Craniofacial findings in fibrodysplasia ossificans progressiva: computerized tomography evaluation
Daniel Rocha Carvalho1, Luciano Farage, Bernardo Jose Alves Ferreira Martins
1Genetics Unit, SARAH Network of Rehabilitation Hospitals, Brasilia-DF, Brazil. danielcarvalho@sarah.br
Objective:
The aim of this study was the evaluation by using computerized tomography (CT) of craniofacial abnormalities in fibrodysplasia ossificans progressiva (FOP) patients regarding jaw restriction and retrognathia.
Study Design:
Seven FOP patients were evaluated retrospectively in this observational study. Inclusion criteria were detection of ACVR1 gene mutation and complete craniofacial CT examination. The age of jaw restriction and presence of retrognathia were clinically determined. The features analyzed were skull base structures and heterotopic ossification (HO).
Results:
Of this group (age range 4-23 years), the 3 oldest patients presented with jaw restriction and retrognathia as well as displayed elongation of the lateral pterygoid plate with HO of the pterygoid muscles that reached the medial surface of the right mandibular ramus. They had significant history of trauma or surgery. The other 4 patients did not have retrognathia or HO involving the facial or masticatory muscles, and the mouth opening was normal.
Conclusions:
CT evaluation can reveal HO of the pterygoid muscles that probably may cause jaw restriction and retrognathia in older FOP patients.
More Related Videos
02:42Analysis of Craniomaxillofacial Malformations in Mice Using Three-dimensional Microcomputed Tomography
Published on: January 17, 2025
13:16Computed Tomography and Optical Imaging of Osteogenesis-angiogenesis Coupling to Assess Integration of Cranial Bone Autografts and Allografts
Published on: December 22, 2015
Related Concept Videos
01:27Imaging Studies III: Computed Tomography
01:22Sutures of the Skull
Sutures are immobile joints between adjacent bones of the skull. The narrow gap between the bones is filled with dense, fibrous connective tissue that unites the bones. The long sutures located between the skull bones are not straight but instead follow irregular, tightly twisting paths. These twisting lines tightly...
01:10Computed Tomography
The technique was invented in the 1970s and is based on the principle that as X-rays pass through the body, they are absorbed or reflected at different levels. In the technique, a patient lies on a motorized platform while a computerized axial tomography (CAT) scanner rotates...
01:29Bone Formation by Intramembranous Ossification
The process begins when mesenchymal cells in the embryonic skeleton gather together and differentiate into osteogenic cells, which then develop into...
01:27Cranial Bones: Lateral View
The temporal bone forms the lower lateral side of the skull. The temporal bone is subdivided into several regions. The flattened upper portion is the squamous portion of the temporal bone. Below this area and projecting anteriorly is the zygomatic process of the temporal bone, which forms the posterior portion of the zygomatic arch. Posteriorly is the mastoid portion of the temporal bone. Projecting...