Duchenne muscular dystrophy - Revision history

Mitchel.DeVita: added content

2022-06-02T06:14:29Z

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← Older revision		Revision as of 23:14, 1 June 2022
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	{{Infobox comorbidity		{{Infobox comorbidity
	\| other_names =		\| other_names = DMD
	\| image =		\| image =
	\| caption =		\| caption =
	\| anesthetic_relevance =		\| anesthetic_relevance = High
			\| anesthetic_management = High risk for hyperkalemia, rhabdomyolysis, arrhythmia, difficult airway, respiratory insufficiency, and cardiomyopathy
	\| specialty =		\| specialty =
	\| signs_symptoms =		\| signs_symptoms = Proximal muscle weakness, respiratory insufficiency, and cardiomyopathy
	\| diagnosis =		\| diagnosis =
	\| treatment =		\| treatment =
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	-Rocuronium is also considered safe however delayed onset of action and prolonged duration have been reported <ref>Bhutia MP, Pandia MP, Rai A. Anaesthetic management of a case of Duchenne muscle dystrophy with Moyamoya disease. ''Indian J Anaesth''. 2014;58(2):219-221. doi:10.4103/0019-5049.130843</ref>		-Rocuronium is also considered safe however delayed onset of action and prolonged duration have been reported <ref>Bhutia MP, Pandia MP, Rai A. Anaesthetic management of a case of Duchenne muscle dystrophy with Moyamoya disease. ''Indian J Anaesth''. 2014;58(2):219-221. doi:10.4103/0019-5049.130843</ref>

	-Sugammadex use has not been fully established however it has minimal adverse cardiovascular events ~~and is likely safe~~. In direct contrast, neostigmine may trigger acute ~~myotony~~, rhabdomyolysis, and arrhythmia.		-Sugammadex use has not been fully established however it has minimal adverse cardiovascular events. In direct contrast, neostigmine may trigger acute myotonia, rhabdomyolysis, and arrhythmia.

	-The risk of malignant hyperthermia is similar to that of the general population <ref>Birnkrant DJ, Panitch HB, Benditt JO, et al. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. ''Chest'' 2007; 132:1977–1986.</ref>		-The risk of malignant hyperthermia is similar to that of the general population <ref>Birnkrant DJ, Panitch HB, Benditt JO, et al. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. ''Chest'' 2007; 132:1977–1986.</ref>. The clinical presentation can be similar to MH (muscle rigidity, hyperkalemia, arrhythmia, cardiac arrest) however it fails to respond to dantrolene.

	===Postoperative management<!-- Describe how this comorbidity may influence postoperative management. -->===		===Postoperative management<!-- Describe how this comorbidity may influence postoperative management. -->===
			-High sensitivity to opiates and higher risk for post op respiratory depression

			-NSAIDs should be used carefully due to the risk of triggering rhabdomyolysis

			-Consider transporting to ICU intubated, delaying extubation 24 to 48 hours, or consider the use of non-invasive mechanical ventilation

	==Related surgical procedures<!-- List and briefly describe any procedures which may be performed specifically to treat this comorbidity or its sequelae. If none, this section may be removed. -->==		==Related surgical procedures<!-- List and briefly describe any procedures which may be performed specifically to treat this comorbidity or its sequelae. If none, this section may be removed. -->==
			Surgical airway including tracheostomy

	==Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. -->==		==Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. -->==
	X linked recessive, progressive myopathy resulting form a dystrophin gene mutation. Located on chromosome Xp21. Typically diagnosed during the ages of 3 to 5 and most commonly in males. ~~Survival of these patients is generally only 20 to 30 years, and they ultimately succumb to ventilatory failure or heart failure.~~		X linked recessive, progressive myopathy resulting form a dystrophin gene mutation. Located on chromosome Xp21. Typically diagnosed during the ages of 3 to 5 and most commonly in males.

	== Signs and symptoms ==		== Signs and symptoms ==
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	===Prognosis<!-- Describe the prognosis of this comorbidity -->===		===Prognosis<!-- Describe the prognosis of this comorbidity -->===
			Survival of these patients is generally only 20 to 30 years, and they ultimately succumb to ventilatory failure or heart failure.

	==Epidemiology<!-- Describe the epidemiology of this comorbidity -->==		==Epidemiology<!-- Describe the epidemiology of this comorbidity -->==

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 ===Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. -->===
 ===Intraoperative management<!-- Describe how this comorbidity may influence intraoperative management. -->===
 ===Postoperative management<!-- Describe how this comorbidity may influence postoperative management. -->===
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 The patients have associated difficult airway anatomy in the form of macroglossia and limited mobility of mandible and cervical spine. They also can develop contractures of bilateral upper and lower extremities.
 ==Diagnosis<!-- Describe how this comorbidity is diagnosed. -->==

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{{Infobox comorbidity
| other_names =
| image =
| caption =
| anesthetic_relevance =
| specialty =
| signs_symptoms =
| diagnosis =
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}}Duchenne Muscular Dystrophy (DMD) is an X linked recessive, progressive myopathy resulting form a dystrophin gene mutation. Despite the rarity of this disease it represents a significant anesthetic challenge for both pediatric and adult patients.

==Anesthetic implications==

===Preoperative optimization===

===Intraoperative management===

===Postoperative management===

==Related surgical procedures==

==Pathophysiology==
X linked recessive, progressive myopathy resulting form a dystrophin gene mutation. Located on chromosome Xp21. Typically diagnosed during the ages of 3 to 5 and most commonly in males. Survival of these patients is generally only 20 to 30 years, and they ultimately succumb to ventilatory failure or heart failure.

== Signs and symptoms ==
Typically patients present with progressive proximal muscle weakness at a young age, first manifested as weakness and growth delay. This eventually develops into diaphragmatic weakness and subsequent respiratory distress.

Patients develop florid respiratory distress and cardiomyopathy in advanced stage of the disease.

The patients have associated difficult airway anatomy in the form of macroglossia and limited mobility of mandible and cervical spine. They also can develop contractures of bilateral upper and lower extremities.

These patients are at an increased risk of developing extreme hyperthermia, rhabdomyolysis and hyperkalemic cardiac arrest when exposed to halogenated inhalational anesthetics and depolarizing muscle relaxants

==Diagnosis==

==Treatment==

===Medication===

===Surgery===

===Prognosis===

==Epidemiology==

==References==

[[Category:Comorbidities]]