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Table of Contents
ORIGINAL ARTICLE
Year : 2020  |  Volume : 2  |  Issue : 2  |  Page : 106-112

Prevalence of limb deformities among children having cerebral palsy in Jalandhar District of Punjab


1 Department of Physiotherapy, Lyallpur Khalsa College, Jalandhar, Punjab, India
2 Department of Physiotherapy, Punjabi University, Patiala, Punjab, India

Date of Submission12-Jan-2020
Date of Decision14-Mar-2020
Date of Acceptance07-May-2020
Date of Web Publication04-Jan-2021

Correspondence Address:
Dr. Raju Sharma
Lyallpur Khalsa College, Jalandhar, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijptr.ijptr_78_19

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  Abstract 


Introduction: Cerebral palsy (CP) is a nonprogressive disorder of the developing brain. The newborn child with CP usually has no deformities or musculoskeletal abnormalities at birth. However, the distribution and prevalence of deformities in CP is a less researched area. Most of the studies have dealt with the correction of deformities. The purpose of this study is to determine the prevalence of deformities in CP and explore the relationship between their impairments and function.
Methodology: A database of 248 children with CP between the age group of 3 and 13 years in a survey conducted in Jalandhar District of Punjab constituted the population of this study. Each child was physically examined by the researcher to assess primary and secondary impairments using standard clinical tests, such as Gross Motor Function Measure (GMFM-88), Quality of Upper Extremity Skill Test (QUEST), Gross Motor Function Classification System (GMFCS), and passive range of motion. Cross tabulation with Chi-square and correlation was a tool of statistical analysis for categorical variables, whereas t-test was used for continuous variables.
Results: Equinus deformity of the ankle was most common followed by knee flexion, forearm pronation, and wrist flexion deformities. Deformity score showed statistically significant negative correlation with GMFM score (r = −0.43, P < 0.001) and QUEST score (r = −0.42, P < 0.01) and positive correlation with GMFCS levels (r = 0.42, P < 0.001) and all domains of self-care.
Conclusions: Occurrence of deformities is the most common secondary impairment observed in children with CP. Deformities limit gross as well as the fine motor activities and hence the overall functional outcome in these children.

Keywords: Cerebral Palsy, Deformity, Prevalence


How to cite this article:
Sharma R, Sinha AG. Prevalence of limb deformities among children having cerebral palsy in Jalandhar District of Punjab. Indian J Phys Ther Res 2020;2:106-12

How to cite this URL:
Sharma R, Sinha AG. Prevalence of limb deformities among children having cerebral palsy in Jalandhar District of Punjab. Indian J Phys Ther Res [serial online] 2020 [cited 2021 Oct 18];2:106-12. Available from: https://www.ijptr.org/text.asp?2020/2/2/106/190046




  Introduction Top


Cerebral palsy (CP) is a nonprogressive disorder of developing brain. It is a disorder of posture, movement, and tone due to a static encephalopathy acquired during brain growth in fetal life, infancy, or early childhood.[1] Children with CP exhibit a wide variety of symptoms including lack of muscle coordination when performing voluntary movements (ataxia), stiff muscles and exaggerated reflexes, walking with one foot or leg dragging, walking on the toe, excessive drooling or difficulties in swallowing and shaking, or random involuntary movements.[2],[3]

Cerebral lesion of CP is static, but the musculoskeletal status runs a progressive course. The newborn child with CP usually has no deformities or musculoskeletal abnormalities at birth.[4] However, over the course of development, the child acquires deformities of limbs and spine. A key feature of the musculoskeletal pathology is a failure of longitudinal growth of skeletal muscle.[5]

The different patterns of joint involvement are described in CP. However, the distribution and prevalence of deformities in CP is a less researched area. Most of the studies have dealt with the correction of deformities. While it is agreed that deformities affect the abilities and functioning of child, no quantitative data are available that link deformity status with functioning and abilities. Keeping this in mind, the present study was aimed at determining the prevalence of limb deformities in CP and explore the relationship of deformity scores with primary impairment, ability, functioning and participation restriction, and physiotherapy service utilization.


  Methodology Top


A cross-sectional survey in two phases on the family members of 248 children with CP (males = 159 and females = 89) was conducted in the Jalandhar district of Punjab to study the epidemiology of disability in children with CP. In the first phase (2008–2011) of the survey, a database of children with CP of age group 3–13 years (mean age 7.95 ± 3.33) of Jalandhar district of Punjab was prepared by the investigator, which constituted the population of this study. The detail of preparation of the database has been described previously.[6] Each identified child was physically examined and was retained in the database only if the following criteria of inclusion were satisfied: (a) diagnosed cases of CP (all types) and/or; (b) presence of motor delay and motor disorder, abnormal muscle tone, abnormal posture, or asymmetry and persistence of primitive reflexes;[7] (c) have completed the age of 3–13 years on March 2008; and (d) resident of Jalandhar.

In the second phase, each child was physically examined to assess gross motor abilities, fine motor function, the severity of condition, and secondary impairment using a clinical test battery. Information on the existing comorbidity, service utilization (physiotherapy, occupational, orthoses, surgeries, etc.), beliefs, concern, constraints, and demography was obtained by a scheduled interview of parents. The clinical test battery consisted of Gross Motor Function Measure (GMFM),[8],[9] Quality of Upper Extremity Skill Test (QUEST),[10],[11] Gross Motor Function Classification System (GMFCS),[12],[13] and passive range of motion (PROM) measurement (International Classification of Functioning b700).[14]

GMFM is a widely used clinical observation tool that was developed and validated for children with CP or Down syndrome. It measures gross motor function in lying and rolling, crawling and kneeling, sitting, standing, and walk-run-jump activities. GMFM has high level of intra-observer reliability and inter-observer reliability.[15]

QUEST is an impairment-based measure that includes items specifically related to hand function but also assesses movements of the adjacent joints and activities such as picking up a piece of cereal, a pencil, and a cube. The test battery consists of 36 items under these four domains of dissociated movements, grasps, weight bearing, and protective extension. High test–retest, inter-observer, and intra-observer reliability of QUEST has been reported.[11]

The GMFCS has been widely adopted as the prime method for describing the severity of the motor disability in children with CP in both research and clinical contexts.[13] GMFCS describes the locomotor ability of children with CP in one of the following five ordinal levels.[16] The general description for different levels is as follows: Level I – walks without limitations, Level II – walks with limitations, Level III – walks with handheld mobility device, Level IV – self mobility with limitations may use powered mobility, and Level V – transported in manual wheelchair.

A PROM was assessed with a handheld goniometer.[17] Each joint was moved gently to avoid stretch reflex and taken beyond the first catch up to the second limitation. The help of parents was taken to stabilize the limb to prevent any compensatory movement. All angles were measured to the nearest 5°. All PROM measurements were done in supine position with exception to forearm supination and wrist extension, and rotation of hip, which were measured in sitting and prone lying. For hip extension PROM (flexion contracture), the pelvis was stabilized and the contralateral hip and knee were flexed up to the chest to measure the angle between the table and the leg. Internal and external rotation was tested in prone with knee flexed to 90° of flexion. Knee extension limitation was measured with tested leg hip at 90° and contralateral hip in extension. Ankle dorsiflexion and plantar flexion were measured with the knee extended. Foot inversion and eversion were measured with hindfoot stabilized with knee extension. Inability to extend Metacarpophalangeal (MCP) joint and interphalangeal (IP) joint and abduction past the index finger was used to evaluate limitations in PROM for thumb. Inability to extend MCP and IP joints when wrist in neutral (0°) was considered as the limitation of PROM for fingers.

Inter- and intra-rater error of measures of PROM in children with CP has been reported as 6°–18°.[18] Therefore, for determination of deformity status, passive joint range of motion (JROM) measurement was compared with normative values, and value outside 2SD of normal distribution[19] was considered as deviation and a joint was considered as deformed if deviation was observed in any degree of freedom. Inability to move thumb past the index finger was construed as deformity of the thumb, Whereas for hand, inability to extend MCP and IP joints completely when wrist is in neutral (0°) was construed as deformed hand.

Data analysis

Data were analyzed using SPSS version 16.0 for Windows by Microsoft Corp., NY, USA. The number of joints having range of motion (ROM) limitation was recorded and transformed into a total deformity score, according to Ostensjø et al.,[20] giving a possible score of 0–20. Descriptive statistics were prepared. Correlation and cross tabulation with Chi-square and t-test were used to analyze categorical and continuous variables, respectively. t-test and one-way ANOVA were the tools of analysis with statistical significance set at P < 0.05.


  Results Top


[Figure 1] presents the distribution of joint-wise deformities. In limbs, bilateral joint involvement was more prevalent. Deformities of ankle (66.1%) was most common followed by knee (58.5%), foot (36.7%), and hip (32.3%) in lower limbs, whereas in upper limbs, forearm (48%) was common followed by wrist joint (46%), elbow (34.3%), hand (23.0%), thumb (22.2%), and shoulder (21.4%).
Figure 1: Prevalence of joint deformities in children with cerebral palsy

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The distribution of deformities according to the type of CP is presented in [Table 1]. The highest prevalence of deformities of both upper and lower limbs was seen in children with quadriplegia. In comparison with diplegia, children with hemiplegia showed a higher proportion of upper limb involvement than in lower limb. Ankle and foot deformities were equally distributed across the categories of topographical classification. Nearly 60%–70% of children in all topographical categories had ankle deformities, whereas foot deformities were reported in about 40% of children. In dyskinetic type, deformity of ankle (48.3%) was most common followed by wrist (48.3%); knee (44.8%); elbow and forearm (37.9% each); foot (31.0%); hip (24.1%); and shoulder, hand, and thumb (20.7% each). In ataxic type, only knee, ankle, and foot were deformed in 40% of patients. In mixed type, ankle and foot (57.1%) were most commonly deformed followed by wrist (28.6%) and forearm and knee (14.3% each).
Table 1: Distribution of joint-wise deformities according to the subtypes of cerebral palsy

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[Table 2] presents the distribution of deformities according to GMFCS levels of the patients. Level V had the highest proportions of deformities of all the joints, with more than 80% of Level V children demonstrating deformities of shoulder (94.3%), hand (93.0%), thumb (92.7%), hip (82.5%), and elbow (82.4%). In Level V, the minimum (54.9%) distribution of deformities was reported in foot. In Level I children, the deformities of foot were most common (13.2%), followed by wrist (10.5%), ankle (9.8%), forearm (5.9%), knee (4.8%), hip (2.5%), hand (1.8%), and thumb (1.8%). No deformity of shoulder was reported in Level I, II, and III children.
Table 2: Association between gross motor function classification system levels and joint involvement in children having cerebral palsy

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[Figure 2] presents the distribution of patients according to deformity scores. Twelve children (4.8%) had no deformity of any joint. The majority of children(46%) had deformities of 1–5 joints, followed by 6–10 joints (29.0%), 15–20 joints (17.3%), and 11–15 joints (2.8%).
Figure 2: Distribution of patients according to their deformity score

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[Table 3] presents the comparison of deformity scores among various clinical and demographic categories. The mean deformity score of the cohort was 7.10 ± 6.08, (median 5; mode 2). Comparison of deformity scores of categories of type of CP, gender, and socioeconomic status did not reveal statistically significant difference though significant differences in deformity scores were observed across the categories of topography, mental retardation, and GMFCS levels. Locality, exposure to physiotherapy, and advice for rehabilitation also significantly influenced the deformity score. The mean deformity scores of quadriplegia (9.58 ± 6.85) were significantly different from that of diplegia (5.52 ± 5.02), triplegia (4.89 ± 6.85), and hemiplegia (3.59 ± 2.27). A comparison of deformity score among GMFCS levels was very highly significant (F = 14.81). Deformity score of Level V was significantly higher than other levels, but difference between other levels was not significant.
Table 3: Comparison of deformity score according to various clinical and demographic variables

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Significant positive but weak correlation of deformity score was observed with chronological age (r = 0.24, P < 0.001) and age of diagnosis (r = 0.18, P < 0.01). Deformity score showed statistically significant negative correlation with GMFM score (r = −0.43, P < 0.001) and QUEST score (r = −0.42, P < 0.01) and significant positive correlation GMFCS levels (r = 0.42, P < 0.001) and with all the five domains of self-care [Table 4].
Table 4: Correlation of deformity score with age, motor abilities, and functional independence

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  Discussion Top


The mean deformity score of the study population was 7.10 ± 6.08. The distribution of the deformity score was statistically significant across different topographical categories. On an average, patients with quadriplegia had involvements of nine joints in comparison of three joints' involvements in hemiplegia and patients with diplegia had five joints' involvements.

Limitation of JROM is the most common secondary impairment observed in CP. Joint range limitation in CP population is the result of tissue adaptations associated with immobilization, disuse and excessive use, or unfavorable biomechanical load.[21],[22] Muscle hypoextensibility associated with reduction of movement may produce changes in the joint capsule and other connective tissues. Contractures, muscle atrophy, and changes in muscle architecture are the consequences of these adaptations. Another feature associated with the development of deformity in CP is failure of longitudinal growth of skeletal muscle. Interaction among reduced amount and variability of movement pattern imbalance in musculoskeletal growth and postural asymmetry may also contribute to deformation of bone.[23]

Studies reporting the prevalence of joint-wise deformities in CP are limited. In one such study of 234 children with CP, Park et al. reported that limitation of forearm supination was the most common upper limb deformity in children with spastic CP. Nearly 70.5% of children of their study had a limitation in forearm supination and 62.8% had problems with wrist and finger extension in at least one limb. Thumb-in-palm deformity of at least one hand was found in 47.0% of patients.[24] In the present study, a high prevalence of deformities was observed. Deformities of ankle (66.1%) were most common followed by those of knee (58.5%), forearm (48.0%), and wrist (45.6%). The prevalence of hip, shoulder, and elbow joint deformities was 32.3%, 21.4%, and 34.3%, respectively. Deformity of thumb was observed in 22.2% of children and 23.0% of children were unable to extend their fingers completely. The present report is in accordance with the findings of a Swedish study[25] and a European study,[26] where they reported that all patients had developed deformities of the lower limbs and 65.6% had deformities of the upper limbs. Margre et al. also reported similar results.[27] In contrast, the findings of a study conducted in the Netherlands[28] were in variance with our findings where overall 76.5% had no ROM deficits of the upper extremities and similarly, 48.7% of children had no lower extremity ROM deficits.

In the present study, 95.2% of children had limitations in passive JROM in one or more joints of upper and lower limbs. Twelve children (4.8%) had no limitation of JROM in any joint, whereas eight children (3.2%) had deformity of only one joint. Rest 92% had deformity of two and more joints. Nineteen children (7.7%) had ROM limitation in all the twenty joints. Involvement of two joints was the most frequent presentation observed in 20.2% of children.

Topography also influenced the distribution of joint-wise deformity. The children with quadriplegia had the highest prevalence of deformities of both upper and lower limbs. In comparison with diplegia, children with hemiplegia showed a higher proportion of upper limb involvement though with opposite was seen in lower limb proportion of upper limb involvement though with opposite was seen in lower limb of topographical classification. Nearly 60% -70% of children among all topographical categories had ankle deformities. Ankle and foot deformities were equally distributed across the categories, whereas foot deformities affected about 40% of children.

In spastic and dyskinetic type, deformities of all joints were seen. However, in ataxic and mixed types, deformities of hip, shoulder, elbow, hand, and thumb were not seen. Deformity of ankle was the most prevalence deformity in all the four types. The spasticity causes difficulty in movement, abnormal posture in sitting and standing, contractures leading to deformities, pressure sores, and pain. Spasticity affects muscle growth, and bone growth is distorted by the abnormal resistance of shortened muscles. If spasticity is not managed properly at an early stage, bone deformities occur.[29],[30]

Children with mental retardation (MR) had relatively more deformed joints so was the case with rural children. Deformity scores did not show any significant difference according to socioeconomic status of the family or gender of child. The utilization of physiotherapy service and advice for rehabilitation significantly influenced the deformity score.

The deformity score significantly influenced functional status. Deformity score was negatively correlated with gross motor function (GMFM) and fine motor abilities (QUEST). A highly significant positive correlation was observed between deformity scores and the ambulatory status as well as difficulty in self-care activities. Nonambulatory children had significantly higher deformity score and a relatively higher proportion of deformities of all joints.

The present study was delimited to one district of Punjab and retrospective in nature. Data were gathered using clinical examinations and schedule interviews only and no investigations were carried out. The development of deformities during the growth period affects the functional improvement in these children and outcome of management. The occurrence of secondary impairments is the result of interactions of multiple factors in these children, which should be explored in future prospective study design.


  Conclusions Top


The JROM limitation is a common secondary problem among children having CP. It gets worse with advancing age, lack of advice, and treatment. Its impact is reflected in poor gross motor, fine motor, and self-care domains of children. Therefore, prospective studies should be conducted to understand the phenomenon of deformity development in these children and to plan preventive strategies.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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  [Table 1], [Table 2], [Table 3], [Table 4]



 

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