Oral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation.
BACKGROUND
Hurler Syndrome is associated with a deficiency of a specific lysosomal enzyme involved in the degradation of glycosaminoglycans. Hematopoietic stem cell transplantation (HSCT) in early infancy is undertaken to help prevent the accumulation of glycosaminoglycans and improve organ function.


OBJECTIVE
To investigate the oral features and dental health of patients with Hurler Syndrome who have undergone successful HSCT.


METHODS
Twenty-five patients (median age 8.6 years) post-HSCT (mean age 9.4 months) underwent oral assessment (mean of 7.5 years post-HSCT).


RESULTS
Dental development was delayed. Numerous occlusal anomalies were noted including: open-bite, class III skeletal base, dental spacing, primary molar infra-occlusion and ectopic tooth eruption. Dental anomalies included hypodontia, microdontia, enamel defects, thin tapering canine crowns, pointed molar cusps, bulbous molar crowns and molar taurodontism. Tooth roots were usually short/blunted/spindle-like in permanent molars. The prevalence of dental caries was low in the permanent dentition (mean DMFT 0.7) but high in the primary dentition (mean dmft 2.4). Oral hygiene instruction with plaque and or calculus removal was indicated in 71% of those that were dentate.


CONCLUSIONS
Patients with Hurler Syndrome post-HSCT are likely to have delayed dental development, a malocclusion, and dental anomalies, particularly hypodontia and microdontia.
McGovern
Eleanor
E
Dental Department, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland. eleanor.mcgovern@olchc.ie
Owens
Laura
L
Nunn
June
J
Bolas
Andrew
A
Meara
Anne O'
AO
Fleming
Padraig
P
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004245:DMF Index; D003731:Dental Caries / Q000209:etiology; D005145:Face / Q000002:abnormalities; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D008310:Malocclusion / Q000209:etiology*; D008059:Mucopolysaccharidosis I / Q000150:complications* / Q000628:therapy; D011795:Surveys and Questionnaires; D014071:Tooth Abnormalities / Q000209:etiology*; D014077:Tooth Erosion / Q000209:etiology; D055815:Young Adult