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Fever and Stiff Neck
Sara C. McIntire, MD;
Michael Green, MD, MPH
Arch Pediatr Adolesc Med. 2001;155:603-606.
Clinical Presentation: An 11-month-old boy
was seen in the emergency department by his pediatrician for evaluation of
fever (body temperature, 38.5°C) and a stiff neck. The patient's illness
began 5 days earlier when he developed nasal congestion and fever. His pediatrician
prescribed amoxicillin on the third day of illness. The fever persisted, however,
and by the fifth day, the patient was irritable and refused to flex his neck.
New symptoms reported in the emergency department included decreased
activity, poor intake of food and fluids, and decreased urine output. The
parents also reported that after 1 day of amoxicillin therapy, the patient
developed a faint red rash over his trunk, arms, and legs. The rash faded
after 2 days. Symptoms of confusion, headache, conjunctivitis, cough, vomiting,
or diarrhea were denied; however, the parents reported that his usual "noisy
breathing" seemed worse. Medical history included a diagnosis of laryngomalacia
diagnosed by bronchoscopy at age 5 months.
The infant was born at term after an uneventful pregnancy to a healthy
mother. He attended a day care center while his parents worked. There was
no exposure to cigarette smoke or pets. Immunizations were up-to-date, but
he had not yet received pneumococcal vaccine.
Dr Green: The patient's illness began as a
common upper respiratory tract infection associated with a low-grade fever
and congestion, and his condition has worsened after several days of illness.
He has developed irritability, neck stiffness, lethargy, and poor intake of
foods and fluid. Decreased urine output indicates dehydration. Meningitis
must first be excluded in anyone with a fever and stiff neck, but there are
other concerns. The change in the patient's breathing pattern from his usual
noisy breathing is worrisome. There are several bacterial and/or viral illnesses
that when combined with laryngomalacia might make the stridor worse. Fifteen
or more years ago, when invasive Haemophilus influenzae type b disease was a more imminent threat, I would have greater concern
about the possibility of epiglottitis in a febrile infant who does not want
to move his neck or swallow. Epiglottitis may in some ways mimic meningitis.
Supraglottitis is also a consideration. I am more concerned about bacterial
disease than a secondary viral process because this patient seems more ill
than one who simply has viral croup superimposed on laryngomalacia. Therefore,
if meningitis is ruled out, I would suspect a disease that involves the airway.
Extrinsic compression of the airway by a neck infection such as bacterial
adenitis is possible, although it would be uncommon for this to cause airway
compression. Bacterial adenitis may cause neck stiffness but would not cause
the patient to be more symptomatic from his previously diagnosed laryngomalacia.
However, a deep space infection could cause both neck stiffness and stridor.
An unrecognized congenital cyst with a secondary bacterial infection could
explain his predominant symptoms. The physical examination should reveal further
information.
Physical Examination: In the emergency department
the patient had a temperature of 39.3°C, heart rate, 165 beats/min; respiratory
rate, 35 breaths/min; and blood pressure, 100/65 mm Hg. His height was in
the 25th percentile for his age, and his weight was in the fifth percentile.
He was alert, acyanotic, ill-appearing, and mildly dehydrated. There was no
rash. Perfusion and pulses were normal. His neck was stiff, held in an extended
position, and turned slightly to the left. Examination of the nose, ears,
and throat revealed only moderate erythema of the tonsils and soft palate
and dryness of the oral mucosa. One left anterior cervical lymph node was
enlarged (3 cm), tender, and firm, but not red or fluctuant. Mild inspiratory
stridor was present and loudest with the patient in the supine position. His
chest was clear on auscultation. No cardiac abnormalities were present. The
remainder of the examination findings were normal. Room air oxyhemoglobin
saturation was 98%.
Dr Green: The patient has a high-grade fever
and an elevated heart rate. The tachycardia is most likely owing to the fever
and dehydration. It is reassuring that his blood pressure is normal although
this does not guarantee that he is not bacteremic or in the early stages of
sepsis. His respiratory rate is abnormal but he is not drooling or using accessory
breathing muscles. His height is in the 25th percentile and his weight is
in the fifth percentile. Poor growth is a common problem in infants and children
with inherited or acquired immunodeficiency syndromes; however, a single point
on a patient's growth curve is not enough to formulate a differential diagnosis,
so will not be considered at this time.
Although the patient has a stiff neck, his head is turned to the left.
He could have torticollis opposing nuchal rigidity from meningitis, except
that children with bacterial meningitis generally do not prefer one side to
the other, nor do children with epiglottitis. Perhaps the patient has a deep
space neck infection. I would now expand my differential diagnoses to include
peritonsillar and retropharyngeal abscesses. I can exclude a peritonsillar
abscess because the tonsils are red but not enlarged or asymmetric. A retropharyngeal
abscess may cause torticollis and stridor, and affected patients can have
symptoms similar to epiglottitis. A single suppurative lymph node can cause
torticollis but that seems unlikely to fully explain this patient's condition.
Before considering diagnostic testing, I want to be sure that there
is nothing I need to do emergently to protect him from further airway compromise.
If I unquestionably thought he had epiglottitis, I would call the epiglottitis
"team" even before obtaining a lateral neck radiograph. The team at our hospital
consists of otolaryngologists, anesthesiologists, and pediatric intensivists.
However, the fact that he is not in distress, not drooling, and is able to
be examined in the supine position is evidence that epiglottitis is highly
unlikely. Finally, an 11-month-old infant who is fully immunized is at very
low risk for invasive H influenzae type b infection,
which causes nearly all cases of bacterial epiglottitis.
At this point I want to examine the patient for a deep space neck infection
by obtaining a contrast-enhanced computed axial tomographic (CAT) scan of
the neck. A lateral neck x-ray film is quicker to obtain and might reveal
characteristics such as a widened retropharyngeal space, but I do not think
the findings will be conclusive. I would also obtain a complete blood cell
count with differential and platelet counts, electrolytes, serum urea nitrogen,
creatinine, and a blood culture. Even though the patient has been treated
with an oral antibiotic, it is still important to obtain culture for organisms.
Laboratory Data: A lumbar puncture revealed
clear colorless fluid containing 0.12 x 109 white blood cells
(0.05 neutrophils, 0.75 lymphocytes, 0.20 monocytes) and 0.43 x 109 red blood cells. A Gram stain of the cerebrospinal fluid (CSF) did
not show bacteria. The level of glucose in the CSF was 3.33 mmol/L (60 mg/dL)
and the CSF protein level was 17 mg/dL. Serum electrolyte, serum urea nitrogen,
creatinine, and glucose levels were normal. A complete blood cell count revealed
a white blood cell count of 10.6 x 109 with a predominance
of neutrophils. The platelet count was normal at 301.0 x 109.
A urinalysis revealed a specific gravity of 1.025, a pH of 6.0, and 15 white
blood cells per high-power field, but no red blood cells or bacteria. Blood
and a throat culture specimens were also obtained. The erythrocyte sedimentation
rate (ESR) was 40 mm/h. A plain radiograph of the neck showed a widened retropharyngeal
space. A contrast-enhanced CAT scan of the neck showed left anterior cervical
adenopathy, mild left parapharyngeal node enlargement without abscess formation,
and a round cystic lesion at the base of the tongue that was not enhanced
(Figure 1). The results of a chest
radiograph were normal.
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Contrast-enhanced computed axial tomographic scan showing cervical
adenopathy on the left and a cystic lesion at the tongue base.
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Dr Green: The CSF examination, which is always
indicated when there is concern for meningitis, revealed pleocytosis with
a predominance of lymphocytes, normal levels of glucose and protein, and no
organisms on Gram stain analysis. These findings are confounded by the fact
that the patient received amoxicillin for 2 days before the spinal tap was
obtained. Oral amoxicillin can partially treat bacterial meningitis, particularly
if Neisseria meningitidis is the culprit organism.
In contrast, Streptococcus pneumoniae usually will
not respond at all to oral amoxicillin. I am skeptical that the patient has
partially treated bacterial meningitis because his illness has progressed
despite amoxicillin therapy. Aseptic or viral meningitis is possible since
the CSF findings are consistent with those of aseptic meningitis. However,
aseptic meningitis would explain neither the progression of his illness nor
the worsening of his breathing.
The widened retropharyngeal space on the lateral neck radiograph suggests
a deep space neck infection, specifically retropharyngeal cellulitis or abscess.
Such infections are parameningeal and may be accompanied by a CSF pleocytosis
that mimics aseptic meningitis. However, in contrast with what was seen on
the lateral neck radiograph, the retropharyngeal space did not appear to be
widened on the CAT scan. Because the CAT scan is a more sensitive imaging
test, the results obtained here exclude a large retropharyngeal abscess. Often
in early deep space neck infections, the scan may not be sensitive enough
to detect a small abscess or cellulitis. The adenopathy is consistent with
a neck infection although it may not be the source of all symptoms. The nonenhancing
cystic lesion at the base of the tongue is an unexpected finding. It is not
clear whether this is an incidental finding of a congenital cyst or whether
this is an infected cyst. The cyst did not enhance with contrast, which contraindicates
infection.
The remainder of the findings from laboratory studies are consistent
with an infection. The ESR is elevated but is not specific enough to be helpful.
The urinalysis showing 15 white blood cells per high-power field is also not
helpful, although a group A streptococcus infection is sometimes associated
with sterile pyuria.
A bacterial infection seems to be the most likely diagnosis at this
time; therefore, I want to consider potential etiologic organisms and contemplate
therapy. Although I think the patient probably has a deep space neck infection,
I would still include coverage for meningitis. Because this is a current case
and the patient is immunized, I am not worried about H influenzae type b. The most likely pathogens in meningitis are S pneumoniae and N meningitidis. In an era
of bacterial resistance to penicillin, an infant or child who attends a day
care center is at greater risk for infection by penicillin-resistant streptococci.
I would thus treat this patient with both vancomycin and cefotaxime sodium,
using doses appropriate for a central nervous system infection. This regimen
also provides coverage for pathogens that cause deep space neck infections,
such as staphylococci and streptococci. Anaerobic organisms can also cause
deep space neck infections. A third-generation cephalosporin would probably
provide adequate coverage in anaerobic infections above the diaphragm. If
I were treating this patient for just a neck infection and not meningitis,
then single-drug therapy with either ampicillin sodium–sulbactam sodium
or ticarcillin disodium–clavulanate potassium would be adequate. Finally,
although I think it is unlikely that the cyst is secondarily infected with
bacteria, treatment with vancomycin and cefotaxime will be effective against
the pathogens involved in an infected congenital cyst. Infection of a thyroglossal
cyst, or even the thyroid itself, would likely be caused by S aureus, and vancomycin would be more than sufficient coverage. Superinfection
of a subglossal cyst, typically caused by mouth flora such as group A streptococcus
and staphylococci, would be treated the same way.
After starting antibiotic treatment, I will admit the patient to the
hospital and monitor his airway carefully. I will also consult my colleagues
in otolaryngology to interpret the cystic lesion at the base of the tongue
and review all of the radiographs with an attending radiologist to help define
the cyst and any other abnormalities.
Hospital Course: The patient was admitted to
the hospital and placed on intravenous (IV) vancomycin and cefotaxime. An
otolaryngologist was consulted and subsequently recommended observation and
IV antimicrobial therapy. A review of the existing hospital record revealed
that during the original evaluation for stridor, magnetic resonance imaging
of the chest was obtained and the findings were normal. However, when the
test was reviewed, a cyst at the tongue base was clearly present and was not
changed in size compared with what was currently found.
Dr Green: Any congenital cyst is at risk for
becoming secondarily infected, which would be a potential explanation for
all of the symptoms. However, 2 findings argue against secondary infection
of a congenital cyst. First, there has been no change in the size of the cyst.
In my experience, when cysts become secondarily infected, they invariably
enlarge. Second, unless it was very early in the course of the infection,
one would see enhancement by contrast on the CAT scan, which was not the case.
So I must conclude that this is a congenital cyst unrelated to the current
illness.
Hospital Course: Intravenous fluids corrected
the patient's dehydration but his oral intake remained poor. Fever occurred
(body temperature, 39°C-40°C) for the next 4 days while he was treated
with antibiotics. His stridor was unchanged. Blood, throat, urine, and CSF
cultures showed no growth by the third day of his hospital stay. On the fourth
day of hospital admission, the patient's lips became red and cracked, and
he developed swelling over the dorsum of both hands and swelling of the digits
of his hands and feet.
Dr Green: The patient has not responded to
our therapy, has prolonged fever, and now has new physical findings of red,
cracked lips and swollen hands and feet. These factors lead me to consider
2 possibilities. First, he did not respond to the antibiotics because the
neck infection has progressed to the formation of an abscess. The initial
CAT scan may have missed it because it was not fully developed. If that is
the case, his symptoms did not alleviate because successful treatment of an
abscess includes drainage in addition to antimicrobial therapy. While an abscess
could explain all of the persistent symptoms, it would not necessarily explain
the new findings involving the lips, hands, and feet.
The second possibility, and the more likely one, is that the patient
has Kawasaki syndrome (KS). His collective symptoms and signs meet the criteria
for this syndrome, which is diagnosed on clinical grounds.1
He has had 9 days of fever, a single swollen lymph node, pharyngeal erythema,
red and cracked lips, and swollen hands and feet. Although he does not have
a rash now, the parents reported one earlier in the illness, and the rash
of KS may be early and transient. The only criterion that he lacks is conjunctivitis.
Irritability, lethargy, and poor eating may be seen in patients with KS, but
these are very nonspecific features. Findings from the laboratory studies
are also consistent with KS. His ESR is elevated and his platelet count is
normal, which is expected early in the illness. Sterile pyuria is often present.
Finally, CSF pleocytosis is consistent with the aseptic meningitis observed
in patients with KS. What is atypical is the late onset of lip changes and
hand and foot swelling. Up to that point, although he had 8 days of persistent
fever, a single enlarged lymph node, and a history of a transient rash, he
did not meet criteria for KS. Incomplete or atypical KS has been described
in young infants, usually younger than 6 months.2
The other striking features that appeared early on were distractions from
the diagnosis of KS.
This patient meets criteria for KS on the ninth day of illness. Given
that I do not have much more time to treat him without losing the potential
benefit of IV immunoglobulin (IVIg) to prevent the development of coronary
artery disease, I will start treatment with standard therapy of aspirin and
IVIg. I will also obtain another complete blood cell count to see if his platelet
count has increased as I would expect in a patient with KS entering the second
week of the illness. He will, of course, need evaluation of his heart, including
an electrocardiograph and ultrasonic cardiography. However, because of his
neck stiffness, I will obtain another CAT scan of his neck to rule out a retropharyngeal
abscess.
Hospital Course: A repeated white blood cell
count revealed a platelet count of 750.0 x 109. Results of
a second ESR were 55 mm/h. Findings from a plain chest radiograph, a 12-lead
electrocardiograph, and ultrasonic cardiography were unremarkable. A second
CAT scan of the neck was obtained and showed no change from the first study.
Aspirin and IVIg were administered in doses standard for the treatment of
KS. All acute symptoms and signs resolved within 24 hours of treatment. Antibiotics
were discontinued prior to the initiation of IVIg therapy.
Dr Green: The rapid resolution of symptoms
following the administration of both aspirin and IVIg is consistent with the
diagnosis of KS, along with the elevated platelet count and ESR. In fact,
this impressive response to therapy may represent the only true test of diagnosis
for KS short of the development of coronary artery aneurysms. I am reassured
that the infant does not have abnormal coronary arteries at this time. If
this is a case of KS, prompt treatment is likely to prevent subsequent development
of a coronary artery aneurysm.
I will continue administering the high-dose aspirin until he has defervesced
for approximately 2 to 3 days and then lower the dose of aspirin, maintaining
this treatment until his ESR is less than 20 mm/h and his platelet count is
normal. Some experts recommend low-dose aspirin for a minimum of 6 to 8 weeks.
He will need another cardiac ultrasound in 4 to 6 weeks. If this patient were
receiving aspirin during influenza season, he would be at risk for Reye syndrome,
and appropriate precautions would have to be taken. I would want to know whether
he was susceptible to varicella because of the risk of Reye syndrome in patients
receiving aspirin who contract the varicella virus. At age 11 months, he is
too young to receive the varicella vaccine, but the IVIg has probably temporarily
protected him against chicken pox. Still, the parents should be advised to
call his pediatrician if their son is exposed to or develops varicella while
he is receiving aspirin.
Physical Examination: The patient was discharged
to his home with instructions for the parents to administer aspirin as prescribed.
At 6 weeks' follow-up, he was well although stridor persisted. The parents
reported peeling of his hands and feet. His ESR and platelet count were normal.
The results of a follow-up cardiac ultrasound were also normal. After evaluation
of his thyroid gland, the patient was found to have a large vallecular cyst,
which was resected. The stridor resolved after surgery.
COMMENT
Diagnostic accuracy in clinical care depends on a complete history and
physical examination. In the hospital setting, the initial assessment of a
patient is typically the most comprehensive. However, patients may come into
treatment at a point in the natural history of their particular illness before
all salient features are evident. This patient did not meet criteria for the
diagnosis of KS when he was first evaluated. Moreover, atypical or uncommon
manifestations of disorders can be traps for the inexperienced or unwary clinician.
This has been emphasized in the literature on KS.2, 3
For example, Stamos et al4 described 11 children
with KS whose initial symptoms suggested bacterial lymphadenitis and who developed
other features of KS later. In conditions that are diagnosed only on clinical
grounds, repeated interviews and physical examinations are essential.
The initial consideration of meningitis seemed wise because of the patient's
symptoms and the serious consequences of failure to diagnose and treat such
a life-threatening disorder. Although neck stiffness is a cardinal sign of
meningitis, the physician thought carefully about other serious conditions,
including epiglottitis and deep space neck infection. He wisely avoided the
trap of assuming that the patient had previously been correctly diagnosed
with laryngomalacia alone and thus was able to entertain the possibility that
an infected congenital cyst was a cause of the patient's illness. As it turned
out, the cyst was a classic "red herring," which the physician recognized
and rejected as the primary cause of the patient's illness.
The experienced clinician sifts through physical findings, extracts
pertinent information, and builds a factual case for the diagnosis or subsequent
investigation. In this case, as the course of the illness was revealed to
the physician, he used each new piece of information to expand the differential
diagnoses. In the beginning, he favored a diagnosis of a deep space neck infection;
as new physical evidence was disclosed, he concluded that an atypical presentation
of KS was the most likely diagnosis. The clinician with an open mind allows
emerging data to enhance the course of his or her thinking.
AUTHOR INFORMATION
Accepted for publication January 20, 2001.
| Editors' Note: With this article we inaugurate
a new feature of the ARCHIVES. The aim of "Clinical Problem Solving" is to
demonstrate how master clinicians approach complex problems. The thinking
process is more important than the final diagnosis. We thank the New England Journal of Medicine for the concept and for advice on implementation.
Contributions are welcome; however, informal inquiries via e-mail to the editorial
office are advised prior to preparing and submitting a manuscript.—Frederick P. Rivara, MD, MPH
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From the Diagnostic Referral Service (Dr McIntire) and the Division
of Allergy, Immunology, and Infectious Disease (Dr Green), University of Pittsburgh
School of Medicine, Pittsburgh, Pa.
Corresponding author and reprints: Sara McIntire, MD, University
of Pittsburgh School of Medicine, Division of Allergy, Immunology, and Infectious
Disease, 3705 Fifth Avenue at Desoto Street, Pittsburgh, PA 15213.
REFERENCES
1. Diagnostic guidelines for Kawasaki disease: American Heart Association
Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. AJDC. 1990;144:1220-1219.
2. Burns JC, Wiggins JW Jr, Toews WH, et al. Clinical spectrum of Kawasaki disease in infants younger than 6 months
of age. J Pediatr. 1986;109:759-763.
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3. Levy M, Koren G. Atypical Kawasaki disease: analysis of clinical presentation and diagnostic
clues. Pediatr Infect Dis. 1990;90:122-126.
4. Stamos JK, Corydon K, Donaldson J, Shulman ST. Lymphadenitis as the dominant manifestation of Kawasaki disease. Pediatrics. 1994;93:525-528.
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