Spinal Anesthesia in a Patient With a History of Systemic Capillary Leak Syndrome (Clarkson Disease)

The aim of this study was to review the relative risks, benefits, and anesthetic considerations of neuraxial, regional, and general anesthesia in a patient with known systemic capillary leak syndrome (SCLS) who presents for elective total knee replacement.

Systemic capillary leak syndrome (or Clarkson disease) is a rare disorder involving endothelial dysfunction of uncertain pathogenesis characterized by the development of hypotension, hemoconcentration, and hypoalbuminemia in the absence of secondary causes of shock. Literature regarding the anesthetic management of such patients is extremely sparse. To date, all published cases involved those who experienced exacerbations preoperatively, intraoperatively, or postoperatively. There are no reports pertaining to the anesthetic management of patients with a history of the disease who are under remission. We present a case report of a patient with a history of SCLS who underwent elective unilateral total knee arthroplasty under spinal anesthesia. The patient tolerated the procedure well without any evidence of perioperative capillary leak exacerbation.

Spinal anesthesia constitutes a viable option for a patient with a history of SCLS in sustained remission. It remains unclear whether general or neuraxial anesthesia confers the best risk-to-benefit ratio for such patients.