PMCCPMCCPMCC

Search tips
Search criteria 

Advanced

 
Logo of rjoRomanian Journal of Ophthalmology
 
Rom J Ophthalmol. 2015 Jul-Sep; 59(3): 167–171.
PMCID: PMC5712963

RETINAL ANGIOMATOUS PROLIFERATION CASE REPORT

Abstract

The diagnosis of RAP is similar with the diagnosis of the AMD, but PED, exudate and superficial hemorrhages are more common in RAP.

A 75-year-old male presented himself at the Ophthalmology Department of the Emergency County Hospital in Craiova in January 2015, with a 4 months history of vision loss. In his right eye the visual acuity was 4/50 eye non-optical correctable and 5/5 with optical correction in the left eye.

According to the FA and OCT that were performed in both eyes, in the right eye was found intraretinal neovascularization and sub and intraretinal fluid. A normal aspect was found in the left eye. After the investigations we decided to start the treatment consisting in one single intravitreal injection with Triamcinolone Acetonide (IVTA) in the right eye. The VA improved 1 week after the treatment from 4/50 to 5/30. In comparison with the improved VA, the macular edema gradually resorbed 1 month after the IVTA injection.

In spite of the late presentation of the patient in the Ophthalmology Department, the VA and OCT outcome after a single dose of IVTA injection was very good.

Keywords: Retinal angiomatous proliferation, Triamcinolone acetonide, Age related macular degeneration AMD, Intravitreal injection

Introduction

Retinal angiomatous proliferation (RAP) is defined as a variant of exudative age related macular degeneration (AMD) in which the retinal-choroidal neovascularization is characterized by intraretinal capillary proliferation or, if the origin of the process is in the choroid, then it is an early retinal-choroidal anastomoses (RCA) without the presence of a neovascular membrane type 1. Although dry AMD represents the majority of all diagnosed cases, wet AMD associated with RAP is responsible for the majority of the severe vision loss and it can be very difficult to treat. The diagnosis of RAP is similar with the AMD but exudate and superficial and multiple hemorrhages are more common. [1,2,3] We describe the case of a 75-year-old male with unilateral RAP treated successfully with intravitreal triamcinolone acetonide injection (IVTA).

Case presentation

A 75-year-old male presented in the Ophthalmology Department of the Emergency County Hospital Craiova in January 2015 with a 4 months history of vision loss in his right eye.

The medical history of the patient revealed in the right eye: a cataract surgery with a posterior chamber pseudofak 2 years ago, a laser YAG iridotomy for closed angle glaucoma 2 years ago and a macular edema; in his left eye a laser YAG iridotomy for closed angle glaucoma 2 years ago and a senile cataract in evolution. He was also diagnosed with essential arterial hypertension 10 years ago and impaired glucose tolerance 8 months ago.

At the admission his visual acuity (VA) was 4/50 in the right eye non-optical correctable and 5/5 with optical correction in the left eye.

The slit lamp examination revealed laser YAG iridotomy in both eyes, posterior chamber pseudofak in the right eye, cortico-nuclear densifications in the left eye.

The indirect ophthalmoscopy examination revealed normal optic disc, a few macular hemorrhages situated in the superior-temporal sector, a white epiretinal membrane and macular edema in the right eye (Fig. 1). The left eye indirect ophthalmoscopy could not be performed due to the miotic pupil.

Fig. 1
Right eye – white epiretinal membrane, macular edema

The angiofluorography (FA) in the right eye shows intraretinal neovascularization (Fig. 2) and late hyperfluorence due to staining (Fig. 3).

Fig. 2
intraretinal neovascularisation
Fig. 3
late hyperfluorence due to staining

An optical coherence tomography (OCT-Fig. 4) was performed to the patient before the IVTA injection in both eyes as shown below. OCT revealed sub- and intraretinal fluid in the right eye with central retinal thickness of 752 µm and perifoveolar thickness of 549.3 µm and normal macular aspect with normal thickness in the center 232 µm and perifoveolar 260.9 µm.

Fig. 4
3d macula right eye comparing to the left eye

After the investigations we decided to start the treatment consisting in one single intravitreal injection with Triamcinolone Acetonide (IVTA) in the right eye.

The follow-up visits were made at 2 days, 1 week, 4 weeks, 3 months after the IVTA injection. Two days after the IVTA injection the VA improved at about 5/40. At the 2nd follow-up visit the BCVA was 5/30. In comparison with the improved VA the macular edema gradually resorbed and 1 months after the IVTA injection the central retinal thickness was 527µm and perifoveolar was 417.4 µm.

Fig. 5
After 1 week from IVTA injection
Fig. 6
Comparing before and after the IVTA

Discussions

The first author who described the presence of anastomoses between the retinal and choroidal circulations in eyes with disciform scars was Oller over 100 years ago [4]. Chorioretinal anastomoses were later described, associated with laser photocoagulation [5], radiotherapy [6], chorioretinal inflammatory diseases [7] and parafoveal telangiectasias [8].

Later Hartnet et al described in 1992 nine cases of retinal neovascularization, to which they referred as “deep retinal vascular anomalous complex” [9]. In 2000, Slakter et al. described chorioretinal anastomoses in eyes with pigment epithelial detachment and indocyanine green (ICG) hot spots [10].

In 2001, Yannuzzi et al. described chorioretinal anastomosis as neovascular proliferation with origin in the retina, and proposed the designation of RAP – retinal angiomatous proliferation [2]. Several authors [11-16] maintained the designation of chorioretinal anastomosis, proposing a choroidal origin for this clinical entity. In 2008, Yannuzzi et al. described 5 cases of RAP with the neovascular complex originating in the choroid instead of the retina, and proposed that RAP should be called type 3 neovascularization [17].

According to Yannuzzi et al. [2] classified RAP initial neovascularization process in 3 stages:

1) Stage I– Intraretinal neovascularization. This stage is mainly diagnosed when the second eye is already affected. The presence of associated small retinal hemorrhages constitutes a very useful sign in early RAP diagnosis. A small elevation of the inner/intermediate retina caused by angiomatous tissue may be observed under a slit lamp; this elevation may extend tangentially, assuming telangiectasic appearance. In FA, the angiomatous complex is observed as a hyperfluorescent area in front of the RPE, identical to that occurring in classic choroidal and, more frequently occult neovascularization. Dilated retinal vessels may perfuse and drain the intraretinal neovascularization (IRN) and form retino-retinal anastomoses. ICG may reveal a hot spot with staining and leakage.

2) Stage II– Involvement of the subretinal space with localized neurosensory detachment of the retina, edema and retinal hemorrhages at the edges may already be observed in fundus color photography. The angiomatous formation draining vein becomes visible, originating in the deep retina. PED is observed in 94% of eyes; FA may reveal well-delimited hyperfluorescence in the diffuse leakage area associated with PED, identical to that occurring for a minimally classic choroidal neovascular membrane. ICG clearly establishes stage II diagnosis as the presence of a hot spot with leakage, as well as a hyperfluorescent area associated with serous pigment epithelium detachment (SPED). Leakage might not be revealed by FA, probably because fibrin from retinal exudates cannot be impregnated with fluorescein, contrary to what occurs with ICG [18]. FA is rarely useful in differential diagnosis between classic, occult or minimally classic membranes and stages I and II [2], contrary to ICG, where a slow-growing extra, juxta or subfoveal hot spot, sometimes asymptomatic, is observed in stages I and II. Retino-retinal anastomoses may also be observed in stage II.

3) Stage III– Choroidal neovascularization is clearly visible, sometimes with the appearance of vascularized PED.

According to Gass [19], RAP would progress in 5 stages instead of 3, easily identifiable by FA and ICG:

I) Pre-clinical stage– Atrophy of the outer retina, with retinal capillaries moving closer to a choroidal neovascular complex located below the RPE – type 1 choroidal neovascularization – and no clinical signs of chorioretinal anastomosis. ICG would be necessary to identify type 1 neovascularization.

II) Early clinical signs– Anastomosis between dilated capillaries of the deep retina and the choroidal neovascular complex is associated with small intraretinal haemorrhages, which would constitute the first clinical sign of chorioretinal anastomosis.

This stage may occur weeks or months before stage 3, where subretinal choroidal neovascularization is already observed.

III) Proliferation of choroidal neovascularization over the RPE– subretinal neovascularization – type 2 CNV.

IV) Appearance of serous PED caused by activation of newly formed subepithelial vessels.

V) Mixed neovascularization– piggyback-type neovascularization, with two levels – type 1 and type 2 with cicatricial disciform lesion, making chorioretinal anastomosis visible.

Stage 3 in Gass’s classification corresponds to stage I in Yannuzzi’s classification, with stages 4 and 5 in Gass’s classification corresponding to stages II and III, respectively.

Currently, Yannuzzi’s classification is the most widely used.

In our case, we diagnosed the patient with RAP stage I after Yanuzzi and stage II/III after Gass.

The average and median ages of a series of 108 patients studied by Yannuzzi et al. [2] were 80 and 81 years respectively.

Both eyes were similarly affected in 51.9% of patients, with RAP occurring in the second eye with an average of 15 months after appearing in the first eye.

At 3 years, both eyes were affected in 100% of patients [20,21].

In our case, the patient was diagnosed with RAP in only one eye but we will be monitoring him every six months.

Most of the published studies use intravitreal injection with ranibizumab in treating RAP as a form of exudative AMD. Due to the inflammatory process involved in RAP pathogenesis, recent research has proved the efficacy of IVTA injection in the treatment of this condition.

Saito M et al observed in a retrospective, observational case series over a 3 years period, that the combined therapy intravitreal bevacizumab (IVB) plus PDT was more effective than IVTA plus PDT in the treatment of RAP. [22]

Adnaan Haq et al demonstrated in a case report the successful RAP treatment with combined intravitreal ranibizumab and IVTA. [23]

Conclusions

In spite the patient late presentation in the Ophthalmology Department (4 months after he noticed the vision loss in the right eye) the VA and OCT outcome after a single dose of IVTA injection was very good.

Further studies are required to establish the difference between monotherapy with IVTA or combined therapy IVTA with ranibizumab as the optimal treatment for RAP.

References

1. Jack J. Kanski, Brad Bowling . Clinical Ophthalmology a Systematic Approach. Seventh Edition. Elsevier Saunders; 2011 . pp. 627–628. ISBN –13:9780702040931.
2. Yannuzzi LA, Negrao S , Iida T, Carvalho C, Rodriguez-Coleman H, Slakter J, Freund KB, Sorenson J, Orlock D, Borodoker N. Retinal angiomatous proliferation in age-related macular degeneration. Retina. 2001;21:416–434. [PubMed] [PubMed]
3. Myron Yanoff, Jay S. Duker. Ophthalmology. Third edition. Mosby Elsevier; 2009. pp. 651–663. ISBN 978-0-323-04332-8.
4. Oeller JN. Atlas of rare ophthalmoscopic conditions and supplementary plates to the atlas of ophthalmoscopy [Snowball T, translation] part C. Wiesbaden, Germany: JF Bergman; 1904. pp. 61–66.
5. Slusher MM, Tyler ME. Choroidoretinal vascular anastomoses. Am J Ophthalmol. 1980;90:217–222. [PubMed]
6. Boozalis GT, Schachat AP, Green WR. Subretinal neovascularization from the retina in radiation retinopathy. Retina. 1987;7(3):156–162. [PubMed]
7. Kennedy JE, Wise GN. Retinochoroidal vascular anastomosis in uveitis. Am J Ophthalmol. 1971;71(6):1221–1225. [PubMed]
8. Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol . 1982;100:769–780. [PubMed]
9. Hartnett ME, Weiter JJ, Garsd A, Jalkh AE. Classification of retinal pigment epithelial detachments associated with drusen. Graefes Arch. Clin Exp Ophthalmol. 1992;230:11–19. [PubMed]
10. Slakter JS, Yannuzzi LA, Schneider U, Sorenson JA, Ciardella A, Guyer DR, Spaide RF, Freund B, Orlok DA. Retinal choroidal anastomoses and occult choroidal neovascularization in age-related macular degeneration. Ophthalmology. 2000;107:742–753. [PubMed]
11. Coscas G. Chorioretinal anastomoses. International Symposium of fluorescein angiography. Toronto. Canada: 1994.
12. Coscas G. Chorioretinal anastomoses. Clinical features and OCT follow-up. In: OCT in AMD. Annual report of the French Ophtalmic Societies. Springer Medelzin Verlag Heidelberg. 2009:277–300.
13. Kuhn D, Meunier I, Soubrane G, Coscas G. Imaging of chorioretinal anastomoses in vascularized retinal pigment epithelium detachments. Arch Ophthalmol. 1995;113(11):1392–1398. [PubMed]
14. Gass JD, Agarwal A, Lavina AM, Tawansy KA. Focal inner retinal hemorrhages in patients with drusen: an early sign of occult choroidal neovascularization and chorioretinal anastomosis. Retina. 2003;23(6):741–751. [PubMed]
15. Silva RM, Faria de Abreu JR, Travassos A, Cunha-Vaz JG. Stabilization of visual acuity with photodynamic therapy in eyes with chorioretinal anastomoses. Graefes Arch Clin Exp Ophthalmol . 2004;242(5):368–376. [PubMed]
16. Silva R, Cachulo ML, Figueira J, Faria de Abreu JR, Cunha-Vaz JG. horioretinal anastomoses and photodynamic therapy: a two-year follow-up study. Graefe´s Arch. Clin. Exp. Ophthalmol. 2007;245:1131–1139. [PubMed]
17. Yannuzzi LA, Freund KB, Takahashi BS. Review of retinal angiomatous proliferation or type 3 neovascularization. Retina. 2008;28(3):375–384. [PubMed]
18. Lafaut BA, Aisenbrey S, Vanden Broecke C, Bartz-Schmidt KU. Clinicopathological correlation of deep retinal vascular anomalous complex in age related macular degeneration. Br J Ophthalmol. 2000;84(11):1269–1274. [PMC free article] [PubMed]
19. Gass JD, Agarwal A, Lavina AM, Tawansy KA. Focal inner retinal hemorrhages in patients with drusen: an early sign of occult choroidal neovascularization and chorioretinal anastomosis. Retina. 2003;23(6):741–751. [PubMed]
20. Massacesi AL, Sacchi L, Bergamini F, Bottoni F. The prevalence of retinal angiomatous proliferation in age-related macular degeneration with occult choroidal neovascularization. Graefes Arch Clin Exp Ophthalmol. 2008;246(1):89–92. [PubMed]
21. Gross NE, Aizman A, Brucker A, Klancnik JM Jr, Yannuzzi LA. Nature and risk of neovascularization in the fellow eye of patients with unilateral retinal angiomatous proliferation. Retina. 2005;25(6):713–718. [PubMed]
22. Saito M, Shiragami C, Shiraga F, Kano M, Iida T. Comparison of intravitreal triamcinolone acetonide with photodynamic therapy and intravitreal bevacizumab with photodynamic therapy for retinal angiomatous proliferation. Am J Ophthalmol. 2010;149:472–481. [PubMed] [PubMed]
23. Adnaan Haq, Bharat Kapoor, Maharatnam Logendran, Gopinath Reddy. Successful Treatment of Retinal Angiomatous Proliferation with Intravitreal Triamcinolone and Ranibizumab Injections in a 67-Year-Old Male. Case Rep Ophthalmol. 2014 Sep-Dec;5(3):392–399. Published online 2014 Nov 26.doi:10.1159/000369611. [PMC free article] [PubMed]

Articles from Romanian Journal of Ophthalmology are provided here courtesy of Romanian Society of Ophthalmology