Anesthetic Management of a Patient with Pompe Disease & Kartagener Syndrome

Abstract

Pompe disease is an autosomal recessive disorder characterized by prominent cardiomyopathy, hepatomegaly, skeletal muscle weakness, and hypotonia. Kartagener Syndrome is a rare genetic disorder that has the triad of sinusitis, bronchiectasis, and situs inversus.

We report our experience of management of anesthesia in an infant with Pompe disease & Kartagener Syndrome who underwent general anesthesia for venous port insertion.  The patient had growth retardation, severe respiratory failure, global hypertrophic left ventricle, and dextrocardia. The existence of various comorbidities and severe respiratory failure, in this case, led us to use general anesthesia with fewer medications as possible for being on the safe side of anesthesia management.

Bulbar and facial muscle weakness, lung and heart problems, multiple drug use, presence of multiple systemic clinical disorders make anesthesia management difficult in such patients. Therefore, we preferred to use dexmedetomidine, fentanyl, and low dose muscle relaxants for patient safety