Anaesthetic Management in Duchenne Muscular Dystrophy Patient with TIVA Using Combination of Propofol and Dexmedetomidine Complimented with USG Guided ESPB Block- A Case Report
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Abstract
Muscular dystrophies are a group of genetic diseases which cause progressive degeneration of skeletal muscle along with weakness. They are characterized by an impaired synthesis or regeneration of contractile protein. Patients with muscular dystrophies possess high preoperative risk with significant anaesthetic implications (hyperkalaemia, rhabdomyolysis, cardiovascular instability, sudden death, etc.). We herein describe the anaesthetic management of a 31yr old male who was a known case of Duchenne muscular dystrophy posted for right percutaneous nephrolithotomy (PCNL). The patient was induced and maintained with TIVA and using nitrous oxide as the only inhalational agent supplemented with USG guided Erector spinae plane block (ESPB) for post-operative analgesia. A thorough preoperative evaluation and multidisciplinary approach is essential for perioperative management of such cases.
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[2] Wefki Abdelgawwad Shousha AA, Sanfilippo M, Sabba A, Pinchera P. Sugammadex and reversal of neuromuscular block in adult patient with duchenne muscular dystrophy. Case Rep Anesthesiol. 2014; 2014:680568.
[3] Kaur H, Singh G, Saini N, Singh G, Singh A. Duchenne muscular dystrophy and anesthesia: revisited. Res Opin Anesth Intensive Care. 2021; 8(1):60-2
[4] Marín E, Ceciliaa P, Bustamante-Vega, Maríab A. Anesthetic implications of muscular dystrophies. Rev Col Anest. 2018; 46(3):228-239.
[5] Halsall PJ, Hopkins PM. Malignant hyperthermia. Br J Anaesth CEPD Rev. 2003; 3:5-9.
[6] Keifer HM, Singer WD, Reynolds RN. Malignant hyperthermia in a child with Duchenne muscular dystrophy. Pediatrics. 1983; 71:118–9.
[7] Kleopa KA, Rosenberg H, Heiman-Patterson T. Malignant hyperthermia-like episode in Becker muscular dystrophy. Anesthesiology. 2000; 93:1535–7.
[8] Azar I. The Response of Patients with Neuromuscular Disorders to Muscle Relaxants: A Review. Anesthesiology. 1984; 61:173-187.
[9] Kulshrestha A, Bajwa SJ, Singh A, Kapoor V. Dexmedetomidine and fentanyl combination for procedural sedation in a case of Duchenne muscular dystrophy. Anesth Essays Res. 2011; 5(2):224-226.
[10] Ishizawa Y, Yamaguchi H, Dohi S, Koyama K. A serious complication due to gastrointestinal malfunction in a patient with myotonic dystrophy. Anesth Analg 1986; 65:1066–1068.
[11] Diefenbach C, Lynch J, Abel M, Buzello W. Vecuronium for muscle relaxation in patients with dystrophia myotonica. Anesth Analg. 1993; 76(4):872–4.
[12] Kosinova M, Stourac P, Harazim H, Janku P, Huser M, Vohanka S. Anaesthesia and orphan disease: rocuronium and sugammadex in the anaesthetic management of a parturient with Becker's myotonia congenital. Eur J Anaesthesiol. 2016; 33(7):545-7.
[13] Gronert GA, Fowler W, Cardinet GHIII, GrixAJr, Ellis WG, Schwartz MZ. Absence of malignant hyperthermia contractures in Becker-Duchenne dystrophy at age 2. Muscle Nerve. 1992; 15(1): 52-6.
[14] Muenster T, Mueller C, Forst J, Huber H, Schmitt HJ. Anaesthetic management in patients with Duchenne muscular dystrophy undergoing orthopaedic surgery: A review of 232 cases. Eur J Anaesthesiol. 2012; 29(10):489-94.
[15] Wick S, Muenster T, Schmidt J, Forst J, Schmitt HJ. Onset and duration of rocuronium-induced neuromuscular blockade in patients with Duchenne muscular dystrophy. Anesthesiology. 2005; 102(5):915-9
[16] Ririe DG, Shapiro F, Sethna NF. The response of patients with Duchenne's muscular dystrophy to neuromuscular blockade with vecuronium. Anesthesiology. 1998; 88:351-4.
[17] Liu X, Huang G, Zhong R, Hu S, Deng R. Comparison of Percutaneous Nephrolithotomy Under Regional versus General Anesthesia: A Meta-Analysis of Randomized Controlled Trials. Urol Int. 2018; 101:132-142.
[18] Bennun M, Goldstein B, Finkelstein Y, Jedeikin R. Continuous propofol anaesthesia for patients with myotonic dystrophy. Br J Anaesth. 2000; 85(3):407-9.
[19] Rozmiarek A, Corridore M, Tobias JD. Dexmedetomidine-ketamine sedation during bone marrow aspirate and biopsy in a patient with duchenne muscular dystrophy. Saudi J Anaesth. 2011; 5(2):219-22.
[20] Kako H, Corridore M, Kean J, Mendell JR, Flanigan KM, Tobias JD. Dexmedetomidine and ketamine sedation for muscle biopsies in patients with Duchenne muscular dystrophy. Paediatr Anaesth. 2014; 24(8):851-6.
[21] Gaszynski T. Opioid-free general anesthesia in patient with Steinert syndrome (myotonic dystrophy): Case report. Medicine (Baltimore). 2016; 95(37):e4885.
[22] Brett K, Farrah K. Sugammadex for the Reversal of Rocuronium-Induced Neuromuscular Blockade in Surgical Patients: A Review of Clinical Effectiveness [Internet]. Ottawa (ON): Canadian Agency for Drugs and Technologies in Health; 2019.
[23] Chuang MC, Duggan LV, van Heest RD, MacLeod W. Laparoscopic cholecystectomy under spinal anesthesia in a patient with limb-girdle muscular dystrophy. Can J Anaesth. 2013; 60(12):1276-7.
[24] Munster T. Distrofia muscular de Duchenne. 2011; [Cited 2017 Feb 6]. Available at: http://www.orphananesthesia.eu/en/component/ docman/doc_download/131-distrofia-muscular-de-duchenne.html.
[25] Van Obbergh LJ, Corteel J, Papadopoulos J, et al. Anesthesia for a child suffering from a deletion in the Xp21 loci resulting in Duchenne disease, glycerol kinase deficiency and congenital adrenal hypoplasia. Paediatr Anaesth. 2011; 21(10):1085-7.
[26] Kumar G S S, Balakundi P, Deo A. ESRA19-0663 A new indication of erector spinae plane block for perioperative analgesia is percutaneous nephrolithotomy (PCNL) surgery: case series. Reg Anesth Pain Med. 2019; 44:A254-A255.
[27] Ibrahim M, Elnabtity AM. Analgesic efficacy of erector spinae plane block in percutaneous nephrolithotomy : A randomized controlled trial. Anaesthesist. 2019; 68(11):755-761. English.
[2] Wefki Abdelgawwad Shousha AA, Sanfilippo M, Sabba A, Pinchera P. Sugammadex and reversal of neuromuscular block in adult patient with duchenne muscular dystrophy. Case Rep Anesthesiol. 2014; 2014:680568.
[3] Kaur H, Singh G, Saini N, Singh G, Singh A. Duchenne muscular dystrophy and anesthesia: revisited. Res Opin Anesth Intensive Care. 2021; 8(1):60-2
[4] Marín E, Ceciliaa P, Bustamante-Vega, Maríab A. Anesthetic implications of muscular dystrophies. Rev Col Anest. 2018; 46(3):228-239.
[5] Halsall PJ, Hopkins PM. Malignant hyperthermia. Br J Anaesth CEPD Rev. 2003; 3:5-9.
[6] Keifer HM, Singer WD, Reynolds RN. Malignant hyperthermia in a child with Duchenne muscular dystrophy. Pediatrics. 1983; 71:118–9.
[7] Kleopa KA, Rosenberg H, Heiman-Patterson T. Malignant hyperthermia-like episode in Becker muscular dystrophy. Anesthesiology. 2000; 93:1535–7.
[8] Azar I. The Response of Patients with Neuromuscular Disorders to Muscle Relaxants: A Review. Anesthesiology. 1984; 61:173-187.
[9] Kulshrestha A, Bajwa SJ, Singh A, Kapoor V. Dexmedetomidine and fentanyl combination for procedural sedation in a case of Duchenne muscular dystrophy. Anesth Essays Res. 2011; 5(2):224-226.
[10] Ishizawa Y, Yamaguchi H, Dohi S, Koyama K. A serious complication due to gastrointestinal malfunction in a patient with myotonic dystrophy. Anesth Analg 1986; 65:1066–1068.
[11] Diefenbach C, Lynch J, Abel M, Buzello W. Vecuronium for muscle relaxation in patients with dystrophia myotonica. Anesth Analg. 1993; 76(4):872–4.
[12] Kosinova M, Stourac P, Harazim H, Janku P, Huser M, Vohanka S. Anaesthesia and orphan disease: rocuronium and sugammadex in the anaesthetic management of a parturient with Becker's myotonia congenital. Eur J Anaesthesiol. 2016; 33(7):545-7.
[13] Gronert GA, Fowler W, Cardinet GHIII, GrixAJr, Ellis WG, Schwartz MZ. Absence of malignant hyperthermia contractures in Becker-Duchenne dystrophy at age 2. Muscle Nerve. 1992; 15(1): 52-6.
[14] Muenster T, Mueller C, Forst J, Huber H, Schmitt HJ. Anaesthetic management in patients with Duchenne muscular dystrophy undergoing orthopaedic surgery: A review of 232 cases. Eur J Anaesthesiol. 2012; 29(10):489-94.
[15] Wick S, Muenster T, Schmidt J, Forst J, Schmitt HJ. Onset and duration of rocuronium-induced neuromuscular blockade in patients with Duchenne muscular dystrophy. Anesthesiology. 2005; 102(5):915-9
[16] Ririe DG, Shapiro F, Sethna NF. The response of patients with Duchenne's muscular dystrophy to neuromuscular blockade with vecuronium. Anesthesiology. 1998; 88:351-4.
[17] Liu X, Huang G, Zhong R, Hu S, Deng R. Comparison of Percutaneous Nephrolithotomy Under Regional versus General Anesthesia: A Meta-Analysis of Randomized Controlled Trials. Urol Int. 2018; 101:132-142.
[18] Bennun M, Goldstein B, Finkelstein Y, Jedeikin R. Continuous propofol anaesthesia for patients with myotonic dystrophy. Br J Anaesth. 2000; 85(3):407-9.
[19] Rozmiarek A, Corridore M, Tobias JD. Dexmedetomidine-ketamine sedation during bone marrow aspirate and biopsy in a patient with duchenne muscular dystrophy. Saudi J Anaesth. 2011; 5(2):219-22.
[20] Kako H, Corridore M, Kean J, Mendell JR, Flanigan KM, Tobias JD. Dexmedetomidine and ketamine sedation for muscle biopsies in patients with Duchenne muscular dystrophy. Paediatr Anaesth. 2014; 24(8):851-6.
[21] Gaszynski T. Opioid-free general anesthesia in patient with Steinert syndrome (myotonic dystrophy): Case report. Medicine (Baltimore). 2016; 95(37):e4885.
[22] Brett K, Farrah K. Sugammadex for the Reversal of Rocuronium-Induced Neuromuscular Blockade in Surgical Patients: A Review of Clinical Effectiveness [Internet]. Ottawa (ON): Canadian Agency for Drugs and Technologies in Health; 2019.
[23] Chuang MC, Duggan LV, van Heest RD, MacLeod W. Laparoscopic cholecystectomy under spinal anesthesia in a patient with limb-girdle muscular dystrophy. Can J Anaesth. 2013; 60(12):1276-7.
[24] Munster T. Distrofia muscular de Duchenne. 2011; [Cited 2017 Feb 6]. Available at: http://www.orphananesthesia.eu/en/component/ docman/doc_download/131-distrofia-muscular-de-duchenne.html.
[25] Van Obbergh LJ, Corteel J, Papadopoulos J, et al. Anesthesia for a child suffering from a deletion in the Xp21 loci resulting in Duchenne disease, glycerol kinase deficiency and congenital adrenal hypoplasia. Paediatr Anaesth. 2011; 21(10):1085-7.
[26] Kumar G S S, Balakundi P, Deo A. ESRA19-0663 A new indication of erector spinae plane block for perioperative analgesia is percutaneous nephrolithotomy (PCNL) surgery: case series. Reg Anesth Pain Med. 2019; 44:A254-A255.
[27] Ibrahim M, Elnabtity AM. Analgesic efficacy of erector spinae plane block in percutaneous nephrolithotomy : A randomized controlled trial. Anaesthesist. 2019; 68(11):755-761. English.
| Files | ||
| Issue | Vol 8 No 3 (2022): Summer |
|
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/aacc.v8i3.9620 | |
| Keywords | ||
| Duchenne Muscular Dystrophy (DMD) Total Intravenous Anesthesia (TIVA) Erector Spinae Plane Block (ESPB) | ||
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Deshpande J, Chavan P, Jacob M. Anaesthetic Management in Duchenne Muscular Dystrophy Patient with TIVA Using Combination of Propofol and Dexmedetomidine Complimented with USG Guided ESPB Block- A Case Report. Arch Anesth & Crit Care. 2022;8(3):252-256.
