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Case Report |
1
Institut für Diagnostiche Radiologie,
Medizinische Einrichtungen der
Heinrich-Heine-Universität
Düsseldorf, Moorenstr. 5, D-40225
Düsseldorf, Germany.
2
Institut für Kinderheilkunde, Medizinische
Einrichtungen der Heinrich-Heine-Universität
Dusseldorf, D-40225 Düsseldorf, Germany.
Received March 13, 2000;
accepted after revision June 2, 2000.
Address Corespondence to P. Luckey.
Introduction
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In this case report, we would like to emphasize the important role of thoracic helical CT in revealing the extent of respiratory tract chondritis and in supporting the diagnosis.
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The patient's general condition did not improve with intensive immunosuppressive treatment and was predominated by the respiratory tract affection. A surgical widening of the glottis aperture was necessary during the further course of disease because of progressive dyspnea. Histopathologic examination of the cartilage showed a proliferation of mesenchyma and a lymphocytic infiltration, confirming the diagnosis of relapsing polychondritis. A postoperatively performed bronchoscopy revealed signs of tracheo- and bronchomalacia but did not reveal the bronchial status distal of the carina.
Helical CT of the thorax was performed (Somatom Plus; Siemens, Erlangen, Germany) and was used to assess the full extent of respiratory tract involvement. Helical CT revealed edematous tracheobronchial wall thickening combined with deformity of cartilaginous structures and localized narrowing of the lumen in both the trachea (Fig. 1A) and main bronchi. Additional single scans of the upper, middle, and lower thorax were obtained in maximum inspiration and expiration. The in inspiration mean density average of all three levels of the right lung was -740.9 ± 68.2 H (range, -737.4 to -745.3 H) compared with -804.1 ± 69.1 H (range, -801.7 to -807.3 H) in the left lung (Fig. 1B). The in expiration mean density average of all three levels of the right lung physiologically increased to -694.0 ± 85.0 H (range, -688.4 to -699.3 H), whereas the density average in the left lung remained almost constant, -799.5 ± 81.0 H (range, -795.2 to -809.7 H), indicating an air-trapping (Fig. 1C). A complete collapse of the left main bronchus had occurred and was visualized (Fig. 1D). The CT findings were compatible with tracheobronchial malacia caused by severe destructive polychondritis.
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TopIntroductionCase ReportDiscussionReferences |
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Diagnosis is made according to the empirically defined clinical criteria of McAdam et al. [4] and may be confirmed by typical histologic and immunohistochemical findings in examined cartilage samples.
Although there is no clear concept for treatment of relapsing polychondritis in children, in adults pharmacotherapy includes various immunosuppressive drugs [5]. Tracheostoma, application of nasal continuous positive airways pressure [6], and endotracheobronchial stenting [5] improve the clinical situation in critical airways compromise.
The sensitivity of conventional chest radiography is not high enough for an accurate visualization of tracheobronchial chondritis. The role of MR imaging is still limited to the assessment of the upper airways [2]. A pathologic pulmonary function test may support the diagnosis of respiratory tract chondritis but cannot be performed in younger children or in patients with a tracheostoma. Inflammatory and fibrotic alterations of the mucosa as well as tracheobronchial stenosis and collapse are characteristic bronchoscopic findings that prove a respiratory involvement. During bronchoscopy, assessment of poststenotic parts of the bronchial tree is difficult or impossible, and anesthesia may be required, especially in children. The risk of postbronchoscopic exacerbation of relapsing polychondritis is described in the literature [7].
Thoracic CT is an alternative noninvasive examination that is useful for an early, accurate, and rapid assessment of the complete respiratory tract. Typical CT findings include diffuse or localized edematous thickening; calcification and fibrous scarring of the airway wall; and deformity and narrowing of larynx, trachea, or bronchi caused by the destruction of cartilage [8]. Additional scans in expiration should always be obtained. With these scans, expiratory airways collapse can be proved, and it is possible to quantify air-trapping by measuring the mean density in both lungs.
We recommend that thoracic CT be included in the routine workup of patients with relapsing polychondritis. Confirmation of respiratory tract involvement on CT may improve the disease's management, especially in pediatric patients.
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