SESSION TYPE: Airway Global Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Kartagener syndrome is a rare, ciliopathic, autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract and fallopian tube. Patients usually present with chronic recurrent rhinosinusitis, otitis media, pneumonia, and bronchiectasis usually caused by pseudomonal infection. Situs inversus can be seen in about 50% of cases. We present a case of a 9-year-old boy with chronic recurrent upper respiratory infections. He was diagnosed with Kartagener syndrome based on his clinical presentation and radioimaging Keywords: bronchiectasis, immotile cilia syndrome, situs inversus
CASE PRESENTATION: A 9 year old male child admitted in our hospital, presenting with recurrent nasal blockade, cough with expectoration and intermittent fever since early infancy. His previous records showed a lot of investigations for tuberculosis and examinations of the sputum. On examination he was febrile with wheezy chest and bilateral coarse crackles. Initial suspicion was that of bronchial asthma with recurrent chest infections or pneumonia but the possibility of other causes were also kept in mind. His heart sounds were heard best on the right side of the chest. On further work up, basic investigations were under normal limits and in ECG and radiological imaging following were observed, Chest X Ray showed dextrocardia with gastric bubble on right side and liver dullness on left side.ECG showed tall R waves in lead V1 and absent R waves in V4,V5 and V6, Chest Tomography showed Total Situs Inversus with Liver on rleft side and spleen and stomach on right side. Lung parenchyma showed bronchiectatic changes mainly in posterior basal segments of left lower lobes while CT PNS showed B/L maxillary Sinusitis, mucosal thickening in both maxillary sinuses with opacified frontal sinusitis while ECHO showed trivial MR with TR
DISCUSSION: Although there is no specific treatment for this condition, failure to recognize the condition may subject the patient to unnecessary repeated admissions and investigations and inappropriate treatment.
CONCLUSIONS: Considering the patient clinically, along with sinusitis,bronchiectasis and situs inversus, the clinical diagnosis of Kartagener's syndrome was made. By early diagnosis of this rare entity, patient and his family is saved from unnecessary investigations and inappropriate treatment and later in life when child will attain puberty it is advisable to go under semen analysis that may affect his marital life considering social impact of this entity.
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DISCLOSURE: The following authors have nothing to disclose: Saurabh Kansal, Gopal Chawla, A.p. Kansal, Amitesh Gupta
No Product/Research Disclosure InformationGovt. Medical College, Patiala, India