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Anesthetic Management of Patients with Craniovertebral Junction Anomalies

  • Archana ManeEmail author
  • Yarnell Lafortune
Chapter
  • 25 Downloads

Abstract

A 2-year-old female presents for surgical correction of a cleft palate. A diagnosis of Kippel-Feil syndrome is made in this child. Associated pathology, need for a thorough preoperative assessment, strategy for intraoperative management of the airway and potential issues in the post-operative period are discussed.

References

  1. 1.
    Pledger SR. Cervical spine disease. Ortho secrets. 3rd ed. 2003.Google Scholar
  2. 2.
    Samartzis D, Kalluri P, Herman J, Lubicky JP, Shen FH. “Clinical triad” findings in pediatric Klippel-Feil patients Scoliosis. Spinal Disord. 2016;11:15.  https://doi.org/10.1186/s13013-016-0075-x. Published online 2016 Jun 27. PMCID: PMC4922059.CrossRefGoogle Scholar
  3. 3.
    Agarwal AK, Goel M, Bajpai J, Shukla S, Sachdeva N, et al. J Orthop Case Rep. 2014;4(3):53–5. PMCID: PMC4719329 PMID: 27298984.PubMedPubMedCentralGoogle Scholar
  4. 4.
    Zaki SA, Shenoy P, Shanbag P. Klippel Feil syndrome with isolated hypokinesia of the left ventricle: a rare association. Ann Pediatr Cardiol. 2010;3(1):92.  https://doi.org/10.4103/0974-2069.64354. PMCID: PMC2921529 PMID: 20814486.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Mahiroğullari M, Ozkan H, Yildirim N, Cilli F, Güdemez E. Klippel-Feil syndrome and associated congenital abnormalities: evaluation of 23 cases. Acta Orthop Traumatol Turc. 2006;40(3):234–9. PMID: 16905897.PubMedGoogle Scholar
  6. 6.
    Srivastava M, Aggarwal A, Batra P, Datana S, Kumar P, Macrcusson KA. Association of cervical vertrebra anomalies with cleft lip and palate. J Cleft Lip Palate Craniofac Anomalies. 2014;1(1):43–7.CrossRefGoogle Scholar
  7. 7.
    Klippel-Feil syndrome. Genetics home reference. June 2015. http://ghr.nlm.nih.gov/condition/klippel-feil-syndrome.
  8. 8.
    Kaplan KM, Spivak JM, Bendo JA. Embryology of the spine and associated congenital abnormalities. Spine J. 2005;5:564–76. Review ArticleCrossRefGoogle Scholar
  9. 9.
    Tassabehji M, Fang ZM, Hilton EN, McGaughran J, Zhao Z, de Bock CE, Howard E, Malass M, Donnai D, Diwan A, Manson FD, Murrell D, Clarke RA. Mutations in GDF6 are associated with vertebral segmentation defects in Klippel-Feil syndromes. Hum Mutat. 2008;29:1017–27.  https://doi.org/10.1002/humu.20741.CrossRefPubMedGoogle Scholar
  10. 10.
    Tsirikos AI, McMaster MJ. Goldenhar-associated conditions (hemifacial microsomia) and congenital deformities of the spine. Spine. 2006;31(13):E400–7.  https://doi.org/10.1097/01.brs.0000219876.45640.2a.CrossRefPubMedGoogle Scholar
  11. 11.
    Giampietro PF, Raggio CL, Blank RD, McCarty C, Broeckel U, Pickart MA. Clinical, genetic and environmental factors associated with congenital vertebral malformations. Mol Syndromol. 2013;4(1–2):94–105.  https://doi.org/10.1159/000345329. Published online 2012 Nov 15. PMCID: PMC3638777 PMID: 23653580.CrossRefPubMedGoogle Scholar
  12. 12.
    Samartzis DD, Herman J, Lubicky JP, Shen FH. Classification of congenitally fused cervical patterns in Klippel-Feil patients: epidemiology and role in the development of cervical spine-related symptoms. Spine. 2006;31(21):E798–804. [PubMed].CrossRefGoogle Scholar
  13. 13.
    Menger RP, Notaranni C. Klippel Feil syndrome. STATPEARLS( Internet). Last Update: October 27, 2018.Google Scholar
  14. 14.
    Wybier M, Bossard P. Musculoskeletal imaging in progress: the EOS imaging system. Joint Bone Spine. 2013;80(3):238–43.  https://doi.org/10.1016/j.jbspin.2012.09.018. Epub 2012 Nov 22. PMID: 23177915.CrossRefPubMedGoogle Scholar
  15. 15.
    Masuda H, Arikawa K, Yuda T, Taira A. Total anomalous pulmonary venous connection associated with Klippel-Feil syndrome: a case report. [Article in Japanese]. Kyobu Geka. 1991;44(5):417–20.PubMedGoogle Scholar
  16. 16.
    McGaughran JM, Kuna P, Das V. Audiological abnormalities in the Klippel-Feil syndrome. Arch Dis Child. 1998;79:352–5.CrossRefGoogle Scholar
  17. 17.
    Vaidyanathan S, Hughes PL, Soni BM, Singh G, Sett P. Klippel-Feil syndrome—the risk of cervical spinal cord injury: a case report. BMC Fam Pract. 2002;3:6.  https://doi.org/10.1186/1471-2296-3-6. © Published: 11 April 2002.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
  19. 19.
    Adewale L. Review article: anatomy and assessment of the pediatric airway. Pediatr Anesth. 2009;19(Suppl.1):1–8. Robert W M Walker Management of the difficult airway in children J R Soc Med. 2001 Jul; 94(7): 341–344. PMCID: PMC128159821.CrossRefGoogle Scholar
  20. 20.
    Walker RW, Ellwood J. The management of difficult intubation in children. Pediatr Anesth. 2009;19(Suppl 1):77–87.  https://doi.org/10.1111/j.1460-9592.2009.03014.x.CrossRefGoogle Scholar
  21. 21.
    Chura M, Odo N, Foley E, Bora V. Cervical deformity and potential difficult airway management in Klippel–Feil syndrome. Anesthesiology. 2018;V128(5):1007.CrossRefGoogle Scholar
  22. 22.
    Ramkumar V. Preparation of the patient and the airway for awake intubation. Indian J Anaesth. 2011;55(5):442–7.  https://doi.org/10.4103/0019-5049.89863. PMCID: PMC3237141 PMID: 22174458.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    The use of dexmedetomidine and Airwayscope in airway ...—NCBI https://www.ncbi.nlm.nih.gov/pubmed/25098144 by A Furuya—2014 fetanyl. Dexmedetomidine infusion was also useful to maintain adequate spontaneous breathing and to achieve awaking before extubation.
  24. 24.
    Green M, Chatterjee D, Meyers M. High dose dexmedetomidine and ketamine for managing difficult pediatric airways. J Anesth Clin Res. 2016;7:652.  https://doi.org/10.4172/2155-6148.1000652.CrossRefGoogle Scholar
  25. 25.
    Walker RWM. Consultant Paediatric Anaesthetist, Royal Manchester Children’s Hospital, Pendlebury, Manchester M27 4HA.Google Scholar
  26. 26.
    Walker RWM. Management of the difficult airway in children. FRCA J R Soc Med. 2001;94(7):341–4.CrossRefGoogle Scholar
  27. 27.
    Ramesh S, Jayanthi R. Supraglottic airway devices in children. Indian J Anaesth. 2011;55(5):476–82.CrossRefGoogle Scholar
  28. 28.
    Raj D, Luginbuehl I. Managing the difficult airway in the syndromic child. Contin Educ Anaesth Crit Care Pain. 2015;15(1):7–13.CrossRefGoogle Scholar
  29. 29.
    Desalu I, Adeyemo WL, Akintimoye MO, Adepoju AA. Airway and respiratory complications in children undergoing cleft lip and palate repair. Ghana Med J. 2010;44(1):16–20. PMCID: PMC2956308 PMID: 21326986.PubMedPubMedCentralGoogle Scholar
  30. 30.
    Tracy MR, Dormans JP, Kusumi K. Klippel-Feil syndrome: clinical features and current understanding of etiology. Clin Orthop Relat Res. 2004;424:183–90.CrossRefGoogle Scholar
  31. 31.
    Mascarenhas O. Anaesthesia management in craniovertebral junction anomalies. J Craniovertebr Junct Spine. 2016;7(4):193–6.CrossRefGoogle Scholar
  32. 32.
  33. 33.
    Jain VK, Behari S. Management of congenital atlanto -axial dislocation: some lessons learnt. Neurol India. 2002;50:386–97.PubMedGoogle Scholar
  34. 34.
    Epstein NE. Surg Neurol Int. 2013;4(Suppl 5):S383–91. Published online 2013 Oct 29.  https://doi.org/10.4103/2152-7806.120782.CrossRefGoogle Scholar
  35. 35.
    Sala F, Meneghelli P. Intraoperative neurophysiological monitoring for craniovertebral junction surgery. Acta Neurochir Suppl. 2019;125:369–80.  https://doi.org/10.1007/978-3-319-62515-7_53.CrossRefPubMedGoogle Scholar
  36. 36.
    Stecker MM. A review of intraoperative monitoring for spinal surgery. Surg Neurol Int. 2012;3(Suppl 3):S174–87. Published online 2012 Jul 17.  https://doi.org/10.4103/2152-7806.98579. PMCID: PMC3422092 PMID: 22905324.CrossRefGoogle Scholar
  37. 37.
    Hill GE, Frawley WH, Griffith KE, Forestner JE, Minei JP. Allogeneic blood transfusion increases the risk of post-operative bacterial infection: a meta-analysis. J Trauma. 2003;54:908–14. [PubMed].CrossRefGoogle Scholar
  38. 38.
    Mascarenhas O. Anaesthesia management in craniovertebral junctional anomalies. J Craniovertebr Junct Spine. 2016;7(4):193–6.  https://doi.org/10.4103/0974-8237.193256.CrossRefGoogle Scholar
  39. 39.
    Gauvin F, Robillard P, Hume H, Grenier D, Whyte RK, Webert KE, Fergusson D, Lau W, Froese N, Delage G. Transfusion-related acute lung injury in the Canadian paediatric population. 2012;17(5):235–9. PMCID: PMC3381913 PMID: 23633895.Google Scholar
  40. 40.
    Rosomoff HL. Occult respiratory and autonomic dysfunction in craniovertebral anomalies and upper cervical spinal disease. Spine (Phila Pa 1976). 1986;11(4):345–7.CrossRefGoogle Scholar
  41. 41.
    Hase Y, Kamekura N, Fujisawa T, Fukushima K. Repeated anesthetic management for a patient with Klippel-Feil syndrome. Anesth Prog. 2014;61(3):103–6.CrossRefGoogle Scholar
  42. 42.
    Ozmete O, Bali C, Cok OY, Turk HE, Ozyilkan NB, Civi S, Aribogan A. Sugammadex given for rocuronium-induced neuromuscular blockade in infants: a retrospective study. J Clin Anesth. 2016;35:497–501.CrossRefGoogle Scholar
  43. 43.
    Liu G, Wang R, Yan Y, Fan L, Xue J, Wang T. The and safety of sugammadex for reversing postoperative residual neuromuscular blockade in pediatric patients: a systematic review. Sci Rep. 2017;7(1):5724.  https://doi.org/10.1038/s41598-017-06159-2.CrossRefPubMedPubMedCentralGoogle Scholar
  44. 44.
    Newth CJ, Venkataraman S, Willson DF, Meert KL, Harrison R, Dean JM, Pollack M, Zimmerman J, Anand KJ, Carcillo JA, Nicholson CE, Eunice Shriver Kennedy National Institute of Child Health and Human Development Collaborative Pediatric Critical Care Research Network. Weaning and extubation readiness in pediatric patients. Pediatr Crit Care Med. 2009;10(1):1–11.  https://doi.org/10.1097/PCC.0b013e318193724d.CrossRefPubMedPubMedCentralGoogle Scholar
  45. 45.
    Laham JL, Breheny PJ, Rush A. Do clinical parameters predict first planned extubation outcome in the pediatric intensive care unit? J Intensive Care Med. 2015;30(2):89–96.  https://doi.org/10.1177/0885066613494338. Epub 2013 Jun 27.CrossRefPubMedGoogle Scholar
  46. 46.
    Cavallone LF, Vannucci A. Review article: extubation of the difficult airway and extubation failure. Anesth Analg. 2013;116(2):368–83.  https://doi.org/10.1213/ANE.0b013e31827ab572. Epub 2013 Jan PMID: 23302983.CrossRefPubMedGoogle Scholar
  47. 47.
    Buck ML. Dexmedetomidine use in pediatric intensive care and procedural sedation. J Pediatr Pharmacol Ther. 2010;15(1):17–29. PMCID: PMC3017406 PMID.PubMedPubMedCentralGoogle Scholar
  48. 48.
    Faberowski L, Nargozian C. Utility of airway exchange catheters in pediatric patients with a known difficult airway. Pediatr Crit Care Med. 2005;6(4):454–6.CrossRefGoogle Scholar
  49. 49.
    Dokai T, Nagashima H, Nanjo Y, Tanida A, Teshima R. Posterior occipitocervical fixation under skull-femoral traction for the treatment of basilar impression in a child with Klippel-Feil syndrome. J Bone Joint Surg Br. 2011;93(11):1571–4.CrossRefGoogle Scholar
  50. 50.
    Menezes A, Lindley T. Craniovertebral abnormalities and their neurosurgical management. Last modified 13 Mar 2015, Chapter 181.Google Scholar
  51. 51.
    Craniocervical junction abnormalities, MERCK manual. By Michael Rubin, MDCM, Photo courtesy of Depuy/Synthes. Ref -Clinical Gate, Filed under Neurosurgery.
  52. 52.
    Morota N. Pediatric craniovertebral junction surgery. Neurol Med Chir (Tokyo). 2017;57(9):435–60 . Published online 2017 Aug 1. PMCID: PMC5638788 PMID: 28768919.  https://doi.org/10.2176/nmc.ra.2017-0032.CrossRefGoogle Scholar
  53. 53.
    Dickman CA, Spetzler RF, Sonntag VKH. In: Bambakidis N, Dickman C, Spetzler R, et al., editors. Surgery of the craniovertebral junction. 2nd ed. New York: Thieme; 2012.  https://doi.org/10.1055/b-002-85485.CrossRefGoogle Scholar
  54. 54.
    Dlouhy BJ, Dawson JD, Menezes AH. Intradural pathology and pathophysiology associated with Chiari I malformation in children and adults with and without syringomyelia. J Neurosurg Pediatr. 2017;20(6):526–41.CrossRefGoogle Scholar
  55. 55.
  56. 56.
    Hidalgo JA, Varacallo M. Arnold Chiari malformation. Treasure Island, FL: StatPearls Publishing; 2018.Google Scholar
  57. 57.
    Lin W, Duan G, Xie J, Shao J, Wang Z, Jiao B. Comparison of results between posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation type I: a systematic review and meta-analysis. World Neurosurg. 2018;110:460–474.e5.CrossRefGoogle Scholar
  58. 58.
    Giammattei L, Borsotti F, Parker F, Messerer M. Chiari I malformation: surgical technique, indications and limits. Acta Neurochir. 2018;160(1):213–7.CrossRefGoogle Scholar
  59. 59.
    Smith’s anesthesia for children and infants. 8th ed. Chapter 22. Pages 734.Google Scholar
  60. 60.
    Mundakel GT, et al. Down syndrome. Medscape. https://emedicine.medscape.com/article/943216-overview. Accessed 8 Jan 2019.
  61. 61.
    Rogers PT, Roizen NJ, Capone GT. Down syndrome. In: Capute AJ, Accardo PJ, editors. Developmental disabilities in infancy and childhood. 2nd ed; 1996. p. 221–4.Google Scholar
  62. 62.
    Pueschel SM, Scola FH. Atlantoaxial instability in individuals with Down syndrome: epidemiologic, radiographic, and clinical studies. Pediatrics. 1987;80:555–60.PubMedGoogle Scholar
  63. 63.
    Gregory GA, Andropoulos DB. Gregory’s pediatric anesthesia. 5th ed. Hoboken, NJ: Wiley-Blackwell.Google Scholar
  64. 64.
    Craniovertebral junction anomalies. https://www.amboss.com/us/knowledge/Craniovertebral_junction_anomalies. Accessed 10 Jan 2019.

Copyright information

© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.Department of AnesthesiologyAlbany Medical CenterAlbanyUSA
  2. 2.University of Maryland Upper Chesapeake HealthBel AirUSA
  3. 3.Albany Medical CenterAlbanyUSA

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