Optical Coherence Tomography Demonstrates Subretinal Macular Edema from Papilledema

CLINICAL SCIENCES Optical Coherence Tomography Demonstrates Subretinal Macular Edema From Papilledema

Vincent J. Hoye III, MD; Audina M. Berrocal, MD; Thomas R. Hedges III, MD; Maria Luz Amaro-Quireza, OD

Objective: To evaluate macular changes in eyes with duction in visual acuity. The subretinal fluid appeared papilledema from increased intracranial pressure using to arise from the peripapillary region, and all showed optical coherence tomography (OCT). some improvement in central vision as the fluid resolved. Methods: Fifty-five patients with papilledema seen during 1998 and 1999 were studied with OCT of the optic Conclusions: Subretinal fluid accumulations can cause nerve and retinal nerve fiber layer. Nineteen of these also decreased visual acuity in patients with papilledema. Op- had OCT of the macula during periods of acute, sub- tical coherence tomography can demonstrate subretinal acute, or recurrent papilledema and were evaluated in fluid and can be used to follow the course of this impor- detail for this report. tant visual complication of papilledema.

Results: Seven patients had OCT evidence of subretinal fluid involving the macula. All had some re- Arch Ophthalmol. 2001;119:1287-1290

OSS OF visual acuity from pap- mal findings on neuroimaging scans and illedema or optic nerve head elevated opening pressures on lumbar swelling due to increased in- puncture. Two patients had brain metas- tracranial pressure is uncom- tases, one from breast cancer and the other mon. The visual conse- from testicular cancer. Six patients were quencesL of papilledema usually are limited treated medically with acetazolamide to visual field defects and enlargement of and/or furosemide. One patient received the blind spot.1 Macular abnormalities such corticosteroids and underwent optic nerve as intraretinal macular edema and hemor- sheath fenestration. The 5 patients with id- rhage occasionally cause visual acuity loss iopathic intracranial hypertension were fol- in papilledema.2 Well-documented cases of lowed until there was resolution of the subretinal macular edema in papilledema macular subretinal fluid and stabiliza- are rare in the medical literature.2,3 We used tion of visual acuity (Table). optical coherence tomography (OCT), a All 7 patients with OCT evidence of new technique for imaging the retina, to subretinal fluid had some degree of visual evaluate the macular region in a group of acuity loss (Ͻ20/25) or visual field loss in patients with acute, subacute, and recur- one or both eyes. Visual acuities at initial rent papilledema. examination ranged from 20/25 to 5/200. Seven of 9 eyes had initial visual acuities RESULTS of 20/25 to 20/80. The remaining 5 eyes had visual acuities between 20/200 and 5/200 There was a distinctive space underlying (the eye with toxoplasmosis had a visual the retina in at least 1 eye of 7 patients who acuity of 2/200). Of the 12 patients who had underwent OCT of the macula during an macular OCT findings without evidence of From the Departments of active phase of papilledema. In 2 cases subretinal fluid, visual acuities ranged from Ophthalmology (Drs Hoye, OCT was not evaluated in 1 eye because 20/15 to 20/20, except for 1 eye in 1 pa- Berrocal, Hedges, and Amaro-Quireza) and Neurology of a toxoplasmosis scar in one (case 2) and tient in which there was an altitudinal vi- (Dr Hedges), Tufts University because of recent optic nerve sheath de- sual field defect. School of Medicine, and The compression surgery in another (case 3). Final visual acuity, after treatment of New England Medical Center, Five patients were diagnosed with idio- the papilledema, ranged from 20/20 to Boston, Mass. pathic intracranial hypertension with nor- 20/60, with 6 of 11 eyes achieving 20/30

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 The thickness of the macula and underlying fluid collection in all 12 eyes studied correlated roughly with the PATIENTS AND METHODS degree of visual acuity loss. In the 8 eyes with an initial visual acuity of 20/80 or better, the thickness of the macula and underlying fluid layer measured 370 µm or less (nor- Optical coherence tomography has been used in our 4 clinic to measure changes in the retinal nerve fiber mal macular thickness is 147±17 µm). In the 3 most se- layer to quantitatively follow the clinical course of verely affected eyes, the measurement of macula plus sub- papilledema. We have also obtained images of the retinal fluid layer ranged from 510 to 670 µm (case 4, left macular region in many of these patients. In this study, eye; case 5, both eyes). The eye with the worst vision (left OCT was performed using a prototype instrument us- eye, case 5) had the most fluid present. In some cases fluid ing techniques described elsewhere.4 In addition to appeared confined to the macula (cases 1, 4, and 5) circular scans 3.38 mm in diameter around the op- (Figure 1A and C). However, the others showed appar- tic disc, horizontal scans through the macula were ent communication of subretinal fluid between the macula obtained in some cases. In some cases the scans were and the peripapillary subretinal space (cases 2, 3, 6, and oriented to include the temporal peripapillary space and the macula in the same section. Macular sec- 7) (Figure 1D). Visual fields showed enlarged blind spots tions were evaluated for the presence of a distinct area extending to fixation in 13 eyes tested by perimetry. of absent reflectivity between the retina and cho- In the 12 cases of acute, subacute, or recurrent pap- roid. Macular thickness measurements were made illedema in which subretinal fluid was not seen on OCT from horizontal OCT sections of the retina. of the macula, macular thickness measurements ranged We reviewed the records of 55 patients with pap- from 150 to 210 µm, and visual acuities ranged from 20/20 illedema who underwent OCT of the retina during to 20/50. In those with increased macular thickness greater 1998 and 1999. Most of these patients underwent than 170 µm, there was decreased reflectivity in the pap- OCT of the optic nerve and retinal nerve fiber lay- illomacular retina on OCT, but no definite space was seen ers. Of these, 19 also underwent OCT of the macu- under the retina in all but 5 eyes. Decreased visual acu- lar regions when they had acute, subacute, or recurrent optic disc swelling. The rest had chronic atrophic ity was present in 1 of these cases in which macular thick- or resolved papilledema or underwent OCT of the op- ness was 190 µm. This eye also had significant loss of tic disc and retinal nerve fiber layer only and were visual field and a relative afferent pupillary defect con- not included in this study. A complete neuro- sistent with optic neuropathy. ophthalmic examination was performed on each patient, including visual acuity, color vision (using AOHRR plates), and automated threshold visual field CASE REPORT testing (Humphrey 30-2; Humphrey Inc, Dublin, Calif), except 1 eye that had undergone surgical op- A 30-year-old woman with a 4-month history of head- tic nerve sheath decompression the day previously ache and neck pain developed visual blurring in both eyes. (case 3). Fluorescein angiography was performed in 2 cases (cases 1 and 2). Lumbar punctures were ob- Her visual symptoms consisted of dimming of vision and tained in all patients except one who had cerebral difficulty with color vision. She also noticed visual phe- hemorrhage from brain metastasis, and magnetic reso- nomena reminiscent of “splinters of glass” in her periph- nance imaging or computed tomography was ob- eral vision of both eyes. She weighed 101 kg and was 162.5 tained in all cases. cm tall. Corrected Snellen visual acuity was 20/50 OD and 20/60 OS. The pupils were equal and reactive without a relative afferent pupillary defect. Fundus examination was notable for optic nerve edema in both eyes. or better. The 2 patients with metastatic carcinoma had A peripapillary hemorrhage was present in the right eye initial improvement and then further decline in visual (Figure 2A). The macula of each eye showed some ir- acuity during treatment (cases 6 and 7). regularity of the light reflex. Automated visual field test- Optical coherence tomography in all 7 patients ing (Humphrey 30-2) showed enlargement of the blind revealed subretinal fluid collections posterior to the spot in both eyes, an inferior arcuate defect and a para- macula in at least 1 eye. The fluid collection was present central scotoma in the right eye (Figure 2B), and dense unilaterally in 1 patient (case 1). In 2 patients only 1 eye superior and inferior arcuate defects in the left eye. Com- was evaluated (cases 2 and 3). Subretinal fluid was pres- puted tomography of the head showed no abnormality. ent in both eyes in the other patients (cases 4-7). Clini- A lumbar puncture showed an opening pressure of cally, the foveal reflexes were ill defined in all patients, 23 cm H2O and normal components. In the right eye, OCT and, in those with marked macular thickening on OCT, demonstrated a space separating the retina from the un- there was ophthalmoscopic evidence of elevation of the derlying choroid, suggesting subretinal fluid in the macula peripapillary retina extending toward the fovea (cases 5 of the right eye (Figure 1A). In the left eye, OCT showed and 6). In one case a mild amount of yellowish exudate thickening of the macula but no definite subretinal space. involving the retinal nerve fiber layer in the form of a A fluorescein angiogram revealed no leakage of dye into “macular star” was seen in both eyes (case 4). Fluores- the macula, only late staining of the optic disc (Figure cein angiography in 2 cases showed progressive staining 2C). The patient was treated with acetazolamide, 500 mg, of the optic discs but demonstrated that fluid was not 3 times daily, and furosemide, 40 mg, twice daily, for 5 leaking from the retinal blood vessels or choroid (cases weeks. Her visual acuity subsequently improved to 20/25 1 and 2). OU, the papilledema improved, and follow-up OCT dem-

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Visual Acuity Patient Affected Test Opening Thickness of Macula

No. Sex Age, y Eye(s) Initial Final Performed Diagnosis Pressure, cm H2O and Fluid Layer, µm 1 F 30 Right (left) 20/50 OD 20/25 OD OCT, FA IIH 23 310 OD (20/60 OS) (20/20 OS) 180 OS 2 M 16 Left 20/25 OS 20/20 OD OCT, FA IIH (toxoplasmosis, 38 340 OS (NA, OD) (2/200 OD) (2/200 OS) OD) 3 F 24 Right (left) 20/30 OD 20/20 OD OCT IIH (nerve sheath 55 310 OD (NA, OS) (Hand motions (20/30 OS) surgery, OS) OS) 4 F 15 Both 20/50 OD 20/20 OD OCT IIH 50 290 OD 20/200 OS 20/20 OS 510 OS 5 F 48 Both 20/400 OD 20/50 OD OCT IIH 28 610 OD 5/200 OS 20/60 OS 670 OS 6 F 54 Both 20/30 OD 20/30 OD OCT Metastatic cancer 55 300 OD 20/30 OS 20/60 OD to brain 320 OS 7 M 36 Both 20/40 OD 20/40 OD OCT Metastatic cancer Not obtained owing 310 OD 20/80 OS 20/100 OS to brain to hemorrhage 370 OS

*OCT indicates optical coherence tomography; FA, fluorescein angiography; IIH, idiopathic intracranial hypertension; and NA (not available), the OCT was not evaluated (owing to toxoplasmosis) or not performed (owing to previous surgery).

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38 dB 43 dB C D Log Reflection Log Reflection Figure 1. A, Optical coherence tomograph (OCT) of the macular region of the right eye (case 1). B, OCT of the macular region of the right eye (case 1) one month after treatment. C, OCT of the left eye (case 4) showing apparently localized subretinal fluid in the macular region. D, OCT of the left eye (case 6) showing separation of retina between the optic disc and the macula.

onstrated resolution of the subretinal fluid in the macula lar retinal nerve fiber layer edema.11,2 Retinal pigment epi- of the right eye (Figure 1B). thelial changes consistent with chronic macular edema may also be seen.3 Additionally, subretinal neovascular 12,13 COMMENT membranes may result from papilledema. Optical coherence tomography demonstrated sepa- Papilledema causes several different effects on the vi- ration of the retina from the underlying choroid by sub- sual pathways. Chronic papilledema can lead to progres- retinal fluid in association with decreased visual acuity sive loss of the optic nerve fibers.5 This is associated with in 7 cases. In some cases the subretinal fluid appeared characteristic arcuate visual field defects, especially in the to be localized to the subfoveal region (cases 1, 4, and inferior periphery, along with nerve fiber layer loss more 5) but in others, there appeared be continuity between superiorly.6 Occasionally, loss of central vision may be the subfoveal region and the optic disc, suggesting that related to a sudden ischemic episode in an already edema- the subretinal fluid came from the optic nerve heads (cases tous nerve.7 2, 3, 6, and 7). The absence of fluorescein accumulation Visual acuity is usually preserved in patients with in the macular region indicates that it did not come from papilledema. Macular abnormalities that may affect vi- the retinal or choroidal vasculature. In many of the other sion include choroidal folds,8 hemorrhages,9,10 or macu- patients who had OCT of the retina during active pap-

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30°

Figure 2. Case 1. A, Fundus photograph of the right optic disc. B, Visual field examination of the right eye. C, Fundus fluorescein angiogram of the right eye.

illedema in which there was no distinct separation of the While other macular abnormalities do occur, improve- retina from the choroid, there was thickening of the macu- ment of visual acuity in our patients after resolution of lopapillary retina, suggesting intraretinal edema in these the subretinal edema further supports a link between vi- cases. However, this did not seem to affect visual acuity sual acuity loss and the presence of subretinal macular as much as the subretinal fluid appeared to do in the 7 edema in some patients with papilledema. patients in whom OCT showed separation of the retina from underlying choroid. Accepted for publication March 8, 2001. In 6 cases, we felt that acetazolamide was appropri- Corresponding author and reprints: Thomas R. Hedges ate initial therapy (cases 1, 2, 4, 5, 6, and 7). All but the 2 III, MD, 750 Washington St, Box 381, Boston, MA 02111. patients with metastatic carcinoma responded well, showing resolution of the subretinal fluid and improvement in visual acuity within a few weeks. One patient had more REFERENCES severe and acute visual loss, and we chose optic nerve sheath fenestration in the more severely affected eye as the 1. Savino PJ, Thompson HS, Kansu T, et al. Visual loss in pseudotumor cerebri: primary treatment. She received acetazolamide in the post- follow-up of 75 cases from 5-41 years and a profile of 14 patients with perma- nent, severe visual loss. Arch Neurol. 1982;39:461-474. operative period until resolution of macular edema and 2. Morris AT, Sanders MD. Macular changes resulting from papilloedema. Br J Oph- papilledema occurred with restoration of visual acuity. thalmol. 1980;64:211-216. Carter and Seiff14 demonstrated a similar response to op- 3. Gittinger JW, Asdourian GK. 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