Atlantoaxial rotatory displacement in children.

AIM: To correlate the Pang and Lee class with the clinical course in a consecutive series of patients presenting with painful torticollis. METHODS: Forty-seven dynamic rotational computed tomography (CT) scans in 35 patients were classified into one of the five types defined by Pang and Li, including types I (atlantoaxial rotatopry fixation), II ("pathologic stickiness" without crossover of C1 on C2), III ("pathologic stickiness" with crossover of C1 on C2), IV (normal or muscular torticollis), and V (diagnostic grey zone). The Pang and Li class was then compared with the radiologist's report, which was graded abnormal, diagnosis of rotatory subluxation or fixation, or non-diagnostic. Medical records were reviewed and the clinical course was compared among the five sub-types. RESULTS: We reviewed 47 CT scans in 35 patients, and the majority were performed without sedation. The average age was 7.7 years (4-14 years old) and associated conditions included minor trauma (20%), surgical procedures around the head and neck (29%), and Grisels syndrome (20%). Twenty-six percent of our studies fell within the pathologic spectrum (5% type 1 or rotatory fixation, 21% types 2 and 3 or rotatory subluxation), while 45% were classified as muscular torticollis (45%) and 28% fell within the diagnostic grey zone. Seven radiologists interpreted these studies, and their interpretation was discordant in 45% of cases. Clinical resolution occurred in 27 of 29 cases for which follow-up was available. One of two patients with fixed rotatory subluxation required a C1-C2 arthrodesis. CONCLUSION: The Pang and Li classification characterizes a spectrum of abnormalities in rotation to facilitate communication, although the indications for dynamic CT scan should be further defined.

Unusual presentation of osteoblastoma as vertebra plana-a case report and review of literature.

BACKGROUND: Osteoblastoma is rare and accounts for 3% of all benign tumors and 1% of all bone tumors. The spine is the most common site of occurrence, constituting 32% to 45% of all osteoblastomas. It has a strong predilection for the posterior elements, most often occurring in the lumbar spine. METHOD: In this case report, we describe an unusual presentation of spinal osteoblastoma presenting as thoracic T9 vertebra plana in a 20-year-old female. She presented with discomfort over the midback with unsteadiness of gait. The patient underwent detailed investigations including computed tomography (CT), magnetic resonance imaging, and CT-guided biopsy. To our knowledge, this is the first case report of vertebra plana due to spinal osteoblastoma in the English literature. RESULT: The patient successfully underwent posterior decompression of T9 with laminectomy followed by minimally invasive surgery posterior instrumentation from T7 to T11. Histopathology of the intraoperative specimen was consistent with osteoblastoma. The patient had an uneventful postoperative recovery and no evidence of tumor recurrence could be demonstrated on positron emission tomography scan at 15 months' follow-up. CONCLUSION: In conclusion, the differential diagnosis for vertebra plana is extensive and we add spinal osteoblastoma as another etiology to the existing list. Diagnosis and treatment of vertebra plana involve multimodality radiological imaging, and careful histological and surgical evaluation to identify the underlying etiology.

The Long-term Outcome of Early Spine Fusion for Scoliosis in Children With Cerebral Palsy.

STUDY DESIGN: Retrospective review of radiographs and charts (case-only). OBJECTIVE: The aim of this study was to describe the long-term outcomes of spine fusion for neuromuscular scoliosis in children below 10 years of age with cerebral palsy (CP). SUMMARY OF BACKGROUND DATA: Severely involved children with CP may develop early-onset scoliosis. The outcome of spine fusion is not clear and there are no studies focused on spine fusion in this young patient population. METHODS: This is a retrospective review of 33 children who underwent spine fusion with unit-rod instrumentation between 1989 and 2006 for CP neuromuscular scoliosis, aged below 10 years at spine fusion, and with follow-up >5 years. Demographic, medical, and radiographic data were retrospectively assessed. Repeated measure analysis of variance and Kaplan-Meier survival estimates were used for data assessment. RESULTS: Thirty-three of 42 patients who underwent spine fusion in this period, 19 boys and 14 girls, met the inclusion criteria. Of 9 patients who were excluded, 3 were lost to follow-up and remaining 6 died within 5 years of surgery. Mean age at surgery was 8.3 years (range, 4.4-9.9 y). Mean follow-up was 9.8 years (range, 5.5-15.8 y). Gross motor function classification system level was V in 31 patients and IV in 2 patients. Thirty-one patients (94%) had seizure disorder, 29 patients (88%) had gastric feeding tubes, and 9 patients (27%) had tracheostomy tubes. Eighty-five percent of the patients had posterior-only surgery. Mean Cobb angles preoperative, immediately postoperative, and at final follow-up were 85, 21, and 24 degrees, respectively. Mean postoperative pelvic obliquity correction was 15±9 degrees (P<0.001). At final follow-up, there was no significant change from the postoperative measurements. Complications included 1 deep wound infection and 10 other problems. Eleven patients (28.2%) died after a mean follow-up of 5.6±3.8 years. CONCLUSIONS: In our cohort with early-onset neuromuscular scoliosis, spine fusion was associated with minimal short-term and long-term morbidity, but there was 28% mortality at 10 years of follow-up and 50% predicted mortality at 15 years.

Pamidronate Treatment to Prevent Reoccurring Fractures in Children With Cerebral Palsy.

BACKGROUND: Some children with cerebral palsy (CP) have frequent fractures due to low bone mineral density and receive treatment with pamidronate, an intravenous bisphosphonate. Our review evaluates the outcome of pamidronate treatment in these children. METHODS: A retrospective chart review was performed, and 32 patients (14 girls and 18 boys) with CP Gross Motor Function Classification System level III (2 patients), IV (3 patients), and V (27 patients) treated with 5 courses of pamidronate for low mineral density were identified. Patients with a minimum of 2 years of follow-up were included in the study. Data collection was a review of the demographics and pretreatment, peritreatment, and posttreatment fracture history. RESULTS: The mean age at treatment was 11.6 years (range, 2.9 to 19.6 y). There were 102 fractures (mean duration 2.5 y) pretreatment and 28 fractures posttreatment. With an average follow-up of 6.4 years, posttreatment rate of fracture decreased to 0.10 fractures per year from the pretreatment rate of 2.4 fractures per year (P<0.001). The femur was the most common bone fractured both pretreatment (54%) and posttreatment (61%); the major site was the distal third of the femur. There were 11 fractures during the course of pamidronate treatment at a rate of 0.33 fractures per year. Only 11 patients (34%) sustained fracture posttreatment. No correlation with fracture pattern or occurrence was found with patient age, number of pretreatment fractures, or sex. Most fractures were caused by low-energy injuries, and most were managed nonoperatively. CONCLUSIONS: In patients with CP and disuse osteoporosis, the most common fracture sustained involved the distal femur via low-velocity injury, and most fractures were treated nonoperatively. Although the fracture pattern and the treatment remained unchanged, reoccurring fractures in these children can be effectively treated medically to interrupt the fracturing tendency.

Prediction of Curve Progression in Idiopathic Scoliosis: Validation of the Sanders Skeletal Maturity Staging System.

STUDY DESIGN: Retrospective case series. OBJECTIVE: This study aimed to validate the Sanders Skeletal Maturity Staging System and to assess its correlation to curve progression in idiopathic scoliosis. SUMMARY OF BACKGROUND DATA: The Sanders Skeletal Maturity Staging System has been used to predict curve progression in idiopathic scoliosis. This study intended to validate that initial study with a larger sample size. METHODS: We retrospectively reviewed 1100 consecutive patients with idiopathic scoliosis between 2005 and 2011. Girls aged 8 to 14 years (<2 yr postmenarche) and boys aged 10 to 16 years who had obtained at least 1 hand and spine radiograph on the same day for evaluation of skeletal age and scoliosis curve magnitude were followed to skeletal maturity (Risser stage 5 or fully capped Risser stage 4), curve progression to 50° or greater, or spinal fusion. Patients with nonidiopathic curves were excluded. RESULTS: There were 161 patients: 131 girls (12.3 ± 1.2 yr) and 30 boys (13.9 ± 1.1 yr). The distribution of patients within Sanders stage (SS) 1 through 7 was 7, 28, 41, 45, 7, 31, and 2 patients, respectively; modified Lenke curve types 1 to 6 were 26, 12, 63, 5, 38, and 17 patients, respectively. All patients in SS2 with initial Cobb angles of 25° or greater progressed, and patients in SS1 and SS3 with initial Cobb angles of 35° or greater progressed. Similarly, all patients with initial Cobb angles of 40° or greater progressed except those in SS7. Conversely, none of the patients with initial Cobb angles of 15° or less or those in SS5, SS6, and SS7 with initial Cobb angles of 30° or less progressed. Predictive progression of 67%, 50%, 43%, 27%, and 60% was observed for subgroups SS1/30°, SS2/20°, SS3/30°, SS4/30°, and SS6/35° respectively. CONCLUSION: This larger cohort shows a strong predictive correlation between SS and initial Cobb angle for probability of curve progression in idiopathic scoliosis. LEVEL OF EVIDENCE: 3.

Simplified skeletal maturity scoring system: learning curve and methods to improve reliability.

STUDY DESIGN: Retrospective radiographical review by 5 independent observers. OBJECTIVE: To validate the intra- and interobserver reliability of the simplified skeletal maturity scoring (SSMS) system in a large cohort for each stage and for the overall cohort. SUMMARY OF BACKGROUND DATA: The SSMS has been used to successfully predict curve progression in idiopathic scoliosis. METHODS: A total of 275 patients with scoliosis (8-16 yr) with 1 hand radiograph were included from 2005 to 2011. Five participants independently scored images on 2 separate occasions using the SSMS (stage, 1-8). Observers (listed in order of increasing SSMS experience) included orthopedic surgery resident, clinical fellow (CF), research fellow, and senior faculty. Intraobserver agreement between the 2 sets of scores was estimated using the Pearson and Spearman rank correlation coefficients. Interobserver agreement was estimated with the unweighted Fleiss κ coefficient for the overall cohort and for junior (orthopedic surgery resident, CF, research fellow) versus senior faculty. RESULTS: Intrarater reliability for orthopedic surgery resident, CF, research fellow, senior faculty was 0.956, 0.967, 0.986, 0.991, and 0.998, respectively (Spearman). Intrarater agreement improved with greater familiarity using the SSMS. The inter-rater reliability for junior faculty (κ = 0.65), senior faculty (κ = 0.652), and the overall group (κ = 0.66) indicated agreement between all observers but no improved inter-rater agreement with experience. However, 98% of disagreements occurred only within 1 stage. Stages 2, 3, and 4 accounted for most of the variability; stage 3 was the most commonly scored stage, corresponding to peak growth velocity. CONCLUSION: The SSMS has excellent intraobserver agreement with substantial interobserver agreement. Intraobserver--but not interobserver agreement--improves with familiarity using the SSMS. Expectancy bias may contribute to a higher likelihood of assigning an SSMS 3. Discrepancies when classifying stages 2 to 4 may be resolved by improved descriptions of epiphyseal capping in stages 2 and 3. LEVEL OF EVIDENCE: 2.

Occipitocervical fusion in skeletal dysplasia: a new surgical technique.

STUDY DESIGN: Retrospective cohort study. OBJECTIVE: This study describes clinical and radiological results of a new cable technique for occipitocervical fusion (OCF) in children with skeletal dysplasia (SD). SUMMARY OF BACKGROUND DATA: Anatomical variability and poor bone quality make upper cervical surgery technically challenging in patients with SD. We present a new cable technique for OCF in children with SD when the posterior elements are not of a size or quality for other types of instrumentation. METHODS: Retrospective review of 24 patients with SD (8 boys, 16 girls) who underwent OCF between 2001 and 2011. In this technique, cables provide compression across a bone graft that is prevented from entering the canal and the graft resists excessive lordosis. Demographic and radiographical data are presented. All patients were followed for initial outcomes of surgery, and 20 patients (83%) were followed for 2 years or more for mid- and long-term outcomes. RESULTS: Mean age at surgery was 6.5 years and mean follow-up was 4.1 ± 2.4 years. This technique was used as a primary procedure in 20 and a revision procedure in 4 patients. Diagnoses included Morquio syndrome (6), spondyloepiphyseal dysplasia (9), spondyloepimetaphyseal dysplasia (5), metatropic dysplasia (3), and Kniest syndrome (1). Ten patients had upper cervical instability and features of cervical myelopathy, and the remaining 14 patients had instability and signal changes on magnetic resonance image. Fusion extended from occiput to C2 in 71% patients, and upper cervical decompression was needed in 92% patients. Postoperatively, all patients were immobilized in a halo vest for mean duration of 12 weeks. Fusion was achieved in all patients. Complications included halo pin-tract infections (7), junctional instability (2), and extension of fusion (4). CONCLUSION: This new cable technique is a good alternative for OCF in patients with SD who have altered anatomy at the craniocervical junction not amenable to rigid internal fixation. LEVEL OF EVIDENCE: 4.

Epidemiology of surgical admissions to a children's disability hospital in Nepal.

BACKGROUND: The goal of this retrospective review was to characterize the spectrum of surgical admissions to a tertiary hospital specializing in musculoskeletal conditions in children and adolescents in Nepal. METHODS: We reviewed hospital records from 1996 to 2006 to categorize all major surgical procedures. Additional information collected included diagnosis, patient age, gender, and home district. RESULTS: Of 7,556 major surgical procedures performed from 1996 to 2006, 91% could be placed within the following categories: congenital (n = 2,984; 38%), burn contracture (n = 1,400; 19%), posttraumatic (n = 1,047; 14%), neuromuscular (n = 750; 10%), and infection (n = 731; 10%). The most common diagnosis was congenital clubfoot, which accounted for 2,348 cases (31%). Thirty-three percent of cases (n = 2,447) involved injuries (burns, fractures, dislocations). While burns most commonly involved the upper extremity, fractures in the elbow region were the most frequent posttraumatic problem. The most common neuromuscular diagnosis was poliomyelitis. The sequelae of musculoskeletal sepsis represented 731 cases (10%), most commonly chronic osteomyelitis. Other diagnoses termed miscellaneous included angular deformities of the lower extremities, scoliosis and other spinal deformities, hip dysplasia, and tumors. CONCLUSIONS: The majority of surgical pathology involved injuries and congenital problems (mainly clubfoot). The presentation was delayed in most patients, and in such cases, the treatment is more complex and costly, and the desired functional outcome is difficult to achieve. In addition to preventive measures, morbidity cases could have been reduced by the timely provision of services at the primary referral level. Strengthening the delivery of basic orthopedic services at primary health care facilities may eliminate or reduce the need for complex reconstructive procedures and diminish the likelihood of permanent disability in our population.

Ponseti method for untreated idiopathic clubfeet in Nepalese patients from 1 to 6 years of age.

UNLABELLED: Although the Ponseti method has been effective in patients up to 2 years old, limited information is available on the use of this method in older patients. We retrospectively reviewed the records of 171 patients (260 feet) to determine whether initial correction of the deformity (a plantigrade foot) could be achieved using the Ponseti method in untreated idiopathic clubfeet in patients presenting between the ages of 1 and 6 years. A mean of seven casts was required, and there were no differences in the number of casts between the different age groups. Two hundred fifty (95%) of the 260 feet were treated surgically for residual equinus after a plateau in casting, and procedures included percutaneous tendo-Achilles release (n = 205 [79%]), open tendo-Achilles lengthening (n = 8 [3%]), posterior release (n = 21 [8%]), and extensive soft tissue release (posteromedial release, n = 16 [6%]). The mean dorsiflexion after removal of the last cast was 12.5 degrees for the entire group and was greater in 1 year olds compared with 3 year olds. Although all patients achieved a plantigrade foot, the importance of the mild loss of passive dorsiflexion remains to be determined. An extensive soft tissue release was avoided in 94% of patients using the Ponseti method. We intend a followup study to ascertain whether the correction is maintained. LEVEL OF EVIDENCE: Level III, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.