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 treatment of idiopathic toe walking in children can include surgical lengthening of the gastrocnemius/soleus complex after conservative options have been ineffective. Previous outcome reports of surgery for idiopathic toe walkers have largely been limited to assessing the sagittal plane motion of dorsiflexion/plantar flexion with minimal quantitative preoperative and postoperative analysis. The purpose of this study was to comprehensively assess the outcome of idiopathic toe walkers that had been treated surgically. Fourteen children seen in our motion analysis laboratory that underwent gastrocnemius or tendo-Achilles lengthening for idiopathic toe walking were retrospectively reviewed. Preoperatively, this group had significantly greater anterior pelvic tilt than normal, decreased peak knee flexion in swing, greater external foot progression, and the expected increased plantar flexion (P < 0.01). Postoperatively, anterior pelvic tilt decreased by a mean of about 4 degrees (P < 0.01), only for the group that had tendo-Achilles lengthening because the gastrocnemius group was close to normal preoperatively, and peak knee flexion normalized. The foot progression angle of this group did not change from preoperative values and remained significantly more external than normal, although dorsiflexion in stance significantly improved after surgery (indicating the goal of the surgery was achieved). Increased external foot progression in idiopathic toe walkers is apparently due to increased external tibial torsion and/or external hip rotation but was unaffected by gastrocnemius/soleus surgical lengthening. Significant improvement occurred on an overall index of gait variables, indicating surgery can be an effective treatment of idiopathic toe walkers.

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.

The purpose of this study was to assess whether the Pediatric Outcomes Data Collection Instrument (PODCI) was able to detect changes in function, as perceived by the parents of children and adolescents with cerebral palsy who had undergone lower limb soft tissue and/or bony surgeries. This was a retrospective study of 80 ambulatory patients who were seen in the motion laboratory and classified with the Gross Motor Functional Classification System (GMFCS). Significant changes (P < 0.05) were detected in the PODCI scores for upper extremity function, transfers and mobility, physical function and sports, and global function after surgery, by approximately 4% to 5%, whereas comfort (pain-free) did not significantly change. There was a significant difference in the PODCI scores preoperatively between GMFCS levels I, II, and III for upper extremity function, transfers and mobility, physical function and sports, and global function. Postoperative improvements were of equal magnitude for each GMFCS level. This suggests that the PODCI did not have a ceiling effect for high-functioning children. Age (+/-10 years) and surgery (soft tissue/soft tissue plus bony) were not significant factors for any of the subcategories preoperative to postoperative. In conclusion, the PODCI detected improvement as perceived by the parents in ambulatory children with cerebral palsy after lower-limb soft tissue and/or bony surgeries in 4 areas by a magnitude of approximately 4% to 5%.

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.

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.

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.

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.

BACKGROUND: Previous studies have demonstrated that the use of tranexamic acid (TXA) reduces blood loss and transfusion requirements in children undergoing scoliosis surgery. Although TXA is safe and effective, significant adverse events have been reported. Using the lowest effective dose of TXA is advisable. We evaluated a new low dosing regimen for TXA based on an improved pharmacokinetic model and therapeutic plasma concentration. The purpose of this study is to evaluate the effectiveness of this new low dosing regimen in reducing blood loss and transfusion requirements in patients with adolescent idiopathic scoliosis undergoing posterior spinal fusion when compared with a control group who did not receive TXA. METHODS: We retrospectively reviewed 90 consecutive patients with idiopathic scoliosis undergoing posterior spinal fusion at our institution from 2017 to 2020. Forty patients received TXA at the new dosing regimen (10 mg/kg load, 5 mg/kg/h infusion) and 50 patients were in the non-TXA control group. The same 2 orthopaedic surgeons, working as a team, performed all surgical procedures. We assessed the use of TXA as an independent risk factor for estimated blood loss and transfusion requirement after adjusting for age, surgical duration, body mass index, major coronal curve, and sex. RESULTS: A comparison of the intraoperative cumulative blood loss in the 2 groups showed a significantly lower blood loss in the TXA group. (583.5±272.0 vs. 479.5±288.7 mL, P=0.03) This difference persisted when blood loss was calculated as percent of total blood volume and per vertebral level. Transfusion requirements were lower in the TXA group (4/50 patients vs. 0/40 patients, P=0.13). No patient in the TXA group required a blood transfusion during their hospitalization. CONCLUSION: This study is the first to provide evidence that a new low dosing regimen of TXA can significantly reduce blood loss and transfusion requirements for idiopathic scoliosis patients and supports the need for a prospective, randomized clinical trial to confirm these findings. LEVEL OF EVIDENCE: Level III-retrospective cohort study.

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