Accepted Manuscript Title: To Exenterate or Not? An Unusual Case of Pediatric Rhinocerebral Mucormycosis Author: Sean Mutchnick Daniel Soares Mahdi Shkoukani PII: DOI: Reference:
S0165-5876(14)00643-0 http://dx.doi.org/doi:10.1016/j.ijporl.2014.11.028 PEDOT 7378
To appear in:
International Journal of Pediatric Otorhinolaryngology
Received date: Revised date: Accepted date:
21-7-2014 21-11-2014 21-11-2014
Please cite this article as: S. Mutchnick, D. Soares, M. Shkoukani, To Exenterate or Not? An Unusual Case of Pediatric Rhinocerebral Mucormycosis, International Journal of Pediatric Otorhinolaryngology (2014), http://dx.doi.org/10.1016/j.ijporl.2014.11.028 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
1
ip t
To Exenterate or Not?
cr
An Unusual Case of Pediatric Rhinocerebral Mucormycosis
Sean Mutchnick, M.D.1, Daniel Soares, M.D.1, Mahdi Shkoukani, M.D.1 1
an
us
Wayne State University, Department Of Otolaryngology - Head And Neck Surgery, Detroit, MI, USA
M
Running title: Rhinocerebral Mucormycosis: Exenterate or Not?
We wish to confirm that there are no known conflicts of interest associated with this publication
te
outcome.
d
and there has been no significant financial support for this work that could have influenced its
Ac ce p
Please direct correspondence to Sean Mutchnick, M.D. at [email protected], Wayne State University School of Medicine Department of Otolaryngology Head & Neck Surgery, 4201 St Antoine, 5E UHC, Detroit, MI 48201.
This case report was presented at the Combined Otolaryngology Spring Meetings 2014 in Las Vegas, NV during the American Society of Pediatric Otolaryngology poster session, May 16 & 17, 2014.
Page 1 of 14
2 Abstract
Rhinocerebral mucormycosis (RM) is a rare, potentially lethal fungal infection. Traditional teaching encourages aggressive surgical resection until viable bleeding tissue is encountered,
ip t
often leading to orbital exenteration, skull base resection, and cerebral debridement, in addition to systemic antifungal therapy. We present a 2yo male with acute lymphocytic leukemia
cr
undergoing chemotherapy presenting with RM and unilateral orbital and intracranial
involvement. After aggressive sinonasal debridement, systemic antifungal and hyperbaric
us
oxygen therapies, he recovered without need for further aggressive tissue resection. We report the successful management of invasive orbital and intracranial RM without orbital exenteration
Ac ce p
te
d
M
an
or cerebral debridement.
Page 2 of 14
3
Introduction Rhinocerebral mucormycosis (RM) is a rare and potentially lethal invasive infection
ip t
caused by saprophytic fungi of the class Zygomycetes, specifically of the Mucorales order. This disease most often develops in hosts made vulnerable by metabolic derangements such as
cr
diabetes, or, immunosuppression, as in hematological diseases [1,2]. Clinical manifestations
us
parallel the rapid progression from sinonasal infection to involvement of the orbit, skull base and brain. Sinusitis might be the earliest expected clinical presentation; the more ominous signs of
an
advanced disease largely reflect the fungi’s propensity for angioinvasion resulting in thrombosis and necrosis of affected tissues and direct extension to neighboring structures [1,2,3].
M
The early diagnosis and treatment of RM is paramount to decreasing morbidity and mortality. The treatment of RM commonly involves aggressive surgical debridement and
d
systemic antifungal therapy, along with correction of underlying metabolic abnormalities and
te
reversion of immunocompromised states, when possible [3,4]. The degree to which radical surgical debridement of infected tissue plays a role in the successful management of disease is
Ac ce p
unknown. Traditional teaching encourages aggressive surgical resection until viable, bleeding tissue is encountered [5,6,7]. This has often lead to orbital exenteration, skull base resection, and cerebral debridement due to the characteristically rapid involvement of these structures. The potential for great and permanent physical and mental disability resulting from such aggressive surgical management presents a significant challenge to the control of RM particularly in the pediatric patient.
Case Presentation A 2-year-old male was admitted to an outside facility with 1-day history of right eye swelling and found to be febrile, pancytopenic and septic. This was day 21 of induction chemotherapy for B-Cell Precursor Acute Lymphocytic Leukemia. He subsequently developed a small black eschar on his right medial canthus. One week later the patient was transferred to Page 3 of 14
4 our institution due to eschar progression and worsening post-septal cellulitis on repeat imaging. On initial examination there was significant swelling and erythema of the right periorbita with a black eschar in the area of the right medial canthus (Figure 1). There was minor periorbital swelling noted on the left and a midline ulcer was observed on the hard palate. The
ip t
initial computed tomography scan revealed pansinusitis without bony destruction, with pre- and post-septal cellulitis of the right orbit (Figure 2). Broad-spectrum antibiotics along with
cr
Amphotericin B, Posaconazole and Micafungin antifungal agents were started; Induction
us
chemotherapy was discontinued.
On hospital day (HD) 3 the patient underwent external incision and drainage of the right
an
orbital post-septal debris, debridement of the right medial canthus ulceration, right middle meatal antrostomy with removal of necrotic tissue and right anterior and posterior
M
ethmoidectomy with debridement of the nasal cavity. Due to expansion of the right medial canthal ulcer the patient returned to the operating room (OR) on HD 7. On endoscopy the right
d
inferior turbinate, the remaining middle turbinate and the superior turbinate and fovea area were
te
all found to be black and necrotic; These structures were debrided as much as possible. The septum was noted to be discolored but no tissue was removed at this time. Endoscopy showed
Ac ce p
the left nasal cavity to be filled with mucoid tissue and black, gangrenous appearing mucosa; No action was taken at this time. Debridement of the black, necrotic base of the medial canthal ulcer was carried down to bone, and the palatal ulcer was debrided until free bleeding occurred. Magnetic resonance imaging (MRI) on HD 8 demonstrated bilateral inferior-frontal cerebritis with persistence of orbital disease and suspected involvement of the fovea ethmoidalis especially on the right, the cribriform plate, the anterior clinoid and anterior body of the sphenoid (Figure 3). Upon return to the OR on HD 9, the right nasal cavity showed extensive signs of acute, invasive fungal sinusitis with extensive black crusting. The right maxillary antrostomy was revised with a right medial maxillectomy extending to the nasolacrimal duct anteriorly, the floor of the nasal vault inferiorly, and posteriorly to include takedown of the posterior maxillary wall as well as the orbital floor. On entering the infratemporal fossa, the adipose tissue displayed nonbrisk bleeding which suggested fungal invasion, but not complete destruction, and frozen Page 4 of 14
5 sections were suspicious for fungal hyphae. However, due to clinical suspicion of limited infratemporal involvement, repeat biopsy showed mature adipose tissue with no fungi. The remaining ethmoid cells were taken to complete a total ethmoidectomy, facilitating removal of the lamina papyracea and exposure of the orbit. Decompression of the orbit revealed necrotic
ip t
fat and allowed for biopsy of the orbital apex, which would also return positive for mucor infection. The sphenoid sinus was then debrided of necrotic tissue and dark, non-bleeding,
cr
necrotic bone was encountered throughout. The nasal cavity floor was necrotic and
us
debridement revealed dark, bony involvement, however it was not as dark as areas previously encountered. Biopsy from the hard and soft palate junction at the margin of debridement would
an
return negative for fungal elements.
From the left nasal cavity, it was observed that the superior and anterior septum was
M
necrotic necessitating total septectomy, extending anteriorly to within approximately one finger width of the columella where fresh, clean cartilage was encountered. The left middle turbinate
d
was necrotic and removed. After a left maxillary antrostomy the sinus appeared to be in good
te
condition with healthy bleeding. The pursuit of involved tissue lead to a left total ethmoidectomy and sphenoidotomy which showed healthy, bleeding mucosa anteriorly and necrotic tissue
Ac ce p
posteriorly which was removed. The palatal ulcer was further debrided, and biopsies returned positive for fungal involvement. At this time orbital exenteration had been deferred by the family and care team until the extent of intracranial disease could be further evaluated. On HD 11, MRI confirmed involvement of the inferior frontal lobes and base-of-skull and a palliative right orbital exenteration was planned with the hope that decreasing the fungal burden could affect the patients poor prognosis. However, on HD 14 healthy-appearing, bleeding tissue was encountered in the nasal cavity and yellow, bleeding fat was seen upon reentering the right orbit. Biopsy of the extra-conal fat and medial rectus muscle would return negative for invasive fungal disease. Recent imaging had demonstrated increased enhancement of the left maxillary sinus yet revision of the maxillary antrostomy revealed nothing of concern. There was continued bleeding without signs of invasive fungal disease throughout the left nasal cavity. The left medial orbital wall was decompressed, and biopsy of the left extraPage 5 of 14
6 conal fat returned negative for fungal disease as well. The palatal ulcer was further debrided of its necrotic base. The negative biopsies and unexpected improvement aborted the planned orbital exenteration. Management over the following 2 months consisted of serial debridement with
ip t
concomitant systemic antifungal and hyperbaric oxygen therapy, as well as management of the chronic right medial canthal wound. Recovery was complicated by osteomyelitis of both the
cr
palate and right maxillary facial skeleton. Serial MRIs demonstrated improvement in orbital and
us
intracranial disease; The disease was controlled and the patient recovered without the need for further aggressive tissue resection. The chronic right medial canthal wound involving the lower
an
eyelid was then reconstructed in coordination with oculoplastic surgery (Figure 4). At 6 months follow-up, the patient demonstrated normal globe function and binocular vision.
M
Discussion
d
Rhinocerebral mucormycosis remains a rare and highly lethal disease despite
te
substantial improvements in early diagnosis, antifungal and adjunct therapy over the last fifty years [1]. The combination of surgical and medical management has long been shown to lower
Ac ce p
mortality more than either alone [1,2,3,4]. Advancements in medical therapy, including greater optimization of poor medical states, allows consideration for limiting the extent of surgical debridement.
The principle of combination antifungal therapy and aggressive surgical debridement in the management of RM, as with all invasive fungal rhinosinusitis, is not age dependent [2,3,4,6]. The necrotic foci of infection resulting from the fungi’s pathogenic angioinvasion prevents therapeutic delivery of systemic antifungal therapy [2,3,7]. However, surgical debridement should be guided by the extent of invasion as well as severity of disease, characterized by the rate of progression [3,7]. Even in the presence of orbital involvement successful management has been reported without orbital exenteration due to the regression of early disease with medical therapy and decreased fungal burden [2,3,7,8]. Early antifungal therapy, discontinuation of the patient’s induction chemotherapy, Page 6 of 14
7 aggressive debridement and adjunct hyperbaric oxygen therapy were crucial in stabilizing the disease in this case of pediatric RM. Multimodal therapy managed by a multidisciplinary team of otolaryngology, intensive care, hematology/oncology and infectious disease professionals allowed for the successful management of extensive craniofacial disease without radical
ip t
surgical intervention. This case adds to a trend in the literature of leveraging multimodal
cr
therapies to limit the degree of radical surgery in the management of RM [3,8].
us
Conclusion
Despite the rarity and lethality of pediatric RM, this case presentation reports the
an
successful management of invasive orbital and intracranial RM without orbital exenteration or
Ac ce p
te
d
M
cerebral debridement.
Page 7 of 14
8
ip t
References [1] Nussbaum ES, Hall WA. Rhinocerebral Mucormycosis: Changing Patterns of Disease.
cr
Surg Neurol. 41 (1994) 152-56.
[2] Spellberg B, Edwards J, Ibrahim A. Novel perspectives on mucormycosis:
us
pathophysiology, presentation, and management. Clinical Microbiology Reviews.18(3) (2005) 556–69.
an
[3] Peterson KL, Wang M, Canalis RF, Abemayor E. Rhinocerebral Mucormycosis: Evolution of the Disease and Treatment Options. Laryngoscope. 107 (1997) 855-862.
M
[4] Däbritz J, Attarbaschi A, Tintelnot K, et al. Mucormycosis in paediatric patients:
e785–e788.
d
demographics, risk factors and outcome of 12 contemporary cases. Mycoses. 54 (2011)
te
[5] Gillespie MB, O’Malley BW, Francis HW. An approach to fulminant invasive fungal
Ac ce p
rhinosinusitis in the immunocompromised host. Archives of Otolaryngology - head & neck surgery. 124(5) (1998) 520–6. [6] Park AH, Muntz HR, Smith ME, et al. Pediatric invasive fungal rhinosinusitis in immunocompromised children with cancer. Otolaryngol Head Neck Surg. 133(3) (2005) 411–6.
[7] Talmi YP, Goldschmied-Reouven A, Bakon M. Rhino-orbital and rhino-orbito-cerebral mucormycosis. Otolaryngol Head Neck Surg. 127 (2002) 22-31. [8] Pelton RW, Peterson E, Patel BC, Davis K. Successful treatment of rhino-orbital mucormycosis without exenteration: The use of multiple treatment modalities. Ophthalmic Plastic and Reconstructive Surgery. 17(1) (2001) 62–6.
Page 8 of 14
9
Figure Legend
ip t
Figure 1: Right medial canthal wound on initial presentation. Figure 2: Initial CT prior to any surgical intervention.
cr
Figure 3a: MRI demonstrating inferior-frontal cerebral enhancement, prior to endoscopic resection.
us
Figure 3b: MRI demonstrating improvement of inferior-frontal cerebral enhancement, 3 months post-op.
an
Figure 4: Right medial canthal wound following reconstruction utilizing left paramedian forehead
Ac ce p
te
d
M
flap with cartilage graft to right inferior medial tarsal plate.
Page 9 of 14
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ce
pt
ed
M
an
us
cr
i
Figure 1
Page 10 of 14
Ac
ce
pt
ed
M
an
us
cr
i
Figure 2
Page 11 of 14
Ac ce p
te
d
M
an
us
cr
ip t
Figure 3a
Page 12 of 14
Ac ce p
te
d
M
an
us
cr
ip t
Figure 3b
Page 13 of 14
Ac ce p
te
d
M
an
us
cr
ip t
Figure 4
Page 14 of 14
S0165-5876(14)00643-0 http://dx.doi.org/doi:10.1016/j.ijporl.2014.11.028 PEDOT 7378
To appear in:
International Journal of Pediatric Otorhinolaryngology
Received date: Revised date: Accepted date:
21-7-2014 21-11-2014 21-11-2014
Please cite this article as: S. Mutchnick, D. Soares, M. Shkoukani, To Exenterate or Not? An Unusual Case of Pediatric Rhinocerebral Mucormycosis, International Journal of Pediatric Otorhinolaryngology (2014), http://dx.doi.org/10.1016/j.ijporl.2014.11.028 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
1
ip t
To Exenterate or Not?
cr
An Unusual Case of Pediatric Rhinocerebral Mucormycosis
Sean Mutchnick, M.D.1, Daniel Soares, M.D.1, Mahdi Shkoukani, M.D.1 1
an
us
Wayne State University, Department Of Otolaryngology - Head And Neck Surgery, Detroit, MI, USA
M
Running title: Rhinocerebral Mucormycosis: Exenterate or Not?
We wish to confirm that there are no known conflicts of interest associated with this publication
te
outcome.
d
and there has been no significant financial support for this work that could have influenced its
Ac ce p
Please direct correspondence to Sean Mutchnick, M.D. at [email protected], Wayne State University School of Medicine Department of Otolaryngology Head & Neck Surgery, 4201 St Antoine, 5E UHC, Detroit, MI 48201.
This case report was presented at the Combined Otolaryngology Spring Meetings 2014 in Las Vegas, NV during the American Society of Pediatric Otolaryngology poster session, May 16 & 17, 2014.
Page 1 of 14
2 Abstract
Rhinocerebral mucormycosis (RM) is a rare, potentially lethal fungal infection. Traditional teaching encourages aggressive surgical resection until viable bleeding tissue is encountered,
ip t
often leading to orbital exenteration, skull base resection, and cerebral debridement, in addition to systemic antifungal therapy. We present a 2yo male with acute lymphocytic leukemia
cr
undergoing chemotherapy presenting with RM and unilateral orbital and intracranial
involvement. After aggressive sinonasal debridement, systemic antifungal and hyperbaric
us
oxygen therapies, he recovered without need for further aggressive tissue resection. We report the successful management of invasive orbital and intracranial RM without orbital exenteration
Ac ce p
te
d
M
an
or cerebral debridement.
Page 2 of 14
3
Introduction Rhinocerebral mucormycosis (RM) is a rare and potentially lethal invasive infection
ip t
caused by saprophytic fungi of the class Zygomycetes, specifically of the Mucorales order. This disease most often develops in hosts made vulnerable by metabolic derangements such as
cr
diabetes, or, immunosuppression, as in hematological diseases [1,2]. Clinical manifestations
us
parallel the rapid progression from sinonasal infection to involvement of the orbit, skull base and brain. Sinusitis might be the earliest expected clinical presentation; the more ominous signs of
an
advanced disease largely reflect the fungi’s propensity for angioinvasion resulting in thrombosis and necrosis of affected tissues and direct extension to neighboring structures [1,2,3].
M
The early diagnosis and treatment of RM is paramount to decreasing morbidity and mortality. The treatment of RM commonly involves aggressive surgical debridement and
d
systemic antifungal therapy, along with correction of underlying metabolic abnormalities and
te
reversion of immunocompromised states, when possible [3,4]. The degree to which radical surgical debridement of infected tissue plays a role in the successful management of disease is
Ac ce p
unknown. Traditional teaching encourages aggressive surgical resection until viable, bleeding tissue is encountered [5,6,7]. This has often lead to orbital exenteration, skull base resection, and cerebral debridement due to the characteristically rapid involvement of these structures. The potential for great and permanent physical and mental disability resulting from such aggressive surgical management presents a significant challenge to the control of RM particularly in the pediatric patient.
Case Presentation A 2-year-old male was admitted to an outside facility with 1-day history of right eye swelling and found to be febrile, pancytopenic and septic. This was day 21 of induction chemotherapy for B-Cell Precursor Acute Lymphocytic Leukemia. He subsequently developed a small black eschar on his right medial canthus. One week later the patient was transferred to Page 3 of 14
4 our institution due to eschar progression and worsening post-septal cellulitis on repeat imaging. On initial examination there was significant swelling and erythema of the right periorbita with a black eschar in the area of the right medial canthus (Figure 1). There was minor periorbital swelling noted on the left and a midline ulcer was observed on the hard palate. The
ip t
initial computed tomography scan revealed pansinusitis without bony destruction, with pre- and post-septal cellulitis of the right orbit (Figure 2). Broad-spectrum antibiotics along with
cr
Amphotericin B, Posaconazole and Micafungin antifungal agents were started; Induction
us
chemotherapy was discontinued.
On hospital day (HD) 3 the patient underwent external incision and drainage of the right
an
orbital post-septal debris, debridement of the right medial canthus ulceration, right middle meatal antrostomy with removal of necrotic tissue and right anterior and posterior
M
ethmoidectomy with debridement of the nasal cavity. Due to expansion of the right medial canthal ulcer the patient returned to the operating room (OR) on HD 7. On endoscopy the right
d
inferior turbinate, the remaining middle turbinate and the superior turbinate and fovea area were
te
all found to be black and necrotic; These structures were debrided as much as possible. The septum was noted to be discolored but no tissue was removed at this time. Endoscopy showed
Ac ce p
the left nasal cavity to be filled with mucoid tissue and black, gangrenous appearing mucosa; No action was taken at this time. Debridement of the black, necrotic base of the medial canthal ulcer was carried down to bone, and the palatal ulcer was debrided until free bleeding occurred. Magnetic resonance imaging (MRI) on HD 8 demonstrated bilateral inferior-frontal cerebritis with persistence of orbital disease and suspected involvement of the fovea ethmoidalis especially on the right, the cribriform plate, the anterior clinoid and anterior body of the sphenoid (Figure 3). Upon return to the OR on HD 9, the right nasal cavity showed extensive signs of acute, invasive fungal sinusitis with extensive black crusting. The right maxillary antrostomy was revised with a right medial maxillectomy extending to the nasolacrimal duct anteriorly, the floor of the nasal vault inferiorly, and posteriorly to include takedown of the posterior maxillary wall as well as the orbital floor. On entering the infratemporal fossa, the adipose tissue displayed nonbrisk bleeding which suggested fungal invasion, but not complete destruction, and frozen Page 4 of 14
5 sections were suspicious for fungal hyphae. However, due to clinical suspicion of limited infratemporal involvement, repeat biopsy showed mature adipose tissue with no fungi. The remaining ethmoid cells were taken to complete a total ethmoidectomy, facilitating removal of the lamina papyracea and exposure of the orbit. Decompression of the orbit revealed necrotic
ip t
fat and allowed for biopsy of the orbital apex, which would also return positive for mucor infection. The sphenoid sinus was then debrided of necrotic tissue and dark, non-bleeding,
cr
necrotic bone was encountered throughout. The nasal cavity floor was necrotic and
us
debridement revealed dark, bony involvement, however it was not as dark as areas previously encountered. Biopsy from the hard and soft palate junction at the margin of debridement would
an
return negative for fungal elements.
From the left nasal cavity, it was observed that the superior and anterior septum was
M
necrotic necessitating total septectomy, extending anteriorly to within approximately one finger width of the columella where fresh, clean cartilage was encountered. The left middle turbinate
d
was necrotic and removed. After a left maxillary antrostomy the sinus appeared to be in good
te
condition with healthy bleeding. The pursuit of involved tissue lead to a left total ethmoidectomy and sphenoidotomy which showed healthy, bleeding mucosa anteriorly and necrotic tissue
Ac ce p
posteriorly which was removed. The palatal ulcer was further debrided, and biopsies returned positive for fungal involvement. At this time orbital exenteration had been deferred by the family and care team until the extent of intracranial disease could be further evaluated. On HD 11, MRI confirmed involvement of the inferior frontal lobes and base-of-skull and a palliative right orbital exenteration was planned with the hope that decreasing the fungal burden could affect the patients poor prognosis. However, on HD 14 healthy-appearing, bleeding tissue was encountered in the nasal cavity and yellow, bleeding fat was seen upon reentering the right orbit. Biopsy of the extra-conal fat and medial rectus muscle would return negative for invasive fungal disease. Recent imaging had demonstrated increased enhancement of the left maxillary sinus yet revision of the maxillary antrostomy revealed nothing of concern. There was continued bleeding without signs of invasive fungal disease throughout the left nasal cavity. The left medial orbital wall was decompressed, and biopsy of the left extraPage 5 of 14
6 conal fat returned negative for fungal disease as well. The palatal ulcer was further debrided of its necrotic base. The negative biopsies and unexpected improvement aborted the planned orbital exenteration. Management over the following 2 months consisted of serial debridement with
ip t
concomitant systemic antifungal and hyperbaric oxygen therapy, as well as management of the chronic right medial canthal wound. Recovery was complicated by osteomyelitis of both the
cr
palate and right maxillary facial skeleton. Serial MRIs demonstrated improvement in orbital and
us
intracranial disease; The disease was controlled and the patient recovered without the need for further aggressive tissue resection. The chronic right medial canthal wound involving the lower
an
eyelid was then reconstructed in coordination with oculoplastic surgery (Figure 4). At 6 months follow-up, the patient demonstrated normal globe function and binocular vision.
M
Discussion
d
Rhinocerebral mucormycosis remains a rare and highly lethal disease despite
te
substantial improvements in early diagnosis, antifungal and adjunct therapy over the last fifty years [1]. The combination of surgical and medical management has long been shown to lower
Ac ce p
mortality more than either alone [1,2,3,4]. Advancements in medical therapy, including greater optimization of poor medical states, allows consideration for limiting the extent of surgical debridement.
The principle of combination antifungal therapy and aggressive surgical debridement in the management of RM, as with all invasive fungal rhinosinusitis, is not age dependent [2,3,4,6]. The necrotic foci of infection resulting from the fungi’s pathogenic angioinvasion prevents therapeutic delivery of systemic antifungal therapy [2,3,7]. However, surgical debridement should be guided by the extent of invasion as well as severity of disease, characterized by the rate of progression [3,7]. Even in the presence of orbital involvement successful management has been reported without orbital exenteration due to the regression of early disease with medical therapy and decreased fungal burden [2,3,7,8]. Early antifungal therapy, discontinuation of the patient’s induction chemotherapy, Page 6 of 14
7 aggressive debridement and adjunct hyperbaric oxygen therapy were crucial in stabilizing the disease in this case of pediatric RM. Multimodal therapy managed by a multidisciplinary team of otolaryngology, intensive care, hematology/oncology and infectious disease professionals allowed for the successful management of extensive craniofacial disease without radical
ip t
surgical intervention. This case adds to a trend in the literature of leveraging multimodal
cr
therapies to limit the degree of radical surgery in the management of RM [3,8].
us
Conclusion
Despite the rarity and lethality of pediatric RM, this case presentation reports the
an
successful management of invasive orbital and intracranial RM without orbital exenteration or
Ac ce p
te
d
M
cerebral debridement.
Page 7 of 14
8
ip t
References [1] Nussbaum ES, Hall WA. Rhinocerebral Mucormycosis: Changing Patterns of Disease.
cr
Surg Neurol. 41 (1994) 152-56.
[2] Spellberg B, Edwards J, Ibrahim A. Novel perspectives on mucormycosis:
us
pathophysiology, presentation, and management. Clinical Microbiology Reviews.18(3) (2005) 556–69.
an
[3] Peterson KL, Wang M, Canalis RF, Abemayor E. Rhinocerebral Mucormycosis: Evolution of the Disease and Treatment Options. Laryngoscope. 107 (1997) 855-862.
M
[4] Däbritz J, Attarbaschi A, Tintelnot K, et al. Mucormycosis in paediatric patients:
e785–e788.
d
demographics, risk factors and outcome of 12 contemporary cases. Mycoses. 54 (2011)
te
[5] Gillespie MB, O’Malley BW, Francis HW. An approach to fulminant invasive fungal
Ac ce p
rhinosinusitis in the immunocompromised host. Archives of Otolaryngology - head & neck surgery. 124(5) (1998) 520–6. [6] Park AH, Muntz HR, Smith ME, et al. Pediatric invasive fungal rhinosinusitis in immunocompromised children with cancer. Otolaryngol Head Neck Surg. 133(3) (2005) 411–6.
[7] Talmi YP, Goldschmied-Reouven A, Bakon M. Rhino-orbital and rhino-orbito-cerebral mucormycosis. Otolaryngol Head Neck Surg. 127 (2002) 22-31. [8] Pelton RW, Peterson E, Patel BC, Davis K. Successful treatment of rhino-orbital mucormycosis without exenteration: The use of multiple treatment modalities. Ophthalmic Plastic and Reconstructive Surgery. 17(1) (2001) 62–6.
Page 8 of 14
9
Figure Legend
ip t
Figure 1: Right medial canthal wound on initial presentation. Figure 2: Initial CT prior to any surgical intervention.
cr
Figure 3a: MRI demonstrating inferior-frontal cerebral enhancement, prior to endoscopic resection.
us
Figure 3b: MRI demonstrating improvement of inferior-frontal cerebral enhancement, 3 months post-op.
an
Figure 4: Right medial canthal wound following reconstruction utilizing left paramedian forehead
Ac ce p
te
d
M
flap with cartilage graft to right inferior medial tarsal plate.
Page 9 of 14
Ac
ce
pt
ed
M
an
us
cr
i
Figure 1
Page 10 of 14
Ac
ce
pt
ed
M
an
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i
Figure 2
Page 11 of 14
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te
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M
an
us
cr
ip t
Figure 3a
Page 12 of 14
Ac ce p
te
d
M
an
us
cr
ip t
Figure 3b
Page 13 of 14
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te
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M
an
us
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ip t
Figure 4
Page 14 of 14
