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Ann Thorac Surg 2007;84:1021-1023
© 2007 The Society of Thoracic Surgeons
a Department of Anesthesia and Intensive Care, Mater Misericordiae University Hospital, Dublin, Ireland
b Department of Cardiothoracic Surgery, Harefield Hospital, London, United Kingdom
c Department of Anesthesia and Intensive Care, Dunedin Hospital, Dunedin, New Zealand
Accepted for publication April 5, 2007.
* Address correspondence to Dr Conlon, Department of Anesthesia and Intensive Care, Mater Misericordiae University Hospital, Eccles St, Dublin, 7, Ireland (Email: tallniamh{at}hotmail.com).
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Abstract |
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Introduction |
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The vasculopathy of NF-1 can result in the development of arterial aneurysms that may rupture with potentially life-threatening consequences. We report the case of a 40-year-old man with spontaneous rupture of his right internal mammary artery causing massive hemothorax. He was discovered postoperatively to have a concurrent pheochromocytoma, which likely contributed to the rupture of the vessel wall.
A 40-year-old man presented to the emergency department after sudden onset of severe abdominal and right shoulder pain, with collapse. He had known NF-1, but no other notable medical history, no history of trauma, and no previous thoracic surgery. Blood pressure on admission was 90/35 mm Hg with a tachycardia of 140 beats per minute. Respiratory rate was 36 breaths per minute and oxygen saturation was not recordable. Chest expansion was asymmetrical, with decreased air entry and dullness to percussion over the right hemithorax and tenderness in the right upper quadrant.
Initial blood tests reported a hemoglobin of 7.6 g/dl, platelets of 328 x 109 L–1, and normal coagulation. Arterial blood gas analysis showed a metabolic acidosis.
Sinus tachycardia was evident on a 12-lead electrocardiogram with left ventricular hypertrophy by voltage criteria. Chest roentgenogram confirmed a right-sided opacification consistent with a hemothorax (Fig 1), with needle thoracocentesis aspirating bright red blood. Abdominal ultrasound suggested a retroperitoneal collection with a deteriorating hemodynamic state; therefore, a blood transfusion and vasopressors were commenced, and the trachea was intubated and the lungs were ventilated. Transesophageal echocardiography was performed that excluded aortic dissection and showed marked concentric left ventricular hypertrophy with low stroke volume consistent with the shocked state.
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A fifth intercostal space lateral thoracotomy was performed with 5 L of blood and clot evacuated. The source of bleeding was identified at the apex of the right hemithorax. Rupture had occurred just distal to where the right internal mammary artery branched from the subclavian artery. No other aneurysms were identified. Gross bullous changes were noted at the apex of the right lung. Marked hypotension persisted until arterial ligation and transfixion with a 2-0 Vicryl suture (Ethicon, Somerville, NJ) was achieved, after which the patient was stabilized and vasopressors were weaned. At this stage, 16 units of red cells, 6 units of fresh frozen plasma, and 2 units of platelets had been transfused.
Postoperatively in the intensive care unit, the arterial blood pressure became very labile with systolic pressures swinging from 30 to 300 mm Hg, with exaggerated responses to vasopressors and antihypertensive agents. Review of the preoperative abdominal computed tomographic scan (Fig 2) revealed a 2-cm adrenal mass suspicious for a pheochromocytoma. For the next few days inotropic support was weaned and the patient was commenced on phenoxybenzamine and later on metoprolol. The patient had bilateral pneumothoraces develop on postoperative day 2, presumed secondary to rupture of bullae noted at surgery. He had an otherwise uneventful intensive care unit stay and was moved to nonintensive care after 5 days. Then he was discharged home a week later. Histology of the internal mammary artery showed hyaline intimal thickening, elastic fibers and membrane disruption, loss of the media, and normal adventitia with aneurysm formation.
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Comment |
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The incidence of pheochromocytoma in patients with NF-1 is 0.1% to 5.7%. Clinical presentation can vary considerably with sustained hypertension in 50% to 60% of patients, and paroxysmal hypertension in 30% [6]. It is likely that hypertension in our patient contributed to the rupture of the aneurysmal mammary artery. The paroxysmal hypertension may not have been apparent on presentation due to the massive blood loss, but once the bleeding was controlled it became manifest. The marked left ventricular hypertrophy on echocardiography is also suggestive of prolonged hypertension, although left ventricular hypertrophy has also been reported as part of a cardiomyopathy in association with neurofibromatosis alone [7].
Both pulmonary fibrosis and cystic lung disease in the form of honeycomb lung structure or large apical cysts [8] are also associated with NF-1.
In conclusion, this case demonstrates three of the rarer features of neurofibromatosis type-1: (1) vasculopathy, (2) pheochromocytoma, and (3) cystic lung disease, all occurring in the same patient and presenting as a life-threatening arterial aneurysm rupture and bleed.
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This article has been cited by other articles:
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  M. Rapp, J. Martino, and G. B. Allen A 35-Year-Old Patient With Midscapular Pain and Hypertension Chest, July 1, 2011; 140(1): 258 - 261. [Full Text] [PDF]
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