Purpose

To describe a surgical method of bleb revision for hypotony maculopathy, to evaluate its long-term efficacy, and to define the relationship between the duration of hypotony maculopathy and visual acuity (VA) outcomes.

Design

Noncomparative retrospective case series.

Methods

Medical records of 33 patients with hypotony maculopathy who underwent primary bleb revision between June 1999 and September 2012 by a single surgeon at an academic medical center were reviewed. Hypotony maculopathy was characterized by the presence of a decrease in VA, retinal striae, and macular edema in the setting of decreased intraocular pressure (IOP) after glaucoma filtering surgery. The main outcome measure was final logMAR VA after bleb revision at 6 and 12 months.

Results

Thirty-three eyes of 33 patients were followed for 4.68 ± 3.56 years (range 0.55-12.69 years). Mean duration of hypotony maculopathy was 4.98 ± 8.93 months. LogMAR VA improved from 0.78 ± 0.40 at baseline to 0.34 ± 0.34 (P < .001) 6 months after bleb revision and to 0.45 ± 0.55 (P < .001) 12 months after bleb revision. Spearman rank coefficient (rs) correlating duration of hypotony and BCVA at both 6 and 12 months was significant (P = .015 and rs = 0.426, P = .028 and rs = 0.416, respectively). Mean IOP increased from 3.51 ± 2.27 mm Hg to 12.06 ± 4.06 mm Hg (P < .001) at 12 months. Fifty-two percent were on no antiglaucoma medications at last follow-up. Five eyes (15%) required a second bleb revision to correct persistent hypotony maculopathy.

Conclusion

Surgical repair for hypotony maculopathy provided a significant improvement in VA at 6 and 12 months. Surgical bleb revision is associated with good long-term control of IOP and improved VA in eyes with hypotony maculopathy after previous glaucoma filtering surgery.

Purpose

To compare choroidal thickness (CT) between individuals with and without glaucomatous damage and to explore the association of peripapillary and submacular CT with glaucoma severity using spectral domain optical coherence tomography (SD-OCT).

Methods

Ninety-one eyes of 20 normal subjects and 43 glaucoma patients from the UCLA SD-OCT Imaging Study were enrolled. Imaging was performed using Cirrus HD-OCT. Choroidal thickness was measured at four predetermined points in the macular and peripapillary regions, and compared between glaucoma and control groups before and after adjusting for potential confounding variables.

Results

The average (± standard deviation) mean deviation (MD) on visual fields was -0.3 (±2.0) dB in controls and -3.5 (±3.5) dB in glaucoma patients. Age, axial length and their interaction were the most significant factors affecting CT on multivariate analysis. Adjusted average CT (corrected for age, axial length, their interaction, gender and lens status) however, was not different between glaucoma patients and the control group (P=0.083) except in the temporal parafoveal region (P=0.037); nor was choroidal thickness related to glaucoma severity (r=-0.187, P=0.176 for correlation with MD, r=-0.151, P=0.275 for correlation with average nerve fiber layer thickness).

Conclusions

Choroidal thickness of the macular and peripapillary regions is not decreased in glaucoma. Anatomical measurements with SD-OCT do not support the possible influence of the choroid on the pathophysiology of glaucoma.

Background

Sturge-Weber syndrome (SWS) is a sporadic, neurocutaneous syndrome involving the skin, brain, and eyes. Because of the variability of the clinical manifestations and the lack of prospective studies, consensus recommendations for management and treatment of SWS have not been published.

Objective

This article consolidates the current literature with expert opinion to make recommendations to guide the neuroimaging evaluation and the management of the neurological and ophthalmologic features of SWS.

Methods

Thirteen national peer-recognized experts in neurology, radiology, and ophthalmology with experience treating patients with SWS were assembled. Key topics and questions were formulated for each group and included (1) risk stratification, (2) indications for referral, and (3) optimum treatment strategies. An extensive PubMed search was performed of English language articles published in 2008 to 2018, as well as recent studies identified by the expert panel. The panel made clinical practice recommendations.

Conclusions

Children with a high-risk facial port-wine birthmark (PWB) should be referred to a pediatric neurologist and a pediatric ophthalmologist for baseline evaluation and periodic follow-up. In newborns and infants with a high-risk PWB and no history of seizures or neurological symptoms, routine screening for brain involvement is not recommended, but brain imaging can be performed in select cases. Routine follow-up neuroimaging is not recommended in children with SWS and stable neurocognitive symptoms. The treatment of ophthalmologic complications, such as glaucoma, differs based on the age and clinical presentation of the patient. These recommendations will help facilitate coordinated care for patients with SWS and may improve patient outcomes.