Laparoscopic Management of Cervical Agenesis: A Rare Case Report

To present the successful laparoscopic management of a rare case of congenital obstructive Müllerian anomaly, identified as partial vaginal aplasia and cervical agenesis.

Surgical technique description.

A 13-year-old girl was referred for management of cyclic pelvic pain. Despite not having reached menarche, she exhibited secondary sexual characteristics. Magnetic resonance imaging revealed the presence of a uterus with a 6.4 × 5.2 cm hematometra. However, imaging could not conclusively confirm the presence of a proximal vagina and a cervix. Both ovaries and kidneys were observed to be in their normal positions. Because of the failure of hormonal and analgesic therapy to alleviate her pain, the patient underwent a mini-invasive surgical procedure.

Under general anesthesia, the external genitalia appeared normal. A 2 cm vaginal cul-de-sac was identified with the absence of the upper two-thirds of the vagina. Laparoscopy revealed endometriotic peritoneal lesions with widespread deposits of hemosiderin throughout the abdominal cavity. An enlarged uterus with a dilated isthmic portion (hematometra) was confirmed. In this video presentation, we describe the surgical steps involved in performing a direct laparoscopic utero-vaginal anastomosis to restore continuity of the genital tract. The patient experienced immediate relief postoperatively. Two months later, an elective vaginoscopy revealed a 3 cm long vagina with a permeable opening at the level of the anastomosis. Hysteroscopy indicated an endocervical canal, still dilated, with the presence of mucus. Passage through the endocervix allowed visualization of a uterus presenting a partial septum. Repeated hysteroscopy at 5 months showed no stenosis, and the patient reported regular and painless menstrual cycles after 2-year follow-up. Long-term follow-up is crucial to monitor for vaginal stenosis, prevent recurrence of pain and secondary amenorrhea. Fertility prognosis remains uncertain, as the functionality of the cervical anastomosis to sustain pregnancy is unknown. Nonetheless, pregnancy remains possible given the resolution of obstruction. Careful obstetrical monitoring will be required, and cesarean delivery is recommended.

Cervical agenesis can be effectively managed conservatively, with long-term success achievable using a direct laparoscopic utero-vaginal anastomosis. This case highlights the importance of a multidisciplinary approach involving pediatricians, gynecologists, and radiologists in the management of complex and rare Müllerian anomalies.