Complete androgen insensitivity syndrome: the role of the endocrine surgeon.
Androgen insensitivity syndrome (AIS) is an X-linked recessive disorder characterized by varying degrees of feminization secondary to androgen receptor insensitivity. We report a case of a 34-year-old female with complete androgen insensitivity who presented with a history of primary amenorrhea and two intra-abdominal gonads found on CT scan. The patient underwent successful laparoscopic bilateral gonadectomy without complications. AIS is present in 1:20,000 to 64,000 males; complete androgen insensitivity is seen in 2-5:100,000 phenotypic females and should therefore be considered in any otherwise healthy female with abnormal pubertal development and infertility. Although controversial, most endocrinologists agree that gonadectomy may be performed after puberty with minimal risk of testicular neoplasm.
Alvarez
Nicolas R
NR
Dewitt Daughtry Family Department of Surgery, Division of Endocrine Surgery, University of Miami/Jackson Memorial Medical Center and Sylvester Comprehensive Cancer Center, Miami, Florida 33101, USA.
Lee
Theresa M
TM
Solorzano
Carmen C
CC
D000328:Adult; D013734:Androgen-Insensitivity Syndrome / Q000175:diagnosis* / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D010535:Laparoscopy / Q000379:methods; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009919:Orchiectomy / Q000379:methods*; D018570:Risk Assessment; D012720:Severity of Illness Index; D013736:Testicular Neoplasms / Q000517:prevention & control; D016896:Treatment Outcome