Korean J Ophthalmol.  2017 Jun;31(3):194-201. 10.3341/kjo.2016.0034.

Orbital Lymphangioma: Characteristics and Treatment Outcomes of 12 Cases

• Woo YJ
• Kim CY
• Sgrignoli
• Yoon JS

• KMID: 2379875
• DOI: http://doi.org/10.3341/kjo.2016.0034

Abstract

PURPOSE
To report the patient characteristics and treatment outcomes in 12 cases of orbital lymphangioma.
METHODS
In 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.
RESULTS
Four 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.
CONCLUSIONS
Orbital 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.

Keyword

Hemorrhage; Lymphangioma; Sclerotherapy; Treatment outcome

MeSH Terms

Adrenal Cortex Hormones
Drainage
Hematoma
Hemorrhage
Humans
Injections, Intralesional
Intraocular Pressure
Lymphangioma*
Orbit*
Sclerotherapy
Treatment Outcome
Adrenal Cortex Hormones

Figure

• 

  Fig. 1 Patients with orbital lymphangioma that were successfully treated with oral corticosteroids. (A) Case 6, (B) case 8 (left column, pretreatment; right column, posttreatment).

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  Fig. 2 Treatment modalities of patients with orbital lymphangioma who underwent surgical intervention and experienced recurrent hemorrhage.

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  Fig. 3 Four-year-old girl with left orbital lymphangioma. (A) Proptosis at initial visit. (B) Axial T2-weighted magnetic resonance image showing multiple diffusely-infiltrating cysts. (C) Coronal T2-weighted magnetic resonance image showing cysts with fluid-fluid levels. (D) Lymphangioma observed via the anterior orbitotomy site. (E) Partially-resected lymphangioma. (F) Axial T2-weighted magnetic resonance image showing that the cystic mass had been removed from the left orbit. (G) Bleeding recurred 30 months after the first surgical intervention. (H) Axial T2-weighted magnetic resonance image indicating bleeding from deep residual lymphangiomatous tissue. (I) Appearance 6 months after the repeated surgery; bleeding has not recurred, but the patient's visual acuity has changed to no light perception.

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  Fig. 4 Nineteen-year-old man with a 16-year history of left orbital lymphangioma. (A) Severe proptosis and conjunctival swelling before resection. (B) Exophthalmometry showing 35-mm protrusion of the left eyeball. (C) Coronal T2-weighted magnetic resonance image showing lymphangioma that occupied most of the left orbit. (D) Axial T2-weighted magnetic resonance image showing a large lymphangioma with organized hematoma anteriorly displacing the left eyeball. (E) Reduced proptosis after partial resection. (F) Computed tomography image after partial resection.

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