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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 17  |  Issue : 1  |  Page : 71-75

Congenital vaginal agenesis, davydov procedure: A case report and review of literature


1 Department of Obstetrics and Gynaecology, Bayero University Kano/Aminu Kano Teaching Hospital, Kano, Kano State, Nigeria
2 Department of Obstetrics and Gynaecology, Aminu Kano Teaching Hospital, Kano, Kano State, Nigeria

Date of Submission02-May-2018
Date of Acceptance16-Oct-2019
Date of Web Publication30-May-2020

Correspondence Address:
Dr. Ayyuba Rabiu
Department of Obstetrics and Gynaecology, Bayero University Kano/Aminu Kano Teaching Hospital, P.M.B. 3011 Kano, Kano State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njbcs.njbcs_18_18

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  Abstract 


Congenital vaginal agenesis, also called Müllerian agenesis or Rokitansky–Mayer–Küster–Hauser syndrome, is a rare anomaly of the female genital tract. The affected individuals present with primary amenorrhea, varying degree of uterovaginal anomalies, infertility, and other associated renal, spine, and skeletal malformations. Embryologically, the vagina is formed from the fusion of the two embryonic structures: the upper part from the Müllerian duct system and the lower third from the urogenital sinus. Müllerian agenesis is caused by embryologic underdevelopment of the Müllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. We report the case of a 25-year-old woman who presented with complaints of delayed onset of menses and inability to have sexual intercourse with her partner. She had well-developed secondary sexual characteristics. She had never experienced penetrative sexual intercourse despite being married for 2 years. A general physical examination revealed a young woman of normal stature. She had well-developed breast (Tanner stage 5). The clitoris and pudendal cleft were normal. There was normal external urethral meatus with blind ended vagina of 2 cm long. A digital rectal examination revealed good sphincteric tone with no uterus or cervix palpated. A diagnosis of congenital uterovaginal anomaly was made. A buccal smear done revealed XX karyotype. She was counseled about her condition. Consents for surgery and media release were sought and obtained. A Davydov procedure for neovaginal construction was made and her postoperative condition was satisfactory. Congenital vaginal agenesis is a rare condition with profound effects on the affected individuals. Therefore, proper counseling on future fertility options and the need for neovaginal construction for improved sexual satisfaction should never be overemphasized among others.

Keywords: Davydov procedure, Nigeria, vaginal agenesis


How to cite this article:
Rabiu A, Ahmed ZD, Garba I, Balogun MO, Lawal M. Congenital vaginal agenesis, davydov procedure: A case report and review of literature. Niger J Basic Clin Sci 2020;17:71-5

How to cite this URL:
Rabiu A, Ahmed ZD, Garba I, Balogun MO, Lawal M. Congenital vaginal agenesis, davydov procedure: A case report and review of literature. Niger J Basic Clin Sci [serial online] 2020 [cited 2020 Aug 7];17:71-5. Available from: http://www.njbcs.net/text.asp?2020/17/1/71/285464




  Introduction Top


Congenital vaginal agenesis, also called Müllerian agenesis or Müllerian aplasia, is a rare anomaly of the female genital tract that were first described by Aetios of Amanda (502–575 A.D.).[1] Müllerian agenesis is caused by embryologic underdevelopment of the Müllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both.[2] Congenital absence of vagina occurs in 0.001%–0.025% of population.[3] These disorders of vaginal malformations typically do not occur alone, but in combination with several developmental problems in a female baby such as Bardet–Biedl syndrome, Fraser syndrome, or Rokitansky–Mayer–Küster–Hauser syndrome.[4] Many classifications for congenital uterovaginal anomalies exist and are useful for guidance in the diagnosis and management of this condition; however, the modified American Fertility Society (AFS) classification by Rock and Adam [5] is worthy of mention due to its simplicity and addressing the fundamental developmental problems of patients with this condition. In the modified AFS classification by Rock and Adam, the emphasis was put on correlating the anatomic anomalies with embryologic arrests. As such, uterovaginal anomalies are categorized as dysgenesis disorders or vertical or lateral fusion defects. There is a further subdivision into obstructive forms, for which immediate treatment is required due to retrograde flow of menstrum with increasing pressure on the adjacent structures and nonobstructive forms. The division is into four classes.

In Class 1, there is a dysgenesis of the Müllerian ducts. Hypoplasia of the Müllerian duct derivatives such as the uterus and upper two-thirds of the vagina falls into this class. Class 2 is a disorder of vertical fusion. Here, there is failure of fusion of the Müllerian system with the sinovaginal bulb which results in cervical dysgenesis and obstructive or nonobstructive transverse vaginal septa. Class 3 are disorders of lateral fusion. These categories consist of a duplicated or partially duplicated reproductive tract. Examples include anomalies due to failure of fusion of the paired Müllerian ducts, for example, didelphic and bicornuate uteri, and septate uterus. They are further subdivided into (a) the symmetric nonobstructive form which includes unicornuate, bicornuate, didelphic, septate, and diethylstilbestrol (DES)-related uteri) and (b) the asymmetric obstructive form such as the unicornuate uterus with obstructed horn, double uterus with unilaterally obstructed horn, and double uterus with unilaterally obstructed vagina. The last in the category is Class 4. Here, there are unusual configurations and combinations of defects. The rationale behind this report is not unconnected with limited skills and equipment in laparoscopic surgeries coupled with poor sexual satisfactions associated with William's vaginoplasty.


  Case Report Top


She was a 25-year-old Hausa Muslim Housewife who presented with complaints of delay in onset of menses and inability to have sexual intercourse with her partner for 2 years. She had never menstruated since the attainment of secondary sexual characteristics such as breast development and axillary and pubic hair growth. There was no associated history of cyclical abdominal pains, no difficulty in urination, or chronic pelvic pain. There were no other urinary symptoms and no musculo skeletal complaints. There was associated history of apareunia, as the patient had never experienced penetrative sexual intercourse despite being married for 2 years. She was not involved in strenuous exercise. There was no significant weight change. There was no history of excessive eating or undereating. There was no associated history of milky breast secretions and no heat or cold intolerance. There was no history of excessive hair growth, hoarseness of voice, male pattern, and baldness. There was history of similar condition in two of her sisters, who were both married, but were yet to attain menarche. She was married to a butcher in a polygamous setting of two wives. Her cowife has four children.

A general physical examination revealed a young woman of normal stature. She was not pale and afebrile. She had no acne, hirsutism, or striae. She had pubic (female pattern) and axillary hair growth. Her pulse rate and blood pressure were 82 beats/min and 110/60 mmHg, respectively, and the heart sounds were first and second, respectively. She had well-developed breast (Tanner stage 5).

The abdomen was full with no mass palpable and no inguinal swelling. The clitoris and pudendal cleft were normal [Figure 1]. There was normal external urethral meatus with blind ended vagina of 2 cm long [Figure 2]. A digital rectal examination revealed good sphincteric tone with no uterus or cervix palpated. A diagnosis of congenital uterovaginal anomaly was made.
Figure 1: Female external genitalia, depicting clitoral hood, labia majora, and pudendal cleft

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Figure 2: Female external genitalia with urethral catheter (Foley) in situ and blind ended vagina

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A buccal smear done revealed XX karyotype. Her serum electrolytes were within normal limits. Available hormonal assays done were also within normal female levels. An abdominopelvic ultrasound scan done revealed normal kidneys and other abdominal organs. There was absence of uterus. She was counseled about her condition; management options and the future options for her to have children were also addressed. She consented for surgery. Consents for surgery and media release were sought and obtained.

The patient was cleaned and draped in modified lithotomy position under spinal anesthesia enhanced by sedation. A simultaneous abdominal and vagina approach was made; a midline subumbilical incision was made and deepened by a sharp and blunt dissection until the peritoneum was opened. The findings of absent uterus, cervix and upper vagina, and normal ovaries bilaterally were noted [Figure 3]. The broad ligament was identified.
Figure 3: Absence of uterus in the pelvis with peritoneal reflection covering the bladder and the pouch of Douglas

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Vaginally, the summit of the blind vagina was identified and incised transversely; the flaps were opened and graded Hegar's dilators were inserted with the tip held within the tissue plane between the bladder and broad ligament.

Abdominally, the lateral ends of the broad ligament were held and an incision was made in front of the transverse bundle joining the two ovaries and tubes and the peritoneum of the posterior fold was gently dissected away to create a free fold of peritoneum.

The posterior leaf of the new peritoneal fold was dragged down and sutured to the posterior leaf of the vagina cuff. Also, the two borders of the peritoneal incision were drawn downward and stitched to the ipsilateral lips of the vestibulotomy. The vault of the neovagina was created using a so-called purse-string stitch performed by taking peritoneum of the bladder, the right-sided round ligament, the right-sided “utero-ovarian” ligament, the pelvic peritoneum between the right ovary and rectum with utmost care to avoid ureter, and then the right-side surface of the rectum before going to its ventral surface to repeat the same technique in reverse order on the patient's left side [Figure 4].
Figure 4: Construction of the vaginal vault from the peritoneal reflection on the rudimentary uterine bud and the sigmoid

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Hemostasis was secured and the abdomen was closed in layers with vicryl 1 to the rectus sheath continuously, vicryl 2/0 to the subcutaneous layer, and nylon 2/0 to the skin by interrupted mattresses sutures. Foley's catheter was inserted into the bladder through the urethra. A vagina mold of about 9 cm in length was made and covered with a condom and inserted into the vagina to maintain patency while the labia majora were apposed over it to prevent the mold from falling off [Figure 5] and [Figure 6]. The perineum was cleaned. Immediate postoperative condition was satisfactory. She was discharged after a week and was seen 2 weeks after at a gynecology clinic. She had no complaints. She was counseled about the need for early commencement of sexual intercourse with her husband to maintain the patency and capacity of the neovagina and the preventive measures for sexually transmitted infections. Fertility options, such as adoptions and surrogacy, and the need for psychological evaluation were also discussed.
Figure 5: Neovagina constructed with a mold in situ

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Figure 6: Labial stitches applied to keep the mold in situ

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  Discussion Top


Vaginal atresia commonly results as a part of complex anomalies but in rare occasions as an isolated developmental defect. Clinical features depend on the extent of the anomalies, the severity, and the age of the patient at presentation. Signs and symptoms of the condition are usually unnoticed until they reach puberty where they present with primary amenorrhea.[2] In this case, she presented with primary amenorrhea and infertility. She did not have uterus and cervix, and as such there was no cramping cyclical lower abdominal pain which signifies hematocolpus or hematometria in those that have uterus with outflow obstruction.[6] Up to 53% of patients with Müllerian agenesis have other congenital anomalies usually involving the urinary and musculoskeletal system.[2] This patient did not present with urinary symptoms and musculoskeletal problems even though there were limited availability of investigative measures in our center coupled with poor financial status of the patient which hindered her to afford other relevant investigations. Penetrative sexual intercourse was not achievable and she presented with apareunia. This was because of the absent or poorly developed vagina; they are faced with serious challenges of being unable to get pregnant, bear children, and enjoy sexual satisfaction.[1] There were no classical features of androgen insensitivity syndrome such as undescended testis and hypospadias,[7] and buccal smear kryotyping revealed XX chromosome. Also, there were no clinical features suggestive of congenital adrenal hyperplasia. In well-developed centers where there are standard laboratory backings, diagnosis of congenital adrenal hyperplasia (CAH) is easily achieved by demonstration of inadequate production of cortisol, aldosterone, or both with accumulation of excess concentration of precursor hormones.[8] For example, in 21-hydroxylase deficiency, there is high serum concentration of 17-hydroxyprogesterone (>1000 ng/dL) and presence of urinary pregnanetriol with clinical features of salt wasting, ambiguous genitalia, clitorimegaly, premature phallic enlargement in the absence of testicular enlargement, precocious pubic hair, hirsutism, oligomenorrhea, and female infertility.[9]

An examination showed she had normal stature with well-developed breast, pubic and axillary hair, and no features of hyperandrogenism. Pelvic ultrasound scan done confirmed the absence of uterus, hence the possibility of the diagnosis of vaginal Müllerian agenesis.

The Davydov procedure for vaginal reconstruction was chosen in this case after proper counseling, even though the first-line treatment approaches were discussed. In this case, a mold was inserted in the neovaginal cavity to prevent spontaneous collapsing and was kept in situ with the help of labial stitches for the first 24 h after surgery. Thereafter, it was removed and changed until complete epithelialization of the neovaginal has occurred as suggested by Giannesi et al.[10] With regard to commencement of sexual intercourse, some authorities believed it is recommended as soon as epithelialization of the neovagina allows it without discomfort.[10]

The American College of Obstetricians and Gynecologists (ACOG) guidelines for patients with Müllerian agenesis recommend that sexually active women with Müllerian agenesis should be aware of their risk of getting sexually transmitted infections, and barrier methods such as condoms should be used for intercourse.[2] This issue was properly addressed at her follow-up clinic visit.


  Conclusion Top


Congenital vaginal agenesis is a rare condition that has profound effects on the affected individuals. Therefore, proper counseling on future fertility options, the need for neovaginal construction either surgical or nonsurgical for improved sexual satisfaction, and the strong relationship with other congenital anomalies should never be overemphasized among others.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Prabhakar U. A study on reconstructive options for vaginal agenesis. 2007:1-124. Available from: http://repository-tnmgrmu.ac.in/3483/1/180300107prabhakar.pdf. [Last accessed on 2019 Oct 02].  Back to cited text no. 1
    
2.
ACOG. Müllerian Agenesis: Diagnosis, Management, and Treatment. ACOG Committee Opinion. Number 728, January 2018. Available from: https://www.acog.org/Clinical-Guidance-and-Publications/Committee-Opinions/Committee-on-Adolescent-Health-Care/Mullerian-Agenesis-Diagnosis-Management-and-Treatment?IsMobileSet=false. [Last accessed on 2019 Oct 02].  Back to cited text no. 2
    
3.
Fligner J. Congenital atresia of the vagina. Surg Gynecol Obs 1987;165.  Back to cited text no. 3
    
4.
Vaginal Atresia __ Nationwide Children's Hospital; 2017: Available from: https://www.nationwidechildrens.org/vaginal-atresia--1. [Last accessed on 2019 Oct 02].  Back to cited text no. 4
    
5.
Rock JA, Adam RA. Surgery to repair disorders of development. In: Nichols DH, Clark-Pearson DL, editors. Gynecologic, Obstetric and Related Surgery. 2nd ed. St. Louis, MO: Mosby; 2000. p. 780-813.  Back to cited text no. 5
    
6.
Vijayalakshmi S, Mahendra G, Prathyusha N, Pukale R, Durgam S, Subrahmani. Cervical and vaginal agenesis – A rare case report. IAIM 2015;2:146-150.  Back to cited text no. 6
    
7.
Androgen insensitivity syndrome_ MedlinePlus Medical Encyclopedia. 2018. Available from: https://medlineplus.gov/ency/article/001180.htm. [Last accessed on 2019 Oct 02].  Back to cited text no. 7
    
8.
Turcu A, Auchus R. The next 150 years of congenital adrenal hyperplasia. J Steroid Biochem Mol Biol 2015;153:63-71.  Back to cited text no. 8
    
9.
Wilson T. Congenital Adrenal Hyperplasia Workup_ Laboratory Studies, Imaging Studies, Other Tests. Medscape 2018. Available from: https://emedicine.medscape.com/article/919218-workup. [Last accessed on 2019 Oct 02].  Back to cited text no. 9
    
10.
Giannesi A, Marchiole P, Benchaib M, Chevret-Measson M, Mathevet P, Dargent D. Sexuality after laparoscopic Davydov in patients affected by congenital complete vaginal agenesis associated with uterine agenesis or hypoplasia. Hum Reprod 2005;20:2954-7.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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