Intestinal Ischemia and Gastrointestinal Bleeding Secondary to Calciphylaxis

Abstract

Introduction: Calciphylaxis is associated with a high mortality that approaches 80%. The diagnosis is usually made when the obvious skin lesions (painful violaceous mottling of the skin resembling livedo reticularis) are present. However, visceral involvement is rare. We present a case of calciphylaxis leading to lower gastrointestinal (GI) bleeding and rectal ulceration of the GI mucosa. Case-Report: A 66-year-old woman with past medical history of diabetes mellitus, hypertension, endstage renal disease (ESRD) on hemodialysis (HD), recently diagnosed ovarian cancer presented with painful black necrotic eschar on both the legs (Figure- 1A, 1B). The radiograph of legs demonstrated extensive calcification of the lower extremity arteries (Figure-1C, 1D). Hospital course was complicated with lower GI bleeding. CT scan of the abdomen revealed severe circumferential calcification of the abdominal aorta, celiac artery, superior and inferior mesenteric arteries and their branches (Figure-2A, 2B). Colonoscopy revealed severe rectal necrosis (Figure- 3). She was also found to have pneumoperitoneum although no extravasation of oral contrast was seen within the peritoneal cavity. She was deemed to be a poor surgical candidate due to comorbidities and presence of extensive vascular calcifications. She had recurrent episodes of profuse GI bleeding. Hence the decision was made to manage her conservatively with as needed blood transfusions.Figure 1Figure 2Figure 3Following her diagnosis of calciphylaxis; supplementation with vitamin D and calcium containing phosphate binders was stopped. She was started on daily hemodialysis with low calcium dialysate bath as well as intra-venous sodium thiosulphate. The calcium and phosphate levels significantly decreased following the therapy but the intra-peritoneal free air persisted. The clinical condition of the patient deteriorated and the patient died secondary to multiorgan failure. Discussion: The most common GI manifestation of calciphylaxis is lower GI bleed. Other manifestations include abdominal pain, diarrhea, malabsorption, mucosal edema, diffuse ulcer formation and bowel infarction leading to bowel perforation. Rarely, there can be an elevation of the liver function tests from involvement of the portal vein and hepatic arteries leading to hepatic ischemia. Conclusion: Calciphylaxis should be kept as one of differential in patients with ESRD on HD presenting with abdominal pain or GI bleeding.