Case studies |
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Case study one JACK , aged 11 months , is brought to his GP as his mum has noticed a sudden onset of clouding of his right cornea ( see figure A ) He is an otherwise well child who was born at term without complication .
Jack is the third child of healthy parents with no family history of eye disease . His mother said the only significant issue with his eyes was that strangers would often comment how big and beautiful they were .
He is referred and seen by an ophthalmologist the same day . Jack is very photophobic and both corneae are hazy , with the right cornea being significantly more affected . The horizontal corneal diameters are significantly increased at 12-13mm in each eye , the intraocular pressures measures over 30mmHg and a hand-held slit lamp examination is positive for Haab ’ s striae ( splits in Descemet ’ s membrane ) in both eyes .
Jack proceeds to an examination under anaesthesia , where the diagnosis of primary congenital glaucoma is confirmed . He undergoes right- then left-angle surgery without complication . This results in an immediate improvement in his corneal clarity , but only a temporary improvement in his intraocular pressures .
He undergoes another angle procedure in each eye a few weeks
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A Figure A . Aged 11 months , with bilaterally enlarged corneae and corneal clouding , right eye affected more significantly than the left . Figure B . Aged three years , with clear corneae following management for primary congenital glaucoma .
later , but despite this , several months later , his intraocular pressures are supraoptimal . Jack then undergoes right and then left glaucoma tube implants .
Now aged three years ( figure B ), with patent tube implants and twice daily ocular antihypertensive eyedrops , Jack has clear corneas , excellent control of his intraocular pressures ( R = 12mmHg , L = 11mmHg ) and excellent central vision ( R = L = 6 / 12 ) despite some mild myopia . He continues to have his eyes checked every three months .
B Case study two Annie , aged five months , has a large angle , constant esotropia since birth . She was born at term and is otherwise well . She is the third child of healthy parents and there was no significant ophthalmic history in the family . Annie is seen by her GP , who , concerned about congenital esotropia , refers her to an ophthalmologist .
The ophthalmologist notes symmetrical vision and a large-angle constant esotropia with alternating fixation and cross-fixation .
Annie ’ s eyes move fully without
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any abduction deficit . The internal ocular examination is normal , with healthy discs , maculae and low hypermetropia ( R = L =+ 2.0DS ). She is diagnosed with a congenital esotropia without amblyopia . Her management is discussed in principle , and a review is planned in three months .
At the three-month review , the examination is stable , so surgery is scheduled for when Annie is 12 months old .
Given the large size of the deviation , she is given a 30 % chance of needing more than one operation to fully treat the angle of the squint .
On the day of surgery , Annie is found to have a 50 prism dioptre esotropia . She undergoes right and left-eye medial rectus recessions ( weakening surgery ) of 6mm .
Annie has an uneventful recovery , but three months after the surgery is found to have a persisting esotropia of 30 prism dioptres .
At the age of 20 months , she has her second operation , comprising right and left lateral rectus resections ( strengthening surgery ) of 5.5mm .
A month after the second surgery , Annie has excellent ocular alignment and has recovered well . She retains symmetrical vision , and remains under regular review to monitor her visual development and ocular alignment .
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THE challenges in assessing and diagnosing paediatric eye disease within the first year of life are significant . These challenges are manageable when one incorporates the following into practice : an understanding of normal visual developmental milestones ; the ‘ red flag ’ signs of dangerous eye disease ; information on the common and serious conditions presenting in the first year of life ; and an appreciation that it is always appropriate to refer anything you are concerned about to an ophthalmologist .
Disclaimer
Consent to publish the photographs has been obtained from the parents of the children shown in this How to Treat .
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1 . Which THREE are ‘ red flags ’ on history and examination of the paediatric eye ? a ) Corneal clouding . b ) A birthmark involving the nasion or glabella . c ) Asymmetry of the red reflex . d ) Ocular misalignment .
2 . Which THREE are the most significant risk factors for the development of retinopathy of prematurity ? a ) Low birth weight b ) Caesarean section c ) Low gestation d ) Supplemental oxygen
3 . Which THREE statements regarding congenital cataract are correct ? a ) Unilateral cataract is usually associated with systemic disease . b ) Any abnormality of the red reflex or any whiteness or dullness within the pupil can be a sign of cataract . c ) Roving eye movements and nystagmus are associated with a poorer visual outcome . d ) Visually significant congenital cataracts are managed surgically .
4 . Which TWO statements regarding
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congenital glaucoma are correct ? a ) Glaucoma refers to any condition in which elevated intraocular pressure causes a progressive optic neuropathy . b ) Congenital glaucoma is a bilateral condition with symmetrical signs . c ) Clinical signs include abnormality of the red reflex , leukocoria or dullness within the pupil . d ) The management of congenital glaucoma is surgical , including concurrent management of any myopia and amblyopia .
5 . Which THREE modalities are indicated for the management of congenital nasolacrimal duct obstruction ? a ) Topical antibiotics if there is associated infection of the conjunctiva . b ) Massage of the lacrimal sac . c ) Probing of the nasolacrimal duct . d ) Intubation and permanent stenting with silicon tubing .
6 . Which THREE organisms can commonly cause ophthalmia neonatorum ? a ) Herpes simplex virus b ) Staphylococcus aureas c ) Gonococcus d ) Chlamydia
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7 . Which THREE statements regarding strabismus in the infant are correct ? a ) Variable , intermittent strabismus is very common in newborns up until the age of 16 weeks . b ) Pseudo-esotropia is an uncommon differential diagnosis of a true esotropia in early childhood . c ) The alternation of fixation in congenital esotropia may protect the infant from developing amblyopia . d ) Congenital trochlear nerve palsy may result in a head tilt or head turn away from the affected side .
8 . Which TWO statements regarding retinoblastoma in infants are correct ? a ) Retinoblastoma is the most common malignant ocular tumour of childhood . b ) There have not been other malignancies described that are associated with the hereditary germline RB1 mutation . c ) The most common presenting sign of retinoblastoma is leukocoria or photoleukocoria . d ) Hereditary cases tend to be unilateral and tend to present after the first year of life , but
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usually before the age of 3-5 years .
9 . Which THREE statements regarding birthmarks and lid lesions are correct ? a ) Port-wine stains involving the upper eyelid increase the risk of secondary glaucoma . b ) Capillary haemangiomas may require treatment with topical or systemic propranolol . c ) Chalazions are the most common eyelid conditions in children under one year of age . d ) Orbital dermoid cysts are at risk of rupture when the child becomes mobile .
10 . Which TWO statements regarding the blind baby are correct ? a ) Babies with very poor vision from birth show no visual fixation . b ) Roving eye movements and nystagmus are more likely to develop at the age of 2-3 months . c ) Children with poor vision and those with delayed vision will catch up on their visual milestones , but it can take up to 18 months to do so . d ) Diagnosis of cortical visual impairment relies on specialist investigation and collaboration with a paediatrician .
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CPD POINTS |