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Diffuse Pulmonary Lymphangiomatosis
Melissa L. Rosado-de-Christenson, MD, FACR; Tyler H. Ternes, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Diffuse pulmonary lymphangiomatosis (DPL)
            • Synonyms

              • Diffuse pulmonary angiomatosis; uncertain nature of vascular abnormalities
            • Definitions

              • Congenital disorder: Increased number & complexity of anastomosing lymphatic channels
              • Involves lymphatics in interlobular septa, pleura, & peribronchovascular regions

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR 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

                                        Selected References

                                        1. Ozeki M et al: Propranolol for intractable diffuse lymphangiomatosis. N Engl J Med. 364(14):1380-2, 2011
                                        2. Shah V et al: Mediastinal involvement in lymphangiomatosis: a previously unreported MRI sign. Pediatr Radiol. 41(8):985-92, 2011
                                        3. Raman SP et al: Imaging of thoracic lymphatic diseases. AJR Am J Roentgenol. 193(6):1504-13, 2009
                                        4. El Hajj L et al: Diagnostic value of bronchoscopy, CT and transbronchial biopsies in diffuse pulmonary lymphangiomatosis: case report and review of the literature. Clin Radiol. 60(8):921-5, 2005
                                        5. Yekeler E et al: Diffuse pulmonary lymphangiomatosis: imaging findings. Diagn Interv Radiol. 11(1):31-4, 2005
                                        6. Swensen SJ et al: Diffuse pulmonary lymphangiomatosis: CT findings. J Comput Assist Tomogr. 19(3):348-52, 1995
                                        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

                                                  • Abbreviations

                                                    • Diffuse pulmonary lymphangiomatosis (DPL)
                                                  • Synonyms

                                                    • Diffuse pulmonary angiomatosis; uncertain nature of vascular abnormalities
                                                  • Definitions

                                                    • Congenital disorder: Increased number & complexity of anastomosing lymphatic channels
                                                    • Involves lymphatics in interlobular septa, pleura, & peribronchovascular regions

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR 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

                                                                              Selected References

                                                                              1. Ozeki M et al: Propranolol for intractable diffuse lymphangiomatosis. N Engl J Med. 364(14):1380-2, 2011
                                                                              2. Shah V et al: Mediastinal involvement in lymphangiomatosis: a previously unreported MRI sign. Pediatr Radiol. 41(8):985-92, 2011
                                                                              3. Raman SP et al: Imaging of thoracic lymphatic diseases. AJR Am J Roentgenol. 193(6):1504-13, 2009
                                                                              4. El Hajj L et al: Diagnostic value of bronchoscopy, CT and transbronchial biopsies in diffuse pulmonary lymphangiomatosis: case report and review of the literature. Clin Radiol. 60(8):921-5, 2005
                                                                              5. Yekeler E et al: Diffuse pulmonary lymphangiomatosis: imaging findings. Diagn Interv Radiol. 11(1):31-4, 2005
                                                                              6. Swensen SJ et al: Diffuse pulmonary lymphangiomatosis: CT findings. J Comput Assist Tomogr. 19(3):348-52, 1995