Laparoscopic management of the median arcuate ligament syndromeans_5966 265..268
Tam Nguyen,* Michael Neale,* Rodney Lane,* Vivienne Schiavone,† Jaswinder S. Samra† andThomas J. Hugh†*Royal North Shore Hospital, Department of Vascular Surgery, University of Sydney, St Leonards, New South Wales, Australia and†Royal North Shore Hospital, Upper Gastrointestinal Surgical Unit, University of Sydney, St Leonards, New South Wales, Australia
Key words
abdominal pain, laparoscopy, median arcuate ligament,upper GI, vascular surgery.
Correspondence
Dr Thomas J. Hugh, Upper GI Surgical Unit, RoyalNorth Shore Hospital, Building 52, St Leonards, NSW2065, Australia. Email: [email protected]
T. Nguyen MBBS; M. Neale MM (Syd), FRACS;R. Lane MD, FRACS; V. Schiavone RN; J. S. Samra
D Phil (Oxon), FRCS (Eng & Ed), FRACS; T. J. Hugh
MD, FRACS.
Accepted for publication 12 August 2011.
doi: 10.1111/j.1445-2197.2011.05966.x
Abstract
Background: The median arcuate ligament syndrome (MALS) is an infrequent causeof abdominal pain. This diagnosis is made after exclusion of other more commoncauses of upper abdominal symptoms. Mesenteric duplex and a computerized tomo-graphy mesenteric angiography demonstrate dynamic compression of the coeliac axisduring expiration.Methods: Retrospective analysis of presenting symptoms, preoperative findings andpostoperative outcomes.Results: Five consecutive patients who underwent laparoscopic division of themedian arcuate ligament over a 4-year period (2006–2010) are presented. This pro-cedure was associated with low morbidity and complete relief of symptoms in allpatients.Conclusion: A minimally invasive procedure is the treatment of choice in selectedpatients with MALS.
Introduction
The median arcuate ligament (MAL) is formed by muscular fibresand defines the anterior margin of the aortic hiatus.1 It is a fibrousarch that unites the diaphragmatic crura on either side of the aortichiatus.1 The median arcuate ligament syndrome (MALS) is an infre-quent phenomenon that causes compression of the coeliac axis.2 It isthought to be due to a low insertion of the diaphragmatic crus or bymalposition of the median arcuate ligament sometimes in associationwith hypertrophy of the diaphragmatic muscle fibres.2 Symptomsinclude vague epigastric or low retrosternal pain, especially post-prandially. Weight loss, nausea, vomiting, bloating, diarrhoea andreduced appetite also commonly occur.1 The diagnosis of MALS isone of exclusion as these same symptoms are seen in a variety ofdisorders.3
Traditionally, treatment of MALS has consisted of a laparotomyvia an upper abdominal incision, division of the MAL, and resectionof all associated peri-arterial neural tissue comprising the coeliacplexus or ganglion.4 Direct arterial reconstruction or coeliac arterybypass has also been performed in the past in order to restorepatency.4 With the advent of advanced laparoscopic instrumentation,a minimally invasive approach offers an attractive option for patientswishing to avoid a potentially morbid open procedure. Here wepresent a consecutive series of patients seen between 2006 and 2010
who underwent laparoscopic division of the median arcuate liga-ment for the MALS.
Methods
All patients in this series underwent an extensive preoperativeworkup in consultation with a vascular surgeon in order to confirmthe diagnosis and exclude other causes for their upper abdominalsymptoms. Upper and lower endoscopy, biliary ultrasound andgastric and biliary emptying studies were performed on all patientsto exclude other possible causes for their symptoms. Targeted inves-tigations included a dynamic duplex ultrasound and computerizedtomography (CT) mesenteric angiography done during inspirationand expiration. MALS was diagnosed only after demonstration ofdynamic compression of the coeliac axis by the median arcuateligament especially during expiration (Fig. 1a,b). Patients with fixedvascular abnormalities such as coeliac artery stenosis were notoffered laparoscopic treatment.
Operative details
Laparoscopic division of the median arcuate ligament was per-formed by a single surgeon (TJH). All operations were performedunder general anaesthesia with patients in the supine position. A
single dose of prophylactic, intraoperative antibiotic as well asregular postoperative thromboembolic-prophylaxis were provided.With the surgeon standing between the legs, a pneumoperitoneumwas established through a direct cut-down Hasson cannula located inthe mid-line approximately 2 cm above the umbilicus. Two further5-mm ports were placed just below the costal cartilage in the rightand left mid-clavicular lines, respectively. A Nathanson liver retrac-tor was passed through an additional 5-mm epigastric port in order toretract the left lateral liver. Dissection commenced by division of the
gastro-hepatic omentum using the LigaSure® (ValleyLab Inc,Boulder, CO, USA) instrument. The left crus was dissected with thehook diathermy in order to expose the anterior surface of theabdominal aorta. The dissection continued inferiorly on a planeimmediately anterior to the abdominal aorta until the left gastricvessels were identified. These structures were then slung with avessel loop and retracted laterally via the left 5-mm port. Thedissection was completed by dividing all tissues at the base of theleft gastric artery and overlying the origin of the coeliac axis.After the median arcuate ligament was identified it was dividedusing the LigaSure® (ValleyLab Inc) instrument but only after firstconfirming that all other vascular anatomy was intact. In all cases,division of this enlarged ligament led to a dramatic release of thecompressed coeliac axis (Fig. 2a,b). There was no intraoperativeblood loss and no drains were used in any case in this series. Thesupra-umbilical wound was closed with a 1 polydioxanone suturefollowed by a dissolvable subcuticular suture for all skin incisions.
(a)
(b)
Fig. 1. (a) CT mesenteric angiogram showing compression of the coeliacaxis during inspiration. (b) CT mesenteric angiogram showing dynamiccompression of the coeliac axis during expiration. CT, computerizedtomography.
(a)
(b)
Fig. 2. (a) Laparoscopic view of the median arcuate ligament compressingthe coeliac axis. (b) Laparoscopic view showing decompression of thecoeliac axis after dividing the median arcuate ligament.
Patients were allowed a diet as tolerated in the immediate post-operative period.
Results
The five patients in this series were all found to have a markedlyenlarged median arcuate ligament causing compression of thecoeliac axis. In all cases, the ligament was successfully dividedlaparoscopically with no patients requiring conversion to laparo-tomy. The perioperative details of all patients are summarized inTable 1. There were no intra-operative complications and the post-operative course was uneventful in three of the five patients. Twopatients had moderate nausea and abdominal discomfort during thefirst 48–72 h, but these symptoms resolved spontaneously prior todischarge. At the time of discharge, all patients were symptom-free.The median operative time was 120 min (60–210 min) and themedian hospital stay postoperatively was 4 days (3–4 days). Therewas no mortality in this series. At a median follow-up of 19 months(1–56 months) all patients had complete relief of their preoperativesymptoms. Furthermore, three patients have since resumed a vigor-ous exercise regime without any recurrence of their symptoms.
Discussion
MALS was first described in 1963 in a case report by Harjola et al.4,5
It is also referred to as coeliac artery compression syndrome orDunbar’s syndrome.4 The median arcuate ligament usually passessuperior to the origin of the coeliac axis.1 However, there is a variantin a subset of the population whereby the ligament inserts low andconsequently crosses the proximal portion of the coeliac axiscausing compression of the origin of the coeliac trunk.1 In 10–24%of people in this group, the compression leads to a significant reduc-tion in coeliac blood flow and causes clinical symptoms.1 This con-dition is most often seen in young patients and is more common infemale patients compared with male patients.1
The patients described in this series all presented with typicalsymptoms of the MALS. They underwent an extensive preoperativeworkup to exclude other more common causes for their symptoms.Typically, most of our patients had a relatively long duration ofsymptoms and had undergone multiple investigations elsewherebefore a diagnosis was finally made. Of interest, was the fact that allof our patients were female and all had been undertaking what wouldbe considered above average amounts of physical activity prior totheir presentation. This suggests that part of the explanation forcompression of the coeliac axis may relate to hypertrophy of themuscle fibres of the diaphragmatic crus. The median age in ourpatients was 23 years, which is much lower than in other series.4,6
Importantly, we did not offer laparoscopic division of the MAL topatients with fixed vascular wall deformities as demonstrated bypost-stenotic dilatation or collateral flow. This may explain theexcellent long-term results achieved in our patients.
MALS remains controversial in terms of the pathophysiologicorigin of the symptoms, diagnosis, and optimal treatment and man-agement.7 The traditional operative approach involves a midlinelaparotomy with division of the anomalous fibrous diaphragmaticbands overlying the coeliac artery, along with the coeliac plexus and T
lymphatic tissues.7 It is not clear whether symptoms attributed toMALS are due to a temporary ischaemic event as the coeliac trunkis compressed or whether it is due to compression of the neuralplexus around the coeliac axis. We did not perform an extensivedissection of the neural plexus around the coeliac trunk and the factthat all of our patients had good symptomatic relief suggests thatrelease of the compressed vessel is the key component.
There have been some case reports and several small series ofpatients who underwent laparoscopic division of the median arcuateligament.2,4,7,8 In the largest of these, Tulloch et al. published aretrospective review of outcomes in patients with the MALS andcompared laparoscopic versus open coeliac ganglionectomy over a10-year period. In this study, 10 patients were treated laparoscopi-cally and two of these required conversion to an open procedure.4
The median time to return to a normal diet and the average hospitalstay were significantly shorter in those treated laparoscopically com-pared with patients who had an open procedure.4 The median hos-pital stay of only 4 days and the associated minimal postoperativemorbidity is similar to outcomes reported by others.4,6 Two of ourpatients had moderate nausea and abdominal pain during the first48 h postoperatively but, fortunately, these symptoms resolvedspontaneously.
Laparoscopic management of the MALS in carefully selectedpatients results in similar long-term outcomes as with the openapproach, but, in comparison, leads to shorter inpatient hospital stay,earlier return to normal activities and better cosmetic result.
Conclusion
The diagnosis of MALS should be considered in at-risk patientswhen other more common causes for atypical upper abdominalsymptoms have been excluded. Duplex scanning combined with CT
or magnetic resonance mesenteric angiography are most helpful inconfirming the diagnosis. Patients should be offered a minimallyinvasive operation to divide the ligament and release compression ofthe coeliac axis after demonstration of dynamic compression of thecoeliac axis during respiration. This procedure can be performedsafely with minimal morbidity and early return to normal activities.At long-term follow-up, carefully selected patients usually havecomplete resolution of their symptoms and with no limitation ontheir physical activities.
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