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

Abstract

IntroductionEpidermolysis bullosa (EB) is a group of genetic disorders characterized by fragility of the skin and mucosal membranes. Dystrophic EB (DEB) is caused by mutations in the gene coding for type VII collagen. Among the most frequent oral manifestations in Recessive DEB (RDEB) are oral ulcers and blisters, absence of tongue papillae and palatal rugae, ankyloglossia, oral vestibule obliteration, and microstomia. The following report describes a modified impression technique used in a patient with severe RDEB and severe microstomia to obtain models for orthodontic treatment with aligners.Case ReportA 25‐year‐old female patient with severe RDEB was referred for orthodontic treatment. Severe microstomia (8 mm), hindered the use of conventional trays or intraoral scanners to design the aligners. Therefore, a contracture release surgery in combination with a modified impression technique was performed to obtain an optimal impression and subsequent aligners for orthodontic treatment.DiscussionThis case presents an alternative strategy to provide orthodontic treatment with aligners in patients with severe microstomia due to severe RDEB. Reports of orthodontic treatment in people living with EB, especially in RDEB, are still rare, with few publications about fixed braces, early teeth extraction and removable devices, and none using aligners. Most of the impression techniques reported are aimed at oral rehabilitation. The multidisciplinary approach and impression technique reported should broaden the alternatives of orthodontic techniques provided to patients with EB and severe microstomia.ConclusionsThis article describes an oral contracture release surgery and modified impression technique for obtaining good quality impression for the design of orthodontic aligners in patients with severe microstomia due to severe RDEB.

Comprehensive orthodontic treatment using miniscrews and digital rehabilitation in a patient with severe recessive dystrophic epidermolysis bullosa

“…Even after this, the authors had to develop a flexible custom‐made tray because the mandibular width at the lower molar area was larger than the width of the mouth during maximal mouth opening. They used a thermoplastic sheet to create a flexible tray and obtained the impressions for the aligner's design for treating the patient 17 . Similarly in our case, the multidisciplinary team approach was crucial for achieving success, as the periodontal surgery to deepen the oral vestibule enabled the placement of the miniscrews, and the oral rehabilitation team provided an aesthetic and stable anterior teeth rehabilitation.…”

“…First, the lack of attached gingiva did not allow a correct placement of the miniscrews. With a 2 mm depth, it was necessary to increase the vestibule with a periodontal surgery, similar to the surgery described by Véliz et al 17 Once the miniscrews were placed, the vestibule obliteration and the lack of mobility of the upper lip occasionally produced traumatic wounds in the mucosa due to the head of the miniscrew, which measured 3 mm approximately. This was managed with the use of orthodontic wax, commonly used in orthodontics and also described by Pacheco and de Souza.…”

“…[10][11][12][13] It also has been reported that there is reduced maxillary length, possibly due to poor nutritional intake and peri-oral scarring. 5,14 Orthodontic techniques available for patients with severe RDEB include removable 15 and fixed appliances, 12 early teeth extraction 16 and aligners, 17 all of which require clinical modifications due to scarring and skin fragility. The challenges of using miniscrews in EB have not been documented in the literature.…”

Prevention and treatment of microstomia

“…After the occurrence of microstomia due to various causes such as trauma, cancer ablation surgery, or burns, patients may be treated by either reusing their existing dentures or creating new ones using different types of dental impression trays, which include both conventional and digital methods applied serially [ 1 , 2 , 20 , 53 ]. There are studies that categorize oral appliances based on their shape, direction, and position.…”

“…It has been reported that excellent outcomes can be achieved by employing the various surgical treatment methods introduced earlier and by immediately placing dentures or prosthetics [ 53 , 90 ].…”