A healthy 26-year-old male was referred to Illinois Retina Associates for macular edema in his right eye. He has mild myopia, but was otherwise healthy and took no medications. He noticed visual distortion in his right eye for two months.
He received three bevacizumab (Avastin) injections with no improvement of his symptoms but an outside retina specialist.
Posterior Segment Exam
On examination, visual acuity was 20/20 OU, but the patient complained of metamorhpopsia OD. An optic nerve pit was identified along the inferotemporal rim (Figure 1) of the optic nerve head. There was a serious retinal detachment with subretinal exudates extending from the optic nerve towards the macula (Figure 1).
A macular spectral domain optical coherence tomography scan (OCT) showed intraretinal and subretinal fluid in the macular area (Figure 2). Fluorescein angiogram (FA) revealed extensive leakage in the macula (Figure 3).
- Optic nerve pit associated maculopathy
- Central serous chorioretinopathy
- Idiopathic juxtapapillary choroidal neovascularisation
- Myopic degeneration with choroidal neovascularisation
- Uveal effusion syndrome
Optic Nerve Pit
Optic nerve pits are small congenital optic nerve cavities. They are rare, occurring in less than 1/10,000 patients, with 15% of cases being bilateral. The main complication of optic disease pits is optic disc pit maculopathy (macular edema and subretinal fluid), occurring in up to 2/3 of patients, and presenting in early adulthood. The typical presentation consists of schisis-like cavities in the outer retina, and can also be associated with subretinal fluid. In a minority of cases there can be spontaneous improvement, however, poor visual outcomes have been described in untreated patients.
There are a few hypotheses attempting to explain the origin of the macular fluid:
- Fluid from the vitreous cavity
- Cerebrospinal fluid from the subarachnoid space
- Leaky blood vessels at the pit base
- Fluid from the orbital space surrounding the optic nerve dura
- Component of vitreoretinal transaction
A number of treatments, performed either alone, or in combination with vitrectomy, have been described, including:
- Peripapillary laser, in an attempt to limit passage of fluid into the macula
- Intravitreal gas injection, which could promote fluid resorption through tamponade
- Pars plana vitrectomy, thought to relieve traction from the vitreoretinal interface
- Closing the pit with tissue glue or atuologous platelet concentrate during vitrectomy, thus preventing intraocular and/or subarachnoid fluid from entering the pit
- Internal limiting membrane peeling
- Unroofing the pit or creating inner retinal fenestrations, to equalize pressure differential between inside the eye and the subarachnoid space and avoid fluid from being pushed into the retina.
- Macular buckle surgery
Modern vitrectomy procedures have been good long term outcomes and result in visual improvement in a majority of patients.
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