From the New England Society for Vascular Surgery 2014 Darling Award
Contemporary management of median arcuate ligament syndrome provides early symptom improvement Jesse A. Columbo, MD,a Thadeus Trus, MD,b Brian Nolan, MD, MS,a Philip Goodney, MD, MS,a Eva Rzucidlo, MD,a Richard Powell, MD,a Daniel Walsh, MD,a and David Stone, MD,a Lebanon, NH Objective: Optimal diagnosis and management of median arcuate ligament (MAL) syndrome (MALS) remains unclear in contemporary practice. The advent and evolution of laparoscopic and endovascular techniques has redirected management toward a less invasive therapeutic algorithm. This study examined our contemporary outcomes of patients treated for MALS. Methods: All patients treated for MALS at Dartmouth-Hitchcock Medical Center from 2000 to 2013 were retrospectively reviewed. Demographics and comorbidities were recorded. Freedom from symptoms and freedom from reintervention were the primary end points. Return to work or school was assessed. Follow-up by clinic visits and telephone allowed quantitative comparisons among the patients. Results: During the study interval, 21 patients (24% male), with a median age of 42 years, were treated for MALS. All patients complained of abdominal pain in the presence of a celiac stenosis, 16 (76%) also reported weight loss at the time of presentation, and 57% had a concomitant psychiatric history. Diagnostic imaging most commonly used included duplex ultrasound (81%), computed tomography angiography (66%), angiography (57%), and magnetic resonance angiography (5%). Fourteen patients (67%) underwent multiple diagnostic studies. All patients underwent initial laparoscopic MAL release. Seven patients (33%) underwent subsequent celiac stent placement in the setting of recurrent or unresolved symptoms with persistent celiac stenosis at a mean interval of 49 days. Two patients required surgical bypass after an endovascular intervention failed. The 6-month freedom from symptoms was 75% and freedom from reintervention was 64%. Eighteen patients (81%) reported early symptom improvement and weight gain, and 66% were able to return to work. Conclusions: A multidisciplinary treatment approach using initial laparoscopic release and subsequent stent placement and bypass surgery provides symptom improvement in most patients treated for MALS. The potential placebo effect, however, remains uncertain. A significant minority of patients will require reintervention, justifying longitudinal surveillance and prudent patient selection. Patients can anticipate functional recovery, weight gain, and return to work with treatment. (J Vasc Surg 2015;62:151-6.)
The optimal diagnosis and management of median arcuate ligament (MAL) syndrome (MALS) remains unclear in contemporary practice. Accordingly, variation in the workup and treatment of MALS patients persists, reflecting a myriad of diagnostic studies and therapeutic interventions.1,2 Furthermore, the optimal surgical approach remains unclear, even in the setting of an established diagnosis of MALS. Recent literature highlights such variation in practice among this often young patient cohort, From the Sections of Vascular Surgerya and Minimally Invasive Surgery,b Dartmouth-Hitchcock Medical Center. Author conflict of interest: none. Presented during the Plenary Session at the Joint Annual Meeting of the New England Society for Vascular Surgery and Eastern Vascular Society, Mass, September 11-14, 2014. Reprint requests: Jesse A. Columbo, MD, Section of Vascular Surgery, Dartmouth-Hitchcock Medical Center, One Medical Center Dr, Lebanon, NH 03756 (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214 Copyright Ó 2015 by the Society for Vascular Surgery. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jvs.2015.01.050
with some studies still advocating routine diagnostic angiography and the prevalent use of open surgical MAL release. In addition, other studies have documented an w10% open conversion rate for laparoscopically treated patients, leading to ongoing debate about the optimal surgical approach.3-12 The advent and evolution of laparoscopic techniques has also introduced minimally invasive general surgeons into the management fold of MALS patients, who in fact, will often evaluate patients antecedently to vascular surgical consultation. Furthermore, definitive management of recalcitrant symptoms with percutaneous techniques vs the role for definitive mesenteric bypass in this young patient cohort remains unknown.3,4,12-15 Therefore, the purpose of this study was to better define the optimal diagnostic and management algorithm for patients undergoing MALS treatment and report contemporary clinical and functional outcomes. METHODS This study was conducted in compliance with the Dartmouth Geisel School of Medicine Institutional Review Board. All patients were deidentified and thus specific consent for publication was not required by our protocol. 151
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Table. Patient demographics, diagnostic, and therapeutic details and outcomes Interventions Pt.
Sex
Age, years
OR date
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21
M F F F F F M M F F F F M F F F F F F F M
26 42 49 20 43 20 29 59 23 21 49 27 49 60 46 26 36 69 51 16 50
Aug ’04 Sep ’08 Jan ’09 Feb ’09 Apr ’09 May ’09 Mar ’10 Dec ’10 Jun ’12 Sep ’12 Jan ’13 Jan ’13 Mar ’13 Apr ’13 Apr ’13 May ’13 Jul ’13 Jul ’13 Aug ’13 Sep ’13 Oct ’13
Presenting symptoms Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain Pain, Pain, Pain,
N, D weight loss N, weight loss N, D N, D, weight loss D, weight loss N, D N, D, weight loss N, weight loss N, weight loss N, weight loss N, weight loss N, D, weight loss N, weight loss D, weight loss N, weight loss N, weight loss N, D, weight loss N N, D, weight loss
Psych diagnosis No No Yes Yes Yes No Yes No No No Yes No Yes Yes Yes Yes No No Yes Yes Yes
Study CA DUS, CTA, DUS, CTA, DUS, CTA, DUS DUS, CTA, DUS, CA DUS, CTA, MRA DUS DUS, CTA, DUS, CTA, DUS DUS, CTA, DUS, CTA, DUS, CTA DUS, CTA CTA, CA CTA DUS DUS, CTA
CA CA CA CA CA CA CA CA CA
Total
Final
Symptoms at last visit
2 4 4 1 1 1 2 2 1 1 2 1 1 1 1 1 1 1 2 1 1
CS MB MB LMR LMR LMR CS CS LMR LMR CS LMR LMR LMR LMR LMR LMR LMR CS LMR LMR
Improved Improved Improved Improved Improved Improved Improved Improved Improved Unchanged Improved Improved Unchanged Unchanged Improved Improved Improved Improved Improved Improved Improved
CA, Conventional angiography; CS, celiac stenting; CTA, computed tomography angiography; D, diarrhea; DUS, duplex ultrasound; F, female; LMR, laparoscopic median arcuate ligament release; M, male; MB, mesenteric bypass; N, nausea; OR, operating room; pain, intermittent abdominal pain; Pt, patient.
Subjects and data collection. All patients treated for MALS at Dartmouth-Hitchcock Medical Center from 2000 to 2013 were identified from the Section of Vascular Surgery and Section of Minimally Invasive Surgery databases (n ¼ 21). Patient demographics and comorbidities were collected. Presenting symptoms and the presence or absence of weight loss was recorded. Preoperative imaging protocols and laparoscopic vs open surgical MAL release practice patterns were assessed. Celiac artery stenosis was defined as peak systolic velocity >200 cm/s or end diastolic velocity >55 cm/s.16 Outcomes and end points. The primary study end points included freedom from symptoms, freedom from reintervention, and return to work or school as a broad proxy for functional quality of life. Postoperative return to work or school was determined at the time of the follow-up clinic visit or by telephone interview. Mean values were reported where data were normally distributed, and median values were incorporated in the setting of potential variance. Statistical analysis. Kaplan-Meier life-table analysis was used to determine freedom from symptoms and freedom from reintervention. Statistical analyses were performed using Stata software (StataCorp LP, College Station, Tex). RESULTS We retrospectively identified 21 patients who were treated for MALS during the study interval. This represented the entire institutional operative experience for MALS. All laparoscopic procedures were performed by a
single minimally invasive surgeon. The distribution of procedures was weighted toward the latter half of the study interval (Table). The median age at diagnosis was 42 years (range, 16-69 years), and 16 patients (76%) were female. Given their relative young age, few conventional vascular risk factors and comorbidities were observed, including diabetes mellitus, coronary artery disease, congestive heart failure, chronic renal insufficiency, or chronic obstructive pulmonary disease. Six patients (29%) had a history of smoking. No patients had evidence of superior mesenteric artery disease. Interestingly, 12 patients (57%) maintained a coexisting psychiatric diagnosis at the time of presentation, and six (29%) were taking antidepressants at the time of diagnosis. All patients uniformly complained of abdominal pain at presentation, which was postprandial in nature in 18 of 21 (86%). Also common was concomitant nausea in 17 (81%) and diarrhea in 10 (47%). Observed weight loss was both prevalent (16 of 21 [76%]) and variable among patients, ranging from 2.7 to 27 kilograms during the year before presentation. Quantifiable absolute weight loss was known in 14 patients (67%) and had a median value of 9 kilograms. Nine patients had weight loss of $9 kilograms. Median body mass index (BMI) was generally low in this cohort at 20 kg/m2 (range, 14.5-33.8 kg/m2) and available in 20 patients (95%). BMI and weight loss, when known, did not appear to be predictive of reintervention or postoperative symptom improvement. Preoperative imaging studies varied among patients. Duplex ultrasound imaging was the most common (17 of 21 [81%]) first-line modality over time. Multiple imaging
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Fig 1. Kaplan-Meier 6-month freedom from (A) symptoms and (B) reintervention. SE, Standard error.
studies were obtained in 66% of patients. These included computed tomography angiography in 14 (66%), conventional angiography in 11 (57%), and magnetic resonance angiography in one (5%). Ten patients underwent multiple angiographic imaging studies. In some cases this reflected imaging obtained before the vascular surgical consultation. In others, multiple studies were obtained at the request of the consulting surgeon. Duplex ultrasound imaging was the only preoperative imaging study in four patients. All 21 patients underwent initial laparoscopic MAL release, reflecting our institutional bias toward an initial minimally invasive approach. Laparoscopic MAL release was well tolerated among all patients, with no observed incidence of myocardial infarction, renal demise, or mortality. The median length of stay associated with a laparoscopic MAL release was 2 days. Median blood loss using a laparoscopic technique was low (5 mL). Median operative time was 118 minutes. No patients required reoperation for bleeding complications, and there were no instances of perioperative arterial thrombosis. No procedures required emergency open surgical conversion. Eighteen patients (81%) experienced immediate postoperative symptom improvement after laparoscopic MAL release, with associated weight gain and complete resolution of abdominal pain. Data on return to work or school was available for 19 of 21 patients (90%). Of these, 14 experienced durable return to work or school postoperatively. Median follow-up for the cohort was 197 days (interquartile range, 466 days). Length of follow-up did not affect treatment success or failure. At the most recent follow-up, the observed 6-month freedom from symptoms was 75% and from reintervention was 64% (Fig 1). Seven patients whose symptoms initially resolved after laparoscopic release ultimately required percutaneous transluminal angioplasty (PTA) with celiac artery stent placement for a recurrent symptomatic stenosis. Balloonexpandable bare metal stents were used in all cases at the time of the initial endovascular intervention. No patients were treated with angioplasty alone. Five (71%) remained symptom free at their most recent follow-up. Conversely, one patient underwent balloon expandable stent grafting 5 months after initial stenting, and a second underwent
repeat angioplasty of a stent 90 days after placement. Despite repeat percutaneous intervention and initial improvement, both patients had recurrent symptoms and ultimately underwent aortoceliac bypass using a 7-mm Dacron (DuPont, Wilmington, Del) graft (Table). Of note, 10 of 12 patients, with a coexisting psychiatric condition at the time of diagnosis experienced improvement in their respective symptoms with associated weight gain at the time of their most recent follow-up, whereas two patients in this subgroup reported persistent abdominal pain and concomitant weight loss. These patients ultimately underwent diagnostic angiography and were determined to have no residual underlying celiac arterial defect. Accordingly, these patients were subsequently referred to gastroenterology for further work-up and alternative management. DISCUSSION This study effectively demonstrates the successful diagnosis and treatment of MALS using a multidisciplinary approach predicated on astute diagnosis often relying on initial duplex ultrasound imaging coupled with an initial laparoscopic surgical approach. Perhaps more importantly, this study documents that most patients can anticipate a high likelihood for functional improvement with return to work or school, although a percentage of patients are likely to require a subsequent intervention. Interestingly, a significant percentage of MALS patients in this series also had an associated psychiatric diagnosis, a risk factor that is uncommonly reported in any recent literature.4,7,12,17 The implications of this observation remain unknown but may confound optimal patient selection or the effect of any placebo effect. Optimal patient selection for MALS treatment has remained historically elusive.7 This likely reflects the variation and evolution of diagnostic and therapeutic tools at the surgeon’s disposal over time, thus perpetuating variation in work-up and treatment. Moreover, as mentioned, prevalent coexisting psychiatric history has precluded any consistent patterns of presentation. Finally, patient selection has remained an additional challenge in the setting of prevalent asymptomatic anatomic celiac compression
154 Columbo et al
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Fig 2. A, Intraoperative photograph shows the laparoscopic approach to the celiac axis. B, Laparoscopic division of median arcuate ligament (MAL) fibers. Note celiac stenosis and poststenotic dilatation (arrow).
findings demonstrated on widespread imaging studies obtained for alternative indications.18 As noted previously, variation also persists in the diagnostic work-up and treatment paradigms for patients with symptoms suggestive of MALS, which may have implications surrounding timely referral to a vascular specialist. Not infrequently, patients may have seen multiple providers before consulting a vascular surgeon, necessitating unnecessary tests before definitive management. Specifically, this testing may include a gamut of gastrointestinal studies, including endoscopy, gastrointestinal series, computed tomography, magnetic resonance, and even gastric exercise tonometry.1,2,9,10,19 As demonstrated in this study, several patients with a clinical picture and duplex ultrasound consistent with MALS did not require additional imaging or consultation, although others underwent additional imaging given the variability in presentation. The role of laparoscopy in treating MALS has increased over time, supplanting open surgical reconstruction in most circumstances as an initial approach. In our study, a laparoscopic approach was associated with low perioperative morbidity, short length of stay, and minimal blood loss, consistent with other reports.3-6,12-14,19 A minimally invasive surgical approach has obvious appeal in this relatively young, active patient population. Other management strategies have also been reported, however. Van Petersen et al9 demonstrated satisfactory results in a series of MALS patients managed with a retroperitoneal endoscopic MAL release, with 83% of operative patients experiencing symptom improvement. In addition, Relles et al10 reported a roboticassisted MAL release technique to be effective, albeit in a small cohort of patients, which has not been widely adopted. Accordingly, we have incorporated with success an initial laparoscopic approach to ligament release. In addition to excellent perioperative outcomes, this technique permits excellent, if not superior, visualization of the celiac origin and the MAL, thereby permitting safe and effective anatomic release (Fig 2). Interestingly, although several historical series reported an expected open conversion rate of 0% to 26%,3,4,6,8,12-14 no patients in this series required open surgery at their initial operation.
Despite successful laparoscopy, a significant number of patients can anticipate subsequent interventions. Not infrequently, a persistent anatomic compression of the celiac artery can persist despite a thorough dissection of the celiac artery and MAL release (Fig 3. A). Seven patients in this study demonstrated a persistent symptomatic celiac stenosis, ultimately prompting subsequent angiography and intervention in the setting of recurrent symptoms at variable lengths of follow-up. All of these patients were ultimately treated with percutaneous stent placement at a median interval of 48 days. The patient shown in Fig 3, B underwent successful PTA and stent placement with resolution of the anatomic defect and associated symptoms. Five of these patients remain improved on the most recent evaluation. Conversely, metachronous recurrent symptoms after a PTA intervention prompted consideration for mesenteric bypass, which was ultimately performed in two patients at a mean interval of 8 months after MAL release. Although mesenteric bypass is typically at the end of a thorough treatment algorithm for MALS patients, it is associated with excellent results in this highly selective subgroup, which was seen in our series and has been described in previous reports,4,12,13,15 with both patients in our cohort doing well at 4 years. On the basis of this experience, we have adopted a multidisciplinary diagnostic and therapeutic algorithm (Fig 4). Patients referred with symptoms compatible with MALS are evaluated with initial duplex ultrasound imaging in which provocative testing is performed during inhalation and exhalation. If positive duplex findings suggest the presence of a celiac stenosis in the context of symptoms consistent with MALS, the patient is considered for potential laparoscopic MAL release. Those presenting with an atypical constellation of symptoms or who demonstrate equivocal duplex findings undergo more definitive imaging with computed tomography angiography, although conventional angiography or magnetic resonance angiography remain acceptable alternatives. Patients without evidence of celiac stenosis or compression are subsequently referred to gastroenterology for further work-up for alternative diagnoses.
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Fig 3. A, Diagnostic angiography shows a persistent celiac artery defect (arrow) after laparoscopic median arcuate ligament (MAL) release in the setting of recurrent symptoms. B, Technically successful celiac artery stenting of the persistent defect.
Fig 4. Multidisciplinary clinical diagnostic and therapeutic algorithm. CT, Computed tomography; GI, gastrointestinal; MAL, median arcuate ligament; PTA, percutaneous transluminal angioplasty.
Patients treated with laparoscopic ligament release are subsequently followed up with a routine visit at 1 month. Patients who are asymptomatic at follow-up do not undergo routine longitudinal surveillance in the absence of symptoms. Repeat duplex imaging is reserved for patients who have persistent or recurrent symptoms. Patients with evidence of persistent or recurrent stenosis or those with ongoing symptoms are considered for conventional angiography. If angiographic evidence of a persistent celiac stenosis is identified in the setting of recurrent symptoms, patients may undergo percutaneous intervention at the discretion of the surgeon. In this series, seven patients ultimately required PTA with stent placement. Although the durability of endovascular mesenteric intervention has historically remained a just cause for concern, most of these series reported outcomes among a different patient population with atherosclerotic occlusive disease, thus potentially limiting their applicability to a MALS patient
population.4,6,12,13 Moreover, there did not appear to be any major adverse clinical sequelae among this cohort. However, we note that the experience of this patient subset remains small, with a paucity of long-term follow-up. Finally, in the setting of a failed percutaneous intervention and recalcitrant symptoms, patients should be ultimately considered for mesenteric bypass. This algorithmic multidisciplinary approach has demonstrated good clinical outcomes with low morbidity. As noted, most patients can anticipate immediate symptom improvement after laparoscopic release (18 of 21 [86%]), with a high likelihood (14 of 21 [67%]) of returning to work or school. Furthermore, this approach is associated with acceptable perioperative and 6-month freedom from symptoms and freedom from reintervention, although we emphasize that a subset of MALS patients will have recurrent symptoms necessitating subsequent intervention. Although previous series have reported a substantial open surgical conversion rate,3,4,6,12-14 this trend was not observed at our institution, which may partly reflect the ongoing evolution of laparoscopic technology and techniques. This study has several intrinsic limitations. First, the true prevalence of MALS seen at our institution remains unknown because of the retrospective nature of the study. This is often because many patients are never referred for surgical evaluation or in other cases because another etiology for abdominal pain is found and thus no diagnosis of MALS is assigned. Secondly, although a thorough preoperative evaluation is required, we cannot account for the nuances in assessment performed by all nonprocedural specialists involved. In addition, the small size of the study cohort limits our ability to derive broader more generalizable conclusions. Nevertheless, this study does depict contemporary results, which we believe are applicable in current practice. Moreover, prior reports had similar sample sizes given the
156 Columbo et al
relative rarity of the condition. We cannot make conclusions surrounding the durability of reinterventions in these patients given the small subset who required them. Longer follow-up among a larger cohort of MALS patients will be required to better address the durability of such interventions. In addition, the substantial prevalence of coexisting psychiatric illness documented in our cohort may confound accurate diagnosis, treatment, and follow-up. We are unable to comment on any quantifiable amount of weight gain after intervention. However, we would emphasize that symptom improvement, marked by abrogation of abdominal pain or associated qualitative weight gain, or both, is more reflective of treatment success rather than any specific quantity of gained weight. Finally, the effect of any placebo effect in this atypical patient cohort remains unknown. CONCLUSIONS Patients will often require multiple imaging studies to establish a diagnosis of MALS, although initial duplex ultrasound imaging appears to be a sensitive first option. Laparoscopic MAL release is the preferred contemporary surgical approach, providing superior visualization of the celiac axis. Most MALS patients can anticipate effective symptom relief postoperatively, although dedicated longitudinal surveillance is warranted because a subset of patients will likely experience recurrent symptoms warranting potential subsequent intervention. Although the effect of underlying psychiatric disease and the placebo effect remains unknown, it is difficult to overlook their potential confounding in managing this challenging subset of patients. Accordingly, a dedicated multidisciplinary MALS diagnostic and treatment algorithm appears to provide effective treatment in this often clinically desperate and debilitated patient population. AUTHOR CONTRIBUTIONS Conception and design: JC, DS Analysis and interpretation: JC, BN, DS Data collection: JC Writing the article: JC, DS Critical revision of the article: JC, TT, BN, PG, ER, RP, DW, DS Final approval of the article: JC, DS Statistical analysis: BN Obtained funding: Not applicable Overall responsibility: DS REFERENCES 1. Gruber H, Loizides A, Peer S, Gruber I. Ultrasound of the median arcuate ligament syndrome: a new approach to diagnosis. Med Ultrason 2012;14:5-9.
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2. Mensink PB, van Petersen AS, Kolkman JJ, Otte JA, Huisman AB, Geelkerken RH. Gastric exercise tonometry: the key investigation in patients with suspected celiac artery compression syndrome. J Vasc Surg 2006;44:277-81. 3. Jimenez JC, Harlander-Locke M, Dutson EP. Open and laparoscopic treatment of median arcuate ligament syndrome. J Vasc Surg 2012;56: 869-73. 4. Roseborough GS. Laparoscopic management of celiac artery compression syndrome. J Vasc Surg 2009;50:124-33. 5. Vaziri K, Hungness ES, Pearson EG, Soper NJ. Laparoscopic treatment of celiac artery compression syndrome: case series and review of current treatment modalities. J Gastrointest Surg 2009;13:293-8. 6. El-Hayek KM, Titus J, Bui A, Mastracci T, Kroh M. Laparoscopic median arcuate ligament release: are we improving symptoms? J Am Coll Surg 2013;216:272-9. 7. Williams S, Gillespie P, Little JM. Celiac axis compression syndrome: factors predicting a favorable outcome. Surgery 1985;98: 879-87. 8. Aschenbach R, Basche S, Vogl TJ. Compression of the celiac trunk caused by median arcuate ligament in children and adolescent subjects: evaluation with contrast-enhanced MR angiography and comparison with Doppler US evaluation. J Vasc Interv Radiol 2011;22: 556-61. 9. van Petersen AS, Vriens BH, Huisman AB, Kolkman JJ, Geelkerken RH. Retroperitoneal endoscopic release in the management of celiac artery compression syndrome. J Vasc Surg 2009;50: 140-7. 10. Relles D, Moudgill N, Rao A, Rosato F, DiMuzio P, Eisenberg J. Robotic-assisted median arcuate ligament release. J Vasc Surg 2012;56: 500-3. 11. Sultan S, Hynes N, Elsafty N, Tawfick W. Eight years experience in the management of median arcuate ligament syndrome by decompression, celiac ganglion sympathectomy, and selective revascularization. Vasc Endovasc Surg 2013;47:614-9. 12. Berard X, Cau J, Deglise S, Trombert D, Saint-Lebes B, Midy D, et al. Laparoscopic surgery for coeliac artery compression syndrome: current management and technical aspects. Eur J Vasc Endovasc Surg 2012;43: 38-42. 13. Baccari P, Civilini E, Dordoni L, Melissano G, Nicoletti R, Chiesa R. Celiac artery compression syndrome managed by laparoscopy. J Vasc Surg 2009;50:134-9. 14. Tulloch AW, Jimenez JC, Lawrence PF, Dutson EP, Moore WS, Rigberg DA, et al. Laparoscopic versus open celiac ganglionectomy in patients with median arcuate ligament syndrome. J Vasc Surg 2010;52: 1283-9. 15. Delis KT, Gloviczki P, Altuwaijri M, McKusick MA. Median arcuate ligament syndrome: open celiac artery reconstruction and ligament division after endovascular failure. J Vasc Surg 2007;46: 799-802. 16. Zwolak RM, Fillinger MF, Walsh DB, LaBombard FE, Musson A, Darling CE, et al. Mesenteric and celiac duplex scanning: a validation study. J Vasc Surg 1998;27:1078-87; discussion: 1088. 17. Reilly LM, Ammar AD, Stoney RJ, Ehrenfeld WK. Late results following operative repair for celiac artery compression syndrome. J Vasc Surg 1985;2:79-91. 18. Park CM, Chung JW, Kim HB, Shin SJ, Park JH. Celiac axis stenosis: incidence and etiologies in asymptomatic individuals. Korean J Radiol 2001;2:8-13. 19. Mak GZ, Speaker C, Anderson K, Stiles-Shields C, Lorenz J, Drossos T, et al. Median arcuate ligament syndrome in the pediatric population. J Pediatr Surg 2013;48:2261-70. Submitted Dec 3, 2014; accepted Jan 21, 2015.
Contemporary management of median arcuate ligament syndrome provides early symptom improvement Jesse A. Columbo, MD,a Thadeus Trus, MD,b Brian Nolan, MD, MS,a Philip Goodney, MD, MS,a Eva Rzucidlo, MD,a Richard Powell, MD,a Daniel Walsh, MD,a and David Stone, MD,a Lebanon, NH Objective: Optimal diagnosis and management of median arcuate ligament (MAL) syndrome (MALS) remains unclear in contemporary practice. The advent and evolution of laparoscopic and endovascular techniques has redirected management toward a less invasive therapeutic algorithm. This study examined our contemporary outcomes of patients treated for MALS. Methods: All patients treated for MALS at Dartmouth-Hitchcock Medical Center from 2000 to 2013 were retrospectively reviewed. Demographics and comorbidities were recorded. Freedom from symptoms and freedom from reintervention were the primary end points. Return to work or school was assessed. Follow-up by clinic visits and telephone allowed quantitative comparisons among the patients. Results: During the study interval, 21 patients (24% male), with a median age of 42 years, were treated for MALS. All patients complained of abdominal pain in the presence of a celiac stenosis, 16 (76%) also reported weight loss at the time of presentation, and 57% had a concomitant psychiatric history. Diagnostic imaging most commonly used included duplex ultrasound (81%), computed tomography angiography (66%), angiography (57%), and magnetic resonance angiography (5%). Fourteen patients (67%) underwent multiple diagnostic studies. All patients underwent initial laparoscopic MAL release. Seven patients (33%) underwent subsequent celiac stent placement in the setting of recurrent or unresolved symptoms with persistent celiac stenosis at a mean interval of 49 days. Two patients required surgical bypass after an endovascular intervention failed. The 6-month freedom from symptoms was 75% and freedom from reintervention was 64%. Eighteen patients (81%) reported early symptom improvement and weight gain, and 66% were able to return to work. Conclusions: A multidisciplinary treatment approach using initial laparoscopic release and subsequent stent placement and bypass surgery provides symptom improvement in most patients treated for MALS. The potential placebo effect, however, remains uncertain. A significant minority of patients will require reintervention, justifying longitudinal surveillance and prudent patient selection. Patients can anticipate functional recovery, weight gain, and return to work with treatment. (J Vasc Surg 2015;62:151-6.)
The optimal diagnosis and management of median arcuate ligament (MAL) syndrome (MALS) remains unclear in contemporary practice. Accordingly, variation in the workup and treatment of MALS patients persists, reflecting a myriad of diagnostic studies and therapeutic interventions.1,2 Furthermore, the optimal surgical approach remains unclear, even in the setting of an established diagnosis of MALS. Recent literature highlights such variation in practice among this often young patient cohort, From the Sections of Vascular Surgerya and Minimally Invasive Surgery,b Dartmouth-Hitchcock Medical Center. Author conflict of interest: none. Presented during the Plenary Session at the Joint Annual Meeting of the New England Society for Vascular Surgery and Eastern Vascular Society, Mass, September 11-14, 2014. Reprint requests: Jesse A. Columbo, MD, Section of Vascular Surgery, Dartmouth-Hitchcock Medical Center, One Medical Center Dr, Lebanon, NH 03756 (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214 Copyright Ó 2015 by the Society for Vascular Surgery. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jvs.2015.01.050
with some studies still advocating routine diagnostic angiography and the prevalent use of open surgical MAL release. In addition, other studies have documented an w10% open conversion rate for laparoscopically treated patients, leading to ongoing debate about the optimal surgical approach.3-12 The advent and evolution of laparoscopic techniques has also introduced minimally invasive general surgeons into the management fold of MALS patients, who in fact, will often evaluate patients antecedently to vascular surgical consultation. Furthermore, definitive management of recalcitrant symptoms with percutaneous techniques vs the role for definitive mesenteric bypass in this young patient cohort remains unknown.3,4,12-15 Therefore, the purpose of this study was to better define the optimal diagnostic and management algorithm for patients undergoing MALS treatment and report contemporary clinical and functional outcomes. METHODS This study was conducted in compliance with the Dartmouth Geisel School of Medicine Institutional Review Board. All patients were deidentified and thus specific consent for publication was not required by our protocol. 151
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Table. Patient demographics, diagnostic, and therapeutic details and outcomes Interventions Pt.
Sex
Age, years
OR date
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21
M F F F F F M M F F F F M F F F F F F F M
26 42 49 20 43 20 29 59 23 21 49 27 49 60 46 26 36 69 51 16 50
Aug ’04 Sep ’08 Jan ’09 Feb ’09 Apr ’09 May ’09 Mar ’10 Dec ’10 Jun ’12 Sep ’12 Jan ’13 Jan ’13 Mar ’13 Apr ’13 Apr ’13 May ’13 Jul ’13 Jul ’13 Aug ’13 Sep ’13 Oct ’13
Presenting symptoms Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain, Pain Pain, Pain, Pain,
N, D weight loss N, weight loss N, D N, D, weight loss D, weight loss N, D N, D, weight loss N, weight loss N, weight loss N, weight loss N, weight loss N, D, weight loss N, weight loss D, weight loss N, weight loss N, weight loss N, D, weight loss N N, D, weight loss
Psych diagnosis No No Yes Yes Yes No Yes No No No Yes No Yes Yes Yes Yes No No Yes Yes Yes
Study CA DUS, CTA, DUS, CTA, DUS, CTA, DUS DUS, CTA, DUS, CA DUS, CTA, MRA DUS DUS, CTA, DUS, CTA, DUS DUS, CTA, DUS, CTA, DUS, CTA DUS, CTA CTA, CA CTA DUS DUS, CTA
CA CA CA CA CA CA CA CA CA
Total
Final
Symptoms at last visit
2 4 4 1 1 1 2 2 1 1 2 1 1 1 1 1 1 1 2 1 1
CS MB MB LMR LMR LMR CS CS LMR LMR CS LMR LMR LMR LMR LMR LMR LMR CS LMR LMR
Improved Improved Improved Improved Improved Improved Improved Improved Improved Unchanged Improved Improved Unchanged Unchanged Improved Improved Improved Improved Improved Improved Improved
CA, Conventional angiography; CS, celiac stenting; CTA, computed tomography angiography; D, diarrhea; DUS, duplex ultrasound; F, female; LMR, laparoscopic median arcuate ligament release; M, male; MB, mesenteric bypass; N, nausea; OR, operating room; pain, intermittent abdominal pain; Pt, patient.
Subjects and data collection. All patients treated for MALS at Dartmouth-Hitchcock Medical Center from 2000 to 2013 were identified from the Section of Vascular Surgery and Section of Minimally Invasive Surgery databases (n ¼ 21). Patient demographics and comorbidities were collected. Presenting symptoms and the presence or absence of weight loss was recorded. Preoperative imaging protocols and laparoscopic vs open surgical MAL release practice patterns were assessed. Celiac artery stenosis was defined as peak systolic velocity >200 cm/s or end diastolic velocity >55 cm/s.16 Outcomes and end points. The primary study end points included freedom from symptoms, freedom from reintervention, and return to work or school as a broad proxy for functional quality of life. Postoperative return to work or school was determined at the time of the follow-up clinic visit or by telephone interview. Mean values were reported where data were normally distributed, and median values were incorporated in the setting of potential variance. Statistical analysis. Kaplan-Meier life-table analysis was used to determine freedom from symptoms and freedom from reintervention. Statistical analyses were performed using Stata software (StataCorp LP, College Station, Tex). RESULTS We retrospectively identified 21 patients who were treated for MALS during the study interval. This represented the entire institutional operative experience for MALS. All laparoscopic procedures were performed by a
single minimally invasive surgeon. The distribution of procedures was weighted toward the latter half of the study interval (Table). The median age at diagnosis was 42 years (range, 16-69 years), and 16 patients (76%) were female. Given their relative young age, few conventional vascular risk factors and comorbidities were observed, including diabetes mellitus, coronary artery disease, congestive heart failure, chronic renal insufficiency, or chronic obstructive pulmonary disease. Six patients (29%) had a history of smoking. No patients had evidence of superior mesenteric artery disease. Interestingly, 12 patients (57%) maintained a coexisting psychiatric diagnosis at the time of presentation, and six (29%) were taking antidepressants at the time of diagnosis. All patients uniformly complained of abdominal pain at presentation, which was postprandial in nature in 18 of 21 (86%). Also common was concomitant nausea in 17 (81%) and diarrhea in 10 (47%). Observed weight loss was both prevalent (16 of 21 [76%]) and variable among patients, ranging from 2.7 to 27 kilograms during the year before presentation. Quantifiable absolute weight loss was known in 14 patients (67%) and had a median value of 9 kilograms. Nine patients had weight loss of $9 kilograms. Median body mass index (BMI) was generally low in this cohort at 20 kg/m2 (range, 14.5-33.8 kg/m2) and available in 20 patients (95%). BMI and weight loss, when known, did not appear to be predictive of reintervention or postoperative symptom improvement. Preoperative imaging studies varied among patients. Duplex ultrasound imaging was the most common (17 of 21 [81%]) first-line modality over time. Multiple imaging
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Fig 1. Kaplan-Meier 6-month freedom from (A) symptoms and (B) reintervention. SE, Standard error.
studies were obtained in 66% of patients. These included computed tomography angiography in 14 (66%), conventional angiography in 11 (57%), and magnetic resonance angiography in one (5%). Ten patients underwent multiple angiographic imaging studies. In some cases this reflected imaging obtained before the vascular surgical consultation. In others, multiple studies were obtained at the request of the consulting surgeon. Duplex ultrasound imaging was the only preoperative imaging study in four patients. All 21 patients underwent initial laparoscopic MAL release, reflecting our institutional bias toward an initial minimally invasive approach. Laparoscopic MAL release was well tolerated among all patients, with no observed incidence of myocardial infarction, renal demise, or mortality. The median length of stay associated with a laparoscopic MAL release was 2 days. Median blood loss using a laparoscopic technique was low (5 mL). Median operative time was 118 minutes. No patients required reoperation for bleeding complications, and there were no instances of perioperative arterial thrombosis. No procedures required emergency open surgical conversion. Eighteen patients (81%) experienced immediate postoperative symptom improvement after laparoscopic MAL release, with associated weight gain and complete resolution of abdominal pain. Data on return to work or school was available for 19 of 21 patients (90%). Of these, 14 experienced durable return to work or school postoperatively. Median follow-up for the cohort was 197 days (interquartile range, 466 days). Length of follow-up did not affect treatment success or failure. At the most recent follow-up, the observed 6-month freedom from symptoms was 75% and from reintervention was 64% (Fig 1). Seven patients whose symptoms initially resolved after laparoscopic release ultimately required percutaneous transluminal angioplasty (PTA) with celiac artery stent placement for a recurrent symptomatic stenosis. Balloonexpandable bare metal stents were used in all cases at the time of the initial endovascular intervention. No patients were treated with angioplasty alone. Five (71%) remained symptom free at their most recent follow-up. Conversely, one patient underwent balloon expandable stent grafting 5 months after initial stenting, and a second underwent
repeat angioplasty of a stent 90 days after placement. Despite repeat percutaneous intervention and initial improvement, both patients had recurrent symptoms and ultimately underwent aortoceliac bypass using a 7-mm Dacron (DuPont, Wilmington, Del) graft (Table). Of note, 10 of 12 patients, with a coexisting psychiatric condition at the time of diagnosis experienced improvement in their respective symptoms with associated weight gain at the time of their most recent follow-up, whereas two patients in this subgroup reported persistent abdominal pain and concomitant weight loss. These patients ultimately underwent diagnostic angiography and were determined to have no residual underlying celiac arterial defect. Accordingly, these patients were subsequently referred to gastroenterology for further work-up and alternative management. DISCUSSION This study effectively demonstrates the successful diagnosis and treatment of MALS using a multidisciplinary approach predicated on astute diagnosis often relying on initial duplex ultrasound imaging coupled with an initial laparoscopic surgical approach. Perhaps more importantly, this study documents that most patients can anticipate a high likelihood for functional improvement with return to work or school, although a percentage of patients are likely to require a subsequent intervention. Interestingly, a significant percentage of MALS patients in this series also had an associated psychiatric diagnosis, a risk factor that is uncommonly reported in any recent literature.4,7,12,17 The implications of this observation remain unknown but may confound optimal patient selection or the effect of any placebo effect. Optimal patient selection for MALS treatment has remained historically elusive.7 This likely reflects the variation and evolution of diagnostic and therapeutic tools at the surgeon’s disposal over time, thus perpetuating variation in work-up and treatment. Moreover, as mentioned, prevalent coexisting psychiatric history has precluded any consistent patterns of presentation. Finally, patient selection has remained an additional challenge in the setting of prevalent asymptomatic anatomic celiac compression
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Fig 2. A, Intraoperative photograph shows the laparoscopic approach to the celiac axis. B, Laparoscopic division of median arcuate ligament (MAL) fibers. Note celiac stenosis and poststenotic dilatation (arrow).
findings demonstrated on widespread imaging studies obtained for alternative indications.18 As noted previously, variation also persists in the diagnostic work-up and treatment paradigms for patients with symptoms suggestive of MALS, which may have implications surrounding timely referral to a vascular specialist. Not infrequently, patients may have seen multiple providers before consulting a vascular surgeon, necessitating unnecessary tests before definitive management. Specifically, this testing may include a gamut of gastrointestinal studies, including endoscopy, gastrointestinal series, computed tomography, magnetic resonance, and even gastric exercise tonometry.1,2,9,10,19 As demonstrated in this study, several patients with a clinical picture and duplex ultrasound consistent with MALS did not require additional imaging or consultation, although others underwent additional imaging given the variability in presentation. The role of laparoscopy in treating MALS has increased over time, supplanting open surgical reconstruction in most circumstances as an initial approach. In our study, a laparoscopic approach was associated with low perioperative morbidity, short length of stay, and minimal blood loss, consistent with other reports.3-6,12-14,19 A minimally invasive surgical approach has obvious appeal in this relatively young, active patient population. Other management strategies have also been reported, however. Van Petersen et al9 demonstrated satisfactory results in a series of MALS patients managed with a retroperitoneal endoscopic MAL release, with 83% of operative patients experiencing symptom improvement. In addition, Relles et al10 reported a roboticassisted MAL release technique to be effective, albeit in a small cohort of patients, which has not been widely adopted. Accordingly, we have incorporated with success an initial laparoscopic approach to ligament release. In addition to excellent perioperative outcomes, this technique permits excellent, if not superior, visualization of the celiac origin and the MAL, thereby permitting safe and effective anatomic release (Fig 2). Interestingly, although several historical series reported an expected open conversion rate of 0% to 26%,3,4,6,8,12-14 no patients in this series required open surgery at their initial operation.
Despite successful laparoscopy, a significant number of patients can anticipate subsequent interventions. Not infrequently, a persistent anatomic compression of the celiac artery can persist despite a thorough dissection of the celiac artery and MAL release (Fig 3. A). Seven patients in this study demonstrated a persistent symptomatic celiac stenosis, ultimately prompting subsequent angiography and intervention in the setting of recurrent symptoms at variable lengths of follow-up. All of these patients were ultimately treated with percutaneous stent placement at a median interval of 48 days. The patient shown in Fig 3, B underwent successful PTA and stent placement with resolution of the anatomic defect and associated symptoms. Five of these patients remain improved on the most recent evaluation. Conversely, metachronous recurrent symptoms after a PTA intervention prompted consideration for mesenteric bypass, which was ultimately performed in two patients at a mean interval of 8 months after MAL release. Although mesenteric bypass is typically at the end of a thorough treatment algorithm for MALS patients, it is associated with excellent results in this highly selective subgroup, which was seen in our series and has been described in previous reports,4,12,13,15 with both patients in our cohort doing well at 4 years. On the basis of this experience, we have adopted a multidisciplinary diagnostic and therapeutic algorithm (Fig 4). Patients referred with symptoms compatible with MALS are evaluated with initial duplex ultrasound imaging in which provocative testing is performed during inhalation and exhalation. If positive duplex findings suggest the presence of a celiac stenosis in the context of symptoms consistent with MALS, the patient is considered for potential laparoscopic MAL release. Those presenting with an atypical constellation of symptoms or who demonstrate equivocal duplex findings undergo more definitive imaging with computed tomography angiography, although conventional angiography or magnetic resonance angiography remain acceptable alternatives. Patients without evidence of celiac stenosis or compression are subsequently referred to gastroenterology for further work-up for alternative diagnoses.
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Fig 3. A, Diagnostic angiography shows a persistent celiac artery defect (arrow) after laparoscopic median arcuate ligament (MAL) release in the setting of recurrent symptoms. B, Technically successful celiac artery stenting of the persistent defect.
Fig 4. Multidisciplinary clinical diagnostic and therapeutic algorithm. CT, Computed tomography; GI, gastrointestinal; MAL, median arcuate ligament; PTA, percutaneous transluminal angioplasty.
Patients treated with laparoscopic ligament release are subsequently followed up with a routine visit at 1 month. Patients who are asymptomatic at follow-up do not undergo routine longitudinal surveillance in the absence of symptoms. Repeat duplex imaging is reserved for patients who have persistent or recurrent symptoms. Patients with evidence of persistent or recurrent stenosis or those with ongoing symptoms are considered for conventional angiography. If angiographic evidence of a persistent celiac stenosis is identified in the setting of recurrent symptoms, patients may undergo percutaneous intervention at the discretion of the surgeon. In this series, seven patients ultimately required PTA with stent placement. Although the durability of endovascular mesenteric intervention has historically remained a just cause for concern, most of these series reported outcomes among a different patient population with atherosclerotic occlusive disease, thus potentially limiting their applicability to a MALS patient
population.4,6,12,13 Moreover, there did not appear to be any major adverse clinical sequelae among this cohort. However, we note that the experience of this patient subset remains small, with a paucity of long-term follow-up. Finally, in the setting of a failed percutaneous intervention and recalcitrant symptoms, patients should be ultimately considered for mesenteric bypass. This algorithmic multidisciplinary approach has demonstrated good clinical outcomes with low morbidity. As noted, most patients can anticipate immediate symptom improvement after laparoscopic release (18 of 21 [86%]), with a high likelihood (14 of 21 [67%]) of returning to work or school. Furthermore, this approach is associated with acceptable perioperative and 6-month freedom from symptoms and freedom from reintervention, although we emphasize that a subset of MALS patients will have recurrent symptoms necessitating subsequent intervention. Although previous series have reported a substantial open surgical conversion rate,3,4,6,12-14 this trend was not observed at our institution, which may partly reflect the ongoing evolution of laparoscopic technology and techniques. This study has several intrinsic limitations. First, the true prevalence of MALS seen at our institution remains unknown because of the retrospective nature of the study. This is often because many patients are never referred for surgical evaluation or in other cases because another etiology for abdominal pain is found and thus no diagnosis of MALS is assigned. Secondly, although a thorough preoperative evaluation is required, we cannot account for the nuances in assessment performed by all nonprocedural specialists involved. In addition, the small size of the study cohort limits our ability to derive broader more generalizable conclusions. Nevertheless, this study does depict contemporary results, which we believe are applicable in current practice. Moreover, prior reports had similar sample sizes given the
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relative rarity of the condition. We cannot make conclusions surrounding the durability of reinterventions in these patients given the small subset who required them. Longer follow-up among a larger cohort of MALS patients will be required to better address the durability of such interventions. In addition, the substantial prevalence of coexisting psychiatric illness documented in our cohort may confound accurate diagnosis, treatment, and follow-up. We are unable to comment on any quantifiable amount of weight gain after intervention. However, we would emphasize that symptom improvement, marked by abrogation of abdominal pain or associated qualitative weight gain, or both, is more reflective of treatment success rather than any specific quantity of gained weight. Finally, the effect of any placebo effect in this atypical patient cohort remains unknown. CONCLUSIONS Patients will often require multiple imaging studies to establish a diagnosis of MALS, although initial duplex ultrasound imaging appears to be a sensitive first option. Laparoscopic MAL release is the preferred contemporary surgical approach, providing superior visualization of the celiac axis. Most MALS patients can anticipate effective symptom relief postoperatively, although dedicated longitudinal surveillance is warranted because a subset of patients will likely experience recurrent symptoms warranting potential subsequent intervention. Although the effect of underlying psychiatric disease and the placebo effect remains unknown, it is difficult to overlook their potential confounding in managing this challenging subset of patients. Accordingly, a dedicated multidisciplinary MALS diagnostic and treatment algorithm appears to provide effective treatment in this often clinically desperate and debilitated patient population. AUTHOR CONTRIBUTIONS Conception and design: JC, DS Analysis and interpretation: JC, BN, DS Data collection: JC Writing the article: JC, DS Critical revision of the article: JC, TT, BN, PG, ER, RP, DW, DS Final approval of the article: JC, DS Statistical analysis: BN Obtained funding: Not applicable Overall responsibility: DS REFERENCES 1. Gruber H, Loizides A, Peer S, Gruber I. Ultrasound of the median arcuate ligament syndrome: a new approach to diagnosis. Med Ultrason 2012;14:5-9.
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2. Mensink PB, van Petersen AS, Kolkman JJ, Otte JA, Huisman AB, Geelkerken RH. Gastric exercise tonometry: the key investigation in patients with suspected celiac artery compression syndrome. J Vasc Surg 2006;44:277-81. 3. Jimenez JC, Harlander-Locke M, Dutson EP. Open and laparoscopic treatment of median arcuate ligament syndrome. J Vasc Surg 2012;56: 869-73. 4. Roseborough GS. Laparoscopic management of celiac artery compression syndrome. J Vasc Surg 2009;50:124-33. 5. Vaziri K, Hungness ES, Pearson EG, Soper NJ. Laparoscopic treatment of celiac artery compression syndrome: case series and review of current treatment modalities. J Gastrointest Surg 2009;13:293-8. 6. El-Hayek KM, Titus J, Bui A, Mastracci T, Kroh M. Laparoscopic median arcuate ligament release: are we improving symptoms? J Am Coll Surg 2013;216:272-9. 7. Williams S, Gillespie P, Little JM. Celiac axis compression syndrome: factors predicting a favorable outcome. Surgery 1985;98: 879-87. 8. Aschenbach R, Basche S, Vogl TJ. Compression of the celiac trunk caused by median arcuate ligament in children and adolescent subjects: evaluation with contrast-enhanced MR angiography and comparison with Doppler US evaluation. J Vasc Interv Radiol 2011;22: 556-61. 9. van Petersen AS, Vriens BH, Huisman AB, Kolkman JJ, Geelkerken RH. Retroperitoneal endoscopic release in the management of celiac artery compression syndrome. J Vasc Surg 2009;50: 140-7. 10. Relles D, Moudgill N, Rao A, Rosato F, DiMuzio P, Eisenberg J. Robotic-assisted median arcuate ligament release. J Vasc Surg 2012;56: 500-3. 11. Sultan S, Hynes N, Elsafty N, Tawfick W. Eight years experience in the management of median arcuate ligament syndrome by decompression, celiac ganglion sympathectomy, and selective revascularization. Vasc Endovasc Surg 2013;47:614-9. 12. Berard X, Cau J, Deglise S, Trombert D, Saint-Lebes B, Midy D, et al. Laparoscopic surgery for coeliac artery compression syndrome: current management and technical aspects. Eur J Vasc Endovasc Surg 2012;43: 38-42. 13. Baccari P, Civilini E, Dordoni L, Melissano G, Nicoletti R, Chiesa R. Celiac artery compression syndrome managed by laparoscopy. J Vasc Surg 2009;50:134-9. 14. Tulloch AW, Jimenez JC, Lawrence PF, Dutson EP, Moore WS, Rigberg DA, et al. Laparoscopic versus open celiac ganglionectomy in patients with median arcuate ligament syndrome. J Vasc Surg 2010;52: 1283-9. 15. Delis KT, Gloviczki P, Altuwaijri M, McKusick MA. Median arcuate ligament syndrome: open celiac artery reconstruction and ligament division after endovascular failure. J Vasc Surg 2007;46: 799-802. 16. Zwolak RM, Fillinger MF, Walsh DB, LaBombard FE, Musson A, Darling CE, et al. Mesenteric and celiac duplex scanning: a validation study. J Vasc Surg 1998;27:1078-87; discussion: 1088. 17. Reilly LM, Ammar AD, Stoney RJ, Ehrenfeld WK. Late results following operative repair for celiac artery compression syndrome. J Vasc Surg 1985;2:79-91. 18. Park CM, Chung JW, Kim HB, Shin SJ, Park JH. Celiac axis stenosis: incidence and etiologies in asymptomatic individuals. Korean J Radiol 2001;2:8-13. 19. Mak GZ, Speaker C, Anderson K, Stiles-Shields C, Lorenz J, Drossos T, et al. Median arcuate ligament syndrome in the pediatric population. J Pediatr Surg 2013;48:2261-70. Submitted Dec 3, 2014; accepted Jan 21, 2015.
