Conjunctival Lymphangiectasis

Introduction

Leber was the first to report and name lymphangiectasis haemorrhagica conjunctivae in 1880.¹ The disease occurs as the result of a connection between conjunctival lymphatic and blood vessels, which gives rise to intermittent rapid filling of the former with blood.² Lymphangiectasis is a clear channel separated by diaphanous septate walls.³ Despite the high rate of spontaneous resolution, lymphangiectasis rarely causes tear film disruption via chronic conjunctival swelling.³

Case Report

A 41-year-old woman presented to the outpatient clinic complaining of intermittent redness, itching, and soreness of the right eye. She had right frontal headache throughout the exacerbation period of the disease. She had no remarkable pertinent history, such as trauma, surgery, irradiation, or diseases. The patient’s best-corrected visual acuity was 20/20 in the right eye (−3.25 sphere −1.00 cylinder) and 20/20 in the left eye (−2.75 sphere −1.00 cylinder). Intraocular pressure was 16 mm Hg in the right eye and 15 mm Hg in the left eye.

Slit-lamp biomicroscopy of the left eye revealed conjunctival swelling, mild hyperemia, dilated lymphatic vessels filled with blood during the exacerbation period, and clear fluid spaces throughout the remission (Figs. 1 and 2). No lymphangiectasis was detected in the fellow eye. The ophthalmoscopic examination of the fundus was normal in both eyes.

Figure 1. Conjunctival lymphangiectasis in the right eye with dilated lymphatic vessels filled with blood.

Figure 2. Transparent conjunctival lymphatic vessels in the right eye, which were previously filled with blood.

Serum thyroid-stimulating hormone level was 0.50 uIU/mL. Serum thyroxine level was 1.25 ng/dL. Hertel exophthalmometry measurement was 19 mm in both eyes. Orbital magnetic resonance imaging revealed normal findings. Tear break-up time was 16 seconds in the right eye and 18 seconds in the left eye. Schirmer’s test scores in 5 minutes were 15 mm in the right eye and 17 mm in the left eye. A diagnosis of left conjunctival lymphangiectasis was made.

Discussion

Lymphangiectasia haemorrhagica conjunctivae is a benign, recurrent disease that resolves spontaneously. Slit-lamp biomicroscopy reveals segmental constriction and balloon-shaped dilatations of conjunctival lymphatics that are already filled with blood.²

Failure of the valvular mechanism of conjunctival lymphatics may be responsible for their filling with blood, although the actual cause remains unknown in most cases.¹ Similarly, we could not find any discernable etiology in our case.

Treatment options for persistent cases with lymphangiectasis are surgical removal of the communication, diathermy, and argon laser photocoagulation.^1,4 A wider conjunctival excision is suggested to prevent recurrence.³ Precautions not to violate limbal stem cells or the inferior fornix should be taken during the surgical procedure.³

Differential diagnosis should include conjunctival edema resulting from previous surgery, infection, or trauma. Conjunctival cyst, conjunctivochalasis, allergic conjunctivitis, elevated hydrostatic pressure due to fluid overload, dysthyroid ophthalmopathy, orbital neoplasm, and elevated osmotic pressure due to hypo-proteinemia should also be mentioned in the differential diagnosis.^5,6 Additionally, subconjunctival hemorrhage, a common cause of conjunctival redness, can be easily differentiated from lymphangiectasis because of its diffuse pattern.²

Ophthalmologists should be aware of lymphangiectasia haemorrhagica conjunctivae as a differential diagnosis for conjunctival swelling or redness.