A 45-Year-Old Man With Recurrent Nausea, Vomiting, and Abdominal Distension

Abstract

Question: A 45-year-old African American man with end-stage renal disease secondary to polycystic kidney disease, on peritoneal dialysis for the last 15 years, presented with worsening of nausea, vomiting, and abdominal distension, associated with a 20-kg (44-lbs) weight loss over the past year. He had a total of 4 prior episodes of peritonitis since initiation of his dialysis, where the known causative agent was Pseudomonas aeruginosa for 1 episode, and Staphylococcus epidermidis for another. He was treated with antibiotics during these incidents. During one episode, he underwent catheter replacement. Soon after, he developed an incarcerated incisional hernia and bowel obstruction requiring exploratory laparotomy for hernia repair. On examination, the patient was tachycardic to low 100s. He was thin and seemed to be mildly uncomfortable from nausea, with dry mucous membranes, hypoactive bowel sounds, and mild abdominal distension, but no tenderness to deep palpation or guarding. Laboratory tests were pertinent for bicarbonate 33 mEq/L, erythrocyte sedimentation rate 127 mm/h, and albumin 2.7 g/dL; white blood cell count, lipase, amylase, and liver panel were all within normal limits, and blood cultures were negative. Quantiferon Gold and PPD were negative. A key computed tomography (CT) image from this admission is shown (Figure A), where imaging 3 years prior was unremarkable. What is the diagnosis? What are the management options? Look on page 1196 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Diffuse peritoneal and serosal calcifications on the CT imaging confirmed a diagnosis of encapsulating peritoneal sclerosis (EPS), also known as “abdominal cocoon” (Figure A, arrows). It is known mostly as a rare complication of peritoneal dialysis (PD), but may also be idiopathic.1Kawaguchi Y. Kawanishi H. Mujais S. et al.Encapsulating peritoneal sclerosis: definition, etiology, diagnosis, and treatment.Perit Dial Int. 2000; 20: 1-6Crossref Google Scholar Because the long-term deterioration of the peritoneum expected from PD does not lead to more frequent presentation of EPS, the “2-hit theory” suggests that EPS occurs in the combination of long-term PD and some other causative agents that lead to intraperitoneal inflammation.1Kawaguchi Y. Kawanishi H. Mujais S. et al.Encapsulating peritoneal sclerosis: definition, etiology, diagnosis, and treatment.Perit Dial Int. 2000; 20: 1-6Crossref Google Scholar Common risk factors for development of EPS include duration of PD and episodes of peritonitis, especially when due to infections with S aureus, Pseudomonas spp., or fungi.1Kawaguchi Y. Kawanishi H. Mujais S. et al.Encapsulating peritoneal sclerosis: definition, etiology, diagnosis, and treatment.Perit Dial Int. 2000; 20: 1-6Crossref Google Scholar The presence of peritoneal calcifications on imaging confirms late stage of the disease. The diagnosis can only be made earlier by histopathologic confirmation with biopsy from laparoscopy (in early stages) or laparotomy. The 1-year mortality is as high as 57% after a diagnosis of EPS, because it is often diagnosed with late-stage findings of CT calcification or on laparotomy after obstruction.2Petrie M.C. Traynor J.P. Mactier R.A. Incidence and outcome of encapsulating peritoneal sclerosis.Clin Kidney J. 2016; 9: 624-629Crossref PubMed Scopus (14) Google Scholar The most common symptoms before diagnosis are nonspecific: abdominal pain, weight loss, vomiting, and bowel obstruction.2Petrie M.C. Traynor J.P. Mactier R.A. Incidence and outcome of encapsulating peritoneal sclerosis.Clin Kidney J. 2016; 9: 624-629Crossref PubMed Scopus (14) Google Scholar If diagnosed before the development of irreversible fibrosis and encapsulation from calcification, data from case series have suggested improved 1-year survival after treating the underlying cause (in this case the removal of peritoneal dialysis catheter), medical treatment with corticosteroids with possible adjuvant therapy with immunosuppressive agents.3Habib S.M. Betjes M.G.H. Fieren M.W.J.A. et al.Management of encapsulating peritoneal sclerosis: A guideline on optimal and uniform treatment.Neth J Med. 2011; 69: 500-507PubMed Google Scholar Therefore, early suspicion and a low threshold for diagnostic laparoscopy/laparotomy may be an effective strategy for early diagnosis and treatment, with possible improvement in mortality. Our patient was treated with tamoxifen, prednisone, total parenteral nutrition for bowel rest, and dialysis regimen change to hemodialysis. One year later, when he presented with abdominal pain, he was found to have a small bowel obstruction and perforation. He was switched to comfort care and discharged to hospice, where he passed away 5 days later.