Journal of Pediatric Ophthalmology and Strabismus

Short Subjects 

Severe Atypical Orbital Cellulitis in a Child With Chickenpox

Markus Groppe, PhD, MRCOphth; Ian D. Bottrill, BM, FRCS(ORL); Jonathan H. Norris, FRCOphth

Abstract

A 2.5-year-old boy developed a right-sided eyelid swelling 3 days after onset of chickenpox. Orbital imaging confirmed an orbital cellulitis. The response to antibiotics was poor. The patient improved with intravenous steroids and an ischemic vasculitis secondary to chickenpox was postulated as the primary cause of the severe clinical picture. [J Pediatr Ophthalmol Strabismus 2013;50:e49–e51.]

From the Nuffield Laboratory of Ophthalmology, University of Oxford, Oxford, United Kingdom (MG); and the Oxford Eye Hospital (MG, JHN) and ENT Department (IDB), Oxford University Hospitals, Oxford, United Kingdom.

The authors have no financial or proprietary interest in the materials presented herein.

The authors thank Mr. Raman Malhotra for his input regarding this case.

Correspondence: Markus Groppe, PhD, MRCOphth, Oxford Eye Hospital, West Wing Level LG1, Oxford University Hospitals, Headington Way, Oxford, OX3 9DU, United Kingdom. E-mail: markus.groppe@gmail.com

Received: January 06, 2012
Accepted: June 12, 2013
Posted Online: October 15, 2013

Abstract

A 2.5-year-old boy developed a right-sided eyelid swelling 3 days after onset of chickenpox. Orbital imaging confirmed an orbital cellulitis. The response to antibiotics was poor. The patient improved with intravenous steroids and an ischemic vasculitis secondary to chickenpox was postulated as the primary cause of the severe clinical picture. [J Pediatr Ophthalmol Strabismus 2013;50:e49–e51.]

From the Nuffield Laboratory of Ophthalmology, University of Oxford, Oxford, United Kingdom (MG); and the Oxford Eye Hospital (MG, JHN) and ENT Department (IDB), Oxford University Hospitals, Oxford, United Kingdom.

The authors have no financial or proprietary interest in the materials presented herein.

The authors thank Mr. Raman Malhotra for his input regarding this case.

Correspondence: Markus Groppe, PhD, MRCOphth, Oxford Eye Hospital, West Wing Level LG1, Oxford University Hospitals, Headington Way, Oxford, OX3 9DU, United Kingdom. E-mail: markus.groppe@gmail.com

Received: January 06, 2012
Accepted: June 12, 2013
Posted Online: October 15, 2013

Introduction

Varicella zoster virus causes chickenpox, which is one of the most common childhood diseases. Most cases of chickenpox will present with minor ocular manifestations in the form of eyelid and conjunctival vesicles with or without eyelid edema. These signs normally resolve without any clinical sequelae.

Complications in the form of acute retinal necrosis,1,2 retinal vasculitis,3 optic neuritis, and retinitis4 have been described. One case report of orbital involvement following chickenpox infection in an immunocompetent patient has been reported.5

We report a case of severe viral orbital cellulitis secondary to chickenpox (varicella zoster virus), which responded poorly to initial management. This case report highlights the aggressive nature of this condition and the difficulties in making an early diagnosis.

Case Report

A 2.5-year-old boy presented to an emergency department with a 3-day history of chickenpox and a 12-hour history of right-sided eyelid swelling. On examination, severe eyelid erythema and mild conjunctival injection was noted (Figure 1A). The patient was apyrexial and with a raised C-reactive protein level (65 mg/mL) and normal neutrophil count. An urgent magnetic resonance imaging scan showed extensive periorbital soft tissue swelling with no orbital involvement and an extensive periorbital cellulitis was confirmed. A working diagnosis of preseptal bacterial cellulitis was made and treatment with intravenous ceftriaxone and clindamycin was initiated after admission to the pediatric unit.

Color photographs of the patient (A) 2 hours after onset of onset of cellulitis, (B) 7 days after commencing intravenous antibiotic treatment, and (C) 2 and (D) 10 days following treatment with systemic intravenous steroids. (D) The ‘chin up position’ is shown due to the degree of postoperative ptosis.

Figure 1.

Color photographs of the patient (A) 2 hours after onset of onset of cellulitis, (B) 7 days after commencing intravenous antibiotic treatment, and (C) 2 and (D) 10 days following treatment with systemic intravenous steroids. (D) The ‘chin up position’ is shown due to the degree of postoperative ptosis.

The condition failed to improve over the next 7 days (Figure 1B) and a repeat magnetic resonance imaging scan showed extensive right periorbital soft tissue swelling and enhancement, with extraconal extension into the superior and lateral orbit extending to the orbital apex. This caused mass effect resulting in proptosis and downward displacement of the globe. There was enhancement of the posterior portions of the superior and lateral rectus muscles, but no intraconal extension. A small abscess in the superior extraconal space was suggested at this stage (Figure 2A). The patient was referred to a tertiary otolaryngology center. A sub-brow incision was performed to drain a presumed abscess, although no collection was ultimately found. Necrotic orbital fat was noted at the time of surgery, but no fluid collection or abscess was noted. Microbiology, microscopy, and culture were all negative and an orbital fat and fibrous tissue biopsy revealed no evidence of malignancy or causative agent. There was evidence of chronic inflammation but no signs of vasculitis or necrosis in the sample. The antibiotic regimen was changed to metronidazole, vancomycin, and meropenem.

(A) Coronal short T1-weighted inversion recovery magnetic resonance imaging (MRI) scan 8 days after commencing antibiotics showing extraconal extension of enhancement into the superior and lateral orbit. A mass effect in the orbit with proptosis and downward displacement of the globe can be seen. A small abscess in the superior extraconal space was suggested. (B) Sagittal T1-weighted MRI scan with gadolinium contrast 10 days after onset of cellulitis showing enhancement, with no reduction of the extraconal extension. The enhancement is avid and predominantly solid.

Figure 2.

(A) Coronal short T1-weighted inversion recovery magnetic resonance imaging (MRI) scan 8 days after commencing antibiotics showing extraconal extension of enhancement into the superior and lateral orbit. A mass effect in the orbit with proptosis and downward displacement of the globe can be seen. A small abscess in the superior extraconal space was suggested. (B) Sagittal T1-weighted MRI scan with gadolinium contrast 10 days after onset of cellulitis showing enhancement, with no reduction of the extraconal extension. The enhancement is avid and predominantly solid.

By day 10, examination revealed persistently severe eyelid edema and nonaxial proptosis with inferior globe displacement associated with severe, prolapsing conjunctival chemosis (Figure 1C). The retina and optic disc were normal and no relative afferent pupillary defect was noted. A repeat postoperative orbital magnetic resonance imaging scan revealed unchanged right periorbital soft tissue swelling and enhancement, with no reduction of the extraconal extension. The right optic nerve was displaced inferiorly and the optic nerve sheath was effaced near the orbital apex consistent with external compression. The right lacrimal gland was no longer distinguishable and has been replaced by non-enhancing material (Figure 2B).

A primary herpes zoster orbital vasculitis was suspected and intravenous dexamethasone (1 mg) was commenced. The skin involvement of the chickenpox infection resolved (Figure 1C) and antiviral treatment was not indicated. The eyelid swelling and proptosis reduced significantly but prolapsed and chemosed conjunctiva was still present, resulting in occlusion of the visual axis. Two days after commencing the steroids, conjunctival-fornix retraction sutures were placed to clear the visual axis (Figure 1D). At that point, the visual acuity seemed to be reduced (although this was difficult to formally assess) and occlusion therapy was started to reduce the risk of deprivation amblyopia.

Two weeks after commencing steroids, a computed tomography scan showed residual periorbital tissue swelling and enhancement with some stranding of the extraconal fat tissue posterior to the lacrimal gland. The visual acuity improved to 0.200 logMAR and the patient was discharged with a reducing dose of oral prednisolone (Figure 1D).

Visual acuity at last follow-up remained stable at 0.200 logMAR bilaterally with normal extraocular movements and no proptosis or globe displacement. There is a residual right upper eyelid ptosis with a degree of altered head position, which is improving slowly.

Discussion

This case illustrates the potential difficulty in the diagnosis and management of orbital cellulitis, which is associated with chickenpox disease. The clinical presentation and the initial magnetic resonance imaging scan were suggestive of periorbital cellulitis without orbital involvement. This rapidly progressed to an aggressive sight-threatening orbital cellulitis despite clinical resolution of the systemic chickenpox disease and the use of intravenous antibiotics. These findings led to the diagnosis of primary herpes zoster orbital cellulitis.

The pathogenesis is likely to be an ischemic vasculitis, which was described previously in the orbit5 and central nervous system. The inflammatory reaction of zoster-related vasculitis ranges from small vessel angiitis to granulomatous arteritis involving the larger blood vessels.6–8 Orbital involvement is a rare complication after herpes zoster ophthalmicus9 and a vasculitis is also believed to be the underlying cause. Fortunately, our patient experienced no sequelae that can occur with orbital involvement of zoster ophthalmicus.9,10 Primary orbital vasculitis in association with herpes zoster virus should be suspected in all cases of orbital involvement of patients with chickenpox or zoster ophthalmicus. Oral acyclovir treatment can reduce the symptoms, severity, and duration of chickenpox.11 Acyclovir treatment used at the time of initial presentation may have been beneficial in this case. There are no studies regarding use of acyclovir later than 72 hours of onset of symptoms. The high-dose steroid treatment was crucial in reducing the vasculitis and resulted in rapid improvement of the inflammation.

This case report highlights the importance of considering herpetic orbital disease as a possible diagnosis in children of this age and further illustrates the potentially devastating complications of a viral orbital cellulitis.

References

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10.3928/01913913-20131008-01

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