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DOI:10.2214/AJR.05.0367
AJR 2006; 187:W503-W506
© American Roentgen Ray Society

Case Report

Congenital Intercostal Arteriovenous Malformation

Peter P. Rivera1,2, Max K. Kole1,3,4, David M. Pelz1, Irene B. Gulka1, F. Neil McKenzie5 and Stephen P. Lownie1,3

1 Department of Diagnostic Radiology, Neuroradiology Section, London Health Sciences Centre, University Campus, University of Western Ontario, 339 Windermere Rd., PO Box 5339, London, ON, Canada N6A 5A5.
2 Present address: Department of Neurosciences, University of the Philippines, College of Medicine and the Philippine General Hospital, Manila, Philippines.
3 Department of Clinical Neurological Sciences, London Health Sciences Centre, University of Western Ontario, London, ON, Canada.
4 Present address: Department of Neurosurgery, Henry Ford Hospital, Detroit, MI.
5 Department of Surgery, London Health Sciences Centre, University of Western Ontario, London, ON, Canada.

Received March 2, 2005; accepted after revision May 25, 2005.

 
The authors have no financial interest in MTI Corporation. Address correspondence to D. M. Pelz (pelz{at}uwo.ca).

WEB

This is a Web exclusive article.

Keywords: anatomy • arteriography • arteriovenous fistula • arteriovenous malformation • cardiovascular imaging • digital subtraction angiography • embolization • MRI


Introduction
Top
 Introduction
Case Report
 Discussion
 References
 
Intercostal arteriovenous malformations (AVMs) and fistulas (AVFs) are rare lesions, and few case reports have been published [1-7]. Most have been secondary to trauma or iatrogenic therapeutic procedures [1-6], and one case was presumably congenital in origin. All have had single arterial feeders and draining veins. We present a case of congenital intercostal AVM in a young patient initially diagnosed on the basis of MRI findings and treated by a combination of transarterial and transvenous endovascular therapy and direct surgery.


Case Report
Top
 Introduction
 Case Report
Discussion
 References
 
A 17-year-old girl of the Jehovah's Witness faith, a religion that forbids followers from receiving blood transfusions, presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for a faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. An MRI examination showed an abnormality of the left eighth rib, consisting of focal bone expansion and several large vascular spaces (Fig. 1A), that was thought to be an AVM.


Figure 1
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  		Fig. 1A —17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Axial T1-weighted MR image of thorax shows dilated vascular channels (straight arrow) associated with left eighth rib that are draining into enlarged intercostal vein (curved arrow).

 
Subsequent spinal angiography revealed a large, complex, high-flow AVM centered at the T8 level with feeders originating from the left T6-T9 intercostal arteries (Figs. 1B and 1C). Nidal and fistulous components were identified. Spinal arteries originated from the left T5, T9, and T11 intercostal arteries, and venous drainage was into a large intercostal vein and the hemiazygous system.


Figure 2
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  		Fig. 1B —17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Left T7 intercostal arteriogram (anteroposterior view), early arterial phase, shows nidal (open arrow), fistulous (curved arrow), and racemose (solid straight arrow) feeders to intercostal arteriovenous malformation (AVM).

 

Figure 3
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  		Fig. 1C —17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Left T7 intercostal arteriogram (anteroposterior view), midarterial phase, shows large intercostal draining vein (arrow).

 
A natural history of this lesion was unknown. A decision was made to treat the lesion because of the increasing intensity of the patient's bruit, her back discomfort and exertional dyspnea, and the likelihood of these symptoms all worsening with age. The first transfemoral transarterial endovascular procedure involved superselective catheterization and embolization of all feeding arteries using n-butyl cyanoacrylate and nondetachable platinum coils. Angiography performed after embolization showed near complete obliteration of the AVM (Figs. 1D and 1E), and the bruit was no longer audible.


Figure 4
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  		Fig. 1D —17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Left T7 intercostal arteriogram (anteroposterior view) obtained after occlusion of superior feeding pedicle with n-butyl cyanoacrylate (straight arrow) shows residual filling of AVM from other components (curved arrow).

 

Figure 5
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  		Fig. 1E —17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Left T7 intercostal arteriogram (anteroposterior view) obtained after embolization of all feeding branches to AVM. No filling of large draining vein is seen.

 
During a follow-up examination 2 months later, a faint recurrent bruit was heard, and a repeat MRI examination showed continued filling of the large intercostal vein. A repeat spinal angiogram showed striking recanalization of all previously embolized feeders via a network of small, tortuous collateral vessels, primarily from the T7 and T8 intercostal arteries (Figs. 1F and 1G), and recruitment of supply from the previously uninvolved T5 and T10 intercostal arteries.


Figure 6
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  		Fig. 1F —17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Left T7 intercostal arteriogram (anteroposterior view) obtained 3 months after initial embolization shows continued filling of AVM from multiple small recanalized feeding arteries (arrows).

 

Figure 7
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  		Fig. 1G —17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Left T7 intercostal arteriogram (anteroposterior view), late arterial phase, shows recanalized feeders (small arrows) and intercostal vein, which drains into hemiazygos system (large arrow).

 
An attempt was then made to access the vein pouch and obliterate the malformation by a transfemoral, transvenous approach via the azygos and hemiazygos systems; however, the tortuosity of the venous anatomy precluded safe positioning of the microcatheter for embolization.

It was then decided to perform a combined endovascular and direct surgical procedure to minimize blood loss. A new liquid polymer (Onyx, MTI Corp.) was chosen as the embolic agent to occlude the venous pouch and malformation. Onyx is a mixture of ethylene vinyl alcohol, dimethyl sulfoxide, and tantalum. It has been used for the endovascular occlusion of brain AVMs [8, 9] and has several advantages over traditional liquid polymers such as n-butyl cyanoacrylate. It is nonadhesive and non-flow-directed, remaining confluent in a continuous column during slow injection. This property is thought to be advantageous in a high-flow situation such as an AVM with fistulas, although this use of Onyx has not, to our knowledge, been previously reported.


Figure 8
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  		Fig. 1H —17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Left T8 intercostal arteriogram (anteroposterior view), early arterial phase, obtained immediately after intraoperative injection of Onyx (MTI Corp.), a mixture of ethylene vinyl alcohol, dimethyl sulfoxide, and tantalum. Radiopaque cast of Onyx is seen in lateral aspect of draining vein (arrow).

 


Figure 9
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  		Fig. 1I —17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Left T8 intercostal arteriogram (anteroposterior view), late venous phase, shows there is slow filling of medial aspect of draining vein (arrow).

 


Figure 10
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  		Fig. 1J — 17-year-old girl presented with mild shortness of breath on exertion and mild midthoracic back pain on inspiration. Physical examination was unremarkable except for faint midthoracic intercostal bruit. There was no history of chest trauma or thoracic intervention. Axial gadolinium-enhanced T1-weighted MR image of thorax obtained 10 months after treatment shows Onyx-filled venous varix (arrow). No flow could be identified in arterial feeders, nidus, or draining veins.

 
The combined procedure was performed in an operating room using intraoperative fluoroscopy with the patient under general anesthesia. A 6-French sheath had been placed in the left femoral artery before positioning for a left lateral thoracotomy. The chest was opened along the lower border of the left seventh rib; after a flush aortogram and fluoroscopic localization, the AVM was found just below the pleural surface along the inner surface of the eighth rib. A 4-French micropuncture sheath was then inserted into the lateral aspect of the venous pouch, and approximately 3.5 mL of Onyx was then injected under fluoroscopic control over 60 minutes with manual compression of the medial aspect of the pouch. A subsequent spinal angiogram showed no residual filling of the AVM and only very slow filling of the medial vein pouch (Figs. 1H and 1I). A follow-up MR image obtained 10 months after the procedure showed no filling of the vascular channels or draining vein (Fig. 1J). The bruit could no longer be heard, and the patient reported a subjective increase in her exercise tolerance.


Discussion
Top
 Introduction
 Case Report
 Discussion
References
 
In this case report, we describe a nontraumatic, presumably congenital, intercostal AVM diagnosed using MRI. Only one nontraumatic or iatrogenic intercostal AVF has been reported previously [7], and that case occurred in a 6-year-old boy who was treated surgically. Similar lesions reported previously [1-6] all originated from trauma or therapeutic procedures, and only one was treated using endovascular methods [6]. Our patient presented a therapeutic challenge because of the apparent rarity of this condition, the unknown natural history, and the fear of excessive blood loss in a patient of the Jehovah's Witness faith. The new embolic agent Onyx was used in a novel way for direct surgical obliteration of the venous pouch after transfemoral arterial and venous approaches were unsuccessful. Tortuous venous anatomy precluded the use of relatively inflexible balloon catheters. Although catheter angiography is the gold standard for imaging of vascular malformations, MRI was valuable for the diagnosis and follow-up imaging of this lesion.


Acknowledgments
 
We would like to acknowledge the technical assistance of Lynn Denning and Andrew Cormack (MTI) and the secretarial assistance of Cathy Lockhart and Keith MacDougall.


References
Top
 Introduction
 Case Report
 Discussion
 References
 

  1. Coulter TD, Maurer JR, Miller MT, et al. Chest wall arteriovenous fistula: an unusual complication after chest tube placement. Ann Thorac Surg 1999; 67:849 -850[Abstract/Free Full Text]
  2. Cox PA, Keshishian JM, Blades BB. Traumatic arteriovenous fistula of the chest wall and lung secondary to insertion of an intercostal catheter. J Thorac Cardiovasc Surg 1967;54 : 109-112[Medline]
  3. Derdeyn CP, Middleton WD, Allen BT, Nordlicht SM. Acquired intercostal arteriovenous fistula: color Doppler ultrasonographic diagnosis. J Ultrasound Med 1993;12 : 679-681[Medline]
  4. Howell JB. Intercostal fistula due to pleural biopsy. (letter) Thorax 1991; 46:688[Free Full Text]
  5. Lai JH, Yan HC, Kao SJ, et al. Intercostal arteriovenous fistula due to pleural biopsy. Thorax 1990;45 : 976-978[Abstract/Free Full Text]
  6. Siddhartha W, Parmar H, Shrivastav M, et al. Endovascular glue embolization of an intercostal arteriovenous fistula: a non-surgical option. J Postgrad Med 2000;46 : 213-214[Medline]
  7. Yamasaki N, Hata H, Kusaga M, Kubo K. A surgical case of congenital intercostal arteriovenous fistula [in Japanese]. Nippon Kyobu Geka Gakkai Zasshi 1977; 25:936 -939[Medline]
  8. Murayama Y, Vinuela F, Ulhao A, et al. Nonadhesive liquid embolic agent for cerebral arteriovenous malformations: preliminary histopathological studies in the swine rete mirabile. Neurosurgery1998; 43:1164 -1175[CrossRef][Medline]
  9. Jahan R, Murayama Y, Gobin YP, Duckwiler GR, Vinters HV, Vinuela F. Embolization of arteriovenous malformations with Onyx: clinicopathologic experience in 23 patients. Neurosurgery2001; 48:984 -997[CrossRef][Medline]

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