Getting SMAS-hed: A Guide for Learning Superior Mesenteric Artery Syndrome Causes, Signs, and Resources

If you've recently received a SMAS diagnosis or you’re actively seeking information about this condition, you're not alone. In this article, we'll delve into the causes, symptoms, and treatment options for SMAS, providing valuable resources to navigate this condition and empower you on your journey to better digestive health.

What is SMA Syndrome?

The abbreviation SMA stands for Superior Mesenteric Artery. However, SMA syndrome is a gastrointestinal disease; a disease that affects your digestive system. Why is a disease that affects your gut named after a blood vessel? To understand this a bit more, we need to learn about the players involved, the superior mesenteric artery and the duodenum.

The superior mesenteric artery (SMA) is a blood vessel that branches off the aorta. This creates an angle known as the aorto-mesenteric angle. The aorta is the biggest blood vessel that brings blood to the body. This artery supplies blood to the pancreas, duodenum, and small intestine. The duodenum runs through the space between these blood vessels. The duodenum is the first part of the intestines connected to the stomach. Its job is to absorb nutrients. There is fat padding in between all of these organs to help protect and separate them.

Other names for SMA syndrome include Wilkie’s Syndrome, Aorto-Mesenteric Syndrome, and Cast Syndrome.

What causes SMA syndrome?

When the fat surrounding the SMA, aorta, and duodenum decreases with weight loss, the space between all of these organs decreases. Without that extra padding to separate these organs, the SMA and aorta close in on the duodenum. One way to picture this is like a traffic jam on the freeway. When there’s lane closures, traffic builds up. In a similar way, the SMA and the aorta close in and obstruct the duodenum. This blocks the digestive system and prevents food from moving through smoothly and getting absorbed. This can cause discomfort and pressure to build up.

SMAS & Scoliosis Corrective Surgery

Approximately 1-4.7% of people who undergo scoliosis surgery develop SMAS. This is why SMAS also goes by Cast Syndrome, referring to the cast worn to correct spine curvature. When the curve is corrected drastically, this pushes the duodenum harder against the superior mesenteric artery.

What are the symptoms of SMAS?

Some symptoms of SMAS include:

• Pain in your upper middle stomach after eating (mild obstruction)
• Feeling full after eating small amounts of food (mild obstruction)
• Nausea (severe obstruction)
• Vomiting (severe obstruction)
• Food fear (pain surrounding food can lead to avoidance)
• Weight loss

Guava can be used to help track symptoms like nausea, vomiting, and weight trends over time with symptom logging and correlations.

Who is most likely to be affected by SMAS?

Those who are typically affected by SMAS are those who have a small, thin frame, low BMI, experienced significant weight loss, or underwent scoliosis corrective surgery.

Gastroparesis and SMAS

Gastroparesis can often occur with SMAS. Gastroparesis is a condition where food is delayed from leaving your stomach and getting digested, resulting in abdominal pain. SMAS has similar symptoms to gastroparesis including nausea, vomiting, and feeling full after small meals.

How rare is SMA syndrome?

It’s estimated that between 0.1-0.3% of people in the United States (340,000 - 1,000,000) have Superior Mesenteric Artery Syndrome.

Getting Diagnosed with SMAS

SMAS diagnosis is often delayed since it is rare and has nonspecific symptoms.

SMAS diagnostic procedures can include:

• X ray
• Barium x-ray
• Endoscopy
• CT scan (standard for diagnosis)

Timely diagnosis is crucial, since patients can suffer from malnutrition, dehydration, GI bleeding, or gastric perforation (holes in the digestive tract).

Doctors & Specialists for SMAS

If you've developed SMAS symptoms, received a SMAS diagnosis, are seeking a treatment plan, or looking for tools to learn coping skills, types of healthcare professionals to consult include:

Doctors/Specialists to consult for SMAS

• Gastroenterologists
• Dietitians or nutritionists
• Vascular specialists
• Psychologists or therapists
• Psychiatrist

What are the treatments available for SMAS?

Nonsurgical Treatments

• Nutrition support through parenteral nutrition (tube feeding)
• Correction of electrolyte imbalances
• Gastrointestinal decompression

Conservative Therapies

Conservative therapy is typically tried before surgery and may include habit changes such as:

• Eating small, frequent meals
• Sitting with knees to chest

If these treatments and therapies do not relieve symptoms within 6 to 8 weeks, then surgery is typically considered.

Surgical Treatments

Different surgeries that SMAS patients often undergo include:

• Laparoscopic Duodenojejunostomy (Most Common)
• Gastrojejunostomy
• Strong procedure

Community Resources and Support for People affected by SMAS

If you have been diagnosed recently with SMAS or suspect you have it, it is important to know what community resources are available to you as you navigate the challenges accompanied by this disease. Some different components to help you through this include:

• Medical care/nutritional support
• Mental and emotional support from loved ones
• SMAS support groups
• Educational resources
• Advocacy organizations (SMAS Awareness Foundation)
• Health apps (like Guava) that offer symptom tracking, med logging, and quick access to medical records to ease health management

Raising SMAS Awareness

In this personal case report, written by a medical student who battled with Superior Mesenteric Artery Syndrome (SMAS) herself, Rachel Nyenhuis shares her experience with SMAS diagnosis and treatment. At just 16 years old, Rachel faced significant weight loss triggered by a stomach virus, leading to an onslaught of symptoms — including decreased appetite, early satiety, and mild nausea — which further exacerbated her condition. Her journey was marked by a distressing period of misdiagnoses and skepticism from healthcare providers, undermining the trust she had in the medical system.

Eventually, Rachel's gastroenterologist recognized a symptom: relief when she sat with her knees to her chest. Her gastroenterologist had Rachel take a Barium swallow test, which eventually led to her diagnosis of SMAS. Despite attempting non-surgical treatments, including a high-calorie diet and nutritional supplements, her symptoms worsened, culminating in food aversion. Multiple unsuccessful interventions, surgeries, and post-operative complications impacted her physical and emotional well-being, significantly altering the course of her life.

Motivated by her personal experience, Rachel decided to pursue a medical career, driven by the empathy she developed for patients enduring similar suffering. Her story underscores the importance of early consideration of SMAS in diagnosis and the critical need to believe and validate patients' experiences, ultimately advocating for increased awareness and accurate diagnosis of this debilitating condition.

Conclusion

Not every SMAS diagnosis story is like Rachel’s, but it reveals that raising awareness about SMAS among healthcare professionals can help avoid misdiagnoses and mistrust between patients and doctors. By raising awareness for SMAS, we can increase empathy, timely diagnosis, and understanding of the physical and emotional challenges that individuals with SMAS face. By increasing awareness and keeping SMAS on the radar of medical practitioners, we can offer better support and care to those affected by this complex disease.