Congenital Ankylosis of the Knee Treated with Van Nes Rotationplasty: A Case Report.

CASE: This is a case of a female patient born with thrombocytopenia-absent radius syndrome, with bilateral upper extremity phocomelia, bilateral hip dislocations, and congenital fusion of the right knee with progressively worsening flexion contracture. At age 3 years and 5 months, the patient was treated with excision of the knee ankylosis and Van Nes rotationplasty. This proved durable at age 20 years (final follow-up) without any need for further surgery and without complication. CONCLUSION: This is the first known report of Van Nes rotationplasty as a durable treatment option in the management of congenital knee ankylosis, which may avoid reoperation and eliminate risk of recurrence.

Anatomic all-epiphyseal ACL reconstruction with "inside-out" femoral tunnel placement in immature patients yields high return to sport rates and functional outcome scores a minimum of 24 months after reconstruction.

PURPOSE: To understand if anatomic physeal-sparing ACL reconstruction in the immature host preserves range of motion, permits a return to sports, and avoids limb length discrepancy and accelerated intra-articular degeneration with a cross-sectional radiographic, physical examination and patient-reported outcomes analysis. METHODS: A cross-sectional recall study included 38 patients aged 7-15 who underwent all-epiphyseal ACL reconstruction with hamstring allograft performed by a single surgeon at a large academic medical center. All-epiphyseal reconstructions were performed using a modified Anderson physeal-sparing technique, with the femoral tunnel placed using an "inside-out" technique. Assessments consisted of a physical exam, long leg cassette radiographs, KT-1000 measurements, subjective patient metrics, and magnetic resonance imaging. RESULTS: Thirty-eight (56.7%) of 66 eligible patients returned for in-person clinical and radiographic exams. Patients were 11.4 ± 1.8 years at the time of surgery. Five patients were females (13.2%). Mean follow-up was 5.5 ± 2.4 years. ACL re-injuries occurred in four patients (10.5%), all of whom underwent revision reconstructions. Thirty-three of the remaining 34 (97.1%) patients returned to sports following their reconstruction, and 24 (70.6%) returned to their baseline level of competition. Mean limb length discrepancy (LLD) was 0.2 ± 1.4 cm. Nine patients had an LLD of > 1 cm (26.5%), which occurred at an equivalent age as those with < 1 cm LLD (10.8 ± 2.0 vs. 11.7 ± 1.7, n.s.). Pre-operative Marx scores (13.1 ± 3.5) were not significantly different from post-operative values (12.3 ± 5.1, n.s.). Patients who required ACL revisions had significantly lower Marx scores than those with intact primary grafts (8.3 ± 7.1 vs. 13.4 ± 4.5, p = 0.047). Cohort mean International Knee Documentation Committee (IKDC) score was 89.7 ± 12.7. CONCLUSION: Anatomic all-epiphyseal anatomic ACL reconstruction appears to be useful in patients with significant projected remaining growth, with good return-to-sport outcomes and minimal risk of clinically significant physeal complications. However, given the limited patient recall possible in the present study, further large sample size, high-quality works are necessary to validate our findings. LEVEL OF EVIDENCE: Level IV.

Intermediate-term annualized curve progression of adolescent idiopathic scoliosis curves measuring 40° or greater.

STUDY DESIGN: Prospective cohort study. OBJECTIVES: The objective of this study was to examine intermediate-term progression for a large series of patients with adolescent idiopathic scoliosis (AIS) with curves 40° or greater. BACKGROUND: Curve progression in AIS has been well documented for smaller curves in adolescence up to skeletal maturity; however, the data on curve progression past 40° or into adulthood are limited. With many surgeons recommending surgical correction when patients reach this threshold, it is important to understand the radiographic progression of curves into adulthood. METHODS: A database of all patients seen by a single surgeon from 1984 through 2018 with AIS curves progressing to at least 40° entered prospectively was utilized for this study. This included a total of 738 patients. Curve progression was analyzed overall and stratified by length of follow-up, curve location, and Risser stage at the time of presentation among other variables. Curve magnitude and Risser stage designations in this study were validated by performing a separate inter- and intrarater agreement study using four independent reviewers reading 50 patients' Cobb angle and Risser stage blinded in triplicate to examine the reliability of the study measurements. RESULTS: Annualized curve progression (ACP) averaged 6.3 ± 10.4°. ACP varied with length of follow-up: patients with up to 1 year of follow-up had an average ACP of 11.5 ± 17.0°, while those with 1-2 years had 8.2 ± 8.8°, and 2-5 years had 3.7 ± 4.1°, tapering off further from there. Risser stage 0 or 1 was associated with the highest ACP as compared to Risser stage 2-3 or 4-5. Intraclass correlation (ICC) values for Cobb angle measurement and Risser stage designations from four raters measuring 50 patients' measures, blinded and in triplicate, were all > 0.80, signifying a high degree of reliability within and between readers. CONCLUSIONS: Annualized curve progression for 40° and greater curves was not linear over time; it was greatest immediately after a curve reaches 40° and tapered off over the next decade. Immature Risser stage at presentation was strongly associated with increasing ACP at all time frames. LEVEL OF EVIDENCE: Prognostic Level I.

SRS-22r Scores in Nonoperated Adolescent Idiopathic Scoliosis Patients With Curves Greater Than Forty Degrees.

STUDY DESIGN: Case control comparative series. OBJECTIVE: Describe surgical range adolescent idiopathic scoliosis (AIS) patients electing to forgo surgery and compare health-related quality-of-life outcomes to a similar cohort of operated AIS patients by the same single surgeon. SUMMARY OF BACKGROUND DATA: No data have been published either documenting SRS-22r scores of nonoperated patients with curves ≥40° or comparing these scores to a demographically similar operated cohort. METHODS: Individuals with curves ≥40°, age ≥18 years, and electing to forgo surgery were identified. All patients completed an SRS-22r questionnaire. This nonoperated cohort's SRS-22r scores were compared to those of a large demographically similar cohort operated by the same surgeon. Group differences between the SRS-22r scores were evaluated by comparing these to published Minimal Clinically Important Differences (MCID) for the SRS-22r. RESULTS: One hundred ninety subjects with nonoperated curves were compared to 166 individuals who underwent surgery. The nonoperated cohort averaged 23.5 years of age, averaged 7.7 years since curve reached 40°, and had an average 50° Cobb angle at last follow-up. No statistical significant differences were found between the groups on the Pain, Function, or Mental Health domains of the SRS-22r. Statistically significant differences in favor of the operative cohort were found for self-image, satisfaction, and total score. The observed group differences did not meet the established thresholds for minimal clinically important differences in any of the domain scores, the average total score, or raw scores. CONCLUSION: There are no meaningful clinically significant differences in SRS-22r scores at average 8-year follow-up between AIS patients with curves ≥40° treated with or without surgery. These data in conjunction with an absence of long-term evidence of serious medical consequences with nonsurgical management of curves ≥40° should encourage surgeons to reevaluate the benefits of routine surgical care. LEVEL OF EVIDENCE: 3.

Does a Weekly Didactic Conference Improve Resident Performance on the Pediatric Domain of the Orthopaedic In-Training Examination?

BACKGROUND: Performance on the Orthopaedic In-training Examination (OITE) has been correlated with performance on the written portion of the American Board of Orthopaedic Surgery examination. Herein we sought to discover whether adding a regular pediatric didactic lecture improved residents' performance on the OITE's pediatric domain. METHODS: In 2012, a didactic lecture series was started in the University of Pittsburgh Medical Center (UPMC) Hamot Orthopaedic Residency Program (Hamot). This includes all topics in pediatric orthopaedic surgery and has teaching faculty present, and occurs weekly with all residents attending. A neighboring program [UMPC Pittsburgh (Pitt)] shares in these conferences, but only during their pediatric rotation. We sought to determine the effectiveness of the conference by comparing the historic scores from each program on the pediatric domain of the OITE examination to scores after the institution of the conference, and by comparing the 2 programs' scores. RESULTS: Both programs demonstrated improvement in OITE scores. In 2008, the mean examination score was 19.6±4.3 (11.0 to 30.0), and the mean percentile was 57.7±12.6 (32.0 to 88.0); in 2014, the mean examination score was 23.5±4.2 (14.0 to 33.0) and the mean percentile was 67.1±12.1 (40.0 to 94.0). OITE scores and percentiles improved with post graduate year (P<0.0001). Compared with the preconference years, Hamot residents answered 3.99 more questions correctly (P<0.0001) and Pitt residents answered 2.93 more questions correctly (P<0.0001). Before the conference, site was not a predictor of OITE score (P=0.06) or percentile (P=0.08); there was no significant difference found between the mean scores per program. However, in the postconference years, site did predict OITE scores. Controlling for year in training, Hamot residents scored higher on the OITE (2.3 points higher, P=0.003) and had higher percentiles (0.07 higher, P=0.004) than Pitt residents during the postconference years. CONCLUSIONS: This study suggests that adding a didactic pediatric lecture improved residents' scores on the OITE and indirectly suggests that more frequent attendance is associated with better scores. LEVEL OF EVIDENCE: Level III-retrospective case-control study.

The Occurrence of Occult Acetabular Dysplasia in Relatives of Individuals With Developmental Dysplasia of the Hip.

BACKGROUND: This study sought to determine the hip pathology of family members of patients with developmental dysplasia of the hip (DDH). The authors evaluated 120 people from 19 families known to have at least 1 member with surgically treated DDH. Each individual's functional outcome scores and pelvic radiographs were assessed for hip symptoms or pathology. METHODS: Using a genetic population database and a pediatric hospital patient population, 19 families with high rates of DDH were identified. All family members (n=120) underwent physical examination, radiographic assessment, and completion of outcome instruments [American Academy of Orthopedics (AAOS) Hip and Knee; Harris Hip Score (HHS); and Western Ontario and McMaster Universities Arthritis Index (WOMAC)]. RESULTS: The 120 subjects ranged from 1 to 84 years, 34 had orthopaedically treated DDH. Of the remaining 86 supposedly normal subjects, 23 (27%) had occult acetabular dysplasia (OAD) as defined by center edge angle (CEA) <20 and/or a Severin score of III or greater. Sixty percent of the 86 individuals were less than 30 years old, 74% of the OAD group were less than 30. Outcome scores of the treated DDH patients (AAOS, HHS, and WOMAC) were worse on the involved side regardless of age. Over age 30 individuals with OAD had statistically significant decreases in their AAOS Hip and Knee and WOMAC scores on the dysplastic side, but their HHS scores were not significantly different. CONCLUSIONS: Twenty-seven percent of first-degree and second-degree relatives of patients with DDH had unsuspected radiographic acetabular dysplasia in our study. Most of the subjects with OAD were younger than 30. After age 30, many of these patients developed symptoms. CLINICAL RELEVANCE: In families with a significant history of DDH, radiographic screening of siblings of patients with DDH to define OAD may be prudent. LEVEL OF EVIDENCE: Level I­diagnostic study.

Imaging of physeal bars in children.

The growth plate, also known as the physis or epiphyseal plate, is essential for longitudinal growth of bones in the immature skeleton. A variety of insults to the growth plate from trauma to infection to idiopathic causes can lead to physeal bar formation, an interruption in normal growth plate cartilage, where a bony or fibrous bridge develops between the metaphysis and epiphysis. This bridge restricts subsequent bone growth, leading to limb shortening and/or angular deformities. Early recognition of the presence of a physeal bar can help direct appropriate surgical management to restore linear growth of the bone.

Using the freehand pedicle screw placement technique in adolescent idiopathic scoliosis surgery: what is the incidence of neurological symptoms secondary to misplaced screws?

STUDY DESIGN: Retrospective case series. OBJECTIVE: This study evaluated the incidence of postoperative neurological symptoms after a freehand pedicle screw insertion technique in idiopathic posterior scoliosis surgery. SUMMARY OF BACKGROUND DATA: It is generally accepted that pedicle screws can be inserted by a freehand technique in the thoracic and lumbar spine in patients with adolescent idiopathic scoliosis (AIS) with a very low frequency of major complications. The prevalence of clinically significant screw misplacement, with or without the need for revision surgery is less well defined. METHODS: Between January 1, 2000, and October 2, 2012, five hundred fifty-nine patients with AIS had thoracolumbar posterior instrumented spine surgery at the Children's Hospital of Pittsburgh. Each patient's chart and radiographs were reviewed and only those with AIS were included. Patients with neuromuscular and syndromic diagnoses were excluded as well as those with congenital or traumatic etiologies, incomplete charts, less than 3 months of follow-up and those without pedicle screws. The records were studied for complaints of radicular pain, neurological deficit, or severe headache that could be indicative of potential screw misplacement. RESULTS: Four hundred eighty-one patients with 5923 pedicle screws met the inclusion criteria. Nine patients (1.9%) developed symptoms and underwent computed tomographic scanning. Six patients were found to have pedicle screw malposition (8 screws) and 3 of these patients underwent revision surgery. Of the 3 revision patients, 2 presented with radicular symptoms (leg pain) and 1 with an orthostatic headache due to cerebrospinal fluid leakage. At the final follow-up, all revision patients had complete symptom resolution. In total, there were 8 symptomatic, misplaced pedicle screws (0.14%) in 6 patients (1.25%). CONCLUSION: During a 12-year period in a dedicated pediatric orthopedic hospital using the freehand placement technique, the incidence of symptomatic misplaced pedicle screws was exceedingly low. LEVEL OF EVIDENCE: 4.

Clinical Decision Making in Early Wound Drainage Following Posterior Spine Surgery in Pediatric Patients.

STUDY DESIGN: Retrospective. OBJECTIVES: To identify the clinical factors suggestive of infected and non-infected drainage to help clinical decision making. SUMMARY OF BACKGROUND DATA: Differentiating between drainage caused by a benign seroma and deep spinal infection may be difficult in the early postoperative period. METHODS: Institutional spine surgery database was searched to identify the cases that were taken back to the operating room for drainage from the surgical wound in the early postoperative period between 2000 and 2012. RESULTS: A total of 38 cases of early wound drainage (within 6 weeks postoperatively) were identified that were treated with opening all layers, irrigation, and debridement. Intraoperative cultures were sent in all cases. Twenty-five patients proved to have non-infected drainage and did not require further treatment. In 13 patients, infection was confirmed with intra-operative findings and cultures; these patients were treated with serial debridements. In 4 cases, implants had to be removed after multiple debridements (after a quiescent period). The group with non-infected drainage differed from the infection group in that most patients (21 of 25) had non-neuromuscular deformities, whereas 77% of the infected group had neuromuscular etiology (10 of 13) (p = .0004). Average number of days to revision was 8.5 (range, 5-14 days) for the non-infected group. Of the 25 patients, 23 presented in the first 10 days. In the infected group, average number of days to revision was 19. Ten of the 13 patients presented on postoperative day 14 or later. Logistic regression analysis showed a significant association between increased likelihood of infection and increased time from the index procedure (p = .0085). CONCLUSIONS: The findings suggest that early presenting drainage in pediatric idiopathic spine deformity is often not infected. Drainage, especially presenting after the second postoperative week in neuromuscular patients, proved to be mostly deep spinal wound infections.

Total hip arthroplasty, hip osteoarthritis, total knee arthroplasty, and knee osteoarthritis in patients with developmental dysplasia of the hip and their family members: a kinship analysis report.

BACKGROUND: Developmental dysplasia of the hip (DDH) is a familial condition with a wide phenotypic expression. Families with high rates of DDH may have individuals with subtle phenotypic expression that can progress to osteoarthritis and require total hip arthroplasty (THA). This study compares the rates of THA in relatives of individuals with DDH with individuals in control families. METHODS: Probands with a diagnosis of DDH were identified using medical records linked to the Utah Population Database. Ten age-matched and sex-matched controls were randomly selected from a pool of unaffected individuals within the Utah Population Database. Diagnostic and procedural codes were used to determine the incidence of hip and knee osteoarthritis (HOA and KOA) and of THA and total knee arthroplasty (TKA) among the cases and controls and their relatives. Relative risks (RR) for HOA and KOA and for THA and TKA were calculated for the probands/controls and their family members. RESULTS: The RR of HOA was significantly increased in probands (RR=82.4; P<2e-16), their parents (R=2.22; P=0.0003), and in their grandparents (RR=1.33; P=0.011). The RR of THA was also significantly increased in probands (RR=1168; P <3e-08) and in their grandparents (RR=2.06; P=0.01). The RR of KOA was significantly increased in probands with a diagnosis of DDH (RR=20.96; P=2.2e-8) but not in their parents or grandparents. The RR of TKA was also increased in probands alone (RR=57.47; P=1.7e-05). CONCLUSIONS: Parents and grandparents of individuals with diagnosed DDH are significantly more likely to be diagnosed with HOA and undergo THA than members of the general population. These first-degree and second-degree relatives were not at higher risk for KOA or TKA. Given the known familial association of DDH, this association with osteoarthritis of the hip suggests a risk of undiagnosed hip dysplasia in individuals whose families have a high rate of DDH. LEVEL OF EVIDENCE: Level III.

Long-term follow-up of open reduction surgery for developmental dislocation of the hip.

BACKGROUND: We posed 2 questions: what is the long-term result of open reduction surgery in developmental dysplasia of the hip, and is there an age at surgery above which the outcome was too poor to recommend the operation? METHODS: Between 1955 and 1995, 148 patients with 179 dislocated hips had open reduction surgery for developmental dysplasia of the hip (141 anterior and 38 Ludloff medial approaches). We attempted to locate all 148 patients for the follow-up evaluation. RESULTS: Fifty-three patients (36%) with 66 hips (37%) were located and participated in the study. These 66 hips represented 34% of the anterior open reductions and 47% of the Ludloff medial reductions. Twenty-two of the 66 hips had Severin IV or worse outcomes and included 7 with total hip arthroplasties and 2 with hip fusions. Age at surgery was significantly lower for Severin I, II, and III, compared with Severin IV and above (P=0.003, 0.001, 0.003) with outcomes deteriorating substantially after age 3. Approximately half of the hips required further surgery for dysplasia. All hips that sustained osseous necrosis had Severin IV or worse outcomes, and hips that redislocated and required revision surgery only achieved Severin I or II ratings 18% of the time. Nine "normal" hips became dysplastic and 3 had pelvic osteotomies as teenagers. Two other normal hips developed osseous necrosis during treatment of the contralateral hip. CONCLUSIONS: Results deteriorate as the age at surgery increases. Osseous necrosis and redislocation predict a poor functional and radiographic result. The "normal" hip may develop insidious dysplasia and also may be injured during treatment of the involved hip. Above age 3, some patients may not have sufficient acetabular growth to remodel a surgically reduced hip. LEVEL OF EVIDENCE: Level IV--case series.

Measurement of the center edge angle and determination of the Severin classification using digital radiography, computer-assisted measurement tools, and a Severin algorithm: intraobserver and interobserver reliability revisited.

BACKGROUND: Numerous studies underscore the poor intraobserver and interobserver reliability of both the center edge angle (CEA) and the Severin classification using plain film measurements. In this study, experienced observers applied a computer-assisted measurement program to determine the CEA in digital pelvic radiographs of adults who had been previously treated for dysplasia of the hip (DDH). Using a teaching aid/algorithm of the Severin classification, the observers then assigned a Severin rating to these hips. Intraobserver and interobserver errors were then calculated on both the CEA measurements and the Severin classifications. METHODS: Four pediatric orthopaedic surgeons and 1 pediatric radiologist calculated the CEAs using the OrthoView TM planning system and then determined the Severin classification on 41 blinded digital pelvic radiographs. The radiographs were evaluated by each examiner twice, with evaluations separated by 2 months. All examiners reviewed a Severin classification algorithm before making their Severin assignments. The intraobserver and interobserver reliability for both the CEA and the Severin classification were calculated using the interclass correlation coefficients and Cohen and Fleiss κ scores, respectively. RESULTS: The intraobserver and interobserver reliability for CEA measurement was moderate to almost perfect. When we separated the Severin classification into 3 clinically relevant groups of good (Severin I and II), dysplastic (Severin III), and poor (Severin IV and above), our interobserver reliability neared almost perfect. CONCLUSION: The Severin classification is an extremely useful and oft-used radiographic measure for the success of DDH treatment. Our research found digital radiography, computer-aided measurement tools, the use of a Severin algorithm, and separating the Severin classification into 3 clinically relevant groups significantly increased the intraobserver and interobserver reliability of both the CEA and Severin classification. This finding will assist future studies using the CEA and Severin classification in the radiographic assessment of DDH treatment outcomes.

The current medical practice of the pediatric orthopaedic surgeon in North America.

Musculoskeletal disorders in children are common and comprise 20% to 30% of the complaints observed by primary care physicians. Most primary care physicians prefer to refer patients with pediatric musculoskeletal conditions to the pediatric orthopaedic surgeon; most of whom are treated nonoperatively. Pediatric orthopaedic surgeons are well trained to provide efficient, cost-effective, and definitive quality care. This article supports the supposition that pediatric orthopaedic surgeons are the primary care physicians for children with musculoskeletal disorders. This article focuses on the primary clinical responsibilities of the pediatric orthopaedic surgeon, describes the value of this practice, and contrasts their responsibilities from that of other orthopaedic subspecialties.

Transfer of ipsilateral fibula on vascular pedicle for treatment of congenital pseudarthrosis of the tibia.

BACKGROUND: Although the use of free vascularized fibula grafts has frequently been reported in the treatment of congenital pseudarthrosis of the tibia, the use of ipsilateral fibula graft on a vascular pedicle is uncommon. We reviewed the long-term results of this procedure in 11 patients. METHODS: The records of 11 patients who underwent transfer of the ipsilateral fibula on a vascular pedicle between 2.1 and 10.8 years of age were retrospectively reviewed. Average follow-up was 11 years after the index procedure. Seven patients had reached skeletal maturity. Clinical records and radiographs were reviewed to determine patient demographics, surgical parameters, union rate, refracture rate, residual deformity, and functional outcome. RESULTS: Eight patients (73%) achieved union at an average of 20.1 months. Additional bone grafting procedures were required in 4 patients with distal nonunions. There were 3 refractures (38%). Four patients eventually underwent amputation, and 1 patient had a persistent nonunion at final follow-up. Residual deformity included tibial valgus and procurvatum deformities, limb length discrepancy, and ankle valgus. Use of the ipsilateral fibula did not seem to increase the risk of ankle valgus. Functional outcomes were good in all but one patient. CONCLUSIONS: Use of the ipsilateral fibula as a pedicle graft provides reasonable results in healing congenital pseudarthrosis of the tibia. Patients should be monitored for the sequelae of this condition, including nonunion, refracture, shortening, and angular deformity, and treated accordingly. LEVEL OF EVIDENCE: Therapeutic Level IV.

"Does the outcome of adolescent idiopathic scoliosis surgery justify the rising cost of the procedures?".

BACKGROUND: As the cost of medical care has steady risen, patients, insurance companies, and the government, have all appropriately questioned the benefit of the care provided versus the cost. Expensive treatments such as surgery for spinal deformity have been especially scrutinized. This article reviews the history of spinal implant usage in deformity surgery, including the benefits of these implants to the patient and also the associated costs. The paper was presented at the One Day Course during the 2009 Pediatric Orthopaedic Society of North America annual meeting in Boston. METHODS: A review was conducted regarding the benefits and costs of the care provided to patients as spinal implants became more clinically effective. RESULTS: Compared with postoperative casting, spinal implants provide better deformity correction and better stability of the fusion mass with resulting lower rates of secondary surgery, mostly because of fewer pseudarthoses. Many of these advantages were achieved with the less-expensive second and third-generation implants. Unfortunately, patient outcomes when the latest, most expensive implants are used are not significantly different from outcomes when older, less-expensive implants are used. CONCLUSIONS: Although the cost of spinal deformity surgery has risen the benefit to the patient from modern spinal implants has also increased. Nevertheless, patient outcomes have not improved in proportion to the increase in costs. Outcomes from the newest, all pedicle screw constructs are not significantly better than outcomes from the older, less-expensive hybrid constructs. Rising expenses and dramatic variation in the cost of the same implant have led payors, hospitals, and the government to question the value added to the care of the patient. Some implant costs should fall as hospitals use competitive bidding. Surgeons should help their hospitals in the competitive bidding process and declare a willingness to switch to an equivalent system if price differences are excessive. LEVELS OF EVIDENCE: Level IV Economic Analysis.

Adult consequences of spina bifida: a cohort study.

BACKGROUND: Patients with spina bifida potentially experience social isolation with limited employment opportunities and restriction of independent living, neurologic and intellectual impairment, and orthopaedic and urologic consequences of their condition. However, the degree of disability as these individuals age into adulthood has not been completely delineated. QUESTIONS/PURPOSE: In adults with spina bifida we determined the cause of death for those who had died, IQ, level of education, work history, independent living status, physical disabilities, and urologic status; we then identified surgical procedures that led to better adult function. METHODS: We retrospectively reviewed 84 individuals with myelomeningocele, examining social, cognitive, and physical disabilities. The minimum age at followup was 20 years (mean, 31 years; range, 20-64 years). RESULTS: Forty-two percent had normal IQs. Seventy percent never needed a neurosurgical shunt and those without shunts had higher IQs. Forty-four percent had regular education and 8% achieved college degrees. Fifty-six percent were unemployed. Thirty percent lived independently. Twenty-three percent were either married or divorced with nine normal offspring. Eighty-five percent dressed themselves, 65% shopped independently, 54% drove. Thirty-one percent were at the thoracic neurologic level (all used wheelchairs), 12% were at L1-L3 (all used a wheelchair except one), 33% were at L4-L5 (78% used a wheelchair at least part-time), and 24% were at S1 and below (all walked). Fifty-four percent experienced decubiti and as a consequence, four required major extremity amputations. Spinal fusions protected sitting balance, but hip surgery did not produce congruent hips and occasionally resulted in debilitating stiffness. Pressure sores resulted in partial foot amputations despite plantigrade feet. CONCLUSIONS: Our observations confirm the consequences associated with the physical and cognitive disabilities of patients with spina bifida. Many of these consequences can likely be mitigated by modern multidisciplinary care and special education to increase independence.

Involvement of the spine in patients with multiple hereditary exostoses.

BACKGROUND: Extension of a vertebral exostosis into the spinal canal is rare, but many isolated cases have been reported in the literature. Three existing patients with multiple hereditary exostoses at our institution had development of neurologic findings and were found to have exostoses in the spinal canal. These findings led us to perform magnetic resonance imaging or computed tomographic scans for the remaining patients with multiple hereditary exostoses at our institution. METHODS: Forty-four patients at our institution (including twenty-six male patients and eighteen female patients) had multiple hereditary exostoses. Forty-three patients were evaluated with magnetic resonance imaging and one was evaluated with computed tomography to look for spinal column involvement. RESULTS: Thirty (68%) of the forty-four patients had exostoses arising from the spinal column, and twelve (27%) had lesions encroaching into the spinal canal. Thirty-six of the forty-four patients also had plain radiographs, but only six had radiographs that accurately identified the lesions and another six had radiographs that mistakenly identified lesions that were not confirmed with magnetic resonance imaging or computed tomography. Patients with lesions inside the spinal canal were typically asymptomatic and neurologically normal, with radiographs that did not demonstrate the lesion. Compared with female patients, male patients were more likely to have spinal lesions and more likely to have lesions encroaching into the spinal canal (p = 0.014). CONCLUSIONS: The risk that a patient with multiple hereditary exostoses has a lesion within the spinal canal is much higher than previously suspected (27%). Because the potential exists for serious neurologic injury to occur, we have begun to use magnetic resonance imaging to screen all patients who have multiple hereditary exostoses at least once during the growing years.

Familial predisposition to developmental dysplasia of the hip.

BACKGROUND: Developmental dysplasia of the hip (DDH) is a common birth defect and is thought to have genetic contributions to the phenotype. It is likely that DDH is genetically heterogeneous with environmental modifiers. The Utah Population Database (UPDB) is a computerized integration of pedigrees, vital statistics, and medical records representing over 6 million individuals, and is a unique resource providing the ability to search for familial factors beyond the nuclear family, decreasing the effect of a shared environment. The purpose of this study is to assess the degree of relationship between individuals with DDH. METHODS: Datasets were created from UPDB statewide birth certificates and from the University of Utah Health Sciences Center enterprise data warehouse using records for DDH and linked to the UPDB. Controls for the dataset were selected that matched cases on birth year and sex and 10 controls were selected per case. Statistics computed for each family were the number of descendants, the observed number of affected, the expected number of affected, P value, familial standardize incidence ratio, relative risks (RRs), and standard error. A kinship analysis tool was used to find pedigrees with excess DDH. RESULTS: The combined data resulted in 1649 distinct individuals with DDH. RR was significantly increased in first-degree relatives (RR=12.1; P<0.000001), siblings (RR=11.9; P<0.000001) and first cousins (RR=1.7; P=0.04). A total of 468 families were identified with at least 5 affected individuals in a family. These results were then filtered to only contain families that had a P value of less than 0.01. This resulted in 141 founders with anywhere between 4 and 30 affected living descendants with a P value of less than 0.01 with family sizes ranging from 594 to 44,819 descendants. A total of 28 founders had a familial standardize incidence ratio of greater than 5.0. CONCLUSIONS: These data suggest a genetic contribution to DDH with a 12-fold increase in risk for first-degree relatives. Better phenotypic characterization and classification will be critical for future genetic analyses.

Is research presented at the scoliosis research society annual meeting influenced by industry funding?

STUDY DESIGN: All abstracts submitted to the 2006 SRS annual meeting were reviewed. OBJECTIVE: To determine the rate of funding in abstracts submitted for presentation at the 2006 Annual Scoliosis Research Society (SRS) meeting and whether funding produced bias toward a positive outcome. SUMMARY OF BACKGROUND DATA: Financial conflicts of interest have been attributed to bias in research. METHODS: Three members the SRS Program Committee reviewed 610 abstracts submitted for presentation at the 2006 annual meeting. The committee's average grade was correlated with type of funding (industry, professional society, university); abstract conclusions (favorable, unfavorable, or only descriptive); and subject category [adolescent idiopathic scoliosis (AIS), motion preservation, etc.]. RESULTS: Of the 610 submitted articles, 72% (n = 440) were unfunded. Of the 170 funded articles, 140 were supported by industry, 7 by government agency, 8 by professional societies, 4 by universities, and 11 by private foundations. There was no statistically significant difference between the reviewers' grades of funded versus unfunded articles (P = 0.39). Comparing AIS articles to all the other categories, the number of funded articles were significantly greater only in motion preservation (P < 0.001) and genetics (P = 0.039). When a consultant/employee relationship was present, there was a significant difference in the proportion of funded articles and favorable findings (P = 0.048). CONCLUSION: The higher percentage of funded articles in motion preservation and genetics compared to AIS articles could reflect a bias in those 2 areas. However, although there were more funded articles in those 2 areas there were not more funded, favorable articles (motion preservation P = 0.059, and genetics P = 0.3). Thus, certain categories attracted more funding than others but there was not a bias toward favorable findings within the funded articles unless the funding was due to a consultant/employee relationship.