Prevalence: 1/20,000 births. Most frequent malignant eye disease in childhood, representing 3% of all childhood cancers. It is caused by a mutation of a specific tumor suppressor gene (RB1 gene on 13q14). This mutation is often spontaneous, but it can be transmitted, then giving to the disease a family character, especially in bilateral forms (1/3 of cases). This gene normally codes for a nucleophosphoproteine that suppresses cell growth: it is an anti-oncogene. Monosomy 13q14 is another cause of retinoblastoma. The presence of a single normal allele gene is sufficient to produce the antioncogenic effect; for retinoblastoma to develop, both alleles must be involved. It is usually diagnosed in infants in presence of the onset of glaucoma, strabismus, or of leucocoria (white spot at the level of the pupil).
Retinoblastoma can spread along the optic nerve to the brain, and from there, metastasize to the skeleton, lungs and lymphatic system. There is a significant risk of secondary tumor: sarcoma, pinealoblastoma (3-5 %, suprasellar tumor, see the image below), glial tumor, melanoma, carcinoma. In case of association of a bilateral retinoblastoma with a pineoblastoma, the disease is called trilateral retinoblastoma.
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The treatment depends on the size of the lesion and tumoral extension. It may require laser surgery, cryotherapy, photocoagulation, or even enucleation, in addition to chemotherapy and/or radiation therapy.
Superselective chemotherapic injection of melphalan in the ophthalmic artery with a microcatheter introduced by the femoral artery is now used instead of radiotherapy in case of failure or relapse after systemic chemotherapy.
Anesthetic implications:
repeated anesthesias for treatments and monitoring of intraocular lesions. In case of intraocular superselective chemotherapy, cardiorespiratory autonomic reactions (decreased thoracic compliance without bronchospasm, major bradycardia, arterial hypotension) are observed in approximately 1/3 of cases mainly from the second session of chimioembolisation. These reactions are probably due to stimulation of the trigeminal nerve (trigeminocardiac reflex). and the preventive administration of atropine is ineffective.
References :
- Lohmann D.
Retinoblastoma.
Adv Exp Med Biol. 2010; 685:220-7.
- Shields CL, Shields JA.
Retinoblastoma management: advances in enucleation, intravenous chemoreduction, and intra-arterial chemotherapy.
Curr Opin Ophthalmol 2010; 21:203-12.
- Phillips TJ, McGuirk SP, Chahal HK, Kingston J et al.
Autonomic cardio-respiratory reflex reactions and superselective ophtalmic arterial chemotherapy for retinoblastoma.
Pediatr Anesth 2013; 23: 940-5.
- Kato MA, Green N, O’Connell K, Till SD, Kramer J et al.
A retrospective analysis of severe intraoperative respiratory compliance changes during ophtalmic arterial chemosurgery for retinoblastoma.
Pediatr Anesth 2015; 25: 595-602.
- Scharoun JH, Han JH, Gobin YP.
Anesthesia for ophthalmic artery chemosurgery.
Anesthesiology 2017; 126: 165-72
Updated: March 2022