Endocrine Abstracts

Introduction: Puberty is the process of transition from childhood to adult reproductive capability, body composition, and adult height. In males, the lack of Tanner stage progression after achieving a testicular size of at least 4 cubic cm (cc) suggests pubertal arrest. While delayed puberty could be a normal variant, arrested puberty is usually pathological, and points towards an acquired pathology rather than a congenital disorder. We describe an unusual case of arrested puberty from subclinical pituitary apoplexy.

Case report: An 18-year-old male was referred to our adult endocrine clinic with arrested puberty. Initial examination revealed short stature (height 163 cm), eunuchoid body habitus, a high-pitched voice and minimal secondary sexual characteristics (Tanner Stage 1 for pubic hair). Bilateral testes were descended with testicular volumes of 8cc (Tanner Stage 2 for external genitalia). Initial investigations revealed central hypothyroidism (FT4 8.0 pmol/l, TSH 1.54 mIU/l), hypogonadotropic hypogonadism (total testosterone <1 nmol/l, FSH 2 IU/l, LH 2 IU/l) and growth hormone deficiency (GHD) (IGF-1 43 mg/l). The bone age was delayed at 15 years old when the patient’s chronological age was 18 years old and 9 months. MRI brain showed a 2.6 cm T1- hyperintense sellar-suprasellar mass with chiasmal compression, suggestive of pituitary apoplexy. Endonasal transsphenoidal marsupialisation was performed and histology revealed necrotic tissue. His post-operative course was complicated by pituitary abscess, requiring surgical drainage. He developed panhypopituitarism and central diabetes insipidus and was treated with thyroxine, hydrocortisone and intranasal desmopressin. Glucagon stimulation test confirmed persistent GHD (peak GH 0.06 mg/l). SC somatropin 1.2 mg daily was initiated to optimise adult height. The patient was counselled regarding options for puberty induction including testosterone replacement or gonadotrophin therapy. He opted for testosterone replacement and was initiated on IM testosterone 50 mg every 4 weeks. Whilst on concurrent growth hormone replacement and puberty induction, he grew 8 cm over 11 months with increase in penile length and progression to Tanner stage 2.

Conclusion: Pubertal arrest is usually pathological. In this case, pubertal arrest was an atypical presentation of subclinical pituitary apoplexy. Puberty induction in an adolescent with delayed presentation of pubertal arrest and hypopituitarism is challenging. It requires careful titration of multiple hormonal regimens to optimise the final adult height, secondary sexual characteristics and bone health.