Quality of life and developmental disabilities.
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Child↗
Biomedical subjects
J F McLaughlin
Publications and source records attributed to J F McLaughlin.
Selective dorsal rhizotomy: efficacy and safety in an investigator-masked randomized clinical trial.
The objective of this single-center investigator-masked randomized clinical trial was to investigate the efficacy and safety of selective dorsal rhizotomy (SDR) in children with spastic diplegia. Forty-three children with spastic diplegia were randomly assigned on an intention-to-treat basis to receive SDR plus physical therapy (PT), or PT alone. Thirty-eight children completed follow-up through 24 months. Twenty-one children received SDR (SDR+PT group) and 17 received PT (PT Only group). SDR was guided with electrophysiological monitoring and performed by one experienced neurosurgeon. All subjects received equivalent PT. Spasticity was quantified with an electromechanical torque measurement device (spasticity measurement system [SMS]). The Gross Motor Function Measure (GMFM) was used to document changes in functional mobility. Primary outcome measures were collected at baseline, 6, 12, and 24 months by evaluators masked to treatment. At 24 months, the SDR+PT group exceeded the PT Only group in mean reduction of spasticity by SMS measurement (-8.2 versus +5.1 newton meters/radian, P=0.02). The SDR+PT group and the PT Only group demonstrated similar improvements in independent mobility on the GMFM (7.0 versus 7.2 total percent score, P=0.94). Outcomes on secondary variables were consistent with primary outcomes. There were no serious adverse events. We conclude that SDR is safe and reduces spasticity in children with spastic diplegia. SDR plus PT and equivalent PT without SDR result in equal improvements in independent mobility at 24 months. SDR may not be an efficacious treatment for children with mild spastic diplegia.
Adolescent↗
Electrophysiological monitoring during selective dorsal rhizotomy, and spasticity and GMFM performance.
The relation between abnormal electrophysiological responses to intraoperative stimulation during selective dorsal rhizotomy (SDR) and the degree of spasticity and motor dysfunction was explored in 92 children with spastic cerebral palsy (CP) who underwent SDR at a single center. The proportion of abnormally responding rootlets was compared with the degree of spasticity measured with the modified Ashworth Scale (MAS) and with the spasticity measurement system (SMS) at discrete segmental levels. Motor impairment measured with the Gross Motor Function Measure (GMFM) was also compared with the proportion of abnormally responding dorsal rootlets. A consistent relation between the proportion of abnormally responding rootlets and the degree of spasticity and gross motor abnormality at the corresponding muscles could not be demonstrated. There was also no consistent association between the proportion of rootlets ablated during SDR and the change in spasticity measured with the MAS and SMS, or to the change in motor function as measured with the GMFM. These data suggest that the intraoperative monitoring technique most commonly used for SDR is unlikely to identify accurately those neural elements which contribute to spasticity in children with CP.
Adolescent↗
Vertebral column and spinal cord malformation in children with exstrophy of the cloaca, with emphasis on their functional correlates.
Exstrophy of the cloaca is a dramatic malformation whose embryology is poorly understood. While the management of this disorder has received significant attention in the urology and general surgery literature, the neurologic status of these children has been poorly addressed. In order to better characterize the spinal cord and vertebral column malformations found in children with exstrophy of the cloaca, we undertook a clinical review of 26 consecutive children with exstrophy of the cloaca who had been seen at a single institution over 28 years. The prevalence of vertebral malformations in the 25 children who could be evaluated was 25/25 (100%). Twenty (80%) of the children had at least one vertebral fusion, most frequently at T-7. Twenty-two (88%) of the children had at least one vertebra with deficient posterior elements, and the spinal levels most frequently involved were S-2, S-3, S-4 and S-5. Nine (36%) of the children had at least one vertebra with a narrowed interpedicular distance, most frequently at T-7. Nine (36%) of the children had at least one vertebra with atrophic facet anatomy, most frequently at L-3. The prevalence of myelodysplasia in the 19 children for whom spinal magnetic resonance imaging or intraoperative findings were available was 100%. Of these 19 children, 15 (79%) had myelocystocele, 2 (11%) had a lipomeningocele, 2 (11%) had a meningocele, 2 (11%) had hydromyelia, and 4 (21%) had a tethered cord. These data suggest that spinal cord and vertebral column malformations are very common in children with exstrophy of the cloaca.
Abnormalities, Multiple↗
Acquired hydrocephalus associated with superior vena cava syndrome in infants.
We report observations on children with the unusual combination of superior vena cava syndrome in infancy followed by communicating hydrocephalus. Following retrospective review of hospital discharges at a tertiary children's hospital, three children were identified in a 13-year period. Two term infants were treated with extracorporeal membrane oxygenation for pulmonary failure associated with congenital diaphragmatic hernia. These infants had septic complications of central venous lines. A post-term infant required reconstruction of the superior vena cava following cannulation for cardiac bypass surgery to repair transposition of the great vessels. Thrombosis occurred and was followed by the sequential development of superior vena cava syndrome and communicating hydrocephalus. The findings in these patients suggest that communicating hydrocephalus may be caused by superior vena cava syndrome. This is an unusual complication of therapeutic manipulation of the heart and great veins. Cerebrospinal fluid shunting may be required.
Extracorporeal Membrane Oxygenation↗
Exotic perspectives and salient cases.
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Alaska↗
Marinesco-Sjögren syndrome: clinical and magnetic resonance imaging features in three children.
The authors describe clinical and MRI features of a girl and two boys, aged 9, 17 and 19 years, respectively, with Marinesco-Sjögren syndrome. The clinical findings included the major features of the syndrome, including growth deficiency, ataxia, cataracts, hypogonadism (in two) and seizures (in two). Truncal hypotonia (in three), microcephaly (in two) and leg spasticity (in two) were also present. MRI showed a very small cerebellar vermis in all three patients, various supratentorial abnormalities, an apparently small anterior pituitary gland in two and the absence of a posterior pituitary gland in all three. The MRI features are similar to the few reported pathologic findings for persons with Marinesco-Sjögren syndrome. MRI may be helpful in the early diagnosis of the disorder.
Adolescent↗
Marinesco-Sjögren syndrome: clinical and magnetic resonance imaging features in three children.
The authors describe clinical and MRI features of a girl and two boys, aged 9, 17 and 19 years, respectively, with Marinesco-Sjögren syndrome. The clinical findings included the major features of the syndrome, including growth deficiency, ataxia, cataracts, hypogonadism (in two) and seizures (in two). Truncal hypotonia (in three), microcephaly (in two) and leg spasticity (in two) were also present. MRI showed a very small cerebellar vermis in all three patients, various supratentorial abnormalities, an apparently small anterior pituitary gland in two and the absence of a posterior pituitary gland in all three. The MRI features are similar to the few reported pathologic findings for persons with Marinesco-Sjögren syndrome. MRI may be helpful in the early diagnosis of the disorder.
Adolescent↗
Clean intermittent catheterization.
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Humans↗
Lessons from clinical research: adverse events or complications?
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Adverse Drug Reaction Reporting Systems↗
Pain management for children following selective dorsal rhizotomy.
Selective dorsal rhizotomy (SDR) is a neurosurgical procedure used for treating lower extremity spasticity in patients with cerebral palsy. The purpose of this paper is to present a review of our institution's first three years' experience with postoperative pain and spasticity management in patients who have undergone SDR. The medical records of the 55 patients who had an SDR during the study period were reviewed. The basis of postoperative analgesia was morphine, with the majority of patients receiving continuous morphine infusions (20-40 micrograms.kg-1.hr-1 (n = 49), 60 micrograms.kg-1.hr-1 (n = 1)). Four patients used a patient-controlled delivery system. One patient had successful analgesia with epidural morphine. Ketorolac (1 mg.kg-1 i.v. loading dose followed by 0.5 mg.kg-1 i.v. every six hr for 48 hr) was used as an adjunct to morphine in six patients. For management of postoperative muscle spasm, an intravenous benzodiazepine was used (diazepam 0.1 mg.kg-1 (n = 2), or midazolam infusion 10-30 micrograms.kg-1.hr-1 (n = 51)). All patients were cared for on a ward where nurses were familiar with the use of continuous opioid and benzodiazepine infusions. All patients received continuous cardiorespiratory monitoring as well as frequent nursing assessment. There were no episodes of postoperative apnoea or excessive sedation. We have found the use of continuous infusions of morphine and midazolam, along with adjunct ketorolac, to be effective in treating postoperative pain and muscle spasms following SDR.
Adolescent↗
The role of selective dorsal rhizotomy in cerebral palsy: critical evaluation of a prospective clinical series.
This is a prospective observational study of a consecutive series of 34 children with spastic cerebral palsy treated at a single center. 10 had spastic quadriplegia and 24 had spastic diplegia. All were followed for at least one year. After selective dorsal rhizotomy (SDR), all children received one month of physical therapy at the center and were prescribed a program of physical therapy in their community. The children were assessed before and one year after SDR and physical therapy, using the Ashworth Scale, deep tendon reflex response, range of motion and the Gross Motor Function Measure. The results show that there is often a decrease in lower-extremity spasticity and functional improvement after SDR with physical therapy, but that there is considerable variability in outcome. Randomized prospective clinical trials with masked objective outcome measures are needed to determine the efficacy of SDR.
Cerebral Palsy↗
Prospective measurement of functional changes after selective dorsal rhizotomy.
Selective dorsal rhizotomy (SDR) followed by intensive postoperative therapy is commonly performed to reduce spasticity in carefully selected children with cerebral palsy. Reduction in spasticity and improvement in range and quality of movements are frequently reported. Functional gains have also been indicated but have not often been examined in a systematic manner. Twenty-nine children with spastic diplegia or quadriplegia received SDR and postoperative physical and occupational therapy. Upper-limb movement, self-care, and functional mobility were evaluated prospectively through preoperative, 6-month, and 12-month follow-up examinations. Significant improvements in the Pediatric Evaluation of Disability Inventory scores for functional mobility and self-care domains were observed for children with spastic diplegia, but not spastic quadriplegia. Children's scores in upper-limb reach and coordination tasks did not consistently improve. When attributing improvements in self-care and mobility independence to SDR and specialized therapy, continued development, new goals, and positive beliefs about progress should also be considered as factors influencing outcome.
Activities of Daily Living↗
Mechanical efficiency of children with spastic cerebral palsy.
In this feasibility study, the authors compared the mechanical efficiency of nine children with spastic diplegia with that of nine able-bodied children, matched for gender. They also compared resting energy expenditure in the two groups. The children with CP had significant reductions in mechanical efficiency at maximum stable work-load and marginally significant reductions when they were compared at 200 kg-m/min work-load. The efficiency of able-bodied children at 200 kg-m/min was unexpectedly reduced. There were no differences in resting energy expenditure. Mechanical efficiency may be a useful objective technique to assess the effects of interventions intended to improve the efficiency of the movement of children with CP.
Biomechanical Phenomena↗
The promise of independence.
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Activities of Daily Living↗
Positional asphyxia in individuals with severe cerebral palsy.
The authors report the accidental death by positional asphyxia of three individuals with cerebral palsy occurring after they had been placed in bed for the night. During the five-year period between 1984 and 1989, 12 per cent of deaths from positional asphyxia in King County, Washington, involved individuals with cerebral palsy. Such accidents are similar to those occurring among healthy young children, perhaps because both groups have limited motor skills. Further research into accidents among disabled persons may clarify their special risks and possibly lead to modifications in bed design.
Accidents, Home↗
Severe gastric distension in seven patients with cerebral palsy.
This is a report of two children who had severe recurrent gastric distension and vomiting, and five who experienced severe gastric distension without vomiting. Two of the five died during an episode of acute gastric distension. All had marked nutritional depletion, and severe spastic quadriplegia due to either cerebral palsy or acquired brain injury. None of the patients had significant gastroesophageal reflux. Positioning the patients in the left lateral decubitus position temporarily relieved their obstructions. Complete resolution of the distension and/or vomiting did not occur until after adequate weight gain. Loss of fat stores may lead to this type of recurrent gastric distension.
Adolescent↗
Quantitative measurement of spasticity in children with cerebral palsy.
Spasticity was quantified in nine children with spastic diplegia, using a sinusoidal displacement of the foot at frequencies from 3 to 12Hz. Ankle-joint stiffness was separated into elastic (energy-storing) and viscous (energy-dissipating) components. 'Path length' was used to represent the variation in stiffness over this frequency range. Compared with 11 unaffected children, a significant difference in path lengths was demonstrated for the children with spasticity. An age-dependent effect was demonstrated when path lengths of unaffected children were compared with those of 10 unaffected adults. A modified path-length measure is proposed which minimizes age dependency, yet enables detection of spasticity. Passive stiffness properties of unaffected adults showed higher elastic stiffness, viscosity and friction than unaffected children. A method was developed to evade the need for temporary nerve blocks to calculate inerital properties of the foot in persons with spasticity.
Biomechanical Phenomena↗