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

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.

Severe Agnathia-Otocephaly Complex: Surgical Management and Longitudinal Follow-up From Birth Through Adulthood.

Agnathia-otocephaly complex (AOC) is characterized by mandibular hypo- or aplasia, ear abnormalities, microstomia, and microglossia. Though rare and often fatal, this is the first report detailing various reconstructive strategies beyond infancy as well as longitudinal follow-up into adulthood. All patients with AOC treated at our institution over a 30 year period were reviewed. Four patients were identified, one with agnathia, one with micrognathia. Two males with nanognathia (defined as a symphyseal remnant without body nor ramus) were also included. The mean follow-up was 17 years. All four underwent perinatal tracheostomy and gastrostomy-tube placement. Commissuroplasties were typically performed before 3 years of age and repeated as necessary to allow for oral hygiene. Mandibular reconstruction was most successful with rib between ages 3 and 8, after which time, free fibula transfer was utilized. Due to some resoprtion or extrusion, all patients underwent repeated bone grafting procedures. Tissue expansion of the neck was used to restore the lower third of the face, but was most successful in the teenage years. At last follow-up of the eldest patients, one was in college while another was pursuing graduate education. AOC need not be a fatal nor untreatable condition; a reasonable quality of life can be achieved. Although the lower-facial contour may be improved, and a stoma created, the lack of musculature make deglutition virtually impossible with current therapies. Just as transplantation has emerged as a modality for facial restoration following severe trauma, so too may it be a future option for congenital deformities.

Freeman-Sheldon syndrome: combined surgical and non-surgical approach.

BACKGROUND: Many Freeman-Sheldon syndrome patients suffer from extensive microstomia resulting in possible inhibition of dental and skeletal development as well as difficulties in eating, speech and dental hygiene. Oral commissure contraction treatments vary from patient education to complicated surgical and/or prosthetic treatments, but recurrence is often described. This article reports on a combined surgical and non-surgical approach in order to increase maximum mouth opening and maintain the result of the surgery. TECHNIQUE: The patient underwent bilateral commissuroplasty using 2 rhomboid flaps for each side. After two steps of intraoral and perioral tissue impression taking, a master cast was poured and a retractor was fabricated from thermoplastic material. The patient was encouraged to wear the retractor the entire day and night except at meal times. RESULTS: After surgery maximum mouth opening was increased from 20 mm to 37 mm and remained the same six months after the surgery. Psychosocial factors were improved and the patient was satisfied with the results of her treatment. CONCLUSION: The use of bilateral commissuroplasty in combination with this customized dynamic oral commissure retractor may be applicable in other syndromic patients with a small oral commissure and also in patients with circumoral burns.