The non-operative correction of ear anomalies in infants using the EarWell infant corrective system in the Netherlands.

BACKGROUND: Congenital ear anomalies are regular but often overlooked occurrences. The golden standard of treatment has been to surgically correct these anomalies at a minimum age of 5 to 7 years. As of the last century, ear molding has developed to be a safe, reliable, and effective treatment method. Different treatment methods are still under investigation. This study aims to investigate the use of the EarWell Infant Corrective System in the Dutch population. METHODS: Children aged 0-12 weeks were included in the Zuyderland Medical Center to be treated with the EarWell Infant Corrective System in case of ear deformations. Every 2 weeks, the system was replaced and correction was evaluated by both physician and parents. RESULTS: Seventy-three participants were included, of whom 123 ears in total were treated. Age at initiation was 35.5 days on average; treatment lasted an average of 59 days. Parents and physicians both reported an amelioration of all ear anomalies after treatment, scoring the correction grade an 8.8. Overall satisfaction with the treatment method was 9 or higher for both groups. CONCLUSIONS: The EarWell Infant Corrective System is a safe, reliable, and effective treatment method for the correction of ear anomalies in infants.

The Potential of the HeartLogicTM Algorithm in Patients with a Left Ventricular Assist Device, an Initial Report.

BACKGROUND: Survival and quality-of-life of left ventricular assist device (LVAD) recipients improved significantly because of growing experience and technological advances. However, LVAD-related complication rates, including recurrent episodes of congestion, remain high. Early detection of fluid retention to provide a time-window for medical intervention is the pillar in preventing hospitalizations. The multisensory HeartLogicTM algorithm accurately detected impending congestion in ambulant heart failure patients. The aim of the current study is to investigate the feasibility of HeartLogicTM-driven care in LVAD patients. METHODS: Consecutive LVAD destination therapy patients were followed-up according the structured HeartLogicTM-based heart failure carepath. An alert triggered a device check-up, and the heart failure team contacted the patient to evaluate for signs and symptoms of impending congestion. An alert was adjudicated as true positive or unexplained. An episode of congestion not preceded by an alert was deemed as a false negative. RESULTS: Data from 7 patients were included: the median age was 67 years [IQR 61-71], 71% were male and 71% had a non-ischemic aetiology. Total follow-up entailed 12 patient-years. All patients experienced at least one alert. In total, 33 alerts were observed. Majority of alerts (70%, n = 23) were driven by congestion and one alerts (15%) were clinically meaningful but not primarily fluid-retention-related (e.g., altered hemodynamic triggered by a pump thrombosis). Of all the alerts, five (15%) were classified as an unexplained alert, and during follow-up, four false negative episodes were documented. CONCLUSIONS: HeartLogicTM-driven care with continuous monitoring to detect impending fluid retention in LVAD patients was feasible and deserves further prospective validation.

A case report of an intracardiac paraganglioma attached to the left main coronary artery in a patient with a succinate dehydrogenase complex subunit D mutation.

Background: Cardiac paragangliomas are extremely rare neuroendocrine tumours derived from neural crest cells that represent <2% of all paragangliomas. Approximately 35-40% of all paragangliomas are associated with inherited syndromes such as mutation in the succinate dehydrogenase (SDH) enzyme. Case summary: A 44-year-old male with an SDH complex subunit D (SDHD) mutation was diagnosed with an intracardiac paraganglioma attached to the left main coronary artery. Multimodality imaging, including gallium dotatate positron emission tomography computed tomography, cardiac magnetic resonance imaging, and coronary computed tomography angiography (CCTA) confirmed the suspected intracardiac paraganglioma. During follow-up with a CCTA, the mass showed growth, and surgical removal was recommended to anticipate on the risk of compression of the left main coronary artery. Prior to surgery, coronary angiography was performed, which showed no coronary calcifications. The highly vascularized paraganglioma was visible near the left main and proximal left anterior descending artery. The intracardiac paraganglioma was successfully removed through a median sternotomy with cardiopulmonary bypass, without any complications. The post-operative course was uneventful, and histological examination confirmed the diagnosis of a paraganglioma. Discussion: Intracardiac paragangliomas in the vicinity of the left main coronary artery are rare, and surgical removal may be challenging. Therefore, screening and the use of multiple imaging modalities in patients with SDHD mutations prior to surgery is of major importance.

One-Year Mortality in Patients Undergoing an Implantable Cardioverter Defibrillator or Cardiac Resynchronization Therapy Pulse Generator Replacement: Identifying Patients at Risk.

Implantable cardioverter defibrillators (ICDs) significantly contribute to the prevention of sudden cardiac death in selected patients. However, it is essential to identify those who are likely to not have benefit from an ICD and to defer a pulse generator exchange. Easily implementable guidelines for individual risk stratification and decision making are lacking. This study investigates the 1-year mortality of patients who underwent an ICD or cardiac resynchronization therapy with defibrillator function (CRT-D) pulse generator replacement in a contemporary real-world tertiary hospital setting. The cause of death and patient- and procedure-related factors are stratified, and predictive values for 1-year mortality are evaluated. Patients with a follow-up of ≥365 days (or prior mortality) after an ICD or CRT-D exchange at the Leiden University Medical Center from 1 January 2018 until 31 December 2021 were eligible. In total, 588 patients were included (77% male, 69 [60-76] years old, 59% primary prevention, 46% ischemic cardiomyopathy and 37% mildly reduced left ventricular ejection fraction (LVEF)). Patients undergoing a CRT-D replacement or upgrade had a significantly higher 1-year all-cause mortality (10.7% and 11.9%, respectively) compared to patients undergoing ICD (2.8%) exchange (p = 0.002). LVEF ≤ 30%, New York Heart Association class ≥ 3, estimated glomerular filtration rate ≤ 30 mL/min/m2 and haemoglobin ≤ 7 mmol/L were independently associated with mortality within 1 year after pulse generator replacement. There is a growing need for prospectively validated risk scores to weight individualized risk of mortality with the expected ICD therapy benefit and to support a well-informed, shared decision-making process.

Fewer Worsening Heart Failure Events With HeartLogic on top of Standard Care: a Propensity-Matched Cohort Analysis.

BACKGROUND: The implantable cardiac defibrillator-based HeartLogic algorithm aims to detect impending fluid retention in patients with heart failure (HF). Studies show that HeartLogic is safe to integrate into clinical practice. The current study investigates whether HeartLogic provides clinical benefit on top of standard care and device telemonitoring in patients with HF. METHODS: A multicenter, retrospective, propensity-matched cohort analysis was performed in patients with HF and implantable cardiac defibrillators, and it compared HeartLogic to conventional telemonitoring. The primary endpoint was the number of worsening HF events. Hospitalizations and ambulatory visits due to HF were also evaluated. RESULTS: Propensity score matching yielded 127 pairs (median age 68 years, 80% male). Worsening HF events occurred more frequently in the control group (2; IQR 0-4) compared to the HeartLogic group (1; IQR 0-3; P = 0.004). The number of HF hospitalization days was higher in controls than in the HeartLogic group (8; IQR 5-12 vs 5; IQR 2-7; P = 0.023), and ambulatory visits for diuretic escalation were more frequent in the control group than in the HeartLogic group (2; IQR 0-3 vs 1; IQR 0-2; P = 0.0001). CONCLUSION: Integrating the HeartLogic algorithm in a well-equipped HF care path on top of standard care is associated with fewer worsening HF events and shorter duration of fluid retention-related hospitalizations.

Helmet therapy for positional cranial deformations; a 5-year follow-up study.

The aim of this study was to assess the long-term effects of helmet therapy in its use for positional cranial deformation, such as plagiocephaly and brachycephaly, by evaluating head shapes in children with a 5-year interval. These children were included at a neonatal age, with a deviating cranial shape classified as positional deformation, and received different forms of therapy, varying from none to physical and helmet therapy combined. Cranial shape was measured before and after therapy, using plagiocephalometry. Both positional plagiocephaly and positional brachycephaly decrease significantly in prevalence and severity over time (P = 0.031 and P < 0.001, respectively), with average relative reductions of 194.5% in ODDI (P = 0.001) and of 878.4% in CPI (P < 0.001). Although the decrease in ODDI and CPI did not significantly vary between treatment methods, ODDI is shown to significantly decrease in case of therapy opposed to no therapy, especially if treatment consists of both physical therapy and helmet therapy (P < 0.001). The long-term effects of therapy are therefore beneficial in the treatment of positional plagiocephaly and will increase the reduction in ODDI, especially in case of both physical and helmet therapy.

Performance of a HeartLogicTM Based Care Path in the Management of a Real-World Chronic Heart Failure Population.

Aim: Early detection of impending fluid retention and timely adjustment of (medical) therapy can prevent heart failure related hospitalizations. The multisensory cardiac implantable electronic device (CIED) based algorithm HeartLogicTM aims to alert in case of impending fluid retention. The aim of the current analysis is to evaluate the performance of the HeartLogicTM guided heart failure care path in a real-world heart failure population and to investigate whether the height of the index and the duration of the alert state are indicative of the degree of fluid retention. Methods: Consecutive adult heart failure patients with a CIED and an activated HeartLogicTM algorithm were eligible for inclusion. Patients were followed up according to the hospital's heart failure care path. The device technician reviewed alerts for a technical CIED checkup. Afterwards, the heart failure nurse contacted the patient to identify impending fluid retention. An alert was either true positive or false positive. Without an alert a patient was true negative or false negative. Results: Among 107 patients, [82 male, 70 (IQR 60-77) years, left ventricular ejection fraction 37 ± 11%] 130 HeartLogicTM alerts were available for analysis. Median follow up was 14 months [IQR 8-23]. The sensitivity to detect impending fluid retention was 79%, the specificity 88%. The positive predictive was value 71% and the negative predictive value 91%. The unexplained alert rate was 0.23 alerts/patient year and the false negative rate 0.17 alerts/patient year. True positive alerts [42 days (IQR 28-63)] lasted longer than false positive alerts [28 days (IQR 21-44)], p = 0.02. The maximal HeartLogicTM index was higher in true positive alerts [26 (IQR 21-34)] compared to false positive alerts [19 (IQR 17-24)], p < 0.01. Patients with higher HeartLogicTM indexes required more intense treatment (index height in outpatient setting 25 [IQR 20-32], day clinic treatment 28 [IQR 24-36] and hospitalized patients 45 [IQR 35-58], respectively), p < 0.01. Conclusion: The CIED-based HeartLogicTM algorithm facilitates early detection of impending fluid retention and thereby enables clinical action to prevent this at early stage. The current analysis illustrates that higher and persistent alerts are indicative for true positive alerts and higher index values are indicative for more severe fluid retention.

Influence of the sentinel node outcome analysed by one-step nucleic acid amplification on the risk for postmastectomy radiation therapy and the scheduling of immediate breast reconstruction.

BACKGROUND: In patients with cT1-T3N0 breast cancer, postmastectomy radiation therapy (PMRT) is considered a contraindication for immediate breast reconstruction (IBR) due to a high risk of complications. The sentinel node biopsy (SNB) is an important determinant for PMRT. In this study, we evaluated the impact of SNB outcome on the planning of IBR after mastectomy. METHODS: Data of patients undergoing mastectomy and SNB in Zuyderland Medical Centre between 2016 and 2019 were retrospectively analysed. Perioperative factors influencing the planning of IBR and SNB results assessed by the intraoperative one-step nucleic acid amplification (OSNA) were registered. RESULTS: Of 397 patients, 169 opted for IBR. One hundred and seven IBRs were performed: 101 tissue expanders, 5 deep inferior epigastric perforator flaps and 1 latissimus dorsi flap.  Eighteen patients (18/107) had macrometastases in the SNB, in six of them IBR was cancelled due to the indication for PMRT (33%). In the other 12 patients IBR was executed as planned.  A delayed reconstruction was performed in 59 patients. CONCLUSION: In breast cancer patients undergoing mastectomy with macrometastases in the SNB, IBR was postponed in 33% due to risk on PMRT. To predict this risk, we advise to acknowledge the SNB outcome by using OSNA before proceeding to IBR.

The Perception of Esthetic Importance of Craniofacial Elements.

ABSTRACT: The human face is an important human structure, often judged by its esthetics. It serves great purpose, as it is and always has been involved in both cultural matters, such as art, as well as biological matters, such as exhibition of emotion and mate selection. Throughout history, there have been multiple studies on what makes a face attractive. Despite the myriad ways in which the face has been explored academically, there has been a lack of reports on which craniofacial elements are deemed the most important for attractiveness subjectively. To fill up this gap on the perception of the esthetics of the human face, as well as to provide a contemporary view on the matter, this research was conducted. Participants were asked to fill out a questionnaire with a visual analogue scale for each craniofacial element and questions about appearance satisfaction. A total of 299 participants were enrolled in this study. Eyes, hair and teeth were deemed to be the most important craniofacial elements for attractiveness. Women scored teeth, hair, and eyebrows significantly higher than men, men scored higher in satisfaction with their own appearance than women. Older people, married people and people in a relationship care less about the opinion of others regarding their appearance. The results from this study can be used to identify a focus in the future development of esthetically related matters, such as plastic surgery or art and will add to the academic knowledge on the perception of craniofacial beauty.

Demographics of Positional Plagiocephaly and Brachycephaly; Risk Factors and Treatment.

ABSTRACT: In the last 3 decades, the incidence of positional cranial deformations in infants, such as positional plagiocephaly and positional brachycephaly, has increased. The deviating shape often causes parental concern for the later psychosocial wellbeing of the child. Treatment options are nonoperative, varying from positional change to helmet therapy, of which the effect has often been debated. Multiple risk factors have been associated with an increased risk on the development of these deformations. The goal of this study was to assess the impact of known risk factors on the type and severity of resulting positional cranial deformation.Parents were asked to fill out a questionnaire regarding the presence of risk factors, such as gender, age, pregnancy duration, method of delivery and reasons for atypical deliveries, breech position, birth weight, developmental status, positional preference, family history, number of siblings, and torticollis presence. Treatment methods were documented and plagiocephalometry was used to measure the cranial proportions. All children were invited to participate in short-term follow-up. The significance of the risk factors and the effect of different kinds of therapy are discussed. Although no significant correlation was found between severity and risk factors, some risk factors could be correlated with the kind of positional cranial deformation. Of the different therapies, helmet therapy had a significant impact on the reduction of positional plagiocephaly.

Early Detection of Fluid Retention in Patients with Advanced Heart Failure: A Review of a Novel Multisensory Algorithm, HeartLogicTM.

Heart failure (HF) hospitalisations due to decompensation are associated with shorter life expectancy and lower quality of life. These hospitalisations pose a significant burden on the patients, doctors and healthcare resources. Early detection of an upcoming episode of decompensation may facilitate timely optimisation of the ambulatory medical treatment and thereby prevent heart-failure-related hospitalisations. The HeartLogicTM algorithm combines data from five sensors of cardiac implantable electronic devices into a cumulative index value. It has been developed for early detection of fluid retention in heart failure patients. This review aims to provide an overview of the current literature and experience with the HeartLogicTM algorithm, illustrate how the index can be implemented in daily clinical practice and discuss ongoing studies and potential future developments of interest.

The Effect of Cranial Shape on Esthetic Self-Worth in Bald Men.

ABSTRACT: During the last 3 decades, the incidence of positional plagiocephaly and brachycephaly has increased. Treatment options are conservative and can include physiotherapy and molding helmet therapy. The decision to start therapy is based on patient history and subjective assessment of cranial shape by the physician and the parents. To determine the impact of these deformations on esthetic self-worth, as well as cranial shape, this study was conducted on a population of adult bald men.Participants were asked to fill in a questionnaire about the amount of comments received on cranial shape, as well as grade their general esthetic and grade craniofacial features using a visual analogue scale. The cranial proportions and symmetry were measured using plagiocephalometry.50 adult bald men were included in this study. The prevalence of nonsynostotic plagiocephaly was 12%; the prevalence of nonsynostotic brachycephaly was 0%. No significant correlation was found between plagiocephaly or brachycephaly, cranial circumference, width or length and amount of comments on cranial shape, cosmetic assessment of appearance or cosmetic assessment of cranial shape. On average, teeth and eyes were given the highest cosmetic value, eyebrows and chin were given the lowest scores.Cranial shape does not have a significant impact on the amount of comments received on cranial shape, nor the cosmetic assessment of either overall appearance or cranial shape in bald men. For bald men, eyes and teeth are more important craniofacial features in cosmetic assessment. There is no clear esthetic need to treat deviating cranial shape with helmet therapy in male patients.

Non-surgical Correction of Congenital Ear Anomalies: A Review of the Literature.

Congenital ear anomalies have been known to cause lasting psychosocial consequences for children. Congenital ear anomalies can generally be divided into malformations (chondro-cutaneous defect) and deformations (misshaped pinna). Operative techniques are the standard for correction at a minimal age of 5-7, exposing the children to teasing and heavy complications. Ear molding is a non-operative technique to treat ear anomalies at a younger age. Having been popularized since the 1980s, its use has increased over the past decades. However, uncertainties about its properties remain. Therefore, this review was conducted to look at what is known and what has been newly discovered in the last decade, comparing different treatment methods and materials. A literature search was performed on PubMed, and 16 articles, published in the last decade, were included. It was found that treatment initiated at an early age showed higher satisfactory outcome rates and a shorter duration of treatment. A shorter duration of treatment also led to higher satisfactory rates, which might be attributable to age at initiation, individual moldability, and treatment compliance. Complications were minor in all articles. Recurrence rate was low and mostly concerned prominent ears, which proved to be the most difficult to correct deformity as well. Malformations, however, were even more difficult to treat than deformations. Our analysis shows ear molding to be a successful treatment method for ear anomalies with a preference for early diagnosis and treatment.

Prevalence and Consequences of Positional Plagiocephaly and Brachycephaly.

The incidence of positional plagiocephaly and brachycephaly in infants has increased. Treatment options include physiotherapy and helmet therapy.No information exists on the prevalence, cosmetic, and psychological consequences of plagiocephaly and brachycephaly later in life. This study was performed to assess the prevalence and cosmetic relevance of plagiocephaly and brachycephaly, as well as its influence on quality of life in adolescence.The authors performed plagiocephalomety to assess cranial shape and used a questionnaire that included question about educational level, medical history, cosmetic appearance, and cranial shape. To evaluate quality of life, the authors used the KIDSCREEN-27 questionnaire.All data were analyzed using SPSS version 19.0 2010. The authors included 87 adolescents. The prevalence of plagiocephaly, defined as an oblique diameter difference index (ODDI) higher than 106%, was 10.3%; the prevalence of brachycephaly, defined as a cranial proportional index (CPI) higher than 95%, was 0%.There was no significant correlation between the level of the ODDI or CPI and the cosmetic assessment of cranial shape (P = 0.128/0.541). There was no significant correlation with the level of the ODDI (P = 0.428).There was no significant correlation between the level of the ODDI or CPI and the average T-value for quality of life using the KIDSCREEN quality-of-life questionnaire (P = 0.461/0.713). The prevalence of positional deformities in our population of adolescents, born after the "back to sleep" campaign, is low. There was no significant correlation between the presence of a cranial deformity and the cosmetic judgement and quality of life.

Positional plagiocephaly and brachycephaly: is there a correlation between subjective and objective assessment of cranial shape?

INTRODUCTION: During the last 2 decades, the incidence of positional plagiocephaly and brachycephaly has increased. Treatment options are conservative and can include physiotherapy and molding helmet therapy. The decision to start helmet therapy is based on patient history and subjective assessment of cranial shape by the physician and the parents. Recently, a noninvasive, objective, reliable, and valid measurement instrument became available: the plagiocephalometry (PCM). Because there are no data available comparing the result of PCM with subjective assessment of cranial shape, we performed the current study. METHODS: All consecutive children with positional plagiocephaly and brachycephaly admitted to the craniofacial outpatient clinic between October 2008 and July 2009 were included. Physician and parents assessed the cranial shape of the child, using a numeric scale from 1 to 10. In consultation with the parents, the physician decided whether helmet therapy was indicated. Plagiocephalometry was performed after visit to the physician. RESULTS: We included 75 patients in our study. There was a significant correlation between the numeric score from the physician and both the oblique diameter difference index (P < 0.001) and the cranial proportional index (P = 0.023). There was no significant correlation between the numeric score from the parents and both the oblique diameter difference index (P = 0.427) and the cranial proportional index (P = 0.155). CONCLUSIONS: There is a significant correlation between the subjective assessment of cranial shape by the physician and PCM results. Plagiocephalometry can be a useful additive tool to assess cranial shape.

[Babies with cranial deformity]. / Zuigelingen met een scheef hoofd.

Plagiocephaly was diagnosed in a baby aged 4 months and brachycephaly in a baby aged 5 months. Positional or deformational plagio- or brachycephaly is characterized by changes in shape and symmetry of the cranial vault. Treatment options are conservative and may include physiotherapy and helmet therapy. During the last two decades the incidence of positional plagiocephaly has increased in the Netherlands. This increase is due to the recommendation that babies be laid on their backs in order to reduce the risk of sudden infant death syndrome. We suggest the following: in cases of positional preference of the infant, referral to a physiotherapist is indicated. In cases of unacceptable deformity of the cranium at the age 5 months, moulding helmet therapy is a possible treatment option.