Kordjalik Paulina, Szmyd Bartosz, Karuga Filip Franciszek, Daszkiewicz Gabriela, Strzelecka Iwona, Respondek-Liberska Maria
Department of Prenatal Cardiology, Polish Mother's Memorial Hospital, 93-338 Łódź, Poland.
Department of Pediatrics, Oncology, and Hematology, Medical University of Lodz, 91-738 Łódź, Poland.
J Clin Med. 2022 Feb 16;11(4):1035. doi: 10.3390/jcm11041035.
Lymphangiomas are uncommon, benign (from a histopathology viewpoint) malformations of the lymphatic system with thin-walled vessels; however, these tumors may be dangerous for fetal or neonatal life. They are observed in 1:6000 newborns at birth and in 1:750 spontaneous abortions. We aimed to investigate the role of fetal echocardiography in the prognosis of lymphangioma. Selected data of 19,836 pregnant women studied between 1999 and 2020 were retrospectively analyzed. In total, 32 cases of lymphangioma meeting the following criteria were further analyzed: (1) ultrasound availability from the 1st trimester of pregnancy and (2) nuchal translucency ≤ 2.5 mm. Echocardiographic findings, karyotype, size, and location of the possible lesion were juxtaposed with the clinical follow-up. The statistical analysis was performed using Statistica 13.1 software (StatSoft, Tulsa, OK, USA). Lymphangioma in the analyzed material coexisted with abnormalities in fetal echo in 78% ( = 25) of cases, especially: heart defect in 50% ( = 16) and with normal heart structure with functional changes in 28% ( = 9). Karyotype was available in 50% of the analyzed cases ( = 16). Normal cytogenetic results were observed in 62.5% ( = 10) of cases. In the remaining cases, the following were observed: Turner Syndrome: 25% ( = 4) and Down Syndrome 12.5% ( = 2). The rate of alive newborns was significantly higher among fetuses with isolated lymphangioma in comparison to those with lymphangioma associated with abnormal ECHO examination: 38.46% ( = 5) vs. 15.38% ( = 2; = 0.037). Abnormal ECHO exam was a poor prognostic sign for fetuses with lymphangioma; therefore, we think it is important to refer these cases for detailed echocardiography in tertiary centers. Moreover, it should be highlighted that in all lymphangioma cases there was an indication to perform the karyotype assessment, as there was a high risk of aneuploidy.
淋巴管瘤是一种罕见的、（从组织病理学角度来看）良性的淋巴管系统畸形，其血管壁薄；然而，这些肿瘤对胎儿或新生儿的生命可能是危险的。出生时在6000名新生儿中可观察到1例，在1:750的自然流产中可观察到1例。我们旨在研究胎儿超声心动图在淋巴管瘤预后中的作用。回顾性分析了1999年至2020年间研究的19836名孕妇的选定数据。总共对32例符合以下标准的淋巴管瘤病例进行了进一步分析：（1）妊娠早期可进行超声检查，（2）颈部透明带≤2.5毫米。将超声心动图检查结果、核型、可能病变的大小和位置与临床随访情况进行了对比。使用Statistica 13.1软件（美国俄克拉何马州塔尔萨市的StatSoft公司）进行统计分析。在所分析的材料中，78%（n = 25）的病例中淋巴管瘤与胎儿超声异常并存，尤其是：50%（n = 16）有心脏缺陷，28%（n = 9）心脏结构正常但有功能改变。50%（n = 16）的分析病例可获得核型结果。62.5%（n = 10）的病例观察到细胞遗传学结果正常。在其余病例中，观察到以下情况：特纳综合征：25%（n = 4），唐氏综合征12.5%（n = 2）。与伴有异常超声心动图检查的淋巴管瘤胎儿相比，孤立性淋巴管瘤胎儿的活产新生儿率显著更高：38.46%（n = 5）对15.38%（n = 2；p = 0.037）。异常超声心动图检查对患有淋巴管瘤的胎儿是一个不良预后指标；因此，我们认为将这些病例转诊至三级中心进行详细的超声心动图检查很重要。此外，应该强调的是，在所有淋巴管瘤病例中都有必要进行核型评估，因为存在非整倍体的高风险。
