A systematic approach is helpful when assessing a child. Look at the overall appearance, taking note of the child’s colour (pale or healthy), their stature and posture. How does the child stand? Look at the leg alignment. Is there evidence of asymmetry, such as genu varum/valgum (Figure 22.1), an abnormal rotational profile or leg length difference? Is there evidence of dysfunction? For example, tripping up, a limp, reduced range of motion in a joint or disability.

Normal variants

Many referrals from general practitioners to orthopaedic services relate to concerns regarding deviation from what parents believe to be the norm. Moloney et al., (2006) determined that half of child referrals (53%) to the hospital present with normal variants, predominated by in-toeing gait and flexible flat feet. In-toeing may be caused by femoral anteversion, tibial torsion or metatarsus adductus (forefoot adduction). An out-toeing gait may be due to femoral retroversion, external tibial torsion or flat feet. All of these are normal variants and are expected to resolve naturally with growth.

Developmental dysplasia of the hip, a neuromuscular problem, clubfoot or a slipped capital femoral epiphysis, however, contribute to in-toeing or out-toeing torsional abnormalities and will require orthopaedic intervention. Flat feet are commonly referred as a result of concern raised by a shoe shop. If the foot is flexible with the arch restored on tip toe standing, then parents can be reassured. The normal age for arch development is by age six although, if the child has ligamentous laxity, they may always have normal flexible flat feet. Orthotics are not required to restore an arch in a normal foot and are used only for the symptomatic foot.

Genu varum (bow legs) and genu valgum (knock knees) deformities can be physiological or pathological and diagnosis is made based on the age of the child, X-ray appearances and progression versus resolution. At birth, a baby’s legs are naturally bowed and this can look progressively worse, especially if the child walks at a young age. By the age of two years the legs will straighten, followed by valgus deformity between the ages of three to four years and gradual correction to normal by age six. It is important to exclude pathology such as rickets, Blounts disease or a metaphyseal dysplasia and to be aware that a unilateral deformity is probably pathological in origin.

Common conditions presenting in the neonate, infant, child and young person

Congenital muscular torticollis

Congenital muscular torticollis is a benign condition that is usually detected in early infancy. The baby tilts their head towards the affected side and turns their face in the opposite direction. This is caused by a fibrous tissue mass within the sternocleidomastoid muscle (SCM). The reason for this is unclear, but may be due to in-utero crowding or a decrease in the blood supply to the muscle. On palpation a firm tumour can be felt in the neck and there may be accompanying plagiocephaly (Luther 2002). Hollier et al. (2000) report a high incidence of 1 per 250 live births, associating difficult births as a causative factor. Resolution is usually within four to six months with stretching exercises if the condition is without other association. The incidence of developmental dysplasia of the hip in an infant with torticollis varies between 2–29% and should be excluded with a hip scan at six weeks of age (von Heideken et al., 2006). Physiotherapy is the mainstay of treatment and parents are taught stretching exercises to continue at home. For those who do not respond favourably by twelve months, a surgical release of the SCM is performed.

Developmental dysplasia of the hip

Developmental dysplasia of the hip (DDH) is the term used to describe a spectrum of disorders affecting the infant hip. Previously known as congenital dislocation of the hip (CDH), the term was changed to DDH to reflect that the condition is dynamic, can change and is not always detectable at birth. The hip joint may be dysplastic with a shallow acetabulum (acetabular dysplasia) that is unstable and subluxing or completely dislocated. Early recognition and appropriate treatment by skilled practitioners, predetermines a good outcome. Clinical guidelines (NIPE 2010) promote a uniform approach to infant screening and detecting abnormality promptly. Treatment decisions are determined by abnormal clinical and sonographic examination (Clarke and Castaneda 2012) and are reliant on the competence of the practitioner (RCN 2012). The primary aim of DDH treatment is to achieve a concentric and stable hip joint and ultimately normal development of the acetabulum and proximal femur with minimal possibility for subsequent reconstructive surgical intervention (Bolland et al., 2010). Early osteoarthritis, chronic pain and a reduction in activity levels are all possible sequelae of DDH and the earlier the intervention the more likely a successful outcome.

Aetiology and epidemiology

DDH is the most common congenital newborn defect (75%). The cause is unknown, although there are multifactorial traits; gender, hormonal influence, race, hyperlaxity, uterine malposition, geographic and environmental influences. Box 22.1 outlines some of the associated risk factors. The incidence of true DDH is difficult to determine accurately as the definition lends itself to a broad spectrum of the condition, giving variance to the accurate incidence. Over the years, authors have strived to clarifiy the terminology and there have been improved methods of detection and diagnosis. The introduction of screening of neonates at risk and sonography has improved diagnostic technique (Kokavec and Bialik 2007). Reported incidence of hip instability is as high as 20 per 1000 live births and varies depending on geographic location. Many of these, which are due to ligamentous laxity, stabilise within the first couple of weeks. The incidence of true hip dislocation in the United Kingdom (UK) is reported as one to two per 1000 live births (Clarke and Taylor 2012).

DDH is more common in females, with increased prevalence in first born infants. The latter is believed to be due to the tight structure of the uterus and subsequent reduced capacity for foetal movement. The risk of late DDH was found to be 29% in breech presentation at four to six month follow-up (Imrie et al., 2010), despite normal ultrasound at six weeks. Research studies to assess prophylactic hip abduction splints for this category of patient are ongoing (Clarke and Judd 2012).

Box 22.1 Associated risk factors for DDH

The infant hip is at risk of DDH if the baby is subject to the recognised risk factors. Early appropriate intervention will result in normal hip development in the majority of babies.

Diagnosis

A routine part of the neonate’s postpartum check is hip examination. The practitioner assesses for equal leg length and looks for asymmetry of the gluteal and thigh folds. More importantly, reduced hip abduction and instability are indicative of DDH. In the UK the NHS Newborn and Physical Examination Programme (NIPE) recommend examiners be trained and are competent in the Ortolani and Barlow tests (NIPE 2010). The Barlow test demonstrates hip instability as the hip displaces posteriorly out of the actebulum and the Ortolani test produces an audible clunk as the dislocated hip is relocated back into the acetabulum. Babies with a positive clinical examination and those that meet the screening criteria are referred to a specialist clinic for hip ultrasound (see Box 22.2 and Figure 22.2).

Box 22.2 Infant hip screening criteria (NIPE 2010)

As an adjunct to clinical assessment, ultrasound clarifies clinical findings. It is not feasible, however, to scan all babies’ hips due to the inordinate expense and requirement for resources (American Academy of Pediatrics 2000). Guidelines ensure practitioners make the appropriate referrals at the correct time (Box 22.2).

It is important to obtain a good family and birth history (Judd 2012a) prior to a clinical and sonographic examination. The ossific nucleus (secondary ossification centre) is normally present in the infant’s hip at the approximate age of four months but later in an infant with DDH. An X-ray therefore is not helpful in determining the hip structure and ultrasound is the gold standard to show true hip dislocation or dysplasia. A static coronal view can be measured using the Graf method to assess for dyplasia (Eastwood and de Gheldere 2010). A further dynamic stress test in the transverse plane will assess for instability. Combined, the two images detect the degree of abnormal hip morphology.

Late diagnosis of DDH is commonly detected by the parent who notices their child has a short leg or a limp when there is a unilateral presentation. A child with bilateral dislocated hips will present with a waddling gait. Confirmation is made by X-ray (see Figure 22.4) and clinical examination. Hip abduction is markedly reduced and if unilateral, the affected side shows a shortened limb. The later the diagnosis, the more interventional the treatment and potential for the development of degenerative joint disease. Because DDH is often neglected or treated inappropriately it has become the most common cause of secondary osteoarthritis of the hip.

Guidelines (NIPE 2010) advise the practitioner on when to refer to a specialist paediatric orthopaedic doctor and tested algorithms of treatment are available in the literature (Clarke and Taylor 2012). Multi-centred research studies aim to amalgamate findings to improve knowledge of DDH management and to test new theories. The Institute of Infant Hip Dysplasia (IHDI) strives to create a gold standard for referral and treatment, looking at timing of intervention, method of treatment, failure and complication rates.

Treatment

Treatment of DDH is dependent on the age of the child at presentation (Clarke and Taylor 2012):

Newborn to four months

The Pavlik harness is the most common hip abduction orthosis for the treatment of DDH. Reported as having a 95% success rate, it is effective for dysplastic and unstable hips. It should be abandoned early if a fixed irreducible hip fails to respond to treatment in the first week as there is an increased risk of subsequent avascular necrosis (AVN). It is also contraindicated in neurological hip dislocation (Clarke and Taylor 2012). The harness consists of shoulder and leg straps secured to a chest band (Figure 22.3).

The optimum position for treatment and prevention of complications, such as femoral nerve palsy and AVN, is 90 degrees of hip flexion and 60 degrees of hip abduction. Weekly ultrasound scans confirm successful treatment and allows the practitioner to check and adjust the harness position and ensure compliance. The infant wears the harness for six weeks full time, following which they are gradually weaned out of it. Follow-up is recommended with serial X-rays until the age of five years to exclude residual dysplasia and late onset complications.

Parents are often upset by the diagnosis and the visual appearance of their baby in the harness. Close observation of the infant and reassurance and support of the parents are required to ensure successful treatment (Atalar et al., 2007). The consequence of not treating the infant’s hip is extremely detrimental and will condemn the child to significant surgical intervention later. It is important to give parents written information on how to care for their baby (Box 22.3 shows an abbreviated sample information leaflet) and advice on appropriate and useful websites (for further information of the care of a baby in a harness, see: www.steps-charity.org.uk).

Box 22.3 Parent’s information – Care of your baby in a Pavlik harness (NIPE 2010). Reproduced with permission from Crown copyright Clothing General care

Early years treatment

Between the ages of 4–12 months, surgical correction is necessary to reduce the hip. For these infants either the Pavlik harness failed and was abandoned early or the diagnosis was not detected on initial clinical examination. Surgical correction is usually deferred until the ossific nucleus is evident on scan, to prevent potential for subsequent AVN (Luedtke et al., 2000).

There is some consensus that a week of pre-operative gallows traction to stretch the vascular supply to the femoral head reduces the risk of post-operative AVN although this is not proven (Luedtke et al., 2000). Gallows traction is not a pre-requisite and does not affect whether the hip reduces by closed manipulation or by open surgery. An arthrogram and adductor tenotomy is performed as part of the surgical procedure and if the hip does not reduce concentrically with a closed manipulation, the hip joint is opened to remove obstructing soft tissue such as the labrum. The hip position may be confirmed post-operatively by CT scan to confirm concentric reduction. A hip spica in the human position maintains the femoral head in the acetabulum for six weeks if an open reduction or 12 weeks for a closed reduction (Chapter 21). Sequential broomstick casts (six weeks) and night splint casting (six weeks) may be used to complete the surgical programme (Table 22.1) or as an alternative ring splints or a hip abduction orthosis. For the older child who presents late (aged >18 months) a femoral shortening is required in addition to the open hip reduction to reduce the tension on the hip and protect from AVN. The 18-month-old child is too heavy for Gallows traction as they would be heavier than the upper weight limit of 16 kg (Davis and Barr 1999).

Further surgical procedures

Children who have undergone a closed or open hip reduction for either late presentation or failed Pavlik harness may subsequently need further surgery to improve acetabular cover. Residual hip dysplasia following treatment for DDH has a reported incidence of 2–17% (Cashman et al., 2002). Of those children requiring pelvic osteotomy 60% previously underwent a closed reduction of the hip and 20% open reduction. This is usually by the age of three to four years to prevent progressive dysplasia, instability and eventual early osteoarthritis. The most important predictive factor for further surgery was found to be the initial achievement of a stable concentric reduction at closed or open reduction (Bolland et al., 2010).

A pelvic osteotomy aims to realign the bony structure of the hip joint and therefore the weight bearing forces. A triangular shaped bone graft taken from the iliac crest is inserted into the osteotomy site above the acetabulum. This is fixed with two pins which are removed at six weeks under general anaesthetic (Figure 22.5). Post-operatively the child is nursed in a one and half hip spica cast to immobilse the hip joint until the bone graft has fused. The optimum timing is before school age, giving the femoral head and acetabulum opportunity to remodel with growth.

Specific nursing considerations

The child in a hip spica requires considerable care. It is a stressful time for the parents who require support emotionally and practically as well as needing written information on caring for their child (Clarke and McKay 2006).

An epidural is effective pain management for the first 48 hours if the child has had an open hip reduction after which Paracetamol is usually sufficient. This modality can provide excellent pain relief but can also be problematic. The line can migrate (comes out of epidural space), the child requires close observation and monitoring and the epidural site cannot not be visualised due to being covered by the spica.

The spica needs to dry naturally and the child is nursed on pillows alternating from front to back for the first 24 hours. A fibreglass covering is applied on day one post-operatively and waterproof tape is secured around the edges of the spica cast for protection (Sparks et al., 2004). The parents are taught ‘nappy care’ and instructed on the frequency of checking the nappy for dampness and changing promptly to prevent the spica from becoming soiled. The parents should check their child’s skin daily for signs of friction or rash. They can use a cloth to wash their child taking care not to get the cast wet. Hair washing is possible with the child resting on the legs of an adult who sits beside the bath. Holding the child’s head over the edge of the bath, another adult can use the shower attachment to wash the hair.

The child in a spica cast is both heavy and awkward to lift; refer to Chapter 21 to view a picture of a hip spica in the human position. The occupational therapist (OT) can teach the parents handling techniques and how to turn their child safely. An assessment of the home will highlight issues early. A hoist may be required in the home. If the child cannot be securely and safely transported in the car, it may be necessary to arrange ambulance transfer home.

There are a number of ways to clothe a child in a spica. Trousers can be split and Velcro added to secure seams. Dresses are the easiest clothing for girls. Online companies advertise specific clothing made especially for children in spica casts.

Intervention for the young person

As the child gets older, a hip with residual dysplasia will give intrusive pain. A total hip arthroplasty for young and active patients, however, is not the best option. There is a risk of loosening and the revision rates are high. Alternative non-arthroplasty choices for the young patient include proximal femoral and periacetabular osteotomies to re-align the femoral head or re-position the acetabulum to delay the onset of arthritis. Surgical management of the problematic hip in adolescent and young adult patients can be challenging and technically difficult to do. As well as hip pain the patients may also have associated chronic instability. The optimum time for operative intervention is before there is too much wear on the cartilage and before arthritis sets in.

Legg Calvé Perthes disease

Legg Calvé Perthes disease (LCPD) (also known as Perthes disease) is a condition of the child’s hip of unknown aetiology which results in a deformed femoral head due to avascular necrosis. The condition is believed to be due to ischemia of the femoral head due to an interruption of its blood supply. Studies suggest that the articular cartilage, the bony femoral epiphysis, the growing physis and the metaphysis are all affected (Catterall et al., 1982). The aim of treatment, whether conservative or surgical, is to manage the child’s symptoms and preserve hip joint congruency throughout the approximate two year disease process (Herring, 1998). Outcomes of Perthes disease are largely dependent on age at diagnosis as well as on treatment modalities (Daly et al., 1999). Studies suggest that >50% of patients will develop osteoarthritis in their 60s (Perry et al., 2012) and require early total hip replacements. Recent research has investigated the effect of bisphosphonates on animals with induced Perthes. Early results of ongoing research have shown effectiveness on increasing bone density, bone mineral content and strength (Little and Kim 2011).

Aetiology and epidemiology

The cause of Perthes is unknown. A study by Glueck et al. (1996), however, found 75% of participants had abnormal coagulation properties. Thrombophilia as a cause, however, has not been proven and debate regarding the reason for the temporary deficient blood supply of the femoral head continues (Kim 2010). Studies of epidemiology indicate a varied incidence of between 6–15.6 per 100 000. A higher incidence is reported in lower socioeconomic and urban areas and a lower incidence in rural areas. Ethnicity may be a factor as Caucasians are affected more than other races with fewer numbers affected in the African and Chinese populations. It is also more common in the Japanese and in some parts of central Europe (Nochimson 2011). The condition predominantly affects boys from four to eight years and children who are small for their age and have a low BMI (body mass index) (Judd and Wright 2005). The condition is bilateral in 10% of cases.

Diagnosis

The child usually presents to the general practitioner with a history of limp and complaints of pain in either their hip or knee. Clinical examination frequently reveals reduced hip abduction with pain at the extreme of movement on the affected side. An initial radiograph may show evidence of the disease, but often it is the subsequent X-rays or an MRI which demonstrates changes in the appearance of the femoral head and confirms the diagnosis (Dillman and Hernandez 2009) (see Figure 22.6). There is a number of classification systems used to grade the stage of the disease and used in the further monitoring of the disease status (Box 22.4). A bone age (left hand X-ray) is also useful in determining the child’s actual skeletal and chronological ages which assists with treatment planning strategies. MRI can also be used.

Box 22.4 Herring classification for grading stage of Perthes disease (Herring et al., 2004)