Reconstruction: One small component of Parry-Romberg Syndrome
Alex Cappitelli, Olivia Langa, Ingrid Ganske
Department of Plastic & Oral Surgery, Boston Children's Hospital, Boston, MA
Parry-Romberg Syndrome is a rare disorder causing unilateral, progressive wasting of the face, including the skin, soft tissue, muscle, cartilage, and bone. This condition is managed surgically by many facial plastic or oromaxillofacial surgeons; however, understanding the inflammatory and relapsing nature of the disease is important for tailoring multidisciplinary care.
This is a retrospective review of patients with Parry-Romberg Syndrome seen at our institution from 1980-2020. Variables collected included: age at onset, duration of wasting, incidence of relapse, and treatment strategies implemented.
One hundred six patients were identified. 65.1% of patients were women (n=69) and 34.9% were men (n=37). Atrophy began at a median age of 6.5 years old (IQR = 7.88 years), and lasted for a median length of 18 months (IQR = 30 months) before stabilizing. 72.6% of patients (n=77) received medication in response to their initial flare, most commonly a steroid pulse regime supplemented by methotrexate. 29.2% of patients (n=31) reported secondary flares a median 2 years (IQR= 3.42 years) after their initial flare was deemed stable. 5.6% of patients (n=6) reported tertiary flares a median 2.3 years (IQR=3.46 years) after secondary stabilization. 29.1% of all patients (n=25) underwent reconstructive procedures, 56% of whom (n=14) did not receive other medical treatment.
Understanding the relapsing nature of this inflammatory condition is important for surgical planning. Reconstructive surgeons should be aware of the medical management options and prognosis for Parry-Romberg Syndrome.
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