A novel histopathologic finding in the Descemet's membrane of a patient with Peters Anomaly: a case-report and literature review

Peters anomaly is a rare developmental abnormality of the anterior segment of the eye and is one of the main causes of congenital corneal opacities. Typically, histopathology of Peters anomaly shows immature or absent Descemet’s membrane and attenuated endothelial cells in the area of the corneal opacity, in addition to thinning or absence of Bowman’s membrane and defects in the posterior stroma. In this report, we present a novel histopathological finding, which has not been previously reported, in the Descemet's membrane of a patient who is clinically diagnosed with Peters anomaly.

A 7-years old female child with developmentally delayed was born of a normal pregnancy, labor, and delivery. Apparent bilateral corneal opacifications were present at birth. On ophthalmologic examination, the child had a visual acuity of FC/20 cm in the right eye and that of FC/10 cm in the left one. Horizontal nystagmus and congenital cataract were found in both eyes. Slit-lamp examination revealed bilateral central corneal opacities which covered the iris and pupils. High-frequency UBM and AS-OCT both showed a shallow anterior chamber with multiple areas of iridocorneal adhesions and no corneal lenticular touch in each eye. A corneal specialist performed a penetrating keratoplasty with extra-capsular cataract extraction and intraocular lens implantation. Histopathologic procedures were conducted on the host corneal button, including Hematoxylin-Eosin stain and Periodic Acid-Schiff stain. All the sections were examined by light microscopy.

The “multiple-layer” structure of the Descemet’s membrane described in our case has not been reported before as in association with abnormalities of the cornea tissues in Peters anomaly. Such pathological finding need to be reported to enhance further understanding of the special structure of Descemet's membrane as an abnormality during embryogenesis and neural crest cell differentiations.