Parry-Romberg syndrome: rare disorder of progressive facial hemiatrophy. Slowly progressive atrophy of the soft tissues of one side of the face, associated with trigeminal neuralgia and involvement of the eyes and hair. Muscles, cartilage and the underlying bony structures may also be involved.
The prevalence is estimated to be at least 1/700,000 individuals, women being slightly more affected than men.
PHA usually presents during the first 20 years of life, characterized by slowly progressive unilateral atrophy of various tissues (skin, connective tissue, fat, muscle and rarely, underlying bony structures) in the territory of the 5th cranial nerve.
Seizures and headaches are the most common neurologic symptoms. Ophthalmologic anomalies (enophthalmos, globe retraction, ptosis, iris heterochromia, uveitis, retinal vasculitis, glaucoma and eyelid atrophy) may result in visual impairment or blindness
1825 – Caleb Hillier Parry included an account of the condition now known as progressive hemifacial atrophy. This description appeared in Collections from Unpublished Papers in 1825, 3 years after his death, published by his eldest son, the physician, Charles Henry Parry.
Miss F., aged twenty-eight, tall, thin, and with a somewhat florid complexion, thirteen or fourteen years ago, when at school, was rather suddenly seized with some degree of hemiplegia of the left side, accompanied with transient confusion of intellects. The exact state of the original symptoms it is now impossible to ascertain. It is certain, however, that she has at various times been affected with headaches, and that from the period of the attack the left side of the face began to grow more thin than the right, and the eye to become less prominent, and therefore to appearance smaller.,,when she protrudes her tongue, it turns to the left ; but there is no dropping of the eyelids, or corner of the mouth, nor any weakness or numbness of the limbs on the affected side.Parry 1825: 478-480
1871 – Albert Eulenburg (1840–1917), a German neurologist, was the first to use the descriptive term ‘Hemiatrophia facialis progressiva‘ [Progressive Hemifacial Atrophy (PHA)]
1983 – Lewkonia and Lowry definitive review.
- Progressive hemifacial atrophy (PHA)
- Hemiatrophia facialis progressiva
- Romberg syndrome
- Parry CH. Hemiplegia Miss F. In: Collections from the unpublished medical writings of the late Caleb Hillier Parry. London. 1825; 1: 478-480.
- Romberg MH. Trophoneurosen. In: Klinische Ergebnisse. 1846: 75-81
- Eulenburg A. Lehrbuch der functionellen Nervenkrankheiten auf physiologischer basis. Berlin: Hirschwald. 1871: 712
- Wartenberg R. Progressive facial hemiatrophy. Arch NeurPsych. 1945;54(2):75-96
- Rogers BO. Progressive facial hemiatrophy: Romberg’s disease: a review of 772 cases. Proceedings of the 3rd International Conference on Plastic Surgery. Excerpta Med Int Cong Series 1963; 66: 681-686
- Lewkonia RM, Lowry RB. Progressive hemifacial atrophy (Parry-Romberg syndrome) report with review of genetics and nosology. Am J Med Genet. 1983 Feb;14(2):385-90.
- Tolkachjov SN, Patel NG, Tollefson MM. Progressive hemifacial atrophy: a review. Orphanet Journal of Rare Diseases 2015
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