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Mounier-Kuhn Syndrome
Carlos S. Restrepo, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Mounier-Kuhn syndrome (MKS)
            • Synonyms

              • Tracheobronchomegaly
              • Congenital tracheobronchomegaly
            • Definitions

              • Rare disorder likely secondary to congenital defect or atrophy of elastic and smooth muscle components of trachea and mainstem bronchi with resultant significant central airway dilatation

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Krustins E: Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years. Clin Respir J. 10(1):3-10, 2016
                                      2. Payandeh J et al: A clinical classification scheme for tracheobronchomegaly (Mounier-Kuhn syndrome). Lung. 193(5):815-22, 2015
                                      3. Krustins E et al: Mounier-Kuhn syndrome or congenital tracheobronchomegaly: a literature review. Respir Med. 107(12):1822-8, 2013
                                      4. Celik B et al: Mounier-Kuhn syndrome: a rare cause of bronchial dilation. Tex Heart Inst J. 38(2):194-6, 2011
                                      5. Marom EM, Goodman PC, McAdams HP. Diffuse abnormalities of the trachea and main bronchi. AJR 2001;176;(3):713-7.
                                      6. Woodring et al: Acquired tracheomegaly in adults as a complication of diffuse pulmonary fibrosis. AJR 1989;152: 743-7.
                                      7. Shin MS et al: Tracheobronchomegaly (Mounier-Kuhn syndrome): CT diagnosis. AJR Am J Roentgenol. 150(4):777-9, 1988
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Mounier-Kuhn syndrome (MKS)
                                                • Synonyms

                                                  • Tracheobronchomegaly
                                                  • Congenital tracheobronchomegaly
                                                • Definitions

                                                  • Rare disorder likely secondary to congenital defect or atrophy of elastic and smooth muscle components of trachea and mainstem bronchi with resultant significant central airway dilatation

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Staging, Grading, & Classification

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Krustins E: Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years. Clin Respir J. 10(1):3-10, 2016
                                                                          2. Payandeh J et al: A clinical classification scheme for tracheobronchomegaly (Mounier-Kuhn syndrome). Lung. 193(5):815-22, 2015
                                                                          3. Krustins E et al: Mounier-Kuhn syndrome or congenital tracheobronchomegaly: a literature review. Respir Med. 107(12):1822-8, 2013
                                                                          4. Celik B et al: Mounier-Kuhn syndrome: a rare cause of bronchial dilation. Tex Heart Inst J. 38(2):194-6, 2011
                                                                          5. Marom EM, Goodman PC, McAdams HP. Diffuse abnormalities of the trachea and main bronchi. AJR 2001;176;(3):713-7.
                                                                          6. Woodring et al: Acquired tracheomegaly in adults as a complication of diffuse pulmonary fibrosis. AJR 1989;152: 743-7.
                                                                          7. Shin MS et al: Tracheobronchomegaly (Mounier-Kuhn syndrome): CT diagnosis. AJR Am J Roentgenol. 150(4):777-9, 1988