Fetal open spina bifida: a natural history of disease progression in utero

Joseph R Biggio1, Katharine D Wenstrom, John Owen

  • 1University of Alabama at Birmingham, Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine and Reproductive Genetics, Birmingham, Alabama 35249-7333, USA. jbiggio@uab.edu

Prenatal Diagnosis
|April 6, 2004
PubMed

Insights

Most fetuses with open spina bifida develop ventriculomegaly by 21 weeks gestation. Congenital talipes is less common and often develops after 20 weeks, making it difficult to predict early in pregnancy.

Area of Science:

  • Prenatal Development
  • Fetal Medicine
  • Pediatric Surgery

Background:

  • Open spina bifida is a complex congenital condition affecting fetal development.
  • Ventriculomegaly and talipes are common co-occurring anomalies in fetuses with open spina bifida.
  • Understanding the natural history of these anomalies is crucial for prenatal counseling and management.

Purpose of the Study:

  • To investigate the prenatal development and natural history of ventriculomegaly and talipes in fetuses diagnosed with open spina bifida.
  • To identify the timing of onset and progression of these anomalies during gestation.
  • To assess the predictive value of early-midtrimester sonography for these conditions.

Main Methods:

  • Retrospective evaluation of fetuses with isolated open spina bifida managed between 1996 and 2000.
  • Review of serial ultrasonographic images and reports from diagnosis to delivery.
  • Assessment of lesion level and type, ventriculomegaly (atrial width >= 10 mm), and lower extremity appearance.

Main Results:

  • Of 53 pregnancies, 20 were terminated. In 33 ongoing gestations, 55% had early-onset ventriculomegaly (diagnosed by 22 weeks), 33% developed late-onset ventriculomegaly (by 29 weeks), and 12% had normal ventricular size.
  • Late-onset ventriculomegaly resulted in significantly smaller ventricular size at birth compared to early-onset.
  • Only 6% had talipes at initial sonogram; 18% developed talipes later (by 30 weeks).

Conclusions:

  • The majority of fetuses with open spina bifida develop ventriculomegaly, often by 21 weeks' gestation.
  • Late-onset ventriculomegaly is associated with less severe ventricular dilation at delivery.
  • Congenital talipes is less frequent and typically develops after 20 weeks, limiting early sonographic prediction.
Abstract