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Syndrome-related outcomes following posterior vault distraction osteogenesis

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Abstract

Purpose

The most commonly occurring syndromic craniosynostoses are Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, and Saethre-Chotzen syndrome. There is insufficient data regarding postoperative syndrome–related outcomes following the posterior vault distraction osteogenesis (PVDO) procedure, as well as data addressing whether or not additional procedures will be subsequently necessary to comprehensively treat children who undergo PVDO. Thus, the objective of this study is to describe and compare syndrome-related potential complications and outcomes associated with the PVDO procedure.

Methods

An observational retrospective study was performed on consecutive patients (n=24) with Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, or Saethre-Chotzen syndrome, respectively, who underwent PVDO between 2012 and 2019. Demographic data (patient gender and age when the PVDO procedure was performed), diagnosis, surgery-related data, and outcome data (perioperative and midterm complications and need for additional surgery) were verified.

Results

Total relative blood transfusion volumes per kilogram for the patients were as follows: 22.75 ± 9.30 ml for Apert syndrome, 10.73 ± 2.28 ml for Crouzon syndrome (Apert versus Crouzon, p<0.05), 18.53 ± 8.08 ml for Pfeiffer syndrome, and 19.74 ± 9.12 ml for Saethre-Chotzen syndrome. None of the patients required a secondary procedure to alleviate intracranial pressure except for a Saethre-Chotzen patient.

Conclusion

PVDO is an effective technique to address elevated intracranial pressure in SC patients that alleviates the need for secondary procedures at midterm follow-up. Apert syndrome patients presented relatively higher total blood transfusion rates than Crouzon syndrome patients who were operated on at a later age and weighed more.

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References

  1. Allam KA, Wan DC, Khwanngern K, Kawamoto HK, Tanna N, Perry A, Bradley JP (2011) Treatment of Apert syndrome: a long-term follow-up study. Plast Reconstr Surg 127:1601–1611. https://doi.org/10.1097/PRS.0b013e31820a64b6

    Article  CAS  PubMed  Google Scholar 

  2. Asaad M, Goyal S, Klement KA, Denny AD (2018) Improvement of color vision following posterior cranial vault distraction for Crouzon syndrome. J Craniofac Surg 29:868–870. https://doi.org/10.1097/SCS.0000000000004353

    Article  PubMed  Google Scholar 

  3. Choi M, Flores RL, Havlik RJ (2012) Volumetric analysis of anterior versus posterior cranial vault expansion in patients with syndromic craniosynostosis. J Craniofac Surg 23:455–458. https://doi.org/10.1097/SCS.0b013e318240ff49

    Article  PubMed  Google Scholar 

  4. Chouairi F, Torabi SJ, Alperovich M (2019) National 30-day outcomes for posterior cranial vault distraction. J Craniofac Surg 30:761–766. https://doi.org/10.1097/SCS.0000000000005285

    Article  PubMed  Google Scholar 

  5. Clavien PA, Barkun J, de Oliveira ML, Vauthey JN, Dindo D, Schulick RD, de Santibanes E, Pekolj J, Slankamenac K, Bassi C, Graf R, Vonlanthen R, Padbury R, Cameron JL, Makuuchi M (2009) The Clavien-Dindo classification of surgical complications: five-year experience. Ann Surg 250:187–196. https://doi.org/10.1097/SLA.0b013e3181b13ca2

    Article  PubMed  Google Scholar 

  6. Derderian CA, Bastidas N, Bartlett SP (2012) Posterior cranial vault expansion using distraction osteogenesis. Childs Nerv Syst 28:1551–1556. https://doi.org/10.1007/s00381-012-1802-0

    Article  PubMed  Google Scholar 

  7. Derderian CA, Wink JD, McGrath JL, Collinsworth A, Bartlett SP, Taylor JA (2015) Volumetric changes in cranial vault expansion: comparison of fronto-orbital advancement and posterior cranial vault distraction osteogenesis. Plast Reconstr Surg 135:1665–1672. https://doi.org/10.1097/PRS.0000000000001294

    Article  CAS  PubMed  Google Scholar 

  8. Fanganiello RD, Sertie AL, Reis EM, Yeh E, Oliveira NA, Bueno DF, Kerkis I, Alonso N, Cavalheiro S, Matsushita H, Freitas R, Verjovski-Almeida S, Passos-Bueno MR (2007) Apert p.Ser252Trp mutation in FGFR2 alters osteogenic potential and gene expression of cranial periosteal cells. Mol Med 13:422–442. https://doi.org/10.2119/2007-00027.Fanganiello

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Fearon JA, Podner C (2013) Apert syndrome: evaluation of a treatment algorithm. Plast Reconstr Surg 131:132–142. https://doi.org/10.1097/PRS.0b013e3182729f42

    Article  CAS  PubMed  Google Scholar 

  10. Florisson JM, Barmpalios G, Lequin M, van Veelen ML, Bannink N, Hayward RD, Mathijssen IM (2015) Venous hypertension in syndromic and complex craniosynostosis: the abnormal anatomy of the jugular foramen and collaterals. J Craniomaxillofac Surg 43:312–318. https://doi.org/10.1016/j.jcms.2014.11.023

    Article  PubMed  Google Scholar 

  11. Goldstein JA, Paliga JT, Wink JD, Low DW, Bartlett SP, Taylor JA (2013) A craniometric analysis of posterior cranial vault distraction osteogenesis. Plast Reconstr Surg 131:1367–1375. https://doi.org/10.1097/PRS.0b013e31828bd541

    Article  CAS  PubMed  Google Scholar 

  12. Lin LO, Zhang RS, Hoppe IC, Paliga JT, Swanson JW, Bartlett SP, Taylor JA (2019) Onset and resolution of Chiari malformations and hydrocephalus in syndromic craniosynostosis following posterior vault distraction. Plast Reconstr Surg 144:932–940. https://doi.org/10.1097/PRS.0000000000006041

    Article  CAS  PubMed  Google Scholar 

  13. Marucci DD, Dunaway DJ, Jones BM, Hayward RD (2008) Raised intracranial pressure in Apert syndrome. Plast Reconstr Surg 122:1162–1168; discussion 1169-1170. https://doi.org/10.1097/PRS.0b013e31818458f0

    Article  CAS  PubMed  Google Scholar 

  14. Mazzaferro DM, Ter Maaten NS, Wes AM, Naran S, Bartlett SP, Taylor JA (2019) A Craniometric analysis of the posterior cranial base after posterior vault distraction. J Craniofac Surg 30:1692–1695. https://doi.org/10.1097/SCS.0000000000005496

    Article  PubMed  Google Scholar 

  15. McMillan K, Lloyd M, Evans M, White N, Nishikawa H, Rodrigues D, Sharp M, Noons P, Solanki G, Dover S (2017) Experiences in performing posterior calvarial distraction. J Craniofac Surg 28:664–669. https://doi.org/10.1097/SCS.0000000000003458

    Article  PubMed  Google Scholar 

  16. Mondal A, Rodriguez-Florez N, O'Hara J, Ong J, Jeelani NUO, Dunaway DJ, James G (2019) Lack of association of cranial lacunae with intracranial hypertension in children with Crouzon syndrome and Apert syndrome: a 3D morphometric quantitative analysis. Childs Nerv Syst 35:501–507. https://doi.org/10.1007/s00381-019-04059-6

    Article  PubMed  Google Scholar 

  17. Nowinski D, Saiepour D, Leikola J, Messo E, Nilsson P, Enblad P (2011) Posterior cranial vault expansion performed with rapid distraction and time-reduced consolidation in infants with syndromic craniosynostosis. Childs Nerv Syst 27:1999–2003. https://doi.org/10.1007/s00381-011-1563-1

    Article  PubMed  Google Scholar 

  18. Ong J, Harshbarger RJ 3rd, Kelley P, George T (2014) Posterior cranial vault distraction osteogenesis: evolution of technique. Semin Plast Surg 28:163–178. https://doi.org/10.1055/s-0034-1390169

    Article  PubMed  PubMed Central  Google Scholar 

  19. Park C, Wormald J, Miranda BH, Ong J, Hare A, Eccles S (2018) Perioperative blood loss and transfusion in craniosynostosis surgery. J Craniofac Surg 29:112–115. https://doi.org/10.1097/SCS.0000000000004098

    Article  PubMed  Google Scholar 

  20. Raposo-Amaral CE, Denadai R, do Monte Lameiro TM, de Oliveira YM, Raposo-Amaral CA (2019) Hand function in Apert syndrome. Plast Reconstr Surg Glob Open 7:e2230. https://doi.org/10.1097/GOX.0000000000002230

    Article  PubMed  PubMed Central  Google Scholar 

  21. Raposo-Amaral CE, Denadai R, Furlan P, Raposo-Amaral CA (2018) Treatment of Apert hand syndrome: strategies for achieving a five-digit hand. Plast Reconstr Surg 142:972–982. https://doi.org/10.1097/PRS.0000000000004815

    Article  CAS  PubMed  Google Scholar 

  22. Raposo-Amaral CE, Denadai R, Maximo G, Raposo-Amaral CA, Ghizoni E (2020) Pfeiffer syndrome: a therapeutic algorithm based on a modified grading scale. Plast Reconstr Surg Glob Open 8:e2788. https://doi.org/10.1097/GOX.0000000000002788

    Article  PubMed  PubMed Central  Google Scholar 

  23. Raposo-Amaral CE, Denadai R, Oliveira YM, Ghizoni E, Raposo-Amaral CA (2020) Apert syndrome management: changing treatment algorithm. J Craniofac Surg 31:648–652. https://doi.org/10.1097/SCS.0000000000006105

    Article  PubMed  Google Scholar 

  24. Raposo-Amaral CE, Denadai R, Zanco GL, Ghizoni E, Raposo-Amaral CA (2020) Long-term follow-up on bone stability and complication rate after monobloc advancement in syndromic craniosynostosis. Plast Reconstr Surg 145:1025–1034. https://doi.org/10.1097/PRS.0000000000006646

    Article  CAS  PubMed  Google Scholar 

  25. Renier D, Sainte-Rose C, Marchac D, Hirsch JF (1982) Intracranial pressure in craniostenosis. J Neurosurg 57:370–377. https://doi.org/10.3171/jns.1982.57.3.0370

    Article  CAS  PubMed  Google Scholar 

  26. Sakamoto Y, Miwa T, Nakajima H, Yoshida K, Kishi K (2016) A new technique for posterior distraction in craniosynostosis: the double-door technique. J Craniofac Surg 27:950–952. https://doi.org/10.1097/SCS.0000000000002661

    Article  PubMed  Google Scholar 

  27. Serlo WS, Ylikontiola LP, Lahdesluoma N, Lappalainen OP, Korpi J, Verkasalo J, Sandor GK (2011) Posterior cranial vault distraction osteogenesis in craniosynostosis: estimated increases in intracranial volume. Childs Nerv Syst 27:627–633. https://doi.org/10.1007/s00381-010-1353-1

    Article  PubMed  Google Scholar 

  28. Shimizu A, Komuro Y, Shimoji K, Miyajima M, Arai H (2016) Quantitative analysis of change in intracranial volume after posterior cranial vault distraction. J Craniofac Surg 27:1135–1138. https://doi.org/10.1097/SCS.0000000000002739

    Article  PubMed  Google Scholar 

  29. Steinbacher DM, Skirpan J, Puchala J, Bartlett SP (2011) Expansion of the posterior cranial vault using distraction osteogenesis. Plast Reconstr Surg 127:792–801. https://doi.org/10.1097/PRS.0b013e318200ab83

    Article  CAS  PubMed  Google Scholar 

  30. Swanson JW, Samra F, Bauder A, Mitchell BT, Taylor JA, Bartlett SP (2016) An algorithm for managing syndromic craniosynostosis using posterior vault distraction osteogenesis. Plast Reconstr Surg 137:829e–841e. https://doi.org/10.1097/PRS.0000000000002127

    Article  CAS  PubMed  Google Scholar 

  31. Swanson JW, Xu W, Ying GS, Pan W, Lang SS, Heuer GG, Bartlett SP, Taylor JA (2020) Intracranial pressure patterns in children with craniosynostosis utilizing optical coherence tomography. Childs Nerv Syst 36:535–544. https://doi.org/10.1007/s00381-019-04448-x

    Article  PubMed  Google Scholar 

  32. Ter Maaten NS, Mazzaferro DM, Wes AM, Naran S, Bartlett SP, Taylor JA (2018) Craniometric analysis of frontal cranial morphology following posterior vault distraction. J Craniofac Surg 29:1169–1173. https://doi.org/10.1097/SCS.0000000000004473

    Article  PubMed  Google Scholar 

  33. Thompson DN, Harkness W, Jones B, Gonsalez S, Andar U, Hayward R (1995) Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management. Childs Nerv Syst 11:269–275. https://doi.org/10.1007/BF00301758

    Article  CAS  PubMed  Google Scholar 

  34. White N, Evans M, Dover MS, Noons P, Solanki G, Nishikawa H (2009) Posterior calvarial vault expansion using distraction osteogenesis. Childs Nerv Syst 25:231–236. https://doi.org/10.1007/s00381-008-0758-6

    Article  PubMed  Google Scholar 

  35. White N, Marcus R, Dover S, Solanki G, Nishikawa H, Millar C, Carver ED (2009) Predictors of blood loss in fronto-orbital advancement and remodeling. J Craniofac Surg 20:378–381. https://doi.org/10.1097/SCS.0b013e31819b9429

    Article  PubMed  Google Scholar 

  36. Wiberg A, Magdum S, Richards PG, Jayamohan J, Wall SA, Johnson D (2012) Posterior calvarial distraction in craniosynostosis - an evolving technique. J Craniomaxillofac Surg 40:799–806. https://doi.org/10.1016/j.jcms.2012.02.018

    Article  PubMed  Google Scholar 

  37. Woods RH, Ul-Haq E, Wilkie AO, Jayamohan J, Richards PG, Johnson D, Lester T, Wall SA (2009) Reoperation for intracranial hypertension in TWIST1-confirmed Saethre-Chotzen syndrome: a 15-year review. Plast Reconstr Surg 123:1801–1810. https://doi.org/10.1097/PRS.0b013e3181a3f391

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital, Av. Adolpho Lutz, 100, Caixa Postal 6028, Campinas, São Paulo, 13084-880, Brazil

    Cassio Eduardo Raposo-Amaral, Yuri Moresco de Oliveira, Rafael Denadai, Cesar Augusto Raposo-Amaral & Enrico Ghizoni

  2. Division of Neurosurgery, University of Campinas (UNICAMP), São Paulo, Brazil

    Cassio Eduardo Raposo-Amaral & Enrico Ghizoni

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  1. Cassio Eduardo Raposo-Amaral
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Correspondence to Cassio Eduardo Raposo-Amaral.

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Raposo-Amaral, C.E., de Oliveira, Y.M., Denadai, R. et al. Syndrome-related outcomes following posterior vault distraction osteogenesis. Childs Nerv Syst 37, 2001–2009 (2021). https://doi.org/10.1007/s00381-021-05169-w

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