Encapsulating peritoneal sclerosis (EPS) is an uncommon medical condition where a thick, fibrous membrane develops around the intestines. This membrane, often described as an “abdominal cocoon,” can progressively tighten, restricting the movement of the bowels. This encapsulation can cause a complete or partial blockage of the intestines, making it difficult for food and waste to pass through the digestive system.
Causes and Risk Factors
Long-term peritoneal dialysis (PD) is the primary cause of encapsulating peritoneal sclerosis. This kidney treatment involves using the peritoneum, the lining of the abdomen, to filter waste products from the blood. Prolonged exposure of the peritoneum to the dialysis solutions can cause chronic inflammation and scarring, leading to the formation of the fibrous membrane.
The duration of peritoneal dialysis significantly influences the risk, with the likelihood of developing EPS increasing after five to eight years of treatment. Recurrent episodes of peritonitis, an inflammation of the peritoneum often caused by infection, also contribute to the risk. Additionally, the type of dialysis solution used, particularly those with high glucose concentrations and bio-incompatible properties, can damage the peritoneal membrane and promote the condition. While PD is the primary association, EPS can also arise from other, much rarer causes such as prior abdominal surgeries, certain medications like beta-blockers, endometriosis, or even some abdominal cancers.
Symptoms and Complications
Individuals affected by encapsulating peritoneal sclerosis typically experience symptoms that indicate a progressive obstruction of their bowels. These symptoms often begin subtly and become more pronounced over time, making early recognition challenging. Common manifestations include recurring abdominal pain, persistent nausea, and frequent vomiting, especially after eating.
People may also notice a reduced desire to eat, a feeling of fullness in the abdomen, or unexplained weight loss due to poor nutrient absorption. These signs are directly related to the intestines becoming increasingly constricted by the fibrous cocoon. Untreated intestinal obstruction can lead to severe complications such as malnutrition, as the body struggles to absorb nutrients, and even sepsis, a life-threatening response to infection.
The Diagnostic Process
Diagnosing encapsulating peritoneal sclerosis involves a careful evaluation of medical history and imaging findings. An abdominal computed tomography (CT) scan is the primary diagnostic tool. On a CT scan, doctors look for characteristic signs.
These include a thickened peritoneal membrane, often appearing as a dense, enhancing layer. They also observe clumped or tethered bowel loops and signs of intestinal obstruction, such as dilated bowel segments. While imaging provides strong evidence, diagnosis combines these radiological findings with clinical symptoms and a history of risk factors, particularly long-term peritoneal dialysis. In some instances, definitive diagnosis is confirmed during surgery (e.g., laparotomy or laparoscopy), when the fibrous cocoon is directly seen.
Treatment Approaches
Managing encapsulating peritoneal sclerosis involves a multifaceted approach. Nutritional support is a cornerstone of treatment, especially with intestinal obstruction. Total parenteral nutrition (TPN) is administered intravenously, providing nutrients directly into the bloodstream to prevent malnutrition and support recovery. This feeding often begins before surgery and continues into the postoperative period, sometimes for weeks.
Medical management aims to reduce peritoneal inflammation and fibrosis. Corticosteroids, like prednisolone, are prescribed to suppress inflammation and may reduce peritoneal thickening. Tamoxifen, an anti-fibrotic medication, is also used to slow scarring progression, particularly in earlier stages. These medications are used for months; corticosteroids typically for six months, tamoxifen for up to two years.
When medical therapies are insufficient or bowel obstruction symptoms become severe, surgical intervention (peritonectomy and enterolysis) is considered. This procedure involves dissecting and removing the fibrous cocoon from around the intestines, freeing bowel loops and relieving obstruction. While challenging, this surgery can improve outcomes, though patients require extensive pre- and post-operative care, including continued nutritional support and close monitoring. A multidisciplinary team, including nephrologists, dieticians, and surgeons, collaborates to determine the most appropriate treatment.
Prognosis and Life After Treatment
Encapsulating peritoneal sclerosis is a serious condition that has historically been associated with a significant mortality rate, sometimes ranging from 25% to 55% within the first year after diagnosis. However, advancements in earlier diagnosis and combined medical and surgical strategies have led to improved outcomes. A successful recovery often means a considerable period of recuperation, with hospital stays potentially lasting several months.
For many patients, life after treatment involves discontinuing peritoneal dialysis, which is a major risk factor for EPS, and transitioning to hemodialysis for ongoing kidney support. While surgery can relieve obstruction, there is a possibility of recurrence, reported in about 25% of cases, requiring ongoing monitoring. Despite these challenges, a proactive approach to diagnosis and a comprehensive treatment plan can offer a more positive long-term outlook for individuals managing this condition.