Cavernous malformations of the basal ganglia in children

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>In this patient population, the natural history of small and asymptomatic CMs of the basal ganglia was benign. The children with large (&gt; 1.5 cm) symptomatic lesions underwent excision; neurological impairment was apparently minimal, and no hemorrhage or neurological deterioration occurred later.</jats:p></jats:sec>

Cerebral cavernous malformations: natural history and clinical management

“…Eloquent CMs that may have previously been symptomatic or that are minimally symptomatic may also be considered for observation [18,20,21,48]. Given the dwindling risk of hemorrhage over time for ruptured CMs [33,34,36], eloquent CMs with a remote history of a single hemorrhage may also be considered for observation [18,20,21,48].…”

“…Other predictors included surgery within 1 year of seizure onset, CM size less than 1.5 cm, solitary CMs, partial seizures only and medical control of the seizures preoperatively. Overall 1.6-3.1% [10,12,14] Incidental CM 0.08-0.2% [5,32] Any unruptured CM 0.3-0.6% [10,12,33,34] Ruptured CM 4.5-22.9% [5,10,12,[33][34][35] Ruptured, within first year 14-18% Resection of eloquent CMs is a subject of significant neurosurgical interest as supratentorial deep CMs and brainstem CMs require both creative and meticulous surgical approaches and technique to allow for successful resection with minimal operative morbidity [18][19][20][21][22][23][24]52,53,[55][56][57][58]. Most of these lesions would be considered 'grade III' CMs in the scheme of Kivelev and colleagues.…”

“…Parkinsonism and extrapyramidal symptoms have been attributed to CMs of the basal ganglia or thalamus as well [20][21][22][23][24][25]. Patients with ganglionic CMs have also presented with hemichorea [20,21,26,27].…”

“…They are significantly less likely to present with seizures in contrast to their supratentorial lobar counterparts [20][21][22][23]. Most commonly, they present with contralateral sensorimotor deficits [20][21][22][23].…”

Childhood Cancer for the Primary Care Physician

“…Infectious causes such as abscesses or fungal lesions of the brain are possible in children. Cavernous malformations, 24 arteriovenous malformations, 25 and congenital malformations 26 may lead to seizure and/or neurologic changes and mimic tumor on imaging. Rare causes like Rosai-Dorfman 27 or Langerhans cell histiocytosis (LCH) 28 should be considered, especially with diabetes insipidus and pituitary stalk changes, which are often seen with LCH.…”