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Girl presents with headache, binocular diplopia
Previous imaging showed evidence of sinusitis.
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An 8-year-old girl presented to Tufts Medical Center with complaints of a persistent headache and new-onset binocular diplopia. She was initially seen by her primary care provider 1 week earlier for her headache and ultimately diagnosed with sinusitis and started on oral amoxicillin. However, 2 days after starting oral antibiotics, she developed fevers, intermittent nausea and binocular diplopia. She otherwise felt well and had no history of headaches. She had no significant medical or ocular history.
Examination
On examination, the patient’s vision was 20/20 in the right eye and 20/30 in the left eye. With testing of extraocular motility, she had a left abduction deficit with a measured 30 ∆D esotropia in primary and left gaze and 16 ∆D esotropia in right gaze (Figure 1). The rest of her motility exam was normal. She had no proptosis, and her IOP, color vision, confrontation visual fields and pupillary exam were normal. Her slit lamp exam and funduscopy were likewise unremarkable.
The patient had prior imaging for her headaches, which included a reportedly negative head CT 1 week prior and subsequent MRI/MRV that showed evidence of sinusitis.
Figure 1. Left abduction deficit on left gaze. There were no other extraocular motility abnormalities.
Images: Liang MC, Strominger MB
What is your diagnosis?
Headache, sinusitis, abduction deficit
The differential diagnosis for a headache with sinusitis and an abduction deficit included infectious and noninfectious inflammatory causes, as well as a post-viral nerve palsy and ophthalmoplegic migraine.
Infectious causes included orbital cellulitis with or without subperiosteal abscess, cavernous sinus thrombosis, intracranial abscess and meningitis. Noninfectious etiologies included orbital pseudotumor and Tolosa-Hunt syndrome.
Our patient was admitted to the hospital for further work-up. Initial blood work showed an elevated white count and thrombocytosis but was otherwise normal. She also had blood cultures that were negative as well as a negative monospot and Lyme antibodies.
Although prior scans showed evidence of sinusitis, neuroimaging did not reveal evidence of orbital cellulitis or subperiosteal abscess, enlarged superior ophthalmic vein or intracranial pathology. A repeat CT scan, however, was performed after development of new diplopia.
Aside from showing diffuse sphenoid and ethmoid sinusitis, there was concern for extension into the intracranial compartment near the left orbital apex. In addition, there was visible enlargement of the left cavernous sinus with enhancement of the dura, focal bony erosion and poor outline of the left internal carotid artery (ICA) (Figure 2). Repeat MRI with MRA showed a hypoplastic intracranial left ICA with focal dilation of the cavernous segment, worrisome for a pseudoaneurysm or mycotic aneurysm (Figure 3).
Figure 2. CT scan with contrast showed ethmoid and sphenoid sinusitis with a possible area of fluid near the orbital apex that was worrisome for intracranial extension (a). Further evaluation of the scans revealed enlargement of the left cavernous sinus with possible focal bone dehiscence, dural enhancement and poor visualization of the left ICA. There was no orbital inflammation (b).
Figure 3. MRI/MRA showed a hypoplastic left intracranial ICA with focal dilation of the cavernous segment, concerning for possible pseudoaneurysm or mycotic aneurysm.
Figure 4. After readmission to the hospital, the patient developed complete ptosis of the left upper eyelid and limited extraocular motility in all gazes due to a new third nerve palsy and resolving sixth nerve palsy. Her pupillary exam was normal.
Figure 5. Imaging at the time of surgery revealed a left cavernous ICA aneurysm just proximal to the origin of the left ophthalmic artery.
During her admission, the patient was treated with intravenous vancomycin and intravenous ceftriaxone. She also underwent endoscopic sinus surgery with ENT for presumed sinusitis causing a sixth cranial nerve palsy. With close postoperative clinical and radiologic follow-up, she was discharged home 5 days after surgery after resolution of her headaches and fevers with a planned 2-week course of intravenous antibiotics. However, she was readmitted to the hospital the day after discharge with a recurrent fever to 102°F and worsening headache. Her clinical exam was otherwise unchanged, and she went back to the OR for repeat sinus washout with improvement again in her symptoms.
Three days after her second surgery, however, she developed new left-sided ptosis and restricted extraocular motility (Figure 4). Her pupillary exam was still normal. With development of a new apparent pupil-sparing third nerve palsy, there was concern for further intracranial extension involving the cavernous sinus or enlargement of the previously identified aneurysm. She was taken to the operating room immediately for coiling of the left ICA aneurysm (Figure 5).
Discussion
Acute sinusitis is a predominant cause of orbital infection in children. Usual presenting symptoms and signs are headache, fever, upper respiratory symptoms, periorbital erythema and edema, proptosis and limited extraocular movements. Although ethmoid sinusitis is most common, sphenoid sinusitis is often more concerning due to complications from its close proximity to the intracranial and orbital contents. Headache and orbital symptoms are usually more apparent than nasal symptoms with this location of sinusitis, and the most common intracranial complication is meningitis, followed by cavernous sinus thrombosis.
Kavita Bhavsar
Michelle C. Liang
Diplopia can occur in acute sinusitis due to an inflammatory tissue reaction of either the extraocular muscles or cranial nerves. The sixth cranial nerve is the most commonly affected due to its medial location in the cavernous sinus. The third nerve is less commonly affected but can be due to ischemia or compression, the latter more often seen with pupillary involvement due to loss of the parasympathetic fibers running along the nerve. In patients, specifically children, with symptoms and signs of sinusitis, it is important to obtain a CT with contrast to rule out post-septal involvement or abscess formation. A head MRI may also be indicated if there is suspicion for intracranial involvement.
Patients should be admitted to the hospital if they do not improve on outpatient oral therapy or if there is ophthalmic or intracranial involvement. Usual treatment includes broad-spectrum intravenous antibiotics, nasal decongestants and steroids. Sinus surgery may also be required if more conservative treatment fails or if there is ocular or neurologic sequelae. Specifically, infectious, or mycotic, aneurysms, although rare, can occur secondary to systemic or local infections. Imaging options include CTA or MRA, and treatment includes intravenous antibiotics and neurosurgery, if necessary.
Follow-up
One month after hospital discharge, our patient was 20/20 in both eyes with correction and had mild left upper lid ptosis and diplopia only on left lateral gaze. Five months later, she had no residual ptosis or diplopia and measured only a small esophoria on left gaze.