C. Paya

Évaluation des propriétés biomécaniques de la cornée de patients glaucomateux et témoins par la technologie dynamique Scheimpflug de visualisation cornéenne

PURPOSE To compare corneal biomechanical properties measured with Corvis Scheimpflug technology (Corvis ST) between a group of patients with chronic open-angle glaucoma and a group of control patients. DESIGN Prospective observational case-control study. METHODS This study enrolled 56 right eyes of 56 patients (G1 [chronic open-angle glaucoma] n=37/G2 [control] n=19). Each patient underwent measurement of corneal biomechanical properties by dynamic Scheimpflug (Corvis ST) camera and the Ocular Response Analyser (ORA), then a measurement of intraocular pressure (IOP) by Goldmann applanation tonometry (GAT) and measurement of central corneal thickness (CCT) by optical coherence tomography during the same visit, by a single clinician. The parameters determined by Corvis ST are: Corvis IOP (IOP Corvis ST), the corneal deformation amplitude (CDA), corneal velocity, the time at highest concavity (TIME CONCAV), the lengths of applanation and their corresponding applanation time. Those studied by ORA are: compensated IOP (IOPcc), non-compensated IOL (IOPg), corneal hysteresis (CH) and corneal resistance factor (CRF). RESULTS IOP measured on all patients by Corvis ST was positively correlated to GAT (Spearman r=0.569, P<0.001) and PIOcc (Spearman r=0.531, P<0.001). After adjusting for age effect, CCT and GAT, the CDA was significantly lower in G1 than in G2, respectively 1.10 ± 0.12 mm and 1.15 ± 0.10mm (P<0.001). The TIME CONCAV is significantly shorter in G1 than in G2, respectively 16.88 ± 0.63 ms and 17.11 ± 0.29 ms, P=0.029. CH was significantly lower in G1 (G1: 9.58 ± 1.94 than G2: 10.89 ± 2.16, P=0.026). CONCLUSIONS This study showed differences in corneal biomechanical properties between glaucoma and control patients. The cornea of glaucoma patients appears less deformable.

Lessons of a day hospital: comprehensive assessment of patients with albinism in a European setting

Albinism is a rare genetic disease, comprising syndromic and non‐syndromic forms. We assessed clinical and genetic characteristics in a prospective evaluation of 64 patients (33 children and 31 adults) seen at a specialized day hospital. Causative genetic mutations were found in TYR (23/64, 35.9%), OCA2 (19/64, 29.7%), TYRP1 (1/64, 1.6%), SLC45A2 (12/64, 18.7%), C10orf11 (1/64, 1.6%), HPS1 (3/64, 4.7%), HPS5 (1/64, 1.5%), HPS6 (1/64, 1.6%) and GPR143 (2/64, 3.1%). Causative mutations remained undetermined for one patient (1.6%). Heterogeneity for hair and skin phenotype was noted across and within the different genotypes. Skin and hair hypopigmentation did not correlate with visual impairment. The diagnosis of unrecognized syndromic forms and of cases of ocular albinism in this prospective and comprehensive series of patients with albinism in a European setting is remarkable. Photoprotection was overall good but not optimal.

Orbital melanocytoma: Multimodal imaging and review of the literature

An 11-year-old female, without past medical history, was referred with a two years’ history of proptosis of her left eye and a superotemporal eyelid mass. Her best-corrected visual acuity was 20/20 and the clinical examination found a smooth tumor in front of the lacrimal area and leading to an eyeball infero-medial dystopia. Firstly, a Doppler-ultrasonography was performed and showed a vascular solid lesion. Secondly, a computed tomographic scan demonstrated a large solid hyperdense and well-circumscribed lesion in the right superior orbit, enhanced after intravenous injection of Iobitridol (Guerbet, France) causing a mass effect on the eyeball and repelling the orbital roof without osteolysis (Fig. 1A—B). Finally, a magnetic resonance imaging (MRI) was performed. The mass appeared hyper intense on T1-weighted images and iso intense on T2-weighted images (with few hypo intense areas) homogenously enhancement after gadolinium injection (gadoteric acid, Dotarem, Guerbet, France) and these results were confirmed by subtraction between T1-weighted images without and gadolinium injection (Fig. 1C—D). The preoperative diagnosis was intraorbital cavernoma. After multidisciplinary discussion, a surgical treatment with a complete excision was decided (Fig. 1E). There were no postoperative complications.

Spontaneous rupture of chorioretinal coloboma in an 8-year-old child is treated by temporal fascia graft

We report the rare case of an 8-year-old boy with spontaneous scleral perforation secondary to an isolated congenital chorioretinal coloboma. Visual acuity was 20/200 and examination revealed severe hypotony with subcapsular cataract, complete exudative retinal detachment, hypotonous optic nerve swelling, and hypotony retinal fold. In the temporal periphery, there was a chorioretinal coloboma with a central full-thickness defect. The scleral defect was successfully treated with an autologous temporalis fascia graft. One year later, and after cataract surgery, visual acuity had improved to 20/20, with normal intraocular pressure.