The WeeFIM [R] instrument--a paediatric measure of functional independence to predict longitudinal recovery of paediatric burn patients.

INTRODUCTION: Burns create a myriad of complications that affect the child's developmental, functional and aesthetic status. The WeeFIM is a standardized measure of functional performance developed for use in children 6-months to 8-years of age but with application through adolescence. It includes 18 domains of performance which are scored on a 7-point scale from 'total assistance' to 'complete independence'. In this study, the WeeFIM was used to evaluate the influence of burn size on functional independence and on time to recovery. METHODS: Children, 6 months to 16 years of age, with total body surface area (TBSA) bums of 10-100% burn injury were recruited for a 2-year longitudinal study. Due to unstable WeeFIM measurements on children 6 months to 6 years, analyses on normalized WeeFIM scores among subjects 6-16 years are presented. Children were evaluated at discharge from acute care, 6 months, 1 year and 2 years after burn injury. FINDINGS: In this analysis, 454 WeeFIM evaluations from 249 patients, 6-16 years of age, were reviewed. While mean WeeFIM scores varied significantly at discharge based on the size of burn, there were no significant differences in any of the WeeFIM scales at 24 months post-burn. At 24 months, the mean WeeFIM score for all children, independent of size of their bum, indicated full independence. Hands-on assistance was not required for performing activities of daily living (ADLs). The rates of improvement differed statistically by size of bum. Maximum improvement was attained by 6 months for 10-15% TBSA burns, 12 months for 16-30% burns, 12 months for 31-50% burns and 24 months for 51-100% TBSA. CONCLUSION: The WeeFIM can be utilized by burn centres to describe diminished functional capacity at discharge from acute care for severely burnt children. The tool can be used to track return to baseline independence after a major burn injury in a paediatric population.

Acute transverse myelitis in childhood: center-based analysis of 47 cases.

OBJECTIVE: To relate clinical characteristics associated with acute transverse myelitis (ATM) in children with functional outcomes at follow-up. METHODS: We identified 47 patients for whom ATM occurred under the age of 18 years. Chart analysis, clinical evaluation, and administration of functional measures were completed. RESULTS: The age at onset clustered between ages 0 to 2 and 5 to 17. Febrile illness had occurred in 47% and vaccination in 28%. Major disability at the nadir of the clinical course was noted. Eighty-nine percent were unable to walk, required assisted ventilation, or both. At a median of 3.2 years after acute illness, 43% were unable to walk 30 ft and 21% required a walker or other support, 68% experienced urinary urgency, 50% required bladder catheterization, 54% were troubled by persistent dysesthesias, and 75% had numbness. Factors associated with a better functional outcome included older age at time of diagnosis, shorter time to diagnosis, lower sensory and anatomic levels of spinal injury, absence of T1 hypointensity on spinal MRI obtained during the acute period, lack of white blood cells in the CSF, and fewer affected spinal cord segments. Neither rapid progression to maximum impairment in less than 1 day nor any antecedent illness, immunization, or trauma was associated with a worse outcome. CONCLUSION: Persisting disability was present in many children with acute transverse myelitis. Urinary problems and sensory symptoms were the most common issues. Age at onset below 3 years was associated with worse functional outcomes.

The rehabilitation/school matrix: a model for accommodating the noncompliant child with severe burns.

Some children with severe burns may have difficulty following therapeutic recommendations after discharge from a burn center. Noncompliance may result in complications that affect function, surgical management, community reintegration, and successful reentry into school. We present a case study in which a child with significant compliance issues was managed in a coordinated interdisciplinary model. This model extended from acute recovery through reintegration into the classroom. A behavior-modification program was implemented across different levels of care. Educational reentry was facilitated by including a transitional period in a special-needs classroom in a freestanding special-needs school. Requirements for using educational resources to which some children are legally entitled are reviewed. Rehabilitation services that interface with the educational system for children with burns may improve outcomes as these children reintegrate into the community.

Age-dependent effects of trihexyphenidyl in extrapyramidal cerebral palsy.

Trihexyphenidyl (Artane) is a centrally active muscarinic antagonist commonly used to treat patients with generalized dystonia. In a retrospective survey of 22 consecutive children with extrapyramidal cerebral palsy, we evaluated trihexyphenidyl on upper extremity and lower extremity function, expressive language, and drooling. Functional changes were assessed using a parental questionnaire (rating scale 1-5: from 1 = little or no change to 5 = tremendous change, with scores in either a positive or negative direction). Improvements of +4 or +5 were reported in eight children for upper extremity function, in eight children for verbal expressive language, in five for drooling, and in none for lower extremity function. Using bivariate linear regression modeling to investigate variables associated with treatment effects, there was a significant inverse relationship between age at initiation of medication and therapeutic response. Furthermore, beneficial responses were specific to upper-extremity function and expressive language. These results suggest that younger children are more likely to respond to trihexyphenidyl and that primary functional benefits include improved fine motor abilities and expressive language. A prospective masked study with a standardized clinical instrument is needed to confirm these findings.

Trihexyphenidyl in posthemorrhagic dystonia: motor and language effects.

Trihexyphenidyl has been found to be an effective treatment for dystonic movement disorders, improving gross motor function in patients with axial and torsional dystonia, tremors, and myoclonus. In this report, improvements in fine motor control, language, and oral motor skills are described with trihexyphenidyl in an 8-year-old female who developed dystonia after spontaneous bilateral putamenal hemorrhages. No adverse side effects occurred. The mechanism of action of trihexyphenidyl is believed to be in the basal ganglia where it inhibits muscarinic cholinergic receptors and increases the turnover of dopamine.

Late onset of syringomyelia after traumatic brain injury: association with Chiari 1 malformation.

Syringomyelia should be suspected when progressive weakness or sensory loss develops. It most commonly occurs as a congenital malformation but has also been associated with spinal cord injury and the Chiari malformation. Symptomatic Chiari malformation after head injury is rare and usually presents with signs of acutely increased intracranial pressure. This article describes syringomyelia as a late onset complication of head injury in the presence of an underlying Chiari malformation. A possible causal relationship between head injury, the brain malformation, and an extensive syrinx is postulated.

Early transfer to a rehabilitation hospital for infants with chronic bronchopulmonary dysplasia.

Shortly after being weaned off the respirator, 43 infants with severe chronic bronchopulmonary dysplasia (BPD) were transferred from an intensive-care nursery at a teaching hospital to an affiliated children's rehabilitation hospital in a program that included special staff instruction. Morbidity, measured by rate of transfer back to the acute-care hospital, was lower than in a comparison group of 15 infants treated for severe BPD during the previous two years. Average length of stay was significantly shortened and an average of $60,000 per patient was saved. Using a rehabilitation hospital as a step-down unit shifts the emphasis from acute needs to chronic and developmental needs and from intensive monitoring and nursing care to care given at home by parents with nursing assistance.

Neurodevelopment of preterm infants: neonatal neurosonographic and serum bilirubin studies.

In this study of 249 preterm infants of less than 34 weeks' gestation, the relationships between maximal serum total bilirubin concentrations during the neonatal period, neonatal cranial ultrasonographic abnormalities, and severe neurodevelopmental sequelae are described. The subjects, who were selected on the basis of serial cranial ultrasonographic findings, had repeated neurologic and developmental examinations during late infancy and early childhood that established the presence (n = 45) or absence (n = 204) of spastic forms of cerebral palsy. Of the 204 subjects without cerebral palsy, 23 scored abnormally low on standardized developmental testing during early childhood. All but seven of the subjects with cerebral palsy had grade III/IV intracranial hemorrhage or moderate to severe periventricular echogenicity or both, ultrasonographic abnormalities that probably reflect a disruption in the blood-brain barrier as well as extravasation of blood into brain tissue; however, analysis of the data did not suggest that these cranial ultrasonographic abnormalities increased either the maximum serum bilirubin concentration during the neonatal period or the susceptibility of the subjects to neurologic damage from hyperbilirubinemia. Also, there was no evidence to suggest that bilirubinemia in the range studied (2.3 to 22.5 mg/100 mL total serum bilirubin) was causally related to cerebral palsy, early developmental delay, or the development of periventricular cysts in this population of preterm infants.(ABSTRACT TRUNCATED AT 250 WORDS)

Neurosonographic features of periventricular echodensities associated with cerebral palsy in preterm infants.

We studied the value of neonatal neurosonograms in preterm infants for predicting the development of cerebral palsy (CP). All infants born at less than 33 weeks of gestation who were admitted to the intensive care nursery of Thomas Jefferson University Hospital from 1982 to 1986 were serially studied with cranial ultrasound methods that reliably detect neonatal periventricular echodensities (PVE) and cysts that are 2 mm in diameter or larger. PVE were graded as mild or as moderate to severe, and cyst size was classified by widest diameter as either large (greater than or equal to 3 mm) or small (less than 3 mm). All 127 surviving infants with PVE or cyst formation or both were followed until spastic forms of CP could be diagnosed or excluded during late infancy. All 26 of the infants in whom spastic CP developed had moderate or severe PVE in the area superior and lateral to the caudothalamic notch as noted on the parasagittal images of the neonatal neurosonograms. All these infants also developed cysts in the periventricular region within the area of the previously noted PVE. Mild or moderate to severe PVE were not associated with the development of spastic CP in 101 infants. Cysts developed in the area of PVE in the neurosonographic studies of 42 of these 101 infants. Mild PVE without cysts and moderate to severe PVE without cysts had negative predictive values for CP of 69% and 76%, respectively. By contrast, the presence of moderate to severe PVEs with large cyst formation had positive and negative predictive values of 90% and 93%, respectively, and was the most sensitive and specific neurosonographic finding for predicting CP, with an efficiency of 92%. The presence or absence of intracranial hemorrhage did not increase the efficiency of the ultrasound test results.