Accepted Manuscript Simultaneous Open Surgical Treatment of Aortic Coral Reef and Leriche Syndrome: Case Report and Literature Review C. Pranteda, D. Menna, L. Capoccia, P. Sirignano, W. Mansour, F. Speziale PII:

S0890-5096(16)00045-5

DOI:

10.1016/j.avsg.2015.11.003

Reference:

AVSG 2656

To appear in:

Annals of Vascular Surgery

Received Date: 13 March 2015 Revised Date:

8 July 2015

Accepted Date: 8 November 2015

Please cite this article as: Pranteda C, Menna D, Capoccia L, Sirignano P, Mansour W, Speziale F, Simultaneous Open Surgical Treatment of Aortic Coral Reef and Leriche Syndrome: Case Report and Literature Review, Annals of Vascular Surgery (2016), doi: 10.1016/j.avsg.2015.11.003. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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SIMULTANEOUS OPEN SURGICAL TREATMENT OF AORTIC CORAL REEF AND LERICHE SYNDROME: CASE REPORT AND LITERATURE REVIEW

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Pranteda C. 1, Menna D. 1, Capoccia L. 1, Sirignano P. 1, Mansour W. 1, Speziale F 1.

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Vascular and Endovascular Surgery Division, Department of Surgery "Paride Stefanini", Policlinico Umberto I, "Sapienza" University of Rome, Italy

Corresponding author: Dr. Danilo Menna: [email protected]

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The Authors declare no conflict of interest

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Tel & fax: +39064940532

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Viale del Policlinico 155, 00161, Rome, Italy.

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ABSTRACT The coral reef aorta (CRA) is a rare syndrome commonly referred to a distribution of calcified plaques in the visceral part of the aorta. Since those plaques can cause malperfusion of the lower limbs, visceral ischemia or renovascular hypertension, surgical treatment is recommended. Transaortic endarterectomy is accepted as a standard repair and it is often performed through an extensive thoraco-abdominal approach. CRA has been reported in association with polidistrectual atherosclerotic disease, such as Leriche syndrome. When these two conditions coexist, surgical invasivity increases raising several issues concerning the type of surgical access as well as the revascularization techniques. We report the case of a patient with CRA and Leriche syndrome treated by simultaneous aortic endarterectomy and aortibifemoral bypass at our institution. Intervention was performed through left lumbotomy at 10th intercostal space extended by a left pararectal abdominal incision with section of 11th rib. Through extraperitoneal access visceral vessels were isolated. Aortic cross clamping was performed at supraceliac and infrarenal levels and a longitudinal arteriotomy was performed on the posterolateral wall of visceral aorta for an overall 4 cm extension. Aortic endarterectomy was then performed and complete plaque excision was easily achieved. SMA angioplasty was then performed by a DeBakey dilator, gaining an optimal backflow. The aortotomy was then closed with running 3-0 polypropilene suture. Subsequently, through a trans-peritoneal access an aortobifemoral bypass was performed by a dacron knitted graft. Post-operative course was uneventful. At a 6 months follow-up the patient is in good clinical condition with normal patency of visceral vessels.

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INTRODUCTION The coral reef aorta (CRA) is a rare syndrome commonly referred to a distribution of calcified plaques in the visceral part of the aorta1. Since those plaques can cause malperfusion of the lower limbs, visceral ischemia or renovascular hypertension, surgical treatment is recommended. Transaortic endarterectomy is accepted as a standard repair and it is often performed through an extensive thoraco-abdominal approach2. CRA has been reported in association with polidistrectual atherosclerotic disease, such as Leriche syndrome. When these two conditions coexist, surgical invasivity increases raising several issues concerning the type of surgical access as well as the revascularization techniques3. We report the case of a patient with CRA and Leriche syndrome treated at our institution.

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CASE REPORT A 52-year-old woman with history of smoking, hypertension and dyslipidemia was admitted because of an intermittent calf claudication with rapid onset of post-prandial abdominal pain and weight loss. At a physical examination femoral and distal pulses were not palpable. Renal function was normal (serum creatinine level 0.8 mg/dl), as well as serum calcium level (2.24 mmol/l) and no increase in Cross-Reactive Protein (CRP) and Erytro-Sedimentation Rate (ESR) was detected. The patient underwent a computed tomographic angiography (CTA) revealing severe stenosis of visceral aorta caused by a heavily calcified plaque, occluding the celiac trunk (CT) and causing a tight stenosis of superior mesenteric artery (SMA) as well (Figure 1). The right renal artery (RRA) was hypoplasic and the right kidney was atrophic. Left renal artery (LRA) was normally patent. Furthermore, a complete obstruction of infrarenal aorta was detected, with distal reperfusion at level of left iliac bifurcation and right femoral bifurcation (Figure 2). Final diagnosis was then CRA and Leriche syndrome. Under the suspicion of an infective etiology, Veneral Disease Research Laboratory (VDRL) and Treponema Pallidum Haemoagglutination Assay (TPHA) were performed in order to exclude a syphilitic aetiology, and both of them were normal. An open surgical repair was planned in order to perform an aortic endarterectomy and an aortobifemoral bypass graft. A left lumbotomy at 10th intercostal space was performed and extended by a left pararectal abdominal incision with section of 11th rib. In order to achieve a better proximal aortic control, the left diaphragmatic pillar was dissected. Through extraperitoneal access visceral vessels were isolated. After systemic anticoagulation through intravenous administration of Heparine Sodium 100/kg IU, aortic cross clamping was performed at supraceliac and infrarenal levels and a longitudinal arteriotomy was performed on the posterolateral wall of visceral aorta for an overall 4 cm extension. Aortic endarterectomy was then performed and complete plaque excision was easily achieved thus allowing a selective cold perfusion (ringer lactate at 4°C, mannitol 12.5 g/l, methylprednisolone 125 mg/l)4 of LRA through an irrigation Fogarty catheter (Edwards Lifesciences Irvine, USA). Since a poor backflow from RRA was detected, this vessel was considered as not functional and then not perfused. SMA angioplasty was then performed by a DeBakey dilator,

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gaining an optimal backflow. The aortotomy was then closed with running 3-0 polypropilene suture. Overall cross-clamping time was 14 minutes. Subsequently, through a trans-peritoneal access an aortobifemoral bypass was performed by a dacron knitted graft. Post-operative course was uneventful. At a 6 months follow-up the patient is in good clinical condition with normal patency of visceral vessels (Figure 3).

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DISCUSSION In 1984 Qvarfordt et al.1 firstly described a series of nine patients with isolated stenosis of suprarenal aorta due to calcified lesions; because of its macroscopic morphology, Authors called this syndrome coral reef. Since then, several papers dealing with this pathology have been published, but despite this aortic coral reef remains a rare syndrome: it has been estimated that its frequency ranges between 0.6%5 and 1.8%6. Despite the low incidence this condition may represent a life-threatening pathology2, since the presence of suprarenal aortic obstructive lesions may cause severe malperfusion of all abdominal organs, leading to bowel ischemia and renovascular hypertension, as well as chronic lower limbs ischemia. Intermittent claudication is reported up in approximately half of the patients, renovascular hypertension in 42% and chronic visceral ischemia in 9%2. Commonly, patients are young women, heavy smokers, having multi-level vascular disease7,8 as in the presented case (Table 1). The pathophysiological basis of CRA is not fully elucidated: according to Blay et al.9 common risk factors for cardiovascular disease such as smoking and hypertension are not sufficient to explain its pathogenesis. For that reason, other possible etiopathogenetic agents such as syphilis10, neurofibromatosis11, rubella12 and amyloidosis13 have been advocated, all of them deriving from observation of comorbidities in each single patient. While the hypothesis of the influence of parathyroid hormone and serum calcium level as possible promoters of the process of calcification in the aorta was discarded1,14 the role of Fetuin-A, an inhibitor of the formation of vessel calcification, is still debated15,16. In our case microbiological tests as well as serum calcium level titration were performed. All of those tests were normal, and then we concluded that in our patient CRA derived from an atherosclerotic process. Because of the variable extension of aortic plaque, open surgery for CRA is burdened by high mortality and morbidity rates17; despite this, transaortic endarterectomy still represents the treatment of choice in such patients3. In order to reduce surgical invasivity, some Authors have reported a successful endovascular treatment by placement of a stent graft15,18. However, the risk for distal embolization and the technical difficulty in the deployment of the stentgraft remain important concerns14, and then this strategy could be addressed only to less severe forms of CRA7. In our patient, any endovascular approach was excluded because of the severity of atherosclerotic lesions at level of visceral aorta and of the extensive aorto-iliac obstruction. In literature, several surgical accesses have been proposed: from more invasive laparotomy3 or thoracotomy1,2 to lesser invasive lumbotomy7 and laparoscopy19.

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In our case, we preferred an extraperitoneal approach through a left lumbotomy with section of the left diaphragmatic pillar: this strategy allowed us an optimal proximal aortic control without the incision of the pleura, then avoiding any post-operative respiratory impairment as the patient needed no further mechanical ventilation. After aortic cross-clamping and aortotomy, the complete removal of aortic plaque was an easy procedure taking a short time. Despite this we selectively perfused left renal artery with cold crystalloid solution in order to minimize renal damage due to ischemia, as we generally use in case of open repair of juxtarenal aortic aneurysms4. It may be discussed that in case of short clamping time the risk for post-operative renal impairment is reduced20 and then any surgical adjunct aimed to renal protection may be avoided. In our case we perfused the LRA in order to preserve the only functional kidney, as right kidney artery was atrophic. The selective perfusion ensured a good post-operative renal function.

REFERENCES 1

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CONCLUSIONS CRA is a rare pathology, still with unclear etiopathogenesis, usually requiring a highly invasive open surgical treatment. The need for revascularization of visceral vessel as well as lower limbs may furtherly increase surgical invasivity. The use of selective renal perfusion may represent an optimal strategy to preserve post-operative renal function.

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Qvarford PG, Reilly LM, Sedwitz MM, Ehrenfeld WK, Stoney RJ. “Coral reef” atherosclerosis of the suprarenal aorta: a unique clinical entity. J Vasc Surg 1984;1:903– 8.

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Grotemeyer D, Pourhassan S, Rehbein H, Rehbein H, Voiculescua, Reinecke P, Sandmann W. The coral reef aorta - a single centre experience in 70 patients. Int J Angiol. 2007;16(3):98-105.

Pulli R, Dorigo W, Azas L. ‘Coral reef’ atherosclerosis of suprarenal aorta: case report and literature review. Eur J Vasc Endovasc Surg EXTRA 2001;1(6):1–3. 4

Speziale F, Ruggiero M, Sbarigia E, Marino M, Menna D. Factors influencing outcome after open surgical repair of juxtarenal abdominal aortic aneurysms. Vascular. 2010 May-Jun;18(3):141-6.

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De Bakey ME, Cullen SC. Year book of general surgery 1957-1958. Chicago: Year Book Medical Publishers, 1958:247.

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Sako Y. Arteriosclerotic occlusion of the mid abdominal aorta. Surgery 1966;59:709-12.

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DeRubertis BG, Jabori SO, Quinones-Baldrich W, et al. Retroperitoneal trapdoor endarterectomy for paravisceral ‘‘coralreef’’ aortic plaque. J Vasc Endovasc 2012;46:487e91.

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Minnee RC, Idu MM, Balm R. Coral reef aorta: case reports and review of the literature. Eur J Vasc

ACCEPTED MANUSCRIPT Endovasc Surg. 2005;29(6):557. 9

Blay E Jr., Zhou W. Rapid Renal Function Deterioration: An Unusual Presentation of Coral Reef Plaque. Ann Vasc Surg. 2014 Jan;28(1):260.e13-6.

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Axilrod HD. Obstruction of the aortic isthmus by a calcified thrombus. Arch Pathol 1946;41:63-5.

11

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Bloor K, Williams RT. Neurofibromatosis and coarctation of the abdominal aorta with renal artery involvement. Br J Surg. 1963;50:811-3.

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Fortuin NJ, Morrow AG, Roberts WC. Late vascular manifestations of the rubella syndrome. A roentgenographic-pathologic study. Am J Med 1971;51:134-40. 13

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Combe J, Huart JY, Kantelip B, Sibe M, al Sayed MA, Camelot G. Amylose d’une aorte sous-rénale en pierre de corail. J Mal Vasc 1997;22:43-7.

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Schulte KM, Reiher L, Grabitz L, Sandmann W. Coral reef aorta: A long-term study of 21 patients. Ann Vasc Surg 2000;14:626-33. 15

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Verma H, Baliga K, George RK, Tripathi RK. Surgical and endovascular treatment of occlusive aortic syndromes. J Cardiovasc Surg (Torino). 2013 Feb;54:55-69.

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Schlieper G1, Grotemeyer D, Aretz A, Schurgers LJ, Krüger T, Rehbein H, Weirich TE, Westenfeld R, Brandenburg VM, Eitner F, Mayer J, Floege J, Sandmann W, Ketteler M. Analysis of calcifications in patients with coral reef aorta. Ann Vasc Surg. 2010 Apr;24(3):408-14.

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Belczak SQ, Sincos IR, Aun R, Costa KV, Araujo EA. Coral reef aorta, emergency surgical: case report and literature review. Einstein (Sao Paulo). 2014 Apr;12(2):237-41. Holfeld J, Gottardi R, Zimpfer D, et al. Treatment of symptomatic coral reef aorta by endovascular stentgraft placement. Ann Thorac Surg 2008;85:1817e9.

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Di Centa I, Coggia M, Javerliat I, Alfonsi P, Maury JM, Kitzis M, Goëau-Brissonnière O. Total laparoscopic suprarenal aortic coral reef removal. J Vasc Surg. 2006 Jul;44(1):194-7.

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Chiesa R, Marone EM, Brioschi C, Frigerio S, Tshomba Y, Melissano G. Open repair of pararenal aortic aneurysms: operative management, early results, and risk factor analysis. Ann Vasc Surg. 2006 Nov;20(6):739-46.

ACCEPTED MANUSCRIPT Figure legend

Figure 1: Preoperative CT scan in axial projection (A) and multi-planar reconstruction (B).

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Figure 2: Aortotomy and selective renal perfusion. Figure 3: Post-operative CT scan in multi-planar (A) and three-dimensional (B) reconstructions.

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Table 1: Literature review.

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Figure 1: Preoperative CT scan in axial projection (A) and multi-planar reconstruction (B) documenting Coral Reef syndrome. Figure 2: Preoperative CT scan in volume-rendering reconstruction.

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Figure 3: Post-operative CT scan in multi-planar (A) and three-dimensional (B) reconstructions.

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Table 1: Literature review.

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Female Gender

Mean age (±)

Smoke

AH

Dysl.

DM

HD

RD

Risk Factors

9

100%

51

100%

100%

44.4%

11.1%

33.3%

-

Pulli 2001

1

100%

50

-

Yes

Yes

-

-

-

Minnee 2005

3

66.6%

56.6

100%

100%

66.6%

-

33.3%

Di Centa 2006

3

33.3%

48.6

-

-

-

-

Grotemeyer 2007

70

65.7%

59.5

70%

40%

NA

NA

Holfeld 2008

2

50%

80

-

-

-

De Rubertis 12

1

100%

67

Yes

Yes

Yes

Blay 2014

1

100%

49

-

Yes

Belczak 2014

1

100%

54

Yes

Present Case

1

100%

52

Yes

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-

-

-

NA

-

Mortality

100% TE

Retroperitoneal thoracoabdominal

22.2%

TE

Transabdominal

-

66.6% TE 33.3% retrograde Bypass on SMA

1 Thoracoabdominal 1 retroperitoneal 1 laparotomy

33.3%

Laparoscopic TE

Laparoscopic

-

82.6% TE

27 median laparotomy 11% 2 retroperitoneal

-

-

50%

2 TEVAR

-

-

Yes

TE

Retroperitoneal

-

-

-

-

TE

Thoracoabdominal

-

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-

Access

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Qvarfordt 1984

Principal Treatment

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Pts

-

-

-

-

-

-

TE

NA

-

Yes

Yes

-

-

-

TE

Lumbotomy

-

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Legend

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DM: Diabetes mellitus; AH : arterial hypertension; Dysl.: Dyslipidemia; HD: Heart Disease; RD: Renal Disease; TE: Transaortic Endoarterectomy; TEVAR: Thoracic EndoVascular Aortic Repair.

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Figure 1

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Figure 3

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Simultaneous Open Surgical Treatment of Aortic Coral Reef and Leriche Syndrome: Case Report and Literature Review.

The coral reef aorta (CRA) is a rare syndrome commonly referred to a distribution of calcified plaques in the visceral part of the aorta. Because thos...
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