DOI QR코드

DOI QR Code

Chiari Type III : Experience of Outcome for 15 Cases

  • Gabr, Mohamed (Department of Neurosurgery, Kasr Alainy University Hospital) ;
  • Elmataeshy, Mahmoud (Department of Neurosurgery, Kasr Alainy University Hospital) ;
  • Abdullah, Ahmed A. (Department of Neurosurgery, Kasr Alainy University Hospital)
  • Received : 2021.09.25
  • Accepted : 2022.02.21
  • Published : 2022.11.01

Abstract

Objective : Chiari III malformations are extremely rare hindbrain malformations that are associated with a high early mortality rate, or severe neurologic deficits in the survivors. The treatment is early operative closure and cerebrospinal fluid diversion (CSF) shunting. Methods : We operated on 15 patients by repair and excision between July 2014 till June 2020 and retrospective data collection was done. Only one patient doesn't need ventriculoperitoneal (VP) shunt and the other 14 patients need a VP shunt. We described stepwise dissection and untethering of the cerebellum from the bony edge to regrowth and herniation of cerebellum again into this potential space and simple dural closure or repair with graft was done. Results : We started with VP in eight patients (53%) and the other seven patients (46.7%) started with excision and then six patients need VP shunt later on because four patients developed CSF leak and two patients developed increased high intracranial tension. Only four patients (26.7%) needed a blood transfusion. Conclusion : There are variations in the outcome and not all cases of Chiari malformation III will die and severe developmental delay is not a must. Proper management and repair carry a good prognosis.

Keywords

References

  1. Aleksic S, Budzilovich G, Greco MA, Reuben R, Feigin I, Pearson J, et al. : Cerebellocele and associated central nervous system anomalies in the meckel syndrome. Childs Brain 11 : 99-111, 1984
  2. Aribal ME, Gurcan F, Aslan B : Chiari III malformation: MRI. Neuroradiology 38 : S184-S186, 1996 https://doi.org/10.1007/BF02278154
  3. Caldarelli M, Rea G, Cincu R, Di Rocco C : Chiari type III malformation. Childs Nervous System 18 : 207-210, 2002 https://doi.org/10.1007/s00381-002-0579-y
  4. Cama A, Tortori-Donati P, Piatelli GL, Fondelli MP, Andreussi L : Chiari complex in children--neuroradiological diagnosis, neurosurgical treatment and proposal of a new classification (312 cases). Eur J Pediatr Surg 5 Suppl 1 : 35-38, 1995 https://doi.org/10.1055/s-2008-1066261
  5. Castillo M, Quencer RM, Dominguez R : Chiari III malformation: imaging features. AJNR Am J Neuroradiol 13 : 107-113, 1992
  6. Chiari H : Ueber veranderungen des kleinhirns infolge von hydrocephalie des grosshirns. Dtsch Med Wochenschr 17 : 1172-1175, 1891 https://doi.org/10.1055/s-0029-1206803
  7. Haberle J, Hulskamp G, Harms E, Krasemann T : Cervical encephalocele in a newborn--Chiari III malformation. case report and review of the literature. Childs Nerv Syst 17 : 373-375, 2001 https://doi.org/10.1007/s003810000434
  8. Isik N, Elmaci I, Silav G, Celik M, Kalelioglu M : Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature. Pediatr Neurosurg 45 : 19-28, 2009 https://doi.org/10.1159/000202620
  9. Ivashchuk G, Loukas M, Blount JP, Tubbs RS, Oakes WJ : Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst 31 : 2035-2040, 2015 https://doi.org/10.1007/s00381-015-2853-9
  10. Jaggi RS, Premsagar IC : Chiari malformation type III treated with primary closure. Pediatr Neurosurg 43 : 424-427, 2007 https://doi.org/10.1159/000106397
  11. Kannegieter LS, Dietrich RB, Pais MJ, Goldenberg TM : Pediatric case of the day. Chiari III malformation. Radiographics 14 : 452-454, 1994 https://doi.org/10.1148/radiographics.14.2.8190967
  12. Kriss VM, Kriss TC, Desai NS, Warf BC : Occult spinal dysraphism in the infant. Clin Pediatr (Phila) 34 : 650-654, 1995 https://doi.org/10.1177/000992289503401205
  13. Lee R, Tai KS, Cheng PW, Lui WM, Chan FL : Chiari III malformation: antenatal MRI diagnosis. Clin Radiol 57 : 759-761, 2002 https://doi.org/10.1053/crad.2001.0960
  14. MacLellan DL, Bauer SB : Neuropathic dysfunction of the lower urinary tract : Campbell-Walsh Urology. Elsevier, 2012, pp3431-3456.e8
  15. Maroun FB : Syringomyelia and the chiari malformations. Can J Neurol Sci 25 : 175, 1998 https://doi.org/10.1017/S0317167100033898
  16. Menezes AH, Smoker WRK, Dyste GN : Syringomyelia, chiarimalformations, and hydromyelia in Youmans JR (ed) : Neuro-logical Surgery. Philadelphia : W. B. Saunders Co., 1990, pp1421-1459
  17. Pittman HW : The Chiari crisis. BNI Quart 6 : 10-16, 1990
  18. Sirikci A, Bayazit YA, Bayram M : The Chiari III malformation: an unusual and asymptomatic variant in an 11-year old child. Eur J Radiol 39 : 147-150, 2001 https://doi.org/10.1016/S0720-048X(01)00334-5
  19. Snyder WE Jr, Luerssen TG, Boaz JC, Kalsbeck JE : Chiari III malformation treated with CSF diversion and delayed surgical closure. Pediatr Neurosurg 29 : 117-120, 1998 https://doi.org/10.1159/000028704
  20. Tubb RS, Pugh A, Oakes WJ : Chiari Malformations, 6th ed. Elsevier Inc., 1918
  21. Young RM, Shafa JS, Myseros JS : The Chiari 3 malformation and a systemic review of the l-terature. Pediatr Neurosurg 50 : 235-242, 2015 https://doi.org/10.1159/000438487