Further observations on the syndrome of pure gonadal dysgenesis

The syndrome of pure gonadal dysgenesis covers eunuchoidal women with primary amenorrhea who are found at laparotomy to have aplastic gonads or complete absence of gonads. Early destruction of fetal gonads and, therefore, the lack of hormonal substances normally secreted by them, is the basis for this syndrome, which leads to the development of female eunuchoidal habitus, independent of the genetic sex of the patient. No clinical or histologic difference between cases of female, male, or abnormal karyotypes was found.