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Imaging of fetal lymphangiectasias: prenatal and postnatal imaging findings

  • Fetal Imaging
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Abstract

Lymphangiectasias are lymphatic malformations characterized by the abnormal dilation and morphology of the lymphatic channels. The classification and treatment of these disorders can be challenging given the limited amount of literature available in children. Various imaging modalities are used to confirm suspected diagnosis, plan the most appropriate treatment, and estimate a prognosis. Prenatal evaluation is performed using both prenatal US imaging and fetal MRI. These modalities are paramount for appropriate parental counseling and planning of perinatal care. During the neonatal period, chest US imaging is a useful modality to evaluate pulmonary lymphangiectasia because other modalities such as conventional radiography and CT display nonspecific findings. Finally, the recent breakthroughs in lymphatic imaging with MRI have allowed us to better classify lymphatic disorders. Dynamic contrast-enhanced lymphangiography, conventional lymphangiography and percutaneous lymphatic procedures offer static and dynamic evaluation of the central conducting lymphatics in children, with excellent spatial resolution and the possibility to provide treatment. The purpose of this review is to discuss the normal and abnormal development of the fetal lymphatic system and how to best depict it by imaging during the prenatal and postnatal life.

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References

  1. Faul JL, Berry GJ, Colby TV et al (2000) Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 161:1037–1046

    Article  CAS  PubMed  Google Scholar 

  2. Laurence KM (1959) Congenital pulmonary lymphangiectasis. J Clin Pathol 12:62–69

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Bellini C, Mazzella M, Campisi C et al (2004) Multimodal imaging in the congenital pulmonary lymphangiectasia–congenital chylothorax–hydrops fetalis continuum. Lymphology 37:22–30

    CAS  PubMed  Google Scholar 

  4. Jacquemont S, Barbarot S, Boceno M et al (2000) Familial congenital pulmonary lymphangectasia, non-immune hydrops fetalis, facial and lower limb lymphedema: confirmation of Njolstad's report. Am J Med Genet 93:264–268

    Article  CAS  PubMed  Google Scholar 

  5. Blei F (2008) Congenital lymphatic malformations. Ann N Y Acad Sci 1131:185–194

    Article  PubMed  Google Scholar 

  6. Malone LJ, Fenton LZ, Weinman JP et al (2015) Pediatric lymphangiectasia: an imaging spectrum. Pediatr Radiol 45:562–569

    Article  PubMed  Google Scholar 

  7. Victoria T, Johnson AM, Edgar JC et al (2016) Comparison between 1.5-T and 3-T MRI for fetal imaging: is there an advantage to imaging with a higher field strength? AJR Am J Roentgenol 206:195–201

    Article  PubMed  Google Scholar 

  8. Pugash D, Brugger PC, Bettelheim D, Prayer D (2008) Prenatal ultrasound and fetal MRI: the comparative value of each modality in prenatal diagnosis. Eur J Radiol 68:214–226

    Article  PubMed  Google Scholar 

  9. Shaikh R, Biko DM, Lee EY (2019) MR imaging evaluation of pediatric lymphatics:: overview of techniques and imaging findings. Magn Reson Imaging Clin N Am 27:373–385

    Article  PubMed  Google Scholar 

  10. Bouchard S, Di Lorenzo M, Youssef S et al (2000) Pulmonary lymphangiectasia revisited. J Pediatr Surg 35:796–800

    Article  CAS  PubMed  Google Scholar 

  11. Yuan SM (2017) Congenital pulmonary lymphangiectasia. J Perinat Med 45:1023–1030

    Article  CAS  PubMed  Google Scholar 

  12. Marugan V, Lopez-Gutierrez J (2016) Primary intestinal lymphangiectasia and its association with generalized lymphatic anomaly: case series and review. J Pediatr Rev 4

  13. International Society for the Study of Vascular Anomalies (2018) ISSVA classification for vascular anomalies. https://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf. Accessed 7 Mar 2020

  14. Chavhan GB, Amaral JG, Temple M, Itkin M (2017) MR lymphangiography in children: technique and potential applications. Radiographics 37:1775–1790

    Article  PubMed  Google Scholar 

  15. Noonan JA, Walters LR, Reeves JT (1970) Congenital pulmonary lymphangiectasis. Am J Dis Child 120:314–319

    CAS  PubMed  Google Scholar 

  16. Reiterer F, Grossauer K, Morris N et al (2014) Congenital pulmonary lymphangiectasis. Paediatr Respir Rev 15:275–280

    PubMed  Google Scholar 

  17. Wagenaar SS, Swierenga J, Wagenvoort CA (1978) Late presentation of primary pulmonary lymphangiectasis. Thorax 33:791–795

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Esther CR Jr, Barker PM (2004) Pulmonary lymphangiectasia: diagnosis and clinical course. Pediatr Pulmonol 38:308–313

    Article  PubMed  Google Scholar 

  19. Oliver G (2004) Lymphatic vasculature development. Nat Rev Immunol 4:35–45

    Article  CAS  PubMed  Google Scholar 

  20. Adams RH, Alitalo K (2007) Molecular regulation of angiogenesis and lymphangiogenesis. Nat Rev Mol Cell Biol 8:464–478

    Article  CAS  PubMed  Google Scholar 

  21. Patan S (2004) Vasculogenesis and angiogenesis. Cancer Treat Res 117:3–32

    Article  CAS  PubMed  Google Scholar 

  22. Wigle JT, Oliver G (1999) Prox1 function is required for the development of the murine lymphatic system. Cell 98:769–778

    Article  CAS  PubMed  Google Scholar 

  23. Cueni LN, Detmar M (2006) New insights into the molecular control of the lymphatic vascular system and its role in disease. J Invest Dermatol 126:2167–2177

    Article  CAS  PubMed  Google Scholar 

  24. Schoenwolf GC, Bleyl SB, Brauer PR, Francis-West PH (2015) Development of the vasculature. In: Bleyl SB, Brauer PR, Francis-West PH, Larsen WJ (eds) Larsen’s human embryology. Elsevier, Philadelphia, pp 304–340

    Google Scholar 

  25. Butler MG, Isogai S, Weinstein BM (2009) Lymphatic development. Birth Defects Res C Embryo Today 87:222–231

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Saul D, Degenhardt K, Iyoob SD et al (2016) Hypoplastic left heart syndrome and the nutmeg lung pattern in utero: a cause and effect relationship or prognostic indicator? Pediatr Radiol 46:483–489

    Article  PubMed  Google Scholar 

  27. Graziano JN, Heidelberger KP, Ensing GJ et al (2002) The influence of a restrictive atrial septal defect on pulmonary vascular morphology in patients with hypoplastic left heart syndrome. Pediatr Cardiol 23:146–151

    Article  CAS  PubMed  Google Scholar 

  28. Rychik J, Rome JJ, Collins MH et al (1999) The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. J Am Coll Cardiol 34:554–560

    Article  CAS  PubMed  Google Scholar 

  29. Bellini C, Boccardo F, Campisi C, Bonioli E (2006) Congenital pulmonary lymphangiectasia. Orphanet J Rare Dis 1:43

    Article  PubMed  PubMed Central  Google Scholar 

  30. Feltes TF, Hansen TN (1989) Effects of an aorticopulmonary shunt on lung fluid balance in the young lamb. Pediatr Res 26:94–97

    Article  CAS  PubMed  Google Scholar 

  31. Datar SA, Johnson EG, Oishi PE et al (2012) Altered lymphatics in an ovine model of congenital heart disease with increased pulmonary blood flow. Am J Physiol Lung Cell Mol Physiol 302:L530–L540

    Article  CAS  PubMed  Google Scholar 

  32. Chitra N, Vijayalakshmi IB (2017) Fetal echocardiography for early detection of congenital heart diseases. J Echocardiogr 15:13–17

    Article  PubMed  Google Scholar 

  33. Victoria T, Andronikou S (2014) The fetal MR appearance of ‘nutmeg lung’: findings in 8 cases linked to pulmonary lymphangiectasia. Pediatr Radiol 44:1237–1242

    Article  PubMed  Google Scholar 

  34. Biko DM, Johnstone JA, Dori Y et al (2018) Recognition of neonatal lymphatic flow disorder: fetal MR findings and postnatal MR lymphangiogram correlation. Acad Radiol 25:1446–1450

    Article  PubMed  Google Scholar 

  35. Seed M, Bradley T, Bourgeois J et al (2009) Antenatal MR imaging of pulmonary lymphangiectasia secondary to hypoplastic left heart syndrome. Pediatr Radiol 39:747–749

    Article  PubMed  Google Scholar 

  36. McElhinney DB, Tworetzky W, Lock JE (2010) Current status of fetal cardiac intervention. Circulation 121:1256–1263

    Article  PubMed  PubMed Central  Google Scholar 

  37. Nobre LF, Muller NL, de Souza Junior AS et al (2004) Congenital pulmonary lymphangiectasia: CT and pathologic findings. J Thorac Imaging 19:56–59

    Article  PubMed  Google Scholar 

  38. Lam CZ, Bhamare TA, Gazzaz T et al (2017) Diagnosis of secondary pulmonary lymphangiectasia in congenital heart disease: a novel role for chest ultrasound and prognostic implications. Pediatr Radiol 47:1441–1451

    Article  PubMed  Google Scholar 

  39. Trinavarat P, Riccabona M (2014) Potential of ultrasound in the pediatric chest. Eur J Radiol 83:1507–1518

    Article  PubMed  Google Scholar 

  40. Dori Y (2016) Novel lymphatic imaging techniques. Tech Vasc Interv Radiol 19:255–261

    Article  PubMed  Google Scholar 

  41. Biko DM, Smith CL, Otero HJ et al (2019) Intrahepatic dynamic contrast MR lymphangiography: initial experience with a new technique for the assessment of liver lymphatics. Eur Radiol 29:5190–5196

    Article  PubMed  Google Scholar 

  42. Biko DM, Reisen B, Otero HJ et al (2019) Imaging of central lymphatic abnormalities in Noonan syndrome. Pediatr Radiol 49:586–592

    Article  PubMed  Google Scholar 

  43. Nadolski G, Itkin M (2013) Thoracic duct embolization for the management of chylothoraces. Curr Opin Pulm Med 19:380–386

    PubMed  Google Scholar 

  44. Chen E, Itkin M (2011) Thoracic duct embolization for chylous leaks. Semin Intervent Radiol 28:63–74

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Dori Y, Keller MS, Rome JJ et al (2016) Percutaneous lymphatic embolization of abnormal pulmonary lymphatic flow as treatment of plastic bronchitis in patients with congenital heart disease. Circulation 133:1160–1170

    Article  PubMed  Google Scholar 

  46. Mon RA, Treadwell MC, Berman DR et al (2018) Outcomes of fetuses with primary hydrothorax that undergo prenatal intervention (prenatal intervention for hydrothorax). J Surg Res 221:121–127

    Article  PubMed  Google Scholar 

  47. Carr BD, Sampang L, Church JT et al (2018) Fetal intervention for congenital chylothorax is associated with improved outcomes in early life. J Surg Res 231:361–365

    Article  PubMed  Google Scholar 

  48. Desai AP, Guvenc BH, Carachi R (2009) Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. Eur J Pediatr Surg 19:241–245

    Article  CAS  PubMed  Google Scholar 

  49. Kuroiwa G, Takayama T, Sato Y et al (2001) Primary intestinal lymphangiectasia successfully treated with octreotide. J Gastroenterol 36:129–132

    Article  CAS  PubMed  Google Scholar 

  50. Ozeki M, Hori T, Kanda K et al (2016) Everolimus for primary intestinal lymphangiectasia with protein-losing enteropathy. Pediatrics 137:e20152562

    Article  PubMed  Google Scholar 

  51. Gray M, Kovatis KZ, Stuart T et al (2014) Treatment of congenital pulmonary lymphangiectasia using ethiodized oil lymphangiography. J Perinatol 34:720–722

    Article  CAS  PubMed  Google Scholar 

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Acknowledgments

The authors would like to thank Dr. Yoav Dori, MD, PhD, director of the Jill and Mark Fishman Center for Lymphatic Disorders, for his contributions and mentorship.

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Authors and Affiliations

  1. Department of Radiology, Children’s Hospital of Philadelphia,, 3401 Civic Center Blvd., Philadelphia, PA, 19104, USA

    Christian A. Barrera, Teresa Victoria & David M. Biko

  2. Department of Radiology, Section of Interventional Radiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Fernando A. Escobar & Ganesh Krishnamurthy

  3. Division of Cardiology, Children’s Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Christopher L. Smith

  4. Center for Fetal Diagnosis and Treatment,, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Julie S. Moldenhauer

  5. Department of Surgery, Perelman School of Medicine,, University of Pennsylvania, Philadelphia, PA, USA

    Julie S. Moldenhauer

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  1. Christian A. Barrera
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  2. Teresa Victoria
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  3. Fernando A. Escobar
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  4. Ganesh Krishnamurthy
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  6. Julie S. Moldenhauer
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Correspondence to Christian A. Barrera.

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Barrera, C.A., Victoria, T., Escobar, F.A. et al. Imaging of fetal lymphangiectasias: prenatal and postnatal imaging findings. Pediatr Radiol 50, 1872–1880 (2020). https://doi.org/10.1007/s00247-020-04673-6

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  • DOI: https://doi.org/10.1007/s00247-020-04673-6

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