Biliary dyskinesia in child with dermatomyositis - Forum

[Scalpel]

I would be reluctant to perform cholecystectomy in a child with dermatomyositis with poor gallbladder emptying but no stones.

My limited experience (1 patient) with surgery in an adult with dermatomyositis was bad. This was an about 69 year old white female who perforated a sigmoid diverticulum. Her intestines and mesentery were like butter. Her abdominal incision never healed--I closed her with retention sutures and she developed open necrotic ulcers around and beneath each retention suture--I debrided these areas daily for weeks. She developed Candida sepsis and eventually died.

I would want a very good indication before operating on another patient with dermatomyositis. One of the maxims at the University of Kansas where I trained was never to do GI surgery on a patient with scleroderma. My experience with dermatomyositis was similar.

On the other hand, I just performed a lap chole on a long-term rheumatoid arthritic on low dose (5 mg prednisone) and low dose methotrexate with abnormal gallbladder emptying and no stones. 2 weeks later, her long-term RUQ pain is gone, but now she has lower abdominal pain--so it is too early to tell whether this was helpful.

I have done a lot of lap choles for poor gallbladder emptying. Most of these patients are happy with the result. I do forewarn them all, though, that there is only about a 50% chance that cholecystectomy will relieve their pain.

[Sweden surgeon]

I do lap choles in patients with poor emptying fractions on hepatobiliary scans.

I still also do duodenal drainages looking for gallbladder bile crystals. I do a regular UGI endoscopy but as soon as I get through the cricopharyngeus muscle, I have the Anesthetist give Cholecystokinin. It takes about 5 minutes for gallbladder bile to reach the duodenum. I collect bile from the duodenum (into a mucus trap attached to the scope suction channel). If this bile contains crystals, cholecystectomy helps at least 50% of the patients.

[Surgeon]

Currently, in a patient with RUQ pain that sounds like a gallbladder problem, I start with gallbladder ultrasound. If it shows stones, I recommend chole.

If normal gallbladder sonogram and pain persists, I get a hepatobiliary scan with CCK (gallbladder emptying fraction). If poor gallbladder emptying, I recommend chole.

If normal gallbladder emptying and patient wants to pursue diagnosis further, I do UGI endoscopy with CCK and collect bile for crystals. This lets me look at distal esophagus, stomach and duodenum and be sure the problem isn't reflux or ulcers. If the bile contains crystals, I recommend chole with caveat that it will help about 50%.

[Alalo]

We don't indicated chole in patients without stones. Naturally if they don't have complications as acute cholecistites. When they have crystals we use oral medications to dissolutions. Is current use in your Hospital infusions with MTBE to stones dissolution?

[Jorjo]

If symptoms seem classic for g.b. disease and sono negative, I do an EGD and collect bile as previously described by Dr. surgeon. If crystals are seen and there is no unsuspected PUD or gastritis, I proceed to L/S Chole. If there is "significant" gastritis and/or PUD, I treat with H2 Blocker or Prilosec (as indicated) and for helicobacter. If symptoms persist I then proceed to L/S Chole.

If NO crystals are seen acalculous disease is not excluded. If I continue to think that symptoms are biliary I then get a gallbladder ejec tion fraction study using 35% as my cutoff OR the reproduction of pain symptom s (not just nausea) as the criteria for proceeding with operation.

Where I trained (Ohio State University) this disorder was not given much credence. The problem is in practice I continue to run into patients who have longterm symptoms that no one will deal with because they "didn't re ad the book." For example, I just operated on a woman who has had severe symptoms for >4 years!! who wasn't being helped by that gastroenterologis t, but who wouldn't refer her. Her ejection fraction was est. 0% and immedi ate relief from pain on eating was noted.

Using the above criteria I have had a long string of very happy patients. I can think of one out of an estimated 30-35 patients who have not been helped. But I have had my share of unsuspected (based on H&P alone) PUD; once treated sxs resolved.

[Grandpa Phil]

I tend to operate those patients with biliary dyskinesia who don't respond to a trial of H2 blockers and who don't have any identifiable pathology on standard EGD. Because I'm going to operate them anyway, I question the utility of bile sampling for crystals for anything other than academic interest. I'm at a small hospital without the support of a gastroenterologist. I never got trained in ERCP due to "turf battles" at my training hospital. It is a skill I would dearly love to acquire. This, by the way, makes my conversion rate somewhat higher, as I'm forced to open those cases with common duct stones. On the few cases when I know there is choledocholithiasis, I can send the patients 200 miles away to get an ERCP and sphincterotomy as an outpatient prior to doing their LGB. Thanks for your response.