How are the ocular manifestations of sickle cell disease (SCD) treated?

Updated: Sep 30, 2020
  • Author: Mark Ventocilla, OD, FAAO; Chief Editor: Hampton Roy, Sr, MD  more...
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Answer

Answer

Ocular treatment is directed toward preventing vision loss from vitreous hemorrhage, retinal detachment, and epiretinal membranes. Medical ocular management may include topical medications; however, avoid carbonic anhydrase inhibitors, because they may cause further sickling and worsen the outflow obstruction. If the intraocular pressure remains elevated after a judicious trial of medical therapy, surgical intervention with an anterior chamber lavage is indicated.

The goal of treatment is to eliminate existing neovascularization and, thus, to eliminate the sequelae of proliferative sickle retinopathy (PSR). Modalities to treat proliferative sickle retinopathy include diathermy, cryotherapy, xenon arc photocoagulation, and argon laser photocoagulation.

Diathermy is used infrequently because of the high incidence of complications accompanying this procedure. Cryotherapy, both single freeze-thaw and triple freeze-thaw, has been used to treat PSR. Triple freeze-thaw has a high complication rate. Single freeze-thaw is used to treat peripheral vitreous hemorrhage in the presence of vitreous hemorrhage. Xenon arc and argon laser photocoagulation have been used to treat either the peripheral neovascularization or the feeder vessels to the neovascularization.

Photocoagulation applied through various techniques (eg, feeder vessel, focal scatter, peripheral circumferential scatter) is effective for treating proliferative sickle retinopathy and reducing the risk of vision loss. Because of potential complications from photocoagulation and the tendency for regression, patients older than 40 years probably do not require treatment. Complications of photocoagulation include choroidal neovascularization, retinal breaks, and peripheral choroidal ischemia.


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