Sturge-webber syndrome: An uncommon entity

A nine month old male child† (non-consanguinous parentage, normal delivery) was referred to the department of Dermatology, College of Medicine and Sagore Dutta Hospital, Kolkata, India, in July 2019, with reddish, raised lesions on his face. Multiple discreet, coalescent elevated reddish lesions restricted to his left face were observed, which swelled on crying. There were two previous episodes of seizure, relieved by medication. Family, drug and maternal antenatal history were unremarkable.

A general survey revealed tachycardia, tachypnea and raised lesional temperature. The dermatological examination demonstrated coalescent erythematous maculopapular lesions, soft and compressible on palpation suggestive of port wine stain. Only the left forehead and cheek were affected, including ipsilateral eyelids and upper lip, extending up to the scalp (Figure A). Hair, nails and mucosae were spared. Neurologic, ophthalmologic and other systemic examinations were normal. Routine biochemistry was also unremarkable. A clinical diagnosis of Sturge-Weber syndrome was made. Skull radiograph revealed characteristic gyrate/'tram-track' appearance over the cortex (Figure B). Computed tomography-scan of the skull was suggested but the parents refused. Topical emollients were prescribed and parents were counselled and advised regular follow up. At three months follow up, no significant clinical improvement was noted.

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Figure

(A) Port-wine stain affecting the left side of the face, extending up to the scalp. (B) X-ray of skull showing tram-track appearance (arrow).

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In the absence of specific treatment, symptomatic management is the key. For the cutaneous port-wine stain/nevus flammeus, pulsed-dye laser may be tried after 18 years of age.