April 2020 HSC Section 4 - Plastic and Reconstructive Problems

A 26-Year Experience with Microsurgical Reconstruction of Hemifacial Atrophy and Linear Scleroderma PEDIATRIC/CRANIOFACIAL Reprinted by permission of Plast Reconstr Surg. 2018; 142(5):1275-1283.

Jenny T. Chen, M.D. Daniel B. Schmid, M.D. Jacqueline S. Israel, M.D. John W. Siebert, M.D. Madison, Wis.; and Livingston, N.J.

Background: Parry Romberg disease (hemifacial atrophy) and linear sclero- derma (coup de sabre) are progressive, usually unilateral facial atrophies of unknown cause. The gold standard treatment for these patients has been mi- crosurgical reconstruction following the “burning out” of the facial atrophy and stable contour for 2 years. Methods: The authors report their experience treating patients with hemifacial atrophy and linear scleroderma with free tissue transfers between 1989 and 2016. A modified parascapular flap based on the circumflex scapular artery was most commonly used. Results: A total of 177 patients were included. The most common complication was hematoma, occurring in 12 patients (7 percent). Follow-up ranged from 1 to 26 years. All patients subjectively experienced improved facial symmetry and aesthetics. No disease process has recurred to date, even in cases of severe, progressive disease. Conclusions: In the authors’ experience, patients treated early in their dis- ease course have immediate and sustained correction of their deformity, with slowing or in most cases cessation of the disease process following free tissue transfer. The authors now advocate for immediate reconstruction for active disease, especially in young children. ( Plast. Reconstr. Surg. 142: 1275, 2018.) CLINICAL QUESTIONS/LEVEL OF EVIDENCE: Therapeutic, IV.

P rogressive hemifacial atrophy, as first described by Parry in 1824 and Romberg in 1846, is a rare disorder of unknown cause usually developing in the first or second decade of life. 1,2 It is characterized by slowly progressive, uni- lateral facial atrophy of the skin, soft tissue, muscle, and bony skeleton, most commonly affecting the dermatomes of one or more branches of the tri- geminal nerve. Atrophy of deeper structures may be preceded by hyperpigmentation or depigmen- tation of the facial skin and cicatricial alopecia in the affected areas of the scalp. The disease has a 1.5:1 female-to-male ratio. 3 This clinical presenta- tion is very similar to linear scleroderma en coup de sabre, and in fact both diseases may be coexistent, making them difficult to distinguish. 4–6 Neurologic findings can range from subclinical abnormalities From the Division of Plastic and Reconstructive Surgery, University of Wisconsin School of Medicine and Public Health; and Summit Medical Group. Received for publication May 2, 2017; accepted June 5, 2018. Copyright © 2018 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0000000000004922

to facial pain, epileptic seizures, and migraines. 5,7–10 Suggested causes include local facial trauma, hyper- activity/hypoactivity of the sympathetic nervous sys- tem, abnormality of the trigeminal nerve, vasculitis related to a collagen vascular disorder, and bacterial or viral infection, including Borrelia burgdorferi . 7,8,11

Disclosure: The authors have no financial interest to declare in relation to the content of this article.

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