Unusual presentation of more common disease/injury
CASE REPORT
Orbital apex syndrome secondary to herpes zoster virus infection Alexia Merino-Iglesias, Javier Antonio Montero, Maria Calabuig-Goena, Luisa Fernanda Giraldo-Agudelo Department of Ophthalmology, Pio del Rio Hortega University Hospital, Valladolid, Valladolid, Spain Correspondence to Dr Javier Antonio Montero, [email protected], [email protected] Accepted 11 February 2014
SUMMARY A male patient with herpes zoster ophthalmicus (HZO) presented with left exophthalmos, external and internal ophthalmoplegia and decreased visual acuity. A CT scan revealed myositis without significant compression of the optic nerve. Intravenous acyclovir and oral steroids were started with improvement of the symptoms and eventual complete recovery.Orbital apex syndrome is a rare complication of HZO. Multiple pathogenic mechanisms are involved, including a direct cytopathic effect of the virus as in the present case. Early diagnosis and therapy may lead to complete recovery of visual function.
BACKGROUND Herpes zoster ophthalmicus is a condition in which the ophthalmic branch of the trigeminal nerve is affected by the varicella zoster virus. Neurological complications, such as ophthalmoplegia and optic neuritis, are rare but may lead to severe visual acuity loss.1–3 However, early diagnosis and therapy may lead to complete recovery of visual function.
To cite: Merino-Iglesias A, Montero JA, CalabuigGoena M, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013203200
CASE PRESENTATION A 61-year-old Caucasian man was referred to our clinic with a 5-day history of inflammation and pain in his left eye (LE). He presented left facial oedema extending to the frontal region, cheek and tip of the nose associated with vesicles and crusting papules, as well as low-grade fever (37.3°C), malaise and dizziness.
INVESTIGATIONS LE visual acuity (VA) was counting fingers at 1 m and the pupil was 3 mm wide with relative afferent defect. External examination revealed a complete external ophthalmoplegia and ptosis (figure 1) with Hertel exophthalmometry readings 11 and 15 mm, in his right and LE, respectively, and a left submandibular lymph node. Anterior segment and fundus examination were unremarkable. Optical coherence tomography (OCT) (Cirrus, Carl Zeiss Meditech, Dublin, California, USA) revealed an average retinal nerve fibre layer (RNFL) thickness within normal values (79 mm). An orbital CT disclosed unilateral left exophthalmos and increased
Figure 1 Left herpes zoster ophthalmicus with ptosis and limited supraduction and adduction of the left eye.
Merino-Iglesias A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203200
1
Unusual presentation of more common disease/injury thickness of the medial and superior rectus without significant compression of the optic nerve and no signs of sinusitis (figure 2).
TREATMENT The patient was admitted to the hospital and was started on intravenous acyclovir 10 mg/kg/8 h and oral methyl prednisolone 60 mg/day. After 7 days of treatment, LE VA was 20/400, with persistent intrinsic ophthalmoplegia, partial ptosis and restricted supraduction. The patient was discharged, and the treatment with oral antiviral and corticosteroid was completed at home. During the following 10 weeks, steroid treatment was slowly tapered as visual acuity improved to 20/25 and ocular motility was completely restored.
OUTCOME AND FOLLOW-UP After 1 year of follow-up, visual acuity was 20/20. OCT RNFL remained unchanged showing no atrophic changes.
DISCUSSION
The signs and symptoms described in this case report can be ascribed to OAS. However, the orbital CT revealed myositis with no signs of orbital apex or optic nerve compression that are necessary to establish this diagnosis. We consider that the OAS was mainly secondary to a direct cytopathic effect, which might be responsible for the complete resolution of the patient’s condition with restoration of visual acuity. Pion and Salu have reported on one case of herpes zoster ophthalmicus complicated with OAS without CT compressive signs; the authors hypothesised that the syndrome was the result of perineural inflammation caused by viral dissemination from the fifth cranial nerve.4 CT-confirmed myositis related to herpes zoster ophthalmicus has been described in two occasions in which acute compression of the orbital apex could not be demonstrated.1 2 In both cases, the authors considered that the OAS might involve a multiple mechanism such as myositis, a direct cytopathic effect and a reactive immunological response to the virus. OAS is an infrequent though potentially blinding complication of HZO. Specific conservative treatment with systemic antivirals and steroids may reduce orbital inflammation preserving final visual function.
It has been hypothesised that orbital apex syndrome (OAS) associated with ophthalmic zoster can be secondary to compression caused by intraorbital inflammation or myositis.1 4–6 Other authors have proposed a direct viral cytopathic effect caused by viral dissemination from the herpes zoster virus infecting the fifth cranial nerve to the neighbouring cranial nerves in the orbital apex. A reactive, immunologically mediated response to the virus might induce perineural inflammation, peripheral nerve demyelisation, contiguous orbital inflammation, cranial vasculitis, myositis, brainstem encephalitis and meningitis.1 2 4–6
Learning points ▸ Orbital apex syndrome is an infrequent though potentially blinding complication of OHZ. ▸ Early specific conservative treatment with systemic antivirals and steroids may reduce orbital inflammation preserving final visual function. ▸ Orbital computerised scan can aid in the differential diagnosis of orbital apex syndrome. ▸ Management of orbital apex syndrome associated with herpes zoster ophthalmicus should include systemic antiviral and steroidal therapy.
Contributors AM-I wrote the manuscript. JAM was involved in the patient diagnosis, management and critical advice. MC-G and LF-GA were involved in the patient follow-up and critical advice. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2 3 4
Figure 2 Axial (A) and coronal (B) computerised scans revealing left extrinsic muscular engorgement, especially affecting the medial and superior rectus with left exophthalmos and soft tissue swelling. Notice the absence of orbital apex compression.
2
5 6
Arda H, Mirza E, Gumus K, et al. Orbital apex syndrome in herpes zoster ophthalmicus. Case Rep Ophthalmol Med 2012;2012:854503. Kurimoto T, Tonari M, Ishizaki N, et al. Orbital apex syndrome associated with herpes zoster ophthalmicus. Clin Ophthalmol 2011;5:1603–8. Ugarte M, Dey S, Jones CA. Ophthalmoplegia secondary to herpes zoster ophthalmicus. BMJ Case Rep 2010;2010:pii: bcr1220092532. Pion B, Salu P. Herpes zoster ophthalmicus complicated by complete ophthalmoplegia and signs of pilocarpine hypersensitivity. A case report and literature review. Bull Soc Belge Ophtalmol 2007;303:23–6. Bourke RD, Pyle J. Herpes zoster ophthalmicus and the orbital apex syndrome. Aust N Z J Ophthalmol 1994;22:77–80. Sanjay S, Chan EW, Gopal L, et al. Complete unilateral ophthalmoplegia in herpes zoster ophthalmicus. J Neuroophthalmol 2009;29:325–37.
Merino-Iglesias A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203200
Unusual presentation of more common disease/injury
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Merino-Iglesias A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203200
3
CASE REPORT
Orbital apex syndrome secondary to herpes zoster virus infection Alexia Merino-Iglesias, Javier Antonio Montero, Maria Calabuig-Goena, Luisa Fernanda Giraldo-Agudelo Department of Ophthalmology, Pio del Rio Hortega University Hospital, Valladolid, Valladolid, Spain Correspondence to Dr Javier Antonio Montero, [email protected], [email protected] Accepted 11 February 2014
SUMMARY A male patient with herpes zoster ophthalmicus (HZO) presented with left exophthalmos, external and internal ophthalmoplegia and decreased visual acuity. A CT scan revealed myositis without significant compression of the optic nerve. Intravenous acyclovir and oral steroids were started with improvement of the symptoms and eventual complete recovery.Orbital apex syndrome is a rare complication of HZO. Multiple pathogenic mechanisms are involved, including a direct cytopathic effect of the virus as in the present case. Early diagnosis and therapy may lead to complete recovery of visual function.
BACKGROUND Herpes zoster ophthalmicus is a condition in which the ophthalmic branch of the trigeminal nerve is affected by the varicella zoster virus. Neurological complications, such as ophthalmoplegia and optic neuritis, are rare but may lead to severe visual acuity loss.1–3 However, early diagnosis and therapy may lead to complete recovery of visual function.
To cite: Merino-Iglesias A, Montero JA, CalabuigGoena M, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013203200
CASE PRESENTATION A 61-year-old Caucasian man was referred to our clinic with a 5-day history of inflammation and pain in his left eye (LE). He presented left facial oedema extending to the frontal region, cheek and tip of the nose associated with vesicles and crusting papules, as well as low-grade fever (37.3°C), malaise and dizziness.
INVESTIGATIONS LE visual acuity (VA) was counting fingers at 1 m and the pupil was 3 mm wide with relative afferent defect. External examination revealed a complete external ophthalmoplegia and ptosis (figure 1) with Hertel exophthalmometry readings 11 and 15 mm, in his right and LE, respectively, and a left submandibular lymph node. Anterior segment and fundus examination were unremarkable. Optical coherence tomography (OCT) (Cirrus, Carl Zeiss Meditech, Dublin, California, USA) revealed an average retinal nerve fibre layer (RNFL) thickness within normal values (79 mm). An orbital CT disclosed unilateral left exophthalmos and increased
Figure 1 Left herpes zoster ophthalmicus with ptosis and limited supraduction and adduction of the left eye.
Merino-Iglesias A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203200
1
Unusual presentation of more common disease/injury thickness of the medial and superior rectus without significant compression of the optic nerve and no signs of sinusitis (figure 2).
TREATMENT The patient was admitted to the hospital and was started on intravenous acyclovir 10 mg/kg/8 h and oral methyl prednisolone 60 mg/day. After 7 days of treatment, LE VA was 20/400, with persistent intrinsic ophthalmoplegia, partial ptosis and restricted supraduction. The patient was discharged, and the treatment with oral antiviral and corticosteroid was completed at home. During the following 10 weeks, steroid treatment was slowly tapered as visual acuity improved to 20/25 and ocular motility was completely restored.
OUTCOME AND FOLLOW-UP After 1 year of follow-up, visual acuity was 20/20. OCT RNFL remained unchanged showing no atrophic changes.
DISCUSSION
The signs and symptoms described in this case report can be ascribed to OAS. However, the orbital CT revealed myositis with no signs of orbital apex or optic nerve compression that are necessary to establish this diagnosis. We consider that the OAS was mainly secondary to a direct cytopathic effect, which might be responsible for the complete resolution of the patient’s condition with restoration of visual acuity. Pion and Salu have reported on one case of herpes zoster ophthalmicus complicated with OAS without CT compressive signs; the authors hypothesised that the syndrome was the result of perineural inflammation caused by viral dissemination from the fifth cranial nerve.4 CT-confirmed myositis related to herpes zoster ophthalmicus has been described in two occasions in which acute compression of the orbital apex could not be demonstrated.1 2 In both cases, the authors considered that the OAS might involve a multiple mechanism such as myositis, a direct cytopathic effect and a reactive immunological response to the virus. OAS is an infrequent though potentially blinding complication of HZO. Specific conservative treatment with systemic antivirals and steroids may reduce orbital inflammation preserving final visual function.
It has been hypothesised that orbital apex syndrome (OAS) associated with ophthalmic zoster can be secondary to compression caused by intraorbital inflammation or myositis.1 4–6 Other authors have proposed a direct viral cytopathic effect caused by viral dissemination from the herpes zoster virus infecting the fifth cranial nerve to the neighbouring cranial nerves in the orbital apex. A reactive, immunologically mediated response to the virus might induce perineural inflammation, peripheral nerve demyelisation, contiguous orbital inflammation, cranial vasculitis, myositis, brainstem encephalitis and meningitis.1 2 4–6
Learning points ▸ Orbital apex syndrome is an infrequent though potentially blinding complication of OHZ. ▸ Early specific conservative treatment with systemic antivirals and steroids may reduce orbital inflammation preserving final visual function. ▸ Orbital computerised scan can aid in the differential diagnosis of orbital apex syndrome. ▸ Management of orbital apex syndrome associated with herpes zoster ophthalmicus should include systemic antiviral and steroidal therapy.
Contributors AM-I wrote the manuscript. JAM was involved in the patient diagnosis, management and critical advice. MC-G and LF-GA were involved in the patient follow-up and critical advice. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2 3 4
Figure 2 Axial (A) and coronal (B) computerised scans revealing left extrinsic muscular engorgement, especially affecting the medial and superior rectus with left exophthalmos and soft tissue swelling. Notice the absence of orbital apex compression.
2
5 6
Arda H, Mirza E, Gumus K, et al. Orbital apex syndrome in herpes zoster ophthalmicus. Case Rep Ophthalmol Med 2012;2012:854503. Kurimoto T, Tonari M, Ishizaki N, et al. Orbital apex syndrome associated with herpes zoster ophthalmicus. Clin Ophthalmol 2011;5:1603–8. Ugarte M, Dey S, Jones CA. Ophthalmoplegia secondary to herpes zoster ophthalmicus. BMJ Case Rep 2010;2010:pii: bcr1220092532. Pion B, Salu P. Herpes zoster ophthalmicus complicated by complete ophthalmoplegia and signs of pilocarpine hypersensitivity. A case report and literature review. Bull Soc Belge Ophtalmol 2007;303:23–6. Bourke RD, Pyle J. Herpes zoster ophthalmicus and the orbital apex syndrome. Aust N Z J Ophthalmol 1994;22:77–80. Sanjay S, Chan EW, Gopal L, et al. Complete unilateral ophthalmoplegia in herpes zoster ophthalmicus. J Neuroophthalmol 2009;29:325–37.
Merino-Iglesias A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203200
Unusual presentation of more common disease/injury
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Merino-Iglesias A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203200
3
