Lymphangioma is a congenital, slowly progressive vascular hamartoma. It is usually diagnosed in the first decade of life.1 Periocular lymphangioma can involve the orbit, eyelids, conjunctiva, or mulripie locations. In some cases, it is associated with lymphangioma of the face, nasal cavity, palate, paranasal sinuses, or remote systemic sites.1"3
Lymphangioma is uncommon in the ocular region. In a review of 9,000 ocular specimens from an ophthalmic pathology laboratory, Jones reported that only 34 cases (0.37%) were lymphangioma of the ocular adnexa.4 The conjunctiva is a rare site for lymphangioma. We describe a child who presented with conjunctival lymphangioma manifesting as diffuse Subconjunctival hemorrhage.
A 4-year-old girl had Subconjunctival hemorrhage in the right eye on awakening. She denied trauma and bleeding abnormalities, but had a history of coughing secondary to upper respiratory infection 2 weeks earlier. She was born at term and had a capillary hemangioma on the right side of the scalp, which regressed spontaneously during the first year of life. She had repeated episodes of right epistaxis beginning at 6 months and recurring every 1 to 2 months from abnormally dilated blood vessels in the right nasal cavity. This was managed with nasal cautery at 9 months. Repeated epistaxis occurred every few months thereafter and was managed conservatively. There was no palate lymphangioma.
Ophthalmic examination showed visual acuity of 20/20 in both eyes. The anterior segment of die right eye showed diffuse, multilobulated cystic channels in the inferior bulbar conjunctiva, most of which were filled with blood (Figs. 1 and 2). The blood visibly flowed through the channels. On magnetic resonance imaging, the orbit and brain were normal. The remainder of findings on examination were normal. The patient was diagnosed as having diffuse conjunctival lymphangioma of the right eye, and observation was advised. Within 2 months, the blood in the lymphatic channels resolved, leaving transparent cystic channels (Figs. 3 and 4). The lymphatic channels did not regress after a 2-year follow-up period.
Lymphangioma is a rare tumor that can affect the periocular region. In a clinical review of 1,264 patients with orbital tumors, Shields et al. noted only 55 patients (4%) with lymphangioma.5
Similarly, in a clinical review of 1,643 patients with conjunctival tumors, Shields et al. found only 15 patients (1%) with conjunctival lymphangioma.6
Conjunctival lymphangioma manifests as multiple cystic channels in the conjunctival stroma. It can lead to subconjunctival bleeding that usually resolves spontaneously. Histopathologically, lymphangioma consists of dilated, ectatic, lymph-filled channels lined by endothelium and separated by thin, delicate walls containing small blood vessels and lymphoid cells.2'7 These vessels are easily disrupted and tend to hemorrhage spontaneously or after minor trauma. Episodes of rapid enlargement can result from either hyperplasia of lymphoid tissue, typically induced by upper respiratory infection, or intralesional bleeding.7
In 1880, Leber described the first case of diffuse conjunctival lymphangioma, and the condition disappeared spontaneously.8 Donders reported in his article that Heydenreich published the first photograph of conjunctival lymphangioma in 1956; the lesion also resolved spontaneously.9 Donders reported a case of an 18-year-old man with conjunctival lymphangioma presenting with subconjunctival hemorrhage involving the entire bulbar conjunctiva. Spontaneous resolution began within 6 weeks and was complete within 12 weeks.9 Similarly, in our case, the subconjunctival hemorrhage involved the entire bulbar conjunctiva at presentation, and the hemorrhage resolved spontaneously in 8 weeks. It is unclear what precipitated the acute event. Perhaps a recent viral illness or coughing caused it. On resolution of the blood, a dilated cystic channel remained on the inferior bulbar conjunctiva.
The differential diagnosis of conjunctiva! lymphangioma includes capillary hemangioma; varix; arteriovenous malformations; subco njunctival hemorrhage from trauma, aspirin use, or blood coagulation problems; acute hemorrhagic conjunctivitis; lymphoma; amelanotic malignant melanoma; and Kaposi sarcoma.1,2
Our patient had right epistaxis repeatedly, presumably from nasal cavity lymphangioma. The symptoms of nasal lymphangioma are nasal obstruction and epistaxis. The nasal obstruction is proportionate to the size of the tumor.10 The epistaxis was reported to be from an abnormal blood vessel in the nasal cavity and required cautery to manage the bleeding. On magnetic resonance imaging and magnetic resonance angiography of the orbit and head, no intranasal channels were visualized. Superiorly, on the bulbar conjunctiva, the subco njunctival hemorrhage resolved without residual recognizable lymphatic channels. Perhaps the subco njunctival hemorrhage diffused superiorly while the patient was sleeping.
The management of conjunctival lymphangioma is usually observation, unless drainage of blood or resection of tumor is necessary to protect vision. Our patient responded well to conservative observation. Surgical resection can be an option in patients who have conjunctival lymphangioma and cosmetic problems or frequent episodes of bleeding. However, complete resection may be difficult because conjunctival lymphangioma can diffusely infiltrate die conjunctiva.2,3,11 On the other hand, in well-circumscribed cases, the lesion can be removed completely.
Conjunctival lymphangioma must be considered in the differential diagnosis of spontaneous subco njunctival hemorrhage in a child.
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