European Spine Journal

, Volume 23, Supplement 1, pp 72–75 | Cite as

Surgical correction of scoliosis in Rett syndrome: cord monitoring and complications

  • T. HammettEmail author
  • A. Harris
  • B. Boreham
  • S. M. H. Mehdian
Original Article



Rett syndrome is a progressive neurodevelopmental disorder that predominantly affects females and is associated with a high incidence of scoliosis and epilepsy. There is scant published work about intraoperative spinal cord monitoring in these patients and little more regarding the rate of perioperative complications. We investigated our institutions’ experience with both.


We retrospectively reviewed the records of 11 patients with Rett syndrome who underwent surgical correction of scoliosis at our institution between 2004 and 2010.


Eleven patients underwent successful correction of their scoliosis at an average age of 12. Eight of the patients suffered one or more significant complications. The average curve was corrected from 71° to 27°. Successful spinal cord monitoring was achieved in eight of the nine patients where it was attempted. No patient suffered any neurological complications. Average inpatient stay was 18.2 days.


Scoliosis surgery in patients with Rett syndrome carries a very high rate of complications and an average hospital stay approaching 3 weeks. Both caregivers and surgeons should be aware of this when planning any intervention. These patients frequently have useful lower limb function and spinal cord monitoring is a valid tool to aid in its preservation. We would suggest aggressive optimisation of these patients prior to surgery, with an emphasis on nutrition.


Scoliosis Rett Monitoring 


Conflict of interest



  1. 1.
    Rett A (1966) On a unusual brain atrophy syndrome in hyperammonemia in childhood. Wien Med Wochenschr 116(37):723–726PubMedGoogle Scholar
  2. 2.
    Hagberg B, Aicardi J, Dias K, Ramos O (1983) A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett’s syndrome: report of 35 cases. Ann Neurol 14(4):471–479PubMedCrossRefGoogle Scholar
  3. 3.
    Fehr S, Bebbington A, Nassar N, Downs J, Ronen GM, DE Klerk N, Leonard H (2011) Trends in the diagnosis of Rett syndrome in Australia. Pediatr Res 70(3):313–319PubMedCentralPubMedCrossRefGoogle Scholar
  4. 4.
    Amir RE, Van den Veyver IB, Wan M, Tran CQ, Francke U, Zoghbi HY (1999) Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nat Genet 23(2):185–188PubMedCrossRefGoogle Scholar
  5. 5.
    Samaco RC, Neul JL (2011) Complexities of Rett syndrome and MeCP2. J Neurosci 31(22):7951–7959PubMedCentralPubMedCrossRefGoogle Scholar
  6. 6.
    Kirby RS, Lane JB, Childers J, Skinner SA, Annese F, Barrish JO, Glaze DG, MacLeod P, Percy AK (2010) Longevity in Rett syndrome: analysis of the North American Database. J Pediatr 156:135–138PubMedCentralPubMedCrossRefGoogle Scholar
  7. 7.
    Harrison DJ, Webb PJ (1990) Scoliosis in the Rett syndrome: natural history and treatment. Brain Dev 12(1):154–156PubMedCrossRefGoogle Scholar
  8. 8.
    Downs J, Bergman A, Carter P, Anderson A, Palmer GM, Roye D, van Bosse H, Bebbington A, Larsson EL, Smith BG, Baikie G, Fyfe S, Leonard H (2009) Guidelines for management of scoliosis in Rett syndrome patients based on expert consensus and clinical evidence. Spine (Phila Pa 1976) 34(17):E607–E617CrossRefGoogle Scholar
  9. 9.
    Downs J, Young D, de Klerk N, Bebbington A, Baikie G, Leonard H (2009) Impact of scoliosis surgery on activities of daily living in females with Rett syndrome. J Pediatr Orthop 29(4):369–374PubMedCrossRefGoogle Scholar
  10. 10.
    Gabos PG, Inan M, Thacker M, Borkhu B (2012) Spinal fusion for scoliosis in Rett syndrome with an emphasis on early postoperative complications. Spine (Phila Pa 1976) 37(2):E90–E94CrossRefGoogle Scholar
  11. 11.
    Oddy WH, Webb KG, Baikie G, Thompson SM, Reilly S, Fyfe SD, Young D, Anderson AM, Leonard H (2007) Feeding experiences and growth status in a Rett syndrome population. J Pediatr Gastroenterol Nutr 45(5):582–590PubMedCrossRefGoogle Scholar
  12. 12.
    Motil KJ, Morrissey M, Caeg E, Barrish JO, Glaze DG (2009) Gastrostomy placement improves height and weight gain in girls with Rett syndrome. J Pediatr Gastroenterol Nutr 49(2):237–242PubMedCrossRefGoogle Scholar
  13. 13.
    Master DL, Thompson GH, Poe-Kochert C, Biro C (2008) Spinal cord monitoring for scoliosis surgery in Rett syndrome: can these patients be accurately monitored? J Pediatr Orthop 28(3):342–346PubMedCrossRefGoogle Scholar
  14. 14.
    Hammett TC, Boreham B, Quraishi NA, Mehdian SM (2013) Intraoperative spinal cord monitoring during the surgical correction of scoliosis due to cerebral palsy and other neuromuscular disorders. Eur Spine J 22 Suppl 1:S38–S41PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • T. Hammett
    • 1
    Email author
  • A. Harris
    • 2
  • B. Boreham
    • 1
  • S. M. H. Mehdian
    • 1
  1. 1.Centre for Spinal Studies and SurgeryQueen’s Medical CentreNottinghamUK
  2. 2.Department of Paediatric SurgeryBirmingham Childrens’ HospitalBirminghamUK

Personalised recommendations