Dysplasia epiphysealis hemimelica of the elbow.

Dysplasia epiphysealis hemimelica (DEH) or Trevor's disease is a rare disorder affecting the epiphyses and short bones of the limbs, and characterized by a benign overgrowth of the medial-half of the epiphysis resembling osteochondroma. Upper limb involvement is very rare and most commonly affects the carpal bones. Only five established cases of DEH around the elbow were found in the orthopedic literature. We herein report an additional case of Trevor's disease of the elbow in a child with a symptomatic ulnar nerve compression and its management. To the best of our knowledge, this is the first case of DEH of the elbow presenting with nerve compromise.

Is percutaneous adductor tenotomy as effective and safe as the open procedure?

BACKGROUND: Percutaneous adductor longus tenotomy (PAT) is a frequently used procedure, yet no study has ever compared its effectiveness and safety with those of open adductor longus tenotomy (OAT). We conducted this prospective study to describe the effects of PAT and to compare them with those of OAT. METHODS: This consisted of a cross-over randomized controlled trial including 50 consecutive hips from 27 patients with cerebral palsy scheduled for adductor tenotomy in the setting of multilevel tendon lengthening/release procedures or hip surgery (femoral or Dega osteotomy) in a university hospital. A pediatric orthopaedic surgeon conducted a PAT. Another surgeon extended the wound to explore what had been cut during the PAT, and completed the tenotomy if necessary. Hip abduction (HA) was assessed by a third surgeon immediately before PAT, after PAT, and then after OAT, using a goniometer, in a standardized reproducible manner. All 3 surgeons were blinded to the others' findings. Primary end-points included the percentage of tendon/muscle portion sectioned percutaneously, and the HA measure. Comparison between HA after PAT and OAT was done using a paired t-test with a 95% confidence interval. The influence of anatomic variants of adductor longus origin was also assessed. RESULTS: Mean HA (hips flexed) measured 40.36 degrees preoperatively and increased to 50.04 degrees after PAT (P<0.0001). After OAT, HA averaged 53.32 degrees with no statistical gain compared with that observed after PAT (P=0.2). The tendinous portion of adductor longus was cut to an average of 98% by PAT (completely in 46 cases and more than 75% in only 4 cases). The muscular portion of adductor longus origin was cut to an average of 83.7% (completely in only 15 cases, cut to more than 75% in 26 cases, and approximately 50% in 9 cases). The gain in HA positively correlated with the extent of the tendinous portion divided (P=0.03) but not with the extent of muscular portion divided. Results were independent of the anatomic variants of adductor longus origin. Partial section of adductor brevis after PAT was encountered in 6 cases. No major iatrogenic lesion was observed (obturator nerve, major vessels). CONCLUSIONS: This is the only prospective study concerning the effects of PAT. The anatomic factor associated with gain in HA seems to be the extent of the section of the tendinous portion of adductor longus origin, which was found to be cut to more than 90% in all cases after PAT. The extent of muscular portion section does not seem to influence the gain in HA. The researchers detail the technique of percutaneous adductor tenotomy and show that when done correctly, PAT is a fast and simple procedure, as reliable and effective as the open release and without any major risks. LEVEL OF EVIDENCE: Level II therapeutic study-prospective comparative study.

Lateral shelf acetabuloplasty in the treatment of Legg-Calvé-Perthes disease: improving mid-term outcome in severely deformed hips.

PURPOSE: To evaluate the efficacy and safety of lateral shelf acetabuloplasty in Legg-Calvé-Perthes (LCP) hips classically associated with poor prognosis. METHODS: A retrospective study was conducted on 30 consecutive pediatric patients (average age 8.6 years) presenting with a severe and progressive form of LCP disease, with (16 hips) or without (14 hips) femoral varus osteotomy (FVO), and treated by lateral shelf acetabuloplasty. Shelf was done on hips presenting an aspherical incongruency with flattening, subluxation, and lack of femoral head coverage, as demonstrated on pre-operative radiographs and arthrography. All patients were reexamined at an average follow-up of 9.5 years (range 5.2-12 years). Clinical, radiological, and computed tomography scan evaluations were undertaken. Stulberg and Mose classifications were applied as radiological indicators of prognosis. Statistical analysis was performed using Student's t test and the Pearson correlation test with variance analysis for repetitive measures. RESULTS: At the last follow-up, all patients were pain free and had normal or almost normal hip motion. Twenty-seven patients are now able to walk normally or with a slight limp. Eighteen hips are classified as Stulberg 1 or 2, and 18 hips are classified as Mose 1 or 2. The average neck-shaft angle is 127°. A statistically significant improvement in the majority of radiographic parameters was found. There was no statistically significant worsening of leg length discrepancy following the procedure. The scanographic study found neither offset nor migration of the shelf in any of the hips. The average coronal and anteroposterior length of the shelf was 9.9 and 25 mm, respectively. A borderline positive correlation was found between Wiberg angle improvement and young age at the time of surgery. There was no statistically significant difference between hips that underwent shelf procedures alone and those in which it was combined with FVO. CONCLUSION: Lateral shelf acetabuloplasty improves the outcome of hips with severe LCP. The combined procedure insures a better and lasting coverage and remodeling of the femoral head, while preserving acetabular roof growth.

Risk of recurrent dislocation and avascular necrosis after proximal femoral varus osteotomy in children with cerebral palsy.

A retrospective review was conducted on 50 children with cerebral palsy, having 89 femoral varus osteotomy at an average age of 7.4 years, trying to identify risk factors for complications, particularly redislocation and avascular necrosis. Among the well-reduced hips, new subluxation developed in 12 cases; postoperative radiographic measurements showed a mean neck-shaft angle of 135 degrees and an acetabular slope of 32 degrees . Main risk factors for secondary dislocation seem to be insufficient correction of preexisting valgus and uncorrected acetabular dysplasia. Avascular necrosis was observed in 33 hips (37%): 26 minor and seven severe. Older age at surgery and high preoperative Reimer's migration index seem statistically significant risk factors.

Is visual estimation of passive range of motion in the pediatric lower limb valid and reliable?

BACKGROUND: Visual estimation (VE) is an essential tool for evaluation of range of motion. Few papers discussed its validity in children orthopedics' practice. The purpose of our study was to assess validity and reliability of VE for passive range of motions (PROMs) of children's lower limbs. METHODS: Fifty typically developing children (100 lower limbs) were examined. Visual estimations for PROMs of hip (flexion, adduction, abduction, internal and external rotations), knee (flexion and popliteal angle) and ankle (dorsiflexion and plantarflexion) were made by a pediatric orthopaedic surgeon (POS) and a 5th year resident in orthopaedics. A last year medical student did goniometric measurements. Three weeks later, same measurements were performed to assess reliability of visual estimation for each examiner. RESULTS: Visual estimations of the POS were highly reliable for hip flexion, hip rotations and popliteal angle (rhoc >or= 0.8). Reliability was good for hip abduction, knee flexion, ankle dorsiflexion and plantarflexion (rhoc >or= 0.7) but poor for hip adduction (rhoc = 0.5). Reproducibility for all PROMs was verified. Resident's VE showed high reliability (rhoc >or= 0.8) for hip flexion and popliteal angle. Good correlation was found for hip rotations and knee flexion (rhoc >or= 0.7). Poor results were obtained for ankle PROMs (rhoc < 0.6) as well as hip adduction and abduction, the results of which not being reproducible. Influence of experience was clearly demonstrated for PROMs of hip rotations, adduction and abduction as well as ankle plantarflexion. CONCLUSION: Accuracy of VE of passive hip flexion and knee PROMs is high regardless of the examiner's experience. Same accuracy can be found for hip rotations and abduction whenever VE is performed by an experienced examiner. Goniometric evaluation is recommended for passive hip adduction and for ankle PROMs.

Radiofrequency application to the growth plate in the rabbit: a new potential approach to epiphysiodesis.

BACKGROUND: Radiofrequency energy is being used more and more frequently in orthopaedics, mainly in the treatment of bone tumors. We postulated that radiofrequency ablation may produce growth plate lesions similar to those observed in the bone and conducted this study to see whether radiofrequency may be used as a technique for producing epiphysiodesis. METHODS: We randomized 60 8-week-old female New Zealand white rabbits into 3 groups. Group A was destined for a total epiphysiodesis at 60 degrees C, group B was destined for a total epiphysiodesis at 90 degrees C, and group C for a lateral hemiepiphysiodesis at 90 degrees C. Radiofrequency energy was delivered in 1 minute in all 3 groups. Using fluoroscopic imaging, radiofrequency was applied percutaneously to the left proximal tibial physis whereas the right growth plate received a sham procedure. A bicortical pin was used to evaluate the longitudinal growth rate at every monthly radiologic control, beginning 8 weeks after the procedure. Comparisons between the right and left side and between groups A and B were achieved using a paired t test. A histopathologic study was conducted in parallel to the radiographic study. RESULTS: In a radiograph at the 8-week point, pin migration was 4.74 mm on the left side compared with 9.72 mm on the right (P<0.0001), in group A. In group B, pin migration on the left was 1.37 mm compared with 5.49 mm on the right (P<0.0001). In group C, mean angular deviation was 11.6 degrees on the left compared with 1.9 degrees on the right (P=0.0001). These differences were maintained until the end of growth. Pathology specimens revealed cellular anarchy, loss of columnar stratification, and height of the physis on the left side, which occurred earlier and were more pronounced in group B than in group A. In group C, these changes involved only the lateral half of the left physis whereas its medial counterpart remained normal. There was no evidence of articular cartilage damage. CONCLUSIONS: This experimental study shows that radiofrequency can efficiently and rapidly achieve epiphysiodesis. It is one of many methods that can be used for this purpose. The development of new electrodes suitable for use on human growth plates and the elaboration of specific utilization protocols may lead to its use in children. Its simplicity and precision may lead to a quick and efficient growth arrest with little pain and postoperative disability in addition to reduced costs. CLINICAL RELEVANCE: Radiofrequency has proved to be effective in producing growth arrest in rabbits with no complications. Technical improvements and adaptations may allow its use for pediatric limb inequalities or angular deformities in the near future.

Free vascularized fibular flap for congenital ulnar pseudarthrosis: a report of two cases and review of the literature.

Congenital pseudarthrosis of the ulna (CPU) is an extremely rare disorder that has been treated with various traditional methods with poor results on long-term deformity correction and bony union. Free vascularized fibular grafting (FVFG) has been used to treat congenital pseudarthroses with improved success rates. We report herein 2 cases of CPU treated with FVFG in which bone union was obtained without major difficulty. The distal ulnar hypoplastic physis and epiphysis were spared during excision of the pseudarthrosis in both cases leading to continuous ulnar growth following bone healing. The main challenge concerned the stability of the radiocapitellar joint. There was no complication at the donor site. Free vascularized fibula is a safe and effective method for the treatment of congenital pseudarthrosis of the ulna.

Is bone lengthening over an intramedullary prosthesis stem possible and safe?

Here we report the case of a 13-year-old girl with a history of massive left knee arthroplasty after excision of a proximal tibial osteosarcoma with subsequent leg length inequality. Owing to her short stature and her parents' refusal of surgical shortening by epiphysiodesis of the right lower limb, tibial lengthening over the prosthesis stem using an Ilizarov apparatus was undertaken and achieved 5 cm good quality regenerate but with a secondary infection leading to prosthesis removal. To the best of the authors' knowledge, no similar case has been previously reported in the literature. Although the goal of bone lengthening was achieved, this procedure should be avoided because of the high risk of secondary infection.

Multifocal Ewing sarcoma of the foot.

Multifocal skeletal Ewing sarcoma is very rare. A 10-year-old boy presented with multiple independent lesions involving the first phalanx of the middle toe, the second metatarsal bone, the cuneiform and cuboid bones, and the talus. Diagnosis of Ewing sarcoma was confirmed by open biopsy of 2 nonadjacent bones. Chest computed tomography disclosed the presence of multiple lung metastases. The patient underwent chemotherapy, below-knee amputation, and lung irradiation. To the best of the authors' knowledge, no similar case has been previously reported in the literature.

Skeletal features of primary hyperoxaluria type 1, revisited.

PURPOSE: The purpose of this study was to describe the skeletal manifestations of primary hyperoxaluria type 1 (PH1), the most common of the primary hyperoxalurias. METHODS: We clinically and radiographically reviewed 12 consecutive patients diagnosed with PH1, aged between 2 and 17 years. All patients had evidence of some type of renal involvement, 4 of whom were at end-stage renal disease (ESRD) and were under dialysis. RESULTS: The main symptom was skeletal pain and was present only in the 4 severely involved patients and appeared during the second year of dialysis. The 2 most severely involved patients had evidence of pathological fractures. Radiological signs were present in patients with or without symptoms. These radiological signs were of two distinct types: those almost specific of oxalosis, such as dense and radiolucent metaphyseal bands and vertebral osteocondensations, which are found mainly in the severely involved individuals, and those less specific, such as signs of renal osteodystrophy, which are also found in less severely involved patients. Interestingly, our study revealed the presence of spondylolysis in 25% of cases. This latter finding is unique and has not previously been reported in the literature. CONCLUSIONS: The skeletal manifestations of PH1 include specific and less specific radiological signs, with some patients being asymptomatic, and others presenting with bone pain and pathological fractures, as well as spondylolysis.

Pediatric cervical spine instability.

Cervical spine instability in children is rare but not exceptional and may be due to many factors. Although it mostly occurs at the upper cervical spine, all vertebrae from the occiput to T1 may be involved. It may be acute or chronic, occurring secondary to trauma or due to congenital anomaly, skeletal or metabolic dystrophy or rheumatoid arthritis. It can be isolated or associated with other musculoskeletal or visceral anomalies. A thorough knowledge of embryology, anatomy, physiology and physiopathology of the cervical spine in children is essential to avoid pitfalls, recognize normal variants and identify children at risk of developing cervical spine instability and undertake the appropriate treatment.

Atlantoaxial rotatory fixation owing to neck burn.

The purpose of this paper is to present a case of severe atlantoaxial rotatory fixation owing to a previously unreported etiology, and to discuss its pathogenesis and management. Conservative measures were unable to prevent progression, thus requiring surgical intervention.

A newly recognized autosomal recessive syndrome with short stature and oculo-skeletal involvement.

This report describes a young girl and her cousin presenting with postnatal short stature, strabismus, photophobia, retinitis pigmentosa, short neck, rhizomelic shortening of the long bones, short and slightly bowed humeri with prominent deltoid tuberosities, short and wide ribs and clavicles, dorso-lumbar scoliosis, biconcave vertebral bodies of the thoraco-lumbar spine, and narrowed lumbar canal. In addition, in the girl there were amelogenesis imperfecta of the hypomaturation type, and the radiographs showed short distal ulnae, sloping epiphyses of the radii, short femoral necks, and slightly flat uncovered femoral heads. The children's parents are first cousins. Differential diagnoses are discussed and the possibility of a newly recognized oculo-skeletal syndrome is raised.

The risk of neuro-orthopaedic malformations following in-vitro fertilization.

The purpose of this study was to assess the risk of neuro-orthopaedic malformations after in-vitro fertilization. We compared the prevalence of neuro-orthopaedic malformations in two groups of pregnancies conceived either naturally or by in-vitro fertilization, and used multivariate analysis to study the impact of each variable. The results showed a prevalence of 0.89% neuro-orthopaedic malformations in the in-vitro fertilization cohort and 0.32% in the natural conception cohort. The three times greater risk of malformations in the in-vitro fertilization cohort was reduced after adjustment to other variables. We concluded that the increased risk of neuro-orthopaedic malformations after in-vitro fertilization is not due to the technique itself but rather to factors associated with it.

Recurrent Legg-Calvé-Perthes disease revisited: fake or reality?

The objective of this study was to assess the course, the epidemiologic features and the prognosis of recurrent Legg-Calvé-Perthes disease. We reviewed seven reported cases and one personal case of recurrent Perthes' disease. In all cases, complete recovery from the previous episode was documented, and a thorough diagnostic work-up to rule out other diseases was undertaken. There were five boys. Age at onset ranged from 2 to 6 years. Five patients had bilateral involvement. Recurrence took place 1-5 years after complete healing of the initial one. Clinical and radiological pictures were similar to those found in primary Perthes' disease of the adolescent and consisted in a greater epiphyseal involvement. Recurrent Perthes' disease is very rare. We did not identify any risk factors for recurrence. A worse outcome is more closely related to an older age of the patient at recurrence than to recurrence itself.

Percutaneous radiofrequency coagulation of osteoid osteoma of the "Neural Spinal Ring".

STUDY DESIGN: Three cases of osteoid osteomas of the spine, adjacent to neural elements, were treated with CT-guided thermal coagulation. OBJECTIVE: To present an alternative method to conventional treatment of spinal osteoid osteoma. SUMMARY OF BACKGROUND DATA: Percutaneous CT-guided radiofrequency coagulation has been successfully used for osteoid osteoma of the extremities. The conventional management of spinal osteoid osteoma is by surgical resection. METHOD: Three patients with osteoid osteoma of the spine, adjacent to neural structures, were treated by percutaneous radiofrequency ablation. The procedure was performed in the CT room under general anesthesia and CT guidance. The thermocoagulation electrode was heated at 90 degrees C for 4 min. RESULTS: Two patients left the hospital the same day; the third one was kept 24 h for observation. All of them had total pain relief within 48 h following the procedure. No complications were recorded. There was no evidence of recurrence after a mean follow-up period of 17 months. CONCLUSION: Osteoid osteoma of the spine, adjacent to neural structures, can effectively and safely be treated by minimally invasive percutaneous CT guided radiofrequency coagulation.

Osteomyelitis of the rib due to Streptococcus pneumoniae: a very rare condition in children.

Rib osteomyelitis is a rare disease. We present a previously unreported case of Streptococcus pneumoniae osteomyelitis of the rib. A 4-month-old-infant presented with fever, irritability and abdominal tenderness. Pericostal collection was discovered incidentally on ultrasound; it was first drained by needle aspiration and appropriate antibiotic therapy was given, with resolution of fever in 24 h, but recurrence of symptoms 4 days later, with swelling over the affected rib. Surgical drainage with resection of the infected portion of the rib were done followed by a prolonged course of intravenous and then oral antibiotics, without any recurrence at 6 months follow-up. A review of the pertinent literature was made. This case demonstrates that the spectrum of pathogens potentially responsible for rib osteomyelitis may be broader than previously reported. The management and outcome are similar to rib osteomyelitis due to any other bacteria.

Percutaneous radiofrequency coagulation of osteoid osteoma in children and adolescents.

The purpose of this paper was to evaluate the effectiveness of percutaneous radiofrequency ablation of osteoid osteoma in children and adolescents. Twenty-three patients aged 4.5-19.5 years were retrospectively reviewed. All patients with lower limb lesions were able to bear full weight on their operated limb immediately after the procedure, and to resume their daily activities within 24-48 h. Pain disappeared immediately after surgery in 21 cases, and in lower limb lesions the gait was back to normal after an average of 5 days. No septic or neurovascular complications were observed. At an average follow-up of 3.5 years, all the patients including two cases of initial failure were free of pain and had a normal gait. Clinical healing was confirmed by computed tomography and bone scan performed in 12 cases. This precise and minimally invasive method is safe, effective and associated with reduced health care resources. It could be recommended as the treatment of choice for osteoid osteoma of the limbs in children and adolescents.