Anesthetic Management of a Patient With Situs Inversus for Bilateral Orthotopic Lung Transplantation

PRIMARY CILIARY DYSKINESIA(PCD),also known as immotile-cilia syndrome,is a rare, autosomal recessive disorder that affects several different organ systems. The underlying defect typically is a mutation in dynein, leading to abnormal ciliary structure and function. Patients with PCD may experience hearing loss, recurrent pulmonary infections, chronic sinusitis, and infertility because cilia are ubiquitous in the middle ear, respiratory tract, nasal passages, and reproductive tract. Cilia also are responsible for determining proper visceral laterality in utero. Thus, during embryogenesis in patients with PCD, visceral laterality will be determined at random, and half of these patients will have situs inversus totalis, or mirror-image visceral anatomy. An additional subset of patients with PCD will have situs ambiguous, wherein visceral anatomy is neither “typical” (situs solitus) nor mirror-image (situs inversus). When present in combination with bronchiectasis and chronic sinusitis, situs inversus related to PCD represents Kartagener syndrome. The authors present the case of a patient with Kartagener syndrome who underwent bilateral orthotopic lung transplantation. Written informed consent to publish this case was obtained from the patient