The typical rectal cuff should be only 1-2cm, basically, the smallest possible to make the connection.

Twisting of the pouch could even be due to the too long rectal cuff. A few people here have had this issue and I believe they wound up needing pouch reconstruction surgery.

Not sure about the proctitis cystica profunda, as it seems rather rare. It would need to be confirmed by biopsy. Regardless, I am not sure it would change the treatment in any significant way.

Hopefully, the plan for pouch redo will solve his issues.

Jan

Thank you Jan. Appreciate your knowledge and quick response.

One more question actually -- what is strange is my son's symptoms are more like pouchitis -- frequency and leakage and some urgency but not a lot of blood -- so the lack of blood makes it seem like the cuff would not be the problem? Can you have cuffitis/UC in remaining rectum without blood?

What do you mean by "not a lot of blood"? Even some blood, or a little blood, would be associated with cuffitis. But, yeah, you definitely can have UC in the retained rectum without bleeding. My husband has had UC for more than 10 years and hardly ever bleeds, even with a flare. For me, I nearly always would bleed with a flare.

There is a lot of overlap with pouchitis and cuffitis symptoms. It would be pretty easy for the doctor to know on the scope what was inflamed.

Jan

I have chronic cuffitis/UC and the symptoms are the same as pouchitis, except for the blood is only with UC. I've also had c.diff or pouchitis at the same time as cuffitis. I feel better when given an antibiotic when I have those but I don't feel better when I have cuffitis. Your son's GI could tell by looking at his pouch during the scope what it was cuffitis/UC and the biopsies results will verify it.

If he is going to have surgery please check into the hand sewn procedure vs the double stapled one. In the hand sewn one they don't leave any of the rectum at the rectal cuff and hand sew the pouch to the cuff. In the double stapled procedure they leave 1-2 cm of the rectum at the rectal cuff and use a stapling procedure to attach the pouch to that tissue.

The reason the double stapled procedure with the 1-2 cm of rectum tissue left at the rectal cuff is preferred these days is that it has better outcomes as far as leakage and potential incontinence. I was told there are not too many surgeons that performed the hand sewn procedure anymore. I'm at the point where it has been suggested that I might need to look into the same surgery as your GI is talking about your son having. I do not have any twisting but my take down was two and a half years ago and I've got chronic pouchitis and pain. That's why I'm thinking your GI is probably right. If he is getting UC/cuffitis in there already would he probably get it with 1-2 cm? Would none would be preferable?

I'm sorry he is facing surgery so soon again and hope all turns out well. Please let us know how he's doing.

Frequency may be due to his inability to completely empty the pouch if the cuff is too long. This is also called efferent limb syndrome. Basically the exit channel is too long and remains angulated thus preventing complete evacuation and leaving him with a feeling of always needing to go. Also not fully emptying keeps bad bacteria hanging out causing pouchitis. Twisted pouch could cause same problems.

Has he had a defecogram test?

Is he on any meds for cuffitis like canasa, hydrocort suppositories or rowasa? May help.

Also might want to have him experiment with "toilet gymnastics", different positions when trying to empty. I had efferent limb ANd too big of a pouch, both preventing complete emptying. What helped some was sitting in "airline crash position", bending over as far as possible with head tucked in by my knees when on the toilet.

Seems like you might want to get him to some real experts, like at cleveland clinic. There may be treatments to avoid surgery. But if surgery is required, particularly with pouch redos or repairs thats where you want to be.

I just wanted to add that I had SEVERE refratory cuffitis and never bled. It does happen although is not the norm.

vstRN,
It appears as they left all or part of the rectum that was attached to your rectal cuff and performed a Mucosectomy during your pouch advancement. Is this correct? I'm assuming they also made your pouch smaller too. How are you doing?

Thank you all.

Liz11 - Yes - I get the feeling he cannot empty completely because he does go into that airplane crash position and takes 30-45 minutes in the bathrooom sometimes. On scopes his pouch and cuff had some ulcers but drs had seen much worse in other pouchitis/cuffitis cases. So whether that is from a twist of efferent limb syndrome -- is the answer surgery? Is that a pouch re-do or a pouch "advancement"?
He did have a pouchogram test where they watched him empty liquid from his pouch (not in sitting position) and that was OK but I imagine it would be harder with stool than liquid.
The canasa and hydrcortisone rectal foam only heped the first night and then stopped helping so he sis not continue to use them -- maybe that was a mistake and he needs to try them for a longer time?
TEMarie- thanks for warning re continence problems with hand sewn procedure. For peds in LA there seems to be more hand sewn but in NY the colorectal surgeons we met did stapling.

boy's mom - I am so sorry that your son is having to go through this. As an adult, these surgeries have beaten me down both physically and emotionally. It must be so difficult for you to have to watch your child go through this I really hope the redo will be the answer for him. I had to go through a pouch advancement even though I had just 1 cm of cuff left. VERY frustrating. As TE Marie mentioned, I would definitely do some research of surgeons who have experience with hand sewn. While most people do very well with just a tiny bit of cuff left, some of us unfortunate ones don't. I would hate for him to have to go through any more surgeries after this. While I have no idea if hand sewn would be the best option for him, I would certainly get some second opinions about this.

TE Marie - yes, that is correct. I had a mucosectomy to scrape out all diseased cuff and then a pouch advancement. I really got to a point (although I only had a working j pouch for about 4 months) where I really didn't have much choice. I was in REALLY rough shape. I'm doing pretty well now I guess - normal takedown adjustments which are miserable in general. Only this time, after 6 major surgeries in just short of 1 1/2 years, I have such minimal tolerance and patience for another recovery. I also had to go back to work 2 weeks post-op. I'm a nurse so long shifts and on my feet all the time - not a great combination. But overall, although it has been a lot to go through I'm glad I just went ahead with it and didn't suffer with the cuffitis any longer. My surgeon did it in 3 steps as well so it was a long, drawn out process with a dreaded loop ileostomy. The pouch advancement/mucosectomy surgery was actually the easiest surgery out of all the surgeries I have had. Good luck in your decision!

boy's mum-
I definitely think a defecogram would be in order. That is a test where they can actually see all of the "mechanical" stuff going on when emptying. That is the test I had which gave them the definitive answer that I had efferent limb syndrome (though it was initially diagnosed by Shen within 10seconds of him scoping me) AND my entire pouch was collapsing on itself when emptying because it was too large.

Also - if he was prescribed rectal meds - sometimes you have to stay on them for awhile to get it working.

Just another reminder too, that seems his case might be best handled by experts - both expert GI docs and an expert surgeon.

Thank you Liz11 I think a defogram test would be meaningful. Is surgery the only solution for efferent limb syndrome? In the most recent scope (which is new since my original post in this thread)severe cuffitis was the problem -with the slight twist in the pouch being considered minor in relation to the cuffitis. I would assume a defogram could be done in NYC or LA, not just Cleveland, but so far our drs in both places have not suggested. I will ask. How long is "a while" to get the rectal meds working?