Blue 3-3 Flashcards
(36 cards)
What is the most important goal in managing a child with retinoblastoma?
Survival of the patient.
What is the second most important goal in retinoblastoma management?
Preservation of the globe.
What comes after patient safety and globe preservation in retinoblastoma care?
Focus on visual acuity.
When is enucleation used in unilateral retinoblastoma?
If the eye is classified as Reese–Ellsworth group V.
When is chemoreduction used in bilateral retinoblastoma?
In most cases, unless one eye has advanced disease.
What is the eye preservation rate for group A retinoblastoma using selective ophthalmic arterial injection?
1
What focal therapies are used for retinoblastoma?
Laser photocoagulation, thermotherapy, cryotherapy, plaque radiotherapy.
When is plaque radiotherapy used in retinoblastoma?
For tumours that fail other focal therapies, up to 8–10 mm thick.
What is the most common soft-tissue sarcoma in childhood?
Rhabdomyosarcoma (RMS).
Which RMS subtype is most common in the orbit?
Embryonal.
Which RMS subtype has worse prognosis?
Alveolar.
Which quadrant is most often affected in orbital RMS?
Superior nasal quadrant.
What are common signs of orbital RMS?
Non-axial unilateral exophthalmos, rapid ptosis, mimic orbital cellulitis.
What is a hallmark of Kearns-Sayre syndrome?
Chronic progressive external ophthalmoplegia before age 20 and pigmentary retinopathy.
What are systemic features of Kearns-Sayre syndrome?
Cardiac conduction defects, high CSF protein, cerebellar syndrome.
What is the hallmark sign of Brown syndrome?
Limited elevation in adduction.
What causes acquired Brown syndrome?
Often trauma, surgery or inflammation.
What is often heard or felt in acquired Brown syndrome?
Audible/palpable nasal click with up and nasal gaze.
What are hallmark skin signs in NF1?
Café au lait spots and neurofibromas.
What is the most common ocular finding in NF1?
Lisch nodules.
What eye findings may be seen in NF1?
Choroidal hamartomas in the posterior pole.
What is the classic visual field defect in LHON?
Centrocaecal scotoma.
Are pupillary reflexes preserved in LHON?
Yes.
What are common fundus findings in LHON?
Vascular tortuosity, telangiectasia, RNFL swelling.
