Surgical aspects of sclerosing encapsulating peritonitis

Sclerosing encapsulating peritonitis (SEP) is associated with the administration of beta-blocking agents as well as continuous ambulatory peritoneal dialysis. The predisposing factors in the latter group are recurrent peritonitis, presence of acetate in the dialysate, and antiseptics used during bag exchanges. We report a case of SEP following chronic ambulatory peritoneal dialysis and review the literature on this benign yet potentially lethal condition. Sclerosing encapsulating peritonitis frequently leads to intestinal obstruction, small-bowel necrosis, enterocutaneous fistulas, and malnutrition. There is a high incidence of anastomotic failure when a resection and primary intestinal anastomosis is performed in patients with SEP. Although SEP is not commonly reported in the surgical literature, its importance to surgeons is indicated by the fact that the overall mortality rate is close to 60% in patients with SEP who develop surgical complications.     

Sclerosing Peritonitis: Report of Three Cases

Sclerosing peritonitis is a dramatic complication of continuous ambulatory peritoneal dialysis and chronic peritoneal inflammation. Both visceral and parietal surfaces of the peritoneal cavity are involved. A thickened peritoneum encloses the small intestine in a «cocoon» formation which often leads to intestinal occlusion. CT scan may help obtaining an early diagnosis but diagnosis is often established with some delay or even at the time of laparotomy. Our report describes three cases of this uncommon peritoneal fibrosis syndrome which caused intestinal obstruction requiring surgical intervention.     

Sclerosing encapsulating peritonitis: report of a case with small bowel obstruction managed by long-term home parenteral hyperalimentation, and a review of the literature

Sclerosing peritonitis has recently emerged as a complication of peritoneal dialysis associated with a high morbidity and mortality. These patients experience the characteristic syndrome of nausea, vomiting, abdominal pain, partial small bowel obstruction, and impaired ultrafiltration. A pathologic finding is the replacement of mesothelial cells with a thick layer of nondistensible fibroconnective tissue. We report here a 58-year-old white woman who developed peritoneal sclerosis after 4 years of peritoneal dialysis, including 3 years of continuous ambulatory peritoneal dialysis. Risk factors included peritoneal exposure to low concentrations of formaldehyde and a 1-week exposure to long-dwell acetate dialysate. Laparotomy for partial small bowel obstruction with resection of the involved segment was complicated by enterocutaneous fistulae, which improved only on cessation of oral intake and treatment with home parenteral nutrition. We have reviewed the literature to find 20 cases of sclerosing peritonitis in patients on peritoneal dialysis. A 78% mortality rate is reported in cases that had surgical intervention. We conclude that the use of long-term parenteral nutrition with cessation of oral intake may be necessary in the management of sclerosing encapsulating peritonitis.     

Sclerosing peritonitis: report of three cases

Sclerosing peritonitis is a dramatic complication of continuous ambulatory peritoneal dialysis and chronic peritoneal inflammation. Both visceral and parietal surfaces of the peritoneal cavity are involved. A thickened peritoneum encloses the small intestine in a "cocoon" formation which often leads to intestinal occlusion. CT scan may help obtaining an early diagnosis but diagnosis is often established with some delay or even at the time of laparotomy. Our report describes three cases of this uncommon peritoneal fibrosis syndrome which caused intestinal obstruction requiring surgical intervention.     

Tamoxifen therapy in encapsulating sclerosing peritonitis in patients after kidney transplantation

Sclerosing encapsulating peritonitis (SEP) is a serious complication of long-term continuous ambulatory peritoneal dialysis (CAPD) associated with obstructive symptoms and sclerosis of the peritoneal membrane. We present two cases that were successfully treated with tamoxifen and corticosteroids. Case 1: A 40-year-old patient developed end-stage renal failure (ESRF) and was managed with CAPD. He was hospitalized with symptoms of small bowel obstruction. He underwent laparotomy confirming the diagnosis of SEP. The patient was given tamoxifen 20 mg twice a day. Case 2: A 55-year-old patient with ESRF secondary to membranous glomerulonephritis. After having a cadaveric renal transplant in 1978 that failed 20 years later, the patient returned to CAPD. Six years later he had an uneventful kidney transplant and the peritoneal dialysis catheter was removed. However, 8 months later he presented with symptoms of small bowel obstruction and gross blood stained ascites. He also underwent a laparotomy that confirmed the diagnosis of SEP after biopsy. The patient was started on 20 mg of tamoxifen twice a day. Both patients' symptoms were improved gradually with an increase of serum albumin and body weight. Tamoxifen may be useful in the treatment of patients diagnosed with SEP.     

Idiopathic encapsulating peritonitis: Report of two cases

This report presents two cases of young males who developed the rare idiopathic form of sclerosing encapsulating peritonitis (SEP) presented as partial bowel obstruction, both diagnosed during surgical treatment, with satisfactory outcomes. Sclerosing encapsulating peritonitis is a rare and enigmatic condition, characterized by intraperitoneal fibrosclerosis, which causes intestinal obstruction. It is a chronic entity with a poorly elucidated pathophysiology, leading to the constitution of a thick white nacreous fibrosis membrane that wraps the bowel in a concertina-like fashion with some adhesions configuring an intra-abdominal cocoon. Sclerosing encapsulating peritonitis is reported in a wide variety of patients, including those who have undergone peritoneal dialysis, young adolescent girls, cirrhotic patients after peritoneal-venous shunting, and patients treated with β-blockers. Nevertheless, the etiology of SEP remains obscure. This entity presents many difficulties in preoperative diagnosis because of its peculiar characteristics. Recognition of the SEP results in proper management and prevents unnecessary bowel resection. Regardless of cause, the treatment of the obstruction is surgical, with dissection of the encasing membrane from the intestine and separation of adherent loops of small bowel until they are laid free and returned to their normal configuration. The prognosis after appropriate surgical therapy is good, but depends on coexisting diseases.     

Sclerosing encapsulating peritonitis: regional changes of peritoneum

Sclerosing encapsulating peritonitis (SEP) is characterized by the diffuse appearance of marked sclerotic thickening of the peritoneal membrane. We experienced a case with SEP accompanied by regional changes of peritoneum. A 37-year-old woman with end-stage renal failure was started on continuous ambulatory peritoneal dialysis in 1985 and was transferred to hemodialysis in 1997. She was admitted because of ileus in 1998 with SEP and died of septicemia. The diagnosis of SEP was confirmed via the autopsy. The root of the mesentery was retracted and shortened. Since the peritoneal change was marked in the regions with free margin of mesentery and was less apparent in the regions not adhered to mesentery, it is indicated that mechanical stress also contributes to the occurrence of SEP. Since calcification and ossification were only seen in a free margin of small bowel from mesentery, it is suggested that there is a close relationship between calcification and ossification. Since fibrosis invaded into the muscle layer, dysfunction of bowel movement as well as bowel obstruction contributed to the appearance of ileus. It is suggested that mechanical stress by the root of mesentery which is retracted and shortened also contributes to the appearance of SEP.     

Encapsulating peritonitis. Report of 2 cases

Walled-off peritonitis which is a frequent occurrence in patients treated by continuous ambulatory peritoneal dialysis may also occur in cases where there is no obvious pathological factor. This was the case in the two patients reported. Both were male patients aged 28 and 32 years hospitalised and operated on for acute intestinal obstruction. Positive diagnosis was made at the time of operation. Decortication with total enterolysis was performed in the two cases. However, a jejunal fistula with a favorable outcome occurred in one case, investigation for acid fast bacilli was negative and histological examination was non specific.     

Continuous ambulatory peritoneal dialysis and sclerosing encapsulating peritonitis: tamoxifen as a new therapeutic agent?

Sclerosing encapsulating peritonitis (SEP) is a serious complication of long-term continuous ambulatory peritoneal dialysis (CAPD), very likely related to a persisting expression of the transforming growth factor beta1 (TGFbeta1) gene on peritoneal mesothelial cells. We report the case of a 67-year-old uremic woman who developed SEP eight years after being placed on CAPD, complicated by eight episodes of bacterial peritonitis. CAPD was therefore stopped and the patient transferred to hemodialysis. The diagnosis of SEP was confirmed by physical findings (vomiting, abdominal pain with palpable mass, ileus, cachexia) and CT data. The patient was treated with tamoxifen (10 mg/day) for three months, and gradually recovered, a subsequent CT showing a significant reduction of the thickness of peritoneal and intestinal loops. Tamoxifen probably interferes with TGFbeta1 and may be useful in the treatment of this CAPD complication.     

Sclerosing peritonitis

Sclerosing peritonitis (also described as sclerosing obstructive peritonitis, encapsulating peritonitis, obliterative adhesive peritonitis, abdominal cocoon syndrome) is not well-known by the general surgeon; most reviews of this subject occur in the nephrology literature. Peritoneal dialysis and continuous hyperthermic peritoneal perfusion are the main causes. No controlled study exists to evaluate the optimal therapeutic approach. Pre-operative diagnosis is difficult since the clinical signs and radiologic findings are non-specific. Nevertheless, the diagnosis should be considered when small bowel obstruction with proximal dilatation, ascites, and thickened or calcified peritoneum are seen on imaging. The surgeon should recognize sclerosing peritonitis grossly when it is encountered and adapt his surgical strategy accordingly.     

Advanced glycation end-products and peritoneal sclerosis

Long-term continuous ambulatory peritoneal dialysis (CAPD) often causes peritoneal fibrosis and sclerosis with a loss of function, and some CAPD patients develop sclerosing encapsulating peritonitis. Glucose-based peritoneal dialysis fluids readily produce glucose degradation products by heat sterilization, and glucose degradation products accelerate the formation of advanced glycation end-products (AGE) in the peritoneal cavity. The accumulation of AGE is observed in peritoneal mesothelial and submesothelial layers in CAPD patients, accompanied by enhanced expression of various growth factors and peritoneal thickening. The expression of transforming growth factor-beta1 (TGF-beta1), macrophage-colony stimulating factor, and vascular endothelial growth factor (VEGF) is distributed in the peritoneum similarly to that of AGE. In CAPD patients with low ultrafiltration (UF) capacity, peritoneal membrane is thickened owing to an increase in the number of cells such as fibroblasts and macrophages and collagen in the submesothelial layer. AGE is detected in the fibroblasts and macrophages as well as degenerated collagen. These cells in the submucosal layer are almost positive for the receptor for AGE (RAGE) and uptake AGE. The intensity of AGE accumulation and the expression of growth factors are associated with the severity of UF impairment. In fact, the accumulation of AGE and the expression of growth factors are recognized most markedly in the peritoneum of CAPD patients with low UF and sclerosing encapsulating peritonitis. In conclusion, long-time CAPD with heat-sterilized peritoneal dialysis fluid promotes AGE accumulation in the peritoneal membrane and alteration in peritoneal cell function and dialysis quality, followed by peritoneal sclerosis, and, finally, sclerosing encapsulating peritonitis.     

Sclerosing encapsulating peritonitis--a rare complication of ventriculoperitoneal shunts

Sclerosing encapsulating peritonitis (SEP) is a rare cause of bowel obstruction, most commonly associated with chronic ambulatory peritoneal dialysis. It has not previously been reported as a complication of ventriculoperitoneal (VP) shunts. We describe the clinical features of shunt-associated SEP and the important management considerations. Two children presented with small bowel obstruction after long-standing VP shunting of hydrocephalus. Neither had a history of recent shunt infection/revision nor evidence of shunt malfunction. In each case, the bowel was “cocooned” in a fibrous sheath with a notable absence of parietal adhesions. Both children were managed by meticulous adhesiolysis accompanied by shunt exteriorization. Both had prolonged ileus and required total parenteral nutrition. One required further laparotomy at which adhesiolysis was accompanied by irrigation with icodextrin 4% and systemic high-dose methylprednisolone. Weaning of steroids was accompanied by the introduction of azathioprine. A notable feature of intestinal obstruction because of SEP was severe pain despite adequate decompression. The restrictive “cocoon” that envelops the bowel prevents bowel dilatation and accounts for atypical radiologic findings in these cases.     

Differential Effect of sirolimus vs prednisolone in the treatment of sclerosing encapsulating peritonitis.

Differential effect of sirolimus vs prednisolone in the treatment of sclerosing encapsulating peritonitis Sir, A 56-year-old man presented in Autumn 2001 with increasing constipation,nausea, vomiting, abdominal swelling and pain, weight loss andmalaise. His original renal disease was chronic glomerulonephritis,presenting at end-stage renal failure in 1979. He was haemodialysedbriefly then received a cadaveric renal allograft which thenfailed in 1984, and so he started peritoneal dialysis. He switchedfrom continuous ambulatory peritoneal dialysis (CAPD) to APDin 1994, and was treated with PD for a total of 16 years and9 months. He had experienced nine episodes of peritonitis (threeStaphylococcus aureus, five coagulase-negative Staphylococcusand one sterile peritonitis) and     

Ten years' experience with continuous ambulatory peritoneal dialysis

Up to January 1989, 171 patients were trained at our center on continuous ambulatory peritoneal dialysis (CAPD), and 17 on continuous cyclic peritoneal dialysis (CCPD). Over 10 years, we have gained 5,068 patient-months experience. Patient survival was 60% and 31% at 5 and 10 years, respectively. In contrast, diabetics had a survival of 32% at 5 years. Major complications included 499 new episodes of peritonitis, 304 exit-site infections, 22 hernias, five bowel perforations, one hydrothorax, and three episodes of sclerosing encapsulating peritonitis. Our technique survival has been 62% and 40% at 5 and 10 years, respectively. We believe that CAPD is a viable dialysis technique for long-term treatment of chronic renal failure and it should be offered as an option to intermittent hemodialysis.     

Idiopathic sclerosing encapsulating peritonitis — Is a preoperative diagnosis possible? Report of three cases

Sclerosing encapsulating peritonitis (SEP) is a rare cause of intestinal obstruction that is usually diagnosed during a laparotomy; however, a preoperative diagnosis is possible. Sclerosing encapsulating peritonitis can be classified as primary or idiopathic and secondary types and only about 70 cases of idiopathic type have been reported since it was first described. It is characterized by a total or partial encasement of the small bowel by a thick fibrotic membrane. This report presents a series of three cases in which a diagnosis of idiopathic SEP was made preoperatively based on the clinical features and radiological findings, which were confirmed by a laparotomy and histopathology. All of the cases were successfully managed by the excision of the membrane. This report demonstrates that based on the clinical features and radiological investigations, in the absence of other plausible etiologies for intestinal obstruction, it is possible to suspect a preoperative diagnosis of SEP, thereby preventing a “surprise“ finding during a laparotomy and allowing for better management.     

Splenic infarction: an unrecognized cause of culture-negative peritonitis in a continuous ambulatory peritoneal dialysis patient

The appearance of cloudy peritoneal dialysis effluent in combination with the clinical manifestations of peritonitis usually heralds infectious peritonitis and the diagnosis is established with routine cultures. However, patients may present with culture-negative cloudy dialysate effluent and after ruling out atypical infectious etiologies, other intraabdominal causes should be considered in the differential diagnosis. A 57-year-old male with uremia on continuous ambulatory peritoneal dialysis with a pertinent history of restrictive cardiomyopathy and chronic atrial fibrillation suffered from abdominal pain and persistent culture-negative cloudy peritoneal dialysate. Clinical improvement was limited after empiric antibiotic treatment and all bacteriologic workups were negative. Isolated spleen infarction, a rare cause of culture-negative peritonitis, was disclosed by abdominal computed tomography. Spleen infarction is still an unrecognized cause of culture-negative peritonitis and is frequently overlooked. A high degree of suspicion is needed in CAPD patients with thromboembolism risk who present with unexplained persistent abdominal pain and cloudy PD effluent.     

Sclerosing peritonitis: the experience in Australia

Background: Sclerosing peritonitis (SP) is a rare but serious complication of peritoneal dialysis (PD). Small-bowel obstruction (SBO) due to encapsulation, dense adhesions, or mural fibrous is characteristic, often associated with peritonitis. The aim of the study was to determine the incidence, clinical features, effect of duration of dialysis, and other possible aetiological factors in severe SP. Methods: All dialysis units in Australia were surveyed for possible cases up to 1994. Patients were included if there was either surgical or radiological evidence of sclerosing encapsulating peritonitis or SBO with tanned or thickened peritoneum in the absence of other causes of SBO. Results: Fifty-four patients were analysed. The duration of continuous PD was mean 52±30 months, median 48 months and range 8-27 months. Nineteen cases were diagnosed between 1980 and 1989 and 35 between 1990 and 1994, giving mean annual incidences 1.9 and 4.2 per 1000 PD periods respectively. The overall prevalence was 0.7%, which increased progressively with the duration of PD being 1.9, 6.4, 10.8, and 19.4% for patients on dialysis for >2, 5, 6 ad 8 years respectively. Sclerosing encapsulating peritonitis was diagnosed in 87% of cases. SBO in 92%, and haemoperitoneum in 8%. Peritoneal calcification was present in seven cases, all of which had been on PD >7 years. Peritonitis was associated with 38% of cases with fungal infection in 7%. Treatment with immunosuppression in five patients appeared to result in a favourable outcome in three. The mortality rate was 56%. Conclusion: Severe sclerosing peritonitis is a serious complication of peritoneal dialysis and there is a time dependent increase on CAPD.     

Tuberculous peritonitis complicating long-term peritoneal dialysis. Report of 5 cases and review of the literature

The characteristics of 5 patients who developed tuberculous peritonitis while receiving long-term peritoneal dialysis (PD) are presented. There were 2 males and 3 females. 3 patients were on intermittent and 2 were on continuous ambulatory peritoneal dialysis when tuberculous peritonitis was first diagnosed. None of the patients had recently received immunosuppression therapy or were diabetics. The clinical presentations were similar to other forms of peritonitis complicating PD except for a more insidious onset. As extraperitoneal involvement and peritoneal lymphocytosis were rarely present, the diagnosis was mainly dependent on the direct demonstration of Mycobacterium tuberculosis with smear (1 patient) and culture (4 patients). In 1 patient with a pleuroperitoneal communication, the diagnosis was made by pleural biopsy and a positive response to antituberculous therapy. All patients responded to treatment with a combination of three antituberculous drugs which included streptomycin, isoniazid, rifampicin and pyrazinamide. Two patients were transferred to hemodialysis. In 3 patients, peritoneal dialysis was continued. Peritoneal clearance and ultrafiltration capacity were unchanged for up to 16 months after treatment in 2 patients who continued peritoneal dialysis but was reduced by 30 and 50%, respectively, in the remaining patient. Only 1 patient died, but her death was not directly related to tuberculous peritonitis. It was concluded that with a high index of suspicion and early institution of treatment, tuberculous peritonitis complicating PD can be successfully treated with low mortality and without compromising the dialysis capacity of the peritoneal membrane.     

Surgical Management of Peritoneal Dialysis Peritonitis: The Impact of Peritoneal Sclerosis

Background  Peritonitis is a life-threatening complication of peritoneal dialysis. Peritoneal sclerosis is associated with long-term peritoneal dialysis. The aim of this study was to assess the effect of peritoneal sclerosis on outcomes following laparotomy for peritoneal dialysis peritonitis. Methods  A series of 63 consecutive patients underwent laparotomy for peritoneal dialysis peritonitis. Patients were divided into two groups, those with and those without simple peritoneal sclerosis identified at laparotomy. Medical, anaesthetic, and surgical notes were used for data collection. Patients with known encapsulating peritoneal sclerosis were excluded from the study. Results  Patients with simple peritoneal sclerosis had a statistically significant longer duration of peritoneal dialysis. They also had a significantly higher risk of major complications postoperatively and a greater relative risk for mortality. Conclusions  There is an increased prevalence of simple peritoneal sclerosis with long-term peritoneal dialysis. Patients with simple peritoneal sclerosis have higher incidence of postlaparotomy complications. Patients on long-term peritoneal dialysis should be treated aggressively for peritoneal dialysis peritonitis to reduce complication/mortality rates. Evidence of simple peritoneal sclerosis at laparotomy should preclude further peritoneal dialysis.