Bronchiolitis as a Feature of Kartagener Syndrome

A Case Report

Abstract

Kartagener syndrome (KS), also known as immotile cilia syndrome or as a primary ciliary dyskinesia, is characterized by the triad of situs inversus, bronchiectasis, and chronic pansinusitis. A few studies reported that diffuse bronchiolitis might be one of the characteristic features of the lung in KS. We aimed to present the radiologic characteristics of KS, including diffuse bronchiolitis, sinus aplasia, and situs inversus totalis in a single case.

Kartagener syndrome (KS), also known as immotile cilia syndrome or as a primary ciliary dyskinesia (PCD), is characterized by the triad of situs inversus, bronchiectasis, and chronic pansinusitis.^1–3 Although bronchiectasis is the main finding of KS, diffuse respiratory bronchiolitis has occasionally been reported. Bronchiectasis occurs in as many as 25% of patients with situs inversus. Sinus involvement in KS is the least distinctive sign of the syndrome and includes absence or hypoplasia of one or more sinuses.⁴ Patients with KS may have situs solitus, that is, dextrocardia only or situs inversus totalis in which all the viscera are on the opposite side.⁵ We present the radiologic characteristics of KS including diffuse bronchiolitis, sinus aplasia, and situs inversus totalis in a case.

CASE REPORT

A 25-year-old male nonsmoker was admitted to the hospital with complaint of productive cough and dyspnea. He had developed recurrent lower respiratory tract infections. He had received many courses of antibiotics but the symptoms continued to recur. Physical examination showed bilateral fine end-inspiratory crackles over lower lung fields. Chest roentgenogram showed dextrocardia (Fig. 1). Pulmonary function tests showed obstructive impairment involving small airways. The computed tomographic (CT) scans of the thorax showed the dextrocardia, bronchiectasis, and bilateral micronodular infiltration (bronchiolitis) in the basal segments of lung (Fig. 2). Abdominal CT scans showed the situs inversus totalis with dextrocardia (Fig. 3). The Waters roentgenogram showed the bilateral aplasia of frontal sinus and maxillar sinusitis (Fig. 4).

FIGURE 1: Chest roentgenogram showing dextrocardia.

FIGURE 2: The computed tomographic scans of the thorax showing the dextrocardia, bronchiectasis (white arrow), and bilateral bronchiolitis (black arrows) in the basilar segments of the lungs.

FIGURE 3: Abdominal computed tomographic scans showing the situs inversus totalis with dextrocardia (white arrow).

FIGURE 4: The Waters roentgenogram showing the bilateral aplasia of frontal sinus and maxillar sinusitis.

DISCUSSION

Bronchiectasis with situs inversus was described for the first time by Siewert,⁶ in 1904. However, Kartagener¹ described a syndrome characterized by the triad of situs inversus, bronchiectasis, and chronic pansinusitis, in 1933. KS was a rare form of PCD that was inherited as an autosomal recessive trait. It has been proposed that normal ciliary beating is necessary for visceral rotation during the embryonic development. In patients with PCD, half of the patients will have situs inversus, which will be cases of KS, and the other half will have normal situs because of random rotation.⁵ Our patient had situs inversus totalis, sinus aplasia, and bronchiectasis. A CT scan also showed diffuse bronchiolitis. Defects on the ciliary motility results in impairment of respiratory defense mechanisms and leads to recurring upper and lower respiratory tract infections.

Amitani et al⁷ reported 9 cases of PCD, including 6 cases of KS, 2 cases of DPB, and 1 case of bronchiectasis in 1990. The investigators described diffuse micronodular shadows on the chest radiograph in 1 of 6 patients with KS and in both patients with DPB. In 1999, Homma et al,⁸ published the first report that described the histopathologic characteristics of the diffuse centrilobular nodules in patients with KS. In that study, 6 of 8 patients with KS showed diffuse centrilobular small nodules of up to 2 mm in diameter throughout both lungs, in addition to bronchiectasis and dextrocardia on chest CT scan, as seen in our patient. The investigators concluded that diffuse bronchiolitis might be one of the characteristic features of the lung in KS. The findings of our case confirmed this definition. Homma et al⁸ explained the superinfection because of ciliary immobility in the former and the disturbance of the immunologic defense mechanisms in the latter. With regard to this suggestion, Vevaina et al⁹ showed that cilia exhibited complete absence of only the inner arms, while retaining the outer arms, and mucociliary clearance was totally absent, with normal neutrophil chemotaxis and other immunologic functions in a patient with KS.

In conclusion, diffuse bronchiolitis should be included in the diagnostic criteria of KS.