|Year : 2015 | Volume
| Issue : 2 | Page : 329-334
|Encapsulating peritoneal sclerosis: Presentation without preceding symptoms
Petros Yiannoullou, Kavitha Kanesalingam, David van Dellen, Titus Augustine
Department of Renal and Pancreas Transplantation (a UK referral center for Encapsulating Peritoneal Sclerosis), Manchester Royal Infirmary, Manchester, United Kingdom
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|Date of Web Publication||3-Mar-2015|
| Abstract|| |
Encapsulating peritoneal sclerosis (EPS) is a rare but devastating complication of peritoneal dialysis (PD), resulting in malnutrition and ultimately overt intestinal obstruction. We present the case of a 71-year-old man diagnosed with EPS incidentally at laparotomy for removal of PD catheter following an episode of PD peritonitis. He had been treated with continuous ambulatory PD for 18 months. He presented with anasarca and did not exhibit persistent symptoms of gastrointestinal dysfunction to suggest the EPS. Computed tomography scanning obtained 18 days prior to confirmation of the diagnosis did not demonstrate any features suggestive of EPS, highlighting a deficiency in the sensitivity of the diagnostic investigations. Management of the EPS is typically complicated by late diagnosis and concomitant malnutrition. This case highlights both the insidious nature of the EPS and a management problem to the surgeon faced with an unexpected abdominal cocoon. It further accentuates the necessity for increasingly sensitive diagnostic investigations to allow earlier diagnosis, thereby facilitating successful treatment.
|How to cite this article:|
Yiannoullou P, Kanesalingam K, van Dellen D, Augustine T. Encapsulating peritoneal sclerosis: Presentation without preceding symptoms. Saudi J Kidney Dis Transpl 2015;26:329-34
|How to cite this URL:|
Yiannoullou P, Kanesalingam K, van Dellen D, Augustine T. Encapsulating peritoneal sclerosis: Presentation without preceding symptoms. Saudi J Kidney Dis Transpl [serial online] 2015 [cited 2020 Jun 2];26:329-34. Available from: http://www.sjkdt.org/text.asp?2015/26/2/329/152499
| Introduction|| |
Peritoneal dialysis (PD) remains an important prevalent modality for renal replacement therapy (RRT), with approximately 5000 patients with end-stage renal failure (ESRF) currently dialyzing through this modality in the United Kingdom.  Encapsulating peritoneal sclerosis (EPS) is an uncommon but under-recognized sequelae of prolonged PD, with a reported incidence of 0.7-3.3% in multiple published series. ,,, It is a potentially catastrophic condition characterized by progressive thickening of the peritoneal membrane coupled with concomitant bowel encapsulation, which ultimately results in symptoms of bowel dysfunction or obstruction.  The exact triggers for this condition remain controversial, but several potential etiologies have been postulated, including severe non-resolving or recurrent PD-related peritonitis  besides peritoneal and abdominal malignancies, tuberculosis, systemic autoimmune diseases, β-blockers, talc or particulate matter exposure (intraperitoneal disinfectants) and after liver transplantation. ,,,
A potential "two hit" hypothesis for the pathogenesis of this condition has evolved. This hinges on an initial insult resulting from a persistent peritoneal exposure to prolonged PD, particularly with hypertonic glucose solutions, and the second hit is peritonitis or rapid withdrawal of PD, such as after transplantation or rapid conversion to hemodialysis (HD) as the mode of RRT. ,, Symptoms result from both the development of intestinal adhesions coupled with unregulated fibrosis, with pathognomonic signs of bowel encapsulation and cocooning presenting as symptoms of early satiety, vomiting and, ultimately, frank intestinal obstruction. This may result in clinical signs of significant patient malnutrition. ,,
The disease provides distinct clinical difficulties in early recognition, diagnosis and appropriate treatment, which are exacerbated by the insidious pattern of disease onset. This is characterized by initial symptoms that are vague and include abdominal discomfort, early satiety and altered bowel habit. The patient may also experience "membrane failure" characterized by decreased solute or fluid removal, requiring alterations to dialysate constituents or even eventual conversion to HD.  The EPS onset is characteristically gradual, but there are reports of fulminant EPS following foreign body reactions or bacterial or fungal peritonitis. ,,
We report a case of acute encapsulating peritoneal sclerosis following a fulminant case of PD peritonitis, which was diagnosed at the time of laparotomy in the absence of any preceding objective obstructive symptoms to suggest the diagnosis.
| Case Report|| |
A 71-year-old man of Indo-Asian origin with ESRF secondary to hypertension was admitted with increasing generalized edema coupled with fluid retention and weight gain over a one-month period.
The patient had been using continuous ambulatory peritoneal dialysis (CAPD) as his chosen modality of RRT for the preceding 18 months. He had previously experienced two short-duration (72 h) episodes of PD peritonitis secondary to coagulase-negative Staphylococcus over this period. His other significant past medical history included a cerebrovascular accident and ischemic heart disease.
On clinical examination, there was evidence of generalized edema with a 13 kg gain above his previous "dry weight." There were no associated abdominal signs at this juncture. Fluid restriction resulted in a reduction of anasarca, but there was a persistence of bilateral scrotal edema. A computed tomography (CT) scan with intraperitoneal contrast was undertaken and extra-peritonealization of the dialysate was excluded. An incidental diagnosis of bilateral inguino-scrotal hernias was made. There was no comment at this juncture of any intrabdominal abnormalities or features consistent with the EPS. On this basis, the patient underwent bilateral open inguinal hernia Liechtenstein mesh repairs. The CAPD was continued as the modality of RRT in the perioperative period.
His post-operative recovery was complicated by constipation and vomiting, both of which symptoms were ascribed to a post-operative ileus. Blood tests demonstrated a peak in C-reactive protein (CRP) to 427 mg/L, with a leukocytosis of 16 × 10 9 /L. A repeat CT scan demonstrated global dilatation of the colon with gas and feces present in the rectum but with no overt evidence of obstruction, peritoneal thickening or calcification. The patient recovered gastrointestinal function following conservative management for his post-operative ileus. A CT scan was repeated 14 days post-operatively, which confirmed resolution of the colonic dilatation, once again with no signs of any peritoneal thickening, calcification or loculated fluid collections to suggest the possibility of the EPS [Figure 1]. The patient underwent a period of intense rehabilitation and was discharged 26 days post-operatively, still continuing with CAPD.
|Figure 1: Normal CT scan, without any evidence of the encapsulating peritoneal sclerosis taken 18 days prior to diagnosis.|
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He presented six days later with abdominal pain, fever and cloudy dialysate, in keeping with dialysis-related peritonitis. PD fluid analysis confirmed the presence of Pseudomonas aeruginosa and the patient was commenced on anti-pseudomonal intravenous antibiotic therapy (gentamicin and vancomycin adjusted to his renal function). He had clinical improvement and was subsequently elected for a laparotomy for removal of his PD catheter and a planned subsequent conversion to HD.
At laparotomy, dense adhesions with a classical abdominal cocoon were encountered along with a small volume turbid fluid collection [Figure 2]. In view of the unexpected diagnosis, lack of consent and absence of any overt obstructive symptoms, formal enterolysis and peritonectomy were not performed at this juncture. The PD catheter was removed and paracolic and pelvic tube drains were inserted following thorough saline lavage. Peritoneal biopsies were taken and analyzed histologically. These demonstrated inflammatory cell infiltrate and vascular proliferation consistent with acute peritonitis as well as dense fibrosis of the peritoneal tissue, in keeping with the EPS.
|Figure 2: Appearances of encapsulating peritoneal sclerosis at initial laparotomy with pathognomonic cocoon entirely incorporating the small intestine with a thickened parietal peritoneum.|
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The patient was initiated on total parenteral nutrition (TPN) peri-operatively with commencement of enteral feeding within 48 h in view of the absence of vomiting, abdominal pain and constipation. This was all done to ensure pre-operative nutrition for a potential subsequent enterolysis and peritonectomy.
He continued to tolerate oral diet with a good appetite, until he unfortunately sustained a head injury secondary to a mechanical fall in the ward 16 days following surgery. This resulted in a large acute on chronic subdural hemorrhage not amenable to intervention, ultimately resulting in cardiorespiratory arrest and death.
| Discussion|| |
EPS is a condition that, despite increasing awareness in the nephrological and surgical communities, remains difficult to diagnose due to variability in clinical presentation, lack of sensitive imaging techniques as well as the absence of any reliable clinically applicable biomarkers. The disease's poor prognosis is at least partly explained by the diagnostic difficulties thus demonstrated. However, there are certain pathognomonic features, including the abdominal cocoon, which aids in clinical confirmation at the time of laparotomy. Furthermore, the presence of complete intestinal cocooning in the absence of any overt clinical signs and symptoms of gastrointestinal obstruction has not previously been described.
The natural course and evolution of the EPS has not yet been well characterized, and this case raises the question of whether an asymptomatic precursor of the EPS, which may cause non-specific symptoms in patients on PD, exists, particularly over a longer treatment duration. This may be of importance to surgeons lacking experience in both the diagnosis and appropriate management of this unexpected challenging problem at emergency laparotomy prior to formal diagnosis and specialist center referral. Presently, a diagnosis of the EPS requires fulfillment of two criteria, namely that the patient has symptoms of gastrointestinal dysfunction and that this is secondary to bowel encapsulation with specialist center referral mandated for optimal disease management. , However, our case highlights the potential deficiency in both the sensitivity and specificity of these criteria and poses the question of early screening of asymptomatic patients on PD either utilizing serial radiology or potential novel biomarkers.
Duration of PD represents the greatest reported predictive risk factor for the EPS development, with treatment durations of greater than four years associated with an approximately 8% of disease development.  Our patient only had a total PD exposure of 19 months, demonstrating an unusually rapid onset temporally, although he did experience recurrent episodes of dialysis-associated peritonitis, including a documented episode secondary to Pseudomonas aeruginosa. Pseudomonal PD peritonitis is associated with poor outcomes, including CAPD abandonment, and has been implicated as an important causative factor of the EPS. ,
CT remains currently accepted as the confirmatory tool of choice in well-established cases of the EPS.  CT signs of peritoneal thickening and calcification, loculated fluid, small bowel feces sign and small bowel obstruction have been proposed as predictors of abdominal cocoon.  However, early detection and radiological prediction of EPS remains a significant challenge, with one study reporting that only 31% of the EPS patients had abnormal CT findings four months prior to the EPS diagnosis.  Screening of asymptomatic PD patients with abdominal CT is currently not indicated.  It is therefore clear that there is currently a paucity of suitable imaging tools, which in turn may impact on timely diagnosis and referral to specialist centers.
In our case, two abdominal CT scans were performed in the month immediately preceding the diagnosis of the EPS, both of which failed to reveal any pathognomonic features of the EPS. This further demonstrates the deficiencies of CT in the EPS detection and highlights the necessity of a more sensitive and discerning diagnostic tool. Dynamic Cinemagnetic resonance imaging (MRI) has yielded some exciting results in a recent pilot study,  although further work is required to fully characterize its ability to mandate routine use. This methodology elegantly demonstrated abnormalities in intestinal movement patterns within the EPS group, suggesting that this modality may facilitate the earlier diagnosis of the EPS.
The incidental finding of an abdominal cocoon at a formal laparotomy for PD catheter removal, coupled with the absence of obstructive symptoms, raises a significant problem and requires a robust management protocol. It appears from longitudinal observational studies that these patients will almost invariably develop signs of gastrointestinal compromise over prolonged periods as well as the fact that outcomes are definitely improved in cases operated on in the absence of overt obstructive symptoms or nutritional deficiency. However, these benefits need to be countered by the risk of mortality and morbidity in these patients, even in the elective scenario. 
Investigators have previously postulated the existence of four categories of the EPS: Pre-EPS, an inflammatory stage, encapsulation and, finally, obstruction with a temporal progression through the various stages.  Coupled with the EPS development risks inherent to a change in dialysis modality, it is therefore likely that our patient would have progressed on to the obstructive stage in the months following the RRT modality change. Heightened clinical suspicion in the setting of PD peritonitis with or without bowel symptoms is necessary, regardless of the PD duration, to facilitate early investigation and diagnosis. Indeed, early identification allows management to proceed in the setting of a satisfactory nutritional state giving better outcomes. The search for an accurate diagnostic tool to allow early identification and timely management of these patients remains crucial to the successful management of this potentially life-threatening illness.
| Acknowledgment|| |
The authors thank Linda Birtles for technical assistance.
Conflict of interest: None declared.
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Dr. Petros Yiannoullou
Department of Renal and Pancreas Transplantation, Manchester Royal Infirmary, Manchester
[Figure 1], [Figure 2]
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