HZO Warrants Quick Recognition & Action

A rare combination of ophthalmoplegia, panuveitis and optic neuritis from herpes zoster emphasizes the need for prompt diagnosis and action.

Samuel Yun, MD, Ryan Wong, MD, Ajay Malholtra, MBBS, John Huang, MD, and Flora Levin, MD, New Haven, Conn.
6/5/2014

Herpes zoster ophthalmicus is a relatively common disease that often affects elderly population with rare, but devastating intraocular, neuro-ophthalmic complications if not recognized promptly and treated appropriately. To re-emphasize the importance of prompt diagnosis and treatment, we share this brief review and a case report of what clincians may encounter in practice.

Background

The lifetime risk of having herpes zoster is 20 to 30 percent, with 10 to 20 percent of the patients developing herpes zoster ophthalmicus.1 HZO may present with diverse ocular and central nervous system symptoms with 50 percent of patients having ophthalmic manifestations.1 HZO has caused extraocular muscle palsies of the third, fourth and sixth cranial nerves in 7 to 31 percent of patients and is usually a self-limiting condition.2 Optic neuritis and orbital involvement from HZO has been described.3-6 However, panuveitis and orbital disease in one patient is rare.

Diagnosis and Treatment

As initially described in 1965, HZO reactivation is largely dependent on depressed cellular immunity as is often the case in patients on immunosuppression therapy.1,7 Various ocular complications of the HZO may include inflammation in any part of the eye including blepharitis; keratoconjunctivitis; iritis; and scleritis as well as intraocular involvement of anterior uveitis and posterior uveitis involving the retina.8 Optic neuritis has been documented as well and may be bilateral even in unilateral HZO.5

 
Figure 1. External photography of eye positions in four directions.
Several cases of orbital apex syndrome in HZO have been reported.9 There is one case report of an immunocompromised patient with anterior uveitis, optic neuritis, complete ptosis and ophthalmoplegia due to myositis.10 Our case is unique in that it involves panuveitis, orbital apex syndrome and optic neuritis. Although the pathophysiology of cranial nerve involvement is not yet clear, it is suggested to be due to either direct viral invasion or a combination of inflammation and occlusive vasculitis.11-13

Treatment of HZO is mainly antivirals. Combination of IV acyclovir as initial therapy in the acute phase and valacyclovir as an outpatient regimen can be often utilized. Tapering doses of oral prednisone in 60, 30 and 15 mg/day per week may be used as well in cases of severe pain, severe rash and cranial polyneuritis.14,15 Our patient’s condition significantly improved with oral antiviral alone in terms of visual acuity, motility and inflammation.

Without increased awareness of the disease, herpes virus often has protean presentations that may be misdiagnosed initially, as it was in this case.

Case Report

An 82-year-old retired nun with a history of dementia was referred for evaluation of right visual loss and ophthalmoplegia. The patient initially presented to an outside emergency department for altered mental status and was discharged to a short-term rehabilitation facility with a diagnosis of worsened depression. During the admission, she was treated with topical and oral antibiotics for presumed right conjunctivitis. Two weeks later she was seen by an outside ophthalmologist and found to have right vision loss and ophthalmoplegia. She was referred to neuro-ophthalmology for further evaluation.

The patient complained of a gradual decrease in vision, redness and swelling of the right eye over one month. She also described a non-painful rash over her right eye, which preceded all other symptoms. Her systemic review of systems was otherwise negative. Pertinent medical history includes history of herpes zoster over the abdomen 10 years previously.

On examination, the patient was awake and alert. Best-corrected visual acuity was count finger at 3 feet in the right eye, and 20/30 in the left. Pupillary exam showed a dilated, poorly reactive right pupil with a relative afferent defect. Intraocular pressure was 24 mmHg in the right eye and 12 mmHg in the left. There was ophthalmoplegia with -3 restriction of supraduction, -2 abduction, -2 infraduction, and -4 adduction (See Figure 1).

Figure 2. Axial post-contrast, fat suppressed T1-weighted MRI images showing the right optic sheath enhancement (black arrow) with orbital apex involvement (white arrow); asymmetric enhancement in the right superior orbital fissure extending along the right cavernous sinus, right Meckel’s cave and cisternal segment of the right trigeminal nerve.
External exam revealed near complete ptosis of the right eye with eyelid swelling and a scattered vesicular rash with crusts in the V1 and 2 distributions. There was right corneal hypoesthesia. Slit-lamp exam showed right conjunctival injection, corneal edema and a hyphema with neovascularization of the iris. There was dense nuclear sclerosis and vitritis that precluded fundoscopic examination. Right B-scan ultrasonography showed dense vitreous debris without retinal detachment.

The patient was admitted to the hospital and started on IV acyclovir (10 mg/kg), and topical prednisolone and cyclopentolate eye drops.

Laboratory evaluations for other infectious and inflammatory etiologies were negative. HIV status was not documented. A contrast-enhanced MRI of the brain and orbit showed right optic nerve sheath enhancement with intraconal inflammation and right trigeminal nerve enhancement of V1 and V2 distribution (See Figure 2).

After 48 hours of systemic therapy with acyclovir, the patient’s vision and orbital findings began to improve. On one week follow-up after discharge with oral acyclovir, the patient’s right visual acuity improved to 20/60 and intraocular pressure decreased to 18 mmHg without any ocular hypotensive therapy. Motility improved in all directions. Vitritis subsided to allow dilated fundus exam, which showed vitreous debris. The optic nerve appeared sharp and pink with no retinal involvement.  REVIEW


The authors are at the Yale School of Medicine. Drs. Yun, Wong, Huang and Levin are in the Department of Ophthalmology and Visual Sciences; Dr. Malhotra is in the Department of Neuroradiology.



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