Abstract
Background
Radial club hand (RCH) is characterized by a wide array of hand and forearm anomalies. Various treatment approaches have been described depending upon the stages of RCH. The major drawback of these studies is that the effectiveness of these interventions was reported on clinical and radiological outcomes. With the increasing focus on patient-centered care nowadays, we wanted to identify the components associated with functioning and evaluate the effectiveness of two surgical procedures on functional outcomes using the International Classification of Functioning, Disability and Health (ICF)-based tools.
Materials and Methods
We identified 14 children from our records (nine boys, five girls) with a mean age of 5.6 years, classified as Bayne types III–IV and classified them into two groups; those who were operated by centralization (group 1) and ulnarization of the carpus with ulnar shortening osteotomy procedure (group 2). The outcomes were evaluated by the brief ICF core set for the child and youth with cerebral palsy up to the age of 5 and the brief ICF core set for hand conditions for a period of 1 year after surgery.
Results
The results showed that both the operative techniques showed improvement in the structure component (s730-structure of upper extremity). ICF categories of d445-hand and arm use, d530-toileting, and d880-engagement in play showed a change in frequencies of more than 40% after surgery and were maintained till follow-up. However, categories related to muscle power functions (b730), muscle tone (b735), fine hand use (d440), hand and arm use (d445) and engagement in play (d880) showed no significant improvement (p > 0.05). There were no differences between both the surgical procedures in improving the outcomes (p > 0.05).
Conclusion
We conclude that surgical techniques may be more appropriate to improve the cosmetic or structural appearance of the upper extremity than functioning.
Keywords: Congenital hand deformities; Disabled children; International Classification of Functioning, Disability, and Health; Operative; Outcome; Rehabilitation; Surgical procedures; Treatment outcome
Introduction
Radial club hand [RCH], also called as radial longitudinal deficiency, is a rare congenital deformity with an estimated incidence of 1 in 30,000 to 1 in 100,000 live births [1]. RCH is characterized by a wide array of hand and forearm anomalies characterized by radial hypoplasia of bones, joints, muscles, tendons, ligaments, nerves, and vasculature on the radial aspect of the forearm [2]. RCH affects both the sexes equally and occurs bilaterally 50% of the time [3]. RCH is associated either with a number of congenital syndromes or occurs sporadically with no etiology. These children with RCH suffer from functional impairment due to thumb dysfunction, shortness of upper extremities and wrist instability [3].
For clinical purposes, RCH is classified as per the different deformities observed in the radius alone [4] or those that include anomalies of the thumb and carpal bones [5]. The types III and IV as per the Bayne and Klug classification represent the cases of RCH deformity that is characterized by either partial or complete absence of the radius. These two forms are considered to be the most common [6]. The treatment of RCH depends upon the severity of the deformity. Type I is treated by stretching and splinting. Any surgical attempts on RCH focus on creating a centralized wrist to improve the function and appearance of the upper limb [4]. The various surgical procedures currently employed for managing hands with RCH include preliminary soft tissue distraction and tendon transfer with or without ulnar osteotomy [7, 8], surgical centralization of the wrist [9, 10], use of external fixators [11], proximal fibular transplants, arthrodesis [12] and recently ulnarization of the carpus with ulnar shortening osteotomy [13].
Centralization of the wrist is considered as the benchmark for the current management of this condition [14]. Despite the popularity of centralization as the most preferred surgical procedure for the management of RCH, several studies have shown recurrence of wrist radial deviation with long-term follow-up [3, 15]. To counteract the drawbacks of centralization procedure, Paley [16] advocated the ulnarization procedure as an alternative. This technique resulted in ulnar deviation of wrist due to gravity on the longer term. Recently, the same author put forward a revised technique called as ulnarization of the carpus with ulnar shortening osteotomy [13] to nullify the wrist ulnar deviation and has reported satisfactory results on a 2-year follow-up.
The studies that had studied the effectiveness of surgery in patients with RCH had evaluated the results based on clinical outcomes in terms of the range of motion and radiological assessment [4, 17–19] and not on functional activities that the patients’ consider as useful and meaningful. The treatment is focused on person-centered care nowadays [20], implying that health care professionals understand functioning and disability from the perspective of the patient.
Functioning is an umbrella term that encompasses body functions, body structures, and activities and participation. Hence, functioning should be considered as the main outcome measure in evaluating the effectiveness of surgical procedures. The International Classification of Functioning, Disability, and Health [ICF] are a biopsychosocial framework that is used to describe functioning in persons with disabilities [21]. The ICF describes functioning via different components which shows the interactions between the person, their ability and the environment [22]. The ICF core sets have been developed that serve as the reference standards for measuring functioning. Though these core sets were developed as a framework and classification system, studies have shown that ICF checklists and qualifiers indicate the changes in patients’ functional profiles over time and hence, ICF can be used to directly measure human functioning when coded as per established coding guidelines [23–25].
Ekblom et al. [26] investigated the relation between a short and bowed forearm, radial deviation of the wrist, a non-functional or absent thumb, limited range of motion in the fingers, impaired grip strength and activity and participation in children with radial dysplasia using the International Classification of Functioning and Health, version for Children and Youth [ICF-CY]. The results of the study showed that total range of motion of wrist and digits is of more importance than the radial dysplasia of the wrist in determining functional outcomes and manual performances. Another study by the same investigators [27] concluded that grip strength, key pinch, forearm length, elbow, and digital motion are more important for activity and participation than the radial angulation of the wrist in adults with RCH. These studies did not compare the effectiveness of surgical procedures on functional outcomes by comparing pre scores to post scores, and hence it is not fully known about the effectiveness of surgical procedures on functioning.
Other studies that had evaluated the effectiveness of surgical procedures on functional outcomes in children with RCH have shown mixed results. A study by Kotwal et al. [28] showed that hand alignment, finger and wrist range of motion and grip strength are better in children treated surgically compared to those who are conservatively managed. The results of Buffart et al. study [29] showed that children with RCH had good functional activities and the relationships between impairments in hand function and limitation of activities were not linear when the functions were measured by Assisting Hand Assessment and the Prosthetic Upper Extremity Functional Index. These results are in contradiction to the results of Goldfarb et al. study [16] which showed that hand function remained markedly abnormal after centralization procedure. The study also noted that improvement in wrist alignment did not correlate with improved upper-extremity function, as measured by the Jebsen–Taylor hand test and the Disabilities of the Arm, Shoulder, and Hand questionnaire. The difference in all these studies may be attributed to the different outcome measures employed in these studies. The purpose of this study is to report the changes in functioning over a 1-year period using the ICF in children who were operated for RCH using a pre–post design and to analyze if one surgical procedure is effective than other (centralization and ulnarization of carpus with ulnar osteotomy procedures) in improving functional outcomes.
Materials and Methods
The institute is a tertiary-level multi-disciplinary rehabilitation setup, offering outpatient and inpatient rehabilitation services for the persons with locomotor disability. We considered the medical records of children with RCH who underwent surgery (centralization and ulnarization of carpus with ulnar osteotomy procedures), and fulfilled our criteria, between the years of 2015 and 2018 in this report. The inclusion criteria were children aged between 6 months and 11 years and having unilateral or bilateral Bayne type III–IV RCH; children who underwent selective first-time centralization and ulnarization of carpus with ulnar osteotomy procedures; evaluation of functioning using the ICF core set for child and youth with cerebral palsy up to the age of 5 and the brief ICF core set for hand conditions. Exclusion criteria were: prior surgery for the management of RCH; children who have radial club hands of Bayne type I and II; older children who have RCH with good function; children who suffer from RCH that is attributed to other reasons [e.g., arthrogryposis, post-traumatic radial club hand]; children who have RCH with elbow extension contracture who rely on radial deviation; children with multiple congenital anomalies not compatible with life.
Nineteen children [24 hands] were identified during this period fulfilling the inclusion and exclusion criteria. We purposively classified the children who received centralization or ulnarization of the carpus with ulnar shortening osteotomy procedure as groups 1 and 2, respectively. Ten children [13 hands] were operated by centralization procedure [group 1] and nine children [11 hands] by ulnarization of the carpus with ulnar shortening osteotomy [group 2]. Three children in group 1 and 2 children in group 2 were lost on final follow-up. The data for 14 children [9 boys and 5 girls (7 children and 9 hands in each group); mean age of 5.6 years (range 2 years to 9 years)] were recorded for analysis.
Surgical Procedure
The children in group 1 were operated by centralization procedure. The centralization procedure was conducted as per the recommendations of Manske et al. [10]. This procedure was accompanied by the release of soft tissues on the radial side and transfer of flexor and extensor carpi radialis tendons to the ulnar side where indicated. Two children required carpectomy and distal ulnar excision. The surgical correction was maintained by a longitudinal pin in a long arm cast for 6–8 weeks. After the removal of pin around 8–12 weeks, the forearm and hand were placed in the appropriate splint as per the child’s comfort and therapy was initiated.
The children in group 2 (ulnarization of the carpus with ulnar shortening osteotomy) were operated as per the guidelines of Paley [13]. Active elbow and finger exercises were initiated after the surgery with the hand in elevation. The hand was maintained in splint thereafter and the wires were removed after 12 weeks. Wrist mobilization was initiated thereafter.
Outcome Measure
The institute had a comprehensive written manual to ensure a standardized approach to ICF application. The most appropriate core sets that were considered appropriate for this population are the combined brief ICF core set for child and youth with cerebral palsy up to the age of 5 and the brief ICF core set for hand conditions (Table 1). Activities and participation was rated from the perspective of performance only considering the objectives of the study and the age and functioning level of the children at the time of study. The environmental factors [facilitators and barriers] were not evaluated too.
Table 1.
ICF core set used for evaluation
| Body structures | |
| s720 | Structure of shoulder region |
| s730 | Structure of upper extremity |
| Body functions | |
| b265 | Touch function |
| b270 | Sensory functions related to temperature and other stimuli |
| b280 | Sensation of pain |
| b710 | Mobility of joint functions |
| b715 | Stability of joint functions |
| b730 | Muscle power functions |
| b735 | Muscle tone functions |
| b760 | Control of voluntary movement functions |
| b810 | Protective functions of the skin |
| Activities and participation | |
| d155 | Acquiring skills |
| d230 | Carrying out daily routine |
| d430 | Lifting and carrying objects |
| d440 | Fine hand use |
| d445 | Hand and arm use |
| d530 | Toileting |
| d550 | Eating |
| d760 | Family relationships |
| d880 | Engagement in play |
Demographic data were collected from the records. The limitations in functioning that were recorded via interview with the children/parents/caregivers and the surgeon and physiotherapist’s perspectives were retrieved. ICF qualifiers were used to rate the extent of a children’s difficulties based on case history, clinical examination, radiological reports and/or technical investigation. However, not many appropriate outcome measures and scales [patient-reported questionnaire, interviews, etc.] that measure hand and upper limb functions in children less than 5 years are found in the literature. The results were assigned on a 0–4 Likert-type scale [where 0 = no difficulty, 1 = mild difficulty, 2 = moderate difficulty, 3 = severe difficulty, 4 = complete difficulty] [22] after integrating all the accessible and relevant information according to the established ICF linking methodology [30]. The response options ‘8— specified’ and ‘9—not applicable’ were used when available information was not sufficient enough to quantify the severity of the problem and when a category was not applicable to a particular child, respectively.
Data Analysis
The frequencies and the extent of impairments and limitations were calculated for all the ICF categories studied for all the children before and after surgery and was followed up till 1 year. The within-group differences over time to compare the functioning levels postoperatively to baseline for all the ICF categories were calculated as the mean difference [95% CI]. The non-parametric Mann–Whitney U test was used to compare the means between both the groups. All statistical analyses were performed using SPSS [SPSS Inc., Chicago, USA] 10.0 computer programme.
Results
We were able to identify the records of 14 children with RCH who were operated using the techniques studied and followed up till 1 year. The demographic characteristics of the children are presented in Table 2. The numbers and frequency of ICF categories identified by at least 40% of the study participants (or their caregivers) are presented in Table 3. Almost all the children were identified with problems in s730-structure of upper extremity, b710-mobility of joint functions, b730-muscle power functions, b760-control of voluntary movement functions, d430-lifting and carrying objects, d440-fine hand use, d445-hand and arm use, d530-toileting, and d880-engagement in play. The ICF categories of d445-hand and arm use, d530-toileting, and d880-engagement in play showed a change in frequencies of more than 40% after surgery.
Table 2.
Demographic characteristics
| Group 1 | Group 2 | |
|---|---|---|
| Gender | 4 boys, 3 girls | 5 boys, 2 girls |
| Age (in years) | 5.71 (SD 2.36) | 5.57 (SD 2.22) |
| Side | 2 bilateral, 3 right, 2 left | 2 bilateral, 4 right, 1 left |
| Bayne classification | ||
| Type III | 5 | 3 |
| Type IV | 4 | 6 |
Table 3.
Frequency count of the ICF components identified by at least 40% of the population
| Group 1 | Group 2 | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Before surgery | Post surgery | At follow-up | Before surgery | Post surgery | At follow-up | ||||||||
| Number of counts | Frequency (in %) | Number of counts | Frequency (in %) | Number of counts | Frequency (in %) | Number of counts | Frequency (in %) | Number of counts | Frequency (in %) | Number of counts | Frequency (in %) | ||
| Body structures | |||||||||||||
| s720 | Structure of shoulder region | 1 | 11.11 | 1 | 11.11 | – | – | 2 | 22.22 | 2 | 22.22 | – | – |
| s730 | Structure of upper extremity | 9 | 100 | 9 | 100 | 9 | 100 | 9 | 100 | 9 | 100 | 9 | 100 |
| Body functions | |||||||||||||
| b265 | Touch function | 1 | 11.11 | 1 | 11.11 | – | – | 0 | – | 0 | – | – | – |
| b270 | Sensory functions related to temperature and other stimuli | 1 | 11.11 | 1 | 11.11 | – | – | 0 | – | 0 | – | – | – |
| b280 | Sensation of pain | 1 | 11.11 | 1 | 11.11 | – | – | 0 | – | 2 | 22.22 | – | – |
| b710 | Mobility of joint functions | 9 | 100 | 8 | 88.88 | 4 | 44.44 | 9 | 100 | 7 | 77.77 | 2 | 22.22 |
| b715 | Stability of joint functions | 4 | 44.44 | 2 | 22.22 | 1 | 11.11 | 6 | 66.66 | 2 | 22.22 | – | – |
| b730 | Muscle power functions | 9 | 100 | 7 | 77.77 | 7 | 77.77 | 9 | 100 | 8 | 88.88 | 7 | 77.77 |
| b735 | Muscle tone functions | 2 | 22.22 | 2 | 22.22 | – | – | 3 | 33.33 | 2 | 22.22 | – | – |
| b760 | Control of voluntary movement functions | 9 | 100 | 5 | 55.55 | 4 | 44.44 | 9 | 100 | 7 | 77.77 | 4 | 44.44 |
| b810 | Protective functions of the skin | 0 | – | 2 | 22.22 | – | – | 0 | – | 1 | 11.11 | – | – |
| Activities and participation | |||||||||||||
| d155 | Acquiring skills | 4 | 44.44 | 3 | 33.33 | 1 | 11.11 | 2 | 22.22 | 2 | 22.22 | – | – |
| d230 | Carrying out daily routine | 7 | 77.77 | 4 | 44.44 | 3 | 33.33 | 8 | 88.88 | 5 | 55.55 | 2 | 22.22 |
| d430 | Lifting and carrying objects | 9 | 100 | 8 | 88.88 | 5 | 55.55 | 9 | 100 | 7 | 77.77 | 5 | 55.55 |
| d440 | Fine hand use | 9 | 100 | 8 | 88.88 | 7 | 77.77 | 9 | 100 | 7 | 77.77 | 7 | 77.77 |
| d445 | Hand and arm use | 9 | 100 | 4 | 44.44 | 4 | 44.44 | 9 | 100 | 5 | 55.55 | 4 | 44.44 |
| d530 | Toileting | 9 | 100 | 5 | 55.55 | 3 | 33.33 | 9 | 100 | 4 | 44.44 | 2 | 22.22 |
| d550 | Eating | 2 | 22.22 | 1 | 11.11 | 1 | 11.11 | 2 | 22.22 | 2 | 22.22 | 1 | 11.11 |
| d760 | Family relationships | 3 | 33.33 | 2 | 22.22 | 2 | 22.22 | 2 | 22.22 | 2 | 22.22 | 2 | 22.22 |
| d880 | Engagement in play | 9 | 100 | 4 | 44.44 | 3 | 33.33 | 9 | 100 | 5 | 55.55 | 3 | 33.33 |
The changes in ICF qualifiers are presented in Table 4. Whilst some of the categories of activities and participation categories showed an improvement after surgery within the group, categories related to muscle power functions [b730], muscle tone [b735], fine hand use [d440], hand and arm use [d445] and engagement in play [d880] had little or no difference [p > 0.05]. The results of Mann–Whitney test suggested no differences between both the groups [p > 0.05], implying that centralization procedure or ulnarization of the carpus with ulnar shortening osteotomy procedure is no better than one another in improving the functional outcomes in children with RCH.
Table 4.
Changes in qualifiers in the International Classification of Functioning, Disability and Health categories from admission to discharge and admission to follow-up
| Domains | Group 1 | Group 2 | Between group differences | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Time of measurement | Difference 95% CI | Time of measurement | Difference 95% CI | ||||||||
| ICF code | Pre-operative Mean (S.D) |
Post-operative Mean (S.D) |
Follow-up Mean (S.D) |
Baseline and 16 weeks | Baseline and 52 weeks | Post-operative Mean (S.D) |
Pre and post operative | Follow-up Mean (S.D) |
Baseline and 16 weeks | Baseline and 52 weeks | |
| s720 Structure of shoulder region | 1 | 1 | – | – | 1.5 (0.70) | 1.5 (0.70) | – | – | – | ||
| s730 Structure of upper extremity | 3.22 (0.66) | 2.44 (0.52) | 2.6 (0.57) | (0.26, 1.29) | (0.58, 1.9) | 3.22 (0.44) | 2.22 (0.44) | 2.7 (0.42) | (0.45, 1.54) | (0.44, 1.8) | 0.450 |
| b265 Touch function | 1 | 1 | – | – | – | 0 | 0 | – | – | – | – |
| b270 Sensory functions related to temperature and other stimuli | 1 | 1 | – | – | – | 0 | 0 | – | – | – | – |
| b280 Sensation of pain | 1 | 1 | – | – | – | 0 | 1.5 (0.70) | – | – | – | – |
| b710 Mobility of joint functions | 3.33 (0.70) | 2.33 (0.70) | 2.1 (0.40) | (0.33, 1.66) | (0.67, 1.6) | 3.22 (0.66) | 2.11 (0.78) | 2.2 (0.63) | (0.39, 1.82) | (0.61, 1.7) | 0.605 |
| b715 Stability of joint functions | 3.5 (0.57) | 2.5 (0.57) | 1.8 (0.91) | (− 0.83, 2.83) | (1.1, 1.7) | 3.3 (0.81) | 2.5 (0.54) | 1.6 (0.48) | (0.04, 1.62) | (1.1, 1.8) | 1.0 |
| b730 Muscle power functions* | 3.6 (0.5) | 3.4 (0.52) | 2.8 (0.78) | (− 0.29, 0.73) | (0.90, 1.9) | 3.6 (0.50) | 3.3 (0.70) | 2.6 (0.57) | (− 0.33, 0.99) | (0.72, 1.4) | 0.863 |
| b735 Muscle tone functions* | 2.0 (0) | 1.5 (0.70) | – | (− 5.85, 6.85) | – | 2.0 (0.0) | 1.3 (0.57) | – | (− 0.76, 2.1) | – | 0.800 |
| b760 Control of voluntary movement functions | 3.11 (0.26) | 2.33 (0.23) | 1.9 (0.37) | (0.26, 1.29) | (1.2, 1.7) | 3.22 (0.44) | 2.22 (0.83) | 2.0 (0.54) | (0.23, 1.76) | (1.1, 1.8) | 0.650 |
| b810 Protective functions of the skin | 0 | 1 | 0 | – | 0 | 1 | 0 | – | – | – | |
| d155 Acquiring skills | 2.33 (0.50) | 1.55 (0.52) | 1.3 (0.54) | (0.13, 1.41) | (0.19, 0.46) | 2.22 (0.83) | 1.55 (0.52) | 1.4 (0.52) | (0.28, 1.05) | (0.14, 0.44) | 0.815 |
| d230 Carrying out daily routine | 3.8(0.44) | 2.4 (0.54) | 2.0 (0.51) | (0.28, 2.5) | (0.95, 1.4) | 3.7 (0.46) | 2.29 (1.03) | 2.1 (0.62) | (0.72, 2.27) | (0.96, 1.4) | 0.724 |
| d430 Lifting and carrying objects | 3.33 (0.70) | 2.55 (0.72) | 2.3 (0.68) | (0.03, 1.52) | (1.4, 2.2) | 3.33 (0.70) | 2.44 (0.52) | 2.2 (0.67) | (0.42, 1.35) | (1.1, 2.2) | 0.863 |
| d440 Fine hand use* | 3.77 (0.44) | 3.33 (0.50) | 3.6 (0.50) | (− 0.11, 1.00) | (− 0.15, 0.89) | 3.88 (0.33) | 3.55 (0.52) | 3.4 (0.44) | (− 0.21, 0.87) | (− 0.09, 0.94) | 0.436 |
| d445 Hand and arm use* | 3.77 (0.44) | 3.33 (0.50) | 1.9 (0.51) | (− 0.11, 1.00) | (0.83, 1.7) | 3.88 (0.33) | 3.55 (0.52) | 1.6 (0.29) | (− 0.21, 0.87) | (0.90, 1.8) | 0.436 |
| d530 Toileting | 3.44 (0.52) | 2.66 (0.50) | 2.8 (0.50) | (0.26, 1.29) | (0.41, 1.3 | 3.44 (0.52) | 2.77 (0.44) | 2.5 (0.64) | (0.12, 1.21) | (0.45, 1.2) | 0.525 |
| d550 Eating | 2.88 (0.33) | 2.11 (0.78) | 1.4 (0.56) | (0.26, .29) | (0.92, 1.8) | 2.44 (0.72) | 1.77 (0.44) | 1.1 (0.45) | (0.00, 1.33) | (0.89, 1.6) | 0.389 |
| d760 Family relationships | 3.00 (0.50) | 2.55 (0.88) | 2.1 (0.40) | (− 0.11, 1.00) | (0.64, 1.2) | 2.77 (0.44) | 2.33 (1.00) | 2.3 (0.38) | (− 0.42, 1.31) | (0.61, 1.2) | 0.489 |
| d880 Engagement in play* | 3.55 (0.52) | 3.00 (0.50) | 3.1 (0.78) | (− 0.12, 1.23) | (− 0.99, − 0.04) | 3.55 (0.52) | 3.11 (0.60) | 3.3 (0.49) | (− 0.23, 1.12) | (− 1.0, − 0.08) | 0.654 |
P.S-no between group changes for all the ICF categories studied (p > 0.05) at final follow-up
*No significant changes within group (p > 0.05)
Discussion
This study highlighted the ICF categories of body functions, activities, and participation that are identified as significant problems in children with RCH before and after surgery. Previous studies have evaluated the outcomes in these population using many clinical and radiological tools and measures. However, not many studies have evaluated the upper limb functioning from the perspective of activities and participation. In a systematic review, Murphy et al. [31] reported that studies choose to report only morphological outcomes (hand-forearm angle, ulnar length, and “wrist” active range of motion) on long term (> 9 years of follow-up), and do not consider the outcomes which are important to patients. The outcome measures should measure the functioning of the child’s routine functional abilities and not body functions alone.
We were able to identify only one study [26] that had investigated the functioning abilities of children with radial dysplasia using the ICF-CY. The main objectives of the Ekblom et al. [26] were to identify the parameters that may be considered important for activity and participation in children with RCH. Their study had wide inclusion criteria. The participants were children between 4 and 17 years belonging to Bayne type II–IV category. Some of the children recruited had pre-existing clinical syndromes and the treatment interventions were heterogeneous in nature. Though the study had used the ICF framework, the constructs of the ICF were not utilized. Another limitation of this study was that they had included only the ICF-CY framework and not any other core set that evaluated hand functions.
It has been found that no outcome measure or assessment tool measures all the domains for measuring the upper extremity functioning using the ICF-CY core set for children with cerebral palsy [32]. Hence, a combination of measures is needed to evaluate the upper extremity functioning when using this core set. We had used the combined brief ICF core set for child and youth with cerebral palsy up to the age of 5 and the brief ICF core set for hand conditions to measure the functional outcomes on a prospective pre–post design in a sample with strict inclusion and exclusion criteria. The changes in frequencies and numbers provided an over-all picture of changing patterns in functional profiles before and after surgery and at 1 year. The study showed that both the surgeries were effective in improving only certain components of functioning and that no difference was observed between both the groups in improving outcomes.
The objective of surgical correction of RCH is to have a cosmetically acceptable and functional upper limb. The surgical techniques are expected to achieve this objective by correcting the radial deviation of the wrist, providing a stable wrist motion and sufficient forearm length. The results of the study showed that the ICF category of s730-structure of upper extremity showed a significant improvement after surgery in both the groups. The centralization procedure attempts to correct the radial angulatory deformity by aligning the 3rd metacarpal at right angles to the plane of ulna epiphysis. This procedure increases the length of the forearm by placing the wrist on the ulna and thereby aligning the position of digital tendons [10]. The ulnarization procedure is essentially an advancement of the procedure suggested by Buck-Gramcko [9]. The carpus is displaced in an ulnar direction and the flexor carpi ulnaris tendon with the pisiform is moved to the dorsal aspect of the hand, thereby using it as a balancing force [13]. These results are in line with the results of other studies that showed an improvement in the cosmetic appearance of hand after centralization [8, 33] and ulnarization procedure [13].
This improvement in appearance, however, was not accompanied by any significant functional improvements in upper limb functions reflecting that a well-aligned hand may not necessarily improve the functioning. Previous work has documented the limited improvement in function after centralization [2]. One reason for the limited effectiveness on functional outcomes may be attributed to the fact that the current surgical techniques are restricted to correcting the radial deviation and enhancing grasp by pollicization and the surgical techniques do not attempt at enhancing active finger motion [26]. Further, the notched centralization procedure has been found to have an adverse effect on wrist motion [9, 18]. Reasons unrelated to surgical procedures include abnormalities identified in the deltoid, pectoralis major, biceps brachii, brachialis, brachioradialis, supinator, small finger extensor, and the extrinsic and intrinsic muscles of the thumb; abnormalities of the musculocutaneous and the radial nerves; and the absence or underdevelopment of radial artery [34].
One major drawback of this study was that we followed the children for only 1 year. Though the duration of follow-up is important (usually up to 9 years to include two growth spurts or till skeletal maturity) in pediatric hand surgery, it has been shown that the hand functioning develops rapidly during the first few years of life and then at a lower rate till adulthood in normal children [35]. The study by Ekbolm et al. [23] has shown that grip strength did not increase with age in RCH as for normally developed children and the performance in box and block test was lower compared to norms. The effectiveness of many of the surgical techniques on clinical outcomes over long-term follow-up has been demonstrated already and the results show that a well-aligned, stable “wrist” is no more functional than an unstable wrist [31]. We evaluated the functioning in this population by measuring the body functions and participation perspectives by measuring what the child did in his/her actual environment. The outcomes were measured after a period of intensive hand and upper limb rehabilitation; we do not think the results would change in the longer term as previous works have shown that functioning is not related to passive range of motion or radial dysplasia, but to other motor skills (active range of motion, muscle tone/strength/coordination, dexterity, and quality of movement) [26, 27]. Further, the need for additional surgeries would suggest that the original procedure may not be effective and an indication for revision surgeries itself might mean the desired outcomes are not met. Another limitation of the study was that the study sample was small which might prevent extrapolation of our results and interpretations should be made with caution. The other potential limitations of this study were that only two surgical procedures were studied and we were unable to compare the results compared to a control group. All children who underwent centralization procedure were more than 1 year old. No simultaneous pollicization was performed and personal impressions of the evaluator might have affected the quantifications. One child in group 1 developed pin tract infection which was treated with antibiotics. One patient in group 2 developed necrosis of skin, which was treated by excision and skin graft. No other major complication associated with these procedures like growth arrest of the distal ulna, ankylosis of the wrist, and recurrent instability of the wrist was observed, as the follow-up was rather short.
Considering the limited improvements in specific functional activities after surgery as reported by this study and the previous works, there is a need for consensus regarding the objectives of orthopedic surgery for the management of RCH. Studies may monitor the development of hand function over time in children with RCH. Future research should evaluate if the surgical procedures for RCH are cost-effective compared to the functional outcomes and other benefits [prevention of the recurrence of radial deviation deformity and growth arrest in this population]. Future techniques may explore ways to improve the total range of motion of wrist and digits, grip strength, key pinch and manual performance of hand over prevention of recurrence of radial deviation deformity.
Conclusion
Based on the results, we would like to conclude that both these surgical techniques are effective in improving the structural appearance of the upper limb. The wide array of abnormalities seen in the entire upper limb seriously poses a threat to the functional performance of the children even after surgery. Studies with long-term functional follow-up are needed to elucidate if the surgical techniques are any better over another. The parents of the children have to be educated about the co-existing morbidities in RCH and it is worthwhile to advise the parents of the children about the anticipated cosmetic and limited functional benefits after surgery. Future studies on RCH shall consider evaluating the effectiveness of surgical procedures on functional outcomes rather on radiological parameters.
Compliance with ethical standards
Conflict of interest
The authors declare that there is no conflict of interest regarding the publication of this article.
Ethical Standard Statement
This study was approved by the local ethics committee and has therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.
Informed Consent
Informed consent was obtained from all the primary caregivers before the surgical procedure.
Footnotes
Publisher's Note
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Contributor Information
Sakti Prasad Das, Email: sakti2663@yahoo.com.
G. Shankar Ganesh, Email: shankarpt@rediffmail.com.
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