Neuro-ophthalmological complications of enlargement ofthe ... · British JournalofOphthalmology, 1978, 62, 536-542 Neuro-ophthalmological complications of enlargement ofthe third

British Journal of Ophthalmology, 1978, 62, 536-542

Neuro-ophthalmological complications ofenlargement of the third ventricleROBERT H. OSHER, JAMES J. CORBETT, NORMAN J. SCHATZ,PETER J. SAVINO, AND LINDA S. ORRFrom the Neuro-ophthalmology Unit of the Wills Eye Hospital, and the Departments of Ophthalmologyand Neurology, Jefferson Medical College, Philadelphia

SUMMARY A wide variety of visual sensory and ocular motor problems may occur as a directresult of enlargement of the third ventricle. Four patients are described with optic nerve dysfunction,partial third nerve palsy, proptosis, and Sylvian aqueduct syndrome all resulting from an enlargedthird ventricle. The pathogenetic mechanisms are discussed.

The close relationship of the third ventricle to theoptic chiasma, optic nerves, and cavernous sinusprovides the anatomical substrate for a variety ofneuro-ophthalmological disorders that can resultwhen there is ventricular enlargement. Posteriorfossa lesions may produce a false localising chiasmalsyndrome with bitemporal hemianopia and bilateraloptic atrophy, secondary to an enlarged thirdventricle acting as a suprasellar mass (Weber, 1906;Oppenheim, 1911; Wilbrand and Saenger, 1913;Learmonth, 1931; Sinclair and Dott, 1931; Wohlwill,1931; Wagener and Cusick, 1937; Weinberger andWebster, 1941; Walsh and Hoyt, 1969). Unilateraloptic nerve dysfunction, infranuclear motilitydisturbances, unilateral proptosis, and the Sylvianaqueduct syndrome produced by internal hydro-cephalus are rare (Lassman et al., 1960; Lerner et al.,1969; Swash, 1974; Shapiro et al., 1976). Fourpatients are described who demonstrate theseunusual clinical neuro-ophthalmological problems ofdilatation of the third ventricle.

Case reports

CASE 1A 38-year-old woman complained of progressiveblurring of vision in her left eye for 3 months. Anormal eye examination had been recorded 8 monthsearlier. On neuro-ophthalmological examinationvisual acuity was 6/5 OD and 6/60 OS. Ocularmotility was normal. The pupils were equal, witha left afferent defect (Marcus Gunn pupil). Visual

Address for reprints: Dr Norman J. Schatz, Neuro-ophthalmology Unit, Wills Eye Hospital, 1601 SpringGarden Street, Philadelphia, Pa. 19130, USA

fields were full in the right eye, and the left visualfield was markedly constricted, with only a smallparacentral island of vision remaining (Fig. 1). Theright optic disc was normal but the left optic discwas pale. The neurological examination was other-wise normal.

Skull radiographs showed that the left optic canalwas 2 mm larger than the right. Computerised axialtomography showed dilatation of the third andlateral ventricles with no evidence of a mass lesion(Fig. 2). Pancerebral arteriography demonstrated asmall arteriovenous (A-V) malformation in the leftoccipital region supplied by a branch of the leftanterior cerebral artery, middle cerebral artery, andposterior cerebral artery. The fourth ventricle wasof normal size on pneumoencephalography, but airdid not pass beyond the rostral end of the Sylvianaqueduct. Ventriculography was not performed.A ventriculoperitoneal shunt was placed, and

over the next week visual acuity improved from 6/60to 6/15 OS, where it has remained to the present.

Comment. A 38-year-old woman with visualblurring in 1 eye was found to have monocularvisual loss, an afferent pupillary defect, a constrictedvisual field, and a pale disc. This was thought to besecondary to left optic nerve compression by anenlarged third ventricle resulting from aqueductalstenosis.

CASE 2A 9-year-old boy was found by a school nurse tohave poor vision in his right eye 2 years before ourinitial examination. Neuro-ophthalmological exam-ination revealed hand motion vision OD and 6/6 OS.There was left blepharoptosis, and the eye could notbe elevated in abduction. Pupils were equal, with a

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Neuro-ophthalmological complications of enlargement of the third ventricle

Fig. 1 Case 1. Severelyconstricted left visualfieldconsistent with an optic nervedefect. Visual field in the righteye is normal

LE_ 6/60 RE 6/5ACUITY 6/0ACUITY_____

right afferent defect. Only a temporal island ofvision remained in the right eye, and the blind spotin the left eye was enlarged (Fig. 3). Ophthalmoscopyrevealed a pale right disc. The left disc was elevatedand the margins were blurred. Neurological examin-ation was otherwise normal.A technetium pertechnetate brain scan was

normal. Skull x-rays demonstrated suture separation;however, the optic foramina were normal in size.Pancerebral arteriography showed extreme ventri-cular enlargement. Combined ventriculography andpneumoencephalography revealed substantial hydro-cephalus secondary to aqueductal stenosis, with anenlarged third ventricle extending anteriorly to theplanum spenoidale (Fig. 4). A ventriculospinal-subarachnoid (Torkildsen) shunt was inserted, andwithin 48 hours the patient's vision improved fromhand motion to counting of fingers, and the papill-oedema subsequently resolved. Visual function hasremained stable for the last 6 years, and levatorfunction completely recovered.Comment. A 9-year-old boy had monocular

reduction in visual acuity, an afferent pupillarydefect, an optic nerve visual field defect, and afundus picture characteristic of the Foster-Kennedysyndrome. These findings, in addition to a superiordivision third-nerve paresis, resulted from anenlarged third ventricle secondary to aqueductalstenosis.

CASE 3A 19-year-old man complained of pain in his righteye and intermittent double vision for 2 years. Atthat time the right conjunctiva was said to have beencongested, and there was 3 mm of right proptosis.The patient was referred for neuro-ophthalmologicalconsultation because of bilateral visual blurring andfrequent dull frontal headaches of 2 months'duration. The patient described difficulty with

concentration and memory, and had becomewithdrawn.Head circumference was 60 cm. Examination of

his mental status confirmed impaired memory, dis-

Fig. 2 Case 1. Computerised axial tomography scanshows dilated third (arrows) and lateral ventricles withdecreased density at the angles of the frontal hornswithin the cerebral white matter consistent with increasedperiventricular water

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Robert H. Osher, James J. Corbett, Norman J. Schatz, Peter J. Savino, and Linda S. Orr

Fig. 3 Case 2. Visual field inright eye shows a residualtemporal island of hand movementvision. The blind spot in the lefteye was enlarged

LE 6/6 RE, HM

Fig. 4 Case 2. Combinedpneumoencephalogram andventriculogram showing amarkedly dilated third ventricleextending to the planumsphenoidale. (Arrows define theanterior extent of the thirdventricle)

orientation to time and place, and poor abstractingand calculating abilities. There was 5 mm proptosisof the right eye, with conjunctival congestion. Visualacuity was 6/15 OD and 6/18 OS. Ocular motilitywas full. Both pupils reacted sluggishly without arelative afferent defect. Visual fields were unreliablebecause of the patient's dementia. There wasbilateral chronic papilloedema.

Skull x-rays and tomography of the parasellararea revealed separation of sutures, completedestruction of the sella turcica and upper third ofthe clivus, with obliteration of the sphenoid sinus(Fig. 5). The sphenoid wings and planum sphenoidalewere demineralised, a finding consistent with long-standing increased intracranial pressure. The superiororbital fissures were widened and the internalauditory canals were enlarged (Fig. 6). Ventriculo-

graphy showed dilatation of the lateral and thirdventricles. The remnants of the sella turcica wereoccupied by a massively dilated third ventricle.Review of skull x-rays taken at the time of a fall 7years earlier revealed an enlarged sella turcica andseparation of sutures which were unrecognised atthat time.

Ventriculoperitoneal shunting was performed andvisual acuity returned to 6/6 OD and 6/9 OS, withreduction of proptosis of the right eye. He has beenfollowed up for 3 years, with no further problems.

Comment. A 19-year-old man with deteriorationof mental function, personality change, visual loss,proptosis, and papilloedema was found to have adilated third ventricle secondary to aqueductalstenosis. The third ventricle extended into theparasellar area and it is postulated that proptosis

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Fig. 5 Case 3. Obliteration ofthe bony landmarks of the sellaturcica and upper third of theclivus due to third ventriclecompression. There is a markedthinning of the squamous portionsof the temporal and parietal bones

resulted from obstruction of venous return bycompression of the cavernous sinus.

CASE 4An 18-year-old girl presented with frontal headacheand transient episodes of visual blurring. Theheadaches were increased in severity with theValsalva manoeuvre. Three months before she wasadmitted to hospital a swishing sound developed inher head.

Visual acuity was 6/6 OU and bilateral papill-oedema was present. She had an incongruous leftinferior quandrantanopsia. Pupils were 4 mm ODand 5 mm OS; both pupils reacted sluggishly tolight but were briskly reactive to convergence.Ocular motility was normal. A bruit could be heardover the entire head. Pancerebral angiography,computerised axial tomography scan, and pneumo-encephalography were consistent with a diagnosis ofstenosis of the Sylvian aqueduct. One month afterplacement of a Torkildsen tube the patient re-appeared complaining of bifrontal headache,recurrence of the 'swishing' sound, and transientvisual obscurations. Initial examination revealedonly papilloedema and coarse upbeating nystagmusin the primary position. Subsequently over the nextweek she developed convergence-retraction nystag-mus on attempts at saccadic eye movement in alldirections, paralysis of upgaze with preserved doll'shead manoeuvre, and pathological lid retraction onattempts at up- or downgaze. Her eyes becameprogressively more convergent and tended to remainin tonic downgaze. The Torkildsen tube wasreplaced by a ventriculoperitoneal shunt, withprompt disappearance of all findings.

Comment. A young woman with aqueductalstenosis had a Torkildsen tube placed, and shuntdysfunction promptly produced a classic Sylvianaqueduct syndrome associated with massive ventri-

Fig. 6 Case 3. Marked demineralisation of the sphenoidbones and widening of the superior orbital fissures is seen.Enlargement of the internal auditory canals (arrows) isa sign of long-standing increased intracranial pressure.Prominent separation of sutures is unusual in a patientthis age (19 years old). The optic canals were not enlarged

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cular dilatation. Relief of pressure resulted inremission of signs and symptoms.

Discussion

The neuro-ophthalmological complications of anenlarged third ventricle result from the anatomicalproximity of this structure to the cavernous sinus,anterior visual pathways, pretectum, and the cranialnerves responsible for ocular motility. Compressionof these structures may produce proptosis, opticnerve dysfunction, ophthalmoplegia, or the Sylvianaqueduct syndrome (Table 1).

PROPTOSISProptosis, ordinarily a sign of orbital disease, hasbeen reported with hydrocephalus. Gardner (1948)reported a patient with unilateral exophthalmos anda large haemangiomatous cerebellar cyst. Anipsilateral post-traumatic orbital plate defect andencephalocele provided the orbital cause forproptosis related to her hydrocephalus. Green andOliver (1968) described bilateral exophthalmos in apatient with aqueductal stenosis and concomitantthyrotoxicosis. Remarkably, both the eye signs andabnormal thyroid function values remitted after aventricular-jugular shunt was performed. Shapiroand associates (1976) reported a 46-year-old womanwith a history of monocular, post-traumatic blind-ness and optic atrophy, who developed dementia,proptosis, conjunctival chemosis, and ophthalmo-plegia in the blind eye. In the contralateral eye visualacuity was 6/30, with a superior temporal quad-rantanopia. The fundus was reportedly normal.Combined pneumoencephalography and ventriculo-graphy showed aqueductal stenosis with a dilatedthird ventricle filling the sella turcica. After aventriculoperitoneal shunt the conjunctival chemosis,ophthalmoplegia, and field defect resolved. Shuntmalfunction resulted in return of the chemosis.

Raised cavernous sinus venous pressure canproduce proptosis. This has been produced experi-mentally (Brunton, 1949) and documented clinically

Table 1

A. Anterior visual pathway compression1. Optic nerve2. Chiasm3. Optic tract

B. Motility disturbance1. Supranuclear-Sylvian aqueduct syndrome2. Infranuclear-Cranial nerve palsy III, IV, VI

(complete and partial)

C. ProptosisCavernous sinus compressionErosion of sphenoid wings by enlarged temporal lobe

in patients with a carotid-cavernous fistulas, as wellas those with parasellar lesions such as pituitaryadenomas (Boschi and Campailla, 1932; Spaeth,1937; Weinberger et al., 1940; Dixon, 1941;Meadows, 1945; Walsh, 1947 and with aneurysms(Jefferson, 1937; Dixon, 1941). A dilated thirdventricle which fills the sella turcica can clearlyproduce cavernous sinus compression and result inproptosis, as in our third patient.

OPHTHALMOPLEGIAThe ocular motility patterns described in patientswith an enlarged third ventricle have been limited tovertical supranuclear gaze palsies with posteriorthird ventricular dilatation (see below) and non-specific abducens nerve palsies which reflect increasedintracranial pressure.The patient described by Shapiro et al. (1976) had

marked unilateral limitation of eye movement,which resolved when a shunt was placed. Oursecond patient had an enlarged third ventricle whichproduced superior division third nerve paresis,presumably related to cavernous sinus compressionas well as direct unilateral optic nerve compression.

SYLVIAN AQUEDUCT SYNDROMEDownward deviation of the eyes in children withaqueductal stenosis was ascribed by West (1848) todepression of the orbital roof. Later reports suggestthat this symptom is actually due to the Sylvianaqueduct syndrome. This clinical constellation ofconvergence-retraction nystagmus, light-near dis-sociation of the pupils, pathological lid retraction,and inability to perform saccadic or pursuit upwardmovements, secondary to an enlarged posterior thirdventricle and dilatation of the anterior Sylvianaqueduct, has been described in a number ofpublications since Pennybacker's observations on 5persons with aqueductal stenosis (Pennybacker,1940; Lerner, 1969; Swash, 1974; Chattha andDeLong, 1975). This syndrome is characteristic inpatients with aqueductal stenosis who have becomeshunt-dependent or in whom aqueductal stenosis isrelatively acute in onset (Chattha and DeLong,1975). Shunt failure promptly results in re-appearance of the symptoms and signs, which remitwhen the shunt is again functional (Swash, 1974).

VISUAL PATHWAY INVOLVEMENTAs early as 1713 Cheselden described an optic nervewhich was 'crushed flat' in a patient with hydro-cephalus. Bitemporal hemianopia resulting frombulging of the floor of the third ventricle in apatient with hydrocephalus was first reported byWeber (1906), and individual patients have sub-sequently been reported by Oppenheim (1911),

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Wilbrand and Saenger (1913), Learmonth et al.(1931), Sinclair and Dott (1931), Wohlwill (1931),Wagener and Cusick (1937), Weinberger and Webster(1941), and Walsh and Hoyt (1969). A neoplasm inthe posterior fossa (usually cerebellar) was theunderlying lesion in most of these patients. Aque-ductal stenosis and postmeningitic adhesions wereless common causes, Bitemporal hemianopias inpatients with internal hydrocephalus are probablyproduced by downward displacement of a distendedthird ventricle and compression of the optic chiasma.Ventriculostomy resulting in postoperative visualfield improvement has provided additional evidenceof the mass effect of the third ventricle (Sinclair andDott, 1931).Homonymous hemianopia has also been reported

as a result of a dilated third ventricle. Wagener andCusick (1937) described 1 patient with an incongru-ous homonymous hemianopia due to a posteriorfossa lesion. Four patients each with homonymoushemianopia and a cerebellar tumour were reportedby Weinberger and Webster (1941). These visualfield defects may be accounted for by either of 2mechanisms. The first suggests that downwarddisplacement of a distended third ventricle againsta prefixed chiasma may result in optic tract compres-sion. An alternative explanation proposes that thedistended third ventricle ventrally displaces the opticchiasma and stretches the optic tract over theposterior communicating artery (Fay and Grant,1923; Weinberger and Webster, 1941; Hughes,1946).

Binasal hemianopia has been described as afeature of third ventricular distension (Cushing andWalker, 1912). They postulated that the dilatedthird ventricle ventrally displaced the optic nerveand adjoining chiasma against the resistant, pulsatilecarotid arteries, thus producing the binasal fielddefect. The illnesses reported by Walker andCushing were heterogeneous, and the only commonfeatures were chronic papilloedema and secondaryoptic atrophy. It is our opinion that binasal defectsdescribed by Cushing and Walker were more likelyto have been caused by the long-standing papill-oedema than by chiasmal compression by adilatated third ventricle (Dersh and Schlezinger,1959).A wide variety of other visual field defects have

been reported in association with ventriculardilatation, including bilateral central scotomas(Wagener and Cusick, 1937). Unilateral optic nervedysfunction was described in 1 patient (Lassmannet al., 1960). A 10-year-old boy with normal visualacuity developed temporal disc pallor in the righteye followed by superior temporal sector visual fielddefect in the left eye associated with bilateral

papilloedema over a 9-month period. Cerebrospinalfluid pressure was raised, and the diagnosis ofaqueductal stenosis was made. After a ventricularshunt operation his papilloedema disappeared andthe visual field defect partially regressed. Theauthors concluded that the dilated third ventriclehad pushed the chiasma forward and downward,causing 1 optic nerve to impinge against the lateralaspect of the tuberculum sellae.

Patients 1 and 2 had purely unilateral optic nervedysfunction, shown by a monocular reduction invisual acuity, afferent pupillary defect, visual fielddeficit, and an atrophic disc. Asymmetrical expansionof an enlarged third ventricle resulting in opticnerve compression is an attractive explanation of thisunilateral optic nerve syndrome.

Dilatation of the third ventricle can produce a widevariety of visual symptoms. Compression of opticnerve, chiasma, or optic tract may produce mono-cular or binocular visual field defects. Disturbancesof ocular motility may result either from directcompression of the cavernous sinus and the motornerves or from distension of the pretectal andperiaqueductal regions. More remote effects withmonocular proptosis can also be seen as the result ofcavernous sinus compression and venous distension.

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Cheselden, W. (1713). Some anatomical observations.Philadelphia Transactions, 28, 281.

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Neuro-ophthalmological complications of enlargement ofthe ... · British JournalofOphthalmology, 1978, 62, 536-542 Neuro-ophthalmological complications of enlargement ofthe third