A 40-year-old male Emirati patient with a known history of hypertension, psoriasis and end-stage renal disease, presented to the emergency department because of progressive, generalized, dull abdominal pain with distention, and severe constipation. However, the pain was not radiating to the back and there was no associated vomiting, fever, or diarrhea. The patient's weight and height were 66.7 kg and 169 cm, respectively. He had been on automated PD prescription (6 cycles, 2-liter exchanges of 1.36 alternating with 3.38% dextrose) for the past 6 months. Previously, he had been maintained on chronic ambulatory peritoneal dialysis 2-liter exchanges of 1.5% dextrose for a number of years. Recently, he had evidence of ultra-filtration membrane failure with declining drain volumes, hence, the change to automated PD, after which he showed good improvement. Prior to hospitalization, his spouse noticed turbidity in the PD fluid, but no other problems were reported.
Past clinical history was significant for end-stage renal disease of unknown etiology for over 20 years and hypertension. He had a living related renal transplantation from his father. Unfortunately, he suffered allograft rejection within 3 months of transplantation. Subsequently, he underwent allograft nephrectomy and was on maintenance hemodialysis for 13 years. Later, he switched to PD modality which has been ongoing for 8 years. He denied prior history of peritonitis. His current medications include: nifedipine 60 mg OD, atenolol 25 mg OD, CaCO3 1.2 g TID, and omeprazole 20 mg OD.
Physical examination revealed a mildly distressed, unwell looking gentleman who was afebrile (36.8 C), blood pressure 144/86 mm Hg, pulse 72/min, and respiratory rate 18 breaths/min. Cardiovascular examination was unremarkable with normal heart sounds, regular rhythm, and no murmur. Peripheral pulses were palpable with no edema. Lungs were clear and abdominal examination showed a distended abdomen with left lower quadrant tenderness and rebound, but normal bowel sounds. There was evidence of a previous surgical scar and the PD exit site had no redness or discharge.
Laboratory investigations revealed a white blood cell count of 11,000 x 109/l with 85% neutrophils and 8.2% lymphocytes. Serum C-reactive protein was high at 344 mg/l, serum sodium: 135 mmol/l, potassium: 4.1 mmol/l, chloride: 94 mmol/l, bicarbonate: 26 mmol/l, and glucose: 6.4 mmol/l; all levels were within normal limits. Blood urea nitrogen was 21 mmol/l and creatinine 1,540 micromol/l. Liver function tests were normal. Also, chest X-ray did not show any significant abnormalities. Abdominal ultrasound showed a mixed echogenicity fluid collection in the pelvis. The PD catheter was in place without any other intra-abdominal abnormalities. Abdominal CT scan showed evidence of peritonitis with paralytic ileus and a thickened wall of the sigmoid colon (fig. 1). No cytology of the dialysate was available.
The patient was started on cefazolin (1 g/IP daily) and gentamicin (40 mg/IP daily) as per ISPD guidelines 2000. He continued to exhibit temperature spikes during his first 2 days in hospital with continued abdominal pain and turbid PD fluid. On day 5, the surgical team, who had been monitoring the patient since admission, elected to perform an exploratory laparotomy and remove the PD catheter. The procedure revealed inflamed bowel loops with cocooning (fig. 2). The patient was then switched to hemodialysis via left AV fistula. However, despite removing the PD catheter, he remained febrile with continued abdominal pain and distention. Serum C-reactive protein level remained >200 mg/l. After consultation with infectious disease physicians, he was switched to meropenem 1 g/day following hemodialysis. Nonetheless, his fever and abdominal distention persisted unabated for over 3 months. He further underwent multiple treatment courses of antibiotics and antifungal agents including piperacillin-tazobactam, meropenem, tigecycline, voriconazole, caspofungin, metronidazole, vancomycin, and levofloxacin with no significant improvement. Throughout the course of hospitalization, he had intermittent loose stools. Moreover, he was marginally tolerating oral intake and required nutritional supplementation. Overall, his general condition deteriorated during his hospital stay, he lost approximately 10% of his body weight, and his serum albumin decreased to 32 mmol/l. Of note is that the culture of various specimens including blood, PD fluid, stool, and clostridium difficile antigen and toxin remained negative throughout his hospitalization.
Entertaining a wide spectrum of differential diagnoses to include tuberculous peritonitis, autoimmune disease, and familial Mediterranean fever, the following investigations were performed: QuantiFERON-TB and other serological investigations, namely ANA, ANCA, C3, C4, CMV, hepatitis B, C and HIV, were also negative. After removal of the PD catheter, the wound was healing well and wound cultures were negative with the exception of one which grew non-albicans Candida, hence, the antifungal treatment. Repeat abdominal ultrasound showed no major changes since admission. He then underwent a trial of discontinuing all antibiotics. Re-culture analyses were carried out, but remained negative. Subsequently, the patient was prescribed a trial anti-tuberculosis treatment regimen consisting of isoniazid (300 mg/day), rifampicin (600 mg/day), moxifloxacin (400 mg/day), ethambutol (1,200 mg post HD) and pyridoxine (50 mg/day). He was discharged home (4.5 months later) to continue on thrice weekly HD.
However, due to intolerance, he stopped anti-tuberculosis medications 2 weeks after discharge. Despite not taking his medication regularly, his condition slowly improved, and he became afebrile after 4 weeks with complete resolution of all his symptoms. It was at this juncture that the diagnosis of EPS was entertained in retrospect. Since then, the patient has had two similar presentations with abdominal pain secondary to subacute small bowel obstructions that responded well to supportive treatment with hydration, Nasogastric drainage, and pain control.