If It’s Serous, Then It’s Serious
An 11-year-old Hispanic female presented with complaints of intermittent flashes of light in the inferior temporal visual field OD and a large dark spot surrounded by smaller spots. She noted that her symptoms were persistent while reading. She had no significant medical or ocular history, although a positive family history for type 2 diabetes was reported.
CLINICAL FINDINGS
On examination, the patient’s BCVA was 20/30 OD, 20/25 OS, and 20/25 OU. Refraction yielded -0.50 sphere (20/25) OD and -1.00 -0.25 x 099 (20/20) OS. Color vision was abnormal OD and normal OS. Confrontation fields showed temporal constriction OD and were full OS. Pupils and extraocular muscles were normal. IOP was 12 mm Hg OD and 13 mm Hg OS.
Posterior segment examination of the macula showed retinal changes consistent with early maculopathy OD. The cup-to-disc ratio was 0.15, with temporal gray-white depression evident in the optic nerve OD. Fundus photography showed temporal schisis-like changes confirmed on imaging OD. OCT highlighted retinal fluid accumulation with schisis extending from the optic pit to the macula OD. Photography and imaging findings can be seen in Figures 1-4.
DIAGNOSIS AND DISCUSSION
The patient was diagnosed with optic pit maculopathy OD. Differential diagnoses included X-linked juvenile retinoschisis and enhanced S-cone syndrome.
Optic disc pits are rare congenital anomalies that occur in approximately one in 11,000 individuals and often involve a temporal gray-white depression within the optic nerve head. These pits can lead to optic disc maculopathy, often presenting as serous macular detachment or schisis, which results in visual disturbances that can include blurred vision, flashes, and scotomas.
The exact mechanism of optic pit maculopathy is unclear, but fluid from the vitreous or cerebrospinal fluid (via the pit) can seep into the retina, causing retinal schisis and/or neurosensory detachment.
Most optic pit maculopathy cases are unilateral. Patient presentation may include decreased visual acuity, metamorphopsia, central or peripheral scotomas, and visual disturbances such as flashes and floaters.
Optic pit maculopathy can significantly affect a patient’s vision if left untreated. Studies show that 25% to 75% of patients with optic disc pits develop maculopathy over time.1
MANAGEMENT AND TREATMENT
The management of optic pit maculopathy requires a multidisciplinary approach, including comanagement with a retina specialist. Pediatric cases may spontaneously resolve without intervention. A 6-month observation period is warranted for stable cases, particularly in children.2 One study reported spontaneous regression of optic disc maculopathy in a 6-year-old patient within 6 months, with full anatomic and functional recovery.3
The most common surgical approach involves vitrectomy with or without internal limiting membrane peeling to eliminate traction. In cases of serous detachment, intraocular gas can help reposition the retina. Targeted laser treatment may help create a barrier against fluid migration from the optic pit to the macula.
One study found that 21% of optic pit maculopathy cases required surgical intervention, with a 76% success rate in achieving anatomic (dry fovea) and significant visual acuity improvement.3 Patients with subretinal fluid were more likely to worsen without treatment compared with patients with intraretinal fluid.3
Regular OCT imaging is essential to monitor macular changes, fluid progression, and recurrence.
PROMPT AND PRECISE
Optic pit maculopathy is a rare but potentially vision-threatening condition. Although observation may suffice in stable cases, surgical intervention remains the gold standard for progressive cases involving serous macular detachment. Regular monitoring and timely referral are key to preserving vision and optimizing patient outcomes.
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