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Association between "plagiocephaly" and hemifacial microsomia
B L Padwa1, R J Bruneteau, J B Mulliken
1Craniofacial Centre, Children's Hospital, Harvard Medical School, Boston, MA 02115.
American Journal of Medical Genetics
|December 1, 1993
Summary
Frontal plagiocephaly in hemifacial microsomia patients is usually deformational, not synostotic. This finding suggests a link between these conditions and associated anomalies, challenging rigid diagnostic labels.
Area of Science:
- Craniofacial Surgery
- Pediatric Plastic Surgery
- Developmental Biology
Background:
- Hemifacial microsomia (HFM) is a congenital condition affecting facial development.
- Frontal plagiocephaly, a flattening of the forehead, can occur in HFM patients.
- Differentiating deformational plagiocephaly from synostotic plagiocephaly is crucial for appropriate management.
Purpose of the Study:
- To investigate the etiology of frontal plagiocephaly in patients with hemifacial microsomia.
- To determine if frontal plagiocephaly in HFM is typically deformational or due to unilateral coronal synostosis.
- To identify associated anomalies in HFM patients with frontal plagiocephaly.
Main Methods:
- Clinical examination of 155 patients with HFM, focusing on 15 who presented with frontal plagiocephaly.
- Assessment of supraorbital rims, nasal root, ears, malar eminences, chin point, and palpebral fissure height to categorize plagiocephaly.
- Documentation of extracranial anomalies and associated conditions like torticollis and cervical spine abnormalities.
Main Results:
- 93% of HFM patients with frontal plagiocephaly exhibited deformational abnormalities.
- Only one patient (7%) showed signs suggestive of unilateral coronal synostosis, which was not radiographically confirmed.
- Frontal deformational plagiocephaly was ipsilateral to the affected side in most HFM cases and often associated with torticollis and other anomalies.
Conclusions:
- Frontal plagiocephaly in hemifacial microsomia is predominantly deformational in origin.
- The findings challenge a strict etiological classification of deformational versus malformative anomalies in HFM.
- Associated conditions like torticollis and extracranial anomalies are common in HFM with deformational frontal plagiocephaly.