Acquired choroidal folds: a sign of idiopathic intracranial hypertension

Choroidal folds may be caused by several underlying ocular and orbital diseases; they are classified as idiopathic when no obvious cause is found. The objective of this study was to determine whether acquired choroidal folds are associated with idiopathic intracranial hypertension.

In this observational case series, three patients with normal fundus examination later developed choroidal folds. They underwent complete ocular examination, B-scan ultrasonography, fluorescein angiography, optic coherent tomography (OCT) and magnetic resonance imaging. A neurological consultation, lumbar puncture and measurement of opening pressure of cerebrospinal fluid (CSF) were also obtained for all patients.

Three patients with previously normal ocular fundus developed choroidal folds, and optic nerve subarachnoid space enlargement was seen on B-scan. Clinical and radiological evaluations identified elevation of intracranial pressure, and biochemical analysis of CSF was normal, suggesting a diagnosis of pseudotumour cerebri, which was later confirmed. The first patient developed choroidal folds in one eye first, and 4 years later in the fellow eye. The second patient presented with bilateral optic disk swelling secondary to intracranial hypertension. After treatment, which lowered CSF pressure, bilateral choroidal folds were identified by OCT, fluorescein angiography and ocular fundus photograph. The third patient also developed choroidal folds in one eye, but his fellow eye was difficult to evaluate due to a chorioretinitis scar on the macula. OCT identified choroidal folds in all three patients and in the second patient, it also revealed retinal nerve fiber layer damage.

Acquired choroidal folds and optic nerve subarachnoid space enlargement may be signs of idiopathic intracranial pressure elevation. Nevertheless, this diagnosis should be confirmed by lumbar puncture. Comprehensive imaging studies should be performed to rule out expanding tumors. The lower CSF pressure in our first two patients suggests that choroidal folds or optic disk swelling may depend on the level of intracranial pressure and may be points in a continuum of clinical presentations.