MURCS association and rectovestibular fistula: case report of a patient treated with one-stage posterior sagittal anorectoplasty and sigmoid loop vaginoplasty.
MURCS association is rare, first described by Duncan in 1979, including nonrandom association of Mullerian duct aplasia or hypoplasia (MU), renal agenesis or ectopy (R), and cervicothoracic somite dysplasia. A 5-year-old girl was admitted to the clinic with a complaint of anteriorly located anus. Physical and radiologic examination of the patient found MURCS association with anorectal malformation (ARM) of rectovestibular-type fistula. She had multiple vertebral anomalies, left renal agenesis, vaginal and uterine agenesia, with a normal female 46,XX karyotype. Posterior sagittal anorectoplasty (PSARP) and sigmoid loop vaginal reconstruction was performed as a one-stage operation for the treatment of vaginal agenesis and ARM. There were no complications in the postoperative period. This combined one-stage operation can be used easily in the treatment of some components of the MURCS association such as vaginal agenesis and ARM as an associated anomaly.
Günşar
Cüneyt
C
Department of Pediatric Surgery, Celal Bayar University, Medical Faculty, Manisa, Turkey.
Genç
Abdülkadir
A
Sencan
Aydin
A
Dağlar
Zafer
Z
Alparslan
Oğuz
O
Mir
Erol
E
D001003:Anal Canal / Q000002:abnormalities* / Q000601:surgery*; D002675:Child, Preschool; D012809:Colon, Sigmoid / Q000601:surgery / Q000637:transplantation; D005260:Female; D006130:Growth Disorders; D006801:Humans; D007668:Kidney / Q000002:abnormalities; D009333:Neck / Q000002:abnormalities; D010502:Perineum / Q000601:surgery; D012006:Rectovaginal Fistula / Q000601:surgery*; D012007:Rectum / Q000601:surgery; D012272:Ribs / Q000002:abnormalities; D012600:Scoliosis / Q000151:congenital; D013131:Spine / Q000002:abnormalities; D013577:Syndrome; D014599:Uterus / Q000002:abnormalities; D014621:Vagina / Q000002:abnormalities* / Q000601:surgery*