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Joint deformity patterns in severely physically disabled patients
Brain & Development 23 (2001) 371–374 www.elsevier.com/locate/braindev
Original article
Joint deformity patterns in severely physically disabled patients Kenji Yokochi* Department of Rehabilitation, Ohzora Hospital, Inasa, 7448 Nakagawa, Hosoe, Inasa,Shizuoka 431-1304, Japan Received 25 September 2000; received in revised form 2 June 2001; accepted 4 June 2001
Abstract Deformity patterns of the spine and upper and lower extremities were investigated in 64 patients with severe physical disability. Among the subjects, C-shaped and S-shaped scoliosis was found in 48 and nine, respectively. The hips were windblown in 20, adducted in 22, and abducted in seven. Knees were flexed in 39 and extended in four. Deformities of the ankle and upper extremities were variable. The most common combination, which was C-shaped scoliosis with convexity to the adducted side of hips, windblown hips, flexed knees, and dorsiflexed ankles, was noted among nine patients. Joint deformity patterns in the 43 patients with spastic tetraplegia were not significantly different from those in the 21 patients with dyskinesia. q 2001 Elsevier Science B.V. All rights reserved. Keywords: Joint-deformity; Windblown deformity; Severely physically disabled patient
1. Introduction Among the central motor disorders of childhood, with cerebral palsy the manner in which joints of the upper or lower extremities deviate and develop contractures is generally known for each type of cerebral palsy [1,2]. In children with severe physical disability originating from CNS lesions, the trunk is involved and spinal deformities develop in association with deformities of the upper and lower extremities [3–11]. However, details of combinations of deformities affecting the spine and upper and lower extremities of children with severe physical disability have not been fully elucidated, although such knowledge is important for physical therapy and nursing. In the present study, joint deformity patterns were investigated in patients with severe physical disability from late childhood to early adulthood, and the relationship between each deformity pattern and the clinical profiles of the subjects was examined.
2. Materials and methods Sixty-four patients (38 males and 26 females) were studied. The subjects were selected among patients attending or being admitted to the Ohzora Hospital from 1990 to 1999. At the time of assessment of joint deformity, the patients ranged in age from 10 to 30 years; ranges of ages of 22, 22, 13 and seven patients were 10–15 years, 16–20 * Fax: 181-53-437-8714. E-mail address: [email protected] (K. Yokochi).
years, 21–25 years, and 26–30 years, respectively. They were severely physically disabled; sitting, rolling, and locomotion in a prone or supine posture could not be performed alone. Forty-three patients had spastic tetraplegia, and the remaining 21 had a dyskinetic syndrome [12]. The subjects had profound mental retardation; their mental ages were assessed to be less than 2 years. None spoke meaningful words. Sixty-one had epilepsy. Nine patients underwent tracheostomy for respiratory problems. Nineteen patients received tube feeding for dysphagia. Patients who underwent orthopedic surgery to correct joint deformity were excluded from this study. None of the subjects had a degenerative disease with intellectual or motor deterioration. In 18 patients, the brain damage occurred prenatally. Thirteen had multiple minor anomalies which were revealed by physical assessment or an anomaly of the brain that was revealed by neuroimaging. The remaining five were included in this group only because there were no perinatal or postnatal events that would possibly result in brain damage. No patient had a chromosomal aberration. In 28 patients, the brain damage occurred perinatally. Of these, brain damage was caused by complications associated with preterm birth in seven; gestational age was 36 weeks or less. In 17, the cause was neonatal asphyxia; gestational age was 37 weeks or more. Apgar scores were 6 or below at 1 min, and signs of postpartum encephalopathy began during the first 48 h of life as manifested by seizures, lethargy, coma, extensive hypo- or hypertonia, or pathological spontaneous movements. Neonatal bacterial meningitis and kernicterus was the cause in three and one, respectively, of those with perinatal brain damage. In the remaining 18 patients, brain
0387-7604/01/$ - see front matter q 2001 Elsevier Science B.V. All rights reserved. PII: S 0387-760 4(01)00240-6
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K. Yokochi / Brain & Development 23 (2001) 371–374
damage occurred postnatally between 5 months and 9 years of age; causes were acute encephalopathy or encephalitis in eight, anoxic encephalopathy in five, head trauma in four and bacterial meningitis in one. Joint deformities were assessed at 10 years or longer after the occurrence of brain damage in these 18 patients. Scoliosis was evaluated as a representative deformity of the spine. It was confirmed to be structural by physical examination, and vertebral anomalies were not recognized in radiographs. Side of convexity, type of curve (C- or Sshaped), and apex vertebra were examined as characteristics of the curve [5]. Anteroposterior radiographs of the chest and/ or abdomen in the supine position taken within 3 years before the time of assessment and physical examination were used for evaluating scoliosis, because radiographs of the spine were not taken regularly as a policy of the author’s hospital. When the curve roughly measured by the Cobb method was less than 208, scoliosis was judged to be ‘minimal’. Abduction or adduction contracture was evaluated as a representative deformity of the hips. The range of abduction or adduction of each hip was measured with the hips flexed 908 or with the hips flexed maximally less than 908. When one hip could be abducted 108 or less and adducted 408 or more (adducted side), and the other hip (abducted side) could be abducted 408 or more and adducted 208 or less, the hips were judged to be ‘windblown’. When both hips could be adducted 308 or more and abducted 208 or less, they were judged to be ‘adducted’. When both hips could be abducted 308 or more and adducted 08 or less, the hips were judged to be ‘abducted’. When the ranges of hip-motion did not correspond to the above-mentioned three patterns, hips were classified as ‘other’. Hip dislocation was not examined in this study, because radiographs of the hips were not taken regularly as a policy of the author’s hospital. Flexion or extension contracture was evaluated as a representative deformity of the knees. When both knees could be extended 2308 or less with the hips extended maximally, knees were judged to be ‘flexed’ When both knees could be flexed 408 or less, they were judged to be ‘extended’. When the range of knee-motion did not correspond to either of the above-mentioned two patterns, a classification of ‘other’ was given. Plantarflexion or dorsiflexion contracture was evaluated as a representative deformity of the feet. When both feet could be dorsiflexed 0 degree or less with the knees extended maximally, feet were judged to be ‘plantarflexed’. When both feet could be planrtarflexed 108 or less, they were judged to be ‘dorsiflexed’. A classification of ‘other’ was assigned when ranges of motion of the feet did not correspond to either of these two patterns. Flexion contracture of the elbow and flexion or extension contracture of the wrist were evaluated as representative deformities of the upper extremities. When the elbow could be extended -308 or less, the elbow was judged to be ‘flexed’. When the wrist could be extended 0 degree or less, the wrist was judged to be ‘flexed’. When the wrist could be flexed 08 or
less, the wrist was judged to be ‘extended’. When both elbows were flexed and both wrists were flexed or extended, the upper extremities were termed as ‘contracted arms’. When both elbows were not flexed and both wrists were either flexed or extended, the designation of ‘contracted wrists’ was assigned. When both elbows and wrists did not have the above-mentioned contractures, the upper extremities were not judged to be contracted and were given the designation of ‘none’. When the ranges of elbow and wrist motions did not correspond to any of the above-mentioned three patterns, the upper extremities were classified as ‘other’. 3. Results Joint deformity patterns and clinical profiles of the subjects are shown in Tables 1 and 2. The majority of subjects, 48 of the 64 had C-shaped scoliosis. Of these 48 subjects, 33 had convexity to the left. Judging from the apex vertebra, C-shaped scoliosis was thoracolumbar in 30 patients, lumbar in 17, and thoracic in 1. A minority of the patients, i.e. 16, had S-shaped or minimal scoliosis. Windblown and adducted deformity was found in 20 and 22 subjects, respectively, and were the major types of hip deformity. Fifteen of the 20 patients with windblown hip deformity had C-shaped scoliosis. In those 15 patients the convex side of scoliosis and the adducted side of the windblown hips were same. In four patients with S-shaped scoliosis, the convex side of the lower curve and the adducted side of windblown hip deformity were opposite. Of the 64 patients, 40 had a flexed knee deformity. Only four patients had an extended knee deformity associated with an adducted hip deformity. In the majority of patients (37/64), the ankle deformity was classified as ‘other’. Plantarflexed ankle deformity was not evident in patients with the windblown hip deformity or in those with the abducted hip deformity. The dorsiflexed ankle deformity was predominant in patients with windblown hips. Nine patients had C-shaped scoliosis with the convexity toward the adducted side of the hips, windblown hips, flexed knees, and dorsiflexed ankles, which was the most common combination of spine, hip, knee and foot deformities among the subjects. The deformity pattern of the upper extremities was variable. The joint deformity pattern in patients with spastic tetraplegia was not significantly different from that in patients with dyskinesia (chi-squared test, Table 2). 4. Discussion C-shaped scoliosis was found in many of our subjects in agreement with previous reports of spine deformity in cerebral palsied patients [3–11]. The spine of nonambulatory, especially bedridden, patients with central motor disorders is thought to develop mostly C-shaped scoliosis. Patients who are bedridden and have C-shaped scoliosis are known to be at risk of progression of the curve with advancing age
K. Yokochi / Brain & Development 23 (2001) 371–374
373
Table 1 Joint deformity patterns and clinical profiles in study subjects Patterns of hip deformity
Windblown (n ¼ 20) a
Adducted (n ¼ 22)
Abducted (n ¼ 7)
Others (n ¼ 15)
Total (n ¼ 64)
Spine C (R:L) b Sc Minimal
15 (4:11) d 4e 1
14 (3:11) 3 5
6 (4:2) 1 0
13 (4:9) 1 1
48 (15:33) 9 7
16 0 4 0 12 8
11 4 7 4 4 14
7 0 0 0 4 3
6 0 9 2 1 12
40 4 20 6 21 37
Upper extremities Contracted arms Contracted wrists Other None
4 3 7 6
3 6 8 5
2 3 1 1
1 5 2 7
10 17 18 19
Cause of brain damage Prenatal Perinatal (asphyxia) Postnatal
7 8 (5) 5
5 10 (9) 7
1 5 (2) 1
5 5 (1) 5
18 28 (17) 18
17 5
5 2
9 6
43 21
Lower extremities Knee
Ankle
Clinical features Spastic Dyskinetic a b d c e
Flexed Extended Other Plantarflexed Dorsiflexed Other
12 8
Adductive side of windblown hips is right in 6 and left in 14. (R:L), side of the convexity of C-shaped scoliosis. Adductive side of windblown hips is same as the side of the convexity of C-shaped scoliosis in all of the patients. Side of the convexity of lower curve is right in 6 and left in 3. Adductive side of windblown hips was right in 2 and left in 2; it is opposite to the side of the convexity of the lower curve in all four patients.
[7,8,10,11]. Differing from previous reports [3,4,9,11], a curve with the convexity to the left was commonly seen in patients with C-shaped scoliosis in this study. Although the cause of this difference cannot be determined in this study, this difference may result from the degree of gross motor function among patients in various studies. Our study subjects were more severely disabled than those in previous reports [3,4,9,11] in that they were unable to sit or to locomote in a prone or supine posture. Patients with severe impairment of gross motor function resulting from brain lesions may manifest C-shaped scoliosis with the convexity to the left. Among other possibilities, different curves may result from different kinds of brain lesions or the various postures in which mothers or caregivers usually place the patient. Windblown hips associated with hip dislocation on the adducted side have been noted in nonambulatory patients with cerebral palsy [3,4,6,7]. Windblown hips have also been reported to accompany variable forms of scoliosis [3,4,6,7]. In this study, S-shaped scoliosis was found only in a small number of subjects with windblown hips, differing from previous reports [3,4,6]. An additional difference was that the relationship between the laterality of windblown hips
and the convexity of curvatures was relatively simple; the adducted side of windblown hips was the same as the side of the convexity of C-shaped scoliosis and was opposite to the side of the convexity of the lower curve in S-shaped scoliosis. This difference may also result from the difference in subjects between our study and other studies. Most patients with windblown hips inevitably have instability in sitting, because C-shaped scoliosis with the convexity to the adducted side and the thigh form a longer curvature. Although the hypothesis that hyperactivity of the iliopsoas results in windblown hips and later in scoliosis may explain the development of a lumbar curve with the convexity to the abducted side [6], it cannot apply to the development of C-shaped scoliosis with the convexity to the adducted side. The combinations of hip and spine deformities were variable in this study. The mechanisms for development of scoliosis may not be uniform and may be different from those for development of hip deformity in patients with severe physical disability. Adducted hip deformity mostly associated with flexed knees and C-shaped scoliosis are common hip deformities in patients with severe physical disability. Hip adduction and knee flexion are also likely to be found in children
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Table 2 Joint deformity patterns in patients with spastic tetraplegia and in those with dyskinesia Clinical features
Spastic (n ¼ 43)
Dyskinetic (n ¼ 21)
Total (n ¼ 64)
Spine C (R:L) a S Minimal
30 (11:19) 7 6
18 (4:14) 2 1
48 (15:33) 9 7
12 17 5 9 30 2 11 4 17 22
8 5 2 6 10 2 9 2 4 15
20 22 7 15 40 4 20 6 21 37
Upper extremities Contracted arms Contracted wrists Other None
8 13 16 6
2 4 2 13
10 17 18 19
Cause of brain damage Prenatal Perinatal (asphyxia) Postnatal
6 25 (17) 12
12 3 (0) 6
18 28 (17) 18
Lower extremities Hip
Knee
Ankle
a
Windblown Adducted Abducted Other Flexed Extended Other Plantarflexed Dorsiflexed Other
(R:L), side of the convexity of C-shaped scoliosis.
with spastic diplegia [1,2]; both sides of the lower extremities are affected by spasticity in such children. These deformities are thought to result from a pathophysiological mechanism similar to that of spastic diplegia, although they have been noted in patients with dyskinesia. Abducted hip deformity associated with flexed knees and extended knee deformity associated with adducted hips were found in a minority of patients with severe physical disability, although no clinical profiles corresponded to these two conditions. Although the plantarflexed ankle deformity is common in children with spastic diplegia [1,2], it is found in only a minority of patients with severe physical disability. Complex mechanisms may result in the ankle deformity. This study indicates that joint deformity patterns are variable among patients with severe physical disability. Clearly, more research on motor symptoms of severe central motor disability is needed to establish effective methods of physical therapy and nursing for patients according to the type of joint deformity pattern. References [1] Bleck EE. Orthopedic management of cerebral palsy, Clinics in developmental medicine, no. 99/100. London: MacKeith Press, 1987.
[2] Rang M. Cerebral palsy. In: Morrissy RT, editor. Lovell and Winter’s pediatric orthopedics, 3rd ed. Philadelphia, PA: Lippincott, 1990. pp. 465–506. [3] Samilson RL, Bechard R. Scoliosis in cerebral palsy: incidence, distribution of curve patterns, natural history, and thought of etiology. Curr Pract Orthop Surg 1973;5:183–205. [4] Madigan RR, Wallace SL. Scoliosis in the institutionalized cerebral palsy population. Spine 1981;6:583–590. [5] Lonstein JE, Akbarnia BA. Operative treatment of spinal deformities in patients with cerebral palsy or mental retardation. An analysis of one hundred and seven cases. J Bone Joint Surg Am 1983;65:43–55. [6] Letts M, Shapiro L, Mulder K, Klassen O. The windblown hip syndrome in total body cerebral palsy. J Pediatr Orthop 1984;4:55–62. [7] Okamura Y, Tohno S, Harata S, Moriyama A, Hayashi A, Nara K, et al. Scoliosis in cerebral palsy (in Japanese). Seikeigeka (Tokyo) 1985;36:23–30. [8] Thometz JG, Simon SR. Progression of scoliosis after skeletal maturity in institutionalized adults who have cerebral palsy. J Bone Joint Surg Am 1988;70:1290–1296. [9] Nagaya M, Akashi K, Tomita M, Kawamura T. The deformities of foot and spine in institutionalized children with mental and physical handicaps (in Japanese). Sogo Rehabil (Tokyo) 1991;19:109–114. [10] Majd ME, Muldowny DS, Holt RT. Natural history of scoliosis in the institutionalized adult cerebral palsy population. Spine 1997;22:1461– 1466. [11] Saito N, Ebara S, Ohotsuka K, Kumeta H, Takaoka K. Natural history of scoliosis in spastic cerebral palsy. Lancet 1998;351:1687–1692. [12] Hagberg B, Hagberg G, Olow I. The changing panorama of cerebral palsy in Sweden, 1954–1970. Acta Paediatr Scand 1975;64:187–192.
Original article
Joint deformity patterns in severely physically disabled patients Kenji Yokochi* Department of Rehabilitation, Ohzora Hospital, Inasa, 7448 Nakagawa, Hosoe, Inasa,Shizuoka 431-1304, Japan Received 25 September 2000; received in revised form 2 June 2001; accepted 4 June 2001
Abstract Deformity patterns of the spine and upper and lower extremities were investigated in 64 patients with severe physical disability. Among the subjects, C-shaped and S-shaped scoliosis was found in 48 and nine, respectively. The hips were windblown in 20, adducted in 22, and abducted in seven. Knees were flexed in 39 and extended in four. Deformities of the ankle and upper extremities were variable. The most common combination, which was C-shaped scoliosis with convexity to the adducted side of hips, windblown hips, flexed knees, and dorsiflexed ankles, was noted among nine patients. Joint deformity patterns in the 43 patients with spastic tetraplegia were not significantly different from those in the 21 patients with dyskinesia. q 2001 Elsevier Science B.V. All rights reserved. Keywords: Joint-deformity; Windblown deformity; Severely physically disabled patient
1. Introduction Among the central motor disorders of childhood, with cerebral palsy the manner in which joints of the upper or lower extremities deviate and develop contractures is generally known for each type of cerebral palsy [1,2]. In children with severe physical disability originating from CNS lesions, the trunk is involved and spinal deformities develop in association with deformities of the upper and lower extremities [3–11]. However, details of combinations of deformities affecting the spine and upper and lower extremities of children with severe physical disability have not been fully elucidated, although such knowledge is important for physical therapy and nursing. In the present study, joint deformity patterns were investigated in patients with severe physical disability from late childhood to early adulthood, and the relationship between each deformity pattern and the clinical profiles of the subjects was examined.
2. Materials and methods Sixty-four patients (38 males and 26 females) were studied. The subjects were selected among patients attending or being admitted to the Ohzora Hospital from 1990 to 1999. At the time of assessment of joint deformity, the patients ranged in age from 10 to 30 years; ranges of ages of 22, 22, 13 and seven patients were 10–15 years, 16–20 * Fax: 181-53-437-8714. E-mail address: [email protected] (K. Yokochi).
years, 21–25 years, and 26–30 years, respectively. They were severely physically disabled; sitting, rolling, and locomotion in a prone or supine posture could not be performed alone. Forty-three patients had spastic tetraplegia, and the remaining 21 had a dyskinetic syndrome [12]. The subjects had profound mental retardation; their mental ages were assessed to be less than 2 years. None spoke meaningful words. Sixty-one had epilepsy. Nine patients underwent tracheostomy for respiratory problems. Nineteen patients received tube feeding for dysphagia. Patients who underwent orthopedic surgery to correct joint deformity were excluded from this study. None of the subjects had a degenerative disease with intellectual or motor deterioration. In 18 patients, the brain damage occurred prenatally. Thirteen had multiple minor anomalies which were revealed by physical assessment or an anomaly of the brain that was revealed by neuroimaging. The remaining five were included in this group only because there were no perinatal or postnatal events that would possibly result in brain damage. No patient had a chromosomal aberration. In 28 patients, the brain damage occurred perinatally. Of these, brain damage was caused by complications associated with preterm birth in seven; gestational age was 36 weeks or less. In 17, the cause was neonatal asphyxia; gestational age was 37 weeks or more. Apgar scores were 6 or below at 1 min, and signs of postpartum encephalopathy began during the first 48 h of life as manifested by seizures, lethargy, coma, extensive hypo- or hypertonia, or pathological spontaneous movements. Neonatal bacterial meningitis and kernicterus was the cause in three and one, respectively, of those with perinatal brain damage. In the remaining 18 patients, brain
0387-7604/01/$ - see front matter q 2001 Elsevier Science B.V. All rights reserved. PII: S 0387-760 4(01)00240-6
372
K. Yokochi / Brain & Development 23 (2001) 371–374
damage occurred postnatally between 5 months and 9 years of age; causes were acute encephalopathy or encephalitis in eight, anoxic encephalopathy in five, head trauma in four and bacterial meningitis in one. Joint deformities were assessed at 10 years or longer after the occurrence of brain damage in these 18 patients. Scoliosis was evaluated as a representative deformity of the spine. It was confirmed to be structural by physical examination, and vertebral anomalies were not recognized in radiographs. Side of convexity, type of curve (C- or Sshaped), and apex vertebra were examined as characteristics of the curve [5]. Anteroposterior radiographs of the chest and/ or abdomen in the supine position taken within 3 years before the time of assessment and physical examination were used for evaluating scoliosis, because radiographs of the spine were not taken regularly as a policy of the author’s hospital. When the curve roughly measured by the Cobb method was less than 208, scoliosis was judged to be ‘minimal’. Abduction or adduction contracture was evaluated as a representative deformity of the hips. The range of abduction or adduction of each hip was measured with the hips flexed 908 or with the hips flexed maximally less than 908. When one hip could be abducted 108 or less and adducted 408 or more (adducted side), and the other hip (abducted side) could be abducted 408 or more and adducted 208 or less, the hips were judged to be ‘windblown’. When both hips could be adducted 308 or more and abducted 208 or less, they were judged to be ‘adducted’. When both hips could be abducted 308 or more and adducted 08 or less, the hips were judged to be ‘abducted’. When the ranges of hip-motion did not correspond to the above-mentioned three patterns, hips were classified as ‘other’. Hip dislocation was not examined in this study, because radiographs of the hips were not taken regularly as a policy of the author’s hospital. Flexion or extension contracture was evaluated as a representative deformity of the knees. When both knees could be extended 2308 or less with the hips extended maximally, knees were judged to be ‘flexed’ When both knees could be flexed 408 or less, they were judged to be ‘extended’. When the range of knee-motion did not correspond to either of the above-mentioned two patterns, a classification of ‘other’ was given. Plantarflexion or dorsiflexion contracture was evaluated as a representative deformity of the feet. When both feet could be dorsiflexed 0 degree or less with the knees extended maximally, feet were judged to be ‘plantarflexed’. When both feet could be planrtarflexed 108 or less, they were judged to be ‘dorsiflexed’. A classification of ‘other’ was assigned when ranges of motion of the feet did not correspond to either of these two patterns. Flexion contracture of the elbow and flexion or extension contracture of the wrist were evaluated as representative deformities of the upper extremities. When the elbow could be extended -308 or less, the elbow was judged to be ‘flexed’. When the wrist could be extended 0 degree or less, the wrist was judged to be ‘flexed’. When the wrist could be flexed 08 or
less, the wrist was judged to be ‘extended’. When both elbows were flexed and both wrists were flexed or extended, the upper extremities were termed as ‘contracted arms’. When both elbows were not flexed and both wrists were either flexed or extended, the designation of ‘contracted wrists’ was assigned. When both elbows and wrists did not have the above-mentioned contractures, the upper extremities were not judged to be contracted and were given the designation of ‘none’. When the ranges of elbow and wrist motions did not correspond to any of the above-mentioned three patterns, the upper extremities were classified as ‘other’. 3. Results Joint deformity patterns and clinical profiles of the subjects are shown in Tables 1 and 2. The majority of subjects, 48 of the 64 had C-shaped scoliosis. Of these 48 subjects, 33 had convexity to the left. Judging from the apex vertebra, C-shaped scoliosis was thoracolumbar in 30 patients, lumbar in 17, and thoracic in 1. A minority of the patients, i.e. 16, had S-shaped or minimal scoliosis. Windblown and adducted deformity was found in 20 and 22 subjects, respectively, and were the major types of hip deformity. Fifteen of the 20 patients with windblown hip deformity had C-shaped scoliosis. In those 15 patients the convex side of scoliosis and the adducted side of the windblown hips were same. In four patients with S-shaped scoliosis, the convex side of the lower curve and the adducted side of windblown hip deformity were opposite. Of the 64 patients, 40 had a flexed knee deformity. Only four patients had an extended knee deformity associated with an adducted hip deformity. In the majority of patients (37/64), the ankle deformity was classified as ‘other’. Plantarflexed ankle deformity was not evident in patients with the windblown hip deformity or in those with the abducted hip deformity. The dorsiflexed ankle deformity was predominant in patients with windblown hips. Nine patients had C-shaped scoliosis with the convexity toward the adducted side of the hips, windblown hips, flexed knees, and dorsiflexed ankles, which was the most common combination of spine, hip, knee and foot deformities among the subjects. The deformity pattern of the upper extremities was variable. The joint deformity pattern in patients with spastic tetraplegia was not significantly different from that in patients with dyskinesia (chi-squared test, Table 2). 4. Discussion C-shaped scoliosis was found in many of our subjects in agreement with previous reports of spine deformity in cerebral palsied patients [3–11]. The spine of nonambulatory, especially bedridden, patients with central motor disorders is thought to develop mostly C-shaped scoliosis. Patients who are bedridden and have C-shaped scoliosis are known to be at risk of progression of the curve with advancing age
K. Yokochi / Brain & Development 23 (2001) 371–374
373
Table 1 Joint deformity patterns and clinical profiles in study subjects Patterns of hip deformity
Windblown (n ¼ 20) a
Adducted (n ¼ 22)
Abducted (n ¼ 7)
Others (n ¼ 15)
Total (n ¼ 64)
Spine C (R:L) b Sc Minimal
15 (4:11) d 4e 1
14 (3:11) 3 5
6 (4:2) 1 0
13 (4:9) 1 1
48 (15:33) 9 7
16 0 4 0 12 8
11 4 7 4 4 14
7 0 0 0 4 3
6 0 9 2 1 12
40 4 20 6 21 37
Upper extremities Contracted arms Contracted wrists Other None
4 3 7 6
3 6 8 5
2 3 1 1
1 5 2 7
10 17 18 19
Cause of brain damage Prenatal Perinatal (asphyxia) Postnatal
7 8 (5) 5
5 10 (9) 7
1 5 (2) 1
5 5 (1) 5
18 28 (17) 18
17 5
5 2
9 6
43 21
Lower extremities Knee
Ankle
Clinical features Spastic Dyskinetic a b d c e
Flexed Extended Other Plantarflexed Dorsiflexed Other
12 8
Adductive side of windblown hips is right in 6 and left in 14. (R:L), side of the convexity of C-shaped scoliosis. Adductive side of windblown hips is same as the side of the convexity of C-shaped scoliosis in all of the patients. Side of the convexity of lower curve is right in 6 and left in 3. Adductive side of windblown hips was right in 2 and left in 2; it is opposite to the side of the convexity of the lower curve in all four patients.
[7,8,10,11]. Differing from previous reports [3,4,9,11], a curve with the convexity to the left was commonly seen in patients with C-shaped scoliosis in this study. Although the cause of this difference cannot be determined in this study, this difference may result from the degree of gross motor function among patients in various studies. Our study subjects were more severely disabled than those in previous reports [3,4,9,11] in that they were unable to sit or to locomote in a prone or supine posture. Patients with severe impairment of gross motor function resulting from brain lesions may manifest C-shaped scoliosis with the convexity to the left. Among other possibilities, different curves may result from different kinds of brain lesions or the various postures in which mothers or caregivers usually place the patient. Windblown hips associated with hip dislocation on the adducted side have been noted in nonambulatory patients with cerebral palsy [3,4,6,7]. Windblown hips have also been reported to accompany variable forms of scoliosis [3,4,6,7]. In this study, S-shaped scoliosis was found only in a small number of subjects with windblown hips, differing from previous reports [3,4,6]. An additional difference was that the relationship between the laterality of windblown hips
and the convexity of curvatures was relatively simple; the adducted side of windblown hips was the same as the side of the convexity of C-shaped scoliosis and was opposite to the side of the convexity of the lower curve in S-shaped scoliosis. This difference may also result from the difference in subjects between our study and other studies. Most patients with windblown hips inevitably have instability in sitting, because C-shaped scoliosis with the convexity to the adducted side and the thigh form a longer curvature. Although the hypothesis that hyperactivity of the iliopsoas results in windblown hips and later in scoliosis may explain the development of a lumbar curve with the convexity to the abducted side [6], it cannot apply to the development of C-shaped scoliosis with the convexity to the adducted side. The combinations of hip and spine deformities were variable in this study. The mechanisms for development of scoliosis may not be uniform and may be different from those for development of hip deformity in patients with severe physical disability. Adducted hip deformity mostly associated with flexed knees and C-shaped scoliosis are common hip deformities in patients with severe physical disability. Hip adduction and knee flexion are also likely to be found in children
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Table 2 Joint deformity patterns in patients with spastic tetraplegia and in those with dyskinesia Clinical features
Spastic (n ¼ 43)
Dyskinetic (n ¼ 21)
Total (n ¼ 64)
Spine C (R:L) a S Minimal
30 (11:19) 7 6
18 (4:14) 2 1
48 (15:33) 9 7
12 17 5 9 30 2 11 4 17 22
8 5 2 6 10 2 9 2 4 15
20 22 7 15 40 4 20 6 21 37
Upper extremities Contracted arms Contracted wrists Other None
8 13 16 6
2 4 2 13
10 17 18 19
Cause of brain damage Prenatal Perinatal (asphyxia) Postnatal
6 25 (17) 12
12 3 (0) 6
18 28 (17) 18
Lower extremities Hip
Knee
Ankle
a
Windblown Adducted Abducted Other Flexed Extended Other Plantarflexed Dorsiflexed Other
(R:L), side of the convexity of C-shaped scoliosis.
with spastic diplegia [1,2]; both sides of the lower extremities are affected by spasticity in such children. These deformities are thought to result from a pathophysiological mechanism similar to that of spastic diplegia, although they have been noted in patients with dyskinesia. Abducted hip deformity associated with flexed knees and extended knee deformity associated with adducted hips were found in a minority of patients with severe physical disability, although no clinical profiles corresponded to these two conditions. Although the plantarflexed ankle deformity is common in children with spastic diplegia [1,2], it is found in only a minority of patients with severe physical disability. Complex mechanisms may result in the ankle deformity. This study indicates that joint deformity patterns are variable among patients with severe physical disability. Clearly, more research on motor symptoms of severe central motor disability is needed to establish effective methods of physical therapy and nursing for patients according to the type of joint deformity pattern. References [1] Bleck EE. Orthopedic management of cerebral palsy, Clinics in developmental medicine, no. 99/100. London: MacKeith Press, 1987.
[2] Rang M. Cerebral palsy. In: Morrissy RT, editor. Lovell and Winter’s pediatric orthopedics, 3rd ed. Philadelphia, PA: Lippincott, 1990. pp. 465–506. [3] Samilson RL, Bechard R. Scoliosis in cerebral palsy: incidence, distribution of curve patterns, natural history, and thought of etiology. Curr Pract Orthop Surg 1973;5:183–205. [4] Madigan RR, Wallace SL. Scoliosis in the institutionalized cerebral palsy population. Spine 1981;6:583–590. [5] Lonstein JE, Akbarnia BA. Operative treatment of spinal deformities in patients with cerebral palsy or mental retardation. An analysis of one hundred and seven cases. J Bone Joint Surg Am 1983;65:43–55. [6] Letts M, Shapiro L, Mulder K, Klassen O. The windblown hip syndrome in total body cerebral palsy. J Pediatr Orthop 1984;4:55–62. [7] Okamura Y, Tohno S, Harata S, Moriyama A, Hayashi A, Nara K, et al. Scoliosis in cerebral palsy (in Japanese). Seikeigeka (Tokyo) 1985;36:23–30. [8] Thometz JG, Simon SR. Progression of scoliosis after skeletal maturity in institutionalized adults who have cerebral palsy. J Bone Joint Surg Am 1988;70:1290–1296. [9] Nagaya M, Akashi K, Tomita M, Kawamura T. The deformities of foot and spine in institutionalized children with mental and physical handicaps (in Japanese). Sogo Rehabil (Tokyo) 1991;19:109–114. [10] Majd ME, Muldowny DS, Holt RT. Natural history of scoliosis in the institutionalized adult cerebral palsy population. Spine 1997;22:1461– 1466. [11] Saito N, Ebara S, Ohotsuka K, Kumeta H, Takaoka K. Natural history of scoliosis in spastic cerebral palsy. Lancet 1998;351:1687–1692. [12] Hagberg B, Hagberg G, Olow I. The changing panorama of cerebral palsy in Sweden, 1954–1970. Acta Paediatr Scand 1975;64:187–192.