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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 19  |  Issue : 1  |  Page : 45-48

Dunbar (or median arcuate ligament) syndrome: A case series


Department of Radiology, Saveetha Medical College, Chennai, Tamil Nadu, India

Date of Submission09-Nov-2021
Date of Acceptance15-Jan-2022
Date of Web Publication07-Mar-2022

Correspondence Address:
Dr. M Meyyappan
Department of Radio-Diagnosis, Saveetha Medical College and Hospital, Saveetha Nagar, Thandalam, Chennai - 602 105, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcls.jcls_50_21

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  Abstract 


Dunbar syndrome (DS) (or median arcuate ligament [MAL] syndrome) is a rare entity of the vascular compression syndrome, where there is focal proximal coeliac axis compression by MAL. It results in an insufficient supply of blood to the respective organs of the gastrointestinal tract. Multidetector computed tomography is a very convenient, noninvasive modality in diagnosing this condition and helpful in distinguishing it from other conditions, such as atherosclerotic disease. DS can further be treated disorder surgically by relieving the compression and sometimes may need vascular reconstruction. We present five cases of the DS.

Keywords: Celiac artery compression, computed tomography, Dunbar syndrome, Harjola–Marable syndrome, median arcuate ligament


How to cite this article:
Sharma PK, Mathapati P, Meyyappan M, Vatsan K. Dunbar (or median arcuate ligament) syndrome: A case series. J Clin Sci 2022;19:45-8

How to cite this URL:
Sharma PK, Mathapati P, Meyyappan M, Vatsan K. Dunbar (or median arcuate ligament) syndrome: A case series. J Clin Sci [serial online] 2022 [cited 2022 May 28];19:45-8. Available from: https://www.jcsjournal.org/text.asp?2022/19/1/45/339151




  Introduction Top


Dunbar syndrome (DS), also called median arcuate ligament (MAL) syndrome/celiac artery compression syndrome/Harjola–Marable syndrome, is characterized by external compression of celiac artery root by the MAL [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d. Harjola first described DS in 1963.[1] The reported incidence of 2 per 100,000 is between 1.76% and 4%,[2] more commonly seen in young (20–40 years of age) and thin women. In 1965, Dunbar et al. reported this condition causing intestinal angina linked to the anatomic anomaly.[3]
Figure 1: Picture illustrating the sagittal views (a) The median arcuate ligament crosses anterosuperior to the aorta and origin of the celiac axis is typical anatomy, (b) the median arcuate ligament crosses the proximal portion of the celiac axis is variant anatomy causing an indentation, significant abnormality, (c) celiac artery axis orientation during inspiration and (d) celiac artery axis orientation during expiration

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  Case Presentation Top


Case Presentation 1

A 55-year-old male presented with painless hematuria and epigastric pain, lasting for 1 month. The clinicians suspected could be pyelonephritis. The ultrasonography and gastrointestinal endoscopy were inconclusive. Contrast-enhanced multidetector computed tomography (MDCT) abdomen showed an MAL of size 4 mm in diameter. It caused focal extrinsic compression of the anterior wall of the proximal celiac artery/trunk and poststenotic segment dilatation of “hooking configuration” [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d. With clinical–imaging findings, a diagnosis of DS was made. The patient underwent celiac ganglion destruction as the symptoms were only pain and then further was symptom-free.
Figure 2: Contrast-enhanced multidetector computed tomography of the abdomen of a 55-year-old male: (a) Axial section and (b) sagittal section showing a median arcuate ligament (short white arrow) causing focal extrinsic compression of anterior wall of the proximal celiac artery/trunk with the poststenotic segment dilatation (short black arrow), (c) three-dimensional volume-rendered (sagittal) and (d) maximum intensity projection (sagittal) images showing the stenotic and poststenotic segments of the proximal celiac artery/trunk of “hooking configuration” (long white arrow).

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Case Presentation 2

A 50-year-old male came to the hospital with complaints of postprandial epigastric pain, nausea, and vomiting lasting for several months; the provisional diagnosis was pancreatitis. The ultrasonography and gastrointestinal endoscopy findings were inconclusive. Contrast-enhanced MDCT abdomen showed an MAL of size 4 mm in diameter. It caused focal extrinsic compression of the anterior wall of the proximal celiac artery/trunk associated with poststenotic segment dilatation of “hooking configuration” [Figure 3]a,[Figure 3]b,[Figure 3]c,[Figure 3]d. With clinical–imaging findings, a diagnosis of DS was made, and the patient underwent open surgical division of MAL and celiac ganglion destruction; the patient successfully underwent surgery and then further was symptom-free.
Figure 3: A 50-year-old male presented with postprandial epigastric pain, nausea, and vomiting lasting for several months. Contrast-enhanced multidetector computed tomography abdomen: (a) Axial section and (b) sagittal section showing a median arcuate ligament (short white arrow) causing focal extrinsic compression of anterior wall of the proximal celiac artery/trunk with the poststenotic segment dilatation (short black arrow), (c) three-dimensional volume-rendered (sagittal) and (d) maximum intensity projection (sagittal) images showing the stenotic and poststenotic segments of the proximal celiac artery

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Case Presentation 3

A 68-year-old male came to the hospital with complaints of right hypochondriac pain, nausea, and vomiting lasting for several months. The clinicians provide a provisional diagnosis as cholelithiasis; ultrasonography and gastrointestinal endoscopy findings were inconclusive. Contrast-enhanced MDCT abdomen showed MAL merge of size 4 mm in diameter. It caused focal extrinsic compression of the anterior wall of the proximal celiac artery/trunk associated with poststenotic segment dilatation of “hooking configuration” [Figure 4]a,[Figure 4]b,[Figure 4]c,[Figure 4]d. With clinical–imaging findings, a diagnosis of DS was made. The treatment we recommended was open surgical division of MAL and celiac ganglion destruction as patient was having Space occupying lesion (SOL) Hepatocellular carcinoma(HCC); he underwent hepatectomy with celiac ganglion destruction.
Figure 4: Contrast-enhanced multidetector computed tomography of the abdomen of a 68-year-old male: (a) Axial section and (b) sagittal section showing a median arcuate ligament (short white arrow) causing focal extrinsic compression of anterior wall of the proximal celiac artery/trunk with the poststenotic segment dilatation (short black arrow), (c) three-dimensional volume-rendered (sagittal) and (d) maximum intensity projection (sagittal) image showing the stenotic and poststenotic segments of the proximal celiac artery/trunk of “hooking configuration” (long white arrow)

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Case Presentation 4

A 20-year-old male came to the hospital with complaints of postprandial epigastric pain, nausea, and vomiting lasting for several months; the provisional diagnosis was pancreatitis. The ultrasonography and gastrointestinal endoscopy findings were inconclusive. Contrast-enhanced MDCT abdomen showed MAL merge of size 4 mm in diameter. It caused focal extrinsic compression of the anterior wall of the proximal celiac artery/trunk associated with poststenotic segment dilatation of “hooking configuration” [Figure 5]a,[Figure 5]b,[Figure 5]c,[Figure 5]d. With clinical–imaging findings, a diagnosis of DS patient was advised surgery; however, our patient refused for surgery.
Figure 5: Contrast-enhanced multidetector computed tomography abdomen of a 20-year-old male: (a) Axial section and (b) sagittal section showing a median arcuate ligament (short white arrow) causing focal extrinsic compression of anterior wall of the proximal celiac artery/trunk with the poststenotic segment dilatation (short black arrow), (c) three-dimensional volume-rendered (sagittal) and (d) maximum intensity projection (sagittal) image showing the stenotic and poststenotic segments of the proximal celiac artery/trunk of “hooking configuration” (long white arrow)

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Case Presentation 5

A 33-year-old male came to the hospital with complaints of upper abdomen pain increased on expiration compared to inspiration and associated with vomiting lasting for 2 years; the clinicians suspected and gave provisional diagnosis as gastritis, The ultrasonography and gastrointestinal endoscopy findings were inconclusive. Contrast-enhanced MDCT abdomen showed MAL merge of size 4 mm in diameter. It caused focal extrinsic compression of the anterior wall of the proximal celiac artery/trunk associated with poststenotic segment dilatation of “hooking configuration” [Figure 6]a,[Figure 6]b,[Figure 6]c,[Figure 6]d. With clinical–imaging findings, a diagnosis of DS was made. The patient was advised surgery; however, our patient refused for surgery.
Figure 6: A 33-year-old male. Contrast-enhanced multidetector computed tomography abdomen: (a) Axial section and (b) sagittal section showing a median arcuate ligament (short white arrow) causing focal extrinsic compression of anterior wall of the proximal celiac artery/trunk with the poststenotic segment dilatation (short black arrow), (c) three-dimensional volume-rendered (sagittal) and (d) maximum intensity projection (sagittal) image showing the stenotic and poststenotic segments of the proximal celiac artery/trunk of “hooking configuration” (long white arrow)

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  Discussion Top


The MAL connects the diaphragmatic crura, which usually courses superior to the origin of the celiac trunk. Two anatomic conditions can predispose to DS: the higher origin of the celiac artery or the lower insertion of the MAL.[4] The anatomical variants are present in about 10%–24% of the general population. Celiac axis compression leads to intimal hyperplasia. It also leads to the proliferation of elastic fibers in the media and adventitia disorganization. The classic clinical symptoms include postprandial epigastric pain, vomiting, and weight loss. On physical examination, epigastric bruit which varies with respiration was observed.[3] During inspiration, the coeliac artery migrates caudally in the abdominal cavity, attaining a vertical orientation, and thereby compression gets relieved, and during expiration, the coeliac artery gets compressed and narrowed.[2]

MDCT abdominal angiography with multiplanar reconstruction, maximum intensity projection, and three-dimensional volume rendering plays a significant role in establishing the diagnosis. MAL thickness of >4 mm is considered abnormal.[5] DS is diagnosed with MDCT abdominal angiography, which demonstrates a characteristic focal narrowing of the proximal celiac axis with a typical “hooked” configuration caused by the inferior displacement of the celiac artery by the MAL on sagittal views.[6] Poststenotic dilatation due to proximal compression of celiac artery. Sugae et al.[7] proposed a grading system of celiac artery stenosis caused by MAL compression based on stenosis grade, length of a stenosis, distance from the abdominal aorta, and presence of small collateral vessels found on CT angiography.

Celiac artery stenosis is relevant with pancreaticoduodenectomy, a risk factor for upper abdominal organs infarction. The most concerning consequence of DS is the visceral artery aneurysm formation. Severe celiac artery stenosis leads to altered hemodynamic in the pancreaticoduodenal arcades, causing intimal damage. The medial layer dysfunction with the formation of pancreaticoduodenal arteries true aneurysms with a mortality rate of 50%.[8] Few of the differentials include anomalous coeliac–mesenteric artery junction and abnormally fibrosed coeliac ganglia.

The treatment options for DS include surgical transection of the MAL to restore normal blood flow in the celiac artery and symptom relief.[9] Others include aortoceliac bypass and reimplantation of the celiac artery required in some patients.[10]


  Conclusion Top


Coeliac artery compression by MAL is an important anatomical variant. DS is diagnosed noninvasively on high-resolution MDCT abdominal aortography with multiplanar reformated, maximum intensity projection volume-rendered images.

Acknowledgment

The authors acknowledge the patient as a great source of learning.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form. the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Harjola PT. A rare obstruction of the coeliac artery. Report of a case. Ann Chir Gynaecol Fenn 1963;52:547-50.  Back to cited text no. 1
    
2.
Göya C, Hamidi C, Hattapoğlu S, Çetinçakmak MG, Teke M, Kuday S. Diagnosis of median arcuate ligament syndrome on multidetector computed tomography. J Med Cases 2013;4:616-9.  Back to cited text no. 2
    
3.
Dunbar JD, Molnar W, Beman FF, Marable SA. Compression of the celiac trunk and abdominal angina. Am J Roentgenol Radium Ther Nucl Med 1965;95:731-44.  Back to cited text no. 3
    
4.
Kim EN, Lamb K, Relles D, Moudgill N, DiMuzio PJ, Eisenberg JA. Median Arcuate Ligament Syndrome – Review of this rare disease. JAMA Surg 2016;151:471-7.  Back to cited text no. 4
    
5.
Eliahou R, Sosna J, Bloom AI. Between a rock and a hard place: Clinical and imaging features of vascular compression syndromes. RadioGraphics 2012;31:E33-49.  Back to cited text no. 5
    
6.
Horton KM, Talamini MA, Fishman EK. Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics 2005;25:1177-82.  Back to cited text no. 6
    
7.
Sugae T, Fujii T, Kodera Y, Kanzaki A, Yamamura K, Yamada S, et al. Classification of the celiac axis stenosis owing to median arcuate ligament compression, based on severity of the stenosis with subsequent proposals for management during pancreatoduodenectomy. Surgery 2012;151:543-9.  Back to cited text no. 7
    
8.
Nasr LA, Faraj WG, Al-Kutoubi A, Hamady M, Khalifeh M, Hallal A, et al. Median Arcuate Ligament Syndrome: A single-center experience with 23 patients. Cardiovasc Intervent Radiol 2017;40:664-70.  Back to cited text no. 8
    
9.
Jimenez JC, Harlander-Locke M, Dutson EP. Open and laparoscopic treatment of median arcuate ligament syndrome. J Vasc Surg 2012;56:869-73.  Back to cited text no. 9
    
10.
Hongsakul K, Rookkapan S, Sungsiri J, Tubtawee T. A severe case of median arcuate ligament syndrome with successful angioplasty and stenting. Case Rep Vasc Med 2012;2012:129870.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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