This case report describes a patient with Usher syndrome and retinitis pigmentosa who developed macular edema. She was treated with intravitreal injections of the corticosteroid Ozurdex, which led to resolution of the macular edema. However, the effect was temporary and she required multiple injections over time. The response to Ozurdex supports the hypothesis that inflammation contributes to macular edema in retinitis pigmentosa patients. Ozurdex may be considered for treating refractory macular edema, but the temporary response requires re-injections.

1. Ozurdex for macular edema in
retinitis pigmentosa: a case report
Dra. Inés Contreras
Clínica Rementería
Dra. Marta S Figueroa
Hospital Universitario Ramón y Cajal
Presentación basada en el Poster presentado en congreso EURETINA 2014

2. Ozurdex for macular edema in retinitis pigmentosa: a case report
Dra. Inés Contreras, Dra. Marta S Figueroa
• The rationale for the use of intravitreal
corticosteroids in macular edema (ME) associated
with retinitis pigmentosa (RP) is that an inflammatory
response against actively degenerating
photoreceptors and retinal pigment epithelium may
be involved in its pathogenesis1,2.
• Multiple studies have reported positive responses
with intravitreal triamcinolone (IVT). However, the
effect of IVT is short and adverse effects are relatively
high. Recently, several case reports have reported
good results with intravitreal dexamethasone
(Ozurdex®)3-5.

3. Ozurdex for macular edema in retinitis pigmentosa: a case report
Dra. Inés Contreras, Dra. Marta S Figueroa
Case report
• A 24-year old woman diagnosed with Usher syndrome was seen in 2012. Visual
acuity was 0.1 in her right eye (RE) and 0.4 in her left eye (LE). Fundus
examination showed the typical signs of RP as well as ME and optic nerve head
drusen. Optical coherence tomography (OCT) showed severe ME. The patient
did not tolerate oral acetazolamide. Treatment with intravitreal anti-VEGF
(bevacizumab and ranibizumab) lead only to a slight reduction in ME. Therefore,
treatment was stopped.
• In September 2013 the patient reported a decrease in visual acuity: it had
dropped to 0.05 in the RE and 0.16 in the LE. ME had increased. The patient was
offered treatment with intravitreal Ozurdex. The LE was treated in October
2013. One week later, ME had improved dramatically. Subjectively, the patient
reported better contrast sensitivity. These results lead to the decision to treat
the RE.
• In December 2013, 9 and 6 weeks after the injection in the LE and RE visual
acuity had improved to 0.1 in the RE and remained stable at 0.16 in the LE. ME
was almost resolved in both eyes and an epiretinal membrane (ERM) could be
seen in the RE and a taut posterior hyaloid in the LE. Intraocular pressure (IOP)
was 36 mmHg in both eyes. Treatment with a fixed combination of timolol and
dorzolamide twice daily was started and one week later IOP was 16 mmHg RE
and 17 mmHg LE.

4. Ozurdex for macular edema in retinitis pigmentosa: a case report
Dra. Inés Contreras, Dra. Marta S Figueroa
1 week after
1st Ozurdex
6 weeks after
1st Ozurdex
15 weeks after
1st Ozurdex
1 week after
1st Ozurdex
9 weeks after
1st Ozurdex
18 weeks after
1st Ozurdex

5. Ozurdex for macular edema in retinitis pigmentosa: a case report
Dra. Inés Contreras, Dra. Marta S Figueroa
Case report
• In February 2014, 15 and 18 weeks after treatment, ME had reappeared. A
second injection of Ozurdex was performed in both eyes, with an excellent
anatomical response. The patient was offered vitrectomy for ERM removal but
chose to postpone surgery.
• In June 2014, a third injection was performed in both eyes due to recurrent ME.
Visual acuity remained stable at 0.1 in the RE and improved to 0.2 in the LE. A
subcapsular posterior cataract had developed in the RE. Phacovitrectomy was
performed in the RE in July 2014.
• One month after surgery, visual acuity remained stable at 0.1 and OCT
evidenced partial restoration of the foveal anatomy.

6. Ozurdex for macular edema in retinitis pigmentosa: a case report
Dra. Inés Contreras, Dra. Marta S Figueroa
6 weeks after
2nd Ozurdex
15 weeks after
2nd Ozurdex
2 weeks after
3rd Ozurdex
7 weeks after
2nd Ozurdex
16 weeks after
2nd Ozurdex
2 weeks after
3rd Ozurdex
1 month after
phacovitrectomy
RE

7. Ozurdex for macular edema in retinitis pigmentosa: a case report
Dra. Inés Contreras, Dra. Marta S Figueroa
Discussion
• The anatomical resolution of ME in RP after Ozurdex supports the hypothesis
that there is an underlying inflammatory response involved in its pathogenesis.
Ozurdex may be considered for ME refractory to medical treatment. However,
an important drawback is the temporary response and the need for re-injection.
• In our case, macular traction may also be an important factor; this will be
clarified with further follow-up.
1. Heckenlively et al. Association of antiretinal antibodies and cystoid macular edema in patients with retinitis
pigmentosa. Am J Ophthalmol 1999;127:565–573.
2. Yoshida et al. Clinical evidence of sustained chronic inflammatory reaction in retinitis pigmentosa. Ophthalmology
2013;120:100–105.
3. Saatci et al. Bilateral Intravitreal Dexamethasone Implant for Retinitis Pigmentosa- Related Macular Edema. Case Rep
Ophthalmol 2013;4:53–58.
4. Alhassan M and Quintyn JC. Unilateral intravitreal dexamethazone implant for bilateral retinitis pigmentosa-related
macular edema. Graefes Arch Clin Exp Ophthalmol 2013;251:2827–2828.
5. Srour et al. Intravitreal dexamethasone implant (Ozurdex) for macular edema secondary to retinitis pigmentosa.
Graefes Arch Clin Exp Ophthalmol 2013;251:1501–1506.

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