Accepted Manuscript

Herpes zoster ophthalmicus and strabismus: a unique cause of secondary Brown

syndrome

Kevin M. Broderick, MD, William R. Raymond, IV, MD, John H. Boden, MD

PII: S1091-8531(17)30476-7
DOI: 10.1016/j.jaapos.2017.06.003
Reference: YMPA 2627

To appear in: Journal of AAPOS

Please cite this article as: Broderick KM, Raymond IV WR, Boden JH, Herpes zoster ophthalmicus and
strabismus: a unique cause of secondary Brown syndrome, Journal of AAPOS (2017), doi: 10.1016/
j.jaapos.2017.06.003.

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 ACCEPTED MANUSCRIPT

Herpes zoster ophthalmicus and strabismus: a unique cause of secondary Brown syndrome

Kevin M. Broderick, MD,a William R. Raymond IV, MD,b and John H. Boden, MDc

Author affiliations: aOphthalmology Service, Brooke Army Medical Center, San Antonio, Texas;
b
Ophthalmology Service, Madigan Army Medical Center, Tacoma, Washington; cOphthalmology
Service, Fort Belvoir Community Hospital, Ft. Belvoir, Virginia

Disclaimer: The opinions in this manuscript are those solely of the authors and do not represent
the views or official policies of the United States Army or Department of Defense.

Submitted August 19, 2016.

Revision accepted February 2, 2017.

Correspondence: Kevin M. Broderick, MD, Ophthalmology Service, Brooke Army Medical

Center, 3551 Roger Brooke Dr., Ft. Sam Houston, TX 78234 (email:
kevin.m.broderick2.mil@mail.mil).

Word count: 1,006

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Herpes zoster ophthalmicus can be associated with a variety of ocular and visual sequelae,

including isolated or even multiple cranial neuropathies, potentially affecting the

oculomotor, trochlear, or abducens nerves. We report a case of a secondary Brown

syndrome following resolution of a unilateral isolated trochlear nerve palsy associated with

herpes zoster ophthalmicus in an immunocompetent 57-year-old man.

Case Report

A 57-year-old man diagnosed in early January 2016 with a herpes zoster infection in the right V1

dermatome presented at Fort Belvoir Community Hospital, Ft. Belvoir, Virginia, with acute

onset of worsening binocular diplopia over 2 days. He had been diagnosed with herpes zoster by

his primary care provider 5 days prior to presentation based on the classic dermatologic changes

and started on oral valacyclovir. His past medical, surgical, ocular, and family history were

otherwise unremarkable, except for receiving monovision LASIK in his left eye in 2015.

Review of systems was largely unremarkable, and the patient denied any systemic symptoms or

neurosensory changes aside from his acute binocular diplopia. Prior to this episode, the patient

was in an excellent health, with no underlying medical issues or chronic medication use and a

healthy exercise regimen.

Initial evaluation on presentation revealed a vesicular rash in various stages of healing

involving the right V1 dermatome. A few vesicular lesions were noted on the right upper eyelid,

with mild associated lid edema and erythema. No involvement of the nose was noted. Corrected

Snellen visual acuity was 20/20 in the right eye and J1+ on near card in the left eye. Intraocular

pressure and pupillary examination were within normal limits. Ocular motility was full in both

eyes, although he had a notable right hypertropia and associated left head tilt of approximately

10 degrees. The Parks-Bielschowsky three-step test isolated a right trochlear nerve palsy. The
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remainder of his ophthalmologic examination was unremarkable.

The Neurology and Infectious Disease Services recommended magnetic resonance

imaging (MRI) of the brain, lumbar puncture, and admission for a 10-day course of inpatient

intravenous acyclovir, given the apparent involvement of the trochlear nerve and concern for

associated central nervous system involvement. The brain MRI revealed nonspecific cerebral

deep white matter foci but was otherwise unremarkable, including normal anatomy of the orbit,

lacrimal glands, extraocular muscles, and optic nerves. The lumbar puncture showed elevated

protein and lymphocytic predominance. Further cerebrospinal fluid testing with varicella zoster

virus PCR was positive, consistent with zoster meningitis.

After the patient completed his course of acyclovir he was followed closely. His trochlear

nerve palsy persisted, with unchanged measurements and stable diplopia over the month

following discharge. He did not develop any other ocular or visual complaints or any systemic

sequelae. His systemic health status was monitored closely during this time period, and he

remained overall asymptomatic outside of his diplopia symptoms. Extensive systemic evaluation

by the Infectious Disease and Neurology Services was completely unremarkable, including a

negative HIV test. Routine labs were ordered by his primary care provider in March 2016 and

included an unremarkable lipid panel, complete blood count, comprehensive metabolic panel,

Hemoglobin A1c, and prostate-specific antigen level. The only abnormality noted was a slightly

low Vitamin D and B12 level, for which the patient was started on oral supplementation.

At his follow-up examination in April 2016, approximately 4 months after onset of his

vesicular lesions, the patient had noted a progressive change in his diplopia symptoms. He no

longer endorsed any diplopia in primary gaze and was only able to induce diplopia on left gaze.

His corrected Snellen visual acuity was 20/20 in the right eye and J1+ on near card in the left
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eye. His intraocular pressure and pupillary examination were within normal limits. His ocular

motility examination was notable for a duction deficit of his right eye in upgaze, worse in

adduction (Figure 1). Double Maddox rod testing showed no torsional component. Given the

significant and unexpected change in the patient’s examination, a follow-up MRI of the brain

and orbits was obtained and found to be unremarkable.

At his follow-up visit 1 month later his muscle balance testing and symptoms remained

stable. A forced ductions test was performed in the clinic and found to be positive for a right

Brown syndrome. Treatment options with local or oral steroids versus watchful waiting were

discussed with the patient. Because he was asymptomatic in primary gaze, the patient elected

not to start steroids but to return for follow-up examination in 3 months.

Discussion

This case illustrates a clinical complication and uniquely evolving course of herpes zoster

ophthalmicus (HZO). Several case reports and series have sited multiple different cranial

neuropathies as sequela of HZO, both as isolated syndromes and in conjunction with other ocular

or orbital changes.1-4 Chhabra and Golnik1 report an incidence of ocular motor cranial nerve

palsies of 5%-31% of HZO cases. They also had a total of 5 patients who developed trochlear

nerve palsy as part of their HZO course. Ryu and colleagues2 describe a healthy 13-year-old girl

who developed an isolated trochlear nerve palsy following HZO. None of these cases, however,

was reported to have subsequently developed a Brown syndrome.

To our knowledge, there are currently no reported cases of a secondary Brown syndrome

after HZO, especially following apparent resolution of a cranial neuropathy. One possible

explanation for the development of a Brown syndrome after a HZO associated superior oblique

palsy is that the inflammatory process of the trochlear nerve and superior oblique muscle could
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have resulted in subsequent scaring and fibrosis of the trochlea. Another possible cause could be

secondary to a zoster-related orbital myositis. Although it has never been associated with a

Brown syndrome, orbital myositis associated with herpes zoster has been reported in the

literature.5-7 The patient’s follow-up MRI, however, did not reveal any abnormalities of the orbit

or extraocular muscles that would suggest an underlying orbital myositis as the etiology.

Literature Search

PubMed/MEDLINE was searched as of August 18, 2016, without date restriction, for English-

language articles using combinations of the following terms: herpes zoster, herpes zoster

ophthalmicus, HZO, zoster, strabismus, Brown syndrome, and superior oblique tendon sheath

syndrome.
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References

1. Chhabra MS, Golnik KC. Recovery of ocular motor cranial nerve palsy after herpes

zoster ophthalmicus. J Neuroophthalmology 2014;34:20-22.

2. Ryu WY, Kim NY, Kwon YH, Ahn HB. Herpes zoster ophthalmicus with isolated

tochlear nerve palsy in an otherwise healthy 13-year-old girl. J AAPOS 2014;18;193-4.

3. Park KC, Yoon SS, Yoon JE, Rhee HY. A case of herpes zoster ophthalmicus with

isolated trochlear nerve involvement. J Clin Neurol 2011;7:47-9.

4. Sanjay S, Chan EW, Gopal L, Hegde SR, Chang BC. Complete unilateral

ophthalmoplegia in herpes zoster ophthalmicus. J Neuroophthalmol 2009; 29:325-37.

5. Kawasaki A, Borruat FX. An unusual presentation of herpes zoster ophthalmicus: orbital

myositis preceding vesicular eruption. Am J Ophthalmol 2003; 136: 574-5.

6. Badilla J, Dolman PJ. Orbital myositis involving the oblique muscles associated with

herpes zoster ophthalmicus. Ophthal Plast Reconstr Surg 2007; 23:411-13.

7. Paraskevas GP, Anagnostou E, Vassilopoulou S, Spengos K. Painful ophthalmoplegia

with simultaneous orbital myositis, optic and oculomotor nerve inflammation and

trigeminal nucleus involvement in a patient with herpes zoster ophthalmicus. BMJ Case

Rep 2012 Oct 29;2012.

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FIG 1. Clinical photographs of nine cardinal gaze positions. The patient is orthophoric with

normal head position in primary gaze, with a right hypotropia in upgaze worsening on up-and-

left gaze, consistent with a Brown syndrome.

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