DISORDER OF SEX DEVOLPMENT: A CASE OF MISSED OPPORTUNITY

A 14 year boy born of nonconsanguineous parents presented to the panel of ekta institute of child health.
with frequent vomitiing his illness dated back to the neonatal period when he started having frequent episodes of non-bilious vomitting. managment by local practioner brought tempraory relief . At 4 months of  age the parents noticed empty sacroyotal sacs and the medical advice sought suggessted to " wait till 4 yrs of age" Abdominal sonography was done at that age showed intra abdominal gonads, child was subjected to surgery and the gonads were removed histology confirmed the gonads as overies the symptoms continued unabted .
At 12 year of age when the problem increased in severity , an abdominal sonography was done showed " rudimentary fallopian tubes and uterus and a cervix ending blindly".An abdominal laproscopy was done and these structures were removed .even the second surgery was failed to relieve his symptoms .

EKTA :
The growth parameters were below third percintile stretched phallic lenghth was 5.2 cm both sacrotal sacs were empty and confirmed  " Adernal hyperplasia" (CAH).A proper reconstrution surgery and replacement therapy would have allowed the child to be reared up as a normal female.Paradoxical signs are commonly seen during treatment of TB steroids have found to be useful in their treatment. They Stated that adverse drug reactions was only possibly related to Isoniazid.

Gurbinder Sharma