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Zygomycosis

Mucormycosis

Zygomycosis is a devastating fungal infection whose manifestations vary according to the organ system affected. Although it is usually seen in immunocompromised patients, it can also occur in individuals with an intact immune system.


Presentation

Zygomycosis in humans is caused by Rhizopus, Mucor, and Rhizomucor while Cunninghamella, Absidia, Saksenaea, and Apophysomyces are genera that are less often associated [1]. It typically affects immunocompromised individuals or those with diabetes mellitus and can manifest in five major forms: rhinocerebral, pulmonary, abdominopelvic and gastric (gastrointestinal), primary cutaneous, and disseminated [2] [3] [4] [5].

Right Upper Quadrant Pain
  • We report a case of a 22-year-old man with right upper quadrant pain and fever. The CT of the abdomen revealed renal cell carcinoma and subsequent nephrectomy was performed. The histopathological diagnosis of nephrectomy established zygomycosis.[ncbi.nlm.nih.gov]
Decreased Bowel Sounds
  • GI zygomycosis Decreased bowel sounds, guarding or rebound tenderness, and localized-to-diffuse abdominal tenderness may be present.[symptoma.com]
Oral Ulcers
  • Pain and swelling precede oral ulceration and the resulting tissue necrosis can result in palatal perforation. Infection can sometimes extend from the sinuses into the mouth and produce painful, necrotic ulcerations of the hard palate.[ncbi.nlm.nih.gov]
Purpura
  • The patient was suffering from idiopathic thrombopenic purpura, diagnosed 1 year earlier.[ncbi.nlm.nih.gov]
Focal Neurologic Deficit
  • Disseminated zygomycosis Sudden onset focal neurologic deficits, lethargy, altered sensorium, coma, with necrotic ulcerations on the skin or oral cavity, are found.[symptoma.com]
  • Patients present with sudden onset of focal neurological deficits or coma. The mortality associated with dissemination to the brain approaches 100% ( 144 ).[doi.org]
Insomnia
  • Personal Health Photo Credit Gracia Lam Insomnia Can Kill You Chronic insomnia is linked to an increased risk of developing hypertension, Type 2 diabetes, heart attack, depression, anxiety and premature death. By JANE E.[health.nytimes.com]

Workup

Diagnosis of zygomycosis depends on a high index of suspicion, especially in immunocompromised patients, along with the identification of the characteristic broad aseptate fungus with its right angle branching pattern in tissue samples obtained from necrotic areas [3] [4]. Special stains like Grocott's methenamine silver stain or periodic acid-Schiff (PAS) are necessary to demonstrate the fungal hyphae. Laboratory tests, blood culture, and serology tests do not help in the diagnosis [5] [7] [8].

Rhinocerebral zygomycosis

Physical examination may reveal periorbital cellulitis, proptosis, and ophthalmoplegia and black necrotic eschar on the hard palate or nasal mucosa. Plain sinus radiographs are non-specific and may show only mucosal thickening, with or without air-fluid levels. Computed tomography (CT) scans with contrast and magnetic resonance imaging (MRI) of the sinuses, orbit and brain are required to delineate the extent of the disease.

Pulmonary zygomycosis

Pleuritic rub and rhonchi can be detected on examination. Sputum smear and culture are not helpful but diagnosis can be confirmed with bronchoscopic bronchoalveolar lavage and transbronchial biopsy. If bronchoscopy findings are unclear, then open lung biopsy may be required. Radiographs of the chest demonstrate mass-like or nodular or cavitary lesions indistinguishable from lesions caused by aspergillosis. CT scan with contrast can provide information about the extent of disease while CT guided lung biopsy can provide tissue for histology [9].

GI zygomycosis

Decreased bowel sounds, guarding or rebound tenderness, and localized-to-diffuse abdominal tenderness may be present. Upper GI endoscopy helps to detect zygomycosis in the esophagus and stomach but a majority of cases are diagnosed during surgery or postmortem with a tissue biopsy. Plain radiographs of the abdomen may demonstrate air under the diaphragm in patients with perforation and barium studies of the upper GI tract or colon may show filling defects suggestive of zygomycosis. Abdominopelvic CT scan could demonstrate lesions within the intraabdominal organs and the omentum.

Cutaneous zygomycosis

Solitary lesions initially indurated and erythematous progressing to necrotic ulceration are seen. A biopsy sample from the lesion helps to diagnose the condition.

Disseminated zygomycosis

Sudden onset focal neurologic deficits, lethargy, altered sensorium, coma, with necrotic ulcerations on the skin or oral cavity, are found. As the cerebrospinal fluid analysis is nonspecific, a brain biopsy is the mainstay of diagnosis. CT scan of the chest and head may demonstrate invasive disease and delineate the extent of disease.

Multiple Pulmonary Nodules
  • Kutsuzawa TMatsuura YSakuma HNarimatsu HOhta YYamabayashi H Multiple pulmonary nodules caused by zygomycosis in a patient without predisposing factors. Jpn J Med. 1991;30435- 437 Google Scholar Crossref 45.[doi.org]

Treatment

  • Rapid treatment with amphotericin B and supportive care are essential in the care of individuals with zygomycosis.[ncbi.nlm.nih.gov]
  • Posaconazole is sometimes used for salvage treatment, as continuation of treatment after initial administration of amphotericin B, or in combination.[ncbi.nlm.nih.gov]
  • Use of voriconazole for prophylaxis or treatment of aspergillosis.[patient.info]
  • The early diagnosis and immediate initiation of treatment with an antifungal agent in combination with surgical intervention has proved critical for the favourable outcome of the disease. Few antifungal agents are available for treatment.[ncbi.nlm.nih.gov]
  • Epidemiological characteristics, predisposing conditions, clinical presentation, diagnostic findings and treatment outcomes were recorded. Characteristic radiological findings were recorded.[ncbi.nlm.nih.gov]

Prognosis

  • If diagnosed early, it generally has a good prognosis. Secondary zygomycosis is usually a complication and extension of the rhinocerebral variety that starts as a palpebral fistula and progresses to a necrotic lesion with a poor prognosis.[ncbi.nlm.nih.gov]
  • The present study is aimed to evaluate its histopathologic features as an aid to prognosis in order to guide the physician at the stage of tissue diagnosis to optimize surgery, chemotherapy and immunosuppression.[ncbi.nlm.nih.gov]
  • GCS is an independent marker of prognosis in cases of invasive zygomycosis.[ncbi.nlm.nih.gov]
  • This report underscores the importance of prior susceptibility testing of zygomycetes to guide therapy with the most effective antifungal agent for an improved prognosis.[ncbi.nlm.nih.gov]
  • Prognosis [ 8 ] Mucormycosis has a very high mortality rate of at least 50%. Mortality from pulmonary and gastrointestinal (GI) disease is even higher due to late diagnosis.[patient.info]

Etiology

  • Enterocolitis, Necrotizing/diagnosis* Enterocolitis, Necrotizing/surgery Fasciitis, Necrotizing/etiology* Fasciitis, Necrotizing/surgery Fatal Outcome Fetal Growth Retardation Gangrene Humans Ileal Diseases/congenital Ileal Diseases/surgery Infant, Newborn[ncbi.nlm.nih.gov]
  • We present a case of acute renal artery thrombosis due to disseminated zygomycosis in a healthy young adult male.The diagnosis of renal artery occlusion was made on contrast-enhanced CT (CECT) and confirmation of etiology was made only on post mortem[ncbi.nlm.nih.gov]
  • An extensive workup was unrevealing of the etiology, and the patient expired. A full autopsy was declined, but an in situ post-mortem transbronchial lung biopsy revealed pulmonary zygomycosis.[ncbi.nlm.nih.gov]
  • The major reported etiologic agents in China include Rhizomucor spp., Rhizopus spp., Mucor spp., and Lichtheimia spp. (formerly Absidia spp.).[ncbi.nlm.nih.gov]
  • In both cases a percutaneous nephrostomy (PCN) was performed and the etiologic agents were identified by direct microscopy and culture. Amphotericin B was administered and both patients recovered completely.[ncbi.nlm.nih.gov]

Epidemiology

  • Presented herein is our investigation of the epidemiological factors associated with an increasing incidence of the disease at the University of Geneva Hospitals, Geneva, Switzerland, over the past five years.[ncbi.nlm.nih.gov]
  • CONCLUSION: These results of the epidemiological analysis of autopsy cases with mycosis demonstrate that clinicians should promptly recognize and treat zygomycosis.[ncbi.nlm.nih.gov]
  • INTRODUCTION: No comprehensive reports have been published on epidemiological status of Rhinocerebral zygomycosis infections and its outcome in our population, Hence, the current study came to address epidemiological characteristics as well as clinical[ncbi.nlm.nih.gov]
  • However, it is not known if this association reflects a true epidemiological link or represents a marker of changing immunosuppression occurring in parallel with the evolution of transplant practices and immunosuppression strategies.[ncbi.nlm.nih.gov]
  • Although microscopy reveals the fungal aetiology, the need for species identification is highlighted to better understand the species and establish an epidemiological pattern as it is reported from restricted geographical locations.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • Etiology and Pathophysiology Risk factors Immunocompromising conditions are the main risk factor for mucormycosis. Patients with uncontrolled diabetes mellitus, especially those with ketoacidosis, are at high risk.[emedicine.com]
  • Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clinical Microbiology Reviews. 2005 Jul 1;18(3):556-69. PubMed Google Scholar[panafrican-med-journal.com]
  • Spellberg B, Edwards JJ, Ibrahim A: Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18: 556–569.[karger.com]
  • Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clin Microbiol Rev 2005 ;18: 556 - 569 35. Kontoyiannis DP, Lewis RE. How I treat mucormycosis. Blood 2011 ;118: 1216 - 1224 36.[doi.org]

Prevention

  • The use of higher doses of antifungal treatment such as amphotericin can prevent both mortality and prolonged hospital stay. The cancer patients may need longer hospital stay because of needing comprehensive in-hospital treatment.[ncbi.nlm.nih.gov]
  • This case is being presented not only because of its rarity, but to emphasize the role of early diagnosis and appropriate treatment to prevent serious complications due to proximity to major structures.[ncbi.nlm.nih.gov]
  • Mucormycosis and similar fungal infections that enter the skin through puncture wounds can usually be prevented once a wound is disinfected in a hospital, health officials said.[nytimes.com]

References

Article

  1. Roden MM, Zaoutis TE, Buchanan WL, et al. Epidemiology and outcome of zygomycosis: a review of 929 reported cases.Clin Infect Dis. 2005;41(5):634.
  2. Kontoyiannis DP, Wessel VC, Bodey GP, et al. Zygomycosis in the 1990s in a tertiary-care cancer center. Clin Infect Dis. 2000 Jun. 30(6):851-6.
  3. Binder U, Maurer E, Lass-Flörl C. Mucormycosis--from the pathogens to the disease. Clin Microbiol Infect. 2014 Jun; 20 Suppl 6:60-6.
  4. Tacke D, Koehler P, Markiefka B, Cornely OA. Our 2014 approach to mucormycosis. Mycoses. 2014 Sep.; 57 (9):519-24.
  5. Walsh TJ, Gamaletsou MN, McGinnis MR, Hayden RT, Kontoyiannis DP. Early clinical and laboratory diagnosis of invasive pulmonary, extrapulmonary, and disseminated mucormycosis (zygomycosis). Clin Infect Dis. 2012 Feb; 54 Suppl 1:S55-60.
  6. Thomson SR, Bade PG, Taams M, et al. Gastrointestinal mucormycosis. Br J Surg. 1991 Aug; 78(8):952-4.
  7. Gonzalez CE, Rinaldi MG, Sugar AM. Zygomycosis. Infect Dis Clin North Am. 2002 Dec; 16(4):895-914, vi.
  8. Kwon-Chung KJ. Taxonomy of fungi causing mucormycosis and entomophthoramycosis (zygomycosis) and nomenclature of the disease: molecular mycologic perspectives. Clin Infect Dis. 2012 Feb; 54 Suppl 1:S8 -S15.
  9. Lass-Flörl C, Resch G, Nachbaur D, Mayr A, Gastl G, Auberger J, et al. The value of computed tomography-guided percutaneous lung biopsy for diagnosis of invasive fungal infection in immunocompromised patients. Clin Infect Dis. 2007 Oct 1; 45(7):e101-4.

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Last updated: 2019-07-11 22:25