Perioperative Management of Ehlers-Danlos Type III Syndrome Associated With Postural Orthostatic Tachycardia in Patients Undergoing General Anesthesia

Abstract

Ehlers-Danlos syndrome (EDS) is an autosomal dominant inherited disorder of connective tissue with common clinical features of skin hyperelasticity, joint hypermobility, and easy bruising. Postural orthostatic tachycardia syndrome (POTS) refers to more than three months of a sustained increase in heart rate of more than 30 beats per minute and symptoms of orthostatic intolerance within 10 minutes of assuming a standing position without associated hypotension. These medical conditions can be associated with each other, potentially creating significant perioperative challenges. This paper describes two cases of young women with POTS and EDS hypermobility type (III) who presented for surgery under general anesthesia. The anesthesiologist performed an extensive preoperative evaluation, provided adequate preoperative hydration, ensured careful positioning during anesthetic induction, and avoided neck hyperextension during intubation. Gentle emergence and extubation were practiced with vigilance towards complications of cervical subluxation and airway mucosal injury. Robust communication between postoperative caregivers was prioritized. All these considerations facilitated the achievement of good outcomes. Here, a literature review and subsequent flow diagram of the anesthetic management and perioperative considerations for these patients is purposed.

Keywords: anesthesiology; ehlers-danlos syndrome; general anesthesia; perioperative management; postural orthostatic tachycardia syndrome.

Figure 1. Perioperative flow diagram for the management of patients with EDS III and POTS

POTS: Postural Orthostatic Tachycardia Syndrome, EDS III: Ehlers-Danlos Syndrome Type III, ASA: American Society of Anesthesiologists, PACU: Post-Anesthesia Care Unit, ETT: endotracheal tube, IV: Intravenous