Braithwaite J M, Economides D L
University Department of Obstetrics and Gynaecology, Royal Free Hospital, London, U.K.
Prenat Diagn. 1995 Dec;15(12):1168-70. doi: 10.1002/pd.1970151215.
Meckel-Gruber syndrome is a rare autosomal recessive disorder often resulting in neonatal death within a few hours of life. The condition is usually diagnosed ultrasonographically in the second trimester and earlier diagnosis has been mainly confined to high recurrence risk pregnancies. We describe the earliest non-invasive diagnosis of this condition at 12+2 weeks' gestation in a patient with no previous history using transabdominal ultrasound. Pregnancy termination was declined. Subsequent development of anhydramnios in the mid-trimester resulted in poor fetal visualization. We propose that first-trimester diagnosis of this condition is not only possible but preferable, as normal liquor volume facilitates visualization of fetal anatomy.
梅克尔-格鲁伯综合征是一种罕见的常染色体隐性疾病,常导致新生儿在出生后数小时内死亡。这种疾病通常在孕中期通过超声检查诊断,而早期诊断主要局限于高复发风险的妊娠。我们描述了一名无既往病史的患者在妊娠12+2周时通过经腹超声对该病进行的最早非侵入性诊断。患者拒绝终止妊娠。妊娠中期羊水过少的后续发展导致胎儿可视化不佳。我们认为,对这种疾病进行孕早期诊断不仅是可能的,而且是更可取的,因为正常羊水量有助于胎儿解剖结构的可视化。