Department of Gynaecology, Coimbra Hospital and University Centre, Coimbra, Portugal
Department of Reproductive Medicine, Dexeus University Hospital, Barcelona, Catalunya, Spain.
BMJ Case Rep. 2021 Oct 27;14(10):e242475. doi: 10.1136/bcr-2021-242475.
Isolated cervical aplasia (ESHRE/ESGE U0C4V0) is a rare condition with an incidence of approximately 1:100,000 births.This congenital malformation of the female genital tract represents an impairment of the outflow tract and is an inevitable cause of infertility. Patients usually present with pelvic pain or haematometra and surgical treatment is needed. Conservative management is the first line of approach, allowing for future fertility. However, complications are not negligible. Choosing the best surgical technique remains controversial as few follow-up studies have been published.We describe a case report of isolated cervical aplasia diagnosed in a 16-year-old patient, managed by a canalisation procedure using a Foley catheter. Following failure of this approach, a levonorgestrel intrauterine system was inserted, which remained efficient after 4 years.This case adds to the few reports of success in the management of this challenging clinical entity and might guide clinicians to avoid non-conservative approaches in young patients.
孤立性宫颈发育不全（ESHRE/ESGE U0C4V0）是一种罕见疾病，发病率约为每 10 万出生婴儿中有 1 例。这种女性生殖道的先天性畸形代表了流出道的损伤，是不可避免的不孕原因。患者通常表现为盆腔疼痛或积血，需要手术治疗。保守治疗是首选方法，可保留未来的生育能力。然而，并发症不容忽视。选择最佳的手术技术仍然存在争议，因为发表的随访研究很少。我们描述了一例 16 岁患者孤立性宫颈发育不全的病例报告，采用 Foley 导管进行了管腔化手术进行治疗。该方法失败后，放置了左炔诺孕酮宫内节育系统，4 年后仍保持有效。该病例增加了少数成功管理这种具有挑战性的临床实体的报告，并可能指导临床医生避免对年轻患者采取非保守方法。
