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Nalin Gupta, Jody A. Farrell, Larry Rand, Charles B. Cauldwell and Diana Farmer

S uccessful fetal surgery requires the active participation and interaction of several clinical teams ( Table 1 ). Each group has a specific role with overlap often required at different points of the evaluation and treatment plan. Similar to other multidisciplinary groups, a weekly case discussion allows for a review of prospective patients and their preoperative considerations, as well as ongoing follow-up of postoperative patients. Extensive counseling with the patient and family is critical before the patient can be expected to reach a carefully

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Sergio Cavalheiro, Marcos Devanir Silva da Costa, Jardel Mendonça Nicácio, Patricia Alessandra Dastoli, Italo Capraro Suriano, Mauricio Mendes Barbosa, Hérbene Jose Milani, Stéphanno Gomes Pereira Sarmento, Tereza Cristina Carbonari de Faria and Antonio Fernandes Moron

addition, fetal MRI displays the anatomical details of the malformation, which confirms the ultrasound-based diagnoses and identifies possible associated anomalies. 5 , 11 Moreover, the results of the Management of Myelomeningocele Study (MOMS), which support the efficacy of fetal surgery for myelomeningocele, 12 encouraged our group to correct some cases of OE, aiming to reduce the progression of microcephaly and neural tissue extrusion into the herniated sac. In this study, we retrospectively reviewed these cases and demonstrate the technical aspects of an innovative

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Vijay M. Ravindra, Guillermo Aldave, Howard L. Weiner, Timothy Lee, Michael A. Belfort, Magdelena Sanz-Cortes, Jimmy Espinoza, Alireza A. Shamshirsaz, Ahmed A. Nassr and William E. Whitehead

H istorically , fetal surgery has been limited to use for severe conditions that will result in fetal or neonatal death. All procedural risks to mother and fetus are considered in the setting of almost-certain death without intervention. Recently, the Management of Myelomeningocele Study (MOMS) demonstrated benefits from fetal surgery in children with myelomeningocele (MMC). Since MMC is not uniformly lethal, these benefits are more difficult to balance against the risks of intervention. 1 The option for fetal surgery is also limited by time because the surgery

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Maria Licci, Raphael Guzman and Jehuda Soleman

spinal cord lesion and can be even higher than the location of the vertebral defect. 1 , 26 , 27 , 29 The use of maternal-fetal prenatal surgery for nonlethal conditions, such as MMC, is highly controversial. 24 Nonetheless, MMC has been the first, nonlethal condition explored by fetal surgery, due to the increasing rate of early gestational diagnosis through screening programs and advancement of prenatal imaging methods on one hand, and data suggesting a progressive deterioration of both central and peripheral neurological damage during fetal gestation, due to the

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Fetal surgery for spina bifida

JNSPG 75th Anniversary Invited Review Article

Michael C. Dewan and John C. Wellons III

fetal surgery. 29 The sheep that underwent in utero repair exhibited superior neurological function and restoration of normal hindbrain architecture. 4 , 34 Exploration of fetal MM repair (fMMR) in humans began via an endoscopic approach in the early 1990s in several centers in the US and Europe. 8 , 15 , 24 Dismal results in these early series shifted the field toward open techniques, such that by the end of the millennium, several institutions produced more encouraging data for fMMR. Investigators at Vanderbilt University and Children’s Hospital of Philadelphia

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Giorgio Carrabba, Francesco Macchini, Isabella Fabietti, Luigi Schisano, Giulia Meccariello, Rolando Campanella, Giulio Bertani, Marco Locatelli, Simona Boito, Giuliana A. Porro, Lorenzo Gabetta, Odoardo Picciolini, Claudia Cinnante, Fabio Triulzi, Fabrizio Ciralli, Fabio Mosca, Denise A. Lapa, Ernesto Leva, Paolo Rampini and Nicola Persico

prenatal surgical closure of MMC is to minimize toxic injury to the exposed spinal cord during pregnancy due to amniotic fluid. A randomized controlled study, Management of Myelomeningocele Study (MOMS), was designed to compare the benefits of prenatal repair of the malformation via open fetal surgery with standard postnatal repair. This trial demonstrated a potential role of prenatal surgery in improving neurological outcomes and preventing neurological complications such as hydrocephalus and hindbrain herniation. Indeed, the incidence of hydrocephalus decreased from 82

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Pravesh S. Gadjradj, Jochem K. H. Spoor, Alex J. Eggink, René Wijnen, Jena L. Miller, Mara Rosner, Mari L. Groves, Philip L. J. DeKoninck, Biswadjiet S. Harhangi, Ahmet Baschat, Marie-Lise van Veelen and Tjeerd H. R. de Jong

Initially, fetal surgery served as an option to treat potentially lethal conditions such as congenital diaphragmatic hernia, improving early mortality rates. 19 , 28 With increasing knowledge and experience, the indications for fetal surgery have broadened toward treating nonlethal diseases such as MMC. 1 , 8 At the end of the last century, early case studies of fetal MMC repair showed promising results and eventually led to a randomized controlled trial published in 2011. In the Management of Myelomeningocele Study (MOMS), women diagnosed with a fetus having MMC were

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Noel Tulipan, John C. Wellons III, Elizabeth A. Thom, Nalin Gupta, Leslie N. Sutton, Pamela K. Burrows, Diana Farmer, William Walsh, Mark P. Johnson, Larry Rand, Susan Tolivaisa, Mary E. D’alton and N. Scott Adzick

can be spared the potential risks of intrauterine repair, which include preterm delivery and uterine dehiscence. If this could be accomplished, our ability to counsel pregnant women would be substantially improved, thus simplifying what is currently still a difficult decision-making process for prospective parents and treating physicians alike. Methods The MOMS was conducted by Children’s Hospital of Philadelphia, Vanderbilt University, and the University of California, San Francisco, each an established maternal-fetal surgery center, and by an independent

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Jochem K. H. Spoor, Pravesh S. Gadjradj, Alex J. Eggink, Philip L. J. DeKoninck, Bart Lutters, Jeroen R. Scheepe, Jetty van Meeteren, Peter C. J. de Laat, Marie Lise van Veelen and Tjeerd H. R. de Jong

procedures, and decompression interventions for CMTII in relation to clinical outcome. This series provides a contemporary cohort that could improve counseling parents about the long-term complications and risks of postnatal repair. This also allows comparison to much-awaited long-term outcomes of the MOMS trial, in particular to TSC rates. Furthermore, as our institution is currently transitioning toward the establishment of a fetal surgery center, the present study provides a center-specific historical cohort to compare with future antenatal-treated MMC cases. Methods

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Enrico Danzer, Linda M. Ernst, Natalie E. Rintoul, Mark P. Johnson, N. Scott Adzick and Alan W. Flake

secondarily and progressively damaged by amniotic fluid toxicity, mechanical trauma, hydrodynamic pressure, or a combination of these factors. 9 , 10 , 13 , 18 , 25 Fetal surgical coverage of the exposed spinal cord was recently initiated as a potential treatment for MMC with the goal of protecting the neural placode from the intrauterine environment. 33 Although early clinical experience with in utero MMC closure suggests that midgestational fetal surgery in this highly selected population of children with MMCs may reduce disability related to Chiari malformations Type