Total thyroidectomy performed under general anesthesia with venovenous extracorporeal membrane oxygenation during a thyroid storm: a case report

Abstract

Background

Thyroid storm (TS) is an endocrine emergency requiring aggressive medical management. In severe cases, hemodynamic instability may necessitate extracorporeal membrane oxygenation (ECMO) support as a bridge to definitive surgical treatment. ECMO is categorized into two types: venoarterial (V-A) ECMO, which provides both cardiac and pulmonary support, and venovenous (V-V) ECMO, which supports only pulmonary function. Surgery is generally not recommended for patients with unstable TS due to the high risk of complications, even when ECMO support is in place. Here, we present a case of a 44-year-old man initially improved with V-A ECMO for TS with cardiogenic shock, but later developed refractory hypoxemia due to pulmonary thromboembolism (PTE). He subsequently underwent emergency thyroidectomy with continuous support from V-V ECMO.

Case presentation

A 44-year-old man presented to our hospital with complaints of palpitations. He had a recent history of coronavirus disease of 2019 (COVID-19) infection, which may have exacerbated undiagnosed hyperthyroidism, leading to thyroid storm and cardiogenic shock (left ventricular ejection fraction [LVEF], 13%). Heart failure improved with immediate medical management and V-A ECMO for 4 days, resulting in LVEF, 30%. V-A ECMO provide both respiratory and cardiac support, allowing myocardial recovery. Although the patient's cardiac output improved, uncontrolled tachycardia persisted. Medical treatment for hyperthyroidism-associated tachycardia was continued after V-A ECMO weaning but failed to achieve adequate rate control. Ten days after weaning V-A ECMO, the patient suddenly developed pulmonary thromboembolism and hypoxia despite ongoing heparinization. To manage refractory hypoxia, V-V ECMO was initiated, as it exclusively provides respiratory support.

Given that persistent TS was the underlying cause of the patient's instability, we proceeded with thyroidectomy under general anesthesia with V-V ECMO support, despite the associated risks. On postoperative day 4, the patient was successfully weaned off V-V ECMO. By postoperative day 18, he was discharged without complications, with an improved LVEF of 52.5%.

Conclusions

This is the first reported case of total thyroidectomy performed while on V-V ECMO support for TS complicated by PTE. Although V-V ECMO is more susceptible to hemodynamic instability than V-A ECMO, this case demonstrates that thyroidectomy can be successfully performed with appropriate anesthesia management. Additionally, careful selection of the ECMO modality based on the patient’s condition is crucial for optimal management.

Background

Thyroid storm (TS) is a life-threatening condition and presents with variable clinical manifestations of thyrotoxicosis. The incidence of thyroid storm ranges from 0.57 to 0.76 cases per 100,000 people in the United States annually [1]. It is characterized by fever, abdominal pain, tachyarrhythmia, multi-organ failure, and cardiogenic shock. TS is a medical emergency with a high mortality rate (8–25%) [2]. Immediate medical treatment includes antithyroid agents and supportive care. Additional treatment options include radioactive iodine ablation, plasmapheresis, and thyroidectomy [3].

Extracorporeal membrane oxygenation (ECMO) is an advanced lifesaving intervention for patients with acute cardiorespiratory failure and can be beneficial for those with endocrine disorders refractory to medical treatments. Numerous studies have reported favorable outcomes, with a survival rate of 85.2%, with the use of ECMO in patients with TS [4], as a successful bridge to achieving a euthyroid state [5,6,7,8,9,10]. However, in all previously reported cases, ECMO was weaned at least one day before thyroidectomy due to the high risk of ECMO-related complications, including renal failure, pneumonia, sepsis, and hemorrhage [11].

Total thyroidectomy is the definitive treatment for hyperthyroidism and is typically recommended after achieving a euthyroid state with medication. However, TS often occurs in the postoperative period in patients who have not received adequate preoperative treatment, such as beta-blockers [12]. If TS occurs, the surgical risk is significantly elevated, with an overall mortality rate of 10% [13]. To date, the surgical indications and optimal surgical timing for refractory TS with ECMO support remain undefined, and clinical evidence is also lacking. Only one case of total thyroidectomy has been successfully performed while on venoarterial (V-A) ECMO for TS complicated by cardiogenic shock [14].

Here, we present the case of a 44-year-old man with TS complicated by refractory hypoxemia due to pulmonary thromboembolism (PTE), successfully treated with emergent total thyroidectomy under general anesthesia while on continuous venovenous (V-V) ECMO support.

Case presentation

Initial presentation

A 44-year-old man presented to a local hospital with complaints of palpitations. He had no significant limitations in daily activities except for frequent diarrhea and fatigue. Three weeks prior to presentation, he had contracted coronavirus disease 2019 (COVID-19) and subsequently experienced persistent fatigue and dyspnea. Over time, his symptoms worsened, and he had been experiencing chest discomfort and palpitations for one week. There were no identifiable factors influencing the worsening or improvement of his symptoms.

A physical examination revealed a goiter, and radiographic imaging showed cardiomegaly. Electrocardiography demonstrated atrial fibrillation with rapid ventricular response (182 beats/min). The patient was transferred to our emergency room for further evaluation and treatment of suspected thyrotoxicosis. On arrival, he was normotensive (140/80 mm Hg), with normal temperature (36.2℃). However, he exhibited persistent severe tachycardia. To control his heart rate, amiodarone was administered immediately. Given the unknown status of his cardiac function, amiodarone was chosen over beta-blockers as the initial treatment.

Interventions

Thyrotoxicosis was diagnosed based on thyroid function test results: free thyroxine (fT4), 7.90 ng/dL; free triiodothyronine (T3), 3.98 ng/mL; and thyroid-stimulating hormone (TSH), < 0.008 µIU/mL. Given the risk of amiodarone-induced thyrotoxicosis [15], amiodarone infusion was discontinued. Echocardiography revealed tachycardia-associated biventricular dysfunction with global hypokinesia with left ventricular ejection fraction (LVEF) of 27.4%. Mild pleural effusion was observed on chest computed tomography (CT). Treatment for thyrotoxicosis was initiated with methimazole and propranolol. The patient’s blood pressure decreased from 140/80 mmHg to 90/60 mmHg, and he exhibited persistent hyperlactatemia (3.5–15 mmol/L over half a day). Given his rapidly deteriorating condition, venoarterial (V-A) extracorporeal membrane oxygenation (ECMO) was initiated in the emergency room via cannulation of the right femoral artery and left femoral vein (settings: FiO₂, 0.8; flow, 3.0 L/min; 3500 RPM). He was then admitted to the intensive care unit (ICU). Heparin administration was started with the target ACT set to 180–200 s (1.5–2.5 times normal). V-A ECMO was deemed appropriate due to the patient’s elevated lactate levels, which were indicative of reduced systemic perfusion and decreased cardiac output, as confirmed on echocardiography. Hyperlactatemia, defined as a lactate level > 2 mmol/L, is a hallmark of shock states. Lactate levels reliably reflect disease severity and are associated with short-term mortality in critically ill patients.

On the following day, the patient developed cardiogenic shock with liver failure, as evidenced by markedly elevated liver enzymes (AST, 205–5752 U/L; ALT, 110–1837 U/L). There were no signs of jaundice or ascites. The left ventricular EF decreased from 27 to 13%. Despite ongoing V-A ECMO support, his oxygen saturation dropped to 70% following aspiration of gastric contents. A high-flow nasal cannula (HFNC) was applied (FiO₂, 0.5; 30 L/min) to improve oxygenation. HFNC has been reported to increase end-tidal lung volume and lung compliance, making it a suitable intervention for protecting the lungs in cases of aspiration-related lung injury.

By the fourth day after V-A ECMO initiation, his condition improved with continued medical management. Oxygen saturation was maintained at 95–98% with a high-flow nasal cannula. Even after successful V-A ECMO weaning, his vital signs remained stable, with a blood pressure of 110/70 mmHg and a heart rate of 75 beats per minute. It has been confirmed that cardiac function has been restored to EF of 30%. Oxygen support was transitioned from HFNC to a simple mask (5 L/min), and oxygen saturation remained above 95% without any complaints of dyspnea. However, despite ongoing antithyroid and beta-blocker therapy, the patient occasionally experienced nocturnal episodes of tachycardia, with his heart rate increasing to 110–120 beats per minute.

The day after the patient was weaned off V-A ECMO, heparinization was continued due to recurrent atrial fibrillation. Despite an increased dose of propranolol (target heart rate < 100 beats per minute), heart rate control remained ineffective. Consequently, propranolol was replaced with bisoprolol, which is reported to be more cardioselective than propranolol.

On the 10 th day of hospitalization, liver function tests showed improvement (AST, 410 U/L; ALT, 586 U/L); however, total bilirubin levels increased from 5.17 to 13.01 mg/dL. Methimazole was suspected as the cause of hyperbilirubinemia, prompting a switch to methimazole in Lugol’s solution. Lugol’s solution is composed of elemental iodine (5%) and potassium iodide (10%) in distilled water. As the patient’s condition remained unstable, elective total thyroidectomy was scheduled for the 20 th day of hospitalization. Although thyroid function tests showed minor improvement (fT4, 7.01 ng/mL; T3, 2.6 ng/mL; TSH, < 0.008 µIU/mL), his heart rate and rhythm remained uncontrolled despite escalating doses of bisoprolol and diltiazem. Meanwhile, vital signs, including blood pressure, body temperature, and oxygen saturation, remained stable. Echocardiography revealed severe left ventricular systolic dysfunction with an ejection fraction (EF) of 32%.

At midnight on the 14 th day of hospitalization, the patient experienced sudden-onset dyspnea, tachypnea (respiratory rate > 40 breaths/min), and tachycardia (heart rate > 140 beats/min). Electrocardiography showed atrial fibrillation (126 beats/min) with oxygen saturation at 70%, and normal blood pressure. Due to persistent atrial fibrillation with a rapid ventricular response, synchronized electrical cardioversion (energy: 150 J) was performed. Following cardioversion, electrocardiography demonstrated a normal sinus rhythm, although dyspnea persisted. A high-flow nasal cannula (FiO₂, 0.6; 60 L/min) was initiated to alleviate the worsening dyspnea. However, PaO₂ remained at only 41.3 mmHg. Despite intubation and mechanical ventilation (FiO₂, 1.0; P/F ratio: 51 mmHg), hypoxia persisted, necessitating the initiation of venovenous (V-V) extracorporeal membrane oxygenation (ECMO). The patient met the criteria for hypoxemic respiratory failure (PaO₂/FiO₂ < 80 mmHg), which is a common indication for V-V ECMO.

During intubation, sedation with midazolam led to hemodynamic instability, with blood pressure dropping from 120/80 mmHg to 70/40 mmHg and heart rate decreasing to 45 beats per minute. Norepinephrine (0.03 mcg/kg/min) was administered immediately, and once the patient regained consciousness, his vital signs stabilized.

To determine the cause of dyspnea, chest computed tomography (CT) and echocardiography were performed. Echocardiography revealed right ventricular (RV) dilation, decreased RV motion, and reduced left ventricular ejection fraction (LVEF, 35%) with diastolic relaxation abnormality (E = 0.64, A = 0.33, E/A = 1.92, E'= 0.05, E/E'= 14.05). Furthermore, chest CT showed pulmonary thromboembolism (PTE) involving the bilateral main, lobar, and segmental pulmonary arteries (Fig. 1). Despite continuous beta-blocker administration, the patient remained tachyarrhythmic, and his body temperature rapidly increased from 36.3 °C to 38.2 °C within four hours. Given the sudden exacerbation of symptoms, thyroid storm (TS) was suspected, prompting the decision to proceed with an emergency total thyroidectomy. Prior to intubation, the patient exhibited no symptoms suggestive of other infections, such as phlegm, rhinorrhea, or polyuria. However, since an underlying infection could not be entirely ruled out and surgery was planned, prophylactic antibiotics (piperacillin/tazobactam) were administered. Heparin was discontinued four hours before surgery. Due to the patient’s lack of cooperation, extubation was performed to reduce the risk of airway injuries associated with self-extubation. Sedation was avoided whenever possible, as it could further lower blood pressure and heart rate, while catecholamine use to maintain hemodynamic stability could potentially exacerbate TS.

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Operation

The patient was admitted to the operating room while on V-V ECMO using an oxygen reservoir mask (15 L/min). His mental state showed signs of anxiety and irritability. Laboratory tests revealed no electrolyte abnormalities, except for an elevated pH of 7.56. Standard monitoring was implemented, including noninvasive blood pressure (NIBP), pulse oximetry (SpO₂), electrocardiography (ECG), an esophageal temperature sensor, bispectral index (BIS) for anesthetic depth monitoring, and brachial arterial pressure monitoring with stroke volume variation. A nerve stimulator was also used to monitor the response of the adductor muscles. The initial vital signs were as follows: blood pressure, 130/75 mmHg; heart rate, 92 beats per minute; and body temperature, 37.7 °C. To manage potential arrhythmias, esmolol and amiodarone were prepared, and an external defibrillator was placed near the bedside. Additionally, norepinephrine was prepared to prevent hypotension following anesthesia induction. Anesthesia was induced using propofol (70 mg, 1 mg/kg) and remifentanil (1 mcg/kg/min), both of which have a rapid onset and short duration, allowing for better control in response to blood pressure fluctuations. After confirming the loss of consciousness, rocuronium (50 mg) was administered to facilitate endotracheal intubation. Prior to intubation, lidocaine (1 mg/kg) was administered to reduce the risk of arrhythmias. Endotracheal intubation was performed using videolaryngoscopy to minimize airway stimulation and to accommodate tracheal deviation secondary to the goiter. A left subclavian central venous catheter was inserted for central venous pressure monitoring ECG revealed atrial fibrillation and frequent premature ventricular contractions; however, other vital signs remained stable throughout the surgery without the need for additional medication. The total duration of surgery was three hours. The patient was transferred to the intensive care unit (ICU) while intubated, mechanically ventilated, and supported by V-V ECMO. The anesthesia record is shown in Fig. 2.

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Postoperative course

In the intensive care unit (ICU), the patient was sedated with dexmedetomidine and sufentanil. On the day following surgery, he was extubated and transitioned to supplemental oxygen via a high-flow nasal cannula (FiO₂, 0.5; flow, 60 L/min). The next day, oxygen support was switched to a simple mask (5 L/min). The patient did not report any difficulty breathing or shortness of breath, and oxygen saturation was maintained above 95%. Heparin was restarted after confirming the absence of bleeding from the surgical site. However, the initial postoperative thyroid function tests showed no significant improvement (fT4, 8.57 ng/dL; T3, 2.15 ng/mL; TSH, < 0.026 µIU/mL).

On postoperative day 4, the patient was successfully weaned off V-V ECMO without any breathing difficulties. Echocardiography demonstrated improved systolic function (LVEF, 40%), and the pulmonary thromboembolism (PTE) was nearly resolved. Thyroid function tests also showed improvement (fT4, 1.59 ng/dL; T3, 0.54 ng/dL; TSH, 0.009 µIU/mL). Levothyroxine therapy was initiated five days after thyroidectomy. On postoperative day 7, the patient was transferred from the ICU to the general ward. By postoperative day 14, heparin was replaced with dabigatran, a novel oral anticoagulant (NOAC). Echocardiography revealed complete resolution of tachycardia-induced cardiomyopathy, with preserved left ventricular systolic function (LVEF, 52.5%), and electrocardiography showed a normal sinus rhythm. On postoperative day 18, the patient was discharged without complications. Thyroid function test results (Fig. 3) and the patient’s clinical course (Supplemental Table 1) are summarized. Written informed consent for publication was obtained from the patient.

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Discussion and conclusions

This case report describes a complex clinical course involving three sequential events. First, V-A ECMO was initiated for the management of cardiogenic shock secondary to TS. Second, V-V ECMO was implemented for hypoxia management following the development of PTE during supportive care for TS. Third, emergency total thyroidectomy was performed while the patient remained on V-V ECMO due to worsening TS.

There are no universally accepted criteria or validated clinical tools for diagnosing thyroid storm. However, the scoring system introduced by Burch and Wartofsky in 1993 is widely used. This system evaluates multiple parameters, including body temperature, central nervous system dysfunction, tachycardia, atrial fibrillation, heart failure, gastrointestinal dysfunction, and the presence of precipitating factors [16]. Thyroid function tests in TS typically show suppressed TSH levels (< 0.01 mU/L), along with elevated fT4 and/or free fT3 levels.

Several factors can trigger the transition from hyperthyroidism to TS, including thyroid or non-thyroid surgery, trauma, infection, acute iodine load, parturition, and long-standing untreated hyperthyroidism [17]. In our patient, a recent history of COVID-19 may have exacerbated previously undiagnosed hyperthyroidism, leading to TS. It has been reported that thyroid function alterations during COVID-19 are more likely a result of pro-inflammatory signaling and impaired central regulation rather than direct infection of follicular cells by SARS-CoV-2 [18].

ECMO is an advanced life support system with two primary configurations: V-A ECMO and V-V ECMO. V-A ECMO provides both respiratory and cardiac support and is indicated for patients with low cardiac output (cardiac index < 2 L/min/m2) and hypotension (systolic blood pressure < 90 mmHg) that persist despite inotropic therapy and intra-aortic balloon pump (IABP) support. In contrast, V-V ECMO is used exclusively for respiratory support in patients with acute, potentially reversible respiratory failure who do not respond to mechanical ventilation [19].

ECMO can be a viable option for patients with TS in critical condition, with the choice of ECMO type determined based on the patient’s clinical status [5,6,7,8,9,10]. In this case, V-A ECMO was initiated early in hospitalization when cardiac output decreased, and lactate levels increased. Later, on the 14 th day of hospitalization, V-V ECMO was initiated due to hypoxia secondary to PTE. The likely cause of PTE in this patient was prolonged bed rest combined with recurrent atrial fibrillation with a rapid ventricular response.

This patient presented several challenges in both the surgical and anesthetic management processes. First, the patient had multiple overlapping conditions associated with high mortality rates. The in-hospital mortality rate for patients receiving ECMO is reported to be 56.7% [20] and among TS patients with unstable thyroid function undergoing surgery, the overall mortality rate is approximately 10% [13]. Second, anesthetic agents can cause varying degrees of hypotension due to their vasodilatory effects. Additionally, general anesthesia requires positive pressure ventilation, which can further lower blood pressure. In this case, the patient was maintained on V-V ECMO, which limited the ability to increase cardiac output, posing an additional challenge. Third, anticoagulation is necessary to maintain ECMO circuit patency, increasing the risk of bleeding. Current guidelines recommend discontinuing heparin 4–6 h before elective surgery, and in this case, heparin was stopped 4 h before surgery [21].

Thyroidectomy under local anesthesia was considered in consultation with the surgical team but was ultimately ruled out for the following reasons. First, with light sedation, there was a risk that the patient’s movements might not be adequately controlled, such as reaching toward the surgical site or attempting to remove the ECMO cannula. Second, deep sedation carried the potential for recurrent hemodynamic instability.

General anesthetic management in thyroid storm focuses on minimizing sympathetic nervous system activation, administering medications related to hyperthyroidism, assessing the patient’s overall condition, and understanding the physiological implications of V-V ECMO [22]. Wee et al. recently reported a case of total thyroidectomy performed with V-A ECMO support [14]. V-A ECMO can augment arterial blood flow by increasing pump speed in response to hypotension. However, our case differs due to the use of V-V ECMO, which is hemodynamically less favorable. During V-V ECMO, factors such as decreased vascular tone, reduced preload, and impaired cardiac function can contribute to hypotension. These mechanisms overlap with the causes of hypotension observed during general anesthesia, further complicating hemodynamic management in this setting.

In conclusion, this case demonstrates that ECMO can be a viable option for patients with thyroid storm (TS) in critical condition, with the choice of ECMO type determined based on the patient’s clinical status. Furthermore, if appropriate anesthetic management is implemented, thyroidectomy can be considered even in TS patients maintained on V-V ECMO. This case underscores the importance of individualized treatment beyond standard management protocols. A thorough understanding of the patient’s condition, medication status, and life support system enables anesthesiologists to effectively manage complex and challenging situations.

Data availability

No datasets were generated or analysed during the current study.

Abbreviations

TS:

Thyroid storm

ECMO:

Extracorporeal membrane oxygenation

V-A:

Venoarterial

V-V:

Venovenous

COVID-19:

Coronavirus disease of 2019

LVEF:

Left ventricular ejection fraction

PTE:

Pulmonary thromboembolism

BIS:

Bispectral index

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