Denise A L Pedreira1, Nelci Zanon2, Koshiro Nishikuni3, Renato A Moreira de Sá4, Gregório L Acacio5, Ramen H Chmait6, Eftichia V Kontopoulos7, Rubén A Quintero7. 1. Fetal Therapy Program, Albert Einstein Hospital, São Paulo, Brazil; Department of Obstetrics, Instituto de Assistência ao Servido Público do Estado de São Paulo, São Paulo, Brazil; A collaboration between the Brazilian Fetal Network. Electronic address: denise.pedreira@gmail.com. 2. Department of Neurosurgery, Federal University of São Paulo, São Paulo, Brazil. 3. Department of Neurosurgery, Instituto de Assistência ao Servido Público do Estado de São Paulo, São Paulo, Brazil. 4. Department of Obstetrics, University Federal Fluminense, Niterói, Brazil; A collaboration between the Brazilian Fetal Network. 5. Department of Obstetrics, University of Taubaté, Taubaté, Brazil; A collaboration between the Brazilian Fetal Network. 6. Department of Obstetrics and Gynecology, Keck School of Medicine, University of Southern California, Los Angeles, CA; USFetus Consortium. 7. Division of Maternal-Fetal Medicine and Fetal Therapy, Jackson Fetal Therapy Institute, Miami, FL; USFetus Consortium.
Abstract
BACKGROUND: A recent randomized clinical trial named Management of Myelomeningocele Study (MOMS trial) showed that prenatal correction of open spina bifida (OSB) via open fetal surgery was associated with improved infant neurological outcomes relative to postnatal repair, but at the expense of increased maternal morbidity. OBJECTIVE: We sought to report the final results of our phase I trial (Cirurgia Endoscópica para Correção Antenatal da Meningomielocele [CECAM]) on the feasibility, safety, potential benefits, and side effects of the fetoscopic treatment of OSB using our unique surgical technique. STUDY DESIGN: Ten consecutive pregnancies with lumbosacral OSB were enrolled in the study. Surgeries were performed percutaneously under general anesthesia with 3 ports and partial carbon dioxide insufflation. After appropriate surgical positioning of the fetus, the neuroplacode was released with scissors and the skin was undermined to place a biocellulose patch over the lesion. The skin was closed over the patch using a single running stitch. Preoperative, postoperative, and postnatal magnetic resonance imaging were performed to assess hindbrain herniation. Neurodevelopmental evaluation was performed before discharge and at 3, 6, and 12 months. All cases were delivered by cesarean delivery, at which time the uterus was assessed for evidence of thinning or dehiscence. RESULTS: The median gestational age at the time of surgery was 27 weeks (range 25-28 weeks). Endoscopic repair was completed in 8 of 10 fetuses. Two cases were unsuccessful due to loss of uterine access. The mean gestational age at birth was 32.4 weeks with a mean latency of 5.6 weeks between surgery and delivery (range 2-8 weeks). There was 1 fetal and 1 neonatal demise, and 1 unsuccessful case underwent postnatal repair. Of the 7 infants available for analysis, complete reversal of hindbrain herniation occurred in 6 of 7 babies. Three babies required ventriculoperitoneal shunting or third ventriculostomy. Functional motor level was the same or better than the anatomical level in 6 of 7 cases. There was no significant maternal morbidity and no evidence of myometrial thinning or dehiscence. However, surgeries were complicated by premature rupture of membrane and prematurity. CONCLUSION: Our study suggests that the antenatal treatment of OSB using a fetoscopic approach and our unique surgical technique can result in a watertight seal, reversal of the hindbrain herniation, and better than expected motor function. Our technique differs substantially from the classic repair of OSB used in prior open fetal surgery and fetoscopic studies, in which the dura mater is dissected and the defect is closed in multiple layers. Instead, we use a biocellulose patch placed over the lesion and simple closure of the skin. As such, our technique is an alternative to the current paradigms in the antenatal treatment of OSB. Our clinical outcomes are in line with the results of our extensive prior animal work. Maternal benefits of our approach and technique include minimal morbidity and no myometrial legacy. Current limitations of the approach include potential loss of access, premature rupture of membranes, and attendant prematurity. Phase II trials are needed to prevent these complications and to further assess the risks and benefits of our distinct surgical approach and technique.
BACKGROUND: A recent randomized clinical trial named Management of Myelomeningocele Study (MOMS trial) showed that prenatal correction of open spina bifida (OSB) via open fetal surgery was associated with improved infant neurological outcomes relative to postnatal repair, but at the expense of increased maternal morbidity. OBJECTIVE: We sought to report the final results of our phase I trial (Cirurgia Endoscópica para Correção Antenatal da Meningomielocele [CECAM]) on the feasibility, safety, potential benefits, and side effects of the fetoscopic treatment of OSB using our unique surgical technique. STUDY DESIGN: Ten consecutive pregnancies with lumbosacral OSB were enrolled in the study. Surgeries were performed percutaneously under general anesthesia with 3 ports and partial carbon dioxide insufflation. After appropriate surgical positioning of the fetus, the neuroplacode was released with scissors and the skin was undermined to place a biocellulose patch over the lesion. The skin was closed over the patch using a single running stitch. Preoperative, postoperative, and postnatal magnetic resonance imaging were performed to assess hindbrain herniation. Neurodevelopmental evaluation was performed before discharge and at 3, 6, and 12 months. All cases were delivered by cesarean delivery, at which time the uterus was assessed for evidence of thinning or dehiscence. RESULTS: The median gestational age at the time of surgery was 27 weeks (range 25-28 weeks). Endoscopic repair was completed in 8 of 10 fetuses. Two cases were unsuccessful due to loss of uterine access. The mean gestational age at birth was 32.4 weeks with a mean latency of 5.6 weeks between surgery and delivery (range 2-8 weeks). There was 1 fetal and 1 neonatal demise, and 1 unsuccessful case underwent postnatal repair. Of the 7 infants available for analysis, complete reversal of hindbrain herniation occurred in 6 of 7 babies. Three babies required ventriculoperitoneal shunting or third ventriculostomy. Functional motor level was the same or better than the anatomical level in 6 of 7 cases. There was no significant maternal morbidity and no evidence of myometrial thinning or dehiscence. However, surgeries were complicated by premature rupture of membrane and prematurity. CONCLUSION: Our study suggests that the antenatal treatment of OSB using a fetoscopic approach and our unique surgical technique can result in a watertight seal, reversal of the hindbrain herniation, and better than expected motor function. Our technique differs substantially from the classic repair of OSB used in prior open fetal surgery and fetoscopic studies, in which the dura mater is dissected and the defect is closed in multiple layers. Instead, we use a biocellulose patch placed over the lesion and simple closure of the skin. As such, our technique is an alternative to the current paradigms in the antenatal treatment of OSB. Our clinical outcomes are in line with the results of our extensive prior animal work. Maternal benefits of our approach and technique include minimal morbidity and no myometrial legacy. Current limitations of the approach include potential loss of access, premature rupture of membranes, and attendant prematurity. Phase II trials are needed to prevent these complications and to further assess the risks and benefits of our distinct surgical approach and technique.
Authors: R M Antiel; C A Collura; A W Flake; M P Johnson; N E Rintoul; J D Lantos; F A Curlin; J C Tilburt; S D Brown; C Feudtner Journal: J Perinatol Date: 2017-06-15 Impact factor: 2.521
Authors: R A Didier; J S Martin-Saavedra; E R Oliver; S E DeBari; L T Bilaniuk; L J Howell; J S Moldenhauer; N S Adzick; G G Heuer; B G Coleman Journal: AJNR Am J Neuroradiol Date: 2020-09-17 Impact factor: 3.825
Authors: Ahmet A Baschat; Sean B Blackwell; Debnath Chatterjee; James J Cummings; Stephen P Emery; Shinjiro Hirose; Lisa M Hollier; Anthony Johnson; Sarah J Kilpatrick; Francois I Luks; M Kathryn Menard; Lawrence B McCullough; Julie S Moldenhauer; Anita J Moon-Grady; George B Mychaliska; Michael Narvey; Mary E Norton; Mark D Rollins; Eric D Skarsgard; KuoJen Tsao; Barbara B Warner; Abigail Wilpers; Greg Ryan Journal: Obstet Gynecol Date: 2022-05-02 Impact factor: 7.623