Severe stricturing Crohn's disease of the duodenum: A case report and review of surgical options

Abstract

INTRODUCTION

Duodenal Crohn's disease is a rare clinical entity that occurs in 0.5–4.0% of patients with Crohn's disease. A unique case of Crohn's disease of the upper gastrointestinal tract characterized by multiple strictures within the duodenum and jejunum is described in our review.

PRESENTATION OF CASE

A 41-year-old male presented with a 2-month history of intermittent, crampy abdominal pain accompanied by nausea, bilious emesis, early satiety, anorexia and weight loss. Physical examination revealed fullness in the epigastric region. Imaging demonstrated strictures in the proximal and distal duodenum with dilatation of the intervening segments. There was also gross dilatation of the proximal jejunum, which was followed by a 9 cm strictured segment. There was no evidence of acute Crohn's disease. Although a Whipple's resection was initially considered as a form of operative intervention given the extent of disease within the duodenum, the discovery of unexpected disease intra-operatively presented a surgical dilemma. In this case, strictureplasty, surgical resection and bypass were used to treat the patient.

DISCUSSION

Diffuse stricturing of the proximal gastrointestinal tract is a rare manifestation of Crohn's disease. Although imaging can aid in surgical planning, intra-operative decision-making to deal with unexpected findings will remain an important aspect of the management of this entity.

CONCLUSION

The fundamental goal of the surgical management of strictures secondary to Crohn's disease is to relieve obstruction while maximizing bowel conservation. A variety of operative techniques are currently described for the management of duodenal Crohn's disease and are reviewed in this case report.

1. Introduction

Although Crohn's disease can involve any part of the alimentary tract, involvement of the duodenum is a rare clinical entity that occurs in only 0.5–4.0% of patients, with severe stricturing an even less common occurrence.^1–3 This report describes a 41-year-old male with Crohn's disease of the upper gastrointestinal tract characterized by multiple strictures within the duodenum and proximal jejunum. The surgical options for management of complicated duodenal Crohn's disease include resection, bypass or strictureplasty. In this case all three modalities were utilized to relieve the obstruction.

2. Case

A 41-year-old male with a 10-year history of Crohn's disease presented to the Emergency Department with a 2-month history of intermittent, diffuse, crampy abdominal pain accompanied by progressive nausea, bilious emesis, early satiety, anorexia and weight loss of approximately 3.6 kg. He was initially diagnosed with duodenal Crohn's disease by abdominal X-ray and esophagogastroduodenoscopy with biopsies that demonstrated active Crohn's disease. At that time he was started on prednisone treatment and had a dramatic symptomatic improvement. However, after approximately 8 years of maintenance steroid therapy, he developed severe osteopenia/osteoporosis (Z score: −2.7); prednisone was subsequently discontinued and maintenance therapy with Infliximab was initiated. He noticed a significant symptomatic improvement following the Infliximab infusions and did quite well for approximately 2 years prior to presenting to the emergency department on this occasion.

Physical examination demonstrated fullness in the epigastric region. Routine bloodwork revealed a mild normocytic anemia (Hgb 121 g/L, normal range: 135–170 g/L), but was otherwise unremarkable. He subsequently underwent an esophagogastroduodenoscopy (EGD), computerized axial tomography (CAT) of the abdomen and pelvis and an upper gastrointestinal (UGI) series.

EGD revealed inflammation of the duodenal cap and a tight stricture (<10 mm) between the duodenal cap and the second portion of the duodenum preventing further advancement of the gastroscope. Multiple biopsies taken of the stricture and surrounding mucosa revealed focal reactive changes but no evidence of active disease. CAT revealed strictures in both the proximal and distal portions of the duodenum with dilatation of the intervening segments. There was also gross dilatation of the proximal jejunum immediately followed by a 9 cm strictured segment of the mid-jejunum (Fig. 1a and b). UGI series demonstrated strictures within the 2nd, 3rd and 4th portions of the duodenum and marked dilation of the proximal jejunum with tapering at the distal margin (Fig. 2a and b).

Fig. 1.

(a) Coronal image from CT scan demonstrating strictures within the 1st and 3rd portions of the duodenum (arrows) with dilatation of the intervening segment; there is also evidence of gross dilatation of the jejunum (asterisk) secondary to a distal stricture (block arrow). (b) Axial image from CT scan demonstrating strictures within the 3rd and 4th portions of the duodenum (arrow) with dilatation of the 2nd portion of the duodenum and the proximal jejunum (asterisk).

Fig. 2.

(a) Upper gastrointestinal series demonstrating strictures within the 2nd, 3rd and 4th portions of the duodenum (arrows); also note the dilatation within the duodenal bulb, 2nd portion of the duodenum and jejunum. (b) Upper gastrointestinal series demonstrating severe dilatation of the 2nd portion of the duodenum and proximal jejunum (arrows), with stricturing of the intervening segments. Also note the stricture in the proximal ileum just distal to the dilated region (block arrow).

Total parenteral nutrition (TPN) was initiated and surgical consultation was sought. He responded extremely well to TPN therapy over a period of 13 days with a profound increase in both his albumin (26–33 g/L, normal range: 35–52 g/L) and pre-albumin (0.17–0.36 g/L, normal range: 0.18–0.45 g/L) levels. He was then taken to the operating room for surgical management of his severe stricturing disease of the upper gastrointestinal tract. Pre-operatively it was felt that the patient may require a pancreaticoduodenectomy to treat his disease; however, at the time of surgery, there was more extensive disease than originally thought. As a result, a small bowel resection (including the 2nd portion of the duodenum distal to the ampulla of Vater, 3rd and 4th portions of the duodenum and proximal jejunum), duodenojejunostomy and retro-colic gastrojejunostomy were performed. The duodenojejunostomy permitted drainage of the second portion of the duodenum; however, because there was still a more proximal stricture we thought it would be best to fashion a separate gastrojejunostomy. By doing so we avoided placing two anastamoses in close proximity to each other in the duodenum. An additional stricture was identified in the mid-ileum which was treated with strictureplasty (Fig. 3a and b). The patient tolerated the procedure well and was discharged home on post-operative day ten; he had a rapid return to normal function and was noted to be tolerating a full diet at his 6-month follow-up with a weight gain of 11.8 kg.

Fig. 3.

(a) Intra-operative photograph demonstrating massively dilated proximal jejunum. (b) Intra-operative photograph demonstrating additional jejunal stricture.

3. Discussion

Crohn's disease is a chronic intestinal disorder of multi-factorial etiology that can cause varying symptoms and complications. Although Crohn's disease can occur anywhere within the alimentary tract, involvement of the duodenum is rare and severe stricturing of the duodenum is even less common. The first case of duodenal involvement was described by Gottlieb and Alpert in 1937 and since that time the prevalence has been reported to be between 0.5% and 4% of all patients with Crohn's disease.^1,4,5 Patients with duodenal Crohn's disease usually present with Crohn's disease affecting other areas of the gastrointestinal tract and thus the majority of cases occur in association with one another. In general, symptoms from duodenal Crohn's disease are initially managed with a combination of acid-reducing and immunosuppressive agents. A significant number of these patients however, eventually experience severe symptoms thereby necessitating surgical intervention. The most common indication for surgical intervention is progressive obstruction, but others include failure of medical management with intractable pain, bleeding, perforation and fistulous disease.^1,6,7

Options for surgical management of complicated duodenal Crohn's disease most commonly described in the literature include resection, bypass or strictureplasty. Surgical resection with procedures such as that described by Dr. Allen Whipple have been associated with significant morbidity and mortality in this setting. Therefore, bypass procedures and strictureplasty have become the preferred methods of surgical treatment. Complications associated with resection include short gut syndrome, diarrhea, chronic malnutrition, electrolyte derangements, vitamin B₁₂ and folate deficiencies and chronic anemia.⁸ In an effort to preserve the duodenum and prevent these complications, bypass procedures such as gastrojejunostomy, gastroduodenostomy and Roux-en-Y-gastrojejunostomy have become increasingly favored and are considered the standard surgical approach to operative management of this disease.

Strictureplasty was popularized by Lee and Papiaoannu in the 1970s and further refined by Alexander-Williams.^9,10 Although a variety of different strictureplasty techniques have been described, the two most common utilized for duodenal Crohn's disease are the Heineke-Mikulicz procedure for strictures less than 10 cm and the Finney strictureplasty for longer segments of disease, up to 15–25 cm in length.⁸ The durability, safety and efficacy of these techniques were firmly established in the early 1990s and thus became increasingly favored over bypass procedures. Despite this trend however, the operative procedure of choice should be dictated by the patient's presentation of disease and assessed on a case-by-case basis. This in turn requires detailed pre-operative imaging which is essential in order to plan an effective and safe surgical approach. Occult strictures and secondary involvement of the colon are common, and thus it is important to evaluate the entire small bowel and colon.⁸

Surgeons performing strictureplasty for the treatment of duodenal Crohn's disease were initially less enthusiastic due to the retroperitoneal location of the duodenum and its relationship to vascular and pancreatobiliary structures.¹¹ These intimate anatomic relationships necessitate extensive mobilization of the duodenum compared to that of a bypass procedure and may, in the face of an acute inflammatory process, be technically challenging. In contrast, bypass procedures, which are thought to be more technically feasible, are not without their own pitfalls. More specifically, they require mobilization of portions of small bowel which may be involved either by Crohn's disease or adhesions from previous surgery, and are more frequently associated with blind-loop syndrome, dumping, bile reflux gastritis, marginal ulceration and malignancy.¹¹

Given the rarity of duodenal Crohn's disease, only a limited number of studies addressing its surgical management have been published and little data describing the optimal treatment exists. Two studies highlighted in a recent meta-analysis however, compared outcomes after strictureplasty and bypass surgery for duodenal disease.

In the Birmingham study (Yamamoto et al., 1999) 13 patients underwent duodenal strictureplasty, 9 of which required further surgical intervention due to early post-operative complications (anastomotic leak with fistula, n = 2; persistent obstruction, n = 1) and restricture at the strictureplasty site (n = 6) noted at a median follow-up of 143 months. Similarly, 13 patients underwent bypass procedures. In this case, no patients required reoperation for early post-operative complications; however, 6 patients required further surgical intervention at a later date (median follow-up 192 months) for stomal ulceration (n = 2) and anastomotic obstruction (n = 4).¹² In the Cleveland Clinic study (Worsey et al., 1999), strictureplasty was performed in 13 patients, 1 of which developed an early post-operative complication (enterocutaneous fistula) and 1 which required reoperation for recurrence at a mean follow-up of 42 months. Furthermore, 21 patients underwent bypass procedures, 2 of which developed early post-operative complications (persistent obstruction and enterocutaneous fistula), and 1 which required further operation for recurrent disease at a mean follow-up of 96 months.¹¹

These two studies had conflicting conclusions. Worsey et al. concluded that duodenal strictureplasty is safe and effective and may in fact have potential anatomic and physiologic advantages over bypass procedures, whereas Yamamoto et al. showed that strictureplasty conferred no obvious advantages over bypass and was in fact associated with a higher incidence of early complications and repeat stricturing.^11,12 This difference, in part, may be explained by the longer follow-up period in the Birmingham study as compared to the Cleveland Clinic study as well as the anatomic limitations of the duodenum which make anastomotic closure technically difficult.¹³

Although strictureplasty has become an established surgical option in the management of obstructive Crohn's disease, the role of this technique in duodenal Crohn's disease is less well defined given the rarity of this clinical entity. The optimal management of duodenal Crohn's disease has not yet been clearly defined and therefore further studies are required to determine the best way to manage this challenging disease.

In our case report, failure of medical management with symptoms of progressive obstruction eventually led to surgical intervention. In this particular case, a Whipple's resection was initially considered given the extent of disease within the 2nd, 3rd and 4th portions of the duodenum. However, given the patient's nutritional state and performance status pre-operatively, we decided that the most appropriate option was a combination of surgical resection, bypass and strictureplasty. Collectively, this combination of procedures maximized conservation of bowel while permitting complete alleviation of symptoms, both of which are fundamental to the surgical treatment of Crohn's disease.

Conflict of interest

None reported.

Funding

None.

Ethical approval

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Author contributions

J.R. was primarily responsible for acquisition of data. J.R. and W.D. contributed to the analysis and interpretation of data, writing of the manuscript and reviewing the manuscript critically for important intellectual content and have both approved the final version to be published.