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Buntitar Lertsuwanroj, Thomas H Dohlman, Michelle N. Lee, Kimberly C Sippel, Ana G. Alzaga Fernandez, Edward C. Lai, Jessica Ciralsky; Prosthetic Replacement of the Ocular Surface Ecosystem treatment for patients with Stuve-Weidemann Syndrome. Invest. Ophthalmol. Vis. Sci. 2017;58(8):3057.
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© ARVO (1962-2015); The Authors (2016-present)
Stuve-Weidemann syndrome (STWS) is a rare skeletal disorder that has manifestations of dysautonomia. Visually significant corneal opacities are a common feature of this disorder and stem from the combination of corneal hypoesthesia, decreased blink reflexes, and hypolacrimation or alacrima. Traditional treatments for this disorder have included lubricating drops, punctal occlusion, lateral tarsorrhaphy, and optical iridectomy. Many other patients with syndromes marked by dysautonomia have benefited greatly from treatment with Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE). In this case series, we describe the first two patients with STWS successfully treated with PROSE.
A retrospective review of two siblings with STWS who underwent PROSE treatment at Weill Cornell Department of Ophthalmology between 2014 and 2016. Patient demographics, clinical presentations, visual acuity, duration of follow-up, recurrent epithelial defects and complications were described.
Two male siblings presented at ages 4 and 9 years old. Both patients presented with typical features of STWS, including short stature, skeletal dysplasia with bowing of long bones, painless fractures and scoliosis. The ophthalmic examination was significant for bilateral corneal scars in the setting of lagophthalmos, alacrima and decreased corneal sensation. Both patients had previously received treatment with aggressive lubricants and a nighttime tape-assisted tarsorrhaphy. Three eyes had suffered from persistent corneal epithelial defects prior to PROSE initiation. In all four eyes, best-corrected visual acuity improved with PROSE therapy compared to pre-PROSE visual acuity. In the eighteen months of follow-up, there have been no recurrent epithelial defects or other complications associated with PROSE wear.
PROSE may be considered as an adjunctive therapy in patients with STWS who develop progressive corneal pathology despite conventional treatment.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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