Open intrauterine repair of spina bifida aperta: Historical aspects, current availability, and clinical outcomes from the Latin American Spina Bifida Consortium

Abstract

Objective: To determine the historical aspects, current availability, and clinical outcomes of open intrauterine repair of spina bifida aperta (IRSBA) in Spanishspeaking Latin American countries. Methods: Cases were collected from centers with at least 2 years of experience and a minimum of 10 open IRSBA interventions by December 2020. Clinical variables were compared to the results of the Management of Myelomeningocele Study (MOMS) trial.Results: Clinical experience with 314 cases from seven centers was reviewed. Most cases (n = 189, 60.2%) were performed between 24 and 25.9 weeks' gestation.Delivery at less than 30 weeks' gestation occurred in 36 cases (11.5%) and the overall perinatal mortality rate was 5.4% (17 of 314). The rate of maternal complications was low, including the need for blood transfusion (n = 3, 0.9%) and dehiscence or a thin uterine scar (n = 4, 1.3%). No cases of maternal death were recorded. Fifteen neonates required additional surgical repair of the spinal defect (4.8%) and 63 of 167 infants (37.7%) required a cerebrospinal fluid diversion procedure. Only two of the seven centers reported preliminary experience with fetoscopic IRSBA.Conclusions: Clinical experience and outcomes were within the expected results reported by the MOMS trial. There is still very limited experience with fetoscopic IRSBA in this part of the world. Key pointsWhat is already known about this topic? � Intrauterine repair of spina bifida aperta (IRSBA) is a well-established option for the management of second-trimester fetuses affected by this neural tube defect.� Aside from Brazil, there is little information available on outcomes from fetal surgery centers in Latin America.Collaborators are listed in the Appendix section of the manuscript.

Impact on postoperative, neonatal and 2‐year neurodevelopmental outcomes of UA‐AREDF during and after fetal spina bifida repair

“…About 1 in 3000 pregnancies is affected by open fetal spina bifida (OSB), which is the most common neural tube defect 1,2 . Nowadays, intrauterine repair of OSB is a valid therapeutic option in selected cases [3][4][5][6] . It has been shown to reduce the need for ventriculoperitoneal shunt placement and to improve long-term neurological function 3,5 .…”

Outcomes of late open fetal surgery for intrauterine spina bifida repair after 26 weeks. Should we extend the Management of Myelomeningocele Study time window?

“…1 A decade after its publication, this intervention is being offered in a growing number of countries throughout the world and the advantages of prenatal spina bifida repair have been validated in several experienced centers. [2][3][4][5][6][7][8][9] The rationale for fetal surgery of open fetal dysraphism is based on the "two-hit" hypothesis, which states that in addition to the primary neurulation defect during embryogenesis, a significant portion of the postnatal functional handicap seen in patients with open spina bifida (OSB) is attributable to further accumulative damage to the neural cord due to mechanical and chemical injury, especially during the third trimester, leading to motor deterioration. [10][11][12][13] The effect of covering the defect to stop tissue destruction and cerebrospinal fluid leakage was demonstrated initially in several animal models, 14,15 leading to the first human cases in the late 90s.…”

“…The Management of Myelomeningocele Study (MOMS) trial established both the benefits and risks of fetal surgery for open spinal dysraphism 1 . A decade after its publication, this intervention is being offered in a growing number of countries throughout the world and the advantages of prenatal spina bifida repair have been validated in several experienced centers 2‐9 …”