Romberg syndrome (parry-romberg syndrome; progressive hemifacial atrophy; progressive facial hemiatrophy; facial hemiatrophy) 1

General: Autosomal dominant; irritation in the peripheral trophic sympathetic system; onset in the second decade; both sexes affected.

Ocular: Enophthalmos; outer canthus lowered; absence of nasal portion of eyebrow; ptosis; paresis of ocular muscles; miosis; iritis; iridocyclitis; heterochromia iridis; keratitis; neuroparalytic keratitis; cataracts; choroiditis; Fuchs heterochromic cyclitis; retinal vascular abnormalities; association with Coats syndrome and exudative stellate neuroretinopathy; scleral melting.

Clinical: Atrophy of soft tissue on one side of the face, including tongue; trigeminal neuralgia and/or paresthesia; alopecia and poliosis not uncommon.

Aracena T, et al. Progressive hemifacial atrophy (Parry-Romberg syndrome): report of two cases. Ann Ophthalmol 1979; 11:953-958.

Gass JD, et al. Exudative stellate neuroretinopathy and Coats' syndrome in patients with progressive hemifacial atrophy. Eur J Ophthalmol 1991; 1:2-l0.

Hoang-Xuan T, et al. Romberg's progressive hemifacial atrophy: an association with scleral melting. Cornea 1991; 10:361-366.

La Hey E, Baarsma GS. Fuchs' heterochromic cyclitis and retinal vascular abnormalities in progressive hemifacial atrophy. Eye 1993; 7[Part 3]:426-428.

Parry CH. Collections from unpublished papers, vol. I. London: Underwood, 1825.

Romberg MH. Trophoneurosen, Klin. Ergebn. Berlin: A. Forster, 1846.