Evidence map›Paper›PMID 41419823›Full record

ArticleBMC pregnancy and childbirth2025

An unusual case of Kippel-Trenaunay syndrome complicated with intrahepatic cholestasis of pregnancy and disseminated intravascular coagulation.

Juan Ye, Kun Huang

Abstract readCase Reports
In one paragraph

Article in BMC pregnancy and childbirth, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

2 authors.

Juan YeDepartment of Pediatrics, The First Affiliated Hospital of Zhejiang University School of Medicine, 1367 Wenyi West Road, Yuhang District, Zhejiang, 311121, HangZhou, China.
Kun HuangDepartment of Pediatrics, The First Affiliated Hospital of Zhejiang University School of Medicine, 1367 Wenyi West Road, Yuhang District, Zhejiang, 311121, HangZhou, China. KunHuang@zju.edu.cn.ORCID http://orcid.org/0000-0003-1641-1472

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundKlippel-Trenaunay syndrome (KTS) is a rare congenital vascular disorder characterized by capillary malformation, varicose veins, and limb hypertrophy. Women with KTS face increased risks of thromboembolism and hemorrhage during pregnancy, delivery, and the postpartum period. Intrahepatic cholestasis of pregnancy (ICP), the most common pregnancy-related liver disorder, is associated with adverse perinatal outcomes. However, coexistence of KTS and ICP has not been previously reported. CASE PRESENTATION: We describe the case of a 29-year-old pregnant woman with known KTS who developed ICP in the third trimester. She was managed with ursodeoxycholic acid and prophylactic anticoagulation. Despite therapy, her serum bile acids remained elevated, and fetal monitoring revealed decelerations, prompting cesarean delivery at 35 weeks of gestation. The newborn was preterm but otherwise healthy. Postpartum, the patient experienced progressive coagulopathy with markedly elevated D-dimer levels, superficial vein thrombosis, and clinical signs of disseminated intravascular coagulation (DIC). With adjustment of anticoagulation and administration of fresh frozen plasma and fibrinogen, her coagulation profile gradually normalized. She was discharged on rivaroxaban and remained stable during follow-up.

conclusionThis case highlights the complexity of managing pregnancy in women with KTS complicated by ICP. The coexistence of two high-risk conditions poses challenges in balancing thrombosis and bleeding risks, as well as safeguarding fetal outcomes. Multidisciplinary care, close monitoring, and individualized anticoagulation strategies are essential to optimize maternal and neonatal prognosis.

Indexed as

Cholestasis, IntrahepaticDisseminated Intravascular CoagulationKlippel-Trenaunay-Weber SyndromePregnancy ComplicationsAdultCesarean SectionFemaleHumansInfant, NewbornPregnancyPregnancy Trimester, ThirdUrsodeoxycholic AcidUrsodeoxycholic AcidDisseminated intravascular coagulationIntrahepatic cholestasis of pregnancyKippel-Trenaunay Syndrome(KTS)

Identifiers

PMID41419823
PMCPMC12836794

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