Anesthesia Considerations 

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