SPINA BIFIDA – CHILDHOOD NEUROSURGICAL PROBLEM

Authors

  • Mirjana Raičević University Children's Clinic, Belgrade, Serbia
  • Milan Mrdak University Children's Clinic, Belgrade, Serbia
  • Maša Radovanović University Children's Clinic, Belgrade, Serbia
  • Srđan Nikolovski University Children's Clinic, Belgrade, Serbia

DOI:

https://doi.org/10.46793/PP170305009R

Keywords:

spina bifida, neural tube defects, spinal dysraphism, tethered cord syndrome

Abstract

Spina bifida is one of the most complex and serious birth defects. Former term spina bifida today is suppressed by the terms that better describe the essence of anomalies: neural tube defects (NTDS) or spinal dysraphism. In the basis of this anomaly is inadequate "closing – coalescence of center line" of the spine, so the term spinal dysraphism is the most adequate. Midline fusion defect may occur in different levels of the spinal canal, so there are the cervical, thoracic, lumbar and sacral spinal dysraphism. Spinal dysraphism occurs in two basic entities - open and closed, that are fundamentally different. The incidence of this anomaly in the world shows the geographic and ethnic particularity - is most common in the Celtic people (12.5 per 10,000 live births.), and it is rare in Asians and the Negro population. Girls are more often affected than boys. The etiology is still unknown. Diagnosis open spinal dysraphism must be set prenatally. Treatment of open spina bifida is surgical. In the world there are medical centers where it is applied pre-natal - fetal surgery. After birth, surgery should be performed within the first 48 h with appropriate antibiotic support. Newborn should be fully investigated and put on multidisciplinary treatment (neurosurgeon, neurologist, urologist, orthopedist, physiatrist). Closed spinal dysraphism is very insidious, hidden anomalie, and hence the name of occult spinal dysraphism (OSD). The incidence of this anomaly is unknown, it is known only that it is more common in female children. Etiology is also unknown. OSD diagnosis is practically impossible to set prenatally. Tethered cord syndrome - Syndrome of fixed spinal cord, is a condition in which the spinal cord is attached to a fixed structure such as the dura, vertebra, lipoma, skin. Treatment of occult spinal dysraphsm is surgical.

References

Bannister CM. Suggested goals for intrauterine surgery for the repair of myelomeningoceles. Eur J Pediatr Surg. 2000;10 (Suppl I): 42.

Begeer JH, Yierstema GPA, Breukers SME, Mooy JJA, Weeme CA. Tethered cord syndrome; clinical signs and results of operation in 42 patients with spina bifida aperta and occulta. Z.Kinderchir. 1989;44: 5-7.

Jimenez DF, Barone CM. Encephloceles, Meningoceles, and Dermal sinuses in Priciples and Practice of Pediatric Neurosurgery, Thieme; New York, 1999:189-206.

Reigel DH, Rotenstein D. Spina bifida in: Cheek WR, Marlin AE, McLone DG, Reigel DH, Walker ML. Pediatrics Neurosurgery; Surgery of the Developing Nervous system. W.B.Saunders Company; Philadelphia. 1994: 51-76.

Shurtleff DB. 44 years experience with managment of myelomeningocele: presidental adress, socety for reserch into hydrocephalus and spina bifida. Eur pediatr Surg. 2000; l0 (Suppl I):5-8.

French BN. Midline fusion defects and defects of formation in Youmans JR, Neurological Surgery. W.B.Saunders Company; Philadelphia. 1990: 1081-1214.

Lichtenstein BW. Spinal dysraphism, Spina bifida and myelodisplasia. Arch.Neurol. 1940;44:792-809.

Dias MS., McLone DG. Normal and abnormal early development of the nervous sistem in: Pediatric Neurosurgery: surgery of the developing nervous system. W.B.Saunders Company, Philadelphia. 2001:31-66.

Agrawal A, Sampley S. Spinal dysraphism: A challenge continued to be faced by neurosurgeons in developing countries. Asian J Neurosurg. 2014; 9(2): 68-71.

McComb JG. Spinal meningoceles . In :Albright AL, Pollack IF, Adelson PD; Principles and Practice of Pediatric Neurosurgery .Thieme; New York, 1999: 189-208, 271-289.

Park TS. Myelomeningocele. In Albright AL, Pollack IF, Adelson PD; Principles and Practice of Pediatric Neurosurgery. Thieme; New York. 1999:291–320.

Greene NDE, Stanier P, Copp AJ. Genetics of human neural tube defects. Hum Mol Genet. 2009; 18 (2): R113-R129.

Bruner JP, Tulipan N, Paschal RL et al. Fetal surgery for meningomyelocele and the incidence for shunt – dependet hydrocephalus. JAMA. 1999;282:1819-1825.

Iskandar BJ, Oakes WJ: Occult spinal Dysrphism in : Principles and Practice of Pediatric Neurosurgery, Thieme; New York. 1999:321–351.

Nair N, Sreenivas M, Gupta AK, Kandasamy D, Jana M. Neonatal and infantile spinal sonography: A useful investigation often underutilized. Indian J Radiol Imaging. 2016; 26(4): 493-501.

Dhingani DD, Boruah DK, Dutta HK, Gogoi RK. Ultrasonography and magnetic resonance imaging evaluation of pediatric spinal anomalies. J Pediatr Neurosci. 2016; 11(3): 206-212.

Joyeux L, Engels AC, Russo FM, Jimenez J, van Mieghem T, de Coppi P et al. Fetoscopic versus Open Repair for Spina Bifida Aperta: A Systematic Review of Outcomes. Fetal Diagn Ther 2016;39:161-171.

Radcliff E, Cassell CH, Laditka SB, Thibadeau JK, Correia J, Grosse SD, Kirby RS. Factors associated with the timeliness of postnatal surgical repair of spina bifida. Childs Nerv Syst. 2016; 32(8): 1479-1487.

Mishra VV, Nanda S, Aggarwal R, Tanvir. Successful Pregnancy Outcome in an Operated Case of Lipomeningomyocele: A Rare Case. J Clin Diagn Res. 2016; 10 (9): QD04-QD025.

Hoffman HJ, Hendrick EB, Humphreys RB. The tethered spinal cord: its protean manifestiations, diagnosis and surgical correction. Child s Brain. 1976;2:145-155.

Sattar MT, Bannister CM, Turnull IW. Occult spinal Dysraphism-The common combination of lesions and the clinical manifestations in 50 patiens. Eur J.Pediatr Surg 1996;6 (Suppl I): 10-14.

Netto JMB, Bastos AN, Figueiredo AA, Perez LM. Spinal Dysraphism: A Neurosurgical Review for the Urologist. Rev Urol. 2009; 11(2): 71-81.

Riegel DH, McLone DG. Tethered spinal cord. In Cheek WR, Marlin AE, MCLone DG, Reigel DH, Walker ML.Pediatrics Neurosurgery; Surgery of the Developing Nervous system. W.B.Saunders Company; Philadelphia. 1994:77– 95.

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Published

04/28/2017

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Review Articles