BSPED2015 e-Posters Pituitary and growth (18 abstracts)
Achieving a consensus on managing idiopathic thickening of the pituitary stalk through a national multidisciplinary forum, meeting virtually
Aparna Kesavath Raman Nambisan 1 , Maria Michaelidou 2 , Eftychia Dimitrakopoulou 1 , Ash Ederies 1 , Helen Spoudeas 1 , Peter Clayton 3 , Indi Banerjee 3 , Johannes Visser 3 , Jo Blair 3 , Evelien Gevers 3 , Mehul Dattani 3 , Kristian Aquilina 3 , Benedetta Pettorini 3 , Assunta Albanese 3 , Ian Kamaly 3 , Marta Korbonits 3 , Antony Michalski 3 , Darren Hargrave 3 , Barry Pizer 3 & Yen Chang 3
1Great Ormond Street Hospital, London, UK; 2University of Manchester, Manchester, UK; 3On behalf of the HPAT Group, MDT, UK.
Objectives: In 2010 we piloted a national multidisciplinary (MDT), meeting virtually to improve management of rare suprasellar (HPAT) tumours. In 2014 we reported centralised treatment decision-making in craniopharyngioma and now wished to examine whether centre based management of idiopathic thickening of the pituitary stalk (iTPS) differs and can be streamlined by wider debate. This might also inform current commissioned BSPED and CCLG guidance.
Methods: We assessed centre uptake of HPAT MDT recommendations of 20 iTPS cases from five centres between 1.1.12 and 1.6.15 and their endocrine outcomes by case note review. MRI 3D volume was analysed with ITK-SNAP v 3.2.0 (www.itksnap.org) Software and with centralised radiology review.
Results: Patients presented at mean age 8.9 (range 3–16) years with low height −0.8 (−4.5 to 1.1) and weight −0.2 (−3.63 to 2.26) SDS but higher BMI +0.44 (−1.59 to 2.43) SDS. 75% (15) had polydipsia but 80% (16) confirmed DI, 55%(11) were GHd with ACTHd (4), TSHd (2), or Gnd(1) and two had visual dysfunction. One centre presented two biopsied histiocytosis cases. In 60% (12) a watch and wait strategy was recommended, and centres deferred GH replacement in 50%. All underwent PST, serum HCG and AFP, but only 55% had CSF markers, 66% plasma ACE, and 77% skeletal surveys and ophthalmic review. MDT discussion influenced management in 40% and debate in 60%. At an average 2 (0.26–4.5) years follow up, three more (15%) developed TSHd and ACTHd but not DI or GHd. Height 0.2 (−3.4 to 2.3) SDS improved and BMI +0.46 (−3.1 to 3.7) were unchanged. 8 (40%) cases remained idiopathic with stable scans, TPS disappeared in 2 (10%) within 2 years, decreased in 3 (15%), worsened in 1 (5%) and 20% awaited scans. MRI 3Dvolume did not correlate with initial endocrinopathy.
Conclusion: Centres have valued the opportunity to discuss this rare and complex condition where management is controversial and differs. This forum has enabled wider discussion, consensus and audit of practice and outcomes which can inform national guidance.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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