Median arcuate ligament syndrome: Open celiac artery reconstruction and ligament division after endovascular failure
2007; Elsevier BV; Volume: 46; Issue: 4 Linguagem: Inglês
10.1016/j.jvs.2007.05.049
ISSN1097-6809
AutoresKonstantinos T. Delis, Péter Gloviczki, Maraya Altuwaijri, Michael A. McKusick,
Tópico(s)Catalytic Processes in Materials Science
ResumoMedian arcuate ligament syndrome (MALS) is a rare disorder resulting from extrinsic compression and narrowing of the celiac artery, and—less often—the superior mesenteric artery, by the relatively low insertion of the ligament and/or prominent fibrous bands or ganglionic periaortic tissue of the celiac nervous plexus. We report on a young woman who after three consecutive attempts of endovascular therapy with balloon angioplasty and stenting for MALS, each followed by gross symptom recurrence and a cumulative weight loss of 10 kg, underwent open surgical division of the ligament and reconstruction of the celiac artery. Despite the initial response of MALS to endovascular therapy, the extrinsic pressure exerted on the celiac artery by the surrounding dense fibrous/ganglionic tissue resulted in slippage of the stents and/or failure of their material. These findings militate against the use of balloon angioplasty and stenting primarily in patients with MALS without prior release of the extrinsic compression on the celiac (and/or superior mesenteric) artery by dividing the surrounding median arcuate ligament and/or ganglionic tissue with open or laparoscopic surgery. Median arcuate ligament syndrome (MALS) is a rare disorder resulting from extrinsic compression and narrowing of the celiac artery, and—less often—the superior mesenteric artery, by the relatively low insertion of the ligament and/or prominent fibrous bands or ganglionic periaortic tissue of the celiac nervous plexus. We report on a young woman who after three consecutive attempts of endovascular therapy with balloon angioplasty and stenting for MALS, each followed by gross symptom recurrence and a cumulative weight loss of 10 kg, underwent open surgical division of the ligament and reconstruction of the celiac artery. Despite the initial response of MALS to endovascular therapy, the extrinsic pressure exerted on the celiac artery by the surrounding dense fibrous/ganglionic tissue resulted in slippage of the stents and/or failure of their material. These findings militate against the use of balloon angioplasty and stenting primarily in patients with MALS without prior release of the extrinsic compression on the celiac (and/or superior mesenteric) artery by dividing the surrounding median arcuate ligament and/or ganglionic tissue with open or laparoscopic surgery. The median arcuate ligament (MAL) is a fibrous arch crossing the aorta, usually superior to the celiac artery takeoff and at the level of the insertion of the diaphragm bridging the crura.1Horton K.M. Talamini M.A. Fishman E.K. Median arcuate ligament syndrome: evaluation with CT angiography.Radiographics. 2005; 25: 1177-1182Crossref PubMed Scopus (246) Google Scholar, 2Kopecky K.K. Stine S.B. Dalsing M.C. Gottlieb K. Median arcuate ligament syndrome with multivessel involvement: diagnosis with spiral CT angiography.Abdom Imaging. 1997; 22: 318-320Crossref PubMed Scopus (40) Google Scholar, 3Taylor Jr, L.M. Moneta G.L. Management of visceral ischemic syndromes.in: Rutherford R.B. Vascular surgery. WB Saunders, Philadelphia2000: 1501-1511Google Scholar Occasionally, the ligament may insert at a lower level, thus crossing the proximal portion of the celiac artery.1Horton K.M. Talamini M.A. Fishman E.K. Median arcuate ligament syndrome: evaluation with CT angiography.Radiographics. 2005; 25: 1177-1182Crossref PubMed Scopus (246) Google Scholar, 2Kopecky K.K. Stine S.B. Dalsing M.C. Gottlieb K. Median arcuate ligament syndrome with multivessel involvement: diagnosis with spiral CT angiography.Abdom Imaging. 1997; 22: 318-320Crossref PubMed Scopus (40) Google Scholar, 3Taylor Jr, L.M. Moneta G.L. Management of visceral ischemic syndromes.in: Rutherford R.B. Vascular surgery. WB Saunders, Philadelphia2000: 1501-1511Google Scholar, 4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar, 5Takach T.J. Livesay J.J. Reul Jr, G.L. Cooley D.A. Celiac compression syndrome: tailored therapy based on intraoperative findings.J Am Coll Surg. 1996; 183: 606-610PubMed Google Scholar MAL syndrome is the rare disorder caused by the extrinsic compression that the relatively inferior insertion of the MAL and/or prominent fibrous bands or the ganglionic periaortic tissue of the celiac nervous plexus may exert on the celiac artery.1Horton K.M. Talamini M.A. Fishman E.K. Median arcuate ligament syndrome: evaluation with CT angiography.Radiographics. 2005; 25: 1177-1182Crossref PubMed Scopus (246) Google Scholar, 2Kopecky K.K. Stine S.B. Dalsing M.C. Gottlieb K. Median arcuate ligament syndrome with multivessel involvement: diagnosis with spiral CT angiography.Abdom Imaging. 1997; 22: 318-320Crossref PubMed Scopus (40) Google Scholar, 3Taylor Jr, L.M. Moneta G.L. Management of visceral ischemic syndromes.in: Rutherford R.B. Vascular surgery. WB Saunders, Philadelphia2000: 1501-1511Google Scholar, 4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar, 5Takach T.J. Livesay J.J. Reul Jr, G.L. Cooley D.A. Celiac compression syndrome: tailored therapy based on intraoperative findings.J Am Coll Surg. 1996; 183: 606-610PubMed Google Scholar Typically, celiac artery compression is clinically characterized by weight loss, postprandial abdominal pain, and nausea and vomiting: symptoms believed to be secondary to intermittent foregut ischemia.3Taylor Jr, L.M. Moneta G.L. Management of visceral ischemic syndromes.in: Rutherford R.B. Vascular surgery. WB Saunders, Philadelphia2000: 1501-1511Google Scholar, 4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar, 5Takach T.J. Livesay J.J. Reul Jr, G.L. Cooley D.A. Celiac compression syndrome: tailored therapy based on intraoperative findings.J Am Coll Surg. 1996; 183: 606-610PubMed Google Scholar Atypical presentations of MAL syndrome are exceedingly variable and may range from exercise-related severe abdominal pain and diarrhea in elite athletes, fully reversed with surgical division of the constricting MAL,6Desmond C.P. Roberts S.K. Exercise-related abdominal pain as a manifestation of the median arcuate ligament syndrome.Scand J Gastroenterol. 2004; 39: 1310-1313Crossref PubMed Scopus (48) Google Scholar to rupture of a pancreaticoduodenal artery aneurysm, formed by the increasing poststenotic dilatation in the celiac axis caused by the MAL.7Akatsu T. Hayashi S. Yamane T. Yoshii H. Kitajima M. Emergency embolization of a ruptured pancreaticoduodenal artery aneurysm associated with the median arcuate ligament syndrome.J Gastroenterol Hepatol. 2004; 19: 482-483Crossref PubMed Scopus (16) Google Scholar, 8Habre J. Bernard J.L. Bereder J.M. Rahili A. Benchimol D. Bourgeon A. Rupture of a pancreaticoduodenal artery aneurysm with median arcuate ligament syndrome: report of a case.Ann Chir. 2005; 130 ([in French]): 178-180Crossref PubMed Scopus (7) Google Scholar Occasionally, in addition to the celiac artery, the constricting effects of MAL may be also exerted on the superior mesenteric artery and the renal arteries.9Kopecky K.K. Stine S.B. Dalsing M.C. Gottlieb K. Median arcuate ligament syndrome with multivessel involvement: diagnosis with spiral CT angiography.Abdom Imaging. 1997; 22: 318-320Crossref PubMed Scopus (63) Google Scholar Percutaneous transluminal angioplasty (PTA) of the celiac artery in MAL syndrome for postoperative stenosis10Saddekni S. Sniderman K.W. Hilton S. Sos T.A. Percutaneous transluminal angioplasty of nonatherosclerotic lesions.AJR Am J Roentgenol. 1980; 135: 975-982Crossref PubMed Scopus (74) Google Scholar or primarily11Matsumoto A.H. Tegtmeyer C.J. Fitzcharles E.K. Selby Jr, J.B. Tribble C.G. Angle J.F. et al.Percutaneous transluminal angioplasty of visceral arterial stenoses: results and long-term clinical follow-up.J Vasc Interv Radiol. 1995; 6: 165-174Abstract Full Text PDF PubMed Scopus (102) Google Scholar, 12Cina C.S. Safar H. Successful treatment of recurrent celiac axis compression syndrome A case report.Panminerva Med. 2002; 44: 69-72PubMed Google Scholar has been performed by different clinicians over the past 27 years. The increasing applicability of stent deployment for mesenteric occlusive disease, the associated technological advancements, and mounting evidence of acceptable mid-term patency rates13Sharafuddin M.J. Olson C.H. Sun S. Kresowik T.F. Corson J.D. Endovascular treatment of celiac and mesenteric arteries stenoses: applications and results.J Vasc Surg. 2003; 38: 692-698Abstract Full Text Full Text PDF PubMed Scopus (154) Google Scholar, 14Brown D.J. Schermerhorn M.L. Powell R.J. Fillinger M.F. Rzucidlo E.M. Walsh D.B. et al.Mesenteric stenting for chronic mesenteric ischemia.J Vasc Surg. 2005; 42: 268-274Abstract Full Text Full Text PDF PubMed Scopus (128) Google Scholar have given vascular interventionists the opportunity to expand the use of PTA and stenting to patients with MAL syndrome before or after primary surgical intervention and extrinsic tension relief.10Saddekni S. Sniderman K.W. Hilton S. Sos T.A. Percutaneous transluminal angioplasty of nonatherosclerotic lesions.AJR Am J Roentgenol. 1980; 135: 975-982Crossref PubMed Scopus (74) Google Scholar, 11Matsumoto A.H. Tegtmeyer C.J. Fitzcharles E.K. Selby Jr, J.B. Tribble C.G. Angle J.F. et al.Percutaneous transluminal angioplasty of visceral arterial stenoses: results and long-term clinical follow-up.J Vasc Interv Radiol. 1995; 6: 165-174Abstract Full Text PDF PubMed Scopus (102) Google Scholar, 12Cina C.S. Safar H. Successful treatment of recurrent celiac axis compression syndrome A case report.Panminerva Med. 2002; 44: 69-72PubMed Google Scholar, 13Sharafuddin M.J. Olson C.H. Sun S. Kresowik T.F. Corson J.D. Endovascular treatment of celiac and mesenteric arteries stenoses: applications and results.J Vasc Surg. 2003; 38: 692-698Abstract Full Text Full Text PDF PubMed Scopus (154) Google Scholar, 14Brown D.J. Schermerhorn M.L. Powell R.J. Fillinger M.F. Rzucidlo E.M. Walsh D.B. et al.Mesenteric stenting for chronic mesenteric ischemia.J Vasc Surg. 2005; 42: 268-274Abstract Full Text Full Text PDF PubMed Scopus (128) Google Scholar, 15Matsumoto A.H. Angle J.F. Spinosa D.J. Hagspiel K.D. Cage D.L. Leung D.A. et al.Percutaneous transluminal angioplasty and stenting in the treatment of chronic mesenteric ischemia: results and longterm followup.J Am Coll Surg. 2002; 194: S22-S31Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar We report on a young woman with MAL syndrome who, after three consecutive attempts of endovascular therapy with PTA and stenting, each one resulting in initial clinical improvement but followed by gross symptom recurrence, and a cumulative weight loss of 10 kg, had to undergo open surgical division of the MAL and reconstruction of the celiac artery. A 21-year-old woman presented with a history of postprandial abdominal pain and cramping, nausea and bloating associated with weight loss (10 kg), and constipation. Four months earlier she had been hospitalized (elsewhere) for acute postprandial abdominal pain. Esophagogastroduodenoscopy had revealed marked gastric dilatation and focal duodenal mucosal erosions. Mesenteric angiography had disclosed a 70% to 75% celiac artery stenosis. Celiac artery PTA and stenting resulted in clinical improvement at that time; however, symptoms recurred 2 months later as a result of restenosis distal to the previous stent site; repeat PTA and stenting was performed. Early remission was followed by recurrence within 6 weeks. At that time, the patient sought medical consultation at our institution. Meticulous gastroenterology (esophagogastroduodenoscopy, radionuclide gastric emptying, and gastroduodenal manometry) assessment proved unremarkable. Magnetic resonance (MR) angiography disclosed a high-grade (>75%) celiac artery restenosis between the two previous stents (Fig 1, Fig 2). The diagnosis of MAL syndrome was made on the basis of the presentation and underlying anatomy. In light of the young age of the patient, and because the two previous stents laid just above and below the celiac artery stenosis, thus forming obvious landing zones for stent deployment, our institution’s interventional radiologists believed that a third endovascular procedure with a stiff, balloon-expandable stent was worth considering before open surgery. The option of releasing the extrinsic compression on the celiac artery laparoscopically before its PTA and stenting was not addressed at the time. A third celiac PTA and stenting resulted in improvement of abdominal bloating in the ensuing months, yet postprandial abdominal cramps, often lasting many hours, restricted food intake and thus prevented weight gain. A pinhole celiac restenosis was detected 6 months later, with a well-developed pancreaticoduodenal arcade on computed tomography (CT) angiography. Symptom exacerbation and psychiatric clearance of somatoform or eating disorders paved the way to elective surgery. Through a 10-cm-long upper midline abdominal incision, the aorta was reached at the level of the crura of diaphragm, and the MAL and adjacent ganglionic tissue encasing the celiac artery and the aorta were divided. Transection of the severely damaged stented segment of the celiac artery (Fig 3) was followed by its reconstruction with a-side-to-end aortoceliac 8-mm Hemashield Dacron interposition graft (DuPont, Wilmington, Del; Fig 4). Patch angioplasty at the hepatic artery takeoff with bovine pericardium was performed to widen the arterial lumen (Fig 4). Uneventful recovery and unimpaired celiac artery flow on CT angiography (Fig 5) was associated with relief of her symptoms. She remained free of the symptoms linked to MAL syndrome at her 28-month follow up.Fig 2Three-dimensional multichannel computed tomographic angiography discloses a high-grade celiac artery restenosis between the two previous stents.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 3After division of the crura of the diaphragm and the median arcuate ligament, the previous stents are removed from the celiac artery.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 4The severely damaged segment of the artery is transected, and its reconstruction is performed with a side-to-end aortoceliac 8-mm Hemashield Dacron interposition graft and patch plasty with bovine pericardial patch to the hepatic artery takeoff, with the aim of widening its lumen.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 5Three-dimensional multi channel computed tomographic angiography of the reconstructed celiac artery at follow-up, confirming its uncompromised patency.View Large Image Figure ViewerDownload Hi-res image Download (PPT) In the presence of significant clinical manifestations, radiographic evidence of MAL syndrome is offered traditionally with lateral mesenteric aortoangiography, yet recent advances in multichannel CT and MR imaging have enabled identification of celiac artery compression by using three-dimensional CT or MR angiography, respectively.1Horton K.M. Talamini M.A. Fishman E.K. Median arcuate ligament syndrome: evaluation with CT angiography.Radiographics. 2005; 25: 1177-1182Crossref PubMed Scopus (246) Google Scholar, 2Kopecky K.K. Stine S.B. Dalsing M.C. Gottlieb K. Median arcuate ligament syndrome with multivessel involvement: diagnosis with spiral CT angiography.Abdom Imaging. 1997; 22: 318-320Crossref PubMed Scopus (40) Google Scholar, 3Taylor Jr, L.M. Moneta G.L. Management of visceral ischemic syndromes.in: Rutherford R.B. Vascular surgery. WB Saunders, Philadelphia2000: 1501-1511Google Scholar, 4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar, 5Takach T.J. Livesay J.J. Reul Jr, G.L. Cooley D.A. Celiac compression syndrome: tailored therapy based on intraoperative findings.J Am Coll Surg. 1996; 183: 606-610PubMed Google Scholar, 16Lee V.S. Morgan J.N. Tan A.G. Pandharipande P.V. Krinsky G.A. Barker J.A. et al.Celiac artery compression by the median arcuate ligament: a pitfall of end-expiratory MR imaging.Radiology. 2003; 228: 437-442Crossref PubMed Scopus (52) Google Scholar CT, MR, or digital subtraction mesenteric arteriography reveal high-grade proximal celiac artery stenosis on inspiration.1Horton K.M. Talamini M.A. Fishman E.K. Median arcuate ligament syndrome: evaluation with CT angiography.Radiographics. 2005; 25: 1177-1182Crossref PubMed Scopus (246) Google Scholar, 2Kopecky K.K. Stine S.B. Dalsing M.C. Gottlieb K. Median arcuate ligament syndrome with multivessel involvement: diagnosis with spiral CT angiography.Abdom Imaging. 1997; 22: 318-320Crossref PubMed Scopus (40) Google Scholar, 3Taylor Jr, L.M. Moneta G.L. Management of visceral ischemic syndromes.in: Rutherford R.B. Vascular surgery. WB Saunders, Philadelphia2000: 1501-1511Google Scholar, 4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar, 5Takach T.J. Livesay J.J. Reul Jr, G.L. Cooley D.A. Celiac compression syndrome: tailored therapy based on intraoperative findings.J Am Coll Surg. 1996; 183: 606-610PubMed Google Scholar, 16Lee V.S. Morgan J.N. Tan A.G. Pandharipande P.V. Krinsky G.A. Barker J.A. et al.Celiac artery compression by the median arcuate ligament: a pitfall of end-expiratory MR imaging.Radiology. 2003; 228: 437-442Crossref PubMed Scopus (52) Google Scholar The characteristic focal narrowing in the proximal celiac artery, with its hooked appearance, aids in distinguishing MAL syndrome from other etiologies of stenosis.1Horton K.M. Talamini M.A. Fishman E.K. Median arcuate ligament syndrome: evaluation with CT angiography.Radiographics. 2005; 25: 1177-1182Crossref PubMed Scopus (246) Google Scholar, 2Kopecky K.K. Stine S.B. Dalsing M.C. Gottlieb K. Median arcuate ligament syndrome with multivessel involvement: diagnosis with spiral CT angiography.Abdom Imaging. 1997; 22: 318-320Crossref PubMed Scopus (40) Google Scholar, 3Taylor Jr, L.M. Moneta G.L. Management of visceral ischemic syndromes.in: Rutherford R.B. Vascular surgery. WB Saunders, Philadelphia2000: 1501-1511Google Scholar, 4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar, 5Takach T.J. Livesay J.J. Reul Jr, G.L. Cooley D.A. Celiac compression syndrome: tailored therapy based on intraoperative findings.J Am Coll Surg. 1996; 183: 606-610PubMed Google Scholar, 16Lee V.S. Morgan J.N. Tan A.G. Pandharipande P.V. Krinsky G.A. Barker J.A. et al.Celiac artery compression by the median arcuate ligament: a pitfall of end-expiratory MR imaging.Radiology. 2003; 228: 437-442Crossref PubMed Scopus (52) Google Scholar In 10% to 24% of people, the MAL may cross anterior to the artery,17Horton K.M. Talamini M.A. Fishman E.K. Median arcuate ligament syndrome: evaluation with CT angiography.Radiographics. 2005; 25: 1177-1182Crossref PubMed Scopus (78) Google Scholar whereas in 13% to 50%, angiographic features of celiac axis compression (to a variable degree) may be present.18Szilagyi D.E. Rian R.L. Elliott J.P. Smith R.F. The cardiac artery compression syndrome: does it exist?.Surgery. 1972; 72: 849-863PubMed Google Scholar, 19Bron K.M. Redman H.C. Splanchnic artery stenosis and occlusion: incidence, arteriographic, and clinical manifestations.Radiology. 1969; 92: 323-328PubMed Google Scholar Open3Taylor Jr, L.M. Moneta G.L. Management of visceral ischemic syndromes.in: Rutherford R.B. Vascular surgery. WB Saunders, Philadelphia2000: 1501-1511Google Scholar, 4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar, 5Takach T.J. Livesay J.J. Reul Jr, G.L. Cooley D.A. Celiac compression syndrome: tailored therapy based on intraoperative findings.J Am Coll Surg. 1996; 183: 606-610PubMed Google Scholar or laparoscopic20Dordoni L. Tshomba Y. Giacomelli M. Jannello A.M. Chiesa R. Celiac artery compression syndrome: successful laparoscopic treatment—a case report.Vasc Endovascular Surg. 2002; 36: 317-321Crossref PubMed Scopus (39) Google Scholar, 21Roayaie S. Jossart G. Gitlitz D. Lamparello P. Hollier L. Gagner M. Laparoscopic release of celiac artery compression syndrome facilitated by laparoscopic ultrasound scanning to confirm restoration of flow.J Vasc Surg. 2000; 32: 814-817Abstract Full Text Full Text PDF PubMed Scopus (101) Google Scholar surgical division of the MAL and dissection of the abundant celiac ganglionic tissue over the aorta, if present, relieve compression. Celiac or mesenteric artery dilation or reconstruction by interposition grafting, patch aortoplasty, or primary reanastomosis may be required in persisting stenosis after decompression.3Taylor Jr, L.M. Moneta G.L. Management of visceral ischemic syndromes.in: Rutherford R.B. Vascular surgery. WB Saunders, Philadelphia2000: 1501-1511Google Scholar, 4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar, 5Takach T.J. Livesay J.J. Reul Jr, G.L. Cooley D.A. Celiac compression syndrome: tailored therapy based on intraoperative findings.J Am Coll Surg. 1996; 183: 606-610PubMed Google Scholar Among 51 patients who underwent open surgery for symptomatic celiac artery compression, 44 (86%) were available for late follow-up (mean, 9 years; range, 1-18 years). Sustained symptom relief occurred more often with a preoperative postprandial pain pattern (81% cure), age between 40 and 60 years (77%), and weight loss of 20 pounds (10 Kg) or more (67%).4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar Clinical improvement was inversely correlated with an atypical pain pattern with periods of remission (43% cure), a history of a psychiatric disorder or alcohol abuse (40%), age greater than 60 years (40%), and weight loss of less than 20 pounds (53%).4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar Eight (53%) of 15 patients treated by celiac decompression alone remained asymptomatic at late follow-up, in contrast to 22 (76%) of 29 patients treated by celiac decompression plus celiac revascularization.4Reilly L.M. Ammar A.D. Stoney R.J. Ehrenfeld W.K. Late results following operative repair for celiac artery compression syndrome.J Vasc Surg. 1985; 2: 79-91PubMed Scopus (179) Google Scholar Late follow-up arteriograms revealed a widely patent celiac artery in 70% of asymptomatic patients but a narrowed or occluded celiac artery in 75% of the symptomatic ones. This report points at the partial, short-lived relief of symptoms and early celiac artery restenosis after repeat PTA and stenting in the presence of high-grade celiac compression generated by the anatomic and functional abnormality in MAL syndrome which compelled surgical treatment within months of the onset of symptoms in a 21-year-old woman. A literature search revealed a very limited account of pertinent clinical data. Twenty-seven years have elapsed since PTA of the celiac artery was performed for recurrent stenosis after open surgery for MAL syndrome (1980).10Saddekni S. Sniderman K.W. Hilton S. Sos T.A. Percutaneous transluminal angioplasty of nonatherosclerotic lesions.AJR Am J Roentgenol. 1980; 135: 975-982Crossref PubMed Scopus (74) Google Scholar Eighteen months after that procedure, the patient remained symptom-free. Fifteen years later (1995), Matsumoto et al11Matsumoto A.H. Tegtmeyer C.J. Fitzcharles E.K. Selby Jr, J.B. Tribble C.G. Angle J.F. et al.Percutaneous transluminal angioplasty of visceral arterial stenoses: results and long-term clinical follow-up.J Vasc Interv Radiol. 1995; 6: 165-174Abstract Full Text PDF PubMed Scopus (102) Google Scholar reported on seven PTA procedures for symptomatic visceral artery stenosis that were immediate failures, five of which were secondary to extrinsic arterial compression caused by the MAL or an occult malignancy.11Matsumoto A.H. Tegtmeyer C.J. Fitzcharles E.K. Selby Jr, J.B. Tribble C.G. Angle J.F. et al.Percutaneous transluminal angioplasty of visceral arterial stenoses: results and long-term clinical follow-up.J Vasc Interv Radiol. 1995; 6: 165-174Abstract Full Text PDF PubMed Scopus (102) Google Scholar More recently, the same team15Matsumoto A.H. Angle J.F. Spinosa D.J. Hagspiel K.D. Cage D.L. Leung D.A. et al.Percutaneous transluminal angioplasty and stenting in the treatment of chronic mesenteric ischemia: results and longterm followup.J Am Coll Surg. 2002; 194: S22-S31Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar reported on the immediate clinical failure of PTA in a patient with MAL syndrome who later had immediate relief of symptoms with surgical release of the MAL. Cina and Safar12Cina C.S. Safar H. Successful treatment of recurrent celiac axis compression syndrome A case report.Panminerva Med. 2002; 44: 69-72PubMed Google Scholar also performed celiac artery angioplasty without effect in a 62-year-old woman with a previous partial gastrectomy presenting with postprandial abdominal pain and marked weight loss; previous angiography had revealed celiac artery compression, and gastrointestinal tract investigation was unremarkable. The patient underwent surgical division of the MAL, with complete relief of symptoms, which recurred 4 months later. Angiography demonstrated a restenosis of the celiac artery. Complete and persistent relief of symptoms at 4 years’ follow-up was achieved with aortoceliac artery bypass grafting.12Cina C.S. Safar H. Successful treatment of recurrent celiac axis compression syndrome A case report.Panminerva Med. 2002; 44: 69-72PubMed Google Scholar These data indicate that secondary endovascular therapy in the celiac artery for MAL syndrome after its extrinsic compression has been relieved is associated with acceptable clinical outcomes, contrary to those linked to primary PTA and stenting without surgical release of compression. The traumatic effects of PTA on the vessel wall produce multiple tears and cracks in the intima and splits of various grades in the media, weaken the vessel wall, and render it more susceptible to collapse by external compression,22Sullivan T.M. Ainsworth S.D. Langan E.M. Taylor S. Snyder B. Cull D. et al.Effect of endovascular stent strut geometry on vascular injury, myointimal hyperplasia, and restenosis.J Vasc Surg. 2002; 36: 143-149Abstract Full Text PDF PubMed Scopus (74) Google Scholar thus adding to the vessel recoil that occurs invariably without the structural support of a stent.23Gardiner Jr, G.A. Bonn J. Sullivan K.L. Quantification of elastic recoil after balloon angioplasty in the iliac arteries.J Vasc Interv Radiol. 2001; 12: 1389-1393Abstract Full Text Full Text PDF PubMed Google Scholar However, stent deployment may be compromised by slippage, mechanical fatigue, and, ultimately, crashing secondary to the intense compression exerted by the MAL. Perhaps the potential benefits of the developing stent fabrication and drug-eluting stent technology could have significant implications in the prevention of intimal hyperplasia after stent deployment and mechanical fatigue of stent material, especially in patients with higher grades of extrinsic compression from the MAL.24Palmaz J.C. Intravascular stents in the last and the next 10 years.J Endovasc Ther. 2004; 11 ([review]): II200-II206PubMed Google Scholar, 25Nelken N. Schneider P.A. Advances in stent technology and drug-eluting stents.Surg Clin North Am. 2004; 84 ([review]): 1203-1236Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar With current standards, though, and in keeping with the fundamental precept of endovascular therapy, primary stent deployment should be avoided in the likely occurrence of extrinsic compression or motion. In this respect, the MAL syndrome could be compared with the thoracic outlet, the inguinal ligament, and perhaps the popliteal space. As treatment of this young patient has shown, the MAL syndrome proved to be an additional setting in which primary stenting would be unable to withstand the power of extrinsic forces with impunity. The MAL syndrome has been disputed historically, not only on grounds of its pathophysiology, which remains largely obscure, but also in the absence of a clear causal relationship between treatment and symptom relief. On the basis of gastric Pco2 measurements during a 10-minute bicycle exercise, the detection of patients with gastrointestinal ischemia due to MAL-related celiac artery compression was recently reported.26Mensink P.B.F. van Petersen A.S. Kolkman J. Otte J.A. Huisman A.B. Geelkerken R.H. Gastric exercise tonometry: the key investigation in patients with suspected celiac artery compression syndrome.J Vasc Surg. 2006; 44: 277-281Abstract Full Text Full Text PDF PubMed Scopus (94) Google Scholar Accordingly, gastric stress testing enabled identification of the true symptomatic individuals with MAL syndrome, and after open surgical MAL division, 83% remained symptom free after a median follow-up of 39 months, thus adding to the objective, quantitative evidence on the validity of MAL syndrome. Distinct differences in background pathophysiology suggest that the favorable clinical efficacy of endovascular therapy in atherosclerotic mesenteric occlusive disease13Sharafuddin M.J. Olson C.H. Sun S. Kresowik T.F. Corson J.D. Endovascular treatment of celiac and mesenteric arteries stenoses: applications and results.J Vasc Surg. 2003; 38: 692-698Abstract Full Text Full Text PDF PubMed Scopus (154) Google Scholar, 14Brown D.J. Schermerhorn M.L. Powell R.J. Fillinger M.F. Rzucidlo E.M. Walsh D.B. et al.Mesenteric stenting for chronic mesenteric ischemia.J Vasc Surg. 2005; 42: 268-274Abstract Full Text Full Text PDF PubMed Scopus (128) Google Scholar is not applicable to MAL syndrome, although both entities may involve the celiac artery, superior mesenteric artery, or both. This report highlights the sound failure of primary PTA and stenting for high-grade celiac artery stenosis in MAL syndrome without prior surgical release of the extrinsic compression exerted on the vessel wall by the MAL and/or stiff para-aortic ganglionic tissue. In the presence of excellent superior mesenteric artery flow, the intensity of symptoms associated with MAL syndrome points at a multifactorial etiology, pertaining not only to the mesenteric circulation, but also to other functions, such as neurogenic and/or endocrine functions, as some investigators have alluded to.27Trinidad-Hernandez M. Keith P. Habib I. White J.V. Reversible gastroparesis: functional documentation of celiac axis compression syndrome and postoperative improvement.Am Surg. 2006; 72: 339-344PubMed Google Scholar, 28Balaban D.H. Chen J. Lin Z. Tribble C.G. McCallum R.W. Median arcuate ligament syndrome: a possible cause of idiopathic gastroparesis.Am J Gastroenterol. 1997; 92: 519-523PubMed Google Scholar
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