Matthew Matson

COMPARISONS IN THE EPIDEMIOLOGY, DIAGNOSTIC FEATURES AND CURE RATE BY TRANSSPHENOIDAL SURGERY BETWEEN PAEDIATRIC AND ADULT-ONSET CUSHING'S DISEASE

OBJECTIVE There are few published comparisons between paediatric and adult-onset Cushings disease (CD). We compare the epidemiology, diagnostic features and cure rate by transsphenoidal surgery (TSS) in these groups. DESIGN Retrospective review of patient databases in a single university hospital centre. PATIENTS Totally, 41 paediatric (mean age 12.3 ± 3.5 years; range 5.7-17.8) and 183 adult (mean age 40 ± 13 years; range 18.0-95.0) patients with CD were investigated. RESULTS Paediatric CD was characterised by male (63%) and adult CD by a female predominance (79%, P<0.0001). There were small but significant differences in clinical presentation. Biochemical features of CD were comparable except the serum cortisol increase during a CRH test: mean change (105%, n=39) in paediatric and (54%, n=123) in adult subjects (P<0.0001). Macroadenomas were more common in adult (15%, 28/183) than in paediatric (2%, 1/41, P=0.04) CD. Corticotroph microadenomas were more easily visualised by pituitary magnetic resonance imaging (MRI) in adult (76%, 50/66) compared with paediatric (55%, 21/38, P=0.045) CD with poorer concordance of imaging with surgical findings in children (P=0.058). The incidence of ACTH lateralisation by bilateral simultaneous inferior petrosal sinus sampling was comparable in paediatric (76%, 25/33) and adult (79%, 46/58; P=0.95) patients with good surgical concordance in both (82% paediatric and 79% adult). Cure rates by TSS were comparable, with a paediatric cure rate of 69%. CONCLUSION Several features of paediatric CD are distinct: increased frequency of prepubertal CD in males, the different clinical presentation, the decreased presence of macroadenomas and the frequent absence of radiological evidence of an adenoma on MRI.

Diagnosis and localisation of insulinoma: the value of modern magnetic resonance imaging in conjunction with calcium stimulation catheterisation

CONTEXT Preoperative localisation of insulinoma improves cure rate and reduces complications, but may be challenging. OBJECTIVE To review diagnostic features and localisation accuracy for insulinomas. DESIGN Cross-sectional, retrospective analysis. SETTING A single tertiary referral centre. PATIENTS Patients with insulinoma in the years 1990-2009, including sporadic tumours and those in patients with multiple endocrine neoplasia syndromes. INTERVENTIONS Patients were identified from a database, and case notes and investigation results were reviewed. Tumour localisation by computed tomography (CT), magnetic resonance imaging (MRI), octreotide scanning, endoscopic ultrasound (EUS) and calcium stimulation was evaluated. MAIN OUTCOME MEASURE(S) Insulinoma localisation was compared to histologically confirmed location following surgical excision. RESULTS Thirty-seven instances of biochemically and/or histologically proven insulinoma were identified in 36 patients, of which seven were managed medically. Of the 30 treated surgically, 25 had CT (83.3%) and 28 had MRI (90.3%), with successful localisation in 16 (64%) by CT and 21 (75%) by MRI respectively. Considered together, such imaging correctly localised 80% of lesions. Radiolabelled octreotide scanning was positive in 10 out of 20 cases (50%); EUS correctly identified 17 lesions in 26 patients (65.4%). Twenty-seven patients had calcium stimulation testing, of which 6 (22%) did not localise, 17 (63%) were correctly localised, and 4 (15%) gave discordant or confusing results. CONCLUSIONS Preoperative localisation of insulinomas remains challenging. A pragmatic combination of CT and especially MRI predicts tumour localisation with high accuracy. Radionuclide imaging and EUS were less helpful but may be valuable in selected cases. Calcium stimulation currently remains useful in providing an additional functional perspective.

Joint UK societies' 2014 consensus statement on renal denervation for resistant hypertension.

Resistant hypertension continues to pose a major challenge to clinicians worldwide and has serious implications for patients who are at increased risk of cardiovascular morbidity and mortality with this diagnosis. Pharmacological therapy for resistant hypertension follows guidelines-based regimens although there is surprisingly scant evidence for beneficial outcomes using additional drug treatment after three antihypertensives have failed to achieve target blood pressure. Recently there has been considerable interest in the use of endoluminal renal denervation as an interventional technique to achieve renal nerve ablation and lower blood pressure. Although initial clinical trials of renal denervation in patients with resistant hypertension demonstrated encouraging office blood pressure reduction, a large randomised control trial (Symplicity HTN-3) with a sham-control limb, failed to meet its primary efficacy end point. The trial however was subject to a number of flaws which must be taken into consideration in interpreting the final results. Moreover a substantial body of evidence from non-randomised smaller trials does suggest that renal denervation may have an important role in the management of hypertension and other disease states characterised by overactivation of the sympathetic nervous system. The Joint UK Societies does not recommend the use of renal denervation for treatment of resistant hypertension in routine clinical practice but remains committed to supporting research activity in this field. A number of research strategies are identified and much that can be improved upon to ensure better design and conduct of future randomised studies.

The Current Role of Venous Sampling in the Localization of Endocrine Disease

Endocrine venous sampling plays a specific role in the diagnosis of endocrine disorders. In this article, we cover inferior petrosal sinus sampling, selective parathyroid venous sampling, hepatic venous sampling with arterial stimulation, adrenal venous sampling, and ovarian venous sampling. We review their indications and the scientific evidence justifying these indications in the diagnosis and management of Cushing’s syndrome, hyperparathyroidism, pancreatic endocrine tumors, Conn’s syndrome, primary hyperaldosteronism, pheochromocytomas, and androgen-secreting ovarian tumors. For each sampling technique, we compare its diagnostic accuracy with that of other imaging techniques and, where possible, look at how it impacts patient management. Finally, we incorporate venous sampling into diagnostic algorithms used at our institution.

A prospective evaluation of postural stimulation testing, computed tomography and adrenal vein sampling in the differential diagnosis of primary aldosteronism

Context  In primary aldosteronism (PA), discriminating unilateral from bilateral disease is crucial because adrenalectomy is frequently curative in the former case but rarely helps in the latter. Various series have reported the utility of postural stimulation testing (PST), cross‐sectional imaging and adrenal vein sampling (AVS) in the assessment of PA, but most of these studies were retrospective.

The radiological management of the thrombosed arteriovenous dialysis fistula

Patent vascular access is a prerequisite for adequate haemodialysis, and is a major determinant of quality of life and long-term survival of patients with end-stage renal disease. Autogenous haemodialysis fistulas (AVFs) have demonstrated superior clinical outcome when compared to synthetic grafts, but both types of access remain susceptible to venous stenoses, and consequent thrombotic occlusion. Recent publications have reported primary patency rates of up to 100% following percutaneous de-clotting of AVFs incorporating techniques such as pharmacological thrombolysis, mechanical thrombectomy, and thrombo-aspiration. Endovascular management also provides information regarding the underlying cause of access thrombosis, with option to treat. Consequently, there has been a paradigm shift in the management of fistula thrombosis, with interventional radiology assuming a lead role in initial salvage procedures. This article will attempt to provide the reader with an insight into the multiple radiological techniques that can be employed to salvage a thrombosed AVF based on current published literature.

Diagnosing unilateral primary aldosteronism - comparison of a clinical prediction score, computed tomography and adrenal venous sampling.

In patients with primary aldosteronism (PA), adrenalectomy is potentially curative for those correctly identified as having unilateral excessive aldosterone production. It has been suggested that a recently developed and published clinical prediction score (CPS) may correctly identify some patients as having unilateral disease, without recourse to adrenal venous sampling.

Endonasal endoscopic transsphenoidal pituitary surgery: early experience and outcome in paediatric Cushing's disease

Selective adenomectomy remains the first‐line treatment for Cushings disease (CD), until recently by microscopic transsphenoidal pituitary surgery. Endonasal transsphenoidal endoscopic surgery (ETES) is emerging as a novel, less invasive treatment for pituitary adenomas and has become the optimal surgical approach.

64-section CT angiography in patients with critical limb ischaemia and severe claudication: Comparison with digital subtractive angiography

AIM To assess the utility of 64 section multidetector computed tomography (MDCT) lower-limb angiography in the evaluation of patients with critical limb ischaemia (CLI) or severe intermittent claudication (IC) in grading disease before endovascular treatment. MATERIALS AND METHODS Forty-one consecutive patients with CLI or severe IC were assessed using 64 section MDCT angiography. The MDCT examinations were compared with subsequent intra-arterial digital subtraction angiography (IADSA) examinations performed at the time of endovascular intervention. The MDCT and IADSA examinations were independently scrutinized by readers blinded to the results of the other imaging method. RESULTS For arterial segments with haemodynamically significant disease (stenosis ≥50%), the overall sensitivity, specificity, and accuracy of MDCT in patients with severe claudication and CLI was 99% (95% CI: 98-100%), 98% (95% CI: 97-100%) and 98% (95% CI: 97-99%), respectively. The positive predictive value (PPV) was 97% and the negative predictive value (NPV) was 99%. CONCLUSIONS MDCT angiography is a useful tool in the assessment of patients with severe claudication and CLI and can be reliably used to grade disease severity and plan treatment.

Mechanical Thrombectomy of Occluded Hemodialysis Native Fistulas and Grafts Using a Hydrodynamic Thrombectomy Catheter: Preliminary Experience

The purpose of this study was to evaluate the efficacy and safety of a new hydrodynamic percutaneous thrombectomy catheter in the treatment of thrombosed hemodialysis fistulas and grafts. Twenty-two patients (median age: 47 years; range: 31–79 years) underwent mechanical thrombectomy for thrombosed hemodialysis fistulas or polytetrafluoroethylene (PTFE) grafts. In all cases, an Oasis hydrodynamic catheter was used. Five patients had native fistulas and 17 had PTFE grafts. Six patients required repeat procedures. All patients with native fistulas and 15 of the 17 with PTFE grafts also underwent angioplasty of the venous limb following the thrombectomy. Major outcome measures included technical success, clinical success, primary and secondary patency, and complication rates. Twenty-eight procedures were performed in total. The technical success rate was 100% and 90% and clinical success was 86% and 76% for native fistulas and grafts, respectively. The primary patency at 6 months was 50% and 59% for fistulas and grafts, respectively, and the secondary patency at 6 months was 75% and 70% for fistulas and grafts, respectively. Two patients died of unrelated causes during the follow-up period. The Oasis catheter is an effective mechanical device for the percutaneous treatment of thrombosed hemodialysis access. Our initial success rate showed that the technique is safe in the treatment of both native fistulas and grafts.