Overview of the management of myelomeningocele (spina bifida)
- David G McLone, MD, PhD
David G McLone, MD, PhD
- Professor of Pediatric Neurosurgery
- Northwestern University's Feinberg School of Medicine
- Ann and Robert Lurie Children's Hospital of Chicago
- Robin M Bowman, MD
Robin M Bowman, MD
- Associate Professor of Neurological Surgery
- Northwestern University's Feinberg School of Medicine
- Ann and Robert H Lurie Children's Hospital of Chicago
- Section Editors
- Marc C Patterson, MD, FRACP
Marc C Patterson, MD, FRACP
- Section Editor — Pediatric Neurology
- Professor of Neurology, Pediatrics, and Medical Genetics
- Chair, Division of Child and Adolescent Neurology
- Mayo Clinic College of Medicine
- Leonard E Weisman, MD
Leonard E Weisman, MD
- Section Editor — Neonatology
- Professor of Pediatrics
- Baylor College of Medicine
- Teresa K Duryea, MD
Teresa K Duryea, MD
- Section Editor — General Pediatrics
- Associate Professor of Pediatrics
- Baylor College of Medicine
Neural tube defects (NTDs), are the most common congenital central nervous system anomaly, and are the cause of chronic disability of between 70,000 and 100,000 individuals in the United States. Myelomeningocele (spina bifida) is the most common NTD. It is characterized by a cleft in the vertebral column, with a corresponding defect in the skin so that the meninges and spinal cord are exposed. Patients with myelomeningocele may have weakness and absence of sensation affecting the lower extremities and bowel/bladder dysfunction, depending upon the level of the spinal lesion.
An overview of the management of myelomeningocele and its complications will be presented here. Occult spinal dysraphism is discussed separately. The pathophysiology, clinical manifestations, prenatal diagnosis, orthopedic management, and urologic management of myelomeningocele are also discussed separately:
- Wilson RD, SOGC GENETICS COMMITTEE, SPECIAL CONTRIBUTOR. Prenatal screening, diagnosis, and pregnancy management of fetal neural tube defects. J Obstet Gynaecol Can 2014; 36:927.
- Johnson CY, Honein MA, Dana Flanders W, et al. Pregnancy termination following prenatal diagnosis of anencephaly or spina bifida: a systematic review of the literature. Birth Defects Res A Clin Mol Teratol 2012; 94:857.
- Sutton LN. Fetal surgery for neural tube defects. Best Pract Res Clin Obstet Gynaecol 2008; 22:175.
- Adzick NS, Thom EA, Spong CY, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364:993.
- Brock JW 3rd, Carr MC, Adzick NS, et al. Bladder Function After Fetal Surgery for Myelomeningocele. Pediatrics 2015; 136:e906.
- Tulipan N, Wellons JC 3rd, Thom EA, et al. Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr 2015; 16:613.
- Moldenhauer JS, Soni S, Rintoul NE, et al. Fetal myelomeningocele repair: the post-MOMS experience at the Children's Hospital of Philadelphia. Fetal Diagn Ther 2015; 37:235.
- Committee on Obstetric Practice, Society for Maternal–Fetal Medicine. Committee Opinion No. 720: Maternal-Fetal Surgery for Myelomeningocele. Obstet Gynecol 2017; 130:e164.
- Cohen AR, Couto J, Cummings JJ, et al. Position statement on fetal myelomeningocele repair. Am J Obstet Gynecol 2014; 210:107.
- Luthy DA, Wardinsky T, Shurtleff DB, et al. Cesarean section before the onset of labor and subsequent motor function in infants with meningomyelocele diagnosed antenatally. N Engl J Med 1991; 324:662.
- Cohen AR, Robinson S. Early management of myelomeningocele. In: Pediatric neurosurgery, McLone DG (Ed), WB Saunders, Philadelphia 2001. p.241.
- Rendeli C, Nucera E, Ausili E, et al. Latex sensitisation and allergy in children with myelomeningocele. Childs Nerv Syst 2006; 22:28.
- Cremer R, Kleine-Diepenbruck U, Hoppe A, Bläker F. Latex allergy in spina bifida patients--prevention by primary prophylaxis. Allergy 1998; 53:709.
- McLone DG. Care of the neonate with a myelomeningocele. Neurosurg Clin N Am 1998; 9:111.
- Charney EB, Melchionni JB, Antonucci DL. Ventriculitis in newborns with myelomeningocele. Am J Dis Child 1991; 145:287.
- Burke R, Liptak GS, Council on Children with Disabilities. Providing a primary care medical home for children and youth with spina bifida. Pediatrics 2011; 128:e1645.
- McLone DG. Technique for closure of myelomeningocele. Childs Brain 1980; 6:65.
- McLone DG, Dias MS. Complications of myelomeningocele closure. Pediatr Neurosurg 1991-1992; 17:267.
- Chakraborty A, Crimmins D, Hayward R, Thompson D. Toward reducing shunt placement rates in patients with myelomeningocele. J Neurosurg Pediatr 2008; 1:361.
- Davis BE, Daley CM, Shurtleff DB, et al. Long-term survival of individuals with myelomeningocele. Pediatr Neurosurg 2005; 41:186.
- Miller PD, Pollack IF, Pang D, Albright AL. Comparison of simultaneous versus delayed ventriculoperitoneal shunt insertion in children undergoing myelomeningocele repair. J Child Neurol 1996; 11:370.
- Bowman RM, McLone DG. Tethered cord in children with spina bifida. In: Spina Bifida: Management and outcome, Ozek MM (Ed), Springer, Milan 2008.
- Hoffman HJ, Hendrick EB, Humphreys RP. The tethered spinal cord: its protean manifestations, diagnosis and surgical correction. Childs Brain 1976; 2:145.
- Yamada S, Won DJ, Yamada SM. Pathophysiology of tethered cord syndrome: correlation with symptomatology. Neurosurg Focus 2004; 16:E6.
- Bowman RM, McLone DG, Grant JA, et al. Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 2001; 34:114.
- Mehta VA, Bettegowda C, Ahmadi SA, et al. Spinal cord tethering following myelomeningocele repair. J Neurosurg Pediatr 2010; 6:498.
- Miller E, Widjaja E, Blaser S, et al. The old and the new: supratentorial MR findings in Chiari II malformation. Childs Nerv Syst 2008; 24:563.
- Messing-Jünger M, Röhrig A. Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst 2013; 29:1553.
- Volpe JJ. Intracranial hemorrhage: Neural tube formation and prosencephalic development. In: Neurology of the Newborn, 4th ed, WB Saunders, Philadelphia 2001. p.3.
- Nagler J, Levy JA, Bachur RG. Stridor in an infant with myelomeningocele. Pediatr Emerg Care 2007; 23:478.
- La Marca F, Herman M, Grant JA, McLone DG. Presentation and management of hydromyelia in children with Chiari type-II malformation. Pediatr Neurosurg 1997; 26:57.
- Dennis M, Hetherington R, Speigler BJ, et al. Functional consequences of congenital cerebellar dysmorphologies and acquired cerebellar lesions of childhood. In: The changing nervous system - neurobehavioural consequences of early brain disorder, Broman SH, Fletcher JM (Eds), Oxford, New York 1999.
- McLone DG, Czyzewski D, Raimondi AJ, Sommers RC. Central nervous system infections as a limiting factor in the intelligence of children with myelomeningocele. Pediatrics 1982; 70:338.
- Matson MA, Mahone EM, Zabel TA. Serial neuropsychological assessment and evidence of shunt malfunction in spina bifida: a longitudinal case study. Child Neuropsychol 2005; 11:315.
- Fletcher JM, Brookshire BL, Landry SH, et al. Attentional skills and executive functions in children with early hydrocephalus. Dev Neuropsychol 1996; 12:53.
- Lindquist B, Persson EK, Uvebrant P, Carlsson G. Learning, memory and executive functions in children with hydrocephalus. Acta Paediatr 2008; 97:596.
- Dennis M, Barnes MA. The cognitive phenotype of spina bifida meningomyelocele. Dev Disabil Res Rev 2010; 16:31.
- Holmbeck GN, Westhoven VC, Phillips WS, et al. A multimethod, multi-informant, and multidimensional perspective on psychosocial adjustment in preadolescents with spina bifida. J Consult Clin Psychol 2003; 71:782.
- Sawin KJ, Liu T, Ward E, et al. The National Spina Bifida Patient Registry: profile of a large cohort of participants from the first 10 clinics. J Pediatr 2015; 166:444.
- Smith K, Neville-Jan A, Freeman KA, et al. The effectiveness of bowel and bladder interventions in children with spina bifida. Dev Med Child Neurol 2016; 58:979.
- Mattsson S, Gladh G. Tap-water enema for children with myelomeningocele and neurogenic bowel dysfunction. Acta Paediatr 2006; 95:369.
- López Pereira P, Salvador OP, Arcas JA, et al. Transanal irrigation for the treatment of neuropathic bowel dysfunction. J Pediatr Urol 2010; 6:134.
- Ausili E, Focarelli B, Tabacco F, et al. Transanal irrigation in myelomeningocele children: an alternative, safe and valid approach for neurogenic constipation. Spinal Cord 2010; 48:560.
- Pacilli M, Pallot D, Andrews A, et al. Use of Peristeen® transanal colonic irrigation for bowel management in children: a single-center experience. J Pediatr Surg 2014; 49:269.
- Van Savage JG, Yohannes P. Laparoscopic antegrade continence enema in situ appendix procedure for refractory constipation and overflow fecal incontinence in children with spina bifida. J Urol 2000; 164:1084.
- Webb HW, Barraza MA, Stevens PS, et al. Bowel dysfunction in spina bifida--an American experience with the ACE procedure. Eur J Pediatr Surg 1998; 8 Suppl 1:37.
- Curry JI, Osborne A, Malone PS. The MACE procedure: experience in the United Kingdom. J Pediatr Surg 1999; 34:338.
- Chu DI, Balsara ZR, Routh JC, et al. Experience with glycerin for antegrade continence enema in patients with neurogenic bowel. J Urol 2013; 189:690.
- Ekmark EM. Risky business: Preventing skin breakdown in children with spina bifida. J Pediatr Rehabil Med 2009; 2:37.
- Tennant PW, Pearce MS, Bythell M, Rankin J. 20-year survival of children born with congenital anomalies: a population-based study. Lancet 2010; 375:649.
- Oakeshott P, Reid F, Poulton A, et al. Neurological level at birth predicts survival to the mid-40s and urological deaths in open spina bifida: a complete prospective cohort study. Dev Med Child Neurol 2015.
- Danzer E, Gerdes M, Bebbington MW, et al. Preschool neurodevelopmental outcome of children following fetal myelomeningocele closure. Am J Obstet Gynecol 2010; 202:450.e1.
- Danzer E, Thomas NH, Thomas A, et al. Long-term neurofunctional outcome, executive functioning, and behavioral adaptive skills following fetal myelomeningocele surgery. Am J Obstet Gynecol 2016; 214:269.e1.
- PRENATAL COUNSELING AND CHOICE OF MANAGEMENT
- FETAL SURGERY
- LABOR AND DELIVERY
- MANAGEMENT OF THE NEONATE
- Surgical closure
- SUBSEQUENT MANAGEMENT
- Neurologic complications
- - Routine surveillance
- - Shunt malfunction
- - Tethered cord
- - Chiari II
- - Hydromyelia
- - Seizures
- - Learning disabilities
- Urinary tract complications
- Bowel management
- Pressure ulcers
- Orthopedic problems
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS