Table of Contents  
Year : 2016  |  Volume : 9  |  Issue : 2  |  Page : 237-240  

A rare case of primary amenorrhea

1 Department of Obstetrics and Gynaecology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India
2 Department of Surgery, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India

Date of Web Publication1-Mar-2016

Correspondence Address:
Himadri Bal
Department of Obstetrics and Gynaecology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-2870.177674

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Crytomenorrhea arises usually due to the imperforate hymen, sometimes due to vaginal septum, rarely due to cervical agenesis. Here, we present a case of primary amenorrhea resulting from cervical agenesis in a 38-year-old woman. She presented with primary amenorrhea and cyclic lower abdominal pain. She had undergone some surgery 5 years back, details of which were not available. On examination secondary sexual characters were normal, per abdomen there was an 18 weeks size firm abdominopelvic mass. Local vaginal examination showed a blind vaginal pouch. A clinical diagnosis of hematometra due to transverse vaginal septum was made. However, magnetic resonance imaging pelvis suggested hematometra with cervical stenosis. The patient was taken up for examination under anaesthesia (EUA) and exploratory laparotomy. On opening the abdomen uterus found to be enlarged with dense adhesions all around and signs of endometriosis. Extensive adhesiolysis revealed bilateral chocolate cysts of ovaries with hematosalpinges and peritubal adhesions. Hysterotomy and drainage of tarry contents were followed by an exploration of the uterine cavity. The lower pole ended blindly with no evidence of any cervix. Peroperative diagnosis of cervical agenesis leading to hematometra and endometriosis was made. A subtotal hysterectomy with bilateral salpingo-oophorectomy was done. Histopathological examination confirmed ovarian endometriosis and adenomyosis of uterus. Though reconstructive surgery for cervical dysgenesis has been successful in some cases, hysterectomy is generally recommended for cervical agenesis.

Keywords: Cervical agenesis, cervical dysgenesis, cervicovaginal anastomosis, cryptomenorrhea

How to cite this article:
Bal H, Sharma S, Ali I, Ashtekar A. A rare case of primary amenorrhea. Med J DY Patil Univ 2016;9:237-40

How to cite this URL:
Bal H, Sharma S, Ali I, Ashtekar A. A rare case of primary amenorrhea. Med J DY Patil Univ [serial online] 2016 [cited 2023 Sep 30];9:237-40. Available from:

  Introduction Top

Primary amenorrhea resulting from outflow tract obstruction of genitalia may be due to various causes. The most common reason is an imperforate hymen; next is transverse vaginal septum and rarely cervical agenesis. [1]

The incidence of cervical agenesis is around 1 in 80,000 to 100,000 births, [2] and <50 cases have been reported in world literature. [3]

Patients with this developmental anomaly usually present early because of cyclic abdominal pain every month. We came across such a case that presented to us in mid-reproductive age, and on evaluation turned out to be a case of cervical agenesis with associated complications.

  Case Report Top

A 38-year-old patient reported with a history of cyclic lower abdominal pain every month from the age of 13 years which lasted for 2-3 days. She had not started menstruating and visited a doctor who said menses will start after marriage when coitus is established! She was married at the age of 28 years, did not achieve menses and obviously did not conceive; although she had no coital problem. She kept on having cyclical pain abdomen and sought consultation 6 years postmarriage for amenorrhea and infertility. She underwent a laparotomy thereafter, i.e., 4 years back, documents of which were not available with the patient. She reported to our outpatient department (OPD) with severe pain abdomen off and on for last 1 year.

On examination, she was found to have normal secondary sexual characters and no thyromegaly. Per abdomen, she had a firm to hard 18 weeks size irregular abdominopelvic mass with restricted mobility. Genital examination revealed normal external genitalia and a blind vaginal pouch of 5 cm depth [Figure 1]. Per vaginal and per rectal examination confirmed the abdominopelvic mass. Hence, a provisional clinical diagnosis of cryptomenorrhea due to transverse vaginal septum and leading to hematometrocolpos was made.
Figure 1: Blind vagina

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Her blood count, urine, and biochemical parameters were within normal limits. Sonography showed hematometrocolpos (9.4 cm × 6.1 cm × 6 cm), bulky right ovary with a simple cyst (4.5 cm × 3.9 cm), left ovary hemorrhagic cyst ( 3.3 cm × 2.8 cm). At this stage, the plan was to excise the vaginal septum and establish continuity of genital outflow tract. However, magnetic resonance imaging (MRI) done subsequently showed evidence of hematometra with cervical stenosis, bilateral hematosalpinges, and right ovarian cyst. Hematometra was ending blindly with no communication with the vagina [Figure 2].
Figure 2: Magnetic resonance imaging images showing hematometra

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The plan was changed to laparotomy, drainage of hematometra and try and establish communication from above by channelizing the stenosis. Informed consent was taken, where fertility issues were also discussed. The patient pleaded to be relieved of her unbearable pain even at the cost of fertility.

On laparotomy, it was found that the uterus was enlarged with extensive dense intestinal adhesions all around the uterus including the sigmoid colon and also bladder. There were features of widespread pelvic endometriosis. Extensive adhesiolysis revealed bilateral chocolate cysts of ovaries, dilated  Fallopian tube More Detailss with gross peritubal adhesions. Hysterotomy was done and tarry fluid was drained [Figure 3].
Figure 3: Intraoperative drainage of hematometra

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Exploration of uterine cavity showed uterus ending blindly without any opening resembling the internal os and no tissue resembling cervix. Peroperative diagnosis of cervical agenesis leading to hematometra and endometriosis was confirmed. A supracervical hysterectomy with bilateral salpingo-oophorectomy was carried out in view of the extensive endometriosis and adhesions. There were severe adhesions between the bladder and lower portion of uterus and vagina. Thus to avoid any injury to bladder a small isthmic portion of the uterus was left behind. The patient had an uneventful recovery and was totally asymptomatic on her follow-up visit.

Histopathological examination confirmed ovarian endometriosis and uterus showed evidence of adenomyosis.

  Discussion Top

Cryptomenorrhea is a condition where menstruation occurs but is not visible due to obstruction of the outflow tract. Cryptomenorrhea due to imperforate hymen and transverse vaginal septum are well known but cervical agenesis though mentioned in literature is a rarity. Cervical agenesis or dysgenesis falls into category II B of the American Fertility Society classification of uterovaginal anomalies. [4]

Two basic categories of cervical anomalies have been observed in several configurations. [5] Patients exhibiting the first type cervical aplasia or agenesis, lack a uterine cervix [Figure 4]A.
Figure 4: (A) Cervical agenesis, (B) cervical body is a fibrous band, (C) obstructed os, (D) midportion of cervix hypoplastic with bulbous tip, (E) fragmented cervix

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The second type, cervical dysgenesis has been subdivided into four distinct types as under:

  1. The cervical body consists of a fibrous band of variable length and diameter that contain endocervical glands [Figure 4]B.
  2. The cervical body is intact with obstruction of cervical os [Figure 4C].
  3. Stricture of midportion of cervix which is hypoplastic with a bulbous tip [Figure 4D].
  4. Cervical fragmentation in which portion of the cervix are noted with no connection to the uterine body [Figure 4]E.

Cervical agenesis is a rare mullerian anomaly. This anomaly is often associated with the partial or complete absence of the vagina. The patient complains of cyclic lower abdominal pain due to retention of menstrual blood. With cervical agenesis, there is no vaginal dilatation with accumulation of blood as seen with a transverse vaginal septum. Diagnosis before surgery is difficult but with the advent of modern diagnostic tools like MRI; preoperative diagnosis is possible.

The primary aim of treatment of cervical agenesis should be re-establishment of patency of outflow tract. However, it is difficult to obtain a fistulous tract through which menstrual blood can flow in cervical agenesis. Many methods have been tried including placement of a stent to keep the tract open. Eventually, the uterovaginal tract closes from constriction by fibrous tissue. Occasional success in maintaining an open pathway and normal cyclic menstruation have been reported, but endocervical glands do not develop, and there is no way to compensate for the absence of the cervical mucus, which plays an important role in sperm transport. Even though cyclic ovulatory periods can be achieved in a few patients, pregnancy is unlikely. Endometriosis can develop in ovaries and other pelvic tissues because of retrograde menstruation. Recurrent and severe pelvic infection is a common problem and may subsequently require a hysterectomy. If the hysterectomy is performed soon enough before the problems become magnified, it may be possible to conserve the ovaries and their useful functions. Hysterectomy done in the first place eliminates much needless suffering. Similarly, in cases with the fragmented cervix, hysterectomy is usually warranted.

However, recent reports in literature have suggested that, in the absence of the cervix, surgical uterovaginal communication may give a chance to the patient to conceive with the help of advanced reproductive technologies. [6] A cerclage may help following uterovaginal anastomosis to sustain the pregnancy. Most patients have reported delivery by cesarean section. [7]

Despite the overall poor results of reconstruction of dysgenetic cervices, canalization procedures can be worthwhile for a few carefully selected cases with adequate stroma to allow a cervicovaginal anastomosis. Data published by Rock et al. reports that only those with well-formed cervical bodies or those having only distal obstruction have been successful in achieving a pregnancy following surgery.

There are no carefully designed studies or trials to support best surgical practice in the treatment of cervical dysgenesis. Various authors have recommended different surgical alternatives for patients with cervical dysgenesis based upon the specific cervical anatomy. [8]

Uterovaginal anastomosis can lead to complications which are sometimes life-threatening. These include recurrent endometritis, pelvic inflammatory disease, bowel and bladder injury, re-stenosis requiring repeat surgery. [5]

  Conclusion Top

Cervical agenesis/dysgenesis is a rare cause of cryptomenorrhea. Once diagnosed it should be treated at the earliest to prevent further complications mainly in the form of endometriosis due to retrograde menstruation. The type of abnormality and patient preference should be taken into account while planning corrective surgery. Hysterectomy is the preferred treatment for cervical agenesis. However, with the advancement of assisted reproductive technologies conservative management strategies are being explored.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Rock JA, Jones HW 3 rd . Te Linde′s Operative Gynecology. 10 th ed. Wolters Kluwer India: Lippincott Williams & Wilkins; South Asian edition; 2008. p. 560-5.  Back to cited text no. 1
Creighton SM, Davies MC, Cutner A. Laparoscopic management of cervical agenesis. Fertil Steril 2006;85:1510.e13-5.  Back to cited text no. 2
Rock JA, Carpenter SE, Wheeless CR, Jones HW. The clinical management of maldevelopment of the uterine cervix. J Pelvic Surg 1995;1:129-33.  Back to cited text no. 3
American Fertility Society classification of mullerian anomalies. Fertil Steril 1988;49:952-3.  Back to cited text no. 4
Roberts CP, Rock JA. Surgical methods in the treatment of congenital anomalies of the uterine cervix. Curr Opin Obstet Gynecol 2011;23:251-7.  Back to cited text no. 5
Lai TH, Wu MH, Hung KH, Cheng YC, Chang FM. Successful pregnancy by transmyometrial and transtubal embryo transfer after IVF in a patient with congenital cervical atresia who underwent uterovaginal canalization during caesarean section. Hum Reprod 2001;16:268-71.  Back to cited text no. 6
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. 7
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. 8


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


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