Program including abstracts as pdf available here
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P 79<br />
Title<br />
CbCT IN THE EVALuATION OF GOLDENHAR SyNDROME PATIENTS<br />
Authors<br />
P. FIGuEIREDO 1 , A.P. RESENDE 1 , P.M. yAMAGuTI 1 , A.R. OLIVEIRA 1 , R.O. COSTA 1 ,<br />
M.S. CÓRDObA 2 , J.F. MAzzEu 3 , H.D. MESTRINHO 1<br />
Affiliations<br />
1 University of Br<strong>as</strong>ília, Oral Care Center for Inherited Dise<strong>as</strong>es, Br<strong>as</strong>ília, BRAZIL, 2 University<br />
of Br<strong>as</strong>ília, Faculty of Medicine - Department of Medical Genetics, Br<strong>as</strong>ília, BRAZIL, 3 University<br />
of Br<strong>as</strong>ília, Department of Genetics and Morphology, Br<strong>as</strong>ília, BRAZIL<br />
Body<br />
Objectives: Goldenhar syndrome (hemifacial microsomia, OMIM 164210) is a rare <strong>here</strong>ditary<br />
condition characterized by anomalies arised from the first and second brachial arches, <strong>including</strong><br />
craniofacial anomalies. CBCT is a volumetric imaging method to analyze the morphology<br />
of the craniofacial bones. T<strong>here</strong>fore, it may be useful to detect the craniofacial anomalies<br />
related to Goldenhar Sydrome. The objective of this study w<strong>as</strong> to describe the main craniofacial<br />
imaging findings <strong>as</strong>sociated with Goldenhar syndrome patients.<br />
Methods: After being diagnosed by the Clinical Genetics Center (HUB), four Goldenhar Syndrome<br />
patients were referred for oral evaluation at the Oral Care Center for Inherited Dise<strong>as</strong>es of<br />
the University Hospital of Br<strong>as</strong>ilia (HUB). The patients were examined and panoramic radiographs<br />
and cone beam computed tomography (CBCT) were performed in order to characterize<br />
the craniofacial features.<br />
Results: Three female and one male patient aging 4, 6, 12 and 37, respectively, were examined.<br />
All patients presented ear deformities, micrognathia and malocclusion. Panoramic radiographs<br />
suggested the presence of mandibular anomalies, mainly hypopl<strong>as</strong>ia or apl<strong>as</strong>ia of the mandibular<br />
condyle and/or ramus. CBCT revealed the following findings: high-angle mandibular growth<br />
pattern, condyle`s and mandibular ramus apl<strong>as</strong>ia, and mandibular ramus and coronoid process<br />
hypopl<strong>as</strong>ia. Bony ankylosis of temporomandibular joint w<strong>as</strong> detected in one patient.<br />
Conclusion: CBCT w<strong>as</strong> useful to characterize the temporomandibular joint and mandibular<br />
ramus anomalies presented in the four Goldenhar Syndrome patients evaluated.<br />
Keywords<br />
Goldenhar Syndrome,Cone-beam computed tomography,Craniofacial abnormalities<br />
194
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