Anesthesia management of a patient with a femoral neck fracture and hered-itary hemorrhagic telangiectasia.

BACKGROUND: The hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu syndrome is an autosomal dominant genetic disorder affecting the small blood vessels due to mutations in specific genes that lead to angiogenesis errors. HHT represents a clinical entity with great clinical interest as severe, unpredicted, and life-threatening bleeding, sepsis, ischemia, and hemodynamic failure might occur. Literature regarding anesthesia and perioperative management of such patients is limited, with no published papers for orthopedic surgery in patients with HHT.

CASE REPORT: An 82-year-old HHT female patient with femoral neck fracture was scheduled for hemiarthroplasty. Computerized tomography scan revealed an arteriovenous malformation (AVM) in the right lung. The nature of surgery in association with AVM presence suggested the use of regional anesthesia (RA) as the optimal choice. Midazolam (2 mg) was administered as premedication. Perioperatively, Levobupivacaine hydrochloride (15 mg) was administered in the subarachnoid space, at the L4-L5 lumbar intervertebral space, using a 29 Gauge needle. The patient was in lateral position with close monitoring of her blood pressure, electrocardiogram, oxygen saturation, and urine output. Two units of blood were transfused perioperatively. Her recovery was uneventful and she was discharged after ten days.

CONCLUSION: Patients with HHT require careful preoperative evaluation to identify and appreciate possible symptoms as well as to assess their pulmonary and cardiac function accurately. Meticulous preoperative planning is required to minimize perioperative risks and additionally close perioperative monitoring is essential. RA was preferred to general anesthesia as positive pressure ventilation could lead to hypoxia, AVM rupture, embolism, and hemodynamic collapse, while RA offers absence of respiratory stress, excellent muscle relaxation, and decreases blood loss, lowers probability of venal thrombosis and pulmonary embolism, and assists to better perioperative management of analgesia, thus contributing to positive outcome of surgery. Hippokratia 2016, 20(4): 303-305.