Department with idiopathic lipid keratopathy with symmetrical bilateral annular corneal lipid infiltration and describe examination findings performed at our center.

ULS São José, Ophthalmology Department, Lisbon, Portugal

Patient case report with past and present history, clinical findings and exams performed in our tertiary center, namely biomicroscopy, intraocular pressure measurement, fundoscopic examination, anterior segment photography, PENTACAM Tomography and Anterior segment swept-source optical coherence tomography (AS-OCT) ANTERION.

A 24-year old male presented at our tertiary center with complaints of a progressive whitish centripetal opacification of peripheral cornea, bilaterally. Best corrected visual acuity was of 20/20 on both eyes. Biomicroscopy and Anterior Segment Photography taken at the time of presentation revealed annular lipid infiltration in the medium and deep stroma, paracentrally and progressing in a crescent fashion to form a lipid infiltration ring in both eyes. The central cornea area of about 7mm showed no infiltration, thus sparring the visual axis and preserving visual acuity. Blood lipidogram was normal. PENTACAM Tomography was unremarkable. Anterior segment swept-source optical coherence tomography  depicted hyper-reflective stromal lesions.

Idiopathic lipid keratopathy is a challenging disease of unknown etiology, based on the presence of lipid deposits in the cornea without preceding vascularization or inflammatory process underneath, accompanied by absence of serum lipid elevation. Lipids might accumulate due to excessive lipid production or deficient lipid metabolization, in a process similar to arcus senilis. It is usually a bilateral condition, and due to its slow progression, visual acuity can remain good for years with very slow deterioration. However, in more advanced disease, vision impairment can occur and a penetrating keratoplasty might be necessary.