Complete androgen insensitivity syndrome: An uncommon presentation with acute abdomen

Abstract

uterus and only a partially formed vagina, and pubic and axillary hair is scant or absent. The increased testosterone production is secondary to increase in serum luteinizing hormone (LH) concentrations resulting from resistance to the feedback effect of androgens on LH secretion at the hypothalamic-pituitary level. Both the frequency and amplitude of pulses of LH secretion are increased. [6] Serum follicle stimulating hormone (FSH) concentrations are usually normal.We hereby submit a rare presentation of CAIS with acute abdomen due to torsion of intra-abdominal testis which required laparoscopic gonadectomy. Simultaneously, the rarity of such presentation and timing of removal of in situ gonad is also discussed. CASE REPORTA 17-year-old phenotypic female with history of primary amenorrhea, presented to the gynecologist with sudden onset of severe right lower quadrant abdominal pain for 1 day [ Figure 1]. The pain was not associated with any bowel or urinary symptoms. Examination revealed tenderness and guarding over the left lower quadrant of the abdomen. The rest of the abdomen was soft and non-tender. Urgent ultrasound examination was performed. The provisional diagnosis of torsion of the right ovary was made, with uterus not being visualized. Emergency laparoscopic gonadectomy was carried out and the surgeon too noted absence of uterus. Postoperatively, the patient made a satisfactory recovery AbsTRACT CAsE REPORT Access this article online Quick Response Code: Website:www.e-jams.org