Hereditary Hemorrhagic Telangiectasias (HHT) (Osler-Weber-Rendu Disease)

 

 

Considerations

 

  • Risk of excessive bleeding from variety of surfaces/organs:

    • Nose: epistaxis (nasal intubation contraindicated)

    • Upper airway hemorrhage (potential for difficult airway)

    • Spinal AVM: risk of paralysis from epidural hematoma (image before neuraxial technique!)

    • Pulmonary hemorrhage requiring lung isolation

    • Cerebral AVM: risk of intracranial hemorrhage

    • GI bleeding

    • Hepatic AVM: portal HTN, high output heart failure

  • Paradoxical emboli from intrapulmonary shunts (AVM): de-air lines!

  • Chronic anemia with potential for a difficult cross-match

  • Coagulopathy: low grade DIC, reduced platelet function

 

 

Goals & Conflicts

 

  • May present for laser treatment of epistaxis, embolization of pulmonary AVMs, craniotomy for AVM resection, treatment of GI bleeding

  • Thorough preoperative workup if suspected AVMs:

    • Pulmonary AVM: chronic hypoxemia, risk of pulmonary hemorrhage

    • Renal & liver function

    • Spinal MRI to rule out spinal AVM prior to neuraxial anesthesia

    • Rule out preoperative anemia, group & screen

    • Antibiotic prophylaxis if AVMs

  • Intraoperative care:

    • De-airing of all lines

    • Avoidance of airway manipulation if telangiectasias are present

    • Avoid nasal intubation if history of epistaxis

    • Modify anesthesia if high-output cardiac failure

 

 

Pregnancy Considerations

 

  • High risk for worsening of AVMs during pregnancy & peripartum period

  • Pulmonary hemorrhage, intracerebral & spinal hemorrhage, GI bleeding

  • Shunt-induced high output cardiac failure

  • Systemic embolism

  • Spinal MRI prior to epidural & spinal anesthesia

  • Avoid HTN during anesthetic management