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.
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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.
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