Download A 15-Year-Old Boy with Congenital Heart Disease, Fevers, and

Case Challenge
A 15-Year-Old Boy with
Congenital Heart Disease, Fevers,
and Acute Onset of Dysarthria
Joshua T.B. Williams, MD; and Heather A. Fagan, MS, MD
questioning of the patient’s mother
revealed no additional pertinent medical or surgical history. The patient was
taking no medications, and he had no
known drug allergies.
On physical examination in the
emergency department, the patient
was moaning and ill-appearing. His
Images courtesy of Joshua T.B. Williams, MD.
A
previously healthy 15-yearold boy with a remote history
of repaired coarctation of the
aorta and ventricular septal defect developed 3 days of fatigue, diarrhea,
and intermittent fevers. He was seen
by his primary care physician and diagnosed with viral enteritis. Enteral
fluids were encouraged to maintain adequate hydration. However, his clinical course deteriorated over the next 4
days with worsening fatigue, intermittent chest palpitations, and shortness
of breath. On the morning of hospital
admission (approximately 10 days after the first fever), he developed pink
urine, difficulty walking, and slurred
speech. He was rushed to the emergency department for evaluation. Further
Joshua T.B. Williams, MD, is a Pediatric Intern,
The Children’s Hospital Colorado, University of
Colorado School of Medicine. Heather A. Fagan,
MS, MD, is Program Director, Pediatric Residency
Training Program; and an Associate Professor,
Pediatric Critical Care, Department of Pediatrics,
Comer Children’s Hospital, University of Chicago.
Address correspondence to: Joshua T.B. Williams, 1458 Dexter Street, Denver, CO 80220;
email: [email protected].
Disclosure: The authors have no relevant financial relationships to disclose.
doi: 10.3928/00904481-20140127-05
64 | Healio.com/Pediatrics
Figure 1. Computed tomography results in axial view (left) and coronal view (right) demonstrate a hypodense lesion in the distribution of the right middle cerebral artery, suggestive of an evolving infarct.
A = anterior; S = superior; R = right.
For diagnosis, see page 66
Editor’s note: Each month, this department features a discussion of an unusual diagnosis in genetics, radiology, or dermatology. A description and images are presented,
followed by the diagnosis and an explanation of how the diagnosis was determined. As
always, your comments are welcome via email at [email protected].
PEDIATRIC ANNALS 43:2 | FEBRUARY 2014
Case Challenge
vital signs were as follows: temperature 38.7° C, heart rate 111 beats per
minute, respiratory rate 28 breaths per
minute, blood pressure 92/64 mm Hg,
and oxygen saturation 98% on room
air. He had a 2/6 diastolic murmur at
the left sternal border, diffusely warm
extremities with bounding pulses, a
left-sided facial droop, 2/5 strength
in his left upper extremity, and 4/5
strength in his left lower extremity.
Initial laboratory study abnormalities included a white blood cell count
of 13.2 × 109 cells/L, a Na+ of 123
mEq/L, and a Cl– of 85 mEq/L. The
remainder of the values from the patient’s initial complete blood count,
basic metabolic panel, and coagulation
studies were within normal limits. An
electrocardiogram in the emergency
department revealed sinus tachycardia
without dysrhythmia.
The patient was admitted to the intensive care unit with presumed septic shock and concern for acute neurologic compromise. Aggressive fluid
resuscitation was initiated and blood
and urine cultures were obtained. The
patient received broad-spectrum antibiotic coverage with intravenous vancomycin, ceftriaxone, clindamycin,
and metronidazole. Soon after, ionotropic support was initiated secondary
to persistent hypotension.
Noncontrast computed tomography
(CT) of the head and a trans-esophageal echocardiogram (TEE) were then
obtained (Figures 1-2).
Given the large vegetations noted
on TEE, the hypodensity on head CT
scan (with the patient’s neurologic
signs), the patient’s prior cardiac history, and his fever, a definitive diagnosis of infective endocarditis (IE) was
made according to the Duke criteria
(Table 1).
PEDIATRIC ANNALS 43:2 | FEBRUARY 2014
TABLE 1.
Summary of Modified Duke Criteria for
Diagnosis of Infective Endocarditis*
Major Criteria
• Positive blood cultures for IE.
- Typical microorganisms for IE from two separate blood cultures.
- Persistently positive blood cultures with a microorganism consistent with IE.
- Single positive blood culture for Coxiella burnetii or IgG antibody titer > 1:800.
• Evidence of endocardial involvement.
• Positive echocardiogram for IE.
• New valvular regurgitation.
Minor Criteria
• Predisposing heart condition or history of intravenous drug use.
• Fever, defined as temperature > 38.0° C.
• Vascular phenomena (eg, major arterial emboli, mycotic aneurysm, Janeway lesions).
• Immunologic phenomena (eg, glomerulonephritis, Osler’s nodes, Roth spots) .
• Microbiologic evidence not meeting major criteria as noted above.
*A definitive diagnosis of infective endocarditis is established if two major criteria, one major and three minor criteria, or five
minor criteria are fulfilled. A diagnosis is “possible” if one major criterion and one minor criterion are fulfilled or if three minor
criteria are fulfilled.
IE = infective endocarditis; IgG = immunoglobulin G.
Data from Li et al9
Figure 2. Trans-esophageal echocardiography reveals two mobile vegetations, approximately 15 mm
× 20 mm, growing from the leaflets of the patient’s bicuspid aortic valve. AA = ascending aorta; LV =
left ventricle.
Healio.com/Pediatrics | 65
Case Challenge
Diagnosis:
Infective Endocarditis
HOSPITAL COURSE
After acute resuscitation efforts, the
patient underwent surgical replacement of the diseased aortic valve with
a homograft; intraoperatively, an aortic
valve annular abscess was discovered
and drained. Three days postoperatively,
two blood cultures grew out multiple
species of Staphylococcus epidermidis.
Pathologic analysis of the aortic valve
vegetations (Figure 3) with genotyping
confirmed the bacterial agent.
The patient was treated with 2 weeks
of parenteral gentamicin and 6 weeks
of parenteral vancomycin and ampicillin/sulbactam. He required several
months of physical and occupational
therapy. Six months postoperatively,
the patient was functioning at a nearnormal baseline with only mild left–
upper extremity residual weakness.
He was prescribed 5 mg of enalapril
by mouth twice daily for mechanical
valve–related aortic regurgitation.
Figure 3. Pathologic sections of the patient’s aortic valve vegetations. (A) The presence of granulation
tissue suggests the duration of inflammation exceeded 7 days. Significant necrosis of valve tissue was
also visible. (B) Gram stain of the vegetation revealed numerous gram-positive cocci in clusters.
DISCUSSION
In healthy children, IE is a rare diagnosis. A longitudinal study of children
in Arkansas between 1990 and 2002 reported only approximately 0.6 cases of
IE per 100,000 patients per year.1 Conversely, the incidence among children
with congenital heart disease (CHD)
is orders of magnitude greater, perhaps as high as 150 cases per 100,000
children.2-4 Abnormal cardiac anatomy
impairs cardiac function and disrupts
blood flow, which allows bacteria to
colonize damaged valves or infect sterile thrombotic clots. Echocardiography
revealed a bicuspid aortic valve. This
anatomic variant, found in 30% to 40%
of patients with coarctation of the aorta,
is a well-known risk factor for IE.5
Of all complications of IE, cardiac
complications are most common, but the
rate of neurologic sequelae is not negligible. A single-center, 17-year chart
review in the United States reported
that 6% (7 of 115) of cases of IE were
complicated by stroke,6 close to the 11%
reported among Japanese children with
IE at 66 institutions between 1997 and
2001.7 Patients who suffer neurologic
sequelae classically present with the
triad of neurologic signs (eg, weakness,
dysarthria), fever, and new-onset murmur. Morbidity and mortality are high.
Of those children who develop neurologic sequelae, up to 60% may suffer
66 | Healio.com/Pediatrics
A
B
permanent deficits.8 In-hospital mortality is approximately 5% for patients
without pre-existing heart disease, but it
approaches 50% for children with severe
cyanotic disease.8
The diagnosis of IE may be clinical
or pathologic, according to the Duke
criteria listed in Table 1.9 On the day
of presentation, this patient fulfilled one
major criterion (oscillating intracardiac
valvular mass on TEE) and three minor
criteria (predisposing cardiac lesion,
temperature > 38° C, and evidence of
arterial embolism). Of note, as stated
in the most recent revisions of the Duke
criteria, the role of radiologic imaging
in the early diagnosis of patients can be
PEDIATRIC ANNALS 43:2 | FEBRUARY 2014
Case Challenge
crucial. In this case, without the TEE results, a major criterion would not have
been fulfilled until several days after presentation, when multiple blood cultures
returned positive for S. epidermidis.
When an infectious organism is
identified, Staphylococci and Streptococci are the usual agents.6 However,
the mechanism of infection is rarely
known. Interestingly, in this case, an interval history from the patient’s mother
revealed that the patient’s ex-girlfriend
bit into his right arm 1 week prior to
the onset of his symptoms. A review of
the pertinent literature found that up to
40% of salivary and 60% of gingival
plaque samples may contain multiple
isolates of S. epidermidis.10 Thus, it
is presumed that the patient’s ex-girlfriend inoculated his bloodstream with
her oral flora, which attached to his
bicuspid aortic valve, developed into
IE, and precipitated an embolic stroke.
Yet, although bites — human or otherwise — comprise an estimated 1% of
all emergency department visits,11 IE
remains an extremely rare complication. IE resulting from bites has been
described mainly in case reports, and a
thorough literature review found only
one other documented case in which a
human bite was the source of IE further
complicated by neurologic sequelae.12
Due to the risk of IE in patients with
CHD, the American Heart Association
publishes guidelines for the prescription
of prophylactic antibiotics prior to dental and minor surgical procedures, which
are well-recognized mechanisms for
bacterial inoculation of the blood. The
most recent revisions were published
in 2007 and endorsed by the American
Academy of Pediatrics.13
PEDIATRIC ANNALS 43:2 | FEBRUARY 2014
CONCLUSIONS
Children with CHD are at increased
risk of IE. Therefore, extremely vigilant
follow-up is necessary to avoid significant morbidity and mortality from seemingly innocent clinical presentations. In
the evaluation of IE by the Duke criteria, radiologic findings — namely CT
and TEE — may be extremely useful
for a timely diagnosis. This case, to our
knowledge, is only the second documented report of IE complicated by neurologic sequelae that is postulated to be
due to a human bite.
ACKNOWLEDGMENTS
The authors would like to thank Dr.
Anthony Montag and Dr. Jennifer Pogoriller for assistance in locating and analyzing the patient’s tissue specimens.
The authors would also like to thank Dr.
Joseph Hageman for his assistance in reviewing this manuscript.
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