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Acute primary angle-closure in Sturge-Weber syndrome
Wei-Wen Su1,2
1Department of Ophthalmology, Chang Gung Memorial Hospital Linkou Branch, Taoyuan Taiwan, ROC.
American Journal of Ophthalmology Case Reports
|March 16, 2018
Summary
Sturge-Weber syndrome (SWS) patients can develop acute primary angle-closure (PAC). Phacoemulsification with intraocular lens (IOL) implantation successfully treated PAC in an SWS patient, avoiding bleeding risks associated with laser iridotomy.
Area of Science:
- Ophthalmology
- Neurology
- Genetics
Background:
- Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder.
- It involves facial port-wine stains, leptomeningeal angiomas, and ocular abnormalities.
- Acute primary angle-closure (PAC) is an uncommon complication of SWS.
Purpose of the Study:
- To report a case of SWS with ipsilateral acute PAC.
- To evaluate treatment options for PAC in SWS patients.
Main Methods:
- A 64-year-old female with SWS and a facial port-wine stain presented with acute PAC.
- Initial treatment included intravenous Mannitol and oral Acetazolamide.
- Phacoemulsification and intraocular lens (IOL) implantation were performed due to bleeding risks with laser iridotomy.
Main Results:
- The patient's acute PAC resolved with medical management.
- Phacoemulsification and IOL implantation led to successful IOP control and improved visual acuity.
- Anterior segment optical coherence tomography confirmed significant angle opening post-surgery.
Conclusions:
- Ipsilateral acute PAC in SWS is rarely documented.
- Phacoemulsification and IOL implantation offer a viable alternative to laser iridotomy in SWS patients with PAC, mitigating bleeding risks.
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