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CASE REPORT
 

MEANDERING VESSELS: A SIGN OF ARTERIAL TORTUOSITY ON PLAIN CHEST RADIOGRAPHY

Venkatraman Bhat, MD, FRCR; Ahmed Al Muzrakchi, FRCR
Department of Radiology, Hamad Medical Corporation, Doha, Qatar

Introduction

Tortuous arteries traversing in unusual winding path (meandering) is a feature of vascular disease. These observations are often made in arteriography or on echocardiography1. In the elderly, these represent signs of atherosclerosis or hypertension whereas in children, they may be a sign of underlying systemic disease like Ehlers Donlos syndrome, Marfans syndrome or more recently recognized syndrome of arterial tortuosity1,2,3. Visualization of meandering vessels is possible in plain radiography of the chest because of high tissue contrast between the air filled lungs and fluid containing vascular structure. This finding on plain radiography may lead to diagnosis of unsuspected underlying disease. This report demonstrate such a classical example of meandering arteries on plain radiography of chest subsequently confirmed by multidetector computed tomography (MDCT) in a patient with clinical diagnosis of Ehlers Donlos syndrome.
A twelve years old male patient underwent plain radiography of chest for pulmonary assessment. Patient was known to be asthmatic with intermittent asthma medications. He had short stature and peculiar facial features. On physical examination, he had pectus excavatum and right inguinal hernia. Clinical examination of respiratory, cardiovascular system and CNS were unremarkable. He had no skin or joint abnormality. Along with clinical data and chest radiographic impression, the patient was suspected to have Ehlers Donlos syndrome.

Chest radiograph revealed elongated, tortuous aortic arch, projecting in to left lung field (Fig 1a and 1b) and lateral chest radiography showed a rounded opacity at upper lung field representing an unusually projecting aortic arch (Fig 2a). The patient was also found to have a retro-cardiac shadow representing a hiatus hernia and was referred for contrast enhanced computed tomography for evaluation of mediastenal vascular structures. Multidetector spiral computed tomographic examination confirmed gross tortuosity of aortic arch, which was partially located within the left upper lobe (Fig 2b). Additional arterial tortuosity was demonstrated in all branches of aortic arch, proximal and intrapulmonary branches of pulmonary arteries (Fig 2c). Lungs were essentially normal except for a subpleural cyst adjacent to tortuous upper lobe pulmonary artery. Also hiatus hernia was confirmed in lower chest sections containing predominantly fatty components.
 

Discussion

Tortuous, dilated aorta and pulmonary arteries are well known signs of Ehlers Danlos syndrome, Marfans syndrome and syndrome of arterial tortuosity in children1,3,4. Congenital heart diseases like patent ductus arteriosus and post-ductal aortic coarctation may also present with large aorta. In adults, atherosclerosis and hypertension are the main causes. When seen in the context of a known disease, the impact of observation is rather limited. However many patients present with unrelated symptoms and have chest radiography incidentally. In these patients, especially in the pediatric age group, appreciating signs of aortic and pulmonary tortuosity are vital, in order to suspect and make the correct diagnosis.
Intrapulmonary course of aorta is very rare, and results when there is extreme tortuosity and elongation of the aortic arch. It is uncommon to observe this anomalous aortic course. Our patient showed classical signs of aortic unfolding and elongation leading to a short intrapulmonary course, visible on plain radiography. Tortuous course of the pulmonary arteries were also present albeit less evident. In young patients, these signs are definite indication of underlying vascular abnormality as mentioned earlier. Though there is a non-invasive assessment of cardiovascular structures by echocardiography, it has limitations for extra-cardiac vascular structures. Magnetic resonance imaging must be considered as a valuable alternative wherever facilities are available. However, MDCT, has become the investigation of choice for vascular, pulmonary and airway assessment5. Associated complications like vascular stenosis and aneurysms can also be delineated. Additional lesions like hiatus hernia as shown in our patient are also best shown by computed tomography.

Conclusion

Meandering vessels are signs of arterial tortuosity. In children, meandering vessels are seen in association with connective tissue syndromes and syndrome of arterial tortuosity. Plain chest radiography can reveal signs of aortic and pulmonary tortuosity, at times, leading to unsuspected underlying clinical condition. Definitive investigation is multidetector-computed tomography, which delineates the vascular anatomy along with associated lesions.¨

References:

1. Echocardiographic Features of Arterial Tortuosity: A New Syndrome? Andrej Robida, M.D., Vladimir Bricelj, M.D., Tohami A. Tohami, M.D. International Journal of Angiology. 2001,10:241-245.
2. A new type of Ehlers-Danlos syndrome associated with tortuous systemic arteries in a large kindred from Qatar. Abdul Wahab A, Janahi IA, Eltohami A, Zeid A, Ul Haque NF, Teebi AS. Acta Pedsitrcs, 2003 Apr; 92(4): 456-462.
3. Thoracic aortic aneurysm syndrome in children. Zanotti G, Vricella L, Cameron D. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2008:11-21.
4. A family exhibiting arterial tortuosity syndrome displays homozygosity formarkers in the arterial tortuosity locus at chromosome 20q13 SHE Zaidi, V Peltekova,S Meyer, A Lindinger, AD Paterson,, L-C Tsui , M Faiyaz-Ul-Haque and AS Teebi, Clin Genet 2004: 67: 183-188.
5. Paediatric multidetector CT angiography: spectrum of congenital thoracic vascular anomalies: Pictorial review : B Oguz, MD, M Haliloglu, MD and M Karcaaltincaba, MD. British Journal of Radiology 2007, 80: 376-383.