Lucie Guilbaud1, Paul Maurice2, Pauline Lallemant3, Timothée De Saint-Denis4, Emeline Maisonneuve2, Ferdinand Dhombres2, Stéphanie Friszer2, Federico Di Rocco5, Catherine Garel6, Marie-Laure Moutard7, Mohamed-Ali Lachtar8, Agnès Rigouzzo9, Véronique Forin3, Michel Zérah4, Jean-Marie Jouannic2. 1. Sorbonne University, AP-HP, Trousseau Hospital, DMU ORIGYNE, Department of Fetal Medicine, 26 Avenue du Dr Arnold Netter, 75012 Paris, France; National Reference Center for Rare Disease: Vertebral and Spinal Cord Anomalies (MAVEM Center), AP-HP, Trousseau Hospital, 26 Avenue du Dr Arnold Netter, 75012 Paris, France. Electronic address: lucie.guilbaud@aphp.fr. 2. Sorbonne University, AP-HP, Trousseau Hospital, DMU ORIGYNE, Department of Fetal Medicine, 26 Avenue du Dr Arnold Netter, 75012 Paris, France; National Reference Center for Rare Disease: Vertebral and Spinal Cord Anomalies (MAVEM Center), AP-HP, Trousseau Hospital, 26 Avenue du Dr Arnold Netter, 75012 Paris, France. 3. National Reference Center for Rare Disease: Vertebral and Spinal Cord Anomalies (MAVEM Center), AP-HP, Trousseau Hospital, 26 Avenue du Dr Arnold Netter, 75012 Paris, France; Sorbonne University, AP-HP, Trousseau Hospital, Department of Physical Medicine and Rehabilitation, 26 Avenue du Dr Arnold Netter, 75012 Paris, France. 4. National Reference Center for Rare Disease: Vertebral and Spinal Cord Anomalies (MAVEM Center), AP-HP, Trousseau Hospital, 26 Avenue du Dr Arnold Netter, 75012 Paris, France; Paris University, AP-HP, Necker Enfants Malades Hospital, Department of Pediatric Neurosurgery, 149 Rue de Sèvres, 75015 Paris, France. 5. Lyon Claude Bernard University, hôpital Femme-Mère-Enfant, Department of Pediatric Neurosurgery, 59 Boulevard Pinel, 69500 Bron, France. 6. National Reference Center for Rare Disease: Vertebral and Spinal Cord Anomalies (MAVEM Center), AP-HP, Trousseau Hospital, 26 Avenue du Dr Arnold Netter, 75012 Paris, France; Sorbonne University, AP-HP, Trousseau Hospital, Department of Pediatric Radiology, 26 Avenue du Dr Arnold Netter, 75012 Paris, France. 7. National Reference Center for Rare Disease: Vertebral and Spinal Cord Anomalies (MAVEM Center), AP-HP, Trousseau Hospital, 26 Avenue du Dr Arnold Netter, 75012 Paris, France; Sorbonne University, AP-HP, Trousseau Hospital, DMU ORIGYNE, Department of Pediatric Neurology, 26 Avenue du Dr Arnold Netter, 75012 Paris, France. 8. Sorbonne University, AP-HP, Trousseau Hospital, DMU ORIGYNE, Neonatal Intensive Care Unit, 26 Avenue du Dr Arnold Netter, 75012 Paris, France. 9. Sorbonne University, AP-HP, Trousseau Hospital, Department of Anesthesiology, 26 Avenue du Dr Arnold Netter, 75012 Paris, France.
Abstract
INTRODUCTION: Open fetal myelomeningocele (MMC) surgery is currently the standard of care option for prenatal MMC repair. We described the population referred to our center and reviewed outcome after open fetal MMC repair. MATERIAL AND METHODS: All patients referred to our center for MMC were reviewed from July 2014 to June 2020. For all the patients who underwent fetal MMC repair, surgical details, maternal characteristics and data from the neonatal to the three-years-old evaluations were collected. RESULTS: Among the 126 patients referred to our center, 49.2% were eligible and 27.4% (n = 17) of them underwent fetal MMC repair. Average gestational age at fetal surgery was 24+6 weeks. There was no case of fetal complication and the only maternal complication was one case of transfusion. We recorded 70% of premature rupture of membranes and 47% of premature labor. Average gestational age at delivery was 34+2 weeks and no patient delivered before 30 weeks. There was no case of uterine scar dehiscence or maternal complication during cesarean section. After birth, 59% of the children had a hindbrain herniation reversal. At 1-year-old, 42% were assigned a functional level of one or more better than expected according to the prenatal anatomic level and 25% required a ventriculoperitoneal shunt. At 3-year-old, all the children attended school and 75% were able to walk with orthotics or independently. CONCLUSION: Open fetal surgery enables anatomical repair of the MMC lesion, a potential benefit on cerebral anomalies and motor function, with a low rate of perinatal and maternal complications.
INTRODUCTION: Open fetal myelomeningocele (MMC) surgery is currently the standard of care option for prenatal MMC repair. We described the population referred to our center and reviewed outcome after open fetal MMC repair. MATERIAL AND METHODS: All patients referred to our center for MMC were reviewed from July 2014 to June 2020. For all the patients who underwent fetal MMC repair, surgical details, maternal characteristics and data from the neonatal to the three-years-old evaluations were collected. RESULTS: Among the 126 patients referred to our center, 49.2% were eligible and 27.4% (n = 17) of them underwent fetal MMC repair. Average gestational age at fetal surgery was 24+6 weeks. There was no case of fetal complication and the only maternal complication was one case of transfusion. We recorded 70% of premature rupture of membranes and 47% of premature labor. Average gestational age at delivery was 34+2 weeks and no patient delivered before 30 weeks. There was no case of uterine scar dehiscence or maternal complication during cesarean section. After birth, 59% of the children had a hindbrain herniation reversal. At 1-year-old, 42% were assigned a functional level of one or more better than expected according to the prenatal anatomic level and 25% required a ventriculoperitoneal shunt. At 3-year-old, all the children attended school and 75% were able to walk with orthotics or independently. CONCLUSION: Open fetal surgery enables anatomical repair of the MMC lesion, a potential benefit on cerebral anomalies and motor function, with a low rate of perinatal and maternal complications.