Nazim Syed Muhammad, Zaidi Zafar
The Indus Hospital, Karachi, Pakistan.
J Pak Med Assoc. 2012 Oct;62(10):1086-9.
Agenesis of Urinary bladder is an extremely rare congenital genitourinary anomaly and is associated with other severe malformations that are incompatible with life. About 60 cases and 22 live births only have been reported in English literature. We report an eight year old girl with triad of complete agenesis of bladder and urethra, solitary functioning left kidney and an ectopic ureter opening into the vagina. The management of the child along with embryo-pathogenesis and literature review is discussed.
膀胱缺如为一种极其罕见的先天性泌尿生殖系统畸形,常伴有其他严重的、与生命不相容的畸形。英文文献中仅报道了约60例病例,其中仅有22例存活。我们报告了一名8岁女孩,其患有膀胱和尿道完全缺如、孤立的左肾有功能以及异位输尿管开口于阴道的三联征。本文讨论了该患儿的治疗方法以及胚胎发病机制,并进行了文献综述。