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Woman reports 'sparks' during routine eye exam

Fundus exam of the left eye showed an inferotemporal excavation in the optic nerve head with a serous retinal detachment.

Issue: July 25, 2014

ByKristen E. Dunbar, MD

ByCaroline R. Baumal, MD

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A 19-year-old female college student was referred by an outside optometrist for retinal evaluation of her left eye. She initially presented for a routine annual exam with no visual complaints. On further questioning, she noted occasional “sparks” in her left eye when standing. She denied new floaters, spider webs or other visual symptoms. She reported no recent illnesses or history of trauma. She was born prematurely weighing 5.5 pounds and was placed on a ventilator in the neonatal intensive care unit for 3 weeks after birth. Her ocular history was significant for myopia and contact lens wear.

Examination

Best corrected visual acuity was 20/20-2 in the right eye and 20/30-2 in the left eye. Pupils were round without afferent pupillary defect or anisocoria in light or dark. Extraocular movements and IOP were normal. Slit lamp examination was unremarkable, and the posterior segment of the right eye was normal.

On fundus exam of the left eye, there was an inferotemporal excavation in the optic nerve head with adjacent peripapillary atrophy and serous detachment extending through the macula (Figures 1 and 2). Small yellow dots of exudate were visible temporal to the fovea. Optical coherence tomography demonstrated subretinal fluid detaching the fovea and adjacent intraretinal schisis. Multiple OCT line scans did not reveal a connection between the pit and detachment (Figure 3).

Figure 1. Fundus photo of the left eye.

Images: Dunbar K, Baumal C

Figure 2. Red free fundus photo of the left eye shows inferotemporal excavation with serous detachment.

Figure 3. OCT images through the optic pit and adjacent serous retinal detachment with overlying retinoschisis show no visualized connection between the inferotemporal pit and serous detachment.

What is your diagnosis?

Optic nerve pit

The differential diagnosis of a serous retinal detachment is extensive. Congenital causes include optic nerve pits, colobomas and morning glory anomalies. Given the location and appearance of the disc excavation and the lack of symptoms, this serous detachment was clearly associated with the congenital optic nerve pit seen on exam. Inflammatory causes of serous retinal detachments are posterior scleritis, Vogt-Koyanagi-Harada disease and sympathetic ophthalmia. Vascular abnormalities, such as malignant hypertension, disseminated intravascular coagulopathy, Coats’ disease and macular degeneration, can also lead to serous retinal detachments. Neoplastic etiologies include choroidal melanoma and other metastatic lesions.

Discussion

Optic nerve pits are small circular or oval excavations in the optic nerve head caused by incomplete closure of the superior edge of the embryonic fissure. Congenital pits of the optic nerve are often observed within 1 clock hour of the horizontal meridian, most commonly the inferotemporal side, while acquired pits are typically found within 1 clock hour of the vertical poles of the optic nerve. The rate of occurrence is one in 10,000 people, and the ratio of male patients to female patients is 1:1. Congenital optic nerve pits are bilateral in 10% to 15% of cases.

It is estimated that one-half to one-third of congenital optic nerve pits are associated with maculopathy, which classically is a serous retinal detachment. Larger and more temporal located optic pits are more at risk. The pathophysiology of the development of serous detachment is unclear; however, it is hypothesized that the subretinal fluid originates from either the vitreous cavity or the subarachnoid space.

The treatment for optic pit maculopathy is controversial and includes observation, juxtapapillary laser photocoagulation, pars plana vitrectomy (PPV) with internal gas tamponade that may be combined with internal limiting membrane (ILM) peeling, or scleral buckling. Juxtapapillary laser is performed by placing one to three rows of laser spots in a peripapillary distribution temporal to the optic nerve extending superior and inferior to the subretinal fluid. It is hypothesized that the chorioretinal adhesion produced by laser may block the passage of fluid from the optic pit to the inner retinal schisis cavity and subretinal space. Alternatively, complete separation of the posterior hyaloids induced by PPV is thought to relieve vitreous traction and may be combined with ILM peeling to help fully remove all tractional components. Scleral buckling converts the hyaloid traction from an inward to outward vector, thus relieving traction and promoting reattachment of the macula.

Figure 4. OCT shows slightly improved subretinal fluid and retinoschisis.

There are multiple reports of the efficacy of laser photocoagulation in serous detachment in optic pits. Annesley and colleagues reported the results of five patients with serous detachment and optic nerve pit who were treated with laser photocoagulation. The subretinal fluid resolved after laser in four out of five eyes. A recent review of 20 patients treated with laser, laser with intravitreal gas injection, or PPV with or without ILM peeling revealed variable outcomes. Of six patients treated with laser alone, there was a 33% success rate. In 11 patients treated with laser and intravitreal gas injection, there was a 72% success rate. Of eight patients treated with PPV, posterior hyaloid dissection, laser and gas tamponade, there was a higher success rate of 87% without recurrence of subretinal fluid, and five of these patients had ILM peeling during PPV. This study suggests that while all treatments appear to have some degree of efficacy, PPV with or without membrane peel may have the highest anatomical success rate and least likelihood of recurrence in eyes, especially when other treatments fail to lead to resolution.

Given our patient’s lack of symptoms and age, and after a discussion of the various treatment options, the decision was made to perform juxtapapillary laser. At 2 months, visual acuity had improved to 20/25- in the left eye, while OCT showed slightly improved subretinal fluid and schisis (Figure 4).