Case report: acute bowel obstruction with an isolated transition point in peritoneal dialysis patients; a presentation of encapsulating peritoneal sclerosis?

Encapsulating peritoneal sclerosis (EPS) is a rare but devastating complication of long-term peritoneal dialysis (PD). It is characterized by progressive thickening and fibrosis of the peritoneal membrane, which eventually leads to encapsulation of the abdominal content by the abnormal fibrous tissue [1].

The incidence of EPS increases with time on PD, and presents most frequently after cessation of the therapy. Kawanishi et al. reported an incidence of 17.2 % after 15 years of PD therapy [2‐5]. Similarly, the mortality rate from EPS increases with increased time on PD. For example, in the Pan-Thames EPS study, 42 and 100 % of patients succumbed at 3 and 15 years respectively [6]. Duration of exposure to PD therapy seems to be the principal risk factor for developing EPS, along with other currently identified factors including young age, genetic predisposition, and receipt of a kidney transplant.

There is an early inflammatory prodrome in many cases that can be missed. Manifestations include vague abdominal discomfort or anorexia, new-onset infusion pain, hemoperitoneum, and development of rapid transport status. Other clinical symptoms of EPS are directly related to disturbances in gastrointestinal transit, due to abnormal fibrous tissue formation around the bowel loops. The most common findings are abdominal pain, nausea, vomiting, anorexia, abdominal fullness, and eventually partial or complete small-bowel obstruction. Since the initial signs and symptoms of EPS are non-specific and subtle, a high index of suspicion is needed to establish an early diagnosis [7‐9].

The classical macroscopic appearance of advanced EPS is total encapsulation of the bowel by a fibrotic membrane [10]. Laparotomy has usually revealed a thickened, brownish peritoneum with adhesions, and intestines that are partially or totally encapsulated in thick fibrous tissue. In advanced cases, a sclerotic layer has completely covered the intestines, giving the appearance of a cocoon [11, 12].

Localized involvement of parts of the bowel with fibrosis has been reported in the past, and it has been suggested that it could be more common than previously recognized [13, 14].

We report two cases of bowel obstruction with obvious transition point, appearing to be localized, which happened in patients after being on PD for many years. We postulate that localized fibrosis represents an atypical form of EPS.

We present two cases of bowel obstruction with transition point in the context of long term peritoneal dialysis. Patients with longstanding PD and concurrent autoimmune disease have previously identified as being particularly at risk for EPS [13]. EPS has classically been described as marked thickening and sclerosis of the entire peritoneal surface, a retraction of the root of the mesentery, and striking finding of encasement of the entire small bowel. There has been, however, limited number of case reports, describing “localized” EPS. Kirkman et al. reported a case of localized encapsulation of small bowel loops at the level of terminal ileum in a post-transplant patient previously on longstanding PD therapy [14].

Similarly Habib et al. reported 3 cases of “localized” EPS, where intra-operatively, an obvious sclerotic and thickened peritoneal membrane was observed, predominantly covering and constricting the terminal ileum [15].

By reporting these cases, although surgical exploration was not performed in our first case, we aim to emphasize the presence of macroscopic variations in presentation of EPS. We speculate that some of the cases of localized small bowel obstruction, with obvious transition points, in the context of prolonged PD therapy, may in fact represent non-classic forms of EPS. The approach to management of these patients should therefore be similar to those proposed for the more generalized form of encapsulation. A conservative approach with resting the bowels should be tried, and in cases where the index of suspicion for EPS is significant, we suggest termination of PD and switching to a different dialysis modality. In case of persistent bowel obstruction despite conservative management, a surgical approach is suitable, as described in our second case, in addition to termination of PD. Further case studies are needed to investigate the use of pharmacologic agents such as tamoxifen or corticosteroids.

It is suggested that clinicians have a high index of suspicion for EPS in patients on long-term PD therapy who present with acute bowel obstruction with an isolated transition point.

The patients provided full informed consent for gathering the data and publishing the case.