Androgen insensitivity syndrome surgery: Difference between revisions
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===Reifenstein syndrome=== | ===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. | *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''' is usually followed by one or more operations in infancy by a pediatric urologist 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. | * '''Male assignment''' is usually followed by one or more operations in infancy by a pediatric urologist 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. | ||
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*More radical suggestions such as attempting to avoid assigning a sex at all, or teaching the child to identify __self as a third sex have not found much acceptance among those who are actually raising and caring for children, or among many intersex advocates. | *More radical suggestions such as attempting to avoid assigning a sex at all, or teaching the child to identify __self as a third sex have not found much acceptance among those who are actually raising and caring for children, or among many intersex advocates. | ||
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=== | ||
Revision as of 00:27, 11 July 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]: Associate Editor(s)-in-Chief:
Overview
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 oestrogen 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 oestrogen.
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 is usually followed by one or more operations in infancy by a pediatric urologist 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 is usually followed by gonadectomy in childhood to prevent further masculinization, especially at puberty; sometimes by surgery to enlarge the vaginal opening and reduce clitoral size. Estrogen is replaced at puberty. This course has the advantage that future tissue sensitivity to testosterone is irrelevant for a girl. This course may involve fewer surgical procedures than male assignment and surgery, and may produce a better cosmetic outcome, but a higher percentage of women raised with early surgical repair describe impairment of sexual sensation or function.
- A third option has been advocated in the last decade by some: to tentatively assign male or female sex but postpone all surgery until early adolescence. This approach is intended to make it easier for an adolescent to reject or confirm the gender assigned in infancy by parents and doctors, and to choose or refuse reconstructive surgery. This approach might reduce the frequency of double surgery or unsatisfactory surgical outcomes, and would not jeopardize erectile tissue. Potential disadvantages of this course would be the psychosocial challenges to child and parents of the anatomic differentness, the potential adverse effect on adolescent social development of unresolved sexual identity issues combined with a 1" phallus and large breasts, and perhaps the unfairness of presenting a child in early adolescence with choices deferred as too difficult by parents and physicians 12 years earlier. The greatest appeal of this choice may be that we don't yet know the magnitude or frequency of the potential problems as well as we do for the more traditional choices.
- More radical suggestions such as attempting to avoid assigning a sex at all, or teaching the child to identify __self as a third sex have not found much acceptance among those who are actually raising and caring for children, or among many intersex advocates.
- 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
- Sex hormone level ratio changed significantly after being treated with castration compared with preoperative levels; mainly testosterone and estrogen decreased significantly, while luteinizing hormone and follicle-stimulating hormone significantly increased. However, prolactin did not change significantly.[1]
- Removal of the testes in CAIS patients after puberty is safe and reliable. However, it is essential to provide a hormone drug after being treated with castration.[1]
Indications
- The risk of gonadal tumour development in CAIS may increase with age; incidence rates have been reported to be 0.8–22% in patients who have retained their gonads into adulthood. gonadectomy has been recommended either during childhood or after puberty is complete, although there is no consensus on the optimal timing for this procedure. [2]
- Bilateral testicles with Sertoli cell tumors. Prophylactic gonadectomy following puberty is recommended due to the progressive risk of neoplastic transformation in the residual gonads. [3]
Contraindications
References
- ↑ 1.0 1.1 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.
- ↑ 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.