Connect
Anyone else have Superior Mesenteric Artery Syndrome (SMAS)?
"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
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
Like
Helpful
HugInterested in more discussions like this? Go to the Digestive Health Support Group.
Connect
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
-
Like -
Helpful -
Hug
1 ReactionHello @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
-
Like -
Helpful -
Hug
3 ReactionsHi, 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.
-
Like -
Helpful -
Hug
5 ReactionsHI @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?
-
Like -
Helpful -
Hug
2 ReactionsMy 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.
-
Like -
Helpful -
Hug
1 Reaction@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
-
Like -
Helpful -
Hug
1 ReactionIs this for MP? If so, has she been put on any type of steroid therapy?
-
Like -
Helpful -
Hug
1 ReactionMy 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.
-
Like -
Helpful -
Hug
3 Reactions@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
-
Like -
Helpful -
Hug
1 Reaction@gregh Hello Greg:
Thank you so much for the update! I'm glad to hear that she is doing better. Yes, I believe that a 6 pound gain is worth celebrating!!
Please keep us posted on the progress of your Princess, we are all in her corner and cheering her on.
Teresa
-
Like -
Helpful -
Hug
3 Reactions