Anaesthetic and intensive care management of a patient with Ehlers–Danlos Type IV syndrome after laparotomy

Solan, K.; Davies, P.

doi:10.1111/j.1365-2044.2004.03959.x

Cited by 14 publications

(7 citation statements)

References 11 publications

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“…The vascular type is the result of structural defects in the pro-alpha1(III) chain of collagen type III encoded by COL3A1 . 19,36 Type III collagen is abundant in major blood vessels 46 and is also present in the gastrointestinal tract, uterus, and skin.…”

Section: Etiology and Classificationmentioning

confidence: 99%

Ehlers-Danlos Syndrome in Orthopaedics

Shirley

DeMaio

Bodurtha³

2012

Sports Health

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Ehlers-Danlos syndrome is a heterogeneous connective tissue condition characterized by varying degrees of skin hyperextensibility, joint hypermobility, and vascular fragility. Joint dislocations, musculoskeletal pain, atrophic scars, easy bleeding, vessel/viscera rupture, severe scoliosis, and obstetric complications may occur. These manifestations are secondary to abnormal collagen, with specific molecular defects in types I, III, and V collagen; they may also be related to tenascin-X, which has been identified in some patients. Ehlers-Danlos syndrome has been classified into 6 types, with variable degrees of joint instability, skin hyperextensibility, wound healing difficulty, and vascular fragility. Diagnosis begins with recognition of the signs and symptoms of global hypermobility and referring appropriate patients for genetic consultation. It is important to accurately identify patients with Ehlers-Danlos syndrome to initiate appropriate musculoskeletal treatment, optimize anesthetic and postoperative management, perform appropriate vascular screening, and help families address their concerns with other families and advocacy groups.

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Section: Etiology and Classificationmentioning

confidence: 99%

Ehlers-Danlos Syndrome in Orthopaedics

Shirley

DeMaio

Bodurtha³

2012

Sports Health

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“…However, as with our two cases, few complications with vascular access have been reported. While central venous cannulation was complicated by carotid artery punctures and subsequent erosion through a vein wall in one case report 5 , it has been uneventful in most others 6,7 . No complications of arterial line placement have been reported [5][6][7][8] .…”

Section: Discussionmentioning

confidence: 98%

“…While central venous cannulation was complicated by carotid artery punctures and subsequent erosion through a vein wall in one case report 5 , it has been uneventful in most others 6,7 . No complications of arterial line placement have been reported [5][6][7][8] . In our first case, a decision was made to avoid an arterial cannula because of the potential risks with this procedure in EDS, but in case 2 when the diagnosis was not made for some three months after admission and no such precautions were taken, no complications arose from arterial cannulation.…”

Section: Discussionmentioning

confidence: 98%

Anaesthetic Implications of Vascular Type Ehlers-Danlos Syndrome

Lane

2006

Anaesth Intensive Care

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Vascular type Ehlers-Danlos syndrome is an inherited connective tissue disease, which is typified by tissue fragility, joint hypermobility, a tendency to bleed excessively and rupture of the uterus, the bowel and arteries. Two case reports are presented which describe the anaesthetic management of patients with spontaneous bowel perforations due to vascular type Ehlers-Danlos syndrome. Both cases were associated with relatively minor problems. The implications of this condition for anaesthesia include difficulty (and risk of complications) with vascular access, a risk of spontaneous arterial rupture in the perioperative period, possible cervical spine instability and airway trauma on intubation. There is also a risk of excessive bleeding, potentially difficult neuraxial anaesthesia and an increased risk of epidural haematoma. There also may be associated cardiac or pulmonary disease. However, these two cases and the limited previously published literature suggest that such potential complications are actually rare and that anaesthesia may be conducted in a relatively standard manner in most circumstances.

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“…The use of ultrasound for central venous catheterization is recommended [[32],[33]]. If central lines are needed in EDS patients (high risk surgery, emergencies), ultrasound guidance is mandatory, including visualization of correct wire localization within the blood vessel [[34]]. Use of dilatators should be restricted as they might worsen vascular damage.…”

Section: Discussionmentioning

confidence: 99%

Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)

Wiesmann

Castori

Malfait

et al. 2014

Orphanet J Rare Dis

101

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Ehlers-Danlos syndrome (EDS, ORPHA98249) comprises a group of clinically and genetically heterogeneous heritable connective tissue disorders, chiefly characterized by joint hypermobility and instability, skin texture anomalies, and vascular and soft tissue fragility. As many tissues can be involved, the underlying molecular defect can manifest itself in many organs and with varying degrees of severity, with widespread implications for anesthesia and perioperative management. This review focuses on issues relevant for anesthesia for elective and emergency surgery in EDS. We searched the literature for papers related to all EDS variants; at the moment most of the published data deals with the vascular subtype and, to a lesser extent, classic and hypermobility EDS. Knowledge is fragmented and consists mostly of case reports, small case series and expert opinion. Because EDS patients commonly require surgery, we have summarized some recommendations for general, obstetrical and regional anesthesia, as well as for hemostatic therapy.

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Anaesthetic and intensive care management of a patient with Ehlers–Danlos Type IV syndrome after laparotomy

Abstract: A 31-year-old woman with Ehlers-Danlos Type IV syndrome developed multiple intensive care related complications following laparotomy for perforated bowel. Complications are more likely to occur with the Ehlers-Danlos syndrome.

Cited by 14 publications

References 11 publications

Ehlers-Danlos Syndrome in Orthopaedics

Ehlers-Danlos Syndrome in Orthopaedics

Anaesthetic Implications of Vascular Type Ehlers-Danlos Syndrome

Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)

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