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OCT can be useful in diagnosis of conjunctival lymphangiectasis
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Lymphangiomas are classified as benign, uncommon, hamartomatous, lymphatic or venolymphatic vascular lesions with characteristic lymphatic vessel dilation that can involve the orbit, eyelid or conjunctiva. They represent 1% to 3% of all orbital masses.
When conjunctival lymphangiectasia involves the orbit, it can present with proptosis, ptosis and extraocular motility disturbances; however, isolated conjunctival lymphangiectasia can appear as a localized visible mass in the form of either a conjunctival cystic lesion or as dilated lymphatic vessels giving the appearance of a string of pearls or a bag of worms.
The etiology of conjunctival lymphangiectasia is not fully understood. However, it may be considered a secondary phenomenon resulting from local lymphatic scarring or distal obstruction. It is usually unilateral in occurrence, unless associated with Turner syndrome or Nonne-Milroy-Meige syndrome. A common site for appearance is the bulbar conjunctiva, where it may occur as either solitary or multifocal conjunctival lesions. This is often representative of a deeper, diffuse orbital lymphangioma.
Exacerbation of lymphangiectasia can be due to intralesional hemorrhage secondary to a connection between the lymphatic channel and a vein, resulting in subconjunctival hemorrhage or a large “chocolate cyst,” or due to episodes of upper airway infections that result in lymphoid hyperplasia.
Patients with lymphangiectasia are often initially asymptomatic and become symptomatic when dessication of the overlying conjunctiva occurs, which can lead to redness and irritation, or when the overlying conjunctiva occludes the lacrimal puncta, causing epiphora, pain and blurred vision. Conjunctival lymphangiectasia can be associated with focal or diffuse bulbar chemosis.
Diagnosis is aided by clinical evaluation and ultra-high resolution optical coherence tomography. It is then confirmed by histopathology of tissue specimen showing typical, dilated lymph vessels as a non-encapsulated, irregular mass lined by endothelial cells and made up of numerous cyst-like channels filled with fluid, blood or both. A conservative approach would encompass clinical observation, ocular lubricants and anti-inflammatory agents, but spontaneous resolution over time has been reported, as well. Various interventional treatment approaches have also been described, including the use of liquid nitrogen cryotherapy, carbon dioxide laser, beta radiation, high-frequency radio-wave electrosurgery, surgical excision with primary closure, amniotic membrane transplantation and conjunctival graft.
In this column, Vu, Karp, Galor and Wester describe their approach to the management of diffuse conjunctival lymphangiectasis.
Thomas “TJ” John, MD
OSN Surgical Maneuvers Editor
A 68-year-old patient presented with sudden onset of a subconjunctival hemorrhage in the left eye (Figure 1), with the area of hemorrhage expanding progressively over the course of 3 months. The patient had multiple episodes of spontaneous resolution and recurrence of hemorrhage.
B-scan showed no evidence of orbital mass or superior ophthalmic vein enlargement. MRI and venography with gadolinium showed mild subconjunctival edema and no orbital mass or carotid cavernous fistula. During a period of hemorrhage resolution (Figure 2), slit lamp examination confirmed subconjunctival edema suggestive of lymphangiectasis. An OCT confirmed the diagnosis of lymphangiectasis. The opposite, less-symptomatic eye had moderate hemorrhage and some inferior-temporal conjunctivochalasis. Surgical therapy was elected.
Figure 1. Subconjunctival hemorrhage with lymphangiectasis.
Figure 2. Slit lamp imaging of subconjunctival edema present with lymphangiectasis.
Images: Vu S, Karp CL, Galor A, Wester ST
Preoperative evaluation
Where there is a concern for a vascular etiology, a B-scan is often performed to rule out mass lesion or superior ophthalmic vein enlargement. OCT may be of great assistance when the clinical diagnosis is unclear.
For this condition, OCT imaging demonstrated clear septated fluid spaces in the conjunctiva (Figure 3). Other preoperative testing is as for standard ophthalmic surgery.
Figure 3. Optical coherence tomography imaging of conjunctiva demonstrating septated spaces.
Figure 4. Marks are made with a surgical pen to delineate margins of the lesion.
Operative technique
When excision and cryotherapy are planned, a retrobulbar block is given. Before an incision is made, a marking pen is used to delineate the margins of the lesion, which in this case were approximately 320° (Figure 4). In cases in which previous surgical scarring exists, such as scleral tunnel or filtering bleb, this area is avoided.
The conjunctiva around the previously marked area is excised using Wescott scissors, with careful attention so as to leave a 2-mm rim around the limbus to preserve limbal stem cells (Figure 5). Areas with no evidence of lymphangiectasis, such as the small area medially seen on Figure 6, are left on the ocular surface. During excision, care must be taken not to damage the extraocular muscles at their insertion onto the globe. In areas where the lymphangiectasis overlies the muscle, the muscle should be identified and isolated prior to excision of the lesion.
The lesion is placed onto marked filter paper to ensure proper orientation (Figure 7). Once passed off the field, it should be marked with pencil, because the ink dissolves with the formalin. After the lesion is removed, cautery is applied for hemostasis. The periphery of the remaining conjunctival tissue is then frozen using a cryotherapy probe in a double freeze-thaw technique. Cryotherapy should be performed around the entirety of the remaining conjunctival tissue bordering the lesion (Figure 8).
Figure 5. The lesion is excised with Wescott scissors while preserving a 2-mm rim around the limbus.
Figure 6. An area with no lymphangiectasis located medially is left attached.
Figure 7. The lesion is placed on filter paper with eye outline and landmarks to ensure proper orientation.
Figure 8. Cryotherapy is performed on the remaining conjunctival tissue bordering the lesion.
Figure 9. Amniotic membrane is peeled from nitrocellulose paper then placed on ocular surface, stromal side down.
Figure 10. Operated eye shown 19 months postoperatively.
Amniotic membrane (in this case, AmnioGraft, Bio-Tissue) is peeled from nitrocellulose paper and then placed with the stromal side touching the ocular surface (Figure 9). Adhesive fibrin glue (in this case, Evicel, Ethicon 360) is then applied with a cannula, dispensing first the thrombin and then the fibrinogen component. The glue components may also be applied together if desired. The amniotic membrane is then smoothed onto the exposed sclera with a muscle hook. To ensure proper adherence of a large graft, additional fibrin glue may be added as needed to the membrane.
The patient’s own remaining overlying conjunctiva is glued over the amniotic membrane. If needed, an 8-0 Vicryl suture may be placed. Topical corticosteroid and antibiotic ointment are placed on the eye, and a pressure patch is placed for a 24-hour period following surgery.
Postoperatively, topical steroids are applied every 2 hours for the first week, followed by four times per day for 1 week, and then a 4-week taper. Topical antibiotic is used twice a day for 1 week.
Outcome
The patient was followed up at 7, 15, and 19 months with no recurrence of lymphangiectasis (Figure 10).
In summary, conjunctival lymphangiectasis is a benign condition that is usually managed conservatively. Histopathologic examination of the tissue is often used as the definitive diagnostic technique, revealing dilated lymphatics and positive identification of lymphatic marker D2-40, but OCT can be very helpful in making the diagnosis preoperatively.
In severe cases, lymphangiectasis can cause the patient significant ocular surface irritation and/or cosmetic distress, especially when hemorrhagic in nature. In these situations, excision with cryotherapy and amniotic membrane ocular surface reconstruction may be effective.