INTRAOCULAR TUMOURS Children

INTRAOCULAR TUMOURS Children

Retinoblastoma, the most common ocular malignancy of childhood, is a malignant tumour of the retina that can be bilateral in around one-third of cases. Half of cases are heredi tary (autosomal dominant) and are due to mutation of the gene on chromosome 13; children with a family history should be carefully monitored from birth. Remaining cases occur sporadically . Inherited retinoblastoma is more likely to be bilateral. Retinoblastoma is often not spotted until the tumour fills the globe and presents as a white reflex in the pupil or as a squint ( Figure 49.13 ). The di ff erential diagnosis includes reti - nopathy of prematurity , persistent fetal vasculature (PFV) and - intraocular infections. If the tumour is large, enucleation may be required, but radiotherapy , cryotherap y , chemotherapy or laser treatment can cure small lesions. Liaison with a paediatric oncologist is essential. Summary box 49.2 Intraocular tumours /uni25CF /uni25CF - RB1

Figure 49.12 Capillary haemangioma in a child. An orbital venogram demonstrates displacement of the second part of the superior oph thalmic vein (arrow) (courtesy of Dr Glyn Lloyd). Figure 49.13 Retinoblastoma giving rise to a white pupillary re /f_l ex. This child was /f_i rst seen with a convergent squint and discharged without a fundus examination. He was next seen many years later with a ‘white re /f_l ex’ and died soon after diagnosis (courtesy of MA Bedford, FRCS). Any child with a white pupil (leukokoria) should be referred to an ophthalmologist to exclude retinoblastoma, although congenital cataracts may also cause this sign A blind painful eye may hide a melanoma or other ocular tumour

INTRAOCULAR TUMOURS Children

Retinoblastoma, the most common ocular malignancy of childhood, is a malignant tumour of the retina that can be bilateral in around one-third of cases. Half of cases are heredi tary (autosomal dominant) and are due to mutation of the gene on chromosome 13; children with a family history should be carefully monitored from birth. Remaining cases occur sporadically . Inherited retinoblastoma is more likely to be bilateral. Retinoblastoma is often not spotted until the tumour fills the globe and presents as a white reflex in the pupil or as a squint ( Figure 49.13 ). The di ff erential diagnosis includes reti - nopathy of prematurity , persistent fetal vasculature (PFV) and - intraocular infections. If the tumour is large, enucleation may be required, but radiotherapy , cryotherap y , chemotherapy or laser treatment can cure small lesions. Liaison with a paediatric oncologist is essential. Summary box 49.2 Intraocular tumours /uni25CF /uni25CF - RB1

Figure 49.12 Capillary haemangioma in a child. An orbital venogram demonstrates displacement of the second part of the superior oph thalmic vein (arrow) (courtesy of Dr Glyn Lloyd). Figure 49.13 Retinoblastoma giving rise to a white pupillary re /f_l ex. This child was /f_i rst seen with a convergent squint and discharged without a fundus examination. He was next seen many years later with a ‘white re /f_l ex’ and died soon after diagnosis (courtesy of MA Bedford, FRCS). Any child with a white pupil (leukokoria) should be referred to an ophthalmologist to exclude retinoblastoma, although congenital cataracts may also cause this sign A blind painful eye may hide a melanoma or other ocular tumour

INTRAOCULAR TUMOURS Children

Retinoblastoma, the most common ocular malignancy of childhood, is a malignant tumour of the retina that can be bilateral in around one-third of cases. Half of cases are heredi tary (autosomal dominant) and are due to mutation of the gene on chromosome 13; children with a family history should be carefully monitored from birth. Remaining cases occur sporadically . Inherited retinoblastoma is more likely to be bilateral. Retinoblastoma is often not spotted until the tumour fills the globe and presents as a white reflex in the pupil or as a squint ( Figure 49.13 ). The di ff erential diagnosis includes reti - nopathy of prematurity , persistent fetal vasculature (PFV) and - intraocular infections. If the tumour is large, enucleation may be required, but radiotherapy , cryotherap y , chemotherapy or laser treatment can cure small lesions. Liaison with a paediatric oncologist is essential. Summary box 49.2 Intraocular tumours /uni25CF /uni25CF - RB1

Figure 49.12 Capillary haemangioma in a child. An orbital venogram demonstrates displacement of the second part of the superior oph thalmic vein (arrow) (courtesy of Dr Glyn Lloyd). Figure 49.13 Retinoblastoma giving rise to a white pupillary re /f_l ex. This child was /f_i rst seen with a convergent squint and discharged without a fundus examination. He was next seen many years later with a ‘white re /f_l ex’ and died soon after diagnosis (courtesy of MA Bedford, FRCS). Any child with a white pupil (leukokoria) should be referred to an ophthalmologist to exclude retinoblastoma, although congenital cataracts may also cause this sign A blind painful eye may hide a melanoma or other ocular tumour