The results of a functional, clinical and radiological study of 30 children (60 hips) with whole body cerebral palsy are presented with a mean follow-up of ten years. Bilateral simultaneous combined soft-tissue and bony surgery was performed at a mean age of 7.7 years (3.1–12.2). Evaluation involved interviews with patient/carers and clinical examination. Plain radiographs of the pelvis assessed migration percentage and centre-edge angle. Twenty two patients were recalled. Five had died of unrelated causes and three were lost to follow-up. Pain was uncommon, present in 1 patient (4.5%). Improved handling was reported in 18 of 22 patients (82%). Carer handling problems were attributed to growth of the patients. All patients/carers considered the procedure worthwhile. The range of hip movements improved, with a mean windsweep index of 36 (50 pre-operatively) Radiological containment improved, with mean migration percentage of 20 degrees (50 preoperatively) and mean centre-edge angle of 29 degrees (−5 preoperatively) No statistical difference was noted between the three year and ten year follow-up results demonstrating maintained clinical and radiological outcome improvement. In conclusion, we consider that bilateral simultaneous combined hip reconstruction in whole body cerebral palsy provides painless, mobile and anatomically competent hips in the long term.
Patients were reviewed clinically and completed questionnaires documenting pain, function and satisfaction before and after treatment at a mean follow-up of 44 months (range 14–131). All patient’s notes and radiographs were examined.
Complications included almost universal minor pin-site infections, flexion contractures of the toes in 5 feet and skin ulceration in 2 feet, 1 requiring a muscle flap.
17 patients have undergone 20 microdrilling procedures to stimulate bone union in cases of established non-union. This occurred at the docking site following completion of bone transport using a stacked Taylor Spatial Frame, non-union following arthrodesis or non-union in long bone fracture. Additional bone grafting was performed in only one patient. Further stimulation of union via injection of Bone Morphogenetic Protein was undertaken with 3 microdrilling procedures. Of the 20 microdrilling procedures, 8 were considered fully successful in terms of stimulation of union, 7 were partially successful and 5 were not felt to have been successful. The mean time to fully successful union following microdrilling was 11.4 weeks, ranging from 6 to 19 weeks. There were 2 complications, both acute infections at the microdrilling site. Both of these were in patients with previous significant pin site infections. We present the use of a microdrilling technique as a safe and effective minimally invasive technique that promotes union in cases of refractory non-union, whilst avoiding the donor site morbidity associated with open bone grafting. We present, as a pilot study, our experience in the use of this technique in patients treated with circular frames for acute fractures, at the docking site in cases of bone transport and in cases of non-union following arthrodesis.
The majority of the available literature considers the short term outcome of surgical reconstruction of the hips in this condition. This paper demonstrates that the initial improvements in structure and function are maintained in the longer term.
We have treated 17 patients with bone defects of the tibia by internal bone transport using a stacked Taylor Spatial Frame. There were 12 cases of infected non unions, 2 cases of osteomyelitis, 1 case of acute traumatic bone loss, 1 case of non union in a patient with neurofibromatosis, and 1 case of pseudoarthrosis of the tibia. The mean bone defect was 51.8mm (range 10–100mm). Leg length has been restored to within 10mm in 16 cases and to within 15mm in one case. All patients have united. Residual deformity at the docking site or regenerate was negligible in 4 patients and less than 5 degrees in any plane in the remaining 13 patients. There have been two cases of re-fracture which have united with conservative treatment and 1 case of partial peroneal nerve palsy which is recovering. The use of a stacked Taylor Spatial Frame system is effective in mediating bone transport resulting in predictable regenerate, accurate docking and minimal induced bone deformity.
The purpose of this study is to demonstrate that definitive surgery (extraperiosteal excision) is required in patients with osteofibrous dysplasia (OFD) due to the risk of recurrence and co-existent adamantinoma OFD is an unusual childhood condition, 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 with some authors believing that they are part of one histological process. This therefore provides difficulty in recommending treatment options A retrospective review of OFD was conducted. Using the Stanmore Bone Tumour Unit database 22 cases were identified who were initially diagnosed with OFD or were diagnosed on final histology. All cases were tibial except one lesion in the ulna and one in the fibula Management was diverse depending on the severity of symptoms and the extent of the lesions encountered. Definitive (extraperiosteal) surgery in the majority of our patients was localized excision for small lesions (less than 50% of the bony circumference) and segmental excision followed by reconstructive surgery for more extensive ones. Seven patients had a sharkbite excision and a further seven were treated with fibula autografting. Of the latter group, one required further excision and bone transport due to recurrence of OFD. An additional five underwent bone transport &
distraction osteogenesis using the Ilizarov technique and one had a proximal tibial replacement. Nine initially underwent curettage, but eight recurred (recurrence rate 88.9%). No recurrences occurred following localized extraperiosteal excisions and bone transport. There were three confirmed cases of adamantinoma. In view of the risk of association of OFD with adamantinoma, and to some extent the continuous morbidity of OFD if left untreated, we believe that radical extraperiosteal excision is indicated in most if not all cases of OFD
The aim of the study was to assess the outcome of treatment of recurrent clubfoot deformity using the Ilizarov frame.
Objective: To review of an uncommon deformity arising in four patients. Method: A clinical and radiological review. Conclusions: heightened awareness and early treatment with monitoring is required. We describe four cases of distal radial epiphyseal dysplasia associated with a localised area of cutis aplasia congenita (CAC) over the dorsum of the distal forearm. The cutis aplasia was diagnosed at birth in all cases, but the radial dysplasia was not recognised until presentation to our orthopaedic department between the ages of 5 and 10 years. Radial dysplasia describes a spectrum of osseous, musculotendinous, and neurovascular dysplasias of the pre-axial border of the upper limb, and is the most common form of longitudinal deficiency. Cutis aplasia congenita involves an ulcerated area lacking in normal skin formation, present at birth. The most common site is on the scalp, but it has been described on the extremities, and overlying embryological malformations. This association, the long-term implications and the requirement for follow-up until skeletal maturity have not previously been described. We emphasise the importance of continued monitoring of these patients as the effects of radial dysplasia did not become obvious for several years, and the potential benefit from achieving early skin cover with grafting rather than allowing healing by secondary intention is discussed. It is important to increase awareness of this condition so that early orthopaedic and plastic surgical opinions can be sought, in order to reduce the disabling effect on the underlying radius.
The Proteus syndrome involves asymmetrical gigantism, verrucous epidermal naevi, vascular malformations, hamartomas and hyperostosis. The clinical features have frequently been described, but the radiological features have not been studied in detail. This paper describes the radiological features of a group of 18 patients (12 male, 6 female) that presented to the Department of Dermatology and Orthopaedic Surgery of this institution. Plain radiographs of each affected area were obtained to assist in the diagnosis and subsequent management of each patient. These radiographs were evaluated in an attempt to define the radiological anatomy of the osseous lesions. The abnormalities were classified as involving abnormal ossification, hyperostotic overgrowth or ectopic calcification. The individual features of each group will be presented. We attempted to define radiological parameters that were specific to this condition and therefore useful in diagnosis. We considered a number of radiological measurements and found a consistent alteration in bony architecture of the upper and lower limbs of affected individuals. This association was not detected in other overgrowth symptoms. This is the first objective radiological parameter that assists in the diagnosis of this rare condition.
Hip pain in cerebral palsy is regarded to be underreported. Management of these patients at home is difficult as the patients mature. In the ‘non walker’ category, the aims of surgery are to relieve pain and to allow sitting and transfer. Neuromuscular hips may have variable acetabular deficiencies ie) anterior /posterior / lateral. Many forms of surgical management, of varying complexity, have been described to address these problems. To describe a new technique with multidirectional coverage that achieves pain free hips, 15 patients were reviewed over a 4 year period. Inclusion criteria :- 1 Subluxated / dislocated hips with hip pain. 2 Patients who have failed conservative management. 3. Those not suitable for redirectional osteotomies. A standardised technique was performed by one surgeon, at one institution. In summary, the technique involves initially a standard derotation varus osteotomy. Via an anterior approach, a lateral iliac unicortical graft and strips of cancellous graft are harvested. The cancellous graft is laid on top of the intact capsule, in the areas of deficiency. The cancellous graft is held by the unicortical graft with a single screw. 15 patients were reviewed. Patients were categorised as ‘walkers’ (3) and ‘non- walkers’ (12.) The mean age was 13.2 years. All patients were pain free after recovery. This was defined as not requiring analgesia and parental satisfaction. The radiological appearances showed that all the shelves had incorporated, with satisfactory cover of the femoral head. This technique addresses multidirectional cover of femoral head. The technique is relatively easy to perform. All the patients have achieved a pain free outcome to date.