Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype

Wijngaarden, Roderick F. A. de Lind van; Klerk, Luuk W. L. de; Festen, Dederieke; Hokken-Koelega, Anita

doi:10.1136/adc.2007.123836

Cited by 60 publications

(52 citation statements)

References 30 publications

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“…La literatura reporta que en pacientes con esta afección, la escoliosis se presenta entre un 15% y un 86% 2,11,12 , siendo la frecuencia mayor a mayor edad 13 . No podemos estabelecer prevalencia de escoliosis entre los portadores de este Sindrome en Chile ,dado que se refieren al Departamento de Escoliosis sólo aquellos portadores de deformidad de columna y no existen estadísticas nacionales.…”

Section: Materials Y Metodosunclassified

“…Al momento del diagnóstico de escoliosis, el 44% de los pacientes presenta curvas sobre 20º 13 y sólo el 10% sobre los 40º 14 , siendo generalmente niños mayores de 12 años 15 . En nuestros pacientes, extrañamente fueron los pacientes de menor edad quienes presentaron ángulos iniciales mayores, es asi como el caso de 3.3 años tenía 83º y el de 6.6 años tenía 72º (Tabla 1).…”

Section: Materials Y Metodosunclassified

“…Los reportes señalan que entre el 20% y el 44% de los pacientes tienen curvas mayores a 20º 13,23 , por lo que pudieran requerir tratamento ortopédico y sólo el 10% tiene curvas sobre los 40º 14 , por lo que requerirían tratamento quirúrgico, siendo la mayoría dobles curvas. Como no tenemos datos del universo del que salen nuestros pacientes, no podemos estabelecer frecuencias.…”

Section: Materials Y Metodosunclassified

“…Como no tenemos datos del universo del que salen nuestros pacientes, no podemos estabelecer frecuencias. La prevalencia de escoliosis publicada es de 30% en menores de 10 años y 80% en adolescentes 13 . Algunos autores describen asociación entre escoliosis y mal alineamiento de extremidades o sexo femenino 2 .…”

Section: Materials Y Metodosunclassified

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Escoliosis y síndrome de Prader-Willi: a propósito de 5 casos intervenidos quirúrgicamente

Pedrals

Marcuson

Gallegos³

2012

Coluna/Columna

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RESUMENObjetivo: Analizar los resultados y las complicaciones del tratamiento quirúrgico de la escoliosis, en pacientes portadores del Síndrome de Prader-Willi, para verificar si se justifica este tipo de tratamiento. Métodos: Análisis retrospectivo de las fichas clínicas y radiografías de los cinco pacientes portadores del Síndrome de Prader-Willi que consultaron en el Departamento de Escoliosis entre los años 2005 y 2010, y fueron operados. Resultados: La edad de la consulta inicial fue en promedio 3,6 años; el promedio de escoliosis fue 61º, el que aumentó a 65º al momento de la cirugía. El promedio de operaciones realizadas fue 2,8 por paciente, lográndose una corrección promedio de 35%. Los motivos de las reintervenciones fueron pérdida de corrección en 4 casos (28,5%), aflojamiento de ganchos en 2 (14,3%) y xifosis referente a la instrumentación en uno (7,1%) No hubo complicaciones graves. Conclusiones: Aún cuando la literatura muestra autores poco proclives a indicar cirugía en este tipo de pacientes, por la elevada tasa de complicaciones graves, la ausencia de ellas en nuestro medio hace mantener válidos los mismos criterios quirúrgicos que en escoliosis idiopática.

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Section: Materials Y Metodosunclassified

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Escoliosis y síndrome de Prader-Willi: a propósito de 5 casos intervenidos quirúrgicamente

Pedrals

Marcuson

Gallegos³

2012

Coluna/Columna

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“…1,[8][9][10][11][12] One study estimated that between 43% and 66% of those with PWS developed scoliosis. 8 The Queen Elizabeth Hospital for Children in London did a retrospective study that demonstrated that 62% of PWS patients had clinical scoliosis.…”

Section: Introductionmentioning

confidence: 99%

Inclusion of chiropractic care in multidisciplinary management of a child with Prader-Willi syndrome: a case report

Wittman

Vallone

2009

Journal of Chiropractic Medicine

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Objective: The purpose of this paper is to present a case of a child with Prader-Willi syndrome and the observed improvement in the degree of scoliosis, immune function, and behavior documented during the course of her treatment. Clinical Features: A 7-year-old girl presented to Kentuckiana Children's Center with a 15°l umbar levoscoliosis and diagnosis of Prader-Willi syndrome. Intervention and Outcome: The treatment plan consisted of chiropractic adjustments, craniosacral therapy, movement therapy, and nutritional therapy. Over the course of treatment, her muscle strength, tone, and motor activity increased. She improved in coordination of gait and balance. Over the course of 3 years, her scoliosis decreased to 4°to 5°. Improvements in immune function and a reduction in anxiety type behaviors were documented by the parents and doctor of chiropractic over the course of 5 years. Conclusions: This case report describes the improvements and progression of one female child with Prader-Willi syndrome under chiropractic and multidisciplinary care.

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Scoliosis in Prader–Willi syndrome: Effect of growth hormone therapy and value of paravertebral muscle volume by CT in predicting scoliosis progression

Murakami

Obata

Abe

et al. 2012

American J of Med Genetics Pt A

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Growth hormone (GH) therapy is now widely given to Prader-Willi syndrome (PWS) patients to encourage growth in body height and to prevent obesity. Scoliosis, one of the complications in this syndrome, is thought to be accelerated in parallel with a rapid increase in body height, especially during adolescence. To determine whether GH therapy aggravates scoliosis and to identify any factor which might predict the progression of scoliosis, we studied 35 (22 males and 13 female) PWS patients between the ages of 2-16 years on GH therapy whose scoliosis was followed with spinal X-rays every 6 months. Thirteen (37.1%) of 35 patients had scoliosis with a Cobb angle of over 10°. Scoliosis was unchanged in five patients (14.3%), became worse in six (17.1%) and improved in two (5.7%). All 22 (62.9%) of 35 patients who did not have scoliosis did not develop scoliosis with GH therapy. Since abnormal paraspinal muscle development was thought to induce scoliosis, we measured cross-sectional areas of paraspinal muscles by using one slice CT scan at the level of the umbilicus at the level of L4. Since there was a delay in the increase in total paravertebral muscle area and prolonged asymmetry in patients with progressive scoliosis, both were thought to be useful predictors of progressive scoliosis in PWS patients with GH therapy.

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Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype

Abstract: The prevalence of scoliosis in children with Prader-Willi syndrome is high (37.5%). Many children with scoliosis (13%) had undergone brace treatment or surgery. The type of scoliosis is affected by age and trunkLBM/BSA ratio.

Cited by 60 publications

References 30 publications

Escoliosis y síndrome de Prader-Willi: a propósito de 5 casos intervenidos quirúrgicamente

Escoliosis y síndrome de Prader-Willi: a propósito de 5 casos intervenidos quirúrgicamente

Inclusion of chiropractic care in multidisciplinary management of a child with Prader-Willi syndrome: a case report

Scoliosis in Prader–Willi syndrome: Effect of growth hormone therapy and value of paravertebral muscle volume by CT in predicting scoliosis progression

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