Treatment Options for Encapsulating Peritoneal Sclerosis Based on Progressive Stage

Hideki Kawanishi, Yoshiko Harada, Toshio Noriyuki, Toru Kawai, Syunsuke Takahashi, Misaki Moriishi, Shinichiro Tsuchiya
From: Akane Foundation, Tsuchiya General Hospital, Hiroshima, Japan.



Sclerosing encapsulating peritonitis (SEP) is recognized as a serious complication of peritoneal dialysis. However, recovery is possible if an appropriate diagnosis and treatment are made. The term SEP is used most often, but is inaccurate, particularly the reference to peritonitis. A more accurate description would be “encapsulating peritoneal sclerosis” (EPS).
From the therapeutic perspective, the diagnosis should be established before EPS develops. Early diagnosis is important. Furthermore, it is also important to determine the therapeutic tactics for EPS according to the disease stage.
Most cases of EPS develop with manifestations of fever, increased levels of C-reactive protein (CRP), and slight ileus symptoms, accompanied by increased ascites (“inflammatory stage”). Following precise identification of the inflammatory stage, steroid administration should be initiated immediately with the onset of EPS. Methylprednisolone pulse therapy is recommended during the early stage.
If the EPS is not relieved, or if it recurs within 1 month, the steroid dose should be decreased and the patient should be managed by total parenteral nutrition (TPN) (“encapsulating stage”).
If ileus symptoms remain despite the absence of inflammatory findings and decreased ascites, laparotomy and enterolysis should be considered within 6 months (“ileus stage”). However, it is important that the enterolysis be performed without damaging the capsule-covered intestine.
To date, we have successfully treated EPS in 18 of 19 patients using these options. In 3 patients, EPS was relieved by steroid administration. In 15 patients, EPS was relieved by total intestinal enterolysis. Enterolysis patients had satisfactory operative outcomes and eventually returned to their previous social activities. One patient experienced perforation of the small intestine and pan-peritonitis, and died of sepsis.
In summary, EPS is not an incurable disease. It can be completely overcome by active diagnosis and treatment.

Key words

Sclerosing encapsulating peritonitis (SEP), encapsulating peritoneal sclerosis (EPS), enterolysis

Introduction

Sclerosing encapsulating peritonitis (SEP) is recognized as a serious complication of continuous ambulatory peritoneal dialysis (CAPD). Previously, total parenteral nutrition (TPN), which is for rest of the intestine, has been the main treatment for SEP (1–3). However, some cases of SEP have been reported to be successfully relieved when treatment appropriate to the particular SEP conditions was performed. Recent studies have shown the effectiveness of steroids in some patients (4–7); this option is currently being investigated. Surgical treatment for SEP is considered to be unsuitable (1,2,8), but patients who have recovered as a result of enterolysis have also been reported (9–11). A careful evaluation of the surgical techniques used to treat SEP is therefore necessary.
The pathologic condition previously referred to as sclerosing encapsulating peritonitis (SEP) does not actually exhibit the state of peritonitis, making the term potentially confusing. Because the Ad Hoc Committee of the International Society for Peritoneal Dialysis (ISPD) proposed that SEP should be called encapsulating peritoneal sclerosis [EPS (12)], that term is used in the present report.
We have encountered 19 patients with EPS. Of these patients, 15 were treated by laparotomy and total enterolysis, which relieved the EPS in all cases so treated. This report presents surgically treated cases of EPS and discusses therapeutic tactics for EPS.

Patients and methods

We have encountered 19 patients with EPS and have obtained satisfactory results in 18 cases (Table I). The 19 patients (13 men, 6 women) ranged in age at PD start from 11 – 53 years, with a mean age of 32 years. The underlying disease in all 19 patients was found to be chronic glomerulonephritis. All of the patients were treated with lactate-buffered dialysate. The PD period for the patients ranged from 61 months to 176 months, with a mean period of 112 months.
Of the 19  patients with EPS, 18 were diagnosed after transfer to hemodialysis (HD), including 2 patients whose peritoneal catheter remained in place. The reasons for transfer to HD were: ultrafiltration failure (8 patients), refractory or recurrent peritonitis (5 patients), hypoalbuminemia (2 patients), catheter-related infection (1 patient), renal transplantation (2 patients), and occurrence of EPS (1 patient). The period between the transfer to hemodialysis and diagnosis of EPS ranged from 1 week to 29 months, with a mean period of 8 months. Six patients had no history of peritonitis.
Fifteen patients were treated by steroid administration. Steroid administration relieved the EPS in 3 patients. A patient (no. 16, HH) was successfully relieved by repeated steroid pulse therapy. However, that patient suffered a perforation of the small intestine, and pan-peritonitis suddenly developed. He died of sepsis 1 month later.
Laparotomy and enterolysis were performed in 15 patients. Of these, 11 patients had received steroid therapy after the onset of EPS. However, laparotomy was performed owing to residual ileus symptoms. The duration between the onset of EPS and laparotomy ranged from 1 month to 33 months with a mean of 9.6 months.
In all 15 patients, the abdominal organs were covered with white capsules that were severely adherent to the intestine. However, the severity of the adhesions between the capsules and the intestine varied among the patients. The postoperative course for all patients was satisfactory during the follow-up period of 1 – 66 months.

Discussion

Therapeutic approaches for EPS
Encapsulating peritoneal sclerosis develops with peritoneal sclerosis and encapsulation, plus intestinal adhesion. From the therapeutic perspective, the diagnosis should be established before the development of EPS. Early diagnosis of EPS is important. It is also important to determine the therapeutic tactics for EPS according to the disease stage.
Table II shows the stage classification and therapeutic tactics that we propose for EPS. Most cases of EPS develop several weeks or months after the removal of a peritoneal catheter, with manifestations of fever, increased C-reactive protein (CRP) levels, and slight ileus symptoms accompanied by increased ascites (“inflammatory stage”). Following precise identification of the inflammatory stage, steroid administration should be initiated immediately with the onset of EPS. Methylprednisolone pulse therapy is recommended during the early stage.
If the EPS is not relieved or if it recurs within 1 month, the dose of steroids should be decreased, and the patient should be managed by TPN (“encapsulating stage”).
If ileus symptoms remain despite the absence of inflammatory findings and decreased ascites, laparotomy and enterolysis should be considered within 6 months (“ileus stage”). However, it is important that the enterolysis be performed without damaging the capsule-covered intestine.

Timing of the indication of surgery
As described earlier, surgery should be performed after the inflammation has subsided. The duration of PD and the presence or absence of peritoneal calcification are important factors for determining the indication for surgical treatment.
In patients who have received long-term PD, the intestinal wall is degenerated: the result is an ill-defined boundary between the capsule and the intestinal wall. And because peritoneal calcification extends from the serosae to the muscular layer, enterolysis can easily induce intestinal perforation in such patients.
If ileus symptoms are suspected, surgery should be performed in patients who have received PD for less than 10 years without complication by peritoneal calcification. Surgery may also be indicated in those whose ileus symptoms are induced by increased oral food intake.
Patients who have received PD for more than 10 years without complication by peritoneal calcification should be treated surgically even when peritoneal degeneration is suspected. Surgery may be indicated for patients with local peritoneal calcification. However, surgery should be carefully performed in those with peritoneal calcification extending to the entire intestine. The indication for surgery in patients with EPS should be determined by surgeons in consultation with physicians who specialize in PD.

Surgical techniques
In patients with EPS, the entire intestine is generally covered with capsules. Furthermore, because fibrous tissue causes the intestine in these patients to adhere together, enterolysis is performed basically to isolate the intestine. Enterolysis should be initiated from the region where the capsule can be most easily ablated. At the least, the ileus-producing lesion should be ablated.
Ablation of the stomach and duodenum is not required in most cases of EPS. If encapsulation-induced stenosis or loss of peristalsis is observed in the large intestine, ablation may be required, particularly in the sigmoid colon. However, complete ablation is not required in the large intestine; linear excision of the capsule is sufficient for recovering peristalsis. Moreover, linear excision of the capsules is often performed at several regions of the small intestine if complete enterolysis is difficult to perform.
When the capsule is not well demarcated from the intestinal serosae owing to degeneration of the intestinal wall in patients who have received long-term PD, enterolysis should be performed at the muscular layer. However, enterolysis at the muscular layer should be limited to the minimum level in such patients.
Because enterolysis at the calcified region easily causes intestinal perforation, careful ablation is required. If the intestinal wall is perforated, the perforation should be closed by suture, using reinforcing materials such as an absorbing sheet.
Intestinal resection and anastomosis are contraindicated in EPS. Although some reports have described favorable results from intestinal resection and anastomosis for EPS, most cases of EPS treated in this manner have tragic outcomes owing to anastomotic failure (1,2,8). Intestinal resection and anastomosis can be performed in patients who received short-term PD without complication by degeneration of the intestinal wall. However, complete enterolysis can be performed in such patients. Intestinal resection and anastomosis should not be performed in patients who have received long-term PD, even when the calcified region cannot be easily ablated. The surgical technique for EPS basically consists of persevering with enterolysis.

Conclusion

In 18 patients, EPS was successfully relieved by the therapeutic tactics described in this paper. Because EPS has been considered a fatal complication of peritoneal dialysis, the induction rate of peritoneal dialysis may have decreased in Japan. However, EPS is not an incurable disease. We believe that EPS can be successfully treated after accurate diagnosis.

References

  1. Rottembourg J, Gahl GM, Poignet JL, et al. Severe abdominal complications in patients undergoing continuous ambulatory peritoneal dialysis. Proc Eur Dial Transplant Assoc 1983; 20:236–42.
  2. Pusateri R, Ross R, Marshall R, Meredith JH, Hamilton RW. Sclerosing encapsulating peritonitis: report of a case with small bowel obstruction managed by long-term home parenteral hyperalimentation, and a review of the literature. Am J Kidney Dis 1986; 8:56–60.
  3. Hendricks P, Ho-dac-Pannekeet M, van Gulik TM, et al. Peritoneal sclerosis in chronic peritoneal dialysis patients: analysis of clinical presentation, risk factors, and peritoneal transport kinetics. Perit Dial Int 1997; 17:136–43.
  4. Hawley C, Wall D, Johnson D, et al. Recovery of gastrointestinal function following renal transplantation in a patient with sclerosing peritonitis secondary to continuous ambulatory peritoneal dialysis. Am J Kidney Dis 1995; 26:658–61.
  5. Junor BJR, McMillan MA. Immunosuppression in sclerosing peritonitis. Adv Perit Dial 1993; 9:187–89.
  6. Bhandari S, Wilkinson A, Sellars L. Sclerosing peritonitis: value of immunosuppression prior to surgery. Nephrol Dial Transplant 1994; 9:436–7.
  7. Mori Y, Matsuo S, Sutoh H, Toriyama T, Kawahara H, Hotta N. A case of a dialysis patients with sclerosing peritonitis successfully treated with corticosteroid therapy alone. Am J Kidney Dis 1997; 30:275–8.
  8. Kittur DS, Korpe SW, Raytch RE, Smith GW. Surgical aspects of sclerosing encapsulating peritonitis. Arch Surg 1990; 125:1626–8.
  9. Bowers VD, Ackermann JR, Richardson W, Carey LC. Sclerosing peritonitis. Clin Transplant 1994; 8:369–72.
  10. Smith L, Collins JF, Morris M, Teele RL. Sclerosing encapsulating peritonitis associated with continuous ambulatory peritoneal dialysis: surgical management. Am J Kidney Dis 1997; 29:456–60.
  11. Kawanishi H, Harada Y, Sakikubo E, Moriishi M, Nagai T, Tsuchiya S. Surgical treatment for sclerosing encapsulating peritonitis. Adv Perit Dial 2000; 16:252–6.
  12. Kawaguchi Y, Kawanishi H, Mujais S, Topley N, Oreopoulos DG. Encapsulating peritoneal sclerosis: definition, etiology, diagnosis, and treatment. International Society for Peritoneal Dialysis Ad Hoc Committee on Ultrafiltration Management in Peritoneal Dialysis. Perit Dial Int 2000; 20(Suppl 4): S43–55.

Corresponding author:

Hideki Kawanishi, md, Akane Foundation, Tsuchiya General Hospital, 3–30 Nakajima-cho Naka-ku, Hiroshima 730-8655 Japan.