[Acquired foot drop]. / Verworven klapvoet.

A dropping foot is the consequence of a variety of debilitating conditions and is oftentimes treated conservatively by general practitioners and other specialists. Typically, it is caused by peroneal nerve palsy secondary to compression or a hernia nucleosipulpei at the level L4-L5. Identifying the underlying pathology requires a neurological work-up oftentimes including ultrasound and electromyographic investigation. When a peroneal nerve compression is found, decompression can be achieved operatively. Should the underlying cause of the dropping foot have been treated adequately without an effect on the foot itself, then a posterior tibial tendon transfer may be considered. Generally, a posterior tibial tendon transfer has good outcomes for the treatment of dropping foot although it is partly dependent on the physiotherapy that accompanies it.

Backside versus Frontside SN2 Reactions of Alkyl Triflates and Alcohols.

Nucleophilic substitution reactions are elementary reactions in organic chemistry that are used in many synthetic routes. By quantum chemical methods, we have investigated the intrinsic competition between the backside SN2 (SN2-b) and frontside SN2 (SN2-f) pathways using a set of simple alkyl triflates as the electrophile in combination with a systematic series of phenols and partially fluorinated ethanol nucleophiles. It is revealed how and why the well-established mechanistic preference for the SN2-b pathway slowly erodes and can even be overruled by the unusual SN2-f substitution mechanism going from strong to weak alcohol nucleophiles. Activation strain analyses disclose that the SN2-b pathway is favored for strong alcohol nucleophiles because of the well-known intrinsically more efficient approach to the electrophile resulting in a more stabilizing nucleophile-electrophile interaction. In contrast, the preference of weaker alcohol nucleophiles shifts to the SN2-f pathway, benefiting from a stabilizing hydrogen bond interaction between the incoming alcohol and the leaving group. This hydrogen bond interaction is strengthened by the increased acidity of the weaker alcohol nucleophiles, thereby steering the mechanistic preference toward the frontside SN2 pathway.

Communicating Emotion: Vocal Expression of Linguistic and Emotional Prosody in Children With Mild to Profound Hearing Loss Compared With That of Normal Hearing Peers.

OBJECTIVES: Emotional prosody is known to play an important role in social communication. Research has shown that children with cochlear implants (CCIs) may face challenges in their ability to express prosody, as their expressions may have less distinct acoustic contrasts and therefore may be judged less accurately. The prosody of children with milder degrees of hearing loss, wearing hearing aids, has sparsely been investigated. More understanding of the prosodic expression by children with hearing loss, hearing aid users in particular, could create more awareness among healthcare professionals and parents on limitations in social communication, which awareness may lead to more targeted rehabilitation. This study aimed to compare the prosodic expression potential of children wearing hearing aids (CHA) with that of CCIs and children with normal hearing (CNH). DESIGN: In this prospective experimental study, utterances of pediatric hearing aid users, cochlear implant users, and CNH containing emotional expressions (happy, sad, and angry) were recorded during a reading task. Of the utterances, three acoustic properties were calculated: fundamental frequency (F0), variance in fundamental frequency (SD of F0), and intensity. Acoustic properties of the utterances were compared within subjects and between groups. RESULTS: A total of 75 children were included (CHA: 26, CCI: 23, and CNH: 26). Participants were between 7 and 13 years of age. The 15 CCI with congenital hearing loss had received the cochlear implant at median age of 8 months. The acoustic patterns of emotions uttered by CHA were similar to those of CCI and CNH. Only in CCI, we found no difference in F0 variation between happiness and anger, although an intensity difference was present. In addition, CCI and CHA produced poorer happy-sad contrasts than did CNH. CONCLUSIONS: The findings of this study suggest that on a fundamental, acoustic level, both CHA and CCI have a prosodic expression potential that is almost on par with normal hearing peers. However, there were some minor limitations observed in the prosodic expression of these children, it is important to determine whether these differences are perceptible to listeners and could affect social communication. This study sets the groundwork for more research that will help us fully understand the implications of these findings and how they may affect the communication abilities of these children. With a clearer understanding of these factors, we can develop effective ways to help improve their communication skills.

A systematic review on the impact of auditory functioning and language proficiency on psychosocial difficulties in children and adolescents with hearing loss.

OBJECTIVE: Approximately 20% to 40% of children with hearing loss encounter psychosocial difficulties. This prevalence may be outdated, given the advancements in hearing technology and rehabilitation efforts to enhance the psychosocial well-being of these children. A systematic review of up-to-date literature can help to identify factors that may contribute to the children's psychosocial well-being. DESIGN/STUDY SAMPLE: A systematic review was conducted. Original articles were identified through systematic searches in Embase, Medline, PsychINFO, and Web of Science Core Collection. The quality of the papers was assessed using the Newcastle-Ottawa Quality Assessment Scale and custom Reviewers' Criteria. RESULTS: A search was performed on 20 October 2022. A total of 1561 articles were identified, and 36 were included for review. Critical appraisal led to 24 good to fair quality articles, and 12 poor quality articles. CONCLUSION: Children with hearing loss have a twofold risk of experiencing psychosocial difficulties compared to normal hearing peers. Estimates for functioning in social interactions, like speech perception (in noise) or language proficiency, have proven to be more adequate predictors for psychosocial difficulties than the degree of hearing loss. Our findings can be useful for identifying children at risk for difficulties and offering them earlier and more elaborate psychological interventions.

Total joint arthroplasty versus trapeziectomy in the treatment of trapeziometacarpal joint arthritis: a randomized controlled trial.

The aim of this double anonymized, randomized controlled trial was to determine whether total joint arthroplasty has superior outcomes than trapeziectomy 1 year after surgery for trapeziometacarpal osteoarthritis. A total of 62 women aged 40 years and older, scheduled for surgery for stage II or III osteoarthritis of the trapeziometacarpal joint, were included and randomized to trapeziectomy or total joint arthroplasty. The primary outcome was the total score of the Michigan Hand Outcomes Questionnaire. Secondary outcomes were the Michigan Hand Outcomes Questionnaire subscale scores, Disability of the Arm, Shoulder and Hand Questionnaire, active range of motion, strength, return to work, patient satisfaction and complications. Data were collected at baseline and at 3 and 12 months. At 1 year, we found no superiority of total joint arthroplasty over trapeziectomy regarding the total score of the Michigan Hand Outcomes Questionnaire. The total joint arthroplasty did show a significant advantage in strength and range of motion.Level of evidence: I.

First results of the Strengths and Difficulties Questionnaire, applied as a screening tool for psychosocial difficulties in pediatric audiology.

PURPOSE: Despite major improvements in rehabilitation possibilities, children with sensorineural hearing loss are at risk for psychosocial difficulties. These difficulties can impact their educational and career achievements and may be two to three times more common in children with hearing loss compared to those with normal hearing. Early identification of psychosocial difficulties can be facilitated using the Strengths and Difficulties Questionnaire (SDQ) and may improve outcomes and quality of life. We implemented the SDQ into the clinical follow-up of children with hearing loss in a tertiary referral hospital. With this, prevalence and severity of difficulties in specific psychosocial domains and several predictors were investigated. METHODS: A retrospective, cross-sectional investigation was performed of the following factors in association with the SDQ results: type of hearing device, type and degree of hearing loss, speech perception in quiet and in noise, and type of schooling. RESULTS: Between June 2020 and January 2022, parents of 312 children (age 4-18) completed the SDQ. An additional 113 child-reports were completed. The response rate of the parents was 69%. Problems with peer relationships and prosocial behavior were the most affected areas with clinically elevated scores in 22% of the children. Psychosocial difficulties were distributed similarly across types of hearing device, nature and degrees of hearing loss, and educational settings. Better speech perception in quiet was significantly associated with fewer parent-reported conduct problems. CONCLUSION: The results of the present study suggest that children with hearing loss may be at risk of experiencing challenges with social interactions and attachment in social contexts. Using the SDQ in clinical follow-up may improve the chances for early psychological assessment and intervention. In addition, the study found that children's mental health may be impacted by their communication abilities.

Development of a simulator for training of fetoscopic myelomeningocele surgery.

OBJECTIVE: To develop a realistic simulation model for laparotomy-assisted fetoscopic spina bifida aperta (SBa) surgery, to be used for training purposes and preoperative planning. METHODS: The predefined general requirement was a realistic model of an exteriorized uterus, allowing all neurosurgical steps of the intervention. The uterus was modelled using ultrasound and MRI images of a 25 weeks' gravid uterus, consisting of flexible polyurethane foam coated with pigmented silicone. The fetal model, contained an opening on the dorsal side for a customizable spinal insert with all the aspects of a SBa, including a cele, placode, and myofascial and skin layer. The model was assessed in a series of validation experiments. RESULTS: Production costs are low, uterus and fetus are reusable. Placental localization and the level and size of the spinal defect are adjustable, enabling case-specific adaptations. All aspects of the simulator were scored close to realistic or higher for both appearance and functional capacities. CONCLUSIONS: This innovative model provides an excellent training opportunity for centers that are starting a fetoscopic SBa repair program. It is the first simulation model with adjustable spinal defect and placental localisation. Further objective validation is required, but the potential for using this model in preoperative planning is promising.

The role of routine radial styloidectomy in proximal row carpectomy: a retrospective review of 120 patients.

Impingement between the radial styloid and the trapezium can occur after a proximal row carpectomy (PRC). We hypothesized that a PRC with primary radial styloidectomy reduces the risk of radial impingement, without affecting clinical or functional outcomes. In this retrospective cohort study, 120 patients were divided into two groups: PRC with or without primary radial styloidectomy. Patient-related outcome, strength and range of motion after proximal row carpectomy were measured in a subgroup. The occurrence of radial impingement was significantly lower in the group with primary radial styloidectomy versus those without (p = 0.002). Five patients in the latter group were subsequently treated by a secondary radial styloidectomy, as compared with one patient who underwent primary radial styloidectomy (p = 0.034). There were no significant differences in range of motion or patient-related outcome observed between the two groups. From our study, a radial styloidectomy is recommended as a routine part of the PRC procedure to prevent radial impingement without negatively impacting on function.Level of evidence: IV.

Child- and Environment-Related Factors Influencing Daily Cochlear Implant Use: A Datalog Study.

OBJECTIVES: To understand the varying levels of daily cochlear implant (CI) use in children, previous studies have investigated factors that may be of influence. The objective of this study was to investigate the degree with which new child-related and environment-related characteristics were associated with consistent CI use. DESIGN: The design of this study was retrospective. Data were reviewed of 81 children (51% females, mean age 6.4 years with a range of 1.3 to 17.7 years) who received a CI between 2012 and 2019. Developmental status, quantified burden of comorbidity, hearing experience, and hearing environment were investigated for correlation with consistency in daily CI use. The CIs datalog was used to objectively record the wearing times. Associations were examined using univariate correlation analyses and a linear regression analysis. RESULTS: On average, the CI was worn 8.6 hr per day and 59% of the children wore it more than 8 hr daily. The latter children's hearing performance was significantly higher than that of the others. Consistency in CI use correlated significantly with the child-related characteristics chronological age, nonverbal intelligence quotient (IQ), American Society of Anesthesiologists physical status class, pre CI acoustic experience, CI experience, and one of the environment related characteristics "parental communication mode." In a multivariate linear regression model, consistency in CI use was significantly dependent on nonverbal IQ and parental communication mode. These together accounted for 47% of the variation in daily CI use. CONCLUSIONS: The findings indicate that children with lower nonverbal IQ scores and low exposure to oral communication by their parents are at risk of inconsistent CI use.

Radiographic goniometry of the PIP-joint: A reliable measurement method.

INTRODUCTION: Arthrodesis of the proximal interphalangeal joint (PIP joint) is a good treatment option for relief of arthritic pain. The angle of the fused PIP joint is considered important for postoperative digital function. It is therefore important to reliably measure the actual angle of fusion. Goniometry performed by hand therapists or hand surgeons can generate measurement errors. PURPOSE OF THE STUDY: The purpose of this study is therefore to investigate the intraobserver and interobserver reliability of measuring the angle of fusion of the PIP joint on X-rays. STUDY DESIGN: A clinimetric study was conducted investigating the reliability of X-ray goniometry. METHODS: Three blinded observers measured the angle of the PIP-joint fusion on a lateral postoperative X-ray. The intraobserver and interobserver reliability was calculated. RESULTS: Ninety-four patient X-rays were measured. The intraobserver reliability showed an intraclass correlation coefficient (ICC) of 0.94, and the ICC for the interobserver reliability ranged between 0.92 and 0.95. DISCUSSION: Because this is the first reliability study on radiographic goniometry, comparison of our data with other similar studies is difficult. The best comparison can be made to in vivo goniometry. Based on the investigation of ICC values of various studies on the reliability of in vivo goniometry, radiographic goniometry seems to be more reliable. CONCLUSIONS: These results indicate an excellent reliability, which is generally higher than the reliability for in vivo goniometry of the PIP joint performed by hand therapists or hand surgeons. We can conclude that radiographic goniometry of the PIP joint is a highly reliable measurement method.

Ischemia of the fingers after carpal tunnel syndrome treatment.

We present a case in which a depot triamcinolone acetonide (Kenacort) was unintentionally injected intra-arterially into the ulnar artery, resulting in microembolic capillary occlusion in the digits supplied by the artery. Ischemic changes and subungual petechial hemorrhages were seen in the ulnar three digits. Angiography confirmed microembolic occlusion. The patient was treated with systemic vasodilative agents and a brachial plexus blockade. Tissue necrosis did not develop, however, the patient suffered lasting cold intolerance in the affected digits. Steroid suspension particles injected to treat CTS or other indications, can cause capillary occlusion and thereby microembolic tissue ischemia if injected intra-arterially. Choosing the right injection site and aspirating prior to injection is a simple though effective and indispensable measure to help prevent intra-arterial injection of steroid suspensions.

Neurosurgeons' opinions on the prenatal management of myelomeningocele.

OBJECTIVE: Improvements in imaging and surgical technological innovations have led to the increasing implementation of fetal surgical techniques. Open fetal surgery has demonstrated more favorable clinical outcomes in children born with open myelomeningocele (MMC) than those following postnatal repair. However, primarily because of maternal risks but also because of fetal risks, fetal surgery for MMC remains controversial. Here, the authors evaluated the contemporary management of MMC in the hope of identifying barriers and facilitators for neurosurgeons in providing fetal surgery for MMC. METHODS: An online survey was emailed to members of the Congress of Neurological Surgeons (CNS) and the International Society for Pediatric Neurosurgery (ISPN) in March 2019. The survey focused on 1) characteristics of the respondents, 2) the practice of counseling on and managing prenatally diagnosed MMC, and 3) barriers, facilitators, and expectations of fetal surgery for MMC. Reminders were sent to improve the response rate. RESULTS: A total of 446 respondents filled out the survey, most (59.2%) of whom specialized in pediatric neurosurgery. The respondents repaired an average of 9.6 MMC defects per year, regardless of technique. Regardless of the departments in which respondents were employed, 91.0% provided postnatal repair of MMC, 13.0% open fetal repair, and 4.9% fetoscopic repair. According to the surgeons, the most important objections to performing open fetal surgery were a lack of cases available to become proficient in the technique (33.8%), the risk of maternal complications (23.6%), and concern for fetal complications (15.2%). The most important facilitators according to advocates of prenatal closure are a decreased rate of shunt dependency (37.8%), a decreased rate of hindbrain herniation (27.0%), and an improved rate of motor function (18.9%). Of the respondents, only 16.9% agreed that open fetal surgery should be the standard of care. CONCLUSIONS: The survey results showed diversity in the management of patients with MMC. In addition, significant diversity remains regarding fetal surgery for MMC closure. Despite the apparent benefits of open fetal surgery in selected pregnancies, only a minority of centers and providers offer this technique. As a more technically demanding technique that requires multidisciplinary effort with less well-established long-term outcomes, fetoscopic surgery may face similar limited implementation, although the surgery may pose fewer maternal risks than open fetal surgery. Centralization of prenatal treatment to tertiary care referral centers, as well as the use of sophisticated training models, may help to augment the most commonly cited objection to the implementation of prenatal closure, which is the overall limited caseload.

Contemporary management and outcome of myelomeningocele: the Rotterdam experience.

OBJECTIVE: Myelomeningocele (MMC) is the most common form of spina bifida, with a lifelong impact on the quality of life for infants born with this condition. In recent decades, fetal surgery has evolved from an experimental therapy to standard of care for many centers in the world. In this study, the authors aimed to provide an overview of the current management and outcomes for infants with MMC managed at their institution. This then provides a center-specific historical cohort for comparison with future antenatal-treated MMC cases. METHODS: This is a retrospective, single-institution cohort study including all consecutive MMC cases between January 1, 2000, and June 1, 2018, at Erasmus MC. Outcome data included closure of the defect (location, timing, and surgical parameters), hydrocephalus management, Chiari malformation type II (CMTII) management, incidence of spinal cord tethering and outcome, motor outcomes, and continence. RESULTS: A total of 93 patients were included with predominantly lumbosacral lesions. Two patients died during follow-up. Hydrocephalus was present in 84%, with a 71% ventriculoperitoneal shunt reoperation rate. Surgery was performed in 12% for a tethered spinal cord at a mean age of 8 years. Decompression surgery was performed in 3 patients for CMTII. Special education in 63% was significantly associated with hydrocephalus (p < 0.015). Nineteen percent of patients were able to walk independently, and 47% were nonambulators. Social continence for urine was obtained in 75% of patients, 4% had fecal incontinence. CONCLUSIONS: This study provides an overview of current MMC outcomes at the authors' center and will serve as a historical cohort for comparison with future fetal surgery cases operated on at the center in the coming years. Apart from a relatively low surgical untethering rate, the authors' outcome data are comparable to those in the literature. Hydrocephalus is highly prevalent in postnatally treated MMC patients; in this study as in much of the literature, hydrocephalus is correlated with a low cognitive function. Fetal surgery for MMC halves the need for shunt treatment in a select group of MMC pregnancies, constituting a major indication for us to undergo the transition to a fetal surgery center. The fetal benefits of open antenatal surgery for MMC are well established, yet long-term data on especially tethered spinal cord are eagerly awaited.

Bi-allelic Loss-of-Function Mutations in the NPR-C Receptor Result in Enhanced Growth and Connective Tissue Abnormalities.

The natriuretic peptide signaling pathway has been implicated in many cellular processes, including endochondral ossification and bone growth. More precisely, different mutations in the NPR-B receptor and the CNP ligand have been identified in individuals with either short or tall stature. In this study we show that the NPR-C receptor (encoded by NPR3) is also important for the regulation of linear bone growth. We report four individuals, originating from three different families, with a phenotype characterized by tall stature, long digits, and extra epiphyses in the hands and feet. In addition, aortic dilatation was observed in two of these families. In each affected individual, we identified a bi-allelic loss-of-function mutation in NPR3. The missense mutations (c.442T>C [p.Ser148Pro] and c.1088A>T [p.Asp363Val]) resulted in intracellular retention of the NPR-C receptor and absent localization on the plasma membrane, whereas the nonsense mutation (c.1524delC [p.Tyr508∗]) resulted in nonsense-mediated mRNA decay. Biochemical analysis of plasma from two affected and unrelated individuals revealed a reduced NTproNP/NP ratio for all ligands and also high cGMP levels. These data strongly suggest a reduced clearance of natriuretic peptides by the defective NPR-C receptor and consequently increased activity of the NPR-A/B receptors. In conclusion, this study demonstrates that loss-of-function mutations in NPR3 result in increased NPR-A/B signaling activity and cause a phenotype marked by enhanced bone growth and cardiovascular abnormalities.

Complete pseudoepiphyses with associated enhanced growth in hands and feet: a report of 2 siblings--case report.

We present 2 siblings with multiple complete pseudoepiphyses in their hands and feet with associated symptomatic enhanced growth. Physical examination of the 6-year-old boy revealed long slender fingers and hyperplastic great toes. Radiography showed complete pseudoepiphyses in the first metacarpals, proximal and middle phalanges of the hands, and proximal phalanges of the feet. The patient's younger brother had a similar phenotype with slightly milder functional complaints. Genetic analysis did not reveal an underlying syndrome in these siblings.

Papilledema in patients with Apert, Crouzon, and Pfeiffer syndrome: prevalence, efficacy of treatment, and risk factors.

Patients with syndromic craniosynostosis are at risk for elevated intracranial pressure because of various physiologic and anatomic abnormalities. The aims of this study were to determine the prevalence of papilledema in syndromic craniosynostosis, to evaluate the results of the treatment, and to examine the risk factors. This is a retrospective study on 84 patients with Apert, Crouzon, or Pfeiffer syndrome. Papilledema was defined as blurring of the margins of the optic disk. The association between clinical symptoms, beaten-copper pattern on skull radiograph, ventricular dilatation on computed tomography scan, and papilledema was assessed. Papilledema was present in 51% of the patients. No relation between specific clinical symptoms and papilledema was found. The significant associations were complex craniosynostosis, exorbitism, and ventricular dilatation. The prevalence of papilledema in patients with Apert, Crouzon, or Pfeiffer syndrome is high, not only before cranial decompression but also after vault expansion. Annual fundoscopy is recommended to screen for papilledema. We consider that early decompressive surgery (within the first year of age) prevents the development of papilledema and, most likely, elevated intracranial pressure.

Condition variables in children with craniofacial anomalies: a descriptive study.

The objective of the study is to describe the prevalence of a number of condition variables and their interrelations in children with craniofacial anomalies (CFAs). The participants were 217 children with CFAs (125 boys and 92 girls), aged 5 to 16 years. The medical files and brain imaging provided information on most condition variables except for the variable phenotypical expression, on which information was obtained in a rating experiment. Brain anomalies were present in 77 subjects (36%), absent in 79 subjects (36%), and undefined in 61 subjects (28%). Craniosynostosis occurred in 160 children (74%), a syndromic diagnosis in 108 (50%), and clinical hypertelorism in 72 (33%). The mean phenotypical expression score was 4.8 (SD = 2.1), the mean number of hospitalizations was 6.2 (SD = 5.6), and mean age at craniotomy was 10.9 months (SD = 9.3). Many of the condition variables were significantly interrelated. Brain anomalies occur frequently (36%) in syndromic and nonsyndromic forms of CFAs. The most salient condition variables are the presence of brain anomalies, a syndromic diagnosis, clinical hypertelorism, a severe phenotypical expression, and female gender. Individuals presenting with one or more of these condition variables probably form the most severely affected group and require more treatment.