Günşar Cüneyt, Genç Abdülkadir, Sencan Aydin, Dağlar Zafer, Alparslan Oğuz, Mir Erol
Department of Pediatric Surgery, Celal Bayar University, Medical Faculty, Manisa, Turkey.
J Pediatr Surg. 2003 Feb;38(2):262-4. doi: 10.1053/jpsu.2003.50060.
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.
MURCS综合征较为罕见，由邓肯于1979年首次描述，包括苗勒管发育不全或发育不良（MU）、肾缺如或异位（R）以及颈胸段体节发育异常的非随机关联。一名5岁女童因主诉肛门位于前方而入院。对该患者进行体格检查和影像学检查发现，MURCS综合征合并直肠前庭瘘型肛门直肠畸形（ARM）。她有多处椎体异常、左肾缺如、阴道和子宫发育不全，核型为正常女性46,XX。行一期后路矢状位肛门直肠成形术（PSARP）和乙状结肠袢阴道重建术，以治疗阴道发育不全和ARM。术后无并发症。这种一期联合手术可轻松用于治疗MURCS综合征的一些合并症，如阴道发育不全和作为相关异常的ARM。
