Publications by authors named "Karen Van Hulst"

Aim: To evaluate negative effects on oral motor function after concurrent submandibular and parotid (four-gland) botulinum neurotoxin A (BoNT-A) injections as a treatment for paediatric drooling.

Method: This was a retrospective cohort study of 125 children (median age 7 years 7 months [interquartile range 4 years 5 months]) with developmental disabilities, including cerebral palsy, treated with four-gland injections. Most children (90.

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Article Synopsis
  • Anterior drooling significantly affects social interactions and emotional well-being in children and young people (CYP) with neurodevelopmental disabilities, especially those with a developmental age of 6 years and older.
  • The study analyzed parental perceptions through questionnaires, revealing that a substantial number of CYP experience frequent drooling, which impacts their social acceptance and perceived cognitive abilities.
  • The findings highlight the need for clinical care to address the social-emotional consequences of drooling, as many CYP feel dissatisfied with their appearance and struggle with acceptance by peers.
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Purpose: Quantitative muscle ultrasound (QMUS) is potentially valuable as a diagnostic tool in central neurological disorders, as it provides information about changes in muscle architecture. This study aimed to investigate whether ultrasound images of the submental and masticatory muscles in children with spastic cerebral palsy (CP) differ from those obtained in a reference group, and whether observed ultrasound abnormalities differ between subgroups of children with different Eating and Drinking Ability Classification System (EDACS) levels to support its construct validity.

Methods: A prospective cohort study was conducted in 25 children with spastic CP aged 3-18 years.

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Unlabelled: Anterior and posterior drooling are prevalent comorbidities in children with neurodevelopmental disabilities. Considering the heterogeneity of the patient population and the multifactorial aetiology of drooling, an interdisciplinary and individualised treatment approach is indispensable. However, no tool for stepwise decision-making in the treatment of paediatric drooling has been developed previously.

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Background: Despite the established efficacy of glycopyrronium bromide in reducing drooling among children with neurodevelopmental disabilities, evidence on its impact on the daily lives of children and parents and effectiveness in a real-world setting are scarce, especially among long-term users. This study explored timing and duration of glycopyrronium treatment, effect and impact on daily life, and occurrence of side effects to inform clinical practice.

Methods: This was a retrospective cohort study at a national referral centre for drooling, including 61 children with nonprogressive neurodevelopmental disabilities, treated with glycopyrronium for anterior and/or posterior drooling between 2011 and 2021.

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Introduction: Examination of oral movements is often part of an assessment undertaken by a speech and language therapist (SLT). Until now, there have been no specific instruments or tests with reference values for typically developing children in Dutch that exclusively evaluate non-speech oral movements in young children. Therefore, a non-speech oral motor observation list was designed to attempt to bridge this gap: the Non-Speech Oral Movement Assessment for Children (NOMAC).

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Aim: To develop robust multivariable prediction models for non-response to (1) submandibular botulinum neurotoxin A (BoNT-A) injections and (2) concurrent submandibular and parotid (four-gland) injections, to guide treatment decisions for drooling in children with neurodevelopmental disabilities, including cerebral palsy.

Method: This was a retrospective cohort study including 262 children (155 males/107 females, median age 7 years 11 months [IQR 5 years 1 month], range 4 years 0 months - 17 years 11 months) receiving submandibular injections and 74 children (52 males/22 females, median age 7 years 7 months [IQR 4 years 3 months], range 4 years 9 months - 18 years 8 months) receiving four-gland injections. Multivariable logistic regression analyses were used to estimate associations between candidate predictors and non-response 8 weeks after injection.

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Paediatric anterior drooling has a major impact on the daily lives of children and caregivers. Intraglandular botulinum neurotoxin type-A (BoNT-A) injections are considered an effective treatment to diminish drooling. However, there is no international consensus on which major salivary glands should be injected to obtain optimal treatment effect while minimizing the risk of side effects.

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Aim: Submandibular gland excision (SMGE) is suitable for the management of drooling in patients with non-progressive neurodisabilities. We aimed to investigate the long-term effects of SMGE.

Method: Patients who had SMGE between 2007 and 2018 were included.

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Article Synopsis
  • The study evaluated the effectiveness of two-duct ligation (2-DL) for managing drooling in patients with nonprogressive neurodisabilities over a period of 1 to 12 years.
  • Results showed a significant initial improvement in drooling severity, but some patients reported a return to higher levels of severity over time, with 33% needing additional treatment.
  • Despite some recurrence, a majority (64%) of patients indicated they would recommend 2-DL to others, highlighting perceived long-term benefits.
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Aim: To assess the dysphagia limit in children with cerebral palsy (CP) according to Eating and Drinking Ability Classification System (EDACS) level, sex, and age compared to typically developing children.

Method: Seventy-seven children with CP (54 males, 23 females; mean age 7y 6mo, SD 2y 2mo, age range 4-12y) were assessed with the Maximum Volume Water Swallow Test. Median dysphagia limit in the CP group was compared with data of typically developing children.

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SATB2-associated syndrome (SAS) is a neurodevelopmental disorder caused by heterozygous pathogenic variants in the SATB2 gene, and is typically characterized by intellectual disability and severely impaired communication skills. The goal of this study was to contribute to the understanding of speech and language impairments in SAS, in the context of general developmental skills and cognitive and adaptive functioning. We performed detailed oral motor, speech and language profiling in combination with neuropsychological assessments in 23 individuals with a molecularly confirmed SAS diagnosis: 11 primarily verbal individuals and 12 primarily nonverbal individuals, independent of their ages.

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Aim: To compare the effect of bilateral submandibular duct ligation and botulinum neurotoxin A (BoNT-A) on drooling severity and its impact on daily life and care in children and adolescents with moderate-to-severe drooling.

Method: This was a randomized, interventional, controlled trial in which 53 children and adolescents (31 males, 22 females, mean age 11y, range 8-22y, SD 2y 10mo) with cerebral palsy (58.5%) or other non-progressive developmental disorders (41.

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Aim: To evaluate the effect of botulinum neurotoxin A (BoNT-A) injections, submandibular gland excision (SMGE), and bilateral submandibular duct ligation (2DL) for the control of posterior drooling in children with neurological impairment.

Method: In a retrospective cohort, children with neurological impairment (e.g.

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Aim: To evaluate the effect of repeated onabotulinum neurotoxin A injections for the treatment of drooling in children with neurodisabilities.

Method: This was a retrospective cohort study, in which the first, second, and third onabotulinum neurotoxin A injection were compared within children treated between 2000 and 2020. Primary outcomes included drooling quotient, visual analogue scale (VAS), and treatment success defined as ≥50% reduction in drooling quotient and/or VAS 8 weeks after treatment.

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Aim: To assess: (1) the effect on drooling of bilateral submandibular duct ligation as surgical therapy after the administration of submandibular botulinum neurotoxin A (BoNT-A) for excessive drooling and (2) the predictive value of treatment success with BoNT-A on treatment success after bilateral submandibular duct ligation.

Method: This was a within-participant retrospective observational study in which 29 children with severe drooling (15 males, 14 females) received BoNT-A treatment at a mean age of 9 years 6 months (SD 2y 5mo), followed by bilateral submandibular duct ligation at a mean age of 10 years 11 months (SD 2y 4mo). Fifteen children were diagnosed with cerebral palsy (CP), with 12 children classified in Gross Motor Function Classification System levels IV and V.

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Background: Drooling is dependent on various clinical variables. However, while drooling proves refractory to two-duct ligation in 40% of patients, predictors for treatment success are sparse and to date there is little evidence why some respond well while others are non-responders. We aim to find predictors for treatment success and study the effectiveness of two-duct ligation for drooling in neurodisabilities.

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Aims: To examine changes in objective and subjective drooling severity measures after submandibular botulinum neurotoxin A injection in children with neurodevelopmental disabilities, explore their relationship, and evaluate if clinically relevant responses relate to changes in the impact of drooling.

Method: This longitudinal, observational cohort study involved 160 children (92 males, 68 females; 3-17y, mean 9y 1mo, SD 3y 6mo) treated between 2000 and 2012 at the Radboud University Medical Center, Nijmegen, the Netherlands. Repeated measures analysis of variance was used to compare the 5-minute Drooling Quotient (DQ5) and Visual Analogue Scale (VAS) for drooling severity pretreatment and posttreatment, and Pearson's rho to assess their association.

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Objectives: To evaluate if drooling recurrence after surgery of the submandibular ducts is due to surgical failure or other variables.

Methods: Historic cohort with prospective collected data of all patients with severe drooling who underwent unsuccessful submandibular duct surgery with subsequent re-intervention between 2003 and 2018. A reference cohort was used for comparison of clinical variables.

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Aim: This study evaluated whether the effect of submandibular gland botulinum neurotoxin A (BoNT-A) injection can predict the outcome of submandibular duct relocation with sublingual gland excision (SMDR) in children with drooling. Furthermore, we compared the effectiveness of both procedures.

Method: A retrospective cohort study was performed in 42 children and adolescents (25 males, 17 females; mean [SD] age at BoNT-A injection 11y [4], range 4-20y; mean [SD] age at SMDR 15y [4], range 7-23y) with cerebral palsy or another non-progressive developmental disability who had undergone both BoNT-A injection and SMDR for drooling.

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Objective: To compare the effect of submandibular duct ligation (2-DL) and submandibular botulinum neurotoxin type A (BoNT-A) for drooling in children and adolescents with neurodevelopmental disabilities.

Methods: A randomized, interventional, controlled, and partly single-blinded study was performed in which submandibular BoNT-A was compared with 2-DL to treat excessive drooling. Main outcomes included a Visual Analog Scale (VAS), drooling quotient (DQ), drooling severity (DS) scale and drooling frequency (DF) scale.

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Purpose: To assess the interrater reliability, construct validity and usability of the Eating and Drinking Ability Classification System (EDACS) among Dutch children with Cerebral Palsy (CP) when used by speech and language therapists (SLTs) familiar and unfamiliar with the child's eating and drinking performance and parents.

Methods: Translation was undertaken using the method of Eremenco. Agreement between SLTs and parents when using EDACS was determined by intraclass correlation coefficient (ICC) and linear weighted Kappa (κW).

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Objective: To evaluate the effectiveness of submandibular duct relocation (SMDR) in drooling children with neurological disorders.

Design: Prospective cohort study.

Setting: Academic Outpatient Saliva Control Clinic.

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Communication disorder is common in Koolen de Vries syndrome (KdVS), yet its specific symptomatology has not been examined, limiting prognostic counselling and application of targeted therapies. Here we examine the communication phenotype associated with KdVS. Twenty-nine participants (12 males, 4 with KANSL1 variants, 25 with 17q21.

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Aim: The aims of this study were: (1) to determine the incidence and nature of adverse effects on oral motor function after first injections of botulinum neurotoxin A (BoNT-A) in submandibular glands for excessive drooling in children with central nervous system disorders; and (2) to identify independent predictors of these adverse effects.

Method: A cohort study involved 209 children (123 males, 86 females, aged 4-27y, median 8y 4mo), who received submandibular BoNT-A injections for drooling. Adverse effects were categorized into swallowing, eating, drinking, articulation, and other problems.

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