E u ropen Journl of Ophthlmology / Vol. 14 no. 3, 2004 / pp. 2 6 4-2 6 8 S H O RT COMMUNICAT I O N Cse re p o r t Polypoidl horoidl vsulopthy ssoited with Doyne s fmilil horoiditis: tretment with therml lser I.D. LADAS, D.A. KARAGIANNIS, I. GEORGALAS, A.A. ROUVAS, M.M. MOSCHOS, M. APOSTOLOPOULOS Deprtment of Ophthlmology, Medil Shool of Athens University, Athens - Gre e e PU R P O S E. To report the unusul ourrene of polypoidl horoidl vsulopthy (PCV) in ptient with Doyne s fmilil honeyom horoiditis (DFHC) nd its ourse fter lser tre t- m e n t. DE S I G N. Interventionl se re p o r t. ME T H O D S. Indoynine green (ICG) ngiogrphy guided lser ws performed on tive polypoidl lesions. RE S U LT S. A 45-yer-old mn with 15-yer history of ilterl DFCH nd srred mulr horoidl neovsulriztion in the right eye (RE) ws re f e r red to us with exudtive mulopthy in the left eye (LE). His est-orreted visul uity (BCVA) ws 20/800 in the RE nd 20/40 in the LE. ICG ngiogrphy reveled piture tht ws hrteristi for PCV in oth eyes. ICG guided rgon green lser ws performed on the tive prppillry nd perifovel polypoidl lesions of the LE. Eight months fter the lser photoogultion tre t- ment, the mulr exudtive lesions hd susided nd the BCVA improved to 20/20. The fvorle ntomi nd funtionl results hve remined stle over 3 yers. CO N C L U S I O N S. This is, to our knowledge, the first se of PCV tht ourred seondry to DFHC. (Eur J Ophthlmol 2004; 14: 2 6 4-8 ) KE Y WO R D S. Polypoidl horoidl vsulopthy, Doyne s fmilil honeyom horoiditis, Indoynine green ngiogrphy, Choroidl neovsulriztion Aepted: Ferury 29, 2004 I N T R O D U C T I O N For out 15 yers, polypoidl horoidl vsulopthy (PCV) hs een reognized s new distint exudtive mulopthy hrterized y peulir network of fine rnhing vessels externl to horiopillris nd multiple terminl neurysml diltions t the order of the vsulr network. It is generlly epted tht indoynine green (ICG) ngiogrphy is the exmintion of hoie for identifition of the vsulr polypoidl lesions of PCV with gret sensitivity nd speifiity (1-6). D o y n e s fmilil honeyom horoiditis (DFHC) is one of the dominntly inherited drusen syndromes hrterized y multiple lrge yellow-white soft drusen ll over the posterior pole tht pper t re l t i v e l y erly ge in more thn one memer of the sme fmily nd osionlly my led to horoidl neovs- 1 1 2 0-6 7 2 1 /2 6 4-0 5 $ 1 5. 0 0 / 0 Wihtig Editore, 2004
Lds et l ulriztion (CNV) (7-9). We report, to our knowledge, the first se of PCV in ptient with DFHC nd its ourse fter therml lser tre t m e n t. Cse report A 45-yer-old mn with 15-yer history of DFCH ws re f e r red to us with the dignosis of exudtive mulopthy seondry to mulr CNV in his left eye (LE). His fmily history ws positive for DFCH (his 36- y e r-old rother presented onfluent soft drusen in the mul of oth eyes). He ws in otherwise good helth nd tking no medition. His right eye (RE) ws dignosed with mulr CNV on the ground of DFHC 6 yers go. Best-orreted visul uity (BC- VA) ws 20/800 in the RE nd 20/40 in the LE. Fundus exmintion of the RE reveled n extensive geogrphi horoidl trophy in the mul tht hd ourred on the ground of n old srred CNV (Fig. 1). Results of fundus exmintion of the LE showed serosnguineous mulr neurosensory dethment with hrd exudtes prppillry nd two ttendnt perifovel dethments of the retinl pigment epithelium. All these lesions hd een developed on the gro u n d of multiple lrge soft drusen onfluents in the mul re (Fig. 2). Fluoresein ngiogrm (FA) delineted the ove lesions (Fig. 2). However, ICG ngiogrphy of the LE reveled, just nsl to the opti dis, peulir network of vessels t the level of horiopillris with multiple terminl neurysml diltions (Fig. 2d). Some more vsulr polypoidl lesions were deteted in the LE just inferior to the opti dis, s well s in the perifovel re (Fig. 2, -e). Results of ICG ngiogrphy of the RE showed some similr polypoidl lesions lose to the superior mrgin of the opti disk nd t the temporl order of the geogrphi horoidl trophy (Fig. 1, nd ). ICG-guided rgon green lser photoogultion ws performed diretly on the tive vsulr polypoidl lesions of the LE ording to similr tehnique tht we hve desried previously in treting ptients with dieti mulr edem (10). Prtiulrly, the sites of lser pplition were mpped on the ICG ngiogrm imge nd trnsferred to the re d - f ree fundus photogrph using the Topon Imgenet 2000 Digitl Imging System with Imgenet 2000 (version 2.14) softw re nd the TRC-50IA fundus mer (Fig. 3). We performed the lser tretment looking k nd forth Fig. 1 - Right eye. ) Color fundus photogrph. Chorioretinl sr in the entrl retin., ) Indoynine green ngiogrms. V s u l r polypoidl lesions just superior to the opti disk nd temporl to the mulr sr (rrows). etween the olor imge of the ptient s fundus thro u g h the iomirosope of the lser nd the proessed re d f ree fundus imge displyed on the omputer monitor djent to the lser. The desired gol of the lser 2 6 5
PCV ssoited with Doyne s fmilil horoiditis: tretment with therml lser d Fig. 2 - Left eye efore tretment. ) Color fundus photogrph demonstrting exudtive mulopthy on the ground of lrge soft onfluent drusen. ) F l u o resein ngiogrm shows two lrge perifovel retinl pigment epithelil dethments., e) Indoynine gre e n ngiogrm demonstrting typil horoidl vessels with tive polypoidl diltions prppillry d ), s well s in the mul, e). pplition ws to hieve moderte retion in the sites of tretment. Eight months fter the lser opertion, the mulr exudtive lesions hd ompletely susided nd the B C VA improved to 20/20 (Fig. 3, nd ). The fvorle ntomi nd funtionl results hve re m i n e d stle over 3 yers. D I S C U S S I O N DFHC is n utosoml dominnt disese hrterized y multiple yellow-white lrge soft drusen tht umulte eneth the retinl pigment epithelium in the mul nd they my led to pigmentry hnges e nd/or to mulr CNV (7-9). The importne of this disese is due in lrge prt to its lose similrity to g e - relted mulr degenertion, espeilly when the mulr CNV develops in ptients older thn 50 yers. Our se onerns 45-yer-old mn with 15-yer history of DFHC nd n old mulr firovsulr sr in the RE, who presented n exudtive mulopthy 2 6 6
Lds et l suggesting the development of mulr CNV in the other eye. Although the ptient ws re f e r red to us with the initil dignosis of mulr CNV, he ws ultimtely dignosed with PCV. The role of ICG ngiogrphy to set the orret dignosis ws deisive. With ICG ngiogrphy we ould identify the polypoidl vsulr lesions nd distinguish them from the CNV with g ret sensitivity nd speifiity (Fig. 1, nd, nd Fig. 2, -e). Further, ICG ngiogrphy ws very importnt s guide to the lser photoogultion tre t- ment preisely on the tive polypoidl lesions. The results of the rgon green lser photoogultion in our ptient were very stisftory. Eight months fter the lser tretment, the mulr exudtive lesions showed omplete resolution (Fig. 3) nd the B C VA hd improved to 20/20. These fvorle ntomi nd funtionl results hve remined unhnged during long follow-up period. Our ptient is, to our knowledge, the first re p o r t e d se of PCV on the ground of DFCH. ICG-guided rgon lser tretment of the tive polypoidl lesions hd exellent results. Reprint requests to: Ionnis D. Lds, MD 8 Meg. Alexndrou Str. GR-152 36 P. Penteli Athens, Gre e e lds@th.forthnet.gr R E F E R E N C E S Fig. 3 - Left eye. ) Proessed red free fundus imge used to guide lser tretment. Yellow tres: Ative polypoidl lesions (lser spots must e pplied on them)., ) Color fundus photogrph ) nd fluo resein ngiogrm ) demonstrting omplete resolution of the exudtive lesions of the mul 8 months post lser tretment. 1. Ynnuzzi LA, Sorenson J, Spide RF, Lipson B. Idiopthi polypoidl horoidl vsulopthy (IPCV). Retin 1990; 10: 1-8. 2. Spide RF, Ynnuzzi LA, Slkter JS, et l. Indoynine g reen video ngiogrphy of idiopthi horoidl vs u l o p t h y. Retin 1995; 15: 100-10. 3. Ahuj RM, Stng PE, Vingerling JR, et l. Polypoidl h o roidl vsulopthy in exudtive nd hemorrhgi pigment epithelil dethments. Br J Ophthlmol 2000; 84: 479-84. 4. Sssellti-Sforzolini B, Mriotti C, Bryn R, et l. Polypoidl horoidl vsulopthy in Itly. Retin 2001; 21: 1 2 1-5. 5. Kwok AKH, Li TYY, Chn CWN, et l. Polypoidl horo i d l vsulopthy in Chinese ptients. Br J Ophthlmol 2002; 86: 892-7. 2 6 7
PCV ssoited with Doyne s fmilil horoiditis: tretment with therml lser 6. Lds ID, Rouvs A, Moshos M, et l. Polypoidl horo i d l vsulopthy nd exudtive ge relted mulr degenertion in Greek popultion. Eye 2004; 18: 455-9. 7. P e re WG. Doyne s honeyom retinl degenertion. Br J Ophthlmol 1968; 52: 73-8. 8. Piguet B, Himovii R, Bird A. Dominntly inherited drusen re p resent more thn one disorder: historil re v i e w. Eye 1995; 9: 34-41. 9. Uym M, Mtsurt, Fukusim I, et l. Idiopthi polypoidl horoidl vsulopthy in Jpnese ptients. Ar h Ophthlmol 1999; 117: 1035-42. 1 0. Lds ID. Use of digitized fluoresein ngiogrm system to guide lser tretment of dieti mulr edem: new tehnique. Ophthlmologi 2003; 217: 194-8. 2 6 8