Stroke with postoperative visual loss as the first clinical manifestation of antiphospholipid antibody syndrome: Case report

Abstract

Introduction

Postoperative visual loss (POVL) is a rare and devastating complication. Its incidence in nonophthamologic surgeries varies from 0.056 % to 1.3 %. Autoimmune rheumatic diseases with a predisposition to thrombotic events, such as antiphospholipid antibody syndrome (APS), may constitute an important risk factor for this complication.

Presentation of case

A 34-year-old female patient, who was a former smoker and had no other comorbidities. She underwent orthopedic surgery and presented with bilateral POVL associated with the loss of secondary muscle strength and intraoperative venous and arterial cerebral thrombosis. She was thoroughly investigated regarding the etiology of her condition, and high levels of antiphospholipid antibodies were found.

Discussion

APS is an autoimmune disease that predisposes the patient to thrombotic events. Among these, stroke is one of the main causes of POVL secondary to ischemia of the cortical territory, or also known as “cortical blindness.”

Conclusion

The rare incidence of POVL in nonophthalmological surgeries and the consequence and preservation in the literature on the subject, explain the limitations of its pathophysiology, and especially the development of guidelines focused on the prevention of patients with risk factors for this condition. Thus, this case report warns about the risks and anesthetic care that patients with risk factors should have when undergoing nonophthalmological surgeries.

Highlights

• •Postoperative visual loss is a rare complication in non-ophthalmologic surgeries
• •Antiphospholipid syndrome is characterized by multiple organ thrombosis
• •Cortical blindness and stroke are the leading causes of postoperative visual loss

1. Introduction

Postoperative vision loss (POVL) is a rare complication in the postoperative period of nonophthalmic surgeries, with an incidence of 0.056 %–1.3 %. Cardiac (0.09 %) and spine (0.2 %) surgeries pose the highest risks for this event [1].

Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disease associated with persistent levels of antiphospholipid antibodies, which predisposes the individual to thrombotic events and obstetric complications, including multiple miscarriages [2], [3]. Thrombosis resulting from this disease can occur in arterial, venous, or small vessel regions in any tissue or organ. In addition, APS is typically associated with other systemic autoimmune diseases, including systemic lupus erythematosus, but clinical presentation in isolation is uncommon, in this case being called primary APS [2], [4]. Although nonthrombotic ocular changes can occur as the first symptom, thrombotic ocular events in central retinal vessels are the primary cause of manifestations [5].

This is a case report of POVL as the earliest clinical manifestation of previously undiagnosed APS, secondary to central venous thrombosis and ischemic stroke during orthopedic surgery. Written informed consent was obtained from the patient, and this case was previously approved by the ethics committee for publication. This case report follows the SCARE 2020 Guidelines [4], [6].

2. Presentation of case

A 34-year-old woman, 92 kg, 170 cm tall, body mass index 31.09 kg/m², who underwent right hip replacement 1 year ago because of trauma from a car accident, was admitted to the orthopedic emergency room with pain and difficulty in walking associated with an antalgic gait, right lower-limb edema, and large amounts of seropurulent exudate from the operative scar. She had no chills or fever and denied consuming antibiotics. A diagnosis of loosening of the femoral component of the hip prosthesis was suggested, and urgent surgery was proposed for prosthesis removal and surgical debridement.

In preanesthetic evaluation, she reported having fibromyalgia, being a smoker, and use of oral contraceptives. She also denied previous thromboembolic events or other associated comorbidities. Previous and preoperative laboratory test results revealed anemia and elevated inflammatory biomarkers (Table 1). The patient received a transfusion of packed red blood cells the day before the surgery.

Table 1.

Preoperative laboratory tests.

Laboratory tests	First admission	Actual admission	Normal values
Alanine aminotransferase		21 IU/mL	15.00–32.00 IU/mL
Amylase		19 IU/L	30–118 IU/L
Aspartate aminotransferase		5 IU/mL	17.00–31.00 IU/mL
Calcium		7.9 mg/dL	8.5–10.5 mg/dL
Creatinine		0.57 mg/dL	0.60–1.10 mg/dL
Chagas disease		Negative	Negative
Hemoglobin	8.71 g/dL	7.42 g/dL	13.00–17.00 g/dL
Hematocrit	30.3 %	22.6 %	42.00 %–50.00 %
Hepatitis B		Negative	Negative
Hepatitis C		Negative	Negative
Human immunodeficiency virus		Negative	Negative
Lipase		12 IU/L	13–60 IU/L
Platelets		611 cells/mm3	150.000–450./mm3
Potassium		3.8 mEq/L	3.2–5.6 mmol/L
Prothrombin time		16.7 s	9.80–12.10 s
C-reactive protein (CRP)	11 mg/dL	40.8 mg/dL	0.00–0.50 mg/dL
Erythrocyte sedimentation rate (ECR)	7 mm/h	45 mm/h	Up to 25 mm/h
Sodium		142.2 mEq/L	135–145 mmol/L
Total leukocytes		11,238 cells/mm3	4000.0–11,000.0/mm3
Urine sediment examination	No changes	No changes	Normal

In the operating room, noninvasive monitoring was established and antibiotic prophylaxis with 2-g cefazolin was administered. Combined regional-general anesthesia was performed with spinal analgesia using 5-mg hyperbaric bupivacaine and 80-μg morphine, then anesthetic induction with 250-μg fentanyl, 80-mg lidocaine, 150-mg propofol, and 80-mg rocuronium, followed by orotracheal intubation. A right radial arterial line was acquired, as well as a central venous access in the right subclavian vein, and central temperature monitoring was started. These procedures occurred with no untoward manifestations. The patient was positioned in lateral decubitus, and the eyes were protected. Anesthesia was maintained with sevoflurane (minimum alveolar concentration, 0.7–0.9) and dexmedetomidine in a continuous infusion pump (0.2–0.7 μg/kg/h).

Intraoperative bleeding led to a reduction in hemoglobin levels (6.8 g/dL, Table 2), resulting in a total transfusion of three units of packed red blood cells and one cryoprecipitate during surgery. No significant hemodynamic variations, changes in core temperature, or need for intraoperative use of vasoactive drugs was noted. Intraoperative vital signs are shown in Fig. 1.

Table 2.

Serial arterial blood gas analyses.

Parameters	Results
pH	7.36	7.32	7.36	7.35
pO2	102.7	110.8	128.0	114.0
pCO2	43	40	38	42
SatO2	99.8 %	99.8 %	98.7 %	98.7 %
Hemoglobin	8.9	6.8	8.2	9.3
Hematocrit	24.6 %	20.4 %	24.2 %	27.9 %
Serum HCO3−	22.6	23.6	22.0	20.0
Base excess	−2.2	−2.5	−2.0	−2.0
Glucose	117	126	118	119
Lactate	0.99	2.03	1.58	1.22
Na+	142	144	142	139
K+	3.57	3.53	4.03	3.53
Ca++	0.928	1.028	0.927	1.056

Results of serial arterial blood gases in chronological order from left to right.

Fig. 1.

Intraoperative vital signs: The horizontal axis presents the total anesthesia time of 05 h divided by 15/15 and the vertical axis presents the numerical values of the recording of each vital sign over time.

The patient was extubated without complications, and had good respiratory pattern and hemodynamic stability. The immediate postsurgical neurological pattern was similar to the initial one. During the postanesthesia care unit (PACU) stay, the patient had a fever (37.9 °C) and tremors. The visual analog scale for pain was 0 points, and the patient had no verbal reports of pain or nausea. On the first postoperative day, the patient suddenly presented with bilateral amaurosis and vertical nystagmus with nonphotoreactive pupils bilaterally, associated with right upper-limb monoparesis (grade III on a scale of muscle strength), and preserved facial expression. Noncontrast computed tomography (Fig. 2) and angiography (Fig. 3) were suggestive of intraluminal thrombus.

Fig. 2.

Noncontrast computed tomography scan: Hypodense lesions in the upper portion of the left cerebellar hemisphere and posterior portion of the left temporal and occipital lobes (white arrow) due to ischemic injury sequelae, without any signs of intracranial hemorrhage or deviation of midline structures.

Fig. 3.

Carotid computed tomography angiography: Filling defect of the P2 segment of the left posterior cerebral artery, suggestive of an intraluminal thrombus (white arrow). Hypodense lesions in the upper portion of the left cerebellar hemisphere and posterior portion of the left temporal and occipital lobes (yellow arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

The patient was clinically followed up for a thorough diagnostic investigation. Findings of a transthoracic echocardiogram were within normal limits and showed no images compatible with valvular vegetation. Serological and thrombophilias tests were performed evidencing the diagnosis of APS (Table 1, Table 3).

Table 3.

Thrombophilia investigation tests.

Thrombophilia tests	Results	Normal values
Lupus anticoagulant antibodies	2.01	<1.2
Anticardiolipin IgG	25.1	<10
Antinuclear antibodies (ANA)	1:320 with dense fine dotted nuclear pattern	Negative
Factor V Leiden (G1691A)	Normal	Normal
Prothrobin (G20210A)	Normal	Normal
Total protein S	85 %	Female (65 %–154 %)
		Male (67 %–142 %)
Free protein S	68 %	Female (52 %–118 %)
		Male (69 %–123 %)
Antithrombin III	92 %	83 %–28 %
Plasminogen	85 %	80 %–133 %
Tissue plasminogen activator	1.0 ng/mL	2–12.0 ng/mL
C-reactive protein (CRP)	43.8 mg/dL	0.00–0.50 mg/dL

Thrombophilia test results demonstrating elevated serum levels of lupus anticoagulant antibodies, IgG anticardiolipin antibody, and antinuclear antibody (ANA), in addition to reduced levels of tissue plasminogen activator.

The patient in the present case is currently under routine medical, psychological, and physical therapeutic follow-up. She has continuously used gabapentin and dabigatran and still has right hemiparesis and crural predominance with force grade 0 in the right lower limb and grade IV in the right upper limb. The patient's lateral visual field has partially improved, which has helped in the return of some of the daily routine activities.

3. Discussion

As a complication of surgery, POVL can have severe implications for the patient's quality of life [1]. The incidence of this condition varies depending on the type and characteristics of the surgery and the patient-specific modifiable risk factors. Risk factors associated with POVL include the male sex, a prone position during surgery, severe hypotension, prolonged anesthesia procedures, anemia, and restricted use of colloids. Modifiable factors include obesity, cardiovascular diseases, hyperlipidemia, diabetes, and smoking. The leading causes of POVL include ischemic optic neuropathy, retinal artery occlusion, and cortical blindness.

Cortical blindness is considered the third leading reason of POVL, with an estimated incidence of 0.0038 % [1], [2]. The pathophysiological mechanism of visual loss involves ischemia or extreme hypoperfusion of the occipital lobes, manifesting itself as complete or partial bilateral visual loss. The leading causes of this condition include stroke (32 %), cardiac surgery (20 %), and cerebral angiography (12 %) [1], [2].

In addition, APS is an autoimmune disease characterized by the presence of lupus anticoagulant antibodies that predispose the individual to thrombotic events. The pathophysiological mechanism involves the binding of antiphospholipid antibodies to glycoprotein I (β2GPI), a plasma protein that avidly binds to phospholipid surfaces, positively regulating the expression of prothrombotic cell-adhesion molecules, including E-selectin and tissue factor [6], [7]. Ischemic stroke and transient ischemic attacks are the most common arterial events in patients with APS. The most common venous thrombotic events involve deep-vein thrombosis or pulmonary embolism [8].

Fregoso et al. [9] reported that an International normalized ratio (INR) over 1.5 before surgery was significantly associated with severe bleeding, and appropriate management was significantly associated with a reduced incidence of complications. These findings highlight the significance of maintaining a low INR before surgery and attempting to minimize bleeding incidents. Although the most widely used plasma product to treat clotting factor deficiency is fresh frozen plasma (FFP), it is not known whether the use of FFP will increase the risk of thrombotic events or just decrease the bleeding risk seen in these patients [10].

The pathophysiological mechanisms of POVL are still unknown, and the literature regarding specific perioperative guidelines or protocols for patients or surgeries with risk factors is still scarce. The current recommendation is general perioperative care, such as mechanical eye protection based on specific ophthalmic ointments and eye protectors, correction of intraoperative hemodynamic changes (e.g., hypovolemia, hypotension, and anemia), and the choice of the best anesthetic technique for the current procedure. In the latter, greater care should be taken in patients with risk factors in cases involving general anesthesia [1], [2].

The patient in this case was a smoker and had APS, which was never before been clinically manifested and therefore undiagnosed until the complication described occurred. The intraoperative stroke in the occipital region, secondary to arterial and venous thrombosis, predisposed the patient to a picture of cortical blindness, clinically manifested by bilateral amaurosis.

4. Conclusion

POVL is a devastating and rare condition in nonophthalmologic surgeries. Its pathophysiological mechanism is still unknown, and therefore, recommendations for its prevention are scarce in the literature. Thus, even with these basic preventive measures, there may be inherent and undiagnosed factors in the patient that predispose him to POVL. This reinforces the importance, in these cases, of a contextualized and multidisciplinary approach in the perioperative period, with the objective of correctly diagnosing the etiology that led to POVL, as well as in the postoperative period, with adequate patient follow-up, also seeking partial improvement of the current condition.

Patient consent

Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

The case report was approved by the Ethics and Research Committee (CEP) of the Foundation for Teaching and Research in Health Sciences of the secretary of health of the Federal District - DF. The report protocol number is 64836522.2.0000.5553 and a statement of ethical approval will be included in the publication of the article along with the informed consent.

Funding

All funding sources have been acknowledged.

Author contribution

Angelo Rossi: Conceptualization, Investigation, Writing - Original Draft.

Fabricio Tavares: Writing - Review & Editing, Supervision.

Marcus Alexandre: Project administration, Visualization.

Ricardo Del Negro: Resources, Investigation.

Rodrigo Gomes Minas Novas: Resources, Investigation

Guarantor

Angelo Rossi Neto

Research registration number

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Conflict of interest statement

The authors declare no conflict of interest.