The Ophthalmology Department,  Universiti Malaysia Sarawak (UNIMAS), Kuching, Sarawak.

The Ophthalmology Department, Sarawak General Hospital, Kuching, Sarawak, East Malaysia.

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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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