Dr. Blizzard has abstracted and commented upon two extraordinarily
important manuscripts by Migeon and
colleagues. These investigators have provided the first
analysis of the long-term outcome of 75 adults with male
pseudohermaphroditism or micropenis (46XY or 45X/46XY) managed as
children at Johns Hopkins Hospital. These children had been
assigned to either the male or female gender. All of 18
patients with feminine external genitalia (androgen insensitivity
syndrome or complete gonadal dysgenesis) were raised as females;
5/18 subjects with micropenis (stretched length <1.9 cm) without
hypospadias were reared as females. In 39 subjects with
ambiguous genitalia, 18 of whom were raised as female and in whom
in depth information concerning their “sexuality” was sought, the
assigned sex was at least “satisfactory” in the majority.
Indeed, those reared as male had greater incidence of atypical
external genitalia and greater dissatisfaction with perceived
“body image”. In general, however, the outlook for normal
adult heterosexual adjustment reared as either male or female was
quite good in this group.
Until more complete data are available, these observations can
serve as the basis upon which to counsel the parents of a neonate
with male pseudohermaphroditism in regard to their choice in the
gender assignment of their offspring. Dr. Blizzard correctly
states that the “paternalistic” approach to medical practice is no
longer tenable.
In my opinion, in the context of this psychosocial emergency, it
remains extremely important that the experienced physician assist,
perhaps even guide, the parents through the decision making
process. In the absence of androgen insensitivity, complete
gonadal dysgenesis, deficiency of P450side chain cleavage
or 17-hydroxylase/17-20 lyase, and related disorders, it seems
most appropriate to rear the incompletely virilized male in the
masculine gender if there is at all sufficient penile corpus to do
so or to permit its surgical amplification.
Dr. Blizzard critically analyzes the current thinking concerning
the problem of when to perform reconstructive genital surgery in
the patient with male pseudohermaphroditism assigned to the female
gender.
In my opinion, he correctly rejects the extremist position that no
reconstruction be undertaken until the patient herself can
consent. Clearly, this approach will lead to great duress in
the lives of the patient and her parents. (One can barely
imagine the stress that a parent would be under in raising a child
whose gender may change or that of the child who will surely learn
at a surprisingly early age that her genitalia differ from those
of other girls.) While each child must be considered
individually, cliteroplasty during
infancy and vaginoplasty at adolescence seem reasonable in my
opinion once feminine gender has been assigned until the long-term
efficacy of earlier vaginal reconstructive techniques have been
evaluated.
Dr. Blizzard discusses the issue of intra-cultural differences in
attitude toward the problem of intersex and the challenging
question of whether all children with 46XX female
pseudohermaphroditism should be reared as females.
His thoughtful and insightful comments are seconded by this
writer, although my inclination is to rear all females with
virilizing congenital adrenal hyperplasia as girls.
Individualization of care and informed parental choice are the
keystones upon which management of the neonate with atypical
external genitalia must be based.
Readers who wish to be brought up-to-date concerning some of the
conundrums of intersex issues and what the current concepts are
concerning intersex issues will benefit from Dr. Blizzard’s
commentary.
Blizzard RM.
Pediatrics 2002;110(3):616-621.
Allen W. Root, MD
Dr. Blizzard’s Comment:
Comments about one’s commentary are not necessarily legitimate.
However, I comment relating the above abstract and editorial
comment by Dr. Root to the lead article in this issue by Dr. Sheri
Berenbaum. Her studies and
writings are always logical, intelligent, and scientifically
based. In her article, Dr. Berenbaum
demonstrates the applicability of my adjectives used to describe
her approaches to solving the conundrums of intersex. I
highly recommend each reader contemplate her description of the
complexities in this field. Hopefully others will approach
the conundrums of intersex in the same contemplative way as does
she.
Robert M. Blizzard, MD
