Review of the Current Medical and Surgical Treatment Options for Microstomia in Patients With Scleroderma

Abstract

BACKGROUND Most patients with scleroderma suffer from microstomia, which can have debilitating consequences on their quality of life. Unfortunately, treatment options remain limited. No specific guidelines exist; hence, microstomia remains a challenge to treat in this patient population. OBJECTIVE This review aims to evaluate the different medical and surgical treatment modalities currently available for microstomia in patients with scleroderma and make recommendations for future research. MATERIALS AND METHODS A search of PubMed, Ovid MEDLINE, and Ovid Embase was conducted to identify articles discussing the treatment of microstomia in scleroderma. Twenty articles discussing surgical therapy and one article discussing medical therapy were reviewed. RESULTS Mostly because of a scarcity of high-level evidence, no individual therapy has documented long-term efficacy. Some treatments demonstrate positive results and warrant further research. CONCLUSION Given the variability of results, specific recommendations for the treatment of microstomia in patients with scleroderma are difficult to establish. A multifaceted approach that includes surgical and medical therapy is likely the best option to improve oral aperture in this patient population. Surgical treatments such as neurotoxins, autologous fat grafting, and ultraviolet A1 phototherapy may hold the most potential for improvement.

Impression technique modification and oral contracture release surgery for orthodontic treatment in a patient with severe microstomia due to recessive dystrophic epidermolysis bullosa

“…Other syndromes have been described with microstomia, such as Richieri-Costa-Pereira syndrome, 14 Freeman-Sheldon Syndrome 15 or Scleroderma. 16 Previous reports have shown patients with similar characteristics to the patient presented in this report, such as deep bite, skeletal class II 17 or difficulties for taking impressions due to microstomia. 8 In patients with EB, Shah et al 18 suggested a reduced maxillary size caused by malnutrition and scarring tissue, leading to severe crowding.…”

Use of Botulinum Toxin and Hyaluronic Acid Filler to Treat Oral Involvement in Scleroderma

“…1 Although immunomodulatory therapies are helpful for addressing visceral complications of scleroderma, they have not been demonstrated to be particularly efficacious in addressing cutaneous manifestations. 2…”

“…Other approaches include use of pulsed carbon dioxide laser, intense pulsed light, or ultraviolet A1 phototherapy, although evidence thus far is limited. 2 Recently, onabotulinumtoxin type A has been shown to be beneficial, possibly through paralysis of the orbicularis oris, inhibitory effects on fibroblasts, and increased expression of matrix metalloproteinases. 3…”

Hyaluronidase as a Successful Treatment Modality for Scleroderma-Induced Microstomia

“…Current therapies consist of immunomodulating therapies; however, these are primarily intended for treating visceral manifestations and have demonstrated negligible effect in treating cutaneous symptoms. 1 There have been only two previous reports of the use of hyaluronidase for scleroderma-related microstomia. Both reports demonstrated improvement of oral aperture after sustained treatment over multiple sessions.…”

“…Scleroderma is a chronic autoimmune disorder of connective tissue characterized by excess extracellular matrix production such as glycosaminoglycans such as hyaluronic acid. 1 These changes in the facial skin can result in smooth, taut, mask-like facies that cause psychological distress and decrease self-image. Microstomia is defined as an abnormally reduced oral aperture with an interincisal distance (IID) of less than 50 mm that causes functional impairment.…”