Spina bifida: Difference between revisions

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Revision as of 21:47, 9 January 2019


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Classification

  • Spina bifida may be classified according to the level of the lesion into 3 subtypes:[1][2]
  1. Thoracic, high-lumbar
  2. Low-lumbar
  3. Sacral
  • Spina bifida also may be classified according to the tyoe of the vertebrate defect into 2 subtypes:[3][4]
    1. Spina bifida occulta: In this type of spina bifida, the defect of vertebrate is covered by skin ("Occulta" means "hidden"). The spinal cord does not stick out through the skin, although the skin over the lower spine may have a patch of hair, a birthmark, or a dimple above the groove between the buttocks.
    2. Spina ifida aperta: In this type of spina bifida , the defect is widely open and is sub classified into 2 types: Meningocele and Myelomeningocele.
  • Meningocele is a protrusion of the meninges, unaccompanied by neural tissue, through a bony defect in the vertebral column.
  • Myelomeningocele is the most severe form of spina bifida. It happens when both the meninges and the bottom end of the spinal cord push through the hole in the spine, forming a large fluid-filled sac that bulges out of a patients back.

Pathophysiology

  • Spina bifida is a congenital malformation in which the spinal column is split (bifid) as a result of failed closure of the embryonic neural tube, during the fourth week post-fertilization.[3]
  • Spina bifida occulta: In this type of spina bifida, the defect of vertebrate is covered by skin ("Occulta" means "hidden"). The spinal cord does not stick out through the skin, although the skin over the lower spine may have a patch of hair, a birthmark, or a dimple above the groove between the buttocks.[3]
  • Spina ifida aperta: In this type of spina bifida , the defect is widely open and is sub classified into 2 types: Meningocele and Myelomeningocele.[3]

Differentiating spina bifida from other Diseases

  • Spina bifida must be differentiated from other diseases that cause vertebral column defects, such as:

Epidemiology and Demographics

  • The prevalence of spina bifida is approximately 187 to 890 per 100,000 live births.[10]

Age

  • Spina bifida is more commonly observed among preterm newborns.[4]

Gender

  • Female newborns are more commonly affected with spina bifida than male newborns.[11]

Race

  • Spina bifida usually affects individuals of the Malays and Chinese and Indians race.[10]

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Symptoms

Physical examination

  • Physical examination of patients with spina bifida may be remarkable for:[4][3]
  • Spinal area discoloration or birthmarks may be the only sign in the newborns with spina bifida occulta
  • Protrusions in the lumbar spine
  • Dimples in the lumbar spine
  • Hair patch along the spine

Laboratory Findings

  • There are no specific laboratory findings associated with spina bifida.
  • An elevated concentration of Maternal Serum Alpha-Fetoprotein may be predictive for contemporary detection of spina bifida.[17]

Imaging Findings

  • Three-dimensional ultrasound is the imaging modality of choice for characterisation of the open spina bifida spinal lesions.[18]
  • Ultrasound also is the gold standard diagnostic tool for spina bifida.[18]
  • On Three-dimensional ultrasound, spina bifida is characterized by:[19]

Treatment

Surgery

  • Prenatal surgery is the preferred treatment for spina bifida and it usually is done before the 26th week of pregnancy.[20][21]
  • Intrauterine repair of spina bifida confers multiple advantages to infants, including:[21]
  • Surgery after birth is done in patients who did not underwent prenatal surgery but the prognosis is worse and there are more possible complications after surgery in comparison with the prenatal surgery.

Prevention

  • A protective effect of folate against the development of neural tube defects (NTDs), specifically, spina bifida, is now well recognized, having been established by a lot of clinical research studies over the past half-century.[22]
  • Effective measures for the primary prevention of spina bifida include:[23]
    • Not drinking alcohol
    • Not smoking
    • Not taking drugs
    • Taking Folic Acid
  • Taking oral daily folate is recommended for all pregnant women.[22]
  • Neural tube closure is completed 28 days (four weeks) from conception, and the preventive effect of folic acid is not effective after that period.[24]
  • So, folate supplementation should start at least 4 weeks before conception and it should continue until at least two months after conception.[24]
  • The recommended intakes of folate are 4 mg/d for those at high-risk pregnancies(by virtue of a previous NTD pregnancy outcome) and 0.4 mg/d for all others.[22]

References

  1. ↑ Lannering B, Albertsson-Wikland K (July 1989). "Improved growth response to GH treatment in irradiated children". Acta Paediatr Scand. 78 (4): 562–7. PMID 2782071.
  2. ↑ Swank M, Dias LS (1994). "Walking ability in spina bifida patients: a model for predicting future ambulatory status based on sitting balance and motor level". J Pediatr Orthop. 14 (6): 715–8. PMID 7814582.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 Kenworthy ME (July 1966). "Introducing the American Orthopsychiatric Association's president for 1966-67: Norman V. Lourie". Am J Orthopsychiatry. 36 (4): 587–9. PMID 5327787.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 Bannur BB, Purandare GM (February 1969). "Microbial production of L-lysine". Hindustan Antibiot Bull. 11 (3): 191–205. PMID 4898641.
  5. ↑ Meyer SH, Morris GF, Pretorius DH, James HE (March 1998). "Terminal myelocystocele: important differential diagnosis in the prenatal assessment of spina bifida". J Ultrasound Med. 17 (3): 193–7. PMID 9514174.
  6. ↑ Scott RM, Wolpert SM, Bartoshesky LE, Zimbler S, Karlin L (April 1988). "Segmental spinal dysgenesis". Neurosurgery. 22 (4): 739–44. PMID 3374785.
  7. ↑ Kremser E, Mitchell GM (February 1971). "Treatment of primary dysmenorrhea with a combined type oral contraceptive--a double blind study". J Am Coll Health Assoc. 19 (3): 195–6. PMID 4925436.
  8. ↑ Benedetti-Valentino F J, De Feo V, Pistolese GR, Fiorani P (September 1966). "[Nephroptosis and fibromuscular hyperplasia of the tunica media of the renal arteries]". Minerva Cardioangiol (in Italian). 14 (9): 528–34. PMID 5991006. Vancouver style error: name (help)
  9. ↑ Naccarato R, Sturniolo GC, Martin A, D'Odorico A, Montino C (March 1988). "[Irritable bowel syndrome]". G Clin Med (in Italian). 69 (3): 163–8. PMID 3169446.
  10. ↑ 10.0 10.1 Csaba G, Körösi J (1968). "A new antitumour agent: phenazathionium-mustard salt". Neoplasma. 15 (4): 443–5. PMID 5684468.
  11. ↑ Doutre MS, Beylot C, Busquet M, Barberis C, Fauchier JM, Lecastereyres D, Beylot J (April 1986). "[Familial scleroderma of the Thibierge-Weissenbach type]". Rev Rhum Mal Osteoartic (in French). 53 (4): 290–1. PMID 3738390.
  12. ↑ De Marco P, Merello E, Calevo MG, Mascelli S, Pastorino D, Crocetti L, De Biasio P, Piatelli G, Cama A, Capra V (July 2011). "Maternal periconceptional factors affect the risk of spina bifida-affected pregnancies: an Italian case-control study". Childs Nerv Syst. 27 (7): 1073–81. doi:10.1007/s00381-010-1372-y. PMID 21207040.
  13. ↑ 13.0 13.1 13.2 13.3 Kondo A, Morota N, Ihara S, Saisu T, Inoue K, Shimokawa S, Fujimaki H, Matsuo K, Shimosuka Y, Watanabe T (September 2013). "Risk factors for the occurrence of spina bifida (a case-control study) and the prevalence rate of spina bifida in Japan". Birth Defects Res. Part A Clin. Mol. Teratol. 97 (9): 610–5. doi:10.1002/bdra.23179. PMID 24078478.
  14. ↑ 14.0 14.1 Soonawala N, Overweg-Plandsoen WC, Brouwer OF (March 1999). "Early clinical signs and symptoms in occult spinal dysraphism: a retrospective case study of 47 patients". Clin Neurol Neurosurg. 101 (1): 11–4. PMID 10350196.
  15. ↑ 15.0 15.1 Horton D, Barnes P, Pendleton BD, Pollay M (January 1989). "Spina bifida occulta: early clinical and radiographic diagnosis". J Okla State Med Assoc. 82 (1): 15–9. PMID 2647936.
  16. ↑ 16.0 16.1 16.2 Kozlowski BW, Taylor ML, Baer MT, Blyler EM, Trahms C (August 1987). "Anticonvulsant medication use and circulating levels of total thyroxine, retinol binding protein, and vitamin A in children with delayed cognitive development". Am. J. Clin. Nutr. 46 (2): 360–8. doi:10.1093/ajcn/46.2.360. PMID 2441590.
  17. ↑ Racusin DA, Villarreal S, Antony KM, Harris RA, Mastrobattista J, Lee W, Shamshirsaz AA, Belfort M, Aagaard KM (December 2015). "Role of Maternal Serum Alpha-Fetoprotein and Ultrasonography in Contemporary Detection of Spina Bifida". Am J Perinatol. 32 (14): 1287–91. doi:10.1055/s-0035-1562930. PMID 26332586.
  18. ↑ 18.0 18.1 Trudell AS, Odibo AO (April 2014). "Diagnosis of spina bifida on ultrasound: always termination?". Best Pract Res Clin Obstet Gynaecol. 28 (3): 367–77. doi:10.1016/j.bpobgyn.2013.10.006. PMID 24373566.
  19. ↑ Lee W, Chaiworapongsa T, Romero R, Williams R, McNie B, Johnson A, Treadwell M, Comstock CH (June 2002). "A diagnostic approach for the evaluation of spina bifida by three-dimensional ultrasonography". J Ultrasound Med. 21 (6): 619–26. PMID 12054297.
  20. ↑ Ciovîrnache M, Simionescu L, Ioaniţiu D (1990). "[A study of palmar flexion folds by the Perera-Kolski quantitative method in acromegaly]". Endocrinologie (in French). 28 (2): 57–62. PMID 2293328.
  21. ↑ 21.0 21.1 Tilles DS, Goldenheim PD, Johnson DC, Mendelson JH, Mello NK, Hales CA (April 1986). "Marijuana smoking as cause of reduction in single-breath carbon monoxide diffusing capacity". Am. J. Med. 80 (4): 601–6. PMID 3963040.
  22. ↑ 22.0 22.1 22.2 Pitkin RM (January 2007). "Folate and neural tube defects". Am. J. Clin. Nutr. 85 (1): 285S–288S. doi:10.1093/ajcn/85.1.285S. PMID 17209211.
  23. ↑ Srb V, Kubzová E (1987). "[Comparison of the effects of 1st and 3d generation platinum cytostatics on the chromosomes of lymphocytes in human peripheral blood in vitro]". Sb Ved Pr Lek Fak Karlovy Univerzity Hradci Kralove Suppl (in Czech). 30 (4): 475–83. PMID 3504607.
  24. ↑ 24.0 24.1 Alt TH (March 1988). "Aids to scalp reduction surgery". J Dermatol Surg Oncol. 14 (3): 309–15. PMID 3279093.


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