The Turkish Journal of Pediatrics
2020 , Vol 62 , Num 1
A rare cause of acute abdominal pain in a patient with Primary ciliary dyskinesia with situs inversus totalis
1 Departments of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey2 Departments of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey
3 Departments of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey DOI : 10.24953/turkjped.2020.01.024 Primary ciliary dyskinesia (PCD) is a rare, genetic disease characterized by ciliary dysfunction. Patients may present with respiratory distress during neonatal period; chronic sinopulmonary disease, bronchiectasis, recurrent otitis media, sinusitis and infertility in later periods. About 50% of PCD patients have situs inversus totalis and 6-12% have situs ambiguous known as heterotaxy syndromes. Herein, we present a case of PCD and accompanying situs inversus who had acute abdominal pain and was diagnosed with torsion of one of the multiple spleens. Evaluation of acute abdominal pain in these patients has great importance since the internal organs are not at their typical locations. Keywords : Kartagener syndrome, primary ciliary dyskinesia, spleen torsion