Posterior ischemic optic neuropathy (PION) is a medical condition characterized by damage to the retrobulbar portion of the optic nerve due to inadequate blood flow (ischemia) to the optic nerve. Despite the term posterior, this form of damage to the eye's optic nerve due to poor blood flow also includes cases where the cause of inadequate blood flow to the nerve is anterior, as the condition describes a particular mechanism of visual loss as much as the location of damage in the optic nerve. In contrast, anterior ischemic optic neuropathy (AION) is distinguished from PION by the fact that AION occurs spontaneously and on one side in affected individuals with predisposing anatomic or cardiovascular risk factors.
Signs and symptoms
PION is characterized by moderate to severe painless vision loss of abrupt onset. One or both eyes may be affected and color vision is typically impaired.
Ophthalmoscopic exam
Looking inside the person's eyes at the time of onset, ophthalmoscope exam reveals no visible changes to the optic nerve head. Weeks after ischemic insult, nerve atrophy originating from the damaged posterior optic nerve progresses to involve the anterior optic nerve head. Four to eight weeks after onset, atrophy of the optic nerve head is observable upon ophthalmoscope exam.
Pupils
If both eyes are affected by PION, the pupils may look symmetrical. However, if the eyes are asymmetrically affected, i.e. one eye's optic nerve is more damaged than the other, it will produce an important sign called an afferent pupillary defect.
Defective light perception in one eye causes an asymmetrical pupillary constriction reflex called the afferent pupillary defect (APD).
Arteritic PION
A-PION most commonly affects Caucasian women, with an average age of 73. At onset vision loss is unilateral, but without treatment it rapidly progresses to involve both eyes. Vision loss is usually severe, ranging from counting fingers to no light perception. Associated symptoms are jaw pain exacerbated by chewing, scalp tenderness, shoulder and hip pain, headache and fatigue.
Cause
PION is a watershed infarction of the optic nerve that may cause either unilateral or, more often, bilateral blindness. PION typically occurs in two categories of people:
- People who have undergone non-ocular surgery that is particularly prolonged or is associated with a significant blood loss.
- People who have experienced significant bleeding from an accident or ruptured blood vessels. In these cases, the person may develop anemia (too few oxygen-delivering red blood cells in the bloodstream) and often have low blood pressure as well. This combination can produce circulatory shock, and PION has sometimes been called shock-induced optic neuropathy.
The combination of anemia and low blood pressure means that the blood is carrying less oxygen to the tissues. The optic nerve can be at very high risk for damage from insufficient blood supply due to swelling (from lack of oxygen) in a confined bony space resulting in a compartment syndrome. Restricted blood flow can lead to permanent damage to the optic nerve and result in blindness (often in both eyes). For technical reasons this occurs more frequently with spinal surgeries.
Cardiovascular risk factors
Perioperative PION patients have a higher prevalence of cardiovascular risk factors than in the general population. Documented cardiovascular risks in people affected by perioperative PION include high blood pressure, diabetes mellitus, high levels of cholesterol in the blood, tobacco use, abnormal heart rhythms, stroke, and obesity. Men are also noted to be at higher risk, which is in accordance with the trend, as men are at higher risk of cardiovascular disease. These cardiovascular risks all interfere with adequate blood flow, and also may suggest a contributory role of defective vascular autoregulation. This evidence suggests that optic nerve injury in PION patients is caused by more than just anemia and low blood pressure. When T-cells damage arteries supplying the optic nerve, a blood clot forms and stops blood flow. When blood flow stops, oxygen delivery stops and optic nerve fibers die.
Perioperative PION
The exact cause of perioperative PION is unknown. Many risk factors have been identified, all of which contribute to inadequate delivery of oxygen to optic nerve cells. Alone, none of these risk factors is enough to cause PION. However, in susceptible individuals, a combination of these risk factors produces devastating blindness. This evidence suggests that PION is a disease of multifactorial origin.
Risks of perioperative PION can be divided into two categories, intraoperative ischemic pressures, and cardiovascular risk factors.
Intraoperative ischemic pressures
Many causes of decreased blood flow during surgery are systemic, i.e. they decrease blood flow throughout the body. Studies have shown that nearly all perioperative PION patients had prolonged periods of low blood pressure during the operation and postoperative anemia. The average perioperative PION patient loses 4 liters of blood during surgery, and the majority receive blood transfusions. Massive blood loss is just one cause of low blood pressure. Medications used for general anesthesia can also lower blood pressure. The average surgery duration in PION cases is 7 to 9 hours, which increases the risk of prolonged low blood pressure.
Surgeries with the highest estimated incidence of PION are surgeries with a higher risk of the aforementioned conditions. In spine surgery, patients are susceptible to significant blood loss, and they are positioned face down for long periods of time, which increases venous pressure, decreases arterial perfusion pressure, and often causes facial swelling (increased tissue pressure). Spine surgery is estimated to have the highest incidence of PION, 0.028%.
Diagnosis
The diagnosis of PION is often difficult since the optic nerves initially appear normal. The injury occurs posterior to that portion of the nerve visible during ophthalmoscopic examination. There may be an abnormal relative pupillary response (APD) if the injury is confined to one optic nerve, but often it is bilateral and the symmetry of pupillary responses is maintained. Furthermore, MRI scanning may not be helpful. It is not uncommon for the erroneous diagnoses of malingering or cortical blindness to be made. If possible, an urgent neuro-ophthalmology consult is most likely to lead to the correct diagnosis.
There is no confirmatory test for PION. PION is a diagnosis of exclusion. To prevent impending blindness, it is urgent to rule out giant cell arteritis when a patient over 50 presents with sudden vision loss.
Differential diagnosis
In the postoperative setting, without gross eye injury, visual loss requires an assessment of the whole visual system for ischemic damage. The optic nerve is not the only tissue of the visual pathway susceptible to decreased blood flow. Decreased oxygenation of the retina or brain could also impair vision.
Prevention
Individuals with a history of high blood pressure, diabetes, and smoking are most susceptible to PION as they have a compromised system of blood vessel autoregulation. Hence, extra efforts may need to be taken for them in the form of careful or staged surgery or the controlling the anemia from blood loss (by administration of blood transfusions), and the careful maintenance of their blood pressure.
Treatment
Once visual loss has occurred, it becomes more problematic, but there are reports of recovered vision if blood transfusions and agents that raise blood pressure are administered within hours.
A-PION
If a diagnosis of GCA is suspected, treatment with steroids should begin immediately. A sample (biopsy) of the temporal artery should be obtained to confirm the diagnosis and guide future management, but should not delay initiation of treatment. Treatment does not recover lost vision, but prevents further progression and second eye involvement. High dose corticosteroids may be tapered down to low doses over approximately one year.
Perioperative
Rapid blood transfusions, to correct anemia and raise blood pressure, may improve PION outcomes. In one report of a related disease, hypotension-induced AION, 3 out of 3 patients who received rapid transfusions reported partial recovery of vision.
There is a higher than expected prevalence of atherosclerotic risk factors and comorbid vascular disease, especially in patients with nonarteritic (idiopathic) PION, with 87 percent of patients having at least one risk factor for, or one other manifestation of, atherosclerotic vascular disease.
While anterior ischemic optic neuropathy (AION) appears to be more common than PION after cardiac surgery, PION is relatively more common in cases of spine surgery.