About This Presentation
• This is the PowerPoint from the video,
it contains:
1) The definition and rules of the Clinical
Pathologic Case (CPC) competition
2) A sample CPC presentation
About This Presentation
• Please note:
1) The “notes” section contains written text of
the video dialogue for you to follow during
this presentation
The CPC Competition
Explained
John C. Southall, M.D.
Maine Medical Center, USA
What to Expect
• You will receive case 4 weeks in advance
– History and Physical
– Other pertinent facts
– Ancillary tests
• May include: Labs, ECGs, X-rays
What to Do
• Create a 20 minutes PowerPoint
presentation
• Presentations must be in English
• Non-native English speakers allowed 25
minutes
Judging
1) The quality of the differential diagnosis
•
Thought process of achieving final diagnosis
2) Presentation skills
•
•
Presentation impact
Adherence to time limits
Arriving at the correct diagnosis is a bonus
but not necessary to win
Questions?
Email me at:
southj@mmc.org
(John C. Southall, M.D.)
Title: A 34 Year-old Woman
with Visual Loss and Eye Pain
• Chief Complaint
– 34 year-old Caucasian woman with two days of
decreased vision in her right eye and throbbing
pain behind her eye
History of Present Illness
– For several weeks she states that she has “not
felt quite right.” 3 days ago she was seen in the
Emergency Department of a local community
hospital. The patient was told that her physical
exam including pelvic exam were “normal” and
some lab tests were sent. She was discharged
and told to follow up with her primary medical
doctor. She sees a doctor at a community
health center, and has an appointment for next
week.
Sample Case:
1st Presented 2004
San Francisco, California
Moderator:
Dr. Amanda Young, M.D.
Title: A 34 Year-old Woman
with Visual Loss and Eye Pain
• Chief Complaint
– 34 year-old Caucasian woman with two days of
decreased vision in her right eye and throbbing
pain behind her eye
History of Present Illness
– For several weeks she states that she has “not
felt quite right.” 3 days ago she was seen in the
Emergency Department of a local community
hospital. The patient was told that her physical
exam including pelvic exam were “normal” and
some lab tests were sent. She was discharged
and told to follow up with her primary medical
doctor. She sees a doctor at a community
health center, and has an appointment for next
week.
History of Present Illness
– 2 days ago she noticed that she couldn’t see the
“bottom half of the world” from her right eye.
This came on gradually, but has not resolved
and continues today. Yesterday she developed
pain behind her right eye. The pain is worse
with coughing or straining, but doesn’t change
with movement of her eyes. She denies any
symptoms in her left eye.
Past History
–
–
–
–
PMH: Depression, GERD, Bulimia
G0P0
No eyeglasses or contacts
PSH: Splenectomy 6 months prior for ruptured spleen
sustained in a car accident. She required blood
transfusions during her hospitalization.
– Meds: Citalopram, Pantoprazole, Birth Control Pills,
MVI, FeS04
– Allergies: None
– FH: Mother had breast cancer, Father died of a heart
attack. No blindness or glaucoma in her family.
Social History
– Rhode Island native, never lived out of state
– No recent travel out of Northeastern U.S.
– Unemployed, currently lives at residential drug
treatment center
– Uses crack cocaine and tobacco
– Has been in prison once – 3 years prior
– Claims one sexual partner currently
– Negative H.I.V. test 3 years prior
– Denies having had or been exposed to TB
Review of Systems
• Positive for:
– 10 lb weight loss x 2
weeks
– Fatigue, dizziness,
insomnia, headache x 1
month
– Hair loss x 2 weeks
– LMP 3 weeks ago
• Negative for:
–
–
–
–
Trauma
Fevers, night sweats
Back pain
Cough, chest pain,
shortness of breath
– Abdominal pain, dysuria,
discharge, dyspareunia
– Focal weakness or sensory
complaints
Physical Exam
• T 98.8, 110/66, P 76, RR 16, SpO2 97%
• Young woman, in no distress, holding R eye
Eye
• Visual Acuity: L 20/20, R
20/40
• Pupils: Equal, Round,
Reactive to light and
accommodation
• EOM: Full range of
motion, No INO
• Conjunctiva – clear
• Lid, Iris, Lens all appear
normal
• Normal fluoroscein exam
• Anterior Compartment: L
trace cells, R 1+ cells
• Intra-ocular Pressure: L
14, R 10
• Fundoscopy performed
with PanOpthalmic scope
• Visual deficit as follows
Fundoscopy
Right Eye
Left Eye
Eye
• Visual Acuity: L 20/20, R
20/40
• Pupils: Equal, Round,
Reactive to light and
accommodation
• EOM: Full range of
motion, No INO
• Conjunctiva – clear
• Lid, Iris, Lens all appear
normal
• Normal fluoroscein exam
• Anterior Compartment: L
trace cells, R 1+ cells
• Intra-ocular Pressure: L
14, R 10
• Fundoscopy performed
with PanOpthalmic scope
• Visual deficit as follows
Right Quadrantopsia
Right Eye
Left Eye
Physical Exam
• Neck: Anterior cervical lymphadenopathy, thyroid
normal size, non-tender
• Cardiac: Normal S1 and S2, no murmur, normal
distal pulses, no peripheral edema.
• Lungs: Clear bilaterally. Bilateral axillary
lymphadenopathy
• Abdomen: soft, non-tender, no hepatomegaly,
shotty inguinal lymphadenopathy bilaterally, no
CVA tenderness.
Physical Exam
• Pelvic:
– Normal external exam
– No vaginal bleeding
– No cervical motion tenderness, non-tender uterus and
adnexae
• Rectal Occult blood negative
• Skin
– Irregular hair loss on scalp, no scabbing
– Several irregular pigmented lesions on back
Labs
• CBC and Chemistries unremarkable
• ESR 48
• LFTs
– Alk phos: 64
– AST/ALT: 126/249
• PT normal at 12.2
Lumbar Puncture
• Tube 1
– 15 WBCs
– 9 RBCs
• Tube 4
– 20 WBCs
– 1 RBC
– Protein 32
– Glucose 57
– Colorless
EKG
Head CT – Non Contrast
Read as “normal non-contrast head”
Contestant/Discussant
Dr. John Southall, M.D.
Maine Medical Center, USA
“A 34 Year-old Woman with
Visual Loss and Eye Pain”
Generating a differential diagnosis
shouldn’t be a problem!
Problem List
•
•
•
•
•
•
•
•
•
•
•
•
Eye Pain
Visual loss
Malaised x 1 month
Depression
GERD
Bulemia
Splenectomy
Blood transfusions
Daily meds
Crack cocaine
Hx incarceration
10 lb weight loss over 2 weeks
• Hair loss over 2 weeks
• Cervical, axillary, inguinal
Lymphadenopathy
• Ant comp 1+ RBCs
• Abnormal fundoscopy
• Field cut
• Irregular hair loss
• Irregular pigmented skin lesions
• ESR 48
• AST/ALT elevated
• CSF WBCs
• EKG/CXR/CTH
“Real World”
• CC: Visual loss
– Painful vs. Painless?
Ptn: Painful visual loss
– Instantly generates a list of emergent conditions
“Real World”
DDx: Eye Pain
•
•
•
•
•
•
•
•
•
•
Foreign body
Herpes zoster
Trauma
Conjunctivitis
Iritis
Iridocyclitis
Uveitis
Blepharitis
Ingrown lashes
Orbital or periorbital
cellulitis/abscess
•
•
•
•
•
•
•
•
•
•
•
Sinusitis
Glaucoma
Inflammation of lacrimal gland
Tic douloureux
Cerebral aneurysm
Cerebral neoplasm
Entropion
Retrobulbar/optic neuritis
Ultraviolet keratitis
Dry eyes (ie lasik)
Irritation or inflammation from
eye drops, dust, cosmetics
“Real World”
• Differential
diagnosis narrowed
– By history
– By physical exam
– By diagnostics
“Real World”
• Differential
diagnosis narrowed
– By history
– By physical
exam
– By diagnostics
“Real World”
• Differential
diagnosis narrowed
– By history
– By physical exam
– By diagnostics
“Real World”
Easy Rule Outs
•
•
•
•
•
•
•
•
Foreign body
Herpes zoster
Trauma
Conjunctivitis
Iridocyclitis
Blepharitis
Ingrown lashes
Glaucoma
• Inflammation of
lacrimal gland
• Tic douloureux
• Entropion
• Ultraviolet keratitis
• Dry eyes (ie lasik)
• Irritation or
inflammation from eye
drops, dust, cosmetics
“Real World”
Rule Outs Requiring Basic Diagnostics
•
•
•
•
Orbital or retro-orbital cellulitis
Deep abscess
Sinusitis
Cerebral neoplasm
“Real World”
Can Not Rule Out
• Uveitis
• Cerebral aneurysm/dissection
• Optic neuritis or neuropathy
This Is Not The “Real World”
This is California!
Problem List
•
•
•
•
•
•
•
•
•
•
•
•
Eye Pain
Visual loss
Malaised x 1 month
Depression
GERD
Bulemia
Splenectomy
Blood transfusions
Daily meds
Crack cocaine
Hx incarceration
10 lb weight loss over 2 weeks
• Hair loss over 2 weeks
• Cervical, axillary, inguinal
Lymphadenopathy
• Ant comp 1+ RBCs
• Abnormal fundoscopy
• Field cut
• Irregular hair loss
• Irregular pigmented skin lesions
• ESR 48
• AST/ALT elevated
• CSF WBCs
• EKG/CXR/CTH
Specific vs. Nonspecific
Specific
• Visual loss
Nonspecific
• Supporting data or
– Specific field cut
• Retinal injury
– Abnormal fundoscopy
• Eye pain
Red Herring?
Visual Loss
Specific Field Cut (thanks!)
Right Quadrantopsia
Right Eye
Left Eye
This must narrow the differential
Monocular Quadrantopsia
• Medline through 1969 yields nothing
Monocular Quadrantopsia
• Medline through 1969 yields nothing
•
search yields nothing
– ever seen that?
Back to the basics!
Field Cut Basics
Monocular vs. Binocular?
Our patient
Right Eye
Left Eye
Monocular!
Place the Lesion!
Our patient
Right Eye
Left Eye
By definition…
Acquired Optic
Neuropathy
Acquired Optic Neuropathy
•
•
•
•
•
•
•
Compressive
Infiltrative
Inflammatory
Vascular
Toxic/Nutritional
Traumatic
Mechanical
Immediate Rule Out
•
•
•
•
Increased intracranial pressure
Glaucoma
Nutritional and toxic optic neuropathy
Traumatic
Acquired Optic Neuropathy
•
•
•
•
Optic neuritis
Ischemic optic neuropathy
Optic nerve compression
Inflammatory optic neuropathy
Acquired Optic Neuropathy
• Optic neuritis
• Ischemic optic neuropathy
• Optic nerve compression
• Inflammatory optic neuropathy
Optic Neuritis
Acute monocular loss of vision caused by
focal demyelination of the optic nerve
Pros
• Acute monocular
vision loss
Cons
Optic Neuritis
Acute monocular loss of vision caused by
focal demyelination of the optic nerve
Pros
• Acute monocular vision
loss
• Age: 15 to 45 years
Cons
Optic Neuritis
Acute monocular loss of vision caused by
focal demyelination of the optic nerve
Pros
• Acute monocular
vision loss
• Age: 15 to 45 years
• Painful
Cons
Optic Neuritis
Acute monocular loss of vision caused by
focal demyelination of the optic nerve
Pros
• Acute monocular
vision loss
• Age: 15 to 45 years
• Painful
Cons
• Afferent
pupillary defect
not present
Optic Neuritis
Acute monocular loss of vision caused by
focal demyelination of the optic nerve
Pros
• Acute monocular
vision loss
• Age: 15 to 45 years
• Painful
Cons
• Afferent pupillary defect
not present
• No pain with
EOEM
Acquired Optic Neuropathy
• Optic neuritis
• Ischemic optic neuropathy
• Optic nerve compression
• Inflammatory optic neuropathy
Ischemic Optic Neuropathy
• Not embolic
– Central retinal artery occlusion
– Branch retinal artery occlusion
CRAO
BRAO
Ischemic Optic Neuropathy
Generalized decrease of blood flow to
the optic nerve
–
Most common cause of optic neuropathy
worldwide
1) Non-arteritic
2) Artertic
Arteritic ION
(Temporal Arteritis)
Pros
– Secondary Sx:
•
•
•
•
Weight loss
Malaise
Headache
Scalp tenderness (hair loss)
Arteritic ION
(Temporal Arteritis)
Pros
Temporal Arteritis
– Weight loss, malaise, headache
– Optic disc has pallor
and swelling
Our Patient
Arteritic ION
(Temporal Arteritis)
Pros
– Weight loss, malaise,
headache
– Optic disc has pallor
and swelling
– Elevated ESR
ESR = 48
Arteritic ION
(Temporal Arteritis)
Pros
– Weight loss, malaise,
headache
– Optic disc has pallor
and swelling
– Elevated ESR
Cons
– Extremely rare
younger than 50
years of age
34 years old
Arteritic ION
(Temporal Arteritis)
Pros
– Weight loss, malaise,
headache
– Optic disc has pallor
and swelling
– Elevated ESR
Cons
– Patient too young
– Large afferent
pupillary defect
Arteritic ION
(Temporal Arteritis)
Pros
– Weight loss, malaise,
headache
– Optic disc has pallor
and swelling
– Elevated ESR
Cons
– Patient too young
– No afferent pupillary
defect
– Painless
visual loss
Acquired Optic Neuropathy
• Optic neuritis
• Ischemic optic neuropathy
• Optic nerve compression
• Inflammatory optic neuropathy
Optic Nerve Compression
Pros
• Occurs at any age
– Tumor, aneurysm,
sphenoid sinusitis,
blunt trauma
Cons
Optic Nerve Compression
Pros
• Occurs at any age
• Prechiasmal
disorder
Cons
Optic Nerve Compression
Pros
• Occurs at any age
• Prechiasmal
disorder
Cons
• Compressive
syndromes tend
to involve other
cranial nerves
Optic Nerve Compression
Pros
• Occurs at any age
• Prechiasmal
disorder
Cons
• No other cranial nerve
involvement
• No afferent
pupillary defect
Optic Nerve
Compression
The Anatomy:
Willis!
Ophthalmic Artery
Optic Nerve Compression:
Ophthalmic Artery Aneurysm
Pros
• Adequately &
eloquently explains
chief complaint
Cons
• Does not explain
ancillary data
• No other cranial nerve
involvement
• No afferent pupillary
defect
Acquired Optic Neuropathy
• Optic neuritis
• Ischemic optic neuropathy
• Optic nerve compression
• Inflammatory optic
neuropathy
Inflammatory Optic Neuropathy
Non-Infectious vs. Infectious
Inflammatory Optic Neuropathy
Non-Infectious vs. Infectious
Sarcoidosis
• Multi-system granulomatous disorder
– Up to 50% ocular involvement
• Ocular sarcoidosis
– Occurs early in the course of the disease
• fever, fatigue, weight loss, and malaise
Ocular Sarcoidosis
Pros
• Anterior uveitis common
– Our patient had 1+ cells
Ocular Sarcoidosis
Pros
• Posterior disease common
– Vitritis, uveitis, retinal vasculitis
Our Patient
Ocular Sarcoidosis
Sarcoid
Pros
• Anterior/posterior segment
manifestations
• Punched-out
choroidoretinal
lesions
Our Patient
Ocular Sarcoidosis
Pros
• Anterior/posterior segment manifestations
• Punched-out choroidoretinal lesions
• Skin lesions are found in 35% of
the patients
Ocular Sarcoidosis
Pros
• Anterior/posterior segment manifestations
• Punched-out choroidoretinal lesions
• Skin lesions
• Generalized lymphadenopathy
Ocular Sarcoidosis
Pros
•
•
•
•
Anterior/posterior segment manifestations
Punched-out choroidoretinal lesions
Skin lesions
Generalized lymphadenopathy
• Hepatic involvement
Ocular Sarcoidosis
Pros
•
•
•
•
•
Anterior/posterior segment manifestations
Punched-out choroidoretinal lesions
Skin lesions
Generalized lymphadenopathy
Hepatic involvement
• CSF pleocytosis
Ocular Sarcoidosis
Cons
• Conjunctiva usually involved (70%)
Ocular Sarcoidosis
Cons
• No conjunctival involvement
• Typical skin lesions
– Dissimilar to patient’s
Ocular Sarcoidosis
Cons
• No conjunctival involvement
• Typical skin lesions
– Dissimilar to patient’s
• Lung is the most commonly affected
organ
Ocular Sarcoidosis
Sarcoidosis “stage IV”
Our Patient
Ocular Sarcoidosis
Pros
•
•
•
•
•
•
Constitutional Sx
Anterior uveitis
Posterior uveitis
Fundoscopy
Skin lesions
Generalized
lymphadenopathy
• Hepatic involvement
• CSF pleocytosis
•
•
•
•
Cons
No conjunctival
involvement
Skin lesions atypical
No lung involvement
Does not explain
– Lymphadenopathy
without hilar involvement
– Hair loss
Inflammatory Optic Neuropathy
Non-Infectious vs. Infectious
From Breviary of Helth, 1547
…it maye come by syttenge on
a draught or sege where as a
pocky person did lately syt, it
may come by drynkynge oft
with a pocky person, but
specially it is taken when one
pocky person doth synne in
lechery the one with another…
Syphilis?
•
•
•
•
•
•
•
Henry VIII of England
Ivan the Terrible
Francis I of France
Napoleon Bonaparte
Ludwig von Beethoven
Lord Randolph Churchill
Franz Schubert
Syphilis
Treponema pallidum
Characterized by lipid outer surface with paucity of
antigenic proteins
Secondary Syphilis
Our Patient
• Irregular rash on back
Secondary Syphilis
• Irregular rash
– 90% of patients
•
•
•
•
Macular
Maculopapular
Papular
Pustular
Secondary Syphilis
Our Patient
• Generalized
lymphadenopathy
– Anterior cervical
– Bilateral axillary
– Bilateral shotty inguinal
Secondary Syphilis
• Generalized
lymphadenopathy
– 90% of patients
Secondary Syphilis
Our Patient
• Consitutional Sx
– Two weeks
– Malaise, headache,
insomnia, anorexia,
10 pound weight loss
Secondary Syphilis
• Consitutional Sx
– Malaise, headache,
anorexia, weight loss
– 70% of patients
Secondary Syphilis
Our Patient
Secondary Syphilis
• Hair loss
– Two weeks
– Irregular
• Irregular hair loss typical
Secondary Syphilis
Our Patient
• Elevated LFTs
Secondary Syphilis
• Hepatic, renal,
intestinal involvement
all described
Secondary Syphilis
Our Patient
• Two pelvic exams
– “Normal”
Secondary Syphilis
• Pelvic exam normal
– 80% of the time
What about the chief complaint?
“A 34 Year-old Woman with Visual
Loss and Eye Pain”
Ocular Syphilis
One to ten percent of syphilis cases…
An uncommon illness presenting commonly?
Ocular Syphilis
Ocular Syphilis
Anterior Uveitis
– Occurs during
secondary syphilis
– 56% unilateral
Our Patient
– Unilateral anterior
uveitis
• 1+ cells OD
• No cells OS
Posterior Uveitis
40% of ocular syphilis
Hallmark: painful visual loss
Syphilitic
Uveitis/Chorioretinitis
Our Patient
Ocular Syphilis:
Neurosyphilis by Definition
Ocular Syphilis
• CSF pleocytosis is
the classic finding
Our Patient
• CSF Tube #4
– 20 WBCs
– 1 RBC
Ocular Syphilis?
• Irregular rash on back
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
• 10 pound weight loss
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
• 10 pound weight loss
• Irregular hair loss
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
• 10 pound weight loss
• Irregular hair loss
• Elevated LFTs
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
• 10 pound weight loss
• Irregular hair loss
• Elevated LFTs
• CSF pleocytosis
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
• 10 pound weight loss
• Irregular hair loss
• Elevated LFTs
• CSF pleocytosis
• Anterior uveitis
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
• 10 pound weight loss
• Irregular hair loss
• Elevated LFTs
• CSF pleocytosis
• Anterior uveitis
• Posterior uveitis
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
• 10 pound weight loss
• Irregular hair loss
•
•
•
•
Elevated LFTs
CSF pleocytosis
Anterior uveitis
Posterior uveitis
• Painful visual loss
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
• 10 pound weight loss
• Irregular hair loss
•
•
•
•
•
Elevated LFTs
CSF pleocytosis
Anterior uveitis
Posterior uveitis
Painful visual loss
• Irregular monocular
field cut
Ocular Syphilis
• Irregular rash on back
• Generalized
lymphadenopathy
• Malaise, headache,
insomnia, anorexia
• 10 pound weight loss
• Irregular hair loss
•
•
•
•
•
•
Elevated LFTs
CSF pleocytosis
Anterior uveitis
Posterior uveitis
Painful visual loss
Irregular monocular
field cut
Browning DJ - Ophthalmology - 01-NOV-2000; 107(11): 2015-23
The Answer:
Ocular Syphilis
My Predictions…
• Diagnostic test?
– Blood/CSF
• RPR/VDRL/FTA-ABS/MHA-TP
• HIV testing
– 41% chance of being HIV(+)
• Treated per neurosyphilis protocols
– IV penicillin G
• 12 to 24 million units qd X 6-21 days
Our Patient’s Ocular Future?
85% chance of return to near normal vision
Our Patient
Normal
Thank You for This Opportunity
Moderator:
Dr. Amanda Young
The Final Diagnosis:
Ocular Syphilis
The Final Diagnosis:
• Diagnostic test: (+) VDRL
• Clinical course
– Two weeks of IV penicillin
• Outcome
– Almost complete recovery at 6
month follow up
CPC Mediterranean Emergency
Medicine Congress
• Space is very limited
• Please express interest early
Questions & Interest
southj@mmc.org
John C. Southall, M.D.
Maine Medical Center
Department of Emergency Medicine