Fungal sinusitis with ocular involvement: Case report

doi:10.4236/crcm.2013.29134

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Vol.2, No.9, 513-516 (2013) Case Reports in Clinical Medicine

http://dx.doi.org/10.4236/crcm.2013.29134

Fungal sinusitis with ocular involvement: Case report

Achyut N. Pandey1*, Anil Kakde2

1Department of Ophthalmolog y, VCSG Medical College and Research Institute, Srinagar-Garhwal, Uttarakhand, India;

*Corresponding Author: [email protected]

2Eye Q Super Speciality Eye Hospital, Gurgaon, India

Received 7 October 2013; revised 5 November 2013; accepted 3 December 2013

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

ABSTRACT

Rhino-orbital-cerebral mucormycosis (ROCM) is

an acute, often fatal, fungal infection caused by

members of the class Zygomycetes and the or-

der Mucorales. The genus Rhizopus accounts

for most cases of ROCM. The disease is char-

acterized by fungal hyphal invasion of blood

vessels resulting in thrombosis and i nfarction of

the nasal, paranasal sinus, orbital, and cerebral

tissues. The most commonly associated condi-

tion is diabetes mellitus; other associated con-

ditions include immunocompromised states, re-

nal disease, deferoxamine use and acidotic sta-

tes. The most frequent sites of infection are

pulmonary, rhinocerebral, cutaneous and dis-

seminated. Rhino-orbital and Rhino-cerebral are

two forms of the disease. As such the condition

is a medical emergency. Early recognition and

treatment are essential because it may lead to

death in a few days. CROP usually begins in the

palate or paranasal sinuses and rapidly spreads

to the orbital contents. Proptosis, loss of vision

and ophthalmoplegia occur and death from ce-

rebral involvement commonly ensues. The fun-

gus tends to invade arteries and cause throm-

bosis and tissue infarction. Rhizopus is the

most commonly isolated genus in CROP, ac-

counting for almost all cases. The diagnosis can

be strongly suspected by the characteristic

clinical manifestations. Therapy includes the

treatment of the underlying disease, surgical

excision of the necrotic tissue containing fungal

element s and the systemic administr ation of am-

photericin-B. Here we report the clinical fea-

tures of a 32-years-old man presented mucor-

mycosis.

Keywords: Diabetes Mellitus; Mucormycosis;

Rhizopus spp.

1. CASE REPORT

A 32-years-old male patient presented a history of pain

in the nose and defective vision in the left eye since one

week. The pain was not relieved. He was apparently all

right one week prior. He was a diabetic incidientally

found one week back only. On admission, he was found

to be a afebrile, conscious and well oriented to the time

and place, with a BP of 140/90 mmHg, a pulse of 82/min

and with a swelling on the nose. Vision is in RE 6/18 and

LE 6/HM. On examination patient is found to have con-

vergent squint. Extra ocular movements were restricted

in all six gazes, indicating the involvement of 3, 5 and 6

cranial nerve (Figure 1). Corneal sensation is decreased

in BE .There was no proptosis presented. IOP is in RE

22.4 and LE 18.9. People in both eyes were round, re-

acting to light both direct and consensual light reaction.

Pt was dilated for fundus examination, and BE fundus

found to be normal. His fasting blood sugar was 296

mg/dl, sodium was 12 mEq/L, potassium was 2.5 mEq/L.,

urea was 47 mg/dl, creatinin e was 0.9 mg/dl and haemo-

globin was 11.7 gm%. All other lab investigations are

found to be normal. His nasal swab and maxillary and eth-

moid sinus curettages were received in the microbiology

laboratory for KOH mounts and fungal cultures to check

for mucormycosis. The microscopic examination of the

biopsy material and the nasal discharge was done in 10%

KOH wet mounts. It showed the characteristic broad,

aseptate, branched hyphae (Figures 2, 3). The fungus

was grown on Sabouraud’s dextrose agar at 37˚C. A mi-

croscopic examination of the growth which was done in

a lactophenol cotton blue mount, revealed that it was Rhi-

zopus (Figure 4). Further patient is advised for CT scan

orbit and para nasal sinuses for orbital or cerebral spread

A. N. Pandey, A. Kakde / Case Reports in Clinical M edicine 2 (2013) 513-516

514

Figure 1. Patient with convergent squint, EOM

restriction and swollen nose.

Figure 2. KOH.

Figure 3. Growth on SDA.

Figure 4. Lactophenol cotton blue.

(Figure 5). CT scan was found to be normal. The patient

was started on an intravenous insulin infusion and am-

photericin B at 0.3 mg/kg/day and this was gradually

increased to 1 mg/kg/day with the monitoring of the se-

rum electrolytes and the renal functions.

2. DISCUSSION

Mucormycosis are a group of invasive infections

which are caused by filamentous fungi of the order, Mu-

corales of the Mucoraceae Family. Rhino-orbital mu-

cormycosis is an aggressive, angioinvasive fungal infec-

tion which is seen inimmunocompromised hosts. The

risk factors are poorly controlled Diabetes mellitus, hae-

matological malignancies and a prolonged corticosteroid

treatment. The infections which are caused by members

of the order mucorales are primarily opportuneistic in-

fections and they represent the third leading cause of in-

vasive fungal infections following Aspergillus and Can-

dida species. The most frequently isolated species is Rhi-

zopus oryzae, followed by Rhizopus microsporeus and

Absidia corymbifera [1] .

Rhino-Orbital Mucormycosis (ROM) is a rare disease

with an overall prevalence in 0.15% of the diabetics.

However, rhino-orbital-cerebral mucormycosis, as a pre-

senting manifestation, is rare [2]. Despite the advances in

the diagnosis and treatment, a high mortality rate of 30 %

- 70% still exists for this disease. Death may occur wi-

thin two weeks in untreated or unsuccessfully treated

patients. It is an acute opportunistic infection which is

caused by a broad, nonseptate, saprophytic fungus which

is found in soil, air, bread mould, rotten fruit and vegeta-

bles.It can be cultured from the mouth, nasal tract, throat

and the faeces of healthy persons. The fungus belongs to

the Phycomycetes class, whose most common genera are

Mucor, Rh izopus, Absidi a and Basid iobolus. Contact

with the micro organism occurs through spore inhalation.

The infection spreads along the vascular and neuronal

structures and it infiltrates the walls of the blood vessels.

It causes erosion of the bony walls of the ethmoid si-

nuses and it may spread into the orbit and the retro-

A. N. Pandey, A. Kakde / Case Reports in Clinical M edicine 2 (2013) 513-516 515

Figure 5. CT scan.

orbital area and in the brain (cerebro-rhino-orbital mu-

cormycosis). Death may occur due to the cerebral ab-

scesses. The infection with this organism usually com-

plicates any underlying chronic disease, as in our patient.

Commonly, mucormycosis attacks people with compro-

mised immune systems. The reduced ability of serum to

bind iron at a low pH may be the basic defect in the

body’s defense systems. The high iron, glucose-rich acid

milieu facilitates fungal growth. Th e human resistance to

fungal infections rests on the body’s ability to restrict the

availability of iron to the invading fungus, by binding it

to proteins such as apotransferrin. The fungal hyphae

produce a substance called rhizoferrin, which binds iron

avidly. This iron-rhizoferrin complex is then tak en up by

the fungus and it becomes available for the vital intra-

cellular processes. Diabetic patients are predisposed to

mucormycosis because of the decreased ability of their

neutrophils to phagocytose and adhere to the endothelial

walls. Furthermore, the acidosis and hyperglycaemia

provide an excellent environment for the fungus to grow

[3]. In this case, the patient was a known diabetic and

later on, he had developed ketosis. Initially, he had pre-

sented with pain in his nose which was there since seven

days, which had progressed to Rhino-Orbital Mucormy-

cosis (ROM) within the next five days. It was followed

by a periorbital swelling and an inability to open the

mouth (facial weakness). The prognosis of mucormyco-

sis has markedly improved over the past 30 years, with a

90% survival rate [2]. The factors which are related to a

poor survival are delays in the diagnosis and treatment.

Mucormycosis, also known as Zygomycosis or Phyco-

mycosis, was first described by Paultauf in 1885, who

coined the term “mycosis mucorina”. In 1943, three

cases of rhinocerebral mucormycosis were reported in

diabetic patients. The most pathogenic species of the

family, Mucoraceae is Rhizopus. Rhizopus oryzae is the

predominant path ogen which accounts for 60% of all the

forms and 90% of the rhinocerebral cases [4]. In this case

also, we isolated the Rhizopus oryzae species. Roden et

al., [5] who reviewed 929 reports of zygomycosis in the

English-language literatu re since 1885, found co-ex isting

diabetes in 36% of the patien ts. Zygomycosis had caused

death in 44% of them. However, Chakrabarti et al., [6]

who analyzed 178 cases of Zygomycosis in hospitalized

patients in northern India, found co-existing, uncon-

trolled diabetes in 73.6% of the patients. Moreover,

Schwartz et al., [7] noticed that Cerebro-Rhino-Orbital

Phycomycosis (CROP) which was mainly caused by

Rhizopus spp., occurred predominantly in the individuals

with diabetic ketoacidosis. CROP leads to proptosis, loss

of vision, ophthalmoplegia and death which results from

a cerebral involvement.

3. CONCLUSION

Mucormycosis remains a severe infectious disease in

diabetic patients and it is characterized by a high mortal-

ity rate. The clinical diagnosis is often difficult and it

gets delayed. It should be underlined that in diabetics,

physicians should always pay special attention to the in-

fections within the facial skeleton, especially which do

not respond to antibiotic therapy. Aggressive diagnostic

procedures are required for histo-microbiological studies

to confirm this disease. An early diagnosis, combined with

medical and surgical treatments, is necessary to improve

the outcome.

4. ACKNOWLEDGEMENTS

The author is thankful to his patient and family. The author also ac-

knowledges the valuable support of di vyam pandey.

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