Child's Nervous System

, Volume 25, Issue 10, pp 1221–1228

Surgical considerations in fourth ventricular ependymoma with the transcerebellomedullary fissure approach in focus

  • Kazuaki Shimoji
  • Masakazu Miyajima
  • Kostadin Karagiozov
  • Kenji Yatomi
  • Toshio Matsushima
  • Hajime Arai
Invited Paper



Within the existing consensus for the best management of pediatric infratentorial ependymomas (PIE), surgery is the most important stage, where complete removal should be the perfect aim, before complementing it with chemo- or radiotherapy. That, however, remains a challenge even for the most skillful surgeons because of the vicinity of important brainstem and cranial nerve structures involved and is particularly difficult in lateral extensions.

Materials and methods

The paper analyzes the current trends of PIE treatment with emphasis on resection difficulties created by lateral extensions. Anatomical analysis and clinical application of the cerebellomedullary fissure dissection has created specific approaches, providing safe route to the lateral recess and cerebellopontine area by dividing safely tenia and tonsils and biventer lobes retraction.

Discussion and conclusion

Bilateral and unilateral approaches have been developed. This approach prevents the damage of transvermian access and the resulting cerebellar mutism in some cases. Indications, technique and benefits of transcerebellomedullary fissure types of approaches are discussed.


Ependymoma Fourth ventricle Cerebellomedullary fissure Childhood Brain tumor 


  1. 1.
    Barone B, Elvidge A (1970) Ependymomas. A clinical study. J Neurosurg 33:428–438PubMedCrossRefGoogle Scholar
  2. 2.
    Bastian AJ, Mink JW, Kaufman BA (1998) Posterior vermian split syndrome. Ann Neurol 44:601–610PubMedCrossRefGoogle Scholar
  3. 3.
    Copeland DR, deMoor C, Moore BD 3rd, Ater JL (1999) Neurocognitive development of children after a cerebellar tumor in infancy: a longitudinal study. J Clin Oncol 17:3476–3486PubMedGoogle Scholar
  4. 4.
    Coulon R, Till K (1977) Intracranial ependymomas in children: a review of 43 cases. Childs Brain 3:154–168PubMedGoogle Scholar
  5. 5.
    Cully D, Berger M, Shaw D, Geyer R (1994) An analysis of factors determining the need for ventriculoperitoneal shunts following posterior fossa tumor surgery in children. Neurosurgery 34:402–408Google Scholar
  6. 6.
    Dailey AT, McKhann GMII, Berger MS (1995) The pathophysiology of oral pharyngeal apraxia and mutism following posterior fossa tumor resection in children. J Neurosurg 83:467–475PubMedCrossRefGoogle Scholar
  7. 7.
    Dietze DD Jr, Mickle JP (1990/1991) Cerebellar mutism after posterior fossa surgery. Pediatr Neurosurg 16:25–31CrossRefGoogle Scholar
  8. 8.
    Dohrmann GJ (1996) Ependymomas. In: Wilkins RH, Rengachary SS (eds) Neurosurgery, 2nd edn. McGraw-Hill, New York, pp 1195–1200Google Scholar
  9. 9.
    Duffner PK, Krischer JP, Horowitz ME, Cohen ME, Burger PC, Friedman HS, Kun LE (1998) Second malignancies in young children with primary brain tumors following treatment with prolonged postoperative chemotherapy and delayed irradiation: a Pediatric Oncology Group study. Ann Neurol 44:313–316PubMedCrossRefGoogle Scholar
  10. 10.
    El-Bahy K (2005) Telovelar approach to the fourth ventricle: operative findings and results in 16 cases. Acta Neurochir (Wien) 147:137–142CrossRefGoogle Scholar
  11. 11.
    Figarella-Branger D, Civatte M, Bouvier-Labit C, Gouvernet J, Gambarelli D, Gentet JC, Lena G, Choux M, Pellissier JF (2000) Prognostic factor in intracranial ependymomas in children. J Neurosurg 93:605–613PubMedCrossRefGoogle Scholar
  12. 12.
    Ferrante L, Mastronardi L, Acqui M, Fortuna A (1990) Mutism after posterior fossa surgery in children. Report of three cases. J Neurosurg 72:959–963PubMedCrossRefGoogle Scholar
  13. 13.
    Fokes E, Earle K (1969) Ependymomas: clinical and pathological aspects. J Neurosurg 30:585–594PubMedCrossRefGoogle Scholar
  14. 14.
    Gelabert-González M, Fernández-Villa J (2001) Mutism after posterior fossa surgery. Review of the literature. Clin Neurol Neurosurg 103:111–114PubMedCrossRefGoogle Scholar
  15. 15.
    Geyer JR, Zeltzer PM, Boyett JM, Rorke LB, Stanley P, Albright AL, Wisoff JH, Milstein JM, Allen JC, Finlay JL, Ayers GD, Shurin SB, Stevens KR, Bleyer WA (1994) Survival of infants with primitive neuroectodermal tumors or malignant ependymomas of the CNS treated with eight drugs in 1 day: a report from the children’s cancer group. J Clin Oncol 12:1607–1615PubMedGoogle Scholar
  16. 16.
    Gök A, Alptekin M, Erkutlu T (2004) Surgical approach to the fourth ventricle cavity through the cerebellomedullary fissure. Neurosurg rev 27:50–54PubMedCrossRefGoogle Scholar
  17. 17.
    Grundy RG, Wilne SA, Weston CL, Robinson K, Lashford LS, Ironside J, Cox T, Chong WK, Campbell RHA, Bailey CC, Gattamaneni R, Picton S, Thorpe N, Mallucci C, English MW, Punt JAG, Walker DA, Ellison DW, Machin D, for the Children’s Cancer and Leukaemia Group (formerly UKCCSG) Brain Tumour Committee (2007) Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children: the UKCCSG/SIOP prospective study. Lancet Oncol 8:696–705PubMedCrossRefGoogle Scholar
  18. 18.
    Healey EA, Barnes PD, Kupsky WJ, Scott RM, Sallan SE, Black PM, Tarbell NJ (1991) The prognostic significance of postoperative residual tumor in ependymoma. Neurosurgery 28:666–672PubMedCrossRefGoogle Scholar
  19. 19.
    Horn B, Heideman R, Geyer R, Pollack I, Packer R, Goldwein J, Tomita T, Schomberg P, Ater J, Luchtman-Jones L, Rivlin K, Lamborn K, Prados M, Bollen A, Berger M, Dahl G, Mcneil E, Patterson K, Shaw D, Kubalik M, Russo C (1999) A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21:203–211PubMedCrossRefGoogle Scholar
  20. 20.
    Ikezaki K, Matsushima T, Yokoyama N, Kaneko Y, Fukui M (1993) Correlation of microanatomical localization with postoperative survival in posterior fossa ependymomas. Neurosurgery 32:38–44PubMedCrossRefGoogle Scholar
  21. 21.
    Inoue T, Matsushima T, Inamura T, Kawamura T, Ishihara S, Fukui M (1998) Surgical approach to the mesencephalic vascular malformation-transcerebellomedullary fissure approach. Surg Cereb Stroke 26:287–291Google Scholar
  22. 22.
    Kellogg JX, Piatt JH Jr (1997) Resection of the fourth ventricle tumors without splitting the vermis: the cerebbelomedullary fissure approach. Pediatr Neurosurg 27:28–33PubMedCrossRefGoogle Scholar
  23. 23.
    Kobayashi S, Sugita K, Tanaka Y, Kyoshima K (1983) Infratentorial approach to the pineal region in the prone position: concord position. Technical note. J Neurosurg 58:141–143PubMedCrossRefGoogle Scholar
  24. 24.
    Lannering B, Marky I, Lundberg A, Olsson E (1990) Long-term sequelae after pediatric brain tumors: their effect on disability and quality of life. Med Pediatr Oncol 18:304–310PubMedCrossRefGoogle Scholar
  25. 25.
    Louis DN, Ohgaki H, Weistler OD, Cavenee WK (2007) World Health Organization classification of tumours of the central nervous system Chapter 3 : Ependymal tumours Pp 69-80 IARC, LyonGoogle Scholar
  26. 26.
    Matsushima T, Inoue T, Inamura T, Natori Y, Ikezaki K, Fukui M (2001) Transcerebellomedullary fissure approach with special reference to methods of dissecting the fissure. J Neurosurg 94:257–264PubMedCrossRefGoogle Scholar
  27. 27.
    Matsushima T, Ikezaki K, Inoue T (1998) Surgery of ependymomas in the fourth ventricle. In: Shibata S (ed) Surgery of glioma. Medica Suita City pp 54-62Google Scholar
  28. 28.
    Matsushima T, Fukui M, Inoue T, Natori Y, Baba T, Fujii K (1992) Microsurgical and magnetic resonance imaging anatomy of the cerebellomedullary fissure and its application during fourth ventricle surgery. Neurosurgery 30:325–330PubMedCrossRefGoogle Scholar
  29. 29.
    Matsushima T, Fukui M, Rhoton AL Jr (1989) Microsurgical anatomy of the fourth ventricle- special reference to the suboccipital midline approach. In: Kobayashi S (ed) Surgical anatomy for microneurosurgery II. SciMed, Tokyo pp 198-211Google Scholar
  30. 30.
    Matsushima T, Rhoton AL Jr, Lenkey C (1982) Microsurgery of the fourth ventricle. Part I: Microsurgical anatomy. Neurosurgery 11:631–667PubMedCrossRefGoogle Scholar
  31. 31.
    Merchant TE, Fouladi M (2005) Ependymoma: new therapeutic approaches including radiation and chemotherapy. J Neurooncol 75:287–299PubMedCrossRefGoogle Scholar
  32. 32.
    Mork S, Loken A (1977) Ependymoma. A follow-up study of 101 cases. Cancer 40:907–915PubMedCrossRefGoogle Scholar
  33. 33.
    Morota N, Deletis V, Epstein FJ, Kofler M, Abbott R, Lee M, Ruskin K (1995) Brain stem mapping: neurophysiological localization of motor nuclei on the floor of the fourth ventricle. Neurosurgery 37:922–930PubMedCrossRefGoogle Scholar
  34. 34.
    Morota N, Deletis V, Lee M, Epstein FJ (1996) Functional anatomic relationship between brain stem tumors and cranial motor nuclei. Neurosurgery 39:787–794PubMedCrossRefGoogle Scholar
  35. 35.
    Morota N, Deletis V, Epstein FJ (2002) Brainstem mapping. In: Deletis V, Shils JL (eds) Neurophysiology in neurosurgery. Academic, New York, pp 319–335CrossRefGoogle Scholar
  36. 36.
    Morota N, Deletis V (2006) The importance of brainstem mapping in brainstem surgical anatomy before the fourth ventricle and implication for intraoperative neurophysiological mapping. Acta Neurochir (Wien) 148:499–509CrossRefGoogle Scholar
  37. 37.
    Mulhern RK, Merchant TE, Gajjar A, Reddick WE, Kun LE (2004) Late neurocognitive sequelae in survivors of brain tumours in childhood. Lancet Oncol 5:399–408PubMedCrossRefGoogle Scholar
  38. 38.
    Mussi ALM, Rhoton AL Jr (2000) Telovelar approach to the fourth ventricle: microsurgical anatomy. J Neurosurg 92:812–823PubMedCrossRefGoogle Scholar
  39. 39.
    Nagib MG, O’fallon MT (1996) Posterior fossa lateral ependymoma in childhood. Pediatr Neurosurg 24:299–305PubMedCrossRefGoogle Scholar
  40. 40.
    Nazar GB, Hoffman HJ, Becker LE, Jenkin D, Humphreys RP, Hendrick EB (1990) Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72:408–417PubMedCrossRefGoogle Scholar
  41. 41.
    Oya N, Shibamoto Y, Nagata Y, Negoro Y, Hiraoka M (2002) Postoperative radiotherapy for intracranial ependymoma : analysis of prognostic factors and patterns of failure. J Neurooncol 56:87–94PubMedCrossRefGoogle Scholar
  42. 42.
    Palma L, Celli P, Mariottini A, Cantore G (1993) Supratentorial ependymomas of the first two decades of life. Long-term follow-up of 20cases (including two subependymomas). Neurosurgery 32:169–175PubMedCrossRefGoogle Scholar
  43. 43.
    Pierre-Kahn A, Hirsch JF, Roux FX, Renier D, Sainte-Rose C (1983) Intracranial ependymomas in childhood. Survival and functional results of 47 cases. Childs Brain 10:145–156PubMedGoogle Scholar
  44. 44.
    Pollak IF, Polinko P, Albright AL, Towbin R, Fitz C (1995) Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: Incidence and pathophysiology. Neurosurgery 37:885–893Google Scholar
  45. 45.
    Pollack IF, Gersten PC, Martinez AJ, Lo KH, Shultz B, Albright AL, Janosky J, Deutsch M (1995) Intracranial ependymoma of childhood: long-term outcome and prognostic factors. Neurosurgery 37:655–666, discussion 666-667PubMedCrossRefGoogle Scholar
  46. 46.
    Raimondi AJ, Tomita T (1981) Hydrocephalus and infratentorial tumors: Incidence, clinical picture and treatment. J Neurosurg 55:174–182PubMedCrossRefGoogle Scholar
  47. 47.
    Rhoton AL Jr (2000) Cerebellum and fourth ventricle. Neurosurgery 47([Suppl]):7–27CrossRefGoogle Scholar
  48. 48.
    Riva D, Giorgi C (2000) The neurodevelopmental price of survival in children with malignant brain tumours. Childs Nerv Syst 16:751–754PubMedCrossRefGoogle Scholar
  49. 49.
    Robertson PL, Muraszko KM, Holmes EJ, Sposto R, Packer RJ, Gajjar A, Diaz MS, Allen JC (2006) Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: a prospective study by the Children’s Oncology Group. J Neurosurg 105(6 Suppl Pediatrics):444–451PubMedGoogle Scholar
  50. 50.
    Sala F, Talacchi A, Mazza C, Prisco R, Ghimenton C, Bricolo A (1998) Prognostic factors in childhood intracranial ependymomas: the role of age and tumor location. Pediatr Neurosurg 28:135–142PubMedCrossRefGoogle Scholar
  51. 51.
    Siffert J, Poussaint TY, Goumnerova LC, Scott RM, LaValley B, Tarbell NJ, Pomeroy SL (2000) Neurological dysfunction associated with postoperative cerebellar mutism. J Neurooncol 48:75–81PubMedCrossRefGoogle Scholar
  52. 52.
    Smyth MD, Horn BN, Russo C, Berger MS (2000) Intracranial ependymomas of childhood: current management strategies. Pediatr Neurosurg 33:138–150PubMedCrossRefGoogle Scholar
  53. 53.
    Spangnoli D, Tomei G, Ceccarelli G, Grimoldi N, Lanterna A, Bello L, Sinisi MM, Santis AD, Villani RM (2000) Combined treatment of fourth ventricle ependymomas: report of 26 cases. Surg Neurol 54:19–26CrossRefGoogle Scholar
  54. 54.
    Spiegler BJ, Bouffet E, Greenberg ML, Rutka JT, Mabbott DJ (2004) Change in neurocognitive functioning after treatment with cranial radiation in childhood. J Clin Oncol 22:706–713PubMedCrossRefGoogle Scholar
  55. 55.
    Spoudeas H, Kirkham F (2004) Toxicity and late effects. In: Walker DA, Perilongo G, Punt JAG, Taylor RE (eds) Brain and spine tumours of childhood. Arnold, London, pp 433–462Google Scholar
  56. 56.
    Strauss C, Romstock J, Nimsky C, Fahlbusch R (1993) Intraoperative identification of motor areas of the rhomboid fossa using direct stimulation. J Neurosurg 79:393–399PubMedCrossRefGoogle Scholar
  57. 57.
    Strauss C, Romstock J, Fahlbusch R (1999) Pericollicular approaches to the rhomboid fossa. Part II. Neurophysiological basis. J Neurosurg 91:768–775Google Scholar
  58. 58.
    Sutton LN, Goldwein J, Perilongo G, Lang B, Schut L, Rorke L, Packer R (1990–1991) Prognostic factor in childhood ependymomas. Pediatric Neurosurg 16:57–65CrossRefGoogle Scholar
  59. 59.
    Teo C, Nakaji P, Symons P, Tobias V, Cohn R, Smee R (2003) Ependymoma. Childs Nerv Syst 9:270–285CrossRefGoogle Scholar
  60. 60.
    Tomita T (2001) Ependymomas. In: Mclone DG (ed) Pediatric neurosurgery, 4th edn. Saunders, Philadelphia, pp 822–834Google Scholar
  61. 61.
    Tomita T, Rosenblatt SS (1991) Management of hydrocephalus secondary to posterior fossa tumor in childhood. In: Matsumoto S, Tamaki N (eds) Hydrocephalus-pathogenesis and treatment. Springer, New York, pp 306–310Google Scholar
  62. 62.
    Van Callenberg F, De Laar AY, Plets C, Goffin J, Ceasar P (1995) Transient cerebellar mutism after posterior fossa surgery in children. Neurosurgery 37:894–898Google Scholar
  63. 63.
    Van Dongen HR, Catsman-Berrevoets CE, van Mourik M (1994) The syndrome of ‘cerebellar’ mutism and subsequent dysarthria. Neurology 44:2040–2046PubMedGoogle Scholar
  64. 64.
    Winston KR (2001) Surgery for tumors in the posterior cranial fossa. In: Mclone DG (ed) Pediatric neurosurgery, 4th edn. Saunders, Philadelphia, pp 844–854Google Scholar
  65. 65.
    Ziyal IM, Sekhar LN, Salas E (1999) Subtonsillar-transcerebellomedullaty approach to lesions involving the fourth ventricle, the cerebellomedullary fissure and the lateral brainstem. Br J Neurosurg 13:276–284PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Kazuaki Shimoji
    • 1
  • Masakazu Miyajima
    • 2
  • Kostadin Karagiozov
    • 2
  • Kenji Yatomi
    • 2
  • Toshio Matsushima
    • 3
  • Hajime Arai
    • 2
  1. 1.Department of NeurosurgeryJuntendo Nerima HospitalTokyoJapan
  2. 2.Department of NeurosurgeryJuntendo University School of MedicineTokyoJapan
  3. 3.Department of NeurosurgerySaga University School of MedicineSaga CityJapan

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