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Pediatric Oncall Journal

Successful Outcome of Rhino-Orbital Mucormycosis in a Case of Type I Diabetes Mellitus 01/09/2014 00:00:00

Successful Outcome of Rhino-Orbital Mucormycosis in a Case of Type I Diabetes Mellitus

MR Wade1, AR Jadhav1, RA Bradoo2, MV Manglani1.
1Department of Pediatrics, Lokmanya Tilak Municipal Medical College & General Hospital, Sion, Mumbai 400 022, India,
2Department of ENT, Lokmanya Tilak Municipal Medical College & General Hospital, Sion, Mumbai 400 022, India.

Mamta Manglani, A - 202, Casaurina, Evershine Green Apartment, New link road, Andheri (West), Mumbai 400 102.
Mucormycosis is a rare and life threatening fungal infection in children that occurs in association with immunocompromised states. Very few case reports of rhino-orbital mucormycosis have been documented in pediatric age group and most of these have been fatal. We report a case of mucormycosis in a child with type I Diabetes Mellitus who presented with sero-sanginous nasal discharge along with orbital cellulitis. Nasal mucosal biopsy revealed aseptate hyphae diagnostic of mucormycosis. The CT showed involvement of ethmoid and maxillary sinuses along with retro-orbital extension, sparing cerebral involvement. We managed the child with lipid complex amphotericin B coupled with repeated surgical debridement. On follow up at six months, the child was disease free.

Key Messages:

  1. Mucormycosis is a rare but an acute and frequently fatal infection in young patients with type 1 diabetes mellitus.

  2. High index of suspicion and combined approach with amphotericin B and appropriate surgery is rewarding in management of mucormycosis.

Mucormycosis, Rhino-Orbital, Type I Diabetes Mellitus
Mucormycosis is caused by Phycomycete ferrophilic fungi from the Mucoraceae family, genera Mucor or Rhizopus. Rhino cerebral mucormycosis (RM) is a rare, rapidly progressive opportunistic infection. 1 The organism is ubiquitous, and people with immune disorders are more susceptible to the infection with this organism. One population based study estimated the incidence of mucormycosis as 1.7 per million people per year. In high risk patients, undergoing allogenic bone marrow transplantation, prevalence in different countries is 1-3 %. There have been only 2 cases of rhino-orbital mucormycosis reported in Indian literature to the best of our knowledge 2 . The disease was called mucormycosis by Paltauf who described the first case in 1885.

We report a case of rhino-orbital mucormycosis in a pediatric patient, who had a successful recovery.
Case Report
KB, an 11-year-old girl was diagnosed to have type 1 diabetes mellitus when she presented with ketoacidotic coma 14 days prior to admission to our institute. She presented with reddish-black discharge from the nostril and swelling over the left eye over 6-7 days. There was orbital swelling without chemosis or restriction of movement. There was no headache, vomiting or focal neurological deficit. The biopsy from the nasal mucosa revealed the presence of aseptate hyphae with right-angled branching, which are typical of mucormycosis. Imaging studies of orbit, paranasal sinuses and brain was done to determine the extent of mucormycosis. It showed involvement of ethmoid and maxillary sinuses along with retro-orbital extension. There was no cerebral involvement. Immunocompromised status was ruled out by doing HIV - ELISA, which was negative. Sinoscopy showed findings suggestive of involvement of nasal mucosa with debris of blackish discharge bilaterally.

Along with stabilization of diabetes, patient was started on Lipid Complex Amphotericin-B. Additionally, debridement using sinoscopy was done every 2 weeks by our ENT colleagues. The sample thus obtained was sent for histopathology and it confirmed the diagnosis of mucormycosis. The debridement done again after 2 weeks was negative for mucor. Regular surgical debridement was done along with Lipid Complex Amphotericin B- 5mg/kg/day for 6 weeks. Check scopy done at the end of 6 weeks and 3 months was normal. Child is doing well, has no residual visual impairment and is presently well controlled for her diabetes too. Her survival is attributed to early recognition of mucormycosis with diagnostic support of imaging studies, surgical debridement and antifungal therapy, along with intensive blood glucose control.
Conditions most commonly associated with mucormycosis include uncontrolled diabetes mellitus, chronic steroid use, metabolic acidosis, organ transplantation, leukemia/lymphoma, treatment with desferoxamine, and AIDS 1. Rhinocerebral disease represents one-third to one-half of all cases of zygomycosis. Rhinocerebral mucormycosis usually is acquired by nasal inhalation of spores, followed by direct hyphal invasion of vessel walls. Subsequent hemorrhage, thrombosis, tissue necrosis, and propagation of a hypoxic and acidotic environment further facilitate fungal growth and inhibit effective systemic therapy. As a result, ROCM often is associated with sinusitis, orbital cellulitis, orbital apex syndrome, cavernous sinus syndrome, and internal carotid artery thrombosis. These last two syndromes have a much more ominous prognosis than the others 3,4 With extensive central nervous system involvement, this disease is uniformly fatal within weeks. The detection of aseptate hyphae with right-angled branching is pathognomonic of mucormycosis 2,5.

The prognosis of the disease surely depends on how early and aggressively it is managed. Factors associated with poor survival in ROCM include delay in diagnosis and treatment, hemiparesis, bilateral sinus involvement and facial necrosis. 4,6 Medical management alone is not effective because of poor drug delivery to the site of due to extensive vascular thrombosis. This rare disorder can be treated successfully without radical resection, particularly if multimodality treatment options are implemented.

The possible complications of ROCM, includes loss of neurological function. There may be thrombotic complications involving cerebral or pulmonary vessels. Mucormycosis has an extremely high mortality rate even with aggressive surgical intervention. Death rates range from 25-80% depending on the site involved as well as the underlying immune problems. 2,4

The fungi that cause mucormycosis are widespread, the most appropriate preventive measures involve improved control of the underlying illnesses associated with the infection. A high level of suspicion for zygomycosis in patients who are at risk can aid an early diagnosis and implementation of appropriate therapy. 4

RO (Rhino-orbital), ROCM (Rhino-orbitocerebral mucormycosis), C.T. scan (Computerized Tomography), HIV (Human Immunodeficiency Virus), ENT (Ear Nose Throat department).
Authors Contribution
MW collected data, compiled and drafted the manuscript. AJ helped in drafting the manuscript. MM, AJ and RB were involved in patient care. MM finalized the draft.
Compliance with Ethical Standards
Funding None
Conflict of Interest None
  1. John F. Hamilton, Henry B. Bartkowski, Jack P. Rock. Management of CNS Mucormycosis in the Pediatric Patient Pediatric Neurosurgery 2003;38:212-215.  [CrossRef]  [PubMed]
  2. Bhadada,. Bhansali, A, Reddy, K.S.S, Rekha V. Bhat, N. Khandelwal,A. K. Gupta. Rhino-orbital-cerebral mucormycosis in Type I Diabetes Mellitus, Indian J.Pediatr, 2005; 72(8):671 - 674.  [CrossRef]  [PubMed]
  3. Gonis, Gena B.Sc. Hons.; Starr, Michael M.B. Fatal rhino-orbital mucormycosis caused by saksenaea vasiformis in an immunocompromised child. Pediatric Infectious Disease Journal. 16(7):714-716, July 1997.  [CrossRef]  [PubMed]
  4. Yohai RA, Bullock JD, Aziz AA, Markert RJ. Rhino-orbital-cerebral mucormycosis in type 1 diabetes mellitus, Survival factors in rhino-orbital-cerebral mucormycosis: Major Review. Surv Ophthalmol 1994; 39: 3-22.  [CrossRef]
  5. Ali A. Hilal , Saad J. Taj-Aldeen , Abdulla H. Mirghani , Rhino orbital mucormycosis secondary to Rhizopus oryzae: a case report and literature review. Ear, Nose & Throat Journal , August, 2004 : 102 - 106.
  6. Patel LR, Modi TH, Shah PD, Deokule JS. Mucormycosis. A clinicomycological spectrum. Indian J Med Microbiol 2004;22:133-133.  [PubMed]

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