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Role of “major” and “minor” lambdoid arch sutures in posterior cranial fossa changes: mechanism of cerebellar tonsillar herniation in infants with multisutural craniosynostosis

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Abstract

Purpose

This study aimed to explain the functional role of lambdoid arch sutures in the development of cerebellar tonsillar herniation. Posterior cranial fossa (PCF) changes were investigated in infants with premature synostosis of the major and minor sutures of the lambdoid arch without premature synostosis of the PCF synchondroses.

Methods

Morphometric and volumetric PCF measurements were performed on preoperative high-resolution CT studies in 12 infants with multisutural craniosynostosis involving the lambdoid arch and compared with those of 12 age-matched healthy subjects.

Results

All 12 patients had hypoplasia of PCF bone structures and normal volumes of the PCF and neural structures. PCF hypoplasia was related to exocciput length in infants with isolated involvement of major sutures, while it was related to posterior skull base hemifossae in infants with isolated involvement of minor lambdoid arch sutures. Foramen magnum AP diameter was reduced in babies with major suture involvement and tonsillar herniation, while foramen magnum AP and LL diameters were reduced in babies with minor suture involvement without tonsillar herniation. Right and left jugular foramen (JF) areas differed in all infants; however, the area of the smaller JF was significantly reduced only in infants with involvement of minor lambdoid arch sutures.

Conclusion

Hypoplasia of PCF bone structures due to sutural synostosis of the lambdoid arch is a required predisposing but not sufficient factor for the development of cerebellar tonsillar herniation through the foramen magnum. Normal PCF volume and foramen magnum anatomy may partly explain the development of cerebellar tonsil herniation in infants with lambdoid arch synostosis.

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References

  1. 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

    Article  PubMed  Google Scholar 

  2. Canpolat A, Akcakaya MO, Altunrende E, Ozlu HM, Duman H, Ton T, Akdemir O (2014) Chiari type I malformation yielded to the diagnosis of Crouzon syndrome. J Neurosci Rural Pract 5:81–83

    Article  PubMed  PubMed Central  Google Scholar 

  3. Chumas PD, Cinalli G, Arnaud E, Marchac D, Renier D (1997) Classification of previously unclassified cases of craniosynostosis. J Neurosurg 86:177–181

    Article  CAS  PubMed  Google Scholar 

  4. Cinalli G, Renier D, Sebag G, Sainte-Rose C, Arnaud E, Pierre-Kahn A (1995) Chronic tonsillar herniation in Crouzon’s and Apert’s syndromes: the role of premature synostosis of the lambdoid suture. J Neurosurg 83:575–582

    Article  CAS  PubMed  Google Scholar 

  5. Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F, Cianciulli E, Renier D (2005) Chiari malformation in craniosynostosis. Childs Nerv Syst 21:889–901

    Article  PubMed  Google Scholar 

  6. Di Rocco C, Frassanito P, Massimi L, Peraio S (2011) Hydrocephalus and Chiari type I malformation. Childs Nerv Syst 27:1653–1664

    Article  PubMed  Google Scholar 

  7. Fearon JA, Rhodes J (2009) Pfeiffer syndrome: a treatment evaluation. Plast Reconstr Surg 123:1560–1569

    Article  CAS  PubMed  Google Scholar 

  8. Francis PM, Beals S, Rekate HL, Pittman HW, Manwaring K, Reiff J (1992) Chronic tonsillar herniation and Crouzon’s syndrome. Pediatr Neurosurg 18:202–206

    Article  CAS  PubMed  Google Scholar 

  9. Johnston I, Jacobson E, Besser M (1998) The acquired Chiari malformation and syringomyelia following spinal CSF drainage: a study of incidence and management. Acta Neurochir (Wien) 140:417–427, discussion 427–418

    Article  CAS  Google Scholar 

  10. Kreiborg S, Marsh JL, Cohen MM Jr, Liversage M, Pedersen H, Skovby F, Borgesen SE, Vannier MW (1993) Comparative three-dimensional analysis of CT-scans of the calvaria and cranial base in Apert and Crouzon syndromes. J Craniomaxillofac Surg 21:181–188

    Article  CAS  PubMed  Google Scholar 

  11. Leikola J, Koljonen V, Valanne L, Hukki J (2010) The incidence of Chiari malformation in nonsyndromic, single suture craniosynostosis. Childs Nerv Syst 26:771–774

    Article  PubMed  Google Scholar 

  12. Loukas M, Shayota BJ, Oelhafen K, Miller JH, Chern JJ, Tubbs RS, Oakes WJ (2011) Associated disorders of Chiari type I malformations: a review. Neurosurg Focus 31:E3

    Article  PubMed  Google Scholar 

  13. Medina LS, Richardson RR, Crone K (2002) Children with suspected craniosynostosis: a cost-effectiveness analysis of diagnostic strategies. AJR Am J Roentgenol 179:215–221

    Article  PubMed  Google Scholar 

  14. Noudel R, Jovenin N, Eap C, Scherpereel B, Pierot L, Rousseaux P (2009) Incidence of basioccipital hypoplasia in Chiari malformation type I: comparative morphometric study of the posterior cranial fossa. Clinical article. J Neurosurg 111:1046–1052

    Article  PubMed  Google Scholar 

  15. Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C (2008) The natural history of the Chiari type I anomaly. J Neurosurg Pediatr 2:179–187

    Article  PubMed  Google Scholar 

  16. Pouratian N, Sansur CA, Newman SA, Jane JA Jr, Jane JA Sr (2007) Chiari malformations in patients with uncorrected sagittal synostosis. Surg Neurol 67:422–427, discussion 427–428

    Article  PubMed  Google Scholar 

  17. Richtsmeier JT, Lele S (1990) Analysis of craniofacial growth in Crouzon syndrome using landmark data. J Craniofac Genet Dev Biol 10:39–62

    CAS  PubMed  Google Scholar 

  18. Rodrigues VJ, Elsayed S, Loeys BL, Dietz HC, Yousem DM (2009) Neuroradiologic manifestations of Loeys-Dietz syndrome type 1. AJNR Am J Neuroradiol 30:1614–1619

    Article  CAS  PubMed  Google Scholar 

  19. Saldino RM, Steinbach HL, Epstein CJ (1972) Familial acrocephalosyndactyly (Pfeiffer syndrome). Am J Roentgenol Radium Ther Nucl Med 116:609–622

    Article  CAS  PubMed  Google Scholar 

  20. Schweitzer T, Bohm H, Meyer-Marcotty P, Collmann H, Ernestus RI, Krauss J (2012) Avoiding CT scans in children with single-suture craniosynostosis. Childs Nerv Syst 28:1077–1082

    Article  CAS  PubMed  Google Scholar 

  21. Sgouros S, Natarajan K, Hockley AD, Goldin JH, Wake M (1999) Skull base growth in craniosynostosis. Pediatr Neurosurg 31:281–293

    Article  CAS  PubMed  Google Scholar 

  22. Stovner LJ, Bergan U, Nilsen G, Sjaastad O (1993) Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation. Neuroradiology 35:113–118

    Article  CAS  PubMed  Google Scholar 

  23. Strahle J, Muraszko KM, Buchman SR, Kapurch J, Garton HJ, Maher CO (2011) Chiari malformation associated with craniosynostosis. Neurosurg Focus 31:E2

    Article  PubMed  Google Scholar 

  24. Taylor WJ, Hayward RD, Lasjaunias P, Britto JA, Thompson DN, Jones BM, Evans RD (2001) Enigma of raised intracranial pressure in patients with complex craniosynostosis: the role of abnormal intracranial venous drainage. J Neurosurg 94:377–385

    Article  CAS  PubMed  Google Scholar 

  25. Thompson DN, Harkness W, Jones BM, Hayward RD (1997) Aetiology of herniation of the hindbrain in craniosynostosis. An investigation incorporating intracranial pressure monitoring and magnetic resonance imaging. Pediatr Neurosurg 26:288–295

    Article  CAS  PubMed  Google Scholar 

  26. Tubbs RS, Elton S, Blount JP, Oakes WJ (2001) Preliminary observations on the association between simple metopic ridging in children without trigonocephaly and the Chiari I malformation. Pediatr Neurosurg 35:136–139

    Article  CAS  PubMed  Google Scholar 

  27. Vatansever D, Kyriakopoulou V, Allsop JM, Fox M, Chew A, Hajnal JV, Rutherford MA (2013) Multidimensional analysis of fetal posterior fossa in health and disease. Cerebellum 12:632–644

    Article  PubMed  Google Scholar 

  28. Yushkevich PA, Piven J, Hazlett HC, Smith RG, Ho S, Gee JC, Gerig G (2006) User-guided 3D active contour segmentation of anatomical structures: significantly improved efficiency and reliability. Neuroimage 31:1116–1128

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Institute of Radiology, Università Cattolica Sacro Cuore, L.go A. Gemelli 8, 00168, Rome, Italy

    Rosalinda Calandrelli, Gabriella D’Apolito, Marco Panfili & Cesare Colosimo

  2. Institute of Neurosurgery, Università Cattolica Sacro Cuore, L.go A. Gemelli 8, 00168, Rome, Italy

    Luca Massimi & Massimo Caldarelli

Authors
  1. Rosalinda Calandrelli
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  2. Gabriella D’Apolito
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  3. Marco Panfili
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  4. Luca Massimi
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  5. Massimo Caldarelli
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  6. Cesare Colosimo
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Correspondence to Rosalinda Calandrelli.

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Calandrelli, R., D’Apolito, G., Panfili, M. et al. Role of “major” and “minor” lambdoid arch sutures in posterior cranial fossa changes: mechanism of cerebellar tonsillar herniation in infants with multisutural craniosynostosis. Childs Nerv Syst 32, 451–459 (2016). https://doi.org/10.1007/s00381-015-2956-3

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  • DOI: https://doi.org/10.1007/s00381-015-2956-3

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