Cornea And Anterior Chamber Angle Evaluation Using Anterior Segment Optical Coherence Tomography In Pediatric Patients With Wilson Disease

To evaluate the anterior segment optical coherence tomography (AS-OCT)  as an objective tool in Kayser-Fleischer (KF) ring detection and compare its results to those obtatained ftom slit lamp biomicroscopic examination in pediatric patients at the early stages of Wilson Disease (WD). In addition to the imaging of the anterior chamber angle (ACA) to assess the risk of developing glaucoma in children with WD.

Patients were recruited from the pediatric hospital at Cairo University according to the Wilson diagnosis scoring system (Leipzig score), followed by the Cairo University Pediatric Hospital, “Hepatology Department”.

The patients were enrolled in the study during their outpatient clinic visits

Forty patients diagnosed with WD according to the Leipzig score were enrolled in this study. Detailed medical history was taken, and a complete ophthalmic examination was performed in each participant, consisting of visual acuity testing, slit lamp examination to look for a KF ring in either eye, IOP measurement, and a dilated fundus examination.

Bilateral AS-OCT using the Optovue-RTVue Spectral domain OCT (RTVue XR Avanti) (Optovue, California, USA) device and an add-on lens of the corneal adaptor module (CAM-L mode: 6.0–2.0mm) was done to assess the cornea for the existence of copper deposits at the level of Descemet membrane and the presence of copper in the angle of the anterior chamber.

Forty children diagnosed with WD aged from 7-17.5 years (mean of 13.1 years) were examined. The presence of KF ring was evaluated using both AS-OCT and slit-lamp examination. KF ring was detected using AS-OCT as hyperreflectivity seen at the level of the Descemet's membrane only in 14 patients (35%) with a negative Slit lamp examination, while in 7 patients (17.5%) KF ring was positive in both AS-OCT and Slit lamp. In 65% of the cases, both the AS-OCT and slit-lamp examinations were negative. Fifty-six percent of the recently diagnosed (less than 6 months) showed positive AS-OCT KF ring, while the slit-lamp couldn’t detect any. AS-OCT imaging of the angle showed an open ACA with no copper deposits detected at this stage of the disease.

The sensitivity of AS-OCT in detecting the KF ring was higher than the slit lamp biomicroscopic examination, especially in recently diagnosed patients. AS-OCT can detect KF rings in the very early stages of Wilsons Disease, so that diagnosis and treatment accuracy can be evaluated effectively. Hence, it's recommended to be done early, even if slit-lamp examination was negative.