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ISOLATED CONGENITAL MEGACYSTIS: CASE SERIES AND LITERATURE REVIEW
Hesham ELAGAMI
1
, Mark WOODWARD
2
, Guy NICHOLLS
2
and Mohamed SHALABY
2
1) Bristol Children Hospital, Paediatric Surgery, Bristol, UNITED KINGDOM - 2) Bristol Royal Hospital for Children,
Paediatric urology, Bristol, UNITED KINGDOM
PURPOSE
Congenital megacystis usually occurs in association with urological pathology (urethral obstruction or vesico-ureteric
reflux (VUR)) or as part of a syndromic condition.
Isolated congenital megacystis (ICM) is very rare and long-term outcome data are limited.
We describe our experience of ICM and review the published literature.
MATERIAL AND METHODS
ICM cases seen over 15yr were identified from a departmental database and the notes reviewed. A comprehensive
literature search was performed.
RESULTS
Three cases (2M; 1F) were identified; all were diagnosed antenatally; a family history was noted in one. In the boys,
renal impairment was noted initially which resolved with bladder drainage. Recorded bladder capacities at birth were
180-250ml, and MCUG confirmed normal urethral anatomy and no VUR. Clean intermittent urethral catheterisation
(CIC) was attempted in all; vesicostomy was required in one boy. In the other two, CIC was discontinued at 9 and 30
months.
EMBASE, PubMed and Medline were searched for megacystis (congenital or isolated). Five articles were identified (1984-
2014) describing 5 patients (3M; 2F). Antenatal diagnosis was made in four, and post-natal bladder capacities were
150-400ml. Renal function was normal in all. Vesicostomy was performed in one patient, CIC in another, and no active
bladder management was undertaken in three.
CONCLUSIONS
ICM is usually diagnosed antenatally, but postnatal ultrasound and MCUG are required to exclude urethral pathology or
VUR. Renal impairment may be seen initially, especially in boys, but seems to improve with adequate bladder drainage.
CIC is recommended, but can usually be discontinued once bladder emptying improves.