Treatment of dysplasia epiphysealis hemimelica: a systematic review of published reports and a report of seven patients.

Dysplasia epiphysealis hemimelica is a rare entity. It is characterized by cartilage overgrowth in the epiphyses and is considered to be an epiphyseal osteochondroma. It usually presents during childhood and can cause pain and functional limitations. The aim of the present study was to describe the entity through our cases and published data and to present the results of our suggested treatment. A systematic search was performed to retrieve and describe all studies on dysplasia epiphysealis hemimelica. Studies were included if sufficient information on the patient characteristics, lesion location, treatment, and outcomes were described. In addition, we report on 7 of our patients. In our review, 48 studies were found, of which 42 could be included. Owing to the large heterogeneity in the studies, no pooling of data was performed. The studies included 138 patients with 255 lesions. Approximately 27% were female patients. The age at presentation ranged from 3 months to 66 years; however, in most, the entity was discovered before 8 years of age. No apparent guidelines were presented concerning the treatment strategy; however, most investigators performed resection because of pain or limitations in the range of motion. Most lesions were located in the ankle or foot (139 of 255). In our own cases, 5 of 7 patients experienced pain, limited range of motion, or activity restriction. The mean follow-up period was 4 years. Dysplasia epiphysealis hemimelica is a rare entity, occurring predominantly in the lower extremity of in young male patients. Its aggressive growth warrants aggressive treatment in the case of pain or functional or range of motion limitations.

The results of downgrading moderate and severe slipped capital femoral epiphysis by an early Imhauser femur osteotomy.

PURPOSE: Patients with moderate and severe slipped capital femoral epiphysis (SCFE) develop osteoarthritis earlier in life in association with mechanical impingement. METHODS: To correct deformity and diminish impingement, we performed epiphysiodesis combined with an Imhauser intertrochanteric osteotomy (ITO) in moderate and severe slipped capital femoral epiphysis. We downgraded the angle of the head relative to the acetabulum into an angle corresponding to a mild slip or even an anatomical position. Our hypothesis is that the avoidance of anterior impingement at an early stage can prevent the development of osteoarthritis. RESULTS: The results of 28 patients (32 hips) were evaluated. Outcome parameters were SF-36, Harris Hip Score, range of motion, Kellgren-Lawrence score, chondrolysis and avascular necrosis. After a median follow-up of 8 (range 2-25) years, the group was clinically, functionally and socially performing well. Radiologically, there was no sign of chondrolysis or avascular necrosis, and more than 80% of the patients did not show any signs of osteoarthritis. CONCLUSIONS: Based on these results, we conclude that a one-stage Imhauser ITO combined with epiphysiodesis performed on patients with moderate and severe SFCE gives satisfactory results.

Awareness and use of intertrochanteric osteotomies in current clinical practice. An international survey.

Current literature shows that intertrochanteric osteotomies can produce excellent results in selected hip disorders in specific groups of patients. However, it appears that this surgical option is considered an historical one that has no role to play in modern practice. In order to examine current awareness of and views on intertrochanteric osteotomies among international hip surgeons, an online survey was carried out. The survey consisted of a set of questions regarding current clinical practice and awareness of osteotomies. The second part of the survey consisted of five clinical cases and sought to elicit views on preoperative radiological investigations and preferred (surgical) treatments. The results of our survey showed that most of these experts believe that intertrochanteric osteotomies should still be performed in selected cases. Only 56% perform intertrochanteric osteotomies themselves and of those, only 11% perform more than five per year. The responses to the cases show that about 30-40% recommend intertrochanteric osteotomies in young symptomatic patients. This survey shows that the role of intertrochanteric osteotomies is declining in clinical practice.

Outcome after corrective osteotomy for malunited fractures of the forearm sustained in childhood.

We analysed the operative technique, morbidity and functional outcome of osteotomy and plate fixation for malunited fractures of the forearm sustained in childhood. A total of 20 consecutive patients underwent corrective osteotomy of 21 malunited fractures at a mean age of 12 years (4 to 25). The mean time between the injury and the osteotomy was 30 months (2 to 140). After removal of the plate, one patient suffered transient dysaesthesia of the superficial radial nerve. The mean gain in the range of movement was 85 degrees (20 degrees to 140 degrees ). The interval between injury and osteotomy, and the age at osteotomy significantly influenced the functional outcome (p=0.011 and p=0.004, respectively). Malunited fractures of the forearm sustained in childhood can be adequately treated by osteotomy and plate fixation with excellent functional results and minimal complications. In the case of established malunion it is advisable to perform corrective osteotomy without delay.

[Pyomyositis of the M. quadriceps femoris in an 8-month-old boy following pneumococcal meningitis]. / Een 8 maanden oude jongen met een pyomyositis van de m. quadriceps femoris volgend op een pneumokokkenmeningitis.

An 8-month-old boy developed pyomyositis of the M. quadriceps femoris following meningitis caused by Streptococcus pneumoniae. The child had no underlying immune defect. Conservative treatment with antibiotics led to complete clinical and radiological resolution of the infection. In Europe, pyomyositis is rarely seen in children. Pyomyositis should be considered in the differential diagnosis in children with fever and complaints of joint pain or myalgia. Ultrasound and MRI are helpful techniques for establishing the diagnosis and for differentiating pyomyositis from other pathological conditions. Treatment consists of adequate antibiotics and in some cases drainage.

Long-term results of multiple-stage treatment for posttraumatic osteomyelitis of the tibia.

BACKGROUND: The treatment of posttraumatic osteomyelitis of the tibia requires meticulous debridement and adequate soft tissue coverage. At our institution, we perform a staged procedure consisting of surgical debridement followed by muscle coverage. If necessary, implantation of a cancellous iliac bone graft was always performed as a three-stage treatment. METHODS: We performed a retrospective analysis of 47 patients treated for posttraumatic osteomyelitis of the tibia between 1987 and 1998. RESULTS: Twenty-two patients originally had a Gustilo grade III fracture, 21 patients had a Gustilo grade I or II or closed fracture, the Gustilo grade was not known for 2 patients, and 2 patients had no fracture. Using the Cierny-Mader classification, most patients had a localized osteomyelitis. To cover the debrided area, 20 pedicled muscle transfers and 28 microvascular free flaps were used; one patient had two localizations of osteomyelitis (both proximal and distal) and received two muscle flaps. Flap failure was 8% and was successfully treated by additional flap coverage in two cases; one was closed by a split skin graft and one was closed by secundum. Twenty-six patients received a cancellous bone graft. During an average follow-up of 94 months, 9% had a recurrence of osteomyelitis for which additional surgical interventions were necessary. Finally, all the infections were eventually cured. CONCLUSION: Our staged surgery proved to be an excellent method of treating osteomyelitis after open or closed fractures of the tibia.

Operative treatment of mid-shaft clavicular non-union.

Between 1974 and 1999, we treated operatively 28 patients with a symptomatic mid-shaft clavicular non-union using AO osteosynthesis, including bone grafting. Nine patients had a wave-plate osteosynthesis and 19 a standard AO/ASIF osteosynthesis. The mean follow-up was 10 (2-25) years. All but one non-union showed full bony consolidation. Pre-operatively, brachialgia was present in 12 patients. Of these patients, six were treated using standard osteosynthesis technique and six by wave-plate osteosynthesis. Post-operatively, patients treated by wave-plate osteosynthesis had no brachialgia and also had a higher Constant score than those patients treated using standard AO/ASIF techniques.

Humeral shaft nonunion: evaluation of uniform surgical repair in fifty-one patients.

OBJECTIVE: To determine whether a standardized treatment of aseptic humeral shaft nonunions results in union after one operation without substantial complications. DESIGN: Retrospective study. SETTING: University hospital. PATIENTS: Between 1975 and 1998, fifty-eight patients sustained a humeral shaft fracture that resulted in an aseptic nonunion. Fifty-one patients between the ages of twenty-three and eighty-six years (mean 50.8 years) were included and evaluated. INTERVENTION: Surgical treatment consisted of an anterolateral approach with radial nerve identification and subsequent neurolysis, decortication, compression plating, and frequent application of autogenous bone grafts. No immobilization with brace or cast after treatment was functional. MAIN OUTCOME MEASURES: Success rate of nonunion repair, reinterventions, complications, range of motion, patient satisfaction. RESULTS: After one year, consolidation was apparent in all cases. All but two patients had an essentially normal range of motion of the shoulder and elbow, and 96 percent rated their result as excellent or good. After nonunion repair, two patients had a transient sensory neuropathy of the radial nerve. In one patient, a second intervention was needed after a fall from bed one week after the index operation. CONCLUSIONS: The results of this study indicate that our standard surgical procedure for treatment of nonunion of the humeral shaft is reliable and achieves consolidation in one procedure without significant complications.

Subtalar arthrodesis with correction of deformity after fractures of the os calcis.

We have reviewed the long-term results of 22 patients (23 fusions) with fractures of the os calcis, who had subtalar arthrodesis with correction of the deformity between 1975 and 1991. The mean follow-up was nine years (5 to 20). All patients were evaluated according to a modified foot score. A radiological assessment was used in which linear and angular variables were measured including the fibulocalcaneal abutment, the height of the heel and fat pad, the angle of the arch and the lateral talocalcaneal and the lateral talar declination angles. The technique used restores the normal relationship between the hindfoot and midfoot and corrects the height of the heel. This leads to better biomechanical balance of the neighbouring joints and gives a favourable clinical outcome. The modified foot score showed a good or excellent result in 51% of the feet. Residual complaints were mostly due to problems with the soft tissues. Subjectively, an excellent or good score was achieved in 78% of the cases. After statistical analysis, except for the height of the heel and the degenerative changes in the calcaneocuboid joint, no significant difference was found in the measured variables between the operated and the contralateral side.

Comparison of the results of the Girdlestone pseudarthrosis with reimplantation of a total hip replacement.

A Girdlestone pseudarthrosis of the hip (resection arthroplasty) is nowadays mainly carried out for failed hip replacements. A decision may have to be made whether to reimplant a new hip prosthesis or to accept the result of a pseudarthrosis. We followed 2 groups of patients: 32 patients had a long standing pseudarthrosis; in the other group of 16 patients, a total hip replacement was reimplanted at an average of 3 years after a pseudarthrosis. The improvement in hip function after the reimplantation was marginal and the results were comparable to a good functioning pseudarthrosis. However, personal satisfaction and the activities of daily living were better in the reimplantation group, and their Harris hip score was 64 compared to 58 in those with a pseudarthrosis. The Girdlestone procedure still seems to be a reasonable salvage operation for some complications following hip surgery, but when there are the correct indications, reimplantation of a total hip prosthesis is recommended.

The influence of subtalar and triple arthrodesis on the tibiotalar joint. A long-term follow-up study.

From 1975 to 1990 we performed subtalar or triple arthrodesis on 54 patients; 48 of them were reviewed after a mean follow-up of 10 years (6 to 15). There were 17 subtalar fusions in 14 patients and 37 triple arthrodeses in 28 patients. We assessed tibiotalar ankle function using the criteria of Mazur which gives a points score of a maximum of 100. Radiological evidence of degenerative change was graded on a scale of 0 to 4. The mean Mazur score was 85 for the subtalar fusions and 78 for the triple arthrodeses. The radiological score showed no degenerative changes in 36 feet (24 triple and 12 subtalar arthrodeses) and an increase of one grade in 14 feet (10 triple and 4 subtalar), of two grades in three feet (all triple arthrodeses) and of three grades in one foot after a subtalar arthrodesis. We found no statistically significant difference in the radiological score in unilateral fusions between feet with subtalar and triple arthrodeses and the contralateral foot. In all four feet which showed an increase in degenerative changes of two or more grades, there was an abnormality of the tibiotalar joint before the fusion operation. Of the 14 feet which showed an increase of one grade, there was a similar increase on the contralateral side in nine. Our findings show that subtalar or triple arthrodesis has little adverse influence on the function of the tibiotalar joint, even after many years.

[Pseudarthrosis of the proximal femur]. / Pseudarthrosen am proximalen Femur.

Mechanical and biological factors are responsible for non-unions of the proximal femur. We analyse the causal treatment-possibilities of the different localisations. Fifty-five patients with non-unions of the femoral neck (average age 53 years) with or without preexistent femoral head necrosis (44%) were treated by abduction osteotomy and followed up at regular intervals. In 15% of cases a second operation was necessary after an average of 9.3 years, including the early complications. At the latest control 90% of the patients were satisfied, with an average Harris hip score (HHS) of 91. The survivorship analysis with end point total hip replacement is favourable. In the same period 22 patients were treated with a total hip replacement. The 11 survivors had a clearly worse HHS of 65. The low-risk, technically demanding valgization osteotomy should be the first step in the treatment of femoral neck non-unions, even in the presence of femoral head necrosis; secondary operations are not compromised. Pertrochanteric non-unions are rare. The pertrochanteric fragment very often heals, leaving a lateral femoral neck non-union which can be treated with valgization osteotomy. Depending on the type of non-union and the age of the patient, anatomical reduction, medial displacement and valgization osteotomy can be employed. With the angulated plates of the ASIF (95 degrees, 120 degrees, 130 degrees) 23 of the 24 non-unions could be healed in one operation. Fourteen patients underwent total hip replacement. In the subtrochanteric area mechanical and vascular instability leads to implant failure or fatigue fracture. Rigid compression-re-osteosynthesis is the therapy of choice, the 95 degrees condylar plate the implant. Twenty-three of our documented 24 subtrochanteric non-unions healed, 4 in the presence of an infection. Multiple operations have been necessary in 2 of the 4 non-unions following a pathological fracture.

Failure of hypertrophy in revascularised fibula grafts due to stress protection.

Three cases of long bone reconstruction with vascularised fibula grafts are described. Hypertrophy of the graft did not occur after a follow-up of 3-5 years. Three different kinds of stress shielding appeared to prevent transformation of the graft.

Reconstruction of congenital tibial pseudarthrosis by revascularized fibular transplants.

Seven patients with congenital Boyd type II pseudoarthrosis of the tibia and fibula were treated with a revascularized osteocutaneous fibular graft from the contralateral side. Follow-up ranged from 20 to 96 months (mean 67 months). All patients had previously undergone major surgery (mean three operations). Union was successful in all patients although three required further surgery. At follow-up all patients were able to walk without external support. The results were classified as good in two cases and fair in five cases. The postoperative period was, however, often complicated by stress-fracture and nonunion. Leg length discrepancy and valgus deformity are still unsolved problems in some cases. It seems probable that a primary reconstruction at an early age will prevent many of these subsequent problems.

[Malunited juvenile upper and lower leg fractures. Spontaneous correction, complications, indication for corrective intervention]. / Fehlverheilte kindliche Ober- und Unterschenkelschaftbrüche. Spontankorrektur, Komplikationen, Indikation zu Korrektureingriffen.

If malunion occurs after shaft fractures of the femur or tibia, it often corrects itself spontaneously. If the remaining growth period is +/- 2 years, side to side dislocations, shortening and varus deformities (up to 15 degrees) are equalized. Valgus, recurvation and antecurvation equalize much more slowly and rotation deformities only at the femur. The indication to perform a corrective osteotomy before the end of growth is therefore rare, and the remaining axis deviations have a good long-term prognosis as far as arthritic changes are concerned. Exceptions of this rule are rotation deformities in the lower leg and progressive valgus after high tibial fractures. Contractures instabilities and overuse of neighbouring joints represent another indication for early correction osteotomies. Risk of damage to the growth plate limits the possibilities for performing corrective osteotomies in young children. On the other hand, stable fixation is of less importance. At the end of growth, the procedures are the same as for adults.

[Malalignment following injuries of the distal tibia and epiphysis of the fibula]. / Fehlstellungen nach Verletzungen der distalen Tibia und Fibulaepiphysen.

If the correct treatment is applied, growth disturbances following epiphyseal fractures of the distal tibia are rare. Even if the prognosis is good after the correct treatment of epiphyseal fractures of the distal tibia, the parents and child may have to be informed about growth disturbances. Most frequent is a varus deformity after asymmetric closure of the medial growth plate. If at least 2 years of growth can be expected, a callus resection according to Langenskjöld is indicated. If this procedure fails, the ideal timing for a correction is the end of the growth. Imminent contractures can be an indication for early correction even if a recurrent deformity can be expected. An open-wedge osteotomy permits all three procedures to be carried out: callus resection, axis correction and leg-length correction. If an incongruence of the joint is present, the closed wedge technique using an external fixator is indicated; correction of the leg length can be postponed. Joint incongruence reacts well to axis alignment. The pre-arthrotic joint can remain free of symptoms for years. We never encountered epiphyseal fractures of the distal fibula, but we did see one case of deformity of the ankle joint as a result of closure of the proximal fibular growth plate.

Results of revision of hip arthroplasty with cement. A five to fourteen-year follow-up study.

We reviewed the results of sixty revisions of cemented total hip replacement in fifty-four patients who had complete clinical and radiographic follow-up. All of the revisions were performed by a standardized operative technique, in which both components of a single model of prosthesis were fixed with cement. In nineteen of the sixty revisions, autogenous grafts from the iliac crest were used for a deficiency in the roof of the acetabulum. After five to fourteen years, there had been two infections and four additional revisions for aseptic failure. Three cups had migrated, and another seven were surrounded by a complete radiolucent line. Five femoral components had subsided, and another eleven were surrounded by a complete radiolucent line. Survivorship analysis of eighty revisions showed a cumulative survival of about 85 per cent at fourteen years. In these relatively old patients, the outcome of revision of hip replacement with the described operative technique was very satisfactory.

[Treatment of tibial shaft fractures using plate osteosynthesis]. / Behandeling van tibiaschachtfracturen met plaatosteosynthese.

A retrospective study of 203 fractures of the tibial shaft, treated with ASIF-plate osteosynthesis between 1975 and 1986, revealed that the rate of osteitis and pseudarthrosis in closed and first-degree open fractures compares favourably with nationwide figures. The same applies to the duration of disability. Early treatment of osteitis, delayed union and pseudarthrosis in all cases led to complete recovery. Moving to modern hospital premises appears to have lowered the prevalence of infectious complications. Despite several negative prognostic factors, the results showed that ASIF-plate osteosynthesis if correctly performed on the right indication, is still the treatment of choice for a large group of patients with fractures of the tibial shaft.

[Acute infantile osteomyelitis]. / Acute zuigelingen-osteomyelitis.

In infants acute haematogenous osteomyelitis presents with few and subtle clinical signs. Only early diagnosis and proper treatment of this orthopedic emergency are able to prevent late deformities. Delay in diagnosis, persistence in apparently not effective conservative therapy and inadequate surgery lead to ugly and severe deformities, of which the corrective treatment is a heavy burden for the child and its parents.

[Fractures in children]. / Fracturen bij kinderen.

Children's fractures can be divided into shaft fractures, epiphysiolysis and epiphyseal fractures. Shaft fractures always heal with conservative methods. Slight axis deviations and shortening will correct spontaneously, while fracture disease and pseudarthrosis are extremely rare. Nevertheless, acute complications such as compartment syndromes deserve special attention. Epiphyseal fractures call for anatomical reduction and foolproof fixation to prevent growth disturbance through partial or complete closure of the growth plate. Such growth disturbance is not to be expected in case of epiphysiolysis.