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2015 | 74 | 4 |

Tytuł artykułu

Craniovertebral anomalies associated with pituitary gland duplication

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Background: An extremely rare occurrence of the pituitary gland duplication inspired us to examine in detail the accompanying craniovertebral congenital anomalies in a patient involved. Materials and methods: T1-wighted magnetic resonance imaging (MRI) was performed, as well as the multislice computerised tomography (MSCT) and MSCT angiography in our patient, as well as in a control group of 10 healthy subjects. Results: In a 20-year-old male a double pituitary gland was identified, as well as hypothalamic enlargement, tuberomamillary fusion and hamartoma. In addition, the patient also showed a duplicated hypophyseal fossa and posterior clinoid processes, notch of the upper sphenoid, prominent inner relief of the skull, inverse shape of the foramen magnum, third occipital condyle, partial aplasia of the anterior and posterior arches of the atlas with a left arcuate foramen, duplication of the odontoid process and the C2 body, and fusion of the C2–C4 and T12–L1 vertebrae. The MSCT angiography presented a segmental dilatation of both vertebral arteries and the A2 segment of the anterior cerebral artery, as well as a duplication of the basilar artery. Conclusions: This patient is unique due to complex craniovertebral congenital anomalies associated with a duplication of the pituitary gland. (Folia Morphol 2015; 74, 4: 524–531)

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  • Neuroanatomy and Gross Anatomy, Institute of Anatomy, Faculty of Medicine, University of Belgrade, Serbia
  • Neuroanatomy and Gross Anatomy, Institute of Anatomy, Faculty of Medicine, University of Belgrade, Serbia
  • Neuroanatomy and Gross Anatomy, Institute of Anatomy, Faculty of Medicine, University of Belgrade, Serbia
  • Neuroanatomy and Gross Anatomy, Institute of Anatomy, Faculty of Medicine, University of Belgrade, Serbia
  • Neuroanatomy and Gross Anatomy, Institute of Anatomy, Faculty of Medicine, University of Belgrade, Serbia
  • Neuroanatomy and Gross Anatomy, Institute of Anatomy, Faculty of Medicine, University of Belgrade, Serbia


  • 1. Ahmed R, Traynelis VC, Menezes AH (2008) Fusions at the craniovertebral junction. Child Nerv Syst, 24: 1209–1224.
  • 2. Alvarez Caro F, Pumarada Prieto PM, Alvarez Berciano F (2008) Congenital defect of the atlas and axis. A cause of misdiagnose when evaluating an acute neck trauma. Am J Emerg Med, 26: 840.e1–e2.
  • 3. Arvin B, Fournier-Gosselin MP, Fehlings MG (2010) Os odontoideum: etiology and surgical management. Neurosurgery, 66 (3 suppl.): A22–A31.
  • 4. Bagherian V, Graham M, Gerson LP, Armstrong DL (1984) Double pituitary glands with partial duplication of facial and forebrain structures with hydrocephalus. Comput Radiol, 8: 203–210.
  • 5. Bale PM, Reye RD (1976) Epignathus, double pituitary and agenesis of corpus callosum. J Pathol, 120: 161–164.
  • 6. Bliemel C, Kuehl H, Ruchholtz S, Kühne CA (2009) Partial aplasia of the atlas in a child. Unfallchirurg, 112: 513–516.
  • 7. Burke M, Zinkovsky S, Abrantes MA, Riley W (2000) Duplication of the hypophysis. Pediatr Neurosurg, 33: 95–99.
  • 8. Chau AM, Wong JH, Mobbs RJ (2009) Cervical myelopathy associated with congenital C2/3 canal stenosis and deficiencies of the posterior arch of the atlas and laminae of the axis: case report and review of the literature. Spine, 34: E886–E891.
  • 9. Currarino G, Rollins N, Diehl JT (1994) Congenital defects of the posterior arch of the atlas: a report of seven cases including an affected mother and son. Am J Neuroradiol, 15: 249–254.
  • 10. Davis D, Gutierrez FA (1977) Congenital anomaly of the odontoid in children. A report of four cases. Child Brain, 3: 219–229.
  • 11. de Penna GC, Pimenta MP, Drummond JB, Sarquis M, Martins JC, de Campos RC, Dias EP (2005) Duplication of the hypophysis associated with precocious puberty: presentation of two cases and review of pituitary embryogenesis. Arq Bras Endocrinol Metabol, 49: 323–327.
  • 12. El Asri AC, Akhaddar A, Gazzaz M, Okacha N, Boulhroud O, Baallal H, Belfquih H, Belhachmi A, Mandour C, El Mostarchid B, Boucetta M (2010). Dynamic CT scan of the craniovertebral junction: a role in the management of os odontoideum. Neurol Neurochir Pol, 44: 603–608.
  • 13. Fielding W, Hensinger RN, Arbor A, Hawkins RJ (1980) Os odontoideum. J Bone Joint Surg Am, 62: 376–383.
  • 14. Ganau M, Spinelli R, Tacconi L (2013) Complex developmental abnormality of the atlas mimicking a Jefferson fracture: diagnostic tips and tricks. J Emerg Trauma Shock, 6: 47–49.
  • 15. Garant M, Oudjhane K, Sinsky A, O’Gorman AM (1997) Duplicated odontoid process: plain radiographic and CT appearance of a rare congenital anomaly of the cervical spine. Am J Neuroradiol, 18: 1719–1720.
  • 16. Garrett M, Consiglieri G, Kakarla UK, Cang SW, Dickman CA (2010) Occipitoatlantal dislocation. Neurosurgery, 66 (3 suppl.): A48–A55.
  • 17. Goel A, Shah A (2010) Unusual bone formation in the anterior rim of foramen magnum: cause, effect and treatment. Eur Spine J, 19 (suppl. 2): S162–S164.
  • 18. Gupta S, Phadke RV, Jain VK (1993) C1–C2 block vertebra with fusion of anterior arch of atlas and the odontoid. Australas Radiol, 37: 95–96.
  • 19. Gurney JW (1986) Absent dens on submentovertex view of the skull: new sign of an abnormal odontoid. Can Assoc Radiol J, 37: 38–39.
  • 20. Hamon-Kérautret M, Ares GS, Demondion X, Rouland V, Francke JP, Pruvo JP (1998) Duplication of the pituitary gland in a newborn with median cleft face syndrome and nasal teratoma. Pediatr Radiol, 28: 290–292.
  • 21. Hankinson TC, Anderson RCE (2010) Craniovertebral junction abnormalities in Down syndrome. Neurosurgery, 66 (3 suppl.): A32–A38.
  • 22. Hähnel S, Schramm P, Hassfeld S, Steiner HH, Seitz A (2003) Craniofacial duplication (diprosopus): CT MR imaging, and MR angiography indings. Case report. Radiology, 226: 210–213.
  • 23. Hori A (1983) A brain with two hypophyses in median cleft face syndrome. Acta Neuropathol, 59: 150–154.
  • 24. Hu Y, Ma W, Xu R (2009) Transoral osteosynthesis C1 as a function-preserving option in the treatment of bipartite: atlas deformity: a case report. Spine, 34: E418–E421.
  • 25. Iikko E, Tikkakoski T, Pyhtinen J (1998) The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia. Eur J Radiol, 29: 55–60.
  • 26. Ilina EG, Laziuk GI (1989) A new case of the “double hypophysis-multiple congenital developmental defect” complex. Tsitol Genet, 23: 45–46.
  • 27. Klimo P JR, Blumenthal DT, Couldwell WT (2003) Congenital partial aplasia of the posterior arch of the atlas causing myelopathy: case report and review of the literature. Spine, 28: E224–228.
  • 28. Lampropoulou-Adamidou K, Athanassacopoulos M, Karampinas PK, Vlamis J, Korres DS, Pneumaticos SG (2013). Congenital variations of the upper cervical spine and their importance in preoperative diagnosis. A case report and a review of the literature. Eur J Orthop Surg Traumatol, 23 (suppl. 1): S101–S105.
  • 29. Manara R, Citton V, Rossetto M, Padoan A, D’Avella D (2009) Hypophyseal triplication: case report and embryologic considerations. Am J Neuroradiol, 30: 1328-1329.
  • 30. Manjila S, Miller EA, Vadera S, Goel RK, Khan FR, Crowe C, Geertman RT (2012) Duplication of the pituitary gland associated with multiple blastogenesis defects: Duplication of the pituitary gland (DPG)-plus syndrome. Case report and review of literature. Surg Neurol Int, 3: 23–35.
  • 31. Martin MD, Bruner HJ, Majman DJ (2010) Anatomic and biomechanical considerations of the craniovertebral junction. Neurosurgery, 66 (3 suppl.): A2–A6.
  • 32. Menezes AH, VanGilder JC, Graf CJ, McDonnell DE (1980) Craniocervical abnormalities. A comprehensive surgical approach. J Neurosurg, 53: 444–455.
  • 33. Michie I, Clark M (1968) Neurological syndromes associated with cervical and craniocervical anomalies. Arch Neurol, 18: 241–247.
  • 34. Mutlu H, Paker B, Bunes N, Emektar A, Keceli M, Kantarci M (2004) Pituitary duplication associated with oral dermoid and corpus callosum hypogenesis. Neuroradiology, 46: 1036–1038.
  • 35. Phan N, Marras C, Midha R, Rowed D (1998) Cervical myelopathy caused by hypoplasia of the atlas: two case reports and review of the literature. Neurosurgery, 43: 629–633.
  • 36. Quinteiro Antolin T, Castellano Romero I, Yáñze Calvo J (2012) Aplasia of the posterior arches of the atlas: a presentation of 2 cases. Rev Esp Cir Ortop Traumatol, 56: 381–384.
  • 37. Roessmann U (1984) Duplication of the pituitary gland and spinal cord. Arch Pathol Lab Med, 109: 518–520.
  • 38. Sabuncuoglu H, Ozdogan S, Karadag D, Kaynak ET (2011) Congenital hypoplasia of the posterior arch of the atlas: case report and extensive review of the literature. Turk Neurosurg, 21: 97–103.
  • 39. Salunke P, Futane S, Sharma M, Sahoo S, Kovilapu U, Khandelwal NK (2015) “Pseudofacets” or “supernumerary facets” in congenital atlanto-axial dislocation: boon or bane? Eur Spine J, 24: 80-87.
  • 40. Samartzis D, Kalluri P, Herman J, Lubicky JP, Shen FH (2008) 2008 young investigator award: The role of congenitally fused cervical segments upon the space available for the cord and associated symptoms in Klippel-Feil patients. Spine, 33: 1442–1450.
  • 41. Shah P, Likeman M, Munro-Davies L (2007) Bradycardia in minor trauma: don’t be slow on the uptake! Emerg Med J, 24: e13.
  • 42. Shah S, Pereira JK, Becker CJ, Roubal SE (1997) Duplication of pituitary gland. J Comput Assist Tomogr, 21: 459–461.
  • 43. Shen W, Cui J, Chen J, Ji Y, Zou J, Chen H, Xiongzheng M (2013) Partial midfacial duplication. J Craniofac Surg, 24: 934–936.
  • 44. Shroff M, Blaser S, Jay V, Chitayat D, Armstrong D (2003) Basilar artery duplication associated with pituitary duplication: a new finding. Am J Neuroradiol, 24: 956–961.
  • 45. Slavotinek A, Parisi M, Heike C, Hing A, Huang E (2005) Craniofacial defects of blastogenesis: Duplication of pituitary with cleft palate and oropharyngeal tumors. Am J Med Genet A, 35: 13–20.
  • 46. Smith JS, Shaffrey CI, Abel MF, Menezes AH (2010) Basilar invagination. Neurosurgery, 66 (3 suppl.): A39–A47.
  • 47. Steinmetz MP, Mroz TE, Benzel EC (2010) Craniovertebral junction: biomechanical considerations. Neurosurgery, 66 (3 suppl.): A7–A12.
  • 48. Stevens CA, Pearce RG, Burton EM (2009) Familial odontoid hypoplasia. Am J Med Genet A, 49A: 1290–1292.
  • 49. Tagliavini F, Pilleri G (1986) Mammillo-hypophyseal duplication (diplo-mammillo-hypophysis). Acta Neuropathol, 69: 38–44.
  • 50. Travan L, Saccheri P, Sabbadini G, Crivellato E (2011) Bilateral arcuate foramen associated with partial defect of the posterior arch of the atlas in a medieval skeleton: case report and review of the literature. Looking backward to go forward. Surg Radiol Anat, 33: 495–500.
  • 51. Tsuang FY, Chen JY, Wang YH, Lai DM (2011). Neurological picture. Occipitocervical malformation with atlas duplication. J Neurol Neurosurg Psychiatry, 82: 1101–1102.
  • 52. Vieira TC, Chinen RN, Ribeiro MR, Nogueira RG, Abucham J (2007) Central precocious puberty associated with pituitary duplication and midline defects. J Pediatr Endocrinol Metab, 20: 1141–1144.
  • 53. Vittore CP, Murray RA, Martin LS (2005) Case 79: pituitary duplication. Radiology, 234: 411–414.
  • 54. Westermeyer RR (2003) Odontoid hypoplasia presenting as torticollis: a discussion of its significance. J Emerg Med, 24: 15–18.
  • 55. Yamamoto T, Kurosawa K, Masuno M, Okuzumi S, Kondo S, Miyama S, Okamoto N, Aida N, Nishimura G (2005) Congenital anomaly of cervical vertebrae is a major complication of Rubinstein-Taybi syndrome. Am J Med Genet A, 135: 130–133.

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