Skip to main content

Advertisement

SpringerLink
Log in

What’s New in Craniosynostosis?

  • Pediatric Anesthesia (J Lerman, Section Editor)
  • Published:
Current Anesthesiology Reports Aims and scope Submit manuscript

Abstract

Craniosynostosis is a syndrome of premature skull ossification. Children with craniosynostosis exhibit an abnormal skull shape and are often surgically corrected within the first year of life to minimize the long-term sequelae of the condition. In this article, we describe the epidemiology and pathophysiology of craniosynostosis, surgical corrective approaches, and anesthetic management including preoperative evaluation, intraoperative management, and postoperative treatment. Lastly, we explore recent advances in blood conservation strategies for these surgeries.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Stricker PA, Fiadjoe JE. Anesthesia for craniofacial surgery in infancy. Anesthesiol Clin. 2014;32:215–35.

    PubMed  Google Scholar 

  2. Pattisapu VJ, Gegg CA, Olavarria G, et al. Craniosynostosis: diagnosis and surgical management. Atlas Oral Maxillofac Surg Clin North Am. 2010;18:77–91.

    PubMed  Google Scholar 

  3. French LR, Jackson IT, Melton LJ III. A population-based study of craniosynostosis. J Clin Epidemiol. 1990;43:69–73.

    PubMed  CAS  Google Scholar 

  4. Zeiger JS, Beaty TH, Hetmanski JB, et al. Genetic and environmental risk factors for sagittal craniosynostosis. J Craniofac Surg. 2002;13:602–6.

    PubMed  Google Scholar 

  5. Rasmussen SA, Yazdy MM, Carmichael SL, et al. Maternal thyroid disease as a risk factor for craniosynostosis. Obstet Gynecol. 2007;110:369–77.

    PubMed  Google Scholar 

  6. Cohen MM Jr. Etiopathogenesis of craniosynostosis. Neurosurg Clin N Am. 1991;2:507.

    PubMed  Google Scholar 

  7. Hackshaw A, Rodeck C, Boniface S. Maternal smoking in pregnancy and birth defects: a systematic review based on 173,687 malformed cases and 11.7 million controls. Hum Repod Update. 2011;17:589–604.

    CAS  Google Scholar 

  8. Schweitzer T, Böhm H, Meyer-Marcotty P. Avoiding CT scans in children with single-suture craniosynostosis. Childs Nerv Syst. 2012;28:1077–82.

    PubMed  CAS  Google Scholar 

  9. Caldarelli Tamburrino G, Massimi L, et al. Complex craniosynostoses: a review of the prominent clinical features and the related management strategies. Childs Nerv Syst. 2012;28:1511–23.

    PubMed  Google Scholar 

  10. Lekovic GP, Bristol RE, Rekate HL. Cognitive impact of craniosynostosis. Semin Pediatr Neurol. 2004;11:305–10.

    PubMed  Google Scholar 

  11. Speltz ML, Kapp-Simon KA, Cunningham M, et al. Single-suture craniosynostosis: a review of neurobehavioral research and theory. J Pediatr Psych. 2004;29:651–68.

    Google Scholar 

  12. Kapp-Simon KA, Speltz ML, Cunningham ML, et al. Neurodevelopment of children with single suture craniosynostosis: a review. Childs Nerv System. 2007;22:269–81.

    Google Scholar 

  13. Driessen C, Joosten KF, Bannink N, et al. How does obstructive sleep apnoea evolve in syndromic craniosynostosis? A prospective cohort study. Arch Dis Child. 2013;98:538–43.

    PubMed  Google Scholar 

  14. Thomas K, Hughes C, Johnson D. Anesthesia for surgery related to craniosynostosis: a review. Part 1. Pediatr Anesth. 2012;22:1033–41.

    Google Scholar 

  15. Boop FA, Shewmake K, Chadduck WM. Synostectomy vs. complex cranioplasty for the treatment of sagittal synostosis. Childs Nerv Syst. 1996;12:371–5.

    PubMed  CAS  Google Scholar 

  16. Maugans TA, McComb JG, Levy ML. Surgical management of sagittal synostosis: a comparative analysis of strip craniectomy and calvarial vault remodeling. Pediatr Neurosurg. 1997;27:137–48.

    PubMed  CAS  Google Scholar 

  17. Barone CM, Jimenez DF. Endoscopy craniectomy for early correction of craniosynostosis. Plast Reconstr Surg. 1997;104:1965–73.

    Google Scholar 

  18. Proctor MR. Endoscopic cranial suture release for the treatment of craniosynostosis—is it the future? J Craniofac Surg. 2012;23:225–8.

    PubMed  Google Scholar 

  19. Meier PM, Goobie SM, DiNardo JA, et al. Endoscopic strip craniectomy in early infancy: the initial five years of anesthesia experience. Anesth Analg. 2011;112:407–14.

    PubMed  Google Scholar 

  20. Mackenzie KA, Davis C, Yang A, MacFarlane MR. Evolution of surgery for sagittal synostosis: the role of new technologies. J Craniofac Surg. 2009;20:129–33.

    PubMed  Google Scholar 

  21. Ririe DG, David LR, Glazier SS, et al. Surgical advancement influences perioperative care: a comparison of two surgical technique for sagittal craniosynostosis repair. Anesth Analg. 2003;97:699–703.

    PubMed  Google Scholar 

  22. David LR, Sanger C, Thompson J, et al. Analysis of a 6 year clinical experience with spring mediated distraction. Craniofac Surg. 2007;12:391–4.

    Google Scholar 

  23. Taylor JA, Maugans TA. Comparing of spring-mediated cranioplasty to minimally invasive strip craniectomy and barrel staving for early treatment of sagittal craniosynostosis. J Craniofac Surg. 2011;22:1225–9.

    PubMed  Google Scholar 

  24. David LR, Plikaitis CM, Couture D, et al. Outcome analysis of our first 75 spring-assisted surgeries for scaphocephaly. J Craniofac Surg. 2010;21:3–9.

    PubMed  Google Scholar 

  25. Maugans TA, McComb JG, Levy ML. Surgical management of sagittal synostosis: a comparative analysis of strip craniectomy and calvarial vault remodeling. Pediatr Neurosurg. 1997;27:137–48.

    PubMed  CAS  Google Scholar 

  26. Fearon JA, Yu J, Bartlett SP, et al. Infections in craniofacial surgery: a combined report of 567 procedures from two centers. Plast Reconstr Surg. 1997;100:862–8.

    PubMed  CAS  Google Scholar 

  27. Faberowski LW, Black S, Mickle JP. Incidence of venous air embolism during craniectomy for craniosynostoses repair. Anesthesiology. 2000;92:20–3.

    PubMed  CAS  Google Scholar 

  28. Tobias JD, Johnson JO, Jimenez DF, et al. Venous air embolism during endoscopic strip craniectomy for repair of craniosynostoses in infants. Anesthesiology. 2001;95:340–2.

    PubMed  CAS  Google Scholar 

  29. White N, Marcus R, Dover S, et al. Predictors of blood loss in fronto-orbital advancement and remodeling. J Craniofac Surg. 2009;20:378–81.

    PubMed  Google Scholar 

  30. Stricker PA, Shaw TL, Desouza DG, et al. Blood loss, replacement, and associated morbidity in infants and children undergoing craniofacial surgery. Pediatr Anesth. 2010;20:150–9.

    Google Scholar 

  31. Stricker PA, Lin EE, Fiadjoe JE, et al. Absence of tachycardia during hypotension in children undergoing craniofacial reconstruction surgery. Anesth Analg. 2012;115:139–46.

    PubMed  Google Scholar 

  32. Stricker PA, Lin EE, Fiadjoe JE, et al. Evaluation of central venous pressure monitoring in children undergoing craniofacial reconstruction surgery. Anesth Analg. 2013;116:411–9.

    PubMed  Google Scholar 

  33. de Souza Pereira, Neto E, Grousson S, Duflo F, et al. Predicting fluid responsiveness in mechanically ventilated children under general anaesthesia using dynamic parameters and transthoracic echocardiography. Br J Anaesth. 2011;106:856–64.

    Google Scholar 

  34. Choi AS, Ahmad NS, Beer DA. Metabolic changes during major craniofacial surgery. Pediatr Anesth. 2010;20:851–5.

    Google Scholar 

  35. Society of Pediatric Anesthesia. Hyperkalemia statement. 2011. www.wakeupsafe.org/Hyperkalemia_statement.pdf. Accessed 16 Aug 2011.

  36. Josking J, Dowling K, Costi D. Intraoperative and postoperative hyponatremia with craniosynostosis surgery. Pediatr Anesth. 2012;22:654–60.

    Google Scholar 

  37. Bhananker SM, Ramamoorth C, Geiduschek JM, et al. Anesthesia-related cardiac arrest in children: update from the Pediatric Perioperative Cardiac Arrest Registry. Anesth Analg. 2007;105:344–50.

    PubMed  Google Scholar 

  38. Faberowski LW, Black S, Mickle JP. Blood loss and transfusion practice in the perioperative management of craniosynostosis repair. J Neurosurg Anesthesiol. 1999;11:167–72.

    PubMed  CAS  Google Scholar 

  39. Lacroix J, Hebert PC, Hutchinson JS, et al. Transfusion strategies for patients in pediatric intensive care units. N Engl J Med. 2007;356:1609–19.

    PubMed  CAS  Google Scholar 

  40. Vega RA, Lyon C, Kierce JF, et al. Minimizing transfusion requirements for children undergoing craniosynostosis repair: the CHoR protocol. J Neurosurg Pediatrics. 2014;14:190–5.

    Google Scholar 

  41. • Haas T, Goobie S, Spielmann N, et al. Improvements in patient blood management for pediatric craniosynostosis surgery using a ROTEM®-assisted strategy—feasibility and costs. Pediatr Anesth 2014; 24: 774–80. A study looking at the use of intraoperative thromboelastography to guide blood transfusion, which resulted in a reduction in fresh frozen plasma and platelet transfusions.

    PubMed  Google Scholar 

  42. • Lavoie J. Blood transfusion risks and alternative strategies in pediatric patients. Paediatr Anaesth 2011; 21: 14–24. A comprehensive review article on the risk of blood transfusions and blood conservation strategies in pediatric patients.

  43. Weldon BC. Blood conservation in pediatric anesthesia. Anesthesiol Clin North America. 2005;23:347–61.

    PubMed  Google Scholar 

  44. Hans P, Collin V, Bonhomme V, et al. Evaluation of acute normovolemic hemodilution for surgical repair of craniosynostosis. J Neurosurg Anesth. 2000;12:33–6.

    CAS  Google Scholar 

  45. Jimenez DF, Barone CM. Intraoperative autologous blood transfusion in the surgical correction of craniosynostosis. Neurosurgery. 1995;37:1075–9.

    PubMed  CAS  Google Scholar 

  46. Sugai Y, Sugai K, Fuse KA. Current status of bacterial contamination of autologous blood for transfusion. Transfus Apher Sci. 2001;24:255–9.

    PubMed  CAS  Google Scholar 

  47. Fearon JA, Cook TK, Herbert M. Effects of hypotensive anesthesia on blood transfusion rates in craniosynostosis corrections. Plast Reconstr Surg. 2014;133:1133–6.

    PubMed  CAS  Google Scholar 

  48. Helfaer MA, Carson BS, James CS, et al. Increased hematocrit and decreased transfusion requirements in children given erythropoietin before undergoing surgery. J Neurosurg. 1998;88:704–8.

    PubMed  CAS  Google Scholar 

  49. Fearon JA, Weinthal J. The use of recombinant erythropoietin in the reduction of blood transfusion rates in craniosynostosis repair in infants and children. Plast Reconstr Surg. 2002;109:2190–6.

    PubMed  Google Scholar 

  50. Meneghini L, Zadra N, Aneloni V, et al. Erythropoietin therapy and acute preoperative normovolemic haemodilution in infants undergoing craniosynostosis surgery. Paediatr Anesth. 2003;13:392–6.

    Google Scholar 

  51. Stricker PA, Cladis FP, Fiadjoe JE, et al. Perioperative management of children undergoing craniofacial reconstruction surgery: a practice survey. Paediatr Anaesth. 2011;21:1026–35.

    PubMed  Google Scholar 

  52. Song G, Yang P, Zhu S, et al. Tranexamic acid reducing blood transfusion in children undergoing craniosynostosis surgery. J Craniofac Surg. 2013;24:299–303.

    PubMed  Google Scholar 

  53. • Dadure C, Sauter M, Bringuier S, et al. Intraoperative tranexamic acid reduces blood transfusion in children undergoing craniosynostosis surgery: A randomized double-blind study. Anesthesiology 2011; 114: 856–61. One of two studies demonstrating the efficacy of tranexamic acid in pediatric craniosynostosis surgery.

  54. • Goobie SM, Meier PM, Pereira LM, et al. Efficacy of tranexamic acid in pediatric craniosynostosis surgery: A double-blind, placebo-controlled trial. Anesthesiology 2011; 114: 862–71. One of two studies demonstrating the efficacy of tranexamic acid in pediatric craniosynostosis surgery.

    PubMed  CAS  Google Scholar 

  55. Martin K, Breuer T, Gertler R, et al. Tranexamic acid vs. ε-aminocaproic acid: efficacy and safety in paediatric cardiac surgery. Eur J Cardiothorac Surg. 2011;39:892–7.

    PubMed  Google Scholar 

  56. Goobie SM, Meier PM, Sethna NF, et al. Population pharmacokinetics of tranexamic acid in paediatric patients undergoing craniosynostosis surgery. Clin Pharmacokinet. 2013;52:267–76.

    PubMed  CAS  Google Scholar 

  57. Oppenheimer AJ, Ranganathan K, Levi B, et al. Minimizing transfusions in primary cranial vault remodeling: the role of aminocaproic acid. J Craniofac Surg. 2014;25:82–6.

    PubMed  Google Scholar 

  58. Pardey Bracho GF, de Souza Pereira, Neto E, Grousson S, et al. Opioid consumption after levobupivacaine scalp nerve block for craniosynostosis surgery. Acta Anaesthesiol Taiwan. 2014;52:64–9.

    PubMed  Google Scholar 

  59. Chiaretti A, Pietrini D, Piastra M, et al. Safety and efficacy of remifentanil in craniosynostosis repair in children less than 1 year old. Pediatr Neurosurg. 2000;33:83–8.

    PubMed  CAS  Google Scholar 

  60. Czerwinski M, Hopper RA, Gruss J, Fearon JA. Major morbidity and mortality rates in craniofacial surgery: an analysis of 8101 major procedures. Plast Reconstr Surg. 2010;126:181–6.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Anesthesia and Perioperative Care, University of California, San Francisco, 513 Parnassus Ave, Room S-436, San Francisco, CA, 94143, USA

    Tina T. Dong & Marla Ferschl

Authors
  1. Tina T. Dong
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marla Ferschl
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Marla Ferschl.

Additional information

This article is part of the Topical Collection on Pediatric Anesthesia.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Dong, T.T., Ferschl, M. What’s New in Craniosynostosis?. Curr Anesthesiol Rep 5, 168–176 (2015). https://doi.org/10.1007/s40140-015-0099-4

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40140-015-0099-4

Keywords

Search

Navigation