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The Intrauterine Treatment of Open Spinal Dysraphism
Corinna Keil1, Benjamin Sass, Maximilian Schulze
1Department of Obstetrics and Gynecology, University Hospital Giessen and Marburg, Campus Marburg, Marburg, Germany; Department of Neurosurgery, University Hospital of Giessen and Marburg, Campus Marburg, Marburg, Germany; Department of Neuroradiology, University Hospital Giessen and Marburg, Campus Marburg, Marburg, Germany; Center for Prenatal Medicine and Fetal Therapy, University Hospital Giessen and Marburg, Campus Giessen, Giessen, Germany.
Insights
Prenatal surgery for open spinal dysraphism improves motor function and reduces the need for ventriculoperitoneal shunts. However, long-term outcomes are unknown, and risks like preterm birth exist.
Area of Science:
- Neuroscience
- Developmental Biology
- Surgical Innovation
Background:
- Open spinal dysraphism is a congenital malformation leading to significant morbidity, including motor and sensory deficits and bladder/bowel dysfunction.
- Prenatal ventriculomegaly is common, often requiring postnatal ventriculoperitoneal shunting in up to 80% of cases.
- Prenatal intervention for neural tube defects can potentially mitigate shunt rates and preserve motor function.
Purpose of the Study:
- To review and describe various prenatal surgical procedures for open spinal dysraphism.
- To evaluate the outcomes associated with these different surgical techniques.
Main Methods:
- A selective literature search was conducted across major databases (MEDLINE, Web of Science, EMBASE, Scopus, Cochrane).
- Included studies were published in English or German between 2010 and 2024, excluding case reports.
- Pertinent keywords were used to retrieve relevant publications.
Main Results:
- The MOMS trial demonstrated intrauterine surgery reduced neural tissue damage and shunt rates (40% vs. 82% postnatally).
- Established prenatal procedures include hysterotomy-assisted, percutaneous fetoscopic, and laparotomy-assisted fetoscopic closure, yielding comparable motor function and shunt rates.
- A significant drawback is the increased likelihood of preterm birth, varying by procedure type.
Conclusions:
- Prenatal surgery offers benefits for motor function and shunt reduction in open spinal dysraphism.
- Long-term outcomes beyond adolescence require further investigation.
- Comprehensive, interdisciplinary counseling is crucial to inform parents about the benefits, risks, and limitations of prenatal treatment.
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.
