Hemifacial microsomia

Case contributed by Dr Mostafa Mahmoud El Feky

Presentation

Facial deformity since birth. Right ear deformity and deafness. CT examination is prior to mandible graft.

Patient Data

Age: 3
Gender: Female
CT

CT facial bones

The right ear shows complete absence of the structures derived from the first branchial appendage (complete abscence of external and middle ear), apart from anteriorly displaced dysmorphic auricle.

Hypoplastic right hemi-mandible and absence of right tempro-mandibular joint. Muscular atrophy and partial fat replacement of the related muscles of mastication. TThe mastoid process and body of the right zygoma are absent, developed frontal and maxillary processes. Bony graft is seen continuous with hypo-plastic right hemi-mandible 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 is receding in relation to maxillary counterpart. 

Cranio-facial asymmetry, manifested as asymmetrically large right side of the head.

CT

3D images

Hypo-plastic right hemi-mandible and absence of right tempro-mandibular joint. The mastoid process and body of the right zygoma are absent. The mandibular symphysis and alveolus is 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.

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Case information

rID: 55370
Case created: 31st Aug 2017
Last edited: 24th Sep 2017
Inclusion in quiz mode: Included
Institution: DAR ELASHAA center

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