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Posterior Fossa Syndrome

MedGen UID:
853820
•Concept ID:
C2721675
•
Pathologic Function

Definition

A postoperative syndrome, usually presenting after midline posterior fossa tumor resection, that involves a variety of signs and symptoms including aphasia, mutism or speech disturbances, dysphagia, mobility problems, cranial nerve palsies and emotional instability. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPosterior Fossa Syndrome

Professional guidelines

PubMed

Neurological Complications of the Treatment of Pediatric Neoplastic Disorders.
Sun LR, Cooper S
Pediatr Neurol 2018 Aug;85:33-42. Epub 2018 Jun 2 doi: 10.1016/j.pediatrneurol.2018.05.011. PMID: 30126755
Examination of risk factors for intellectual and academic outcomes following treatment for pediatric medulloblastoma.
Schreiber JE, Gurney JG, Palmer SL, Bass JK, Wang M, Chen S, Zhang H, Swain M, Chapieski ML, Bonner MJ, Mabbott DJ, Knight SJ, Armstrong CL, Boyle R, Gajjar A
Neuro Oncol 2014 Aug;16(8):1129-36. Epub 2014 Feb 3 doi: 10.1093/neuonc/nou006. PMID: 24497405Free PMC Article
Parent-reported social outcomes after treatment for pediatric embryonal tumors: a prospective longitudinal study.
Brinkman TM, Palmer SL, Chen S, Zhang H, Evankovich K, Swain MA, Bonner MJ, Janzen L, Knight S, Armstrong CL, Boyle R, Gajjar A
J Clin Oncol 2012 Nov 20;30(33):4134-40. Epub 2012 Oct 15 doi: 10.1200/JCO.2011.40.6702. PMID: 23071220Free PMC Article

Recent clinical studies

Etiology

The posterior fossa syndrome questionnaire: using science to inform practice.
Wickenhauser ME, Khan RB, Raches D, Ashford JM, Russell KMW, Lyons K, Robinson GW, Gajjar A, Klimo P Jr, Conklin HM
J Neurooncol 2022 May;157(3):465-473. Epub 2022 Apr 5 doi: 10.1007/s11060-022-03990-0. PMID: 35380295Free PMC Article
Association between cerebral perfusion and paediatric postoperative cerebellar mutism syndrome after posterior fossa surgery-a systematic review.
Ahmadian N, van Baarsen KM, Robe PAJT, Hoving EW
Childs Nerv Syst 2021 Sep;37(9):2743-2751. Epub 2021 Jun 21 doi: 10.1007/s00381-021-05225-5. PMID: 34155533Free PMC Article
Cerebellar Mutism Syndrome after surgical resection of posterior fossa neoplastic lesions.
Bakhshi SK, Mitha R, Mushtaq N, Shamim MS
J Pak Med Assoc 2020 Sep;70(9):1667-1668. PMID: 33040137
Radiology of post-operative paediatric cerebellar mutism syndrome.
Avula S
Childs Nerv Syst 2020 Jun;36(6):1187-1195. Epub 2019 Jun 11 doi: 10.1007/s00381-019-04224-x. PMID: 31183530
Posterior fossa syndrome.
Pollack IF
Int Rev Neurobiol 1997;41:411-32. doi: 10.1016/s0074-7742(08)60362-1. PMID: 9378600

Diagnosis

The posterior fossa syndrome questionnaire: using science to inform practice.
Wickenhauser ME, Khan RB, Raches D, Ashford JM, Russell KMW, Lyons K, Robinson GW, Gajjar A, Klimo P Jr, Conklin HM
J Neurooncol 2022 May;157(3):465-473. Epub 2022 Apr 5 doi: 10.1007/s11060-022-03990-0. PMID: 35380295Free PMC Article
Characterizing Posterior Fossa Syndrome: A Survey of Experts.
Wickenhauser ME, Khan RB, Raches D, Ashford JM, Robinson GW, Russell KM, Conklin HM
Pediatr Neurol 2020 Mar;104:19-22. Epub 2019 Nov 29 doi: 10.1016/j.pediatrneurol.2019.11.007. PMID: 31911026Free PMC Article
Postoperative posterior fossa syndrome: unraveling the etiology and underlying pathophysiology by using magnetic resonance imaging.
Patay Z
Childs Nerv Syst 2015 Oct;31(10):1853-8. Epub 2015 Jul 5 doi: 10.1007/s00381-015-2796-1. PMID: 26143277
Cerebellar mutism.
Küper M, Timmann D
Brain Lang 2013 Dec;127(3):327-33. Epub 2013 Feb 9 doi: 10.1016/j.bandl.2013.01.001. PMID: 23398780
Posterior fossa syndrome.
Pollack IF
Int Rev Neurobiol 1997;41:411-32. doi: 10.1016/s0074-7742(08)60362-1. PMID: 9378600

Therapy

The posterior fossa syndrome questionnaire: using science to inform practice.
Wickenhauser ME, Khan RB, Raches D, Ashford JM, Russell KMW, Lyons K, Robinson GW, Gajjar A, Klimo P Jr, Conklin HM
J Neurooncol 2022 May;157(3):465-473. Epub 2022 Apr 5 doi: 10.1007/s11060-022-03990-0. PMID: 35380295Free PMC Article
Cerebellar mutism syndrome in children with brain tumours of the posterior fossa.
Wibroe M, Cappelen J, Castor C, Clausen N, Grillner P, Gudrunardottir T, Gupta R, Gustavsson B, Heyman M, Holm S, Karppinen A, Klausen C, Lönnqvist T, Mathiasen R, Nilsson P, Nysom K, Persson K, Rask O, Schmiegelow K, Sehested A, Thomassen H, Tonning-Olsson I, Zetterqvist B, Juhler M
BMC Cancer 2017 Jun 21;17(1):439. doi: 10.1186/s12885-017-3416-0. PMID: 28637445Free PMC Article
Posterior fossa syndrome following brain tumour resection: review of pathophysiology and a new hypothesis on its pathogenesis.
Avula S, Mallucci C, Kumar R, Pizer B
Childs Nerv Syst 2015 Oct;31(10):1859-67. Epub 2015 Sep 9 doi: 10.1007/s00381-015-2797-0. PMID: 26351235
Posterior fossa syndrome after posterior fossa surgery in children with brain tumors.
Küpeli S, Yalçın B, Bilginer B, Akalan N, Haksal P, Büyükpamukçu M
Pediatr Blood Cancer 2011 Feb;56(2):206-10. Epub 2010 Oct 25 doi: 10.1002/pbc.22730. PMID: 21157890
Cerebellar mutism: review of the literature.
Gudrunardottir T, Sehested A, Juhler M, Schmiegelow K
Childs Nerv Syst 2011 Mar;27(3):355-63. doi: 10.1007/s00381-010-1328-2. PMID: 21061011

Prognosis

Diffusion Tensor Imaging Technique Delineating the Prognosis for Cerebellar Mutism in Posterior Fossa Tumors: A New Tool.
Sinha VD, Ankur P, Gaurav J
Acta Neurochir Suppl 2023;135:53-59. doi: 10.1007/978-3-031-36084-8_10. PMID: 38153449
The posterior fossa syndrome questionnaire: using science to inform practice.
Wickenhauser ME, Khan RB, Raches D, Ashford JM, Russell KMW, Lyons K, Robinson GW, Gajjar A, Klimo P Jr, Conklin HM
J Neurooncol 2022 May;157(3):465-473. Epub 2022 Apr 5 doi: 10.1007/s11060-022-03990-0. PMID: 35380295Free PMC Article
Quantitative MRI in post-operative paediatric cerebellar mutism syndrome.
Toescu SM, Hales PW, Aquilina K, Clark CA
Eur J Radiol 2018 Nov;108:43-51. Epub 2018 Sep 10 doi: 10.1016/j.ejrad.2018.09.007. PMID: 30396670
Cerebellar disorders: clinical/radiologic findings and modern imaging tools.
Manto M, Habas C
Handb Clin Neurol 2016;135:479-491. doi: 10.1016/B978-0-444-53485-9.00023-4. PMID: 27432679
Cerebellar mutism: review of the literature.
Gudrunardottir T, Sehested A, Juhler M, Schmiegelow K
Childs Nerv Syst 2011 Mar;27(3):355-63. doi: 10.1007/s00381-010-1328-2. PMID: 21061011

Clinical prediction guides

The posterior fossa syndrome questionnaire: using science to inform practice.
Wickenhauser ME, Khan RB, Raches D, Ashford JM, Russell KMW, Lyons K, Robinson GW, Gajjar A, Klimo P Jr, Conklin HM
J Neurooncol 2022 May;157(3):465-473. Epub 2022 Apr 5 doi: 10.1007/s11060-022-03990-0. PMID: 35380295Free PMC Article
Telovelar surgical approach.
Ghali MGZ
Neurosurg Rev 2021 Feb;44(1):61-76. Epub 2019 Dec 5 doi: 10.1007/s10143-019-01190-5. PMID: 31807931
Quantitative MRI in post-operative paediatric cerebellar mutism syndrome.
Toescu SM, Hales PW, Aquilina K, Clark CA
Eur J Radiol 2018 Nov;108:43-51. Epub 2018 Sep 10 doi: 10.1016/j.ejrad.2018.09.007. PMID: 30396670
Postoperative posterior fossa syndrome: unraveling the etiology and underlying pathophysiology by using magnetic resonance imaging.
Patay Z
Childs Nerv Syst 2015 Oct;31(10):1853-8. Epub 2015 Jul 5 doi: 10.1007/s00381-015-2796-1. PMID: 26143277
Posterior fossa syndrome.
Pollack IF
Int Rev Neurobiol 1997;41:411-32. doi: 10.1016/s0074-7742(08)60362-1. PMID: 9378600

Recent systematic reviews

Risk factors for postoperative cerebellar mutism syndrome in pediatric patients: a systematic review and meta-analysis.
Pettersson SD, Kitlinski M, Miękisiak G, Ali S, Krakowiak M, Szmuda T
J Neurosurg Pediatr 2022 Apr 1;29(4):467-475. Epub 2021 Dec 31 doi: 10.3171/2021.11.PEDS21445. PMID: 34972081
Association between cerebral perfusion and paediatric postoperative cerebellar mutism syndrome after posterior fossa surgery-a systematic review.
Ahmadian N, van Baarsen KM, Robe PAJT, Hoving EW
Childs Nerv Syst 2021 Sep;37(9):2743-2751. Epub 2021 Jun 21 doi: 10.1007/s00381-021-05225-5. PMID: 34155533Free PMC Article
Neuropsychiatric presentations and outcomes in children and adolescents with primary brain tumours: Systematic review.
Zyrianova Y, Alexander L, Faruqui R
Brain Inj 2016;30(1):1-9. Epub 2015 Nov 5 doi: 10.3109/02699052.2015.1075590. PMID: 26536821

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