Double Myelomeningocele Repair by Fetal Surgery with a Single Micro-Hysterotomy.

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.

Management of Late Twin-to-Twin Transfusion Syndrome beyond 26 Weeks: Comparison between Fetoscopic Laser Therapy versus Traditional Management.

INTRODUCTION: A proportion of monochorionic diamniotic (MCDA) twin pregnancies complicated by twin-to-twin transfusion syndrome (TTTS) can present after 26 weeks of gestation. The aim of this study was to compare perinatal outcomes of late TTTS treated by fetoscopic laser coagulation versus traditional management with amniodrainage and/or emergency preterm cesarean delivery (CD). METHODS: Retrospective cohort from January 2012 to January 2023 of consecutive MCDA twin pregnancies complicated by TTTS after 26 weeks and evaluated in our referring centers. We analyzed perinatal outcomes of cases treated with fetoscopic laser surgery at our national referral fetal surgery center in Queretaro, Mexico, and compared them with those managed with traditional management (amniodrainage and/or emergency preterm CD). The primary outcome was survival at discharge and the secondary outcome was gestational age (GA) at birth. RESULTS: Among the study population, 46 TTTS cases were treated by fetoscopy at 27+6 (26+0-31+0) weeks+days and were compared with a group of 39 cases who underwent emergency preterm CD. In comparison to the group who underwent traditional management, the group treated by laser fetoscopy showed a significantly higher GA at birth (32+3 vs. 29+1 weeks+days, p < 0.001), lower frequency of preterm delivery below 37 weeks (91.3% vs. 100%, p = 0.06), 34 weeks (63.0% vs. 100%, p < 0.001), 32 weeks (50% vs. 74.4%, p = 0.02), or 30 weeks (28.3% vs. 53.8%, p = 0.01), and significantly higher perinatal survival (89.1% vs. 71.8%, p < 0.05 of at least one twin; and 65.2% vs. 38.5%, p = 0.01 of both twins, respectively). CONCLUSION: MCDA twins complicated with TTTS can be treated with fetoscopic laser surgery between 26 and 31 weeks of gestation, which is a feasible and safe option, and such cases are associated with a higher GA at birth and better perinatal survival than those managed with amniodrainage and/or emergency preterm CD.

Twin-to-twin transfusion syndrome and coronavirus disease 2019: impact on diagnosis, referral, eligibility for fetoscopic laser therapy, and outcomes.

BACKGROUND: Because of the progressive nature of twin-to-twin transfusion syndrome, difficulties in healthcare access during the COVID-19 pandemic may lead to delayed diagnosis and referral to fetal surgery centers, which may have repercussions on outcomes. OBJECTIVE: This study aimed to assess the clinical impact of the COVID-19 pandemic on pregnancies complicated with twin-to-twin transfusion syndrome. STUDY DESIGN: A retrospective cohort study of consecutive monochorionic diamniotic twin pregnancies complicated with twin-to-twin transfusion syndrome evaluated in our national referral fetal surgery center at Queretaro, Mexico, for possible surgical fetoscopy was conducted. Maternal-fetal characteristics and perinatal outcomes of cases evaluated during the first year of the World Health Organization's COVID-19 pandemic declaration (March 11, 2020 to March 10, 2021) were retrospectively compared with outcomes of cases evaluated during the same period in the previous year (March 11, 2019 to March 10, 2020). RESULTS: Overall, 109 consecutive twin-to-twin transfusion syndrome cases were evaluated during the 2-year study period, 54 during the COVID-19 pandemic and 55 in the previous year. In the former group, a higher proportion of cases with fetal surveillance interval longer than 2 weeks (70.4% vs 47.3%; P=.01); twin-to-twin transfusion syndrome complications precluding laser therapy, such as intrauterine fetal demise, preterm rupture of membranes, or cervical dilatation with prolapsed amniotic membranes (18.5% vs 1.8%; P<.01); advanced twin-to-twin transfusion syndrome (53.7% vs 36.4%; P=.07); preoperative short cervix (25.9% vs 10.9%; P<.05); and lower overall perinatal survival (56.9% vs 80.0% [P=.01; at least 1 twin] and 39.2% vs 56.4% [P=.08; both twins], respectively) were observed. A significantly lower number of cases were selected for fetoscopic laser therapy during the pandemic (75.9% vs 92.7%; P=.01), with similar postoperative outcomes seen in both study periods. CONCLUSION: In pregnancies with twin-to-twin transfusion syndrome, the COVID-19 pandemic has shown an adverse impact involving suboptimal fetal surveillance, advanced stages at diagnosis, poorer survival rates, and higher number of complications that preclude fetoscopic laser therapy.