Limb lengthening and correction of deformity in the lower limbs of children with osteogenesis imperfecta

We performed limb lengthening and correction of deformity of nine long bones of the lower limb in six children (mean age, 14.7 years) with osteogenesis imperfecta (OI). All had femoral lengthening and three also had ipsilateral tibial lengthening. Angular deformities were corrected simultaneously. Five limb segments were treated using a monolateral external fixator and four with the Ilizarov frame. In three children, lengthening was done over previously inserted femoral intramedullary rods. The mean lengthening achieved was 6.26 cm (mean healing index, 33.25 days/cm). Significant complications included one deep infection, one fracture of the femur and one anterior angulation deformity of the tibia. The abnormal bone of OI tolerated the external fixators throughout the period of lengthening without any episodes of migration of wires or pins through the soft bone. The regenerate bone formed within the time which is normally expected in limb-lengthening procedures performed for other conditions. We conclude that despite the abnormal bone characteristics, distraction osteogenesis to correct limb-length discrepancy and angular deformity can be performed safely in children with OI.     

Humeral lengthening in hypoplasia of the upper limb

Surgical treatment for the correction of upper limb discrepancies with primary involvement of the humerus is reported. The operative technique, the lengthening procedure with the G3-IOR distractor, and the successive external fixation are described. In the seven extremities treated, the mean lengthening was 5.0 cm, the mean percentage lengthening was 25.2%, and the mean residual discrepancy was 2.9 cm. The authors believe that humeral lengthening is advisable for 13- to 15-year-old patients with hypoplasia of the humerus when the discrepancy is greater than 3 cm.     

Lower limb deformities in children: two-stage correction using the Taylor spatial frame

The Taylor spatial frame is a new external ring fixation system developed for correction of axial, sagittal, planar and rotational deformities of the extremities. Between August 1999 and October 2000 five children were treated by this system. Four had shortened and deformed femurs after fracture, osteomyelitis or a congenital short femur. One patient had a pseudoarthrosis of the tibia. Mean corrections were leg lengthening 5.9 cm (1.7-7.2), valgus correction 10.6 degrees (0-19), lateral translation 12.6 mm (0-33) and rotation 10 degrees. The mean healing index was 48.4 days/cm. In all patients the bones fused without major complications. We recommend the Taylor spatial frame for corrections of complex limb deformities.     

Use of double-row monolateral Trauma-Fix unibar lengthener for femur lengthening in patients with leg length discrepancy

We report the case of a patient in whom the use of monolateral double-row Trauma-Fix lengthener was successful for femur lengthening after initial difficulty in lengthening by using the single-row lengthener.Problem and case reportA girl 12 years of age presented with a leg-length discrepancy (LLD) of 8 cm and left genu valgum deformity due to a trauma-induced left distal femur physial lesion during her infancy. The patient's weight and height were 38 kg and 148 cm, respectively, and the mechanical axis was 16 degrees of valgus at the time of operation. The lengthening procedure was initiated on postoperative day 7 after the initial correction of the genu valgum deformity. However, no distraction was observed at the osteotomy site, and a convergent angulation deformity developed at the pin-clamp joint.Method and outcomeThis problem was resolved successfully with the addition of another row of the Trauma-Fix lengthening device, which was linked via the previously applied pins. A final lengthening of 7.5 cm was achieved in 3 months by using the double-row lengthener without pin tract infection or breakage. The monolateral frame of the Trauma-Fix lengthener was removed in the 11th postoperative month after a solid union of the femur was achieved. The postoperative mechanical axis of the knee was 4 degrees varus. The monolateral double-row lengthener device that showed high efficiency and improved the strength of the ball joints were used along the single-row lengthener, and it may serve as a good alternative for augmentation of insufficient ball joints during limb lengthening.     

Multi-axial correction system in the treatment of radial club hand

Background

Radial club hand is a well-recognized congenital malformation characterized by hypoplasia of bone and soft tissue on the radial aspect of the forearm and hand. The modalities of treatment have traditionally varied from stretching casts with soft-tissue procedures to the use of multiple corrective osteotomies. These osteotomies can be stabilized by a variety of methods, including external fixators that allow the possibility of gradual distraction with neohistiogenesis. This current study outlines the usage of one such device (multi-axial correction system [MAC]) in the management of deformity associated with severe radial club hand.

Methods

Three consecutive cases of unilateral or bilateral severe (Bayne type IV) congenital radial club hand were corrected using MAC fixation in the last 5 years. This is a retrospective review of all three cases. Data parameters included: patient demographics, presentation findings, degree of deformity, amount of correction/lengthening, length of procedure, length of treatment, and associated complications. The surgical technique is described in detail for the benefit of the readership.

Results

The three patients with severe congenital radial club hand had a total of four limb involvements that underwent correction using osteotomies and usage of the MAC device for external fixation. All three patients underwent successful correction of deformity with the restoration of alignment, lengthening of forearm for improvement of function, and stabilization of the wrist (mean duration, mean lengthening, mean time to consolidation). The MAC system was well tolerated in all patients and associated complications were limited.

Conclusion

The MAC fixator seems to be a good alternative modality of stabilization and correction for severe congenital radial club hand deformities. Its usage is fairly simple and it provides the ease of application of a mono-lateral fixator with far superior three-dimensional control, like the circular external fixator. We recommend that clinicians should add this modality to their armamentarium for the deformity correction of severe radial club hand and others in general.     

The management of leg-length discrepancy in Ollier's disease with a fully implantable lengthening nail

Ollier's disease is characterised by severe deformity of the extremities and retarded growth because of multiple enchondromas. For correction of deformity, the Ilizarov method has been used although it has many complications. A 17-year-old boy with Ollier's disease had a limb-length discrepancy of 17.4 cm, with a valgus deformity of the right knee and recurvatum of the femur of 23 degrees . He had undergone three unsuccessful attempts to correct the deformities by using external fixators. We used a fully implantable, motorised, lengthening and correction nail (Fitbone) to achieve full correction of all the deformities without complications. We decided to carry out the procedure in three stages. First, we lengthened the femur by 3.6 cm and the tibia by 4 cm. We then exchanged the femoral nail for a longer implant and achieved a further 6 cm of length. This reduced the shortening to 3.8 cm. When the boy has finished secondary school we will adjust the remaining discrepancy.     

Transverse debridement and acute shortening followed by distraction histogenesis in the treatment of open tibial fractures with bone and soft tissue loss

This retrospective case series evaluates the technique of transverse debridement, acute shortening and subsequent distraction histogenesis in the management of open tibial fractures with bone and soft tissue loss, thereby avoiding the need for a soft tissue flap to cover the wound. Thirty-one patients with Gustilo grade III open tibial fractures between 2001 and 2011 were initially managed with transverse wound extensions, debridement and shortening to provide bony apposition and allowing primary wound closure without tension, or coverage with mobilization of soft tissue and split skin graft. Temporary monolateral external fixation was used to allow soft tissues resuscitation, followed by Ilizarov frame for definitive fracture stabilization. Leg length discrepancy was corrected by corticotomy and distraction histogenesis. Union was evaluated radiologically and clinically. Patients’ mean age was 37.3 years (18.3–59.3). Mean bone defect was 3.2 cm (1–8 cm). Mean time to union was 40.1 weeks (12.6–80.7 weeks), and median frame index was 75 days/cm. Median lengthening index (time in frame after corticotomy for lengthening) was 63 days/cm. Mean clinic follow-up was 79 weeks (23–174). Six patients had a total of seven complications. Four patients re-fractured after frame removal, one of whom required a second frame. Two patients required a second frame for correction of residual deformity, and one patient developed a stiff non-union which united following a second frame. There were no cases of deep infection. Acute shortening followed by distraction histogenesis is a safe method for the acute treatment of open tibial fractures with bone and soft tissue loss. This method also avoids the cost, logistical issues and morbidity associated with the use of local or free-tissue transfer flaps and has a low rate of serious complications despite the injury severity.     

Humeral lengthening and deformity correction in Ollier's disease: distraction osteogenesis with a multiaxial correction frame

A case of Ollier's disease with deformity and shortening of the humerus is presented. Lengthening of 9 cm and deformity correction of 50 degrees were accomplished with excellent functional and cosmetic results. Unique features of this case were the use of a multiaxial correction monolateral frame and the formation of normal bone within the region of diseased Ollier's bone.     

Limb lengthening and deformity correction with externally controlled motorized intramedullary nails: evaluation of 50 consecutive lengthenings

Background and purpose — Limb lengthening with an intramedullary motorized nail is a relatively new method. We investigated if lengthening nails are reliable constructs for limb lengthening and deformity correction in the femur and the tibia.

Patients and methods — 50 lengthenings (34 Precice and 16 Fitbone devices) in 47 patients (mean age 23 years [11–61]) with ≥12 months follow-up are included in this study. 30 lengthenings were done due to congenital and 20 because of posttraumatic deformity (21 antegrade femora, 23 retrograde femora, 6 tibiae). Initial deformities included a mean shortening of 42?mm (25–90). In 15 patients, simultaneous axial correction was done using the retrograde nailing technique.

Results — The planned amount of lengthening was achieved in all but 2 patients. 5 patients who underwent simultaneous axial correction showed minor residual deformity; unintentionally induced minor deformities were found in the frontal and sagittal plane. The consolidation index was 1.2 months/cm (0.6–2.5) in the femur and 2.5 months/cm (1.6–4.0) in the tibia. 2 femoral fractures occurred in retrograde femoral lengthenings after consolidation due to substantial trauma. There were 8 complications, all of which were correctable by surgery, with no permanent sequelae.

Interpretation — Controlled acute axial correction of angular deformities and limb lengthening can be achieved by a motorized intramedullary nail. A thorough preoperative planning and intraoperative control of alignment are required to avoid residual and unintentionally induced deformity. In the femur relatively fast consolidation could be observed, whereas healing was slower in the tibia.     

Lengthening of the femur over an existing intramedullary nail

Leg length discrepancies can occur despite successful union of femur fractures after intramedullary nailing (IMN). Often, the leg length discrepancy can result in significant disability to the patient, altered gait biomechanics, pelvic obliquity, and pain. Therefore, a successful clinical result for such deformities after IMN involves addressing the leg length inequality. Femoral reconstruction with an osteotomy around an existing intramedullary nail was introduced to address axial deformity correction and limb lengthening without changing or removing a previously inserted IMN. This technique uses the principles of lengthening over an IMN. The presence of the nail has minimized the time needed for the external fixator because the nail supports the regenerate bone or osteotomy during the consolidation phase. With this technique, surgery is minimized by avoiding the need for exchange nailing.     

Correction of post-traumatic lower limb deformities using the Taylor spatial frame

Twenty-five post-traumatic leg length discrepancies combined with axial deviation in 22 patients were treated with the Taylor spatial frame. We retrospectively analysed the accuracy of correction, the complication rate and the clinical outcome including the duration of treatment. The cases were divided into three sub-groups according to the level of the osteotomy. The mean age at operation was 22.7 years; the mean follow-up was 21.1 months. The patients presented uniplanar and multiplanar deformities in combination with leg length discrepancy. Twenty-one of 25 cases showed a frontal plane malalignment preoperatively. Only three of those 21 cases continued to show minimal malalignment postoperatively. The preoperative mechanical axis deviation present in 15 of 17 lower extremities was fully corrected in 13 extremities. The 25 lengthening and correction procedures were associated with a total of 61 difficulties, including 44 problems, seven obstacles and ten complications. In conclusion, the Taylor spatial frame allows accurate results in correction of complex post-traumatic deformities with minimal morbidity.     

Correction of distal femoral deformity

This retrospective study reviews 12 distal femoral osteotomies in nine patients performed for angular and rotational deformities, using the Orthofix external fixator. All osteotomies progressed to solid union with reliable correction of the deformity. Few complications were encountered. Simultaneous lengthening was performed on one patient successfully. This method proved to be a safe and precise means of correcting distal femoral deformity with the option of simultaneous correction of length discrepancy.     

Functional outcome after lengthening with and without deformity correction in polio patients

Poliomyelitis is one of the causes of limb length discrepancy. The aim of lengthening and deformity correction in such patients is to improve the functional mobility of the patient. This study aims to find out whether or not improvement of limb length inequality with or without deformity correction affects or improves ambulation. This prospective study included 32 skeletally mature patients managed using the Ilizarov technique and external fixation for limb lengthening with or without deformity correction. Functional Mobility Scale scoring was used for assessment of ambulation before lengthening and at the final follow-up. The average duration of follow-up was 2 years and 9 months. Lengthening alone did not change the Functional Mobility Scale score. While lengthening associated with deformity correction improved the mobility scale at 5 m only (in the house), it had no effect on the 50 and 500 m score.     

Indikationsspektrum intramedullärer oder fixateurgebundener Achskorrekturen und Extremitätenverlängerungen

Corrective osteotomy is a well established operative option for correction of predominantly congenital skeletal deformities on the growing skeleton. The indications for the use of a suitable corrective instrument result partly from the location, severity of the deformity and the presence of concomitant leg length discrepancies. External fixators are predominantly used for correction of combined deformities. For isolated leg length differences limb lengthening nails can be implanted but only after growth plates have become closed; however, only a few combined deformities can be addressed in this way. Often several surgical steps are necessary. A careful treatment plan can only be created if the analysis of the deformity is carried out with appropriate parameters and a correct prognosis of the further development. The aim of this paper is to describe the indications for the corrective instruments, the planning of corrections and the presentation of typical indications for axis correction and limb lengthening.     

Acute correction of lower limb deformity and simultaneous lengthening with a monolateral fixator

We have reviewed, retrospectively, all children with a lower limb deformity who underwent an acute correction and lengthening with a monolateral fixator between 1987 and 1996. The patients were all under the age of 19 years and had a minimum follow-up of eight months after removal of the fixator. A total of 41 children had 57 corrections and lengthening. Their mean age was 11.3 years (3.2 to 18.7) and there were 23 girls and 18 boys. The mean maximum correction in any one plane was 23 degrees (7 to 45). In 41 bony segments (either femur or tibia) a uniplanar correction was made while various combinations were carried out in 16. The site of the osteotomy was predominantly diaphyseal, at a mean of 47% (17% to 73%) of the total bone length and the mean length gained was 6.4 cm (1.0 to 17.0). Univariate analysis identified a moderately strong relationship between the bone healing index (BHI), length gained, maximum correction and grade-II to grade-III complications. For logistic regression analysis the patients were binary coded into two groups; those with a good outcome (BHI < or = 45 days/cm) and those with a poor outcome (BHI > 45 days/cm). Various factors which may influence the outcome were then analysed by calculating odds ratios with 95% confidence intervals. This analysis suggested a dose response between increasing angular correction and poor BHI which only reached statistical significance for corrections of larger magnitude. Longer lengthenings were associated with a better BHI while age and the actual bone lengthened had little effect. Those patients with a maximum angulatory correction of less than 30 degrees in any one plane had an acceptable consolidation time with few major complications. The technique is suitable for femoral deformity and shortening, but should be used with care in the tibia since the risk of a compartment syndrome or neurapraxia is much greater.     

Comparative clinical study on deformity correction accuracy of different external fixators

Purpose

In this study, the correction accuracy of Smart Correction spatial fixators and of Ilizarov-type external fixators are compared in terms of deformity complexity.

Methods

Seventy-seven (40 male, 37 female) bone segments of 57 patients treated with a Smart Correction device were compared with 94 (51 male, 43 female) segments of 68 patients treated with an Ilizarov fixator. Mean age of the Smart Correction group was 20.69?±?12.94 years, and or the Ilizarov group 22.45?±?12.18 years. Patients were categorised according to limb lengthening and the number of deformity planes.

Results

A longer correction period was found with Ilizarov (66.53?±?47.7 days) compared with Smart Correction (49.05?±?35.6 days) devices. The bone healing index of the Ilizarov group was significantly better compared with the spatial group. Residual deformity after treatment was significantly lower with the Smart Correction device; however, this relationship could not be shown between subgroups. Although there was no significant difference between subgroups, mean residual deformity was higher with the increasing number of planes of the deformity.

Conclusions

The Smart Correction fixator is an accurate device that allows ease of application and planning. It demonstrates higher accuracy for correcting deformities compared with an Ilizarov external fixator. With an increasing number of planes, the difference between the two devices becomes even more pronounced. The relationship between the complexity of the deformity and residual deformity may possibly be significantly greater in favour of the Smart Correction fixator in a study with a larger sample size.     

Deformity correction planning for hindfoot, ankle, and lower limb

Many patients with foot and ankle deformities have concurrent deformities (osseous and soft tissue), with or without limb length discrepancies. Lower extremity deformities and limb length discrepancies typically result from trauma, congenital abnormality, avascular necrosis, previous surgery, nonunion, and malunion. Limb deformity correction requires extensive surgical experience because many considerations and factors apply to realignment. The considerations and factors regarding realignment are highlighted throughout this article.     

Deformity correction and lengthening of lower legs with an external fixator

Deformities combined with shortening in 34 lower limb segments of 28 patients were treated with an Ilizarov external fixator or a Taylor spatial frame at the same level as the osteotomy. We compared an acute correction group (A) with a gradual correction group (G) in patients undergoing deformity correction followed by lengthening. We retrospectively examined the amount of deformity correction, length gained, distraction index, maturation index, and external fixation index in both groups. The mean age of subjects was 12.9 years for A, 17.9 years for G. The mean deformity correction was 17.8° for A, 25.1° for G. Mean lengthening was 5.5 cm for A, 5.0 cm for G. Mean distraction index was 16.4 days/cm for A, 10.6 days/cm for G (P<0.05). Mean maturation index was 40.5 days/cm for A, 29.5 days/cm for G (P=0.081). Mean external fixation index was 58.6 days/cm for A, 42.5 days/cm for G (P<0.05). The distraction index and external fixation index differences between the two groups were statistically significant. Gradual correction may represent a better approach than acute correction with the use of external fixators to treat deformity combined with shortening.

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Résumé Des déformations associées au raccourcissement de 34 membres inférieurs chez 28 patients étaient traités, au niveau de l’ostéotomie, avec un fixateur externe d’Ilizarof ou un cadre de Taylor. Nous avons comparés un groupe avec une correction instantanée (A) et un autre avec une correction progressive (G) de la déformation suivie de l’allongement. Nous avons mesuré rétrospectivement l’importance de la correction, le gain de longueur, les index de distraction, de maturation et de fixation externe dans les deux groupes. L’age moyen des patients était de 12,9 ans pour le groupe A et de 17,9 ans pour le groupe G.. La correction moyenne de déformation était de 17,8° pour le groupe A et de 25,1° pour le G.. L’allongement moyen était de 5,5 cm pour le A et de 5,0 cm pour le G.. L’index de distraction était de 16,4 jours/cm pour le A et de 10,6 jours/cm pour le G. L’index de maturation était de 40,5 jours/cm pour le A et de 29,5 jours/cm pour le G. L’index de fixation externe était de 58,6 jours/cm pour le A et de 42,5 jours /cm pour le G.. La différence entre les index de distraction et de fixation externe étaient significative. La correction progressive est préférable à la correction instantanée pour traiter des déformations combinées au raccourcissement.

     

Tibial lengthening and concomitant foot deformity correction in 14 patients with permanent deformity after poliomyelitis

BACKGROUND: In neuromuscular diseases, limb lengthening and foot deformity correction are associated with a high risk of complications associated with distraction callus and joint contracture. We have found no published articles of tibial lengthening and concomitant foot deformity correction using the Ilizarov method or traditional methods. To compare result of gradual distraction with triple arthrodesis for foot deformity combined with tibial lengthening, we investigated healing index and complications of two methods. PATIENTS AND METHODS: We reviewed 14 patients with permanent deformity after poliomyelitis who underwent tibial lengthening and concomitant foot deformity correction using the Ilizarov external fixator. Tibial lengthening over an intramedullary nail was performed in 3 patients and lengthening without a nail was performed in 11 patients. RESULTS: The mean external fixation time was 6 (3.6-10) months without nail and 1.6 (1.5-1.7) months with nail, whereas the mean healing index was 1.8 (0.8-3.1) months/cm without nail and 2 (1.8-2.3) months/cm with nail. Concomitant foot treatments included triple arthrodesis in 7 patients, pantalar arthrodesis in 2 patients with flail ankle, and gradual foot frame distraction without bony foot procedures in 5 patients. Delayed consolidation and recurrent equinus contracture of the ankle requiring additional lengthening of the Achilles tendon were the most common bone and joint complications during tibial lengthening. INTERPRETATION: The gradual foot frame distraction method was associated with major complications, such as recurrent foot deformity, joint luxation, and arthritis. We therefore recommend triple arthrodesis as a concomitant procedure during tibial lengthening