Oral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation

Abstract

Patients with Hurler Syndrome post-HSCT are likely to have delayed dental development, a malocclusion, and dental anomalies, particularly hypodontia and microdontia.

Oral manifestations in children with mucopolysaccharidosis

“…The extent of the oral affections depends on the severity of the disease and the general state of health of the patient, and this will, in turn, impact upon oral care. 8,21,22 Our results showed a greater prevalence of macroglosia and lingual interposition in patients with mucopolysaccharidosis, which is corroborated in the literature by several authors. 7,9,[13][14][15][20][21][22][23][24][26][27][28][29] The macroglossia is due to the deposition of glycosaminoglycans (GAG) in the tongue structure that usually results in an anterior open bite.…”

“…8,21,22 Our results showed a greater prevalence of macroglosia and lingual interposition in patients with mucopolysaccharidosis, which is corroborated in the literature by several authors. 7,9,[13][14][15][20][21][22][23][24][26][27][28][29] The macroglossia is due to the deposition of glycosaminoglycans (GAG) in the tongue structure that usually results in an anterior open bite. 7,9,13,15,[19][20][21][22][23][24][25][26][27][28]30,31 The higher prevalence of lingual interposition in patients with MPS may be due to the presence of macroglossia, anterior open bite and strong maxillary compression.…”

“…Radiographic features that have been reported include short mandibular rami with abnormal condyles; reports also include delayed eruption teeth related to the presence of multiple unerupted teeth with large thickened dental follicles secondary to excessive dermatan sulfate and collagen deposition. 3,[7][8][9][13][14][15][17][18][19][20][21][22][23][24][25][26][27] Notwithstanding, to date there are no studies comparing oral features of patients with mucopolysaccharidosis with controls without the disease. The evaluation of dental conditions in patients with MPS is scarce considering that in literature only clinical cases are described.…”

“…3,7-9,13-15,17-27 Thick lips with an open mouth posture are also features associated with this syndrome. 3,7-9,13-15, [17][18][19][20][21][22][23][24][25][26][27] Few studies have reported the oral and dental changes in MPS patients. The reported alterations include a short and broad mandible, macroglossia, anterior open-bite, high arched palate with pronounced rugae, spaced dentition, lip hypotonia, gingival hyperplasia and enlargement of the alveolar process.…”

“…The accumulation of mucopolysaccharides impacts the stomatognathic system, leading to changes in oral health and to the development of the dentition of patients with this disease. 3,[7][8][9][13][14][15][17][18][19][20][21][22][23][24][25][26][27] Craniofacial features consist of coarse facial features high prominent forehead, snub nose with a depressed nasal bridge, broad nasal tip, and anteverted nostrils. 3,7-9,13-15,17-27 Thick lips with an open mouth posture are also features associated with this syndrome.…”