title: Umbilical KeyPort bilateral laparoscopic orchiectomy in patient with complete androgen insensitivity syndrome.
abstract: RESULTS
  A 22-year-old woman with complete androgen insensitivity syndrome (CAIS) presenting with primary amenorrhea and normal female external genitalia was referred for laparoscopic gonadectomy. She had been diagnosed several years earlier but was reluctant to undergo surgery. Case
  
  
  OBJECTIVE
  Diagnosis of this X-linked recessive inherited syndrome characterizes by disturbance of virilization in males with an AR mutation, XY karyotipe, female genitalia and severely undescended testis with risk of malignization. The optimal time to orchidectomy is not settled; neither the real risk of malignancy in these patients. Early surgery impacts development of a complete female phenotype, with enlargement of the breasts. Based on modern diagnostic imaging using DCE-MRI and surgical technology with single port laparoscopic access we hypothesize that the optimum time for gonadectomy is not at the time of diagnosis, but once feminization has completed.
  An umbilical laparoendoscopic single-site access for bilateral gonadectomy appears to be the first choice approach as leaves no visible incision and diminishes the psychological impact of surgery in a patient with CAIS absolutely reassured as female. KeyPort, a single port access with duo-rotate instruments developed by Richard Wolf facilitates this surgery and allows excellent cosmetic results.
authors.affiliation: Servicios de Urologia, Genetica y Anatomia Patologica, Hospital Universitario de Getafe, Madrid, Spain.
authors.forename: Felipe P
authors.initials: FP
authors.lastname: Andrade
authors.forename: Pedro M
authors.initials: PM
authors.lastname: Cabrera
authors.forename: Felipe
authors.initials: F
authors.lastname: Cáceres
authors.forename: Belen
authors.initials: B
authors.lastname: Gil
authors.forename: José M
authors.initials: JM
authors.lastname: Rodríguez-Barbero
authors.forename: Javier C
authors.initials: JC
authors.lastname: Angulo
mesh_terms: D013734:Androgen-Insensitivity Syndrome; D005260:Female; D006801:Humans; D010535:Laparoscopy / Q000295:instrumentation / Q000379:methods*; D008297:Male; D009919:Orchiectomy / Q000295:instrumentation / Q000379:methods*; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014472:Umbilicus; D055815:Young Adult