Bladder wall telangiectasis causing life-threatening haematuria in ataxia-telangiectasia: a new observation.
We report two cases of life-threatening haemorrhage from bladder telangiectasia in children with ataxia-telangiectasia (A-T) who had been treated for lymphoma earlier in life. Whilst oculocutaneous telangiectasiae are an almost universal finding in this syndrome, bladder wall telangiectasis has not been reported previously. Both teenagers presented with recurrent severe haematuria due to extensive bladder telangiectasis. Recurrent haemorrhage was controlled with cystoscopic diathermy treatment. As A-T is a DNA repair disorder, it is possible that chemotherapy-mediated damage to the bladder mucosa prompted the development of clinically significant telangiectasis in these patients.


CONCLUSIONS
We advocate early cystoscopy for A-T patients who develop haematuria to investigate the cause, and cystodiathermy to pre-emptively treat developing lesions prior to haemodynamically significant haemorrhage.
Cohen
Jonathan M
JM
Department of Immunology, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK.
Cuckow
Peter
P
Davies
E Graham
EG
D000293:Adolescent; D001260:Ataxia Telangiectasia / Q000150:complications* / Q000503:physiopathology; D003972:Diathermy; D017809:Fatal Outcome; D006417:Hematuria / Q000209:etiology*; D006801:Humans; D008297:Male; D001745:Urinary Bladder Diseases / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy