caa a22 4500 44583367X CHVBK 20180317145323.0 cr unu---uuuuu 170323e20110701xx s 000 0 eng 10.1007/s00586-010-1685-2 doi (NATIONALLICENCE)springer-10.1007/s00586-010-1685-2 Sotos syndrome and scoliosis surgical treatment: a 10-year follow-up [Elektronische Daten] [Romina Corrado, Alejandra Wilson, Carlos Tello, Mariano Noel, Eduardo Galaretto, Ernesto Bersusky] Sotos syndrome is caused by a gene deletion with an autosomal dominant pattern of inheritance. Cerebral gigantism, hypotonia and joint hyperextensibility are characteristic features of this syndrome. A percentage of these patients develop progressive scoliosis early in life. In the literature, few studies on the evolution of scoliosis in Sotos syndrome have been published. We retrospectively evaluated eight patients diagnosed with Sotos syndrome and scoliosis treated at the Garrahan Children Hospital between 1988 and March 2009. Clinical charts and imaging studies were assessed. Eight patients (19%) presented with scoliosis and seven of them (87.5%) required surgical treatment. The mean follow-up was 9.5years (range 3-18). Mean age at first consultation was 5.2years (range 1.1-11.2). Mean Cobb angle for scoliosis at first consultation was 34.3° (range 20°-42°) and the mean Cobb angle for kyphosis was 45.6° (range 30°-90°). Mean age at surgery was 11.2years (range 3.7-18.10). The surgical procedures performed were instrumented posterior arthrodesis, alone or combined with anterior arthrodesis, instrumented anterior arthrodesis, while one patient is currently in treatment with growing rods. Preoperative mean Cobb angle for scoliosis was 72.3° (range 54°-130°) and for kyphosis was 59.8° (range 30°-108°); postoperative mean Cobb angle for scoliosis was 45.5° (range 6°-90°) and for kyphosis was 40.2° (range 30°-80°). There were three early complications (pleural effusion in two cases and death due to sepsis in one) and two late complications (kyphosis above the instrumentation area and dislodgement of the proximal hooks). Incidence of scoliosis in Sotos syndrome is high and thus close monitoring of patients with Sotos syndrome during growth is important for early detection of this entity. Joint hyperextensibility and hypotonia that are characteristic of the syndrome should be considered at the moment of surgery to avoid short fusions. Springer-Verlag, 2011 Cerebral gigantism nationallicence Scoliosis deformity nationallicence Corrado Romina J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut Wilson Alejandra J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut Tello Carlos J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut Noel Mariano J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut Galaretto Eduardo J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut Bersusky Ernesto J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut European Spine Journal Springer-Verlag 20(2011-07-01), 271-277 0940-6719 20<271 2011 20 586 https://doi.org/10.1007/s00586-010-1685-2 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00586-010-1685-2 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Corrado Romina J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut NATIONALLICENCE 700 1- Wilson Alejandra J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut NATIONALLICENCE 700 1- Tello Carlos J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut NATIONALLICENCE 700 1- Noel Mariano J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut NATIONALLICENCE 700 1- Galaretto Eduardo J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut NATIONALLICENCE 700 1- Bersusky Ernesto J.P. Garrahan Pediatric Hospital, Pichincha, 1889, Ciudad Autónoma de Buenos Aires, Argentine aut NATIONALLICENCE 773 0- European Spine Journal Springer-Verlag 20(2011-07-01), 271-277 0940-6719 20<271 2011 20 586 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer