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Show /. Clin. NCUfll-ophth,l/nlll/. 1: 135-140, Iqt'I. Quadrigeminal Plate Compression Caused by a Pulsion Diverticulum ASHWANI KAPILA, M.D. TERENCE G. KLINGELE, M.D. THOMAS L. 5LAMOVIT5, M.D. RONALD M. BURDE, M.D. ROBERT RATCHE50N, M.D. MOKHTAR H. GADO, M.D. Abstract A 35-year-old woman presented with progressive visuai loss. She was found to have an ependymoma causing an obstructive hydrocephalus with a pulsion diverticulum. Intermittent expansion of this diverticulum was associated with the development of a Sylvian aqueduct syndrome. Obstructive hydrocephalus producing distension of the third ventricle and compression of the anterior visual system is rarely seen clinically, I although it has been demonstr~ted with autopsy material': Similarly, distension of the ventricular sYstem has been i~plicated as a cause of disturbed v~rtical eye movements.': I We recently had the opportuni'ty to see a patient in whom obstruction of outflow from the third ventricle, associated with marked enlargement of the third and lateral ventricles, initially produced anterior visual system dysfunction. Subsequently the patient developed an eye moyement disturbance which we believe was caused by a unique hydrostatic mechanism. Case Report This 35-year-old woman was referred to the Neuro-Ophthalmology Consult Office at Washington University Medical Center on April 21, 1980. Approximately 1 year before, she began having episodes of blurred vision, lasting a few seconds and almost always occurring after rising from a sitting or lying to a standing position. She had approximately 10 of these episodes over a period of 1 month when they spontaneously disappeared. Approximately 2 months prior to being seen by us From the Departments of Ophthalmology (TCK, TLS, RMB). Neurology (RMB), Neurological Surgery (RMB, RR). "nd R"diology (AK, MHG), WashIngton Unive"ity School of Medicine, St. Louis, Missouri. June 1981 she noted the onset of episodes of more abrupt, more serious loss of vision in both eyes lasting 35 seconds, that continued for the next 6 weeks, at which time she noted that she was having difficulty driving. She described her visual difficulty as a constant blurring not associated with episodes of visual blackout. She thought that the vision in her right eye was worse than her left eye. In addition, she had been experiencing moderately severe headaches over the past 6 months. These headaches were dull and continuous, located in the occipital region. She had been treated by a chiropractor with some subjective improvement. Her past medical history, family history, and review of systems were unremarkable. Examination revealed a best corrected visual acuity of 20/50 in the right eye and 20/30 in the left eye. On color vision testing with AO pseudoisochromatic plates, the patient could identify only 1 out of 15 plates with the right eye and 4 out of 15 with the left eye. Intraocular pressures were normal Fundus ex~mination revealed markedly swollen, elevated discs in both eyes with the bright bodies and gliotic changes of chronic p,lpilledema (Fig. 1). The maculas appe,ued essenti'llly norm,ll Kinetic visual fields (Fig. 2) reve,lled ,1 ne.uly ,lltitudinal defect with ,lbsence of reSpl)nSe in the superior n,lsal quadrant in the right eye and ,1 central scotoma with an enl.uged blindspot in her left eye. Both fields demonstrated gener,llized constriction. On admission, thin section CT SC,lllS revealed the following. Tr,lllsverse sections showed ,1 contrast enhancing tumor 1ll,1SS in the posteril)r ,llld mid-portion of the third ventricle. The ,lllterior portion of the third ventricle, rostral to the for,lmen of Munro, was free of tumor (Fig. 3b), and the dilated anteroinferior end of the third ventricle extended into the sella turcica (Fig. 3a). There was severe enlargement of the lateral ventricles, and an inferiorly and medially directed diverticulum of the right lateral ventricle was identified (Fig. 3c), displacing the internal cerebral veins downward 135 Quadrigemin.ll Plate Compression ~d) lin Figures 1.1 and lb. Right and left optic discs demonstrating elevation with bright bodies and gliotic changes of chronic papilledema. LEFT RIGHT Figure 2. Right eye: Superior altitudin.ll defect crossing the vertic.llmeridion with a relative central scotoma. Left eye: Marked enlargement of the blindspot with .1 relative centr.ll snltllm.1. and contralaterally across the midline. Sagittally reconstructed images (Fig. 4) confirmed the intrasellar extension of the ballooned anterior third ventricle and the extension of the lateral ventricul.u diverticulum into the quadrigeminal cistern (Fig. 4). The direct coronal scan (Fig. 5) showed the diverticulum arising from the region of the right ventricular atrium and extending downward towdrd the collicular pldte. Carotid dngiogrdphy demonstrated vdsculdr displacement secondary to L1ter.l1 ventricular enlargement, but the tumor itself was avascular. Eight hours after the angiogram, the patient was noted to be drowsy, and pupillary light-near dissociation and paresis of upgaze were noted. The patient underwent an emergency ventriculostomy. Five minutes later the patient was much more alert, and within 30 minutes after the procedure im- Journal of Clinical Neuro-ophthalmology Kapil.J, Klingele, Siamovits, Burde, R.Jtcheson, Gada H'I Figure 3. Tr,msverse CT scans of the he.Jd after intr,lVenou., Cllnlr.lst injeLlilln. (.1) The dil.lted .lnt£'rillr end 01 the third ventricle occupies th£' suprasellar space. (b) The Cllntr.Jst enh,lI1cing tumor lies within Ihe third ventricle. The cerebrospinal fluid in the ventricle caps the ant£'rior end of th£' tunlllr. (e) A diverticulum .Hising frl)m the inferomedial aspect of th£' right lateral ventricl£' displaces th£' internal cer£'!:>r.J1 veins tl) the I£'ft side. provement in upgaze was evident. By the next morning the pupillary reactions and extraocul.H movements were normal. Four days later the patient underwent cr.miotomy via a transcallosal approach. The mass W.lS removed from within the third ventricle and proved to be an ependymoma. With the exception of a postoperative right Horner's syndrome and two grand mal seizures on day L the patient waS essentially normal until the third day after surgery when she again had progressive limitation of up- June 1981 gaze, light-ne.H dissociatilH1 of her pupillary reactions, .md impairment of (l)J1SCil)USness. Following .1 ventricuI.H-.1tri.11 shunt, the p.ltient's level of consciousness .1I1d pupill,lry re.lCtions immediately returned to norm.11. Her upg.1ze paresis slowly resolved over the next 2 weeks. At the time of discharge the patient's best corrected visual acuity was 20/100 in the right eye .1I1d 20/30 in the left eye. The p,ltient's ViSU.11 field defects had become more pronounced. The papilledema gradually resolved, and although there were chronic changes, 137 Qu.ldrigemin.ll !'I.lte Compression the optic discs remained relatively well vascularized. Radiation therapy was initiated for minimal residual tumor within the third ventricle. Discussion This patient's visual symptoms, the accompanying visual field defects with diminished central acuity, and defective color vision point directly to a disturbance of the anterior visual system. Her papilledema, while quite pronounced with chronic features (bright bodies and gliosis), did not appear to be sufficiently atrophic in character to explain the significant visual field loss or reduction of visual acuity. The striking dilatation of the anterior Figure 4. Sagittal reconstruction of the axial scans. (a) Reconstructed image close to the midline. (b) Schematic drawing of the reconstructed image in Fig. 4.1. 1. Dilated anterior end of the third ventricle impinging upon and occupying the sella turcica. 2. Contrast enhancing tumor. 3. Diverticulum of the inferomedial aspect of the right lateral ventricle at the junction between the body and trigone. 4. Clivus. 5. Occipital bone. third ventricle with herniation into the chiasmatic cistern and pituitary fossa with pressure on the optic nerves .md chiasm (Fig. 4) would appear to be the mL)st lilo..ely cause of her visual disturbance. This mechanism' has been previously describedl and demonstrated at autopsy.~ Upgaze paresis associated with light-near dissoci. ltion .He two of the cardinal signs of the Sylvian aqueduct or Parinaud's syndrome.:l Transient Parinaud's syndrome has been reported in association with aqueductal stenosis.:J-7 It was suggested in these Cases that ballooning of the suprapineal recess resulted in compression of the posterior commissure and tectaI plate. A congenital aqueductal diverticulumH also has been reported to cause a journal of Clinical Neuro-ophthalmology KapiL], Klingele, Slamovits, BurdE', RatchE'son, Cado Figure 5. Direct coronal scan of the head. Note a diverticulum (ll arising from the inferomedial aspect of the right lateral ventricle. loss of upward gaze associated with impaired pupillary reactions. The finding of Parinaud's syndrome in our case, unlike those previously reported, is most adequately explained by direct pressure of the pulsion diverticulum of the right lateral ventricle on the tectaI plate. The diverticulum was demonstrated radiographically. It extended downward from the medial atrial wall of the lateral ventricle, through the tentorial hiatus into the posterior fossa over the quadrigeminal plate. Pulsion diverticula of the lateral ventricle are most frequently secondary to a subpial rupture" of the ventricle wall or to a protrusion of the ventricular ependymoma III through the posteromedial aspect of the trigone."-17 The rupture or protrusion is usually caused by obstructive hydrocephalus secondary to ventricularl~.l7 or midbrain"·I,.l', tumor, aqueductal stenosis l'.I•. l'; or atresia of the for.:Jmen of Munro. 11 Pulsion diverticula are gener.:JlIy asymptomatic. Thus, they are different from the diverticulations related to focal cerebral lesions associated with loss of tissuel".I" or false diverticula,~II.~l both of which usually are associated with neurologic deficits due to parenchymal damage. Our case is .:J unique example of a pulsion diverticulum associated with definite neurologic findings. We suggest that the light-near dissociation of the pupillary reactions and paresis of upward gaze were caused by the June 1981 hydrostatic pressure of the lateral ventricular diverticulum impinging on the quadrigeminal plates. References 1. Osher, R.H., Corbett, ]'j., Schatz, N./.. Savino, P.j., and Orr, L.S.: Neuro-ophthalmological complications of enlargement of the third ventricle. 8r. r. OphthaJmoJ. 62: 53b-542, 10 78. 2. Lindenberg, R., Walsh, F.B" and Sacks, r.G.: Neurop.: JthoJogy of Vision: An At/.IS. Lea & Febiger, Philadelphia, 1073, p. 230 3. Swash, M.: DisLHders of OCU!.H mllVE'ment In hydmcephalus. ['roc R. Soc. MeJ. 69: 480-484, 1070. 4. Lerner, M.A., Kosary, I.Z" .1I1d Cohen, B.E.: P.uinaud's syndrome in aqueduct stenosis: Its mechanism .1nd ventriculographic fe.ltures. 8r. r. R.1JioJ. 42: 310-312, 1%0. 5. Swash, M.. Periaqueduct.ll dvsfunction (the Sylvian aqueduct syndromE'): A sign llf hvdmceph.llus? 1. NcuroJ. Ncurosurg. l'svchi.ltrv 37: 21-2t" 1074. b. Sh.ll1dt, R.F., f'.lwl, R.P., .1I1d !Prv.l, M.I.: Significance of upward g.lZE' palsy (['-Hin.lUd's svndrllme) in hydroccph., lus due to shunt mdlfunction. r. Neurosurg. 38: 717-721, 1073. 7. Hdmer, ,., and Martin, K.: f'arindud-Syndrom Bei Nicht tumllrbedingter hydrozephdler hirndruckstcigerung. NeuropeJi.Jtric 7: 217-223, 197b. 8. Fredericks, E.j., dnd V.lIlNuis, C: Diverticulum of the rostrdl cerebral aqueduct with ocular dysfunctions. Arch. NcuroJ. 16: 32-3b, 19b7. 9. f'ennybdcker, /.. dnd Russell, OS: Spontaneous ven- 139 Qu,lllrigelllin,ll 1'I,IIp Compression Iricul.lr ruplure in hydroc('ph,llus wilh subtpnlori..ll cyst form,ltilln. /. Neur"/. I'svl'hi"try 6: 31\-45, 1943. III Leslie, LV., .md Au...(' r, (;,1., Jr.. Sponl,lJ1eous v('ntriclIlostollly. 1~,ldio/"gy 83: l,1\3-t,l\t" 19b4. II Alonso, A., T,lbo,ld,l, I)., Alv..Hcz, '.1\., 1'..H,lmO, C, .Ind Vil,l, M.: Spont.mel'us ventriniloslomy .md ventrinil.. H divertil·ululll. 1~.Jdi<l/"gv 133: t,51-b54, 1979. 12. Childe, A.E., .lIld Ml-N.lllghton, F.L.: DiVl'rtirul.l of the I.IIN,II venlricles exlending inl" Ihe n'rebell..H fl'ss.l. An·h. Neunl/. I'svchi,ltry 47: 7bl\-771\, /942. 13. Dvke, c.e.: An]lIired subl(·n!llri.,1 pressure diVl'rlicululll l,f .1 cerebr,ll I.ller..l1 v('nlric!e. 1<,Jdi"/"gy 39: Ib7-174, Ill42. 14. M,KF..IrI,lJ1p, W.V., ,md Fillconer, M.A.: Diverliculum of thp 1,Itprai ventricl(' pxtending into the posleril'r cr.1I1ial foss.l: Report of .1 c,lse succ('ssfully relievpd by operation. I Neuro/. Neurosurg. T'sychi. ltry 10: 101-lOb, 1947. IS. Sweet, W.H.: Spontaneous cerpbr..ll ventriculostium. Arch. Neuro/. Psychidtry 44: 532-540, 1940. lb. Zilkha, A.: Spontaneous venlriculostomy. R..Idio/ogy Ill: b33-b37, 1974. 17. ppnfipld, W.: Diencephalic .lllionomic ppilepsy. Arch. Neuro/. Psychi..ltry (Chic) 22: 351\-374, Ill29. 11'1. Adams, R.D.: Diverliculation of the cerebral ventricles: A cause of progressive focal encephalopathy. Dev. Med. Child. Neuro/. (SuppJ.)35: 135-137, 1975. 19. Sperber, E., and Vessal, K.: An acquired lateral v('nlriculM diverticulum appearing as a positive br..lin sC..ln. Br. /. Radiol. 47: 429-431, 1974. 20. jenkins, R.: f'Mavpntricular porencephalic diverticulum with /..Itenl hemiparesis as a complication of vpnlriculography. /. Neural. Neurosurg. Psychiatry 30: 2bl-263, /967. 2 I. Northfield, D. W.e., and Russell, D.S.: False diverticulum of a lateral ventricle causing hemiplegia in l-hronic interndl hydrocephalus. Brain 62: 311-320, 1939. Acknowledgments This work was supported in part by a grant from Research To f'rpvent Blindness, Inc., New York, New York (Departmpnt of Ophthalmology). Write for reprints to.' Ronald M. BurdI', M.D., Department of Ophthalmology-Box 8096, 660 South Euclid Avpnue, 51. Louis, Missouri b311O. Journal of Clinical Neuro-ophthalmology |
