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Quintessence Publishing: Journals: QI
Quintessence International

Edited by Eli Eliav

ISSN 0033-6572 (print) • ISSN 1936-7163 (online)

Publication:
April 2011
Volume 42 , Issue 4

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Hallermann-Streiff syndrome: Case report and literature review

Robotta, Peter / Schäfer, Edgar

Pages: 331 - 338
PMID: 21516279

Hallermann-Streiff syndrome is a rare genetic disorder characterized primarily by head and face abnormalities. Patients show birdlike faces; hypotrichosis; various ophthalmic disorders; and dental abnormalities including absence of teeth, natal and neonatal teeth, enamel hypoplasia, and supernumerary teeth. In addition, delayed eruption of existing teeth and severe agenesis of permanent teeth are frequent findings. Dental and hereditary disorders can be associated with disturbances during tooth development and cause shortened roots. Short roots are a rare developmental anomaly in the permanent dentition, and the etiology is not well established. The generalized form is extremely rare. Generalized diminished root formation can lead to early loss of teeth. This article provides a case report of a 9-year-old boy with Hallermann-Streiff syndrome. Extraoral examination revealed a brachycephalic head, proportionate short stature, sparse hair, and atrophic skin. His face was characterized by a thin beak-shaped nose and retrognathia, resulting in a characteristic birdlike appearance. Radiographically, all teeth of the permanent dentition showed severely underdeveloped roots and partially underdeveloped crowns. The predisposition to severe dental caries and dental malformations makes it imperative to schedule effective prevention measures, especially since root canal treatment to preserve teeth can be hampered due to underdeveloped roots.

Keywords: anodontia, dental anomalies, Francois syndrome, short roots, underdeveloped roots

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