Surgical options

The most recent international recommendations, supported by leading human rights organizations, advocate postponing medical-surgical treatments whenever possible, until the intersex person has sufficient cognitive maturity to express their informed consent about their body.

Rarely, certain anatomical variations (e.g., the obstruction of the urine flow or hernias in the pelvic area) can pose an imminent risk for the child’s physical health. Other VSCs/DSDs may entail physical consequences, such as the obstruction of the menstrual flow, or the long-term risk of tumours in the case of undescended testicles. Finally, other VSCs/DSDs (e.g., variations of the appearance of the external genitalia or of the depth of the vagina) are not associated with immediate or long-term physical risks for the person. To date, when there is no immediate risk to the person's physical health, the criterion is to postpone surgery.

The surgical approach must always involve a multidisciplinary and adequately trained team care including the following disciplines: urology, general surgery, plastic surgery, endocrinology, pediatrics, gynecology, psychology/social work, genetics/counselling, nursing and, if possible, bioethics, and social sciences. The team must provide skill-based counselling, outlining possible risks and benefits of early and late interventions, and engage the person concerned – and/or their legal guardians, in the case of a minor – in the decision-making process. It is recommended that this counseling be accompanied by meetings with peers through community support groups.

The non-urgent surgical options, where requested by the person directly concerned, must be individualized on the basis of the specific VSC/DSD, based on the person's age at the beginning of the clinical pathway, as well as on their familial and cultural background. It is also necessary to contact an expert in genital or gonadal surgery to evaluate the best solution together. There are, in fact, different surgical options for specific VSC/DSD. When it comes to surgeries involving the genitals, “open” surgery is typically employed. This type of surgery allows for direct access to the external structures of the genital area. For VSCs/DSDs involving ureter, bladder, urethra and for some vaginal procedures, or when further diagnostic investigation is necessary, an endoscopic approach is suggested. Minimally invasive, it consists in passing a long, thin tube with a small camera inside (an endoscope) into the body through a natural opening to explore the body pathways without making incisions. Some operating instruments or laser fibres can also be used. For more complex situations, however, abdominal or combined perineal-abdominal surgical approaches, with or without the use of minimally invasive laparoscopic or robotic abdominal surgery, are considered. In cases such as aesthetic reconstructions –reconstructions with skin flaps or when it is necessary to manipulate the intestine – a plastic surgeon or a general surgeon may be involved. 

Surgical options can be considered when the functionality of the urogenital system is impaired and may put the person's life at risk, e.g., difficulties emptying the bladder when urinating, feeling of incomplete emptying after urinating or recurring urinary infections. The urologist may find the following associated conditions: neurogenic bladder (i.e., a malfunction of the bladder and urethra due to defective innervation or nerve impulse transmission resulting in urinary incontinence, urinary retention and urinary infections); vesicoureteral reflux (backward flow of urine from the bladder down the ureter to the kidney); ureteral pelvis syndrome (urine cannot flow properly from the kidney into the ureter due to an obstruction of the passageway between the renal pelvis –the part of the kidney where urine collects – and the ureter itself). If there are more complex issues such as the presence of cloacae, a single channel for the expulsion of both urine and faeces, early surgery may be necessary to protect the person's physical health.

In the case of atypical female or male external genitalia – for example, increased size of the clitoris or labia (clitoral or labia hypertrophy), bifid (divided in two) scrotum, curved penis, etc. – the surgeon, generally a urologist, will assess together with the person, their legal guardians in the case of minors, and the multidisciplinary team: i) a conservative approach through active surveillance, ii) surgery. In the latter case, the urological surgeon, supported by the multidisciplinary team, will propose the most effective approach or help the person and/or their family to discuss the alternatives if there are multiple treatment options.

It must be noted that early surgery, if carried out for reasons other than a medical emergency, is often complex and controversial. This is especially true when the minor cannot actively participate in the decision-making process that involves their body. VSCs/DSDs experts have not yet come to a unanimous agreement as to when is the best time to undergo elective surgery. 

The international scientific literature reports cases of adults with VSCs/DSDs who have experienced physical and mental health problems following early interventions. Furthermore, some early surgical procedures can cause aesthetic, urinary, reproductive and sexual complications with long-lasting negative consequences into adulthood. Finally,  some intersex people may go through negative experiences after gonad removal surgery, although it is necessary to carefully assess the possible risk  of any malignancy based on the specific VSC/DSD (for details relating to the oncological risk, it is possible to consult the dedicated pages to individual VSC/DSD).

Although there is still a heated debate among medical professionals regarding the appropriateness of early genital surgery for conditions that do not pose a risk to physical health, experts recommend postponing surgery until the individual can actively participate in the decision-making process. This is a human-rights-based, case-by-case approach.

Bibliography
de Vries ALC et al. Mental Health of a Large Group of Adults With Disorders of Sex Development in Six European Countries. Psychosomatic Medicine. 2019 Sep;81(7):629-640
Kon AA. Ethical issues in decision-making for infants with disorders of sex development [Abstract]. Hormone and metabolic research. 2015 May;47(5):340-3 
Meyer-Bahlburg HFL. The Timing of Genital Surgery in Somatic Intersexuality: Surveys of Patients' Preferences. Hormone Research Paediatrics. 2022;95(1):12-20
National Academies of Sciences, et al. Understanding the Well-Being of LGBTQI+ Populations. Washington (DC): National Academies Press (US); 2020 Oct 21
Pediatric Endocrine Society. Position statement on genital surgery in individuals with differences of sex development (DSD)/intersex traits (care guidelines consensus statement). 2020 Oct 20

Surgical treatment of vaginal agenesis
According to scientific research, the following persons  with Variations of Sex Characteristics (VSCs)/Differences of Sex Development (DSDs) should not undergo surgery for the treatment of vaginal agenesis (partial or complete absence of the vagina): those in prepubertal age; those who have no interest in vaginal sex; those without adequate counselling about non-surgical dilation therapy or other surgical techniques; those who are uninformed about necessary follow-up treatment.

Surgery for vaginal agenesis should be performed only if dilator therapy has failed and when the person strongly desires it (after a thorough discussion of the advantages and disadvantages of the different techniques). The treatment should be offered when the person feels emotionally and physically ready, and expresses the desire to undertake the therapeutic process. It should be noted that surgical treatment may as well require post-operative vaginal dilation to maintain adequate vaginal length and diameter.

There are different vaginoplasty surgical techniques. Because of the lack of follow-up data or comparative studies on the long-term results of the various techniques, to date there is no evidence supporting the preference of one technique over another. Each procedure has advantages and disadvantages, and there is no “perfect” option. Complications of vaginoplasty, as compared to those following non-surgical vaginal dilation, are much more common and include:

• intraoperative complications (intestinal or bladder perforation in 1-4% of cases and infection in 4-7% of cases)
• post-operative complications (tissue necrosis 1-3.5%, scarring disorders 9%, fistulas 1-3%)
• long-term complications (need for further surgery 40%, vaginal stenosis 4-9%, copious secretions 3%, prolapse 3%)

Bibliography
Callens N et al. An update on surgical and non-surgical treatments for vaginal hypoplasia. Human Reproduction Update. 2014 Sep-Oct;20(5):775-801
Committee on Adolescent Health Care. ACOG Committee Opinion No. 728: Müllerian Agenesis: Diagnosis, Management, And Treatment. Obstetrics and Gynecology. 2018 Jan;131(1):e35-e42
Imparato E et al. Long-term results of sigmoid vaginoplasty in a consecutive series of 62 patients [Abstract]. International urogynecology journal and pelvic floor dysfunction. 2007 Dec;18(12):1465-9
Kim C et al. Robotic sigmoid vaginoplasty: a novel technique. [Abstract] Urology. 2008 Oct;72(4):847-9
Liguori G et al. Laparoscopic mobilization of neovagina to assist secondary ileal vaginoplasty in male-to-female transsexuals [Abstract]. Urology. 2005 Aug;66(2):293-8; discussion 298