Urethral duplication in girls: Three cases associating an accessory epispadiac urethra and a main hypospadiac urethra.
BACKGROUND
Urethral duplication is extremely rare in girls, with less than 40 cases reported so far. Most of them present as a prepubic sinus. Literature is scare regarding aetiology, classification and management in other forms. This study presents three cases of sagittal urethral duplication in girls presenting a main hypospadiac urethra and an accessory epispadiac urethra.


METHODS
Medical records were retrospectively reviewed of three girls with urethral duplication managed over a 30-year period at a single institution. Circumstances of diagnosis, management and outcomes were analysed.


RESULTS
The oldest case presented as a neonatal retrovesical mass with an accessory clitoral stream, whereas the two more recent cases presented with antenatal hydrocolpos and bilateral ureterohydronephrosis. Cases 1 and 3 had an incomplete duplication, while Case 2 had a complete form. In Case 3, the duplication was associated with a urogenital sinus and an anteriorly placed anus. Management involved resection of the epispadiac accessory urethra to achieve continence, with dilatation and/or mobilisation of the hypospadiac one. All girls are now aged >5 years old and are continent, and one is old enough to have normal menstruation. Renal function is normal in all. The summary table presents the schematic anatomical description as shown on micturating cystourethrogram and endoscopy, as well as the management for each patient.


CONCLUSIONS
Step-by-step management is necessary in urethral duplication. The neonatal emergency is to release the urinary tract compression by evacuating urinary retention or hydrocolpos. Later in infancy, decision has to be taken regarding the urethras. If the resection of the epispadiac accessory urethra seems acceptable to achieve continence, the attitude towards the hypospadiac channel is more controversial and should be individualised. Embryologic and aetiopathogenic pathways are still missing to uniformly characterise the malformation.


CONCLUSIONS
Paediatric urologists should remember that there is a wide spectrum of urethral duplication in girls, and that various presentations exist beside the more classic prepubic sinus.
Department of Urology, Royal Children's Hospital, 50 Flemington Road, Parkville 3052, Australia. Electronic address: Aurore.Bouty@rch.org.au.
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Bouty
Department of Paediatric Surgery, Hôpital Pellegrin enfants-CHU de Bordeaux, Place Amélie Raba-Léon, 33076 Bordeaux, France.
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Lefevre
Department of Paediatric Surgery, CHU Saint Denis de La Reunion, Bellepierre, 97400 Saint Denis, France.
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Harper
Department of Paediatric Surgery, Hôpital Pellegrin enfants-CHU de Bordeaux, Place Amélie Raba-Léon, 33076 Bordeaux, France.
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Dobremez
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000601:surgery; D004842:Epispadias / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D006801:Humans; D007021:Hypospadias / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D014521:Urethra / Q000002:abnormalities* / Q000000981:diagnostic imaging
Girl; Urethra; Urethral duplication