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Case 40 Answers
by
Professor Chua Chung Nen
A 9 year-old boy presented with a 5-year
history of progressive painless closure of the left upper eyelid (Figure 1).
The vision was 6/6 in both eyes. There was a bluish discoloration over the
medial aspect of the left upper eyelid. The eyelid was soft too touch and
showed no transillumination. The left globe showed non-axial proptosis in a
down-and-out position which was not reducible with palpation and did not
change in size with position or Valsalva's maneuver. The left eye also showed limited adduction and upgaze
(Figure 2).
Figure 1. Left complete ptosis with bluish
discoloration of the left medial upper eyelid.
Figure 2. Restricted left upgaze and
adduction.
CT scan showed a large hyperdense soft
tissue mass on the anteromedial aspect of the left orbit that extended
anteriorly into the upper eyelid A blood fluid fluid level was present in
one of the axial scans (Figure 3). The lesion involves both the extra and
intraconal space and measures about 2.2 X 4.5 cm in axial dimensions. It
partially engulfed the left medial and superior recti (Figure 4 and 5). A
MRI scan was requested but not done because the patient was claustrophobic.
Figure 3. A blood fluid level is evident in
this axial CT scan.
a. What is
the differential diagnosis of this lesion? What is the most likely diagnosis?
The presence of blood-fluid level within
the lesion suggests this is a vascular tumour. Major reviews show that
vascular lesions account for 6.2 to 12.0% of all histopathologically
documented orbital space-occupying lesions1-5. The most
common vascular lesions of the orbit are:
-
cavernous haemangioma
-
capillary haemangioma
-
lymphangioma
-
orbital varix
Taking into account the history, age of
the patient, location and CT appearance of the tumour. The most likely
diagnosis in this case is a lymphangioma (this is subsequently confirmed
on histopathology).
-
Cavernous haemangioma typically
present in patient over the age of 30 and the lesion tends to be
intraconal. Blood fluid level is uncommon.
-
Capillary haemangioma usually becomes
appear ant at birth or within the first 8 weeks of birth. The lesion
typically gives a red cutaneous appearance.
-
Orbital varix shows positional
proptosis and typically involves the superior ophthalmic vein.
Lymphangioma most commonly presents in
patient under the age of 10. On CT or MRI scan the lesion is usually
extraconal but may show intraconal extension. The lesion has ill-defined
edges but with time partial encapsulation may occur due to the attempt
of the body to form barrier to limit its growth. Bleeding into cystic
spaces gives a blood-fluid level.
Clinically, lymphangioma causes a slowly
progressive proptosis with globe displacement, ptosis, and restrictive
eye movement. The eyelid may have a bluish discoloration from
subcutaneous involvement. Sudden worsening of the proptosis may be
caused by bleeding into the cystic spaces. Enlargement of the lesion
following upper respiratory tract infection is a characteristic feature;
this is caused by lymphoid reaction in response to infection.
Macroscopically, the lesion usually appears dark red with cystic spaces
which may contain dark blood. Histologically, the lesion is made
up of bloodless ectatic lymph channels and contain spaces lined by a
single layer of flat mesothelial cells.
b. Which
surgical technique is best employed to excise the tumour?
In this patient, the
tumour extended beyond the equator of the globe and therefore the standard anterior orbitotomy approach through an upper lid crease would be very difficult
or impossible to access the whole lesion. The best approach is a vertical lid split
approach which allows one to reach the deeper region of the extraconal and intraconal
spaces of the medial and superior orbit.
Figure 6. a. A vertical incision line was drawn on the upper
eyelid at the junction of the medial 1/3 and lateral 2/3 of the eyelid.
A scalpel blade was used to incise the margin. b. a Stevens scissors
was used to extend the wound full thickness through the eyelid to expose
the lesion. c. and d. The cut
extended above the tarsus, dividing the levator aponeurosis, Muller's muscle
and the conjunctiva. e. and f. Because the lesion was large, a needle was
inserted into the lesion to withdraw blood in order to reduce its size.
About 15ml of dark blood was aspirated. g. and h. To avoid unnecessarily
enlargement of the incision and dissection, the anterior portion of the
tissue was clamped with a pair of artery forceps and excised to allow
posterior orbital dissection.
Figure
7. a. The excised anterior portion of the lesion showing cystic spaces. b. A
large cystic space was visible when the artery clamped was released. c.
Posterior dissection was now made easy with the removal of the anterior part
of the lesion. d. The remaining lesion was excised. e, f, g and h. The split
upper eyelid was closed in layers with 6/0 vicryl. A drainage tube was
inserted before bandage to prevent intraorbital haematoma.
Figure 8. Improved eyelid opening 10 days
post-operative (Right) compared
with pre-operative (Left). The medial
ptosis was due to the presence of haematoma.
Figure 9. Improved adduction and upgaze 10
days post-operative.
Facial appearance at three months
post-operative.
References
1. Shields JA, Bakewell B, Augsburger JJ, Flanagan JC. Classification and
incidence of space-occupying lesions of the orbit. Arch Ophthalmol
1984;102:1606–1611.
2. Henderson JW, Campbell RJ, Farrow GM,
Garrity JA. Orbital Tumors. New York: Raven Press; 1994:43–52.
3. Günalp I, Gündüz K. Biopsy-proven orbital
lesions in Turkey. A survey of 1092 cases over 30 years. Orbit
1994;13:67–79.
4. Seregard S, Sahlin S. Panorama of orbital
space-occupying lesions. The 24-year experience of a referral center. Acta
Ophthalmol Scand 1999;77:91–98.
5. Sen DK. Aetiological pattern of orbital
tumors in India and their clinical presentations. A 20-year retrospective
study. Orbit 1990;9:299–302.
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