82020ournal ofNeurology, Neurosurgery, and Psychiatry 1993;56:820-822
SHORT REPORT J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.7.820 on 1 July 1993. Downloaded from
Cavum septum pellucidum and obstructive hydrocephalus
P L Silbert, S S Gubbay, R J Vaughan
Abstract pellucidum. Five of these were boxers, who Five patients presented with symptoms have a recognised increased incidence of related directly to pressure effects from cavum septum pellucidum.' their cavum septum pellucidum with The septum pellucidum extends between persistent or intermittent obstructive the anterior portion of the corpus callosum hydrocephalus. The most characteristic and the body of the fornix. The cavum sep- presenting symptoms were intermittent tum pellucidum and the more posterior postural headache and postural loss of cavum vergae are potential cavities that lie consciousness. If cysts of the cavum sep- between the leaflets of the septum pellu- tum pellucidum are symptomatic and cidum. As a potential cavity, they are a nor- stereotactic cyst puncture or fenestration mal finding. Controversy has existed for many are ineffective, ventriculoperitoneal years about whether enlargement of these cav- shunting should be carried out before ities is responsible for any disease state. Many resorting to more radical excision of the symptoms and diseases have been attributed cyst. to cysts of the septum pellucidum, but a cavum septum pellucidum is such a common (7 Neurol Neurosurg Psychiatry 1993;56:820-822) finding that its previous associations are being questioned. Earlier series were biased because The presence of a cavum septum pellucidum pneumoencephalography and necropsy repre- represents a normal anatomical variant which sented the only available diagnosed modali- is usually asymptomatic. Its prevalence in ties,3 and descriptions of the septum childhood declines with age, being present at pellucidum syndrome, characterised by men- necropsy in all premature infants, 85% of one tal disturbances, ataxia, disordered speech, month old infants, and 12-15% of six month epilepsy, and bilateral pyramidal signs, are old infants. In adults the prevalence varies now being questioned.4
significantly depending on the criteria used to We present a series of five patients (three of http://jnnp.bmj.com/ diagnose a cavum septum pellucidum as some whom have been previously reported on5) remnant should be present in all patients.' In who presented because of symptoms related a recent series 14 out of 1914 adults (0-73%) directly to pressure effects from their cavum showed CT evidence of a cavum septum septum pellucidum.
in cases cavum Table Summary ofclinical details and results of treatment andfollow up five of septum pellucidum on September 25, 2021 by guest. Protected copyright. Case No 1 2 3 4 5 Clinical details Presenting age 42 Years 61 Years 46 Years 60 Years 6 Months Sex M M M F M Duration of symptoms 3 Years 3 Months 4 Years 23 Years 4 Months* Royal Perth Hospital, Previous head injury - + + - Box X2213, GPO, Non-postural headache - + + - Unknown Postural headache + + + + Unknown Perth, Western Postural unconsciousness + + + Australia, 6001, Postural dizziness - + - - Unknown Australia Ataxia - + Department of Seizures - - - - Postoperatively Neurology P L Silbert Surgical treatment and resultstf SS Fenestration Unsuccessful Unsuccessful Successful Gubbay stereotactic Department of Excision Successful Neurosurgery Shunting Successful Unsuccessful R J Vaughan (ventriculoatrial (ventriculoperitoneal (and cyst) shunt) shunt) Correspondence to: Professor Gubbay Follow up Received 11 March 1992 Duration 22 Years 15 Years 36 Months 3 Years (died) and in revised form 27 August 1992. *Presented with delayed milestones at 6 months of age. Accepted tCase 3 was lost to follow up. 11 September 1992 INot all procedures were performed on all patients. Cavum septum pellucidum and obstructive hydrocephalus 821
Case reports of the cavum septum pellucidum. It is an
The case histories are summarised in the important part of the limbic system, with J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.7.820 on 1 July 1993. Downloaded from table. Cases 1 to 4 were of four adult patients prominent connections in both the medial (or presenting with the common symptom of Papez) limbic circuit and the basolateral lim- headache between the ages of 42 and 61 bic circuit. This provides direct and indirect years. In two instances there was a history of afferent and efferent connections to the hip- closed head injury with total recovery. The pocampus, cingulate gyrus, medial frontal patients in cases 1 and 3 had experienced loss cortex, anterior nucleus of the thalamus, of consciousness when lying supine. The mamillary bodies, hypothalamus, and amyg- patient in case 2 became dizzy on lying daloid nuclei.' Disease of the cavum septum supine and had occasionally lost conscious- pellucidum could therefore be expected to ness when bending forward. cause symptoms either by mass effect or by The patient in case 5 presented in infancy disturbance of the emotional and behavioural with delayed milestones of development and functions of the limbic system. hydrocephalus which failed to respond to Cysts of the cavum septum pellucidum ventriculoperitoneal shunting because of con- have been classified into two major groups.' tinued distension of the cyst and possible Incidental (asymptomatic) cava are seen in shunt malfunction. No further motor or almost 1% of cranial CTs. Pathological social development occurred and he died at 3 (symptomatic) cava are comparatively rare, years of age of bronchopneumonia. The true are always non-communicating, and may be cause of his condition only became evident at associated with other structural or inflamma- necropsy (figure: left). tory brain disease. Stereotactic puncture of the cyst was Since the first cases reported by Dandy of successful treatment in case 4. Case 1 was an adult and a child with hydrocephalus due initially treated by fenestration of the cyst but to a cyst of the cavum septum pellucidum symptoms recurred and at reoperation the (and vergae)6 further cases in adults, children, fenestrated walls of the cyst were found to be and infants have been reported occasionally, covered by a fine arachnoid membrane. The usually in single case reports or reviews.' 7-11 cyst walls were then excised with no recur- We have described five patients with symp- rence of symptoms. In case 2 wide fenestra- toms of persistent or intermittent obstructive tion of the walls of the cyst resulted in only a hydrocephalus associated with an enlarged temporary amelioration of symptoms, but cavum septum pellucidum. The most com- ventriculoatrial shunting (with passage of the mon presenting symptoms were intermittent drainage catheter through the cavum septum headache (all four adult cases) and loss of pellucidum) was effective. consciousness (three of the four adult cases), presumably due to sudden transient obstruc- tion of the foramina of Monro. In the more Discussion recent patients who underwent magnetic res- The cavum septum pellucidum and the more onance imaging and pathological examination posterior (often interconnecting) cavum ver- respectively (cases 4 and 5; fig) the foramina gae are often incorrectly called the fifth and of Monro were patent. Amin has proposed a sixth ventricles. They are not, however, part mechanism whereby intermittent obstruction of the ventricular systems as they have a dif- of the foramina of Monro may occur." After http://jnnp.bmj.com/ ferent embryological origin and are not lined foraminal obstruction the ventricles expand, by ependymal or choroid plexus cells.' The causing alteration in the shape of the cyst and cavum is a potential cavity and therefore release of the obstruction. All four of the significant enlargement is best termed a cyst adult cases had a distinct postural component on September 25, 2021 by guest. Protected copyright. Figure Left: Inferior surface ofbrain showing cyst ofseptum pellucidum, with compression of the dorsal aspect of the brainstem; the lateral ventricles do not communicate with the cyst (case 5). Right: Coronal Ti weighted scan showing F: large cavum septum pellucidum (case 4).
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i:_ : i _i,,,.* to their headache and in three of these gressive hydrocephalus. Cavum septum pellu- patients a colloid cyst of the third ventricle cidum has been previously described as a J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.7.820 on 1 July 1993. Downloaded from was therefore the provisional diagnosis on ini- cause of shunt malfunction.'5 tial clinical assessment. Whereas the patients In conclusion, symptomatic enlargement of in cases 1 and 2 were investigated before CT the cavum septum pellucidum is rare and became available the response to surgery most commonly causes intermittent obstruc- strongly supports the causative relation tive hydrocephalus with headache and loss of between the cyst of the cavum septum pellu- consciousness; it is amenable to surgical cidum and the patient's symptoms. In case 4 treatrment such as cyst puncture or shunting, the history was suggestive and neuroimaging ventriculoperitoneal shunting, or radical that clearly showed a large cavum septum excision. pellucidum without ventricular dilatation (fig- We thank Drs J B Stokes, D A Prentice, T Welborn, and ure: right). Stereotactic puncture of the cyst M Adonis for referring the patients. The surgery in cases 1 of the cavum septum pellucidum produced a and 2 were performed by the late Mr J S Lekias, and in cases 4 and 5 by Mr M Lee. Postmortem examination and photo- sustained remission from further headaches. graphy in case 5 was performed at the Department of The patient in case 5 had severe hydro- Pathology, Princess Margaret Hospital. Other illustrations were provided by the Department of Medical Illustrations, cephalus due to a combination of obstruction Royal Perth Hospital, and Department of Medical of the lateral ventricles and mass effect of the Illustrations, Sir Charles Gairdner Hospital. Magnetic reso- nance imaging was performed by Dr Mark Khangure, Sir cyst of the cavum septum pellucidum (figure: Charles Gairdner Hospital. We are grateful to the editor and left). The final recognition of the contribution publishers of Clinical and Experimental Neurology for permis- of the cyst of the cavum septum pellucidum sion to include cases 1-3.5 to his hydrocephalus was unfortunately only made at necropsy. With more advanced 1 Shaw CM, Alvord EC. Cava septi pellucidi et vergae: current neuroimaging techniques earlier diag- their normal and pathological states. Brain 1969;92: nosis and appropriate therapy should now be 213-24. 2 Bogdanoff B, Natter HM. Incidence of cavum septum possible. pellucidum in adults: a sign of boxer's encephalopathy. The possible contribution of enlargement Neurology 1989;39:991-2. 3 Bruyn GW. Agenesis septi pellucidi, cavum septi pelluci- of the cavum to either psychiatric distur- di, cavum vergae, and cavum veli interposti. In: Vinken bances or epilepsy is controversial. None of PJ, Bruyn GW, eds. Handbook of clinical neurology, Vol 30. Amsterdam: North Holland Publishing our adult patients showed psychiatric abnor- Company, 1977:299-337. malities, behavioural changes, or epilepsy 4 Wilder J. Hydrops des Cavum septi pellucidi. Klin Wochenschr 1938;17:414. preoperatively, although postoperatively one 5 Gubbay SS, Vaughan R, Lekias JS. Intermittent hydro- patient (case 1) was confused and depressed, cephalus due to cysts of the septum pellucidum: a study of three cases. Clinical and Experimental Neurology 1978; and another (case 2) confused with a tran- 14:93-9. sient left hemiparesis post-operatively. Earlier 6 Dandy WE. Congenital cerebral cysts of the cavum septi pellucidi (fifth ventricle) and cavum vergae (sixth ventri- series evaluating the psychiatric aspects of the cle). Archives of Neurology and Psychiatry (Chicago) cavum septum pellucidum were subject to 1931;25:44-66. 7 Dooling EC, Barlow JF, Murphy JV, Richardson EP. Cyst appreciable referral bias,3 but several recent of the cavum septi pellucidi. Arch Neurol 1972;27: reports describe possible associations with 79-84. 8 Heiskanen 0. Cyst of the septum pellucidum causing psychosis'2 13 and a reversible Korsakoff's psy- increased intracranial pressure and hydrocephalus. chosis was found after a cyst of the septum J Neurosurg 1973;38:771-3. 9 Cowley AR, Moody DM, Alexander E, Ball MR, Laster pellucidum was excised.'4 DW. Distinctive CT appearance of cyst of the cavum Craniotomy with fenestration (and biopsy) septi pellucidi. AJR 1979;133:548-50. http://jnnp.bmj.com/ 10 Garza-Mercado R. Giant cyst of the septum pellucidum. may not be as effective as shunting proce- J Neurosurg 1981-55:646-50. dures in treating symptomatic cavum septum 11 Amin BH. Symptomatic cyst of the septum pellucidum. Child's Nerv Syst 1986;2:320-2. pellucidum. Stereotactic puncture is less 12 Lewis SW, Mezey GC. Clinical correlates of septum pel- invasive but would be liable to the same lucidum cavities: an unusual association with psychosis. Psychol Med 1985;15:43-54. mechanism of potential failure as open fenes- 13 Mizukami K, Ofuiku K, Shiraishi H, Koizumi J. Neuro- tration. The child who died of progressive psychiatric studies of the "pellucidum syndrom". Jpn J did not to ventricular Psychiatry Neurol 1989;43:67-75. hydrocephalus respond 14 Gil Neciga E, Gil Peralta A, Polaina M, Sureda B, on September 25, 2021 by guest. Protected copyright. shunting, but the shunt was probably only Bautista J. Cyst of the septum pellucidum and Korsakoff's psychosis. Eur Neurol 1989;29:99-101. partially draining the cyst and ventricular 15 Mapstone TB, White RJ. Cavum septi pellucidi as a cause drainage was not effective, which led to pro- of shunt dysfunction. Surg Neurol 1981;16:96-8.