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Posterior fossa syndrome: identifiable risk factors and irreversible complications
1Children's Medical Center of Dallas, Neurosurgeons for Children, Tex 75235-7794, USA. ddoxey@childmed.dallas.tx.us
Abstract:
Cerebellar mutism was first described by Rekate et al. in 1985 as a transient condition which occurs after posterior fossa operations in children. Posterior fossa syndrome (PFS) and cerebellar mutism are often used interchangeably in the literature. In our experience, we found cerebellar mutism to be a reversible component of a persistent neurologic syndrome. The cause and identifiable risk factors have not been clearly elucidated in the literature. To further characterize PFS, we reviewed 253 children with posterior fossa tumors who underwent surgical resection. We documented 20 cases of PFS (8%), 12 males and 8 females. Age ranged from 1.5 to 13 years (mean = 6.5). Of the 20, 16 were medulloblastoma, 3 ependymoma and 1 astrocytoma. There was a 21 % incidence (16/76) of PFS in medulloblastoma of the posterior fossa. The incidence for ependymoma was 13% (3/24) and 1% (1/102) for astrocytoma. All 20 cases (100%) had brainstem involvement by the tumor. The most frequent postoperative findings included mutism, ataxia, 6th and 7th nerve palsies and hemiparesis. Mutism had a latency range of 1-7 days (mean = 1.7) and a duration of 6-365 days (mean = 69.2, median = 35). Although mutism resolved in all cases, the remaining neurologic complications which characterized our findings of PFS were rarely reversible. We describe potential risk factors for developing PFS after surgery with hopes of making neurosurgeons more aware of potential problems following the removal of lesions in this area. Early recognition of PFS would further promote patient and family understanding and coping with this syndrome.
Insights
Posterior fossa syndrome (PFS) in children, characterized by cerebellar mutism, is a reversible component of a persistent neurological condition after posterior fossa surgery. Identifying risk factors is crucial for early recognition and management.
Area of Science:
- Pediatric Neurosurgery
- Neuro-oncology
- Neurology
Background:
- Cerebellar mutism, often termed Posterior Fossa Syndrome (PFS), is a recognized post-surgical complication in children.
- While often transient, its relationship to persistent neurological deficits requires further clarification.
- Risk factors and precise characterization of PFS remain incompletely understood.
Purpose of the Study:
- To characterize Posterior Fossa Syndrome (PFS) in pediatric patients undergoing surgical resection of posterior fossa tumors.
- To identify the incidence and clinical features of PFS, including cerebellar mutism.
- To elucidate potential risk factors associated with the development of PFS.
Main Methods:
- Retrospective review of 253 children with posterior fossa tumors who underwent surgical resection.
- Documentation of clinical presentation, tumor type, and postoperative neurological deficits.
- Analysis of incidence, latency, and duration of mutism and other neurological complications.
Main Results:
- Twenty cases (8%) of PFS were identified, with a higher incidence in medulloblastoma (21%) compared to ependymoma (13%) and astrocytoma (1%).
- All patients with PFS had tumors involving the brainstem; common postoperative findings included mutism, ataxia, and cranial nerve palsies.
- While mutism resolved in all cases, other neurological deficits associated with PFS were often persistent.
Conclusions:
- Cerebellar mutism is a reversible manifestation of a broader, persistent neurological syndrome (PFS) following posterior fossa surgery.
- Brainstem involvement and tumor type (particularly medulloblastoma) are associated with PFS development.
- Increased awareness and early recognition of PFS are vital for improved patient and family management.