Choroidal Neovascular Membrane in Chronic Papilledema

A 21-year-old woman with papilledema from idiopathic intracranial hypertension developed a peripapillary choroidal neovascular membrane (PCNVM) complicating untreated chronic papilledema 10 years later.

Choroidal Neovascular Membrane in Chronic Papilledema George Alencastro, MD Valérie Biousse, MD A 21-year-old woman was seen for blurred vision and chronic papilledema Her examination showed: • Best-corrected visual acuity: 20/20 OU • Pupils: No relative afferent pupillary defect (RAPD) • Color vision: 14/14 correct Ishihara plates OU Figure 1a and 1b: Color photographs of the posterior poles of both eyes showing bilateral optic disc edema suggestive of papilledema from intracranial hypertension Right eye Left eye Figure 1a Figure 1b Brain MRI/MRV and lumbar puncture confirmed the diagnosis of idiopathic intracranial hypertension (IIH). Visual fields were full OU with only mild enlargement of the blind spots. She was treated with acetazolamide and weight loss. Her symptoms improved and she was lost follow up. She presented 2 years later with recurrence of the symptoms. Examination was unchanged, with best corrected visual acuity 20/20 OU, no RAPD and full color plates Figure 2a and 2b: Color photographs of the posterior poles of both eyes showing persistent bilateral papilledema two years after initial presentation Right eye Figure 2a Left eye Figure 2b Figure 3a: 24-2 Humphrey visual field SITA-Fast OS showing mild constriction and inferior nasal step Figure 3b: 24-2 Humphrey visual field SITA-Fast OD showing non-specific mild depression Left eye Right eye Figure 3a Figure 3b She was restarted on acetazolamide but was lost to follow up again and returned 8 years later for decreased vision OD. Examination 10 years after first discovery of papilledema: • Best-corrected visual acuity: 20/60 OD and 20/25 OS • Pupils: No relative afferent pupillary defect (RAPD) • Color vision: 14/14 correct Ishihara color plates OU Figure 4a: 24-2 HVF SITA-Fast OS showing enlargement of blind spot and an inferior nasal step Figure 4b: 24-2 HVF SITA-Fast OD showing enlargement of blind spot and depression with nasal step Left eye Right eye Figure 4a Figure 4b Wide field fundus photographs OU Figure 5a (right eye): disc edema with peripapillary changes Figure 5b (left eye): disc edema Figure 5a Figure 5b Figure 6: Color fundus photographs OU Right eye Left eye Figure 6a: There is severe optic disc edema associated with peripapillary yellowish elevation temporal to the disc (arrows) Right eye Figure 6b: There is persistent optic disc edema with peripapillary wrinkles (arrows) Left eye Figure 6a: Choroidal neovascular membrane Extensive subretinal fluid in posterior pole Right eye Retinal folds Optical coherence tomography (OCT) of the peripapillary retinal nerve fiber layer (pRNFL) showed increased thickness of the pRNFL in both eyes Figure 7 Figure 6b Figure 8a: EDI-OCT of the right eye showing the peripapillary choroidal neovascular membrane with optic disc edema Figure 8b: EDI-OCT of the left optic nerve showing optic disc edema Right eye Figure 8a Figure 8b Left eye Figure 9: EDI-OCT of the right eye showing optic disc edema with peripapillary choroidal neovascular membrane type 2, associated cystic changes of the retina and superficial retinal folds Retinal Folds Cystic retinal changes Choroidal neovascular membrane complex Figure 9 Peripapillary choroidal neovascular membrane (PCNVM) complicating chronic papilledema Is a rare manifestation of persistent papilledema from idiopathic intracranial hypertension Reported incidence of 0.53% The disruption of the axoplasmic flow due to chronic intracranial hypertension leads to chronic optic disc swelling The pressure effect of the disc swelling disrupts and deforms adjacent chorioretinal layers causing a break in Bruch’s membrane and induces hypoxia Chronic hypoxia leads to subsequent growth of vessels from the choriocapillaris through the break in Bruch’s membrane, resulting in PCNVM