Download CE horses or zebras Monterey handout

9/9/13 The challenges of diagnosis When you hear hoo;eats, think horses…don’t think zebras versus Horses or Zebras?-­‐ You make the call in diagnosing pediatric cases It's not rare… If it's siCng in your chair Rachel A. “Stacey” Coulter, OD, MS Associate Professor Dipl, Binocular Vision, PercepCon, and Pediatric Optometry Horses or Zebras? SomeCmes it is easy to make the call…. Horses or Zebras? SomeCmes it’s not… Horses or Zebras? Context maGers It is harder when your target is moving! 1 9/9/13 Diagnosing disease in the pediatric populaCon •  Children are usually healthy •  BUT vision-­‐threatening and life-­‐threatening condiCons do present •  How do we differenCate the common and/or relaCvely common condiCon from the more serious condiCon in the pediatric populaCon? •  Recognize differences in epidemiology and presentaCon of a condiCon •  Differences in diagnosCc tesCng and management Choosing a management opCon-­‐ the art of paCent care •  Are treatment opCons the same for a child as for an adult? Does the intervenCon work the same way? •  Will pediatric adherence equal adult adherence? •  When should you order addiConal tesCng or make a referral? •  If treatment includes pharmaceuCcal use, is agent approved for pediatric paCents? Case 1
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CC 11 yo girl and parents note HA
Onset - began 6 mos before
Location – temples and frontal region
Quality – throbbing
Associated Symptoms - appeared pale, complains of noise and light intolerance, nausea Frequency- 2-3 x month
Context – unknown
Severity – moderate to severe
Modifying Factors –some relief with Advil or Tylenol
Duration – less than 1 hour
Previous interventions - none
Differences between pediatric and adult paKents •  Children aren’t miniature adults! •  Epidemiological differences •  Differences in HOW condiCons present and children’s ability to describe symptoms •  DiagnosCc tesCng different for pediatric populaCon •  Pediatric paCents require addiConal informaCon for prescribing drugs •  The art of paCent care -­‐ special consideraCons in managing kids •  Case presentaCon •  Horse vs. Zebra ? •  DifferenCal diagnosis, differences in epidemiology and tesCng in children •  Case comparison •  Discuss management for pediatric paCents Case 1
•  Ocular Hx first eye exam
•  Sys Hx –ROS unremarkable; taking no medications; no
allergies
•  Fam Hx – unremarkable; no HA in family
•  Acad Hx good student
2 9/9/13 Case 1 Examination Findings
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Refraction
VA
OD -1.50 sph
20/20-2
OS -2.50 sph
20/25+2
Cover Test c Rx
Orthophoria at distance
6 exophoria at near
Visual Field grossly normal OD, OS
Case 1
•  Pupils 6 mm OU +direct consensual response; no APD
detected
•  SLX unremarkable
•  IOP
•  OD 14 mm Hg
•  OS 15 mm Hg
•  DFE optic nerve C/D .2 OD, OS; + distinct disc margins;
pink and healthy OD, OS; no overt pathology
Pediatric headache
What is your management of a child with headaches? When do you refer? Whom do you refer to? When is a scan indicated? Tension headache
•  Characteristics
–  Band-like sensation around head
with neck or shoulder pain
–  May last for days
–  Continuous pain; occurs on palpation
posterior neck muscles
–  #1 cause of tension headache- stress
–  Associated with school -straight A students
at-risk
–  Contributing factors- poor eating habits,
lack of sleep, depression, bullying
classmates, family problems
•  Primary- not the result of another condition
–  Migraine
–  Tension-type headaches
•  Secondary to another condition
–  Acute – seek Dr/ER;
include sinus,
otitis, pharyngitis
–  Chronic – second to pathology
Migraine
•  Characteristics
–  Pulsating
–  Bilateral or unilateral
–  Moderate to severe in intensity
–  With aura 14-30% of children
•  reversible aura develops
•  lasts up to one hr
•  pain occurs 1 hr after aura
•  May be accompanied by vomiting or
photophobia
–  Lasts 1-48 hrs
–  Aggravated by physical activity
3 9/9/13 Headaches secondary to
CNS pathology
Sinus headache
•  Characteristics
•  Intracranial mass
–  Severe occipital HA
exacerbated by
sneezing, coughing
–  Pain worse in
morning
•  Meningitis
•  Hydrocephalus
–  Throbbing HA worse in morning
–  Pain varies with head position
–  May have referred pain
–  Hx nasal discharge, congestion
–  Fever may be present
Pediatric headache Discussion • 
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Encephalitis
Hemorrhage
Tumor
Blood clots
Head trauma
Pediatric headache Discussion •  ObservaConal study 815 paCents seen at headache clinic 1 -­‐ 18 ys old over seven years •  Data collected child’s age, sex, duraCon of illness, frequency and duraCon of headache a_acks, diagnosis, invesCgaCon, treatment, and follow up arrangement. •  Headache diagnosed using InternaConal Headache Society’s diagnosCc criteria Diagnosis
No.
Migraine without aura
227
Migraine with aura
39
Hemiplegic migraine
15
Episodic tension headache
128
Chronic tension headache
178
Mixed migraine and tension headache 87
Cluster headache
1
Unclassified headaches
97
Others
43
Abu-­‐Arafeh K, Macleod S. Serious neurological disorders in children with chronic headache. Arch Dis Child 2005; 90:937-­‐940. Abu-­‐Arafeh K, Macleod S. Serious neurological disorders in children with chronic headache. Arch Dis Child 2005; 90:937-­‐940. Eye problems that may contribute to pediatric headaches •  Uncorrected refractive error
–  Astigmatism
–  High hyperopia
•  Binocular vision problems
–  Convergence Insufficiency, convergence excess,
vertical deviations, fusional vergence dysfunction,
basic esophoria, basic exophoria
•  Accommodative problems - spasm
•  Computer Vision Problem
•  Ocular Disease
–  Uveitis
–  Optic neuritis
%
27.9
4.8
1.8
21.8
21.8
10.6
0.1
11.9
5.3
Recap Case 1
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CC 7 yo girl parents note HA
Onset - began 6 mos before
Location –frontal, temples
Quality – throbbing
Associated Symptoms - appeared pale, complained of noise and light intolerance, nausea Frequency- 2-3 x month
Context – unknown
Severity – moderate to severe
Modifying Factors –some relief with Advil or Tylenol
Duration – less than 1 hour
Previous interventions – none
Sys Hx, Acad Hx, Oc Health-unremarkable
4 9/9/13 Recap Case 1
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Refraction
VA
OD -1.50 sph
20/20-2
OS -2.50 sph
20/25+2
Cover Test c Rx
Orthophoria at distance
6 exophoria at near
Visual Field grossly normal OD, OS
Recap Case 1
•  Pupils 6 mm OU +direct consensual response; no APD
detected
•  SLX unremarkable
•  IOP
•  OD 14 mm Hg
•  OS 15 mm Hg
•  DFE optic nerve C/D .20 OD, OS; distinct disc margin;
pink and healthy; no overt pathology
•  Macular clear OD, OS, + foveal reflex OD, OS
Recap Case 1 •  Dx- Headache not linked to vision or
ocular health causes
•  Mgmt- Initiate headache log; Suspect
migraine; Referral for evaluation by
neurologist
•  Neurologist Diagnosed – Pediatric
Migraine; managed with NSAIDs;
Sumatriptan Nasal Spray-> decrease in
HA frequency and severity
Another headache case
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Refraction
VA
OD -2.50 -0.25 x 178
20/20-2
OS -2.50 sph
20/20
Cover Test c Rx
Orthophoria at distance
4 exophoria at near
Visual Field normal finger count OD, OS
Another headache case
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CC 10 yo girl complains of HA, intermittent blurred vision
Onset - began 6 mos before
Location –all over
Quality – throbbing
Associated Symptoms – nausea and vomiCng Other Symptoms -­‐numbness lower back, decreased appeCte Frequency- increased; now daily
Context – early morning
Severity – moderate to severe
Duration – less than 1 hour
Another headache case
•  Pupils 6 mm OU +direct consensual response; no APD
detected
•  SLX unremarkable
•  IOP
•  OD 16 mm Hg
•  OS 15 mm Hg
•  DFE optic nerve C/D .15 OD, OS; distinct disc margin;
pink and healthy; no overt pathology
•  Referred to neurologist who diagnosed migraine without aura •  Recommended headache log be completed •  No show to two follow-­‐up appointments 5 9/9/13 Another headache case 2 years later… Presents to Emergency Room CC-­‐ HA increasing frequency x 4 mos Neurological exam – normal Completes HA log Dx-­‐ migraine without aura and tension type HA Management-­‐ Ibuprofen for tension HAs RTC 6 weeks… Decrease in tension HA; conCnued migraine HA Recommend prophylaxis of sumatriptan nasal spray -­‐excellent response Pediatric headache summary
•  Yes, HA are common in kids
•  Small % are related to vision and treated by refractive
correction, management of accom/binocular prob and
ocular conditions
•  Rarely (<1 %) due to intracranial pathology
•  Most HAs require detective work to determine pattern
i.e. migraine vs tension
•  A headache log is very useful
•  Management may result in referral to pediatrician,
neurologist, neuropsychologist
Pediatric headache
•  Signs indicating need for neuroimaging
•  Features of cerebellar dysfunction
–Ataxia
–Nystagmus
–Intention tremor
•  Features of increased intracranial pressure
–Papilledma
–Night or early morning vomiting
–Large head
•  Focal new neurological deficits
–Recent development of strabismus
–Focal seizures
Another headache case •  1 yr later … •  paCent returns to doctor •  CC-­‐ daily brief headaches, usually in early morning; someCmes associated with vomiCng •  Other symptoms-­‐ intermi_ent blurred vision, numbness lower back, decrease in appeCte •  Increased frequency and changing associated
symptomatology
•  MRI scan ordered
•  Shows left cerebellar hemisphere tumor with obstructive
hydrocephalus; Tumor removed and biopsied
•  Histopathology revealed a pilocytic astrocytoma
Pediatric headache
•  When to image? Usually NO
•  Pattern in the patient’s history of:
–  HA < 6 mos unresponsive med tx
–  Persistent HA; no fam hx migraine
–  Persistent HA w/episodes of confusion,
disorientation
–  Personality change
–  Deterioration of school work
–  HA waking a child from sleep
–  + Fam Hx condition w/ CNS lesions
–  Change in headache symptomatology or
personality – lowers the threshold for
scanning
Case 2 •  8 yo black female •  CC-­‐ pain in OD and cheek area; difficulty opening eye to clean it; also notes tearing, blurred vision •  Systemic Hx-­‐ ROS +fever and upper resp inecCon •  Past Ocular Hx, Med Hx-­‐ unremarkable •  Meds-­‐ none •  Allergies-­‐ none 6 9/9/13 Case 2 •  BVA: OD 20/25 OS 20/20 •  Pupils: ERRL, (-­‐)APD OU •  ConfrontaConal visual fields: Full OD/OS SLX •  Lids/Lashes: Case 2 •  MoClity – full; no restricCons OD, OS •  Cover Test aligned •  DFE limited views –  vitreous: No cell OD/OS –  opCc nerve C/D .40 OD, OS; disCnct disc margin; pink and healthy; –  macula clear OD, OS –  reCna limited view periphery – clear as viewed OD, OS –  OD +redness, edema (lower lid > upper lid), edema –  OS normal • 
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ConjuncCva/Sclera: normal OD/OS Cornea: normal OD/OS AC: formed, no cell/flare OD/OS Lens: normal OD/OS IOP: OD 14 mm Hg, OS 13 mm Hg Causes of Pediatric Red Eye When is a red eye more than a red eye? What informaCon do you want? What are the management consideraCons? • 
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KeraCCs Anterior UveiCs ScleriCs/episcleriCs EndophthalmiCs Bacterial ConjuncCviCs Orbital CelluliCs Orbital Tumor-­‐
rhabdomyosarcoma, reCnoblastoma • 
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Viral conjuncCviCs Allergic conjuncCviCs Chlamydial conjuncCviCs Contact dermatoconjuncCviCs BlephariCs Stye DacryocysCCCs Orbital pseudotumor PotenCally vision or life threatening Bacterial conjuncCviCs vs preseptal celluliCs Pediatric Red Eye -­‐> Increase severity •  Bacterial conjuncCviCs –  Usually bilateral –  common in pediatric populaCon –  most common organisms-­‐Streptococcus pneumoniae and Haemophilis influenzae –  highly contagious •  Preseptal celluliCs –  Unilateral –  eyelid Cssue distension –  causes SinusiCs, spread of infecCon from other nearby sites or blood, insect bite, allergic reacCon Bacterial conjuncCviCs Preseptal celluliCs Orbital celluliCs 7 9/9/13 Orbital CelluliCs Orbital septum -­‐ fibrous membrane separates eyelid skin from deeper structures of orbit Preseptal vs Orbital CelluliCs •  For BOTH •  Fever •  Eyelid edema •  Pain •  Red eye •  Child is ill-­‐appearing •  Increase in winter months •  BUT orbital celluliCs osen has: •  RestricCon in eye movements •  Decreased vision •  Papilledema •  Proptosis Preseptal vs Orbital CelluliCs Preseptal Orbital Age Usually < 5 years Tend to be older; mean age 7-­‐12 yrs old Risk factors ConCguous spread infecCon; SinusiCs, orbital trauma osen break in skin Pathogenesis Staph aureus, strep, methocillin-­‐resistant staph aureus Diagnosis Normal vision, no proptosis, Proptosis, blurred vision, EOMs full range of moCon ophthalmoplegia Management Clindamycin, Bactrim; oral, iv for severe cases Cephalosporin, Unasyn + Bactrim; Vancomycin (severe cases) DuraCon tx 7-­‐10 days 2-­‐3 weeks ComplicaCons Can spread into orbit Vision loss (blindness 10 % cases), orbital or brain abcess, cavernus sinus thrombosis Staph aureus , strep, anaerobes, H. flu, pseudomonas, methocillin-­‐
resistant staph aureus Another case •  9 yr old female presents with swollen OS •  HPI: started 4 days ago “gevng worse”; more difficult to open eye; + discharge; +diplopia in all gazes •  Saw a pediatrician 2 days ago; dx preseptal celluliCs iniCated amoxicillin, tylenol •  Past Ocular Hx, Med Hx: unremarkable •  Allergies: none •  ROS: + fever; (-­‐) neck sCffness Another case •  Returned to pediatrician yesterday •  Changed meds to AugmenCn •  Ordered a maxillofacial CT Another case • 
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VA: OD 20/20 OS 20/20 Pupils: 4mm → 2mm, brisk, equal, (-­‐)APD OU ConfrontaConal visual fields: Full OD/OS Lids/Lashes: –  OD normal –  OS +redness, edema-­‐ upper and lower lids; proptosis •  ConjuncCva/Sclera: normal OD/OS •  Cornea: normal OD/OS •  AC: formed, no cell/flare OD/OS 8 9/9/13 Another case Ocular moClity TesCng Lens: normal OD/OS •  IOP: OD 14 mm Hg, OS 13 mm Hg •  Post segment evaluaCon –  OpCc Nerve 0.2 –  Vitreous: No cell OD/OS –  ReCna periphery as viewed normal MoClity: OD — normal OS — -­‐0.5 adducCon and superior gaze, -­‐1.5 abducCon (Notes diplopia in all field of gaze) EvaluaCon of proptosis Hertels: OD 13mm, OS15mm Another case •  Laboratory tests: •  CBC : –  WBC 11.6 K/mm3 •  DifferenCal: – 
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Neutrophils 8050/mm3 Lymphocytes 2010/mm3 Monocytes 880/mm3 Eosinophils 60/mm3 Basophils 40/mm3 Hgb: 12.5 G/DL Platelets: 294 K/mm3 ESR: 65 C-­‐ReacCve Protein: 4.6 •  Vital signs: BP 115/68, Pulse 76, T 36.9 CT Scan Max/Face Abscess formaCon adjacent to les orbit and sinusiCs les ehthmoid sinus Another case •  Dx: Orbital celluliCs •  Tx: Admi_ed to hospital; IV celsriazone and clindamycin; Afrin spray •  1 day later-­‐moClity was almost restored •  3 days later-­‐ full moClity restored; decreased redness, edema; diplopia eliminated 9 9/9/13 Summary •  Can be difficult to disCnguish preseptal from orbital celluliCs; Any doubt? Tx for orbital celluliCs •  Preseptal –  usually starts with well-­‐demarcated edema, erythema of periorbital skin, sos Cssues –  able to idenCfy an event (bug bite, injury, stye) •  Orbital –  erythema, edema of eyelids and periorbital Cssues, painful EOMs, decreased vision, pupillary deficits, proptosis –  usually starts as bacterial sinusiCs –  tx iv anCbioCcs targeCng most likely organism; switch to oral 2-­‐3 weeks aser tx starts Case 3 •  10 yo black female
presents for routine
eye exam
•  Systemic Hx -­‐ROS unremarkable •  Past Ocular Hx, Med Hx, Fam Hx: unremarkable •  Meds: none •  Allergies: none Case 3 •  BVA –  OD -­‐2.75 sph 20/20 –  OS -­‐2.25 sph 20/20 •  EOM –  full and unrestricted OD, OS •  Pupils ERRL -­‐APD •  SLX –  Lids, lashes, conj, cornea clear –  A/C deep and quiet –  neg for cells, flare •  IOP Goldmann –  OD 31 mm Hg –  OS 31 mm Hg Case 3 •  DFE C/D raKo OD>OS •  Gonioscopy •  angles open 360 degrees OD, OS ciliary body visible •  (-­‐) cells, pigment DiagnosCc TesCng in Pediatric PaCents •  Elevated IOP, opCc disc damage, myopia, SLX, gonioscopy show open angle-­‐ bilateral or unilateral? What are your concerns when suspecKng pediatric glaucoma? What informaCon do you want? What tests/findings are likely to work on a paCent of this age? •  Visual fields offer marginal help –  PaCent osen must be 8-­‐10 years old •  IOPs are elusive –  Icare may be promising •  Pachymetry – cauCon interpreCng central corneal thickness and measured pressure; thinner cornea may underesCmate pressure •  OpCcal Coherence Tomography (OCT) promising –  Study compared •  ped gla eyes 125 -­‐ 148 •  ped normal eyes 82 -­‐ 102 Stuart A. The challenge of diagnosing pediatric glaucoma. Eyenet 2009; 25-­‐26. 10 9/9/13 Red flags for pediatric glaucoma •  Fam Hx-­‐ sibling, parent with gla in infancy or childhood •  Oc Hx –  Hx of cataract –  Tx with high dose steroids on face •  Ocular findings –  Large myopic shiss in infancy –  Port wine stain on eyelid –  Aniridia or other ocular abnormality –  UveiCs, trauma, neoplasm Stuart A. The challenge of diagnosing pediatric glaucoma. Eyenet 2009; 25-­‐26. Childhood Glaucomas Congenital 38%
Aphakia 20%
Sturge-­‐Weber 10%
Ant Segment Dysgenesis 6%
Trauma 6%
Aniridia 5%
Steroid induced 3% Juvenile 2% ROP 2% UveiCs 2% Other condiCons 5% • 
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–  1 in 50,000 –  Autosomal dominant -­‐ chromosome 1; MutaConal analysis of the myocilin gene at chromosomal region 1q21-­‐q31 can be performed –  Impaired ou{low aqueous in trabecular meshwork –  Osen associated with myopia –  PaCents osen have extremely high IOPs Case 4
•  8 yo black male in
•  Med Hx seasonal
third grade
allergies
•  CC of blur at distance •  Fam Hx uncle with
and near x few
asthma
months with glasses
•  Meds none
•  Oc Hx last eye exam
•  Allergies none
and current Rx from 7
mos ago; negative for •  ROS unremarkable
eye infection, injury,
trauma
.5 yrs 4.5 yrs 1.4 yrs 1.1 yrs 6.8 yrs .5 yrs 8.6 yrs 7.6 yrs 6.0 yrs –  Ocular abnormaliCes, systemic syndromes, neoplasms, rubeosis Taylor RH, Ainsworth JR, Evans AR, Levin AV. The epidemiology of pediatric glaucoma: The Toronto Experience Journal of AAPOS 1999. 3(5): 308-­‐315. Juvenile open-­‐angle glaucoma •  Juvenile open-­‐angle glaucoma Management •  MedicaCons to temporarily control IOP •  Glaucoma surgery –  goniotomy –  trabeculectomy –  cyclocryotherapy, external laser procedures, endoscopic cyclophotocoagulaCon (ECP) – reduce fluid producCon from ciliary body Examination Findings
•  Habitual Rx
OD -1.00-0.25 x 020
OS -1.25-0.50 x 158
•  Refraction and VAs
OD -1.50-1.25 x 063
OS -1.50-1.75 x 122
•  Pupils
–  direct, consensual; no
APD
20/3020/25-
•  Confrontation Visual
Field full OU
•  Amsler grid normal OU
•  NPC TTN
•  MEM +1.00 OD, OS
11 9/9/13 Slit Lamp Exam
Corneal Findings
•  Vogt’s Striae
•  Munson’s Sign
•  Fleischer’s Ring
Corneal Topography
Simulated K readings:
45.40/44.30 @ 060 OD 46.30/44.40 @ 122 OS Ocular Health •  IOP
OD 15 mm HG
OS 16 mm HG
•  DFE
–  optic nerve C/D .35 OD, OS; distinct disc margins;
pink and healthy; no overt pathology
–  Macular clear OD, OS, + foveal reflex OD, OS
Keratoconus Diagnosis • 
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Keratometry SLX Computerized corneal mapping DiagnosCc rigid CL Corneal thinning Regarding these findings.. What is the differenCal diagnosis? What management is appropriate? What is the prognosis for this paCent? Keratoconus Characteristics
•  Most common corneal dystrophy in U.S
•  Progressive corneal thinning creating mid-cornea
bulges; affects both eyes, may be asymmetrical
•  Refractive error changes- increasing astigmatism and
myopia
•  Later - corneal swelling and scarring
•  Onset – usually late teens, early 20s
•  Sxs – blur, glare sensitivity
Flat fivng contact lens 12 9/9/13 Signs of Keratoconus
•  Early
Keratoconus in Young Patients
•  Moderate
–  Minimal corneal signs
–  Frequent spec Rx
change
–  Oblique astigmatism
–  Dx
•  Computerized corneal
mapping
•  diagnostic rigid CL
(base curve = flat K)
observe nipple
–  K readings 45-52 D
–  Distortion retinoscopy
–  Munson sign
–  Fleischer ring
–  Paraxial stromal
thinning
–  Scarring at
Descemet’s
membrane
Keratoconus Causes
•  Possible Causes
–  Inherited corneal abnormality
–  Eye injury
•  Typically presents puberty progressive until 3rd or 4th
decade
•  Can occur from very young children to middle-age
•  Most patients progress slowly, but may progress more
rapidly in early stages
DifferenCal Diagnosis •  Pellucid marginal degeneraCon •  Dz associations
–  Retinitis Pigmentosa
–  Retinopathy of Prematurity
–  Vernal Keratoconjunctivitis
–  Leber’s congenital amaurosis
–  Ehlers-Danlos Syndrome
–  Down syndrome
–  Osteogenesis imperfecta
Keratoconus
•  Management
–  Change Rx
–  Specialty Contact Lenses
•  Phakic intraocular lenses
–  Collagen crosslinking (CXL) –  Deep Lamellar Anterior Keratoplasty (DALK)
–  INTACs, intrastromal corneal ring segment
–  (Worse Cases) Corneal Transplant
•  Keratoglobus •  Terrien's marginal degeneraCon Case 5
•  8 yo black male 2nd
grade student
•  CC + itching x 5 days
OU; matter noted on
lashes in the AM
•  Oc Hx first eye
exam; negative for
eye infections,
injuries, surgeries
•  Med Hx ROSunremarkable
•  Fam Hx blind
grandfather
•  Meds none
•  Allergies none
13 9/9/13 Case 5 •  RefracKon and VAs OD +1.50-­‐0.75 x 180 20/20-­‐ OS +1.50-­‐0.75 x 180 20/30-­‐ •  ConfrontaKon Visual Field full OU •  Amsler grid normal OU •  NPC TTN •  MEM +1.00 OD, OS •  Pupils direct, consensual; no APD •  SLX capped meibomian glands, 1+ papillae •  IOP OD 15 mm Hg OS 16 mm Hg Dilated Fundus Examination
•  OD, OS optic nerve
disc C/D 0.2; pink and
healthy disc
•  OS- posterior pole
–  1 disc diameter
melanotic gray lesion
in inferior macula
•  Periphery
–  at 6:30 white calcified
lesion without fluid
DifferenCal diagnosis for reCnal lesion What is your diagnosis? What informaCon do you want? Is this a typical presentaCon? What is the prognosis for the paCent? Diagnosis
•  Coat’s disease •  Toxocara EndophthalmiCs •  OpCc nerve glioma •  Other tumors… Evaluation Bascom Palmer
•  Examination under anesthesia two weeks later by
retinal oncologist
–  Found interval change; txed by laser ablation
–  Dx Reactivation of previous retinocytoma (RB1 mutation)
•  Evaluation three weeks later
•  Referred to retinal specialist
•  Impression Late presentation of a multifocal
unilateral retinoblastoma without familial hx
•  Lesions Primary Retinocytoma
–  Laser ablative management to two previously noted foci
and to new tumor
•  Follow in 3 weeks
–  new activity noted in periphery treated with ablation
•  Followed 6 mos later no new tumors noted
14 9/9/13 ReCnoblastoma presentaCon Case Management
•  Additional Recommendations
–  Baseline Neuro-imaging Studies with CT
imaging of head and orbit:
–  Trilateral retinoblastoma –syndrome that
consists of unilat or bilat retinoblastoma with
intracranial neuroblastic tumor
–  Recommend that child with germline
retinoblastoma be screened by MRI every 6
mos after dx for 4 yrs
Leukocoria (52 %) Orbital celluliCs (<1%) Strabismus (24 %) Iris changing color Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ,Boyd NW. PresenCng signs of reCnoblastoma. J Pediatr 1998; 132(3):505-­‐508. Another Case
Leukocoria Differential Diagnosis
•  Congenital cataract
•  Toxocaris canis
•  Coat’s Disease
•  Beware the white
reflex!
•  The story of Joey
•  Persistent Hypertrophic Primary Vitreous
Case 6 •  CC 10 yo black male; no complaints
•  Ocular Hx, Med Hx unremarkable
•  Meds none
•  Allergies none
Case 6 • 
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Refraction
OD -3.00-0.50 x 89
OS -2.50 sph
Cover Test c Rx
VA
20/25
20/25
–  Orthophoria at distance
–  4 exophoria at near
•  Visual Field by confrontation normal OD, OS
15 9/9/13 Case 6 Case 6 •  Pupils 6 mm OU +direct consensual
response; no APD detected
•  SLX unremarkable
•  IOP
•  OD 14 mm Hg
•  OS 15 mm Hg
DifferenCal Diagnosis Papilledema What quesCons/informaCon do you want? What is the differenCal diagnosis? What tesCng is indicated? Pseudo vs True Papilledema Pseudo-papilledema Margins lumpy Pink/yellow Elevation peripapillary NFL True Papilledema Margins indistinct Hyperemic Elevation confined to disc Anomalous vessels Normal vessel appearance No hemes or exudates +hemes, exudates, cotton
wool spots •  Papilledema -­‐Increased intracranial pressure due to tumor or pseudotumor •  OpCc glioma •  OpCc neuriCs •  OpCc drusen DifferenCal Diagnoses OpCc neuriCs •  VA markedly decreased •  autoimmune disorder •  history of recent illness or immunizaCon OpCc glioma •  Bow-­‐Ce atrophy •  low-­‐grade tumors •  Monitored eye exams and CT scans 16 9/9/13 Pseudopapilledema OpCc Nerve Drusen • 
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Papilledema
Composed of mucopolysaccharides and mucoproteins In children, may be buried; appear with advancing age bilateral 70% autosomal trait-­‐irregular penetrance Pediatric Pseudotumor Cerebri in Papilledema Managing a suspicious opCc nerve •  OpKons: –  A good fundus photograph –  Repeat exam to reassess the finding: follow-­‐up photographs in a week and a month. –  An exam under anesthesia –  An exam with angiography –  A spiral CT –  A sedated MRI •  Increased intracranial pressure, normal ventricles, and normal cerebrospinal fluid •  Epidemiology-­‐ rare -­‐ 1/100,000; no gender difference; associaCon with growth hormone •  Symptoms-­‐ Worse in morning –  headache (62-­‐91%) –  nausea (30%) –  Vision-­‐-­‐ diplopia (~30%), blur (~30-­‐60%), –  Other symptoms: neck pain, nystagmus, Cnnitus, verCgo –  no symptoms (33 %) Pseudotumor cerebri in pediatric populaCon •  Ocular findings –  10% VA < 20/40 –  25% visual field deficits •  Dx –  QuanCtaCve perimetry and opCc nerve examinaCon > more sensiCve than VA Distelmaier F, Sengler U, Messing-­‐Juenger M, Assmann B, Mayatepek E, Rosenbaum T. Pseudotumor cerebri as an important differenCal diagnosis of papilledema in children. Brain Dev 2006: 28(3):190-­‐195. Phillips PH, Repka MX, Lambert SR. Pseudotumor cerebri in children. JAAPOS 1998; 2(1): 33-­‐38 Pseudotumor cerebri in pedriatric populaCon • 
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Image to eliminate tumor; lumbar puncture Tx-­‐Acetazolamide; Furosemide, Prednisolone Most cases resolve in 4 months Residual effects –  33% opCc nerve atrophy –  15% visual field defects –  10% decreased VA •  Risk of recurrence –  6-­‐22% -­‐ conCnue to monitor 17 9/9/13 TesCng Recap Case 6 •  Neurologic imaging before lumbar puncture •  Lumbar puncture •  CSF values for children-­‐ lateral decubitus posiCon •  CC 10 yo black male; no complaints
•  Ocular Hx, Med Hx unremarkable
•  Meds none
•  Allergies none
–  <8 years: 10 to 100 mm H2O –  >8 years •  60 to 200 mm H2O •  200-­‐280 borderline ( consider clinical evidence) •  280 mm abnormal Recap Case 6 • 
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Refraction
OD -3.00-0.50 x 89
OS -2.50 sph
Cover Test c Rx
VA
20/25
20/25
–  Orthophoria at distance
–  4 exophoria at near
•  Visual Field by confrontation normal OD, OS
Recap Case 6 Recap Case 6 •  Pupils 6 mm OU +direct consensual
response; no APD detected
•  SLX unremarkable
•  IOP
•  OD 14 mm Hg
•  OS 15 mm Hg
Another case •  17 yo white male •  CC: verCcal diplopia x 6 weeks; blurred vision OS > OD; HA intermi_ent for 2 years; increased frequency and severity x 6 mos •  Med Hx: ADHD x 3 yrs aser experiencing restlessness, poor concentraCon, difficulty sleeping •  Meds: dextroamphetamine 18 9/9/13 Another case • 
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Refraction
VA
OD pl
20/30
OS pl
20/80
Pupils - APD
Oc Motility
Noncomitant-4th nerve palsy
Visual Field normal finger count OD, OS
Another case •  SLX unremarkable •  IOP OD 15 mm Hg OS 16 mm Hg •  DFE Another case Neuroimaging •  CT scan of head •  Pineal tumor Case 7 •  CC 6 yo black female presents for
evaluation of irritated eye x 3 days;
+ pain, redness OD
•  Ocular Hx saw a pediatrician 1 mos ago; dx was
allergic conjunctivitis; txed with OTC allergy
drops
•  Med Hx – GI- today vomited, not eating;
Constitutional-lethargy
•  Meds none
•  Allergies none
Case 7 Case 7 •  VA –  unaided OD 20/50 OS 20/40 –  pinhole OD 20/40 OS 20/30 •  Pupils 4/4 ERR-­‐ APD •  EOM full OD, OS •  Very photophobic in OD •  SLX Adnexa OD mild edema; OS normal Eyelids OD mild swollen; OS normal ConjuncCva OD 2+ injecCon OS white and quiet Cornea OD normal (-­‐) staining; OS (-­‐) staining Anterior Chamber OD 3+-­‐4 cells and flare; OS deep & quiet Iris OD, OS normal Lens OD+ fibrin 19 9/9/13 UveiCs Review What is the differenKal diagnosis for pediatric uveiKs? What quesCons/informaCon do you want? When do you work up pediatric uveiCs? Whom do you refer to? What tests may be ordered?? •  UveiCs-­‐inflammaCon of any structures of the uvea (iris, ciliary body, choroic) •  Types –  Anterior uveiCs and iridocycliCs –
inflammaCon of the iris and ciliary body –  Posterior uveiCs –
inflammaCon of choroid Pediatric UveiCs Discussion •  Causes –  Autoimmune disorders (most common) •  Juvenile Idiopathic ArthriCs •  Others-­‐ Kawasaki syndrome; chronic infanCle neurological cutaneous and arCcular/neonatal-­‐onset mulCsystem inflammatory disease syndrome; sarcoidosis; HLA-­‐B27-­‐
associated uveiCs; tubulonephriCs and uveiCs syndrome; psoriasis; and familial juvenile systemic granulomatosis. Pediatric UveiCs Discussion •  More reported cases since the 1960s •  PanuveiCs secondary to toxoplasmosis and toxocariasis decreased •  Idiopathic uveiCs most common cause •  Challenges in diagnosis –  Children may have it and not be aware of it –  Does not have noCceable symptoms –  InfecCous origins (second most common) –  Intraocular foreign body (rare) –  Cancer (rare) UveiCs due to Juvenile idiopathic arthriCs Pediatric UveiCs Epidemiology • 
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RetrospecCve study N=269 children 53.5 % females; 46.5 % males 82 % Caucasian 82 % born in U.S.A. 74.4 % bilateral 74.4 percent of paCents Mean age 8 yo Mean follow-­‐up 22 months • 
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Also known as juvenile rheumatoid arthriCs Commonly occurs in 7 to 12 yr old 1 out of 1000 children Chronic anterior uveiCs Symptoms-­‐ swelling of joints, pain DiagnosCc TesCng-­‐ ANA, RF, knee radiograph 20 9/9/13 Discussion Pediatric UveiCs •  Lower incidence of uveiCs in children than adults, but visual loss may be greater RelaCvely rare •  Prevalence –  Childhood uveiCs 30 cases in 100,000 –  Adult uveiCs 93 cases per 100,000 Pediatric UveiCs ComplicaCons •  band keratopathy, glaucoma, phthisis, cataract formaCon, macular edema, opCc nerve problems •  Challenges in diagnosis –  Children may have it and not be aware of it –  Does not have noCceable symptoms UveiCs due to Pediatric Orbital Pseudotumor •  Systemic signs: headache, vomiCng, anorexia, lethargy, fever in up to 50% of pediatric paCents but rarely reported in adult paCents •  Bilateral-­‐ 45% of cases •  Dx-­‐ CT-­‐scan and MRI studies-­‐diffuse orbital mass, uveoscleral thickening, proptosis, opCc nerve and EOM enlargement •  Mgmt-­‐ systemic corCcosteroid-­‐ monitor scan and lab work Chaudhry IA, Shamsi FA, Arat YO, Riley FC. Orbital pseudotumor: DisCnct diagnosCc features and management. Middle East Afr J Ophthalmol 2008; 15(1): 17–27. Recap Case 7 •  SLX Adnexa OD mild edema; OS normal Eyelids OD mild swollen; OS normal ConjuncCva OD 2+ injecCon OS white and quiet Cornea OD normal (-­‐) staining; OS (-­‐) staining Anterior Chamber OD 3+-­‐4 cells and flare; OS deep & quiet Iris OD, OS normal Lens OD+ fibrin Recap Case 7 •  CC 6 yo black female presents for
evaluation of irritated eye x 3 days;
+ pain, redness OD
•  Ocular Hx saw a pediatrician 1 mos ago; dx was
allergic conjunctivitis; txed with OTC allergy
drops
•  Med Hx – GI- today vomited, not eating;
Constitutional-lethargy
•  Meds none
•  Allergies none
Recap Case 7 •  DiagnosCc TesCng-­‐ (-­‐) ANA, RF, knee radiograph •  CT-­‐scan + orbital mass •  + response to systemic corCcosteroid •  ResoluCon -­‐ monitoring scan and lab work 21 9/9/13 Horses or Zebras? [email protected] 22