Introduction

Median arcuate ligament syndrome (MALS) is a rare (2/100,000) entity characterized by severe, debilitating, postprandial epigastric pain associated with a constellation of other vague symptoms including nausea, vomiting, occasional diarrhea, and abdominal bruit. and/or weight loss, and even leads to an abnormal aversion to food, “sitophobia” [1,2]. The classic triad of abdominal pain related to meals, weight loss, and abdominal bruit may not always present. Typically, the diagnosis of MALS is one of exclusion, only entertained after more common conditions have been ruled out. MALS has traditionally been thought to be attributed to compression of celiac trunk, also called celiac artery compression syndrome (CACS), resulting in intestinal angina [3,4]. Therefore, surgery is often used in the treatment of symptomatic MALS. Median arcuate ligament (MAL) release with resection of surrounding neural and lymphatic tissue to restore celiac arterial flow is the most common procedure performed [2-4]. The other theory of MALS is neuropathic, the result of median arcuate ligament impingement on the celiac plexus instead of the artery [5]. Celiac plexus block (CPB) is a radiological intervention for the treatment of visceral pain in the upper abdominal organs. It also can be used as a diagnostic tool to determine whether flank, retroperitoneal, or upper abdominal pain is sympathetically mediated via the celiac plexus or to improve prognostic accuracy before performing celiac plexus neurolysis [6]. This report is to share our experience of a successful treatment of MALS by percutaneous neurolysis with bilateral CPB, instead of surgery for MAL release.

Case presentation

This case report was approved by the Institutional Review Board of Taipei Veterans General hospital (IRB-TPEVGH No.: 2023-08-009BC). Informed consent has been obtained from the patient for the publication of this report. A 63-year-old female presented with a 6-month history of severe chronic epigastric dull pain, which typically worsened after meal and could be alleviated by adopting a knee-to-chest position. This bothersome symptom significantly affected her daily life activities, resulting in a weight loss of 6 kg within a span of 4 months. Computed tomography-angiogram (CTA) revealed the classic “j-hook” sign, anterior indentation/compression of celiac trunk (Figure 1a). Angiography demonstrated a 2.1 cm aneurysm originating from the dilated inferior pancreaticoduodenal artery (IPDA), with retrograde contrast medium filling of the celiac branches through the dilated IPDA (Figure 1b). To prevent rupture of the IPDA aneurysm, total obliteration of the aneurysm was achieved by utilizing Nester® and Tornado® Embolization Coils (Cook Group Incorporated, Bloomington, Indiana, USA). Subsequently, a GORE® VIABAHN® Endoprosthesis stent (W. L. Gore & Associates, Inc. Flagstaff, AZ) was introduced and deployed across the aneurysm neck to seal aneurysm neck and maintain collateral flow patency to the hepatic arteries from the IPDA (Figure 1c). Neurolysis with bilateral CPB at the level of celiac trunk was performed by introducing a 22-guage Chiba needle to the left and right celiac ganglia, which were subsequently blocked using a mixture of 4 c.c. ethanol + 4 c.c. 2% Xylocaine + 2 c.c. contrast medium (a total of 10 c.c. mixture) respectively under CT-guidance (Figure 2a-d).

Figure 1: (a) Anterior indentation, the classic “j-hook” sign, of celiac artery on computerized tomography-angiogram (CTA), leading to the development of median arcuate ligament syndrome (MALS); (b) Presence of a 2.1 cm aneurysm (indicated by the white arrow) originating from the dilated inferior pancreaticoduodenal artery (IPDA), accompanied by retrograde contrast medium filling of the celiac branches through the dilated IPDA, resulted from the stenosis of celiac trunk; (c) Successful total obliteration (indicated by the white arrow) of the aneurysm using Nester® and Tornado® Embolization Coils, followed by a GORE® VIABAHN® Endoprosthesis stent across the aneurysm neck to seal it and maintain collateral flow patency to the hepatic arteries from the IPDA.