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.

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.

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.

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.

Corrección de «labio ondulado» en elsíndrome de Melkersson-Rosenthal / Upper lip «ruffle deformity» correction in Melkerson-Rosenthal’s syndrome

El Síndrome de Melkersson-Rosenthal es una entidad rara caracterizada por edema granulomatoso oro facial, episodios de parálisis facial y lengua plegada. La presentación monosintomática de labios se denomina queilitis granulomatosa de Miescher. Clásicamente se ha tratado con múltiples medicamentos con respuesta parcial o insatisfactoria, por lo que el tratamiento ideal es el quirúrgico, que obtiene resultados permanentes y permite al paciente relaciones interpersonales satisfactorias. Sin embargo la corrección propuesta por los diferentes autores disminuye el grosor del labio a expensas de dejar un estigma de deformidad de labio “ondulado” ya que no se acorta el labio en sentido transverso y únicamente se adelgaza. El presente artículo describe el tratamiento propuesto para corregir esta deformidad mediante la resección en bloque del tercio medio del labio superior. Además hace referencia a la afectación del cuello en el caso que presentamos, no descrita en la bibliografía actual al respecto, que daba al paciente un aspecto de cuello de “iguana”, y que fue corregida mediante resección en huso y liposucción (AU)

Melkersson-Rosenthal Syndrome is a rare entity characterized by granulomatous orofacial swelling, recurrent facial palsy and lingua plicata. It’s monosyntomatic form affecting only lips is known as Miescher (..) (AU)

Anesthetic management of patients with Melkersson Rosenthal syndrome.

Melkersson Rosenthal Syndrome (MRS) is a rare disorder characterized by relapsing facial paralysis, persistent or recurrent orofacial edema, and lingua plicata. It may cause difficult airway, drug allergy, and angioedema. In our anesthetic management of two patients with MRS, preanesthetic immunological blood examination and skin tests for hypersensitivity to anesthetic drugs were applied. Because the principal goal is to avoid all factors that may stimulate, an allergic reaction, anesthetic drugs known to trigger urticaria were avoided. Body and operating room temperatures, changes of which may trigger allergic reactions, were kept constant during the perioperative period. Emergency precautions were taken for probable angioedema. MRS is a rare syndrome, and if its manifestations are misunderstood as simple facial paralysis, it may be overlooked by anesthesiologists. Anesthesiologists must be careful of several problems in patients with MRS.

Plastic surgical solutions for melkersson-rosenthal syndrome: facial liposuction and cheiloplasty procedures.

OBJECTIVE: : Melkersson-Rosenthal Syndrome (MRS) is a rare granulomatous disease characterized by a triad, including orofacial swelling, facial palsy and lingua plicata with usually recurrent or progressive course. Orofacial swelling, the most often sign of MRS, leads to the both esthetic and functional deformities. Because of its unknown etiology, a rational treatment is difficult and management of MRS still remains symptomatic, aiming at to remove orofacial swelling mainly. Although the many nonsurgical therapies have been mentioned in the literature, none has been proved uniformly and predictably successful to date. In this paper, we present different surgical procedures and their outcomes in a series of 4 cases with MRS. The procedures including mucosa, submucosa and tangential muscle resection, crescent shaped commissuroplasty, and facial liposuction may be considered in surgical armamentarium when orofacial swelling becomes persistent. We think that the plastic surgeons may act more effectively in the management of the syndrome in the future.

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.

Lip reduction cheiloplasty for Miescher's granulomatous macrocheilitis (Cheilitis granulomatosa) in childhood.

Miescher's granulomatous macrocheilitis (cheilitis granulomatosa) is a mono- symptomatic presentation of Melkersson-Rosenthal Syndrome which is characterized by granulomatous swelling of the lips, and can affect both children and adults. We report the successful outcome following lip reduction surgery on an 11-year-old boy. We suggest that surgery may have a role in the management of those patients who have failed to respond to medical treatment.

Treatment of Miescher's cheilitis granulomatosa in Melkersson-Rosenthal syndrome.

Cheilitis granulomatosa is the most frequent dermatological sign in the Melkersson-Rosenthal syndrome. We reviewed 27 cases of this syndrome diagnosed and treated in the last 20 years. All the patients were surgically treated and received intralesional corticosteroids during surgery and tetracycline hydrochloride (500 mg, twice daily) after the operation to prevent recurrence.

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: reduction cheiloplasty utilizing a transmodiolar labial suspension suture.

Melkersson-Rosenthal syndrome is a rare condition which consists of progressive and recurrent orofacial edema, intermittent facial palsy, and lingua plicata. Labial swelling is the most common feature of this syndrome complex. The lips may enlarge up to three times their normal size, resulting in aesthetic deformity and functional disability. Traditional medical interventions are only marginally successful in treating this syndrome. We present a case of Melkersson-Rosenthal syndrome with massive lower lip enlargement, which was successfully treated by combining a fleur-de-lis resection with a transmodiolar labial suspension suture. Five-year follow-up photographs are 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.

Long-term results after surgical reduction cheiloplasty in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa.

Cheilitis granulomatosa in patients with Melkersson-Rosenthal syndrome has proven difficult to treat, and various forms of symptomatic treatment of orofacial swellings have been used with varying success. To evaluate long-term results after surgical reduction cheiloplasty in patients suffering from persistent lip swellings, a follow-up study was performed on 13 patients after 16 years (median). The operations were performed after unsuccessful conservative treatment and regardless of disease activity. Pre- and postoperative medical histories were updated and a clinical examination evaluated the following criteria for a good result: symmetry of the lip, a normalized anterior projection, dimensional harmony between upper and lower lip, no disfiguring cicatrices, and no sensory disturbances. In spite of postoperative disease activity in 6 patients, all 13 patients were satisfied with the long-term result. Clinically, the results in 12 patients were considered good. We therefore recommend lip resection as described when a stationary swelling becomes associated with permanent aesthetic deformity or functional disturbances and conservative measures are unsuccessful.