The laparoscopic management of XXXX : Case series.

@entity1 @entity5 @entity5000 @entity4997 @entity4134 @entity444 @entity6126 @entity2588 @entity4903
@entity4997 , also known as 46 XY pure @entity444 , is a rare @entity4903 . Affected individuals are phenotypically female with female genitalia, normal Mullerian structures, absent testicular tissue, and a 46 XY chromosomal constitution. We report a series of eight cases of @entity4997 , of which six were managed by laparoscopic gonadectomy. The two other cases had to undergo an exploratory laparotomy in view of their presentation with @entity4134 . Two of the @entity1 were siblings. The chief presenting complaint was primary @entity2588 . Four @entity1 also presented with a history of poor development of secondary sexual characters. The average age at presentation was 16.19  2.85 years. The average height was 158.33   4.63 cm, and the average weight was 49.33  8.44 kg. Breast development was either Tanner 2 or 3 in four @entity1 , whereas three @entity1 had a Tanner 1 underdeveloped breasts. Axillary and pelvic hair was sparse in all the @entity1 . The vagina was well canalized in all the @entity1 . Hormonal evaluation revealed @entity6126 with a mean follicle-stimulating hormone (FSH) level of 95.81 mIU/L and a mean luteinizing (LH) level of 24.15 mIU/L. Imaging analysis revealed the presence of a small uterus in all the cases, except one. Bilateral ovaries were either not visualized or streak gonads were present. @entity4134 was detected in two of the six cases with raised carcinoembryonic antigen (CA) 125 levels in one case. Genetic analysis revealed a karyotype of 46 XY in six @entity1 , 46 XY/45 X in one, and the culture repeatedly failed in one @entity1 . Because of the risk of @entity5 , bilateral gonadectomy was performed in all cases. Histopathological analysis revealed that three of the six cases had @entity5000 . The @entity1 have been started on hormone replacement therapy. Laparoscopy is a minimally invasive modality for the definitive diagnosis and treatment of cases with @entity4997 . An early diagnosis of @entity4997 is possible during workup for primary @entity2588 before they present with @entity4134 .
