Late puerperal hemorrhage of a patient with Klippel-Trenaunay syndrome: A case report

Abstract

Introduction: The Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder. The obstetric course of women with KTS varies. Complications include bleeding, disseminated intravascular coagulation (DIC), thromboembolic events, etc. PATIENT CONCERNS:: Here, we report a case of late puerperal hemorrhage of a Chinese puerpera with KTS. The repeating severe hemorrhage, the DIC, and the Kasabach-Merrit syndrome made the treatment more difficult.

Diagnosis: KTS is a mixed malformation with a vascular component that is characterized by abnormal development of veins, capillaries, and lymphatics. Our patient was first diagnosed with KTS at the last trimester of pregnancy.

Interventions: Massive infusion of blood products, two laparotomies, as well as bilateral internal iliac artery embolization was carried out.

Outcomes: Although the patient survived from the life-threatening hemorrhage, she lost her uterus forever.

Conclusion: An interdisciplinary cooperation of obstetrician, anesthesiologist, vascular surgeon, and intensive care physician is highly recommended. Prophylactic anticoagulation is generally advised in the gestational and postpartum period.

Figure 1

(A–C) Multiple subcutaneous hemangiomas, varicosities and port-wine capillaries distributed over the left buttock and the whole left lower extremity. (D) The left labia majora contained a large complex of varicosities.