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The surgical management of Melkersson-Rosenthal syndrome
L T Glickman1, J S Gruss, B D Birt
1Division of Plastic Surgery, Sunnybrook Health Science Centre, Toronto, Ont., Canada.
Abstract:
A triad of facial palsy, orofacial edema, and furrowed tongue constitutes an uncommon condition known as Melkersson-Rosenthal syndrome (MRS). We report on 14 patients with Melkersson-Rosenthal syndrome. Two patients had facial palsy, 12 had orofacial edema, and 1 patient had a furrowed tongue. Nine patients were treated medically. Intralesional steroid therapy had a 75 percent recurrence rate. Systemic steroid therapy resulted in remission in two of three patients. Surgical excision and reconstruction were carried out in five patients with chronic lip or eyelid edema. This provided relief in all cases. The etiology, clinical presentation, and histologic features are discussed. Three illustrative cases are presented. An algorithm is provided that guides the surgeon with regard to both the medical and surgical treatment of the patient with Melkersson-Rosenthal syndrome.
Insights
Melkersson-Rosenthal syndrome (MRS) is rare, characterized by facial palsy, orofacial edema, and furrowed tongue. Surgical intervention offers relief for chronic edema, while systemic steroids show promise for remission.
Area of Science:
- Neurology
- Dermatology
- Otolaryngology
Background:
- Melkersson-Rosenthal syndrome (MRS) is an uncommon neurological disorder.
- It is characterized by a triad of facial palsy, orofacial edema, and furrowed tongue.
Observation:
- This study reports on 14 patients diagnosed with Melkersson-Rosenthal syndrome.
- Facial palsy was observed in 2 patients, orofacial edema in 12, and a furrowed tongue in 1 patient.
- Medical treatments included intralesional and systemic steroid therapy, with varying recurrence and remission rates.
Findings:
- Intralesional steroid therapy for MRS demonstrated a high recurrence rate of 75%.
- Systemic steroid therapy achieved remission in two out of three treated patients.
- Surgical excision and reconstruction provided complete relief for all five patients with chronic lip or eyelid edema.
Implications:
- The findings suggest surgical intervention is highly effective for chronic orofacial edema in MRS.
- Systemic steroids may offer a viable medical treatment option for inducing remission.
- An updated treatment algorithm aids clinicians in managing Melkersson-Rosenthal syndrome patients effectively.