Eur Arch Otorhinolaryngol DOI 10.1007/s00405-016-4148-5

CASE REPORT

Necrotising fasciitis of the paranasal sinuses Richmond Quan Qing Lim1 • Tze Choong Charn1 • David Loke1 • Jin Keat Siow2

Received: 3 May 2016 / Accepted: 13 June 2016 Ó Springer-Verlag Berlin Heidelberg 2016

Background Necrotising fasciitis (NF) is an uncommon and aggressive soft-tissue infection characterised by rapid spre ad of necrotising infection across the subcutaneous tissue and necrosis of the overlying skin. It usually affects the extremities, the genital area (Fournier’s gangrene), or the abdominal wall. Patients often have multiple co-morbidities, have vascular insufficiency, or are immunocompromised. Seemingly, harmless trauma, e.g., surgical wounds, or mild external injuries may precede the infection. Due to its rapidly spreading and potentially fatal nature, early diagnosis and treatment with antibiotics and surgery are paramount. NF of the paranasal sinuses is uncommon. Paranasal sinus NF may present only with facial symptoms, due to the relatively hidden location and anatomy of the paranasal sinuses. Accurate diagnosis, essential for effective treatment, may only be obtained after nasal endoscopy and CT or MRI imaging.

Science, and Scopus for ‘necrotising’, ‘necrotizing’, ‘fasciitis’, and ‘sinus’. A total of 268 hits were returned. These articles were screened for relevance by sequentially reviewing the journal titles, abstract titles, abstract content, and paper. The criteria used for screening were: (a) case reports or case series’ describing patients with necrotising fasciitis involving the paranasal sinuses and (b) papers written in English. 6 articles were selected for our review. The references for these articles were reviewed, and three additional papers were found to have relevant case reports. We also compare paranasal necrotising fasciitis to necrotising fasciitis, in general, and acute invasive fungal sinusitis. Articles identiied in Pubmed, Scopus, Web of Science, Science Direct. N = 268 Articles excluded due to languages other than English. N=5 Articles screened for inclusion. N = 263 Articles excluded after screening journal and abstract. N = 240

Methods We describe two cases of necrotising fasciitis of the paranasal sinuses. One case was seen each in two tertiary hospitals in Singapore. We then report a literature review of the condition. This was done by searching PubMed, Science Direct, Web of & Richmond Quan Qing Lim [email protected] 1

Department of Otorhinolaryngology, Khoo Teck Puat Hospital, Singapore, Singapore

2

Department of Otorhinolaryngology, Tan Tock Seng Hospital, Singapore, Singapore

Abstracts screened for inclusion. N = 20 Duplicates excluded. N=5 Full text retrieved for evaluation. N = 15 Articles excluded based on content of paper. N=9 Articles included after reviewing references. N=3

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Results Case 1 Mdm KHT is a 74-year-old Chinese lady with a history of lymphoplasmacytic lymphoma. She was treated for medial subperiosteal abscess of the right orbit initially responding well with antibiotic treatment. However, she developed a recurrence of pain and chemosis a month later. This was initially thought to be idiopathic orbital inflammatory disease, and she was treated with prednisolone. A week later, her eye symptoms worsened further. CT of the orbits revealed right pansinusitis with bony erosion and orbital abscess formation at the orbital apex on both sides of her optic nerve. She became blind in her right eye. Acute invasive fungal infection in an immunocompromised patient was suspected. She was started on broad-spectrum intravenous antibiotics and anti-fungals and underwent trans-nasal endoscopic drainage of the medial aspect of her right orbit. Pre-operatively, pus was noted lateral to her optic nerve. Drainage of this lateral collection by a transnasal endoscopic route would not have been possible. Her condition deteriorated further over a week, and a follow-up CT scan revealed worsening abscess formation at the lateral aspect of her orbit with adjacent air pocket formation (Fig. 1). She then underwent right orbital exenteration. Tissue and blood cultures grew Pseudomonas aeruginosa. Anti-fungals were stopped. She received IV antibiotics for 8 weeks in total and improved clinically. She was well at last follow-up 1 year later. Case 2 Mr FHH is a 48-year-old Chinese gentleman with a history of poorly controlled diabetes mellitus (DM). He presented with a 1-day history of left-eye swelling, loss of vision, and sepsis. CT scans revealed left pansinusitis, left orbital cellulitis, and extraconal gas locules (Fig. 2) . He underwent FESS and debridement of the orbit, and was started on broad-spectrum intravenous antibiotics and anti-fungals in view of the intraoperative findings of necrotic sinus tissue suspicious for invasive fungal sinusitis. Cultures grew Fusobacterium varium and Methicillin-sensitive Staphylococcus aureus. In view of worsening eye involvement clinically and radiologically, the patient was offered orbital exenteration, but declined repeatedly, despite understanding the risks. He subsequently underwent multiple debridements and endoscopic sinus surgeries, and eventually improved. Cellulitis and pus formation in this patient formed on the medial side of the orbit only, and were thus accessible for drainage via a trans-nasal endoscopic approach. He was well at last followup 5 months later.

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Fig. 1 Axial and coronal CT of Mdm KHT right pansinusitis with erosion of the right lamina papyracea and adjacent air pocket formation

A search on PubMed, Scopus, Web of Science, and Science Direct revealed nine publications describing 11 patients with necrotising fasciitis involving the paranasal sinuses. Including the two patients from our study, there were a total of 13 patients who were analysed. The majority (10/13) of patients were male. The median age was 44, the youngest was 13, and the oldest was 79 years old. Six patients had pre-existing immunocompromised states, including diabetes mellitus (n = 4), chronic alcoholism (n = 1), and history of treated lymphoma (n = 1). Two patients had pre-existing medical conditions that were possible sources of infection (one had an infected

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Twelve patients had CT scans performed. Eight had evidence of gas collection in the subcutaneous tissue. Only one patient had disease that extended down to the neck. Bacterial cultures revealed a polymicrobial infection in seven patients, Staphylococcus only infections in three patients, and a Gram-negative infection in one patient. All patients received multiple broad-spectrum intravenous antibiotics. Three patients received intravenous anti-fungal therapy as well. Eleven patients underwent sinus surgeries, either external approach or endoscopic, in addition to local wound debridement. One patient underwent orbital evisceration (defined as the removal of globe contents while leaving the sclera and extraocular muscles intact), and one patient underwent orbital exenteration (defined as the removal of all orbital content down to bone and part of the bony orbit). Seven patients underwent multiple operations for disease control. Two patients underwent hyperbaric oxygen therapy. Two patients succumbed to the disease. Two patients had residual ptosis and two had reduced visual acuity at last follow-up.

Discussion Pathophysiology of necrotising fasciitis

Fig. 2 Axial and coronal CT scans of Mr. FHH showing left pansinusitis, periorbital edema, and extraconal gas locules

alloplastic nasal implant and one had a history of chronic sinusitis and a conservatively managed ipsilateral orbital blowout fracture). Five patients presented after an identifiable triggering event. One occurred after blunt trauma to the orbit, two occurred after dental extraction, one occurred after forceful pulling of nasal hair, and one occurred after being started on short-course oral steroids. Nine patients presented with orbital symptoms. Three had nasal symptoms and one had dental complaints. Median duration of symptoms prior to diagnosis was 2 days and ranged between 1 h and 4 days (Tables 1, 2, 3).

Necrotising fasciitis is an aggressive, potentially lethal condition involving the rapid spread of soft-tissue infection across the fascial planes. It classically develops in the extremities and in the perineum, but rarely in the face [10]. The most common risk factor is diabetes mellitus, with a prevalence of 39–64 % in patients with NF [11–16]. Trauma, which could be iatrogenic in nature [10], is often the inciting event. NF is traditionally classified into four types. Type I includes polymicrobial infections with aerobes and anaerobes and commonly affects patients with multiple comorbidities or an immunocompromised state. Type 1 NF is responsible for 70–90 % of NF. Type II includes monomicrobial infections with Gram-positive organisms and commonly affects previously healthy patients. Type 2 NF is responsible for up to 20 % of cases. Types III and IV NF are rare. Type III NF describes Gram-negative organisms that are often marine-related. Type IV NF describes fungal NF [10, 17]. The pathogenesis of NF is related to the unique interaction between the characteristics of the host and the characteristics of the bacteria. Elderly and immunocompromised patients are at risk. Diabetes mellitus (DM) has been shown to impair host immunological mechanisms via multiple pathways. In vitro studies have proven decreased polymorphonuclear leukocyte mobilisation, chemotaxis and phagocytic activity, deficiencies in the complement

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References

Gurdal et al. [1]

Yang et al. [2]

Stone and Harshbarger [3]

Ng et al. [4]

Ng et al. [4]

Shindo et al. [5] Shindo et al. [5]

Swaminath et al. [6]

Gupta et al. [7]

Raboso et al. [8]

Bilbault et al. [9]

Case 1

Case 2

Case

1

2

3

4

5

6 7

8

9

10

11

12

13

48

74

44

79

50

42

44 46

13

30

16

45

33

Age

M

F

M

M

F

M

M M

M

M

M

F

M

Sex

DM

Lymphoplasmacytic lymphoma

Nil

DM

Nil

Hypertension

Alcoholism DM

Nil

History of chronic sinusitis, conservatively managed orbital blowout fracture

Nil

DM, previous alloplastic nasal implant

Nil

Medical background

Table 1 Background and presentation of patients with paranasal sinus NF

Nil

Short course steroid therapy

Dental extraction

Nil

Nil

Nasal hair pulling

Nil Dental extraction

Nil

Nil

Nil

Nil

Trauma to orbit

Inciting event

Eye swelling. Sepsis

Eye pain and swelling

Face and neck pain and swelling

Facial swelling, sepsis

Cheek and upper lip swelling

Eye pain and swelling. Nasal pain and congestion. Sepsis

Eye pain and swelling Eye pain, swelling and loss of vision. Purulent rhinorrhea

Eye pain and swelling. Sepsis

Toothache and cheek pain. Sepsis

Eye pain and swelling. Nasal pain and congestion. Sepsis

Eye pain, redness and pustules. Sepsis

Eye pain and swelling. Sepsis

Presenting

1 day

2 days

2h

1 day

3 days

3 days

2 days 2 days

2 days

12 h

4 days

1 day

1h

Duration of symptoms

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Gurdal et al. [1]

Yang et al. [2]

Stone and Harshbarger [3]

Ng et al. [4]

Ng et al. [4]

Shindo et al. [5]

Shindo et al. [5]

Swaminath et al. [6]

Gupta et al.7]

Raboso et al.8]

Bilbault [9]

Case 1

Case 2

1

2

3

4

5

6

7

8

9

10

11

12

13

References

Case

Eye: Staphylococcus epidermidis, Fusobacterium varium Blood: Fusobacterium varium

Sinus: MSSA, Fusobacterium varium

CT: left pansinusitis. Extensive gas pockets in soft tissue. Left orbital cellulitis

Sinus, eye, blood cultures: Pseudomonas aeruginosa

Propionibacterium

Staphylococcus epidermidis

Escherichia coli

Staphylococcus aureus

Streptococcus pyogenes

Haemophilus influenzae

Beta hemolytic Group A Streptococcus

Staphylococcus aureus

Pseudomonas

Beta hemolytic Group A Streptococcus

Nasal septum, eye: MRSA

Gram-positive bacillus

Enterobacter cloacae

Propionibacterium acnes

Peptostreptococcus

Porphyromonas

Prevotalla

Microaerophillic streptococcus

Staphylococcus epidermidis

Eye:

Blood: Staphylococcus, Streptococcus, Serratia, and yeast

Eye: Serratia

Eye: Streptococcus aginosus

Eye: Streptococcus aginosus

Anaerobic Gram-neg bacilli

Sinus: B-haemolytic Group F Streptococcus

Eye: Arcanobacterium hemolyticum

Sinus, eye, blood: MSSA

Sinus, eye: MRSA

Cultures

MRI: no cavernous sinus involvement

CT: right pansinusitis, lamina papyrecea erosion with air pocket formation. Right subperiosteal abscess compressing optic nerve

CT: right maxillary sinus floor fracture with soft-tissue thickening. Soft-tissue inflammation and air in right face, neck, and parapharyngeal space

CT: left maxillary sinusitis

CT: right maxillary sinusitis. Soft-tissue swelling of right cheek to temporal region. Thickening of deep and superficial fascia

MRI: right eye proptosis and superior ophthalmic vein dilation suggestive of cavernous sinus thrombosis

CT: right frontal sinusitis. Fluid and gas collection in anterior nasal cavity. Osteomyelitis of nasal septum

CT: gas in cheek soft-tissue and temporal fossa

CT: left maxillary fluid, mild ethmoid, and sphenoid mucosal disease. No orbital abscess

CT: left maxillary, ethmoid, sphenoid sinusitis. Left periorbital tissue swelling. Air in temporal fossa and posterior to globe

CT: left maxillary sinusitis. Air in tissue planes in temporal fossa. Left postseptal soft-tissue swelling. Old left orbital blowout fracture

CT: right frontal and ethmoid sinusitis. Right periorbital swelling with air pocket adjacent to frontal sinus

CT: left maxillary, ethmoid disease. Orbital apex edema and impingement of optic nerve

CT: right frontal sinus anterior table fracture. Right preseptal soft-tissue swelling

Imaging

Table 2 Imaging and microbiology results for patients paranasal sinus NF

I

III

I

I

I

II

I

I

II

I

II

II

II

Type

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123

Case 1

Case 2

12

13

Swaminath et al. [6]

8

Bilbault et al. [9]

Shindo et al. [5]

7

11

IV ceftriaxone ? IV gentamicin ? IV metronidazole

Shindo et al. [5]

6

Gupta et al. [7]

Ng et al. [4]

5

Raboso et al. [8]

Ng et al. [4]

4

9

Stone and Harshbarger [3]

3

10

IV Daptomycin ? topical gentamicin ? topical vancomycin (6 weeks)

Yang et al. [2]

2

IV amphotericin B ? IV piperacillin-tazocin then IV cefazolin ? IV metronidazole (8 weeks)

IV cefepime ? IV voriconazole ? PO metronidazole (8 weeks)

Cefotaxime ? gentamycin ? metronidazole

IV imipenem ? IV vancomycin

IV penicillin ? IV clindamycin ? IV gentamicin ? IV vancomycin ? IV amphotericin B (2 weeks)

IV aztreonam ? IV clindamycin ? IV ampicillinsulbactam ? IV imipenem ? IV vancomycin

IV benzylpenicillin ? IV chloramphenicol

IV cefuroxime ? IV metronidazole ? IV rifampicin

IV ampicillin-sulbactam ? IV vancomycin ? IV clindamycin

IV oxacillin ? IV ceftriaxone

IV crystalline penicillin ? IV 3rd Gen cephalosporins ? IV metronidazole (2 weeks)

Gurdal et al. [1]

1

Antibiotic choice (total duration)

References

Case

Table 3 Treatment and outcome of patients with paranasal sinus NF

Serial debridement of periorbital region

Serial FESS

Serial FESS Orbital exenteration

Serial debridement of face and neck

Maxillary sinus drainage

Debridement

Antral washout

Debridement

Nasal septal abscess drainage

Serial debridement

External approach ethmoidectomy, Caldwell Luc, frontal trephine

Serial debridement

Serial debridement

Lynch incision maxillary and ethmoid drainage and 2nd FESS

Debridement

Antral washout Orbital evisceration

Serial external approach ethmoidectomy and medial orbital wall debridement

Debridement of periorbital region

FESS, removal of implant

Debridement of periorbital region

External approach frontal curettage

Surgery

Nil

Yes

Nil

Nil

Nil

Cure

Cure

Cure

Death

Cure

Cure

Cure

Nil

Nil

Death

Nil

Cure

Cure

Nil

Nil

Cure

Cure

Cure

Outcome

Nil

Nil

Yes

HBOT

2m

12 m

Unknown

1w

4m

2m

Unknown

Unknown

5m

Unknown

Unknown

2m

2m

Duration of follow up

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activation of humoral immunity, diminished anti-oxidant burst capabilities, and lowered secretion of inflammatory cytokines in patients with DM. DM also results in microangiopathy and vascular insufficiency, leading to a higher prevalence of infections [18–20]. Other previously reported risk factors include alcoholism, peripheral vascular disease, chronic renal failure, liver dysfunction, heart disease, acquired immunodeficiency syndrome, malignancy, non-steroidal anti-inflammatory drug use, intravenous drug abuse, trauma, decubitus ulcer, chronic obstructive pulmonary disease, corticosteroid use, obesity, and malnutrition [21–23]. In Group A streptococcal (Type II) infections, bacterial toxins and enzymes induce coagulative necrosis, while a polysaccharide capsule inhibits phagocytosis. Bacteria can also cause vessel thrombosis, worsening tissue ischemia, and necrosis. Anaerobic bacteria thrive in anaerobic environments and may produce gas, giving rise to crepitus and appearance of gas pockets on radiology. Hence, the unique host-pathogen relationship results in a rapidly spreading infection. Comparison between paranasal sinus NF and NF in general Necrotising fasciitis of the paranasal sinuses is an uncommon condition with only 11 reported cases in the literature. Many authors have attributed the rarity of NF in the head and neck regions to the excellent blood supply to the area [8, 22]. We postulate that the scarcity of paranasal sinus NF may also be related to the excellent blood supply of the nasal cavity and sinuses. We also believe that there may be under-reporting of paranasal sinus NF, as not all cases of NF of the face undergo CT imaging, and nasal symptoms may not be as apparent in the presence of severe diffuse facial pain and swelling. Several cases of facial NF, where CT or MRI imaging was not part of the investigations, have been previously reported. A paranasal sinus source of infection cannot be excluded in such cases [23, 24, 34]. Martinschek et al. reported that 52.7 % of patients with NF had predisposing risk factors, while 27.3 % had DM [25]. Other authors have reported prevalence of DM as a risk factor to be between 39 and 64 % [11–16]. For patients with paranasal sinus NF, 46 % had one of the previously mentioned risk factors and 31 % had DM, matching statistics for necrotizing fasciitis in general. The majority of patients have a history of trauma preceding the infections. Yeung reported a case series, where 73 % of patients had either penetrating or blunt trauma before developing NF of the extremities [26], while in a systemic review, Angoules reported that 11 % of patients with NF of the extremities occurred spontaneously on intact skin [27]. In our review, trauma seems to be less

often an inciting factor for NF of the paranasal sinuses, with only 31 % of the patients experiencing prior trauma (trauma to orbit with subsequent anterior table frontal sinus fracture, dental extraction, and nasal hair pulling). Dental extraction though seemingly innocuous, has been known to cause maxillary sinusitis, especially in the event of a Schneiderian membrane perforation [28, 29]. The presence of DM can result in a more severe form of infection as seen in the patient described by Shindo [5]. Nasal hair pulling has also been implicated in furunculosis [30]. In Swaminath’s report, it led to septal abscess, NF of the anterior nasal space and frontal sinus and eventually led to cavernous sinus thrombosis. What was surprising is that the patient had no co-morbidities apart from hypertension, which was not considered as a risk factor, but a negative prognostic factor [13, 31]. This was also observed in the patient described by Bilbault [9], who was also previously healthy. Only 54 % of paranasal sinus NF was Type I. 38 % were Type II and 8 % were Type III. Compared to NF in general, paranasal sinus NF carries a higher proportion of Type II infections. It is possible that the different bacterial flora in the sinuses gives rise to this difference. In our review, many isolates grew Staphylococcus aureus, which corresponds to an increased proportion of Type II NF. Interestingly, one of the cases with Type I NF grew Staphylococcus epidermidis, Microaerophillic Streptococcus, Prevotalla, Porphyromonas, Peptostreptococcus, and Propionibacterium acnes, in addition to other bacteria. In a study by Brooks, these were also found to be the predominant growths in sinus swabs from patients having chronic rhinosinusitis with or without polyps [32]. Gas pockets seen on CT scans were reported in 54 % of the patients. This is echoed by Misiakos who reported that half had gas pockets seen on CT [10]. Treatment of NF, in general, involves aggressive surgical debridement and IV antibiotics. Multiple serial debridements may be necessary for complete clearance of the disease; radical surgery, including orbital evisceration or exenteration, should be carefully, but readily considered. Choice of empirical antibiotics depends on the type of NF. Subsequent antibiotic choice should be culture directed. This was also adhered to in our review of paranasal sinus NF. Recent advances have described the use of intravenous immunoglobulins (IVIG) in Type 2 NF, which aims to neutralise superantigens and exotoxins produced by Streptococcal and Staphylococcal antigens [10, 17, 33]. None of the patients in our review received IVIG, though as more than a third of the patients with paranasal sinus NF had Type 2 infections, IVIG should be strongly considered as part of treatment. Vacuum-assisted closure (VAC) dressings have also been used to encourage faster healing [10, 17]. Though the benefits are tangible, its application in

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paranasal sinus NF is less likely due to the intricate anatomy of the paranasal sinuses. There has also been a report of uncomplicated eyelid NF that was treated with antibiotics alone [34]. However, as most of the cases of paranasal sinus NF present late with facial swelling and pain after the infection has become more extensive, we feel that this is not a feasible option. Hyperbaric oxygen therapy (HBOT) was used in two patients with paranasal sinus NF. This has also been used as adjunctive therapy for NF, in general, with some centres reporting promising results [35–37], while others report no decrease in morbidity or mortality with HBOT [38, 39] In theory, increasing the oxygen tension in necrotic tissue reverses tissue ischemia, promotes angiogenesis, inhibits toxins, potentiates antibiotics effectivity, and increases leukocyte efficiency [40]. A recent Cochrane study published in 2015 was unable to make any recommendations due to lack of Randomised Controlled Trials [41]. Survival of patients with paranasal sinus NF is better than patients with NF, in general. A third of patients with NF, in general, do not survive [25, 26, 42], compared to mortality of 15 % of the patients in our review. This is despite a similar prevalence of negative prognostic factors, such as DM, and similar median age at presentation. This may be related to the rich vascularity of the face. The higher survivability may also be related to the higher proportion of Type II infections in paranasal sinus NF, which predominantly affects previously healthy patients. Comparison between paranasal sinus NF and acute invasive fungal sinusitis Three patients received anti-fungal therapy in addition to antibiotics. This is unsurprising given the similarity of the presentation of paranasal sinus NF to acute invasive fungal sinusitis. Although Type IV NF describes NF caused by fungal organisms, acute invasive fungal sinusitis is classically described as a separate entity. In the event of acute invasive fungal sinusitis, the definitive diagnosis is only made with histological confirmation of invasive fungal elements in sinus mucosa, submucosa, or bone, although anti-fungal treatment is often started as soon, as there is any suspicion of acute invasive fungal sinusitis [43]. Presentation of both diseases is similar with rapidly progressing symptoms of facial pain, swelling, and worsening vision that may occur within the span of hours. Acute invasive fungal sinusitis differs in that about a fifth of patients may also present with altered mental status or cranial nerve neuropathy suggestive of intracranial/skull base extension [44, 45], whereas none of the patients in our review presented with symptoms suggestive of intracranial involvement.

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Patients are less likely to be immunocompetent in acute invasive fungal sinusitis. According to Blitzer, only 4 % of patients had no predisposing risk factors [46]. About half had a history of DM, and half of the patients with DM had diabetic ketoacidosis at presentation [44, 45]. In addition to the immunocompromised state and microangiopathy that affects patients with DM as earlier described, an acidotic state in patients with DKA results in altered transferrin binding and increased serum free iron concentration. This was found to promote fungal growth, particularly that of the Mucor species [47]. Mucor species can also invade the vessels, resulting in thrombosis, further ischemia, and acidosis. Treatment is similar with the mainstay being intravenous anti-fungals and surgical debridement of necrotic tissue. Hyperbaric oxygen therapy has also been shown to be an effective adjunct in multiple case reports [48–50], though in the recently published European Society of Clinical Microbiology and Infectious Diseases (ESCMID) and European Confederation of Medical Mycology (ECMM) guidelines for management of mucormycosis, evidence for use of HBOT was marginal at best, due to the lack of data of substantial quality [51]. Survival of patients with paranasal sinus NF is fortunately much better than that of acute invasive fungal sinusitis. About half of the patients with acute invasive fungal sinusitis survived compared to 85 % of patients with paranasal sinus NF [44, 45]. This could be related to the higher proportion of patients with multiple co-morbidities and immunocompromised state in acute invasive fungal sinusitis.

Conclusion Paranasal sinus NF is an uncommon subset of necrotising fasciitis with only 11 cases reported in the literature thus far. A multi-disciplinary approach utilising high dose intravenous antibiotics and rigorous surgical debridement is the cornerstone of treatment. Intravenous immunoglobulin administration and hyperbaric oxygen therapy may have a role in adjunctive management, although definite benefits are yet to be proven in evidence-based medicine. Owing to the distinct microbiological environment of the paranasal sinuses, Type II necrotising fasciitis is more common. The rich vascularity of the paranasal sinuses may possibly contribute to the improved outcomes. Despite relatively high cure rates, life-saving but aggressive surgical debridement can lead to large soft-tissue defects, facial deformities, and negative socio-economic outcomes in patients.

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