Corneal densitometry: a potential indicator for early diagnosis of Fabry disease
- PDF / 427,263 Bytes
- 8 Pages / 595.276 x 790.866 pts Page_size
- 43 Downloads / 191 Views
CORNEA
Corneal densitometry: a potential indicator for early diagnosis of Fabry disease Senmao Li 1 & Robert Siggel 2 & Yongwei Guo 1,3 & Niklas Loreck 1 & Alexander C. Rokohl 1 & Christine Kurschat 4,5 & Ludwig M. Heindl 1,6 Received: 15 July 2020 / Revised: 3 November 2020 / Accepted: 23 November 2020 # The Author(s) 2020
Abstract Purpose To assess corneal densitometry in patients with Fabry disease (FD) and to compare corneal densitometry differences in FD patients to different corneal manifestations. Methods Ten participants (20 eyes) with FD and 10 age-matched healthy volunteers (20 eyes) were recruited. All participants were assessed by standardized ophthalmic examinations and the corneal densitometry analysis by Pentacam HR. Densitometry measurements were analyzed in standardized grayscale units. Results Seven patients developed conjunctival vessel tortuosity, cornea verticillata appeared in 6 patients, and two patients had Fabry cataract. Retinal vessel tortuosity occurred in 4 patients, and dilation of retinal vessels appeared in 3 patients, all symptoms occurred in both eyes. The first diagnosis of FD up to examination was 4.7 ± 3.23 years, and first ERT up to examination was 2.6 ± 2.27 years. The initial time to diagnosis was negatively related to the corneal densitometry value of the 0–2-mm (r = − 0.556, p = 0.011) and 2–6-mm (r = − 0.482, p = 0.032) zones in the posterior layer. FD group have significantly higher corneal densitometry in anterior 0–2-mm zone and 2–10-mm zone anterior and posterior layer than the control group (p ≤ 0.035, respectively). When divided into two groups by the existence of cornea verticillata, there was a statistically significant difference in the anterior layer, 6–10-mm zone (p = 0.031); in the central layer, 0–2 mm (p = 0.012), 2–6 mm (p = 0.001), 6–10 mm (p = 0.002), and total (p = 0.002); and in the posterior layer, 6–10 mm (p = 0.004) and total (p = 0.002). Conclusions FD patients show higher corneal densitometry, and corneal densitometry may have potential for early diagnosis and reminding progress of FD.
Key messages Patients with cornea verticillata have higher corneal densitometry than normal. Patients with Fabry disease have higher corneal densitometry than normal. Fabry disease Patients with cornea verticillata have different corneal densitometry to those who have Fabry disease but without cornea verticillata. Corneal densitometry may have potential for early diagnosis and reminding progress of FD.
* Ludwig M. Heindl [email protected] 1
Department of Ophthalmology, Faculty of Medicine, University of Cologne, University Hospital Cologne, Kerpener Strasse 62, 50937 Cologne, Germany
2
Department of Ophthalmology, Helios University Hospital Wuppertal, Witten/Herdecke University, Wuppertal, Germany
3
Eye Center, School of Medicine, Second Affiliated Hospital, Zhejiang University, Hangzhou, China
4
Department II of Internal Medicine, Center for Molecular Medicine Cologne and Center for Rare Diseases Cologne, Faculty of Medicine, University of Cologne,
Data Loading...
