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Chylothorax
Randy R. Richardson, MD; A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Synonyms

          • Chylous fluid in pleural space
        • Definitions

          • Chylothorax: Lymphatic fluid in pleural space secondary to congenital or acquired conditions, including congenital anomaly, ↑ pressure, impaired drainage/obstruction, or trauma to thoracic duct
            • Thoracic duct is major central lymphatic vessel
            • Extends in paraspinal tissues from cisterna chyli to point of drainage into left subclavian/brachiocephalic vein
            • Drains lymph from lower limbs, pelvis, abdomen
          • Lymphangiectasia: Abnormal dilation of lymphatics
            • 1°: Abnormal development of lymphatics in isolation or with genetic anomaly
            • 2°: Related to congenital heart disease (CHD) or repair
          • Neonatal/congenital lymphatic flow disorders (central lymphatic conducting channel anomaly)
            • Pulmonary lymphatic perfusion syndrome: Abnormal lymphatic flow in lungs & pleura → chylothorax
            • Central lymphatic flow disorder: Abnormal flow into additional tissues/cavities → body wall, peritoneum, etc.
            • Congenital chylous ascites

        IMAGING

        • General Features

          • Radiographic Findings

            • Ultrasonographic Findings

              • CT Findings

                • MR Findings

                  • Lymphangiogram

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Barrera CA et al: Imaging of fetal lymphangiectasias: prenatal and postnatal imaging findings. Pediatr Radiol. 50(13):1872-80, 2020
                                      2. Biko DM et al: Pediatric pulmonary lymphatic flow disorders: diagnosis and management. Paediatr Respir Rev. 36:2-7, 2020
                                      3. Chavhan GB et al: Magnetic resonance lymphangiography. Radiol Clin North Am. 58(4):693-706, 2020
                                      4. Hannah DM et al: In utero congenital chylothorax treatment with fetal thoracoamniotic shunt: case report. J Neonatal Perinatal Med. 13(3):427-30, 2020
                                      5. Pinto E et al: Neonatal lymphatic flow disorders: impact of lymphatic imaging and interventions on outcomes. J Perinatol. 41(3):494-501, 2020
                                      6. Rubalcava NS et al: Efficacy of early pleurectomy for severe congenital chylothorax. J Surg Res. 256:433-8, 2020
                                      7. Zheng Q et al: Quantification of thoracic lymphatic flow patterns using dynamic contrast-enhanced MR lymphangiography. Radiology. 296(1):202-7, 2020
                                      8. Biko DM et al: Imaging of central lymphatic abnormalities in Noonan syndrome. Pediatr Radiol. 49(5):586-92, 2019
                                      9. De Angelis LC et al: Congenital chylothorax: current evidence-based prenatal and post-natal diagnosis and management. Lymphology. 52(3):108-25, 2019
                                      10. Shaikh R et al: MR imaging evaluation of pediatric lymphatics:: overview of techniques and imaging findings. Magn Reson Imaging Clin N Am. 27(2):373-85, 2019
                                      11. Biko DM et al: Recognition of neonatal lymphatic flow disorder: fetal MR findings and postnatal mr lymphangiogram correlation. Acad Radiol. 25(11):1446-50, 2018
                                      12. Pimpalwar S et al: Dynamic contrast enhanced magnetic resonance lymphangiography: categorization of imaging findings and correlation with patient management. Eur J Radiol. 101:129-35, 2018
                                      13. Brock WW et al: Congenital chylothorax: a unique presentation of nonimmune hydrops fetalis in a preterm infant. Adv Neonatal Care. 16(2):114-23, 2016
                                      14. Bang JH et al: Anatomic variability of the thoracic duct in pediatric patients with complex congenital heart disease. J Thorac Cardiovasc Surg. 150(3):490-5, 2015
                                      15. Bialkowski A et al: Congenital chylothorax: a prospective nationwide epidemiological study in Germany. Arch Dis Child Fetal Neonatal Ed. 100(2):F169-72, 2015
                                      16. Krishnamurthy R et al: Imaging the central conducting lymphatics: initial experience with dynamic MR lymphangiography. Radiology. 274(3):871-8, 2015
                                      17. Bengtsson BO: Outcome of neonatal chylous effusions: what do you tell the parents? A 10-year experience in a community hospital. Am J Perinatol. 31(12):1037-42, 2014
                                      18. Downie L et al: Congenital chylothorax: associations and neonatal outcomes. J Paediatr Child Health. 50(3):234-8, 2014
                                      19. Gray M et al: Treatment of congenital pulmonary lymphangiectasia using ethiodized oil lymphangiography. J Perinatol. 34(9):720-2, 2014
                                      20. Lomauri Kh: Congenital chylothorax in newborn with trisomy 21. Georgian Med News. 74-7, 2014
                                      21. Lopez-Gutierrez JC et al: Chylothorax and chylous ascites: management and pitfalls. Semin Pediatr Surg. 23(5):298-302, 2014
                                      22. Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
                                      23. Nadolski GJ et al: Thoracic duct embolization for nontraumatic chylous effusion: experience in 34 patients. Chest. 143(1):158-63, 2013
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Synonyms

                                              • Chylous fluid in pleural space
                                            • Definitions

                                              • Chylothorax: Lymphatic fluid in pleural space secondary to congenital or acquired conditions, including congenital anomaly, ↑ pressure, impaired drainage/obstruction, or trauma to thoracic duct
                                                • Thoracic duct is major central lymphatic vessel
                                                • Extends in paraspinal tissues from cisterna chyli to point of drainage into left subclavian/brachiocephalic vein
                                                • Drains lymph from lower limbs, pelvis, abdomen
                                              • Lymphangiectasia: Abnormal dilation of lymphatics
                                                • 1°: Abnormal development of lymphatics in isolation or with genetic anomaly
                                                • 2°: Related to congenital heart disease (CHD) or repair
                                              • Neonatal/congenital lymphatic flow disorders (central lymphatic conducting channel anomaly)
                                                • Pulmonary lymphatic perfusion syndrome: Abnormal lymphatic flow in lungs & pleura → chylothorax
                                                • Central lymphatic flow disorder: Abnormal flow into additional tissues/cavities → body wall, peritoneum, etc.
                                                • Congenital chylous ascites

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • Ultrasonographic Findings

                                                  • CT Findings

                                                    • MR Findings

                                                      • Lymphangiogram

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Barrera CA et al: Imaging of fetal lymphangiectasias: prenatal and postnatal imaging findings. Pediatr Radiol. 50(13):1872-80, 2020
                                                                          2. Biko DM et al: Pediatric pulmonary lymphatic flow disorders: diagnosis and management. Paediatr Respir Rev. 36:2-7, 2020
                                                                          3. Chavhan GB et al: Magnetic resonance lymphangiography. Radiol Clin North Am. 58(4):693-706, 2020
                                                                          4. Hannah DM et al: In utero congenital chylothorax treatment with fetal thoracoamniotic shunt: case report. J Neonatal Perinatal Med. 13(3):427-30, 2020
                                                                          5. Pinto E et al: Neonatal lymphatic flow disorders: impact of lymphatic imaging and interventions on outcomes. J Perinatol. 41(3):494-501, 2020
                                                                          6. Rubalcava NS et al: Efficacy of early pleurectomy for severe congenital chylothorax. J Surg Res. 256:433-8, 2020
                                                                          7. Zheng Q et al: Quantification of thoracic lymphatic flow patterns using dynamic contrast-enhanced MR lymphangiography. Radiology. 296(1):202-7, 2020
                                                                          8. Biko DM et al: Imaging of central lymphatic abnormalities in Noonan syndrome. Pediatr Radiol. 49(5):586-92, 2019
                                                                          9. De Angelis LC et al: Congenital chylothorax: current evidence-based prenatal and post-natal diagnosis and management. Lymphology. 52(3):108-25, 2019
                                                                          10. Shaikh R et al: MR imaging evaluation of pediatric lymphatics:: overview of techniques and imaging findings. Magn Reson Imaging Clin N Am. 27(2):373-85, 2019
                                                                          11. Biko DM et al: Recognition of neonatal lymphatic flow disorder: fetal MR findings and postnatal mr lymphangiogram correlation. Acad Radiol. 25(11):1446-50, 2018
                                                                          12. Pimpalwar S et al: Dynamic contrast enhanced magnetic resonance lymphangiography: categorization of imaging findings and correlation with patient management. Eur J Radiol. 101:129-35, 2018
                                                                          13. Brock WW et al: Congenital chylothorax: a unique presentation of nonimmune hydrops fetalis in a preterm infant. Adv Neonatal Care. 16(2):114-23, 2016
                                                                          14. Bang JH et al: Anatomic variability of the thoracic duct in pediatric patients with complex congenital heart disease. J Thorac Cardiovasc Surg. 150(3):490-5, 2015
                                                                          15. Bialkowski A et al: Congenital chylothorax: a prospective nationwide epidemiological study in Germany. Arch Dis Child Fetal Neonatal Ed. 100(2):F169-72, 2015
                                                                          16. Krishnamurthy R et al: Imaging the central conducting lymphatics: initial experience with dynamic MR lymphangiography. Radiology. 274(3):871-8, 2015
                                                                          17. Bengtsson BO: Outcome of neonatal chylous effusions: what do you tell the parents? A 10-year experience in a community hospital. Am J Perinatol. 31(12):1037-42, 2014
                                                                          18. Downie L et al: Congenital chylothorax: associations and neonatal outcomes. J Paediatr Child Health. 50(3):234-8, 2014
                                                                          19. Gray M et al: Treatment of congenital pulmonary lymphangiectasia using ethiodized oil lymphangiography. J Perinatol. 34(9):720-2, 2014
                                                                          20. Lomauri Kh: Congenital chylothorax in newborn with trisomy 21. Georgian Med News. 74-7, 2014
                                                                          21. Lopez-Gutierrez JC et al: Chylothorax and chylous ascites: management and pitfalls. Semin Pediatr Surg. 23(5):298-302, 2014
                                                                          22. Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
                                                                          23. Nadolski GJ et al: Thoracic duct embolization for nontraumatic chylous effusion: experience in 34 patients. Chest. 143(1):158-63, 2013