Acute Bilateral Painless Vision Loss in a Pediatric Patient With Infective Endocarditis

Abstract

Infective endocarditis (IE) is a complex cardiac condition with a myriad of systemic complications, affecting approximately 5% of patients with ocular manifestations. This report details a rare and severe presentation of IE, marked by the acute onset of bilateral vision loss attributed to central retinal arterial occlusion (CRAO) and posterior ischemic optic neuropathy (PION), both critical conditions resulting from vascular impairments associated with IE. CRAO, characterized by the sudden obstruction of blood flow to the retina, leads to rapid vision deterioration, showcasing the critical impact of vascular emboli originating from infective endocardial lesions. On the other hand, PION, a less common vascular disorder, affects the posterior segment of the optic nerve, typically sparing the optic disc from edema but resulting in significant visual impairment. This case emphasizes the importance of recognizing ocular symptoms in patients with IE, as early detection and intervention are vital for managing these rare but devastating complications.

Case Report

An 18-year-old man with a complex medical history, including congenital heart disease, and multiple cardiac surgeries was admitted to the pediatric cardiac intensive care unit for an evaluation of aortic valve repair, presenting with acute bilateral vision loss. His medical history was significant for a prior episode of infective endocarditis (IE) 18 months earlier, which resulted in severe complications, including septic emboli, a mycotic aneurysm, and rupture in the left middle cerebral artery, resulting in hemorrhagic infarction and right-sided hemiplegia. Treatment for these complications included surgical clipping of the aneurysm, decompressive hemicraniectomy to relieve increased intracranial pressure, and a 4-week course of targeted vancomycin therapy. Due to prolonged intubation and airway complications, the patient also required a tracheostomy.

Preoperative evaluations with echocardiography and magnetic resonance imaging (MRI) revealed left ventricular dilation, moderate aortic stenosis, severe aortic regurgitation, and a mobile 18 mm × 6 mm vegetation on the non-coronary cusp of the aortic valve. Further neuroimaging identified a partially thrombosed aneurysm in the left supraclinoid internal carotid artery.

Following anesthesia for imaging, the patient experienced acute bilateral vision loss, fever, and hypotension. Blood cultures confirmed the presence of Staphylococcus aureus. An ophthalmic examination of the right eye showed retinal whitening around the macula and a white lesion on the optic disc, indicative of septic emboli leading to central retinal artery occlusion (CRAO). In the left eye, there was no pupillary response to light and a reverse afferent pupillary defect, while the fundoscopic examination appeared normal, suggesting posterior ischemic optic neuropathy (PION).

The primary goal of our management was to maintain adequate blood pressure to support ocular perfusion pressure while treating the underlying infection. Systemic management included the administration of fluids and vasoactive agents, such as epinephrine, norepinephrine, and vasopressin, to maintain appropriate blood pressure within the target range of 110 to 125/60 to 70 mmHg and a mean arterial pressure of 60 to 70 mmHg, ensuring optimal ocular perfusion pressure and mitigating the risk of further ischemic damage to the optic nerve and targeted antibiotic therapy with vancomycin and rifampin was initiated to treat the underlying Staphylococcus aureus infection in the patient’s bloodstream, aiming to clear blood stream infection and prevent systemic complications that could worsen the ocular pathology.

Table 1.

Ocular Examination findings.

Examination area	OD	OS
Visual acuity without correction	No light perception	No light perception
Extraocular motility	Normal	Normal
Pupils	Normal	No pupillary response to light, reverse afferent pupillary defect
Intraocular pressure	Normal	Normal
Anterior segment
Lids	Normal	Normal
Conjunctiva/Sclera	Normal	Normal
Cornea	Normal	Normal
AC	Normal	Normal
Iris	Normal	Normal
Lens	Normal	Normal
Dilated fundus examination
Vitreous	Normal	Normal
Optic nerve	White lesion within the artery on the optic disc, indicative of CRAO	Normal appearance (PION indicated by clinical symptoms rather than fundus appearance)
Macula	Retinal whitening around the macula	Normal
Vessels	Normal	Normal
Periphery	Normal	Normal

Supportive therapy included meticulous monitoring of vital signs, ensuring adequate fluid resuscitation, and providing respiratory support as indicated and maintaining the head of the bed below a 10-degree elevation to improve the optic nerve perfusion. A multidisciplinary team, including cardiologists, cardiothoracic surgeons, infectious disease specialists, neurosurgeons, and cardiac intensivists, continuously monitored the patient to assess cardiac function and ensure an optimal response to treatment.

After completing the antibiotic regimen and resolving the infection, the patient successfully underwent aortic valve repair using the Ozaki procedure, which was well tolerated. The patient was then discharged to a rehabilitation facility.

An ophthalmological evaluation 4 weeks after discharge revealed ongoing central retinal artery occlusion (CRAO) and posterior ischemic optic neuropathy (PION), with no improvement in visual acuity despite appropriate antibiotic treatment. This suggests that the optic nerve had sustained irreversible ischemic damage.

Discussion

The case illustrates the profound impact of infective endocarditis (IE) beyond cardiac complications, particularly in the context of devastating ocular manifestations such as central retinal artery occlusion (CRAO) and posterior ischemic optic neuropathy (PION). These complications, although rare, underscore the importance of prompt recognition and management to mitigate irreversible visual impairment.^1,2

Central retinal arterial occlusion is relatively rare, with an estimated incidence of 0.001% to 0.002% of the population. ³ Central retinal arterial occlusion mostly unilateral and in IE, it is primarily attributed to embolic phenomena from septic emboli originating from vegetations. This blockage of retinal blood flow, resulting from embolism or local thrombus formation, leads to sudden and severe vision impairment. ⁴ Bilateral CRAO is particularly rare, especially in younger patients, and is usually linked to autoimmune diseases such as lupus, Takayasu’s arteritis, and granulomatosis with polyangiitis.^5,6 The incidence of posterior ischemic optic neuropathy (PION) is also quite low. In a study of 3450 spinal surgeries over 9 years, only 3 patients (0.087%) experienced vision loss consistent with PION. ⁷

Posterior ischemic optic neuropathy (PION) involves impaired perfusion to the posterior segment of the optic nerve, leading to significant visual impairment. The intraorbital optic nerve relies on a network of small arteries from the ophthalmic artery.^8,9 This complex blood supply forms areas, known as watershed zones, which are vulnerable to reduced perfusion.^10,11

In our case, acute bilateral vision loss prompted ophthalmologic examination, revealing evidence of both central retinal artery occlusion (CRAO) and posterior ischemic optic neuropathy (PION). The presence of a mobile vegetation on the aortic valve increased the risk of embolization to distal organs, including the ocular system,^12,13 leading to CRAO in the right eye. Subsequently, recent exposure to anesthesia and the development of septic shock induced a state of low perfusion, resulting in PION in the left eye. Simultaneous occurrence of vision loss with differing etiologies is rare, particularly in young patients. Multiple comorbidities heightened the risk of these rare ocular complications in this case. Effective management of ocular complications in a complex systemic disease requires a coordinated and interdisciplinary approach involving cardiologists, ophthalmologists, infectious disease specialists, and intensivists. Close collaboration ensures timely diagnosis, appropriate medical and surgical interventions, and comprehensive patient care. In this case, the involvement of a multidisciplinary team facilitated optimal management, including systemic antibiotic therapy, maintenance of hemodynamic stability, and surgical intervention for the underlying cardiac pathology.

For acute CRAO, treatment strategies focus on lowering intraocular pressure, enhancing vasodilation, and thrombolysis of clots through pharmacological means or mechanically via procedures like anterior chamber paracentesis and laser embolectomy.^14-16 Despite these approaches, strong evidence for their efficacy is scarce. Similarly, for perioperative PION, recommendations include systemic corticosteroids, intraocular pressure management, and hemodynamic stabilization, but evidence is limited, making prevention the preferred strategy.^9,10,17 Our management prioritized maintaining optimal blood pressure to ensure ocular perfusion and addressing underlying infections. Despite aggressive management and targeted antibiotic therapy, the patient experienced persistent visual impairment secondary to irreversible ischemic damage to the optic nerve. This highlights the potential for long-term sequelae in patients with severe ocular complications of IE, underscoring the need for continued monitoring and supportive care to optimize functional outcomes and quality of life. Education and awareness among healthcare providers regarding the ocular manifestations of IE are essential for timely diagnosis and intervention.