Kartagener Syndrome (KS): Presentation of Five Pediatric Clinical Cases and Review of Recent Literature

Abstract

Objectives: To describe the clinical presentation, diagnostic journey, and outcomes of five pediatric patients with Kartagener syndrome (KS), in order to highlight the challenges of early diagnosis and multidisciplinary management within a Moroccan context. Study Design: Descriptive case series. Place and Duration of Study: Department of Pediatrics, Mohamed V Military Teaching Hospital, Rabat, Morocco; January 2023 to June 2025. Methodology: Five patients (4 females, 1 male), aged 4.5 to 20 years, diagnosed with KS were included. Data were collected retrospectively from clinical files. All patients presented with chronic respiratory symptoms including productive cough, recurrent sinusitis, and bronchorrhea. Imaging studies (chest X-ray [CXR], thoracoabdominal computed tomography [CT], and echocardiography [Echo]) were used to confirm the presence of situs inversus and bronchiectasis. Treatment protocols included antibiotics, respiratory physiotherapy (RP), and vaccination. One patient developed chronic respiratory failure requiring long-term oxygen therapy (O2T). Results: Five patients were included, with a mean age of 9.7 ± 6.7 years and a median of 6 years (range 4.5–20); four were female and one male. The mean delay between the onset of symptoms and diagnosis was 4.2 ± 3.6 years, with a median of 3 years (range 1–10). All patients (100%) presented with chronic respiratory symptoms, complete situs inversus, and bilateral bronchiectasis on imaging. ENT complications were observed in 2 patients (40%): one case of recurrent otitis media and one case of mastoiditis. Documented bacterial infections included Pseudomonas aeruginosa in 2 cases (40%) and Haemophilus influenzae in 1 case (20%). Two patients (40%) required repeated hospitalizations for lower respiratory tract infections, and the only adult patient (20%) developed chronic respiratory failure requiring long-term oxygen therapy. No genetic testing could be performed due to unavailability, but all patients received antibiotics, regular respiratory physiotherapy, and vaccination updates as indicated. Conclusion: KS remains underdiagnosed in low-resource settings due to lack of awareness and diagnostic tools. Early recognition of the association between chronic respiratory symptoms and situs inversus is essential. A multidisciplinary approach is key to improving patient outcomes and preventing severe respiratory complications.