Update on Treatment of Orbital Venous-Lymphatic Malformations

International Society for the Study of Vascular Anomalies. (2025) ISSVA classification of vascular Anomalies. issva.org/classification. Accessed 25 Mar 2025.

Harris GJ. Orbital vascular malformations: a consensus statement on terminology and its clinical implications. Orbital society. Am J Ophthalmol. 1999;127:453–5.

CAS  PubMed  Google Scholar 

Nassiri N, Rootman J, Rootman DB, Goldberg RA. Orbital lymphaticovenous malformations: current and future treatments. Surv Ophthalmol. 2015;60:383–405.

Article  PubMed  Google Scholar 

Wiegand S, Eivazi B, Barth PJ, von Rautenfeld DB, Folz BJ, Mandic R, Werner JA. Pathogenesis of lymphangiomas. Virchows Arch. 2008;453:1–8.

Article  PubMed  Google Scholar 

Mäkinen T, Boon LM, Vikkula M, Alitalo K. Lymphatic malformations: Genetics, mechanisms and therapeutic strategies. Circ Res. 2021;129:136–54.

Article  PubMed  Google Scholar 

Osborn AJ, Dickie P, Neilson DE, Glaser K, Lynch KA, Gupta A, Dickie BH. Activating PIK3CA alleles and lymphangiogenic phenotype of lymphatic endothelial cells isolated from lymphatic malformations. Hum Mol Genet. 2015;24:926–38.

Article  CAS  PubMed  Google Scholar 

Petkova M, Ferby I, Mäkinen T. Lymphatic malformations: mechanistic insights and evolving therapeutic frontiers. J Clin Invest. 2024. https://doi.org/10.1172/JCI172844.

Article  PubMed  PubMed Central  Google Scholar 

Harnarayan P, Harnanan D. The Klippel-Trénaunay syndrome in 2022: unravelling its genetic and molecular profile and its link to the limb overgrowth syndromes. Vasc Health Risk Manag. 2022;18:201–9.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Joseph AK, Guerin JB, Eckel LJ, Dalvin LA, Keating GF, Liebo GB, Benson JC, Brinjikji W, Laack NN, Silvera VM. Imaging findings of pediatric orbital masses and tumor mimics. Radiographics. 2022;42:880–97.

Article  PubMed  Google Scholar 

Tunç M, Sadri E, Char DH. Orbital lymphangioma: an analysis of 26 patients. Br J Ophthalmol. 1999;83:76–80.

Article  PubMed  PubMed Central  Google Scholar 

Williams HB. Hemangiomas and lymphangiomas. Adv Surg. 1981;15:317–49.

CAS  PubMed  Google Scholar 

Goto H, Yamakawa N, Komatsu H, Asakage M, Tsubota K, Ueda S-I, Nemoto R, Umazume K, Usui Y, Mori H. Clinico-epidemiological analysis of 1000 cases of orbital tumors. Jpn J Ophthalmol. 2021;65:704–23.

Article  PubMed  Google Scholar 

Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology. 2004;111:997–1008.

Article  PubMed  Google Scholar 

Bonavolontà G, Strianese D, Grassi P, Comune C, Tranfa F, Uccello G, Iuliano A. An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011. Ophthalmic Plast Reconstr Surg. 2013;29:79–86.

Article  PubMed  Google Scholar 

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.

Article  CAS  PubMed  Google Scholar 

Yeager LB, Kassotis A, Frank T, Li CY, Marr BP. A review of pediatric ophthalmic tumors. Pediatr Rev. 2024;45:119–31.

Article  PubMed  Google Scholar 

Katz SE, Rootman J, Vangveeravong S, Graeb D. Combined venous lymphatic malformations of the orbit (so-called lymphangiomas). Association with noncontiguous intracranial vascular anomalies. Ophthalmology. 1998;105:176–84.

Article  CAS  PubMed  Google Scholar 

Gerrie SK, Rajani H, Navarro OM, Lyons CJ, Marie E, Frayn CS, Hughes ECM, Branson HM. Pediatric orbital lesions: non-neoplastic extraocular soft-tissue lesions. Pediatr Radiol. 2024;54:910–21.

Article  PubMed  Google Scholar 

Bisdorff A, Mulliken JB, Carrico J, Robertson RL, Burrows PE. Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations. AJNR Am J Neuroradiol. 2007;28:335–41.

CAS  PubMed  PubMed Central  Google Scholar 

Colafati GS, Piccirilli E, Marrazzo A, et al. Vascular lesions of the pediatric orbit: A radiological walkthrough. Front Pediatr. 2022. https://doi.org/10.3389/fped.2022.734286.

Article  PubMed  PubMed Central  Google Scholar 

Eivazi B, Wiegand S, Negm H, Teymoortash A, Schulze S, Bien S, Werner JA. Orbital and periorbital vascular anomalies – an approach to diagnosis and therapeutic concepts. Acta Otolaryngol. 2010;130:942–51.

Article  PubMed  Google Scholar 

Chadha V, Awan MA, Gonzalez P, Cauchi P, Roberts F, Kemp E. Orbital venous-lymphatic malformation. Eye (Lond). 2009;23:2265–6.

Article  CAS  PubMed  Google Scholar 

Simas N, Farias JP. Orbital lymphangiomas: surgical treatment and clinical outcomes. World Neurosurg. 2014;81:e8425–10.

Article  Google Scholar 

Mulliken JB, Fishman SJ, Burrows PE. Vascular anomalies. Curr Probl Surg. 2000;37:517–84.

Article  CAS  PubMed  Google Scholar 

Lam SC, Yuen HKL. Medical and sclerosing agents in the treatment of orbital lymphatic malformations: what’s new? Curr Opin Ophthalmol. 2019;30:380–5.

Article  PubMed  Google Scholar 

Goble RR, Frangoulis MA. Lymphangioma circumscriptum of the eyelids and conjunctiva. Br J Ophthalmol. 1990;74:574–5.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Boulos PR, Harissi-Dagher M, Kavalec C, Hardy I, Codère F. Intralesional injection of tisseel fibrin glue for resection of lymphangiomas and other thin-walled orbital cysts. Ophthalmic Plast Reconstr Surg. 2005;21:171–6.

Article  PubMed  Google Scholar 

Hayasaki A, Nakamura H, Hamasaki T, Makino K, Yano S, Morioka M, Kuratsu J. Successful treatment of intraorbital lymphangioma with tissue fibrin glue. Surg Neurol. 2009;72:722–4. discussion 724.

Article  PubMed  Google Scholar 

Cheung SSL, Lam SC, Yuen HKL. Transnasal endoscopic sclerotherapy for orbital apex lymphatic malformation. Ophthalmic Plast Reconstr Surg 37:S154–6.

Barnacle AM, Theodorou M, Maling SJ, Abou-Rayyah Y. Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience. Br J Ophthalmol. 2016;100:204–8.

Article  PubMed  Google Scholar 

Kahana A. Subtotal excision with adjunctive sclerosing therapy for the treatment of severe symptomatic orbital lymphangiomas. Arch Ophthalmol. 2011;129:1073.

Article  PubMed  PubMed Central  Google Scholar 

Horbach SER, Lokhorst MM, Saeed P, de Goüyon Matignon de Pontouraude CMF, Rothová A, van der Horst CMAM. Sclerotherapy for low-flow vascular malformations of the head and neck: A systematic review of sclerosing agents. J Plast Reconstr Aesthet Surg. 2016;69:295–304.

Article  PubMed  Google Scholar 

Giguère CM, Bauman NM, Sato Y, Burke DK, Greinwald JH, Pransky S, Kelley P, Georgeson K, Smith RJH. Treatment of lymphangiomas with OK-432 (Picibanil) sclerotherapy: a prospective multi-institutional trial. Arch Otolaryngol Head Neck Surg. 2002;128:1137–44.

Article  PubMed  Google Scholar 

Stuart S, Barnacle AM, Smith G, Pitt M, Roebuck DJ. Neuropathy after sodium tetradecyl sulfate sclerotherapy of venous malformations in children. Radiology. 2015;274:897–905.

Article  PubMed  Google Scholar 

Hurewitz AN, Liang Wu C, Mancuso P, Zucker S. Tetracycline and Doxycycline inhibit pleural fluid metalloproteinases. Chest. 1993;103:1113–7.

Article  CAS  PubMed  Google Scholar 

Cheng J. Doxycycline sclerotherapy in children with head and neck lymphatic malformations. J Pediatr Surg. 2015;50:2143–6.

Comments (0)

No login
gif