Skip to main content

Respiratory System Lymphatic Disorders

  • Chapter
  • First Online:
Imaging in Pediatric Pulmonology

Abstract

The lymphatic system begins to develop by the end of the sixth embryonic week. By the eighth week, six primary lymph sacs emerge as buds from the adjacent developing veins. The lymphatic vessels develop in a manner similar to that of the blood vessels and join the primitive lymph sacs. By the ninth week of gestation, there are two large lymphatic vessels (the right and left thoracic ducts) that subsequently merge to form the thoracic duct. Failure of the many connections to form between the embryonic lymphatic channels results in congenital malformations of the lymphatic system, alteration in lymphatic drainage, and development of lymphatic disease.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Lee EY, editor. Pediatric radiology: practical imaging evaluation of infants and children. Philadelphia: Wolters Kluwer; 2018. p. 609–39.

    Google Scholar 

  2. Lee EY, editor. Pediatric thoracic imaging. Philadelphia: Wolters Kluwer; 2018. p. 150–219.

    Google Scholar 

  3. White CL, Olivieri B, Restrepo R, McKeon B, Karakas SP, Lee EY. Low-flow vascular malformation pitfalls: from clinical examination to practical imaging evaluation – part 1, lymphatic malformation mimickers. AJR Am J Roentgenol. 2016;206(5):940–51.

    Article  PubMed  Google Scholar 

  4. Kollipara R, Dinneen L, Rentas KE, et al. Vascular anomalies in pediatric patients; updated classification, imaging, and therapy. Radiol Clin N Am. 2013;51(4):659–72.

    Article  PubMed  Google Scholar 

  5. Lowe LH, Marchant TC, Rivard DC, Scherbel AJ. Vascular malformation: classification and terminology the radiologist needs to know. Semin Roentgenol. 2012;47(2):106–17.

    Article  PubMed  Google Scholar 

  6. Semo J, Nicenboim J, Yaniv K. Development of the lymphatic system: new questions and paradigms. Development. 2016;143(6):924–35.

    Article  CAS  PubMed  Google Scholar 

  7. Brouillard P, Boon L, Vikkula M. Genetics of lymphatic anomalies. J Clin Invest. 2014;124(3):898–904.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69(3):412–22.

    Article  CAS  PubMed  Google Scholar 

  9. Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. 2005;32(1):99–116. ix

    Article  PubMed  Google Scholar 

  10. Faul JL, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med. 2000;161(3. Pt 1):1037–46.

    Article  CAS  PubMed  Google Scholar 

  11. Hilliard RI, McKendry JB, Phillips MJ. Congenital abnormalities of the lymphatic system: a new clinical classification. Pediatrics. 1990;86(6):988–94.

    CAS  PubMed  Google Scholar 

  12. Dasgupta R, Fishman SJ. ISSVA classification. Semin Pediatr Surg. 2014;23(4):158–61.

    Article  PubMed  Google Scholar 

  13. Wassef M, et al. Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 2015;136(1):e203–14.

    Article  PubMed  Google Scholar 

  14. Hochman M, Adams DM, Reeves TD. Current knowledge and management of vascular anomalies, II: malformations. Arch Facial Plast Surg. 2011;13(6):425–33.

    Article  PubMed  Google Scholar 

  15. Elluru RG, Azizkhan RG. Cervicofacial vascular anomalies. II. Vascular malformations. Semin Pediatr Surg. 2006;15(2):133–9.

    Article  PubMed  Google Scholar 

  16. de Serres LM, Sie KC, Richardson MA. Lymphatic malformations of the head and neck. A proposal for staging. Arch Otolaryngol Head Neck Surg. 1995;121(5):577–82.

    Article  PubMed  Google Scholar 

  17. Elluru RG, Balakrishnan K, Padua HM. Lymphatic malformations: diagnosis and management. Semin Pediatr Surg. 2014;23(4):178–85.

    Article  PubMed  Google Scholar 

  18. Brown LR, et al. Intrathoracic lymphangioma. Mayo Clin Proc. 1986;61(11):882–92.

    Article  CAS  PubMed  Google Scholar 

  19. Drut R, Mosca HH. Intrapulmonary cystic lymphangioma. Pediatr Pulmonol. 1996;22(3):204–6.

    Article  CAS  PubMed  Google Scholar 

  20. O TM, et al. Lymphatic malformations of the airway. Otolaryngol Head Neck Surg. 2013;149(1):156–60.

    Article  PubMed  Google Scholar 

  21. Hancock BJ, et al. Complications of lymphangiomas in children. J Pediatr Surg. 1992;27(2):220–4; discussion 224–6.

    Article  CAS  PubMed  Google Scholar 

  22. Mishra P, et al. Lymphangioma presenting as cardiac tamponade in a child. J Card Surg. 2015;30(7):613–6.

    Article  PubMed  Google Scholar 

  23. Khobta N, et al. Solitary cystic mediastinal lymphangioma. Eur Respir Rev. 2013;22(127):91–3.

    Article  PubMed  Google Scholar 

  24. Papsin BC, Evans JN. Isolated laryngeal lymphangioma: a rare cause of airway obstruction in infants. J Laryngol Otol. 1996;110(10):969–72.

    Article  CAS  PubMed  Google Scholar 

  25. Wright CC, et al. Intrathoracic cystic hygroma: a report of three cases. J Pediatr Surg. 1996;31(10):1430–2.

    Article  CAS  PubMed  Google Scholar 

  26. Wittekindt C, et al. Lymphatic malformations of the head and neck: introduction of a disease score for children, Cologne Disease Score (CDS). Int J Pediatr Otorhinolaryngol. 2006;70(7):1205–12.

    Article  PubMed  Google Scholar 

  27. Tazelaar HD, et al. Diffuse pulmonary lymphangiomatosis. Hum Pathol. 1993;24(12):1313–22.

    Article  CAS  PubMed  Google Scholar 

  28. Carlson KC, Parnassus WN, Klatt EC. Thoracic lymphangiomatosis. Arch Pathol Lab Med. 1987;111(5):475–7.

    CAS  PubMed  Google Scholar 

  29. Takahashi K, et al. An adult case of lymphangiomatosis of the mediastinum, pulmonary interstitium and retroperitoneum complicated by chronic disseminated intravascular coagulation. Eur Respir J. 1995;8(10):1799–802.

    Article  CAS  PubMed  Google Scholar 

  30. Dunkelman H, et al. Generalised lymphangiomatosis with chylothorax. Arch Dis Child. 1989;64(7):1058–60.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Shah AR, et al. Generalized lymphangiomatosis and chylothorax in the pediatric age group. Pediatr Pulmonol. 1992;14(2):126–30.

    Article  CAS  PubMed  Google Scholar 

  32. Luisi F, Torre O, Harari S. Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis). Eur Respir Rev. 2016;25(140):170–7.

    Article  PubMed  Google Scholar 

  33. Lala S, et al. Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skelet Radiol. 2013;42(7):917–24.

    Article  Google Scholar 

  34. Nikolaou VS, et al. Vanishing bone disease (Gorham-Stout syndrome): a review of a rare entity. World J Orthop. 2014;5(5):694–8.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Aviv RI, McHugh K, Hunt J. Angiomatosis of bone and soft tissue: a spectrum of disease from diffuse lymphangiomatosis to vanishing bone disease in young patients. Clin Radiol. 2001;56(3):184–90.

    Article  CAS  PubMed  Google Scholar 

  36. Dupond JL, et al. Plasma VEGF determination in disseminated lymphangiomatosis-Gorham-Stout syndrome: a marker of activity? A case report with a 5-year follow-up. Bone. 2010;46(3):873–6.

    Article  CAS  PubMed  Google Scholar 

  37. Radhakrishnan K, Rockson SG. Gorham’s disease: an osseous disease of lymphangiogenesis? Ann N Y Acad Sci. 2008;1131:203–5.

    Article  PubMed  Google Scholar 

  38. Lee S, et al. Gorham Stout syndrome (disappearing bone disease): two additional case reports and a review of the literature. Arch Otolaryngol Head Neck Surg. 2003;129(12):1340–3.

    Article  PubMed  Google Scholar 

  39. Abrahams J, et al. Massive osteolysis in an infant. AJR Am J Roentgenol. 1980;135(5):1084–6.

    Article  CAS  PubMed  Google Scholar 

  40. Tie ML, Poland GA, Rosenow EC 3rd. Chylothorax in Gorham’s syndrome. A common complication of a rare disease. Chest. 1994;105(1):208–13.

    Article  CAS  PubMed  Google Scholar 

  41. Toro-Sola MA. Distichiasis-lymphedema syndrome and the Turner phenotype. Bol Asoc Med P R. 1991;83(12):543–4.

    CAS  PubMed  Google Scholar 

  42. Cordasco EM Jr, et al. Clinical features of the yellow nail syndrome. Cleve Clin J Med. 1990;57(5):472–6.

    Article  PubMed  Google Scholar 

  43. Szuba A, Rockson SG. Lymphedema: classification, diagnosis and therapy. Vasc Med. 1998;3(2):145–56.

    Article  CAS  PubMed  Google Scholar 

  44. Schirger A, Harrison EG Jr, Janes JM. Idiopathic lymphedema. Review of 131 cases. JAMA. 1962;182:14–22.

    Article  CAS  PubMed  Google Scholar 

  45. Kinmonth JB, et al. Primary lymphoedema; clinical and lymphangiographic studies of a series of 107 patients in which the lower limbs were affected. Br J Surg. 1957;45(189):1–9.

    Article  CAS  PubMed  Google Scholar 

  46. Browse NL, Stewart G. Lymphoedema: pathophysiology and classification. J Cardiovasc Surg. 1985;26(2):91–106.

    CAS  Google Scholar 

  47. Connell F, et al. A new classification system for primary lymphatic dysplasias based on phenotype. Clin Genet. 2010;77(5):438–52.

    Article  CAS  PubMed  Google Scholar 

  48. Connell FC, et al. The classification and diagnostic algorithm for primary lymphatic dysplasia: an update from 2010 to include molecular findings. Clin Genet. 2013;84(4):303–14.

    Article  CAS  PubMed  Google Scholar 

  49. Connell F, Brice G, Mortimer P. Phenotypic characterization of primary lymphedema. Ann N Y Acad Sci. 2008;1131:140–6.

    Article  PubMed  Google Scholar 

  50. Peyron N, Dandurand M, Guillot B. [Malignant tumors as complications of lymphedema]. J Mal Vasc. 1993;18(4):293–8.

    Google Scholar 

  51. Inde Y, et al. Morphological changes observed via fetal ultrasound in prenatally diagnosed and isolated congenital lymphangiomas: three case reports. J Med Ultrason (2001). 2013;40(3):265–9.

    Article  Google Scholar 

  52. Adaletli I, et al. Anterior mediastinal lymphangioma: pre- and postnatal sonographic findings. J Clin Ultrasound. 2013;41(6):383–5.

    Article  PubMed  Google Scholar 

  53. Koelblinger C, et al. Fetal magnetic resonance imaging of lymphangiomas. J Perinat Med. 2013;41(4):437–43.

    Article  PubMed  Google Scholar 

  54. Greene AK, Perlyn CA, Alomari AI. Management of lymphatic malformations. Clin Plast Surg. 2011;38(1):75–82.

    Article  PubMed  Google Scholar 

  55. Esther CR Jr, Barker PM. Pulmonary lymphangiectasia: diagnosis and clinical course. Pediatr Pulmonol. 2004;38(4):308–13.

    Article  PubMed  Google Scholar 

  56. Dempsey EM, et al. Congenital pulmonary lymphangiectasia presenting as nonimmune fetal hydrops and severe respiratory distress at birth: not uniformly fatal. Pediatr Pulmonol. 2005;40(3):270–4.

    Article  CAS  PubMed  Google Scholar 

  57. Witte CL, et al. Advances in imaging of lymph flow disorders. Radiographics. 2000;20(6):1697–719.

    Article  CAS  PubMed  Google Scholar 

  58. Levine C. Primary disorders of the lymphatic vessels--a unified concept. J Pediatr Surg. 1989;24(3):233–40.

    Article  CAS  PubMed  Google Scholar 

  59. Caro PA, Mahboubi S, Faerber EN. Computed tomography in the diagnosis of lymphangiomas in infants and children. Clin Imaging. 1991;15(1):41–6.

    Article  CAS  PubMed  Google Scholar 

  60. Shaffer K, et al. Thoracic lymphangioma in adults: CT and MR imaging features. AJR Am J Roentgenol. 1994;162(2):283–9.

    Article  CAS  PubMed  Google Scholar 

  61. Siegel MJ, et al. Lymphangiomas in children: MR imaging. Radiology. 1989;170(2):467–70.

    Article  CAS  PubMed  Google Scholar 

  62. Bouchard S, et al. Pulmonary lymphangiectasia revisited. J Pediatr Surg. 2000;35(5):796–800.

    Article  CAS  PubMed  Google Scholar 

  63. Nobre LF, et al. Congenital pulmonary lymphangiectasia: CT and pathologic findings. J Thorac Imaging. 2004;19(1):56–9.

    Article  PubMed  Google Scholar 

  64. Jang HJ, Lee KS, Han J. Intravascular lymphomatosis of the lung: radiologic findings. J Comput Assist Tomogr. 1998;22(3):427–9.

    Article  CAS  PubMed  Google Scholar 

  65. Wunderbaldinger P, et al. CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients. AJR Am J Roentgenol. 2000;174(3):827–32.

    Article  CAS  PubMed  Google Scholar 

  66. Lynch DA, et al. Pediatric diffuse lung disease: diagnosis and classification using high-resolution CT. AJR Am J Roentgenol. 1999;173(3):713–8.

    Article  CAS  PubMed  Google Scholar 

  67. Pui MH, Yueh TC. Lymphoscintigraphy in chyluria, chyloperitoneum and chylothorax. J Nucl Med. 1998;39(7):1292–6.

    CAS  PubMed  Google Scholar 

  68. Bagrodia N, Defnet AM, Kandel JJ. Management of lymphatic malformations in children. Curr Opin Pediatr. 2015;27(3):356–63.

    Article  PubMed  Google Scholar 

  69. Smith MC, et al. Efficacy and safety of OK-432 immunotherapy of lymphatic malformations. Laryngoscope. 2009;119(1):107–15.

    Article  CAS  PubMed  Google Scholar 

  70. Nehra D, et al. Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children. J Pediatr Surg. 2008;43(3):451–60.

    Article  PubMed  Google Scholar 

  71. Burrows PE, Mason KP. Percutaneous treatment of low flow vascular malformations. J Vasc Interv Radiol. 2004;15(5):431–45.

    Article  PubMed  Google Scholar 

  72. Poldervaart MT, et al. Treatment of lymphatic malformations with OK-432 (Picibanil): review of the literature. J Craniofac Surg. 2009;20(4):1159–62.

    Article  PubMed  Google Scholar 

  73. Berenguer B, et al. Sclerotherapy of craniofacial venous malformations: complications and results. Plast Reconstr Surg. 1999;104(1):1–11; discussion 12–5.

    Article  CAS  PubMed  Google Scholar 

  74. Fliegelman LJ, et al. Lymphatic malformation: predictive factors for recurrence. Otolaryngol Head Neck Surg. 2000;123(6):706–10.

    Article  CAS  PubMed  Google Scholar 

  75. Goswamy J, et al. Radiofrequency ablation in the treatment of paediatric microcystic lymphatic malformations. J Laryngol Otol. 2013;127(3):279–84.

    Article  CAS  PubMed  Google Scholar 

  76. Grimmer JF, et al. Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg. 2006;132(11):1251–6.

    Article  PubMed  Google Scholar 

  77. Niti K, Manish P. Microcystic lymphatic malformation (lymphangioma circumscriptum) treated using a minimally invasive technique of radiofrequency ablation and sclerotherapy. Dermatol Surg. 2010;36(11):1711–7.

    Article  PubMed  CAS  Google Scholar 

  78. Savas JA, et al. Carbon dioxide laser for the treatment of microcystic lymphatic malformations (lymphangioma circumscriptum): a systematic review. Dermatol Surg. 2013;39(8):1147–57.

    Article  CAS  PubMed  Google Scholar 

  79. Glade RS, Buckmiller LM. CO2 laser resurfacing of intraoral lymphatic malformations: a 10-year experience. Int J Pediatr Otorhinolaryngol. 2009;73(10):1358–61.

    Article  PubMed  Google Scholar 

  80. Swetman GL, et al. Sildenafil for severe lymphatic malformations. N Engl J Med. 2012;366(4):384–6.

    Article  CAS  PubMed  Google Scholar 

  81. Danial C, et al. An open-label study to evaluate sildenafil for the treatment of lymphatic malformations. J Am Acad Dermatol. 2014;70(6):1050–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  82. Hammill AM, et al. Sirolimus for the treatment of complicated vascular anomalies in children. Pediatr Blood Cancer. 2011;57(6):1018–24.

    Article  PubMed  Google Scholar 

  83. Adams DM, et al. Efficacy and Safety of Sirolimus in the Treatment of Complicated Vascular Anomalies. Pediatrics. 2016;137(2):e20153257.

    Article  PubMed  PubMed Central  Google Scholar 

  84. Laforgia N, et al. Lymphatic malformation in newborns as the first sign of diffuse lymphangiomatosis: successful treatment with sirolimus. Neonatology. 2016;109(1):52–5.

    Article  CAS  PubMed  Google Scholar 

  85. Margolin JF, Soni HM, Pimpalwar S. Medical therapy for pediatric vascular anomalies. Semin Plast Surg. 2014;28(2):79–86.

    Article  PubMed  PubMed Central  Google Scholar 

  86. Raju A, Chang DW. Vascularized lymph node transfer for treatment of lymphedema: a comprehensive literature review. Ann Surg. 2015;261(5):1013–23.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Edward Y. Lee .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Quizon, A., Lee, E.Y., Krone, K. (2020). Respiratory System Lymphatic Disorders. In: Cleveland, R., Lee, E. (eds) Imaging in Pediatric Pulmonology. Springer, Cham. https://doi.org/10.1007/978-3-030-23979-4_14

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-23979-4_14

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-23978-7

  • Online ISBN: 978-3-030-23979-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics