Williams Gynecology, Second Edition (Schorge,Williams Gynecology), 2nd Edition

CHAPTER 18
Anatomic Disorders

18–1. Congenital anatomic disorders of the female reproductive tract may result from which of the following mechanisms?

a. Genetic mutation

b. Developmental arrest

c. Abnormal hormonal exposure or exposure to environmental insults

d. All of the above

18–2. Which of the following organs is correctly paired with its embryologic origin?

a. Uterus—mesonephric duct

b. Bladder—mesonephric duct

c. Kidney—paramesonephric duct

d. Testes and ovaries—genital ridge

18–3. What percentage of females with uterovaginal malformations have associated urinary tract anomalies?

a. 5

b. 25

c. 50

d. 75

18–4. Which of the following statements is true regarding sexual differentiation in humans?

a. The SRY acts as a testis-deleting factor (TDF).

b. Without the influence of SRY, gonads develop as testes.

c. The gene is located on the Y chromosome and is named the sex-determining region of the Y (SRY).

d. The presence or absence of gonadal determinant genes is not thought to determine fetal gender.

18–5. Anti-müllerian hormone is involved in all EXCEPT which of the following?

a. Regression of the ipsilateral paramesonephric system

b. Peaking of testosterone production as a result of stimulation of the testes

c. Rapid gubernacular growth necessary for the transabdominal descent of testes

d. Prediction of successful ovarian hyperstimulation cycles during assisted reproduction

18–6. In female development, when do germ cells that carry two X chromosomes reach their peak number of 5 to 7 million oocytes?

a. At birth

b. After menarche

c. Just before menarche

d. In utero at 20 weeks’ gestation

18–7. The vagina forms partly from both the müllerian ducts and which other structure?

a. genital ridge

b. urogenital sinus

c. mesonephric duct

d. paramesonephric duct

18–8. The hymen is the partition that remains between which structures?

a. Cloacal membrane and genital tubercle

b. Sinovaginal bulb and urogenital sinus

c. Unfused cephalad portions of the two müllerian ducts

d. None of the above

18–9. Which of the following anatomic structures is correctly paired with its embryologic origin?

a. Genital tubercle—clitoris

b. Urethral folds—labia minora

c. Labioscrotal folds—labia majora

d. All of the above

18–10. Which of the following is the most common cause of female pseudohermaphroditism?

a. Maternal exposure to androgen-derivative medications

b. Maternal virilizing ovarian tumors, such as Sertoli-Leydig tumor

c. Fetal congenital adrenal hyperplasia due to deficiency of 21-hydroxylase

d. Fetal congenital adrenal hyperplasia due to deficiency of 11-beta hydroxylase

18–11. Female pseudohermaphroditism (category I) and male pseudohermaphroditism (category II) share which of the following characteristics?

a. Patients have testes.

b. Patients with either disorder are potentially fertile.

c. Both disorders result from excessive androgen exposure.

d. Surgery plays an important role in their respective treatment plans.

18–12. All of the following statements regarding complete androgen insensitivity syndrome (CAIS) are true EXCEPT which of the following?

a. On external examination, patients appear as pheno-typically normal females.

b. These women develop breasts during puberty due to abundant androgen-to-estrogen conversion.

c. Surgical excision of the testes is recommended prior to puberty to decrease the associated risk of germ cell tumors.

d. Estrogen replacement after puberty is important to maintain bone mass and to provide relief from vasomotor symptoms.

18–13. Which of the following statements are true regarding Turner syndrome?

a. There are classic physical stigmata of the syndrome.

b. They comprise the majority of patients with gonadal dysgenesis.

c. Although the uterus and vagina are normal, patients with the syndrome have streak gonads and subsequently present with primary amenorrhea.

d. All of the above

18–14. What is the male (46,XY) gonadal dysgenesis syndrome called?

a. Swyer syndrome

b. Klinefelter syndrome

c. Mixed gonadal dysgenesis

d. Embryonic testicular regression

18–15. All EXCEPT which of the following characteristics of Klinefelter syndrome are true?

a. These patients are tall, overvirilized men.

b. Patients have significantly reduced fertility.

c. The syndrome occurs in 1 to 2 percent of all males.

d. Men with Klinefelter syndrome are at increased risk for germ cell tumors, osteoporosis, and breast cancer.

18–16. When faced with ambiguous external genitalia of a newborn at delivery, the obstetrician should do which of the following?

a. Examine the mother for signs of hyperandrogenism.

b. Refer to the newborn as “your baby” and not as “it.”

c. Refrain from gender assignment by explaining that the genitalia are incompletely formed.

d. All of the above

18–17. All EXCEPT which of the following statements regarding bladder exstrophy are true?

a. This anomaly displays a predilection for females of 2:1.

b. It is characterized by an exposed bladder lying outside the abdomen.

c. Surgical closure is performed early in life and as a staged procedure.

d. It occurs from failure of the cloacal membrane to be reinforced by an ingrowth of mesoderm.

18–18. Causes of newborn clitoromegaly include which of the following?

a. Prematurity

b. Neurofibromatosis

c. Fetal exposure to excessive androgens

d. All of the above

18–19. A 14-year-old nulligravida presents to the emergency department with complaints of worsening lower abdominal pain over the past few days. She states that she has had a similar pain in the past, usually for a few days each month, but then it subsides. She is afebrile with stable vital signs. Although she has breasts and axillary and pubic hair, she has never had a period. Examination reveals a tender abdominal mass as well as a bluish bulging vaginal mass, shown here. Based on her history and your physical examination, which of the following conditions is your most likely diagnosis?

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Photograph contributed by Dr. Ellen Wilson. Reproduced, with permission, from Bradshaw KD: Anatomic disorders. In Schorge JO, Schaffer JI, Halvorson LM, et al (eds): Williams Gynecology, 1st ed. New York, McGraw-Hill, 2008, Figure 18-10.

a. Bartholin cyst

b. Imperforate hymen

c. Gartner duct cyst

d. Longitudinal vaginal septum

18–20. Appropriate techniques for the surgical correction of the condition diagnosed in Question 18–19 involve all EXCEPT which of the following?

a. Hymenectomy

b. Laparoscopy to exclude endometriosis

c. Repair in infancy or after thelarche

d. Needle aspiration of the hematocolpos

18–21. Compared with other müllerian duct defects, transverse vaginal septum is associated with which of the following?

a. Lower rates of endometriosis

b. Lower rates of urologic abnormalities

c. Lower success rates of surgical correction

d. None of the above

18–22. A 16-year-old nulligravida presents to the emergency department with complaints of abdominal and vaginal pain, worsening during the past several months. She describes the pain as being mostly on her right side and much worse during menstruation. During your physical examination, a patent vagina and cervix are noted, but a unilateral vaginal and pelvic mass is palpated. Transvaginal sonography demonstrates a single uterus and cervix but also a large pelvic mass filled with complex fluid, as shown below, and absence of the right kidney. What is your most likely diagnosis?

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a. Imperforate hymen

b. Uterine didelphys

c. Transverse vaginal septum

d. Longitudinal vaginal septum

18–23. Why is careful preoperative planning warranted with congenital vaginal cysts?

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a. The recurrence rate after excision is high.

b. They are frequently large, measuring greater than 8cminsize.

c. Their typical location is in the posterior-lateral wall of the vagina.

d. Some may extend up into the broad ligament and anatomically approximate the distal course of the ureter.

18–24. Müllerian anomalies are associated with anomalies of all EXCEPT which of the following systems?

a. Anal

b. Liver

c. Renal

d. Skeletal

18–25. A 22-year-old G3P0 presents to your office as a new patient for consultation regarding her history of multiple miscarriages. Her gynecologic history is otherwise unremarkable. During physical examination, you note that the uterus is markedly deviated to the left. Transvaginal sonography is performed, and three-dimensional images reveal a single uterus that is deviated and has a banana-shaped cavity, as shown below. What is your diagnosis?

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a. Septate uterus

b. Bicornuate uterus

c. Uterine didelphys

d. Unicornuate uterus

18–26. The pathogenesis of poor pregnancy outcomes with a unicornuate uterus is thought to be related to which of the following factors?

a. Cervical incompetence

b. Reduced uterine capacity

c. Anomalous distribution of the uterine artery

d. All of the above

18–27. Which of the müllerian anomalies, as illustrated in this sonogram, has the best reproductive prognosis? A gestational sac is seen on the image’s left.

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a. Septate uterus

b. Bicornuate uterus

c. Uterine didelphys

d. Unicornuate uterus

18–28. When should surgical reconstruction of a bicornuate uterus (Strassman metroplasty) be performed?

a. Only for complete bicornuate anomalies

b. In all women with the diagnosis of bicornuate uterus

c. Strassman metroplasty is not performed for repair of bicornuate uterus.

d. For women in whom recurrent pregnancy loss occurs with no other identifiable cause except their uterine anomaly

18–29. Septate uterus has a significantly higher spontaneous abortion rate and early pregnancy loss rate than bicornuate uterus. As illustrated in the figure below, what is the primary mechanism thought to be responsible for this extraordinarily high pregnancy wastage?

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a. Associated cervical anomalies

b. Distortion of the uterine cavity

c. Distortion of the uterine serosal contour

d. Partial or complete implantation on the largely avascular septum

18–30. Which of the following are seen in offspring of women who took diethylstilbestrol (DES)?

a. Impaired conception rates in female offspring

b. Cryptorchidism, testicular hypoplasia, and malformations of the penis in male offspring

c. T-shaped uterus, “cockscomb” cervix, and increased rates of clear cell adenocarcinoma in female offspring

d. All of the above

Chapter 18 ANSWER KEY

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