since first described by quain in 1847 ( 1 ) , hundreds of abdominal aortic stenosis have been reported with many variations in anatomy .
though , the complete absence of infrarenal aorta , seen in presenting case , is extremely rare .
this represents the first case of complete segmental occlusion of infrarenal aorta in the old patient .
a 52-yr - old woman presented to the clinic with one - year history of bilateral claudication .
blood pressures were measured at 130/80 mmhg in the forearms and at 100/70 mmhg in the thighs , with ankle - brachial indexes of 0.77 .
serologic tests including c3 , c4 , venereal disease research laboratory test ( vdrl ) , and immunoglobulin g were unremarkable except mild elevated erythrocyte sedimentation rate ( esr ) of 22 mm / hr and low tittered anti - nuclear antibody of 1:80 with speckled pattern .
the abdominal aorta was reconstituted by multiple collaterals , such as marginal artery of drummond , the anastomosis between superior mesenteric and inferior mesenteric artery , bilateral epigastric , lumbar , and abdominal superficial arteries .
for her knee joint arthritis , she was started with methylprednisolon of 8 mg and non - steroid anti - inflammatory drug .
after negative results of pathergy test and human lymphocyte antigen b 51 , we ruled out the behcet 's disease and she tapered the medications to stop successfully .
she wanted the antihypertensive drug for mild hypertension and was taken amlodipine at 5 mg daily .
one year later , computer tomographic angiography showed patent bypass graft and decreased collateral vessels ( fig .
the etiology of abdominal aortic stenosis is poorly understood . the young age of most of the patients suggests that the malformation may be congenital .
maycock first proposed an unequal fusion of embryonic aorta as a congenital mechanism ( 3 ) . in first month of fetus , the paired primitive dorsal aortas fuse to form the abdominal aorta .
incomplete fusion due to infection or inflammation may lead of kinking of the aorta resulting to permanent constriction .
congenital rubella has been suggested for such vascular anomaly ( 4 , 5 ) . in alagille syndrome which is
congenital pulmonary artery stenosis transmitted in an autosomal dominant inheritance , deletion of the jagged 1 gene has been suggested to relate with abdominal aortic coarctation ( 6 ) .
acquired etiologies were also suggested in fibromuscular dysplasia or takayasu 's arthritis ( 7 , 8) . in our patients , several inflammatory presentations including the knee joint arthritis , recurrent oral ulcers , and elevation of esr have suggested the possibility of behcet 's disease .
we ruled out the behcet 's disease after the negative study of pathergy test . considering the complete absence of infrarenal aorta without any inflammatory reaction ,
in review of cases for abdominal aortic stenosis , coarctation , hypoplasia , and middle aortic syndrome were used diversely .
the term " coarctation " implies a congenital anomaly , and " hypoplasia " is to longer segment of coarctation .
middle aortic syndrome is the clinico - anotomical entity involving long segmental narrowing of the abdominal aorta and various visceral arteries irrespective of its etiology .
we described this case by " hypoplasia " , because 4 cm of infrarenal aorta was absent .
presenting symptoms of abdominal aortic hypoplasia are related to the extent of the stenosis , and the collateral system . in case involving renal artery , patients typically present with uncontrolled hypertension .
infrarenal aortic hypoplasia can give rise to claudication when the collateral circulation is not sufficient .
an abdominal aortogram via angiography , ultrasound , computed tomography , or magnetic resolution imaging can be used to diagnose and to determine the location and extent of disease . in case of renal artery stenosis
claudication and aneurysmal dilatation also need surgical correction . in this case , as the subrenal aortic stump was too short , graft was interposed between the subdiaphragmatic thoracic aorta to the iliac bifurcation .
careful physical examination for inspection of dilated superficial arteries and auscultation of bruits can provide a clue for rare causes of ileofemoral insufficiency such as abdominal aortic stenosis .
when suspected , an angiography is mandatory to diagnose , and surgical intervention is necessary to improve severe claudication .
 we describe a case of infrarenal aortic hypoplasia in a 52-yr - old woman who presented with claudication . 
 computed tomographic angiography revealed an abrupt absence of the infrarenal aorta , with collateral flow reconstituting the iliofemoral systems . 
 after a polytetrafluoroethylene graft was interposed between the aortic stump and the iliac bifurcation , the patient 's claudication resolved . 
INTRODUCTION
CASE REPORT
DISCUSSION