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J Thorac Cardiovasc Surg 2002;124:1230-1232
© 2002 The American Association for Thoracic Surgery
Brief Communications |
From the Departments of Vascular Radiology,a Thoracic Surgery,b and Pathology,c Hospital "Princeps d'Espanya," Barcelona, Spain.
Received for publication Feb 7, 2002. Accepted for publication Feb 18, 2002. Address for reprints: Department de Radiologia Vascular, Hospital "Princeps d'Espanya," Feixa Llarga s/n, 08907—L'Hospitalet de Llobregat, Barcelona, Spain.
Acute hemothorax caused by intercostal artery aneurysm rupture is a life-threatening complication of type I neurofibromatosis (NF I), which was successfully treated with percutaneous embolization.
Clinical summary
A 44-year-old man with NF I had sudden pleuritic pain and dyspnea 36 hours before admission to our hospital. Previously, he had been admitted to another center, where massive hematic pleural effusion was detected, and chest tube drainage yielded 1800 mL of blood. There was no past history of trauma, surgical intervention, or rib fracture.
On arrival at our hospital, the patient was conscious and had an arterial pressure of 114/69 mm Hg, a heart rate of 120 beats/min, an oxygen saturation of 92%, a hematocrit value of 21%, and a hemoglobin value of 7.1 g/dL. The chest x-ray film showed massive left pleural effusion and severe high dorsal scoliosis (Figure 1). Auscultation revealed left hemithorax hypophonesis.
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, B).
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Histologic study of a fragment of tissue adjacent to the aneurysm identified pseudoneoplastic mesenchymal proliferation that tested positive for CD34 and S100 markers, which is consistent with the diagnosis of neurofibroma plexiforme without atypia.
The patient was discharged from the hospital 10 days later, after a favorable postoperative period, and remains free of symptoms at 19 months.
Discussion
Von Recklinghausen disease or NF I is a hereditary autosomal dominant disorder originating in the neuroectoderm and mesoderm. It is expressed in approximately 80% of those who have the gene, and it has an incidence of about 1:3000. NF I is characterized by abnormal cutaneous pigmentation and multiple skin tumors, and it is frequently associated with scoliosis and other skeletal abnormalities. The incidence of vascular lesions has been reported to be only 3.6%, mostly vascular stenoses and aneurysms.
In addition to the present case, the literature contains 22 other cases of massive intrathoracic hemorrhage in patients with NF I, 1-9 including a series of 12 cases reported by Miura and colleagues 3 in Japan. Only 2 presented as hemomediastinum, 7,9 and the others presented as hemothorax. 1-6,8
The causes described in these cases are diverse and can be divided into 2 groups: bleeding by vascularized tumors of mesenchymal origin, such as ganglioneuromas or neurofibromas, 1,2,8 and bleeding caused by rupture of weak medium- to large-caliber arteries. 2-7,9 According to Greene and coworkers, 10 these arteries are either surrounded by neurofibromatous or ganglioneuromatous tissue or have weak walls caused by intimal proliferation, thinning of the muscle layer, and fragmentation of the elastic layer, making them susceptible to formation of aneurysms.
The arteries where bleeding originates are located in the mediastinum, in the region of the thoracic cage or in the diaphragm. The subclavian (7 cases) or intercostal (6 cases) arteries are most often affected, but other affected arteries, including the tyrocervical trunk, the internal thoracic artery, the phrenic artery, and the left vertebral artery arising directly from the aorta, have also been reported.
Initially, the bleeding is extrapleural, but it does not remain confined to this space; the pleura ruptures, and a massive hemothorax develops. 2 The proximity to vertebral segments with severe scoliosis has suggested, in some cases, the implication of mechanical factors in the triggering of arterial rupture. 2,11
Aneurysms in NF I have been described in all the vascular territories 11-18 and are usually of the saccular type. Thread-like aneurysms are infrequent and are often poststenotic. 14
Samples of the aneurysm were not obtained in our case, but the adjacent tissue was described during the operation as fungous and easy bleeding material. After histologic examination, the tissue was described as "pseudoneoplastic mesenchymal proliferation, consistent with the diagnosis of neurofibroma."
Regarding the development of the diagnostic and therapeutic process, we highlight the importance of fast action once the hemothorax is detected, in spite of transitory stable intervals that might mask the severity of the situation. 3 When clinical signs suggest acute massive bleeding, which has been localized, confirmed by diagnostic puncture in the pleural space, arteriography should be carried out to determine the position of the lesion. The options for hemostasia by the percutaneous route, whether through embolization or covered prosthesis, should then be considered according to the caliber of the vessel and the collateral circulation.
When feasible, sandwich aneurysm embolization is faster and cheaper than filling the cavity with multiple coils. 5 In this case the difficulty in progressing the guide wire beyond the aneurysm, along with the knowledge of vascular fragility in this type of lesion, compelled us to use the second option. Obviously, sealing of the bleeding focus should always precede hemothorax decompression with a chest tube to avoid quick exsanguination. 1,3
Surgical repair is aggressive and complex, and reconstruction of vessels is limited by arterial fragility. 12 We believe surgical intervention should be a second option for use when percutaneous treatment is not totally effective. An operation can also be used as complementary therapy, as in the case of our patient, who required decompression of the lung constricted by the hematoma.
Finally, prevention of the vascular complications in NF I is highly desirable, more because of severity of complications than frequency. Various diagnostic methods can be used for the screening and periodic control of large- and medium-caliber vessels, including Doppler echocardiography, magnetic resonance angiography, and computed tomographic angiography. 13
References
This article has been cited by other articles:
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  S Y Sundram-Novelend, D S Appleton, N J Screaton, R J Davies, and E R Chilvers Spontaneous rupture of an intercostal artery aneurysm during air flight Thorax, March 1, 2008; 63(3): 294 - 294. [Full Text] [PDF]
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 N. P. Conlon, K. C. Redmond, and L. A. Celi Spontaneous Hemothorax in a Patient With Neurofibromatosis Type 1 and Undiagnosed Pheochromocytoma Ann. Thorac. Surg., September 1, 2007; 84(3): 1021 - 1023. [Abstract] [Full Text] [PDF]
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 L. M Fedoruk, J. English, and G. J Fradet Spontaneous Hemothorax and Neurofibromatosis: A Review of a Lethal Combination Asian Cardiovasc Thorac Ann, August 1, 2007; 15(4): 342 - 344. [Abstract] [Full Text] [PDF]
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I. Matsumoto, Y. Ohta, Y. Tsunezuka, M. Tamura, M. Oda, and G. Watanabe Treatment for Intercostal Arterial Aneurysm in Neurofibromatosis Type 1 Asian Cardiovasc Thorac Ann, February 1, 2007; 15(1): e16 - e19. [Abstract] [Full Text] [PDF]
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