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Spinal intradural arachnoid cysts Y. St ern. R. Sp ieqelmann. M. Sadeh Departm eruof'Neurology a nd Neurosurgery.The Chat m Sheba Medt cal Center. Tel Hash omer and Sackler Schoo l of'Medicin e . Tel Aviv Unive rs lty, Israel

Case Reports

Three pati ents with indural spina l arachnoid cysts presented with back or neck pain of several months' du ration. They gradually developed weakness and difficulty in wa lking followed hy rapid deterioration with signs of spinal cord or nerve root cornpress ion. Radiologieal studies sh owed a posteriorly located mass in the cervical, dorsal and lum ba r region ; one cyst filled with contrast medium and the diagno sis of the others was made at operation. Surg er y resulted in full recover y oftwo patien ts a nd partial recovery ofthe third. Spinale intradur al e Arach noida lzysten 3 Patienten mit intraduralen spinalen Arachnoida lzysten hallen Rücken - bzw. Nackenschme rzen für mehrere Monate. Sie entw ickelten allmählich Schwäche in den Bein en und Schwierigkeiten beim Geh en. gefolgt von eine r ra piden Verschlecht erung mit Zeichen de r Wurzel- oder myelären Kompr ession. Radi ologisch e Untersuchungen zeigten eine dor sa le lokalisierte Raumford erung in der zervikalen, thorakalen oder lum balen Region . Eine Zyste füllte sich mit Kontrastmittel, die Diagnose der anderen erfolgte während der Operation. Die chirurgischen Ergebnisse zeigten eine völlige Wied erherstellung bei zwei Patienten und eine teilweise Erh olung bei ein em Patient en . Key-Words Spin al arachnoid cysts - Spina l cord com pression

Spinal extradura l or intradural a rachnoid cysts are frequ en tly observ ed during myelogr aph y but only rarely com pre ss the spinal cord , producing neurological deficits (3. 6. 9. 10). During one yea r we encountere d 3 cases of symptomatic spinal arachnoid cysts located a t the cervical, thoracic and lumbar regions resp ectively. The c1inical, radiological and operative findings in these patient s are described.

Neurochirurgia 34 ( 99 1) 127-1 30 © Georg Thieme Verlag Stuttgart New Yo rk

Case 1. A 56 year old wc ma n was a dmitte d to th e depar tm en t of neurology becau se of low back pain and difflculty in wa lking of 6 mo nths ' duration. Past history was otherwise unremar ka ble. The gene ra l physical exa mina tion was norm al. Neu rolo gical examination revealed s pastici ty of lower Iimbs but no we akn ess. Patellar and Ach illes reflexes we re brisk bilaterally an d pla ntar responses flexor : pin-p rick sensation as mildl y an d inco nsi ste ntly decreased below the level ofTs. Spine X-rays showed spondyloa rthrotic cha nges . On metr izamid e myelogra phy, cent ras t medium was obse rve d to I lowfree ly in the dorsal ca na l with no sign of cord cornpressio n. A post -myelogr ap hy compute rize d tomog ra phy (CT) revealed only a slight central disc pr otru sion at T1O- T I1 ; this finding was not conside red to be the ca use of her compla ints a nd s he was disc harged to outpatie nt clinic follow up. Nine months later the patient noticed weakness of both legs and difficulty in urination . Weakness rapid ly pr ogressed a nd sh e was rea dmitted. Severe sp ast ic parap ar esls with bilate ra l ankl e clon us and extensor plan ta r resp onses were now noted . All sensory modali ties wer e irnpai red below the level of T 3. Urina ry retention necessitated an indwe lling cat heter. Metrizamide myelography showed a co mplete hold-u p ofco ntrast medium et T 3 (Fig. 1) and on myelograp hy-CT sca n, l1att enin g an d ante rior d isplacement of the spinal cord d ue to a dorsally located contrast filled lesion (Fig. 2). On laminectom y at T 2-T 4 a n arachnoid cyst was secn on opcning the dura fro m wh lch CSF und er pr essure escaped on incision . The arac hno ld membran e was thickened and in some a reas adhered to the dura mater. Th e cyst wall was pa rtially excised: un derlying s pina l cor d a ppea red normal and no othe r lesion was obse rved. Microscopic exa minat ion ofthe cyst wall showed fibrous tissue with ede ma an d mild lyrnphocytlc infiltration. On the ensu ing days ma rked improvem en t was noted and the patie nt was discharged walking witho ut aid, with normal ur ina ry funetio n. Seen two years late r. she wa s sign and sym pto m-free.

Case 2. A 68 yea r old hypertensive ma le started 10 suffer fro m neck pain four months before admission , agg ravated by head movem en ts and from pr ogr essive weakness of a11 four limbs du ring the prev ious two weeks. Neck movem en ts in all directio ns were limited by pain. He was qua driparetic with marked atroph y of the upp er lim b pr oximal muscles and mild spasticity of th o lower limbs . Tendo n re flexes were hyperactive and plan tar res po nses exte nsor. Hypoesthesia was noted below the level of C3-C4 . Radi ographs of the cervieal spine were normal; metrizami de myelogr aph y s howe d obstruc tion of ce ntrast medium flow at C, with d ispla ceme nt of th e cord to the right (Fig. 3). C1-C2 lateral punctu re myelography out lined the up per level of the obstruction at C2-C 3 (Fig. 4). Laminectomy from C2 to Cf> exposed a poste rio rly located intradural swelling und er high pr essure, fro m which clear CSF bu rst on incision . Th e a rac h noid was th ickened a nd since no other pathology was d isclosed the operat ive diagnosis was cor d compression by an arae hno id cyst. Histological cxa mination ofthe cyst wal l showe d ede mato us fibrous tissue with mild inflammatory inflltratlon .

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Summary

Neurochirurgia 34 (1991 J

Y Stern. R. Spi eqelmann. M. Sadeh Fig.la

Fig. 2 Case 1- Cl-myelography. Flatteningandantericr displacementof the spinal cord dueto a dorsallylocated centrast filled scherteer cystic lesicn Downloaded by: University of Pennsylvania Libraries. Copyrighted material.

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Fig.lb

Fig. 1 Case 1- Metrizamidemyelography. A.APviewB. Lateral view:A postenorlylocated mass causesanalmostcorro'ete obstructionoferentatflow of contrastmedium

Fig. 3 Case 2- Metrizamidemyelography APview.Centrast med iumflow ts blockedat C5 with displacement of thecord to the right

Seve re weakne ss , Iimited howeve r to the uppe r limbs, followe d the ope ra tion but after several days gra dua lly impr oved a nd eve ntually the pa tie nt was abl e to wa lk ind epen den tly, with only m oder ate res idual weakncss ofthe left arm .

Lumb ar a nd tho racic radio gra phs were norm al. Nerve cond uction velocity stud ies were normal but electro myogra phy s howe d mild de nerv ation cha nges in the right ant erior tibial and glute us m aximu s mus eIes.

Case 3. A 16 year old boy com pla ine d ofl ow back pai n radiating to the th ighs of 6 we eks ' duratio n and for flve days befor e ad m ission , wea kness of the legs a nd difficulty in wa lking. The gene ra l ph ysical exa minaüon was normal. The re was seve re bila ter al weakncss of the iIiop soas an d glutei, and moder ate we akness of the dist a l m useIes of the lower Iim bs. Tondo n reflexes wer e norm al in upper Iimbs an d absent in the lowc r. Pla ntar r es pon ses were flexor an d se ns atio n wa s d im inis hed belaw Lz level.

In the ens uing da ys he developed sovcr o parapar esis acco m pa nied by sensory deficit belo w the level of Lz. On m etr izam ide myelograp hy flow of contrast was slowc d at tho L4 -L2 level and it ente r ed the a nte rior suba rac hno id space only. CSF obtai ne d befo re injection wa s clea r a nd contained 900 mg% protein (Fig. 5). Post -myelograph y CTscan showe d nor ma l spinal sac at L1, bu t no contrast med ium at 1.2 an d 1.3 ; ca uda equina r oots below th is level ap peared no rma l at L4 (Fig. 6).

Spinal intradural oraclmotd custs

Neurochiru rgia 34 (1991)

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Fig.4 Gase 2- C,-Czlateralpuncturemyelographyshows a completeblock of metrizamide ftow at C2- C3 Ievel. No displacement of the spinal cord ts seen

Urgent Lz-L3 laminectomy was performed. The epidural spaee appea red nor mal. but on incislon ofthc dura ma ter CSF burst out under pr essure: cauda equina roots wcre engorged but no other pat hology was encount er ed. The surgi cal findings were believed to be compatlble with tearing of a n ar achnoid eyst at the time the dura mate r was ope ned. wlth consequent relief of pressure on the ca uda equina . Ra pid recovery followed . the patient was discha rged with no ne urological de flcit. and remained fit when see n 2 yea rs later.

Fig. Sb

Discussion The arac hnoid cysts in these 3 patients located at different regions along the spinal axis, all eaused pain for weeks or months, th en the gra dua l development of limb weakness. followed by sudden seve re deteri oration with signs of spinal cord or cauda equina compression occurring seve ra l days before hospitalizati on. All cysts were intradural an d situated posterior to the neu roaxis. Two did not fill with contrast med ium and were not diagnosed pr ior to operation. The etiology of spina l araehnoid eysts is obseure . Congenilal (7. 8). post-traumatie (9.10) an d inflammatory (4.5) theor ies have been postulated. However the ages of two ofo ur patients, 56 and 68 years, and the lack ofany history of trau ma sugges t that inflammation preeeding eyst form ati on is the most plausible explana tion in our cas e. This ass um ption is supported by the pathologieal findings of thiekened arae hno id mem brane with edema and mild cellular infiltra tion in two patients (no pathological exa minati on was performed in the third), Myelogra phy preeeding the neuro logieal deterior ation by 9 months might have bee n the cause of ar achnoiditis in case 1. lt is also possible that congenital arachnoid diverticula asymptoma tic for man y yea rs become infl am ed and change their nature. The norma l first myelograp hy in case 1 does not rule out this possi-

Fig.5 Case 3 - Metrtzarnide myelography A. AP view. B. Lateral supine view. Centrastmediumflowwas delayed at L2 -L 4 where somecontrast materialwasseen onlyattheanterior aspectof the subarachnoid space. Nocontrsst mediumwasobserved pcstenorly even onthe lateral supine view

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Fig. 5a

Y. S tern. R. S piegelmarm, M. S adeh

Xeuroch irurgia 34 (1991) a

b

In most reported cases the cysts were visuaIized since they mied with contrast medium (2. 3. 10). The ro utine use ofwater soluble medium an d perfor ming a postmyelogra phy CTscan have now increased the prob ab ility of thei r detection. However , even these techni ques failed to demonstra te the cysts in two of our patients. When an intradu ral extramedullary und iagnosed mass is poste riorly located, an arac hnoid cyst should none the less be suspected: a delayed CT scan may show opacification of the cyst fluid. The treat men t of choice of a symptomatie ara chnoid cyst is surgical removal ofas much ofi ts wa ll as is safe (10.11). Since inadequate drain age may lead to recurrence of cyst growt h and spina i comp ress ion (1). In almost all cas es so treat ed pain was quickly relieved, but full neu rological restitution was not always achieved (3. 10). The prognosis depends ma inly on the duration of spinal cord or ne rve root cornpression an d early diagnosis an d treatment are rnandatory for a good recovery. Recently, it has been reported that late worsening of various degree s occurs in some patients postopera tively. This has not happ ened so far in our patients over aperiod of 2- 3 years follow-up. However, a nothe r patient previously reported by one of us (91 deterlorated 2 years after su rgery: repeat ed CT-myelogra phy showed recurr ence ofa sma ll cyst. She was reoperated on and the cyst was removed with return to previous intact neurological status. Perhaps some ofthe poor long term results are due to cyst recurrence, a point not discussed in a long-ter m study (1). Referen ces

c.. ,\4. Ceri soli. M . Giulioni. L. Guerr a: Leng-term results of su rgically treated congen ital intrad ura l spinal a rachn oid cysts. J .

, Atrin,

~eu ros u rg .6 7( 1 98 7l 33 3-335

Jacobs. L. G.. J. K. Smt th. P. S. VaT/ Horn: Myelograph ic dc monstration of cysts of spinal membr a nes. Rad iology 62 (195412 15- 22 1 a Kendat. 8. E.. A. H. vatenune, lJ. Keis: Spinal ar achnoid cysts : Clinical a nd rad iologieal correlauc n with pr ognosis. Neuroradiology 22 (1982) 225-2 34 4 Lonq. H. IV.. l".... Rachmaninoff: Spinal adhes tve ar achnoiditis with cyst formatic n: injectio n of eyst d uring myelograph y. Case report . J. Keu rosurg .27( 1967l 73-76 5 Meson. M. S .. J. Haar Complicat ions of pa ntopaque myelograph y. Case rep or t a nd revtew. J. Neuros urg . 19 (1962) 302-3 11 (, Patmer. J. J.: Spinal arac hnoid cysts . Hepo rt of 6 cases. J. Neurosurg . 4 111974) 728- 735 7 Perr et. G.,D.Green, 1. Keller: Diagnosis an d trea tme nt ofi ntradu ra l ar achnoid cysts ofthe tho racic spi ne. Radi ology 79 (1962 )425-4 29 II Haja. l. A .. J. ttankineon: Congenit al spinal a rac hnoid cysts . Report of two cas es a nd revlew of literat ure. J. Neura l. Neuros urg. Psychiatr. 33 ( 970) 105- 110 9 Stneqelmann, R; Z. 11. Rapp cpo rt . A. Sc har: Spina l a rac hnoid eyst with unusua l pr esen tation. J. Neuros urg. 60 (1984 ) 61 3- 616 \ 0 S te uxut. D. II.jr., D. E. Re d: Spinal a ra ehno id dive rtieula. J . Neu rosurg.3 5( 197 1165 -70 11 Zumpono.B, J.. H. L. S c und ers: Lumba r intradura l ar ach noid dtvertieulum wlth ca uda eq uina compression. Surg . Neuro l. 5 (1976 ) 349 -353 2

Fig. 6 Case 3 - Cl -myelography.A.l , level. B. l ) Ievel.C. L ievet Normal subarachnoidspaee andnerve rootswere seen at LI andL fevels. however. no centrast medium was observableat l ] (ablackspotat L tevel ls anair bubble from the myeJography)

bi lity sin ce an arachnoid cyst may fail to be visualized by myelogra phy especially if su plne views and post-rnyelography CT at the cyst level are not undertaken. ln allthreecases CSF er upted from the cyst with high pressure , indicating presumably that CSF was trap ped within the cyst by a valve meehanism which, if a reeent developme nt eould aeeount for the aeute c1inical aggravation.

Mena chem Sadeh . M. D.

Depa rtme nt of'Ne urology The Chaim Shcba Medical Center Tel Hashomer 5262 1 Tel Aviv/lsrae l

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Spinal intradural arachnoid cysts.

Three patients with indural spinal arachnoid cysts presented with back or neck pain of several months' duration. They gradually developed weakness and...
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