Collaborative Establishment of Difficult Vascular Access for General Anesthetic Management of an Adult With Tetra-Amelia: A Case Report

Abstract

Tetra-amelia is a rare congenital disorder characterized by the absence of limbs. We describe the anesthetic management of a 29-year-old woman with tetra-amelia who underwent general anesthesia for tympanomastoidectomy with meatoplasty for an extensive right ear cholesteatoma. Anesthetic challenges related to tetra-amelia include difficult intravenous access, lack of sites for blood pressure monitoring, and possible difficult airway management due to craniofacial anomalies. Our case report focuses on the complex establishment of iliac artery access for invasive blood pressure monitoring by initially cannulating the carotid artery.

Peri‐operative management of an adult with tetra‐amelia undergoing major abdominal surgery

“…Case reports relating to the anaesthetic management of patients with isolated tetra‐amelia have described the use of near infra‐red spectroscopy as an alternative to blood pressure monitoring [3], which may have been a useful adjunct to intra‐operative monitoring in our patient. Other reports describe novel techniques in interventional radiology to gain invasive arterial access for blood pressure monitoring [4]. Although the cause of limb absence is unknown, it is hypothesised to be due to vascular disruption during embryonic formation, often resulting in the subclavian and femoral vessels being underdeveloped or absent [3].…”

“…The published literature regarding tetra-amelia has mainly focused on post-mortem findings and its other associated birth defects [1,2]. Case reports on the provision of anaesthesia for adults with isolated tetra-amelia have described difficulties in blood pressure monitoring, suggesting the use of novel techniques and devices [3,4], or have used regional anaesthetic techniques and clinical observations to mitigate the absence of intra-operative blood pressure monitoring [5]. We are not aware of reports highlighting the other challenges encountered in the peri-operative care of patients with tetra-amelia, such as temperature and fluid management or titration and dosing of analgesic and anaesthetic drugs.…”