Background: To identify different choroidal patterns in Stargardt disease (STGD) and to assess their clinical correlates. Methods: 100 STGD eyes (29 males; mean age 42.6 ± 16.5 years) and 100 control eyes (29 males; mean age 43.2 ± 8.5 years) were included. Optical coherence tomography (OCT) and OCT angiography (OCTA) images were obtained. Four different choroidal patterns, quantitative OCT and OCTA parameters were assessed and statistically analyzed. The main outcome was the correlation between each choroidal pattern and anatomical and functional retinal status. Furthermore, we assessed structural and best corrected visual acuity (BCVA) changes of each STGD subgroup after one-year. Results: Mean BCVA was 0.63 ± 0.44 LogMAR for STGD patients and 0.0 ± 0.0 LogMAR for controls (p < 0.01). All quantitative parameters appeared deteriorated in STGD compared to controls (p < 0.01). Choroidal patterns were distributed as follows: Pattern 1 (normal appearing choroid) (15%), Pattern 2 (reduced Sattler or Haller layer) (29%), Pattern 3 (reduced Sattler and Haller layers) (26%), Pattern 4 (Pattern 3 + choroidal caverns) (30%). More advanced patterns significantly correlated with a more severe loss of retinal structural integrity. Furthermore, only Pattern 3 and Pattern 4 showed remarkable signs of progression after one year. Conclusions: Choroidal patterns were related with retinal structural status and BCVA loss, and with different disease progression.
Inherited retinal dystrophies (IRD) are a heterogeneous group of rare chronic disorders caused by genetically determined degeneration of photoreceptors and retinal pigment epithelium cells. Ultra-widefield (UWF) imaging is a useful diagnostic tool for evaluating retinal integrity in IRD, including Stargardt disease, retinitis pigmentosa, cone dystrophies, and Best vitelliform dystrophy. Color or pseudocolor and fundus autofluorescence images obtained with UWF provide previously unavailable information on the retinal periphery, which correlates well with visual field measurement or electroretinogram. Despite unavoidable artifacts of the UWF device, the feasibility of investigations in infants and in patients with poor fixation makes UWF imaging a precious resource in the diagnostic armamentarium for IRD.
Purpose: To describe a combinatory technique made of Mustardè cheek advancement flap and paramedian forehead flap as a reconstructive option after orbital exenteration. Methods: We retrospectively reviewed all patients who underwent orbital exenteration and subsequent reconstruction with this technique at our Head and Neck Department, Divisions of Ophthalmology and Otolaryngology, at San Raffaele Hospital, Milan, Italy. Results: Three patients were treated with the aforementioned technique, following orbital exenteration due to malignancies. All of them were affected by recurrent diseases arising from the ocular components or periorbital structures: one basal cell carcinoma and two squamous cell carcinomas. Excellent result was achieved considering skin texture and colour match, aesthetic results and intra and post-operative complications (only minor, surgically controlled haemorrhage and minimal dehiscence subsequent to defective healing occurred). Conclusion: The proposed technique can be considered by the head and neck reconstructive surgeon as a good option in extended orbital exenteration.
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