Radiographic and clinical outcomes after definitive spine fusion for skeletally immature patients with idiopathic scoliosis.

PURPOSE: To assess the intermediate-term radiographic and clinical outcomes of skeletally immature idiopathic scoliosis (IS) patients that underwent definitive fusion (DF). METHODS: A retrospective review of patients with IS who were Risser 0 with open tri-radiate cartilages at the time of DF with minimum 5-year follow-up. Outcomes included Scoliosis Research Society (SRS)-30 scores, major Cobb angle, pulmonary function tests (PFTs), and unplanned returns to the operating room (UPROR). Adding-on was defined as progression of the major Cobb angle > 5° or tilt of the lowest instrumented vertebra > 5°. RESULTS: Thirty-two patients (78% female, mean age 12.2 ± 1.3 years old, mean preoperative major Cobb 64.8° ± 15.9) were included. Of these patients, 20 (62.5%) experienced adding-on and 6 (18.8%) required a revision surgery to correct their progressive spinal deformity. Adding-on was associated with lower 5-year postoperative SRS scores for appearance (3.7 ± 0.7 vs 4.4 ± 0.3, p = 0.0126), mental health (4.2 ± 0.6 vs 4.6 ± 0.3, p = 0.0464), satisfaction with treatment (4.0 ± 0.8 vs 4.7 ± 0.4, p = 0.0140), and total score (4.0 ± 0.4 vs 4.4 ± 0.2, p = 0.0035). The results of the PFTs did not differ between groups. Patients experienced an average of 0.53 UPROR/patient. CONCLUSION: DF in skeletally immature patients results in a high rate of adding-on, which adversely affects Health-Related Quality of Life. However, reoperation rates, both planned and unplanned, remain lower when compared to patients undergoing growth-friendly treatment.

Long-Term Outcomes at Skeletal Maturity of Combined Pelvic and Femoral Osteotomy for the Treatment of Legg-Calve-Perthes Disease.

Surgical treatment for Legg-Calve-Perthes disease (LCPD) is recommended for older children with moderate to severe disease. We sought to determine whether double osteotomies lead to improved radiologic outcomes compared to reported non-operative outcomes. Patients older than 6 years of age diagnosed with LCPD lateral pillar B or C who were treated with pelvic and femoral osteotomies were included. Radiologic outcomes and leg-length discrepancies were assessed using the Stulberg classification and were compared with the current literature. Fifteen hips in fourteen patients were treated with double osteotomy for LCPD, and seven had lateral pillar C disease (47%). The mean age at surgery was 8.6 years (range, 7.2-10.4) and the mean age at follow-up was 20.2 years (range, 14.2-35.6). At a mean 11.6-year follow-up (range: 6.3-25.2), double osteotomy resulted in 40% of patients having Stulberg I/II scores, 27% having Stulberg III scores, and 33% having Stulberg IV/V scores. The mean leg-length discrepancy was 1.4 cm in lateral pillar C patients compared to 0.8 cm in lateral pillar B patients. Four patients underwent additional surgeries, including two who required total hip arthroplasty. Double osteotomy as an alternative surgical procedure for the treatment of LCPD did not show improved outcomes when compared to historic non-operative cohorts.

Minimum 5-Year Results of Elongation Derotation Flexion Casting for Early Onset Scoliosis: The Story Is Not Over Until Skeletal Maturity.

BACKGROUND: Early conservative treatment for patients with idiopathic infantile scoliosis (IIS) with elongation derotation flexion (EDF) casting and subsequent serial bracing has become widely utilized. However, the long-term outcomes of patients treated with EDF casting are limited. METHODS: We performed a retrospective chart review of all patients who had undergone serial elongation derotation flexion casting and subsequent bracing for scoliosis presenting at a single large tertiary center. All patients were followed for a minimum of 5 years or until surgical intervention. RESULTS: Our study included 21 patients diagnosed with IIS and treated with EDF casting. At a mean 7-year follow-up, 13 of the 21 patients were considered successfully treated with a mean final major coronal curvature of 9 degrees compared to a pretreatment coronal curve of 36 degrees. These patients, on average, began casting at 1.3 years old and spent 1 year in a cast. Patients that did not have substantial improvement began casting at mean 4 years old and remained in a cast for 0.8 years. Three patients initially had substantial improvement with the correction to <20 degrees at a mean age of 7; however, their curves worsened in adolescence with poor brace compliance. All 3 patients will require surgical intervention. Of the patients not successfully treated with casting, 7 required surgery at a mean 8.2 years of age, 4.3 years after initiation of casting. A significant predictor of treatment failure was older age of cast initiation ( P <0.001). CONCLUSIONS: EDF casting can be an effective cure for IIS patients if initiated at a young age with 15 of 21 patients successfully treated (76%). However, 3 patients had a recurrence in adolescence resulting in an overall success rate of only 62%. Casting should be initiated early to maximize the likelihood of treatment success and periodic monitoring should be continued through skeletal maturity as recurrence during adolescence can occur.

Predictors of Total Hip Arthroplasty Following Pediatric Surgical Treatment of Developmental Hip Dysplasia at 20-Year Follow-Up.

Long-term outcomes of surgical treatment for pediatric developmental dysplasia of the hip (DDH) are not well defined. The purpose of this study was to report long-term radiographic and clinical outcomes, survivorship free of total hip arthroplasty (THA), and predictors of subsequent THA following childhood treatment of DDH. This study was a single-institution retrospective review of hips treated for DDH with closed or open reduction at a minimum 10-year follow-up. 107 patients (119 hips) were included with a mean patient age of 3.3 years at childhood treatment. At mean 30.5 years follow-up, 24 hips had undergone THA (20%). Mean patient age at time of THA was 33.5 years. None of the hips treated with closed reduction alone required THA, whereas 8 hips treated with open reduction (25%) underwent THA. Hips with patient age > 4 years at the time of treatment had lower survivorship at 35 years follow-up (50% vs. 85%; p < 0.001). Additionally, femoral osteotomy (OR 2.0, p < 0.001), and previous treatment elsewhere (27% vs. 16%; p < 0.01) were associated with subsequent THA. Early referral and appropriate intervention may prove important, as age and prior treatment were predictive of subsequent THA.

Primary Aneurysmal Bone Cyst of the Spine in Children: Updated Outcomes of a Modern Surgical Technique.

OBJECTIVE: Aneurysmal bone cysts (ABC) are benign but locally aggressive lesions. Treating children with spinal ABC poses risks due to the proximity of the lesion to the spinal cord and the need to preserve spinal stability after surgery. This study reports the updated outcomes of a uniform aggressive initial surgical technique for the treatment of spinal ABC in children. METHODS: Twenty-nine cases of spinal ABC were collected from a tertiary pediatric tumor center over a 24-year period (January 1990-September 2014). The study patients were divided into 2 groups based on the performed procedure: a traditional approach consisting of curettage and bone grafting (group 1) and a 4-step approach consisting of intralesional curettage, high-speed bur, electrocautery, and bone grafting (group 2). RESULTS: The study population was composed of 12 males and 17 females with a mean age of 12.2 years at the time of diagnosis and a mean postoperative follow-up of 4.03 years. Twenty-one patients underwent the 4-step approach, while 8 patients were treated with the traditional technique. The recurrence rate was 50% (4/8 patients) with the traditional technique (group 1) and 19% (4/21) in the 4-step technique (group 2) (P = .164). Regardless of the technique used, the presence of sensory symptoms at the time of presentation was a statistically significant factor for recurrence (P = .016). CONCLUSIONS: We suggest that addressing spinal ABC in pediatric patients with a 4-step approach is a safe technique that may decrease long-term recurrence and reoperation rates compared to the traditional technique of intralesional curettage and bone grafting. LEVEL OF EVIDENCE: 4.

Surgical resection of osteoid osteoma and osteoblastoma of the spine.

Intraoperative radiographic guidance has traditionally been utilized in orthopedic surgery through 2-D navigation with the C-arm and recently with 3-D navigation with the O-arm. The aim of this study was to describe the outcome of surgical treatment of spinal osteoblastoma and osteoid osteoma with the utilization of the O-arm and conventional C-arm guidance. This is a retrospective cohort study of patients with spinal osteoid osteoma and or osteoblastoma who were treated at our institution between 2002 and 2011. Seventeen patients were examined in this study including seven with spinal osteoblastoma and 10 with spinal osteoid osteoma. The mean age of the patients at surgery was 11.5±3.9 years. The O-arm was used in seven patients and the C-arm in 10 patients. The C-arm failed to identify the tumor in one case and needed transport to perform a computed tomographic-scan. The length of surgery was shorter when the O-arm was used, especially in the osteoblastoma group. Thirteen patients were pain free at the last follow-up visit and two patients developed recurrence. Radiographs at the last follow-up did not show signs of vertebral instability following tumor resection. Safe and effective localization of spine tumors and confirmation of tumor removal during surgery was achieved by intraoperative radiographic guidance specifically with the O-arm 3-D navigation system. LEVEL OF EVIDENCE: III.

Comparative results of percutaneous Achilles tenotomy to combined open Achilles tenotomy with posterior capsulotomy in the correction of equinus deformity in congenital talipes equinovarus.

PURPOSE: The purpose of this study is to compare the efficacy of percutaneous Achilles tenotomy (AT) to combined open Achilles tenotomy and posterior capsulotomy (PC+AT) in the correction of residual equinus deformity in congenital talipes equinovarus after Ponseti serial casting in both idiopathic and non-idiopathic clubfeet. METHODS: The authors retrospectively reviewed 591 patients treated for congenital talipes equinovarus between January 1, 2001 and January 1, 2011. Available medical and operative records were reviewed for basic demographic data as well as ankle dorsiflexion pre-operatively, postoperatively and at latest follow up. RESULTS: A total of 167 children with 260 discrete clubfeet that met our inclusion criteria were identified. Of them, 189/260 clubfeet (72.7 %) were idiopathic and 71/260 clubfeet (27.3 %) were non-idiopathic with a mean total follow up of 4.8 ± 2.4 years (minimum follow-up of two years). At latest follow up, there was no statistically significant difference in the mean ankle dorsiflexion (p = 0.333) or recurrence rate (p = 0.545) between PC+AT and AT groups in both idiopathic and non-idiopathic clubfeet. CONCLUSION: In our series, the addition of posterior capsulotomy to Achilles tenotomy did not improve the mean dorsiflexion at latest follow up or decrease the rate of recurrence of equinus deformity in both idiopathic and non-idiopathic clubfeet. It is therefore advisable that percutaneous Achilles tenotomy alone be used in the correction of equinus deformity in both idiopathic and non-idiopathic congenital talipes equinovarus after successful Ponseti serial casting.

Postural orthostatic tachycardia syndrome (POTS): association with Ehlers-Danlos syndrome and orthopaedic considerations.

BACKGROUND: Postural orthostatic tachycardia syndrome (POTS) is the most common of several types of dysautonomia, characterized by dysfunction of the autonomic nervous system manifesting with symptoms of orthostatic intolerance with or without associated orthostatic hypotension and excessive autonomic excitation. Given the numerous presenting musculoskeletal symptoms of POTS and its known associations with other clinical entities like Ehlers-Danlos syndrome, POTS constitutes an unusual treatment challenge of which the orthopaedic surgeon and other related healthcare providers should be aware. CASE DESCRIPTION: We describe two patients with a diagnosis of POTS and musculoskeletal manifestations. The first is a 13-year-old boy with a concurrent diagnosis of Ehlers-Danlos syndrome and worsening back pain. The patient had resolution of his musculoskeletal symptoms after treatment with a Boston Overlap LSO brace and physical therapy and resolution of his cardiovascular symptoms after pharmacologic treatment. The second patient is a 17-year-old girl with an L1 vertebral hemangioma and pain of sudden onset radiating to her lower extremities. Her symptoms were controlled with therapy and gabapentin. LITERATURE REVIEW: POTS is a poorly understood and controversial clinical entity with lack of awareness and knowledge regarding the syndrome by the majority of medical specialists. Its complex and wide range of clinical presentation has led to being commonly mistaken with malingering, depression and anxiety disorders. Review of the literature did not yield any previous studies addressing POTS and the special considerations for the orthopaedic surgeon caring for these patients; previous studies of large patient series focus on investigating the pathologic substrate of POTS and the efficacy of various treatment modalities on the cardiovascular dysregulation. CLINICAL RELEVANCE: These two cases show that understanding and knowledge of this syndrome and its comorbidities should guide any diagnostic approach or therapeutic intervention by the orthopaedic surgeon caring for patients with this syndrome. It is important that the surgeon is aware of the benefits of detailed patient education and physical conditioning, the increased perioperative complications, and the need for specialized anesthesia.

Synovial chondromatosis of the spine in the skeletally immature: case report and updated review of the literature.

Synovial chondromatosis is a benign disorder characterized by the formation of multiple cartilaginous nodules in the synovium of large joints. Synovial chondromatosis of the spine is very rare; this is the first reported case in the skeletally immature pediatric spine. A 12-year-old female presented with a posteriolateral neck mass and discomfort. A two-stage surgical resection of the mass was performed. This case shows that synovial chondromatosis can occur in the immature pediatric spine. Synovial chondromatosis should be included in the differential diagnosis of a calcified paravertebral or lateral neck mass in children.

Scoliosis in Children With Aicardi Syndrome.

BACKGROUND: Aicardi syndrome (ACS) is a rare neurodevelopmental disorder that was classically characterized by the triad of agenesis of corpus callosum, infantile spasms, and chorioretinal lacunae. As new cases emerge and new common phenotypic features are being described in subsequent reports, new modified diagnostic criteria have been proposed that now classify the observed costovertebral abnormalities as supporting diagnostic features. To our knowledge there are no previous studies focusing and describing the scoliosis observed in children with ACS. METHODS: We screened billing lists to identify patients seen in the Division of Orthopaedic Surgery at our institution with a diagnosis of ACS that were treated for scoliosis after 2001. A total of 5 patients were identified. Medical records and radiographs were retrospectively reviewed in all cases. In all of the patients the diagnosis of ACS had been confirmed through complete genetic evaluation and advanced imaging. RESULTS: The mean age when scoliosis was first noticed was 3.9±4.2 years (range, 0.5 to 10.5 y) with a mean Cobb angle of 22.5±6.7 degrees (range, 10 to 27 degrees). The mean age at the first orthopedic visit was 5.8±5.0 years (range, 1.5 to 13 y) with a progressed mean Cobb angle of 39.5±17.3 degrees (range, 15 to 57 degrees). Congenital vertebral anomalies were observed in 1 patient. Three patients were treated surgically; 1 of the 3 patients had a surgical complication with loss of intraoperative neuromonitoring signals. Two patients had not undergone surgery at the last visit with a mean Cobb angle of 75.5 degrees. The mean postoperative follow-up for the surgical group (cases 1 to 3) was 3±3.6 years (range, 0.6 to 7.2 y) and the mean total follow-up for both groups was 6.6±2.5 years (range, 2.6 to 8.6 y). CONCLUSIONS: Scoliosis in ACS can represent a clinically significant problem that is underdiagnosed and overshadowed by the other severe medical complications associated with the syndrome. Our data suggest that scoliosis in patients with ACS is rapidly progressive and bracing is ineffective; early screening, close observation, and low threshold for referral to an orthopedic surgeon are crucial. LEVEL OF EVIDENCE: Level IV-case series.