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Cervical and Vaginal Agenesis: a Novel Anomaly

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Cervical and Vaginal Agenesis: a Novel Anomaly Cervical and Vaginal Agenesis: A Novel Anomaly Case Report Cervical and Vaginal Agenesis: A Novel Anomaly Irum Sohail1, Maria Habib 2 1Professor Obs/Gynae, 2Postgraduate Trainee, Wah Medical College, Wah Cantt Address of Correspondence: Dr. Maria Habib, Postgraduate Trainee, Wah Medical College, Wah Cantt Email: [email protected] Abstract Background: Cervical agenesis with vaginal agenesis is an extremely rare congenital anomaly. This mullerian anomaly occurs in 1 in 80,000-100,000 births. It is classified as type IB in the American Fertility Society Classification of mullerian anomalies. Case report: We report a case presented to POF Hospital, Wah cantt with primary amenorrhea and cyclic lower abdominal pain. She was diagnosed to have cervical agenesis associated with completely absent vagina. Conservative surgical approach to these patients involve uterovaginal anastomosis and cervical reconstruction. Creation of neovagina is necessary in these cases. Due to high failure rate and potential for complications, total hysterectomy with vaginoplasty is the treatment of choice by many authors. Conclusion: A thorough investigation of the patients with primary amenorrhea is necessary and total hysterectomy with vaginoplasty is feasible and should be considered as a first-line treatment option in cases of cervical and vaginal agenesis. Key words: Primary amenorrhea, Cervical agenesis, Vaginal agenesis, Hysterectomy. Introduction The female reproductive organs develop from the Society of Human Reproduction and Embryology fusion of the bilateral paramesonephric (Müllerian) (ESHRE)/European Society for Gynaecological ducts to form the uterus, cervix, and upper two- Endoscopy (ESGE) classification system of female thirds of the vagina.1 The lower third of the vagina genital anomalies is designed for clinical orientation develops from the sinovaginal bulbs of the and it is based on the anatomy of the female urogenital sinus.2 Mullerian duct anomalies (MDAs) genital tract. The cases which could not be are congenital defects of the female genital tract previously classified according to American Society that arise from abnormal embryological development of Reproductive Medicine system can now easily of the Mullerian ducts. These include failure of be classified according to this new system.6 The development, fusion, canalization, or reabsorption reported incidence of cervical agenesis is 1 in which normally occurs between 6 and 22 weeks in 80,000-100,000 births.7 When it co-exists with utero.3 vaginal agenesis/aplasia, it is even rarer. A case The prevalence of Müllerian hypoplasia/agenesis of a young female with cervical agenesis, (type I anomalies) seems to be uncommon, associated with a functioning uterine corpus and representing 3% of uterine malformations or an absent vagina is reported. 0.1% of the general population.4 Congenital cervical anomalies∼ are classified as type IB in the Case Report ∼ American Society of Reproductive Medicine system A 18 year old, unmarried, girl presented to outpatient (formerly called American Fertility Society gynaecologic clinic with history of primary amenorrhea Classification of mullerian anomalies). They can and cyclic lower abdominal pain. This was an unusually further be classified into complete agenesis and late presentation for the investigation of primary dysgenesis where, congenital agenesis of the amenorrhea as the parents of the patient had 5 cervix is a rare condition. The new European been counseled by various doctors to wait till 18 232 JSOGP 2015, Vol.5, No.4 Irum Sohail, Maria Habib years of age. The medical and surgical history was of her data in an anonymized scientific report. otherwise normal. There was no history of in utero Examination under general anesthesia revealed exposure to diethylstilbestrol. On examination, showed underdeveloped labial folds and absent vagina development of secondary sex characteristics was on inspection. normal (Tanner stage 5 for breast and pubis). Thyroid was normal. All the routine blood tests, hormonal profile and thyroid function tests were within normal limits. Karyotype was 46 XX. On abdominal ultrasound, the liver, kidneys, spleen, ureters and urinary bladder were all normal. (Figure 1) Pelvic ultrasound showed normal ovaries (right ovary volume 5.8ml, left ovary volume 5.1ml), uterus measuring 4*0.9cm, thin endometrial strip and absent cervix. (Figure 2) There was no haematocolpos or hematometra. Figure 3: Vulval appearance of patient with absent vagina On Bimanual rectal examination, cervix and vagina were entirely absent and uterus was hardly palpable. Patient and her family was counseled. Hysterectomy with vaginoplasty was planned to be done along Figure 1. Ultrasonographic appearance of uterus with psychiatric support in the long run. with absent cervix and vagina. Discussion We reported a rather rare case of cervical agenesis with entirely absent vagina and functioning uterine corpus. A woman with this condition is hormonally normal, therefore secondary sexual characters are achieved during puberty and chromosome constellation is 46XX. Uterus and ovaries are functioning and ovulation usually occurs. These women are seen by gynaecologist at the age of 14-16 years due to primary amenorrhea. Pelvic endometriosis often complicates anatomical integrity and fertility potential of these women. Hydrosalpinges, firm adhesions, ovarian endometriotic cysts, and frozen pelvis because of retrograde Figure 2. Ultrasonographic appearance of both menstruation offer unfavourable prognosis in these kidneys cases.8 MRI pelvis (without contrast) was done which Many authors have recommended total hysterectomy revealed hypoplastic uterus, absent cervix and as a treatment of choice for patients of functioning vagina. Examination under anesthesia was planned. uterine corpus with cervical and vaginal agenesis. Patient and her family was counseled. We obtained Conservative surgical approach to these patients informed consent from our patient for examination involves uterovaginal anastomosis and cervical under anesthesia, as well as consent for inclusion reconstruction. In patients with coexistent vaginal JSOGP 2015, Vol.5, No.4 233 Cervical and Vaginal Agenesis: A Novel Anomaly aplasia/agenesis, creation of neovagina is necessary. customary to perform this procedure at the age of Knowing this anomaly at a young age is a shock 17-20 years when the patients are emotionally for the patient and is accompanied by low self- mature and intellectually reliable to manage post- esteem so psychiatric help should be initiated operative requirements of care like manipulation of before surgical approach.2 vaginal form.2 Uterovaginal anastomosis involves suturing of the Finally, the application of uterine surrogacy offers isthmus uteri to the vagina to restore genital tract. an alternative to form their own family.9 Cervical reconstruction is an attempt to restore the functional anatomy of the genital tract with the Conclusion creation of a neocervix which involves the use of In conclusion, thorough investigation and total grafts such as slim thickness skin grafts, full hysterectomy along with vaginoplasty and thickness skin grafts, bladder mucosa graft or psychiatric support seem to be the first line saphenous vein graft. The overall success rate is approach in such cases. 60% for these techniques and there is still small Acknowledgements: We are grateful to the patient potential for pregnancy. Moreover, it is important to and her family for giving consent for her case realize the potential for complications such as record to get published. sepsis and failure.9 It is not thus surprising that total hysterectomy still offers numerous benefits References and is supported as a treatment option by several 1. Hefny AF, Kunhivalappil FT, Nambiar R, Bashira MO. A rare case of first-trimester ruptured bicornuate uterus in authors especially in cases of cervical agenesis. a primigravida. Int J Surg Case Rep. 2015; 14: 98–100. Hysterectomy eliminates needless suffering of young 2. Rock JA, Jones HW, editors. Te Linde's Operative adolescents from associated problems like retrograde Gynecology. Lippincott Williams and Wilkins; 2008. menstruation, sepsis and multiple procedures and If Surgery for anomalies of the mullerian ducts; p. 539. it is performed early, it is possible to preserve 3. Rezai S, Bisram P, Alcantara IL, Upadhyay R, Lara C, Elmadjian M. Didelphys Uterus: A Case Report and ovaries and their beneficial function.For the Review of the Literature. Case Rep Obstet Gynecol. reconstruction of vagina, surgeon should be prepared 2015; 865821. for vaginoplasty with use of split-thickness graft 4. Grimbizis GF, Camus M, Tarlatzis BC, Bontis JN, Devroey (McIndoe technique)2 (figure. 4). P. Clinical implications of uterine malformations and hysteroscopic treatment results. Hum Reprod Update. 2001;7(2):161-74. 5. American Fertility Society classification of mullerian anomalies. Fertil Steril. 1988;49:952. 6. Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M, Li TC, Tanos V, Brölmann H, Gianaroli L et al. The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies. Hum Reprod 2013b;28:2032–2044. 7. 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 Figure 4. McIndoe technique. 8. Jacob HJ and Griffin TW. Surgical reconstruction of the congenitally atretic cervic: two cases. Obstet Gynecol In the past, it was advised to delay vaginoplasty Surv. 1989;44(7):556-69. for these patients until just before their marriage. 9. Grimbizis GF, Tsalikis T, Mikos T, Papadopoulos N, This led to difficulties particularly when complications Tarlatzis BC, Bontis JN. Successful end-to-end cervico- cervical anastomosis in a patient with congenital cervical arose that caused a delay in marriage till complete fragmentation: case report. Hum Reprod. 2004 healing of vagina. More recently, it has become ;19(5):1204-10. 234 JSOGP 2015, Vol.5, No.4.
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