Sir,

First described by Leber in 1880, lymphangiectasia haemorrhagica conjunctivae (LHC) is a rare condition in which the lymphatic channels in the bulbar conjunctiva are dilated and filled with blood.1, 2 Only a few cases of spontaneous attacks followed by rapid resolution have been reported.1, 2, 3, 4

Case report

An otherwise healthy 36-year-old woman, with no previously known ophthalmic or systemic pathology complained of spontaneous ocular redness and swelling in her right eye. Slit-lamp biomicroscopy revealed a circumferential (360°) engorgement of the bulbar conjunctival vessels, which appeared as worm-like conjunctival lymph vessels filled with blood (Figure 1). The clinical picture was consistent with the diagnosis of LHC. Cranial computed tomography scan was unremarkable. The blood took 4 weeks to clear. Varicose tortuous and transparent lymphatic vessels at the site of previously blood-filled vessels were noted as linear bulbar swelling with the classic ‘string of pearls’ appearance (Figure 2). No recurrence was observed after 18-month follow-up.

Figure 1
figure 1

A circumferential (360°) engorgement of the bulbar conjunctival vessels appeared as worm-like conjunctival lymph vessels filled with blood (arrows).

Full size image
Figure 2
figure 2

Varicose tortuous and transparent lymphatic vessels at the site of previously blood-filled vessels showing classic ‘string of pearls’ appearance (arrows).

Full size image

Comment

There are connections between the deep conjunctival venous plexus and the collector channels. Occasionally, retrograde flow results in the lymphatic channel filling with blood. Approximately 4–8 mm behind the limbus, large collector channels run circumferentially receiving lymph from the radial lymphatic vessels. LHC might be an isolated finding2 or may be a rise in the periocular or orbital pressure, resulting in venous engorgement and a backflow of blood from the venous into the conjunctival lymphatic system by overcoming the valve mechanism.3 The clinical picture of this condition consists of segmental constrictions and balloon-shaped dilations of blood-filled lymphatic vessels in the conjunctiva. Although LHC usually resolves spontaneously, it may have tendency for recurrence. The blood resolved spontaneously in our patient and did not require any further intervention. Our case is exceptional because of the anatomic location and the full haemorrhagic component of the dilated vessels. Previous reports of LHC consist in localised haemorrhagic dilated vessels generally in the temporal conjunctiva.2, 3 To our knowledge, this is the first reported case of LHC involving 360° of bulbar conjunctiva. The absence of associated pathology, which might raise the periorbital or orbital pressure, suggests an idiopathic form.