A case of idiopathic encapsulating peritoneal sclerosis with intractable ileus successfully treated by surgery and steroid therapy

Encapsulating peritoneal sclerosis (EPS) occurring without a history of peritoneal dialysis is rare. We report on a patient with idiopathic EPS following intractable ileus who was successfully treated by surgery and postoperative steroid therapy without any sign of recurrence. A 67-year-old woman was referred to our department for further treatment of intractable ileus. Abdominal CT scanning revealed wall thickening of the proximal jejunum. Double-balloon enteroscopy disclosed stenosis of the jejunum at 20 cm anally from the Treitz ligament, although the intestinal mucosa appeared normal without specific biopsy findings. In addition, FDG-PET showed no abnormal accumulation, thus discounting a malignant lesion. Since conservative therapy failed to improve the ileus, we performed an operation on her in order to release the ileus and make a histological diagnosis. Surgical findings included a whitish thickening of the serosa extending to the intestine and the whole mesentery. Accordingly, we made a diagnosis of idiopathic encapsulating peritoneal sclerosis because of her negative history of peritoneal dialysis, laparotomy or peritonitis, in addition to the above-noted findings. Postoperative oral administration of steroid has suppressed EPS recurrence. In patients with intractable ileus, EPS should be added to the list of differential diagnoses, even if they have not undergone peritoneal dialysis.