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Table of Contents
Year : 2021  |  Volume : 10  |  Issue : 4  |  Page : 247-251

The path of birth is not always normal: A case report of cervicovaginal agenesis

Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Date of Submission02-Jun-2020
Date of Decision10-Sep-2020
Date of Acceptance09-Dec-2020
Date of Web Publication5-Nov-2021

Correspondence Address:
Dr. Kavita Khoiwal
Department of Obstetrics and Gynaecology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/GMIT.GMIT_57_20

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Cervical agenesis is a rare congenital Mullerian anomaly. Its association with vaginal agenesis is further rare, reported in 39% of cases of cervical agenesis. The conventional treatment for this condition was hysterectomy. However, with evolving surgical skills and assisted reproductive techniques, conservative surgery could be considered as the first-line treatment in the current era. We report one such case of cervicovaginal agenesis in a 13-year-old adolescent girl managed successfully with cervicovaginoplasty. The patient was relieved of cyclical pain abdomen and resumed menstrual cycles postoperatively.

Keywords: Cervicovaginal agenesis, cervicovaginoplasty, cyclical pain abdomen, primary amenorrhea

How to cite this article:
Khoiwal K, Singh M, Agarwal A, Chaturvedi J. The path of birth is not always normal: A case report of cervicovaginal agenesis. Gynecol Minim Invasive Ther 2021;10:247-51

How to cite this URL:
Khoiwal K, Singh M, Agarwal A, Chaturvedi J. The path of birth is not always normal: A case report of cervicovaginal agenesis. Gynecol Minim Invasive Ther [serial online] 2021 [cited 2022 Aug 8];10:247-51. Available from: https://www.e-gmit.com/text.asp?2021/10/4/247/329873

  Introduction Top

In adults, Mullerian duct forms uterus, cervix,  Fallopian tube More Details, and upper one-third of the vagina. Its anomaly has a mean prevalence of 4.3% of general population.[1] In 2013, European Society of Human Reproduction and Embryology (ESHRE) has proposed a newer classification system for Mullerian anomalies on the basis of uterine anatomy. In addition, anomalies of the cervix and vagina are classified separately.[2] In the ESHRE classification, cervical and vaginal agenesis are classified as C4 and V4, respectively.

Cervical agenesis is a rare congenital Mullerian anomaly, with an incidence of 1:80,000–1:100,000.[3] Its association with vaginal agenesis is further rare, reported in 39% of cases of cervical agenesis.[4]

Cervical or cervicovaginal agenesis usually presents around the age of menarche with complaints of primary amenorrhea and/or cyclical abdominal pain. In these cases, endometrium functions normally, but menstrual blood has no path to exit from vagina, leading to hematocolpos followed by hematometra formation. The condition can further aggravated with hematosalpinx and endometriosis if left undiagnosed. Diagnosis and management should be done early in these cases to avoid progression of the disease. We present a case of 13-year-old female with cervicovaginal agenesis, who was managed by conservative surgery.

  Case Report Top

A 13-year-old girl presented in Gynecology Emergency Department with the complaint of severe abdominal pain for 1 day not relieving on injectable analgesics. She had a history of primary amenorrhea with cyclical abdominal pain for the last 1 year. There was no significant past, personal, or family history. There was no history of in utero exposure to diethylstilbestrol.

General physical examination revealed average built with normal secondary sexual characters (breast - tanner stage 4, pubic hairs - tanner stage 4). Thyroid and breast examination was normal. Abdomen was soft, no organomegaly was noted, while tenderness present in the lower abdomen on deep palpation. A gentle vaginal examination revealed a blind vaginal pouch (2 cm × 1 cm) and no cervical tissue felt. She was carrying ultrasound (USG) and magnetic resonance imaging (MRI) reports with her. USG pelvis showed collection in the endometrial cavity along with hematocolpos. MRI pelvis revealed uterine enlargement (8.9 cm × 6 cm × 4.2 cm) with collection in the endometrial cavity, suggestive of hematometra along with hematocolpos. Bilateral ovaries and renal system were normal. On the basis of imaging findings, a provisional diagnosis of low transverse vaginal septum was made.

In view of intractable pain, the patient was taken to the emergency operation theater for vaginal septum excision followed by hematocolpos and hematometra drainage. Consent was taken for examination under anesthesia (EUA) with transverse vaginal septum excision with laparotomy if required. EUA revealed a blind vaginal pouch (2 cm × 1 cm) [Figure 1], and surgical excision of the vaginal septum was attempted; however, soon, we realized that there was no vaginal cavity as well as no cervical tissue, making it a case of cervicovaginal agenesis (U0C4V4). A decision of cervicovaginoplasty was made by abdominoperineal approach. Informed and written consent of abdominoperineal cervicovaginoplasty was taken. An approximately 6 cm × 3 cm neovagina was created by dissecting in between bladder and rectum by McIndoe technique. After that, suprapubic transverse minilaparotomy was performed. Uterus was enlarged up to 8 weeks size with left-sided hematosalpinx, while right tube and bilateral ovaries were healthy. Endometriotic spots were present on the surface of uterus, ovaries, and pouch of Douglas. A stab incision was made over anterior wall of the uterus; 80–100 ml of chocolate-colored fluid was drained from the endometrial cavity [Figure 2]a. A long curved artery forceps was then introduced through the cavity toward neovagina [Figure 2]b, and an incision was given from below vaginally to create neocervix. A silicone Malecot catheter No. 18 was inserted in the uterine cavity through neocervix by rail-road technique to keep neocervix patent. Uterine wall was sutured with delayed absorbable suture. Foam mold (6 cm × 3 cm) encircling Malecot catheter [Figure 2]c was kept in the neovagina [Figure 2]d to avoid re-approximation of vaginal walls. Her postoperative course was uneventful. On the 7th postoperative day, foam mold was changed to glass mold and Malecot catheter was kept in situ, 1–2 cm outside the cervical os. Mold care was explained to the patient and her mother on discharge. She had three follow-up visits till now at 1, 3, and 6 months postoperatively. The patient had uneventful postoperative period and has resumed normal menstrual cycles. Consent for publication was obtained from the patient and her parents and institutional ethical board (AIIMS/IEC/20/338).
Figure 1: Examination under anesthesia revealed a blind vaginal pouch

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Figure 2: (a) Drainage of chocolate-colored fluid from endometrial cavity, (b) a long curved artery forceps was introduced through the cavity toward neovagina. (c) Foam mold (6 cm × 3 cm) encircling silicone Malecot catheter. (d) Foam mold being inserted in the neovagina with silicone Malecot catheter in the neocervix and endometrial cavity

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  Discussion Top

Cervicovaginal agenesis is a rare congenital anomaly of the female genital tract. Only few scattered cases and case series are reported in the literature[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18] [Table 1].
Table 1: Previous reported cases of cervicovaginal agenesis

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Most common presenting complaint is primary amenorrhea with cyclical abdominal pain and/or chronic pelvic pain, palpable mass per abdomen, and urinary complaints. Differential diagnosis includes imperforate hymen, vaginal agenesis, or transverse vaginal septum, which should be excluded before definitive management. Although clinical history and examination are crucial in such cases, pelvic imaging should be considered to know the exact anatomical anomaly. MRI is considered as the gold standard for diagnosis as well as to look for associated renal tract anomalies.[5] In our case, imaging was not really helpful as it suggested a diagnosis of transverse vaginal septum while there were no vaginal cavity and hematocolpos present during surgery.

Surgical treatment for imperforate hymen and transverse vaginal septum is easy and has minimal complications. However, creation of the neovagina and neocervix requires more complex operations and is associated with high morbidity and limited success; many of these patients ultimately require hysterectomy. In the literature, many authors[6],[7] have concluded that the treatment of choice should be hysterectomy, and psychological support as reconstructive surgery has serious complication as sepsis, endometriosis, and need for multiple surgeries due to restenosis. Moreover, the patient has low possibility of spontaneous conception due to endometriosis. On the contrary, as surgical skills and assisted reproductive technique are evolving over the time, conservative surgery could be considered as the first-line treatment.[9],[10],[11],[12],[14],[16],[17],[18] Patient's femininity and a distortion of her physical image which can affect her self-esteem should be kept in mind. In our case, we preferred conservative surgery over hysterectomy with due consent from the patient and her parents. At the same time, we also emphasize that the management option should be individualized and possibility of hysterectomy should always be considered particularly in extreme cases.[8],[13],[15] Minimally invasive approach is preferred whenever feasible.

Regular follow-up with clinical examination and USG pelvis is required. Patency of track is determined by occurrence of regular menstrual flow without recurrence of hematometra.

Cervicovaginal agenesis is to be kept in mind whenever a young girl presents with primary amenorrhea and cyclical abdominal pain. Early diagnosis and management are crucial for successful outcome. Exact delineation of pelvic anatomy on imaging is must to avoid complications. First-line surgical option is cervicovaginoplasty, but management has to be individualized in each patient with keeping possibility of need of hysterectomy. Psychological counseling and long-term follow-up are essential.

Ethical review

This report has been approved by Institutional Ethics Committee of All India Institute of Medical Sciences, Rishikesh (approval no. AIIMS/IEC/20/338).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parent has given consent for images and other clinical information to be reported in the journal. The patient's parent understands that the names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Grimbizis GF, Camus M, Tarlatzis BC, Bontis JN, Devroey P. Clinical implications of uterine malformations and hysteroscopic treatment results. Hum Reprod Update 2001;7:161-74.  Back to cited text no. 1
Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M, et al. The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies. Hum Reprod 2013;28:2032-44.  Back to cited text no. 2
Suganuma N, Furuhashi M, Moriwaki T, Tsukahara Si, Ando T, Ishihara Y, et al. Management of missed abortion in a patient with congenital cervical atresia. Fertil Steril 2002;77:1071-3.  Back to cited text no. 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. 4
Rock JA, Roberts CP, Jones HW Jr. Congenital anomalies of the uterine cervix: Lessons from 30 cases managed clinically by a common protocol. Fertil Steril 2010;94:1858-63.  Back to cited text no. 5
Rock JA, Schlaff WD, Zacur HA, Jones HW Jr. The clinical management of the congenital absence of the uterine cervix. Int J Gynaecol Obstet 1984;22:231-5.  Back to cited text no. 6
Hampton HL. Role of the gynaecologic surgeon in the management of urogenital anomalies in adolescents. Curr Opin Obstet Gynaecol 1990;2:812-8.  Back to cited text no. 7
Meena J, Bharti J, Roy KK, Kumar S, Singhal S, Shekhar B, et al. Bicornuate uterus with complete cervico-vaginal agenesis and skeletal deformity: A Case report. J Obstet Gynaecol India 2019;69:67-70.  Back to cited text no. 8
Bagga R, Muthyala T, Saha PK, Kalra J, Singla R, Arora A, et al. Functioning left uterine horn with cervico-vaginal atresia and ovarian maldescent – An unclassified Müllerian anomaly treated with horn-vaginal anastomosis. J Obstet Gynaecol 2018;38:1176-8.  Back to cited text no. 9
Mishra V, Saini SR, Nanda S, Choudhary S, Roy P, Singh T, et al. Uterine conserving surgery in a case of cervicovaginal agenesis with unicornuate uterus. J Hum Reprod Sci 2016;9:267-70.  Back to cited text no. 10
[PUBMED]  [Full text]  
Jeon GH, Kim SH, Chae HD, Kim CH, Kang BM. Simple uterovaginal anastomosis for cervicovaginal atresia diagnosed by magnetic resonance imaging: A report of two cases. J Obstet Gynaecol Res 2016;42:738-42.  Back to cited text no. 11
Kisku S, Varghese L, Kekre A, Sen S, Karl S, Mathai J, et al. Cervicovaginal atresia with hematometra: Restoring menstrual and sexual function by utero-coloneovaginoplasty. Pediatr Surg Int 2014;30:1051-60.  Back to cited text no. 12
Gasim T, Al Jama FE. Massive hematometra due to congenital cervicovaginal agenesis in an adolescent girl treated by hysterectomy: A Case report. Case Rep Obstet Gynecol 2013;2013:640214.  Back to cited text no. 13
Kriplani A, Kachhawa G, Awasthi D, Kulshrestha V. Laparoscopic-assisted uterovaginal anastomosis in congenital atresia of uterine cervix: Follow-up study. J Minim Invasive Gynecol 2012;19:477-84.  Back to cited text no. 14
Jain N, Sircar R. Laparoscopic management of congenital cervico-vaginal agenesis. J Gynecol Endosc Surg 2011;2:94-6.  Back to cited text no. 15
Raudrant D, Chalouhi G, Dubuisson J, Golfier F. Laparoscopic uterovaginal anastomosis in Mayer-Rokitansky-Küster-Hauser syndrome with functioning horn. Fertil Steril 2008;90:2416-8.  Back to cited text no. 16
Gurbuz A, Karateke A, Haliloglu B. Abdominal surgical approach to a case of complete cervical and partial vaginal agenesis. Fertil Steril 2005;84:217.  Back to cited text no. 17
Grimbizis GF, Tsalikis T, Mikos T, Papadopoulos N, Tarlatzis BC, Bontis JN. Successful end-to-end cervico-cervical anastomosis in a patient with congenital cervical fragmentation: Case report. Hum Reprod 2004;19:1204-10.  Back to cited text no. 18


  [Figure 1], [Figure 2]

  [Table 1]


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