Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension

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✓ Spinal cerebrospinal fluid (CSF) leaks are often implicated as the cause of the syndrome of spontaneous intracranial hypotension, but they have rarely been demonstrated radiographically or surgically. The authors reviewed their experience with documented cases of spinal CSF leaks of spontaneous onset in 11 patients including their surgical observations in four of the patients.

The mean age of the six women and five men included in the study was 38 years (range 22–51 years). All patients presented with a postural headache; however, most had additional symptoms, including nausea, emesis, sixth cranial-nerve paresis, or local back pain at the level of the CSF leak. All patients underwent indium-111 radionucleotide cisternography or computerized tomographic (CT) myelography. The location of the spontaneous CSF leak was in the cervical spine in two patients, the cervicothoracic junction in three patients, the thoracic spine in five patients, and the lumbar spine in one patient. The false negative rate for radionucleotide cisternography was high (30%). Subdural fluid collections, meningeal enhancement, and downward displacement of the cerebellum, resembling a Chiari I malformation, were commonly found on cranial imaging studies. In most patients, the symptoms resolved in response to supportive measures or an epidural blood patch. Leaking meningeal diverticula were found to be the cause of the CSF leak in four patients who underwent surgery. In three patients these diverticula could be ligated with good result but in one patient an extensive complex of meningeal diverticula was found to be inoperable. Two patients had an unusual body habitus and joint hypermobility, and two other patients had suffered a spontaneous retinal detachment at a young age.

In conclusion, spontaneous spinal CSF leaks are uncommon, but they are increasingly recognized as a cause of spontaneous intracranial hypotension. Most spinal CSF leaks are located at the cervicothoracic junction or in the thoracic spine, and they may be associated with meningeal diverticula. The radiographic study of choice is CT myelography. The disease is usually self-limiting, but in selected cases our experience with surgical ligation of leaking meningeal diverticula has been satisfactory. An underlying connective tissue disorder may be present in some patients with a spontaneous spinal CSF leak.

Article Information

Contributor Notes

Address reprint requests to: Wouter I. Schievink, M.D., Department of Neurologic Surgery, Mayo Clinic, 200 First St., SW, Rochester, Minnesota 55905.

© AANS, except where prohibited by US copyright law.

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References
  • 1.

    Beighton P: Serious ophthalmological complications in the Ehlers-Danlos syndrome. Br J Ophthalmol 54:2632681970Beighton P: Serious ophthalmological complications in the Ehlers-Danlos syndrome. Br J Ophthalmol 54:263–268 1970

    • Search Google Scholar
    • Export Citation
  • 2.

    Bell WEJoynt RJSahs AL: Low spinal fluid pressure syndromes. Neurology 10:5125211960Bell WE Joynt RJ Sahs AL: Low spinal fluid pressure syndromes. Neurology 10:512–521 1960

    • Search Google Scholar
    • Export Citation
  • 3.

    Bourekas ECLewin JSLanzieri CF: Postcontrast meningeal MR enhancement secondary to intracranial hypotension caused by lumbar puncture. J Comput Assist Tomogr 19:2993011995Bourekas EC Lewin JS Lanzieri CF: Postcontrast meningeal MR enhancement secondary to intracranial hypotension caused by lumbar puncture. J Comput Assist Tomogr 19:299–301 1995

    • Search Google Scholar
    • Export Citation
  • 4.

    Capobianco DJKuczler FJ Jr: Case report: primary intracranial hypotension. Milit Med 155:64661990Capobianco DJ Kuczler FJ Jr: Case report: primary intracranial hypotension. Milit Med 155:64–66 1990

    • Search Google Scholar
    • Export Citation
  • 5.

    Davenport RJChataway SJSWarlow CP: Spontaneous intracranial hypotension from a CSF leak in a patient with Marfan's syndrome. J Neurol Neurosurg Psychiatry 59:2131995 (Abstract)Davenport RJ Chataway SJS Warlow CP: Spontaneous intracranial hypotension from a CSF leak in a patient with Marfan's syndrome. J Neurol Neurosurg Psychiatry 59:213 1995 (Abstract)

    • Search Google Scholar
    • Export Citation
  • 6.

    Dunbar SAKatz NP: Failure of delayed epidural blood patching to correct persistent cranial nerve palsies. Anesth Analg 79:8068071994Dunbar SA Katz NP: Failure of delayed epidural blood patching to correct persistent cranial nerve palsies. Anesth Analg 79:806–807 1994

    • Search Google Scholar
    • Export Citation
  • 7.

    Erkulvrawatr SEl Gammal THawkins Jet al: Intrathoracic meningoceles and neurofibromatosis. Arch Neurol 36:5575591979Erkulvrawatr S El Gammal T Hawkins J et al: Intrathoracic meningoceles and neurofibromatosis. Arch Neurol 36:557–559 1979

    • Search Google Scholar
    • Export Citation
  • 8.

    Fishman RADillon WP: Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology 43:6096111993Fishman RA Dillon WP: Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology 43:609–611 1993

    • Search Google Scholar
    • Export Citation
  • 9.

    Garcia-Albea ECabrera FTejeiro Jet al: Delayed postexertional headache, intracranial hypotension and racket sports. J Neurol Neurosurg Psychiatry 55:9751992 (Letter)Garcia-Albea E Cabrera F Tejeiro J et al: Delayed postexertional headache intracranial hypotension and racket sports. J Neurol Neurosurg Psychiatry 55:975 1992 (Letter)

    • Search Google Scholar
    • Export Citation
  • 10.

    Gaukroger PBBrownridge P: Epidural blood patch in the treatment of spontaneous low CSF pressure headache. Pain 29:1191221987Gaukroger PB Brownridge P: Epidural blood patch in the treatment of spontaneous low CSF pressure headache. Pain 29:119–122 1987

    • Search Google Scholar
    • Export Citation
  • 11.

    Gibson BEWedel DJFaust RJet al: Continuous epidural saline infusion for the treatment of low CSF pressure headache. Anesthesiology 68:7897911988Gibson BE Wedel DJ Faust RJ et al: Continuous epidural saline infusion for the treatment of low CSF pressure headache. Anesthesiology 68:789–791 1988

    • Search Google Scholar
    • Export Citation
  • 12.

    Good DCGhobrial M: Pathologic changes associated with intracranial hypotension and meningeal enhancement on MRI. Neurology 43:269827001993Good DC Ghobrial M: Pathologic changes associated with intracranial hypotension and meningeal enhancement on MRI. Neurology 43:2698–2700 1993

    • Search Google Scholar
    • Export Citation
  • 13.

    Gormley JB: Treatment of postspinal headache. Anesthesiology 21:5655661960 (Letter)Gormley JB: Treatment of postspinal headache. Anesthesiology 21:565–566 1960 (Letter)

    • Search Google Scholar
    • Export Citation
  • 14.

    Greco GMAmbrosino L: Treatment of retinal detachment in Marfan syndrome. Ann Ophthalmol 25:72761993Greco GM Ambrosino L: Treatment of retinal detachment in Marfan syndrome. Ann Ophthalmol 25:72–76 1993

    • Search Google Scholar
    • Export Citation
  • 15.

    Hochman MSNaidich TPKobetz SAet al: Spontaneous intracranial hypotension with pachymeningeal enhancement on MRI. Neurology 42:162816301992Hochman MS Naidich TP Kobetz SA et al: Spontaneous intracranial hypotension with pachymeningeal enhancement on MRI. Neurology 42:1628–1630 1992

    • Search Google Scholar
    • Export Citation
  • 16.

    Horton JCFishman RA: Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak. Ophthalmology 101:2442511994Horton JC Fishman RA: Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak. Ophthalmology 101:244–251 1994

    • Search Google Scholar
    • Export Citation
  • 17.

    Huber M: Die spontane Hypoliquorrhoe. Darlegung von sieben eigenen Beobachtungen. Schweiz Arch Neurol Neurochir Psychiat 106:9231970Huber M: Die spontane Hypoliquorrhoe. Darlegung von sieben eigenen Beobachtungen. Schweiz Arch Neurol Neurochir Psychiat 106:9–23 1970

    • Search Google Scholar
    • Export Citation
  • 18.

    Jacobs MBWasserstein PH: Spontaneous intracranial hypotension. An uncommon and underrecognized cause of headache. West J Med 155:1781801991Jacobs MB Wasserstein PH: Spontaneous intracranial hypotension. An uncommon and underrecognized cause of headache. West J Med 155:178–180 1991

    • Search Google Scholar
    • Export Citation
  • 19.

    Kraemer GHopf HCEissner D: CSF hyperabsorption: a cause of spontaneous low CSF pressure headache. Neurology 37 (Suppl 1):2381987 (Abstract)Kraemer G Hopf HC Eissner D: CSF hyperabsorption: a cause of spontaneous low CSF pressure headache. Neurology 37 (Suppl 1):238 1987 (Abstract)

    • Search Google Scholar
    • Export Citation
  • 20.

    Kumar RTaha JGreiner AL: Herniation of the spinal cord. Case report. J Neurosurg 82:1311361995Kumar R Taha J Greiner AL: Herniation of the spinal cord. Case report. J Neurosurg 82:131–136 1995

    • Search Google Scholar
    • Export Citation
  • 21.

    Labadie ELvan Antwerp JBamford CR: Abnormal lumbar isotope cisternography in an unusual case of spontaneous hypoliquorrheic headache. Neurology 26:1351391976Labadie EL van Antwerp J Bamford CR: Abnormal lumbar isotope cisternography in an unusual case of spontaneous hypoliquorrheic headache. Neurology 26:135–139 1976

    • Search Google Scholar
    • Export Citation
  • 22.

    Madigan WPWertz DCockerham GCet al: Retinal detachment in osteogenesis imperfecta. J Pediatr Ophthalmol Strabismus 31:2682691994Madigan WP Wertz D Cockerham GC et al: Retinal detachment in osteogenesis imperfecta. J Pediatr Ophthalmol Strabismus 31:268–269 1994

    • Search Google Scholar
    • Export Citation
  • 23.

    Marcelis JSilberstein SD: Spontaneous low cerebrospinal fluid pressure headache. Headache 30:1921961990Marcelis J Silberstein SD: Spontaneous low cerebrospinal fluid pressure headache. Headache 30:192–196 1990

    • Search Google Scholar
    • Export Citation
  • 24.

    Masuzawa HNakayama HShitara Net al: Spinal cord herniation into a congenital extradural arachnoid cyst causing Brown-Séquard syndrome. Case report. J Neurosurg 55:9839861981Masuzawa H Nakayama H Shitara N et al: Spinal cord herniation into a congenital extradural arachnoid cyst causing Brown-Séquard syndrome. Case report. J Neurosurg 55:983–986 1981

    • Search Google Scholar
    • Export Citation
  • 25.

    McCrum CWilliams B: Spinal extradural arachnoid pouches. Report of two cases. J Neurosurg 57:8498521982McCrum C Williams B: Spinal extradural arachnoid pouches. Report of two cases. J Neurosurg 57:849–852 1982

    • Search Google Scholar
    • Export Citation
  • 26.

    Miles JPennybacker JSheldon P: Intrathoracic meningocele. Its development and association with neurofibromatosis. J Neurol Neurosurg Psychiatry 32:991101969Miles J Pennybacker J Sheldon P: Intrathoracic meningocele. Its development and association with neurofibromatosis. J Neurol Neurosurg Psychiatry 32:99–110 1969

    • Search Google Scholar
    • Export Citation
  • 27.

    Mokri BKrueger BRMiller GMet al: Meningeal gadolinium enhancement in low pressure headaches. Ann Neurol 30:2942951991 (Abstract)Mokri B Krueger BR Miller GM et al: Meningeal gadolinium enhancement in low pressure headaches. Ann Neurol 30:294–295 1991 (Abstract)

    • Search Google Scholar
    • Export Citation
  • 28.

    Mokri BParisi JEScheithauer BWet al: Meningeal biopsy in intracranial hypotension. Meningeal enhancement on MRI. Neurology 45:180118071995Mokri B Parisi JE Scheithauer BW et al: Meningeal biopsy in intracranial hypotension. Meningeal enhancement on MRI. Neurology 45:1801–1807 1995

    • Search Google Scholar
    • Export Citation
  • 29.

    Molins AAlvárez JSumalla Jet al: Cisternographic pattern of spontaneous liquoral hypotension. Cephalalgia 10:59651990Molins A Alvárez J Sumalla J et al: Cisternographic pattern of spontaneous liquoral hypotension. Cephalalgia 10:59–65 1990

    • Search Google Scholar
    • Export Citation
  • 30.

    Murros KFogelholm R: Spontaneous intracranial hypotension with slit ventricles. J Neurol Neurosurg Psychiatry 46:114911511983Murros K Fogelholm R: Spontaneous intracranial hypotension with slit ventricles. J Neurol Neurosurg Psychiatry 46:1149–1151 1983

    • Search Google Scholar
    • Export Citation
  • 31.

    Nabors MWPait TGByrd EBet al: Updated assessment and current classification of spinal meningeal cysts. J Neurosurg 68:3663771988Nabors MW Pait TG Byrd EB et al: Updated assessment and current classification of spinal meningeal cysts. J Neurosurg 68:366–377 1988

    • Search Google Scholar
    • Export Citation
  • 32.

    Nosik WA: Intracranial hypotension secondary to lumbar nerve sleeve tear. JAMA 157:111011111955Nosik WA: Intracranial hypotension secondary to lumbar nerve sleeve tear. JAMA 157:1110–1111 1955

    • Search Google Scholar
    • Export Citation
  • 33.

    Pannullo SCReich JBKrol Get al: MRI changes in intracranial hypotension. Neurology 43:9199261993Pannullo SC Reich JB Krol G et al: MRI changes in intracranial hypotension. Neurology 43:919–926 1993

    • Search Google Scholar
    • Export Citation
  • 34.

    Pappas DG JrHoffman RACohen NLet al: Spontaneous temporal bone cerebrospinal fluid leak. Am J Otol 13:5345391992Pappas DG Jr Hoffman RA Cohen NL et al: Spontaneous temporal bone cerebrospinal fluid leak. Am J Otol 13:534–539 1992

    • Search Google Scholar
    • Export Citation
  • 35.

    Paulson GWKlawans HL Jr: Benign orgasmic cephalgia. Headache 13:1811871974Paulson GW Klawans HL Jr: Benign orgasmic cephalgia. Headache 13:181–187 1974

    • Search Google Scholar
    • Export Citation
  • 36.

    Pemberton JWFreeman HMSchepens CL: Familial retinal detachment and the Ehlers-Danlos syndrome. Arch Ophthalmol 76:8178241966Pemberton JW Freeman HM Schepens CL: Familial retinal detachment and the Ehlers-Danlos syndrome. Arch Ophthalmol 76:817–824 1966

    • Search Google Scholar
    • Export Citation
  • 37.

    Pyeritz REFishman EKBernhardt BAet al: Dural ectasia is a common feature of the Marfan syndrome. Am J Hum Genet 43:7267321988Pyeritz RE Fishman EK Bernhardt BA et al: Dural ectasia is a common feature of the Marfan syndrome. Am J Hum Genet 43:726–732 1988

    • Search Google Scholar
    • Export Citation
  • 38.

    Rando TAFishman RA: Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 42:4814871992Rando TA Fishman RA: Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 42:481–487 1992

    • Search Google Scholar
    • Export Citation
  • 39.

    Rohr-Le Floch JGauthier G: Hypotension spontanée du liquide céphalo-rachidien: intérêt de l'IRM cérébrale. Schweiz Med Wochenschr 123:9829861993Rohr-Le Floch J Gauthier G: Hypotension spontanée du liquide céphalo-rachidien: intérêt de l'IRM cérébrale. Schweiz Med Wochenschr 123:982–986 1993

    • Search Google Scholar
    • Export Citation
  • 40.

    Rupp SMWilson CB: Treatment of spontaneous cerebrospinal fluid leak with epidural blood patch. Case report. J Neurosurg 70:8088101989Rupp SM Wilson CB: Treatment of spontaneous cerebrospinal fluid leak with epidural blood patch. Case report. J Neurosurg 70:808–810 1989

    • Search Google Scholar
    • Export Citation
  • 41.

    Sable SGRamadan NM: Meningeal enhancement and low CSF pressure headache. An MRI study. Cephalalgia 11:2752761991Sable SG Ramadan NM: Meningeal enhancement and low CSF pressure headache. An MRI study. Cephalalgia 11:275–276 1991

    • Search Google Scholar
    • Export Citation
  • 42.

    Sathi SStieg PE: “Acquired” Chiari I malformation after multiple lumbar punctures: case report. Neurosurgery 32:3063091993Sathi S Stieg PE: “Acquired” Chiari I malformation after multiple lumbar punctures: case report. Neurosurgery 32:306–309 1993

    • Search Google Scholar
    • Export Citation
  • 43.

    Schaltenbrand G: Neuere Anschauungen zur Pathophysiologie der Liquorzirkulation. Zentralbl Neurochir 3:2902991938Schaltenbrand G: Neuere Anschauungen zur Pathophysiologie der Liquorzirkulation. Zentralbl Neurochir 3:290–299 1938

    • Search Google Scholar
    • Export Citation
  • 44.

    Schievink WIReimer RFolger WN: Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. Case report. J Neurosurg 80:7367391994Schievink WI Reimer R Folger WN: Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. Case report. J Neurosurg 80:736–739 1994

    • Search Google Scholar
    • Export Citation
  • 45.

    Snead MPPayne SJBarton DEet al: Stickler syndrome: correlation between vitreoretinal phenotypes and linkage to COL 2A1. Eye 8:6096141994Snead MP Payne SJ Barton DE et al: Stickler syndrome: correlation between vitreoretinal phenotypes and linkage to COL 2A1. Eye 8:609–614 1994

    • Search Google Scholar
    • Export Citation
  • 46.

    Stern WE: Dural ectasia and the Marfan syndrome. J Neurosurg 69:2212271988Stern WE: Dural ectasia and the Marfan syndrome. J Neurosurg 69:221–227 1988

    • Search Google Scholar
    • Export Citation
  • 47.

    Stickler GBBelau PGFarrell FJet al: Hereditary progressive arthro-ophthalmopathy. Mayo Clin Proc 40:4334551965Stickler GB Belau PG Farrell FJ et al: Hereditary progressive arthro-ophthalmopathy. Mayo Clin Proc 40:433–455 1965

    • Search Google Scholar
    • Export Citation
  • 48.

    Tarlov IM: Spinal perineurial and meningeal cysts. J Neurol Neurosurg Psychiatry 33:8338431970Tarlov IM: Spinal perineurial and meningeal cysts. J Neurol Neurosurg Psychiatry 33:833–843 1970

    • Search Google Scholar
    • Export Citation
  • 49.

    Teng PPapatheodorou C: Primary cerebrospinal fluid hypotension. Bull Los Angeles Neurol Soc 33:1211281968Teng P Papatheodorou C: Primary cerebrospinal fluid hypotension. Bull Los Angeles Neurol Soc 33:121–128 1968

    • Search Google Scholar
    • Export Citation
  • 50.

    Weber WEJHeidendal GAKde Krom MCTFM: Primary intracranial hypotension and abnormal radio-nuclide cisternography. Report of a case and review of the literature. Clin Neurol Neurosurg 93:55601991Weber WEJ Heidendal GAK de Krom MCTFM: Primary intracranial hypotension and abnormal radio-nuclide cisternography. Report of a case and review of the literature. Clin Neurol Neurosurg 93:55–60 1991

    • Search Google Scholar
    • Export Citation
  • 51.

    Welch KShillito JStrand Ret al: Chiari I “malformation”—an acquired disorder? J Neurosurg 55:6046091981Welch K Shillito J Strand R et al: Chiari I “malformation”—an acquired disorder? J Neurosurg 55:604–609 1981

    • Search Google Scholar
    • Export Citation
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