Abstract
Sturge–Weber syndrome consists of facial capillary malformation (port-wine stain)
and abnormal blood vessels in the brain or eye. Seizures, developmental delay and
intracranial and airway angiomata are principal concerns. We report a 28-year-old
primiparous woman at 41 weeks of gestation with Sturge–Weber syndrome who developed unilateral weakness, aphasia,
blurred vision and confusion. Preeclampsia was excluded. Neuroimaging showed left
sided cerebral oedema and a right parieto–occipital lesion, most likely an angioma.
Caesarean section was planned to avoid the risk of angioma rupture during labour.
General anesthesia was avoided due to the haemodynamic response to laryngoscopy and
reports of seizure-related mortality. Despite the possibility of raised intracranial
pressure and precipitation of cerebral herniation, a lumbar epidural block was administered
but failed. A subarachnoid block was successfully performed and a healthy infant delivered.
The choice of anaesthesia was strongly influenced by detailed radiological investigations
and multidisciplinary participation.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to International Journal of Obstetric AnesthesiaAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Advances in Sturge–Weber syndrome.Curr Opin Neurol. 2006; 19: 124-128
- Sturge–Weber syndrome: a review.Pediatr Neurol. 2004; 30: 303-310
- Sturge–Weber disease with subarachnoid hemorrhage.Stroke. 1974; 5: 509-511
- Sturge–Weber syndrome in pregnancy.Am J Obstet Gynecol. 1995; 173: 969-971
- An uncomplicated pregnancy associated with Sturge–Weber angiomatosis.Eur J Obstet Gynecol Reprod Biol. 2008; 137: 125-126
- Anaesthesia and the Sturge–Weber syndrome.Can J Anaesth. 1994; 41: 133-136
- Anesthesia for patients with Sturge–Weber disease and Klippel–Trenaunay syndrome.J Clin Anesth. 1991; 3: 409-413
- Anesthetic management of a patient with Sturge–Weber syndrome undergoing oral surgery.Anesth Prog. 2006; 53: 17-19
- Sturge–Weber syndrome associated with other abnormalities: a medical record and literature review.Arch Neurol. 2005; 62: 1924-1927
- Perioperative seizures in patients with a history of a seizure disorder.Anesth Analg. 2010; 111: 729-735
- Update on Sturge–Weber syndrome: diagnosis, treatment, quantitative measures, and controversies.Lymphat Res Biol. 2007; 5: 257-264
- Status epilepticus and venous infarction in Sturge–Weber syndrome.Childs Nerv Syst. 1998; 14: 693-696
- Lumbar puncture in the presence of raised intracranial pressure.BMJ. 1969; 1: 407-409
- Fatal brain lesion following spinal anaesthesia. Report of a case.Acta Anaesthesiol Scand. 1981; 25: 115-116
- Herniation secondary to critical postcraniotomy cerebrospinal fluid hypovolemia.Neurosurgery. 2005; 57: 286-292
- Acute symptomatic cerebellar tonsillar herniation following intraoperative lumbar drainage.J Neurosurg. 2009; 110: 800-803
- Cranial computed tomography before lumbar puncture: a prospective clinical evaluation.Arch Intern Med. 1999; 159: 2681-2685
- Regional blockade in patients with a history of a seizure disorder.Anesth Analg. 2009; 109: 272-278
Article info
Publication history
Published online: February 11, 2011
Accepted:
November 27,
2010
Identification
Copyright
© 2010 Elsevier Ltd. Published by Elsevier Inc. All rights reserved.