British Journal of Ophthalmology, 1987, 71, 757-761

Retinal microangiopathy in pigmented paravenouschorioretinal atrophySURESH R LIMAYE AND MUNEERA A MAHMOOD

From the Ophthalmology Service, DC General Hospital and Centerfor Sight, Georgetown University MedicalCenter, Washington, DC, USA

SUMMARY This report describes an atypical case of pigmented paravenous chorioretinal atrophy,associated with focal progressive peripheral retinal microangiopathy, in a 51-year-old blackfemale. The eyes were asymmetrically involved. Although several cases have been reported withtypical features of this uncommon entity, none of them have been known to be associated withretinal microangiopathy. The occurrence of such microangiopathy supports the hypothesis thatdamage to the retinal photoreceptors may induce retinal microangiopathy, as suggested in otherclinical and experimental studies.

Pigmented paravenous chorioretinal atrophy is anasymptomatic disease characterised by the presenceof bilaterally symmetrical chorioretinal atrophyalong the distribution of the retinal veins with vari-able pigment migration. The disease progressesslowly and its aetiology remains unknown. As thecondition is restricted to the paravenous area, it isthought not to be a sight-threatening generaliseddegenerative disorder of the retina.' Between 1973and 1980 we examined three patients with pigmentedparavenous chorioretinal atrophy, only one of whomhad an unusual progressive, peripheral, retinalmicroangiopathy in the more involved eye. Such acase of Tetinal microangiopathy has not to ourknowledge been previously reported.

Case report

A 51-year-old black female from the West Indieswas examined for a routine eye examination inSeptember 1976. She denied symptoms of nightblindness or previous ocular disease. Her pastmedical history was also unremarkable, and therewas no family history of night blindness or any otherocular diseases. Visual acuity was 6/6 in each eye.External examination did not reveal any abnor-mality. Extraocular motility was full, and she wasorthophoric for distance and near. Slit-lamp exami-

Correspondence to Suresh R Limaye, MD, District of ColumbiaGeneral Hospital, Ophthalmology Service, Room 3325, CoreBuilding, 19th Street and Massachusetts Avenue, SE, Washington,DC 20003, USA.

nation showed only minor cuneiform cataracts ineach eye. Intraocular pressure by applanationtonometry was 16 mmHg in each eye.The fundus of the right eye showed typical

paravenous corpuscular pigmentation, with chorio-retinal atrophy extending from the optic disc to theequator and beyond (Fig. 1). In the temporalperiphery microaneurysms and telangiectasia of theretinal vessel was seen, but without exudates (Fig. 2).The fundus of the left eye, however, showed only

one isolated patch of chorioretinal atrophy along theinferior temporal vein (Fig. 3).The visual field of the right eye was markedly

constricted, especially on the nasal aspect, and that ofthe left eye was full on Goldmann perimetry (Figs.4a, 4b). A rod dominant (scotopic) electroretino-gram of the right eye revealed subnormal b-waveamplitude (150 [tv) with a normal implicit time (45m/s). The electroretinogram of the left eye in a roddominated condition showed a b-wave amplitude of350 [tv with an implicit time of 45 mls. Electro-oculography showed the light peak to dark troughratio of 2-0 for the right eye and 2*3 for the left eye.

Fluorescein angiography cof the right eye showedan extensive area of choriocapillaris atrophy withprominently visible choroidal vessels along the majorretinal veins adjacent to the disc in an early arterialphase. In the arteriovenous phase hyperfluorescencewas seen at the edge of the atrophic area andhypofluorescence was seen corresponding to theareas of pigment migration (Fig. 5).

Systemic examination was unremarkable and757

Suresh R Limaye and Muneera A Mahmood

Fig. 1 Rightfundus: a typicalparavenous chorioretinal atrophywith pigment migration, anduninvolved macular area.

routine laboratory tests, including serology forsyphilis and skin test for tuberculosis, were negative.Haemoglobin electrophoresis did not reveal any

abnormal haemoglobinopathy.Three years after the initial examination she was

found to have intraretinal hard exudates in thearea of previously noted microaneurysms andtelangiectasia, namely, in the temporal periphery ofthe fundus (Fig. 6). Fluorescein angiography of theperipheral fundus showed areas of capillary non-

perfusion, arteriovenous communications, andmicroaneurysms (Fig. 7).

Discussion

Pigmented paravenous chorioretinal atrophy is an

uncommon disease of unknown aetiology. The firstcase of this disorder was described by Brown in 1937

under the name of retinochoroiditis radiata.2 In 1962Franceschetti coined the term pigmented paravenouschoroiditis.3 Several other cases have been publishedsome under different names.' Noble and Carrrecently reported six additional cases and reaffirmedits characteristics,' previously well described byKrill.7 The linear extent of chorioretinal atrophy andparavenous pigmentation is remarkably variableamong these cases. A slow progression of the diseasewithout involvement of the macular area has beenemphasised.1

This case report, however, is unusual because suchan asymmetrical involvement is rare, and thepresence of retinal microangiopathy has not beendescribed before. As in previously reported cases,there was no evidence of intraocular inflammation,and the laboratory tests for associated systemicdisease were negative. The patient also denied

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Retinal microangiopathy inpigmentedparavenous chorioretinal atrophy

Fig. 2 Microaneurysm and telangiectasia seen in thetemporalperiphery (arrow) ofthe right eye.

having a family history of any ocular disorder. Atthe initial examination only telangiectatic micro-vascular changes were observed, without any intra-retinal exudate. Three years later a collection ofhard exudates in the inner retina was observed.Fluorescein angiography in this area showedcapillary non-perfusion, arteriovenous communica-tion, microaneurysmal dilatation and vessel wall

staining. A microangiopathy associated with otherdisorders such as diabetes mellitus, vein occlusion,sickle cell retinopathy, sarcoidosis, Eales' disease,and radiation retinopathy was excluded on the basisof clinical examination and laboratory investigation.Unfortunately not all her family members wereavailable for eye examination, though they claimedto have 'good eyes'.

It is noteworthy that the retinal microangiopathy,though not previously described with pigmentedparavenous chorioretinal atrophy, has been noted byCleary et al.8 in five cases of congenital hypertrophyof retinal pigment epithelium. Similar retinalvascular abnormalities have been described in ratswith hereditary visual cell degeneration,9 the lightdamaged rat retina,"' and after argon and ruby laserphotocoagulation of monkey retina.' Dantzker andGerstein"' observed obliteration of retinal capillariesin cats with iodoacetate induced photoreceptordamage. Telangiectatic changes are known to occuron the optic nerve head in patients with cone-roddegeneration.'2 Therefore it is postulated that in ourpatient the presence of microangiopathy may signifyphotoreceptor damage. Whether photoreceptordamage was secondary to primary degeneration ofthe retinal pigment epithelium is debatable. Amarked constriction of the visual field and a sub-normal ERG are, however, indicative of photo-receptor damage. It is not clear why the disease hasappeared so asymmetrically. The pathogenesis ofpigmented paravenous chorioretinal atrophy by itselfand in association with retinal microangiopathyremains unresolved.

Fig. 3 Leftfundus: only anisolated patch ofchorioretinalatrophy seen.

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Fig. 4a Fig. 4b

Fat. 5

Fig. 4A Visualfield ofthe right eye.

Fig. 4B Visualfield ofthe left eye.

Fig. 5 Fluorescein angiogram oftherighteyeshowingtransmitted hyperfluorescence and blockedfluorescence.

Fig. 6 Temporalperiphery ofthe right eyeshowingaccumulation ofhard exudates not seen on the initialexamination.

Fig. 7 Fluorescein angiogram ofthe temporalperipheryshowing capillary non-perfusion, microaneurysm, andarteriovenous communications.

Retinal microangiopathy in pigmentedparavenous chorioretinal atrophy

References

1 Noble KG, Carr RE. Pigmented paravenous chorioretinalatrophy. Am J Ophthalmol 1983; 96: 338-44.

2 Brown TH. Retino-choroiditis radiata. Br J Ophthalmol 1937;21: 645-8.

3 Franceschetti A. A curious affection of the fundus oculi.Helicoid peripapillary chorioretinal degeneration. Its relation topigmentary paravenous chorioretinal degenerations. DocOphthalmol 1962; 16: 81-110.

4 Morgan OG. Congenital pigmentation of the retina. Proc R SocMed 1948; 41: 726-7.

5 Pearlman JT, Kamin DF, Kopelow SM. Pigmented paravenous

retinochoroidal atrophy. Am J Ophthalmol 1975; 80: 630-5.6 Skalka HW. Hereditary pigmented paravenous chorioretinal

atrophy. Am J Ophthalmol 1979; 87: 286-91.7 Krill AE. Krill's hereditary retinal and choroidal diseases.Hagerstown: Harper and Row, 1977; 2: 625-35.

8 Cleary PE, Gregor Z, Bird AC. Retinal vascular changes incongenital hypertrophy of retinal pigment epithelium. Br JOphthalmol 1976; 60: 499-503.

9 Gerstein DD, Dantzker DR. Retinal vascular changes inhereditary visual cell degeneration in dystrophic rats. ArchOphthalmol 1969; 81: 99-105.

10 Dantzker DR, Gerstein DD. Retinal vascular changes followingtoxic effects on visual cells and pigment epithelium. ArchOphthalmol 1969; 81: 106-14.

11 Okisaka S, Kuwabara T, Aiello LM. The effects of laserphotocoagulation in the retinal capillaries. Am J Ophthalmol1975; 80: 591-601.

12 Heckenlively, JR, Martin DA, Rosales TO. Telangiectasia andoptic atrophy in cone-rod degeneration. Arch Ophthalmol 1981;99: 1983-91.

Accepted for publication 9 October 1986.

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