Sclerosing encapsulating peritonitis secondary to CAPD: the effect of fibrotic debridement on further dialysis

S. KLIMOPOULOS, IE. KATSOULIS, V. MARGELLOS* and N. NIKOLOPOULOU*

2nd Department of Surgery and Department of Nephrology* Evangelismos General Hospital, 45-47 Ipsilanlou str, 10676, Athens, Greece

    

Introduction

Patients and methods

Results

 

Discussion

References

J.R.Coll.Surg.Edinb., 47 April 2002, 485-490

Background: Sclerosing peritonitis (SCP) is a complication of continuous ambulatory peritoneal dialysis (CAPD) and is characterized by progressive fibrosis of the peritoneum. Entrapment of the intestine in a fibrous sac resulting in complete intestinal obstruction is called sclerosing-encapsulating peritonitis (SEP) and represents the most severe form of the disease. Various reports have been pessimistic regarding the surgical outcome when SEP has caused complete intestinal obstruction. Continuation of CAPD after laparotomy is generally considered not feasible. The aim of this article is to present our experience in the surgical management of SEP and, in particular, in the postoperative continuation of CAPD. Material and Methods: Seventeen consecutive patients with SCP among 175 patients undergoing CAPD during a period of 14 years in a single Unit were retrospectively reviewed. Two groups of patients were recognized. The SCP group included 9 patients with incomplete intestinal obstruction that were treated with single peritoneal catheter removal and switching to haemodialysis. The SEP group included 8 patients with complete obstruction that necessitated laparotomy for surgical debridement of the fibrotic tissue and release of the intestinal loops. Results: Switching to haemodialysis improved the majority of the group of patients. In 2 of the SEP group of patients (early in the series), where enterectomy was inevitable, performance of an intestinal anastomosis resulted in leakage with subsequent fatal outcome. Two of the SEP group of patients were transferred to haemodialysis after the laparotomy. In the remaining 4 SEP patients (50%), exposure of a significant portion of active peritoneal surface was achieved - called “neoperitonization”-and allowed effective continuation of peritoneal dialysis for an average duration of 16 months (range 1-32). Conclusions: In patients with SEP, careful release of the intestinal loops avoiding enterectomies and even inadvertent intestinal wounds is mandatory. Continuation of peritoneal dialysis after meticulous debridement and removal of the fibrotic tissue is possible and may be effective. To the best of our knowledge, there have not been previously reported cases of continuations of CAPD after laparotomy for SEP.

Key Words: sclerosing peritonitis, sclerosing-encapsulating peritonitis, peritoneal dialysis.

INTRODUCTION

Sclerosing peritonitis (SCP) is a relatively rare but serious complication of continuous ambulatory peritoneal dialysis (CAPD). It has been estimated to affect between 0.54 and 7.3% of CAPD patients. 1,2,3,4 Sclerosing peritonitis is a progressive fibrosis of both the visceral and the parietal peritoneal surfaces resulting in reduced ultrafiltracion capacity, dysfunctional peristalsis of the intestine and/or luminar obstruction.5 The severity of the peritoneal lesion and the subsequent clinical features vary. The disorder, therefore, is best considered as a group of peritoneal sclerosing syndromes. 6 The most severe condition, in which the small bowel is enclosed in a sac or “cocoon” of fibrous tissue, is called sclerosing-encapsulated peritonitis (SEP). 7,8 The aetiology of this condition is unclear but is believed to be multifactorial.8 The most important factors seem to be the length of time on peritoneal dialysis and previous episodes of peritonitis.6 The early clinical features are non-specific and include colicky abdominal pain, weight loss, progressive impairment of peritoneal ultra-filtration capacity and, occasionally, a bloody dialysis effluent.6 Partial or intermittent bowel obstruction and malnutrition predominate and without treatment the condition may lead to complete intestinal obstruction, small-bowel necrosis and enterocutaneous fistulas.9 The clinical outcome, when SEP has caused complete intestinal obstruction, is regarded as poor. 9,10

The present report includes a series of 17 patients with SCP among 175 patients undergoing CAPD during a period of 14 years in a single Unit. The surgical treatment corresponded to the severity of the condition, ranging from single catheter removal to laparotomy for intestinal release. We present our experience in the surgical management of SEP and a review of the literature on the subject.

PATIENTS AND METHODS

Between April 1983 and July 1997, 175 patients with end-stage renal failure were treated with CAPD in our Peritoneal Dialysis Unit. During that period, seventeen patients (9.7%) developed SCP secondary to CAPD. Two groups of patients were recognized. The SCP group included nine patients with incomplete intestinal obstruction. The SEP group, characterized by complete intestinal obstruction due to encapsulation of the small bowel in a sac of fibrous tissue, included eight patients. Diagnosis was confirmed either by imaging techniques or by laparotomy findings. The clinical records were retrospectively reviewed including the clinical presentation, the management and the outcome in all 17 patients in this study (Table I). The average follow-up of the patients was 45 months (range 0-125 months). The Mann- Whitney U test and the Independent t test were used to evaluate the differences between patients with incomplete intestinal obstruction and patients with complete obstruction. Analyses were performed with the software program SPSS 8.0 for Windows.

RESULTS

There were 12 males and 5 females ranging in age from 14 to 65 (average age, 47.5 years). These 17 patients developed SCP 10 to 145 months (average 60.6 months) after starting CAPD. One patient had been transferred to haemodialysis 4 months before the time of diagnosis due to loss of ultrafiltration capacity.

The average frequency of peritonitis before developing sclerosing peritonitis was 4.5 episodes. One patient had no history of peritonitis, although it is possible that he might have experienced subclinical peritonitis during the study period. Peritonitis incidence in patients with SCP was one episode every 13.4 months on CAPD. The overall episodes of peritonitis were 77. In Table 2 the incidence of the causative pathogens is demonstrated. The Staphylococcus species were the most common pathogens (75%) in 57 episodes of peritonitis. In 16 episodes with negative cultures and in 4 episodes where a culture was not obtained peritonitis was treated empirically. In the patient without a history of peritonitis acetate-buffered dialysate was a possible aetiologic factor.

Acetate-buffered dialysate had been used totally in 41% of the patients in this study, whilst medication with b-blockers had been used in 47% of the patients. None of these two risk factors appeared to have a significant relationship with more severe presentation of the disease in this study. Furthermore, povidone iodine was exclusively used as a disinfectant in all our patients.

In the 9 patients of the SCP group, intestinal obstruction was intermittent and clinical manifestations included colicky abdominal pain, nausea, anorexia leading to malnutrition and weight loss (patients 7,8,9,10,11,13,14,16,17).

In these patients single catheter removal and switching to haemodialysis improved the patient’s clinical status, except for one patient who continued to suffer from malnutrition and subsequently died from hyperkalaemia. Furthermore, 2 patients experienced transient episodes of subacute intestinal obstruction, none of which required a surgical intervention. The average survival in this group of patients was 58.2 months (range 4-125 months) during the study period.

The 8 patients of the SEP group, including the patient on haemodialysis, developed complete intestinal obstruction necessitating operative intervention (patients 1,2,3,4,5,6,12,15). At laparotomy, the diagnosis of SEP or “abdominal cocoon” was confirmed. The parietal and visceral peritoneum was thickened, tanned and wrinkled. The small bowel and accompanying mesentery were partially or totally drawn together within a fibrous cocoon-like sac in a small area of the peritoneal cavity, the remainder being filled with ascitic fluid. Within this cocoon thick fibrous adhesions formed between the loops of bowel. Meticulous peeling of the fibrotic membrane, adhesiolysis and release of the intestinal loops was attempted in all cases resulting in disclosure of a significant portion of normal, active peritoneal surface. In the first patient of this group, surgical debridement of the fibrotic tissue was followed by removal of the catheter and the patient was transferred to haemodialysis. Early in the series, in 2 cases with SEP intestinal resection and primary anastomosis was performed. Unfortunately, both patients died from sepsis after anastomotic leakage. The patient with SEP while on haemodialysis, after laparotomy for intestinal release, continued haemodialysis. In the remaining 4 patients with SEP (patients 2,3,6,12), a meticulous peeling of the fibrotic membrane was feasible, resulting in disclosure of normal active peritoneal surface. In all those patients, ultrafiltration capacity improved allowing successful continuation of peritoneal dialysis for an average time of 16 months (range 1-32 months). In our series, this treatment was chosen due to the particular geography of our country, which has many small islands without Haemodialysis Units. The average survival of patients with SEP was 30.3 months (range 0-91 months) in the study period. Nevertheless, the difference in survival between these patients and patients with incomplete intestinal obstruction was not significant (p=0.13).

Of all the risk factors, only medication with b-blockers was found to have a significant difference between patients with incomplete intestinal obstruction and patients with SEP (77.8% and 12.5%, respectively, p=0.02). This finding might mean a possible difference in the pathogenesis of the two forms of the disease, but this has yet been proved.

Case No Sex Age Aetiology of renal failure CAPD duration (months) Episodes of peritonitis Type of buffer User of b-blockers
1 Male 61 Chronic interstitial nephritis 63 2 Lactate (L) No
2 Male 38 Vascular disease 72 5 Acetate (A) No
3 Male 65 Chronic glomerulonephritis 92 12 A No
4 Female 35 Chronic interstitial nephritis 87 3 A+L No
5 Female 59 Chronic interstitial nephritis 77 6 L No
6 Female 34 Idiopathic 11 2 L No
7 Female 43 Chronic glomerulonephritis 83 6 L No
8 Male 40 Chronic interstitial nephritis 47 4 L Yes
9 Male 51 Chronic interstitial nephritis 73 5 L Yes
10 Male 61 Diabetic nephrology 30 7 L Yes
11 Male 14 Chronic interstitial nephritis 67 4 L Yes
12 Male 39 Systematic lupus erythematosus 145 8 A+L Yes
13 Male 53 Chronic interstitial nephritis 34 0 A No
14 Male 48 Chronic glomerulonephritis 44 2 A Yes
15 Male 63 Chronic glomerulonephritis 10 2 A No
16 Male 38 Vascular disease 56 6 L Yes
17 Female 65 Chronic interstitial nephritis 39 3 L Yes

Table 1: Characteristics of continuous ambulatory peritoneal dialysis in patients with sclerosing peritonitis

Pathogens No of Cases
Staphylococcus
aureus
19
Staphylococcus
epidermidis
24 
Pseudomonas 4
Escherichia
coli

 

Citrobacte
Diphtheoides
Euterococcus 
Serratia 
Streptococcus faecalis
Streptococcus viridans
Streptococcus
Candida albicans
1 case of each     
Negative cultures 16
No cultures 4

Table 2: Causative pathogens in 77 episodes of peritonitis

DISCUSSION

Sclerosing peritonitis remains an uncommon and enigmatic condition affecting the peritoneum. Although the idiopathic variety occurs in young women in warm climates, it has also been described in middle-aged men.11 It is a recognized complication of treatment with practolol and has been associated with luteinized thecoma of the ovary, sarcoidosis, systemic lupus erythematosus and ventriculo-peritoneal shunting.12-16 In patients with end-stage renal failure, a form of SCP has been associated with continuous ambulatory peritoneal dialysis (CAPD). It has been estimated to affect between 0.54 and 7.3% of CAPD patients. 1,2,3,4 Gandhi et al in 1980 first described the condition as a marked sclerotic thickening of the peritoneal membrane in 5 patients on intermittent peritoneal dialysis, but almost all the cases subsequently reported have been associated with CAPD.17 Duration of peritoneal dialysis is considered a significant risk factor. 4,6,7,18 In a recent report, Rigby and Hawley (1998) found that the prevalence of SCP increased progressively with the duration of peritoneal dialysis, being 1.9, 6.4, 10.8, and 19.4% for patients on dialysis for more of 2, 5, 6 and 8 years, respectively. 18 Nevertheless, the condition has been occasionally reported in patients on CAPD for as little as 3 months and even after only 7 courses of PD.7,19 The aetiology is unclear but it is believed to be multifactorial. 8 A variety of factors have been implicated in contributing to the clinical entity. These include the peritoneal catheter, 6,9 recurrent episodes of bacterial or fungal peritonitis, use of certain types of b-blockers, acetate buffer, particulate matter present in the dialysate, use of alcoholic chlorexidine as disinfectant, intraperitoneal use of vancomycin, hyperosmolarity of the dialysate and the heat sterilization process used in dialysis fluid manufacture. 1, 2, 6-9, 12, 21-25

From the above-mentioned risk factors, recurrent episodes of bacterial or fungal peritonitis appear to be the most significant. 1,2,6,7 Recurrent bacterial peritonitis is commonest in this group.1, 3,10 No specific organisms are implicated but staphylococcus (especially aureus) and streptococcus infection is commonest. 1 Other less common pathogens are Escherischia coli, pseudomonas and fungi. In our series, the average frequency of peritonitis, before developing SCP, was 4.5 times; one patient had no history of peritonitis. Regardless of the predisposing factor, the most plausible pathophysiologic event in the development of SCP is an inflammatory process resulting in loss of the mesothelial layer of the peritoneum and fibroconnective tissue proliferation. 8 Mesothelial cells damaged by any of the above-mentioned risk factors secrete cytokines and interleukin-6 stimulates the fibroblasts to produce collagen. 8 The inflammatory process results in deposition of fibrin, hyalinization and subsequently fibros and replacement of the normal mesothelial lining of the peritoneum. There is a spectrum of macroscopic changes ranging from opacification to a wrinkled, dry, tanned appearance of both the visceral and the parietal peritoneal surfaces. 8 Sclerosing encapsulating peritonitis is a more severe condition in which the small bowel is partially or totally entrapped in a fibrous cocoon-like sac and adhesions develop between the intestinal loops. Some patients develop variable calcification of the peritoneum, which can be seen radiologically. Many, but not all, of these patients have hyperparathyroidism. 26

Clinically, partial or intermittent bowel obstruction, progressive impairment of peritoneal ultrafiltration capacity and malnutrition predominate. The early symptoms include colicky abdominal pain, anorexia, nausea, vomiting, fever, weight loss, peripheral oeldema, ascites and occasionally a bloody dialysis effluent. 6 Without treatment the condition may lead to complete intestinal obstruction and even more ominous complications of small-bowel necrosis and enterocutaneous fistulas. 9

Diagnosis of SCP at an early stage can be suspected in high-risk patients from the typical clinical features and may be suggested by imaging techniques. Plain abdominal radiographs may show centrally located, gas-filled and sometimes dilated loops of bowel. 28 However, radiographs may be normal, in a patient who clinically appears to be obstructed. 28 Small bowel barium studies may show varying lengths of adherent intestinal loops, proximal small bowel dilatation and an increased transit time. 28 Characteristic ultrasonographic features comprise increased small bowel peristalsis, tethering of the bowel to the posterior abdominal wall, intraperitoneal echogenic strands and in the late stages of the disease formation of a previsceral membrane. 29 Optimal visualization of these features in the early stages of the disease is obtained with the abdomen full of dialysate. Finally, computerised tomography (CT) findings include loculated fluid, adherent bowel loops and thickened peritoneal membranes. 30 Campbell et al (1991), evaluated the imaging techniques in the early identification of SCP in a series of 15 patients and suggested that ultrasound and colon transit time studies are safe and useful screening procedures and that CT might reasonably be used as a less frequent screening tool or to investigate patients with minor abnormalities on other screening tests. 6

Management of SCP, in the absence of intestinal obstruction involves cessation of CAPD, catheter removal and transfer to haemodialysis. When oral intake is not possible due to intestinal obstruction, institution of total parenteral nutrition is recommended and, in the case of SEP, surgical release of the intestine should be attempted, if possible. If diagnosis occurs before complete obstruction, the sclerotic membrane frequently can be separated from the small intestine. Since progression of the sclerosing process cannot be always avoided with these measures, some investigators have reported improvement after renal transplantation or with immunosuppressive therapy alone, although others have not confirmed this. 8,10 Hawley et al (1995) described the rapid and dramatic improvement in gastrointestinal function after successful transplantation in a patient with SCP secondary to CAPD and postulated that the anti-inflammatory effect of the immunosuppressive agents was the most important factor leading to the patient’s recovery. 31 Bhandari et al (1994) suggested that immunosuppresion in CAPD-related SCP improves operability and should be considered as initial treatment with a view to subsequent surgery. 32 Junor and McMillan (1993) observed that among their 15 patients with SEP, the only patients who survived were those who went on to renal transplantation and/or those who received immunosuppression. 33

When SEP has caused complete intestinal obstruction the outlook is poor. 9,10,34 Rottembourg et al (1983) suggested that successful surgical release is almost impossible when SEP has caused complete intestinal obstruction, and mortality exceeds 60%. The high mortality rate is related to bowel obstruction or complications of surgery. 9,10,34 Kittur et al (1990) believe that an aggressive surgical approach may be more hazardous than long-term nutritional management and claim that laparotomy must be performed only if necrotic intestine is present. 9 In the latter case, enterostomies are preferable to primary anastomoses. Pusateri et al (1986) reported the development of enterocutaneous fistulae after enterectomy for a case of SEP, managed success-fully with long-term home parenteral hyperalimentation.34

In contrast, successful outcomes of surgical treatment have been reported. Bowers et al (1994) have reported three cases in which the fibrotic and sclerosing process progressed after termination of peritoneal dialysis and successful renal transplantation. 35 The 3 cases were relieved of mechanical bowel obstruction by adhesiolysis and no recurrence of fibrosis and intestinal obstruction occurred within a one-year period of follow-up. Smith et al (1997), reported two patients with SEP, where progressive peeling away of the sclerotic and encapsulating membrane was achieved and both patients survived with good outcomes. 36 The authors suggested consideration of surgery as the preferred treatment in SEP, rather than life-long parenteral nutrition. 36

In a recent report, Celicout et al (1998) reviewed the early and late results of surgical management in 32 cases with SEP and suggested that: (1) resection of the sclerotic membrane should be attempted when feasible, (2) in case of inadvertent intestinal injury(ies), the most proximal one should be brought out as a stoma and partial resections should not be anastomosed primarily, but (3) no surgical treatment is required in ascites, asymptomatic SEP or subacute intestinal obstruction. 37 The normal healing of bowel anastomoses may well be hampered by the peritoneal thickening, resulting in an increased incidence of anastomotic leaks and enterocutaneous fistulas. 9 This suggests that, when patients with SEP need intestinal resection, primary anastomosis may be unwise and complications may be avoided by construction of an enterostomy. 9 The malnutrition that is common in these patients may further delay intestinal healing and should be treated by long -term parenteral hyperalimentation. 9,34

In our series of 8 patients, including one patient on haemodialysis, developed complete intestinal obstruction necessitating operative intervention. Unfortunately in two cases, where intestinal resection was inevitable, the small bowel was primarily anastomosed resulting in anastomotic leakage and death from sepsis. In the other patients, adhesiolysis and meticulous resection of the sclerotic membrane were uneventful. In 4 of these patients with SEP, ultrafiltration capacity improved after peeling of the fibrotic membrane, allowing successful continuation of peritoneal dialysis for an average time of 16 months (range1-32 months). To the best of our knowledge, there have not been previously reported cases of continuation of CAPD after laparotomy for SEP. In 1992, presenting our early experience in surgical treatment of SEP, we proposed the term “neoperitonization” for the meticulous peeling of the fibrotic membrane, resulting in disclosure of functional perito-neal surface. 38

In conclusion, SCP on patients in CAPD must be recognized early and, if it is not complicated by complete intestinal obstruction, should be treated conservatively. In SEP, where surgical release of the intestinal obstruction is necessary, surgeons must be aware of the decreased intestinal healing process and should avoid enterectomies and inadvertent intestinal injury. In cases where that is not possible, enterostomies are preferable to primary anastomoses. Finally, the present report shows that continuation of peritoneal dialysis after surgical debridement of the fibrotic tissue may be effective.

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Copyright: 16 January 2002

Correspondence: Dr. Iraklis E. Katsoulis, Royal Lancaster Infirmary, Ashton Road, Lancaster LA1 4RP, E-mail: hrkats@yahoo.co.uk