To block, or not to block is it still the question? Effectiveness of alpha- and beta-blockade in phaeochromocytoma surgery: an institutional analysis.

INTRODUCTION: Phaeochromocytomas/paraganglioma (PPGL) surgery was historically associated with significant risks of perioperative complications. The decreased mortality (<3.0%) had been attributed in part to optimal preoperative alpha-blockade. The value of alpha-blockade in decreasing morbidity is being challenged. The aim of our study is to present an institutional experience of preoperative alpha-blocking of PPGL and its effect on cardiovascular stability and postoperative morbidity. METHODS: A retrospective study using data from our institutional database was conducted. All patients undergoing adrenalectomy for PPGL from October 2011 to September 2020 were included. All patients were routinely alpha-blocked. Intraoperative cardiovascular instability (ICI) was assessed through number of systolic blood pressure (SBP) episodes >160mmHg, SBP <90mmHg, the need for vasoactive drugs and volume of intraoperative crystalloids administered. Morbidity was also evaluated. RESULTS: A total of 100 consecutive patients undergoing surgery were identified of whom 53 patients had complete anaesthetic records available for analysis. Thirty-two patients (60%) had at least one episode with an SBP >160mmHg. Nine (17%) cases had no intraoperative hypotensive episodes, while 3 (6%) patients had >10 intraoperative episodes of an SBP <90mmHg. Twenty-one (40%) patients received vasoactive drugs during surgery. The median volume of intraoperative crystalloids was 2 litres (1-4). Postoperatively, no patient experienced cardiovascular complications, including arrhythmia or myocardial ischaemia. Only two were admitted to an intensive care unit (ICU) and one 30-day readmission occurred. CONCLUSIONS: Cardiac instability remained significant in PPGL surgery despite optimal alpha- and beta-blockade. While omitting blockade would appear empirically questionable, a randomised controlled trial (RCT) of surgery with and without alpha-blockade will provide an answer.

To block, or not to block is it still the question? Effectiveness of alpha- and beta-blockade in phaeochromocytoma surgery: an institutional analysis.

INTRODUCTION: Phaeochromocytomas/paraganglioma (PPGL) surgery was historically associated with significant risks of perioperative complications. The decreased mortality (<3.0%) had been attributed in part to optimal preoperative alpha-blockade. The value of alpha-blockade in decreasing morbidity is being challenged. The aim of our study is to present an institutional experience of preoperative alpha-blocking of PPGL and its effect on cardiovascular stability and postoperative morbidity. METHODS: A retrospective study using data from our institutional database was conducted. All patients undergoing adrenalectomy for PPGL from October 2011 to September 2020 were included. All patients were routinely alpha-blocked. Intraoperative cardiovascular instability (ICI) was assessed through number of systolic blood pressure (SBP) episodes >160mmHg, SBP <90mmHg, the need for vasoactive drugs and volume of intraoperative crystalloids administered. Morbidity was also evaluated. RESULTS: A total of 100 consecutive patients undergoing surgery were identified of whom 53 patients had complete anaesthetic records available for analysis. Thirty-two patients (60%) had at least one episode with an SBP >160mmHg. Nine (17%) cases had no intraoperative hypotensive episodes, while 3 (6%) patients had >10 intraoperative episodes of an SBP <90mmHg. Twenty-one (40%) patients received vasoactive drugs during surgery. The median volume of intraoperative crystalloids was 2 litres (1-4). Postoperatively, no patient experienced cardiovascular complications, including arrhythmia or myocardial ischaemia. Only two were admitted to an intensive care unit (ICU) and one 30-day readmission occurred. CONCLUSIONS: Cardiac instability remained significant in PPGL surgery despite optimal alpha- and beta-blockade. While omitting blockade would appear empirically questionable, a randomised controlled trial (RCT) of surgery with and without alpha-blockade will provide an answer.

IgG4-related disease in a multi-ethnic community: clinical characteristics and association with malignancy.

BACKGROUND: Immunoglobulin-G4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory condition that can affect multiple organs. Despite growing interest in this condition, the natural history and management of IgG4-RD remain poorly understood. AIM: To describe the clinical characteristics, treatment and outcomes of IgG4-RD in a multi-ethnic UK cohort, and investigate its possible association with malignancy. DESIGN: Retrospective analysis of case-note and electronic data. METHODS: Cases were identified from sub-specialty cohorts and a systematic search of an NHS trust histopathology database using 'IgG4' or 'inflammatory pseudotumour' as search terms. Electronic records, imaging and histopathology reports were reviewed. RESULTS: In total, 66 identified cases of IgG4-RD showed a similar multi-ethnic spread to the local population of North West London. The median age was 59 years and 71% of patients were male. Presenting symptoms relating to mass effect of a lesion were present in 48% of cases and the mean number of organs involved was 2.4. Total of 10 patients had reported malignancies with 6 of these being haematological. 83% of those treated with steroids had good initial response; however, 50% had relapsing-remitting disease. Rituximab was administered in 11 cases and all achieved an initial serological response. Despite this, seven patients subsequently relapsed after a mean duration of 11 months and four progressed despite treatment. CONCLUSIONS: We report a large UK-based cohort of IgG4-RD that shows no clear ethnic predisposition and a wide range of affected organs. We discuss the use of serum IgG4 concentrations as a disease marker in IgG4-RD, the association with malignant disease and outcomes according to differing treatment regimens.