Spina Bifida Differential Diagnoses

  • Author: Mark R Foster, MD, PhD, FACS; Chief Editor: Consuelo T Lorenzo, MD   more...
 
Updated: Aug 25, 2011
 
 

Diagnostic Considerations

Meningocele and myelomeningocele must be differentiated. Meningocele is the herniated protrusion of only the meninges through a defect in the cranium or vertebral column. This lesion does not contain neural tissue in the sac.

Spina bifida occulta is a common radiographic finding characterized by simple lack of fusion of vertebral spinous processes. The spinal cord itself is normal.

Proceed to Workup
 
 
Contributor Information and Disclosures
Author

Mark R Foster, MD, PhD, FACS  President and Orthopedic Surgeon, Orthopedic Spine Specialists of Western Pennsylvania, PC

Mark R Foster, MD, PhD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Surgeons, American Physical Society, Christian Medical & Dental Society, Eastern Orthopaedic Association, North American Spine Society, Orthopaedic Research Society, and Pennsylvania Orthopaedic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Kat Kolaski, MD  Assistant Professor, Departments of Orthopedic Surgery and Pediatrics, Wake Forest University School of Medicine

Kat Kolaski, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine and American Academy of Physical Medicine and Rehabilitation

Disclosure: Nothing to disclose.

Lee H Riley III, MD  Chief, Division of Orthopedic Spine Surgery, Associate Professor, Departments of Orthopedic Surgery and Neurosurgery, Johns Hopkins University School of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Teresa L Massagli, MD  Professor of Rehabilitation Medicine and Pediatrics, University of Washington School of Medicine

Teresa L Massagli, MD is a member of the following medical societies: American Academy of Pediatrics, American Academy of Physical Medicine and Rehabilitation, and Association of Academic Physiatrists

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Kat Kolaski, MD  Assistant Professor, Departments of Orthopedic Surgery and Pediatrics, Wake Forest University School of Medicine

Kat Kolaski, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine and American Academy of Physical Medicine and Rehabilitation

Disclosure: Nothing to disclose.

Mary Ann E Keenan, MD  Professor, Vice Chair for Graduate Medical Education, Department of Orthopedic Surgery, University of Pennsylvania School of Medicine; Chief of Neuro-Orthopedics Program, Department of Orthopedic Surgery, Hospital of the University of Pennsylvania

Mary Ann E Keenan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Orthopaedic Foot and Ankle Society, American Society for Surgery of the Hand, and Orthopaedic Rehabilitation Association

Disclosure: Nothing to disclose.

Chief Editor

Consuelo T Lorenzo, MD  Physiatrist, Department of Physical Medicine and Rehabilitation, Alegent Health, Immanuel Rehabilitation Center

Consuelo T Lorenzo, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation

Disclosure: Nothing to disclose.

References

  1. Vinck A, Nijhuis-van der Sanden MW, Roeleveld NJ, et al. Motor profile and cognitive functioning in children with spina bifida. Eur J Paediatr Neurol. Jan 2010;14(1):86-92. [Medline].

  2. Thompson DN. Postnatal management and outcome for neural tube defects including spina bifida and encephalocoeles. Prenat Diagn. Apr 2009;29(4):412-9. [Medline].

  3. Fletcher JM, Copeland K, Frederick JA, et al. Spinal lesion level in spina bifida: a source of neural and cognitive heterogeneity. J Neurosurg. Apr 2005;102(3 Suppl):268-79. [Medline].

  4. McLone DG, Knepper PA. The cause of Chiari II malformations: a unified theory. Pediatr Neurosci. 1989;15:1-12.

  5. Ausili E, Focarelli B, Tabacco F, et al. Bone mineral density and body composition in a myelomeningocele children population: effects of walking ability and sport activity. Eur Rev Med Pharmacol Sci. Nov-Dec 2008;12(6):349-54. [Medline].

  6. Wu YW, Croen LA, Henning L, et al. Potential association between infertility and spinal neural tube defects in offspring. Birth Defects Res A Clin Mol Teratol. Oct 2006;76(10):718-22. [Medline].

  7. Canfield MA, Ramadhani TA, Shaw GM, et al. Anencephaly and spina bifida among Hispanics: maternal, sociodemographic, and acculturation factors in the National Birth Defects Prevention Study. Birth Defects Res A Clin Mol Teratol. Jul 2009;85(7):637-46. [Medline].

  8. Milunsky A, Jick H, Jick SS, Bruell CL, MacLaughlin DS, Rothman KJ, et al. Multivitamin/folic acid supplementation in early pregnancy reduces the prevalence of neural tube defects. JAMA. Nov 24 1989;262(20):2847-52. [Medline].

  9. Centers for Disease Control and Prevention. Spina bifida and anencephaly before and after folic acid mandate--United States, 1995-1996 and 1999-2000. MMWR Morb Mortal Wkly Rep. May 7 2004;53(17):362-5. [Medline].

  10. Bell KN, Oakley GP Jr. Update on prevention of folic acid-preventable spina bifida and anencephaly. Birth Defects Res A Clin Mol Teratol. Jan 2009;85(1):102-7. [Medline].

  11. Holmes LB. Does taking vitamins at the time of conception prevent neural tube defects?. JAMA. Dec 2 1988;260(21):3181. [Medline].

  12. Mulinare J, Cordero JF, Erickson JD, Berry RJ. Periconceptional use of multivitamins and the occurrence of neural tube defects. JAMA. Dec 2 1988;260(21):3141-5. [Medline].

  13. Cotter AM, Daly SF. Neural tube defects: is a decreasing prevalence associated with a decrease in severity?. Eur J Obstet Gynecol Reprod Biol. Apr 1 2005;119(2):161-3. [Medline].

  14. Canfield MA, Marengo L, Ramadhani TA, Suarez L, Brender JD, Scheuerle A. The prevalence and predictors of anencephaly and spina bifida in Texas. Paediatr Perinat Epidemiol. Jan 2009;23(1):41-50. [Medline].

  15. Cotton P. Finding neural tube 'zippers' may let geneticists tailor prevention of defects. JAMA. Oct 13 1993;270(14):1663-4. [Medline].

  16. Lemire RJ. Neural tube defects. JAMA. Jan 22-29 1988;259(4):558-62. [Medline].

  17. Velie EM, Shaw GM, Malcoe LH, et al. Understanding the increased risk of neural tube defect-affected pregnancies among Mexico-born women in California: immigration and anthropometric factors. Paediatr Perinat Epidemiol. May 2006;20(3):219-30. [Medline].

  18. Williams LJ, Rasmussen SA, Flores A, et al. Decline in the prevalence of spina bifida and anencephaly by race/ethnicity: 1995-2002. Pediatrics. Sep 2005;116(3):580-6. [Medline].

  19. Lindquist B, Uvebrant P, Rehn E, Carlsson G. Cognitive functions in children with myelomeningocele without hydrocephalus. Childs Nerv Syst. Aug 2009;25(8):969-75. [Medline].

  20. Vachha B, Adams R. Implications of family environment and language development: comparing typically developing children to those with spina bifida. Child Care Health Dev. Sep 2009;35(5):709-16. [Medline].

  21. Dias MS. Neurosurgical causes of scoliosis in patients with myelomeningocele: an evidence-based literature review. J Neurosurg. Jul 2005;103(1 Suppl):24-35. [Medline].

  22. Meeks JJ, Hagerty JA, Chaviano AH. Bulbar urethral ligation for managing persistent urinary incontinence in young men with myelomeningocele. BJU Int. Jul 2009;104(2):221-4. [Medline].

  23. Lewis D, Tolosa JE, Kaufmann M, Goodman M, Farrell C, Berghella V. Elective cesarean delivery and long-term motor function or ambulation status in infants with meningomyelocele. Obstet Gynecol. Mar 2004;103(3):469-73. [Medline].

  24. Danzer E, Gerdes M, Bebbington MW, et al. Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery. Fetal Diagn Ther. 2009;25(1):47-53. [Medline].

  25. Fichter MA, Dornseifer U, Henke J, et al. Fetal spina bifida repair--current trends and prospects of intrauterine neurosurgery. Fetal Diagn Ther. 2008;23(4):271-86. [Medline].

  26. Adzick NS, Thom EA, Spong CY, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. Mar 17 2011;364(11):993-1004. [Medline].

  27. Alman BA, Bhandari M, Wright JG. Function of dislocated hips in children with lower level spina bifida. J Bone Joint Surg Br. Mar 1996;78(2):294-8. [Medline].

  28. Eichholzer M, Tönz O, Zimmermann R. Folic acid: a public-health challenge. Lancet. Apr 22 2006;367(9519):1352-61. [Medline].

  29. Robbins JM, Tilford JM, Bird TM, et al. Hospitalizations of newborns with folate-sensitive birth defects before and after fortification of foods with folic acid. Pediatrics. Sep 2006;118(3):906-15. [Medline].

  30. De Wals P, Tairou F, Van Allen MI, et al. Reduction in neural-tube defects after folic acid fortification in Canada. N Engl J Med. Jul 12 2007;357(2):135-42. [Medline].

  31. De Wals P, Tairou F, Van Allen MI, et al. Spina bifida before and after folic acid fortification in Canada. Birth Defects Res A Clin Mol Teratol. Sep 2008;82(9):622-6. [Medline].

  32. Oakley GP Jr. The scientific basis for eliminating folic acid-preventable spina bifida: a modern miracle from epidemiology. Ann Epidemiol. Apr 2009;19(4):226-30. [Medline].

 
Previous
Next
 
The lumbar region of a newborn baby with myelomeningocele. The skin is intact, and the placode-containing remnants of nervous tissue can be observed in the center of the lesion, which is filled with cerebrospinal fluid.
Myelomeningocele in a newborn.
T1-weighted, coronal magnetic resonance imaging (MRI) scans of the brain show a Chiari II malformation. Note the stretching of the brainstem, aqueduct, and fourth ventricle.
Neonate with a lumbar myelomeningocele with an L5 neurologic level. Note the diaphanous sac filled with cerebrospinal fluid and containing fragile vessels in its membrane. Also, note the neural placode plastered to the dorsal surface of the sac. This patient underwent closure of his back and an untethering of his neural placode. The neural placode was circumnavigated and placed in the neural canal. A dural sleeve was fashioned in a way that reconstructed neural tube geometry.
Sagittal, T1-weighted magnetic resonance imaging (MRI) scan of a child after closure of his myelomeningocele. Child is aged 7 years. Note the spinal cord ends in the sacral region far below the normal level of T12-L1. It is tethered at the point at which the neural placode was attached to the skin defect during gestation. The MRI scan showed dorsal tethering, and the child complained of back pain and had a new foot deformity on examination. By definition, all children with a myelomeningocele have a tethered cord on MRI, but only about 20% of children require an operation to untether the spinal cord during their first decade of life, during their rapid growth spurts. Thus, the MRI scan must be placed in context of a history and examination consistent with mechanical tethering and a resultant neurologic deterioration.
Axial T1-weighted MRI scan of a 15-year-old girl who was born with thoracic myelomeningocele, hydrocephalus, and Arnold-Chiari II syndrome. She was treated with a ventriculoperitoneal shunt. The ventricular system has a characteristic shape, with small frontal and large occipital horns, which are typical in patients with spina bifida. The shunt tube is shown in the right parietal region.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.