Common paediatric conditions

Dr. Mackie provides treatment for children’s (paediatric) orthopaedic conditions primarily through the Royal Hobart Hospital.

Developmental Dysplasia of the Hip

Dr. Mackie treats this condition at the Royal Hobart Hospital due to the requirements of paediatric physiotherapy support, brace specialist support, and sometimes the need for paediatric surgery hospital admission.

Developmental dysplasia of the hip (DDH - previously called congenital dislocation of the hip) is a condition in which the hip socket does not develop normally or the hip is more lax than it should be, allowing the hip ball to slide out of its socket. The hip is a 'ball and socket' joint. Normally the top of the thighbone (femur) has a round ball shape which fits into a cup like socket on the pelvis (acetabulum). DDH may be present at birth or develop during the 1st year or two of life. There are many grades of severity of the condition, from almost imperceptible abnormal motion of the hip to obvious dislocation of the hip.

There is a significant genetic component to DDH.

The left hip is much more commonly affected due to the normal positioning of babies in the uterus and the pressure effect on the left hip by the mother’s spine. Both hips may be affected in ~ 20 % of the babies with DDH.

There is a higher risk in:

Known family history of DDH
1st born girls
Breech presentation late in pregnancy or birth
Low volume of amniotic fluid in the uterus
Sometimes associated with foot and neck deformities

The acetabulum will not develop properly unless the femoral head is properly positioned (to allow the acetabulum to “mould” to the correct shape). If the femoral head is allowed to stay out of position too long the hip is at risk of longer-term arthritis and pain.

All babies are examined shortly after birth and during early baby checks for hip instability.

Approximately 1 in 10 newborn babies will have a “clicky hip” or hip that partially slides out of position. Most of these will resolve and normal hip development will continue by around 3 months of age, defining them as “immature hips”.

Approximately 1 in 100 babies will require treatment to hold the hip in its correct position and permit normal hip development. Babies will have a click or clunk in their hip when it is moved. The hip is not painful. They may have asymmetric skin folds in the groin. On careful examination the amount of movement is usually less in the affected hip. Some children present with DDH at an older age because a limp is noticed as they stand and start to walk.

Approximately 1 in 1000 babies will have a hip that is fully dislocated, with an obvious difference in their leg length and very differing motion in each hip.

DDH generally causes no pain to babies, therefore an examination focuses on the apparent stability of the hip, the appearance of the creases around the hip, and the range of motion. X-rays are generally not helpful in the first 3 months because most of the hip joint is cartilage (not seen on X-rays).

Ultrasound scans are performed and involve no radiation to the baby, are not invasive and provide information on the position of the hip and its development (and response to treatment).

A variety of treatment options have been used, primarily involving some form of early bracing. Some babies with a “borderline” examination and ultrasound simply need to be monitored with a repeat clinical and ultrasound assessment. Many of these will fall into the “1:10” group of babies in whom the hip is just a little slower to develop and will not need treatment. Most babies who require intervention will have a good response to brace treatment, although surgery can help those in whom hip development is still poor.  If treatment is suggested but not commenced there is a higher chance of later hip restrictions and pain. Untreated hips will likely develop very early arthritis although modern treatments are expected to delay this arthritis risk.

The use of “double or triple nappies” instead of a splint or harness is no longer recommended.

Pavlick Harness

The harness should be considered a “dynamic brace”. It helps to redirect the hips into a more normal position and allows the baby to kick their legs and keep the hip mobile. As the baby kicks their knee and ankle the straps on the Pavlick harness ensure the hip is even better positioned to allow femoral head contact to the acetabulum.

Babies adjust to a Pavlick harness very quickly. Most parents find that they can adjust to bathing and clothing changes within the first week.

Treatment begins as soon as possible after diagnosis.

The harness is fitted by an Orthotics / Prosthetics specialist or specially trained physiotherapist and is adjusted as required. It is kept on at all times. Depending on the degree of severity in individual patients the brace may be able to be removed for bathing times only. Parents will be educated about the brace and what to watch for to ensure it causes their baby no problems. Difficulties with the harness are very rare.

The effectiveness of the brace is monitored by regular clinical and ultrasound review.

Treatment is required for a minimum of 8 weeks, although often for longer periods. It is very much Dr. Mackie’s preference to continue brace treatment for a short while after the hip examination and imaging improve. This helps reduce recurrent deformity and the need for further treatment. The brace is often “weaned” off over many weeks.

If a Pavlick harness treatment has been unsuccessful or if a child presents at a later stage (with a high chance of Pavlick harness failure) then a surgical intervention may be required. The type and extent of surgery is individualised to the presentation and clinical findings.

Further Treatments

Several surgical treatment options are outlined separately below:

Closed reduction and spica cast

Under a brief general anaesthetic the baby’s hip is examined (with all muscles relaxed). If the hip is able to be safely and accurately positioned a plaster “spica” cast is applied. A hip spica cast is applied with plaster or fiberglass and holds the legs in an outset position which helps direct the femoral heads into the acetabulum. A cutout in the plaster allows for toileting and nappy application. Babies very quickly adjust to being in a spica and parents will receive education from nurses and therapists on how to care for babies in a spica cast.

Occasionally an injection of dye into the hip joint will allow a more accurate XRAY assessment of the hip position. After the procedure a scan will be performed to ensure the hip remains in the correct position. A spica cast is usually left on for a minimum of 6 - 8 weeks. A second period in the cast is often required (with the cast needing to be changed under an anaesthetic). The cast change may be required because of ongoing growth making the initial cast too tight to leave on beyond 8 - 10 weeks.

This form of management is commonly required for babies who are first identified as having dysplasia after 6 months of age.

Tenotomy, closed reduction and spica cast

In some babies the hip cannot be safely positioned because of tension in a muscle and tendon on the inner thigh. This tension predisposes to the hip losing position or to undue pressure on the cartilage of the hip joint. A small incision in the groin allows release of this tendon and easier hip positioning. The incision is only 2 - 3 cm long, heals rapidly and requires only 24 - 48 hours of pain relief. Most babies can be discharge within 48 hours from hospital after spica checks and scans.

Medial open reduction of the hip

If the hip joint has been out of position for some time the soft tissues around the hip tighten and prevent reduction / positioning. An operative procedure via a groin incision releases all tight structures to allow the reduction. This is most commonly required in babies presenting after 1 year of age. The scar is a little longer than for tenotomy but most babies will not need pain killers beyond ~ 3 - 4 days. A spica cast is also required to hold the corrected position whilst everything heals.

Anterolateral reduction of the hip

In older babies and children (> 2 years old) the operative release of tight structures is best performed from the side of the hip, rather than via the groin incision. Often the older child has already started to develop an abnormal bone growth and requires one of the osteotomies outlined below.

Pelvic osteotomy

If the acetabulum (cup) has failed to develop normally there is often inadequate support for the ball of the hip joint. The pelvic bone may be cut and redirected to provide the necessary support. Dr. Mackie performs this surgery on children between the ages of 18 months and 4 years, especially if they have a very late-presenting hip dislocation. The commonest type of pelvic osteotomy performed is a “Salter Osteotomy” which was developed by a Canadian surgeon with whom Dr Mackie spent some time during an international fellowship in Toronto.

The pelvis bone is cut immediately above the hip joint. If required the hip joint is also operated upon at the time to position the femoral head better within the acetabulum.

The lower part of the pelvis (with the acetabulum) is rotated by approximately 25 degrees to make the acetabulum roof more horizontal. A wedge of bone is cut from the top of the pelvis.

The wedge of bone is inserted (bone graft) into the gap to hold the corrected position. 2 pins are drilled into the bone to further protect the new position and bone graft. The pins are generally removed in a “day-case” operation 3 months later when the bone is solid in its new position.

A plaster spica is required for protection of the surgery until bone healing. After approximately 6 – 8 weeks the spica can be removed in the outpatient clinic and a different plastic brace used for at least the next 1 – 2 months to allow a gradual transition to normal walking. Children may require a period of physiotherapy assistance as their muscles and ligaments will be tight and sore after the plaster immobilisation.

The pins are generally removed in a “day-case” operation 3 – 6 months after osteotomy, when the bone is solid in its new position.

Femoral osteotomy

Dr. Mackie tends to reserve this operation for older children with more severe deformities and it will normally be performed in conjunction with a pelvic osteotomy. To promote normal hip stability the femoral neck and head may be redirected to angle in towards the acetabulum. A plate and screws holds the femur in its new position until the bone has healed. Again, the children are restricted in their activities and weight bearing for at least 8 weeks.

Long Term Monitoring

All children who have had some form of intervention for DDH should be monitored during their growth. A very small number may require future surgery (similar to a pelvic osteotomy) because of a recurrence of the deformity.

In general the monitoring involves yearly or 2-yearly checks with an X-ray, although if children limp or develop pain in their hip they should be reassessed earlier

Most children who have a good early response to treatment should still have an XRAY check at approximately 4 years of age, 8-9 years of age and again at 13 - 14 years.

Clubfeet

“Clubfeet” or “Congenital Talipes Equinovarus” is a condition usually evident at birth in which a baby’s foot (or feet) is turned inwards and rotated. If only one foot is affected it is often smaller than the other and the calf is thinner.

Most babies with clubfeet will be referred within days to weeks of birth for assessment although some are even diagnosed prior to birth by ultrasound.

The in-turning that occurs in clubfeet is likely due to a combination of factors.

There are abnormalities in how the foot has developed in:

Bones
Joints
Muscles / Tendons
Blood Supply

Occasionally different deformities in the foot are apparent at birth, which have a different underlying abnormality than clubfeet. These conditions need to be separately addressed.

Why Do Clubfeet Occur?

We are still uncertain as to the exact cause of clubfeet. There is quite likely a significant genetic component. But parents have not done anything “wrong” during the pregnancy to cause the deformity.

Clubfeet occur in approximately 1 in 1000 births, although if one child has clubfeet in a family there is closer to a 1 in 30 chance of other children having the deformity.

Symptoms of Clubfeet

Clubfeet are not painful to babies. Treatment however is required to prevent long-term deformity, which does become painful in older children and adults. Although babies cry when plasters are being applied during treatment this is usually due simply to “handling” the feet. Most babies appear quite comfortable in their casts.

Assessing Clubfeet

Clubfeet are diagnosed by clinical examination of the position and flexibility of the foot.

X-rays in babies are rarely performed in assessing clubfeet. Most of the bones in a baby’s foot have not yet calcified and cannot be seen on X-ray. An X-ray may occasionally be performed to exclude a different congenital foot deformity.

Treatment Options for Clubfeet

For many years the principal method of treatment for clubfeet involved a period of manipulation and plaster immobilisation, followed by an extensive surgical procedure. In some countries an intensive physiotherapy and manipulation program has proven successful (although often requiring prolonged hospitalisation).

Dr. Mackie uses a treatment developed by Dr. Ignacio Ponsetti in the U.S.A. that involves a series of weekly manipulations and cast immobilization focusing on a technique to reduce the number and extent of surgical procedures to children. The excellent results of “The Ponsetti Technique” which have been reproduced worldwide have been dependent on a very active role being played by parents in their child’s treatment.

The treatment is best provided at The Royal Hobart Hospital by a specially trained physiotherapist (who oversees the manipulation, plaster casting and braces). The weekly casting that is performed by the one physiotherapist allows a uniform technique to be followed and requires the assistance of an experienced cast technician as available at the Royal Hobart Hospital outpatient clinic. Dr. Mackie reviews the progress on a regular basis and intervenes with surgical procedures when requested by the physiotherapist.

Stage 1: Manipulation and Casting

Treatment should begin right away to have the best chance for a successful outcome without the need for surgery. Most babies will have their first cast applied in the first 1 - 2 weeks of life.

Each week the cast(s) is removed immediately prior to the next manipulation. Parents should try to time feeding of their baby so that the baby can feed shortly before the manipulation. This helps to relax the baby. A pacifier or bottle should also be available at the time.

An assessment of the current status of the deformity and its flexibility is made and the foot is then gently manipulated and held for a short period in a corrected position. A plaster cast is applied to the foot and leg (extending above the knee to help provide more control of the foot position). The process may take ~15 - 30 minutes.

Casts are changed weekly for the first 4 - 5 weeks of treatment. It is important to understand that the Ponsetti technique involves a systematic and slow correction of the “total” deformity and the first 2 casts will often be applied in a manner that makes the in-turning look very obvious. Subsequent casts will progressively rotate the foot outwards in relation to the leg.

Stage 2: Tenotomy

In many babies the final deformity correction may be impeded by a tight achilles tendon (heel cord). A 5-minute surgical procedure may be required to cut the heel cord (via a tiny skin incision) to permit complete correction in the final cast (commonly ~ 5th cast). This can be performed safely under local anaesthetic in the outpatient department. If parents prefer their child to have this procedure performed under a general anaesthetic the baby will need to fast, be admitted to hospital and will need to stay overnight for observation after their anaesthetic.

Approximately 80 % of babies will require the heel cord release.

This final cast will be left on for approximately 2 - 3 weeks instead of just 1 week.

Stage 3: Bracing

Once the final correction has been obtained it must be maintained during further foot growth. Babies will be fitted with a specially designed set of boots with a connecting bar which positions the feet in a manner that allows them to grow without recurrence of the deformity. These boots will need to be used full-time (except for bathing) for the next 3 months. The boots are then fitted at night-time only (for sleeping) for 2 - 3 years. Babies and infants tolerate these boots very well and (importantly) will be free of the boots during daytime by the time they commence standing / walking.

With this method the number of subsequent operations required is very low. Some children may undergo a tendon transfer operation after 4 years of age if there remains a minor muscle imbalance.

Tibialis Anterior Tendon Transfer

This operation is performed on ~ 1/3 of children which have been treated with Ponsetti casting and involves balancing the muscle forces in the older foot. It is a relatively minor surgical procedure, usually requiring a single night admission in hospital and the protection of a plaster cast for 6 weeks. A splint may need to be manufactured to wear for another 2 – 3 months to help protect the tendon that has been moved in the foot while it regains strength.

More Complex Operations

With the use of the Ponsetti method for clubfoot management extensive surgical procedures are minimised. Very few babies require a surgical release of other tight structures around the back of the foot and heel.

Occasionally a late-presenting patient or a patient who has unsuccessfully been treated with the casting techniques will have the surgical release of all of the tight structures in the hindfoot and ankle. They will be in hospital for 1 - 2 days only. Postoperatively a cast is applied for 6 weeks and then a splint is manufactured for long-term use.

Coping with Casts and Boots

As indicated: Babies tolerate the casts and boots very well. Once parents become used to the “ritual” of fitting boots they also notice very little inconvenience. Most reports on the Ponsetti technique show a greater degree of parent satisfaction with the treatment than other techniques.

When a baby has casts on it is important to check the casts on a regular basis. Any obvious discoloration of toes, increasing swelling, reduced movement should be reported. The cast should be kept dry (requiring a modification on bathing the baby). When a baby with heavy casts on is lying on their back there should be a cushion beneath the knee portion of the cast to avoid constant pressure on the heel.

When the boots are fitted

If a baby has only one foot affected by a clubfoot condition then up until this stage a cast will have only been applied to that one leg. During the bracing period boots must be fitted to both feet so that a bar connecting the boots can correctly position the affected foot. Most babies will be upset in the first 24 hours at not being able to move their feet independently of each other. All babies very quickly adjust. Parents will too!

Some parents may use 2 pairs of cotton socks until their baby has adjusted to the boots.
It is important to ensure the heel is positioned right down on the sole of the boot.
It is important to ensure the straps are tight enough to prevent the heel from lifting out of the boot.
It is important to check that all toes are visible and have good circulation.
It is easy to commence a “routine” to which both baby and parents become accustomed – i.e. “putting on the shoes before bedtime” should be a normal routine for toddlers in the last stages of the Ponsetti treatment.

Although the foot generally has a good appearance at the end of treatment a single-sided clubfoot usually leaves children with a smaller calf muscle.

Outcomes

Without treatment:

Ongoing deformity produces pain and impaired function.

With treatment:

Most children have a near normal looking foot at adulthood, although the foot may be smaller than the other side and the calf muscles are always a little thinner. Normal function is possible and children participate in sports with their peers.

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