Deepak MİSHRA, Prashant BHUSHAN, Mahendra K SİNGH, Om P MİSHRA

Sturge-Weber syndrome with involvement of chest dermatomes and unilateral buphthalmos: A rare association

Abstract. Sturge-Weber syndrome is a rare neuro-cutaneousdisease presenting as port- wine stain over distribution of trigeminal nerve.There is a wide variability in manifestations of the disease. Associatedmalformations in the form of buphthalmos is a rare occurrence with thisdisorder. We report a 2 months old infant who had bilateral port- wine stainover face and extending over the upper part of chest. There were no seizuresand developmental milestones were normal. The patient had associatedbuphthalmos in the right eye. The intraocular pressure in the right eye wasraised while it was normal in left eye. Cranial CT scan did not reveal anycalcifications. In conclusion, Sturge- Weber syndrome may not presentclinically in its typical form. The presence of buphthalmos is to be lookedespecially when there is extensive bilateral port-wine stains.Key words: Bilateral port-winestains, buphthalmos

Keywords:

Bilateral port-wine stains, buphthalmos,

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Joanna GDC, Luiz Flipe GDS, Thais PDSB, Vanessa DASS, Maria EBR, Monica I. Sturge weber syndrome : A case report. RSBO 2011; 8:469-472. Osborn AG. Disorders of Histogenesis: Neurocutaneous syndromes. In: Osborn AG (editor). Diagnostic Neuroradiology. Mosby Inc. Co 1994; 74Arya SK, Kochhar S, Dhir SP, Sharma RL, Munjal VP. Variant of Sturge-Weber Syndrome. Ann Ophthalmol 2001;33: 337-339.
Comi AM. Topical Review: Pathophysiology of Sturge -Weber syndrome. J Child Neurol 2003; 18: 509-5
Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge Weber Syndrome: A review. Paed Neurol 2004; 30:303-310.
Tallman B, Tan OT, Morelli JG, Piepenbrink J,Stafford TJ, Trainor S,Weston WL. Location of port wine stains and the likelihood of ophthalmic and central nervous system complications. Pediatrics 1991; 87:323-327.
Sujansky E, Conradi S. Sturge Weber Syndrome: age of onset of seizures and glaucoma and the prognosis of affected children. J Child Neurol 1995; 10: 49-58.
Aylett SE, Nevile BG, Cross JH. Sturge Weber Syndrome: cerebral hemodynamics during seizure activity. Dev Med Child Neurol 1999; 41: 480-485.