Cervical agenesis

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Cervical agenesis
Classification and external resources
Specialty Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value).
ICD-10 Q51.5
ICD-9-CM 752.43
Patient UK Cervical agenesis
[[[d:Lua error in Module:Wikidata at line 863: attempt to index field 'wikibase' (a nil value).|edit on Wikidata]]]

Cervical agenesis is a congenital disorder of the female genital system that manifests itself in the absence of a cervix, the connecting structure between the uterus and vagina. Milder forms of the condition, in which the cervix is present but deformed and nonfunctional, are known as cervical atresia or cervical dysgenesis.

Epidemiology

Cervical agenesis is estimated to occur in 1 in 80,000 females.[1] It is often associated with deformity of the vagina; one study found that 48% of patients with cervical agenesis had a normal, functional vagina, while the rest of the cases were accompanied by vaginal hypoplasia.[2]

Presentation and diagnosis

Patients with cervical agenesis typically present in early adolescence, around the time of menarche, with amenorrhea and cyclic pelvic pain caused by the obstruction of menstrual flow from the uterus.[2]

The diagnosis of cervical agenesis can be made by magnetic resonance imaging, which is used to determine the presence or absence of a cervix. Although MRI can detect the absence of a cervix (agenesis), it is unable to show cervical dysgenesis (where the cervix is present, but malformed).[2] Ultrasound is a less reliable imaging study, but it is often the first choice by gynecologists to establish a diagnosis and can identify a hematometra secondary to cervical agenesis.[3]

Pathophysiology

Cervical agenesis arises during fetal development, during which time the paramesonephric duct fails to canalize in formation of the cervix.[4]

Management

The first line of therapy after diagnosis typically involves the administration of the combined oral contraceptive pill, medroxyprogesterone acetate or a gonadotropin-releasing hormone agonist to suppress menstruation and thereby relieve pain.[3] Surgically, cervical agenesis has historically been treated through hysterectomy (removal of the uterus) to relieve symptoms caused by hematocolpos (the accumulation of menstrual fluid in the vagina).[4] Other surgical methods of management involve the creation of an anastomotic connection between the uterus and vagina by neovaginoplasty or recanalization of the cervix. Outcomes in these cases are generally poor, since the natural functions of the cervix—such as mucus production and providing a barrier against ascending infection—cannot be replicated.[4] Furthermore, the success rate of uterovaginal anastomosis is less than 50% and most patients require multiple surgeries while many develop cervical stenotis.[3][4] Despite this, several pregnancies have been reported in women with cervical agenesis who underwent surgical treatment.[1]

Complications

If untreated, the accumulation of menstrual fluid in the uterus caused by cervical agenesis can lead to hematocolpos, hematosalpinx, endometriosis, endometrioma and pelvic adhesions.[2][3]

See also

References

  1. 1.0 1.1 Arulkumaran, Sabaratnam, ed. (2011). Oxford Desk Reference: Obstetrics and Gynaecology. Oxford University Press. p. 533. ISBN 9780199552214.<templatestyles src="Module:Citation/CS1/styles.css"></templatestyles>
  2. 2.0 2.1 2.2 2.3 Falcone, Tommaso; Hurd, William W. (2013). Clinical Reproductive Medicine and Surgery: A Practical Guide. Springer Science+Business Media. pp. 312–314. ISBN 9781461468370.<templatestyles src="Module:Citation/CS1/styles.css"></templatestyles>
  3. 3.0 3.1 3.2 3.3 Sokol, Andrew I.; Sokol, Eric R. (2013). General Gynecology: The Requisites in Obstetrics and Gynecology. Elsevier. p. 217. ISBN 9780323032476.<templatestyles src="Module:Citation/CS1/styles.css"></templatestyles>
  4. 4.0 4.1 4.2 4.3 Drutz, Harold P.; Herschorn, Sender; Diamant, Nicholas E. (2007). Female Pelvic Medicine and Reconstructive Pelvic Surgery. Springer Science+Business Media. p. 54. ISBN 9781846282386.<templatestyles src="Module:Citation/CS1/styles.css"></templatestyles>