Resumen

CORRADO, Romina María Rosa et al. Sotos syndrome and scoliosis Surgical treatment: a 10-year follow-up. Rev. Asoc. Argent. Ortop. Traumatol. [online]. 2010, vol.75, n.3, pp.268-274. ISSN 1852-7434.

Background: 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. Methods: We retrospectively evaluated 42 patients with a diagnosis of Sotos syndrome treated at the Garrahan Children Hospital between 1988 and March 2009. Clinical charts and imaging studies were assessed. Results: Eight patients (19%) presented with scoliosis and 7 of them (87%) required surgical treatment. The mean follow-up was 9.5 years (range, 3-18 years). Mean age at first consultation was 5.2 years (range, 1.2-11.2). Mean angle value (MAV) at first consultation was 34.3° (range 20º-42º). Mean age at surgery was 11.2 years (range, 3-18.10). The surgical procedures performed were instrumented posterior arthrodesis, alone or combined with anterior arthrodesis, instrumented anterior arthrodesis, and one patients being currently treated with growing rods. Presurgical MAV was 72.3° (range 46º-130º) and postsurgical MAV was 45.5° (range 6º-90º). 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 and dislodgement of the proximal hooks). Conclusiones: The incidence of scoliosis in Sotos syndrome is higher than that reported for the general population. Close monitoring of these patients during growth is important for early diagnosis. At the moment of surgery, joint hyperextensibility and hypotonia should be considered and short fusions should not be used in order to avoid complications.

Palabras clave : Cerebral gigantism; Scoliosis deformity.

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