long-termfollow-up of central serous chorioretinopathy · described central serous...

British Journal of Ophthalmology, 1984, 68, 815-820

Long-term follow-up of central serouschorioretinopathyC. MITCHELL GILBERT, SARAH L. OWENS, PATRICIA D. SMITH,AND STUART L. FINE

From Retinal Vascular Center, the Wilmer Ophthalmological Institute, the Johns Hopkins MedicalInstitutions, USA

SUMMARY The Wilmer Retinal Vascular Center's experience with central serous chorioretinopathyfrom 1970 to the end of 1979 was reviewed and compared with previous studies. Retrospectiveanalysis of 73 patients seen at follow-up suggests no clinically significant effect of focal argon laserphotocoagulation on final visual acuity or recurrence rate. Patients with initial visual acuity of 20/20remained at that level, and patients with initial visual acuity of less than 20/30 gained, on average,two to three Snellen lines at follow-up. Approximately one-third of both untreated and treatedpatients had recurrence or presumed persistence during the follow-up period. With the inclusion ofepisodes that occurred before the first Wilmer Institute visit about half of each group hadrecurrence or presumed persistence. Recurrences were most often due to leakage from a site withinone disc diameter of the original site of leakage.

Central serous chorioretinopathy (CSCR) is anidiopathic serous detachment of the macula causedby focal leakage of choroidal interstitial fluid throughthe retinal pigment epithelium. Since von Graefe firstdescribed central serous chorioretinopathy in 1866'dozens of papers have reported its demographiccharacteristics, presenting visual symptoms, andophthalmological signs. Patients usually report thesudden onset of central blurring, metamorphopsia,and relative scotoma. Serous detachment usually lastsabout three months. Focal photocoagulation of theleak site significantly decreases the duration ofdetachment to about one month, but has no significanteffect on visual acuity.2-5 Indirect photocoagulation,which has been employed to avoid direct coagulationofsubretinal foveal leaks, has no effect on the durationof detachment.56We found in the literature 12 series that in-

cluded CSCR patients with follow-up of one or moreyears.2-5 7-13 Seven are retrospective follow-up studies,four are prospective randomised studies, and one is aprospective, nonrandomised study. The studies byKlein et al.'0 Dellaporta," and Nanjiani'2 are mostcomparable with our study.

Correspondence to Stuart L. Fine, MD, Maumenee 229, WilmerOphthalmological Institute, Johns Hopkins Medical Institutions,600 North Wolfe Street, Baltimore, Maryland 21205, USA.

In our retrospective long-term follow-up study ofCSCR patients we studied visual acuity outcome,recurrence tendency, and pigment epithelial leakagepattern and mottling features. We searched forpossible prognostic indicators and attempted todetermine the long-term effects of focal argon laserphotocoagulation.

Patients and methods

We reviewed 157 cases of CSCR with classic focalretinal pigment epithelial dye leakage and maculardetachment that were identified from the WilmerRetinal Vascular Center files. Cases with coexistingocular disease were not included. Of these 157 casesof active central serous retinopathy 105 (67%) werenot treated and 52 (33%) were treated with focalargon laser photocoagulation. We performed afollow-up examination on approximately half of eachgroup: 47 untreated, 26 treated. We obtained follow-up for an additional 45 patients from their local oph-thalmologists or by detailed questionnaire.The initial and interim examinations were per-

formed by the Wilmer Retinal Vascular Center staff.All initial examinations included visual acuitymeasurement (existing correction plus pinhole) by atechnician, direct and indirect ophthalmoscopy, con-tact lens biomicroscopy, and stereoscopic colour

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C. Mitchell Gilbert, Sarah L. Owens, Patricia D. Smith, and Stuart L. Fine

fundus photography. All but two patients with activeCSCR had fundus fluorescein angiography. Inpatients who were treated, the coherent radiationargon laser was used to produce a light to mediumintensity coagulation. Typical treatment parameterswere 100-200 ,um size, 0-1 to 0-2 s duration, and100-300 mW power. At the follow-up examinationthe same diagnostic procedures were performed byone or two of us (M.G., S.L.F.). Visual acuity was

again measured by a technician who did not knowwhether the patient had been treated or not. Stereocolour photographs were obtained on all patientswho returned for follow-up, and fluorescein angio-graphy was obtained on all but four patients.Separately and then together two ofus (M.G., S.L.F.)evaluated all colour photographs and fluoresceinangiograms for the following: location and size ofdetachment; number, location, type, and size ofretinal pigment epithelial dye leakage; and location,extent, and severity of retinal pigment epithelialmottling. Detachment and leakage characteristicswere easily agreed upon. Evaluation of retinal pig-ment epithelium mottling was much more subjectiveand therefore will not be discussed.

Results

Demographic data. The 47 untreated and 26 treatedpatients seen for follow-up were representative for allbaseline data of the 157 patients whose charts we

reviewed with respect to the following: percentagetreated, age, sex, laterality, onset to first visit,duration, onset to treatment, and potential follow-upduration. We will discuss in detail only the data forpatients seen in follow-up.

Basic demographic characteristics were typical ofthe previously reported series. Median age at initialvisit was 43 years, with a range of 27 to 66 years. Mencomprised 81% of the patients. 14% of the patientshad bilateral detachments (not necessarily simult-aneously), but in all of these patients one eye was

significantly more symptomatic.Temporal data. Most patients were referred to the

Wilmer Institute early in the course of the disorder.For the 47 untreated patients the median delaybetween onset of symptoms and first visit was threeweeks; for the 26 treated patients the median delaywas four weeks. Treatment was performed at a

median of five weeks after onset of symptoms. Twopatients were treated after more than three years ofpersistent symptoms.Median follow-up time for the 47 untreated patients

was 58 months, with a range of 1 to 10 years. Medianfollow-up time for the 26 treated patients was 84months, with a range of 1 to 9 years. The longermedian follow-up time for treated patients probably

reflected more liberal use of laser photocoagulationfor CSCR before the 1974 study by Watzke andBurton, which showed no beneficial effect of treat-ment on final visual acuity.4

Visual acuity. Thirty-five of the 47 untreatedpatients had 20/20 visual acuity at follow-up, and 19of the 26 treated patients attained 20/20 visual acuity.For both treated and untreated patients, if the initialvisual acuity was greater than or equal to 20/30, therewas no net change in visual acuity. Similarly, if theinitial vision was less than 20/30, there was an averageimprovement of between 2 and 3 Snellen lines.

Recurrences. By history, 13 (28%) of the 47 un-treated and five (19%) of the 26 treated patients hadone or more episodes of symptoms consistent withCSCR before the presenting episode. For the other34 untreated and 21 treated patients the presentingepisode was their first symptom. One or more docu-mented recurrences occurred during the follow-uptime in 10 (21%) untreated and 9 (35%) treatedpatients (Table 1). Seven (15%) of the untreatedpatients had detachment at the last routine visit andat the follow-up visit, and persistent symptoms duringthe interim. We presume that these were persistentdetachments. Similarly, three (12%) of the treatedpatients had presumed persistent detachment. In 30(64%) of the untreated and 14 (53%) of the treatedpatients the presenting episode resolved within oneyear, and there were no subsequent recurrences.Considering both the historical episodes and thedocumented recurrences, we found that about half ofboth the untreated (49%) and the treated (54%)patients had recurrent or presumed persistent centralserous chorioretinopathy.The recurrence frequency of the 45 patients for

whom follow-up information was obtained by thelocal ophthalmologist or by questionnaire agreedclosely with the results obtained on the follow-uppatients. Ofthe 30 untreated patients nine (30%) hadrecurrences during the follow-up period. Of the 15treated patients five (33%) had recurrences. Thus wehave clinical or historical follow-up information on73% of the 105 untreated patients and 79% of the 52treated patients. About one-third of both untreatedand treated patients had a recurrence or presumed

Table I Documented recurrences ofcentral serouschorioretinopathy

Number of recurrences Number ofpatients

Untreated Treated

0 30 (64%) 14 (53%)>-- 1 10(21%) 9 (35%)Presumed persistence 1I year 7 (15%) 3 (12%)Median follow-up time (months) 56 78

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Long-termfollow-up ofcentralserous chorioretinopathy

Table 2 Number ofleakage sites in long-term follow-up ofcentral serous chorioretinopathy

Number of Untreated Treatedleakage sites

Initial visit Follow-up visit Initial visit Follow-up visit

A. All active cases 0 2 2 0 01 36(80%) 29 (64%) 17 (65%) 10 (38%)2 4 10 5 5

:-:-3 3 4. 4 11Total .45 (100%) 45 (100%) 26(100%) 26(100%)

B. Recurrent and persistent cases 0 0 0 0 01 20 (87%) 14 (61%) 9 (60%) 3 (20%)2 3 8 2 2¢3 0 1 4 10

Total 23 (100%) 23 (100%) 15 (100%) 15 (100%)

persistence during the follow-up period. Whenhistorical episodes before the follow-up time areincluded, about half of each group had a recurrenceor presumed persistence.Detachment characteristics. The macula was

detached at the initial examination in all but twoof the patients. Mean detachment size was 2 discdiameters, with a range of 0-75 to 4 disc diameters.The detachments were usually centered about thefovea but often drifted downward with time. Thepatients occasionally reported an upward-movingrelative scotoma. Subretinal yellow precipitates werepresent at some time during the observation in sevenof the 73 patients seen for follow-up. These precipi-tates were absent at follow-up in all 10 patients withpresumed persistent detachment.

Retinal pigment epithelial leakage. The number ofretinal pigment epithelial leakage sites, at initialpresentation and during follow-up, is summarised inTable 2. Initial fluorescein angiograms available for

45 untreated patients showed no discernible leakagein two patients, one leakage site in 36 patients (80%),two leakage sites in four patients, and three or moreleakage sites in three patients. During the follow-upperiod new leakage sites appeared in seven of thepatients with only one leakage site initially; 29 (64%)of the untreated patients had only one documentedleakage site during the follow-up period.Of the 23 untreated patients with recurrence or

presumed persistence 20 (87%) had only one leakagesite initially and three had two leakage sites. Six ofthe patients initially seen with one leakage sitedeveloped other leakage sites during the follow-upperiod; 14 (61%) of the untreated patients withrecurrence or presumed persistence had only onedocumented leakage site. The untreated patients withrecurrence or presumed persistence tended to havesimilar numbers of leakage sites as those with onlyone episode which resolved. Treated patients wereanalysed similarly. Ofthe 26treated patients 17 (65%)

Fig. Iai Fig. lbFig. 1 Fluorescein angiogram showing 'dot' leakage pattern at (a) 26 seconds, (b) 224 seconds.

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had only one leakage site initially, but in the longterm 10 (38%) had only one documented leakagesite. Treated patients with recurrence or presumed.persistence had one initial leakage site in nine (60%)cases but in only three (20%) cases in the long-term.The patients selected for treatment were more likelyto have multiple initial and subsequent leakage sites.The distribution of pretreatment leakage sites is

similar to the previously reported series. "I 14 Of the 45untreated patients with baseline fluorescein angio-grams, leakage sites were distributed as follows:foveal avascular zone, 8; extrafoveal macula (i.e.,one disc-diameter centered about the foveal avascularzone), 27; papillomacular bundle, 4; elsewhere in theposterior pole, 6. Leakage sites in treated patientswere distributed as follows: extrafoveal macula, 16;papillomacular bundle, 5; elsewhere in the posteriorpole, 5. No patient with leakage from the fovealavascular zone was treated.We recognised four distinct types of leakage: a

gradually enlarging dot of hyperfluorescence whichbegan from a site smaller than the diameter of aretinal vein (Fig. 1), pinpoint leakage ascending toform a classical 'smokestack plume' (Fig. 2), diffuseretinal pigment epithelial leakage, and pinpointleakage from a pigment epithelial detachment. Atinitial presentation the 45 untreated patients withangiograms had the following distribution of leakagetypes: dot, 33; 'smokestack,' 9; diffuse, 0; dot arisingfrom a pigment epithelial detachment, 3.We were particularly interested in the location of

the leakage sites in recurrent and presumed persistentdetachments relative to the initial leakage sites. Fourof the 10 untreated patients with documentedrecurrent detachment had leakage from the original

site, three had new sites within 0-5 disc diameter ofthe original, and one had a new leakage site at adistance of 2 disc diameters from the original site. Intwo cases fluorescein angiograms of the recurrentdetachment were not available. Of the seven un-treated patients with presumed persistent detachmentat follow-up five had leakage from the same site andthree had leakage from new sites within 0-5 discdiameter ofthe original site. (One patient had leakageat the original site and from a new site.) Of the 26treated patients only one had recurrent leakage froma treated site. The adequacy of treatment in this caseis questionable owing to the absence of post-treatment scarring. Four treated patients had recur-rent leakage from sites within 0.5 disc diameter of theoriginal site, and four had leakage within the next 0-5disc diameter. In two patients recurrent detachmentwas caused by leakage at sites which had closed spon-taneously without treatment. Presumed persistentdetachments in treated patients were associated withdiffuse, slow leakage from the treatment site in onepatient, new sites within 0-5 disc diameter of theinitial sites in two patients, and a new site within thenext 0-5 disc disc diameter in one patient. One treatedpatient with presumed persistent detachment atfollow-up had leakage from a site which had pre-viously closed spontaneously without treatment.

Complications. The only complication of laserphotocoagulation was the development of relativeparacentral scotomas, to which patients adapted well.No case of treated active CSCR seen for follow-uphad developed choroidal neovascularisation,although Schatz et al. reported 27 cases with thiscomplication. 16

Fig.2Fo Fite. ()2

Fig. 2 Fluorescein angiogram showing 'smokestack' leakage pattern at (a) 21 seconds, (b) 164 seconds.

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Long-termfollow-up ofcentral serous chorioretinopathy

Discussion

VISUAL ACUITYThis study agrees with all previous reports that thevisual prognosis for patients with CSCR, untreatedand treated, is favourable. We were impressed by therange of visual acuities with similar-appearingmacular detachments. Also, despite 'good' final visualacuities, patients were seldom asymptomatic afterresolution of the serous detachment. CSCR is aunique serous macular detachment disorder, follow-ing an often recurrent course without serious loss ofvisual acuity in most cases.

RECURRENCEPrevious CSCR studies have considered recurrencerates and the effect of laser treatment on theserates.2-5 7-13 It is difficult to compare the papers owingto different or unspecified durations of follow-up andto different criteria for recurrence-i.e., documentedand historical. The papers reported recurrence in25% to 50% of untreated patients. In the two mostrigorous retrospective long-term studies Dellaportaand Nanjiani documented recurrences in about halfof each group of untreated eyes." 12 Fewer of ouruntreated eyes had recurrence (21%, all docu-mented), but 15% had presumed persistent detach-ment. We cannot explain the lower recurrence rate,especially over a longer follow-up time. By consider-ing previous historical episodes, documented recur-rence, and presumed persistent detachment we foundthat about half (51%) of the untreated patients had asingle resolving episode of detachment, and abouthalf (49%) had a more complicated course.

In their prospective, randomised trial of ruby laserphotocoagulation Watzke et al. found no significanteffect of treatment on recurrence rate.3 The samplesize was small, and follow-up time was short (median-23 weeks). Nanjiani found that 'about half ofuntreated and treated groups had recurrence. 12Dellaporta concluded from his prospective, non-randomised study of 68 patients that recurrences are3-3 times more likely in untreated than in treatedeyes; therefore he strongly recommended treat-ment. " Landers 13 found a recurrence rate of9% overa median follow-up of 15 weeks in his study of 33 eyestreated with laser phocoagulation. At the 1980 meet-ing of the American Academy of Ophthalmology,Spitznas reported a decreased recurrence rate from32% to 9% in untreated versus treated eyes, respec-tively. Xenon arc photocoagulation, rather than laserphotocoagulation, was used.'5 Most recentlyRobertson and Ilstrup showed in a prospectiverandomised study of 42 eyes that untreated and in-directly laser-treated eyes had a recurrence rate of34% over 18 months, but no eye treated with focal

argon laser photocoagulation had a recurrence.5There are probably two main reasons for the variablerecurrence rates: (1) different follow-up durations,and (2) different treatment techniques (especiallysize of laser spot).

Recognising the weaknesses of retrospectivestudies we believe that our untreated and treatedgroups are similar enough to make conclusions aboutthe effect of direct laser photocoagulation on recur-rence rate. Both groups had reported a previousepisode in about25% ofcases: untreated28%, treated19%. Documented recurrences occurred slightlymore often in treated (35%) than in untreated (21%)patients. This difference might be due to the longermedian follow-up time available for treated patients.Presumed persistence occurred in about 15% of bothuntreated and treated groups. The results of follow-up by the local ophthalmologist and by questionnaireconfirm those ofdocumented follow-ups. Focal argonlaser photocoagulation did not appreciably decreasethe recurrence rate of CSCR in our non-randomised,though comparable, patient groups. Our findings ofrecurrent leakage sites explain how this is possible.The number and relative locations of leakage sites

are important factors which are related to the effectof focal laser photocoagulation on recurrence rate.Our treated patients more often had multiple leakagesites at the initial examination than did our untreatedpatients (35% versus 16% respectively). Multipleleakage sites may have been used as a relative indica-tion for treatment. When the entire follow-up periodfor each group is considered, treated patients stillmore often had multiple leakage sites than did un-treated patients (62% versus 31% respectively).When only the treated and untreated patients whohad recurrence or presumed persistence (80% versus40%, respectively) are considered, this difference isfound to have also existed during the follow-upperiod.

In the 17 untreated patients with documentedrecurrence or presumed persistence leakage wasoccurring at new, adjacent sites about as often as atthe original site. This suggests a disorder of theadjacent retinal pigment epithelium which predis-poses it to leakage. Leakage occurred at treated sitesin only two cases. However, new leakage sitesappeared within 1 disc diameter of original treatedsites often enough to nullify completely the lowrecurrence rate from treated sites. New leakage sitesalways appeared in regions of retinal pigment epi-thelial mottling, but these sites were otherwiseuncharacteristic before leakage appeared. Our studyconfirms that ofSpitznas, '5who showed that recurrentleakages arise adjacent to original sites.

Pretreatment fluorescein angiograms of all activecases showed focal or multifocal dye leakage. In

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agreement with previous studies, most leakage (73%)appeared as slowly enlarging pinpoint dots of hyper-fluorescence. Classical 'smokestack' leakage trans-formed to dot leakage in three cases, but no reversetransformations occurred. 'Smokestack' leakage mayindicate a higher leakage rate and lower density ofthe dye than the subretinal serous fluid. Focal leakagefrom pigment epithelial detachments was present inmany patients. This supports Gass's hypothesis of acommon pathology for pigment epithelial detach-ments and CSCR. 17 We are impressed, however, thatbefore treatment dye always leaked from a site smallerthan typical pigment epithelial detachments, and thepigment epithelial detachments never had diffuseleakage into the subretinal space.

CONCLUSIONThe results of this retrospective follow-up study con-firmed the findings of several previous studies con-cerning epidemiological features, natural course ofuntreated eyes, prognosis for treated eyes, andabsence of difference in final visual acuity betweentreated and untreated eyes. However, we found nosignificant difference in recurrence rate betweentreated and untreated eyes and no significantcomplications of argon laser photocoagulation. Weshall be interested in a follow-up of Robertson andIlstrup's study group4 in five to 10 years.

We are grateful to Maureen Maguire, PhD, for advice regardingdata interpretation and presentation, and also to Judy Belt andRichard Belt for colour photography and fluorescein angiography.Lee Bridgeforth and Lillian Fox assisted in the preparation of themanuscript.

References

1 von Graefe A. Ueber centrale recidivirende retinitis. Albrechtvon Graefes Arch Klin Ophthalmol 1866; 12: 211-5.

2 Leaver P, Williams C. Argon laser photocoagulation in thetreatment of central serous retinopathy. Br J Ophthalmol 1979;63: 674-7.

3 Burton TC. Central serous retinopathy. In: Blodi FC, ed. Currentconcepts in ophthalmology. St Louis: Mosby, 1972: 3: 1-28.

4 Watzke RC, Burton TC, Leaverton PE. Ruby laser photocoagu-lation therapy of central serous retinopathy. Trans Am AcadOphthalmol Otolaryngol 1974; 78: 205-11.

5 Robertson D, Ilstrup D. Direct, indirect and sham laser photo-coagulation in the management of central serous retinopathy.Am J Ophthalmol 1983; 95: 457-66.

6 Watzke RC, Burton TC, Woolson RF. Direct and indirect photo-coagulation of central serous retinopathy. Am J Ophthalmol1979; 88: 914-8.

7 Mitsui Y, Sakanashi R. Central angiospastic retinopathy. Am JOphthalmol 1956; 41: 105-13.

8 Straatsma BR, Allen PA, Pettit TH. Central serous retinopathy.Trans Pac Coast Otoophthalmol Soc 1966; 47: 107-25.

9 Nordholm 1. Central serous retinitis. Acta Ophthalmol (Kbh)1969; 47: 890-9.

10 Klein M, Van Buskirk M, Friedman E, et al. Experience withnon-treatment of central serous retinopathy. Arch Ophthalmol1974; 91: 247-50.

11 Dellaporta A. Central serous retinopathy. TransAm OphthalmolSoc UK 1976; 74: 144-51.

12 Nanjiani M, Long-term follow-up of central serous retinopathy.Trans Ophthalmol Soc UK 1977; 97: 656-61.

13 Landers M. Argon laser treatment of central serous chorioretin-opathy. Ann Ophthalmol 1977; 9: 1567-72.

14 Shimizu K, Tobari 1. Central serous retinopathy dynamics ofsubretinal fluid. Mod Probl Ophthalmol 1971; 9: 152-7.

15 Spitznas M. Paper presented at the Annual Meeting of theAmerican Academy of Ophthalmology, Chicago, 1980.

16 Schatz H, Yannuzzi LA, Gitter KA. Subretinal neovasculariza-tion following argon laser photocoagulation treatment for centralserous retinopathy: complication or misdiagnosis? Trans AmAcad Ophthalmol Otolaryngol 1977; 83: 893-906.

17 Gass JDM. Pathogenesis of disciform detachment of the neuro-epithelium. II: Idiopathic central serous retinopathy. Am J Oph-thalmol 1967; 63: 587-615.

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