Denouement and Discussion
Hemifacial Atrophy (Romberg's Syndrome)
Major manifestations include progressive atrophy of the face accompanied by seizures, trigeminal neuralgia, and increased pigmentation of the overlying skin.The atrophy involves skin, subcutaneous tissue, muscles, and occasionally cartilage and bone. It usually appears by age 10 and, initially, frequently involves the temporal or buccinator muscles; it then extends to the brow, angle of the mouth, neck; and subsequently it may involve other parts of the body. The left side is more frequently affected than the right. The atrophy continues for approximately three years and then becomes nonprogressive. Changes in the hair may precede the skin findings and include alopecia and poliosis. Eye abnormalities, frequently secondary to the atrophy, may occur and consist of enophthalmos, outward slanting of the outer canthus, muscular paralyses, inflammation of the eyes, heterochromia of the iris, and Homer's syndrome. Epilepsy, mainly of the Jacksonian type, is a common neurologic occurrence and usually appears late.
Gellis SS, Feingold M, Tirosh E, Hardoff D, Friedman-Birnbaum R. Picture of the Month. Am J Dis Child. 1980;134(9):883–884. doi:10.1001/archpedi.1980.02130210065017
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