Clinical History
A 15-year old female patient was referred to our institution because of bilateral lumbar and pelvic pain.
Imaging Findings
A 15-year old female patient was referred to our institution because of bilateral lumbar and pelvic pain. Pain was lasting since 10 months and was continous, with variable intensity, but no
relationship with the menstrual cycle, that was normal. Gynaecological examination demonstrated a large palpable pelvic mass, displacing the left lateral wall of the vagina, and extending superiorly
until the transverse umbilical plane. The patient underwent ultrasound and MR imaging of the pelvis (the exams were performed during the menstrual period). The diagnosis was confirmed by surgery.
Discussion
Uterus didelphys with an imperforate hemivagina is an embryonic malformation of the genitourinary system of the female that occurs between the 12th and 16th week of pregnancy. It is caused by the
failure of fusion of the paramesonephric (Mullerian) ducts. This defect may manifest itself as a duplication of all or part of the female reproductive system. The obstruction of one hemivagina will
block outflow, resulting in complications such as hematocolpos, hematometra and hematosalpinx. The persistence of this situation also may be complicated by the occurrence of endometriosis as a result
of blood reflux into the abdominal cavity. These anomalies frequently are accompanied by kidney and urinary tract malformations (i.e., kidney agenesis and dysplasia, double collecting system, ectopic
ureter) on the same side as the defect . The manifesting symptoms usually appear only after menarche and consist of dysmenorrhea, severe abdominal pain, and the presence of an intraabdominal or
pelvic mass. Ultrasonography is the first choice technique: after a careful anamnesis and physical examination, transabdominal US can confirm suspicions in most cases revealing the existence of two
hemiuteruses, hematocolpos and eventually the presence of unilateral renal agenesis. After either an inconclusive US or an US examination confirming the suspect, the MR imaging is mandatory in order
to obtain a precise diagnosis or to have a definitive preoperatory confirmation and a multiplanar spatial balance. MR imaging completes the diagnostic sequence in an non-invasive way and allows an
early and limited surgical treatment, substituting explorative laparotomic or laparoscopic procedures and assuring the best fertility prognosis. The aim of surgery is preservation of normal
fertility. Excision of the vagina septum is recommended as the procedure of choice.
Differential Diagnosis List
Hematocolpos with uterus didelphys and vaginal duplication.
Final Diagnosis
Hematocolpos with uterus didelphys and vaginal duplication.