Shriners Hospitals for Children - Spokane

CONTEXT: Information on the use of oral bisphosphonate agents to treat pediatric osteogenesis imperfecta (OI) is limited. OBJECTIVE: The objective of the investigation was to study the efficacy and safety of daily oral alendronate (ALN) in children with OI. DESIGN AND PARTICIPANTS: We conducted a multicenter, double-blind, randomized, placebo-controlled study. One hundred thirty-nine children (aged 4-19 yr) with type I, III, or IV OI were randomized to either placebo (n = 30) or ALN (n = 109) for 2 yr. ALN doses were 5 mg/d in children less than 40 kg and 10 mg/d for those 40 kg and greater. MAIN OUTCOME MEASURES: Spine areal bone mineral density (BMD) z-score, urinary N-telopeptide of collagen type I, extremity fracture incidence, vertebral area, iliac cortical width, bone pain, physical activity, and safety parameters were measured. RESULTS: ALN increased spine areal BMD by 51% vs. a 12% increase with placebo (P < 0.001); the mean spine areal BMD z-score increased significantly from -4.6 to -3.3 (P < 0.001) with ALN, whereas the change in the placebo group (from -4.6 to -4.5) was insignificant. Urinary N-telopeptide of collagen type I decreased by 62% in the ALN-treated group, compared with 32% with placebo (P < 0.001). Long-bone fracture incidence, average midline vertebral height, iliac cortical width, bone pain, and physical activity were similar between groups. The incidences of clinical and laboratory adverse experiences were also similar between the treatment and placebo groups. CONCLUSIONS: Oral ALN for 2 yr in pediatric patients with OI significantly decreased bone turnover and increased spine areal BMD but was not associated with improved fracture outcomes.

PURPOSE: Asparaginase therapy is an important component in the treatment of children with acute lymphoblastic leukemia. Polyethylene glycol-conjugated asparaginase (PEG-ASNase) has significant pharmacological advantages over native Escherichia coli asparaginase. We investigated the pharmacokinetics of PEG-ASNase, presence of antibodies to PEG-ASNase, and concentrations of asparagine in serum and cerebrospinal fluid (CSF) in combination chemotherapy for relapsed pediatric acute lymphoblastic leukemia. EXPERIMENTAL DESIGN: Twenty-eight pediatric patients with relapsed medullary (n = 16) and extramedullary (n = 11) acute lymphoblastic leukemia were enrolled at three pediatric institutions and had at least two serum and CSF samples obtained for analysis. Patients received induction therapy (including PEG-ASNase 2500 IU/m2 intramuscularly weekly on days 2, 9, 16, and 23) and intensification therapy (including PEG-ASNase 2500 IU/m2 intramuscularly once on day 7). Serum samples were obtained weekly during induction and intensification. CSF samples were obtained during therapeutic lumbar punctures during induction and intensification. RESULTS: Weekly PEG-ASNase therapy resulted in PEG-ASNase activity of >0.1 IU/ml in 91-100% of patients throughout induction. During intensification, PEG-ASNase on day 7 resulted in PEG-ASNase activity >0.1 IU/ml in 94% and 80% of patients on days 14 and 21, respectively. Serum and CSF asparagine depletion was observed and maintained during induction and intensification in the majority of samples. PEG-ASNase antibody was observed in only 3 patients. CONCLUSIONS: Intensive PEG-ASNase therapy in the treatment of relapsed acute lymphoblastic leukemia reliably results in high-level serum PEG-ASNase activity, and asparagine depletion in serum and CSF is usually achieved. Incorporation of intensive PEG-ASNase in future trials for recurrent acute lymphoblastic leukemia is warranted.

This study set out to determine whether cumulative radiograph exposure of children significantly increases their risk of radiation-induced carcinogenesis or hereditary defects. Records of children treated for idiopathic scoliosis, hip dysplasia, or leg-length discrepancy between 1980 and 1993 at the Shriners Hospital in Spokane, WA, were retrospectively reviewed. Total radiation and organ dose exposures were calculated using information from individual radiology reports. Surgically treated idiopathic scoliosis patients had the largest total radiation skin entrance and organ dose exposures. This group's risks for developing leukemia, breast cancer, or a heritable defect, respectively, were 0.8%, 2.1%, and 3.0% higher than baseline risks. The other treatment groups had increased carcinogenic risks of <1%. The use of serial radiographs during the treatment of idiopathic scoliosis, hip dysplasia, and leg-length discrepancy appears relatively safe. The increased risk of carcinogenesis or hereditary defects in these patients is minimal.

The question addressed in this study was whether static measurements of hip, knee, and ankle range of motion correlate to dynamic measurements of hip and knee function during gait. Range-of-motion measures of the lower extremities taken during physical examination (static variables) were recorded on 80 adolescents with cerebral palsy and 30 adolescent normal controls. Kinematic measurements collected during gait analysis (dynamic variables) were recorded on the same patients and controls. Results indicated no correlation greater than r = 0.50 (R2 < 0.25) between any static and dynamic variable for either group--cerebral palsy patients or controls. The lack of good correlation of these measures indicates static physical examination variables such as popliteal angle and straight-leg raise are not good predictors of dynamic gait, such as knee-extension and hip-flexion variables measured during ambulation in controls or cerebral palsy populations.

BACKGROUND: Hamstring lengthening procedures are commonly performed on children with cerebral palsy (CP) to improve gait. The purpose of this study was to determine the efficacy of percutaneous hamstring tenotomy surgery for children with ambulatory CP. METHODS: In this retrospective study, subjects were included if they had a diagnosis of CP and had computerized gait analysis data collected before and after surgery. Subjects were not included in the study if they had any open hamstring lengthening on the same side. Other concomitant lower extremity surgeries were not exclusionary. Short- and long-term follow-up groups were established: if the time from their surgery to their gait laboratory was less than 18 months, they were placed in the short-term follow-up group, and if the time from their surgery to their gait laboratory was greater than 18 months, they were placed in the long-term follow-up group. RESULTS: The results demonstrated that for short- and long-term groups on preoperative to postoperative analysis, there was significantly improved knee extension at initial contact, increased velocity, increased stride length, improved overall gait as indicated by a decrease in a 16 variable multivariate index (Gillette Gait Index), and a decreased popliteal angle. For the short-term group only, additional significant findings included increased peak knee extension in stance and reduced plantar flexion at initial contact. The absolute values of peak knee extension in stance and plantar flexion at initial contact were equivalent at follow-up for the short- and long-term groups. Increased anterior pelvic tilt was also significant for the short-term follow-up group only. CONCLUSIONS: The findings of this study demonstrate that the minimally invasive technique of percutaneous hamstring tenotomy is effective in improving key dynamic gait parameters for individuals with CP for a short period, and these benefits are maintained in the long term. LEVEL OF EVIDENCE: Level IV.

BACKGROUND: Torsional deformities of the lower extremity are common in children and are often corrected with rotational osteotomy. The effects of torsional abnormalities, and the effects of corrective osteotomy, are not well understood. A study of children with isolated idiopathic tibial torsional pathology undergoing a single corrective procedure may assist in understanding the biomechanics of torsional deformities and the effect of surgical correction. METHODS: Preoperative and postoperative gait analyses were performed for eight subjects (eleven sides) with idiopathic excessive inward tibial torsion and ten subjects (fourteen sides) with excessive outward tibial torsion. Sagittal ankle and frontal knee moments were assessed and compared with those for age-matched controls. RESULTS: Preoperatively, subjects exhibited abnormal frontal knee moments at push-off. Subjects with inward tibial torsion demonstrated excessive internal valgus moments, and subjects with outward tibial torsion demonstrated reduced internal valgus or relative internal varus moments compared with the control subjects. Ankle power was significantly reduced in the inward torsion group but not in the outward torsion group. Surgical correction of the torsional deformities normalized frontal plane knee moments in both inward and outward torsion groups and restored ankle power in the inward torsion group. CONCLUSIONS: In the present study, excessive tibial torsion adversely affected frontal knee moments and was associated with other kinematic and kinetic abnormalities. Corrective osteotomies improved all variables studied here and restored many to the values found in the control group.

BACKGROUND: Most patients with idiopathic clubfeet require a percutaneous tendoachilles tenotomy to correct residual equinus deformity. This procedure is typically performed with the child awake in an outpatient setting. Percutaneous tendoachilles tenotomy under general anesthesia offers the potential advantages of better pain control, the ability to perform the procedure in a more controlled manner, and the possibility of lessening the pain response of the infant. Potential disadvantages include concerns regarding the safety of general anesthesia in infants. The purpose of this study is to review the safety of this procedure performed in the operating room under general anesthesia. METHODS: A retrospective review was carried out of patients with idiopathic clubfoot less than 1 year of age who underwent percutaneous tendoachilles tenotomy under general anesthesia from 2000 to 2008. Patient medical records were reviewed for gestational age, age at surgery, risk factors for anesthesia, and surgical/anesthesia-related complications. To be discharged on the day of surgery, patients met the accepted criteria. Children at risk for apnea were considered for overnight observation using established criteria of postconception age under 44 weeks, premature birth, pulmonary comorbidities, and history of an apneic event. RESULTS: One hundred and thirty-seven patients underwent a total of 182 tenotomies under general anesthesia. Ninety-two tenotomies were unilateral, 45 were bilateral. The average postconception age at time of surgery was 53.9 weeks (range, 41 to 90 wk, SD 9.8 wk). Eighty-nine patients were under 3 months of age. Twenty-one patients (15.3%) met the criteria for the observation for postoperative monitoring for apnea because of postconception age under 44 weeks or gestational age under 37 weeks. Three patients were admitted overnight because of a maternal history of drug abuse. No patients had earlier apneic events or were American Society of Anesthesiologists Class III for comorbidities. No patient showed apnea or anesthesia-related complications. CONCLUSIONS: Percutaneous tendoachilles tenotomy under general anesthesia can be safely performed in infants with clubfeet. No complications related to anesthesia were identified in this group and nearly all patients were discharged on the day of surgery. LEVEL OF EVIDENCE: Prognostic level 3.

BACKGROUND: Ambulatory children with cerebral palsy (CP) often present with multiple deviations in all planes including increased internal hip rotation during gait. Excessive femoral anteversion is a common cause of deviation managed surgically with an external femoral derotational osteotomy (FDO). The purpose of this study was to evaluate the gait and functional outcomes of a group of subjects with CP who underwent surgical intervention that included an FDO compared with a match group with indications of internal hip rotation that did not receive an FDO. METHODS: For this retrospective study, subjects were identified from the Motion Analysis Laboratory database that had orthopaedic surgery including an FDO (FDO group). A control group was established from a chart review identifying subjects that had indications for an FDO, but did not have this surgery (No-FDO group). All subjects had preoperative and postoperative gait studies. Subjects categorized as Gross Motor Function Classification System (GMFCS) levels I and II in both FDO and No-FDO groups were combined for analysis. Subjects rated as GMFCS level III were analyzed separately. Preoperative to postoperative kinematic and kinetic variables, Gait Deviation Index, net oxygen cost, and PODCI scores were analyzed with paired t tests. RESULTS: Typical sagittal plane kinematic variables improved significantly by equivalent magnitudes for both FDO and No-FDO groups (GMFCS I/II and III). Transverse plane improvements were only seen for the FDO group (GMFCS I/II and III). The Gait Deviation Index, an overall index of kinematics, improved by a significantly greater amount for the FDO group across GMFCS levels I/II and III. Net oxygen cost improved for both FDO and No-FDO for GMFCS I/II. PODCI scores improved for FDO and No-FDO in GMFCS I/II, but only the FDO group for GMFCS III. CONCLUSIONS: For children with CP, inclusion of an FDO in the surgical intervention, when indicated, resulted in improved outcomes. Overall gait kinematic improvements were significantly greater when an FDO was included in the surgical management. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Patients with "idiopathic-like" spinal deformities associated with syringomyelia were retrospectively reviewed. Ten patients had surgical stabilization of their curvatures with at least a 2-year follow-up, and an additional five patients were evaluated for deformity pattern with <2 years of follow-up. Paralytic curve patterns, scoliosis associated with spina bifida, congenital scoliosis, or other associated syndromes were discarded. All 10 patients with surgery who were followed for an average of 46 months lost 10 degrees correction above, through, or below the instrumented segments. A total of 50% lost correction through the instrumented segments. Anterior fusion stabilized the instrumented portion of the spine better than posterior instrumentation alone. Eighty percent of the 15 patients had thoracic kyphosis >40 degrees. Only one patient was lordoscoliotic. Syringomyelia deformities tend to be kyphoscoliotic in 80% of cases and behave more like paralytic curvatures postoperatively. MRI is recommended for apparent idiopathic scoliotic curvatures that are kyphoscoliotic and not lordoscoliotic.

Back pain in children has long been considered a serious condition with an organic cause. The staff of the Shriner's Hospital had been informally noting that diagnoses among children with back pain had been changing. We conducted a retrospective study of 648 patients with spinal disorders treated at the Shriner's Hospital for Crippled Children, Spokane Unit, between January 1991 and June 1993. The patients were divided into two groups: those with pain and those without pain. It was found that back pain with no organic cause was the primary diagnosis in 57% of the group having pain. Only one child had a diagnosis of malignancy, and there were no cases of active infection. In the group with pain, three interesting associations were found concerning psychosocial problems, disability, and litigation. It was found that the diagnoses in children with back pain treated at this referral center during the study period parallel those in the adult population with back pain.

This study conceptually and empirically examined two hope scales with a community-based sample of older adults. These findings suggest that the two hope scales used in this study measure Global Hope and Interactive Hope. However, these results also suggest unique characteristics of hope that should be incorporated into future studies of hope with older adults.

Most patients (95%) with fibular hemimelia have an absent anterior cruciate ligament (ACL). The purpose of this study was to assess the long-term outcome of such patients with respect to pain and knee function. We performed a retrospective review of patients with fibular hemimelia and associated ACL deficiency previously treated at our institution. Of a possible 66 patients, 23 were sent the Musculoskeletal Outcomes Data Evaluation and Management System (MODEMS) questionnaire and Lysholm knee score to complete. In all, 11 patients completed the MODEMS and nine completed the Lysholm score questionnaire. Their mean age was 37 years (27 to 57) at review. Five patients had undergone an ipsilateral Symes amputation. There was no significant difference in any subsections of the Short-Form 36 scores of our patients compared with age-matched controls. The mean Lysholm knee score was 90.2 (82 to 100). A slight limp was reported in six patients. No patients had episodes of locking of the knee or required a supportive device for walking. Four had occasional instability with sporting activities. These results suggest that patients with fibular hemimelia and ACL deficiency can live active lives with a similar health status to age-matched controls.

Three patients (ages 12, 13, and 15 years) had histologically verified osteoid osteomas of the spine (specifically in the facet processes of L3, L2, and C5) which were localized preoperatively with nuclear scintigraphy utilizing technetium 99m diphosphonate. Intraoperative nuclear scintigraphy utilizing the mobile gamma camera was essential in both localizing the tumor during surgical excision and ensuring adequacy of excision. The authors strongly recommend intraoperative scintigraphy, and en bloc resection rather than shelling out and curettage. All three patients required facet process excision for successful tumor removal, and therefore required local fusion with internal fixation.

AIM: To understand the long-term effects of comprehensive spasticity treatment, including selective dorsal rhizotomy (SDR), on individuals with spastic cerebral palsy. METHOD: This was a pre-registered, multicenter, retrospectively matched cohort study. Children were matched on age range and spasticity at baseline. Children at one center underwent spasticity treatment including SDR (Yes-SDR, n=35) and antispastic injections. Children at two other centers had no SDR (No-SDR, n=40 total) and limited antispastic injections. All underwent subsequent orthopedic treatment. Participants returned for comprehensive long-term assessment (age ≥21y, follow-up ≥10y). Assessment included spasticity, contracture, bony alignment, strength, gait, walking energy, function, pain, stiffness, participation, and quality of life. RESULTS: Spasticity was effectively reduced at long-term assessment in the Yes-SDR group and was unchanged in the No-SDR group. There were no meaningful differences between the groups in any measure except the Gait Deviation Index (Yes-SDR + 11 vs No-SDR + 5) and walking speed (Yes-SDR unchanged, No-SDR declined 25%). The Yes-SDR group underwent more subsequent orthopedic surgery (11.9 vs 9.7 per individual) and antispastic injections to the lower limbs (14.4 vs <3, by design). INTERPRETATION: Untreated spasticity does not cause meaningful impairments in young adulthood at the level of pathophysiology, function, or quality of life.

Jacobs, Joshua J. MD; King, Toby R.W. CAE; Klippel, John H. MD; Berven, Sigurd H. MD; Burr, David B. PhD; Caskey, Paul M. MD; Elderkin, Ann L. PA; Esposito, Paul W. MD; Gall, Eric P. MD; Goldring, Steven R. MD; Pollak, Andrew N. MD; Sandborg, Christy I. MD; Templeton, Kimberly J. MDAuthor Information

This study was designed to determine the incidence of clubfoot in our fibular hemimelia population. A retrospective review of fibular hemimelia patients treated at our institution over the past seventy years was conducted. We identified twenty-three cases of clubfoot in 121 patients with 147 involved limbs. Nineteen of twenty-three limbs retained the foot, four were converted to Syme's amputation because a plantigrade functional foot could not be obtained. Hindfoot coalitions were present in eighteen limbs and nineteen had one or more missing lateral rays. In summary, the association of clubfoot deformity with fibular hemimelia is more common than previously reported. It was not always evident to the surgeon treating these patients that fibular hemimelia syndrome was present in addition to clubfoot deformity. The presence of a coalition is a relatively constant finding in this condition and should be anticipated by the treating clubfoot surgeon.

With the hope that we could handle such a situation better next time, we interviewed, with the help of an interpreter, members of 13 Hmong families from five clans, now resettled around Spokane, Washington. To learn how to incorporate their beliefs and practices into nursing care, we talked about how the Hmong define health and illness, and their views on health care. We also talked with people who have helped the refugees get settled here. The Hmong, which means free men, are from the mountainous area of Laos. They worked closely with the The strong beliefs and

Context Early single-sport specialization and the relative age effect are often cited as improving the chances of sport success. Both concepts suggest that genetics and the environment have little influence on sport success. Objective To compare National Collegiate Athletic Association Division I student-athletes (SAs) with their undergraduate nonathlete peers (NAs) in terms of birth month, age of sport initiation, and age of single-sport specialization. A family history of sport participation was examined as a potential marker for genetic and social influences. Design Cross-sectional survey. Setting Large urban university. Patients or Other Participants A total of 273 Division I SAs (138 women, 135 men) and 155 NAs (78 women, 77 men) participated. The NAs had been involved in competitive youth sports before entering the university. Main Outcome Measure(s) Participants were asked to complete a questionnaire that addressed the age of sport initiation, birth month, age of single-sport specialization, and parental and sibling sport achievement. Main Results Neither birth month nor the age of sport initiation differed between groups (age of sport initiation = 7.16 ± 2.6 years for the SAs versus 7.71 ± 3.5 for the NAs; P = .176). A larger proportion of SAs began participating before 10 years of age (80% versus 63%; P = .02). The parents of SAs were more likely to have participated in collegiate (32.4% versus 8.4%; P &amp;lt; .0001) and professional (10.9% versus 1.3%; P = .0005) sports. The SAs specialized in a single sport at an older age (15.38 ± 2.7 years versus 14.30 ± 2.6 years; P = .002). Both groups participated in multiple sports in childhood (SAs = 3.9 ± 1.8 sports, NAs = 3.2 ± 1.8 sports; P = .366). Conclusions The Division I SAs did not specialize in a single sport at a younger age than the NAs. No evidence of a relative age effect was present. Importantly, higher levels of sport achievement among the parents and siblings of SAs suggest that genetic endowment and family or other environmental dynamics play a large role in athletic performance. Overall, the results are not consistent with deliberate practice theory and point toward an alternative model that includes not only sport-specific skill development but also genetic and social factors as key elements of long-term sport achievement.

A pouch protruding from the free wall of the left ventricle may be either a congenital ventricular diverticulum (CVD) or aneurysm (CVA). Being aware of these rare congenital anomalies is critical in making the diagnosis. Differentiating the two is important for treatment decisions. We describe a patient with dextrocardia, Tetralogy of Fallot, and a congenital left ventricular apical diverticulum diagnosed following the induction of anesthesia. CVD and CVA may present in the antenatal period through late adulthood with differing morphology, location, and symptoms. Echocardiography is paramount in the diagnosis and characterization of these lesions. If this anomaly is encountered after the induction of anesthesia or during intraoperative echocardiography, the cardiothoracic anesthesiologist should make the surgical team aware so it can be further characterized and a treatment plan made prior to incision.

This paper describes seven persons in a family affected with an autosomal dominant syndrome of short stature with mesomelic shortness of upper and lower limbs, abnormal carpal and tarsal bones, hypoplastic or absent middle phalanges of hands and feet, and delayed coalescence of bipartite calcanei. All affected relatives are of normal intelligence, are free of eye problems, and have a normal skull, spine, shoulders, and hips. The digits of the hands and feet are short, broad, and angulated. The hypoplastic or absent middle phalanges effectively result in one interphalangeal joint for each digit, with decreased mobility. The bones of the carpus and tarsus coalesce with increasing age. None of the previously described syndromes or brachydactylies encompasses the findings noted in this kindred.