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ARTICLES |
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Year : 1978 | Volume
: 26
| Issue : 1 | Page : 34-38 |
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Orbito-cranial Aspergillosis
SP Dhir, AK Banerjee, JS Chopra, P Talwar
Dept. of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
Correspondence Address: S P Dhir Dept. of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh India
Source of Support: None, Conflict of Interest: None | Check |
PMID: 101444
How to cite this article: Dhir S P, Banerjee A K, Chopra J S, Talwar P. Orbito-cranial Aspergillosis. Indian J Ophthalmol 1978;26:34-8 |
Infection with aspergillus usually occurs as an opportunistic infection in patients with lowered resistance to infection. Cases have been reported where the resistance to infection has been lowered because of neoplasms, uraemia, chemotherapeutic agents, antibiotics and harmones[5],[8],[11]],[12],[11] However, in most of the cases reported from India [12],[4],[10]. and Sudan[9] no such predisposition has been seen.
This paper presents a fatal case of aspergillosis of the orbit which spread to the cranium and had no overt predisposition to this opportunistic fungus.
Case Report | | |
R.K. 17 years male (C.R. A 0576673) was admitted to Nehru Hospital on 26.7.1975. He was labourer by profession and was in good health 3 months ago. He noted gradual painless forward protrusion of the left eyeball. It has been slow and progressive. There was no history of diminution of vision from either eye.
On general physical as well as systemic examination no abnormality was detected. Pulse was 78 per minute and blood pressure was 120/80 mm Hg.
On local examination, left eyeball was pushed forwards, upwards and outwards [Figure - 1]. Retro-ocular resistance was raised. A deeply situated mass was palpated on the nasal side behind and above the medial palpebral ligament. Visual acuity was 6/6 both eyes.
Pupils were normally reacting and fundi were normal. Ocular movements were full. Ear, nose and throat check up did not reveal any abnormality.
Investigations | | |
Hb. 12.56 per cent ESR 9mm first hour, TLC 12,000/cumm. Polymorphs 76%, Lymphocytes 18%, Eosinophils 6%, Urire normal. Fasting blood sugar 100 mg% Postprandial blood sugar 117 mg% X-ray, Paranasal sinuses and chest were normal.
Biopsy by anterior orbitotomy shoved inflammatory granuloma which with special stains revealed hyphae [Figure - 2] A surgical removal of the mass was attempted. The mass was found plastered to the orbital walls near the apex. Piece meal removal was carried out and amphotericin B drops (1 mg per ml) were instilled locally. Histology of the material was similar to biopsy. On culture of material aspergillus flavus was identified. Five days after removal of mass patient started getting s%ncopal attacks and moderate irregular fever. On neurological examination he had signs of meningitis, but no focal neurological deficit. Patient had a downhill course and the proptosis increased. He developed bilateral ophthalmoplegia and papilloedema. Guarded lumbar puncture revealed raised pressure, 250 cells/ cumm, mostly polymorphs, proteins 140mg%, globulins positive, sugar 37mg% and aspergillus was cultured from the cerebro-spinal fluid. Subsequently, during his long stay in the hospital, CSF was examined on nine occasions and it always showed polymorph leucocytes ranging from 40 to 850 cells/cumm. Proteins ranged between 60-180 mg% and sugar between 43-80 mg% and globulins were always positive. Patient had a waxing and waning course for a further period of two months and developed left knee swelling and thrombophlebitis of leg veins.
Patient was put on Amphotericin B by intravenous and intraventricular routes. He received a total of 2.5 gm of Amphotericin B by both these routes over a period of three months.
There was gross papilloedema with haemorrhages over the disc margin in the right eye. The left optic disc became pale and atrophic and he also developed right hemiparesis. Left carotid angiography showed stretching of anterior cerebral artery and a doubtful mass in the frontoparietal region.
He died of sudden cardio-respiratory arrest on 20.12.1975 after a total course of 8 months since the onset of first complaint of proptosis.
Autopsy Findings | | |
On removal of the brain a roughly globular midline mass measuring 4x3x2 cms. was seen attached to the inferior surfaces of the frotal lobes overing the cribriform plates of the ethmoid does [Figure - 3]. The crista galli had produced a deep midline indentation in the mass. Similar tissue formed a nodule of 1 5 x 1 cm over the optic chiasma. Continuation of this nodule was traced into the orbit through the optic canals, On further exposure the mass was seen occupying considerable portions of both the orbital cavities displacing and incarcerating their contents. The eyeballs were grossly normal. The optic nerves could also be seen, but towards the cranial cavity these were completely embedded in the mass [Figure - 4]. Similar tissue was also detected in the sphenoidal and ethmoidal sinuses. The frontal sinuses were free whereas the maxillary sinuses were not examined, In all these areas the mass possessed similar appearance which was firm and greyish with foci of necrosis.
The brain was oedematosus. The basal cisterns were markedly dilated and there was diffuse leptomeningitis. The exudate in the subarachnoid space was particularly thick and purulent at the base. Slicing showed dilated ventricles with purulent exudate in the cavity. Focal haemorrhages and softening in the right corpus striatum and haemorrhages over the right frontal lobe corresponded with the site of surgical ventricle puncture.
The spinal cord was firmly encased by a thick exudate completely obliterating the subarachnoid space. The dura was also adherent to the cord at several foci. The cauda equina region showed focal nodular thickening around some of the spinal nerves.
The essential microscopic picture was one of chronic granulomatous inflammation [Figure - 5]. The inflammatory exudate consisted of lymphocytes, plasma cells, other mononuclear cell s and large number of multi nucleated giant cells, predominantly of the foreign body type. There was marked but irregular fibrosis and several foci of necrosis. Small and medium sized arteries showed concentric intimal proliferation in some foci, but no real inflammation. With periodic acid schiff and Grocott's methenamine silver methods large number of fungi with septate and branching hyphae were detected. This picture was seen in the grossly visible lesions in both the orbits, one overlying the cribriform plates, the sphenoidal sinuses and subdural space of the base of the cranial cavity and spinal canal. The ependymal surface of the cerebral ventricles, choroid plexus and the leptomeninges of the brain and spinal cord, however, showed the picture of acute pyogenic inflammation. No fungi were detected in these exudates.
Sections of the eyeballs showed features of papilloedema with foci of haemorrhages. [Figure - 6]. Myelin preparations of the optic nerves did not show significant loss of myelin. Section of synovial tissue from the left knee joint showed non specific inflammation. Apart from bronchopneumonia and mild focal chronic non specific, interstitial inflammation in the kidneys no abnormality was detected in the sections of other organs. Fungi were not detected in any other site.
Mycological examination of post mortem brain tissue proved the fungi to be belonging to the group of Aspergillus flavus.
Comments | | |
The present patient was a labourer and had worked with soil in the past. Aspergillus grows on decaying vegetation in soils at certain temperature[5]. Infection is more common in farmers and people who work with soil. He had no predisposition in the form of diabetes, malignancy, radiotherapy or immunosuppressive therapy which is usually seen in deep fungal infections. Aspergillosis of the orbit and paranasal sinuses is also known to occur as a primary infection even in the absence of any general predisposing factors in the host. The reasons for this altered pathogenicity are not known. This case though had no clinical evidence of paranasal sinuses involvement with aspergillus during life but on autopsy sphenoidal sinus and ethmoidal sinuses were found infected. The fungal spores which may be normally present in the nasal, buccal and pharyngeal mucosa may become pathogenic when local conditions are altered. The spores mixed with dust may be inhaled which settle down in the sinuses especially ethmoids, and become pathogenic.
It is unlikely that the patient had cerebral mycosis first which extended to the orbit as no neurological deficit was present before the proptosis. Rarely orbital aspergillosis may arise as a metastatic lesion from pulmonary lesion. The clinical course and histopathological picture of such lesion is quite different from that of local invasion from sinuses or cranium-[5],[8] . Patient had chronic granulomatous inflammatory reaction seen in orbital aspergillosis from local invasion. In pulmonary or metastatic lesions, the Aspergillus fungus grows luxuriantly within necrotic foci of tissue without any, or rarely noted granulomatous change.
The diagnosis in this patient was established after histopathological examination of the orbital biopsy. It is of interest to rote that anti bodies to aspergillus flavus could be demonstrated in the serum of the patient by agar-gel immunodiffusion. Delayed hypersensitivity to aspergillus antigens was also demonstrated by skin test. Thus this patient did not have any immune deficiency disease to account for this fatal fungal infection.
The surgical excision might have led to the spread of infection to the cranium. Five cases have been reported in the literature where cerebral involvement has occurred secondary to a focus in the orbit[6]. Majority of patients with aspergillus orbital infection have fatal outcome. Wolter[13] has reported success in one case treated by surgical excision and injections of amphotericin B. In the present case, the treatment was not successful.
Based on the autopsy findings it seems that the infection by Aspergillus flavus started in the paranasal sinuses involving particularly the sphenoidal and ethmoidal sinuses. There was, however, no clinical or radiological evidence to detect these lesions. From the sinuses the lesion then spread to the right and later, into the left orbital cavities. Intra-cranial extension occurred through the optic canals and the cribriform plates of the ethmoid bone. After gaining access into the cranial subdural space at the base, the lesion progressed downwards along the spinal subdural space. The intracranial pathology was further complicated by development of an acute ventriculitis and leptomengitis most probably as a result of secondary bacterial infection following the surgical ventricle puncture. Throughout his stay in the hospital, repeated CSF examination revealed picture which could be consistent with pyogenic meningitis. However, there was no cytological or biochemical change in the CSF in response to a-.Tribiotic therapy. At autopsy there were findings in the brain consistent with fungal granuloma as well that of a secondary bacterial infection. The nature of arthritis of the left knee joint remains undetermined.
Aspergillus infection of the orbit is not rare in this part of the world. Green et al[8] reviewing the 20 cases of orbital aspergillosis found that 6 of them were reported from India.
Summary | | |
A fatal case of orbital aspergillosis is reported. The infection spread to the cranium following local excision. On autopsy paranasal sinuses were found infected with the fungus despite lack of clinical and radiological evidence anti-mortem. There was no predisposition in the form of diabetes, malignancy, radiotherapy or immunosuppressive therapy[14].
References | | |
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6. | Fetter. B.F., Klinttiork, G.K. and Hendry W.S„ 1967. Published by Williams and Mielkins Company Baltimore, 28. |
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8. | Green, W.R., Font, R.L. and Zimmerman, L.E., 1969 Arch. Ophthal., 82, 302. |
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13. | Wolter, J.R., 1976, Ana. Ophthal., 8, 17, |
14. | Zimmerman, L.E., 1955, Amer. J. Clin. Pathology 25, 46. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]
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