Parry Romberg Syndrome: 7 Cases and Literature Review

A 14-year-old boy who presented with progressive atrophy of the left face. A, T1-weighted image shows striking paucity of fat in the left face. B, 3D reconstruction of a CT series demonstrates left facial hemiatrophy, particularly involving the mandible. C, T1-weighted image at the level of the orbits reveals left enophthalmos.

A 24-year-old man with progressive atrophy of the left face. A and B, Axial T1-weighted MR images demonstrate left hemifacial atrophy primarily involving the skin and subcutaneous fat; only subtle asymmetry of the masseter is noted. C, T2-weighted image at the same level demonstrates normal T2 signal in the affected structures.

An 11-year-old girl with left-face sensitivity and left-temple pain. A, 3D reconstructions of a CT series show osseous asymmetry, most prominently in the left maxilla. B, Axial CT image at soft-tissue windows demonstrates deviation of the nose toward the affected side. C, Axial bone window demonstrates marked asymmetry in the volume of ethmoidal air cells.

A 30-year-old woman with a history of PRS who presented for MR imaging evaluation for weakness and recurrent headaches. A, Coronal enhanced T1-weighted MR image demonstrates mild asymmetry of scalp thickness with relative paucity of subcutaneous fat on the right. B and C, Axial FLAIR images demonstrate abnormal hyperintensity of the white matter in the right corona radiata, internal capsule, and temporal region. D, Axial susceptibility-weighted image demonstrates innumerable punctate foci of susceptibility throughout the right cerebral hemisphere.

A 35-year-old woman with a history of PRS presented for MR imaging evaluation for headaches and weakness. Axial FLAIR images demonstrate hyperinstensity in the left cerebral periventricular to subcortical white matter. The pattern of signal abnormality is geographic, with subtotal involvement of frontal, parietal, and temporal lobes.