Is peak hamstrings muscle-tendon length criterion a sufficient indicator to recommend against surgical lengthening of hamstrings?

BACKGROUND: Excessive knee flexion during stance in children with cerebral palsy is often treated by surgical hamstrings lengthening. Pre-operative hamstrings muscle-tendon length can be estimated from kinematics and often used for decision making to rule out surgical lengthening if peak hamstrings muscle-tendon length is 'Not Short'. RESEARCH QUESTION: If peak hamstrings muscle-tendon length is within two standard deviations of typical, is that a sufficient indicator to rule out surgical hamstrings lengthening? METHODS: Three motion analysis centers retrospectively identified children with cerebral palsy, age 6-17 years, who had consecutive gait analyses with knee flexion at initial contact > 20° and popliteal angle > 35° at initial study. Three groups were considered: Medial Hamstrings Lengthening (MHL), Medial and Lateral Hamstrings Lengthening (MLHL), no surgical intervention (Control). Peak hamstrings muscle-tendon length at initial gait study was computed and categorized as 'Short' or 'Not Short'. Two outcomes variables were considered: change in peak knee extension (PKE) and change in pelvic tilt. Univariate comparisons of all variables were assessed along with a multivariate stepwise regression analysis to identify pre-operative characteristics that may predict post-operative improvement. RESULTS: 440 individuals met inclusion criteria. Percentage of individuals with improved PKE by grouping were- MHL-'Short': 60%, MHL-'Not Short': 65%, MLHL-'Short': 74%, MLHL-'Not Short': 74%, Control 'Short': 20%, Control 'Not Short': 19%. Percentage of individuals with worsened pelvic tilt were- MHL-'Short': 25%, MHL-'Not Short': 11%, MLHL-'Short': 42%, MLHL-'Not Short': 21% with significantly more individuals in MHL-'Short' subgroup compared to MHL-'Not Short'. Multivariate analysis suggested that pre-operative pelvic tilt and weak hip extensor strength have the largest effect on predicting post-operative increase in APT. Peak muscle-tendon length was not a significant predictor of post-operative knee kinematics or increase in APT. SIGNIFICANCE: This study suggests that hamstrings muscle-tendon length criteria by itself is not a sufficient indicator to recommend against hamstrings lengthening.

Cerebral Palsy: Current Concepts and Practices in Musculoskeletal Care.

Cerebral palsy is a neurologic disorder characterized by a spectrum of motor and cognitive deficits resulting from insults to the developing brain. The etiologies are numerous and likely multifactorial; an increasing portion of cases may be attributable to genetic causes, although the exact mechanisms responsible remain poorly understood. Major risk factors include intrauterine stroke and prematurity and neonatal infection, trauma, and hypoxia, which may occur in the prenatal, perinatal, or postnatal period. The Gross Motor Function Classification System (GMFCS) is a widely used tool to establish a child's level of function and to guide treatment; however, additional metrics are necessary to formulate long-term prognoses. Goals of care are to maximize function and independence, which directly correlate with overall quality of life, and family participation is key to establishing goals early in treatment. Nonpharmaceutical treatments include physical, occupational, and speech therapy, as well as bracing, equipment, and technology. There is a breadth of medical interventions for managing hypertonia, including medications, botulinum toxin injections, intrathecal baclofen pumps, and selective dorsal rhizotomy. Orthopedic interventions are indicated for symptomatic or progressive musculoskeletal sequelae. Treatments for dysplastic hips and/or hip instability range from soft tissue releases to bony procedures. Neuromuscular scoliosis is managed with posterior spinal fusion because bracing is ineffective against these rapidly progressive curves. The degree of care varies considerably depending on the child's baseline GMFCS level and functional capabilities, and early screening, diagnosis, and appropriate referrals are paramount to initiating early care and maximizing the child's quality of life.

Can Use of Default Dispensing Quantities in Electronic Medical Record Lower Opioid Prescribing?

OBJECTIVES: Leftover opioids can contribute to misuse and abuse. Recommended dosing quantities in the electronic medical record can guide prescribing patterns. We hypothesized that decreasing the default from 30 doses to 12 doses would decrease the overall number of opioids prescribed without increasing second opioid prescriptions or additional health utilization. METHODS: We performed a single-center retrospective study of children with forearm and elbow fractures who presented to the emergency department for evaluation and subsequent orthopedic follow-up between January 15, and September 19, 2017. The default dispensing quantity was decreased on June 1, 2016 from 30 doses to 12 doses. Patients were categorized to preintervention and postintervention groups. We compared the number of opioids prescribed, second opioid prescriptions, emergency department visits, and pain-related telephone calls and orthopedic visits with χ2 and logistic regression analyses. RESULTS: There were 1107 patients included. Rates of opioid prescribing were similar preintervention and postintervention (61% vs 56%, P = 0.13). After the change to the default quantity, the median number of doses decreased from 18 to 12 doses, with opioid prescriptions of 30 or more doses dropping from 35% to 11%. No significant association was found between preintervention versus postintervention, opioid prescription at discharge, and having 1 or more pain-related or unexpected follow-up visits. CONCLUSIONS: Lowering the default dispensing quantity of opioids in the electronic medical record decreases the number of opioids prescribed without increasing second prescriptions or additional health care utilization. These findings suggest that a further reduction in the number of opioids prescribed for upper-extremity fractures may be possible.

Intraoperative Neurological Monitoring in Lower Limb Surgery for Patients With Mucopolysaccharidoses.

BACKGROUND: There are reports of spinal cord injury (SCI) occurring after lower extremity (LE) surgery in children with mucopolysaccharidoses (MPS). Intraoperative neurological monitoring (IONM) has been adopted in some centers to assess real-time spinal cord function during these procedures. The aim of this investigation was to review 3 specialty centers' experiences with MPS patients undergoing LE surgery. We report how IONM affected care and the details of spinal cord injuries in these patients. METHODS: All pediatric MPS patients who underwent LE surgery between 2001 and 2018 were reviewed at 3 children's orthopaedic specialty centers. Demographic and surgical details were reviewed. Estimated blood loss (EBL), surgical time, positioning, use of IONM, and changes in management as a result of IONM were recorded. Details of any spinal cord injuries were examined in detail. RESULTS: During the study period, 92 patients with MPS underwent 252 LE surgeries. IONM was used in 83 of 252 (32.9%) surgeries, and intraoperative care was altered in 17 of 83 (20.5%) cases, including serial repositioning (n=7), aggressive blood pressure management (n=6), and abortion of procedures (n=8). IONM was utilized in cases with larger EBL (279 vs. 130 mL) and longer operative time (274 vs. 175 min) compared with procedures without IONM. Three patients without IONM sustained complete thoracic SCI postoperatively, all from cord infarction in the upper thoracic region. These 3 cases were characterized by long surgical time (328±41 min) and substantial EBL (533±416 mL or 30.5% of total blood volume; range, 11% to 50%). No LE surgeries accompanied by IONM experienced SCI. CONCLUSIONS: Patients with MPS undergoing LE orthopaedic surgery may be at risk for SCI, particularly if the procedures are long or are expected to have large EBL. One hypothesis for the etiology of SCI in this setting is hypoperfusion of the upper thoracic spinal cord due to prolonged intraoperative or postoperative hypotension. IONM during these procedures may mitigate the risk of SCI by identifying real-time changes in spinal cord function during surgery, inciting a change in the surgical plan. LEVEL OF EVIDENCE: Level III-retrospective comparative series.

Checklists in Femur Fractures: High Adherence After Implementation of Computer-based Pediatric Femur Guidelines.

INTRODUCTION: The American Academy of Orthopaedic Surgeons (AAOS) created an evidence-based clinical practice guideline for the care of pediatric diaphyseal femur fractures in 2010. Our institution implemented checklists based off these guidelines embedded in a standardized EMR order. The purpose of this study was to describe compliance with checklist completion and to assess safety improvement in a large urban pediatric hospital. METHODS: Retrospective and prospective data were collected from 2 years before and 5 years after checklist implementation. This included the patient safety checklists from August 2011 through August 2016. Patients aged 0 to 18 years with a diaphyseal femur fracture were queried from the EMR and included in this study. Patient charts were reviewed for complications, including nerve injury, pressure sore, leg length discrepancy, loss of reduction, failure of fixation, nonunion, delayed union, and infection. Compliance rates were reported based on the AAOS clinical practice guidelines. RESULTS: A total of 313 patients for the postchecklist period were reviewed in this study. Of 219 patients eligible for inclusion, 198 had checklists completed (group B). This group was compared with 100 patients with diaphyseal femur fractures from the period before implementation of the checklist (group A). We found no statistical difference in the number of patients with complications between groups (12% in both groups, P = 0.988). Postoperative checklists demonstrated that 89.9% of patients (178/198) received age-appropriate treatment consistent with the AAOS guideline recommendations after implementation of the checklist. Before the checklist implementation (group A), 94% (94/100) adhered to the guidelines. CONCLUSION: This study reveals high compliance rates with the AAOS evidence-based clinical practice guideline for the management of pediatric femur fractures. Implementing standardized checklists is possible by embedding them into the EMR. Implementation of checklists did not improve compliance or patient outcomes.

Foot and Ankle Musculoskeletal Imaging of Pediatric Patients With Cerebral Palsy.

OBJECTIVE. Any combination of abnormal positioning of the ankle, hindfoot, midfoot, and forefoot is possible in the context of cerebral palsy, but some patterns are more common than others. The purpose of this article is to discuss the radiographic manifestations and surgical management of the following common conditions: equinus, equinoplanovalgus, equinocavovarus, vertical talus, oblique talus, hallux valgus, and ankle valgus. CONCLUSION. CP is defined by abnormalities of the developing fetal or infant brain that result in permanent central motor dysfunction. Foot and ankle deformities are very common in the patients with CP, occurring in up to 93% of such patients as a result of underlying abnormal muscle tone, impaired motor control, and dynamic muscle imbalance. Radiologists must develop knowledge of the most common changes in alignment and basic techniques for correction to better recognize abnormalities and improve communication with orthopedic colleagues.

Cerebral palsy - beyond hip deformities.

Cerebral palsy is a neurologic condition with myriad musculoskeletal and articular manifestations. While every patient is unique with innumerable variations in presentation, symptoms and treatments, there are broad themes and recognizable patterns of development. Many of these findings spill over to other neurodevelopmental disorders, and lessons learned from children with cerebral palsy translate well to multiple neurologic conditions. This review focuses on the more common manifestations involving the spine, knee, foot and ankle, with an emphasis on collecting and describing imaging features, along with clinical and radiologic pearls and pitfalls.

Comparison of Ponte Osteotomies and 3-Column Osteotomies in the Treatment of Congenital Spinal Deformity.

BACKGROUND: Congenital spinal deformity (CSD) has traditionally been treated with 3-column osteotomies [hemivertebrectomy (HV) or vertebral column resection (VCR)] to address rigid deformities. Alternatively, multiple Ponte osteotomies (PO) may provide correction while minimizing risk. The purpose of this study was to compare safety and outcomes of patients undergoing surgical treatment for CSD with these 3 procedures. METHODS: Retrospective review of CSD patients treated with posterior spinal fusion between 1996 to 2013. Patients treated with multiple Ponte osteotomies (PO group) were compared with those managed with 3-column osteotomies (HV/VCR group). Patients with previous instrumentation, isolated cervical deformity, growing spine instrumentation, or <2 year follow-up were excluded. Deformity angular ratio (DAR) was calculated as curve magnitude divided by number of levels of the deformity. RESULTS: There were 49 patients [17 PO, 32 HV/VCR (26 HV, 6 VCR)]. For the PO group, mean age was 14 years, and they had an average of 4 ponte osteotomies and 11 levels fused. Mean total DAR was 25 and mean number of congenital anomalies was 1.8 in the PO group. The HV/VCR group had a mean age of 7 years and 5 levels fused. Mean total DAR was 28 and mean number of congenital anomalies was 2.1 in the HV/VCR group. Patients had a mean of 54.1% correction of coronal deformity in the PO group and 54.4% in the HV/VCR group (P=0.78). Signal changes were observed less frequently with PO (1/17) and HV (1/26) than with VCR (4/6), P=0.001. Revision rates were 17.6% (3/17) in the PO group and 37.5% (12/32) in the HV/VCR group (P=0.35). CONCLUSIONS: Patients with CSD and a mean total DAR of 25 treated with multiple PO and long fusions had correction comparable with the HV/VCR group. Patients treated with VCR had the highest incidence of signal changes and postoperative neurologic deficits. LEVEL OF EVIDENCE: Level III.

Rectus Femoris Transfer Surgery Worsens Crouch Gait in Children With Cerebral Palsy at GMFCS Levels III and IV.

BACKGROUND: Previous study has shown that children with cerebral palsy (CP) functioning at Gross Motor Function Classification System (GMFCS) levels III and IV do not benefit from distal rectus femoris transfer (DRFT) due to lack of improvement in stance knee extension. The fate of knees in such subjects who do not undergo DRFT is unknown. The purpose of this study was to compare knee kinematic outcomes in patients with CP and stiff knee gait who underwent single-event multilevel surgery with and without DRFT. METHODS: Preoperative and postoperative gait analysis data were retrospectively reviewed for ambulatory (GMFCS levels I to IV) patients with CP with crouch and stiff knee gait whom underwent single-event multilevel surgery, including hamstring lengthening either with DRFT (N=34) or without DRFT (N=40). Statistical analyses included t tests and χ tests, and multiple regression analysis was performed to adjust for covariates. Data were stratified by GMFCS level groups I/II and III/IV. RESULTS: Improved maximum knee extension in stance was seen for both the DRFT (P=0.0002) and no DRFT groups (P≤0.0006) at GMFCS levels I/II, and the no DRFT group at GMFCS levels III/IV (P=0.02). Excessive stance knee flexion persisted for those at GMFCS level III/IV after DRFT. Maximum knee flexion in swing was maintained after DRFT, but significantly decreased in the no DRFT group (P<0.002) for both GMFCS groups. Change in total knee range of motion improved after DRFT only in the GMFCS I/II group subjects with unilateral involvement (P=0.01). Timing of maximum knee flexion in swing improved for all patients regardless of DRFT or GMFCS level group (P<0.0001). CONCLUSIONS: In patients with CP functioning at GMFCS levels III and IV, DRFT results in persistent crouch postoperatively. Given the importance of maintaining upright posture in these patients, we do not recommend DRFT in patients functioning at GMFCS levels III and IV. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Percutaneous Hamstring Lengthening Surgery is as Effective as Open Lengthening in Children With Cerebral Palsy.

BACKGROUND: Surgical lengthening of the hamstrings is often performed to correct crouch gait in children with cerebral palsy (CP). Previous studies have demonstrated the effectiveness of open hamstring lengthening (oHSL) in improving knee extension static and dynamic range of motion; however, literature regarding percutaneous hamstring lengthening (pHSL) is limited. The purpose of this study was to investigate the effect of open versus pHSL for improving crouch gait and knee function in children with CP. METHODS: This retrospective cohort study included 87 ambulatory children with CP who underwent HLS surgery with both preoperative and postoperative gait analysis (mean time, 29.4±19.9 mo after surgery) testing between 1997 and 2015. In total, 65 patients underwent oHLS surgery (mean age, 8.5±2.5 y) and 22 patients underwent pHSL surgery (mean age, 8.3±2.3 y). Lower extremity three-dimensional kinematic data were collected while subjects walked at a self-selected speed. Outcome variables for operative limbs were compared within and between groups using t tests, χ tests, and multiple regression analysis. RESULTS: Significant postoperative decreases in knee flexion at initial contact were seen for both open (Δ12.7±13.4 degrees; P<0.001) and percutaneous (Δ19.1±13.1 degrees; P<0.001) groups. Increased postoperative maximum knee extension in stance was found for both open (Δ8.2±16.8 degrees; P=0.001) and percutaneous (Δ14.4±16.5 degrees; P=0.001) groups. No significant differences between open and percutaneous groups were found when comparing postoperative changes in kinematic variables between groups after adjusting for covariates. Postoperative changes in static range of motion were similar between lengthening groups. CONCLUSIONS: pHSL is as effective as open lengthening in improving stance phase knee kinematics during gait in children with CP. This is the first study to compare the kinematic effects of open versus pHSL in the pediatric population. Percutaneous lengthening is tolerated well by patients, and as it allows for rapid rehabilitation it may be preferable to the open procedure. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Incidence, Risk Factors, and Definition for Nonunion in Pediatric Lateral Condyle Fractures.

INTRODUCTION: Lateral condyle fractures are a common pediatric elbow injury. Nonunion is a known complication of this injury but its incidence and causative risk factors are unknown. Further, a clear and consistent definition of nonunion for pediatric lateral condyle fractures does not exist. The purpose of this study is to determine the rate of nonunion, the risk factors associated with it and to provide a clear definition. METHODS: A retrospective chart review of all pediatric lateral condyle fractures treated at an urban, tertiary pediatric care center between 2001 and 2014. Data collected included demographics, Weiss classification, type of treatment, follow-up, time from injury to surgery and complications. For patients with nonunions, additional treatments and final range of motion were also recorded. A nonunion was defined as lack of callus with fragment migration by 8 weeks after initiation of treatment. RESULTS: A total of 530 patients were identified of which 500 were available for review. There were 7/500 (1.4%) nonunions in the cohort. Nonunion occurred in 1.4% (2/140) of type I fractures, 0% (0 /178) of type II fractures, and 3% (5/168) of type III fractures. The only significant risk factor for nonunion was the presence of a type III fracture (P=0.05). Five patients with nonunion underwent revision surgery with a partially threaded cannulated cancellous screw. All of these patients went on to union. Four of the 5 patients had their screws removed after union. CONCLUSION: Nonunion after pediatric lateral condyle fracture is rare and is defined by lack of any callus with fragment migration at 8 weeks. The only significant risk factor for nonunion development was the presence of a type III fracture. Revision surgery with a partially threaded screw achieved union in all cases. LEVEL OF EVIDENCE: Level IV-retrospective case review.

Periosteal turndown flap for posterior occipitocervical fusion: a technique review.

PURPOSE: Recently, several authors have proposed techniques for improving the fusion rate in pediatric posterior occipitocervical fusion including a variety of implants and the use of bone morphogenetic protein. A technique by Koop et al. using a periosteal flap for occipitocervical arthrodesis was described in 1984. METHODS: A straight incision is made about the posterior neck to expose the occipitocervical region from the inion superiorly to the lowest cervical vertebrae to be fused inferiorly. The occiput is exposed superficial to the periosteum, which is then reflected and elevated from the occiput. The attachment is preserved at the caudal base of the flap and reflected over the intended area of fusion. When possible, fixation is then performed with cables, wires, screws, hooks, or plates. CASE EXAMPLE: A 6-year-old male with an occiput to C2 distraction injury underwent posterior spinal fusion from occiput to C3 using sublaminar wires, periosteal turndown flap, and autologous iliac crest bone graft. CONCLUSION: In small children with traumatic upper cervical spine instability, the periosteal turndown technique may be used as a safe adjunct for occipitocervical fusions.

Benign Natural History of Spondylolysis in Adolescence With Midterm Follow-Up.

STUDY DESIGN: Retrospective chart review. OBJECTIVES: To use the Micheli Functional Scale to assess adolescent patients with spondylolysis treated conservatively at midterm follow-up. SUMMARY OF BACKGROUND: Spondylolysis is a common source of back pain for adolescents and is generally managed with bracing and physical therapy. There is little data regarding the results of conservative management of spondylolysis over time. METHODS: Four major academic pediatric institutions performed a retrospective chart review of patients from 5 to 21 years of age with the initial diagnosis of spondylolysis. Inclusion criteria were patients who initially underwent conservative management and had a minimum of 2 years' follow-up. The patients were contacted and asked to complete the Micheli Functional Scale Survey. RESULTS: A total of 295 patients with the diagnosis of spondylolysis were identified and contacted. Sixty-one subjects with spondylolysis completed the follow-up survey. Sixty of 61 respondents (98%) answered questions regarding their current pain level. Thirty-five of 60 (58.3%) reported no pain (0/10) and 47/60 (78%) rated their pain at 3 or less, whereas 22% (13/60) rated their pain as 4 or higher. There was no correlation with pain ratings on the follow-up survey and radiographic healing at initial management. Of the 61 patients, 50 returned to sports (82%), 8 did not return (13%), and 5 returned to most but not all of their sports (8%). No correlation was observed between radiographic healing and return to sports (p = .4885). CONCLUSION: Using a validated functional scale, this study demonstrated that with conservative management of spondylolysis a majority of patients at an average of 8 years out self-report a return to sports (90%), though many reported continued pain (42%) and interference with activities (67%). There was no correlation observed between radiographic evidence of healing and pain or return to sports with a mean follow-up of 8 years. LEVEL OF EVIDENCE: Multicenter retrospective case series.

Accelerated Discharge Protocol for Posterior Spinal Fusion Patients With Adolescent Idiopathic Scoliosis Decreases Hospital Postoperative Charges 22.

STUDY DESIGN: A retrospective study of consecutive patients. OBJECTIVE: The purpose of this study was to determine implementing an accelerated protocol could decrease our average hospital stay and what impact this had on postoperative pain management. SUMMARY OF BACKGROUND DATA: To our knowledge, no prior studies have reviewed the effect of an accelerated discharge protocol on postoperative pain control for adolescent idiopathic scoliosis (AIS) following posterior spinal fusion. METHODS: This is a retrospective review of all consecutive patients undergoing posterior spinal fusion (PSF) for AIS before (June 1, 2008-May 31, 2013 = traditional protocol) and after (June 1, 2013-October 22, 2014 = accelerated protocol) protocol implementation. Subjective response to the FACES Pain Intensity scale was collected for each postoperative day while in the hospital by the nursing staff. RESULTS: There were 194 patients in the traditional pathway and 90 patients in the accelerated pathway. No significant differences in age at surgery, sex, or number of levels fused were present between the groups. Patients managed under the accelerated discharge had an average hospital stay of 3.7 days compared with 5.0 days for the traditional discharge (P < 0.001). There was no increased incidence of wound complications between the two groups [3.6% (7/194) vs. 3.3% (3/90), P = 0.91] or readmission [1.5% (3/194) vs. 4.4% (4/90), P = 0.213]. Hospital charges for postoperative care were significantly less in the accelerated discharge group than in the traditional group ($18,360 vs. $23,640, P < 0.0001). This corresponded to a 22% ($5280/$23,640) decrease in postoperative hospital charges. Patients had a small (<1 point change on FACES pain scale) but statistically significant increase in pain on postoperative days 2, 3, and 4 (P = 0.0001, P = 0.0079, P = 0.0076). CONCLUSION: Accelerated discharge following PSF for AIS was associated with a 22% decrease in hospital charges in the postoperative period. LEVEL OF EVIDENCE: 4.

Musculoskeletal functional outcomes in children with osteogenesis imperfecta: associations with disease severity and pamidronate therapy.

BACKGROUND: Intravenous pamidronate has been used off-label in the treatment of severe osteogenesis imperfecta (OI) for almost 20 years. Previous studies have found correlations between function and bone density in patients with OI, but have not studied the functional outcomes of these patients after bisphosphonate therapy with a validated outcome measure. The goal of this investigation is to describe the functionality and comfort of children with OI. We hypothesize that function is impaired in children with severe OI as measured using the Pediatric Outcomes Data Collection Instrument (PODCI) and that improvements in the function of children with severe OI may be observed in association with intravenous bisphosphonate therapy. METHODS: A total of 25 patients with OI were evaluated, of those, 15 received pamidronate therapy. Children with >2 long bone fractures per year were classified as having severe OI and were eligible for pamidronate therapy. Functional evaluation was performed using the PODCI for children who qualified for pamidronate therapy (severe OI) and those who did not qualify for pamidronate therapy (mild OI). PODCI evaluation was also performed after treatment with pamidronate in the group that qualified for pamidronate therapy. RESULTS: There was a statistically significant difference at baseline between patients with "mild" and "severe" OI in the sports/physical functioning scale (P=0.0032). Among the children who received bisphosphonate therapy, PODCI scores in the sports/physical functioning domain were significantly improved after pamidronate therapy (P=0.0364). CONCLUSIONS: This study indicates that children with mild forms of OI can be differentiated from their more severe counterparts by their ability to participate in high-level play activities. Furthermore, patients with "severe" OI show a significant improvement in their ability to participate in high-level play after 1 year of pamidronate. LEVEL OF EVIDENCE: Level IV.

Mucopolysaccharide disorders in orthopaedic surgery.

The mucopolysaccharidoses (MPSs) are a family of disorders characterized by the accumulation of glycosaminoglycans, which is caused by enzyme deficiencies in the lysosomal metabolism of these normal cellular byproducts. Skeletal abnormalities are early and prominent features of MPS, and the orthopaedic surgeon is often the first healthcare provider to raise suspicion for this diagnosis. Recently developed medical therapies for the management of MPS (ie, hematopoietic stem cell transplantation, intravenous enzyme replacement therapy) have led to increased lifespan but have not had much effect on the development of skeletal deformities. Patients must be monitored carefully and treated surgically as necessary. Conditions that may require surgical management include cervical spine and atlantoaxial instability, gibbus deformity, hip dysplasia and osteonecrosis, genu valgum, and carpal tunnel syndrome. The orthopaedic surgeon should have a basic understanding of MPS and of the clinical presentation, musculoskeletal abnormalities, and radiographic findings associated with this group of disorders.