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.