Lymphatic malformations: Review of current treatment
Corresponding Author
Jonathan A. Perkins DO
Division of Pediatric Otolaryngology-Head and Neck Surgery, Seattle Children's Hospital, Seattle, WA
Department of Otolaryngology-Head and Neck Surgery, University of Washington, Seattle, WA
Corresponding author: Jonathan A. Perkins, DO, Seattle Children's Hospital, Division of Otolaryngology–Head and Neck Surgery, 4800 Sand Point Way N.E./Mailstop W-7729, Seattle, WA 98105-0371. E-mail address: [email protected]Search for more papers by this authorScott C. Manning MD
Division of Pediatric Otolaryngology-Head and Neck Surgery, Seattle Children's Hospital, Seattle, WA
Department of Otolaryngology-Head and Neck Surgery, University of Washington, Seattle, WA
Search for more papers by this authorRichard M. Tempero MD, PhD
Boys Town National Research Hospital, Omaha, NE
Search for more papers by this authorMichael J. Cunningham MD
Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Department of Otology and Laryngology, Harvard Medical School, Boston, MA
Search for more papers by this authorJoseph L. Edmonds Jr. MD, FAAP
Children's ENT of Houston, Houston, TX
Department of Otolaryngology and Division of Plastic Surgery, Baylor College of Medicine, Houston, TX
Department of Otolaryngology, Weill Cornell College of Medicine, New York, NY
Department of Otolaryngology, University of Texas School of Medicine, Houston, TX
Search for more papers by this authorFredric A. Hoffer MD, FAAP, FSIR
Department of Radiology, Seattle Children's Hospital, University of Washington, Seattle, WA
Search for more papers by this authorMark A. Egbert DDS
Department of Oral and Maxillofacial Surgery, Dental Medicine, Seattle Children's Hospital, University of Washington, Seattle, WA
Search for more papers by this authorCorresponding Author
Jonathan A. Perkins DO
Division of Pediatric Otolaryngology-Head and Neck Surgery, Seattle Children's Hospital, Seattle, WA
Department of Otolaryngology-Head and Neck Surgery, University of Washington, Seattle, WA
Corresponding author: Jonathan A. Perkins, DO, Seattle Children's Hospital, Division of Otolaryngology–Head and Neck Surgery, 4800 Sand Point Way N.E./Mailstop W-7729, Seattle, WA 98105-0371. E-mail address: [email protected]Search for more papers by this authorScott C. Manning MD
Division of Pediatric Otolaryngology-Head and Neck Surgery, Seattle Children's Hospital, Seattle, WA
Department of Otolaryngology-Head and Neck Surgery, University of Washington, Seattle, WA
Search for more papers by this authorRichard M. Tempero MD, PhD
Boys Town National Research Hospital, Omaha, NE
Search for more papers by this authorMichael J. Cunningham MD
Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Department of Otology and Laryngology, Harvard Medical School, Boston, MA
Search for more papers by this authorJoseph L. Edmonds Jr. MD, FAAP
Children's ENT of Houston, Houston, TX
Department of Otolaryngology and Division of Plastic Surgery, Baylor College of Medicine, Houston, TX
Department of Otolaryngology, Weill Cornell College of Medicine, New York, NY
Department of Otolaryngology, University of Texas School of Medicine, Houston, TX
Search for more papers by this authorFredric A. Hoffer MD, FAAP, FSIR
Department of Radiology, Seattle Children's Hospital, University of Washington, Seattle, WA
Search for more papers by this authorMark A. Egbert DDS
Department of Oral and Maxillofacial Surgery, Dental Medicine, Seattle Children's Hospital, University of Washington, Seattle, WA
Search for more papers by this authorSponsorships or competing interests that may be relevant to content are disclosed at the end of this article.
This article is the result of a discussion at the American Society of Pediatric Otolaryngology Vascular Anomaly Task Force Head and Neck Lymphatic Malformation Symposium, September 17, 2007, Washington, DC.
Abstract
Objective
Summarize current knowledge of lymphatic malformation medical, sclerotherapy, and surgical treatment; and highlight areas of treatment controversy and treatment difficulty that need improvement.
Methods
Panel presentation of various aspects of lymphatic malformation treatment.
Results
The mainstay of lymphatic malformation treatment has been surgical resection, which has been refined through lesion staging and radiographic characterization. Intralesional sclerotherapy in macrocystic lymphatic malformations is effective. Suprahyoid microcystic lymphatic malformations are more difficult to treat than macrocystic lymphatic malformations in the infrahyoid and posterior cervical regions. Bilateral suprahyoid lymphatic malformations require staged treatment to prevent complications. Lymphatic malformation treatment planning is primarily determined by the presence or possibility of functional compromise. Problematic areas include chronic lymphatic malformation inflammation, dental health maintenance, macroglossia, airway obstruction, and dental malocclusion.
Conclusions
Lymphatic malformation treatment improvements have been made through radiographic characterization and staging of lymphatic malformations. Direct malformation involvement of the upper aerodigestive tract can cause significant functional compromise that is difficult to treat.
References
- 1Kennedy TL, Whitaker M, Pellitteri P, et al. Cystic hygroma/lymphangioma: a rational approach to management. Laryngoscope 2001; 111: 1929–37.
- 2de Serres LM, Sie KC, Richardson MA. Lymphatic malformations of the head and neck. A proposal for staging. Arch Otolaryngol Head Neck Surg 1995; 121: 577–82.
- 3Fliegelman LJ, Friedland D, Brandwein M, et al. Lymphatic malformation: predictive factors for recurrence. Otolaryngol Head Neck Surg 2000; 123: 706–10.
- 4Raveh E, de Jong AL, Taylor GP, et al. Prognostic factors in the treatment of lymphatic malformations. Arch Otolaryngol Head Neck Surg 1997; 123: 1061–5.
- 5Harsha WJ, Perkins JA, Lewis CW, et al. Pediatric admissions and procedures for lymphatic malformations in the United States: 1997 and 2000. Lymphat Res Biol 2005; 3: 58–65.
- 6Perkins JA, Maniglia C, Magit A, et al. Clinical and radiographic findings in children with spontaneous lymphatic malformation regression. Otolaryngol Head Neck Surg 2008; 138: 772–7.
- 7Perkins JA, Garrison M. Variation in lymphatic malformation treatment in pediatric hospitals (unpublished data). 2007.
- 8Bloom DC, Perkins JA, Manning SC. Management of lymphatic malformations. Curr Opin Otolaryngol Head Neck Surg 2004; 12: 500–4.
- 9Borecky N, Gudinchet F, Laurini R, et al. Imaging of cervico-thoracic lymphangiomas in children. Pediatr Radiol 1995; 25: 127–30.
- 10Legiehn GM, Heran MK. Classification, diagnosis, and interventional radiologic management of vascular malformations. Orthop Clin North Am 2006; 37: 435,74, vii–viii.
- 11Hamoir 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: 326–37.
- 12Ricciardelli EJ, Richardson MA. Cervicofacial cystic hygroma. Patterns of recurrence and management of the difficult case. Arch Otolaryngol Head Neck Surg 1991; 117: 546–53.
- 13Charabi B, Bretlau P, Bille M, et al. Cystic hygroma of the head and neck—a long-term follow-up of 44 cases. Acta Otolaryngol Suppl 2000; 543: 248–50.
- 14Riechelmann H, Muehlfay G, Keck T, et al. Total, subtotal, and partial surgical removal of cervicofacial lymphangiomas. Arch Otolaryngol Head Neck Surg 1999 Jun;125: 643–8.
- 15Hartl DM, Roger G, Denoyelle F, et al. Extensive lymphangioma presenting with upper airway obstruction. Arch Otolaryngol Head Neck Surg 2000; 126: 1378–82.
- 16Grimmer JF, Mulliken JB, Burrows PE, et al. Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg 2006; 132: 1251–6.
- 17Roy S, Reyes S, Smith LP. Bipolar radiofrequency plasma ablation (coblation) of lymphatic malformations of the tongue. Int J Pediatr Otorhinolaryngol 2009; 73: 289–93.
- 18Bloom DC, Perkins JA, Manning SC. Management of lymphatic malformations and macroglossia: results of a national treatment survey. Int J Pediatr Otorhinolaryngol 2009; 73: 1114–8.
- 19Bloom DC, Perkins JA, Manning SC. Management of lymphatic malformations and macroglossia: results of a national treatment survey. Int J Pediatr Otorhinolaryngol 2009; 73: 1114–8.
- 20Lee GS, Perkins JA, Oliaei S, et al. Facial nerve anatomy, dissection and preservation in lymphatic malformation management. Int J Pediatr Otorhinolaryngol 2008; 72: 759–66.
- 21Suver DW, Perkins JA, Manning SC. Somatostatin treatment of massive lymphorrhea following excision of a lymphatic malformation. Int J Pediatr Otorhinolaryngol 2004; 68: 845–50.
- 22Fronkalsrud EW. Disorders of the lymphatic system. In: KJ Welch, JG Randolph, MM Ravitch, editors. Pediatric surgery. 4th ed. Chicago: Year Book Medical; 1986. p. 1506–7.
- 23Levine C. Primary disorders of the lymphatic vessels—a unified concept. J Pediatr Surg 1989; 24: 233–40.
- 24Emery PJ, Bailey CM, Evans JN. Cystic hygroma of the head and neck. A review of 37 cases. J Laryngol Otol 1984; 98: 613–9.
- 25Broomhead IW. Cystic hygroma of the neck. Br J Plast Surg 1964; 17: 225–44.
- 26Smith RJ, Burke DK, Sato Y, et al. OK-432 therapy for lymphangiomas. Arch Otolaryngol Head Neck Surg 1996; 122: 1195–9.
- 27Orford J, Barker A, Thonell S, et al. Bleomycin therapy for cystic hygroma. J Pediatr Surg 1995; 30: 1282–7.
- 28Okazaki T, Iwatani S, Yanai T, et al. Treatment of lymphangioma in children: our experience of 128 cases. J Pediatr Surg 2007; 42: 386–9.
- 29Ogita S, Tsuto T, Tokiwa K, et al. Intracystic injection of OK-432: a new sclerosing therapy for cystic hygroma in children. Br J Surg 1987; 74: 690–1.
- 30Smith MC, Zimmerman MB, Burke DK, et al. Efficacy and safety of OK-432 immunotherapy of lymphatic malformations. Laryngoscope 2009; 119: 107–15.
- 31Rautio R, Keski-Nisula L, Laranne J, et al. Treatment of lymphangiomas with OK-432 (picibanil). Cardiovasc Intervent Radiol 2003; 26: 31–6.
- 32Saito M, Ebina T, Koi M, et al. Induction of interferon-gamma in mouse spleen cells by OK-432, a preparation of Streptococcus pyogenes. Cell Immunol 1982; 15;68: 187–92.
- 33Ishida N, Hoshino T. A streptococcal preparation as a potent biological response modifier, OK-432. In: Excerpta Medica. 2nd ed. Amsterdam: 1985. p. 1, 2–5, 26–47, 60–2.
- 34Hall N, Ade-Ajayi N, Brewis C, et al. Is intralesional injection of OK-432 effective in the treatment of lymphangioma in children? Surgery 2003; 133: 238–42.
- 35Muir T, Kirsten M, Fourie P, et al. Intralesional bleomycin injection (IBI) treatment for haemangiomas and congenital vascular malformations. Pediatr Surg Int 2004; 19: 766–73.
- 36Sanlialp I, Karnak I, Tanyel FC, et al. Sclerotherapy for lymphangioma in children. Int J Pediatr Otorhinolaryngol 2003; 67: 795–800.
- 37Shiels WE2nd, Kenney BD, Caniano DA, et al. Definitive percutaneous treatment of lymphatic malformations of the trunk and extremities. J Pediatr Surg 2008; 43: 136–9; discussion 140.
- 38Alomari AI, Karian VE, Lord DJ, et al. Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-evaluated improvement. J Vasc Interv Radiol 2006; 17: 1639–48.
- 39Hoffer FA, Hancock ML, Hinds PS, et al. Pleurodesis for effusions in pediatric oncology patients at end of life. Pediatr Radiol 2007; 37: 269–73.
- 40Kim YC, Oh JH, Yoon Y. An experimental study for efficacy of acetic acid as a sclerosing agent. J Korean Radiol 1997; 37:233, 234–6.
- 41Van Hoof M, Joris JP, Horsmans Y, et al. Acute renal failure requiring haemodialysis after high doses percutaneous acetic acid injection for hepatocellular carcinoma. Acta Gastroenterol Belg 1999; 62: 49–51.
- 42Lee BB, Kim YW, Seo JM, et al. Current concepts in lymphatic malformation. Vasc Endovascular Surg 2005; 39: 67–81.
- 43Dubois J, Garel L, Abela A, et al. Lymphangiomas in children: percutaneous sclerotherapy with an alcoholic solution of zein. Radiology 1997; 204: 651–4.
- 44Edmonds J. Unreleased data from ongoing study at Baylor College of Medicine. 2007. Data accessed September 13, 2007.
- 45Sires BS, Goins CR, Anderson RL, et al. Systemic corticosteroid use in orbital lymphangioma. Ophthal Plast Reconstr Surg 2001; 17: 85–90.
- 46Tempero RM, Hannibal M, Finn LS, et al. Lymphocytopenia in children with lymphatic malformation. Arch Otolaryngol Head Neck Surg 2006; 132: 93–7.
- 47Padwa BL, Hayward PG, Ferraro NF, et al. Cervicofacial lymphatic malformation: clinical course, surgical intervention, and pathogenesis of skeletal hypertrophy. Plast Reconstr Surg 1995; 95: 951–60.
- 48Edwards PD, Rahbar R, Ferraro NF, et al. Lymphatic malformation of the lingual base and oral floor. Plast Reconstr Surg 2005; 115: 1906–15.