The hip in cerebral palsy

Hip displacement in children with cerebral palsy (CP) is a complex problem and requires a multidisciplinary team approach. It is the most common orthopaedic problem in the non-ambulatory group of children and causes significant pain, which can be prevented or treated with appropriate management. Hip subluxation occurs due to asymmetrical muscle imbalance in the context of abnormal proximal femoral morphology. Work from CP registries has shown strong correlation between Gross Motor Function Classification System (GMFCS) level and rate of hip displacement. This has led to the development of preventative surveillance programmes. This review focuses particularly on non-ambulatory patients (GMFCS IV/V) and the options open to the paediatric orthopaedic surgeon in managing associated hip displacement. Surgical strategies can be broadly grouped into preventative, reconstructive and salvage options.     

痉挛性脑瘫髋关节发育异常的外科治疗

脑性瘫痪简称脑瘫,是目前导致全世界儿童肢体残疾的主要疾病之一.痉挛性脑瘫以锥体系受损为主,继发性肌肉骨骼问题已成为其研究热点,其中髋关节发育不良及髋关节脱位在行走不能及严重型痉挛性脑瘫患儿中发生率越来越高.患儿出生时髋关节一般正常,约2岁左右开始出现股骨头外侧偏移为特征的髋关节发育不良,约4～12岁可发展为髋关节半脱位或脱位,约25％～75％的病例最终进展为影响日常生活的疼痛性、退行性髋关节炎.外科手术治疗配合系统的康复训练,可以改善痉挛性脑瘫儿童的步态和姿势,维持髋关节功能,提高其生活质量和社会参与度.本文就痉挛性脑瘫继发髋关节发育异常的术前评估、外科手术方式的选择、预后等研究进展进行综述.     

Cerebral palsy in Victoria: motor types, topography and gross motor function

Objectives:   To study the relationships between motor type, topographical distribution and gross motor function in a large, population-based cohort of children with cerebral palsy (CP), from the State of Victoria, and compare this cohort to similar cohorts from other countries.
Methods:   An inception cohort was generated from the Victorian Cerebral Palsy Register (VCPR) for the birth years 1990–1992. Demographic information, motor types and topographical distribution were obtained from the register and supplemented by grading gross motor function according to the Gross Motor Function Classification System (GMFCS).
Results:   Complete data were obtained on 323 (86%) of 374 children in the cohort. Gross motor function varied from GMFCS level I (35%) to GMFCS level V (18%) and was similar in distribution to a contemporaneous Swedish cohort. There was a fairly even distribution across the topographical distributions of hemiplegia (35%), diplegia (28%) and quadriplegia (37%) with a large majority of young people having the spastic motor type (86%).
Conclusions:   The VCPR is ideal for population-based studies of gross motor function in children with CP. Gross motor function is similar in populations of children with CP in developed countries but the comparison of motor types and topographical distribution is difficult because of lack of consensus with classification systems. Use of the GMFCS provides a valid and reproducible method for clinicians to describe gross motor function in children with CP using a universal language.     

Sleep quality and respiratory function in children with severe cerebral palsy using night-time postural equipment: a pilot study

Background:  Night-time postural equipment (NTPE) prevents contractures and hip subluxation in children with severe physical disabilities. However, impact on sleep quality and respiratory function has not been objectively studied.
Methods:  Ten children with severe cerebral palsy (CP), mean age of 10.9 (range: 5.3–16.7) years, were recruited from a community population. Polysomnography was undertaken on two nights, once with the child sleeping in their NTPE and once sleeping unsupported. Randomization to first night condition controlled for first night effects.
Results:  Night-time postural equipment use was associated with higher mean overnight oxygen saturation for three children but lower values for six children compared with sleeping unsupported. There were no differences in sleep quality between the conditions. The study group had lower overnight oxyhaemoglobin saturation values, less rapid eye movement (REM) sleep and higher arousal indices compared with typically developing children.
Conclusion:  This pilot study indicated that children with severe CP risk respiratory compromise in sleep irrespective of positioning. Further study will determine if the observed trend for mean overnight oxygen saturation to be lower within positioning equipment reflects random night-to-night variation or is related to equipment use. We suggest that respiratory function is assessed when determining optimal positioning for children using night-time positioning equipment.     

发育性髋关节脱位发病相关因素的研究进展

发育性髋关节脱位(developmental dislocation of the hip,DDH)是小儿骨科常见的疾病、严重危害儿童健康.髋关节发育不良包括髋臼发育不良、髋关节半脱位及全脱位等病理类型.导致该病发病的常见因素包括:阳性家族史、性别、臀位生产、不正确的襁褓方式和母体激素水平等.但是具体病因尚不明确.本文从DDH的发病率、DDH的危险因素、激素对DDH的影响、DDH的易感基因4个方面综述了DDH的研究现状,从而进一步探讨DDH的发病因素.     

Passive range of motion in a population-based sample of children with spastic cerebral palsy who walk

While passive range of motion (PROM) is commonly used to inform decisions on therapeutic management, knowledge of PROM of children with spastic cerebral palsy (CP) is limited. A population-based sample of 178 children with spastic CP (110 male; unilateral, n = 94; bilateral, n = 84; age range 4-17 years) and 68 typically developing children (24 male; age range 4-17 years) were recruited to the study. All children were able to walk a minimum of 10 m over a straight flat course, with or without assistive devices. Gross Motor Function Classification System (GMFCS) levels of participants with CP were: Level I = 55, Level II = 88, Level III = 21, and Level IV = 14. Ankle dorsiflexion, knee extension, popliteal angle, hip abduction, hip internal rotation, and hip external rotation were measured using a goniometer. The results indicate that the children with CP had significantly reduced PROM compared to the children with typical development. Children with CP demonstrated reduced length in the hamstrings, hip adductor, iliopsoas and gastrocnemius-soleus musculature, and contracture at the knee joint. Among children with CP, there were significant reductions in range with increasing functional limitation (higher GMFCS level) and variations based on unilateral or bilateral involvement. This was particularly the case for the hamstrings and hip adductor musculature, where PROM varied considerably across GMFCS Levels I to IV.     

Botulinum neurotoxin treatment in children with cerebral palsy: a population-based study in Norway

Background/aimTo study proportions and characteristics of children treated and un-treated with Botulinum neurotoxin (BoNT) in a population-based cohort of children with cerebral palsy (CP).MethodsAll children with CP born during 1999–2003, recorded in the Norwegian CP Register were included (N = 411). Gross motor function was assessed using the gross motor classification system (GMFCS).ResultsSixty-eight percent of children with bilateral spastic, 63% with unilateral spastic and 41% with dyskinetic CP had received BoNT. The percentage of children treated increased from 62% at GMFCS level I to 88% at level IV, but was only 38% among children at level V. A similar trend was seen for fine motor function. Ninety-four percent of the children received BoNT in their lower limbs. Children without significant cognitive impairment were more often treated than children with such impairment (OR: 2.61; 95% CI: 1.49–4.58).InterpretationIn this first population-based study, approximately 2/3 of all children with spastic CP were treated with BoNT. The results suggest preference for treatment of children with potential for functional improvement, while treatment to relieve pain and facilitate care, and of children with cognitive impairment appeared to be less common. Whether the latter groups are treated appropriately requires further studies.     

The hip in cerebral palsy

Cerebral Palsy (CP) is a common cause of progressive musculo–skeletal deformity and loss of function in children; with an incidence of around 3/1000 live births. The majority of even the most severely involved children survive to adulthood.Children with CP have complex needs and need to be regularly assessed by a multidisciplinary team in order to target appropriate interventions. These do not alter the underlying chronic condition but can provide improvement in function and comfort, and reduce the burden of care if considered within the context of the overall needs of the child.Hip displacement is seen in up to 60–80% of severely affected children. Hip surgery is therefore unsurprisingly the second most common orthopaedic intervention in children with CP. Surveillance of at risk populations is now well established.Setting functional goals and assessing outcomes of any intervention is necessary, with medical, anaesthetic, nursing and therapy components all contributing significantly to the positive outcome if surgery is deemed necessary. Any surgical intervention in a child with CP should always be a process rather than a discrete event.     

Developmental dysplasia of the hip: Risk markers, clinical screening and outcome

Background: Early detection, diagnosis and treatment of developmental dysplasia/dislocation of hip (DDH) are essential in preventing further disability and quality of life impairment in children. DDH risk markers and association between the age of clinical screening and outcome, were evaluated.
Methods: Clinical screening at ages birth, 6 and 13 weeks was performed in 8145 infants by pediatricians. Infants suspected for DDH were referred to the community hospital clinic for clinical evaluation by a pediatric orthopedic surgeon, imaging procedures and follow up. Demographic and perinatal characteristics of the children with suspected ( n = 77) and diagnosed DDH ( n = 51) were compared to matched controls ( n = 154).
Results: The rate of suspected DDH was 0.95% and that of diagnosed DDH was 0.63%. Female gender, firstborn child and breech presentation were significantly more frequent among cases versus controls (odds ratio [OR]: 4.3, 2.7, and 6 respectively; P < 0.05). The highest positive predictive value (95.5%) in physical evaluation was any evidence of a dislocatable hip. The proportion of DDH among infants referred from the newborn department was significantly higher (OR, 4.4). DDH diagnosis after 6 weeks of age was associated with a higher likelihood of subsequent surgery and motor disability. Untoward outcome was significantly associated with increasing age of referral both at ages of 6 and 13 weeks ( P < 0.05).
Conclusions: Children with DDH have certain specific demographic and perinatal risk markers. Clinical screening targeted towards early diagnosis may lessen the need for surgical intervention and the risk of disability or motor handicap.     

CONGENITAL DISLOCATION OF THE HIP IN NORWAY Late Diagnosis CDH in the Years 1970 to 1974

ABSTRACT. A survey of 274 late detected cases of CDH born in the years 1970–74 is presented. The incidence of late cases in southeast Norway was calculated to 2.2 per 1000 live births. The hips of all patients were examined at birth, the majority by trained pediatricians, without disclosing any hip affections. 86% were females. Only 6.9% were delivered in the breech position. One–half of the patients had luxation (20%) or subluxation (30%), the rest had dysplasia without dislocation. In 19% both hips were involved. The low incidence of breech presentations in late CDH-cases compared with the incidence among neonatal cases (15.7%) point to some etiological differences. It seems that we in Norway have two types of CDH, one caused by joint laxity and detectable at birth and another, not present at birth, with progressive dysplasia of the hip and dislocation developing during the first year of life.     

CPIPS: musculoskeletal and hip surveillance for children with cerebral palsy

CPIPS (Cerebral Palsy Integrated Pathway Scotland) provides access for all children with cerebral palsy (CP) aged between 2 and 16 years in Scotland for a standardized musculoskeletal examination of the spine and lower limbs by paediatric physiotherapists. Children with more severe CP are at risk of developing a displaced hip joint which can become painful and interfere with seating and daily activities. Therefore all children also receive regular X-rays of their hips based on their age and severity of CP. CPIPS has been universally accepted and approved by the children, parents, carers and clinicians alike. It provides useful data on epidemiology, hip subluxation, physiotherapy participation and orthotic use as well as much more. It is already producing a significant improvement in hip displacement rates in Scotland. CPIPS has also greatly improved communication between community based care and hospital services. It is now fully embedded in the day to day care of these vulnerable children in Scotland and is now deemed essential in improving and maintaining their musculoskeletal health.     

Psychosocial adjustment in a Dutch sample of children with cerebral palsy

BackgroundOver the last couple of years, there has been increasing interest for QoL in children with CP. Psychosocial adjustment in these children remains underrepresented in current literature.AimsTo describe psychosocial adjustment in children with CP by means of the Psychosocial Adjustment and Role Skills Scale III (PARS-III), to describe the psychometric properties of this questionnaire, to identify a cut-off score for psychosocial maladjustment and to investigate the relationship between patient characteristics (i.e. predictive factors) and psychosocial adjustment.MethodsThe parents of 93 children with CP (59 boys, 34 girls; mean age 12.3 years, SD 3.8; 4–18; GMFCS 1: 28, GMFCS 2: 5, GMFCS 3: 19, GMFCS 4: 18, GMFCS 5: 23) completed the PARS-III and the Child Behavior Checklist (CBCL) concerning the psychosocial and behavioral functioning of their child.ResultsCronbach’s alpha-coefficient for the PARS-III was 0.89 indicating good internal consistency. High correlation with the CBCL was found. Confirmatory factor analysis confirmed the 6 domain structure of the PARS-III. Overall, children with CP achieved lower psychosocial adjustment scores compared to healthy children. A cut-off score (1 SD below the mean) of 78 was found. When predicting psychosocial maladjustment in children with CP, less gross motor function, hand function, communication skills and bilateral involvement of CP are the most important factors, but these can only explain 36% of variation in psychosocial adjustment.ConclusionUsing the by-proxy version of the PARS-III it was found that children with CP are reported to achieve lower psychosocial adjustment scores than healthy children.     

Effect of upper limb botulinum toxin-A therapy on health-related quality of life in children with hemiplegic cerebral palsy

Aim:   Currently, the use of upper limb botulinum toxin-A (UL BTX-A) is based on evidence of functional efficacy without supporting evidence of positive change in health-related quality of life (HRQOL). While function may improve, this cannot be directly correlated with an improvement in HRQOL. Most paediatric studies use caregiver/parent proxy reports. The inclusion of child self-reports is increasing as poor correlation with proxy reports is being demonstrated. This paper aims to study the effect of UL BTX-A therapy on HRQOL in children with hemiplegic cerebral palsy (CP).
Method:   Design: Pilot prospective randomised trial. Participants: 22 children with hemiplegic CP aged 7 years 0 month−13 years 11 months (12 treatment, 10 control). Treatment: One series BTX-A injections into UL. HRQOL assessed at baseline, and 1, 3 and 6 months post-injection by completion of Pediatric Quality of Life (PedsQL) 4.0 Generic Core Scales and PedsQL 3.0 CP Module. Outcome: 1. Change in PedsQL scores. 2. Concordance between child self-report and parent proxy-report scores.
Results:   No statistically significant difference between treatment and control groups was observed for any domain of HRQOL. Intraclass concordance was good for the PedsQL CP Module Daily Activities, and Speech and Communication scores ( P  = 0.0005).
Conclusion:   This pilot work adds to the emerging evidence that UL BTX-A therapy has no statistically significant effect on the HRQOL of children with hemiplegic CP. With the increasing use of this therapy in children with CP, further research across the broader CP population is needed to identify whether this therapy is indicated in other target populations. Both child and parent proxy reports should be collected when assessing HRQOL in this population.     

Immunisation practices in infants born prematurely: Neonatologists' survey and clinical audit

Aim:   To determine Australian neonatologists' recommendations for the immunisation of ex-preterm infants and compare their actual immunisation status with recommended Australian guidelines.
Methods:   A self-administered nine-part questionnaire of current immunisation practices was sent to all Neonatologists in Australia (2006). A complementary retrospective immunisation audit was conducted in two tertiary neonatal units in Melbourne. Hospital records and the Australian Childhood Immunisation Register (ACIR) were reviewed; consenting parents were interviewed and primary care physicians' vaccination records were requested. A random sample of preterm infants born between July 2003 and June 2005 at <32 weeks' gestation were selected.
Results:   (i) Neonatologists Survey: The response rate was 68% and the majority of neonatologists (89%) were aware of the current guidelines, but adherence to them varied from 43% to 79%. One-fifth of neonatologists personally do not receive annual influenza vaccination; and (ii) Immunisation Audit: Conducted between October 2006-May 2007 it included: 100 hospital records; 97 ACIR records; 47 parent interviews and 43 primary care vaccination records. Overall vaccination coverage was 90% at 12 months of age. Only 20% (10/50) of infants with chronic lung disease received an influenza vaccination. Vaccines were delayed by greater than one month in 15% of participants for the 2 month DTPa vaccine and 43% at 6 months.
Conclusions:   The neonatologists survey highlighted variable adherence with immunisation guidelines. The audit confirmed preterm infants are frequently experiencing delayed vaccination and recommended additional vaccinations are often not being received. Formulation of strategies to ensure complete and timely immunisation are required, including better utilisation of the ACIR.     

Acute septic arthritis in children

Background:  Acute septic arthritis is a health-care problem in growing children. The aim of the present study was to determine the demographic features, clinical presentation, diagnostic evaluation, treatment and outcome of children suffering from acute septic arthritis.
Methods:  Retrospective review of consecutive admissions of children ≤14 years of age with a bacteriologically and/or radiologically confirmed diagnosis of septic arthritis during the 10 year period, January 1997–December 2006 at King Fahad Hospital, King Abdulaziz Medical City was undertaken.
Results:  Sixty-five patients (male : female, 2.4:1), 10 days–14 years of age (mean ± SD: 3.3 ± 3.2 years), met the inclusion criteria. More than half of the patients were under 2 years. The most frequent clinical features were pain (92%), fever (77%), limitation of joint movement (77%) and joint swelling (72%). Joint involvement was monoarticular in 97% of knees, and hips were affected in 75% of patients. Bacteria were isolated from joint aspirate or blood in 28 patients (43%), and Staphylococcus aureus was the most common organism, comprising 39% of the total bacterial isolates. Radiological diagnostic findings were observed in 19 (32%) of 60 patients with plain radiographs, nine (69%) of 16 patients with sonograms of the hip, 36 (80%) of 45 patients with Tc-labeled bone scan and nine (100%) of nine patients with magnetic resonance imaging. The joint infection resolved with no sequelae in 46 patients who were followed up for 6–36 months, but two had bone deformity and 17 were lost to follow up.
Conclusion:  According to the present series, septic arthritis is most common in young infants, mainly monoarticular, and is frequently localized in the knee and hip joints. Early diagnosis and appropriate treatment are associated with excellent outcome.     

中文版脑瘫儿童粗大运动功能分级系统的信度和效度研究

目的 确定中文版脑瘫儿童粗大运动功能分级系统（Gross Motor Function Classification System ,GMFCS）的信度和效度。方法 共有来自上海三家康复机构的91名0-12岁脑瘫儿童参加了此项研究，选择35名脑瘫儿童测定GMFCS的重测信度；以66名脑瘫儿童为对象测定GMFCS的评价者间信度；分别以88名脑瘫儿童的粗大运动功能评估量表（GMFM）和54例脑瘫儿童的Peabody粗大运动发育量表（PDMS-GM）的各项测试结果为效标确定GMFCS的平行效度；对88例同时接受GMFCS和GMFM评价的儿童的测试结果进行结构效度检测，以GMFCS为应变量，GMFM五个功能区的百分比为自变量进行多重逐步回归分析，判断粗大运动中五个分区功能对GMFCS的影响程度。结果 GMFCS具有良好的重测信度（ICC值为0.99），同时具有良好的评估者间信度（ICC为0.95-0.98）；GMFCS与GMFM和PDMS-GM各项分值之间有良好的平行效度，Spearman相关系数在-0.57到-0.84在之间；粗大运动功能中的坐位能力和行走能力是影响GMFCS的主要因素，校正决定系数为0.709（p<0.001）。 结论 中文版脑瘫儿童粗大运动功能分级系统的具有良好的信度和效度，适用于国内对脑瘫儿童进行功能分级。     

Predictors (0-10 months) of psychopathology at age 11/2 years - a general population study in The Copenhagen Child Cohort CCC 2000

Background:  Epidemiological studies of mental health problems in the first years of life are few. This study aims to investigate infancy predictors of psychopathology in the second year of life.
Methods:  A random general population sample of 210 children from the Copenhagen Child Birth Cohort CCC 2000 was investigated by data from National Danish registers and data collected prospectively from birth in a general child health surveillance programme. Mental health outcome at 1½ years was assessed by clinical and standardised measures including the Child Behavior Check List 1½–5 (CBCL 1½–5), Infant Toddler Symptom Check List (ITSCL), Checklist for Autism in Toddlers (CHAT), Bayley Scales of Infant Development (BSID II), Mannheim Eltern Interview (MEI), Parent Child Early Relational Assessment (PC ERA) and Parent Infant Relationship Global Assessment Scale (PIR-GAS), and disordered children were diagnosed according to the International Classification of Diseases (ICD-10) and Diagnostic Classification Zero to Three (DC: 0–3).
Results:  Deviant language development in the first 10 months of life predicted the child having any disorder at 1½ years, OR 3.3 (1.4–8.0). Neuro-developmental disorders were predicted by deviant neuro-cognitive functioning, OR 6.8 (2.2–21.4), deviant language development, OR 5.9 (1.9–18.7) and impaired social interaction and communication, OR 3.8 (1.3–11.4). Unwanted pregnancy and parents' negative expectations of the child recorded in the first months of the child's life were significant predictors of relationship disturbances at 1½ years.
Conclusions:  Predictors of neuro-developmental disorders and parent–child relationship disturbances can be identified in the first 10 months of life in children from the general population.     

三维计算机X线断层照像术测量股骨颈前倾角在发育性髋关节脱位治疗中的应用

目的探讨三维CT测量股骨颈前倾角(FNA)在小儿发育性髋关节脱位(DDH)治疗中的应用价值。方法DDH患儿12例,其中双髋脱位4例,单髋脱位8例,均行闭合复位、蛙式石膏外固定术,采用GE公司Hip speed Fi/x双层螺旋CT进行表面遮盖成像、最大密度投影、多平面重建等三维观察和FNA测量,分别在术前对股骨头颈、髋臼及臼头关系行三维重建。结果髋脱侧FNA明显增大,与其同年龄段患儿正常髋关节侧比较差异有显著性(P<0.05)。结论三维CT可直观、全面及分解地显示髋关节结构,更加精确地测量FNA,从而达到对DDH患儿个体化治疗的目的。     

Fire‐related child deaths: What do newspaper case reports tell us?

Objective:   To describe the accuracy and public health relevance of newspaper accounts of child deaths from fire-related incidents.
Methods:   Domestic fire-related deaths of children aged under 15 years in Auckland, New Zealand, over a 10-year period were retrospectively identified from fire service records and the national minimum mortality dataset. Forensic pathology and fire service records were reviewed and this information was compared with reports published within 3 days of the index event in the region's sole daily newspaper.
Results:   All 14 fatal fire-related events (19 deaths) identified using fire service records and the national minimum dataset during the study period were reported in the newspaper with a high degree of detail and accuracy. Only four news items informed readers of specific measures that could prevent such events.
Conclusions:   Daily newspapers can provide reliable, useful and timely surveillance data on the incidence of fire-related childhood deaths. However, these reports often represented missed opportunities to disseminate public health messages that raised awareness of sources of risk and means of preventing fire-related deaths.     

Screening for hip abnormality in the neonate

Clinical screening policies for the detection of hip instability or dysplasia of the hip vary internationally. There is general agreement in the Western world that at birth all hip joints should be clinically assessed by the Ortolani and Barlow tests. Currently, there is no consistency regarding who should undertake the examination, the results being worse when inexperienced personnel are used. These clinical tests have poor sensitivity and should be regarded as surveillance, not screening methods. Since the 1980s ultrasonographic assessment of the hip has become a valuable diagnostic tool. However there is continuing controversy on whether this imaging method should be used universally or selectively for 'at risk' and clinically unstable hip joints. Universal ultrasonographic evaluation may result in over-treatment and selective screening may be no better than the best clinical screening programs in reducing the incidence of 'late' irreducible dislocation of the hip. It is generally accepted that all clinically unstable hips should be imaged by ultrasound by static and dynamic methods in order to confirm the diagnosis and to monitor treatment.