Surgical Debulking of Persistent Orofacial Swelling in a Patient with Melkersson-Rosenthal Syndrome

对患有梅尔克森-罗森塔尔综合征的患者进行持续性口面部肿胀的手术减容

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Abstract

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous granulomatous disorder that is likely underdiagnosed due to its variability on presentation. Few patients present with the characteristic triad of orofacial swelling, recurrent facial palsy, and lingua plicata; in fact, most patients present with only one or two of the symptoms. Additionally, patients are evaluated by a variety of specialists, including ophthalmologists, otolaryngologists, dermatologists, and plastic surgeons, depending on their particular symptom. There is no consensus standard of care for MRS due to a limited understanding of the disease. We describe a case of a patient with a 5-year history of persistent orofacial edema in the setting of Melkersson-Rosenthal syndrome refractory to medical management, who was treated with surgical debulking of the upper lip. We encourage increased clinical suspicion for Melkersson-Rosenthal syndrome when evaluating persistent orofacial edema in the plastic surgery clinic and highlight the importance of obtaining a full patient history. Surgical debulking can correct for aesthetic deformity while sparing oral function.

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