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J Thorac Cardiovasc Surg 2004;128:780-782
© 2004 The American Association for Thoracic Surgery

Brief communication

Tracheal obstruction from mediastinal arteriovenous malformation

Hermes C. Grillo, MDa,c,*, Christos A. Athanasoulis, MDb,d

a Division of General Thoracic Surgery, Massachusetts General Hospital, Boston, Mass, USA
b Division of Vascular Radiology, Massachusetts General Hospital, Boston, Mass, USA
c Department of Surgery, Harvard Medical School, Boston, Mass, USA
d Department of Radiology, Harvard Medical School, Boston, Mass, USA

Received for publication October 6, 2003; accepted for publication March 4, 2004.

* Address for reprints: Hermes C. Grillo, MD, Massachusetts General Hospital, Blake 1570, 55 Fruit St, Boston, MA 02114, USA
pguerriero{at}partners.org

Vascular malformations that appear as mediastinal masses are extremely uncommon.1-3 In highly unusual instances, a major mediastinal arteriovenous malformation (AVM) may extend into the trachea as an obstructive mass. These lesions are developmental anomalies that are distinct from mediastinal or tracheal hemangiomas.4 They do not exhibit endothelial cell proliferation as hemangiomas do, nor do they regress with age.

Clinical summaries

Patient 1
A 25-year-old man had undergone right upper and middle lobectomies at age 4.5 years for "multiple hemangiomas," with residual disease that encroached on the superior vena cava. He reported progressive dyspnea on exertion, increasing fatigue, cough productive of mucus, and three episodes of pneumonia, but no hemoptysis.

Chest radiographs showed clear lungs and a mediastinal mass projecting to the right. Bronchoscopy revealed an obstructing lesion 7.5 cm below the cricoid, composed of large pulsating vessels. Biopsy was inadvisable. Diagnostic angiography through the femoral artery revealed a huge vascular malformation (Figure 1). In five separate sessions, major arterial branches arising from the internal thoracic, thyrocervical, bronchial, inferior phrenic, and right coronary arteries were embolized with balloons and coils (CAA). This reduced the size of the lesion, decreased blood flow, and markedly diminished visible pulsations. Submucosal vascularity was unchanged.



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  		Figure 1. Large, diffuse mediastinal AVM in patient 1. A, Thoracic aortogram, anteroposterior view, early arterial phase. Large tortuous tributaries from right internal thoracic artery (arrows)..and left bronchial artery (curved arrow)..supply AVM. B, Thoracic aortogram, anteroposterior view, late arterial phase. Tortuous, serpigenous vessels are noted throughout mediastinum. Contrast-filled arterial sac, supplied by right internal thoracic artery, protrudes like mass lesion into trachea (arrowhead)...C, Thoracic aortogram, lateral view, late arterial phase. Arrowhead..points to masslike vascular lesion protruding into trachea. D, Anteroposterior view, arterial phase. Enlarged right phrenic artery (arrows)..supplies mediastinal AVM. E, Arterial phase. Thoracic aortogram after multiple embolizations. Tributaries from right internal thoracic artery (arrows)..and from left bronchial artery (curved arrow)..have been occluded with detachable balloons and spring coils. Black arrowhead..points to detachable balloons occluding vascular structure, which protruded into tracheal lumen.

 
Resection of the trachea was accomplished through a collar incision plus upper sternotomy (HCG). Extensive tortuous vessels penetrating the tracheal walls were meticulously ligated until the segment containing the obstructing lesion could be mobilized sufficiently to allow resection (1.5 cm. length) with end-to-end anastomosis. Complete extirpation of the mediastinal malformation was impossible.

The specimen showed partly thrombosed channels with thick, artery-like walls. The patient was fully relieved of respiratory symptoms and has remained symptom free for 20 years.

Patient 2
A 25-year-old woman had a 5-year history of worsening dyspnea on exertion. A nodule removed from her right supraclavicular region and another from the left thigh were classified as cavernous hemangiomata. Chest radiographs showed a large superior anterior mediastinal mass extending from neck to aortic arch (Figure 2, A... Tomography showed localized nodular indentation of the midtrachea at the thoracic inlet (Figure 2, B... Bronchoscopy revealed 40% obstruction of the trachea by a pulsating vascular mass 5 cm distal to the vocal cords.



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  		Figure 2. A, Chest radiograph, posteroanterior view, in patient 2. Mass of AVM is most prominent in left side of mediastinum. B, Tomogram of trachea demonstrating intraluminal protrusion of AVM (arrow)..

 
Angiography demonstrated a large vascular malformation (Figure 3). Feeding vessels included both subclavian arteries, internal thoracic arteries, thyrocervical trunks, and at least one bronchial artery. Extensive and early shunting into the superior vena cava and brachiocephalic veins was observed. A conservative course was elected at that time. The patient was followed up in several other hospitals. Her dyspnea later worsened, persistent cough appeared, cervical fullness increased and further narrowing of the trachea was observed.



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  		Figure 3. Large diffuse mediastinal AVM in patient 2. A and B, Thoracic aortogram, anteroposterior view. Early phase (A) and late venous phase (B). There is arteriovenous shunting. AVM involves superior mediastinum. Vascular, masslike structure is noted to right of trachea (arrows)...Tracheal lumen is constricted (open arrowhead)...C and D, Mediastinal AVM is supplied from branches of right (C) and left (D) subclavian arteries.

 
Twenty years after the patient was first seen, she underwent right thoracotomy elsewhere, without benefit. This was followed by tracheostomy for severe respiratory distress. Right hemiparesis followed embolization. In yet another medical center, further embolizations were done. She had severe tracheal bleeding and died of airway hemorrhage.

Discussion

Embolization of AVMs is effective only when the core of the lesion or nidus is occluded, commonly with small-caliber particles (polyvinyl alcohol) or with liquid embolic materials (alcohol or plastics). Occlusion of tributaries to an AVM with balloons or coils does not ablate the lesion. If as many tributaries as possible are embolized, however, the flow to the lesion may be substantially reduced. The risk of tissue necrosis in the mediastinum would have been high with particulate or liquid embolic media. Rather, we attempted to reduce blood flow as much as possible to facilitate operation.

Complete surgical resection of a mediastinal AVM is rarely possible. Anti-angiogenic therapy, corticosteroid treatment, interferon administration, and intralesional sclerotherapy have not been helpful.4 A carefully planned program of embolization to reduce flow, followed by surgical excision, was effective in controlling the first patient's tracheal disease. An earlier aggressive approach for the second patient, when the tracheal lesion also appeared more localized, might have produced a similar long-term palliative result.

References

  1. Hirata S, Ogino K, Saji K. A case of mediastinal arteriovenous fistula. Classification on thoracic arteriovenous fistula and review of the literature. Nihon Kyobu Shikkan Gakkai Zassh.. 1975;13:477–481
  2. Hammerer I, Stampfel G. Ausgedehntes haemangioma racemosum des Mediastinums mit Zwerchfellparese. Ein fallbericht und beitrag zur differentialdiagnose des arteriovenösen aneurysmas im thoraxbereich. Padiatr Pado.. 1977;12:63–72
  3. Lunde P, Sörlie D, Bolz KD, Eide TJ. Huge arteriovenous malformation in the mediastinum. Scand J Thorac Cardiovasc Sur.. 1984;18:75–80
  4. Fishman SJ. Vascular anomalies of the mediastinum. Semin Pediatr Sur.. 1999;8:92–98




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