Orthopaedic manifestations of pseudoachondroplasia.

PURPOSE: In 1959, Maroteaux and Lamy initially designated pseudoachondroplasia as a distinct dysplasia different from achondroplasia the most common form of skeletal dysplasia. Pseudoachondroplasia is caused by a mutation in the collagen oligomeric matrix protein gene (COMP) gene on chromosome 19p13.1-p12 encoding the COMP. The COMP gene mutations result in rendering the articular and growth plate cartilages incapable of withstanding routine biomechanical loads with resultant deformity of the joints. The purpose of the study was to characterize the typical orthopaedic findings in pseudoachondroplasia. METHODS: The charts and radiographs of 141 patients with pseudoachondroplasia were analyzed. This cohort, to our knowledge, represents the largest group of patients describing the typical orthopaedic manifestations of pseudoachondroplasia. RESULTS: Patients with pseudoachondroplasia have normal craniofacial appearance with normal intelligence. Short stature is not present at birth and generally appears by two to four years of age. The condition is a form of spondyloepiphyseal dysplasia and the long bones are characterized by dysplastic changes in the epiphysis, metaphysis and vertebral bodies. Radiographically the long bones have altered the appearance and structure of the epiphyses with small irregularly formed or fragmented epiphyses or flattening. The metaphyseal regions of the long bones show flaring, widening or 'trumpeting'. The cervical (89%) and thoracic and lumbar vertebrae show either platyspondyly, ovoid, 'cod-fish' deformity or anterior 'beaking'. Kyphosis (28%), scoliosis (58%) and lumbar lordosis (100%) are commonly seen. The femoral head and acetabulum are severely dysplastic (100%). The knees show either genu valgum (22%), genu varum (56%) or 'windswept' deformity (22%). CONCLUSION: Most commonly these distortions of the appendicular and the axial skeleton lead to premature arthritis particularly of the hips and often the knees not uncommonly in the 20- to 30-year-old age group. LEVEL OF EVIDENCE: III.

Radiographic results of proximal femoral varus osteotomy in Legg-Calvé-Perthes disease.

A retrospective review of 116 Legg-Calvé-Perthes patients with varus osteotomy for Herring group B and C disease involvement was conducted. Preoperative assessment consisting of anteroposterior pelvis and frog leg lateral radiographs and an arthrogram was performed on all patients. Radiographic extent of disease was classified using Herring's lateral pillar classification system. Radiographic outcome was assessed utilizing Stulberg's classification to grade residual deformity. Follow-up averaged 6 years 9 months (range, 3 years to 15 years 3 months). Stulberg class I and II (spherically congruent) results were obtained in 86% of patients younger than 9 years old with Herring class B disease. Patients 9 years and older with Herring class B disease had spherically congruent results in 67% of cases. Patients with Herring class C disease 9 years and older had spherically congruent results in 30% of cases and 43% spherically congruent results in those younger than 9 years.

The management of forearm fractures in children: a plea for conservatism.

A retrospective review was undertaken to evaluate the efficacy of primary nonoperative treatment (closed reduction and long-arm casting) along with pins and plaster as a salvage technique for those reduction failures. A total of 730 closed fractures (1987-1993) was compiled, of which 300 required closed reductions and casting. Excluded from the study were teenagers whose growth plates were closed. Of the 300 fractures requiring closed reductions, 22 went on to require remanipulations, and 12 required the use of pins-and-plaster technique to obtain or maintain satisfactory reduction. Complications in the group treated in this manner included two superficial pin infections treated with antibiotics and two forearms with moderate loss of pronation/supination not requiring treatment. We believe that closed reduction of pediatric forearm fractures remains the accepted standard and the technique of pins and plaster should be considered a reliable alternative for the unstable injuries.

Severely displaced proximal humeral epiphyseal fractures: a follow-up study.

Forty-eight patients with severely displaced proximal humeral epiphyseal fractures were reviewed. Twenty-one patients were followed up for an average of 9 years after injury with a personal interview, examination, and radiographs. Average age was 14.1 years, half of whom were age 15 years or older. Initial head-shaft displacement averaged 80%, and 31 fractures were displaced by > or = 80%. All underwent attempted closed reduction. Radiographs for all except three were sufficient to judge the adequacy of reduction. Twenty-six of 45 attempted closed-reductions failed to improve displacement significantly. Nine patients underwent operative treatment, and the remainder had a closed reduction. In the operative group, complications occurred in three of nine patients. No complications occurred in the nonoperative group. No patients seen at late follow-up identified any activity or employment restrictions as a result of their injuries. Several patients incurred humeral shortening or imperfect radiographic remodeling, but this did not correlate with clinical outcome. Comparing the operative complications with the excellent late results after nonoperative treatment in this series supports previous recommendations to avoid operative intervention with few exceptions. The magnitude of displacement alone does not appear to justify operative treatment.

Mitchell osteotomy for adolescent hallux valgus.

Sixty-nine Mitchell osteotomies augmented with smooth-pin fixation and a trapezoidal step-off osteotomy to maintain ray length were performed on 46 adolescent patients with a painful hallux valgus deformity. Average follow-up time was 6 years. By using radiographic, clinical, and subjective patient data, we found an overall excellent or good result in 91% of cases. Nine feet were found to be cosmetically unsatisfactory, 11 required special shoewear, 10 had mild residual pain with high heels or strenuous running, and three had pain severe enough to restrict activities. Range of motion was normal in 56 feet, slightly decreased in 11 feet, and significantly reduced in two feet. We conclude that, given a success rate of 91%, this slightly modified Mitchell osteotomy is a satisfactory procedure for adolescent hallux valgus deformity with chronic bunion discomfort.

Acute slipped capital femoral epiphysis: the value and safety of urgent manipulative reduction.

A 40-year experience consisting of 91 cases of acute slipped capital femoral epiphysis (SCFE) was reviewed to assess the safety of manipulative reduction and to determine whether urgent reduction has an effect on the development of avascular necrosis (AVN) of the capital femoral epiphysis. All patients had a history of sudden onset of severe hip pain and were documented to have an unstable (acute) slipped epiphysis. Treatment modalities included manipulative reduction under general anesthesia followed by internal fixation (41 hips), epiphysiodesis and internal fixation (15 hips), epiphysiodesis and cast immobilization (31 hips), and cast immobilization alone (three hips). One case was treated with cast immobilization after reduction by skeletal traction. Patient follow-up averaged 44 months, and ranged from 12 to 216 months. Radiographic review identified 13 (14%) cases of AVN in the series of 91 hips. Of 42 hips reduced in <24 h from presentation, AVN developed in three (7%). Of 49 hips reduced in >24 h from presentation, AVN developed in 10 (20%). Manipulative reduction of the acute SCFE may be accomplished without increased risk of AVN. Time to reduction may be an important risk factor for development of AVN after acute SCFE.

Complications in proximal tibial osteotomies in children with presentation of technique.

Proximal tibial osteotomies in children have been associated with a number of complications, including peroneal nerve palsies, compartment syndromes, deep and superficial infections, vascular injuries, iatrogenic fractures, apophyseal or growth plate damage, and recurrence of deformity. A retrospective review of the results of 22 children who had 30 primary and seven repeat proximal tibial osteotomies was performed to assess the frequency of complications at our institution. Using a specified technique that included prophylactic anterior compartment fasciotomy and distal fibulectomy, there were three superficial wound infections and one delayed union but no peroneal nerve palsies, vascular injuries, compartment syndromes, or fixation failures. There were no sequelae from the superficial infections. This is a lower rate of complications than previously reported in the literature and suggests a relationship to our current surgical technique and attention to detail. We believe that proximal tibial osteotomies can be performed successfully and safely in children when the surgeon recognizes the potential problems and takes steps to prevent them.

The treatment of rigid metatarsus adductovarus with the use of a new hinged adjustable shoe orthosis.

A retrospective review of the early results of treatment of metatarsus adductovarus with a new hinged adjustable shoe orthosis, the Bebax shoe, is presented. The results in 120 cases (210 feet) are included in the study. Criteria for severity of the initial deformity were developed, and all cases of true clubfoot and those cases with prior treatment were excluded. The average age at institution of treatment was 4.5 months, and treatment time averaged 2.7 months (range 1-7 months). Overall, 96% of the cases were completely resolved with the use of the orthotic alone, 3% required additional casting, and 1% required a bar and shoes for complete correction of the deformity. No treatment complications were encountered except some minor problems with hinge loosening, particularly in the child of walking age. We believe that the Bebax shoe is a lightweight, convenient, safe, and both cost- and treatment-effective device in the management of metatarsus adductovarus.

Management of metatarsus adductovarus.

A retrospective analysis of 795 patients treated for metatarsus adductovarus by a standardized outpatient approach by one of the authors (D.S.W.) from 1970 to 1983 was conducted to evaluate the efficacy of a straight metal bar and attached reverse last shoe protocol. Criteria for inclusion were developed excluding those milder cases in which spontaneous resolution was expected and indeed occurred. Nearly uniform excellent results were encountered, with a 99% likelihood of obtaining a fully corrected foot. Surgical intervention was deemed necessary in under 1% of cases seen. The authors also examined birth weights and rank in relation to metatarsus adductovarus. Those children with the deformity had a mean birth weight of 7.6 pounds, without statistical variance from normal average birth weights. We were unable to establish any statistical association with birth rank. In light of the results of this study, which compare favorably with serial casting, we recommend this approach as a more economical and less disruptive and cumbersome technique for parent, patient, and physician.

Avascular necrosis complicating fracture of the distal tibial epiphysis.

We have described a patient with a very rare case of distal tibial epiphyseal AVN after a severe fracture. This patient subsequently had complete asymptomatic revascularization and closure of the epiphyseal plate without deformity. Practitioners should be aware, however, that in younger persons a similar lesion can lead to premature closure of the plate and resultant deformity.

Pitfalls in treatment of Legg-Calvé-Perthes disease using proximal femoral varus osteotomy.

A femoral varus osteotomy can be used to contain the femoral head in Legg-Calvé-Perthes (LCP) disease if certain pitfalls can be avoided. We reviewed 74 patients who underwent 79 femoral varus osteotomies. The study addressed the pitfalls that should be avoided with this technique. It was concluded that the amount of varus angulation should barely position the femoral head beneath the lateral rim of the acetabulum, avoiding varus less than 105 degrees, and that consideration should be given to performing a greater trochanteric epiphysiodesis at the time of initial femoral osteotomy. The short-term results reflect a positive attitude toward femoral varus osteotomy in treatment of LCP if these pitfalls can be avoided.

Hazards of internal fixation in the treatment of slipped capital femoral epiphysis.

We reviewed the records of 202 patients (308 hips) in whom a slipped capital femoral epiphysis had been fixed with pins or screws. A serious complication that was directly related to the use of internal fixation developed in eighty hips (26 per cent). The rate of complications in the 202 patients was 40 per cent. In thirty-six (18 per cent) of the 202 patients, an additional procedure was done to correct a pin-related complication. Forty-one hip joints had been penetrated by a pin. Other complications included avascular necrosis (fourteen hips), chondrolysis (nine), fracture (one), infection (one), further slippage (one), sciatic-nerve injury (one), and breakage of a screw (eight). Ways of decreasing the incidence of complications of fixation were explored.

Surgical treatment of symptomatic accessory tarsal navicular.

We report a retrospective review of 50 consecutive patients (75 feet) with chronically symptomatic accessory tarsal naviculars that failed to improve with conservative treatment. Surgical treatment consisted of excision of the accessory tarsal navicular or medial protuberance of a prominent cornuate-shaped navicular combined with simple replication of the tibialis posterior tendon without altering its course. Good and excellent results were obtained in 45 (70 feet) of the 50 patients (90%). The procedure has a low rate of minor complications, is easy to perform, and is extremely well accepted by the patients.

Treatment of osteoid osteoma by computed tomography guided excision in the pediatric patient.

An osteoid osteoma is a benign lesion that can occur in any bone. Controversy exists regarding treatment of this condition. This study demonstrates that the nidus of an osteoid osteoma can be disrupted and removed by use of the CORB biopsy system guided by computed tomography (CT) scan. Of nine patients followed for an average of 42.5 months (range 18-76 months), seven have had complete resolution of their pain after this procedure. The technique failed in two patients, who each required two separate en bloc excisions with bone grafting to resolve each lesion. CT-directed CORB appears to be useful in localization and removal of the nidus of an osteoid osteoma.

Results of slipped capital femoral epiphysis in the black population.

This retrospective study, based on a chart and radiographic review of blacks treated for slipped capital femoral epiphysis (SCFE), showed a 93.2% incidence of excellent or good results, and an incidence of only 2.3% of major complications with poor results. Blacks can generally be expected to have a good result after treatment of SCFE in both males and females with a low incidence of avascular necrosis (AVN) or acute cartilage necrosis (ACN).

Use of pins and plaster in the treatment of unstable pediatric forearm fractures.

The treatment of unstable forearm fractures in children remains a challenging and controversial subject. There are times when more rigid fixation is needed to obtain and maintain fracture reduction. Open reduction/internal fixation or intramedullary nailing can be avoided by the use of the technique of pins and plaster. This study reviewed 20 forearm fractures treated between 1978 and 1984. All fractures healed uneventfully in satisfactory alignment. No complications were encountered. We conclude that the method of pins and plaster is a safe and reliable method to treat unstable pediatric forearm fractures.

Redisplacement after closed reduction of forearm fractures in children.

Reangulation and displacement after closed reduction of pediatric forearm fractures were reviewed in 90 patients treated with 1978-1984. All fractures were remanipulated and followed to union. No complications occurred and all had a functionally satisfactory result. Both intrinsic and extrinsic factors were identified as contributing to the complication of reangulation/displacement. Nonepiphyseal fractures were safely remanipulated up to 24 days postfracture, with the majority at 1-2 weeks. We conclude that 7% of pediatric forearm fractures treated by closed reduction are subject to reangulation and/or displacement following routine acceptable primary treatment, and that remanipulation provides a safe, effective means to obtain and maintain reduction.