A 46-year-old man presented with a history of progressive breathlessness on exertion over a period of several months plus an acute onset left calf pain. He was an ex-smoker and worked in the Information Technology industry. His physical examination revealed slight tenderness of the left calf. His chest x-ray revealed bilateral hilar prominence and loss of volume in the upper lobes with patchy faint opacities. Computed tomography pulmonary artery demonstrated widespread pulmonary thromboembolic disease with multiple segmental occlusions. Bronchocentric areas of ground-glass opacification and fibrosis with extensive mediastinal lymphadenopathy were additionally noted. Unusually, prominent bronchial vessels originating from the aorta were identified within the subcarinal space (Fig. 1). A diagnosis of chronic or subacute pulmonary emboli (PE), on a background of chronic sarcoidosis with systemic collateral vessel formation, was made. One month later, after a period of anticoagulation and clinical improvement, bronchoscopy with endobronchial ultrasound (EBUS) was performed to confirm, pathologically, the diagnosis of sarcoidosis. Standard prebronchoscopy precautions were undertaken with regard to warfarin withdrawal and administration of low–molecular weight heparin as per national guidelines.1
During standard flexible bronchoscopy, intense mucosal vascularity was noted. Given this finding, the presence of significant systemic collateral vessel formation and recent anticoagulation therapy, transbronchial lung biopsy was deemed a significant risk for bleeding and was not performed. Examination with EBUS facilitated sampling of a pathologically enlarged right paratracheal lymph node. However, EBUS also confirmed an engorged bronchial artery encircling a subcarinal lymph node, preventing further pathologic sampling; thrombus was also visualized in the right pulmonary artery (Figs. A, B).
This case provides a number of interesting discussion points. First is the possible association between sarcoidosis and PE. Two large studies from the United States and the United Kingdom have both provided evidence in favor of such an association. Swigris et al2 analyzed death certificate information from over 46 million decedents in the United States and found a 2-fold increase in the risk of PE among decedents with sarcoidosis compared with the background population. This risk was independent of sex, race, and age. In the United Kingdom, Crawshaw and colleagues analyzed the risk of PE in a cohort of 1000 patients with sarcoidosis compared with a reference population of over 500,000 patients using a regional National Health Service hospital admissions database. This study also demonstrated a 2-fold increase in the risk of PE among patients with sarcoidosis.3 The authors postulate that the proinflammatory and procoagulant activity demonstrated in blood and bronchoalveolar lavage samples from patients with sarcoidosis may underpin the apparent predisposition to PE.
Second, this case reinforces the need for a thorough assessment of individual patient risk for all bronchoscopic procedures, including where different sampling methods are planned within the same procedure. The risk of severe bleeding during bronchoscopy is highest following transbronchial lung biopsy compared with endobronchial biopsy,1 but lower for EBUS-guided transbronchial needle aspiration (EBUS-TBNA). In this case, all 3 of these sampling methods have the potential to provide a pathologic diagnosis of sarcoidosis. Given the concerns over bleeding described above, it is appropriate to select the technique with the lowest risk of bleeding, EBUS-TBNA, and omit sampling by other techniques. Furthermore, this case also illustrates the benefit of detailed anatomic imaging before bronchoscopy, for both planning of the bronchoscopic approach and risk assessment. The presence on CT of significant systemic vessels within the subcarinal space highlighted the need for extra caution during bronchoscopy and dictated the need for ultrasound-guided TBNA rather than conventional TBNA for any attempted nodal sampling. The findings on CT coupled with the endobronchial evidence of mucosal vascularity significantly altered the approach to this patient, illustrating the impact of preprocedural and intraoperative findings on the decision-making process.
Finally, this case provides interesting and novel EBUS images, namely a bronchial artery encircling a subcarinal lymph node and PE in the right pulmonary artery. Casoni et al4 and Senturk et al5 have previously reported cases of PE identified with EBUS; in a single patient by Casoni and in a case series of 8 patients by Senturk. Furthermore, Aumiller and colleagues undertook a pilot study in 32 patients with CT-confirmed central PE. EBUS was performed at the bedside within 24 hours of diagnosis and detected 97/101 emboli seen on CT.6 At least 1 clot was seen in every patient. Overall, although this is an interesting and novel application of EBUS, a role within routine clinical practice has yet to be demonstrated.
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