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CONGRESS OF THE ESPU
15:16–15:19
S18-3 (PP)
LONG TERM OUTCOMES FOR XY CLOACAL EXSTROPHY
RAISED AS FEMALE
Dan WOOD
1
, Ben NAMDARIAN
1
, Sarah CREIGHTON
2
, Polly CARMICHAEL
3
and
Abraham CHERIAN
3
1) University College London Hospitals, Adolescent Urology, London, UNITED KINGDOM - 2) University College
London Hospitals, Womens Health, London, UNITED KINGDOM - 3) Great Ormond Street Hospital, Paediatric Urology,
London, UNITED KINGDOM
PURPOSE
Cloacal exstrophy is a complex anomaly with overt pelvic, bladder, genital and bowel deformities.
Other associated anomalies include the spine and kidneys. Surgical advances have meant survival
for these patients. Historically, those with an XY karyotype were often felt to have such major genital
deformity - they underwent gonadectomy, genitoplasty, and were raised female. This paper exam-
ines the outcomes for this unique and complex cohort.
MATERIAL AND METHODS
A retrospective review of patient records was performed of XY cloacal extrophy cases at UCLH
(2007–2017). Outcomes related to previous surgery, renal function and gender reassignment were
reviewed.
RESULTS
18 patients were identified. 2 were lost to follow-up, data for the 16 remaining patients were ana-
lysed – one had no data for childhood surgery. Mean age at review was 27.7 years (22–38) with all
raised female. All had an enterocystoplasty, 9 had a Mitrofanoff channel (4 required revision). 8 had
a childhood bowel vaginoplasty, 7 required revision. 2 further chose vaginoplasty in early adulthood.
2 had bladder stones, 1 nephrolithiasis. Median creatinine was 88 umol/l (range 48–253) – 7 had
chronic renal failure, 1 patient underwent renal transplant. 2 patients had reassigned to male gender.
CONCLUSIONS
These patients represent early success of surgical innovation leading to the current expectation of
survival with a rare and complex anomaly. Many have undergone complex procedures requiring
later revision, particularly mitrofanoff channel. There were poor outcomes from ileal vaginoplasty in
childhood. Renal function requires close monitoring for CKD progression, gender reassignment is
a potential issue.