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Pediatric lymphatic malformations: evolving understanding and therapeutic options

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Abstract

Multimodal treatment of lymphatic malformations continues to expand as new information about the biology and genetics of these lesions is discovered, along with knowledge gained from clinical practice. A patient-centered approach, ideally provided by a multidisciplinary medical and surgical team, should guide timing and modality of treatment. Current treatment options include observation, surgery, sclerotherapy, radiofrequency ablation, and laser therapy. New medical and surgical therapies are emerging, and include sildenafil, propranolol, sirolimus, and vascularized lymph node transfer. The primary focus of management is to support and optimize these patients’ quality of life. Researchers continue to study lymphatic malformations with the goal of increasing therapeutic options and developing effective clinical pathways for these complicated lesions.

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References

  1. Kennedy TL, Whitaker M, Pellitteri P, Wood WE (2001) Cystic hygroma/lymphangioma: a rational approach to management. Laryngoscope 111:1929–1937

    Article  CAS  PubMed  Google Scholar 

  2. Wassef M, Blei F, Adams D et al (2015) Vascular anomalies classification: recommendations from the international society for the study of vascular anomalies. Pediatrics 136:e203–e214

    Article  PubMed  Google Scholar 

  3. Fevurly RD, Fishman SJ (2012) Vascular anomalies in pediatrics. Surg Clin North Am 92:769–800

    Article  PubMed  Google Scholar 

  4. Florez-Vargas A, Vargas SO, Debelenko LV et al (2008) Comparative analysis of D2-40 and LYVE-1 immunostaining in lymphatic malformations. Lymphology 41:103–110

    CAS  PubMed  Google Scholar 

  5. Bruder E, Perez-Atayde AR, Jundt G et al (2009) Vascular lesions of bone in children, adolescents, and young adults. A clinicopathologic reappraisal and application of the ISSVA classification. Virchows Arch 454:161–179

    Article  PubMed  Google Scholar 

  6. Schook CC, Mulliken JB, Fishman SJ et al (2011) Primary lymphedema: clinical features and management in 138 pediatric patients. Plast Reconstr Surg 127:2419–2431

    Article  CAS  PubMed  Google Scholar 

  7. Connell F, Brice G, Mortimer P (2008) Phenotypic characterization of primary lymphedema. Ann N Y Acad Sci 1131:140–146

    Article  PubMed  Google Scholar 

  8. Scholl J, Durfee SM, Russell MA et al (2012) First-trimester cystic hygroma: relationship of nuchal translucency thickness and outcomes. Obstet Gynecol 120:551–559

    Article  PubMed  Google Scholar 

  9. Yadav P, De Castro DK, Waner M et al (2013) Vascular anomalies of the head and neck: a review of genetics. Semin Ophthalmol 28:257–266

    Article  PubMed  Google Scholar 

  10. Brouillard P, Boon L, Vikkula M (2014) Genetics of lymphatic anomalies. J Clin Investig 124:898–904

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Lindhurst MJ, Sapp JC, Teer JK et al (2011) A mosaic activating mutation in AKT1 associated with the Proteus syndrome. N Engl J Med 365:611–619

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Zhou F, Chang Z, Zhang L et al (2010) Akt/Protein kinase B is required for lymphatic network formation, remodeling, and valve development. Am J Pathol 177:2124–2133

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Kurek KC, Luks VL, Ayturk UM et al (2012) Somatic mosaic activating mutations in PIK3CA cause CLOVES syndrome. Am J Hum Genet 90:1108–1115

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Boscolo E, Coma S, Luks VL et al (2015) AKT hyper-phosphorylation associated with PI3K mutations in lymphatic endothelial cells from a patient with lymphatic malformation. Angiogenesis 18:151–162

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Makinen T, Veikkola T, Mustjoki S et al (2001) Isolated lymphatic endothelial cells transduce growth, survival and migratory signals via the VEGF-C/D receptor VEGFR-3. EMBO J 20:4762–4773

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Karkkainen MJ, Haiko P, Sainio K et al (2004) Vascular endothelial growth factor C is required for sprouting of the first lymphatic vessels from embryonic veins. Nat Immunol 5:74–80

    Article  CAS  PubMed  Google Scholar 

  17. Dellinger MT, Hunter RJ, Bernas MJ et al (2007) Chy-3 mice are Vegfc haploinsufficient and exhibit defective dermal superficial to deep lymphatic transition and dermal lymphatic hypoplasia. Dev Dyn 236:2346–2355

    Article  CAS  PubMed  Google Scholar 

  18. Jeltsch M, Kaipainen A, Joukov V et al (1997) Hyperplasia of lymphatic vessels in VEGF-C transgenic mice. Science 276:1423–1425

    Article  CAS  PubMed  Google Scholar 

  19. Villefranc JA, Nicoli S, Bentley K et al (2013) A truncation allele in vascular endothelial growth factor c reveals distinct modes of signaling during lymphatic and vascular development. Development 140:1497–1506

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Connell FC, Ostergaard P, Carver C et al (2009) Analysis of the coding regions of VEGFR3 and VEGFC in Milroy disease and other primary lymphoedemas. Hum Genet 124:625–631

    Article  CAS  PubMed  Google Scholar 

  21. Irrthum A, Karkkainen MJ, Devriendt K et al (2000) Congenital hereditary lymphedema caused by a mutation that inactivates VEGFR3 tyrosine kinase. Am J Hum Genet 67:295–301

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Karkkainen MJ, Ferrell RE, Lawrence EC et al (2000) Missense mutations interfere with VEGFR-3 signalling in primary lymphoedema. Nat Genet 25:153–159

    Article  CAS  PubMed  Google Scholar 

  23. Gordon K, Schulte D, Brice G et al (2013) Mutation in vascular endothelial growth factor-C, a ligand for vascular endothelial growth factor receptor-3, is associated with autosomal dominant milroy-like primary lymphedema. Circ Res 112:956–960

    Article  CAS  PubMed  Google Scholar 

  24. Sevick-Muraca EM, King PD (2014) Lymphatic vessel abnormalities arising from disorders of RAS signal transduction. Trends Cardiovasc Med 24:121–127

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. King PD, Lubeck BA, Lapinski PE (2013) Nonredundant functions for RAS GTPase-activating proteins in tissue homeostasis. Sci Signal 6:re1

    Article  PubMed  CAS  Google Scholar 

  26. Burrows PE, Gonzalez-Garay ML, Rasmussen JC et al (2013) Lymphatic abnormalities are associated with RASA1 gene mutations in mouse and man. Proc Natl Acad Sci USA 110:8621–8626

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Lapinski PE, Kwon S, Lubeck BA et al (2012) RASA1 maintains the lymphatic vasculature in a quiescent functional state in mice. J Clin Investig 122:733–747

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Wu JK, Kitajewski C, Reiley M et al (2015) Aberrant lymphatic endothelial progenitors in lymphatic malformation development. PLoS One 10:e0117352

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  29. Surico D, Amadori R, D’Ajello P et al (2013) Antenatal diagnosis of fetal lymphangioma by ultrasonography. Eur J Obstet Gynecol Reprod Biol 168:236

    Article  PubMed  Google Scholar 

  30. Aslan A, Buyukkaya R, Tan S et al (2013) Efficacy of ultrasonography in lymphatic malformations: diagnosis, treatment and follow-up: a case report. Med Ultrasonogr 15:244–246

    Article  Google Scholar 

  31. Adaletli I, Towbin AJ, Ozbayrak M, Madazli R (2013) Anterior mediastinal lymphangioma: pre- and postnatal sonographic findings. J Clin Ultrasound 41:383–385

    Article  PubMed  Google Scholar 

  32. Koelblinger C, Herold C, Nemec S et al (2013) Fetal magnetic resonance imaging of lymphangiomas. J Perinat Med 41:437–443

    Article  PubMed  Google Scholar 

  33. Calvo-Garcia MA, Kline-Fath BM, Adams DM et al (2015) Imaging evaluation of fetal vascular anomalies. Pediatr Radiol 45:1218–1229

    Article  PubMed  Google Scholar 

  34. Liu NF, Yan ZX, Wu XF (2012) Classification of lymphatic-system malformations in primary lymphoedema based on MR lymphangiography. Eur J Vasc Endovasc Surg 44:345–349

    Article  CAS  PubMed  Google Scholar 

  35. Colletti G, Valassina D, Bertossi D et al (2014) Contemporary management of vascular malformations. J Oral Maxillofac Surg 72:510–528

    Article  PubMed  Google Scholar 

  36. Schoinohoriti OK, Theologie-Lygidakis N, Tzerbos F, Iatrou I (2012) Lymphatic malformations in children and adolescents. J Craniofac Surg 23:1744–1747

    Article  PubMed  Google Scholar 

  37. Bajaj Y, Hewitt R, Ifeacho S, Hartley BE (2011) Surgical excision as primary treatment modality for extensive cervicofacial lymphatic malformations in children. Int J Pediatr Otorhinolaryngol 75:673–677

    Article  CAS  PubMed  Google Scholar 

  38. Berg EE, Sobol SE, Jacobs I (2013) Laryngeal obstruction by cervical and endolaryngeal lymphatic malformations in children: proposed staging system and review of treatment. Ann Otol Rhinol Laryngol 122:575–581

    Article  PubMed  Google Scholar 

  39. Adams MT, Saltzman B, Perkins JA (2012) Head and neck lymphatic malformation treatment: a systematic review. Otolaryngol Head Neck Surg 147:627–639

    Article  PubMed  Google Scholar 

  40. Balakrishnan K, Edwards TC, Perkins JA (2012) Functional and symptom impacts of pediatric head and neck lymphatic malformations: developing a patient-derived instrument. Otolaryngol Head Neck Surg 147:925–931

    Article  PubMed  Google Scholar 

  41. Hill RH III, Shiels WE II, Foster JA et al (2012) Percutaneous drainage and ablation as first line therapy for macrocystic and microcystic orbital lymphatic malformations. Ophthal Plast Reconstr Surg 28:119–125

    Article  PubMed  Google Scholar 

  42. Perkins JA, Manning SC, Tempero RM et al (2010) Lymphatic malformations: review of current treatment. Otolaryngol Head Neck Surg 142:795–803, 803 e791

  43. Puig S, Casati B, Staudenherz A, Paya K (2005) Vascular low-flow malformations in children: current concepts for classification, diagnosis and therapy. Eur J Radiol 53:35–45

    Article  PubMed  Google Scholar 

  44. Tempero RM, Hannibal M, Finn LS et al (2006) Lymphocytopenia in children with lymphatic malformation. Arch Otolaryngol Head Neck Surg 132:93–97

    Article  PubMed  Google Scholar 

  45. Jamal N, Ahmed S, Miller T et al (2012) Doxycycline sclerotherapy for pediatric head and neck macrocystic lymphatic malformations: a case series and review of the literature. Int J Pediatr Otorhinolaryngol 76:1127–1131

    Article  PubMed  Google Scholar 

  46. Sandler G, Adams S, Taylor C (2009) Paediatric vascular birthmarks—the psychological impact and the role of the GP. Aust Fam Physician 38:169–171

    PubMed  Google Scholar 

  47. Kirkham EM, Edwards TC, Weaver EM et al (2015) The lymphatic malformation function (LMF) instrument. Otolaryngol Head Neck Surg 153:656–662

    Article  PubMed  Google Scholar 

  48. Mattila KA, Kervinen K, Kalajoki-Helmio T et al (2015) An interdisciplinary specialist team leads to improved diagnostics and treatment for paediatric patients with vascular anomalies. Acta Paediatr 104:1109–1116

    Article  PubMed  Google Scholar 

  49. Alqahtani A, Nguyen LT, Flageole H et al (1999) 25 Years’ experience with lymphangiomas in children. J Pediatr Surg 34:1164–1168

    Article  CAS  PubMed  Google Scholar 

  50. Mulliken JB, Fishman SJ, Burrows PE (2000) Vascular anomalies. Curr Prob Surg 37:517–584

    Article  CAS  Google Scholar 

  51. Manning SC, Perkins J (2013) Lymphatic malformations. Curr Opin Otolaryngol Head Neck Surg 21:571–575

    Article  PubMed  Google Scholar 

  52. Laje P, Peranteau WH, Hedrick HL et al (2015) Ex utero intrapartum treatment (EXIT) in the management of cervical lymphatic malformation. J Pediatr Surg 50:311–314

    Article  PubMed  Google Scholar 

  53. Sheikh F, Akinkuotu A, Olutoye OO et al (2015) Prenatally diagnosed neck masses: long-term outcomes and quality of life. J Pediatr Surg 50:1210–1213

    Article  PubMed  Google Scholar 

  54. Gilony D, Schwartz M, Shpitzer T et al (2012) Treatment of lymphatic malformations: a more conservative approach. J Pediatr Surg 47:1837–1842

    Article  PubMed  Google Scholar 

  55. Marwan A, Crombleholme TM (2006) The EXIT procedure: principles, pitfalls, and progress. Semin Pediatr Surg 15:107–115

    Article  PubMed  Google Scholar 

  56. Eivazi B, Teymoortash A, Wiegand S et al (2010) Intralesional endoscopy of advanced lymphatic malformations of the head and neck: a new diagnostic approach and a potential therapeutic tool. Arch Otolaryngol Head Neck Surg 136:790–795

    Article  PubMed  Google Scholar 

  57. Gaied F, Emil S (2012) Laparoscopic excision of a gastric lymphatic malformation. Am Surg 78:E232–E234

    PubMed  Google Scholar 

  58. Acevedo JL, Shah RK, Brietzke SE (2008) Nonsurgical therapies for lymphangiomas: a systematic review. Otolaryngol Head Neck Surg 138:418–424

    Article  PubMed  Google Scholar 

  59. Farnoosh S, Don D, Koempel J et al (2015) Efficacy of doxycycline and sodium tetradecyl sulfate sclerotherapy in pediatric head and neck lymphatic malformations. Int J Pediatr Otorhinolaryngol 79:883–887

    Article  PubMed  Google Scholar 

  60. Leung M, Leung L, Fung D et al (2014) Management of the low-flow head and neck vascular malformations in children: the sclerotherapy protocol. Eur J Pediatr Surg 24:97–101

    Article  PubMed  Google Scholar 

  61. Mulligan PR, Prajapati HJ, Martin LG, Patel TH (2014) Vascular anomalies: classification, imaging characteristics and implications for interventional radiology treatment approaches. Br J Radiol 87:20130392

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. Hoff SR, Rastatter JC, Richter GT (2015) Head and neck vascular lesions. Otolaryngol Clin North Am 48:29–45

    Article  PubMed  Google Scholar 

  63. Weitz-Tuoretmaa A, Rautio R, Valkila J et al (2014) Efficacy of OK-432 sclerotherapy in treatment of lymphatic malformations: long-term follow-up results. Eur Arch Otorhinolaryngol 271:385–390

    Article  PubMed  Google Scholar 

  64. Molitch HI, Unger EC, Witte CL, van Sonnenberg E (1995) Percutaneous sclerotherapy of lymphangiomas. Radiology 194:343–347

    Article  CAS  PubMed  Google Scholar 

  65. Shergill A, John P, Amaral JG (2012) Doxycycline sclerotherapy in children with lymphatic malformations: outcomes, complications and clinical efficacy. Pediatr Radiol 42:1080–1088

    Article  PubMed  Google Scholar 

  66. Alomari AI, Karian VE, Lord DJ et al (2006) Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-evaluated improvement. J Vasc Interv Radiol 17:1639–1648

    Article  PubMed  Google Scholar 

  67. Hurewitz AN, Lidonicci K, Wu CL et al (1994) Histologic changes of doxycycline pleurodesis in rabbits. Effect of concentration and pH. Chest 106:1241–1245

    Article  CAS  PubMed  Google Scholar 

  68. Cordes BM, Seidel FG, Sulek M et al (2007) Doxycycline sclerotherapy as the primary treatment for head and neck lymphatic malformations. Otolaryngol Head Neck Surg 137:962–964

    Article  PubMed  Google Scholar 

  69. Nehra D, Jacobson L, Barnes P et al (2008) Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children. J Pediatr Surg 43:451–460

    Article  PubMed  Google Scholar 

  70. Burrows PE, Mitri RK, Alomari A et al (2008) Percutaneous sclerotherapy of lymphatic malformations with doxycycline. Lymphat Res Biol 6:209–216

    Article  CAS  PubMed  Google Scholar 

  71. Luo QF, Gan YH (2013) Pingyangmycin with triamcinolone acetonide effective for treatment of lymphatic malformations in the oral and maxillofacial region. J Craniomaxillofac Surg 41:345–349

    Article  CAS  PubMed  Google Scholar 

  72. Orford J, Barker A, Thonell S et al (1995) Bleomycin therapy for cystic hygroma. J Pediatr Surg 30:1282–1287

    Article  CAS  PubMed  Google Scholar 

  73. Muir T, Kirsten M, Fourie P et al (2004) Intralesional bleomycin injection (IBI) treatment for haemangiomas and congenital vascular malformations. Pediatr Surg Int 19:766–773

    Article  CAS  PubMed  Google Scholar 

  74. Zheng JW, Qin ZP, Zhang ZY (2005) Management of lymphatic malformations in oral and maxillofacial regions: the rationale according to the new classification. Shanghai J Stomatolo 14:553–556

    Google Scholar 

  75. Mai HM, Zheng JW, Zhou Q et al (2013) Intralesional injection of pingyangmycin is a safe and effective treatment for microcystic lymphatic malformations in the tongue. Phlebol Venous Forum R Soc Med 28:147–152

    Article  CAS  Google Scholar 

  76. Bai Y, Jia J, Huang XX et al (2009) Sclerotherapy of microcystic lymphatic malformations in oral and facial regions. J Oral Maxillofac Sur 67:251–256

    Article  Google Scholar 

  77. Shiels WE II, Kang DR, Murakami JW et al (2009) Percutaneous treatment of lymphatic malformations. Otolaryngol Head Neck Surg 141:219–224

    Article  PubMed  Google Scholar 

  78. Balakrishnan K, Menezes MD, Chen BS et al (2014) Primary surgery vs primary sclerotherapy for head and neck lymphatic malformations. Otolaryngol Head Neck Surg 140:41–45

    Article  Google Scholar 

  79. Alghonaim Y, Varshney R, Sands N, Daniel SJ (2012) Coblation technique as an alternative treatment modality for oral lymphangioma. Int J Pediatr Otorhinolaryngol 76:1526–1527

    Article  PubMed  Google Scholar 

  80. Grimmer JF, Mulliken JB, Burrows PE, Rahbar R (2006) Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg 132:1251–1256

    Article  PubMed  Google Scholar 

  81. Leboulanger N, Roger G, Caze A et al (2008) Utility of radiofrequency ablation for haemorrhagic lingual lymphangioma. Int J Pediatr Otorhinolaryngol 72:953–958

    Article  PubMed  Google Scholar 

  82. Balakrishnan K, Perkins J (2012) Management of head and neck lymphatic malformations. Facial Plast Surg 28:596–602

    Article  CAS  PubMed  Google Scholar 

  83. Thottam PJ, Al-Barazi R, Madgy DN, Rozzelle A (2013) Submucosal resection of a microcystic oropharyngeal lymphatic malformation using radiofrequency ablation. Int J Pediatr Otorhinolaryngol 77:1589–1592

    Article  PubMed  Google Scholar 

  84. Kim SW, Kavanagh K, Orbach DB et al (2011) Long-term outcome of radiofrequency ablation for intraoral microcystic lymphatic malformation. Arch Otolaryngol Head Neck Surg 137:1247–1250

    Article  PubMed  Google Scholar 

  85. Colbert SD, Seager L, Haider F et al (2013) Lymphatic malformations of the head and neck-current concepts in management. Br J Oral Maxillofac Surg 51:98–102

    Article  PubMed  Google Scholar 

  86. Civelek S, Sayin I, Ercan I et al (2012) Bipolar radiofrequency-induced interstitial thermoablation for oral cavity vascular malformations: preliminary results in a series of 5 children. Ear Nose Throat J 91:488–492

    PubMed  Google Scholar 

  87. Franca K, Chacon A, Ledon J et al (2013) Lasers for cutaneous congenital vascular lesions: a comprehensive overview and update. Lasers Med Sci 28:1197–1204

    Article  PubMed  Google Scholar 

  88. Shumaker PR, Dela Rosa KM, Krakowski A (2013) Treatment of lymphangioma circumscriptum using fractional carbon dioxide laser ablation. Pediatr Dermatol 30:584–586

    Article  PubMed  Google Scholar 

  89. Torezan LA, Careta MF, Osorio N (2013) Intra-oral lymphangioma successfully treated using fractional carbon dioxide laser. Dermatol Surg 39:816–817

    Article  CAS  PubMed  Google Scholar 

  90. Tsilika K, Bahadoran P, Passeron T (2013) Superficial lymphangioma treated with fractional ablative laser: a case report with clinical and reflectance confocal microscopy evaluation. Dermatol Surg 39:141–143

    Article  CAS  PubMed  Google Scholar 

  91. Hochman M, Adams DM, Reeves TD (2011) Current knowledge and management of vascular anomalies, II: malformations. Arch Facial Plast Surg 13:425–433

    Article  PubMed  Google Scholar 

  92. Waner M (2013) The role of surgery in the management of congenital vascular anomalies. Tech Vasc Interv Radiol 16:45–50

    Article  PubMed  Google Scholar 

  93. Bailin PL, Kantor GR, Wheeland RG (1986) Carbon dioxide laser vaporization of lymphangioma circumscriptum. J Am Acad Dermatol 14:257–262

    Article  CAS  PubMed  Google Scholar 

  94. Smith H, Genesen MC, Feddersen RM (1999) Dermal lymphangiomata of the vulva and laser therapy: a case report and literature review. Eur J Gynaecol Oncol 20:373–378

    CAS  Google Scholar 

  95. Egan CA, Rallis TM, Zone JJ (1998) Multiple scrotal lymphangiomas (lymphangiectases) treated by carbon dioxide laser ablation. Br J Dermatol 139:561–562

    Article  CAS  PubMed  Google Scholar 

  96. Treharne LJ, Murison MS (2006) CO2 laser ablation of lymphangioma circumscriptum of the scrotum. Lymphat Res Biol 4:101–103

    Article  PubMed  Google Scholar 

  97. Lai CH, Hanson SG, Mallory SB (2001) Lymphangioma circumscriptum treated with pulsed dye laser. Pediatr Dermatol 18:509–510

    Article  CAS  PubMed  Google Scholar 

  98. Saluja S, Petersen M, Summers E (2015) Fractional carbon dioxide laser ablation for the treatment of microcystic lymphatic malformations (lymphangioma circumscriptum) in an adult patient with Klippel–Trenaunay syndrome. Lasers Surg Med. doi: 10.1002/lsm.22379

  99. Swetman GL, Berk DR, Vasanawala SS et al (2012) Sildenafil for severe lymphatic malformations. N Engl J Med 366:384–386

    Article  CAS  PubMed  Google Scholar 

  100. Gandhi NG, Lin LK, O’Hara M (2013) Sildenafil for pediatric orbital lymphangioma. JAMA Ophthalmol 131:1228–1230

    Article  PubMed  Google Scholar 

  101. Danial C, Tichy AL, Tariq U et al (2014) An open-label study to evaluate sildenafil for the treatment of lymphatic malformations. J Am Acad Dermatol 70:1050–1057

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  102. Koshy JC, Eisemann BS, Agrawal N et al (2015) Sildenafil for microcystic lymphatic malformations of the head and neck: a prospective study. Int J Pediatr Otorhinolaryngol 79:980–982

    Article  PubMed  Google Scholar 

  103. Rankin H, Zwicker K, Trenor CC III (2015) Caution is recommended prior to sildenafil use in vascular anomalies. Pediatr Blood Cancer 62:2015–2017

    Article  PubMed  Google Scholar 

  104. Ozeki M, Kanda K, Kawamoto N et al (2013) Propranolol as an alternative treatment option for pediatric lymphatic malformation. Tohoku J Exp Med 229:61–66

    Article  CAS  PubMed  Google Scholar 

  105. Maruani A, Brown S, Lorette G et al (2013) Lack of effect of propranolol in the treatment of lymphangioma in two children. Pediatr Dermatol 30:383–385

    Article  PubMed  Google Scholar 

  106. Akyuz C, Atas E, Varan A (2014) Treatment of a tongue lymphangioma with sirolimus after failure of surgical resection and propranolol. Pediatr Blood Cancer 61:931–932

    Article  PubMed  Google Scholar 

  107. Francis CS, Rommer EA, Kane JT et al (2012) Limited-incision surgical debulking of lymphatic malformations using ultrasound-assisted liposuction. Plast Reconstr Surg 130:920e–922e

    Article  CAS  PubMed  Google Scholar 

  108. Mitsukawa N, Satoh K (2012) New treatment for cystic lymphangiomas of the face and neck: cyst wall rupture and cyst aspiration combined with sclerotherapy. J Craniofac Surg 23:1117–1119

    Article  PubMed  Google Scholar 

  109. Raju A, Chang DW (2015) Vascularized lymph node transfer for treatment of lymphedema: a comprehensive literature review. Ann Surg 261(5):1013–1023. doi: 10.1097/SLA.0000000000000763

    Article  PubMed  Google Scholar 

  110. Becker C, Vasile JV, Levine JL et al (2012) Microlymphatic surgery for the treatment of iatrogenic lymphedema. Clin Plast Surg 39:385–398

    Article  PubMed  Google Scholar 

  111. Lin CH, Ali R, Chen SC et al (2009) Vascularized groin lymph node transfer using the wrist as a recipient site for management of postmastectomy upper extremity lymphedema. Plast Reconstr Surg 123:1265–1275

    Article  CAS  PubMed  Google Scholar 

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Ann M. Defnet and Naina Bagrodia have contributed equally.

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Defnet, A.M., Bagrodia, N., Hernandez, S.L. et al. Pediatric lymphatic malformations: evolving understanding and therapeutic options. Pediatr Surg Int 32, 425–433 (2016). https://doi.org/10.1007/s00383-016-3867-4

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