Bilateral Squamosal Suture Craniosynostosis Presenting with Abducens Nerve Palsy and Severe Papilledema.

2020 
Abstract Background Patients with single-suture or minor suture craniosynostosis are typically asymptomatic at early presentation; intervention is aimed at reducing the risk of elevated intracranial pressure and associated developmental sequelae. Patients may be symptomatic in cases of major multi-suture syndromic synostoses or delayed diagnosis. Clinical presentation in this context may include headaches, papilledema, cognitive delay, or behavioral issues. Cranial nerve palsies are atypical symptoms of intracranial hypertension in this patient population. Case Description An 11-month-old otherwise healthy female infant presented with bilateral severe papilledema and left abducens nerve palsy due to non-syndromic near complete bilateral squamosal suture synostosis with associated incomplete sagittal and right lambdoid synostoses. The patient underwent urgent open cranial expansion, with resolution of her papilledema and improvement in eye position and motility. Conclusions Cranial nerve palsies may be presenting symptoms of intracranial hypertension in patients with craniosynostosis. Multi-disciplinary evaluation and treatment is paramount for appropriate management.
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