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A Difficult Diagnosis

Is this patient's fistula caused by Crohn disease of the pouch?

Esterow, Joanna PA-C; Esen, Eren MD; Remzi, Feza H. MD

Author Information
Journal of the American Academy of Physician Assistants: July 2020 - Volume 33 - Issue 7 - p 54-56
doi: 10.1097/01.JAA.0000668856.35200.4b
  • Free

CASE

A 50-year-old woman with a history of ulcerative colitis was referred to the inflammatory bowel disease (IBD) center about a pouch-vaginal fistula that had been impairing her quality of life over the past 3 years.

History

The patient was diagnosed with ulcerative colitis in her 20s during her first pregnancy. The diagnosis was made by colonoscopy and biopsy. The history of small bowel disease and absence of perianal disease, fistulae, or abscesses around her anus were reassuring for the diagnosis of ulcerative colitis compared with Crohn disease as her initial presentation. Medical therapy failed, so she underwent a two-stage ileal pouch anal anastomosis (IPAA) with a J-pouch procedure 18 years ago. The two-stage procedure consisted of total proctocolectomy with J-pouch creation and creation of a loop ileostomy, and closure of the ileostomy after 3 months. After surgery, she continued to have up to 15 bowel movements per day. Three years ago, she developed a pouch-vaginal fistula and had feculent material draining from her vagina. This was initially diagnosed as Crohn disease of the pouch, and a seton, a plastic string allowing the fistula to drain without developing an abscess, was placed. The patient considered the seton uncomfortable and had it removed a year before presenting to the IBD center.

At the initial IBD center appointment, she rated her quality of life as a 2 on a 0-to-10 scale, with increased restrictions in four categories of life (social, sexual, work, and dietary). She reported the decrease in quality of life as mainly secondary to her fistula causing pain and embarrassment due to a foul smell. She did not work or leave her house often. She denied any stool coming from her vagina at the time of her visit, but had fecal incontinence and needed to wear diapers. Her past medical history was significant for ulcerative colitis and hypothyroidism. Family history was unremarkable. She denied smoking or use of alcohol or recreational drugs.

Physical examination

The patient's vital signs were stable. Her weight was 113 lb (51.3 kg) and BMI was 22 kg/m2. She appeared well developed and well nourished without acute distress. Her abdomen was soft. Bowel sounds were normal. Abdominal examination was significant for a midline scar, a right lower quadrant scar (old stoma site), and a large ventral hernia. Rectal examination revealed excoriated skin; no clear fistula orifice was visualized. A digital rectal examination was not performed at visit because of the patient's refusal. The rest of her physical examination was unremarkable.

Diagnostic testing

MRI of the pelvis revealed a moderate to large burden of stool in the ileal pouch but no signs of pouchitis or active bowel inflammation. Perianal abscess or fistula were not visualized.

A gastrograffin enema (GGE) showed that the anal canal was strictured and the patient had a perianal fistula resulting in dilation of the immediately proximal small bowel (Figure 1).

FIGURE 1.
FIGURE 1.:
GGE showing a strictured anal canal, perianal fistula, and dilation of the proximal small bowel

DIFFERENTIAL DIAGNOSIS

  • Mechanical issue of the J-pouch
  • Crohn disease of the pouch
  • Pouchitis

OUTCOME

The patient was offered an examination under anesthesia to further assess her anatomy and better define what and if any surgical intervention would improve her quality of life and bowel function. The examination revealed a tight pouch stricture with a dilated pouch. The stricture was dilated manually via finger, and flexible pouchoscopy was performed. With this finding, it was likely that the patient had a mechanical issue of her J-pouch, rather than Crohn disease. The lack of inflammation also meant pouchitis was unlikely. The mechanical issue likely caused the stricture, fistula, and overflow incontinence. A three-stage revision J-pouch procedure was recommended.

After consent, the patient underwent a three-stage revision IPAA, with the initial procedure being a diverting loop ileostomy. Six months later, she had a revised J-pouch construction with diverting loop ileostomy. Her operative report from this second surgery was significant with a very inflamed, dirty, and infected chronic pelvic abscess that was cleared. The pouch also was twisted and had to be untwisted during this second stage. After disconnection of the pouch from the IPAA, the pouch was excised and a new J-pouch was created. During the anastomosis of the ileal pouch to the anus, the surgeon must elongate the pouch straight; a rotated or twisted pouch can cause an obstruction. The patient spent 5 days in the hospital and was discharged home without complications.

A repeat GGE was done about 6 weeks postoperatively to ensure no anastomotic leak before closing her ileostomy (Figure 2). She had no signs of extravasation, fistula, or stricture. The ileostomy was closed after 3 months without complications.

FIGURE 2.
FIGURE 2.:
Repeat GGE done 6 weeks postoperatively

Eighteen months after the last surgery, the patient had a surveillance pouchoscopy, which was unremarkable. Her quality of life has improved and she has no social, sexual, work, or dietary restrictions. She moves her bowels three to eight times a day without pain or difficulty, has no incontinence or fistula.

DISCUSSION

IBD consists of ulcerative colitis and Crohn disease, both chronic autoinflammatory intestinal conditions that cause abdominal pain, bloating, bloody diarrhea, weight loss, fatigue, and frequency/urgency. Patients also may have extraintestinal manifestations including pyoderma gangrenosum, arthralgia, and episcleritis. Diagnosis is made by colonoscopy and biopsy. Prevalence of IBD is 0.5% in industrialized countries and reported incidence is around 20 per 100,000 persons.1 Initial treatment of IBD is mostly medical, and great improvements in medical treatment have been made in recent years with the development of immunomodulatory and biologic agents. However, despite recent advances in medical treatment, 20% of patients with ulcerative colitis require surgery in their lifetime.2

Restorative proctocolectomy with IPAA (RP/IPAA) construction is the procedure of choice in these patients. RP/IPAA maintains intestinal continuity, minimizes neoplasia risk, and gives patients acceptable quality of life.3 However, the procedure is not without risk and even in the most experienced hands, about 10% of the IPAAs may fail, mostly due to ongoing pelvic infection.4 Those patients present with bloating, obstructive defecation, weight loss, abdominal or anal pain, diarrhea, fecal incontinence, and fistula. Revising an IPAA is a viable option to maintain intestinal continuity in patients who are otherwise left no choice except pouch excision and a permanent ileostomy. IPAA revision carries a high morbidity and outcomes depend on the expertise of the surgeon and management team. Patients with ileal pouch-related complications should be referred to a facility with expertise on managing complicated ileal pouches.

This patient was diagnosed with ulcerative colitis when she had her primary IPAA. After IPAA, she had never felt right, indicating a possible surgical complication. Her symptomatology was similar to that of patients with Crohn disease of the pouch, which occurs in 2% to 7% of patients after IPAA surgery.5 Because of the similarity in presentation, patients with surgical complications of IPAA may easily be labeled as having Crohn disease of the pouch, and treated with corticosteroids and biologics. Referring a patient with pouch-related symptoms to a specialized IBD center may prevent misdiagnosis. Our initial consultation is a team approach (surgeon and PA) including a review of previous records and updated imaging as needed. Before undergoing an IPAA revision, patients meet with pain management, preadmission testing, stoma nurses, and a PA. Psychology, nutrition, and integrative medicine teams also are available if needed.

CONCLUSION

Recognizing IBD and its complications is crucial to knowing when to refer patients for surgery or to a specialty center. Patients who have undergone a J-pouch procedure need careful and thorough evaluation with a focus on timing of symptoms and medical therapy as well as other interventions. Listening to these experienced patients often is the key to understanding their pouch. When in doubt, contact a specialty center for another opinion. It may dramatically improve the quality of life of these patients.

REFERENCES

1. Molodecky NA, Soon IS, Rabi DM, et al. Increasing incidence and prevalence of the inflammatory bowel diseases with time, based on systematic review. Gastroenterology. 2012;142(1):46–54.e42.
2. Bernell O, Lapidus A, Hellers G. Risk factors for surgery and recurrence in 907 patients with primary ileocaecal Crohn's disease. Br J Surg. 2000;87(12):1697–1701.
3. Remzi FH, Lavryk OA, Ashburn JH, et al. Restorative proctocolectomy: an example of how surgery evolves in response to paradigm shifts in care. Colorectal Dis. 2017;19(11):1003–1012.
4. Remzi FH, Aytac E, Ashburn J, et al. Transabdominal redo ileal pouch surgery for failed restorative proctocolectomy: lessons learned over 500 patients. Ann Surg. 2015;262(4):675–682.
5. Shen B. Crohn's disease of the ileal pouch: reality, diagnosis, and management. Inflamm Bowel Dis. 2009;15(2):284–294.
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