Uterine cervix and proximal third of vagina agenesis with functional uterus: Case report and literature review

Ana Luíza Fonseca Siqueira, Marta Ribeiro Hentschke, Martina Wagner, Luiza Machado Kobe, Charles Schneider Borges, Vanessa Devens Trindade, Marcelo Moretto, Andrey Cechin Boeno, Adriana Arent
In: Human Reproduction Archives · 2019 · vol. 34 · doi:10.4322/hra.001319 · W3096792202
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This case report and literature review describe management options for a patient with uterine cervix and proximal vaginal agenesis, including hormonal suppression to prevent endometriosis or surgical reconstruction to preserve fertility.

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This case report describes a 19-year-old patient with cervical and proximal vaginal agenesis who presented with primary amenorrhea, cyclic pelvic pain, and dyspareunia despite having a functional uterus. The authors review treatment options ranging from hysterectomy to reconstructive surgeries like uterine-vaginal anastomosis, noting that hormonal suppression is often chosen for patients not seeking immediate pregnancy to alleviate symptoms. The paper explicitly mentions that suppressing menstrual flow can help prevent or treat possible foci of endometriosis, which may develop due to retrograde menstruation in this anatomical context. Relevance to endometriosis: listed as one indication for GnRH antagonists, though the paper's main focus is uterine fibroids.

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Abstract

We aimed to describe the case of a patient presenting cervix agenesis with presence of vagina and functioning uterus. Methods: A 19-year-old patient was referred to Human Reproduction service due to primary amenorrhea, cyclic pelvic pain, and dyspareunia. She was diagnosed with cervical and vaginal agenesis, and menstrual flow suppression was the chosen treatment. Results: Regarding treatment options, hysterectomy is the classic treatment; however, due to advances in minimally invasive surgery and reproductive medicine, procedures such as uterine-vaginal anastomosis have been proposed. Young patients with no current reproductive wish, may opt for hormonal suppression of the menstrual flow to minimize cyclical discomfort and prevent or treat possible foci of endometriosis. However, for those seeking pregnancy, techniques of assisted reproduction can be considered. The approach should always be individualized, considering the anatomical details, clinical aspects, and patient's opinion. Conclusions: Management of cervical agenesis is a challenge due to the complexity of the malformation and the difficulty in restoring and preserving fertility. Lastly, report such rare conditions and its treatment options, seems to be beneficial to help other patients with similar conditions.
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Abstract

Objectives: We aimed to describe the case of a patient presenting cervix agenesis with presence of vagina and functioning uterus. Methods: A 19-year-old patient was referred to Human Reproduction service due to primary amenorrhea, cyclic pelvic pain, and dyspareunia. She was diagnosed with cervical and vaginal agenesis, and menstrual flow suppression was the chosen treatment. Results: Regarding treatment options, hysterectomy is the classic treatment; however, due to advances in minimally invasive surgery and reproductive medicine, procedures such as uterine-vaginal anastomosis have been proposed. Young patients with no current reproductive wish, may opt for hormonal suppression of the menstrual flow to minimize cyclical discomfort and prevent or treat possible foci of endometriosis. However, for those seeking pregnancy, techniques of assisted reproduction can be considered. The approach should always be individualized, considering the anatomical details, clinical aspects, and patient’s opinion. Conclusions: Management of cervical agenesis is a challenge due to the complexity of the malformation and the difficulty in restoring and preserving fertility. Lastly, report such rare conditions and its treatment options, seems to be beneficial to help other patients with similar conditions.

Keywords

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