Magnetic Resonance Lymphangiography and Lymphatic Embolization in the Treatment of Pulmonary Complication of Lymphatic Malformation

Abstract

Lymphatic malformations (LMs; especially those involving the central conducting lymphatic channels) are characterized by dysplastic and incompetent lymphatic channels in multiple tissues and organs. The major cause of mortality and morbidity in patients with thoracic LM is deterioration of pulmonary function due to chronic chylous effusions and progressive interstitial lung disease. The etiology of these pulmonary processes is unknown, although lymphatic involvement is certain. Understanding of the changes in the lymphatic anatomy in patients with LM has been hindered by difficulty of imaging of the lymphatic system. Recently developed dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) allows dynamic MR imaging of the lymphatic system by injecting gadolinium contrast agent in the groin lymph nodes. Using this technique, pathological lymphatic flow from the central lymphatic system and/or retroperitoneal and mediastinal masses into lung parenchyma (“pulmonary lymphatic perfusion syndrome”) has been demonstrated in patients with LM. This abnormal lymphatic perfusion overflows pulmonary parenchyma and results in deterioration of pulmonary function due to interstitial process and/or compression effect of chylous effusions. Percutaneous thoracic duct embolization or lymphatic interstitial embolization of the lymphatic masses results in cessation of the pulmonary lymphatic overflow and significant improvement in pulmonary symptoms in these patients.

Keywords

lymphangiography - lymphatic malformations - embolization - pulmonary function - interventional radiology

• References

• 1 Wassef M, Blei F, Adams D. , et al; ISSVA Board and Scientific Committee. Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics 2015; 136 (01) e203-e214
• 2 Faul JL, Berry GJ, Colby TV. , et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000; 161 (3, Pt 1): 1037-1046
• 3 Mitsumori LM, McDonald ES, Wilson GJ, Neligan PC, Minoshima S, Maki JH. MR lymphangiography: how I do it. J Magn Reson Imaging 2015; 42 (06) 1465-1477
• 4 Nadolski GJ, Itkin M. Feasibility of ultrasound-guided intranodal lymphangiogram for thoracic duct embolization. J Vasc Interv Radiol 2012; 23 (05) 613-616
• 5 Kerlan Jr RK, Laberge JM. Intranodal lymphangiography: coming soon to a hospital near you. J Vasc Interv Radiol 2012; 23 (05) 617
• 6 Dori Y, Zviman MM, Itkin M. Dynamic contrast-enhanced MR lymphangiography: feasibility study in swine. Radiology 2014; 273 (02) 410-416
• 7 Dori Y, Keller MS, Rychik J, Itkin M. Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient. Pediatrics 2014; 134 (02) e590-e595
• 8 Gray M, Kovatis KZ, Stuart T. , et al. Treatment of congenital pulmonary lymphangiectasia using ethiodized oil lymphangiography. J Perinatol 2014; 34: 720-722
• 9 Itkin M. Interventional treatment of pulmonary lymphatic anomalies. Tech Vasc Interv Radiol 2016; 19 (04) 299-304
• 10 Okuda I, Udagawa H, Takahashi J, Yamase H, Kohno T, Nakajima Y. Magnetic resonance-thoracic ductography: imaging aid for thoracic surgery and thoracic duct depiction based on embryological considerations. Gen Thorac Cardiovasc Surg 2009; 57 (12) 640-646
• 11 Erden A, Fitoz S, Yagmurlu B, Erden I. Abdominal confluence of lymph trunks: detectability and morphology on heavily T2-weighted images. AJR Am J Roentgenol 2005; 184 (01) 35-40
• 12 Dori Y, Keller MS, Fogel MA. , et al. MRI of lymphatic abnormalities after functional single-ventricle palliation surgery. AJR Am J Roentgenol 2014; 203 (02) 426-431
• 13 Malone LJ, Fenton LZ, Weinman JP, Anagnost MR, Browne LP. Pediatric lymphangiectasia: an imaging spectrum. Pediatr Radiol 2015; 45 (04) 562-569
• 14 Brauer RW. Liver circulation and function. Physiol Rev 1963; 43: 115-213
• 15 Dori Y, Keller MS, Rome JJ. , et al. Percutaneous lymphatic embolization of abnormal pulmonary lymphatic flow as treatment of plastic bronchitis in patients with congenital heart disease. Circulation 2016; 133 (12) 1160-1170
• 16 Itkin MG, McCormack FX, Dori Y. Diagnosis and treatment of lymphatic plastic bronchitis in adults using advanced lymphatic imaging and percutaneous embolization. Ann Am Thorac Soc 2016; 13 (10) 1689-1696
• 17 Itkin M. Lymphatic intervention techniques: look beyond thoracic duct embolization. J Vasc Interv Radiol 2016; 27 (08) 1187-1188
• 18 Itkin M, Krishnamurthy G, Naim MY, Bird GL, Keller MS. Percutaneous thoracic duct embolization as a treatment for intrathoracic chyle leaks in infants. Pediatrics 2011; 128 (01) e237-e241
• 19 Baek Y, Won JH, Chang SJ. , et al. Lymphatic embolization for the treatment of pelvic lymphoceles: preliminary experience in five patients. J Vasc Interv Radiol 2016; 27 (08) 1170-1176
• 20 Chick JFB, Reddy SN, Nadolski GJ, Dori Y, Itkin M. Single-session endolymphatic glue embolization of lymphocele after heart transplantation. J Vasc Interv Radiol 2016; 27 (06) 929-930
• 21 Hur S, Shin JH, Lee IJ. , et al. Early experience in the management of postoperative lymphatic leakage using lipiodol lymphangiography and adjunctive glue embolization. J Vasc Interv Radiol 2016; 27 (08) 1177-1186.e1