Androgen insensitivity syndrome surgery: Difference between revisions
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'''Female assignment''' | '''Female assignment''' | ||
* | *In order to prevent further [[masculinization]], female assignment is usually followed by gonadectomy at puberty. Surgery may involve enlarging the vaginal opening and reducing clitoral size. | ||
*[[Estrogen]] is replaced at puberty. This | *[[Estrogen]] is replaced at puberty. This helps the individuals in having a better cosmetic outlook rather than undergoing surgery involving assignment of sex. Those involved in the surgical procedures have expressed dissatisfaction with sexual sensation and function. | ||
'''Other Options''' | '''Other Options''' | ||
* | *Other option involves temporarily assigning male or female sex and postponing surgerical interventions until early adolescence. | ||
*This approach helps an adolescent to accept or decline gender assigned during [[infancy]], and to make a decision to explore options of reconstructive surgery. | |||
*The disadvantages would be the [[psychosocial]] challenges of differences in the outlook and the gender identity issues. | |||
*Over the last 40-50 years, the second path, female assignment with reconstructive surgery in [[infancy]], has been the course most often chosen by parents and physicians, and the hazards of this course are most familiar. Since 1997, male assignment with early surgery is increasing in popularity, and even the third course of delaying surgery is sometimes followed. Advantages and disadvantages of this course will become apparent over the next two decades. | *Over the last 40-50 years, the second path, female assignment with reconstructive surgery in [[infancy]], has been the course most often chosen by parents and physicians, and the hazards of this course are most familiar. Since 1997, male assignment with early surgery is increasing in popularity, and even the third course of delaying surgery is sometimes followed. Advantages and disadvantages of this course will become apparent over the next two decades. | ||
===Post-operative followup=== | ===Post-operative followup=== | ||
* | *Difference in the sex hormone levels ratio change observed significantly after being treated with [[castration]] compared with preoperative levels. [[Testosterone]] and [[estrogen]] levels have been observed to decrease significantly, whereas [[luteinizing hormone]] and [[follicle-stimulating hormone]] have increased significantly. Whereas, [[prolactin]] levels have not been shown to change significantly.<ref name="pmid25170741">{{cite journal| author=Wang Z, Sa YL, Ye XX, Zhang J, Xu YM| title=Complete androgen insensitivity syndrome in juveniles and adults with female phenotypes. | journal=J Obstet Gynaecol Res | year= 2014 | volume= 40 | issue= 9 | pages= 2044-50 | pmid=25170741 | doi=10.1111/jog.12455 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25170741 }} </ref> | ||
* | *In CAIS patients, the removal of the testes after puberty is considered to be safe and reliable. But, it has been observed that providing a hormone drug after being treated with [[castration]] is considered essential.<ref name="pmid25170741">{{cite journal| author=Wang Z, Sa YL, Ye XX, Zhang J, Xu YM| title=Complete androgen insensitivity syndrome in juveniles and adults with female phenotypes. | journal=J Obstet Gynaecol Res | year= 2014 | volume= 40 | issue= 9 | pages= 2044-50 | pmid=25170741 | doi=10.1111/jog.12455 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25170741 }} </ref> | ||
==Indications== | ==Indications== | ||
* | *With the increase of age, the risk of gonadal tumor development in CAIS may also increase. <ref name="pmid8351649">{{cite journal| author=Forsyth CS, Frank AA| title=Evaluation of developmental toxicity of coniine to rats and rabbits. | journal=Teratology | year= 1993 | volume= 48 | issue= 1 | pages= 59-64 | pmid=8351649 | doi=10.1002/tera.1420480110 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8351649 }} </ref> | ||
* Bilateral testicles with [[Sertoli Cell Tumor|Sertoli cell tumors]]. | *Incidence rates have been shown to be 0.8–22% in patients choosing to retain their gonads into adulthood. Gonadectomy has been recommended either during childhood or after puberty. <ref name="pmid8351649">{{cite journal| author=Forsyth CS, Frank AA| title=Evaluation of developmental toxicity of coniine to rats and rabbits. | journal=Teratology | year= 1993 | volume= 48 | issue= 1 | pages= 59-64 | pmid=8351649 | doi=10.1002/tera.1420480110 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8351649 }} </ref> | ||
*Bilateral testicles with [[Sertoli Cell Tumor|Sertoli cell tumors]]. Due to the potential risk of malignant transformation in the residual gonads, prophylactic gonadectomy following puberty has been recommended. <ref name="pmid28386495">{{cite journal| author=de Souza RF, Pereira da Silva J, Vieira Balla B, Neves Ferreira R, Chambô Filho A| title=Bilateral Sertoli Cell Tumors in a Patient with Androgen Insensitivity Syndrome. | journal=Case Rep Obstet Gynecol | year= 2017 | volume= 2017 | issue= | pages= 8357235 | pmid=28386495 | doi=10.1155/2017/8357235 | pmc=5366775 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28386495 }} </ref> | |||
==Contraindications== | ==Contraindications== |
Revision as of 16:10, 29 September 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]: Associate Editor(s)-in-Chief:
Overview
Surgical approach to the Androgen insensitivity syndrome involves vaginal dilation or gonadectomy or determination of sex which depend on various factors such as the type of AIS, age, sex and preventive measures to be taken in adolescence and adulthood.
Surgery
Complete Androgen Insensitivity Syndrome
Vaginal enlargement
- For women for whom vaginal shallowness is a problem, enlargement can be achieved by a prolonged course of self-dilation. Surgical construction of a vagina is sometimes performed for adults but may have its own potential problems.
Gonadectomy
- Optimal timing of removal of the testes has been the management issue most often debated by physicians. The advantage of retaining the (usually intra-abdominal) testes until after puberty is that pubertal changes will happen "naturally," without hormone replacement due to the conversion of testosterone to estrogen in the body tissues by aromatisation.
- Testes remaining in the abdomen throughout life may develop benign or malignant tumors and confer little benefit. Evidence suggests that women with CAIS and PAIS retaining their testes after puberty have a 25% chance of developing benign (harmless) tumors and a 4-9% chance of malignancy.
- Benefit of testes in CAIS is the estradiol produced from testosterone. Although this can be provided pharmaceutically post-gonadectomy, many CAIS women have trouble adjusting to artificial HRT and regret losing their natural source of estrogen.
- The current recommendations state that gonads in CAIS can be retained until early adulthood. The small number of individuals with gonadectomy after age 20 years do not allow firm conclusion regarding later adulthood. Therefore, it is recommended that the option of gonadectomy be discussed in adulthood. Some form of regular surveillance of the gonads is then recommended, although none of the available options are ideal.[1]
Incomplete or partial AIS
Management issues for PAIS are typically the same as for CAIS. Most women with PAIS do not seek genital reconstructive surgery for anatomic differences.
Reifenstein syndrome
- The first major management decision is the assignment of sex, whether to assign as a male or a female. Assignment depends partly on predicting likely pubertal development, potential response of the phallus to testosterone, and likely outcome of surgical reconstruction attempts. The Reifenstein form of AIS can present one of the most challenging sets of decisions imaginable as parents and physicians try to choose the "least bad" of several undesirable options.
Male assignment
- Male assignment is usually followed by one or more operations in infancy to completely repair the hypospadias, close the midline pouch, and (if possible) place the testes in the scrotum.
- Gonadal status and potential testosterone responsiveness is reassessed around age 12.
- Breast tissue can be removed surgically in adolescence if excessive.
- Gonads should be removed if scrotal placement is impossible.
- High dose testosterone replacement will sometimes achieve further virilization. An advantage of this choice commonly cited by parents is consistency with karyotype. A survey of adults brought up this way reported that nearly all were comfortable with the gender assignment made at birth and the sexual function of their genitalia, but many were dissatisfied with the size.
Female assignment
- In order to prevent further masculinization, female assignment is usually followed by gonadectomy at puberty. Surgery may involve enlarging the vaginal opening and reducing clitoral size.
- Estrogen is replaced at puberty. This helps the individuals in having a better cosmetic outlook rather than undergoing surgery involving assignment of sex. Those involved in the surgical procedures have expressed dissatisfaction with sexual sensation and function.
Other Options
- Other option involves temporarily assigning male or female sex and postponing surgerical interventions until early adolescence.
- This approach helps an adolescent to accept or decline gender assigned during infancy, and to make a decision to explore options of reconstructive surgery.
- The disadvantages would be the psychosocial challenges of differences in the outlook and the gender identity issues.
- Over the last 40-50 years, the second path, female assignment with reconstructive surgery in infancy, has been the course most often chosen by parents and physicians, and the hazards of this course are most familiar. Since 1997, male assignment with early surgery is increasing in popularity, and even the third course of delaying surgery is sometimes followed. Advantages and disadvantages of this course will become apparent over the next two decades.
Post-operative followup
- Difference in the sex hormone levels ratio change observed significantly after being treated with castration compared with preoperative levels. Testosterone and estrogen levels have been observed to decrease significantly, whereas luteinizing hormone and follicle-stimulating hormone have increased significantly. Whereas, prolactin levels have not been shown to change significantly.[2]
- In CAIS patients, the removal of the testes after puberty is considered to be safe and reliable. But, it has been observed that providing a hormone drug after being treated with castration is considered essential.[2]
Indications
- With the increase of age, the risk of gonadal tumor development in CAIS may also increase. [3]
- Incidence rates have been shown to be 0.8–22% in patients choosing to retain their gonads into adulthood. Gonadectomy has been recommended either during childhood or after puberty. [3]
- Bilateral testicles with Sertoli cell tumors. Due to the potential risk of malignant transformation in the residual gonads, prophylactic gonadectomy following puberty has been recommended. [4]
Contraindications
- There are no specific contraindications advised as such from a surgical approach point of view. General factors such as a age for consideration of surgery and other co-morbid conditions have to be taken in to consideration before planning for a surgery.
References
- ↑ Chaudhry S, Tadokoro-Cuccaro R, Hannema SE, Acerini CL, Hughes IA (2017). "Frequency of gonadal tumours in complete androgen insensitivity syndrome (CAIS): A retrospective case-series analysis". J Pediatr Urol. doi:10.1016/j.jpurol.2017.02.013. PMID 28351649.
- ↑ 2.0 2.1 Wang Z, Sa YL, Ye XX, Zhang J, Xu YM (2014). "Complete androgen insensitivity syndrome in juveniles and adults with female phenotypes". J Obstet Gynaecol Res. 40 (9): 2044–50. doi:10.1111/jog.12455. PMID 25170741.
- ↑ 3.0 3.1 Forsyth CS, Frank AA (1993). "Evaluation of developmental toxicity of coniine to rats and rabbits". Teratology. 48 (1): 59–64. doi:10.1002/tera.1420480110. PMID 8351649.
- ↑ de Souza RF, Pereira da Silva J, Vieira Balla B, Neves Ferreira R, Chambô Filho A (2017). "Bilateral Sertoli Cell Tumors in a Patient with Androgen Insensitivity Syndrome". Case Rep Obstet Gynecol. 2017: 8357235. doi:10.1155/2017/8357235. PMC 5366775. PMID 28386495.