Adults from both genders in the age range of 20-60 years having >2 missing teeth in posterior region and complaint of chronic orofacial pain, mouth opening less than 40 mm in the absence of microstomia
, and with normal facial morphology were included.
Se acompana de otras malformaciones como: hipoplasia maxilar, implantacion baja de pabellones auriculares, sinotia, ausencia de la lengua, microstomia
y persistencia de la membrana bucofaringea (Suarez-Obando & Prieto, 2007; Kauvar et al, 2010; Oviedo et al., 2011; CoronaRivera et al, 2013).
Among the cutaneous features (Table 1), fingertip ulceration was found in 36 (66.7%), sclerodactyly in 48 (88.8%) (Figure 1), digital pitted scars in 30 (55.5%) (Figure 2), digital gangrene in 3 (5.6%), calcinosis cutis in 4 (7.4%), microstomia
in 48 (88.8%) (Figure 3), radial furrows around mouth in 18 (33.3%) (Figure 4), pinched nose in 33 (61.1%), diffuse alopecia in 10 (18.5%) and nail changes in 10 (18.5%) cases.
Mellerio noted that EB can also adversely affect oral health and lead to the formation of painful blisters, scarring, microstomia
, and ankyloglossia, which "can contribute to difficulties eating and speaking and make it hard to keep the teeth clean." Analgesics can be helpful, as can an NSAID mouthwash or spray or mucoprotectants like Episil that coat the surface of lesions.
There are limited treatment options for scleroderma microstomia
. The recommended treatment for limited mouth opening is based on stretching exercise for 3 months that need to be continued in the long term .
Patients typically show a distinct physiognomy, initially also described as craniocarpotarsal dysplasia [1, 4]: contractures of musculature and soft tissues lead to characteristic circumoral fibrosis, microstomia
, pursed lips, micrognathia, and a short webbed neck with severely limited range of motion.
Lin, "Integrating hinge axis approximation and the virtual facial simulation of prosthetic outcomes for treatment with CAD-CAM immediate dentures: a clinical report of a patient with microstomia
," Journal of Prosthetic Dentistry, 2017.
Physical examination revealed disharmonic short stature (Figure 1), a craniofacial dysmorphism characterized by: macrocephaly with flat occiput, wide fontanels, high forehead, high anterior hairline, nevus flameus on forehead, hypertelorism, flat nasal bridge with anteverted nostrils and wide nasal base, short and deep philtrum, microretrognathia, microstomia
, cleft palate, low-set and posteriorly rotated ears and short neck (Figure 2).
It is characterised by facial and distal limb contractures, the most common being microstomia
with pouting lips, camptodactyly with ulnar deviation of the fingers and talipes equinovarus.
[20.] Geckili O, Cilingir A, Bilgin T (1982) Impression procedures and construction of a sectional denture for a patient with microstomia
: a clinical report.
Malignant hyperthermia in a 3-year-old child with microstomia
. J Craniofac Surg 2015;26:217-9.