An incomplete Kartagener syndrome presenting with recurrent exacerbation of bronchiectasis: a case report

Kartagener syndrome (KS), a rare subgroup of primary ciliary dyskinesia (PCD) with an autosomal recessive pattern of inheritance, is characterized by the clinical findings affecting the ciliary movement. It typically presents with a triad of sinusitis, bronchiectasis, and situs inversus, while cases lacking situs inversus are termed as incomplete KS posing diagnostic challenges.

A 42-year-old, non-smoker, non-alcoholic Nepalese male with a history of recurrent cough, nasal discharge, and infertility was diagnosed with incomplete KS. His clinical examination revealed coarse crackles bilaterally and rhonchi while spirometry showed mixed obstruction and restrictive patterns. His imaging findings clearly indicated infective bronchiectasis. Symptomatic management and multidisciplinary care were initiated for him.

This case reveals the complexity of diagnosing incomplete KS, a subgroup of PCD. The presence of post-infective bronchiectasis, recurrent sinusitis strongly supported the diagnosis. Successful medical intervention and ongoing multidisciplinary management are crucial for addressing the patient's diverse clinical manifestations.

This case highlights the importance of recognizing the varied presentations of KS and/or PCD and the need for a multidisciplinary approach to its management. Increased clinical awareness and timely diagnosis are essential for improving patient outcomes and effectively managing potential complications.