EMPYEMA THORACIS IN KARTAGENER SYNDROME: AN UNUSUAL PRESENTATION WITH ABDOMINAL HETEROTAXY, LEVOCARDIA, AND POLYSPLENIA

Kartagener’s syndrome (KS) is a rare autosomal recessive disorder classified under primary ciliary dyskinesia (PCD), characterized by the classic triad of bronchiectasis, chronic sinusitis, and situs inversus. However, atypical presentations can occur, posing diagnostic challenges. We present a unique case of a 35-year-old female with KS, exhibiting abdominal heterotaxy with isolated levocardia, empyema thoracis, and polysplenia. The patient, who had a lifelong history of recurrent respiratory tract infections, presented with a chronic cough, pleuritic chest pain, and an abdominal mass on the right side. Imaging including a high-resolution computed tomography (HRCT) of the chest revealing bilateral bronchiectasis, granuloma and pleural effusion on the left side, and a computed tomography (CT) with contrast of the abdomen & pelvis revealed a left-sided liver, and multiple spleens on the right depicting as a mass, consistent with situs inversus, yet the heart remained in its normal left-sided position (levocardia). The absence of prior flu vaccinations, and history of recurrent respiratory infections, underscores the importance of early recognition and intervention in KS to prevent complications such as empyema thoracis. This case highlights the variability in KS presentations and emphasizes the need for awareness of its potential impact on fertility, as evidenced by the patient’s decade-long history of primary infertility. Treatment remains symptomatic, focusing on managing respiratory infections and preventing future complications.

Keywords: Kartagener’s syndrome, Abdominal Heterotaxy, Bronchiectasis, Empyema thoracis, Situs inversus, Infertility