Dieulafoy’s disease of the bronchus: report of a case and review of the literature

Abstract

BackgroundDieulafoy’s disease is a vascular anomaly characterized by the presence of a tortuous dysplastic artery in the submucosa. Although frequently occurring in the gastrointestinal tract, multiple cases of Dieulafoy’s disease in the bronchus have been reported in the literature.Methods and resultsWe report a case of a 15-year-old boy suffering recurrent massive hemoptysis. Bilobectomy stopped bleeding after unsuccessful treatment with embolization of bronchial artery.ConclusionIt is concluded a congenital origin of this disease. Angiography and endobronchial ultrasonography can be used to diagnose Dieulafoy’s disease of bronchus whereas bronchoscopy biopsy should be avoided. Surgery is needed when embolization fails.Electronic supplementary materialThe online version of this article (doi:10.1186/s13019-014-0191-8) contains supplementary material, which is available to authorized users.

Dieulafoy’s disease of the bronchial tree: a case report

“…Dieulafoy's disease of the bronchus may have a congenital origin, arising from either the systemic or the pulmonary circulation. 2 Spontaneous bleeding has been described in these cases, but bleeding in such cases often occurs after a biopsy on a lesion that has not been diagnosed as a vascular anomaly. Age and tobacco use have an influence on occurrences of this disease.…”

“…3 The characteristics of the lesion are nonspecific, but it can be suspected when a small (usually less than 1 cm) sessile non-pulsatile nodular lesion with a white cap and apparently normal mucosa is seen. 2 It has been suggested that, after the diagnosis has been made, angiography and embolization can be the preferred treatment 5,6 and that surgical resection would only be needed in a few cases. 4 However, the failure rate of embolization is not negligible, whereas surgery alone or after failure of embolization has had a success rate of nearly 100% in all reports.…”

Dieulafoy’s disease with mediastinal arteriovenous malformation

“…Of the few reports on bronchial Dieulafoy’s disease causing haemorrhage, there are cases when active bleeding was triggered by iatrogenic injury, including bronchoscopic biopsy1 and saline wash 3. The effects of haematopoietic factor dysregulation, including qualitative and quantitative platelet defects, in acute leukaemia could have contributed to a heightened risk of bleeding in our patient.…”

“…Although usually occurring in the gastrointestinal tract, cases of bronchial Dieulafoy’s disease have been reported 1. Mediastinal AVM consists of multiple direct communications between arterial and venous branches, predominately in the posterior mediastinum 2.…”

“…Selective angioembolisation should be considered as the first-line management for each of these two anomalies due to its minimal invasiveness,1–3 with angiography done prior to determine locality of the abnormal vessels 1. Surveillance of embolised vessels thereafter is recommended due to possible revascularisation and neovascularisation leading to failure of initially successful embolisation.…”