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Cerebellar cavernous malformation in pediatric patients: defining clinical, neuroimaging, and therapeutic
Friederike Knerlich-Lukoschus1,2, Paul Steinbok1, Christopher Dunham3
1Division of Pediatric Neurosurgery, British Columbia Children's Hospital and Department of Surgery, University of British Columbia;
Insights
Pediatric cerebellar cavernous malformations (CCMs) present with severe headache and cerebellar dysfunction. Surgical removal of these large lesions offers excellent outcomes in children.
Area of Science:
- Neurology
- Pediatric Neurosurgery
- Neuroradiology
Background:
- Cerebellar cavernous malformations (CCMs) are rare in children.
- Limited data exists on their specific clinical, radiological, and surgical characteristics in pediatric populations.
Observation:
- Five pediatric patients (2.5-14 years) with CCM presented with acute severe headache and cerebellar dysfunction.
- Neuroimaging revealed large, hemorrhagic cerebellar lesions with characteristic features, often mimicking tumors.
- Developmental venous anomalies (DVAs) were present in 3 of 5 patients.
Findings:
- Pediatric CCMs present acutely and exhibit distinct imaging findings, including heterogeneous signal intensities and blooming on susceptibility-weighted imaging.
- Lesions are often large, with spherical, cystic, or pseudotumoral morphology.
- Surgical resection was performed in 4 patients, achieving complete removal and excellent functional outcomes without complications.
Implications:
- Cerebellar cavernous malformations in children have specific clinical and imaging features.
- Early diagnosis and surgical intervention are crucial for favorable outcomes.
- Surgery is a safe and effective treatment for pediatric cerebellar cavernous malformations.
Object:
Cerebellar cavernous malformations (CCMs) have not been specifically described in the pediatric age group. Authors of this study, after considering the published literature, describe the characteristic clinical, radiological, and surgical features of CCM in children. METHOSDS: Patients younger than 18 years of age who were known to have CCM and had undergone surgery between 1992 and 2014 at the authors' institution were reviewed. Pediatric CCM cases reported in the literature (case reports and cases included in series on CMs in the pediatric age group) were also analyzed for specific features of this entity.
Results:
Four male patients and 1 female patient (2.5-14 years of age) with CCM presented acutely with severe headache followed by cerebellar dysfunction. In all patients, neuroimaging (cranial CT and MRI) demonstrated hemorrhagic cerebellar lesions with heterogeneous T1 and T2 signal intensities and hyperintense blooming on susceptibility-weighted imaging. The lesions reached large sizes exhibiting spherical, cystic, and often "pseudotumoral" morphology. In 3 patients, developmental venous anomalies (DVAs) were found. In 4 of the 5 patients, the CCMs and hematomas were totally removed. All patients had a clinically excellent functional outcome without surgical complication and with complete resolution of their presenting symptoms.
Conclusions:
Cerebellar CMs occur in all pediatric age groups and display characteristic clinical and imaging features. In children, CCMs reach large sizes and can result in massive hemorrhage, often leading to a possible diagnosis of hemorrhage into a tumor. An associated DVA is quite common. Surgery is a safe and efficient treatment option with excellent outcomes in patients.
