Myelodysplasia: The Natural History of Kyphosis and Scoliosis. A Preliminary Report

Abstract

SUMMARY The results of this study support the concept that the levels of the bifid vertebral arch and of functional motor activity are significant influences on the development of scoliosis and kyphosis in patients born with myelodysplasia. ‘Significant’ degrees of scoliosis and kyphosis may appear at any time during life, but most frequently occur during late childhood or adolescence. Not all ‘significant’ curvatures require treatment, nor do all children with myelodysplasia and factors predisposing to kyphosis and scoliosis develop these complications. It is believed that the preliminary cumulative percentage curves presented in this study will be of value in counselling parents of afflicted newborn infants, and in planning the management of patients born with myelodysplasia. RÉSUMÉ Histoire naturelle de la cyphose et de la scoliose dans la myelodysplasie: rapport préliminaire Les résultats de cette étude favorisent l'hypothèse que les particularités de l'are vertébral bifide et de l'activité motrice fonctionnelle constituent les influences significatives du développement de cyphose et de scoliose chez les sujets nés avec myélodysplasie. Des degrés ‘significatifs’ de scoliose et de cyphose peuvent apparaitre à n'importe quel moment de la vie, mais se manifestent le plus habituellement durant la troisième enfance et l'adolescence. Toutes les courbures ‘significatives’ ne nécessitent pas de traitement et tous les enfants atteints de myélodysplasie avec facteurs prédisposant à la cyphose et à la scoliose ne présentent pas ces complications. Les auteurs pensent que les courbes préliminaries de pourcentage cumulatif présentées dans cette étude auront une bonne valeur dans le conseil aux parents de nouveaux‐nés atteints et dans la prévision de traitement des sujets atteints de myélodysplasie. ZUSAMMENFASSUNG Biologie der Kyphose und Skoliose bei Myelodysplasie: ein vorläufiger Bericht Die Ergebnisse dieser Arbeit unterstützen das Konzept, daß das Ausmaß der Wirbelbogenspaltung und der Funktionsstörung der motorischen Aktivität stark die Entwicklung der Skoliose und Kyphose bei Patienten mit einer Myelodysplasie beeinflussen. ‘Signifikante’ Grade von Skoliose und Kyphose konnen in jedem Lebensabschnitt auftreten, aber am allermeisten entwickeln sie sich während der Pubertät und im Adoleszentenalter. Nicht jede ‘signifikante’ Verbiegung bedarf einer Behandlung, noch entwickeln alle Kinder mit Myelodysplasie und Faktoren, die als eine Disposition für eine Kyphose und Skoliose angesehen werden, diese Kompiikationen. Die Autoren sind der Auffassung, daß diese vorläufigen Percentilenkurven, die in der Arbeit vorgelegt werden, bei der Beratung der Eltern von betroffenen neugeborenen Kindern wertvoll sein werden, ferner für die Planung der weiteren Behandlung von Patienten mit der angeborenen Myelodysplasie. RESUMEN Historia natural de la cifosis y escoliosis en la mielodisplasia: comunicación previa Los resultados de este estudio apoyan el concepto de que los niveles del arco vertebral bífido y de la actividad motora fun...

Obesity and Myelomeningocele: Anthropometric Measures

“…As demonstrated in Tables 3 through 5 and Figures 1 and 2, we would agree with the observation that arm span is not equivalent to height for all patients with meningomyelocele (20,21) because of deformities of the spine and lower extremities in many of these children (1)(2)(3)(10)(11)(12). That arm span is not equivalent to height is, we believe, irrelevant to the stated objective of this paper.…”

“…While patients were followed there, no linear measures are recorded; the last height measure may have been 1 to 11 months before surgery. These smaller differences than those demonstrated in Figure 7b and Tables 3 through 5 may also be explained by fewer patients requiring surgical correction of the scoliosis, the minimal levels of curve remaining after insertion of rods that are extended periodically to account for growth, and the large percent of patients in all 3 groups who developed scoliosis but did not progress to surgery (12).…”

“…The increasing divergence after age 8 years is due to the added effect of scoliosis (10,12) during this time of rapid growth (15) associated with the beginning of puberty (32). The difference in the widening of the discrepancy between height and arm span is less evident in the intermediate paralyzed group (LL2) (Tables 3-5, Figure 2) and less so in the least paralyzed (LL3) (Tables 3-5, Figure 3) because of less severe paralysis (1) and a lower frequency of scoliosis (12). Figure 7 illustrates the effect of scoliosis on BMI when calculated using height vs arm span.…”

“…These children have a high incidence of decreased height or linear length secondary to kyphosis, scoliosis, abnormal vertebrae, and lower extremity hypoplasia associated with their myelomeningocele (3,4,6,(10)(11)(12). A number of studies have reported the value of using arm span as a substitute for estimating body functions (eg, pulmonary function, growth) and for calculating medication dosages for people with scoliosis or when height or length are difficult to obtain (13)(14)(15)(16)(17)(18)(19).…”

Management of spinal cord injury–related scoliosis using pedicle screw–only constructs

“…Although the more rostral extent of neurological impairment in patients with myelomeningocele has been strongly correlated with the risk of scoliosis development, that association has not been consistently reproduced among patients with SCI. 2,4,16,18,24,26,30,31 Furthermore, patients with myelomeningocele represent a congenital developmental process as opposed to SCI, which is a postdevelopmental injury. Modi et al compared pedicle screw constructs in neuromuscular scoliosis cases and noted a 58% correction from 82° to 34°.…”