Laparoscopic Treatment of Median Arcuate Ligament Syndrome: A Rare Cause of Chronic Severe Abdominal Pain

Abstract

Median arcuate ligament syndrome is a rare disorder characterized by chronic postprandial abdominal pain and weight loss caused by compression on celiac artery. A 17-year-old girl with chronic severe abdominal pain and weight loss was referred to our clinic. Other causes of chronic abdominal pain were investigated and excluded. The compression on celiac artery was detected on Doppler ultrasound and diagnosis was confirmed by computed tomography angiography. The patient underwent laparoscopic release of median arcuate ligament. There were no intraoperative complications; however, partial pain response was observed postoperatively that necessitated para-spinal ganglion blockage. The patient is symptom-free in 1-year follow-up period.

INTRODUCTION

Median arcuate ligament syndrome (MALS) or celiac artery compression syndrome is a rare disorder caused by compression of celiac artery by the median arcuate ligament. This syndrome was first described by Harjola in 1963.[1] Clinical symptoms include postprandial abdominal pain, nausea, vomiting, epigastric bruit and weight loss. Most of the patients with this syndrome are investigated with several diagnostic procedures and managed as functional gastrointestinal disorder (FGID) before a certain diagnosis is established. Doppler ultrasonography reveals the stenosis/compression of celiac artery with increased flow velocity and computed tomography (CT) angiography confirms the compression of celiac trunk and therefore the diagnosis of MALS. Most of the reports focus on adults in this clinical entity.[2,3] In recent years, MALS has begun to be recognized more frequently in pediatric/adolescent population.[4,5,6]

We report a case of a teenage female patient treated with laparoscopic release of the median arcuate ligament at our institution.

CASE REPORT

A 17-year-old girl with chronic postprandial abdominal pain and weight loss was referred to our clinic. The other causes of chronic abdominal pain were investigated by several studies including gastrointestinal endoscopies and imaging; however, no definitive cause could be identified. Doppler ultrasonography was able to reveal a compression on the celiac artery. Peak systolic velocity (PSV) in the celiac trunk was measured as 250 cm/s, and dropped to 170 cm/s with deep inspiration (ΔPSV = 80 cm/s). Diagnosis was confirmed by three-dimensional (3D) CT angiography confirming the narrowing of celiac artery at the beginning of the celiac trunk arising from abdominal aorta [Figure 1].

Figure 1.

The narrowing of celiac artery at the beginning of the celiac trunk was demonstrated on computed tomography angiography

The procedure was carried out via four 5 mm trocars. After opening the lesser omentum, crural fibers of diaphragm was dissected to expose median arcuate ligament. Celiac ganglion was cauterized and incised with hook cautery. Then, arcuate ligament was dissected above celiac artery and released with a sealing device (Ligasure; Covidien^®) [Figure 2]. After the releasing of the ligament, celiac trunk was free, and pulsation can be seen clearly at the distal branches of celiac artery. There were no intraoperative complications and the patient was discharged on the second postoperative day.

Figure 2.

Celiac artery was released by cutting median arcute ligament with a sealing device

In the postoperative period, patient was admitted to our clinic with recurrent symptoms of mild abdominal pain. The patient underwent postoperative Doppler ultrasonography. PSV in the celiac trunk was measured as 202 cm/s, and dropped to 162 cm/s with deep inspiration (ΔPSV = 40cm/s). Also, 3D CT angiography was performed to demonstrate the improvement in narrowing of celiac artery. However, surgical clips did not allow for demonstration of celiac artery properly. Although the pain was described to be of lower intensity when compared with presurgical state, it continued to be disturbing and required frequent use of analgesics. Therefore, the patient was referred to the pain management service in the department of anesthesiology for paraspinal ganglion blockage. The patient is free of symptoms in the 9-month follow-up, gained weight and resumed normal daily activity.

DISCUSSION

Since the first description of MALS in 1961, there are several debates in the diagnosis and treatment of this rare clinical entity. Most of the studies about this disorder are from adult literature.[2,3] It is being to be recognized more frequently in pediatric population in recent years.[4,5,6] Postprandial abdominal pain, vomiting, and weight loss are the typical clinical symptoms for this syndrome. However, these patients are frequently diagnosed and treated as FGID before the correct diagnosis is confirmed. It is speculated that compression on celiac artery may result in impairment of intestinal perfusion, especially following enteral intake, and results in intestinal angina. Other investigators, on the other hand, implicate that the course is a neurological pathology of the celiac ganglion, and consider this disease a primary neurological disorder.[7]

Several management strategies are instituted for the treatment of MALS including interventional angioplasty or stenting and surgical procedures such as releasing of the median arcuate ligament compressing the celiac artery or bypass surgeries.[8] The releasing of the ligament may be done by open, laparoscopic or robotic surgery.[9,10] Minimally invasive techniques have gained popularity in the surgical management of this condition limiting open surgery to more complicated celiac by-pass procedures which do not appear in the pediatric surgical literature. Minimally invasive techniques are very efficient in approaching to this surgical site and may be performed safely by an experienced surgeon. There are reports in the literature that complete resolution of symptoms may not be achieved in some patients as in our case. Some patients may require prolonged use of analgesics while some others may necessitate para-spinal celiac ganglion blockage as an additional measure to completely alleviate the symptoms.

CONCLUSION

MALS should be considered in the differential diagnosis of patients with chronic abdominal pain, especially in those that are triggered postprandially. The diagnosis of FGID virtually requires to be scrutinized if the symptoms are persistent, and should be compelling for the physician/surgeon to search for this perhaps more frequent but rarely recognized entity. Doppler ultrasonography revealing increased PSV on celiac artery and CT angiography demonstrating compression on the truncus are diagnostic. Laparoscopic release of the median arcuate ligament is safe and effective technique in the management of this disorder. Postoperative ganglion blockage may also be necessary to provide total relief of symptoms.

FINANCIAL SUPPORT AND SPONSORSHIP

Nil.

CONFLICTS OF INTEREST

There are no conflicts of interest.