///2009 Abstract Details
2009 Abstract Details2019-08-03T15:55:31-06:00

Anesthetic Management of a Parturient with Klippel-Trenaunay-Weber Syndrome and Systemic Lupus Erythematosus

Abstract Number: 78
Abstract Type: Case Report/Case Series

Sara C Nelson MD1 ; Isaac A Gooding MD2; Michael L Kent MD3; Catherine M Oberholzer MD4; Karen L Wu MD5

Introduction: A 31-year-old, G3P2 parturient at 36+1 weeks gestation, with Klippel-Trenaunay-Weber syndrome (KTWS) and systemic lupus erythematosus (SLE) (stable on hydroxychloroquine), presented for an emergent cesarean section (CS) due to loss of fluid and onset of active labor. Massive hemorrhage from pelvic and vaginal arteriovenous malformations (AVMs) complicated her previous classic CSs and required embolization and sclerotherapy. This history prompted a multidisciplinary (obstetrics, anesthesia, and radiology) preoperative work-up and a well-communicated plan for elective CS, however, she went into labor six hours before her scheduled CS. Physical examination demonstrated ankle deformities, no significant cutaneous findings, and a favorable airway. MRI revealed a slow flow venous malformation abutting the uterus and extending into the right quadriceps and no epidural vascular malformations. Laboratory values were significant for a hematocrit of 33%, with normal electrolyte, renal, and coagulation indices.

Methods: After placing standard ASA monitors, we proceeded with a rapid sequence induction. A radial arterial line and an internal jugular vein central line were secured. The obstetricians performed a modified classic CS avoiding the venous malformations. A 2.4kg male infant with APGAR scores of eight and nine was delivered. Post-delivery serial arterial blood gasses and blood counts revealed metabolic acidosis and anemia (pH=7.24, BE=-12, lactate=2.2, Hct=23%) prompting resuscitation with 5.5L of crystalloid, 500ml of colloid, and two units packed red blood cells. Despite less-than-expected blood loss (600ml), continued fluid resuscitation, and stable hemodynamics, the patients metabolic acidosis persisted. Subsequent to placing an oral gastric tube, we observed blood emerging from the patients mouth. After 10 minutes of gentle suction, we observed no further bleeding but considered the possibility of a pharyngeal AVM. Intraoperative ENT consultation failed to demonstrate a source of bleeding. Due to the unresolved acidosis and possible pharyngeal bleed, the patient was left intubated and transferred to the ICU. The acidosis normalized rapidly without additional resuscitation and no further pharyngeal bleeding was noted. The patient was extubated by the intensive care service within three hours of ICU admission and discharged home on post-operative day two.

Conclusion: KTWS is a congenital disease characterized by cutaneous hemangiomas, venous varicosities, unilateral limb hypertrophy, and AVMs. The hemorrhagic and thrombotic complications of this disorder are exacerbated by the physiological changes of pregnancy. Despite a complicated medical history, clear communication and thorough multidisciplinary preoperative planning allowed the successful management of this patient through an emergent CS under general anesthesia. We would consider airway imaging as a part of future KTWS patients preoperative plan.

SOAP 2009