Mucormycosis is an angio invasive disease that is characterized by tissue infarction and necrosis [1]. The data indicates that the estimated prevalence of mucormycosis in India is nearly 70 times higher than the global data, which were estimated to be at 0.02 to 9.5 cases(with a median of 0.2 cases) per 100,000 persons [2]. Mucor infection is associated with impaired immune status as person with diabetes, cancer, organ transplant and who are taking steroid for long time are at higher risk for developing mucormycosis infection. Classification of the mucormycosis infections depend on the area involvement in the body like pulmonary mucormycosis,cutaneous mucormycosis,gastrointestinal mucormycosis and rhino-orbital-cerebral mucormycosis (ROCM).ROCM is a rare invasive fungal infection of the nasal and maxillary sinuses and the orbit. The causing agent, Mucorales, from the family of mucormycetes fungi, includes more than 300 species and may be responsible for opportunistic infections, especially in immunosuppressed patients [3]. Particular attention to the ROCM was brought about following the first and second epidemic COVID-19 waves in India and other countries [4,5], where mucormycosis was mediatised by several world-renowned media [6].It remains one of the most difficult diseases to manage, with a high rate of morbidity and mortality. There are limited research and experiments regarding the surgical management of rhino-orbital-cerebral mucormycosis. Debridement of mucormycosis fungal infections lacking vast knowledge gap about which approach is best, which investigation most useful and when to operate patients. Recently rising in mucormycosis incidence gives opportunity to compare the data regarding outcome , approach and useful investigation.

This is retrospective observational study to be conducted at GG Hospital Jamnagar, from April 2021 to October 2022. The patients admitted in the mucormycosis ward are included in the study. 279 patients are included in the study. The data is obtained from the hospital records of the patients and regular follow up by patients. Inclusion Criteria: Patients admitted in mucormycosis ward in G.G. Hospital, Jamnagar Exclusion criteria: Patients who are not willing for participation, unwilling for admission or referred to higher center without admission. All patients investigated and throughly examined for the clinical symptoms. Pre operative KOH culture sent for fungal examination. Nasal endoscopy, CT SCAN and MRI were done in every patient to know the area of involvement. Holistic approach by ophthalmology, dental and ENT department taken according to area of involvement. Regular follow up of the patients taken to know complications and disease clearance. All patients gone under the medical treatment after operation for injectable anti fungal amphotericin-B.

Results were obtained with all clinical examinations whenever patients admitted in our department. All patients examined post operative on follow up within their next 1 week, 3 week , 2 month, 6 month and 1 year visit to the hospital. 1.Clinical features: The common complaints were facial pain and tenderness, visual impairment and loosening of teeth. Fever was also a common complain, although it can also be associated with COVID 19. 2. Investigations: Potassium hydroxide (KOH) mount was positive in only 112 of all the patients, and histopathology in 172 patients. 3. Area of involvement: Nose and paranasal sinuses were most commonly involved, followed by palate, CNS and then orbit, based on the radiological findings. 4. Radiological investigations: 5.Management: 6. Surgical management:

The meta-analysis by Vaughan C et al7 and Yohai R.A. et al8 found fever and nasal discharge to be the most common non ophthalmic feature, and ophthalmoplegia and impaired vision the most common ophthalmic features. Hence, Nasal discharge and fever were the most common clinical features, however both of these are fairly non -specific, and are seen in only 50-60% of the patients. There are no pathognomic features of mucormycosis, and the disease has a rapid progression of the disease, make it imperative to investigate all the suspicious cases rigorously. That being said, necrosis on nasal endoscopy is diagnostic of mucormycosis, although it is not present in early stages and in all patients. Full spectrum of orbital, palatal and CNS symptoms are encountered, depending upon the area of involvement. Diplopia, ptosis, chemosis, ophthalmoplegia, visual impairment or visual loss, proptosis or frank orbital cellulitis can be present. Many patients present with severe inflammatory features but near normal vision and paradoxically patients with no or minimal orbital inflammation have complete loss of vision. This is because of different modes of orbital involvement. In patients with direct spread of mucor to the orbit, the inflammatory features are predominant, whereas in those who have orbital apex involvement the visual impairment is more pronounced and an early feature. Irritability and drowsiness are the earliest features of intracranial involvement. Altered sensorium, persistent headache, nausea and vomiting, neck rigidity, photophobia and diplopia are indicative of developing meningitis. Brain abscesses have additional focal neurological deficits depending on the area of the brain involved. Cavernous sinus involvement can cause ophthalmoplegia. ICA thrombosis can spread further along the circle of Willis which leads to neurological deficits or even hemiplegia. Patients with orbital involvement have higher probability of progressing to intracranial involvement. Loosening of the teeth of upper alveolus, palatal ulcer, gum swelling, oro-antral fistula is present. Patients sometimes present with fallen teeth and associated oro-antral fistula. In other cases, there is development on palatal ulcers, these are non-healing and non-tender, the mucosa becomes de-vascularized and is eventually develops necrosis. The Kolekar9 study also used direct microscopy, histopathology and culture to establish the diagnosis.The low sensitivity of KOH mount, is mainly due to factors related to handling of the specimen. It was found in our study that, it is ideal to take multiple endoscopy guided biopsy from suspicious areas, and these are to be sent separately in normal saline for KOH wet mount and 10% formalin for a formal histopathological examination. Improper sampling techniques, sampling from improper sites, incorrect transport media, and a long transit time are some of the factors for false negative results. MRI was the radiological investigation of choice in our institute, however ideally it should be performed in conjugation with a CT scan. Computed Tomography: CT scan is useful for diagnosing the bony involvement and erosions. The sinuses may show features of sinusitis and mucosal thickening. It is not useful in picking up early soft tissue changes, and usually under diagnoses the extent of the disease. Magnetic resonance imaging: This remains the investigation of choice for soft tissue involvement. The characteristic black turbinate sign in the lack of enhancement of the necrotic turbinate following gadolinium contrast administration. Other features include enhancement of the areas with inflammatory changes indicative of early stages of involvement. MRI is superior to the CT scan in correctly identifying the extent of the disease. Fat suppressed images are especially useful for orbital images, as this helps to differentiate between the peri-orbital fat and inflammation. The useful sequences are T2 weighted gadolinium contrast images. The areas of hypo-enhancement, imply necrosis, whereas hyper-enhancement is seen in cases with inflammation. This helps to differentiate sinusitis from mucor, as in sinusitis there is enhancement of the mucosa lining the sinuses, which is almost always absent in mucormycosis. Another useful feature is in cases of sudden visual loss, without any other orbital features. Diffusion weighted images can detect early ischemic changes in the optic nerve, even before the onset of blindness. MR angiography is useful for detecting thrombosis of the supplying artery. Fatterpekar G et al10 found MRI to be more sensitive than CT especially for orbital extension. Herrera et al11 also found MRI, particularly contrast enhanced T1 images were useful in identifying meningeal and cavernous sinus involvement. They also advocated using multimodality imaging techniques for adequate assessment of the disease.Surgical debridement should be undertaken as early as possible, and it is imperative to have a recent MRI of the patient. Without the MRI there is a high probability of leaving some disease behind, as it has been observed that the infection spreads even beyond intact bony margins. This is especially true for the retro-maxillary and peri-orbital region. Interdepartmental co-ordination is required in cases with alveolar and orbital involvement. It was however found that most of the cases with early intra-cranial did not require neurosurgical intervention. Modified Denker’s approach was employed in most of the cases, with satisfactory results. Tissot et al12 and Cornely OA et al13 have noted that surgical debridement of the necrotic tissue is the keystone for treating mucormycosis.Radical surgery has been noted to provide better local control (90%) as compared to limited surgery (41.6%), as seen by Vironneau et al. Nithyanandam S et al15 reported that early surgical debridement of the affected sinuses and the retro-orbital space, often prevents the infection from extending into the eye, and hence nullifies the need for enucleation and results in extremely high cure rates (>85%).

In our study , we found that early diagnosis was extremely important in mucormycosis fungal infections, whenever patients present with facial numbness, pain with loosing of teeth, nasal discharge than go for suspicious of mucormycosis without any delay. Histopathology and KOH are to be done for all patients whenever there is nasal lesions. However, the are not awaited before starting the treatment in clinically evident cases. MRI is the imaging modality of choice for the assessing the extent of disease and for intra-orbital and intra-cranial extension. CT scan is useful for analyzing the bony anatomy of the paranasal sinuses. Early treatment with antifungal agents and early surgical debridement proved to be helpful in improving the prognosis of the patients. Modified Denker’s procedure has proved to be very well suited for patients with mucormycosis, as it is an endoscopic procedure but provides very good exposure and clearance of diseased areas, as well as for post operative surveillance. A slightly increased mortality in patients undergoing medical line of management only, without surgical debridement. Hence early and aggressive debridement has been advised even in complicated cases and cases with intracranial extension.

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