Michele Todman, MD   Yoash Enzer, MD November 3, 2009 Grand Rounds
Presentation <ul><li>19 y/o F with bilateral periorbital erythema/edema and left facial/cheek erythema and swelling x 5 da...
Hospital Day #1
Hospital Day #5 <ul><li>On unasyn x 1 day changed to zosyn (GN and pseudomonas) x 4 days and vancomycin x 2 days with no i...
Hospital Day #5
Hospital Day #5
Hospital Day #5
Exam on Hospital Day #5 <ul><li>Va: 20/20 OD, 20/30 OS </li></ul><ul><li>Pupils: no RAPD OU </li></ul><ul><li>Motility: (-...
Hospital Day #5
Sinus Surgery x 1  on Hospital Day #5 <ul><li>Left ethmoid contents left maxillary sinus aspirate and left cheek aspirate,...
Hospital Day #5
Preliminary Culture Results  Hospital Day #6 <ul><li>Found to have presumed mucormycosis (aseptate broad band hyphae) invo...
MRI to r/o intracranial abscess and cavernous sinus thrombosis on Hospital Day #6 <ul><li>No evidence of cavernous sinus t...
Sinus surgery x 2  combined with Oculoplastics Hospital Day # 7 <ul><li>Left orbitotomy with spinal catheter placement </l...
Hospital Day #7
Hospital Day #9
Sinus Surgery x 3 Hospital Day #10 <ul><li>Incision and drainage of anterior cheek abscess  </li></ul><ul><li>Left Maxilla...
Exam Hospital Day #11 <ul><li>Va:20/20 OU </li></ul><ul><li>Pupils:4mm OU, PERRL OU, No RAPD OU </li></ul><ul><li>Ta: + Re...
Hospital Day #11
Sinus Surgery x 4 Hospital Day #16 <ul><li>Replace the existing catheter with a Bardport MRI implanted port, 6.6-French, o...
Hospital Day #16
Hospital Day #16
MRI Hospital Day #17 <ul><li>Stable left orbital cellulitis with extraconal phlegmon adjacent to the left lamina papyracea...
Discharged hospital day #23 <ul><li>Va 20/20 OU; EOMS: Full OU; Pupils: No RAPD OU; Globe soft </li></ul><ul><li>Drains re...
Mortality Rate of Mucormycosis is 50%-80%, once CNS involvement mortality rate is  ≥  80% This is War! <ul><li>Must have h...
 
Questions <ul><li>What are the keys to successful management of these patients? </li></ul><ul><li>How long should antifung...
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Mucoropfinallast

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Mucoropfinallast

  1. 1. Michele Todman, MD Yoash Enzer, MD November 3, 2009 Grand Rounds
  2. 2. Presentation <ul><li>19 y/o F with bilateral periorbital erythema/edema and left facial/cheek erythema and swelling x 5 days prior to admission; seen by PCP 3 days prior to admission and prescribed Augmentin with no improvement </li></ul><ul><ul><li>s/p renal transplant cadaveric x 2 (1996, 2006) for focal segmental glomerulosclerosis with post transplant diabetes (Hgba1c=14.7) </li></ul></ul><ul><ul><li>Meds : glipizide, lisinopril, cellcept 750mg 2x daily, prednisone 5mg daily, sirolimus 2mg 1xdaily </li></ul></ul><ul><ul><li>No history of trauma </li></ul></ul><ul><ul><li>No new meds, ocular discharge, fevers or chills </li></ul></ul><ul><li>Exam on hospital day #2: Va 20/25 OU; trace chemosis OU, remainder of eye exam WNL </li></ul><ul><ul><li>No dacryocystitis, clear demarcation line or visible skin lesions </li></ul></ul><ul><ul><li>No RAPD, ophthalmoplegia, proptosis, or ocular hypertension </li></ul></ul>
  3. 3. Hospital Day #1
  4. 4. Hospital Day #5 <ul><li>On unasyn x 1 day changed to zosyn (GN and pseudomonas) x 4 days and vancomycin x 2 days with no improvement </li></ul>
  5. 5. Hospital Day #5
  6. 6. Hospital Day #5
  7. 7. Hospital Day #5
  8. 8. Exam on Hospital Day #5 <ul><li>Va: 20/20 OD, 20/30 OS </li></ul><ul><li>Pupils: no RAPD OU </li></ul><ul><li>Motility: (-3 laterally, -4 inferiorly, -1 nasally OD); </li></ul><ul><li>full OS </li></ul><ul><li>Confrontation VF full OU </li></ul><ul><li>No proptosis or resistance to retropulsion </li></ul><ul><li>External exam: Hard, indurated, edematous and erythematous left side tracking down to upper lip with bilateral periorbital erythema and edema </li></ul><ul><li>SLE: Chemosis inferiorly OS </li></ul><ul><li>DFE:WNL OU </li></ul>
  9. 9. Hospital Day #5
  10. 10. Sinus Surgery x 1 on Hospital Day #5 <ul><li>Left ethmoid contents left maxillary sinus aspirate and left cheek aspirate, all sent for cultures. </li></ul><ul><li>Changed antibiotic coverage and added antifungal to include: meropenem 1g IV q 8 hours and posaconazole 200 mg qid </li></ul>
  11. 11. Hospital Day #5
  12. 12. Preliminary Culture Results Hospital Day #6 <ul><li>Found to have presumed mucormycosis (aseptate broad band hyphae) involving her left sinuses, her left cheek and her left ethmoid bone </li></ul><ul><li>ID added Amphotericin B 5mg/kg IV per 24 hours and continued vancomycin, meropenem and posaconazole. Stopped sirolimus, decreased Cellcept from 750mg 2x daily to 250mg 2x daily and continued prednisone 5mg daily. </li></ul><ul><li>Control of blood sugars followed by endocrine and glipizide and insulin drip on board. Hgba1c=14.7. </li></ul><ul><li>Acid/base status also tightly controlled. </li></ul>
  13. 13. MRI to r/o intracranial abscess and cavernous sinus thrombosis on Hospital Day #6 <ul><li>No evidence of cavernous sinus thrombosis, intracranial abscess, or intraconal abscess. </li></ul><ul><li>Thickening and enhancement of the medial left orbital periosteum, compatible with post-septal, extraconal extension of the inflammatory process involving the preseptal and facial soft tissues along the periosteum. No definite evidence of intraconal extension. Motion Artifact. </li></ul>
  14. 14. Sinus surgery x 2 combined with Oculoplastics Hospital Day # 7 <ul><li>Left orbitotomy with spinal catheter placement </li></ul><ul><li>Debridement of sinuses and medial orbit with drain placement in maxillary sinus </li></ul><ul><li>External ethmoidectomy for necrotic ethmoid bone </li></ul><ul><li>2ml of amphotericin B at 0.25mg/ml infused through catheter in both orbit and maxillary sinus q 6 hours </li></ul>
  15. 15. Hospital Day #7
  16. 16. Hospital Day #9
  17. 17. Sinus Surgery x 3 Hospital Day #10 <ul><li>Incision and drainage of anterior cheek abscess </li></ul><ul><li>Left Maxillary sinus debridement, left inferior turbinectomy(necrotic), caldwell luc procedure (intraoral procedure for entering the maxillary antrum through the canine fossa above the maxillary premolar teeth) </li></ul>
  18. 18. Exam Hospital Day #11 <ul><li>Va:20/20 OU </li></ul><ul><li>Pupils:4mm OU, PERRL OU, No RAPD OU </li></ul><ul><li>Ta: + Resistance to retropulsion OS </li></ul><ul><li>EOMS:(-1)medially and superiorly, </li></ul><ul><li>(-2)laterally and inferiorly </li></ul>
  19. 19. Hospital Day #11
  20. 20. Sinus Surgery x 4 Hospital Day #16 <ul><li>Replace the existing catheter with a Bardport MRI implanted port, 6.6-French, open-ended single lumen catheter so that MRI could easily be performed with this new catheter </li></ul><ul><li>Further debrided necrotic tissue in the sinuses or orbit </li></ul>
  21. 21. Hospital Day #16
  22. 22. Hospital Day #16
  23. 23. MRI Hospital Day #17 <ul><li>Stable left orbital cellulitis with extraconal phlegmon adjacent to the left lamina papyracea </li></ul><ul><li>There is no drainable fluid collection. Stable pansinusitis. Interval decrease in size of the 1.1 x 0.7 cm rim-enhancing fluid collection anterior to the left maxilla consistent with improving abscess </li></ul><ul><li>No intracranial extension of infection </li></ul>
  24. 24. Discharged hospital day #23 <ul><li>Va 20/20 OU; EOMS: Full OU; Pupils: No RAPD OU; Globe soft </li></ul><ul><li>Drains removed ( 2ml of amphotericin B at 0.25mg/ml infused through catheter in both orbit and maxillary sinus q 6 hours x 2 weeks) </li></ul><ul><li>Discharged home on: </li></ul><ul><li>IV amphotericin 385mg once daily, glipizide 20mg twice a day, CellCept 250mg every 12 hours, posaconazole 400mg po 2xdaily, and prednisone 5mg once a day. Sirolimus held. </li></ul><ul><li>Once MRI fully resolved and creatinine completely normalized will </li></ul><ul><li>stop all antifungals except posaconazole 400mg 1x daily (not </li></ul><ul><li>treatment dose) and restart immunosuppressive therapy at full strength. </li></ul>
  25. 25. Mortality Rate of Mucormycosis is 50%-80%, once CNS involvement mortality rate is ≥ 80% This is War! <ul><li>Must have high index of suspicion early </li></ul><ul><li>Aggressive management is a must and should include an all out war: debridement, a polyene both IV and direct through catheter in orbit and sinus, combination IV therapy (LFAB-deferasirox) and if possible hyperbaric oxygen. </li></ul><ul><li>Must reverse underlying disease- Control DM tightly, normalize acid/base status, and decrease or stop immunosuppressive therapy so body can fight infection </li></ul><ul><li>MRI is key to check for CNS or orbital involvement </li></ul><ul><li>Total duration of therapy is individualized but stop when: resolution of clinical signs and symptoms of infection, resolution or stability on serial imaging, and resolution of underlying immunosuppression. </li></ul><ul><li>If no resolution of immunosuppression possible then prophylaxis such as posaconazole should be instituted for life. </li></ul>
  26. 27. Questions <ul><li>What are the keys to successful management of these patients? </li></ul><ul><li>How long should antifungal therapy be given and how to balance that with restarting immunosuppressive therapy? </li></ul><ul><li>What about prophylactic antifungal therapy for life? </li></ul>

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