We appreciate the excellent observation from Musetti and associates regarding the association of increased intracranial hypertension and chorioretinal folds (CRFs). We also agree with the need for further consideration of increased intracranial pressure as a cause of CRFs that our series did not report.
To summarize these case series, Greibel and associates reported 6 patients with CRFs and no papilledema. Four presented with a hyperopic shift. The other 2 had “subjective complaints localizing to the affected eye.” The authors postulated that the retrolaminar optic nerve sheath may swell and cause indentation of the posterior globe without concomitant optic nerve head swelling. Sharma and associates reported a single case with a hyperopic shift and CRFs. This patient presented with bifrontal headaches and, the authors suggest, “distention of the optic nerve subarachnoid space with posterior segment flattening and chorioretinal folds.” Ten months later, the patient had bilateral disc edema and headaches. Finally, Taban and associates reported a unilateral case of CRFs with no papilledema, who was lost to follow-up. After 2 years, the patient was noted to have a relative afferent pupillary defect, papilledema, and temporal disc pallor, and a parasellar meningioma was detected on magnetic resonance imaging.
Musetti and associates raise an important issue regarding suspicion that increased intracranial pressure may lead to idiopathic CRFs in the absence of papilledema. More specifically, one should consider obtaining central imaging or a lumbar puncture in cases of idiopathic CRFs. Based on the referenced literature, the clinician should be particularly alert when there has been a recent hyperopic shift, headache, or enlargement of the optic nerve with ultrasound. Although we have outlined the various etiologies in our study, we have not mentioned cases that result from an increased intracranial pressure. We inquired about CRFs with the neuro-ophthalmology services at both the University of Minnesota and Emory. To summarize, we found remarkable consistency in their responses. At the University of Minnesota, only 8 of 226 patients (4%) with idiopathic intracranial hypertension (IIH) had CRFs. There was only 1 patient with CRFs and no optic disc edema (personal communication, April 16, 2014, Michael Lee, MD, Professor of Ophthalmology). At Emory, an estimate of <5% of all IIH had CRFs; many of these were classified as Paton’s lines or radiating, circumferential folds that originate around a swollen optic nerve (personal communication, April 16, 2014, Nancy Newman, MD, and Valérie Biousse, MD, Professors of Ophthalmology). Furthermore, our neuro-ophthalmology colleagues indicated that they would rarely consider referring such patients to the retina service. Therefore, we believe that we may be subject to a referral bias in our study. Patients with headaches, recent hyperopic shift, or neurologic symptoms may be referred outside of the retina clinic and more appropriately referred to the neuro-ophthalmology service.
We thank Musetti and associates for pointing out this important consideration for the evaluation and management of individuals with idiopathic CRFs. For completeness, we have reviewed the folds imaged in these studies. Of the images presented, all have linear folds (parallel with the vascular arcades). Given that most had a hyperopic shift, such eyes should clearly be considered for central imaging and a lumbar puncture.