We read the recent article “Chorioretinal Folds: Associated Disorders and a Related Maculopathy,” by Olsen and associates, with great interest. The authors describe a large case series of patients with choroidal folds and the related ocular and extraocular diseases. Interestingly, only 15% of cases were considered idiopathic, a label attributed when no apparent cause for choroidal fold development is detectable. Their results confirm previous data and the authors aptly propose that with improved diagnostic testing, the patients showing choroidal folds and referred to as idiopathic represent a smaller portion of the total.
Moreover, the theory proposed by Friberg suggesting that previous undiagnosed episodes of posterior scleritis could lead to persisting choroidal folds seems to be confirmed by the results from their study as well. Here we add that different reports have linked choroidal folds with intracranial hypertension, which has therefore been recognized as the underlying mechanism in some patients with what appears to be the well-recognized condition of idiopathic acquired hyperopia with choroidal folds. Interestingly, papilledema may not be present at the time of evaluation and choroidal folds may be the only sign reflecting increased intracranial pressure. Sharma and associates described a patient with idiopathic intracranial hypertension and unilateral choroidal folds followed 3 months later by bilateral papilledema. Griebel and Kosmorsky found increased intracranial pressure by performing lumbar punctures in 5 patients with choroidal folds without papilledema who were considered as having idiopathic choroidal folds after ultrasonography of the orbit, intravenous fluorescein angiography, and imaging studies of the head. As a consequence, an undiagnosed intracranial hypertension could further reduce the chance of choroidal folds being idiopathic.
Therefore, we agree with those authors who suggest that performing a lumbar puncture should be considered in patients presenting with choroidal folds without papilledema and no other apparent causes, especially if imaging is unrevealing, to exclude the possibility of raised intracranial pressure. This is not a criticism of Olsen and associates, since they performed a retrospective study based on the medical record review of patients investigated for the presence of choroidal folds. Our comments corroborate the idea that an appropriate ocular and systemic evaluation is needed once choroidal folds are recognized, as only a small number of cases are idiopathic. The diagnosis of the underlying disorder may be somewhat challenging and often requires a number of imaging studies and investigations, including a lumbar puncture in selected cases.