Raised a eunuch: a case report of testicular loss without androgen replacement in a pre-pubescent boy

Abstract

Introduction & Importance:

Bilateral testicular torsion with testicular loss is a rare condition in prepubertal youth. This case represents one of the only documented cases of a modern, pre-pubertal youth raised as a eunuch through adolescence.

Case Presentation:

This is a case of a pre-pubertal boy (Z) who suffered bilateral testicular torsion and gonadal loss who was raised as a eunuch through adolescence.

Clinical Discussion:

Throughout his adolescence, Z demonstrated no deficits in psychosocial functioning, and displayed no intellectual or learning deficits. Compared to his peers and younger brother, Z was less interested in physical and competitive activities, preferring more supportive, pro-social behaviors, like caring for children.

Conclusion:

In this case, hormonal deprivation and delayed puberty did not appear to impact neurodevelopmental processes or psychosocial development. This case has potential issues for other areas of healthcare where concerns have arisen around long-term pubertal suppression.

Introduction

Testicular torsion is a rare condition where the spermatic cord and its contents become twisted, obstructing blood flow to the testis^[1]. The annual incidence of unilateral testicular torsion has been estimated to be 3.8 per 100 000 for boys under the age of 18 years^[2]. Bilateral cases are even less common^[1]. This condition is a medical emergency resulting in orchiectomy in up to 42% of cases^[2]. In cases where there is bilateral testicular loss, the standard recommendation is androgen supplementation with the goal of normal pubertal maturation and development^[3].

The effects of long-term androgen deprivation have been documented in accounts of historic eunuchs^[4,5] and modern hypogonadal conditions^[6–10]. Based on studies of testosterone loss in adulthood (primarily in prostate cancer patients) some of the primary concerns of long-term androgen deprivation in adults include fatigue, depression, bone loss, sarcopenic obesity, metabolic syndrome increasing the risk of cardiovascular disease and diabetes, and cognitive deficits^[6–10]. Testosterone treatment can overcome the adverse effects of androgen deprivation^[11]. Lifestyle interventions may also be helpful^[7].

HIGHLIGHTS

• Bilateral testicular torsion is a medical emergency often requiring orchiectomy.

• Cases of agonadal youth raised as eunuchs without hormonal replacement are rare.

• A lack of androgens through adolescence results in a eunuchoid body habitus and osteopenia.

• No psychological or neurocognitive deficits were observed despite a lack of hormones and pubertal progression.

• In terms of psychosocial development, there was little interest in competitive physical activities and more caring, pro-social behaviors

A recent review by Salimi et al^[12] suggests that androgen deprivation during pre- and mid-adolescence has little or no impact on learning and memory, while androgen loss after adolescence into adulthood could significantly impair learning and memory functions. While that review focused on animal studies, it has potential implications for human subjects in situations where androgen supplementation may not be available or is declined. It also has potential implications in gender diverse populations where concerns about learning and memory have been raised as a potential negative side effect of pubertal suppression.

Findings are reported in line with the 2023 Surgical CAse REport (SCARE) guidelines^[13]. This is the second installment of our ongoing study of the individual. The first part was published as Johnson TW Wassersug RJ. My son was castrated as a result of a medical error. Is it OK to raise him as a eunuch?. Ann Med Surg (Lond) 2021;68:102586.

Methods

This report is the result of an extensive ongoing written correspondence over several years. No direct clinical care was provided by the authors. Informed consent to publish the results of this correspondence and the deidentified image (Fig. 3), was received from the guardian of the patient and from the patient themselves once they reached the legal age of consent.

Figure 3.

Image of Z.

Unable to find adequate information from local sources to help his son, 2 years after his emergency orchiectomy, X contacted us. We have subsequently received detailed reports from him with information about Z’s development, including photographs and medical records from his local hospital. X has responded in detail to our questions and has passed our questions on to both Z and his younger brother. X has provided translations of the boys’ responses and PDF copies of Z’s handwritten responses in their original language.

Case report

Z is an Asian boy, currently in his late teens, who suffered from bilateral testicular torsion and testes loss around Tanner stage 2 (testicular volume approximately 5 mL). At that time, the boy’s father (X) consulted with a pediatric endocrinologist who recommended testosterone supplementation for Z to support pubertal development. X discussed that with his son Z and they both declined that treatment, opting instead for Z to be raised as a eunuch.

X’s choice to raise Z as a eunuch was not without concern. Z’s endocrinologist educated X on the potential negative effects including a lack of sexual and physical maturation, and a possible increased risk for conditions like osteoporosis and cardiovascular disease^[11]. X expressed openness to revisit androgen therapy for Z, if the boy began to experience psychosocial challenges at home or in school, if Z began to demonstrate any learning deficits, if any mental health or adjustment issues arose, and/or if Z desired to be on hormone replacement.

As part of their regular medical follow-up, Z and his younger brother had occasional blood draws to assess serum concentrations of follicle-stimulating hormone (FSH) and testosterone (T). Under normal physiologic conditions, puberty begins with the pulsatile release of gonadotropin-releasing hormone (GnRH) from the hypothalamus. GnRH stimulates the anterior pituitary to release luteinizing hormone (LH) and FSH, which stimulate the production testosterone in boys. Testosterone then exerts negative feedback on the production of GnRH, LH, and FSH. As seen in Fig. 1, Z’s T remained very low due to his testicular loss. In contrast, his younger brother’s continued to rise over time. In Fig. 2, we see Z’s FSH rise over time due to a lack of T, while Z’s brother’s FSH remained at a low level.

Figure 1.

Changes in testosterone over time.

Figure 2.

Changes in follicle-stimulating hormone over time.

At the time of his orchiectomy, Z lived with his father, X, and his younger brother, G. X was a widower who had not remarried. Z did have additional support from extended family. “Empathetic” was a term used by X to describe Z’s interactions with family. Over the following years, Z was observed to prefer the company of younger peers and to enjoy the care of young children in family gatherings. X described Z’s prosocial behavior to be “more feminine” than his younger brother and other boys, which X attributed to Z’s lack of androgens and pubertal progression.

After his orchiectomy, physical activity, both cardiovascular and resistance training, were encouraged. Z previously enjoyed basketball, but became less committed to athletics. Because his testicular torsion occurred after a vigorous basketball game, he credited that with his reduced interests in sports. To avoid possible osteopenia, daily calcium and vitamin D supplementation were initiated. At 15 years and 2 months age, Z had a bone age of 13 years and 4 months, and a Z-score of −1.2. Z began to gain weight around his waist and hips about a year and a half after his orchiectomy despite personal efforts to not gain excessive weight. This resulted in a eunuchoid body habitus (see image 1 below). Due to his lack of pubertal progression, Z’s voice remained higher pitched than other boys his age, which has bothered him. He has failed to develop common secondary sexual characteristics of puberty such as facial and pubic hair, which pleased him as he found them as unappealing features of boys of his age.

As Z progressed through school, his relationships with age-matched same-sex peers declined. Z and X identified several possible factors that may have contributed to this change. In an attempt to prevent rumors about Z’s eunuch status, X attempted to explain Z’s condition to his neighborhood peers, which led to their social distancing from Z. Z himself described having interests different from his peers. Z also stated that he didn’t like the smell of the eugonadal boys of his own age when he lived with them in a student dormitory.

Another consequence of Z’s agonadal status was a lack of genital development and sex drive. Perhaps due to this lack of hormonal growth and stimulation, Z developed phimosis and inflammation of the glans penis. Z’s lack of sex drive made it difficult for him to relate to other boys his age. He also reported little interest or enjoyment in competitive activities typical of males in puberty. For these reasons, Z felt content focusing on his family relationships and his academic pursuits.

Over the years from his orchiectomy to the present, Z has maintained an above average academic performance. He excelled in subjects like English and biology, but had some difficulty in subjects like mathematics and chemistry. At the time of this writing, Z received acceptance to a well ranked university in his application pool. Z was pleased to report that, due to his youthful appearance, he was perceived as a child prodigy during his compulsory pre-university military training. He reported that his fellow students treated him as a younger brother. His long-term goal is to work in healthcare and medicine.

From the time of his orchiectomy, Z has been aware of his agonadal status, both his sterility and the potential consequences of delaying puberty. He has witnessed the effects of puberty first hand from his younger brother, who is currently at Tanner Stage 4, and from his roommates when he lived in a high school dormitory. Z has expressed no regret about his shared decision to not initiate testosterone treatment. X describes Z as happy and exhibiting no adverse mental health effects of androgen deprivation.

Discussion

Most of our understanding of the long-term physiological effects of androgen deprivation in young people comes from accounts of historical eunuchs^[4,14,15]. For example, in imperial China the preferred eunuchs were those castrated before puberty^[16]. Far from being cognitively impaired, these eunuchs often held important positions of state^[5,16]. In the great Islamic empires, eunuchs were frequently military commanders and government officials. During the Fatimid Caliphate (909–1171 CE), they made up such a high proportion of provincial governors that some governors were described as “not castrated”^[17]. These accounts of eunuchs castrated before puberty are in contrast to studies of androgen suppression in adult prostate cancer patients, which often show cognitive impairments^[6,8].

Similar to historical eunuchs and studies in animals^[4,12,16], Z did not demonstrate significant cognitive effects from androgen deprivation. Z’s academic performance scored above average. Physically, as Z developed, he did begin to exhibit a eunuchoid body habitus: beardlessness, some gynecomastia, prepubertal fat distribution, and long arms and legs relative to his height (see image below). Metabolic studies were not available, but Z’s father acquired from his two sons’ physican data on their folical stimulating hormone and testosterone status over time and provided us with copies (see Figure 1 and 2).

Should Z continue to choose to be a eunuch, he may be prone to a number of conditions observed in historical and modern eunuchs. Kyphosis of the spine, often a manifestation of osteoperosis, has been observed in Skoptzy and Chinese eunuchs^[4]; however, nutritional factors, such as lack of calcium and vitamin D, may have also contributed. Other concerns include metabolic syndrome, diabetes, and cardiovascular disease^[18]. It is unclear what Z’s actual risk for these latter conditions would be as the data on males largely come from adults treated for prostate cancer, who were four or more decades older at the time of androgen deprivation than was Z^[18,19]. In contrast, there is evidence that eunuchs castrated before puberty have an extended life expectancy of 10 to 15 years^[14,15].

Z’s social and emotional temperament are likely influenced by his androgen status. Modern voluntary eunuchs often describe a desirable state which they term the “eunuch calm,” which is a state of decreased libido and decreased reactive aggression^[20]. In men treated for prostate cancer, effects of androgen deprivation are quite variable, with some adapting well, and others developing anxiety, depression, and mood lability^[18]. Several factors appear to contribute to these variable effects. Similar to the cognitive effects observed in animal studies^[12], the mood effects of androgen deprivation in humans may be partially dependent on when the androgen loss occurs, with younger persons being less effected. Embodiment goals may also be important. For an adult with prostate cancer, androgen deprivation may directly challenge their identity as a man^[21]; whereas, androgen loss in an adult voluntary eunuch is most often ego-syntonic^[20]. Z likely falls somewhere in between, which may explain his reticence to start hormone replacement.

While clinical and psychometric assessments would have helped confirm Z’s neurocognitive and psychosocial functioning, such evaluations are rarely pursued in the absence of reported concerns. Z’s strong academic performance, along with X’s observations of Z’s adaptability and ability to maintain relationships at school and home, suggest normal cognitive development and psychological stability.

Some will argue that X’s decision to raise Z as a eunuch was unethical despite the boy sharing the decision-making. Similar to other youth who may experience a life changing medical event that has long-lasting implications, X’s primary concern was Z’s autonomy to make an informed choice about his body when mature. Knowing that treatment could be initiated at any time, and equipped with the knowledge that delaying puberty is generally considered safe^[22], X chose to raise his son as a eunuch. This choice respected the autonomy of both the father and patient, with no apparent harm to Z’s psychosocial or functional development.

This case report has several strengths and limitations. The primary strength of this case report is the lengthy and detailed correspondence between the authors and Z and his father, which included lab reports, photos of physical changes, education reports, and accounts of psychosocial functioning. Limitations include: (1) Direct clinical and/or psychometric assessments were not conducted to confirm the reported neurocognitive and psychosocial outcomes. (2) There may have also been both positivity and confirmation bias in the father’s reporting of Z’s experience. (3) All correspondence was through email. (4) The authors had no direct clinical contact with either Z or his father, which makes it difficult to confirm reported observations.

Conclusion

Cases of bilateral testicular torsion requiring orchiectomy are rare^[1]. Among these cases, no modern report has detailed the psychosocial and physical development of a youth raised as a eunuch without androgen replacement.

This case has implications for other areas of healthcare where puberty suppression is the recommended intervention^[22,23]. While expert consensus supports these interventions^[22,23], one of the concerns of delaying puberty is the potential impact on neurodevelopment. While human studies are few, evidence suggests that puberty blockers do not impact Intelligence Quotient in a clinically significant manner^[24]. Z’s case supports that view.

Ethical approval

No ethical approval was required for the publication of this case study.

Consent

Consent to publish this case report was received by the patient and his father.

Sources of funding

This work did not receive funding.

Author contributions

Study Concept/Design: N/A; Data Collection: J.A., R.W., T.J.. Analysis/Interpretation: J.A., R.W., T.J.. Writing: J.A., R.W., T.J.

Conflicts of interest disclosure

No conflicts of interest.

Guarantor

The authors Jamie D. Agapoff, Richard J. Wassersug, and Thomas W. Johnson are the guarantors of this case report.

Research registration unique identifying number (UIN)

Not applicable.

Provenance and peer review

This paper was not invited.

Data availability statement

Not Applicable.