September 12, 2011 Eu Ro PE aN JouR Nal of MEd I cal RE SEaRcH 411 Eur J Med Res (2011) 16: 411-414 © I. Holzapfel Publishers 2011 SuccESSful NoN-INvaSIvE TREaTMENT of STRIcTuRINg fIbRoSINg coloNoPaTHy IN aN adulT PaTIENT g. Terheggen1, d. dieninghoff 2, E. Rietschel2, u. drebber3, W. Kruis4, l. leifeld4 1department of Medicine, Evangelisches Krankenhaus duesseldorf, duesseldorf, germany 2cf-center, children's Hospital, university of cologne, cologne, germany 3Institute of Pathology, university of cologne, cologne, germany 4department of Internal Medicine, Evangelisches Krankenhaus Kalk, cologne, germany Abstract the right-lower quadrant and normal bowel sounds. Objective: fibrosing colonopathy (fc) is a rare entity laboratory values showed an elevated c-reactive pro- associated with cystic fibrosis (cf). until now, patients tein (10.7 mg/dl) and a slight anemia (hemoglobin: with stricturing fc have usually been treated surgically. 10.6 g/dl). Transabdominal ultrasound demonstrated In this instance, we aimed at avoiding surgery by ap- hypoechoic bowel wall thickness of the transverse plying a new conservative approach. colon (10mm) and a 30mm long stricture in the right Methods: case report on an adult with cf who devel- transverse colon (fig. 1). The bowel wall of the as- oped persistent abdominal pain due to a non-passable cending colon and cecum was mildly thickened, while stricture in the right transverse colon. Histology con- no abnormalities could be observed in the terminal firmed fibrosing colonopathy. ileum. colonoscopy was incomplete because of a non- Results: Initially we treated the patient with pred- passable circular stricture in the right transverse colon nisolone pulse therapy and additive antibiotic therapy. (fig. 1). distal of the stricture the colonic mucosa ap- for maintenance therapy we administered budesonide. peared hyperemic, edematous and ulcerated for about The patient underwent clinical, laboratory and endo- 20cm. The remaining left-sided colon did not show scopic follow-up over a three-year period. The stric- any abnormalities. The histopathologic examination of ture healed and was easy to pass. a relapse in the ce- the endoscopic biopsies showed ulcerative inflamma- cum at the ileocecal valve again improved under tion, cryptitis, irregular hyperplasia of goblet cells, steroid and antibiotic therapy. submucosal fibrosis and disruption of the muscularis Conclusions: We present a novel therapeutic approach mucosa consistent with the diagnosis of fibrosing for advanced stricturing fc in an adult patient which colonopathy (fig. 1). after the patient had given his successfully avoided surgery (right hemicolectomy) informed consent with regard to the experimental over a three year follow up. character of the therapy we initiated intravenous pred- nisolone pulse therapy (75mg/d) for 10 days followed Key words: fibrosing colonopathy; cystic fibrosis; by a slow dose tapering (10mg/week to 25mg, than colonic stricture 5mg/week) over 10 weeks. In addition, we adminis- tered intravenous antibiotic therapy (mezlocillin 6g/d, caSE REPoRT metronidazole 1g/d). The patient`s symptoms im- proved significantly and c-reactive protein levels de- a 25-year-old man suffering from pre-existing cystic creased. after 3 weeks control-colonoscopy showed fibrosis was admitted with persistent pain in the right healed ulcerations. furthermore, the stricture had lower-quadrant. He reported a history of meconium healed with some minor scars remaining and could ileus at birth, appendectomy and recurrent distal in- now be passed allowing a view on the ascending colon testinal obstruction syndrome (dIoS). His genotype and cecum. Here we found multiple pseudopolyps and (compound heterozygote Δf508/R553X) is associated a lack of haustration while the terminal ileum ap- with pancreatic insufficiency [1]. Throughout child- peared normal. Histologically, fc with residual inflam- hood he was substituted with high-strength pancreatic mation was confirmed. for maintenance therapy we enzymes (pancreatin 150.000 units/d). during adoles- administered budesonide 9mg/d. Half a year later the cence he took pancreatin 10.000 units for fat-rich nu- patient presented without any abdominal pain and in- trition only. on his first presentation in July 2007, the creasing body weight. He had further decreased the patient reported about a 20-month history of recur- dosage of budesonide to 6mg/d by himself. colono - rent pain in the right lower abdominal quadrant which scopy showed an unchanged status. Therapy with now had increased dramatically. under intake of laxa- budesonide 6mg/d was continued. tives he had had daily bowel movements without visi- However, in January 2009, 18 months after his first ble blood. on admission he appeared in good physical admission, the patient once more presented with condition with a stable body weight (bMI 22.2 kg/m²) crampy pain in the right lower abdominal quadrant. and physical examination revealed focal tenderness in While the region of the former stricture in the right 412 EuRoPEaN JouRNal of MEdIcal RESEaRcH September 12, 2011 Prior to treatment 07/2007 after treatment 03/2011 abdominal ultrasound Hypoechoic bowel wall thickening (10mm) of the transverse colon, unremarkable bowel wall of the ascending colon and a stricture of nearly 30mm length colonoscopy Non-passable circular stricture in the right Scarredly healed and easily passable stric- transverse colon with ulcerative inflamma- ture in the right transverse colon tion Histology colonic biopsy with hypercellular lamina colonic biopsy with regenerative epitheli- propria with lymphocytes, eosinophilic um, mucosal edema and lymphocytic infil- granulocytes, ulcerative inflammation, ar- trate in the lamina propria chitectural distortion of the crypts and sub- mucosal fibrosis Fig. 1. transverse colon was unremarkable, we now found ar- treated the patient with prednisolone pulse therapy eas of ulcerative inflammation in the cecum and at the and antibiotics with again good clinical response and ileocecal valve which could not be passed due to in- administered budesonide 9mg/d for maintenance flammatory stenosis. as during the first episode we therapy. In July 2009, colonoscopy demonstrated that September 12, 2011 EuRoPEaN JouRNal of MEdIcal RESEaRcH 413 the stricture in the right transverse colon was now clinical symptoms from this stenosis should accelerate healed and easy to pass. In the cecum multiple in the future, balloon dilatation of the ileocecal valve pseudopolyps without additional inflammation were or a limited surgical intervention might be the treat- found. The ileocecal valve was constricted over a very ment of choice. analogue to crohn´s disease mainte- short distance and nearly passable with the colono- nance therapy with thiopurins might be another exper- scope. imental option. However, until now we avoided the finally after more than three years, in March 2011 risk of long-term immunosuppression due to the fear the patient presented in an excellent physical condition of bronchopulmonary infections in cf. body weight with stable weight under maintenance therapy. The (bMI 25kg/m²) and physical condition improved un- colonoscopy showed an unchanged status (fig. 1). der therapy with budesonide. Interestingly, lung func- tion improved remarkably under therapy demonstrat- dIScuSSIoN ing an increase of forced expiratory volume in one second (fEv1) from 67% to 93% which may point to fibrosing colonopathy is a rare entity associated to pa- a relationship between the inflammatory status of the tients with cf. It has generally been reported in young bowel and pulmonary affection in cf. according to children approximately 12 months after exposure to the current guidelines we recommended a pancreatic high dose pancreas enzyme supplementation (HdPE). enzyme supplementation on demand which should The exact etiopathogenesis still remains unsolved. Pre- not exceed 10.000 units of lipase per kg per day [14, disposing factors are young age (2-13 years), history of 15]. gastrointestinal complications (dIoS, meconium In conclusion, this case demonstrates a non-pass- ileus), previous intestinal surgery, HdPE and use of able colonic stricture in fc which was discovered en- histamine H2-receptor blockers, corticosteroids or re- doscopically and was successfully treated conservative- combinant human deoxyribonuclease (dNase) [2]. To ly over a three-year period. This suggests a new thera- our knowledge, only five cases of fc in adults were peutic approach in cf patients with stricturing fc. reported [3-7]: three patients with cf, one developed fc after HdPE in pancreatic insufficiency and one Conflict of interest: for all authors no conflicts of interest suspicious of crohn`s disease and treated with exist. mesalazine. Interestingly, development of fc in these five adults was not clearly associated with HdPE: two REfERENcES patients developed fc under treatment with HdPE, 1. The cystic fibrosis genotype-Phenotype consortium. one patient after discontinuation of HdPE and two correlation between genotype and phenotype in patients without HdPE at any time. all of these patients were with cystic fibrosis. N Engl J Med. 1993;329:1308-13 symptomatic due to strictures and the diagnosis of fc 2. fitzSimmons Sc, burkhart ga, borowitz d, et al. High- was discovered during surgery. dose pancreatic enzyme supplements and fibrosing clinical symptoms are usually those of intestinal colonopathy in children with cystic fibrosis. N Engl J obstruction with abdominal pain, constipation