A Case of Graves' Disease in a Patient with Kartagener's Syndrome (Complete Visceral Inversion, Chronic Sinusitis, and Bronchiectasis).

Abstract

Introduction: Kartagener's syndrome (KS) is a rare disease characterized by a triad of situs inversus totalis, chronic sinusitis, and bronchiectasis. The disorder is caused by a hereditary genetic abnormality that impairs ciliary movement. Although aberrant pass course of the inferior laryngeal nerves due to visceral inversion should be considered during thyroid surgery in patients with KS, no report of surgical treatment for Graves' disease (GD) in patients with KS has been found to date.

Case presentation: A Japanese male in his 40s was referred to our hospital for surgical treatment for drug-refractory GD. He was diagnosed to have KS by genetic alteration of the DNAH5 gene as well as clinical triad. No abnormal branching in the mediastinal great vessels was identified in the present case, and left-sided non-recurrent inferior laryngeal nerve (NRLN) was not observed during surgery. Previous literature has demonstrated that the presence of a right-sided aortic arch and an anomalous branch of the left subclavian artery, as well as the absence of a left ductus arteriosus demonstrable on preoperative imaging studies, are prerequisites for the development of the extremely rare left-sided NRLN.

Conclusion: We reported the first case of surgical treatment for GD in a patient with KS and discussed the preoperative diagnosis of NRLN.