PITUITARY AND STALK

Visual Loss and Hormonal Alteration
in a Young Transgender Woman

A. SCOTT EMMERT, BS
BRENDAN WILSON, PA-C
AHMED HUSSEIN, MD
JONATHAN A. FORBES, MD

Note: In this chapter, the authors will refer to the patient by their preferred pronoun and name throughout, even when discussing events that occurred pre-transition.

Craniopharyngiomas (CPA) are benign tumors that originate near the hypothalamic region, pituitary stalk, and pituitary gland.¹ As CPAs enlarge, mass effect from the tumor can cause several problems, including visual deterioration and hormonal imbalance. The most common form of visual deterioration in patients with CPA involves loss of vision in the outer halves of both eyes, referred to as bitemporal hemianopsia. CPAs that are symptomatic are often treated with surgical resection. Increasingly, neuro-surgeons have come to favor use of a type of rod/lens camera known as an endoscope to improve visualization during surgical removal of CPAs through the endonasal corridor. While CPAs are benign tumors, they are notoriously prone to delayed recurrence after resection.

Doctors refer to the pituitary gland as the “master gland” because it controls so many functions in the body.² In particular, the pituitary gland helps regulate the body’s metabolism, response to stress, musculoskeletal growth, sex drive and development, and fluid/electrolyte balance.³ Deficiency in any one of the many pituitary hormones can have profound effects on a patient. This is especially true in pediatric patients, where the pituitary axes are especially integral to early development. The majority of CPAs are either found in children between the ages of five to 14 years old or in adults 50 to 74 years of age.⁴ Because the population of transgender patients with CPA is relatively small, little is known about the effects of craniopharyngiomas on gender identity in transgender (trans) individuals, or those whose gender identity does not align with their assigned sex at birth. In this chapter, we describe the story of Arianna,^* a 26-year-old transgender woman, who was found to have a CPA originating from her pituitary stalk and compressing her optic nerves, resulting in visual deterioration.

Arianna was assigned male at birth. She recalls a typical childhood growing up, where she played sports and liked to draw. In her teenage years, Arianna began to feel as if something about her was different. While her peers became interested in dating, Arianna notes that she never had much interest in sexual topics. Moreover, she remembers that when all the other boys her age began to grow facial and body hair, she had failed to develop many of these secondary sexual characteristics. As Arianna grew older, she began to feel her gender identity didn’t align with her assigned gender. Ultimately, she decided to proceed with gender transition.

During Arianna’s first visit with her family doctor to discuss her possible transition, the doctor sent off labs to evaluate levels of pituitary-related hormones in her bloodstream. At this time, it was discovered Arianna was producing abnormally low levels of testosterone. As a significant percentage of patients have naturally lower levels of testosterone, her physician did not feel the need to investigate this further. After a few weeks of counseling, Arianna began treatment with feminizing hormone replacement therapy as a part of her gender transition. This consisted of a daily oral regimen of estrogen (estradiol) and spironolactone, a testosterone blocker. Two months into hormone therapy, Arianna felt very happy with the changes that were occurring. Around this time, she found a steady job and met a man named James, with whom she ultimately fell in. It was not until Arianna and James had moved in together that James began to notice that something was wrong with Arianna’s vision. Specifically, Arianna always seemed to be running into walls, doorways, or other objects that should have appeared in her peripheral vision.

The tunnel vision progressed to blurriness that Arianna became more aware of. As James recalls, “Things got to the point where I would have to stand right in front of her just to get her to notice me.” At a check-up with her optometrist, serious abnormalities in both visual fields and acuity were noted. Arianna was immediately referred to a neuro-ophthalmogist, where Humphrey visual fields confirmed progressive visual loss in the outer half of both eyes consistent with bitemporal hemianopsia (Figure 7.1).⁵ An MRI scan of the brain was subsequently ordered. The MRI demonstrated an enhancing lesion involving the sellar and suprasellar regions with severe associated compression of the optic nerves and chiasm (Figure 7.2). The radiographic features of the tumor were most consistent with a craniopharyngioma.

Arianna was then referred to Dr. Jonathan Forbes, a neurosurgeon who specializes in tumors of the skull base. Dr. Forbes informed Arianna LEFT EYE RIGHT EYE Outer Visual Inner Visual Inner Visual Outer Visual Field Field Field Field that her pituitary gland was not working correctly and she would need to be evaluated by an endocrinologist for supplementation of pituitary hormones. He also informed her that, following hormonal optimization, surgical removal of the tumor would be necessary to prevent additional visual deterioration. After the visit with Dr. Forbes, Arianna was seen by the endocrinology team, who acknowledged that the tumor may have been affecting the function of her pituitary gland for many years prior to her visual symptoms. Feeling better on medications prescribed by endocrinology, Arianna returned to Dr. Forbes’ office to review the surgical plan in greater detail. During the surgery, a camera known as an endoscope would be passed into Arianna’s nose to provide visualization (Figure 7.3). No external incisions would be used. Arianna was ready to proceed with the surgery.

On the day of the procedure, Dr. Forbes met Arianna and James in the pre-operative waiting area. In the operating room, the otolaryngology team performed the initial exposure and then turned over care to Dr. Forbes. After opening the leathery covering overlying the brain and optic nerves, Dr. Forbes identified the craniopharyngioma. He utilized high magnification with the endoscope to meticulously dissect the tumor from Arianna’s optic nerves. The entire tumor could be removed. Arianna noticed an improvement in her vision almost immediately after awakening from anesthesia.

Arianna and her doctors have been very pleased with her recovery after the surgery. MRIs obtained at six months (Figure 7.4), and twelve months following the operation have shown no evidence of residual or recurrent tumor. Arianna’s vision has been completely restored; all other symptoms have resolved under the care of the endocrinology team. She has been able to restart her hormone therapy regimen to reaffirm her identity as a transgender woman. After defeating struggles with identity, hormonal fluctuation, visual loss, and tumor removal, Arianna was ready to live life to its fullest.

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* a pseudonym