Introduction: Myelomeningocele patients deal with multiple medical issues, including lower extremity neurological deficits, bowel and bladder incontinence and the sequelae of hydrocephalus secondary to a Chiari II malformation. In utero intervention holds the promise of reversing some of the sequelae and improving outcome. Material and Methods: Between 1998 and 2003 (preceding the formal Management of Myelomeningocele Study, MOMS), an initial group of 58 patients underwent in utero repair of their myelomeningocele between 21 and 25 weeks' gestation. Long-term (5-year) follow-up has occurred in this cohort of patients. Previous reports have documented decreased incidence of ventriculoperitoneal shunting and neuromotor functioning, showing improved outcomes compared with historical controls. Results: Overall, 4 fetal deaths occurred, while the majority of patients returned for follow-up for up to 5 years after closure. Phone follow-up has also been conducted for those who could not return. To date, 10 patients (18.5%) have successfully toilet-trained, while 2 patients have bowel continence and 1 has bladder continence but requires enemas; 2 patients who successfully toilet-trained developed spinal dermoid cysts requiring surgical resection. Discussion: Historically, in utero repair of myelomeningocele patients yields a greater percentage of patients who have achieved continence compared with those undergoing postnatal repair. The MOMS trial will compare contemporary urological outcomes of those patients undergoing either prenatal or postnatal repair in a randomized fashion. The results of this trial showed a decreased need for ventriculoperitoneal shunting in those patients who underwent in utero repair as well as an improvement in lower extremity function.

1.
Adzick NS: Management of fetal lung lesions. Clin Perinatol 2009;36:363-376.
2.
Bauer SB: The management of myelodysplastic child: a paradigm shift. BJU Int 2003;92:23.
3.
Kaefer M, Pabby A, Kelly M, Darby M, Bauer SB: Improved bladder function after prophylactic treatment of the high risk neurogenic bladder in newborns with myelomeningocele. J Urol 1999;162:1068.
4.
Park JM, McGuire EJ, Koo HP, Schwartz AC, Garwood CK, Bloom DA: External urethral sphincter dilation for the management of high risk myelomeningocele: 15-year experience. J Urol 2001;165:2383.
5.
Koff SA, Gigax MR, Jayanthi VR: Nocturnal bladder emptying: a simple technique for reversing urinary tract deterioration in children with neurogenic bladder. J Urol 2005;174:1629.
6.
Schulte-Baukloh H, Krispel HH, Michael T: Botulism - a toxin in the treatment of neurogenic bladder in children. Pediatrics 2002;110:420.
7.
Marte A: Onabotulinumtoxin A for treating overactive/poor compliant bladders in children and adolescents with neurogenic bladder secondary to myelomeningocele. Toxins (Basel) 2012;5:16-24.
8.
Chancellor MB, Patel V, Leng WW, Shenot PJ, Lam W, Globe DR, Loeb AL, Chapple CR: OnabotulinumtoxinA improves quality of life in patients with neurogenic detrusor overactivity. Neurology 2013;81:841-848.
9.
Kask M, Rintala R, Taskinen S: Effect of onabotulinumtoxinA treatment on symptoms and urodynamic findings in pediatric neurogenic bladder. J Pediatr Urol 2014;10:280-283.
10.
Meuli M, Meuli-Simmer C, Hutchins GM, Yingling CD, Hoffman KM, Harrison MR, et al: In utero surgery rescues neurological function at birth in sheep with spina bifida. Nat Med 1995;1:342.
11.
Holzbeierlein J, Pope JV IV, Adams MC, Bruner J, Tulipan N, Brock JW 3rd: The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation. J Urol 2000;164:1336.
12.
Holmes NM, Nguyen HT, Harrison MR, Farmer DL, Baskin LS: Fetal intervention for myelomeningocele: effect on postnatal bladder function. J Urol 2001;166:2393.
13.
Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D'Alton ME, Farmer DL; MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011;364:993-1004.
14.
Johnson MP, Adzick NS, Rintoul N, Crombleholme TM, Flake AW, Howell LJ, Hedrick HL, Wilson RD, Sutton LN: Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol 2003;189:482-487.
15.
Akbal C, Genc Y, Burgu B, Ozden E, Tekgul S: Dysfunctional voiding and incontinence scoring system: quantitative evaluation of incontinence symptoms in pediatric population. J Urol 2005;173:969-973.
16.
Schast AP, Zderic SA, Richter M, Berry A, Carr MC: Quantifying demographic, urological and behavioral characteristics of children with lower urinary tract symptoms. J Pediatr Urol 2008;4:127-133.
17.
Koh CJ, DeFelippo RE, Borer JG, Khoshbin S, Bauer SB: Bladder and external sphincter function after prenatal closure of myelomeningocele. J Urol 2006;176:2232.
18.
Sutton LN, Adzick NS, Bilaniuk LT, Johnson MP, Crombleholme TM, Flake AW: Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA 1999;282:1826.
19.
Bruner JP, Tjlipan N, Paschall RL, Boehm FH, Walsh WF, Silva SR, et al: Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 1999;282:1819-1825.
20.
Jackson EM, Schwartz DM, Sestokas AK, Zarnow DM, Adzick NS, Johnson MP, Heuer GG, Sutton LN: Intraoperative neurophysiological monitoring in patients undergoing tethered cord surgery after fetal myelomeningocele repair. J Neurosurg Pediatr 2014;13:355-361.
21.
Danzer E, Gerdes M, Bebbington MW, Sutton LN, Melchionni J, Adzick NS, Wilson RD, Johnson MP: Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery. Fetal Diagn Ther 2009;25:47-53.
22.
Tarcan T, Bauer S, Olmedo E, Khoshbin S, Kelly M, Darbey M: Long-term follow-up of newborns with myelodysplasia and normal urodynamic findings: is follow-up necessary? J Urol 2001;165:564-567.
23.
Mazzola CA, Albright AL, Sutton LN, Tuite GF, Hamilton RL, Pollack IF: Dermoid inclusion cysts and early spinal cord tethering after fetal surgery for myelomeningocele. N Engl J Med 2002;349:256.
24.
Danzer E, Adzick NS, Rintoul NE, Zarnow DM, Schwartz ES, Melchionni J, Ernst LM, Flake AW, Sutton LN, Johnson MP: Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings. J Neurosurg Pediatr 2008;2:406-413.
25.
Herman JM, McLone DG, Stoers BB, Dauser RC: Analysis of 153 patients with myelomeningocele or spinal lipoma reoperated upon for a tethered cord. Pediatr Neurosurg 1993;19:243.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.