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Atypical cause of episodic abdominal pain and unintentional weight loss

Published:March 21, 2021DOI:https://doi.org/10.1016/j.ejim.2021.03.012

      Keywords

      Case description

      A 23-year-old female with past medical history of generalized anxiety disorder and migraine presents to the clinic with unintentional weight loss of approximately 20 lbs and chronic episodic abdominal pain associated with nausea and vomiting for 6-8 weeks. Physical examination and initial blood workup were unremarkable.
      Dynamic CT angiogram of the abdomen and pelvis was performed, with image acquisition during inspiratory phase (Fig. 1A), and end-expiratory phase (Fig. 1B and 1C). 3D reconstruction image is depicted in Fig. 1D.
      Fig. 1:
      Fig. 1Dynamic CT angiogram of the abdomen and pelvis with images acquired during inspiratory (A), and end-expiratory phase (B and C). 3D reconstruction image during end-expiratory phase is illustrated in D. There is dynamic compression of proximal celiac artery (arrow) due to external compression from median arcuate ligament, most accentuated during end-expiratory phase, compatible with Median Arcuate Ligament Syndrome.
      What is the most likely etiology of patients’ symptoms?

      Diagnosis

      Median Arcuate Ligament Syndrome

      Discussion

      Dynamic CT angiogram of the abdomen and pelvis with image acquisition during inspiratory phase (Fig. 1A), and end-expiratory phase (Fig. 1B and 1C) that demonstrates dynamic compression of proximal celiac artery (arrow) due to external compression from median arcuate ligament. 3D reconstruction image during end-expiratory phase is illustrated in Fig. 1D. Patient was diagnosed with median arcuate ligament syndrome [
      • Gozzo C
      • Giambelluca D
      • Cannella R
      • et al.
      CT imaging findings of abdominopelvic vascular compression syndromes: what the radiologist needs to know.
      ]. She underwent robotic-assisted median arcuate ligament release with complete resolution of the symptoms [
      • Akici M
      • Cilekar M.
      Can superior mesenteric artery syndrome really be treated surgically?.
      ].
      Median arcuate ligament syndrome, also known as Dumbar or Harjola-Marable syndrome, is a rare condition with reported incidence of 2 per 100,000 population [
      • Gozzo C
      • Giambelluca D
      • Cannella R
      • et al.
      CT imaging findings of abdominopelvic vascular compression syndromes: what the radiologist needs to know.
      ,
      • Akici M
      • Cilekar M.
      Can superior mesenteric artery syndrome really be treated surgically?.
      ]. It presents with chronic positional abdominal pain from compression of celiac trunk. The degree of compression varies with respiration, most accentuated during end-expiration when the two structures move close together. The etiology of pain is hypothesized to be ischemic from impaired blood flow secondary to compression [
      • Bayat I
      • Wang J
      • Ho P
      • et al.
      Intravascular ultrasound-guided laparoscopic division of the median arcuate ligament.
      ].
      CT angiography can play a role in the diagnosis of this condition by demonstrating the characteristic focal narrowing of the celiac artery in patients presenting with the appropriate clinical symptoms [
      • Gozzo C
      • Giambelluca D
      • Cannella R
      • et al.
      CT imaging findings of abdominopelvic vascular compression syndromes: what the radiologist needs to know.
      ]. The surgical management of median arcuate ligament syndrome is controversial [
      • Akici M
      • Cilekar M.
      Can superior mesenteric artery syndrome really be treated surgically?.
      ]. Since, many patients have compression of celiac axis from low insertion of median arcuate ligament without any clinical symptoms, and are incidentally diagnosed on imaging [
      • Akici M
      • Cilekar M.
      Can superior mesenteric artery syndrome really be treated surgically?.
      ].

      Submission declaration and verification

      The authors have not published, posted, or submitted any related papers from this same study.

      Declaration of Competing Interest

      The authors have no conflicts of interest to disclose.

      References

        • Gozzo C
        • Giambelluca D
        • Cannella R
        • et al.
        CT imaging findings of abdominopelvic vascular compression syndromes: what the radiologist needs to know.
        Insights Imaging. 2020; 11: 48
        • Akici M
        • Cilekar M.
        Can superior mesenteric artery syndrome really be treated surgically?.
        Ann Ital Chir. 2020; 91: 35-40
        • Bayat I
        • Wang J
        • Ho P
        • et al.
        Intravascular ultrasound-guided laparoscopic division of the median arcuate ligament.
        J Vasc Surg Cases Innov Tech. 2020; 6: 147-151