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Fibromuscular Dysplasia, Kidney
T. Gregory Walker, MD, FSIR; Andrew J. Gunn, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Fibromuscular dysplasia (FMD): Noninflammatory, nonatherosclerotic arterial disorder
                • Characterized by segmental areas of collagen deposition and smooth muscle overgrowth
                  • Affects multiple arterial territories
                    • Renal artery: Most commonly involved
                    • Carotid artery: 2nd most commonly involved
                  • May cause stenosis, dissection, aneurysms
                    • Known cause of renovascular hypertension
                • Classified based on involved layer of arterial wall
                  • Intimal fibroplasia (< 10%)
                    • Focal concentric or long-segment tubular stenosis
                    • Internal elastic lamina may be disrupted
                  • Medial fibroplasia (3 subtypes)
                    • Medial dysplasia (80%): Multiple stenoses and aneurysms involving mid to distal renal artery; causes classic "string of beads" appearance
                    • Perimedial fibroplasia (10-15%): May also cause "string of beads" appearance; beads have smaller diameter than parent vessel
                    • Medial hyperplasia (1-2%): Smooth stenosis
                  • Adventitial fibroplasia (< 1%)
                    • Similar appearance to intimal fibroplasia

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Baumgartner I et al: Renovascular hypertension: screening and modern management. Eur Heart J. 32(13):1590-8, 2011
                                              2. Olin JW et al: Diagnosis, management, and future developments of fibromuscular dysplasia. J Vasc Surg. 53(3):826-36, 2011
                                              3. Blondin D et al: Fibromuscular dysplasia in living renal donors: still a challenge to computed tomographic angiography. Eur J Radiol. 75(1):67-71, 2010
                                              4. Liu PS et al: CT angiography of the renal circulation. Radiol Clin North Am. 48(2):347-65, viii-ix, 2010
                                              5. Slovut DP et al: Fibromuscular dysplasia. N Engl J Med. 350(18):1862-71, 2004
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Definitions

                                                          • Fibromuscular dysplasia (FMD): Noninflammatory, nonatherosclerotic arterial disorder
                                                            • Characterized by segmental areas of collagen deposition and smooth muscle overgrowth
                                                              • Affects multiple arterial territories
                                                                • Renal artery: Most commonly involved
                                                                • Carotid artery: 2nd most commonly involved
                                                              • May cause stenosis, dissection, aneurysms
                                                                • Known cause of renovascular hypertension
                                                            • Classified based on involved layer of arterial wall
                                                              • Intimal fibroplasia (< 10%)
                                                                • Focal concentric or long-segment tubular stenosis
                                                                • Internal elastic lamina may be disrupted
                                                              • Medial fibroplasia (3 subtypes)
                                                                • Medial dysplasia (80%): Multiple stenoses and aneurysms involving mid to distal renal artery; causes classic "string of beads" appearance
                                                                • Perimedial fibroplasia (10-15%): May also cause "string of beads" appearance; beads have smaller diameter than parent vessel
                                                                • Medial hyperplasia (1-2%): Smooth stenosis
                                                              • Adventitial fibroplasia (< 1%)
                                                                • Similar appearance to intimal fibroplasia

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Angiographic Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Baumgartner I et al: Renovascular hypertension: screening and modern management. Eur Heart J. 32(13):1590-8, 2011
                                                                                          2. Olin JW et al: Diagnosis, management, and future developments of fibromuscular dysplasia. J Vasc Surg. 53(3):826-36, 2011
                                                                                          3. Blondin D et al: Fibromuscular dysplasia in living renal donors: still a challenge to computed tomographic angiography. Eur J Radiol. 75(1):67-71, 2010
                                                                                          4. Liu PS et al: CT angiography of the renal circulation. Radiol Clin North Am. 48(2):347-65, viii-ix, 2010
                                                                                          5. Slovut DP et al: Fibromuscular dysplasia. N Engl J Med. 350(18):1862-71, 2004