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Teaching Case of the Week

Teaching Case of the Week. Dr. W. A. Ciccotelli Sept 14, 2005. The Patient. 82 y M Past Hx Low grade B cell lymphoma Pancytopenia/transfusion dependent Interstitial lung dz HTN Ex-smoker. The Patient. Meds Amlodipine Prednisone (taperingx 4 mos) NKDA 2-4x EtOH/wk. The Case.

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Teaching Case of the Week

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  1. Teaching Case of the Week Dr. W. A. Ciccotelli Sept 14, 2005

  2. The Patient • 82 y M • Past Hx • Low grade B cell lymphoma • Pancytopenia/transfusion dependent • Interstitial lung dz • HTN • Ex-smoker

  3. The Patient • Meds • Amlodipine • Prednisone (taperingx 4 mos) • NKDA • 2-4x EtOH/wk

  4. The Case • Referred to ID for peri-orbital cellulitis • 3 day Hx of progressive • R eye swelling • R frontal headache • Reactive clear discharge • FB sensation • No fever/chills • No other ocular symptoms • Vision ok • On cefotaxime 36 hrs

  5. The Case • Afebrile, VSS • Peri-orbital cellulitis • R eye proptosis, mild ptosis, chemosis • Loss of EOM R eye • CNs normal otherwise • Visual acuity normal

  6. The Case • WBC 4.9, Hgb 99, plts 54, grans 1.7 • Lytes N • Cr 123 • TSH 1.1 • Panculture neg • CXR: unchanged chronic interstitial pattern

  7. The Case • CT scan head • R pre-septal edema • Minimal proptosis R eye • R Maxillary & ethmoidal sinusitis • R nasal septum deviation • No bony lesions • No retro-orbital masses • ENT consulted

  8. The Case • Not responding on Cefotaxime • Febrile • New diplopia • Worsening peri-orbital cellulitis

  9. The Case • Abx changed to Clinda/Cipro • MRI head • Small fluid collection lat. R eye ?abscess • Maxillary & ethmoid sinusitis (L & R) • Meninges inflammatory changes in R middle cranial fossa • No cavernous vein thrombosis • Nasal culture: commensal flora

  10. The Case • Now really bad! • Delirious • Febrile • Clonus in lower ext. • R Facial droop

  11. The Case • Urgent ethmoidectomy • necrotic sinus • painless procedure • LP aseptic meningitis • ANCAs neg • Lipo Ampho B started 5 mg/kg/day

  12. The Case • Repeat MRI • Early cerebritis R temporal operculum • Ongoing inflammatory changes of all sinuses • Inflammatory changes around R orbit, masticator space, cavernous sinus

  13. Case Resolution • Further CNS deterioration • Sinus Bx • Broad ribbon like non-septate fungal filament on microscopy • ZN & PAS stains confirm non-septate hyphae • Dx of Rhinocerebral zygomycosis • Lipo Ampho B to 10 mg/kg/day • Family withdrew care given degree of surgery needed

  14. Zygomycosis • Mucorales order • Ubiquitous in environment • Thick walled non-septate hyphae with right angle branching • Rare & mimics other invasive mould infections • Inherent resistance to antifungal agents • Angioinvasive disease

  15. Zygomycosis • Multiple clinical forms • Cutaneous • Pulmonary • Gastrointestinal • Rhinocerebral • Sino-orbital • Disseminated • Direct inoculation, inhalation, ingestion of spores

  16. Zygomycosis • Immunocompromised state hallmarks • DM ketoacidosis • Neutropenia • Chemotherapy • BMT patients • Lymphoma/leukemia • Trauma with exposure to contaminated soil

  17. Zygomycosis • Dx is difficult & delayed • Poor recovery from culture • Non specific presentation • Not on everyone’s DDx • Mimics other invasive molds (Aspergillus) • Dx generally made with invasive testing for histopathological sampling • Dx commonly made at autopsy • Yet increasingly problematic in Heme-Onc patients over 1990s

  18. Zygomycosis • Treatment is multifaceted • Immune reconstitution • Aggressive surgical debridement • Ampho B • Prayer • Posaconazole as oral alternative • Despite this still highly fatal (mortality 50-80%)

  19. Zygomycosis • Prognosis is poor • Late Dx • Not able to recover immune system • Disseminated • Death usually from hemorrhage • Best prognosis • Limited disease • Early surgery • Non Heme-Onc patients

  20. Zygomycosis • Tip offs • Right patient population (esp neutropenia) • Unexplained thrombosis • Necrotic eschar • Unexplained hemorrhage • Common clinical situations • Culture neg despite real disease • Not responsing to reasonable Abx

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