Course Review: Birmingham and Coventry Hand and Forearm Cadaveric Dissection Course.

Hand surgery constitutes an important part of plastic surgery and orthopedic training curricula. Although the value of simulation-based training is being increasingly recognized, attendance at such courses may be limited owing to cost, availability, and non-learner-centered schedules.The Birmingham and Coventry hand and forearm cadaveric dissection course is a 1-day course with a loose agenda that offers an inexpensive and learner-centered approach toward surgical access, dissection, and soft tissue coverage in the upper limb. This course is held in Coventry, United Kingdom, at a cost of £165 ($220 US dollars). In this review, we provide an evaluation of the course.

Course Review: AOTrauma-Hand Fixation for Surgeons.

Hand trauma forms an important part of plastic surgery and orthopedic training curricula. Although numerous practical hand trauma courses exist, there is always difficulty in achieving the right balance between educational content and practical workshops. AOTrauma-Hand Fixation for Surgeons is a 2.5-day course for plastic surgery and orthopedic trainees that aims to instill the principles of hand fracture fixation and soft tissue coverage through self-directed learning, lectures, small group discussion, and dry bone practical workshops. This course with international faculty and delegates is held in Leeds, United Kingdom, at a cost of 860 Swiss francs (880 US dollars). In this review, we provide an evaluation of the course.

Course Review: Plastic Surgery for Surgical Trainees.

Early in their careers, trainees contemplating a career in plastic surgery may have limited specialty experience. Courses can provide valuable insight into specialty training and help develop necessary basic skills. However, this is not without a significant financial burden.Plastic Surgery for Surgical Trainees is a 1-day course that only costs £50 GBP (US $65). Accredited by the Royal College of Surgeons, this course held in Bristol, United Kingdom, offers an inexpensive practical introduction toward plastic surgical techniques for trainees interested in plastic surgery and those in other surgical specialties. In this review, we provide an evaluation of the course.

Phenotype, cancer risk, and surveillance in Beckwith-Wiedemann syndrome depending on molecular genetic subgroups.

Patients with Beckwith-Wiedemann syndrome (BWS) have an increased risk to develop cancer in childhood, especially Wilms tumor and hepatoblastoma. The risk varies depending on the cause of BWS. We obtained clinical and molecular data in our cohort of children with BWS, including tumor occurrences, and correlated phenotype and genotype. We obtained similar data from larger cohorts reported in the literature. Phenotype, genotype and tumor occurrence were available in 229 of our own patients. Minor differences in phenotype existed depending on genotype/epigenotype, similar to earlier studies. By adding patients from the literature, we obtained data on genotype and tumor occurrence of in total 1,971 BWS patients. Tumor risks were highest in the IC1 (H19/IGF2:IG-DMR) hypermethylation subgroup (28%) and pUPD subgroup (16%) and were lower in the KCNQ1OT1:TSS-DMR (IC2) subgroup (2.6%), CDKN1C (6.9%) subgroup, and the group in whom no molecular defect was detectable (6.7%). Wilms tumors (median age 24 months) were frequent in the IC1 (24%) and pUPD (7.9%) subgroups. Hepatoblastoma occurred mostly in the pUPD (3.5%) and IC2 (0.7%) subgroups, never in the IC1 and CDKN1C subgroups, and always before 30 months of age. In the CDKN1C subgroup 2.8% of patients developed neuroblastoma. We conclude tumor risks in BWS differ markedly depending on molecular background. We propose a differentiated surveillance protocol, based on tumor risks in the various molecular subgroups causing BWS. © 2016 Wiley Periodicals, Inc.

Does tranexamic acid effectively minimise blood loss in cleft palate repair? A rigorous assessment through comprehensive systematic review and meta-analysis.

Cleft palate repair is a common reconstructive procedure that can involve significant blood loss. Tranexamic acid (TXA) has been proposed to minimise blood loss during various surgical procedures, but its effectiveness in cleft palate repair remains unclear. This systematic review and meta-analysis aimed to assess the effectiveness of TXA to reduce postoperative blood loss. Adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we conducted a comprehensive search across multiple databases, including PubMed, Cochrane, and Web of Science, to identify relevant studies published up to September 2023. Only randomised controlled trials (RCTs) were included. Primary outcomes measured were total blood loss, transfusion rates, and postoperative complications. We identified four relevant RCTs, which included 275 cleft palate patients with a mean (range) age of 28.7 (6-65) months. The pooled analysis found no significant difference in duration of surgery (MD -18.40 minutes, p = 0.09), preoperative haemoglobin (MD 0.46 g/dl, p = 0.27), or postoperative haemoglobin (MD 0.07 g/dl, p = 0.86) between TXA and control groups. Intraoperative blood loss was lower with TXA, but with TXA, the difference was not statistically significant (MD -16.63 ml, p = 0.15). TXA significantly improved surgical field visibility (p = 0.004). No adverse events occurred with its use. While no significant differences were found in surgical outcomes with TXA, surgical field visibility significantly improved, and TXA showed a promising safety profile. Larger and higher-quality RCTs are still needed to validate these preliminary findings before TXA can be considered as a standard treatment.

Comparison of Patient Satisfaction on a Day-case Mastectomy Pathway for Breast Cancer Versus a Traditional Inpatient Delivery Model, Using a Validated Questionnaire.

BACKGROUND: In 2011, a guidance was issued by the National Health Service (NHS) Improvement a model on how mastectomy could be offered in the day-case setting. The goal of this guidance was to reduce inpatient bed days and cost to the NHS, and demonstrate that it can be performed within an acceptable safety profile. The aim of this study was to assess whether patients find the day-case pathway for mastectomy an acceptable management model. We compared complication rates between the day-case and inpatient delivery model. PATIENTS AND METHODS: This study was a retrospective analysis of patients' experience undergoing day-case (n=26) and inpatient mastectomy (n=60). The primary outcome measure was based on a telephone interview using a validated, standardised questionnaire. RESULTS: No statistically significant difference in the satisfaction levels between the two groups (raw scores 6.76 day-case vs. 6.15 inpatient, p=0.37) was demonstrated. We found no statistically significant difference between the two groups when specifically analysing whether patients found the first night harder as a day-case or inpatient (3.192 vs. 2.80, p=0.59, range 0-10). Our overall complications were 11.4% (day-case) and 18.3% (inpatients). Rates were comparable between the two groups and equivalent to published rates in the literature. CONCLUSION: There was no statistically significant difference in satisfaction scores between patients who had a mastectomy as an inpatient versus those who had their operation as a day-case procedure. In addition, there were no significant differences in the complication rates between the two groups. We conclude then that it is feasible and safe to offer day-case mastectomy, with no loss in patient satisfaction.

The use of polymeric clips in securing the appendiceal stump during laparoscopic appendicectomy: a systematic review.

PURPOSE: A key step during laparoscopic appendicectomy is securing the appendiceal stump. This has traditionally been achieved using vicryl endoloops, but increasing evidence suggests that the use of polymeric clips (Hem-o-lok) may be a safe and viable method. Current evidence for its clinical use in laparoscopic appendicectomy is unknown. We performed a systematic review of the literature examining the clinical outcomes of laparoscopic appendicectomy using polymeric clips compared to other methods of stump closure. METHODS: A systematic literature review based on PRISMA guidelines was performed using MEDLINE, PubMed, EMBASE and Cochrane library databases between 2000 and 2017. All studies analysing appendiceal stump closure during laparoscopic appendicectomy using polymeric clips compared to other methods of stump closure were included. The methodological quality of the included studies was assessed using the Cochrane Handbook for Systematic Reviews. The review was registered with the PROSPERO register of systematic reviews. RESULTS: Ten studies were included, involving 702 patients, 7 of which were prospective studies and 1 a randomised control trial. Polymeric clips were found to be the cheapest method (€20.47 average per patient) and also had the lowest rate of complications (2.7%) compared to other commonly used closure methods. Meanwhile, operative time and duration of in-patient stay were similar between groups. CONCLUSIONS: Current evidence suggests that polymeric clips are an effective and cost-efficient method for stump closure in laparoscopic appendicectomy for acute appendicitis. Further high-quality evidence is required before polymeric clips can be recommended as the gold standard for appendiceal stump closure.

Managing aesthetic referrals in NHS Scotland: Outcomes from 1122 patients in the East of Scotland.

The Adult Exceptional Aesthetic Referral Protocol (AEARP) encompasses a series of aesthetic procedures which, as they do not treat an underlying disease process, are not routinely available within the National Health Service. Provision of these services can only be provided on an exceptional basis. In this prospective study, we evaluated the referral process and outcomes of 1122 patients referred under the AEARP over a 3.5-year period. Referrals were screened by a vetting panel comprising of a plastic surgeon, clinical nurse specialist, and clinical psychologist. Following initial vetting, supported patients underwent psychological assessment. Patients supported by psychology were assessed in clinic, and if deemed clinically suitable, were offered surgery. Overall, 20% (225/1122) of referrals were supported for surgery. Following primary vetting, 57% (640/1,122) of referrals were supported, 40% (197/492) of referrals to clinical psychology were supported, and 65% (225/345) of the remaining cases referred for consultation were supported for surgery. Unsupported referrals included those not fulfilling the referral guidelines or those with contraindications. The AEARP is simple and effective to implement, and has been instrumental in streamlining the referral-to-outcome process in a centralised, transparent, and fair manner. It reduces a potential high number of clinic appointments where patients do not meet the aesthetic criteria and/or fail to attend - thereby helping to streamline other surgical pathways by improving clinic efficiency. Moreover, it aids referring clinicians and patient education around aesthetic issues including a holistic approach. Wide adoption of such standards may reduce waiting times, facilitate cost savings, and ultimately enhance patient outcomes.

Vitamin D deficiency in sickle cell disease patients in the Eastern Province of Saudi Arabia.

BACKGROUND: Vitamin D deficiency (VDD) is a major global health problem. In sickle cell disease (SCD), VDD is highly prevalent, reaching up to 96% of populations. VDD may contribute to many of the complications of SCD. OBJECTIVE: Estimate the 25-hydroxyvitamin D [25(OH)D] level and the frequency of VDD and insufficiency among among SCD patients by age group and disease status. DESIGN: Analytical cross-sectional. SETTING: Ministry of Health (MOH) secondary care hospital. PATIENTS AND METHODS: Non-probability purposive sampling was used to select SCD patients, aged 12 years and older, of both sexes, who had visited the hospital during a period of 5 years (2010-2014). Blood samples were processed by electrochemiluminescence technology. MAIN OUTCOME MEASURE(S): 25(OH)D levels by demographic data, and disease activity. SAMPLE SIZE: 640 patients. RESULTS: Of those, 82% (n=523) had suboptimal 25(OH)D (0-<30 ng/ mL), and 67% were deficient (0-<20 ng/mL). Patients with any SCD crisis (20.7%, 144/694) had lower 25(OH)D (median, IQR: 10.1 ng/mL [8.6] ng/mL) compared to patients without crisis (71.0%, 493/694) (15.7 ng/ mL [18.2] ng/mL) (P<.001). Deficiency was more common in the young.er age groups and in sickle cell anemia patients with crisis. CONCLUSIONS: VDD is highly prevalent in this population. Established vitamin D screening is a necessity, so that affected patients can be treated. LIMITATIONS: Presence of residual confounders such as nutritional status, physical activity, lack of sun exposure, medications that alleviate SCD crises (such as hydroxyurea), and comorbid illnesses. The relationship between sickle cell disease genotype and vitamin D level was not analyzed. CONFLICT OF INTEREST: None.

Expert consensus document: Clinical and molecular diagnosis, screening and management of Beckwith-Wiedemann syndrome: an international consensus statement.

Beckwith-Wiedemann syndrome (BWS), a human genomic imprinting disorder, is characterized by phenotypic variability that might include overgrowth, macroglossia, abdominal wall defects, neonatal hypoglycaemia, lateralized overgrowth and predisposition to embryonal tumours. Delineation of the molecular defects within the imprinted 11p15.5 region can predict familial recurrence risks and the risk (and type) of embryonal tumour. Despite recent advances in knowledge, there is marked heterogeneity in clinical diagnostic criteria and care. As detailed in this Consensus Statement, an international consensus group agreed upon 72 recommendations for the clinical and molecular diagnosis and management of BWS, including comprehensive protocols for the molecular investigation, care and treatment of patients from the prenatal period to adulthood. The consensus recommendations apply to patients with Beckwith-Wiedemann spectrum (BWSp), covering classical BWS without a molecular diagnosis and BWS-related phenotypes with an 11p15.5 molecular anomaly. Although the consensus group recommends a tumour surveillance programme targeted by molecular subgroups, surveillance might differ according to the local health-care system (for example, in the United States), and the results of targeted and universal surveillance should be evaluated prospectively. International collaboration, including a prospective audit of the results of implementing these consensus recommendations, is required to expand the evidence base for the design of optimum care pathways.

Attenuated familial adenomatous polyposis manifests as autosomal dominant late-onset colorectal cancer.

Colorectal cancer (CRC) risk is well defined for families of patients with classical familial adenomatous polyposis (FAP). However, the risk for those with an attenuated form of FAP is less well characterised. In this study, we estimated CRC risks for carriers of a novel germline mutation in the APC gene that causes attenuated FAP (AFAP). We performed genetic testing on 53 individuals from seven AFAP families harbouring an identical APC:c.288T>A mutation. Using a modified segregation analysis, we estimated relative and absolute CRC risks for mutation carriers. Twenty-three individuals harboured the disease causing mutation. CRC occurred in 28 individuals (mean 61.7 years, range 32-80 years). The estimated CRC relative risks for mutation carriers aged 60-69 and ≥70 years were 19 (95% CI: 1.77-204.08) and 45 (95% CI: 11.32-180.10), respectively, while the absolute CRC lifetime risk for men was 94% (95% CI: 67.5-99.9%), and for women, 84% (95% CI: 50.9-99.0%). This study shows that AFAP can manifest as autosomal dominant late-onset CRC. These findings highlight a subgroup of inherited CRCs that require new criteria for identification and surveillance.

Methylation analysis and diagnostics of Beckwith-Wiedemann syndrome in 1,000 subjects.

BACKGROUND: Beckwith-Wiedemann syndrome (BWS), a congenital overgrowth disorder with variable expressivity and a predisposition to tumorigenesis, results from disordered expression and/or function of imprinted genes at chromosome 11p15.5. There are no generally agreed clinical diagnostic criteria, with molecular studies commonly performed to confirm diagnosis. In particular, methylation status analysis at two 11p15.5 imprinting control centres (IC1 and IC2) detects up to 80% of BWS cases (though low-level mosaicism may not be detected). In order to evaluate the relationship between the clinical presentation of suspected BWS and IC1/2 methylation abnormalities we reviewed the results of >1,000 referrals for molecular diagnostic testing. RESULTS: Out of 1,091 referrals, 507 (46.5%) had a positive diagnostic test for BWS. The frequency of tumours was 3.4% in those with a molecular diagnosis of BWS. Previously reported genotype-phenotype associations with paternal uniparental disomy, IC1, and IC2 epimutation groups were confirmed and potential novel associations detected. Predictive values of previously described clinical diagnostic criteria were compared and, although there were differences in their sensitivity and specificity, receiver operating characteristic (ROC) analysis demonstrated that these were not optimal in predicting 11p15.5 methylation abnormalities. Using logistic regression, we identified clinical features with the best predictive value for a positive methylation abnormality. Furthermore, we developed a weighted scoring system (sensitivity 75.9%, and specificity 81.8%) to prioritise patients presenting with the most common features of BWS, and ROC analysis demonstrated superior performance (area under the curve 0.85, 95% CI 0.83 to 0.87) compared to previous criteria. CONCLUSIONS: We suggest that this novel tool will facilitate selection of patients with suspected BWS for routine diagnostic testing and so improve the diagnosis of the disorder.

Brain MRI features of merosin-negative congenital muscular dystrophy.

The purpose of this article is to report brain MRI findings of merosin-negative congenital muscular dystrophy (CMD) in two cases and to discuss its differentiation from other CMD subtypes. The cases were investigated by 1.5 Tesla MRI equipment using T1-weighted, T2-weighted, fluid-attenuated inversion recovery (FLAIR), 3D fast spoiled gradient recalled (SPGR) and post-contrast gadolinium enhanced sequences and the diagnosis was confirmed by muscle biopsy with immunohistochemical staining for merosin. Magnetic resonance imaging showed bilateral subcortical U-fibre white matter changes with periventricular sparing in the first case. Though this imaging finding is non-specific and could be seen in other white matter diseases like Canavan disease, the absence of other distinguishing MRI and clinical features of those conditions allowed a proper diagnosis. The second case showed diffuse subcortical and deep white matter involvement and cerebellar cysts. There was no brainstem involvement, polymicrogyria or cobblestone lissencephaly in both of our cases which allowed differentiation of merosin-negative CMD from other CMDs. Muscle biopsy in both these patients showed dystrophic changes with absent staining for merosin confirming the diagnosis. Merosin-negative CMD should be considered in the imaging differential diagnosis of white matter diseases. In a patient presenting with features of CMD, MRI can be of help in further differentiation of the various CMD subtypes.

Diabetic foot: clinical presentation and management in Al-Gamhuoria Teaching Hospital

The aim of this study is to determine the mode of presentation, medical and surgical treatment. and prevention of diabetic foot. This is a prospective descriptive study performed in the period from June 2004-December 2005. Eighty-three patients with diabetic foot lesion were presented to Al-Gamhorea Teaching Hospital. Data of these patient were collected for sex, age, duration of diabetes, blood sugar control, mode of presentation, presence of peripheral neuropathy, peripheral vascular disease, concurrent medical illness [ischemic heart disease and chronic renal failure], microbial flora, treatment by antibiotics, surgical treatment and duration of healing. Majority of patients were male above 50 years 68.7%, mean age 58.3 +/- 12 and female 3 1.3%. Most of them were type 2 on oral hypoglycemic drugs [89.16%]. Blood sugar was uncontrolled in 77.1% of them. History of trauma preceding diabetic foot infection was present in 74.7%. Foot ulcer was the most common presentation in 47% of patients. There was a significant association between the peripheral neuropathy and the following risk factors: duration of diabetes more than ten years 100% [p = 0.0000], poor glycemic control 95.3% p.000] and trauma 83.9% [p = 0.0000]. There was also a significant relationship between the major limb amputation and some predictive variables: previous foot ulcer 35.3% [p=0.0000]. poor glycemic control 17.2% [p = 0.04], peripheral neuropathy 17.7% [p = 0.03], peripheral vascular disease 75% [p = 0.000], and heart disease 33.3% [p = 0.01]. Debridement done for 9.4% and toe/s amputation for 30.2%, however 11 patients [13,3%] had major amputation. Staphylococcus aureus was the most common organism isolated in 34.9%. Duration of healing was 9-16 weeks for most of the patients 63.4%. Diabetic foot infection is a common health problem in Al-Gamhorea Teaching Hospital, particularly among men. Poor glycemic control, as well as peripheral neuropathy and peripheral vascular disease are the most precipitating factors. which can be prevented. Control of blood sugar, good debridement, and proper dressing can lead to early and complete healing in most of diabetic foot lesions