Campbell-Walsh Urology, 11th Edition

PART XV

Pediatric Urology

SECTION F

Reconstruction and Trauma

151

Surgical Management of Disorders of Sexual Development and Cloacal and Anorectal Malformations

Richard C. Rink

Questions

1. Which of the following statements is FALSE regarding the construction of a vagina-utilizing bowel?
2. Failure to develop an adequate space between the rectum and bladder can result in compromised blood flow to the segment used for vaginal construction.
3. In general, colon is preferred versus ileum because of its lower incidence of associated postoperative stenosis.
4. When compared with the McIndoe procedure, the bowel vagina suffers from a higher incidence of postoperative stenosis.
5. An advantage of a bowel vagina versus the McIndoe procedure includes the lubricating properties of mucus (which may help to facilitate intercourse).
6. One specific indication for the use of ileum is a previous history of pelvic radiation.
7. Urogenital sinus anomalies in disorders of sexual development states are most commonly seen in association with:
8. congenital adrenal hyperplasia.
9. mixed gonadal dysgenesis.
10. true hermaphroditism.
11. cloacal anomalies.
12. gonadal dysgenesis.
13. The most common finding in cloacal anomalies that have been diagnosed by antenatal ultrasonography is:
14. ascites.
15. distended rectum.
16. distended bladder.
17. distended vagina.
18. distended bladder and rectum.
19. What is the most common vaginal anatomy in cloacal malformation?
20. Single vagina, single uterus
21. Single vagina, double uterus
22. Two vaginas, two uteri
23. Two vaginas, one uterus
24. Single vagina, no uterus
25. Neonatal vaginoplasty combined with clitoroplasty and labioplasty has all of the following advantages EXCEPT:
26. it allows phallic skin for vaginal reconstruction.
27. maternal estrogens increase vaginal thickness and vascularity.
28. tissues are less scarred.
29. vaginal stenosis is clearly less.
30. The cut-back vaginoplasty is appropriate for:
31. labial fusion.
32. low vaginal confluence.
33. high vaginal confluence.
34. vaginal atresia.
35. vaginal agenesis.
36. Surgical management of cloacal malformations involves all of the following steps EXCEPT:
37. decompression of the gastrointestinal tract.
38. decompression of the genitourinary tract.
39. vaginostomy.
40. definitive repair of the cloaca.
41. correction of nephron destructive anomalies.
42. Fecal continence after cloacal reconstruction is most closely related to:
43. the level of rectal confluence.
44. associated urinary anomalies.
45. neurologic status.
46. the type of repair.
47. the timing of the repair.

Answers

1. c. When compared with the McIndoe procedure, the bowel vagina suffers from a higher incidence of postoperative stenosis.A high incidence of postoperative vaginal stenosis necessitates postoperative vaginal dilatation in the McIndoe procedure.
2. a. Congenital adrenal hyperplasia.Urogenital sinus abnormalities are most often seen in disorders of sexual differentiation states, most commonly in association with congenital adrenal hyperplasia, which has been noted to have an incidence as frequent as 1 in 500 in the nonclassic mild forms.
3. d. Distended vagina.The common finding in all reports has been a cystic pelvic mass between the bladder and rectum, representing a distended vagina.
4. c. Two vaginas, two uteri.In Hendren's report on 154 patients with cloacal anomalies, 66 patients had one vagina, 68 had two vaginas, and the vagina was absent in 20 (Hendren, 1998). The incidence of vaginal duplication is even higher in the author’s own patient population. The uterus anomaly generally is similar to the vaginal anomaly, that is, two vaginas with two uteri.
5. d. Vaginal stenosis is clearly less.Other investigators, including the author’s group, have thought that vaginoplasty, regardless of the vaginal location, is best combined with clitoroplasty in a single stage. This allows the redundant phallic skin to be used in the reconstruction, adding flexibility for the surgeon, which is compromised when the skin has been previously mobilized. Furthermore, the authors and others have noted that maternal estrogen stimulation of the child's genitalia results in thicker vaginal tissue, which is better vascularized, making vaginal mobilization more easily performed.
6. a. Labial fusion.The cut-back vaginoplasty is rarely used and is appropriate only for simple labial fusion.
7. c. Vaginostomy.Surgical management now involves four basic steps: decompression of the gastrointestinal tract, decompression of the genitourinary tract, correction of nephron-destructive or potentially lethal urinary anomalies, and definitive repair of the cloaca.
8. c. Neurologic status.Fecal continence is directly related to neurologic status.

Chapter review

1. The communication of the vagina with the urinary tract usually occurs in the mid to distal urethra.
2. In patients with congenital adrenal hyperplasia, the location of the confluence of the vagina and the urethra is the critical determinant in the surgical management.
3. Hydrometrocolpos is frequently the initial sign of a urogenital sinus abnormality. It is caused by urine draining into the vagina with poor vaginal drainage.
4. Persistent clitoral hypertrophy may occur in premature infants without DSDs.
5. A cervical impression in the dome of the vagina seen on genitography denotes normal female internal organs.
6. When gonads in the neonatal period require biopsy, a deep biopsy is appropriate because the ovarian component of an ovotestis may cover the testicular component.
7. Renal anomalies commonly occur in patients with a persistent cloaca.
8. Women with CAH are less satisfied as adults with their genitalia than controls.
9. Genital reconstruction must address clitoroplasty, labioplasty, and vaginoplasty.
10. When clitoroplasty is performed, the glans, tunics, and neurovascular bundles should be preserved. The neurovascular bundles should not be mobilized.
11. The timing of surgery for genital reconstruction is controversial: vaginoplasty is best combined with clitoroplasty and labioplasty as a single procedure.
12. Vaginoplasty may be performed with a posterior-based perineal flap for low vaginal confluence, a pull-through vaginoplasty for a high confluence, and vaginal replacement for an absent or rudimentary vagina.
13. The flap in a flap vaginoplasty must reach the normal caliber of the vagina; that is, it must be placed cephalad to the narrowed area of the distal vagina.
14. A vaginoplasty performed in the neonatal period will usually require a secondary procedure after puberty.
15. In cloacal malformations surgical management initially involves decompression of the gastrointestinal tract, generally with a colostomy, and decompression of the genitourinary tract. This is followed by correction of urinary collecting-system abnormalities that impair urine flow. A single stage repair of rectal, vaginal, and urethral abnormalities is performed at a later date when the child is stable.
16. Intermittent catheterization of the vagina may successfully decompress the genitourinary tract in cloacal malformations.
17. Spinal cord abnormalities are frequently found in patients with persistent cloaca.
18. In patients who have had corrective surgery for cloacal abnormalities, a high percentage have a neuropathic component to both urinary and fecal incontinence.
19. In planning treatment for a transverse vaginal septum, it is critical to determine whether there is a cervix and, if present, its exact location relative to the septum.
20. Vaginal atresia differs from vaginal agenesis and testicular feminization in that the Müllerian structures are not affected. As a result, the uterus, cervix, and upper portion of the vagina are normal.