THE INCIDENCE AND NATURAL HISTORY OF SCOLIOSIS IN RETT SYNDROME George S . Bassett Vernon T. Tolo
Rett syndrome is a progressive neurological disorder of unknown etiology which develops in females after apparently normal psychomotor development for the first six months of life (Trevathan et al. 1988). Characteristic features include autism, dementia, gait apraxia, truncal ataxia, loss of verbal and hand skills, and stereotypic hand-movements. These patients become microcephalic and in most cases develop seizures. They are hypotonic initially, and eventually develop hyperreflexia and spasticity. A variety of musculoskeletal abnormalities have been reported in girls with Rett syndrome, including scoliosis, kyphosis, joint contractures, hip subluxation or dislocation, toe-walking , and planovalgus foot deformities. Scoliosis is the most common orthopaedic problem encountered in this syndrome. The incidence of spinal deformities ranges from 35 to 100 per cent according t o various reports (Hagberg and Witt-Engerstrom 1986, Naidu et al. 1986, Hennessy and Haas 1988, Keret et al. 1988, Loder et al. 1989). The reported differences appear to be related to the age of the patients studied. We undertook this survey in an attempt to define more accurately the incidence and natural history of spinal deformities in girls with Rett syndrome.
Material and method In 1987, a questionnaire was mailed to 350 American members of the International Rett Syndrome Association who have involved daughters. Information was obtained about the age of the patient, the age at diagnosis of Rett syndrome, the presence of scoliosis or kyphosis and the types of treatment used for the spinal deformities. After the questionnaires had been returned, the patients' physicians were contacted to obtain appropriate medical records and copies of roentgenograms of the spine. Of necessity, the study was heavily dependent on the accuracy of medical records supplied to us. The diagnosis of Rett syndrome was made by a neurologist in the majority of cases, but we were not able to document specifically the criteria used, nor the accuracy of the diagnosis, apart from what was detailed in the medical records or from the parents' responses. We had access to at least one set of spinal radiographs for two-thirds of the patients with scoliosis or kyphosis. Obviously we were not able to control the positioning of the patient for these radiographs, and standing, sitting and supine radiographs were sent to us. For the patient's for whom radiographs were not available, we had to rely on the written reports of the consulting orthopaedic
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1987 Retts Survey
150
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106
2-5
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11-15
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Age
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Fig. I . Age-groups of 258 paiienIs wiih Rett syndrome surveyed in 1987.
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surgeons. For patients with scoliosis but with no radiographs available, we accepted the reports of the orthopaedic surgeons if their records included the specific limits and magnitude of the curve, measured by the Cobb method. An increase in the Cobb angle of at least 7" was necessary t o document curve progression. In spite of all these limitations, satisfactory information was obtained for 258 of the possible 350 females with Rett syndome (74 per cent). The results were analyzed according t o the four age-groups two t o five years (group l), six to 10 years (group 2), 1 1 to 15 years (group 3), and 16 years or older (group 4).
Results
964
The ages of the 258 patients ranged from 2 years 2 months to 31 years 5 months. 19 per cent were in group 1 , 41 per cent in group II,26 per cent in group 3 and 14 per cent in group 4 (Fig. 1). Scoliosis or kyphosis was present in 119 patients (48 per cent), with curves ranging in size from 1 1 t o 100". Spinal orthoses had been prescribed and worn by 46 patients with curves ranging from 1 1 to 56" (average 31"). In 17 of these patients (38 per cent) the curve had progressed by an average of 26" (range 10 to 45"), in spite of bracing. Fusions had been performed or were scheduled for 26 patients with curves ranging from 40 to 120" (average 62") preoperatively. Three patients with excessive kyphosis ranging from 65 to 100" (average 86") had undergone surgery. The average age for patients in group 1
was 3 years 9 months, the youngest patient being 2 years 2 months. Only four in this group (8 per cent) had evidence of scoliosis or excessive kyphosis. Two girls had been braced for 28" scoliotic curves, one of whom had documented progression of 14" before treatment. The third patient was being observed for a 13" thoracolumbar curve, and the fourth patient had been started in a bracing program for thoracic kyphosis measuring 83". No patients in this group had had surgery. Group 2 contained the largest number of respondents in this survey. The average age was 7 years 5 months. There was no evidence of scoliosis in 65 girls (61 per cent). 25 patients with scoliosis had not had any treatment for curves averaging 21 " (range 1 1 to 35"). 13 patients were in a bracing program for curves ranging from 1 1 to 46" (average 29"). Three of the 13 had documented progression averaging 18" (range 10 to 31") during brace treatment, and one of these patients had been fused for a 55" curve. Another patient had surgery for scoliosis of 70" and the scoliosis of a third patient scheduled for surgery had progressed from 36 to 52". One girl with 80" scoliosis was being observed and one with kyphosis measuring 90" had undergone surgical correction. Forty-four of 68 girls in group 3 (66 per cent) had scoliosis. The average age of this group was 12 years 2 months. 22 girls had not had any treatment for their scoliosis; their curves ranged from 10 to 100" (average 31"). 16 patients were in a bracing program, with curves ranging from 18 to 56" (average 32"). Eight of these 16 had evidence of progression during their brace treatment, averaging 32" (range 10 to 45") and seven of these underwent surgical correction. A total of 12 girls were scheduled for or had already undergone surgery for scoliosis. Preoperative curves ranged from 40 to 90" (average 65"). One patient had surgery for a thoracic kyphosis measuring 96". The age-distribution of the 35 patients in group 4 was 16 to 31 years (average 22 years 6 months). Six patients (17 per cent) had no evidence of scoliosis or excessive kyphosis. No treatment had been started for six patients with scoliosis averaging 29" (range 15 to 47"). Spinal orthoses had
been used for 14 patients in this group, whose curves averaged 30" (range 15 to 48") before bracing. Curve progression during brace treatment was documented in 10 of these patients, ranging from 1 1 to 39" (average 23"). 1 1 patients had had posterior spinal fusion with instrumentation for scoliosis, seven of whom had previously been treated with a brace. Preoperatively, their curves had ranged from 37 to 120" (average 60"). Five patients in this group had excessive thoracic kyphosis, ranging from 65 to 100" (average 85"). Three patients had been braced, without improvement. One patient underwent surgery for kyphosis measuring 75" preoperatively.
Discussion Scoliosis occurs frequently in patients with Rett syndrome, and previous studies have suggested that the occurrence of scoliosis is age-dependant. Naidu el al. (1986) reported scoliosis in 25 of their 70 patients (36 per cent), whose ages ranged from two to 34 years. Loder et al. (1989) documented scoliosis in 42 per cent of their 36 patients, ranging in age from one to 19 years. Eight of 10 patients (80 per cent) were treated for scoliosis in the series reported by Keret et al. (1988): the two who did not have spinal deformity were aged 1 1 and 24 years. Scoliosis occurred in 15 of 16 patients (94 per cent) in the series reported by Hennessy and Haas (1988). Hagberg and Witt-Engerstom (1986) analyzed the physical findings in 29 patients, all of whom were over the age of 13 years: all 29 had scoliosis. We recognize the problems inherent in the present survey, including the possible inclusion of patients who may not fulfil all the classic criteria for Rett syndrome and the reliance on medical records supplied to us by a large number of individual physicians. In spite of these possible deficiencies, however, there is clear evidence that scoliosis increases with advancing age. Scoliosis was present in 8 per cent of the youngest group, increasing to 83 per cent of the oldest group (Fig. 2). While curve progression most typically occurred during the second decade, scoliosis frequently developed between the ages of six and 10 years (39 per cent). Nine patients in group 2 had already
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2-5
6-10 Age
11-15
>15
Fig. 2. Scoliosis according to age-group.
g developed curves measuring >30" at the time of this survey. We recommend a biannual clinical evaluation of the spine for girls with Rett syndrome after the age of five years. Standing or sitting radiographs of the spine, depending on the functional ability of the patient, should be obtained when there is evidence of a spinal deformity. Referral to an orthopaedic surgeon should be made when scoliosis or excessive kyphosis is evident. This study was not intended specifically to address the issue of treatment for these patients. Firm conclusions about the efficacy of bracing cannot be made, since a variety of braces were used for varying durations and compliance could not be determined, nor the appropriateness of brace application and fit. Nevertheless, certain trends emerged. 38 per cent of these patients had evidence of curve progression during brace treatment, and we would anticipate additional brace failures as the younger children approach skeletal maturity. In contrast to idiopathic scoliosis, orthotic treatment may not alter the natural history of scoliosis in patients with Rett syndrome.
s . 9
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2
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6
Accepted for publication 20th March 1990. Authors' Appointments *George S . Bassett, M.D.; Vernon T. Tolo, M.D.; Childrens Hospital Los Angeles, 4650 Sunset Boulevard, Los Angeles, CA 90027. *Correspondence to first author.
965
SUMMARY A survey on spinal deformity was conducted by means of a questionnaire mailed t o the 350 American families who are members of the International Rett Syndrome Association. 258 questionnaires were completed and further information (medical records and radiographs) was received from treating physicians. Scoliosis was present in 119 patients, whose clinical details are discussed. Patients with Rett syndrome frequently develop progressive scoliotic deformities. The incidence increases with age, occurring most commonly during the second decade. Bracing to control curve progression has been largely unsuccessful for adolescent patients. RESUME
L ’incidence et I’histoire naturelle de la scoliose dans le syndrome de Rett
E
8
0
B e
-0
C x v1
t:
2 .-C
Une revue des deformations vertebrales a ete conduite au moyen d’un questionnaire adresse aux 350 familles membres de I’International Rett Syndrome Association. 258 questionnaires furent completes et des indications complementaires (dossiers medicaux et radiographies) furent obtenus des medecins traitants. la scoliose etait presente chez 119 patients, les details cliniques &ant dtcrits. Les sujets presentant un syndrome de Rett developpent frequemment une deformation scoliotique progressive. L’incidence s’accroit avec I’lge, apparaissant le plus habituellement dans la deuxieme decade. Les corsets pour contrdler la progression des courbures ont ete globalement inefficaces chez les adolescents. ZUSAMMENFASSUNG Haufigkeit und Entstehung der Skolise beim Rett Syndrom Anhand eines Fragebogens, der an 350 Familien verschickt wurde, die Mitglieder der internationalen Rett Syndrom Gesellschaft sind, wurde eine Ubersicht iiber,spinale Fehlbildungen erstellt. 258 Gragebogen wurden ausgefiillt und von den behandelnden Arzten wurden weitere Informationen (Krankengeschichte und Rontgenbefunde) eingeholt. 119 Patienten hatten eine Skoliose, die klinischen Einzeltheiten dazu werden diskutiert. Patienten mit Rett Syndrom entwickeln haufig skoliotische Fehlbildungen. Die Haufigkeit steigt mit zunehmendem Alter, in der Regel treten sie in der zweiten Dekade auf. Der Versuch, durch eine Versteifung das Fortschreiten der Skoliose zu verhindern, war bei heranwachsenden Patienten weitesgehend erfolglos. RESUMEN Incidencia e historia de la escoliosis en el sindrome de Rett Se realizo un estudio de la deformidad espinal por medio de un cuestionario enviado por correo a 350 families americanas miembros de la Asociacion Internacional del Sindrome de Rett. Se completaron 258 cuestionarios y se obtuvo informacion mas completa a partir de 10s medicos que trataban a 10s niflos. La escoliosis estaba presente en 119 pacientes; se discuten 10s detalles clinicos. Los pacientes con sindrome de Rett con frecuencia desarrollan deformidades escolioticas. La incidencia aumenta con la edad, teniendo lugar mas corrientemente durante la segunda decada. La aplicacion de corses para controlar la progresion ha sido ampliamente ineficaz en pacientes adolescentes.
References Bassett, G. S., Bunnell, W. P., MacEwen, G. D. (1986) ‘Treatment of idiopathic scoliosis with the Wilmington brace-results in patients with a twenty to thirty-nine degree curve.’ Journal of Bone and Joint Surgery, 68A, 602-605. Hagberg, B., Witt-Engerstrom, 1. (1986) ‘Rett syndrome: a suggested staging system for describing impairment profile with increasing age towards adolescence.’ American Journal of Medical Genetics, 24 (Suppl. I ) , 47-60. Hennessy, M. J., Haas, R. H. (1988) ‘The orthopedic management of Rett syndrome.’ Journal of Child Neurology, 3 (Suppl.), s43447. Keret, D., Bassett, G. S., Bunnell, W. P., Marks,
966
H . G. (1988) ‘Scoliosis in Rett syndrome.’ Journal of Pediatric Orthopedics, 8, 138-142. Loder, R. T., Lee, C. L., Richards, B. S. (1989) ‘Orthopedic aspects of Rett syndrome: a multicenter review.’ Journal of Pediatric Orthopedics, 9, 557-562. Naidu, S., Murphy, M., Moser, H. W., Rett, A. (1986) ‘Rett syndrome-natural history in 70 cases.’ American Journal of Medical Genetics, 24 (SUPPI. l), 61-72. Trevathan, E., Moser, E., Opitz, J. M., Percy, A. K., Naidu, S., Holm, J. A., Boring, C. C., Jansen, R. S., Yeargin-Allsop, M., Adams, M. J . (1988) ‘Diagnostic criteria for Rett syndrome.’ Annals of Neurology, 23, 425-428.
Rett syndrome is a progressive neurological disorder of unknown etiology which develops in females after apparently normal psychomotor development for the first six months of life (Trevathan et al. 1988). Characteristic features include autism, dementia, gait apraxia, truncal ataxia, loss of verbal and hand skills, and stereotypic hand-movements. These patients become microcephalic and in most cases develop seizures. They are hypotonic initially, and eventually develop hyperreflexia and spasticity. A variety of musculoskeletal abnormalities have been reported in girls with Rett syndrome, including scoliosis, kyphosis, joint contractures, hip subluxation or dislocation, toe-walking , and planovalgus foot deformities. Scoliosis is the most common orthopaedic problem encountered in this syndrome. The incidence of spinal deformities ranges from 35 to 100 per cent according t o various reports (Hagberg and Witt-Engerstrom 1986, Naidu et al. 1986, Hennessy and Haas 1988, Keret et al. 1988, Loder et al. 1989). The reported differences appear to be related to the age of the patients studied. We undertook this survey in an attempt to define more accurately the incidence and natural history of spinal deformities in girls with Rett syndrome.
Material and method In 1987, a questionnaire was mailed to 350 American members of the International Rett Syndrome Association who have involved daughters. Information was obtained about the age of the patient, the age at diagnosis of Rett syndrome, the presence of scoliosis or kyphosis and the types of treatment used for the spinal deformities. After the questionnaires had been returned, the patients' physicians were contacted to obtain appropriate medical records and copies of roentgenograms of the spine. Of necessity, the study was heavily dependent on the accuracy of medical records supplied to us. The diagnosis of Rett syndrome was made by a neurologist in the majority of cases, but we were not able to document specifically the criteria used, nor the accuracy of the diagnosis, apart from what was detailed in the medical records or from the parents' responses. We had access to at least one set of spinal radiographs for two-thirds of the patients with scoliosis or kyphosis. Obviously we were not able to control the positioning of the patient for these radiographs, and standing, sitting and supine radiographs were sent to us. For the patient's for whom radiographs were not available, we had to rely on the written reports of the consulting orthopaedic
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z! 5 2
e
2 2 is
2
u .2
.':
h
s . 5 s \r
,o z
6 963
1987 Retts Survey
150
-
7 I
r
106
2-5
6-10
11-15
>15
Age
B
2 -0
Fig. I . Age-groups of 258 paiienIs wiih Rett syndrome surveyed in 1987.
x
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2
.-C
surgeons. For patients with scoliosis but with no radiographs available, we accepted the reports of the orthopaedic surgeons if their records included the specific limits and magnitude of the curve, measured by the Cobb method. An increase in the Cobb angle of at least 7" was necessary t o document curve progression. In spite of all these limitations, satisfactory information was obtained for 258 of the possible 350 females with Rett syndome (74 per cent). The results were analyzed according t o the four age-groups two t o five years (group l), six to 10 years (group 2), 1 1 to 15 years (group 3), and 16 years or older (group 4).
Results
964
The ages of the 258 patients ranged from 2 years 2 months to 31 years 5 months. 19 per cent were in group 1 , 41 per cent in group II,26 per cent in group 3 and 14 per cent in group 4 (Fig. 1). Scoliosis or kyphosis was present in 119 patients (48 per cent), with curves ranging in size from 1 1 t o 100". Spinal orthoses had been prescribed and worn by 46 patients with curves ranging from 1 1 to 56" (average 31"). In 17 of these patients (38 per cent) the curve had progressed by an average of 26" (range 10 to 45"), in spite of bracing. Fusions had been performed or were scheduled for 26 patients with curves ranging from 40 to 120" (average 62") preoperatively. Three patients with excessive kyphosis ranging from 65 to 100" (average 86") had undergone surgery. The average age for patients in group 1
was 3 years 9 months, the youngest patient being 2 years 2 months. Only four in this group (8 per cent) had evidence of scoliosis or excessive kyphosis. Two girls had been braced for 28" scoliotic curves, one of whom had documented progression of 14" before treatment. The third patient was being observed for a 13" thoracolumbar curve, and the fourth patient had been started in a bracing program for thoracic kyphosis measuring 83". No patients in this group had had surgery. Group 2 contained the largest number of respondents in this survey. The average age was 7 years 5 months. There was no evidence of scoliosis in 65 girls (61 per cent). 25 patients with scoliosis had not had any treatment for curves averaging 21 " (range 1 1 to 35"). 13 patients were in a bracing program for curves ranging from 1 1 to 46" (average 29"). Three of the 13 had documented progression averaging 18" (range 10 to 31") during brace treatment, and one of these patients had been fused for a 55" curve. Another patient had surgery for scoliosis of 70" and the scoliosis of a third patient scheduled for surgery had progressed from 36 to 52". One girl with 80" scoliosis was being observed and one with kyphosis measuring 90" had undergone surgical correction. Forty-four of 68 girls in group 3 (66 per cent) had scoliosis. The average age of this group was 12 years 2 months. 22 girls had not had any treatment for their scoliosis; their curves ranged from 10 to 100" (average 31"). 16 patients were in a bracing program, with curves ranging from 18 to 56" (average 32"). Eight of these 16 had evidence of progression during their brace treatment, averaging 32" (range 10 to 45") and seven of these underwent surgical correction. A total of 12 girls were scheduled for or had already undergone surgery for scoliosis. Preoperative curves ranged from 40 to 90" (average 65"). One patient had surgery for a thoracic kyphosis measuring 96". The age-distribution of the 35 patients in group 4 was 16 to 31 years (average 22 years 6 months). Six patients (17 per cent) had no evidence of scoliosis or excessive kyphosis. No treatment had been started for six patients with scoliosis averaging 29" (range 15 to 47"). Spinal orthoses had
been used for 14 patients in this group, whose curves averaged 30" (range 15 to 48") before bracing. Curve progression during brace treatment was documented in 10 of these patients, ranging from 1 1 to 39" (average 23"). 1 1 patients had had posterior spinal fusion with instrumentation for scoliosis, seven of whom had previously been treated with a brace. Preoperatively, their curves had ranged from 37 to 120" (average 60"). Five patients in this group had excessive thoracic kyphosis, ranging from 65 to 100" (average 85"). Three patients had been braced, without improvement. One patient underwent surgery for kyphosis measuring 75" preoperatively.
Discussion Scoliosis occurs frequently in patients with Rett syndrome, and previous studies have suggested that the occurrence of scoliosis is age-dependant. Naidu el al. (1986) reported scoliosis in 25 of their 70 patients (36 per cent), whose ages ranged from two to 34 years. Loder et al. (1989) documented scoliosis in 42 per cent of their 36 patients, ranging in age from one to 19 years. Eight of 10 patients (80 per cent) were treated for scoliosis in the series reported by Keret et al. (1988): the two who did not have spinal deformity were aged 1 1 and 24 years. Scoliosis occurred in 15 of 16 patients (94 per cent) in the series reported by Hennessy and Haas (1988). Hagberg and Witt-Engerstom (1986) analyzed the physical findings in 29 patients, all of whom were over the age of 13 years: all 29 had scoliosis. We recognize the problems inherent in the present survey, including the possible inclusion of patients who may not fulfil all the classic criteria for Rett syndrome and the reliance on medical records supplied to us by a large number of individual physicians. In spite of these possible deficiencies, however, there is clear evidence that scoliosis increases with advancing age. Scoliosis was present in 8 per cent of the youngest group, increasing to 83 per cent of the oldest group (Fig. 2). While curve progression most typically occurred during the second decade, scoliosis frequently developed between the ages of six and 10 years (39 per cent). Nine patients in group 2 had already
, 1 1 1
W W o\
Scoliosis
100 %
50
--
'
8 3 # 1
m
W
m
m hi
66%
8YQ n
2-5
6-10 Age
11-15
>15
Fig. 2. Scoliosis according to age-group.
g developed curves measuring >30" at the time of this survey. We recommend a biannual clinical evaluation of the spine for girls with Rett syndrome after the age of five years. Standing or sitting radiographs of the spine, depending on the functional ability of the patient, should be obtained when there is evidence of a spinal deformity. Referral to an orthopaedic surgeon should be made when scoliosis or excessive kyphosis is evident. This study was not intended specifically to address the issue of treatment for these patients. Firm conclusions about the efficacy of bracing cannot be made, since a variety of braces were used for varying durations and compliance could not be determined, nor the appropriateness of brace application and fit. Nevertheless, certain trends emerged. 38 per cent of these patients had evidence of curve progression during brace treatment, and we would anticipate additional brace failures as the younger children approach skeletal maturity. In contrast to idiopathic scoliosis, orthotic treatment may not alter the natural history of scoliosis in patients with Rett syndrome.
s . 9
5
$
2
9
6
Accepted for publication 20th March 1990. Authors' Appointments *George S . Bassett, M.D.; Vernon T. Tolo, M.D.; Childrens Hospital Los Angeles, 4650 Sunset Boulevard, Los Angeles, CA 90027. *Correspondence to first author.
965
SUMMARY A survey on spinal deformity was conducted by means of a questionnaire mailed t o the 350 American families who are members of the International Rett Syndrome Association. 258 questionnaires were completed and further information (medical records and radiographs) was received from treating physicians. Scoliosis was present in 119 patients, whose clinical details are discussed. Patients with Rett syndrome frequently develop progressive scoliotic deformities. The incidence increases with age, occurring most commonly during the second decade. Bracing to control curve progression has been largely unsuccessful for adolescent patients. RESUME
L ’incidence et I’histoire naturelle de la scoliose dans le syndrome de Rett
E
8
0
B e
-0
C x v1
t:
2 .-C
Une revue des deformations vertebrales a ete conduite au moyen d’un questionnaire adresse aux 350 familles membres de I’International Rett Syndrome Association. 258 questionnaires furent completes et des indications complementaires (dossiers medicaux et radiographies) furent obtenus des medecins traitants. la scoliose etait presente chez 119 patients, les details cliniques &ant dtcrits. Les sujets presentant un syndrome de Rett developpent frequemment une deformation scoliotique progressive. L’incidence s’accroit avec I’lge, apparaissant le plus habituellement dans la deuxieme decade. Les corsets pour contrdler la progression des courbures ont ete globalement inefficaces chez les adolescents. ZUSAMMENFASSUNG Haufigkeit und Entstehung der Skolise beim Rett Syndrom Anhand eines Fragebogens, der an 350 Familien verschickt wurde, die Mitglieder der internationalen Rett Syndrom Gesellschaft sind, wurde eine Ubersicht iiber,spinale Fehlbildungen erstellt. 258 Gragebogen wurden ausgefiillt und von den behandelnden Arzten wurden weitere Informationen (Krankengeschichte und Rontgenbefunde) eingeholt. 119 Patienten hatten eine Skoliose, die klinischen Einzeltheiten dazu werden diskutiert. Patienten mit Rett Syndrom entwickeln haufig skoliotische Fehlbildungen. Die Haufigkeit steigt mit zunehmendem Alter, in der Regel treten sie in der zweiten Dekade auf. Der Versuch, durch eine Versteifung das Fortschreiten der Skoliose zu verhindern, war bei heranwachsenden Patienten weitesgehend erfolglos. RESUMEN Incidencia e historia de la escoliosis en el sindrome de Rett Se realizo un estudio de la deformidad espinal por medio de un cuestionario enviado por correo a 350 families americanas miembros de la Asociacion Internacional del Sindrome de Rett. Se completaron 258 cuestionarios y se obtuvo informacion mas completa a partir de 10s medicos que trataban a 10s niflos. La escoliosis estaba presente en 119 pacientes; se discuten 10s detalles clinicos. Los pacientes con sindrome de Rett con frecuencia desarrollan deformidades escolioticas. La incidencia aumenta con la edad, teniendo lugar mas corrientemente durante la segunda decada. La aplicacion de corses para controlar la progresion ha sido ampliamente ineficaz en pacientes adolescentes.
References Bassett, G. S., Bunnell, W. P., MacEwen, G. D. (1986) ‘Treatment of idiopathic scoliosis with the Wilmington brace-results in patients with a twenty to thirty-nine degree curve.’ Journal of Bone and Joint Surgery, 68A, 602-605. Hagberg, B., Witt-Engerstrom, 1. (1986) ‘Rett syndrome: a suggested staging system for describing impairment profile with increasing age towards adolescence.’ American Journal of Medical Genetics, 24 (Suppl. I ) , 47-60. Hennessy, M. J., Haas, R. H. (1988) ‘The orthopedic management of Rett syndrome.’ Journal of Child Neurology, 3 (Suppl.), s43447. Keret, D., Bassett, G. S., Bunnell, W. P., Marks,
966
H . G. (1988) ‘Scoliosis in Rett syndrome.’ Journal of Pediatric Orthopedics, 8, 138-142. Loder, R. T., Lee, C. L., Richards, B. S. (1989) ‘Orthopedic aspects of Rett syndrome: a multicenter review.’ Journal of Pediatric Orthopedics, 9, 557-562. Naidu, S., Murphy, M., Moser, H. W., Rett, A. (1986) ‘Rett syndrome-natural history in 70 cases.’ American Journal of Medical Genetics, 24 (SUPPI. l), 61-72. Trevathan, E., Moser, E., Opitz, J. M., Percy, A. K., Naidu, S., Holm, J. A., Boring, C. C., Jansen, R. S., Yeargin-Allsop, M., Adams, M. J . (1988) ‘Diagnostic criteria for Rett syndrome.’ Annals of Neurology, 23, 425-428.
