QOL-12. LESSONS LEARNED FROM LONGITUDINAL CARE IN POSTERIOR FOSSA SYNDROME

Abstract

Abstract BACKGROUND Posterior fossa syndrome (PFS) is a constellation of neurobehavioral symptoms occurring in 8–24% of children following surgical resection of posterior fossa tumors. Symptoms may include a combination of mutism or reduced verbal output, emotional lability, hypotonia, dysphagia, ataxia and weakness. Direct surgical injury to the dentato-thalamo-cortical pathway has been the proposed pathological mechanism. The trajectory of recovery and the functional outcomes of PFS are not well described. Khan et al. published a prospective study on children after medulloblastoma resection where they divided PFS into type 1 (complete mutism) and type 2 (diminished speech). They showed that PFS type 1 patients had a worse prognosis and a delayed recovery with 44.4% of children being non-ambulatory at one year. Ricci et al. recently published a more detailed severity grading algorithm which over time will hopefully help with data collection and analysis leading to improved prognostication regarding functional recovery. METHODS We present a unique longitudinal look (range 12 months to 19 years) at 5 pediatric patients with posterior fossa tumors who developed PFS following their tumor resections. RESULTS Tumor diagnoses included ependymoma and medulloblastoma. Rehabilitation interventions consisted of acute inpatient rehabilitation where they received intensive physical, occupational, speech and feeding therapy. Most patients benefited from early utilization of trunk and lower extremity bracing as well as a variety of assistive devices and durable medical equipment. Patients continued their rehabilitation for months to years in an outpatient and school setting. All patients demonstrated significant neurological and functional gains however, none of them had a complete resolution of symptoms. We discuss the anatomy, the time to symptom onset, the trajectory of our patients’ recoveries and the rationale behind the applied rehabilitation strategies. CONCLUSIONS This work gives insights on long term sequalae of PFS and demonstrates current rehabilitation approach for children with this complex condition.