myasthenia gravis general thoracic surgery chapter 168
TRANSCRIPT
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MYASTHENIA GRAVIS
GENERAL THORACIC SURGERY
CHAPTER 168
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Myasthenia gravis
• A neuromuscular disorder, • Characterized— 1) abnormal fatigability of voluntary
muscle on repetitive activity, and recover by rest.
2) electrophysiologically decremental response to repetitive stimulation by single-fiber electromyography.
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Myasthenia gravis
3) improve by administration of anticholinesterase drugs.
4) association with abnormality of thymus.
5) presence of circulating antibody to AChRs and complement-mediated damage to receptor.
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Clinical picture
• Involvement of various voluntary muscle group.
• Abnormal fatigability on repetitive activity with improvement after rest.
• Progressive worsening symptoms through the day from morning to evening.
• Ocular symptoms as diplopia and ptosis, • Weakness and fatigue transiently.
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Clinical picture
• Bulbar innervated musculature is affected — dysphagia, dysarthria, difficult in mastication, failure of respiratory muscle.
• Most serious symptoms are ventilatory failure.
• In women — the symptoms may affected by pregnancy, menses and stress.
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Electrophysiology
• Single fiber electromyography. • Record the jitter phenomenon. • Jitter is variable temporal separation of the
response of individual muscle fibers of the same motor unit during activation.
• Abnormally variable separation may found more than 80% patients with myasthenia gravis.
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Pharmacologic treatment
• Loewi ( 1932 ) noted acetylcholine is neuromuscular transmission in cardiac muscle, Sir Henry Dale ( 1935 ) noted acetylcholine is liberated at motor nerve ending in voluntary striated muscle—1936 Nobel prize.
• Propagation of action potential down a motor nerve fiber, release acetylcholine from synaptic vesicles, depolarization muscle.
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Pharmacologic treatment
• Only a small fraction of the 30-40 million receptors per neuromuscular junction are activated normally in response to a nerve impulse.
• The receptors excess provide large safety ensure the repetitively neuromuscular transmission.
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Pharmacologic treatment
• Anticholinesterase—physostigmine, neostogmine, pyridostigmine— block the cholinesterase inactivation of acetylcholine,
• Tensilon ( edrophonium chloride ) test—rapid action and rapid subsidence, a basic diagnostic test.
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Pathogenesis and immunobiology
• Simpson (1960) — Autoimmune origin.
• Almon (1974) — Demonstrate circulation antibodies to AChR site of neuromuscular junction.
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Pathogenesis and immunobiology
• Three possible mechanism—
1)Accelerating the degradation of anticholinesterase receptor through the cross-linking phenomenon.
2)Direct blocking receptor site.
3)Actual degradation the receptor site by complement activation.
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Pathogenesis and immunobiology
• Elevated antibody level are found in 90% patient and roughly correlated with clinical severity.
• Immunosuppressive agents as azathioprine, corticosteroid, cyclosporine may have benefit effect.
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Pathology
• Thymoma is present in 10-15% patient with myasthenia gravis.
• Normal 10-25%.
• Other is thymic hyperplasia.
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Thymectomy
• Von Haberer 1917 — transcervical thymectomy because of thymic hyperplasia often found in thyrotoxicosis.
• Blablock 1936— upper sternotomy incision and introduced neostigmine the operation is success.
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Thymectomy
• Carlens (1968), Crile (1965), Akakura (1965) — re-describe the old technique of transcervical thymectomy.
• Papatestas (1987) — perform more than 700 transcervical thymectomy.
• Incomplete thymectomy is the most important problem.
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Thymectomy
• Advantage of transcervical incision– incision only involve soft tissue, rarely enter the pleural space, well tolerated by patients.
• Cooper (1988) —add self-retaining retractor to aid in transcervical exposure and able to extent transternal resection.
• Type of surgical exposure is most important determinant of the extent of resection.
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Thymoma
• 10-15% patient with MG has thymoma.
• 30-50% thymoma are associated with MG.
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Classifications
• Modified Osserman and Genkin classification.
• Oosterhuis classification.
• Result classification.
• Immunobiological classification.
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Present indications for thymectomy
• Patient with thymoma — the thymectomy is indicated all.
• If no thymoma — the patient age, symptoms, duration, severity, response to medication, sex are factors in decision-making.
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Present indications for thymectomy
• Thymectomy is not recommended for the neonatal type of myasthenia gravis.
• In juvenile form — the reserve thymectomy for patient with more severe symptoms and lack of response to medical therapy.
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Present indications for thymectomy
• In adult — Cooper, Jaretzki and Papatestas all believed patient with general symptoms should receive early thymectomy as soon as the diagnosis established.
• The ocular type — may try medical therapy for a year and if the symptoms interfere the daily life, the thymectomy should be considered.
• High incidence of unsuspected thymoma in patient older than 40y/o with ocular symptom only.
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Result
• Adult patient without thymoma undergoing thymectomy has higher incidence of complete remission.
• Complete remove all thymic tissue fom mediastium and lower neck from standard transternal incision is required in surgical treatment of myasthenia gravis.
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