Department of Obstetrics and Gynecology, Yeouido St. Mary`s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Department of Obstetrics and Gynecology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
J Child Neurol. 2024 Nov;39(13-14):481-486. doi: 10.1177/08830738241282037. Epub 2024 Oct 3.
The study aimed to investigate the clinical outcomes of fetuses diagnosed with isolated mega cisterna magna in utero. A multicenter retrospective cohort study was conducted across 18 university hospitals from 2010 to 2019. Cases diagnosed with isolated mega cisterna magna, defined as a cisterna magna >10 mm with a normal cerebellar vermis and no cystic dilation of the fourth ventricle, were included. Cases with other central nervous system (CNS) anomalies, extra-central nervous system anomalies, chromosomal abnormalities, or congenital infections were excluded. Maternal demographics, prenatal findings, delivery outcomes, and postnatal outcomes were analyzed. The mean gestational age at initial diagnosis was 28.6 ± 3.9 weeks, and the mean anteroposterior diameter of the cisterna magna was 12.5 ± 3.2 mm. Of the 71 cases initially assessed, 48 (67.6%) showed self-regression in utero. Postnatally, 13 cases (18.3%) had persistent isolated mega cisterna magna, whereas 10 cases (14.1%) were diagnosed with other central nervous system anomalies. Among the persistent isolated mega cisterna magna cases, one child exhibited delayed neurodevelopment. There was a higher incidence of isolated mega cisterna magna in male fetuses, which exhibited a significantly larger mean cisterna magna diameter compared with female fetuses ( = .045). Male fetuses exhibited a higher incidence of isolated mega cisterna magna compared with female fetuses and had larger anteroposterior diameters of the cisterna magna. The study demonstrated favorable neurodevelopmental outcomes associated with isolated mega cisterna magna, with self-regression observed in two-thirds of the cases. However, consecutive prenatal and postnatal evaluations for additional central nervous system or extra-central nervous system malformations need to be performed, considering the differences between prenatal and postnatal diagnoses.
本研究旨在探讨产前诊断孤立性巨大后颅窝池的胎儿的临床结局。这是一项 2010 年至 2019 年在 18 所大学医院进行的多中心回顾性队列研究。纳入病例为孤立性巨大后颅窝池,定义为后颅窝池 >10mm,小脑蚓部正常,第四脑室无囊性扩张。排除伴有其他中枢神经系统(CNS)异常、中枢神经系统外异常、染色体异常或先天性感染的病例。分析了母亲的人口统计学资料、产前发现、分娩结局和产后结局。初始诊断时的平均孕龄为 28.6±3.9 周,后颅窝池的平均前后径为 12.5±3.2mm。在最初评估的 71 例病例中,48 例(67.6%)在宫内出现自发消退。产后,13 例(18.3%)持续存在孤立性巨大后颅窝池,10 例(14.1%)被诊断为其他中枢神经系统异常。在持续存在孤立性巨大后颅窝池的病例中,有 1 例患儿表现出神经发育迟缓。男性胎儿孤立性巨大后颅窝池的发生率较高,其后颅窝池的平均直径明显大于女性胎儿( = .045)。与女性胎儿相比,男性胎儿孤立性巨大后颅窝池的发生率更高,且后颅窝池的前后径也更大。该研究表明孤立性巨大后颅窝池与良好的神经发育结局相关,三分之二的病例出现自发消退。然而,考虑到产前和产后诊断之间的差异,需要对额外的中枢神经系统或中枢神经系统外畸形进行连续的产前和产后评估。