Abstract：
Objective. 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.Methods. 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.Results. 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.Conclusions. 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.
摘要：
Objective.描述在妊娠晚期诊断为大宫颈肿块的病例，并在产后立即发展为Kasabach-Merritt现象（KMP），以及文献综述。方法。通过Medline/Pubmed进行病例报告和文献检索的描述，从开始到2022年12月进行了与KMP产前和产后诊断有关的文章。结果。一名36岁的经产妇女在妊娠40周时因宫缩入院，在原本平静的怀孕中。入院超声显示颈部后侧存在14x15厘米的大量肿块，高度血管化，没有血液动力学失衡的迹象.出生后,血液检查显示存在严重的贫血和血小板减少症，需要多次输血，等离子体,血小板和凝血因子。由于先天性血管瘤和血小板减少症的关联，因此诊断为KMP。在尝试保守治疗后，需要手术切除以停止血液学级联反应,症状消退.对文献的回顾确定了14篇文章，其中包括9例产前可疑的KMP和6例在产后立即诊断且没有胎儿积水的迹象。不良围产期结局，在产后死亡/终止妊娠方面，在产前可疑组中有67％的病例（6/9），在产后诊断为KMP的病例中观察到33％的病例。83％的围产期不良结局病例存在胎儿积水。Conclusions.Kasabach-Merrit综合征是一种罕见的疾病,当它在子宫内或出生后即刻发展时，它可能具有危险的进化，具有约50％的围产期死亡率的风险。即使胎儿没有贫血或心力衰竭的迹象，在产后立即发展它的风险很高，应该向夫妇提及。
