Stefan Holubar, MD, on Twisted Pouch Syndrome

Dr Holubar discusses the phenomenon of "twisted pouch syndrome" among patients who have had ileal pouch-anal anastamosis surgery and his experience in correcting the condition through surgery.

Stefan Holubar, MD, is the section chief of IBD Surgery and director of research at Cleveland Clinic in Cleveland, Ohio.

TRANSCRIPT:

Stefan Holubar:  Hi, I'm Stefan Holubar. I'm the IBD Surgery section chief and also the director research here in the Department of Colorectal Surgery at Cleveland Clinic main campus.

Today we're going to speak about a recent case series that we're working on here at Cleveland Clinic in Department of Colorectal Surgery of a subgroup of redo pouch patients who have something that we diagnosed as twisted pouch syndrome.

About a year and a half ago, I did a virtual visit during the pandemic for a patient who had had an ileal pouch-anal anastomosis in the remote past and was plagued postoperatively with abdominal pain, especially in her pelvis, and it was very severe, requiring medical therapy.

Patient also had quite erratic bowel habits and symptoms of obstruction. This particular patient ultimately underwent a diverting ileostomy and also had had another exploratory laparotomy.

Despite having her pouch disconnected and having an ileostomy, she continued to have the pelvic pain, which was pretty unusual, and it sounded like it was very ischemic type pain to me, based on her description.

I suspected that she may have had a mechanically twisted pouch that was inadvertently made in a malrotated manner at the time of construction. We brought her to Cleveland Clinic for some additional workup and testing as well as the examination under anesthesia.

Ultimately, we did find that, unfortunately, her pouch had been twisted in a 360-degree manner. This is one of the things that surgeons have to take especially extra care to avoid during the time of the pouch construction.

What we did was we disconnected her pouch and we reconnected it. Several months later, we reversed the ileostomy. The patient had a successful outcome and is now free of pain and having normal pouch function.

At the time, I suspected twisted pouch syndrome and I looked it up on PubMed and did research in MEDLINE to see if I could find. I assumed someone had written this up, but it's very rare.

So I started a clinical series of reviewing almost 600 redo pouches here at Cleveland Clinic. We found about 31 patients who had been diagnosed with a twisted pouch and successfully had a redo pouch surgery, where specifically the pouch itself was good but it needs to be disconnected, untwisted, and then reconnected.

There's multiple interesting things in that study that we found. We did find the majority of the patients did have the triad of obstructive defecation with erratic bowel habits and severe, often opiate-requiring pain.

We also found that these patients were heavily pretreated with biologics, presumed infectious inflammatory complications of their pouch, such as Crohn's disease of the pouch, and I think half of the patients had actually received biologic therapy for this mechanical complication because it was very hard to recognize.

The other striking thing—other than that a lot of them had unfortunately been misdiagnosed or not properly diagnosed and had been poorly pretreated—was that majority of the cases were actually only diagnosed at the time of exploratory surgery here at Cleveland Clinic.

I think about half of the patients before they came here had already had a surgery as well as an extensive workup but the twisted pouch is still not being diagnosed. It is very, very difficult to diagnose. Pouchoscopy, pouchograms, cross-section imaging with a CT and MR—the majority of these were not able to make a successful diagnosis, although some of the time they were. The majority, I believe it was 75% of the patients, had a diagnosis done at the time of rediversion or at the time of exploratory laparotomy here at Cleveland Clinic.

That just emphasizes the difficulty with which to make the diagnosis. It is. It's not surprising in light of that that a lot of patients were heavily pretreated with biologics.

The good news that we found was that when it's been recognized and properly treated with revisional surgery, the outcomes were very, very good. At 1 year, the pouch survival rate was about 90%, which was very high for redo pouch population.