A Management Protocol for Gonad Preservation in Patients with Androgen Insensitivity Syndrome

Two cases of gonad retention in adolescent patients with complete androgen insensitivity syndrome (CAIS)

“…Individuals with CAIS who decide to retain their gonads and defer surgery do so for a variety of reasons, including the inconvenience of surgery, lack of a desire to take hormone replacements throughout their life, and fears of the risks of having surgery [ 9 ]. Recommendations for those wishing to defer surgery have included regular MRI or US imaging to monitor for signs of malignant change [ 4 , 5 , 10 ]. For those individuals diagnosed prenatally, parents should be counseled regarding the gonads and their role in puberty and allowing the individual to participate in the shared decision-making process regarding lifelong implications for treatment.…”

“…Deferring surgery is an option not widely discussed with patients with CAIS; however, care models are emerging that include consistent imaging and careful monitoring with the possibility of avoiding gonadectomy over the course of a patient’s lifetime [ 4 , 5 ].…”

“…Recommendations then included lifelong hormone replacement therapy. Care is shifting to defer early surgery in these individuals which includes a shared decision-making checklist and careful monitoring of the gonads [ 4 – 6 ]. The following cases describe the shared decision-making process used to care for two patients who chose to defer gonadectomy.…”

Laparoscopic Repositioning of Gonads from the Labia Majora or Inguinal Canal into the Abdominal Cavity in Pediatric Complete Androgen Insensitivity Syndrome Patients with Inguinal Hernia

“…In the past, prophylactic gonadectomy was performed in patients with AIS discovered during pediatric IH repair because of the risk of subsequent gonadal cancer [Dreuning et al, 2020]. Recent studies recommend preserving the gonads until puberty to maintain secondary sexual characteristics and bone mineral density in women [Döhnert et al, 2017;King et al, 2017;Weidler et al, 2019]. Invasive testicular germ cell tumors were reported at a low prevalence of <1% in patients with CAIS [Cools et al, 2005[Cools et al, , 2006.…”

“…There is a debate over the need for concurrent gonadectomy during IH surgery. The prevalent opinion is that gonadectomy should not be performed before puberty, but after puberty gonadectomy is determined through sufficient consultation between patients, parents, and medical staff [Weidler et al, 2019]. Because patient decisions are paramount, it is important to preserve the gonads until the age of self-determination.…”