Author Information
Supriya
Poonia*, Shruti Panchbudhe**, M. N. Satia***
(* Third Year Resident, ** Assistant Professor,
*** Professor. Department of Obstetrics and Gynecology, Seth GS Medical College & KEM
Hospital , Mumbai , India
Abstract
An atypical case of scar
endometriosis with cervical agenesis in a 17 year old girl who presented with
complaints of primary amenorrhea, cyclical abdominal pain and cyclical bloody
discharge at the previous scar site of exploratory laparotomy is presented
here. In view of these complaints a definitive surgery in the form of
hysterectomy for a functional non communicating horn was performed.
Introduction
Mullerian duct anomalies
occur in 0.1-3.5 % of all women.[1] Congenital absence of the cervix
occurs in 80,000 to 100,000 births.[2]
Cervical agenesis is a
relatively uncommon complex mullerian malformation which results in disordered
vertical fusion of mullerian ducts. It usually presents with a normal
functional uterus, endometrium and tubes. External genitalia are apparently
normal. Presence of functional endometrium
with outflow tract obstruction (cervical agenesis) and patent fallopian
tube is found to be associated with endometriosis in 77% of cases.[3] The
ASRM classification of mullerian anomalies (American Fertility Society (AFS),
1988) Class Ia includes cases with
vaginal agensis combined with functional uterus and endometrium: the uterus may
be normal or present with fusion or resorption defects and the cervix may be
present, absent or hypoplastic. Cases with cervical agensis are included in
class Ib.[4] According to the AFS classification of uterovaginal
anomalies cervical agensis is categorised into Class IIb which includes
disorders of vertical fusion of mullerian ducts. Partial or complete cervical
atresia has been described in the presence or absence of vaginal atresia.
Case Report
A 17 year old unmarried woman presented with complaints of bloody
discharge from previous exploratory laparotomy site incision along with
cyclical abdominal pain for last 2.5 years, and primary amenorrhea. She had undergone an exploratory laparotomy with
right ovarian cystectomy followed by creation of neovagina 3 years ago at a
peripheral hospital for primary amenorrhoea with right tuboovarian mass. No intraoperative
details were available but histopathology report of ovarian cyst was suggestive
of endometrioma. The patient did not have menstrual cycles even after surgery. Her
abdominal pain was relieved for 6 months after which it had recurred. There was
no history of any other medical or surgical illness in the past. On examination
her vital parameters were stable and systemic examination revealed no abnormality.
Her secondary sexual characters were well developed. Her external genitals were
normal. Her abdomen was soft. There was
a Pfannensteil scar with induration of 2 cm at the right end of scar site. A
firm mass of 12 weeks’ size was felt arising from the pelvis deviated to the
right side. It was fixed at the scar site. Vaginal examination revealed a blind
vaginal pouch 2 cm deep. On per rectal examination the uterus was 12 weeks in
size, deviated to right side and was firmly adherent to the right side of the
scar. Ultrasonography (USG) of the pelvis showed left ovarian simple cyst 3.5 cms × 1 .5 cms and
hemorrhagic cyst of 3.9 × 2.7 cms and uterus was measuring 10×4.8×6 cm
with fluid in the endometrial cavity
with endometrial thickness of 1.8 cm. Sinogram was tried but could not be
performed. Magnetic resonance imaging of pelvis and abdomen showed a bulky
uterus adherent to the scar site with cervical aplasia and endometrial cavity
containing chronically retained blood products; a hemorrhagic cyst measuring
3.5×3 cm and a simple cyst in the left ovary. Both kidneys were normal. On
admission the patient had acute pain in the abdomen which was followed by
appearance of blood filled vesicles on the right side of the scar with
induration, which burst to discharge bloody fluid over 3 to 4 days. A diagnosis
of cervical and vaginal agenesis, hematometra and utero-abdominal fistula was
made. The patient and her parents were counseled about the advantages and
disadvantages of reconstructive surgery and definitive surgery in the form of
hysterectomy and they opted for definitive surgery. A laparotomy was performed.
There were dense adhesions between right side of the anterior abdominal wall
layers which were separated by blunt and sharp dissection. The rectus sheath
had a small opening in right corner through which chocolate brown material was
seen coming out which could have been due to presence of a sinus tract between
the uterus and rectus sheath or scar endometriosis. The uterus was 12 weeks in
size, deviated to right side and stuck to the rectus muscle and sheath by dense
adhesions. There were omental adhesions with the uterus which were divided.
There was a right sided tuboovarian mass of 4×5 cm which was adherent densely
to overlying anterior abdominal wall and the uterus. This was accidentally
ruptured during separation of adhesions and clear fluid was seen coming through
it. Left fallopian tube and ovary were normal and cervix was absent. In view of
these findings a hysterectomy with right sided salpingoophorectomy was
performed. The patient made an uneventful recovery and was discharged on day 7.
Histopathology report of hysterectomised specimen was suggestive of disordered
proliferation with fibrosis and occasional endometrial glands with evidence of
right sided corpus luteum cyst. Histopathlogy of the excised scar tissue in
rectus sheath showed endometriosis. The patient had no complaints of pain in
the scar and pelvis at regular follow up examinations in the outpatient
department.
Figure 2. T1 Saggital image show absent cervix (Yellow arrow) with blind
vaginal pouch (black arrow) with uterus distended with T1 hyper intense blood
products (Red arrow).
Figure 3. Appearance of bluish vesicle (black arrow) on the right side
of the scar followed by bursting of the vesicle and discharge of bloody fluid
suggestive of scar endometriosis.
Figure 4. Chocolate coloured material (black arrow) coming from the
right side of the rectus sheath.
Figure 5. A. Dense adhesions (black arrow) between the anterior
abdominal wall and the uterus. B. Dense adhesions between the omentum and
uterus.
Figure 6. Right sided tuboovarian mass (black arrow) with was densely
adherent to the right uterine surface.
Figure 7. Endometrial cavity with chocolate colored fluid (black arrow)
seen after bisection of uterus with absent cervix (green arrow) in bisected
uterus.
Discussion
Mullerian ducts form fallopian tubes, uterus and cervix and upper two
thirds of vagina. Anomalies in any of the three phases of organogenesis, fusion
or septal resorption results in a mullerian duct anomaly. The cervix is formed
at 20 weeks by condensation of stromal cell at a particular site around the
fused mullerian duct. Its differentiation involves both mesodermal and
endodermal tissue. Cervical agensis occurs due to a defect in elongation of
mullerian duct. Cervical agensis often occurs in association with absence of a
portion or all of the vagina.[5] A patient with cervical agensis with
functioning endometrium typically presents with primary amenorrhoea and
cyclical abdominal pain as in our case. Endometriosis is often associated with
outflow obstruction and functional endometrium leading to hematometra as in our
case. Scar endometriosis as seen in our patient is attributed to implantation
during previous gynaecological surgery. USG is helpful in suspected case of
mullerian agensis and is a preliminary investigation of choice. Confirmation of
the diagnosis with magnetic resonance imaging is mandatory. Various
reconstructive surgeries like uterovaginal anastomosis, cervical cannulation,
and cervical stent placement have been reported in literature for cervical
agensis; but the patient should be counseled about the complications such as
endometriosis , pelvic inflammatory disease, endometritis, persistent pelvic
pain, restenosis, pelvic infections, need of repeated surgeries, bladder and
bowel injuries due to adhesions in subsequent surgeries associated with
reconstructive surgeries and death due to reconstructive surgeries.[6] The
newly created cervix lacks cervical mucus glands necessary for sperm transport.
Hysterectomy is a definitive surgery and is recommended as the procedure of
choice in patients such as ours. It reduces the complications such as
cryptomenorrhoea, infections, endometriosis and need for multiple surgeries.
Reconstructive surgeries should be considered in selected patients after a
thorough discussion with the patient and her family after explaining the risk
and morbidity associated with reconstructive surgeries, and hysterectomy should
always be offered as a definitive treatment modality.
References
- Strassmann EO. Operations for double uterus and endometrial atresia. Clin Obstet Gynecol. 1961;4:240- 255.
- Suganuma N,Furuhashi M,Moriwaki T,Tsukahara S,Ando T, Ishihara Y. Management of missed abortion in a patient with congenital cervical atresia. Fertil Steril 2002;77: 1071-3.
- Shoupe D. Uterovaginal anaomalies: Management. In Mishell DR , Godwin TM, Brenner PF, (eds) Management of common problems in obstetrics and gynaecology, 4 th edition. Massachusetts, Blackwell publishing: 2002: 400-405.
- The American Fertility Society. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary of tubal ligation , tubal pregnancies, Mullerian anomalies and intrauterine adhesions.Fettil Steril 1988;49:944- 55.
- Rock JA, Breech LL. Surgery for Anomalies of the Mullerian Ducts. In Rock JA, Jones HW III, editors, Te Linde’s Operative Gynaecology. 10th edition: Wolters Kluwer Health Lippincott Williams and Wilkins 2008: pp 539-584.
- Casey AC, Laufer MR. Cervical agenesis: septic death after surgery. Obstet Gynecol 1997;90: 706 – 707.
Poonia S, Panchbudhe S, Satia MN. Cervical Agenesis With Scar Endometriosis. JPGO 2014 Volume 1 Number 9 Available from: http://www.jpgo.org/2014/09/cervical-agenesis-with-scar.html