Transcript What`s your diagnosis?

Pediatric Photo Diagnostic
Challenges
Laura Drach DO, MSN, FAAP
All Children’s Hospital -Johns Hopkins Medicine
• I have no financial disclosures.
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• A 5 month male old presents with
macrocephaly, intermittent fever and
extreme irritability. Hits his head with
hand at times. Presents to ER after
having a 30 second seizure. You obtain
an MRI and see the following.
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What’s your diagnosis?
• Glioblastoma
• Subdural abscess
• Epidural hematoma
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• Glioblastoma
• Subdural abscess
• Epidural hematoma
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• Right frontal subdural abscess.
• Right parasagittal subdural abscess.
• Left temperoparietal subdural abscess.
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Subdural Abscess
• Occurs in 13 percent of patients with
neonatal meningitis.
• Occurs in 11 to 19 percent of patients with
gram-negative neonatal bacterial
meningitis.
• The risk of brain abscess is increased in
neonates with meningitis caused by
Citrobacter koseri , Serratia marcescens,
Proteus mirabilis, and Enterobacter
sakazakii.
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Subdural Abscess
• Usually needs a combination of
antibiotics and surgical intervention.
• Physical exam findings might include
vomiting, bulging fontanelle, increased
head circumference, separation of the
cranial sutures, hemiparesis, focal
seizures, and increased peripheral
white blood cell (WBC) count.
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• CSF will show pleocytosis with elevated
WBC, depressed glucose and elevated
protein.
• Initial treatment of brain abscess
includes Vancomycin, Ceftriaxone or
Cefotaxime and Flagyl.
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8 y/o female spent 10
days in Tennessee
mountains in June. She
presents with fever,
myalgia and
photophobia. Rash
started on the
wrist/hands and feet.
What’s your diagnosis?
• Rickettsia rickettsii
• Borrelia burgdorferi
• Parvovirus B19
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• Rickettsia rickettsii
• Borrelia burgdorferi
• Parvovirus B19
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2010 incidence of Rickettsia
Rickettsii by CDC
http://www.cdc.gov/rmsf/stats/index.html
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Rocky Mountain Spotted Fever
Rickettsia rickettsii
• 2-14 days after bite-fever, erythematous
macular rash, myalgia, nausea,
vomiting, photophobia, conjunctivitis,
headache
• May progress to severe disease with
multiorgan involvement
• Diagnosis with acute and convalescent
serology
• Treatment with doxycyline for seven
days
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Dog Tick
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One week of fever
• 18 month female is admitted with five
days of fever (Tm 104) and irritability.
She was treated as an outpatient with
Amoxil for neck lymphadenitis without
improvement. She is admitted to the
hospital and found to have other
findings on physical exam.
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Exam findings
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What’s your diagnosis?
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Staphylococcal lymphadenitis
Kawasaki Disease
Disseminated S. pyogenes infection
Hypersensitivity reaction to antibiotics
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Staphylococcal lymphadenitis
Kawasaki Disease
Disseminated S. pyogenes infection
Hypersensitivity reaction to antibiotics
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Kawasaki Disease
• Systemic vasculitis of small arteries
• Fever for at least 5 days with at least
four other physical exam findings;
-conjunctivitis
-rash
-oral mucosal changes, strawberry tongue
-edema of the hands and feet
-lymphadenopathy
Kawasaki Disease
• Atypical (incomplete) diagnosis made if
not all 4 criteria met, with (+) ancillary
data
- CRP > 3.0, ESR >40
- Hypoalbuminemia
- Sterile pyuria
- Elevated ALT, AST
- Anemia, hyponatremia
Pediatrics, 2004:114:1708
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Kawasaki Disease Pearls
• Positive throat culture or adenovirus does not rule out
KD.
• Treat with 2 gm/kg IVIG no more than 2 times.
• High dose steroids for IVIG failures
• Normal echocardiogram at 6 weeks predicts normal
echocardiogram at 6 months
• Bilateral conjunctival injection occurs simultaneously
• Infants are more likely to develop aneurysms
• Defer MMR for 11 months after IVIG.
• High dose to low dose aspirin when symptoms resolve
• Influenza immunization during the “flu” season since
the patient will be on aspirin
A 8 year old has relapsed ALL.
She is s/p stem cell transplant
& chemotherapy. Oncologist
states outcome is grave but
she is eligible for a phase 1
study. Family asks you what
you would do. They want to
keep their child comfortable
but also provide every chance
at cure possible.
What should you advise?
Go home with
hospice
-Seek treatment
with phase 1 trial
-Advise the family
to seek
concurrent care.
-
What should
you advise?
-Go home with
hospice
-Seek treatment
with phase 1 trial
-Advise the family
to seek
concurrent care.
Concurrent Care Act
-Allows qualified hospice providers to provide
interdisciplinary palliative care to the entire family unit,
including parents and siblings, and be reimbursed for this
care.
-PIC: individual, family, and group counseling, nursing and
personal attendant care, physician pain and symptom
management consultation, volunteer support, and respite.
-Medicaid enrollment
-Physician attestation that the child is not expected to live
to age 21
8 year old patient with
cerebral palsy, global
developmental delay and gtube dependence. Mom states
he cries at times during the
day, possibly related to his
feeds he receives through the
g-tube. She thinks he is in
pain but all current testing has
been negative. The GI doctor
has done a full work up, and
you have found no etiology to
this point. What is your next
step?
What should you advise?
- Trial of lortab
- Trial of gabapentin
- Follow up with neurologist
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- Trial of lortab
- Trial of gabapentin
- Follow up with neurologist
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Visceral Hyperalgesia
-Altered response to visceral
stimulation, causing decreased
activation threshold for pain in
response to a stimulus.
-Retching, pain related to feeding
intolerance, flatus and bowel
movements, & prolonged crying.
Medications that maybe used;
-gabapentin
-cyprohepatadine
-tricyclic antidepressants
Gabapentin
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Used for neuropathic pain and seizures
Exact mechanism is unknown
Few drug interactions
Side effects include sedation, confusion,
ataxia and dizziness.
• Titrate slowly until desired effect
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• A 6 year old male presents to your
office with history of fever and difficulty
walking. He has no pets at home, but
there are some neighborhood cats and
dogs.
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What’s your diagnosis?
Staphylococcus aureus
Bartonella quintana
Bartonella henselae
Bartonella bacilliformis
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Staphylococcus aureus
Bartonella quintana
Bartonella henselae
Bartonella bacilliformis
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Bartonella henselae
Cat-Scratch Disease
• Begins as papule at site of tick, scratch,
or bite
• 85% of patients solitary lymph node
• Dissemination may occur and present as
FUO in children
• Other organ systems involved: liver,
spleen, CNS, ocular, and bone
• In-vitro susceptibility to multiple classes
of antibiotics
Bartonella henselae;
Cat-Scratch Disease
<
<
Bartonella henselae
Cat-Scratch Disease
8-month old male with a
history of severe atopic
dermatitis presents with a
pruritic and painful rash
involving the face.
dermatlas.med.jhmi.edu
What’s your diagnosis?
-Papular Acrodermatitis of Childhood
-Eczema herpeticum
-Impetigo
-Scabies
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Papular Acrodermatitis of Childhood
Eczema herpeticum
Impetigo
Scabies
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Eczema Herpeticum
• Complication of eczema with
herpes simplex virus infection
– HSV-1 or HSV-2
• Vesicles begin to cluster and
eventually become
hemorrhagic
• Surface viral culture and HSV
DNA PCR
• Treatment with acyclovir
• Monitor for bacterial
superinfection
pediatricsconsultant360.com
7 y/o girl with cellulitis is
admitted to the hospital after
she fails to improve on seven
days of PO TMPsulfamethoxazole. Her 10 y/o
sister was recently diagnosed
with Streptococcal
pharyngitis. What would be
your empiric antibiotic
treatment?
Which is the correct drug?
Penicillin IV
TMP-sulfamethoxazole IV
Ciprofloxacin IV
Metronidazole IV
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Penicillin IV
TMP-sulfamethoxazole IV
Ciprofloxacin IV
Metronidazole IV
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Streptococcus pyogenes
• 4-5 million infections/year
• > Winter and spring
months
• Streptococcal infection
common in ages 5-12
years
• Children 6-15 y/o have
highest streptococcal
titers
• Manifestations of disease:
toxin, suppurative and
immune mediated
Streptococcus pyogenes
• Suppurative sequelae
(invasive disease)
– Bacteremia, toxic
shock, and deep
tissue/bone/joint
• Non- suppurative
sequelae
– glomerulonephritis
– acute rheumatic fever
– arthritis
Streptococcus pyogenes
• If concerned about non-Suppurative sequale,
obtain ASO and Anti- DNAse B together.
• Glomerulonephritis occurs more often after
impetigo than pharyngitis,
• Pharyngitis, both ASO & Anti DNAse B are
increased but with impetigo, ASO does not
rise as high as anti DNAse B.
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Streptococcus pyogenes
Invasive Disease Treatment
• Trimethoprim-sulfamethoxazole has poor
activity against S. pyogenes
• Treatment of choice penicillin,cephalosporins
and clindamycin
• Invasive infections consider combination
therapy with penicillin and clindamycin
Scarlet fever
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Cellulitis/ Necrotizing fasciitis
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• 14 y/o male presents with headache
and fever. A few days into his illness he
develops a petechial rash on his
extremities. Immunizations are UTD and
there is no recent travel history or tick
bite. He looks well on exam.
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WBC 2500 (ANC 700)
. 12
Hgb
PLT 93,000
Albumin 3.4 ALT/AST
43/38
LDH 838 Uric Acid 4.4
PT 14.8
What’s your diagnosis?
Meningococcemia
Rickettsial disease
Norovirus infection
Parvovirus B19 infection
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Meningococcemia
Rickettsial disease
Norovirus infection
Parvovirus B19 infection
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Parvovirus B19
• DNA viruses
• Replicate in human
erythrocyte precursors
• Droplet transmission
• Most infections in
temperate climates occur
in the spring months
Parvovirus particles
(Courtesy of Linda M. Stannard
University of Cape Town)
Parvovirus
• Viremia resolves with the development of
IgG and IgM response in
immunocompetent patients
– Viremia may be detected by PCR for
months after acute infection
• Immunocompromised patients may
develop a chronic viremia
Parvovirus B19
• Clinical manifestations
described
– Fifth disease (erythema
infectiosum)
– Transient aplastic crisis
– Anemia
– Hydrops fetalis
– Polyarthropathy
Parvovirus B19 infection (erythema
infectiosum, fifth disease) with typical facial
erythema, "slapped cheek sign.“
Red Book Online Visual Library, 2009
Slapped cheek and lacy rash
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Parvovirus B19
• Petechial rash described in 13/17 children with
confirmed parvovirus B 19 infection
• Children (12/13) presented with a mild febrile
illness with petechiae involving the trunk and
extremities. Petechiae usually absent from
head and neck.
• Leukopenia (10/13) and thrombocytopenia
(4/13)
• All patients had uncomplicated illness
– 6 had short hospitalizations
– 1 patient underwent bone marrow
examination
Pediatrics 2010;125:e787
Stocking Glove
distribution
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10-month old male presents with
failure to thrive and recurrent
infections. He is coughing and
hypoxic.
He is diagnosed with
Pneumocystis jiroveci pneumonia
(PCP) and Severe Combined
Immunodeficiency (SCID).
Two weeks into treatment with
TMP-SMX he develops hepatitis.
The liver biopsy shows the
following.
Cryptosporidium
Disseminated PCP
Mycobacteria avium-intracellulare
Hepatotoxicity secondary to antibiotics
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Cryptosporidium
Disseminated PCP
Mycobacteria avium- intracellulare
Hepatotoxicity secondary to antibiotics
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SCID Patient with Disseminated
Pneumocystis jiroveci
• PCP first recognized to cause pneumonia in
malnourished and premature infants after WWII
• 1980s associated with the AIDS epidemic
– MMWR publication June 1981
• Five men (29-33 years old) treated for biopsy
confirmed Pneumocystis carinii pneumonia at
3 different Los Angeles hospitals.
• Extrapulmonary disease has been described in
AIDS patients
– Eye, skin, lymph nodes, GI tract, liver, kidney
and spleen
MMWR 1981;30(21);1-3
SCID Patient with Disseminated
Pneumocystis jiroveci
• Children with Severe Combined
Immunodeficiency have defects in T and B
cell function
• Usually present in the first few months of
life with failure to thrive, chronic diarrhea,
frequent fungal and viral infections
• Mortality approaches 100% by age 2
13 y/o male sustains a
cat bite. He does not
seek medical attention
and within 3 days
develops significant
swelling of the hand.
He has an allergy to
penicillin. What would
be the best antibiotic
choice?
What’s the correct drug?
Cefdinir
Levofloxacin
Clarithromycin
Combination of TMP- sulfamethoxazole
and clindamycin
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Cefdinir
Levofloxacin
Clarithromycin
Combination of TMP- sulfamethoxazole
and clindamycin
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Cat Bites
• Rate of infection is high with cat bites
• Organisms involved include
– Pasteurella sp., Capnocytophaga sp.,
Staphylococcus aureus, Moraxella sp. and
various anaerobes
• Oral amoxicillin-clavulanate is the drug of
choice
• Patients with PCN allergy
– Alternatives include TMP-sulfamethoxazole
with clindamycin or extended-spectrum
cephalosporin with clindamycin
12 y/o male develops
acute onset fever,
headache, and vomiting.
He develops a rash on
extremities and trunk. He
is thrombocytopenic and
neutropenic. No ill
contacts. No travel history.
Adolescent immunizations
are delayed.
What’s your diagnosis?
S. pneumoniae sepsis
Neisseria meningitidis infection
Henoch-Schonlein Purpura
Norovirus infection
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S. pneumoniae sepsis
Neisseria meningitidis infection
Henoch-Schonlein Purpura
Norovirus infection
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Meningococcemia
• Disease is most common in
children < 5 year of age with
highest case-fatality rate in
teenage population
• Serogroups causing disease A, B,
C, Y, W-135
• Onset of disease may be abrupt
with progression to fulminant
disease
• Treatment; cefotaxime,
ceftriaxone, ampicillin or penicillin
• Prevention of infection using the
conjugated meningococcal
vaccine (A, C, Y, W-135)
Meningococcal Group B
• Type B meningitis breakout at Princeton
University and UC Santa Barbara
(2013-2014)
• Drexel University student dies
• To control the outbreak, FDA grants
Breakthrough Therapy designation for
meningococcal serogroup B vaccine
which had been approved in Europe,
Canada and Australia.
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2-week old male admitted
with vomiting and fever. The
CSF has 60 WBC and O
RBC with normal glucose
and protein. Despite
antibiotics, the infant
remains febrile. Cultures
from the urine, blood and
CSF have no growth at 48
hours. On hospital day #4
the patient has a
generalized seizure. An MRI
is obtained.
What’s your diagnosis?
Herpes Simplex
Enterovirus
Congenital Cytomegalovirus
Parvovirus B19
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Herpes Simplex
Enterovirus
Congenital Cytomegalovirus
Parvovirus B19
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Neonatal Herpes Simplex
Infection
• 1 in 2000-5000 neonates yearly
• Only 40% of the mothers knew they had
genital herpes
– Mothers can lack a history of genital
herpes
• Most infants infected during birth
.
Neonatal Herpes Simplex
Infection
• Early symptoms may be vague
• Types of infection
– SEM: skin, eye, mucosa
– CNS: encephalitis
• HSV encephalitis is not always “severe”
– Disseminated: pneumonia, hepatitis, DIC
• 72.5% of untreated babies that present with skin
vesicles alone progress to CNS or disseminated
disease
Whitley RJ, et al. Pediatrics 1980;66:495-501.
Neonatal Herpes Simplex
Infection
• Culture
– Ideal for testing of skin vesicles and surveillance
cultures of mucosal surfaces
– Poor sensitivity in blood or CSF
• Polymerase Chain Reaction (PCR)
– Test of choice in CSF for diagnosing CNS disease
– Less sensitive in blood but should also be
checked
• Diagnostic testing
– Serology (blood)
• Not all patients will seroconvert (some false
negatives)
• Maternal IgG may be present
Neonatal Herpes Simplex
Infection
• Neuroimaging
– Diffuse, nonfocal involvement
• MRI with contrast is more sensitive than
CT.
– CT has poor sensitivity of ~40-50%.
• Abnormal CT correlates with poor
outcome
– Sensitivity of MRI and CT improve ~1
week after onset of symptoms.
Tien RD, et al. AJR 1993;161:167-76.
Neonatal Herpes Simplex
Infection
• Treatment of neonatal HSV
– Acyclovir 60 mg/kg/day  every 8 hours
– Duration
• 14 days for SEM disease
• 21 days for CNS or disseminated disease
• Check CSF PCR near end of therapy
– Positive result requires longer treatment and
correlates with worse prognosis
• Suppressive therapy with oral acyclovir for 6
months after IV treatment
– Infants with CNS disease had improved
neurodevelopmental outcomes when they
received 6 months of oral acyclovir N Engl J Med 2011; 365:1284-1292
6 y/o female developed
fever, headache and
nasal congestion 1 week
ago. She gets better, but
than develops severe
abdominal pain. A CT
scan shows pancreatitis.
3 days later a rash
develops.
Immunizations are up to
date.
Urine is suspicious for
nephritis.
What’s your diagnosis?
Meningococcemia
Kawasaki disease
Measles
Henoch-Schonlein Purpura (HSP)
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Meningococcemia
Kawasaki disease
Measles
Henoch-Schonlein Purpura (HSP)
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Henoch-Schonlein Purpura
• Incidence: 10/100,000 children per
year
– Most common vasculitis of
childhood
– Peak incidence at 5 years
– Preceding URI in at least 36%
– Palpable purpura, abdominal pain,
arthralgia/arthritis, and/or nephritis
• Platelet count normal
• Management with NSAIDs
– Steroids for severe abdominal
pain, nephritis and arthritis
– Dapsone for chronically recurring
disease
3-month old female
develops a rash with
crusting greater in the
skin folds of the axilla.
The rash relatively spares
the face and extremities.
The father recently had a
rash on his hands.
What’s your diagnosis?
Cutaneous Larva Migrans
Herpes Simplex Virus
Infantile scabies
Mosquito bites
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Cutaneous Larva Migrans
Herpes Simplex Virus
Infantile scabies
Mosquito bites
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Infantile Scabies
• Sarcoptes scabiei (mite)
• Transmission by close personal
contact
– Incubation 4 to 6 weeks if
not previously exposed
• Mites concentrate on hands
and fingers
• Rash can be widespread due to
sensitization to the mite
– Norwegian scabies
• Immunocompromised
patients and children on
long term steroids
• Widespread crusting and
hyperkeratotic lesions
Infantile Scabies
• Permethrin cream 5% (>=2
months age)
– 1% lindane cream or lotion
(reserve for patients that
fail to respond to
permethrin)
– Ivermectin as single dose
for severe or Norwegian
scabies if refractory to
topical treatment
Scabies
• Antihistamines, antibiotics
and topical steroids as
indicated
• Treat all household contacts
topically to prevent
reinfestation
• Hot water wash and dry all
linens
– If linens or clothing
cannot be washed, store
in a bag for one week
Red Book
Online Visual
Library, 2009
Blistering Rash
5 yo male of Indian descent with rash,
(resembling sunburn) and fever. Family
called PCP on Friday afternoon about the
fever and were advised to give Motrin.
Later that night, dad noticed rash that
resembled a sunburn. The rash started on
cheeks and chest and progressed to back
and legs over the weekend.
What’s your diagnosis?
• Staph Scalded Skin
• Stevens Johnson Syndrome
• Toxic Epidermolysis Necrosis
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• Staph Scalded Skin
• Stevens Johnson Syndrome
• Toxic Epidermolysis Necrosis
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Stevens- Johnson syndrome: involvement of skin
and 2 mucus membranes. Usually involves less
than 30% of the body. Usually a prodrome of fever,
headache, malaise and sore throat. Drugs are most
common trigger, followed by infection and
autoimmune diseases.
Toxic Epidermal Necrolysis (TEN): Involves more
than 30% of the skin surface.
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Common medications
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SJS/TEN
• Patients should be treated as burn patients
• Ophthalmology should be involved, especially
for TEN Patients.
• Treatment for Stevens-Johnson is usually
supportive. IVIG, steroids, plasmapheresis,
cyclosporine, and immunomodulators have
been used with no proven effectiveness.
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Thank You
• Special thanks to Dr. David Berman and
Dr. Alexis Major for sharing their photos.
References
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Degregory (2013, May). To his mother, Trisomy 18 child in St.
Petersburg is forever her perfect boy. The Saint Petersburg Times.
Newburger JW, Takahashi, M, Gerber, MA et al. Diagnosis, Treatment
and Long Term Management of Kawasaki Disease: A Statement of
Health Professionals form the committee on Rheumatic Fever,
Endocarditis and Kawasaki Disease, Council on Cardiovascular
Disease in the Young, American Heart Association. Pediatrics,
2004;114;1708.
Orloff, Stacy. ChiPPS Pediatric Palliative Care Newsletter Issue #33;
November, 2013.
Rauch DA, Gershel, JC. Caring for the Hospitalized Child. American
Academy of Pediatrics. 2012.
Wolfe, Hinds, Sourkes. Textbook of Interdisciplinary Pediatric Palliative
Care. Philadelphia, 2011.
Zorc JJ and Hall, CB. Bronchiolitis: recent evidence on Diagnosis and
Management. Pediatrics 2010:125:342-349.