University Department of Obstetrics & Gynaecology, San Salvatore Hospital, L'Aquila, Italy.
Department of Life, Health and Environmental Sciences, University of L'Aquila, L'Aquila, Italy.
J Matern Fetal Neonatal Med. 2023 Dec;36(2):2274803. doi: 10.1080/14767058.2023.2274803. Epub 2023 Oct 31.
To describe the case of a large cervical mass diagnosed in the late third trimester with development of Kasabach-Merritt phenomenon (KMP) in the immediate postnatal period, along with a literature review.. Description of case-report and literature search through Medline/Pubmed, performed from inception to December 2022 for articles relating to the pre and postnatal diagnosis of KMP. A 36-year-old multiparous woman was admitted to hospital for contractions at 40 weeks of gestation, in an otherwise uneventful pregnancy. Admission's ultrasound showed the presence of a voluminous mass of 14x15 cm of the posterior side of the neck, highly vascularized, and no signs of hemodynamic imbalance. Postnatally, blood tests showed the presence of severe anemia and thrombocytopenia requiring several transfusions of blood, plasma, platelets and clotting factors. Due to the association of congenital hemangioma and thrombocytopenia a diagnosis of KMP was made. After attempts of conservative treatment, surgical removal was needed to stop the hematological cascade with regression of symptoms. The review of the literature identified 14 articles including 9 cases of prenatally suspected KMP and 6 diagnosed in the immediate postnatal period and without signs of fetal hydrops. Adverse perinatal outcome, in terms of postnatal death/termination of pregnancy, was observed in 67% of cases (6/9) in the prenatally suspected group and 33% of cases in those with a postnatal diagnosis of KMP. Fetal hydrops was present in 83% of cases with adverse perinatal outcome. The Kasabach-Merrit syndrome is a rare condition, which can have a dangerous evolution when it develops in utero or in the immediate postnatal period carrying a risk of perinatal mortality of approximately 50%. Even if the fetus shows no signs of anemia or heart failure, the risk of developing it in the immediate postnatal period is high and should be mentioned to the couple.
描述了一例在妊娠晚期第三 trimester 诊断出的巨大颈部肿块病例，在产后即刻出现了 Kasabach-Merritt 现象（KMP），并进行了文献回顾。通过 Medline/Pubmed 进行病例报告和文献检索，检索时间从开始至 2022 年 12 月，检索内容与 KMP 的产前和产后诊断相关。一位 36 岁的多产妇因妊娠 40 周时出现宫缩而入院，在此之前妊娠过程无特殊。入院时的超声检查显示，颈部后侧有一个 14x15cm 的巨大肿块，高度血管化，没有血流动力学失衡的迹象。产后，血液检查显示严重贫血和血小板减少，需要多次输血、血浆、血小板和凝血因子。由于先天性血管瘤和血小板减少症的存在，诊断为 KMP。在尝试保守治疗后，需要进行手术切除以阻止血液学级联反应，使症状消退。文献回顾共确定了 14 篇文章，包括 9 例产前疑似 KMP 和 6 例产后即刻诊断且无胎儿水肿的病例。在产前疑似组中，67%（6/9）的病例发生不良围产期结局（产后死亡/终止妊娠），而在产后即刻诊断组中，该比例为 33%。有 83%的不良围产期结局病例存在胎儿水肿。Kasabach-Merrit 综合征是一种罕见的疾病，当它在宫内或产后即刻发展时可能会有危险的演变，导致围产儿死亡率约为 50%。即使胎儿没有贫血或心力衰竭的迹象，在产后即刻发展的风险也很高，应该告知夫妇。
