The "black evil" affecting patients with diabetes: a case of rhino orbito cerebral mucormycosis causing Garcin syndrome

Abstract

Mucormycosis is a life-threatening infection affecting patients with diabetes. It is an angioinvasive disease often resistant to treatment with a debilitating course and high mortality. Here, we report a case of a 45 year old woman with type 2 diabetes mellitus who presented to us with history of right-sided ptosis and facial palsy, and subsequently developed loss of vision and palatal palsy. She was in diabetic ketoacidosis. Nervous system examination revealed involvement of right second, third, fourth, sixth, seventh, ninth, and tenth cranial nerves, suggestive of Garcin syndrome. The hard palate had been eroded with formation of black eschar. Computed tomography of paranasal sinuses revealed right maxillary and ethmoid sinusitis, with spread of inflammation to infratemporal fossa and parapharynygeal neck spaces. Debridement of sinus mucosa was done, and culture of the same yielded growth of rhizopus species. Histopathological examination of the tissue showed angioinvasion and fungal hyphae suggestive of mucormycosis. She was treated with amphotericin B, posaconazole, and periodic nasal sinus debridement, but her general condition worsened after 8 weeks due to secondary sepsis and she succumbed to death.

Epidemiology, clinical features, diagnosis and treatment of cerebral mucormycosis in diabetic patients: A systematic review of case reports and case series

“…Interestingly, using posaconazole as a treatment option in eight cases led to the infection control (Table 1). Noteworthy, in the case of the two patients whose treatment failed after using posaconazole and died afterwards, one was subject to sepsis while posaconazole had a good inhibitory effect on CM control 17 . However, another patient died despite the use of posaconazole, which could be related to the occurrence of posaconazole‐resistant mucormycosis or under‐dosing of this antibiotic as clinicians did not perform therapeutic drug monitoring 29 .…”

“…Furthermore, CM in two patients with T2D caused Garcin syndrome, which is usually induced by skull base, rhino pharyngeal tumours or metastases, and basal meningitis. Thus, clinicians are advised to consider the significance of CM in patients with Garcin syndrome, and for prompt diagnosis, a high index of clinical suspicion is required 17,39,52 . As mentioned earlier, common symptoms of CM in patients with diabetes include non‐specific symptoms such as facial swelling, headache and facial pain, which make it difficult to differentiate this infection from other diseases.…”

“…The use of posaconazole in combination with AMB and LAMB has positive effects on controlling CM in diabetic patients and even in patients that experience no improvement after 3 weeks of antifungal monotherapy, the combined use of posaconazole and LAMB facilitates treatment 17,28,43,64 . Moreover, in patients with AMB‐resistant infections or after‐treatment failure with this antibiotic, which is used as the first line of CM treatment in diabetic patients, posaconazole can be a good alternative treatment 24,30,45 .…”

“…Finally, although treatment can sometimes control CM in patients, bacterial infections can cause death due to the patient's physiological condition, multiple surgeries, metabolic disorders caused by diabetes and the long hospital stay 17,67 . Therefore, the survivors of mucormycosis are high‐risk patients who are highly susceptible to disease recurrence and exhibit predisposition to more conventional bacterial infection and sepsis by the insertion of a foreign body; therefore, it is recommended that one consult an infectious disease expert from the outset of the treatment process.…”

“…Fungal spores colonise the paranasal sinus upon inhalation, and orbital involvement usually occurs because of the spread of the disease from (a) paranasal sinuses through the lamina papyracea, (b) the maxillary sinus roof and (c) direct traumatic penetration into the orbital compartment. Orbital involvement can result in periorbital swelling and oedema, orbital cellulitis, chemosis or orbital apex syndrome with the involvement of the third, fourth and sixth cranial nerves 17 . Infection can reach the brain in three ways: (a) from frontal, ethmoid and sphenoid sinuses directly into the cranial cavity, (b) from the retro‐orbital region into ethmoid and sphenoid sinuses or (c) from the superior orbital fissure by perineural route or cribriform plate.…”