Sir,
We report the first case of a choroidal neovascular membrane (CNV) in a patient with late-onset retinal degeneration (L-ORD) successfully treated with intravitreal Ranibizumab (Lucentis).
Case report
A 61-year-old man, heterozygous for the Ser163Arg mutation in C1QTNF5, presented with a 4-week history of distortion in his right eye. His visual acuity (VA) was 68 ETDRS letters OD and 82 letters OS. Fundus fluorescein angiography (FFA) showed a juxtafoveal classic CNV (Figure 1) and optical coherence tomography (OCT) confirmed intra-retinal oedema over the area of the CNV (Figure 1). Owing to the reported poor outcome from the use of focal argon laser photocoagulation in such cases,1 the patient received an intravitreal injection of Ranibizumab (0.5 mg). This was followed by two further injections at 4-weekly intervals. Four weeks after receiving the third treatment, the VA was 59 letters OD and 85 letters OS. An FFA showed closure of the lesion (Figure 2) and resolution of the intraretinal oedema on OCT (Figure 2). At the last follow-up visit (month 12), the VA was 57 letters OD and 85 letters OS (loss of 11 ETDRS letters from baseline), and FFA and OCT showed continued inactivity of the lesion. No additional Ranibizumab treatments had been required during this time. During follow-up, fundus autofluorescence imaging showed no significant enlargement of the areas of chorioretinal atrophy in the paramacular area (Figure 3).
Comment
L-ORD is an autosomal dominant retinal dystrophy caused by a mutation (Ser163Arg) in the protein C1QTNF5, which leads to the formation of a thick extracellular sub-RPE deposit.2, 3 Early features of the disease include nyctalopia and fine macular drusen, followed later by peripheral retinal and macular atrophy. Patients are also predisposed to choroidal neovascularisation, usually by the sixth decade.4 If untreated, the natural history of the lesions is poor.1 To date, only laser photocoagulation of lesions in three eyes has been reported, with poor results.1 We report the successful 12-month outcome of a juxtafoveal CNV treated with intravitreal Ranibizumab. Closure of the lesion was achieved after three treatments, with stabilisation of vision and no evidence of recurrence at month 12. Intravitreal Ranibizumab appears to be safe and more effective than laser photocoagulation in the treatment of CNV in patients with L-ORD.
References
Ayyagari R, Griesinger IB, Bingham E, Lark KK, Moroi SE, Sieving PS . Autosomal dominant hemorrhagic macular dystrophy not associated with the TIMP3 gene. Arch Ophthalmol 2000; 118: 85–92.
Hayward C, Shu X, Cideciyan AV, Lennon A, Barran P, Zareparsi S et al. Mutation in a short chain collagen gene, CTRP5, results in extracellular deposit formation in late-onset retinal degeneration: a genetic model for age-related macular degeneration. Hum Mol Genet 2003; 12: 2657–2667.
Ayyagari R, Mandal MNA, Athanasios JK, Chen L, McLaren NC, Lichter M et al. Late-onset macular degeneration and long anterior lens zonules result from a CTRP5 gene mutation. Invest Ophthalmol Vis Sci 2005; 46: 3363–3371.
Borooah S, Collins C, Wright A, Dhillon B . Late-onset retinal macular degeneration: clinical insights into an inherited retinal degeneration. Br J Ophthalmol 2009; 93: 284–289.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
The authors declare no conflict of interest.
Rights and permissions
About this article
Cite this article
Aye, K., Gupta, R., Talks, S. et al. Treatment of a choroidal neovascular membrane in a patient with late-onset retinal degeneration (L-ORD) with intravitreal Ranibizumab. Eye 24, 1528–1530 (2010). https://doi.org/10.1038/eye.2010.71
Published:
Issue Date:
DOI: https://doi.org/10.1038/eye.2010.71