Current knowledge about the management of congenital cervical malformations: a literature review

Abstract

The prevalence of congenital cervical agenesis or dysgenesis ranges from 1/80,000 to 1/100,000, and in about 50% of these cases it coexists with congenital vaginal agenesis. This narrative review summarizes the contemporary knowledge in the field of conservative surgical restoration of the reproductive tract. The management of congenital cervical malformations aims to [1] provide relief from the obstructive symptoms, [2] establish normal sexual function, and [3] preserve the uterus for future fertility. In cases of cervical agenesis and vaginal aplasia, the surgical approach involves the creation of neovagina, the creation of neocervix, and then subsequent restoration of the continuity of the genital tract. In cases where vagina is not congenitally absent, the surgical approach involves either a direct uterovaginal anastomosis or initial creation of neocervix and then subsequent restoration of the continuity of the genital tract. The neocervix can be surgically created with small intestinal submucosa, split-thickness skin graft, full-thickness skin graft, peritoneal flap, or vaginal mucosa lined with a polytetrafluoroethylene graft. Most of the published cases report long-term menstruation and sporadic pregnancies. Conservative surgery of cervical congenital malformations could serve as a first-line treatment. Sexual function and menstruation are established in the majority of patients. Extirpatory surgery may be preserved for surgical failures after initial restoration of the continuity of uterus-cervix-vagina or in cases with more complex anatomy. (Fertil Steril Ò 2020;113:723-32. Ó2020 by American Society for Reproductive Medicine.

MANAGEMENT OF ENDOCRINE DISEASE: Diagnosis and management of primary amenorrhea and female delayed puberty

“…Obstruction of the menstrual flow results in hematometra, retrograde menstrual flow, endometriosis and pelvic adhesions which may complicate surgical treatment. Different treatment modalities exist (92).…”

Congenital Obstructive Müllerian Anomaly: The Pitfalls of a Magnetic Resonance Imaging-Based Diagnosis and the Importance of Intraoperative Biopsy

“…Nonetheless, many cases of successful reconstructive surgery, even with vaginal [ 15 ] or minimally invasive laparoscopy [ 16 , 17 ], and some instances of successful pregnancies and deliveries after surgery in patients with cervical agenesis or dysgenesis, have also been reported [ 18 ]. Therefore, conservative surgical management, such as uterovaginal or uterovestibular anastomosis, has been considered as a treatment option by several authors [ 18 , 19 ]. However, consensus on the optimal treatment for these rare obstructive MDAs remains controversial.…”

Successful trachelectomy and re‐vaginoplasty for cervico‐vaginal stenosis following unsuccessful uterovaginal anastomosis and vaginoplasty in congenital cervical and vaginal aplasia: description of technique and a case report

“…7,8 At worst, such patients may have to undergo hysterectomy for recurrent atresia despite having undergone rescue surgical treatment or experienced severe complications. 3 Herein, we present a case of successful trachelectomy and vaginoplasty following unsuccessful uterovaginal anastomosis and vaginoplasty in a patient with congenital cervical and vaginal aplasia.…”