Physical deformities of bones and joints are common correlates of the disease of cerebral palsy; they often impede movement and sometimes cause pain. Deformation may occur in almost any part of the body, from head to foot. Windswept hip deformity, pelvic obliquity, and scoliosis are among the more common types of physical deformations found in cerebral palsy. However, deformation may also occur in the hands, feet and face of the cerebral palsy sufferer. In some cases surgical interventions may be carried out to correct – at least to some extent – such deformations.
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The asymmetrical deformities in 20 children with various types of cerebral palsy are compared with 20 children without cerebral palsy who have the so-called 'squint' baby syndrome (asymmetrical deformities of plagiocephaly, unilateral bat ear, facial and thoracic asymmetry, pelvic obliquity and apparent shortening of one leg). It is suggested that the 'squint' baby syndrome and the 'windswept' child syndrome in children with cerebral palsy are stages of the same syndrome and that in both the deformities are caused by the effect of gravity on an immobile growing child, rather than spasticity or muscle imbalance. Asymmetrical deformity should therefore be amenable to physiotherapeutic intervention, rather than trying to modify maturation of the damaged brain. As the 'windswept' cerebral palsied child can develop some of the most severe deformities seen in cerebral palsy, it is important that asymmetrical deformities should be prevented.
[Fulford, F. E. & Brown, J. K. (1976). Position as a cause of deformity in children with cerebral palsy. Developmental Medicine and Child Neurology, 18(3), 305-14.]
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BACKGROUND: Thumb-in-palm deformity in patients with spastic cerebral palsy is a deformity that impairs the ability to use the thumb and thus severely limits hand function. From the variety of operative procedures that have been described, it may be clear that there is no consensus on the surgical treatment of thumb-in-palm deformity. OBJECTIVES: To review the efficacy of surgical interventions for the thumb-in-palm deformity in patients with spastic cerebral palsy; to review the selection criteria to surgically treat thumb-in-palm deformity in these patients; and to review the outcome assessment used in these studies. SEARCH STRATEGY: We identified studies for inclusion from searches of several electronic databases: the Cochrane Central Register of Controlled Trials (Issue 4, 2003), MEDLINE (1966 to December 2004), EMBASE (1980 to December 2004) and CINAHL (1982 to December 2004).We also cross-checked the reference lists of these studies to identify additional studies. SELECTION CRITERIA: We considered a trial eligible for inclusion when it met the following criteria. 1) It was described as a randomized clinical trial, clinical controlled trial or prospective study that compared pre-operative- with post-operative outcome assessment. 2) It concerned patients with thumb-in-palm deformity affected by spastic cerebral palsy. There was no restriction in age. 3) It compared or described any surgical intervention to the thumb. 4) It followed subjects for at least six months. 5) Outcomes described included one or more of the following items: rate of success; functional improvement; active and passive range of motion of the thumb; grasp and release; pinch grip; complications and side effects; and quality of life. DATA COLLECTION AND ANALYSIS: Two authors assessed each study using a scoring system. Meta-analysis was not possible because the selected studies were poorly designed, and the results were presented in an incompatible form. Therefore, we compiled a descriptive summary of the results of the individual studies. We did not attempt to acquire the raw data for re-analysis. MAIN RESULTS: We identified 14 prospective studies that compared preoperative and postoperative outcomes as eligible for inclusion in this review. We found no randomized clinical trials or controlled clinical trials. The studies with the best available evidence were prospective studies that compared pre- with post-operative assessment. After assessment, we ultimately included nine studies. Participants In all the included studies the participants were more or less homogeneous regarding the most important prognostic indications. The nine included studies treated 234 patients. Age at operation ranged from 4-48 years (Median approximately 11 years).Interventions Twenty-four different specific interventions were performed, or combined, aiming to 1) stabilize the first metacarpophalangeal joint, 2) weaken the spastic thumb adductors, and 3) augment thumb abduction and extension. Outcome measures All of the included studies assessed whether the thumb had stayed out of the palm at follow-up. Additional outcome measures varied among studies. Selection criteria There was no consensus on the selection criteria for eligibility for surgical treatment. There was also considerable variety in the use of methods of assessment among the studies. There is no standardized method to evaluate the pre- and post-operative data, and most of the assessment methods were not validated. It was impossible to compare the outcomes among studies. Judgement about the effectiveness of one particular surgical intervention was not possible, because different surgical interventions and co-interventions were used within most studies. Nonetheless, generally, the outcome of surgical treatment of thumb-in-palm deformity was considered satisfactory to both patients and to surgeons in all studies. AUTHORS' CONCLUSIONS: Because the methodological quality of the studies is poor, it is impossible to provide a reliable judgment of the role of surgery for thumb-in-palm deformity. This review has demonstrated the need for randomized clinical trials or controlled clinical trials on the surgical treatment of thumb-in-palm deformity. Surgical reconstruction appears to improve hand function, to facilitate hygiene, and to improve the appearance and quality of life. For patient selection, a validated classification system should be developed to determine the type and extent of the cerebral palsied hand. The influence of age, intelligence, and voluntary muscle control still needs to be investigated. Investigators should focus on one particular surgical intervention or a specific group of interventions to find out which procedures produce the best functional improvement.
[Smeulders, M., Coester, A. & Kreulen, M. (2005). Surgical treatment for the thumb-in-palm deformity in patients with cerebral palsy. Cochrane Database of Systematic Reviews (online), 4, CD0004093.]
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OBJECTIVE: To investigate: (a) associations between the direction of scoliosis, direction of pelvic obliquity, direction of windswept deformity and side of hip subluxation/ dislocation in non-ambulant people with cerebral palsy; and (b) the lateral distribution of these postural asymmetries. DESIGN: Cross-sectional observational study. SETTING: Posture management services in three centres in the UK. SUBJECTS: Non-ambulant people at level five on the gross motor function classification system for cerebral palsy. MAIN MEASURES: Direction of pelvic obliquity and lateral spinal curvature determined from physical examination, direction of windswept hip deformity derived from range of hip abduction/adduction, and presence/side of unilateral hip subluxation defined by hip migration percentage. RESULTS: A total of 747 participants were included in the study, aged 6-80 years (median 18 years 10 months). Associations between the direction of scoliosis and direction of pelvic obliquity, and between the direction of windswept hip deformity and side hip subluxation/dislocation were confirmed. A significant association was also seen between the direction of scoliosis and the direction of the windswept hip deformity (P<0.001) such that the convexity of the lateral spinal curve was more likely to be opposite to the direction of windsweeping. Furthermore, significantly more windswept deformities to the right (P=0.007), hips subluxed on the left (P=0.002) and lateral lumbar/lower thoracic spinal curves convex to the left (P=0.03) were observed. CONCLUSIONS: The individual asymmetrical postural deformities are not unrelated in terms of direction and not equally distributed to the left/right. A pattern of postural deformity was observed.
[Porter, D., Michael, S. & Kirkwood, C. (2007). Patterns of postural deformity in non-ambulant people with cerebral palsy: What is the relationship between the direction of scoliosis, direction of pelvic obliquity, direction of windswept hip deformity and side of hip dislocation? Clinical Rehabilitation, 21(12), 1087-96.]
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PURPOSE OF THE STUDY: Children with cerebral palsy who cannot walk have an oblique pelvis and scoliosis. There is a certain degree of controversy in the literature on the best way to manage this difficult situation. We present a descriptive analysis of a population of non-ambulatory adults with cerebral palsy in order to formulate hypotheses concerning the factors determining scoliosis. MATERIAL AND METHODS: This descriptive cross-sectional study was conducted in 234 patients aged over 15 years who had cerebral palsy and could not walk. Physical examination and an x-ray of the pelvis and spine in the reclining position were obtained for all patients. The following variables were recorded: luxation and subluxation of the hip, spontaneous deviation attitude, ability or not to turn over in bed, pelvic obliquity, history of bone surgery, defective hip abduction. The statistical analysis accounted for laterality and pelvis obliquity to the scoliosis convexity and the laterality of the hip excentration. RESULTS: Scoliosis was observed in 66.2% of the patients; it was more than 60 degrees in 34.5%. Two basic groups were distinguished: thoracolumbar scoliosis (41.6%) and lumbar scoliosis (41.6%). The prevalence of oblique pelvi was 59.9% with important difference by side: 31.6% right oblique and 68.4% left oblique pelvi. We were unable to find any relationship between the side of the pelvic obliquity and the side of the scoliosis convexity, the side of the hip excentration, or the deviation attitude, but the deviation attitude appeared to be a risk factor for pelvic obliquity, which itself was a risk factor for excentration, which was a risk factor for scoliosis. DISCUSSION: Scoliosis is an important problem in this population. Hip luxation is a direct risk factor for scoliosis, but the deviation attitude and pelvic obliquity are intermediary stages. The prevalence of oblique pelvi was greater on the left than the right. This finding should be confirmed in other series before hypotheses can be formulated concerning this difference.
[Hodgkinson, I., Berard, C., Chotel, F. & Berard, J. (2002). Pelvic obliquity and scoliosis in non-ambulatory patients with cerebral palsy: A descriptive study of 234 patients over 15 years of age [French]. Revue de Chirurgie Orthopedique et Reparatrice de l’Appareil Moteur, 88(4), 337-41.]
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The incidence of scoliosis in cerebral palsy is related to the severity of the neurological involvement, being most prevalent in patients with spastic quadriplegia. Neuromuscular spinal deformity, when present, may progress after cessation of skeletal growth, and the success of orthotic treatment for scoliosis is unpredictable. Hip pathology is directly related to pelvic obliquity but has no causal relationship to the development of scoliosis. Adequate preoperative nutritional assessment is vital to reduce perioperative complications, and segmental spinal fixation is the instrumentation of choice. Anterior arthrodesis is indicated for rigid deformities and for those thoracolumbar and lumbar curves extending into the pelvis with pelvic obliquity and spinal decompensation.
[Banta, J. V. (1992). Spinal disorders in cerebral palsy--surgical procedure [German]. Der Orthopade, 21(5), 309-15.]
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Windswept hip deformity describes an abduction and external rotation position of one hip with the opposite hip in adduction and internal rotation. Windswept hip deformity may occur in association with hip dislocation and scoliosis. We analysed the prevalence of this deformity in a total population of children with cerebral palsy, and the impact of hip prevention and early treatment of contractures on the prevalence and severity of windswept hip deformity. The frequency of windswept hip deformity was 12% in the control group and 7% in the study group, comprising children in the hip prevention programme. The children with this deformity in the study group had a lower frequency of scoliosis and none had hip dislocation. It thus seems that the hip prevention programme results in a decrease in the number of children with windswept hip deformity, and a decrease in the severity of the deformity.
[Persson-Bunke, M., Hagglund, G. & Lauge-Pedersen, H. (2006). Windswept hip deformity in children with cerebral palsy. Journal of Pediatric Orthopedics. Part B, 15(5), 335-8.]
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Bone and joint changes in cerebral palsy result from muscle spasticity and contracture. The spine and the joints of the lower extremity are most commonly affected. Scoliosis may progress rapidly and may continue after skeletal maturity. Increased thoracic kyphosis and lumbar lordosis, spondylolisthesis, spondylolysis, and pelvic obliquity may accompany the scoliosis. Progressive hip flexion and adduction lead to windswept deformity, increased femoral anteversion, apparent coxa valga, subluxation, deformity of the femoral head, hip dislocation, and formation of a pseudoacetabulum. In the knee, flexion contracture, patella alta, and patellar fragmentation are the most commonly seen abnormalities. Recurvatum deformity can also develop in the knee secondary to contracture of the rectus femoris muscle. Progressive equinovalgus and equinovarus of the foot and ankle are associated with rocker-bottom deformity and subluxation of the talonavicular joint. Early recognition of progressive deformity in patients with cerebral palsy allows timely treatment and prevention of irreversible change. Copyright RSNA, 2002
[Morrell, D. S., Pearson, J. M. & Sauser, D. D. (2002). Progressive bone and joint abnormalities of the spine and lower extremities in cerebral palsy. Radiographics, 22(2), 257-68.]
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Scoliosis is common in children with cerebral palsy. The incidence and curve pattern depend on the degree of neurologic involvement. These children carry a higher risk of complications because of the increased presence of associated medical comorbidities. Accordingly, a careful preoperative evaluation is required that should involve assessment of the patient's pulmonary, nutritional, gastrointestinal, and neurologic systems as well as a thorough evaluation of the spine and musculoskeletal system. Children with progressive curves >40 degrees to 50 degrees are candidates for spinal fusion, especially when the deformity interferes with sitting or is unresponsive to bracing. The goal of surgery is to obtain a stable, balanced, and painless spinal fusion. Although posterior spinal fusion with multisegmental fixation is the most common technique, others, such as anterior release and/or fusion or combined procedures, are now considered. In patients with significant pelvic obliquity or who are at risk of developing pelvic obliquity, instrumentation should extend to the pelvis, particularly in the nonambulatory child.
[McCarthy, J. J., D’Andrea, L. P., Betz, R. R. & Clements, D. H. (2006). Scoliosis in the child with cerebral palsy. The Journal of the American Academy of Orthopaedic Surgeons, 14(6), 367-75.]
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Spinal deformities concern 25% of patients with cerebral palsy. Thoracolumbar scoliosis represents the most common pattern of deformity. The balance capacities and postural control are poor in cerebral palsy patients. Spinal curvature creates additional problems for child's posture and locomotion. This can result in loss of functional capacities such as walking, standing or sitting. Pelvic obliquity is an important pathogenic factor. Brace treatment may be effective in small and flexible curves. Indications for surgery should balance the advantages and potential risks of operation. The typical procedure consists of stable segmental instrumentation from posterior approach. The pelvis is usually included in non-ambulatory patient or a case of important pelvic obliquity. The anterior approach may be necessary to improve the flexibility of the curve. Rapid verticalisation, stable fixation sparing the brace immobilization, special nutrition program and respiratory exercises are used in postoperative period.
[Kotwicki, T. & Szulc, A. (2002). Curvature of the spine in children with cerebral palsy. Ortopedia, Traumatologia, Rehabilitacja, 4(1), 42-7.]
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Treatment of patients with cerebral palsy need particular management: diagnostics, early interventions, programme of functional estimations, rehabilitation, therapy with conservative methods (injections of botulin toxin, immobilizing cast) and surgical methods. Correction of foot deformations is only one part of general process of treatment, which target is usually equalization strength between antagonistic muscle groups, what influences on improvement of locomotion, enlargement of range joint mobility, diminution of pain and making possible orthopedic supplies. In 1997-2001 we treated 58 children with foot deformation in cerebral palsy. One talk overed mechanism of deformation and management in dependences from type, degree of deformation, and age of patient.
[Barczynski, A., Pasierbek, M., Gazdzik, T. S. & Klosa, Z. (2002). Management of foot deformity in cerebral palsy. Ortopedia, Traumatologia, Rehabilitacja, 4(1), 21-6.]
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Surgery based on an accurate determination of deforming forces, muscle weakness, and joint instability achieves reasonably predictable results in patients with cerebral palsy. Thumb deformities can be classified into those of mainly intrinsic nature, extrinsic nature, and those of a combined nature. This allows identification of which muscles are released most appropriately and which muscle function requires augmentation. Joint instability needs to be taken into consideration because tendon transfers across unstable joints will be ineffective. The various surgical procedures are outlined in the text. In 32 patients, application of these principles and techniques allowed removal of the thumb from the palm in 29 patients during fist formation. Lateral pinch was established in 26 thumbs, and functional assessment revealed improvement in one functional grade. Functional benefit may be determined by other factors beyond the control of the surgeon. Therefore, the decision to proceed to surgery may be undertaken only after detailed and repeated clinical examinations are conducted that are coordinated with the assessments of parents, caregivers, physicians, occupational and physical therapists, and social counselors.
[Tonkin, M. A. (2003). Thumb deformity in the spastic hand: Classification and surgical techniques. Techniques in Hand & Upper Extremity Surgery, 7(1), 18-25.]
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