Crohn's Disease of the Esophagus
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MINI-REVIEW Crohn’s disease of the esophagus: Three cases and a literature review Ivan Rudolph MD, Franz Goldstein MD, Anthony J DiMarino Jr MD I Rudolph, F Goldstein, AJ DiMarino Jr. Crohn’s disease of Trois cas de maladie de Crohn de l'œsophage the esophagus: Three cases and a literature review. Can J Gastroenterol 2001;15(2):117-122. Three cases of esophageal et examen de la documentation Crohn’s disease (CD) are described, each with dysphagia and/or RÉSUMÉ : Voici trois cas de maladie de Crohn (MC) de l'œsophage odynophagia caused by esophageal ulceration. All three patients caractérisée par une dysphagie et/ou une odynophagie causée par une had associated ileocolitis. One patient followed for a prolonged ulcération œsophagienne. Les trois patients présentaient également une period responded to treatment with sulfasalazine and pred- iléo-colite. Le suivi prolongé d'un patient a montré que ce dernier avait nisone. A computer search back to 1967 produced 72 additional bien réagi au traitement à la sulfasalazine et à la prednisone. Une cases of esophageal CD. Among these 75 patients (total), who recherche informatisée des dossiers depuis 1967 a permis de relever were, on average, 34 years old, esophageal disease was the pre- 72 cas de MC de l'œsophage. Un trouble de l'œsophage a été le premier signe de la maladie chez 41 des 75 patients (55 %), âgés en moyenne de senting disease symptom in 41 patients (55%). The diagnosis 34 ans. Le diagnostic a été difficile à poser chez 13 d'entre eux étant don- was difficult in 13 patients, in whom no distal bowel disease was né qu'aucune maladie intestinale distale n'avait été décelée au moment detected at the time of initial esophageal presentation. The most de l'apparition du premier signe de trouble œsophagien. La manifesta- common presentation was dysphagia associated with aphthous tion la plus courante a été une dysphagie associée à une ulcération aph- or deeper ulcerations (52 patients). In 11 of these patients, oral teuse ou profonde (52 patients); onze souffraient également d'aphtes aphthous ulcerations were also present. Esophageal stenosis or buccaux. Vingt-sept patients présentaient une sténose œsophagienne ou fistulas to surrounding structures were present in 27 patients and des fistules et 17 d'entre eux ont dû être opérés. La plupart des résultats led to surgery in 17 patients. Most of the unfavourable outcomes négatifs, dont cinq morts, ont été observés dans le groupe de patients were in this group of 27 patients with esophageal complications, présentant des complications œsophagiennes. Quatorze autres patients including five deaths. Fourteen additional patients required sur- ont été opérés pour une MC touchant d'autres régions. Le traitement de gery for CD of other areas. Responses of uncomplicated ulcera- la forme non compliquée de l'ulcération œsophagienne semblait donner tive disease of the esophagus tended to be favourable if the de bons résultats si celui-ci comprenait de la prednisone. Les signes clin- iques de la MC de l'œsophage ont été divisés en trois catégories : ulcères, medical regimen included prednisone. Clinical patterns of sténose et absence de symptômes (maladie aiguë chez les enfants). esophageal CD were divided into three categories: ulcerative, stenosing and asymptomatic (acute disease in children). Key Words: Crohn’s disease; Esophageal disease he prevalence of esophageal involvement in Crohn’s were prospectively surveyed by means of esophagogastro- Tdisease (CD) has been estimated in the literature to be duodenoscopy (EGD) during the acute phase of CD (2). between 1% and 2% (1). However, the pediatric literature Case reports are typically brief, and not all questions can be has reported a prevalence of 20% to 40% when all patients answered by reviewing them. Three cases of esophageal CD This mini-review was prepared from a presentation made at the 1998 meeting of the Bockus International Society of Gastroenterology, Graz, Austria, August 31 to September 3, 1998 Division of Gastroenterology, Thomas Jefferson University, Philadelphia, Pennsylvania, USA Correspondence and reprints: Dr I Rudolph, Division of Gastroenterology and Hepatology, Thomas Jefferson University, 132 South 10th Street, 480 Main Building, Philadelphia, Pennsylvania 19107-5244, USA. Telephone 215-955-8900, fax 215-955-0872, e-mail [email protected] Received for publication July 6, 1999. Accepted July 12, 1999 Can J Gastroenterol Vol 15 No 2 February 2001 117 Rudolph et al Figure 1) Discrete small ulcerations in the distal esophagus of the Figure 2) Multiple aphthous ulcers in the mid- and distal esophagus of patient described in case 1. Markers indicate two ulcers the patient described in case 2 recently encountered in the authors’ practices are 1986 also showed antral gastritis, with biopsies confirming described, together with a review of the pertinent litera- nonspecific inflammation of the prepyloric area with ture. Helicobacter pylori positivity. Treatment with ampicillin seemed to help. Follow-up radiographs in 1989 showed par- CASE 1 tial improvement of the ileal changes. Repeat radiographs A 24-year-old married woman was seen in February 1998; obtained in 1998 showed minimal residual scarring of the her chief complaint was odynophagia. She had suffered terminal ileum. Treatment was initiated with mesalamine, from prolonged heartburn. Treatment with H2-receptor but the patient was lost to follow-up before the response to blockers, and later with proton pump inhibitors, produced treatment could be evaluated. only minor symptom relief. Subsequently, odynophagia The interpretation of events from 1986, 1989 and 1998 developed, brought on by swallowing any food or even sali- suggests that they were due to CD, and that the recent pre- va. Avoidance of spicy foods produced minimal benefit. dominant problem is esophageal CD with multiple ulcers Past medical history recalled by the patient included ‘func- and corresponding symptoms. tional’ gastrointestinal problems in 1986, when the patient was 12 years old. Unrelated events also recalled included CASE 2 bouts of cystitis, and asthma treated with oral prednisone A 28-year-old married woman presented in April, 1991 and an albuterol inhaler. On physical examination, the with three weeks of abdominal cramps and up to six watery patient appeared well developed and nourished, vital signs stools per day. Five days before her visit, she developed a were normal and general examination failed to show any sore throat, and her husband, who was a physician, noted abnormalities. Because of the odynophagia, EGD was per- aphthous ulcers in her mouth. The patient developed dys- formed. EGD revealed three distinct esophageal ulcers, phagia and odynophagia by the time of her evaluation. She each appearing as a sharply demarcated, red, approximately had fevers with temperatures of up to 102°C. Past medical 5 mm round area in the lower esophagus surrounded by history included uveitis treated at a university clinic with normal-appearing mucosa (Figure 1). The Z-line was dis- steroid eye drops. tinct without clinically apparent esophagitis or stricture. On examination, the patient’s blood pressure was The stomach and duodenum appeared normal. Biopsies 100/70 mmHg and her pulse was 112 beats/min. On the hard from the lower esophagus revealed dense lymphocytic infil- palate and buccal surfaces, multiple white papules – some trates in several areas and ulcerations in others. Granu- ulcerated – were noted. The remainder of the examination lomas were not found. was unremarkable, except for a grade 2 systolic murmur at the The patient’s mother recalled that her daughter had apex and mild, lower to mid-abdominal tenderness. On the been suspected of having CD in 1986. Records and x-rays EGD, multiple small aphthous ulcerations on slightly elevat- were retrieved. The patient had presented in 1986 with ed bases were noted, which were similar to the ones in the diarrhea and abdominal pain. Barium contrast studies of mouth and measured from 2 mm to 6 mm in diameter the gastrointestinal tract were reviewed and showed a dis- (Figure 2). Similar lesions were also found diffusely distrib- tinct segment of ileocolitis involving about 6 cm of the ter- uted in the stomach and proximal duodenum. The duodenal minal ileum and 3 cm of the adjacent cecum. The EGD in loop was normal. Biopsies and brushings taken from the 118 Can J Gastroenterol Vol 15 No 2 February 2001 Crohn’s disease of the esophagus ulcers failed to show viral inclusions but did show acute and the anal sphincteric region and was seen by his attending chronic superficial gastritis and esophagitis, with some gran- gastroenterologist; he was subsequently referred to a col- ulation tissue and scattered ulcerations. Flexible sigmoi- orectal surgeon. A fissure from the Crohn’s disease was not- doscopy, done at the same time, showed erythema and ed, and local care, incision and drainage were judiciously shallow irregular ulcerations scattered over the distal 35 cm. performed over the next several years. Biopsies revealed acute and chronic inflammation with crypt In 1985, an air contrast barium enema revealed scarring abscesses, consistent with inflammatory bowel disease. of the ileocecal valve with aphthous ulcerations consistent Granuloma formation in submucosal lymphoid aggregates with Crohn’s disease. was suggested. In 1986, the patient described dysphagia, particularly with The patient was treated with sulfasalazine 2 g/day and solid foods, and was evaluated by a gastroenterologist with prednisone 40 mg/day, followed by a dramatic response – upper endoscopy. Findings at that time revealed an exudative the disappearance of dysphagia within a few days and distal esophageal mucosa with multiple small ulcerations. improvement in the diarrhea. When the patient was seen in The amount of esophageal involvement was approximately the office three weeks later, the aphthous ulcerations of the 2 cm to 3 cm in length. He was treated with high dose H2- mouth were no longer seen, and she reported to be free of receptor antagonists without improvement.