Ophthalmic Presentation of Diffuse Intrinsic Pontine Glioma in Children (Case Series and Literature Review).

Abstract

Diffuse intrinsic pontine glioma (DIPG) is a rare aggressive brainstem lesion, affecting mainly young children. This report describes sixth cranial nerve palsy as the initial ophthalmic presentation in children with this pathology. Case series and literature review. All children presented with sixth nerve palsy were consecutively recruited from the pediatric clinic at the East Sussex NHS Healthcare Trust within the last 10 years. Full ophthalmic examination, orthoptic assessment, and refraction check were done in three patients. Magnetic Resonance Imaging was carried out using 1.5 Tesla (Siemens Symphony, Erlangen, Germany) to establish the diagnosis. The patients' age ranged from 5 to 14 years at the time of presentation. All presented with sudden onset esotropia and limited abduction, suggestive of presence of sixth nerve palsy, requiring urgent medical attention. On detailed questioning and assessment, all children showed various neurological symptoms including nystagmus, liquid dysphagia, balance problems, and nocturnal enuresis. Two out of three patients died within 7 months following diagnosis. Sudden onset esotropia, especially due to sixth nerve palsy in children, should be considered a red flag symptom, prompting proper urgent specialist assessment. Sixth nerve palsy in patients with DIPG was associated with severely reduced life expectancy in this case series of three patients, shorter than in reported non-ophthalmic presentations.