Chronic invasive fungal rhinosinusitis is relatively rare. At MCV we see 2-3 cases of non invasive fungal sinusitis in a week and 2-3 cases of invasive fungal disease in 6 months. It is a severe and potentially fatal infection. This is also known as chronic fungal sinusitis, mycetoma and aspergilloma and typically affects people whose immune system is weakened by disease, such as uncontrolled diabetes or renal transplants. Mucormycosis (phycomycosis) is infection caused by a fungus of the group Mucorales. Depending on environmental and host factors, especially genetic tendencies toward IgE-mediated allergy, a mixed picture of non invasive and invasive disease can be present in a given individual. It most commonly affects the nose and brain (rhinocerebral muscormycosis), other common site of infection is the lung, and arely the skin and digestive system are involved.

Histopathologically there should be evidence of subepithelial tissue invasion that may include the underlying bone and vessels. The vessel involvement can lead to the acute tissue necrosis. The allergic mucin is typically absent. It may be associated with a granulo-matous picture on histopathology, it may be very difficult to identify unless the index of suspicion is very high.

Clinical presentation:

This patient seen in figure 1 with diabetes mellitus presented with facial edema ,burning sensation over the face ,loss of vision in the left eye, foul smelling nasal discharge, multiple cranial nerve palsy (left Optic nerve, Bilateral Occulomotor, trochlear, Abduscent and facial nerve palsy). He developed all these problems following left FESS surgery done at a different centre. On examination multiple nodular swellings were noted on the face predominantly on the forehead, swelling of the right jaw, bilateral periorbital edema and gross odema of upperlip. On examination of Oral cavity an ulcerative lesion found in the hard palate with exposed necrotic bone. Diagnostic nasal endoscopy revealed crusts in the middle meatus and right inferior turbinate was not seen. Left middle meatus showed polyps with AFS debris. This is our 2nd case with a picture of combined AFS and invasive mycosis. This presentation extremely rare. To our knowledge Dr. Prabodh Karnic, Mumbai had a similar patient. Litrature ref is not available.

Investigations:
Histopathology-From mandibular lesion showed Mucormycosis.From para nasal sinuses repeated biopsies did not show invasive fungus.

CT PNS showed post op changes in the operated sinus cavity with opacification of the right sinuses, focal bony defect noted in the superio lateral wall of the left sphenoid sinus, erosion of alveolar process of left maxilla

CT Neck lytic lesion in the ramus & body of right mandible.

MRI of Head & Neck bilateral cavernous sinus thrombosis with right internal carotid thrombosis , soft mass seen in the left infra orbital region extending to the orbital apex, diffuse subcutaneous abscess pockets in the face, cheek, perioral, periorbital region.

Carotid Doppler study showed right internal carotid thrombosis, critical narrowing with <80% open at the origin.

Treatment :
For a disease with high mortality rate an aggressive approach with radical surgery is the option. Does a conservative approach have a role?

Important principle in treatment of the fungal disease is it can be spread by improper tissue handling. Owing in part to their vascularity, the periorbita and dura appear to be substantial barriers to spread, while bone is not. Thus, it is preferred to leave these tissues intact unless they are obviously involved. Violation of these barriers may promote introduction of the invasive fungus into the orbit or cranium. Though we suggested patient refused to undergo exenteration of one blind eye and hence he was put on a conservative line.

In our institute the patient underwent a Sequestrectomy & curettage of the mandibular lesion under GA, requiring a prophylactic tracheostomy to avoid intubation to prevent any embolisation from the involved neck vessels. Histopathology from the mandibular lesion showed MUCORMYCOSIS. Subsequently he under went a Left Baywatch with optic nerve decompression and clearance of the soft tissue mass from the orbit however HPE showed nonspecific inflammation. A month later he underwent a debridement of the necrotic hard palate and clearance of the sinuses. post operatively endoscopic cleaning of the sinus cavity and topical application of gel foam soaked Amphotericin B was done twice a day .he was also started on parentral Amphotericin B (conventional ) and received a total of 3 gm in two sittings. There was a persistence of debris and polyps in the left frontal sinus region hence a Lynch Howarth frontospheno ethmoidectomy was done with clearance of the sinuses followed by Amphotericin nasal washes twice a day. However he developed a CSF leak from the posterior wall of left frontal sinus which was managed conservatively. Post operatively the facial swelling reduced, multiple nodular swelling on the face disappeared, minimal swelling of the right jaw is persistent (fig. 2). His nasal endoscopy shows absence of disease and healing cavity (fig. 3).

This driver survived an ordeal of a dreaded disease. He was an inpatient for 60 days and treatment was provided free by the MCV trust hospital and monetary assistance from Dr. Ajay MDS and his friends from Coimbatore.