Ears, Eyes, Teeth Flashcards

Question

Mastoiditis - general

Answer 1

1. Classification a. Acute mastoiditis i. Complication of AOM with retroauricular inflammation & protrusion of auricle ii. Patients are younger iii. Inflammation of post-auricular area, with displacement of the pinna inferiorly and anteriorly iv. Strep pneumoniae and strep pyogenes v. Diagnosis – CT of temporal bones b. Chronic mastoiditis i. Extensive history of OM, including grommets ii. Patients are older iii. Less than 50% have retroauricular swelling iv. Most likely cause is pseudomonas aeruginosa 2. Treatment a. Myringotomy b. Mastoidectomy c. IV antibiotics 3. Complications a. Petrositis – temporal bone involvement where eye pain prominent symptom due to irritation of ophthalmic branch CN5 b. Gradenigo syndrome – triad suppurative OM, paralysis external rectus and eye pain on same side

Answer 2

= Benign paroxysmal positional vertigo a. Disorder of early childhood manifested by recurrent episodes of brief disequilibrium i. A family history of migraine headaches is frequently present b. Pathogenesis i. Particles form in the semicircular canals (most often posterior) c. Clinical manifestations i. During the attacks, the child appears frightened and off balance ii. Associated with nystagmus, diaphoresis, nausea, and vomiting iii. Vertigo or dizziness iv. Episodes usually last less than a minute and are not associated with an altered consciousness d. Natural history i. Episodes recur in clusters, occurring daily for several days in a row, then remitting for several weeks, and recurring again ii. Spontaneously remits by age 5 iii. Many patients subsequently develop typical migraine headaches e. Treatment = Hallpike manoeuvre

Answer 3

1. Function + anatomy a. Immunologically reactive tissue at the entrance to the aerodigestive tract b. Framework of fibrous tissue and lymphocytes, covered by epithelium c. Waldeyer’s ring – circle of lymphoid tissue i. Lingual, Palatine, Pharyngeal, Lateral pharyngeal d. Role in immunity from exposure to both inhaled + ingested antigens e. Composed of mainly B lymphocytes f. Exposure to Ag produces secretory Ab + lymphokines g. Removal does NOT produce significant immunological problems 2. Indications for T + A a. Recurrent tonsillitis i. 7 infections in one year ii. 5 infections/year for 2 consecutive years iii. 3 infections/year for 3 consecutive years iv. >2 weeks missed school b. Recurrent quinsy c. OSA i. Severity NOT proportional to tonsil size ii. Combination of anatomical + neuromotor factors iii. Present in 2% of young children – peak 2-8 years iv. Most common groups 1. Marked tonsil + adenoid hypertrophy without obesity 2. Obesity with milder upper airway lymphoid hyperplasia 3. Craniofacial + neuromuscular disorders – Crouzon and Apert, T21, CP d. Suspected malignancy (Fanconi anaemia)

Answer 4

1. Indications a. Airway obstruction b. Prolonged ventilation c. Airway toileting d. Airway surgery 2. Complications a. Early i. Stomal/tracheal granuloma ii. Obstructed tube iii. Haemorrhage iv. Pneumothorax v. Pneumomediastinum vi. Infection b. Late i. Stoma/tracheal granuloma ii. Tracheal stenosis iii. TOF iv. Erosion into vessels v. Tracheocutaneous fistula

Answer 5

``` Birth • Brief fixation on face, light, object • No contrast, no colour differentiation • Hyperopic (farsighted) • VA 20/400 • May have imperfect coordination of eye movements + alignment during early days and weeks • Test = visual evoked potential ``` 6 weeks • Maintain fixation on face, object or light and follow through 90 degrees • Smiling and visual response (especially to a face) • Test = fix and follow using bright objects (180 degrees) 6 months • Reach for small object • Actively follow object • Proper eye coordination – if noted >6 months should b referred • Test = identify and pick up small object One year • Vision similar to adult • Test = Reach for a tiny object (eg. 100 and 1000s) 2-3 years • VA 20/40 • Test = Picture matching (Kay picture test) 3-4 years • VA 20/30 • Test = Letter matching (Sheridan Gardiner test), Tumbling E test, HOV test (modified Snellen) 5 years • VA 20/20 • Test = Snellen acuity, LogMAR/STYCAR tests, Tumbling E test 5. When to refer a. Suspect poor or subnormal vision b. Visual acuity of 6/12 or worse c. Nystagmus at 8-12 weeks of age can be a sign of poor vision

Answer 6

1. Key points a. Decrease in visual acuity (unilateral or bilateral) that occurs in visually immature children b. Occurs due to a lack of a clear image projecting onto the retina c. Amblyopia occurs ONLY during the critical period of development before the cortex is visually mature – within the first decade of life d. The younger the child, the more susceptible to amblyopia 2. Pathogenesis a. Developmental of visual acuity normally proceeds rapidly in infancy + early childhood b. Anything that interferes with the formation of a clear retinal image during this time can produce amblyopia 3. Aetiology + classification a. Strabismic (50%) i. Due to a deviated eye ii. Abnormal interaction between two foveas -> different + unfusable images iii. Visual cortex suppresses the image from one eye b. Refractive/ ametropic (15-20%) i. Unequal refractive errors -> one eye not focused on the fovea c. Anisometropic - unequal need for vision correction d. Derivational (<5%) i. Congenital cataracts ii. Vitreous haemorrhage iii. Severe refractory error e. 15-20% is combined 4. Treatment a. Remove media opacity b. Glasses c. Good eye is covered (occlusion therapy) or blurred with glasses (fogging) or drops – stimulates the proper visual development of the more affected eye

Answer 7

Sensitive measure of retinal and/or optic nerve dysfunction. | Direct pupil dilates when light shined in that eye.

Answer 8

= farsightedness [can see far] o Parallel rays of light come to focus posterior to the retina o Results from short AP diameter of eye or lower refractive power of cornea or lens o Majority of children hyperopic at birth o Manifests as eye strain, headaches or fatigue o Results in great accommodative effort o If not treated can result in bilateral amblyopia (ametropic amblyopia) or esotropia

Answer 9

= near sightedness [can see near] o Parallel rays of light come to focus anterior to the retina o Results from long AP diameter of the eye or higher refractive power of the cornea or lens o Tend to squint – as results in improved VA (pinhole effect) o Infrequent in infants and preschool aged children o More common in infants with retinopathy of prematurity o Hereditary tendency o Degree of myopia increases with age

Answer 10

= refractive powers of the various meridians of the eye differ o Most cases due to irregularity in the curvature of the cornea o Mild cases do not require treatment and are asymptomatic o With greater degrees can result in visual distortion o May require glasses Causes consistent blurred vision both near and far (but as above often mild/asymptomatic)

Answer 11

= differences in refractive state of each eye | o If uncorrected can develop amblyopia due to constantly being out of focus

Answer 12

o Accommodation = ciliary muscle contracts, the suspensory fibers of the lens relax, and the lens assumes a more rounded shape increasing the lens power o Amplitude of accommodation is greatest in childhood and gradually decreases with age (= presbyopia) o Disorders in accommodation is relatively rare

Answer 13

1. Definitions a. Vision impaired = VA 20/70 – 20/200 i. Prevalence 1/500 school aged children b. Blindness = VA >20/200 2. Epidemiology a. Severe visual impairment (corrected vision poorer than 6/60) and blindness in children is uncommon b. Incidence 2.5 per 100,000 children c. Incidence highest in developing countries, LBW infants and in first year of life 3. Aetiology a. Mild-moderate i. Usually refractory errors 1. Most commonly myopia near sighted b. Blindness/severe impairment i. Most common is retinopathy of prematurity ii. Cataracts iii. Albinism iv. Congenital infection v. HIE vi. Hydrocephalus ``` Key Question – Is there nystagmus? • If no o Cortical visual impairment o Delayed visual development o COMA – oculomotor apraxia; abnormality in enervation of saccades, cannot generate rapid eye movements; blink end fixation • If yes o Anterior – cataract, aniridia, albinism, cornea o Posterior segment pathology ```

Answer 14

• Generally, results from misalignment of the visual axes • Binocular o Occluding either eye relieves the diplopia if it is binocular in effect o Affected eye commonly squint, cover one eye with a hand, or assume abnormal head tilt o Onset of diplopia requires full evaluation o DDX  Increased ICP/ brain tumour  Infection  Migraine  GBS  Orbital mass • Monocular o Monocular diplopia results from dislocation of the lens, cataract, dry eyes or defect in media or macula o Covering the non-diplopic eye will not relieve symptoms o May be psychological

Answer 15

= night blindness ``` • Vision that is defective in reduced illumination • Aetiology o Inherited – AD, AR, X linked o Vitamin A deficiency o Drugs – quinine ```

Answer 16

1. Amaurosis = partial or total loss of vision; describes profound impairment, blindness or near blindness 2. Aetiology a. Congenital (neurological, developmental malformation, infection, hypoxia, trauma) b. Acquired i. Ocular disease 1. Cataract 2. Chorioretinitis 3. Retinoblastoma 4. Retinitis pigmentosa ii. Rapid onset 1. Infectious process 2. Vasculitis 3. Migraine 4. Leukaemia 5. Demyelination 3. Clinical manifestations a. Nystagmus or strabismus b. Timidity, clumsiness, behavioural change 4. Investigations a. Complete ophthal assessment b. Imaging

Answer 17

• Visual impairment caused by a brain problem rather than eye problem ``` • Aetiology o Hypoxia o Developmental brain abnormality o Head injury o Infections – meningitis, encephalitis ```

Answer 18

• Describes infants who do not exhibit the ability to fix or follow objects in the environment o Child appears blind – unable to focus attention, fixate or follow • Normal ocular and neurological examination • Improves by age of 6 months without treatment • Pathophysiology unknown

Answer 19

a. Hypoplastic iris tissue b. May be associated with other eye abnormalities – macular and optic nerve hypoplasia c. Bilateral in 98% of cases d. Aetiology = genetic i. 2/3 of cases AD ii. 1/3 of cases sporadic e. Consequences i. Glaucoma – develops in 75% ii. Wilm’s tumour – develops in 20% of individuals with sporadic aniridia 1. Gene for aniridia close to the Wilm’s tumour gene f. Management i. Screening using renal USS 3-6/12 until 5 years of age if there is an 11p13 region deletion

Answer 20

a. Developmental defect of the iris – defect of a sector, hole or notch in the pupillary margin b. May be associated with other abnormalities c. Always located inferiorly d. AD in most cases

Answer 21

a. Pupils appear dilated and do not constrict significantly to light or near gaze b. Respond minimally to miotic agents c. Iris is otherwise normal d. Affected individuals usually healthy

Answer 22

a. Inequality of pupils – due to local or neurological disorders b. Note i. If inequality is more pronounced in the presence of bright focal illumination or near gaze – defect in pupillary constriction and the larger pupil is abnormal (CNIII) ii. If inequality is more pronounced with reduced illumination – defect in dilation the smaller pupil is abnormal (Horner’s syndrome) c. Aetiology i. Neurological = parasympathetic or sympathetic lesions 1. CNIII palsy 2. Horner’s syndrome ii. Local causes 1. Synechiae = adhesions 2. Congenital iris defects

Answer 23

a. Paradoxical constriction of the pupils to darkness b. Mechanism unclear c. Aetiology i. Night blindness ii. Albinism iii. Retinitis pigmentosa iv. Leber congenital retinal amaurosis

Answer 24

a. The irides are of different colour b. Simple heterochromia AD c. Congenital heterochromia also a feature of Waardenburg syndrome i. AD ii. Lateral displacement of the inner canthi and puncta iii. Pigmentary disturbances – median forelock and patches of hypopigmentation of the skin iv. Defective hearing

Answer 25

a. Includes any white pupillary reflex b. Aetiology i. Cataract ii. Chorioretinal coloboma iii. Retinoblastoma iv. Persistent hyperplastic primary vitreous v. Cicatricial retinopathy of prematurity vi. Retinal detachment xi. Leukaemia

Answer 26

a. Commonly seen in NF b. Melanocytic hamartomas of the iris c. No visual disturbance d. Found in 92-100% of individuals >5 years of age e. Slit-lamp may be required

Answer 27

1. Key points a. Misalignment of the eyes b. Definition = abnormality in ocular alignment resulting in inadequate binocular vision and loss of depth perception c. Most common eye problem in children – affects 4% of children <6 years d. Can result in vision loss (amblyopia) + psychological effects e. 30-50% develop amblyopia f. Restoration of proper visual axis essential for normal binocular vision 2. Classification a. Esotropia / convergent – inward rotation, with nasal deviation relative to fixated eye b. Exotropia / divergent – outward rotation, with temporal deviation relative to fixated eye c. Hypertropia – upwards rotation d. Hypotropia – downwards rotation 3. Consequences a. Child i. Amblyopia ii. Loss of binocular depth perception b. Adults i. Diplopia c. Children + adults i. Head may be turned to maximise binocular function - abnormal or compensatory head posture

Answer 28

a. Benign and common i. Primary failure of binocular vision ii. Abnormality of convergence or divergence iii. Refractory error b. Loss of vision in one eye i. Trauma ii. Eye – Retinoblastoma, cataract, prematurity iii. CNS – hydrocephalus, increased ICP with tumor, CN 6 palsy (esotropia), CN3 palsy (exotropia), CNS infection iv. NM – GBS, myasthenia, MS, botulinism v. Acquired - Grave’s disease, drugs, toxins c. Genetic component d. Syndromes - monocular elevation deficiency - Duane syndrome (retraction of the globe on adduction) - Mobius syndrome (congenital facial paresis, bilateral/asymmetric/incomplete) - Brown syndrome - Parinaud syndrome

Answer 29

6. Clinical manifestations a. Head posturing b. Strabismus 7. Key complications = amblyopia a. Amblyopia is a functional reduction in VA caused by visual dysfunction during critical period of eye development b. Head postures c. Loss of binocular depth position 8. Patterns a. Esotropia = convergent (2/3 most common) i. Can occur as normal variant <6months ii. Hyperopia in one eye – usual onset 0-3years 1. Begins as intermittent or latent (worse when tired), gets worse with looking at near objects iii. Pathological causes (as per aetiology card) 1. Suspect if late onset and constant 2. Constant, no change with near/far objects b. Exotropia = divergent (1/3) i. Congenital – present from infancy ii. Myopia in one eye – usual onset >4years 1. Intermittent, latent, worse when looking at far away objects iii. Pathological causes 1. Suspect if late onset and constant

Answer 30

a. Visual acuity – cover eye test assumes VA b. Eye movements – cover eye test assumes movements c. Red reflex – cataract/Rb/coloboma d. Fundoscopy and slit lamp Ex for intraocular pathology e. Observation i. Test for pseudostrabismus (appearance of strabismus due to flat nasal bridge, epicanthal folds, closely spaced eyes) = Hirschberg corneal light reflex test – shine light onto corneal surface at distance of 1m 1. Symmetry on both sides = pseudostrabismus 2. Asymmetry = strabismus ii. Head posturing 1. Multiple causes 2. Can differentiate eye problem from muscular – cover eyes and if eyes then will correct, if binocular vision and cover one eye then will correct f. Cover/uncover eye test i. Cover 1. If tropia, affected eye will fix when normal eye covered ii. Uncover 1. If tropia and there is immediate return to previous strabismus then can assume decreased VA in affected eye 2. If tropia and there is delay in return to previous strabismus then assume equal VA between eyes g. Other i. Measure vision (including Stereoacuity) ii. Document diplopia iii. Measure deviation iv. Measure refractive index v. Examine for other diseases

Answer 31

a. Aims of treatment i. Treat amblyopia ii. Minimise/ overcome diplopia iii. Maximise binocular depth perception iv. Improve binocular visual field (convergent squints only) v. Improve cosmesis b. Types i. Correct refractive errors with glasses ii. Eye patch 1. Indicated if amblyopia 2. First line in preference to surgical management 3. Remove patch for 2 hours each day iii. Surgery

Answer 32

1. Key points a. Nystagmus = rhythmic oscillation of one or both eyes b. Caused by abnormality in any one of the 3 mechanisms which maintain position + movement of eye i. Fixation ii. Conjugate gaze iii. Vestibular mechanism c. Name the nystagmus by naming the fast movement d. Pathological nystagmus more likely dysconjugate e. NOTE: INO = abducting nystagmus + adducting defect 3. Aetiology a. Idiopathic b. Chiasmal misrouting = albinism, achiasma c. Associated with other ocular disease i. Achromatopsia ii. Congenital stationary night blindness iii. Ciliopathies d. Neurological abnormalities – eg. T21, Noonan, brain tumours, periventricular leukomalacia 4. Classification a. Motor i. Congenital nystagmus ii. Variably reduced vision 1. Vision not determined by how fast or how much it moves – determines on how often it fixates 2. Only needs to be still for 100th of a second to perceive things iii. Null point with compensatory head position b. Sensory i. Anything that reduces vision significantly in the first 3 months can cause nystagmus ii. Albinism iii. Untreated bilateral cataract iv. Retinal abnormality

Answer 33

a. Complete closure of the lids over the globe is difficult or impossible b. Exposure of the eye may result in drying, infection, corneal ulceration or perforation of the cornea c. Aetiology i. Facial nerve palsy ii. Thyrotoxicosis iii. Structural – due to burns or injury to the eyelid iv. Craniosynostosis

Answer 34

a. Ectropion = eversion of the lid margin b. Entropion = inversion of the lid margin c. Epiblepharon = commonly seen in children; roll of skin beneath the lower eyelid lashes causes the lashes to be directed vertically and touch the cornea

Answer 35

a. Inflammation of the lid margin b. Commonly bilateral and chronic or recurrent c. 2 main types = staphylococcal, seborrheic

Answer 36

a. Infection of the glands of the lid may be acute or subacute b. Usually caused by staphylococcal aureus

Answer 37

= meibomian cyst a. Granulomatous inflammation of a meibomian gland b. Characterised by firm, non-tender nodule in the upper or lower eyelid c. May require surgery d. Often spontaneously resolve

Answer 38

= congenital nasolacrimal duct obstruction 1. Key points a. Most common disorder of lacrimal system b. Occurs in 20% of newborn infants c. Usually caused by failure of canalization of duct d. Partial or complete 2. Clinical manifestations a. Excessive tear lake b. Overflow of tears c. Reflux of mucoid material that is produced in the lacrimal sack d. Erythema or maceration of the skin due to irritation and rubbing 3. Risk factors a. T21 f. CHARGE syndrome g. Goldenhar syndrome Other syndromes 4. Complications a. Dacrocystitis = inflammation and infection b. Pericystitis = inflammation of surrounding tissue c. Periorbital cellulitis 5. Treatment a. Nasolacrimal massage 2-3 x per day b. Topical antibiotics c. 96% resolve by age 1 of year d. If not improved by 1 year the nasolacrimal duct may be probed with topical anaesthesia

Answer 39

Dry eye 1. Key points a. Reduced or absent tear secretion 2. Aetiology a. Syndromes i. Familial dysautonomia ii. Anhidrotic ectodermal dysplasia iii. Triple A syndrome (achalasia (denervation of oesophage), Addisons, alacrima) b. Aplasia of CN and lacrimal aplasia/ hypoplasia 3. Clinical manifestations a. Asymptomatic b. Dry eye c. Photophobia d. FB sensation e. Reduced vision 4. Treatment a. Aggressive lubrication

Answer 40

Wet/watery eye, excessive watering of the eye ``` DDx • Acute o Corneal FB – eye often erythematous, acute o Corneal abrasion o Subtarsal foreign body • Chronic o Nasolacrimal duct obstruction o Allergic conjunctivitis o Glaucoma ```

Answer 41

1. Bacterial conjunctivitis a. Characterised by conjunctival hyperaemia, edema, mucopurulent exudate, glued eyes, pain and discomfort b. Usually bacterial infection c. More common in young children (<5 years) d. Most frequently caused by H influenza (60-80%), pneumococci (20%) and staphylococci (5-10%) e. Treatment with topical antibiotics 2. Viral conjunctivitis a. Characterised by watery discharge, follicular changes (aggregates of lymphocytes) in the palpabebral conjunctiva b. May be unilateral c. Often associated with periauricular nodes d. More common in older children e. Adenovirus most common 3. Epidemic keratoconjunctivitis a. Due to adenovirus type 8 b. Presents as FB sensation, itching, burning c. Subsequently edema and photophobia develop with large oval follicles in the conjunctiva 4. Allergic conjunctivitis a. Characterised by intense itching, clear watery discharge, conjunctival edema (chemosis) b. Seen in Spring 5. Vernal conjunctivitis a. Usually begins in pre-pubertal years b. Large, flattened cobble stone like papillary lesions of the palpebral conjunctivae are characteristic c. Stringy exudate and a milky conjunctival pseudomembrane are common 6. Parinaud oculoglandular syndrome a. Form of cat-scratch disease caused by Bartonella hensalae b. Bacteria can deposit in the conjunctiva after rubbing eye after handling cat c. Lymphadenopathy and conjunctivitis are hallmarks of the disease

Answer 42

1. Megalocornea a. Non-progressive enlarged cornea b. Myopia frequently present c. Complication is the development of opacities in adult life d. All modes of inheritance described e. May be associated with Marfan syndrome, craniosynostosis and Alport syndrome 2. Microcornea a. Small cornea in an otherwise normal eye b. Associated defects – coloboma, microphakia, congenital cataract, glaucoma, aniridia

Answer 43

a. Infection with HSV – tree-like ulceration b. Accompanied by pain, photophobia, tearing, blapherospasm, and conjunctival injection c. Requires aggressive treatment with systemic antivirals d. Topical corticosteroids may exacerbate superficial herpetic disease resulting in corneal perforation

Answer 44

a. Result in focal or diffuse corneal haze, hyperaemia, lid edema, pain, photophobia, tearing, and blepharospasm b. Hypopyon (pus in anterior chamber) common c. Usually occur in contact lens wearers d. Serious infections – Pseudomonas, Neisseria e. Aggressive treatment required f. Unexplained corneal ulcers in children should raise concerns of sensory defect – Riley-Day, Goldenhar-Gorlin syndrome, or metabolic disorder such as tyorisnaemia

Answer 45

a. Cystinosis = polychromatic crystals deposited through the cornea b. MPS = corneal deposits c. GM gangliosidoses = corneal deposits d. Kayser-Fleischer ring = Wilson disease

Answer 46

1. Key points a. Cataract = lens opacity b. 60% are isolated, 20% part of a syndrome c. More common in LBW infants 3. Disorders associated with cataracts a. Craniofacial syndromes = Smith-Lemli-Opitz, inherited craniosynostosis syndromes, Pierre-Robin b. Metabolic = galactosaemia, mannosidosis, refsum, Zellweger c. Skeletal = Stickler syndrome, chondrodysplasia punctate d. CNS disorders e. Intrauterine infection = toxo, rubella, CMV, HSV, syphilis f. Dermatological = ectodermal dysplasia, incontentia pigmenti, Rothmund-Thomson syndrome, congenital icthyosis, Cockayne syndrome g. Renal = Lowe syndrome, Alport syndrome h. Muscular disorders = myotonic dystrophy, walker-warburg syndrome i. Apical = Rubenstein-Taybi, Bardet-Biedl, oculodentodigital dysplasia j. Chromosomal abnormalities = T21, T1, T13, Wolf-Hirschhorn syndrome (4p syndrome), Turner, Cri du chat syndrome (5p syndrome) k. Miscellaneous = aniridia, Stickler, ROP, NF type 2

Answer 47

Lens displacement 1. Key points a. Abnormality in the suspensory system of the lens may result in displacement b. Results in blurred vision, diplopia 2. Aetiology a. Trauma b. Ocular disease – uveitis, tumour, congenital glaucoma, high myopia, megalocornea, aniridia c. Systemic diseases i. Marfan syndrome – occurs in 80%, evident in 50% by 5 years of age 1. Usually superior and temporal 2. Usually bilateral ii. Homocystinuria – often evident by 5 years of life 1. Inferiorly and nasally displaced 3. Treatment a. Surgical repair

Answer 48

• Anterior lenticonus = rare bilateral condition in which the anterior capsule of the lens thins, allowing the lens to bulge forward centrally o Prominent feature of Alport syndrome*** (pathognomonic - anterior) • Posterior lenticonus = bulge of the posterior lens capsule and lens o More common than anterior

Answer 49

1. Key points a. Uveal tract = iris, ciliary body + choroid b. Uveitis = inflammation of any component of the uvea c. Most important cause in children is JIA*** 2. Aetiology - Anterior: rheum/autoimmune/systemic d. Beware the PANAFY = pauciarticular ANA positive female young -> more regular screening for JIA patients who are ANA positive - Posterior: infection, TINU - some overlap between causes 3. Clinical manifestations a. Anterior uveitis (pain+red, no visual change) i. Pain and redness ii. Erythema primarily at the limbus iii. Constricted pupil + pain iv. Variable visual loss b. Posterior + intermediate uveitis (no pain/red, changed vision) i. Usually painless ii. Non-specific visual changes such as floaters and/or reduced visual acuity iii. Erythema is NOT a prominent feature 4. Complications a. Band keratopathy = deposition of calcium in the epithelium of the cornea b. Posterior synechiae = adhesion of the iris to the lens which lies posterior to it c. Cataract = resulting from inflammation in some patients or glucocorticoid treatment in others d. Intraocular hypertension + glaucoma 5. Investigations a. Slit lamp examination diagnostic 6. Treatment a. Treatment dependent on underlying cause b. Immunosuppression for JIA

Answer 50

1. Key points a. Spectrum of manifestations b. Caused by persistence of various portions of the fetal hyaloid vascular system + associated fibrovascular tissue c. Usually unilateral d. May occur in infants with no other abnormalities or history of prematurity 2. Pathogenesis a. During eye development the hyaloid artery extends from the optic disc to the posterior aspect of the lens – sends branches into the vitreous and ramifies to form the posterior portion of thee vascular capsule of the lens b. Posterior portion of the hyaloid system normally regresses by the 7th fetal month and the anterior portion by the 9th fetal month c. Remnants = PFV 3. Clinical manifestations a. Presence of vascularised plaque of tissue on the back surface of the lens b. Eye is microphthalmic or slightly smaller than usual c. Anterior PFV = leukocoria, strabismus, nystagmus i. Usually progressive ii. Poor outcome iii. Complications = intra-ocular haemorrhage, swelling of the lens caused by rupture of the posterior capsule, and glaucoma 4. Treatment a. Surgery

Answer 51

MRCPCH book: a pigmentary retinopathy characterised by night blindeness (earliest), progressive loss of peripheral visual field and loss of central vision (final). Symptoms usually do not develop until 2nd-3rd decade. Early retinal changes show pigment deposition as seen in midperipheral retina. Systemic associations - Usher syndrome = most common, deafness then vision - Refsum's disease (peroxisomal disorder) - Abetalipoproteinaemia (fat malabsorption -> steatorrhoea, ataxia) 1. Key points a. Group of inherited dystrophies b. Characterised by progressive degeneration and dysfunction of the retina - primarily affect the photoreceptor and pigment epithelial function c. May occur alone or as part of a syndrome d. Family history in 70% 3. Clinical manifestations a. Age of onset i. Varies, childhood to adulthood ii. Photoreceptor degeneration can be detected many years before become aware of visual problems b. Night blindness earliest symptom i. Very gradual, can go unnoticed c. Loss of visual field i. Starts in midperiphery, progresses more peripherally ii. Results in constricted visual field d. Ophthalmoscopy i. Optic disc pallor, attenuated vessels, pigment deposits in bone-spicule pattern ii. Macula affected in advanced disease 4. Investigations a. Dark adaptometry: Pt exposed to bright light stimulus, then put in the dark for 30 minutes, minimum intensity light that can be detected by pt measured at interval time points. b. Full-field and multifocal electroretinography (ERG): Electrode placed on cornea or skin of eyelid, and colour and intensity of a light stimulus are manipulated to activate cones or rods.

Answer 52

• Cherry red spot = bright to dull red spot at the center of the macula surrounded and accentuated by grayish-white or yellowish halo o Halo forms due to loss of transparency of the retinal ganglion cell layer secondary to edema or lipid accumulation o As ganglion cells are not present in the fovea the retina surrounding the fovea is opacified by the fovea transmits the normal underlying choroidal colour (red) ``` • Conditions o Sphingolipidoses***  Tay-Sachs – GM2 type 1  Sandhoff variant – GM2 type 2  Generalised gangliosidoses – GM1 type 1 o Similar but less distinctive  Metachromatic leukodystrophy  Niemann-Pick disease  Galactosialidosis  Mucolipidoses ``` • NOTE: cherry red spot that characteristically occurs as a result of retinal ischaemia secondary to vasospasm, ocular contusion or occlusion of central retinal artery must be differentiated

Answer 53

• Herald lesions of hamartomatous disorders • Tuberous sclerosis = refractile, yellowish, multinodular cystic lesion arising from the disc or retina o Benign astrocytic proliferations • NF = rarely have similar lesions • VHL = haemangioblastoma • Sturge-Weber = choroidal haemangioma

Answer 54

Boys, 8-10 years Unilateral Peripheral retinal telangiectasias and aneurysmal dilatation lead to extensive areas of exudates giving the retina a yellow-white appearance, which may produce leukocoria. 2. Clinical manifestations a. Blurred vision b. Leukocoria c. Strabismus 3. Complications a. Rubeosis of the iris b. Glaucoma c. Cataract 4. Treatment a. Photocoagulation b. Laser

Answer 55

1. Key points a. Prevalence and course relates to child’s age b. Detectable microvascular changes are rare in pre-pubertal children c. Prevalence of retinopathy increases after puberty – especially >14 years 2. Classification a. Non-proliferative i. Retinal micro-aneurysms ii. Venous dilation iii. Retinal hemorrhages = dot blot iv. Exudates b. Proliferative = more serious i. Neovascularisation + proliferation of fibrovascular tissue on the retina ii. Traction results in haemorrhage + scarring iii. Vision-threatening due to risk of haemorrhage, cicatrization, traction + retinal detachment iv. Neovascularisation of the iris may lead to secondary glaucoma 3. Treatment a. Laser photocoagulation b. VEGF inhibitors

Answer 56

1. Optic nerve aplasia a. Rare congenital anomaly b. Typically unilateral c. Optic nerve, retinal ganglion cells + retinal blood vessels absent d. Occurs in otherwise healthy individual 2. Optic nerve hypoplasia a. Non-progressive condition characterised by a subnormal number of optic nerve axons b. Nerve head typically appears small and pale, with a pale or pigmented peripapillary halo or double-ring sign c. Main feature of septo-optic dysplasia of de Morsier – disorder associated with midline anomalies d. Children with periventricular leukomalacia also develop an unusual form of optic nerve hypoplasia

Answer 57

a. Swelling of the nerve head secondary to raised ICP b. Features = oedematous blurring of the disc margins, fullness or elevation of the nerve head, partial or complete obliteration of the disc cup, capillary congestion and hyperaemia of the nerve head, generalised engorgement of the veins, loss of spontaneous venous pulsation, nerve fibre layer hemorrhages, peripapillary exudates

Answer 58

b. Inflammation or demyelination of the optic nerve with reduced function c. Characterised by pain with eye movement d. Results in decreased visual acuity, decreased colour vision + RAPD e. Optic disc may or may not appear normal – if appear normal termed ‘retrobulbar optic neuritis’, if abnormal ten ‘ papillitis’ or ‘intraocular optic neuritis’

Answer 59

UTD: Glaucoma is a heterogeneous group of eye diseases that are characterized by a progressive optic neuropathy, manifested by cupping of the optic disc and usually, but not always, associated with increased intraocular pressure (IOP). 1. Key points a. Glaucoma = damage to the optic nerve with visual field loss that is caused by elevated pressure within the eye b. Infantile/congenital = <3 years c. Juvenile = 3 to 30 years 2. Aetiology a. Primary (>50%) = isolated anomaly of the drainage apparatus of the eye (trabecular meshwork) b. Secondary = other ocular or systemic abnormalities are present i. Traumatic ii. Intra-ocular neoplasm iii. Uveitis iv. Lens-induced v. Surgery vi. Steroid induced vii. Rubeosis viii. Angle-closure glaucoma ix. Increased venous pressure x. Maternal rubella xi. Intraocular infection 3. Clinical manifestations a. Classic triad i. Epiphoria (tearing/watery) (differentiated from nasolacrimal duct obstruction by the presence of rhinorrhoea - absent with duct obstruction) ii. Photophobia (sensitivity to light) iii. Blepharospasm (eyelid squeezing) b. Other features i. Corneal edema = results in cloudiness/ haziness ii. Corneal and ocular enlargement iii. Conjunctival injection iv. Corneal enlargement (buphthalmos = ox eye) c. Examination i. Cupping of the optic nerve 4. Treatment a. Medical i. Beta blocker ii. Alpha agonist iii. Carbonic anhydrase inhibitors (acetazolamide) b. Surgical 5. Prognosis a. Blindness if untreated

Answer 60

* Inflammation of the lacrimal gland * Uncommon in childhood • Acute o May occur with mumps or EBV o Staphylococcus aureus results in suppurative dacryoadenitis • Chronic o Associated with sarcoidosis, TB, syphilis

Answer 61

* Infection of the lacrimal sac * Generally requires obstruction of the nasolacrimal system to allow its development * Acute dacryocystitis presents with redness + swelling overlying the lacrimal sac * Treated with warm compression + IV antibiotics * If occurs in newborns require systemic antibiotics and pressure to relieve obstruction – sometimes require probing to open the duct

Answer 62

1. Classification a. Periorbital = inflammation of the eyelids and periorbital tissue without signs of true orbital involvement b. Orbital = inflammation of the tissue of the orbit (fat and ocular muscles), with proptosis, limitation of eye movement, edema of the conjunctiva, swelling of eyelid +/- reduced visual acuity 2. Pathogenesis a. Orbital cellulitis may result from i. Direct infection from a wound ii. Bacteraemia iii. Direct extension from contiguous site – particularly paranasal (ethmoid) sinuses 3. Aetiology a. Hib – previously most common b. Streptococci - more likely if contiguous skin lesion c. S. aureus - more likely if contiguous skin lesion d. Pneumococcus e. Rarely – fungi and mycobacteria particularly if immunocompromised i. Mucorales – mucormycosis ii. Aspergillus

Answer 63

4. Clinical manifestations a. Periorbital i. Common in young children ii. Eyelid swelling + edema – difficult to assess the globe iii. Generally mild, normal vision/pupil reactions b. Orbital i. Mean age 7 years ii. Systemic symptoms – fever toxicity, leukocytosis iii. Specific features or orbital cellulitis 1. Pain with eye movements 2. Proptosis 3. Ophthalmoplegia with diplopia 4. Chemosis (may occasionally occur in severe periorbital cellulitis) Impaired pupil reflexes (RAPD) 5. Investigations a. CT scan if suspicion of orbital cellulitis

Answer 64

6. Treatment a. IV ceftriaxone + flucloxacillin b. LP contraindicated until post imaging 7. Complications (orbital) a. Subperiosteal abscesses b. Orbital abscesses c. Extra-orbital extension i. Meningitis ii. Epidural or subdural empyema iii. Brain abscesses iv. Cavernous sinus thrombosis d. Vision loss i. Optic neuritis ii. Ischaemia resulting form thrombophlebitis/ compression of orbital veins iii. Pressure resulting in central retinal artery occlusion e. Other = optic atrophy, exposure keratitis, retinal or choroidal ischaemia

Answer 65

1. Key points a. Serious eye injuries can be under-appreciated when children present with a painful eye or blurred vision. b. The following traumatic conditions threaten vision: i. Ruptured globe ii. FB- either intraocular or deep corneal iii. Large hyphaemas (intra-ocular bleeding -> causing acute glaucoma) iv. Retinal detachment v. Corneal burns, either chemical or thermal- alkalis penetrate deeper and have greater potential for serious and delayed burns. vi. Contact lens-related corneal infections (bacterial keratitis) 1. NOTE: a. Relative afferent pupillary defect in the eye suggests presence of i. Traumatic optic neuropathy ii. Vitreous haemorrhage iii. Retinal detachment iv. Intracranial injury b. Traumatic optic neuropathy may result from: i. Compressive optic neuropathy ii. Optic nerve sheath haematoma iii. Optic nerve head avulsion iv. Optic nerve laceration

Answer 66

1. Key points a. Penetrating, perforating or blunt injury b. One of the most life-threatening injuries that can be sustained c. Ophthalmological emergency 2. Clinical manifestations a. Severe loss of vision b. Squashed or distorted appearance to globe c. Ocular contents extruding from globe d. Distorted or peaked pupil e. Loss of red reflex f. Relative afferent pupil defect g. Loss of ocular motility h. Shallow anterior chamber i. Chemosis - bulging of the conjunctiva 3. Treatment a. Do not force eyelids open, remove protruding body b. Surgery c. Caution with opioids – aim to avoid vomiting as increases ICP d. Imaging 4. Complications a. Vision loss = from corneal scarring, loss of intraocular contents, or infection

Answer 67

* If very large or deep defect seen, presume full thickness * Exclude foreign body, including under eyelid * Opthal referral if large, deep or central corneal foreign body * Remove under direct vision of slit lamp * Chloramphenicol ointment/ drops * Pad the eye for four hours (to prevent accidental further eye injury due to anaesthetic effect) * Cycloplegic eye drops (cyclopentolate 1% or homatropine 2%) can be used for relief of a very painful eye * Patients need daily review until corneal ulceration healed

Answer 68

1. Key points a. Ophthalmological emergency b. Alkali burns = more destructive than acid burns as they react with fats to form soaps, which damage cell membranes and allow further penetration c. Acid burns = more localised tissue damage – precipitate proteins creating physical barrier against further penetration 2. Treatment a. Copious irrigation with water or saline – minimum 3 L N saline until pH normal (6-8) b. Particulate matter – require GA

Answer 69

1. Key points a. Any bone of the orbit may fracture in a traumatic incident b. Superior and lateral wall fractures are LEAST common c. Orbital floor followed by medial wall most common – referred to as a blowout fracture i. Fracture may act as a trapdoor entrapping orbital contents 2. Clinical manifestations a. Periorbital trauma + pain b. Diplopia, eyelid swelling, eye movement restriction, hypesthesia c. May have associated nausea + bradycardia if inferior rectus is entrapped 3. Investigations a. CT scan 4. Treatment = surgery

Answer 70

1. Key points a. Traumatic hyphema = blood in the anterior chamber b. Common complication of blunt or penetrating injury to the eye and can result in permanent vision loss. 2. Clinical manifestations a. Bright red or dark fluid level between the cornea and iris b. Photophobia c. Decreased visual acuity d. Anisocoria (unequal pupils) and poor pupil reactivity e. Elevated intraocular pressure f. Damage to nearby structures (open globe, corneal abrasion, iritis, lens subluxation, optic neuropathy etc) 3. Treatment a. Initial management aiming to avoid secondary rebleed and/or increased intraocular pressure i. Eye shield ii. Bed rest and dim lighting iii. Elevate head of the bed (promotes inferior settling of blood ) iv. Control pain b. Avoid NSAIDs 4. Complications a. Spontaneous rebleed is most common acute complication (within the first few days) i. Occurs in 0-38%. b. Increased intraocular pressure c. Optic atrophy d. Corneal blood staining e. Long-term = increased incidence of glaucoma

Answer 71

* Retinal hemorrhages | * Retinal fold – specific for severe crush injury or shake injury

Answer 72

1. Key points a. Primary teeth form in dental crypts b. By 12 weeks of fetal life bud like enlargements are present c. SUMMARY i. Primary/deciduous dentition = 6 months to 6 years ii. Mixed dentition = 6 years to 12 years iii. Permanent dentition = 12 years and over 2. Primary dentition (front teeth until 3rd molar) a. Starts in utero, completed by 6 months b. FIRST i. Central and lateral incisors at 6-9months ii. Lower central- lower lateral – upper central – upper lateral c. NEXT i. Canines, molars at 1-3years ii. Canine – first molar – second molar iii. Upper and lower occur at the same time 3. Permanent teeth (posterior molars and second lot front teeth) a. Often develop in utero 3-4months, complete by 3 years b. Eruption from 6-18yrs c. FIRST i. Central incisors – lateral incisors – 1st molar at 6-9years ii. Mandible slightly before maxilla d. THEN i. Canine, first and second premolars 6-9years ii. 2nd molar at 12 years, 3rd molar at 18years

Answer 73

a. Absence of tooth | b. Teeth most commonly affected – 3rd molars, maxillary lateral incisors, mandibular 2nd premolars

Answer 74

a. May be normal or occur with cleidocranial dysplasia

Answer 75

a. Hypopituitarism b. Hypothyroidism c. Rickets d. Gaucher disease (glucocerebrosidase deficiency results in accumulation of cerebroside in the visceral organs +/- the brain) e. Down’s syndrome f. Cleidocranial dysplasia

Answer 76

a. Present from birth or erupt very soon after b. Usually lower anterior region c. Issues – can be mobile, aspiration risk d. Managed with removal e. Can result in inflammatory ulceration i. Sublingual ulceration ii. Common in children with CP iii. Important to exclude congenital sensory neuropathies – Riley Day, Lesch Nyan

Answer 77

Connecting upper lip to upper gum a. Maxillary frenulum b. Speech – no conclusive evidence c. Feeding – no conclusive evidence d. Increased risk of decay

Answer 78

Tongue-tie a. Prevalence 2-5% b. Increased frequency in some syndromes c. Speech - no evidence to support management d. Indications for management i. FTT ii. Unable to latch

Answer 79

Enamel and dentine injuries - see local dentist in a few weeks Exposed pulp (pink, painful) - if primary tooth, call dental reg ?extraction - if permanent tooth, keep any fragments (milk) and call reg ?reattachement Tooth concussion = tender but firm - outpatient dental Subluxation = tender and loose - call dental reg Lateral luxation or extrusion - primary = not repositioned or extracted - permanent = reposition and splint Intrusion - call reg Avulsion - primary - no reimplantation - permanent = milk, replace into socket and bite on gauze

Answer 80

a. Infectious disease of bacterial origin – relationship between tooth surface, dietary CHO, oral bacteria i. Particularly streptococci b. Most common chronic disease in childhood in Australia c. >50% of Australian 6-year-old children have caries – association with low socioeconomic status d. Clinical manifestations i. Initial appearance – opaque white spot ii. Small lesions may be difficult to diagnose – large areas evident by darkened or cavitated lesions on tooth surface iii. Childhood caries – early colonisation of child with cariogenic bacteria and frequent ingestion of sugar, either in the bottle or in solid food e. Risk factors i. Bad bacteria ii. Absence of saliva iii. Poor dietary habits f. Protective factors i. Saliva ii. Antibacterials iii. Fluoride iv. Effective diet g. Complications i. Destroy the tooth and invade the dental pulp – resulting in inflammation (pulpitis) and significant pain ii. Pulpitis can progress to pulp necrosis – ultimately resulting in bacterial invasion + dental abscess formation h. Treatment i. Oral hygiene ii. Diet – avoidance of bottle drinking + fruit juice iii. Dentist visits iv. Fluoride water + toothpaste – prevention v. If infection – penicillin treatment of choice

Answer 81

i. Granulomatous disorder of the face and oral cavity – presents as chronic, non-caseating granular mucogingivitis involving the peri-oral tissues and the oral mucosa ii. OFG may be part of Crohn’s disease or may exist alone

Answer 82

i. Secondary to phenytoin + cyclosporine

Answer 83

a. Soft palate not working as valve - Hypernasal speech and difficulty feeding (food out the nose in infant) b. VCF velo-cardio-facial syndrome – screen for cardiac anomalies, learning/behavioral issues, typical facies c. Needs speech path, DO NOT remove adenoids

Answer 84

a. Look for midline associations – UDT, hypospadias, umbilical hernia, choanal atresia = growth hormone deficiency

Answer 85

a. Peutz-Jeghers syndrome | b. Addison’s disease

Answer 86

1. Key points a. Clefts of the lip and palate are distinct entities closely related embryologically, functionally and genetically b. Cleft lip = due to hypoplasia of the mesenchymal layer, resulting in failure of the medial nasal and maxillary processes to joint c. Clef palate = failure of the palatal shelves to approximate or fuse 2. Epidemiology a. Cleft lip +/- cleft palate = 1/750 b. Cleft palate = 1/2,5000 births 3. Risk factors a. Gender = cleft lip more common in males b. Maternal drug exposure c. Syndrome-malformation complex d. Genetic factors 5. Treatment a. Feeding support i. Soft artificial nipples with large openings, squeezable bottle + support b. Surgical closure i. Usually done around 3 months ii. Done <1 year to ensure normal speech development 6. Sequelae a. Recurrent otitis media + hearing loss b. Malpositioned teeth requiring correction c. Misarticulations and velopharyngeal dysfunction

Answer 87

E.g. secondary to cleft lip and palate a. Velopharyngeal dysfunction = inability to form an effective seal between the oropharynx and nasopharynx during swallowing or phonation b. Clinical manifestations i. Hypernasal speech ii. Conspicuous constrictive movement of the nares during speech iii. Inability to whistle, gargle, blow out a candle, or inflate a balloon iv. Loss of liquid through the nose when drinking with head done v. Otitis media vi. Hearing loss

Answer 88

a. Bilateral submaxillary enlargement i. AIDS ii. CF iii. EBV iv. Malnutrition b. Parotid enlargement i. Chronic vomiting c. Benign salivary gland hypertrophy i. Endocrinopathies – thyroid disease, diabetes, Cushings d. Infiltrative + tumours uncommon

Answer 89

a. Acute i. Usually caused by blockage and superinfection ii. Due to salivary stone or mucous iii. Stones can be removed by physical manipulation, surgery or lithotripsy b. Recurrent parotitis i. Can occur in otherwise healthy children ii. Usually lasts 2-3 weeks iii. Treatment = local heat, massaging the gland, and antibiotics c. Suppurative parotitis i. Caused by Staphylococcus aureus ii. Usually unilateral, may be accompanied by fever