Journal of Gynecological Endoscopy and Surgery
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 Table of Contents  
CASE REPORT
Year : 2011  |  Volume : 2  |  Issue : 2  |  Page : 94-96  

Laparoscopic management of congenital cervico-vaginal agenesis


1 Vardhman Infertility and Endoscopy Centre, Muzaffarnagar, Uttar Pradesh, India
2 Department of Obstetrics and Gynecology, CMC and H, Ludhiana, Punjab, India

Date of Web Publication29-Jun-2013

Correspondence Address:
Reema Sircar
Street No. 9, Satgur Nagar, Saloh Road,Nawanshahr Doaba - 144 514, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-1216.114158

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   Abstract 

We report a case of cervical agenesis or lack of uterine cervix. It is a rare mullerian anomaly and occurs in 1 in 80,000-100,000 births. The patient presented to us with primary amenorrhea and cyclical left lower abdominal pain. She was diagnosed to have cervical agenesis associated with vaginal agenesis and left endometriotic cyst. Neovagina was created laparoscopically. Utero-vaginal anastomosis was tried but it was not technically feasible. Subsequently, laparoscopic hysterectomy was done due to recurrent endometriotic cyst formation.

Keywords: Cervical agenesis, cervical atresia, laparoscopic, mullerian anomaly


How to cite this article:
Jain N, Sircar R. Laparoscopic management of congenital cervico-vaginal agenesis. J Gynec Endosc Surg 2011;2:94-6

How to cite this URL:
Jain N, Sircar R. Laparoscopic management of congenital cervico-vaginal agenesis. J Gynec Endosc Surg [serial online] 2011 [cited 2017 May 25];2:94-6. Available from: http://www.gynecendoscopy.org/text.asp?2011/2/2/94/114158


   Introduction Top


Cervical aplasia is a mullerian anomaly, in which patients lack a uterine cervix and the lower uterine segment narrows to terminate in a peritoneal sleeve at a point well above the normal communication with the vaginal apex. Various portions of vagina can also be atretic. We report a case of cervical aplasia, associated with a functioning uterine corpus and an absent vagina.


   Case report Top


Reporting the case of an 18-year-old unmarried girl, who presented to us with history of primary amenorrhea and cyclical lower abdominal pain. On examination, she had normal secondary sexual characters. Local examination revealed absent vagina. Bimanual rectal examination was normal. Her hormonal profile and all the other routine blood tests were normal. Karyotype was XX which is the normal female karyotype. Ultrasound confirmed the presence of uterus and both the ovaries. There was a 10 cm × 10 cm endometriotic cyst in left ovary. Both the kidneys were normal. She was duly counseled and taken up for laparoscopic evaluation. Examination under anesthesia confirmed absent vagina. On inserting the laparoscope, a big endometriotic cyst in left ovary was visualized. Right tube and ovary were normal, and uterus was small in size. The endometriotic cyst was enucleated and base fulgurated. Neovagina was created laparoscopically by our modification of Vecchietti's technique. The patient was discharged but unfortunately, was lost to follow up. A year later, she again presented to us with pain abdomen and another endometriotic cyst in the left ovary. She was taken up for a re-look laparoscopy in an attempt to create a passage-way for menstruation. We had good experience in this as we had successfully created such a menstrual passage-way in the past in two of our patients with fragmented cervix. The tentative plan for this was to establish the lower limit of uterus and upper limit of neovagina and henceforth, suture them together. Uterus and bladder were mobilized. A longitudinal incision was given over fundus of the uterus with harmonic scalpel and a suction cannula was passed through this incision till the lower limit of uterus. Similarly, upper limit of the neovagina was ascertained using a sound. However, utero-vaginal anastomosis could not be performed, because the intervening distance in this patient was so much that mobilization of the uterus was not technically possible and the procedure had to be abandoned. Hysterectomy was suggested as an alternative but the relatives refused. The endometriotic cyst was drained, cyst wall removed, and the base fulgurated.

Later, this patient got married and presented to us again after 5 years of initial treatment with left lower abdominal pain. She reported satisfactory coital function and her husband had two issues from his previous marriage. Sonographic evaluation showed another endometriotic cyst (11 cm × 8 cm) in left ovary. The case was discussed with the patient and her family and prognosis regarding reproductive potential was explained. They decided in favor of hysterectomy and the patient was taken up for laparoscopy again. On inserting the laparoscope, a pelvic mass was visualized which was formed by a jumble of dilated left tube, left ovarian cyst with left ovary, and loops of small bowel which were all matted together [Figure 1] and [Figure 2]. Careful adhesiolysis was done. The cyst drained chocolate material during adhesiolysis and was enucleated and removed. Multiple peritoneal jellies were present which were also punctured. After adhesiolysis, uterus could be visualized beneath this mass. Anatomy was reassessed and ureter was catheterized on left side to delineate its course. A hysterectomy was planned so round ligament and infundibulopelvic were cut with harmonic on the left side. Right tube and ovary were found to be normal in appearance. Hence they were retained. Carefully, upper limit of bladder was determined with the help of a bougie inside the bladder. Uterovesical fold of peritoneum was incised and bladder pushed down. Uterine arteries could not be seen on either side even after a conscious attempt and dissection and the uterus seemed to be deriving its entire blood supply from the ovarian and tubal vessels. Uterus with left tube and ovary were separated from the underlying loose peritoneal fold [Figure 3] with the help of harmonic which completed the hysterectomy then the uterus was morcellated and removed [Figure 4] and [Figure 5]. Cystoscopy was done after the procedure which revealed an ectopic position of left ureteric opening.
Figure 1: Dense adhesion of omentum covering the entire uterus, left tube and ovary


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Figure 2: Totally jumbled mass of dilated left tube and ovarian cyst


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Figure 3: Loose fold of peritoneum replacing the cervix


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Figure 4: Uterus being removed


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Figure 5: Final picture after hysterectomy with left salpingo-oopherectomy


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   Discussion Top


Congenital cervical anomalies are classified as type IB in the American Fertility Society Classification of mullerian anomalies. [1] They can further be classified into complete agenesis and dysgenesis where, Congenital agenesis of the cervix is a rare condition. The reported incidence is 1 in 80,000-100,000 births. [2] It is absence of a portion or the whole of cervix. Vaginal atresia and renal tract anomalies are commonly associated. Hence, thorough pre-operative work-up including an intravenous urograph is mandatory. The patient usually presents with primary amenorrhea, and cyclical lower abdominal pain. Diagnosis is suspected on the basis of history, clinical examination, and ultrasonography and can be confirmed by means of magnetic resonance imaging. Differentiation from a high vaginal septum can be made as there is no vaginal dilatation with the accumulation of blood due to an absent passage-way. Previously, the recommended treatment for cervical agenesis was hysterectomy. [3],[4] Conservative laparoscopic surgery consisting of uterovaginal anastomosis has also been described and recommended as first line treatment by some authors. [5] In our own experience, conservative management is a better treatment option in cases of cervical fragmentation and dysgenesis. Our purpose of reporting, this case is to highlight the need to individualize treatment in all such patients of cervical anomalies. While, we also recommend uterovaginal anastomosis as the first line of management, both surgeon and the patient should be prepared for hysterectomy, especially, in extreme cases like this. This will save repeated morbid procedures as well as financial and psychological burdens as correct decisions help provide timely management to the patient.

 
   References Top

1.American Fertility Society classification of mullerian anomalies. Fertil Steril 1988;49:952.  Back to cited text no. 1
    
2.Suganuma N, Furuhashi M, Moriwaki T, Tsukahara Si, Ando T, Ishihara Y. Management of missed abortion in a patient with congenital cervical atresia. Fertil Steril 2002;77:1071-3.  Back to cited text no. 2
[PUBMED]    
3.Deffarges JV, Haddad B, Musset R, Paniel BJ. Utero-vaginal anastomosis in women with uterine cervix atresia: Long-term follow-up and reproductive performance. A study of 18 cases. Hum Reprod 2001;16:1722-5.  Back to cited text no. 3
[PUBMED]    
4.Rock JA, Schlaff WD, Zacur HA, Jones HW Jr. The clinical management of congenital absence of the uterine cervix. Int J Gynaecol Obstet 1984;22:231-5.  Back to cited text no. 4
[PUBMED]    
5.Creighton SM, Davies MC, Cutner A. Laparoscopic management of cervical agenesis. Fertil Steril 2006;85:1510.e13-5.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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