Presentation
Facial deformities with history of pentalogy of Fallot.
Patient Data
Facial asymmetry with underdeveloped right hemifacial bony and soft tissue structures as follows:
Osseous:
small-sized, hypoplastic right mandibular condyle with foreshortened and underdeveloped right mandibular ramus and mild subsequent ipsilateral deviation of the chin to the right side
relatively small-sized right maxilla and aplastic pterygoid plate
Soft Tissue:
small-sized right masseter, temporalis, and pterygoid muscles, as well as the overlying subcutaneous soft tissue
Auditory:
right microtia with aplastic external auditory canal and middle ear cleft, as well as mastoid air cells
Additional Finding:
right cerebellopontine angle arachnoid cyst
Case Discussion
This case presents multiple radiological features of hemifacial microsomia, a condition characterized by hypoplasia of structures derived from the first and second branchial arches.
It is associated with extracranial anomalies in 55% of cases, as seen in this patient who has pentalogy of Fallot.
The main differential diagnosis is Treacher-Collins syndrome, which is characterized by bilateral involvement.
Another differential is Parry-Romberg syndrome, which is characterized by CNS and orbital manifestation and usually spares the ear structures.
Unable to process the form. Check for errors and try again.