Aims. Given the possible radiation damage and inaccuracy of radiological investigations, particularly in children, ultrasound and superb microvascular imaging (SMI) may offer alternative methods of evaluating new
Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD. A retrospective review at three paediatric tertiary centres identified 101 cases of tibial OFD in 99 patients. The clinical records, radiological images, and histology were analyzed.Aims
Methods
Two types of fracture, early and late, have been
reported following limb lengthening in patients with achondroplasia (ACH)
and hypochondroplasia (HCH). . We reviewed 25 patients with these conditions who underwent 72
segmental limb lengthening procedures involving the femur and/or
tibia, between 2003 and 2011. Gender, age at surgery, lengthened
segment, body mass index, the shape of the callus, the amount and
percentage of lengthening and the healing index were evaluated to determine
predictive factors for the occurrence of early (within three weeks
after removal of the fixation pins) and late fracture (>
three weeks
after removal of the pins). The Mann‑Whitney U test and Pearson’s
chi-squared test for univariate analysis and stepwise regression
model for multivariate analysis were used to identify the predictive factor
for each fracture. Only one patient (two tibiae) was excluded from
the analysis due to excessively slow formation of the regenerate,
which required supplementary measures. A total of 24 patients with
70 limbs were included in the study. There were 11 early fractures in eight patients. The shape of
the callus (lateral or central callus) was the only statistical
variable related to the occurrence of early fracture in univariate
and multivariate analyses. Late fracture was observed in six limbs
and the mean time between removal of the fixation pins and fracture
was 18.3 weeks (3.3 to 38.4). Lengthening of the tibia, larger healing
index, and lateral or central callus were related to the occurrence
of a late fracture in univariate analysis. A multivariate analysis
demonstrated that the shape of the callus was the strongest predictor
for late fracture (odds ratio: 19.3, 95% confidence interval: 2.91
to 128). Lateral or central callus had a significantly larger risk
of fracture than fusiform, cylindrical, or concave callus. Radiological monitoring of the shape of the callus during distraction
is important to prevent early and late fracture of lengthened limbs
in patients with ACH or HCH. In patients with thin callus formation,
some measures to stimulate
The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation.Aims
Patients and Methods
To compare the structural durability of hydroxyapatite-tricalcium
phosphate (HATCP) to autologous iliac crest bone graft in calcaneal
lengthening osteotomy (CLO) for pes planovalgus in childhood. We present the interim results of ten patients (HATCP, n = 6
and autograft, n = 5) with a mean age of 11.5 years (8.2 to 14.2)
from a randomised controlled non-inferiority trial with six months
follow-up. The primary outcome was the stability of the osteotomy
as measured by radiostereometric analysis. A non-inferiority margin
of ≤ 2 mm osteotomy compression was set.Aims
Patients and Methods
The aims of this study were to describe the course of non-operatively
managed, bilateral Perthes’ disease, and to determine specific prognostic
factors for the radiographic and clinical outcome. We identified 40 children with a mean age of 5.9 years (1.8 to
13.5), who were managed non-operatively for bilateral Perthes’ disease
from our prospective, multicentre study of this condition, which
included all children in Norway who were diagnosed with Perthes’
disease in the five-year period between 1996 and 2000. All children
were followed up for five years. The hips were classified according to the Catterall classification.
A modified three-group Stulberg classification was used as an outcome
measure, with a spherical femoral head being defined as a good outcome,
an oval head as fair, and a flat femoral head as a poor outcome. Aims
Patients and Methods
We describe three cases of infantile tibia vara
resulting from an atraumatic slip of the proximal tibial epiphysis
upon the metaphysis. There appears to be an association between
this condition and severe obesity. Radiologically, the condition
is characterised by a dome-shaped metaphysis, an open growth plate
and disruption of the continuity between the lateral borders of
the epiphysis and metaphysis, with inferomedial translation of the
proximal tibial epiphysis. All patients were treated by realignment
of the proximal tibia by distraction osteogenesis with an external
circulator fixator, and it is suggested that this is the optimal
method for correction of this complex deformity. There are differences
in the radiological features and management between conventional
infantile Blount’s disease and this ‘slipped upper tibial epiphysis’
variant.
Proximal femoral resection (PFR) is a proven
pain-relieving procedure for the management of patients with severe cerebral
palsy and a painful displaced hip. Previous authors have recommended
post-operative traction or immobilisation to prevent a recurrence
of pain due to proximal migration of the femoral stump. We present
a series of 79 PFRs in 63 patients, age 14.7 years (10 to 26; 35
male, 28 female), none of whom had post-operative traction or immobilisation. A total of 71 hips (89.6%) were reported to be pain free or to
have mild pain following surgery. Four children underwent further
resection for persistent pain; of these, three had successful resolution
of pain and one had no benefit. A total of 16 hips (20.2%) showed
radiographic evidence of heterotopic ossification, all of which
had formed within one year of surgery. Four patients had a wound
infection, one of which needed debridement; all recovered fully.
A total of 59 patients (94%) reported improvements in seating and
hygiene. The results are as good as or better than the historical results
of using traction or immobilisation. We recommend that following
PFR, children can be managed without traction or immobilisation,
and can be discharged earlier and with fewer complications. However,
care should be taken with severely dystonic patients, in whom more
extensive femoral resection should be considered in combination
with management of the increased tone. Cite this article:
Congenital pseudarthrosis of the tibia is an uncommon manifestation of neurofibromatosis type 1 (NF1), but one that remains difficult to treat due to anabolic deficiency and catabolic excess. Bone grafting and more recently recombinant human bone morphogenetic proteins (rhBMPs) have been identified as pro-anabolic stimuli with the potential to improve the outcome after surgery. As an additional pharmaceutical intervention, we describe the combined use of rhBMP-2 and the bisphosphonate zoledronic acid in a mouse model of NF1-deficient fracture repair. Fractures were generated in the distal tibiae of neurofibromatosis type 1-deficient ( When only rhBMP but no zoledronic acid was used to promote repair, 75% of fractures in These data support the concept that preventing bone loss in combination with anabolic stimulation may improve the outcome following surgical treatment for children with congenital pseudarthoris of the tibia and NF1.
We present a retrospective study of 27 patients treated by callus distraction using a unilateral external fixator of our own design for nonunion with bone loss and shortening of the femur caused by suppurative osteomyelitis. The unilateral external fixator was used either alone or in combination with an intramedullary nail. The mean age of the patients was 13.6 years (8 to 18). The fixator was used alone in 13 patients and with an intramedullary nail in 14. The bone results at a mean follow-up of 88 months (37 to 144) were excellent in 16 patients and good in 11. The functional results were excellent in 18 patients and good in nine. However, four patients still had draining sinuses at the latest follow-up. A residual deformity greater than 7° was present in seven femora, but this did not adversely affect function or require further treatment.
Extensive limb lengthening may be indicated in achondroplastic patients who wish to achieve a height within the normal range for their population. However, increasing the magnitude of lengthening is associated with further complications particularly adjacent joint stiffness and fractures. We studied the relationship between the magnitude of femoral lengthening and callus pattern, adjacent joint stiffness and fracture of the regenerate bone in 40 femoral lengthenings in 20 achondroplastic patients. They were divided into two groups; group A had lengthening of less than 50% and group B of more than 50% of their initial femoral length. The patterns of radiological callus formation were classified according to shape, type and features. The incidence of callus features, knee stiffness and regenerate bone fracture were analysed in the two groups. Group B was associated with an increased incidence of concave, lateral and central callus shapes, adjacent joint and stiffness and fracture. Statistically, the incidence of stiffness in adjacent joints and regenerate bone fracture was significantly associated with the magnitude of lengthening. We suggest that careful radiological assessment of the patterns of callus formation is a useful method for the evaluation and monitoring of regenerate bone.
The use of recombinant human bone morphogenetic protein-2 (rhBMP-2) for the treatment of congenital pseudarthrosis of the tibia has been investigated in only one previous study, with promising results. The aim of this study was to determine whether rhBMP-2 might improve the outcome of this disorder. We reviewed the medical records of five patients with a mean age of 7.4 years (2.3 to 21) with congenital pseudarthrosis of the tibia who had been treated with rhBMP-2 and intramedullary rodding. Ilizarov external fixation was also used in four of these patients. Radiological union of the pseudarthrosis was evident in all of them at a mean of 3.5 months (3.2 to 4) post-operatively. The Ilizarov device was removed after a mean of 4.2 months (3.0 to 5.3). These results indicate that treatment of congenital pseudarthrosis of the tibia using rhBMP-2 in combination with intramedullary stabilisation and Ilizarov external fixation may improve the initial rate of union and reduce the time to union. Further studies with more patients and longer follow-up are necessary to determine whether this surgial procedure may significantly enhance the outcome of congenital pseudarthrosis of the tibia, considering the refracture rate (two of five patients) in this small case series.
Nonunion of the humerus with bone loss and shortening due to osteomyelitis is rare but difficult to treat. We describe our experience with a callus distraction technique using a monolateral external fixator for the treatment of this condition. Between October 1994 and January 2004, 11 patients were treated. There were seven males and four females, with a mean age of 14 years (10 to 17). The mean bone loss was 1.9 cm (1 to 2.7) and the mean length discrepancy in the upper limb was 5.6 cm (3.5 to 8.0). The mean follow-up was for 106 months (54 to 166). The mean external fixation index was 34.8 days/cm (29.8 to 40.5). The mean lengthening was 9.5 cm (5.5 to 13.4). There were seven excellent results, three good and one poor. There were nine excellent functional results and two good. The treatment of humeral nonunion with bone loss and shortening due to osteomyelitis by callus distraction is a safe and effective means of improving function and cosmesis.
This nationwide prospective study was designed to determine prognostic factors and evaluate the outcome of different treatments of Perthes’ disease. A total of 28 hospitals in Norway were instructed to report all new cases of Perthes’ disease over a period of five years and 425 patients were reported and followed for five years. Of these, 368 with unilateral disease were included in the present study. The hips were classified radiologically according to a modified two-group Catterall classification and the lateral pillar classification. A total of 358 patients (97%) attended the five-year follow-up, when a modified three-group Stulberg classification was used as a radiological outcome measure. For patients over six years of age at diagnosis and with more than 50% necrosis of the femoral head (152 patients), the surgeons at the different hospitals had chosen one of three methods of treatment: physiotherapy (55 patients), the Scottish Rite abduction orthosis (26), and proximal femoral varus osteotomy (71). Of these hips, 146 (96%) were available for the five-year follow-up. The strongest predictor of outcome was femoral head involvement of more or less than 50% (odds ratio (OR) = 7.76, 95% confidence interval (CI) 2.82 to 21.37), followed by age at diagnosis (OR = 0.98, 95% CI 0.92 to 0.99) and the lateral pillar classification (OR = 0.62, 95% CI 0.40 to 0.98). In children over six years at diagnosis with more than 50% of femoral head necrosis, proximal femoral varus osteotomy gave a significantly better outcome than orthosis (p = 0.001) or physiotherapy (p = 0.001). There was no significant difference between the physiotherapy and orthosis groups (p = 0.36), and we found no difference in outcome after any of the treatments in children under six years (p = 0.73). We recommend proximal femoral varus osteotomy in children aged six years and over at the time of diagnosis with hips having more than 50% femoral head necrosis. The abduction orthosis should be abandoned in Perthes’ disease.
Deformity of the forearm due to growth disturbance of the ulna occurs in a number of conditions such as ulnar deficiency, multiple exostoses, and neurofibromatosis. We report a previously unrecognised form, caused by focal cortical indentation. We have treated five children with this condition, three girls and two boys; the mean age at presentation was 5 years (2 to 8). The deformity was first recognised about the age of two years, and progressed gradually. The radiological findings were the same in all cases. The focal cortical indentation was seen at the distal end of the ulna with anteromedial bowing and dysplasia. The radial head was dislocated posterolaterally. In one patient the histological findings at the site of indentation were of a fold of tissue resembling periosteum, which interfered with enchondral ossification. Treatment by ulnar lengthening using an external fixator and osteotomy which corrected both the ulnar deformity and reduced the dislocated radial head in two cases gave the best results.
