O R I G I N A L

A R T I C L E

Notes on the practicalities of circular frame treatment for limb lengthening and deformity correction in children
Alison Edwards, Rajesh Ramaswamy and Christopher Bradish
This article describes one hospital teams experience of limb lengthening and deformity correction using the Ilizarov technique of gradual distraction of soft tissues and bone. Some of the practical issues, which need to be considered by the multidisciplinary team involved in such patients care are explained as the surgical skills are only part of such a treatment method. c 2002 Elsevier Science Ltd. All rights reserved.

Editors comment This article describes one units multidisciplinary approach to an expanding area of orthopaedic surgery. PD

KEY WORDS: Circular frames, Ilizarov xators, Limb lengthening, Complications, Extremities

CHOOSING SUITABLE PATIENTS

Alison Edwards FRCS Specialist Registrar in Orthopaedics, Royal Orthopaedic Hospital, Birmingham, UK. Rajesh Ramaswamy MRCS Clinical Research Fellow in Orthopaedics, Royal Orthopaedic Hospital, Birmingham, UK. Christopher Bradish MA FRCS Consultant Orthopaedic Surgeon, Royal Orthopaedic Hospital, Birmingham, UK. Correspondence to: Christopher Bradish Royal Orthopaedic Hospital, Northeld, Birmingham, B31 2AP, UK. Tel: +44-121-6854000; Fax: +44-121-6854111; E-mail: bradish@ blueyonder.co.uk

Certain conditions lend themselves to circular frame treatment. A few of the more common conditions are related to:
Limb length discrepancies or short stature

The degree of length discrepancy or deformity usually dictates the treatment: Limb length discrepancy

Less than 2 cm usually not corrected as this

has been shown not to be associated with later morbidity. 24 cm usually treated with a shoe raise, may be a combination of internal and external raises to make cosmesis more satisfactory. The patient is usually left under-corrected by about 1 cm. Greater than 4 cm consider surgery (lengthening, epiphyseodesis, or shortening) Deformity

Congenital deformities achondroplasia, bular hemimelia, focal femoral defects, unilateral hypertrophy, or hypoplasia. Acquired conditions shortening due to premature epiphyseal closure, infection, juvenile chronic arthritis. Deformity

Club feet, especially if relapsed. Pseudarthrosis of the tibia, Blounts disease. Malunions.

Deformities resulting in a non-plantigrade

foot, and mobility diculties, consider surgery (Stanitski 1999).

Journal of Orthopaedic Nursing (2002) 6, 198203 2002 Elsevier Science Ltd. All rights reserved. doi:10.1016/S1361-3111(02)00071-7

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During limb lengthening at a single site, it is possible to lengthen approximately 2025% of the length of the bone. As a rule of thumb, a xator is applied for 1 month/cm of length achieved (Gugenheim 1998). The xators are secured to the limb by wires, nuts, bolts, and clickers (Figures 1ac). In fact the lengthening itself usually takes place at about 1 mm per day until the length is achieved, but the xator remains on until radiological evidence of corticalization of the regenerate. At least three cortices should be visible on the AP and lateral limb views before frame removal. Strength is also judged under anaesthesia before the frame is fully removed (Cattaneo et al. 1990). Children below the age of ve are not usually considered for limb lengthening. Around the age of 12 is said to be ideal, but it may be necessary to start earlier if more than one lengthening is anticipated. In deformity correction, the correction may be achieved acutely or gradually. The frame is used for stabilisation, and correction. If only used for stabilisation, then the length of time the frame will need to be on may be shorter, of the order of 612 weeks. Frames have the advantage that simultaneous deformity correction and lengthening may be undertaken if necessary, and ne adjustments made to the apparatus in terms of hinges and dierential lengthening on either side of the bone. Circular frames are more stable than monolateral xators and secondary deformities are seen less frequently.

psychological assessment pre-operatively. Many patients do experience sleep disturbance during treatment however.

Alternatives
In simple leg length discrepancies epiphyseodesis of the distal femur, proximal tibia, or both, may be performed in the longer leg in order to allow the other leg to catch up. If the child is predicted to have a normal or above average adult height then this is a good alternative. Timing is critical, however, to avoid over-shortening or not enough growth left to allow the short leg to catch up. The use of GreenAnderson and Moseley charts for prediction of the nal discrepancy and the amount of compensation that may be achieved by epiphyseodesis allows the most suitable time for surgery to be determined. Limb shortening, usually performed in the femur, where a segment of bone is removed and the bone internally xed to achieve stabilisation is another alternative. Acute correction of deformities with osteotomy and internal xation may also be performed. In conditions where the length discrepancy is very large or where the foot is severely deformed or insensate, then amputation may be an alternative. This has the advantage of the child mobilising soon afterwards with an appropriate prosthesis. Monolateral xators are an alternative to circular frames but whilst these are less bulky they are less adaptable. Predominant non-surgical alternatives include modication of footwear or the use of a prosthesis to compensate for the dierence in length.

Family considerations Treatment with ring xators in children requires a huge commitment on the part of the child and family. Pin-site cleaning, turning lengthening devices, attending physiotherapy classes 23 times per week, and clinics involves a lot of time and attention. Patients also need to continue their exercises at home with the help and the encouragement of family members. If the family cannot guarantee this then other forms of treatment should be considered.

SURGERY
This is a vital component of the decision making process for both patient and sta. In our unit in Birmingham special multidisciplinary clinics are held, which include the following components to help the patient and their family decide whether they wish to embark on the treatment, and for the sta to decide whether they are suitable or not. It also prepares patients for discharge and follow up care.

Psychological considerations It has been suggested that such frames are psychologically damaging to children. Many patients have psychological and social problems before the start of treatment as a result of their deformity. The duration of circular frame treatment and the many possible complications may be stressful. Serious emotional problems have been reported (Hrutkay and Eilert 1990), but other studies have shown no long-term serious psychological disturbances (Ramaker et al. 2000). This has in part been attributed to strong specialist support from the team and to careful

Visiting the ward and watching videos about

Ilizarov treatment

Seeing and talking to other children with

xators on.

Explanation of the surgery, possible

complications, pain control.

Walking about with a practice xator on. Explanation of physiotherapy regimes and
pin-site cleaning.

Psychological assessment.

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Fig. 1

Practicalities of circular frame treatment 201

Social work assessment. Occupational therapy assessment. Seeing the teaching unit and play leaders
Patients are usually in hospital for 57 days, until they are mobile and safe with crutches. The frame should be strong enough for the patient to partially weight bear, the frame taking the load across the distraction gap.

saline. See Lee-Smith et al. (2001) for a more detailed review of the literature. Certainly during the lengthening phase there is a tendency for inammation as the wire drags a track through the skin.

Postoperative care
Passing wires through the limb is not without the potential complications of neural or vascular injury, although this is uncommon. Post-operative pain control is important, and patients are often given epidural, regional, or patient controlled analgesia to cover the post-op period. Almost all children with xators on will have some form of complication. Paley divided these into; problems, obstacles, and complications (Paley 1990). Problems occur in 90% of children, but are manageable without surgical intervention and do not jeopardise the nal outcome. Pin-site infections would be an example, and are usually treated with oral antibiotics and local cleaning. Obstacles occur in fewer than 50% of cases and may need surgical intervention but again should not jeopardise the nal outcome, for example a pin-site abscess may need to be incised and drained. True complications occur in fewer than 5% of cases but may result in a sub-optimal outcome. There is a reported fracture rate of approximately 9% (Velazques et al. 1993), and fractures or deformity of the regenerate bone may necessitate re-application of a frame. Once a child has had a pin-site infection, he/she may need to remain on antibiotics until the xator is removed. Wires may lose tension and require re-tensioning; wires may break and require re-insertion. Joint subluxation may occur, particularly at the hip or knee. Extension of the frame to cross the knee joint or ankle may be required to prevent joint deformities. Patients sometimes need manipulation of a sti joint under anaesthesia. Some children may need elongation of their tendo-Achilles at the time of frame application if they have reduced foot dorsiexion pre-operatively. Children with a short leg will often have developed an equinus deformity and toe walk to compensate for the dierence in leg length.

How and when to turn the elongation devices Turning of the lengthening or correction device usually begins at about day 7. With an Ilizarov xator each nut is usually turned 4 or 5 times per day 1/4 turn each time. Each full turn represents 1 mm. Sometimes this is done slower or faster to keep pace with the formation of the regenerate bone. Some conditions produce regenerate quickly e.g., Olliers disease, some produce it slowly e.g., congenital short femur. Too slow a distraction rate and the bone will heal, too fast and the bone ends will be too far apart to allow good regenerate to form. Occasionally it is necessary to close the regenerate gap down the concertina manoeuvre, if bone is not forming in the gap, and then begin lengthening again once bone is forming. Towards the end of the treatment, the frame may be backed o or shortened by 2 mm or so, this does not really shorten the bone, but allows more weight to be transmitted through the bone as opposed to the frame, stimulating consolidation. Turning the correct nuts in the correct direction is obviously of great importance. In deformity correction, this may mean shortening one part and lengthening another. In most cases, special clickers (Figure 1c) are inserted, which have a rod inside a barrel, and the barrel is turned by pressing in a button, which allows 1/4 turn in either direction. There are indicators on the barrel to show whether the strut is increasing or decreasing in length. In some situations it is not possible to use these devices and therefore, instructions are given which nuts to turn. We often paint the appropriate nuts with nail varnish to distinguish them. We also avoid telling patients and carers to turn nuts clockwise or anticlockwise as obviously, depending on whether you are at the top or the bottom of the bed, clockwise will be in opposite directions. Schooling Some schools will not allow children with xators to return to school for health and safety reasons. Frequent visits to the hospital also cause disruption of education. As part of our pre-operative clinic assessment the hospital teachers and social workers formulate a plan so that a childs schooling is not severely interrupted. Home schooling takes some time to set up. Some children can attend school part time and have home support for the rest.

Pin-site care There is controversy about the extent to which patients need to clean their pins. A recent prospective study reported no increase in infection rate when children simply used daily showering to clean their frame (Gordon et al. 2000). Others advocate a more intensive approach using cotton buds to clean around each pin with

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LONG-TERM CARE
Most children are seen for clinical assessment and frame checks every two weeks, and X-rays taken to assess regenerate (Figures 2a and b). Adjustments often need to be made to the frames, in the form of changing of struts and clickers to allow continued lengthening. There must, however, be an open door policy for patients who are encountering diculties to return and be seen by an experienced member of sta. Frames are usually removed under GA, and may be followed by a brief period in a cast. Children may need to wear orthotic appliances, especially if they have had soft tissue correction, to prevent recurrence of the deformity. The patients are usually cast for their orthotic appliances at the time of frame removal. The role of the physiotherapists and occupational therapists is as much with the parents as patients, teaching them how to continue the ex-

ercises with their children and allaying fears. Patient motivation and compliance is hugely important in achieving a good result. It is aimed that the patients will be independent with an appropriate walking aid, able to manage stairs, perform transfers, toileting, and showering. Limb lengthening lengthens not only bone, but of course muscles, nerves, and vessels too. This is by growth of new tissue not simply stretching what is already there. However, muscles do need to be gently stretched to avoid contraction of an adjacent joint, since there will be tension generated across the joint and a tendency to exion of the joint to relax the muscle tension. Stretching can be uncomfortable, and the patient must be warned about it. The continuation of physiotherapy is vital despite some discomfort. It is often dicult to retain a full range of movement at joints adjacent to lengthening or correction sites, as there may be some tethering

Fig. 2

Practicalities of circular frame treatment 203

of muscles and tendons by the wires. This is kept to a minimum by proper placement of the wires. The range of movement usually returns following removal of the frame. A dynamic shoe raise is either a real shoe, or plaster shoe, with a series of soles stuck together with Velcro strips. As the patients leg increases in length, successive layers are peeled away, such that the patient remains level during the course of the treatment. Spring attachments may be put on the front of the shoe to keep the foot dorsiexed in the resting position or an ankle-foot orthosis to prevent equinus deformity. Following removal of the frame children may need to wear an orthotic appliance to prevent deformity recurrence, and this is especially true when the correction has been mainly soft tissue.

the patient and family at all stages of the treatment is essential.

REFERENCES Cattaneo R, Villa A, Catagni M, Bell D (1990) Modern techniques in limb lengthening: lengthening of the humerus using the Ilizarov technique. CORR 250: 117124 Gordon J, Kelly-Hahn J, Carpenter C, Schoenecker P (2000) Pin-site care during external xation in children: results of a nihilistic approach. Journal of Paediatric Orthopaedics 20: 163165 Gugenheim J (1998) The Ilizarov method. Clinics in Plastic Surgery 25(4): 567578 Hrutkay J, Eilert R (1990) Operative lengthening of the lower extremity and associated psychological consequences: the childrens hospital experience. Journal of Paediatric Orthopaedics 3: 373377 Lee-Smith J, Santy J, Davis P, Jester R, Kneale J (2001) Pin-site management. Towards a consensus: part 1. Journal of Orthopaedic Nursing 5: 3742 Paley D (1990) Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clinical Orthopaedics 250: 81104 Ramaker R, Lagro S, van Roermund P, Sinnema G, 2000. Acta Orthopaedic Scandinavia 71(1): 5559 Stanitski DF (1999) Limb length inequality: assessment and treatment options. Journal of American Academy of Orthopaedic Surgeons 79B: 433437 Velazques RJ, Bell DF, Armstrong PF, Babyn P, et al. (1993) Complications of use of the Ilizarov technique in the correction of limb deformities in children. Journal of Bone and Joint Surgery [Am] 75A: 1148 1156

CONCLUSION
This complex method of treatment of limblength discrepancy and deformity correction can produce good results. The intensive nature of the therapy and its duration can lead to disruption in a number of aspects of a childs life and relationships within the family, and the extent to which support and motivation are needed should not be underestimated. A multidisciplinary team approach, which involves, informs and educates