Ophthalmology in Craniosynostosis Flashcards
What are the main roles of ophthalmology in craniosynostosis?
Diagnose raised ICP
Treat amblyopia
Manage ocular complications (exposure keratosis etc)
What % of patients with syndromic craniosynostosis have reduced vision?
40-60%
Why do craniosynostosis patients develop optic neuropathy?
Chronically raised ICP (most commonly in Crouzon and Apert)
Nerve elongation / compression
What is the most common cause of pseudopapilloedema?
Optic disc drusen (slow accumulation of cytoplasmic material buried within the retinal fibre layers)
What layer is swollen in papilloedema?
The peripapillary nerve fibre layer
What is the sensitivity of papilloedema in syndromic craniosynostosis?
20-40% - so the absence of papilloedema does not rule out raised ICP
What factors contribute to raised ICP in syndromic craniosynostosis?
OSA
Midface hypoplasia
Venous sinus stenosis
Hydrocephalus
Craniocerebral disproportion
What type of strabismus (squint) is seen in craniosynostosis?
Excyclotorsional syndrome - Vertico-torsional with a variable horizontal component due to abnormal position of the trochlear of the superior oblique muscle within the orbit.
What are the V-pattern seen in excyclotorsional syndrome?
The eyes are further apart in up-gaze compared to down-gaze
What are the features of exposure keratopathy?
Redness, pain and photophobia - occurs with exorbitism
How can exposure keratopathy be prevented?
Lubricants if the eye does not close completely at night with Vitamin A ointment
Lateral tarsorrhaphy
How can amblyopia be prevented in young patients?
Patching of the dominant eye
What are the ophthalmological findings in Crouzon / Apert syndrome?
Exorbitism
Hypertelorism
Exotropia
Excyclotorsional syndrome
Corneal exposure keratopathy
Papilloedema / Optic neuropathy
Down slanting palpebral fissures & congenital glaucoma (Apert only)
What are the ophthalmological findings in Saethre-Chotzen syndrome?
Ptosis
Hypertelorism
Vertical strabismus
Optic neuropathy
How frequent should ophthalmology assessments be perforemd in syndromic synostosis?
Twice yearly in syndromic
(Yearly in non-syndromic)
