Ehlers-Danlos syndromes - Revision history

Nkamdar at 22:52, 10 July 2021

2021-07-10T22:52:03Z

← Older revision		Revision as of 15:52, 10 July 2021
Line 67:		Line 67:
	Mutations to genes that code for fibrillar collagen proteins in connective tissue		Mutations to genes that code for fibrillar collagen proteins in connective tissue

	Their are six ~~manor~~ subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal\|last=Parapia\|first=Liakat A.\|last2=Jackson\|first2=Carolyn\|date=2008-04\|title=Ehlers-Danlos syndrome--a historical review\|url=https://pubmed.ncbi.nlm.nih.gov/18324963\|journal=British Journal of Haematology\|volume=141\|issue=1\|pages=32–35\|doi=10.1111/j.1365-2141.2008.06994.x\|issn=1365-2141\|pmid=18324963}}</ref><ref>{{Cite journal\|last=Wiesmann\|first=Thomas\|last2=Castori\|first2=Marco\|last3=Malfait\|first3=Fransiska\|last4=Wulf\|first4=Hinnerk\|date=2014-07-23\|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)\|url=https://doi.org/10.1186/s13023-014-0109-5\|journal=Orphanet Journal of Rare Diseases\|volume=9\|issue=1\|pages=109\|doi=10.1186/s13023-014-0109-5\|issn=1750-1172\|pmc=PMC4223622\|pmid=25053156}}</ref>		Their are six major subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal\|last=Parapia\|first=Liakat A.\|last2=Jackson\|first2=Carolyn\|date=2008-04\|title=Ehlers-Danlos syndrome--a historical review\|url=https://pubmed.ncbi.nlm.nih.gov/18324963\|journal=British Journal of Haematology\|volume=141\|issue=1\|pages=32–35\|doi=10.1111/j.1365-2141.2008.06994.x\|issn=1365-2141\|pmid=18324963}}</ref><ref>{{Cite journal\|last=Wiesmann\|first=Thomas\|last2=Castori\|first2=Marco\|last3=Malfait\|first3=Fransiska\|last4=Wulf\|first4=Hinnerk\|date=2014-07-23\|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)\|url=https://doi.org/10.1186/s13023-014-0109-5\|journal=Orphanet Journal of Rare Diseases\|volume=9\|issue=1\|pages=109\|doi=10.1186/s13023-014-0109-5\|issn=1750-1172\|pmc=PMC4223622\|pmid=25053156}}</ref>

	==Signs and symptoms==		==Signs and symptoms==
Line 74:		Line 74:
	* kyphoscoliosis		* kyphoscoliosis
	* Skin fragility		* Skin fragility
	* Aortic root ~~dilitation~~ causing aortic insufficiency		* Aortic root dilatation causing aortic insufficiency

	==Diagnosis<!-- Describe how this comorbidity is diagnosed. -->==		==Diagnosis<!-- Describe how this comorbidity is diagnosed. -->==

Chris.Rishel: Formatting

2021-07-08T05:59:51Z

Formatting

← Older revision		Revision as of 22:59, 7 July 2021
Line 9:		Line 9:
	\| image =		\| image =
	\| caption =		\| caption =
	}}		}}'''Ehlers-Danlos syndromes''' ('''EDS''') are a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organs. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the underlying connective tissue abnormalities, these patients often present for orthopedic surgery. Overall, there is very little evidence-based knowledge for the management of EDS. Much of the literature is based upon expert opinion.

	Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organs. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the underlying connective tissue abnormalities, these patients often present for orthopedic surgery. Overall, there is very little evidence-based knowledge for the management of EDS. Much of the literature is based upon expert opinion.

	== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==		== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==

Nkamdar: Changed main body of document.

2021-07-03T16:13:07Z

Changed main body of document.

@@ Line 52: / Line 52: @@
 * EDS patients can have pneumothorax after positive pressure ventilation
 * EDS patients with epidurals have greater risk for PDPH and epidural hematoma formation
 ==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. -->==

Nkamdar: Changed main body of document.

2021-07-03T15:57:05Z

Changed main body of document.

← Older revision		Revision as of 08:57, 3 July 2021
Line 1:		Line 1:
	{{Infobox comorbidity		{{Infobox comorbidity
	\| other_names =		\| other_names = EDS
	\| anesthetic_relevance =		\| anesthetic_relevance = Moderate
	\| specialty =		\| anesthetic_management = Care with airway management, invasive monitoring, and positioning.
	\| signs_symptoms =		\| specialty = Vascular, cardiology
	\| diagnosis =		\| signs_symptoms = Joint/skin laxity, joint hypermobility, aortic insufficiency and aortic root dilation
			\| diagnosis = Genetic testing
	\| treatment =		\| treatment =
	\| image =		\| image =
Line 10:		Line 11:
	}}		}}

	Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other ~~organ~~. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the connective tissue abnormalities, these patients often present for orthopedic surgery. Overall, there is very little evidence-based knowledge for the management of EDS. Much of the literature is based upon expert opinion.		Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organs. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the underlying connective tissue abnormalities, these patients often present for orthopedic surgery. Overall, there is very little evidence-based knowledge for the management of EDS. Much of the literature is based upon expert opinion.

	== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==		== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==
Line 17:		Line 18:

	* Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency		* Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency
	* ~~Aortic root~~ dilation ~~may exacerbate~~ aortic ~~insufficiency~~		* Vascular instability may cause major vessel dilation and cause aortic or mitral insufficiency
	* EDS patients can have both aortic and mitral insufficiency

	===Intraoperative management===		===Intraoperative management===
Line 24:		Line 24:
	===== <u>Monitoring</u>: =====		===== <u>Monitoring</u>: =====

	* Arterial lines run the risk of vascular wall dissection. Consider ultrasound placements ~~for EDS patients~~		* Arterial lines run the risk of vascular wall dissection. Consider ultrasound-guided arterial line placements
	* EDS patients can often demonstrate orthostatic tachycardia syndrome (POTS)		* EDS patients can often demonstrate orthostatic tachycardia syndrome (POTS)

	===== <u>Positioning:</u> =====		===== <u>Positioning:</u> =====

	* Eyes should be protected to ~~present~~ retinal detachment and globe rupture		* Eyes should be protected to prevent retinal detachment and globe rupture
	* Careful padding of extremities		* Careful padding of extremities

Line 61:		Line 61:

	==Signs and symptoms==		==Signs and symptoms==
	~~Joint hypermobility/instability~~

	kyphoscoliosis		* Joint hypermobility/instability
			* kyphoscoliosis
	Skin fragility		* Skin fragility
			* Aortic root dilitation causing aortic insufficiency
	Aortic root dilitation causing aortic insufficiency

	==Diagnosis<!-- Describe how this comorbidity is diagnosed. -->==		==Diagnosis<!-- Describe how this comorbidity is diagnosed. -->==

	==Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. -->==		==Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. -->==
			No treatments currently exist

	===Medication<!-- Describe medications used to manage this comorbidity. -->===		===Medication<!-- Describe medications used to manage this comorbidity. -->===
			Incidence is low and hence lacks randomized drug trials

			Drug therapy will be protected under Orphan Drug development policies

	===Surgery<!-- Describe surgical procedures used to treat this comorbidity. -->===		===Surgery<!-- Describe surgical procedures used to treat this comorbidity. -->===

Nkamdar at 15:45, 3 July 2021

2021-07-03T15:45:11Z

← Older revision		Revision as of 08:45, 3 July 2021
Line 10:		Line 10:
	}}		}}

	Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the connective tissue abnormalities, these patients often present for orthopedic surgery. Overall, there is very little evidence-based knowledge for the management of EDS. Much of the literature is based upon expert opinion. Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal\|last=Parapia\|first=Liakat A.\|last2=Jackson\|first2=Carolyn\|date=2008-04\|title=Ehlers-Danlos syndrome--a historical review\|url=https://pubmed.ncbi.nlm.nih.gov/18324963\|journal=British Journal of Haematology\|volume=141\|issue=1\|pages=32–35\|doi=10.1111/j.1365-2141.2008.06994.x\|issn=1365-2141\|pmid=18324963}}</ref><ref>{{Cite journal\|last=Wiesmann\|first=Thomas\|last2=Castori\|first2=Marco\|last3=Malfait\|first3=Fransiska\|last4=Wulf\|first4=Hinnerk\|date=2014-07-23\|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)\|url=https://doi.org/10.1186/s13023-014-0109-5\|journal=Orphanet Journal of Rare Diseases\|volume=9\|issue=1\|pages=109\|doi=10.1186/s13023-014-0109-5\|issn=1750-1172\|pmc=PMC4223622\|pmid=25053156}}</ref>		Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the connective tissue abnormalities, these patients often present for orthopedic surgery. Overall, there is very little evidence-based knowledge for the management of EDS. Much of the literature is based upon expert opinion.

	== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==		== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==
Line 57:		Line 57:
	==Pathophysiology==		==Pathophysiology==
	Mutations to genes that code for fibrillar collagen proteins in connective tissue		Mutations to genes that code for fibrillar collagen proteins in connective tissue

			Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal\|last=Parapia\|first=Liakat A.\|last2=Jackson\|first2=Carolyn\|date=2008-04\|title=Ehlers-Danlos syndrome--a historical review\|url=https://pubmed.ncbi.nlm.nih.gov/18324963\|journal=British Journal of Haematology\|volume=141\|issue=1\|pages=32–35\|doi=10.1111/j.1365-2141.2008.06994.x\|issn=1365-2141\|pmid=18324963}}</ref><ref>{{Cite journal\|last=Wiesmann\|first=Thomas\|last2=Castori\|first2=Marco\|last3=Malfait\|first3=Fransiska\|last4=Wulf\|first4=Hinnerk\|date=2014-07-23\|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)\|url=https://doi.org/10.1186/s13023-014-0109-5\|journal=Orphanet Journal of Rare Diseases\|volume=9\|issue=1\|pages=109\|doi=10.1186/s13023-014-0109-5\|issn=1750-1172\|pmc=PMC4223622\|pmid=25053156}}</ref>

	==Signs and symptoms==		==Signs and symptoms==

Nkamdar at 00:24, 3 July 2021

2021-07-03T00:24:42Z

← Older revision		Revision as of 17:24, 2 July 2021
Line 15:		Line 15:

	===Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. -->===		===Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. -->===
	~~Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency~~

	Aortic root dilation may exacerbate aortic insufficiency		* Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency
			* Aortic root dilation may exacerbate aortic insufficiency
	EDS patients can have both aortic and mitral insufficiency		* EDS patients can have both aortic and mitral insufficiency

	===Intraoperative management===		===Intraoperative management===
Line 41:		Line 40:

	===== <u>Airway Management:</u> =====		===== <u>Airway Management:</u> =====
	~~Care should be taken for TMJ joint laxity during laryngoscopy~~

	EDS patients may present with occipitalatlantoaxial instability causing TMJ joint dislocation		* Care should be taken for TMJ joint laxity during laryngoscopy
			* EDS patients may present with occipitalatlantoaxial instability causing TMJ joint dislocation

	===Postoperative management===		===Postoperative management===
	~~Early mobilization is helpful to prevent muscle loss~~

	Inspect surgical sites for hematomas or bleeding		* Early mobilization is helpful to prevent muscle loss
			* Inspect surgical sites for hematomas or bleeding
			* Patients with POTS should be monitored early after surgery for bleeding and cardiovascular instability
			* Treat PONV aggressively to avoid esophageal rupture from violent vomiting
			* EDS patients can have pneumothorax after positive pressure ventilation
			* EDS patients with epidurals have greater risk for PDPH and epidural hematoma formation

	~~Patients with POTS should~~ be ~~monitored early after surgery for bleeding and cardiovascular instability~~		==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. -->==

	~~Treat PONV aggressively~~ to ~~avoid esophageal rupture from violent vomiting~~

	~~EDS patients can have pneumothorax after positive pressure ventilation~~		==Pathophysiology==
			Mutations to genes that code for fibrillar collagen proteins in connective tissue

	~~EDS patients with epidurals have greater risk for PDPH~~ and ~~epidural hematoma formation~~		==Signs and symptoms==
			Joint hypermobility/instability

	~~==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. -->==~~		kyphoscoliosis

	~~==Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. -->==~~		Skin fragility

	~~==Signs and symptoms<!-- Describe the signs and symptoms of this comorbidity. -->==~~		Aortic root dilitation causing aortic insufficiency

	==Diagnosis<!-- Describe how this comorbidity is diagnosed. -->==		==Diagnosis<!-- Describe how this comorbidity is diagnosed. -->==

Nkamdar: Changed main body of document.

2021-07-03T00:14:28Z

Changed main body of document.

← Older revision		Revision as of 17:14, 2 July 2021
Line 10:		Line 10:
	}}		}}

	Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the connective tissue abnormalities, these patients often present for orthopedic surgery. Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal\|last=Parapia\|first=Liakat A.\|last2=Jackson\|first2=Carolyn\|date=2008-04\|title=Ehlers-Danlos syndrome--a historical review\|url=https://pubmed.ncbi.nlm.nih.gov/18324963\|journal=British Journal of Haematology\|volume=141\|issue=1\|pages=32–35\|doi=10.1111/j.1365-2141.2008.06994.x\|issn=1365-2141\|pmid=18324963}}</ref><ref>{{Cite journal\|last=Wiesmann\|first=Thomas\|last2=Castori\|first2=Marco\|last3=Malfait\|first3=Fransiska\|last4=Wulf\|first4=Hinnerk\|date=2014-07-23\|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)\|url=https://doi.org/10.1186/s13023-014-0109-5\|journal=Orphanet Journal of Rare Diseases\|volume=9\|issue=1\|pages=109\|doi=10.1186/s13023-014-0109-5\|issn=1750-1172\|pmc=PMC4223622\|pmid=25053156}}</ref>		Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the connective tissue abnormalities, these patients often present for orthopedic surgery. Overall, there is very little evidence-based knowledge for the management of EDS. Much of the literature is based upon expert opinion. Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal\|last=Parapia\|first=Liakat A.\|last2=Jackson\|first2=Carolyn\|date=2008-04\|title=Ehlers-Danlos syndrome--a historical review\|url=https://pubmed.ncbi.nlm.nih.gov/18324963\|journal=British Journal of Haematology\|volume=141\|issue=1\|pages=32–35\|doi=10.1111/j.1365-2141.2008.06994.x\|issn=1365-2141\|pmid=18324963}}</ref><ref>{{Cite journal\|last=Wiesmann\|first=Thomas\|last2=Castori\|first2=Marco\|last3=Malfait\|first3=Fransiska\|last4=Wulf\|first4=Hinnerk\|date=2014-07-23\|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)\|url=https://doi.org/10.1186/s13023-014-0109-5\|journal=Orphanet Journal of Rare Diseases\|volume=9\|issue=1\|pages=109\|doi=10.1186/s13023-014-0109-5\|issn=1750-1172\|pmc=PMC4223622\|pmid=25053156}}</ref>

	== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==		== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==
Line 16:		Line 16:
	===Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. -->===		===Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. -->===
	Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency		Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency

			Aortic root dilation may exacerbate aortic insufficiency

			EDS patients can have both aortic and mitral insufficiency

	===Intraoperative management===		===Intraoperative management===

	===== <u>Monitoring</u>: =====		===== <u>Monitoring</u>: =====

			* Arterial lines run the risk of vascular wall dissection. Consider ultrasound placements for EDS patients
			* EDS patients can often demonstrate orthostatic tachycardia syndrome (POTS)

	===== <u>Positioning:</u> =====		===== <u>Positioning:</u> =====
Line 29:		Line 36:

	* Minor forces can result in skin injuries such as tape, stickers, and other monitors that attach to the patient		* Minor forces can result in skin injuries such as tape, stickers, and other monitors that attach to the patient
	* Bruising and hematoma formation occurs with non-invasive BP monitoring		* Bruising and hematoma formation occurs with repetitive non-invasive BP monitoring
			* Special attention to removing tape from eyes and skin
			* Discuss the risk/benefits of using a tourniquet in these patients with surgical team

			===== <u>Airway Management:</u> =====
			Care should be taken for TMJ joint laxity during laryngoscopy

			EDS patients may present with occipitalatlantoaxial instability causing TMJ joint dislocation

			===Postoperative management===
			Early mobilization is helpful to prevent muscle loss

			Inspect surgical sites for hematomas or bleeding

			Patients with POTS should be monitored early after surgery for bleeding and cardiovascular instability

			Treat PONV aggressively to avoid esophageal rupture from violent vomiting

			EDS patients can have pneumothorax after positive pressure ventilation

	~~===Postoperative management<!-- Describe how this comorbidity may influence postoperative management. -->===~~		EDS patients with epidurals have greater risk for PDPH and epidural hematoma formation

	==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. -->==

Nkamdar: Changed main body of document.

2021-07-02T20:39:45Z

Changed main body of document.

@@ Line 17: / Line 17: @@
 Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency
-===Intraoperative management<!-- Describe how this comorbidity may influence intraoperative management. -->===
+===Intraoperative management===
 ===Postoperative management<!-- Describe how this comorbidity may influence postoperative management. -->===

Nkamdar at 20:33, 2 July 2021

2021-07-02T20:33:31Z

← Older revision		Revision as of 13:33, 2 July 2021
Line 10:		Line 10:
	}}		}}

	Ehlers-Danlos is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal\|last=Parapia\|first=Liakat A.\|last2=Jackson\|first2=Carolyn\|date=2008-04\|title=Ehlers-Danlos syndrome--a historical review\|url=https://pubmed.ncbi.nlm.nih.gov/18324963\|journal=British Journal of Haematology\|volume=141\|issue=1\|pages=32–35\|doi=10.1111/j.1365-2141.2008.06994.x\|issn=1365-2141\|pmid=18324963}}</ref><ref>{{Cite journal\|last=Wiesmann\|first=Thomas\|last2=Castori\|first2=Marco\|last3=Malfait\|first3=Fransiska\|last4=Wulf\|first4=Hinnerk\|date=2014-07-23\|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)\|url=https://doi.org/10.1186/s13023-014-0109-5\|journal=Orphanet Journal of Rare Diseases\|volume=9\|issue=1\|pages=109\|doi=10.1186/s13023-014-0109-5\|issn=1750-1172\|pmc=PMC4223622\|pmid=25053156}}</ref>		Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the connective tissue abnormalities, these patients often present for orthopedic surgery. Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal\|last=Parapia\|first=Liakat A.\|last2=Jackson\|first2=Carolyn\|date=2008-04\|title=Ehlers-Danlos syndrome--a historical review\|url=https://pubmed.ncbi.nlm.nih.gov/18324963\|journal=British Journal of Haematology\|volume=141\|issue=1\|pages=32–35\|doi=10.1111/j.1365-2141.2008.06994.x\|issn=1365-2141\|pmid=18324963}}</ref><ref>{{Cite journal\|last=Wiesmann\|first=Thomas\|last2=Castori\|first2=Marco\|last3=Malfait\|first3=Fransiska\|last4=Wulf\|first4=Hinnerk\|date=2014-07-23\|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)\|url=https://doi.org/10.1186/s13023-014-0109-5\|journal=Orphanet Journal of Rare Diseases\|volume=9\|issue=1\|pages=109\|doi=10.1186/s13023-014-0109-5\|issn=1750-1172\|pmc=PMC4223622\|pmid=25053156}}</ref>

	== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==		== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==

Kevin.Youssefzadeh: started working on the page

2021-07-02T19:17:19Z

started working on the page

← Older revision		Revision as of 12:17, 2 July 2021
Line 10:		Line 10:
	}}		}}

	~~Provide~~ a ~~brief summary~~ of ~~this comorbidity here~~.		Ehlers-Danlos is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal\|last=Parapia\|first=Liakat A.\|last2=Jackson\|first2=Carolyn\|date=2008-04\|title=Ehlers-Danlos syndrome--a historical review\|url=https://pubmed.ncbi.nlm.nih.gov/18324963\|journal=British Journal of Haematology\|volume=141\|issue=1\|pages=32–35\|doi=10.1111/j.1365-2141.2008.06994.x\|issn=1365-2141\|pmid=18324963}}</ref><ref>{{Cite journal\|last=Wiesmann\|first=Thomas\|last2=Castori\|first2=Marco\|last3=Malfait\|first3=Fransiska\|last4=Wulf\|first4=Hinnerk\|date=2014-07-23\|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)\|url=https://doi.org/10.1186/s13023-014-0109-5\|journal=Orphanet Journal of Rare Diseases\|volume=9\|issue=1\|pages=109\|doi=10.1186/s13023-014-0109-5\|issn=1750-1172\|pmc=PMC4223622\|pmid=25053156}}</ref>

	== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. --> ==		== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->==

	=== Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. --> ===		===Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. -->===
			Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency

	=== Intraoperative management<!-- Describe how this comorbidity may influence intraoperative management. --> ===		===Intraoperative management<!-- Describe how this comorbidity may influence intraoperative management. -->===

	=== Postoperative management<!-- Describe how this comorbidity may influence postoperative management. --> ===		===Postoperative management<!-- Describe how this comorbidity may influence postoperative management. -->===

	== 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. -->==

	== Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. --> ==		==Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. -->==

	== Signs and symptoms<!-- Describe the signs and symptoms of this comorbidity. --> ==		==Signs and symptoms<!-- Describe the signs and symptoms of this comorbidity. -->==

	== Diagnosis<!-- Describe how this comorbidity is diagnosed. --> ==		==Diagnosis<!-- Describe how this comorbidity is diagnosed. -->==

	== Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. --> ==		==Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. -->==

	=== Medication<!-- Describe medications used to manage this comorbidity. --> ===		===Medication<!-- Describe medications used to manage this comorbidity. -->===

	=== Surgery<!-- Describe surgical procedures used to treat this comorbidity. --> ===		===Surgery<!-- Describe surgical procedures used to treat this comorbidity. -->===

	=== Prognosis<!-- Describe the prognosis of this comorbidity --> ===		===Prognosis<!-- Describe the prognosis of this comorbidity -->===

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

	== References ==		==References==

	[[Category:Comorbidities]]		[[Category:Comorbidities]]
			<references />