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
Background:
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.
Methods:
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.
Results:
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.
Conclusion:
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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