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Case Report/Case Series |

Sildenafil for Pediatric Orbital Lymphangioma

Nandini G. Gandhi, MD1; Lily Koo Lin, MD1; Mary O’Hara, MD1
[+] Author Affiliations
1Department of Ophthalmology, University of California–Davis, Sacramento
JAMA Ophthalmol. 2013;131(9):1228-1230. doi:10.1001/jamaophthalmol.2013.4201.
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Importance  Orbital lymphangiomas, congenital hamartomas of the lymphovascular tissue, are often associated with significant ocular complications and can be challenging to treat. To date, therapeutic approaches have demonstrated variable results and have significant ocular and systemic risks. We present 2 cases of pediatric orbital lymphangioma that responded to treatment with oral sildenafil.

Observations  This is a report of a series of 2 patients at the University of California–Davis Medical Center treated between March 2011 and October 2012. The first patient was a 12-month-old male infant whose extensive orbital and facial lymphangioma responded to sildenafil after repeated sclerosing and drainage procedures failed to achieve remission. The second patient was a 12-year-old boy whose orbital lymphangioma and associated ocular pain improved with sildenafil, making enucleation unnecessary.

Conclusions and Relevance  These reported cases demonstrate promise for sildenafil as a noninvasive therapy for pediatric lymphangioma. Larger clinical trials are needed to clarify the optimal length of treatment, use as monotherapy, and long-term adverse effects.

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Figure 1.
Coronal Image

Magnetic resonance image demonstrates a lymphangioma originating in the pterygopalatine fossa, extending into the right orbit, and exerting mass effect on the right optic nerve and inferior globe. R indicates right.

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Figure 2.
Photograph of Case 1

Massive periorbital swelling and exposure during an acute bleed of the lymphangioma.

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Figure 3.
Follow-up Photograph of Case 1

Improved periorbital swelling and chemosis after treatment with sildenafil. The patient was able to initiate amblyopia therapy shortly thereafter.

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