Rhino-orbito-cerebral mucormycosis complicated with an ophthalmic artery occlusion followed by subarachnoid hemorrhage

doi:10.4236/crcm.2013.26093

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Vol.2, No.6, 345-347 (2013) Case Reports in Clinical Medicine

http://dx.doi.org/10.4236/crcm.2013.26093

Rhino-orbito-cerebral mucormycosis complicated

with an ophthalmic artery occlusion followed by

subarachnoid hemorrhage

Kun Wook Kang, Young Ki Kwon, Jae Pil Shin, In Taek Kim, Dong Ho Park*

Department of Ophthalmology, Graduate School of Medicine, Kyungpook National University, Daegu, South Korea;

*Corresponding Author: [email protected]

Received 14 July 2013; revised 5 August 2013; accepted 12 August 2013

which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

A 70-year-old female with poorly controlled dia-

betes developed sudden visual loss, ptosis and

complete ophthalmoplegia of the right eye. Fun-

duscopic examination showed the pale retina

and the cherry red spot in the right eye. Fluo-

rescein angiography and indocyanine green an-

giography demonstrated the absence of retinal

arterial filling and choroidal perfusion in the

right eye even 20 minutes after injecting the dye.

The p atient was diagnosed with right ophthalmic

artery occlusion. Computed tomography (CT)

showed diffuse mucosal thickening in the right

ethmoidal sinus. Based on the clinical findings

and endoscopic biopsy result, mucormycosis

was confirmed. Amphotericin B (40 mg/day) and

ceftriaxone (2 g/day) were intravenously admi-

nistered. Despite the improvement of the right

ethmoidal sinusitis and the right proptosis, the

patient deteriorated into a comatose state after

19 days of systemic amphotericin B therapy.

Although the previous CT showed no cerebral

aneurysm, a repeated CT showed newly devel-

oped posterior communicating artery aneurysm

and the subarachnoid hemorrhage. Despite the

amphotericin B treatment and the improvement

of the sinusitis, mucormycosis could cause sud-

den cerebral aneurysm rupture and subarach-

noid hemorrhage resulting in coma.

Keyw ords: Intracranial Aneurysm; Mucormycosis;

Ophthalmic Artery; Rhino-Orbito-Cere bral

Mucormycosis; Subarach noid Hemorrhage

1. INTRODUCTION

Mucormycosis is a fungal infection caused by the

family Mucoraceae-pathogenic genera Rhizopus, Absidia

and Mucor [1]. Infections typically occur in immuno-

compromised patients with diabetic ketoacidosis, lym-

phoma, leukemia, corticosteroid treatment, radiation ther-

apy, chemotherapy, myelodysplastic syndrome, aplastic

anemia and the acquired immunodeficiency syndrome

[2]. Rhino-orbito-cerebral mucormycosis is a rare oppor-

tunistic fungal infection that is potentially fatal.

We report a rare case of rhino-orbito-cerebral mucor-

mycosis complicated with an ophthalmic artery occlu-

sion followed by sudden onset subarachnoid hemorrhage.

2. CASE REPORT

A 70-year-old female presented to hospital with a three

day history of visual loss and ptosis of the right eye. The

patient was diagnosed with diabetes 30 years ago but

took the medication irregularly for recent 2 weeks. Blood

glucose level at admission was 516 mg/dL.

The visual acuity was no light perception in the right

eye and 20/40 in the left eye with a relative afferent pu-

pillary defect on the right side. Complete restriction of

ocular motility in all fields of gaze, ptosis and proptosis

of the right eye were observed. Funduscopic examination

showed the pale, edematous retina and the cherry red

spot in the right eye (Figure 1(A)). Fluorescein angio-

graphy an d indocyan ine gre en angiography demonstrated

the absence of retinal arterial filling and choroidal p erfu-

sion in the right eye even 20 minutes after injecting the

dye (Figure 1(B)). The patient was diagnosed with right

ophthalmic artery occlusion.

Computed tomography (CT) of the brain and orbit

showed diffuse mucosal thickening in the righ t eth moidal

sinus but there was no involvement of the cavernous si-

nus or the cavernous carotid artery (Figure 1(C)). The

patient underwent an urgent endoscopic nasal exploration.

Rhinologic exam revealed a dark brown colored eschar

K. W. Kang et al. / Case Reports in Clinical Medicine 2 (2013) 345-347

346

at the right nasal septum. The pathologic examination of

the surgical specimen at the right nasal septum revealed

broad, aseptate hyphae, morphologically consistent with

mucormycosis. The definitive diagnosis of mucormyco-

sis was made on histopathologic examination (Figure

1(D)). Amphotericin B (40 mg/day) and ceftriaxone (2

g/day) were intravenously administered.

Despite the improvement of the right ethmoidal si-

nusitis and the right proptosis after 9 days of systemic

amphotericin B therapy (Figure 2(A)), the patient dete-

riorated into a comatose state after 19 days of systemic

amphotericin B therapy. Although the previous CT show e d

no cerebral aneurysm (Figure 2(C)), a repeated CT scan

of the brain showed newly developed right posterior

communicating artery aneurysm and the subarachnoid

hemorrhage with a right temporal lobe hematoma (Fig-

ures 2(B) and 2( D)).

Rhino-orbito-cerebral mucormycosis was diagnosed.

Endovascular coil embolization of posterior communi-

cating artery and craniotomy for subarachnoid hemor-

rhage were performed. The patient was still comatose.

Figure 1. Fundus photograph revealed a pale retina and cherry

red spot in the right eye (A). Fluorescein angiography revealed

absence of retinal arterial filling and choroidal perfusion in the

right eye (B). Computed tomography showed diffuse mucosal

thickening in the right ethmoidal sinus (C, black arrow). Biopsy

of right nasal septum showed broad aseptate fungal hyphae

with acute angle branching (H&E, ×400) (D).

Figure 2. Brain CT showing the improvement of the right eth-

moidal sinusitis (A). Brain CT showing the subarachnoid hem-

orrhage with a right temporal lobe hematoma ((B), arrow). Al-

though the previous CT showed no cerebral aneurysm ((C), ar-

row), a repeated CT scan showed newly developed right poste-

rior communicating artery aneurysm ((D), arrow).

3. DISCUSSION

Rhino-orbito-cerebral mucormycosis is a fulminant

opportunistic infection in diabetic or immunocompro-

mised patients. In the present case, the patient had poorly

controlled diabetes and the fungal ethmoidal sinusitis.

The patient was diagnosed with right ophthalmic artery

occlusion and complete ophthalmoplegia. Visual loss re-

sults from retinal infarction due to hypoperfusion of the

central retinal artery, anterior ischemic optic neuropathy

and choroidal ischemia due to compromised posterior

ciliary arteries [3]. It is caused by mucorthrombosis of

the ophthalmic artery and direct invasion of the optic

nerve by fungus infection [4]. Ophthalmoplegia results

from impaired blood supply to th e extraocu lar muscles or

to the ocular motor nerves [5].

Direct local invasion and spread via blood stream are

two common modes of spreading in cephalic mucormy-

cosis. By spreading through vessels and nerves, this fun-

gal infection causes the vascular invasion, cranial nerve

involvement, distant brain ab scess, septic (mycotic) cere-

bral embolism, cerebral infarction and aneurysm [6,7].

Infectious aneurysms of cerebral blood vessels are rare,

representing only 2% to 5% of all intracranial aneurysms.

Although infectious aneurysms usually result from bac-

terial infection, they may also result from fungal infec-

tion, causing true mycotic or fungal aneurysms. Fungal

intracranial aneurysms arise almost exclusively in the

K. W. Kang et al. / Case Reports in Clinical Medicine 2 (2013) 345-347

347

setting of an extravascular infection in an immunocom-

promised host [8]. In the present case, the patient with

poorly controlled diabetes complained of only ocular

symptom and brain CT showed no infectious cerebral

aneurysm at admission. After 9 days of systemic ampho-

tericin B therapy, brain CT showed the improvement of

the proptosis of the right eye and the resolution of mu-

cosal thickening of ethmoidal sinus. However, 19 days

after systemic amphotericin B therapy, the patient dete-

riorated into a comatose state. Brain CT showed the dif-

fuse subarachnoid hemorrhage with a right temporal lobe

hematoma due to ruptured aneurysm. Within 19 days,

new cerebral aneurysm developed and progressed rapidly.

It supported our su spicion of a fungal aneur ysm.

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Survival of mucormycosis with intracerebral involve-

ment was thought to be virtually impossible un til the first

case of clinical recovery reported in 1955 [9]. The intro-

duction of amphotericin B in the 1960s reduced mortality

from 70% to 40% [10]. Nevertheless, the prognosis of

rhino-orbito -cerebral mucormycosis remains poo r and 20

to 50% of patients expire [11]. In the present case, the

ethmoidal sinusitis and the proptosis of the right eye

were improved after amphotericin B treatment. However

the subarachnoid hemorrhage due to ruptured aneurysm

occurred in a short period. Although endovascular coil

embolization of posterior communicating artery and cra-

niotomy for subarachnoid hemorrhage were performed,

the patient was still comatose.

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In conclusion, systemic amphotericin B antifungal

treatment improves the sinusitis and the proptosis. Nev-

ertheless, new cerebral aneurysm can develop in a short

period and it can cause subarachnoid hemorrhage. Oph-

thalmologists shou ld raise the suspicion of the infectious

mycotic cerebral aneurysm and the subarachnoid hem-

orrhage resulting in coma, even if the sinusitis is im-

proved after amphotericin B treatment.

[8] Asari, S., Nishimoto, A. and Murakami, M. (1988) A rare

case of cerebral aspergillus aneurysm at the site of tem-

porary clip application. No Shinkei Geka, 16, 1079-1082.

[9] Harris, J.S. (1955) Mucormycosis; report of a case. Pedi-

atrics, 16, 857-867.

[10] Shah, P.D., Peters, K.R. and Reuman, P.D. (1997) Recov-

ery from rhinocerebral mucormycosis with carotid artery

occlusion: A pediatric case and review of the literature

Pediatric Infectious Disease Journal, 16, 68-71.

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4. ACKNOWLEDGEMENTS

This research was supported by the Basic Science Research Program

through the National Research Foundation of Korea (NRF) funded by

the Ministry of Education, Science and Technology (2012004585) and

by the Korea Health Technology R&D Project, Ministry of Health &

Welfare, Repu blic of Korea (A111345).

[11] Toumi, A., Larbi Ammari, F., Loussaief, C., Hadhri, R.,

Ben Brahim, H., Harrathi, K., Ben Romdhane, F., Koubaa,

J. and Chakroun, M. (2012) Rhino-orbito-cerebral mucor-

mycosis: Five cases. Médecine et Maladies Infectieuses,

42, 591-598. doi:10.1016/j.medmal.2012.10.001