Then rare reproductive system disorders

Both male and female genitalia play a key role not only for procreation, but also recreational purposes. However, sometimes they may have anomalies that may hinder them from achieving their intended purpose as Milliam Murigi outlines.

Uterine Didelphys

Uterine Didelphys is a disorder present before birth in which a female develops two uteruses instead of one.

Sometimes there is only one cervix for both wombs, other times each womb has a cervix.

Often, the vagina in women with a double uterus is divided into two separate openings by a thin membrane.

The best thing is that it’s entirely possible for women with a double uterus to carry a baby to term.

However, the condition does come with an increased risk of miscarriage or premature labour.

Swyer syndrome

This is a rare disorder characterised by the failure of sex glands (that is testicles or ovaries) to develop.

Swyer syndrome is classified as a Disorder of Sex Development (DSD), which encompasses any disorder in which chromosomal, gonadal, or anatomic sex development is abnormal.

Girls with Swyer syndrome have an XY chromosomal make-up (as boys normally do) instead of an XX chromosomal make-up (as girls normally do).

Despite having the XY chromosomal make-up, girls with Swyer syndrome look female and have functional female genitalia and structures including a vagina, uterus, and fallopian tubes.

Girls with Swyer syndrome lack sex glands (ovaries). Instead of sex glands, women with Swyer syndrome have “gonadal streaks”, in which the ovaries do not develop properly (aplasia) and are replaced by functionless scar (fibrous) tissue.

Because they lack ovaries, girls with Swyer syndrome do not produce sex hormones and will not undergo puberty unless treated using hormone replacement therapy.

Cervical agenesis

Cervical agenesis occurs when the girl’s reproductive system fails to develop fully while in the womb making the child be born without a cervix, the opening at the bottom of the uterus that connects to the vagina. Other reproductive organs may also be missing or smaller than usual.

The cause of this abnormal foetal development is not yet known. Signs you have this disorder include failure to start having periods at puberty (primary amenorrhea) as well as abdominal pain. 

Vaginal agenesis

This occurs when the girl’s reproductive system fails to develop fully in the womb making the child to be born without a vagina.

This disorder affects one out of 5,000 to 7,000 female infants. Though sometimes it is recognised at birth, most of the time, the condition isn’t diagnosed until puberty.

Young women with this disorder have normal ovaries and normal external genitalia and thus go through puberty and develop breasts, underarm and pubic hair, except they will not have periods.

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH)

This is a disorder that occurs in females and causes the vagina and uterus to be underdeveloped or absent, although external genitalia is normal.

Affected women usually do not have menstrual periods due to the absent uterus and are unable to carry a pregnancy.

However, they may be able to have children through assisted reproduction.

Penile agenesis

Penile agenesis is a birth anomaly in humans, occurring about once in five to six million male births, in which a male child is born without a penis.

This disorder occurs often as a consequence of testicular agenesis (lack of testes), but the reverse is never the case.

Since the problem for people with penile agenesis is the absence of a urinary outlet, the urethra can be surgically redirected to the rim of the anus immediately after birth to enable urination and avoid consequent internal irritation from urea concentrate. 

Diphallia

Also known as penile duplication (PD), diphallic terata, or diphallasparatus is an extremely rare developmental abnormality in which a male baby is born with two penises.

The first reported case was by Johannes Jacob Wecker in 1609 and since then, about one hundred cases have been reported to date.

The two external genitalia may vary in size and shape, either lying beside each other in a sagittal plane or one above the other in a frontal plane.

Treatment is a case-by-case analysis taking into account considerations for medical and ethical reasons and involves surgical excision of the non-functioning penis.

Aphallia

This is a congenital malformation, especially for intersex children in which the phallus (penis or clitoris) is absent.

Aphallia has no known cause, it is not linked to deficient hormone amounts or action, but rather to a failure of the foetal genital tubercle to form between three and six weeks after conception.

It is a rare condition, with only approximately 60 cases reported as of 1989, and 75 cases as of 2005. 

Anorchism

Also called anorchidism, this is a disorder of sex development in which a person with XY karyotype, which usually corresponds to the male sex, is born with one or without testes.

This disorder is rare and is associated with the absence of various other structures of the spermatic tract.

Turner syndrome 

Also called gonadal dysgenesis this is a female condition in which a female is born with only one X chromosome instead of two.

Ovaries for girls with this disorder develop normally at first, but egg cells (oocytes) usually die prematurely and most ovarian tissue degenerates before birth.

Many affected girls do not undergo puberty unless they receive hormone therapy, and most are unable to conceive, hence are infertile.