Peds Flashcards
The average axial length of the human eye in a newborn is __-__ mm. The majority of growth of the axial length occurs in the first year of life; it increases by __ mm in the first 6 months, and an additional 2 mm during the subsequent 6 months. Final axial length is reached between __ and __ years.
The average axial length of the human eye in a newborn is 14.5-15.5 mm. The majority of growth of the axial length occurs in the first year of life; it increases by 4 mm in the first 6 months, and an additional 2 mm during the subsequent 6 months. Final axial length is reached between 13 and 16 years.
Chalazia are caused by blockage of the meibomian glands. Hordeola arise form from blocked ___ or ___ glands.
Chalazia are caused by blockage of the meibomian glands. Hordeola arise form from blocked eccrine or apocrine glands.
Tenderness over the area of the ___ in the superonasal orbit may be seen in patients with acquired Brown syndrome, and symptoms are often worst when the globe is ___ and ___ on the affected side.
Tenderness over the area of the trochlea in the superonasal orbit may be seen in patients with acquired Brown syndrome, and symptoms are often worst when the globe is elevated and adducted on the affected side.
What imaging tests should be obtained in a patient with morning glory disc anomaly? (2) What conditions are you screening for? (3)
MRI/MRA brain to rule out basal encephalocele, midface anomalies, Moyamoya disease
What does PHACE stand for? What ocular condition is it associated with?
Posterior fossa abnormalities Hemangioma Arterial lesions Cardiac abnormalities Eye abnormalities
Morning glory disc anomaly
Most common cause of glaucoma-like optic nerve cupping in preterm infants?
Periventricular leukomalacia (associated with premature birth).
The optic nerve cupping is a result of transsynaptic degeneration from bilateral lesions in the optic radiations. It presents as a large cup within a normal-sized optic disc, described as an unusual form of optic nerve hypoplasia.
Exotropic Duane syndrome results from the abnormal development of the ___ nerve/nucleus. The result is anomalous innervation of the ___ rectus muscle by aberrant branches of ___ nuclei.
Exotropic Duane syndrome results from the abnormal development of the abducens nerve/nucleus. The result is anomalous innervation of the lateral rectus muscle by aberrant branches of oculomotor nuclei.
Much less common than esotropic (Type I) Duane syndrome
Surgical treatment options for exotropic Duane syndrome include ___ rectus muscle ___ in one or both eyes. However, ___ rectus muscle ___ should be avoided because this procedure often worsens globe retraction.
Surgical treatment options include lateral rectus muscle recession in one or both eyes. However, medial rectus muscle resection should be avoided because this procedure often worsens globe retraction.
The Congenital Esotropia Observational Study showed that when patients present with constant esotropia of at least __Δ after __ weeks of age, the deviations are unlikely to resolve spontaneously.
The Congenital Esotropia Observational Study showed that when patients present with constant esotropia of at least 40Δ after 10 weeks of age, the deviations are unlikely to resolve spontaneously.
Hyperopia or astigmatism greater than ___ diopters suggests a refractive strabismus.
Hyperopia or astigmatism greater than +2.50 diopters suggests a refractive strabismus.
An apparent abduction deficit may result from cross-fixation. The infant’s ability to abduct can be elicited by the ___ ___ ___ spinning the infant to stimulate the vestibulo-ocular reflex, or by ___ with either eye patched.
An apparent abduction deficit may result from cross-fixation. The infant’s ability to abduct can be elicited by the doll’s head maneuver, spinning the infant to stimulate the vestibulo-ocular reflex, or by observation with either eye patched.
Causes of ophthalmia neonatorum and time of onset (4)
Chemical - first 24 hours
Gonorrhea - first 3-4 days
Chlamydia - first week
HSV - second week
Genetic disorder most commonly associated with congenital iris ectropion
NF1
Fusional convergence eliminates ___ retinal disparity and controls an ___. Fusional divergence eliminates ___ retinal disparity and controls an ___.
Fusional convergence eliminates bitemporal retinal disparity and controls an exophoria. Fusional divergence eliminates binasal retinal disparity and controls an esophoria.
Vertical fusional vergences are used to correct a hyperphoria.
What is a useful clinical finding often seen with disc drusen versus true papilledema?
Anomalous branching of the large retinal vessels over the disc
For acquired esotropia, the maximal angle of strabismus and fusional potential can be assessed by what test?
Prism adaptation: patient fitted with prisms to induce orthophoria and reexamined every 1-2 weeks, and the prism correction is increased until a stable amount of prism to achieve orthophoria is found.
How can lymphatic malformations of the orbit present? When do exacerbations tend to occur? How are they managed?
Lymphatic malformations of the orbit may present with sudden proptosis caused by spontaneous intralesional hemorrhage. Exacerbations tend to occur during upper respiratory tract infections and can be managed with a short course of systemic corticosteroids. Surgical intervention is deferred unless vision is affected.
Interpupillary distance, if large, can result in ___.
Interpupillary distance, if large, can result in pseudoexotropia.
Most common retinal finding in patient with morning glory disc anomaly?
Retinal detachment (up to 30%!)
Neurofibromatosis (1 and 2) shows ___ ___ inheritance. However, about __% of patients do not have a family history of the disease, reflecting the high rate of new mutations in the responsible gene.
Neurofibromatosis (1 and 2) shows autosomal dominant inheritance. However, about 50% of patients do not have a family history of the disease, reflecting the high rate of new mutations in the responsible gene.
3 conditions with findings of intraocular cartilage
Patau syndrome (trisomy 13), medulloepitheliomas, PFV
Surgical approaches to DVD if small-moderate, large, and in association with IOOA (3)
Large superior rectus muscle recessions
If residual, inferior rectus muscle resection or plication
If also IOOA, inferior oblique muscle anterior transposition/anteriorization
Surgical treatment of IOOA without DVD
Inferior oblique myectomy
The nerves to the rectus muscles and the superior oblique muscle enter the muscles approximately ___ of the distance from the origin to the insertion (or trochlea). An instrument thrust more than __ mm posterior to a rectus muscle’s insertion may cause injury to the nerve.
The nerves to the rectus muscles and the superior oblique muscle enter the muscles approximately one-third of the distance from the origin to the insertion (or trochlea). An instrument thrust more than 25 mm posterior to a rectus muscle’s insertion may cause injury to the nerve.
Searching nystagmus suggests that the child has worse than ___ vision. Pendular nystagmus typically occurs in patients with visual acuity better than ___ in at least 1 eye. Jerk nystagmus often is associated with visual acuity between ___ and ___.
Searching nystagmus suggests that the child has worse than 20/200 vision. Pendular nystagmus typically occurs in patients with visual acuity better than 20/200 in at least 1 eye. Jerk nystagmus often is associated with visual acuity between 20/60 and 20/100.
What test can be used to determine potential for postoperative single binocular vision?
Amblyoscope
Tests fusional potential, retinal correspondence, detect suppression, measure primary/secondary deviations and suppression scotomas
What is the earliest sign of anterior segment ischemia following strabismus surgery?
AC cell and flare
Can also see corneal edema, irregular pupil
How does postoperative steroid treatment in pediatric cataract surgery patients with IOL placement compare to treatment in those without IOL placement?
Patients with an IOL require more aggressive steroid treatment postoperatively compared to patients without an IOL
How might leukemia present in advanced stages? (orbital manifestation)
Unilateral or bilateral proptosis
Chloroma (also known as granulocytic sarcoma or myeloid sarcoma) is a rare extramedullary variant of myelogenous leukemia that can develop as the sole manifestation of relapse after apparently successful treatment of AML
What are 3 causes of acquired Brown syndrome, and what work-up should be considered?
Local trauma to the region of the trochlea, systemic inflammatory conditions, sinusitis
CT scan should be considered
Positive angle kappa occurs when the fovea is usually slightly ___ to the pupillary axis, making the corneal light reflection slightly ___ to the center of the cornea.
Positive angle kappa occurs when the fovea is usually slightly temporal to the pupillary axis, making the corneal light reflection slightly nasal to the center of the cornea. This causes a pseudoexotropia, e.g. foveal dragging in ROP.
Congenital stationary night blindness is a group of hereditary conditions (___ most common form) causing ___ dysfunction.
Congenital stationary night blindness is a group of hereditary conditions (X-linked most common form) causing rod dysfunction, resulting in night blindness and electronegative dark-adapted ERG (large a-wave with reduced b-wave).
How do patients with CSNB typically present? How might those with the X-linked form differ?
Patients typically present in infancy with nystagmus and a normal appearing fundus. The X-linked form is the most common, and presents with myopic tilt and temporal pallor.
Compare and contrast iris stromal cysts from cysts of the iris pigment epithelium (pathogenesis and presentation)
Iris stromal cysts: enlarging congenital lesions caused by sequestration of epithelial tissues during embryogenesis. May cause visual obstruction, glaucoma, and corneal decompensation.
Cysts of the iris pigment epithelium: caused by separation of the 2 layers of the iris pigment epithelium, not by sequestration of tissue. They typically are stable and do not usually cause clinical complications.
What does CHARGE syndrome stand for, and what ocular finding is it associated with?
C – Coloboma of the eye (iris and CB colobomas), CNS anomalies
H – Heart defects
A – Atresia of the choanae
R – Retardation of growth and/or development
G – GU defects
E – Ear anomalies (bowl-shaped and concave ears, known as “lop ears”)
What are the steps of the 3-step test? What is this test used for?
(1) hypertropic eye with distance fixation
(2) misalignment worse in left or right gaze
(3) misalignment worse in left or right head tilt
Used to identify which paralytic muscle is causing cyclovertical strabismus
The 3-step test does not work with patients who have had ___ ___ surgery, have a ___ disease, or have ___ syndrome.
The 3-step test does not work with patients who have had previous strabismus surgery, have a restrictive disease (such as thyroid eye disease), or have Brown syndrome.
___ ___, disconjugate eye movements, and ___ ___ deviation of both eyes can be noted in healthy newborns but should not persist beyond __ months of age.
Skew deviation, disconjugate eye movements, and tonic downward deviation of both eyes (sunsetting) can be noted in healthy newborns but should not persist beyond 4 months of age.
Signs of poor visual development include ___ eye movements, lack of response to ___ ___, ___, and forceful rubbing or poking of the eyes.
Signs of poor visual development include searching eye movements, lack of response to familiar faces/objects, nystagmus, and forceful rubbing or poking of the eyes (oculodigital reflex).
What distinguishes type 1 Duane from 6th nerve palsy?
Limited adduction of affected eye
The ___ ___ is the primary anatomical site affected in the development of amblyopia.
The visual cortex is the primary anatomical site affected in the development of amblyopia.
___ ___ or ___ ___ would be associated with the sensation of a soft stop while probing.
Lacrimal tumor or canalicular obstruction would be associated with the sensation of a soft stop while probing.
Paradoxical pupils, a pupillary constriction in response to darkness, are most commonly associated with ___ ___, although they can occur with ___ ___.
Paradoxical pupils, a pupillary constriction in response to darkness, are most commonly associated with retinal dystrophies, although they can occur with optic neuropathies.
Delayed visual maturation is the condition wherein normal visual ___ and ___ do not develop in an infant within the first __ months of life.
Delayed visual maturation is the condition wherein normal visual fixation and tracking do not develop in an infant within the first 3 months of life.
Cortical or cerebral visual impairment is a condition in which visual deficits result from pathology posterior to the ___ ___ ___.
Cortical or cerebral visual impairment is a condition in which visual deficits result from pathology posterior to the lateral geniculate nucleus.
What is the inheritance pattern for most patients with primary congenital glaucoma?
Sporadic
Craniosynostosis characterized by clover leaf skull
Pfeiffer syndrome
Clinical findings of the 3 types of Duane syndrome
Type 1: most common form, poor abduction and esotropia, narrowing of palpebral fissure on attempted adduction
Type 2: limited adduction and exotropia
Type 3: poor abduction and adduction; either esotropia or exotropia may result
The 2 strongest risk factors for ROP are ___ ___ and ___ ___.
The 2 strongest risk factors for ROP are gestational age and birth weight.
Optic neuritis in children, in contrast with that in adults, is more frequently ___ and associated with ___ ___.
Optic neuritis in children, in contrast with that in adults, is more frequently bilateral and associated with disc edema.
Congenital anomalies:
Agenesis is a developmental ___
Hypoplasia is a developmental ___
Hyperplasia is developmental ___
Dysraphia is a failure to ___
Agenesis is a developmental failure (anophthalmos).
Hypoplasia is a developmental arrest (optic nerve hypoplasia).
Hyperplasia is developmental excess (distichiasis)
Dysraphia is a failure to fuse (choroidal coloboma).
Others:
Failure to divide or canalize: congenital NLDO
Persistence of vestigial structures: PFV
___, ___, and ___ constitute the classical clinical triad of primary congenital glaucoma.
Epiphora, photophobia, and blepharospasm constitute the classical clinical triad of primary congenital glaucoma.
What is the basic pathologic defect of congenital glaucoma?
Increased resistance to aqueous outflow through the trabecular meshwork due to abnormal development of neural crest–derived tissue of the anterior chamber angle
What are the secondary diagnostic positions?
Straight up, straight down, right gaze, left gaze
What are the tertiary diagnostic positions?
Up and right, up and left, down and right, down and left, right and left head-tilt
What feature of morning glory disc anomaly differentiates it from optic disc coloboma?
Increased number of blood vessels coming off the disc
Y pattern strabismus (pseudo-overaction of the inferior obliques) results from aberrant innervation of the ___ ___ muscles in ___.
Y pattern strabismus (pseudo-overaction of the inferior obliques) results from aberrant innervation of the lateral rectus muscles in upgaze.
(There is no fundus torsion, no difference in vertical deviation on head tilt, and no superior oblique underaction.)
X patterns are usually associated with ___ and ___ in ___.
X patterns are usually associated with overelevation and overdepression in adduction.
What is Panum’s area?
Space within which objects that fall on disparate retinal points are seen singly
What are conditions associated with these nystagmus syndromes?
- Fusional maldevelopment nystagmus
- Congenital vertical nystagmus
- Periodic alternating nystagmus (2)
- See-saw nystagmus
Fusion maldevelopment nystagmus (latent): infantile esotropia
Congenital vertical nystagmus: inherited retinal dystrophies
Periodic alternating nystagmus: albinism and Arnold-Chiari malformation
See-saw nystagmus: rostral midbrain lesion such as a craniopharyngioma
When is an A pattern considered clinically significant? V pattern?
A pattern: difference in measurement between upgaze and downgaze is at least 10 prism diopters
V pattern: difference at least 15 prism diopters
What structures do Leber congenital amaurosis affect? What ancillary testing would you obtain, and what do you expect it to show?
Leber congenital amaurosis is a group of hereditary retinal dystrophies that affect both the rod and cone photoreceptors. An ERG typically shows extinguished waveforms.
Wedge-shaped cortical cataract is ___ or associated with occult ___ ___ ___, previous ___ ___, or retinal ___.
Wedge-shaped cortical cataract is idiopathic or associated with occult posterior segment tumor, previous blunt trauma, or retinal coloboma.
Ocular findings in Goldenhar syndrome can include ___ ___, ___, upper eyelid coloboma, iris abnormalities, and corneal ___.
Ocular findings in Goldenhar syndrome can include Duane syndrome, microphthalmia, upper eyelid coloboma, iris abnormalities, and corneal hypoesthesia.
Indications for NLD probing in congenital NLDO (3)
(1) NLDO that fails to resolve with age and conservative treatment
(2) Dacryocystocele in the newborn that is unresponsive to massage
(3) Previously failed NLD probing procedure
Ocular motor recordings in patients with:
- Infantile nystagmus syndrome
- Latent nystagmus
Infantile nystagmus syndrome: accelerating slow phase (diagnostic)
Latent nystagmus: 2 types of slow phases, linear and decelerating
3 major classification categories of visual dysfunction in infants and children
(1) Delayed visual maturation
(2) Pregeniculate visual impairment (pathology anterior to LGN)
(3) Retrogeniculate visual impairment (pathology posterior to LGN), aka cerebral visual impairment
Most frequent cause of childhood visual impairment in developed countries
Retrogeniculate visual impairment (amblyopia considered a cause of this)
Origins of:
- Superior oblique
- Inferior oblique
- Remaining extraocular muscles
Superior oblique: structural origin is orbital apex above the annulus of Zinn. Functional origin is the trochlea
Inferior oblique: orbital rim
Remaining extraocular muscles: annulus of Zinn
What is the difference between hypertelorism and exorbitism?
Hypertelorism is the excessive distance between the medial orbital walls and is detected radiographically.
Exorbitism is prominent eyes due to shallow orbits or an increased angle of divergence of the orbital walls.
In ___, the skull is long in anterior-posterior axis and narrow bitemporally, usually from premature closure of the ___ suture.
In scaphocephaly, the skull is long in anterior-posterior axis and narrow bitemporally, usually from premature closure of the sagittal suture.
Patients with infantile esotropia have asymmetric smooth pursuit, which is better in the ___-to-___ direction.
Patients with infantile esotropia have asymmetric smooth pursuit, which is better in the temporal-to-nasal direction.
What distinguishes congenital fibrosis syndrome from CPEO?
Progressive loss of EOM over time with CPEO
Distinguish clinical optic nerve appearances of:
- Dominant optic atrophy
- Leber hereditary optic neuropathy
- Optic nerve hypoplasia
Dominant optic atrophy: temporal pallor with an area of triangular excavation
LHON: peripapillary telangiectasia
ONH: yellow peripapillary halo with a ring of pigmentation
If restriction persists after the repair of the orbital floor and release of the entrapped muscle, ___ of the restricted muscle is the next step.
If restriction persists after the repair of the orbital floor and release of the entrapped muscle, recession of the restricted muscle is the next step.
DVD should be treated if it manifests frequently during the day and is noticeable (__ to __ prism diopters).
DVD should be treated if it manifests frequently during the day and is noticeable (6 to 8 prism diopters).
Demographic and epidemiologic characteristics of pediatric uveitis: slight \_\_\_ (male/female) preponderance, mostly \_\_\_ (unilateral/bilateral), age \_\_-\_\_ at diagnosis, \_\_\_ (yes/no) family history
Slight female preponderance, mostly bilateral, age 8–9 at diagnosis, no family history
Patients with pseudoxanthoma elasticum have a higher incidence of what optic nerve abnormality?
Disc drusen
___ of the lateral rectus muscle is the preferred surgical therapeutic option for upshoots and downshoots in patients with Duane syndrome.
Y-splitting of the lateral rectus muscle is the preferred surgical therapeutic option for upshoots and downshoots in patients with Duane syndrome.
Latent nystagmus is a ___, ___ jerk nystagmus that reverses direction with change in fixation. A ___ jerk nystagmus occurs with when the right eye is covered, and a ___ jerk occurs when the left is covered.
Latent nystagmus is a conjugate, horizontal jerk nystagmus that reverses direction with change in fixation. A left jerk nystagmus occurs with when the right eye is covered, and a right jerk occurs when the left is covered.
Extorsion of __ degrees or more in downgaze, or more than __ degrees of extorsion in primary position is highly suggestive of bilateral superior oblique palsies.
Extorsion of 10° or more in downgaze, or more than 15° of extorsion in primary position is highly suggestive of bilateral superior oblique palsies.
Ocular abnormalities in fetal alcohol syndrome (6)
Short palpebral fissures Telecanthus Strabismus (esotropia) Ptosis Optic nerve hypoplasia (uni- or bilateral) Retinal vascular tortuosity
Searching eye movements and variable visual attentiveness with normal exam suggest ___ ___ ___.
Searching eye movements and variable visual attentiveness with normal exam suggest cerebral visual impairment.
Inheritance of ectopia lentis et pupillae
Autosomal recessive
Surgical approach for infantile esotropia (V-pattern esotropia and dissociated vertical deviation)
Bilateral medial rectus recessions and inferior oblique recessions
Inheritance of oculocutaneous albinism? Ocular albinism?
Autosomal recessive; X-linked recessive
Surgical approach for right superior oblique palsy
Right inferior oblique recession (lessens right hypertropia most effectively because worse in left gaze)
Right inferior rectus resection can be considered, but not as effective because deviation is worst in gaze up and left
Weakening procedures for superior oblique muscle
Tenotomy, tenectomy, split-tendon lengthening, spacer, recession
To test for horizontal deviation using the Maddox rod, what direction should cylinders be oriented in front of right eye? What does the patient see, and what are the possible findings?
Horizontally (180 degrees)
Patient sees vertical line with right eye, point source of light with left eye
If point is right of line, exodeviation
If point is left of line, esodeviation
Most common strabismus associated with craniosynostosis
V-pattern exotropia
Most common inheritance pattern for isolated congenital cataracts
Autosomal dominant
What is the course and prognosis of juvenile xanthogranuloma?
Self-limited; resolves spontaneously by the age of 5 years old
What muscles are supplied by the medial muscular branch of the ophthalmic artery?
Medial rectus, inferior rectus, inferior oblique
What muscles are supplied by the lateral muscular branch of the ophthalmic artery?
Lateral rectus, superior rectus, superior oblique, levator palpebrae superioris
At what age should an infant maintain fixation and react with facial expressions?
6-8 weeks
What family of disorder is Bardet-Biedl syndrome?
Ciliopathy (retinal degeneration, renal disease, cerebral anomalies)
Most common age for onset of accommodative esotropia
1-3 years
What material are extraocular muscle pulleys primarily made of?
Elastin
What feature characterises central fusion disruption?
Misalignment of visual axes and intractable diplopia
What eye movement abnormality can occur in healthy infants in the first months of life?
Intermittent esotropia
Prevalence of amblyopia in the North American population
2-4%
Following congenital cataract surgery, what finding predisposes a child to glaucoma?
Small corneal diameter (micro cornea, persistent fetal vasculature)
Clinical findings of Brown syndrome (4)
Limited elevation in adduction that improves in abduction but not completely, adduction causes widening of the palpebral fissure and downshoot of the involved eye, hypotropia of the involved eye in primary, V pattern due to divergence on upgaze from tight superior obliques
Work-up and management of Brown syndrome
Positive forced duction testing confirms diagnosis
May be congenital or acquired, constant or intermittent. Work up acquired cases with CT scan.
Management can be medical and/or surgical. May also spontaneously resolve.
Most common finding in patients with congenital rubella? Visual prognosis?
Bilateral pigmentary retinopathy
Good if no cataract, microphthalmia, or glaucoma
Weakening procedure for inferior oblique
Anterior transposition or anteriorization
How can you preserve the torsional action of the superior oblique when doing a weakening procedure?
Tenotomy of only the posterior 75%–80% of the tendon to preserve torsion, which is controlled by the most anterior tendon fibers
Lymphocytic choriomeningitis virus can cause severe ___ infections, and is transmitted via exposure to ___. Ocular findings include ___ and ___ ___ scarring.
Lymphocytic choriomeningitis virus can cause severe congenital infections, and is transmitted via exposure to rodents. Ocular findings include macular and chorioretinal peripheral scarring (similar in morphology and distribution to that of congenital toxoplasmosis).
What is the primary purpose of the 4-diopter base-out prism test?
To document a facultative scotoma in a patient with no manifest deviation
What is anomalous retinal correspondence? How is this an adaptation?
Anomalous retinal correspondence is a condition where the fovea of the fixating eye has acquired an anomalous common visual direction with a peripheral retinal element in the deviating eye. It is an adaptation that restores some degree of binocular cooperation despite manifest strabismus.
