understand the importance of the regimen, or preference to be like peers that do not have medical conditions. Children at this stage may dismiss the need to engage in their regimen; as a result, caregivers struggle to nd ways to persuade the child with BEEC to comply. Structured behavioral modications can be useful, as uropsycholo­gists encourage caregivers to use positive reinforcement and incentives as a way to help children adhere to their urological regimen [4]. If developmentally appropriate, the reason for the medical regimen can also be explored and used as a motivator (e.g., achieving continence). Different types of therapy are applied based on the presenting concerns and are typically tailored individually to each child and their family.

Adolescence is a time of many mental and physical changes that are challenging for any individual to experience. Adding in a complex medical condition can exacer­bate this stressful time and cause tension between caregivers and the adolescent with BEEC.Each adolescent differs with the types of concerns they may experi­ence; however, the most common struggles include symptoms of anxiety, depres­sion, irritability, struggles with adherence to medical regimens, fear of peers’ awareness about the condition, and how to handle social and/or romantic relation­ships. Bullying or teasing by peers can occur if others are aware of the medical condition and ostracize the individual with BEEC [10], and action should be taken by caregivers and school staff to ensure safety in the school environment. Therapy can be a useful tool during this stage of development to gure out how to ignore others, problem-solve how to preserve privacy, and maintain safety in school or extracurricular environments if they are fearful of peers targeting them verbally or physically. This topic is difcult for adolescents to discuss with caregivers, mental health providers, and school staff; therefore, a gentle approach and practicing patience about handling these concerns are helpful in allowing the adolescent to feel comfortable discussing solutions to these issues.

recommendations. This behavior can be frustrating for caregivers and medical pro­viders when the adolescent does not see the importance of adherence to prevent further medical concerns as an adult. Therapy helps with normalizing the frustration over having a lifelong medical diagnosis and genital anomaly, reexamining future goals, and nding creative ways to make adherence easier. Adolescents may also benet from therapy focusing on emotional concerns surrounding their diagnosis and feeling different from their peers [11]. Cognitive behavioral therapy and moti­vational interviewing can be useful tools in helping adolescents think more ratio­nally and helpfully about their medical concerns while also creating goals that t their values and continued health maintenance [2].

A sensitive topic that may be brought up in therapy with adolescents includes discomfort and embarrassment regarding sexual activity. Conversations about sex and sexuality are encouraged to start around puberty to give adolescents and care­givers more helpful expectations about future sexual functioning, which can include any surgeries (e.g., penile surgery to create a longer phallus) or concerns they may have prior to sexual activity with a partner [12]. When the adolescent is interested in romantic partners, there is a fear that the partner may not accept the look of their genitalia and/or struggles in their ability to engage in sexual activity [13]. During this stage of development, same-aged peers may not be as accepting of the differ­ences in appearance and ability, and a sense of trust is necessary between partners to help assuage these fears. If the adolescent struggles with rejection, these concerns can be addressed in therapy or with trusted individuals (e.g., caregivers, close friends, and family). Therapy can be helpful at this stage to allow the adolescent to ask uncomfortable questions in a nonjudgmental environment, especially regarding psychological and sexual functioning. There is unfortunately no guide to handling each nuanced situation; however, validation and being present are great ways to support the adolescent with these struggles.

The transition from adolescence to adulthood with a medical condition is compli­cated and difcult for most families. The main struggle includes establishing care with a new hospital system, one that may not connect to the pediatric hospital. In addition, adult care tends to cause hesitation, ambivalence, and fear not only for individuals with BEEC but also for caregivers [14]. As a result, many individuals and families prefer to keep their care within the children’s hospital for as long as possible, which can be difcult with pediatric providers who do not know the com­plexities of adult care. Individuals that wait to start this process further into adult­hood may nd themselves transitioning much later than necessary and struggling to nd the care they need from the right providers.

Although adults with BEEC are not frequently seen for therapy, there are emo­tional struggles that they may face as they get older. These individuals may experi­ence anxiety surrounding body image, self-esteem, sexuality, and sexual functioning [13]. Although there is a preference for adult-based providers with a focus on medi­cal concerns, this specialty can be hard to nd in the community. Most behavioral health providers have a skill set for general emotional concerns (e.g., anxiety, depression, irritability), which may still benet an individual with BEEC even if the provider does not specialize in medical conditions. As an adult with BEEC, sexual activity, incontinence, fertility, and genital appearance can be stressful, and health­related quality of life tends to be impaired from adolescence into adulthood [19, 20]. Even when the individual with BEEC is in good health as an adult, psychiatric concerns can still be present, including anxiety, depression, low self-esteem, poor body concept, and social withdrawal [20].

In addition to these stressors, fertility can be a topic of discussion between part­ners, and this can cause strain in a romantic relationship. While individuals with BEEC are at risk for infertility, some have been successful with reproduction. Male adults may benet from different treatment options, including assisted reproduc­tive techniques or surgical sperm retrieval [21, 23], and there have been successful pregnancies for female adults with BEEC [25]. Pregnancy outcomes can be opti­mized through a successful transition from pediatric to adult urological care, understanding of BEEC and management of complications, and a planned surgical approach to delivery [26]. Despite great medical care, the risks of infertility are still high for individuals with BEEC due to reconstructive surgery affecting repro­ductive ability [23]. While there are other options for individuals with infertility to become parents, the emotional struggles of not being able to conceive should not be overlooked or invalidated. This is largely dependent on the individual and their values (e.g., not all individuals with BEEC may feel this way); therefore, it is best to check in with the individual with BEEC surrounding their preference about this topic.

8]. Delivery should be performed in a center with experience in urological malfor-

mations and urinary diversions. Most deliveries are done through a cesarean sec­tion, which is the preferred and recommended method for delivery with a low complication rate [2, 5, 8–11].

Fertility is impaired in most men with BEEC for multiple reasons. In addition to complications of the reconstruction resulting in urethral strictures, recurrent epi­didymitis, injury of the vas deferens and the testicular vessels, and testicular abnormalities have been observed in BEEC patients. In one study, 50% of the patients had at least one abnormal nding during testicular ultrasound [25]. Recurrent epididymitis may explain the testicular abnormalities as well as the impaired fertility. In one study, 36% of 39 adult males had recurrent epididymitis, and this may increase the risk of an obstruction of the seminal ducts and, as a result impaired ejaculation [26]. In the rare case of a severe obstruction of the urethra after urinary diversion, a vesicostomy of the bladder remnant has been described to prevent recurrent epididymitis [27]. One study (n=14) demonstrated that there was no difference in sperm concentration after reconstruction or urinary diversion [28]. Another study reported a higher paternity rate after primary com­pared to secondary urinary diversion (72% vs 28%), suggesting that fertility is

compromised after failure of primary reconstruction followed by several opera­tions [26]. There are only a few studies with small numbers of patients (n=4–31)

Most case series (n=7–23) report that in patients with paternity, assisted repro­ductive techniques (e.g., insemination, intracytoplasmic sperm injection, and tes­ticular sperm extraction) were used in 25–63% [6, 26, 28, 31, 33]. One study compared paternity rate with normal controls from the same place and found a sig­nicant reduction (22 vs. 45%; p=0.021) [22]. Due to the possible impairment in

seems to be no difference between patients with primary reconstruction or primary urinary diversion [34]. Of note, in a small series of men with BEEC with a substitu­tion phalloplasty, they are satised with their aesthetic, sexual, and psychological outcome [38].

A recent review on the health-related quality of life (HRQOL) in patients with BEEC suggested that the overall HRQOL is favorable; however, urinary inconti­nence and sexual dysfunction do have the greatest negative impact [21]. The lack of validated domain-specic instruments to measure the HRQOL makes it difcult to estimate the inuence of sexual dysfunction on HRQOL.Some studies concluded that HRQOL and sexual functions are comparable to the normal population [31,