www.slcog.lk/sljog Case Report

Cervical Agenesis: Two Case Reports with Review of Literature Nayana Pathak1, Praveen Mohan2

left side salpingo-oophorectomy, Abstract excision of right ovarian cyst and left sided ureteric stenting was done. Cervical agenesis is an extremely rare congenital Mullerian duct anomaly of female genital Intraoperatively, 50-100cc dark tract. It is a complex surgical problem and management is still controversial. Failure to altered blood was seen in pouch of manage these patients correctly may be associated with long-term psychological, sexual, Douglas with endometriotic implants reproductive health damage and suffering. We have managed two cases of cervical agenesis over , adenexae, POD, bladder with . and rectum. of 8-10 wks size with , left sided , endometriotic cyst of characters were well developed. On Introduction left ovary, right sided hydrosalpingx per abdominal examination, a six True incidence of cervical agenesis and simple cyst of right ovary was into six cm mass was felt arising from is unknown but review of literature seen. (Fig1) the pelvis, more on the left side with list less than 200 cases since 1942 evidence of guarding and rigidity. She (1).According to American Fertility was investigated thoroughly and all Society Cervical agenesis is classified Case 2 routine investigations were normal. On as type IB mullerian anomaly(2). Intravenous pyelography, left sided A fifteen years old girl presented These defects are congenital, but hydroureter with hydronephrosis was with history of primary , usually present symptoms at the time detected. Ultrasonography showed a cyclical pain abdomen and gradually of puberty. Patients have normal bicornuate uterus with hematometra increasing mass per abdomen for secondary sexual development and left sided hematosalpinx.When the last six months. She had history and presentations are like primary examined under anesthesia, a blind of laparoscopy one year back for amenorrhea, cyclical lower abdominal ending of 3cm length and acute pain abdomen and extensive pain, mass per abdomen and history cervical agenesis was detected. was diagnosed. After of recurrent surgeries. As patient belonged to a poor proper examination and investigations, socioeconomic group and wanted a she diagnosed as a case of cervical permanent cure from the problem, agenesis with . Case 1 exploratory laparotomy followed by Exploratory laparotomy followed by An eighteen yrs old, unmarried girl total abdominal with total abdominal hysterectomy with presented to Gynae outdoor Deptt. With history of severe abdominal pain for last twenty days, secondary amenorrhea for three yrs and cyclical pain abdomen for the last six months. She underwent exploratory laparotomy, uterovaginal anastomosis with in some other hospital at the age of thirteen yrs for cryptomenorrhea and had regular menstruation for six months following surgery. Her secondary sexual

1 Associate Professor 2 Professor and Head Dept. of Obstetrics & Gynecology, MMIMSR, Mullana, Ambala, India.

Correspondence: Dr. Nayana Pathak E-mail: [email protected] Competing interests: None Figure 1: Uterus with left sided Tubo-ovarian mass & right sided hydrosalpingx with cystic ovary

March 2014 Sri Lanka Journal of Obstetrics and 19 Case Report www.slcog.lk/sljog bilateral salpingo-oophorectomy was done due to extensive endometriosis with complete cervical agenesis. Intraoperatively sixteen wks size enlarged uterus with bilateral and hematosalpinx forming tubo-ovarian masses with extensive was seen. (Fig 2). Postoperative period was uneventful in both patients and cervical agenesis was confirmed histopathlogically.

Discussion Congenital cervical atresia is a recognized clinical entity, but there is lack of uniformity in the literature regarding its classification and management. Embryo logically, female reproductive tract develops from paired mullerian duct. Its complete Figure 2: Cut section of uterus formation and differentiation depends upon three phases of development- organogenesis, fusion (lateral as well repeated surgeries and even death brief discussion regarding risk and as vertical) and septal resorption. has been reported in the literature morbidity associated with different Cervical atresia is considered as defect following reconstructive surgeries. surgical modalities and patient in the elongation of Mullerian Duct. In Rock (1) etal series, success preference must be used for making At about 20wks of gestation, of reconstructive surgeries was treatment strategy, and then we can forms as a condensation of stromal documented in patients with cervical obtain a good surgical outcome. ■ cell at a specific site around the fused dysgenesis and all fourteen patients Mullerian Duct and its differentiation of cervical agenesis underwent is a complex process involves both hysterectomy. In all variants of cervical References mesodermal and endodermal dysgenesis, successful outcome was tissue(3).The management of cervical obtained in only cervical obstruction 1. Rock JA, Roberts CP, Jones HWJ. anomalies is still controversial. Past group. In spite of documented success Congenital anomalies of the uterine and present articles are descriptive of reconstructive surgeries in the cervix: lessons from 30 cases managed and difficult to assess and compare literature, it has challenges. Several clinically by a common protocol. Fertil due to difference in sample size and factors may influence the surgical Steril 2010; 34: 1858-1863. variation in surgical techniques. outcome: the size of created channel, 2. American Fertility Society (1988). The Grimbizis (4) etal published the the duration of stenting of the American Fertility Society classifications success of end to end cervico-cervical channel, the presence of rudimentary of Adnexal adhesions, distal tubal anastomosis in 116 cases of transverse endocervical glands in the range of occlusion, tubal occlusion secondary cervical defect whereas Rober(5) created channel, the presence of native to tubal ligation, tubal pregnancies, etal have outlined the importance of vagina adjacent to created channel Mullerian anomalies and intrauterine cervical anatomy. In the literature and the number of menses allowed adhesions. Fertil Steril 49,944-955. role of conservative surgeries and to flow through the stented channel. successful pregnancy outcome Because the surgical approach for 3. UlfelderH, RobboyS. The embryologic have been reported with the help cervical cannulation is based only on development of the human vagina. Am J of assisted reproductive techniques several case reports, limited data are Obstet Gynecol 1976;126:766-769. but obstetrical complications have available regarding safety and efficacy 4. Grimbiziz GF, Tsalikis T, Mikos T, Et al. not been fully reported. In 50% cases to share with patients and relatives. A Successful end to end cervico-cervical cervical agenesis is associated with frank discussion with patient and her anastomosis in a patient with congenital vaginal atresia and it is difficult to family regarding risk and morbidity cervical fragmentation: case report. Hum make a satisfactory fistulous tract in of reconstructive surgeries is essential. Reprod 2004;5:1204-1210. these types of cases. Complications In our opinion, reconstructive 5. Roberts CP, Rock JA. Surgical methods like endometritis, Pelvic inflammatory surgeries should be considered in in the treatment of congenital anomalies disease, persistent pelvic pain, bowel selected patients and hysterectomy of the uterine cervix. Current Opinion and bladder injury, reobstruction, should always be offered as an option. in Obstetrics and Gynaecology 2011; recurrent and severe pelvic infection, Proper anatomical assessment, 23:251-257.

20 Sri Lanka Journal of Obstetrics and Gynaecology March 2014