Introduction: Trans-sphenoidal surgery (TSS) with minimally invasive techniques is the first choice in the treatment of paediatric Cushings disease (CD). The question is how can high remission rates be achieved with less invasive investigations and TSS. The developments in our centre treating 100 pediatric Cushing patients with long-term follow-up may add some helpful ideas.
Material and methods: Data from our first series 1 (n=55) will be compared with new data from the recent series 2 (n=45) until 2009. All patients were operated by one surgeon by direct transnasal microsurgery (TNS). Special diagnostic methods such as inferior petrosal sinus sampling (IPSS) were replaced by ACTH measurement from the cavernous sinus (CSS) restricted to unclear cases without increase of salivary cortisol in the CRH-test, difficult sella anatomy and/or negative MRI. Multiple direct micro-cytology, micro-doppler and adequate visualization will be described.
Results: In our first series of 55 cases, IPSS was performed in 13 (24%) of whom 46% had false adenoma lateralization. All adenomas could be removed with extensive pituitary exploration and all had intraoperative pathology. Two patients had early successful re-surgery. Recurrence rate 15%. In the second series with more refined MRI and endocrinology: easy repeat CRH saliva tests and CSS sampling used in only 7 patients (15%), all micro-adenomas were initially detected. Early repeat-TNS was necessary and successful in three. Recurrence rate 11%. Side effects of TNS were minimal, as such, children and parents readily accepted re-TNS. Thus, including re-TNS in recurrences, 98 of 100 patients had long-term remission. TNS in recurrences had a better outcome in the last series with less pituitary dysfunction using more intraoperative minute biopsies.
Conclusions: Invasive pre-surgical investigations could be reduced. 98% remission rate could be achieved with advanced investigations, refined TNS, early re-TNS and re-TNS in recurrences. Only two children (2%) were irradiated only one was indicated by the senior surgeon.
20 May 2017 - 23 May 2017