Table of Contents

Mucormycosis

Mucormycosis is an invasive infection caused by filamentous fungi belonging to the order Mucorales, also known as phycomycosis or zygomycosis, is caused by common Zygomycete fungi frequently found in soil and decaying vegetation.

Its pathogenic form was recognized in 1815 1) , with the first case of mucormycosis in humans being described by Palauf in 1855 2) 3). The first documented case of chronic mucormycosis was described by Vignale in 1964 4).

Epidemiology

These mainly infect immunocompromised patients and cause an acute fulminating fungal disease.

It also has been described in immunocompetent patients with an incidence of 4–19% 5) 6) 7) 8).

Etiology

The genera most commonly responsible for mucormycosis are Mucor, Rhizopus and Asbidia 9) 10) 11) 12) 13) 14).


Usually occurs in diabetics


A study aimed to explore the epidemiologic threats and factors associated with the coronavirus disease 2019 (COVID-19)-associated mucormycosis (CAM) epidemic that emerged in Egypt during the second COVID-19 wave. The study also aimed to explore the diagnostic features and the role of surgical interventions of CAM on the outcome of the disease in a central referral hospital.

The study included 64 CAM patients from a referral hospital for CAM and a similar number of matched controls from COVID-19 patients who did not develop CAM.

The most frequent factors among CAM patients were the use of corticosteroids, older age, and diabetes. CAM patients presented with facial pain (98.4%), black coloring on nasal endoscopy examination (87.5%), orbital invasion (70.3%), and loss of vision (68.8%). Despite treatment, CAM led to the death of 30 patients and 34 patients survived until the end of the study. CAM patients with death outcomes had orbital invasion, disturbed consciousness level, referral to intensive care units, and invasive mechanical ventilation. The patients who survived received more surgical interventions than dead patients, including functional endoscopic sinus surgery (FESS) and maxillofacial surgery.

CAM treatment requires complex, time-consuming, and expensive diagnostic approaches. Therefore, preventative measures should focus on early source control, strict glycemic control, and limiting steroids to COVID-19 patients especially older patients (> 40 years). Early antifungal treatment and surgical techniques such as FESS and necrotic tissue debridement were associated with better prognosis, indicating the efficiency of multidisciplinary medical and surgical teams 15).

Types

According to time of evolution the infection can be acute or chronic, with the latter having a low frequency (5.6% of rhinocerebral mucormycosis cases) 16) 17).

Of the different types of mucormycosis, the rhinocerebral type is the most severe one, and its type 2 subtype, the rhino-orbital-cerebral form is the deadliest variety.

Rhinocerebral mucormycosis

see Rhino-Orbital-Cerebral Mucormycosis

Physiopathology

Can result secondary to rhinocerebral or hematogenous spread.

Treatment

Amphotericin B, posaconazole, and aggressive surgical resection are the hallmarks of treatment. While amphotericin is typically administered intravenously, less is known about the use of intrathecal amphotericin B 18).

Outcome

It is associated with a high mortality.

Postoperative complications

On postoperative day 5, a patient developed right hemiplegia, and brain imaging revealed left internal carotid vasospasm 19).

Case series

34 case records of patients with a histopathological diagnosis of ROC mucormycosis treated between 1992 and 2000 were reviewed. Three clinical stages and three treatment groups were identified. Patients with limited sino-nasal disease (Clinical stage I) underwent sino-nasal debridement (Treatment group A). Patients with limited rhino-orbital disease (Clinical stage II) underwent either sino-nasal debridement alone (Treatment group A) or orbital exenteration in addition to sino-nasal debridement (Treatment group B). Patients with rhino-orbito-cerebral disease (Clinical stage III) did not undergo any surgical procedure (Treatment group C). Thirty-three patients received intravenous amphotericin B. Outcome for each group was measured as “Treatment success” (disease free, stable patient with metabolic abnormality under control) and “Treatment failure” (progression of disease with worsening general condition or mortality due to the disease).

Uncontrolled diabetes in 30 (88.2%) of 34 patients was the commonest underlying disease and 16 (53.3%) of 30 diabetics had ketoacidosis. Chronic renal failure (n = 4), hepatic disease (n = 3) and idiopathic thrombocytopenia (n = 1) were the other underlying diseases. Eleven patients had stage I disease, 16 patients had stage II disease and seven patients had stage III disease. All 11 patients with stage I disease received treatment A; of 16 patients with stage II disease, 7 received treatment A and the remaining with stage III disease received treatment B; 7 patients with stage II disease received treatment C. Ten of 11 patients (91%) with stage I disease had treatment success. In patients with stage II disease, 7 of 7 (100%) with treatment A and 1 of 9 (11.1%) with treatment B had treatment success. All seven patients with stage III disease had treatment failure.

Debridement of the sinuses is necessary in all cases of rhino-orbito-cerebral mucormycosis. Diagnosis in the early stage needs a high degree of suspicion. There is a definite role for retention of orbits in patients whose metabolic derangement is rapidly controlled and orbital involvement is non-progressive 20).

1)
Handa K.K., Handa A., Panda N., Mann S.B.S. Primary chronic mucormicosis. Indian J. Otolaryngol. Head. Neck Surg. July-Sept. 1996;Vol 48(3):232–234.
2)
Ferstenfeld J.E., Rose H.D., Cohen S.H., Rytel M.W. Chronic rhinocerebral phycomycosis in association with diabetes. Postgrad. Med. J. June 1977;53:337–342.
3)
Finn D.G., Farmer J.C. Chronic mucormicosis. Laryngoscope. July 1982;92:761–763.
4) , 17)
Harrill W.C., Stewart M.G., Lee A.G., Cernoch P. Chronic Rhinocerebral Mucormycosis. Laryngoscope. 1996;106:1292–1297.
5)
Hemashettar B.M., Patil R.N., O'Donnell K., Chaturvedi V., Ren P., Padhye A.A. Chronic rhinofacial mucormicosis caused by Mucor irregularis (Rhizomucor variabilis) in India. J. Clin. Microbiol. June 2011:2372–2375.
6)
Scharf J.L., Soliman A.M.S. Chronic Rhizopus invasive fungal Rhinosinusitis in an immunocompetent host. Laryngoscope. 2004;114:1533–1535.
7)
Angali R.K., Jeshtadi A., Namala V.A., Gannepalli A. Fatal rhino-orbito-cerebral mucormicosis in a healthy individual. J. Oral Maxillofac. Pathol. 2014 Sep-Dec;18(3):460–463.
8)
Dusart A., Duprez T., Van Snick S., Godfraind C., Sindic C. Fatal rhinocerebral mucormicosis with intracavernous carotid aneurysm and thrombosis: a late complication of transsphenoidal surgery? Acta Neurol. Belg. 2013;113:179–184.
9)
Jindal S, Kulkarni S, Sheikh S, et al. Invasive rhinomaxillary mucormycosis: a case report with a review of the literature. Oral Radiol 2008;24:42–8
10)
Cagatay AA, Oncu SS, Calangu SS, et al. Rhinocerebral mucormycosis treated with 32-gram liposomal amphotericin B and incomplete surgery: a case report. BMC Infect Dis 2001;1:22.
11)
Pastore PN. Mucormycosis of the maxillary sinus and diabetes mellitus: report of a case with recovery. South Med J 1967;60:1164–7
12)
Rumboldt Z, Castillo M. Indolent intracranial mucormycosis: case report. AJNR Am J Neuroradiol 2002;23:932–4
13)
Haliloglu NU, Yesilirmak Z, Erden A, et al. Rhino-orbito-cerebral mucormycosis: report of two cases and review of the literature. Dentomaxillofac Radiol 2008;37:161–6
14)
Mane R, Watve J, Mohite A, et al. Rhinocerebral mucormycosis: a deadly disease on the rise. Indian J Otolaryngol Head Neck Surg 2007;59:112–15
15)
Elserougy S, Abdel-Ghaffar M, Medhat E, Heiba A, Shaheen W, Mostafa E, Mahfouz H, Abdel-Hafez S, Mahfouz MS, Afify S, Ali O, El-Sayed E, Mostafa AM, Salah AM, Elbatawy A, Elghoneimy HM, Elshafey AA, Abou-ElFotouh A, Abdul-Ghani T, Ibrahim FI, Samy SM, Elhussini MS. Epidemiologic threats and outcome of evolving COVID-19-associated mucormycosis from a referral hospital in Egypt. J Infect Dev Ctries. 2024 Dec 31;18(12.1):S369-S380. doi: 10.3855/jidc.19897. PMID: 39863956.
16)
Dimaka K., Mallis A., Naxakis S.S., Maragos M., Papadas T.A., Stathas T., Mastronikolis N.S. Chronic rhinocerebral mucormycosis: a rare case report and review of the literature. Mycoses. 2014;57:699–702.
18)
Grannan BL, Yanamadala V, Venteicher AS, Walcott BP, Barr JC. Use of external ventriculostomy and intrathecal anti-fungal treatment in cerebral mucormycotic abscess. J Clin Neurosci. 2014 May 19. pii: S0967-5868(14)00167-2. doi: 10.1016/j.jocn.2014.01.008. [Epub ahead of print] PubMed PMID: 24852901.
19)
Abuzayed B, Al-Abadi H, Al-Otti S, Baniyaseen K, Al-Sharki Y. Neuronavigation-Guided Endoscopic Endonasal Resection of Extensive Skull Base Mucormycosis Complicated With Cerebral Vasospasm. J Craniofac Surg. 2014 Jun 4. [Epub ahead of print] PubMed PMID: 24902115.
20)
Nithyanandam S, Jacob MS, Battu RR, Thomas RK, Correa MA, D'Souza O. Rhino-orbito-cerebral mucormycosis. A retrospective analysis of clinical features and treatment outcomes. Indian J Ophthalmol. 2003 Sep;51(3):231-6. PubMed PMID: 14601848.