Changes in severity and impact of drooling after submandibular gland botulinum neurotoxin A injections in children with neurodevelopmental disabilities.

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. A parent questionnaire was used to assess drooling impact in responders (defined as ≥50% reduction in DQ5 and/or ≥2 SD reduction in VAS for drooling severity 8wks postintervention) and non-responders. RESULTS: One hundred and twelve children (70%) were responders. Their mean VAS for drooling severity and DQ5 scores were significantly lower 32 weeks postintervention compared to baseline. At baseline, the VAS for drooling severity-DQ5 relationship was 'weak' (rs =0.15, p=0.060), whereas it was 'fair' at 8 weeks (rs =0.43, p=0.000) and 32 weeks (rs =0.30, p=0.000). For responders, a significant change was found regarding the impact of drooling on daily care and social interactions at 8 weeks after intervention; most of these effects were maintained at 32 weeks. INTERPRETATION: A clinically relevant response based on a combination of objective and subjective measures of drooling severity was accompanied by positive changes regarding the impact of drooling on daily care and social interactions. WHAT THIS PAPER ADDS: Botulinum neurotoxin A injection into the submandibular glands reduced drooling severity in 70% of children with neurodevelopmental disabilities. Objective (5-minute Drooling Quotient) and subjective (Visual Analogue Scale for drooling severity) measures correlated 8 and 32 weeks after treatment. Objective and subjective measures complemented each other when changes in drooling severity were assessed. Reduced drooling severity was accompanied by positive changes with regard to the impact of drooling.

Submandibular gland botulinum neurotoxin A injection for predicting the outcome of submandibular duct relocation in drooling: a retrospective cohort study.

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. Main outcomes were the drooling quotient and the visual analogue scale (VAS) on drooling severity at 8 weeks and 32 weeks follow-up. RESULTS: Failure or success of previous BoNT-A injections had no influence on success of consecutive SMDR. Relative change in main outcomes showed no significant relation between BoNT-A injection and SMDR for any follow-up measurement. After 8 weeks, SMDR was more successful than BoNT-A injection in diminishing VAS (VAS 80.0% vs 54.3%; drooling quotient 56.2% vs 51.0%). After 32 weeks, both drooling quotient (64.3% vs 29.5%) and VAS (75.7% vs 37.1%) showed significantly higher proportions of success for SMDR. INTERPRETATION: The effect of submandibular BoNT-A injection does not predict subsequent SMDR success in drooling. Furthermore, SMDR has a larger and longer-lasting positive effect on drooling than BoNT-A injections. WHAT THIS PAPER ADDS: Submandibular botulinum neurotoxin A (BoNT-A) injection effect does not predict submandibular duct relocation with sublingual gland excision outcome. Submandibular duct relocation is more effective and more permanent than BoNT-A injection.

INYECCIÓN DE NEUROTOXINA BOTULÍNICA A EN LA GLÁNDULA SUBMANDIBULAR PARA PREDECIR EL RESULTADO DE LA REUBICACIÓN DEL CONDUCTO SUBMANDIBULAR EN BABEO: UN ESTUDIO DE COHORTE RETROSPECTIVO: OBJETIVO: Este estudio evaluó si el efecto de la inyección de neurotoxina A botulínica submandibular (BoNT-A) puede predecir el resultado de la reubicación del conducto submandibular con escisión de la glándula sublingual (SMDR) en niños con babeo. Además, comparamos la efectividad de ambos procedimientos. MÉTODO: Se realizó un estudio de cohorte retrospectivo en 42 niños y adolescentes (25 varones, 17 mujeres; edad media [DE] en la inyección de BoNT-A 11 años [4], rango 4-20 años; edad media [SD] a SMDR 15 años [4] rango 7-23 años) con parálisis cerebral u otra discapacidad del desarrollo no progresiva que se haya sometido a una inyección de BoNT-A y SMDR para babear. Los resultados principales fueron el cociente de babeo y la escala analógica visual (VAS) en la severidad del babeo a las 8 semanas y 32 semanas de seguimiento. RESULTADOS: El fracaso o el éxito de las inyecciones anteriores de BoNT-A no tuvo influencia en el éxito de la SMDR consecutiva. El cambio relativo en los resultados principales no mostró una relación significativa entre la inyección de BoNT-A y SMDR para cualquier medición de seguimiento. Después de 8 semanas, SMDR fue más exitoso que la inyección de BoNT-A en la VAS disminuida (VAS 80,0% vs 54,3%; cociente de babeo 56,2% vs 51,0%). Después de 32 semanas, tanto el cociente de babeo (64,3% vs 29,5%) como el VAS (75,7% vs 37,1%) mostraron proporciones significativamente más altas de éxito para SMDR. INTERPRETACIÓN: El efecto de la inyección submandibular de BoNT-A no predice el éxito posterior de SMDR en el babeo. Además, el SMDR tiene un efecto positivo mayor y más duradero en el babeo que las inyecciones de BoNT-A.

INJEÇÃO DE NEUROTOXINA BOTULÍNICA NA GLÂNDULA SUBMANDIBULAR PARA PREDIÇÃO DO RESULTADO DA RELOCACÃO DO DUCTO SUBMANDIBULAR NA SIALORRÉIA: UM ESTUDO DE COORTE RETROSPECTIVO: OBJETIVO: Este estudo avaliou se o efeito da neurotoxina botulínica A (NTBo-A) na glândula submandibular pode predizer o resultado da relocação do ducto submandibular com excisão sublingual da glândula (RDSM) em crianças com sialorréia. Ainda, comparamos a efetividade de ambos os procedimentos. MÉTODO: Um estudo de coorte retrospectivo foi realizado em 42 crianças e adolescentes (25 do sexo masculino, 17 do sexo feminino; idade média [DP] no momento da injeção de NTBo-A 11a[4], variação de 4-20a; idade média [DP] no momento da RDSM 15a [4], variação de 7-23a) com paralisia cerebral ou outra desordem não-progressiva do desenvolvimento que passaram por injeção de NTBo-A e RDSM para sialorréia. Os principais desfechos foram o quociente de sialorréia e a escala visual análoga (EVA) sobre a severidade da sialorréia no acompanhamento de 8 e 32 semanas. RESULTADOS: A falha ou sucesso da NTBo-A prévia não teve influência no sucesso da RDSM consecutiva. A mudança relativa nos principais desfechos não mostrou nenhuma relação significativa entre a injeção de NTBo-A e a RDSM para nenhuma das medidas no acompanhamento. Após 8 semanas, a RDSM foi mais bem sucedida do que a NTBo-A na redução da EVA (EVA 80,0% vs 54,3%; quociente de sialorréia 56,2% vs 51,0%). Após 32 semanas, tanto o quociente de sialorréia (64,3% vs 29,5%) quanto a EVA (75,7% vs 37,1%) mostram proporções significativamente mais altas de sucesso para a RDSM. INTERPRETAÇÃO: O efeito da injeção submandibular de NTBo-A não prediz o sucesso da RDSM subsequente na sialorréia. Ainda, a RDSM tem efeito maior e mais duradouro na sialorréia do que injeções de NTBo-A.

Accurate assessment of drooling severity with the 5-minute drooling quotient in children with developmental disabilities.

AIM: The aims of this study were to examine whether objective measurements of the 10-minute drooling quotient (DQ10) and the 5-minute drooling quotient (DQ5) are interchangeable; to assess agreement between the measurements and their accuracy in classifying drooling severity; and to develop a time-efficient clinical assessment. METHOD: The study cohort included 162 children (61 females, 101 males; mean age 11 y 6 mo, SD 4 y 5 mo, range 3 y 9 mo-22 y 1 mo) suffering from moderate to profuse drooling. One hundred and twenty-four had cerebral palsy and 38 had other developmental disabilities. Seventy-four of the participants were ambulant and 88 non-ambulant. The original DQ10 was recalculated into a 5-minute score (DQ5). Assessments were undertaken while the participants were in a rest situation (DQ(R)) and while they were active (DQ(A)). Agreement in scores was quantified using intraclass correlations and Bland-Altman plots. To classify drooling, area under the receiver operating characteristic curve analysis was used to compare accuracy of the DQ10 and DQ5 at rest and during activity. RESULTS: Agreement between DQ10A, and DQ5(A), and between DQ10(R) and DQ5(R) was high (intraclass correlation coefficient >0.90). Moderate agreement existed between DQ(A) and DQ(R). DQ(A) scores were more accurate in classifying children's drooling behaviour. For DQ5(A), a cut-off point of 18 or more (drooling episodes/observation time) might indicate 'constant drooling'. INTERPRETATION: The DQ10 and DQ5 can be used interchangeably. DQ(A) is most discriminative for drooling severity. For evaluating treatment efficiency the cut-off point can be used. For clinical and research purposes, the DQ5 is time efficient and cost saving while validity, and intrarater and interrater reliability are preserved.

Clinical practice: swallowing problems in cerebral palsy.

Cerebral palsy (CP) is the most common physical disability in early childhood. The worldwide prevalence of CP is approximately 2-2.5 per 1,000 live births. It has been clinically defined as a group of motor, cognitive, and perceptive impairments secondary to a non-progressive defect or lesion of the developing brain. Children with CP can have swallowing problems with severe drooling as one of the consequences. Malnutrition and recurrent aspiration pneumonia can increase the risk of morbidity and mortality. Early attention should be given to dysphagia and excessive drooling and their substantial contribution to the burden of a child with CP and his/her family. This review displays the important functional and anatomical issues related to swallowing problems in children with CP based on relevant literature and expert opinion. Furthermore, based on our experience, we describe a plan for approach of investigation and treatment of swallowing problems in cerebral palsy.

Thickened saliva after effective management of drooling with botulinum toxin A.

AIM: The aim of this study was to evaluate the rheological properties of saliva after submandibular botulinum toxin type A (BoNT-A) injections. METHOD: We enrolled 15 children (11 males and six females; age range 3-17 y, mean age 9 y 10 mo) diagnosed with spastic (n=9) or dyskinetic (n=6) quadriplegic cerebral palsy (CP); Gross Motor Function Classification System level IV or V; and two children with intellectual disability (IQ<70) who experienced moderate to severe drooling. Salivary flow rate and drooling quotient were measured at baseline and at different times after BoNT-A injections up to 24 weeks. The mucin concentration of saliva was analysed before and after BoNT-A treatment. RESULTS: Both submandibular salivary flow rate (baseline 0.38 mL/min; 24 wks after injection 0.26 mL/min) and drooling quotient (baseline 42.5%; 24 wks 28.80%) were substantially reduced, with a concomitant increase in mucin concentration within 8 weeks after BoNT-A injection (from 0.612 to 1.830 U/mL). The parents of nine children observed thickened saliva. Swallowing and chewing were problematic in seven children. Two of these children needed treatment with mucolytics because of pooling of thickened saliva in the throat. INTERPRETATION: When making decisions about the use of BoNT-A, the risk of problems with masticatory and swallowing functions as a result of thickening of saliva after BoNT-A treatment should be taken into account.

Botulinum toxin versus submandibular duct relocation for severe drooling.

AIM: Botulinum neurotoxin type A (BoNT-A) has been described as an effective intervention for drooling and is being increasingly adopted. However, its effectiveness compared with established treatments is still unknown. We undertook a within-participants observational study to examine this. METHOD: An historic cohort was formed of 19 children and young adults (10 males, nine females) with severe drooling who underwent BoNT-A injections followed by surgical re-routing of the submandibular duct at least 6 months later. Mean age at time of admission was 11 years 5 months (range 5-17 y) and mean age at the time of surgery was 14 years (range 6-23 y). Fifteen children were diagnosed with bilateral cerebral palsy (CP), three with unilateral CP, and one with non-progressive developmental delay. Gross Motor Function Classification System levels were the following: level I, n=1; level II, n=2; level III, n=7; level IV, n=6; and level V, n=3). The primary outcome was the drooling quotient, which was assessed before each intervention and 8 and 32 weeks thereafter. A multivariate analysis of variance of repeated measures was performed, with the measurement points as the within-participant variables. RESULTS: The drooling quotient was reduced to a greater extent after surgery than after BoNT-A administration (p=0.001). Compared with a baseline value of 28, the mean drooling quotient 8 weeks after surgery was 10, and 32 weeks after surgery was 4 (p<0.001). Among the group treated with BoNT-A, the drooling quotient showed a significant reduction from a baseline value of 30 to 18 after 8 weeks (p=0.02), and a continued but diminished effect after 32 weeks (drooling quotient 22; p=0.05). INTERPRETATION: Both interventions are effective, but surgery provides a larger and longer-lasting effect.

Drooling in cerebral palsy: hypersalivation or dysfunctional oral motor control?

AIM: To investigate whether drooling in children with cerebral palsy (CP) in general and in CP subtypes is due to hypersalivation. METHOD: Saliva was collected from 61 healthy children (30 males, mean age 9y 5mo [SD 11mo]; 31 females, mean age 9y 6mo [1y 2mo]) and 100 children with CP who drooled (57 males, mean age 9y 5mo [3y 11mo], range 3-19y; 43 females, mean age 10y 1mo [4y 9mo], range 4-19y), of whom 53 had spastic, 42 had dyskinetic, and five had ataxic CP. Almost all children were affected bilaterally, and 90 of them were at Gross Motor Function Classification System levels III or higher. The saliva was collected by the swab saliva collection method. The intensity of drooling was evaluated using the drooling quotient. RESULTS: No difference was found in the flow rates, age, or sex between healthy children and children with CP who drooled. On additional subgroup analysis, the flow rates of children with dyskinetic CP differed statistically from those of healthy children (submandibular p=0.047, parotid p=0.040). INTERPRETATION: This study supports the finding in previous studies that no hypersalivation exists in children with CP who drool. Dysfunctional oral motor control seems to be responsible for saliva overflow from the mouth, whereas increased unstimulated salivary flow may occur in children with dyskinetic CP as a result of hyperkinetic oral movements.

Validity and reliability of the VOAA-DDD to assess spontaneous hand use with a video observation tool in children with spastic unilateral cerebral palsy.

BACKGROUND: In 2003 new computer software, the VOAA (Video Observations Aarts and Aarts), was designed to score and evaluate two important aspects of spontaneous upper limb use, i.e. overall duration and frequency of specific behaviours. The aim of this study was to investigate the test-retest, interrater and intrarater reliability and the construct validity of a new module, the VOAA-DDD, to determine developmental disregard in children with spastic unilateral cerebral palsy (CP). METHODS: A test-retest design with three raters for reliability and a two-group design for construct validity were used. Subjects were a total of 20 children with spastic unilateral CP equally divided in two age groups (2.5-5 and 5-8 years), and 56 healthy children of the same age groups. Overall duration and frequency of specific behaviours of the affected arm and hand were assessed during a task demanding ('stringing beads') and a task stimulating ('decorating a muffin') the use of both hands. Reliability was estimated by intraclass correlation coefficients (ICCs). Construct validity was assessed by comparing children with CP to healthy children. RESULTS: All ICCs exceeded 0.87. In contrast with healthy children, children with CP used their affected hand less during the 'muffin' task compared to the 'beads' task. Of the children with CP, 90% in the age group of 2.5-5 years and 50% in the age group of 5-8 years showed values exceeding the extreme values of healthy controls, respectively, indicating developmental disregard. CONCLUSION: The VOAA-DDD is a reliable and valid instrument to assess spontaneous use of the affected arm and hand in order to determine developmental disregard in children with spastic unilateral CP.

Physical therapy treatment in children with cerebral palsy after single-event multilevel surgery: a qualitative systematic review. A first step towards a clinical guideline for physical therapy after single-event multilevel surgery.

BACKGROUND: The aim of this study was to review available evidence for physical therapy treatment (PTT) after single-event multilevel surgery (SEMLS), and to realize a first step towards an accurate and clinical guideline for developing effective PTT for children with cerebral palsy (CP) after SEMLS. METHODS: A qualitative systematic review (PubMed, Medline, Embase, CINAHL, and the Cochrane Library) investigating a program of PTT after SEMLS in children aged 4-18 years with CP classified by Gross Motor Function Classification System level I-III. RESULTS: Six articles meeting the inclusion criteria were selected. The selected studies provide only incomplete descriptions of interventions, and show no consensus regarding PTT after SEMLS. Neither do they show any consensus on the outcome measures or measuring instruments. CONCLUSIONS: Based on the results of this literature review in combination with our best practice, we propose a preliminary protocol of PTT after SEMLS.

Generalization and maintenance of a self-management program for drooling in children with neurodevelopmental disabilities: A second case series.

In this case series (n = 10) with a non-concurrent multiple baseline design, a self-management program was shown to be effective during inpatient training in eight participants with oral-motor problems and normal intelligence or mild intellectual disabilities. They were taught to perform a self-management routine and to remain dry for increasing time intervals. In addition, the program provided differential (self-) reinforcement of swallowing, controlling and wiping behavior, explicit formulation of motivational factors, instruction and feedback for parents and teachers, and continued practice after discharge. Generalization of the initial effect to the school setting was demonstrated in seven participants. Follow-up assessments demonstrated maintenance of positive results in four children up to 24 weeks, and for two other participants until 6 weeks after discharge. These results appeared to be relevant to parents and teachers. Teaching parents and teachers to implement the procedure enables them to support the child without professional supervision in the case of relapse.