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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 99-101

Treatment of highly fatal extensive childhood mucormycosis with complications: A success story


1 Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
2 Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Date of Submission15-Feb-2021
Date of Decision05-May-2021
Date of Acceptance21-May-2021
Date of Web Publication31-May-2021

Correspondence Address:
Dr. Reena Gulati
Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry - 605 006
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_50_21

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  Abstract 

Background: Mucormycosis is a highly fatal infection that affects immunocompromised individuals. Treatment is difficult and mortality is high when associated with complications. It is rare as a presenting feature of diabetes mellitus (DM) in children. Clinical Description: We describe a child who presented with extensive rhino-orbital invasive mucormycosis and subsequently diagnosed as Type 1 DM. She further developed cavernous sinus thrombosis and internal carotid artery thrombosis known to be associated with very high risk of mortality. Management: Aggressive medical management with posaconazole and liposomal amphotericin B for 12 weeks and early debridement of orbito-cerebral lesions led to complete resolution. Conclusion: Saving children with complicated extensive invasive mucormycosis is possible with aggressive prolonged antifungal therapy and early debridement of lesions.

Keywords: Cavernous sinus thrombosis, diabetes mellitus, fatal infection, internal carotid artery thrombosis, rhino-cerebral-orbital mucormycosis


How to cite this article:
Chidambaram AC, Ramamoorthy JG, Gulati R, Badhe BA. Treatment of highly fatal extensive childhood mucormycosis with complications: A success story. Indian Pediatr Case Rep 2021;1:99-101

How to cite this URL:
Chidambaram AC, Ramamoorthy JG, Gulati R, Badhe BA. Treatment of highly fatal extensive childhood mucormycosis with complications: A success story. Indian Pediatr Case Rep [serial online] 2021 [cited 2021 Sep 26];1:99-101. Available from: http://www.ipcares.org/text.asp?2021/1/2/99/317357

Mucormycosis is recognized to be the second most frequent invasive fungal disease, after aspergillosis.[1] Patients with poorly controlled diabetes mellitus (DM), immunosuppressed patients, and persons who have sustained severe trauma to soft tissue are at increased risk of infection. Despite technological advances and advent of newer antifungal drugs, survival has been approximately 60% over the past two decades.[2] Patients with diabetes tend to develop rhino-cerebral-orbital disease, those with hematological malignancies develop sino-pulmonary disease, and patients sustaining trauma manifest necrotizing skin and soft tissue infections.[2],[3],[4]


  Clinical Description Top


An 8-year-old girl was brought to the emergency with complaints of swelling over the right cheek for 5 days and drooping of right eyelid along with diplopia for 2 days. She had polyuria and easy fatigability for the previous 2 weeks. Intravenous antibiotics were administered elsewhere for 2 days and referred due to rapidly worsening cheek swelling. There was no history of fever, dysuria, vomiting, abdominal pain, blurring of vision, or seizures. The diffuse painless swelling of 3 cm × 3 cm over the right maxilla had no tenderness or erythema. Ophthalmic examination revealed a vision of 6/60, ptosis, complete external ophthalmoplegia, and chemosis. Right pupil reacted sluggishly though fundus appeared normal. The left eye was normal. An ulcer was present over the right hard palate opposite the first molar tooth with a defect that extended into the maxillary sinus. Examination of other systems was normal.

Management and Outcome

Initial blood investigations revealed a very high blood glucose (346 mg/dL). Classical osmotic symptoms with high blood sugar levels confirmed a diagnosis of DM. Hemoglobin A1C (HbA1C) was highly elevated (19.5%), suggesting a long duration of the disease unknown to parents. In the absence of ketoacidosis, subcutaneous insulin therapy was initiated. The constellation of the above examination findings with a background of DM roused the suspicion of mucormycosis, and she was immediately started on liposomal amphotericin-B (lampB) (4 mg/kg/day) and posaconazole (5 mg/kg/day). Computed tomography of brain showed extensive rhino-orbital disease. Microscopy of the slough from oral ulcer showed broad aseptate hyphae suggestive of mucor. Hence, urgent functional endoscopic sinus surgery with debridement was performed, opening up the paranasal sinuses. Postsurgery magnetic resonance imaging (MRI) brain revealed extension of lesion with 2 cm × 2 cm abscess in the right temporal region with thrombosis of cavernous sinus and internal carotid artery [Figure 1]a. The lesion appeared to engulf the orbital apex with subtle enhancement of right optic nerve sheath, imposing a danger of perineural spread. A frontotemporal craniotomy with evacuation of abscess was performed.
Figure 1: (a) Magnetic resonance imaging showing internal carotid artery thrombosis. (b) Magnetic resonance imaging showing cavernous sinus thrombosis and abscess in the temporal region

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Her blood glucose was strictly controlled with subcutaneous insulin throughout hospital stay. Biopsy of the debrided tissue confirmed invasive mucormycosis [Figure 2]a and [Figure 2]b, and antifungals were given for 6 weeks. She developed hypokalemia during the course of lampB therapy which was managed medically. A repeat MRI brain after 6 weeks showed a residual lesion [Figure 1]b, and hence, treatment was continued for 6 more weeks. After 12 weeks of lampB and intermittent posaconazole (availability problems), there was a significant clinical improvement with the resolution of radiological lesions. Antifungals were stopped and the child was discharged on subcutaneous insulin and enoxaparin after 3 months of hospital stay. She is currently well at 6-month follow-up, with 6/6 vision in both eyes, complete resolution of cheek swelling, ptosis, and ophthalmoplegia with no cranial nerve deficits. An obturator was placed to close the palatal defect with recovery of good functional speech at present. Enoxaparin was discontinued after 8 months since there was no progression or worsening of the flow void in internal carotid artery and resolution of the thrombotic lesions. Her HbA1C had dropped to 7.1% at follow-up.
Figure 2: (a) Section shows broad aseptate fungal organisms with right-angled branching suggestive of mucormycosis. (×40 magnification). (b) Gomori methenamine silver stain highlighting the fungal organisms

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  Discussion Top


Our patient had undiagnosed DM type-I at the time of presentation, a known risk factor for mucormycosis. Aggressive, relentless tissue invasion, and infarction secondary to angioinvasion are the hallmarks of mucormycosis.

Rhino-orbital-cerebral mucormycosis (ROCM) is the most common form of mucormycosis in diabetic patients. She was diagnosed as ROCM on a strong clinical suspicion with the presence of facial swelling, pain, change in visual acuity, and ocular movements. The mode of spread from the paranasal sinuses to the orbit occurs through the nasolacrimal duct or through the natural dehiscence in the papyraceous blade. Uncontrolled blood glucose and diabetic ketoacidosis are major risk factors for the development of ROCM. Roden et al. found that 66% of patients with mucormycosis in DM had infection of the paranasal sinuses and 43% had cerebral extension.[3] The European registry of mucormycosis and the French RetroZygo cohort identified that rhinocerebral disease was strongly associated with DM.[4] Pediatric data from India reported 9%–36% of mucormycosis cases among children with diabetes.[5],[6] A prospective multicentric study on mucormycosis reported that 23% of the patients with mucormycosis had a previously undiagnosed DM, presenting for the first time with mucormycosis (ROCM) as was the case with our patient.[5]

Cavernous sinus thrombosis (CST) associated with RCOM is less common. Studies show that CST was reported in 11% of RCOM patients in diabetic adults.[5],[6] However, reports of CST or internal carotid artery thrombosis following RCOM in pediatric patients are very few.[7],[8],[9] The mortality rate of 30%–70% in patients with mucormycosis increases further with this complication.[10] Hence, a high index of suspicion and early aggressive treatment including surgery are paramount for survival. Delayed diagnosis, inadequate surgical debridement, and spread beyond sinonasal cavity contribute to poor prognosis. Amphotericin B and isavuconazole are the mainstays of treatment in mucormycosis. Availability and cost are major limiting factors for adequate treatment in resource-limited countries such as India. Our patient responded well to a total 6 weeks of posaconazole and 12 weeks of lampB therapy with complete radiological clearance from the infected regions and is currently well after 2 years of follow-up.

The positive outcome of our patient reaffirms that high index of suspicion for invasive fungal infections in the setting of DM or immunocompromised patients, aggressive debridement with early initiation, and appropriate duration of antifungal therapy can save lives. Although rare, invasive mucormycosis can be the only presenting feature of DM, missing which can lead to devastating consequences for the patients.



Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

[Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

 
  References Top

1.
Chikley A, Ben-Ami R, Kontoyiannis DP. Mucormycosis of the central nervous system. J Fungi (Basel) 2019;5:59.  Back to cited text no. 1
    
2.
Vaughan C, Bartolo A, Vallabh N, et al. A meta-analysis of survival factors in rhino-orbital-cerebral mucormycosis-has anything changed in the past 20 years? Clin Otolaryngol 2018;43:1454-64.  Back to cited text no. 2
    
3.
Roden MM, Zaoutis TE, Buchanan WL, et al. Epidemiology and outcome of zygomycosis: A review of 929 reported cases. Clin Infect Dis 2005;41:634-53.  Back to cited text no. 3
    
4.
Lanternier F, Dannaoui E, Morizot G, et al. A global analysis of mucormycosis in France: The RetroZygo Study (2005-2007). Clin Infect Dis 2012;54 Suppl 1:S35-43.  Back to cited text no. 4
    
5.
Prakash H, Ghosh AK, Rudramurthy SM, et al. A prospective multicenter study on mucormycosis in India: Epidemiology, diagnosis, and treatment. Med Mycol 2019;57:395-402.  Back to cited text no. 5
    
6.
Chakrabarti A, Chatterjee SS, Das A, et al. Invasive zygomycosis in India: Experience in a tertiary care hospital. Postgrad Med J 2009;85:573-81.  Back to cited text no. 6
    
7.
Haber DM, Fernandes AM, Neto DD, et al. Rhino-orbitocerebral mucormycosis associated with cavernous sinus thrombosis: Case report. Int Arch Otorhinolaryngol 2008;12:574-8.  Back to cited text no. 7
    
8.
Simmons JH, Zeitler PS, Fenton LZ, et al. Rhino cerebral mucormycosis complicated by internal carotid artery thrombosis in a pediatric patient with type 1 diabetes mellitus: A case report and review of the literature. Pediatr Diabetes 2005;6:234-38.  Back to cited text no. 8
    
9.
Gelston CD, Durairaj VD, Simoes EA. Rhino-orbital mucormycosis causing cavernous sinus and internal carotid thrombosis treated with posaconazole. Arch Ophthalmol 2007;125:848-9.  Back to cited text no. 9
    
10.
Shinde RV, Karande GS, Mohite ST, et al. Rhino-orbital mucormycosis in diabetes mellitus. J Clin Diagn Res 2013;7:1145-7.  Back to cited text no. 10
    


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