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.
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.
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.
Massive periorbital swelling and exposure during an acute bleed of the lymphangioma.
Improved periorbital swelling and chemosis after treatment with sildenafil. The patient was able to initiate amblyopia therapy shortly thereafter.
Thank you for submitting a comment on this article. It will be reviewed by JAMA Ophthalmology editors. You will be notified when your comment has been published. Comments should not exceed 500 words of text and 10 references.
Do not submit personal medical questions or information that could identify a specific patient, questions about a particular case, or general inquiries to an author. Only content that has not been published, posted, or submitted elsewhere should be submitted. By submitting this Comment, you and any coauthors transfer copyright to the journal if your Comment is posted.
* = Required Field
Disclosure of Any Conflicts of Interest*
Indicate all relevant conflicts of interest of each author below, including all relevant financial interests, activities, and relationships within the past 3 years including, but not limited to, employment, affiliation, grants or funding, consultancies, honoraria or payment, speakers’ bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued. If all authors have none, check "No potential conflicts or relevant financial interests" in the box below. Please also indicate any funding received in support of this work. The information will be posted with your response.
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 3
Customize your page view by dragging & repositioning the boxes below.
More Listings atJAMACareerCenter.com >
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.