Advertisement

Abnormal Venous Drainage

  • Sanjay P. Prabhu
  • Edward Y. Lee
  • Annabelle Quizon
Chapter

Abstract

Congenital pulmonary venous anomalies, which arise from abnormal embryonic venous development, occur in a diverse spectrum in the pediatric population. Although echocardiography with pulsed Doppler remains the initial investigation of choice in evaluation of the pulmonary venous developmental anomalies particularly in infants and young children, noninvasive imaging studies such as Magnetic Resonance Imaging and Multidetector Computed Tomography are currently playing increasing roles in the initial diagnosis and further characterization of this group of disorders. In this chapter, we review (1) embryology; (2) epidemiology; (3) anatomy; (4) clinical presentation; (5) preoperative imaging evaluation; (6) management; and (7) postoperative imaging evaluation of congenital pulmonary venous anomalies in the pediatric population. Disorders of the lymphatic system encompass a number of entities that may lead to chronic and serious pulmonary disease and manifest with a range of clinical presentations. Congenital errors in lymphatic development are rare and their diagnoses and management are difficult.

Keywords

Pulmonary Vein Left Atrium Pulmonary Vein Stenosis Total Anomalous Pulmonary Venous Connection Pulmonary Venous Obstruction 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Neill CA. Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return. Pediatrics. 1956;18(6):880–7.PubMedGoogle Scholar
  2. 2.
    Healey Jr JE. An anatomic survey of anomalous pulmonary veins: their clinical significance. J Thorac Surg. 1952;23(5):433–44.PubMedGoogle Scholar
  3. 3.
    Streeter GL. Developmental horizons in human embryos; a review of the histogenesis of cartilage and bone. Contrib Embryol. 1949;33(213–221):149–68.PubMedGoogle Scholar
  4. 4.
    Webb S, Kanani M, Anderson RH, et al. Development of the human pulmonary vein and its incorporation in the ­morphologically left atrium. Cardiol Young. 2001;11(6):632–42.PubMedCrossRefGoogle Scholar
  5. 5.
    Delisle G, Ando M, Calder AL, et al. Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J. 1976;91(1):99–122.PubMedCrossRefGoogle Scholar
  6. 6.
    Ucar T, Fitoz S, Tutar E, et al. Diagnostic tools in the preoperative evaluation of children with anomalous pulmonary venous connections. Int J Cardiovasc Imaging. 2008;24(2):229–35.PubMedCrossRefGoogle Scholar
  7. 7.
    Drossner DM, Kim DW, Maher KO, et al. Pulmonary vein stenosis: prematurity and associated conditions. Pediatrics. 2008;122(3):e656–61.PubMedCrossRefGoogle Scholar
  8. 8.
    Latson LA, Prieto LR. Congenital and acquired pulmonary vein stenosis. Circulation. 2007;115(1):103–8.PubMedCrossRefGoogle Scholar
  9. 9.
    Saad EB, Marrouche NF, Saad CP, et al. Pulmonary vein stenosis after catheter ablation of atrial fibrillation: emergence of a new clinical syndrome. Ann Intern Med. 2003;138(8):634–8.PubMedGoogle Scholar
  10. 10.
    Sadr IM, Tan PE, Kieran MW, et al. Mechanism of pulmonary vein stenosis in infants with normally connected veins. Am J Cardiol. 2000;86(5):577–9. A10.PubMedCrossRefGoogle Scholar
  11. 11.
    Michielon G, Di Donato RM, Pasquini L, et al. Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions. Eur J Cardiothorac Surg. 2002;22(2):184–91.PubMedCrossRefGoogle Scholar
  12. 12.
    Hancock Friesen CL, Zurakowski D, Thiagarajan RR, et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg. 2005;79(2):596–606. discussion 596–606.PubMedCrossRefGoogle Scholar
  13. 13.
    van Son JA, Hambsch J, Kinzel P, et al. Urgency of operation in infracardiac total anomalous pulmonary venous connection. Ann Thorac Surg. 2000;70(1):128–30.PubMedCrossRefGoogle Scholar
  14. 14.
    Ussia G, Marasini M, Zannini L, et al. Acquired pulmonary vein obstruction after open-heart surgery. Eur J Cardiothorac Surg. 2002;22(3):465–7.PubMedCrossRefGoogle Scholar
  15. 15.
    Redington AN, Raine J, Shinebourne EA, et al. Tetralogy of Fallot with anomalous pulmonary venous connections: a rare but clinically important association. Br Heart J. 1990;64(5):325–8.PubMedCrossRefGoogle Scholar
  16. 16.
    Foerster SR, Gauvreau K, McElhinney DB, et al. Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome. Pediatr Cardiol. 2008;29(3):536–44.PubMedCrossRefGoogle Scholar
  17. 17.
    Berrocal T, Madrid C, Novo S, et al. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 2004;24(1):e17.PubMedCrossRefGoogle Scholar
  18. 18.
    Hubbard AM, Adzick NS, Crombleholme TM, et al. Congenital chest lesions: diagnosis and characterization with prenatal MR imaging. Radiology. 1999;212(1):43–8.PubMedGoogle Scholar
  19. 19.
    Husain AN, Hessel RG. Neonatal pulmonary hypoplasia: an autopsy study of 25 cases. Pediatr Pathol. 1993;13(4):475–84.PubMedCrossRefGoogle Scholar
  20. 20.
    Montani D, Price LC, Dormuller P, et al. Pulmonary veno-occlusive disease. Eur Respir J. 2009;33(1):189–200.PubMedCrossRefGoogle Scholar
  21. 21.
    Henk CB, Prokesch R, Grampp S, et al. Scimitar syndrome: MR assessment of hemodynamic significance. J Comput Assist Tomogr. 1997;21(4):628–30.PubMedCrossRefGoogle Scholar
  22. 22.
    Ou P, Marini D, Celermajer DS, et al. Non-invasive assessment of congenital pulmonary vein stenosis in children using cardiac-non-gated CT with 64-slice technology. Eur J Radiol. 2009;70(3):595–9.PubMedCrossRefGoogle Scholar
  23. 23.
    Breinholt JP, Hawkins JA, Minich LA, et al. Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome. Ann Thorac Surg. 1999;68(1):164–8.PubMedCrossRefGoogle Scholar
  24. 24.
    Devaney EJ, Chang AC, Ohye RG, et al. Management of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg. 2006;81(3):992–5. discussion 995–6.PubMedCrossRefGoogle Scholar
  25. 25.
    van Son JA, Danielson GK, Puga FJ, et al. Repair of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg. 1995;60(1):144–50.PubMedGoogle Scholar
  26. 26.
    Lacour-Gayet F, Rey C, Planche C. Pulmonary vein stenosis. Description of a sutureless surgical procedure using the pericardium in situ. Arch Mal Coeur Vaiss. 1996;89(5):633–6.PubMedGoogle Scholar
  27. 27.
    Yun TJ, Coles JG, Konstantinov IE, et al. Conventional and sutureless techniques for management of the pulmonary veins: evolution of indications from postrepair pulmonary vein stenosis to primary pulmonary vein anomalies. J Thorac Cardiovasc Surg. 2005;129(1):167–74.PubMedCrossRefGoogle Scholar
  28. 28.
    Lock JE, Bass JL, Castaneda-Zuniga W, et al. Dilation angioplasty of congenital or operative narrowings of venous channels. Circulation. 1984;70(3):457–64.PubMedCrossRefGoogle Scholar
  29. 29.
    Ungerleider RM, Johnston TA, O’Laughlin MP, et al. Intraoperative stents to rehabilitate severely stenotic pulmonary vessels. Ann Thorac Surg. 2001;71(2):476–81.PubMedCrossRefGoogle Scholar
  30. 30.
    Mendeloff EN, Spray TL, Huddleston CB, et al. Lung transplantation for congenital pulmonary vein stenosis. Ann Thorac Surg. 1995;60(4):903–6. discussion 907.PubMedCrossRefGoogle Scholar
  31. 31.
    Kasahara H, Aeba R, Tanami Y, Yozu R. Multislice computed tomography is useful for evaluating partial anomalous pulmonary venous connection. J Cardiothorac Surg. 2010;5:40.PubMedCrossRefGoogle Scholar
  32. 32.
    Grosse-Wortmann L, Al-Otay A, Goo HW, et al. Anatomical and functional evaluation of pulmonary veins in children by magnetic resonance imaging. J Am Coll Cardiol. 2007;49(9):993–1002.PubMedCrossRefGoogle Scholar
  33. 33.
    Valsangiacomo ER, Barrea C, Macgowan CK, et al. Phase-contrast MR assessment of pulmonary venous blood flow in children with surgically repaired pulmonary veins. Pediatr Radiol. 2003;33(9):607–13.PubMedCrossRefGoogle Scholar
  34. 34.
    Gaede C, Trotter C. Congenital chylothorax. Neonatal Radiol. 2006;25:371–81.CrossRefGoogle Scholar
  35. 35.
    Hillard RI, McKendry JB, Philips MH. Congenital abnormalities of the lymphatic system: embryology and pathogenesis. Pediatrics. 1990;86:988–94.Google Scholar
  36. 36.
    Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, et al. State of the art: thoracic lymphangiomas, ­lymphangiectasis, lymphagiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med. 2000;161:1037–46.PubMedGoogle Scholar
  37. 37.
    Mulliken JB, Fishman SJ, Burrows PE. Vascular malformations. Curr Prob Surg. 2000;37(8):520–76.CrossRefGoogle Scholar
  38. 38.
    Brown LR, Reiman HM, Rosenow EC, Glovicki PM, Divertie MB. Intrathoracic lymphangioma. Mayo Clin. 1986;61:882–92.Google Scholar
  39. 39.
    Drut R, Mosca HH. Intrapulmonary cystic lymphangioma. Pediatr Pulmonol. 1996;22:204–6.PubMedCrossRefGoogle Scholar
  40. 40.
    Papsin BC, Evans JNG. Isolated laryngeal lymphangioma: a rare cause of airway obstruction in infants. J Laryngol Otol. 1996;110:969–72.PubMedGoogle Scholar
  41. 41.
    Wright CC, Cohen DM, Vegunta RK, Davis JT, King DR. Intrathoracic cystic hygroma: a report of three cases. J Pediatr Surg. 1996;31:1430–2.PubMedCrossRefGoogle Scholar
  42. 42.
    Hancock BJ, St. Vil D, Luks FI, diLorenzo M, Blanchard H. Complications of lymphangiomas in children. J Pediatr Surg. 1992;27:220–6.PubMedCrossRefGoogle Scholar
  43. 43.
    Levine C. Primary disorders of the lymphatic vessels – a unified concept. J Pediatr Surgery. 1989;24(3):233–40.CrossRefGoogle Scholar
  44. 44.
    Zadvinski DP, Benson MT, Kerr HH, Mancuso AA, Cacciarelli AA, Madrazo BL, et al. Congenital malformations of the cervico-thoracic lymphatic system: embryology and pathogenesis. Radiographics. 1992;12:1175–89.Google Scholar
  45. 45.
    Langer JC, Fitzgerald PG, Desa D, Filly RA, Golbus MS, Adzick S, et al. Cervical cystic hygroma in the fetus: clinical spectrum and outcome. J Pediatr Surg. 1990;25(1):58–62.PubMedCrossRefGoogle Scholar
  46. 46.
    France NE, Brown RJK. Congenital pulmonary lymphangiectasis. Arch Dis Child. 1971;46:528–32.PubMedCrossRefGoogle Scholar
  47. 47.
    Noonan JA, Walkers LR, Reeves JT. Congenital pulmonary lymphangiectasia. Arch Pediatr Adol Med. 1970;120:314–9.CrossRefGoogle Scholar
  48. 48.
    Barker PM, Esther CR, Fordham LA, Maygarden SJ, Funkhouser WK. Primary pulmonary lymphangiectasis in infancy and childhood. Eur Respir J. 2004;24:413–9.PubMedCrossRefGoogle Scholar
  49. 49.
    Chung CJ, Fordham LA, Barker P, Cooper LL. Children with congenital pulmonary lymphangiectasis: after infancy. Am J Radiol. 1999;173:1583–8.Google Scholar
  50. 50.
    Bouchard S, DiLorenzo M, Youssef S, Simard P, Lapierre JG. Pulmonary lymphangiectasia revisited. J Pediatr Surgery. 2000;35(5):796–800.CrossRefGoogle Scholar
  51. 51.
    Dunkelman H, Sharief N, Berman L, Ninan T. Generalized lymphangiomatosis with chylothorax. Arch Dis Child. 1989;64:1058–60.PubMedCrossRefGoogle Scholar
  52. 52.
    Shah AR, Dinwiddie R, Woolf D, Ramani R, Higgins JNP, Matthew DJ. Generalized lymphangiomatosis and chylothorax in the pediatric age group. Pediatr Pulmonol. 1992;14:126–30.PubMedCrossRefGoogle Scholar
  53. 53.
    Smeltzer DM, Stickler GB, Fleming RE. Primary lymphatic dysplasia in children: chylothorax, chylous ascites, and generalized lymphatic dysplasia. Eur J Pediatr. 2004;145:286–92.CrossRefGoogle Scholar
  54. 54.
    Hen J, Dolan TF. Late-onset lymphedema complicated by pericardial effusion, cardiac tamponade, and pleural effusions. Arch Pediatr Adolesc Med. 1981;135:380–1.CrossRefGoogle Scholar
  55. 55.
    Esther CR, Barker PM. Pulmonary lymphangiectasia: diagnosis and clinical course. Pediatr Pulmonol. 2004;38:308–13.PubMedCrossRefGoogle Scholar
  56. 56.
    Dempsey EM, Sant’Anna GM, Williams RL, Brouillette RT. Congenital pulmonary lymphangiectasia presenting as nonimmune fetal hydrops and severe respiratory distress at birth: not uniformly fatal. Pediatr Pulmonol. 2005;40:270–4.PubMedCrossRefGoogle Scholar
  57. 57.
    Witte CL, Witte MH, Unger EC, Williams WH, Bernas MJ, NcNeill GC, et al. Advances in imaging of lymph flow disorders. Radiographics. 2000;20:1697–719.PubMedGoogle Scholar
  58. 58.
    Caro PA, Mahboubi S, Faerber EN. Computed tomography in the diagnosis of lymphangiomas in infants and children. Clin Imaging. 1991;15:41–6.PubMedCrossRefGoogle Scholar
  59. 59.
    Shaffer K, Rosado-de-Christenson ML, Patz EF, Young S, Farver CF. Thoracic lymphangioma in adults: CT and MR imaging features. Amer J Roentgen. 1994;162:283–9.Google Scholar
  60. 60.
    Siegel MJ, Glazer HS, St. Amour TE, Rosenthal DD. Lymphangiomas in children: MR imaging. Radiology. 1989;170:467–70.PubMedGoogle Scholar
  61. 61.
    Nobre LF, Muller NL, deSouza AS, Marchiori E, Souza IV. Congenital pulmonary lymphangiectasia: CT and pathologic findings. J Thorac Imaging. 2004;19:56–9.PubMedCrossRefGoogle Scholar
  62. 62.
    Jang HJ, Lee KS, Han J. Intravascular lymphomatosis of the lung: radiologic findings. J Comput Assist Tomogr. 1998;22:427–9.PubMedCrossRefGoogle Scholar
  63. 63.
    Wunderbaldinger P, Paya K, Partik B, Turetschek K, Hormann M, Horcher E, et al. CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients. Amer J Radiol. 2000;174:827–32.Google Scholar
  64. 64.
    Lynch DA, Hay T, Newell JD, Divgi VD, Fan LL. Pediatric ­diffuse lung diseases: diagnosis and classification using high-resolution CT. Amer J Radiol. 1999;173:713–8.Google Scholar
  65. 65.
    Piu MH, Yueh TC. Lymphoscintigraphy in chyluria, ­chyloperitoneum and chylothorax. J Nucl Med. 1998;39:1292–6.Google Scholar
  66. 66.
    Alqahtani L, Nguyen T, Flageole H, Shaw K, Laberge JM. 25 years’ experience with lymphangioma in children. J Pediatr Surg. 1999;34:1164–8.PubMedCrossRefGoogle Scholar
  67. 67.
    Beghetti M, LaScala G, Belli D, Bugmann P, Kalangas A, LeCoultre C. Etiology and management of pediatric chylothorax. J Pediatr. 2000;136:653–8.PubMedCrossRefGoogle Scholar
  68. 68.
    Buttiker V, Fanconi S, Burger R. Chylothorax in children: guidelines for diagnosis and management. Chest. 1999;116:682–7.PubMedCrossRefGoogle Scholar
  69. 69.
    Stajich GV, Ashworth L. Octreotide. Neonatal Netw. 2006;25:365–9.PubMedCrossRefGoogle Scholar
  70. 70.
    Wolff AB, Silen ML, Kokoska ER, Rodgers BM. Treatment of refractory chylothorax with externalized pleuroperitoneal shunts in children. Ann Thorac Surg. 1999;68:1053–7.PubMedCrossRefGoogle Scholar
  71. 71.
    Valentine VG, Raffin TA. The management of chylothorax. Chest. 1992;102:586–91.PubMedCrossRefGoogle Scholar
  72. 72.
    Rockson SG, Miller LT, Senie R, Brennan MJ, Casley-Smith JR, Foldi E, et al. American cancer society lymphedema workshop. Workshop III: diagnosis and management of lymphedema. Cancer. 1998;83:2882–5.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Sanjay P. Prabhu
    • 1
  • Edward Y. Lee
    • 2
  • Annabelle Quizon
    • 3
  1. 1.Department of RadiologyChildren’s Hospital Boston and Harvard Medical SchoolBostonUSA
  2. 2.Division of Thoracic Imaging, Department of Radiology and Medicine, Pulmonary DivisionChildren’s Hospital BostonBostonUSA
  3. 3.Department of Pediatrics, Division of Pediatric PulmonologyUniversity of Miami/Miller School of Medicine, Batchelor Children’s Research InstituteMiamiUSA

Personalised recommendations