SESSION TITLE: Atypical Presentations in the ICU
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Wednesday, October 30, 2013 at 11:30 AM - 12:30 PM
INTRODUCTION: Fibromuscular Dysplasia is a non-inflammatory, non-atherosclerotic disorder affecting the arteries and leads to arterial stenosis, occlusion, aneurysm, and dissection. It is a rare disorder, often misdiagnosed and difficult to manage.
CASE PRESENTATION: We describe the case of a 66 year old Caucasian male admitted to the hospital with acute renal failure. Histopathology from a left renal biopsy confirmed crescentic glomerulonephritis with membranous nephropathy. High dose steroid therapy, plasmapheresis and intermittent hemodialysis were initiated. On hospital day 14, while undergoing hemodialysis, he developed acute left flank pain and hypotension. Urgent, non-contrast CT scan of the abdomen was performed which did not show any acute intra-abdominal pathology. He received one unit of red cell blood transfusion and was transferred to his room. He subsequently experienced syncope secondary to hypotension and acute anemia. He was transferred to the medical intensive care unit for further evaluation. An emergent ultrasound of the abdomen did not reveal any intraabdominal or retroperitoneal fluid collection. A repeat CT scan of the abdomen with intravenous contrast revealed intraperitoneal hemorrhage along with diffuse arterial disease affecting the arteries of the abdomen and pelvis characterized by multifocal dissections, stenoses, and small aneurysms. (Figure 1). Patient became acutely hypotensive after completion of the CT scan and suffered cardio-respiratory arrest. Resuscitative attempts were unsuccessful. Autopsy revealed celiac artery dissection and rupture secondary to fibromuscular dysplasia.
DISCUSSION: Fibromuscular dysplasia (FMD) mostly commonly affects the renal and carotid arteries and predominantly affects females. Etiology is unclear and patients mostly present with hypertension, headaches and claudication pain. Angiographic imaging commonly reveals “string of beads appearance” with areas of stenosis and aneurysmal dilatation. Treatment consists of stenting stenosed arteries and management of hypertension. Rarely surgical revascularization is undertaken. Our case was atypical with extensive involvement of the mesenteric arteries but with sparing of the renal arteries.
CONCLUSIONS: This case highlights the uncommon presentation of a rare condition (Fibromuscular Dysplasia) involving the celiac artery and the catastrophic consequences of a confounding presentation.
Reference #1: Jeffery W. Olin. Recognizing and managing fibromuscular dysplasia. Cleveland Clinic Journal of Medicine. Vol 74. Number 4; April 2007.
DISCLOSURE: The following authors have nothing to disclose: Srikant Nannapaneni, Jon Steuernagle, John Park
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