Syndromes/Craniofacial Anomalies
Tayler J. Miller (she/her/hers)
Dental Student
University of Colorado Anschutz School of Dental Medicine
Aurora, Colorado, United States
Catherine M. Flaitz, DDS, MS
Chair/ Professor
University of Colorado School of Dental Medicine
Aurora, Colorado, United States
Kaci Pickett, MS
University of Colorado Denver - Anschutz Medical Campus
Chaitanya P. Puranik, BDS, MS, MDS, PhD
Assistant Professor and Director of Predoctoral Education
University of Colorado School of Dental Medicine, Aurora, CO, USA
Children's Hospital Colorado and School of Dental Medicine, University of Colorado Anschutz Medical Campus
Aurora, Colorado, United States
Authors: Miller TM1*, Pickett K2, Flaitz CM3, Puranik CP4
*Presenting author, 1Doctor of Dental Surgery Program, School of Dental Medicine, 2Research in Outcomes for Children’s Surgery 3Division of Diagnostic Sciences, School of Dental Medicine, 4Pediatric Dentistry Residency Program, Children’s Hospital Colorado, and School of Dental Medicine. University of Colorado, Aurora, CO, USA.
Abstract
Purpose: The purpose of this retrospective, observational cohort study was to evaluate the prevalence of developmental dental anomalies and pathoses (DDAP) in children with Wolf-Hirschhorn Syndrome (WHS).
Methods: One million electronic medical-dental records of patients (1-18years) reporting at Children’s Hospital Colorado were screened for WHS diagnosis. Twenty-six charts identified with WHS diagnosis were systematically screened by a calibrated examiner for medical and dental information including dental anomalies of shape, number, position, structure, and other developmental anomalies or pathoses. The collected data was descriptively summarized.
Results: All the reviewed WHS patients had otorhinolaryngological, speech or behavioral findings while four-fifths of the patients had gastrointestinal, musculoskeletal, central, or peripheral nervous system findings. Two-thirds of the WHS patients had respiratory and genitourinary findings while ophthalmological and cardiovascular findings were documented in about half of the cases. Endocrine and hematological findings were relatively rare. All the WHS patients had positive craniofacial findings including microcephaly and dysmorphic features while two-thirds had maxillary excess. Twelve children with WHS had complete clinical and radiographic dental documentation. Of these, Microdontia (83.3%), pyramidal molars (66.7%), taurodontism (50%), dilacerated roots (33.3%), dens invaginatus (16.7%), pulp stones (16.7%), and root anomalies (16.7%) were the most common shape anomalies. There was a high prevalence of number anomalies (hypodontia:50.0%) while the most common positional anomalies included rotated (25%), ectopic (16.7%), infra-occluded (8.3%), distally displaced (8.3%), or impacted (8.3%) teeth.
Conclusion: The study provides a comprehensive review of medical-dental findings in WHS patients to assist pediatric dentists in the detection and management of DDAP.