The Intrauterine Treatment of Open Spinal Dysraphism

Abstract

Background: Open spinal dysraphism is a congenital malformation that causes major morbidity. Its consequences include sensory and motor impairment as well as bladder- and bowel dysfunction. It is often also associated with prenatal ventriculomegaly, which, in turn, necessitates postnatal treatment with a ventriculoperitoneal shunt in approximately 80% of cases. Prenatal therapy with coverage of neural tube defect can reduce the shunt rate and preserve motor function. In this review, we describe the different surgical procedures and their outcomes.

Methods: This review is based on publications that were retrieved by a selective literature search in the MEDLINE, Web of Science, EMBASE, Scopus, and Cochrane databases, employing pertinent keywords. Studies of all types (except case reports) that were published in English or German in the period 2010-2024 were included.

Results: The randomized, controlled MOMS trial showed that intrauterine surgery for defect closure resulted in less progressive neural tissue damage than postnatal surgery and reduced the need for shunting by approximately half (40% vs. 82%). Since the publication of these results, various prenatal surgical procedures have been established, including hysterotomy-assisted, percutaneous fetoscopic, and laparotomy-assisted fetoscopic closure. The individual surgical methods yield comparable results in terms of motor function and shunt rate. A problem with these procedures is that they increase the likelihood of preterm birth, to an extent that varies from one type of procedure to another.

Conclusion: Prenatal surgery improves motor function and reduces the shunt rate but long-term outcomes beyond adolescence are still lacking. Transparent and interdisciplinary counseling is essential in prenatal communication to inform parents not only about the potential benefits of this treatment, but also about its limitations and risks.

Figure 1

Hysterotomy-assisted repair Uterus with hysterotomy (*) and retaining sutures placed; during surgery, the amniotic fluid is replaced via the inserted tube. The photo shows the fetal back after completed hysterotomy-assisted repair of the fetal myelomeningocele (arrow). By courtesy of Prof. Ueli Moehrlen, University Children‘s Hospital Zurich, Switzerland

Figure 2

Percutaneous fetoscopic repair Maternal abdominal wall with trocars inserted (arrow) By courtesy of Prof. Cleisson F.A. Peralta, The Heart Hospital, Sao Paulo, Brazil

Figure 3

Laparotomy-assisted fetoscopic repair (hybrid method). Externalized uterus after placement of 3 ports; the fetus is manually maintained in the correct position through trans-uterine manipulation. Images from the Spina bifida Center at the University Hospital Giessen and Marburg (UKGM)

Figure 4

Schematic representation of three different surgical methods for prenatal treatment of fetal open spinal dysraphism, from top to bottom: Hysterotomy-assisted repair involves lower abdominal transverse laparotomy with subsequent hysterotomy and multi-layer repair of the fetal back, similar to the postnatal surgical repair procedure. Laparotomy-assisted fetoscopic repair (hybrid method) combines lower abdominal transverse laparotomy with subsequent externalization of the uterus. Prior to placement of the fetoscopic ports, the amnion is fixed with sutures; the defect repair is performed in three layers (Durapatch, muscle, skin). Percutaneous fetoscopic repair is performed without laparotomy. The repair of the defect is performed using a single-layer patch for coverage or by multi-layer repair, depending on surgical preference. created with Biorender