Interprofessional Management of Median Arcuate Ligament Syndrome (Dunbar Syndrome) Related to Lumbar Lordosis and Hip Dysplasia: A Patient's Perspective.

We present a 53-year-old female patient with median arcuate ligament syndrome (MALS), also known as Dunbar syndrome or celiac artery compression syndrome, related to lumbar lordosis and hip dysplasia. She utilized interprofessional management strategies, which were beneficial in reducing lumbar lordosis and MALS-related symptoms. This finding is important because there are no other reports in the literature describing interprofessional strategies to manage symptoms for patients who are waiting for surgery or are not candidates for surgery. LEARNING POINTS: Excessive lumbar lordosis is related to the development of median arcuate ligament syndrome (MALS) due to the greater distance the median arcuate ligament stretches around the vertebral curves, causing compression of the celiac nerves and artery.It is important to consider the effects MALS has on multiple body systems when diagnosing and developing symptom management strategies.Referrals to interprofessional team members can help the patient manage the vast array of symptoms related to MALS. © EFIM 2020.

CASE DESCRIPTION

At age 51, the patient (the first author) consulted an internal medicine physician for multiple concerns: upper abdominal pain with eating, early satiation, 20-pound weight loss, epigastric pain, and upper body/head swelling, especially in her left temporal area, causing headaches. She had additional symptoms associated with multiple body systems including: dyspnoea, heart palpitations, episodes of dizziness, blood pressure dysregulation, tachycardia episodes and muscular pain. Her medical history indicated the patient was healthy overall but had had hip dysplasia and lumbar lordosis her entire life. At age 50, she contracted a campylobacter infection with long-term systemic inflammation, muscle and joint pain, upper body/head swelling and headaches. Magnetic resonance imaging showed L5-S1 disc degeneration and posterior bulge, an enlarged psoas bursa, bursitis of both hips, and osteoarthritis in both hips. Within a year after campylobacter infection, pain with eating, early satiation and epigastric pain became acute.

Methods and Procedures

Initial tests ordered by the internal medicine physician included: respiratory tests, nerve conduction studies of the lower extremities, blood analyses, and mesenteric duplex ultrasound. None of the tests suggested significant findings except the ultrasound, which showed elevated celiac artery velocities (334 cm/s at inspiration and 513 cm/s at expiration). Computed tomography angiography (CTA) with contrast dye confirmed severe compression of the proximal celiac artery.

The patient was referred to vascular medicine and gastrointestinal physicians and a surgeon for further evaluation, which included: a gastric emptying test; ultrasounds of the head and neck; computed tomography of the chest, abdomen with the pelvis, and neck; blood analysis; and endocrine tests. Physicians confirmed the MALS diagnosis, but they were not convinced that MALS was causing all the symptoms due to the lack of descriptive literature on MALS, so they continued ordering tests for 3 more months to rule out other conditions.

The total duration from onset of acute MALS-related symptoms following campylobacter infection to the diagnosis of MALS was 1 year, during which time the patient struggled to manage the symptoms that were causing significant distress and dysfunction.

She worked with a physical therapist (PT) to address symptoms associated with multiple system involvement in MALS and for sacroiliac dysfunction related to excessive lumbar lordosis, rigid psoas, L5-S1 disc degeneration, and hip dysplasia/arthritis. Evaluation found restriction and pain in the iliopsoas complex and diaphragm bilaterally. The lower rib cage was hypomobile with inhalation/exhalation and passive accessory movement testing.

The most beneficial PT strategies to reduce MALS-related symptoms included: instrument-assisted soft tissue mobilization; and strengthening and stretching to reduce lordosis (Fig. 1); manual lymphatic drainage; and primal reflex release techniques, such as the diaphragm lift, to mobilize the diaphragm[. The diaphragm lift resulted in immediate and long-term (2 weeks) reduction in dyspnoea and pain with eating.

Figure 1

Strategies to reduce lumbar lordosis

As an occupational therapist, the patient integrated the PT home program into her activities of daily living by targeting habits and routines. For example, she did lymphoedema mobilization during hygiene (washing and drying) to move oedematous fluids toward lymphatic vessels and the heart.

During a time period when her diaphragm was most rigid, the patient experienced three episodes of rib subluxation. She went to a chiropractor who was able to reposition the rib(s), resulting in immediate pain reduction.

The patient then had laparoscopic surgery for median arcuate ligament (MAL) release with celiac plexus resection, which was carried out 15 months after the onset of acute symptoms and 38 years after the original onset of epigastric pain. The surgeon excised the ligament, resulting in visible improvement in the calibre of the celiac artery to a normal appearance. He noted significant inflammation in the MAL area.

Aside from the surgical pain, nausea and fatigue, the patient reported complete relief of all MALS-related pain and associated symptoms. She also lost 10 pounds of fluid within the first few days of surgery (Figs. 2 and 3).

Figure 2

Lymphoedema before surgery

Figure 3

Lymphoedema resolution after surgery

A year after MAL release surgery, the patient had increased hip arthritis, rigidity of hip flexors, and increased lumbar lordosis with a return of many of the original MALS-related symptoms but at lower levels of intensity. A repeated mesenteric ultrasound indicated celiac artery velocity to be elevated during expiration (228 cm/s) and normal during inspiration (102 cm/s), suggesting MALS. The patient then saw an orthopaedic surgeon and elected to have a total hip arthroplasty (THA).

Upon recovery from THA the patient was able to resume the stretching and toning exercises to reduce lumbar lordosis, which simultaneously resulted in complete resolution of all MALS-related symptoms. Table 1 provides complete descriptions of symptoms, management strategies and outcomes.

Table 1

Interprofessional management of median arcuate ligament syndrome (MALS)-related symptoms and lumbar lordosis and hip dysplasia

Profession	Age	Signs and symptoms	Evaluations and interventions	Outcomes
Internal medicine	14–53	Intermittent episodes of epigastric pain that was relieved with side lying with hip flexion	Diagnosed with ‘acid stomach’	No relief; patient continued side lying as compensatory strategy
Chiropractic medicine	48–52	SI dysfunction; L5-S1 disc degeneration; lumbar lordosis	4 years of repeated visits for manual adjustments to reduce SI subluxation, decompression L5-S1, and physical agent modalities	Temporary relief after each treatment but pain and symptoms returned
Family medicine	47	Heart palpitations and dyspnoea	Reviewed medical history and concluded she was healthy	No referrals, diagnosis or interventions
Internal medicine	50–51	Campylobacter infection followed by systemic inflammation, pain, dyspnoeaPain with eating, early satiation, 20-pound weight loss, epigastric pain, and upper body/head swelling, headaches; dyspnoea; heart palpitations; episodes of dizziness; blood pressure dysregulation; tachycardia episodes (MALS-related symptoms)	Evaluations: respiratory tests for asthma and sleep apnoea, nerve conduction studies of lower extremities, blood analysesUltrasound of right upper quadrant to assess for gallstones. Patient asked technician to check arteries and he obligedMesenteric duplex ultrasound and CTA with contrast dye	Normal results except positive for mild asthmaNegative for gallstones with ‘incidental’ finding of elevated blood flow velocity (329 cm/s) in celiac artery 334 cm/s at inspiration and 513 cm/s at expiration in the celiac artery and 142 cm/s in the inferior mesenteric artery; CTA confirmed MALS
Pain management	51	Musculoskeletal pain in hips and lower back	Evaluation: history and physicalIntervention: gabapentin	Patient tried gabapentin for 2 weeks but experienced adverse side effects affecting cognition so she stopped taking it
Physical therapy	50–53	Musculoskeletal: SI dysfunction, hip dysplasia and arthritis, excessive lumbar lordosisRespiratory: difficulty breathing due to diaphragm rigidity, epigastric painLymphatic: upper body oedema, with head and facial swelling the most distressing	Strengthen gluteal musculature, rectus abdominus, transverse abdominus, pelvic floor, lower trapezius, rhomboids and latissimus dorsi. Stretch psoas; instrument-assisted soft tissue mobilization [6]Primal reflex release: diaphragm lift technique [1]Manual lymphatic drainage, lymphoedema strategies	Temporary relief after each treatment; however, hip arthritis continued to worsen until hip replacement. After hip replacement, able to do exercises and recurring MALS-related symptoms resolved with reduction of lumbar lordosisImmediately improved mobility of diaphragm during inhalation. Also, mild relief of epigastric pain with eating, lasting 2 weeksImmediate temporary reduction in oedema but needed to be done twice a day for ongoing relief
Occupational therapy	50–53	Musculoskeletal: SI dysfunction, hip dysplasia and arthritis, excessive lumbar lordosisRespiratory: difficulty breathing due to diaphragm rigidityLymphatic: upper body edema, with head and facial swelling as most distressingPain with eating	Integrate exercises and stretches into normal routinesIntegrate PT strategies into swimming habits to decrease lumbar lordosis and thoracic kyphosis (forward reach in scaption plane to recruit lower scapular stabilizers)Complete diaphragm lift technique during swimming and prior to eating. Integrate breathing techniques into sleep hygiene (inhale for count of 4, hold for 6, exhale for 8) to stretch diaphragm and decrease compressionIntegrate manual oedema mobilization into hygiene habits such as washing and drying motions to move lymphatic fluid toward the heartEat in partial side lying/reclining position with C-curve vertebral body position (position of least compression on celiac artery)	Short-term minimal relief of pain related to straighter vertebral alignmentImmediate expansion of diaphragm upon inhalation. Easier to hold breath for longer time, which also relieved compression on celiac nerves and artery to promote healing of damaged tissueModerate decrease in oedema with the increase in consistencyMild decrease in pain with eating
Homeopathic medicine	51–53	Upper body oedemaPain with eating, especially proteins and fats	Evaluation: history and physical; endocrine test; stool analysisIntervention: Recommended Epsom salt baths and HCl acid/enzyme supplements with meals (especially with meat)	Absent protein break-down by-products, suggestive of hypochlorhydria; absent oestradiolEpsom baths reduced oedema minimally. HCl/enzymes eliminated pain/bloating after eating meat. Reduced muscle pain. Hair became thicker
Vascular, gastroenterology, endocrinology and medicine	51	Referred to large hospital organization for further assessment and treatment of MALS	Mesenteric duplex ultrasound, gastric emptying test; ultrasounds of head and neck; computed tomography of the chest, abdomen with pelvis, and neck; blood analysis; and endocrine tests	Confirmed MALS diagnosis. Ruled out other possible pathologies. Providers not convinced that MALS was causing the wide array of all symptoms
Chiropractic Medicine	51	Rib subluxation from diaphragm rigidity	Manual mobilization to replace rib; physical agent modalities	Pain level immediately dropped from 9/10 to 3/10
Vascular Surgery Medicine	51	MALS-related symptoms	Laparoscopic surgery for median arcuate ligament release with celiac plexus resection	Immediate relief of all MALS-related symptoms with exception of nausea, which lasted 2 months post-surgery.Surgeon saw significant inflammation in the MAL area which was irrigated during surgery.Lost 10lbs of fluid within a few days after surgery.Relief of MALS symptoms lasted 1 year; then return of symptoms, but at mild levels of intensity. Returning symptoms co-occurred with exacerbation of lumbar lordosis due to tightening hip flexors from hip inflammation/arthritis
Orthopedic Surgery Medicine	50–53	Hip dysplasia/arthritis, SI dysfunction/pain, lumbar lordosis, L5-S1 pain.Return of mild MALS-related symptoms (pain with eating, epigastric pain, upper body/head swelling, blood pressure dysregulation)	Evaluation: Provocative testing, Xrays, & MRIsIntervention: total hip arthroplasty surgery	Surgery resulted in resolution of hip arthritis and SI pain.3 months post-surgery, pt able to do exercises and stretches to decrease lumbar lordosis, which also resulted in resolution of MALS-related symptoms to date of this publication. Ongoing need to complete exercises to prevent return of symptoms.

The table is organized by age of onset. ‘MALS-related symptoms’ include: upper abdominal pain with eating; early satiation; weight loss; epigastric pain; upper body/head swelling, especially in the left temporal area, causing headaches; dyspnoea; heart palpitations; episodes of dizziness; blood pressure dysregulation; tachycardia episodes; and muscular pain.

CTA, computed tomography angiography; MALS, median arcuate ligament syndrome; PT, physical therapy; SI, sacroiliac**

DISCUSSION

For this patient, there was a musculoskeletal connection between lumbar lordosis, hip dysplasia (and subsequent arthritis), and MALS as evidenced by parallel exacerbation and reduction of symptoms associated with each diagnosis. It is plausible that the congenital hip dysplasia irritated and shortened hip flexors, which led to lumbar lordosis with increased stretching and tightening of the MAL above the L1 vertebral attachment, putting pressure on the celiac nerves and artery. The inflammation from hip arthritis and secondary effects of campylobacter infection exacerbated the MALS-related symptoms. This offers a clue for more research into interprofessional management strategies for MALS, which includes strategies to reduce lumbar lordosis.

Other body systems are also affected by MALS as suggested by the wide array of symptoms in this patient, as follows:

Nervous and cardiac: orthostatic hypotension and tachycardia from celiac nerve impingement/excitation [

Gastrointestinal: decreased hydrochloric acid (HCl) and pancreatic enzymes [

Lymphatic: lymphoedema and inflammation [

Respiratory and vascular: dyspnoea; expiration causing increased compression of the celiac artery and blood flow velocities; organ pain with eating [.

The fact that all symptoms were relieved after MAL release surgery affirms the assertion that MALS is a multisystem problem; hence, the MAL is the epicentre of dysfunction in multiple body systems that are affected by MALS. This warrants consultation with many healthcare providers to develop management strategies for MALS-related symptoms.

This is the only article that describes the extent of MALS symptoms and management strategies using the actual voice of the patient, which enhances authenticity. To manage bias, a panel of experts and direct healthcare providers of this patient contributed to case review and development of this article.

In conclusion, it is important to recognize the relationship between MALS, lumbar lordosis and hip dysplasia. In addition, MALS should be viewed as a multisystem problem that may respond to a variety of interprofessional management strategies provided to patients who are struggling to manage MALS-related symptoms for extended periods of time.

Patient Reflection

The vast array of MALS-related symptoms confused physicians who had never heard of MALS or who were looking exclusively for the classic triad (abdominal pain, weight loss and mid-epigastric bruit), causing delays with diagnosis and surgical intervention. The delays prompted the PT and me to use biomedical resources, clinical reasoning, and experimentation to develop conservative management strategies for MALS symptoms. More physician knowledge about MALS-related symptoms would have expedited the evaluation and intervention process by eliminating unnecessary tests (e.g. sleep study, hormones, cancer).