Orbital Lymphangioma is an intra-orbital, non-encapsulated, congenital vascular tumour with a propensity for recurrent haemorrhage. Lymphangioma is a benign vascular hamartoma commonly noticed in head and neck area. Vascular mesenchyme that has differentiated abnormaly into isolated lymphatic- like spaces with no connection to the systemic circulation,may be the cause. Lymphangioma is a common vascular tumour in children and commonly presents in age group less than ten years. However, it is, sometimes detected later in life following trauma and intralesional haemorrhage. There are two growth patterns of lymphangioma (slow or rapidly explosive). Slow enlargement takes place over decades, producing progressive proptosis. In contrast, explosive growth results from intralesional haemorrhage arising from intrinsic nutrient vessels, or in association with an upper respiratory tract infection with proliferation of lymphoid tissue. In contrast to capillary haemangioma, lymphangiomas do not regress spontaneously. These tumours may become encapsulated due to normal bodily reaction to prevent expansion.
Lymphangiomas do not show any connection to the venous system, as has been studied by arteriography or venography. This is in contrast to orbital varix, which usually show connections to the venous system.
Like haemangiomas, Lymphangiomas may present in both superficial and deep orbital spaces. It consists of dilated, thin walled vascular chambers lined with endothelium. Fibrous stroma supports these and contain proteinaceous material reminiscent of lymph. There are micro-cystic, macro-cystic, or mixed types of Lymphangiomas.
References
Singh Arun D, Hayden Brandy C. Ophthalmic Ultrasonography. Elsevier Saunders 2012. P 171.
https://www.aao.org/eyenet/article/novel-approaches-to-orbital-lymphangioma
https://bjo.bmj.com/content/83/1/76
http://iv.iiarjournals.org/content/31/2/263.full
http://www.djo.org.in/articles/23/3/sclerosing-agents-in-ophthalmology.html
Patient may present with symptoms such as
Lymphangioma is likely to be derived from vascular mesenchyme that has differentiated abnormaly into isolated lymphatic- like spaces with no connection to the systemic circulation. They are benign hamartomatous vascular tumours, which differ from haemangiomas in that they do not display proliferating endothelial cells.
Lymphangiomas are usually located in the head and neck region and may involve eyelid, conjunctiva, and orbit.
Some reports suggest a slight female preponderance.
Clinically, the tumours may be located superficially in the orbit and are cystic. More commonly, these are infiltrative and diffuse in the orbit. Lymphangioma, located apically or with intracranial extension, is more likely to be associated with sub-mucosal oral lesions and intracranial vascular anomalies.
Histopathology:
Lymphangioma is a histologically benign but clinically aggressive tumour. Histopathologically, there are non-encapsulated, thin walled, endothelium lined channels and cystic vascular spaces which may contain fluid resembling lymph. Scattered lymphoid tissue aggregates or haemorrhage may be present.
Imaging studies:
Differential diagnosis:
Differential diagnosis of lymphangioma includes
Management includes
Supportive therapy
Since management of lymphangioma is difficult, it is advisable to observe it initially. Await for spontaneous resolution of haemorrhagic cyst or lymphoid hyperplasia associated with upper respiratory tract infection. Lymphangioma leading to compressive optic neuropathy, should be treated.
Medical therapy
Oral corticosteroids may help in the acute phase to resolve the lymphoid infiltration.
Surgical therapy
Surgery is, generally avoided since manipulation of the tumour may promote further spontaneous haemorrhage. Moreover, complete surgical excision is difficult due to infiltrative nature of the tumour and risk of damage to surrounding structures. Recurrence rate is high.
Indications for surgery are
Surgical procedures are
Prognosis:
The majority of patients present in the first decade of life. It shows either slow or explosive growth patterns. Slow growth occurs over decades. Explosive growth results from intralesional haemorrhage.
Lymphangioma may present in childhood, when it may adversely affect vision. In most cases, it cannot be safely removed and may result in lifelong series of haemorrhages that threaten vision, produce pain, and may result in disfigurement.
Sequlae includes
Complications may be
