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Anesthetic and obstetric considerations in a parturient with Klippel-Trenaunay syndrome

[Considérations anesthésiques et obstétriques chez une parturiente atteinte du syndrome de Klippel-Trenaunay]

Michael J. Sivaprakasam, MD^* and James A. Dolak, MD PhD

^* From the Departments of Anesthesiology, St. Edward’s Mercy Medical Center, Fort Smith, Arkansas; and
General Anesthesiology, The Cleveland Clinic Foundation, Cleveland, Ohio, USA.

Address correspondence to: Dr. James A. Dolak, Department of General Anesthesiology, E31, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44195, USA. Phone: 216-444-0257; Fax: 216-444-9247; E-mail: dolakj{at}ccf.org

Purpose: To explain the considerations governing the anesthetic management of pregnant patients with Klippel-Trenaunay syndrome (KTS). Klippel-Trenaunay syndrome is a congenital vascular disease characterized by cutaneous hemangiomas, venous varicosities, and limb hypertrophy; and is associated with both hemorrhagic and thrombotic complications. The importance of this diagnosis, including the presence of neuraxial vascular anomalies, is often under-appreciated by both obstetric and anesthesia providers. While regional anesthetic management of patients with KTS has been discussed by others, we present a case in which regional anesthesia presented an unwarranted risk to the patient.

Clinical features: An obese, 18-yr-old parturient with a fetus in the breech position underwent Cesarean delivery at 35 weeks gestation secondary to evolving preeclampsia. Unfortunately, no neurovascular imaging of this patient’s spine was available. The patient underwent an attempted external cephalic version, a failed obstetric induction, and, ultimately, a Cesarean delivery under general anesthesia. The resulting infant was without any stigmata of KTS. Both mother and infant did well during the course of their hospitalization, and were discharged home without incident.

Conclusions: The posterior cutaneous hemangiomas of KTS may be associated with underlying epidural and subdural vascular malformations. Disruption of these vascular anomalies during regional anesthesia may lead to neuraxial hematoma formation, which may be further compounded by a consumptive coagulopathy observed in some cases of KTS. If neuraxial vascular anomalies cannot be ruled out radiographically, regional anesthesia should be avoided. Additionally, regardless of the anesthetic technique chosen, the coagulation profile of these patients should be verified for signs of coagulopathy.