Introduction: Pharmacological treatments are still not ideal in ACTH-microadenomas and transnasal-trans-sphenoidal surgery (TSS) is first choice in the treatment of Cushings disease (CD). The question is how can a nearly 100% remission rate be achieved with minimally invasive diagnostic testing and TSS. In the discussion of the main lecture from an expert from the USA in ECE13, it became clear that in many European centres, like ours, the procedures are less invasive. The developments in our centre treating 100 paediatric Cushing patients will be presented.
Material and methods: Data from published series (n=55) of the author will be compared with new data from our recent series (n=45). All patients had been operated when MRI and direct transnasal microsurgery (TSS) were established. Special diagnostic methods like inferior petrosal sampling (IPSS) were replaced by ACTH measurement from the cavernous sinus (CSS) in unclear cases without increase of salivary cortisol in the CRH-test or difficult sella anatomy and negative MRI. Micro-histology, micro-doppler and the importance of a micro-suction irrigation system for visualization and minimization of trauma, will be described.
Results: In our first series of 55 cases, IPSS was performed in 13 cases and showed 46% false lateralization. Only with extensive pituitary exploration could most adenomas be removed (n=52). Three patients had early successful re-surgery. Second series of 45 cases with refined diagnostic tests and preoperative cavernous sinus sampling (n=7), nearly all micro-adenomas were initially detected. Re-surgery became necessary for remission in three. Side effects of surgery were minimal, so children and parents readily accepted re-operation. Thus 98% of 100 patients had long-term remission.
Conclusions: Invasive pre-surgical investigations were mostly avoided, yet with advanced TSS, remission rates of more the 95% could still be achieved. Using feedback from direct postoperative hormone measurements, our remission rates could be improved without increasing complications.
03 - 07 May 2014
European Society of Endocrinology