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Great Ormond Street Hospital London WC1N 3JH, United Kingdom
We read with interest the article by Wunderbaldinger et al. [1]. We concur with the findings of chylous pleural effusion and pulmonary involvement in lymphangiomatosis. Compression of lymphatics by the central mediastinal masses, however, may be one of several mechanisms. Chylous pleural effusion and pulmonary involvement more often occur in the absence of mediastinal involvement. Histology of two such cases from our institution revealed numerous ectatic lymph channels extending from the visceral pleura along the interlobular septa and pulmonary veins [2]. These findings concurred with CT findings of irregular thickening of the interlobular septa without evidence of central obstruction. Diffuse involvement of the visceral and parietal pleura with ectatic lymphatic channels that weep chyle into the pleural space is well described by several authors [3, 4].
Our data support the observation that mediastinal involvement occurs within the pediatric population [2]. We have six patients with proven lymphangiomatosis with associated chylous pleural effusions. One of these patients, a 7-year-old boy, had mediastinal involvement, shown on MR imaging, with a rind of uniformly enhancing soft tissue involving the right hemithorax and contralateral paraaortic region.
References
1
University of Vienna Waehringer Guertel 18-20, A-1090 Vienna,
Austria
2
Massachusetts General Hospital Charlestown, MA 02129
We thank Dr. Aviv and Dr. McHugh for their interesting comment on our article [1]. The authors state that in six of their patients with generalized lymphangiomatosis and chylous pleural effusion, only one patient had evidence of mediastinal involvement. A report about two of these six patients has been published previously [2]. This observation indeed contrasts with our findings: all four of our patients with pleural effusion also had mediastinal involvement. In our article, we speculated that the pleural effusions might be caused by a compressive effect of the mediastinal masses on lymphatic pathways. This speculation was supported by observations from the literature [3,4,5,6,7,8]. By mentioning this speculation, we did not mean to advocate mediastinal compression as the only possible factor causing chylous pleural effusions, and we are well aware that other abnormalities could potentially be at the source of the radiographic findings described in our study. We agree that widespread involvement of the parietal and visceral pleura by multiple ectatic lymph channels that weep chyle into the pleural space has previously been described in detail [2, 3, 8, 9]. It is likely that these alterations could also contribute to the formation of chylothorax in patients with generalized lymphangiomatosis. In the light of conflicting evidence [3, 7,8,9], however, both potential causes for chylothorax lack final proof. Given the rarity of lymphangiomatosis, further efforts are required to provide insight into the pathogenetic effects underlying this disease. We therefore welcome Dr. Aviv's and Dr. McHugh's commentary because it clearly complements our article.
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