Unilateral isolated frontosphenoidal craniosynostosis causing frontal plagiocephaly
Damian D Marucci1, Barry M Jones, David J Dunaway
1Craniofacial Unit, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK.
Journal of Plastic, Reconstructive & Aesthetic Surgery : JPRAS
|January 22, 2008
Summary
Unilateral isolated frontosphenoidal synostosis is a rare cause of frontal plagiocephaly that can mimic unicoronal synostosis. Early and accurate diagnosis through careful assessment is crucial for appropriate management of this condition.
Area of Science:
- Craniofacial Surgery
- Pediatric Neurosurgery
- Developmental Biology
Background:
- Synostotic frontal plagiocephaly typically results from frontoparietal (unicoronal) synostosis.
- Other coronal hemiring fusions can also cause frontal plagiocephaly, though less commonly.
- Frontosphenoidal synostosis is an exceptionally rare cause, with limited prior case reports.
Observation:
- An 8-month-old male infant presented with frontal plagiocephaly.
- Clinical and radiological findings suggested unilateral isolated frontosphenoidal synostosis.
- The presentation mimicked more common frontoparietal (unicoronal) synostosis.
Findings:
- The infant exhibited ipsilateral forehead flattening and maxillary retrusion.
- A midline chin and contralateral deviation of the nasal root and endocranial base were noted.
- Anomalies of the bony orbit were also identified.
Implications:
- Unilateral isolated frontosphenoidal synostosis can be misdiagnosed as unicoronal synostosis.
- Distinguishing between these conditions is vital for effective treatment planning.
- Thorough physical and radiological evaluation is essential for accurate diagnosis of coronal hemiring synostotic disorders.
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