Need Awareness/ Prevention/ Research on Superior Mesenteric Artery Syndrome (SMAS)

Posted by mattis1best @mattis1bestyahoocom, Aug 16, 2011

“With only about 500 reported cases in the history of English-language medical literature, recognition of SMA syndrome as a distinct clinical entity is controversial, with some in the medical community doubting its existence entirely. SMA syndrome is also known as Wilkie’s syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arterio-mesenteric occlusion. It is distinct from Nutcracker syndrome, which is the entrapment of the left renal vein between the AA and the SMA.

SMA syndrome is estimated to have a mortality rate of 1 in 3. Delay in the diagnosis of SMA syndrome can result in fatal catabolysis (advanced malnutrition), dehydration, oliguria, electrolyte abnormalities, hypokalemia, acute gastric rupture or intestinal perforation (from prolonged mesenteric ischemia), gastrectasia, spontaneous upper gastrointestinal bleeding, hypovolemic shock, aspiration pneumonia, or sudden cardiovascular collapse from increased velocity of bloodflow in the SMA due to the reduced mesenteric angle.

Go here to this link to know about my fight http://rare-disease-day-belfast-2011.blogspot.com/2011/02/paula-mattis-is-real-super-hero-she.html ***Visit our sister group, by Samantha Mina: http://www.facebook.com/group.php?gid=170641319067&ref=ts Also go look and my group http://www.facebook.com/group.php?gid=129829963700139&v=info Superior mesenteric artery (SMA) syndrome is a very rare condition characterized by recurrent vomiting, abdominal distention, weight loss, and postprandial distress. The cause is compression of the duodenum between vessels and the vertebrae and paravertebrae muscles when the angle between the superior mesenteric vessels and the aorta is lower than 18°. The diagnosis is difficult and usually is made by exclusion. Conservative management is helpful at first; however, symptom recurrence would indicate surgical treatment. EVERY LIFE COUNTS Let’s get people the Public, Doctors start taking this more seriously and get Awareness/Prevention, researching possible causes and work towards early detection
Thanks, Paula

@gregh

Just wanted to give a quick update on my daughter Shelby. We still haven’t gotten in at Mayo. However she had her TPN pick line put in about three weeks ago. And while it’s early in the process, Shelby has put on about 6 pounds. Doesn’t sound like much to most folks, but to the people on this board they know how important it could be. We had a Sunday family dinner about two weeks ago……….it was the first time in I don’t know how long that I watched her clean her plate. Fried chicken, mash potatoes, biscuits and gravy. Her spirits have been better too.

Like I said, it’s early, I know that. But I’m looking for good anywhere I can find it. We have an appointment with her Gastro on Friday. So maybe there will be more to share.

Thank you so much for thinking about my Princess.

Greg

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@gregh Hello Greg:

I was thinking about you and your daughter. In your last update she was able to work from home and getting parenteral nutrition. I hope that she is still making progress. Also wondering – was she able to get an appointment with the Mayo doctor in Florida?

I would enjoy hearing from you.

Teresa

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My 16 year old was diagnosed at age 14, required NJ feedings for 2 months, then developed gastric dysmotility issues. Anyway she had a relapse in February, but was able to have a ton of IV hydration and it resolved. Well its back. Is anyone familiar with relapses and prevention? Not getting terribly far with the pediatric GI specialist. They wont admit her and do testing until she essentially looses enough weight, and she has only lost 4 lbs in 5 days this time. First time it was 14 lbs in 10 days. She is an active girl, runs track and plays volleyball. I am hoping to get to see a doc at the Mayo, but until then I hate watching her suffer through this. She can't go to school if she vomits, and is too exhausted from nourishment and vomiting.

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Is this for MP? If so, has she been put on any type of steroid therapy?

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@gregh

Just wanted to give a quick update on my daughter Shelby. We still haven’t gotten in at Mayo. However she had her TPN pick line put in about three weeks ago. And while it’s early in the process, Shelby has put on about 6 pounds. Doesn’t sound like much to most folks, but to the people on this board they know how important it could be. We had a Sunday family dinner about two weeks ago……….it was the first time in I don’t know how long that I watched her clean her plate. Fried chicken, mash potatoes, biscuits and gravy. Her spirits have been better too.

Like I said, it’s early, I know that. But I’m looking for good anywhere I can find it. We have an appointment with her Gastro on Friday. So maybe there will be more to share.

Thank you so much for thinking about my Princess.

Greg

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@gregh Hello Greg:

I hope that your daughter is still making progress and gaining weight.

I would enjoy hearing from you when it is convenient.

Teresa

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My 16 yr old has been diagnosed with this syndrome and docs have her on low. Diet for 4 weeks. This isn’t working. Throws up after most ensure feedings. So worried as rare and need to see how others are managing.

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@slballew

My 16 yr old has been diagnosed with this syndrome and docs have her on low. Diet for 4 weeks. This isn’t working. Throws up after most ensure feedings. So worried as rare and need to see how others are managing.

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HI @slballew, welcome to Mayo Clinic Connect. You'll notice that I moved your message to an existing discussion about Superior Mesenteric Artery Syndrome (SMAS). I did this so that you can easily connect with other parents and patients living with SMAS, like @nbackes01 @gregh @jmmb @amyk811 @jenninaloha @carolynlivingjaks @jeffr @sharronbz and others.

If you click VIEW & REPLY you can read through the past messages of this discussion and post your questions to the group.

Is she able to keep anything down?

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@slballew

My 16 yr old has been diagnosed with this syndrome and docs have her on low. Diet for 4 weeks. This isn’t working. Throws up after most ensure feedings. So worried as rare and need to see how others are managing.

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Hi, do you know if they have looked at her esophagus when she swolows food?
My son who is 22 was born with a problem between the spinchter and esophagus. He's had Nissan fundoliplication and Linxs magnetic bead's. He was diagnosed with SMA almost two years ago. He has a feeding tube and had DDJ surgery done back in August of 2017. Which significantly lowered his SMA pain. However, not take it away completely.
He's lost another 8 pounds in 6 months. He is 5"11 and weighs 114 .
We understand, no cure for this. Managing as best as you can.
She may need to stay away from breads, dry meats, anything too dry to be eat.
Good luck. Please let us know how she is doing.

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@slballew

My 16 yr old has been diagnosed with this syndrome and docs have her on low. Diet for 4 weeks. This isn’t working. Throws up after most ensure feedings. So worried as rare and need to see how others are managing.

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Hello @slballew so what exactly was your daughter dx with? I can tell you no matter what eating very small meals more frequently helps some people. Don't try to eat a 'normal' size meal. Does she have MALS with compression? or nerve? Is it just SMAS? There are a lot of good groups out there to connect with and they share ideas as well. I see there are some on facebook….just an idea

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@smfjf101

Hello Amanda,
I’m sorry you are suffering again with symptoms from SMA. My 19 year old daughter had surgery for this. I took her to an ER due to weird uncontrollable vomiting when she stood up. she was admitted and they diagnosed her with SMA. They scheduled laparoscopic surgery for her. She was in hospital for 1 day and it fixed the problem. I don’t know why they wait with some people for so long to surgically fix this. She feels much better and no more vomiting. There were some doctors not agreeing on treatment. One said to put in feeding tube one said to do surgery right away. She was of normal weight, so feeding tube seemed like it would not help in the long run. The surgery was done at the American Center which is part of UW Health in Madison WI. No more issues with SMA. I hope you can get some good treatment for the SMA syndrome. I was so pleased with the way my daughters problem was handled and fixed.

Sue

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Sue,

My daughter who is 25 was diagnosed with SMA syndrome 3 years ago after 3 years of being in the Emergency room ever couple months. She is currently in the hospital with a nasogastric tube continually pumping her stomach and a PICC line for intravenous feeding. The situation in the past has always calmed down after about 12 hours in the hospital but this time it hasn’t reset. The doctors all know she needs surgery but say they don’t know anyone who does it. They are willing to give her an outside referral but can’t seem to find a doctor. I there anyway possible for you to forward me your daughters doctors information? I will be eternally grateful. I am happy your daughter is much better and wish her and you the best.

Joe

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@jcvece

Sue,

My daughter who is 25 was diagnosed with SMA syndrome 3 years ago after 3 years of being in the Emergency room ever couple months. She is currently in the hospital with a nasogastric tube continually pumping her stomach and a PICC line for intravenous feeding. The situation in the past has always calmed down after about 12 hours in the hospital but this time it hasn’t reset. The doctors all know she needs surgery but say they don’t know anyone who does it. They are willing to give her an outside referral but can’t seem to find a doctor. I there anyway possible for you to forward me your daughters doctors information? I will be eternally grateful. I am happy your daughter is much better and wish her and you the best.

Joe

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Where are you located? If you look on a face book group called MALS pals there is a list of surgeons by states who perform MALS surgeries. I had MALS, median arcuate ligament syndrome, but it didn't just affect the celiac artery , it also affected the SMA and MALS surgeon did it. Just an idea….good luck.

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@smfjf101 – Hi Sue, can you tell me who did your daughters surgery in Madison – and what type of surgery she had? I know this is an old post. Hope she is doing well. My daughter has SMAS and we are just looking into surgery. Thanks.

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For a couple years now, I’ve had postprandial pain and cannot eat a normal size meal, poor gastric motility (resulting in regular use of laxatives), pelvic/flank pain, excessive fatigue. Over the last 8 months, I’ve been diagnosed with MALS, then severe celiac artery compression not caused by MALS or atherosclerosis, then SMA, and MALS was being considered again. Both diagnoses were ruled out and my team of surgeons were befuddled. Upon further testing, they found severe renal vein compression (between aorta and SMA). Duodenum swallow test was normal. The latest diagnosis is pelvic congestion syndrome (ovarian varicose). The surgeon wants to do coil embolization of ovarian vein and asked me about four times when I wanted to schedule this procedure. I was hesitant as, ironically, two of the other surgeons said not to do this procedure because the blood would have nowhere to go except back up to the kidney. My eating problem was lost somewhere along the way. I’m so angry, very depressed, and frustrated that this is where I’m at after dozens of tests!!! Please, if there is anyone out there that has any thoughts on this, I am all ears! Thank you for reading my long post.

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I’ve just been diagnosed with. MSMAS AD NCP. I DONT KNOW WHERE TO GO FROM HERE. My vascular doctor gave me. Orders for an endoscopy but cant Get appointment with a GI until October. I’ve lost 10 lb in the last 3 weeks, I don’t think I can wait anymore-uggestions?

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