De Jong 1

Janelle De Jong

Dr. Shreibman

Phil 3109

01 September 2017

Ambiguous Genitals of the Neonate

The first thing usually asked after a baby is born is “is it a boy or a girl?” Most often this

can be answered definitively with one of the two choices. The appearance of a penis and scrotum

signifies a boy or external labia and a vagina, a girl, however sometimes a baby has external

genitalia and possibly inner reproductive organs that do not appear to fit exactly into male or

female categories. This may lead to great distress to the family and doctors. Many causes of

ambiguous genitals are not a medical emergency, but have commonly been treated as such in an

attempt to make babies fit into the ideal of being either a boy or girl. This paper will examine

reasons that may cause variations in genitalia, along with how and why they occur, and how the

response and actions taken have changed over time.

People born with or still with ambiguous genitals into adulthood may be grouped using

the term intersex. For all people who are intersex, somewhere in development, sexual

differentiation did not go as it normally does. This may also be called a disorder of sexual

development (DSD), an “incongruence between molecular, gonadal, and phenotypic sex” (Houk

& Lee, 2012). Failure to achieve sexual differentiation owing to defective gene expression or

abnormal external influences, or both, may result in the congenital anomaly of ambiguous

genitalia (Kenner 2003 541). Sexual differentiation is a sequential process directed by genes with

three stages: (1) fertilization (determination of chromosomal sex), (2) gonadal differentiation,

and (3) differentiation of phenotypic sex (internal ductal system and external genitalia). During
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the early weeks of development, all embryos have gonads with structures that could be either

male or female internal and external genitalia. To create male-specific development, a Y

chromosome and the testis-determining gene (SRY) on that chromosome must be expressed. This

directs the gonad to differentiate to a testis, which produces testosterone, which further

masculinizes the genitalia by enlarging the penis and fusing labia into a scrotum (Kenner 2014

264).

One cause of ambiguous genitals is Androgen Insensitivity Syndrome, or AIS. In an

individual with complete AIS, the body's cells are unable to respond to androgens, or "male"

hormones. Because cells fail to respond to testosterone, the genitals differentiate in the female,

rather than the male pattern, and Wolffian structures (epididymis, vas deferens, and seminal

vesicles) are absent (Chase). The most common cause of genital ambiguity in the newborn is

Congenital Adrenal Hyperplasia (CAH). About 1 in 10,000 to 18,000 children are born with

congenital adrenal hyperplasia, 90% of cases with ambiguous genitalia (Kenner 2014 269). In

CAH the adrenal glands (the glands on top of the kidneys that make various hormones and add

them to the blood stream) produces an excess of androgens. The exposure of an XX fetus to

androgens during a sensitive period of development causes the baby to be born with an enlarged

clitoris that may look like a small penis and fused labia that look very much like a scrotum, yet

they most likely have a uterus (Bloom 108). The person will always produce androgens so they

may develop masculine secondary sex characteristics, such as thick body hair, deep voice, and

high muscle mass unless they take hormone blockers or estrogen.

The treatment protocols for infants with intersex conditions, which have frequently

involved cosmetic genital surgery, were originally formalized by psychologist John Money in the

1950s. For Money and his collaborators, the problem of intersex was that there was no sex or
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gender between male or female, boy or girl. The notion was that in cases of intersex if gender

assignment was settled early by surgery, kids would grow up to be good believable and straight

girls and boys even if their genetics did not match their genitals. Under the theoretic leadership of

psychologist John Money, the Hopkins team believed that gender was all about nurture—that you

could make any child into a “real” girl or boy if you made their bodies look right early (before

about 18 months of age), and made them and their parents believe the gender assignment (Murphy

2435). John Money asserted that when children were assigned a gender at birth that did not match

their chromosomal sex, it was most likely that their adult sense of self would conform to their

assigned gender rather than to their chromosomal sex.

As this opinion spread throughout the developed world, surgeons performed cosmetic

genital surgeries on intersex children, believing this was necessary and effective. The premise

was that for small phallus it was easier to make a hole than make it bigger, and if left small a

man is doomed to live ashamed, apart, and alone (Bloom 105). Depending on the degree of

appearance, for XX females, reconstructive surgery was given to reduce the size of the clitoris,

separate the labia, and create a vaginal orifice capable of penetrative intercourse with a partner

with an average size penis. Enlargement of the vaginal cavity by metal dilators inserted by the

parents daily for six months, beginning two weeks postoperatively had to be done with monthly

dilation until seven-or eight-years-old to prevent narrowing or closure of the vaginal cavity

(Wong 1730). Some endocrinologists, meanwhile, manipulated patients’ hormones to try to get

the bodies of patients to do what they thought was necessary not just for physical health, but for

“psycho-social health (i.e., getting the body to look sexually ‘normal’)” (Feder 2).

In 1990 The American College of surgeons stated that the finding of ambiguous genitalia

in the newborn was a medical and social emergency (Bloom 101). To the late twentieth century,
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doctors across the country routinely treated neonatal cases of ambiguous genitals as medical

emergencies. The situation might not need medical care. The vast majority of intersex conditions

are not life threatening. Not everything has to be “fixed,” especially when the fix is based on social

concerns rather than medical necessity. Among the many causes of intersex, only a form of CAH

represents a real medical emergency in the newborn period. In this form of CAH the adrenal gland

cannot make cortisone which leads to metabolic problems affecting sodium balance. Called “salt

wasting” this can mean that during the first few weeks of life, infants fail to gain weight, and have

low sodium and high potassium levels in their blood. If left untreated cardiac arrest and death can

occur (Wong 1729). If begun early enough, administering cortisone is very effective in preventing

complications.

Many adults, including parents of children who have had “normalizing” surgeries have

spoken out about the physical and psychosocial damage that has resulted from them. Pediatric

surgery on the genitals almost inevitably produces scarring and loss of sensation. Some people

must do hormone replacement therapy for life because their small but otherwise functional testes

were removed. By contrast, there remains little evidence of the success of “corrective (or what

some even call reconstructive) genital surgeries” (Feder70).

Today, Money’s findings have been discredited. The system was and is literally sexist, it

treats children thought to be girls differently than children thought to be boys. The standards used

for genital anatomy are “arbitrary and illogical” (Houk & Lee, 2005). This does not mean doctors

intended to harm their patients. Good intentions are inadequate reasons to maintain a practice that

has shown to be unethical and unscientific (Feder 1). The surgeries were not necessary although

Money and others lied and said they were. Lying to patients is not only unethical, it is bad

medicine. Parents should consistently be told the truth (this includes providers being honest about
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uncertainty). Another major problem is that before performing the surgeries the bioethical

principle of providing informed consent was not performed. Parents were most often not given all

the information about why the surgery was being done, the failure rate of it, and the lack of support

for the surgery (Feder 70). The surgeries are not reversible and the risks may be substantial and

should only be taken if the patient has consented. Currently the decision to wait until puberty and

see what happens or what the person wants is gaining acceptance. This allows the person to decide

what they want to do with their body later in life, if anything. The primary obligation in medicine

is, first, to do no harm, and this new protocol, unlike those of previous centuries, seems to obey

that requirement (Reis xiv). Medical procedures necessary to sustain the physical health of a child

should be performed (Chase). There is no evidence that children who grow up with intersex

genitals are worse off.

Ambiguous genitals or intersex is a relatively common anatomical variation from the

standard male and female types. Intersex is neither a medical nor a “social pathology” (Dreger).

During the twentieth century, guided by narrowminded ideals, physicians performed genital

surgery and prescribed hormone replacement specifically to normalize the bodies of infants with

atypical sex. The concealment of ambiguous genitals that these interventions aim to achieve,

makes of the bodies the problem, when the problem should be not as one concerned with gender

and genitalia, but as an ethical problem. Ambiguous genitals is a problem located within those

who find intolerable the variation that their anatomies embody. Social distress is a reason to

change society, not the bodies of children. Ambiguous genitalia are not diseased, nor do they

cause disease; they just look different to the average.

I learned a lot about reasons why ambiguous genitalia occurs during my research for this

paper. I applied content I learned from previous courses, such as from genetics the SRY gene on
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the Y chromosome contributing male sex characteristics, and the adrenal glands producing

hormones into the bloodstream from hormones and behavior class. I first became interested in

this topic during my high school psychology class when my teacher showed my class video of a

man who was raised as a girl. He never felt comfortable as a girl and was relieved to find out that

he was given surgery as a baby to try to make him a girl, but genetically his makeup is male. I

wanted to learn more about why doctors would change the external appearance of the genitals.

To me another person’s anatomy and especially genitals are none of my business and it baffles

me that surgery was/is done to make genitals better suited for heterosexual intercourse.
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Works Cited

Bloom, Amy. Normal: Transsexual CEOs, Cross-Dressing Cops, and Hermaphrodites with Attitude.

New York: Random House, 2002. Print.

Chase, Cheryl. “What Is Intersex?”. Intersex Society of North America, ISNA, Online.

Dreger, Alice. “Shifting the Paradigm of Intersex Treatment”. Intersex Society of North America.

ISNA. Online.

Feder, Ellen. Making Sense of Intersex: Changing Ethical Perspectives in Biomedicine.

Bloomington, IN: Indiana University Press, 2014. eBook.

Kenner, Carole and Judy W. Lott. Comprehensive Neonatal Nursing Care. Springer Publishing

Company, 2014. eBook.

Kenner, Carole and Judy W. Lott. Comprehensive Neonatal Nursing: A Physiologic Perspective.

Philadelphia: Saunders, 2003. Print.

Murphy, Timothy F. “Gender and Sexual Identity (1995)”. In Encyclopedia of Bioethics, 3rd

Edition, Macmillan Reference USA. New York. 2004. Print.

Reis, Elizabeth. Bodies in Doubt: An American History of Intersex. Baltimore: Johns Hopkins

University Press, 2009. eBook.

Wong, Donna L, Marilyn J. Hockenberry, and David Wilson. Wong's Nursing Care of Infants and

Children. St. Louis, Mo: Mosby/Elsevier, 2011. Print.