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 |