The treatment of tibial aplasia is controversial. Amputation represents the gold standard with good functional results, but is frequently refused by the families. In these patients, treatment with reconstructive limb salvage can be considered. Due to the complexity of the deformity, this remains challenging and should be staged. The present study evaluated the role of femoro-pedal distraction using a circular external fixator in reconstructive treatment of tibial aplasia. The purpose of femoro-pedal distraction is to realign the limb and achieve soft tissue lengthening to allow subsequent reconstructive surgery. This was a retrospective study involving ten patients (12 limbs) with tibial aplasia, who underwent staged reconstruction. During the first operation a circular hexapod external fixator was applied and femoro-pedal distraction was undertaken over several months. Subsequent surgery included reconstruction of the knee joint and alignment of the foot.Aims
Methods
A retrospective study was performed in 18 patients
with achondroplasia, who underwent bilateral humeral lengthening
between 2001 and 2013, using monorail external fixators. The mean
age was ten years (six to 15) and the mean follow-up was 40 months
(12 to 104). The mean disabilities of the arm, shoulder and hand (DASH) score
fell from 32.3 (20 to 40)
pre-operatively to 9.4 (6 to 14) post-operatively (p = 0.037). A
mean lengthening of 60% (40% to 95%) was required to reach the goal
of independent perineal hygiene. One patient developed early consolidation,
and fractures occurred in the regenerate bone of four humeri in
three patients. There were three transient radial nerve palsies. Humeral lengthening increases the independence of people with
achondroplasia and is not just a cosmetic procedure. Cite this article:
This study compares the initial outcomes of minimally invasive techniques for single-event multi-level surgery with conventional single-event multi-level surgery. The minimally invasive techniques included derotation osteotomies using closed corticotomy and fixation with titanium elastic nails and percutaneous lengthening of muscles where possible. A prospective cohort study of two matched groups was undertaken. Ten children with diplegic cerebral palsy with a mean age of ten years six months (7.11 to 13.9) had multi-level minimally invasive surgery and were matched for ambulatory level and compared with ten children with a mean age of 11 years four months (7.9 to 14.4) who had conventional single-event multi-level surgery. Gait kinematics, the Gillette Gait Index, isometric muscle strength and gross motor function were assessed before and 12 months after operation. The minimally invasive group had significantly reduced operation time and blood loss with a significantly improved time to mobilisation. There were no complications intra-operatively or during hospitalisation in either group. There was significant improvement in gait kinematics and the Gillette Gait Index in both groups with no difference between them. There was a trend to improved muscle strength in the multi-level group. There was no significant difference in gross motor function between the groups. We consider that minimally invasive single-event multi-level surgery can be achieved safely and effectively with significant advantages over conventional techniques in children with diplegic cerebral palsy.
The results of further soft-tissue release of 79 feet in 60 children with recurrent idiopathic congenital talipes equinovarus were evaluated. The mean age of the children at the time of re-operation was 5.8 years (15 months to 14.5 years). Soft-tissue release was performed in all 79 feet and combined with distal calcaneal excision in 52 feet. The mean follow-up was 12 years (4 to 32). At the latest follow-up the result was excellent or good in 61 feet (77%) according to the Ghanem and Seringe scoring system. The results was considered as fair in 14 feet (18%), all of whom had functional problems and eight had anatomical abnormalities. Four feet (5%) were graded as poor on both functional and anatomical grounds. The results were independent of the age at which revision was undertaken.
The results of a functional, clinical and radiological study of 30 children (60 hips) with whole-body cerebral palsy were reviewed at a mean follow-up of 10.2 years (9.5 to 11). Correction of windsweep deformity of the hips was performed by bilateral simultaneous combined soft-tissue and bony surgery at a mean age of 7.7 years (3.1 to 12.2). We were able to recall 22 patients; five had died of unrelated causes and three were lost to follow-up. Evaluation involved interviews with patients/carers and clinical and radiological examination. The gross motor functional classification system was used to assess overall motor function and showed improvement in seven patients. Of the 12 patients thought to have pain pre-operatively, only one had pain post-operatively. Improved handling was reported in 18 of 22 patients (82%). Those with handling problems were attributed by the carers to growth of the patients. All patients/carers considered the procedure worthwhile. The range of hip movements improved, and the mean windsweep index improved from 50 pre-operatively to 36 at follow-up. The migration percentage and centre-edge angle were assessed on plain radiographs. Radiological containment improved, the mean migration percentage improved from 50 pre-operatively to 20 at follow-up and the mean centre-edge angle improved from −5° to 29°. No statistical difference was noted between the three-year and ten-year follow-up results, indicating that the improvements in clinical and radiological outcome had been maintained.
In five children, six forearms with a fixed pronation deformity secondary to congenital radioulnar synostosis were treated by a derotation osteotomy of the distal radius and the midshaft of the ulna. There were three boys and two girls with a mean age of 4.9 years (3.5 to 8.25) who were followed up for a mean of 29 months (18 to 43). The position of the forearm was improved from a mean pronation deformity of 68° (40° to 80°) to a pre-planned position of 10° of supination in all cases. Bony union was achieved by 6.3 weeks with no loss of correction. There was one major complication involving a distal radial osteotomy which required exploration for a possible compartment syndrome.
Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium.
