“…4 Clinically, this is of importance to dentists due to its correlation with an increased incidence of anterior open bite (AOB), bimaxillary proclination and a skeletal III base (mandibular prognathism in relation to the maxilla, or cranial base, or maxillary hypoplasia in relation to the cranial base). 20 Such prognathism, which can also be seen in the Alpha mannosidosis and Pompe disease (GSD type II) 5 (in the absence of macroglossia) can cause functional, masticatory, speech, and aesthetic challenges. 14,21 In MPS, the macroglossia can be attributed to glycosaminoglycan accumulation in the head and neck region, with hypertrophy of the adenotonsillar region and upper and lower airway being commonly observed.…”