57
Image-directed stereotactic drainage ofthe symptomatic cavum septi pellucidi et vergae J K. Krauss', M. Mohadjer', E. Milios', R. S cheremet', F. Mundinqer' 2
Department of'Neur osurgery Department ol Stereotaxy and Neuronuclear Medicine. Neurosu rgtcal Clinie. Albert- Ludwigs-Universlt ät. Freiburg
3
St. Josefs-Krankenha us. Freiburg
I
Int roducti o n
Two rare cases of symptomatic non-communicating cava se pti pellucidi et Vergae (CSPV) with successful treatme nt via image-directed stereotactic internal drainage are reported. Because tbe management of these ben ign space-occupying structures by the transcallosal a pp roach bears certa in risks , sa fer meth ods should be a pplied. Th e c1inical significan ce, results, and adva ntages of the CT-stereotactic procedure are rep resent ed . If pati ents with symptomatic CSPVa re scheduled for an ope ration, stereotactic interven tion is proposed as the ther apy of choice . Bildges te ue r te ste r eotaktische Drainage des pathologisch veränd erten cavum septi pellucidi et vergae Über zwei seltene Fälle mit sym ptomatischem großem , nicht-kommuni zierend em Cavum sep ti pellucid i et Verga e (CSPY) wird beri chtet. Bei beid en Patienten wurd e ein von bildgeben den Ve rfah ren ges teuerter stereota ktischer Eingriff - eine innere Drainage des Cavums - erfolgreich du rchgeführt. Weil die operative Verso rgung dieser gutartig en raumfo rd ern den Stru kturen über den bislang angewandten Zugang über den Balken gewiss e Risiken beinh altet . sollten sicherere Methoden zur Anwendung kommen . Die klinische Bedeut ung. die Ergebnisse und die Vorteile des CTster eotaktischen Verfahrens werde n da rgest ellt. Wenn bei Pati ent en mit sympt omatische m CSPVein operativer EingrilTgeplant ist. wird das ste reo taktische Verfah ren als Therapie der Wahl vorgeschlagen. Key-Words Cavum se pti pellucidi - Cavum Verga e Neuro-imaging - Stereotaetie surgery
Neurochirurgia 34 ( 99 1) 57- 61 © Georg Thieme Verlag Stuttgart · New York
The cavum septi pellucidi (CS P) a nd the cavum Vergae (CY) a re often refer red to as cere bral midline malformations (I . 8. 26. 32). It is more ap pro priate. however, to eall them persisting primitive structures, as they are perfectJy norm al during the fetal Iife and in pr eterm infan ts (5.6, 10, 30). CSP was already known by a natomists to the seventeent h cent ury (9). When the Italia n anatomist Verga reported his obs ervation s of a cystic forma tion dorsal of the septum pellucidum, the question arose about the clinieal implications ofth ese structures (34). Wheth er or not persisting cava of the midline may have cliniea l signifieance in certain case s is still a cont rovers ial issue (I . 2. 4. 6,11 -13. 15. 30. 31). While there is general agreeme nt ab out their normal developm ental appearanee in fetu ses and in infants in the first month s of life, ther e a re conflicting data, especially conce rn ing CV, a bou t eventua l path ological signifieanee , prevalenee and neurologie or psychiatrie sym ptoms possibly associated (l , 2.4. 6. 7. 11-13 ,1 5. 18.22.30.31). Usually. they should be conside red as coincidenta l variants . There are seve ral cases reported in the liter atu re, howeve r, in which the rela tionship between c1inical sym ptoms and persisting cava could be convincingly dernonstrated (6- 8. 14. 17. 19. 21. 29. 30. 33). We report on two patients who had c1inieal manifestations of a persis ting non -commu nieating cavum septi pellucidi et Ve rga e (CSPY). By CT-base d stereotactie techniques a catheter was introduced in the cavum to establish an internal drainage with the ventricular system. Case rep ort s
Case I This 29-y ear-old man ,..'as adm itted with an 18month history of headach es. accentuat ed after psychic stres s , the n acco mpanied by nausea and sporad le vertig o. For a yea r he had been sufTering increasingly from Iatlgue. difflculty in sleepi ng and wa king ea rly in the morning. He co mplained of exhaustion, irritability and nervousness. and s uITered from a lack of initiative and sponta neity. He had increas ing difficulty coping at work and in stress situat ions showed a nervous tic-like twitehing of the eyelids. One episode of sudden vcmiting was reported . Some lime s he experienced unpleasent tingling painful se nsations in his left arm. Electromyographic and neurographi c examinations produced 0 0 pathological data . Eleetroencephalography revealed no foeus. There wa s no history of head traum a, intraeranial or other Infeclion.
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Sum mary
Neurochi rurgia 34 (1991)
The neurological examina tion showe d diserete optokineüc disturbanees and tendon re flexes whieh were more pronouneed on the right side than on the left. Computerized tomography revealed a CSPV with CSF-isodense eontents . The later al ventricles wer e slightly asymmetrieal and the re were no slgns of hydrocephalus . MRI sea ns disclosed no ad ditional pat hological cha nges (Fig. 1). The cav um had a maximum exte nsio n of 57 x 29 x 23 mm . The CT-stereotactie intervention was done aeeor ding to th e stand ar ds published elsewhere (3, 23, 24, 27). Aeeording to CT seans a fter fixat ion of the stereotaetic ring , a ta rget point was ealculated in the anter ior pa rt of the cavum . After a bur rhole had been plaeed 1 em behind the right coronal sutu re, parasagittally, the dura was opened an d th e ean ula for cystography was introduced in th e cavum. After a spontaneous outflow of clear liquid (3/3 cells, total protein 29.6mg%), contrast medium was introduced. The cavity did not communicate with the ven tricula r system. Then a 8.5cm silicone cathete r with a Riekham reservoir was plaeed to est ablish a n intern al dra inage with th e right later al ventricle and more liquid was aspirated. The operation was performed und er loeal anaesthesia. The postoperative course was uneve ntful and th e symptoms resolved completely after some weeks , There has been no reeurrenee in 2 yea rs. Follow-up CT sea ns revealed the cavum continuously decreasing in stze. the last CT sca n showed only a small rest (Fig. 2). The lateral ventricles remain ed asym metr ical and showed no cha nge in width. The patie nt returned to wor k free of symptoms.
Case 2 This 2 11/12-year-old girl was presen ted with a histcry of psychomotor retardaüon and delayed sta tomotor develcpment. She had been born prematurely in the 35 lh week of gestatio n. At the age of eight wee ks she was brought to the hospital with shock-synd rome. She showe d rotatin g movement of the eyes. museular hypotonia with augmenta tion of muscle tonu s after touching the skin, and intermitte nt shrill serea ming, while being otherwise apathetic. Concomitta nt sympto ms were a miliary exa nthe ma ofthe trunk and th e arms, pne umonia with ate leetas es , hepati tis a nd necrotizing enterocolitis with ileus. The ap athetic state became worse . grip reflexes were missing. Encepha litis was assum ed . Histological exa mination of resected gut revealed necrotizing angiitis. The etiologyof'the aeute disease remain ed unclear . th e imm unological examination detected only nonspeciflc cha nges. a generalized viral infection or a baeterial or funga l sepsis eould not he esta blished. She gra dually reeover ed , yet her further development was delayed and she lea rned to speak only a few words. On admiss ion th e child was alert , but esta blishing contaet with her was difflcult. She was ment ally retard ed . had a vocabula ry of abo ut forty words. her speec h was slurred . and she was highly distr a ctabl e. The neurologlcal exa mina tion disclosed strabis mu s divergens alt ern ans. a lack of visuomotor eoord ination with "optic atax ia" a nd slight intention tre mor . Walking was dlffieult due to gait ataxia and discrete par aparesis. Electroenceph alography showe d a slight genera lized dysr hythmia withou t foeal cha nges. CT scans reveal ed a large CSPV with CSF-isode nse contents with a maximum exte nsio n of6 8 x 31 x 25m m. both later al vent ricles we re enlarged (Fig. 3). The cere bellum was slightly at ro phie. Because of the patien t's age the ste reotactie intervention had to be done with intub ation a nest hesia . To establish a n internal dr a inage with th e ventr icular system a 5.7 cm silicone catheter was placed as desc ribed in case 1. Again, the cavu m did not commu nicate with th e ventricular system. The postoper ative course was unevent ful. Followup CT sea ns showe d that the cavum had diminished in size. the lateral ventricles were slightly smaller th an before, th e cortieal sulci
J. K. Krauss. M. Mohadjer. E. Milios. R. Scheremet. F. MUlldillger
were enla rged (Fig. 4). The remaining ventric ulomega ly was due to inte rnal brain atrop hy. In th e month s following operat ion symptoms improved an d afte r one year she has mad e further progress in he r motor an d in her ment al development.
Discussion The nom enclature with respect to th e anato mical hord ers of the cava used in the liter ature is not always consistent. According to anatomical studies (6) the bord ers of es p are anteriorly the genu of th e corpus callosum , superiorly the body of the corpus callosum, posteriorly the columnae forni cis, inferio rly the ros trum of the corpus callosum and the anterior commissure and laterally the septal lamella e. The bord ers of ev are ant eriorly the columnae forni cis, sup eriorl y the body of the corpus callosurn. posteriorly the splenium of the corpus callosum, inferi orly the psalterium, and later ally the forni ces and the latera l ventr icles. lsolated ev are rare (8, 6, 16). They are usually accompanied by a e s p (28). The cava are normally present during fetal developm ent (6, 30). They develop in the fourth month ofpregnancy and start regression hetween the 30 th and 40 th week of gestation - first the post erior portion (Cv) , then the anterior portion (eSP) (6). They are physiological cavities in pr em atures and infants in the first montbs oflife and can be detected by ultrasound and Cf (5, 10,2 5). A higher pre valenc e in children with Down's syndrome was found ( 8). The prevalence in adults is estabIished mainly on post mort em and pneumoencephalograp hic studies (pEG) and th e frequ ency varies , due in part to methodolo gical differ ences (4, 12 , 17, 28, 31). In a recentl y published study the relative fre quency of es p in adults was 0.73 % in CF, no ev was describ ed (4). There are diver gen t opinions ab out the ra tio of communicating and non-commun icating forms, the term cyst is used in different ways. Whether or not a non-communicating cavum septi pellucidi or cavum Vergae should be called "cyst" seems to be chiefly a sema ntic problem. We pr efer the term "non-cornmunicating cavum", It is ass umed that the communicating forms are asymptoma tic (30). Unfort una tely in th e literature "cavum septi pellucidi" often is used to denominate CSP, ev or e spv. Thus a compa rison of the cas es reported to date is Iimited . The clinical significa nce of the persisting cava demonstrated with PEG has been a subj ect of controversial discussion 0 , 2, 11, 13, 15). The question is still unclarified , as to whethe r in certa in cases there may be an association with symptoms or othe r disorders, ma y it be cas ual or causa !. We consider it to be unlikely that es p with a tr an sver se diam eter of a few millimeters as see n usu ally may produce any clinical symptorns, hut difficulties ari se concern ing ev and space -occupying es pv. In their C'l'studies Nokano et al. could not recognize statistically valid relationships between neurol ogical symptoms and es p, ev and es pv presence in children (25). Yet Mill er et al, did not find ev or es pv in CT sca ns of children witho ut apparent neurologie abnormalities, but in five children ev or es pv was asso ciate d with delayed developme nt (22). A bab y with ev noted at two weeks of age by C'I', which disapp eared on rep eat ed scan, developed norm ally (22). Berg/e iter pr oposed that both the persisting cava as weil as the "ne uro tic" beh avior and the epileptic seizures in his patient s should be interpreted as a result of a common consti tutiona l factor , a
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58
Flg I
Preoperat ive MRI v iew of patient 1 in the axial plane
lVeurochirurgia 34 (199 1)
Fig. 2 Cl scans of patient 1ontoüow-cp centrotwithdiminishedszeofthe cavum
Fig. 3 PreooeranveaxialCl scans of patient 2 demonstratingthe cavumand the enlarged lateral ventncles
Fig.4 Postoperative Cl scans of patient 2ontcllow-upcontrol showing the introducedsi licone catheter inthe cavum, marked lyreduced in sze
"neuropathische Anlage " ofthe brain (2). Retro spe ctively. it cannot be excluded that at least some ofhis patients, as weil as pati ents described by oth er authors , sutTered from clinical manifestations due to the cava. The relationship betw een dini cal symptoms and CSPV or CV is plaus ible in cases in wh ich operation produced good results (7. 8. 14. 17. 19.21 .29. 33) . Several reports of cava with clinica l symptoms were reviewed by Shaw and A lvord (30). Th ey ditTerentiated between simple and complicated forms of cava, linking com plicated forms with such sequelae as hydrocepha lus. They pro posed th at the clinical symptoms, in conjunction with evidence of considerable pressur e on the su rrounding structures we re due to compression of the septal nuclei and the forn ices. which wou ld result in dysfunction of
the limbic system. Yet the size ofthe cavum does not necessarilycorrelate with c1inical symptoms, as smaller cava may also lead to hydrocephalus (6. 8). According to Bruyn (6). wh o reviewed most of the published reports on series and individual cases , the ass ociated symptoms found in patients with persisting midline cava are non-sp ecific including epileptic seizures, mental. mood or behavioral changes, attacks of headache, increased intracranial pressu re and hemiparesis .
Dandy was the first to establish an operative treatment for symptomatic CSPV or CV. He reported on two pati ents whom 'he ope ra ted on in 1931 by a tra nsca llosa l approach, fenes trating the cava for communication with the
59
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lmage-directed stereotactic drainage
Neu roehirurgia 34 (1991)
ventricular system (7). In subsequent years other re ports were published describing slightly modified techniques (14, 17,19,2 1,29,33). Over the past decade inform ation about treatm ent ofsymptomatic persist ing midline cava has been sparse. Four patients with comparatively small isolated CV causing hydro cephalus were treated by stereotactically placed internal drainage with good results (8). The risk of complications assoeiated with this form of ster eota ctic intervention is low, as could be also demonstrated in 12 patients with colloid cysts (23), To our knowledge, this method has not been applied in large symptomatic non-communicating CSPV previously. The patient described in case 1 sulTered from headaches and psychological disturbanees, and probably focal seizures. Since all these symptoms showed complete regression after stereota ctic implantation ofthe catheter for inter nal drainage of the cavum with the ventricular system and subs equent cavum shrinkage, we consider the clinical signs to be manifestat ions oft he cavum. \Ve propose that these non-specific symptoms wer e due to compression of the adja cent structures, wher eas occasional episodes of interm itten t hydrocephalus cannot be excluded. In case 2 the CT-stereotactic intervention with subsequent cavum shrinkage additionally aimed to deblock the foramina of Monr o, th us avoiding vent riculoatr ial or ventriculoperitonea l shunt procedures and eventual complications in these cases (20, 30). As not all symptoms were attributable to the cavum the thera peutic benefits may be limited and the procedure cannot be cura tive as in case 1. It is possible that the ass umed encephalitic process is involved with the per sistence of the non-commun icating midline structure. No patient with a CV or CSPV should be opera ted on.just because iti s ther e. Usually these persisting pr imitive stru ctures a re asymptomatic . Irin rar e cases ther e are symptoms that ar e attributable to a non-commun icating CSPV (compression on adjacent struct ures or hydrocephalus) an operative approach may be indicated . As there is a relative small risk, compared to the former applied transcallosal operations, we then would propose Imagedirected (CT or NMR) stereotactic intervention as the therapy of choice.
1. K. Krau se. M. M ohadjer. E. Milios. R. Schere m e t. t:Mundlnqer .. Bogdan ojJ. B.. H. M. Natter: Incidence ofcavum septu m pellucidum in ad ults: a sign of boxer 's encephalopat hy. Neurology 29 (1989) 99 1-992 5 Bohlager. R.. H. M. Straßöurq , M. Sau er: Das Cavum septi pellucidi und Cavum Vergae beim Säugling - Eine Untersuchung mit der zweidimensionale n Sektor- Echo- Enzephalographi e. KUn. Pädiat. 19511983192- 96 I> Brugn. G. W : Agenesis septi pellucldl. cavum pellucidi, ca vum ver gae a nd cavum ..'eli interp ositi.ln: Vinken, P. J.• G. W. Bruyn Ieds.). Hand book of Clinical Neurology. Vol. 30 . Amste rda m. Elsevier. 1977. pp 299-336 7 Dandg. W E.: Congenital cereb ral cysts ofthe cavu m septi pellucidi mnh ventriclel and cavum Vergae Islxth ventrtclel, Arch. Neuro!' Psychia t., Chicago 25 (1931144-66 8 Doneuer. E.. J. R. Moringlane. C. B. Ost ertaq : Cavum Vergae cys t as a cause of hydr ocepbalus. Malmost Iorgotten ?". Acta Neurochi r., Wien 83 (1986) 12-19 9 Duncan. D.: Explication nouvelle et mecanlque des actions ani males. Paris 1678 10 Farruggia . S .. D. S. Babcock: The cavum se pti pellucidi: its a ppea rance and incidence with cran ial ultrasonogra phy in infa ncy. Radiolo~' 13 9 ( 1 98 1 ) t 4 7 - 1 5 0
Feld. M. M.: Sur un cas de kyste du sep tum lucidum revele par des crlses d'epilepsie gene ral isee. Rev. neurol. 92 11955165-68 12 Pink e, J.. G. Koch: Das Cavum septi pellucidi: Vorkommen und Aussagewert. Dlsch. Z. Nerven heilk. 193 11 9681154- 157 13 Göilnitz . G.: Über das klinische Bild im erwe iterte n Cavum septl pellucidi. Drsch. Z. Nerven hellk. 163 (1949) t - 11 H Gubbay. S. 5.. R. Vaughan . J. S. Lekias: lntermlu ent bydro cephalus due to cysts of the septu m pellucidum : a study of three cases. Proc. Aust. Assoc. Neu rol. 14 ( 977) 93- 99 15 Hackst ein. F. G.: Über die Klinik des luftgefüllten. weiten Cav um sep ti pellucidi. Dtsch . Nervenheilk. 177 (1958) 348-369 16 Hea /g. 1. E . G. I. w ickbom. E. Jonon: Computed tomographie illustra tlon of cavum Vergae . J . Comput. Tomogr. 5 (1981) 336-339 17 lIughes. R. A.. J. W. Kernehen. W. Me Craig: Caves and cysts of the septum pellucidum. Arch. Neura l. Psychla t. Chicago 74 (1955) 259- 266 18 leshima. A .. 1: Kisa. K. Yostuno. S. Tak ash ima . K. Tak eshita: A morpho metri c CT stud y of Down's syndrome showlng small posteri or fossa a nd calcification of basal ganglia . Neuro radi ology 26 11984>493- 498 19 Lesl ie. ~ v. : Cyst of the cavum Vergae. Ca nad. med . Ass. J . 43 (1940) 433- 435 20 Mopstone. T. B.. R. 1. Wh ite: Cavum septi pellucidi as a cause of shunt dysfunction. Surg. Neurol. 16 (198 1196-98 2 1 Miller, D.: Cyst of the fifth ventricle treated by intr aventr icular dr alnage. J. Neurosurg . 6 (1949) 332-333 22 Miller. .\1. E.. D. Kido, F. Hom er: Cavum Vergae . Association with neuro logic ab normalüy and diagnosis by magnetic resonance imaging. Arch . Neurot. 43 (1986) 821-823 23 .\ fohadj er. M.. E. Tes nmar, F. Mundinger: CT-stereotaxic drainage of colloid cysts in the foramen of Monro and the third vent ricle. J. 11
~ euros urg .67(19 87)220 -22 3
Acknewledgements We would like to thank G. Pfister for technical assistance an d V. Son ntag-O' Brie n for prep aring the manuscript.
H
25
26
Refer en ces 1
2
3
Bannteartn. A. : Über den Nachweis von Gehirn mißbild ungen durch das Röntg enbild und übe r se ine klinische Bedeutung. Arch . Psychiat. Nervenkr. 110 (1939) 314-3 64 Berqleiter, H.. L. Fekas: Das Cavum septi pellucidi und Cavum Vergae in Klinik und Röntgenbild. Fortschr. Neuro!' Psychiat. 32 (1964) 361 - 399 Birg. ~v. . F. Mu ndinqer: Computer calculation s of ta rget pa rame ter s for a stereota ctic appa ratus . Acta Neurochir.. Wien 29 (1973) 123-1 29
27
Zl>
29
30
Mundm qer, F.. W Birg: CT-Ster eotaxy in the clinica l routine. ~ eu ros urg .Rev. 7(1984)219-224 Xaka no. S.. II. 1I0j o. K. Kalaoka . S. Yama saki: Age related incidence of cavum septi pellucidi and ca.."Um Vergae on CTsca ns of pcdia tric patien ts. J. Comput. Assist. Tomogr. 5 (198 1)348- 349 Ratzka. M.. N. Soresen. R. Woda rz: Zerebra le Minellinlenfehlbil dungen im a xialen Computertomogra mm . Radiologe 21 (19811 507-515 Riecnert. T.. F. Mundinger: Beschreibung und Anwendu ng eines Zielgerät es Iiir stereotaktische Him opera tionen (2. Modell). Acta Neurochir.. 3 (195 6) 308 -33 7 S ctucidde, J. T.: Incidence of cavum septi pellucidi and cavum Vergae in 1032 human brai ns. Arch. Neurol. Psychlat. Chicago 67 119521625 -632 Scott. .\1.: Cyst of the sixth vent ricle (cavum of Verga l. Success ful remova l throug h tra nsvent ricular app roach with notes on em bryology and histop athology. J. Neurosurg. 2 (1945 ) 191 -201 S I/aU'. C. /J,f .. E. C. Aloo rd jr. : Cava septi pellucidi et Ver gae: thelr norma l a nd pathological stat es. Brain 92 (1969 ) 213-224
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Neurochirurgia34( 199 1J
Image-directed stereotactic drainage
II
JJ J-I
Sonntag. J.. M . f'l.'adjmi. F. Laj osi. G. Fuchs: Anlagebedin gte Gehirn -
anomalien der Mittellinie, Eine entwicklungsgeschichtliche. rad iologische und klinische Studie. Nervenarzt 42 (1971)53 1-539 S tappenbeck. L.. lY. D. Möller. Zur Diagnostik un d Klinik des nichtkommuni zierend en Cavum septi pellucidi. Radiologe 13 11 97 31 5 12-5 14 Van lt'agenen . lY. P.. R. B. Ai rd: Dilatation of cavny of septum pellucidum and cavum Vergae. Amer . J . Cancer 20 11 9341539 -557 Verga. A. : Sul ventri culo della volta a tre pilastri. Gazz med. lombarda 2. Series 3 00. 7. 1851. G. Ist. lombard. Sci. 89. 1955
Dr. J. K. Krauss Neur ochirurgische Universitä tsklinik Hugstetterst r. 55 78 Freiburg Tel. 00 49-761 -270 3601
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Jl
61
Image-directed stereotactic drainage ofthe symptomatic cavum septi pellucidi et vergae J K. Krauss', M. Mohadjer', E. Milios', R. S cheremet', F. Mundinqer' 2
Department of'Neur osurgery Department ol Stereotaxy and Neuronuclear Medicine. Neurosu rgtcal Clinie. Albert- Ludwigs-Universlt ät. Freiburg
3
St. Josefs-Krankenha us. Freiburg
I
Int roducti o n
Two rare cases of symptomatic non-communicating cava se pti pellucidi et Vergae (CSPV) with successful treatme nt via image-directed stereotactic internal drainage are reported. Because tbe management of these ben ign space-occupying structures by the transcallosal a pp roach bears certa in risks , sa fer meth ods should be a pplied. Th e c1inical significan ce, results, and adva ntages of the CT-stereotactic procedure are rep resent ed . If pati ents with symptomatic CSPVa re scheduled for an ope ration, stereotactic interven tion is proposed as the ther apy of choice . Bildges te ue r te ste r eotaktische Drainage des pathologisch veränd erten cavum septi pellucidi et vergae Über zwei seltene Fälle mit sym ptomatischem großem , nicht-kommuni zierend em Cavum sep ti pellucid i et Verga e (CSPY) wird beri chtet. Bei beid en Patienten wurd e ein von bildgeben den Ve rfah ren ges teuerter stereota ktischer Eingriff - eine innere Drainage des Cavums - erfolgreich du rchgeführt. Weil die operative Verso rgung dieser gutartig en raumfo rd ern den Stru kturen über den bislang angewandten Zugang über den Balken gewiss e Risiken beinh altet . sollten sicherere Methoden zur Anwendung kommen . Die klinische Bedeut ung. die Ergebnisse und die Vorteile des CTster eotaktischen Verfahrens werde n da rgest ellt. Wenn bei Pati ent en mit sympt omatische m CSPVein operativer EingrilTgeplant ist. wird das ste reo taktische Verfah ren als Therapie der Wahl vorgeschlagen. Key-Words Cavum se pti pellucidi - Cavum Verga e Neuro-imaging - Stereotaetie surgery
Neurochirurgia 34 ( 99 1) 57- 61 © Georg Thieme Verlag Stuttgart · New York
The cavum septi pellucidi (CS P) a nd the cavum Vergae (CY) a re often refer red to as cere bral midline malformations (I . 8. 26. 32). It is more ap pro priate. however, to eall them persisting primitive structures, as they are perfectJy norm al during the fetal Iife and in pr eterm infan ts (5.6, 10, 30). CSP was already known by a natomists to the seventeent h cent ury (9). When the Italia n anatomist Verga reported his obs ervation s of a cystic forma tion dorsal of the septum pellucidum, the question arose about the clinieal implications ofth ese structures (34). Wheth er or not persisting cava of the midline may have cliniea l signifieance in certain case s is still a cont rovers ial issue (I . 2. 4. 6,11 -13. 15. 30. 31). While there is general agreeme nt ab out their normal developm ental appearanee in fetu ses and in infants in the first month s of life, ther e a re conflicting data, especially conce rn ing CV, a bou t eventua l path ological signifieanee , prevalenee and neurologie or psychiatrie sym ptoms possibly associated (l , 2.4. 6. 7. 11-13 ,1 5. 18.22.30.31). Usually. they should be conside red as coincidenta l variants . There are seve ral cases reported in the liter atu re, howeve r, in which the rela tionship between c1inical sym ptoms and persisting cava could be convincingly dernonstrated (6- 8. 14. 17. 19. 21. 29. 30. 33). We report on two patients who had c1inieal manifestations of a persis ting non -commu nieating cavum septi pellucidi et Ve rga e (CSPY). By CT-base d stereotactie techniques a catheter was introduced in the cavum to establish an internal drainage with the ventricular system. Case rep ort s
Case I This 29-y ear-old man ,..'as adm itted with an 18month history of headach es. accentuat ed after psychic stres s , the n acco mpanied by nausea and sporad le vertig o. For a yea r he had been sufTering increasingly from Iatlgue. difflculty in sleepi ng and wa king ea rly in the morning. He co mplained of exhaustion, irritability and nervousness. and s uITered from a lack of initiative and sponta neity. He had increas ing difficulty coping at work and in stress situat ions showed a nervous tic-like twitehing of the eyelids. One episode of sudden vcmiting was reported . Some lime s he experienced unpleasent tingling painful se nsations in his left arm. Electromyographic and neurographi c examinations produced 0 0 pathological data . Eleetroencephalography revealed no foeus. There wa s no history of head traum a, intraeranial or other Infeclion.
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Sum mary
Neurochi rurgia 34 (1991)
The neurological examina tion showe d diserete optokineüc disturbanees and tendon re flexes whieh were more pronouneed on the right side than on the left. Computerized tomography revealed a CSPV with CSF-isodense eontents . The later al ventricles wer e slightly asymmetrieal and the re were no slgns of hydrocephalus . MRI sea ns disclosed no ad ditional pat hological cha nges (Fig. 1). The cav um had a maximum exte nsio n of 57 x 29 x 23 mm . The CT-stereotactie intervention was done aeeor ding to th e stand ar ds published elsewhere (3, 23, 24, 27). Aeeording to CT seans a fter fixat ion of the stereotaetic ring , a ta rget point was ealculated in the anter ior pa rt of the cavum . After a bur rhole had been plaeed 1 em behind the right coronal sutu re, parasagittally, the dura was opened an d th e ean ula for cystography was introduced in th e cavum. After a spontaneous outflow of clear liquid (3/3 cells, total protein 29.6mg%), contrast medium was introduced. The cavity did not communicate with the ven tricula r system. Then a 8.5cm silicone cathete r with a Riekham reservoir was plaeed to est ablish a n intern al dra inage with th e right later al ventricle and more liquid was aspirated. The operation was performed und er loeal anaesthesia. The postoperative course was uneve ntful and th e symptoms resolved completely after some weeks , There has been no reeurrenee in 2 yea rs. Follow-up CT sea ns revealed the cavum continuously decreasing in stze. the last CT sca n showed only a small rest (Fig. 2). The lateral ventricles remain ed asym metr ical and showed no cha nge in width. The patie nt returned to wor k free of symptoms.
Case 2 This 2 11/12-year-old girl was presen ted with a histcry of psychomotor retardaüon and delayed sta tomotor develcpment. She had been born prematurely in the 35 lh week of gestatio n. At the age of eight wee ks she was brought to the hospital with shock-synd rome. She showe d rotatin g movement of the eyes. museular hypotonia with augmenta tion of muscle tonu s after touching the skin, and intermitte nt shrill serea ming, while being otherwise apathetic. Concomitta nt sympto ms were a miliary exa nthe ma ofthe trunk and th e arms, pne umonia with ate leetas es , hepati tis a nd necrotizing enterocolitis with ileus. The ap athetic state became worse . grip reflexes were missing. Encepha litis was assum ed . Histological exa mination of resected gut revealed necrotizing angiitis. The etiologyof'the aeute disease remain ed unclear . th e imm unological examination detected only nonspeciflc cha nges. a generalized viral infection or a baeterial or funga l sepsis eould not he esta blished. She gra dually reeover ed , yet her further development was delayed and she lea rned to speak only a few words. On admiss ion th e child was alert , but esta blishing contaet with her was difflcult. She was ment ally retard ed . had a vocabula ry of abo ut forty words. her speec h was slurred . and she was highly distr a ctabl e. The neurologlcal exa mina tion disclosed strabis mu s divergens alt ern ans. a lack of visuomotor eoord ination with "optic atax ia" a nd slight intention tre mor . Walking was dlffieult due to gait ataxia and discrete par aparesis. Electroenceph alography showe d a slight genera lized dysr hythmia withou t foeal cha nges. CT scans reveal ed a large CSPV with CSF-isode nse contents with a maximum exte nsio n of6 8 x 31 x 25m m. both later al vent ricles we re enlarged (Fig. 3). The cere bellum was slightly at ro phie. Because of the patien t's age the ste reotactie intervention had to be done with intub ation a nest hesia . To establish a n internal dr a inage with th e ventr icular system a 5.7 cm silicone catheter was placed as desc ribed in case 1. Again, the cavu m did not commu nicate with th e ventricular system. The postoper ative course was unevent ful. Followup CT sea ns showe d that the cavum had diminished in size. the lateral ventricles were slightly smaller th an before, th e cortieal sulci
J. K. Krauss. M. Mohadjer. E. Milios. R. Scheremet. F. MUlldillger
were enla rged (Fig. 4). The remaining ventric ulomega ly was due to inte rnal brain atrop hy. In th e month s following operat ion symptoms improved an d afte r one year she has mad e further progress in he r motor an d in her ment al development.
Discussion The nom enclature with respect to th e anato mical hord ers of the cava used in the liter ature is not always consistent. According to anatomical studies (6) the bord ers of es p are anteriorly the genu of th e corpus callosum , superiorly the body of the corpus callosum, posteriorly the columnae forni cis, inferio rly the ros trum of the corpus callosum and the anterior commissure and laterally the septal lamella e. The bord ers of ev are ant eriorly the columnae forni cis, sup eriorl y the body of the corpus callosurn. posteriorly the splenium of the corpus callosum, inferi orly the psalterium, and later ally the forni ces and the latera l ventr icles. lsolated ev are rare (8, 6, 16). They are usually accompanied by a e s p (28). The cava are normally present during fetal developm ent (6, 30). They develop in the fourth month ofpregnancy and start regression hetween the 30 th and 40 th week of gestation - first the post erior portion (Cv) , then the anterior portion (eSP) (6). They are physiological cavities in pr em atures and infants in the first montbs oflife and can be detected by ultrasound and Cf (5, 10,2 5). A higher pre valenc e in children with Down's syndrome was found ( 8). The prevalence in adults is estabIished mainly on post mort em and pneumoencephalograp hic studies (pEG) and th e frequ ency varies , due in part to methodolo gical differ ences (4, 12 , 17, 28, 31). In a recentl y published study the relative fre quency of es p in adults was 0.73 % in CF, no ev was describ ed (4). There are diver gen t opinions ab out the ra tio of communicating and non-commun icating forms, the term cyst is used in different ways. Whether or not a non-communicating cavum septi pellucidi or cavum Vergae should be called "cyst" seems to be chiefly a sema ntic problem. We pr efer the term "non-cornmunicating cavum", It is ass umed that the communicating forms are asymptoma tic (30). Unfort una tely in th e literature "cavum septi pellucidi" often is used to denominate CSP, ev or e spv. Thus a compa rison of the cas es reported to date is Iimited . The clinical significa nce of the persisting cava demonstrated with PEG has been a subj ect of controversial discussion 0 , 2, 11, 13, 15). The question is still unclarified , as to whethe r in certa in cases there may be an association with symptoms or othe r disorders, ma y it be cas ual or causa !. We consider it to be unlikely that es p with a tr an sver se diam eter of a few millimeters as see n usu ally may produce any clinical symptorns, hut difficulties ari se concern ing ev and space -occupying es pv. In their C'l'studies Nokano et al. could not recognize statistically valid relationships between neurol ogical symptoms and es p, ev and es pv presence in children (25). Yet Mill er et al, did not find ev or es pv in CT sca ns of children witho ut apparent neurologie abnormalities, but in five children ev or es pv was asso ciate d with delayed developme nt (22). A bab y with ev noted at two weeks of age by C'I', which disapp eared on rep eat ed scan, developed norm ally (22). Berg/e iter pr oposed that both the persisting cava as weil as the "ne uro tic" beh avior and the epileptic seizures in his patient s should be interpreted as a result of a common consti tutiona l factor , a
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Flg I
Preoperat ive MRI v iew of patient 1 in the axial plane
lVeurochirurgia 34 (199 1)
Fig. 2 Cl scans of patient 1ontoüow-cp centrotwithdiminishedszeofthe cavum
Fig. 3 PreooeranveaxialCl scans of patient 2 demonstratingthe cavumand the enlarged lateral ventncles
Fig.4 Postoperative Cl scans of patient 2ontcllow-upcontrol showing the introducedsi licone catheter inthe cavum, marked lyreduced in sze
"neuropathische Anlage " ofthe brain (2). Retro spe ctively. it cannot be excluded that at least some ofhis patients, as weil as pati ents described by oth er authors , sutTered from clinical manifestations due to the cava. The relationship betw een dini cal symptoms and CSPV or CV is plaus ible in cases in wh ich operation produced good results (7. 8. 14. 17. 19.21 .29. 33) . Several reports of cava with clinica l symptoms were reviewed by Shaw and A lvord (30). Th ey ditTerentiated between simple and complicated forms of cava, linking com plicated forms with such sequelae as hydrocepha lus. They pro posed th at the clinical symptoms, in conjunction with evidence of considerable pressur e on the su rrounding structures we re due to compression of the septal nuclei and the forn ices. which wou ld result in dysfunction of
the limbic system. Yet the size ofthe cavum does not necessarilycorrelate with c1inical symptoms, as smaller cava may also lead to hydrocephalus (6. 8). According to Bruyn (6). wh o reviewed most of the published reports on series and individual cases , the ass ociated symptoms found in patients with persisting midline cava are non-sp ecific including epileptic seizures, mental. mood or behavioral changes, attacks of headache, increased intracranial pressu re and hemiparesis .
Dandy was the first to establish an operative treatment for symptomatic CSPV or CV. He reported on two pati ents whom 'he ope ra ted on in 1931 by a tra nsca llosa l approach, fenes trating the cava for communication with the
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lmage-directed stereotactic drainage
Neu roehirurgia 34 (1991)
ventricular system (7). In subsequent years other re ports were published describing slightly modified techniques (14, 17,19,2 1,29,33). Over the past decade inform ation about treatm ent ofsymptomatic persist ing midline cava has been sparse. Four patients with comparatively small isolated CV causing hydro cephalus were treated by stereotactically placed internal drainage with good results (8). The risk of complications assoeiated with this form of ster eota ctic intervention is low, as could be also demonstrated in 12 patients with colloid cysts (23), To our knowledge, this method has not been applied in large symptomatic non-communicating CSPV previously. The patient described in case 1 sulTered from headaches and psychological disturbanees, and probably focal seizures. Since all these symptoms showed complete regression after stereota ctic implantation ofthe catheter for inter nal drainage of the cavum with the ventricular system and subs equent cavum shrinkage, we consider the clinical signs to be manifestat ions oft he cavum. \Ve propose that these non-specific symptoms wer e due to compression of the adja cent structures, wher eas occasional episodes of interm itten t hydrocephalus cannot be excluded. In case 2 the CT-stereotactic intervention with subsequent cavum shrinkage additionally aimed to deblock the foramina of Monr o, th us avoiding vent riculoatr ial or ventriculoperitonea l shunt procedures and eventual complications in these cases (20, 30). As not all symptoms were attributable to the cavum the thera peutic benefits may be limited and the procedure cannot be cura tive as in case 1. It is possible that the ass umed encephalitic process is involved with the per sistence of the non-commun icating midline structure. No patient with a CV or CSPV should be opera ted on.just because iti s ther e. Usually these persisting pr imitive stru ctures a re asymptomatic . Irin rar e cases ther e are symptoms that ar e attributable to a non-commun icating CSPV (compression on adjacent struct ures or hydrocephalus) an operative approach may be indicated . As there is a relative small risk, compared to the former applied transcallosal operations, we then would propose Imagedirected (CT or NMR) stereotactic intervention as the therapy of choice.
1. K. Krau se. M. M ohadjer. E. Milios. R. Schere m e t. t:Mundlnqer .. Bogdan ojJ. B.. H. M. Natter: Incidence ofcavum septu m pellucidum in ad ults: a sign of boxer 's encephalopat hy. Neurology 29 (1989) 99 1-992 5 Bohlager. R.. H. M. Straßöurq , M. Sau er: Das Cavum septi pellucidi und Cavum Vergae beim Säugling - Eine Untersuchung mit der zweidimensionale n Sektor- Echo- Enzephalographi e. KUn. Pädiat. 19511983192- 96 I> Brugn. G. W : Agenesis septi pellucldl. cavum pellucidi, ca vum ver gae a nd cavum ..'eli interp ositi.ln: Vinken, P. J.• G. W. Bruyn Ieds.). Hand book of Clinical Neurology. Vol. 30 . Amste rda m. Elsevier. 1977. pp 299-336 7 Dandg. W E.: Congenital cereb ral cysts ofthe cavu m septi pellucidi mnh ventriclel and cavum Vergae Islxth ventrtclel, Arch. Neuro!' Psychia t., Chicago 25 (1931144-66 8 Doneuer. E.. J. R. Moringlane. C. B. Ost ertaq : Cavum Vergae cys t as a cause of hydr ocepbalus. Malmost Iorgotten ?". Acta Neurochi r., Wien 83 (1986) 12-19 9 Duncan. D.: Explication nouvelle et mecanlque des actions ani males. Paris 1678 10 Farruggia . S .. D. S. Babcock: The cavum se pti pellucidi: its a ppea rance and incidence with cran ial ultrasonogra phy in infa ncy. Radiolo~' 13 9 ( 1 98 1 ) t 4 7 - 1 5 0
Feld. M. M.: Sur un cas de kyste du sep tum lucidum revele par des crlses d'epilepsie gene ral isee. Rev. neurol. 92 11955165-68 12 Pink e, J.. G. Koch: Das Cavum septi pellucidi: Vorkommen und Aussagewert. Dlsch. Z. Nerven heilk. 193 11 9681154- 157 13 Göilnitz . G.: Über das klinische Bild im erwe iterte n Cavum septl pellucidi. Drsch. Z. Nerven hellk. 163 (1949) t - 11 H Gubbay. S. 5.. R. Vaughan . J. S. Lekias: lntermlu ent bydro cephalus due to cysts of the septu m pellucidum : a study of three cases. Proc. Aust. Assoc. Neu rol. 14 ( 977) 93- 99 15 Hackst ein. F. G.: Über die Klinik des luftgefüllten. weiten Cav um sep ti pellucidi. Dtsch . Nervenheilk. 177 (1958) 348-369 16 Hea /g. 1. E . G. I. w ickbom. E. Jonon: Computed tomographie illustra tlon of cavum Vergae . J . Comput. Tomogr. 5 (1981) 336-339 17 lIughes. R. A.. J. W. Kernehen. W. Me Craig: Caves and cysts of the septum pellucidum. Arch. Neura l. Psychla t. Chicago 74 (1955) 259- 266 18 leshima. A .. 1: Kisa. K. Yostuno. S. Tak ash ima . K. Tak eshita: A morpho metri c CT stud y of Down's syndrome showlng small posteri or fossa a nd calcification of basal ganglia . Neuro radi ology 26 11984>493- 498 19 Lesl ie. ~ v. : Cyst of the cavum Vergae. Ca nad. med . Ass. J . 43 (1940) 433- 435 20 Mopstone. T. B.. R. 1. Wh ite: Cavum septi pellucidi as a cause of shunt dysfunction. Surg. Neurol. 16 (198 1196-98 2 1 Miller, D.: Cyst of the fifth ventricle treated by intr aventr icular dr alnage. J. Neurosurg . 6 (1949) 332-333 22 Miller. .\1. E.. D. Kido, F. Hom er: Cavum Vergae . Association with neuro logic ab normalüy and diagnosis by magnetic resonance imaging. Arch . Neurot. 43 (1986) 821-823 23 .\ fohadj er. M.. E. Tes nmar, F. Mundinger: CT-stereotaxic drainage of colloid cysts in the foramen of Monro and the third vent ricle. J. 11
~ euros urg .67(19 87)220 -22 3
Acknewledgements We would like to thank G. Pfister for technical assistance an d V. Son ntag-O' Brie n for prep aring the manuscript.
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Refer en ces 1
2
3
Bannteartn. A. : Über den Nachweis von Gehirn mißbild ungen durch das Röntg enbild und übe r se ine klinische Bedeutung. Arch . Psychiat. Nervenkr. 110 (1939) 314-3 64 Berqleiter, H.. L. Fekas: Das Cavum septi pellucidi und Cavum Vergae in Klinik und Röntgenbild. Fortschr. Neuro!' Psychiat. 32 (1964) 361 - 399 Birg. ~v. . F. Mu ndinqer: Computer calculation s of ta rget pa rame ter s for a stereota ctic appa ratus . Acta Neurochir.. Wien 29 (1973) 123-1 29
27
Zl>
29
30
Mundm qer, F.. W Birg: CT-Ster eotaxy in the clinica l routine. ~ eu ros urg .Rev. 7(1984)219-224 Xaka no. S.. II. 1I0j o. K. Kalaoka . S. Yama saki: Age related incidence of cavum septi pellucidi and ca.."Um Vergae on CTsca ns of pcdia tric patien ts. J. Comput. Assist. Tomogr. 5 (198 1)348- 349 Ratzka. M.. N. Soresen. R. Woda rz: Zerebra le Minellinlenfehlbil dungen im a xialen Computertomogra mm . Radiologe 21 (19811 507-515 Riecnert. T.. F. Mundinger: Beschreibung und Anwendu ng eines Zielgerät es Iiir stereotaktische Him opera tionen (2. Modell). Acta Neurochir.. 3 (195 6) 308 -33 7 S ctucidde, J. T.: Incidence of cavum septi pellucidi and cavum Vergae in 1032 human brai ns. Arch. Neurol. Psychlat. Chicago 67 119521625 -632 Scott. .\1.: Cyst of the sixth vent ricle (cavum of Verga l. Success ful remova l throug h tra nsvent ricular app roach with notes on em bryology and histop athology. J. Neurosurg. 2 (1945 ) 191 -201 S I/aU'. C. /J,f .. E. C. Aloo rd jr. : Cava septi pellucidi et Ver gae: thelr norma l a nd pathological stat es. Brain 92 (1969 ) 213-224
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JJ J-I
Sonntag. J.. M . f'l.'adjmi. F. Laj osi. G. Fuchs: Anlagebedin gte Gehirn -
anomalien der Mittellinie, Eine entwicklungsgeschichtliche. rad iologische und klinische Studie. Nervenarzt 42 (1971)53 1-539 S tappenbeck. L.. lY. D. Möller. Zur Diagnostik un d Klinik des nichtkommuni zierend en Cavum septi pellucidi. Radiologe 13 11 97 31 5 12-5 14 Van lt'agenen . lY. P.. R. B. Ai rd: Dilatation of cavny of septum pellucidum and cavum Vergae. Amer . J . Cancer 20 11 9341539 -557 Verga. A. : Sul ventri culo della volta a tre pilastri. Gazz med. lombarda 2. Series 3 00. 7. 1851. G. Ist. lombard. Sci. 89. 1955
Dr. J. K. Krauss Neur ochirurgische Universitä tsklinik Hugstetterst r. 55 78 Freiburg Tel. 00 49-761 -270 3601
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