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.