Factors predicting “postoperative cerebellar mutism syndrome’’ after posterior fossa tumor excision in pediatric patients

Introduction Cerebellar mutism syndrome (CMS) is a common complication after posterior fossa tumor resection. It is characterized by a significant lack or loss of speech. Its biological origin remains unclear and there are no standardized treatments. However, bromocriptine seems to be a possible treatment for this condition. Case report In this paper, we present three cases of pediatric patients (4, 5, and 17-year old) who developed CMS after posterior fossa tumor surgery. They were treated with bromocriptine to improve neurological symptoms. Management and outcome: Bromocriptine was started at a low dose and was progressively increased to reach the minimum effective dose. After four months of treatment, a normal and fluid speech was observed in the three patients. No discontinuation due to adverse events were reported. Discussion Bromocriptine has shown to be an effective and safe treatment for CMS in pediatric patients after posterior fossa tumor resection.

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Fronto-cerebellar fiber tractography in pediatric patients following posterior fossa tumor surgery

Child s Nervous System ◽

10.1007/s00381-013-2070-3 ◽

2013 ◽

Vol 29 (5) ◽

pp. 715-716

Author(s):

Thora Gudrunardottir ◽

Astrid Sehested ◽

Marianne Juhler ◽

Kjeld Schmiegelow

Keyword(s):

Posterior Fossa ◽

Pediatric Patients ◽

Posterior Fossa Tumor ◽

Tumor Surgery ◽

Fiber Tractography

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Cerebellar mutism after posterior fossa tumor resection in children: a multicenter international retrospective study to determine possible modifiable factors

Child s Nervous System ◽

10.1007/s00381-019-04058-7 ◽

2019 ◽

Vol 36 (6) ◽

pp. 1159-1169 ◽

Cited By ~ 4

Author(s):

Bryan Renne ◽

Julia Radic ◽

Deepak Agrawal ◽

Brittany Albrecht ◽

Christopher M. Bonfield ◽

...

Keyword(s):

Retrospective Study ◽

Posterior Fossa ◽

Tumor Resection ◽

Posterior Fossa Tumor ◽

Cerebellar Mutism ◽

Modifiable Factors

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The pathophysiology of oral pharyngeal apraxia and mutism following posterior fossa tumor resection in children

Journal of Neurosurgery ◽

10.3171/jns.1995.83.3.0467 ◽

1995 ◽

Vol 83 (3) ◽

pp. 467-475 ◽

Cited By ~ 101

Author(s):

Andrew T. Dailey ◽

Guy M. McKhann ◽

Mitchel S. Berger

Keyword(s):

Posterior Fossa ◽

Pediatric Patients ◽

Tumor Resection ◽

Posterior Fossa Tumor ◽

Posterior Fossa Tumors ◽

Lower Cranial Nerve ◽

Content Type ◽

Cranial Nerve Function ◽

Pharyngeal Musculature ◽

Fine Print

✓ Mutism following posterior fossa tumor resection in pediatric patients has been previously recognized, although its pathophysiology remains unclear. A review of the available literature reveals 33 individuals with this condition, with only a few adults documented in the population. All of these patients had large midline posterior fossa tumors. To better understand the incidence and anatomical substrate of this syndrome, the authors reviewed a 7-year series of 110 children who underwent a posterior fossa tumor resection. During that time, nine (8.2%) of the 110 children exhibited mutism postoperatively. They ranged from 2.5 to 20 years of age (mean 8.1 years) and became mute within 12 to 48 hours of surgery. The period of mutism lasted from 1.5 to 12 weeks after onset: all children had difficulty coordinating their oral pharyngeal musculature as manifested by postoperative drooling and inability to swallow. Further analysis of these cases revealed that all children had splitting of the entire inferior vermis at surgery, as confirmed on postoperative magnetic resonance studies. Lower cranial nerve function was intact in all nine patients. Current concepts of cerebellar physiology emphasize the importance of the cerebellum in learning and language. The syndrome described resembles a loss of learned activities, or an apraxia, of the oral and pharyngeal musculature. To avoid the apraxia, therefore, the inferior vermis must be preserved. For large midline tumors that extend to the aqueduct, a combined approach through the fourth ventricle and a midvermis split may be used to avoid injuring the inferior vermis.

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Fronto-cerebellar fiber tractography in pediatric patients following posterior fossa tumor surgery

Child s Nervous System ◽

10.1007/s00381-012-1973-8 ◽

2012 ◽

Vol 29 (4) ◽

pp. 597-607 ◽

Cited By ~ 41

Author(s):

Verena Soelva ◽

Pablo Hernáiz Driever ◽

Alexander Abbushi ◽

Stefan Rueckriegel ◽

Harald Bruhn ◽

...

Keyword(s):

Posterior Fossa ◽

Pediatric Patients ◽

Posterior Fossa Tumor ◽

Tumor Surgery ◽

Fiber Tractography

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Development and application of a diagnostic and severity scale to grade post-operative pediatric cerebellar mutism syndrome

European Journal of Pediatrics ◽

10.1007/s00431-021-04290-x ◽

2021 ◽

Author(s):

Federica S. Ricci ◽

Rossella D’Alessandro ◽

Alessandra Somà ◽

Anna Salvalaggio ◽

Francesca Rossi ◽

...

Keyword(s):

Posterior Fossa ◽

Tumor Location ◽

Posterior Fossa Tumor ◽

Surgical Removal ◽

Cerebellar Mutism ◽

Severity Scale ◽

Working Definition ◽

Prognostic Stratification ◽

Oropharyngeal Dysfunction

AbstractThe post-operative pediatric cerebellar mutism syndrome (CMS) affects about one-third of children and adolescents following surgical removal of a posterior fossa tumor (PFT). According to the Posterior Fossa Society consensus working definition, CMS is characterized by delayed-onset mutism/reduced speech and emotional lability after cerebellar or 4th ventricle tumor surgery in children, and is frequently accompanied by additional features such as hypotonia and oropharyngeal dysfunction/dysphagia. The main objective of this work was to develop a diagnostic scale to grade CMS duration and severity. Thirty consecutively referred subjects, aged 1–17 years (median 8 years, IQR 3–10), were evaluated with the proposed Post-Operative Pediatric CMS Survey after surgical resection of a PFT and, in case of CMS, for 30 days after the onset (T0) or until symptom remission. At day 30 (T1), CMS was classified into mild, moderate, or severe according to the proposed scale. CMS occurred in 13 patients (43%, 95% C.I.: 25.5–62.6%), with mild severity in 4 cases (31%), moderate in 4 (31%), and severe in 5 (38%). At T1, longer symptom persistence was associated with greater severity (p = 0.01). Greater severity at T0 predicted greater severity at T1 (p = 0.0001). Children with a midline tumor location and those aged under 5 years at diagnosis were at higher risk of CMS (p = 0.025 and p = 0.008, respectively). In conclusion, the proposed scale is a simple and applicable tool for estimating the severity of CMS at its onset, monitoring its course over time, and providing an early prognostic stratification to guide treatment decisions. What is Known:• Post-operative pediatric Cerebellar Mutism Syndrome (CMS) is a complex phenomenon with a wide spectrum of symptoms that may manifest in children undergoing the resection of a posterior fossa tumor (PFT) and that can result into long-term impairment. What is New:• This study developed and pilot-tested an easily applicable diagnostic and severity scale to grade the duration and the severity of symptoms of the CMS.• The proposed scale was found to be a sensitive instrument to identify even mild CMS presentations.• By scoring not only the duration but also the severity of symptoms the scale allows a more accurate prognostic stratification for an optimal planning of clinical and rehabilitative interventions.

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