Abstract

Arnold-Chiari malformations (ACM) is a rare congenital hindbrain maldevelopment, leading to downward herniation of the cerebellar tonsils. Clinical features relates to cerebrospinal fluid disturbances, manifesting as symptoms of headaches, pseudotumour-like episodes, cranial nerve palsies and cerebellar dysfunction. Ocular manifestations includes varying ophthalmoloplegia and accommodation abnormalities. Papilloedema has been rarely implicated and remains an uncommon feature of ACM. We report a case of ACM who developed papilloedema and visual disturbance, that was successfully treated with suboccipital decompression. The presentation of patients with ACM-I and papilloedema unaccompanied by localising signs may resemble that of IIH. Neuroimaging with special attention to the craniocervical junction in saggital and transverse planes is crucial. Surgical decompression of the posterior fossa seems to improve headache symptoms and clinical signs of papilloedema.