Effectiveness of surgical treatment of severe macrocheilia in a patient with orofacial granulomatosis.

Orofacial granulomatosis (OFG) is the term given to a group of diseases characterized by the presence of non-necrotizing granulomatous inflammation affecting the soft tissues of the orofacial region. Treatment of OFG is often challenging and unsatisfactory. We report on a 32-year-old man with a 2-year history of oedema and swelling of the upper lip without systemic symptoms. The history, clinical features and histopathological findings led to the diagnosis of cheilitis granulomatosa (CG), a disease included in the spectrum of OFG. The patient was treated with oral diaminodiphenyl sulfone (DDS) and clofazimine without success. Oral doxycycline led to a slight improvement of the disease. Because the volume of the upper lip was twice normal size, surgical reduction was performed, followed by administration of oral doxycycline for 3 months. This therapeutic approach led to complete remission, with no recurrence after 3 years.

Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression is effective to prevent further recurrence.

OBJECTIVE: To study the role of total facial nerve decompression in preventing further recurrence of facial palsy in Melkersson Rosenthal syndrome (MRS). METHODS: Total facial nerve decompression was performed on nine patients with recurrent facial palsy in MRS, and prednisolone treatment was given to 6 cases who declined surgery. They were incorporated into surgery group and control group, respectively. Patients in surgery group and control group were followed up for 5.4 ± 1.4 years (range, 4 to 8 years) and 6.0 ± 1.4 years (range, 4 to 8 years), respectively. RESULTS: Further episodes of facial palsy affected none of 9 cases (0.0%) in surgery group, while they affected 3 of 6 cases (50.0%) in control group, with significant difference (p<0.05). CONCLUSIONS: Total facial nerve decompression was effective to prevent further episodes of facial palsy in MRS.

Functional and Therapeutic Indications of Liposuction: Personal Experience and Review of the Literature.

Liposuction is the most common cosmetic surgical procedure worldwide. It has evolved from being designed primarily for body contouring to becoming essential adjunct to various other aesthetic procedures, greatly enhancing their outcome. Despite its hard clear differentiation between an aesthetic and therapeutic indication for some pathologic conditions, liposuction has been increasingly applied to a gamut of disorders as a therapeutic tool or to improve function. In fact, liposuction has ceased to define a specific procedure and became synonymous to a surgical technique or tool same as the surgical knife, laser, electrocautery, suture material, or even wound-dressing products. At present, there seems to be an enormous potential for the application of the basic liposuction technique in ablative and reconstructive surgery outside the realm of purely aesthetic procedures. The present review contemplates the various nonaesthetic applications of liposuction, displaying the enormous potentials of what should be considered a basic surgical technique rather than a specific aesthetic procedure. Implications of this new definition of liposuction should induce third-party public payers and insurance companies to reconsider their remuneration and reimbursement policies.

Radiofrequency therapy as an effective treatment for granulomatous cheilitis: A CARE case report.

INTRODUCTION: Granulomatous cheilitis is a rare disorder characterized by recurrent, idiopathic, and painless lip swelling. The diagnosis is proven by histopathological examination. The unknown aetiology and poorly understood underlying mechanism contribute to the difficulty in establishing an effective treatment. This case study proposes the effectiveness of radiofrequency therapy in the management of refractory granulomatous cheilitis. CASE SUMMARY: A 68-year-old patient presented with hypertrophy and swelling of the lower lip, and a biopsy revealed actinic cheilitis. The patient underwent lip shaving and an advancement mucosal flap, and definitive histologic examination confirmed the diagnosis of granulomatous cheilitis. No other signs of orofacial granulomatosis were observed, and the complementary aetiological study was negative for systemic disease. The lip swelling reappeared and persisted, which interfered with the quality of life. Hence, radiofrequency therapy was performed in the submucosal and subdermal layers of the lip, resulting in significant aesthetic and functional improvement and no further relapses after five years. DISCUSSION: The management of granulomatous cheilitis is challenging. The current mainstay treatment is corticotherapy or reduction cheiloplasty in severe cases. Radiofrequency has potential as a treatment option in debilitating macrocheilia, presenting worthy long-lasting functional and aesthetical results, with minimal morbidity.

Reduction cheiloplasty with lateral wedge excisions in cheilitis granulomatosa.

This case presents a method of reductive cheiloplasty: Conway's method with lateral and bilateral wedge excisions contained to the vermillion. It was performed on a female patient with cheilitis granulomatosa after 4 years of trying different medical therapies without effect. The surgery successfully reduced the increased volume, facilitating improved aesthetics and function while preserving oral function, muscle strength as well as sensitivity with no recurrence at the 15-month follow-up. We encourage early collaboration between dermatologists and plastic surgeons regarding the timing of a potential surgery when treating cheilitis granulomatosa.

Surgical Treatment Outcome for Familial Melkersson-Rosenthal Syndrome.

BACKGROUND Recurrent facial nerve palsy, orofacial edema, and fissured tongue are a triad of manifestations that characterize a rare disorder named Melkersson-Rosenthal syndrome. It is important to consider this syndrome when diagnosing atypical, unilateral, or bilateral facial palsies with characteristics of familial prevalence. There is no established outcome prediction for this disease and the syndrome does not have a specific duration or prospective timeline. Recurrent facial paralysis can require surgery and a multidisciplinary approach with regular follow-up. CASE REPORT We describe a 38-year-old woman presenting with a third episode of facial paralysis and discuss her pedigree chart and the treatment course chosen. After conservative treatment with oral corticosteroids, antiviral therapy, and motor physical therapy with no significant improvements, the patient underwent facial nerve decompression surgery with outstanding results. Eight months after surgery and intense postoperative physical therapy, the patient improved from grade VI to grade II palsy on the House-Brackmann Scale. The patient's older brother also presented a fissured tongue and had a history of 2 episodes of facial paralysis. The patient's son, mother, and sister also presented tongue fissuring but did not have any other clinical signs of the syndrome. CONCLUSIONS Despite being rare, Melkersson-Rosenthal syndrome is associated with a family inheritance and its diagnosis has prognostic implications. Therefore, it is of the utmost importance to have suspicion of this disorder in order to improve quality of care and target the treatment accordingly. Surgical treatment in these cases seems to be an excellent choice to treat current facial paralysis and prevent further episodes.

A comprehensive review of current treatments for granulomatous cheilitis.

Granulomatous cheilitis (GC) is a poorly understood disease process belonging to the larger group of orofacial granulomatosis. The treatment of GC has proven exceedingly difficult. While various treatments have been applied to GC, there has been no uniform or predictably successful model demonstrated in the literature. Poor understanding of the aetiological mechanisms underpinning GC has significantly hampered the development of an effective approach to treatment. Those therapies that have shown promise principally consist of agents with anti-inflammatory activity such as corticosteroids and immunomodulatory medications. On a careful review of the literature, we have found no systematic review and assessment of current treatments. We seek to address this absence in the available literature by providing a consolidated overview of current treatment for GC.

Melkersson-Rosenthal syndrome associated with Down syndrome.

We present a clinical case of Melkersson-Rosenthal (M-R) syndrome associated with Down syndrome. No evidence of this association is described in the literature. We also present a technique for the macrocheilia treatment of lower lip caused by M-R syndrome in a patient with Down syndrome. This patient during pediatric age had many events of facial nerve paralysis and edematous episodes of lower lip with unknown etiology. This technique is based on a wedge full thickness central excision of the lower lip and on a transversal lozenge excision in the vermilion portion with orbicular muscle to reverse the chin-labial corner. The results are an agreeable aspect of the lip and physiological digestive and phonetic processes. The technique is safe and simple and the aesthetic functional result is very good. In our case, the postoperative complication is caused by an insufficient collaboration of the patient and it is solved in three weeks.

Surgical treatment of persistent macrocheilia in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa.

BACKGROUND: Various conservative methods for treatment of labial swelling in patients with cheilitis granulomatosa have been attempted, often with only moderate success and sometimes with persistent disfiguring lip swelling. Severe macrocheilia can produce an unaesthetic facial deformity associated with functional disturbances. In patients with persistent macrocheilia, reduction cheiloplasty with excision of excess tissue may be indicated when conservative treatment has proven ineffective in reducing swelling but may have been successful in stabilizing disease. OBJECTIVE: To evaluate long-term results after reduction cheiloplasty in patients with macrocheilia caused by Melkersson-Rosenthal syndrome or cheilitis granulomatosa. DESIGN: Follow-up study in 7 patients with severe persisting macrocheilia, including 3 patients with Melkersson-Rosenthal syndrome and 4 patients with cheilitis granulomatosa in a stable state of disease, treated by reduction cheiloplasty at our hospital between January 1, 1987, and December 31, 2002. Preoperative and postoperative medical histories were obtained, and criteria for the success of surgical treatment were evaluated by clinical examination. Different techniques of reduction cheiloplasty are described and demonstrated in representative cases of severe macrocheilia. RESULTS: Surgical treatment in all 7 patients showed satisfying aesthetic and functional outcomes that persisted throughout follow-up (median follow-up, 6.5 years). CONCLUSIONS: Reduction cheiloplasty is an effective method to correct persistent macrocheilia and improve lip aesthetics in patients with Melkersson-Rosenthal syndrome or granulomatous cheilitis in the persistent state of disease. With careful planning, proper sequencing of treatment, and proficiency in the various surgical techniques, optimal results can be achieved.

Total facial nerve decompression for recurrent facial paralysis: an update.

Recurrent facial paralysis (RFP) is a rare disorder that in some individuals may lead to worsening sequelae. Melkersson-Rosenthal syndrome is a variant of RFP that is associated with recurrent facial edema. In the past, decompression of the mastoid segment of the facial nerve has not been successful in preventing recurrences. In 1981 we began performing total facial nerve decompression for RFP and in 1986 reported its efficacy in one patient with Melkersson-Rosenthal syndrome and in another in whom both nerves were decompressed for alternating bilateral paralysis. An additional four cases with 3 to 8 years of followup demonstrate no recurrences in any patient. Total facial nerve decompression for RFP in selected patients appears efficacious in preventing recurrences. Decompression will remain investigational until further followup is obtained. Furthermore, its salutary effect should not be extrapolated to Bell's palsy without further study.

The surgical management of Melkersson-Rosenthal syndrome.

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.

Total decompression of facial nerve for Melkersson-Rosenthal syndrome.

Melkersson-Rosenthal syndrome (MRS) is a rare condition characterized by recurrent facial paralysis in addition to various orofacial manifestations. The condition appears to be a granulomatous disorder causing oedema and inflammation of the soft tissues of the face, lips, oral cavity and particularly, the facial nerve. There is general agreement that the symptoms and signs resolve spontaneously, aided perhaps by an empirical course of oral steroids. However, in some patients the condition may be progressive, leading to disfiguring facial synkinesis and increasing residual paralysis. There is anecdotal evidence in the literature to suggest that surgical decompression of the facial nerve in its entire intratemporal course may prevent further attacks of facial paralysis and its sequelae. We present here our experience with surgical decompression of the facial nerve in a 27-year-old woman with MRS who had sufferent recurrent left-sided facial palsy since the age of four. A review of the literature pertaining to facial nerve decompression for Melkersson-Rosenthal syndrome is presented.

[Melkersson-Rosenthal syndrome. Value of reduction cheiloplasty]. / Syndrome de Melkersson-Rosenthal Intérêt de la chéiloplastie de réduction.

INTRODUCTION: The interest of cheiloplasty is not precisely defined in the treatment of Melkersson-Rosenthal syndrome (MRS). OBSERVATION: The case of a 32-year-old woman, suffering from a typical MRS is reported. The lack of efficacy of various classical molecules (corticoseroids, hydroxychloroquine, dapsone, colchicine, clofazimine) led to a surgical therapy, consisting in cheiloplasty. Systemic corticosteroids were associated. The result was good, with a follow-up of 2 years. COMMENTS: Cheiloplasty is essentially of cosmetical interest, in the MRS, once the inflammatory process has been stopped (almost one year). Medical treatment (corticosteroids), is to be continued after surgery, to avoid recurrence.