Journal of Clinical Ophthalmology and Research

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Year
: 2017  |  Volume : 5  |  Issue : 3  |  Page : 142--144

Conjunctival lymphangioma: A unique case report and review of literature


Anuradha Raj1, Ramesh Chander Nagpal1, Meena Harsh2, Harsh Bahadur1,  
1 Department of Ophthalmology, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Dehradun, Uttarakhand, India
2 Department of Pathology, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Dehradun, Uttarakhand, India

Correspondence Address:
Anuradha Raj
Department of Ophthalmology, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Swami Ram Nagar, Jolly Grant, Dehradun, Uttarakhand
India

Abstract

Lymphangioma is a rare venolymphatic lesion or vascular hamartoma of lymphatic origin characterized by dilation of lymphatic vessels. It may present as an isolated lesion or often represent the surface component of a deep orbital lymphangioma. Conjunctival lymphangioma is a rare condition, in which patients can present with a red eye resistant to topical treatment. Recurrence of lesion can also be the concern of this entity. We report a case of conjunctival lymphangioma in a 60-year-old Indian female as no case has been reported from Indian origin till date to the best of our knowledge.



How to cite this article:
Raj A, Nagpal RC, Harsh M, Bahadur H. Conjunctival lymphangioma: A unique case report and review of literature.J Clin Ophthalmol Res 2017;5:142-144


How to cite this URL:
Raj A, Nagpal RC, Harsh M, Bahadur H. Conjunctival lymphangioma: A unique case report and review of literature. J Clin Ophthalmol Res [serial online] 2017 [cited 2022 Aug 17 ];5:142-144
Available from: https://www.jcor.in/text.asp?2017/5/3/142/216431


Full Text



Lymphangioma represents a spectrum of vascular hamartoma of lymphatic origin due to hemodynamic changes.[1] It is characterized by dilated lymphatic vessels containing lymph which appears as a multiloculated cystic lesion.

The etiology of lymphangioma of the conjunctiva is not yet clear. It usually occurs as an unilateral sporadic case but can be bilateral entity if associated with Nonne–Milroy–Meige disease or Turner syndrome.[2] It can be seen as a solitary or a multifocal conjunctival lesion in the bulbar conjunctiva. It represents the superficial component of a deeper diffuse orbital lymphangioma in several cases.[3]

The hemorrhage within lymphangioma can result in large “chocolate cysts” or lymphoid hyperplasia due to repeated episodes of upper airway infections.[4] Correlation between conjunctival lymphangioma and Alport's syndrome has been reported in literature.[5]

This paper describes a clinical case of a patient with an isolated conjunctival lymphangioma which is a quite rare entity.

 Case Report



A 60-year-old female presented with a history of a swelling on the lateral aspect of the right eye for the past 6 months. She complained of irritation, watering, and foreign body sensation in the same eye. The swelling started as wheat grain in size and increased slowly, progressively, and painlessly in the past 6 months. She came to us when she felt the rapid increase in size of the lesion. She was not on any treatment before she came to us. There was no history of any ocular trauma or previous ocular surgery. The swelling was pinkish, nonpulsatile, and compressible in nature. The swelling was soft in consistency, and the posterior limit was well defined. There was no associated pigmentation. The swelling was located on the inferotemporal aspect of the bulbar conjunctiva of the right eye with well-defined borders associated with mild chemosis and hyperemia [Figure 1].{Figure 1}

On examination, the best corrected visual acuity was 20/20 and N/6 with − 0.75 diopter (D) Sphere and addition of + 2.75 D Sphere for both eyes, respectively. On slit-lamp biomicroscopy of the right eye, anterior segment was unremarkable. Intraocular pressure was 16 mmHg for both eyes with Goldmann applanation tonometry. Pupillary reflexes were normal. The patient had no ptosis or proptosis. Extraocular movements were full, free, and painless for both eyes. Fundus examination of both eyes was within normal limits. Systemic history was unremarkable. Computed tomography scan of the orbit was done to rule out the intraorbital extension of the mass, but that was unremarkable. On the basis of clinical picture, a diagnosis of conjunctival cyst was made.

The mass was excised and sent for histopathological examination. Postoperatively, the patient was put on topical drugs such as moxifloxacin 0.5% 6 times daily, prednisolone acetate 1% 4 times daily, and carboxy-methyl cellulose 0.5% 8 times daily. The lesion healed without any complications 2 weeks postoperatively [Figure 2].{Figure 2}

On gross examination, there was grayish-brown mass measuring 10 mm × 5 mm × 2 mm. On microscopic examination, section showed cystic lesion. The wall showed stratified squamous epithelium with irregular acanthosis. Subepithelial tissue showed dilated lymphatic channels of various shapes and sizes lined by flattened endothelium. A very scanty intervening stroma was seen which shows mild nonspecific chronic inflammation, suggestive of conjunctival lymphangioma [Figure 3]a and [Figure 3]b. Six months later, there was no recurrence.{Figure 3}

 Discussion



Lymphangiomas are benign vascular hamartomatous tumors that may affect the orbits, eyelids, or conjunctiva. Conjunctival lymphangiomas may present as solitary or multifocal lesions. They tend to enlarge slowly and progressively over years till their growth become static. The present case also showed slow progression over a period of 6 months. Spontaneous or traumatic hemorrhage within the lesion may cause sudden enlargement.[6] Lymphangioma constitutes 1%–3% of orbital masses. Coexisting lymphangiomas of the face, nasal sinuses, nasal cavity, or palate are suggestive of the diagnosis.[7]

Isolated conjunctival lymphangioma is a rare condition with an average age of presentation at 25 years of age with range from birth to 65 years,[8] which holds true for the present case also as she was 60 years old. Rootman et al.[9] reported only a single case of lymphangioma affecting the lower fornix, in 13 cases of orbital-adnexal lymphangiomas.

Proptosis, ptosis, and impaired extraocular movements are the common presentations of orbital lymphangiomas. Conjunctival lymphangiomas generally appear as a visible mass lesion without any visual or globe involvement.[10] The present case also presented to us with the mass lesion of conjunctiva in the inferotemporal forniceal area without any involvement of the vision.

Lymphatic channels filled with blood called “lymphangiectasias” can be seen beneath the conjunctiva if lymphangioma involves ocular adnexa.

Lymphangiectasia can lead to irritation and redness due to desiccation of the overlying conjunctival epithelium and epiphora due to occlusion of lacrimal puncta by overhanging conjunctiva, blurred vision, and pain.[11] The chief complaints of the present case were irritation and watering.

Duke–Elder described two principal manifestations of conjunctival lymphangiectasia as a cystic lesion of the conjunctiva, which resembles allergic conjunctival chemosis and dilation of lymphatic vessels in a beaded pattern with a string of pearl appearance.[12] Dilations of the conjunctival lymphatic vessels are usually asymptomatic and unnoticed unless they get communication with a conjunctival vein and becomes hemorrhagic.

Rootman et al. reviewed 13 cases of orbital-adnexal lymphangiomas and classified these lesions into superficial (as in present case), deep, and combined. Superficial lesions were isolated multicystic vascular abnormalities which were of cosmetic significance only. The deep and combined lesions were more symptomatic.[9]

Excisional biopsy with a histopathological confirmation has a vital role in the diagnosis. Due to the dual nature of vascular and lymphatic origin, these lesions were named vascular hamartomas which had characteristics of relative hemodynamic isolation.[8]

Superficial lymphangioma can be treated effectively by surgical excision which was true for the present case also where the surgical excision was done and the raw area created was covered by conjunctival autograft from the superotemporal aspect of the eye.[11] Recurrence of these lesions after surgery is possible with larger classical lesions because of ill-defined boundaries of subcutaneous involvement where complete excision of the lesion becomes impossible.[13]

Various alternative modalities of treatments for conjunctival lymphangiectasia have been described in literature. Jordan et al. reported the use of carbon dioxide laser to treat conjunctival lymphangioma successfully.[3] Conservative and nonsurgical approach with eventual spontaneous resolution has also been reported.[14]

This paper presents a rare case of a lymphangioma involving inferotemporal bulbar and forniceal conjunctiva. Diagnosis was made only by the histopathological examination of the excisional biopsy. These lesions are of diagnostic challenge and the biopsy plays an essential role in its accurate diagnosis. Surgical excision is one of the treatment options which was effective in this patient also. It is, however, necessary to be aware of the possible recurrence of the lesion.

Acknowledgment

The authors would specially thank to Surendra Singh Bhandari, Office Assistant, for photographic documentation.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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