Cas cliniques

DOI of or

Service deSalpetri�ere, As

CorrespondHopital UnivHopitaux de P13, France, E-

Ann Vasc SurDOI: 10.1016/� Annals of V�Edit�e par ELS

Prise en charge chirurgicale d’une coarctationde l’aorte abdominale chez un patient ag�e

Chika Cho, Rapha€el Coscas, Fabien Koskas, Paris, France

La coarctation de l’aorte thoracique basse et/ou de l’aorte abdominale, d�enomm�ee ‘‘middleaortic syndrome’’ (MAS) par les anglo-saxons, est une malformation cardiovasculaire con-g�enitale rare pouvant conduire �a des complications isch�emiques graves. Le diagnostic �etanthabituellement fait au cours de l’enfance ou chez le jeune adulte, la prise en charge du MASchez le patient ag�e est donc exceptionnelle. Nous rapportons le traitement chirurgical d’un MASchez une patiente de 65 ans pr�esentant des signes d’isch�emie menacante r�enale etm�esent�erique. Un pontage thoraco-abdominal implant�e sur l’aorte ascendante et revasculari-sant les art�eres visc�erales �etait r�ealis�e par sterno-laparotomie avec un r�esultat satisfaisant. Lesaspects techniques et th�erapeutiques sont discut�es.

Aortic coarctation is a common congenital cardio-

vascular defect, usually involving the aortic isth-

mus.1 Coarctation of the distal thoracic aorta and/

or the abdominal aorta, namely ‘‘middle aortic

syndrome’’ (MAS), is rare and accounts for 0.5-2.0%

of coarctation cases.2,3 Although diagnosis is usually

made during childhood or in young adulthood, it is

exceptionally made in elderly patients. We here

report the surgical management of a MAS case in a

65-year-old woman.

CASE REPORTS

A 65-year-old woman was referred to our hospital for the

treatment of a complicated MAS. Her past medical history

was consistent for a reno-vascular hypertension in

infancy, for which she underwent a left nephrectomy at

the age of 11 and an aorto-right renal bypass at the age of

iginal article: 10.1016/j.avsg.2009.12.012.

chirurgie vasculaire, Hopital Universitaire de la Piti�e-sistance-Publique Hopitaux de Paris, Paris, France.

ence : Dr Chika Cho, Service de chirurgie vasculaire,ersitaire de la Piti�e-Salpetri�ere, Assistance-Publiquearis, 47-83 boulevard de l’hopital, 75651 Paris, Cedexmail: cmilleka@yahoo.co.jp

g 2010; 24: 694.e5-694.e8j.acvfr.2010.12.047ascular Surgery Inc.EVIER MASSON SAS

13. At that time, anMASwas diagnosed but the correction

had not been performed for an unknown reason. She also

had a transient ischemic attack at the age of 45, and she

underwent a partial gastrectomy for bleeding at the age of

63. For 3 years, her renal function has progressively

worsened leading to hemodialysis. At the time of admis-

sion, she had been recently accepted on the waiting list for

renal transplantation. Six months ago, she presented with

an episode of subacute ischemic colitis with severe anemia

(Hb ¼ 2.5 g/dL), treated medically using corticosteroids.

Given the severity of this recent episode, the antecedents

of reno-vascular hypertension, and the need for future

renal transplantation in the setting of an MAS, she was

referred to our department for surgical correction of her

aortic disease. On examination, upper extremity pulses

were normal but femoral pulses were reduced. A mode-

rate hypertension (142/68 mm Hg) despite oral quadri-

therapy was noted. The patient denied any symptom of

intestinal ischemia at the time of examination (no abdo-

minal pain, normal abdominal palpation), or lower limb

claudication. Laboratory tests noted a creatinine at

3.6 mg/dL. Computed tomography scan demonstrated an

MAS extending from the celiac trunk level down to right

renal artery level (Fig. 1A). Moreover, a 40% stenosis

associated with a post-stenotic aneurysm was noted at the

origin of the celiac trunk. There was a 60% stenosis on the

two first centimeters of the superior mesenteric artery.

The inferior mesenteric artery was occluded. Of note, the

prior aorto-right renal bypass was patent (Fig. 1B).

Given the risk of intestinal ischemia recurrence, the

persistent hypertension, and the need for an adequate

iliac inflow for a future renal transplantation, a

758.e5

Fig. 1. A Preoperative computed tomographic angio-

graphy showed suprarenal coarctation with celiac artery

stenosis and associated aneurysm, and superior mesen-

teric artery stenosis. B Preoperative computed tomo-

graphic angiography showed the ancien aorto-right renal

artery bypass.

758.e6 Cas cliniques Annales de chirurgie vasculaire

revascularization was decided. Through median sterno-

laparotomy, a 20-mm Dacron graft was proximally anas-

tomosed to the ascending aorta. It was tunnelized to the

abdomen through a small incision in themiddle part of the

diaphragm, and positioned behind the left part of the liver

and the pancreas. The graft was then brought straight

down anteriorly to the left side of the abdominal aorta.

The celiac trunk and the superior mesenteric artery

were directly reimplanted into the graft. The infrarenal

aorta was ligated. Distally, the graft was anastomosed in

an end-to-end manner to a 20/10-mm bifurcated graft

sewn to the common iliac arteries. Therefore, the right

kidney was perfused in a retrograde manner through the

patent aorto-right renal artery bypass. Postoperative

course was uncomplicated. Histopathologic study of vis-

ceral vessels showed intimo-medial fibrosis, but the

absence of inflammatory process.

The follow-up was uneventful (no intestinal symp-

toms, no lower limb claudication, good recovery). At

3 months, the patient remained under hemodialysis but

her blood pressure normalized (105/60 mm Hg) under an

oral monotherapy. The computed tomography -scan does

not show any anomaly and her aortic repair remained

patent (Fig. 2).

Fig. 2. Postoperative computed tomography scan. The

bypass is patent and there is a good perfusion of reim-

planted collaterals.

DISCUSSION

Our case demonstrates that MAS is a potential cause

of visceral/renal malperfusion in the elderly

patients, and can be successfully treated using tho-

racoabdominal bypass.

MASwas first described by Sen et al. in 19634 but

its etiology remains poorly understood5 It can be

caused by Takayasu’s or temporal arteritis, neurofi-

bromatosis, fibromuscular dysplasia, retroperitoneal

fibrosis, mucopolysaccharidosis, Williams syn-

drome, and/or a developmental anomaly in the

fusion of the paired embryonic dorsal aortas.6-9 In

the present case, findings of the histopathological

Vol. 24, No. 5, 2010 Cas cliniques 758.e7

examination were not specific and it is probable that

the etiologywas congenital, as attested by thehistory

of renovascular hypertension during childhood, the

intimo-medial fibrosis, and the absence of any

inflammatory process.

Diagnosis of MAS is usually made during child-

hood or young adulthood. Mean age at diagnosis is

about 20 years.10 Symptoms are mainly related to

hypertension in the central vasculature proximal to

the aortic stenosis and to arterial insufficiency in the

vasculature distal to the stenosis (renal, visceral,

and/or iliac arteries). Uncontrolled renovascular

hypertension may lead to left ventricular hyper-

trophy, cardiomegaly, cerebrovascular accidents,

coronary artery disease, and congestive cardiac fai-

lure. The arterial inflow impairment to the kidneys

secondary to aortic coarctation and renal artery

stenosis may result in renal function impairment

and renal failure, as illustrated by our case. Symp-

toms of bowel ischemia are rarer.

Although patients not appropriately treated

experience premature death,3,11 our patient sur-

vived without undergoing any intervention to cor-

rect her aortic coarctation until the age of 65, despite

the occurrence of various symptoms secondary to

MAS since childhood (hypertension, transient

ischemic attack, chronic renal failure, intestinal

angina). The right renal revascularization at the age

of 13, the left nephrectomy, and the maximal

medical management using a quadritherapy may

have had a role to play in postponing the conse-

quences of hypertension.

Anatomically, the association of MAS with visce-

ral/renal stenoses is frequent, as illustrated by our

case. A recent series of 53 cases12 from the Uni-

versity of Michigan identified suprarenal coarcta-

tion in 69%, inter-renal coarctation in 23%, and

infrarenal coarctation in 8%. Eighty-seven percent

had renal artery narrowings or occlusions, and 62%

had celiac or mesenteric artery stenosis or occlu-

sions, with both vessels involved in 82%.

The primary indication for surgical intervention

is usually the level of hypertension3 However,

malperfusion of mesenteric vessels and the need for

an aorto-iliac inflow for further renal transplanta-

tion were the main indication in our case.

In planning an aortic repair for MAS treatment,

the choice of the technique to use remains in ques-

tion. Thoracoabdominal bypass, graft replacement,

patch angioplasty, and transluminal angioplasty

were successfully reported.3,13 The choice between

these techniques is dependent on the patient’s

general status (age, comorbidities), the exact loca-

tion and extension of the MAS, and the existence of

stenoses on critical aortic collaterals. However,

direct replacement carries a theoretical risk of post-

operative paraplegia; patch angioplasty is associated

with late degeneration, and long-term results of

endoluminal angioplasty are controversial.14-16 In

our case, given the good life expectancy of our

patient and the existence of visceral stenosis, we

elected to perform an open surgical reconstruction

using a thoracoabdominal bypass with direct reim-

plantation of the visceral vessels in the graft. The use

of the ascending aorta as bypass inflow avoided the

clamping of the descending thoracic aorta, thereby

minimizing the risk of postoperative paraplegia

while allowing for direct reimplantation of mesen-

teric vessels.

CONCLUSION

MAS is a rare condition in the elderly patients. This

diagnosis should be suspected in patients presenting

with a history of visceral, renal, and/or lower limb

malperfusion. Thoracoabdominal bypass remains a

useful option whenMAS is associated with stenoses

of critical aortic collaterals.

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