Diagnostic and Interventional Imaging (2014) 95, 317—319

LETTER / Thoracic imaging Tension pneumomediastinum secondary to thoracic air-leak syndrome in chronic graft versus host disease Keywords: Pneumomediastinum; GVHD Case report A 23-year old man presented with increasing dyspnea over the last two months. His past history included an allogenic hematopoietic stem cell transplant (allo-HSCT) for nonHodgkin lymphoma. Twenty months after transplantation, he suffered from systemic GHVD (digestive and cutaneous) treated with corticotherapy. A first CT scan showed bilateral bronchiectasies, ground glass opacities (GGO) and consolidations of mainly peribronchovascular distribution (Fig. 1). Two months later, a second CT scan showed progression of GGO, bronchiectasies and severe interstitial emphysema onset (Fig. 2a). A small pneumomediastinum and two cavitated nodules were demonstrated (Fig. 2b). Infection by Aspergillus fumigatus was confirmed by bronchiolo-alveolar lavage and the patient was treated with posaconazole (Noxafil® ). One month later, the patient was admitted for acute respiratory distress in emergency room. A third CT scan demonstrated a massive pneumomediastinum collapsing right and left atria (Fig. 3). Our patient died a few days later of cardiac failure caused by tension pneumomediastinum with context of Aspergillosis infection progression. Discussion Thoracic air-leak syndrome (TALS) was defined by Franquet et al. [1] as the presence of extra-alveolar air in cases of allogenic stem-cells transplantation. The condition is strongly associated with chronic GVHD (graft versus host disease). Extra-alveolar air includes spontaneous interstitial emphysema, pneumomediastinum or pneumothorax. TALS is rare with overall incidence of 0.83% to 2.3% after alloHSCT [1—5], but it reaches 20% in patients with bronchiolitis obliterans (BO) or bronchiolitis obliterans with organizing pneumonia (BOOP) [2]. It is admitted that BO, BOOP and non-classifiable interstitial pneumonia represent lung complications associated with GVHD. In our case, the patient suffered from chronic GVHD and progressive dyspnea leaded to perform chest CT that showed GGO and consolidation in peribronchovascular distribution associated with traction

bronchiectasies. This CT pattern is strongly suggestive of non-classifiable interstitial pneumonia according to the CT classification recently proposed by Song et al. [6]. Nonclassifiable interstitial pneumonia presents some similarities with BOOP that may bring confusion. Corticoid treatment of GVHD is a risk factor for invasive aspergillosis [7] and the association between invasive pulmonary aspergillosis and TALS was previously underlined by the study of Vogel et al. [2]. The physiopathology to explain the air-leak syndrome remains unclear. Spontaneous pneumomediastinum is explained by the Macklin effect: after alveolar rupture, free gas is traveling along peribronchovascular fascial sheaths (interstitial emphysema) into the mediastinum. This process arises by an increased alveolar pressure or an alveolar injury. BO or BOOP causes bronchial obstruction and increases alveolar pressure by ball-valve effect [3]. Indeed, pulmonary diseases such as BOOP, interstitial disease and fibrosis were proven to be risk factor for spontaneous pneumomediastinum [8]. Inflammation due to GVHD cause injury to the lungs and may lead to fibrose changes in the peripheral airways [4]. In nonclassifiable interstitial pneumonia, traction bronchiectasis might have been a consequence of peribronchial fibrosis [6]. Invasive aspergillosis with cavitary nodules worsens the parenchyma injury that could explain the association with TALS. Nevertheless, the exact physiopathology of TALS remains unknown. It is admitted that TALS represents late complication of allo-HSCT that occurs more than 100 days after HSCT [5]. Previous reports described bad prognosis in TALS with a mortality rate between 67 and 100% [2—5]. Nevertheless, we know that late onset non-infectious pulmonary complications (LONIPCS) have bad prognosis too, especially in BO in which the mortality rate is reported from to 14% to 100% (mean 61%) [9—11]. Our patient died 34 days after the diagnosis of air-leakage syndrome. In previous studies, the mean time until death varied between 28 and 45 days [2,4]. Prognosis in GVHD with TALS seems to be worse than in GVHD without TALS, even on short-term. Long-term recovery seems to be very rare for patients who survived the acute phase of air-leak syndrome. Prognosis remains very bad due to progressive worsening of chronic GVHD or infection [3]. In summary, TALS is strongly associated with GVHD and may be associated with Aspergillus infection. To the best of our knowledge, it is the first time that this association can be demonstrated by successive high-resolution CT scanners. The physiopathological process remains unclear but

2211-5684/$ — see front matter © 2013 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved. http://dx.doi.org/10.1016/j.diii.2013.09.006

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Figure 1. Baseline high-resolution chest CT performed because patient complained of progressive dyspnea. Axial view (a) and coronal view (b). Parenchymal ground glass attenuations and consolidations predominantly peribronchovascular in distribution, with traction bronchiectasies. This pattern is compatible with non-classifiable interstitial pneumonia.

Figure 2. High-resolution CT realized 2 months later in increased dyspnea and septic syndrome. a: in axial view, aggravation of bronchiectasis and GGA and apparition of interstitial emphysema: linear hypodensities follow the peribronchovascular sheats (white arrows) and surround the pulmonary artery with the ‘‘ring around artery’’ sign (white circle); b: pneumomediastinum following interstitial emphysema (black arrow) and apparition of cavitary nodules with ‘‘halo sign’’ (white *) in the right and left superiors lobes. Infection by Aspergillus fumigatus was confirmed by bronchiolo-alveolar lavage.

TALS probably expresses the gravity of the GVHD more than any specific disease. Our case underlines the short-term bad prognosis. Disclosure of interest The authors have not supplied their declaration of conflict of interest. References

Figure 3. CT performed three weeks later. The patient presented acute respiratory distress. Contrast-enhanced CT in axial view in pulmonary window demonstrated massive pneumomediastinum (white *) with compression of cardiac cavities (white arrows). The patient died a few days later. OD: right atrium, OG: left atrium, VG: left ventricle.

[1] Franquet T, Rodríguez S, Hernandez JM, Martino R, Giménez A, Hidalgo A, et al. Air-leak syndromes in hematopoietic stem cell transplant recipients with chronic GVHD. High resolution CT findings. J Thorac Imaging 2007;22:335—40. [2] Vogel M, Brodoefel H, Bethge W, Faul C, Hartmann J, Schimmel H, et al. Spontaneous thoracic air-leakage syndrome in patients following allogeneic hematopoietic stem cell transplantation: causes CT-follow up and patient outcome. Eur J Radiol 2006;60:392—7. [3] Moon MH, Sa YJ, Cho KD, Jo KH, Lee SH, Sim SB. Thoracic air-leak syndromes in hematopoietic stem cell transplant recipients with graft-versus-host disease: a possible sign for poor

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response to treatment and poor prognosis. J Korean Med Sci 2010;25(5):658—62. Toubai T, Tanaka J, Kobayashi N. Mediastinal emphysema and bilateral pneumothoraces with chronic GVHD in patients after allogeneic stem cell transplantation. Bone Marrow Transplant 2004;33(11):1159—63. Sakai R, Kanamori H, Nakaseko C, Yoshiba F, Fujimaki K, Sakura T, et al. Air-leak syndrome following allo-SCT in adult patients: report from the Kanto study group for cell therapy in Japan. Bone Marrow Transplant 2011;46(3):379—84. Song I, Yi CA, Han J, Kim DH, Lee KS, Kim TS, et al. CT findings of late-onset non-infectious pulmonary complications in patients with pathologically proven graft-versus-host disease after allogeneic stem cell transplant. Am J Roentgenol 2012;199(3):581—7. Crawford SW. Bone-marrow transplantation and related infections. Semin Respir Infect 1993;8:183—90. Sakai M, Murayama S, Gibo M, Akamine T, Nagata O. Frequent cause of the Macklin effect in spontaneous pneumomediastinum: demonstration by multidetector-row computed tomography. J Comput Assist Tomogr 2006;30(1): 92—4. Solh M, Arat M, Cao Q, Majhail NS, Weisdorf D. Late-onset non-infectious pulmonary complications in adult allogeneic

319 hematopoietic cell transplant recipients. Transplantation 2011;91(7):798—803. [10] Afessa B, Litzow MR, Tefferi A. Bronchiolitis obliterans and other late onset non-infectious pulmonary complications in hematopoietic stem cell transplantation. Bone Marrow Transplant 2001;28:425—34. [11] Yoshihara S, Yanik G, Cooke KR, Mineishi S. Bronchiolitis obliterans syndrome (BOS). Bronchiolitis obliterans organizing pneumonia (boop), and other late-onset non-infectious pulmonary complications following allogeneic hematopoietic stem cell transplantation. Biol Blood Marrow Transplant 2007;13(7):749—59.

G.C. Colin ∗ , B. Ghaye , E. Coche Service d’imagerie médicale, cliniques universitaires St-Luc, université catholique de Louvain, avenue Hippocrate 10, 1200 Bruxelles, Belgique ∗ Corresponding

author. Tel.: +32 474 42 02 62. E-mail address: [email protected] (G.C. Colin)

Tension pneumomediastinum secondary to thoracic air-leak syndrome in chronic graft versus host disease.

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