CRANIOFACIAL PHACOMATOSIS IN EARLY CHILDHOOD: IMAGING SPECTRUM OF PARRY–ROMBERG SYNDROME IN A 3-YEAROLD CHILD.

Parry-Romberg Syndrome (PRS), also known as progressive hemifacial atrophy, is an uncommon neurocutaneous disorder characteristically involving the skin and subcutaneous connective tissues and may later progress to affect the underlying musculature, cartilage, and osseous structures resulting into unilateral atrophy of the same with or without development of neurologic symptoms.PRS typically presents initially in children and young adults and slowly progresses over a highly variable course ranging from 2 to 20 years and radiologic evaluation plays a vital role in diagnosis and assessment of disease extent.We report a case of a 3-year-old female presenting with right-sided facial hemiatrophy, cutaneous tightening, alopecia, eyebrow hair loss and fronto-temporal linear morphea.CT and MRI demonstrated characteristic craniofacial involvement with soft-tissue atrophy, osseous thinning, and intracranial cortical calcifications along with white matter edema.Imaging features strongly favoured the diagnosis of craniofacial phakomatosis consistent with Parry-Romberg Syndrome.