Abstract
Celiac artery compression syndrome (CACS) is a disease caused by celiac artery compression by the median arcuate ligament (MAL), resulting in intestinal ischemic symptoms. However, a clear method for the invasive treatment of CACS has not yet been established because of limited treatment indications. In particular, only a few reports of endovascular therapy (EVT) using stents as the initial invasive treatment are available. Here, we report a case where EVT was performed using a stent in the celiac artery, resulting in good outcomes. A 59-year-old male patient presented to our hospital with postprandial abdominal pain and was diagnosed with MAL-induced CACS since the abdominal contrast computed tomography examination showed stenosis of a celiac artery origin. He was aware of the abdominal pain symptoms; therefore, we decided to treat CACS with EVT as an invasive treatment. A bare metal stent was placed in the celiac artery, whose lumen was well dilated using intravascular ultrasound. Consequently, he no longer felt abdominal pain and had good stent patency after 15 months. Minimally invasive EVT may be considered the first-line treatment for CACS.
Learning objective
The efficacy of endovascular therapy (EVT) using stents for the invasive treatment of celiac artery compression syndrome (CACS) resulting from the compression of the median arcuate ligament has not yet been established. Specifically, the efficacy of EVT using stents for CACS is unknown. We can safely perform EVT with stent placement using intravascular ultrasound for maintaining long-term patency. Therefore, minimally invasive EVT may be considered the first-line treatment for CACS.
Keywords: Celiac artery compression syndrome, Endovascular therapy for mesenteric artery, Stenting
Introduction
Celiac artery compression syndrome (CACS) is a rare disease, where the celiac artery is compressed by the median arcuate ligament (MAL), resulting in impaired blood flow and visceral ischemia. The incidence of symptomatic celiac artery stenosis has been reported to be only 3 %–7 % [1]. Although many patients are asymptomatic, invasive treatment is only indicated when symptoms are present. Celiac artery decompression is an invasive treatment method, and surgical decompression of the celiac axis is frequently performed [2]. In a review of laparoscopic surgical treatment, 95.9 % of patients had immediate postoperative symptom relief, proving to be effective; however, 9.9 % of patients had perioperative complications such as bleeding, and 9.1 % received endovascular therapy (EVT) using stents as additional treatment [3] for residual stenosis of the celiac artery.
The recent emergence of advanced technology, such as intravascular ultrasound (IVUS), has helped demonstrate the effectiveness of EVT for renal and superficial femoral artery region pathologies [4], [5]. Consequently, the indications for EVT in peripheral arterial disease have expanded. However, limited reports are available on the effectiveness of EVT in celiac artery stenosis. Here, we report a case where EVT with a stent was effective as an initial treatment for symptomatic CACS.
Case report
Patient characteristics
A 59-year-old male patient (height, 171 cm; weight, 69 kg) noted postprandial upper abdominal pain, ranging from once every month; however, the patient did not visit the hospital as it improved over time. Subsequently, he visited our emergency room after experiencing abdominal pain that made him faint. However, he had no gastrointestinal symptoms, such as nausea and diarrhea. Physical examination revealed body temperature, blood pressure, pulse rate, and peripheral oxygen saturation of 35.7 °C, 114/79 mm Hg, 86 bpm, and 96 % (in room air), respectively. Additionally, no rales or murmurs were noted on chest examination. Intestinal peristalsis was not enhanced or diminished, and no bruits were observed in the abdomen. Tenderness was noted in the upper abdomen rather than rebound tenderness. The patient had no cardiovascular risk factors, including no family history of cardiovascular disease, collagen disease, or vasculitis, and denied taking any medications or drugs. Serological findings at the visit were as follows: C-reactive protein, 4300 μg/L; white blood cell count, 8.4 × 109/L; creatinine, 81.3 μmol/L; and estimated glomerular filtration rate, 65.9 mL/min/1.73 m2. The abdominal X-ray examination was unremarkable. Abdominal contrast computed tomography (CT) showed stenosis at the celiac artery origin, dilation of the distal celiac artery diameter, and enlargement of the pancreatoduodenal arcade diameter (Fig. 1A–C). Esophagogastroduodenoscopy showed no lesions requiring medical treatment, while blood sampling and physical findings were negative for collagen disease and vasculitis. Ultimately, the patient was diagnosed with CACS due to compression by the MAL. We suggested the following three treatment options to the patient: surgical therapy, EVT using a stent, and follow-up. The patient complained of severe abdominal pain and requested invasive treatment. Therefore, we decided to perform EVT since he refused surgical treatment and strongly wished to perform EVT treatment because he wanted less invasive treatment with a shorter hospital stay.
Fig. 1.
Narrowed origin of the celiac artery (yellow arrow) due to external pressure. (A) Sagittal section of contrast-enhanced computed tomography, (B) a three-dimensional computed tomography, and (C) pancreatic duodenal artery arcade (yellow circle) formed by increased blood flow from the superior mesenteric artery to the pancreatic duodenal artery due to decreased blood flow in the celiac artery.
Procedural technique in endovascular therapy
We inserted a 6-Fr Glidesheath Slender (Terumo, Tokyo, Japan) into the patient's left brachial artery. Since the angiography revealed stenosis at the origin of the celiac artery (Fig. 2A), a 6Fr Mach 1™ multipurpose curve-style guiding catheter (length, 90 cm; Boston Scientific, Marlborough, MA, USA) was advanced up to the entrance of the celiac artery. The lesion was successfully passed through a 0.014 Vassallo support guidewire (length, 300 cm; Cordis, Santa Clara, CA, USA) with the Prominent Advance NEO microcatheter (length, 135 cm; Tokai Medical Products, Aichi, Japan) toward the splenic artery. We examined the lumen of the celiac artery with a 0.014 digital IVUS catheter (Visions PV; Philips, Tokyo, Japan) and found no intimal hyperplasia suggestive of arteriosclerotic lesions. The stenosis site was oval, and extravascular compression was observed. The blood vessel diameter, which is distal to the stenosis, and lesion length were approximately 8.0 mm and 7.5 mm, respectively (Fig. 2A, Online Fig. 1). We decided that pre-dilatation with a balloon was unnecessary and directly placed a 6.0–14-mm balloon-expandable Express Vascular SD stent (Boston Scientific) in the lumen of the celiac artery with a rated burst pressure (14 atm). After dilatation of the stent, using an 8.0–20-mm Sterling™ balloon dilatation catheter (Boston Scientific), the distal and proximal stents were extended with nominal (6 atm) and rated burst (12 atm) pressures, respectively. Using angiography, we confirmed that the celiac artery was dilated (Fig. 2B) and that the stent was sufficiently dilated and crimped to the blood vessel wall with no dissection or hematoma distal to the stent by IVUS (Fig. 2B, Online Fig. 2). Finally, the procedure was terminated after contrast enhancement confirmed no perforations on the peripheral artery.
Fig. 2.
Contrast-enhanced digital subtraction angiography and intravascular ultrasound images. (A) Contrast-enhanced digital subtraction angiography images showing stenosis of the celiac artery (red arrow), and intravascular ultrasound images showing stenotic celiac artery in an elliptical shape due to external compression. (B) Contrast-enhanced digital subtraction angiography images showing improved celiac artery stenosis (red arrow) after stent placement, and intravascular ultrasound images showing dilation of the stent in the celiac artery and good crimping of the stent to the vessel.
Follow-up
Abdominal vascular echocardiography on the day after surgery showed a peak systolic velocity (PSV) of 1.5 m/s in the distal celiac artery, implying no apparent accelerated blood flow. However, PSV of the distal celiac artery 1 month later showed a slight increase of 2.2 m/s, and 3 months later, it showed 3.3 m/s. Contrast CT after 3 months showed good expansion of the stent, a reduction in the distal celiac artery diameter, and a reduction in the pancreatoduodenal arcade (Fig. 3). This finding was suggestive of an improvement in the hemodynamics of the mesenteric artery. Furthermore, the patient received two antiplatelet agents after EVT, which included aspirin 100 mg and clopidogrel 75 mg; however, clopidogrel 75 mg was discontinued 3 months after EVT.
Fig. 3.
Contrast-enhanced computed tomography image 3 months after stent placement showing good stent dilation and decompression of the celiac artery origin (yellow arrow). (A) A sagittal section of contrast-enhanced computed tomography, (B) a three-dimensional computed tomography, and (C) the disappearance of pancreaticoduodenal artery arcade development (yellow circle), consistent with the improvement of the hemodynamics of the celiac artery.
Fifteen months post-stenting, the patient denied any recurrence of abdominal pain, and PSV of the distal celiac artery showed no further changes.
Discussion
This case demonstrated the effectiveness of EVT using stents as an initial therapeutic approach that is minimally invasive compared with surgical decompression for treating CACS.
CACS is more prevalent in women than in men (4:1 ratio), and the median age is typically between 30 and 50 years [6]. Invasive treatment is indicated only in symptomatic cases. The conventional method is surgical celiac artery decompression [7], which is a highly invasive procedure performed under general anesthesia. Treatment with EVT has also been attempted; however, a poor therapeutic effect has been reported since the celiac artery cannot be released with balloon angioplasty alone [6]. Recently, stent placement has become the mainstream treatment for EVT. Therefore, EVT using stents is effective in treating residual stenosis and in patients with recurrent symptoms after the initial surgical decompression of the celiac axis [7]. The long-term durability of stents with extravascular compression, termed myocardial bridging [8], has been demonstrated in the coronary arteries. Additionally, EVT for the common femoral artery stent implantation was avoided because of fear of stent fracture in the past and the risk of stent fracture caused by flexion of the hip joint. However, because of the recent advances in stent technology, the indication of EVT by stent placement is expanding as a revascularization procedure for the common femoral artery as a treatment method instead of surgical endarterectomy. Medium-term patency rates for stenting with EVT for common femoral artery are similar to those for surgical endarterectomy in self-expandable stents [9]; therefore, it is believed that the indications for stent placement at sites of external compression are becoming more widespread. The use of EVT for mesenteric artery disease has recently increased, and advances in devices such as stents have also improved long-term patency rates [10].
The use of IVUS has led to improved clinical outcomes in EVT and has made it possible to accurately determine the stent size for the vascular lumen and evaluate the vascular properties; this is expected to have high initial success and long-term patency rates for EVT. Because a higher primary patency rate using IVUS has been obtained for EVT in simple lesions in the femoral and popliteal artery regions [10], it can be deduced that EVT is a minimally invasive and highly safe treatment.
In this case, a good outcome was obtained only with initial EVT, and positive results on CT were obtained after 3 months, with symptom improvement after 15 months. However, EVT alone cannot release the external compression of the celiac artery by adjacent blood vessels. Stent fracture has been reported as a rare complication after stent placement for myocardial cross-linking and is considered a risk factor for stent restenosis [8]. Additionally, because neuropathic mechanisms are associated with CACS, it may be difficult to relate the element using a stent alone. However, in this case, a risk of stent fracture was observed in the chronic phase; therefore, long-term follow-up is required.
In conclusion, EVT using stents, which is less invasive than surgery, may be considered an effective initial invasive treatment for CACS. However, because CACS management has many uncertainties, further evidence should be accumulated for CACS treatment.
Patient consent statement
Written informed consent was obtained from the patient for publication of this case report, including accompanying image.
Conflicts of interest
The authors declare that there are no conflicts of interest.
Acknowledgments
The results of this case report were presented at the 59th Kantokoshinetsu Regional Conference of the Japanese Association of Cardiovascular Intervention and Therapeutics in May 2022.
Footnotes
Supplementary data to this article can be found online at https://doi.org/10.1016/j.jccase.2023.05.005.
Appendix A. Supplementary data
Supplementary figures
References
- 1.Park C.M., Chung J.W., Kim H.B., Shin S.J., Park J.H. Celiac axis stenosis: incidence and etiologies in asymptomatic individuals. Korean J Radiol. 2001;2:8–13. doi: 10.3348/kjr.2001.2.1.8. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Goodall R., Langridge B., Onida S., Ellis M., Lane T., Davies A.H. Median arcuate ligament syndrome. J Vasc Surg. 2020;71:2170–2176. doi: 10.1016/j.jvs.2019.11.012. [DOI] [PubMed] [Google Scholar]
- 3.Jimenez J.C., Harlander-Locke M., Dutson E.P. Open and laparoscopic treatment of median arcuate ligament syndrome. J Vasc Surg. 2012;56:869–873. doi: 10.1016/j.jvs.2012.04.057. [DOI] [PubMed] [Google Scholar]
- 4.Soga Y., Iida O., Hirano K., Yokoi H., Nanto S., Nobuyoshi M. Mid-term clinical outcome and predictors of vessel patency after femoropopliteal stenting with self-expandable nitinol stent. J Vasc Surg. 2010;52:608–615. doi: 10.1016/j.jvs.2010.03.050. [DOI] [PubMed] [Google Scholar]
- 5.Trinquart L., Mounier-Vehier C., Sapoval M., Gagnon N., Plouin P.F. Efficacy of revascularization for renal artery stenosis caused by fibromuscular dysplasia: a systematic review and meta-analysis. Hypertension. 2010;56:525–532. doi: 10.1161/HYPERTENSIONAHA.110.152918. [DOI] [PubMed] [Google Scholar]
- 6.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 Endovascular Surg. 2013;47:614–619. doi: 10.1177/1538574413500536. [DOI] [PubMed] [Google Scholar]
- 7.Brown D.J., Schermerhorn M.L., Powell R.J., Fillinger M.F., Rzucidlo E.M., Walsh D.B., Wyers M.C., Zwolak R.M., Cronenwett J.L. Mesenteric stenting for chronic mesenteric ischemia. J Vasc Surg. 2005;42:268–274. doi: 10.1016/j.jvs.2005.03.054. [DOI] [PubMed] [Google Scholar]
- 8.Ernst A., Bulum J., Šeparović Hanževački J., Lovrić Benčić M., Strozzi M. Five-year angiographic and clinical follow-up of patients with drug-eluting stent implantation for symptomatic myocardial bridging in absence of coronary atherosclerotic disease. J Invasive Cardiol. 2013;25:586–592. [PubMed] [Google Scholar]
- 9.Gouëffic Y., Della Schiava N., Thaveau F., Rosset E., Favre J.P., Salomon du Mont L., Alsac J.M., Hassen-Khodja R., Reix T., Allaire E., Ducasse E., Soler R., Guyomarc'h B., Nasr B. Stenting or surgery for de novo common femoral artery stenosis. JACC Cardiovasc Interv. 2017;10:1344–1354. doi: 10.1016/j.jcin.2017.03.046. [DOI] [PubMed] [Google Scholar]
- 10.Schermerhorn M.L., Giles K.A., Hamdan A.D., Wyers M.C., Pomposelli F.B. Mesenteric revascularization: management and outcomes in the United States, 1988–2006. J Vasc Surg. 2009;50:341–348.el. doi: 10.1016/j.jvs.2009.03.004. [DOI] [PMC free article] [PubMed] [Google Scholar]
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