SpringerLink
Log in

Computer tomography–based quantitative analysis of the orbital proptosis severity in infants with syndromic craniosynostosis: case-control study

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

Evaluation of orbital proptosis and sutural synostosis pattern along the coronal ring in craniofaciosynostosis patients with or without fibroblastic growth factor receptor 2 (FGFR2) mutation.

Methods

High-resolution computer tomography was used to assess, in children with or without FGFR2 mutation, the early synostotic involvement of the “major” and “minor” sutures/synchondroses of the coronal arch along with the following orbital parameters: interorbital angle, bone orbital cavity volume, globe volume, ventral globe volume, ventral globe index.

Results

Infants with FGFR2 mutation showed an increased number of closed minor sutures/synchondroses along the posterior coronal branch while both groups showed a comparable synostotic involvement of the minor sutures of the anterior coronal branch. FGFR2 infants with posterior coronal branch synostotic involvement showed a higher degree of proptosis due to both reduced bony cavity volume and increased globe volume (p<0.05).

Conclusions

Our data show that FGFR2 mutation together with posterior coronal branch synostotic involvement has a synergic effect in causing a more severe degree of orbital proptosis.

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

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price includes VAT (Canada)

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Forte AJ, Steinbacher DM, Persing JA, Brooks ED, Andrew TW, Alonso N (2015) Orbital dysmorphology in untreated children with Crouzon and Apert syndromes. Plast Reconstr Surg 136:1054–1062. https://doi.org/10.1097/PRS.0000000000001693

    Article  CAS  PubMed  Google Scholar 

  2. Carr M, Posnick JC, Pron G, Armstrong D (1992) Cranio-orbito-zygomatic measurements from standard CT scans in unoperated Crouzon and Apert infants: comparison with normal controls. Cleft Palate Craniofac J 29:129–136. https://doi.org/10.1597/1545-1569_1992_029_0129_cozmfs_2.3.co_2

    Article  CAS  PubMed  Google Scholar 

  3. Batut C, Joly A, Travers N, Guichard B, Paré A, Laure B (2019) Surgical treatment of orbital hypertelorism: historical evolution and development prospects. J Craniomaxillofac Surg 47:1712–1719. https://doi.org/10.1016/j.jcms.2019.07.002

    Article  PubMed  Google Scholar 

  4. Oreroglu AR, Silav G, Ozkaya O, Orman C, Akan M (2013) Concurrent repair of orbital shallowness with craniosynostosis surgery: two late cases of simultaneous orbital decompression. Turk Neurosurg 23:395–400. https://doi.org/10.5137/1019-5149.JTN.5444-11.2

    Article  PubMed  Google Scholar 

  5. Newman SA (1991) Ophthalmic features of craniosynostosis. Neurosurg Clin N Am 2:587–610

    Article  CAS  Google Scholar 

  6. Azoury SC, Reddy S, Shukla V, Deng CX (2017) Fibroblast growth factor receptor 2 (FGFR2) mutation related syndromic craniosynostosis. Int J Biol Sci 13:1479–1488. https://doi.org/10.7150/ijbs.22373

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Festa F, Pagnoni M, Valerio R, Rodolfino D, Saccucci M, d’Attilio M, Caputi S, Iannetti G (2012) Orbital volume and surface after Le Fort III advancement in syndromic craniosynostosis. J Craniofac Surg 23:789–792. https://doi.org/10.1097/SCS.0b013e31824dbeec

    Article  PubMed  Google Scholar 

  8. Imai K, Fujimoto T, Takahashi M, Maruyama Y, Yamaguchi K (2013) Preoperative and postoperative orbital volume in patients with Crouzon and Apert syndrome. J Craniofac Surg 24:191–194. https://doi.org/10.1097/SCS.0b013e3182668581

    Article  PubMed  Google Scholar 

  9. Cohen MM (1993) Sutural biology and the correlates of craniosynostosis. Am J Med Genet 47:581–616. https://doi.org/10.1002/ajmg.1320470507

    Article  PubMed  Google Scholar 

  10. Cruz AA, Garcia DM, Akaishi PM et al (2015) Globe protrusion and interorbital divergence in syndromic faciocraniosynostoses. J Craniofac Surg 26:1163–1164. https://doi.org/10.1097/SCS.0000000000001553

    Article  PubMed  Google Scholar 

  11. Calandrelli R, D’Apolito G, Gaudino S, Sciandra MC, Caldarelli M, Colosimo C (2014) Identification of skull base sutures and craniofacial anomalies in children with craniosynostosis: utility of multidetector CT. Radiol Med 119:694–704. https://doi.org/10.1007/s11547-014-0387-y

    Article  PubMed  Google Scholar 

  12. Ganesh A, Edmond J, Forbes B, Katowitz WR, Nischal KK, Miller M, Levin AV (2019) An update of ophthalmic management in craniosynostosis. J AAPOS 23:66–76. https://doi.org/10.1016/j.jaapos.2018.10.016

    Article  PubMed  Google Scholar 

  13. Runyan CM, Xu W, Alperovich M et al (2017) Minor suture fusion in syndromic craniosynostosis. Plast Reconstr Surg 140:434e–445e. https://doi.org/10.1097/PRS.0000000000003586

    Article  CAS  PubMed  Google Scholar 

  14. Bentley RP, Sgouros S, Natarajan K, Dover MS, Hockley AD (2002) Changes in orbital volume during childhood in cases of craniosynostosis. J Neurosurg 96:747–754. https://doi.org/10.3171/jns.2002.96.4.0747

    Article  PubMed  Google Scholar 

  15. Bentley RP, Sgouros S, Natarajan K, Dover MS, Hockley AD (2002) Normal changes in orbital volume during childhood. J Neurosurg 96:742–746. https://doi.org/10.3171/jns.2002.96.4.0742

    Article  PubMed  Google Scholar 

  16. Calandrelli R, D’Apolito G, Gaudino S, Stefanetti M, Massimi L, di Rocco C, Colosimo C (2014) Radiological assessment of skull base changes in children with syndromic craniosynostosis: role of “minor” sutures. Neuroradiology 56:865–875. https://doi.org/10.1007/s00234-014-1392-5

    Article  PubMed  Google Scholar 

  17. Hill CA, Vaddi S, Moffitt A, Kane AA, Marsh JL, Panchal J, Richtsmeier JT, Aldridge K (2011) Intracranial volume and whole brain volume in infants with unicoronal craniosynostosis. Cleft Palate Craniofac J 48:394–398. https://doi.org/10.1597/10-051

    Article  PubMed  Google Scholar 

  18. Lo LJ, Marsh JL, Kane AA, Vannier MW (1996) Orbital dysmorphology in unilateral coronal synostosis. Cleft Palate Craniofac J 33:190–197. https://doi.org/10.1597/1545-1569_1996_033_0190_odiucs_2.3.co_2

    Article  CAS  PubMed  Google Scholar 

  19. Khonsari RH, Way B, Nysjo J et al (2016) Fronto-facial advancement and bipartition in Crouzon-Pfeiffer and Apert syndromes: impact of fronto-facial surgery upon orbital and airway parameters in FGFR2 syndromes. J Craniomaxillofac Surg 44:1567–1575. https://doi.org/10.1016/j.jcms.2016.08.015

    Article  PubMed  Google Scholar 

  20. Cruz AA, Akaishi PM, Arnaud E et al (2007) Exorbitism correction of faciocraniosynostoses by monobloc frontofacial advancement with distraction osteogenesis. J Craniofac Surg 18:355–360. https://doi.org/10.1097/scs.0b013e3180333b3c

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Roma-UOC Radiologia e Neuroradiologia, Polo Diagnostica per immagini, radioterapia, oncologia ed ematologia, Area diagnostica per immagini, Largo Francesco Vito 1, -00168, Rome, Italy

    Rosalinda Calandrelli, Antonio Marrazzo, Marco Panfili & Cesare Colosimo

  2. Unit of Neurology, Neurophysiology, Department of Medicine, University Campus Bio-Medico, via Álvaro del Portillo 21, 00128, Rome, Italy

    Fabio Pilato

  3. Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Roma -UOC Neurochirurgia Infantile - Polo scienze dell’invecchiamento, neurologiche, ortopediche e della testa-collo, Area Neuroscienze, Rome, Italy

    Luca Massimi

  4. Università Cattolica del Sacro Cuore, Istituto di Neurochirurgia, Rome, Italy

    Luca Massimi

  5. International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  6. Università Cattolica del Sacro Cuore, Istituto di Radiologia, Rome, Italy

    Cesare Colosimo

Authors
  1. Rosalinda Calandrelli
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Fabio Pilato
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Antonio Marrazzo
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Luca Massimi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Marco Panfili
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Concezio Di Rocco
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Cesare Colosimo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Rosalinda Calandrelli.

Ethics declarations

Ethics approval

We declare that all procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study, formal consent is not required.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Conflict of interest

Rosalinda Calandrelli declares that she has no conflict of interest.

Fabio Pilato declares that he has no conflict of interest.

Antonio Marrazzo declares that he has no conflict of interest.

Luca Massimi declares that he has no conflict of interest.

Marco Panfili declares that he has no conflict of interest.

Cesare Colosimo declares that he is scientific consultant for Bracco Diagnostics Inc. and Bayer HealthCare.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Calandrelli, R., Pilato, F., Marrazzo, A. et al. Computer tomography–based quantitative analysis of the orbital proptosis severity in infants with syndromic craniosynostosis: case-control study. Childs Nerv Syst 37, 1659–1668 (2021). https://doi.org/10.1007/s00381-021-05062-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-021-05062-6

Keywords

Search

Navigation