Abstract

P 069

Massive edema of the optic nerve head combined with subretinal bleeding in one eye as first appearance of Arnold-Chiari-Malformation Type I in adolescence

Troeber L.¹, Löw U.¹, Jonescu-Cuypers C.¹, Haaß A.², Käsmann-Kellner B.¹, Seitz B.^1
1Department of Ophthalmology, University-Hospital Saarland, Homburg/Saar; ²Department of Neurology, University-Hospital Saarland, Homburg/Saar

Objective: The rare congenital condition of Arnold-Chiari Malformation Type I (ACM I) is characterized by herniation of the cerebellar tonsils into the upper cervical spinal tract. It is often related to syringomyelia and hydrocephalus. Various neurological manifestations occur in affected patients, such as headache, loss of sensitivity, ataxia and brain nerve paralysis.
Methods: A 59-year-old lady presented with reduced visual acuity of her right eye (OD) for 3 days. Ophthalmic history was empty. Because of burning neck pain since 25 years and vertigo, the patient underwent neurological examination one year before. Besides syringomyelia in her cervical spinal cord, which was interpreted as postinflammatory / posttraumatic condition, no additional pathology was found. Visual acuity was 0.1 fro OD and 1.2 OS (left eye). The optic nerve head (ONH) was extremely edematous in OD and vital but only slightly prominent in OS. OD showed massive subretinal and intraretinal bleedings around the ONH and in the central retina. A subarachnoidal bleeding and an intracranial tumor were excluded via computer tomography. Lumbar puncture showed a high pressure of the cerebrospinal fluid (29 mmHg). Cervical myelography presented a descent of the cerebellar tonsils, syringomyelia in C3 and C5/6, combined with hydromyelia in C2 to C7. Neurosurgeons performed posterior fossa decompression.
Conclusions: Although ONH edema and subretinal bleeding are not typical symptoms of ACM I, they might act as leading signs for this specific diagnosis. Terson-syndrome or paraneoplasies should also be considered as differential diagnosis. Cervical myelography confirms the diagnosis of ACM I.

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