Official ESCRS | European Society of Cataract & Refractive Surgeons

Keratoconus progression in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency or polycystic ovary syndrome

Session Details

Session Title: Special Cases
Session Date/Time: Friday 20/02/2015 | 08:30-10:30
Paper Time: 09:16
Venue: Sadirvan B
Co Author(s): :    S. Sever   R. Cosmin           

Abstract Details


To analyze the clinical association between the presence of congenital adrenal hyperplasia (CAH) due to a decreased synthesis of 21 hydroxylase as well as polycystic ovary syndrome (PCOS) in young females under oral contraceptive (OC) therapy and the appearance and development of corneal ectasia such as keratoconus.We also attempt to evaluate the outcome of these patients after adequate antiandrogenic therapy indicated for their systemic condition.


Randomized controlled trial developed at the Optilens Ophthalmology Clinic,Cluj-Napoca,Cluj,Romania


All 12 patients were reffered to our clinic between june 2010 and december 2014 due to decreased visual acuity in one or both eyes in the past few months. Clinical history, complete ophthalmologic examination, hormonal serologic profiles and corneal topography were performed in all patients. Genetic testing, adrenal CT scan were indicated in all CAH diagnosed patients.Ovarian ultrasound was indicated in PCOS patients and those under OC therapy. Keratometric, refractive, pachymetric, Belin-Ambrosio mapping and Holladay Diagnostic Summary variables were evaluated.


We followed the evolution of the corneal disease as well as the hormonal values before and during the antiandrogenic therapy period for four years in all patients. Corneal topography assesement was used to quantify the progression or arrest of keratoconus during these four years of therapy with normal estrogen, testosterone and other androgenic precursors levels.A stationary pattern was observed in 8 patients the other 4 expressing a rather progressive evolution therefore these patients undergone crosslinking therapy or corneal Intacts implantation.


Congenital adrenal hyperplasia and polycystic ovary syndrome can be associated with keratoconus. An abnormal steroidogenic pathway, affecting the normal development of the cornea, could induce stromal abnormalities that lead to corneal ectasia. Only one study presented the association between CAH and keratoconus in humans, while others confirmed the existence of an androgen dependent theory of developing keratoconus in mice clusters. Adequate antiandrogen therapy can stop the progression of corneal actasia in these patients.

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