Vertebral Anomalies and Myelodysplasias

In the literature there are essentially no reports concerning late problems related to vertebral anomalies in patients with ARM. In the author's consecutive series of 375 ARM during the period 1984-1994, 2 patients have required spinal bracing and 4 required operative spinal stabilization because of progressive scoliosis. A report from the same institution in adults with ARM noted that 18 (16%) out of 116 patients reported symptoms, mainly chronic back pain, related to their vertebral anomalies [62,83].

Recently, much attention has been placed on the occurrence of myelodysplasias in patients with ARM [26,96,97]. The effect of spinal abnormalities, especially tethered cord, on long-term functional outcome in terms of bladder and bowel function or neurological symptoms in the lower extremities is unclear, although some recent reports suggest that worsening of the function due to spinal anomalies is possible. DeGennaro et al. [86] found a higher incidence of spinal dysraphism associated neurovesical dysfunction in older patients than in infants. Sato et al. [97] reported aggravation of neurological symptoms related to tethered cord in a small group of patients with ARM. In the author's series, only 3 out of 375 patients have required detethering because of progressive neurological symptoms, which were completely alleviated only in 1 patient. There appears to be no evidence to support prophylactic detethering of patients who do not have specific symptoms related to tethering [98,99]. There is also no evidence to support the concept that tethered cord affects functional outcome in terms of fecal or urinary continence in patients with ARM [98,100]. Screening for spinal abnormalities for diagnostic purposes is, however, indicated in patients with ARM, including those with low anomalies [52,96]. Normal vertebral anatomy on spinal x-rays does not preclude the presence of spinal cord abnormalities [96]. Screening can be performed by ultrasound during early infancy or by MRI at any age.

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