Investigations:




Important Management points :-

Gender assignment :- The diagnosis and gender assignment is needed promptly but may have to be delayed for weeks until all the tests are available. Psychological support to the family is needed at this difficult time. Gender assignment is based on the potential for future sexual and reproductive function, nature of anatomic abnormalities and the feasibility of surgical correction. Virilized newborns with 46 XX karyotype should be raised as females. Individuals with 46XY with CAIS are also raised as females. In individuals with PAIS with less than 2.0 cms phallic length, response to exogenous androgens is considered favorable for male sex assignment if phallus enlarges to greater than 2.5 cms.

Risk of gonadal malignancy: Presence of Y chromosome in a child with intersex increases the risk of gonadoblastoma and dysgerminoma. Therefore if an XY child with ambiguity is raised as a female, the gonads should be removed. If raised as a male, the gonads should be brought down to the scrotum and examined at a regular interval.

Fertility :- Females with CAH can get naturally fertile provided monitored treatment is given throughout life. Patients with PAIS, gonadal dysgenesis or 17β HSD are all infertile. It is possible for XY individuals with a uterus to become pregnant through In Vitro Fertilization. In true hermaphrodites, few pregnancies are reported but paternity is rare.

Conclusions :- A child with intersex disorder needs an urgent referral to a place where expert opinion and laboratory support is readily available. A prompt diagnosis and management plan needs to be individualized in each case. Effective management can be offered to all children even if they present late. 

Last updated on 01-02-2005 Vol 2 Issue 2 Art # 6
Last created on 23-02-2001

How to cite this url
Khadilkar V.Intersex Disorders.Pediatric Oncall [serial online] 2005 [cited 2005 February 1];2. Art # 6. Available from: