The practice of regular radiological follow-up of infants with a positive family history of developmental dysplasia of the hip is based on the widespread belief that primary acetabular dysplasia is a genetic disorder which can occur in the absence of frank subluxation or dislocation. We reviewed all infants who were involved in our screening programme for developmental dysplasia of the hip, between November 2002 and January 2004, and who had a normal clinical and ultrasound examination of the hip at six to eight weeks of age, but who, because of a family history of developmental dysplasia of the hip, had undergone further radiography after an interval of 6 to 12 months. The radiographs of 89 infants were analysed for signs of late dysplasia of the hip and assessed independently by three observers to allow for variability of measurement. There were 11 infants (11%) lost to follow-up. All the patients had normal radiographs at the final follow-up and none required any intervention. We therefore question the need for routine radiological follow-up of infants with a positive family history of developmental dysplasia of the hip, but who are normal on clinical examination and assessment by ultrasound screening when six to eight weeks old.
We reviewed 31 consecutive patients with Friedreich’s
ataxia and scoliosis. There were 24 males and seven females with
a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean
curve of 51° (13° to 140°). A total of 12 patients had thoracic
curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar.
Two patients had long thoracolumbar collapsing scoliosis with pelvic
obliquity and four had hyperkyphosis. Left-sided thoracic curves in
nine patients (45%) and increased thoracic kyphosis differentiated
these deformities from adolescent idiopathic scoliosis. There were
17 patients who underwent a posterior instrumented spinal fusion
at mean age of 13.35 years, which achieved and maintained good correction
of the deformity. Post-operative complications included one death due
to cardiorespiratory failure, one revision to address nonunion and
four patients with proximal junctional kyphosis who did not need
extension of the fusion. There were no neurological complications
and no wound infections. The rate of progression of the scoliosis
in children kept under simple observation and those treated with bracing
was less for lumbar curves during bracing and similar for thoracic
curves. The scoliosis progressed in seven of nine children initially
treated with a brace who later required surgery. Two patients presented
after skeletal maturity with balanced curves not requiring correction.
Three patients with severe deformities who would benefit from corrective
surgery had significant cardiac co-morbidities.
Patients with skeletal dysplasia are prone to
developing advanced osteoarthritis of the knee requiring total knee replacement
(TKR) at a younger age than the general population. TKR in this
unique group of patients is a technically demanding procedure owing
to the deformity, flexion contracture, generalised hypotonia and ligamentous
laxity. We retrospectively reviewed the outcome of 11 TKRs performed
in eight patients with skeletal dysplasia at our institution using
the Stanmore Modular Individualised Lower Extremity System (SMILES)
custom-made rotating-hinge TKR. There were three men and five women
with mean age of 57 years (41 to 79). Patients were followed clinically
and radiologically for a mean of seven years (3 to 11.5). The mean
Knee Society clinical and function scores improved from 24 (14 to
36) and 20 (5 to 40) pre-operatively, respectively, to 68 (28 to
80) and 50 (22 to 74), respectively, at final follow-up. Four complications
were recorded, including a patellar fracture following a fall, a
tibial peri-prosthetic fracture, persistent anterior knee pain,
and aseptic loosening of a femoral component requiring revision.
Our results demonstrate that custom primary rotating-hinge TKR in
patients with skeletal dysplasia is effective at relieving pain,
with a satisfactory range of movement and improved function. It compensates
for bony deformity and ligament deficiency and reduces the likelihood
of corrective osteotomy. Patellofemoral joint complications are
frequent and functional outcome is worse than with primary TKR in
the general population.
Guiding growth by harnessing the ability of growing bone to undergo plastic deformation is one of the oldest orthopaedic principles. Correction of deformity remains a major part of the workload for paediatric orthopaedic surgeons and recently, along with developments in limb reconstruction and computer-directed frame correction, there has been renewed interest in surgical methods of physeal manipulation or ‘guided growth’. Manipulating natural bone growth to correct a deformity is appealing, as it allows gradual correction by non- or minimally invasive methods. This paper reviews the techniques employed for guided growth in current orthopaedic practice, including the basic science and recent advances underlying mechanical physeal manipulation of both healthy and pathological physes.
The outcome in 83 patients with congenital clubfoot was evaluated at a mean age of 64 years using three validated questionnaires assessing both quality of life (short-form (SF)-36 and EQ-5D) and foot and ankle function (American Academy of Orthopaedic Surgeons (AAOS) Foot and Ankle questionnaire). In SF-36, male patients scored significantly better than male norms in seven of the eight domains, whereas female patients scored significantly worse than female norms in two of the eight. Male patients scored better than male norms in both the EQ-5D index (p = 0.027) and visual analogue scale (VAS) (p = 0.013), whereas female patients scored worse than female norms in the VAS (p <
0.001). Both male and female patients had a significantly worse outcome on the AAOS Core Scale than did norms. There was a significant correlation for both genders between the SF-36 Physical Component Summary Score and the AAOS Core Scale. The influence on activities of daily life was limited to foot and ankle problems in all patients, and in females there was an adverse effect in physical aspects of quality of life.
Compartment syndrome of the foot requires urgent surgical treatment. Currently, there is still no agreement on the number and location of the myofascial compartments of the foot. The aim of this cadaver study was to provide an anatomical basis for surgical decompression in the event of compartment syndrome. We found that there were three tough vertical fascial septae that extended from the hindfoot to the midfoot on the plantar aspect of the foot. These septae separated the posterior half of the foot into three compartments. The medial compartment containing the abductor hallucis was surrounded medially by skin and subcutaneous fat and laterally by the medial septum. The intermediate compartment, containing the flexor digitorum brevis and the quadratus plantae more deeply, was surrounded by the medial septum medially, the intermediate septum laterally and the main plantar aponeurosis on its plantar aspect. The lateral compartment containing the abductor digiti minimi was surrounded medially by the intermediate septum, laterally by the lateral septum and on its plantar aspect by the lateral band of the main plantar aponeurosis. No distinct myofascial compartments exist in the forefoot. Based on our findings, in theory, fasciotomy of the hindfoot compartments through a modified medial incision would be sufficient to decompress the foot.
Rembrandt’s etching of a beggar with a wooden leg is notable because the two lower limbs of the presumed beggar are present and not deformed. Using the facilities of four specialised Dutch art institutes, we carried out a systematic investigation to find other etchings and engravings of subjects with artificial legs supporting non-amputated limbs, from the period 1500 to 1700 AD. We discovered 28 prints produced by at least 18 artists. Several offered clues to a disorder of a knee, the lower leg or the foot. All individuals were adult males, suggesting the probability of traumatic lesions. We conclude that in this period artificial legs were not only used in the case of absence of part of a lower limb, but also for other reasons, notably disorders of the knee, lower leg or foot. They may also have been used to attract compassion.
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 pseudarthrosis in 16 of 19 cases of refracture. The risk of refracture was significantly higher when osteosynthesis was performed below the age of four years, when the tibial cross-sectional area was narrow, and when associated with persistent fibular pseudarthrosis. Refracture occurs frequently after successful osteosynthesis in these patients. Delaying osteosynthesis, maximising the tibial cross-sectional area and stabilising the fibula may reduce the risk of refracture.
We report our initial experience of using the Ponseti method for the treatment of congenital idiopathic club foot. Between November 2002 and November 2004 we treated 100 feet in 66 children by this method. The standard protocol described by Ponseti was used except that, when necessary, percutaneous tenotomy of tendo Achillis were performed under general anaesthesia in the operating theatre and not under local anaesthesia in the out-patient department. The Pirani score was used for assessment and the mean follow-up time was 18 months (6 to 30). The results were also assessed in terms of the number of casts applied, the need for tenotomy of tendo Achillis and recurrence of the deformity. Tenotomy was required in 85 of the 100 feet. There was a failure to respond to the initial regimen in four feet which then required extensive soft-tissue release. Of the 96 feet which responded to initial casting, 31 (32%) had a recurrence, 16 of which were successfully treated by repeat casting and/or tenotomy and/or transfer of the tendon of tibialis anterior. The remaining 15 required extensive soft-tissue release. Poor compliance with the foot-abduction orthoses (Denis Browne splint) was thought to be the main cause of failure in these patients.
Club foot was diagnosed by ultrasonography in 91 feet (52 fetuses) at a mean gestational age of 22.1 weeks (14 to 35.6). Outcome was obtained by chart review in 26 women or telephone interview in 26. Feet were classified as normal, positional deformity, isolated club foot or complex club foot. At initial diagnosis, 69 feet (40 fetuses) were classified as isolated club foot and 22 feet (12 fetuses) as complex club foot. The diagnosis was changed after follow-up ultrasound scan in 13 fetuses (25%), and the final ultrasound diagnosis was normal in one fetus, isolated club foot in 31 fetuses, and complex club foot in 20 fetuses. At birth, club foot was found in 79 feet in 43 infants for a positive predictive value of 83%. Accuracy of the specific diagnosis of isolated club foot or complex club foot was lower; 63% at the initial ultrasound scan and 73% at the final scan. The difference in diagnostic accuracy between isolated and complex club foot was not statistically significant. In no case was postnatal complex club foot undiagnosed on fetal ultrasound and all inaccuracies were overdiagnoses. Karyotyping was performed in 25 cases. Abnormalities were noted in three fetuses, all with complex club foot and with additional findings on ultrasound.
A percutaneous supramalleolar osteotomy with multiple drill holes and closed osteoclasis was used to correct rotational deformities of the tibia in patients with cerebral palsy. The technique is described and the results in 247 limbs (160 patients) are reported. The mean age at the time of surgery was 10.7 years (4 to 20). The radiographs were analysed for time to union, loss of correction, and angulation at the site of the osteotomy. Bone healing was obtained in all patients except one in a mean period of seven weeks (5 to 12). Malunion after loss of reduction at the site of the osteotomy developed in one tibia. Percutaneous supramalleolar osteotomy of the tibia is a safe and simple surgical procedure.
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.