Abstract
Purpose
Cerebellar mutism syndrome (CMS) is a serious source of morbidity following posterior fossa surgery in the pediatric population. However, methods for effectively decreasing its incidence and impact remain unclear. It is our aim to examine the impact of adjusting surgical factors, namely the use of a telovelar approach and avoidance of cavitronic ultrasonic aspirator, on the incidence of CMS in our population as well as outlining potential pre-, intra-, and postoperative factors that may contribute to its development.
Methods
Retrospective review was performed to identify patients undergoing posterior fossa surgery for resection of a medulloblastoma. Demographic, surgical, and postoperative data were collected. These data were analyzed for possible correlations to the risk of developing CMS via univariate analysis. For factors found to be significant, a multivariate analysis was performed to assess their independence.
Results
Seven of 65 patients (10.8%) developed CMS postoperatively. Factors found to be significantly associated with a higher risk of CMS were the degree of retraction utilized during the procedure (p = 0.0000) and incision of the vermis (p = 0.0294). Although they did not reach the threshold of statistical significance, tumor vascularity (p = 0.19), adoption of a transvermian approach (p = 0.19), and lack of intraoperative imaging (p = 0.17) exhibited strongly suggestive trends towards a correlation with CMS.
Discussion
In an effort to reduce the incidence and severity of CMS in our population, our institution adopted surgical practices that minimize tissue trauma and mitigate postoperative edema. This included the use of a telovelar over a transvermian approach to obviate the need for vermian incision, avoidance of the CUSA, and minimization of heavy retraction during surgery. This was successful in reducing the incidence of CMS from 39% in our medulloblastoma patients to 10.8%. The development of CMS after posterior fossa surgery appears to be a “two-hit” phenomenon requiring a combination of existing predisposition, surgical injury, and postoperative exacerbation. Therefore, it is critical to identify the factors involved at each stage and investigate treatments to target them appropriately.
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References
Rekate HL, Grubb RL, Aram DM, Hahn JF, Ratcheson RA (1985) Muteness of cerebellar origin. Arch Neurol 42:697–698. https://doi.org/10.1001/archneur.1985.04060070091023
Fraioli B, null G (1975) Effects of stereotactic lesions of the dentate nucleus of the cerebellum in man. Appl Neurophysiol 38:81–90
Wisoff JH, Epstein FJ (1984) Pseudobulbar palsy after posterior fossa operation in children. Neurosurgery 15:707–709
Robertson PL, Muraszko KM, Holmes EJ, Sposto R, Packer RJ, Gajjar A, Dias 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 105:444–451. https://doi.org/10.3171/ped.2006.105.6.444
Fujisawa H, Yonaha H, Okumoto K, Uehara H, Ie T, Nagata Y, Suehiro E, Suzuki M (2005) Mutism after evacuation of acute subdural hematoma of the posterior fossa. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 21:234–236. https://doi.org/10.1007/s00381-004-0999-y
Al-Anazi A, Hassounah M, Sheikh B, Barayan S (2001) Cerebellar mutism caused by arteriovenous malformation of the vermis. Br J Neurosurg 15:47–50
Baillieux H, Weyns F, Paquier P, de Deyn PP, Mariën P (2007) Posterior fossa syndrome after a vermian stroke: a new case and review of the literature. Pediatr Neurosurg 43:386–395. https://doi.org/10.1159/000106388
Papavasiliou AS, Kotsalis C, Trakadas S (2004) Transient cerebellar mutism in the course of acute cerebellitis. Pediatr Neurol 30:71–74
Coplin WM, Kim DK, Kliot M, Bird TD (1997) Mutism in an adult following hypertensive cerebellar hemorrhage: nosological discussion and illustrative case. Brain Lang 59:473–493. https://doi.org/10.1006/brln.1997.1790
Dunwoody GW, Alsagoff ZS, Yuan SY (1997) Cerebellar mutism with subsequent dysarthria in an adult: case report. Br J Neurosurg 11:161–163
Mariën P, De Smet HJ, Wijgerde E et al (2013) Posterior fossa syndrome in adults: a new case and comprehensive survey of the literature. Cortex 49:284–300. https://doi.org/10.1016/j.cortex.2011.06.018
De Smet HJ, Baillieux H, Catsman-Berrevoets C et al (2007) Postoperative motor speech production in children with the syndrome of “cerebellar” mutism and subsequent dysarthria: a critical review of the literature. Eur J Paediatr Neurol EJPN Off J Eur Paediatr Neurol Soc 11:193–207. https://doi.org/10.1016/j.ejpn.2007.01.007
Gudrunardottir T, Sehested A, Juhler M, Schmiegelow K (2011) Cerebellar mutism: review of the literature. Childs Nerv Syst 27:355–363. https://doi.org/10.1007/s00381-010-1328-2
Morris EB, Phillips NS, Laningham FH, Patay Z, Gajjar A, Wallace D, Boop F, Sanford R, Ness KK, Ogg RJ (2009) Proximal dentatothalamocortical tract involvement in posterior fossa syndrome. Brain 132:3087–3095. https://doi.org/10.1093/brain/awp241
Küper M, Timmann D (2013) Cerebellar mutism. Brain Lang 127:327–333. https://doi.org/10.1016/j.bandl.2013.01.001
Di Rocco C, Chieffo D, Frassanito P, Caldarelli M, Massimi L, Tamburrini G (2011) Heralding cerebellar mutism: evidence for pre-surgical language impairment as primary risk factor in posterior fossa surgery. Cerebellum 10:551–562. https://doi.org/10.1007/s12311-011-0273-2
Levisohn L, Cronin-Golomb A, Schmahmann JD (2000) Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population. Brain J Neurol 123(Pt 5):1041–1050
McEvoy SD, Lee A, Poliakov A et al (2016) Longitudinal cerebellar diffusion tensor imaging changes in posterior fossa syndrome. NeuroImage Clin 12:582–590. https://doi.org/10.1016/j.nicl.2016.09.007
Oh ME, Driever PH, Khajuria RK, Rueckriegel SM, Koustenis E, Bruhn H, Thomale UW (2017) DTI fiber tractography of cerebro-cerebellar pathways and clinical evaluation of ataxia in childhood posterior fossa tumor survivors. J Neuro-Oncol 131:267–276. https://doi.org/10.1007/s11060-016-2290-y
Chua FHZ, Thien A, Ng LP, Seow WT, Low DCY, Chang KTE, Lian DWQ, Loh E, Low SYY (2017) Post-operative diffusion weighted imaging as a predictor of posterior fossa syndrome permanence in paediatric medulloblastoma. Childs Nerv Syst 33:457–465. https://doi.org/10.1007/s00381-017-3356-7
Ojemann JG, Partridge SC, Poliakov AV, Niazi TN, Shaw DW, Ishak GE, Lee A, Browd SR, Geyer JR, Ellenbogen RG (2013) Diffusion tensor imaging of the superior cerebellar peduncle identifies patients with posterior fossa syndrome. Childs Nerv Syst 29:2071–2077. https://doi.org/10.1007/s00381-013-2205-6
Catsman-Berrevoets CE, Van Dongen HR, Mulder PG et al (1999) Tumour type and size are high risk factors for the syndrome of “cerebellar” mutism and subsequent dysarthria. J Neurol Neurosurg Psychiatry 67:755–757
Pols SYCV, van Veelen MLC, Aarsen FK, Gonzalez Candel A, Catsman-Berrevoets CE (2017) Risk factors for development of postoperative cerebellar mutism syndrome in children after medulloblastoma surgery. J Neurosurg Pediatr 20:35–41. https://doi.org/10.3171/2017.2.PEDS16605
Liu J-F, Dineen RA, Avula S, Chambers T, Dutta M, Jaspan T, MacArthur DC, Howarth S, Soria D, Quinlan P, Harave S, Ong CC, Mallucci CL, Kumar R, Pizer B, Walker DA (2018) Development of a pre-operative scoring system for predicting risk of post-operative paediatric cerebellar mutism syndrome. Br J Neurosurg 32:18–27. https://doi.org/10.1080/02688697.2018.1431204
Kellogg JX, Piatt JH (1997) Resection of fourth ventricle tumors without splitting the vermis: the cerebellomedullary fissure approach. Pediatr Neurosurg 27:28–33. https://doi.org/10.1159/000121221
El-Bahy K (2005) Telovelar approach to the fourth ventricle: operative findings and results in 16 cases. Acta Neurochir 147:137–142; discussion 142. https://doi.org/10.1007/s00701-004-0407-0
Tomasello F, Conti A, Cardali S, la Torre D, Angileri FF (2015) Telovelar approach to fourth ventricle tumors: highlights and limitations. World Neurosurg 83:1141–1147. https://doi.org/10.1016/j.wneu.2015.01.039
Zaheer SN, Wood M (2010) Experiences with the telovelar approach to fourth ventricular tumors in children. Pediatr Neurosurg 46:340–343. https://doi.org/10.1159/000321539
Pollack IF, Polinko P, Albright LA, Towbin R, Fitz C (1995) Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children. Neurosurgery 37:885–892. https://doi.org/10.1227/00006123-199511000-00006
Avula S, Mallucci C, Kumar R, Pizer B (2015) Posterior fossa syndrome following brain tumour resection: review of pathophysiology and a new hypothesis on its pathogenesis. Childs Nerv Syst 31:1859–1867. https://doi.org/10.1007/s00381-015-2797-0
Korah MP, Esiashvili N, Mazewski CM, Hudgins RJ, Tighiouart M, Janss AJ, Schwaibold FP, Crocker IR, Curran WJ Jr, Marcus RB Jr (2010) Incidence, risks, and sequelae of posterior fossa syndrome in pediatric medulloblastoma. Int J Radiat Oncol Biol Phys 77:106–112. https://doi.org/10.1016/j.ijrobp.2009.04.058
Gudrunardottir T, Lannering B, Remke M, Taylor MD, Wells EM, Keating RF, Packer RJ (2014) Treatment developments and the unfolding of the quality of life discussion in childhood medulloblastoma: a review. Childs Nerv Syst 30:979–990. https://doi.org/10.1007/s00381-014-2388-5
Wells EM, Khademian ZP, Walsh KS, Vezina G, Sposto R, Keating RF, Packer RJ (2010) Postoperative cerebellar mutism syndrome following treatment of medulloblastoma: neuroradiographic features and origin. J Neurosurg Pediatr 5:329–334. https://doi.org/10.3171/2009.11.PEDS09131
Doxey D, Bruce D, Sklar F, Swift D, Shapiro K (1999) Posterior fossa syndrome: identifiable risk factors and irreversible complications. Pediatr Neurosurg 31:131–136. https://doi.org/10.1159/000028848
For the Iceland Delphi Group, Gudrunardottir T, Morgan AT et al (2016) Consensus paper on post-operative pediatric cerebellar mutism syndrome: the Iceland Delphi results. Childs Nerv Syst 32:1195–1203. https://doi.org/10.1007/s00381-016-3093-3
van Baarsen KM, Grotenhuis JA (2014) The anatomical substrate of cerebellar mutism. Med Hypotheses 82:774–780. https://doi.org/10.1016/j.mehy.2014.03.023
van Baarsen K, Kleinnijenhuis M, Konert T, van Cappellen van Walsum AM, Grotenhuis A (2013) Tractography demonstrates dentate-rubro-thalamic tract disruption in an adult with cerebellar mutism. Cerebellum 12:617–622. https://doi.org/10.1007/s12311-013-0473-z
Avula S, Kumar R, Pizer B, Pettorini B, Abernethy L, Garlick D, Mallucci C (2015) Diffusion abnormalities on intraoperative magnetic resonance imaging as an early predictor for the risk of posterior fossa syndrome. Neuro-Oncol 17:614–622. https://doi.org/10.1093/neuonc/nou299
Iuvone L, Peruzzi L, Colosimo C, Tamburrini G, Caldarelli M, di Rocco C, Battaglia D, Guzzetta F, Misciagna S, di Giannatale A, Ruggiero A, Riccardi R (2011) Pretreatment neuropsychological deficits in children with brain tumors. Neuro-Oncol 13:517–524. https://doi.org/10.1093/neuonc/nor013
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 (2017) Cerebellar mutism syndrome in children with brain tumours of the posterior fossa. BMC Cancer 17:439. https://doi.org/10.1186/s12885-017-3416-0
Dailey AT, McKhann GM, Berger MS (1995) The pathophysiology of oral pharyngeal apraxia and mutism following posterior fossa tumor resection in children. J Neurosurg 83:467–475. https://doi.org/10.3171/jns.1995.83.3.0467
Deshmukh VR, Figueiredo EG, Deshmukh P et al (2006) Quantification and comparison of telovelar and transvermian approaches to the fourth ventricle. Neurosurgery 58:ONS-202-206; discussion ONS-206-207. https://doi.org/10.1227/01.NEU.0000207373.26614.BF
Senft C, Bink A, Franz K, Vatter H, Gasser T, Seifert V (2011) Intraoperative MRI guidance and extent of resection in glioma surgery: a randomised, controlled trial. Lancet Oncol 12:997–1003. https://doi.org/10.1016/S1470-2045(11)70196-6
Miller NG, Reddick WE, Kocak M, Glass JO, Löbel U, Morris B, Gajjar A, Patay Z (2010) Cerebellocerebral diaschisis is the likely mechanism of postsurgical posterior fossa syndrome in pediatric patients with midline cerebellar tumors. Am J Neuroradiol 31:288–294. https://doi.org/10.3174/ajnr.A1821
Catsman-Berrevoets C, Patay Z (2018) Cerebellar mutism syndrome. In: Handbook of Clinical Neurology, Elsevier, pp 273–288
Psarros T, Zouros A, Coimbra C (2003) Bromocriptine-responsive akinetic mutism following endoscopy for ventricular neurocysticercosis: Case report and review of the literature. J Neurosurg 99:397–401. https://doi.org/10.3171/jns.2003.99.2.0397
Nicita F, Paiano M, Liberatore M, Spalice A, Papoff P, Ullo M, Piccirilli M, Clerico A, Schiavetti A (2017) Sudden benzodiazepine-induced resolution of post-operative pediatric cerebellar mutism syndrome: a clinical-SPECT study. Acta Neurochir 159:475–479. https://doi.org/10.1007/s00701-016-3059-y
Akhaddar A, Salami M, El Asri AC, Boucetta M (2012) Treatment of postoperative cerebellar mutism with fluoxetine. Childs Nerv Syst 28:507–508. https://doi.org/10.1007/s00381-012-1719-7
Shyu C, Burke K, Souweidane MM, Dunkel IJ, Gilheeney SW, Gershon T, Khakoo Y (2011) Novel use of zolpidem in cerebellar mutism syndrome. J Pediatr Hematol Oncol 33:148–149. https://doi.org/10.1097/MPH.0b013e3182053a1a
Walker D, Thomas SA, Talbot EJ et al (2014) Cerebellar mutism: the rehabilitation challenge in pediatric neuro-oncology: case studies. J Pediatr Rehabil Med 7:333–340. https://doi.org/10.3233/PRM-140309
Catsman-Berrevoets CE (2017) Cerebellar mutism syndrome: cause and rehabilitation. Curr Opin Neurol 30:133–139. https://doi.org/10.1097/WCO.0000000000000426
Kabatas S, Yilmaz C, Altinors N, Yildiz O, Agaoglu B (2010) Cerebellar mutism syndrome and its relation to cerebellar cognitive and affective function: review of the literature. Ann Indian Acad Neurol 13:23–27. https://doi.org/10.4103/0972-2327.61272
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Cobourn, K., Marayati, F., Tsering, D. et al. Cerebellar mutism syndrome: current approaches to minimize risk for CMS. Childs Nerv Syst 36, 1171–1179 (2020). https://doi.org/10.1007/s00381-019-04240-x
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DOI: https://doi.org/10.1007/s00381-019-04240-x