REVIEW Upper gastrointestinal tract Crohn's disease H UG H) FREEMAN. MD NY SITE WITHIN THI:: GASTROINTEST ABSTRACT: Crohn's disease may involve any site within the gastrointestinal inal tract may involved in Crohn's tract. Usually pathology is present in the ileum and/or colon, but atypical presen­ A be tations may occur with apparently 'isolated' involvement of the oropharynx, esoph­ disease (I) lt is well recognized that agus or gastroduodenum. lf changes typical of Crohn 's disease are detected in the Ueal and/or colonic involvement arc most upper gastrointestinal tract, then a careful assessment is required involving often present, but occasionally 'isolated' radiographic, endoscopic and histologic studies to determine if pathology is present disease locali zed to o ne or more sites in in more distal intestine. In addition, microbiologic studies may be important to the upper gastrointestinal tract occurs. exclude infectious causes, especially of granulomas. If these studies are negative, Indeed , if involvement with Crohn'sd1s­ prolonge; follow-up may be required to establish a diagnosis of Crohn's disease. ease is defined by the presence of subtle Although upper gastrointestinal involvement is increasingly recognized as a sig­ endoscopic changes including aph thoid nificant cause of morbidity in Crohn's disease, the treatment options arc limited, ulcera tions or histologic fe atures alone, largely anecdotal and need to be the subject of detailed cpidemiologic investiga­ rhe stomach and duodenum appear to tion and clinical trials. Can J Gastroenterol 1990;4(1):26-32 be relati vely common sites of Crohn's Key Words: Crohn 's disease, Inflammatory bowel disease , Gastrocolic fiscula . Gran­ disease. C hanges in the esophagus have ulomacous gastritis, Ycrsinia entcrocolitica also been recorded but appear to be less Maladie de Crohn des voies digestives superieures frequen t. lf'isolated ' granulomatous dis­ ease is documented , however, carefu l ex· RESUME: La maladie de Crohn peut etre localisee a un niveau quelconque du clusion of other ca uses of granuloma is tube digestif. Habituellement, elle affecte l'ileon et/ou le colon mais elle peut se required. Generally, this should include manifester de far,:on atypique ct attei nd re l'oropharynx, l'esophage ou le gastro­ an evaluation of the distal small and large duodenum. Si certains changements attribuables ala maladie de Crohn sont de­ bowel to determine if the more typical celes clans la voie digestive superieure, ii faut alors proceder a une evaluation radiographic, endoscopic or histologic soigneuse comprenant des exam ens radiographiques. endoscopiques et histologi­ features of C rohn 's disease are present. ques destines a determiner si la maladie s'est propagee a d'autres segments de l'intestin. De plus, ii peut etre important de proceder ades cxamens microbiolo­ giques afin d'exclure !es origines infecrieuscs - dans le cas des granulomes sur­ GASTRODUODENAL DISEASE tout. Si les resultats sont ncgatifs. un suivi prolonge peut etre necessaire avant Frequency: Some of the earliest re· que le diagnostic definitif de maladie de Crohn ne soit pose. Bien que la partici­ ports recognizing gastric involve ment in pation de la voie digestive superieure soit de plus en plus reconnue com me une Croh n's disease were recorded by Ross cause importante de morbidite dans la maladie de Crohn. les options de traite­ (2) in J949 andComfort etal(3)in 1950. ment sont limitees et largement anecdotiques. Elles meriteraient de faire l'objet Since then , well over 100 patients have d'investigations epidemiologiques detaillees et d 'essais cliniques. heen reported with granulomatous in- ammatory disease of the stomach and Deparrment of Medic111e (Gastroenterology), University Hospiwl; and University of British duodenum attributed to C rohn's disease. Columbia. Vancouver, British Col1m1bia As the majority of these reports have Corres/Jondence and reprints. Dr Hugh Freeman, Head, Gas rroemerology, ACU F-137, University Hospiwl. Univers icy oj Bri rish Colambia, 221I Wesbrook Mall, Vancouver. Br1tish focused largely on clinically significant Columbia V6T IW5. Telephone/604) 228-7216 or 7235 involvement, estimated to be approx1· Received for puhlicanon June I9. J989. Accepted Se/ncmber I9. 1989 rnately 2 to 4% of all patients (4 ). the true 26 CAN J GASTROENTEROL VOL 4 No I JANUARY/FEBRUARY 1990 Crohn's disease frequency is likely to be much higher. tolog1cal features of C ro hn's disease. In entity may represent an early stage of Indeed. initial reports suggested that the addition to blood loss, however. anemia the disease. prevalence of patients with documented may result from deficits of iron or folic ln patients with duodenal disease, a Crohn's disease e lsewhere in the gastro­ acid due in part to reduced absorption concomitant inflammatory change in the intestinal tract was no more than 7°{, from a diseased proximal small intestine. pancreas or biliary tract may be present. (5-7). Subsequent studies. however, in­ 'Isolated' gastric disease: In some In this setting, bi liary tract stones or dicate that disease was r resent in the patients, gastric involvement alone may sludge, as well as the reflux of duodenal upper gastrointestina l tract in over 20% occur ( 13.18). In these patients, pain may content into the pancreatic duct through (8.9). This reflects, in part, an increased be a prominent sy mptom and although an incompetent ampulla. have been de­ appreciation in recent years for the wide­ no obstructio n is evident, abnormal gas­ scribed (29- 31 ). ln others, drug induced spread, often segmental or focal nature tric emptying may still occur. Diagnosis disease o f the pancreas has been re­ of this inflammatory p rocess within the may be suggested by nuclear scans or by ported, especially with sulfasalazine (32). gastrointestinal tract. Moreover, the com­ barium studies showing impaired e mp­ metronidazole ( 33) and immunosup­ bined use of upper e ndoscop y and tying of contrast; in the most extre me pressive agents. including azathioprine mucosa! biopsy has increased the incidence situations, a poorly distensible gastric an­ and 6-mercaptopurine (34-36). Alcohol of recognition, even if the disease is clin­ trum. sometimes with aphthoid ulcer­ associated injury can also occur in these ically or radiographically 'silent' at this site. ation and 'cobblestoning' of antral folds patients. More recently. direct ampullary Clinical features: In ma ny patients, with fissuring, ulceration and pyloric de­ involvement with stenosis as well as com­ symptoms relating to the upper gastro­ fo rmity may be present. In one report. mon bile duct obstruction have been re­ intestinal tract may be limited or fre­ the barium radiographic 'ram's horn' sign corded (37). ln addition, duodenal bezoar quently overshadowed hy diarrhea and was described ( 19). In these patie nts, formation (38). and fistulous communi­ other symptoms associated with small or Crohn 's disease may later become evi­ cati on with the pancreatic duct have large intestinal disease. Even significant dent in the more distal small and la rge been described ( 39). symptoms relating to resistant ulceration intestine. Endoscopic features: Endoscopy may and progessive gastroduodenal stenosis 'Isolated' duodenal disease: Isolated in­ permit a better appreciation o f the may go unrecognized until later in the volvement of the proximal small intes­ cha nges associated with Crohn's disease clinical course. ln this setting, marked tine including the duodenum may also in the upper gastrointestinal tract, even nutritional disturbance and weight lo:,s occur (20-24). Most often evidence of ifir is apparently asymptomatic (8,40-45). are cor.,mon. in part reflecting the pro­ such involvement is limited to focal in­ Gastric and duodenal ulceration, espe­ gressive impairment of food intake and flammatory changes or isolated muco­ ciall y linear or serpiginous, may be ob­ impaired gastric emptying of nutrients sa! granulomas, with multinucleated served. The antrum may be poorly dis­ into the small intestine. In some patients, giant cells or loose aggregates and clus­ tensible with ai r insufflation, and rigid the clinical features of anorexia nervosa ters of cpithe lioid cells. In othe rs. more pyloric or pyloroduodenal stenosis may may be present ( 10.11 ). Symptoms re­ severe changes may be present with mul­ prohibit passage of even 'pediatric­ lated to gastric outlet obstruction such tiple and diffuse ste notic small bowel calibre' endoscopes. Macroscopic changes as nausea and vomiting may result. Be­ segments. Such patients are prone to may also include a minimal colour alter­ cause of the cicatrizing nature of this in­ episodes of bowel obstruction, generalized ation, either patchy or diffuse. focal white flammatory process in the antral and py­ malnutrition and numerous specific nodules, a ph thoid u Ice ration or erosion loric region, the proximal stomach may nutrient deficiencies. The frequency of with exudate and apparent mucosa] 'cob­ appear dila ted with retained food more clinically significant, extensive small blestoning'. Strictures and fistulae from debris and sometimes bezoar formation. bowel disease is not known. In one early the stomach or duode num into nearby Hemorrhage. especiall y from deep study (25). based largely on contrast intestinal loops or the pancreatobiliary ulceration. has been possibly incorrectly radiographic examinations. 18 of 330 tract may be appreciated. More often, considered an uncommon presentation patie nts with C rohn 's disease seen from with the development of fi stulae, a co­ for most granulomatous gastric disorders 1944 to 1970 were described as having existent inflammatory focus is present 112); in part, this idea may reflect a ten­ more than diffuse jejunaI involvement. in an adjacent loop o f small or large dency to attribute the cause of ulceration The early lite rature on Crohn's disease bowel - in this setting, the fistula is to 'acid peptic' disease or commonly pre­ (26-28) also describes the laparotomy ap­ usually considered 'secondary' to the in­ scribed analgesics used to m a n age pearances of the small intestine in rare testinal disease (46-54).