Massive Wormian bone at the cranial apex: identification, correction and outcome
Thomas D Samson1, Stephen P Beals, Harold L Rekate
1Division of Plastic Surgery, Penn State Milton S. Hershey Medical Center, 500 University Drive, Hershey, PA 17033, USA. tsamson@hmc.pdu.edu
The Journal of Craniofacial Surgery
|January 25, 2008
Summary
A rare case of a congenital epidermoid cyst within a large Wormian bone at the cranial apex is presented. Surgical removal was successful, with the infant achieving normal developmental milestones.
Area of Science:
- Pediatric Neurosurgery
- Craniofacial Surgery
- Developmental Pediatrics
Background:
- Congenital skull abnormalities can present with diverse clinical manifestations.
- Wormian bones, or ossicula suturalia, are accessory bones within the cranial sutures.
- Epidermoid cysts are the most common congenital tumors of the central nervous system.
Observation:
- A three-month-old infant presented with abnormal head shape and overlying alopecia areata.
- Intraoperatively, a large Wormian bone (7cm x 8cm) was identified at the cranial apex, involving the coronal and sagittal sutures.
- An intracranial epidermoid cyst was found centrally within the Wormian bone, projecting between the cerebral hemispheres.
Findings:
- The Wormian bone obliterated the anterior fontanelle.
- The epidermoid cyst was successfully excised along with the Wormian bone.
- Subtotal cranial vault reconstruction was performed at ten months of age.
Implications:
- This case highlights a rare association of a large Wormian bone with an intracranial epidermoid cyst.
- Early surgical intervention for such congenital anomalies is crucial for favorable neurodevelopmental outcomes.
- Successful management ensures the achievement of developmental milestones in affected children.
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