Small Bowel Perforation as the Initial Presentation of Crohn’s Disease: A Case Report

B. Ravi Shankar1*, Akhil Konduru1, Vamshi Krishna Boddireddy1 , Prasad Babu TLVD2, B. Shruti Sagar 1

¹Department of Medical Gastroenterology, Yashoda Hospital, Secunderabad, India.
²Department of Surgical Gastroenterology, Yashoda Hospital, Secunderabad, India

*Corresponding author

B Ravi Shankar, Department of Medical Gastroenterology, Yashoda Hospital, Secunderabad, India.

Abstract

Perforation of bowel as the first presentation of inflammatory bowel disease is a rare occurrence. It is a serious event with most of the perforations occurring in ileum.1

This report describes a unique case of a previously asymptomatic 75-year-old woman with chronic pancreatitis presenting with intestinal perforation secondary to Crohn’s disease (CD).
She had crampy abdominal pain, bilious vomiting, fever and abdominal distention of 3 days duration. X-ray erect abdomen and abdominal CT scan were suggestive of pneumoperitoneum with peripherally enhancing collection, air pockets adjacent to small bowel and contrast leak into collection suggestive of perforation. The perforated segment was resected and primary anastomosis performed. Histopathology of the resected small bowel confirmed the diagnosis of Crohn’s disease. She was initiated on  subcutaneous Adalimumab a month after her surgery to prevent relapse of CD.

Keywords: IBD, Peritonitis, Adalimumab, Abdominal pain, Surgery.

Introduction

Crohn’s disease is a chronic inflammatory disease of the gastrointestinal tract and the incidence is increasing worldwide. Typical clinical scenarios are young patients with abdominal pain, chronic diarrhoea, weight loss, and fatigue2. Intestinal perforation as an initial complication of Crohn’s disease is a rare but serious event with most of the perforations occurring in ileum1.

We present the case of a 75-year-old woman who had Crohn's disease and presented as intestinal perforation.

Case Report

A 75-year-old woman with type II diabetes and chronic pancreatitis, presented to the emergency department with fever, severe crampy diffuse abdominal pain, abdominal distension and  bilious vomiting of 3 days duration. She also had abdominal distension and intermittent history of loose, oily stools. There was no weight loss, joint pains, cough, chest pain, or night sweats. There was a previous history of hysterectomy and cholecystectomy.

At the presentation, her blood pressure was 110/70 mmHg, pulse rate 120 beats per minute (bpm), respiratory rate 18 per minute, and temperature 101°F. She had a dry tongue and buccal mucosa. Her chest was clear and cardiac sounds were normal. Abdomen was distended with diffuse tenderness, guarding and rigidity. Bowel sounds were 5-6 per minute. She was conscious, oriented and neurological examination was normal.

With a provisional diagnosis of peritonitis, she was evaluated .

White blood cell count (WBC) 17,670, with Neutrophil 90.1 %,, hemoglobin (Hgb) 11.4 g/dL, Hematocrit (Hct) 34.3 %, mean corpuscular volume (MCV) 89.1 fL, Platelet 3,70,000, Creatinine 0.4, Blood Urea Nitrogen (BUN) 30, Alanine Aminotransferase (ALT) 7 Aspartate Aminotransferase (AST) 14, Alkaline Phosphatase (ALP) 130, Albumin 2.6, total Bilirubin 0.7 and serum was Albumin 2.6.

X-ray abdomen demonstrated air under diaphragm with dilated bowel loops. CECT abdomen was suggestive of pneumoperitoneum with a peripherally enhancing collection and air pockets adjacent to a small bowel loop (proximal ileum) with oral contrast leak suggestive of small bowel perforation.(Fig 1)

Circumferential wall thickening of distal jejunum, proximal ileum and changes of chronic pancreatitis was noted.

Figure 1 :  CT Images:  A- Small Bowel Perforation, B-Air Foci, C-Pneumoperitoneum

Management and outcomes: With the diagnosis of a perforated small bowel, exploratory laparotomy was performed which revealed pus in peritoneal cavity and  entero-enteric fistula between DJ flexure and terminal ileum. Mesentery was thickened with interloop adhesions of terminal ileum. The antimesenteric border of the terminal ileum was perforated at 50 cm from the ileocecal valve with fat stranding of the ileum typical of CD. Otherwise, large bowel, cecum, appendix, and reproductive organs were grossly normal.

70 cm resection of the small intestine including perforated segment, end ileostomy and stapled diversion E-E fistula was done. Hospital stay was seven days.

Histology of the specimen showed foci of deep fissuring ulcers with granulation tissue and dense neutrophilic and lymphoplasmacytic infiltrate. Marked activity in terms of cryptitis and crypt abscess, chronicity in the form of crypt distortion and branching was noted. There was evidence of transmural inflammation without granuloma /dysplasia.

Histopathology confirmed Crohn's Ileitis and she was initiated on subcutaneous Adalimumab for the residual disease as well as to prevent relapse.

Discussion

In this case, it is quite notable that the patient reported no symptoms until her first presentation to the hospital as a perforated bowel. Free perforation of the bowel as first presentation of Crohn’s disease is rare with an incidence of 3.4 %, with majority (86.1 %) involving the ileum3.

Cardinal manifestations of patients with Crohn’s Disease are abdominal pain, diarrhoea with or without gross bleeding, fatigue, and weight loss4. Features of transmural inflammation like stricture, fistula, perforation, hemorrhage, abscess, perianal disease are rare manifestations of Crohn’s disease which would necessitate surgery as mainstay of management5. In 1–16 % of patients undergoing surgery for Crohn’s disease, bowel perforation was the main indication6.

The main aspect of surgical management is to limit the amount of bowel resection as much as possible to avoid complications like short bowel syndrome. Patients who are hemodynamically unstable or who have edematous bowels, intra abdominal contamination from perforation, or other risk factors such as malnutrition or chronic glucocorticoid use should have a diverting stoma rather than a primary anastomosis7.

Crohn’s disease may recur frequently after operative management8. In several studies, the endoscopic recurrence rate was as high as 80 %, one year after surgery; clinical recurrence rate was 10 to 15 % per year7. Between 70 and 90 % of patients will require surgery during their life- time, with up to 39 % requiring repeated surgery7 . Risk factors for recurrence are smoking, genetics (NOD2/CARD15 mutation), shorter preoperative disease duration, presence of proximal gastrointestinal (duodenum and jejunum) disease, prior surgery for Crohn’s disease, first presentation with fistula or perforation8.

The main management of Crohn’s disease is multidisciplinary8. Collaboration of teams with the aim of relieving symptoms and maximizing patient quality of life is the main goal. Prevention of postoperative recurrence of CD requires determining which patients will benefit from early medical therapy and who should be monitored clinically, thereby avoiding the risks of therapy9. Patients with a smoking history, younger than age 30 years, perforating/penetrating/fistulizing/long segment inflammatory disease, history of ≥2 surgeries for CD, shorter disease duration prior to surgery characterize high - risk patients 10 .

For patients at higher risk, it is suggested to initiate immunosuppressive therapy postoperatively. Postoperative therapy with an anti-TNF agent or AZA/6-MP reduces the risk of clinical recurrence. Ileocolonoscopy is performed  at 6 to 12 months post surgery and detection of  endoscopic recurrence despite medical therapy, needs intensification of treatment. If the ileocolonoscopy at 6 to 12 months post  surgery shows no endoscopic recurrence (i.e. Rutgeerts score i0 or i1), the patient can be monitored clinically on this regimen and surveillance ileocolonoscopy is suggested in one to three years 9.

Conclusions

Bowel perforation as the initial presentation of Crohn’s Disease is a rare phenomenon. Adequate resuscitation followed by emergency laparotomy with primary resection and anastomosis could be life-saving for a hemodynamically stable patient. Focus on post operative recurrence is important.

References

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  2. Richards D, Wolf D. Small Bowel Crohn’s Disease in an 85-year-old Woman Presenting with Perforation. Am J Gastroenterol. 2010;105:S360–361. Capsule endoscopy and eventual perforation story  .
  3. Greenstein AJ, Mann D, Sachar DB, Aufses AH Jr. Free Perforation in Crohn’s Disease: Survey of 99 Cases. Am J Gastroenterol. 1985;80(9):682–689. Documents that perforation may be presenting feature and requires urgent surgery  .
  4. Freeman HJ. Spontaneous Free Perforation of the Small Intestine in Adults. World J Gastroenterol. 2014;20(29):9990–9997. Reviews spontaneous small bowel perforation including elderly cases  .
  5. Nwashilli NJ, Nkor SNK, Faleyimu B, Nwajei CO. Crohn’s Disease Presenting as Acute Intestinal Obstruction (69-year-old). Nig J Med Dent Educ. 2020;2(2):86–90. Emphasizes perforation risk in elderly Crohn’s  .
  6. Ikuchi H, Yamamura T. Review of Free Perforation in Crohn’s Disease – Japanese Literature. J Gastroenterol. 2002;37(12):1020–1027. Confirms ileal source and age association  .
  7. Gut Editorial. Crohn’s Disease in the Elderly. Gut. 1987;26(5):461-464. Notes colonic perforation in elderly-onset disease as prognostically significant  .
  8. Ahmed M Makki et al. Spontaneous Colon Perforation in an 82-year-old: Case Report and Literature Review. Case Rep Clin Med. 2014;3:392–397. Demonstrates spontaneous perforation in the elderly  .
  9. Ananthakrishnan AN, Nguyen GC, Bernstein CN. AGA Clinical Practice Update: Management of IBD in Elderly. Gastroenterology. 2021;160:445–451. Offers evidence-based guidance for elderly IBD surgical management  .
  10.  ResearchGate review – Rectosigmoid Crohn’s Leading to Perforation. Cureus. 2022. Illustrates free colonic perforation in Crohn’s across adult ages
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