Abdominal exploration for UDT revealed Mullerian structures ovary, unicornuate uterus, fallopian tube and cervix on left side Fig. On Right side vas and vessels were found into deep inguinal ring. After counseling with the parents it was planned to rear this child as male due to predominant male phenotype.
The persistent Mullerian structures were excised and sent for histopathology. Biopsy was taken from Right testicle in order to find any dysgenesis or ovotesticular tissue. Biopsy report confirmed ovary, fallopian tube and uterus on left side. Right testicular biopsy showed normal histology with semineferous tubules without any dysgenesis or ovarian tissue. Disorder of sexual differentiation is the terms used for a child born without clear male or female phenotype. Proper gender assignment to a neonate born with DSD is a social emergency of the newborn period.
Infants and children born with DSD pose a diagnostic and therapeutic challenge to the clinicians. Success depends upon rapid and precise diagnosis, appropriate gender assignment, proper medical therapy and meticulous surgical technique [ 5 ]. The causes of true hermaphroditism remain enigmatic and the commonest presentation is an abnormal external genitalia ranging from normal male to normal female. In many of these cases such distinction may not be present and chordee, hypospadias and cryptorchidism may be noted.
Similar picture is found in our case. Other presenting symptoms are hematuria, amenorrhea, lower abdominal pain and inguinal hernia [ 6 , 7 , 8 ]. Documentation of location of gonads is important. In true hermaphrodites gonads are always asymmetrical with predominant testis descends and predominant ovary lies in the abdomen above the external ring as noted in index case.
Our patient was of lateral variety in which testis was on right side and ovary on left side. The choice of rearing hermaphrodite as male or female sex is governed by phallus size [ 9 ].
In our patient penis was of adequate size thus plan in consultation with parents was made to rear him as a male. All female structures were thus removed. A repair of hypospadias will be performed in the next stage. Prosthesis can be placed in left hemiscrotum for psychological comfort. True hermaphroditism is rarely associated with gonadal tumours, unlike in mixed gonadal dysgenesis, where the presence of a dysgenetic gonad predisposes to gonadal malignancy. However a few cases of malignancies like dysgerminoma and gonadoblastoma have been reported in true hermaphroditism [ 10 ].
Hence this patient will require close follow up to diagnose any malignancy arising in his remaining testis. Since the incidence of gonadal malignancy is low, estimated at 4. Our case was unique as chromosomal analysis was 46 XY, which is very rare in a case of true hermaphrodite DSD. National Center for Biotechnology Information , U. Therefore, it requires the adequate production and function of male hormones. The person must have both ovarian and testicular tissue.
This may be in the same gonad an ovotestis , or the person might have 1 ovary and 1 testis. The person may have XX chromosomes, XY chromosomes, or both. The external genitals may be ambiguous or may appear to be female or male. This condition used to be called true hermaphroditism. In most people with true gonadal intersex, the underlying cause is unknown, although in some animal studies it has been linked to exposure to common agricultural pesticides.
Many chromosome configurations other than simple 46, XX or 46, XY can result in disorders of sex development. These disorders do not result in a condition in which there is a discrepancy between internal and external genitalia. However, there may be problems with sex hormone levels, overall sexual development, and altered numbers of sex chromosomes.
Ideally, a team of health care professionals with expertise in intersex should work together to understand and treat the child with intersex and support the family. Parents should understand controversies and changes in treating intersex in recent years.
In the past, the prevailing opinion was that it was generally best to assign a gender as quickly as possible. This was often based on the external genitals rather than the chromosomal gender. Parents were told to have no ambiguity in their minds as to the gender of the child. Prompt surgery was often recommended.
Ovarian or testicular tissue from the other gender would be removed. In general, it was considered easier to reconstruct female genitalia than functioning male genitalia, so if the "correct" choice was not clear, the child was often assigned to be a girl. More recently, the opinion of many experts has shifted. Greater respect for the complexities of female sexual functioning has led them to conclude that suboptimal female genitalia may not be inherently better than suboptimal male genitalia, even if the reconstruction is "easier.
Chromosomal, neural, hormonal, psychological, and behavioral factors can all influence gender identity. Many experts now urge delaying definitive surgery for as long as is healthy, and ideally involving the child in the gender decision. Clearly, intersex is a complex issue, and its treatment has short- and long-term consequences. The best answer will depend on many factors, including the specific cause of the intersex.
It is best to take the time to understand the issues before rushing into a decision. An intersex support group may help acquaint families with the latest research, and may provide a community of other families, children, and adult individuals who have faced the same issues.
Different support groups may differ in their thoughts regarding this very sensitive topic. Look for one that supports your thoughts and feelings on the topic.
Please see information on the individual conditions. The prognosis depends on the specific cause of intersex. With understanding, support, and appropriate treatment, overall outlook is excellent.
If you notice that your child has unusual genitalia or sexual development, discuss this with your health care provider. How small does a penis have to be before it counts as intersex? Below we provide a summary of statistics drawn from an article by Brown University researcher Anne Fausto-Sterling. Note that the frequency of some of these conditions, such as congenital adrenal hyperplasia, differs for different populations.
These statistics are approximations.
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