Medical Adjuvants in the Treatment of Surgically Refractory Arteriovenous Malformations of the Head and Face: Case Report and Review of Literature

Cerebrovasc Dis. 2021;50(5):493-499. doi: 10.1159/000515168. Epub 2021 Jul 1.


Background: Arteriovenous malformations (AVMs) of the brain and face present unique challenges for clinicians. Cerebral AVMs may induce hemorrhage or form aneurysms, while facial AVMs can cause significant disfigurement and pain. Moreover, facial AVMs often draw blood supply from arteries providing critical blood flow to other important structures of the head which may make them impossible to treat curatively. Medical adjuvants may be an important consideration in the management of these patients.

Summary: We conducted a systematic review of the literature to identify other instances of molecular target of rapamycin (mTOR) inhibitors used as medical adjuvants for the treatment of cranial and facial AVMs. We also present 2 cases from our own institution where patients were treated with partial embolization, followed by adjuvant therapy with rapamycin. After screening a total of 75 articles, 7 were identified which described use of rapamycin in the treatment of inoperable cranial or facial AVM. In total, 21 cases were reviewed. The median treatment duration was 12 months (3-24.5 months), and the highest recorded dose was 3.5 mg/m2. 76.2% of patients demonstrated at least a partial response to rapamycin therapy. In 2 patients treated at our institution, symptomatic and radiographic improvement were noted 6 months after initiation of therapy. Key Messages: Early results have been encouraging in a small number of patients with inoperable AVM of the head and face treated with mTOR inhibitors. Further study of medical adjuvants such as rapamycin may be worthwhile.

Keywords: Adjuvant; Arteriovenous malformation; Bleeding; Embolization; Inoperable; Medical therapy; Molecular target of rapamycin; Rapamycin; Surgery; Vascular malformation.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Systematic Review

MeSH terms

  • Adult
  • Arteriovenous Malformations / diagnostic imaging
  • Arteriovenous Malformations / enzymology
  • Arteriovenous Malformations / immunology
  • Arteriovenous Malformations / therapy*
  • Combined Modality Therapy
  • Embolization, Therapeutic
  • Face / blood supply*
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Intracranial Arteriovenous Malformations / diagnostic imaging
  • Intracranial Arteriovenous Malformations / enzymology
  • Intracranial Arteriovenous Malformations / immunology
  • Intracranial Arteriovenous Malformations / therapy*
  • Sirolimus / therapeutic use*
  • TOR Serine-Threonine Kinases / antagonists & inhibitors*
  • TOR Serine-Threonine Kinases / metabolism
  • Treatment Outcome
  • Young Adult


  • Immunosuppressive Agents
  • MTOR protein, human
  • TOR Serine-Threonine Kinases
  • Sirolimus