Sex Reassignment at Birth

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جلد:
151
زبان:
english
رسالہ:
Archives of Pediatrics & Adolescent Medicine
DOI:
10.1001/archpedi.1997.02170400084015
Date:
March, 1997
فائل:
PDF, 1.04 MB
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Sex

Reassignment at Birth

Long-term Review and Clinical Implications
Milton Diamond, PhD; H. Keith

Sigmundson, MD

article is a long-term follow-up to a classic case reported in pediatric, psychiatric,
and sexological literature. The penis of an XY individual was accidentally ablated and
he was subsequently raised as a female. Initially this individual was described as developing into a normally functioning female. The individual, however, was later found
to reject this sex of rearing, switched at puberty to living as a male, and has successfully lived as
such from that time to the present. The standard in instances of extensive penile damage to infants
is to recommend rearing the male as a female. Subsequent cases should, however, be managed in
Arch Pediatr Adolesc Med. 1997;151:298-304
light of this new evidence.

This

Among the more difficult decisions physicians have to make involve

cases

of

am-

biguous genitalia or markedly traumatized genitalia. The decision as to how to
proceed typically follows this contempo-

rary advice: "The decision to raise the child
male centers around the potential for
the phallus to function adequately in later
sexual relations"1(p580) and "Because it is sim¬

as a

pler to construct a vagina than a satisfac¬
tory penis, only the infant with a phallus

of adequate size should be considered for
a male gender assignment. "2(pl955) These

management proposals depend on a theory
that says it is easier to make a good vagina

than a good penis and because the iden¬
tity of the child will reflect upbringing and
the absence of an adequate penis would be
psychosexually devastating, fashion the
perineum into a normal looking vulva and
vagina, and raise the individual as a girl.

Such clinical advice, concerned primarily
with surgical potentials, is relatively stan¬
dard in medical texts36 and reflects the cur¬
rent thinking of many physicians.7
This management philosophy is based
on 2 beliefs held strongly enough by pedia¬
tricians and other physicians to b; e consid¬
ered postulates: (1) individuals are psycho¬
sexually neutral at birth and (2) healthy

Department of Anatomy and Reproductive Biology, Pacific Center for Sex
and Society, University of Hawaii-Manoa, John A. Burns School of Medicine,
Honolulu (Dr Diamond); and the Department of Psychiatric Services, Ministry of
Health, Victoria, British Columbia (Dr Sigmundson).

psychosexual development is dependent on
the appearance of the genitals. These ideas
arise most strongly from the original work
of Money and colleagues.8"11<pp46"51)12 Typi¬
cal pronouncements from that research in¬
clude that "erotic outlook and orientation
is

psychological phenom¬
independent of genes and hormones

an autonomous

enon

and

a

permanent ineradicable

one as

For editorial comment
see page 224
well"9(pi397) ancj «[¿j] js more reasonable

to

suppose simply that, like hermaphrodites,
all the human race follow the same pat¬
tern, namely, of psychological undifferentiation at birth."10 The first postulate was
derived not from normal individuals, but

from hermaphrodites and pseudohermaph¬
rodites. The second had only anecdotal
support. Money13 no longer holds such ex¬
treme views, but his involvement in this particular case and acceptance of the
thesis was notable enough that it became a
totem in the lay press and a classic for the
academic and medical community. And, as
already noted, the textbooks have not kept
abreast of the new thinking.
REPORT OF A PATIENT

From the

The case involved a set of normal XY twins,
one of whom, at 8 months of age, had his

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penis accidentally burned to ablation during phimosis re¬
pair by cautery.11 After a great deal of debate, the child
was seen for consultation at The Johns Hopkins Hospi¬
tal, Baltimore, Md, and, following the beliefs mentioned
earlier, the recommendation was made to raise the child
as a girl. The pseudonym John will be used when refer¬
ring to this individual when living as a male and the pseud¬

onym Joan when living as a female. Orchiectomy and pre¬
liminary surgery followed within the year to facilitate
feminization. Further surgery to fashion a full vagina was
to wait until Joan was older. This management was moni¬
tored and reinforced with yearly visits to The Johns Hop¬
kins Hospital. The treatment was described as develop¬
ing successfully with John accepting life as Joan.11

Although the girl is not yet a woman, her record to date offers
convincing evidence that the gender identity gate is open at birth
for a normal child
sex

to
a

organs

no less than for one born with unfinished
who was prenatally over or underexposed
and that it stays open at least for something over

or one

androgen,

year after birth.12(p98)

how well all three of them (parents and child) suc¬
ceeded in adjusting to that decision."12
The effects of such reports were widespread. Soci¬
ology, psychology, and women's study texts were rewrit¬
ten to argue that, as Time magazine (Januarv 8» 1973)

reported,

This dramatic case
provides strong support that con¬
ventional patterns of masculine and feminine behavior can be
altered. It also casts doubt on the theory that major sex differ¬
ences, psychological as well as anatomical, are immutably set
by the genes at conception.
.

psychic trauma.

POSTULATE 1: INDIVIDUALS ARE
PSYCHOSEXUALLY NEUTRAL AT BIRTH

Mother recalls:
he had the surgery, the doctor said I should now
treating him as a girl, doing girl things, and putting him
in girl's clothes. But that was a disaster. 1 put this beautiful little
dress on him
and he [immediately tried] to rip it off; I think
he knew it was a dress and that it was for girls and he wasn't a
As

soon as

start

.

.

.

girl.

On the other hand, Joan could act quite feminine when
she wanted to and at approximately 6 years old was de¬
scribed as doing so, eg, his mother was quoted as say¬
ing: "One thing that really amazes me is that she is so
feminine. I've never seen a little girl so neat and tidy as
she can be when she wants to be. ."11(p119) More often,
however, Joan rejected such behavior. More commonly
she, much more so than the twin brother, would mimic
the father. One incident the mother related was typical.
When the twins were 4 or 5 years old, they were watch¬
ing their parents. Father was shaving and mother was ap¬
plying makeup. Joan applied shaving cream and pre¬
tended to shave. When Joan was corrected and told to
put on lipstick and makeup like mother, Joan said: "No,
I don't want no makeup, I want to shave."
Girl's toys, clothes, and activities were repeatedly
proffered to Joan and most often rejected. Throughout
childhood Joan preferred boy's activities and games; she
had little interest in dolls, sewing, or girl's activities. Ig¬
noring the toys she was given, she would play with her
brother's toys. She preferred to tinker with gadgets and
tools, dress up in men's clothing, and take things apart
to see what made them tick. She was regarded as a tom¬
boy with an interest in playing soldier. Joan did not shun
rough and tumble sports or avoid fights.
John recalls when the Joan of age 8 or 9 years wanted
an umbrella:
.

.

A follow-up stated: "The girl's subsequent history proves

.

munity to reduce the likelihood of others suffering his

.

.

.

.

Lay and social science writings still echo this case as do
medical texts.3"6,14 The following quote is typical:
The choice of gender should be based on the infant's anatomy,
the chromosomal karyotype. Often it is wiser to rear a ge¬
netic male as a female. It is relatively easy to create a vagina if
one is absent, but it is not possible to create a really satisfac¬
tory penis if the phallus is absent or rudimentary. Only those
males with a phallus of adequate size that will respond to tes¬
tosterone at adolescence should be considered for male rear¬
ing. Otherwise, the baby should be reared as a female.15(p396>
not

Our current article challenges those reports and advice.
It is based on a review of the medical clinical notes and

impressions of therapists originally involved with the case

and on contemporary interviews. One of us (H.K.S.) was
head of the psychiatric management team to which the
case was referred in the patient's home area. Although
the patient was assigned to the immediate care of female
psychiatrists to foster female identification and role mod¬
eling, H.K.S. maintained direct supervisory control of the
case. The unique character of this case attracted the at¬
tention of the Britsh Broadcasting Co and they invited
M.D. as a consultant.16 In 1994 and 1995, we collaboratively reinterviewed and recorded John, his mother, and
his wife to provide updated accounts of his progress. Find¬
ings are listed in chronological order under the appro¬
priate postulate for pediatrie sexual assignment. John him¬
self, while desiring to remain anonymous, strongly desires
his case history be made available to the medical com-

a couple of bucks and went to the store to take a look at
the umbrellas, and right beside the umbrellas was the toy sec¬
tion. I started to eyeball a machine gun. I said to myself, 'Do I
have enough money for that?'... I put the gun on the counter
and asked the clerk if I had enough money. She had that look
like 'You don't have enough but we'll let you go anyway.' I used
it to play army with my brother.

I had

Mother recalls Joan was in a dress at the time. The brother
often refused to let Joan play with his toys, so she saved
her allowance money and bought a truck of her own.
Joan's realization that she was not a girl jelled be¬
tween ages 9 and 11 years. John relates:
There were little things from early on. I began to see how dif¬
ferent 1 felt and was, from what I was supposed to be. But I didn't
know what it meant. I thought I was a freak or something
I looked at myself and said I don't like this type of clothing, I
don't like the types of toys I was always being given. I like hang¬
ing around with the guys and climbing trees and stuff like that
and girls don't like any of that stuff. I looked in the mirror and
[saw] my shoulders [were] so wide, I mean there [was] noth¬
ing feminine about me. I [was] skinny, but other than that, noth-

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....

ing. But that [was] how I figured it out. [I figured I was a guy]

but 1 didn't want to admit it. I figured I didn't want to wind up
opening a can of worms.

Joan knew she already had thoughts of suicide caused

by this sort of cognitive dissonance and did not want ad¬
ditional stress.

Joan fought the boys and the girls who were always
"razzing" her about her boy looks and her girl clothes.
She had no friends; no one would play with her. "Every

day I was picked on, every day I was teased, every day I
Mother
was threatened. I said enough is enough
relates that Joan was good looking as a girl. But, "When
he started moving or talking, that gave him away, and
the awkwardness and incongruities became apparent."
The other girls teased Joan so aggressively that she
often retaliated forcefully. At age 14 years one girl sat be¬
"

.

.

..

hind Joan and continued to hit her. The adult John dem¬
onstrated: "I grabbed her like that, by the shirt, and
rammed her round the wall like this, threw her on the
ground until the teacher grabbed me." This resulted
in Joan being expelled from school.
Despite the absence of a penis, Joan often tried to
stand to urinate. This made a mess as it was difficult to
direct the urine stream. Although she learned to sit and
void, she nevertheless continued to occasionally stand
and urinate. Despite admonitions against the behavior
and its untidiness, Joan persisted. At school, at age 14
years, she was caught standing to urinate in the girls' bath¬
room so often that the other girls refused to allow her
entrance. Mother recalls the other girls threatening to
"kill" her if she persisted. Joan would also sometimes go
to the boy's lavatory to urinate.
Joan was put on an estrogen regimen at the age of
12 years but rebelled against taking the hormones. They
made her "feel funny" and she did not want to feminize.
She would often dispose of her daily dose. She was un¬
happy at developing breasts and would not wear a bra.
Things came to a critical point at age 14 years. In dis¬
cussing her breast development with her endocrinologist she confessed, "I suspected I was a boy since the sec¬
ond grade." The physician, who personally believed Joan
should continue her medication and proceed as a girl,
used that opening to explore the possible male and fe¬
male paths available and what either one would mean.
The local psychiatric team had noticed Joan's prefer¬
ence for boy's activities and refusal to accept female sta¬
tus so they already had discussed among themselves the
possibility of accepting Joan's change back to a male. The
endocrinologist explored Joan's options with her. Shortly
thereafter, at age 14 years, Joan decided to switch to liv¬
ing as a male.
Joan was the daily butt of her peers' jibes and the
local therapists, having knowledge of her previous sui¬
cidal thoughts, went along with the idea of sex rereassignment. In a tearful episode followingjohn's prod¬
ding, his father told him of the history of what had
transpired when he was an infant and why. John recalls:
"All of a sudden everything clicked. For the first time
things made sense and I understood who and what I was."
John requested male hormone shots and gladly took
these. He also requested a mastectomy and phallo.

.

.

plasty. The mastectomy was completed at the age of 14
years; surgical procedures for phallus construction were
at ages 15 and 16 years. After the surgical procedures,
John adjusted well. As a boy he was relatively well ac¬
cepted and popular with boys and girls. At 16 years, to
attract girls, John obtained a windowless van with a bed

and bar. Girls, who as a group had been teasing Joan, now
began to have a crush on John. When occasions for sexual
encounters arose, however, he was reluctant to move erotically. When he told 1 girlfriend why he was hesitant, that
he was insecure about his penis, she gossiped at school
and this hurt John very much. Nevertheless, his peers
quickly rallied around him and he was accepted and the

girl rejected.

John's life subsequently was not unlike that of other
boys with an occult physical handicap. After his return
to

male

living he felt his attitudes, behaviors, and body

in concert in a way they had not been when living
as a girl. At age 25 years he married a women several years
his senior and adopted her children.

were

POSTULATE 2: HEALTHY PSYCHOSEXUAL
DEVELOPMENT IS INTIMATELY RELATED
TO THE APPEARANCE OF THE GENITALS
First in Baltimore and then with the local therapists prior

re-reassignment, Joan's expressed feelings of
being girl would draw ridicule. She would be told
something such as: "All girls think such things when
they're growing up." John recalls thinking: "You can't ar¬
gue with a bunch of doctors in white coats; you're just a
little kid and their minds are already made up. They didn't
want to listen." To ease pressures to act as a girl, Joan
would often not argue or fight the assignment and would
"go along."
Beginning at age 7 years, Joan began to rebel against
going for the consultations at The Johns Hopkins Hos¬
pital. Her reasons were discomfort and embarrassment
with forced exposure of her genitals and constant at¬
tempts, particularly after the age of 8 years, to convince
her to behave more like a girl and accept further vaginal
repair. This was always strongly resisted and led to re¬
to the sex
a
not

confrontations. To temper Joan's reluctance to
travel to the consultants, her parents combined such vis¬
its with vacation trips.
In Baltimore the consultants enlisted male-tofemale transsexuals to convince Joan of the advantages
of being female and having a vagina constructed. She was
so disturbed by this that in one instance Joan, at age 13
years, ran away from the hospital. She was found hiding
on the roof of a nearby building. After age 14 years, Joan
adamantly refused to return to the hospital. Joan then
came fully under the care of local clinicians. This group
consisted of several pediatricians, 2 pediatrie surgeons,
an endocrinologist, and a team of psychiatrists.
John recalls thinking, from preschool through el¬
ementary school, that physicians were more concerned
with the appearance of Joan's genitals than was Joan. Her
genitals were inspected at each visit to The Johns Hop¬
kins Hospital. She thought they were making a big issue
out of nothing and they gave her no reason to think oth¬
erwise. John recalls thinking: "Leave me be and then I'll
current

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be fine.. It's bizarre. My genitals are not bothering me;
I don't know why it is bothering you guys so much."
When asked what Joan thought of her genitals as a
youngster, John replied, "I didn't really have anything to
compare myself against other than my brother when we
were taking a bath." Mother confirmed that as a devout
family in a very conservative religious community there
would have been few opportunities for the twins to have
seen anyone else's genitals. Nudity was never accept¬
able. At their yearly visit to The Johns Hopkins Hospi¬
tal, the twins were made to stand naked for inspection
by groups of clinicians and to inspect each other's geni¬
talia. This experience, in itself, was recalled with strong
negative emotions. John's brother, decades later, recalls
the experience with tears.
John recalls frustration, which remains, at not hav¬
his
ing feelings and desires recognized. Without consid¬
eration of genitals, despite the obvious absence of a pe¬
nis, Joan nevertheless knew she was not a girl. When she
tried to express such thoughts the physicians would
change the subject. "[They] didn't want to hear what I
had to say but wanted to tell me how I should feel." Clini¬
cal notes from the time report Joan saying she felt "like
a trapped animal."
In middle school Joan had difficulty making friends.
Her clothes and demeanor did not jibe. Because of her
behavior, her peers teased her with names like "cave¬
man" and "gorilla." Few children would play with her.
None of Joan's peers knew anything of her genitals.
At first, as suggested by the consultants from The
Johns Hopkins Hospital, the local physicians and her par¬
ents continued to treat Joan as a girl, preparing her for
vaginal reconstructive surgery and life as a female. Psy¬
chotherapy, primarily by female therapists, was aimed at
reinforcing her female identity and redirecting her male
ideation. This course of action became increasingly dif¬
ficult because of Joan's growing conviction that she was
not right as a girl and anger at being treated like one. Joan's
reactions were not unlike those in posttraumatic stress
disorder, where the cause of the stress is not remem¬
bered. John recalls, "They kept making me feel as if I was
a freak."
John knew what the clinicians wanted and recog¬
..

nized it was not what he wanted. Beginning at age 14 years,
against the recommendations of the clinicians and fam¬
ily and without yet knowing of the original XY status,
Joan refused to live as a girl. Jeans and shirts, because of
their gender-neutral status, became her preferred man¬
ner of dress; boy's games and pursuits her usual activi¬
ties. Joan's daytime fantasies and night dreams during el¬
ementary school involved seeing herself "as this big guy,
lots of muscles and a slick car and have[ing] all kinds of
friends." She aspired to be a mechanic. She rejected re¬
quests to look at pictures of nude females, which she was
supposed to emulate. Rorschach and Thematic Apper¬
ception Tests at the time elicited responses more typical
of a boy than a girl. Her adamant rejection of female liv¬
ing and her improved demeanor and disposition when
acting as a boy convinced the local therapists of the cor¬
rectness of sexual re-reassignment.
Following the surgery for penile construction there
was

difficulty with urethral closure. Despite repeated at-

tempts

at

repair, the problem was never rectified. John
through a fistula at the base of his penis

urinates

now

while sitting down. Much of the penis is without sensa¬
tion, as are the areas of scarring from where the grafts
were taken.
John's first sexual partner was a girl. He was 18 years
old. While living as a girl and afterward as a boy, John
was approached sexually by males. He claims never to
have been attracted to them and his responses to such
questions are matter-of-fact and not homophobic. John
thinks his first recognizable sexual interest occurred at
about age 16 or 17 years, although he did recall wanting
to go see the "sexy" Rockettes in New York on one of
his trips to see the consultants.
Coitus is occasional with his wife. This frequency,
he claims, is sufficient for his needs but is less than his
wife would desire. They mostly pleasure each other with
a great deal of physical affection and mutual masturba¬
tion. John can have coital orgasm with ejaculation.
John recalls thinking it was small-minded of others
to think all his personality was summed up in the pres¬
ence or absence of a penis. He expressed it thus:
Doctor
said, 'it's gonna be tough, you're going to be picked
on, you're gonna be very alone, you're not gonna find anybody
[unless you have vaginal surgery and live as a female].' And I
thought to myself, you know I wasn't very old at the time, but
it dawned on me that these people gotta be pretty shallow if
that's the only thing they think I've got going for me; that the
.

.

.

only reason why people get married and have children and have
a productive life is because of what they have between their
legs. If that's all they think of me, that they justify my worth
by what I have between my legs, then I gotta be a complete loser.
.

.

.

GENERAL COMMENTS

why he did not accept being
female rather than fighting it. His answer was simple.
Doing so did not feel right. He wanted to please his par¬
ents and placate the physicians so he often went along
with their decisions, but the conflict between his feel¬
ings and theirs was mentally devastating and would have
led to suicide if he had been forced to continue.
The most often voiced and deeply felt emotion ex¬
pressed by Joan was always feeling different from what
was expected or desired by others. At first, as a toddler,
the feeling of being different was relatively amorphous.
Even as a preschooler, it shifted to clearly being differ¬
ent from girls. And later, in elementary school, she be¬
gan to feel not only different from girls but similar to boys.
Having a twin might have made this comparison much
easier for Joan than it might have been for a singleton.
Such a progression in thinking is common for atypical
individuals such as homosexual males and females,17 intersexed individuals, or those with ambiguous genita¬
As an adult John was asked
a

lia.18

The transition was gradual. When Joan thought she
might really be a boy instead of the girl her parents and
physicians told her she was, the psychic discord fright¬
ened her even though she had suspected since the sec¬
ond grade that it was true. When finally told the truth,
she was relieved because her feelings now made sense.

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John's anger at not having been told the truth from the
beginning persists.
Following John's sex re-reassignment, the family de¬

cided to disregard the clinical recommendation to move
from their family home. Instead they stayed and were open
about the change. Aside from the financial concerns, the
parents judged that the word would get out anyway. This
strategy seemed to work and John was accepted in a way
that Joan never was.
John was given testosterone treatment following his
return to male status. As is typical of many teenage boys,
John began to work out with weights. He blossomed into
an attractive muscular young man.
According to John's wife, "Before he came along I
was a lot tougher on the kids because I had to be. [Now]
John is the real hard one and I am the soft one. There is
no doubt who wears the pants in this family."
John is a mature and forward-looking man with a
keen sense of humor and balance. Although still bitter
over his experience, he accepts what happened and is try¬
ing to make the most of his life with support from his
wife, parents, and family. He has job satisfaction and is
generally self-assured.
COMMENT

Long-term follow-up of case reports are unusual but of¬

crucial. This update to a case originally accepted as
"classic" in fields ranging from medicine to the hu¬
manities completely reverses the conclusions and theory
behind the original reports. Cases of infant sex reassign¬
ment require inspection and review after puberty; 5- and
10-year postsex reassignment follow-ups are still insuf¬
ficient.
Possibly the initial impressions of the consult¬
ants1112 were appropriate at the time and Joan's behav¬
ior and thinking shifted with development. However, clini¬
cal notes and the impressions of the local physicians at
the time, as well as John's contemporary recollections,
indicate that he was at no time fully accepting of sex re¬
assignment. The local physicians expressed their reser¬
vations early on.16 Only when it became obvious to the
local team that the original management program of main¬
taining this child as a girl proved no longer tenable and
psychologically damaging, even life threatening, did they
revise their thinking.
It is also possible that interpretations from the early
years were mistaken. Results contrary to one's hypoth¬
eses and management plans are often difficult to see. Mani¬
festations of typical
behaviors would thus repeat¬
edly be interpreted as tomboyish. This seems to have been
the case for preferred activities, games, toys, and cloth¬
ing. The conclusions that hermaphrodites and pseudohermaphrodites offer a model for normal development
had been challenged before.1924 The implications of such
challenges, however, do not seem to have been accepted
ten

a

boy

integrated by most pediatricians or surgeons.7
Joan was repeatedly admonished for behaving like
a boy. Such management is in keeping with the belief that
any agreement with doubt expressed by the patient will
or

decrease the likelihood of a successful outcome.2528 To
contend that Joan did not accept her imposed sex be-

cause

of ambiguity in treatments, though, is circular rea¬

soning. No evidence of such ambiguity exists and the ini¬
tial reports held that the rearing was appropriate.11·12 It
is known, particularly from transsexuals, that casting
doubt on an individual's sexual identity usually forces
that person to introspection and eventual security re¬
garding a preferred life direction, even if it is contrary to
upbringing, parent's wishes, and social and cultural norms,
and if it results in less than adequate genitalia.29·30
In the case under consideration, the initial manage¬
ment protocol was predicated on postulates that consid¬
ered a male's self-image dependent on a functional pe¬
nis. Although such adequacy is important, there is no body
of data establishing its centrality.
Other considerations are in order. Gender reassign¬
ment as proposed for John, and the postulates on which
it is based, assumes the individual will learn to accept

rearing-appropriate, sex-typical behaviors, particularly

when the genitals are at issue. These situations range from
the urinary to erotic to narcissistic. Such behaviors, how¬
ever important, are only one aspect of an individual's to¬
tal sexuality. An individual's sexual profile comprises at
least 5 levels: gender patterns, reproduction, sexual iden¬
tity, arousal and physiological mechanisms, and sexual
orientation, recalled by the acronym PRIMO.31·32
The sex reassignment ofJohn to Joan addressed only
the gender patterns and gender roles to which he would
be subject. Expectations were that adjustments in his iden¬
tity and other levels would follow. Joan did indeed be¬
come aware of the social expectations consistent with the
female gender, but these were not in keeping with those
with which he felt comfortable. Standing to urinate, de¬
spite its housekeeping and social consequences, is a dra¬
matic display of preference. The sex reassignment obvi¬
ously failed in the area in which it was designed most to
succeed.
But it failed at the other 4 levels as well. The con¬
trast between the female gender-typical behaviors the
child was being asked to accept and her inner-directed
behavior preferences presented a discordance that de¬
manded resolution. Joan's analysis of the situation was
that she best fit in not as a girl but as a boy. Despite her
upbringing, Joan's sexual identity developed as a male.
Sex reassignment also obviously went against Joan's or
John's reproductive character. Castration removed any
reproductive capacity. Certainlyjohn was unaware of this
as a child. He resents this now and decries the loss. Cas¬
tration also removed the androgen source for sextypical mechanisms of sexual arousal and other physi¬
ological processes. His ability to ejaculate returned with
androgen treatment. The castration and surgical scar¬
ring, however, have reduced erotic sensitivity to the peri¬
neum and, consequently, reduced this pleasure. And no¬
tably, as many studies strongly indicate, sexual orientation
is prenatally organized or at least predisposed.33"40 The
sex reassignment did nothing to affect sexual orienta¬
tion. Joan remained totally gynecophilic despite being
reared as a girl.
Comments from John's parents reveal another im¬
portant consideration. With a sex reassignment they were
asked to make a dramatic psychological adjustment in
rearing an otherwise normal child. Mother herself re-

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quired psychiatric treatment to help manage her feel¬
ings. The penile ablation aside, the parents were more
comfortable dealing with their child's original sex and
the accident than with the reassigned sex. Although they
had tried to make a success of the sex reassignment, they
were supportive, while guilt ridden, when Joan decided

become John.
The last decade has offered much support for a bio¬
logical substrate for sexual behavior. In addition to the
genetic research mentioned, there are many neurologi¬
cal and other reports that point in this direction.31·3241"56
The evidence seems overwhelming that normal humans
are not psychosexually neutral at birth but are, in keep¬
ing with their mammalian heritage, predisposed and bi¬
ased to interact with environmental, familial, and social
forces in either a male or female mode. This classic case
demonstrates this. And the fact that this predisposition
was particularly expressed at puberty, a critical period,
is logical and has been predicted.20,44
Although this article deals with a classic case of sex
reassignment often cited in the literature, follow-up to
related cases are available. Reilly and Woodhouse57 de¬
scribed 20 patients with micropenises who were reared
as boys. None of them had any doubt as to the correct¬
ness of the assignment as males. Other reports describe
males originally reassigned as females who switched back
and successfully lived as males, despite the absence of a
normal penis.19·32,58"62 Several of these cases offer find¬
ings similar to ours, including the ages at which various
milestones were passed, feelings developed, and the re¬
assignment challenged.59,60 Another more recent case il¬
lustrates this.
Reiner63 described an adolescent Hmong immi¬
grant who precipitously dropped out of school at age 14.
On subsequent interview she declared, though she was
unequivocally raised as a girl from birth, "I am not a girl,
I am a boy." Findings from a physical examination re¬
vealed a 46-chromosome, XY male with mixed gonadal
dysgenesis with a female-appearing pelvis with clitoral
hypertrophy. Her school friends had all been boys. She
enjoyed rough and tumble play, avoided dolls and girl's
activities, and dressed in a gender-neutral or boy's way.
Her feelings of being different, being a boy, developed
to

from the age of 8 years and came to a head at 14 years.
Treatment involved surgery and endocrine therapy. This
individual, after a period of some depression, progres¬
sively developed into a gynecophilic, sexually active male.
These cases of successful gender change, as well as
the present one, also challenge the belief that such a switch
after the age of 2 years will be devastating. Indeed, in these
it was salutary.
It must be acknowledged that cases of males accept¬

cases

ing life as females after the destruction of their penises
has been reported.64 These reports, however, do not de¬
tail the individuals' sexual or personal lives.
CONCLUSIONS

Considering this case follow-up, and as far as an extensive
literature review can attest, there is no known case where
a 46-chromosome, XY male, unequivocally so at birth, has
ever easily and fully accepted an imposed life as an andrò-

philic female regardless of physical and medical interven¬
tion. True, surgical reconstruction of traumatized male or
ambiguous genitalia to those of a female is mechanically
constructing a penis. But the attendant sex re¬
assignment might be an unacceptable psychic price to pay.
easier than

Concomitantly, no support exists for the postulates that
are psychosexually neutral at birth or that
healthy psychosexual development is dependent on the ap¬
pearance of the genitals. Certainly long-term follow-up on
individuals

other cases is needed.
In the interim, however, we offer new guidelines.
We believe that any 46-chromosome, XY individual born
normal and with a normal nervous system, in keeping
with the psychosexual bias thus prenatally imposed,
should be raised as a male. Surgery to repair any genital
problem, although difficult, should be conducted in keep¬
ing with this paradigm. This decision is not a simple one
to make7,13,18,63,65"67 and analysis should continue.
As parents will still want their children to be and
look normal as soon after birth or injury as possible, phy¬
sicians will have to provide the best advice and care con¬
sistent with current knowledge. We suggest referring the
parents and child to appropriate and periodic long-term
counseling rather than to immediate surgery and sex re¬
assignment, which seems a simple and immediate solu¬
tion to a complicated problem. With this management,
a male's predisposition to act as a boy and his actual be¬
havior will be reinforced in daily interactions and on all
sexual levels and his fertility will be preserved. Social dif¬
ficulties may reveal themselves as puberty is experi¬
enced. However, there is no evidence that with proper
counseling and surgical repair when best indicated, ad¬
justment will not be managed as well as teenagers man¬
age other severe handicaps. Future reports will deter¬
mine if we are correct.

Accepted for publication September 5, 1996.
Corresponding author: Milton Diamond, PhD, Uni¬
versity of Hawaii-Manoa, John A. Burns School of Medi¬
cine, 1951 East-West Rd, Honolulu, HI 96822.
REFERENCES
1. Duckett JW, Baskin LS. Genitoplasty for intersex anomalies. Eur J Pediatr. 1993;

152(suppl 2):580-584.

2. Perlmutter AD, Reitelman C. Surgical management of intersexuality. In: Walsh
PC, Retik AB, Stamey TA, Vaughan JR, eds. Campbell's Urology. 6th ed. Philadelphia, Pa: WB Saunders Co; 1992:1951-1966.
3. Behrman RE, Kliegman RM. Nelson Essentials of Pediatrics. 2nd ed. Philadelphia, Pa: WB Saunders Co; 1994:636-637.
4. Blethen SL, Weldon VV. Disorders of external genitalia differentiation. In: Kelly
VC, ed. Practice of Pediatrics. Philadelphia, Pa; Harper & Row Publication Inc;

1985:1-23.
5. Catlin EA, Crawford JD. Neonatal endocrinology. In: Oski FA, ed. Principles
and Practices of Pediatrics. Philadelphia, Pa: JB Lippincott; 1990:420-429.
6. Ratzan SK. Endocrine & metabolic disorders. In: Dworkin PH, ed. Pediatrics.
3rd ed. Baltimore, Md: Williams & Wilkins; 1996:523-565.
7. Kessler SJ. The medical construction of gender: case management of intersexed infants. Signs: J Women Culture Soc. 1990;16:3-26.
8. Money J, Hampson JG, Hampson JL. An examination of some basic sexual
concepts: the evidence of human hermaphroditism. Bull Johns Hopkins Hosp.

1955;97:301-319.

9.

Money J. Sex hormones and other variables in human eroticism. In: Young
WC, ed. Sex and Internal Secretions. 3rd ed. Baltimore, Md: Williams & Wilkins;
1961:1383-1400.

Downloaded From: http://archpedi.jamanetwork.com/ by a Columbia University User on 05/27/2015

10.
11.
12.

13.

14.

15.
16.
17.

18.
19.
20.
21.

22.
23.
24.

Money J. Cytogenetic and psychosexual incongruities with a note on spaceform blindness. Am J Psychiatry. 1963;119:820-827.
Money J, Ehrhardt AA. Man and Woman/Boy and Girl. Baltimore, Md: Johns
Hopkins University Press; 1972.
Money J, Tucker P. Sexual Signatures: On Being a Man or Woman. Boston,
Mass: Little Brown & Co Inc; 1975:95-98.
Money J. Sex Errors of the Body and Related Syndromes: A Guide to Counseling Children, Adolescents, and Their Families. 2nd ed. Baltimore, Md: Paul
H Brookes Publishing Co; 1994:132.
Burg FD, Merrill RE, Winter RJ, Schaible DH. Treatment of Infants, Children
and Adolescents. Philadelphia, Pa: WB Saunders Co; 1990:8-9.
Donahoe PK, Hendren WHI. Evaluation of the newborn with ambiguous genitalia. Pediatr Clin North Am. 1976;23:361-370.
Diamond M. Sexual identity, monozygotic twins reared in discordant sex roles
and a BBC follow-up. Arch Sex Behav. 1982;11:181-185.
Savin-Williams RC. Self-labeling and disclosure among gay, lesbian and bisexual youths. In: Green RJ, Laird J, eds. Lesbians and Gays in Couples and
Families. San Francisco, Calif: Jossey-Bass Inc Pubs; 1996:153-182.
Diamond M. Sexual Identity and Sexual Orientation in Children With Traumatized or Ambiguous Genitalia. J Sex Res. In press.
Cappon D, Ezrin C, Lynes P. Psychosexual identification (psychogender) in
the intersexed. Can Psychiatry J. 1959;4:90-106.
Diamond M. A critical evaluation of the ontogeny of human sexual behavior.
Q Rev Biol. 1965;40:147-175.
Roth M, Ball JRB. Psychiatric aspects of intersexuality. In: Armstrong CN, Marshall AJ, eds. Intersexuality: In Vertebrates Including Man. London, England:
Academic Press Inc Ltd; 1964:395-443.
Money J, Zuger B. Critique and rebuttal. Psychosom Med. 1970;3:463-467.
Zuger B. Gender role determination: a critical review of the evidence from hermaphroditism. Psychosom Med. 1970;32:449-463.
Zuger B. Comments on 'gender role differentiation in hermaphrodites.' Arch
Sex Behav.

1975;4:579-581.

25. Lev-Ran A. Gender role differentiation in hermaphrodites. Arch Sex Behav. 1974;
3:391.
26. Money J. Hormones, hormonal anomalies, and psychological health-care. In:
Kappy MS, Blizzard RM, Migeon CJ, eds. Wilkin's Diagnosis and Treatment of
Endocrine Disorders in Childhood and Adolescence. 4th ed. Springfield III: Charles
C Thomas Publishers; 1994;1141-1178.
27. Stoller RJ. The Intersexed patient\p=m-\counseland management. In: Wahl CW,
ed. Sexual Problems: Diagnosis and Treatment in Medical Practice. New York,
NY: Free Press; 1967:149-162.
28. Stoller RJ. Sex and Gender: On the Development of Masculinity and Femininity. New York, NY: Science House; 1968:231-240.
29. Diamond M. Self-testing: a check on sexual levels. In: Bullough B, Bullough
VL. eds. Cross Dressing and Transgenderism. Buffalo, NY: Prometheus Books.
In press.
30. Diamond M. Self-testing among transsexuals: a check on sexual identity.
J Psychol Hum Sex. 1996;8:61-82.
31. Diamond M. Some genetic considerations in the development of sexual orientation. In: Haug M, Whalen RE, Aron C, Olsen KL, eds. The Development of
Sex Differences and Similarities in Behavior. Dordrecht, the Netherlands: Kluwer Academic Publishers; 1993:291-309.
32. Diamond M. Biological aspects of sexual orientation and identity. In: Diamant
L, McAnulty R, eds. The Psychology of Sexual Orientation, Behavior and Identity: A Handbook. Westport, Conn: Greenwood Press Inc; 1995:45-80.
33. Bailey JM, Pillard RC. A genetic study of male sexual orientation. Arch Gen

Psychiatry. 1991;48;1089-1096.

43. Diamond M. Genetic-endocrine interactions and human psychosexuality. In:
Diamond M, ed. Perspectives in Reproduction and Sexual Behavior. Bloomington, Ind: University of Indiana Press; 1968:417-443.
44. Diamond M. Sexual identity and sex roles. In: Bullough V, ed. The Frontiers of
Sex Research. Buffalo, NY: Prometheus Books; 1979:33-56.
45. Diamond M. Bisexualit\l=a"\taus biologischer sicht. In: Haeberle EJ, Gindorf R,
eds. Bisexualit\l=a"\ten:Ideologie und Praxis des Sexualkontaktes mit beiden Geschlectern. Stuttgart, Germany: Gustav/Fischer Verlag; 1994:41-48.
46. Gorski RA, Gordon JH, Shrayne JE, Southam AM. Evidence for a morphological sex difference within the medial preoptic area for the rat brain. Brain Res.

1978;148:333-346.
47. Gorski RA. Hormone-induced sex differences in hypothalamic structure. Bull
Tokyo Metrop Inst Neurosci. 1988;16:67-90.
48. Hines M. Gonadal hormones and human cognitive development. In: Balthazart
J, ed. Hormones, Brain and Behaviour in Vertabrates: 1. Sexual Differentiation,
Neuroanatomical Aspects, Neurotransmitters and Neuropeptides. Farmington,
Conn: S Karger AG; 1990:51-63.
49. Hines M. Hormonal and neural correlates of sex-typed behavioral in human
beings. In: Haug M, Whalen RE, Aron C, Olsen KL, eds. The Development of
Sex Differences and Similarities in Behavior. Dordrecht, the Netherlands: Kluwer Academic Publishers; 1993:131-149.
50. LeVay S. A difference in hypothalamic structure between heterosexual and homosexual men. Science. 1991;253:1034-1037.
51. LeVay S. The Sexual Brain. Cambridge, Mass: MIT Press; 1993.
52. LeVay S, Hamer DH. Evidence for a biological influence in male homosexuality. Sci Am. 1994:May:44-49.
53. Swaab DF, Fliers E. A sexually dimorphic nucleus in the human brain. Science.

1985;228:1112-1115.
54. Swaab DF, Hofman MA. Sexual differentiation of the human hypothalamus:
ontogeny of the sexually dimorphic nucleus of the preoptic area. Dev Brain
Res. 1988;44:314-318.
55. Swaab DF, Hofman MA. An enlarged suprachiasmatic nucleus in homosexual
men. Brain Res. 1990;537:141-148.
56. Swaab DF, Gooren LJG, Hofman MA. Brain research, gender and sexual orientation. J Homosex. 1995;28:283-301.
57. Reilly JM, Woodhouse CRJ. Small penis and the male sexual role. J Urol. 1989;
142:569-572.
58. Burns E, Segaloff A, Carrera GM. Reassignment of sex: report of 3 cases.
J Urol. 1960;84:126.
59. Dicks GH, Childers AT. The social transformation of a boy who had lived his
first fourteen years as a girl: a case history. Am J Orthopsychaitry. 1934;4:
508-517.
60. Ghabrial F, Girgis SM. Reorientation of sex: report of two cases. Int J Fertil.
61.

Bailey JM,

11-32.

Khupisco V. The tragic boy who refused to be turned into a girl. In: Sunday
Times Johannesburg. May 21, 1995.
63. Reiner WG. Case study: sex reassignment in a teenage girl. J Am Acad Child
Adolesc Psychiatry. 1996;35:799-803.
64. Gearhart JP. Total ablation of the penis after circumcision with electrocautery:
a method of management and long-term followup. J Urol. 1989;142:799-801.
65. Fausto-Sterling A. The five sexes: why male and female are not enough. Science.

66.

Sex Behav. 1982;11:511-520.

38. Pillard R, Weinrich J. Evidence of familial nature of male
Gen Psychiatry. 1986;43:808-812.

1962;7:249-258.
Hoenig J. The origins of gender identity. In: Steiner WB, ed. Gender Dysphoria: Development, Research, Management. New York, NY: Plenum Press; 1985:

62.

Pillard RC, Neale MC, Agyei Y. Heritable factors influence sexual
orientation in women. Arch Gen Psychiatry. 1993;50:217-223.
35. Bailey JM, Bell AP. Familial aggregation of female sexual orientation. Behav
Genet. 1993:23:312-322.
36. Hamer DH, Hu S, Magnuson VL, Hu N, Pattatucci AML. A linkage between
DNA markers on the X chromosome and male sexual orientation. Science. 1993;
261:321-327.
37. Pillard R, Poumadere J, Carretta R. A family study of sexual orientation. Arch
34.

Homosexuality, type 1: an Xq28 phenomenon. Arch Sex Behav.
1995;24:109-134.
40. Whitam F, Diamond M, Martin J. Homosexual orientation in twins: a report on
61 pairs and 3 triplet sets. Arch Sex Behav. 1993;22:187-206.
41. Allen LS, Hines M, Shryne JE, Gorski RA. Two sexually dimorphic cell groups
in the human brain. J Neurosci. 1989;9:497-506.
42. Allen LS, Gorski RA. Sexual orientation and the size of the anterior commissure in the human brain. Proc Natl Acad Sci USA. 1992;89:7199-7202.
39. Turner WJ.

homosexuality.

Arch

67.

1993;1993:20-25.

Meyer-Bahlburg HFL. Gender identity development in intersex patients. Child
Adolesc Psychiatry Clin North Am. 1993;2:501-511.
Zucker KJ, Bradley SJ. Gender Identity Disorder and Psychosexual Problems
in Children and Adolescents. New York, NY: Guilford Press; 1995:265-282.

Downloaded From: http://archpedi.jamanetwork.com/ by a Columbia University User on 05/27/2015