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Background
Cervical agenesis (CA) comprises a rare subset of Mullerian anomalies with prevalence of 1/80,000 to 1/100,000, usually presenting with vaginal agenesis or atresia that can range in presentation from complete or partial absence of cervical tissue [1-3]. We present a rare case with a normal vaginal length with isolated CA with the objective of furthering the discussion to surgical approach. Traditionally, surgical management has included a hysterectomy of the uterine horn due to risk of ascending infection and death or utero-canalization procedures given advancement in MIS [4-8].
Case
We present a 19yo patient who presented at 12yo with monthly pain and amenorrhea. Initial lab workup was unrevealing and was started on COC for menstrual suppression given concern for a transverse vaginal septum. Initial pelvic ultrasound and MRI read as normal, but second radiologist read demonstrated truncation of the uterine horn at the level of the lower uterine segment, raising concern for rudimentary cervix (RC). She underwent EUA and vaginoscopy, revealing normal vaginal length with spongy-like bump at the vault apex, revealing evidence of RC. Patient counseled on options for continued suppression versus removal of the uterine remnant (UR). She wished to proceed with UR removal and underwent a robotic hysterectomy and bilateral salpingectomy. Intra-operative findings of the UR were consistent with MRI and vaginoscopy findings. Evidence of early-stage endometriosis was also noted. Final pathology showed UR with secretory endometrium without cervical mucosa.
Comments
There have been described case series for uterine sparing fertility options with laparoscopic assisted fistula creations, cervical drilling, and canalizations although much of the literature has combined cases of atresia and agenesis, which have different fertility outcomes [9-11]. In this case, we presented isolated CA in the setting of a UR despite normal vaginal length. It is important to distinguish between cases of CA and cervical dysgenesis, where more elements of cervical tissue may be present. Surgical management options vary depending on the partial presence or complete absence of cervical tissue, uterine size, and desire for future fertility. With increasing documentation of cases and more favorable follow up data, a new framework may need to be revisited given the difficulty in diagnosis of CA, while weighing the long-term outcomes.
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© 2023 Published by Elsevier Inc.
