Successful Non-Invasive treatment of stricturing fibrosing colonopathy in an adult patient
© I. Holzapfel Publishers 2011
Received: 1 April 2011
Accepted: 17 May 2011
Published: 12 September 2011
Fibrosing colonopathy (FC) is a rare entity associated with cystic fibrosis (CF). Until now, patients with stricturing FC have usually been treated surgically. In this instance, we aimed at avoiding surgery by applying a new conservative approach.
Case report on an adult with CF who developed persistent abdominal pain due to a non-passable stricture in the right transverse colon. Histology confirmed fibrosing colonopathy.
Initially we treated the patient with prednisolone pulse therapy and additive antibiotic therapy. For maintenance therapy we administered budesonide. The patient underwent clinical, laboratory and endoscopic follow-up over a three-year period. The stricture healed and was easy to pass. A relapse in the cecum at the ileocecal valve again improved under steroid and antibiotic therapy.
We present a novel therapeutic approach for advanced stricturing FC in an adult patient which successfully avoided surgery (right hemicolectomy) over a three year follow up.
Keywordsfibrosing colonopathy cystic fibrosis colonic stricture
However, in January 2009, 18 months after his first admission, the patient once more presented with crampy pain in the right lower abdominal quadrant. While the region of the former stricture in the right transverse colon was unremarkable, we now found areas of ulcerative inflammation in the cecum and at the ileocecal valve which could not be passed due to inflammatory stenosis. As during the first episode we treated the patient with prednisolone pulse therapy and antibiotics with again good clinical response and administered budesonide 9 mg/d for maintenance therapy. In July 2009, colonoscopy demonstrated that the stricture in the right transverse colon was now healed and easy to pass. In the cecum multiple pseudopolyps without additional inflammation were found. The ileocecal valve was constricted over a very short distance and nearly passable with the colonoscope.
Finally after more than three years, in March 2011 the patient presented in an excellent physical condition with stable weight under maintenance therapy. The colonoscopy showed an unchanged status (Figure 1).
Fibrosing colonopathy is a rare entity associated to patients with CF. It has generally been reported in young children approximately 12 months after exposure to high dose pancreas enzyme supplementation (HDPE). The exact etiopathogenesis still remains unsolved. Predisposing factors are young age (2-13 years), history of gastrointestinal complications (DIOS, meconium ileus), previous intestinal surgery, HDPE and use of histamine H2-receptor blockers, corticosteroids or recombinant human deoxyribonuclease (DNase) . To our knowledge, only five cases of FC in adults were reported [3–7]: three patients with CF, one developed FC after HDPE in pancreatic insufficiency and one suspicious of Crohn's disease and treated with mesalazine. Interestingly, development of FC in these five adults was not clearly associated with HDPE: two patients developed FC under treatment with HDPE, one patient after discontinuation of HDPE and two without HDPE at any time. All of these patients were symptomatic due to strictures and the diagnosis of FC was discovered during surgery.
Clinical symptoms are usually those of intestinal obstruction with abdominal pain, constipation or diarrhea and hematochezia. Abnormal bowel wall thickness is a common finding in CF, but it is usually not associated with clinical features and is no predisposing factor for FC [8, 9]. Endoscopy may demonstrate a friable, hyperemic, edematous, stiff or ulcerated mucosa often with strictures, colonic shortening and loss of haustration. FC usually spares the rectum and may be either segmental, mostly in the right colon, or involves the complete colon. Characteristic histologic features include submucosal fibrosis, disruption of the muscularis mucosa, ulcerative inflammation, eosinophilia and cryptitis [10–13]. In contrast, in ulcerative colitis common features are an acute and chronic inflammatory cell infiltrate, distortion of crypts, crypt abscesses and goblet cell depletion. The differentiation to stricturing Crohn's disease can be challenging in the absence of characteristical epitheloid granuloma.
To our knowledge this is the first report of a nonpassable colonic stricture in FC which was successfully treated without surgical intervention. Treatment was based on an antiinflammatory concept with prednisolone pulse therapy. Additional antibiotic therapy aimed at treating bacterial translocation due to barrier dysfunction of the intestinal mucosa. In order to avoid side effects of longterm systemic steroids treatment was continued with topical active budesonide. However, maintenance therapy with budesonide did not protect the patient from a second stenosis at the cecum. If clinical symptoms from this stenosis should accelerate in the future, balloon dilatation of the ileocecal valve or a limited surgical intervention might be the treatment of choice. Analogue to Crohn's disease maintenance therapy with thiopurins might be another experimental option. However, until now we avoided the risk of long-term immunosuppression due to the fear of bronchopulmonary infections in CF. Body weight (BMI 25 kg/m2) and physical condition improved under therapy with budesonide. Interestingly, lung function improved remarkably under therapy demonstrating an increase of forced expiratory volume in one second (FEV1) from 67% to 93% which may point to a relationship between the inflammatory status of the bowel and pulmonary affection in CF. According to the current guidelines we recommended a pancreatic enzyme supplementation on demand which should not exceed 10.000 units of lipase per kg per day [14, 15].
In conclusion, this case demonstrates a non-passable colonic stricture in FC which was discovered endoscopically and was successfully treated conservatively over a three-year period. This suggests a new therapeutic approach in CF patients with stricturing FC.
Conflict of interests
The authors declare that they have no competing interests.
- The Cystic Fibrosis Genotype-Phenotype Consortium. Correlation between genotype and phenotype in patients with cystic fibrosis N Engl J Med 1993, 329: 1308–13.
- FitzSimmons SC, Burkhart GA, Borowitz D, et al.: Highdose pancreatic enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med 1997, 336: 1283–9. 10.1056/NEJM199705013361803PubMedView Article
- Häusler M, Meilicke R, Biesterfeld S, Heimann G: First adult patient with fibrosing colonopathy. Am J Gastroenterol 1998, 93: 1171–2.PubMedView Article
- Mack EH, Brett AS, Brown D: Fibrosing colonopathy in an adult cystic fibrosis patient after discontinuing pancreatic enzyme therapy. South Med J 2004, 97: 901–4. 10.1097/01.SMJ.0000129939.14179.FEPubMedView Article
- Franzen D, Went P, Buhlmann U: Fibrosing colonopathy in absence of pancreatic enzyme supplementation in one adult patient with cystic fibrosis. Indian J Gastroenterol 2008, 27: 133–4.PubMed
- Bansi DS, Price A, Russell C, Sarner M: Fibrosing colonopathy in an adult owing to over use of pancreatic enzyme supplements. Gut 2000, 46: 283–5. 10.1136/gut.46.2.283PubMed CentralPubMedView Article
- Gaia E, Sambatoro A, De Giuli P, Angeli A: Adult fibrosing colonopathy associated with mesalazine treatment. Am J Gastroenterol 2001, 96: 2508–9.PubMedView Article
- Haber HP, Benda N, Fitzke G, et al.: Colonic wall thickness measured by ultrasound: striking differences in patients with cystic fibrosis versus healthy controls. Gut 1997, 40: 406–11.PubMed CentralPubMedView Article
- Dialer I, Hundt C, Bertele-Harms RM, Harms HK: Sonographic evaluation of bowel wall thickness in patients with cystic fibrosis. J Clin Gastroenterol 2003, 37: 55–60. 10.1097/00004836-200307000-00014PubMedView Article
- Reichard KW, Vinocur CD, Franco M, et al.: Fibrosing colonopathy in children with cystic fibrosis. J Pediatr Surg 1997, 32: 237–41. 10.1016/S0022-3468(97)90186-XPubMedView Article
- Schwarzenberg SJ, Wielinski CL, Shamieh I, et al.: Cystic fibrosis-associated colitis and fibrosing colonopathy. J Pediatr 1995, 127: 565–70. 10.1016/S0022-3476(95)70113-3PubMedView Article
- Dodge JA: The aetiology of fibrosing colonopathy. Postgrad Med J 1996,72(suppl 2):S52–5.PubMed
- Lloyd-Still JD, Beno DW, Kimura RM: Cystic fibrosis colonopathy. Curr Gastroenterol Rep 1999, 1: 231–7. 10.1007/s11894-999-0040-4PubMedView Article
- Lloyd-Still JD: Cystic fibrosis and colonic strictures. A new "iatrogenic" disease. J Clin Gastroenterol 1995, 21: 2–5. 10.1097/00004836-199507000-00001PubMedView Article
- Stevens JC, Maguiness KM, Hollingsworth J, et al.: Pancreatic enzyme supplementation in cystic fibrosis patients before and after fibrosing colonopathy. J Pediatr Gastroenterol Nutr 1998, 26: 80–4. 10.1097/00005176-199801000-00014PubMedView Article