Open AccessLate puerperal hemorrhage of a patient with Klippel–Trenaunay syndrome
Author(s) -
Jian Zhang,
Kana Wang,
Jie Mei
Publication year - 2019
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000018378
Subject(s) - medicine , obstetrics and gynaecology , coagulation disorder , disseminated intravascular coagulation , embolization , klippel trenaunay weber syndrome , surgery , pregnancy , obstetrics , coagulation , genetics , biology , muscle hypertrophy
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.