Skip to main content
Top
Published in: Journal of Orthopaedic Science 1/2015

01-01-2015 | Original Article

The characteristics of scoliosis in Prader–Willi syndrome (PWS): analysis of 58 scoliosis patients with PWS

Authors: Yutaka Nakamura, Nobuyuki Murakami, Takahiro Iida, Satoru Ozeki, Satoshi Asano, Yutaka Nohara, Toshiro Nagai

Published in: Journal of Orthopaedic Science | Issue 1/2015

Login to get access

Abstract

Background

The purpose of this study was to compare the characteristics of scoliosis in Prader–Willi syndrome (PWS) patients versus idiopathic scoliosis (IS).

Methods

We identified 193 PWS patients. Scoliosis was found in 58 PWS patients, 39 of whom were treated with GH. Fifty-five IS patients were consecutively selected from an outpatient clinic. We investigated 113 patients (58 PWS group, 55 IS group) followed for a minimum of 2 years. The mean age was 17.9 and 16.1 years, respectively. Deformity was measured using Lenke classification, Cobb angle, thoracic kyphosis at T2–5 and T5–12, lumbar lordosis at T12–S1, and sagittal alignment at the C7 plumb line. BMI was also recorded.

Results

According to the Lenke system, patients were classified as (PWS group/IS group): Type 1 (8/26), Type 2 (1/4), Type 3 (3/15), Type 4 (1/0), Type 5 (32/8), and Type 6 (13/2). The average Cobb angles were 32.6° in the PWS and 35.4° in the IS. No significant differences were found for the thoracic kyphosis (T2–5, T5–12), lumbar lordosis (T12–S1) or C7 plumb line between the two groups. BMI was increased in the PWS group not treated previously with GH as compared with the IS group and the PWS group with GH.

Conclusions

Most PWS patients presented with lumbar or thoracolumbar curves (Type 5, 6), whereas IS patients typically had thoracic scoliosis (Type 1, 2, 3).
Literature
1.
go back to reference Vestergaard P, Kristensen K, Bruun JM, Østergaard JR, Heickendorff L, Mosekilde L, Richelsen B. Reduced bone mineral density and increased bone turnover in Prader–Willi syndrome compared with controls matched for sex and body mass index–a cross-sectional study. J Pediatr. 2004;144:614–9.PubMedCrossRef Vestergaard P, Kristensen K, Bruun JM, Østergaard JR, Heickendorff L, Mosekilde L, Richelsen B. Reduced bone mineral density and increased bone turnover in Prader–Willi syndrome compared with controls matched for sex and body mass index–a cross-sectional study. J Pediatr. 2004;144:614–9.PubMedCrossRef
2.
go back to reference Butler MG, Haber L, Mernaugh R, Carlson MG, Price R, Feurer ID. Decreased bone mineral density in Prader–Willi syndrome: comparison with obese subjects. Am J Med Genet. 2001;103:216–22.PubMedCrossRef Butler MG, Haber L, Mernaugh R, Carlson MG, Price R, Feurer ID. Decreased bone mineral density in Prader–Willi syndrome: comparison with obese subjects. Am J Med Genet. 2001;103:216–22.PubMedCrossRef
3.
go back to reference Holm VA, Laurnen EL. Prader–Willi syndrome and scoliosis. Dev Med Child Neurol. 1981;23:192–201.PubMedCrossRef Holm VA, Laurnen EL. Prader–Willi syndrome and scoliosis. Dev Med Child Neurol. 1981;23:192–201.PubMedCrossRef
4.
go back to reference Butler JV, Whittington JE, Holland AJ, Boer H, Clarke D, Webb T. Prevalence of, and risk factors for, physical ill-health in people with Prader–Willi syndrome: a population-based study. Dev Med Child Neurol. 2002;44:248–55.PubMedCrossRef Butler JV, Whittington JE, Holland AJ, Boer H, Clarke D, Webb T. Prevalence of, and risk factors for, physical ill-health in people with Prader–Willi syndrome: a population-based study. Dev Med Child Neurol. 2002;44:248–55.PubMedCrossRef
5.
go back to reference Kroonen LT, Herman M, Pizzutillo PD, Macewen GD. Prader–Willi syndrome: clinical concerns for the orthopaedic surgeon. J Pediatr Orthop. 2006;26:673–9.PubMedCrossRef Kroonen LT, Herman M, Pizzutillo PD, Macewen GD. Prader–Willi syndrome: clinical concerns for the orthopaedic surgeon. J Pediatr Orthop. 2006;26:673–9.PubMedCrossRef
6.
go back to reference Nagai T, Obata K, Ogata T, Murakami N, Katada Y, Yoshino A, Sakazume S, Tomita Y, Sakuta R, Niikawa N. Growth hormone therapy and scoliosis in patients with Prader–Willi syndrome. Am J Med Genet A. 2006;140:1623–7.PubMedCrossRef Nagai T, Obata K, Ogata T, Murakami N, Katada Y, Yoshino A, Sakazume S, Tomita Y, Sakuta R, Niikawa N. Growth hormone therapy and scoliosis in patients with Prader–Willi syndrome. Am J Med Genet A. 2006;140:1623–7.PubMedCrossRef
7.
go back to reference de Lind van Wijngaarden RF, de Klerk LW, Festen DA, Hokken-Koelega AC. Scoliosis in Prader–Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype. Arch Dis Child. 2008;93:1012-6. de Lind van Wijngaarden RF, de Klerk LW, Festen DA, Hokken-Koelega AC. Scoliosis in Prader–Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype. Arch Dis Child. 2008;93:1012-6.
8.
go back to reference Nakamura Y, Nagai T, Iida T, Ozeki S, Nohara Y. Epidemiological aspects of scoliosis in a cohort of Japanese patients with Prader–Willi syndrome. Spine J. 2009;9:809–16.PubMedCrossRef Nakamura Y, Nagai T, Iida T, Ozeki S, Nohara Y. Epidemiological aspects of scoliosis in a cohort of Japanese patients with Prader–Willi syndrome. Spine J. 2009;9:809–16.PubMedCrossRef
9.
go back to reference Lenke LG, Betz RR, Clements D, Merola A, Haher T, Lowe T, Newton P, Bridwell KH, Blanke K. Curve prevalence of a new classification of operative adolescent idiopathic scoliosis: does classification correlate with treatment? Spine. 2002;27:604–11.PubMedCrossRef Lenke LG, Betz RR, Clements D, Merola A, Haher T, Lowe T, Newton P, Bridwell KH, Blanke K. Curve prevalence of a new classification of operative adolescent idiopathic scoliosis: does classification correlate with treatment? Spine. 2002;27:604–11.PubMedCrossRef
10.
go back to reference Sponseller PD, Flynn JM, Newton PO, Marks MC, Bastrom TP, Petcharaporn M, McElroy MJ, Lonner BS, Betz RR, Harms Study Group. The association of patient characteristics and spinal curve parameters with Lenke classification types. Spine. 2012;1(37):1138–41.CrossRef Sponseller PD, Flynn JM, Newton PO, Marks MC, Bastrom TP, Petcharaporn M, McElroy MJ, Lonner BS, Betz RR, Harms Study Group. The association of patient characteristics and spinal curve parameters with Lenke classification types. Spine. 2012;1(37):1138–41.CrossRef
11.
go back to reference Nagai T, Obata K, Ogata T, Murakami N, Katada Y, Yoshino A, Sakazume S, Tomita Y, Sakuta R, Niikawa N. Growth hormone therapy and scoliosis in patients with Prader–Willi syndrome. Am J Med Genet A. 2006;140:1623–7.PubMedCrossRef Nagai T, Obata K, Ogata T, Murakami N, Katada Y, Yoshino A, Sakazume S, Tomita Y, Sakuta R, Niikawa N. Growth hormone therapy and scoliosis in patients with Prader–Willi syndrome. Am J Med Genet A. 2006;140:1623–7.PubMedCrossRef
12.
go back to reference Ledbetter DH, Riccardi VM, Airhart SD, Strobel RJ, Keenan BS, Crawford JD. Deletions of chromosome 15 as a cause of the Prader–Willi syndrome. N Engl J Med. 1981;304:325–9.PubMedCrossRef Ledbetter DH, Riccardi VM, Airhart SD, Strobel RJ, Keenan BS, Crawford JD. Deletions of chromosome 15 as a cause of the Prader–Willi syndrome. N Engl J Med. 1981;304:325–9.PubMedCrossRef
13.
go back to reference Odent T, Accadbled F, Koureas G, Cournot M, Moine A, Diene G, Molinas C, Pinto G, Tauber M, Gomes B, de Gauzy JS, Glorion C. Scoliosis in patients with Prader–Willi syndrome. Pediatrics. 2008;122:e499–503.PubMedCrossRef Odent T, Accadbled F, Koureas G, Cournot M, Moine A, Diene G, Molinas C, Pinto G, Tauber M, Gomes B, de Gauzy JS, Glorion C. Scoliosis in patients with Prader–Willi syndrome. Pediatrics. 2008;122:e499–503.PubMedCrossRef
14.
go back to reference Takahashi Y, Kou I, Takahashi A, Johnson TA, Kono K, Kawakami N, Uno K, Ito M, Minami S, Yanagida H, Taneichi H, Tsuji T, Suzuki T, Sudo H, Kotani T, Watanabe K, Chiba K, Hosono N, Kamatani N, Tsunoda T, Toyama Y, Kubo M, Matsumoto M, Ikegawa S. A genome-wide association study identifies common variants near LBX1 associated with adolescent idiopathic scoliosis. Nat Genet. 2011;43:1237–40.PubMedCrossRef Takahashi Y, Kou I, Takahashi A, Johnson TA, Kono K, Kawakami N, Uno K, Ito M, Minami S, Yanagida H, Taneichi H, Tsuji T, Suzuki T, Sudo H, Kotani T, Watanabe K, Chiba K, Hosono N, Kamatani N, Tsunoda T, Toyama Y, Kubo M, Matsumoto M, Ikegawa S. A genome-wide association study identifies common variants near LBX1 associated with adolescent idiopathic scoliosis. Nat Genet. 2011;43:1237–40.PubMedCrossRef
15.
go back to reference Willner S, Nilsson KO, Kastrup K, Bergstrand CG. Growth hormone and somatomedin A in girls with adolescent idiopathic scoliosis. Acta Paediatr Scand. 1976;65:547–52.PubMedCrossRef Willner S, Nilsson KO, Kastrup K, Bergstrand CG. Growth hormone and somatomedin A in girls with adolescent idiopathic scoliosis. Acta Paediatr Scand. 1976;65:547–52.PubMedCrossRef
16.
go back to reference Nagai T, Matsuo N, Kayanuma Y, Tonoki H, Fukushima Y, Ohashi H, Murai T, Hasegawa T, Kuroki Y, Niikawa N. Standard growth curves for Japanese patients with Prader–Willi syndrome. Am J Med Genet. 2000;95:130–4.PubMedCrossRef Nagai T, Matsuo N, Kayanuma Y, Tonoki H, Fukushima Y, Ohashi H, Murai T, Hasegawa T, Kuroki Y, Niikawa N. Standard growth curves for Japanese patients with Prader–Willi syndrome. Am J Med Genet. 2000;95:130–4.PubMedCrossRef
17.
go back to reference Myers SE, Carrel AL, Whitman BY, Allen DB. Physical effects of growth hormone treatment in children with Prader–Willi syndrome. Acta Paediatr Suppl. 1999;88:112–4.PubMedCrossRef Myers SE, Carrel AL, Whitman BY, Allen DB. Physical effects of growth hormone treatment in children with Prader–Willi syndrome. Acta Paediatr Suppl. 1999;88:112–4.PubMedCrossRef
18.
go back to reference Obata K, Sakazume S, Yoshino A, Murakami N, Sakuta R. Effects of 5 years growth hormone treatment in patients with Prader–Willi syndrome. J Pediatr Endocrinol Metab. 2003;16:155–62.PubMedCrossRef Obata K, Sakazume S, Yoshino A, Murakami N, Sakuta R. Effects of 5 years growth hormone treatment in patients with Prader–Willi syndrome. J Pediatr Endocrinol Metab. 2003;16:155–62.PubMedCrossRef
19.
go back to reference Dymling JF, Willner S. Progression of a structural scoliosis during treatment with growth hormone: a case report. Acta Orthop Scand. 1978;49:264–8.PubMedCrossRef Dymling JF, Willner S. Progression of a structural scoliosis during treatment with growth hormone: a case report. Acta Orthop Scand. 1978;49:264–8.PubMedCrossRef
20.
go back to reference Morais T, Bernier M, Turcotte F. Age- and sex-specific prevalence of scoliosis and the value of school screening programs. Am J Public Health. 1985;75:1377–80.PubMedCentralPubMedCrossRef Morais T, Bernier M, Turcotte F. Age- and sex-specific prevalence of scoliosis and the value of school screening programs. Am J Public Health. 1985;75:1377–80.PubMedCentralPubMedCrossRef
21.
go back to reference Schuster DP, Osei K, Zipf WB. Characterization of alterations in glucose and insulin metabolism in Prader–Willi subjects. Metabolism. 1996;45:1514–20.PubMedCrossRef Schuster DP, Osei K, Zipf WB. Characterization of alterations in glucose and insulin metabolism in Prader–Willi subjects. Metabolism. 1996;45:1514–20.PubMedCrossRef
22.
go back to reference Angulo M, Castro-Magana M, Mazur B, Canas JA, Vitollo PM, Sarrantonio M. Growth hormone secretion and effects of growth hormone therapy on growth velocity and weight gain in children with Prader–Willi syndrome. J Pediatr Endocrinol Metab. 1996;9:393–400.PubMedCrossRef Angulo M, Castro-Magana M, Mazur B, Canas JA, Vitollo PM, Sarrantonio M. Growth hormone secretion and effects of growth hormone therapy on growth velocity and weight gain in children with Prader–Willi syndrome. J Pediatr Endocrinol Metab. 1996;9:393–400.PubMedCrossRef
23.
go back to reference Zipf WB. Glucose homeostasis in Prader–Willi syndrome and potential implications of growth hormone therapy. Acta Paediatr Suppl. 1999;88:115–7.PubMedCrossRef Zipf WB. Glucose homeostasis in Prader–Willi syndrome and potential implications of growth hormone therapy. Acta Paediatr Suppl. 1999;88:115–7.PubMedCrossRef
Metadata
Title
The characteristics of scoliosis in Prader–Willi syndrome (PWS): analysis of 58 scoliosis patients with PWS
Authors
Yutaka Nakamura
Nobuyuki Murakami
Takahiro Iida
Satoru Ozeki
Satoshi Asano
Yutaka Nohara
Toshiro Nagai
Publication date
01-01-2015
Publisher
Springer Japan
Published in
Journal of Orthopaedic Science / Issue 1/2015
Print ISSN: 0949-2658
Electronic ISSN: 1436-2023
DOI
https://doi.org/10.1007/s00776-014-0651-y

Other articles of this Issue 1/2015

Journal of Orthopaedic Science 1/2015 Go to the issue