Is unilateral uterine adnexa absence a congenital developmental abnormality or posteriority? Summary of 39 cases and literature review.

PURPOSE: Unilateral uterine adnexa absence with a normal uterus is extremely rare but meaningful in clinical. To date, this rare malformation is still not well understood. Here, we present a new case and systematically summarize 38 historical cases to make this rare anomaly be understood better by clinicians.

METHOD: The Chinese and English language medical literature were searched for all cases reported to date, and 39 were identified. All 39 cases were assessed for age, menstrual history, reproductive history, pelvic adhesions, other organ abnormalities, and mode of diagnosis.

RESULTS: Patient age ranged from 6 days to 46 years. Menstrual history included normal (n = 27), irregular (n = 4), or unknown (n = 7). Childbearing history included pregnancy (n = 21), no history of pregnancy (n = 3), and primary infertility (n = 7). The absence of uterine adnexa involved either the left (n = 17) or right (n = 22) structures, showing a right adnexa preferential bias (22/39).

CONCLUSIONS: The unilateral absence of uterine adnexa may be a congenital anomaly of reproductive organs; it does not significantly affect fertility or childbearing and is usually not diagnosed until adulthood.