- Case report
- Open Access
- Open Peer Review
This article has Open Peer Review reports available.
Intravitreal ranibizumab for choroidal neovascularization in a patient with angioid streaks and multiple evanescent white dots
© The Author(s). 2016
Received: 15 February 2016
Accepted: 19 July 2016
Published: 26 July 2016
To report a patient with angioid streaks (ASs) and coincident multiple evanescent white dot syndrome (MEWDS) who developed choroidal neovascularization (CNV).
A 20-year-old woman presented with reduced vision (20/100) in her left eye (LE). Based on a complete ophthalmologic examination the patient was diagnosed with ASs and coincident MEWDS. Two weeks later best-corrected visual acuity (BCVA) improved up to 20/25 and the MEWDS findings almost disappeared. Two months later BCVA dropped again (20/100) due to the development of CNV which was treated by a single intravitreal injection of ranibizumab (0.5 mg/0.05 mL). One month after this BCVA improved up to 20/40, and there was regression of the CNV. There was no need for retreatment at the last follow-up visit, 1 year after the ranibizumab injection, when the patient showed further recovery of BCVA up to 20/25.
In this case of ASs, MEWDS completely resolved after 2 weeks, but 2 months later CNV developed. A single intravitreal injection of ranibizumab had a long-lasting effect. Larger series are necessary to clarify the pathogenesis of CNV in such cases and the role of intravitreal ranibizumab.
Angioid streaks (ASs) are irregular linear breaks in the Bruch’s membrane that typically taper from the optic disk, usually associated with various systemic diseases such as Ehlers-Danlos syndrome, Paget disease, and pseudoxanthoma elasticum (PXE) . The main vision-threatening complication of AS is choroidal neovascularization (CNV) which develops in 72–86 % of eyes [2, 3].
Multiple evanescent white dot syndrome (MEWDS) is a benign inflammatory disorder of unknown etiology that usually affects young women . >It involves unilateral small, transient white dots at the level of the outer retina, the retinal pigment epithelium, and the inner choroid, which have a characteristic appearance on fluorescein angiography (FA) and indocyanine green angiography (ICGA). Patients present various symptoms such as photopsias, blurred vision, sudden decrease in visual acuity and visual field defects, either temporally or paracentrally . The prognosis is generally very good, even though the rare occurrence of CNV may lead to permanent visual loss [6–10].
Here we report a patient with ASs, diagnosed with coincident MEWDS. Two months after the diagnosis of MEWDS the patient developed CNV, which was effectively treated by intravitreal ranibizumab (Lucentis®, Novartis, Basel, Switzerland).
On the basis of all these findings a diagnosis of ASs and coincident MEWDS was made. The patient was prescribed oral prednisone (1 mg/kg) for 7 days then half the dosage for another 7 days.
Two weeks later, LE BCVA improved up to 20/25, with resolution of the MEWDS findings, except for the granular appearance at the macula; RE BCVA was 20/20 with no signs of inflammation at the fundus evaluation.
ASs are often complicated by the appearance of CNV [2, 3]. To date there are no reports of AS associated with acute ocular inflammation. MEWDS is a unilateral inflammatory disease, which usually resolves spontaneously, with full recovery . Here we describe a patient with ASs, who was diagnosed with coincident MEWDS. Two months after this diagnosis she developed CNV, which was effectively treated by intravitreal ranibizumab. In the current literature there are only six cases of CNV that developed after (from 4 weeks to 13 years) the diagnosis of MEWDS [6–10, 12]. Only two were effectively treated by intravitreal anti-vascular endothelial growth factor (VEGF) [10, 12]. In all other cases the visual loss was permanent despite treatment [6–9].
The case described is unusual: the CNV occurred in a patient with ASs, 2 months after the diagnosis of MEWDS. Although both ASs and MEWDS may contribute to the onset of CNV, the patient’s young age, good prognosis after intravitreal ranibizumab and the presence of MEWDS involving the macular area may indicate an inflammatory etiology of the CNV.
Though the pathophysiologic mechanism remains unclear, it has been suggested that alterations of Bruch’s membrane or the outer retinal barrier caused by choroidal inflammation may be associated with ischemic and/or inflammatory CNV . It has also been hypothesized that the inflammatory processes induce the release of chemokines that favor angiogenesis . This patient was in fact younger than the mean age at which CNV usually develops associated with ASs [15, 16]. Moreover, in this case a single intravitreal ranibizumab injection had a lasting effect (up to at least 1 year), as shown by FA and OCT, and by the maintenance of good BCVA.
Our results are in agreement with Rouvas et al. who have described the good responses to intravitreal treatment with ranibizumab for inflammatory retinal diseases . This might be explained by the different nature of the CNV, in which inflammation may have played an important pathogenic role, compared to other CNVs secondary to ASs which tend to be associated with a worse visual prognosis, and need more injections .
In this case of ASs, MEWDS completely resolved after 2 weeks, but 2 months later CNV developed. A single intravitreal injection of ranibizumab had a lasting effect. This illustrates the importance of correct etiological assessment of CNVs, for proper therapeutic management. Although both ASs and MEWS may have contributed to the CNV in our patient, an inflammatory pathogenesis must be considered, especially in young patients, where an idiopathic CNV may be the first manifestation of inflammatory chorioretinal diseases .
ASs, angioid streaks; CNV, choroidal neovascularization; MEWDS, multiple evanescent white dot syndrome; BCVA, best-corrected visual acuity; RE, right eye; LE, left eye; BE, both eyes; FA, fluorescein angiography; ICGA, indocyanine green angiography; SD-OCT, spectral-domain optical coherence tomography
Supported by Retina 3000 Foundation. This study received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
No funding was obtained for this study.
Availability of data and materials
All data supporting our findings are provided in the manuscript.
AP, DA, SK, contributed to conception and design; AP, DA, GQ, LR, to data acquisition, all authors contributed to interpretation of data; all authors drafted the article and approved its final version.
The authors declare that they have no competing interests.
Consent for publication
The patient consented to the submission of the case report to the journal.
Ethics approval and consent to participate
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. The local ethics committee ruled that no formal ethics approval was required in this particular case.
- Mansour AM, Shields JA, Annesley Jr WH, et al. Macular degeneration in angioid streaks. Ophthalmologica. 1988;197:36–41.View ArticlePubMedGoogle Scholar
- Clarkson JG, Altman RD. Angioid streaks. Surv Ophthalmol. 1982;26:235–46.View ArticlePubMedGoogle Scholar
- Singerman LJ, Hatem G. Laser treatment of choroidal neovascular membranes in angioid streaks. Retina. 1981;1:75–83.View ArticlePubMedGoogle Scholar
- Jampol LM, Sieving PA, Pugh D, Fishman GA, Gilbert H. Multiple evanescent white dot syndrome. I. Clinical findings. Arch Ophthalmol. 1984;102:671–4.View ArticlePubMedGoogle Scholar
- Quillen DA, Davis JB, Gottlieb JL, et al. The white dot syndromes. Am J Ophthalmol. 2004;137:538–50.View ArticlePubMedGoogle Scholar
- McCollum CJ, Kimble JA. Peripapillary subretinal neo-vascularization associated with multiple evanescent white- dot syndrome. Arch Ophthalmol. 1992;110:13–4.View ArticlePubMedGoogle Scholar
- Low U, Palmowski AM, Weich CM, Ruprecht KW. [Choroidal neovascularization followed in a patient with multiple evanescent white dot syndrome (MEWDS): a case re- port]. Klin Monatsbl Augenheilkd. 2004;221:1051–3.View ArticlePubMedGoogle Scholar
- Oh KT, Christmas NJ, Russell SR. Late recurrence and choroidal neovascularization in multiple evanescent white dot syndrome. Retina. 2001;21:182–4.View ArticlePubMedGoogle Scholar
- Wyhinny GJ, Jackson JL, Jampol LM, Caro NC. Sub-retinal neovascularization following multiple evanescent white-dot syndrome. Arch Ophthalmol. 1990;108:1384–5.View ArticlePubMedGoogle Scholar
- Rouvas AA, Ladas ID, Papakostas TD, Moschos MM, Vergados I. Intravitreal ranibizumab in a patient with choroidal neovascularization secondary to multiple evanescent white dot syndrome. Eur J Ophthalmol. 2007;17:996–9.PubMedGoogle Scholar
- Polk T, Goldman E. White dot chorioretinal inflammatory syndromes. Int Ophthalmol Clin. 1999;39:33–53.View ArticlePubMedGoogle Scholar
- Papadia M, Herbort CP. Idiopathic choroidal neovascularisation as the inaugural sign of multiple evanescent white dot syndrome. Middle East Afr J Ophthalmol. 2010;17(3):270–4.View ArticlePubMedPubMed CentralGoogle Scholar
- Soubrane G, Coscas G, Binaghi M, Amalric P, Bernard JA. Birdshot retinochoroidopathy and subretinal new vessels. Br J Ophthalmol. 1983;67:461–7.View ArticlePubMedPubMed CentralGoogle Scholar
- Perentes Y, Chan CC, Bovey E, Uffer S, Herbort CP. Massive vascular endothelial growth factor (VEGF) expression in Eales’ disease. Klin Monbl Augenheilkd. 2002;219:311–4.View ArticlePubMedGoogle Scholar
- Alagöz C, Alagöz N, Özkaya A, Çelik U, Turan MF, Yazici AT, Çekiç O, Demirok A. Intravitreal Bevacizumab in the treatment of choroidal neovascular membrane due to angioid streaks. Retina. 2015;35(10):2001–10.View ArticlePubMedGoogle Scholar
- Al-Rashaed S, Arevalo JF. Long-term follow-up of choroidal neovascularization secondary to angioid streaks: case series and literature review. Clin Ophthalmol. 2012;6:1029–34.View ArticlePubMedPubMed CentralGoogle Scholar
- Rouvas A, Petrou P, Douvali M, et al. Intravitreal ranibizumab for the treatment of inflammatory choroidal neovascularization. Retina. 2011;31(5):871–9.View ArticlePubMedGoogle Scholar
- Machida S, Fujiwara T, Murai K, Kubo M, Kurosaka D. Idiopathic choroidal neovascularization as an early manifestation of inflammatory chorioretinal diseases. Retina. 2008;28(5):703–10.View ArticlePubMedGoogle Scholar