Author
Information
Parulekar
SV
Professor
and Head, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM
Hospital , Mumbai , India )
Abstract
Cervical
atresia in presence of a functioning uterus leads to development of
hematometra. Attempts at construction of s new cervix often fail and
necessitate a hysterectomy. The technique of abdominal or laparoscopic
hysterectomy in presence of cervical atresia differs from that for a normally
developed uterus. A case of uterus bicornis unicollis with cervical atresia,
hematometra and pelvic endometriosis treated by abdominal hysterectomy is
presented, with an emphasis on the variation in technique of hysterectomy.
Introduction
Cervical
atresia is an uncommon anomaly of development of the uterus. If the uterus is
functional, hematometra develops due to accumulation of the menstrual blood
above the atretic cervix.[1,2] If there is an absence of the entire
cervix, there is only hematometra. If the lower part of the cervix is atretic,
there is hematocervix to begin with and hematometra in addition to it as the
volume of the collected blood increases.[1] The uterosacral
ligaments are attached to the lower part of the supravaginal cervix. If that
part is atretic, these ligaments are not attached to the uterus. If the portio
vaginalis is atretic, the ligaments are present, attached to the supravaginal
cervix, now distended with accumulated blood. This anatomical variation has to
be kept in mind while performing abdominal or laparoscopic hysterectomy.
Case
Report
A 25
year old single girl presented with primary amenorrhea and cyclical lower
abdominal pain for one year. Her medical, surgical, and family history was not
contributory. Her general and systemic examination revealed no abnormality. Her
breast development was of Tanner’s stage 5, axillary and pubic hair development
was of stage A5 and P5 respectively. The external genitals and vagina were
normal. Rectal examination showed mildly enlarged uterus, but no pelvic masses.
Abdominopelvic ultrasonography showed a bicornuate uterus, atretic cervix
distended with blood, normal vagina, and normal kidneys. A computed tomography
was done. It confirmed the diagnosis. After counseling the patient and her
parents on different forms of operative treatment and the pros and cons of each
of them, they opted for an abdominal hysterectomy. An exploratory laparotomy
was done through an infraumbilical midline vertical incision. The operative
steps were as shown below.
Figure
1. The uterus is bicornis unicollis. Two small horns of the uterus are shown by
black arrows. Two spots of endometriosis are seen on the anterior surface of
the left horn. The urinary bladder (B) is on the right.
Figure
2. The cornual structures are held with straight pedicle clamps (black arrows)
and traction is made in the cranial direction.
Figure
3. The uterovesical fold of peritoneum is held cut transversely and its lower
cut edges (black arrows) are held with curved hemostats.
Figure
4. The cornual structures have been cut and ligated (yellow arrows).The urinary
bladder is dissected away from the supravaginal cervix and retracted with a
bladder retractor.
Figure
5. The right uterine artery is clamped (blue arrow). The cranial part of the
distended hematocervix is seen (black arrows).
Figure
6. Frontal view showing both the uterine arteries clamped, cut and ligated
(green arrows). A hemostat is passed under the serosa covering the posterior
aspect of the hematocervix (black arrow). A plane is created for separation of
the hematocervix from the serosa.
Figure
7. A finger is passed below the hematocervix (HC) from in front for bluntly
dissecting it off the connective tissue in front of and under it.
Figure
8. The hematocervix (black arrows) has been separated from the pelvic floor on
its anterior and inferior aspects. The edge of the uterovesical fold of
peritoneum (green arrows) is held away with a hemostat.
Figure
9. Index finger of the left hand (green arrow) is passed behind the hematocervix
under the posterior serosa, and its tip is made to emerge anteriorly, touched
with the tip of a curved hemostat from in front (black arrow). This
demonstrates the continuity of the planes of separation of the hematocervix
from the pelvic floor in front of, below and behind the hematocervix. Two
endometriotic spots are seen on the right horn (blue arrows). The edges of the
divided uterovesical fold of peritoneum (yellow arrows) are held forward.
After
separation of the hematocervix from the underlying fascia, the uterus and
hematocervix came off without having to cut any other tissues. The anterior and
posterior peritoneal edges were approximated with a continuous suture of No.
1-0 polyglactin. The abdomen was closed in layers. The patient made an
uneventful recovery.
Discussion
Cervical
atresia is a congenital malformation that is associated with development of a
hematocervix above the level of the obstruction, hematometra, and hematosalpinx
due to accumulation of menstrual blood.[1] It may be associated with
the development of pelvic endometriosis due to retrograde spill of the
collected blood through the fallopian tubes.[2] It could also be due
to celomic metaplasia since it seen even in absence of functioning endometrium
in rudimentary horns of the uterus. Efforts at creation of a new cervix usually
fail despite repeated attempts and a hysterectomy is usually required.[3]
Cervical
atresia could be of the vaginal part, in which case the hematocervix would have
the uterosacral ligaments attached to it. If the supravaginal cervix is
atretic, there is no attachment of any uterosacral ligaments to the distended
cervix. In the case presented, there was no attachment of uterosacral and
cardinal ligaments to the supravaginal cervix, indicating its atresia ( [Cx1 5 cm Fn Obst (hematocervix, hematometra)] of EAC classification of congenital malformations of the female genital tract). Another
point of importance is that there is no attachment of the hematocervix to the
vagina if it has developed. So separation of the connective tissue from its
surface would allow removal of the uterus and hematocervix without having to
cut any tissues, as one has to do while performing an abdominal hysterectomy in
case with normally developed uterus.[5,6] Both of these points were
illustrated in the case presented. The purpose of presenting this variation in
the technique of performing an abdominal hysterectomy is to draw the attention
of the residents in training to the anatomical variations in the presence of
cervical atresia, so that they can perform an abdominal hysterectomy without
accidentally cutting into tissues that do not need to be cut. This anatomical
variation has to be kept in mind while performing abdominal or laparoscopic
hysterectomy. That would reduce the incidence of complications like ureteral
injuries, bladder injuries, and hemorrhage from injury to pelvic blood vessels.
Acknowledgment
I thank Dr Durga Valvi for taking photographs of the operative steps and making them available to me.
Acknowledgment
I thank Dr Durga Valvi for taking photographs of the operative steps and making them available to me.
References
- Sadler TW. Langman's Medical Embryology. 12th ed. Philadelphia. Wolters Kluwer - Lippincott Williams Wilkins. 2012; pp. 232-259.
- John A. Rock JA, Breech LL. Surgery for Anomalies of the Mullerian Ducts. In Rock JA, Jones HW III, editors. Te Linde’s Operative Gynecology. 10th ed. New Delhi: Wolters Kluwer Health – Lippincott Williams & Wilkins 2008; pp. 539-584.
- Kansaria JJ, Gupta AS, Parulekar SV. Endometriosis in a case of endometrial agenesis and cervical atresia. J Obstet Gynaecol India 2002;52. 103.
- Parulekar SV. Classification Of Congenital Malformations Of The Female Genital Tract. JPGO 2015. Volume 2 No. 4, Available from: http://www.jpgo.org/2015/04/eac-classification-of-congenital.html
- Howard W. Jones III. Abdominal Hysterectomy. In Rock JA, Jones HW III, editors. Te Linde’s Operative Gynecology. 10th ed. New Delhi: Wolters Kluwer Health – Lippincott Williams & Wilkins 2008; pp. 727-43.
- Parulekar SV. Practical Gynecology and Obstetrics. 5th ed. Mumbai: Vora Medical Publications; 2011.
Citation
Parulekar
SV. Hysterectomy For Cervical Atresia. JPGO 2015. Volume 2 No. 5. Available
from: http://www.jpgo.org/2015/05/hysterectomy-for-cervical-atresia.html