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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.
Abstract:
Fifteen of 155 patients with hemifacial microsomia were noted to have frontal plagiocephaly. These patients were examined to determine whether the frontal flattening was either secondary to deformation, the result of unilateral coronal synostosis, or part of the spectrum of hemifacial microsomia. The patients were categorized as having deformational versus synostotic frontal plagiocephaly by documenting position of the supraorbital rims, nasal root, ears, malar eminences, chin point, and the palpebral fissure height. Other extracraniofacial anomalies were also noted. Fourteen of 15 (93%) patients had characteristic deformational abnormalities. Only 1/15 (7%) had an elevated orbit, suggestive of unilateral coronal synostosis, but this diagnosis was not radiographically confirmed. Frontal deformational plagiocephaly was ipsilateral to the side predominantly affected by hemifacial microsomia in all but one patient. Patients with hemifacial microsomia-deformational frontal plagiocephaly often had ipsilateral torticollis, cervical spine abnormalities, and anomalies outside the craniofacial region. This was in contrast to patients with deformational frontal plagiocephaly, in the absence of hemifacial microsomia, who frequently had ipsilateral torticollis but no other anomalies. This study also underscores possible confusion in differentiating hemifacial microsomia from deformational hemifacial hypoplasia on physical examination. The association of deformational frontal plagiocephaly and hemifacial microsomia belies a rigid etiologic label of deformational versus malformative anomaly.
Insights
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.