| OCR Text |
Show /. Clill. NClIro-ol'lllhalmo!. 5: 45-53, 1985 Juxtapapillary Subretinal Hemorrhages in Pseudotumor Cerebri JAMES R. COPPETO, M.D. t\tARIO L. R. t\10NTEIRO, M.D.* Abstract We report two cases of pseudotumor cerebri with subretinal hemorrhages. Bilateral juxtapapillary subretinal neovascular membranes were present in one. A unilateral hemorrhage occurred adjacent to a small anomalous optic disc in the other. Subretinal hemorrhages in papilledema may occur from juxtapapillary subretinal neovascular membranes that evolve rapidly and then involute. The relationship of the appearance and subsequent evolution of such juxtapapillary subretinal neovascular membranes to the course of the intracranial pressure is discussed. Occurrence in one eye may presage occurrence in the other eye, making control of intracranial pressure important once one eye is afflicted. Fortunately, severe permanent visual loss remains unreported. Some anomalous small optic discs may be particularly at risk for subretinal hemorrhage from papilledema because preexisting axonal crowding is aggravated by the swelling of the optic nerve from elevated intracranial pressure. Conditions related to the Valsalva maneuver that chronically or intermittently elevate intracranial pressure further and produce elevated cephalic venous pressure should be avoided. Introduction A wide variety of intraocular hemorrhages may occur with papilledema (optic disc edema secondarv to elevated intracranial pressure). The most uncommon is subretinal peripapillary hemorrhage. It is not even mentioned in a review of unusual ucular hemurrhages in papilledema, and A"uClate Chnlcal Pwfe"ur of Ophthalmolugy ORC) and Fellu\\' In :\euru-uphthalmology (MLRM). Univef'ity of Connecticut MedICal Centc'r. Farmington, Connecticut; 'lI1d Dl'partment uf Ophthalmology ORC). 51 MarY', Ml·dieal Centc'r, Waterbury, Cunnecllcut • Dr. Monteiro was a fl,llow In Neuw-ophthalmology at the Department of Ophthalmulugy. 11,"pital da' Chnic,l'. University of Sao Paulu Medical School. <',10 Paulo, Rr,lIIl. March 1985 a rl'cent repurt uf subretinal hemurrhage from a ju-..tapapillary subretinal neuvascular membrane in chronic papilledema concluded that the juxtapapillary subretinal neovascular membrane and the elevated intracranial pressure were coincidental findings. I ~ Only three other cases of papilledema have been reported with subretinal hemorrhages from juxtapapillary subretinal neovascular membranes, but the relationship of the evolution of the juxtapapillary subretinal neovascular membranes to the course of the intracranial pressure is unclear3 , We report two cases of pseudotumor cerebri with subretinal hemorrhages. One case was bilateral, and like the only other reported bilateral case, was associated with rapidly evolving and subsequentlv involuting juxtapapillary subretinal neovascular membranes; first in one eye, and shortly thereafter, in the other. 4 Neither' eye received photocoagulation treatment so we' were able to trace the natural history of the condition. Fluorescein angiographic findings throughout the evolution of this case are analyzed. In another case, a juxtapapiliary subretinal neovascular membrane was not demonstrated; unilateral subretinal hemorrhage occurred adjacent to a small uptic disc. As in the previously rt'ported cases, both of uurs were virtually asymptt1matic until subretinal hemurrhage occurred. The hemorrhages appeared to be precipitated by Valsalva maneuver associated with severe bouts of coughing Such a phenumenon has not been reported previlluslv. Case Reports Ca~c 1 A 5R-year-old, 210-lb. woman was examined in May 19RO because of sudden visual loss, left eye. She had a I lh-year history of pseudotumor cerebri (spinal fluid pressures repeatedly over 350 mm). She had recently had an upper respiratory tract infection associated with constant cough. 45 Subretin.lll !l'mllrrh,lMl's in ''.lpilkdl'm.l Figure 1, Case 1, May 1980. Optic dIsc edema, left eye, with subretinal hemorrhage and exudate. She smoked two packs of cigarettes a day. During a paroxysm of severe coughing, she suddenly noted visual loss in the left eye which began as a paracentral scotoma and impaired central vision occurred within 24 hours. Review of systems was positive for mild chronic headache, intermittent dizziness, and transient bilateral obscurations of vision associated with sudden changes in position. Previous attempts to control her pseudotumor cerebri with corticosteroids, diet, acetazolamide, and frequent lumbar punctures had failed. She had refused to stop smoking and refused a lumboperitoneal shunt. Her only medication, in May 1980, was acetazolamide 250 mg three times a day. Her visual acuity was 20/30 right eye and 20/ 200 left eye. Pupillary appearance and reactions, intraocular pressures, and peripheral visual fields were normal. The right optic disc was mildly edematous. The left optic disc was moderately edematous. There were circumferential folds in the adjacent retina. Temporal to the left optic disc was an e'\tensive hemorrhage beneath the sensory retina o.tending through the macula and associatl'd with a white lesion beneath the fovea, resembling an e,udate (Fig. I) FlullreScl'in angiography confirmed a jux- 46 tapapillary subretinal neo\'ascular membrane in the left eye (fig. 2). There was no significant serous subretinal tluid. There was no autotluorescence of either optic disc. Disc edema right eye was confirmed by the presence of tluorescein dye leakage. Over the ne,t 16 months the hemorrhage cleared, and \'ision improved to 20/25, left eye, despite cerebrospinal tluid pressure persistently o\'er 400 mm of spinal fluid and worsening optic disc edema in both eyes. The juxtapapi11ary subretinal neo\'ascular membrane was small and fibrotic. There was no serous detachment of the retina. The patient still refused a lumboperitoneal shunt. In December 1981, small areas of peripapi11ary ne'l\'ascularization were noted nasally in the right eve and inferiorly in the left eye (Figs. 3 and 4). A lumb'lperit,1neal shunt was again refused. In February 1982, she developed pneumonia .lssociated with constant severe coughing. Fol1Ll\\' ing a bout llf coughing, she noted visual loss in the right eye. On examination in March 1982, vision was 20/200 right eye and 20/25 left eye. She now had a large subretinal hemorrhage, right eye (Fig. 5). The patient requested and received a lumboperitoneal shunt. Over the next 6 months, the Journal of Clinical Neuro-ophthalmology March 1985 Figure 2, Ca,t' I, 1.1\' 1llHO, Flullre,ceJn an~illgram; ,ubrelinal nt'll\',,,cular mt'mbrane at tht' ,upt'rllkmpLlral bllfder lli thl' llpllL di,c. Idt eve Figure 3. (.''1' I, Ilt-n'mbl'r 14H I Sm,ll1l"\I,lp,'pill.H\' 'ubrdina' nl'll\',lSnll,lr membr,lnl', right l'\'l' Coppl'lo, Monteiro 47 Figure 4, Cbl' I. December 1981 l\ie'" ,mall lu'\tapaplilarv ,ubrdmal ne,l\'a"ular membrane at I"\\'l'r pule "i "ptlL d,,,. kIt l'Vt' optic di~c edema cleared in both eyes, the subretina) hemorrhage resolved in the right eye, ilnd the ~mall juxtJpapillary subretinal neovilscular membrane inil'riur tu the Jdt optic disc n.1grt'sst'd. Fluure~cein ,1I1giugraphv in Nuvember 1q82 dearly den)(>n~trakdthe ju'\tapapillarv subretinal 11l'(l\',;~cular membral1l' in the right' evt' which 48 had been obscured by subretinal hemorrhage 8 1l1llnths t'arlier (Fig. 6). There was no serouS dt'tachment l)f the retina. The neovascular net i1Ullresct'd, but the optic disc itself did not leak. reilecting improvement of the papilledema. Visual ,'Cuity improved to 20/25, right eye, and there has been no change in either eye since that time. Journal of Clinical NeuTo-ophthalmology Coppeto, Monteiro Figure 6. Case L Nllvember 19H2 Hemllrrhage cleared, nght eye )uxtapaplllarv subrehnal nellvascular membrane nllW visible. Figure 7. Case 2. OptIC dISc l'dl'ma, right t'Yt' with subretin,ll hl'mllrrh,'gt'. Both optic discs have remained flat with sharp margins. Case 2 A 30-vear-old woman had an upper respiratory tract inf~ction associated with persistent and se- March 1985 Vl'rl' coughing. During one particularly severe coughing spell, she felt as if she were 'strangulating." Immediately thereafter, she noticed an alteration in her vision that she found difficult to describe. Past history and review of systems were negative except for mild hypertension. 49 Figure 8. Cast' 2. Flul.rescein angil'graph of optic disc. right eve )u,tapa· pillary subretinal nt'ovascular membrane is not apparent. On examination, her blood pressurt' was 120/ 80. Visual acuity was 20/20, right eye <1nd 20/ 20+2, left eye. Ishihara color card testing was normal both eyes. Visual fields were normal ex- 50 cept ior enlarged blind spots. Otherwise, ophthalmologic and neurologic examinations were positive only for a slightly decreased right arm swing when walking, bilateral disc edema, and a sub- Journal of Clinical Neuro-ophthalmo)ogy Coppeto, Monteiro Figure 10. C~se 2. Res,'!vin" llplic disc edema, ri~ht eye; residual pseudopapilledema. Figure 11. Case 2. Rl''<,!ving optic di,c edem,l, Idl eye retinal juxtapapillary hemorrhage in the right eye (Figs. 7-9). Lumbar puncture revealed an opening pressure of 390 mm of cerebrospinal fluid. She was treated with corticosteroids and weight reduction. Her March 1985 papilledema abated within 5 months, and the peripapillary hemorrhage cleared (Figs. 10 and 11). It was now apparent that her right optic disc was small and anomalous. There were no visible drusen, and the optic disc had not autofluoresced 51 pn previous iluprl'scein angiowaphy. A cranial CT 5C,1(1 did npt shpw drusen of thl' diSc. The left llptic disc rem,linl'd ilat with sharp margins and the right disc rl'lained its distinctivl' appl'arance 2 years 1,1lL'r. Discussion SubrL'linal Ill'mllrrhages in papilledema are exceedinglv unCllmmlln: They may occur with or withllut llbvious juxta papillary subretinal neovascular membranes.: , There are two previous reports of juxta papillary subretinal neovascular membranes occurring near lltherwise normal optic discs involved by chronic papilledema. jamison reported the rapid evolutilll1 oj a juxta papillary subretinal neovascular membrane in the right eye of a case of bilateral papilledema.' The juxtapapillary subretinal neovascular membrane continued to enlarge despite rapid normalization of the intracranial pressure. The patient underwent photocoagulation of the juxtapapillary subretinal neovascular membrane because of increasing subretinal fluid. Treatment apparently produced a moderate decrement in visual acuity. The left eye of that case did not develop a juxtapapillary subretinal neovascular membrane probably because the pseudotumor cerebri responded so quickly to therapy. It is impossible, from jamison's case, to draw conclusions about the full natural history of juxtapapillary subretinal neovascular membranes in chronic papilledema. By contrast, the case described by Morse et aI., like our case 1, suffered from more recalcitrant pseudotumor cerebri, and developed bilateral juxtapapillary subretinal neovascular membranes. In one eye, the juxtapapillary subretinal neovascular membrane spontaneously resolved, but the other eye received photocoagulation treatment to the juxtapapillary subretinal neovascular membrane because oj a persistent small amount of subretinal fluid. It is possible that the juxtapapillary subretinal neovascular membrane in the treated eye eventually would have involuted without treatment and without serious sequelae. Our case is of interest because it documents the development of sequential juxtapapillary subretina I neovascular membranes in both eves oj a patient with severe and recalcitrant pseudotumor cerebri and demonstrates that, despite extensive subretinal hemorrhage and exudation and persistence of severely elevated intracranial pressure, a sizeable juxtapapillary subretinal neovascular membrane spuntaneously involuted. In our case, subretinal fluid was also relatively sparse. Our case, and that of Morris and Sanders, illustrate that despite extensive striae and severe subretinal hemorrhage and exudation, untreated juxtapapil- 52 lary subretinal neovascular membranes in chronic papilledema may have only limited growth potential. and secondary complications may resolve with few residua.' We could find no documented casl' of a slowly progressive decrement in vision owing unequivocably to the accumulation of subretinal fluid in this disorder. If, as appears to be the case, these juxtapapillary subretinal neovascular membranes have little potential to grow far from the optic disc and tend to spontaneously invulutl' and fibrose, the indications for photocoagulation of these lesions may be far more limited than for other juxtapapillary subretinal neuvascular membranes. The juxtapapillary subretinal neovascular membrane in the right eye of our case 1, evolved rapidly This was also characteristic of the case of jamison and Morse et aI., wherein a juxtapapillary subretinal neovascular membrane evolved within a period of weeks.' 4 At the time that the juxtapapillary subretinal neovascular membrane right eye hemorrhaged in our case 1, there appeared to be a similar juxtapapillary subretinal neovascular membrane developing left eye, but it was transient and abortive. Thus, case 1's left eye had a previous juxtapapillary subretinal neovascular membrane at the upper pole of the disc, and later was developing one at the lower pole of the disc. This is the first report of the occurrence of a second juxtapapillary subretinal neovascular membrane in a single eye of a patient with pseudotumor cerebri. The incipient juxtapapillary subretinal neovascular membrane left eye probably involuted partly as a result of the control of the intracranial pressure, and the juxtapapillary subretinal neovascular membrane in the right eye appeared in the context of worsening papilledema. However, the severitv and course of elevation of intracranial pressure is' probably not the most important factor in the progression oj an established juxtapapillary subretinal neovascular membrane, because papilledema left eye was actually worsening as the subretinal hemorrhage there was resolving and the juxtapapillarv subretinal neovascular membrane involuting.' The cases of jamison and Morse et al. dL)(umented enlargement of the juxtapapillar~' subretinal neovascular membranes despite resolving papilledema:' 4 Therefore, it appears that elevated intracranial pressure is necessary for the initiation of the juxtapapillary subretinal neovascular membranes in these cases. Once initiated however, they may progress to a limited extent despite contnll oj the intracranial pressure, or regress despite persistence of the papilledema. Our two cases suggest two factors that may contribute to subretinal hemorrhaging with papilledema. First, chronic coughing probably produces additional engorgement of the optic disc Journal of Clinical Neuro-ophthalmology and aggravates peripapillary isdll'mia. 7 Second, if an anom,llous optic disc h,lS CWWdl'd lll'ur,ll elements, it m,1\' predispl1sl' to ischemi,l in p,lpilledem, l. Twost d ,11.'s C,lse is rekv,1I1t in this reg,ud. That p,ltient Iud pseudl1tun1llr cerebri ,1I1d a unilateral juxtap,lpillarv subretinal llt'ovascular membrane.' Review llf the photl1graphs of till' optic discs and the fluorescein angiogr,lm suggests tll us th,lt till' disc with the neov,lscular membrane W,lS small ,1nd anllm,llous. Th,lt case, like llur case 2, may have h,ld both psCUdOp,lpilledem, l and p,lpilledem,l. r-.tllrse et a!. pl'StUlated that in pSl'udotumor cerebri. there is ischcmi,l llf the peripapillary retina ,1nd chllrllid frllm crl1wding llf vascular and neural elements· Distllrtion llf Bruch's membrane may llCClH ,1nd new \'essels gain access to the space beneath the senSlln' retina and continue to grllw as a result llf ischemia. A small anomalous llptiC disc may be particularly prone to such ischemia: patients with llptiC disc drusen can have subretinal heml'rrhages with llr without juxtapapill. 1rv subretinal neovascular membranes'" When \'ision deteriorates in the face of subretinal heml'rrhages in papilledema, one should not assume that continuous growth of the juxtapapillan' subretinal neovascular membrane will llLCur and that subretinal fluid is an indication for photocoagulation. The diminished vision may be secondary to hemorrhage, retinal striae, or exudates from blood products. Subretinal fluid may simply be the residuum from dissolving subre- March 1985 Coppeto, Monteiro tinal blood. Such juxtapapillary subretinal neov, lscular membranes probably can be managed ClmserV,l ti vel y. Nonetheless, we believe that a vigorous attempt to reduce intracranial pressure is logical ,1I1d indicatl'd with a monocular juxtapapillary subretinal neovascular membrane is detected in a patient with chronic papilledema. We would also recommend that Valsalva-Iike maneuvers be avoided and smoking interdicted in patients with chwnic papilledema. References 1. Keane, j.R: Papilledema with unusual ocular hemllrrhages. Arch. aphtha/mol 99: 262~263, 1981. 2. Troost, BT., Sufit, RL.. and Grand, M.G: Sudden munllcular visual luss in pseudotumur cerebri. Arch. Nfl/wi 36: 440-442, 1979. 3. jamison, R.R.: Subretinal neovascularization and papilledema assuciated with pseudotumor cerebri. Am. f. aphtha/mol. 85: 78-81. 1978. 4. Murse, P.H., Leveille, AS, Antel, jP., and Burch, j.V.: Bilateral Juxtapapillary subretinal neovascularization associated with pseudotumor cerebri. Am. I aphtha/mol. 91: 312-317, 1981. 5. Murris, AT., and Sanders, M.D.: Macular changes resulting from papilloedema. Br. f. aphtha/mol. 64: 211-216,1980. 6. Harris, Mj., Fine, S.L.. and Owens, SL Hemorrhagic complications of optic nerve drusen. Am. f. Ophtha/mol 92: 70-76,1981 7. Victor, 01, and Welch, R.B.: Bilateral retinal hemorrhages and disc edema in migraine. Am. f. Ophtha/mol. 84: 555-558,1977. 53 |
