Clinical features of congenital complete vaginal atresia combined with cervical aplasia: A retrospective study of 19 patients and literature review

Abstract

Congenital vaginal atresia is a rare congenital reproductive tract abnormality. To assess the clinical manifestations and feasibility of preserving uterus for congenital complete vaginal atresia with cervical aplasia, nineteen cases who underwent surgical treatment in West China Second Hospital of Sichuan University were retrospectively studied. The cervical status, clinical manifestations, the rate of vaginal re-stenosis and pelvic inflammation after surgery were assessed. Additional 101 similar cases searched through digital Pub Med were included to analyze the feasibility of preserving the uterus. Periodic abdominal pain, primary amenorrhea, and pelvic mass were the primary signs and symptoms. According to the magnetic resonance imaging (MRI), all the uterine cavities expanded, and the atresia sites were above the inner urethral orifice. Data of the cases preserving uteri from both our hospital and the literature showed the rate of re-stenosis in patients with external cervical obstruction was 15.9% while it was 40% in the other types of cervical aplasia (P = .026). The rate of recurrent pelvic inflammation and hysterectomy was 2.3% for cervical external os obstruction and 8% for the other cervical aplasia types(P = .296). In conclusion, vaginoplasty and cervicovaginal anastomosis could preserve the fertility for complete vaginal atresia with cervical external os obstruction.

Keywords: cervical aplasia; cervicovaginal anastomosis; congenital complete vaginal atresia; tracheloplasty; vaginoplasty.

FIGURE 1

Schematic diagram of three types of congenital vaginal atresia

FIGURE 2

Schematic diagram of four kinds of congenital cervical aplasia

FIGURE 3

T2W pelvic transverse image showing a complete vaginal and cervical atresia case with “T” shaped uterine body. The arrow indicates the “T” shaped uterine cavity

FIGURE 4

T2W pelvic sagittal images showing complete vaginal atresia with different cervical aplasia. A, External cervical os. obstruction. Arrow A indicates an expanded uterine cavity, arrow B indicates a dark cervical interstitial ring, arrow C indicates intermediate brightness cervical muscle, which is continuous with uterine muscular layer, arrow D indicates the level of the inner urethral orifice, and arrow E indicates the obstructed external cervical os. B, Complete cervical atresia. Arrow A indicates an expanded uterine cavity; arrow B indicates atresic cervical canal; arrow C indicates the blinded cervix; arrow D indicates the level of the inner urethral orifice. C, Cervical agenesis. Arrow A indicates an expanded uterine cavity; arrow B indicates a blinded lower segment of the uterus; arrow C indicates the level of the inner urethral orifice