Insights on Epidemiology, Morbidity and Mortality of Cushing's Disease in Northern Ireland.

Cushing's disease is a rare condition occurring due to an adrenocorticotrophin-producing corticotrophinoma arising from the pituitary gland. The consequent hypercortisolaemia results in multisystem morbidity and mortality. This study aims to report incidence, clinicopathological characteristics, remission outcomes and mortality in a regional pituitary neurosurgical cohort of patients diagnosed with Cushing's disease in Northern Ireland from 2000-2019. Clinical, biochemical and radiological data from a cohort of patients operated for Cushing's disease were retrospectively collected and analysed. Fifty-three patients were identified, resulting in an estimated annual incidence of Cushing's disease of 1.39-1.57 per million population per year. Females accounted for 72% (38/53) of the cohort. The majority (74%, 39/53) of corticotrophinomas were microadenomas and in 44% (17/39) of these no tumour was identified on preoperative magnetic resonance imaging. Histopathological characterisation was similarly difficult, with no tumour being identified in the histopathological specimen in 40% (21/53) of cases. Immediate postoperative remission rates were 53% and 66% when considering serum morning cortisol cut-offs of ≤50nmol/L (1.8µg/dL) and ≤138nmol/L (5µg/dL) respectively in the week following pituitary surgery. Approximately 70% (37/53) of patients achieved longer term remission with a single pituitary surgery. Three patients had recurrent disease. Patients with Cushing's disease had a significantly higher mortality rate compared to the Northern Ireland general population (standardised mortality ratio 8.10, 95% confidence interval 3.3 - 16.7, p<0.001). Annual incidence of Cushing's disease in Northern Ireland is consistent with other Northern European cohorts. Functioning corticotrophinomas are a clinically, radiologically and histopathologically elusive disease with increased mortality compared to the general population.

Clinical management and outcome of head and neck paragangliomas (HNPGLs): A single centre retrospective study.

CONTEXT: Head and neck paragangliomas (HNPGLs) are rare, usually benign, slow-growing tumours arising from neural crest-derived tissue. Definitive management pathways for HNPGLs have yet to be clearly defined. OBJECTIVE: To review our experience of the clinical features and management of these tumours and to analyse outcomes of different treatment modalities. METHODS: Demographic and clinical data were obtained from The Northern Ireland Electronic Care Record (NIECR) as well from a prospectively maintained HNPGL database between January 2011 through December 2023. RESULTS: There were 87 patients; 50 females: 37 males with a mean age of 52.3 ± 14.2 years old (range 17-91 years old). 58.6% (n = 51) of patients had carotid body tumours, 25.2% (n = 22) glomus vagal tumours, 6.8% (n = 6) tumours in the middle ear, 2.2% (n = 2) in the parapharyngeal space and 1.1% (n = 1) in the sphenoid sinus. 5.7% (n = 5) of patients had multifocal disease. The mean tumour size at presentation was 3.2 ± 1.4 cm (range 0.5-6.9 cm). Pathogenic SDHD mutations were identified in 41.3% (n = 36), SDHB in 12.6% (n = 11), SDHC in 2.2% (n = 2) and SDHA in 1.1% (n = 1) of the patients. Overall treatment modalities included surgery alone in 51.7% (n = 45) of patients, radiotherapy in 14.9% (n = 13), observation in 28.7% (n = 25), and somatostatin analogue therapy with octreotide in 4.5% (n = 4) of patients. Factors associated with a significantly higher risk of recurrence included age over 60 years (p = .04), tumour size exceeding 2 cm (p = .03), positive SDHx variants (p = .01), and vagal and jugular tumours (p = .04). CONCLUSION: The majority of our patients underwent initial surgical intervention and achieved disease stability. Our results suggest that carefully selected asymptomatic or medically unfit patients can be safely observed provided lifelong surveillance is maintained. We advocate for the establishment of a UK and Ireland national HNPGL registry, to delineate optimal management strategies for these rare tumours and improve long term outcomes.

Generating Operative Workflows for Vestibular Schwannoma Resection: A Two-Stage Delphi's Consensus in Collaboration with the British Skull Base Society. Part 2: The Translabyrinthine Approach.

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 2, we present a codified operative workflow for the translabyrinthine approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Seventeen consultant skull base surgeons (nine neurosurgeons and eight ENT [ear, nose, and throat]) with median of 13.9 years of experience (interquartile range: 18.1 years) of independent practice participated. There was a 100% response rate across both the Delphi rounds. The translabyrinthine approach had the following five phases and 57 unique steps: Phase 1, approach and exposure; Phase 2, mastoidectomy; Phase 3, internal auditory canal and dural opening; Phase 4, tumor debulking and excision; and Phase 5, closure. Conclusion We present Part 2 of a national, multicenter, consensus-derived, codified operative workflow for the translabyrinthine approach to vestibular schwannomas. The five phases contain the operative, steps, instruments, technique errors, and event errors. The codified translabyrinthine approach presented in this manuscript can serve as foundational research for future work, such as the application of artificial intelligence to vestibular schwannoma resection and comparative surgical research.

Generating Operative Workflows for Vestibular Schwannoma Resection: A Two-Stage Delphi's Consensus in Collaboration with the British Skull Base Society. Part 1: The Retrosigmoid Approach.

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 1, we present a codified operative workflow for the retrosigmoid approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus, was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Eighteen consultant skull base surgeons (10 neurosurgeons and 8 ENT [ear, nose, and throat]) with median 17.9 years of experience (interquartile range: 17.5 years) of independent practice participated. There was a 100% response rate across both Delphi's rounds. The operative workflow for the retrosigmoid approach contained three phases and 40 unique steps as follows: phase 1, approach and exposure; phase 2, tumor debulking and excision; phase 3, closure. For the retrosigmoid approach, technique, and event error for each operative step was also described. Conclusion We present Part 1 of a national, multicenter, consensus-derived, codified operative workflow for the retrosigmoid approach to vestibular schwannomas that encompasses phases, steps, instruments, technique errors, and event errors. The codified retrosigmoid approach presented in this manuscript can serve as foundational research for future work, such as operative workflow analysis or neurosurgical simulation and education.

Measuring hip migration percentage in cerebral palsy using the HipScreen app.

Aims: Reimers migration percentage (MP) is a key measure to inform decision-making around the management of hip displacement in cerebral palsy (CP). The aim of this study is to assess validity and inter- and intra-rater reliability of a novel method of measuring MP using a smart phone app (HipScreen (HS) app). Methods: A total of 20 pelvis radiographs (40 hips) were used to measure MP by using the HS app. Measurements were performed by five different members of the multidisciplinary team, with varying levels of expertise in MP measurement. The same measurements were repeated two weeks later. A senior orthopaedic surgeon measured the MP on picture archiving and communication system (PACS) as the gold standard and repeated the measurements using HS app. Pearson's correlation coefficient (r) was used to compare PACS measurements and all HS app measurements and assess validity. Intraclass correlation coefficient (ICC) was used to assess intra- and inter-rater reliability. Results: All HS app measurements (from 5 raters at week 0 and week 2 and PACS rater) showed highly significant correlation with the PACS measurements (p < 0.001). Pearson's correlation coefficient (r) was constantly over 0.9, suggesting high validity. Correlation of all HS app measures from different raters to each other was significant with r > 0.874 and p < 0.001, which also confirms high validity. Both inter- and intra-rater reliability were excellent with ICC > 0.9. In a 95% confidence interval for repeated measurements, the deviation of each specific measurement was less than 4% MP for single measurer and 5% for different measurers. Conclusion: The HS app provides a valid method to measure hip MP in CP, with excellent inter- and intra-rater reliability across different medical and allied health specialties. This can be used in hip surveillance programmes by interdisciplinary measurers.

The utility of 16S rRNA gene sequencing on intraoperative specimens from intracranial infections: an 8-year study in a regional UK neurosurgical unit.

Background: Optimal management of intracranial infections relies on microbiological diagnosis and antimicrobial choice, but conventional culture-based testing is limited by pathogen viability and pre-sampling antimicrobial exposure. Broad-range 16S rRNA gene sequencing has been reported in the management of culture-negative infections but its utility in intracranial infection is not well-described. We studied the efficacy of 16S rRNA gene sequencing to inform microbiological diagnosis and antimicrobial choice in intracranial infections.Methods: This was a retrospective study of all intraoperative neurosurgical specimens sent for 16S rRNA gene sequencing over an 8-year period at a regional neurosurgical centre in the UK. Specimen selection was performed using multidisciplinary approach, combining neurosurgical and infection specialist discussion.Results: Twenty-five intraoperative specimens taken during neurosurgery from 24 patients were included in the study period. The most common reason for referral was pre-sampling antimicrobial exposure (68%). Bacterial rDNA was detected in 60% of specimens. 16S rRNA gene sequencing contributed to microbiological diagnosis in 15 patients and informed antimicrobial management in 10 of 24 patients with intracranial infection. These included targeted antibiotics after detection of a clinically-significant pathogen that had not been identified through other microbiological testing (3 cases), detection of commensal organisms in neurosurgical infection which justified continued broad cover (2 cases) and negative results from intracranial lesions with low clinical suspicion of bacterial infection which justified avoidance or cessation of antibiotics (5 cases).Conclusion: Overall, 16S rRNA gene sequencing represented an incremental improvement in diagnostic testing and was most appropriately used to complement, rather than replace, conventional culture-based testing for intracranial infection.

Research priorities in children requiring elective surgery for conditions affecting the lower limbs: a James Lind Alliance Priority Setting Partnership.

OBJECTIVE: To identify and prioritise research questions concerning the elective surgical management of children with conditions affecting the lower limb by engaging patients, carers and healthcare professionals. DESIGN: A modified nominal group technique. SETTING: UK. PARTICIPANTS: 388 individuals (29 patients, 155 parents/carers, 204 healthcare professionals) were recruited through hospital clinics, patient charities and professional organisations and participated in the initial prioritisation survey; 234 individuals took part in the interim prioritisation survey. 33 individuals (3 patients, 9 parents/carers, 11 healthcare professionals, 7 individuals representing the project's steering group and 3 James Lind Alliance (JLA) facilitators) attended the final face-to-face workshop to rank the top 10 research priorities. INTERVENTIONS: Surveys were distributed using various media resources such as newsletters, internet messaging boards and the 'Paediatric Lower Limb Surgery Priority Setting Partnership (PSP) website. Printed copies of the questionnaire were also made available to families in outpatient clinics. OUTCOME MEASURES: Survey results, top 10 and top 26 priority rankings RESULTS: The process took 18 months to complete (July 2017-January 2019); 388 people generated 1023 questions; a total of 801 research questions were classified as true uncertainties. Following the JLA methodology, 75 uncertainties were developed from the initial 801 questions. Twenty six of those were selected through a second survey and were taken to the final face-to-face workshop where the top 10 research priorities were selected. The top10 priorities included questions on cerebral palsy, common hip conditions (ie, Perthes' disease and developmental dysplasia of the hip) as well as rehabilitation techniques and methods to improve shared decision-making between clinicians and patients/families. CONCLUSIONS: This is the first JLA PSP in children's orthopaedic surgery, a particularly under-researched and underfunded area. We have identified important research topics which will guide researchers and funders and direct their efforts in future research.

Universal versus selective ultrasound screening for developmental dysplasia of the hip: a single-centre retrospective cohort study.

A total of 28 068 infants were studied to investigate whether universal ultrasound screening for developmental dysplasia of the hip results in fewer delayed presentations than selective screening, and whether the screening protocol affects subsequent treatment. The rate of delayed presentation was not lower with selective screening compared with universal screening but all cases of delayed presentation in this cohort were administrative errors. There were no sonographic errors (false-negatives). The treatment rate was significantly higher with universal screening but infants were treated earlier and were significantly less likely to require any surgical intervention. Those requiring surgery were less likely to require open reduction or pelvic osteotomy.

Opinion survey of members of British Society of Children's Orthopaedic Surgery related to specific case scenarios in slipped capital femoral epiphysis.

The aim of this study was to assess contemporary management of slipped capital femoral epiphysis (SCFE) by surveying members of the British Society of Children's Orthopaedic Surgery (BSCOS). A questionnaire with five case vignettes was used. Two questions examined the timing of surgery for an acute unstable SCFE in a child presenting at 6 and 48 h after start of symptoms. Two further questions explored the preferred method of fixation in mild and severe stable SCFE. The final question examined the management of the contralateral normal hip. Responses were entered into an Excel spreadsheet and the data was analysed using a χ-test. The response rate was 56% (110/196). Overall, 88% (97/110) responded that if a child presented with an acute unstable SCFE within 6 h, they would treat it within 24 h of presentation, compared with 41% (45/110) for one presenting 48 h after the onset of symptoms (P<0.0001). Overall, 53% (58/110) of surveyed BSCOS members would offer surgery for an unstable SCFE between 1 and 7 days after onset of symptoms. Single screw fixation in situ for mild stable SCFE was advocated by 96% (106/110) with 71% (78/110) using this method for the treatment of severe stable SCFE. Corrective osteotomy is used by 2% (2/110) and 25% (28/110) of respondents for mild and severe stable SCFE, respectively (P<0.0001). Surgeons preferring osteotomy are more likely to perform an intracapsular technique. Prophylactic fixation of the contralateral normal hip was performed by 27% (30/110) of respondents. There are significant differences in opinions between BSCOS members as to the optimal management of SCFE. This reflects the variable recommendations and quality in the current scientific literature. Further research is therefore required to determine best practice and enable consensus to be reached.

Common Paediatric Elbow Injuries.

BACKGROUND: Paediatric elbow injuries account for a large proportion of childrens' fractures. Knowledge of common injuries is essential to understanding their assessment and correct management. METHODS: A selective literature search was performed and personal surgical experiences are reported. RESULTS: We have described the assessment and management of the five most common paediatric elbow injuries: supracondylar humeral fractures; lateral condyle fractures; medial epicondyle fractures; radial head and neck fractures; radial head subluxation. CONCLUSION: Understanding of the ossification centres around the paediatric elbow is essential to correctly assessing and managing the common injuries that we have discussed in the review. Outcomes after these injuries are usually favourable with restoration of normal anatomy.

In vivo monitoring of bone architecture and remodeling after implant insertion: The different responses of cortical and trabecular bone.

The mechanical integrity of the bone-implant system is maintained by the process of bone remodeling. Specifically, the interplay between bone resorption and bone formation is of paramount importance to fully understand the net changes in bone structure occurring in the peri-implant bone, which are eventually responsible for the mechanical stability of the bone-implant system. Using time-lapsed in vivo micro-computed tomography combined with new composite material implants, we were able to characterize the spatio-temporal changes of bone architecture and bone remodeling following implantation in living mice. After insertion, implant stability was attained by a quick and substantial thickening of the cortical shell which counteracted the observed loss of trabecular bone, probably due to the disruption of the trabecular network. Within the trabecular compartment, the rate of bone formation close to the implant was transiently higher than far from the implant mainly due to an increased mineral apposition rate which indicated a higher osteoblastic activity. Conversely, in cortical bone, the higher rate of bone formation close to the implant compared to far away was mostly related to the recruitment of new osteoblasts as indicated by a prevailing mineralizing surface. The behavior of bone resorption also showed dissimilarities between trabecular and cortical bone. In the former, the rate of bone resorption was higher in the peri-implant region and remained elevated during the entire monitoring period. In the latter, bone resorption rate had a bigger value away from the implant and decreased with time. Our approach may help to tune the development of smart implants that can attain a better long-term stability by a local and targeted manipulation of the remodeling process within the cortical and the trabecular compartments and, particularly, in bone of poor health.

Report of an outbreak of Clostridium difficile infection caused by ribotype 053 in a neurosurgery unit.

BACKGROUND: We describe an outbreak of Clostridium difficile infection (CDI) with ribotype 053, a possible hypervirulent strain that causes outbreaks, in a neurosurgical unit. OUTBREAK INVESTIGATION: The outbreak was investigated by creating a timeline of all toxin positive patients with root cause analysis, supplemented with ribotyping results, hand hygiene and environmental audits. There were five cases of CDI, three caused by ribotype 053 indicating transmission. INFECTION PREVENTION MEASURES: These included a short period of ward closure to allow enhanced cleaning, including use of vaporised hydrogen peroxide, isolation of infected patients, reinforcement of hand hygiene, education of all staff on C. difficile, reduction of shared bay occupancy from six to four, and addressing staffing levels. DISCUSSION: The patients with ribotype 053 all had long inpatient stays and had required several courses of broad-spectrum antibiotics for aspiration pneumonia. They also required enteral feeding, which can cause diarrhoea, and during long inpatient stays they had multiple toxin negative faecal samples making clinical diagnosis of CDI difficult. Hence they were not isolated promptly, leading to transmission. This is the first reported outbreak of C. difficile ribotype 053 in the UK and highlights the unique aspects of an outbreak in neurosurgical patients.

Staged weaning versus immediate cessation of Pavlik harness treatment for developmental dysplasia of the hip.

Outcomes of nonoperative treatment for developmental dysplasia of the hip were compared between two centres. Eighty children in Centre A underwent staged weaning of the Pavlik harness once three consecutive weekly ultrasounds showed Graf Grade I hips, whereas in 48 children in Centre B, the harness was removed immediately. No statistically significant difference was found in the rate of reintervention (repeat harness treatment, closed or open reduction), avascular necrosis or acetabular index between the two methods, although there was a nonsignificant trend towards higher reintervention rate and lower avascular necrosis rate with immediate harness removal. Age at start of treatment significantly impacted upon initial harness success, reinterventions and avascular necrosis.

Developmental dysplasia of the hip.

The close interaction between community practitioners and young families provides an invaluable opportunity for prevention, education, support and even diagnosis in cases of developmental hip dysplasia. This article provides a brief overview of the condition, including clinical findings, screening protocols, 'hip safe' practices, and common treatments that may be encountered in the community.

Functional outcome following direct repair or intervertebral fusion for adolescent spondylolysis: a systematic review.

A systematic review of the literature was performed to establish whether direct repair of the pars defect or intervertebral fusion achieves better Oswestry Disability Index scores in adolescent spondylolysis or low-grade spondylolisthesis. Nine studies fulfilled the inclusion criteria, reporting a minimum total of 80 direct repairs and 108 fusions because of presumed replication of data between studies. Little statistically or clinically significant difference could be established between the two interventions. The only comparative study showed improved long-term outcome with fusion. Further well-designed prospective comparative studies are required to establish the optimum treatment for this condition.

Functional outcome following intramedullary nailing or plate and screw fixation of paediatric diaphyseal forearm fractures: a systematic review.

PURPOSE: Unstable paediatric diaphyseal both-bone forearm fractures requiring fixation have traditionally been treated with rigid internal fixation with plate and screws. Intramedullary stabilisation has grown in popularity over the last 25 years but may be associated with rotational deformity and subsequent loss of pronosupination. This systematic review aims to establish which treatment method provides better functional outcome. METHODS: A systematic review of the published literature was performed, searching Medline, Embase, Pubmed and the Cochrane Library for English-language studies comparing intramedullary nailing with plate and screws in patients less than 18 years old with both-bone diaphyseal forearm fractures. RESULTS: Seven studies met the inclusion criteria. They were all retrospective comparative studies (level III or IV). One was age- and sex-matched. Three looked specifically at older children. No study reported a significant difference in functional outcome with either treatment. CONCLUSIONS: The currently available literature shows no difference in functional outcome between intramedullary nailing and plate and screw fixation, even in older children with less remodelling potential. Intramedullary nailing may therefore be the treatment of choice for simple fracture patterns due to shorter operative time, better cosmesis and ease of removal. Plating may still have a role in more complex injuries.

Clinical results and motion analysis following arthroscopic anterior stabilization of the shoulder using bioknotless anchors.

PURPOSE: Traumatic anterior dislocation of the shoulder is a common occurrence increasingly being treated arthroscopically. This study aims to determine the outcome of arthroscopic anterior stabilization using bioknotless anchors and analyze the motion in a subset of these patients. MATERIALS AND METHODS: The outcome of 20 patients who underwent arthroscopic anterior stabilization using the bioknotless system was studied (average follow-up 26 months). Four of these patients underwent motion analysis of their shoulder pre- and post-operatively. RESULTS: 15% were dissatisfied following surgery and the recurrence of instability was also 15%. Those who were dissatisfied or suffered recurrent symptoms had statistically significant lower constant scores at the final follow up. Pre-operative motion analysis showed a disordered rhythm of shoulder rotation which was corrected following surgery with minimal loss of range of motion. CONCLUSIONS: Our success rate was comparable to similar arthroscopic techniques and results published in the literature. Patient satisfaction depended more on return to usual activities than recurrence of symptoms. There was very little reduction in range of movement following surgery and the rhythm of shoulder motion, particularly external rotation in abduction was improved. LEVEL OF EVIDENCE: Four retrospective series.