Endocrine Abstracts

Selective transsphenoidal adenomectomy remains the accepted first line treatment for Cushing’s disease (CD), until recently by microscopic (sublabial) transsphenoidal pituitary surgery. Endonasal transsphenoidal endoscopic surgery is emerging as a novel, less invasive treatment for pituitary adenomas with lower postoperative complications and morbidity. The safety of endoscopic surgery has been extensively reviewed in adult patients and is now considered best practice for the treatment of pituitary tumours. There are no published series of the treatment of paediatric CD by endoscopic pituitary surgery and we report our centre’s preliminary results.

Methods: Four paediatric patients (median age 14.4 years; range 11.7–16.8 years) fulfilled standard diagnostic criteria for CD. Preoperatively no abnormality was identified on pituitary MR scanning in three (75%) patients. Bilateral petrosal sinus sampling demonstrated central ACTH secretion (IPS/P ACTH ratio >2.0, pre- or post-CRH) in three (75%) patients with lateralisation of ACTH secretion (IPSG post-CRH ≥1.4) in two patients. The same neurosurgeon and endoscopic nasal surgeon undertook all the operations. ‘Cure’ was defined as a 0900 h cortisol level of <50 nmol/l postoperatively on two separate occasions associated with regression of the clinical features of CD.

Results: Clinical recovery and biochemical ‘cure’ was achieved in 3 (75%) patients and a corticotroph adenoma was confirmed histologically in all cured cases. One case developed postoperative CSF leak requiring lumbar drain insertion and patching. At a mean interval of 5.9 years (0.49.8 years) postoperatively, cured patients have shown no recurrence. One patient, who had a large diffuse adenoma requiring more extensive surgery has panhypopituitarism, another patient has GH and gonadotrophin deficiencies.

Conclusions: Our experience shows that endonasal transsphenoidal endoscopic surgery for removing corticotroph adenomas in children, in most cases not visualized on MRI imaging, is minimally invasive and gave excellent postoperative recovery/results. In skilled hands this technique provides an alternative to conventional transsphenoidal microscopic surgery in managing paediatric CD.