International Clinical and Medical Case Reports Journal (ISSN: 2832-5788) | Volume 3, Issue 6 | Case Report | Open Access DOI

Case Report of Sturge Weber Syndrome With Bilateral Port Wine Stain And Bilateral Cerebral Calcification

Govind Pandey M.D*

Tharuna Chandra M.D, Govind Pandey M.D*, Gaurav Singh M.D M.D Paediatrics, Dr. Ram Manohar Lohia Institute of Medical Sciences, India

*Correspondence to: Govind Pandey M.D 

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Abstract

Sturge-Weber Syndrome (SWS) is a congenital disorder affecting the brain, eyes, and skin. It is characterized by a facial cutaneous vascular nevus, angioma, and bupthalmos. The frequency of SWS is between 1:20,000 and 1:50,000. There are very few reported cases of SWS with bilateral intracranial calcification and bilateral port wine stain. We present a case report of an 8-year-old boy present with complaint of generalized tonic-clonic (GTCS) type of convulsion with purple discoloration of the skin on the both side of the face since birth and was diagnosed with SWS with bilateral intracranial calcification, intellectual disability, seizure and bilateral port wine stain. MRI with contrast revealed gyriform hyperdense calcification is seen in bilateral fronto-parietal lobes and T2 suggestive of calcification seen in bilateral fronto- parietal lobes with prominence of supratentorial sulcal- cisternal spaces seen on right side. The patient was counseled about the syndrome and discharged on anti-convulsant treatment with advice for dye laser photocoagulation for port-wine stain.

Keywords:

Neurocutaneous syndrome; Port-wine stain; Sturge weber syndrome (SWS); Nevus flammeus; Tram track appearance

Citation:

Tharuna Chandra, Govind Pandey, Gaurav Singh. Case Report of Sturge Weber Syndrome With Bilateral Port Wine Stain And Bilateral Cerebral Calcification. Int Clinc Med Case Rep Jour. 2024;3(6):1-5.