Bilateral keratoconus, acute hydrops and unilateral corneal perforation due to Tourette syndrome

Abstract

A 27-year-old male with a repetitive behavior of eyeball pressing for six months presented with decreasing visual acuity for three months. Upon arrival his best corrected visual acuity was 0.2 on the right and 0.6 on the left eyes. Scheimpflug camera system demonstrated grade 4 and grade 2 keratoconus, respectively. Psychiatric consultation revealed Tourette syndrome and treatment was started. Despite psychiatric treatment, acute hydrops occurred in both eyes decreasing visual acuity to hand motions bilaterally. Left corneal perforation due to ongoing habit of eyeball pressure was experienced which led the loss of light perception and phtisis bulbi. Although Tourette syndrome is rare, it might cause bilateral acute onset keratoconus when the repetitive movements affect periocular region. Psychiatric treatment should be considered as early as possible in these patients in order to prevent unfavorable complications.

Keywords: Cornea; Eye; Hydrops; Keratoconus; Tourette syndrome.

Fig. 1

Pentacam images (axial/sagittal curvature (front), corneal thickness, elevation (back)) of the right (upper) and left (lower) eyes upon arrival.

Fig. 2

Two weeks after first examination acute hydrops in the right eye (A), clear cornea in the left eye (B). One month after arrival progression of keratoconus in the left eye as measured with Pentacam (C). The perforated cornea that healed with central scar formation in the left eye (D).