Answer:
Parry Romberg Syndrome (progressive hemifacial atrophy)
Discussion:
Parry-Romberg syndrome (PRS), also known as progressive hemifacial atrophy, is a self-limited, sporadic neurocutaneous disease of unknown origin with typical onset in childhood or early adulthood.It remains a poorly understood condition. It is characterized by slow and progressive atrophy affecting one side of the face, including the skin, subcutaneous tissue, muscles, cartilage, and underlying bony structures . It characteristically starts in the maxillary region but may involve the chin and forehead . This degenerative disease affects both the aesthetic aspects and the functionality of the affected hemiface. Imaging findings include bone and soft-tissue atrophy of varying degrees. Atrophy is limited by the midline and does not extend to the other side of the face, as is characteristic of the disease.Neuroimaging findings range from unremarkable to degenerative brain lesions, including atrophy, intracranial calcifications, and nonspecific high-signal-intensity transient lesions in gray or white matter areas.Lesions found at MR imaging are usually ipsilateral, but sometimes they are bilateral or even contra lateral. Cerebral hemi atrophy, monoventricular enlargement, meningocortical dysmorphia,Cortical thickening and dysgenesis, unilateral focal infarctions in the corpus callosum, diffuse deep and sub cortical white matter signal changes, leptomeningeal thickening and enhancement, dense mineraldepositions, aneurysms, and vascular malformations have been associated with this syndrome.Enophthalmos is another characteristic feature seen with this syndrome.
Reference:
Radiology: Volume 262: Number 2—February 2012
Contributed By:
Dr. Karunakaran M. Kalathi MD, Dr. Arun Chelladurai MD
Consultant Radiologists, Aarthi Scans, Thirunelveli, Tamilnadu, India
Parry Romberg Syndrome (progressive hemifacial atrophy)
Discussion:
Parry-Romberg syndrome (PRS), also known as progressive hemifacial atrophy, is a self-limited, sporadic neurocutaneous disease of unknown origin with typical onset in childhood or early adulthood.It remains a poorly understood condition. It is characterized by slow and progressive atrophy affecting one side of the face, including the skin, subcutaneous tissue, muscles, cartilage, and underlying bony structures . It characteristically starts in the maxillary region but may involve the chin and forehead . This degenerative disease affects both the aesthetic aspects and the functionality of the affected hemiface. Imaging findings include bone and soft-tissue atrophy of varying degrees. Atrophy is limited by the midline and does not extend to the other side of the face, as is characteristic of the disease.Neuroimaging findings range from unremarkable to degenerative brain lesions, including atrophy, intracranial calcifications, and nonspecific high-signal-intensity transient lesions in gray or white matter areas.Lesions found at MR imaging are usually ipsilateral, but sometimes they are bilateral or even contra lateral. Cerebral hemi atrophy, monoventricular enlargement, meningocortical dysmorphia,Cortical thickening and dysgenesis, unilateral focal infarctions in the corpus callosum, diffuse deep and sub cortical white matter signal changes, leptomeningeal thickening and enhancement, dense mineraldepositions, aneurysms, and vascular malformations have been associated with this syndrome.Enophthalmos is another characteristic feature seen with this syndrome.
Reference:
Radiology: Volume 262: Number 2—February 2012
Contributed By:
Dr. Karunakaran M. Kalathi MD, Dr. Arun Chelladurai MD
Consultant Radiologists, Aarthi Scans, Thirunelveli, Tamilnadu, India