April 2020 HSC Section 4 - Plastic and Reconstructive Problems

Volume 142, Number 5 • Progressive Hemifacial Atrophy Treatment

Fig. 5. Case 1. A 3-year-old girl with active right hemifacial atrophy.

Fig. 6. Case 1. The patient is shown at age 3, following recon- struction with a parascapular free flap and one secondary revision.

in the overlying tissues are ongoing. The most recent 50 patients were surveyed. There was a 52 percent response rate. Sixty percent of all respon- dents were unhappy with their appearance before surgery, and 50 percent were unhappy with color change. One-third of all patients were extremely satisfied, one-third were very satisfied, and one- third were somewhat satisfied with surgery. Ninety percent of patients would consider another opera- tion in the future, 95 percent would repeat the initial surgery again, and 100 percent of patients would recommend surgery to other patients. Table 1 summarizes the remaining survey results. The appearance subscale demonstrated the low- est scores and likely the greatest negative effect on quality of life compared with the other subscales. Complications included hematoma in 12 patients, one partial flap loss, one patient with partial facial skin slough treated with local wound care, and cellulitis in one patient. One flap was lost at 2 weeks after surgery when the patient began smoking heavily. Recurrence of facial atro- phy was not seen. The following representative case reports further illustrate the above points (Figs. 5 through 11). ( See Figure, Supplemental Digital Content 2 , which shows case example 3, a 16-year-old boy with right hemifacial atrophy. The photograph on the right is shown after one secondary revision, http://links.lww.com/PRS/ D30 . See Figure, Supplemental Digital Content 3 , which shows case example 3, the patient depicted in Supplemental Digital Content 2, now seen at

age 32 with stable results, 16 years after initial cor- rection, http://links.lww.com/PRS/D31 .)

DISCUSSION Romberg disease and linear scleroderma pres- ent the surgeon with the difficult reconstructive problem of facial asymmetry. We believe that our 26-year experience with microsurgical free-flap reconstruction of these defects has shown the technique to yield long-lasting, excellent results. We have previously reviewed our initial expe- rience with microvascular flap reconstruction of severe hemifacial atrophy in 15 patients. 29 In this larger series, we have operated on 162 more such patients and further demonstrate that microvascu- lar reconstruction of facial asymmetry caused by linear scleroderma and Romberg disease is safe, reliable, and highly effective in these patients, even in the setting of active disease and progres- sive atrophy. The majority of patients underwent reconstruction with a modified parascapular free flap. This flap has also been used successfully for other causes of facial asymmetry as reported previ- ously by the senior author (J.W.S). 18,27–35 The para- scapular flap is currently our first choice in facial asymmetry caused by Romberg disease or linear scleroderma unless unavailable. The parascapu- lar flap, including dorsal thoracic fascial exten- sions overlying the latissimus dorsi, trapezius, and other regional muscle fascia, allows for pre- cise simultaneous reconstruction of both severe

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