Download PDF
CC BY-NC-ND 4.0 · J Neuroanaesth Crit Care 2019; 06(01): 037-039
DOI: 10.1055/s-0038-1675892
Case Report
Indian Society of Neuroanaesthesiology and Critical Care

Anesthetic Challenges of a Child with Sturge–Weber Syndrome for Epilepsy Surgery: A Case Report

Roshan Kurian
1   Department of Anaesthesia, Christian Medical College, Vellore, Tamil Nadu, India
,
Karen R. Lionel
1   Department of Anaesthesia, Christian Medical College, Vellore, Tamil Nadu, India
,
Ramamani Mariappan
1   Department of Anaesthesia, Christian Medical College, Vellore, Tamil Nadu, India
› Author Affiliations Funding None.
Further Information

Publication History

Received: 11 July 2018

Accepted: 22 August 2018

Publication Date:
25 January 2019 (online)

Also available at
   Permissions and Reprints

Abstract

Sturge–Weber syndrome is a rare congenital disease, also called encephalotrigeminal angiomatosis, caused by persistence of transitory primordial arteriovenous connections of the fetal intracranial vasculature. It is characterized by vascular malformations with capillary venous angiomas that involve the face, choroid of the eye, and leptomeninges. The main clinical features of this syndrome are port-wine stains, glaucoma, convulsions, and angiomas of the airway. Anesthesia management is directed toward anticipating a difficult airway, avoiding trauma to the hemangioma during airway manipulation, preventing the rise in the intracranial and intraocular pressures, anticipating and managing massive blood loss and the complications associated with massive blood transfusion, and avoiding factors that might trigger a seizure such as hypoxia, hypercarbia, hypotension, hypoglycemia, and hyperthermia.

Keywords

Sturge–Weber syndrome - epilepsy surgery - anesthetic implications
 

  • References

  • 1 Roach ES. Neurocutaneous syndromes. Pediatr Clin North Am 1992; 39 (04) 591-620
    CrossrefPubMedSearch in Google Scholar
  • 2 Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: a review. Pediatr Neurol 2004; 30 (05) 303-310
    CrossrefPubMedSearch in Google Scholar
  • 3 Schmitt J. Visceral aspects of the phakomatosis. In: Vinken PJ, Bruyn GW. eds. Handbook of Clinical Neurology, volume 14. Amsterda North Holland Publishing Co.; 1972: 668-730
    Search in Google Scholar
  • 4 Alexander GL. Sturge-Weber syndrome. In: Vinken PJ, Bruyn GW. eds. Handbook of Clinical Neurology, volume 14. Amsterda North Holland Publ. Co.; 1972: 223-240
    Search in Google Scholar
  • 5 Wong HS, Abdul Rahman R, Choo SY, Yahya N. Sturge-Weber-Syndrome with extreme ocular manifestation and rare association of upper airway angioma with anticipated difficult airway. Med J Malaysia 2012; 67 (04) 435-437
    PubMedSearch in Google Scholar
  • 6 Butler MG, Hayes BG, Hathaway MM, Begleiter ML. Specific genetic diseases at risk for sedation/anesthesia complications. Anesth Analg 2000; 91 (04) 837-855
    CrossrefPubMedSearch in Google Scholar
  • 7 Batra RK, Gulaya V, Madan R, Trikha A. Anaesthesia and the Sturge-Weber syndrome. Can J Anaesth 1994; 41 (02) 133-136
    CrossrefPubMedSearch in Google Scholar