Atypical and Life-threatening Crohn’s Disease Following Colectomy: A Case Report

Although Crohn’s Disease (CD) usually occurs between the second and third decade of life, it also may develop in older adults. Treating elderly patients may be challenging due to other comorbidities, including diverticular disease or intestinal ischemia. PURPOSE: The purpose of this case study was to describe successful treatment of atypical and life-threatening CD due to enterocutaneous fistulas with short-bowel syndrome and multiorgan failure after partial colectomy. CASE REPORT: After an urgent colectomy for an inflammatory colon tumor, a 64-year-old woman with a history of CD and multiple comorbidities developed acute small bowel ischemia. Following an extended bowel resection, she developed a severe surgical site infection, entero- and gastrocutaneous fistulas, multiorgan failure, and short bowel syndrome. Her care included intensive medical and nutritional treatment as well as negative pressure wound therapy (NPWT) using continuous negative pressure of -80 mm Hg. She not only survived, but she also achieved complete wound closure and restoration of digestive tract continuity and metabolic control. She was discharged with a central venous catheter on total parenteral nutrition. CONCLUSION: In this case study, a good outcome was observed using intensive medical treatment, nutritional therapy, and conservative surgical treatment that included NPWT for a patient with CD and major comorbidities who developed postoperative complications. 

Crohn’s Disease (CD) is a chronic, incurable, idiopathic, nonspecific, segmental, transmural inflammatory process of the gastrointestinal tract that may develop in any part of the gut. The disease most frequently occurs between the second and third decade of life.¹ On average, 10% to 30% of CD patients >60 years of age are affected by irritable bowel disease (IBD); this includes patients with inflammation de novo or with recurrences.² According to a population-based cohort study of elderly-onset inflammatory bowel disease,³ patients >60 versus <17 years old with CD frequently experience bleeding from the lower part of gastrointestinal tract (44% vs. 33%) and less commonly have abdominal pain (59% vs. 83%), diarrhea (60% vs. 70%), fever (18% vs. 34%), and weight loss (45% vs. 64%). According to literature reviews,^4,5 the clinical picture of patients with CD sometimes suggests diseases other than IBD, such as colorectal cancer or diverticular disease, that frequently can involve urgent operations. Older patients also may follow a more typical course of CD, with local complications characterized by the occurrence of strictures and/or abscesses and/or internal and external fistulas that often necessitate surgery. 

In addition, CD currently is the most common cause of spontaneous GI fistulas among patients in developed countries. The risk of fistula formation is estimated to range from 30% to 50% at 10 and 20 years after CD diagnosis, respectively.⁶ However, abscesses/fistulas also may develop after surgical procedures for CD.^1,6 The anatomical evaluation of fistula origin, length, branching, presence of a possible distal obstruction, and gut length and its adequate perfusion affect management and prognosis. Fistula evaluation requires radiological evaluations such as ultrasound, barium swallow or enema, computed tomography, and magnetic resonance enterography among others. According to a case report⁷ and a review of the literature,⁸ surgical treatment of CD is considered a last resort and should spare as much of the digestive tract as possible to avoid a short-bowel syndrome (SBS). Postoperative mortality and morbidity are significantly increased in older patients due to age, comorbidities, and the frequent need for urgent surgery.^6,8 

The purpose of this study was to present the case of a 64-year old female patient who, despite having an atypical and life-threatening course of CD due to severe surgical site infection, dehiscence, enterocutaneous fistulas with SBS, and multiorgan failure after partial colectomy, was successfully treated with conservative surgical methods combined with intensive medical therapy.

Ms. N, a 64-year-old woman with CD and a history of coronary disease with circulatory failure, stroke, pulmonary emphysema, and psoriasis, was admitted to the authors’ surgery clinic in November 2016 due to an inflammatory tumor within the splenic flexure of the colon with accompanying intraperitoneal abscesses. A colonoscopy performed in 2010 had shown diverticula of the colon, and in January 2015, Ms. N underwent Hartmann’s sigmoidectomy with a terminal colostomy for critical stricture of the colon. Six (6) months later, a loop-transverse ostomy was performed due to stenosis of the previous terminal colostomy. Previously obtained histopathological results suggested colitis in the course of diverticulitis. Ms. N complained of bleeding through the stoma and weight loss after the primary operation. In addition, the loop ostomy had retracted and become strictured with concomitant peristomal fistulas. Another colonoscopy through the loop ostomy with biopsy performed in October 2015 showed CD contrary to the aforementioned postoperative histopathology assessment from January 2015. Following admission to the authors’ clinic on November 29, 2016, the remnant of the colon and ostomy were excised and a terminal ileostomy created. 

Ms. N’s postoperative course was complicated; she experienced acute ischemia of the small bowel following extended resection of the diseased bowel loops and formation of a terminal jejunostomy on December 8. As a result, she developed SBS; a 60-cm length of the jejunum remained. Because stomal output on oral nutrition exceeded 1500 mL with resulting dehydration, malabsorption, and malnutrition (body mass index [BMI] 15.5), Ms. N was put on total parenteral nutrition. She also was treated using an open-abdominal (OA) technique, undergoing several laparotomies for recurrent intra-abdominal abscesses. In addition, the ischemic perforation of the stomach wall was oversown (see Figure 1); subsequently, an enterocutaneous fistula >20 cm developed over the jejunostomy (see Figure 2). Furthermore, Ms. N developed a recurrent walled-off perforation of the stomach, which together with the intestinal fistula provided up to 2000 mL of digestive content per day, resulting in a narrowing of jejunostomy (see Figure 3). 

Ms. N’s general condition deteriorated, and she was admitted to the High Dependency Unit on December 30 in septic shock and multiorgan failure. Ventilator treatment, hemodialysis, and broad-spectrum antibiotics were provided. Pleural drainage was installed for increasing hydrothorax. Parenteral nutrition was modified with immune modulatory amino acids that included L-arginine and L-glutamine and medium chain triglycerides to address hypoalbuminemia and lymphopenia. Evaluation of Ms. N’s basal metabolic rate, including anthropometric grade and laboratory tests, yielded BMI of 15, albumin serum range 1.54 g/dL, and lymphocyte blood count 0.67 K/uL. Six (6) units of blood, 8 units of fresh frozen plasma, and 1100 mL of 20% albumin were transfused over a period of 6 weeks after the December 8 surgery.

The open wound with fistulas and strictured jejunostomy were treated with negative pressure wound therapy (NPWT) (VivanoMed Abdominal Kit; Paul Hartmann AG, Heidenheim, Germany), installed under general anesthesia due to massive adhesions within the peritoneal cavity. The bottom of the wound was covered with microperforated protective film filled with a regular polyurethane open-pored foam (VivanoMed Foam; Paul Hartmann AG, Heidenheim, Germany), and affixed to the adjacent skin with an adhesive hydrofilm. Next, an opening was created in the film, and a negative pressure port (VivanoTec Port; Paul Hartmann AG, Heidenheim, Germany) was installed. Another opening in the film, created for the jejunostomy, was sealed with stoma paste, and the stoma was protected with an ostomy bag (see Figure 4). Throughout the treatment, continuous negative pressure of -80 mm Hg was maintained to achieve a balance between appropriate conditions for proper wound healing and the risk of increasing the fistula output. 

Analgesic treatment was provided using opioids and nonsteroidal anti-inflammatory drugs. Pain was monitored using a visual analog scale; pain did not exceed level 7 throughout the hospitalization period. In next 7 days, wound size decreased from 17 cm to 13 cm in the sagittal plane, and stoma elevation increased 2 cm. The NPWT dressing was changed twice during this time. In addition, the peritoneal cavity was supplied with 3 drains; discharge of the bowel and stomach content decreased from 1500 mL to 800 mL in 2 weeks. As a result, 2 of the 3 drains were removed. Extended NPWT treatment and mechanical dilatation of the jejunostomy resulted in steadily increasing output from the stoma, and the fistula healed within 6 additional weeks (see Figure 5). However, wound healing was unsuccessful due to protracted discharge of purulent exudate (100 to 150 mL) through the drain that remained in the wound. 

Additionally, Ms. N developed dehiscence of the rectal stump with pathological communication between the stump, peritoneal cavity, and the wound (see Figure 6). Therefore, the endoluminal NPWT dressing was modified using a Foley catheter wrapped with the foam and covered with a protective paraffin cotton dressing; this was introduced into the canal after removal of an abdominal drain. Continuous negative pressure of -10 mm Hg was maintained (see Figure 7). This low negative pressure was introduced to achieve a balance between the effective evacuation of septic discharge and to decrease the risk of damage to the bowel. Subsequently, adequate drainage resulted in healing of the rectal stump in 8 weeks. Wound margins were finally closed with histoacryl glue (see Figure 8) by the end of April 2016. 

Ms. N was released home in May 2016 in good general condition on parenteral nutrition after implantation of a Broviac catheter (Bard Limited, Crawley, UK). Oral nutrition was limited to 250 mL of fluids and 3 to 4 slices of rice or corn bread per day. Ms. N ultimately achieved metabolic balance (evidenced in a BMI of 17 and ostomy output under 1000 mL/ day). She has been in a good general condition for >24 months and is followed-up through the outpatient nutrition department.

Postoperative morbidity and mortality are higher among patients with CD than patients who have other benign or malignant bowel diseases; morbidity is estimated to be 30% and mortality is approximately 7%.⁹ The increased risk may be due to many factors such as malnutrition, older age, use of immunosuppressive and biological treatments and corticosteroids, presence of intraperitoneal abscesses, and the nature of the inflammatory process itself. Surgical techniques such as handsewn end-to-end bowel anastomosis and suturing within inflammatory or ischemic tissues also are thought to increase morbidity.^10-12 The most common and challenging complications are severe surgical site infections, eventration, dehiscence, anastomotic leakage with subsequent abscess/fistula formation, enterocutaneous fistulas, or anastomotic stricture with subsequent bowel obstruction.⁷

The treatment of critically ill patients with severe and complex surgical site infections, abscesses, and enterocutaneous fistulas with eventration or dehiscence developing after primary or redo operations for CD should focus on intensive life-supporting medical management that addresses correction of fluid and electrolyte disturbances, sepsis that is treated with appropriate antibiotics guided by culture sensitivity, and percutaneous drainage of accompanying fluid collections. Treatment also should consider controlling fistula output by restricting hypo-osmolar fluids, administering antisecretory and antimotility agents, managing the underlying disease, weaning off immunosuppressive medications, and providing nutritional therapy.^13,14 

In turn, conservative local surgical treatment should consist of optional wound debridement. In the current case  (an open wound with fistulas and strictured jejunostomy), NPWT was used to heal OA, along with cleansing (debridement) of the wound edges,  management of effective drainage of discharge, and delayed reconstruction of the abdominal wall layers in a case of eventration, loss of domain, or dehiscence. NPWT can provide a kind of surgical drainage; its application in complex surgical site infections with dehiscence and/or with accompanying enterocutaneous fistulas in high- risk patients with multiorgan failure might be a viable alternative to conventional abdominal drainage performed during relaparotomy. Other advantages of NPWT are that it can limit the formation of peritoneal adhesions,^7,15 promote evacuation of septic discharge,^16,17 reduce inflammatory edema,¹⁶ improve blood supply and drainage,¹⁷ reduce wound size as part of the mechanism of micro- and macrodeformations,¹⁸ and help facilitate the formation of granulation tissue.¹⁹

 Furthermore, Rowan et al²⁰ demonstrated on 32 patients with 37 wounds that NPWT can help control overgrowth of bacteria due to a higher concentration of antibiotics within the surgical site. NPWT was the treatment of choice for the investigated patient, in part perhaps because the risk of mortality increases significantly to >50% in elderly patients with hypoalbuminemia and with high Acute Physiology, Age, Chronic Health Evaluation II²⁰ or Sequential Organ Failure Assessment Score scores.^21,22

According to the manufacturer’s instructions for the product used in this study, NPWT can be used safely directly over exposed organs; other options include RENASYS Open Abdominal Solution (Smith & Nephew, Hull, UK) and ABTHERA SENSAT.R.A.C. Open Abdomen Dressing (KCI, an Acelity Company, Fort Worth, TX).

Nutritional treatment should include evaluation of metabolic rate according to European Society for Clinical Nutrition and Metabolism guidelines along with assessment of anthropometric grade (nutritional risk scale and subjective global assessment scores), laboratory tests (prealbumin, albumin, levels), complete lymphocytic count, and nitrogen balance. Patients treated through OA remain in a hypercatabolic state with an estimated nitrogen loss of almost 2 g/L to 4.6 g/L of abdominal fluid output.²³ According to point-of-prevalence study²⁴ based on the assessment of 206 patients, monomeric parenteral nutrition is recommended for patients with acute intestinal failure (IF). Hemodynamically stable patients with a small bowel remnant median length of at least 75 cm should receive enteral polymeric nutrition of 20 to 30 kcal/kg nonprotein calories per day, including 1.5 g/kg to 2.5 g/kg of protein.¹⁹ According to  a review of the literature,¹⁴ the rate of mortality decreases 3.6 times in patients who receive more than 1500 kcal per day. In addition, Alhagamhmad²⁵ suggests that nuclear factor-κB, a factor associated with higher frequency of ileocolonic CD, might be indirectly blocked due to immunomodulatory nutrition enriched with L-arginine and L-glutamine. 

Surgical treatment of an elderly patient with CD and major comorbidtiies who developed postoperative complications required an interdisciplinary approach, ultimately achieving good outcomes. Critically ill patients with severe surgical site infections accompanied by eventration, dehiscence, abscesses, enterocutaneous fistulas, loss of domain, and multiorgan failure rely on intensive life-supporting medical therapy, appropriate nutrition, and conservative local treatment (eg, NPWT). NPWT appropriate for the OA and nutritional realimentation are the fundamental elements of therapy in these cases. 

Dr. Cwaliński is a senior assistant; Dr. Hermann is an assistant professor; Dr. Banasiewicz is a professor and head of Department; and Dr. Paszkowski is an assistant professor, Department of General, Endocrinological Surgery and Gastroenterological Oncology, Poznan University of Medical Sciences, Poznan, Poland.

Please address correspondence to: Jaroslaw Cwaliński, MD, PhD, Department of General, Endocrinological Surgery and Gastroenterological Oncology, Poznan University of Medical Sciences, Przbyszewskiego 49, 60-355 Poznan, Poland; email: jaroslaw.cwalinski@gmail.com.