Author Information
Mehta N*, Parulekar SV**.
(* Third Year Resident, ** Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College & KEM Hospital, Mumbai, India.)
Abstract
It is difficult to differentiate between cervical atresia and vaginal atresia, as they may have similar imaging and clinical examination findings. Differentiating one from the other is essential as their management differs. We present an interesting case of a 12 year old girl who underwent hysterectomy and right ovarian cystectomy in view of cervical atresia with hematometra and hematosalpinx and right ovarian endometriotic cyst.
Introduction
Cervical atresia is a rare Müllerian anomaly found in 50% of the cases with vaginal aplasia.[1] Presence of any abdominal or pelvic, acute or chronic pain, in a pubescent girl must raise suspicion towards an obstructive genital syndrome. A blind vagina associated with a soft cystic mass palpated on rectal examination suggests menstrual blood retention above an obstacle. It is easy to diagnose an imperforate hymen by its bulge and bluish appearance due to accumulated blood behind it. But vaginal atresia and cervical atresia look similar. Transabdominal or transperineal ultrasonography (USG) is also not very reliable to differentiate cervical atresia from a high transverse vaginal septum. [1] Computerized tomography (CT) and magnetic resonance imaging (MRI) are useful in making a correct diagnosis.[2] An accurate preoperative diagnosis is essential for planning the surgical procedure.
Case Report
A 12 year old girl presented with complaints of lower abdominal dull aching pain for 2 months. She had no other significant medical or surgical history. There were no bowel or bladder complaints. She had never menstruated. On examination, her general condition was fair, she was afebrile, and the vital parameters were normal. Abdominal examination showed normal findings. On local examination, no vaginal opening was seen, as in a case of a transverse vaginal septum. On per rectal examination, a 4 cm long fibrous band was felt in the normal position of the vagina. A 4 x 6 cm soft cystic mass was felt above and posterior to it. The patient had an USG report from another center, suggestive of a complex ovarian cyst. USG performed at our center revealed a large hematometra with bilateral hematosalpinx. CT scan showed total vaginal atresia, hematometra and hematocervix. There was atresia of the lower end of the cervix.
The patient was taken up for exploratory laparotomy. The plan was to conserve the uterus if cervix was completely formed, and proceed with hysterectomy if there was cervical atresia. Intraoperatively, there was hematometra, hematocervix, absent external os and portio vaginalis along with a 10 cm long left hematosalpinx measuring 3 cm in diameter and a right ovarian endometrioma measuring 5 cm in diameter. The right fallopian tube was thickened. There were flimsy adhesions between the endometrioma and pelvic peritoneum which were lysed. To differentiate hematocervix from hematocolpos, anterior wall of the hematocervix was incised vertically for 1 cm diameter and the contents (thick blood) removed by suction. The cavity was explored digitally. The internal os admitted tip of a finger and the lower end was closed confirming absence of portio vaginalis. The uterosacral and cardinal ligaments were present and were attached to the hematocervix. Hysterectomy, left salpingectomy and excision of right ovarian endometrioma were done. Postoperative recovery was uneventful. Histopathological examination of the surgical specimen confirmed the diagnosis of atresia of the portio vaginalis, hematocervix, hematometra, left hematosalpinx and right ovarian endometrioma. The patient was counseled to follow up before marriage for vaginoplasty.
Figure 1. CT scan showing hematometra (HM), hematocervix (HC) and absence of vagina. Position of vagina (V) is shown by a solid fibrous tissue shadow.
Figure 2. 3D rendering of hematometra (HM) and hematocervix (HC) on CT scan. The lower end of the hematocervix is rounded, as expected with atresia of the portio vaginalis.
Figure 3. Findings at laparotomy - HM: hematometra, HC: hematocervix, HS: hematosalpinx.
Figure 4. Lateral view near the end of abdominal hysterectomy. It shows hematometra ((HM)) and hematocervix (HC). Lower end of the hematocervix is seen as a rounded closed part.
Figure 5. Surgical specimen. The hematocervix (HC) is opened to show its inside.
Discussion
Congenital cervical atresia can be classified into 4 types, based on clinical examination, USG and intraoperative findings. Type I cervical atresia is characterized by a normal functioning anatomic internal os, a relatively large hematocervix, and hematometra. In Type II cervical atresia, there is a short and solid cervix lacking uterosacral and cardinal ligament attachments to the lower uterine body. Type III cervical atresia is characterized by long and solid tissues below the uterine body, wherein both the uterine isthmus and cervical canal are atretic. Absence of the uterine isthmus is characteristic of Type IV, resulting in expansion of the uterine cavity and blood accumulation during menstruation.[3] Usually USG is adequate to make a diagnosis of the condition. CT and MRI are useful additional diagnostic techniques. Our patient belonged to type I of cervical atresia. In case of atresia of the portio vaginalis, the lower end of the hematocervix (distension of the supravaginal cervix with menstrual blood) is rounded. In case the portio vaginalis is present, there is some part of the upper vagina found below it, and instead of the rounded contour an asymmetrical dumbbell is seen. Total hysterectomy with ovarian conservation is the classical line of management for a case of cervical atresia.[4-7] Canalization techniques like uterovaginal anastomosis, creation of new cervix and its stenting, have a high risk of secondary stenosis of the cervix, up to 40–60%.[1] Other complications also include infections like pyometra, pyosalpinx, rectal/bowel perforation and fistula formation. A series of operations are usually required for failure of the first operation and to manage complications arising thereof. Approximately half of the cases of cervical atresia are associated with pelvic endometriosis.[7] Thus the condition must be diagnosed and hysterectomy done at the earliest to decrease the risk of back flow of menstrual blood and endometriosis.[8] Removal of the uterus is difficult to accept for the pubescent girl and her family. Hence diagnosis of cervical atresia must be carefully confirmed, to prevent unnecessary hysterectomy from being done for a case of isolated vaginal atresia, where a more conservative approach may be taken. If the imaging procedures prove to be inadequate to make a diagnosis, the diagnosis can be confirmed intraoperatively, by making a small incision on the anterior wall of the hematocervix and examining the lower end of the atretic cervix, as was done in this case.
References
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Mehta N, Parulekar SV. Cervical Atresia- A Difficult Diagnosis. JPGO 2017. Volume 4 No.9. Available from: http://www.jpgo.org/2017/09/cervical-atresia-difficult-diagnosis.html