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Stump the Professor
Women’s Health
Symposium
August 2, 2009
Jenny Lovegreen
Elizabeth VonderHaar
Leah Smith
History of Present
Illness
• CO is 14 year old G0 referred from
an outside facility with an 18 month
history of cyclic abdominal pain. The
patient has never had any vaginal
bleeding and for the last two cycles,
her pain was severe enough that she
had to hospitalized for pain control
with IV medication.
Past Medical History
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MEDICATIONS: tetracycline
ALLERGIES: NKDA
SURGICAL HISTORY: appendectomy
OB/GYN HISTORY:
– Began breast development and pubic hair
development at age 11
– Has never had any vaginal bleeding
– Denies sexual activity
– G0
– Is not on contraception
Past Medical History
• FAMILY HISTORY:
– Non-contributory
• SOCIAL:
– Student
– Denies EtOH, Tobacco, Illicit drug use
PHYSICAL EXAM
• GENERAL: 5’ 7” 55.5 KG white
female in NAD
• VITALS: P 92 BP 100/54 R 18
• ABDOMEN: Flat, Soft, non-tender
• Differential Diagnosis
• Further Work up
Pelvic Exam
• Normal external female genitalia
• Tanner stage 3 pubic hair
• No signs of clitoral enlargement
Pelvic Exam
• Speculum Exam: long vaginal canal with no
cervix visualized at the apex
• 1-finger digital exam performed and the
apex of the vagina appeared to cover a
structure that felt like a long thin cervix
• No adenexal masses or tenderness
• Small anteverted uterus
IMAGING
• MRI:
– Vaginal canal with what appears to be a thin
septum covering the external cervical os
– No free fluid outside of the Uterine Cervix
– Normal Appearing Left and Right Ovaries
– Uterus
• Minimal indentation/Possibly suggestive of arcuate
uterus
• Narrowing in the lower segment and cervical canal
• Endometrium noted
IMAGING
• MRI (CONT)
– Small amount of free fluid noted in the pouch
of Douglas
– Urinary Tract:
• Both Kidneys present
• Right Renal collecting system completely
unremarkable
• Left Renal collecting system with what may be some
duplication, although duplicated ureter could not be
confirmed in its entirety
IMAGING
• TRANSABDOMINAL ULTRASOUND:
– 6.8 X 3 X 6 cm uterus.
– Central endometrial echo thickened (1.5
cm) at the fundus, which thins going to
the uterine corpus and is thin going
through what appears to be a cervical
canal
LABS
• H/H: 10.5/30.9
• O positive/Negative ABSC
Operative Findings
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Normal appearing uterine size and shape
Normal appearing left tube and ovary
Normal appearing right tube
Small right ovary with a streak-like
appearance
• Bilateral Ureters
• Posterior cul-de-sac containing multiple
cystic structures, likely endometriosis
WHAT REALLY
HAPPENED . . .
• CO started on continuous oral
contraceptives with significant relief
of her dysmenorrhea
• MRI repeated, diagnosis of most
likely cervical agenesis with a small
vaginal septum
• CO, stepmother and sister included in
discussion of treatment options:
– Continuous OCPs
• Future GIFT procedure
• Future attempt at recanalization
– Attempt at recanalization
– Hysterectomy with retention of her
ovaries
OPERATIVE FINDINGS
• Laparoscopic examination revealed no
identifiable cervical canal at the distal
Uterine segment
• Digital vaginal exam revealed no blood
• Boggy-feeling inferior portion of the
uterus
(specimens removed via the morcilator)
PATHOLOGY
• Uterus
– Cervix not identified grossly or microscopically
– Endometrium: benign inactive endometrium
with stromal pseudodecidual changes
consistent with hormone effect
– Myometrium: extensive adenomyosis
• Posterior cul-de-sac biopsy:
– Changes consistent with endometriosis; no
atypia and no malignancy identified
Cervical Agenesis
• Rare condition: 1 in 80,000 – 100,000
births
• Associated with both partial and
complete vaginal aplasia and renal
anomalies
• Type Ib mullerian anomaly
Cervical Agenesis
• Mullerian ducts develop into the fallopian
tubes, uterus, cervix, and upper vagina.
• Fused mullerian ducts form the corpus and
cervix of the uterus
• Vagina has dual origin – upper portion from
uterine canal and lower from urogenital
sinus
• Atresia of both sides of mullerian ducts
leads to cervical agenesis
– Usually associated with lack of upper vagina
due to common mullerian source
• Uterus develops normally
Diagnosis: Cervical
Agenesis
Presentation
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Presentation of obstructive anomaly
Primary amenorrhea
Cyclic abdominal or pelvic pain
Distended uterus if functional
endometrium is present
• Endometriosis due to retrograde flow
Diagnosis and
Management
• CT scan, ultrasound, and MRI are all
helpful in evaluating anatomy
• If uterus is obstructed – hysterectomy is
recommended
– Creation of epitheliazed endocervical tract
and vagina is an alternative
• Associated with significant morbidity
Diagnosis and
Management
• Conservative management using OCPs
to suppress retrograde menses can
be possible until pt is ready to
evaluate reproduction options
References
• Creighton, SM, et al, “Laparoscopic
management of cervial agenesis,”
Fertility and Sterility. Vol. 85 No. 5
May 2006
• Sadler, TW, “Urogenital System,”
Langman’s Medical Embryology. 9th ed
• William’s Gynecology. “Cervical
Defects.” Access Medicine, 2009.