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Reducing the risk of cerebellar mutism syndrome through surgical guidelines
Posterior fossa syndrome can lead to loss of speech and occurs in patients who have undergone tumor removal in the back of the head (the posterior fossa).
Cerebellar mutism syndrome (CMS), also called posterior fossa syndrome, is a surgical consequence that can occur in patients who have undergone tumor removal in the back of the head (the posterior fossa). The hallmark of CMS is a reduction or absence of speech, but it can also lead to emotional instability, movement impairments, and learning and development difficulties. The syndrome has a spectrum from mild to very severe, with neurologic recovery dependent on how much the child is affected.
Surgeons play a pivotal role in negating CMS but have lacked clear guidance on the condition. To address this, clinicians at St. Jude, as members of the Posterior Fossa Society, put forward a consensus statement on reducing the risk of CMS. The statement, published in the Journal of Neurosurgery-Pediatrics, calls for more comprehensive planning and imaging to identify risk levels before surgery, more onus on matching surgical complexity with surgeon experience and careful use or avoidance of certain surgical instruments, including bipolar cautery and brain retraction systems.
“Even in the best hands, there will always be cases of CMS. We want to eliminate the severe cases and keep milder case rates as low as possible — ideally less than 10%,” said corresponding author Paul Klimo Jr., MD, St. Jude Department of Surgery, Le Bonheur Children’s Hospital and Semmes Murphey Clinic. “We want to give those who do develop CMS the best chance at full or near-full recovery.”
“The symptoms of severe CMS are devastating, and even though they improve with time, they never completely resolve,” said co-author Giles Robinson, MD, St. Jude Division of Neuro-oncology director, Department of Oncology. “As a result, children suffer a lifetime of speech, movement, and cognitive disabilities. This statement comprehensively outlines steps that surgeons can take to drastically lower the rates and reduce the severity. It is a must-read for all providers who take care of kids with brain tumors.”