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Spinal Muscular Atrophy

  • Adam Sachs
Chapter

Abstract

Spinal muscular atrophy (SMA) is an autosomal recessive (principal form), progressive, neuromuscular disease, caused by a defect in the survival motor neuron 1 gene. The physiologic changes of pregnancy are poorly tolerated in SMA patients who frequently experience increasing muscle weakness and progressive respiratory insufficiency throughout pregnancy. Because of respiratory insufficiency, challenging airway anatomy, and spinal deformities, formulating an adequate anesthetic plan is difficult. Although there are typically no contraindications to placing an epidural for labor analgesia, patients with SMA have an increased incidence of difficult epidural placement and inadequate blockade. For cesarean delivery, neuraxial anesthesia is typically preferred although this will ultimately depend on the degree of respiratory insufficiency. Due to various degrees of severity, no single anesthetic plan will be best for all pregnant women with SMA, and a tailored approach should be developed, which optimizes the opportunity for a successful delivery and minimizes risk.

Keywords

Spinal muscular atrophy SMA Muscular Motor Spinal Epidural Cesarean Pregnant Pregnancy Anesthesia 

References

  1. 1.
    Talbot K, Davies KE. Spinal muscular atrophy. Semin Neurol. 2001;21(02):189–98.CrossRefGoogle Scholar
  2. 2.
    Islander G. Anesthesia and spinal muscle atrophy. Paediatr Anaesth. 2013;23(9):804–16.CrossRefGoogle Scholar
  3. 3.
    Bodamer OA. Spinal muscular atrophy [Internet]. UpToDate. 2016 [cited 2 November 2016]. https://www.uptodate.com/contents/spinal-muscular-atrophy.
  4. 4.
    Rudnik-Schöneborn S, Zerres K, Ignatius J, Rietschel M. Pregnancy and spinal muscular atrophy. J Neurol. 1992;239(1):26–30.CrossRefGoogle Scholar
  5. 5.
    Wilson RD, Williams KP. Spinal muscular atrophy and pregnancy. Br J Obstet Gynaecol. 1992;99(6):516–7.CrossRefGoogle Scholar
  6. 6.
    Prior TW, Russman BS. Spinal muscular atrophy. In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Fong CT, Mefford HC, Smith RJH, Stephens K, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 2000. 1993–2016. [updated 2013 Nov 14]. www.ncbi.nih.gov/books/NBK 1352/.Google Scholar
  7. 7.
    Dahl B, Nørregaard FO, Juhl B. Pregnancy and delivery in a woman with neuromuscular disease. Spinal muscular atrophy and severely reduced pulmonary function. Ugeskr Laeger. 1995;157(6):750–1.PubMedGoogle Scholar
  8. 8.
    Pugh CP, Healey SK, Crane JM, Young D. Successful pregnancy and spinal muscular atrophy. Obstet Gynecol. 2000;95(6, Part 2):1034.CrossRefGoogle Scholar
  9. 9.
    Rudnik-Schöneborn S, Breuer C, Zerres K. Stable motor and lung function throughout pregnancy in a patient with infantile spinal muscular atrophy type II. Neuromuscul Disord. 2002;12(2):137–40.CrossRefGoogle Scholar
  10. 10.
    Yim R, Kirschner K, Murphy E, Parson J, Winslow C. Successful pregnancy in a patient with spinal muscular atrophy and severe kyphoscoliosis. Am J Phys Med Rehabil. 2003;82(3):222–5.PubMedGoogle Scholar
  11. 11.
    Sangiuolo F, Filareto A, Giardina E, Nardone AM, Pilu G, Pietropolli A, et al. Prenatal diagnosis of spinal muscular atrophy with respiratory distress (SMARD1) in a twin pregnancy. Prenat Diagn. 2004;24(10):839–41.CrossRefGoogle Scholar
  12. 12.
    Flunt D, Andreadis N, Menadue C, Welsh AW. Clinical commentary: obstetric and respiratory management of pregnancy with severe spinal muscular atrophy. Obstet Gynecol Int. 2009;19(1):1–5.CrossRefGoogle Scholar
  13. 13.
    Howarth L, Glanville T. Management of a pregnancy complicated by type III spinal muscular atrophy. Case Rep. 2011;18:1–3.Google Scholar
  14. 14.
    Habib AS, Helsley SE, Millar S, Deballi P III, Muir HA. Anesthesia for cesarean section in a patient with spinal muscular atrophy. J Clin Anesth. 2004;16(3):217–9.CrossRefGoogle Scholar
  15. 15.
    Perry JJ, Lee JS, Sillberg VA, Wells GA. Rocuronium versus succinylcholine for rapid sequence induction intubation. Cochrane Database Syst Rev. 2008;2:CD002788.Google Scholar
  16. 16.
    Sørensen MK, Bretlau C, Gätke MR, Sørensen AM, Rasmussen LS. Rapid sequence induction and intubation with rocuronium-sugammadex compared with succinylcholine: a randomized trial. Br J Anaesth. 2012;108(4):682–9.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Adam Sachs
    • 1
  1. 1.Department of AnesthesiologyUniversity of Connecticut Medical CentreFarmingtonUSA

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