Keywords
cervicovaginal atresia - cervical hypoplasia - vaginal agenesis - uterovaginal anastomosis
- vaginoplasty - autologous peritoneum - laparoscopic surgery
Cervicovaginal atresia with a functional uterus is rare. This condition is caused
by an abnormal formation or fusion of the Müllerian ducts and is often associated
with vaginal aplasia. It may be diagnosed before or after puberty during medical examinations
for periodic abdominal pain, amenorrhea, or dyspareunia. A total hysterectomy was
the recommended treatment of choice for curative treatment, although conserving fertility
required invasive vaginoplasty and cervicoplasty. However, identifying and reshaping
the cervix during surgery can be difficult, and there is a high risk of damage to
the bladder or intestines. In addition, a postoperative restenosis and closure of
the vaginal or cervical canal can be a recurrence of the symptoms. Additionally, as
many patients are young, minimally invasive surgeries that will not lead to recurrence
are desirable. Recently, there have been a few reports of vaginal surgeries that combined
laparoscopic or robot-guided techniques. We report a case in which we adjusted the
pre- and postoperative management, and combined laparoscopic surgery with vaginal
surgery using autologous peritoneum and a variety of surgical instruments to perform
a minimally invasive and safe vaginoplasty and cervicoplasty with no restenosis or
closure.
Case Presentation
The patient was a 19-year-old nulligravida woman who was examined for primary amenorrhea
at another hospital. On examination, the vulva was normal, but vaginal agenesis was
observed; the uterine cervix was restiform shaped, and the cervical canal line structure
was indistinct, which indicated cervical hypoplasia. The patient was referred to our
hospital for further examination and treatment. A pelvic examination revealed that
the vagina had a slight recession with a blind end. Transrectal ultrasonography showed
absence of a cervical canal line in the uterine cervix, fluid accumulation in the
intrauterine cavity, and normal ovaries on both sides. Pelvic magnetic resonance imaging
showed similar findings ([Fig. 1]). Chromosomal testing was 46 XX. Based on this, congenital vaginal agenesis and
cervical hypoplasia were diagnosed and a decision was made to perform an elective
surgery.
Fig. 1 (A) Vagina with blind end. (B, C) Uterine cervix is thin, restiform shaped, and the cervical canal line structure
is indistinct. Bilateral adnexa are normal. Accumulation of menstrual blood is seen
in the uterine body.
Surgical Technique
The management was divided into three steps: (1) preoperative management, (2) surgery,
and (3) postoperative management. The procedure was as follows:
-
Preoperative management
Due to the presence of vaginal agenesis, the vagina was reshaped preoperatively in
an outpatient setting. The patient was given instructions on the use of a vaginal
Dilator S (Atom Medical Corp., Tokyo, Japan) ([Fig. 2]) to be used at home (Frank's technique). After 10 months, she could accommodate
a larger sized Dilator M and had a depth of 7 cm, which made sexual intercourse possible.
Subsequently, it was decided to perform a radical surgery.
-
Surgery ([Fig. 3])
-
Laparoscopic approach
Peritoneum to be grafted onto the cervical canal was harvested laparoscopically. The
peritoneum at the vesicouterine pouch was incised, and the bladder and uterine cervix
were detached and developed to identify the hypoplastic uterine cervix.
-
Vaginal approach
For the vaginal surgery, a cold knife was used to make a 1.5-cm incision in the blind-end
of the vagina. Taking care not to damage the bladder or rectum, the laparoscopic light
source was used as a landmark while reshaping the vagina by excavating toward the
abdominal cavity.
-
Laparoscopic-assisted vaginal approach
Under laparoscopic observation, traction and fixation of the uterine cervix inside
the vagina were performed from the vaginal side.
-
Vaginal approach
To shape the cervical canal, a 3-mm skin biopsy punch (Kai Industries, Tokyo, Japan)
was used to hollow out and resect the external cervical Os ([Fig. 4]). Next, a communication between the shaped cervical canal and the dilated uterine
cavity was created.
-
Vaginal approach
An H/S Elliptosphere catheter (CooperSurgical, CT, USA) used for hysterosalpingography
was placed in the uterine cavity ([Fig. 5]).
-
Vaginal approach
The laparoscopically harvested autologous peritoneum was wrapped around the catheter
and fixed in the cervical canal. Artificial dermis (Pelnac, Smith & Nephew) was used
to promote epithelization and prevent infections in the vagina, which had damaged
epithelium.
-
Postoperative management
Two weeks postoperatively, epithelization of the vaginal wall was confirmed, and the
patient was discharged from the hospital with the catheter in place. Six weeks postoperatively,
the catheter was removed from the uterus and epithelization of the uterine and cervical
cavities was reconfirmed. To prevent vaginal restenosis, self-dilation with a vaginal
dilator M was resumed, which the patient had stopped when she had become capable of
sexual intercourse.
Two months postoperatively menstruation was confirmed, which was without any pain
or difficulty in discharging the menstrual blood. Eight months postoperatively, restenosis
has not occurred.
Fig. 2 Vaginal dilators (Atom Medical) developed by Takeda are easy to hold and convenient
for self-management. The point of the dilator is sharp and easy to care.
Fig. 3 Schematic diagram of the surgery. (1) Laparoscopically, the hypoplastic uterine cervix
was identified, then peritoneal peritonectomy using for grafted onto the cervical
canal was performed. (2) In the vaginal surgery, incision was made into the blind
end to shape the vagina. (3) Traction and fixation of the hypoplastic uterine cervix
was performed from the vaginal side. (4) Skin biopsy punch of 3 mm was used to hollow
out and resect the external cervical Os; communication between the cervical canal
and dilated uterine cavity was created. (5) Catheter used in hysterosalpingography
was placed in the uterine cavity guided by transrectal ultrasonography. (6) Autologous
peritoneum was fixed to the catheter and placed in the cervical canal. Artificial
dermis was used to promote epithelization and prevent infections in the vagina, which
had damaged epithelium.
Fig. 4 Biopsy punch (Kai Industries) is able to choose diameter and length of blade for
each size of cervix.
Fig. 5 H/S Elliptosphere catheter (CooperSurgical) used for hysterosalpingography is suitable
to place in uterus.
Discussion
The female genitalia differentiate and develop from the Müllerian ducts and urogenital
sinus during the embryonic stage. The former become the ovaries, uterine body, cervix,
and upper third of the vagina, while the latter becomes the lower two-thirds of the
vagina. Formation of the female internal genitalia consists of the following steps:
(1) formation of two Müllerian ducts, (2) fusion of the Müllerian ducts, (3) fusion
of the lower Müllerian ducts and urogenital sinus, and (4) absorption of the septum.
Abnormal development at any of these stages can lead to morphological defects.
Vaginal hypoplasia is seen in 1 out of every 4,000 to 5,000 women, with the Rokitansky–Kuüster–Hauser
syndrome being a well-known congenital vaginal defect. In cases of cervical hypoplasia
or aplasia similar to the present one, the abnormality is thought to have occurred
relatively early during the formation of the Müllerian ducts, although the etiology
remains unclear. Cervicovaginal atresia is even rarer, with less than 200 cases reported
from when it was first described by Roberts et al from 1942 to 2011.[1] Only 2 to 7% of cervicovaginal atresia cases have functional uteruses with vaginal
agenesis.[2] The conventional treatment was to create a communication between the uterine body
and vagina. However, Buttram and Gibbons noted that, as the cervical canal created
in these operations are fistulas that do not secrete cervical mucus and can cause
severe ascending infections and the patients remain infertile. Therefore, hysterectomy
is not recommended.[3] Recently, surgeries that conserve the uterus out of consideration of fertility have
been reported. Most of the reports were of laparotomies,[4]
[5]
[6]
[7] although in 2008 Fedele et al reported using a laparoscope in a minimally invasive
surgery with excellent cosmetic results.[8] Robot-assisted surgeries have also been reported.[9] In the present case, we combined laparoscopic surgery with vaginal surgery. The
surgery was performed laparoscopically because it allowed us to observe the inside
of the abdominal cavity and later harvest the free peritoneum. Further, laparoscopically
developing the peritoneum at the vesicouterine pouch to confirm the uterine cervix
and use of the laparoscope's light source as a landmark to secure the uterine cervix
transvaginally was useful for avoiding complications and shortening the operation.
There are few published reports on cervicoplasty techniques. Most have described creating
a communication with the vagina by inserting a transuterine catheter via the uterine
body and excavating sharply toward the uterine body from the vaginal blind end. However,
a high risk of bladder or rectal damage from proceeding in a mistaken direction and
the difficultly of the manipulations make this a difficult step. Particular care is
needed when there is considerable distance between the uterus and the vaginal blind
end. In the present case, domestic use of Frank's technique as a form of preoperative
therapy shortened this distance and made the vaginal surgery safer. It is the American
College of Obstetricians and Gynecologists committee's recommendation to use Frank's
technique as the first-line approach in cases of vaginal aplasia.[10] At our hospital, we have found that dilators are easy to use at home, and those
developed by Takeda have improved handles and tips and can also be used to prevent
postoperative stenosis.[11]
The most important things to note in the present case are the creation of a wide cervical
canal and the efforts to achieve early epithelization of the cervical mucosa. A canal
with a certain width needs to be created in cases of hypoplasia in which the cervical
canal line is not visible, to allow menstrual blood to discharge sufficiently. In
the present case, we adopted a skin biopsy punch to shape the uterine cervix ([Fig. 4]). Biopsy punches are often used in dermatology and can cut out tube-like holes in
tissue. Blades with different diameters and lengths can be selected to suit the design
of the cervical canal. Others have reported inserting a Foley's catheter from the
uterus to the vagina to prevent natural closing of the hollowed-out cervical area,
obtaining good results with no restenosis.[9] In the present study, we used an H/S Elliptosphere catheter used in hysterosalpingography,
which was passed from the vagina though the cervical canal to the uterine cavity.
In addition, we promoted epithelization of the cervical canal mucosa by wrapping the
autologous peritoneum collected laparoscopically around the catheter and fixing it
in the uterine cervix. This vaginoplasty method was reported by Takeda[12] and is safer and cheaper than Gore-Tex[13] and artificial dermis.[7]
[14] The autologous peritoneum grafted onto the vagina had changed into squamous epithelium
after 3 weeks, and after 8 weeks the patient was able to engage in sexual intercourse.
This indicates that because autologous peritoneum changes the epithelium around where
it is fixed, the peritoneum grafted onto the cervical canal will epithelize quickly
without leaving a scar, which creates a cervical canal that does not restenose or
close after removing the catheter from the uterus.
As discussed, the skillful combination of preoperative management, modifications to
the surgical technique, device selection, early epithelization of the cervical canal
mucosa through peritoneal grafting, and postoperative management were able to create
a cervical canal that did not undergo restenosis or close. Later, care regarding pregnancy
and childbirth will be necessary, which will require long-term follow-up. Furthermore,
this surgical technique may be applicable in conditions such as double uterus or unilateral
cervical canal hypoplasia.
Conclusion
We developed a novel method of cervicoplasty that did not lead to restenosis or closure.
A skin biopsy device was used to hollow out the cervical tissue and create a wide
cervical canal to prevent restenosis. In addition, grafting autologous peritoneum
onto the cervical canal was important in achieving early epithelization of the cervical
mucosa.
