Occurrence of spinal extradural arachnoid cysts in a child with concomitant intracranial midline abnormalities: case report

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Spinal extradural arachnoid cysts (SEACs) are uncommon spinal lesions that may cause myelopathy, most frequently in the 2nd decade of life. There are multiple theories of their pathogenesis, and associated entities include spinal trauma, spina bifida, and the lymphedema-distichiasis syndrome. The authors report the case of an otherwise healthy, developmentally normal 13-year-old boy who presented with multiple SEACs. Upon further neuroimaging workup, he was found to have an asymptomatic retrocerebellar arachnoid cyst, cavum septi pellucidi, and cavum vergae. Three contiguous but separate spinal cysts were identified intraoperatively, and they were completely excised with closure of the dural defects. The patient recovered motor and sensory function of the lower extremities. This collection of uncommon neuroimaging findings provides important clues to the pathogenesis of the disease and guides the optimal management of patients with SEACs. The unusual presentation of SEACs, together with uncommon midline abnormalities, provides further evidence of their congenital, midline origin. Therefore, it is reasonable to pursue imaging of the brain in atypical cases of SEACs.

ABBREVIATIONS SEAC = spinal extradural arachnoid cyst.
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Contributor Notes

Correspondence Alan R. Cohen: The Johns Hopkins University School of Medicine, Baltimore, MD. alan.cohen@jhmi.edu.INCLUDE WHEN CITING Published online March 6, 2020; DOI: 10.3171/2019.12.PEDS19108.Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
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  • 1

    Basaldella LOrvieto EDei Tos APDella Barbera MValente MLongatti P: Causes of arachnoid cyst development and expansion. Neurosurg Focus 22(2):E42007

    • Search Google Scholar
    • Export Citation
  • 2

    Beatriz NLopes S: Meninges: embryology in Lee JH (ed): Meningiomas: Diagnosis Treatment and Outcome. London: Springer-Verlag2009 pp 2529

    • Search Google Scholar
    • Export Citation
  • 3

    Bergland RM: Congenital intraspinal extradural cyst. Report of three cases in one family. J Neurosurg 28:4954991968

  • 4

    Bodensteiner JBSchaefer GB: Wide cavum septum pellucidum: a marker of disturbed brain development. Pediatr Neurol 6:3913941990

  • 5

    Brice GMansour SBell RCollin JROChild AHBrady AF: Analysis of the phenotypic abnormalities in lymphoedema-distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to 16q24. J Med Genet 39:4784832002

    • Search Google Scholar
    • Export Citation
  • 6

    Chen JACoutin-Churchman PENuwer MRLazareff JA: Suboccipital craniotomy for Chiari I results in evoked potential conduction changes. Surg Neurol Int 3:1652012

    • Search Google Scholar
    • Export Citation
  • 7

    Chen JJChen CJChang HFChen DLHsu YCChang TP: Prevalence of cavum septum pellucidum and/or cavum Vergae in brain computed tomographies of Taiwanese. Acta Neurol Taiwan 23:49–542014

    • Search Google Scholar
    • Export Citation
  • 8

    Cloward RBBucy PC: Spinal extradural cyst and kyphosis dorsalis juvenilis. 1937. Surg Neurol 39:4694731993

  • 9

    de Oliveira RSAmato MCMSantos MVSimão GNMachado HR: Extradural arachnoid cysts in children. Childs Nerv Syst 23:123312382007

    • Search Google Scholar
    • Export Citation
  • 10

    Fang JDagenais SLErickson RPArlt MFGlynn MWGorski JL: Mutations in FOXC2 (MFH-1), a forkhead family transcription factor, are responsible for the hereditary lymphedema-distichiasis syndrome. Am J Hum Genet 67:138213882000

    • Search Google Scholar
    • Export Citation
  • 11

    Fortuna ALa Torre ECiappetta P: Arachnoid diverticula: a unitary approach to spinal cysts communicating with the subarachnoid space. Acta Neurochir (Wien) 39:259–2681977

    • Search Google Scholar
    • Export Citation
  • 12

    Funao HNakamura MHosogane NWatanabe KTsuji TIshii K: Surgical treatment of spinal extradural arachnoid cysts in the thoracolumbar spine. Neurosurgery 71:2782842012

    • Search Google Scholar
    • Export Citation
  • 13

    Gortvai P: Extradural cysts of the spinal canal. J Neurol Neurosurg Psychiatry 26:2232301963

  • 14

    Hatashita SKondo AShimizu TKurosu AUeno H: Spinal extradural arachnoid cyst—case report. Neurol Med Chir (Tokyo) 41:3183212001

    • Search Google Scholar
    • Export Citation
  • 15

    Hyndman ORGerber WF: Spinal extradural cysts, congenital and acquired; report of cases. J Neurosurg 3:4744861946

  • 16

    Jensen FKnudsen VTroelsen S: Recurrent intraspinal arachnoid cyst treated with a shunt procedure. Acta Neurochir (Wien) 39:127–1291977

    • Search Google Scholar
    • Export Citation
  • 17

    Lake PAMinckler JScanlan RL: Spinal epidural cyst: theories of pathogenesis. Case report. J Neurosurg 40:7747781974

  • 18

    Lee CHHyun SJKim KJJahng TAKim HJ: What is a reasonable surgical procedure for spinal extradural arachnoid cysts: is cyst removal mandatory? Eight consecutive cases and a review of the literature. Acta Neurochir (Wien) 154:121912272012

    • Search Google Scholar
    • Export Citation
  • 19

    Lin LCJason R: A rare case of spinal extradural arachnoid cyst with cord compression. Asian J Neurosurg 13:4684702018

  • 20

    Liu JKCole CDSherr GTKestle JRWWalker ML: Noncommunicating spinal extradural arachnoid cyst causing spinal cord compression in a child. J Neurosurg 103 (3 Suppl):2662692005

    • Search Google Scholar
    • Export Citation
  • 21

    Marbacher SBarth AArnold MSeiler RW: Multiple spinal extradural meningeal cysts presenting as acute paraplegia. Case report and review of the literature. J Neurosurg Spine 6:4654722007

    • Search Google Scholar
    • Export Citation
  • 22

    McCrum CWilliams B: Spinal extradural arachnoid pouches. Report of two cases. J Neurosurg 57:8498521982

  • 23

    Myles LMGupta NArmstrong DRutka JT: Multiple extradural arachnoid cysts as a cause of spinal cord compression in a child. Case report. J Neurosurg 91 (1 Suppl):1161201999

    • Search Google Scholar
    • Export Citation
  • 24

    Nabors MWPait TGByrd EBKarim NODavis DOKobrine AI: Updated assessment and current classification of spinal meningeal cysts. J Neurosurg 68:3663771988

    • Search Google Scholar
    • Export Citation
  • 25

    Nakano SHojo HKataoka KYamasaki S: Age related incidence of cavum septi pellucidi and cavum Vergae on CT scans of pediatric patients. J Comput Assist Tomogr 5:3483491981

    • Search Google Scholar
    • Export Citation
  • 26

    Neo MKoyama TSakamoto TFujibayashi SNakamura T: Detection of a dural defect by cinematic magnetic resonance imaging and its selective closure as a treatment for a spinal extradural arachnoid cyst. Spine (Phila Pa 1976) 29:E426E4302004

    • Search Google Scholar
    • Export Citation
  • 27

    O’Connell JEA: The cerebrospinal fluid pressure as an aetiological factor in the development of lesions affecting the central nervous system. Brain 76:2792981953

    • Search Google Scholar
    • Export Citation
  • 28

    Ogura YMiyake NKou IIida ANakajima MTakeda K: Identification of HOXD4 mutations in spinal extradural arachnoid cyst. PLoS One 10:e01421262015

    • Search Google Scholar
    • Export Citation
  • 29

    Park HYLee SHKim ESEoh W: Spinal extradural meningeal cyst and Scheuermann’s disease: coincidence or causative factor? Childs Nerv Syst 28:180718102012

    • Search Google Scholar
    • Export Citation
  • 30

    Rabb CHMcComb JGRaffel CKennedy JG: Spinal arachnoid cysts in the pediatric age group: an association with neural tube defects. J Neurosurg 77:3693721992

    • Search Google Scholar
    • Export Citation
  • 31

    Rexed BAWennström KG: Arachnoidal proliferation and cystic formation in the spinal nerve-root pouches of man. J Neurosurg 16:73841959

    • Search Google Scholar
    • Export Citation
  • 32

    Roski RARekate HLKurczynski TWKaufman B: Extradural meningeal cyst. Case report and review of the literature. Childs Brain 11:2702791984

    • Search Google Scholar
    • Export Citation
  • 33

    Sánchez-Carpintero RDominguez PNúñez MTPatiño-García A: Spinal extradural arachnoid cysts in lymphedema-distichiasis syndrome. Genet Med 12:5325352010

    • Search Google Scholar
    • Export Citation
  • 34

    Sarwar M: The septum pellucidum: normal and abnormal. AJNR Am J Neuroradiol 10:98910051989

  • 35

    Schwidde JT: Incidence of cavum septi pellucidi and cavum Vergae in 1,032 human brains. AMA Arch Neurol Psychiatry 67:6256321952

  • 36

    Sencer ASencer STurantan IDevecioğlu O: Cerebrospinal fluid dynamics of the cava septi pellucidi and vergae. Case report. J Neurosurg 94:1271292001

    • Search Google Scholar
    • Export Citation
  • 37

    Shaw CMAlvord EC Jr: Cava septi pellucidi et vergae: their normal and pathogical states. Brain 92:2132231969

  • 38

    Tseng SHLin SM: Surgical treatment of thoracic arachnoiditis with multiple subarachnoid cysts caused by epidural anesthesia. Clin Neurol Neurosurg 99:2562581997

    • Search Google Scholar
    • Export Citation
  • 39

    Vernooij MWIkram MATanghe HLVincent AJPEHofman AKrestin GP: Incidental findings on brain MRI in the general population. N Engl J Med 357:182118282007

    • Search Google Scholar
    • Export Citation
  • 40

    Wilm BJames RGSchultheiss TMHogan BLM: The forkhead genes, Foxc1 and Foxc2, regulate paraxial versus intermediate mesoderm cell fate. Dev Biol 271:1761892004

    • Search Google Scholar
    • Export Citation
  • 41

    Yasuoka SPeterson HAMacCarty CS: Incidence of spinal column deformity after multilevel laminectomy in children and adults. J Neurosurg 57:4414451982

    • Search Google Scholar
    • Export Citation
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