Wielodyscyplinarne postępowanie w ciąży powikłanej rozległym zespołem Sturge-Webera z nieznaną wcześniej zaangażowaniem naczyniowym kręgosłupa: opis przypadku

Abstract Background: Sturge–Weber syndrome is a rare neurocutaneous vascular disorder, and pregnancy complicated by this condition has been reported only occasionally. Evidence regarding optimal delivery planning and anesthetic management remains limited, particularly in patients with extensive multisystem vascular involvement. Most reported cases have involved relatively localized craniofacial or intracranial disease, whereas the implications of widespread vascular abnormalities with potential spinal involvement remain unclear. We report a rare case of extensive Sturge–Weber syndrome with previously unrecognized spinal vascular involvement, highlighting the importance of comprehensive neurovascular assessment for individualized peripartum management. Case presentation: A 27-year-old nulliparous woman at 40 weeks and 1 day of gestation was referred to a tertiary care center for delivery planning because of extensive congenital vascular malformations and a history of epilepsy. She had widespread capillary and vascular malformations involving the face, oral and nasal mucosa, trunk, and extremities, accompanied by left hemispheric leptomeningeal angiomatosis, congenital glaucoma resulting in unilateral blindness, and well-controlled epilepsy. The pregnancy was uneventful, with no seizure recurrence and normal fetal assessments. Brain imaging revealed leptomeningeal vascular abnormalities, and subsequent whole-spine magnetic resonance imaging unexpectedly identified a vascular-rich lesion extending from the fourth lumbar vertebra to the sacral canal, suggestive of an intraspinal vascular malformation. After multidisciplinary evaluation involving obstetrics, anesthesiology, neurology, neurosurgery, vascular surgery, ophthalmology, neonatology, and intensive care medicine, neuraxial anesthesia was considered inappropriate because of the potential risk of hemorrhage and neurological injury. An elective cesarean delivery under general anesthesia was performed with careful airway preparation and hemodynamic monitoring. A healthy male infant weighing 3000 g was delivered, and both maternal and neonatal outcomes were favorable. The mother remained neurologically stable without postpartum seizure recurrence at the 6-week follow-up. Conclusions: In pregnant women with extensive Sturge–Weber syndrome, the extent and distribution of vascular abnormalities should guide delivery and anesthetic decisions rather than the diagnosis alone. Individualized neurovascular assessment, including selective spinal imaging when clinically indicated, and multidisciplinary collaboration may help identify hidden vascular risks and optimize maternal and neonatal outcomes.