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Orbital venous-lymphatic malformation

Sir,

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
figure1

(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
figure2

(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.

Comment

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.

References

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Chadha, V., Awan, M., Gonzalez, P. et al. Orbital venous-lymphatic malformation. Eye 23, 2265–2266 (2009). https://doi.org/10.1038/eye.2009.11

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