Discussion
Managing cervical abnormalities, whether with or without a normal vagina, can be both challenging and rewarding. It demands creativity in devising management approaches.
Patient participation in the decision-making process is critical, as interventions ultimately affect her life and are associated with irreversible loss or negative impact on reproductive function. (3)
Hysterectomy was traditionally primarily the treatment of cervical agenesis to avoid serious complications following reconstructive surgery. (4)
Various techniques have been reported in the literature describing procedures creation of a neocervix and a neovagina if needed, and restoration of the continuity of the genital tract. The choice of neovagina and neocervix method and technique of neocervix and neovagina anastomosis is important. (2) Dornelas, reported eleven patients with vaginal agenesis underwent Utero-neovagina anastomosis using a Silicon mold covered by oxidized cellulose. One major postoperative complication occurred, which culminated in death. (6) In a survey done by Rock et al. cervical reconstruction was performed in 11 patients, 6 eventually experienced hysterectomy after obstruction of the neocervical canal. Two cases with cervical agenesis underwent uterovaginal anastomosis. Both required hysterectomy because of pelvic infection due to re-obstruction. (4)
A variety of treatment options are available for pelvic inflammatory disease (PID), including conservative management with IV antibiotics, laparoscopic aspiration, image-guided aspiration or drainage, laparoscopic salpingostomy with saline irrigation, and salpingectomy.(7) Rupture of the abscess can be fatal as high as 5%–10% of cases even with advanced treatment and surgical intervention.(8) Immediate and aggressive treatment can lead to a favorable outcome.
When OA is suspected, quick treatment is required to prevent adverse outcomes. (9) The treatment recommended for managing infectious complications after reconstructive surgery is hysterectomy.(10) Further, in another case series, 14 patients underwent laparoscopic-assisted uterovaginal anastomosis, nine of whom also underwent concomitant vaginoplasty. Among them, only one patient required a hysterectomy due to restenosis and infection. (11)
Kimble R et al. reported two patients with the combined congenital anomalies of complete vaginal agenesis and partial cervical agenesis presented difficulties encountered with the limitations of MRI in the accuracy of diagnosis and clinical correlation of imaging was not easy, as well as the development of life-threatening sepsis requiring hysterectomy and limited counseling by not being able to make an accurate diagnosis. Both patients were at first imaged as having enlarged endometrial cavities and cervical canals with what was thought to be an obstructive upper vaginal septum and an absent lower vagina. Both required initial neovagina creation, however, the cervixes were never clinically or surgically visualized. (5)
Three cases in the literature discussed sepsis-related deaths and obstruction secondary to cervical agenesis. Initially, these patients were thought to have a high transverse vaginal septum and were treated by creating a neovagina and establishing communication with the uterine cavity. (12) , (13) Despite initially having normal periods, all patients later presented to hospitals with severe infections and obstruction requiring hysterectomy due to infectious morbidity. In one case, the patient continued to decline, developing multi-system organ failure, and ultimately died after a hysterectomy. (12) In all five cases, including the above cases, there was a delay in the accurate definite diagnosis of the abnormality resulting in non-definitive initial treatment. Unfortunately, it was this delay that allowed the development of complications that led to significant morbidity and mortality.
Tareq Maraqa et al. reported conservative management of bilateral recurrent pyosalpinx in a 12-year-old girl secondary to retrograde menstruation caused by obstructed hemivagina due to Mullerian duct anomaly. In addition to irrigation and drainage of the abdomen and pelvis, IV and oral antibiotics were sufficient to achieve complete resolution without the need for a salpingostomy or salpingectomy.