Presentation
Progressive facial asymmetry started years ago. Previous surgical treatment for cleft lip.
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
- small hypoplastic right zygomatic arch
- small sized right maxilla, maxillary antrum and right pterygoid plates
-
Soft tissue
- small sized right masseter, temporalis and pterygoid muscles as well as the overlying subcutaneous soft tissue
- small sized right parotid gland
- right hemimicroglossia
Small bony cleft is seen extending through the right alveolar process between the maxillary central incisor teeth. No evidence of nasal extension. Malaligned right central incisor which is anteriorly displaced.
The axial CT brain cuts revealed right deep parietal/basal ganglia area of encephalomalacia (chronic infraction).
Case Discussion
In that case the patient had progressive facial asymmetry which started during childhood.
The differential diagnosis should include:
- hemifacial microsomia but this is congenital and essentially non-progressive condition (case)
- post-traumatic atrophy and partial lipodystrophy (Barraquer-Simon Syndrome) are also included in the differential diagnosis. However, partial lipodystrophy is usually bilateral and involves primarily the adipose tissue
- linear scleroderma (“en coup de saber”)
- Rasmussen encephalitis (RE)
Parry-Romberg syndrome (PRS), also known as progressive facial hemiatrophy (PFH), is a rare progressive craniofacial disorder (phakomatosis).
It is classically characterized by a slow progressive degeneration (atrophy) of the soft tissues of half of the face (hemifacial atrophy). This involves the skin, underlying soft tissues, cartilaginous structures and bones.
Interestingly, evidence of the disease dates back to ancient Egypt with mummies exhibiting craniofacial dysmorphism consistent with PHA 1.
PHA is associated with multiple extracutaneous findings, of which neurologic complications are the most common. Seizures and headaches are the most common neurologic symptoms in patients with PHA 1.
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