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1.
J Pediatr Orthop B ; 26(4): 313-319, 2017 Jul.
Article in English | MEDLINE | ID: mdl-28151779

ABSTRACT

The behavior and treatment of coxa vara and pseudarthrosis of the proximal femur secondary to sepsis is not well described. The aim of this study is to describe the pathoanatomy for coxa vara and pseudarthrosis in postseptic hips, evaluate progression of neck shaft angle (NSA), and discuss treatment. This is a retrospective case series of 20 patients (21 hips). There were 11 hips with predominant avascular necrosis of the capital femoral epiphysis without pseudarthrosis (type 1) and 10 with pseudarthrosis (type 2). The interobserver κ value was 0.79. There was a decrease in NSA from 110.3° to 99.3° during an average follow-up duration of 5.2 years (range: 2-14 years). The average change in NSA between the initial presentation and the final follow-up was 5.5° in type 1 and 17.1° in type 2. Nine patients underwent a surgical intervention. In cases where subtrochanteric valgus osteotomy was performed, the mean preoperative NSA was 94° and the mean NSA at the final follow-up was 128°; all operated pseudarthroses healed without bone grafting. Acetabuloplasty is not necessary in most cases.


Subject(s)
Arthritis, Infectious/complications , Coxa Vara/surgery , Femur Head Necrosis/surgery , Hip Joint/surgery , Pseudarthrosis/physiopathology , Adolescent , Child , Child, Preschool , Coxa Vara/diagnostic imaging , Coxa Vara/etiology , Disease Progression , Epiphyses, Slipped/surgery , Female , Femur Head Necrosis/pathology , Hip Fractures/surgery , Hip Joint/pathology , Humans , Longitudinal Studies , Male , Pseudarthrosis/complications , Retrospective Studies
2.
Am J Orthop (Belle Mead NJ) ; 45(5): E249-53, 2016.
Article in English | MEDLINE | ID: mdl-27552461

ABSTRACT

The purpose of this study is to evaluate whether the vacuum phenomenon (VP) resolves after posterolateral lumbar fusion, and whether persistence of VP is indicative of failed fusion. We retrospectively reviewed patients with degenerative lumbar spinal stenosis with instability with a positive VP on preoperative computed tomography (CT) who underwent posterolateral lumbar spinal fusion. Lumbar CT and radiographs were evaluated for the presence of VP and fusion at each level. Thirty-six positive VP levels were identified on the preoperative lumbar CT at the levels in the fusion in 18 patients. The mean age at surgery was 67.6 ± 9.4 years and mean follow-up was 1.6 ± 0.86 years. Fusion was seen at 32 levels (88.9%). Of the 15 levels where VP persisted, evidence of fusion was seen in 13 levels and pseudarthrosis was seen at 2. Of the 21 levels where VP disappeared, fusion was seen at 19 levels and pseudarthrosis was seen at 2 .There was no significant difference between the 2 groups (P > .05). We did not find an association between persistence of VP and pseudarthrosis. Persistence of VP after spinal fusion may not be an indicator of pseudarthrosis, and should not be misinterpreted as an indication for additional surgery.


Subject(s)
Lumbar Vertebrae/surgery , Pseudarthrosis/diagnostic imaging , Spinal Fusion/adverse effects , Spinal Stenosis/surgery , Aged , Female , Humans , Lumbar Vertebrae/diagnostic imaging , Male , Middle Aged , Pseudarthrosis/etiology , Retrospective Studies , Spinal Stenosis/diagnostic imaging , Tomography, X-Ray Computed , Treatment Failure , Vacuum
4.
Spine (Phila Pa 1976) ; 39(22): E1311-7, 2014 Oct 15.
Article in English | MEDLINE | ID: mdl-25299170

ABSTRACT

STUDY DESIGN: Retrospective case series. OBJECTIVE: To report the effect of repeated growing rod (GR) lengthenings on the sagittal and pelvic profile in patients with early-onset scoliosis. SUMMARY OF BACKGROUND DATA: Posterior distraction-based GRs have gained popularity as a technique for the surgical management of early-onset scoliosis. However, there are no published studies on the effect of serial GR lengthenings on sagittal balance, thoracic kyphosis (TK), lumbar lordosis (LL), and pelvic parameters. METHODS: We retrospectively reviewed data from a multicenter early-onset scoliosis database. Forty-three patients who were able to walk with minimum 2-year follow-up who underwent single- or dual-GR surgery were included for review. Mean number of lengthenings was 6.4 (range, 3-16). Mean preoperative age was 5.6 years (standard deviation, 2.4 yr), and mean follow-up was 3.5 years. Maximum TK, LL, and sagittal balance were assessed preoperatively, after index surgery, and at the latest follow-up. RESULTS: There was a significant decrease both in TK and LL after index surgery, which then increased during the lengthening period. There was a significant increase in both proximal junctional kyphosis and distal junctional angle. Pelvic parameters (pelvic tilt, pelvic incidence, sacral slope) were unchanged during the treatment period. Significant improvement was observed in sagittal balance. There was a correlation between the change in TK and change in LL. CONCLUSION: TK decreased after index surgery and increased between the index surgery and the latest follow-up, which was accompanied by an increase in LL. All-screw proximal constructs had mean 9° more proximal junctional kyphosis than all-hook proximal constructs. An increase in proximal junctional kyphosis and distal junctional angle was found during the treatment period. Although there was an independent effect of number of lengthenings on TK, there was no significant detrimental effect on other sagittal spinopelvic parameters. GRs had a positive effect on sagittal vertical axis, which returned patients to a more neutral alignment through the course of treatment. LEVEL OF EVIDENCE: 4.


Subject(s)
Internal Fixators , Scoliosis/diagnostic imaging , Scoliosis/surgery , Spine/diagnostic imaging , Age Factors , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Kyphosis/diagnostic imaging , Lordosis/diagnostic imaging , Lumbar Vertebrae , Male , Osteogenesis, Distraction , Pelvic Bones/diagnostic imaging , Postural Balance , Radiography , Retrospective Studies , Spine/growth & development , Spine/surgery , Thoracic Vertebrae
5.
Spine (Phila Pa 1976) ; 39(5): E353-9, 2014 Mar 01.
Article in English | MEDLINE | ID: mdl-24365895

ABSTRACT

STUDY DESIGN: Retrospective cohort (case only). OBJECTIVE: To evaluate the results and survival determinants of 21 patients with sacral chordomas treated with en bloc resection and adjuvant radiotherapy. SUMMARY OF BACKGROUND DATA: There are few long-term studies on treatment of sacral chordomas with more than 20 patients, and factors related to survival are not fully understood. METHODS: Demographics, treatment, complications, and oncological outcomes were analyzed with summary statistics, hypothesis testing with Mantel-Haenszel-Cox analysis, log-rank test, Cox proportional hazard model, and Kaplan-Meier survival estimates as applicable. RESULTS: There were 12 males and 9 females with mean age of 61 years (16-79) and mean follow-up of 5.8 years (2-19.2). Tumor stage was IB in 20 and IIIB in one; mean tumor size was 10.5 cm. Fourteen patients underwent combined anterior-posterior resection and 7 posterior resection alone; 18 received adjuvant radiotherapy. After treatment, bowel and bladder control were present in 4 and 5 patients, respectively. Complications included: wound infection (4), other wound complications (9), fistula (2), deep vein thrombosis (1), and pulmonary embolism (1). Median survival was 7.2 years. Eight (40%) had local recurrence and 4 (19%) metastatic disease. Mean disease-free interval before recurrence was 2.5 years (1-5). No patient (n = 8) treated in the past 9 years has had local or distant disease. Patients treated for recurrent tumor survived 5.7 years on average (range, 0.8-9) after the first recurrence. The only risk factor for tumor recurrence was proximal tumor extent (P = 0.05) There was a statistically significant association between recurrence and survival (RR = 3.8; 95% confidence interval, 1.0-15.3; P = 0.04). CONCLUSION: Despite the complications, increased long-term survival can be achieved with treatment. Proximal tumor extent may be related to recurrence and survival. Recurrence rates have diminished over time, emphasizing the importance of an experienced multidisciplinary surgical team. LEVEL OF EVIDENCE: N/A.


Subject(s)
Chordoma/surgery , Sacrum/surgery , Spinal Neoplasms/surgery , Adolescent , Adult , Aged , Chordoma/radiotherapy , Combined Modality Therapy , Female , Fistula/etiology , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Outcome Assessment, Health Care/methods , Outcome Assessment, Health Care/statistics & numerical data , Postoperative Complications/etiology , Proportional Hazards Models , Radiotherapy, Adjuvant/adverse effects , Retrospective Studies , Sacrum/radiation effects , Spinal Neoplasms/radiotherapy , Time Factors , Venous Thrombosis/etiology , Wound Infection/etiology , Young Adult
6.
J Pediatr Orthop ; 33(6): 667-71, 2013 Sep.
Article in English | MEDLINE | ID: mdl-23812135

ABSTRACT

BACKGROUND: The purpose of this study was to identify the clinical pediatric orthopaedic articles with at least 100 citations published in all orthopaedic journals and to examine their characteristics. METHODS: All journals dedicated to orthopaedics and its subspecialties were selected from the Journal Citation Report 2001 under the subject category "orthopedics." Articles cited 100 times or more were identified using the database of the Science Citation Index Expanded (SCI-EXPANDED, 1900 to present). The articles were ranked in a comprehensive list. Two authors independently reviewed the full text of each article and applied the inclusion and exclusion criteria to the list of articles. The 2 lists were then compared. All disagreements were resolved by consensus with input from the senior author. The final list of pediatric orthopaedic articles was then compiled. RESULTS: There were a total of 49 journals under the search category "orthopedics." Five journals were excluded as they were non-English journals. The remaining 44 journals were screened for articles with at least 100 citations. A total of 135 clinical pediatric orthopaedic articles cited at least 100 times were included. The most cited article was cited 692 times. The mean number of citations per article was 159 (95% confidence interval, 145-173). All the articles were published between 1949 and 2001, with 1980 and 1989 producing the most citation classics (34). The majority (90) originated from the United States, followed by the United Kingdom (12) and Canada (11). Scoliosis/kyphosis was the most common topic with 26 papers. The second most common subject was hip disorders (24). Therapeutic studies were the most common study type (71). Ninety-seven papers were assigned a 4 for level of evidence. CONCLUSIONS: The list of citation classics in pediatric orthopaedic articles is useful for several reasons. It identifies important contributions to the field of pediatric orthopaedics and their originators; it facilitates the understanding and discourse of modern pediatric orthopaedic history and reveals trends in pediatric orthopaedics.


Subject(s)
Orthopedics , Periodicals as Topic/statistics & numerical data , Bibliometrics , Child , Databases, Bibliographic , Humans , Pediatrics
7.
Spine J ; 13(11): 1485-92, 2013 Nov.
Article in English | MEDLINE | ID: mdl-23800823

ABSTRACT

BACKGROUND CONTEXT: Surgeons continue to debate the need for a cross-link (CL) in posterior spinal instrumentation constructs with segmental pedicle screws in adolescent idiopathic scoliosis (AIS). Advantage of CLs is increased stiffness of the construct, and disadvantages include added expense and risk of late operative-site pain and pseudarthrosis. PURPOSE: To compare the effectiveness of using CLs versus using no cross-links (NCLs) in posterior segmental instrumentation in AIS. STUDY DESIGN: Retrospective comparative study, level of evidence 3. PATIENT SAMPLE: Seventy-five AIS patients less than 21 years of age, who underwent posterior spinal instrumentation with segmental pedicle screws (25 with CLs and 50 with NCLs) at a single institution with 2-year follow-up, are described. OUTCOME MEASURES: Physiologic measures include imaging: thoracic and lumbar Cobb angles, correction rate, apical vertebral translation (AVT), and apical vertebral rotation (AVR); self-report measures include Scoliosis Research Society (SRS) domain outcome scores. METHODS: Preoperative (pre-op) and postoperative first erect, 1-year, and 2-year follow-up radiographs were measured. Instrumentation-related complications and normalized SRS scores were recorded. Independent sample t test, χ(2) test, and repeated-measures analysis of variance were used for analyses. RESULTS: The average age at surgery was 14 years, the mean pre-op Cobb angle was 57°, and the mean number of levels fused was 10.9. The groups were similar preoperatively with respect to age, sex, Lenke curve, Cobb angle, AVT, and Risser grade and were similar intraoperatively for levels fused and anchor density. There was no difference in AVR, Cobb angle, correction rate, or AVT between the groups (p>.05). Complications included one wound infection in the CL group and one painful scar in the NCL group. There were no differences in SRS domain scores. CONCLUSION: We observed no differences in maintenance of correction, SRS scores, and complications with or without cross-linking posterior segmental instrumentation in AIS patients over 2-year follow-up. Further follow-up is necessary.


Subject(s)
Kyphosis/surgery , Lumbar Vertebrae/surgery , Scoliosis/surgery , Spinal Fusion/instrumentation , Thoracic Vertebrae/surgery , Adolescent , Child , Female , Humans , Kyphosis/diagnostic imaging , Lumbar Vertebrae/diagnostic imaging , Male , Patient Satisfaction , Radiography , Retrospective Studies , Scoliosis/diagnostic imaging , Spinal Fusion/methods , Thoracic Vertebrae/diagnostic imaging , Treatment Outcome
8.
J Pediatr Orthop B ; 22(1): 59-62, 2013 Jan.
Article in English | MEDLINE | ID: mdl-23010763

ABSTRACT

The gait in children with Morquio syndrome (MPS IV) has not been previously described. We reviewed the charts, gait analysis reports, and radiographs of nine children with no previous lower extremity surgery. Children with MPS IV had a slower walking speed, reduced cadence, and reduced stride length as compared with normal (P<0.05). There was increased knee flexion, genu valgus, and external tibial torsion during stance (P<0.05). Kinetics showed that knee varus moment was increased (P<0.05). There was a strong correlation between genu valgus measured on gait analysis and standing radiographs (r=0.89).


Subject(s)
Gait , Lower Extremity Deformities, Congenital/physiopathology , Mucopolysaccharidosis IV/physiopathology , Child , Humans , Lower Extremity Deformities, Congenital/etiology , Mucopolysaccharidosis IV/complications , Retrospective Studies
9.
J Pediatr Orthop ; 33(1): 63-7, 2013 Jan.
Article in English | MEDLINE | ID: mdl-23232382

ABSTRACT

BACKGROUND: Serial cast correction is a popular treatment option for progressive infantile scoliosis. Body casting can lead to chest and abdominal expansion restriction and result in decreased chest wall compliance. There are no studies evaluating the effects of casting on ventilation in infantile scoliosis. This study examines changes in peak inspiratory pressure (PIP) during serial casting for infantile scoliosis. METHODS: We retrospectively reviewed data obtained from 37 serial Cotrel elongation, derotation, and flexion cast corrections in patients with infantile scoliosis. Patient demographics, radiographic measurements, and anesthesia data were recorded. Anesthesia technique was standardized: children were intubated with rigid endotracheal tubes (ETTs); tidal volume was held constant at 8 to 10 cm(3)/kg using volume control ventilation; and PIP was recorded at baseline, after cast application before window cutout, and after window cutout before extubation. Any complications were documented. We assessed the PIP changes with a repeated measures analysis of variance (ANOVA). RESULTS: The mean age at first casting was 21.8 months (range, 12 to 42 mo) and mean follow-up since first casting was 22.4 months (range, 13 to 40 mo) with mean major Cobb angle of 53±15 degrees. The mean PIP was 15.5±4.9 cm H(2)O before casting, 31.9±7.9 cm H(2)O after cast application, and 20.4±5.6 cm H2O after making windows. There was a 106% increase after casting and 32% increase after window cutout from the baseline PIP levels. There was a significant difference in PIP on repeated measures ANOVA (P<0.0001). Intraoperatively, there was difficulty in maintaining ventilation during 2 procedures and 1 hypotensive episode. One patient developed hypoxemia after casting and another had delayed difficulty in breathing. CONCLUSIONS: Casting resulted in an increased PIP due to transient restrictive pulmonary process; after windows were cut out, the PIP reduced but not to baseline. In patients with underlying pulmonary disease, the casting process may induce respiratory complications, and a proper period of observation after casting is necessary. LEVEL OF EVIDENCE: Case series, level 4.


Subject(s)
Casts, Surgical , Inhalation/physiology , Scoliosis/surgery , Child, Preschool , Female , Humans , Infant , Male , Retrospective Studies
10.
J Pediatr Orthop ; 33(1): 91-8, 2013 Jan.
Article in English | MEDLINE | ID: mdl-23232386

ABSTRACT

BACKGROUND: Skeletal dysplasias may be associated with cervical spinal instability or stenosis. Cervical spine flexion-extension plain radiographs in children with skeletal dysplasia are difficult to interpret. The purpose of this study was to review the indications, efficacy, and safety of performing flexion-extension magnetic resonance imaging (MRI) under sedation/anesthesia in these children. METHODS: Retrospective, Institutional Review Board-approved review of 31 children with skeletal dysplasia who underwent 38 cervical spine flexion-extension MRI studies under sedation/anesthesia. Indications included abnormal neurological examination, suspected instability, stenosis, or inconclusive findings on flexion-extension radiographs. Studies were performed by the radiology technologist as directed by the radiologist with an anesthesiologist present. MRI was evaluated for odontoid hypoplasia, os odontoideum, cerebrospinal fluid effacement, cord compression, spinal cord changes, cervical canal narrowing in the neutral, flexion, and extension positions. Neurological examinations were recorded before and after MRI to assess safety. RESULTS: The average age at MRI was 3 years, 2 months. In 6 patients whose plain radiographs showed C1-C2 or subaxial instability, flexion-extension MRI showed no cord compression. Nine patients with inconclusive plain radiographs had abnormal MRI findings. An os odontoideum not seen on plain radiographs was diagnosed in 3 patients on flexion-extension MRI. On the basis of the MRI findings, 14 patients underwent surgery, 9/14 had increased cord compression in flexion or extension compared with neutral, and observation was continued in 17 others. Patients who underwent surgery had significant cord compression on MRI. There were no significant changes in the neurological examinations after MRI. CONCLUSIONS: Cervical spine flexion-extension MRI under sedation/anesthesia in children with skeletal dysplasia is safe under adequate supervision and is necessary to guide accurate medical and surgical decision making. Flexion-extension MRI is useful for identifying dynamic changes in canal diameter resulting in cord compression not seen on plain radiographs, and it is also useful for identifying patients with suspected plain film instability who may not have stenosis or cord compression on MRI. STUDY DESIGN: Level IV-retrospective case series.


Subject(s)
Bone Diseases, Developmental/diagnosis , Cervical Vertebrae , Magnetic Resonance Imaging/methods , Patient Positioning , Spinal Diseases/diagnosis , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Magnetic Resonance Imaging/adverse effects , Male , Patient Positioning/adverse effects , Patient Positioning/methods , Retrospective Studies
11.
Spine Deform ; 1(3): 196-204, 2013 May.
Article in English | MEDLINE | ID: mdl-27927293

ABSTRACT

STUDY DESIGN: Review of prospective database. OBJECTIVES: To report the results of Ponte osteotomy with pedicle screw instrumentation for major thoracic adolescent idiopathic (AIS) curves. SUMMARY OF BACKGROUND DATA: Ponte osteotomy for achieving coronal and sagittal correction of major thoracic curves in AIS with pedicle screw instrumentation is a widespread technique, but results have not been well described. METHODS: Review of 87 consecutive AIS patients with Lenke 1-4 curves who underwent Ponte osteotomies and pedicle screw instrumentation by 2 surgeons at a single institution. Surgical details, blood loss, and complications were recorded. We evaluated coronal and sagittal radiological measurements and Scoliosis Research Society-22 (SRS-22) questionnaire scores over 2-year follow-up. RESULTS: The mean preoperative thoracic coronal Cobb angle was 57° ± 9.7°, fulcrum flexibility was 47.2%, and lateral Cobb angle was 17.8° ± 4°. The mean estimated blood loss (EBL), expressed as percent estimated blood volume, was 35.8 ± 20.5 mL. There was significant improvement in coronal thoracic Cobb angle, percent correction, and apical vertebral translation over 2-year follow-up (p < .05). In hypokyphotic curves, there was a significant increase in lateral thoracic T5-T12 kyphosis from 8.1° to 18.3° (p < .001). In hyperkyphotic curves, mean lateral thoracic T5-T12 kyphosis improved from 45° to 26° (p < .001). Median SRS-22 domains were higher after treatment (p < .05). Complications included significant hypotension (1), EBL greater than 75% estimated blood volume (2), and wound infection needing drainage (2). There were neuromonitoring signal changes in 7 patients but no significant neurological complications. CONCLUSIONS: In this case series of major thoracic AIS curves treated with segmental pedicle screw instrumentation and Ponte osteotomies, there was an improvement in the coronal and sagittal radiological parameters. A prospective controlled study is needed to determine whether pedicle screw instrumentation and Ponte osteotomies influence outcomes and complications.

12.
J Pediatr Orthop ; 32(5): 490-9, 2012.
Article in English | MEDLINE | ID: mdl-22706465

ABSTRACT

BACKGROUND: Spinal instrumentation in adolescent idiopathic scoliosis (AIS) aims to correct spinal deformity and maintain long-term spinal stability until bony healing is ensured. The purpose of this study was to compare the minimum 2-year postoperative radiographic and clinical results of posterior spine correction and fusion with all-hook instrumentation versus hybrid segmental instrumentation versus pedicle screw instrumentation for AIS from a single institution. METHODS: A total of 105 patients with AIS who underwent a posterior spinal fusion with segmental pedicle screw (35), hook (35), or hybrid (35) instrumentation were sorted and matched according to the following criteria: similar age at surgery, identical Lenke curve types, curve magnitude, and Risser grade. Patients were evaluated before, immediately after, and at 2 years after surgery for radiographic parameters, complications, and outcome, as well as on the basis of the Scoliosis Research Society (SRS) questionnaire. RESULTS: The age and Risser grade, major curve Cobb angle, apical vertebral rotation (AVR), apical vertebral translation (AVT), lowest instrumented vertebral tilt, global coronal and sagittal balance, lumbar lordosis, and thoracic kyphosis were determined as part of preoperative evaluation. All 3 groups showed significant differences between the preoperative and postoperative major curve Cobb angle, lowest instrumented vertebral tilt, AVT, and AVR. At the latest follow-up, lumbar lordosis, thoracic kyphosis, and global coronal and sagittal balance remained similar among the 3 groups. Major curve Cobb angle, AVT, and AVR were significantly different--the hook group's measurements were significantly higher than the other groups, but there was no difference between the pedicle screw and hybrid groups. Major curve correction rate was significantly different among all groups (screw=71.9%±13.8%, hybrid=61.4%±16.6%, hook=48.1%±19.7%) (P<0.001). The pedicle screw group had the least amount of correction loss but there was no statistically significant difference between groups (screw=2.6±6.7 degrees, hybrid=4.5±7.4 degrees, hook=4.4±6.2 degrees) (P=0.35). The hook group had the least amount of AVT correction, but the screw group and the hybrid groups were similar (pedicle=67.3%±15.5%, hybrid=57.5%±22.4%, hook=39.9%±32.5%) (P<0.001). Surgery time and blood loss were higher in the screw group. No differences in global SRS-22 scores were demonstrated between the patients treated with pedicle screw, hybrid, and hook constructs; however, the satisfaction domain was higher in the screw group at the latest follow-up. CONCLUSIONS: Pedicle screw and hybrid instrumentations offer significantly better spinal deformity correction than hook constructs in major curve coronal correction, AVT, and AVR. Patients with pedicle screw instrumentation had the greatest curve correction percentage, maintenance of this correction in the coronal and sagittal planes, and higher patient satisfaction by the SRS outcome scores. Global SRS-22 scores were similar at 2-year follow-up in all groups. LEVEL OF EVIDENCE: Therapeutic level III retrospective comparative study.


Subject(s)
Bone Screws , Orthopedic Fixation Devices , Scoliosis/surgery , Spinal Fusion/methods , Adolescent , Child , Female , Follow-Up Studies , Humans , Kyphosis/diagnostic imaging , Kyphosis/epidemiology , Lordosis/diagnostic imaging , Lordosis/epidemiology , Lumbar Vertebrae , Male , Radiography , Retrospective Studies , Scoliosis/diagnostic imaging , Scoliosis/physiopathology , Spinal Fusion/instrumentation , Surveys and Questionnaires , Thoracic Vertebrae , Time Factors , Treatment Outcome
13.
J Pediatr Orthop ; 32(5): 534-40, 2012.
Article in English | MEDLINE | ID: mdl-22706472

ABSTRACT

BACKGROUND: The modalities and results of surgical intervention in the lower extremity in children with Morquio syndrome type A [mucopolysaccharidosis-IV (MPS-IVA)] have not been well described. The aims of this study are to define the lower extremity deformities, and describe the results of intervention in MPS-IVA patients. METHODS: Retrospective chart and radiograph review of 23 MPS-IVA patients with a minimum follow-up of >2 years. Patients were divided into no intervention and surgical groups. Demographic data, surgical details, clinical results, and complications were recorded. Standard lower extremity radiographic measurements made on standing radiographs at initial presentation, preoperatively (in surgical group), and at the final follow-up were used to study the deformities and effects of hip, knee, and ankle surgery. Descriptive statistics were performed. RESULTS: There were 11 boys and 12 girls. The average age at presentation was 6.8±3.4 years and at the last visit was 13.5±5 years with a mean follow-up of 6.7±3.7 years. Progressive hip subluxation, genu valgum, and ankle valgus were observed in all patients without intervention. Twenty patients had a total of 159 lower extremity surgical procedures (average, 8 procedures per patient). There were 61 hip, 78 knee, and 20 ankle procedures. Surgery resulted in improvement of the center edge angle, femoral head coverage, lateral distal femoral angle, medial proximal tibial angle, tibiofemoral angle, and lateral distal tibial angle. Mechanical axis of the lower extremities improved after intervention. Six patients (12 hips) had recurrence of hip subluxation after acetabular osteotomies and/or femoral varus derotation osteotomy, and 8 patients (16 knees) had postoperative genu valgum recurrence requiring subsequent intervention. There was no recurrent hip subluxation after shelf acetabuloplasty. CONCLUSIONS: Progressive hip subluxation, genu valgum, and ankle valgus were seen and often needed surgery. After shelf acetabuloplasty and varus derotation osteotomy, there was no recurrent hip subluxation. Recurrence after genu valgum correction was common. LEVEL OF EVIDENCE: Level IV, therapeutic case series.


Subject(s)
Ankle Joint/abnormalities , Genu Valgum/etiology , Hip Dislocation/etiology , Mucopolysaccharidosis IV/physiopathology , Acetabulum/pathology , Acetabulum/surgery , Adolescent , Ankle Joint/diagnostic imaging , Ankle Joint/surgery , Child , Child, Preschool , Disease Progression , Female , Follow-Up Studies , Genu Valgum/diagnostic imaging , Genu Valgum/surgery , Hip Dislocation/diagnostic imaging , Hip Dislocation/surgery , Humans , Infant , Lower Extremity , Male , Mucopolysaccharidosis IV/diagnostic imaging , Osteotomy/methods , Radiography , Recurrence , Retrospective Studies
14.
J Pediatr Orthop B ; 21(3): 240-7, 2012 May.
Article in English | MEDLINE | ID: mdl-22186706

ABSTRACT

Hip dislocation is a serious but rarely reported complication of femoral lengthening in congenital short femora. We report a retrospective series of three hip dislocations that occurred during lengthening, analyze the factors contributing to the dislocation, and discuss the treatment of this difficult problem. The average lengthening achieved was 9 cm. We found progressive acetabular dysplasia and decreasing center edge angle. Closed reduction failed. Treatment included soft tissue release, open reduction with femoral shortening, and acetabular procedures. At a mean follow-up of 4.4 years, two patients had a good modified Mckay score and a modified Severin score of 3. Excessive lengthening should be avoided.


Subject(s)
Bone Lengthening/adverse effects , Femur/surgery , Hip Dislocation/etiology , Leg Length Inequality/surgery , Postoperative Complications/etiology , Acetabulum/pathology , Acetabulum/surgery , Adolescent , Bone Diseases, Developmental/etiology , Bone Diseases, Developmental/pathology , Child , Female , Femur/abnormalities , Fracture Fixation, Internal/methods , Hip Dislocation/pathology , Hip Dislocation/physiopathology , Humans , Leg Length Inequality/congenital , Leg Length Inequality/physiopathology , Male , Retrospective Studies
15.
Spine (Phila Pa 1976) ; 37(9): E549-55, 2012 Apr 20.
Article in English | MEDLINE | ID: mdl-22037532

ABSTRACT

STUDY DESIGN: Therapeutic comparative study. OBJECTIVE: To evaluate the safety and efficacy of antifibrinolytic (AF) agents in reducing blood loss and transfusions during posterior spinal fusion (PSF) in children with cerebral palsy (CP) scoliosis. SUMMARY OF BACKGROUND DATA: Scoliosis surgery in CP children is associated with substantial blood loss. Few reports on the role of AFs exist. METHODS: A multicenter, retrospective review of a prospectively collected database of 84 consecutively enrolled patients with CF (age < 18 years) with spinal deformity who underwent PSF and instrumentation. The use of AFs, tranexamic acid (TXA), epsilon-aminocaproic acid (EACA), or none was based on the surgeon preference. Estimated blood loss (EBL), transfusion requirements, and length of stay were recorded. Analysis was performed with the independent-samples t test and 1-way analysis of variance with post hoc Bonferroni analysis. RESULTS: The average age at the time of surgery was 14.4 ± 2.6 years. The groups were well matched in preoperative major deformity, age, levels fused, and operating time. Forty-four patients received AFs (30 TXA and 14 EACA), and 40 received no antifibrinolytics (NAF). The EBL averaged 1684 mL for the AFs group and 2685 mL for the NAF group (P = 0.002). There was more cell salvage transfusion in the NAF group. No significant differences were found in total transfusion requirements. There was a trend for decreased hospital stay in the AFs group. No adverse effects were seen. On comparison of the 3 groups (NAF, TXA, and EACA), a significant difference was observed between the TXA and the other groups with respect to EBL and cell salvage transfusion. CONCLUSION: AFs significantly reduced intraoperative EBL associated with PSF, with no adverse effects; however, we could not demonstrate significant differences in total transfusion, except in cell salvage. TXA was more effective than EACA in decreasing the EBL and cell salvage transfusion.


Subject(s)
Aminocaproic Acid/administration & dosage , Antifibrinolytic Agents/administration & dosage , Blood Loss, Surgical/prevention & control , Blood Transfusion , Cerebral Palsy/complications , Scoliosis/surgery , Spinal Fusion/adverse effects , Tranexamic Acid/administration & dosage , Adolescent , Aminocaproic Acid/adverse effects , Analysis of Variance , Antifibrinolytic Agents/adverse effects , Blood Transfusion, Autologous , Child , Databases, Factual , Humans , Length of Stay , Operative Blood Salvage , Retrospective Studies , Scoliosis/complications , Time Factors , Tranexamic Acid/adverse effects , Treatment Outcome , United States
16.
J Pediatr Orthop B ; 20(6): 413-21, 2011 Nov.
Article in English | MEDLINE | ID: mdl-21897298

ABSTRACT

Delayed or missed diagnosis of septic arthritis of hip in children results in various sequelae. The group of post septic hip dislocations when the capital femoral epiphysis (CFE) is present has not been described in the commonly used classifications. This is a retrospective series of 21 hips in 18 children. The presence of the CFE was confirmed radiologically or at the time of intervention. The mean follow-up after intervention was 6.3 years. Interventions for dislocations included closed reduction ± adductor tenotomy, open reduction ± supplementary femoral procedures, and acetabular procedures. Results were evaluated clinically with Ponseti hip scoring and radiologically with the modified Severin grading. Closed reduction was successful in seven of 20 hips (35%) and open reduction in 13 of 14 hips. At follow-up, good clinical result was seen in nine of 18 cases (50%). The mean neck shaft angle was 129° in all hips and 124° after femoral varus osteotomy. There was one redislocation and three subluxations. The modified Severin classification was class 2 in five hips (good), class 3 in 12 hips (fair), class 4 in three hips (poor), and class 6 in one hip (failure). Septic hip dislocation with CFE present is a distinct entity. MRI is helpful for planning treatment. A significant number of patients need open reduction with other procedures. Femoral varus osteotomy may contribute to coxa vara. In the short term, intervention results in a stable, functional, and mobile hip.


Subject(s)
Arthritis, Infectious/complications , Femur/pathology , Hip Dislocation/therapy , Child , Child, Preschool , Epiphyses/pathology , Female , Hip Dislocation/microbiology , Humans , Infant , Magnetic Resonance Imaging , Osteotomy , Retrospective Studies
17.
J Pediatr Orthop B ; 19(6): 479-86, 2010 Nov.
Article in English | MEDLINE | ID: mdl-20613643

ABSTRACT

We report the results of surgical treatment of congenital postero-medial bowing of the tibia and fibula. Twenty patients with congenital postero-medial bowing were seen with nine patients treated surgically (corrective osteotomy or lengthening and deformity correction with Ilizarov fixator) and 11 patients managed conservatively. The angles of medial and posterior angulation and limb length discrepancy were recorded serially and compared. Surgical complications were recorded. The mean follow-up was 9.5 and 6.1 years after surgery. Although there was a reduction in angulation and correction of limb length discrepancy, we encountered complications in the surgically treated patients. There was no statistically significant difference between the surgically treated and conservatively managed groups with respect to mean angulation, though there was a significant difference in the mean limb length discrepancy. In conclusion, we advocate a one-stage lengthening and correction of the residual deformity closer to skeletal maturity.


Subject(s)
External Fixators , Fibula/surgery , Genu Varum/congenital , Genu Varum/surgery , Osteotomy/methods , Tibia/surgery , Age Factors , Casts, Surgical , Child , Child, Preschool , Cohort Studies , Female , Fibula/abnormalities , Follow-Up Studies , Genu Varum/rehabilitation , Humans , Leg Length Inequality/diagnosis , Leg Length Inequality/surgery , Limb Deformities, Congenital/diagnosis , Limb Deformities, Congenital/rehabilitation , Limb Deformities, Congenital/surgery , Male , Retrospective Studies , Risk Assessment , Severity of Illness Index , Splints , Tibia/abnormalities , Treatment Outcome
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