Aims. 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. Methods. 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. Results. The mean follow-up was 7.1 years (2 to 10). The mean age of the patients at the time of the application of the fixator was 2.3 years (1.1 to 5.0). The mean time under distraction was 139.7 days (81.0 to 177.0). A mean fibular distalization of 38.7 mm (14.0 to 67.0) was achieved. Pin infections occurred in four limbs (33.3%) and osteitis in one. A femoral fracture occurred in one patient. Premature removal of the frame was not required in any patient. Sufficient realignment of the leg as well as soft tissue lengthening was achieved in all patients, allowing subsequent reconstruction. All patients were able to mobilize fully weight bearing after reconstruction. Functional outcome was limited in all limbs, and five patients (50.0%) required additional reconstructive operations. Conclusion. Regarding the functional results in the treatment of tibial aplasia, amputation remains superior to limb salvage. The latter procedure should only be performed in patients whose parents refuse amputation. Femoro-pedal distraction efficiently prepares the limb by realigning the leg and soft tissue lengthening. Minor complications are frequent, but usually do not hinder the continuation of distraction. Even though a fully weight-bearing limb is achieved, the functional outcome of reconstructive treatment remains limited. Recurrent deformities frequently occur and may require further operations. Cite this article: Bone Joint J 2020;102-B(9):1248–1255

Purpose. This study compares outcomes in patients with complete congenital fibula absence, associated with severe lower limb deformity, treated with an amputation protocol to those using an extension prosthesis. Method. 32 patients were identified. 9 patients (2M: 7F, median age at presentation of 22 yrs) utilized an extension prosthesis. 23 patients (16M: 7F, median age at presentation of 10 months) underwent 25 amputations during childhood: only two underwent tibial kyphus correction to facilitate prosthetic wear. Mobility was assessed using the SIGAM and K scores. Quality of life was assessed using the PedsQL inventory questionnaire; pain by a verbal severity score. Patients undergoing amputation were further subdivided by age, below and above 2 yrs at the time of surgery. Results. 19 Syme and one Boyd amputation in 19 patients were performed early (mean age 15 months). 4 Syme and one trans-tibial amputation in 4 patients took place in older children (mean age 6.6 years). K Scores were significantly higher (mean 4 versus 2) and pain scores lower in the amputation group allowing high impact activity compared to community ambulation with an extension prosthesis. The SIGAM and PedsQL scores were all better in the amputation group, but not significantly so. There was no significant difference in the scores based on the time of amputation. Conclusion. Complete fibula absence can present with significant lower limb deformity. Parental counselling regarding management is paramount in achieving the optimum functional outcome. Childhood amputation for severe limb length inequality and foot deformity in congenital fibula absence offers excellent short term functional outcome with prosthetic support. The tibial kyphus deformity does not need routine correction and facilitates prosthetic suspension. Accommodative extension prosthesis does offer reasonable long term function but outcome scores are lower

We reviewed retrospectively 22 patients (23 limb segments) with fibular hemimelia treated by amputation or limb lengthening to evaluate these methods of treatment. There were 12 boys and 10 girls, all with associated anomalies in the lower limbs. Twelve patients (13 limb segments) had early amputation and prosthetic fitting and ten had tibial lengthening using the Ilizarov technique. At the latest follow-up, the twelve patients who had amputation were functioning well and had few complications. The ten patients who had lengthening had suffered numerous complications, and all had needed either further corrective surgery or to wear braces or shoe-raises. Two of the ten lengthened limbs required late amputation for poor function or cosmesis. There were fewer hospital admissions, clinic visits, and periods of absence from school in the amputation group. Our findings suggest that amputation is a more effective method of management than limb-lengthening in severe fibular hemimelia. The Ilizarov method is an attractive alternative for selected patients, but its exact role is not yet established. One problem is that families often have unrealistic expectations of the surgical and prosthetic technology available and may refuse amputation when this has been recommended

Aims. Extendible endoprostheses have been available for more than 30 years and have become more sophisticated with time. The latest generation is ‘non-invasive’ and can be lengthened with an external magnetic force. Early results have shown a worryingly high rate of complications such as infection. This study investigates the incidence of complications and the need for further surgery in a cohort of patients with a non-invasive growing endoprosthesis. Patients and Methods. Between 2003 and June 2014, 50 children (51 prostheses) had a non-invasive growing prosthesis implanted for a primary bone sarcoma. The minimum follow-up was 24 months for those who survived. Their mean age was 10.4 years (6 to 14). The incidence of complications and further surgery was documented. Results. The mean follow-up was 64 months (20 to 145). The overall survivorship of the patients was 84% at three years and 70% at five years. Revision-free survival was 81.7% at three years and 61.6% at five years with competing risk analysis. Deep infection occurred in 19.6% of implants at a mean of 12.5 months (0 to 55). Other complications were a failure of the lengthening mechanism in five prostheses (9.8%) and breakage of the implant in two (3.9%). Overall, there were 53 additional operations (0 to 5 per patient). A total of seven patients (14%) underwent amputation, three for local recurrence and four for infection. Their mean limb length discrepancy was 4.3 mm (0 to 25) and mean Musculoskeletal Tumor Society Score functional score was 26.5 (18 to 30) at the final follow-up. Conclusions. When compared with previously published early results, this mid-term series has shown continued good functional outcomes and compensation for leg-length discrepancy. Infection is still the most common complication: post-operative wound healing problems, central line infection and proximal tibial location are the main risk factors. Cite this article: Bone Joint J 2016;98-B:1697–1703

Purpose. We describe two recent cases of intraosseous (IO) access resulting in amputation in critically ill infants and make contemporary recommendations on the safe practice of this technique. Methods/results. This is a retrospective case series of two children transferred from peripheral hospitals to our unit. One child (5 months) was resuscitated after cardiorespiratory decompensation due to diaphragmatic hernia. The other child (18 months) deteriorated during presentation with purpura fulminans two days after a scald injury. Both children underwent emergent bilateral tibial IO access using power driven needle systems. Initial resuscitation included large volumes of 0.9% normal saline. One child received doses of adrenaline and 8.4% sodium bicarbonate, whole blood and an initial dopamine infusion; the other received 8.4% sodium bicarbonate, 10% calcium gluconate and whole blood. Total infused volumes were 400mL and 730 ml respectively. Progressive lower limb tension became evident in both children after 12 hours; bilateral posterior tibial fractures were noted in one child. Ultimately, both children underwent surgical fasciotomy, but extensive necrosis necessitated unilateral below knee amputation at days 12 and 13 respectively. Conclusions. Fluid extravasation has been implicated in complicating IO access, particularly with high volume or prolonged infusions. Other potentiating factors include inaccurate needle placement, needle dislodgement during transfer and repeated infusion of irritant solutions. All of these contributed to irreversible lower limb ischaemia in these two cases. Adherence to the principles of careful needle placement, splinting, limited length of infusion and repeated monitoring of the limb will help avoid this devastating complication

Peripheral limb ischaemia is rare in children. We have treated only 12 infants and children with this condition in the past 15 years at the Royal Hospital for Sick Children in Glasgow. There were nine neonates and three older children. Most were suffering from life-threatening illnesses or severe infection. Two were born with ischaemic arms with no apparent cause. We have analysed the factors leading to ischaemia, the outcome of the initial treatment and the later orthopaedic problems. Two required amputation of both legs, one of an arm, two of feet and one of toes. Two had skin grafts. All surgery was performed after demarcation was well established and delayed closure was used after amputation. Five children developed limb-length discrepancy or an angular deformity. To date two have required additional corrective surgery

Septicaemia resulting from meningococcal infection is a devastating illness affecting children. Those who survive can develop late orthopaedic sequelae from growth plate arrests, with resultant complex deformities. Our aim in this study was to review the case histories of a series of patients with late orthopaedic sequelae, all treated by the senior author (CFB). We also describe a treatment strategy to address the multiple deformities that may occur in these patients. Between 1997 and 2009, ten patients (seven girls and three boys) were treated for late orthopaedic sequelae following meningococcal septicaemia. All had involvement of the lower limbs, and one also had involvement of the upper limbs. Each patient had a median of three operations (one to nine). Methods of treatment included a combination of angular deformity correction, limb lengthening and epiphysiodesis. All patients were skeletally mature at the final follow-up. One patient with bilateral below-knee amputations had satisfactory correction of her right amputation stump deformity, and has complete ablation of both her proximal tibial growth plates. In eight patients length discrepancy in the lower limb was corrected to within 1 cm, with normalisation of the mechanical axis of the lower limb. Meningococcal septicaemia can lead to late orthopaedic sequelae due to growth plate arrests. Central growth plate arrests lead to limb-length discrepancy and the need for lengthening procedures, and peripheral growth plate arrests lead to angular deformities requiring corrective osteotomies and ablation of the damaged physis. In addition, limb amputations may be necessary and there may be altered growth of the stump requiring further surgery. Long-term follow-up of these patients is essential to recognise and treat any recurrence of deformity

Purpose of the study. We describe a new technique of talar dome osteotomy in the treatment of fixed equinovalgus deformity of the foot in patients with Fibular Hemimelia and successfully applied it in two patients. Background. Fibular Hemimelia is a congenital absence or hypoplasia of fibula with associated fixed equinovalgus deformity of the foot. Treatment for this deformity ranges from corrective osteotomy of the tibia, calcaneum to Syme's amputation. Methods. The procedure of talar dome osteotomy is best applied to children before they start to walk. Through a Cincinnati approach, fibular anlage was excised and a talar dome osteotomy performed in the axial plane to correct the valgus deformity of the ankle. Additional procedures if required include corrective osteotomy of the distal tibia to correct remaining foot deformity after the initial correction, and tendo achillis lengthening. The corrected position is then maintained with a K-wire inserted through the calcaneum, osteotamised talus up into the distal tibia. K-wire was removed at 6 weeks and foot position thereafter maintained in an AFO orthrosis with the foot slightly inverted for next 2 years. Two patients diagnosed with fibular hemimelia (Coventry and Johnson type II) underwent correction of their fixed equino-valgus deformity with the above mentioned technique at the ages of 6 and 10 months respectively. AFO orthosis was used for two years and at 5 years of follow-up the deformity has remained corrected in both the ankles. Both these patients are due to undergo limb-lengthening procedures

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 pseudarthrosis of 15.2 months. 9 of 19 had an established diagnosis of Neurofibromatosis. 8 had a frank pseudarthrosis at presentation, 2 of which were present at birth. There were 3 re-fractures following treatment. One of the 19 required amputation after failed surgical treatment. Each patient had an average of 2.4 surgical procedures. 14 patients had either fixed or elongating rods, 10 had Ilizarov treatment. 2 have outstanding surgery planned. CONCLUSIONS. A philosophy of treatment has evolved which produces acceptable results. Splintage is used for antero-lateral bowing with corrective osteotomies and rodding, without extensive bone or periosteal resection, for significant or progressive deformity or fracture. Ilizarov treatment is used for recalcitrant cases over the age of 6 years. The technique has evolved over time to include bone graft wraps and the use of Bone Morphogenic protein in combination with the Ilizarov technique. Patients have below population and age matched average scores for both the ASK and OAFQ

We report the results of the treatment of 23 patients with macrodactyly. Eighteen had a two-stage bulk-reducing (defatting) procedure; phalangectomy was used to shorten the digits. At a mean follow-up of nine years (2 to 12), two patients had been lost to follow-up, and three await a second-stage procedure. Good cosmetic correction was achieved in 12 patients, with satisfactory results in seven; two patients had poor results and required amputation

Until recently the accepted treatment of choice for severe type-II fibular hemimelia has been Syme’s or Boyd’s amputation. The alternative of distraction lengthening using the Ilizarov technique is now available. We report three patients (four limbs) with type-II fibular hemimelia who were treated by the Ilizarov technique and followed up for two to six years. Severe progressive procurvatum and valgus deformity of the tibia and valgus deformity and lateral subluxation of the ankle were found in all four limbs. Multiple additional soft-tissue and bony surgery was necessary. In view of these problems we feel that reappraisal of the indications for lengthening in type-II fibular hemimelia is necessary

Aims

Following the introduction of national standards in 2009, most major paediatric trauma is now triaged to specialist units offering combined orthopaedic and plastic surgical expertise. We investigated the management of open tibia fractures at a paediatric trauma centre, primarily reporting the risk of infection and rate of union.

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: Bone Joint J 2013;95-B:1027–34.

Children with congenital vertical talus (CVT) have been treated with extensive soft-tissue releases, with a high rate of complications. Recently, reverse Ponseti-type casting followed by percutaneous reduction and fixation has been described, with excellent results in separate cohorts of children with CVT, of either idiopathic or teratological aetiology. There are currently no studies that compare the outcome in these two types. We present a prospective cohort of 13 children (21 feet) with CVT of both idiopathic and teratological aetiology, in which this technique has been used. Clinical, radiological and parent-reported outcomes were obtained at a mean follow-up of 36 months (8 to 57). Six children (nine feet) had associated neuromuscular conditions or syndromes; the condition was idiopathic in seven children (12 feet).

Initial correction was achieved in all children, with significant improvement in all radiological parameters. Recurrence was seen in ten feet. Modification of the technique to include limited capsulotomy at the initial operation may reduce the risk of recurrence.

The reverse Ponseti-type technique is effective in the initial correction of CVT of both idiopathic and teratological aetiology. Recurrence is a problem in both these groups, with higher rates than first reported in the original paper. However, these rates are less than those reported after open surgical release.

Cite this article: Bone Joint J 2014;96-B:274–8.

Between 1990 and 2001, 24 children aged between 15 months and 11 years presented with late orthopaedic sequelae after meningococcal septicaemia. The median time to presentation was 32 months (12 to 119) after the acute phase of the disease. The reasons for referral included angular deformity, limb-length discrepancy, joint contracture and problems with prosthetic fitting. Angular deformity with or without limb-length discrepancy was the most common presentation. Partial growth arrest was the cause of the angular deformity. Multiple growth-plate involvement occurred in 14 children. The lower limbs were affected much more often than the upper. Twenty-three children underwent operations for realignment of the mechanical axis and limb-length equalisation. In 15 patients with angular deformity around the knee the deformity recurred. As a result we recommend performing a realignment procedure with epiphysiodesis of the remaining growth plate when correcting angular deformities.

Type BI rotationplasty is currently indicated for children with tumours of the proximal femur whereas type BIIIa rotationplasty is reserved for those in which the entire femur has to be removed. Our aim was to compare these two types of rotationplasty and determine whether the knee should be preserved in children with tumours of the proximal femur. We compared the post-operative complications, oncological outcome, range of movement, Enneking score and radiographs of six children, who had undergone type BI rotationplasty with those of 12 who had undergone type BIIIa rotationplasty.

Patients with type BI rotationplasty had a mean Enneking score of 21.6 compared with 24.4 in those with type BIIIa rotationplasty, and worse mean results in all of the parameters investigated. We conclude that type BI rotationplasty has a worse functional outcome and more complications than type BIIIa rotationplasty in children under the age of ten years.

We present a retrospective review of 167 patients aged 18 years and under who were treated for chronic haematogenous osteomyelitis at our elective orthopaedic hospital in Malawi over a period of four years. The median age at presentation was eight years (1 to 18). There were 239 hospital admissions for treatment during the period of the study. In 117 patients one admission was necessary, in 35 two, and in 15 more than two.

A surgical strategy of infection control followed by reconstruction and stabilisation was employed, based on the Beit CURE radiological classification of chronic haematogenous osteomyelitis as a guide to treatment. At a minimum follow-up of one year after the end of the study none of the patients had returned to our hospital with recurrent infection.

A total of 350 operations were performed on the 167 patients. This represented 6.7% of all children’s operations performed in our hospital during this period. One operation only was required in 110 patients and none required more than three. Below-knee amputation was performed in two patients with chronic calcaneal osteomyelitis as the best surgical option for function. The most common organism cultured from operative specimens was Staphylococcus aureus, and the tibia was the bone most commonly affected. Polyostotic osteomyelitis occurred in four patients. We believe this is the largest reported series of patients treated for chronic haematogenous osteomyelitis.

We present two children with massive defects of the tibia and an associated active infection who were treated by medial transport of the fibula using the Ilizarov device. The first child had chronic discharging osteomyelitis which affected the whole tibial shaft. The second had sustained bilateral grade-IIIB open tibial fractures in a motor-car accident. The first child was followed up for three years and the second for two years. Both achieved solid union between the proximal and distal stumps of the tibia and the fibula, with hypertrophy of the fibula. The first child had a normal range of movement at the knee, ankle and foot but there was shortening of 1.5 cm. The second had persistent anterior angulation at the proximal tibiofibular junction and the ankle was stiff in equinus.

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.