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.
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- 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
Published online: February 11, 2011
Accepted: November 27, 2010
© 2010 Elsevier Ltd. Published by Elsevier Inc. All rights reserved.