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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_207_библиотеки_им_акад_М_И_Перельмана
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2
Cervix
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Chapter 2.1
Evaluation and Management of Cervical Agenesis
Jonathan D. Kort, Steve n J. Co, Ste ven T. Nakajim a
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GENERAL PRINCIPLES
Definition
Cervical agenesis, also known as congenital cervical atresia, is the absence of a cervix in a woman with a functional uterus
due to failed development or fusion of the Müllerian ducts. It may be an isolated finding, but is also seen in conjunction with
vaginal atresia. It often presents with primary amenorrhea and cyclical or chronic pelvic pain.
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Differential Diagnosis
Müllerian agenesis (Mayer–Rokitansky–Kuster–Hauser syndrome)
Androgen insensitivity
Imperforate hymen
Transverse vaginal septum
Gonadal dysgenesis and other causes of ovarian insufficiency
Isolated gonadotropin deficiency
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Nonoperative Management
Treatment is geared toward relieving pain, facilitating sexual intercourse for patients diagnosed with concomitant vaginal
agenesis, and facilitating fertility when appropriate. Hormonal suppression of the hypothalamic–pituitary–ovarian axis to
prevent cyclic development and shedding of the uterine lining will improve pelvic pain secondary to obstructed menstruation
before definitive surgical treatment. Norethindrone-based steroids (norethindrone acetate, 5 mg/day tablet) are helpful due to
the peripheral conversion to ethinyl estradiol for bone health. For fertility, there are case reports of transmyometrial embryo
transfers in conjunction with in vitro fertilization; however, this is experimental and use of a gestational carrier is typically
advised.
1
When diagnosed in conjunction with vaginal agenesis, vaginal dilator treatment is appropriate for motivated patients
who wish to become sexually active.
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IMAGING AND OTHER DIAGNOSTICS
Magnetic resonance imaging (MRI) of the pelvis will help elucidate if any cervix is present, as well as differentiate the
disorder from müllerian agenesis or a transverse vaginal septum (Figs. 2.1.1 and 2.1.2).
A karyotype will help distinguish from androgen insensitivity syndrome.
An assessment of ovarian reserve (follicle stimulating hormone [FSH], estradiol, anti-müllerian hormone [AMH]) will help
differentiate this cause of amenorrhea from primary ovarian insufficiency.
An abdominal ultrasound will help identify associated renal anomalies if not assessed during the MRI.
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PREOPERATIVE PLANNING
Due to limitations of pelvic imaging, an exam under anesthesia to evaluate vaginal development, in conjunction with
evaluation of the pelvis via laparoscopy or exploratory laparotomy may be required to clarify anatomic pelvic structures
before determining if hysterectomy or cervicovaginal reconstruction is appropriate.
2
This can be done as a separate
procedure or in conjunction with definitive surgery.
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SURGICAL MANAGEMENT
The primary goal of surgical management is to alleviate the pain from obstructed menstruation. Patients should be counseled
that hysterectomy may be the most appropriate definitive surgical treatment for cervical agenesis and be prepared for that
outcome before definitive surgical management is attempted. Reoperation and hysterectomy rates are high among patients
who initially attempt cervicovaginal reconstruction.
2,3
In a patient without contraindications, hormonal suppression of
endometrial development and vaginal dilator therapy can often palliate pelvic pain and sexual dysfunction until they accept the
possibility of hysterectomy.
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Positioning
Patients should be positioned in the dorsal lithotomy position to allow access to evaluate the vagina and abdomen
simultaneously. If the vagina is present, placement of a vaginal sizer or sponge-stick will help identify the proximal vagina
when assessing the pelvis.
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Approach
With the patient in dorsal lithotomy position, the abdomen is entered. Depending on a surgeons’ comfort with minimally
invasive surgery, this can be performed with laparoscopy or an exploratory laparotomy.
Figu re 2 .1.1. A,B: T 2W sagittal images through the pelvis demonst rat e a urethra (blue arrow) and bladder (red arrow) but no findings of a vagina or cervix is
consistent wit h vaginal and cervical agenesis. T here is an isolated or noncommunicating left uterine horn (red arrowhead).
Figu re 2 .1.2. A–D: T2W axial images through the pelvis demonstrate a urethra (blue arrow) and bladder (red arrow) but no findings of a vagina or cervix
consistent wit h vaginal and cervical agenesis. T here is an isolated or noncommunicating left uterine horn (red arrowhead).
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