Researchers from Spain reported the case of a patient with Pompe disease who underwent combined anesthesia for osteosynthesis in his left arm.

The anesthetic management of Pompe disease is challenging as patients with the disease have a high risk of complications related to anesthesia, the researchers noted.

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The case study was published in the journal Revista Española de Anestesiología y Reanimación.

The case presented here is that of a 47-year-old male with Pompe disease, who was admitted to the emergency department with pain and dysfunction in the left arm after a fall. Radiography confirmed a fracture in the proximal end of the humerus.

Since he was clinically stable, doctors decided to operate on him and perform osteosynthesis of the proximal end of the humerus. 

He was given 2 g of cefazolin for antibiotic prophylaxis and 50 mg of ranitidine for broncho-aspiration prophylaxis. Brachial plexus block was performed using an aseptic and ultrasound-guided technique with the administration of 15 mL of 0.5% ropivacaine and 0.7% mepivacaine. 

General anesthesia was induced with 100 ug of 60 mg lidocaine, 120 mg of propofol, and 40 mg of rocuronium and was maintained with remifentanil. No other neuromuscular relaxants were used during the operation, which lasted 1.5 hours. 

At the end of the procedure, analgesic rescue was achieved with 2 g metamizole and 50 mg dexketoprofen. Sugammadex at 2 mg/kg was used to reverse residual neuromuscular blockade. Following extubation, the patient was taken to the recovery unit.

The authors reported that he was hemodynamically stable, with good respiratory mechanics and optimal oxygen saturation. There were no incidents right after the operation, in the postanesthesia recovery unit, or in the hospital ward. 

Pompe disease is a rare genetic disease characterized by the deficiency or absence of the acid alpha-glucosidase (GAA) enzyme, the role of which is to break down glycogen to form glucose. Symptoms of the disease include hypotonia, cardiomegaly, macroglossia, respiratory distress, and progressive loss of ventilation.


Ruano Santiago M, Soto Garrucho E, González Marín Y, et al. Anaesthetic implications for Pompe disease. a case description. Rev Esp Anestesiol Reanim. Published February 20, 2023. doi:10.1016/j.redare.2021.09.012