Year : 2005 | Volume
: 6 | Issue : 3 | Page : 118--120
Eight-year old child with advanced stage Takayasu's arteritis
Muhammad Dilawar1, Zaheer Ahmed2,
1 Pediatric Cardiology, CCS, Hamad Medical Corporation, Doha, Qatar
2 Poole General Hospital, Poole, Dorset, United Kingdom
Hamad Medical Corporation, P.O. Box 3050 Doha
|How to cite this article:|
Dilawar M, Ahmed Z. Eight-year old child with advanced stage Takayasu's arteritis.Heart Views 2005;6:118-120
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Dilawar M, Ahmed Z. Eight-year old child with advanced stage Takayasu's arteritis. Heart Views [serial online] 2005 [cited 2023 Jan 29 ];6:118-120
Available from: https://www.heartviews.org/text.asp?2005/6/3/118/64023
Takayasu.s arteritis (TA) is a chronic,inflammatory disease that involves aorta, it s branches and the pulmonary arteries ,,,, . Although TA is the most common acquired inflammatory vascular disease, it is still a rare problem in pediatric population. This disease most often affects young women and has a predilection for people of Asian descent. There is a paucity of literature addressing this disease in our pediatric population. We present an 8-years old male child diagnosed as TA with a very aggressive disease process.
An 8-year old Afghani male child presented with a history of syncopal attacks and unilateral loss of vision. Syncopal attacks started six months ago, initially were associated with walking but later on, he used to have them even at sitting or standing. Three months later, he had sudden loss of left eye vision. Parents deny any history of convulsions and there was no associated headache, palpitation, weakness, numbness, arthralgia or any skin changes.
The patient was seen at different medical centers and echocardiography, cardiac catheterization and CT scan brain were performed. The only abnormality, which was picked up on these investigations, was left pulmonary artery stenosis.
His initial examination with us revealed an awake, alert child. Temperature, heart rate and respiratory rate were normal. Radial pulses were absent bilaterally; left carotid pulse was absent, femoral and distal leg pulses were of high volume. Right carotid and abdominal bruit was audible. Four-extremity blood pressure revealed un-recordable B.P. in upper limbs and 170/110 mmHg of B.P. in the lower limbs. Auscultation revealed grade II/IV systolic ejection murmur at left upper sternal border. Visual acuity was 6/6 on right side and there was no perception of light on left side. Fundoscopic examination confirmed left sided optic atrophy.
Pertinent laboratory data included a normal complete blood count, raised acute phase reactants with erythrocyte sedimentation rate (ESR) of 102 and CRP value of 48 mg/l. Serum electrolytes, blood urea nitrogen and creatinine were all normal. Additional diagnostic studies included a normal chest radiograph and electrocardiogram. Echocardiography showed moderate narrowing of descending thoracic aorta with a gradient of 66 mmHg, mild distal left pulmonary artery stenosis and carotid doppler showed stenosis of right carotid and no flow in left carotid artery. Finally, aortic angiography was performed and it revealed severe long segment stenosis of right carotid, and severe stenosis of right subclavian and right vertebral arteries [Figure 1], complete occlusion of left subclavian and left carotid arteries and moderate coarctation of descending thoracic aorta [Figure 2] and bilateral proximal renal artery stenosis [Figure 3].
The initial treatment consisted of low dose aspirin, prednisone 2mg/kg/day and antihypertensive drugs. The patient had an uncomplicated hospital course and there was draumatic improvement in acute phase reactants and he was discharged on the 4th day of hospitalization. During follow up clinic visits, his acute phase reactants were intermittently elevated even on high dose of steroid and within few months, he developed complete loss of vision in his right eye as well. After this incidence, we started him on Cyclophosmamide in addition to prednisone and his acute phase reactants are relatively well controlled now.
Takayasu's arteritis, also known as pulseless disease, primarily involves large elastic arteries , . The exact incidence of this disease in our population is unknown, the annual incidence in North American population is estimated to be 2.6 per million people  . Etiology remains unknown, although different hypothesis like bacterial infections including mycobacterium, viral and autoimmune etiologies have been proposed. Lesions produced by this inflammation can be stenotic, occlusive or aneurysmal. TA is described to have three types of presentations, acute, subacute and chronic and symptomatology may vary from non-specific features to advanced stage disease like pulselessness and the usual presenting symptoms are due to hypertension, heart failure or a neurologic event.
Diagnosis of TA is usually established with the help of angiographic studies which reflect only the luminal aspect of the vessels but more recently non-invasive imaging like CAT scan  /MRI  provide information on mural changes of the vessel and these non-invasive modalities may replace angiography not only for the diagnosis but also for the monitoring of therapy and progression of the disease. The diagnostic criterias laid down by American College of Rheumatology  and Ishikawa  are very well recognized. The activity of the disease can be decided on the basis of systemic symptoms, acute phase reactants; and follow up with non-invasive imaging is useful to look for not only the luminal diameter of the vessels but also the inflammatory response in the vascular walls. Steroids, Cyclophosphamide and Azathioprine are the mainstay anti-inflamatory drugs. Carotid artery stenting/surgery has variable results. Children with TA have overall higher morbidity and mortality than adults , .
Diagnosis of TA during early phase of the disease has important prognostic implication. Early diagnosis and institution of aggressive anti-inflamatory therapy could have prevented the subsequent development of wide spread stenosis and bilateral complete loss of vision in our patient.
Our case also emphasizes the importance of thorough physical examination, including 4 extremity blood pressure measurements, relevant investigations and timely diagnosis and aggressive medical management of takayasu arteritis in pediatric population to have a favourable prognosis.
Acknowledgement: Special thanks to Malou S. Rocha, BS. Cardiology and Cardiovascular Surgery Department Secretary for her help in preparing this manuscript.
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