Abstract
Background
Guillain-Barré syndrome (GBS) is an acute immune-mediated disorder that leads to progressive muscular weakness, autonomic dysfunction, and potentially respiratory failure.
Case Report
A 41-year-old Caucasian man presented with progressive bilateral ascending weakness, concerning for GBS, accompanied by rapidly worsening respiratory status. Chest imaging revealed multiple causes of acute hypoxemic respiratory failure. This is the first reported case of GBS supported by veno-venous extracorporeal membrane oxygenation (V-V ECMO) during recovery and discharge.
Guillain-Barré syndrome (GBS) is an acute, atypical, immune-mediated disorder, often triggered by a recent viral infection, which can lead to muscular weakness, autonomic dysfunction, and respiratory failure in severe cases [1]. It targets the peripheral nervous system and may cause acute neuromuscular respiratory failure owing to progressive motor weakness of the diaphragm and oropharynx [1]. GBS is generally considered a recoverable disease process, with 80% of patients regaining the ability to walk again and 50% able to return to baseline functional status by the 1-year mark [1]. Extracorporeal membrane oxygenation (ECMO) is an advanced form of life support that provides support for the heart and/or lungs via a temporary, portable, modified cardiopulmonary bypass machine. Veno-venous extracorporeal membrane oxygenation (V-V ECMO) alone provides respiratory support and can be offered as an intervention when conventional strategies fail.
We present a complex case of GBS requiring V-V ECMO for refractory acute hypoxemic respiratory failure and highlight how early consideration and initiation of ECMO can reduce mortality. To our knowledge, this is the first documented case report of ECMO use in this patient population.
A 41-year-old man with a history of peripheral neuropathy, depression, alcohol use disorder, chronic marijuana use, and prior traumatic pneumothorax presented with a 3-day history of progressive, ascending bilateral muscle weakness and reduced tactile sensation, following a self-limiting respiratory illness 1 month earlier. Physical examination revealed moderate-to-severe muscle weakness throughout the cervical region and all four extremities. Cerebrospinal fluid analysis demonstrated albuminocytologic dissociation (total nucleated cell count, <1; total protein, 116 mg/dL). Initial respiratory mechanics showed mild impairment (vital capacity, 3.92 L; maximum inspiratory pressure, –60 cm H2O; and maximum expiratory pressure, 60 cm H2O). Electromyography revealed acute-to-subacute mixed demyelinating and axonal polyradiculoneuropathy. The most common differential diagnosis was acute inflammatory demyelinating polyradiculopathy or GBS. He was admitted to the neurology department for intravenous immunoglobulin (IVIG) treatment.
On hospital day 2, he developed worsening upper extremity paralysis and progressive autonomic symptoms, manifesting as hypertensive urgency. By hospital day 3, he had new bulbar symptoms, including dysarthria, facial weakness, dysphagia, and poor secretion management. Chest radiography revealed consolidation of the right middle lobe with associated leukocytosis (18,000 cell/μL). Respiratory mechanics acutely declined; vital capacity, 2.26 L; negative inspiratory force, –30 cm H2O; and maximum expiratory pressure, 40 cm H2O, with significant effort. His clinical status rapidly deteriorated, requiring intubation for progressive hypoxemic respiratory failure and secretion burden.
The post-intubation ratio of arterial partial pressure of oxygen (PaO2) to the fraction of inspired oxygen (FiO2) (P/F ratio) was 127, with a FiO2 of 55% and positive end expiratory pressure (PEEP) of 6. Bronchoscopy findings were purulent and suspicious for infection. Empiric vancomycin and piperacillin-tazobactam were initiated for presumed aspiration or hospital-acquired pneumonia, (ultimately diagnosed as Streptococcus pneumoniae infection). Chest computed tomography angiography revealed subtotal atelectasis of the bilateral lower lobes with extensive endobronchial plugging, dependent opacities, and a right upper lobe segmental pulmonary embolism (PE) despite deep venous thrombosis (DVT) prophylaxis (Fig. 1). Systemic heparin therapy was initiated. Hypoxemia worsened with a P/F ratio of 88 despite optimal medical management, including 100% FiO2, optimal PEEP, deep sedation, paralysis, and inhaled nitric oxide. The ECMO consultation team was engaged, and he was considered a favorable candidate for V-V ECMO.
On hospital day 4, the patient underwent peripheral V-V ECMO cannulation using a 25-F venous drainage cannula in the right common femoral vein and a 19-F venous return cannula in the right internal jugular vein. After connection to the ECMO circuit, blood flow of 4 L/min was achieved, providing adequate systemic oxygenation. With V-V ECMO support, paralysis was resolved, sedation was decreased, and mechanical ventilation support was weaned. After 48 hours of ECMO support, antibiotics, and anticoagulation, ECMO weaning trials were initiated. Oxygen flow through the ECMO circuit was trialed off for 24 hours, allowing the ventilator to resume primary management of oxygenation and respiratory mechanics. The patient tolerated the trial well and was successfully decannulated from ECMO on hospital day 7.
Currently, supportive management remains the standard of care for GBS-related respiratory failure. However, intensive care unit (ICU) admissions are increasing owing to complications associated with GBS. Among ICU-admitted patients with GBS, mortality ranges from 3%–7%, typically secondary to sepsis, acute respiratory distress syndrome (ARDS), PE, or cardiac arrest [1]. In GBS patients, 50%–75% develop pneumonia secondary to aspiration and 75% of these patients will require either non-invasive positive pressure ventilation or escalation to intubation and mechanical ventilation [2]. In addition, prolonged immobility, infections, and treatment with IVIG, patients with GBS in the ICU are also at higher risk for venous thromboembolism, with up to 50% of patients developing DVT and 7%–15% developing PE [3]. In each of these complications ECMO may have a role when the current standards of care are not sufficient.
V-V ECMO provides respiratory support only and can be used as a bridge to recovery in patients with reversible respiratory failure or as a bridge to organ transplantation in appropriately selected candidates with irreversible lung disease [4]. The ECMO circuit consists of a venous drainage and return cannula, centrifugal pump, membrane oxygenator, and temperature regulation device. During V-V ECMO therapy, blood is drained from the inferior vena cava to the membrane oxygenator, where gas exchange occurs, removing CO2, oxygenating the blood, and returning the blood back to the superior vena cava (Fig. 2). ECMO allows time for the patient’s lungs to rest and recover. ECMO is not considered as a first-line intervention for respiratory failure because it is resource-heavy and is associated with serious potential complications, including bleeding, thrombosis, hemolysis, thrombocytopenia, infection, limb ischemia, and air embolism.
The decision to initiate ECMO is often the most challenging step in the ECMO process. Because ECMO is resource-intensive and carries significant procedural risk, patient selection is made collaboratively by a multidisciplinary team, typically including a cardiothoracic surgeon or ECMO intensivist to optimize the likelihood of long-term survival without severe disability. Scoring systems, such as the Murray Score and the Respiratory ECMO Survival Prediction (RESP) score, support clinical decision-making. The Murray Score stratifies the severity of lung injury, while the RESP score incorporates pre-ECMO variables associated with hospital survival to guide clinicians in candidate selection [4]. Other major factors considered include age, diagnosis, rate of disease progression, overall disease duration, comorbidities (including acute or chronic multiple organ failure), frailty, patient anatomy, and the possible need for hospital transfer. Our patient was deemed a good candidate because of the acute hypoxemic respiratory failure having a reversible cause, young age, single organ failure, lack of major comorbidities, and no relative contraindications, which would include central nervous system injury or hemorrhage, contraindications to anticoagulation, mechanical ventilation for >7 days with plateau pressures >30 cm H2O or 90% FiO2 [4].
This case demonstrates that V-V ECMO can be successfully used in patients with GBS when conventional respiratory management strategies are insufficient. Early consideration of ECMO can prevent delays in care and improve patient mortality rates. Our patient experienced multiple complications associated with GBS including neuromuscular respiratory failure, aspiration pneumonia, ARDS, and PE. Therefore, the patient was at high risk of mortality. V-V ECMO, in conjunction with conventional pharmacologic treatments, provides additional support and time needed for the patient’s lungs to recover and ultimately discharge to outpatient rehabilitation.
V-V ECMO has been successfully used as a bridge to recovery for the treatment of ARDS with a 40%–60% survival rate and PE with a 49% survival rate, which are the two primary causes of mortality in patients with GBS [5,6]. To decrease mortality, the implementation of a structured escalation plan, including ECMO, would decompensate patients who fail standard treatment. Institutions with ECMO capability are familiar with the ECMO candidacy criteria. Pre-emptively engaging the ECMO consult service for “ECMO watch” can be beneficial in case of rapid patient deterioration. Institutions without ECMO capabilities should establish a protocol to expedite transfer to an ECMO-capable center if the patient fails to respond to standard medical therapy. A proactive care team with a plan for ECMO escalation before decompensation could decrease mortality in patients with severe GBS complications.
Notes
Ethics statement
Institutional review board approval and informed consent were not required because this was a case report without identifiable patient information.
Author contributions
Conceptualization: MAK, TL. Methodology: MAK, JW, TL. Formal analysis: MAK, JW. Data curation: MAK, JW, TL. Visualization: MAK, TL, JG. Project administration: MAK. Writing - original draft: MAK, JW, TL, JG. Writing - review & editing: MK, JG. All authors read and agreed to the published version of the manuscript.
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