Indolent Mucormycosis of the Paranasal Sinus: An Emerging ... · Indolent Mucormycosis of the...
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F: female, M: male, (m): months, NA: non available, NED: no evidence of disease. Indolent Mucormycosis of the Paranasal Sinus: An Emerging Entity Erika Celis-Aguilar, MD 1 , Alan Burgos-Páez 1 , Nadia Villanueva-Ramos 1 , José Solórzano-Barrón 1 , Juan Manjarrez-Velázquez, MD 2 , Lucero Escobar-Aispuro 1 , Alma de la Mora-Fernández, MD 1 1 Department of Otolaryngology, Hospital Civil de Culiacán, México 2 Department of Otolaryngology, Hospital General de Culiacán, México INTRODUCTION DISCUSSION RESULTS ABSTRACT METHODS AND MATERIALS CONCLUSIONS REFERENCES CONTACT Outcome Objectives: 1. Describe the clinical characteristics and management of indolent mucormycosis of the nasal/paranasal sinus 2. Describe the outcomes and follow up of patients with indolent mucormycosis. Methods: Study design: Multicenter clinical chart review, setting: secondary care centers. From November 2012 to January 2015. Subjects included were patients with indolent mucormycosis, defined by pathological confirmation of mucormycosis and evidence of symptoms of paranasal sinus disease longer than 1 month. All patients underwent imaging, laboratory workup & surgical treatment. Results: Six cases were included with indolent mucormycosis, 2 female and 4 male patients, mean age was 54.3 years. Three patients were immunosuppressed and three patients immunocompetent. Symptoms were nonspecific, facial pain, mucoid discharge and cacosmia were the most frequently reported. Maxillary sinus involvement was present in all cases and it was the only paranasal sinus involved in the immunocompetent cases. Mainly, the surgical procedure performed consisted in anterior maxillary approach and endoscopic ethmoidectomy and antrostomy. Only one immunosuppressed patient underwent orbital exenteration. Two immunosuppressed subjects received amphotericin. Posaconazol was the only treatment in one immunosuppressed patient. Immunocompetent cases underwent only surgical treatment. In the follow up, patients have no evidence of disease. Conclusion: Indolent mucormycosis of the paranasal sinus is an emerging entity of immunosuppressed and immunocompetent patients. Single paranasal sinus disease is a frequent presentation and should not be overlooked as a differential diagnosis in these patients. Immunocompetent patients should only be treated surgically. More studies are needed to confirm our results. Results: We included 6 patients, 2 female and 4 male subjects. Mean age was 54.33 years. Three patients were immunosuppressed and 3 immunocompetent. Maxillary sinus involvement was present in all patients. Among the immunosuppressed patients all had diabetes. Symptoms were nonspecific. Facial pain, mucoid discharge and cacosmia were the symptoms most frequently reported. Two patients had asymptomatic mucormycosis, found incidentally on CT scan. Chronic or indolent mucormycosis of the paranasal sinuses has been described as early as 1964 by Vignale with over 30 cases in the literature 1 . Interestingly; it can affect immunocompetent and immunosuppressed individuals. Immunocompetent cases are associated with a less severe disease; there could be predisposing factors such as chronic rhinosinusitis or penetrating trauma or have no identifiable risk factors. Signs and symptoms are similar to those of chronic rhinosinusitis with gradual onset through years 2 . The definition of chronic mucormycosis has been controversial, with the presence of mucormycosis for at least one month 3,4 . All the patients of our series had one month or more of suggestive mucormycosis infection. One common clinical feature we could find in most of our patients is the presence of facial pain or headache, this could be a hallmark symptom in a patient with chronic paranasal sinus disease with evident CT scan sinus occupation. The most common presentation in our series was unilateral maxilar sinus occupation (two right, three left), only one patient also had orbital apex syndrome (Case 1). Necrosis was seen in most of our patients during Caldwell Luc procedure in maxillary mucosa, but no evidence of necrosis was seen on rhinoscopy or nasal endoscopy, a common finding in acute invasive mucormycosis (80% of patients 5 ). There is controversy on the best treatment available for this pathology. Some authors advocate towards a surgical and amphotericin B treatment, while others support only the surgical or medical treatment as monotherapy; it has been reported the successful treatment of chronic paranasal mucormycosis with only surgical treatment; three cases of the study received only surgical procedure without recurrence 6 . In contrast to acute fulminant invasive sinusitis, chronic mucormycosis, could have better survival. All cases reported in this study had a 100% survival rate. Methods and materials: Study design: Multicenter clinical chart review, setting: secondary care centers. Patients were recruited from November 2012 to January 2015. Indolent mucormycosis was defined by pathological confirmation of mucormycosis and evidence of symptoms of paranasal sinus disease longer than 1 month. All patients underwent imaging, laboratory workup & surgical treatment. Surgical procedures consisted in external maxillary approach and endoscopic ethmoidectomy and antrostomy. All surgical specimens were evaluated by a certified pathologist Indolent mucormycosis of the paranasal sinus is an emerging entity of immunosuppressed and immunocompetent patients. Single paranasal sinus disease is a frequent presentation and should not be overlooked as a differential diagnosis in these patients. Immunocompetent patients should only be treated surgically. More studies are needed to confirm our results. Introduction: Mucormycosis refers to any fungal infection by members of the order Mucorales, in the class of Zygomycetes. Rhizopus, Mucor, Rhizomucor and species of Aspergillus are the most common etiologic agents in the sinonasal cavity. Mucormycosis of the nasal cavity and paranasal sinuses is an uncommon infection with a rapid aggressive, life threatening course, often in immunosuppressed patients, potentially fatal. Microscopically, these fungi demonstrate broad non- septate hyphae, thick walled with branching at right angles. In contrast with this fulminant entity, there´s other form of mucormycosis known as chronic or indolent among immunosuppressed and immunocompetent patients. Is limited, chronic (> 1 month), less aggressive and frequently with involvement of single paranasal sinus disease with non-specific nasal symptoms. This study describes 6 cases of indolent mucormycosis. 1. Waizel-Haiat, Salomón, et al. "Mucormicosis rinocerebral invasora crónica."Cirugía y Cirujanos . 2003; 71.2: 145-149. 2. Jung H, Park SK. Indolent mucormycosis of the paranasal sinus in immunocompetent patients: are antifungal drugs needed?. J Laryngol Otol. 2013;127(09):872-5. 3. Manuel, Marín-Méndez Héctor, et al. "Síndrome de ápex orbitario causado por mucormicosis orbitocerebral crónica e indolente: reporte de dos casos." An Orl Mex. 2005; Vol. 50. No. 1: 64-68. 4. Dooley, David P., et al. "Indolent orbital apex syndrome caused by occult mucormycosis." J Neuroophthalmol. 1992; 12: 245-249. 5. Busaba, Nicolas Y., et al. "Chronic invasive fungal sinusitis: a report of two atypical cases.(Original Article)." Ear Nose Throat J. 2002; 81: 462-467. 6. Ketenci, Ibrahim, et al. "Indolent mucormycosis of the sphenoid sinus." Am J Otolaryngol Head Neck Med Surg. 2005; 132: 341-342. Erika Celis-Aguilar MD Hospital Civil de Culiacan Email: [email protected] CASES Fig 1A, 1B: Coronal and sagittal CT scan shows involvement of left maxillary sinus, left nasal cavity and left orbitary apex. 1C: pathology result confirmed mucormycosis with broad non-septate hyphae Case 1 Case 2 Fig 2A: Coronal CT scan showed findings compatible with fungus ball in left maxillary sinus pathology. 2B: PAS staining revealed mucormycosis, with right angle hyphae. 2C: Coronal CT scan of 2 years follow up Fig. 3A: pathology report of mucormycosis with broad non-septate hyphae Case 3 Fig 4A: Axial CT scan demonstrate occupation of left maxillary sinus and osteitis of the sinus walls. 4B: PAS staining; positive suggestive of spores and hyphae that support Zygomycetes Case 4 Fig 5A: Axial CT scan reported occupation of right nasal cavity, no erosion, no bone involvement, with occupation of left maxillary sinus. The mass of right nasal cavity was reported as a polyp and the occupation of left maxillary sinus revealed mucormycosis. 5B: Histopathologic image of Grocott-Gomori stain with abundant broad hyphae and positive staining. Coronal CT scan 1 year follow up (Fig 5C) Case 5 Fig 6A: Coronal CT scan reported occupation of right maxillary sinus with enlargement of maxillary ostium and osteitis of lateral wall. 6B: Pathology reported abundant hyphae compatible with mucormycosis at PAS staining. 6C: Axial CT scan at one year follow up, which shows thickening of the right mucosal of the maxillary sinus. Case 6 TABLE 1. Clinical features, treatment and follow up. Patient number Sex Age Immunocompetent status Anatomic localization Duration of symptoms (m) Clinical features Treatment Follow up 1 F 38 Immunosuppressed Rhino-orbital Left maxillary Left ethmoid Left Orbit 1 Maxillary pain, proptosis, pupil dilation, restriction of ocular movements. Acute facial pain Left Caldwell Luc Ethmoidectomy Orbital exenteration Liposomal Amphotericine 3gr Posaconazol 45 days Two years follow up, NED 2 F 61 Immunosuppressed Left maxillary sinus 6 Headache, facial pain, purulent rhinorrhea, halitosis, cacosmia Left Caldwell Luc Amphotericin B 750 mg 2.5 years follow up, NED 3 M 54 Immunosuppressed Right maxillary sinus NA Asymptomatic Right Cadwell Luc Posaconazol Two years follow up, NED 4 M 42 Immunocompetent Left maxillary sinus 24 Mucoid discharge, nasal obstruction, headache. Left Caldwell Luc One year follow up, NED 5 M 77 Immunocompetent Left maxillary sinus 60 Contralateral nasal mass (right nasal polyp) obstruction, asymptomatic Left Caldwell Luc Resection of contralateral mass One year follow up, NED 6 M 54 Immunocompetent Right maxillary sinus 8 Headache, cacosmia Right Caldwell Luc (Caldwell Luc a year after, no recurrence) 1.6 year, asymptomatic
Transcript of Indolent Mucormycosis of the Paranasal Sinus: An Emerging ... · Indolent Mucormycosis of the...
F: female, M: male, (m): months, NA: non available, NED: no evidence of disease.
Indolent Mucormycosis of the Paranasal Sinus: An Emerging Entity
Erika Celis-Aguilar, MD1, Alan Burgos-Páez 1, Nadia Villanueva-Ramos 1, José Solórzano-Barrón 1, Juan Manjarrez-Velázquez, MD2, Lucero Escobar-Aispuro 1, Alma de la Mora-Fernández, MD 1
1Department of Otolaryngology, Hospital Civil de Culiacán, México2 Department of Otolaryngology, Hospital General de Culiacán, México
INTRODUCTION DISCUSSION
RESULTS
ABSTRACT
METHODS AND MATERIALS
CONCLUSIONS
REFERENCES
CONTACT
Outcome Objectives: 1. Describe theclinical characteristics and managementof indolent mucormycosis of thenasal/paranasal sinus 2. Describe theoutcomes and follow up of patientswith indolent mucormycosis.Methods: Study design: Multicenterclinical chart review, setting: secondarycare centers. From November 2012 toJanuary 2015. Subjects included werepatients with indolent mucormycosis,defined by pathological confirmation ofmucormycosis and evidence ofsymptoms of paranasal sinus diseaselonger than 1 month. All patientsunderwent imaging, laboratory workup& surgical treatment.Results: Six cases were included withindolent mucormycosis, 2 female and 4male patients, mean age was 54.3years. Three patients wereimmunosuppressed and three patientsimmunocompetent. Symptoms werenonspecific, facial pain, mucoiddischarge and cacosmia were the mostfrequently reported. Maxillary sinusinvolvement was present in all casesand it was the only paranasal sinusinvolved in the immunocompetentcases. Mainly, the surgical procedureperformed consisted in anteriormaxillary approach and endoscopicethmoidectomy and antrostomy. Onlyone immunosuppressed patientunderwent orbital exenteration. Twoimmunosuppressed subjects receivedamphotericin. Posaconazol was the onlytreatment in one immunosuppressedpatient. Immunocompetent casesunderwent only surgical treatment. Inthe follow up, patients have noevidence of disease.Conclusion: Indolent mucormycosis ofthe paranasal sinus is an emergingentity of immunosuppressed andimmunocompetent patients. Singleparanasal sinus disease is a frequentpresentation and should not beoverlooked as a differential diagnosis inthese patients. Immunocompetentpatients should only be treatedsurgically. More studies are needed toconfirm our results.
Results: We included 6 patients, 2 female and 4 malesubjects. Mean age was 54.33 years. Three patientswere immunosuppressed and 3 immunocompetent.Maxillary sinus involvement was present in allpatients. Among the immunosuppressed patients allhad diabetes. Symptoms were nonspecific. Facialpain, mucoid discharge and cacosmia were thesymptoms most frequently reported. Two patientshad asymptomatic mucormycosis, found incidentallyon CT scan.
Chronic or indolent mucormycosis of the paranasalsinuses has been described as early as 1964 byVignale with over 30 cases in the literature1.Interestingly; it can affect immunocompetent andimmunosuppressed individuals. Immunocompetentcases are associated with a less severe disease; therecould be predisposing factors such as chronicrhinosinusitis or penetrating trauma or have noidentifiable risk factors. Signs and symptoms aresimilar to those of chronic rhinosinusitis with gradualonset through years2. The definition of chronicmucormycosis has been controversial, with thepresence of mucormycosis for at least one month 3,4.All the patients of our series had one month or moreof suggestive mucormycosis infection.One common clinical feature we could find in most ofour patients is the presence of facial pain orheadache, this could be a hallmark symptom in apatient with chronic paranasal sinus disease withevident CT scan sinus occupation. The most commonpresentation in our series was unilateral maxilar sinusoccupation (two right, three left), only one patientalso had orbital apex syndrome (Case 1). Necrosis wasseen in most of our patients during Caldwell Lucprocedure in maxillary mucosa, but no evidence ofnecrosis was seen on rhinoscopy or nasal endoscopy,a common finding in acute invasive mucormycosis(80% of patients 5).There is controversy on the best treatment availablefor this pathology. Some authors advocate towards asurgical and amphotericin B treatment, while otherssupport only the surgical or medical treatment asmonotherapy; it has been reported the successfultreatment of chronic paranasal mucormycosis withonly surgical treatment; three cases of the studyreceived only surgical procedure without recurrence 6.In contrast to acute fulminant invasive sinusitis,chronic mucormycosis, could have better survival. Allcases reported in this study had a 100% survival rate.
Methods and materials: Study design: Multicenterclinical chart review, setting: secondary care centers.Patients were recruited from November 2012 toJanuary 2015. Indolent mucormycosis was definedby pathological confirmation of mucormycosis andevidence of symptoms of paranasal sinus diseaselonger than 1 month. All patients underwentimaging, laboratory workup & surgical treatment.Surgical procedures consisted in external maxillaryapproach and endoscopic ethmoidectomy andantrostomy. All surgical specimens were evaluatedby a certified pathologist
Indolent mucormycosis of the paranasal sinus is anemerging entity of immunosuppressed andimmunocompetent patients. Single paranasal sinusdisease is a frequent presentation and should not beoverlooked as a differential diagnosis in thesepatients. Immunocompetent patients should only betreated surgically. More studies are needed to confirmour results.
Introduction: Mucormycosis refers to any fungalinfection by members of the order Mucorales, in theclass of Zygomycetes. Rhizopus, Mucor, Rhizomucorand species of Aspergillus are the most commonetiologic agents in the sinonasal cavity.Mucormycosis of the nasal cavity and paranasalsinuses is an uncommon infection with a rapidaggressive, life threatening course, often inimmunosuppressed patients, potentially fatal.Microscopically, these fungi demonstrate broad non-septate hyphae, thick walled with branching at rightangles. In contrast with this fulminant entity, there´sother form of mucormycosis known as chronic orindolent among immunosuppressed andimmunocompetent patients. Is limited, chronic (> 1month), less aggressive and frequently withinvolvement of single paranasal sinus disease withnon-specific nasal symptoms. This study describes 6cases of indolent mucormycosis.
1. Waizel-Haiat, Salomón, et al. "Mucormicosis rinocerebral invasoracrónica."Cirugía y Cirujanos . 2003; 71.2: 145-149.
2. Jung H, Park SK. Indolent mucormycosis of the paranasal sinus inimmunocompetent patients: are antifungal drugs needed?. JLaryngol Otol. 2013;127(09):872-5.
3. Manuel, Marín-Méndez Héctor, et al. "Síndrome de ápex orbitariocausado por mucormicosis orbitocerebral crónica e indolente:reporte de dos casos." An Orl Mex. 2005; Vol. 50. No. 1: 64-68.
4. Dooley, David P., et al. "Indolent orbital apex syndrome caused byoccult mucormycosis." J Neuroophthalmol. 1992; 12: 245-249.
5. Busaba, Nicolas Y., et al. "Chronic invasive fungal sinusitis: a report oftwo atypical cases.(Original Article)." Ear Nose Throat J. 2002; 81:462-467.
6. Ketenci, Ibrahim, et al. "Indolent mucormycosis of the sphenoidsinus." Am J Otolaryngol Head Neck Med Surg. 2005; 132: 341-342.
Erika Celis-Aguilar MDHospital Civil de CuliacanEmail: [email protected]
CASES
Fig 1A, 1B: Coronal and sagittal CT scan shows involvement of left maxillarysinus, left nasal cavity and left orbitary apex. 1C: pathology result confirmedmucormycosis with broad non-septate hyphae
Case
1C
ase
2
Fig 2A: Coronal CT scan showed findings compatible with fungus ball in leftmaxillary sinus pathology. 2B: PAS staining revealed mucormycosis, withright angle hyphae. 2C: Coronal CT scan of 2 years follow up
Fig. 3A: pathology report of mucormycosiswith broad non-septate hyphae
Case
3Fig 4A: Axial CT scan demonstrate occupation of left maxillary sinus andosteitis of the sinus walls. 4B: PAS staining; positive suggestive of sporesand hyphae that support Zygomycetes
Case
4
Fig 5A: Axial CT scan reported occupation of right nasal cavity, no erosion,no bone involvement, with occupation of left maxillary sinus. The mass ofright nasal cavity was reported as a polyp and the occupation of leftmaxillary sinus revealed mucormycosis. 5B: Histopathologic image ofGrocott-Gomori stain with abundant broad hyphae and positive staining.Coronal CT scan 1 year follow up (Fig 5C)
Case
5
Fig 6A: Coronal CT scan reported occupation of right maxillary sinus withenlargement of maxillary ostium and osteitis of lateral wall. 6B: Pathologyreported abundant hyphae compatible with mucormycosis at PAS staining.6C: Axial CT scan at one year follow up, which shows thickening of the rightmucosal of the maxillary sinus.
Case
6
TABLE 1. Clinical features, treatment and follow up.
Patient
numberSex Age
Immunocompetent
status
Anatomic
localization
Duration of
symptoms (m)Clinical features Treatment Follow up
1 F 38 Immunosuppressed
Rhino-orbital
Left maxillary
Left ethmoid
Left Orbit
1
Maxillary pain, proptosis, pupil
dilation, restriction of ocular
movements.
Acute facial pain
Left Caldwell Luc
Ethmoidectomy
Orbital exenteration
Liposomal Amphotericine 3gr
Posaconazol 45 days
Two years
follow up,
NED
2 F 61 ImmunosuppressedLeft maxillary
sinus6
Headache, facial pain, purulent
rhinorrhea, halitosis, cacosmia
Left Caldwell Luc
Amphotericin B 750 mg
2.5 years follow
up, NED
3 M 54 ImmunosuppressedRight maxillary
sinusNA Asymptomatic
Right Cadwell Luc
Posaconazol
Two years
follow up, NED
4 M 42 ImmunocompetentLeft maxillary
sinus24
Mucoid discharge, nasal
obstruction, headache.Left Caldwell Luc
One year
follow up, NED
5 M 77 ImmunocompetentLeft maxillary
sinus60
Contralateral nasal mass
(right nasal polyp) obstruction,
asymptomatic
Left Caldwell Luc
Resection of contralateral mass
One year
follow up, NED
6 M 54 ImmunocompetentRight maxillary
sinus8 Headache, cacosmia
Right Caldwell Luc (Caldwell Luc
a year after, no recurrence)
1.6 year,
asymptomatic
