Lymphangiomas are benign, hamartomatous, vascular tumours that may affect the orbits, eyelids, or conjunctiva [6
]. They represent 1–3% of orbital masses [7
]. Coexisting lymphangiomas of the face, nasal sinuses, nasal cavity, or palate may suggest the diagnosis [7
Isolated conjunctival lymphangioma is a rare condition with an average age of presentation at 25 years old (range from birth to 65 years) [9
]. Rootman et al. [8
] presented only a single case of a lymphangioma isolated to the lower fornix, in a series of 13 cases of orbital-adnexal lymphangiomas.
Orbital lymphangiomas often present with proptosis, ptosis, and impaired extraocular movements. Conjunctival lymphangiomas generally appear as a visible mass without affecting vision, or the globe [10
], as occurred with our patient.
Lymphangiectasia can lead to irritation and redness from desiccation of the overlying conjunctival epithelium, epiphora, if the lacrimal puncta become functionally occluded by overhanging conjunctiva, blurred vision, and pain [11
As described by Duke-Elder [12
], conjunctival lymphangiectasia has two principal manifestations: a cystic lesion of the conjunctiva, which may mimic allergic chemosis, and a beaded dilation of lymphatic vessels with a string of pearl appearance. Unless the latter type acquires communication with a conjunctival vein and becomes hemorrhagic, simple dilations of the conjunctival lymphatic vessels are asymptomatic and usually unnoticed.
In a review of 13 cases of orbital-adnexal lymphangiomas, Rootman et al. [8
] classified these lesions into superficial (as in our case), deep, and combined. Superficial lesions consisted of isolated multicystic vascular abnormalities of cosmetic significance only. The deep and combined lesions were found to be more symptomatic.
Biopsy with a histopathological report has an essential role in the diagnosis. Histopathologically, lymphangioma is a nonencapsulated, irregular mass, lined by endothelial cells, composed of numerous cyst-like channels that contain clear fluid, blood, or a combination of both. Separating the channels is loose connective tissue that contains aggregates of small lymphocytes [9
]. In our case the biopsy was the diagnostic key.
Treatment of superficial lymphangioma by surgical excision is often curative [11
] as occurred with our patient. The larger classical lesions tend to recur following surgery, as the extent of subcutaneous involvement may be difficult to define, making complete excision impossible [15
Alternative treatments for conjunctival lymphangiectasia have been described. In a case report by Jordan and coworkers [4
], a carbon dioxide laser was used to successfully treat what was described as a conjunctival lymphangioma. Behrendt and colleagues [16
] used beta irradiation to effectively treat a case of conjunctival lymphangioma. A case report from Egypt, by Wasfy [17
], describes the use of cryotherapy to successfully treat a case of conjunctival lymphangiectasia. Liquid nitrogen cryotherapy has been proven safe and effective for conjunctival lymphangiectasia [18
]. Conservative, nonsurgical management with eventual spontaneous resolution has also been reported [19
We did not find any correlation between Alport's syndrome and conjunctival lymphangioma in the literature [20
This paper presents a rare case of a lymphangioma isolated to the bulbar conjunctiva that was a differential diagnosis of red eye resistant to therapy. The upper respiratory infection may have exacerbated the signs of the conjunctival lesion. These lesions are a diagnostic challenge, and the biopsy has an essential role in the accurate diagnosis. Surgical excision is one of the treatment options, and it was effective in our patient. It ishowever necessary to be aware of possible recurrence of the lesion.