Skip to main content

Thank you for visiting You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

Orbital venous-lymphatic malformation


An orbital venous-lymphatic malformation is a rare congenital lesion, the clinical and radiological features of which have been highlighted by Liyanage et al.1 We present our surgical experience and histopathological description of a patient having a similar condition.

Case report

A 39-year-old roofer was seen with a 2-week history of an enlarging vascular conjunctival lesion in the medial canthal area and a diffuse swelling in the temporal area. He also reported the presence of a superonasal anterior orbital swelling since birth. His vision, ocular movements, and position of the eyeball were unaffected. A magnetic resonance imaging (MRI) scan showed a serpiginous superior orbital mass having a vascular communication to the subcutaneous soft tissue mass on the temple (Figure 1). The nature of the lesion was consistent with a vascular malformation or extensive orbital varix. A surgical biopsy was carried out to obtain a tissue diagnosis and partially debulk the visible part of the lesion. The lesion had strong adhesions making dissection difficult. A slow persistent ooze was noticed from the lesion throughout the surgery.

Figure 1
figure 1

(a) T1-weighted coronal view showing an intracoronal large serpiginous soft tissue mass involving the superior aspect of the right orbit (black arrow). (b) T1-weighted coronal view after intravenous contrast showing an uptake of contrast in the right orbital soft tissue mass suggesting vascular/haemangiomatous lesion (black arrow). There is some vascular enhancement with some associated soft tissue in the subcutaneous aspect of the right temple (white arrow). This appears to communicate through a leash of vessels with the right orbital mass.

Histopathology showed numerous dilated interconnecting vascular channels in all the excised tissues, which included the mucosa and submucosa of the conjunctiva, Muller's muscle fibres, fat, and connective tissue (Figure 2). The ramifying abnormally dilated channels were in keeping with a lymphatic-venous malformation or lymphangioma. This corroborated with the clinical and radiological picture.

Figure 2
figure 2

(a) Specimen from superficial plica showing conjunctival mucosa with numerous dilated lymphatic channels in the submucosa (*). H&E × 40. (b) Dense fibrous tissue from the upper fornix showing large dilated presumed lymphatic channels (*). H&E × 100. (c) Further tissue from the upper fornix showing fat, skeletal muscle (skm), and smooth muscle (smm) permeated by lymphatic channels (*). H&E, × 40.


Surgical excision of lymphatic-venous malformations is often difficult because of the intricate architecture of these lesions and the risk of associated haemorrhage.2 Multiple surgical excisions may be required for a satisfactory result. Although complete surgical excision has been reported, cicatrisation and recurrence are known problems.3 Any surgery should be conservative and aimed at obtaining a tissue diagnosis and partial debulking. In our patient, the vision and ocular movements were well-preserved and any extensive surgery would have run a high risk of visual loss and diplopia.

There have been anecdotal reports of success with sclerosing agents such as OK-432 and sodium tetradecyl sulphate.4, 5 A prospective study on six patients found sodium morrhuate 5% to be a very effective sclerosing agent.2 These approaches may be considered in suitable cases to limit surgical intervention.


  1. Liyanage SE, Watson GMT, Wearne MJ . Late ocular manifestation of a childhood venous-lymphatic malformation. Eye 2008; 22: 1194–1195.

    CAS  Article  Google Scholar 

  2. Schwarcz RA, Simon GJB, Cook T, Goldberg RA . Sclerosing therapy as first line treatment for low flow vascular lesions of the orbit. Am J Ophthalmol 2006; 141: 333–339.

    Article  Google Scholar 

  3. Harris GJ, Sakol PJ, Bonavolonta G, De Conciliis C . An analysis of thirty cases of orbital lymphangioma: pathophysiologic considerations and management recommendations. Ophthalmology 1990; 97: 1583–1592.

    CAS  Article  Google Scholar 

  4. Suzuki Y, Obana A, Gohto Y, Miki T, Otuka H, Inoue Y . Management of orbital lymphangioma using intralesional injection of OK-432. Br J Ophthalmol 2000; 84: 614–617.

    CAS  Article  Google Scholar 

  5. Wojno TH . Sotradecol (sodium tetradecyl sulfate) injection of orbital lymphangioma. Ophthal Plast Reconstr Surg 1999; 15: 432–437.

    CAS  Article  Google Scholar 

Download references

Author information

Authors and Affiliations


Rights and permissions

Reprints and Permissions

About this article

Cite this article

Chadha, V., Awan, M., Gonzalez, P. et al. Orbital venous-lymphatic malformation. Eye 23, 2265–2266 (2009).

Download citation

  • Published:

  • Issue Date:

  • DOI:


Quick links