Challenging Traditional Thinking: Early Free Tissue Transfer for Active Hemifacial Atrophy in Children

Abstract

Background: Hemifacial atrophy (Parry-Romberg syndrome) is an enigmatic craniofacial disorder characterized by progressive facial atrophy. Early age at onset is associated with significant skeletal involvement and jaw abnormalities. Standard reconstructive dogma dictates that the disease should “burn out,” with at least 2 years of no disease progression, before pursuing reconstructive intervention. The purpose of this article is to present the senior author’s (J.W.S.) experience treating preadolescent children with free soft-tissue transfer for reconstruction of progressing hemifacial atrophy, and to review surgical techniques in this patient population. Methods: Pediatric free tissue transfer cases performed by the senior author were reviewed. Free tissue transfer using a circumflex scapular adipofasciocutaneous flap was performed to treat deformities arising from hemifacial atrophy. Results: Thirty-six patients aged 3 to 6 years underwent free tissue transfer in the presence of progressing, active disease. Follow-up ranged from 7 months to 25 years. There were no cases of flap loss. In all patients, the disease process seemed to be altered, with no symptoms recurring to date. Conclusions: The authors believe that early microsurgical correction of hemifacial atrophy in children as young as 3 years is reliable and effective. The authors theorize that the interposition of vascularized, healthy tissue may alter or even halt the progression of disease, and may also prevent the skeletal hypoplasia classically seen in older patients. Further study incorporating autologous fat grafting as a control procedure may provide additional insight into this challenging disease process. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Craniofacial Microsurgery: An Integrated Approach to Management of Cleft and Craniofacial Syndromes, Surgical Experience, and Insights

“…An illustrative case is depicted in Figure 4. Although traditionally employed once the atrophy has stabilized, there is interesting data suggesting that progression may be halted by early free tissue transfer 5,7 . This is an exciting area of new discovery in craniofacial microsurgery.…”

Soft-Tissue Reconstruction in Progressive Hemifacial Atrophy: Current Evidence and Future Directions

“…2,3 Degeneration may persist over the course of 2 to 20 years, until spontaneous quiescence, with an earlier age of onset associated with more severe bony involvement and poorer outcomes, especially in pediatric patients. [4][5][6][7] Although treatment with agents such as steroids, methotrexate, antimalarials, and retinoids may be initiated early on during the disease process in an attempt to curb progression, 8 softtissue reconstruction is often necessary for restoration of form and function. 4,9 Although there exists a…”

“…Plastic and Reconstructive Surgery • September 2022 growing body of literature investigating outcomes, complications, and specific indications associated with different management approaches [10][11][12][13][14] and, more recently, intervention timing, 7,15 there currently exists no consensus regarding optimal protocol for soft-tissue reconstruction in progressive hemifacial atrophy patients. The present article provides a comprehensive synthesis of the available literature to guide evidence-based management.…”

“…2,3 Degeneration may persist over the course of 2 to 20 years, until spontaneous quiescence, with an earlier age of onset associated with more severe bony involvement and poorer outcomes, especially in pediatric patients. 4–7 Although treatment with agents such as steroids, methotrexate, antimalarials, and retinoids may be initiated early on during the disease process in an attempt to curb progression, 8 soft-tissue reconstruction is often necessary for restoration of form and function. 4,9 Although there exists a growing body of literature investigating outcomes, complications, and specific indications associated with different management approaches 10–14 and, more recently, intervention timing, 7,15 there currently exists no consensus regarding optimal protocol for soft-tissue reconstruction in progressive hemifacial atrophy patients.…”

“…4–7 Although treatment with agents such as steroids, methotrexate, antimalarials, and retinoids may be initiated early on during the disease process in an attempt to curb progression, 8 soft-tissue reconstruction is often necessary for restoration of form and function. 4,9 Although there exists a growing body of literature investigating outcomes, complications, and specific indications associated with different management approaches 10–14 and, more recently, intervention timing, 7,15 there currently exists no consensus regarding optimal protocol for soft-tissue reconstruction in progressive hemifacial atrophy patients. The present article provides a comprehensive synthesis of the available literature to guide evidence-based management.…”