419

QUERESHY ET AL

J Oral Maxillofac Surg

67:419-423, 2009

Report of a Case of Cervicothoracic

Necrotizing Fasciitis Along With a
Current Review of Reported Cases
Faisal A. Quereshy, MD, DDS,* Jonathan Baskin, MD,
Anca M. Barbu, MD, and Marc A. Zechel, MD, DDS
Cervical necrotizing fasciitis (CNF) is an infection that
spreads along the fascial planes causing subcutaneous
tissue death hallmarked by rapid progression and systemic toxicity. The infection is relatively rare in the
head and neck compared with other reported areas of
the body, including the extremities, abdominal fascia,
and perineum. Although the term CNF is somewhat
loosely used as there are no definite criteria for diagnosis, it is generally agreed upon that the presence of
a rapidly evolving polymicrobial infection in a susceptible host (ie, immunocompromised) with suspicious
radiographic and clinical signs is a clear indication for
imminent surgical intervention. On the other hand, a
substantial number of cases present in the otherwise
healthy individual with equivocal radiographic signs.
Also, a number of criteria used to characterize the more
usual localized head and neck infection (eg, leukocytosis, fever, signs of inflammation) tend to overlap with
what is found in CNF cases. Nevertheless, it is clear that
once the diagnosis is suspected, prompt surgical as well
as medical intervention is warranted.

*Assistant Professor and Residency Program Director, Department

of Oral and Maxillofacial Surgery, Case School of Dental Medicine and
University Hospitals Case Medical Center, Cleveland, OH.
Associate Professor and Attending Surgeon, Department of Otolaryngology and Head and Neck Surgery, University Hospitals of
Cleveland, Cleveland, OH.
Resident, Department of Otolaryngology and Head and Neck
Surgery, University Hospitals of Cleveland, Cleveland, OH.
Chief Resident, Department of Oral and Maxillofacial Surgery,
Case School of Dental Medicine and University Hospitals Case
Medical Center, Cleveland, OH.
Address correspondence and reprint requests to Dr Quereshy:
Department of Oral and Maxillofacial Surgery, Case School of Dental
Medicine and University Hospitals Case Medical Center, Case Western
Reserve University, 2123 Abington Road, 10900 Euclid Avenue, Cleveland, OH 44106-4905; e-mail: quereshyoms@hotmail.com
2009 American Association of Oral and Maxillofacial Surgeons

0278-2391/09/6702-0028$36.00/0
doi:10.1016/j.joms.2008.07.017

A significant number of cases of CNF of odontogenic

origin have been reported. These infections appear to
be caused by the same species of bacteria that cause
chronic dental subapical infections, acute localized cellulitis, and tissue abscesses. Typically, there is the recurring theme of the individual that is affected by CNF to be
systemically compromised by either frank immunosuppression, or a multitude of other factors including extremes of age, alcoholism, and diabetes.
The following report aims to present an interesting
case of CNF of odontogenic origin, which rapidly
evolved to the mediastinum, thorax, and great vessels
of the heart, ultimately leading to the patients demise
in short course despite aggressive treatment. In addition, we will review a number of other cases in the
literature as well as current modalities of therapy.

Report of a Case
The following patient was a 52-year-old female with a
past medical history significant for diabetes, alcoholism,
tobacco smoking, hypertension, and status post-Whipple
procedure in 1993 for a benign pancreatic cyst. She was
found confused and lethargic in her home by her daughter, and was transported to a nearby outside hospital by
emergency medical services. A computed tomography
(CT) scan at that time showed significant subcutaneous
emphysema of the anterior neck and mediastinum tracking along the great vessels (Fig 1). Through discussion
with the family, it was found out that over the previous
week the patient had been complaining of some lower
jaw and tooth pain, and was started on amoxicillin by her
primary care physician. She eventually presented to a
dentist who noted that her lower right wisdom tooth
should be extracted secondary to decay, and she was to
remain on antibiotics. Due, at least in part, to her lack of
insurance, she delayed subsequent treatment. Initial laboratory data were significant for a normal white blood
cell count with substantial neutrophil bandemia of 39%.
There was a marked increase in creatine kinase of 1,454
U/L (cf. 0-215 U/L reference range). Her metabolic panel
showed that she was hyperglycemic (435 mg/dL), with
an elevated blood urea nitrogen of 50 mg/dL. She was
febrile, with 38.8C and tachycardic. By the time consultation to our service was made, the patient was intubated
and in the medical intensive care unit. She was on anti-

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UNIQUE CASE OF CERVICOTHORACIC NECROTIZING FASCIITIS

FIGURE 1. Composite of CT axial images with intravenous contrast. Note profound tissue emphysema from inferior spread of the infection.
Infection is also noted around the great vessels.
Quereshy et al. Unique Case of Cervicothoracic Necrotizing Fasciitis. J Oral Maxillofac Surg 2009.

biotic therapy consisting of vancomycin, Imipenem, and

clindamycin. On examination there was notable supraclavicular dusky tissue with marked crepitation, and an area
of erythematous demarcation at approximately the nipple line inferiorly (Fig 2). No frank areas of necrosis were
notable. After joint consultation with cardiothoracic sur-

gery, it was deemed appropriate to urgently bring the

patient to the operating room for extensive debridement.
Upon initial entry into the skin, it was noted there was no
bleeding of the subcutaneous tissues or the platysma
muscle (Fig 3). A fetid odor with purulence was unmistakable throughout. An apron incision was used to identify the platysma muscles bilaterally as well as the ster-

FIGURE 2. Preoperative photograph of patients gross appearance. There is significant duskiness and erythema along the maxillofacial and cervicothoracic area.

FIGURE 3. Intraoperative photograph showing markedly necrotic

tissue.

Quereshy et al. Unique Case of Cervicothoracic Necrotizing Fasciitis. J Oral Maxillofac Surg 2009.

Quereshy et al. Unique Case of Cervicothoracic Necrotizing Fasciitis. J Oral Maxillofac Surg 2009.

421

QUERESHY ET AL

Table 1. FACTORS ASSOCIATED WITH REPORTED CASES OF CERVICAL NECROTIZING FASCIITIS

Demographics:
n
Mean age
Gender male:female
Comorbidities % (n):
Smoking
Alcoholism
Diabetes
HIV
Elderly (65 years)
Malignancy
Other
Etiology % (n):
Odontogenic
Surgery/trauma
Tonsillar

Tung-Yiu et al
(2000)1

Ali et al
(1997)2

Whitesides et al
(2000)3

Lin et al
(2001)4

Umeda et al
(2003)5

Bahu et al
(2001)6

11
60.5
7:4
ND
ND
36% (4)
0
18% (2)
9% (1)
36% (4)

3
56
1:2
ND
ND
33% (1)
0
33% (1)

11
47
7:4
ND
36% (4)
45% (5)
18% (2)
36% (4)

47
57
39:8
49% (23)

9
52.5
3:6
ND
ND
22% (2)
0
22% (2)
0

10
46
7:3
70% (7)
70% (7)
20% (2)
0
0
0

100% (11)*

33% (1)
66% (2)

100% (11)*

100% (9)*

80% (8)
20% (2)

26% (12)
72% (34)
0
47% (22)
13% (6)
11% (5)
36% (17)

Abbreviations: ND, not determined or reported; HIV, human immunodeficiency virus.

*In these studies only cases of odontogenic origin were reported.
Quereshy et al. Unique Case of Cervicothoracic Necrotizing Fasciitis. J Oral Maxillofac Surg 2009.

nocleidomastoid muscles; these were both excised due

to necrosis. Posteriorly, the infection involved to level 5,
including the trapezius muscle. The majority of the thyroid was necrotic and therefore removed; however, the
trachea and esophagus were preserved. There was involvement of the parapharyngeal spaces to the level of
the skull base posteriorly. The mediastinum was entered
and multiple Penrose drains were placed. The entire
wound was irrigated with bacitracin solution and packed
with Sulfamylon-soaked gauze. The patient was returned
to the surgical intensive care unit where she remained
intubated and nonresponsive.
Cultures obtained during the procedure eventually yielded
penicillin-sensitive Streptococcus viridans group. Cultures for
fungus and mycobacteria were negative.
The patient remained on the above antibiotics as recommended by the Infectious Diseases consult service, and we
continued sulfamylon dressing changes to the surgical site
every 2 hours. She had remained hemodynamically stable
for the ensuing 24 hours, at which point we obtained a
repeat CT that showed bilateral lung infiltrates; however,
there was persistent subcutaneous emphysema in the oropharynx and neck.
On postoperative day 2, the patient was hypotensive
despite multiple vasopressors. She was still febrile, and her
arterial blood gas profile was consistent with respiratory
failure. Coagulation studies were suggestive of hepatic insult and disseminated intravascular coagulation (DIC). A
discussion was held with the family in regard to her expected dismal prognosis. The decision was made to withdraw care, and she expired due to multiple organ failure.

Literature Review
The literature was searched for publications over
the last decade reporting encounters of CNF. Ninetyone cases from 6 different institutions were identified.1-6

DEMOGRAPHICS AND COMORBIDITIES

The data are summarized in Table 1. Of note is that

the average patient was in his or her midlife of age. In 4
of the 6 studies, there was a near 2-fold male predilection. Only 2 studies reported the incidence of tobacco
smoking among the patients, and for this, 49% to 70%
was the reported incidence. Alcoholism was reported in
3 studies, with an incidence of 26% to 70%. Diabetes
was a factor in 20% to 72% of the patients. There were
relatively few patients infected with human immunodeficiency virus (HIV). The elderly (65 years of age)
comprised 18% to 47% of patients.
HOSPITAL COURSE

In Table 2, the data from the studies are presented.

From a microbiological standpoint, oral streptococcal
species were cultured from most infections. Staphylococcal and anaerobic organisms were also occasionally identified. The most frequent complication reported was mediastinitis (13%-59%). Septic shock was
noted in 18% to 36% of patients. The mean days of
hospital stay were between 24 and 33.3 days. Mortality ranged from 0% to 33.3%.

Discussion
We reported a case of CNF that was secondary to
an odontogenic infection. Without any doubt, the
patients prognosis was worsened by the fact that she
presented with poorly controlled diabetes mellitus,
alcoholism, and tobacco abuse. The avoidance of definitive care until there was systemic involvement (ie,

422

UNIQUE CASE OF CERVICOTHORACIC NECROTIZING FASCIITIS

Table 2. IDENTIFIED ORGANISMS AND PROGNOSIS IN REPORTED CASES OF CERVICAL NECROTIZING FASCIITIS

Cultures:
Streptococcus
-hemolytic strep
-hemolytic strep
gp D streptococcus
Peptostreptococcus
Staphylococcus sp.
Eikenella sp.
Prevotella sp.
Major complication(s):
Mediastinitis
Septic shock
DIC
Pleural effusion
Hospital stay (mean days):
Mortality

Tung-Yiu et al
(2000)1

Ali et al
(1997)2

Whitesides et al
(2000)3

82% (9)
27% (3)

100% (3)
33% (1)

4
2
2

Lin et al
(2001)4

13%
15%*

Umeda et al
(2003)5

Bahu et al
(2001)6

78% (7)
55% (5)
33% (3)

100% (10)
20% (2)
60% (6)

11% (1)

20% (2)
60% (6)

64% (7)
2
4
ND
36% (4)
18% (2)
9% (1)
18% (2)
31.6
18% (2)

33.3% (1)

42
33.3% (1)

0
13% (6)
36% (17)

31
0%

26.4
25.5%

59% (5)

33.3
0%

24
10%

Abbreviations: DIC, disseminated intravascular coagulation; sp, species.

*Does not include the 15% of cases that were reported as mixed infection including staphylococcus and pseudomonas.
Quereshy et al. Unique Case of Cervicothoracic Necrotizing Fasciitis. J Oral Maxillofac Surg 2009.

she was found down) was also more likely than not to
have complicated the outcome.
The literature review substantiated the findings in our
patient (Table 1). Namely, that diabetes is a known
predisposing factor for CNF. Presumably this is due to
the defect in leukocyte function brought about by
chronic hyperglycemia. There is a clear association between alcoholism, tobacco abuse, and CNF. It is unclear
if this is a direct effect of these agents or a consequence
of the malnutrition and poorer general health often
observed in these patients. In terms of patients with
overt immunosuppression (eg, HIV-positive), it is clear
that these patients make up a small proportion of the
overall number of CNF cases. Whether this is from a
more acute awareness of HIV-infected patients toward
dental care, the placement of many of these patients on
prophylactic antibiotics, or a testament to the efficacy of
current HIV treatment strategies is unclear. In most of
the cases reviewed, there was a clear male predilection.1,3,4,6 This may be due to lifestyle and social factors
that predispose one to CNF.
This review suggests that the presence of septic
shock is associated with mortality from CNF (Table 2).
The presence of mediastinitis may also be a determinant
for survival for example, in the study by Lin et al,4 where
mediastinitis was associated with a 50% mortality. The
degree of thoracic involvement appeared to also correlate with the patients mortality.6 Typically, CNF will
spare the muscles because of their inherent vascularization. It was of interest, therefore, that in our patient the
muscle tissue was deeply necrotic at the time of initial
debridement. Whether depth of invasion of necrosis is a
clear prognostic factor would require further study.

Once CNF is recognized or, at least suspected,

treatment should be targeted toward achieving hemodynamic resuscitation, institution of immediate broadspectrum antibiotics, and surgical debridement. The
patient should typically be admitted to intensive care
unit for frequent monitoring, wound checks, triple antibiotic therapy, central monitoring to assess fluid status,
and hemodynamic parameters. Adjunctive therapy including hyperbaric oxygen (HBO) has been advocated.
It is theorized that HBO works at various levels to aid
in clearing the infection. When tissue oxygen tension
was measured in patients undergoing HBO for necrotizing infections by Korhonen et al,7 it was found that
partial oxygen concentrations rose as high as 7-fold in
the vicinity of the infection. Indeed, in 2 studies, patients with CNF who received HBO as adjunctive therapy all survived their infections. There is a need for a
randomized control trial using HBO to further elucidate
its role in treating CNF. Intravenous immunoglobulin
(IVIG) administration has also been shown to potentially
be helpful in streptococcal toxic shock syndrome.8
A relatively new technique whereby percutaneous
catheters were placed under fluoroscopy to provide
drainage to CNF infections has recently been reported.9
In this study 21 patients survived in the percutaneous
group. The limitation to this study was the diagnostic
criteria used for CNF. The authors relied on clinical and
particularly CT data instead of the traditional standard of
surgical exploration. Also, it is unclear how necrotic
tissue is to be debrided with this new technique.
In our patient, we did not obtain magnetic resonance
imaging (MRI) data. CNF may present thickening with
enhanced signal intensity of the deep fascial planes on

423

SCHMIDT, BRADRICK, AND GABALI

T2-weighted images. However, a number of reports10

suggest that MRI is incapable of differentiating CNF from
nonnecrotizing infection patterns (eg, cellulitis), and
prompt surgical treatment should not be delayed by
waiting for MRI data that may prove inconclusive. The
reported sensitivity and specificity for the detection of
necrotizing fasciitis from 2 studies is 89% to 100% and
46% to 86%, respectively.
It is clear that patients who develop CNF spend a
substantial number of days in the hospital (Table 2). The
mean hospital days spent was between 24 and 42, with
a large number of these spent in the ICU. Prompt identification of the patient at risk for this disease process as
well as aggressive management is critical. What remains
is a methodology to identify the presence of CNF versus
cellulitis or abscess. Clearly, such identification would
be key to the reduction of morbidity, mortality, as well
as hospital resources.

References
1. Tung-Yiu W, Jehn-Shyun H, Ching-Hung C, et al: Cervical necrotizing fasciitis of odontogenic origin: A report of 11 cases.
J Oral Maxillofac Surg 58:1347, 2000

2. Ali MH, Zaued ME: Necrotizing fasciitis of the head and neck:
Report of 3 cases. Ann Saudi Med 17:641, 1997
3. Whitesides L, Cotto-Cumba C, Myers RAM: Cervical necrotizing
fasciitis of odontogenic origin: A case report and review of 12
cases. J Oral Maxillofac Surg 58:144, 2000
4. Lin C, Yeh FL, Lin JT, et al: Necrotizing fasciitis of the head and
neck: An analysis of 47 cases. Plast Reconstr Surg 107:1684,
2001
5. Umeda M, Minamikawa T, Komatsubara H, et al: Necrotizing
fasciitis caused by dental infection: A retrospective analysis of
9 cases and a review of the literature. Oral Surg Oral Med Oral
Pathol Oral Radiol Endod 95:283, 2003
6. Bahu SJ, Shibuya TY, Meleca RJ, et al: Craniocervical necrotizing fasciitis: An 11-year experience. Otolaryngol Head Neck
Surg 125:245, 2001
7. Korhonen K, Kuttila K, Niinikoski J: Tissue gas tensions in
patients with necrotizing fasciitis and healthy controls during
treatment with hyperbaric oxygen: A clinical study. Eur J Surg
166:530, 2000
8. Cawley MJ, Briggs M, Haith LR Jr, et al: Intravenous immunoglobulin as adjunctive treatment for streptococcal toxic shock
syndrome associated with necrotizing fasciitis: Case report and
review. Pharmacotherapy 19:1094, 1999
9. Nakamori Y, Fujimi S, Ogura H, et al: Conventional open
surgery versus percutaneous catheter drainage in the treatment
of cervical necrotizing fasciitis and descending necrotizing
mediastinitis. AJR 182:1443, 2004
10. Arslan A, Pierre-Jerome C, Borthne A: Necrotizing fasciitis:
Unreliable MRI findings in the preoperative diagnosis. Eur J
Radiol 36:139, 2000
J Oral Maxillofac Surg
67:423-427, 2009

Hyperparathyroidism-Jaw Tumor
Syndrome: A Case Report
Brian P. Schmidt, MS, DMD,* Jon P. Bradrick, DDS, and
Ali Gabali, MD, PhD
Hyperparathyroidism-jaw tumor (HPT-JT) syndrome is
a rare multitumor syndrome that includes primary
hyperparathyroidism, fibro-osseous lesions of the
mandible and maxilla, renal tumors and cysts, and
uterine tumors.1,2 It was first reported by Jackson3 in
1958. Since then, multiple cases have been identi-

Received from MetroHealth Medical Center, Cleveland, OH.

*Resident, Division of Oral and Maxillofacial Surgery.
Chairman and Program Director, Division of Oral and Maxillofacial Surgery.
Senior Resident, Department of Pathology.
Address correspondence and reprint requests to Dr Schmidt:
Division of Oral and Maxillofacial Surgery, MetroHealth Medical
Center, 2500 MetroHealth Drive, Cleveland, OH 44109; e-mail:
bschmidt@metrohealth.org
2009 American Association of Oral and Maxillofacial Surgeons

0278-2391/09/6702-0029$36.00/0
doi:10.1016/j.joms.2008.07.015

fied.2,4-13 This syndrome has an autosomal dominant

pattern of inheritance, and is linked to a mutation in
the gene HRPT2 on the long arm of chromosome 1
that encodes for a protein parafibromin.4,5 Although
the exact function of parafibromin is currently unknown, it is believed to be involved in tumor suppression.5
We present a case of HPT-JT syndrome in which the
patient initially presented with multiple mixed radiopaque-radiolucent lesions of the maxilla and mandible, 1 of which was diagnosed via biopsy as a cementoossifying fibroma. Laboratory analysis showed that the
patient had an elevated serum intact parathyroid hormone (PTH) level, but normal calcium levels. Further
testing showed that the patient had a coexisting vitamin D deficiency. After correction of this vitamin D
deficiency, PTH levels remained elevated and the patient was diagnosed with primary hyperparathyroidism. He underwent excision of a parathyroid adenoma, which resulted in normal postoperative PTH