Facial Plast Surg 2012; 28(06): 596-602
DOI: 10.1055/s-0032-1329934
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Management of Head and Neck Lymphatic Malformations

Karthik Balakrishnan
1   Department of Otolaryngology–Head and Neck Surgery, University of Washington, Seattle, Washington
,
Jonathan Perkins
2   Division of Pediatric Otolaryngology, Seattle Children's Hospital, Seattle, Washington
3   Department of Otolaryngology–Head and Neck Surgery, University of Washington, Seattle, Washington
› Author Affiliations
Further Information

Publication History

Publication Date:
27 November 2012 (online)

Abstract

Lymphatic malformations are congenital vascular malformations most commonly found in the head and neck and typically presenting early in life. Although this disease entity has been recognized for more than a century, there is currently no standardization of disease staging or classification, of treatment paradigms, or of outcome measures. A wide variety of treatment modalities have been proposed. This article reviews diagnostic, evaluation, and treatment strategies for head and neck lymphatic malformations. It also provides the reader specific information to improve treatment outcomes in patients with these lesions. Finally, it advocates for standardization of LM assessment and treatment.

 
  • References

  • 1 Kennedy TL, Whitaker M, Pellitteri P, Wood WE. Cystic hygroma/lymphangioma: a rational approach to management. Laryngoscope 2001; 111 (11 Pt 1) 1929-1937
  • 2 Perkins JA, Manning SC, Tempero RM , et al. Lymphatic malformations: current cellular and clinical investigations. Otolaryngol Head Neck Surg 2010; 142: 789-794
  • 3 Renton JP, Smith RJH. Current treatment paradigms in the management of lymphatic malformations. Laryngoscope 2011; 121: 56-59
  • 4 Smith RJ, Burke DK, Sato Y, Poust RI, Kimura K, Bauman NM. OK-432 therapy for lymphangiomas. Arch Otolaryngol Head Neck Surg 1996; 122: 1195-1199
  • 5 Bloom DC, Perkins JA, Manning SC. Management of lymphatic malformations. Curr Opin Otolaryngol Head Neck Surg 2004; 12: 500-504
  • 6 Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982; 69: 412-422
  • 7 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: 577-582
  • 8 Hamoir M, Plouin-Gaudon I, Rombaux P , et al. Lymphatic malformations of the head and neck: a retrospective review and a support for staging. Head Neck 2001; 23: 326-337
  • 9 Hartl DM, Roger G, Denoyelle F, Nicollas R, Triglia J-M, Garabedian E-N. Extensive lymphangioma presenting with upper airway obstruction. Arch Otolaryngol Head Neck Surg 2000; 126: 1378-1382
  • 10 Ricciardelli EJ, Richardson MA. Cervicofacial cystic hygroma. Patterns of recurrence and management of the difficult case. Arch Otolaryngol Head Neck Surg 1991; 117: 546-553
  • 11 Wittekindt C, Michel O, Streppel M , 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: 1205-1212
  • 12 Wiegand S, Eivazi B, Zimmermann AP, Sesterhenn AM, Werner JA. Evaluation of children with lymphatic malformations of the head and neck using the Cologne Disease Score. Int J Pediatr Otorhinolaryngol 2009; 73: 955-958
  • 13 Balakrishnan K, Majesky M, Perkins JA. Head and neck lymphatic tumors and bony abnormalities: a clinical and molecular review. Lymphat Res Biol 2011; 9: 205-212
  • 14 Smith RJH. Lymphatic malformations. Lymphat Res Biol 2004; 2: 25-31
  • 15 Steigman SA, Nemes L, Barnewolt CE , et al. Differential risk for neonatal surgical airway intervention in prenatally diagnosed neck masses. J Pediatr Surg 2009; 44: 76-79
  • 16 Trán-Ngoc-Ninh, Trán-Xuân-Ninh. Cystic hygroma in children: a report of 126 cases. J Pediatr Surg 1974; 9: 191-195
  • 17 Perkins JA, Manning SC, Tempero RM , et al. Lymphatic malformations: review of current treatment. Otolaryngol Head Neck Surg 2010; 142: 795-803 , 803.e1; review
  • 18 Gallagher PG, Mahoney MJ, Gosche JR. Cystic hygroma in the fetus and newborn. Semin Perinatol 1999; 23: 341-356
  • 19 Koeller KK, Alamo L, Adair CF, Smirniotopoulos JG. Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics 1999; 19: 121-146 ; quiz 152–153
  • 20 Stefini S, Bazzana T, Smussi C , et al. EXIT (Ex utero Intrapartum Treatment) in lymphatic malformations of the head and neck: discussion of three cases and proposal of an EXIT-TTP (Team Time Procedure) list. Int J Pediatr Otorhinolaryngol 2012; 76: 20-27
  • 21 Lazar DA, Olutoye OO, Moise Jr KJ , et al. Ex-utero intrapartum treatment procedure for giant neck masses—fetal and maternal outcomes. J Pediatr Surg 2011; 46: 817-822
  • 22 Kathary N, Bulas DI, Newman KD, Schonberg RL. MRI imaging of fetal neck masses with airway compromise: utility in delivery planning. Pediatr Radiol 2001; 31: 727-731
  • 23 Hubbard AM, Crombleholme TM, Adzick NS. Prenatal MRI evaluation of giant neck masses in preparation for the fetal exit procedure. Am J Perinatol 1998; 15: 253-257
  • 24 Santos XM, Papanna R, Johnson A , et al. The use of combined ultrasound and magnetic resonance imaging in the detection of fetal anomalies. Prenat Diagn 2010; 30: 402-407
  • 22 Perkins JA, Sidhu M, Manning SC, Ghioni V, Sze R. Three-dimensional CT angiography imaging of vascular tumors of the head and neck. Int J Pediatr Otorhinolaryngol 2005; 69: 319-325
  • 23 Lee JY, Choi JY, Kim YH , et al. Characterization of congenital lymphatic and blood vascular malformations in the head and neck using blood pool scintigraphy and spect. Lymphology 2010; 43: 149-157
  • 24 Lev S, Lev MH. Imaging of cystic lesions. Radiol Clin North Am 2000; 38: 1013-1027
  • 25 Lloyd C, McHugh K. The role of radiology in head and neck tumours in children. Cancer Imaging 2010; 10: 49-61
  • 26 Harsha WJ, Perkins JA, Lewis CW, Manning SC. Pediatric admissions and procedures for lymphatic malformations in the United States: 1997 and 2000. Lymphat Res Biol 2005; 3: 58-65
  • 27 Adams MT, Saltzman B, Perkins JA. Head and neck lymphatic malformation treatment: a systematic review. Otolaryngol Head Neck Surg 2012; 147: 627-639 ; Epub ahead of print
  • 28 Bloom DC, Perkins JA, Manning SC. Management of lymphatic malformations and macroglossia: results of a national treatment survey. Int J Pediatr Otorhinolaryngol 2009; 73: 1114-1118
  • 29 Thompson DM, Kasperbauer JL. Congenital cystic hygroma involving the larynx presenting as an airway emergency. J Natl Med Assoc 1994; 86: 629-632
  • 30 Bhatt N, Perakis H, Watts TL, Borders JC. Traumatic hemorrhage and rapid expansion of a cervical lymphatic malformation. Ear Nose Throat J 2011; 90: 20-22
  • 31 Greinwald Jr J, Cohen AP, Hemanackah S, Azizkhan RG. Massive lymphatic malformations of the head, neck, and chest. J Otolaryngol Head Neck Surg 2008; 37: 169-173
  • 32 Greene AK, Burrows PE, Smith L, Mulliken JB. Periorbital lymphatic malformation: clinical course and management in 42 patients. Plast Reconstr Surg 2005; 115: 22-30
  • 33 Eivazi B, Teymoortash A, Wiegand S , et al. 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 2010; 136: 790-795
  • 34 Bajaj Y, Hewitt R, Ifeacho S, Hartley BEJ. Surgical excision as primary treatment modality for extensive cervicofacial lymphatic malformations in children. Int J Pediatr Otorhinolaryngol 2011; 75: 673-677
  • 35 Raveh E, de Jong AL, Taylor GP, Forte V. Prognostic factors in the treatment of lymphatic malformations. Arch Otolaryngol Head Neck Surg 1997; 123: 1061-1065
  • 36 Boardman SJ, Cochrane LA, Roebuck D, Elliott MJ, Hartley BEJ. Multimodality treatment of pediatric lymphatic malformations of the head and neck using surgery and sclerotherapy. Arch Otolaryngol Head Neck Surg 2010; 136: 270-276
  • 37 Fliegelman LJ, Friedland D, Brandwein M, Rothschild M. Lymphatic malformation: predictive factors for recurrence. Otolaryngol Head Neck Surg 2000; 123: 706-710
  • 38 Riechelmann H, Muehlfay G, Keck T, Mattfeldt T, Rettinger G. Total, subtotal, and partial surgical removal of cervicofacial lymphangiomas. Arch Otolaryngol Head Neck Surg 1999; 125: 643-648
  • 39 Dasgupta R, Adams D, Elluru R, Wentzel MS, Azizkhan RG. Noninterventional treatment of selected head and neck lymphatic malformations. J Pediatr Surg 2008; 43: 869-873
  • 40 Wiegand S, Zimmermann AP, Eivazi B, Sesterhenn AM, Werner JA. Lymphatic malformations involving the parotid gland. Eur J Pediatr Surg 2011; 21: 242-245
  • 41 Chiara J, Kinney G, Slimp J, Lee GS, Oliaei S, Perkins JA. Facial nerve mapping and monitoring in lymphatic malformation surgery. Int J Pediatr Otorhinolaryngol 2009; 73: 1348-1352
  • 42 Lee GS, Perkins JA, Oliaei S, Manning SC. Facial nerve anatomy, dissection and preservation in lymphatic malformation management. Int J Pediatr Otorhinolaryngol 2008; 72: 759-766
  • 43 Wiegand S, Eivazi B, Zimmermann AP, Sesterhenn AM, Werner JA. Sclerotherapy of lymphangiomas of the head and neck. Head Neck 2011; 33: 1649-1655
  • 44 Smith MC, Zimmerman MB, Burke DK, Bauman NM, Sato Y, Smith RJ. OK-432 Collaborative Study Group. Efficacy and safety of OK-432 immunotherapy of lymphatic malformations. Laryngoscope 2009; 119: 107-115
  • 45 Poldervaart MT, Breugem CC, Speleman L, Pasmans S. Treatment of lymphatic malformations with OK-432 (Picibanil): review of the literature. J Craniofac Surg 2009; 20: 1159-1162
  • 46 Shiels II WE, Kang DR, Murakami JW, Hogan MJ, Wiet GJ. Percutaneous treatment of lymphatic malformations. Otolaryngol Head Neck Surg 2009; 141: 219-224
  • 47 Impellizzeri P, Romeo C, Borruto FA , et al. Sclerotherapy for cervical cystic lymphatic malformations in children. Our experience with computed tomography-guided 98% sterile ethanol insertion and a review of the literature. J Pediatr Surg 2010; 45: 2473-2478
  • 48 Cahill AM, Nijs E, Ballah D , et al. Percutaneous sclerotherapy in neonatal and infant head and neck lymphatic malformations: a single center experience. J Pediatr Surg 2011; 46: 2083-2095
  • 49 Kim SW, Kavanagh K, Orbach DB, Alomari AI, Mulliken JB, Rahbar R. Long-term outcome of radiofrequency ablation for intraoral microcystic lymphatic malformation. Arch Otolaryngol Head Neck Surg 2011; 137: 1247-1250
  • 50 Perkins JA, Maniglia C, Magit A, Sidhu M, Manning SC, Chen EY. Clinical and radiographic findings in children with spontaneous lymphatic malformation regression. Otolaryngol Head Neck Surg 2008; 138: 772-777
  • 51 Burezq H, Williams B, Chitte SA. Management of cystic hygromas: 30 year experience. J Craniofac Surg 2006; 17: 815-818
  • 52 Betz CS, Jäger HR, Brookes JAS, Richards R, Leunig A, Hopper C. Interstitial photodynamic therapy for a symptom-targeted treatment of complex vascular malformations in the head and neck region. Lasers Surg Med 2007; 39: 571-582
  • 53 Jerjes W, Upile T, Hamdoon Z , et al. Interstitial PDT for vascular anomalies. Lasers Surg Med 2011; 43: 357-365
  • 54 Glade RS, Buckmiller LM. CO2 laser resurfacing of intraoral lymphatic malformations: a 10-year experience. Int J Pediatr Otorhinolaryngol 2009; 73: 1358-1361
  • 55 Wiegand S, Eivazi B, Zimmermann AP , et al. Microcystic lymphatic malformations of the tongue: diagnosis, classification, and treatment. Arch Otolaryngol Head Neck Surg 2009; 135: 976-983
  • 56 Grimmer JF, Mulliken JB, Burrows PE, Rahbar R. Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg 2006; 132: 1251-1256
  • 57 Tempero RM, Hannibal M, Finn LS, Manning SC, Cunningham ML, Perkins JA. Lymphocytopenia in children with lymphatic malformation. Arch Otolaryngol Head Neck Surg 2006; 132: 93-97
  • 58 Perkins JA, Tempero RM, Hannibal MC, Manning SC. Clinical outcomes in lymphocytopenic lymphatic malformation patients. Lymphat Res Biol 2007; 5: 169-174
  • 59 Padwa BL, Hayward PG, Ferraro NF, Mulliken JB. Cervicofacial lymphatic malformation: clinical course, surgical intervention, and pathogenesis of skeletal hypertrophy. Plast Reconstr Surg 1995; 95: 951-960