Anesthesia for corrective spinal surgery in a patient with Leigh's disease

We report a case of anesthesia for posterior spinal fusion in a woman with Leigh's disease. This is a syndrome with a heterogeneous phenotype including ocular signs, motor signs, and respiratory disorder. It is associated with defects in the enzymes of the mitochondrial respiratory chain and central neural degeneration. Anesthesia is associated with worsening of the respiratory symptoms. Our patient underwent major spinal surgery as a palliative procedure. Her postoperative course was complicated by acute lung injury and sepsis. She ultimately failed a prolonged respiratory wean. Serial magnetic resonance imaging revealed a rapidly progressive necrosis of her brain stem and cervical spinal cord consistent with activation of her underlying Leigh's disease. This is the first report of spinal surgery in this patient group. It is also the first radiological demonstration of Leigh's disease reactivation in the postoperative period. Anesthesia and surgery are hazardous in this patient population, and respiratory symptoms make this a high-risk group. Surgery should only be undertaken with caution and after frank consent. Early postoperative imaging is recommended if there are respiratory complications. No drug prophylaxis has been shown to alter disease activation.

Patients suffering from Leigh's disease are at high risk of serious postoperative respiratory morbidity. We present a case that demonstrates delayed respiratory complications and link this postoperative adverse outcome to aggressive reactivation of the underlying neurodegenerative condition.