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Principles and Practice of Anesthesia for Thoracic Surgery pp 265–279Cite as

Thymic Surgery and Paraendocrine Syndromes

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Abstract

Myasthenia gravis is a complex autoimmune disease, the pathogenesis of which involves hypertrophy or tumor of the thymus gland. We review recent advances in our understanding of the autoimmune basis of the disease and the place of thymectomy for malignant and nonmalignant disease. We review considerations for anesthetizing the patient with myasthenia gravis, with particular emphasis on the problems of the anterior mediastinal mass, management of muscle relaxants and anticholinesterases, prediction of postoperative ventilatory failure, and differentiation of myasthenic vs cholinergic crisis. We also describe the Lambert-Eaton myasthenic syndrome, with particular reference to the differences in presentation and management compared to myasthenia gravis. Carcinoid syndrome is an infrequently occurring problem in thoracic surgery but potentially serious and difficult to manage without experience, so we review the pathogenesis and clinical presentation of carcinoid syndrome and its prophylaxis and specific treatment with octreotide and serotonin antagonists.

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Abbreviations

ACTH:

Adrenocorticotrophic hormone

CRH:

Corticotrophin-releasing hormone

CT:

Computed tomography

CVP:

Central venous pressure

ECG:

Electrocardiogram

GHRH:

Growth-hormone-releasing hormone

IVIG:

Intravenous immunoglobulin

LES:

Lambert-Eaton syndrome

LRP-4:

Lipoprotein receptor-related protein 4

MEN:

Multiple endocrine neoplasia

MG:

Myasthenia gravis

MGFA:

Myasthenia Gravis Foundation of America

MuSK:

Muscle-specific kinase

NDMR:

Nondepolarizing muscle relaxants

NMJ:

Neuromuscular junction

SCLC:

Small cell lung cancer

SIADH:

Syndrome of inappropriate release of antidiuretic hormone

TEA:

Thoracic epidural anesthesia

TIVA:

Total intravenous anesthesia

TSH:

Thyroid-stimulating hormone

VATS:

Video-assisted thoracic surgery

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Authors and Affiliations

  1. Department of Anesthesia and Pain Management, Toronto General Hospital, University Health Network, Toronto, ON, Canada

    Daniel Sellers & Karen McRae

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  1. Daniel Sellers
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Correspondence to Karen McRae .

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  1. Department of Anesthesiology, University of Toronto, Toronto, ON, Canada

    Peter Slinger

Case Discussion

Case Discussion

Case

A 32-year-old female presents for preoperative assessment. She is booked for a transcervical thymectomy for MG. In addition to the usual preoperative assessment, consider:

  • What additional information, specific to this patient, should be gathered at the preoperative anesthesia consultation?

  • Appropriate investigations

  • How will you optimize her for her upcoming surgery?

  • What premedication will she require including dosing of her current medication?

  • Appropriate preoperative disposition

  • Your anesthetic technique of choice including acute postoperative pain management

What Additional Information, Specific to This Patient, Should Be Gathered at the Preoperative Anesthesia Consultation?

In addition to the usual elements of a preoperative consultation, the preoperative visit should serve to characterize the severity of MG in this patient as well as determine if there are any mediastinal compressive symptoms. A history of associated autoimmune disorders should also be sought.

Determine when the patient was diagnosed with MG and how the disease has progressed. Determine if the symptoms have been purely ocular or if they have involved bulbar muscles (putting the patient at risk for dysphagia and aspiration), axial or limb muscles, or if the patient has had any episodes of respiratory failure requiring ventilator support. Determine the management history, particularly the dose and frequency of pyridostigmine and what consequences are of delaying a dose. Is there a history of steroid requirements or the use of any other immunosuppressant drugs?

A history of palpitations, chest pain, or dyspnea on exertion as well as findings consistent with heart failure should be sought due to the possibility of cardiac involvement. The association with thyroid dysfunction, rheumatoid arthritis, and lupus should prompt screening questions which may or may not require additional medical consultation and optimization.

To screen for a thymoma with mass effect , a history of orthopnea, supine dyspnea, or cough should be sought. Changes in voice (dysphonia, hoarseness), palpitations, syncope, edema of the face or tongue, and dysphagia are other signs of mediastinal compression; however, dysphagia and dysphonia may also be due to MG.

This patient has been diagnosed with MG for 5 years. She initially presented with oculobulbar symptoms which were well controlled with pyridostigmine. Over the last few years, increasing doses have been required eventually prompting referral for surgical treatment. She does not have any associated autoimmune disorders and denies all symptoms of mass compression. To her knowledge, there is no thymoma present. She takes 80 mg of pyridostigmine every 4 h and feels weakness if she is more than an hour later with her dose. Her usual first dose is around 6 o’clock in the morning.

Appropriate Investigations

Bloodwork: CBC (pernicious anemia) and crossmatch are mandatory. Strongly consider electrolytes and creatinine, coagulation profile, and TSH as well.

Imaging must be reviewed, CXR and chest CT for the presence of thymoma. Bloodwork in this patient is all within normal limits. Her TSH is normal. The radiologist’s reports do not mention any sign of aspiration pneumonia nor any thymoma causing compression of cardiopulmonary structures.

How Will You Optimize Her for Her Upcoming Surgery?

Confirm that the patient’s neurologist is aware of the upcoming surgery and discuss possible plasmapheresis or IVIG to optimize the patient’s muscle strength and minimize the chances of postoperative respiratory failure and the need for postoperative ventilation. Pyridostigmine should be continued up to the day of surgery, and the surgery should be scheduled first thing in the morning. The patient should be instructed to take their morning dose of pyridostigmine as close to the time of surgery as possible. If surgery takes longer than expected, an intravenous supplemental anticholinesterase may be given or an additional dose of oral pyridostigmine may be given via nasogastric tube. No premedication with sedating effects should be ordered.

What Is the Anesthetic Technique of Choice Including Postoperative Pain Management ?

General anesthesia with endotracheal intubation is the technique of choice under these circumstances. Induction with propofol and remifentanil is recommended (no neuromuscular blockade). Maintenance may be achieved with a volatile such as desflurane or with a propofol infusion. Remifentanil may be used as an analgesic infusion. Pre- or intraoperative acetaminophen and nonsteroidal anti-inflammatory (such as Naprosyn or ketorolac) should also be used and continued postoperatively. The surgeon should also be asked to infiltrate the wound with local anesthetic solution at closure.

What Is the Most Appropriate Postoperative Disposition?

Patients undergoing transcervical thymectomy for nonthymomatous MG have significantly less pain and respiratory dysfunction than patients undergoing transsternal thymectomy. Provided that the patient can be optimized preoperatively with plasmapheresis or equivalent, an overnight stay in a regular ward is appropriate. Pain is likely to be manageable with oral opioids, and multimodal analgesia with acetaminophen and anti-inflammatories will be beneficial. Based on the history, optimization, and low analgesic requirements, the risk of postoperative respiratory failure is low. Pyridostigmine dosing must be continued, and ongoing collaboration with neurology and the postoperative care team is important.

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Sellers, D., McRae, K. (2019). Thymic Surgery and Paraendocrine Syndromes. In: Slinger, P. (eds) Principles and Practice of Anesthesia for Thoracic Surgery. Springer, Cham. https://doi.org/10.1007/978-3-030-00859-8_15

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  • DOI: https://doi.org/10.1007/978-3-030-00859-8_15

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