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Fetal surgery for myelomeningocele: patient selection, perioperative management and outcomes
Enrico Danzer1, N Scott Adzick
1Center for Fetal Diagnosis and Treatment, The Children’s Hospital of Philadelphia and The University of Pennsylvania School of Medicine, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104-4388, USA.
Abstract:
Myelomeningocele (MMC), one of the most common congenital malformations, can result in severe lifelong disabilities,including paraplegia, hydrocephalus, Chiari II malformation(CM-II), incontinence, sexual dysfunction, skeletal deformations and mental impairment. MMC was the first nonlethal anomaly to be treated by fetal surgery. Experimental and clinical evidence suggests that the primary cause of the neurologic deficit associated with MMC is not simply incomplete neurulation but rather chronic mechanical injury and amniotic fluid-induced chemical trauma that progressively damages the exposed neural tissue during gestation. Case series and a prospective, randomized study show that fetal surgery for MMC before 26 weeks' gestation may preserve neurologic function, reverse the hindbrain herniation of the CM-II and obviate the need for postnatal placement of a ventriculoperitoneal shunt. However, these studies also demonstrate that fetal surgery is associated with significant maternal and fetal risks. Consequently, further research is warranted to further expand our understanding of the pathophysiology of MMC, to evaluate the long-term impact of in utero intervention and to refine the timing and technique of fetal MMC surgery.
Insights
Fetal surgery for myelomeningocele (MMC) before 26 weeks may preserve neurological function and reverse hindbrain herniation. However, this intervention carries significant maternal and fetal risks, necessitating further research.
Area of Science:
- Neuroscience
- Developmental Biology
- Surgical Innovation
Background:
- Myelomeningocele (MMC) is a common congenital malformation causing severe lifelong disabilities.
- Neurologic deficits in MMC are attributed to chronic mechanical and chemical trauma to exposed neural tissue.
- Fetal surgery for MMC represents a novel therapeutic approach to mitigate these effects.
Purpose of the Study:
- To evaluate the efficacy and safety of fetal surgery for myelomeningocele.
- To investigate the impact of in utero intervention on neurologic function and associated malformations.
- To refine the understanding of MMC pathophysiology and surgical techniques.
Main Methods:
- Review of experimental and clinical evidence, including case series and a prospective, randomized study.
- Analysis of fetal surgery outcomes performed before 26 weeks' gestation.
- Assessment of maternal and fetal risks associated with prenatal MMC repair.
Main Results:
- Fetal surgery may preserve neurologic function and reverse Chiari II malformation (CM-II) hindbrain herniation.
- Prenatal intervention can obviate the need for postnatal ventriculoperitoneal shunts.
- Significant maternal and fetal risks are associated with fetal MMC surgery.
Conclusions:
- Fetal surgery for MMC shows promise in improving outcomes but carries substantial risks.
- Further research is crucial to understand MMC pathophysiology and optimize prenatal surgical strategies.
- Long-term evaluation of in utero intervention and refinement of surgical techniques are warranted.
