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Subodh Gururani1, Sreejitha K Sreesailam2, Ramakrishna Narayanan3, Phani K Devarasetti4, Liza Rajasekhar5

Author Affiliations: 

1,2Senior Residents, Department of Clinical Immunology and Rheumatology, Nizam’s Institute of Medical Sciences, Hyderabad, India, 3,4 Assistant professors, Department of Radiology and Imageology, Nizam’s Institute of Medical Sciences, Hyderabad, India, 5Professor and Head, Department of Clinical Immunology and Rheumatology, Nizam’s Institute of Medical Sciences, Hyderabad, India

Correspondence to: 

Liza Rajasekhar, Department of Clinical Immunology and Rheumatology, Nizam’s Institute of Medical Sciences, Hyderabad, India


Journal Issue: 
Volume 50: Issue 3: 2020
Cite paper as: 
J R Coll Physicians Edinb 2020; 50: 295–8



Takayasu arteritis which is reported more commonly from Asia and in females can present as middle aortic syndrome with lower limb claudication. We present a case of a young male with Takayasu arteritis with middle aortic syndrome and Winslow pathway collaterals with lower limb ischaemia, hypertension, coronary occlusion and stroke. The extensive collateral formation was visible as a clinical finding over the abdominal wall. The identification of these collateral pathways is essential in understanding the extent of haemodynamically significant disease and it alerts to the possibility of surgical injury during procedures like laparotomy or harvesting of internal thoracic artery for coronary artery bypass graft.

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Case presentation

A 20-year-old male presented with an eight-year history of bilateral lower limb claudication. His initial claudication distance of 30 minutes gradually worsened to 15 minutes. Six years ago he had a gangrene in the left fifth toe which was followed a year later by a gangrene in the left great toe. Evaluation had revealed hypertension with absent lower limb pulses. There was neither any bruit nor any asymmetry of pulses reported in the upper limbs. CT abdominal angiogram showed vessel wall thickening in the abdominal aorta along with long segment narrowing of the infrarenal abdominal aorta with collaterals reforming the external iliac arteries. He was prescribed antihypertensives, low-dose oral steroids and oral methotrexate but was non-compliant. Two years later he developed sudden onset weakness of the right hand. MRI brain revealed left parietal lobe infarction. He also noticed the development of dilated vessels over his abdomen starting from the flanks. He used nifedipine 10 mg daily as an antihypertensive for the next three years. At presentation to our department, he had a history of breathlessness, chest pain, palpitations and orthopnoea of ten days’ duration. He reported persistent claudication pain in the lower limbs without any decrease in claudication distance. There was no history of fever, cough or haemoptysis. He denied any history of oral or genital ulcers, skin rash or ocular complaints. He was not a smoker. On examination, he had accelerated hypertension (BP = 220/110 mmHg), lower limb pulses were absent bilaterally while the upper limb and neck pulses were bounding and symmetric without any bruit or carotidynia. The first and the fifth toes on the left side had mild pulp loss at the tips. The abdominal wall had thick-walled pulsatile vessels both above and below the umbilicus (Figure 1).

Figure 1 Prominent arterial collaterals over the abdominal wall in a patient with Takayasu arteritis with middle aortic syndrome (MAS)

The rest of the systemic examination was unremarkable. Laboratory investigation revealed thrombocytosis (platelets 7.63 lakh/ml) and a raised erythrocyte sedimentation rate (ESR) of 50 mm in the first hour. The chest radiograph was suggestive of pulmonary oedema, the electrocardiogram (ECG) showed ST elevation in the anterior chest leads and troponin I level was elevated (10078 ng/ml). Echocardiography showed regional wall motion abnormality along the left anterior descending artery (LAD) territory with an ejection fraction of 30%. Coronary angiography revealed single vessel disease with type-3 90% mid-LAD stenosis. Magnetic resonance angiography (MRA) of the aorta showed narrowing of the distal descending thoracic aorta extending up to the origin of the superior mesenteric artery (SMA). Bilateral renal arteries and the SMA were reformed via collaterals. There was near-total stenosis of the infrarenal aorta up to the level of bifurcation. Right common iliac artery, left internal and external iliac arteries were reformed via collaterals. Extensive collateral formation was also noted between the superior and the inferior epigastric arteries in the abdominal parietal wall (Figures 2 and 3).

Figure 2 Computed tomography angiography MIP and VRT images showing well-developed Winslow pathway collaterals bilaterally that start as enlarged internal thoracic arteries (stars)  in the thorax and continue as multiple vascular channels that anastomose the superior and inferior epigastric arteries (arrows and arrowheads respectively) and finally drain into the external iliac arteries (notched arrows).

MIP: maximum intensity projection; VRT: volume rendering technique