Description of Posterior Fossa Syndrome in Children After Posterior Fossa Brain Tumor Surgery

Abstract

Brain tumors are the second most common malignancy in children less than 15 years of age and the most common solid tumor of childhood. Approximately 60% to 70% of pediatric brain tumors originate in the posterior fossa. Since 1989, the two hospitals that comprise the setting for this study have treated 121 children with posterior fossa brain tumors. A postoperative syndrome, labeled posterior fossa syndrome, has been identified in certain children. This syndrome involves a variety of signs and symptoms including mutism or speech disturbances, dysphagia, decreased motor movement, cranial nerve palsies and, emotional lability. These signs and symptoms develop from an average range of 24 to 107 hours after surgery and may take weeks to months to resolve. The exact etiology of the syndrome is unknown. The purpose of this retrospective medical records review of 19 children diagnosed with posterior fossa syndrome is to describe the symptoms of the syndrome. Early recognition of this syndrome could facilitate preventive and restorative patient care, prevent subsequent complications, decrease length of hospital stays, and promote patient and family understanding of and coping with the syndrome.

Clinical Case Study

“…Furthermore, direct structural damage from the tumor can cause problems in the cerebellum, brainstem, and surrounding regions. Children can thus present with nausea, vomiting, ataxia, diplopia, nystagmus, and other cranial nerve deficits (Kirk et al, 1995;Stavrou et al, 2001).…”

“…To put this in perspective, there are approximately 1,200 new central nervous system (CNS) tumors in children in the United States each year. Approximately 60% to 70% of pediatric CNS tumors are located in the posterior fossa, and medulloblastoma accounts for 40% to 50% of posterior fossa tumors (Kirk, Howard, & Scott, 1995).…”

Longitudinal MRI assessment: the identification of relevant features in the development of Posterior Fossa Syndrome in children

“…2 The development of such a syndrome in children hinders their development and highly impacts their quality of life. It is therefore of great importance to identify biomarkers predictive of the onset of this syndrome in order to enhance clinician decision-making over treatment and prognosis, as well as to understand its underlying causes.…”

Identifying quantitative imaging features of posterior fossa syndrome in longitudinal MRI

“…1 This syndrome, also known as cerebellar mutism syndrome, describes a set of neurological symptoms that may develop from 24 to 107 h after surgery. 2,3 Children suffering from PFS characteristically suffer from disturbance in speech and mutism but may also suffer from loss of muscle tone, incontinence, strabismus (crosseyed), dysphagia, and personality changes such as anger, apathy, melancholy, crying, and screaming. 2 The development of such a syndrome in children hinders their development and highly impacts their quality of life.…”

“…2,3 Children suffering from PFS characteristically suffer from disturbance in speech and mutism but may also suffer from loss of muscle tone, incontinence, strabismus (crosseyed), dysphagia, and personality changes such as anger, apathy, melancholy, crying, and screaming. 2 The development of such a syndrome in children hinders their development and highly impacts their quality of life. Although PFS is a postsurgical complication, the exact underlying pathophysiological mechanism remains unclear, although it is widely considered to involve disruption of the proximal efferent cerebellar pathways (pECP) that connect the cerebellum to the forebrain.…”