Acta Neurochirurgica

, Volume 160, Issue 2, pp 287–290 | Cite as

Cerebral and spinal cavernomas associated with Klippel-Trenaunay syndrome: case report and literature review

  • Takaya Yoshinaga
  • Kenji Yagi
  • Takashi Morishita
  • Hiroshi Abe
  • Masani Nonaka
  • Tooru Inoue
Case Report - Vascular


Klippel-Trenaunay-Weber syndrome (KTWS) involves a triad of conditions, including cutaneous hemangiomas, venous varicosities, and osseous and soft-tissue hypertrophy of the affected limb. We describe a rare case of multiple cavernomas in the central nervous system in a patient with KTWS. A-64-year-old man with KTWS and a previous brain hemorrhage presented with sudden onset of gait and vesicorectal disturbance. The magnetic resonance imaging scan showed a cavernoma associated with hemorrhage in his lumbosacral spinal cord. Moreover, numerous cavernomas were identified in his brain. He was treated conservatively with no adverse events. Gait disturbance improved, but vesicorectal disturbance did not improve.


Klippel-Trenaunay syndrome Cavernoma Hemorrhage Vascular anomaly 



No funding was received for this research.

Compliance with ethical standards

Conflicts of interest

K. Yagi has received grant support from the Japan Society for Promotion of Science and AOspine.

T. Morishita has received grant support from the Japan Society for Promotion of Science, St. Luke Life Science Institute, Nakatomi Foundation, Takeda Science Foundation, Uehara Memorial Foundation, and Central Research Institute of Fukuoka University. He has received honoraria from Boston Scientific and Medtronic as a consultant within the past 12 months.

T. Inoue has received grant support from the Clinical Research Promotion Foundation Japan and Japan Agency for Medical Research and Development.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from the individual participant included in the study.


  1. 1.
    Badhiwala JH, Farrokhyar F, Alhazzani W, Yarascavitch B, Aref M, Algird A, Murty N, Kachur E, Cenic A, Reddy K, Almenawer SA (2014) Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data: clinic article. J Neurosurg Spine 21:662–676CrossRefPubMedGoogle Scholar
  2. 2.
    Beume LA, Fuhrmann SC, Reinhard M, Harloff A (2013) Coincidence of ischemic stroke and recurrent brain haemorrhage in a patient with Klippel-Trenaunay syndrome. J Clin Neurosci 20:1454–1455CrossRefPubMedGoogle Scholar
  3. 3.
    Boutarbouch M, Ben Salem D, Gire L, Giroud M, Bejot Y, Ricolfi F (2010) Multiple cerebral and spinal cord cavernomas in Klippel-Trenaunay-Weber syndrome. J Clin Neurosci 17:1073–1075CrossRefPubMedGoogle Scholar
  4. 4.
    Pichierri A, Piccirilli M, Passacantilli E, Frati A, Santoro A (2006) Klippel-Trenaunay-Weber syndrome and intramedullary cervical cavernoma: a very rare association. Case report. Surg Neurol 66:203–206CrossRefPubMedGoogle Scholar
  5. 5.
    Ricks CB, Grandhi R, Ducruet AF (2014) Klippel-Trenaunay syndrome and cavernous malformations. BMJ Case Rep.
  6. 6.
    Rohany M, Shaibani A, Arafat O, Walker MT, Russell EJ, Batjer HH, Getch CC (2007) Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome: a literature search and report of two cases. AJNR Am J Neuroradiol 28:584–589PubMedGoogle Scholar
  7. 7.
    Timur AA, Driscoll DJ, Wang Q (2005) Biomedicine and diseases: the Klippel-Trenaunay syndrome, vascular anomalies and vascular morphogenesis. Cell Mol Life Sci 62:1434–1447CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Torregrosa A, Marti-Bonmati L, Higueras V, Poyatos C, Sanchis A (2000) Klippel-Trenaunay syndrome: frequency of cerebral and cerebellar hemihypertrophy on MRI. Neuroradiology 42:420–423CrossRefPubMedGoogle Scholar
  9. 9.
    Vahidnezhad H, Youssefian L, Uitto J (2016) Klippel-Trenaunay syndrome belongs to the PIK3CA-related overgrowth spectrum (PROS). Exp Dermatol 25:17–19CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2017

Authors and Affiliations

  • Takaya Yoshinaga
    • 1
  • Kenji Yagi
    • 1
  • Takashi Morishita
    • 1
  • Hiroshi Abe
    • 1
  • Masani Nonaka
    • 1
  • Tooru Inoue
    • 1
  1. 1.Department of Neurosurgery, Faculty of MedicineFukuoka UniversityFukuokaJapan

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