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Undiagnosed Normocephalic Pancraniosynostosis Presenting as Bilateral Abducens Nerve Palsy
Marissa E. Baron, MPH1; Lucas A. Dvoracek, MD2; Michael Bykowski, MD2; Jesse A. Goldstein, MD2; Joseph E. Losee, MD2
1. University of Pittsburgh School of Medicine
2. University of Pittsburgh, Department of Plastic Surgery
University of Pittsburgh School of Medicine
2019-02-14
Presenter: Marissa Baron
Affidavit:
I certify that the material proposed for presentation in this abstract has not been published in any scientific journal or previously presented at a major meeting.
Director Name: Vu Nguyen, M.D.
Author Category: Medical Student
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial
INTRODUCTION: Normocephalic pancraniosynostosis is a rare variant defined as the premature fusion of more than three major cranial sutures with a grossly normal head shape and size. It typically presents with symptoms of increased intracranial pressure and is usually diagnosed significantly later than cases with abnormal head shape. Prompt treatment is necessary to prevent serious morbidity resulting from chronically elevated intracranial pressure. Here we present diagnosis and management of a patient presenting with this disorder and unique neurologic sequelae.
METHODS: A 9-year-old boy presented to the ophthalmologist with a 1-month history of double vision, drifting of his right eye toward the nasal bridge, and intracranial hypertension evident with papilledma. Physical exam was notable for mild turricephaly, trigonocephally, and schaphocephaly with occipital bulleting. A computed tomography study demonstrated radiologic thumbprinting, diffuse osseous sclerosis, and fusion of the bilateral coronal, sagittal, metopic, and lambdoid sutures. A cranial vault expansion with fronto-orbital advancement were performed.
RESULTS: Within 4 months of surgery, the papilledema resolved, and at 1-year follow up, the abducens nerve palsies showed improvement and the optic disks remained without evidence of papilledema. The patient had improvement in head shape.
CONCLUSION: Bilateral abducens nerve palsy is a rare presentation of increased intracranial pressure and a previously unreported presentation of pancraniosynostosis with hyptertension. Early diagnosis and treatment are imperative in cases of pancraniosynostosis with intracranial hypertension for the prevention of long-term sequelae.
