Dieulafoy’s disease of the bronchus: a possible mistake
© Barisione et al.; licensee BioMed Central Ltd. 2012
Received: 30 July 2012
Accepted: 29 October 2012
Published: 8 November 2012
We present a case of a 57 year old woman who suffered from massive hemoptysis; she was sent to our Department for a suspect neoformant lesion. We assumed it might be a Dielafoy’s disease and proceeded with an imaging study that confirmed the diagnosis. After embolization the patient no longer showed signs of bleeding. In brief, we concluded that whenever there is a suspect of Dielafoy’s disease, the biopsy has to be avoided.
KeywordsDieulafoy’s disease Massive hemoptysis Vascular lesion
Dieulafoy’s disease of the bronchus is supposed to be a very rare disease. In fact only few cases are reported in literature [4;7]. This condition should be clinically suspected in heavy smokers with recurring and unexplained episodes of massive hemoptysis. The bleeding can occur immediately after the biopsy or delayed up to 12 days. The diagnosis can be made through imaging. Angiographic images document that this vascular malformation is based on a left-to-right shunt, with a bronchial artery draining into a pulmonary artery. Endobronchial ultrasound may be helpful in detecting the vascular nature of the lesion.
Dieulafoy’s disease is an extremely rare vascular anomaly, characterized by the presence of a dysplastic artery in the sub-mucosa. At present, there are few proven cases reported in literature[2–4]. The pathogenesis of Dieulafoy’s disease remains unclear. This anomaly was first reported in the gastrointestinal tract; more recently it has been described also in the respiratory tract. While in the gastrointestinal tract the bleeding is often spontaneous but also fatal, in the bronchial tree profused bleeding often occurs after a biopsy. However, cases of spontaneous bleeding have also been described. It is still unknown whether the origin of the anomaly is congenital or acquired, but age and tobacco use are thought to have an influence on the occurrence of the disease. The trigger factor of the vessel rupture is unknown. Furthermore, the nature of the bleeding vessel remains controversial. Dieulafoy’s disease of the bronchus is probably underestimated. Massive hemoptysis is a life threatening condition associated with a mortality rate exceeding 50% in the absence of adequate treatments[7, 8]. The characteristics of the lesion are non-specific, but in the presence of a small (usually <1 cm), sessile, non pulsating nodular lesion, often with a white cap, and apparently normal mucosa, Dieulafoy’s disease should be taken into account. The respiratory epithelium shows focal squamous metaplasia and diffused thickening of the basal membrane. In bronchial Dieulafoy’s disease, selective embolization has been suggested as a method for stopping the bleeding[9, 10] and only in few cases the patient requires surgical resection.
In brief, Dieulafoy’s disease of the bronchus is more frequent than thought, so this diagnostic option should be considered when there is a patient with recurring massive hemoptysis, which cannot be explained otherwise. Obviously, in this case the biopsy has to be avoided even when no active bleeding is evident.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
- Gurioli C, Casoni GL, Gurioli C, Tomassetti S, Romagnoli M, Ravaglia C, Poletti V: Endobronchial ultrasound in Dieulafoy’s disease of the bronchus: an additional application of EBUS. Monaldi Arch Chest Dis. 2010, 73 (4): 166-168.PubMedGoogle Scholar
- Parrot A, Antoine M, Khalil A, Théodore J, Mangiapan G, Bazelly B, Fartoukh M: Approach to diagnosis and pathological examination in bronchial Dieulafoy disease: a case series. Respir Res. 2008, 9: 58-10.1186/1465-9921-9-58.PubMed CentralView ArticlePubMedGoogle Scholar
- Loschhorn C, Nierhoff N, Mayer R, Zaunbauer W, Neuweiler J, Knoblauch A: Dieulafoy’sdisease of the lung: a potential disaster for the bronchoscopist. Respiration. 2006, 73: 562-565. 10.1159/000088059.View ArticlePubMedGoogle Scholar
- Gharagozloo F, Rennert D, Margolis M, Tempesta B, Schwartz A, Cole V, Wang K-P: Dieulafoy Lesion of the Bronchus: Review of literature and report of the 13thcase. J Bronchol. 2008, 15: 38-40. 10.1097/LBR.0b013e3181606114.View ArticleGoogle Scholar
- Schmulewitz N, Baillie J: Dieulafoy lesions: a review of 6 year experience at a tertiary referral center. Am J Gastroenterol. 2001, 96 (6): 1688-1694. 10.1111/j.1572-0241.2001.03922.x.View ArticlePubMedGoogle Scholar
- Sweerts M, Nicholson AG, Goldstraw P, Corrin B: Dieulafoy’s disease of the bronchus. Thorax. 1995, 50 (6): 697-698. 10.1136/thx.50.6.697.PubMed CentralView ArticlePubMedGoogle Scholar
- Corey R, Hla KM: Major and massive hemoptysis: reassessment of conservative management. Am J Med Sci. 1987, 294 (5): 301-309. 10.1097/00000441-198711000-00003.View ArticlePubMedGoogle Scholar
- Sehhat S, Oreizie M, Moinedine K: Massive pulmonary haemorrhage: surgical approach as choice of treatment. Ann ThoracSurg. 1978, 25 (1): 12-15. 10.1016/S0003-4975(10)63478-9.View ArticleGoogle Scholar
- Bhatia P, Hendy MS, Li-Kam-Wa E, Bowyer PK: Recurring embolotherapy in Dieulafoy’s disease of the bronchus. Can Respir J. 2003, 10: 331-333.PubMedGoogle Scholar
- Hope-Gill B, Prathibha BV: Bronchoscopic and angiographic finding in Dieulafoy’s disease of the bronchus. Hosp Med. 2002, 63: 178-179.View ArticlePubMedGoogle Scholar
- Savale L, Parrot A, Khalil A, Antoine M, Théodore J, Carette MF, Mayaud C, Fartoukh M: Cryptogenetic hemoptysis From a benign to a life-threatening pathological vascular condition. Am J Respir Crit Care Med. 2007, 175: 1181-1185. 10.1164/rccm.200609-1362OC.View ArticlePubMedGoogle Scholar
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