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2018 ; 24
(28
): 3101-3111
Nephropedia Template TP
World J Gastroenterol
2018[Jul]; 24
(28
): 3101-3111
PMID30065556
show ga
Encapsulating peritoneal sclerosis (EPS) is a debilitating condition
characterized by a fibrocollagenous membrane encasing the small intestine,
resulting in recurrent small bowel obstructions. EPS is most commonly associated
with long-term peritoneal dialysis, though medications, peritoneal infection, and
systemic inflammatory disorders have been implicated. Many cases remain
idiopathic. Diagnosis is often delayed given the rarity of the disorder combined
with non-specific symptoms and laboratory findings. Although cross-sectional
imaging with computed tomography of the abdomen can be suggestive of the
disorder, many patients undergo exploratory laparotomy for diagnosis. Mortality
approaches 50% one year after diagnosis. Treatment for EPS involves treating the
underlying condition or eliminating possible inciting agents (i.e. peritoneal
dialysis, medications, infections) and nutritional support, frequently with total
parenteral nutrition. EPS-specific treatment depends on the disease stage. In the
inflammatory stage, corticosteroids are the treatment of choice, while in the
fibrotic stage, tamoxifen may be beneficial. In practice, distinguishing between
stages may be difficult and both may be used. Surgical intervention, consisting
of peritonectomy and enterolysis, is time-consuming and high-risk and is reserved
for situations in which conservative medical therapy fails in institutions with
surgical expertise in this area. Herein we review the available literature of the
etiology, pathogenesis, diagnosis, and treatment of this rare, but potentially
devastating disease.
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