目的: 探讨产前超声对胎儿Dandy-walker综合征的诊断价值及相关信息分析。方法: 选取内蒙古科技大学包头医学院第一附属医院2017年8月-2021年8月产前超声诊断的27例Dandy-walker综合征病例,针对超声图像、超声复查及随访结果进行分析及文献回顾。结果: 27例Dandy-walker综合征病例,其中Dandy-walker畸形8例、Dandy-walker变异型7例、单纯性小脑延髓池增宽12例。13例病例合并多发畸形,最常见为脑积水,另外还涉及骨骼肌肉系统、颜面部、心血管系统及泌尿系统及其他。伴发的阳性超声软指标有单脐动脉、侧脑室轻度增宽、“韦尔加”腔存在、肾盂轻度分离;其中2例Dandy-walker变异型为非整倍体(21、18-三体各1例);多数Dandy-walker畸形及变异型病例确诊后孕妇选择终止妊娠,单纯小脑延髓池增宽胎儿预后较好。结论: 产前超声诊断胎儿Dandy-walker综合征具有重要的临床应用价值,Dandy-walker畸形与变异型胎儿伴发畸形具有多样性及多重性,且易与多倍体相关,单纯小脑延髓池增宽胎儿预后乐观。
Objective: To study the diagnostic and prognostic value of prenatal ultrasound inspection on fetal Dandy-Walker syndrome. Methods:Ultrasound images, ultrasonic reexamination results and follow-up results of 27 fetuses who were diagnosed with Dandy-walker syndrome in our hospital from august 2017 to august 2021 were collected and analyzed. Results: Twenty seven fetuses with Dandy-Walker syndrome were selected, including 8 cases of Dandy-Walker malformation, 7 cases of Dandy-walker variant, 12 cases of simple dilatation of the posterior fossa cistern.Thirteen cases in the 27 fetus were detected with malformation such as hydrocephalus, malformations of cleft lip and palate, cardiovascular abnormalities, skeletal musculature system and urinary tract were also found. Combined ultrasonographic soft makers such as enlarged lateral ventricles (diameter between 10mm and 15mm), slight renal pelvis dilation, single umbilical artery and cavum vergae were found. Two cases of Dandy -Walker variants were aneuploidy. Most pregnant women choose to terminate pregnancy after being diagnosed with Dandy-Walker syndrome combining deformity or variant. Fetuses with simple dilatation of the posterior fossa cistern had good prognosis. Conclusion: Prenatal ultrasound inspection on fetal Dandy-Walker syndrome is of great clinical value. Dandy-Walker malformation and variant fetal with malformation is of diversity and multiplicity and closely related with multiploid. Fetuses with simple dilatation of the posterior fossa cistern usually have good prognosis.
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