Case Report of Optic Atrophy in Pansynostosis: An Unusual Presentation of Scalp Edema from Hair Braiding

Abstract

Pansynostosis (fusion of all cranial sutures) and optic atrophy were found as incidental CT scan and ophthalmological findings in an 8-year-old who presented to the emergency room with scalp edema from tight ‘cornrow’ hair braiding. Cranial vault expansion was successfully performed. Ophthalmological problems have stabilized but have not reversed. Late presentation of craniosynostosis and the pathophysiology of secondary optic atrophy are discussed.

Delayed-Onset Craniosynostosis

“…In 1984, we reported an infant with sagittal craniosynostosis probably due to suture fixation by a calcified subperiostal hematoma [4] . In this patient, histological findings of normal suture tissue beyond the limits of the hematoma tended to support our view, although it might also be that the calcified hematoma brought our attention to a preexisting condition, as the authors comment on the role of the scalp edema in their patient [1] .…”

“…I read with great interest the paper by O'Hara et al [1] . I would like to refer to previous work from our hospital that might be of interest to the journal's readers.…”

“…O'Hara et al [1] also discuss the role of fetal head constraint in the etiology of some forms of craniosynostosis. We quite agree with their view and have contributed to the knowledge of this occurrence in cas-…”

Surgery: Modified Pi with Triple-Bonnet Flap and Fronto-Orbital Advancement

“…Pansynostosis is a rare form of craniosynostosis that involves premature fusion of the cranial sutures (coronal, sagittal, metopic, and occipital) and, particularly in cases of late presentation, there are heightened clinical concerns, both functional and aesthetic. In untreated cases of pansynostosis and increased intracranial pressure (ICP), damage to the optic nerve progresses to optic atrophy and then blindness [2] .…”

“…In the patients with a mean age of 3.1 years (range 1-6 years), the optic atrophy was irreversible. He suggested that when there is increased ICP from craniosynostosis, patients have a poor prognosis [2] .…”