Spontaneous Hemothorax Caused by a Ruptured Intercostal Artery Aneurysm in Von Recklinghausen’S Neurofibromatosis

W.C. Chang, H.H. Hsu, H. Chang, et al

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SPONTANEOUS HEMOTHORAX CAUSED BY A RUPTURED INTERCOSTAL ARTERY ANEURYSM IN VON RECKLINGHAUSEN’S NEUROFIBROMATOSIS

Wei-Chou Chang,1 Hsian-He Hsu,1 Hung Chang,2 and Cheng-Yu Chen1

Abstract: Aneurysms arising from an intercostal artery are very rare vascular malformations in von Recklinghausen’s neurofibromatosis, which often have a silent clinical presentation and are difficult to diagnose before rupture. We report a case of von Recklinghausen’s neurofibromatosis with massive hemothroax caused by spontaneous rupture of an intercostal artery aneurysm in a 29-year-old man. The diagnosis was eventually confirmed by percutaneous angiography and treated with endovascular embolization. During a 10-month follow-up period, the patient had a satisfactory recovery. This case illustrates that angiography and possible endovascular embolization should be the first strategy in managing hemothorax in patients with von Recklinghausen’s disease. Key words: Aneurysm, ruptured; Embolization, therapeutic; Hemothorax; Neurofibromatosis 1; Thoracic arteries

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Von Recklinghausen’s neurofibromatosis, also named neurofibromatosis type 1 (NF-1), is a well-recognized Case Report entity that is characterized by numerous neuro- fibromas, spots of abnormal cutaneous pigmentation, A 29-year-old man presented at the emergency room and a variety of other dysplastic abnormalities of with sudden onset shortness of breath and severe the skin, nervous system, bones, endocrine organs and retrosternal pain radiating to his back. One week prior blood vessels.1,2 Vascular abnormalities in patients to admission, dull discomfort upon breathing was with NF-1 have been described for large, medium- noted, which was not affected by position or physical sized and small muscular arteries in the intracranial, activity. He had no cough at that time. Over the next thoracic and abdominal circulation, the most common few days, the pain progressed and involved his back of which are arterial stenoses or aneurysms involving and right shoulder. The pain was persistent and both kidneys.3–6 radiated to the right chest wall. The pain became more Due to its low incidence of 3.6% in NF-1 patients intense and suddenly exacerbated in the afternoon with vascular involvement, the association of arterial of the day of admission. There was no history of recent aneurysms has rarely been described.3,7 These trauma or seizure. Von Recklinghausen’s neurofibro- aneurysms can potentially rupture and sometimes pose matosis had been diagnosed in this patient at the age a diagnostic and therapeutic dilemma. We report a of 8 years, with the classic symptoms of café-au-lait case of NF-I with rupture of the intercostal artery spots, axillary freckling and plexiform neurofibromas causing subsequent massive hemothorax. To our on the trunk and extremities, together with a familial knowledge, this is the third reported case of treatment history of neurofibromatosis. of this condition by endovascular embolization, and Vital signs on arrival were systolic blood pressure the first such report from Taiwan. 110 mm Hg, pulse rate 112 beats/minute and respiration

1Department of Radiology and 2Division of Thoracic Surgery, Tri-Service General Hospital and National Defense Medical Center, Taipei, Taiwan. Received: 24 May 2004 Revised: 23 June 2004 Accepted: 7 September 2004 Reprint requests and correspondence to: Dr. Hsian-He Hsu, Department of Radiology, Tri-Service General Hospital, 325, Sec. 2, Cheng-Kung Road, Taipei 114, Taiwan.

286 J Formos Med Assoc 2005 • Vol 104 • No 4 Spontaneous Hemothorax in von Recklinghausen’s Neurofibromatosis rate 24 breaths/minute. Physical examination dis- Since the chest CT findings and thoracocentesis closed tachycardia, normal blood pressure and short- were consistent with right-sided hemothorax (Fig. 2), ness of breath. Breath sounds were decreased over emergent thoracotomy was carried out under the the lower one-half of the right hemithorax, with diagnosis of hemothorax of unknown origin. This dullness to percussion and decreased tactile and vocal procedure failed to identify the cause or to stop the fremitus over the same area. bleeding. Surgery was terminated since there was Laboratory data included the following: hemo- no evidence of pulsatile hemorrhage and definitive globin, 13.4 g/dL; white blood cell count, 11,600 cells/ bleeding points could not be identified during mm3 (differential, normal); platelet count, 391,000/ surgery. mm3; normal urinalysis data; and normal values for Emergent aortography was performed to locate the creatinine, prothrombin time and activated partial origin of bleeding. A pool of the contrast medium thromboplastin time. The chest radiographs including was found around the right seventh intercostal artery, anterioposterior and decubitus views showed a large and then selective right seventh intercostal arterio- right pleural effusion with near complete opacification graphy disclosed a ruptured arterial aneurysm (Fig. of the right lower lung zone (Fig. 1). 3). Subsequently, endovascular embolization was Chest computed tomography (CT) scan revealed a performed with a 5 mm coil (Occluding Spring large amount of right-sided high-attenuated fluid Emboli, Cook, Bloomington, IN, USA), which stopped collection associated with a well-circumscribed high- the bleeding successfully. density mass. The mediastinal structures were pushed to the left. These findings were suggestive of hemo- thorax and hematoma formation. Thoracocentesis drained 1500 mL of bloody fluid with a hemoglobin concentration of 15.8 g/dL. The hemoglobin con- centration of the blood subsequently decreased from 13.4 g/dL to 10.2 g/dL.

Fig. 2. Computed tomography scan of the chest reveals a large amount of right-sided high-attenuated fluid collection associated with a well-circumscribed high-density mass, consistent with hemothorax and hematoma formation. The mediastinal structures were pushed to the left.

Fig. 1. Chest radiography shows a large right pleural effusion Fig. 3. Selective angiography of the right seventh inter- with near complete opacification of the right lower lung costal artery reveals the aneurysm, and extravasation of field. the contrast medium displaying the origin of hemorrhage.

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Two weeks later, the patient had an uneventful re- Angiography with endovascular coil embolization covery and he remained well in the ensuing 10 months. should be the preferred method in patients with NF-1, not only because it precisely demonstrates a wide range of vascular malformations but also because it obviates Discussion the need for surgery.5,7,13 Other imaging studies, including Doppler echocardiography, CT angiography Spontaneous hemothorax is an infrequent clinical or even magnetic resonance angiography were much entity that is usually caused by coagulopathy, hyper- less sensitive to detect the presence of arterial aneurysms. vascular tumors, remote traumatic injury or bleeding These methods can be used for a non-invasive screening, from a primary non-traumatic vascular source.8 Von if clinically necessary.5,13 To control bleeding in this Recklinghausen’s neurofibromatosis associated with a emergent condition with a possible aneurysm rupture, ruptured intrathoracic artery aneurysm is an extremely as in our patient, we advocate percutaneous angiography rare condition. Spontaneous rupture and hemorrhage prior to surgical intervention. of these aneurysms is usually unexpected and may In cases of NF-1 with massive spontaneous hemo- sometimes represent a more serious complication. thorax due to a ruptured intercostal artery aneurysm, There are 2 major distinct pathologic mechanisms early recognition of the potentially fatal but curable explaining this condition: 1) vessel wall replaced complication of von Recklinghausen’s disease is by neurofibromatosis tissue such as schwannoma, essential. Appropriate treatment is paramount for the neurofibroma, or neurofibrosarcoma; or 2) aneurys- patient’s survival. The utilization of the endovascular mal formation caused by vascular dysplasia of small techniques appears to be the first choice in managing vessels with disarrangement of the smooth muscle and spontaneous hemothorax, especially when the patient fragmentation of the elastic layer.2,4,5,9 has a history of von Recklinghausen’s disease. Spontaneous hemothorax caused by a ruptured intrathoracic artery aneurysm may manifest with a variety of symptoms. For example, rupture into the References pulmonary parenchyma may cause hemoptysis,10 or rupture into the mediastinum may mimic a dissecting 1. Brasfield RD, Das Gupta TK: Von Recklinghausen's disease: a aortic aneurysm accompanied by radiating back pain clinicopathological study. Ann Surg 1972;175:86-104. and subsequent shock.11,12 Our patient had dyspnea 2. Miura H, Taira O, Uchida O, et al: Spontaneous haemothorax and chest pain. CT scan could only reveal massive associated with von Recklinghausen's disease: review of spontaneous hemothorax and led to a suspicion of occurrence in Japan. Thorax 1997;52:577-8. intrapulmonary bronchial artery or extrapulmonary 3. Huffman JL, Gahtan V, Bowers VD, et al: Neurofibromatosis intercostal artery aneurysm rupture due to a history and arterial aneurysms. Am Surg 1996;62:311-4. of congenital disease. 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