An atypical case of Kartagener’s syndrome
Keywords:Primary ciliary dyskinesia, Oligospermia, Saccharin test, Nasal polyp, Situs inversus
Kartagener’s syndrome is an autosomal recessive disease characterized by the tetrad of situs inversus, bronchiectasis, sinusitis and infertility. It is a subset of a larger group of disease known as primary ciliary dyskinesia. A 23 year old male patient presented to our hospital with complaints of Right sided headache for the past 3 weeks. General Physical examination was normal except that the heart sounds were heard over the right side. Dextrocardia was suspected. Routine ENT and Diagnostic nasal endoscopy examination revealed deviated septum towards the left along with pale greyish polyp like mass in the right nasal cavity. CT PNS was ordered. It showed bilateral frontal, ethmoidal and maxillary sinusitis with Right nasal polyp. Routine Blood investigations were normal but Chest X-ray showed dextrocardia with fundal shadow over the right side and normal ECG was obtained only when the chest leads were reversed. USG abdomen confirmed the suspicion of situs inversus totalis. To rule out Kartageners syndrome HRCT Chest was done which revealed normal lungs with no findings. Sperm analysis was also done which was normal except for the count (<15 million/ml). Saccharin test was performed and it was delayed (>20 min). Anesthetic fitness was obtained and he was taken up for surgery - Septoplasty with fess. The diagnosis of kartageners syndrome was done based on clinical and radiological findings, with variation in form of no bronchiectasis and oligospermia. This type of atypical presentation of Kartagener’s syndrome might be a rare sub type of the disease which is infrequently reported.
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