Figure 1. Ecchymosis and edema of the lids and a diffuse subconjunctival hemorrhage are visible on external anterior segment examination of the left eye.
Figure 2. Mild fullness of the optic nerve, tortuous vessels, and choroidal folds in the macula extending superonasally of the left eye became apparent upon dilated fundus examination.
Figure 3. Optical coherence tomography (OCT) of the patient’s left macula confirmed the presence of nasal/superonasal choroidal folds.
You can skip this ad in 3 seconds.
Figure 4a. An MRI shows the patient’s orbits precontrast.
Figure 4b. This MRI shows the patient’s orbits after contrast.
A 49-year-old man with no significant medical or ocular history presented to the emergency department with painless left eye redness for 1 day. On initial evaluation, visual acuity was 20/20 OU, and slit lamp examination revealed bullous subconjunctival hemorrhage in the left eye. He was otherwise normal. On further questioning, he denied any preceding trauma, but did report recent cold symptoms with coughing and sneezing. He was discharged from the emergency department with instructions for conservative management and return precautions.
He returned to the emergency department 5 days later with worsening subconjunctival hemorrhage, pressure sensation behind the left eye, decreased vision in the left eye, and intermittent binocular horizontal diplopia. Significant examination findings included decreased VA to 20/25 OS, and a trace relative afferent pupillary defect (rAPD), also in the left eye, as well as mild limitations in supraduction, abduction, and adduction. His intraocular pressures (IOPs) were 8 mm Hg OD and 18 mm Hg OS. Hertel exophthalmometry demonstrated 4 mm of left-sided proptosis. The external anterior segment examination demonstrated ecchymosis and edema of the left eyelids, and diffuse subconjunctival hemorrhage (Figure 1). The dilated fundus examination of the left eye revealed mild fullness of the optic nerve, tortuous vessels, and choroidal folds in the macula extending superonasally (Figure 2). An OCT of the left macula confirmed the presence of nasal/superonasal choroidal folds (Figure 3).
Magnetic resonance imaging (MRI) of the orbits with and without contrast (Figure 4) revealed a well-circumscribed left intraconal mass with a fluid-fluid level suggestive of internal hemorrhage, causing mass effect with inferolateral displacement of the left optic nerve and flattening of the posteromedial globe.
Orbital lymphatic malformations (aka lymphangioma) are localized, unencapsulated, multicystic vascular malformations of the venous-lymphatic system. They are typically characterized as “low-flow” or “no-flow” malformations due to minimal internal blood flow.1 Orbital lymphatic malformations are commonly congenital, and the majority are...
Submit your diagnosis to see full explanation.
Orbital lymphatic malformations (aka lymphangioma) are localized, unencapsulated, multicystic vascular malformations of the venous-lymphatic system. They are typically characterized as “low-flow” or “no-flow” malformations due to minimal internal blood flow.1
Orbital lymphatic malformations are commonly congenital, and the majority are diagnosed in the first decade of life. However, given the low-flow state, small malformations can remain clinically imperceptible even into adulthood. In many cases, diagnosis is prompted after gradual onset of proptosis due to slow enlargement of the lesion over time. In some cases, an acute hemorrhage or thrombosis within the lymphatic malformation can occur, leading to sudden-onset proptosis, pain, and compressive optic neuropathy with loss of vision. This can occur spontaneously, or may be triggered by trauma or by recent upper respiratory infection, with increased lymphatic activity in the area (as seen in our patient with preceding cold symptoms).1
Diagnosis of orbital lymphangiomas is based primarily on clinical presentation and radiologic imaging. Computed tomography (CT) or MRI can be used to evaluate the size of the lesion, and often shows septation within the lesions with fluid levels. The differential diagnosis for lymphatic malformations includes other vascular malformations of the orbit, including orbital varix, arterio-venous malformation, and cavernous hemangioma, which imaging can help to differentiate.
Management of symptomatic orbital lymphatic malformations can be challenging, as these lesions are unencapsulated and often located deep in the orbit, making surgical access and en-bloc resection difficult. There is a high recurrence rate, as well as an associated high risk of surgical morbidity given the risk of vision-threatening retrobulbar hemorrhage.2 There may be a role for medical management of these lesions with sildenafil, a phosphodiesterase-5-inhibitor, or sirolimus, an mTOR-inhibitor; however, reports demonstrating the effectiveness of both sirolimus and sildenafil for orbital lymphatic malformations is limited to case reports and small case series at this time.3
Sclerotherapy is a more recently studied and often favorable approach for managing symptomatic or vision-threatening macrocystic orbital lymphatic malformations. Sclerotherapy treatment involves direct injection of a sclerosing agent (examples include bleomycin, pingyangmycin, ethanol, sodium tetradecyl sulfate, and OK-432/Picinibil) into the lesion under ultrasound guidance. The sclerosing agent induces irreversible endothelial cell damage, resulting in fibrosis and obliteration of the intralesional cystic spaces. Patients often require several sclerotherapy treatments before improvement in proptosis, pain, or diplopia is achieved.2,3 Our patient ultimately underwent 3 sclerotherapy treatments with subsequent return to his visual baseline and resolution of proptosis and diplopia.
This case was submitted by Makayla McCoskey, MD and Natalie Wolkow, MD PhD. Grayson W. Armstrong, MD MPH was this case’s clinical editor.
Dr McCoskey is an ophthalmology resident at Massachusetts Eye and Ear and Harvard Medical School in Boston, MA.
Dr Wolkow is an oculoplastic surgeon and assistant professor of Ophthalmology at Massachusetts Eye and Ear and Harvard Medical School in Boston, MA.
Dr Armstrong is an instructor of ophthalmology at Massachusetts Eye and Ear and Harvard Medical School. He is also Director of Ophthalmic Emergency Services at Massachusetts Eye and Ear, and is the Associate Director of Ophthalmic Medical Student Education at Harvard Medical School.
1. Graeb DA, Rootman J, Robertson WD, Lapointe JS, Nugent RA, Hay EJ. Orbital lymphangiomas: clinical, radiologic, and pathologic characteristics. Radiology. 1990;175(2):417-421. doi:10.1148/radiology.175.2.2326469
2. Nassiri N, Rootman J, Rootman DB, et al. Orbital lymphaticovenous malformations: Current and future treatments. Surv Ophthalmol. 2015;60(5):383-405. doi:10.1016/j.survophthal.2015.03.001
3. Patel SR, Rosenberg JB, Barmettler A. Interventions for orbital lymphangioma. Cochrane Database Syst Rev. 2019;5(5):CD013000. Published online May 15, 2019. doi:10.1002/14651858.CD013000