A Case Presentation from Bascom Palmer Eye Institute Grand Rounds
by Dr. Andrea Kossler
A 33-year-old female presented to the Bascom Palmer emergency room with proptosis OD for 5 months. She reported the proptosis being variable and slowly progressive. The patient noted occasional blurry vision but denied pain, double vision or headache. Past medical history was significant for a tracheotomy at birth due to a tracheo-esophageal fistula. Her mother had breast cancer. She is married and unemployed. She denies tobacco or alcohol use. She takes Percocet PRN for the pain. Past ocular history is negative. She has no known drug allergies. Review of systems was negative.
On initial clinical examination, her Va was 20/30 OD and 20/20 OS. Intraocular pressure was 20 OD and 15 OS. Both pupils were equal, round and reactive to light without an afferent papillary defect. Visual fields were full to finger count OU and color plates were 14/14 OU. Extraocular motility was slightly limited to abduction on the right. Hertel exophthalmometer measurements were 19 OD and 15 OS at a base of 104. Anterior segment examination showed injection over the lateral rectus muscle OD, but was otherwise normal.
Optic nerve photos are seen below.
Axial and coronal cuts of an MRI are shown below showing an enlarged lateral rectus muscle on the right.
Two weeks later, the patient returned with pain on abduction OD. Her clinical exam had not changed, but an orbital ultrasound was obtained showing a right lateral rectus infiltration with a thickened tendon, possibly a vascular lesion. The patient was placed on Prednisone for presumed myositis and was sent to her primary care physician to rule out a systemic malignancy.
One month later the patient returned. She did not have any improvement on steroids. She now noticed a bruit on auscultation. Her systemic evaluation was negative. An MRA was planned, but the patient found out she was pregnant, so could not undergo the study. She was otherwise stable, so the decision was to monitor her throughout her pregnancy.
Six months later the patient was 36 weeks pregnant. Her vision had decreased over the previous 2 months OD. She had increasing headaches, double vision, decreased color vision, transient visual obscurations and significantly worsening proptosis. Her Va had decreased OD to 20/70. She now had a trace APD OD and her extraocular movements were -2 in abduction and -1 in vertical gazes OD. Her Hertel measurements were 24 OD and 15 OS at a base of 101.
Follow up MRI is seen below with enlargement near the lateral rectus muscle and a dilated superior ophthalmic vein OD.
The plan at this point was for a planned C-section, keep her on Prednisone 40mg PO and follow up after delivery.
She came back 3 weeks after the C-section complaining of throbbing, blurry vision and swelling and bulging OD. She did not have any improvement on steroids, but had worsening diplopia, tearing and redness with a subjective bruit. Clinical exam was relatively unchanged from previous with the exception of significant pulsatile proptosis with engorged vessels over the right lateral rectus.
She was placed on Timolol and lubrication OD. An ultrasound, visual field and MRA/MRV/Angiogram were performed. A neurosurgery consult was also obtained.
Tests showed the following vascular malformation.
The diagnosis was an orbital arteriovenous malformation. The plan at this point was for an embolization by neurosurgery followed by a cranio-orbitotomy and excisional biopsy of the lesion.
Stay tuned for 'A Sticky Situation' – Part II for a discussion on orbital vascular lesions and follow up on our patient.