Lymphangiectasia haemorrhagica conjunctivae

Lymphangiectasia JONATHAN LOCHHEAD, LARRY BENJAMIN haemorrhagica conjunctivae 2-4 days. The nature of this condition contrasts Abstract Purpose To re-describe a condition that has not been mentioned in the literature for more than four decades and to outline a new method of treatment of the pathology using an argon laser. Methods Nine patients were identified over a 2 year period who fitted a clinical picture consistent with the features of lymphangiectasia haemorrhagica. Three were treated for frequent recurrent haemorrhages with a newly developed technique using the argon laser and followed for up to 2 years. Results None of the three treated patients has had a recurrence of the condition to date. In the six untreated patients the condition resolved spontaneously after repeated attacks over between 8 months and 8 years. Conclusions Lymphangiectasia haemorrhagica conjunctivae is probably more common than expected and may resolve spontaneously, but is readily amenable to treatment with the with that of recurrent subconjunctival haemorrhage by resolving very rapidly and completely over this short period. Careful examination frequently reveals the broken column of blood with segmented constrictions and balloon-shaped dilatations characteristic of this condition (Figs. 1, 2). The distended ampoules of the lymphatic vessels involved may also demonstrate a horizontal level of blood where they are only partially filled. These features are quite distinct from the diffuse haemorrhage visible following a subconjunctival haemorrhage, which may take 10-14 days to clear. In the previous literature, treatment has been described by a surgical means, excising the pathological communication, 1 perhaps with the risk of subsequent haemorrhage, or by a process of diathermic coagulation? The treatment used here is similar to the latter but exploits the magnification and precision of an argon laser. argon laser if attacks become frequent, uncomfortable or unsightly. Treatment protocol Key words Argon laser, Lymphangiectasia Amethocaine 1 % drops were applied to the conjunctival sac. The laser was used in its Green mode at a relatively low power duration. Approximately in diameter were required on each occasion to Clinical features Lymphangiectasia haemorrhagica is a condition that appears to be little recognised. There has been no mention of it in the literature for more than four decades and the most recent textbook to describe the disorder was Duke Elder's Diseases of the Outer Eye published in (0.3 W) and 0.1 s 20-30 burns of 100 "",m obliterate the junction between the blood and lymph vessels. Uptake varied according to the amount of blood in the tissues. Very little, if any, discomfort was experienced by any of the three treated patients. 1965. The condition involves sudden, rapid filling of the conjunctival lymphatics with blood by Results (Table 1) J. Lochhead retrograde filling from the conjunctival vessels. At the time of presentation only two patients The hallmark of the condition is that the blood had visible signs. One of these was treated with clears almost as rapidly as it arrives and almost an argon laser and to date, within a always within period, has had no further trouble. Another QEII Wing 3-4 days. 12 month Department of Ophthalmology St Bartholomew'S Hospital patient who had suffered three attacks within a West Smithfield patients was of a foreign body sensation and 3 month period returned to the clinic during a London EC1A 7BE, UK recurrent 'bleeding on the front of the eye' with recurrence and was subsequently treated, again L. Benjamin � no obvious precipitating factors such as using an argon laser. She has remained surgery, trauma, clotting abnormalities, symptom free over the last A typical history in this series of nine coughing or straining. The 'whole of the white 2 years. A third treated patient returned to the clinic of the eye became covered in blood' in one with 'another episode' approximately patient and typically resolved completely in following treatment. Interestingly, on this Eye (1998) 12, 627-629 © 1998 Royal College of Ophthalmologists 3 weeks Stoke Mandeville Hospital Mandeville Road Aylesbury Buckinghamshire HP21 8AL, UK Fax: +44 (0)1296 315037 627 Fig. 1. A photograph of dilated lymph vessels filled with blood in the inferonasal conjunctiva. This was taken during the resolving phase of an attack. occasion the haemorrhage was entirely subconjunctival Fig. 2. A close-up of the conjunctiva seen in Fig. 1 showing the dilated lymph channels, which when not filled with blood would be transparent. Two days after this picture was taken the blood had virtually completely disappeared from the lymphatic system. in a region adjacent to the previous treatment. There was behind the limbus. These drain into the main lymph no visible haemorrhage into the lymphatics of the treated channels, which are two in number and pass from the area or elsewhere and therefore no further treatment was nasal to the temporal areas of the conjunctiva. The considered. superior channel passes around the upper half of the The remaining patients were asked to return for review immediately on recurrence of the condition. To date none have returned and it is interesting to speculate why this might be. cornea and the inferior one around the lower half. These two channels may then fuse to form one principal trunk or may remain separate as they pass to the external angles. There are two recurrent collector channels that are smaller in size than the main collectors: a superonasal recurrent collector draining the upper nasal quadrant Pathophysiology The conjunctivae contain two vascular systems - a superficial and a deep plexus - between which is a free anastomosis in the loose subconjunctival connective tissue. The superficial vessels contribute primarily to the and a temporal pericorneal descending collector with a recurrent limb. Both drain via the medial canthus. Laterally placed lymphatic channels proceed to drain into the pre-auricular lymph nodes, while those located perilimbal plexus and may only be visible in congested medially proceed to the submaxillary nodes. They states. The deep vessels originate from the nasal and appear as clear, dilated, tortuous, sausage-like lymphatic lacrimal branches to the eyelids. They are clearly visible vessels (not to be confused with conjunctival lymphatic in the anterior segment from their origins in the fornices. cysts) and are thought to result from a local disturbance The conjunctivae are the only components of the globe and orbit to have a lymphatic drainage system. They can be divided into several groups whose channels drain directly into the superficial venous plexus. An associated with haemorrhage around them (subconjunctival haemorrhage) and occasionally with haemorrhage into them. This is then termed incomplete pericorneal lymphatic ring (circulus lymphaticus) forms a rich network in lymphatic flow. Sometimes these lymphangiectasia are 1 mm wide of tiny lymphatic vessels surrounding the edge of the cornea. From here originate lymphatic channels that initially lymphangiectasia haemorrhagica and is thought to evolve from an abnormal connection established between a blood vessel (of the deep venous plexus) and the radiate and then become concentric with the corneal lymphatic vessel such that intermittent episodes of margin at about retrograde flow result in the lymphatic channel filling 4-5 mm from their origin. Large collector channels then emerge circumferentially 7-8 mm with blood. Table 1. Details of the nine patients in this series. Age (years) Sex Location Durationa Treatment Outcome 60 54 58 53 32 40 48 37 52 F Medial Nil Spontaneous resolution Lateral years Nil Spontaneous resolution M Lateral months Argon laser No recurrence M Lateral 18 8 6 2 12 12 8 5 10 months M years Nil Spontaneous resolution F Lateral M Medial M Lateral F Lateral M Medial months Argon laser No recurrence months Nil Spontaneous resolution months Nil Spontaneous resolution months Argon laser No recurrence months Nil Spontaneous resolution aRelates to the length of time since the first occurrence of bleeding. To date no patient has returned with a true recurrence. 628 Discussion Following the first description of lymphangiectasia haemorrhagica by Leber in 1880, there have been a number of very well documented cases such as that of Stepanik,3 where the lymphatics of a 12-year-old girl became suffused with blood each night only to clear up within hours the following day. Histological studies were performed in the pase,2 that rapid and this may be due to direct emptying of the blood into the connecting veins from whence it came, rather than having to be removed as in the case of a sub­ conjunctival haemorrhage. The patients who have not, presumably, had recurrences may have closed off the connection between lymph and blood vessels by fibrosis after repeated attacks. confirmed the nature of the condition but did not shed any light on the possible mechanisms involved. Conrads and Kuhnharde found signs of chronic inflammation Conclusion and went on to postulate that this could promote the Lymphangiectasia haemorrhagica may be more common formation of connective tissue strictures or septa within than expected and an awareness of its characteristics will the lymph spaces and thus contribute to the characteristic segmented appearance. No conjunctival lymph nodes have ever been identified.4 The pattern of the lymphatics also differs in relation to their depth within the conjunctiva. There are three zones: a superficial zone lying just below the epithelium consisting of very fine lymphatics and then a slightly deeper middle zone containing the typically larger vessels; there are no lymphatics to be found in the deepest of these zones. The superficial fine network is be useful in examining future recurrent haemorrhages of the bulbar conjunctiva. Those patients presenting with haemorrhage that clearly has a lymphatic component and who particularly complain of associated discomfort, or voice concerns about the cosmetic appearance accompanying recurrent haemorrhages, may be considered for treatment with the argon laser. The main diagnostic clue in the clinical history is the speed with which the blood clears from the conjunctiva, composed of vessels that are similar in size to the medium-sized veins of the area. They communicate with the deeper network which contains vessels some 2-4 times larger than the veins of this area. It is these vessels that are primarily identified in lymphangiectasia haemorrhagica. They are very irregular in diameter with References 1. Conrads H, Kuhnhardt C. Zur Pathogenese der Lymphangiectasia Haemorrhagica Conjunctivae. Klin Monatsbl Augenheilkd 1957;131:670-4. aneurysm-like outpouchings and valve-like constrictions 2. Leffertstra LJ. Lymphangiectasia hemorrhagica conjunctivae. Ophthalmologica 1962;143:133-6. that often give them a beaded appearance. In older 3. Stepanik J. Periodisch im Schlaf auftretende spontane individuals there is an increasing tendency for these valvules to narrow. Failure of these valvular mechanisms may be responsible for blood gaining access to the lymphatics and might explain the rapid filling of the lymph vessels with blood. Dispersal of the blood is also Blutfullung der Bindehautlymphraume als Begleitsymptom eines Lymphhamangioms der Parotis. Klin Monatsbl Augenheilkd 1958;132:99-103. 4. Sugar HS, RA, Schaffner R. The bulbar conjunctival lymphatics and their clinical significance. Trans Am Acad Ophthalmol 1957;61:212-23. 629