Figure 1
Teaching Case
SectionChest imaging
Case TypeClinical Cases
AuthorsLamhamedi S, Ouezzani N, Eddarei M, Semlali S, Amil T, Hanine A, El Fenni J
Connected authors
12 years, female
A 12-year-old female patient presented with fever, productive recurrent cough and yellow sputum.
The physical examination showed heart sounds on the right side.
Biochemical profile revealed leukocytosis 12000GB/mm3.
The physical examination showed heart sounds on the right side.
Biochemical profile revealed leukocytosis 12000GB/mm3.
Kartagener syndrome
Cystic fibrosis
Post-infectious bronchiectiasis
Other aetiologies of bronchoectasis
The patient had a chest X-ray demonstrating a situs inversus totalis (dextrocardia, right-sided aortic arch, right-sided stomach bubble and suspected left-sided liver). Chest radiograph showed signs of bronchiectasis most marked at the bases associated with tram lining.
The chest CT confirmed the situs inevrsus totalis and extensive bronchiectasis.
The chest CT confirmed the situs inevrsus totalis and extensive bronchiectasis.
Kartagener syndrome is characterized by the triad of bronchiectasis (lower lobe predominant); sinusitis and situs inversus [1]. Kartagener syndrome belongs to a group of disorders classified as primary ciliary dyskinesias (PCD).
It is considered as a rare autosomal recessive disorder which affects the dynein arms in cilia lining the respiratory epithelium and fallopian tubes and within the flagella of sperm [2].
Patients present with chronic lower respiratory tract infections, but also sinusitis and otitis media; this infection is caused by the abnormal ciliary function which reduce mucus clearance and this in turn acts as a substrate for the growth of bacteria. The syndrome is associated with infertility due to defective sperm motility [3].
The “gold-standard” diagnostic test for PCD has been electron microscopic ultrastructural analysis of respiratory cilia obtained by nasal scrape or bronchial brush biopsy [4].
Bronchiectasis is a common finding both on plain radiography and Computed Tomography (especially high resolution CT) associated with situs inversus totalis and sinusitis [1, 2, 3].
The main differential diagnosis includes other aetiologies of bronchiectasis depending on the infant age, in the neonatal period including transient tachypnea of the newborn (TTNB), neonatal pneumonia and meconium aspiration, as well as surfactant protein deficiency 128 and interstitial lung disease [4].
In our case the Kartagener syndrome was not suspected in the early years of the patient's life; however, the chronic infection of the bronchial tree and the imaging findings were pathognomonic of the syndrome.
Early and accurate diagnosis is necessary in order to reduce the incidence of infections and slow the progression of the disease.
Physiotherapy is the treatment of choice to clear inspissated mucus. Aggressive measures to prevent respiratory infections and treatment of superinfection are thought to slow disease progression. Patients may require lung or lung/heart transplantation even after treating the recurrent infections [5].
The radiologist may suggest the Kartagener syndrome based on a chest radiograph or CT showing the association of situs inversus totalis and bronchiectasis.
Early diagnosis can significantly contribute to delaying the disease progression and the onset of respiratory failure.
It is considered as a rare autosomal recessive disorder which affects the dynein arms in cilia lining the respiratory epithelium and fallopian tubes and within the flagella of sperm [2].
Patients present with chronic lower respiratory tract infections, but also sinusitis and otitis media; this infection is caused by the abnormal ciliary function which reduce mucus clearance and this in turn acts as a substrate for the growth of bacteria. The syndrome is associated with infertility due to defective sperm motility [3].
The “gold-standard” diagnostic test for PCD has been electron microscopic ultrastructural analysis of respiratory cilia obtained by nasal scrape or bronchial brush biopsy [4].
Bronchiectasis is a common finding both on plain radiography and Computed Tomography (especially high resolution CT) associated with situs inversus totalis and sinusitis [1, 2, 3].
The main differential diagnosis includes other aetiologies of bronchiectasis depending on the infant age, in the neonatal period including transient tachypnea of the newborn (TTNB), neonatal pneumonia and meconium aspiration, as well as surfactant protein deficiency 128 and interstitial lung disease [4].
In our case the Kartagener syndrome was not suspected in the early years of the patient's life; however, the chronic infection of the bronchial tree and the imaging findings were pathognomonic of the syndrome.
Early and accurate diagnosis is necessary in order to reduce the incidence of infections and slow the progression of the disease.
Physiotherapy is the treatment of choice to clear inspissated mucus. Aggressive measures to prevent respiratory infections and treatment of superinfection are thought to slow disease progression. Patients may require lung or lung/heart transplantation even after treating the recurrent infections [5].
The radiologist may suggest the Kartagener syndrome based on a chest radiograph or CT showing the association of situs inversus totalis and bronchiectasis.
Early diagnosis can significantly contribute to delaying the disease progression and the onset of respiratory failure.
References
[1] Ioannis Bougioukas1 , Dimitrios Mikroulis1 , Bernhard Danner2 , Lukman Lawal1 , Savvas Eleftheriadis3 , George Bougioukas1 , Vassilios Didilis1 (2010) Coronary artery bypass surgery in a patient with Kartagener syndrome: a case report and literature review. Journal of Cardiothoracic Surgery (PMID: 20796272)
[2] Geremek M, Zietkiewicz E, Diehl SR, Alizadeh BZ, Wijmenga C, Witt M. (2006) Linkage analysis localises a Kartagener syndrome gene to a 3.5 cM region on chromosome 15q24-25. J Med Genet (PMID: 16397065)
[3] Armengot M1, Milara J, Mata M, Carda C, Cortijo J. (2010) Cilia motility and structure in primary and secondary ciliary dyskinesia. Am J Rhinol Allergy (PMID: 20537282)
[4] Margaret W. Leigh, MD,1 Jessica E. Pittman, MD,1 Johnny L. Carson, PhD,1 Thomas W. Ferkol, MD,2 Sharon D. Dell, MD,3 Stephanie D. Davis, MD,1 Michael R. Knowles, MD,4 and Maimoona A. Zariwala, PhD5 (2009) Clinical and Genetic Aspects of Primary Ciliary Dyskinesia / Kartagener Syndrome. Genet Med (PMID: 19606528)
[5] Grech R1, Attard L2, Cuschieri S3 (2012) Kartagener syndrome. eurorad
Case information
| URL: | https://www.eurorad.org/case/12088 |
| DOI: | 10.1594/EURORAD/CASE.12088 |
| ISSN: | 1563-4086 |
Figure 2
Figure 1
departement or radiology military hospital rabat morocco.
Figure 2
departement of radiology military hospital rabat morocco