Richard Morton

Respiratory tract infections due to direct and reflux aspiration in children with severe neurodisability

Lower respiratory tract infections in children with severe neurodisability are usually caused by aspiration of stomach contents from gastroesophageal reflux (GOR) or direct aspiration (DA) of food due to oral and pharyngeal motor problems. To determine the contributions and interactions of GOR and DA, oesophageal 24‐hour pH monitoring and feeding videofluoroscopy were performed in 34 children (age range 7 months to 16 years, mean 7 years) who had severe physical and learning disabilities and who were slow feeders. Subjects were divided into three groups according to the frequency of their respiratory tract infections. Subjects in group 1 had no respiratory tract infections (N=10); five had GOR and none had DA. Subjects in group 2 had minor respiratory tract infections but had not received more than one course of antibiotics for this in the previous year (N=8); two had GOR alone, four had DA alone, and two had neither. All subjects in group 3 had recurrent respiratory tract infections (N=16); one had GOR alone, seven had DA alone, and eight had both GOR and DA. This study suggests that oral and pharyngeal motor problems are the major cause of respiratory tract infection in children with severe neurodisability. These problems lead to DA and, if GOR is present, to the aspiration of stomach contents. Those children with both DA and GOR are more likely to have severe respiratory tract infections which may lead to gastrostomy feeding (together with fundoplication). GOR without sufficient oral and pharyngeal motor problems to cause DA is less likely to cause respiratory tract infection in children with severe neurodisability.

Assessment of upper- limb function and movement in children with cerebral palsy wearing lycra garments

It has recently been suggested that lycra garments are helpful for children with cerebral palsy (CP). Twelve children, with athetosis, ataxia, and spasticity, were fitted with lycra garments (Kendall‐Camp UK Ltd). Scores on the Paediatric Evaluation of Disability Inventory (PEDI) scales were determined before and after wearing the garment for at least 6 hours a day for 6 weeks. Five children with motor problems representative of the whole group were investigated during a reach‐and‐grasp task by kinematic motion analysis; reflective markers were used with and without the garment. Carers were given a questionnaire concerning the practicalities of using the garments. All 12 children made improvements in at least one of the functional scales of the PEDI, and scores for the whole group showed significant gains (Wilcoxon X2 test, self‐help p<0.01; mobility p<0.5; social p<0.1). These changes were usually slight, although noticed by carers. Six children made gains of at least one scale of the caregiver assistance scores, two of the children showed losses (due to difficulties removing the garment for toileting), and four showed no change. Motion analysis indicated that (1) two children with athetosis had improved proximal stability in sitting and in smoothness of arm movements, (2) one child with ataxia had improved in proximal and distal stability, and (3) two children with spasticity had more jerky movements, although one improved in proximal stability. All children had problems in wearing the garments, including problems with toileting and incontinence of urine; the parents of only one child wanted to continue using it. Results suggest that the functional benefit of lycra garments for children with CP is mainly due to improvements in proximal stability but this should be weighed against the inconvenience and loss of independence.

Double-blind comparison study of two doses of botulinum toxin A injected into calf muscles in children with hemiplegic cerebral palsy.

Despite several trials showing reductions in tone and improvements in gait, the relation between botulinum toxin A (BTX‐A) dose and response has rarely been investigated. A double‐blind randomized comparison of two doses of BTX‐A in children with spastic hemiplegic cerebral palsy (n=48, mean age 7 years 6 months, range 3 to 15 years) was undertaken. The two doses selected were representative of the lower and the higher doses used in clinical practice (24 units/kg body weight and 8 units/kg body weight). Using gait analysis we evaluated hip, knee, and ankle joint kinetics and sagittal kinematics throughout the gait cycle. Gastrocnemius muscle length was calculated at each visit using the method described by Eames and used as our primary outcome measure. Our secondary outcome variable was maximum ankle angle measured during stance and swing phases. In summary, we found that there were indications that 24 units/kg body weight was more effective and lasted longer than 8 units/kg. Analysis in terms of absolute dose suggested that the dose‐response correlation was non‐linear, and that the optimal range lay between 200 and 500 units BTX‐A (Dysport).

Videofluoroscopy in the assessment of feeding disorders of children with neurological problems

A multidisciplinary assessment, including videofluoroscopy, was carried out on 14 children with feeding difficulties associated with neurological problems. Recommendations were made on all aspects of feeding and the trunk position was changed for half of the patients. A later interview with the parents confirmed that the recommendations had been helpful. The optimum trunk position for feeding was determined during videofluoroscopy by positioning the patient in the erect or reclined position, and also by analysis of relative difficulties during the oral and pharyngeal phases of swallowing. Those with difficulties mainly in the oral phase fed best in the reclined position; those with difficulties mainly in the pharyngeal phase fed best in the erect position. particularly if they had upper‐oesophageal sphincter spasm in association with a tonic labyrinthine reflex.

Aspiration with Dysphagia: the Interaction Between Oropharyngeal and Respiratory Impairments

AbstractIndividuals with neurodisability and dysphagia often aspirate food because of oropharyngeal impairments and poor control of respiration. This study explored the interaction between these factors in 32 participants aged 3–33 years. Each person underwent a modified barium swallow study, during which respiration was recorded and displayed simultaneously on the video screen, in terms of inspiration, expiration, and velocity of airflow (TV data). The duration of time that material remained in the pharynx before the swallow (either because of pharyngeal delay or residue from the previous swallow) was called the pharyngeal dwell time (PDT). The mean PDT of the 5 slowest swallows for each participant was calculated for both liquids and thick purees. The proportions of time spent in inspiration and expiration during the PDT in seconds and a score representing the abnormality of inspiration, including its frequency and velocity, were recorded. The volume of material in the pharynx prior to these swallows was also estimated. Twelve participants aspirated liquids and 3 of the 12 also aspirated thick purees. PDTs were longer among aspirators (6.2 s) than nonaspirators (2.4 s) when consuming liquids. Also, the percentage of the PDT spent in inspiration was greater among aspirators than nonaspirators when taking liquids (31% vs. 11%) or thick purees (35% vs. 14%). During the PDT, aspirators showed more abnormal respiratory patterns for liquids but not for purees. There were no differences in the volumes of liquid or puree in the pharynx before the swallow between aspirators and nonaspirators. A plot of the PDT against a combined respiratory impairment score (i.e., percentage of the PDT spent in inspiration and respiratory abnormality) predicted aspirators with a sensitivity of 83% and specificity of 95%. Aspiration results from oropharyngeal impairments with inadequate respiratory integration. Further research is needed to investigate whether intervention to improve respiratory control can reduce aspiration in people with dysphagia.

Feeding ability in Rett syndrome.

Feeding abilities in 20 individuals with Rett syndrome aged 1% to 33 years were investigated by history and clinical assessment during a meal, followed by videofluoroscopy of feeding. All were shown to have reduced movements of the mid and posterior tongue, with premature spillover of food and liquid from the mouth into the pharynx. They also showed delayed pharyngeal swallow, but otherwise pharyngeal problems were minimal. These findings were noted to be similar to those in Parkinsons disease. Those individuals with the most general neurological impairment tended to have the worst feeding problems and were smaller and malnourished.

Use of a lying hip abduction system in children with bilateral cerebral palsy: a pilot study

Fourteen children aged 4 to 14 years with hip subluxation due to cerebral palsy (CP) were considered for a trial in a lying hip abduction system (Jenx Dreama, Jenx Limited, Sheffield, UK). Baseline data were recorded for 6 months, then assessments of the system were made for one year at 6 and 12 months. Assessments consisted of hip radiographs in the standard position, a parental questionnaire, and a sleep chart, which was completed by parents every Friday night during the trial. Three children could not enter the trial because of general sleep problems, and three could not complete it because they were unable to sleep with the system. One further child withdrew from the study just before the end because of unacceptable deterioration for which surgery was needed. The remaining seven children completed the trial and there was an overall improvement in rate of hip migration percentage on the right from 7% per annum in the baseline period to 4% with the system (p<0.05). On the left, changes were -3% and 0% respectively (ns). Average sleep at night changed from 9 to 9.4 hours (ns) and wakenings/night from 1 to 1.3 (ns). On the parental questionnaire, there were significant improvements noted with the system in position for seating and sleeping, ease of hip abduction for washing, and with pain reduction. This pilot study supports the use of this type of lying system but further studies are needed to establish the acceptability and efficacy of these systems, particularly in children aged 2 to 5 years when irreversible bony deformities of the hip tend to occur.

Air swallowing in Rett syndrome

The possible causes of excessive swallowing of air leading to bloating, which is common in Rett syndrome (RS), were investigated during feeding and at rest. Seven individuals with RS aged between 4 and 33 years (three with air bloat) underwent feeding videoflouroscopy and concurrent respiration monitoring. The results were compared with a randomly selected group of 11 individuals, aged between 2 and 16 years, with quadriplegic cerebral palsy and feeding problems, some of whom had mild air bloat. All individuals from both groups had isolated pharyngeal swallows and several mouth breathed; this may account for some air swallowing but not the severe air bloat characteristic of RS. Thirty‐three individuals with RS aged between 3 and 44 years were monitored for nasal respiration, chest movements, swallowing, and vocal cord position at rest (between feeding). Twenty had air bloat, 17 of whom swallowed air during breath‐holding in the same way, and three gulped air during hyperventilation. Of the 13 without air bloat, eight did not have recurrent breath‐holding and five did, but without concurrent air swallowing. Several methods for reducing air swallowing in apnoea were investigated. The most successful was a dummy with an air leak, but this was poorly tolerated and could only be used for short periods of time. Apnoeas and air bloat are often worse when individuals are distressed and may in some individuals be reduced by anxiolytic medications.

Controlled Study of the Effects of Continuous Intrathecal Baclofen Infusion in Non-Ambulant Children with Cerebral Palsy.

Aim  To measure changes in children with severe spastic cerebral palsy (CP) after continuous intrathecal baclofen (ITB) infusion over 18 months and to compare the results with those of a comparison group awaiting treatment.

Dislocation of the hips in children with bilateral spastic cerebral palsy, 1985–2000

The aim of this study was to assess the rate of hip dislocation at different ages in children with bilateral spastic cerebral palsy attending special schools in southern Derbyshire, UK, between 1985 and 2000. The medical notes of 110 individuals (68 males, 42 females) were obtained. They were divided into four groups according to the Gross Motor Function Classification System (GMFCS). We determined whether or not their hips were dislocated at the ages of 5, 10, and 15 years, and the kind of surgery performed in each case. The percentage of individuals with one or both hips dislocated increased with age and with severity of disease. Of those in GMFCS Level II (n=18), none had dislocations; Level III (n=16), none had dislocations at ages 5 and 10, but 11% had by the age of 15; Level IV (n=35), 8% had dislocations by age 5, 19% by age 10, and 30% by age 15; Level V (n=41), 22% had dislocations by age 5, 48% by age 10, and 50% by age 15. Forty-two per cent of individuals with hip dislocation had not had previous preventive surgery. Twenty-one per cent of hips operated on still proceeded to dislocation. We conclude that there was a high rate of hip dislocation, especially in GMFCS groups Levels IV and V, and that this often occurred very early. Preventive surgery avoided dislocation in many children. However, orthopaedic referral was often not made before dislocation was discovered, or the referral was made too late for surgery on soft tissue to be successful. These results may be compared with those from current programmes of hip management, involving radiological surveillance and early use of conservative and surgical interventions.