Management of pediatric brainstem cavernous malformations: experience over 20 years at the Hospital for Sick Children

Clinical article

Ratan D. Bhardwaj M.D., Ph.D., Kurtis I. Auguste M.D., Abhaya V. Kulkarni M.D., Ph.D., Peter B. Dirks M.D., Ph.D., James M. Drake M.D., M.Sc., and James T. Rutka M.D., Ph.D.
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  • Division of Neurosurgery, The Hospital for Sick Children, Department of Surgery, The University of Toronto, Toronto, Ontario, Canada
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Object

Because of their location and biological behavior, brainstem cavernous malformations (CMs) pose a formidable clinical challenge to the neurosurgeon. The optimal management of these lesions requires considerable neurosurgical judgment. Accordingly, the authors reviewed their experience with the management of pediatric brainstem CMs at the Hospital for Sick Children.

Methods

The authors performed a retrospective chart review of pediatric patients who had received diagnoses of a brainstem CM at the Hospital for Sick Children over the past 20 years.

Results

Twenty patients were diagnosed with brainstem CMs. The mean age at diagnosis was 10.1 ± 5.4 years, and the patients included 13 boys and 7 girls. The mean maximal diameter of the CM was 14.3 ± 11.2 mm. The lesions were evenly distributed on the right and left sides of the brainstem with 4 midbrain, 13 pontine, and 3 medullary lesions. Seven patients underwent surgery for the management of their CMs, with a mean age at presentation of 5.2 years, and a mean CM size of 21.0 mm. Of note from the surgical group, 2 patients had a family history of CMs, 2 lesions were medullary, the CM reached a pial surface in 6 of 7 patients, and 6 of 7 lesions were located on the right side. The mean age at presentation among the 13 patients in the nonsurgical group was 12.7 years, and the mean CM size was 10.6 mm. Seven of these patients had a prior history of radiation for tumor, and only 3 had lesions that reached a pial surface.

Conclusions

The management of brainstem CMs in children is influenced by multiple factors. The majority of patients received conservative management and tended to be asymptomatic with smaller lesions. Patients with larger lesions and direct pial contact, in whom symptoms arose at a younger age were more likely to undergo surgical management. A history of familial CM was also a predictor for receiving surgical treatment. No patients with a prior history of radiation therapy underwent surgery for CMs. The presence of multiple lesions seemed to have no impact on the type of management chosen. Patients who underwent surgery did suffer morbidity related to the procedure, and tended to improve clinically over time. Conservative management was associated with new deficits arising in children, some of which improved with time. Consideration of many clinical and radiological parameters is thus prudent when managing the care of children with brainstem CMs.

Abbreviations used in this paper:

CM = cavernous malformation; CN = cranial nerve; GTR = gross-total resection.

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