Case Report · Globe-Preserving Management · Stage 4c

Beyond Vision

A blind eye, saved. How aggressive local control let one orbit escape exenteration in fulminant rhino-orbito-cerebral mucormycosis.

Presenting authorDr. Paridhi Tiwari
Chief authorsDr. Harapriya Sahoo · Dr. Swati Tamaskar
AffiliationDept. of Ophthalmology, SBIMS, Raipur
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A 17-year-old girl.Uncontrolled diabetes, a blackening cheek, three days.By the ICU, the eye had no light left to give.

In India, this is not a rarity. It is an epidemic hiding inside a pandemic of diabetes.
~70×
India's estimated prevalence versus global data, with a modelled burden near 171,000 cases.1
54-76%
of Indian cases arise on a background of diabetes mellitus, the single dominant risk factor.2
~47%
overall mortality in a prospective multicentre Indian cohort, despite treatment.3
The stakes, and the outcome
6/18 → NLP
Vision, presentation to nadir
Stage 4c
Intracranial (CNS) extension9
> 80%
Mortality once mucor reaches the brain15
Survived
Alive, with the globe intact
01 · Presentation

It looked like a furuncle.

A dusky discolouration over the right cheek and lid, treated first as preseptal cellulitis, the classic and costly early misstep. Within days vision collapsed from 6/18 to no light perception, with proptosis, chemosis and a frozen globe.

Fig 1 · Presentation
Clinical photograph at presentation
👁Clinical image · tap to view
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Blackish furuncle over the cheek with periorbital dusky discolouration.
02 · Angioinvasion

Mucorales do not spread. They invade.

Angioinvasion is the hallmark. Broad, aseptate hyphae penetrate vessel walls, thrombose them, and infarct the tissue downstream. The black eschar is not surface debris; it is dead tissue mapping the vessels the fungus has already taken.

Fig 2 · Progression
Extensive rhino-orbital necrosis with black eschar
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Extensive rhino-orbital necrosis with black eschar.
Mechanism

Why diabetes writes the script.

Mucoral spores are inhaled constantly and cleared harmlessly by most hosts. Diabetic ketoacidosis is uniquely permissive, and the reason is now molecular, not merely "impaired immunity."

Diabetic ketoacidosis
↑ glucose  ·  ↑ free iron  ·  ↑ ketone bodies
↑ GRP78 (host receptor) + ↑ CotH3 (fungal invasin)
Endothelial invasion → thrombosis → necrosis
Glucose, iron and ketones together upregulate the endothelial receptor GRP78 and the spore-coat protein CotH3 that binds it, the molecular handshake that lets Mucorales enter and destroy endothelium.5,6,7 Acidosis strips iron from transferrin, and iron is essential for fungal growth, which is why the iron-chelator deferoxamine is a classic risk factor. In diabetic mice, correcting the ketoacidosis with bicarbonate reverses this susceptibility8 - the mechanistic case for aggressive glycaemic and metabolic control at the bedside.
03 · Confirmation & staging

Broad. Aseptate. Wide-angled.

CT of the paranasal sinuses and orbit (Fig 3) showed maxillary sinusitis breaching the inferior orbital wall. Histopathology (Fig 4) was diagnostic: broad, aseptate, wide-angle branching hyphae, the morphology of Mucor.

On the Honavar Code Mucor scale (nasal → sinus → orbit → brain), the disease had reached stage 4c: central nervous system extension, the far end of the Code Mucor scale and the point of highest mortality. In the largest Indian series, exenteration lowered mortality at this stage, which makes a globe-preserving survival here uncommon.4,9

Fig 3 · CT
CT of paranasal sinuses and orbit
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CT (paranasal sinuses and orbit): maxillary sinusitis with inferior orbital wall involvement.
Fig 4 · Histopathology
Histopathology showing aseptate hyphae
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Broad, aseptate hyphae with wide-angle branching, diagnostic of Mucor.
04 · Local control

The knife, aimed at the disease, not the eye.

Aggressive endoscopic sinus and midface debridement to secure local control, with the globe deliberately preserved and the cheek defect covered by a local flap.

Medical therapy followed the guideline spine: amphotericin B with tight glycaemic and metabolic control, escalated to salvage triazoles (posaconazole / isavuconazole) when the early response fell short. ECMM strongly recommends high-dose liposomal amphotericin B (≥5 mg/kg) with early surgery and reversal of the metabolic derangement.10,11

Fig 5 · Intra-operative
Intra-operative debridement
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Intra-operative debridement of necrotic tissue.
05 · Outcome

The disease stopped.
The eye stayed.

Blind and shrunken, now managed with a cosmetic shell. Not vision, but life, and an intact socket, spared the psychological and reconstructive morbidity of exenteration.

Fig 6 · Post-operative
Post-operative appearance
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Healed appearance after debridement and local flap.
Course

The clinical course.

Presentation
Blackish cheek furuncle, lid oedema, congestion for 3 days. BCVA 6/18. Managed as preseptal cellulitis.
Deterioration
Rapid fall to no light perception, with proptosis, ophthalmoplegia and orbital cellulitis.
Diagnosis
Imaging: sinus, orbital and intracranial involvement. Histopathology: aseptate hyphae, Mucor. Stage 4c.
Surgery
Endoscopic sinus and midface debridement for local control; cheek defect covered with a local flap.
Salvage
Amphotericin B and glycaemic control, escalated to salvage triazoles for inadequate early response.
Outcome
Infection controlled, globe preserved (phthisical), referred for cosmetic rehabilitation.
Exenteration is often the default.
Here, local control preserved the eye, even as the disease reached the brain.

In a young patient with a reversible risk factor and disease that responded to therapy, the globe was preserved even as the infection reached the brain, a rare outcome where exenteration is often the default.

The Evidence

Why the eye could stay.

Take-home

Learning points.

1

A black necrotic lesion in a diabetic is an angioinvasive mould until proven otherwise. Antibiotics for "preseptal cellulitis" buy the fungus time.

2

Uncontrolled diabetes and corticosteroid exposure, here from an unqualified practitioner, are the key and largely preventable risk factors.

3

The cornerstones are early high-dose liposomal amphotericin B, aggressive surgical debridement, and reversal of the ketoacidosis, not any one of them alone.

4

Exenteration is not always mandatory: here the globe was preserved even with intracranial disease, though honestly not vision, an uncommon outcome that argues for individualised decisions.

Interpret with care

Limitations.

This is a single case. It is hypothesis-generating and educational; it cannot establish cause and effect, and its outcome may not generalise.

The evidence favouring globe preservation is observational and prone to selection bias, since more severe eyes are exenterated. It shows exenteration has not been proven to improve survival, which is not the same as showing it worsens survival.

Staging rested on CT. Contrast-enhanced MRI is more sensitive for orbital apex, perineural and cavernous-sinus or intracranial spread, and would have strengthened the assessment.

Follow-up is limited. Recurrence remains possible, and longer surveillance is needed before calling this a durable cure.

Rigour

Ethics & reporting standards.

✓  Reported in accordance with the CARE guidelines
Informed consent

Written informed consent for publication of the case and clinical images was obtained from the patient and her legal guardian (a minor).

De-identification

Identifying facial features are redacted and every clinical image sits behind a deliberate reveal, applied alongside consent, not instead of it.

Ethical guidelines

Prepared in keeping with the ICMR National Ethical Guidelines (2017) for the reporting of clinical case material and for informed consent.

Authorship & integrity

Authorship meets ICMJE criteria and all authors approved the final version. All clinical details and images pertain to the index patient, with no fabrication.

Patient perspective

The family's documented priority throughout was to avoid losing the eye; the globe-preserving plan aligned with that wish and defensible clinical grounds.

Conflicts & funding

No conflicts of interest are declared and no funding was received.

References

The evidence base, connected.

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