PEDS Flashcards
Megalocornea: 1) Indicates a corneal diameter of what?
2) Uni or bilateral?
3) Inheritance?
1) 13mm or greater
2) Bilateral (usually)
3) X-linked inheritance
What late ocular changes are found in children with primary megalocornea?
1) Corneal Mosaic Degeneration
2) Arcus juvenilis
3) Presenile cataracts
4) Glaucoma
- Nonprogressive enlargement of the K
Homocystinuria: 1) how is the diagnosis confirmed?
2) Inheritance?
1) Detection of disulfides in the urine
2) AR
(also a/w lens dislocation, CNS and skeletal defects)
Sulfite oxidase deficiency
1) also known by what name?
2) what are the findings in urine?
1) molybdenum cofactor deficiency
2) Increased excretion of sulfite (enzyme deficiency interferes with conversion of sulfite to sulfate
A patient has ectopia lentis and increased urinary sulfites, what is the likely diagnosis?
Sulfite oxidase deficiency (AKA molybdenum cofactor deficiency)
What is the preferred laboratory testing in a child with unilateral congenital cataract?
None is typically needed
-Unilateral is usually not a/w occult systemic or metabolic disease
Bilateral congenital cataracts:
isolated inherited cataracts have what inheritance pattern?
AD
What is the MC ophthalmologic manifestation of abusive head trauma?
Retinal hemorrhages (bilateral)
- incidence is 80%
- hemorrhages are preretinal, intraretinal, subretinal
Patients with refractive accommodative esotropia have an average of how many diopters of hyperopia?
1) +4.00 D hyperopia
compared to High AC/A ratio esotropia which averages +2.25 D
In High AC/A ratio esotropia, the how does the deviation at near compare to the deviation at distance?
1) The deviation is present only at near or is much larger at near
- In refractive accommodative esotropia, the angle of esotropia is roughly the same at distance and near fixation
Congenital esotropia is present by what age?
Present by 6 months of age
also known as infantile esotropia
When selecting an IOL for a small child undergoing cataract sx, the target refraction must account for the _____ shift that occurs with growth
MYOPIC shift that occurs with growth
- Child’s eye elongates throughout 1st decade and beyond.
Most surgeons implant IOLs with powers that children are anticipated to require in adulthood.
Target refraction also takes into account consideration of the fellow eye and risk of amblyopia
Which cause of leukocoria is a/w microphthalmos?
PFV
What other ocular findings/issues are a/w PFV?
1) Microphthalmos
2) elongated ciliary processes
3) cataract
4) RD
5) Angle-closure glaucoma
Nasolacrimal duct obstruction:
1) Incidence?
2) Usually caused by what?
3) Early treatment includes what?
1) 5-20% newborns
2) Persistent membrane at the valve of Hasner
3) Lacrimal sac massage and antibiotic usage, often spontaneously resolves in first 9-12 months,
What are the signs of a CN3 palsy?
Limitation of adduction, elevation, depression, ptosis, fixed/dilated pupil
- eye is usually turned down and out
What is the primary deviation referring to when discussing strabismus? Secondary deviation?
1) Primary = The amount of strabismus present when the normal eye is fixating
2) Secondary = amount of strabismus with the paretic eye fixating
(Secondary deviation > Primary deviation)
ON Hypoplasia is the MC developmental optic disc anomaly and has what characteristic appearance?
1) Disc may be pale, gray, small, vascular tortuosity
2) Yellow-to-white ring around the disc (known as double-ring sign) may be present
ON hypoplasia is characterized by a decreased # of ON axons and is a/w visual acuity within what range?
20/20 to NLP
What is the inheritance of ON Hypoplasia?
What syndromes or abnormalities can it be a/w with?
1) Usually idiopathic and sporadic
2) More prevalent in Fetal Alcohol Syndrome, maternal ingestion of phenytoin, quinine and LSD
3) Segmental ONH 2/2 maternal DM
4) ONH may be a/w CNS abnorm, Pit gland dysfunction
Periventricular leukomalacia in children can have what findings of the optic nerve?
ONH that may be mistaken for glaucomatous cupping
Morning glory disc anomaly (MGDA) is more common in girls and is typically unilateral has been a/w what type of retinal detachment?
Serous RD in 1/3 of cases
What other abnormalities is MGDA associated with?
1) Basal encephalocele
2) PHACE syndrome
3) carotid circulation abnormalities (moyamoya disease)
What abnormalities are found in PHACE syndrome?
1) Posterior fossa malformations
2) Hemangiomas
3) Arterial lesions
4) Cardiac anomalies
5) Eye anomalies
What imaging should be considered in Morning Glory Disc Anomaly?
1) MRI Brain
2) MRA
What is the MC cause of visual impairment in children with Abusive head trauma?
Cortical or cerebral injury (neurologic damage)
- retinal and optic nerve injury are less-common
1mm of decentration of the corneal light reflection corresponds to how many diopters of deviation using Hirschberg?
15 D per 1 mm (or approx 7 degrees)
An esotropic eye will produce a light reflex that is displaced in what direction from the center of the pupil?
Temporally
The greatest risk of rebleeding in a traumatic hyphema occurs how long after initial injury?
3-7 days
A 4 year old has lytic bony changes on CT and superotemp orbital mass. Biopsy shows fibrous connective tissue and an infiltrate of eosinophils and histiocytes. What is the diagnosis?
1) Eosinophilic Granuloma
- Usually located in superotemp orbit
- commonly results in lytic bony lesions of the orbital roof
- diabetes insipidus is a possible systemic manifestation
What is a possible systemic manifestation of Eosinophilic granuloma?
Diabetes insipidus
What is another name for Fusion maldevelopment nystagmus syndrome (FMNS)?
Latent nystagmus
Latent nystagmus (AKA Fusion maldevelopment nystagmus syndrome) is a marker of fusion maldevelopment such as in infantile esotropia. Describe the exam findings when 1 eye is occluded?
A conjugate jerk nystagmus occurs with the fast phase toward the fixating eye.
- this is the only form of nystagmus that changes direction with a change in fixation
Congenital vertical nystagmus is associated with what type of ocular disease?
Inherited retinal dystrophies
Periodic alternating nystagmus (PAN) may be seen in what conditions?
1) Albinism
2) Arnold-Chiari Malformation
See-saw nystagmus may be associated with a lesion where?
Rostral midbrain lesions such as craniopharyngioma
What is the most frequent cause of childhood visual impairment in developed countries?
Retrogeniculate impairment - such as amblyopic visual deficits which results primarily from visual cortical changes
What is delayed visual maturation?
Normal visual fixation and tracking does not develop within the first 3 months of life
What gram stain findings would be seen in N. gonorrhoeae
GNID (gram negative intracellular diplococci)
How is low serum IGF-1 related to ROP severity?
positively correlated with severity