Postoperative posterior fossa syndrome: unraveling the etiology and underlying pathophysiology by using magnetic resonance imaging.

BACKGROUND: Posterior fossa syndrome (PFS) is a severe, postoperative complication occurring in about 25 % of pediatric patients undergoing surgery for midline tumors in the posterior fossa. In recent years, MRI-based research elucidated the cause of PFS and shed new light on its putative pathophysiology. This review highlights the major advances arising from research of this topic.

RESULTS: Postsurgical damage patterns in patients after posterior fossa surgery show that PFS results from bilateral surgical damage to the proximal efferent cerebellar pathways (pECPs). Surgical pECP disruption has other MRI-detectable effects that are more remote. Patients with PFS tend to develop global supratentorial cortical hypoperfusion, likely representing reversed, cerebello-cerebral diaschisis. Because this hypoperfusion is most prominent in frontal regions, cerebellar mutism may indicate a dominantly frontal lobe dysfunction, hence a peculiar form of speech apraxia. Injury to the pECP also leads to contralateral inferior olivary nucleus degeneration. When bilateral hypertrophic olivary degeneration (HOD) is observed after posterior fossa surgery, affected patients have clinical PFS. Therefore, it is suggested that bilateral HOD may be a sensitive and, in appropriate clinical settings, reliable a posteriori surrogate imaging indicator of bilateral disruption of the pECPs and consequently of PFS. Having such a "validation tool" presents new opportunities to develop better definitions for the phenotypes within the clinical spectrum of PFS.

CONCLUSIONS: Anatomical and functional MRI techniques are suitable and valuable tools with which to detect structural changes and pathophysiological processes in the development and evolution of PFS and may be key, integral components of future clinical research endeavors.