Stüve-Wiedemann syndrome is an autosomal-recessive disorder characterised by bowing of the long bones, progressive scoliosis, episodic hyperthermia and respiratory distress, usually resulting in death in infancy. We reviewed five children with the condition who had been followed since birth and who survived into childhood with a mean age at operation of 7.8 years (5 to 14). There was marked functional impairment with dysplasia of the long bones and scoliosis. Treatment of the triplanar deformities of the femora involved the use of the Ilizarov technique with the Taylor Spatial Frame. Walking was preserved and improved in three children along with considerable enhancement of the appearance. Early insertion of a growing rod to control the progressive juvenile scoliosis was beneficial. The use of the Taylor Spatial Frame is strongly recommended to address the major complex deformities of the lower limbs which are encountered in this condition and to prevent their progression.
We treated 35 brachymetatarsal rays of 18 feet in 12 patients by one-stage lengthening with interpositional bone grafts or by gradual lengthening with callotasis combined with shortening of the adjacent metatarsals and phalanges. Definition of the two parabolas which connect the metatarsal heads and the tips of the toes, and recognition of three patterns of metatarsal length, were helpful guides in treatment. In total, 36 excisions of the phalanges and/or the metatarsals were undertaken. The mean shortening was 8 mm. The radiological results were satisfactory. The mean values were as follows: one-stage lengthening, length gain, 1.3 cm; healing index, 1.3 months/cm; percentage increase, 30%; gradual lengthening, length gain, 2.0 cm; healing index, 2.0 months/cm; percentage increase, 50%. Associated shortening of an adjacent bone can avoid the disadvantages of one-stage lengthening which may not achieve target length and can result in neurovascular complications. Reduction of the target length enables the surgeon to carry out one-stage instead of gradual lengthening. It also shortens the length of treatment in the group undergoing callotasis and improves cosmesis.
Elastofibroma dorsi is an uncommon, benign, slow-growing soft-tissue tumour of uncertain aetiology. It classically presents as an ill-defined mass at the inferior pole of the scapula with symptoms which include swelling, discomfort, snapping, stiffness and occasionally pain. We report the symptoms, function and outcome after treatment of 21 elastofibromas in 15 patients. All were diagnosed by MRI and early in the series four also underwent CT-guided biopsy to confirm the diagnosis. In all, 18 tumours were excised and three were observed. After excision, the mean visual analogue score for pain decreased from 4.6 (0 to 10) pre-operatively to 2.4 (0 to 8) post-operatively (p = 0.04). The mean shoulder function, at a mean follow-up of 4.2 years (3 months to 16 years), was 78.1% (30 to 100) using the Stanmore percentage of normal shoulder assessment scoring system. The mean range of forward flexion improved from 135° (70° to 180°) to 166° (100° to 180°) after excision (p = 0.005). In four patients a post-operative haematoma formed; one required evacuation. Three patients developed a post-operative seroma requiring needle aspiration and one developed a superficial infection which was treated with antibiotics. Our findings support previous reports suggesting that a pre-operative tissue diagnosis is not necessary in most cases since the lesion can be confidently diagnosed by MRI, when interpreted in the light of appropriate clinical findings. Surgical excision in symptomatic patients, is helpful. It has been suggested that elastofibroma is caused by a local tissue reaction and is not a true neoplastic process. A strong association has been noted between elastofibroma and repetitive use of the shoulder, which is supported by our findings.
We evaluated the safety and efficacy of total
knee replacement in patients receiving continuous warfarin therapy. We identified 24 consecutive patients receiving long-term warfarin
therapy who underwent total knee replacement between 2006 and 2008
and compared them with a group of age- and gender-matched patients
not on long-term anticoagulation. Primary observations were changes
in haemoglobin, transfusion rates and complications. Secondary observations
were fluctuations in the international normalised ratio (INR) and
post-operative range of movement. There was no significant difference between the two groups in
pre- or post-operative haemoglobin, incidence of transfusion or
incidence of post-operative complications. There were no surgical
delays due to a high INR level. The mean change in INR during the
peri-operative phase was minimal (mean 0.4; Current American College of Chest Physicians guidelines recommend
bridging therapy for high-risk patients receiving oral anticoagulation
and undergoing major orthopaedic procedures. We have shown that
a safe alternative is to continue the steady-state warfarin peri-operatively
in patients on long-term anticoagulation requiring total knee replacement.
Incomplete intertrochanteric fractures do not extend across to the medial femoral cortex and are stable, without rotational deformity or shortening of the lower limb. The aim of our study was to establish whether they can be successfully managed conservatively. A total of 68 patients over a five-year period presented with a suspected fracture of the femoral neck and underwent an MRI scan for further assessment. From these, we retrospectively reviewed eight patients with normal plain radiographs but with an incomplete, intertrochanteric fracture on MRI scan. Five were managed conservatively and three operatively. The mean length of hospital stay was 16 days for the conservatively-treated group and 15 days for those who underwent surgery; this was not statistically significant (p >
0.5) and all patients were mobilised on discharge. Although five patients were readmitted at a mean of 3.2 years after discharge, none had progressed to a complete fracture. We believe that patients with incomplete intertrochanteric fractures should be considered for conservative treatment.
It may not be possible to undertake revision total hip arthroplasty
(THA) in the presence of massive loss of acetabular bone stock using
standard cementless hemispherical acetabular components and metal
augments, as satisfactory stability cannot always be achieved. We
aimed to study the outcome using a reconstruction cage and a porous
metal augment in these patients. A total of 22 acetabular revisions in 19 patients were performed
using a combination of a reconstruction cage and porous metal augments.
The augments were used in place of structural allografts. The mean
age of the patients at the time of surgery was 70 years (27 to 85)
and the mean follow-up was 39 months (27 to 58). The mean number
of previous THAs was 1.9 (1 to 3). All patients had segmental defects
involving more than 50% of the acetabulum and seven hips had an
associated pelvic discontinuity. Aims
Patients and Methods