Cavernous malformations of the basal ganglia in children

Clinical article

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Object

Cavernous malformations (CMs) of the basal ganglia are relatively rare lesions that can lead to considerable neurological impairment because of their eloquent location. The authors reviewed the clinical course and surgical outcome of a series of children with basal ganglia CMs.

Methods

The authors retrospectively reviewed the operative experience of the senior author (R.M.S.) and the 1997–2011 database of Boston Children's Hospital for children with CM of the basal ganglia (which includes CM of the caudate and/or lentiform nucleus and excludes CM of the thalamus). They evaluated baseline demographics, presenting signs, operative outcomes, and condition at long-term follow-up visits and compared these characteristics among patients who underwent surgery and those who were observed.

Results

Of 180 children with a diagnosis of CM, 11 (6%) had CM of the basal ganglia. The mean age at diagnosis was 9.3 years, and the male/female ratio was 1.8:1. Presenting signs were as follows: hemorrhage (8 children), incidental lesions (2), and seizures (1); 2 children had choreiform movement disorders. Treatment was observation or surgery. Observation was chosen for 5 children either because the lesions were asymptomatic (2 children) or because the risk for neurological dysfunction after attempted excision was believed to be high (3 children). These 5 children were observed over a combined total of 30.4 patient-years; none experienced neurological deterioration or symptomatic hemorrhage from their lesions. The other 6 children underwent microsurgical resection of the lesion because they were symptomatic from hemorrhage or increasing mass effect. All 6 of these children had hemorrhagic lesions, of which the smallest dimension was at least 1.5 cm. Of these 6 lesions, 5 were excised completely, and over a combined total of 46 patient-years of follow-up, no rebleeding or late neurological deterioration after surgery was reported.

Conclusions

In this patient population, the natural history of small and asymptomatic CMs of the basal ganglia was benign. The children with large (> 1.5 cm) symptomatic lesions underwent excision; neurological impairment was apparently minimal, and no hemorrhage or neurological deterioration occurred later.

Abbreviation used in this paper:CM = cavernous malformation.

Article Information

Address correspondence to: R. Michael Scott, M.D., Department of Neurological Surgery, Boston Children's Hospital and Harvard Medical School, 300 Longwood Ave., Boston, MA 02115. email: michael.scott@childrens.harvard.edu.

Please include this information when citing this paper: published online June 21, 2013; DOI: 10.3171/2013.5.PEDS1335.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Axial T2-weighted MR images obtained in a 3-year-old boy with severe headache and subsequent lethargy from hemorrhage of a CM of the caudate nucleus. Left: Preoperative. Right: Postoperative. A ventriculostomy was performed, and the lesion was subsequently completely resected via a transcortical, transventricular route.

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    Axial T2-weighted MR images obtained in a 6-year-old girl with seizures and a hemorrhagic CM of the basal ganglia. A: Preoperative. B: Immediately after partial resection. C: Follow-up 8 years after surgery. No hemorrhage or significant change in lesion morphology occurred.

  • View in gallery

    Axial T2-weighted MR images obtained in a 17-year-old girl with severe headache and a hemorrhagic CM of the caudate nucleus head. A: The lesion was resected via a transcortical approach. B: An initial intraoperative MR image demonstrated residual lesion. C: The lesion was then completely resected.

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