Median arcuate ligamentum syndrome:Four case reports

lNTRODUCTlON

Median arcuate ligamentum syndrome(MALS)is a disease entity with unclear pathogenesis[1,2].If it is not considered in advance,the clinical diagnosis of the disease is very difficult because patients complain of digestive discomfort including pain,but this characteristic is not specific to MALS[3-6].Most of the studies conducted through a retrospective analysis of incidental celiac artery compression in asymptomatic patients have been imaging studies[7-10],but the data are difficult to apply in actual clinical practice.This is because accidental image discovery does not require treatment.In addition,the surgical cases experienced by many surgeons only concern surgical methods for diseases that have already been diagnosed through complex processes[11,12],and no studies have been conducted to help make a quick diagnosis in these patients.

Therefore,it is necessary to introduce MALS as a causal disease,which should be considered in patients complaining of digestive symptoms at the gastroenterology department.Here,we present four patients diagnosed with MALS.

CASE PRESENTATlON

Chief complaints

A 63-year-old male patient with no specific medical history visited our clinic complaining of epigastric pain with heartburn symptoms that started 2 years earlier.

A 64-year-old female patient,complaining of indigestion for 20 years had been treated for stressinduced gastritis,irritable bowel syndrome with constipation,and reflux esophagitis.

A 62-year-old female patient with an underlying alcohol-related disorder complained of indigestion 3 d earlier and took digestive medicines,but there was no improvement.

A 47-year-old female patient was admitted with severe abdominal pain that started several years ago.

History of present illness

The location of the pain was in the upper abdomen,and the pain lasted for 2 to 3 min.The patient mentioned that the pain intensity sometimes even woke him up and that it subsided when he walked and slightly improved when he bent over.Although he had been administered a proton pump inhibitor(PPI),motility-increasing agent,and mucosal protective agent at local clinics for several years,there was no improvement.Endoscopy and a computed tomography(CT)were already done at other hospitals.The CT readings at the other hospitals showed normal findings and malignancy was excluded.

The patients had a free previous medical history.

She underwent cholecystectomy at another hospital because of colicky pain,but she was still complaining of unbearable abdominal pain and came to our hospital.Endoscopy and a CT scan were performed again and normal findings were reported.

The final diagnosis of the presented four cases is median arcuate ligamentum syndrome.

She felt excruciating pain in the upper abdomen that lasted several minutes to hours and the pain was not related to eating or defecation.Extreme pain even woke her up at night.It occurred once or twice a year,and PPI and prokinetics administration had no effect.The pain intensity decreased when leaning forward or breathing in.

She was prescribed gabapentin 100 mg,meloxicam 7.5 mg,and famotidine 20 mg b.i.d,and her symptoms improved significantly.

History of past illness

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Physical examination

The patients’ abdomen was soft and had no tenderness.

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Laboratory examinations

Blood analyses were normal.

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Imaging examinations

We reviewed the medical history based on the characteristics of pain and the CT again,and a focal point of CT stenosis in the celiac axis was suspected(Figure 1A).Therefore,CT angiography(CTA)was performed.On the CTA,it was observed that the celiac trunk originating from the upper abdominal aorta was compressed by the diaphragmatic crus(Figure 1B).As a result,the origin of the celiac trunk showed focal tight narrowing,and along with clinical symptoms,the diagnosis could be made.

Considering the patient's clinical features,we thought of diseases that could be missed by imaging techniques.Celiac artery compression was confirmed by reviewing the imaging studies(Figure 2A-C).This time,the collateral vessels around the pancreas were reviewed for narrowing of the celiac artery.

CTA was performed based on clinical suspicion and celiac luminal narrowing was found due to focal calcification in the imaging tests(Figure 3A and B).

Neuropathic pain was relieved after the administration of gabapentin 100 mg,meloxicam 20 mg,and famotidine 20 mg b.i.d.

FlNAL DlAGNOSlS

She visited the emergency room for severe abdominal pain but returned home without any specific findings on the CT scan.An outpatient clinic history was done again.Her squeezing pain persisted for more than 10 min at one time and was sometimes accompanied by chills.

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TREATMENT

Case 1

The patient’s condition improved after the administration of amitriptyline 10 mg to treat visceral neurologic symptoms and Tegoprazan 50 mg.The patient showed an immediate symptom improvement response to amitriptyline before active analgesic administration.Thus,even before active treatment,through diagnosis alone,improvement was already demonstrated.During follow-up,if any pain remains after observation for more than 3 mo,a pain relief agent and an active agent for reducing neurotransmitters will be used.

Case 2

There were no specific findings on endoscopy and abdominal ultrasonography,so based on clinical symptoms,an abdominal CT was performed,and MALS was diagnosed(Figure 4A-C).

All case participants provided informed written consent for publish.

Case 3

Gabapentin 100 mg,meloxicam 7.5 mg b.i.d,and esomeprazole were prescribed.She was due for an outpatient visit in 3 mo.The patient was asked to visit our hospital again if the symptoms worsened.She has been living without recurrence for a month after being prescribed medications.

Case 4

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OUTCOME AND FOLLOW-UP

The symptoms of all 4 patients were much relieved with medical treatment,and they are being followed up on an outpatient clinics.

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DlSCUSSlON

In all four cases,the patients experienced abdominal pain.In three cases,the diagnosis was possible by the judgment of the clinician shortly after the onset of symptoms.However,in one case that lasted over 20 years,a clear diagnosis was not possible.Even after complaining of colicky pain and performing a cholecystectomy,the diagnosis was made only after symptoms persisted.In all four cases,the symptoms were relieved by neuromodulators.

There are two current common hypotheses of MALS,which have been defined through several studies.The first one is neurologic pain caused by the compression of the celiac plexus and the other one is defined by compression of the celiac artery[2,10].However,most hypotheses are based on images that were retrospectively analyzed and a defined protocol for making a diagnosis has not been developed.If this is due to ischemic arteriality,collateral vessels may develop,but the pain caused regardless of the produced collateral vessels.Importantly,ischemia cannot be reversible when the pain is maintained for a long time.

Therefore,the imaging diagnosis is only an auxiliary diagnosis.If the clinician does not suspect this diagnosis,the latter will happen very late.Most of the patients who came to the outpatient clinic of the department of gastroenterology at the tertiary hospital for indigestion and abdominal pain underwent an endoscopy and CT.However,it is not easy to identify rare diseases without providing clinical information from tens of thousands of still images.In addition,since the focus is on the exclusion of malignant tumors related to mortality pain is often considered a psychological disease despite its chronic nature,and is ignored.

A report in 2017 reported only one case[13].Other case reports that dealt with diagnosed cases relied on images and explained the pathological mechanisms for bowel ischemia,but they could not explain the absence of symptoms of intestinal damage due to ischemia[14].Surgical treatments,such as ganglionectomy along with arterial reconstruction,have been proven to be effective[11],but considering that there are no symptoms even when pressed,physical removal may not be the only solution.This case is very novel because no cases have reported the outcomes of medical treatment and it raises awareness about a diagnosis that should not be missed.This case was written to suggest that a gastroenterologist must make a differential diagnosis.And since the gastroenterologist judges all the clinical results together,the evaluation is performed after confirming these.The following algorithm illustrates this process(Figure 5).

A limitation of this study was that the number of cases studied was too small.However,given that numerous MALS diagnoses have been missed,it is necessary to review many patients in the future and establish the diagnostic flow,considering the possibility that the etiology itself may be wrong.Since considering only four cases is insignificant,it will be necessary to review patients experiencing abdominal pain who have numerous missed diagnoses in the future.A common diagnostic flow is also required.

CONCLUSlON

MALS is a rare disease and it is easy to miss because it is not malignant,but patients experience pain over a long period.For the accurate diagnosis of a patient complaining of abdominal pain,the diagnosis must be differentiated.In addition,as there are asymptomatic patients,patients who need treatment should be carefully selected,and improvement with medical treatment can be expected.Large-scale studies are also needed.

FOOTNOTES

Rhee PL did study concept and design;Kim JE did data acquisition,drafting of the manuscript and critical revision of the manuscript for important intellectual content;all authors did approval of final manuscript.

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No potential conflict of interest relevant to this article was reported.

The authors have read the CARE Checklist(2016),and the manuscript was prepared and revised according to the CARE Checklist(2016).

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South Korea

Ji Eun Kim 0000-0003-2149-7979;Poong Lyul Rhee 0000-0003-0495-5296.

Zhang H

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Zhang H