Pediatric Musculoskeletal Coccidioidomycosis in Central California: A Single Center Experience.

BACKGROUND: Published literature on musculoskeletal coccidioidomycosis is sparse and limited to case reports and case series. This is one of the largest case series to describe the clinical presentation, diagnosis, medical and surgical management and outcomes of pediatric musculoskeletal coccidioidomycosis at a tertiary care hospital. METHOD: A retrospective case review was performed on patients ≤ 21 years old who were followed at a tertiary care center with a diagnosis of musculoskeletal coccidioidomycosis from January 1, 2007, to December 31, 2020. Descriptive data are expressed as medians and interquartile range (IQR) for continuous variables or as frequency and percentage for categorical variables. Categorical values were compared using the χ2 test. RESULTS: Forty-one patients were identified. The median age was 12.8 years, and most were male (71%), Latinx (66%) and healthy (71%). Limb swelling (66%), bone pain (54%) and joint pain (46%) were the most common presenting symptoms. Multiple bone involvement was present in 29% while 12% had the joint disease, and craniofacial (n = 10) and metacarpal/metatarsal bones (n=9) were the most commonly involved sites. Elevated Coccidioides complement fixation (CF) titers ≥1:32 were seen in 90% of the patients. Thirty-three patients (81%) required surgical interventions and of these 16 (48%) required additional surgical procedures. Eleven patients (27%) had disease relapse. Children >13 years of age were more likely to have > 1 organ involvement (16 vs. 7, P = 0.04), multiple bone involvement (10 vs. 2, P = 0.004) and maximum Coccidioides CF titers >1:128 (13 vs. 6, P = 0.02). CONCLUSIONS: In endemic areas, musculoskeletal coccidioidomycosis causes a substantial disease burden in children and should be considered in the differential diagnosis of those presenting with bone and joint pain or swelling. Early diagnosis and treatment are essential to minimize long-term morbidity and mortality.

Unilateral hip reconstruction in children with cerebral palsy: predictors for failure.

BACKGROUND: Unilateral hip reconstruction in patients with cerebral palsy can be complicated by contralateral subluxation and ipsilateral failure. We sought to identify predictors for failure after unilateral reconstruction in patients with GMFCS IV-V CP with unilateral hip involvement. METHODS: We performed an IRB-approved retrospective study on GMFCS IV-V CP patients with unilateral hip reconstruction at a minimum 2-year follow-up. Radiologic data included acetabular index, femoral migration index (FMI), lateral center edge angle (LCE), and pelvic obliquity. The effects of age, sex, pelvic obliquity, scoliosis surgery, and contralateral hip soft-tissue release at the index surgery were analyzed for ipsilateral hip failure and contralateral hip subluxation. Statistical analysis was performed using the χ and t tests. RESULTS: There were 35 patients (M:F, 23:12) with mean age of 110 months (range, 45 to 215 mo) with mean follow-up of 60.5 months (range, 24 to 129 mo). The mean preoperative ipsilateral hip FMI was 60% (range, 40% to 100%) and the mean LCE was -16.7 degrees (range, -85 to 17.2 degrees). Contralateral soft-tissue release was performed in 13/35 patients. Ipsilateral hip failure or contralateral hip subluxation was observed in 51% (18/35) patients. Contralateral hip subluxation developed in 28% (10/35) of patients. Ipsilateral hip failure was observed in 34% (12/35) patients. Four had both ipsilateral failure and contralateral subluxation. Lack of contralateral hip soft-tissue release, reversal of pelvic obliquity angle, and high initial contralateral hip FMI (> 25%) significantly predicted the risk of contralateral hip subluxation (P = 0.03). Similarly, persistence or worsening of preoperative pelvic obliquity significantly predicted ipsilateral hip failure (P < 0.04). There was a strong trend toward contralateral hip subluxation in patients below 8 years of age (P = 0.1) and ipsilateral hip failure in those who had spinal fusion surgery for scoliosis (P = 0.06). CONCLUSIONS: Predictors of contralateral hip subluxation included lack of contralateral soft-tissue release, reversal of pelvic obliquity angle, and larger initial contralateral hip FMI (> 25%). The only predictor of ipsilateral failure was persistence or worsening of preoperative pelvic obliquity.

Predictors for secondary procedures in walking DDH.

BACKGROUND: Persistent or recurrent hip dysplasia and/or loss of reduction can complicate the treatment of developmental dysplasia of the hip (DDH) in walking children. In this study, we identify predictors for secondary procedures after open reduction of the hip in walking children with DDH. METHODS: We performed a retrospective study of walking children with idiopathic DDH treated with open reduction of the hip and followed up for >5 years. Perioperative factors were analyzed to investigate predictors of reoperation. Factors associated with the need for secondary procedures were identified. Acetabular remodeling was analyzed with a graphical plot of serial (0, 6 and 12 mo, and yearly) mean acetabular index and SD. RESULTS: There were 49 open reductions of the hip in 42 patients (34 female, 8 male) at a mean age of 31.3 months (range, 15.3 to 92.6 mo), with a mean follow-up of 9.7 years (5 to 16.9 y). Twelve (24%) patients had open reduction only, 15 (31%) had concurrent pelvic osteotomy, 4 (8%) had femoral osteotomy, and 18 (37%) had both femoral and pelvic osteotomy. Four (8%) patients required repeat open reduction at a mean of 5.1 months (range, 4 to 7.5 mo) after index surgery. Twenty-four (49%) patients had at least 1 secondary surgery for dysplasia at a mean of 3.2 years after index surgery. Six of the 24 (25%) had 2 and 2/24 (8.3%) had 3 additional operations. Of the 27 patients who did not have concurrent femoral osteotomy at index surgery, 19/27 (73%) required a secondary procedure; this significantly predicted the need for reoperation (P<0.001). Only 5/22 patients with femoral osteotomy at index surgery required a secondary procedure. Maximum acetabular remodeling was observed in the first 4 years after primary reduction, and the mean acetabular index remodeled from 43.9 to 20.3 degrees during this period. CONCLUSIONS: Forty-nine percent of the patients in this cohort required secondary procedures to treat hip dysplasia. Open reduction without concurrent femoral osteotomy strongly predicted the need for a secondary procedure. Maximum acetabular remodeling was observed in the first 4 years after open reduction. LEVEL OF EVIDENCE: Retrospective case series, level IV.

Correlation of magnetic resonance imaging and histologic examination of physeal bars in a rabbit model.

BACKGROUND: The formation of a physeal bony bridge, or bar, is frequently observed in pediatric patients after trauma and is visualized using magnetic resonance imaging (MRI). No study to date has validated the indirect MRI bar area measurements with direct measurements. PURPOSE: To create a physeal bar using a radiofrequency (RF) ablation technique in a rabbit model and to validate an indirect measurement of the bar area from MRIs with direct histologic measurements. METHODS: An epiphysiodesis procedure was performed on the proximal tibial growth plates of 15 skeletally immature rabbit knees using radiofrequency ablation. The rabbits were euthanized 6 weeks postoperatively and volumetric ex vivo MRIs of the knees were acquired. The physeal bar area was calculated from a 3-dimensional reconstruction of the segmented MRIs and from matching histologic sections of the tibia. RESULTS: A physeal bar was successfully created in all the rabbits. A strong correlation, r=0.83 (P=0.0001), was found between the MRI and histologic surface area measurements. The mean bar area from MRI measurements was 35.0 ± 20.8 mm² (mean ± SD), and the mean bar area from histologic measurements was 29.8 ± 16.1 mm². CONCLUSIONS: This study found excellent correlation between the MRI and histologic physeal bar area measurements. The measurement differences that were found may be attributed to histologic specimen preparation and the geometry chosen to model the physis. CLINICAL RELEVANCE: The results of this study allow for the quantitative evaluation of in vivo human physes in future studies and development of predictive models for limb length discrepancy.

Diminished spinal cord size associated with congenital scoliosis of the thoracic spine.

BACKGROUND: Intraspinal anomalies are common in patients with congenital scoliosis and are a known risk factor for neurological complications. Smaller but normal-appearing spinal cords have not been described in association with congenital scoliosis, nor have they been considered to be an anomaly with a similar neurological risk. We previously reported on small spinal cords associated with Klippel-Feil syndrome. We hypothesized that patients with congenital thoracic scoliosis would similarly have smaller spinal cords as compared with normal controls, with a potentially increased risk for myelopathy. METHODS: We reviewed thirty patients with congenital scoliosis (including fifteen patients with failure of vertebral formation and fifteen with failure of vertebral segmentation). All patients had adequate axial T2-weighted, digitally formatted magnetic resonance imaging scans that were suitable for cross-sectional measurement. Cross-sectional areas of the spinal cord and spinal canal at each of the twelve thoracic levels were calculated three times by a blinded observer, and the average values were compared with measurements from age-matched normal controls. RESULTS: We observed a significantly smaller mean cross-sectional area at all levels of the spinal cord in the study group as compared with the control group (p < 0.01). When the pathological segments were evaluated, both with and without the inclusion of the adjacent normal segments, the spinal cord was smaller for the study group (p < 0.01). Within the study group, small spinal cords were observed in patients with failed vertebral formation as well as in patients with failed vertebral segmentation, but without an identifiable difference between these two subgroups. The spinal canal was also smaller for the patients in the study group, but not in all cases. No patient showed a reduction of, or encroachment on, the space available for the spinal cord. Spinal cord size increased with age in both the study group and the control group but remained consistently smaller in the study group. Within the study group, the presence of intraspinal anomalies did not appear to influence the spinal cord size. CONCLUSIONS: The spinal cord is significantly smaller in patients with congenital thoracic scoliosis than in normal controls. Because small spinal cords have been reported to be associated with neurological sequelae, we advise that the diminished spinal cord size be considered an important component of intraspinal anomalies associated with congenital scoliosis.

Slipped capital femoral epiphysis update.

PURPOSE OF REVIEW: Slipped capital femoral epiphysis (SCFE) is the most common adolescent hip condition. Its importance lies in its high morbidity if not diagnosed and treated in its early stages, not only in childhood but also as a cause of osteoarthritis in adulthood. This article highlights key diagnostic tools and optimal treatment plans for SCFE. RECENT FINDINGS: SCFE involves displacement between the proximal femoral neck and the femoral head at the level of the open physis, with biomechanical and biochemical factors implicated. Acute major trauma is rarely involved; a gradual onset of symptoms and deformity is more common. Patients with unstable SCFE are in severe pain and unable to bear weight. SCFE occasionally is associated with endocrine or metabolic abnormality (hypothyroidism, panhypopituitarism and renal rickets). On physical examination, limited internal rotation of the affected hip is usual; obligatory external rotation of hip in flexion is classic. Diagnosis is confirmed on anteroposterior and frog-leg lateral radiographs of both hips. Treatment is surgical, with stabilization across the physis by in-situ pinning being the gold standard. SUMMARY: Prompt diagnosis and timely surgical treatment usually lead to excellent long-term results with minimal morbidity. It is crucial to recognize that groin pulls are very rare in adolescents. Children with suggestive groin symptoms should have hip anteroposterior and frog-leg lateral radiographs to rule out the much more common SCFE.

Down syndrome: orthopedic issues.

PURPOSE OF REVIEW: The purpose of this review is to update the role of the orthopedic surgeon in the management of Down syndrome as these patients are living longer and participating in sporting activities. RECENT FINDINGS: Approximately 20% of all patients with Down syndrome experience orthopedic problems. Upper cervical spine instability has the most potential for morbidity and, consequently, requires close monitoring. Other conditions such as scoliosis, hip instability, patellar instability and foot problems can cause disability if left untreated. In some of these conditions, early diagnosis can prevent severe disability. SUMMARY: Surgical intervention in children with Down syndrome has a high risk of complications, particularly infection and wound healing problems. Careful anesthetic airway management is needed because of the associated risk of cervical spine instability.

Advanced imaging of the cervical spine and spinal cord in 22q11.2 deletion syndrome: age-matched, double-cohort, controlled study.

PURPOSE: The 22q11.2 deletion syndrome is a common genetic syndrome with a wide spectrum of abnormalities. We have previously described multiple anomalies of the upper cervical spine in this disorder. The objective of this study was to use advanced imaging to further define the morphology of the cervical spine and spinal cord in the 22q11.2 deletion syndrome, with a comparison to age-matched controls. METHODS: A total of 32 patients with a 22q11.2 deletion underwent advanced imaging (computed tomography/magnetic resonance imaging; CT/MRI) of the cervical spine. In 27 patients, space available for the cord (SAC); the sagittal diameter of the vertebral body, spinal canal, cerebrospinal fluid (CSF), and spinal cord; and the cross sectional area of the spinal canal, CSF, and spinal cord were measured at each cervical level and compared to 29 age-matched controls. Statistical analysis was performed and potential implications were hypothesized. RESULTS: In 22q11.2 patients, advanced imaging identified 40 pathologies not evident on plain radiographs with potential mechanical and/or neurological implications. These patients also had significantly smaller values (P

Femoral neck fracture as an atypical presentation of child abuse.

Femoral neck fractures account for 46% of the proximal femur fractures in children; however, the overall incidence of proximal femur fractures in children is <1%. Pathologic causes for proximal femur fractures include local or global causes of bone weakness, such as metabolic bone conditions or neoplastic causes. Nonpathologic causes of femur fractures are caused almost exclusively by high-energy trauma and are readily identified. Nonaccidental injury leading to femoral neck fracture is unusual and can be challenging to diagnose when the medical history is not forthcoming. Femoral neck fractures in children usually are a consequence of high-energy trauma or less frequently are associated with pathologic conditions. If neither condition is readily identified, the possibility of nonaccidental injury should be considered. This article presents a case of nonaccidental injury with an atypical presentation in a 3-year-old girl with a femoral neck fracture and discusses the diagnostic rationale for a diagnosis of nonaccidental injury. This case report highlights the magnitude of child abuse and its early recognition in the orthopedic community.

Juvenile dermatomyositis with bilateral progressive knee flexion contracture.

Juvenile dermatomyositis is a multisystem, inflammatory vasculopathy that primarily affects muscles and skin. Calcinosis is one of the most debilitating complications affecting patients with juvenile dermatomyositis. Calcifications resulting from calcinosis frequently are located on the elbows, knees, and other joints and can cause considerable disability with severe pain, joint contractures, skin ulcers, and muscle atrophy. Many therapies for calcinosis have been reported including diltiazem, probenecid, and alendronate. We report a patient surgically treated for bilateral knee flexion contractures with the Ilizarov technique. At 2.5 years' followup, the patient had full extension of both knees with 0 degree to 50 degrees flexion and was walking independently. The Ilizarov technique provides an important option for correcting knee flexion contractures secondary to calcinosis in juvenile dermatomyositis.

Normal cervical spine range of motion in children 3-12 years old.

STUDY DESIGN: This study measured active cervical spine range of motion (ROM) in children ages 3-12 years using 2 methodologies: (1) a cervical spine ROM instrument, and (2) a digital videography based technique. OBJECTIVE: To determine the active cervical spine ROM, as defined by flexion/extension, lateral bending, and horizontal rotation, for children ages 3-12 years. SUMMARY OF BACKGROUND DATA: Review of current literature reveals limited studies of cervical spine ROM in children, many of which do not provide data on children younger than 8 years of age when many of the structural changes of the cervical spine are thought to occur. METHODS: A total of 67 children (39 girls) within 3 age groups, 3-5 years (26 children), 6-8 years (22), and 9-12 years (19), were tested in flexion/extension, lateral bending, and horizontal rotation. The subjects repeated the neck movements 3 times for each direction in each method (ROM instrument and videography), and the maximum ROM values were recorded. Within each age group, the mean, standard deviation, and range for each ROM was calculated, and ROM values from the 2 methods were compared. The effect of age and gender on ROM was assessed via analysis of variance. RESULTS: For the ROM instrument, only flexion and right and left rotation were shown to increase with age (P < 0.05). This difference resulted in an increase in ROM of approximately 7 degrees between the youngest and oldest age group. No age effects were present in the videography data. No significant differences between the genders were detected for any of the ROM measures using either method (minimum P = 0.22). In general, the videography method resulted in higher values for flexion, extension, and rotation, and lower values for lateral bending. These differences were greater for the younger children. CONCLUSIONS: This study contributes valuable normative data for pediatric cervical spine ROM in children that can be used as a clinical reference and for biomechanical applications. In children 3-12 years of age, both flexion and rotation increased slightly with age. Of interest, there were no differences in ROM with gender, which contradicts adult literature where females have been shown to have more cervical spine ROM than males.

Occipitalization of the atlas in children. Morphologic classification, associations, and clinical relevance.

BACKGROUND: Occipitalization is defined as a congenital fusion of the atlas to the base of the occiput. We are not aware of any previous studies addressing the morphologic patterns of occipitalization or the implications of occipitalization in children. We present data on what we believe is the largest reported series of children with occipitalization studied with computed tomography and/or magnetic resonance imaging, and we provide a description of their clinical characteristics. METHODS: We retrospectively reviewed all cases of occipitalization in children included in our spine database. Patient charts and imaging studies were reviewed. A new morphologic classification of occipitalization was developed from the two-dimensional sagittal and coronal reformatted computed tomographic reconstructions and/or magnetic resonance images. The classification includes four patterns according to the anatomic site of occipitalization (Zones 1, 2, and 3 and a combination of those zones), and it was applied to this group of patients. Imaging studies were also reviewed for evidence of cervical instability and for other anomalies of the craniovertebral junction. RESULTS: Thirty patients with occipitalization were identified. There were twenty-four boys and six girls with a mean age of 6.5 years. The morphologic categorization was Zone 1 (a fused anterior arch) in six patients, Zone 2 (fused lateral masses) in five, Zone 3 (a fused posterior arch) in four, and a combination of fused zones in fifteen. Seventeen patients (57%) had atlantoaxial instability, and eight of them had an associated C2-C3 fusion. Eleven patients (37%) had spinal canal encroachment, and five of them had clinical findings of myelopathy. The highest prevalence of spinal canal encroachment (63%) was noted in patients with occipitalization in Zone 2. CONCLUSIONS: Occipitalization is associated with abnormalities that lead to narrowing of the space available for the spinal cord or brainstem. The risk of atlantoaxial instability developing is particularly high when there is an associated congenital C2-C3 fusion. Two-dimensional sagittal and coronal reformatted computed tomographic reconstructions and/or magnetic resonance images can help to establish the diagnosis and permit categorization of occipitalization in three zones, each of which may have a different prognostic implication.

Osgood Schlatter syndrome.

PURPOSE OF REVIEW: Osgood Schlatter syndrome presents in growing children (boys, 12-15 years; girls, 8-12 years) with local pain, swelling and tenderness over the tibial tuberosity. Symptoms are exacerbated with sporting activities that involve jumping (basketball, volleyball, running) and/or on direct contact (e.g. kneeling). With increased participation of adolescent children in sports, we critically looked at the current literature to provide the best diagnostic and treatment guidelines. RECENT FINDINGS: Osgood Schlatter syndrome is a traction apophysitis of the tibial tubercle due to repetitive strain on the secondary ossification center of the tibial tuberosity. Radiographic changes include irregularity of apophysis with separation from the tibial tuberosity in early stages and fragmentation in the later stages. About 90% of patients respond well to nonoperative treatment that includes rest, icing, activity modification and rehabilitation exercises. In rare cases surgical excision of the ossicle and/or free cartilaginous material may give good results in skeletally mature patients, who remain symptomatic despite conservative measures. SUMMARY: Osgood Schlatter syndrome runs a self-limiting course, and usually complete recovery is expected with closure of the tibial growth plate. Overall prognosis for Osgood Schlatter syndrome is good, except for some discomfort in kneeling and activity restriction in a few cases.

Giant cell tumor of tendon sheath: largest single series in children.

Giant cell tumors of tendon sheath (GCTTS) are rare in children. We hypothesized that GCTTS in children probably behave in a similar manner to the adult lesions, with regard to clinical features, imaging characteristics, histology, and recurrence rates after surgical excision. We retrospectively reviewed 29 children diagnosed and treated for GCTTS during a 16-year period and evaluated the above characteristics to compare these results with published data for adult patients. A telephone questionnaire survey was also conducted to assess the current symptoms and function, satisfaction with procedure, and incidence of recurrence. In children, we noted similar predilection for lesions in both upper and lower extremities. Most cases, 28 (96%) of 29, presented with a gradual enlarging soft tissue mass. Plain radiography demonstrated soft tissue swelling in 50% of cases. Magnetic resonance imaging features were noted to be variable, although low signal intensity on T1- and T2-weighted images was noted in most lesions. Incisional biopsy with intraoperative frozen section and histological evaluation was the gold standard for accurate diagnosis. We noted no recurrence at final follow-up in any of our cases. Twenty-two (75%) of these 29 patients had follow-up of more than 2 years, with no recurrence, and the remaining 7 had follow-up between 1 to 2 years, which may seemingly be adequate from perspective of expected time interval for early recurrences. Meticulous dissection and excision with appropriate use of magnifying surgical loupes are likely factors that may help to minimize the recurrence rate.

Minimal surgical approach for recurrent hip dislocation using the posterior lip augmentation device for the Charnley hip arthroplasty.

We present the management of recurrent posterior dislocations in cemented Charnley total hip arthroplasties treated with acetabular augmentation. Certain patients are elderly with comorbid conditions and diminished reserves. We have used a minimal surgical approach to implant 21 posterior lip augmentation devices in elderly patients. Ninety percent of the hips are stable with follow-up of 1 to 3 years (mean, 1.9 years). Eighty-four percent of the patients were satisfied with the outcome. Two cases, which redislocated, were further stabilized by changing the position of the posterior lip augmentation device and are stable. Because of a minimal surgical approach, the time duration of surgery, blood loss, transfusion requirement, inpatient stay, and morbidity are reduced. Posterior lip augmentation device surgery with a minimal approach is a safe and effective procedure for elderly patients who do not have any obvious cause for dislocation.

A subjective outcome analysis of tendo-Achilles rupture.

This clinical outcomes study examined factors affecting patient satisfaction after treatment of Achilles tendon rupture. There were 35 patients; 14 treated nonsurgically and 21 treated by open surgical repair. A retrospective chart review was performed to determine the mechanism and type of injury, the time to treatment, any comorbidities, and the length of follow-up. Patients completed a questionnaire that evaluated their pre- and postinjury activity levels, occupation, and overall satisfaction with their function by using a visual analogue scale. The mean follow-up was 2 years. Seventy percent were very satisfied with the outcome of treatment, with a mean visual analogue score of 8.4 of 10. Age, sex, and occupation did not have any significant influence on the satisfaction level. The dissatisfied group showed a significantly reduced postinjury leisure activity level (P =. 003). A delay in initiation of treatment had a significant negative impact on satisfaction (P = .015). Regression analysis showed that physiotherapy after treatment increased the postinjury activity level and the level of satisfaction (P = .034). There was no significant difference in the overall outcome between the surgical and nonsurgical groups.