Akay Emrullah, Uzun Gül Alime Dilayda, Türkoğlu Alper
Department of Obstetrics and Gynecology, Basaksehir Cam and Sakura City Hospital, Istanbul, Turkey.
BMC Pregnancy Childbirth. 2025 Apr 8;25(1):407. doi: 10.1186/s12884-025-07540-0.
In this study, a total of 17 patients with Klippel-Trenaunay Syndrome (KTS) and pregnancy were evaluated. The patients were divided into two groups: those with organ involvement (10 patients) and those without organ involvement (7 patients). The clinical findings, complications, and treatment approaches between the two groups were compared, and the effects of KTS on the pregnancy process and potential risks were examined in detail. Significant clinical differences were observed between pregnant women with involvement of abdominal organs such as the liver, spleen, rectum, sigmoid, kidney, bladder, and uterus, as well as central organs like the brain, and those without such involvement. Organ involvement was defined as organ enlargement or venous anomalies detected by techniques such as ultrasound, magnetic resonance imaging (MRI), or computed tomography. Our literature review found that the risk of postpartum hemorrhage (PPH) was significantly higher in the group with organ involvement (p < 0.05). The presence of varicose malformations in organs such as the spleen, liver, and uterus was identified as an important factor increasing the risk of PPH. Therefore, close monitoring of coagulopathic disorders and taking precautions against thromboembolism in pregnant women with KTS is crucial. The case report discusses the complications and treatment processes experienced by a 26-year-old woman diagnosed with KTS and who developed preeclampsia during her two pregnancies. Complications such as preeclampsia and varices were observed in the first pregnancy, and intrauterine growth restriction (IUGR) and preeclampsia in the second pregnancy. Successful outcomes were achieved in both cases with a multidisciplinary approach and appropriate treatment methods. This study provides important information to understand the effects of KTS on pregnancy and the potential complications associated with this rare condition. Future studies will provide more information on the management of preeclampsia and other complications in pregnant women with KTS.
在本研究中,共评估了17例患有克-特综合征(KTS)且怀孕的患者。患者被分为两组:有器官受累的患者(10例)和无器官受累的患者(7例)。比较了两组之间的临床发现、并发症及治疗方法,并详细研究了KTS对妊娠过程的影响及潜在风险。观察到腹部器官如肝脏、脾脏、直肠、乙状结肠、肾脏、膀胱和子宫以及中枢器官如大脑受累的孕妇与未受累孕妇之间存在显著的临床差异。器官受累定义为通过超声、磁共振成像(MRI)或计算机断层扫描等技术检测到的器官肿大或静脉异常。我们的文献综述发现,器官受累组产后出血(PPH)的风险显著更高(p < 0.05)。脾脏、肝脏和子宫等器官中静脉曲张畸形的存在被确定为增加PPH风险的重要因素。因此,密切监测患有KTS的孕妇的凝血障碍并预防血栓栓塞至关重要。该病例报告讨论了一名26岁被诊断为KTS且在两次怀孕期间发生先兆子痫的女性所经历的并发症及治疗过程。在第一次妊娠中观察到先兆子痫和静脉曲张等并发症,在第二次妊娠中观察到宫内生长受限(IUGR)和先兆子痫。通过多学科方法和适当的治疗方法,两例均取得了成功的结果。本研究为了解KTS对妊娠的影响以及与这种罕见疾病相关的潜在并发症提供了重要信息。未来的研究将提供更多关于患有KTS的孕妇先兆子痫及其他并发症管理的信息。