Association of Perioperative Antibiotics with the Prevention of Postoperative Fistula after Cleft Palate Repair.

Background: There is debate amongst surgeons regarding the use of antibiotics to prevent fistulae after palatoplasty. Prescribing should be evidence based, as antibiotic stewardship is integral to reducing antibiotic resistance. Our aim was to determine whether differing perioperative regimens affect the prevalence of postoperative fistulae. Methods: The sample comprised participants from the Cleft Collective who had undergone palatoplasty. Participants were recruited across all 16 UK cleft centers between 2013 and 2021. The exposure was perioperative antibiotic regimen prescribed at the time of palatoplasty. The primary outcome was the presence of palatal fistula. Results: Fistula data were available for 167 participants when exploring antibiotic regimen and for 159 when exploring antibiotic agent. There was no evidence to suggest a difference in fistula rate between those receiving antibiotics on induction only versus as an inpatient or up to 7 days postoperatively (χ2 = 4.57; P = 0.10). There was no evidence to suggest a difference in fistula rate between those who received co-amoxiclav and those who had an alternative antibiotic (χ2 = 0.16; P = 0.69). Postoperative fistulae increased with the extent of the cleft (χ2 = 20.39; P < 0.001). When adjusting for cleft type, no evidence of an association between antibiotic regimen and fistulae was found (inpatient antibiotics: OR 1.36; 95% confidence interval, 0.53-3.51; antibiotics up to 7 days postoperatively: OR 0.68; 95% confidence interval, 0.26-1.80). Conclusions: The choice of antibiotic and dosing regimen does not influence the formation of postoperative fistulae. These results should be supported by interventional trials.

Syndromes associated with Robin sequence: a national prospective cohort study.

OBJECTIVES: To determine the prevalence of syndromic Robin sequence (RS) in the UK and if this group of patients had an increased need for airway and feeding management compared with a non-syndromic RS cohort. DESIGN: A prospective national multicentre study of cases submitted to the Cleft Collective cohort studies. SETTING: Specialist cleft services in the UK. PATIENTS: 259 participants who fulfilled the diagnosis of RS. This group was compared with 548 participants with cleft palate only (CPO). MAIN OUTCOME MEASURES: The primary outcome measure was the presence of a syndrome in patients with RS and CPO. Secondary outcome measures included the use of airway and feeding adjuncts. RESULTS: An associated syndrome was seen in 28% of patients with RS and 14% of patients with CPO. The most common syndrome for the RS group was Stickler syndrome (27%). Syndromic status was significantly higher among patients with RS compared with those with CPO (OR 2.36, 95% CI 1.65 to 3.39; p<0.001). Patients with syndromic RS have an increased reliance on airway adjuncts compared with the patients without syndromic RS (OR 2.02, 95% CI 1.13 to 3.64; p=0.018). There was no evidence of a difference in the use of feeding adjuncts between syndromic and non-syndromic RS groups (OR 2.43, 95% CI 0.78 to 7.58; p=0.126). CONCLUSION: The presence of a syndrome has implications for management of patients with RS. Early identification of a syndrome may help prevent the consequences of a missed syndromic diagnosis. Routine ophthalmological and genetic screening for Stickler syndrome should be mandatory for all patients with RS.

Cardiometabolic risk factors and hypovitaminosis D in adolescents with overweight from a sunny region in northeast Brazil: a cross-sectional study.

INTRODUCTION: Background: obesity can influence vitamin D levels, which in turn might be associated with cardiometabolic risk factors. Objectives: this study assessed the association between 25-hydroxyvitamin D [25(OH)D] levels and cardiometabolic risk factors in adolescents with overweight living in a region of northeastern Brazil. Material and methods: a cross-sectional study was carried out by non-probabilistic sampling in adolescents diagnosed with overweight or obesity. The subjects were divided according to their 25(OH)D status into two groups: sufficient vitamin D and hypovitaminosis D. Biodemographic, lifestyle, cardiometabolic, and biochemical factors were evaluated. A logistic regression model was applied to determine the predictors of hypovitaminosis D. Results: we found a high frequency of hypovitaminosis D (45.6 %) in adolescents. Weekly sun exposure was negatively associated with hypovitaminosis D (OR = 0.96; 95 % CI: 0.92-0.99), while significant positive associations were observed between hypovitaminosis D and blood pressure above the 95th percentile (OR = 4.00; 95 % CI: 1.19-13.37), body weight (OR = 1.04; 95 % CI: 1.01-1.07), and fasting insulin (OR = 1.13; 95 % CI: 1.05-1.22). Conclusion: hypovitaminosis D showed a high prevalence in adolescents with overweight living in a sunny region of northeastern Brazil, and cardiometabolic risk factors such as systemic arterial hypertension, high body weight, and hyperinsulinemia are predictors of hypovitaminosis D.

INTRODUCCIÓN: Introducción: la obesidad puede influir en los niveles de vitamina D, lo que a su vez podría estar asociado con factores de riesgo cardiometabólico. Objetivos: este estudio evaluó la asociación entre los niveles de 25-hidroxivitamina D [25(OH)D] y los factores de riesgo cardiometabólico en adolescentes con sobrepeso que viven en una región del noreste de Brasil. Material y métodos: se realizó un estudio transversal mediante muestreo no probabilístico con adolescentes diagnosticados de sobrepeso u obesidad. Los sujetos se dividieron según su estado de 25(OH)D en dos grupos: suficiente vitamina D e hipovitaminosis D. Se evaluaron factores biodemográficos, de estilo de vida, cardiometabólicos y bioquímicos. Se aplicó un modelo de regresión logística para determinar los predictores de la hipovitaminosis D. Resultados: encontramos una alta frecuencia de hipovitaminosis D (45,6 %) en los adolescentes. La exposición semanal al sol se asoció negativamente a la hipovitaminosis D (OR = 0,96; IC 95 %: 0,92-0,99), mientras que se observaron asociaciones positivas significativas entre hipovitaminosis D y presión arterial por encima del percentil 95 (OR = 4,00; IC 95 %: 1,19-13,37), peso corporal (OR = 1,04; IC del 95 %: 1,01-1,07) e insulina en ayunas (OR = 1,13; IC del 95 %: 1,05-1,22). Conclusión: la hipovitaminosis D mostró una alta prevalencia entre los adolescentes con sobrepeso que viven en una región soleada del noreste de Brasil, y los factores de riesgo cardiometabólico, como hipertensión arterial sistémica, peso corporal elevado e hiperinsulinemia, son predictores de hipovitaminosis D.

CYSTOID MACULAR EDEMA AFTER FOUR-POINT SCLERAL FIXATION OF INTRAOCULAR LENS.

PURPOSE: To assess the incidence of cystoid macular edema (CME) associated with 4-point Gore-Tex suture intraocular lens (IOL) scleral fixation, before and after institution of routine intravitreal triamcinolone acetonide prophylaxis and long-term topical nonsteroidal anti-inflammatory drug usage. METHODS: Consecutive patients were included after IOL implantation with concurrent pars plana vitrectomy for spontaneous IOL dislocation due to pseudoexfoliation syndrome. We compared short-term prophylactic nonsteroidal anti-inflammatory drugs only (Group A) to prophylactic intravitreal triamcinolone acetonide and long-term nonsteroidal anti-inflammatory drugs (Group B). RESULTS: Twenty-six eyes of 26 patients with pseudoexfoliation syndrome and spontaneous IOL dislocation were studied. Mean logMAR visual acuity improved from 1.27 ± 0.80 (20/375 Snellen equivalent) preoperatively to 0.46 ± 0.39 (Snellen 20/43) postoperatively (P < 0.001). Visual outcomes were similar for Groups A and B. In Group A, 10/16 eyes had CME, 4/16 had chronic CME longer than 6 months, and 1 longer than 12 months. In Group B, 1/10 had CME (which was both chronic and refractory). CONCLUSION: In eyes with pseudoexfoliation syndrome and spontaneous IOL dislocation, 4-point Gore-Tex suture IOL ab externo fixation yielded good visual outcomes, although CME was observed more than reported elsewhere. Prophylactic intravitreal triamcinolone acetonide and long-term nonsteroidal anti-inflammatory drugs seem to reduce the risk of postoperative CME.

Sagittal Synostosis and Its Association With Cognitive, Behavioral, and Psychological Functioning: A Meta-analysis.

Importance: Findings on the cognitive, behavioral, and psychological functioning of individuals with sagittal synostosis (SS) are highly disparate, limiting their clinical utility. Objective: To identify and review research on individuals with SS and to determine whether, and to what extent, they experience cognitive, behavioral, and psychological difficulties compared with their healthy peers or normative data for each measure. Data Sources: PubMed, Scopus, Embase, and PsycINFO were searched through January 2021 with no date restrictions. Scopus citation searches and manual checks of the reference lists of included studies were conducted. Study Selection: Studies included participants of any age who had received a diagnosis of single-suture (isolated or nonsyndromic) SS or scaphocephaly and who had been assessed on cognitive, behavioral, and psychological outcomes. Data Extraction and Synthesis: Data were independently extracted by 2 reviewers. Case-control outcomes (individuals with SS vs healthy peers or normative data) were compared using random-effects models with 3 effect sizes calculated: weighted Hedges g (gw), odds ratios (ORs), and mean prevalence rates. This study follows the Meta-analysis of Observational Studies in Epidemiology (MOOSE) reporting guidelines. Main Outcomes and Measures: Findings were categorized by surgical status (conservatively managed, presurgery, postsurgery, or combined); domain (eg, general cognition); type of cognitive, behavioral, or psychological measure (objective or subjective); and source of comparison data (peers or normative data). Results: Data from 32 studies, involving a pooled sample of 1422 children and adults with SS (mean [SD] age at assessment, 5.7 [6.6] years; median [interquartile range] age, 3.3 [0.5-10.3] years), were analyzed. Data on sex were available for 824 participants, and 642 (78%) were male. Individual study results varied substantially. Objective tests identified significant moderate group differences on 3 of 16 examined domains: presurgical motor functioning (3 studies; gw = -0.42; 95% CI, -0.67 to -0.18; P < .001), postsurgical short-term memory (2 studies; gw = -0.45; 95% CI, -0.72 to -0.17; P < .001), and postsurgical visuospatial ability (6 studies; gw = 0.31; 95% CI, 0.18 to 0.44; P < .001). Prevalence estimates and ORs varied widely, with 15 studies showing prevalence estimates ranging from 3% to 37%, and 3 studies showing ORs ranging from 0.31 (95% CI, 0.01 to 6.12) for processing speed in the conservatively managed sample to 4.55 (95% CI, 0.21 to 98.63) for postsurgical visuospatial abilities. Conclusions and Relevance: In this meta-analysis, findings for the functioning of participants with SS were highly disparate and often of low quality, with small samples sizes and control groups rarely recruited. Nonetheless, the findings suggest that some individuals with SS experience negative outcomes, necessitating routine assessment.

Emerging Clinical Features of COVID-19 Related Pancreatitis: Case Reports and Review of the Literature.

INTRODUCTION: SARS-CoV-2 is fundamentally a respiratory pathogen with a wide spectrum of symptoms. The COVID-19 related pancreatitis is less considered than other clinical features. The purpose is to describe two cases of pancreatitis associated with COVID-19. METHODOLOGY: Patients' demographics, clinical features, laboratory, and instrumental findings were collected. RESULTS: Two patients admitted to the hospital were diagnosed with COVID-19 and severe acute pancreatitis, according to the Atlanta criteria. Other causes of acute pancreatitis were excluded. Treatment included broad-spectrum antibiotics, proton pump inhibitors, and low molecular weight heparin. Steroids, oxygen, antifungal treatment, and pain killers were administered when appropriate. Both patients were asymptomatic, with normal vital parameters and blood exams, and were discharged in a good condition. CONCLUSION: It is recommendable to include lipase and amylase on laboratory routine tests in order to evaluate the need for the abdominal CT-scan and specific therapy before hospital admission of the patients with COVID-19 related life-threatening acute pancreatitis.

The Effect of Therapeutic Meibomian Glands Expression on Evaporative Dry Eye: A Prospective Randomized Controlled Trial.

PURPOSE: To determine the clinical benefits of Meibomian gland expression therapy for the treatment of dry-eye disease caused by Meibomian gland dysfunction (MGD). METHODS: In a prospective randomized controlled double-masked trial, 87 eyes of 44 patients with MGD and dry-eye symptoms were enrolled. Patients were randomly assigned into two groups; a study group that received therapeutic Meibomian gland expression once every month, and a control group that received sham treatment. All patients received treatment with artificial tears. RESULTS: One week after the first treatment, the Ocular Surface Disease Index (OSDI) score improved significantly in the study group (mean change -18.5 ± 21.2, p = .01) but not in the control group (-3.8 ± 15.8, p = .16); after 1 month, both groups improved significantly (-20.5 ± 19 p = .001 in the study group and -6.5 ± 11, p = .016 in the control group). The improvement continued at 2 months in the study group (-28.4 ± 26.1, P < .0001) and in the control group (-9.6 ± 9.9, p = .007). The blepharitis questionnaire score improved in the study group compared to controls after 1 week (-9.95 ± 12.52 versus -1.77 ± 9.1, p = .03) 1 month (-11.5 ± 10.9 versus -1.1 ± 9.4, p = .02) and 2 months (-16.5 ± 8.0 versus -8.8 ± 11.7, p = .02). Burning sensation was significantly reduced only in the study group. Mean change after 2-month treatment was -2.00 ± 1.2583 (p < .0001) vs -0.67 ± 1.44 (p = .08). The trend was similar in Eyelid scales. Conjunctival hyperemia improved only in the study group 1 week after the treatment (-0.12 ± 0.32 p = .03). CONCLUSION: Therapeutic Meibomian gland expression improves dry-eye symptoms in subjects with MGD, compared to conventional treatment with artificial tears.

A multilayered valve leaflet promotes cell-laden collagen type I production and aortic valve hemodynamics.

Patients with aortic heart valve disease are limited to valve replacements that lack the ability to grow and remodel. This presents a major challenge for pediatric patients who require a valve capable of somatic growth and at a smaller size. A patient-specific heart valve capable of growth and remodeling while maintaining proper valve function would address this major issue. Here, we recreate the native valve leaflet structure composed of poly-ε-caprolactone (PCL) and cell-laden gelatin-methacrylate/poly (ethylene glycol) diacrylate (GelMA/PEGDA) hydrogels using 3D printing and molding, and then evaluate the ability of the multilayered scaffold to produce collagen matrix under physiological shear stress conditions. We also characterized the valve hemodynamics under aortic physiological flow conditions. The valve's fibrosa layer was replicated by 3D printing PCL in a circumferential direction similar to collagen alignment in the native leaflet, and GelMA/PEGDA sustained and promoted cell viability in the spongiosa/ventricularis layers. We found that collagen type I production can be increased in the multilayered scaffold when it is exposed to pulsatile shear stress conditions over static conditions. When the PCL component was mounted onto a valve ring and tested under physiological aortic valve conditions, the hemodynamics were comparable to commercially available valves. Our results demonstrate that a structurally representative valve leaflet can be generated using 3D printing and that the PCL layer of the leaflet can sustain proper valve function under physiological aortic valve conditions.

Surgical Implications of the Anteriorly Displaced Segments of the Anterior Cerebral Artery in the Management of Frontoethmoidal Meningoencephalocele.

BACKGROUND: Preservation of the anterior cerebral arteries (ACAs) is important in the surgical management of frontoethmoidal meningoencephalocele (FEME). This would avoid complications related to the loss of blood supply to the part of the brain supplied by the ACA. Previous reports have identified hydrocephalus, microcephaly, cerebral dysplasias amounting to a 15% to 20% prevalence of brain anomalies in patients with FEME. What has not been previously reported are cerebral vasculature changes in the frontal region in FEME and how these may impact on the surgical correction and clinical outcome. METHODS: Two patients of FEME that demonstrate cautionary radiologic findings in relation to the ACAs and anterior fossa anatomy are discussed. RESULTS: The ACA in a 4-year-old boy with FEME was displaced anteriorly with the long A1 segments that extend into the defect. In the 2nd patient, a 4-year-old girl, we report on the complications related to the injury of ACAs from a previously partially resected FEME. CONCLUSION: The importance of the anterior cerebral vasculature around the FEME during surgery is crucial to prevent complications resulting from damage to a looping A1 segment of the ACA.

Atypical Skin Manifestations in FGFR2-Related Craniosynostosis Syndromes Broaden the Phenotypic Spectrum.

Crouzon syndrome (CS) and Beare-Stevenson syndrome (BSS) are craniosynostosis syndromes caused by mutations in the fibroblast growth factor 2 (FGFR2) gene. CS is more common (1 in 60,000 live births) than BSS, where fewer than 20 individuals have been reported. The cardinal features of BSS are craniosynostosis, cutis gyrata, acanthosis nigricans, skin furrows, skin tags, anogenital anomalies, and a prominent umbilical stump. Previously described individuals with BSS have typically had mutations in exon 11 of FGFR2. Here, we present 2 patients with CS who have significant skin manifestations and some phenotypic overlap with BSS. De novo mutations in exon 8 of FGFR2 were identified in both; one is a mutation (c.799T>C; p.Ser267Pro) previously identified in individuals with CS and the other a novel in-frame deletion (c.820_824delinsTT; p.Val274_Glu275delinsLeu). No mutations in exon 11 of FGFR2, where previously reported BSS mutations have been located, were identified. This case expands the phenotypic spectrum of CS and highlights the overlap between conditions caused by mutations in FGFR2.

PREDICTIVE MODELING OF HOSPITAL READMISSION RATES USING ELECTRONIC MEDICAL RECORD-WIDE MACHINE LEARNING: A CASE-STUDY USING MOUNT SINAI HEART FAILURE COHORT.

Reduction of preventable hospital readmissions that result from chronic or acute conditions like stroke, heart failure, myocardial infarction and pneumonia remains a significant challenge for improving the outcomes and decreasing the cost of healthcare delivery in the United States. Patient readmission rates are relatively high for conditions like heart failure (HF) despite the implementation of high-quality healthcare delivery operation guidelines created by regulatory authorities. Multiple predictive models are currently available to evaluate potential 30-day readmission rates of patients. Most of these models are hypothesis driven and repetitively assess the predictive abilities of the same set of biomarkers as predictive features. In this manuscript, we discuss our attempt to develop a data-driven, electronic-medical record-wide (EMR-wide) feature selection approach and subsequent machine learning to predict readmission probabilities. We have assessed a large repertoire of variables from electronic medical records of heart failure patients in a single center. The cohort included 1,068 patients with 178 patients were readmitted within a 30-day interval (16.66% readmission rate). A total of 4,205 variables were extracted from EMR including diagnosis codes (n=1,763), medications (n=1,028), laboratory measurements (n=846), surgical procedures (n=564) and vital signs (n=4). We designed a multistep modeling strategy using the Naïve Bayes algorithm. In the first step, we created individual models to classify the cases (readmitted) and controls (non-readmitted). In the second step, features contributing to predictive risk from independent models were combined into a composite model using a correlation-based feature selection (CFS) method. All models were trained and tested using a 5-fold cross-validation method, with 70% of the cohort used for training and the remaining 30% for testing. Compared to existing predictive models for HF readmission rates (AUCs in the range of 0.6-0.7), results from our EMR-wide predictive model (AUC=0.78; Accuracy=83.19%) and phenome-wide feature selection strategies are encouraging and reveal the utility of such datadriven machine learning. Fine tuning of the model, replication using multi-center cohorts and prospective clinical trial to evaluate the clinical utility would help the adoption of the model as a clinical decision system for evaluating readmission status.

The ophthalmic sequelae of Pfeiffer syndrome and the long-term visual outcomes after craniofacial surgery.

BACKGROUND: Pfeiffer syndrome is a rare, genetic condition characterized by craniosynostosis and midface hypoplasia, with resultant ophthalmic sequelae. The gold standard of treatment is fronto-orbital advancement. We analyzed a large database of Pfeiffer syndrome patients to report the rate of ophthalmic sequelae and the long-term visual outcomes after craniofacial surgery and to compare Pfeiffer syndrome to other craniosynostosis syndromes. METHODS: The medical records of Pfeiffer syndrome patients examined between 1988 and 2010 were examined retrospectively. Diagnosis was based on clinical and genetic testing. Long-term data were presented as a rate of incidence per person-year to overcome variable follow-up times. RESULTS: A total of 22 patients were included. Proptosis (n = 21 [95%]), refractive error (n = 13 [59%]), and strabismus (n = 12 [55%]) were the most common primary features at presentation. Exposure keratitis (n = 9 [41%]) and amblyopia (n = 3 [14%]) were the most common secondary features. At presentation, 24 eyes [86%] with documented best-corrected visual acuity were normal; 4 [14%] were impaired; and none were blind. Fronto-orbital advancement reduced the rate of proptosis from 28%/person-year at presentation to 2%/person-year. There were no cases of active exposure disease postoperatively. At last follow-up, there was a 7%/person-year rate of impaired vision secondary to corneal scarring and amblyopia and a 3%/person-year rate of blindness-all from optic atrophy. CONCLUSIONS: In this study, the rates of proptosis and exposure keratitis were high in Pfeiffer syndrome, especially compared to Apert and Crouzon syndromes. Fronto-orbital advancement was successful in correcting orbital abnormalities. Long-term ophthalmic follow-up is essential to ensure best visual outcome.

Apert syndrome: Surgical outcomes and perspectives.

PURPOSE: Apert syndrome is a rare congenital malformation with severe craniofacial anomalies. The aim of this study was to review the outcomes of craniofacial and neurosurgical interventions in Apert syndrome patients treated at a single institution. MATERIALS AND METHODS: A retrospective review of all patient records with a diagnosis of Apert syndrome assessed and managed in the Australian Craniofacial Unit (ACFU) from 1985 to 2013 was conducted. RESULTS: A total of 94 patients were identified, and 130 transcranial procedures were performed. Of the patients, 83 underwent a fronto-orbital advancement (FOA) as their primary procedure, and 18 patients also underwent a posterior vault procedure. Twenty patients underwent a fronto-facial monobloc advancement. Overall, 70% of patients underwent at least 2 transcranial procedures. Shunts were inserted in 2 patients preoperatively and in 5 patients postoperatively for cerebrospinal fluid (CSF) leaks or acute hydrocephalus. Re-do FOAs were performed in 8 patients. Patients who underwent an FOA at the age of more than 18 months had no recurrence of raised intracranial pressure (ICP). Of 18 patients who also underwent a posterior vault procedure, 1 patient had recurrence of raised ICP. Midfacial surgery was performed early if there was evidence of obstructive sleep apnoea (OSA), but delayed midfacial surgery was preferred. Complications were reported in 18% of procedures. The most common complications were CSF leaks and acute hydrocephalus. CONCLUSION: Shunting is rarely required in Apert syndrome patients, confirming a predominantly nonprogressive ventriculomegaly. FOA appears to be a more stable procedure when performed at an age of more than 18 months. Undergoing a posterior vault procedure may reduce the risk of recurrent raised ICP and lead to fewer transcranial procedures needed in childhood. Midfacial surgery should be delayed until adolescence where there is no evidence of OSA, psychological disturbance, or complications of exorbitism. Complications are rare when these patients are treated by an experienced craniofacial team.

Apert Syndrome: Outcomes From the Australian Craniofacial Unit's Birth to Maturity Management Protocol.

The complex, progressive, multisystem nature of Apert syndrome presents many challenges to managing surgeons. Based on the pioneering work of Paul Tessier, the senior author developed a multidisciplinary birth to maturity management protocol for Apert syndrome. Between 1975 and 2014 the Australian Craniofacial Unit has treated 174 Apert syndrome patients and 28 have completed full protocol management. This paper reviews the scientific contribution made to the management of Apert syndrome by the Australian Craniofacial Unit, the development and evolution of the protocol and presents comprehensive data on the surgical and nonsurgical craniofacial interventions, and outcomes for the 28 patients who have completed the programme; 26 had normal visual acuity, 22 had normal hearing, 20 achieved normal or nearly normal speech, 24 a functional class I occlusion, 18 completed mainstream schooling of whom at least 8 went on to tertiary education, at least 13 gained employment and 15 developed good social groups. These outcomes equal or exceed those presented by other authors and provide compelling evidence of the value of protocol management in clinical outcomes, in addition to their value in international collaboration, and scientific development of future therapeutic strategies for the management of Apert syndrome.

Dolor en el anciano: calidad de vida, funcionalidad y factores asociados. Estudio SABE, Bogotá, Colombia / Pain in the elderly: Quality of life, functionality and associated factors. SABE Study, Bogotá, Colombia

Objetivo. Precisar el impacto del dolor en la calidad de vida y en la funcionalidad de la población anciana. Materiales y métodos. Se analizaron los datos del estudio SABE-Bogotá, que incluyó 2.000 personas de 60 años o más en una muestra transversal probabilística por conglomerados (cobertura del 81,9%). Se utilizó la variable «Presencia de dolor» y se relacionó con los factores sociodemográficos, la autopercepción de salud, las comorbilidades, el estado funcional, el estado cognoscitivo y la calidad de vida, esta última estimada con la Escala visual analógica del grupo EuroQOL. Resultados. La mayoría de los ancianos encuestados fueron mujeres (63,4%), y la edad promedio fue de 71,17 años (DE = 8,05), con una mayoría de individuos entre los 60 y 69 años (48%). Al comparar los grupos con dolor versus no dolor, se encontraron puntajes significativamente más bajos en el grupo con dolor (p < 0,001) tanto en las escalas de funcionalidad como en la de calidad de vida EQ-VAS. Las asociaciones más fuertes con el dolor fueron las enfermedades articulares (OR: 3,08 [2,24-4,23]), la depresión severa (OR: 2,80 [1,63-4,79]) y el compromiso funcional de las actividades básicas de la vida diaria (ABVD) (OR: 2,45 [1,31-4,58]). Conclusiones. El dolor impacta negativamente en la independencia funcional y la percepción de calidad de vida de las personas adultas mayores, predisponiéndolas a desenlaces adversos (AU)

Objective. To determine the impact of pain on the quality of life in older adults and its association with functionality. Materials and methods. Data was taken from SABE Bogota study. A cross-sectional study was carried out during 2012, interviewing 2,000 individuals of 60 years and older, as a probabilistic cluster and representative sample. The variable ‘presence of pain’ to was used to identify associations with sociodemographic factors, self-rated health, comorbidities, functional status, cognitive status, and quality of life. The latter was estimated using a visual analogue scale of the EuroQOL tool (EQ-VAS). Results. The majority of older adults were women (63.4%); the mean age was 71.17 years (SD = 8.05), with a higher frequency of individuals in the age group between 60 and 69 years (48%). When comparing groups with pain vs. no pain, significantly lower scores were found in the group with pain (P < .001) in both the functionality and quality of life EQ-VAS scales. The strongest associations with pain were: joint diseases (OR: 3.08 [2.24-4.23]), severe depression (OR: 2.80 [1.63-4.79]) and functional impairment of the Basic Activities of Daily Living (BADL) (OR: 2.45 [1.31-4.58]). Conclusions. Pain negatively impacts the functional independence and the perception of the quality of life in older adults, predisposing them to adverse outcomes (AU)

The Craniofacial and Upper Limb Management of Nager Syndrome.

INTRODUCTION: Nager syndrome is a rare condition characterized by craniofacial and upper limb abnormalities. It is commonly mistaken for Treacher Collins syndrome, with which it shares the same craniofacial phenotype. However, patients with Treacher Collins do not exhibit hand anomalies, which are seen in patients with Nager syndrome. This paper reviews the multidisciplinary management of patients with Nager syndrome who were treated at the Australian Craniofacial Unit, Adelaide and the Erasmus Medical Centre, Rotterdam. METHODS: The database of both units was scrutinized and the case-notes of the patients with Nager syndrome were reviewed. Data was collected on patient demographics, surgical management, complications, and outcome. RESULTS: Nine patients (6 M:3 F) were identified with Nager syndrome, with a mean age at presentation of 3.7 years (range 8 days to 11.8 years). The mean follow-up time was 2.2 years (2 months to 19 years). SF3B4 mutation was noted in 2 patients and 1 patient had an X:9 translocation. Seven (77.8%) had obstructive sleep apnoea, with 5 patients diagnosed as severe obstructive sleep apnoea. Four patients had pollicization of their index, 2 patients had excision of extra radial digits and 1 patient underwent thumb duplication correction. Craniofacial surgery included mandibular advancement in 5 patients, temporo-mandibular joint reconstruction in 2 patients, and a genioplasty in 1 patient. CONCLUSION: Nager syndrome is a rare acrofacial dysostosis syndrome that is best managed within the realms of a multidisciplinary team. The authors would advocate early pollicization in patients with thumb anomalies to prevent any impairment in manual dexterity.

Central nervous system and cervical spine abnormalities in Apert syndrome.

PURPOSE: Apert syndrome characterized by acrocephalosyndactyly is a rare autosomal dominant congenital malformation with a prevalence of 1/65,000 births. With an extensive range of phenotypic and developmental manifestations, its management requires a multidisciplinary approach. A variety of craniofacial, central nervous system (CNS), and cervical spine abnormalities have been reported in these patients. This study aimed to determine the incidence of these CNS abnormalities in our case series. METHODS: Retrospective review of Australian Craniofacial Unit (ACFU) database for Apert patients was performed. Data collected that included demographics, place of origin, age at presentation, imaging performed, and images were reviewed and recorded. Where available, developmental data was also recorded. RESULTS: Ninety-four patients seen and managed at the ACFU had their CNS and cervical spine abnormalities documented. The main CNS abnormalities were prominent convolutional markings (67 %), ventriculomegaly (48 %), crowded foramen magnum (36 %), deficient septum pellucidum (13 %), and corpus callosum agenesis in 11 %. Major C-spine findings were present in 50.8 % of patients and included fusion of posterior elements of C5/C6 (50 %) and C3/4 (27 %). Multilevel fusion was seen in 20 %. Other abnormalities were C1 spina bifida occulta (7 %) and atlanto-axial subluxation (7 %). CONCLUSION: Multiple CNS and cervical spine (c-spine) abnormalities are common in Apert syndrome. The significance of these abnormalities remains largely unknown. Further research is needed to better understand the impact of these findings on growth, development, and treatment outcomes.

The Management of Nonsyndromic, Isolated Sagittal Synostosis.

AIM: Isolated sagittal synostosis is the commonest form of craniosynostosis. The reasons for surgery are to normalize the head shape and to increase the cranial volume, thus reducing the risk of raised intracranial pressure and allowing for normal brain development. It has been suggested that sagittal synostosis may impair neuropsychological development. This systematic review appraised the literature on the management of sagittal synostosis. METHODS: A literature search was performed with the assistance of a professional librarian. Studies selected had to satisfy the criteria set by PICO (patients, intervention, comparison, and outcome). Cranial index and neuropsychological outcome were used as outcome measures. MINORS was used to assess the methodological quality of the selected articles. A score of 75% was deemed to be of satisfactory quality, and the quality of the evidence from the selected studies was graded using the GRADE system. RESULTS: One hundred forty-eight articles were initially identified. Only 6 articles fulfilled the PICO criteria and scored a minimum of 75% on MINORS. Four studies compared 1 technique to another with documented cranial indices. Two studies compared 1 group to another and assessed the neuropsychological development. According to GRADE, the quality of evidence was deemed to be very low. CONCLUSIONS: This systematic review assessed cranial index and neuropsychological outcome following surgery for isolated, nonsyndromic sagittal synostosis. The quality of the evidence in the published literature was noted to be of very low quality. There is a need for better-designed, prospective studies to guide surgeons involved in management of sagittal synostosis.