2003 Jun - Unusual Haemoptysis Case 1
Dr. Simon Ip, University Department of Medicine, Queen Mary Hospital
A 51-year-old man with good past health presented with mouthfuls of fresh haemoptysis for one day in May 2002. He also reported a similar episode a few years previously. Physical examination was unremarkable. Radiologically, there was consolidative change in the right middle lobe. Complete blood picture, biochemistry were normal; sputum smear for acid-fast bacilli and cytology were negative.
The patient's haemoptysis settled uneventfully and he was discharged home with a course of Augmentin and transamin with a view to perform bronchoscopy 1 week later. On readmission there was marked improvement in the right middle lobe consolidation.
At bronchoscopy there were blood clots visible in the basal segments of right lower lobe. Removal with suctioning caused a fair amount of bleeding and haemostasis was secured with injections of adrenaline. A mass lesion with smooth, shiny looking mucosa was seen protruding from the medial wall of the right middle lobe entrance (Fig 1).
Figure 1: mass lesion protruding from medial wall of right middle lobe entrance
Endobronchial biopsy was performed to the right middle lobe mass which resulted in torrential amount of bleeding uncontrollable by cold saline and adrenaline. He was inbubated with double lumen endotracheal tube and was transferred to intensive care unit for management. The total amount of bleeding from the right side was greater than one litre within the first few hours. Urgent computed tomography of thorax and arteriogram of bronchial and pulmonary arteries did not reveal any bleeding source. The patient was stabilized and transferred to the cardiothoracic unit of Grantham hospital. Right middle lobectomy was performed. Intraoperative stump for frozen section gave no pathological diagnosis. Pathology of the resected lung showed bronchiectasis with large ecstatic bronchi closely packed and filled with blood. Parabronchial arteries including pulmonary and bronchial arteries were prominently distended and showed medial hypertrophy. On review of the piece of tissue taken at bronchoscopy from the right middle lobe, one rather large fragment of arterial wall with discernible elastic lamina and tunica media was seen.
The patient made a full recovery post-operatively and was discharged home.
Discussion
Dieulafoy's disease was named after a French surgeon who described a vascular abnormality/malformation characterized by a dysplastic artery contiguous to the superficial epithelium of the mucosa, usually occurring in the gastrointestinal tract. The vascular lesion can arise either from the pulmonary or bronchial artery. It was reported seven times in the respiratory tract, mainly in the right lung. The preponderance on the right side may be related to the different developmental origins between the right and left lung vasculature. Dieulafoy's disease of the lung has not been described in children and the aetiology is unknown. One postulation is that underlying bronchiectasis and/or tuberculosis lead to increased oxygen requirement to a limited area, with proliferation of granulation tissue. The resulting reduction in flow through pulmonary arteries cause proliferation and dilatation of bronchial arteries. Patients usually present with recurrent haemoptysis and or pneumonia. At bronchoscopy it can either appear as a tubular bulge, mass-like lesion or endobronchial haemangioma. Biopsy of the lesion can lead to fatal bleeding. Treatment of the condition is either by embolotherapy or lobectomy. Many endobronchial pathologies such as tuberculosis can present in a similar fashion as a non-pulsatile vascular lesion and a high clinical awareness is required before taking a biopsy. Arteriographic investigations are desirable when coming across such lesions. Some would also advocate performing needle aspiration in order to avoid occurrence of fatal bleeding.
References
- Katoh O. Bronchoscopic and angiographic comparison of bronchial arterial lesions in patients with haemoptysis. Chest 1987;91:486-9
- Sweerts M, Nicholson AG, Goldstraw P, et al. Dieulafoy's disease of the bronchus. Thorax 1995;50:697-8
- Van der Werf TS, Timmer A, Zijlstra JG. Fatal haemorrhage from Dieulafoy's disease of the bronchus. Thorax 1999;54:184-5
- Park GY, Lee KY, Yoo CG, et al. Bronchoscopic findings of endobronchial vascular lesions in patients with haemoptysis. Respirology 1999;4:401-4
- Maxeiner H. Lethal haemoptysis caused by biopsy injury of an abnormal bronchial artery. Chest 2001;119:1612-5 Stoopen E, Baquera-Heredia J, Cortes D, et al. Dieulafoy's disease of the bronchus in association with a paravertebral neurilemoma. Chest 2001;119:292-4
