The Art of Doing Nothing: Conservative Management of a Clinicoradiologically Diagnosed Type III Sturge–Weber Syndrome with Extensive Leptomeningeal Angiomatosis – A Case Report and Review of Literature
Vemula Venkata Ramesh Chandra, Papasani Anil Kumar Reddy, Mummaka Siva Prasad, Bodapati Chandramouliswara Prasad, Kanduri Prithvi, Chavali Lakshmi Narayana
A
BSTRACT
Background:
Sturge–Weber syndrome (SWS) is a rare sporadic neurocutaneous disorder, characterised by vascular malformations involving the skin, eyes and central nervous system. Type III SWS, the rarest subtype, presents with isolated leptomeningeal angiomatosis without facial port-wine stain, often leading to delayed diagnosis.
Case Report:
A 24-year-old right-handed male presented with recurrent focal seizures progressing to generalised tonic–clonic seizures for 4 years. Neurological examination revealed mild left-sided hemiparesis without cognitive impairment. Dermatological and ophthalmological evaluation showed no cutaneous or ocular involvement. Magnetic resonance imaging demonstrated extensive left hemispheric leptomeningeal enhancement with cortical atrophy. Computed tomography angiography revealed diffuse pial vascular proliferation with venous congestion. Digital subtraction angiography confirmed extensive pial arterial feeders with delayed venous drainage, consistent with chronic venous hypertension. Despite extensive radiological involvement, the patient remained functionally stable. He was managed conservatively with levetiracetam, achieving good seizure control.
Intervention and Outcome:
Considering the absence of pharmacoresistance, progressive neurological decline or cognitive deterioration, surgical intervention was deferred. The patient continues to remain neurologically stable on medical therapy during follow-up.
Conclusion:
This case highlights that extensive radiological disease in Type III SWS does not necessarily mandate aggressive intervention. Individualised, conservative management may be appropriate in selected patients. The ‘art of doing nothing’ emphasises the importance of clinical judgement over imaging severity in guiding management decisions.