Introduction: The Posterior Fossa Syndrome (PFS) has become a well-recognized clinical entity and may occur in children after resection of cerebellar tumors. The most common feature is mutism, but also oropharyngeal dyspraxia, emotional lability and neuropsychiatric symptoms occur. Studies on the long-term outcome of deficits after treatment of cerebellar tumors describe in long-term motor problems as well as problems with memory, attention, language, speech, visual-spatial, executive functions, and affect regulation in different combinations and to different degrees. These deficits fit in the spectrum of the cerebellar cognitive affective syndrome (CCAS). Until now, the consequences of PFS on long term outcome has not been systematically addressed. The purpose of the present study was to study the effect on long term neurologic and cognitive outcome.

Methods: After resection of a cerebellar tumor, children were prospectively followed according to protocol with neurological assessments, neuropsychological investigations and MRI.  Of  31 children with PFS (out of 144 children with cerebellar tumor resection between 1994 and 2012) neurologic, neuropsychological and functional outcome at 1, 2 and 5 years follow up was compared with that of children without  PFS after cerebellar tumor resection.

Results: Children with PFS after cerebellar surgery had significantly more neurological deficits, lower IQ and lower scores on a modified Rankin scale  at all measure points.

Conclusion: MSD and PFS after  surgery are important risk factors for poor long term functional outcome in children that are treated for a cerebellar tumor.