Anesthetic management of a parturient with Ehlers Danlos syndrome type IV

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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“…24 However, epidural blocks have been shown to be safe and efficacious for obstetric patients with EDS. 10 …”

Section: Other Considerationsmentioning

confidence: 99%

Ehlers-Danlos Syndrome in Orthopaedics

Shirley

DeMaio

Bodurtha³

2012

Sports Health

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“…В литературе описаны случаи эрозии сосудистой стенки в месте стояния катетера, осложненные эффузией крови в плевральную полость и перикард. Ввиду этого катетеры необходимо удалять как можно скорее [25]. Также персонал должен быть ориентирован на максимально прецизионную и бережную интубацию, ввиду склонности больных к кровоточивости и травматизации тканей.…”

Section: Discussionunclassified

Diagnosis and surgical treatment of patients with vascular type of Ehlers—Danlos syndrome

Charchyan¹,

Stepanenko²,

Breshenkov³

et al. 2017

Kardiol. serdechno-sosud. khir.

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Отделение кардиохирургическое (хирургии аорты и ее ветвей); 2 лаборатория медицинской генетики; 3 патологоанатомическое отделение Российского научного центра хирургии им. акад. Б.В. Петровского, Москва, Россия Ключевые слова: сосудистый тип синдрома Элерса-Данло, диагностика и хирургическое лечение.

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“…Risks include rupture of the uterus or major vessels in pregnancy and the peripartum period, premature rupture of membranes and severe lacerations of the perineum at delivery. Epidural and spinal anaesthesia have been used successfully in vascular type EDS without complication 8,9 . The theoretically increased risk of neuraxial haematoma due to a tendency to bleed excessively may make the use of smaller spinal needles potentially safer than epidurals; however difficulties with haemostasis can potentially prolong surgery beyond the duration of a single-shot spinal which may then expose the patient to the further risks of general anaesthesia.…”

Section: Discussionmentioning

confidence: 99%

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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Anesthetic management of a parturient with Ehlers Danlos syndrome type IV

Cited by 33 publications

References 15 publications

Ehlers-Danlos Syndrome in Orthopaedics

Ehlers-Danlos Syndrome in Orthopaedics

Diagnosis and surgical treatment of patients with vascular type of Ehlers—Danlos syndrome

Anaesthetic Implications of Vascular Type Ehlers-Danlos Syndrome

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