2016 FSA Posters

P006: COLLABORATIVE MANAGEMENT OF AN EXTENSIVE MAXILLOFACIAL ARTERIOVENOUS MALFORMATION
Mary Zoccoli, MD, Matthew Bargas, MD, Stefanie Schrum, MD, Adebisi Ajala, MD, Rebekah Wheatley, MD, Steven Andreoli, MD, William McDonald, MD; Wolfson Children's Hospital

Introduction: Arteriovenous malformations (AVMs) are abnormal blood vessels that cause multiple irregular connections between arteries and veins.  AVMs of the mandible are rare although 50% of all AVMs within bone occur in the maxillofacial region. Interosseous AVMs often remain undiagnosed until a dramatic bleeding incident occurs, which is usually a result of dental manipulation.  AVMs have a high propensity to bleed and can result in disastrous complications if handled carelessly and result in loss of an airway.

Case Description: A 13 year old 81.2kg female from Peru presented with a complex, pulsatile arteriovenous malformation involving the left lower and mid-face. She underwent mandibulectomy with a fibular flap in Peru however; the lesion continued to grow rapidly upon menarche with ulceration and bleeding.  She presented to our hospital for staged embolization and surgical resection. She was morbidly obese with a large, ulcerated left facial mass causing significant swelling and complete deviation of her mouth to the right. Her pre- and intraoperative anesthetic management was concerning for the potential of a difficult airway, inability to ventilate and massive hemorrhage.  Management of the first staged embolization included maintenance of spontaneous ventilation with titration of a combination of medications, microlaryngoscopy/bronchoscopy and oral intubation in the OR by an ENT surgeon followed by transport to cath lab.  At the conclusion of the procedure the patient was extubated awake in the OR and admitted to the ICU.  Her second embolization included nebulized lidocaine 4% followed by titration of midazolam, ketamine, fentanyl and dexmetatomidine.  Two handed assisted mask ventilation was successful and paralytic was given. She was fiberoptically nasally intubated in the OR, transported to cath lab, and remained intubated for definitive resection the following day.  With concerns for excessive hemorrhage, a rapid infuser, cell saver and numerous blood products were available in the room for surgical resection. Blood loss was approximated to be 400ml and she did not require any blood products. She was extubated on post-operative day 4 and remained in the ICU for monitoring. 

Discussion: Mandibular VMs usually appear during adolescence. They may be capillary, lymphatic, venous, arterial or mixed.  VMs of arterial or arteriovenous origin are often referred to as “high-flow vascular malformations” and are often the cause of massive, sometimes fatal hemorrhages.  Overgrowth can result from hormonal imbalances, vasomotor disturbances, infections or trauma. Numerous treatments in varying combinations and various degrees of success have been described. Embolization followed by surgical treatment is the modern conventional approach.  This procedure controls the acute hemorrhagic phase, but does not eliminate the risk of recurrence.  It does, however, reduce the blood flow, allowing subsequent excision within 48 hours – 2 weeks. 

Conclusion: The rareness of AVMs and associated morbidity/mortality they can cause obviates the urgency in taking measures once detected.  This case describes successful airway management and treatment of an extensive maxillofacial AVM by embolization followed by surgical resection in an adolescent with this potentially life-threatening vascular disorder.