Vitreoretinal surgery for cystoid macular edema associated with retinitis pigmentosa

doi:10.1016/S0161-6420(03)00259-8

Ophthalmology

Volume 110, Issue 6, June 2003, Pages 1164-1169

Presented in part at the annual meeting of the American Academy of Ophthalmology, New Orleans, Louisiana, November 2001.

https://doi.org/10.1016/S0161-6420(03)00259-8 Get rights and content

Abstract

Purpose

To evaluate the anatomic and functional outcome of vitreoretinal surgery in eyes with retinitis pigmentosa (RP) and macular edema.

Design

Prospective noncomparative case series.

Participants

Twelve consecutive eyes of eight patients with RP and a documented decrease in visual acuity (VA) to 20/60 or worse caused by macular edema refractory to medical therapy.

Methods

Pars plana vitrectomy was performed in the 12 eyes, followed by posterior hyaloid dissection, removal of the posterior inner limiting membrane after staining with indocyanine green, and gas tamponade. Preoperative best-corrected VAs ranged from 20/60 to 20/400 (mean, 20/115).

Main outcome measures

Changes in VA and foveal thickness as determined by optical coherence tomography (OCT).

Results

The mean preoperative retinal thickness at the fovea was 477 μm. Optical coherence tomography showed a decrease in macular thickness of >40% in 10 eyes (83.3%), with a mean postoperative foveal thickness of 260 μm. The mean VA increased from 20/115 to 20/45, with an average of three lines of improvement.

Conclusions

Our results suggest that vitreoretinal surgery may effectively manage macular edema in RP.

Section snippets

Materials and methods

We conducted a prospective, nonrandomized study that included 12 eyes of 8 patients with RP and macular edema. Inclusion criteria were visual acuity (VA) of 20/60 or worse caused by macular edema secondary to RP, <12 months of evolution, and previous ineffective treatment with 250 mg of oral acetazolamide twice daily for 1 month. Patients were fully informed of all relevant aspects of the procedure, and all patients provided written, informed consent.

The preoperative data recorded from the

Results

The patients (five women and three men), who ranged in age from 26 to 48 years, were observed for 6 to 12 months after surgery (mean follow-up period, 9 months). The duration of the macular edema ranged from 3 to 11 months (mean, 7 months). The preoperative best-corrected VA ranged from 20/60 to 20/400 (mean, 20/115). The preoperative foveal thickness measured by OCT ranged from 380 to 570 μm (mean, 478 μm) and had a cystic appearance in 75% of patients; the cystic appearance was also

Discussion

Our analysis of these 12 eyes with RP and CME showed a significant postoperative increase in VA in 83.3% of patients. In 90% of these eyes, the decrease in macular leakage observed by fluorescein angiography correlated with the decrease in macular thickness measured by OCT. Measurement of the central foveal thickness with sequential OCT allowed us to accurately measure increases or decreases in retinal thickness with more sensitivity than with slit-lamp biomicroscopy, as shown by other authors.

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Se ha realizado una revisión bibliográfica combinando «inherited retinal dystrophy», «retinitis pigmentosa», «macular oedema» y un protocolo diagnóstico/terapéutico según los niveles de evidencia y recomendaciones de la «US Agency for Healthcare Research and Quality».
Este protocolo se ha discutido en las reuniones mensuales del grupo XAREA DHR con la participación de más de 25 profesionales, creando un documento de consenso.
La etiología del EMQ es multifactorial: disfunción de la barrera hematorretiniana, del epitelio pigmentario de la retina y de las células de Müller, inflamación y tracción vítrea.
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Se propone un esquema de tratamiento en el EMQ en las DHR en adultos, otro para pacientes pediátricos y otra en las DHR y cirugía de catarata.
Los IAC orales y tópicos son efectivos en el tratamiento del EMQ secundario a las DHR. El tratamiento con corticoides, anti-VEGF y vitrectomía son opciones de segunda línea. Se requieren ensayos clínicos aleatorizados para poder establecer la escala terapéutica en estos pacientes.
Inherited retinal dystrophies (IRD) are the leading cause of legal blindness in the working population. Cystic macular edema (CME) is one of the treatable causes of visual loss, affecting up to 50% of the patients.
A bibliographic review has been carried out combining “inherited retinal dystrophy”, “retinitis pigmentosa”, “macular edema” and a diagnostic-therapeutic protocol according to the levels of evidence and recommendations of the “US Agency for Healthcare Research and Quality”.
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The etiology of CME is multifactorial: dysfunction of the blood-retinal barrier, retinal pigment epithelium, and Müller cells, inflammation, and vitreous traction.
OCT is the test of choice for the diagnosis and follow-up of CME associated with IRD.
The drugs with the highest degree of scientific evidence are carbonic anhydrase inhibitors (IAC). Intravitreal corticosteroids, anti-VEGF, and vitrectomy with peeling of the internal limiting membrane do not have sufficient evidence.
A treatment scheme is proposed for the CME in IRD in adults, another for pediatric patients and an another for IRD and cataract surgery.
Oral and topical IACs are effective in the treatment of CME secondary to IRD. Treatment with corticosteroids, anti-VEGF, and vitrectomy are second-line options. Randomized clinical trials are required to establish the therapeutic scale in these patients.
Non-vasogenic cystoid maculopathies
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Besides cystoid macular edema due to a blood-retinal barrier breakdown, another type of macular cystoid spaces referred to as non-vasogenic cystoid maculopathies (NVCM) may be detected on optical coherence tomography but not on fluorescein angiography. Various causes may disrupt retinal cell cohesion or impair retinal pigment epithelium (RPE) and Müller cell functions in the maintenance of retinal dehydration, resulting in cystoid spaces formation. Tractional causes include vitreomacular traction, epiretinal membranes and myopic foveoschisis. Surgical treatment does not always allow cystoid space resorption. In inherited retinal dystrophies, cystoid spaces may be part of the disease as in X-linked retinoschisis or enhanced S-cone syndrome, or occur occasionally as in bestrophinopathies, retinitis pigmentosa and allied diseases, congenital microphthalmia, choroideremia, gyrate atrophy and Bietti crystalline dystrophy. In macular telangiectasia type 2, cystoid spaces and cavitations do not depend on the fluid leakage from telangiectasia. Various causes affecting RPE function may result in NVCM such as chronic central serous chorioretinopathy and paraneoplastic syndromes. Non-exudative age macular degeneration may also be complicated by intraretinal cystoid spaces in the absence of fluorescein leakage. In these diseases, cystoid spaces occur in a context of retinal cell loss. Various causes of optic atrophy, including open-angle glaucoma, result in microcystoid spaces in the inner nuclear layer due to a retrograde transsynaptic degeneration. Lastly, drug toxicity may also induce cystoid maculopathy. Identifying NVCM on multimodal imaging, including fluorescein angiography if needed, allows guiding the diagnosis of the causative disease and choosing adequate treatment when available.
Automated detection of diabetic macular edema involving cystoids and serous retinal detachment
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Citation Excerpt :
In this paper, we present a method to detect edema and identify the different types of DME. We have segmented the region for detection of cystoids by detecting the Vitreous-NFL layer and IS-OS layers [10–12]. This region is used to limit search for presence of cystoids.
Diabetic Macular Edema (DME) is a complex and irreversible ocular disorder which leads to permanent vision loss. DME mostly occurs due to the formation of cyst spaces and storage of serous fluid in retina. We present a fully automated method for the detection two forms of diabetic macular edema using Optical Coherence Tomography (OCT) images. Segmentation of the retinal layers in the OCT image is important in the evaluation of the ocular diseases. Gradient information of the retinal boundaries is used to detect the layers of the retina. Retinal thickness between the vitreous-NFL (Nerve Fiber Layer) and the RPE (Retinal Pigment Epithelium) layer is measured and compared with the pre-defined values of normal retinal thickness to detect the edema in the eye. To detect cystoid macular edema (CME), the cystoid regions which are non-reflective spaces between the vitreoretinal layer and the inner -outer segment (IS-OS) layer are extracted by processing the pixel values inside the restricted search region between these layers. For detecting the serous retinal detachment (SRD), we extract the subretinal fluid region from the retinal image by changing pixel values below the IS-OS layer with zeroes and retracing the light-to-dark boundary. We also suggest criteria to validate the above result and reject any unwanted region, if detected.
Treatment of cystoid macular edema secondary to retinitis pigmentosa: a systematic review
2018, Survey of Ophthalmology
Citation Excerpt :
There are also limited data available on the efficacy of treatments for CME in RP.22 Although the pathophysiology of macular edema secondary to RP is not clearly understood, various pharmacological and surgical treatments for CME in RP have been reported.13,15,20,37 Treatments such as grid laser photocoagulation and oral acetazolamide were first described in the 1980s.13,37
There are various treatments for cystoid macular edema (CME) secondary to retinitis pigmentosa; however, the evidence for these treatments has not been previously systematically reviewed. Our review that includes 23 studies shows that oral carbonic anhydrase inhibitors (including acetazolamide and methazolamide) and topical carbonic anhydrase inhibitors (dorzolamide and brinzolamide) are effective first-line treatments. In patients unresponsive to carbonic anhydrase inhibitor treatment, intravitreal steroids (triamcinolone acetonide and sustained-release dexamethasone implants), oral corticosteroid (deflazacort), intravitreal antivascular endothelial growth factor agents (ranibizumab and bevacizumab), grid laser photocoagulation, pars plana vitrectomy, or ketorolac were also effective in improving CME secondary to retinitis pigmentosa. Oral acetazolamide has the strongest clinical basis for treatment and was superior to topical dorzolamide. Rebound of CME was commonly seen in the long term, regardless of the choice of treatment. Oral acetazolamide should be the first-line treatment in CME secondary to retinitis pigmentosa. Topical dorzolamide is an appropriate alternative in patients intolerant to adverse effects of oral acetazolamide. More studies are required to investigate the management of rebound CME.
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Manuscript no. 210948.

The authors have no financial interest in any aspect of this study.

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Vitreoretinal surgery for cystoid macular edema associated with retinitis pigmentosa☆

Abstract

Purpose

Design

Participants

Methods

Main outcome measures

Results

Conclusions

Section snippets

Materials and methods

Results

Discussion

Am J Ophthalmol

Am J Ophthalmol

Ophthalmology

Ophthalmology

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Ophthalmology

Ophthalmology

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Ophthalmology

Cystoid macular edema in retinitis pigmentosa

Trans Am Acad Ophthalmol Otolaryngol