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  <front>
    <journal-meta>
      <journal-title-group>
        <journal-title>Journal of Medical Insight</journal-title>
      </journal-title-group>
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      <issn pub-type="epub">2373-6003</issn>
      <publisher>
        <publisher-name>JoMI</publisher-name>
        <publisher-loc>Boston, Massachusetts</publisher-loc>
      </publisher>
    </journal-meta>
    <article-meta>
      <article-id pub-id-type="publisher-id">313</article-id>
      <article-id pub-id-type="doi">10.24296/jomi/313</article-id>
      <article-categories>
        <subj-group>
          <subject>Research article</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Prophylactic Laparoscopic Bilateral Gonadectomy for Complete Androgen Insensitivity Syndrome</article-title>
      </title-group>
      <contrib-group/>
      <pub-date pub-type="ppub">
        <year>2021</year>
      </pub-date>
      <volume>2021</volume>
      <issue>12</issue>
      <permissions>
        <copyright-statement>2017 Journal of Medical Insight</copyright-statement>
        <copyright-year>2017</copyright-year>
        <license xlink:href="https://jomi.com/license">
          <license-p>
            You may create an account, or sign in to gain temporary access for evaluation purposes.
                    To maintain access: please let your librarian know you would like a subscription or send us an email at subscribe@jomi.com and we will forward your feedback to your librarian.
            <uri xlink:href="https://jomi.com/license"/>
          </license-p>
        </license>
      </permissions>
      <self-uri content-type="html" xlink:href="https://jomi.com/article/313/prophylactic-laparoscopic-bilateral-gonadectomy-for-complete-androgen-insensitivity-syndrome">Content is available at https://jomi.com/article/313/prophylactic-laparoscopic-bilateral-gonadectomy-for-complete-androgen-insensitivity-syndrome</self-uri>
      <abstract>
        <p>Androgen insensitivity syndrome (AIS) is a rare condition caused by an X-linked mutation of the androgen receptor with an estimated incidence of 1–5 per 100,000 individuals. Varying degrees of presentation exist for complete, partial, or mild depending on the severity of androgen resistance. Patients with complete AIS (CAIS) are born phenotypically female but have male XY chromosomes and testes instead of ovaries. They exhibit normal secondary female sex characteristics such as breast development and external female genitalia but lack a uterus and other Müllerian duct structures due to testicular production of Müllerian-inhibiting factor (MIF). Due to androgen-resistance, androgen-dependent Wolffian duct products fail to develop such as the epididymis, vas deferens, and the seminal vesicles. These patients often present either during infancy with inguinal hernias or sublabial masses, or during adolescence with primary amenorrhea. On physical exam, they will typically have normal breast development, lack pubic or axillary hair, and will have a blind-ending vaginal pouch of varying vaginal lengths. Diagnostic work-up is often conducted using ultrasound or MRI, serum hormone levels, and karyotype analysis. 
For patients with CAIS, their testes can be located within the inguinal canal, sublabially or intra-abdominally. Following puberty, patients with intra-abdominal testes are at a 15% increased risk (range 0–22%) of developing germ cell tumors (GCT). Management consists of prophylactic gonadectomy with subsequent hormone replacement therapy (HRT) to maintain normal pubertal development and promote adequate bone health. The debate regarding the timing of prophylactic gonadectomy is ongoing with some patient support groups arguing against gonadectomy citing concerns with long-term hormone therapy and the desire to preserve fertility. The current convention promotes delaying gonadectomy until after physiologic puberty has been achieved as the risk of developing prepubertal GCT is relatively low (0.8–2%). We outline the presentation, diagnosis, intraoperative techniques, and postoperative considerations for managing CAIS via bilateral laparoscopic gonadectomy.
</p>
      </abstract>
      <kwd-group>
        <kwd>gonadectomy</kwd>
        <kwd>laparoscopic gonadectomy</kwd>
        <kwd>prophylactic surgery</kwd>
        <kwd>phrophylactic gonadectomy</kwd>
        <kwd>CAIS</kwd>
        <kwd>complete androgen insensitivity syndrome</kwd>
        <kwd>germ cell neoplasia in situ</kwd>
        <kwd>paratesticular leiomyoma</kwd>
        <kwd>Female</kwd>
        <kwd>Prophylactic</kwd>
        <kwd>Advanced</kwd>
        <kwd>Pediatric</kwd>
        <kwd>NA</kwd>
        <kwd>Male</kwd>
        <kwd>Has Animation</kwd>
        <kwd>Laparoscopic</kwd>
        <kwd>Elective</kwd>
        <kwd>Specimen Retrieval</kwd>
        <kwd>Rare Indication</kwd>
      </kwd-group>
    </article-meta>
  </front>
</article>