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Craniocervical developmental anatomy and its implications

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Abstract

Introduction

Congenital and developmental osseous abnormalities and anomalies that affect the craniocervical junction complex can result in neural compression and vascular compromise and can manifest itself with abnormal cerebrospinal fluid dynamics. An understanding of the development of the craniocervical junction is essential to recognize the pathological abnormalities.

Materials and methods

Atlas assimilation, segmentation failures, os odontoideum, basilar invagination, and the various syndromes that affect the craniocervical junction have been analyzed. The natural history provides an added insight into its treatment.

Results

Proatlas segmentation abnormalities surrounded the foramen magnum and the posterior arch of C1. Hindbrain herniation was associated in 33 of the 90 children involved. Spastic quadriparesis presented in 80% and lower cranial nerve abnormalities in 33%. Vertebrobasilar dysfunction was observed in 40% and trauma presentation seen in 60% of individuals. Atlas assimilation was present in 550 individuals who were evaluated for craniovertebral junction abnormalities. Hindbrain herniation occurred in 38%. Segmentation failure of C2 and C3 vertebrae compounded the abnormal dynamics resulting in atlantoaxial instability. This was a reducible instability with formation of pannus around the odontoid process until it became irreducible at approximately 14 years of age. Unilateral atlas assimilation caused torticollis in children. Os odontoideum was investigated regarding craniocervical trauma at a young age.

Conclusion

The conclusion was that os odontoideum was associated with an unrecognized fracture in children below the age of 5 with a previously normal odontoid structure as observed in our series. Atlas and axis abnormalities were reviewed in this series. This large database has provided an understanding of the natural history of many entities and allowed treatment protocols to be established that have stood the test of time.

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References

  1. Asakawa H, Yanaka K, Narushima K, Meguro K, Nose T (1999) Anomaly of the axis causing cervical myelopathy. Case report. J Neurosurg 91:121–123

    PubMed  CAS  Google Scholar 

  2. Bhatnagar M, Sponseller PD, Carroll C IV, Tolo VT (1991) Pediatric atlantoaxial instability presenting as cerebral and cerebellar infarcts. J Pediatr Orthop 11:103–107

    PubMed  CAS  Google Scholar 

  3. Chisaka O, Capecchi MR (1991) Regionally restricted developmental defects resulting from targeted disruption of the mouse homeobox gene hox-1.5. Nature 350:473–479

    Article  PubMed  CAS  Google Scholar 

  4. Christ B, Wilting J (1992) From somites to vertebral column. Ann Anat 174:23–32

    PubMed  CAS  Google Scholar 

  5. Condie BG, Capecchi MR (1993) Mice homozygous for a targeted disruption of Hoxd-3 (Hox-4.1) exhibit anterior transformations of the first and second cervical vertebrae, the atlas and the axis. Development 119:579–595

    PubMed  CAS  Google Scholar 

  6. David KM, McLachlan JC, Aiton JF, Whiten SC, Smart SD, Thorogood PV, Crockard HA (1998) Cartilaginous development of the human craniovertebral junction as visualized by a new three-dimensional computer reconstruction technique. J Anat 192:269–277

    Article  PubMed  Google Scholar 

  7. Devi BI, Shenoy SN, Panigrahi MK, Chandramouli BA, Das BS, Jayakumar PN (1997) Anomaly of arch of atlas—a rare cause of symptomatic canal stenosis in children. Pediatr Neurosurg 26:214–218

    Article  PubMed  CAS  Google Scholar 

  8. Dietrich S, Kessel M (1997) The vertebral column. In: Thorogood P (ed) Embryos, genes and birth defects. Wiley, Chichester, pp 281–302

    Google Scholar 

  9. Fielding JW, Griffin PP (1974) Os odontoideum. An acquired lesion. J Bone Jt Surg Am 56(l):187–190

    CAS  Google Scholar 

  10. Ganguly DN, Roy KKS (1964) A study on the craniovertebral joint in man. Anat Anz Bd 114:433–452

    CAS  Google Scholar 

  11. Garg A, Gaikwad SB, Gupta V, Mishra NK, Kale SS, Singh J (2004) Bipartite atlas with os odontoideum. Spine 29(2):E35–E38

    Article  PubMed  Google Scholar 

  12. Gasser RF (1976) Early formation of the basicranium in man. In: Bosma JF (ed) Symposium on Development of the Basicranium. Department of Health Education and Science Publication (NIH), Bethesda, Maryland, pp 29–43

    Google Scholar 

  13. Gehweiler J, Daffner R, Roberts LJ (1983) Malformations of the atlas vertebra simulating the Jefferson fracture. Am J Roentgenol 149:1083–1086

    Google Scholar 

  14. Gholve PA, Hosalka HS, Ricchetti ET, Pollock AN, Dormans JP, Drummond DS (2007) Occipitalization of the atlas in children. J Bone Joint Surg Am 89:571–578

    Article  PubMed  Google Scholar 

  15. Gladstone J, Erickson-Powell W (1914–1915) Manifestation of occipital vertebra and fusion of atlas with occipital bone. J Anat Physiol 49:190–199

    Google Scholar 

  16. Gladstone RJ, Wakeley CPG (1924–1925) Variations of the occipito-atlantal joint in relation to the metameric structure of the craniovertebral region. J Anat 59:195–216

    Google Scholar 

  17. Kessel M, Gruss P (1991) Homeotic transformations of murine vertebrae and concomitant alteration of Hox codes induced by retinoic acid. Cell 67:89–104

    Article  PubMed  CAS  Google Scholar 

  18. Kessel M (1993) Reversal of axonal pathways from rhombomere 3 correlates with extra Hox expression domains. Neuron 10:379–393

    Article  PubMed  CAS  Google Scholar 

  19. Keynes RJ, Stern C (1988) Mechanisms of vertebrate segmentation. Development 103:413–429

    PubMed  CAS  Google Scholar 

  20. Kimura Y, Matsunami H, Inoue T, Shimamura K, Uchida N, Ueno T, Miyazaki T, Takeichi M (1995) Cadherin-11 expressed in association with mesenchymal morphogenesis in the head, somite and limb bud of early mouse embryos. Dev Biol 169:347–358

    Article  PubMed  CAS  Google Scholar 

  21. Komatsu Y, Shibata T, Yasuda S, Ono Y, Nose T (1993) Atlas hypoplasia as a cause of high cervical myelopathy. Case report. J Neurosurg 79:917–919

    Article  PubMed  CAS  Google Scholar 

  22. Koseki H, Wallin J, Wilting J et al (1993) A role of Pax-1 as a mediator of notochordal signals during the dorsoventral specification of vertebrae. Development 119:649–660

    PubMed  CAS  Google Scholar 

  23. Kotil K, Kalayci M (2005) Ventral cervicomedullary junction compression secondary to condyles occipitalis (median occipital condyle), a rare entity. J Spinal Disord Tech 18(4):382–384

    Article  PubMed  Google Scholar 

  24. Kountouri M, Natarajan K, Sgouros S (2004) Skull base growth in children with Chiari I malformation. Childs Nerv Syst 20:672

    Google Scholar 

  25. Lufkin T, Mark M, Hart CP et al (1992) Homeotic transformation of the occipital bones of the skull by ectopic expression of a homeobox gene. Nature 359:835–841

    Article  PubMed  CAS  Google Scholar 

  26. Mantilla-Martin MT, Miller JD (1993) Congenital absence of a pedicle in a cervical vertebra: Report of two cases. Can Assoc Radiol J 44:280–284

    PubMed  CAS  Google Scholar 

  27. Marin-Padilla M, Marin-Padilla T (1981) Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci 50:29–55

    Article  PubMed  CAS  Google Scholar 

  28. Marin-Padilla M (1979) Notochordal-basochondrocranium relationships. Abnormalities in experimentally induced axial skeletal (dysraphic) disorders. J Embryol Exp Morphol 53:15–38

    PubMed  CAS  Google Scholar 

  29. May D, Jenny B, Faundez A (2001) Cervical cord compression due to a hypoplastic atlas. Case report. J Neurosurg (Spine 1). 94:133–136

    PubMed  CAS  Google Scholar 

  30. Menezes AH (1995) Congenital and acquired abnormalities of the craniovertebral junction. In: Youmans J (ed) Neurological surgery. 4th edn. W.B. Saunders Publishers, Philadelphia, pp 1035–1089 (Chapter 42)

    Google Scholar 

  31. Menezes AH (2005) Craniocervical developmental anatomy and its implications. Clin Neurosurg 52:53–64

    PubMed  Google Scholar 

  32. Menezes AH (2003) Developmental abnormalities of the craniovertebral junction. In: Winn HR (ed) Youman’s neurological surgery. vol. 3. 5th edn. WB Saunders, Philadelphia, pp 3331–3345

    Google Scholar 

  33. Menezes AH (1998) Embryology, development and classification of disorders of the craniovertebral junction. In: Dickman CA, Sonntag VKH, Spetzler RF (eds) Surgery of the craniovertebral junction. Thieme Medical Publishers, New York, pp 3–12

    Google Scholar 

  34. Menezes AH (2004) Evaluation and treatment of congenital and developmental anomalies of the cervical spine. J Neurosurg (Spine 1) 2:188–197

    Google Scholar 

  35. Menezes AH (1995) Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): Data base analysis. Pediatr Neurosurg 23:260–269

    Article  PubMed  CAS  Google Scholar 

  36. Muller F, O’Rahilly R (1980) The human chondrocranium at the end of the embryonic period proper with particular reference to the nervous system. Am J Anat 159:33–58

    Article  PubMed  CAS  Google Scholar 

  37. Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y (1997) Pathogenesis of Chiari malformation: A morphometric study of the posterior cranial fossa. J Neurosurg 86:40–47

    PubMed  CAS  Google Scholar 

  38. Pérez-Vallina JR, Riano-Galán I, Cobo-Ruisánchez A, Orejas-Rodriguez-Arango G, López-Muniz C, Fernández-Martínez JM (2002) Congenital anomaly of craniovertebral junction: atlas–dens fusion with C1 anterior arch cleft. J Spinal Disord Tech 15(1):84–87

    PubMed  Google Scholar 

  39. Puca A, Scogna A, Rollo M (2000) Craniovertebral junction malformation and rotational occlusion of the vertebral artery. Br J Neurosurg 14(4):361–364

    Article  PubMed  CAS  Google Scholar 

  40. Rao PV m (2002) Median (third) occipital condyle. Clin Anat 15(2):148–151

    Article  PubMed  Google Scholar 

  41. Sensing EC (1957) The development of the occipital and cervical segments and their associated structures in human embryos. Contrib Embryol 36:152–161

    Google Scholar 

  42. Shen FH, Samartzis D, Herman J, Lubicky JP (2006) Radiographic assessment of segmental motion at the atlantoaxial junction in the Klippel–Feil patient. Spine 31(2):171–177

    Article  PubMed  Google Scholar 

  43. Smoker WRK (2000) MR imaging of the craniovertebral junction. Magn Reson Imaging Clin N Am 8(3):635–650

    PubMed  CAS  Google Scholar 

  44. Stevens JM, Chong WK, Barber C, Kendall BE, Crockard HA (1994) A new appraisal of abnormalities of the odontoid process associated with atlantoaxial subluxation and neurological disability. Brain 117(pt 1):133–148

    Article  PubMed  Google Scholar 

  45. Tominaga T, Takahashi T, Shimizu H, Yoshiomoto T (2002) Rotational vertebral artery occlusion from occipital bony anomaly: A rare cause of embolic stroke. Case report. J Neurosurg 97(6):1456–1459

    PubMed  Google Scholar 

  46. Ulmer JL, Elster AD, Ginsberg LE, Williams DW 3rd (1993) Klippel–Feil syndrome: CT and MR of acquired and congenital abnormalities of cervical spine and cord. J Comput Assist Tomogr 17(2):215–224

    Article  PubMed  CAS  Google Scholar 

  47. VonTorklus D, Gehle W (1972) The upper cervical spine. Regional anatomy, pathology and traumatology. In: Georg Thieme Verlag (ed) A systemic radiological atlas and textbook. Grune & Stratton, New York, pp 1–99

    Google Scholar 

Download references

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Authors and Affiliations

  1. Department of Neurosurgery, University of Iowa Hospitals and Clinics 200 Hawkins Drive, 1824 JPP, Iowa City, IA, 52242, USA

    Arnold H. Menezes

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Correspondence to Arnold H. Menezes.

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Menezes, A.H. Craniocervical developmental anatomy and its implications. Childs Nerv Syst 24, 1109–1122 (2008). https://doi.org/10.1007/s00381-008-0600-1

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