Post-colectomy enteritis—a rare complication in a patient with ulcerative colitis

Abstract

Colectomy is the surgical treatment of choice in patients with severe or complicated ulcerative colitis. Post-colectomy enteritis (PCE) is a rare condition that can develop after proctocolectomy and has been described only in a few individual cases so far. We report the case of a 46-year-old patient with severe and treatment-refractory ulcerative colitis who underwent colectomy. A few days after surgery, the patient presented with acute anemia and hematemesis. Esophagogastroduodenoscopy showed massive inflammation of the duodenum and jejunum. The life-threateningly ill patient was admitted to the intensive-care unit and treated for severe PCE with ciclosporin and corticosteroids. PCE is a distinct inflammation of the small intestine shortly after colectomy. The patient recovered fully and was discharged after prolonged recovery and long-term intensive care therapy.

Introduction

Inflammatory bowel disease (IBD) includes both Crohn’s disease (CD) and ulcerative colitis (UC). Both are chronic and complex disorders of the digestive tract characterized by chronic inflammation with periods of activity interrupted by times of complete remission. Both diseases are increasing in incidence all over the world. ¹ Due to a disabling course, they directly influence the quality of life of all patients and have a huge impact on healthcare systems.

Optimal and early treatment in patients according to the current guidelines is key to prevent the progression of the disease with consecutive tissue remodeling, stricture formation, or the need for surgery in both CD and UC.^2,3 Although a variety of drugs for the treatment of UC and CD are available, therapeutic success is still limited. ⁴

In contrast to UC, CD can involve all parts of the digestive tract from the oral cavity to the anus characterized by “skip lesions” with a predominant involvement of the terminal ileum or the colon but also the small bowel. UC is an inflammatory bowel disorder mainly limited to the colon with continuous involvement. There are three well-known entities where inflammation can spread to the small intestine in UC: backwash ileitis, pouchitis, and prestomal ileitis. ⁵ In addition, extraintestinal manifestations of UC, such as dermatologic, ophthalmic, or liver disorders can occur. Inflammation of the duodenum and small intestine, however, is a rare complication of UC, which occurs in patients after colectomy or proctocolectomy.

We present the case of a 46-year-old man with UC who developed severe post-colectomy enteritis (PCE) following subtotal colectomy.

In addition, we discuss the results of a literature review on PCE following surgical treatment of UC.

Case report

A 46-year-old patient was first diagnosed with UC (initially limited proctitis) in 2016. Local therapy was initiated with budesonide rectal foam and mesalazine. The patient was in clinical remission for 1 year. Details on mucosal healing at that time were not available because treatment was done in another clinic. One year later, a severe flare occurred with pancolitis, and steroid treatment was initiated. Symptoms improved with steroids but worsened again whenever the dose was tapered. Unfortunately, the patient received continuous high-dose steroid treatment for 2 years with an average dose of 100–150 mg prednisolone/day at a body weight of 75 kg.

The patient was referred to our center for further assessment and treatment in 2019. Due to the persistent steroid dependency and lack of clinical remission, biological treatment was planned. At this point, our patient suffered from severe clinical symptoms, including a stool frequency between 15 and 20 bowel movements/day and night, bloody diarrhea, abdominal pain, and muscle dystrophia due to the continuous long-term steroid treatment. Shortly before initiation of treatment with infliximab, the patient presented with severe shortness of breath as a result of pulmonary embolism, post-embolic pneumonia, and deep venous thrombosis of the left leg. Right heart strain was noted in the echocardiogram. Anticoagulation treatment was commenced, and symptoms abated.

Six months later and under treatment with infliximab, follow-up showed near-complete endoscopic and histological remission. The calprotectin level at the beginning of the treatment was 378 µg/g (normal < 50 µg/g). Due to the incompliance of the patient with the lack of further stool samples, further calprotectin levels could not be determined. About 12 months later, the patient experienced clinical and endoscopic relapse due to secondary loss of response to anti-TNF treatment (despite dose intensification every 4 weeks and up to 10 mg/kg body weight, antibody and trough levels were not measured). As a result, the treatment regime was switched to ustekinumab in 2021 (due to its minimal side effects and the convenient application mode of ustekinumab). Initially, clinical response to ustekinumab was noted; however, after a further 12 months, clinical loss of response occurred again. Unfortunately, the patient was lost to clinical and endoscopic follow-up during this year for a total of around 10 months.

In November 2022, the patient presented to our emergency department with abdominal pain, massive and profuse bloody diarrhea (30 bowel movements/day), and weight loss of 8 kg. Laboratory values showed anemia and significant elevation of inflammatory markers (CRP 5.99 mg/dl (ref.: 0–0.5 mg/dl), leukocytes 10.95/nl (ref.: 3.0–10/nl), erythrocytes 3.74/pl (ref.: 4.5–6.1/pl), Hb 73 g/l (ref.: 140–180 g/l), and platelets 571/nl (140–440/nl)). Sigmoidoscopy showed severe proctocolitis with ulcers, friability, and spontaneous bleeding (Mayo score: 11 points). Multidisciplinary team (MDT) discussion recommended colectomy (Figure 1). Because of his poor general condition at that time Eastern Cooperative Oncology Group (ECOG) performance status of 2, presurgical parenteral nutrition was initiated. Three days after admission, the patient developed a secondary bowel perforation and was transferred for emergency colectomy. A subtotal colectomy with a terminal ileostomy was performed. Intraoperatively, the colon was described as friable with several perforations in the transverse colon and the splenic flexure. Histopathology confirmed a highly active, ulcerative, focally perforated colitis with peritonitis.

Figure 1.

Index colonoscopy of the patient presenting with a severe flare of ulcerative colitis: obliterated vascular pattern, deep ulcers, spontaneous bleeding.

The initial postoperative course was complicated by post-surgical rectal stump insufficiency, which was treated conservatively with endoscopic vacuum therapy. In addition, the patient developed upper gastrointestinal (GI) bleeding and was transferred to the intensive care unit. Emergency esophagogastroduodenoscopy (EGD) showed severe inflammation of the duodenum. In the follow-up EGD, a dramatic worsening of inflammation in the duodenum and jejunum was noted (Figures 2 and 3). Possible differential diagnoses included infections (e.g., cytomegalovirus infections) and ischemia, which could be excluded finally. After an extensive literature review as to the cause of severe intestinal inflammation following colectomy for UC, post-colectomy enteritis (PCE) was diagnosed. Treatment was initiated, as described in the few reported cases of PCE, with 60 mg prednisolone/day and 2 mg/kg body weight ciclosporin/day. In addition, anticoagulation with at least low molecular weight heparin was recommended because of the significantly elevated risk of thromboembolic events under the aforementioned treatment regime. However, because of continuous and diffuse bleeding, anticoagulatory therapy was delayed, and the patient developed a thrombosis of the basilic vein.

Figure 2.

Left: Index EGD after colectomy. Inflammation of the duodenum. Right: same duodenum in short-term course.

Figure 3.

Examples of the corresponding histology to Figure 2: left HE staining, 10-fold magnification, showing an ulceration in the duodenum with severe inflammation. Right: same area, PAS staining 10-fold magnification.

Clinical and laboratory improvements were noted in the further course of events and under treatment with prednisolone and ciclosporin. Endoscopic follow-up also showed gradual improvement of inflammation; however, stricturing of the descending duodenum developed. Successful endoscopic dilatation with a 9 mm balloon was performed. Ciclosporin and prednisolone were tapered within the following weeks. After 4 weeks of intensive care and 25 days of treatment on the regular ward, the patient was discharged home under a low-dose prednisolone medication regime.

Discussion

We presented a patient with extensive inflammation of the duodenum and small intestine following colectomy after a severe flare of UC, a so-called “post-colectomy enteritis.” In the literature, only a few case reports of post-colectomy enteritis have been described. Usually, UC is confined to the colon and terminal ileum (backwash ileitis). In rare cases, however, inflammation of the duodenum occurs in a close temporal context to colectomy in patients suffering from UC,^6–14 with a median time of 4 months. ⁹ In almost all reported cases, patients responded well to corticosteroids or calcineurin inhibitors. So did our patient.

In the past, there have been single reports with a small number of patients, indicating that gastroduodenal inflammation may not only occur in patients diagnosed with Crohn’s disease but also in patients with UC.^15–18

Hori et al. conducted a prospective study with 250 UC patients “to examine the prevalence and characteristics of gastroduodenitis associated with UC (GDUC).” ¹³ In 7.6% of the patients, GDUC was diagnosed. They concluded that steroid therapy might mask an inflammation of the upper GI tract and that the severity of a flare increases the risk of developing GDUC. ¹³

A similar finding was described by Sun et al. ¹⁹ In a retrospective study, Kato et al. reported nearly 20% of patients with upper GI tract lesions in UC patients.^3,20

In our case, the patient had been treated with enormous doses of steroids (average doses of 100–150 mg prednisolone/day) at the initial diagnosis despite all recommendations of guidelines ² and still suffered a drastic flare with colonic perforation. In case of steroid dependency or refractoriness, initiation of biologics or small molecules is mandatory. However, our patient received only steroids over the years and had ongoing inflammation until he was transferred to our center. Biologic therapy stabilized the situation only for a short time. Colectomy was perhaps the straw that broke the camel’s back and made the masked GDUC obvious with fulminant post-colectomy enteritis.

Today it is well known that the early use of immunosuppressive therapy or the introduction of biologics in IBD is associated with, for example, a reduced risk of surgery or risk of colectomy. In addition, less hospital admissions are noted. So also in our patient, early treatment with biologics might have prevented a severe course of UC and the need for surgery.^21–24

In our patient, post-colectomy treatment was successful using ciclosporin. However, other medical treatments such as tacrolimus, ustekinumab, or anti-TNFs have also been efficient in case reports or case series.^25–27

Due to the rarity of the complication reported (one study identified 15 cases in over 22 years ¹⁸ ), it has also been discussed whether the diagnosis of UC might have been a misdiagnosed Crohn’s disease. However, histopathological findings of the colon specimen showed, unequivocally, the diagnosis of UC. Furthermore, epithelioid cell granulomas were not detected in the specimen of the duodenum, and there was no other evidence of CD.

While post-colectomy enteritis is supposed to occur only once, ⁸ several authors describe chronic inflammation of the duodenum after colectomy.^9,12,17 In one study, 18 out of 54 cases occurred within 4 weeks after colectomy, as it was the case in our patient. ¹⁴

The underlying pathomechanisms for post-colectomy enteritis are not well understood. In a retrospective analysis, patients with UC and high disease activity and the need for rescue therapies such as anti-TNF or calcineurin inhibitors seem to be at higher risk for post-colectomy enteritis. ²⁸ Another retrospective study revealed postoperative pouchitis as a risk factor for upper GI lesions. ²⁹

Conclusion

Hence, it is important to be aware that UC may not be exclusively restricted to the colon (and terminal ileum). Adequate therapy on a case-to-case basis is decisive in UC: steroid-sparing therapy regimens should be applied. If steroid dependency occurs, early initiation of immunosuppressives, biologics, or small molecules is mandatory for the prevention of progressive disease. However, when colectomy is needed tight follow-up after colectomy might help identify patients with this rare disease condition with involvement of the upper GI tract due to its poor outcome. Until today, our patient has been in close follow-up as recommended in other cases published and is in a clinically stable condition.