Tuesday, February 23, 2010

Malrotation in the Adult



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...

24 comments:

Anonymous said...

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

Sergiu said...

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.

a7mdk said...

Great ..
so inspiring .. and I will keep this lesson in mind. Thanks alot for sharing.

Ahmed.
GS resident PGY-1

radinc said...

the sbft shows the malro -- abnormal position of the djj.. cecum looks a bit high too.

later,
radinc

Anonymous said...

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!

Anonymous said...

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.

Anonymous said...

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?

Melanie said...

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.

Anonymous said...

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.

Anonymous said...

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

Anonymous said...

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.

Anonymous said...

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!

Mallory said...

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.

Anonymous said...

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?

Anonymous said...

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.

Anonymous said...

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.

Anonymous said...

I was diagnosed with intestinal malrotation at twenty one, almost a year ago. I suffered for six yeears, vomiting repeatedly after every meal. One doctor diagnosed me with "an angry belly" one doctor diagnosed me with "reverse ibs" one told me to my face i was a liar. I have been misdiagnosed with acid reflux more times than i can count. I was told that i was suffering from intense ocd and that is why i am vomitting, they actually where about to recomend therapy. After all of that they finnally found out that i had a mess of intestines on the right of my abdomen. the doctor who called me a liar was finally asked to appologize:D I am finally healthy now, but I dont believe i will fully trust a doctor again.

Jodi O. said...

I'm 54 and just found out today that I have congenital malrotation. I have been suffering my entire life. As I child, I was labeled a "picky eater" but I had no way to explain why food hurt me so much. I was pretty much underweight my whole life up until menopause (age 48) in which I gained 20+ lbs. and I am 5'4" and 126 lbs. I had a hysterectomy due to POP (Pelvic Organ Prolapse) 3 years ago. I have a transdermal vaginal mesh holding up my bladder. I have repeatedly gone to the doctors only to be told it could be gas, IBS, food intolerance, heartburn, etc. I've had a sigmoidoscopy, colonoscopy and endoscopy and my results always read normal. I just had a CT scan last week and it revealed my condition. I never understood why I would look so bloated and distended even after I adjusted my diet and eliminated lactose, sugar and gluten. I had an obstruction when I was 20, but they didn't come up with this diagnosis. Perhaps it was something they weren't looking for 30+ years ago. I have to admit I am somewhat relieved with this finding of congenital malrotation because it means it is real and I don't have to diminish the pain I feel every day or make excuses for not feeling well. I don't know if I will consider surgery right away, I want to try to manage the pain with exercise and eating properly and take it one day at a time. Being able to read other's stories has been very therapeutic and I'm glad I had a chance to spill my guts somewhere it would be understood.

Anonymous said...

Woman, 30 years old
I was diagnosed with a malrotation of the cecum--where part of my large intestine along with my appendix are in the upper right quadrant as opposed to the lower right quadrant. I occasionally have some abdominal pain...usually it's associated with constipation or stress. I am ALWAYS constipated, go through bouts of lethargy and moments of feeling run-down, and have chronic lower back pain...which I've had for as long as I can remember. My main question is, Do you think this back pain is due to my malrotation? Also, where do you suggest I go from here in terms of fixing my problem? The last Dr. I met with basically said there's nothing to be done unless I'm in severe pain-at which point I would be considered for surgery. Also, I'd just like to note that I wish there was more information/advice out there for adults--I know it's rare, but some of us older folk have it!!
Thanks so much

Anonymous said...

I am 34 and just diagnosed with malrotation last week. I was already scheduled for surgery to remove a large ovarian cyst and 2 days prior ended up in the ER in so much pain all throughout my abdomen - especially the upper right quadrant under my ribs.. A CT scan reveled malrotation. I have had bowel problems my entire life - but it was somewhat manageable with diet and exercise. I am planning on contacting a pediatric surgeon to see if they know of a doctor who has treated an adult with this condition. I live near Syracuse, NY. I too have found drs so frustrating - I was told I had IBS at one point - I too wish there was a lot more info on this condition for adults. It is so frustrating - there is a fb page - that is private - Awareness for Malrotation - ask to join. Lots of others stories and what they went through.

Karen Weigand said...

My 48 yr old boyfriend has most of the same symptoms you all have mentioned for about 5 years now. Extreme pain....some days he lays in bed just moaning all day....bloating, gas, pain after eating, he can feel things moving through is intestine and hurting. He's been to 2 or 3 gastros, had 2 endoscopies, gall bladder removal, emergency room at least 6-7 times, CTs with and without contrast at each ER visit. He's in pain almost constantly, he often induces vomiting to relieve the pressure but it comes right back, lots of burping and flatulence. He is also unemployed and uninsured now (due to being so ill) and it's almost impossible to find a doctor who will even see him, much less one who actually WANTS to help. We are at the end of our rope and, to be quite frank, he has mentioned shooting himself in the head several time (but assures me he wouldn't). He's lost about 60+ lbs. and looks like a concentration camp victim. He has family in OH and is quite willing to come there to see you if necessary. If you know of someone in Arizona that can help, that would be great too. Please feel free to contact me if you can help in any way (Karen wild-heart@cox.net). Thank you!

Elena said...

Hi! I.m a 32 year-old woman and I have been having trouble with my stomach / upper righ abdomen / back for 5 months now and I have been diagnosed with "acalculous gallbladder" and during the tests they have also found a malrotation (I don't know what type!). I've been scheduled for surgery to remove my gallbladder on the 1st of May and the surgeon does not want to even hear anything about dealing with the malrotation at all!!! I,m a bit concerned because I have the feeling that he.s hoping the pain will go away after surgery but he is not sure there,s anything wrong with it. I feel I am going to have my gallbladder removed with a 50% chance of going for an open procedure for no reason or whatsoever!!! But at the same time, he is the doctor and I want this back pain away!!! Is there any surgeon near Scotland interested in dealing with my case at all? :)... I am very happy to be used as a wee guinea pig if it means more quality of life and peace of mind in the long term!!! Any suggestions welcome... Thanks for your time

Anonymous said...

I just had the ladds procedure on the 12th of August discharged from hospital on post op day 2 am back eating regular food with minimal pain although I did catch a NASTY cold lol I believe that it is helping me already I am having rrgual bowel movements and not feeling bloated I haven't had to take any sort of medication due to upset stomach the doctor who did my procedure did tell me he wasn't positive that the malrotation is what was causing my problem with my stomach since childhood but he went ahead with the procedure thinking I would benefit from it.

camnchar said...

I've had same problem for 4 years it wasn't until I researched on my own under undescended cecum that I had an Barium x-ray which looks similar to the one posted in the article. I have an S-shaped large colon with several twists and looping some specialists claim my small bowell is on the left side and others contradict this. I am very confused.

You sound like my knight in shining armor as all the GI specialists I've seen contradict each other--some say full abdominal colectomy is required others only a portion where I am having most pain (Upper left quad) I sympathize with the emotional state as most finding come back normal and love how you shared the need to LISTEN to your patient--I was accused of being anorexic as I've lost a great deal of weight. If I could I would post a picture of my barium x-ray. Love for someone like you with incredible insight and expertise to take a look.

Either way inspiring article thank you--answered many prayers.

If this sounds familiar to something you've encountered and have any insights would you mind emailing me? camnchar@comcast.net

Thank you!