Abstract Book - 26th ESPU Congress, Joint Meeting with ICCS + SPU + AAP/SOU + AAPU + SFU

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15:50 - 15:53
S3-6 (CP)
URETRAL DUPLICATION WITH ENLARGED CLITORIS AND FEMALE HYPOSPADIAS.
A RARE MALFORMATION.
Daniela Brindusa GORDUZA1, Jacques BIRRAUX2, Faustin MOUAFO TAMBO3, Isabelle VIDAL2, Claire-Lise GAY4, Claude
Pierette LE COULTRE5, Jean Pierre PRACROS6, Pierre MOURIQUAND7 and Pierre-Yves MURE7
1) Hopital Femme-Mere-Enfant, Centre Hospitalo-Universitaire de Lyon, Service d'Urologie Pédiatrique, Lyon, FRANCE -
2) University of Geneva Children's Hospital, Geneva, Switzerland, Pediatric Surgery Unit, Geneva, SWAZILAND - 3)
Yaounde Gynaeco-Obstetric and Paediatrics Hospital, Yaounde, Cameroon, Department of Surgery and Subspecialties,
Paediatrics Surgical Unit, Yaounde, CAMEROON - 4) Hôpital Mère-Enfant - Centre Hospitalo-Universitaire de Lyon,
Service d'Endocrinologie Pédiatrique -, Bron, FRANCE - 5) Hôpital Cantonal, University of Geneva Children's Hospital-
Geneva, Pediatric Surgery Unit, Geneva, SWAZILAND - 6) Hôpital Mère-Enfant - Centre Hospitalo-Universitaire de Lyon,
Service de Radiologie Pédiatrique, Bron, FRANCE - 7) Hôpital Mère-Enfant - Centre Hospitalo-Universitaire de Lyon,
Service d'Urologie Pédiatrique, Bron, FRANCE
PURPOSE
Urethral duplication in girls is of rare occurrence. We report two patients with urethral duplication, enlarged clitoris and
pre-anal vagina. Anatomy, diagnosis and surgical approaches are described.
MATERIAL AND METHODS
Patient 1 was 8 years of age and presented with an enlarged clitoris and a pre-anal orifice. Labia minora were absent
and labia majora were fused. There was no palpable gonad. Voiding was done through the pre-anal orifice with urinary
leakage at the tip of the clitoris. Anus was in normal position.
Patient 2 was a premature baby with a similar anatomical malformation. First gender assignation was made as male.
RESULTS
Karyotype was 46,XX and hormonal profile was normal for female in both patients. Radiologic and endoscopic
investigations showed a normal proximal urethra which divided in two channels below the pubic bone. The functional
urethra was hypospadic and opened in the anterior wall of the pre-anal conduit corresponding to the vagina. The second
urethra was hypoplastic, running along the ventral aspect of the enlarged clitoris.
The first girl had a feminizing genitoplasty with clitoral plasty and ablation of the accessory urethra with good
postoperative evolution. The second patient was reassessed as a girl. She had a vesicostomy and she is waiting for the
surgery.
CONCLUSIONS
Urethral duplication in girls is poorly described. As for boys with urethral duplication, the hypospadic urethra appeared
to be the main urethra which needs to be preserved. No clear explanation was found regarding the clitoris enlargement
even if embryological hypothesis can be proposed. A multidisciplinary approach of an experienced team is highly
recommended for management of such complex anomalies.