Cesarean Delivery in a Patient With Hypermobile Ehlers-Danlos Syndrome: A Case Report

高活动性埃勒斯-当洛斯综合征患者剖宫产:病例报告

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Abstract

Patients with hypermobile Ehlers-Danlos syndrome (hEDS) usually present with generalized joint hypermobility and pain, soft and hyperextensible skin with atrophic scars and easy bruising, periodontitis, mitral valve prolapse, and aortic root dilation. It may also lead to cervical spine instability or collapse of larynx cartilage/trachea, which can result in intubation difficulties and possible mucosal damage. Lung protective ventilation must be performed to prevent pneumothorax. Bruising, scoliosis, spondylosis, meningeal Tarlov cysts, increased risk for postdural puncture headache (PDPH), and resistance to local anesthetics may affect neuraxial anesthesia. This article aims to report an anesthetic approach of a 24-year-old woman with hEDS proposed for cesarean delivery. Additional past medical history included cutaneous psoriasis, polycystic ovarian syndrome, and bilateral hip dysplasia. No previous allergies were reported. She has been previously submitted to an uneventful general anesthesia. After rapid-sequence induction of general anesthesia, endotracheal intubation was performed with videolaryngoscopy. Balanced anesthesia with volatile anesthetics was used. The birth and recovery were safely managed without adverse events. hEDS can present challenges for both neuraxial anesthesia and orotracheal intubation. In this case, the initial anesthetic plan included general anesthesia and an airway approach with a videolaryngoscope. Patient positioning and padding were carefully executed to prevent bruising and joint dislocations.

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