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Unexplained or idiopathic pituitary stalk thickening or central diabetes insipidus not only harbours rare occult malignancies in 40% of cases but can also reflect benign congenital defects. Between 2014 and 2019, a multidisciplinary, expert national guideline development group in the UK systematically developed a management flowchart and clinical practice guideline to inform specialist care and improve outcomes in children and young people (aged

Aim To develop a guideline for the investigation and management of children and young people up to the age of 19 years with idiopathic thickened pituitary stalk (iTPS) and/or central diabetes insipidus (iCDI) where the aetiology is not apparent at presentation. Methods The guideline development group (GDG) identified the objectives and the clinical questions which needed to be addressed. These were reviewed by guideline stakeholders and used to direct a systematic literature search. Published evidence was appraised using the GRADE system. Where the literature search identified sufficient evidence, the GDG made a guideline recommendation. Where there was insufficient evidence, the GDG drafted recommendations based on their expert opinion and reviewed these using a formal Delphi consensus process. This was a joint society (BSPED/CCLG) multidisciplinary national endeavour done to NICE methodology and overseen by RCPCH. Results The literature search identified 568 articles covering the period Jan 1990 –March 2017. The most commonly reported causes of iTPS and iCDI in children are Langerhans Cell Hystiocytosis (LCH), Germ Cell Tumours (GCT) and craniopharingioma (CP). The average prevalence of LCH, GCT and CP in 11 case series (including 741 patients) is 16%, 13% and 12%, respectively. Overall, congenital defects are responsible for 19% of the iTPS/iCDI cases, whilst infectious diseases (2%), trauma (1%) and inflammatory/autoimmune conditions (1%) rarely occur in children. In 29% of the cases no aetiology is identified. Causes of pituitary stalk lesions in adults, metastatic tumours and neurosarcoidosis, do not form part of the differential diagnosis in children. What constitutes a TPS is not consistently defined across studies. High quality evidence was lacking for the majority of the clinical questions and two rounds of Delphi consensus were undertaken. A decision-making flowchart has been developed and will accompany the guideline. Conclusion The likely aetiology of iTPS and iCDI in children differs from that in adults and justifies the development of age appropriate decision making management guidelines to inform best practice nationally. This will form the basis for future audits of practice and outcomes and is intended to improve the quality of care of children and young people with iTPS and iCDI.

AIM: To develop a high-quality national guideline for the assessment and management of CYP presenting with iTPS and/or iCDI before their 19th birthday, as a joint endeavour by the Britsh paediatric endocrine and oncology societies (BSPED/CCLG) and meeting approved commissioning standards (RCPCH/NIHCE). METHODS: The interdisciplinary guideline development group (GDG) identified 64 clinical questions. These were reviewed by stakeholders and used to direct a systematic literature search (January 1990 - March 2017). 568 articles were appraised using the GRADE system. Where there was sufficient evidence, the GDG made a guideline recommendation. Where high quality evidence was lacking, the GDG drafted recommendations based on their expert opinion and reviewed these using two rounds of Delphi consensus with international experts. RESULTS: In 11 case series (741 paediatric patients) the commonest individual causes of TPS/CDI were Langerhans cell hystiocytosis (16%), germ cell tumours (13%) and craniopharingiomas (12%). A range of congenital defects accounted for 19% of cases. Infectious diseases (2%), trauma (1%) and inflammatory/autoimmune conditions (1%) were rare. Twenty-nine percent remained idiopathic and some causes of TPS in adults (metastatic tumours and neurosarcoidosis) were not reported in children. The definition of TPS was not consistent across studies. A guideline and decision-making flowchart were developed. CONCLUSION: The likely aetiology of TPS/CDI in children differs from that in adults and justifies the development of age-appropriate management guidelines. This will form the basis of future audits of practice and outcomes and is intended to improve the care and service provision to CYP with apparent idiopathic TPS/CDI.

Background and Purpose— The purpose of this study was to assess the effect of postcontrast CT (PCCT) leakage (PCL) on hematoma growth in CTA spot negative patients. Methods— A retrospective study of 61 patients presenting within 6 hours of primary ICH onset imaged with CT angiography (CTA) and PCCT. Presence of CTA spot sign and PCL were documented. PCL was defined as the presence of contrast extravasation on the PCCT study at a location remote from the CTA spot sign if present. Hematoma expansion was defined as >6 mL or 30% hematoma enlargement. Patients were dichotomized by CTA spot sign presence and PCL and compared for baseline demographic data, hematoma size, and growth using the unpaired t test and Mann-Whitney test for continuous and categorical data, respectively. A probability value Results— PCL was present in 11/61 patients (18%), occurring in 5 without a spot sign (45%). Spot negative PCL patients demonstrated larger absolute ( P =0.02) and percentage hematoma growth ( P =0.02) compared to those without PCL. The mean volume and percent increase was 6.7 mL and 26%, respectively. Inclusion of PCL together with CTA spot sign as risk factor for hematoma expansion increased sensitivity from 0.78 (95% CI; 0.52 to 0.94) to 0.94 (95% CI; 0.72 to 1.00) and NPV from 0.90 (95% CI; 0.76 to 0.97) to 0.97 (95% CI; 0.85 to 1.00). Conclusion— Inclusion of PCCT in the investigation of ICH patients allows detection of PCL which, together with the CTA spot sign, increases sensitivity and negative predictive value for predicting hematoma expansion. This finding should be validated in larger studies.

Connections between the thalamus and cortex develop rapidly before birth, and aberrant cerebral maturation during this period may underlie a number of neurodevelopmental disorders. To define functional thalamocortical connectivity at the normal time of birth, we used functional MRI (fMRI) to measure blood oxygen level-dependent (BOLD) signals in 66 infants, 47 of whom were at high risk of neurocognitive impairment because of birth before 33 wk of gestation and 19 of whom were term infants. We segmented the thalamus based on correlation with functionally defined cortical components using independent component analysis (ICA) and seed-based correlations. After parcellating the cortex using ICA and segmenting the thalamus based on dominant connections with cortical parcellations, we observed a near-facsimile of the adult functional parcellation. Additional analysis revealed that BOLD signal in heteromodal association cortex typically had more widespread and overlapping thalamic representations than primary sensory cortex. Notably, more extreme prematurity was associated with increased functional connectivity between thalamus and lateral primary sensory cortex but reduced connectivity between thalamus and cortex in the prefrontal, insular and anterior cingulate regions. This work suggests that, in early infancy, functional integration through thalamocortical connections depends on significant functional overlap in the topographic organization of the thalamus and that the experience of premature extrauterine life modulates network development, altering the maturation of networks thought to support salience, executive, integrative, and cognitive functions.