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Trevor R. Carmichael, Nicky D. Welsh, Sadeer B. Hannush; Spontaneous Corneal Perforation Associated With Late-onset Corneal Ectasia (LOCE) In African Patients. Invest. Ophthalmol. Vis. Sci. 2011;52(14):1105.
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© ARVO (1962-2015); The Authors (2016-present)
To describe a new corneal dystrophy/degeneration in Africans with ectasia onset often in 4th or 5th decade complicated by perforation or hydrops.
Case series presenting over 13 years (1997-2010).
Cases included had corneal perforation with linear corneal rupture in the mid-peripheral cornea or a band of mid-peripheral mostly inferior thinning or scarring with mild ectasia/astigmatism of recent onset suggesting the same condition. We excluded cases of typical pellucid marginal degeneration and non-linear perforations or corneal thinning not clinically similar. Included were 18 patients (36 eyes) of which 14 were female (78%). Mean age at presentation was 47.8 years (range 36-68 years). All were black Africans and 7 (39%) were born in Greater Johannesburg. Of the 18 patients, 7 had unilateral pathology and 12 (67%) had perforated corneas, 3 of which had bilateral perforations. In 10 patients with perforation 6 had perforated the side of dominant handedness and four the opposite side. Hydrops was present in 9 eyes with perforations and 5 without. In 11 of the 18 patients (61%) the perforation/hydrops was preceded by ectasia (new astigmatism). Of 18 eyes that could be tested for astigmatism before perforation/hydrops it was over 5D in 9 (50%). Symptoms were pain preceding perforation/hydrops (7 patients) or itching (6 patients) or misty vision. Possible predisposing factors to perforation were; eye rubbing/itching in 7 patients, topical steroids used by 2, 6 of 13 patients had reduced Schirmer tests, 1 was HIV positive, 1 positive rheumatoid factor and anti-nuclear factor. In 4 eyes measured central corneal thickness was reduced to 512, 508, 435 and 436µ. Corneal repair or graft was done in 4 eyes but the rest were managed with soft contact lens or pressure padding. Eventual vision in the 29 affected eyes was; 20/40 or better in 7 and 20/60 to 20/200 in 14 and worse than 20/200 in 8 eyes. All remained stable after the event without recurrence.
LOCE may be a variant of pellucid, more common in black Africans with thin corneas and have a propensity for hydrops and perforation with various predisposing factors.
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