Hello, my name is Coleman Reardon, and today I'll present the natural history of conservative versus operative management of pediatric brainstem cavernous malformations. I have nothing to disclose. Pediatric brainstem cavernous malformations are rare, and previous studies suggest a more aggressive clinical course than supratentorial lesions. There is a lack of consensus on the indications for operative versus conservative management. To address this question, we designed a single-center retrospective case series of pediatric brainstem cavernous malformations. Patients were identified from a prospectively maintained surgical database. Our objective was to characterize our institution's indications for surgical intervention and to compare the natural histories of patients undergoing conservative versus surgical management. At Boston Children's Hospital, patients are surgical candidates if they have history of recent hemorrhage, severely symptomatic presentation, and if their lesion breaches the peel surface. In rare cases, lesions subjacent to the surface with particularly severe symptoms are considered for surgery. Here are MR imaging studies of four patients who were not considered surgical candidates, all of which demonstrate intrinsic lesions with a rind of functional tissue, which is pointed out in the bottom right pane by the red arrows. In comparison, here is a dorsally exophytic left pontine lesion that was deemed appropriate for surgical resection. The bottom panels show the postoperative result of a gross total resection. Demographics and clinical presentation are shown here. Surgical patients were more likely to present with weakness in hemiparesis, ataxia, visual changes, and cranial nerve deficits. Patients with a known genetic predisposition were generally not considered for surgery. Functional status, as determined by modified Rankin score, MRS, did not differ at the time of presentation. Radiographically, the majority of patients presented with a hemorrhage from the brainstem cavernous malformation. Surgical patients were more likely to have a developmental venous anomaly. Mean follow-up for the cohort was six and a half years. All but one patient in the observational group and all but two in the surgical group demonstrated stability or improvement of symptoms. On average, MRS improved from presentation in the observational group, whereas it worsened slightly in the surgical group. The clinical courses of both observational and surgical patients are represented here with interval hemorrhages from the brainstem cavernous malformations represented by black squares. These will be discussed in subsequent slides. Among nine operative cases, five patients achieved apparent gross total resection. Upon follow-up, patients with gross total resection demonstrated an annual re-hemorrhage rate of 2.3% per patient year, compared to 13.2% among patients with subtotal resection. In comparison, the hemorrhage rate in the observational group was 2.6% per patient year. At Boston Children's Hospital, pediatric patients with brainstem cavernous malformations are considered for surgical resection only if they present with severely symptomatic hemorrhage from a lesion breaching or subjacent to the PL surface. Quiescent and deep-seated lesions were not surgical candidates. In our experience, hemorrhage rate in the observational group was low. With careful selection and thorough multidisciplinary evaluation, many patients may experience a relatively benign clinical course with observation alone.

pediatric brainstem cavernous malformations

Boston Children's Hospital

natural history

surgery

observational group