This 14YO presented with mild bilateral optic nerve edema and enlarged blind spots. Evaluation of the right nerve was difficult due to the myelinated nerve fiber layer.
There are prominent circumferential retinal lines (Paton’s folds) surrounding the left nerve.
MRI and MRV scanning were normal, and lumbar puncture opening pressure was elevated at 26mm H2O. The patient was diagnosed with idiopathic intracranial hypertension (pseudotumor cerebri).
Learning Points:
In 1911, Paton and Holmes established that papilledema was due to elevated intracranial pressure rather than inflammation, as previously thought (Brain 1911;33:389-432).
They also noted that nerve edema was associated with “a series of folds which run concentric with the edge of the disc,” which they felt represented retinal folds.
Sibony and Kupersmith, correlating OCT with fundus photography, found that these folds represented a variety of structural stresses on the nerve and peripapillary tissues, including peripapillary wrinkles, inner retinal folds, choroidal folds, and outer retinal folds/creases (Ophthalmology 2016;123:1397-1399).
