We investigated patterns of refracture and their risk factors in patients with congenital pseudarthrosis of the tibia after Ilizarov osteosynthesis. We studied 43 cases in 23 patients. Temporal and spatial patterns of refracture and refracture-free survival were analysed in each case. The refracture-free rate of cumulative survival was 47% at five years and did not change thereafter. Refracture occurred at the previous
The study describes a technique of tibial autograft to augment posterior instrumented spinal fusion in a population of paediatric patients with severe idiopathic, neuromuscular or syndromic scoliosis who are at a higher risk of postoperative
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
We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity. We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a
PURPOSE. To report the experience and evolution of treatment of Congenital Pseudarthrosis of the Tibia in a single surgeon, consecutive series of 19 patients. METHODS. A retrospective case series review, with radiological assessment and functional outcome scores including the Activity Scale for Kids (ASK) and the Oxford Ankle Foot Questionnaire (OAFQ) Child & Teenager version. Patients were classified as per Boyd & Andersen. Alteration in the Tibial mechanical axis was compared pre-treatment and at last review. RESULTS. The average follow up from presentation was 6.3 years, 7 patients had reached skeletal maturity. Union was obtained in 79% at the time of review with a mean time to union of an established
A total of 33 children were treated for acute traumatic dislocation of the elbow between 1994 and 2002; 30 dislocations were posterior and three anterior. Eight children had a pure dislocation and 25 had an associated fracture of the elbow. Two had compound injuries. Two children had injury to the ulnar nerve, one to the radial nerve and one to the median nerve together with injury to the brachial artery. Twenty required open reduction. Complications included
Congenital pseudarthrosis of the tibia (CPT)
is a rare but well recognised condition. Obtaining union of the pseudarthrosis
in these children is often difficult and may require several surgical
procedures. The treatment has changed significantly since the review
by Hardinge in 1972, but controversies continue as to the best form
of surgical treatment. This paper reviews these controversies. Cite this article:
Congenital unilateral anterolateral tibial bowing in combination with a bifid ipsilateral great toe is a very rare deformity which resembles the anterolateral tibial bowing that occurs in association with congenital pseudarthrosis of the tibia. However, spontaneous resolution of the deformity without operative treatment and with a continuously straight fibula has been described in all previously reported cases. We report three additional cases and discuss the options for treatment. We suggest that this is a specific entity within the field of anterolateral bowing of the tibia and conclude that it has a much better prognosis than congenital pseudarthrosis of the tibia, although conservative treatment alone may not be sufficient.
Our aim was to review the efficacy of the wound vacuum-assisted closure (VAC) system in the treatment of deep infection after extensive instrumentation and fusion for spinal deformity in children and adolescents. A total of 14 patients with early deep spinal infection were treated using this technique. Of these, 12 had neuromuscular or syndromic problems. Clinical and laboratory data were reviewed. The mean follow-up was 44 months (24 to 72). All wounds healed. Two patients required plastic surgery to speed up the process. In no patient was the hardware removed and there was no loss of correction or recurrent infection. We believe that the wound VAC system is a useful tool in the armamentarium of the spinal surgeon dealing with patients susceptible to wound infections, especially those with neuromuscular diseases. It allows for the retention of the instrumentation and the maintenance of spinal correction. It is reliable and easy to use.
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:
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.
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 have evaluated the clinical outcomes of simple excision, ulnar lengthening and the Sauvé-Kapandji procedure in the treatment of deformities of the forearm in patients with multiple hereditary osteochondromas. The medical records of 29 patients (33 forearms) were reviewed; 22 patients (22 forearms) underwent simple excision (four with ulnar lengthening) and seven the Sauvé-Kapandji procedure. Simple excision increased the mean supination of the forearm from 63.2° to 75.0° (p = 0.049). Ulnar lengthening did not significantly affect the clinical outcome. The Sauvé-Kapandji technique improved the mean pronation from 33.6° to 55.0° (p = 0.047) and supination from 70.0° to 81.4° (p = 0.045). Simple excision may improve the range of movement of the forearm but will not halt the progression of disease, particularly in younger patients. No discernable clinical or radiological improvement was noted with ulnar lengthening. The Sauvé-Kapandji procedure combined with simple excision of osteochondromas can improve stability of the wrist, movement of the forearm and the radiological appearance.
Osteofibrous dysplasia is an unusual developmental condition of childhood, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial and some authors believe that they are part of one histological process. We retrospectively reviewed 16 patients who were diagnosed as having osteofibrous dysplasia initially or on the final histological examination. Their management was diverse, depending on the severity of symptoms and the extent of the lesion. Definitive (extraperiosteal) surgery was localised ‘shark-bite’ excision for small lesions in five patients. Extensive lesions were treated by segmental excision and fibular autograft in six patients, external fixation and bone transport in four and proximal tibial replacement in one. One patient who had a fibular autograft required further excision and bone transport for recurrence. Six initially underwent curettage and all had recurrence. There were no recurrences after localised extraperiosteal excision or bone transport. There were three confirmed cases of adamantinoma. The relevant literature is reviewed. We recommend extraperiosteal excision in all cases of osteofibrous dysplasia, with segmental excision and reconstruction in more extensive lesions.