Ocular manifestation in Arnold Chiari I syndrome

Author: Dhouha Gouider (Tunisia)

Co-authors: Rim Bouraoui, Khaled El Matri, Racem Choura, Rahma Saidane, Marwa Masmoudi, Laila El Matri

Purpose

To present clinical ocular manifestations related to Chiari I malformation through two clinical cases.

Setting/Venue

Ophthalmology Department B, Hedi Raies Institute

Methods

Two case reports having ocular manifestations related to Chiari I

Results

Case 1: A 40-year-old patient with no medical history presented an acute loss of vision in both eyes. On examination, the patient had a visual acuity limited to 5/63. Biomiscroscopic examination of the anterior segment was without abnormalities. Fundus examination revealed bilateral papilledema. The visual field test showed a borderline diffuse loss in both eyes. Case 2: A 17-year-old patient with no medical history presented intermittent headaches and a progressive loss of vision in both eyes. On examination, the patient had a visual acuity limited to 5/80. Biomiscroscopic examination of the anterior segment was without abnormalities. Fundus examination revealed papillary pallor in both eyes. The visual field test showed nasal and temporal scotomas in both eyes. A significant reduction of the RNFL thickness was observed in the superior, inferior, and temporal quadrants of both eyes. A brain MRI revealed a Chiari type 1 malformation without associated hydrocephalus in both patients. They were addressed to the neurosurgery department.

Conlusions

Chiari I malformation is a rare congenital condition, classically defined as a caudal displacement of the cerebellar tonsils through the foramen magnum into the spinal cord. Cervical pain and occipital headache are the most characteristic symptoms. Oculomotor paralysis and nystagmus are the main ocular signs. Papilledema remains a rare ocular manifestation. OCT of the optic nerve head may further be important for the follow-up of nonsurgical patients to monitor the chronic fiber suffering and axon cell death through the thinning of the RNFL in a long-term follow-up time.

Financial Disclosure

None