Background Illness:
2011
Initially diagnosed as Ulcerative colitis (pancolitis) and started on Mesalamine.
2012
· Developed pancreatitis – mild interstitial, ? 5ASA-induced.
· She received a course of steroids for active disease and experienced flare with tapering of steroids.
· July - Underwent sub-total colectomy with loop ileostomy
· September – Underwent proctectomy with IPAA
· December – ileostomy closure
· On POD6 after ileostomy closure, the patient developed a pouchovaginal fistula, hence diversion ileostomy was done.
2013 à
· May - Pouchovaginal fistula repair done with labial flap.
· November – Ileostomy reversal done.
December 2013 to Dec 2023
· Uneventful
· Stool frequency: 4 to 5 per day, Bristol 5 in consistency, No mucus or blood.
· Weight gain of 12 kg
History of presenting complaints:
From 2024, Jan
· Complained of Increased stool frequency, occasionally associated with blood and recurrent oral ulceration
· Oral examination revealed the presence of ulcers on both soft and hard palate
· A Pouchoscopy was done, and the findings were as follows (figure 1)
Fistulous opening just above the anal verge
Diffuse oedema of the pouch with superficial ulcerations
Changes of cuffitis
Neo terminal Ileum - normal.
Diagnosis:
Post-colectomy with IPAAPouchovaginal fistula s/p repair with Martius (labial) flap
Perianal fistula
Recurrent oral ulceration ?Oral Crohn’s
Pouchitis (PDAI – 10)
The course of illness:
The patient was initiated on a course of antibiotics (ciprofloxacin and metronidazole) after 8 weeks of antibiotics there was no improvement in disease activity (PDAI-8)
Given persistent symptoms on antibiotics, development of fistula, and persistent oral ulceration, a “Crohn’s like disease of pouch” was suspected. Hence a decision was taken to start the patient on Infliximab.
May 2024
· After two doses of infliximab, there was an improvement in symptoms of pouchitis, but the patient again presented with Odynophagia.
· Oral Examination showed persistent oral ulcers involving the buccal mucosa, soft and hard palate.
· An Upper GI Endoscopy showed the presence of erosions throughout the oesophagus, more evident in the lower and middle 1/3rd and the mucosa was easily sloughing off from the submucosal layer. (Figure 2) The stomach and duodenum were normal. With a suspicion of viral esophagitis, upper GI CD. A biopsy was taken from the upper, mid and lower third of the oesophagus.
· Because of painful ulceration in the background of immune suppression, oral candidiasis was suspected and the patient was started on fluconazole for 2 weeks, without response.
· The initial Biopsy of the Esophagus showed focal areas of stripping of mucosa from the basal layer and breaks in oesophagal mucosa with no evidence of inflammation or inclusion bodies. (Figure 3)
Workup for recurrent painful oral ulceration:
· Tissue for HSV, CMV PCR – Negative
· Oral swab for fungal c/s – Negative
· Autoimmune markers: ANA (IF) – Negative, dsDNA – Negative.
· Anti Desmoglein 1 and 3 antibodies: negative
· Pathergy test – Negative
· Procalcitonin –negative
· ESR-21
With no response to infliximab and antifungal and persistent odynophagia, the patient was initiated on oral steroid (prednisolone) 40mg.
1 week: post initiation of steroid, the patient has a reduction in dysphagia. Was able to tolerate oral liquids.
2 weeks: Repeat Upper GI endoscopy was done which showed no erosions in the upper and mid oesophagus and ongoing healing in the lower one-third of the oesophagus. (Figure 4) There was a significant reduction in oral ulcerations.
Final Diagnosis:
Post-colectomy with IPAAPouchovaginal fistula s/p repair with Martius (labial) flap
Perianal fistula
Chronic Antibiotic refractory pouchitis
Recurrent oral ulceration
Esophagitis desiccans superficialis.
s/p 2 doses of Remicade
On Tapering doses of prednisolone.