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Management of complex arteriovenous malformations using a novel combination therapeutic algorithm

JAMA Oct 15, 2018

Chelliah MP, et al. - In managing complicated extracranial head and neck arteriovenous malformations, authors evaluated the effectiveness and tolerability of sirolimus as adjuvant therapy for endovascular embolization. For surgical embolization of complex, extracranial head and neck arteriovenous malformations, a mammalian target of rapamycin inhibitor was found to be beneficial as an adjuvant therapy.

Methods

  • Experts conducted a case series to examine 6 patients with extracranial head and neck arteriovenous malformations treated from January 1, 2013 to December 31, 2017, at a multidisciplinary vascular anomalies clinic within Stanford Hospital and Clinics.
  • Intervention included the initiation of sirolimus at least 1 month prior to endovascular embolization, targeting a trough level of 10 to 15 ng/mL throughout the course of the endovascular embolization series and continued for at least 1 month after the series.
  • Via clinical evaluation and radiographic imaging, they evaluated clinical manifestations and disease progression and overall response to treatment.

Results

  • As per data, a favorable response was seen in all 6 patients (4 male and 2 female patients; mean age, 24.5 years [range, 9-44 years]) to the combination of sirolimus therapy followed by endovascular embolization, with 4 patients exhibiting near-complete response.
  • They noted that 19 months (range, 6-40 months) was the median duration of follow-up.
  • Sirolimus was discontinued by 1 patient soon after embolization and subsequently experienced regrowth of the arteriovenous malformation after 1 year; resuming sirolimus stabilized his disease for more than 2 years.
  • They noted mild adverse effects in 4 patients, and all patients demonstrated good tolerance with the combination therapy.
  • Skin ulceration developed in 1 patient after embolization and required surgical debridement.
  • Pulmonary microthrombi after embolization with cyanoacrylate glue developed in another patient that resolved with a brief course of anti-inflammatory therapy.
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