Etiology

• Congenital unencapsulated hamartomas
• Combined venous and lymphatic malformation
• No known genetic association
• An "abortive vascular system which arborizes among normal structures" (Harris, Ophthalmology 1990).
• Hemorrhage expands portions of the small-caliber network into large blood cysts, prompting clinical recognition.
• Deep orbital lymphangiomas present as spontaneous, acute proptosis due to retrobulbar hemorrhage (Rootman, Ophthalmology 1986).

Epidemiology

• No sex predilection
• Not hereditary
• Relatively rare, but second-most common vascular orbital tumor in childhood

History

• Presents in infancy, later in childhood, or occasionally in adulthood
• Often acute onset with pain and swelling associated with upper respiratory infection

Clinical features

• Proptosis
• Result of hyperplasia of lymphoid tissue, generally due to an upper respiratory infection
• Result of intralesional bleed; "chocolate cyst"
• Small lymphangiomas can be limited to the eyelid.
• Eyelid lymphangiomas present with bleeding, as with orbital lesions (Pang, Ophthalmology 1984).
• Retinal folds can be the presenting finding of an orbital lymphangioma (Reeves, Arch Ophthalmol 2005).
• Deep orbital lymphangioma might not be recognized until late in life; three patients were described in whom the first manifestation was in the eighth and ninth decades of life (Bailey, OPRS 2007).

Testing

• Biopsy
• Incisional biopsy to minimally debulk and establish diagnosis if imaging is uncharacteristic of lymphangioma
• Histopathology
• Resembles lymphangiomas elsewhere in body
• Typical lymphatic architecture with large serum filled spaces with interrupted basement membranes, connective tissue stroma, random smooth muscle bundles, flattened endothelium, and absence of pericytes
• Associated lymphoid tissue can protrude into potential lumens.
• Acute hemorrhage can lead to cysts and/or features of old hemorrhage.
• Computed tomography (CT)
• Infiltrating or multicystic mass with variable contrast enhancement
• Magnetic resonance imaging (MRI)
• Particularly sensitive to identifying cysts within the tumor.
• Infiltrative lesion with variable enhancement
• Pathognomonic layering of blood often seen in recent chocolate cysts on T2‑weighted imaging
• Useful in differentiating between subacute and chronic hemorrhage
• Might detect feeding vessels (Bond, Ophthalmology 1992)
• Ultrasound
• Smooth contour and void areas
• Color Doppler shows no intralesional flow (Neudorfer, Am J Ophthalmol 2004)
• Cystic spaces

Lymphangioma is a congenital anomaly with no known inheritance pattern.

• Capillary hemangiomas
• Varix
• Rhabdomyosarcoma
• Neuroblastoma
• Ewing sarcoma
• Leukemia
• Histiocytosis X
• Optic nerve glioma

Natural history

• Conservative management is the preferred treatment in most cases.
• Cold compresses can be used for proptosis as the hemorrhage resolves spontaneously
• Lubrication for corneal exposure can be a temporizing measure for acute proptosis from an orbital bleed.
• Among 30 cases of orbital lymphangioma reviewed in 1990, poor final visual acuity was associated with multiple surgeries to remove blood clots (Harris, Ophthalmology 1990).
• Therefore, the authors advocated careful surgical selection for blood cysts that will not resolve spontaneously.
• Soon after the recognition of this clinical entity (see Historical Perspective below), others similarly recommended conservative management of recurrent hemorrhage without surgery (Wilson, Ophthalmology 1989).

Medical therapy

• Systemic corticosteroids might be useful for acute proptosis.
• Adults might have more improvement with pain than swelling, whereas children might enjoy improvement in both (Sires, OPRS 2001).
• In both adult and pediatric patients, steroids might be ineffective in reducing severe proptosis and might be unreliable as a treatment modality for acute, severe proptosis, compressive neuropathy, exposure keratopathy, and pain (Tawfik, OPRS 2005).
• Two cases of pediatric lymphangioma responded to Sildenafil (Viagra), one a 12‑month‑old and the other a 12‑year‑old (Arch Ophthalmol 2013; 131:1228).
• The response of pediatric lymphangioma to sildenafil was observed coincidentally in a child being treated with sildenafil for pulmonary hypertension who displayed improvement in chest and arm lymphangiomas (Swetman, New Engl J Med 2012).
• A phospho-diesterase inhibitor, sildenafil causes vasodilation by decreasing the contractility of vascular smooth muscle.
• Cystic dilation of lymphangiomas is thought to result from contraction of the muscular lining of the vascular channels; a proposed explanation for the response to sildenafil is smooth muscle relaxation leading to collapse of the cystic spaces.

Surgery

• Well-delineated extraconal lymphangiomas are most amenable to complete surgical resection (Gunduz, Ophthalmology 2006).
• Orbital decompression is an option for cases with severe proptosis and exposure keratopathy (Hsuan, OPRS 2004).
• The CO₂ laser can be helpful in limiting bleeding during partial surgical debulking.
• Interventional neuroradiology and vascular obliteration can also be helpful.
• For diffuse lesions the surgical goal is limited resection of the involved area
• Complete extirpation is generally impossible for a diffusely infiltrating tumor.

Other management considerations

• Sclerotherapy with sodium morrhuate 5% injected directly into the lymphangioma channel under direct visualization in the operating room just prior to excision can improve hemostasis, facilitate sclerosis of residual tumor, and allow for subtotal excision, which can reduce surgical morbidity (Kahana, Arch Ophthalmol 2011).
• Other sclerosing agents have been used (Wojno, OPRS 1999).
• Intralesional injection with Tisseel fibrin glue can similarly facilitate excision of a lymphangioma by limiting bleeding (Boulos, OPRS 2005).
• Another alternative is injection with intralesional bleomycin, which will shrink the tumor and can be repeated every 4–8 weeks if needed, until maximal regression is achieved (Gooding, OPRS 2014).
• Contraindication for sclerotherapy is lymphangioma that connects with the normal vasculature via venous or arteriovenous malformations because it risks damage to normal vessels. Clarification regarding this issue can be obtained with routine imaging and/or through a multidisciplinary approach with neuroradiology or neurosurgery.

Common treatment responses, follow-up strategies

Management of unresectable tumors can be frustrating with poor visual outcomes.

• Complications
• Orbital hemorrhage is a relatively high risk during and after surgery for these tumors.
• Damage to orbital structures and the optic nerve can occur with deep orbital dissection.
• Recurrence with potential to worsen long-term course of disease is a concern when multiple surgeries are needed.
• Prevention
• Biopsy can be avoided in many cases, minimizing the risk of bleeding.
• Avoid aggressive surgery whenever possible.

• When relatively large in early childhood, pose a significant risk of amblyopia
• As noted above, in 25 orbital lymphangiomas treated at the University of British Columbia, 28% had noncontiguous intracranial vascular anomalies with risk of intracranial hemorrhage (Katz, Ophthalmology 1998).
• Lymphangiomas not associated with intracranial vascular anomalies were more likely anterior, and less diffuse.
• Orbital lymphangiomas can also have contiguous intracranial extension (Zucker, OPRS 1995).
• Chronic periocular pain is a common long-term complaint.
• Strabismus
• Ptosis
• Optic nerve compression
• Exposure keratopathy

Iliff and Green described 19 cases of orbital lymphangioma in 1979, early in the recognition of this clinical entity (Ophthalmology 1979).

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