A case report of a patient with Kartagener syndrome, also presenting bronchiectasis and pneumonia, who was effectively treated by surgical resection is presented in the study, “Left middle lobectomy for bronchiectasis in a patient with Kartagener syndrome: a case report,” published in the open access Journal of Cardiothoracic Surgery.
Kartagener syndrome, also known as primary ciliary dyskinesia or immotile ciliary syndrome, is a rare genetic disease (approximately 1 in 32,000 live births) characterized by defects in the action of the cilia lining the respiratory tract (but also in the fallopian tube, as well as in the flagella of sperm cells), and is accompanied by sinusitis, bronchiectasis, and situs inversus. (Situs inversus is a genetic condition that causes the organs in the chest and abdomen to be positioned in a mirror image from their normal positions.)
The authors reported a case study of a 23-year-old man admitted to the Department of Thoracic Surgery at Renji Hospital (Shanghai, China) with complaints of recurrent cough with purulent expectoration, occurring intermittently for 10 years. In the past three years, episode frequency was three to four times per year, increasing over the previous six months. The patient was diagnosed with pulmonary infection and bronchiectasis of the left upper lobe, situs inversus, and Kartagener syndrome.
Since the patient’s primary problem was recurrent pneumonia due to bronchiectasis, investigators searched for an effective treatment. After careful examination of the chest computed tomography (CT) scan, the team observed that the bronchiectasic lung was damaged and clearly had three lobes, with lesions in the left middle lobe (no signs of bronchiectasis were found in the upper or lower left lobe, or in the right lung). Team members concluded that the source of the repeated pulmonary infections was the left middle lobe, and decided it should be resected (lobectomy), as the infection would otherwise not be curable.
After resection and during the follow-up period, the patient had no respiratory tract infections, although he suffered from occasional nasal congestion and discharge due to the ineffective mucociliary clearance.
The study reports the first case of a left middle lobectomy to treat bronchiectasis in a Kartagener syndrome patient, and showed that surgical resection is an acceptable strategy for patients whose bronchiectasic lungs are localized within one or two lobes.
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