Relationship of factors affecting age of onset of independent ambulation.

Despite the standard available pediatric developmental scales and popular lore that girls walk at an earlier age than boys, no large-scale evaluation of the age of onset of independent ambulation has been previously published. The purpose of this study was the prospective epidemiologic evaluation of a large heterogeneous group of normal children to determine the effect of gender, race, birth order, and socioeconomic status on the age of onset of independent ambulation. The study cohort consisted of 986 children (575 male, 471 female). A multivariable analysis of covariance model was used to examine the effects of race, gender, income, and birth order on age at ambulation. After controlling for the other variables in the model, race was the only statistically significant predictor of age at ambulation (p < 0.0001), with black children walking at a younger age (10.9 +/- 2.1 months) than white children (11.6 +/- 2.3 months). Overall, the independent variables included in the model were only able to explain 2.5% of the variance of age at ambulation.

The effect of pediatric orthopaedic experience on interobserver and intraobserver reliability of the herring lateral pillar classification of Perthes disease.

An analysis was done of the effect of surgeons' pediatric orthopaedic experience on the classification of Perthes disease according to the lateral pillar classification described by Herring. Five observers with varied pediatric orthopaedic experience reviewed anteroposterior (AP) pelvis radiographs of 33 patients in the fragmentation phase of Perthes disease and classified each case on three separate occasions at least 24 hours apart. Frog-leg lateral-view radiographs taken at the same time were also classified using the same criteria based on the femoral head anterior column. Kappa statistics showed good agreement for intra- and interobserver reliability of classification for both AP and frog lateral radiographs. There were no statistically significant variations among the reviewers. Twenty-four percent of the cases had a classification one grade worse on the lateral radiograph compared to the AP view. The lateral pillar classification provides a reproducible radiographic technique for Perthes disease characterization independent of pediatric orthopaedic experience.

Orthopaedic manifestations of Ehlers-Danlos syndrome.

Ehlers-Danlos syndrome is the most prevalent heritable disorder of connective tissue. Musculoskeletal problems include joint pain, swelling and instability, and spinal deformity. This study was undertaken to assess functional orthopaedic problems of patients with Ehlers-Danlos syndrome. Sixty patients with genetically verified Ehlers-Danlos syndrome (range, 8-60 years; mean, 34 years) who attended a National Ehlers-Danlos Syndrome Foundation learning conference were evaluated by questionnaire, clinical examination, and when indicated, radiographs. A database of 250 items per patient was constructed and statistically assessed using analysis of variance. Because of rarity of Types VII and VIII, these two patients were dropped from the analysis. Fifty-eight patients had Ehlers-Danlos syndrome Types I, II, III, or IV and form the study cohort. Among these four types, there were no significant differences in history of joint dislocation, swelling, or types of orthopaedic surgical procedures experienced. Thirty patients with Type III Ehlers-Danlos syndrome reported joint pain more frequently than did patients with Types I, II, or IV. Ambulation was impaired significantly in patients with Type III disorder as a whole, as was functional hand strength and upper extremity function. Back or neck pain was a common (67.2%) report among patients with all types of disease but did not correlate with the presence or absence of spinal deformity. Contrary to most previous reports, the patients in this study showed that Type III Ehlers-Danlos syndrome was the most debilitating form with respect to musculoskeletal function.

Interpretation of radiographs in a pediatric limb deformity practice: do radiologists contribute?

Orthopaedic surgeons make treatment decisions based on their interpretation of patient radiographs. Radiologists' reports of these radiographs are routine but may add little to the patient's management. The authors prospectively compared data initially recorded by a pediatric orthopaedist in the assessment of teleoroentgenograms obtained over a 3-month period in a limb deformity clinic with the subsequent reports of these studies by pediatric radiologists. Two hundred and sixty-four (100%) anatomic axes of femora and tibiae were measured and reported by the surgeon compared to 1.9% by the radiologist. Limb lengths were recorded 100% of the time by the orthopaedist and by the radiologist in 80% of cases. Abnormal bone quality was present in 43 of 264 (16.2%) limb segments and reported in all involved cases by the orthopaedist. Only 26 (9.8%) of the abnormalities were noted by the radiologist. Only eight of 20 (35%) physeal abnormalities such as rickets were noted by the radiologist in contrast to 20 of 20 (100%) noted by the surgeon. There were no findings described by the radiologist not previously reported by the orthopaedic surgeon. In no case did the radiology interpretation influence clinical management decisions.

Spinal manifestations of skeletal dysplasias.

Skeletal dysplasias, disorders of abnormal bone and cartilage development, are a heterogeneous group, each disorder with its own genetics, prevalence, prognosis, and treatment. More than 150 distinct conditions have been identified. Despite their obvious differences, the osteochondrodysplasias share many clinical and radiographic features. These patients present to the orthopedic surgeon for evaluation of disproportionate short stature, which may be apparent at birth or manifest itself only with further growth. This article discusses bone dysplasias commonly associated with spinal abnormalities. Spinal pathology can lead to deformity, neurologic sequelae, pain, and cardiopulmonary compromise and further contribute to short stature.

Limb-length inequality: assessment and treatment options.

Assessment and treatment of limb-length inequality, particularly in the growing child, is a challenging task. Evaluation of the discrepancy requires an understanding of the significance of the disparity, as well as the natural history of the disorder, before formulation of a treatment plan. In the immature patient, consistent longitudinal data are essential to avoid pitfalls in the projection of ultimate length difference. Therapeutic options range from no treatment or use of a simple shoe lift to a surgical shortening or lengthening procedure. The current indication for lengthening is a disparity exceeding 5 to 6 cm. Epiphysiodesis or femoral shortening is useful for smaller discrepancies or for residual differences following a contralateral lengthening. Lengthening is done with a circular or cantilever external fixator, which may be combined with an intramedullary rod.

Syndrome of inappropriate antidiuretic hormone secretion in children following spinal fusion.

OBJECTIVES: a) To determine if antidiuretic hormone (ADH) is elevated in patients undergoing spinal fusion, especially in those who have clinical evidence of syndrome of inappropriate antidiuretic hormone (SIADH); b) to evaluate the relationship between ADH secretion and the secretion of atrial natriuretic peptide (ANP). SETTING: Tertiary care pediatric intensive care unit (ICU) in a university hospital. DESIGN: A prospective cross-sectional, observational study with factorial design. PATIENTS: Thirty patients > or = 10 yrs of age undergoing spinal fusion admitted to the ICU for postoperative care. INTERVENTIONS: Patients underwent anterior, posterior, or both anterior/posterior spinal fusion. Blood was collected for serial measurements of ADH, ANP and serum electrolyte levels. Heart rate, blood pressure and central venous pressure were measured. MEASUREMENTS AND MAIN RESULTS: Thirty children were studied. Nineteen had idiopathic scoliosis, nine had neuromuscular scoliosis, one had Marfan's disease, and one had congenital scoliosis. Ten (33%) children met clinical criteria of SIADH. There was no difference in duration of surgery, blood loss, volume of iv fluid administration pre- and intraoperatively, or type of scoliosis between those who developed SIADH and those who did not. Hemodynamic variables were similar in both groups. ADH levels increased in both groups immediately postoperatively and at 6 hrs after surgery, but were much more elevated in those patients with SIADH. Patients with SIADH also had significantly higher ADH levels preoperatively. In relation to serum osmolality, ADH was considerably higher in those with SIADH compared with those who did not. Although ANP values tended to be higher in the group with SIADH, this did not reach statistical significance. CONCLUSION: SIADH occurs in a subset of children who undergo spinal fusion. The diagnosis of SIADH can be made easily using clinical parameters which are well-defined. In the face of SIADH, continued volume expansion may be harmful, and should therefore be avoided.

Depression of the medial tibial plateau in early-onset Blount disease: myth or reality?

Patients with severe early-onset Blount disease (Langenskiold III or greater) often have the radiographic appearance of depression of the medial tibial plateau and delayed ossification of the epiphysis, adjacent physis, and metaphysis. Schoenecker and other authors recommended elevation of the medial plateau of the tibia for correction of this deformity. The purpose of this study was to assess the nature of the medial proximal tibia in these severe cases. Eight girls and two boys (17 tibiae) with severe early-onset Blount disease had standing radiographs and magnetic resonance imaging (MRI) evaluation. Average patient age was 5.8 years (range, 3-8 years). Eleven knees in eight patients underwent arthrography at the time of surgical correction of deformity. No tibia had MRI or arthrographic evidence of medial tibial joint "depression." The "empty" radiographic space was occupied by cartilage-density material. A smooth dye contour without pooling parallel to the femoral condyles was noted arthrographically in all instances. These findings do not support the concept of routine joint elevation in the correction of severe deformity associated with early-onset Blount disease.

Correction of proximal tibial deformities in adolescents with the T-Garches external fixator.

Frontal-plane deformity of the proximal tibia in children has a variety of etiologies. There are also a number of described surgical techniques for correction of these deformities. The authors reviewed their early experience with the use of the Orthofix T-Garches external fixator for correction of 16 proximal tibial deformities in 14 patients. Mean age at surgery was 14.2 years. The most common diagnosis was adolescent Blount's disease. Average deformity was 12 degrees. Total treatment time averaged 13 weeks for those requiring lengthening and 10.8 weeks for those without. Complications included one ring sequestrum treated by curettage, and two patients with subsequent development of femoral deformity. Tibial anatomic axis averaged 1 degree of varus. This device can achieve excellent correction of deformity isolated to the frontal plane with few complications. It allows functional weight bearing and use of adjacent joints during treatment.

Clotting parameters in patients with adolescent idiopathic scoliosis undergoing posterior spinal fusion and instrumentation.

Thirty-six patients, 9 males and 27 females, average age 13.2 years, undergoing posterior fusion and segmental instrumentation surgery for idiopathic adolescent scoliosis were studied for abnormalities of platelet count (PC), prothrombin time (PT) and partial thromboplastin time (PTT). Intraoperative and immediate postoperative values were obtained at 2, 4, 6, 12, 24, and 48 hours. All patients showed diminution of platelet counts during the study period, but mean values were reduced below normals only at 4 hours after operation. The PT was significantly elevated in all patients intraoperatively and for the first 24 hours postoperatively, with return to normal by 48 hours. The PTT levels were essentially unaffected at any time.

Radiation exposure during skeletal traction treatment of pediatric femoral fractures.

Radiation-exposure data during femoral fracture management has not been previously reported. We report a retrospective analysis of radiation exposure in 45 patients aged 5-12 years (average, 8.3) with isolated femoral shaft fractures treated by 90/90 degrees femoral skeletal traction. Group I had 32 patients aged 5-9 years (average, 7.3), and group II had 13 patients of an average age of 10.7 years. Total average radiation dose before casting was 0.699 rads and was independent of age and gender. In addition to potential complications of tractions and increased hospital stay with attendant fiscal and psychosocial burdens, radiation exposure with this type of management, in this series, was significant.

The prognostic value of quantitative bone scan in knee osteochondritis dissecans. A preliminary experience.

We reviewed the records of 12 patients ages 9 to 16 years with knee osteochondritis dissecans. All patients had clinical histories and examinations, four radiographic views of the knee, and technetium-99m diphosphonate quantitative bone scans. Scan results (symmetric, increased, or decreased activity), clinical course, healing time, and final outcome were correlated to determine the prognostic value of the scan. We divided the patients into those with open physes (distal femoral and proximal tibial) and those with closed physes. Four of the six patients with open physes had increased activity on the bone scan. All four of these knees healed with nonsurgical treatment. The other two patients had decreased activity on bone scan, and both required surgical treatment after nonsurgical treatment failed. Of the six patients with closed physes, all had increased activity on the bone scan, but only two patients had healing of the osteochondral lesion without surgery. Quantitative bone scanning had a 100% predictive value for the prognosis in osteochondritis dissecans patients with open physes, but for those with closed physes the predictive value was less. Because the natural history in the adolescent group is less predictable, it is in this group that the quantitative scan would be most helpful. In this small group of patients, quantitative bone scanning had limited prognostic value.

Rotational deformity in congenital hypoplasia of the femur.

Congenital shortening of the femur is associated with significant limb-length discrepancy, distal femoral valgus with condylar hypoplasia, anteroposterior knee instability, and an external rotation deformity. This latter deformity has not been described or characterized in the literature. Eight patients with hypoplastic femora were evaluated clinically and radiographically including computed tomography (CT) anteversion studies. The average limb-length inequality at the time of examination was 7.8 cm. All affected extremities had significant retroversion averaging 17.4 degrees, representing a mean difference of 43.1 degrees from the contralateral normal anteversion of 25.7 degrees. Associated limb deformities included deficiency of the fibula in five patients and absent lateral foot rays in two patients. Five of the eight patients have undergone femoral lengthening with correction of angular, length, and rotational abnormalities through two-level osteotomy. The patient with femoral hypoplasia should have evaluation of length and angular abnormalities and rotational deformity as well.

Early conservative and operative treatment to gain early normal growth in proximal femoral focal deficiency.

In three children with proximal femoral focal deficiency (PFFD), arthrography of the hip was performed at the respective ages of 9 days, 4 weeks, and 5 weeks. Between the unossified part of the femoral neck and the cartilaginous femoral head, there was moderate flexibility in the first child and lysis with some displacement of the femoral neck and mobility in the other two children. After the children's immobilization in a squatting cast for 3 months, consolidation was achieved and the growth plate developed normally. All three had marked coxa vara and retroversion. The earlier they were treated by valgus osteotomy and rotation to normal anteversion, the earlier normal growth started. Diagnosis at birth and immediate conservative and early operative treatment is therefore indicated in such cases. Only a few centimeters of femoral lengthening is then necessary, and resection of the unossified part of the femoral neck can be avoided.

Tibial torsion in chronic, stable slipped capital femoral epiphyses: evaluation by CT scan.

Externally rotated gait is a hallmark of slipped capital femoral epiphysis (SCFE) attributed to the change of proximal femur structure. This study investigated the potential role of abnormal tibial torsion in 44 patients with chronic, stable SCFE. Comparison of the tibiae of the involved side with the uninvolved side and with previously published normal values showed no evidence of tibial torsional abnormalities in these patients.

Management of late-onset tibia vara in the obese patient by using circular external fixation.

Previously published series of surgery for late-onset tibia vara reported a significant number of complications and fair or poor results. Obesity in many of these patients makes surgical intervention an even more daunting prospect. Circular external fixation is applicable to almost any limb size and allows weight bearing as tolerated, with gradual adjustment of alignment. Twenty-five tibiae in 17 patients who exceeded their ideal body weight by > or =50% underwent correction of late-onset tibia vara with the Ilizarov technique. Average age at surgery was 11 years 7 months (range, 7 years 8 months to 15 years 11 months). Mean varus deformity was 27 degrees (range, 10-55 degrees). Treatment time averaged 12 weeks in patients without lengthening and 16.9 weeks in those requiring lengthening (mean, 3.5 cm). All patients achieved alignment within 5 degrees of normal. Complications included one delayed union, premature consolidation in one, and two residual limb-length inequalities. There were no cases of osteomyelitis, compartment syndrome, or nerve palsy. These results are a significant improvement over reports of traditional methods in these difficult patients.

The effect of femoral lengthening on knee articular cartilage: the role of apparatus extension across the joint.

Loss of joint motion is a common complication of limb lengthening despite newer methods of incremental bone elongation. A pilot canine study has demonstrated that 30% femoral lengthening causes reproducible knee cartilage injury manifest by frank loss of cartilage substance or fibrillation. This study was undertaken to examine the potential of knee joint protection by apparatus extension to the tibia. Four dogs underwent application of a modified Ilizarov apparatus to the femur and tibia with coaxial hinges at the knee. After osteotomy, 30% lengthening was undertaken at 0.75 mm daily in three increments. At the completion of lengthening, experimental and contralateral knee joints were harvested, assessed grossly, decalcified, sagittally sectioned, and stained with safranin-O. All control joints were normal histologically. All experimental joints demonstrated a decrease in proteoglycan staining without evidence of fibrillation or necrosis. These findings suggest a protective effect of the tibial apparatus by avoiding joint compression.

Results of tibial lengthening with the Ilizarov technique.

Between June 1987 and June 1992, 62 tibiae in 52 patients underwent lengthening by using the Ilizarov technique. Follow-up was from 18 months to 5 years. The mean age at surgery was 12.9 years (range, 5-19). The etiology of limb shortening was congenital in 53 and acquired in nine tibiae. Thirty-five tibiae had bifocal and 27 had unifocal treatment. Twenty-two of the 62 limbs had simultaneous treatment of other associated problems, including rotational or angular deformity or foot deformity. The average lengthening was 7.5 cm (range, 3.5-12), which was equivalent to a 32% average overall increase in limb-segment length (range, 6-96%). Complications required 28 (22%) unplanned procedures in the 62 tibiae, including nine osteotomies for iatrogenic malunion or deformation of regenerate bone (31%). Three tendo Achilles lengthenings and posterior ankle capsulotomies were required for persistent equinus contractures. Bony complications declined as experience with the technique increased. This technique allows simultaneous lengthening correction with control of the adjacent foot when required.

Ehlers-Danlos syndrome: relationship between joint hypermobility, urinary incontinence, and pelvic floor prolapse.

OBJECTIVE: To compare the incidence of joint hypermobility and prolapse in incontinent and continent women with Ehlers-Danlos syndrome (EDS). METHODS: Forty-six patients with EDS were evaluated by history and physical examination. The degree of joint mobility of shoulder, elbow, wrist, hip, knee, and ankle was evaluated by orthopedic surgeons and physical therapists. Joint range of motion measurements were obtained using an orthopedic goniometer. RESULTS: The two EDS groups, incontinent (n = 28) and continent (n = 18) women, had a statistically similar mean age, weight, and parity (p = NS). In the EDS population overall joint hypermobility averaged 34.3%. Individuals with EDS type 3 had significantly more joint hypermobility than those with type 1 and 2 EDS. However, logistic regression analysis demonstrated no significant relationship between prolapse and joint hypermobility. Wrist dorsiflexion (p < 0.05) and palmar flexion (p = 0.05) were the only variables related to incontinence. CONCLUSION: It has previously been reported that hypermobility was correlated with pelvic floor prolapse. Of 18 joint measures per patient, hypermobility of both wrist dorsiflexion and palmar flexion was associated with symptoms of incontinence. We were unable to document an association between joint hypermobility and prolapse in the EDS population.