

This was an interesting one. A middle aged woman presented to the hospital with a year and a half history of crampy abdominal pain and weight loss. The pain worsened right after eating and the only relief she could get was by vomiting. She had adjusted her diet such that she only ate small meals with soft foods like mashed potatoes and casseroles. Otherwise she was a very healthy, well adjusted female. Never had surgery before.
Now we see a lot of patients as consults who come in with these complaints of chronic vague abdominal pain and nausea. The work-ups usually end up going nowhere. Negative CT scan. Negative endoscopy. Negative ultrasound. They get diagnosed with Irritable Bowel Syndrome (IBS) and get sent home with a crazy concoction of IBS pharmacology. Surgeons don't like these referrals so much. Some might even use a phrase like "waste of time consult" while commiserating in the OR lounge. (Of course not me!)
So I reviewed this lady's case. Sure enough, she'd been admitted several times over the past year. Her radiographic imaging, up to that point, had always been negative. Her blood work was stone cold normal. So I went in see her thinking I'd cruise through a quick little interview. The exam was unremarkable. Nothing appeared awry. Her abdomen was absolutely benign. But something nagged at me. She just seemed so normal. Often times these patients with chronic abdominal complaints can be a handful to deal with. There's a psychosomatic component to their issues that is very hard to quantify. This lady on the other hand was pleasant and genuinely distressed by the symptoms she was having. She couldn't eat the way she wanted. She was losing weight. And the symptoms seemed to consistently occur whenever she ate a larger meal. So I ordered a small bowel follow through (radiology test where you swallow barium and a series of images are taken tracking the barium through your GI tract).
The SBFT showed external compression of the duodenum, suggesting a mechanical problem. Therefore I booked her for surgery, expecting to find either some form of congential malrotation or perhaps the SMA syndrome. Boom goes the dynamite on the malrotation diagnosis. Her duodenum was the size of a summer squash and there was this rat's nest of snarl on the right side of her abdomen involving the cecum, the mesenteric pedicle and the proximal jejunum, all abnormally affixed together. Bands of congential scar tissue had partially entrapped the duodenum with a resultant partial torsion of the mesenteric vessels. Her SMV, compensating for reduced return over the years, had swollen to a ropish diameter, like what you see in cirrhotics with portal hypertension. The operation to fix this, Ladd's Procedure, is actually quite cool. You divide all the bands, straighten out the mesentery, lyse interloop adhesions, and what you find once everything is freed up is that the cecum wants to lie on the left side of the abdomen. So you can perform a cecopexy, affixing the cecum to the parietal peritoneum under the spleen. You also take out the appendix because if the patient ever develops left upper quadrant abdominal pain in the future, appendicitis won't be suspected.
It's a fun operation. For some reason this was my third case of malrotation on an adult. Fortunately all worked out well. The lesson as always: listen to your patients, especially those who seem reasonable...

16 comments:
Buckeye, you definitely have the x-factor. Lucky patient she is.
What a strong woman. Really. Probably dealing with so much doubt for so long. Questions about her mental status, and everything that goes with that.
It’s possible she might go through a bit of a breakdown, Buckeye. She’s had to be a different person to keep herself going. If she has not had support of those around her while she was without dx, she will feel tremendously betrayed. Hope she makes it through emotionally. You know you are going to be her knight in shining armor now.
-SCRN
Great case. I wish I'll have a patient with malrotation, probably soon. Classic.
Good for you she's been able to give you all the info. I don't want to assist a non-talking patient like the one I had recently. Pure torture.
Great ..
so inspiring .. and I will keep this lesson in mind. Thanks alot for sharing.
Ahmed.
GS resident PGY-1
the sbft shows the malro -- abnormal position of the djj.. cecum looks a bit high too.
later,
radinc
So glad you listened not only to her but your 'gut instinct'. I'm resigned to knowing I'll never know what my gut issues are about. They'll find it at autopsy is my mantra and I keep on truckin as best as I can. I have a suspicion that it's edometriosis tangling up my innards. Had it everywhere (stage IV) years ago and regardless of what some docs say, it never really does 'go away'. Great catch! So glad to know she must be feeling much better!
I like the prone film best. The duodenum is pretty classic the way it does not cross the midline. I would give it 100% straight up.
I am a 46 yr old who was diagnosed with malrotation almost 2 years ago. I have been having symptoms of intermittent obstructions for years now but due to being diagnosed with IBS by a gastroenterologist, I am having a hard time convincing my surgeon that this malrotation should be fixed. Any suggestions?
I wish I lived in Ohio so you could do my surgery ...recently diagnosed with malrotation at age 50. Just a little nervous having the surgery with a doc that isn't all that familiar with this condition. My symptoms are back ache and abdominal pressure...so docs aren't convinced it's the malrotation although nothing else can be found. No pain..no vomiting...just walking around uncomfortable kinda like having a rock in your shoe. It's great there are docs willing to really listen to their patients. The docs here are good too...just wishing for more experience and/or interest in my condition.
Have you ever had a patient with recurring problems after surgery? A little over nine years ago I received this procedure and have always had some issues with distention and discomfort that has gotten progressively worse. Doctors, knowing I have already had the surgery, tell me it is IBS but I am not convinced.
I wish you were my doctor. I was dx colic as child. Grew up with horrible sharp stomach pain. bloated al life with severe constipation, belly bloat would go down after cleansing with laxatives and water... all my life horrible quality of life. now this yr doc dx midgut malrotation. the sm.intestine all bunched up on r.side. has x-rays, ct-scan with contract, more xrays, fluroscopy. my ladds did not develope. i also have fibromyalgia... i think this is really taking a toll on my body.. my ka*ser p*ermenente surgeon has never done this procedure so I asked to see pediatric surgeon, will wait and see, meanwhile i look 4 months pregnant, sharp pain right side almost everyday upon walking or doing chores.. i work 40+ hours week on my feet, mananger at retail and I am so exausted. I would come see you and make payments my whole life if I knew you could fix this or at least try. joyceninneman@hotmail.com
I'm a 40 year old woman and had the LADDs procedure surgery a year ago. I had malrotation with mid gut volvulos that snuck up on me out of no where. It took several CT scans to find it. Listening to the patient and not giving up is essential. My main issue was incredible pain after eating. As food moved through bowels I felt more comfortable. Surgery performed by Colo/Rectal surgeon in Baltimore MD. Did great job. Had other surgeries due to adhesion obstructions and now diagnosed with Crohn's disease. Inflammation and strictures seen in colonoscopy.
Doing better on Crohn's meds and keeping POSITIVE.
So glad to see that I am not the only person out there with this "rarity". My first attack was at 19 and my first surgery laparoscopicly was at 25. Because the doctor did it laparoscopicly they missed 3 Ladd's Bands and I was in pain for 3 more years till I finally found a doctor who believed me. He went in and did a laparotomy and found a balloon in my intenstine from the Ladd's Bands strangling my intestine. The pain I endured from 19 to about 2-3 years AFTER my laparotomy was intense. All doctors would do is throw opiates at me (which only makes it worse since you're slowing down the bowl process). All I can say is: Be your OWN advocate! Own this rare condition and best of luck!
I have a question, I have congenital malrotation, I had surgery when I was twelve. It took the doctors 2 years to figure it out, I have severe abdominal pain again I've been going to the doctor, having test ran etc, for almost a year. they cant find out what is wrong. I am wondering, Is it possible to have SMA syndrome and congenital malrotation. I have one Dr. telling me I have SMA syndrome and another telling me its impossible for me to have SMA syndrome because of my congenital malrotation.
my 4 year old daughter was finally diagnosed with intestinal malrotation last year, after my persistance for further testing after many episodes spanning over 2years of green vomitting for 3-5 days at a time. docs told us each time she had "cyclic vomitting syndrome" and sent us home. July 2011 she underwent sugergy to correct: repositioning intestines, removing Ladd's band (huge..surgeon stated she sees this size in teenagers), and removed appendix. She is now 5 and recently has had a couple of episodes of vomitting again but NOT green (once in Feb 2012 and once in March 2012). and her appetite has drastically decreased in the last 4 weeks. what are the chances of something else going on as a result of her surgery last summer? relapse, or complication?
Dear Doctor,
I am crying right now writing this... This story sounds very similar to mine and i really need someone to believe that my pains are real. I will be contacting you tomorrow. I live in the akron area, been seen by countless dr;s, admitted several times just be thrown pain killers and told to go home. I NEED HELP!!!! PLEASE!!!! I had an attack last night and I can not take it any more, I have 2 kids and I do not want them to see me like this anymore.
Oh and I should mention I am 29 yrs old. I had a Duedinia Atria surugery 11 years ago. That is when the complications....the attacks started to occur.
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