Anesthesia and Airway Management of a Patient with Sturge- Weber Syndrome Associated with Extreme Ocular Manifestations and Giant Facial Hemangioma

  • Mahtab Poor Zamany Nejat Kermany Department of Anesthesiology and Critical Care, Labbafinejad Medical Center, Shahid Beheshti Medical Center, Tehran,Iran.
  • Badiozaman Radpay Department of Anesthesiology and Critical Care, Shohada Tajrish Medical Center, Shahid Beheshti Medical Center, Tehran, Iran
Keywords: sturge–weber syndrome, airway management, hemangioma


Sturge-Weber syndrome (SWS) is a very rare congenital disorder that is manifested by facial capillary malformation (port wine stain) which may be associated with capillary – venous malformations affecting many parts such as the brain, eyes facial skin and mucosa and also airways. In this report we present an unusual 28 years old female case of SWS that presented with signs and symptoms of sudden increased intra ocular pressure (exacerbation of glaucoma) and manifestations of facial and airway involvement. A discussion about anesthetic and airway management of such patient follow the case presentation.


Khan ZH. Airway management, pp. 15-28, 72-78, 177-188, and 223-238; Springer International Publishing Switzerland, 2014

Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of sturge- weber syndrome; J Pediatr Ophthalmol Strabismus. 1992; 29(6):349-56.

Wong HS, Abdul Rahman R, Choo SY, Yahya N. sturge- weber syndrome with extreme ocular manifestations and rare association of upper airway angioma with anticipated difficult airway. Med J Malaysia. 2012; 67(4):435-7.

Fujii N, Usuda I, Hikawa Y. Management of a patient with sturge- weber syndrome associated with a giant facial hemangioma. Masui. 2014; 63(6):689-91.

White A, Kander PL. Anatomical factors in difficult laryngoscopy. Br J Anaesth. 1975; 47(4):468-74.

Khan ZH, Khorasani AM, Yekaninejad MS. Mallampati airway assessment test in upright ans supine positions with and without noisy exhalation in the prediction of difficult mask ventilation. Intern Med. 2013; 3:122.

Khan ZH, Gharabaghian M, Nilli F, Ghiamat M, Mohammadi M. Easy endotracheal intubation of a patient suffering from both cushings and Nelson’s syndroms predicted by the upper lip bite test despite a mallampati class 4 airway. Anesth Analg. 2007; 105(3):786-7.

Mallampati SR, Gatt SP, Gugino LD, Desai SP, Waraksa B, Freiberger D, Liu PL. A clinical sign to predict difficult tracheal intubation. a prospective study. Can Anaesth Soc J. 1985; 32(4):429-34.

Mentzelopoulos SD, Rellos KV, Magoufis GL, Gini CS, Tobris S, Michalopoulos AS. Combined Mc Coy and balloon laryngoscopy for emergency airway management of a patient with acute post operative airway obstruction due to extreme engorgement of the tongue . Anesth Analg. 2003; 96(5):1531

Arya VK, Dutta A, Chari P. Reinforcement of laryngeal mask airway cuff position with endotracheal tube cuff for airway control in a patient with altered upper airway anatomy. Anesth Analg. 2000; 91(5):1303-5.

Toy FJ, Weinstein JD. A percutaneous tracheostomy device. Surgery. 1969; 65(2):384-9.

Ciaglia P, Firsching R, Syniec C. Elective percutaneous dilatational tracheostomy. A new simple bedside procedure; preliminary report. Chest. 1985; 87(6):715-9.

Schachner A, Ovil Y, Sidi J, Rogev M, Heilbronn Y, Levy MJ. Percutaneous tracheostomy--a new method. Crit Care Med. 1989; 17(10):1052-6.

Little CM, Parker MG, Tarnopolsky R. The incidence of vasculature at risk during cricothyroidostomy. Ann Emerg Med.1986; 15(7):805-7.

How to Cite
Poor Zamany Nejat Kermany M, Radpay B. Anesthesia and Airway Management of a Patient with Sturge- Weber Syndrome Associated with Extreme Ocular Manifestations and Giant Facial Hemangioma. AACC. 2(3):231-3.
Case Report(s)