Diagnosis and Management of Marfan Syndrome by Anne H. Child

Author:Anne H. Child
Language: eng
Format: epub
Publisher: Springer London, London

Scoliosis in MFS

The axial skeleton is the most commonly affected part of skeletal system in MFS. The reported incidence of scoliosis in established diagnosis of MFS varies from 40 to 60 % [10]. In 1939, the first reported series, published by Fahey et al., had 45 scoliosis patients from 132 MFS cases [11]. Subsequently, Sinclair et al. (1960), Wilner et al. (1964), and Scheier et al. (1967) observed scoliosis of mild to moderate severity [12–14]. Ford et al. (1968), in their review of MFS, found at least 13 % of patients had severe scoliosis which required aggressive bracing, fusion, or both [15]. Sliman et al. (1971) were the first to observe striking similarities and unique differences between the scoliosis of MFS vs. idiopathic etiology, and they concluded that MFS scoliosis has a poorer prognosis [16]. Robins et al. (1975) reported a 44 % incidence of scoliosis (35 out of 64 MFS patients with scoliosis) and observed poor response to treatment with a Milwaukee brace (MB) [17]. Spinal fusion with Harrington instrumentation yielded a 41 % correction with an average loss of 7° over 2.3 years of follow-up [17]. Sponseller et al evaluated 113 patients with MFS from their hospital database and found that 82 of them were skeletally immature. 52 out of 82 patients had scoliosis, and all but two (i.e. 50 out of 82) had scoliosis which was convex to the right [18]. The incidence of scoliosis in their series was 60 %. The thoracic spine was the most common region affected, followed by the thoracolumbar junction. Though these curves resembled the idiopathic curve type/pattern, certain unique features were evident. MFS patients had a higher incidence of having double thoracic or triple major curves. In addition, on the sagittal plane, there was a loss of thoracic kyphosis (TK) with reversal to thoracic lordosis in the most severe cases [18]. This, coupled with pectus excavatum, resulted in a reduced AP diameter of the chest with resultant mechanical compression of the large airways, which predisposed these patients to recurrent chest infections. Fewer patients had hyperkyphosis (i.e. TK of >50°) which was seen in 40 %.

The scoliosis in MFS differs significantly from idiopathic scoliosis by having rapid progression with a poor response to non-operative treatment. Furthermore, vertebral morphology is affected significantly, which makes operative treatment challenging. The key findings in vertebral bodies and posterior elements in children with MFS scoliosis include [19]:Narrow pedicles,

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