Long term neurological conditions (LTNC) comprise a diverse set of conditions resulting from disease or injury of the nervous system which affects an individual for life. These can include acquired brain or spinal cord injuries, neurogenetic/chromosomal or metabolic disorders, and genetic, muscular and neurodegenerative conditions such as cerebral palsy, epilepsy and scoliosis.

Children with LTNC are often affected by secondary musculoskeletal problems1 such as limb stiffness and weakness, spasticity, hypotonia, dystonia, pain and discomfort. The hip is a commonly reported site of pain2-4; 26 to 35 per cent of young patients with cerebral palsy are affected by hip displacement1,5-7 which can cause significant pain. Pain in the hip has also been associated with a lower health-related quality of life8,9.

These problems and associated issues can lead to impaired posture and mobility, interfere with the patient’s ability and tolerance for sitting and transfers, disrupt their sleeping and feeding patterns, affect their personal hygiene, and decrease participation in activities within the home and community, leading to a poor quality of life for both the child and caregiver.

 

KKH HIP SURVEILLANCE PROGRAMME

KK Women’s and Children’s Hospital (KKH) sees about 300 new cases of children with LTNC each year, and about 21.6 per cent of child patients with LTNC at KKH are found to experience hip displacement10. To optimise the standard of care and quality of life for these patients with a diverse range of conditions, the hospital has introduced a Hip Surveillance Programme for children with LTNC who experience impaired mobility and are at risk for hip displacement and associated complications.

Early identification of pain in the hip area is an essential part of the strategy for prevention of hip displacement and its sequelae, and hip surveillance is the process of identifying and monitoring the critical early indicators of progressive hip displacement.

The KKH Hip Surveillance Programme aims to:

  1. Facilitate the timely identification of children with LTNC who are at risk of hip displacement and dislocation
  2. Aid posture management
  3. Improve quality of life, ease of care, comfort and sleep
  4. Prevent functional musculoskeletal deformities
  5. Minimise the need for invasive limb-salvage surgery

Children who are eligible for the KKH Hip Surveillance Programme include those with cerebral palsy and LTNC who are not able to sit independently by 18 months, not able to walk 10 steps independently by five years, and who exhibit abnormal and/or restricted hip abduction or symptoms upon examination.

Such children can be at risk of hip displacement, and should undergo their first pelvic X-ray between 18 to 24 months, or at first clinical sign of hip problems.

 

HIP SURVEILLANCE SCHEDULE

Initiation and Frequency

Under the Hip Surveillance Programme schedule (Figure 1), hip surveillance for the child with LTNC is recommended to be initiated by two years, and maintained until the child is 16 years old, which is usually when children reach skeletal maturity.

As hip displacement is related to gross motor function, surveillance frequency increases with increasing GMFCS level as defined by the five-level Gross Motor Function Classification System (GMFCS)1,5-7

GMFCS I​Walks without limitation
GMFCS II Walks with limitation
GMFCS III ​Walks using a hand-held mobility device
GMFCS IV ​Self-mobility with limitation; may use powered mobility
GMFCS V ​Transported in a manual wheelchair

 

Clinical Assessment

Hip surveillance for children with LTNC requires both clinical and radiological review. Regular clinical assessment includes asking the child’s caregiver about hip pain during movement, after prolonged activity or while performing perineal care.

It also involves review of growth parameters, feeding history and sleep history. Special attention should be paid to sitting and standing postures. In addition, a focused examination looking for limb length discrepancy (LLD), pelvic obliquity, scoliosis and range of motion of the hip (limited abduction) will allow the physician to detect associated musculoskeletal conditions in children with LTNC. As the expression of pain in children with LTNC may be very varied, great care should be taken to look for pain during the process of clinical examination.

 

Radiological Assessment

Radiological assessment involves taking regular antero-posterior (AP) pelvic radiographs with the legs in neutral abduction and adduction to measure migration percentage (MP) of the hip bones11,12.

MP is a radiographic measure of the amount of ossified femoral head that is not covered by the ossified acetabular roof, and plays a key role in providing an indication of the risk of hip displacement, as well as the recommended clinical and/or rehabilitative management.

A hip is considered ‘at risk’ of displacement when its MP is greater than 30 per cent. Conversely, the frequency of radiological assessment may be reduced when the MP is less than 30 per cent and has remained stable (less than 10 per cent deviation over a 12-month period) over two years.

 

MANAGEMENT AND INTERVENTION

Hip surveillance and management requires a multi-disciplinary approach. Various care teams are involved, including the primary care provider, neurologist and neurorehabilitation physician, orthopaedic surgeon, therapist, orthotist and dietician.

With early recognition and regular monitoring, at-risk patients can be identified early and their caregivers given advice on 24-hour postural management, nutrition and equipment.

The assessment and management protocol for patients with LTNC is informed by the patient’s hip MP measurement (Figure 2). Where a child with LTNC has a hip MP greater than 30 per cent, they will be referred to the KKH Paediatric Integrated Neurorehabilitation Service (PINS) for family-centred inter-disciplinary management.

At PINS, the child and their caregivers will receive closer monitoring and given advice as well as appropriate intervention to delay the progression of hip displacement. If surgical intervention is indicated, post-operative rehabilitation is pre-planned and coordinated.

 

RECOMMENDATIONS FOR HEALTHCARE PROVIDERS

  1. Regular clinical and radiographic hip assessment should be conducted for children with LTNC, especially those with gross motor function levels GMFCS III-V. Refer to Figure 1 for the recommended surveillance schedule.
  2. Patients should be referred to KKH PINS if any of the following symptoms present:
    a) A hip MP of more than 30 per cent
    b) Limited range of motion in hip abduction less than 30 degrees when measured with the hips at zero degree flexion and knees extended
    c) Presence of pain on clinical examination
    d) Any other clinical concern that is felt to be related to the hip

 

CASE STUDY: HIP SURVEILLANCE FOR CHILD WITH SPASTIC QUADRIPLEGIC CEREBRAL PALSY

Hip surveillance was commenced for a child with spastic quadriplegic cerebral palsy with GMFCS IV, at the age of two years (Figure 4A). He subsequently developed left hip displacement, and the hip MP continued to progress despite medical management of spasticity (Figure 4B).

At four years, the child underwent surgical release of the left hip adductor and an iliopsoas tenotomy to facilitate repositioning of the left femoral head (Figure 4C). Post-operatively, a SWASH brace (standing, walking and sitting hip orthosis) was prescribed to maintain the position of the femoral head.

At seven years, the child’s left hip remained enlocated and he remained symptom-free (Figure 4D).

 

 

The co-authors of this article gratefully acknowledge the support and involvement of the following KKH colleagues in the KKH Hip Surveillance Programme: Associate Professor Derrick Chan, Head and Senior Consultant; Associate Professor Choong Chew Thye, Senior Consultant; and Dr Lim Kim Whee, Consultant, Neurology Service; Dr Ehab Shaban Mahmoud Hamouda, Consultant, Department of Diagnostic and Interventional Imaging; Ms Tan Ling Ying, Neurology Specialty Nurse, and the Physiotherapy Department.

 

Dr Ng Zhi Min, Consultant, Neurology Service, KK Women’s and Children’s Hospital

Awarded the SingHealth Health Manpower Developmental Programme fellowship award in 2015, Dr Ng Zhi Min underwent training in paediatric rehabilitation with Harvard Medical School as a clinical fellow at Spaulding Rehabilitation Hospital in Boston, Massachusetts, USA. Dr Ng has a special interest in paediatric neurorehabilitation.

Dr Yeo Tong Hong, Consultant, Neurology Service, KK Women’s and Children’s Hospital

Dr Yeo Tong Hong completed his postgraduate Basic Specialist Training at Children’s Hospital for Wales, Cardiff, and Higher Specialist Training at Southampton Children’s Hospital, Southampton, before joining the Neurology Service at KKH in December 2016. Dr Yeo’s special interests in paediatric neurology include complex motor disorders, movement disorders, neuromodulation, and neurovascular disorders.

Associate Professor Arjandas S/O Mahadev, Head and Senior Consultant, Department of Orthopaedic Surgery, KK Women’s and Children’s Hospital

Associate Professor Arjandas S/O Mahadev’s main interest lies in the management of paediatric foot and ankle, and hip conditions. A/Prof Mahadev completed his fellowship in paediatric orthopaedic at Children's Hospital, San Diego, and is a corresponding member of the Paediatric Orthopaedic Society of North America. A/Prof Mahadev also has a special interest in orthopaedic complications of spastic cerebral palsy, and volunteers with the Cerebral Palsy Alliance Singapore.

 

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