Craniosynostosis refers to early closure of a skull suture. This may occur with just one, or multiple, sutures. Early closure results in lack of ability of brain and skull expansion during infancy and childhood. Although head or facial deformity is often seen, it is not always present, making diagnosis difficult. Premature suture closure can result in increased intracranial pressure, requiring surgical skull expansion. The purpose of this poster is to increase awareness of craniosynostosis as a cause of childhood papilledema.
A 6 year-old boy presents with elevated optic discs, suspicious for subtle papilledema. He has a history of lymphoma, but chemotherapy was discontinued due to presumed side effects of headache and lethargy. Urgent MRI and MRA found apparent significant venous sinus thrombosis, but his symptoms worsened despite treatment. The venous anomaly and increased intracranial pressure were ultimately determined to be secondary to multiple craniosynostosis. Skull vault expansion resulted in resolution of symptoms.
Regardless of the age of the patient, papilledema, even when subtle, is always worrisome for possible intracranial mass or venous sinus thrombosis. However, we must be aware of other potential pathological causes of increased intracranial pressure in childhood. Craniosynostosis often occurs between the ages of 2-6 years, but should be considered in cases of papilledema up to age 10. Traditional neuro-imaging may not detect craniosynostosis; head CT with 3-D reconstruction is the preferred imaging method to make the diagnosis.