Stump the Professor Womens Health Symposium

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Stump the ProfessorWomens Health Symposium

• August 2, 2009
• Jenny Lovegreen
• Elizabeth VonderHaar
• Leah Smith

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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.

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Past Medical History

• 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

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Past Medical History

• FAMILY HISTORY
• Non-contributory
• SOCIAL
• Student
• Denies EtOH, Tobacco, Illicit drug use

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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

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• Differential Diagnosis
• Further Work up

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Pelvic Exam

• Normal external female genitalia
• Tanner stage 3 pubic hair
• No signs of clitoral enlargement

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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

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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

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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

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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

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LABS

• H/H 10.5/30.9
• O positive/Negative ABSC

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Operative Findings

• 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

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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

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• 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

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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)

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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

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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

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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

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Diagnosis Cervical Agenesis
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Presentation

• Presentation of obstructive anomaly
• Primary amenorrhea
• Cyclic abdominal or pelvic pain
• Distended uterus if functional endometrium is
  present
• Endometriosis due to retrograde flow

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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

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Diagnosis and Management

• Conservative management using OCPs to suppress
  retrograde menses can be possible until pt is
  ready to evaluate reproduction options

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References

• Creighton, SM, et al, Laparoscopic management of
  cervial agenesis, Fertility and Sterility. Vol.
  85 No. 5 May 2006
• Sadler, TW, Urogenital System, Langmans
  Medical Embryology. 9th ed
• Williams Gynecology. Cervical Defects. Access
  Medicine, 2009.