Choroidal and peripapillary changes in high myopic eyes with Stickler syndrome

BMC Ophthalmol. 2021 Jan 4;21(1):2. doi: 10.1186/s12886-020-01777-3.


Background: To compare different clinical and Spectral-Domain Optical Coherence Tomography (SD-OCT) features of high myopic eyes with Stickler syndrome (STL) with matched controls.

Methods: Patients with genetically confirmed STL with axial length ≥ 26 mm and controls matched for axial length were included. The following data were obtained from SD-OCT scans and fundus photography: choroidal and retinal thickness (respectively, CT and RT), peripapillary atrophy area (PAA), presence of posterior staphyloma (PS).

Results: Twenty-six eyes of 17 patients with STL and 25 eyes of 19 controls were evaluated. Compared with controls, patients with STL showed a greater CT subfoveally, at 1000 μm from the fovea at both nasal and temporal location, and at 2000 and 3000 μm from the fovea in nasal location (respectively, 188.7±72.8 vs 126.0±88.7 μm, 172.5±77.7 vs 119.3±80.6 μm, 190.1±71.9 vs 134.9±79.7 μm, 141.3±56.0 vs 98.1±68.5 μm, and 110.9±51.0 vs 67.6±50.7 μm, always P< 0.05). Furthermore, patients with STL showed a lower prevalence of PS (11.5% vs 68%, P< 0.001) and a lower PAA (2.2±2.1 vs 5.4±5.8 mm2, P=0.03), compared with controls.

Conclusions: This study shows that high myopic patients with STL show a greater CT, a lower PAA and a lower prevalence of PS, compared with controls matched for axial length. These findings could be relevant for the development and progression of myopic maculopathy in patients with STL.

Keywords: Choroidal thickness; Congenital myopia; Hereditary vitreopathy; High myopia; Stickler syndrome.

MeSH terms

  • Arthritis*
  • Choroid
  • Connective Tissue Diseases* / complications
  • Hearing Loss, Sensorineural*
  • Humans
  • Retinal Detachment* / diagnosis
  • Retinal Detachment* / etiology
  • Tomography, Optical Coherence

Supplementary concepts

  • Stickler syndrome, type 1