by Iris M. Brito, MD
Plastic Surgery Resident
Coimbra University Hospital Center, Portugal
The February 2020 edition of #PRSJournalClub provided a significant contribution to the literature on Hemifacial atrophy (Parry-Romberg syndrome), a rare craniofacial disorder characterized by progressive facial atrophy that usually develops in the first or second decade of life.
The PRS article entitled “Challenging Traditional Thinking: Early Free Tissue Transfer for Active Hemifacial Atrophy in Children” by Israel, Chen, Farmer, and Siebert, provided an outstanding discussion on the treatment of progressive hemifacial atrophy using free soft-tissue transfer, and the advantages of performing reconstruction in early childhood. Click here to read the article.
The study authors answered questions from around the world and provided further insights into the fascinating results of their study in a discussion hosted by PRS Journal on Facebook, on February 9, 2020.
In this study, the authors hypothesize that active disease during early childhood, a time of significant facial growth, may result in future skeletal growth restriction and profound facial asymmetry that worsens in adolescence and adulthood, and is difficult to correct later in life. Early intervention for active disease in young patients would be beneficial to prevent the development of severe facial deformities, challenging the traditional reconstructive approach usually performed after stabilization of disease progression. The study presents a 25-year clinical experience of the senior author’s preferred technique using microvascular free tissue transfer for reconstruction of active hemifacial atrophy; and a literature review of the surgical management, including techniques and perioperative considerations, of Parry-Romberg syndrome in the pediatric population.
The senior author’s preferred approach comprises a two-stage reconstruction, beginning with free tissue transfer as the primary operation, followed by minor flap revision at 6 months postoperatively. The flap of choice was a modified parascapular flap with variable tissue thickness. The affected face was “marked topographically” to provide a roadmap for determining flap positioning based on required thickness and the areas of the hemiface requiring reconstruction.
The authors found that following free tissue transfer, in children between 3 and 6 years-old, no appreciable atrophy of the skin or underlying tissues was developed. As patients approached skeletal maturity, no new asymmetries were observed. No further tissue augmentation was required in areas where the flap had previously been used for facial reconstruction. Subsequent small-volume autologous fat transfer was performed in areas not easily addressed by fascial extensions of the flap, such as the medial eyelid and nasal ala. Children who underwent early soft-tissue free flap reconstruction did not require any subsequent osseous reconstruction, implying that reconstruction may not only halt worsening of soft-tissue atrophy, but may also prevent worsening or manifestation of skeletal involvement.
The genetic footprint of hemifacial atrophy has not been previously described, and its pathophysiology remains poorly understood. To objectively study the authors’ clinical impressions beyond physical examination, they have begun to use RNA sequencing in both adults and pediatric patients with progressive hemifacial atrophy. In the pediatric population study, interesting findings suggested that the diseased skin involved in hemifacial atrophy may undergo a genetic “normalization” after free tissue transfer.
The authors concluded that reconstruction in young children (as early as 3 years of age) with active, rapidly progressing hemifacial atrophy is benefic, safe and reliable. Soft-tissue reconstruction of hemifacial atrophy using a free parascapular flap provides adequate volume to restore facial symmetry, corrects both severe and subtle deficits with the same flap, and appears to facilitate a reversal of the otherwise rapidly progressive disease process that results in less correctable bony and soft-tissue asymmetry later in life. Using this approach may allow for the prevention of disease progression, even to the extent that major secondary and tertiary bony reconstructive procedures are no longer necessary. Biological studies are currently underway, with the intent to correlate phenotypic and genetic findings.
The article was first discussed by the current Resident Ambassadors to the PRS Editorial Board – Min-Jeong Cho, MD, Casey Kraft, MD, and Ara Salibian, MD – and the special guest David Chang, MD. Listen to the podcast discussion here.
An engaging and thought-provoking online discussion also took place on the #PRSJournal Facebook page on February 9th, where established plastic surgeons and residents were able to ask questions, and get answers from the authors of the article themselves! Don’t worry if you missed it! A summary of the interesting discussion is provided here.
We hope you enjoy the top highlights from the discussion and look forward to seeing you at the next #PRSJournalClub on Facebook!
This February #PRSJournalClub article (as well as other Journal Club selections from February), selected classic pairings and videos, and the entire Facebook Q&A are archived on PRSJournal.com here.