Sclerosing Peritonitis With Gross Peritoneal Calcification: A Case Report Stephen V. Cox, MB, BS, Jerome Lai, MB, BS, Michael Suranyi, MB, BS, FRACP, PhD, and Neal Walker, MD, BS, FRCPA • We report the case of a patient on dialysis for 13 years, including continuous ambulatory peritoneal dialysis (CAPO) for 11 years, who developed sclerosing peritonitis with gross peritoneal calcification. The patient first presented with abdominal pain in January 1990, when peritoneal calcification was detected for the first time. Her symptoms settled spontaneously and 1 year later she presented with acute peritonitis and adynamic ileus. The peritonitis settled with antibiotics and Tenchkoff catheter removal, but the ileus persisted. She was commenced on long-term parenteral nutrition, but never recovered useful bowel function. After 8 weeks of hemodialysis and total parenteral nutrition, a further laparotomy for an acute abdomen showed what appeared to be extensive bowel infarction and peritoneal calcification. She died several days later. Of significance, peritoneal calcification was first noted on x-ray and computed tomography (CT) scan while the patient was still largely asymptomatic and before peritoneal ultrafiltration capacity was significantly impaired. Unlike other reported cases of calcifying peritonitis, 1 sclerosing peritonitis was present and calcification was far more extensive. It was not associated with factors such as frequent infective peritonitis or acetate dialysate. Calciphylaxis was not present nor was there any abnormality of calcium-phosphate metabolism. The outcome of this case suggests that patients with recurrent or persistent bowel symptoms on long-term CAPO should have early abdominal x-ray or CT scanning to exclude sclerosing peritonitis or bowel calcification. If present, consideration should be given to transferring the patient to another therapeutic dialysis modality if possible. © 1992 by the National Kidney Foundation, Inc. INDEX WORDS: Continuous ambulatory peritoneal dialysis; complications; calcifying peritonitis; sclerosing peritonitis.

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ESPITE its important role in the therapy of end-stage renal failure, continuous ambulatory peritoneal dialysis (CAPD) may be complicated by bacterial peritonitis, aseptic or allergic peritonitis, and sclerosing peritonitis. In recent years, a new complication, calcifying peritonitis, has been reported.· We present a case of sclerosing peritonitis accompanied by gross calcification of the peritoneum and intestinal wall. Patients with calcifying peritonitis reported previously· have been transferred to hemodialysis with resultant recovery of bowel function . This was not the case in our patient, in whom the calcium deposition was far more extensive. Following a terminal illness characterized by prolonged peritonitis and adynamic ileus, autopsy showed extensive calcification of the intestinal muscularis propria, a change far more extensive than that shown previously.· CASE REPORT

A 27-year-old white woman was first seen at our hospital in 1973 with a diagnosis of end-stage renal failure secondary to hypoplastic kidneys. She had been treated for renal impairment since the age of 18 months and commenced hemodialysis in 1977 at the age of 14 years. During that time she had suffered from renal osteodystrophy and had subsequently undergone subtotal parathyroidectomy. She also had bilateral slipped femoral epiphyses corrected by surgical fixation.

For 2 years after commencing hemodialysis, she had numerous admissions to hospital for hypertension and fluid overload, including hypertensive encephalopathy. Her blood pressure was treated with hydralazine, prazosin, and propranolol with good effect. Due to multiple vascular access problems during the next year she was transferred to peritoneal dialysis in May 1980. She received two Tenchkoff catheters (Pharma Plast, Australia, discontinued) in quick succession due to technical problems with the initial catheter. CAPO was commenced in August 1980 after a short initial period on intermittent peritoneal dialysis (lPD). Her first episode of peritonitis occurred shortly after this on August 12. On CAPO, her blood pressure stabilized, which allowed cessation of most antihypertensives, including propranolol. Over the next 10 years she experienced five separate episodes of peritonitis while on CAPO. The organisms involved included Streptococcus viridans (once), Staphylococcus albus (once with two relapes), Pseudomonas spp (unidentified species, once), Staphylococcus epidermidis (once), and an episode of culture-negative peritonitis. The antibiotics used included intraperitoneal (IP) and/or intravenous (IV) ampicillin (once), cephalothin (five times), flucloxacillin (twice), gentamicin

From the Departments ofNephrology and Pathology, Princess Alexandra Hospital, Brisbane, Queensland, Australia. Received May 1, 1992; accepted in revised fo rm July 7, 1992. Address correspondence to Stephen V Cox, MB, BS, Princess Alexandra Hospital, Ipswich Rd, Woolloongabba, Queensland, 4102, Australia. No reprints available. © 1992 by the National Kidney Foundation, Inc. 0272-6386/92/2006-0018$3.00;0

American Journal of Kidney Diseases. Vol XX. No 6 (December). 1992: pp 637-642

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638 (once), tobramycin (three times), and vancomycin (twice). Her Tenchkoff catheter was replaced twice (Pharma Plast; for infection and poor flow) between August 1980 and December 1981. The final Tenchkoff, a Quinton (Quinton Instrument Co, Seattle, W A), remained in situ from December 1981 till December 1990. She lost two renal transplants (1979 preCAPD and in 1986) due to rejection, and only spent 2 months offCAPD. She was investigated for vague abdominal pain in January 1990. Clinical and laboratory investigations excluded infective peritonitis. A computed tomography (CT) scan of the abdomen and plain abdominal x-ray showed areas of bowel wall calcification (Fig 1). This was not evident on her previous abdominal film dated July 1988. The etiology of this calcification and its significance were considered uncertain, particularly as there was no evidence of failure of peritoneal ultrafiltration capacity and her symptoms had settled spontaneously. She had remained peritonitis-free for 3 years until October 1990 when a Staph epidermidis. infection was treated with IP vancomycin and IP tobramycin. In December 1990, the patient was admitted with abdominal pain and cloudy peritoneal fluid without blood-stained effluent. Dialysate fluid culture yielded an unidentified Pseudomonas spp. This was initially treated with IP vancomycin (1,400 mg stat) and IP tobramycin (16 mg stat, then 10 mg per bag every 4 hours), then changed to tobramycin and ceftazidime (500 mg stat, then 250 mg per bag every 4 hours) when the cultures returned. She failed to settle and went to laparotomy for removal of the Tenchkoff catheter; the same catheter had remained in situ for 10 years. Laparotomy showed thickened, calcified, granular, brown discoloration of the bowel wall (Fig 2) and entire peritoneal cavity lining, with complete loss of bowel pliability and serosal fissuring. There was no evidence of mechanical bowel obstruction, perforation, or collections of pus or free peritoneal blood. She returned to hemodialysis via subclavian venous access, as no other fistula or shunt sites were available. Prolonged and persistent ileus with abdominal pain and vomiting after the ingestion of any solids required the introduction of total parenteral nutrition via this central venous access. Antibiotics (including IV ceftazidime, tobramycin, and aztreonam) were continued for persisting fevers and the ileus; however, repeated blood cultures and ultrasound-guided peritoneal aspirations, both on and off antibiotics, failed to grow any organisms, including mycobacterium or fungi. The fevers settled with cessation of all antibiotics; however, the ileus persisted. Abdominal x-rays showed a marked increase in intraabdominal calcification and a repeat CT scan of the abdomen confirmed marked bowel wall calcification (Fig 3). No cause was found for the refractory ileus apart from the extensive bowel calcification. She remained on total parenteral nutrition and hemodialysis. Ten weeks later, she developed severe abdominal pain and fever to 40°C. A further laparotomy was performed, which showed blood-stained ascites and an appearance suggestive of extensive gangrene and calcification of the small bowel and colon, extending to the sigmoid colon. It was decided that resection could not offer the patient useful extension of life

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Fig 1. Plain supine abdominal film demonstrating extensive fine calcification of the bowel wall, January

1990.

and the abdomen was closed and conservative management adopted. The patient died 5 days later. At autopsy the parietal and visceral peritoneum, including the free surfaces of the liver, spleen, stomach, small and large intestines, mesenteries, omentum, and uterus, appeared thickened by fibrous tissue. Loops of small bowel were bound together by fibrous adhesions and the serosa of the intestinal tract was brown and granular with fissuring of the surface evident (Fig 4). The intestinal wall was gritty when cut, but the mucosa of the stomach and intestines was normal in appearance. Histologically, the serosal surface of the bowel was covered by a thick layer of fibrocellular connective tissue (Fig 5), focally showing organizing fibrinous exudate and containing aggregates ofiymphocytes, plasma cells, and macrophages. The mesothelium was absent and the serosa thickened by acellular fibrous tissue, which often extended to involve the longitudinal layer of the muscularis propria, which showed loss of muscle fibers and partial replacement by large plaques of calcium (Fig 5). Serosal vessels showed obliterative changes. The inner layers of the bowel were unaffected. The bowel mesentery, omentum, liver, and spleen also had a thick overlying layer of fibrocellular connective tissue. The mesothelium

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Fig 2. Laparotomy (December 1990), shows discolored, thickened, and fissured serosal surface of the bowel.

was again absent and the serosa of these structures thickened by acellular connective tissue containing obliterated blood vessels. A sparse inflammatory infiltrate was present at its inner extent. The mesentery and omentum showed lobules of anucleated adipocyte ghosts subserosally, large calcified plaques in the altered fat, and fibrosis around viable adipose cells at the inner extent of these changes. Tissue toward the center of the mesentery and omentum was unaffected. There was no evidence of vasculitis or of vascular calcification. The liver,

spleen, adrenals, and genital system showed no intraorgan calcification.

DISCUSSION

As the use of peritoneal dialysis gains increasing favor as a form of maintenance dialysis therapy, this case acts as a timely reminder that CAPD may not be a completely benign form of

Fig 3. The extensive bowel wall calcification seen on plain film is confirmed on noncontrast CT scan. Barium is seen in the bowel lumen.

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Fig 4. Dark loops of small bowel bound by fibrous adhesions (arrowed).

long-term treatment. We have reported a case complicated by sclerosing peritonitis with progressive calcifying peritonitis that proved to be ultimately fatal. Although other cases of calcifying peritonitis have been reported,1.2 they do not appear to have been as extensive or to have resulted in long-term disability, possibly because CAPD was ceased at an earlier stage. Unfortunately, no other per-

Fig 5. Wall of small bowel covered by a fibrocellular membrane (small arrow) and showing plaque-like calcification of the longitudinal layer of the muscularis externa (large arrows). The inner circular layer of the muscularis extema (bottom of picture) is unaffected. Note the absence of mesothelial cells between the fibrocellular membrane and the bowel wall.

manent dialysis modality was available in this patient given the difficulty with hemodialysis access. Previous cases also differ in that uncontrolled hyperparathyroidism was a feature in two cases, as was blood-stained effluent during episodes of bowel symptoms. I ,2 This case also appears to differ from the majority of cases of sclerosing peritonitis. Sclerosing peritonitis is a recognized cause of ultrafiltration failure in

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CAPD3 and often appears approximately 5 years after initiation ofCAPD. In sclerosing peritonitis, peritoneal thickening, laminar intestinal concrescence, and diffuse hemorrhage occurs, resulting in patient death from ileus. 4 In some reports, exudative bloody ascites, intestinal obstruction, and ultrafiltration failure are features and there is a high mortality rate. 5 Our patient did not have ultrafiltration failure, as her fluid status and biochemistry were well controlled up to her final illness, although formal testing was not undertaken. The ultrasound findings in sclerosing peritonitis include tethering of the bowel and membrane formation, but significant calcification has not been described6 and a CT scan study has also failed to show bowel calcification. 7 Bowel wall calcification was a prominent feature of this case both radiologically and pathologically. The etiology of sclerosing and calcifying peritonitis remains unclear. 8 Marichal et al 1,9 suggested that acetate buffer in the dialysate may play some part. Our patient was never exposed to acetate buffer, lactate being used as the sole buffering agent in all of our patients. In some patients, chlorhexidine antiseptic has been implicated as a causative agent. lO ,11 However, this woman was never exposed to any antiseptic solutions. She used closed systems, which did not require line flushing, minimizing any possible exposure to antiseptics. Beta-blocking drugs were not used to any extent in this patient, but she was repeatedly exposed to IP cephalothin; vancomycin therapy and its contaminants have been implicated by othersY Recent evidence 13 has shown that peritoneal dialysis fluid itself may be cytotoxic and, in particular, the heat sterilization process used in its manufacture. Recurrent episodes of infective peritonitis have also been implicated. 1,14 This patient experienced seven episodes of peritonitis in her 11 years on CAPO and represents one of only four patients with a cumulative time on CAPD greater than 10 years on the Australia and New Zealand Dialysis and Transplant Registry.15 It may, of course, not be the frequency of peritonitis that is important, but the absolute number of episodes. Disordered calcium balance could also be a factor, but this patient's calcium levels were never elevated following her subtotal parathyroidectomy several years before CAPD. Regular parathyroid hormone levels from 1986 onward were

within the normal range. Follow-up x-rays of her hands did not show any changes of hyperparathyroidism or of vascular calcification. The clinical picture and autopsy findings are not consistent with calciphylaxis. Calciphylaxis is a rare severe complication of secondary hyperparathyroidism consisting of skin lesions, vascular calcification, and distal extremity gangrene. 16 ,17 Klemm 17 discusses the role of peritoneal blood as a sensitizing agent in those patients who already have abnormalities of calcium, phosphate, or vitamin D metabolism. The iron apparently acts as a precipitant to calcium deposition within the peritoneum. We are unable to explain our patients pathology on this basis, as none of the known precipitants of calciphylaxis or a calciphylaxis-like syndrome were present. We suggest that calcifying peritonitis may be the end result of many peritoneal insults. The factors that others have found to be associated with sclerosing and calcifying peritonitis do not seem to be strongly implicated in this patient. Perhaps the use of any hypertonic dialysate may predispose to this entity8,13 and calcifying peritonitis may be a risk in any patient who has had CAPO for a long enough period. In CAPO patients who suffer from recurrent peritonitis, prolonged or unexplained bowel symptoms or bowel obstruction, or blood-stained effluent, early abdominal x-ray and or CT scan are warranted. If bowel calcification is detected, then early consideration should be given to transferring the patient to hemodialysis, or to offering a renal transplant if the patient is suitable, even before the loss of ultrafiltration capacity. ACKNOWLEDGMENT We wish to thank the CAPO staff, especially Sister Jill Butler, for advice and the use of their comprehensive notes. We thank the following for their input into this case report: Dr J. Bourke, Professor I. Hardie, Dr C. Hawley, Dr J. Petrie, Dr R. Price, Dr R. Rigby, and Dr D. Wall.

REFERENCES I. Marichal I, Faller B, Brignon P, et al: Progressive calcifying peritonitis: A new complication of CAPO? Report of two cases. Nephron 45:229-232, 1987 2. Francis 0, Busmanis L, Becker G: Peritoneal calcification in a peritoneal dialysis patient; a case report. Perit Dial Int 10:237-240, 1990 3. Mactier R: Investigation and management of ultrafiltration failure in CAPO. Adv Perit Dial 7:57-62, 1991

642 4. Karatson A, Buzogany I, Wagner G, et al: Sclerosing peritonitis caused by disinfectant in a patient under peritoneal dialysis. Int Urol Nephrol 13:185-190, 1991 5. Pusateri R, Ross R, Marshall R, et al: Sclerosing encapsulating peritonitis: Report of a case with small bowel obstruction managed by long-term horne parenteral hyperalimentation and a review of the literature. Am J Kidney Dis 8:56-60, 1986 6. Hollman A, McMillan M, Briggs J, et al: Ultrasound changes in sclerosing peritonitis following continuous ambulatory peritoneal dialysis. Clin RadioI43:176-179, 1991 7. Korzets A, Korzets Z, Peer G, et al: Sclerosing peritonitis. Am J Nephrol 8:143-146, 1988 8. Dobbie J: Pathogenesis of peritoneal fibrosing syndromes (sclerosing peritonitis) in peritoneal dialysis. Perit Dial Int 12: 14-27, 1992 9. Marichal I, Faller B, Brignon P, et al: Peritonitis in continuous ambulatory peritoneal dialysis: A role for the dialysate. Nephron 42:167-170,1986 10. Oules R, Challah S, Brunner F: Case-control study to determine the cause of sclerosing peritonitis. Nephrol Dial Transplant 3:66-69, 1988

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II. Junor B, Briggs J, Forwell M, et al: Sclerosing peritonitis-The contribution of chlorhexidine in alcohol. Perit Dial Bull 5:101-104, 1985 12. Charney D, Gouge S: Chemical peritonitis secondary to intraperitoneal vancomycin. Am J Kidney Dis 17:76-79, 1991 13. Wieslander A, Nordin M, Kjellstrand P, et al: Toxicity of peritoneal dialysis fluids on cultured fibroblasts, L-929. Kidney Int 40:77-79, 1991 14. Slingeneyer A, Elie M: Cooperative international study on sclerosing encapsulating peritonitis: Preliminary report, in Khanna R, Nolph K, Prowant B, et al (eds): Advances in Continuous Ambulatory Peritonitis, 1985. Proceedings of the Fifth Annual CAPD Conference, Missouri. Toronto, Canada University of Toronto Press, 1985, p 118 15. Australia and New Zealand Dialysis and Transplant Registry, held at The Queen Elizabeth Hospital, Woodville Rd, Woodville South, South Australia, 5011. 16. Duh Q, Lim R, Clark 0: Calciphylaxis in secondary hyperparathyroidism. Diagnosis and parathyroidectomy. Arch Surg 126:1213-1218, 1991 17. Klemm G: Peritoneal calcification and calciphylaxis. Nephron 51:124,1989

Sclerosing peritonitis with gross peritoneal calcification: a case report.

We report the case of a patient on dialysis for 13 years, including continuous ambulatory peritoneal dialysis (CAPD) for 11 years, who developed scler...
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