Double Myelomeningocele Repair by Fetal Surgery with a Single Micro-Hysterotomy
Felipe Chavelas-Ochoa1, Ma de la Luz Bermúdez-Rojas2, Virginia Medina-Jiménez2
1Department of Pediatric Neurosurgery, Women and Children Specialty Hospital "Dr. Felipe Núñez-Lara", Queretaro, Mexico.
Insights
This study shows the first successful intrauterine repair of double open spina bifida (OSB) using a single micro-hysterotomy. This fetal intervention offers potential benefits for selected cases of double OSB, improving neurodevelopmental outcomes.
Area of Science:
- Neuroscience
- Fetal Surgery
- Congenital Anomalies
Background:
- Open spina bifida (OSB) is a common central nervous system anomaly causing significant neurodevelopmental and motor impairments.
- Intrauterine repair of OSB can improve neonatal outcomes in select cases.
- Double myelomeningocele, affecting two spinal segments, presents unique challenges for fetal intervention due to limited evidence.
Observation:
- A case of double myelomeningocele (cervicothoracic and lumbosacral) with Arnold-Chiari malformation type II and ventriculomegaly was treated at 25 weeks gestation.
- The double defects were repaired via a single 2-cm micro-hysterotomy using external versions for sequential exposure and repair.
- Postoperative monitoring revealed no progression of ventriculomegaly or procedure-related complications.
Findings:
- Successful intrauterine open micro-neurosurgical repair of double open spina bifida through a single micro-hysterotomy is demonstrated.
- The patient achieved normal neurodevelopmental outcomes at 20 months, including unaided ambulation and no need for shunting.
Implications:
- This case establishes the feasibility of intrauterine repair for double open spina bifida.
- Selected isolated cases of double myelomeningocele may be candidates for fetal intervention.
- Further prospective studies are warranted to evaluate the benefits of double OSB intrauterine open repair.
Introduction:
Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases.
Case Presentation:
We present a case with both cervicothoracic and lumbosacral myelomeningocele, Arnold-Chiari malformation type II and bilateral ventriculomegaly, that was treated successfully at 25 weeks with open micro-neurosurgery. Double myelomeningocele was successfully treated through a single 2-cm micro-hysterotomy, by performing external versions to sequentially expose and repair both defects. Weekly postoperative follow-up showed no progression of ventriculomegaly or complications attributable to the procedure. Preterm rupture of membranes prompted a conventional cesarean delivery at 32 weeks of gestation. Neurodevelopmental outcome at 20 months was within normal ranges, having achieved ambulation without orthopedic support and with no need for ventriculoperitoneal shunting.
Conclusion:
This report demonstrates for the first time the feasibility of double OSB repair through a single 2-cm micro-hysterotomy, suggesting that selected isolated cases of double myelomeningocele could be candidates for fetal intervention. Further prospective studies should be carried out to assess the potential benefit of double OSB intrauterine open repair.
