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Isolated Intraorbital Frontosphenoidal Synostosis.

Paul A Mittermiller1, Kristen W Yeom2, Robert M Menard3

  • 1Division of Plastic and Reconstructive Surgery, Department of Surgery, Stanford Hospital and Clinics.

The Journal of Craniofacial Surgery
|October 26, 2017
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Summary
This summary is machine-generated.

This report details a rare case of intraorbital isolated frontosphenoidal synostosis, a condition causing cranial asymmetry. Early surgical and orthotic intervention led to a successful outcome for the infant patient.

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Area of Science:

  • Craniofacial Surgery
  • Pediatric Neurosurgery
  • Developmental Biology

Background:

  • Unilateral anterior plagiocephaly often results from deformational causes or unilateral coronal synostosis.
  • Other sutures, including the frontosphenoidal suture, can contribute to anterior cranial asymmetries.
  • Previous reports of isolated frontosphenoidal synostosis involved extraorbital fusion, unlike this case.

Observation:

  • A 3-month-old infant presented with left frontal flattening, supraorbital rim retrusion, and temporal bulging.
  • Computed tomography revealed isolated fusion of the intraorbital frontosphenoidal suture on the left.
  • The patient had no family history of cranial anomalies and negative genetic testing.

Findings:

  • This case represents the first documented instance of isolated frontosphenoidal synostosis exclusively within the intraorbital region.
  • The patient underwent successful surgical correction (fronto-orbital advancement and cranial vault remodeling) and orthotic therapy.
  • The treatment resulted in a satisfactory cosmetic and functional outcome.

Implications:

  • Highlights the importance of considering intraorbital frontosphenoidal synostosis in diagnosing anterior plagiocephaly.
  • Demonstrates the efficacy of surgical and orthotic management for this rare condition.
  • Contributes to the understanding of cranial suture development and anomalies.