Necrotizing fasciitis of the head and neck

ORIGINAL ARTICLE

NECROTIZING FASCIITIS OF THE HEAD AND NECK

Henning Wolf, MD, Maria Rusan, Hon BSc, Karin Lambertsen, MD, Therese Ovesen, MD, DMSci

Department of Otorhinolaryngology, Head and Neck Surgery, Aarhus University Hospital, Aarhus, Denmark.E-mail: [email protected]

Accepted 3 December 2009Published online 26 March 2010 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/hed.21367

Abstract: Background. Necrotizing fasciitis of the head

and neck is a rapidly progressing and life-threatening condi-

tion. The purpose of this study was to describe the patients

with a focus on clinical presentation, microbiology, treatment,

and prognosis.

Patients and Results. Seventeen patients (10 men; median

age, 54 years) were included. Nine patients underwent minor head

and neck surgery immediately prior to necrotizing fasciitis. The typ-

ical course was a quickly spreading erythema, pronounced tender-

ness, and severe pain. Imaging demonstrated diffuse swelling of

the soft tissue, poorly differentiated dilated fat layers, and subcuta-

neous gas. All patients underwent surgical debridement within 2

days, and received broad-spectrum antibiotics and hemodynamic

support, hyperbaric oxygen, and immunoglobulin. All patients sur-

vived, although 12 of 17 suffered sequelae.

Conclusions. Early diagnosis is of utmost importance.

Quickly spreading erythema and extreme pain in the affected

area serve as red flags. With the current intensive multimodal-

ity regimen, the mortality was zero, although 70% suffered

sequelae. VVC 2010 Wiley Periodicals, Inc. Head Neck 32:

1592–1596, 2010

Keywords: necrotizing fasciitis; head and neck; treatment;

mortality; sequelae

Necrotizing fasciitis is a rapidly progressingand life-threatening condition that typicallyinvolves necrosis of the superficial layers butmay extend to the deep fat and fascial layers.1

Early on in the disease process, an obliterativevasculitis with microthrombosis occurs at theborders of the infectious focus, accompanied byacute inflammation of the subcutaneous tissueand by edema of the skin and subcutaneous fatlayer, attributed to exudation. With progressionof this process the infected tissue becomes ne-crotic, and there is local intravascular coagula-tion. Bleeding and sepsis are also characteristicof later stages, along with myonecrosis. This isfrequently followed by a rapidly spreading ne-crosis of the superficial fascia, the subcutaneousfat layer, the nerves, the arteries, and the veins.The majority of patients develop sepsis within48 hours of symptom onset. Necrotizing fasciitisis of particular concern in the following clinicalspecialties: odontology, otology, orthopedic sur-gery, and general surgery. The mortality rate isbetween 20% and 60%.1–5 Necrotizing fasciitiswas described in the 18th century but was notclassified until 1952 after the grouping of Strep-tococci.1,4,5 Necrotizing fasciitis is a subgroup ofnecrotizing soft tissue infections, which alsoencompass clostridial gas gangrene, Fournier’sgangrene, necrotizing Vibrio infections, and an-aerobic cellulitis. Necrotizing fasciitis is subcate-gorized into type I infections, caused by non–Group A Streptococci, obligate anerobes, andEnterobacteriaccea, and type II infections,caused by Group A Streptococci.1,4,5

Correspondence to: T. Ovesen

VVC 2010 Wiley Periodicals, Inc.

1592 Head and Neck Necrotizing Fasciitis HEAD & NECK—DOI 10.1002/hed December 2010

Early diagnosis and surgical intervention arekey determinants of prognosis.1,3,4,8 However, thedrastic surgical intervention required for sur-vival often leaves patients with pronounceddisfigurement.2,5 In 2004 guidelines were intro-duced for the management of patients withnecrotizing fasciitis at the Department of Otorhi-nolaryngology, Head and Neck Surgery, AarhusUniversity Hospital, Denmark. These promotethe early use of diagnostic tools, such as radio-logic investigations, acute surgical debridement,broad-spectrum antibiotics, immunoglobulin, andhyperbaric oxygen (HBO) therapy. This is in linewith the guidelines used since 1999 by Rigshospi-talet, Copenhagen, Denmark.6,8–10

The purpose of this study was to describe aDanish population with necrotizing fasciitis ofthe head and neck, with regard to clinical mani-festations; microbiologic, serologic, and radio-logic findings; and treatment outcome in termsof complications, sequelae, and mortality.

PATIENTS AND METHODS

Patients registered with The International Sta-tistical Classification of Diseases and RelatedHealth Problems (ICD) ICD3 code DM725 Abetween 2002 and 2009 in the Danish nationalhospital registry, and who were treated at theDepartment of Otorhinolaryngology, Head andNeck Surgery, Aarhus University Hospital, wereincluded. Patient charts were reviewed for clini-cal manifestations; illness duration; time takento reach a diagnosis; serologic, microbiologic, andradiologic findings; treatment regimen; length ofhospitalization; and outcome in terms of compli-cations, sequelae, and mortality. Patients mistak-enly registered with ICD3 code DM725 A wereexcluded. One patient was excluded because,upon review of the chart, it was concluded thatthe patient actually had, not necrotizing fasciitis,but Lemierre’s disease.

RESULTS

Over the 7-year period of the study, a total of 17patients were treated for necrotizing fasciitis atthe Department of Otorhinolaryngology, Headand Neck Surgery, Aarhus University Hospital,Denmark, of which 10 were men. The medianage was 54 years (range, 18–82 years). Nine ofthe otherwise healthy patients had minor sur-gery in the head and neck region within 1 to 2

days of necrotizing fasciitis symptom onset. Ofthese, 3 had undergone tonsillectomy as a resultof peritonsillar abscess, 3 had lymph node biop-sies from the neck, 2 had an odontogen infec-tion, and 1 had undergone surgery for anendolaryngeal cyst. The remaining 8 patients,who were not operated on, had experiencedother symptoms in the head and neck regionjust prior to onset of necrotizing fasciitis. Fourof these patients had symptoms of acute tonsilli-tis/pharyngitis and 2 of peritonsillar/paraphar-yngeal abscesses, whereas 1 patient had awound on the ala of the nose, and anotherpatient had a furuncle on the right temple (seeFigure 1). Over the course of a day, all patientsdeveloped substantial erythema in the head andneck region, accompanied by edema and tender-ness, with severe pain upon palpation of theaffected area or movement of the head. Allpatients were diagnosed with necrotizing fascii-tis in <48 hours. Eight patients were trans-ferred from another hospital within a distanceof 120 km from Aarhus University Hospital. CTscanning demonstrated diffuse edema in theaffected area, along with dilated and poorly dif-ferentiated fat layers attributable to exudation.Eleven patients had evidence of subcutaneous

FIGURE 1. Patient with necrotizing fasciitis originating from a

furuncle in the right temple, shown 2 months after discharge.

Scarring is seen in particular around the right eye.

Head and Neck Necrotizing Fasciitis HEAD & NECK—DOI 10.1002/hed December 2010 1593

gas. The C-reactive protein values were between266 and 5142 nmol/L, with an average of 2889nmol/L (normal values, <75 nmol/L). Leukocytecounts ranged between 4.7 and 25.3 (normal val-ues, 3.0–10.0 � 109/L), and were characterizedby neutrophilia.

Fifteen of the 17 patients underwent surgicaldebridement within 24 hours of the diagnosis.The remaining patients underwent debridementwithin 48 hours of the diagnosis. All patientsreceived broad-spectrum antibiotics, at the latestupon admission to surgery. Thirteen patientswere managed in accord with the guidelinesimplemented since 2004 at our department; thusthey were initially treated with 2 g of carbape-nem, and thereafter with carbapenem (1 g � 3intravenously), ciprofloxacin (400 mg � 2 intrave-nously), clindamycin (600 mg � 3 intravenously),and c-globulin 25 g daily, for 3 consecutive days.The same 13 patients also received HBO therapy.The remaining 4 patients were all diagnosedprior to the implementation of the 2004 guide-lines and were treated with high-dose penicillin(6.6 g � 2 intravenously) along with metronida-zole (500 mg � 3 intravenously).

Microbiologic analysis was conducted for allpatients using tissue collected from the operativesite. The results are shown in Table 1. The pre-dominant isolate was Group A Streptococci. Only4 patients had not received intravenous antibiot-ics prior to having samples taken for culturing.

Table 2 shows the prognosis, in terms of sur-vival, complications, and sequelae. All patientssurvived. Complications occurred in a total of 11patients (65%). Thus, in the immediate periopera-tive period, 8 patients developed septic shock.Four of these patients had dysfunction of multipleorgans and were thus transferred to Rigshospita-let, Copenhagen, to receive specialized HBO ther-apy because Aarhus University Hospital is notequipped to provide this for unstable patients.

Other complications included deep veinthrombosis, pneumonia, pleural exudate and em-pyema, and subendocardial infarction.

Only 2 patients could manage without a res-pirator. The remaining 15 patients were on arespirator for a duration ranging from 1 to 18days. The duration of hospitalization rangedfrom 10 to 32 days, with a median stay of 22days. Twelve patients (70%) had post–necrotiz-ing fasciitis sequelae; 6 of these had disfiguringscars (Table 2). Two of these were later operatedon at the Plastic Surgery department. Withregard to neural deficits occurring in 6 patients,the affected nerves were: the accessory nerve,the hypoglossal nerve, and the marginal man-dibular branch of the facial nerve, althoughnone of the deficits resulted in a complete loss offunction of the nerve (Table 2). Finally, 3patients had psychiatric difficulties, in particu-lar depression, for which they received psychiat-ric or psychological therapy.

DISCUSSION

Our study supports the notion that necrotizingfasciitis of the head and neck is a rare condition,given that the incidence was 2 per 1 millioninhabitants, per year, in Denmark. Patients weregenerally in good health prior to necrotizing fas-ciitis, and were frequently male. The primary siteof infection was either the tonsils, the teeth, andtraumatic or surgical wounds in the head andneck area. The predominant isolates were Strep-tococcus species, including the nonhemolytic.Furthermore, necrotizing fasciitis was associatedwith a substantial increase in C-reactive proteinand neutrophilia. Symptoms developed over afew days and were characterized by a quicklyspreading erythema, edema, tenderness, andpain in the head and neck area. The extreme anddisproportionate pain present seemed to be

Table 1. Microbiologic analysis associated with necrotizing

fasciitis of the head and neck.

Microorganism No. of patients

Streptococci 8

Nonhemolytic Streptococci 6

Hemolytic Streptococci, Group C/G 1

Arcanobacterium hemolyticum 1

Staphylococcus aureus 1

Table 2. Prognosis of 17 patients with necrotizing fasciitis of

the head and neck.

Prognosis No. of patients (%)

Survival 17 (100%)

Complications 11 (65%)

Shock 8

Deep vein thrombosis 1

Pneumonia 4

Pleural exudate/empyema 4

Subendocardial infarction 1

Sequelae 12 (70%)

Scarring 6

Nerve damage 6

Psychiatric symptoms 3


pathognomonic and was likely based on neuralinvolvement.2,4 The diagnosis was supported to alarge degree by CT scans, with blurring of the fatlayers attributed to exudation and the presenceof gas in the soft tissues, although the final diag-nosis was performed during the surgical debride-ment. Treatment was commenced within 48hours of admission. The duration of hospitaliza-tion was relatively long, of which a considerablepart was spent in the intensive care unit on a res-pirator. The reason for the prolonged respiratoruse was initially to protect the airway, then overthe first few days postoperatively to allow thefrequent wound changes under general anesthe-sia, but most importantly to provide the possibil-ity to control ensuing complications. Theestablished guidelines were followed, and allpatients survived, although some were character-ized by relatively disfiguring and/or psychologicalconsequences.

In comparison with necrotizing fasciitis, ingeneral, the patient population of the currentstudy stands out in various respects.1,3

Whereas the age and sex distribution werein agreement with other studies, the absence ofpredisposing factors in our population was re-markable. Only 3 of the patients had comorbid-ities (ie, hypertension), and 2 werecharacterized by alcohol abuse. In the literature,cancer, heart–lung disease, diabetes mellitus,immunosuppression, and intravenous drugabuse are often listed as risk factors for necrot-izing fasciitis in general. However, on review ofthe somewhat sparse international literature onnecrotizing fasciitis of the head and neck region,it appears that these patients were, as in ourstudy, healthy, to a large degree, prior to necrot-izing fasciitis.5,8–14 Patients commonly hadexternal trauma of the skin, or had recentlyundergone surgical procedures, which is alsoconsistent with our observations,2,5,9,11 and theprimary focus of infection, as in our study, wasfrequently the tonsils or teeth.2,5,9–14

A 2-modality management (surgery and anti-microbial therapy) has not been sufficient toachieve a 100% survival rate among patientswith necrotizing fasciitis.11 Addition of intrave-nous immunoglobulin has reduced the mortalityin patients with severe Group A Streptococciinfections.4,15,16 The effect of immunoglobulin isprobably an inhibition of the super antigen ac-tivity related to exotoxins secreted by Group AStreptococci.15 Adjuvant HBO has also improvedthe outcome for patients with necrotizing fascii-

tis.6,11,17 The use of the same intensive multimo-dality regimen as that in our settings haspreviously been demonstrated to significantlyinfluence the mortality rate from 75% to 0%.8,10

Obviously, we thus strongly recommend the useof such a multimodality regimen.

In relation to the frequency of minor surgicalprocedures in the head and neck region, includ-ing the incidence of dental infections, acute ton-sillitis, and peritonsillar abscess in the generalpopulation, the risk of developing necrotizingfasciitis of the head and neck is extremelysmall. Early treatment with a sufficiently largedose of penicillin can probably not hinder thedevelopment of necrotizing fasciitis in somepatients. The majority of our patient populationreceived early systemic penicillin, and all of theidentified microorganisms showed maximal sen-sitivity to penicillin.

The pathogenesis of necrotizing fasciitis ispoorly described. A lack of Group A Strepto-cocci–specific antibodies predisposes to the de-velopment of necrotizing fasciitis, and thecondition probably arises as the result of a rela-tively large preponderance of viable Streptococciin comparison with the amount of specific anti-bodies.1 The large number of Streptococci, espe-cially Group A Streptococci, quickly synthesizelarge amounts of exotocin, which is the directcause of tissue injury.15,16 Moreover, there ispossibly an aberrant antigen–antibody reac-tion.1,15,16 The antibiotic treatment establishedby Rigshospitalet and implemented in ourdepartment is designed to be bacteriocidalagainst both Gram-positive and Gram-negativeaerobic and anaerobic bacteria. A protein syn-thesis inhibitor is included (clindamycin), thushalting the production of exotocin. The regimenalso promotes phagocytosis of antigen–antibodycomplexes.7 Early and extensive surgical de-bridement has been shown by several studies tobe the only significant parameter for a success-ful outcome, possibly attributable to removal ofthe necrotic tissue, a decrease of the bacterialload, and curbing of the biochemical diseasemechanisms.1–5,8,10,11

Preventing systemic involvement, which gen-erally ensues on the second or third day of thedisease, is essential for survival.1,7 Systemicinvolvement is characterized by symptoms ofseptic shock and organ dysfunction arising fromdisseminated intravascular coagulation. Hemo-dynamic and metabolic support is thus neces-sary. Surrounding non-necrotic tissue is thought

Head and Neck Necrotizing Fasciitis HEAD & NECK—DOI 10.1002/hed December 2010 1595

to be preserved by HBO therapy, given that theoxygen pressure displays a higher increase inthe surrounding infected tissue compared withnormal tissue under HBO conditions.18 Asidefrom returning the hypoxic tissue to normal oxy-gen conditions, thereby increasing its resistanceto exotocins, HBO therapy is hypothesized tohave a direct bactericidal effect, to activate leu-kocytes, and to promote angiogenesis and woundhealing. However, it should be taken into con-sideration that HBO therapy may cause compli-cations such as reversible myopia, barotrauma,pneumothorax, and cramps. Although thesesymptoms are rare in patients, careful observa-tion is needed for patients under treatment.

In conclusion, necrotizing fasciitis of thehead and neck is an extremely rare and life-threatening condition. The diagnosis should bemade within a day of symptom onset, so thatsurgical intervention can be undertaken as soonas possible and organ dysfunction prevented.Quickly spreading erythema, swelling, and rela-tively pronounced pain in the affected area arered flags. With the current diagnostic and multi-modality treatment protocol used at the Depart-ment of Otorhinolaryngology, Head and NeckSurgery, Aarhus University Hospital, we havesucceeded in preventing deaths attributed tonecrotizing fasciitis over a 7-year period.

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3. Simonart T. Group A beta-haemolytic streptococcalnecrotizing fasciitis: early diagnosis and clinical features.Dermatology 2004;208:5–9.

4. Seal DV. Necrotizing fasciitis. Curr Opin Infect Dis2001;14:127–132.

5. Fenton CC, Kertesz T, Baker G, et al. Necrotizing fascii-tis of the face: a rare but dangerous complication of den-tal infection. J Can Dent Assoc 2004;70:611–615.

6. Wang J, Li F, Calhoun JH, et al. The role and effective-ness of adjunctive hyperbaric oxygen therapy in themanagement of muscoloskeletal disorders. J PostgradMed 2002;48:226–231.

7. Heslet L. Retningslinier for nekrotiserende fasciit: visita-tion, behandling, monitorering og diagnostik. Rigshospi-talet 2005:1–7 (www.rh-vejledninger.dk).

8. Krenk L, Nielsen HU, Christensen ME. Necrotizing fas-ciitis in the head and neck region: an analysis of stand-ard treatment effectiveness. Eur Arch Otorhinolaryngol2007;264:917–922.

9. Benavides G, Blanco P, Pinedo R. Necrotizing fasciitis ofthe face: a report of one successfully treated case. Oto-laryngol Head Neck Surg 2003;128;894–896.

10. Nielsen HUK, Rasmussen N. Nekrotiserende fasciitis.Ugeskrift Læger 2000;162:1745–1747.

11. Lin C, Yeh F-L, Lin J-T, et al. Necrotizing fasciitis ofthe head and neck: an analysis of 47 cases. PlastReconstr Surg 2001;107:1684–1693.

12. Djupesland PG. Necrotizing fasciitis of the head andneck: report of three cases and review of the literature.Acta Otolaryngol 2000;543 (Suppl):186–189.

13. Maisel RH, Karlsen R. Cervical necrotizing fasciitis. La-ryngoscope 1994;104:795–798.

14. McMahon J, Lowe T, Koppel DA. Necrotizing soft tissueinfections of the head and neck: case reports and litera-ture review. Oral Surg Oral Med Oral Pathol OralRadiol Endod 2003;95:30–37.

15. Norrby-Teglund A, Muller MP, McGeer A, et al. Success-ful management of severe group A streptococcal soft tis-sue infections using an aggressive medical regimenincluding intravenous polyspecific immunoglobulin to-gether with a conservative surgical approach. Scand JInfect Dis 2005;37:166–172.

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Necrotizing fasciitis of the head and neck