Presentation
Facial deformity since birth. Right ear deformity and deafness. CT examination is prior to mandible graft.
Patient Data
The right ear shows complete absence of the structures derived from the first branchial appendage (complete absence of external and middle ear), apart from anteriorly displaced dysmorphic auricle.
Hypoplastic right hemimandible and absence of right temporomandibular joint. Muscular atrophy and partial fat replacement of the related muscles of mastication. The mastoid process and body of the right zygoma are absent, developed frontal and maxillary processes. A bony graft is seen continuous with hypoplastic right hemimandible with internal fixation by screws.
Small-sized left mandibular ramus and small left mandibular head not articulating with the skull base (absent left TMJ fossa). The mandibular symphysis and alveolus are receding in relation to maxillary counterpart.
Cranio-facial asymmetry manifested as asymmetrically large right side of the head.
Hypoplastic right hemimandible and absence of right temporomandibular joint. The mastoid process and body of the right zygoma are absent. The mandibular symphysis and alveolus are receding in relation to maxillary counterpart. Cranio-facial asymmetry manifested as asymmetrically large right side of the head.
Case Discussion
Hemifacial microsomia (otomandibular dysostosis) is due to asymmetric abnormal development of the first and second branchial arches. It primarily affects the ear, mandible, maxilla, muscles of mastication and soft tissues of the lower face of the affected side. It may also involve the eye, other parts of the skull, as well as nerves and soft tissue.
Other names: Craniofacial microsomia, lateral facial dysplasia, first and second branchial arch anomaly, branchial arch syndrome, facioauriculovertebral syndrome and oculoauriculovertebral spectrum.
Unable to process the form. Check for errors and try again.