April 2020 HSC Section 4 - Plastic and Reconstructive Problems

Plastic and Reconstructive Surgery • November 2018

PATIENTS AND METHODS

The goal in the surgical treatment of these diseases is to restore facial symmetry. Several methods of reconstruction have been proposed, including dermal fat grafts 12,13 ; silicone fluid injec- tions 14 ; cartilage and bone grafts; pedicled cuta- neous, muscular, and musculocutaneous flaps 15 ; lipoaspirated fat injections 16 ; and free flaps. 17–34 The facial asymmetry caused by Parry-Romberg disease and linear scleroderma presents a difficult reconstructive problem. The treatment of facial asymmetry is multifaceted, and the patient ben- efits from a team approach using the expertise of microsurgeons, craniofacial surgeons, ortho- dontists, prosthetists, and psychologists. Early in our series, the disease in most cases was allowed to “burn out” and reach a stable state with halted progression before reconstruction. Later in the series, patients were operated on earlier in the course of their disease and had comparable if not improved outcomes at long-term follow-up, with cessation of the progression of their disease. The bony skeleton must be assessed and dealt with before microsurgical reconstruction. If man- dibular or maxillary hypoplasia was severe enough because of growth disturbances secondary to the disease process, the patient underwent craniofa- cial procedures such as orthognathic jaw surgery, mandibular or maxillary distraction osteogenesis, bone grafting, or alloplastic implant augmenta- tion. For our young patients with preexisting skeletal asymmetries before free-flap surgery, secondary orthognathic surgery or other skeletal surgery proceeded without complication at the time of skeletal maturity. The bony framework in Romberg hemifacial atrophy may resorb slightly but, in general, the skeletal scaffold on which to build is sufficient, unlike hemifacial microso- mia and other craniofacial anomalies. 35 The only exception to this may be with the early childhood patient with aggressively progressive disease. In these patients, skeletal growth disturbances can be severe. Many microsurgical donor sites have been described, including groin, anterolateral thigh, superficial inferior epigastric, and omental flaps. 17–34,36 The senior author (J.W.S.) primarily uses a customized parascapular flap and has dem- onstrated good results in a variety of congenital and acquired facial deformities. 18,27–35 This flap has become a workhorse in the correction of facial asymmetry; in particular, Romberg disease and linear scleroderma. In this article, we present a 26-year experience with reconstruction of hemi- facial atrophy caused by Parry-Romberg and lin- ear scleroderma.

Demographics From 1989 to 2016, 177 patients with Romberg disease and linear scleroderma underwent micro- surgical correction of facial contour deformities. Flaps used included circumflex scapular, groin, and superficial inferior epigastric flaps. Early in the senior author’s career, the groin and super- ficial inferior epigastric artery flaps were used in cases with uniform soft-tissue requirements. Since that time, the parascapular flap has been used exclusively because of the ability to reconstruct variable soft-tissue defects even down to tissue paper thickness with the same flap. The senior author (J.W.S.) preformed all microsurgical pro- cedures. The operative technique for microsurgi- cal correction of facial defects has been described previously by the senior author. 18,27–35 What follows is a brief review. Again, early in the series, the dis- ease was allowed to “burn out” or reach a stable state before free-flap reconstruction. Later in the series, patients were operated on earlier and with active disease, resulting in what we conclude to be superior results. Preoperative Evaluation Medical photography was performed in all cases. Patients were examined in the upright position, and facial contour irregularities were marked in three dimensions. Preoperatively, the patients were marked topographically to aid in

Fig. 1. Topographic markings delineate contour irregularities.

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