Agenesis Mullerian duct

Definition Dysgenesis (segmental agenesis and variable hypoplasia) of the Mullerian ducts (uterus and upper 2/3 vagina) [11,24] (Fig. 4.2). 

Failure of normal development of the mullerian ducts causes uterine agenesis or hypoplasia. Patients present with primary amenorrhea in adolescence. Agenesis or hypoplasia of any part of the genital tract (vagina, cervix, uterus, tubes) may occur either in isolation or, more, commonly, in combination. This relatively uncommon class of anomalies accounts for approximately 5% of mullerian duct anomalies. Vaginal agenesis is the most common subtype, and is often accompanied by uterine agenesis. 

Agenesis of a unilateral mullerian duct causes a single banana-shaped uterus with a single fallopian tube. If the rudimentary horn is either noncommunicating or lacks a cavity, it will not be detected by HSG. Sonographic findings, which are often subtle and easily overlooked, include a small uterine cavity, an... 

Due to the close developmental relationship of the urinary and the genital tract, malformations frequently occur in both of these systems. Major renal anomalies are common in patients presenting with unilateral obstruction or agenesis of duplicated structures derived from the mullerian duct. 

---