Fred Molitor

A multicenter review of toxic epidermal necrolysis treated in U.S. burn centers at the end of the twentieth century.

Toxic epidermal necrolysis (TEN) is a potentially fatal disorder that involves large areas of skin desquamation. Patients with TEN are often referred to burn centers for expert wound management and comprehensive care. The purpose of this study was to define the presenting characteristics and treatment of TEN before and after admission to regional burn centers and to evaluate the efficacy of burn center treatment for this disorder. A retrospective multicenter chart review was completed for patients admitted with TEN to 15 burn centers from 1995 to 2000. Charts were reviewed for patient characteristics, non-burn hospital and burn center treatment, and outcome. A total of 199 patients were admitted. Patients had a mean age of 47 years, mean 67.7% total body surface area skin slough, and mean Acute Physiology and Chronic Health Evaluation (APACHE II) score of 10. Sixty-four patients died, for a mortality rate of 32%. Mortality increased to 51% for patients transferred to a burn center more than one week after onset of disease. Burn centers and non-burn hospitals differed in their use of enteral nutrition (70 vs 12%, respectively, P < 0.05), prophylactic antibiotics (22 vs 37.9%, P < 0.05), corticosteroid use (22 vs 51%, P < 0.05), and wound management. Age, body surface area involvement, APACHE II score, complications, and parenteral nutrition before transfer correlated with increased mortality. The treatment of TEN differs markedly between burn centers and non-burn centers. Early transport to a burn unit is warranted to improve patient outcome.

Impact of Prostheses on Function and Quality of Life for Children with Unilateral Congenital Below-the-Elbow Deficiency

BACKGROUND Children with unilateral congenital below-the-elbow deficiency present a dilemma to clinicians. Parents want the child to have a prosthesis and, because it seems that the deficiency will cause functional problems, one is customarily prescribed for infants. Use of the prosthesis is then encouraged throughout childhood. However, these children frequently abandon the prosthesis. There are no evidence-based guidelines regarding prescription of prostheses or standard methods for assessing use and function. METHODS A multicenter outcomes study was done to assess the quality of life and function of 489 children with a unilateral congenital below-the-elbow deficiency; 321 wore a prosthesis, and 168 did not. The Unilateral Below-the-Elbow Test (UBET) was designed, validated, and administered to these children along with several outcomes measures, including the Pediatric Outcomes Data Collection Instrument (PODCI), the Pediatric Quality of Life Inventory (PedsQL), and the Prosthetic Upper Extremity Functional Index (PUFI). RESULTS Use of a prosthesis was not associated with any clinically relevant differences in PODCI or PedsQL scores. Non-wearers performed either the same as or better than wearers on the UBET. When queried (with use of the PUFI) about performance of various tasks, non-wearers scored themselves higher than wearers. Children with a unilateral congenital below-the-elbow deficiency scored the same as or higher than the general population on the PedsQL. They scored significantly lower than the general population on the PODCI Upper Extremity Physical Function Domain and higher on the Happiness Domain, but the differences were small. CONCLUSIONS Prostheses may help with social acceptance or may be useful as tools for specialized activities, but they do not appear to improve function or quality of life, which are nearly normal for children with unilateral congenital below-the-elbow deficiency regardless of whether they wear a prosthesis. These findings call into question the standard practices of fitting infants with prostheses and encouraging young children to wear the prosthesis.

The Unilateral Below Elbow Test: a function test for children with unilateral congenital below elbow deficiency.

The Unilateral Below Elbow Test (UBET) was developed to evaluate function in bimanual activities for both the prosthesis wearer and non-wearer. Nine tasks were chosen for each of four age-specific categories defined by development stages of hand function (2-4y, 5-7y, 8-10y, and 11-21y). Two scales, Completion of Task and Method of Use, were designed to rate performance. To measure reliability, four occupational therapists scored samples of videotaped UBET performances. For Completion of Task, an interval scale, agreement in scoring was measured with interclass correlation coefficients (ICC; n=9; five females, four males). For Method of Use, a nominal scale, chance-adjusted association was calculated with Cohens kappa coefficients (interobserver n=198; 111 females, 87 males; intraobserver n=93; 56 females, 37 males). For Completion of Task, the average ICC was 0.87 for the prosthesis-on condition, and 0.85 for the prosthesis-off condition. ICCs exceeded 0.80 for eight out of nine tasks for the two older age groups, but for only five out of nine tasks in the younger age groups. Higher inter- and intraobserver kappa coefficients for Method of Use resulted when scoring children with their prostheses on versus off. The oldest age group had lower kappa values than the other three groups. The UBET is recommended for the functional evaluation of Completion of Task in children with unilateral congenital below elbow deficiency with and without their prostheses. Method of Use scoring can evaluate individuals for directed therapy interventions or prosthetic training.

Comparison of pediatric outcomes data collection instrument scores and range of motion before and after shoulder tendon transfers for children with brachial plexus birth palsy

Background: Children with brachial plexus birth palsy (BPBP) may undergo shoulder external rotation tendon transfers (ERTT) to improve function. In terms of outcome measurements, and according to the World Health Organization International Classification of Functioning, Disability, and Health model, ERTT reduces impairment as measured by range of motion (ROM), but has not been shown to improve activity and participation. Furthermore, correlation between these outcome measures has not been established. The Pediatric Outcomes Data Collection Instrument (PODCI) is a well-validated musculoskeletal health questionnaire that addresses activity and participation components of function. The aim of this study is to determine whether PODCI scores improve after ERTT, and whether this improvement correlates with improvement in ROM. Methods: A total of 23 children with a mean age of 6.3 years (range, 4.4-12.8 years) with BPBP and standard indications for ERTT underwent preoperative and 1-year postoperative shoulder ROM measurements, and parental completion of the PODCI. Change in ROM was compared with change in PODCI scores to determine if these were correlated. Results: Average range of active shoulder abduction improved 35 degrees (P < 0.001), and average range of active external rotation improved 41 degrees (P < 0.001). The PODCI scores for Upper Extremity Function, Sports Function, and Global Function improved (12 points [P < 0.001], 4 points [P = 0.04], and 6 points [P = 0.001], respectively). Improvement in ROM did not correlate with improvement in PODCI scores. However, postoperative peak active abduction correlated strongly with postoperative PODCI scores for Upper Extremity Function, and Global Function (rs = 0.712 [P < 0.001], rs = 0.735 [P < 0.001], respectively), and moderately with Transfers and Basic Mobility and Sports Function scores (rs = 0.496 [P=0.016], rs = 0.449 [P = 0.032], respectively). Conclusions: For children with BPBP, ERTT is associated with reduced impairment and improved activity and participation. Maximum postoperative abduction is positively associated with PODCI scores, but change in ROM is not. Further study is needed to determine if ceiling effects or other factors account for the lack of correlation between these outcome measures. Level of Evidence: Level of evidence IV, case series.

Development and pilot test of the shriners pediatric instrument for neuromuscular scoliosis (SPNS): A quality of life questionnaire for children with spinal cord injuries

Abstract Background/Objective: The purpose of this study is to report the results of rater agreement for the International Standards for Neurological Classification of Spinal Cord Injury (ISCSCI) motor and sensory scores before and after training in the testing technique. Methods: Six raters performed sequential motor and sensory examinations on 5 adolescents with SCI according to the ISCSCI manual. After completion of the first examinations, all raters were provided with a half-day formal training session on testing techniques, after which the raters repeated the examinations. lntraclass correlation coefficients (ICCs) and 95% confidence intervals (Cis) were calculated to provide parameters for ICC interpretation: > 0.90 = high agreement; 0.75 to 0.90 = moderate agreement; < 0.75 = poor agreement. Results: After training, there was improvement in rater agreement of summed motor scores (MS) from ICC = 0.809 to 0.862 and discrimination scores from ICC = 0.786 to 0.892. There was moderate rater agreement for light touch scores (LTS) before and after training. After training, there was improvement in 95% Cis except for ICCs for LTS, but for all ICCs, the lower 95% Cl value remained less than 0.75. Conclusions: Training improved rater agreement on MS and discrimination, but 95% Cis remained unacceptably wide. The positive effect of training in motor and sensory testing techniques is supported by the study data. Unlike previous studies that have suggested the ISCSCI has acceptable reliability for clinical trials, the results of this study do not fully support the use of the ISCSCI for clinical trials without better standardization to establish a lower 95% Cl value of at least 0.75.

Tendon transfer surgery in upper-extremity cerebral palsy is more effective than botulinum toxin injections or regular, ongoing therapy

BACKGROUND For children with upper-extremity cerebral palsy (CP) who meet standard indications for tendon transfer surgery, we hypothesized that surgical treatment would result in greater functional improvement than treatment with botulinum toxin injections or regular, ongoing therapy. METHODS Thirty-nine children with upper-extremity CP, who were four to sixteen years of age and surgical candidates for the transfer of the flexor carpi ulnaris to the extensor carpi radialis brevis, pronator teres release, and extensor pollicis longus rerouting with adductor pollicis release, were prospectively assigned, either randomly (twenty-nine patients) or by patient/family preference (ten patients), to one of three treatment groups: surgical treatment (Group 1); botulinum toxin injections (Group 2); or regular, ongoing therapy (Group 3). Seven centers participated. Assessment measurements included active range of motion, pinch and grip strength, stereognosis, and scores as measured with eight additional functional or patient-oriented outcome instruments. Thirty-four patients (twenty-five randomized and nine from the patient-preference arm) were evaluated twelve months post-treatment as the study cohort. RESULTS For the primary outcome of the Shriners Hospital Upper Extremity Evaluation (SHUEE) dynamic positional analysis (DPA), significantly greater improvement was seen in Group 1 than in the other two groups (p < 0.001). Improvements in SHUEE DPA reflected improved supination and wrist extension during functional activities after surgical treatment. Group 1 showed more improvement in the Pediatric Quality of Life Inventory (PedsQL) CP module domain of movement and in the Canadian Occupational Performance Measure (COPM) score for satisfaction than Groups 2 and 3. Both Groups 1 and 3 showed more improvement in pinch strength than did Group 2. CONCLUSIONS For children with upper-extremity CP who were candidates for standard tendon transfer, surgical treatment was demonstrated to provide greater improvement, of modest magnitude, than botulinum toxin injections or regular, ongoing therapy at twelve months of follow-up for the SHUEE DPA, the PedsQL CP module domain of movement, and COPM satisfaction.

Long-term functional outcomes in the elderly after burn injury

Although the elderly represent a substantial proportion of the population, limited information exists on postdischarge long-term outcomes of elderly burn survivors. The purpose of this study was to assess elderly burn patient outcomes 2 to 10 years after discharge. This study was a prospective cross-sectional survey assessment of quality of life and retrospective trauma registry for the American College of Surgeons review of patients ≥ 60 years of age discharged alive after acute burn from 1997 to 2007. In-hospital treatment and burn demographic information were obtained from database and chart review. Surviving patients or their families were contacted, and the Short-Form-12 and Functional Independence Measure (FIM) administered. Of the 344 patients discharged, 232 participated. Mean age was 72.3 (60–85.8) years, TBSA burn was 7.8% (1–79), and length of stay was 11.2 ± 0.9 days (1–51). Most patients were discharged home (71%) or to a skilled nursing facility (SNF; 20%). Mean interval between discharge and survey administration was 46.1 months. In all, 24% of patients sent home died after discharge and prior to interview compared with 58% of patients sent to an SNF. On multivariate analysis, mortality increased with age (confidence interval [CI] 1.04–1.09), and government insurance (CI 0.34–0.94), but decreased with discharge to home (CI 1.68–4.47). There were no differences in FIM or Short-Form-12 scores between groups. Long-term mortality after discharge in elderly burn survivors is substantial. Patients sent to an SNF or with government insurance had increased mortality postdischarge. These data suggest that issues that may influence disposition status of elderly burn patients should be optimized prior to discharge to mitigate adverse outcomes associated with SNF placement.

The validity of compliance monitors to assess wearing time of thoracic-lumbar-sacral orthoses in children with spinal cord injury.

Study Design. Prospective multicenter observation. Objective. To determine the validity of 3 commercially available at recording thoracic-lumbar-sacral orthosis (TLSO) wearing time of children with spinal cord injury (SCI) and to assess each monitors function during daily activities. Summary of Background Data. A major limitation to studies assessing the effectiveness of spinal prophylactic bracing is the patients compliance with the prescribed wearing time. Although some studies have begun to use objective compliance monitors, there is little documentation of the validity of the monitors during activities of daily life and no comparisons of available monitors. Methods. Fifteen children with SCI who wore a TLSO for paralytic scoliosis were observed for 4 days during their rehabilitation stay. Three compliance monitors (2 temperature and 1 pressure sensitive) were mounted onto each TLSO. Time of brace wear from the monitors was compared with the wear time per day recorded in diaries. Results. Observed versus monitored duration of brace wear found the HOBO (temperature sensitive) to be the most valid compliance monitor. The HOBO had the lowest average of difference and variance of difference scores. The correlation between the recorded daily entries and monitored brace wear time was also highest for the HOBO in analysis of dependent and independent scores. Bland-Altman plots showed that the pressure sensitive monitor underestimated wear time whereas the temperature monitors overestimated wear time. Conclusion. Compliance to prescribed wearing schedule has been a barrier to studying TLSO efficacy. All 3 monitors were found to measure TLSO compliance, but the 2 temperature monitors were more in agreement with the daily diaries. Based on its functional advantages compared with the HOBO, the StowAway TidbiT will be used to further investigate the long-term compliance of TLSO bracing in children with SCI.

Predictors of Continued Prosthetic Wear in Children With Upper Extremity Prostheses

The purpose of this study was to determine whether age at initial fit, type of prosthesis, geographic location, gender, or side of limb deficiency were associated with duration of upper extremity prosthetic wear in children. A retrospective chart review of 298 children was conducted at five Shriners Hospitals for Children to determine which factors contributed to longer duration of prosthetic wear in children who were fit with transradial prostheses between 1988 and 1998. Data analysis was performed to answer the following questions: (1) Does age at initial fit or initial type of prosthesis affect prosthetic outcome (defined as more than 3 years of wear)? (2) Does current or final type of prosthesis affect prosthetic outcome? (3) Does geographic location or distance traveled to the clinic affect prosthetic outcome? (4) Does side of limb deficiency or gender affect prosthetic outcome? Children fit with a transradial prosthesis at younger ages wore a prosthesis longer than did children fit at older ages. For current or final type of prosthesis, children who wore a body-powered or myoelectric prothesis were more than two times as likely to wear it > 3 years than were children who wore a passive prosthesis. Finally, children living in Mexico were more than three times as likely to wear a prosthesis > 3 years than were children living in other countries. Initial type of prosthesis, distance traveled to the clinic, gender, or side of limb deficiency were not correlated with duration of prosthetic wear. Age at initial fit, current or final type of prosthesis, and geographic location were predictors that correlated with longer duration of prosthetic wear in children wearing transradial prostheses.

Clavicle Fracture Is Not Predictive of the Need for Microsurgery in Brachial Plexus Birth Palsy

Background:Brachial plexus birth palsy (BPBP) and clavicle fractures occur concurrently as well as in isolation during difficult deliveries. The concept that concurrent clavicle fracture may spare nerve injury has been advocated. Our aim was to compare those children with BPBP presenting to a tertiary care center with and without concurrent clavicle fractures and assess the utility of the presence of a clavicle fracture as a predictor of injury severity in children with BPBP. Methods:Records of all patients belonging to a large prospective multicenter cohort study of infants with BPBP (Treatment and Outcomes of Brachial Plexus Injuries study) were analyzed for demographic information and birth information including risk factors, comorbidity, presence of clavicle fracture, and injury severity. Results:The records of 639 children with BPBP were examined. Thirteen patients were excluded for incomplete data. Ninety-six children who sustained concurrent birth fracture and BPBP were identified (15%). Of these, 57 sustained clavicle fractures (9%), 44 sustained humerus fractures, and 4 sustained other fractures. Of the demographic factors analyzed, only the presence of gestational diabetes was found to be significantly higher in those children with fractures versus those without. The presence of a clavicle fracture did not change the rate of microsurgical intervention, nor did clavicle fracture rates differ by Toronto score, suggesting that there was no difference in injury severity between the 2 groups. Conclusions:In a large multicenter prospective study, 9% of children presenting with BPBP also sustained a clavicle fracture at birth. The presence of a clavicle fracture did not correlate with the severity of brachial plexus injury. We suggest that in study populations of children with severe enough BPBP to present to a tertiary care center, compared with studies collected in the obstetric population, the presence of a clavicle fracture is neither protective from nerve injury nor predictive of injury severity. Level of Evidence:Level 2—prognostic.