dominant exudative vitreoretinopathyxexami ned propositus fig. 1 pedigree 1. detachment was observed...

British Journal of Ophthalmology, 1980, 64, 112-120

Autosomal dominant exudative vitreoretinopathyR. R. OBER, A. C. BIRD, A. M. HAMILTON, AND K. SEHMI

From the Institute of Ophthalmology, Moorfields Eye Hospital, City Road, London

SUMMARY Twelve affected members from 3 families with autosomal dominant exudative vitreo-retinopathy were examined, and the following conclusions were drawn: (1) There is great variabilityin the phenotypic expression of the abnormal gene, such that many patients have very mild diseasewhich can be detected with certainty only by fluorescein angiography. (2) Gene penetrance is closeto 100%. (3) Progress of fundus changes and visual threat is rare after 20 years of age.

Familial exudative vitreoretinopathy was firstreported by Criswick and Schepens' in severalmembers of 2 families with abnormalities of theretina and vitreous which closely simulated retro-lental fibroplasia, but in whom there was no historyof prematurity or oxygen administration during theneonatal period. The disease was described asbilateral and slowly progressive, with a familialincidence, but of uncertain inheritance pattern.Gow and Oliver2 expanded the original observations,identified autosomal dominant inheritance, anddivided the clinical course into 3 stages. The fluores-cein angiographic findings in familial exudativevitreoretinopathy were first described by Cannyand Oliver.3 They demonstrated closure of theperipheral retinal vasculature associated with ele-vated, temporal fibrovascular masses, and stressedagain the similarity between this entity and retro-lental fibroplasia.

This report describes the fundus and fluoresceinangiographic findings in 12 affected members of 3families with autosomal dominant exudative vitreo-retinopathy. In contrast to the impression given byprevious reports, the disease may be asymptomaticand nonprogressive, and the clinical abnormalitiesdifficult to identify.

Case reports

PEDIGREE 1 (Fig. 1)Case 1 (pedigree 1, 11-2). A 12-year-old Chinesefemale was the product of a normal gestation anddelivery, and received no oxygen therapy. At 2years of age extensive peripheral traction retinal

Correspondence to Richard R. Ober, MD, Department ofOphthalmology, University of Southern California, EstelleDoheny Eye Foundation, 1355 San Pablo Street, LosAngeles, California 90033, USA.

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* AFFECTED MALE OR FEMALE

X EXAM I NEDPROPOSITUS

Fig. 1 Pedigree 1.

detachment was observed in the left eye. Exudates,microaneurysms, and 'abnormal vessels' wereidentified in the temporal periphery of the right eye,and a diagnosis of bilateral congenital retinalvascular malformations was made. The right eyeremained stable until the age of 31 years, whenreduction of vision was noted. Macular exudateswere associated with an angiomatous lesion andtraction retinal detachment in the temporal retinalperiphery. Surgery was undertaken in Australia(G. Crock), and after the procedure the retinabecame reattached, and vision with the right eye im-proved and has subsequently remained unchanged.The patient was first seen at Moorfields Eye

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Autosomal dominant exudative vitreoretinopathy

Fig. 2 Fluorescein angiogram of case 1, showingdistortion of the right eye central retinal vessels. andleakage into the macula area.

Hospital on 10 October 1977. Visual acuity was6/9 (20/30) with the right eye and no light perceptionwith the left. The left eye was soft and shrunken,with a cataract and band keratopathy preventing aview of the fundus. The right anterior segment wasnormal. Fundus examination of the right eyerevealed temporal dragging of the posterior retina,chorioretinal scarring and pigmentation in the areaof the previous retinal surgery, and retinal vesselclosure anterior to the equator in all quadrants withexudates nasally. Fluorescein angiography of theright eye revealed leakage of dye from the distorted

retinal vessels in the posterior pole and at thetemporal margin of the optic disc (Fig. 2). In thepre-equatorial retina the retinal vessels were in-creased in number, straight, and radial, and theretinal vessels ceased abruptly anterior to theequator. There was dye leakage from these vessels.

Case 2 (pedigree 1, II-1). The 17-year-old brotherof case 1 was born at term and received no oxygentherapy. He was myopic, but had no other ocularsymptoms and was otherwise healthy. Visual acuitywas 6/5 (20/15) with the right eye (-5-25 d.sph.-3 50 d.cyl. axis 1750), and 6/5 (20/15) with theleft eye (-5 00 d.sph. -2-00 d.cyl. axis 05°). Theanterior segments of both eyes were normal. Fundusexamination of the right eye showed moderatetemporal dragging of the optic disc and macula.The peripheral vessels in all quadrants were morenumerous than normal, straight, and radiallyorientated. The vessels ended abruptly in the pre-equatorial region with retinal nonperfusion anteriorto this zone. At 10 o'clock, at the margin of vesselclosure, forward vessels were associated withwhitish exudates and subretinal fibrosis. Thechanges in the left eye were similar but less severe.Fluorescein angiography of the right eye showed anincrease in the number and calibre of retinal vesselsanterior to the equator; the vessels were straightenedand ceased abruptly forming a scalloped border.Diffuse dye leakage occurred from the vesselsposterior to the marginal closure, and prominentleakage of dye occurred from the neovascularisationin the temporal periphery (Fig. 3). The left eye

Fig. 3 Fluorescein angiogramof the right eye of case 2,showing distortion of the retinalvessels and peripheral vesselclosure, with diffuse leakagefrom the peripheral retinalvessels andfrom the forwardvessels.

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R. R. Ober, A. C. Bird, A. M. Hamilton, and K. Sehmi

IV

*@ AFFECTED MALE OR FEMALE

X EXAMINED/PRPSITUS

FOETAL DEATH

Fig. 4 Pedigree 2.

showed similar but milder changes in the peripheralretinal vasculature.One eye of both parents was examined and no

abnormalities were identified, but fluorescein angio-graphy was not undertaken.

PEDIGREE 2 (Fig. 4)Case 3 (pedigree 2, IV-2). A 17-year-old whitefemale was first seen at the age of 3 years with a3-week history of right exotropia. The patient wasborn at term at home and weighed 3409 g (7j lb).She was otherwise healthy. Initial examinationrevealed widespread peripheral traction retinaldetachment, with vitreous fibrosis in the right eyeand glial tissue on the left optic disc. A congenitalvascular malformation or endophthalmitis wasconsidered a possible cause for these changes. Twoand a half years later progression of the changes inthe left eye was recorded; at that time vision wasfinger counting with the left eye, and there weredense organised vitreous bands with extensiveretinal detachment. Vision was 6/60 (20/200) withthe right eye, and the fundus findings were un-changed. The patient was last examined on 3 April1978, and the visual acuity was 6/60 (20/200) withthe right eye (-200+1-50 axis 1000), and no lightperception with the left. The right anterior segmentwas normal. The right vitreous base was organised,giving rise to circumferential traction and detach-ment of the peripheral retina. The fibrous tissue wasinserted into the optic disc with forward traction onthe central retinal vessels. The left eye was soft andshrunken, and the fundus was not seen. Fluoresceinangiography was not performed.

There was no family history of eye disease, but10 other members of the family were examined;all were born at term with normal birth weight, andnone was given oxygen in the neonatal period.Unless otherwise stated, none had ocular symptoms,abnormalities were limited to the fundi, and thevisual acuity was 6/6 (20/20) or better with each eye.

Case 4 (pedigree 2, IV-1; 22-year-old brother ofthe propositus). The fundi appeared normal exceptfor an area in the left temporal periphery, wherethe retinal vessels appeared more numerous andstraightened. Fluorescein angiography of the lefteye in the temporal periphery showed an increasednumber of straight, parallel vessels that endedabruptly anterior to the equator in a scallopedborder; fluorescein leakage occurred from a few ofthose vessels. Similar vascular changes were seenin the temporal retina of the right eye.Case 5 (pedigree 2, III-15; 40-year-old mother of

the propositus). The posterior retinal vessels in theright eye showed mild temporal dragging (Fig. 5b).In the temporal periphery of the right eye, thevessels were straightened, and several were sheathed.In this zone the vessels were not perfused, and therewere pigmentary changes and minimal yellowish,retinal exudates. The left fundus appeared normal.Fluorescein angiography of the right eye temporalperiphery showed the retinal vessels to be morenumerous and increased in calibre, and leakageoccurred from these vessels (Fig. 5a). No perfusionoccurred anterior to the equator. In the left eye,despite the normal appearance of the vessels, therewas leakage into the posterior retina during angio-graphy (Fig. 5c). The periphery showed similarvascular changes to those in the right eye, andtemporally there was reduplication of the line ofleaking blood vessels (Fig. 5d).Case 6 (pedigree 2, III-17; 32-year-old maternal

aunt of the propositus). The posterior pole in eacheye was normal. The retinal blood vessels werestraightened and more numerous than normal inthe temporal periphery of both eyes. Fluoresceinangiography showed peripheral nonperfusion, andthe vessels ended in arteriovenous anastomoseswithout an intervening capillary network. Centralto the margin of perfused and nonperfused retinathe capillaries were dilated and there was diffuseleakage of dye.Case 7 (pedigree 2, 111-19; 28-year-old aunt of

the propositus). The fundi of each eye were appar-ently normal, but fluorescein angiography showedperipheral retinal vessel closure in the temporalperiphery of each eye, with dye leakage from thevessels at the border of closure.Case 8 (pedigree 2, II-6; 60-year-old maternal

grandmother of propositus). Other than mild adult

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Fig. 5 Fluorescein angiogram in case 5, showing distortion of the central retinal vessels in the right eyeonly (5b), peripheral retinal vessel closure with leakage (5a, d) and leakage from the paramacular vessels in theleft posterior pole (5c).

onset diabetes she was healthy, but had noticedrecent mild blurring of vision. Visual acuity was6/12 (20/40) in the right eye and 6/18 (20/60) in theleft eye. The anterior segments showed bilateralmild nuclear sclerosis. Intraretinal haemorrhagesand exudates were located in the posterior pole ofeach eye, and the peripheral fundus appearednormal. Fluorescein angiography revealed multi-focal retinal vascular disease consistent with back-ground diabetic maculopathy. The temporal peri-phery of each eye showed sharply demarcatedretinal vessel nonperfusion with leakage of dye,and areas of capillary nonperfusion.

Five other members of this family were examined(IV-7, IV-8, 111-16, 111-18, and II-5) and fluoresceinangiography undertaken on 3. They were allasymptomatic, with no history of ocular or systemicdisease. Visual acuities were normal, and no ocularabnormalities were found.

PEDIGREE 3 (Fig. 6)Case 9 (pedigree 3, III-l1). A healthy 15-year-oldIranian female with decreased vision in the righteye since childhood was seen on 17 July 1978. Shewas born at term and was given no oxygen in theneonatal period. Visual acuity was 6/60 (20/200)with the right eye, and 6/9 (20/30) with the left eye.Except for a 30 prism dioptre right esotropia, ex-ternal and anterior segment examination of each eyeshowed no abnormality. In the right fundus therewas severe temporal dragging of the posteriorretina (Fig. 7a), with circumferential traction of thetemporal peripheral retina. The vitreous base wasdense and organised temporally, preretinal vitreousbands were located inferiorly, 'white without pres-sure' was present in all quadrants, and retinalexudates were located temporally. The left eye hadsimilar but slightly less severe changes. Severallarge neovascular fronds resembling 'sea-fans'

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Fig. 6 Pedigree 3.

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M0AFFECTED MALE OR FEMALEX EXAMIIED

00 HISTORY UNILATERAL VISUAL LOSS( HISTORY BILATERAL VISUAL LOSS

/ PROPOSITUS* FOETAL DEATH

with feeder vessels were located in the temporalperiphery.

Fluorescein angiography of the right eye showeddiffuse leakage of dye in the posterior pole (Fig. 7a),and confirmed the large areas of peripheral non-perfusion. The left eye showed leakage of dye atthe disc and macula, and similar peripheral non-perfusion in all quadrants. The forward neovascu-larisation was located at the posterior margin ofthe zone of peripheral closure in the temporalperiphery, and leaked dye profusely (Figs. 7b, c).

Case 10 (pedigree 3, 111-10; 7-year-old sister of-the propositus). She had poor vision since infancybut no systemic disease. Visual acuity was 2/60(6/200) with the right eye (-300 d.sph. +0 50d.cyl. axis 90°), and 1/60 (3/200) with the left eye(-2 50 d.sph. +0 50 d.cyl. axis I 10°). Externalexamination showed 'searching' nystagmus and alarge variable esotropia with no fixation preference;the anterior segment was normal in each eye. Theleft eye showed severe temporal dragging of theposterior retina, many vitreous membranes, and atemporal fibrous mass in the peripheral retina andvitreous base which extended anteriorly to involvethe ciliary body. The inner layers of the temporalretina were highly elevated by traction with severallarge peripheral retinal holes present. The right eyehad similar but slightly milder involvement. Fluo-rescein angiography was not performed.

Case 11 (pedigree 3, 111-12; 22-year-old sister ofthe propositus). The right eye was enucleated ininfancy because of a possible intraocular malignancy.Correct visual acuity was 6/18 (20/60) with the left-eye (-6-50 d.sph. -2-00 d.cyl. axis 1650). The leftanterior segment was normal. The vitreous showedmild degeneration, snowflake vitreous opacities, andpreretinal vitreous membranes inferiorly. The retina

showed moderate temporal dragging of the discand macula, temporal retinal neovascularisation andexudates, peripheral 'white without pressure', andretinal vessel nonperfusion in all quadrants. Fluores-cein angiography demonstrated the mild draggingof the posterior retinal vessels with dye leakage;there was also focal venous leakage above and nasalto the optic disc (Fig. 8a). Angiography also con-firmed peripheral nonperfusion and profuse leakagefrom the peripheral vessels (Fig. 8b) and at theposterior pole.Case 12 (pedigree 3, 11-13; 55-year-old father of

the propositus). Visual acuity was 6/6 (20/20) ineach eye. The anterior segments were normal. Thefundi were normal except for mild retinal exudatesand peripheral vessel closure in the temporalperiphery of the right eye. Fluorescein angiogramswere poor, but were adequate to confirm peripheralretinal vessel closure.The 25-year-old brother of case 8 was healthy

and without ocular symptoms. Ocular examinationsand fluorescein angiography were normal.

Five relatives had poor vision with 1 eye and 1with both. The cause of visual loss was unknown,and there was no opportunity to see the otherfamily members.

Discussion

Criswick and Schepens1 described in detail thevitreoretinal changes in 6 cases of autosomaldominant exudative vitreoretinopathy, and Gowand Oliver2 described 3 clinical stages. Stage 1showed vitreoretinal interface changes, vitreousbands and membranes, and peripheral retinaldegeneration; stage 2 the occurrence of neovascu-larisation, retinal exudates, elevated fibrovascular

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Fig. 7 Fluorescein angiogramof the right eye af case 9,showing distortion of the centralretinal vessels (a). Peripheralr-etinal vessel closure wasassociated wI1ith fJoward niewt-vessels (b, c).

masses in the temporal periphery, localised retinaldetachment, and dragging of the disc and macula;stage 3 total retinal detachment, associated withanterior segment changes comprising cataractformation, iris atrophy, neovascular glaucoma,band keratopathy, and eventual blindness.

Seven of our 12 patients had no ocular symptomsand were mildly affected by the disease (Table 1).None of these patients had 'white with pressure','white without pressure', or vitreous bands and

membranes, as described in stage 1 of the disease,2and only 2 of these patients had dragging of the discand macula in one or both eyes (cases 2 and 5). In3 of these 7 patients (cases 4, 6, and 8) the onlyophthalmoscopic finding was an excess of straightvessels in the periphery, which were radial. However,all of the 7 patients had pre-equatorial nonperfusionof retinal vessels, indicating the abnormal genotype.The peripheral straight vessels and peripheralnonperfusion, which were difficult to detect ophthal-

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Fig. 8 Fluorescein angiogramof case 11, showing distortionof the central retinal vesselswith leakage and dye in themacular region andfrom anasal retinal vein (a). Peripheralnonperfusion was associatedwith retinal telangiectasis (b).

moscopically, were easily demonstrated by fluores-cein angiography. Abnormality of the vitreous, asdescribed in the first reported cases, was not aprominent feature in many of our cases and doesnot appear to be essential in making the diagnosis.We conclude that, if the stated criteria of Gowand Oliver2 had been adhered to, some of thesecases might not have been identified.

Understandably, most emphasis has been placedon the peripheral retinal vascular changes. Thepattern of peripheral closure and forward prolifera-tion of new vessels has been well described,34 butthe retinal vessel abnormalities appear to be morewidespread. In several cases fluorescein leakageoccurred over a wide band of postequatorial retina,and in 3 cases there was leakage from the macularcapillaries even in the absence of gross traction(Fig. 5c).

Progressive traction causing retinal detachmentand vitreous haemorrhage from forward retinalblood vessels may be construed as the major threatto vision in these patients. Criswick and Schepens'judged the chances of complete arrest of the condi-tion to be small, and Gow and Oliver2 also empha-sised the progressive nature of the condition.Photocoagulation or cryotherapy have been recom-mended if progress is identified.'2 In each case itwas implied that therapy should be directed to theproliferating peripheral new vessel complexes. Ifthe object of this treatment was to reduce theincidence of vitreous haemorrhage, there is littleevidence to justify this. Vitreous haemorrhage has

been recorded in only 1 patient.' This patient had ahorseshoe tear, and no evidence was presented toindicate that the forward vessels were the source ofhaemorrhage.

Contrary to the impression given by these earlierpapers,'2 but in accord with the reservations ofGitter and co-workers,4 our findings suggest thatthe disease does not necessarily progress relentlesslyduring life, and in this respect differs from therecorded behaviour of Wagner's hyaloretinopathy.AIn our pedigrees, and in those already published, no

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Table 1 Ocular involvement in affected members of3 families with autosomal dominant vitreoretinopathy

Dragged Peripheral Peripheral Neo- Traction VitreousAge Sex Vision Symptoms disc/ exudates/ non- vasculari- detachment bands/

macula fibrosis perfusion sation membranes

Pedigree 1

Case 1 (11-2) 12 F OD 6/9 (20/30) Yes + + + + Yes No _ _OS NLP No fundus

view

Case 2 (II-1) 17 M OD 6/5 (20/15) No + + + Yes Yes - -OS 6/5 (20/15) + - Yes No - _

Pedigree 2

Case 3 (IV-2) 17 F OD 6/60 (20/200) Yes + + + + + + Yes No +++ + + +OS NLP No fundus

viewCase 4 (IV-1) 22 M OD 6/5 (20/15) No - _ Yes No

OS 6/6 (20/20) - - Yes No

Case 5 (III-15) 40 F OD 6/6 (20/20) No + + Yes NoOS 6/6 (20/20) - - Yes No

Case 6 (111-17) 32 F OD 6/6 (20/20) No - - Yes NoOS 6/5 (20/15) - - Yes No _ _

Case 7 (111-19) 28 F OD 6/5 (20/15) No - - Yes No - _OS 6/5 (20/15) - - Yes No - _

Case 8 (11-6) 60 F OD 6/12 (20/40)* No - - Yes NoOS 6/18 (20/60) - - Yes No

Pedigree 3

Case 9 (III-11) 15 F OD 6/60 (20/200) Yes + + + + + + Yes No + + + + +OS 6/9 (20/30) + + + + Yes Yes + + + +

Case 10 (III-10) 7 F OD 2/60 (6/200) Yes + + + + + + Not No + + ++OS 1/60 (3/200) + + + +++ determined No + + + + + +

Case 11 (III-12) 22 F OD Prosthesis YesOS 6/18 (20/60) + + + + Yes Yes - +

Case 12 (II-13) 55 M OD 6/6 (20/20) No _ + + NoOS 6/6 (20/20) - - - No - -

+ + + = Severe. + + = Moderate. + = Mild. - = Not present.*Decreased vision caused by diabetic maculopathy.

member has been reported as suffering from loss ofvision after the age of 20 years, and the disease wasnot obviously more advanced in older than inyounger patients with the abnormal gene. It isperhaps significant that those workers who under-took treatment as a result of documented progres-sion did so in patients under the age of 18 years.However, even in the young progression is notinevitable. Case 3 was remarkable in that there wastraction detachment of the whole pre-equatorialretina of the right eye at the age of 2j years whichdid not progress during the following 14 years. Theonly case in this series who was treated had retinaldetachment with loss of central vision (case 1), andvision has remained good during the subsequent81 years.

If our observations are representative of thedisease as a whole, and our interpretation of pre-

vious reports is correct, doubt is cast on the needfor any type of prophylactic measure, even in thepresence of marked abnormality. It is possible thattreatment should be limited to those patients inwhom there is visual loss. Furthermore, the lack ofprogression indicates that no visual threat to thepatient is implied by making the diagnosis in anadult during family survey.Gow and Oliver2 described an autosomal domi-

nant pattern of inheritance with reduced penetrance,because family members with the abnormal genewere found to have normal fundi (patients V-29and V-30); we assume fluorescein angiography wasnot performed in these cases. In our second pedi-gree there were 3 affected members out of 6 relativesat 50% risk of inheriting the abnormal gene, whichsuggests that the penetrance is close to 100%, andthe apparent reduction in penetrance is due to

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variable expressivity. Because of possible mildinvolvement we doubt whether an eye can be con-sidered normal without careful indirect ophthal-moscopy, 3-mirror contact lens examination, andfluorescein angiography. It is likely that the pheno-typic expression of the abnormal gene would havebeen detected in one parent of the propositus in the2 families studied by Criswick and Schepens' and inthe other eye of the reported unilateral cases2 iffluorescein angiography had been performed. Oneeye of each parent in pedigree 1 was dilated andfound to be normal, but careful 3-mirror contactlens examination and fluorescein angiography werenot done.The variable expressivity of autosomal dominant

exudative vitreoretinopathy has further implicationsfor the identification of heritable disease and geneticcounselling. A negative family history of poorvision does not exclude the diagnosis of this geneti-cally determined disease. Furthermore, individualsat risk of having the abnormal gene and seekinggenetic advice require a careful examination of theretinal periphery and fluorescein angiography beforeaccurate genetic counselling can be given. Thevariable expressivity also casts doubt on the diag-nosis of retrolental fibroplasia in the absence ofprematurity6 and neonatal administration of oxygen,unless other members of the family have been fullyassessed.The pathogenesis of autosomal dominant exu-

dative vitreoretinopathy is unknown. The sharplydemarcated zone of peripheral retinal blood vesselclosure and the pattern of arteriovenous anasto-moses are remarkably similar to retrolental fibro-plasia.7-9 The 2 conditions appear to be morphologi-cally indistinguishable, and it is possible that there

are similarities in the pathological mechanismsbetween them. Observations of the fundus ofpatients at risk of inheriting the abnormal geneduring the neonatal period may help to explain themorphology of the lesion, and the finding that thevisual prognosis appears to be determined in thefirst 2 decades of life.

Dr Richard R. Ober was supported by the Francis andRenee Hock Fellowship. We thank Miss J. Bramley andMrs E. Barrera for secretarial assistance and are grateful toProfessor G. Crock and Mr A. S. M. Lim for allowing us topublish case 1.

References

'Criswick VG, Schepens CL. Familial exudative vitreo-retinopathy. Am J Ophthalmol 1969;68: 578-594.2Gow J, Oliver GL. Familial exudative vitreoretinopathy.Arch Ophthalmol 1971; 86:150-155.3Canny CLB, Oliver GL. Fluorescein angiographic findingsin familial exudative vitreoretinopathy. Arch Ophthalmol1976; 94:1114-1120.4Gitter KA, Rothschild H, Waltman DD, Scott B, Azar P.Dominantly inherited peripheral retinal neovascularisation.Arch Ophthalmol 1978; 96:1601-1605.5Hirose T, Lee KY, Schepens CL. Wagner's hereditaryvitreoretinal degeneration and retinal detachment. ArchOphthalmol 1973; 89:176-185.6Cohen J, Alfano JE, Boshes LD, Palmgren C. Clinicalevaluation of school-age children with retrolental fibro-plasia. Am J Ophthalmol 1964; 57:41-57.7Flynn JT, O'Grady GE, Herrera J, Kushner BJ, CantolinoS, Milam W. Retrolental fibroplasia: I. Clinical observa-tions. Arch Ophthalmol 1977; 95:217-223.8Cantolino SJ, O'Grady GE, Herrera JA, Israel C, JusticeJ Jr, Flynn JT. Ophthalmoscopic monitoring of oxygentherapy in premature infants. Am J Ophthalmol 1971; 72:322-331.90'Grady GE, Flynn JT, Clarkson J, Clark RD. Retro-lental fibroplasia: Clinical, fluorescein angiographic andpathological correlation. Mod Probl Ophthalmol 1974; 12:144-151.

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dominant exudative vitreoretinopathyxexami ned propositus fig. 1 pedigree 1. detachment was observed...

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