Parry-Romberg syndrome

Parry-Romberg syndrome is a progressive hemifacial atrophy involving the skin, soft tissues, cartilage, and underlying bone.1 It was described first by Parry in 1825,2 then Romberg in 1846,3 and Eulenberg in 1871.4 It is more frequent in women, with a female-to-male ratio of 1.5:1.5 In Rogers’ study of 772 cases, only 5 percent showed bilateral atrophy.5 In most cases, the atrophy is unilateral.6 Parry-Romberg syndrome can appear in early infancy or adolescence. The most serious cases are manifest earlier, with atrophy of the underlying structures.6 Nevertheless, in a recent study, Jeremy et al.1 reported that there was no relationship between the seriousness of the disease and age of onset. Causes of the disease remain unknown, although various hypotheses have been advanced. The most recent and reliable theory is that there is a genetic alteration in the first stage of the embryogenesis of the central nervous system.7 Other hypotheses include viral or bacterial infections,8 loss of the cervical sympathetic nerve9,10 after peripheral neuritis of the trigeminal nerve,11,12 and trauma,13 and still others suggest that the disease is a form of scleroderma.4,5,8 . Progressive hemifacial atrophy is often confused with scleroderma, which led Singh and Bajpai14 to establish criteria to distinguish these two diseases.6 Facial atrophy is localized and progresses according to the specific distribution of one or more branches of the fifth cranial nerve15: in 50 percent of cases, the upper two-thirds of the involved hemiface show the classic “coup de sabre.” The whole face can become involved as the disease progresses. In its late appearance, the lower third of face is commonly affected, with rare involvement of the upper third.16 In some cases, half of the tongue and the corresponding salivary glands are involved.6 The most common eye symptoms are progressive enophthalmos, restrictive and paralytic ocular muscle pathologies, and Duane syndrome.17,18 We present a case of serious progressive hemifacial atrophy treated with grafting of biomaterials, specifically porous polyethylene implant for reconstruction of the orbital bone tissue and a gel polymer comprising networks of alkylimide groups (4 percent) and water (96 percent) for filling the soft tissue.