Spinal dysraphism is a cause of significant morbidity in both children and adults. The occult spinal dysraphic states are characterized by a failure of fusion of midline, neural or bony structures, with intact overlying skin. Open spinal dysraphic lesions, the myelomeningocele being the most common, are manifest by a failure of fusion of all midline tissue elements, including skin. Early and aggressive repair of the myelomeningocele is advocated to reduce infectious complications. In general, patients with spinal dysraphism will require a lifetime of close neurological follow-up. Further developments in the area of spinal dysraphism will include the potential for antenatal repair as well as other neuroprotective strategies for spinal and brain dysfunction.

Suggested Readings

1. Bruner JP, Tulipan N, Paschall RL et al. Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 1999; 282:1819-1825.

2. Cohen AR, Robinson S. Myelomeningocele and myelocystocele. In: Winn HR, ed. Neurological Surgery. 5th ed. Philadephia: W.B. Saunders, 2004:3215-3228.

3. Hunt GM, Oakeshott P. Outcome in people with open spina bifida at age 35: prospective community based cohort study. BMJ 2003; 326:1365-6.

4. Keating RF, Multani J, Cogen PH. Occult spinal dysraphism and the tethered spinal cord. In: Winn HR, ed. Neurological Surgery. 5th ed. Philadephia: W.B. Saunders, 2004:3257-3283.

5. Keith L. Moore KL, Persaud TVN. The developing human: Clinically Oriented Embyrology. 7th ed. Philadelphia: Saunders, 2003:451-487.

6. MRC Vitamin Study Research Group. Prevention of neural tube defects: results of the Medical Research Council Vitamin Study. Lancet 1991; 338:131-137.

7. Toriello H, Higgins J. Occurrence of neural tube defects among first-, second-, and third-degree relatives of probands: Results of a United States study. Am J Med Genet 1983; 15: 601-606.

8. Embryology on the Internet—Offers a comprehensive pictoral view of human embryogenesis. contents.htm—A tutorial for human embryology using well-labeled biological specimens.

Chapter 9

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