Orbital Lymphangioma: Characteristics and Treatment Outcomes of 12 Cases

Abstract

PurposeTo report the patient characteristics and treatment outcomes in 12 cases of orbital lymphangioma.MethodsIn this study, orbital lymphangioma was diagnosed based on clinical, radiologic (computed tomography, magnetic resonance imaging), and histologic findings when possible. Patients whose vision was not compromised by orbital lymphangioma, or that did not have increased intraocular pressure (IOP), received oral corticosteroids. Orbital lymphangioma that affected vision or increased IOP was treated by surgery, which included aspiration of blood or partial resection with or without injection of a sclerosant.ResultsFour patients without compromised vision responded well to oral corticosteroids. Eight patients with compromised vision underwent some form of surgery. Bleeding recurred in three patients after aspiration of blood and in two after partial resection and intralesional injection of a sclerosant. Overall, five patients were treated successfully by aspiration of blood, intralesional injection of a sclerosant, and application of continuous negative pressure by appropriate drainage. Partial resection was successful in two patients with organized hematoma.ConclusionsOrbital lymphangioma that does not compromise vision can be treated medically using oral corticosteroids. Patients with threatened vision or elevated IOP due to acute hemorrhage should be treated by aspiration of blood, intralesional injection of a sclerosant, and application of continuous negative pressure. Partial resection may be effective only in patients with organized hematoma.

Lymphangioma Presenting as Sudden Proptosis in a Toddler

“…in their study, 85% of patients had proptosis; 73% had ptosis; and 46% had reduced ocular motility. Woo et al [4] reported that two out of 12 patients were aged 1 year; and all patients had proptosis and limited ocular motility at presentation. Our patient was a 14 months old boy who was brought to the eye clinic with acute proptosis of two days duration.…”

“…It is congenital and slowgrowing, consisting 0.3 to 1.5% of all orbital tumours [1]. It can present with spontaneous , orbital cellulitis [3] and proptosis [4]. In some of these patients proptosis may develop either slowly as the mass invades the orbit or suddenly during hemorrhage In childhood, the diagnosis is often made when proptosis occurs after bleeding as a result of minor trauma or upper respiratory infection, and may even occur spontaneously.…”

“…It is congenital and slow growing, consisting 0.3 to 1.5% of all orbital tumours [1]. It can present with spontaneous orbital haemorrhage [1], ptosis [2], orbital cellulitis [3] and proptosis [4]. In some of these patients proptosis may develop either slowly as the mass invades the orbit or suddenly during hemorrhage in the lesion [5].…”

Pediatric orbital tumors - An overview

“…Sometimes, massive hemorrhage may occur within the lymphangioma that may require urgent surgical intervention to decompress the orbit. [21] Indications for such an urgent surgical drainage include optic nerve compromise (suggested by appearance of relative afferent pupillary defect and/or deterioration of vision) or corneal exposure due to lagophthalmos. The acute presentation in such cases may often confuse the treating clinician who may misdiagnose it as a malignancy.…”

Orbital Lymphatic-Venous Malformation Accompanied by an Intraocular Vascular Malformation: A Rare Case Study

“…However, orbital LVMs can extend to the frontotemporal region and the cheek and may be associated with intracranial vascular malformations, such as venous anomalies [ 8 , 9 , 10 ]. Lesions can be asymptomatic until they reach a large size, or intralesional hemorrhage occurs and causes swelling, proptosis, decreased motility of the eye, or pain [ 11 ]. Even though they are benign, these lesions can threaten vision by causing a compressive optic neuropathy [ 7 ].…”