Abstract

BACKGROUND: Intrinsic brainstem epidermoid cysts are rare, benign, slow growing lesions. Their eloquence preclude complete excision, however subtotal resection will often result in prolonged or sometimes permanent relief of presenting symptoms and signs. We describe an unusual case and review the literature of this pathology in the paediatric population. CASE DESCRIPTION: We report an intra-axial pontine epidermoid cyst in a 2-year-old girl who presented with developmental delay, multiple cranial nerve palsies and pneumonia. Magnetic resonance imaging (MRI) demonstrated an intrinsic pontine lesion with partial restricted diffusion and an enhancing plaque, the latter not typically seen in congenital lesions like epidermoid. However, gross surgical inspection and histopathology confirmed an epidermoid. CONCLUSION: Our case, supported by the literature, shows that brain stem epidermoid cysts may have atypical radiological characteristics, and that near total resection remains safe and can improve outcome.