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RECONSTRUCTIVE
UROLOGY
Vaginal
reconstruction for ambiguous genitalia and congenital absence of the vagina:
a 27-year experience
Graziano K, Teitelbaum DH, Hirschl RB, Coran AG
Section of Pediatric Surgery, Department of Surgery, the C.S. Mott Children’s
Hospital and the University of Michigan Medical School, Ann Arbor, MI,
USA
J Pediatr Surg. 2002, 37:955-60
- Background/Purpose:
Gender assignment to a neonate with ambiguous genitalia is crucial.
Patients with an absent vagina require the construction of an artificial
vagina. In an effort to improve care, the authors have categorized their
experience with this group of children.
- Methods:
Since 1974, we cared for 114 patients with anomalies of the genitalia.
There were 53 genotypic girls with congenital adrenal hyperplasia (CAH),
16 genotypic boys with testicular feminization syndrome (TFS), 13 with
mixed gonadal dysgenesis (MGD), 9 with Mayer-Rokitansky syndrome, and
4 true hermaphrodites. The remaining 19 had other genital abnormalities.
- Results:
After
1980, patients with CAH underwent clitoral recession and vaginoplasty.
All patients with TFS were raised as girls and underwent orchidectomy.
Eleven of the MGD patients were given a female sex assignment and underwent
gonadectomy. Twenty-eight patients underwent intestinal vaginoplasty
including 8 of the TFS patients, 9 with Mayer-Rokitansky syndrome, 8
patients with cloacal anomalies, 2 patients for rhabdomyosarcoma, and
1 of the MGD patients.
- Conclusions:
1) This review emphasizes the range of diagnoses the surgeon must be
prepared to address in patients with ambiguous genitalia. 2) Colovaginoplasty
is an excellent procedure for replacement of a completely absent vagina.
3) Continued evaluation of this group will delineate appropriate timing
and choice of procedure.
- Editorial
Comment
A correct diagnosis and gender assignment is imperative to be performed
in those patients suffering from ambiguous genitalia. Gender assignment
is based on the possibility to reconstruct a normal individual with
normal sexual function. There are several developmental anomalies on
which vaginoplasty is needed, and numerous techniques to perform it
are described. However, no one surgical procedure can be applied to
reconstruct all cases of ambiguous genitalia. The wide anatomical variability
imposes an individualized approach in these patients.
The authors showed an expressive series of patients suffering of ambiguous
genitalia. They have performed several procedures to correct several
developmental anomalies, which have developed with ambiguous genitalia.
While most patients with a diagnosis of congenital adrenal hyperplasia
and clitoral hypertrophy underwent a clitoral resection and vaginoplasty
before the age of 6 months, patients with testicular feminization syndrome
underwent reconstruction much later, 60% after the age of 15. It is
a key point to perform the surgical procedure in the appropriate moment
and thus, avoiding undesirable psychological effects caused by ambiguous
genitalia. Furthermore, the printing issue has an important role in
the gender reassignment, no matter whether genotype is male or female.
The authors’ results of long-term vaginoplasty with colon are very
interesting. Eighteen of 26 women are sexually active and have none
complications and none of their patients have experienced excessive
mucus production from the neovagina.
Perhaps, the most important message from this article is that the surgeon
needs to be aware of the great variety of diagnoses and surgical options
to correct ambiguous genitalia.
Dr.
E. Alexsandro da Silva
Urogenital Research Unit
State University of Rio de Janeiro
Rio de Janeiro, RJ, Brazil
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